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ISSN 0004-282X

ISSN 0004-282X

Arquivos de Volume 79, Separata, Março 2021


Arquivos de
Neuro-psiquiatriA
Volume 80, Number 9 Suppl 2, September 2022

Neuro-psiquiatriA

Demências no Brasil: aumento da carga no período de 2000 a 2016.


Estimativas do Estudo Carga Global de Doenças 2016

A depressão enquanto um dos principais determinantes das


anormalidades do sono em pacientes com epilepsia

ACADEMIA
BRASILEIRA Material destinado exclusivamente à classe médica
DE NEUROLOGIA
Arquivos de Volume 80, Number 9 Suppl 2, September 2022

Neuro-psiquiatriA
THE OFFICIAL JOURNAL OF THE ACADEMIA BRASILEIRA DE NEUROLOGIA (BRAZILIAN ACADEMY OF NEUROLOGY)

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Ayrton Roberto Massaro (São Paulo, Brazil)
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ASSOCIATE EDITORS
Cerebrovascular Disorders, Interventional Neuroradiology Movement Disorders
and Neurointensive Care José Luiz Pedroso (São Paulo, Brazil)
Gabriel Rodriguez de Freitas (Rio de Janeiro, Brazil) Laura Silveira Moriyama (Campinas, Brazil)
Jamary Oliveira Filho (Salvador, Brazil) Renato Puppi Munhoz (Toronto, Canada)
João Brainer Clares de Andrade (São Paulo, Brazil) Vitor Tumas (Ribeirão Preto, Brazil)
Marcos Christiano Lange (Curitiba, Brazil)
Octávio Marques Pontes Neto (Ribeirão Preto, Brazil) Neuromuscular Disorders and Peripheral Neuropathy
Sheila Cristina Ouriques Martins (Porto Alegre, Brazil) Edmar Zanoteli (São Paulo, Brazil)
Child Neurology Francisco de Assis Aquino Gondim (Fortaleza, Brazil)
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Alexandra Pruffer Queiroz Campos Araújo (Rio de Janeiro, Brazil) Wilson Marques Jr. (Ribeirão Preto, Brazil)
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Luciano De Paola (Curitiba, Brazil)
Luís Otávio Sales Ferreira Caboclo (São Paulo, Brazil)
Paulo José Lorenzoni (Curitiba, Brazil) Neuroimaging
Rosana Hermínia Scola (Curitiba, Brazil) Antonio José da Rocha (São Paulo, Brazil)
Carolina de Medeiros Rimkus (Campinas, Brazil)
Cognitive Neurology and Neuropsychology Celi dos Santos Andrade (São Paulo, Brazil)
Leonardo Cruz de Souza (Belo Horizonte, Brazil) Leandro Tavares Lucato (São Paulo, Brazil)
Michael Hornberger (Norwich, United Kingdom)
Mônica Sanchez Yassuda (São Paulo, Brazil) Neuroimmunology
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Tarso Adoni (São Paulo, Brazil)
Epilepsy
Clarissa Lin Yasuda (Campinas, Brazil) Yara Dadalti Fragoso (Santos, Brazil)
Fábio A. Nascimento (Boston, USA)
Lécio Figueira Pinto (São Paulo, Brazil) Neurological Rehabilitation
Adriana Bastos Conforto (São Paulo, Brazil)
Experimental Neurology and Neuroscience Chien Hsin Fen (São Paulo, Brazil)
Grace Schenatto Pereira Moraes (Belo Horizonte, Brazil)
Karina Braga Gomes (Belo Horizonte, Brazil) Neuro-oncology
Luciene Covolan (São Paulo, Brazil) Suzana Maria Fleury Malheiros (São Paulo, Brazil)
Vivaldo Moura Neto (Rio de Janeiro, Brazil)
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Lea Tenenholz Grinberg (San Francisco, USA)
Eduardo Genaro Mutarelli (São Paulo, Brazil)
Orlando Graziani Povoas Barsottini (São Paulo, Brazil) Sérgio Rosemberg (São Paulo, Brazil)
Mariana Spitz (Rio de Janeiro, Brazil)
Neuropsychiatry
Headache Analuiza Camozzato (Porto Alegre, Brazil)
Mário Fernando Prieto Peres (São Paulo, Brazil) Antonio Lucio Teixeira (Houston, USA)
Pedro André Kowacs (Curitiba, Brazil)
Pedro Sampaio (Recife, Brazil) Pain
History of Neurology Daniel Ciampi de Andrade (São Paulo, Brazil)
Carlos Henrique Ferreira Camargo (Curitiba, Brazil) Fabíola Dach Eckeli (Ribeirão Preto, Brazil)
Ethel Mizrahy Cuperschmid (Belo Horizonte, Brazil)
Ylmar Correa Neto (Florianópolis, Brazil) Sleep Disorders
Dalva Poyares (São Paulo, Brazil)
Images in Clinical Neurophysiology Rosana Cardoso Alves (São Paulo, Brazil)
Fábio A. Nascimento (Boston, USA)
Karen Nunez-Wallace (Houston, USA)
Social Media and Visual Abstract
Infectious Diseases of the Nervous System and Cerebrospinal Fluid Studies Camila Callegari Piccinin (Campinas, Brazil)
Cristiane Nascimento Soares (Rio de Janeiro, Brazil) Elisa de Paula França Resende (Belo Horizonte, Brazil)
Marzia Puccioni-Sohler (Rio de Janeiro, Brazil) Eva Carolina Rocha (São Paulo, Brazil)
Paulo Pereira Christo (Belo Horizonte, Brazil) João Brainer Clares de Andrade (São Paulo, Brazil)
Sérgio Monteiro de Almeida (Curitiba, Brazil) Sylvie Devalle (Rio de Janeiro, Brazil)

EDITORIAL BOARD
Acary de Souza Bulle Oliveira (São Paulo, Brazil) Américo Ceiki Sakamoto (Ribeirão Preto, Brazil)
Alberto J. Espay (Cincinatti, USA) Andrew J. Lees (London, United Kingdom)
Alexis Brice (Paris, France) Bruce L. Miller (San Francisco, USA)
Andrea Slachevsky (Santiago, Chile) Bruce Ovbiagele (Charleston, USA)
Carlos Alberto Mantovani Guerreiro (Campinas, Brazil) Maria José Sá (Porto, Portugal)
Carlos Roberto de Mello Rieder (Porto Alegre, Brazil) Maria Lúcia Brito Ferreira (Recife, Brazil)
Christina Marra (Seattle, USA) Marilisa Mantovani Guerreiro (Campinas, Brazil)
Didier Leys (Lille, France) Mônica Levy Andersen (São Paulo, Brazil)
Fernando Cendes (Campinas, Brazil) Oscar Del Brutto (Guayaquil, Ecuador)
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Jaderson Costa da Costa, (Porto Alegre, Brazil) Ricardo Allegri (Buenos Aires, Argentina)
João José Freitas de Carvalho (Fortaleza, Brazil) Ricardo Nitrini (São Paulo, Brazil)
Joaquim Ferreira (Lisbon, Portugal) Roger Walz (Florianópolis, Brazil)
Joaquim Pereira Brasil Neto (Brasília, Brazil) Rubens José Gagliardi (São Paulo, Brazil)
José Manuel Ferro (Lisbon, Portugal) Sérgio Teixeira Ferreira (Rio de Janeiro, Brazil)
Lineu César Werneck (Curitiba, Brazil) Stefan Schwab (Erlangen, Germany)
Luiz Henrique Martins Castro (São Paulo, Brazil) Ugo Nocentini (Roma, Italy)
Márcia Lorena Fagundes Chaves (Porto Alegre, Brazil) Umbertina Conti Reed (São Paulo, Brazil)
Marco Aurélio Lana-Peixoto (Belo Horizonte, Brazil) Vladimir Hachinski (London, Canada)
Marcos Raimundo Gomes de Freitas (Rio de Janeiro, Brazil) Walter A. Rocca (Rochester, USA)

Arquivos de Neuro-Psiquiatria is a periodic registered in the Departamento de Imprensa e Propaganda (11795), Departamento Nacional
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DIRETORIA EXECUTIVA ABN

Presidente: Dr. Carlos Roberto M. Rieder (RS) – 2020-2024


Vice-presidente: Dr. Delson José da Silva (GO) – 2020-2024
Secretária Geral: Dra. Gisele Sampaio (SP) – 2020-2022
1º Secretária: Dra. Jerusa Smid – (SP) 2020-2022
Tesoureiro Geral: Dr. Edmar Zanoteli (SP) – 2020-2022
1º Tesoureiro: Dr. José Luiz Pedroso (SP) – 2020-2022
Diretor Científico: Rubens José Gagliardi

COMISSÃO ORGANIZADORADO XXIX CONGRESSO BRASILEIRO DE NEUROLOGIA – 2022

Presidente do Congresso: Dr Norberto Frota


Secretária: Dra. Fernanda Maia
Tesoureiro: Dr. Pedro Braga Neto
Coordenador da Comissão Científica: Dr. Manoel Sobreira
Presidente de Honra: Dr. João José Carvalho
COMISSÃO CIENTÍFICA

Abelardo de Queiroz C. Araújo


Alan Luiz Eckeli
Américo Ceiki Sakamoto
Ana Paula Gonçalves
Carlos Rieder
Carolina Figueredo
Claudia Ferreira da Rosa Sobreira
Clécio de Oliveira Godeiro Junior
Cleonisio Leite Rodrigues
Daniel Ciampi de Andrade
Fabrício Oliveira Lima
Francisco Marcos Bezerra Cunha
Jamary Oliveira Filho
Jerusa Smid
José Luiz Pedroso
Leandro Lucato
Lívia Dutra
Lucas Silvestre Mendes
Luis Otávio Caboclo
Marcelo Calderaro
Marcelo Mashura
Marcio Nattan Portes Souza
Marcondes Cavalcante França Jr.
Maria Lucia Brito Ferreira
Mário Peres
Orlando Graziani Povoas Barsottini
Paulo Caramelli
Paulo Ribeiro Nóbrega
Ricardo Nitrini
Samir Câmara Magalhães
Sônia Brucki
Suzete Nascimento Farias da Guarda
Tarso Adoni
Wagner Avelar
Arquivos de Volume 80, Number 9 Suppl 2, September 2022

Neuro-psiquiatriA
XXX CONGRESSO BRASILEIRO DE NEUROLOGIA

SUMÁRIO

RELATO DE CASO

Ataxias .......................................................................................................................................................................... 9
Cefaleia ....................................................................................................................................................................... 15
Distúrbio Vestibulares e do Equilíbrio ..................................................................................................................... 21
Doença Cerebrovascular ..........................................................................................................................................23
Doenças do Neurônio Motor – Esclerose Lateral Amiotrófica ..............................................................................49
Doenças Neuromusculares ......................................................................................................................................50
Dor .............................................................................................................................................................................73
Educação Médica ......................................................................................................................................................75
Epilepsia ..................................................................................................................................................................... 76
Líquido cefalorraquiano ............................................................................................................................................80
Miscelânea .................................................................................................................................................................80
Neuroepidemiologia ................................................................................................................................................104
Neurofisiologia Clínica ............................................................................................................................................104
Neurogenética ......................................................................................................................................................... 107
Neuroimunologia ..................................................................................................................................................... 121
Neuroinfecção .........................................................................................................................................................148
Neurointensivismo .................................................................................................................................................. 170
Neurologia Cognitiva e do Envelhecimento .......................................................................................................... 172
Neurologia Infantil ................................................................................................................................................... 179
Neurologia Intervencionista ...................................................................................................................................183
Neuropatias Periféricas ..........................................................................................................................................184
Neurorreabilitação ..................................................................................................................................................195
Neurossonologia .....................................................................................................................................................196
Sono ..........................................................................................................................................................................196
Transtornos do Movimento .....................................................................................................................................198
Traumatismo cranioencefálico .............................................................................................................................. 210
TRABALHO CIENTÍFICO

Ataxias ...................................................................................................................................................................... 212


Cefaleia ..................................................................................................................................................................... 215
Distúrbio Vestibulares e do Equilíbrio ...................................................................................................................221
Doença Cerebrovascular ........................................................................................................................................221
Doenças do Neurônio Motor – Esclerose Lateral Amiotrófica ............................................................................ 242
Doenças Neuromusculares ....................................................................................................................................246
Dor ...........................................................................................................................................................................262
Educação Médica ....................................................................................................................................................263
Epilepsia ...................................................................................................................................................................267
História da Neurologia ............................................................................................................................................273
Líquido cefalorraquiano ..........................................................................................................................................275
Miscelânea ...............................................................................................................................................................276
Neuroepidemiologia ................................................................................................................................................280
Neurofisiologia Clínica ............................................................................................................................................291
Neurogenética .........................................................................................................................................................293
Neuroimunologia .....................................................................................................................................................297
Neuroinfecção .........................................................................................................................................................308
Neurointensivismo .................................................................................................................................................. 318
Neurologia Cognitiva e do Envelhecimento .......................................................................................................... 318
Neurologia Infantil ...................................................................................................................................................332
Neurologia Intervencionista ...................................................................................................................................340
Neuropatias Periféricas .......................................................................................................................................... 341
Neurorreabilitação ..................................................................................................................................................346
Neurossonologia .....................................................................................................................................................355
Sono ..........................................................................................................................................................................356
Transtornos do Movimento .....................................................................................................................................366
Traumatismo cranioencefálico .............................................................................................................................. 374

ÍNDICE DE AUTORES

Índice de Autores .....................................................................................................................................................377


RELATO DE CASO
XXX Congresso Brasileiro de Neurologia

TL 1104864 TL 1105157 TL 1105253

A POTENTIALLY TREATABLE HEREDITARY PROGRESSIVE ATAXIA AND PALATAL FRAGILE X-ASSOCIATED TREMOR/ATAXIA
SPASTIC PARAPLEGIA MIMICKER: SPINAL TREMOR: CASE PRESENTATION OF A RARE SYNDROME: CASE PRESENTATION
CEREBROTENDINOUS XANTHOMATOSIS DISEASE
Fabiana Carla dos Santos Correia, André Iglesias
Heloisa Lopes Cohim Moreira, Vinícius Lopes Braga, Pedro Machry Pozzobon, Ana Beatriz Marangoni Brandão, Sérgio Roberto Pereira da Silva Júnior,
Wladimir Bocca Vieira de Rezende Pinto, Igor Braga Baston, Patrick Emanuell Mesquita Sousa Santos, Marcus Vinicius de Souza, Rafael Henrique Neves
Farias, Paulo Victor Sgobbi de Souza, Acary Souza Tarcísio Nunes Alvarenga, João Vitor Mortari Lisboa, Gomes, Daniel Sabino de Oliveira, Thiago Cardoso
Bulle Oliveira Igor Oliveira Fonseca, Ana Flavia Andrade Lemos, Vale
José Eduardo Trentim Longo, Helio Aquaroni Farao
Universidade Federal de São Paulo. São Paulo SP, Hospital Universitário da Universidade Federal de
Gomes, Laura Cardia Gomes Lopes
Brazil Juiz de Fora. Juiz de Fora MG, Brazil
Hospital de Clínicas da Faculdade de Medicina de
heloisalcm@gmail.com fabi-karlla@hotmail.com
Botucatu – UNESP. Botucatu SP, Brazil

Case Presentation: A 23-year-old brazilian man pre- pedro_machry_pozzobon@hotmail.com Case Presentation: A 66-year-old man presented with
sented with a slowly progressive history of unsteady gait, imbalance and falls, upper-limb tremor and erectile dys-
lower limb stiffness and numbness involving both feet Case Presentation: A 54-years-old female patient, with function for the last two years. Symptoms progressively
since 15 years old. Parents were both first degree cousins hypertension and obesity, presented with a 3-years his- worsened during the period and patient became more
(consanguineous parents). Examination disclosed global tory of progressive speech disorder, evolving with hoarse irritable, impulsive and developed impaired memory.
brisk tendon reflexes, extensor plantar responses, mod- voice and slurred speech. She also had gait instability Neurological examination revealed ataxic gait, dysmetria,
erate spastic paraparesis, and mild reduction in vibratory and dysphagia. Such changes were better perceived by dysdiadochokinesia, bilateral postural and action tremor,
sense in the lower limbs. Mignarri score: 100. General lab family members than by the patient herself. The physi- and global areflexia with distal lower-limb hypopalles-
and biochemical screening were unremarkable. Nerve cal examination showed slurred speech, palatal tremor, thesia. MOCA test was 19/30 with 11 years of schooling.
conduction studies showed demyelinating and axonal gait with a slightly widened base and presence of dysme- Brain MRI showed cerebral and cerebellar atrophy, and
sensorimotor polyneuropathy. Needle electromyography tria and dysdiadochokinesia in left upper limb. A MRI symmetrical hyperintensities in the middle cerebellar
studies remained unremarkable. Neuroimaging studies brain showed only moderate cerebellar atrophy and peduncles, periventricular regions and corpus callosum,
disclosed mild cervical spine atrophy, marked signal bilateral and signal abnormality in the inferior olivary which was also atrophic (Figure). Electrophysiological
changes involving the dorsal column and lateral corti- nuclei bilaterally. Her clinical presentation and neuro- study revealed a sensory-motor polyneuropathy. Genetic
cospinal tracts of the cervical and thoracic spinal cord. imaging finding supportt the diagnosis of progressive testing for the fragile X mental retardation 1 (FMR1) gene
Gene panel testing including Hereditary Spastic Paraplegia ataxia and palatal tremor (PAPT). Discussion: PAPT is a showed 111 CGG trinucleotide repeats, confirming the
(HSP) and Charcot-Marie-Tooth disease genes disclosed rare and progressive neurodegenerative disease associ- diagnosis of fragile X-associated tremor-ataxia syndrome
the pathogenic variant c.1183C>T (p.Arg395Cys) in homo- ated with brainstem and cerebellum lesions, especially (FXTAS). Discussion: FXTAS is caused by the expansion
zygosis in CYP27A1 gene, defining the genetic diagnosis of at Guillain-Mollaret Triangle or dentato-rubro-olivary of the CGG trinucleotide in the FMR1 gene (CGG between
Cerebrotendinous Xanthomatosis (CTX). Plasma choles- pathway (DROP). PAPT may be divided into sporadic 55-200 repeats. Clinical picture comprises the presence
tanol levels were assessed and showed increased levels. and familial forms. Familial causes includes Alexander’s of one or more characteristics, in different combinations:
Serum cholesterol and triglyceride levels were normal. disease, polymerase gamma mutations, spinocerebellar cerebellar ataxia, parkinsonism, appendicular tremor,
Ophthalmologic evaluation was normal. Patient was ataxia type 20, and GM2 gangliosidosis. Sporadic cases neuropathy, dysautonomia, behavioral and cognitive
started on statins (simvastatin). Chenodeoxycholic acid are still poorly understood, with adulthood onset, and an symptoms. Diagnosis is made by major and / or minor
was not available. Discussion: CTX is a rare autosomal uncertain cause. In some previous reports, histopatho- clinical and radiological signs : The most important find-
recessive inherited metabolic disorder due to pathogenic logical analysis point to TAU 3R and 4R pathology and ing is the middle cerebellar peduncles hyperintensities
variants in the CYP27A1 gene. Typical systemic and neu- hypertrophic olivary degeneration (HOD) as the cause of seen on T2-weighted brain MRI sequences. Minor diag-
roradiological findings represent important clinical clues sporadic form. PAPT clinical features are palatal tremor nostic criteria include other radiological findings such
for high suspicion in different neurological contexts. CTX (usually incidental), ataxia of limbs and gait, dysarthria as white matter hyperintensities and diffuse cerebral
presents with different neurological phenotypes, including and dysphagia, in addition to other signs and symptoms atrophy. Corpus callosum atrophy with hyperintensity
spastic paraparesis (spinal CTX), lower motor neuron dis- of brainstem or autonomic disturbances. Typical MRI of the splenium, dentate nucleus and pons may also
ease, cerebellar ataxia, dystonia, parkinsonism, epilepsy, abnormalities are high signal in the ION and cerebelar occur. Definitive diagnosis requires the presence of the
and polyneuropathy. Spinal CTX has been reported in atrophy. At the present moment, there is no treatment premutation associated with the presence of one major
association with other common systemic signs, includ- to prevent the disease progression. Final Comments: radiological sign plus one major clinical symptom or the
ing chronic diarrhea, juvenile cataracts, and osteopenia. Progressive Ataxia and Palatal Tremor (PAPT) is a rare presence of FXTAS inclusions in neuropathology. Final
However, pure neurological presentations without early neurodegenerative disease and there are relatively few Comments: It is to be emphasized the importance of
systemic signs may also occur, representing a complex studies describing this syndrome. Careful examination is FXTAS investigation in patients with late onset ataxia
diagnostic challenge, mainly in contexts with broad dif- important to identification of this presentation. especially when associated to parkinsonism, tremor and
ferential diagnosis. Spinal CTX is commonly a late diag- peripheral polyneuropathy. However, there is, at present,
nosis. Mignarri index for CTX suspicion must not limit no specific targeted treatment for FXTAS and management
diagnostic evaluation in patients with pure neurological Ataxias remains only symptomatic.
presentation, as early CTX treatment changes disease
natural history. Final Comments: Spinal CTX must be
included in the differential diagnosis of HSP phenotypes, Ataxias
even in cases without marked multisystem involvement.

Ataxias

9
XXX Congresso Brasileiro de Neurologia

TL 1105253 TL 1105438 TL 1105507

FRAGILE X-ASSOCIATED TREMOR/ATAXIA ATYPICAL MANIFESTATIONS OF LATE- A MAN WITH SPORADIC PERRAULT
SYNDROME: CASE PRESENTATION ONSET FRIEDREICH’S ATAXIA – CASE SYNDROME – A CASE PRESENTATION
PRESENTATION
Fabiana Carla dos Santos Correia, André Iglesias Thábata Emanuelle Martins Nunes, Patricia Aurea
Brandão, Sérgio Roberto Pereira da Silva Júnior, André Bertola Vanzan Filho, Mariana Ribeiro Andreucci Martins Bonilha, Beatriz Cassarotti, Luiz
Marcus Vinicius de Souza, Rafael Henrique Neves Pererira, Vivian Pinto de Almeida, Patricia Gomes Eduardo Novis, Léo Coutinho, Francisco Manoel
Gomes, Daniel Sabino de Oliveira, Thiago Cardoso Pinheiro, Rayanne da Silva Souza, Deborah Santos Branco Germiniani, Helio Afonso Ghizoni Teive
Vale Sales, Clarissa de Araújo Davico, Daniel Lucas
Hospital de Clínicas da Universidade Federal do
de Lima Silva Santos, Francisco Ramon Canale
Hospital Universitário da Universidade Federal de Paraná. Curitiba PR, Brazil
Ferreira, Karina Lebeis Pires
Juiz de Fora. Juiz de Fora MG, Brazil
thabatamnunes@gmail.com
Universidade Federal do Estado do Rio de Janeiro.
fabi-karlla@hotmail.com
Rio de Janeiro RJ, Brazil
Case Presentation: Male patient, 35 years old, 12 years
Casa Hunter. Rio de Janeiro RJ, Brazil
Case Presentation: A 66-year-old man presented with ago started with gait ataxia and vertigo. Previous history of
imbalance and falls, upper-limb tremor and erectile dys- karinalebeis1@gmail.com bilateral hearing loss at the age 8 years, had normal neu-
function for the last two years. Symptoms progressively rodevelopment and no other systemic diseases. He is an
worsened during the period and patient became more Case Presentation: A 57-year-old female who four to only child, has no family history of neurological disease
irritable, impulsive and developed impaired memory. five years before, started with a progressive ataxia in four and denies consanguinity between his parents and other
Neurological examination revealed ataxic gait, dysmetria, limbs and gait. She also presents progressive dysarthria family members. Physical examination showed ataxic
dysdiadochokinesia, bilateral postural and action tremor, and dysphagia, and severe weight loss. At examination, gait, monocular diplopia, dysmetria, dysdiadochokinesia
and global areflexia with distal lower-limb hypopalles- she presented exacerbated deep tendon reflexes with and hyperreflexia with an exhaustible clonus in the lower
thesia. MOCA test was 19/30 with 11 years of schooling. Babinski sign, vibratory sensory loss in distal inferior limbs, limbs. He performed an extensive complementary inves-
Brain MRI showed cerebral and cerebellar atrophy, and head tremor, cognitive impairment in executive functions tigation, audiometric exam with bilateral sensorineural
symmetrical hyperintensities in the middle cerebellar and verbal fluency. She had no vestibular symptoms or hearing loss, brain MRI with cerebellar atrophy, EEG and
peduncles, periventricular regions and corpus callosum, parkinsonism findings upon examination. No family his- blood laboratory tests were unremarkable. Afterwards,
which was also atrophic (Figure). Electrophysiological tory of neurological diseases was reported. Laboratory an exome sequence was performed, and mutation of the
study revealed a sensory-motor polyneuropathy. Genetic investigation of neoplastic, infectious, immune-medi- HSD17B4 gene was identified, a missense compound
testing for the fragile X mental retardation 1 (FMR1) gene ated, endocrine and nutritional deficiencies showed no heterozygosity. Thus, the diagnosis of Perrault Syndrome
showed 111 CGG trinucleotide repeats, confirming the abnormalities. Brain magnetic resonance imaging (MRI) was made. Discussion: Perrault syndrome is a rare dis-
diagnosis of fragile X-associated tremor-ataxia syndrome showed extensive microangiopathy findings. Genetic test- ease, with autosomal recessive inheritance. It manifests
(FXTAS). Discussion: FXTAS is caused by the expansion ing presented 100 repetitions in both alleles of Frataxin with bilateral sensorineural hearing loss in both men and
of the CGG trinucleotide in the FMR1 gene (CGG between (FXN) gene for Friedreich ataxia. Friedreich ataxia is an women and varies in severity from moderate to severe,
55-200 repeats. Clinical picture comprises the presence autosomal recessive disease, the main cause of hereditary with onset in early childhood. Ovarian dysfunction in
of one or more characteristics, in different combinations: ataxia. Patients usually present symptoms before 25 years women with a 46, XX karyotype is expected, ranging from
cerebellar ataxia, parkinsonism, appendicular tremor, old. Our patient started symptoms at 52 years old, an atyp- primary ovarian failure to ovarian dysgenesis. Fertility in
neuropathy, dysautonomia, behavioral and cognitive ical presentation. Patients usually will have a positive fam- affected men is generally reported as normal, although
symptoms. Diagnosis is made by major and / or minor ily history for the disease. In most cases, patients present the number of men diagnosed is limited. Neurological
clinical and radiological signs : The most important find- aguanine-adenine-adenine (GAA) trinucleotiderepeat in manifestations are not common but may include learning
ing is the middle cerebellar peduncles hyperintensities intron 1 of both alleles of the FXN gene. Positive cases vary disabilities and developmental delay, cerebellar ataxia and
seen on T2-weighted brain MRI sequences. Minor diag- from 66-1700 repeats. Normal alleles will have between peripheral sensory and motor neuropathy. The diagnosis is
nostic criteria include other radiological findings such 7-34 repeats. Our patient’s number of repeats is located confirmed molecularly by the presence of biallelic patho-
as white matter hyperintensities and diffuse cerebral on the low end of positive cases, probably the cause for genic variants in one of six genes: CLPP, ERAL1, HARS2,
atrophy. Corpus callosum atrophy with hyperintensity her atypical presentation. Ataxia in four limbs and gait HSD17B4, LARS2 and TWNK. Markedly, our patient
of the splenium, dentate nucleus and pons may also is a cardinal sign, present in most patients. Deep tendon manifested this disease with a wide range of neurologic
occur. Definitive diagnosis requires the presence of the reflexes are usually lost. Exacerbated reflexes are atypical. symptoms, besides the expected hearing loss. Therefore,
premutation associated with the presence of one major Motor weakness involving inferior limbs is a common this was a challenging diagnosis, since he had no sister,
radiological sign plus one major clinical symptom or the symptom. Also, sensory loss in the distal limbs affects making in difficult to conclude without genetic testing.
presence of FXTAS inclusions in neuropathology. Final could be present, predominately involving proprioception Final Comments: Perrault syndrome is a rare disease and
Comments: It is to be emphasized the importance of and vibration sense. Dysarthria and dysphagia are com- there are no formal criteria for its diagnosis. The degree
FXTAS investigation in patients with late onset ataxia mon features. The main cause of death in these patientsis of diagnostic difficulty increases when related to male
especially when associated to parkinsonism, tremor and cardiac dysfunction, due to hypertrophic cardiomyopathy patients who do not have an affected sister. Genetic tests
peripheral polyneuropathy. However, there is, at present, frequently present in the disease, absent in our patient, play an important role in the early diagnosis of the disease.
no specific targeted treatment for FXTAS and management probably related to the late onset of the disease. Even
remains only symptomatic. though, case presentation of late onset Friedreich ataxia
are frequently published, we present a report with mainly Ataxias
atypical manifestations and in which the patient has no
Ataxias family history of the disease. Therefore, it is a diagnosis
sorely relying in genetic testing. We should consider in
clinical practice hereditary diseases, even when symp-
toms are atypical, and patients have no family history.

Ataxias

10
XXX Congresso Brasileiro de Neurologia

TL 1105666 TL 1105732 TL 1105738

HYPEREMESIS GRAVIDARUM AND KINETIC TREMOR IN PATIENT WITH TYPE LONG-TERM CEREBELLAR ATAXIA
WERNICKE’S ENCEPHALOPATHY: 2 SPINOCEREBELLAR ATAXIA (SCA2) AND ASSOCIATED WITH CONFOUNDING FACTORS:
A CASE PRESENTATION FAMILY HISTORY OF ESSENTIAL TREMOR: A COMPLEX CASE FOR ASSESSMENT AND
ATYPICAL PRESENTATION OR OVERLAP OF MANAGEMENT
Paulo Henrique Martinelli Oliveira, Lucas Silva Dias,
Raderi Luiz Cardoso dos Santos, Indianara Keila MOVEMENT DISORDERS?
Sephora Sabrina Candido Almeida, Erick Dupont,
Pastório, Lorena Dias Araújo, Francine de Paula Maria Luiza de Sousa Andrade, Denise Maria Paulo Filho Soares Marcelino, Amanda Loureiro
Roberto Domingos, Sayuri Aparecida Hirayama, Meneses Cury Portela, Gustavo Sousa Noleto, Toledo Troian, Sonia Maria Cesar de Azevedo Silva,
Rafael de Almeida, Raquel Mattos Filgueiras Natália Rebeca Alves de Araújo, Lia Leal Laurini, Herval Ribeiro Soares Neto, Alexia Carneiro Almeida,
Luana Larisse de Sousa Araújo, Anna Bheatriz Andre Lopez Fernadez
Hospital Municipal Dr. José de Carvalho Florence.
São José dos Campos SP, Brazil Feitosa Couto, Antonio Levi Farias Borba, Maria
Hospital do Servidor Publico Estadual de São Paulo.
Alice Silva Vasconcelos, Silmara Ferreira de Oliveira
São Paulo SP, Brazil
paulohmoliveira96@gmail.com
Centro Universitário Uninovafapi. Teresina PI, Brazil
sephora_sabrina@yahoo.com.br
Case Presentation: Two cases of pregnant patients are Hospital Universitário da Universidade Federal do
described, who presented with hyperemesis gravidarum Piauí. Teresina PI, Brazil
Case Presentation: Patient 77 years old, 5 months of
(HG), evolving with Wernicke’s Encephalopathy (WE). In Universidade Estadual do Piauí. Teresina PI, Brazil
evolution of rotational vertigo, tremors of upper limbs,
the 1st case, a 33-year-old patient, with gestational dia- Centro Universitário UniFacid. Teresina PI, Brazil
loss of balance with a tendency to fall and tonic-clonic
betes, started presenting HG during the 2nd trimester malu.sousa.andrade@hotmail.com seizures. Performing treatment for gastritis (proton pump
of pregnancy. Denied alcoholism and drug use. During inhibitor – PPI) for a long time and heavy drinker. The
the 15th gestational week, she underwent a laparoscopic Case Presentation: A 59 years-old male patient pre- patient had multidirectional nystagmus with limited
cholecystectomy procedure due to acute cholecystitis, sented at our outpatient clinic with a medical history of gaze fixation, bilateral upper limb myoclonus. Global
without complications. She had a miscarriage at 20 weeks imbalance and dysarthria initiated at the age of 39 and muscular hypotrophy with preserved strength, reflexes
and 6 days of pregnancy. After the abortion, she evolved progressed slowly. By the age of 57, the condition has and sensitivity. She associated movement decomposi-
with temporo-spatial disorientation, bradypsychism and worsened dramatically, with recurrent falls and wheel- tion to the coordination exam, trunk instability, did not
bradykinesia, and with bilateral decomposition of eye chair dependence. At the same period, he presented with remain standing without bilateral support and the gait was
movement. In the 2nd case, a 24-year-old patient with cephalic and upper limbs tremor, hypersomnia, depressive ataxic. She was investigated for the etiology of cerebellar
hyperthyroidism developed HG during the 3rd trimester symptoms, and anxiety. A cranial CT scan showed cere- ataxia and tests were performed to exclude autoimmune,
of pregnancy, with gait ataxia, nystagmus with horizontal bral atrophy and genetic test for ataxia panel confirmed vascular, metabolic and paraneoplastic causes, with no
and vertical downward gazes, and loss of recent memory, the diagnosis of type 2 Spinocerebellar Ataxy (SCA2) changes tests performed. During the investigation, a
with improvement in symptoms after 4 days of Thiamine with 22 and 36 CAG repetitions. Genealogy noticed many hypothesis of autoimmune cerebellar ataxia was raised
replacement. Discussion: Nausea and vomiting are typ- cases of SCA2 (father, paternal grandmother, maternal as a result of high anti GAD (406Ui/ml RV < 10) with IV
ical symptoms of the gestational period, affecting up to grand-grandfather, two sisters, nephew, and daughter). immunoglobulin, but with no change in the clinical pic-
70% of patients. A small portion of this population – about His mother had cephalic essential tremor. On physical ture. In addition, she maintained a persistent condition
0.3-10.8% – has a worsening of this condition, called HG. examination, he showed severe gait ataxia, impaired of hypomagnesemia (serum mg 0.3), initially attributed to
In this context, due to the restrictive condition, some walk, postural tremor on upper limbs and head, dysdi- the refeeding syndrome, was later noticed an association
patients may develop episodes of hypovitaminosis, such adochokinesia and ocular hypsometrical movements. between normalization of serum levels and consistent
as Thiamine Deficiency (vitamin B1), which is associated He was taking Levodopa, Amantadine e Paroxetine with clinical improvement. Renal losses and intestinal disorders
with WE. WE is an acute neurological syndrome, with a no good response. Discussion: Type 2 Spinocerebellar were ruled out to justify hypomagnesemia, and therefore,
reversible characteristic, which leads to changes in men- ataxia (SCA2) is a rare neurodegenerative disease, dom- given the prolonged use of PPIs. Discussion: Cerebellar
tal status, ocular abnormalities and cerebellar dysfunc- inant autosomal, with a prevalence of 1-2 cases in 100000. ataxia can be associated with numerous causes includ-
tion. The association of HG with WE is rare, however, it is The clinical findings are ataxic gait, dysarthria, dysmetria, ing metabolic, paraneoplastic, vascular and autoimmune
capable of causing serious complications if not diagnosed dysdiadochokinesia and ocular hypsometrical move- disorders. There are descriptions in the literature not only
early, with fetal death in up to 50% of cases and maternal ments. About 10% of cases of SCA2 present kinetic tremor. of magnesium-related ataxia but also other neurological
death in 10-20%. Final Comments: Among the differen- Essential tremor is the most common cause of kinetic manifestations such as seizures and involuntary move-
tial diagnoses regarding neurological alterations during tremor, with a global prevalence of 1%, occuring specially ments. Hypomagnesemia caused by chronic PPI use is
pregnancy, the hypothesis of WE should be considered. on males. It presents a dominant autosomal heritage with also described and should be investigated when other
This study is relevant because it exposes cases of the rare reduced penetration. Additonally ET (essential tremor) causes for the disorder have been excluded. Anti GAD
correlation between HG and WE, whose delay in diagnosis possesses a genetical and phenotypical heterogeneity may be positive in approximately 0.4 to 1.7% of the gen-
can lead to morbid complications, and whose treatment is and represents a series of syndromic symptoms, instead eral asymptomatic population, highlighting the need to
often simple, capable of reversing neurological symptoms of just one disease. Final Comments: In the face of these properly interpret the results obtained. Final Comments:
in their entirety and minimizing damage to pregnancy. statements, we can understand this particular case as a Proton pump inhibitors are widely used but they can lead
rare disease, SCA2, associated with the cephalic tremor, to absorptive changes including for magnesium and, as a
oppening the possibility of a diagnosis which considers consequence, lead to significant deficit and be one of the
Ataxias metabolic causes of neurological symptoms, including
the overlapping, of pathologies, ataxia and essential tremor
or an atypical presentation of SCA2. cerebellar ataxia. Patients who present compatible con-
ditions should be investigated for the different etiologies
that may trigger the symptoms, taking into account the
Ataxias patient’s clinical improvement to correctly determine
the diagnosis.

Ataxias

11
XXX Congresso Brasileiro de Neurologia

TL 1105790 TL 1105804 TL 1105808

PENGUIM GAIT, LYNX EARS AND BULLDOG SEVERE TOXIC AND IMMUNE SENSORY- A RARE CAUSE OF SENSORY-MOTOR
SCALP, A DIAGNOSTIC DILEMMA CEREBELLAR ATAXIA IN AN ONCOLOGIC AXONAL NEUROPATHY WITH HEARING
PATIENT IMPAIRMENT AND CEREBELAR ATAXIA
Beatriz Cassarotti, Thábata Emanuelle Martins
Nunes, Patrícia Áurea Andreucci Martins Bonilha, Erick Dupont, Arthur da Veiga Kalil Coelho, Davi Victor Augusto Zanesi Maciel, Anna Letícia de
Léo Coutinho, Luiz Eduardo Novis, Walter Oleschko Vargas Freitas Teixeira, Marcella Canato Toloi, Moraes Alves, Alison Mangolin, Vanessa Lauanna
Arruda, Salmo Raskin, Hélio Afonso Ghizoni Teive Andreas Batista Schelp, Déborah Inayara Mendes Lima Silva, Lucas Gondim Briand Vieira, Rodrigo
Tenório de Albuquerque, Sephora Sabrina Cândido Frezatti, Pedro José Tomaselli, Mary Reilly, Wilson
Hospital de Clínicas da Universidade Federal do
de Almeida, Ana Flávia Pincerno Pouza Marques
Paraná. Curitiba PR, Brazil
Genetika, Centro de Aconselhamento e Laboratório Hospital do Servidor Público Estadual de São Paulo. Hospital das Clínicas da Faculdade de Medicina de
de Genética. Curitiba PR, Brazil São Paulo SP, Brazil Ribeirão Preto. Ribeirão Preto SP, Brazil
Department of Neuromuscular Diseases, UCL Queen
beatriz_cassarotti@yahoo.com.br dupont.erick@gmail.com Square Institute of Neurology – Grã-Bretanha Reino
Unido
Case Presentation: A 29-year-old male reported a Case Presentation: A 73-year-old female patient presents
6-year-history of gait disturbance and cognitive impair- with progressive worsening of gait in the last 6 months, victor93@gmail.com
ment. His parents were first cousins, and a brother started rangin from walking without devices to using a wheelchair
with the same symptoms at 18-year-old. Clinical examina- in 3 months. Besides chronic type 2 diabetes, she also had Case Presentation: A 32-year-old adopted man with a
tion demonstrated dysarthria and spastic paraplegia, with a history of breast and ovarian cancer diagnosed in the history of frequent falls since childhood presented to our
a grade IV muscular strength, spasticity, and hyperreflexia last 5 years, both with surgical and chemotherapy treat- neurogenetic outclinic. He had normal motor milestones
in the lower limbs; his patellar and Achilles reflexes were ment. On admission, the patient had preserved strength, achievement but presented with progressive unsteady gait
brisk, and he presented a spastic gait. In addition, head pseudoathetotic movements in the fingers of the hands, and tremors from the age of five. He has also complained
and skull examination demonstrated a curious feature: severe sensory impairment composed by hypopalesthe- of sensory disturbance, especially at distal legs and feet,
The presence of cutis verticis gyrata. Brain Magnetic sia in all four limbs, worse in the legs, and proprioceptive dysarthria and hearing loss since 8 years old. He denied
Resonance Imaging (MRI) revealed bilateral hyperinten- dysfunction of the halluxes. She had scanning speech any cognitive impairment, seizures, cardiologic or pul-
sities at the periventricular white matter.Whole-exome and bidirectional horizontal nystagmus accompanied monary involvement. In the examination he had multi-
sequencing revealed a mutation in the SPG11 gene (SPG by hypometric saccades with corrective movements. directional nystagmus and mild dysarthria. His strength
11: NM_001160227.2: exon35: c.6493_6494del: pS2165Lfs). Axial and appendicular ataxia shown as the inability to and DTR were normal throughout. Pinprick and vibration
Discussion: Hereditary spastic paraplegia (HSP) is a het- remain seated without aid and dysmetria in all four limbs, sense were impaired distally. He had bilateral severe dys-
erogeneous group of neurodegenerative disorders char- markedly worse when eyes are closed. In orthostasis, she metria and dysdiadochokinesia, and an ataxic gait with a
acterized by progressive lower limb weakness and spas- showed abasia accompanied by extremely cautious gait broad base, besides a positive Romberg test, suggesting
ticity, caused by mutations in the spastic paraplegia gene and Romberg’s sign. Cranial magnetic resonance imaging a mixed sensory-cerebellar ataxia. Nerve conduction
(SPG). SPG11 gene (located on chromosome 15q13-15), revealed marked cerebellar atrophy and electroneuromy- studies revealed a non length dependent sensory-motor
has 100.982 coding nucleotides distributed in 40 exons ography showed severe sensory and motor neuropathy nerve axonal impairment. Needle examination revealed
and translates into spatacsin. Descriptions include more in the 4 limbs. Cerebrospinal fluid (CSF) with 1 leukocyte diffuse chronic denervation affecting cranial, cervical,
than 160 pathogenic variants, mostly missense muta- and 65mg/dL proteins. Vitamin dosages and inflamma- thoracic and lumbo-sacral segments. Brain MRI showed
tions. SPG 11 can present as a pure HSP with autosomal tory markers without alterations. The autoimmunity test only diffuse mild cortical atrophy. Severe neurosensorial
recessive inheritance, but patients frequently develop a was positive for anti-Yo in CSF and blood samples, with hearing loss in the right ear and mild loss in the left ear
complicated HSP phenotype with cognitive impairment, titers of 1: 256 and 1: 15360, respectively. After receiving were evinced in audiometry. His muscle biopsy was unre-
peripheral neuropathy, cerebellar, lower motor neuron, 1 g of Methylprednisolone per day for 5 days, the patient markable. Initial molecular analysis ruled out Friedreich
and extrapyramidal features. A recognized hallmark of had a slight improvement in ataxia and was discharged ataxia, SCA2, SCA3, CMT1A, CMT1B and CMTX1. WES
SPG11 is the presence of a thin corpus callosum (TCC) for outpatient follow-up. Neurotoxicity is a common side revealed a hemizygous missense pathogenic variant
in the brain MRI. HSP-TCC also occurs in peripheral effect in patients undergoing chemotherapy, with periph- (c.512T>C;p.Met171Thr) in the AIFM1 gene. The patho-
neuropathy with agenesis of the corpus callosum and eral nerves being more affected than the central nervous genicity of the variant is supported by this location in a
other HSPs. Final Comments: Clinical findings such as system. Toxicity is usually worse for sensory fibers, gener- hotspot, deleterious computation prediction (13 out of 16),
the age of onset, gait disturbance, and presentation of ating paresthesias or proprioceptive deficits when thick high conservation score (phyloP100: 8.692) and absence
motor and cognitive impairment were usual for SPG11, fibers are affected. In this same context, neuronal death on Genome Aggregation Database. Discussion: AIFM1
but this was not enough to settle the diagnosis. This case can also be induced by paraneoplastic syndromes, which gene is a nuclear gene related to the transcription of a
presented no additional clues to help in the diagnosis, are mediated by antibodies produced by immune activa- mitochondrial homodimeric protein that is required to
such as a thin corpus callosum or “ears of the Lynx’’. The tion in response to cancer. Among them, Paraneoplastic maintain the mitochondrial respiratory complex I. AIFM1
dermatologic sign was a surprise and can open our eyes to Cerebellar Degeneration mediated by anti-Yo (also known related disorders include X-linked deafness, combined
possible future genotype-phenotype correlations toward as anti-Purkinje cell cytoplasmic antibody type-1) stands oxidative phosphorylation deficiency 6, spondyloepime-
SPG11. Although there are no reported cases correlating out, an antibody specifically directed to the cerebellum taphyseal dysplasia with hypomyelinating leukodystrophy
cutis verticis gyrata and SPG11, we suggest that clinicians responsible for generating an ataxic syndrome. Ataxia and and Cowchock syndrome, CMTX4. Our case highlights
be aware of this possible association. It was an unusual sensory changes are common findings in cancer patients the clinical heterogeneity of this rare variant, pointing
case, where clinical findings alone led to doubt. Genetic and may have a multifactorial origin. The final outcome out that in a patient with sensory-motor axonal neurop-
testing was necessary, leading to a surprising outcome. can be influenced by previous pathologies, chemother- athy with hearing impairment and ataxia, features mostly
apeutic-induced toxicity and even autoimmunity in a seen in mitochondrial disorders, AIFM1 gene must be
context of paraneoplastic syndrome. considered. Final Comments: AIFM1 gene variants are
Ataxias extremely rare. Here we describe a case of progressive
ataxia, hearing impairment and sensory-motor neurop-
Ataxias athy in a male patient without known family history, in
that a hemizygous AIFM1 variant has been found.

Ataxias

12
XXX Congresso Brasileiro de Neurologia

TL 1106188 TL 1106199 TL 1106279

ACUTE CEREBELLAR ATAXIA ASSOCIATED A PATIENT WITH CEREBELLAR ATAXIA AND A PNKP MUTATION IN BROTHERS: ONE
WITH DENGUE FEVER: CASE PRESENTATION ITPR1 GENE MUTATION SYNDROME, TWO PHENOTYPES
AND LITERATURE REVIEW
Thábata Emanuelle Martins Nunes, Léo Coutinho, Patricia Aurea Andreucci Martins Bonilha, João
Arthur Cesário de Holanda, Daniel Alves de Oliveira, Patricia Aurea Andreucci Martins Bonilha, Beatriz Vitor M Lisboa, Thabata Emanuelle Martins Nunes,
Vitor Maia Arca, Eduardo Maranhão, Geovane Cassarotti, Emanuel Cassou Santos Santos, Hélio Beatriz Casarotti, Luiz Eduardo Novis, Leo Coutinho,
Gomes Silva, Ana Rosa Santana, Raphaelly Ribeiro Afonso Ghizoni Teive Francisco Manoel Branco Germiniani, Helio Afonso
Campos, Luísa Van der Linden Ferreira da Silva, Ghizoni Teive
Hospital das Clínicas da Universidade Federal do
Marcos Eugênio Ramalho Bezerra, Eduardo Sousa
Paraná. Curitiba PR, Brazil Universidade Federal do Paraná. Curitiba PR, Brazil
de Melo
Universidade Federal do Paraná. Curitiba PR, Brazil
patriciambonilha@hotmail.com
Hospital das Clínicas da Universidade Federal de
thabatamnunes@gmail.com
Pernambuco. Recife PE, Brazil
Case Presentation: Two brothers attend at our clinical
arthur.c.holanda@gmail.com Case Presentation: A previously healthy 40-year-old practice with dysarthria, gait ataxia, polyneuropathy and
female presented a 4-year history of gait imbalance and oculomotor apraxia. The older one, now with 40 years old,
Case Presentation: A 70-year-old female was admit- dysarthria. In the past 2 years, she developed head and began at the age of 1 year with gait ataxia, seizures and
ted due to vertigo and imbalance associated high fever, upper limb tremor. Her parents were both alive at the neuropsychomotor developmental delay, at 18 years old
headache, photophobia, myalgia, nausea, and emesis for age of 66 (father) and 60 (mother), both reported by the was restricted to wheelchair and, actually has severe dys-
5 days. She denied other symptoms, as well as exposure patient as asymptomatic. She was of Portuguese ancestry arthria, fragmented saccades, ocular apraxia and severe
to predisposing agents. At physical examination, there on the maternal side and Italian and German ancestry on muscular atrophy and anesthesia and loss of vibratory
was a global asymmetric cerebellar syndrome, without her paternal side. She had a 16-year-old daughter, also sensation in legs and arms, totalizing 40 at SARA score. The
other abnormalities. Laboratory work-up showed lym- reported asymptomatic. On the neurological examination, younger one is 21 years old and had history of falls during
phocytosis, leucopenia, thrombocytopenia, and increased she presented tandem gait ataxia, dysdiadochokinesia, childhood, but only with 16 years old the gait ataxia was
liver enzymes. Serologic exams were negative for syphilis, dysmetria, nystagmus, hypermetric saccades, and mild evident and, currently, walks with strong support due to
human immunodeficiency virus, hepatitis B and C viruses, lower limb spasticity. Her ocular movements, cognition, intense postural instability, associated with ocular apraxia,
cytomegalovirus (CMV), rubella, toxoplasmosis, and try- and peripheral system were unaffected. Unfortunately, mild dysarthria, mild symmetric atrophy of hands muscles
panosomiasis in peripheral blood, and for CMV, herpes her family members could not be examined, as they cur- and also with compromise of tactile and vibratory sen-
simplex and varicella-zoster viruses, and toxoplasmosis rently live in another city. Ancillary workup for acquired sory in legs and arms. Both had cerebellar atrophy seen
in cerebrospinal fluid. Brain MRI with angiography was ataxias, including vitamin E, alpha-fetoprotein, serology in neuroimaging, nerve conduction study with severe
unremarkable (Figure 1). During Hospital stay, there was testing, and B12 vitamin were all normal. Brain magnetic sensorimotor axonal and demyelinating polyneuropathy,
complete recovery of the systemic symptoms and gradual resonance imaging (MRI) showed only mild cerebellar elevated alfa-fetoprotein and dyslipidemia. The patients
improvement of the cerebellar syndrome. She was dis- atrophy. Cervical MRI and nerve conduction studies were underwent exome evaluation and missense mutation
charged for outpatient follow-up, during which a positive unremarkable. The patient was tested for spinocerebellar of PNKP gene in homozygosis was identified and both
IgM (MAC-ELISA) for Dengue virus was identified. Fifty- ataxias (SCA) type 3 and type 10, both negative. Whole- were diagnosed with Ataxia-oculomotor apraxia type 4.
four days after symptom onset, she had a normal neuro- exome sequencing revealed a heterozygous Arg275Trp Discussion: First described in 2015, ataxia-oculomotor
logical exam, remaining with a mild subjective imbalance variant in the PRKN gene and a heterozygous Asp1882Asn apraxia due to PNKP mutation was identified in 11 patients
complaint. Discussion: Despite uncommon, neurological variant in the ITPR1 gene. With the identification of the of 8 unrelated Portuguese families. Classified as hereditary
involvement in Dengue fever is well recognized, both due ITPR1 mutation, the diagnosis of SCA 15 was established. autosomal-recessive cerebellar ataxia, this is a rare neuro-
to direct viral lesion and to immunomediated mechanism. Discussion: The ITPR1 gene is in the 3p26 chromosome, degenerative disorder and, at the same time, is the second
Encephalitis and encephalopathy are the most frequent and encodes the inositol 1,4,5-triphosphate receptor type most frequent cause for recessive ataxia in Portugal. In
manifestations; however, meningitis, stroke, myelitis, 1. Mutation of the gene is transmitted in an autosomal literature review of ataxia-oculomotor apraxia 4 cases, we
Guillain-Barré syndrome, and neuritis have also been dominant manner, and this phenotype was correlated can find that almost all patients have cerebellar atrophy in
reported. Association between cerebellar syndrome and to several neurological syndromes, such as SCA 15, SCA brain MRI. The PNKP gene has a important role in DNA-
Dengue, however, is extremely rare, with only seven other 29, Under-Tan syndrome, and Gillespie syndrome. The oxidative damage repair and its mutation is associated
cases published (Table 1). Acute cerebellar ataxia, part of mild symptomatology showed by the patient suggests the with Ataxia-oculomotor apraxia type 4, Charcot-Marie-
a spectrum of cerebellar dysfunction related to inflamma- diagnosis of SCA 15. Final Comments: Slowly progressive Tooth disease type 2B2 and Microcephaly, seizures, and
tory conditions, is characterized by normal neuroimaging cases of cerebellar ataxia can often give the impression of developmental delay. Final Comments: PNPK gene-re-
and complete recovery in two months to three years. As a sporadic or acquired etiology due to an apparent lack of lated ataxia-oculomotor apraxia has a wide spectrum of
etiologies are numerous, a throughout investigation must family history. After reasonable exclusion of such causes, the age of onset of symptoms, and its diagnosis can only
be performed before establishing this causal relationship. the possibility of a genetic etiology should be raised and be made with a genetic test, which implies a barrier due
In our patient, it was possible to confirm recent infection properly evaluated. to the cost of the test. Although rare, it should be among
by Dengue and to exclude other agents associated with the possible etiologies in ataxia-oculomotor syndromes.
ataxia. Final Comments: The present report corrobo-
rates with the recognition of Dengue virus as a cause of Ataxias
acute cerebellar ataxia. Considering the infection’s inci- Ataxias
dence worldwide, it is probable that this association is
underdiagnosed, especially considering the number of
cases of acute ataxia without an identified etiology. Active
investigation of Dengue infection should be a part of the
work-up in such cases.

Ataxias

13
XXX Congresso Brasileiro de Neurologia

TL 1106317 TL 1106382 TL 1106533

CEREBELLAR ATAXIA IN PRIMARY ACUTE POSTINFECTIOUS CEREBELLAR SPINOCEREBELLAR ATAXIA 17 OF


SJÖGREN’S SYNDROME WITHOUT ATAXIA: CASE PRESENTATION ACCELERATED EVOLUTION
CEREBELLAR ATROPHY
João Gabriel Pacetti Capobianco, Luis Felipe Davi Bravo Huguinim Légora, Emerson Gisoldi,
Maria Júnia Lira e Silva, Rodrigo André de Souza Magalhães Serne Leite, Helena Moretti Bressane Yngrid Dieguez Ferreira, Matheus Gonçalves Maia,
Araújo, Matheus de Melo Aziz Cardoso, Alex de Diogo Haddad Santos, Sophia Bravo Huguinim
Pontifícia Universidade Católica de Minas Gerais. Légora
Novais Batista, Marcílio José de Oliveira Filho,
Poços de Caldas MG, Brazil
Clauhan Willams Soares dos Santos, Jonata Ribeiro
Irmandade da Santa Casa de Misericórdia de São
de Sousa, Fabíola Lys de Medeiros, João Eudes joao_20_03@hotmail.com Paulo, São Paulo SP, Brazil
Magalhães
Multivix. Cachoeiro de Itapemirim ES, Brazil
Case Presentation: A previously healthy 16-year-old
Universidade de Pernambuco. Recife PE, Brazil
boy presented onset of his clinical condition pain in his davibravohl2@gmail.com
lirajunia@gmail.com right leg and diarrhea, subsequent odynophagia, fever,
and headache. Diagnosed with a viral infection, pre- Case Presentation: Patient, male, 35 years old, previously
Case Presentation: An 86-years-old woman presented scribed azithromycin, which he used without symptom healthy, parents healthy and not consanguineous, started
with imbalance, hand tremors, and frequent falls for 3 improvement. After a three days his symptoms evolved at age 32 with a complaint of imbalance, characterized by
years. She reported systemic hypertension, coronary into mental confusion, dysphonia, photophobia, fever, an insidious and progressive cerebellar ataxic syndrome,
artery disease, previous hepatitis B and C, and a history of emesis, asthenia. While in Hospital, he evolved with nys- and in less than 6 months of progression, he already
alcoholism interrupted for more than 10 years. The neu- tagmus, ataxia, slow speech and vertigo. Brain MRI was depended on walking support. Associated with this, he
rological evaluation showed hypometric saccades in the performed, showing hypersignal T2/FLAIR on cerebellar presented with amnestic and dysexecutive mild cognitive
pursuit of the gaze, altered vestibulo-ocular reflex, axial topography suggestive of viral cerebellitis. Lumbar punc- impairment, in addition to depressive-type mood disor-
and appendicular signs of cerebellar ataxia more evident ture with CSF analysis was performed, without changes. der. In the same period, he started with epileptic seizures
on the left side. Sensory examination was normal. She had The patient was initially treated with acyclovir, with sig- characterized by behavioral arrest and sphincter release,
signs of sicca syndrome, with tongue fissures and thick nificant improvement of symptoms and ataxic syndrome. without motor tremors. Due to the accelerated progression
saliva, as well as ocular biomicroscopy with altered But Later on, pulse therapy with methylprednisolone 1g/ of neurological symptoms, despite the innocent cerebro-
test. Brain MRI showed no cerebellar atrophy, and there day for 5 days was performed, also with improvement spinal fluid, intravenous immunoglobulin was performed
was elevated erythrocyte sedimentation rate and positive of symptoms, leading to him being discharged from the for 5 days, thinking about autoimmune encephalitis, but
anti-RO. Dosages of vitamin E, thiamine, anti-GAD, pro- Hospital. Patient returned for follow-up of postinfectious without improvement. Advanced brain neuroimaging
tein electrophoresis, cryoglobulins, and CSF analysis were cerebellitis after three months. Neurological examination showed pancerebellar atrophy without contrast enhance-
normal. Nerve conduction studies were unavailable. In evidenced that the patient was oriented, without speech ment. Genetic testing confirmed it to be Spinocerebellar
order to exclude occult neoplasm, mammography, thyroid alterations, grade 5 strength in both MS and MI bilaterally, Ataxia type 17 (SCA 17). Discussion: SCA 17 is a rare,
and endovaginal ultrasound, and CT scans of the chest, gait with no alterations, with unaltered cerebellar tests autosomal dominant genetic disease identified by the
abdomen and pelvis were unremarkable. Antineuronal and complete improvement of vertiginous symptoms. abnormal expansion of the CAG/CAA repeat in the TATA-
antibodies were not available. Discussion: This patient Discussion: The acute cerebellitis is a rare condition with box-binding protein (TBP) gene, with affected individuals
met the criteria for Primary Sjögren’s syndrome (PSS) an inflammatory disorder that can cause a different range having more than 41 repeats. The clinical manifestation
according to the EULAR-2016. PSS is an autoimmune of symptoms. The most common symptoms are ataxia, begins between 3 and 75 years (mean: 34.6 years), being
inflammatory disease characterized by lymphocytic infil- vomiting and headache. The most affected population is defined by cerebellar ataxia (95%), cognitive symptoms
tration of the exocrine glands. Up to 70% of patients may the pediatric one. It is important to know that acute post- (90%), psychiatric symptoms, epilepsy, and involuntary
have neurological manifestations, predominating the infectious cerebellar ataxia is a subgroup of acute cerebel- movements (chorea, dystonia). By 2005, fewer than 100
involvement of the peripheral nervous system. Cerebellar litis and they both have symptoms of cerebellar ataxia. By families had been reported to have the disease worldwide.
ataxia is rarely described, especially in the absence of definition an acute cerebellitis is a dysfunction attribute Treatment is currently limited to controlling symptoms,
cerebellar atrophy. In a series of cases that included 14 to a recent infectious illness, vaccination or ingestion of improving quality of life, but there is still no cure. Final
patients and sought to characterize ataxia in patients medication and with a magnetic resonance imaging (MRI) Comments: Genetic tests are increasingly gaining space
with PSS, only one 71-year-old woman with dysmetria showing isolated cerebellar inflammation. There are three and usefulness in clinical practice, elucidating cases and
and associated sensory ataxia did not have cerebellar outcomes for cerebellar ataxia: full recovery, mild neuro- directing conducts, which, although often not curative,
atrophy. Other study reported a 56-year-old woman with logic sequelae not affecting play and school activities or are substantial for the individual and family. In this spe-
PSS, cerebellar ataxia and limbic encephalitis without mobility, or moderate/severe sequelae requiring special cific case of SCA 17, by configuring an autosomal domi-
MRI alterations. Immunohistochemistry for antineuronal treatment or education. The patient in question showed nant inheritance, it made it possible to structure a family
antibodies revealed cerebellar Purkinje cell staining. Final total improvement in his condition. Final Comments: genetic counseling that would guide the family, since the
Comments: This is the third case of PSS with cerebellar Considering that there is a lack of knowledge about the patient had siblings and children.
ataxia without cerebellar atrophy described in the liter- incidence of this neurological condition, it is necessary
ature. Antibodies against Purkinje cells may be involved to discuss and share data related cases of post-infectious
in the pathogenesis of the condition. It is worth men- cerebellitis, in order to possible future prospective studies Ataxias
tioning that due to the latency between the appearance about the incidence and risk factors.
of neurological symptoms and the detection of cancer, a
paraneoplastic syndrome in similar cases remains pos-
sible, although extensive screening has been carried out. Ataxias

Ataxias

14
XXX Congresso Brasileiro de Neurologia

TL 1105101 TL 1105197 TL 1105686

ATYPICAL MIGRAINE WITH TRANSIENT ENCEPHALITIS ASSOCIATED WITH COVID-19 CHALLENGES OF INTRACRANIAL
BINOCULAR BLINDNESS: CASE AND THUNDERCLAP HEADACHE: AN HYPERTENSION
PRESENTATION UNUSUAL ASSOCIATION
Paulo Henrique Martinelli Oliveira, Lucas Silva
Gloria Maria Schitini de Souza, Ingrid Pereira Emanuelle Bianchi da Silva Rocha, Camila Moraes Dias, Raderi Luiz Cardoso dos Santos, Indianara
Marques, Cristiana Pessoa de Queiroz Faria Goes, Eberhardt, Luciana de Azevedo Tubero, Alex Keila Pastório, Lorena Dias Araújo, Francine de
Carolina Rouanet Calvacante de Albuquerque, Bertolazzo Quiterio, Janaina Moraes de Araújo, Paula Roberto Domingos, Rafael de Almeida, Sayuri
Bruno Castelo Branco Rodrigues, Marcos Martins Laura Alonso Matheus Montouro, João Pedro Izidoro Aparecida Hirayama, Raquel Mattos Filgueiras
da Silva Gomes, Rodrigo Fellipe Rodrigues, Gabriel Pina
Hospital Municipal Dr. José de Carvalho Florence.
Paiva, Andressa Regina de Mello Galego
Universidade Federal do Rio de Janeiro. Rio de São José dos Campos SP, Brazil
Janeiro RJ, Brazil Faculdade de Medicina de Ribeirão Preto. Ribeirão
paulohmoliveira96@gmail.com
Preto SP, Brazil
gloria-Salvador@hotmail.com
m.bianchis@hotmail.com Case Presentation: A 13 years old female, in December
Case Presentation: A 58 year-old male with systemic arte- 2021, she started to have daily, moderate-intensity,
rial hypertension, rheumatoid arthritis and HIV started, Case Presentation: 51 years-old woman, previous history tightening holocranial headache, lasting for hours and
3 years ago, episodes of acute bilateral blindness lasting of depression and systemic arterial hypertension reporting improved after the use of simple analgesics. And it was a
about 20 minutes, 5 episodes a year, without other symp- sudden holocranial headache that has progressed to severe headache without warning signs. With persistence of the
toms. He also had a previous history of intense pulsating intensity within minutes at onset (thunderclap headache) headache, but with worsening intensity, the patient was
headache, which started 20 years ago, with nauseas, photo started seven days be¬fore, which progressed with mental admitted to the Hospital on 02/19/2022, complaining of
and phonophobia, about 3 times a week, independent confusion, inattention inability to meet family members binocular diplopia, with worsening at side views. She pre-
of the blindness episodes. His neurological examina- and difficulty of communication at the day of admission. sented new episodes of nausea and vomiting 7 days after
tion showed no alterations. Brain MRI, intracranial and General physical examination, including respiratory aus- admission. We found on the neurological examination:
cervical CT angiography, 24h holter and transthoracic cultation and oxygen saturation, was normal except for she had esotropia and limitation for abduction of the left
echocardiogram were within the normal limits. He was the presence of borderline pyrexia (37.7 °C). Neurological eye; Fundoscopy: bilateral papilledema. Cerebrospinal
started on treatment with valproic acid 250 mg bid, which examination showed only mental and cognitive alterations Fluid (CSF): Opening pressure (OP) 58 cmH2O, eryth-
was suspended due to trunk urticaria. Amitriptyline 50 described as inattention, somnolence, disorientation in rocytes 15/ cells 23 (Poly 10%; Mono 90%) / Glucose 48 /
mg daily was started, with a reduction in intensity and time and space, inability to say her own name or recognize Proteins 25 / VDRL non-reactive / Gram absent / China
frequency of both headache and blindness episodes. her husband and language disturbance, characterized by ink negative. Culture for bacteria and fungi: No growth.
The diagnosis was of migraine with binocular blindness relative fluent speech, incapacity to name objects, repeat PCR Herpes virus: negative. Capillary glucose: 110 mg/dL.
aura. Discussion: Visual aura is the most frequent type words or phrases and to obey commands. Computed Patient received Ceftriaxone 4g/day for 7 days, Topiramate
of migraine associated aura, which mechanism is cortical angio¬tomography (CTA) of the brain excluded bleeding 100 mg/day and Acetazolamide 500 mg/day for 12 days.
spreading depression in the occipital cortex. However, but showed one aneurysm in the supraclinoid internal Venous Angioresonance was performed: transversus
complete vision loss in both eyes associated with migraine carotid artery. Cerebrospinal fluid had no xanthochro- sinus hypoplasia. After 12 days of Hospitalization, patient
is extremely rare. Recent publications described binocular mia and evidenced a lymphomonocytic pleocytosis with was without diplopia with improvement of esotropia. A
blindness as migraine aura and its main hypothesis was discrete hyperproteinorraquia. Electroencephalogram new CSF collect was performed: OP: 38 cmH2O, cells: 0,
retinal spreading depression. Studies with chick retinas showed symmetrical and disorganized brain electrical, erythrocytes: 30, glucose 52. Capillary glucose: 115 mg/
showed that retinal spreading depression occurs similarly theta rhythm diffuse and presence of triphasic waves. Due dL. Discussion: Our patient had a syndromic diagnosis
to cortex. Furthermore, migraine with binocular blindness to Hospital protocol during the Covid-19 pandemic, the of Intracranial Hypertension (IH): she had a prolonged
does not appear to be a subform of basilar-type migraine patient underwent computed tomography of thorax and clinical picture with non-spontaneous clinical resolution
as the blindness occurs in isolation without other symp- we found ground-glass opacities suggesting viral infec- (that needed topiramate and acetazolamide to resolve),
toms required for ICHD-3 basilar type migraine diagno- tion. Then, RT-PCR for Sars-CoV-2 with nasopharyngeal in the context of an altered CSF. In fact, this was the
sis, and retinal migraine is characterized by monocular swab and in CSF was performed with both positives. main reason why our patient did not meet the criteria for
blindness. A recent case presentation described two Therefore, the diagnosis was encephalitis associated with Idiopathic IH. The patient presented criteria for aseptic
unrelated families with repetitive transient daily blind- Covid-19. We opted for supportive care. Patient evolved meningitis (AM), with pleocytosis greater than four cells
ness in five members that had clinical characteristics of with many complications, need of ventila¬tory support in CSF associated with a process free of contamination by
a retinal electrical signal abnormal propagation that may and renal replacement therapy with discharged of the fungi, bacteria or parasites. AM has as main etiologies:
be a retinal spreading depression. One study with many Hospital after 35 days, with no symptoms. At follow-up, systemic diseases with meningeal involvement, induced
migraine patients showed low incidence of binocular two months later, the only finding was mild inattention. by drugs, because of neoplasic and the most common,
blindness. They were all female, had some abnormality Discussion: Covid-19 is characterized mainly by respi- viral. Final Comments: Therefore, our patient presented
of clotting factors and no history of previous aura. Their ratory symptoms, however this virus frequently damages IH associated with CSF with characteristics of AM with a
episodes were infrequent and may even occur only once the nervous system, although the mechanism involved is prolonged clinical picture. Due to the various etiologies of
in life. The authors suggest classifying episodes of tran- still unclear. Headache is the most common neurological AM, diagnosis and treatment becomes a challenge, espe-
sient blindness as an aura-like event of acute onset. Our symptom and has a great hete¬rogeneity, including thun- cially when associated with IH in a young patient with a
patient had no other cause to explain his symptoms. Both derclap headache, which is a red flag and investigation severe impairment who arrives in the emergency room
episodes of bilateral blindness and headache improved is mandatory. Final Comments: Headache is common with CSF so altered. Please note the context of a public
with amitriptyline, contributing to the diagnosis. in patients with Covid-19 and can be the first signal of Hospital with difficulties in identifying viral causes. for AM.
this infection or of a complication of that, like enceph-
alitis. In addition, the spectrum of the pain is range and
Cefaleia includes thunderclap headache mimicking an SAH, as Cefaleia
we approach here.

Cefaleia

15
XXX Congresso Brasileiro de Neurologia

TL 1105693 TL 1105734 TL 1105777

RAEDER SYNDROME: CASE PRESENTATION TRIGEMINAL AUTONOMIC CEPHALALGIA HEADACHE ATTRIBUTED TO SLEEP APNEA:
ATTRIBUTED TO ISCHAEMIC STROKE: A CASE PRESENTATION
Matheus Gurgel Saraiva, Daniel Vicente de Siqueira
Lima Júnior, Maria Yvone Carlos Formiga de Qeuiroz, CASE PRESENTATION
Ana Rosa Santana, Arthur Cesário de Holanda,
Rafael Gonçalves Duarte Cunha, Rafael de Souza Vitor Maia Arca, Geovane Gomes Silva, Ana Rosa Raphaelly Ribeiro Campos, Geovane Gomes Silva,
Andrade, Jeanina Cabral Dionizio, Daiane Pereira Santana, Raphaelly Ribeiro Campos, Arthur Cesário Vitor Maia Arca, Daniel Alves de Oliveira, Luísa
de Farias, Tatheane Couto de Vasconcelos, Thiago de Holanda, Daniel Alves de Oliveira, Víctor de Couceiro de Albuquerque Macêdo, João Pedro
Medeiros Palmeira de Araújo, Juliana Magalhães Moraes Chagas, Marcos Eugenio Ramalho Bezerra, Matos de Santana, Marcos Eugênio Ramalho
Leite Pedro Augusto Sampaio Rocha-Filho Bezerra, Pedro Augusto Sampaio Rocha Filho
Hospital Metropolitano Dom José Maria Pires. João Hospital das Clínicas da Universidade Federal de Hospital das Clínicas da Universidade Federal de
Pessoa PB, Brazil Pernambuco. Recife PE, Brazil Pernambuco. Recife PE, Brazil
matheus_gurgel_pb@hotmail.com vitorm_arca@hotmail.com arthur.c.holanda@gmail.com

Case Presentation: A 33-year-old male patient, without Case Presentation: A 42-year-old man, with hyperten- Case Presentation: A 61-year-old female presented to our
any previous disease, was admitted due to a headache in sion presented with complete right hemi-hypoesthesia, service with chronic headache for 5 years. Initially, she
the left frontal region of the head, pulsating, with strong hemiparesis, and sudden aphasia 2 years ago. He had no had two episodes per week; however, frequency increased
intensity that started 03 days ago. He denied and previous previous history of headaches. Just a few minutes before up to daily attacks throughout the years. Headache was
similar episodes. On admission examination, he had left these neurological symptoms, he had a severe left temporal bilateral, pulsating, moderate to intense, and was even-
palpebral semiptosis associated with ipsilateral myosis, headache. Since then, he continued presenting episodes tually associated with nausea. It lasted on average two
with no other clinical signs. During clinical investiga- of headache with the same characteristics, two to three hours and always occurred during the morning, usually
tion, a brain magnetic resonance was performed, which times a week, associated with psychomotor restlessness, right after awakening. There was no worsening with phys-
showed no alterations. However, the brain angiotomog- ipsilateral conjunctival injection, and lacrimation, which ical activity, nor history of photophobia, phonophobia,
raphy elucidated an important tapering of the left inter- lasts about 90 minutes but occasionally exceeds 10 hours. aura or autonomic signs. She had multiple comorbidi-
nal carotid artery. Later, an angiography was performed The admission CT of the brain in the emergency room ties (i.e., type 2 diabetes, chronic renal failure, obesity,
that confirmed carotid artery dissection on the left of was normal, but the MRI three months later evidenced a hypertension, and glaucoma), as well as nonrestorative
the cervical portion with severe stenosis in the segment, hypointense lesion in the left pons region. Indomethacin sleep, fatigue, snoring, and nocturnal awakenings owing
which led the patient to undergo to angioplasty. Patient 75mg three times a day, carbamazepine 200mg twice a to pain. Neurological examination and magnetic reso-
evolved with improvement of pain and normalization of day, and propranolol 40mg twice a day were started, but nance imaging were unremarkable. Amitriptyline was
the neurological examination Discussion: Paratrigeminal without a good response. Due to the suspicion of second- started and increased up to 75 mg daily. After 8 months,
oculosympathetic syndrome or Raeder’s Syndrome is a ary cluster headache, a neural block with lidocaine and the patient had no improvement of the symptoms. A poly-
rare condition, with an incidence of 1 in 60,000, and was dexamethasone of the greater occipital nerve on the left somnography (PSG) was posteriorly performed, yielding
first described in 1924, consisting of a constant unilat- was performed and the use of sodium divalproate 500mg an apnea/hypopnea index of 62 events per hour. Diagnosis
eral headache, accompanied by Horner’s syndrome. Our daily was started. A 2-month follow-up showed a 90% of obstructive sleep apnea (OSA) was made. After three
patient fulfilled all the criteria according to the ICHD-3 decrease in headache attacks. Discussion: Primary tri- months of continuous positive airway pressure (CPAP)
classification. This syndrome can have several causes, geminal autonomic cephalalgias(TACs) are a rare group treatment, frequency decreased to one attack per week.
such as tumor, infection, and trauma, but carotid dis- of headache disorders. (1) A high number of secondary Discussion: OSA is associated with chronic headache.
section is the most common cause. It is divided into two cases of TACs were described with clinical features equal According to the International Classification of Headache
groups, group I, which includes patients with additional to primary TACs, and become a diagnostic challenge, Disorders – 3rd edition (ICHD-3), sleep apnea headache
involvement of some parasellar nerve (III, IV or VI) and being fundamental for the diagnosis of a complete and is a specific entity, characterized by morning headache
group II, where the parasellar nerves are not affected. careful anamnesis. (3) Qi Lei et al. described a case of presenting upon awakening and lasting for less than 4
This second group usually has a good and self-limiting SUNC secondary to brainstem infarction, demonstrat- hours, in more than 15 days per month, associated with
evolution. Final Comments: We present a rare cause of ing the association of infarction of this topography and confirmed OSA by PSG and pain improvement with apnea
secondary headache that, after its correct etiological inves- trigeminal autonomic cephalgia. (4) Final Comments: treatment. Our patient had a pattern of tension-type head-
tigation, had a favorable clinical outcome for the patient. We reported a case of trigeminal autonomic cephalgia ache without any response to tricyclic antidepressant.
secondary to ischemic vascular injury. Despite being a Besides, she had apnea risk factors (e.g., age, body mass
rare condition, it can lead to high morbidity. Be aware of index, hypertension, and post-menopausal status) and
Cefaleia fulfilled all the criteria above-mentioned. The expressive
this diagnostic possibility promotes a better functional
outcome for the patient. improvement in frequency and intensity after the gold
standard therapy with CPAP confirms the diagnosis.
Final Comments: The relationship of sleep disturbances
Cefaleia and headache is well known. Pain modulation and circa-
dian rhythm share neuroanatomical structures, such as
brainstem and hypothalamus. Insomnia, sleep-related
breathing disorders and circadian rhythm disorders are
associated with increased headache intensity, attack
frequency and risk of chronification. Therefore, it should
always be remembered in the evaluation of patients with
risk factors and non-responsiveness to first-line treatment
of primary headache.

Cefaleia

16
XXX Congresso Brasileiro de Neurologia

TL 1105821 TL 1106039 TL 1106126

SHOCKING, SHARP AND UNILATERAL PAIN HALOPERIDOL IN THE TREATMENT OF UNILATERAL OCULOMOR PALSY SECONDARY
OF ONSET IN THE EIGHTH DECADE OF MIGRATION STATUS : CASE SERIES IDIOPATHIC INTRACRANIAL HYPERTENSION:
LIFE: A CASE-REPORT OF PAROXYSMAL A RARE CASE PRESENTATION
Tiago Abreu Tempone
HEMICRANIA-TIC SYNDROME
Daniel Isoni Martins, Yan da Silva Raposo, Albert
Unimed. Poços de Caldas MG, Brazil
Thales Pardini Fagundes, Matheus Compart Louis Rocha Bicalho, Artemio Moreira Peixoto
Hemerly, Alisson Mangolin, Ellen Silva de Carvalho, tiagoabreu.med@hotmail.com Junior, Jacqueline Gomes da Silveira, Rogério
Natalia de Oliveira Silva, Roberto Satler Cetlin, Vale Estanislau, Renato Pereira da Costa Júnior,
Fabiola Dach Case Presentation: Headache is one of the most common Henrique Guimaraes Pires, Pedro Gadbem Bobst
complaints in the medical activity, especially, in emer-
Hospital das Clínicas da Faculdade de Medicina de Faculdade de Ciências Médicas de Minas Gerais.
gency care. The migraine is, among the primary headaches,
Ribeirão Preto. Ribeirão Preto SP, Brazil Belo Horizonte MG, Brazil
the most common. However, this is a pain that is often
Hospital das Clínicas Samuel Libânio. Pouso Alegre
matheuscompart@hotmail.com crippling and is characterized by being strong, pulsatile,
MG, Brazil
worsening to physical activities, being still duration of 4
Hospital São Lucas. Belo Horizonte MG, Brazil
Case Presentation: A 72-year-old woman with a five- to 72 hours, with a unilateral character in the most cases
Hospital Mater Dei Betim-Contagem. Betim MG,
month history of headaches was admitted at our head- in addition to being accompanied by nausea, photopho-
Brazil
ache outpatient clinic. The pain was localized in the bia and phonophobia. In this context, there is an unusual
periorbital and left temporal regions. It was daily, sharp, event, however of great relevance: the “migraine status”. yanraposo@yahoo.com.br
intense, sometimes throbbing, and with diffuse ipsilat- This is a framework for pain that lasts beyond the 72 hours
eral irradiation. Episodes usually lasted about 30 minutes verified in migraine crises. In this way, it is common for Case Presentation: A 38 years-old-woman, caucasian,
and occurred five times a day. The patient reported left abuse of analgesics in the context of “migraine status” civil engineer, residing in Belo Horizonte, Minas Gerais
hemiface heating during the attacks, however without the use of several anti-inflammatory drugs, ergotamine, State, Brazil. She has a depressive disorder and has been
conjunctival hyperemia, lacrimation, nasal congestion, or tryptans in addition to the non-recommended opium orally using Sertraline daily. She denied using hormonal
rhinorrhea. She sometimes referred shocking, short-last- derivatives. Thus there is in the literature the indication contraceptives. She reported chronic headaches for 10
ing pain triggered by biting food and touching certain of haloperidol in the treatment of migraine, especially years characterized by monthly episodes of moderate
points of the face. Previous treatments with duloxetine, in “migraine status”. Therefore, a series of seven cases of holocranial pressure starting two days before menstruation
amitriptyline, lamotrigine, and carbamazepine, initiated treatment of migranea seizures are reported, taking use and lasting up to three days after, and episodes of bilateral
by a neurologist in another clinic, provided no relief. Her of haloperidol as pain control medication. Discussion: It frontal pressure headache of severe intensity three to four
previous medical history was remarkable for treatment is verified in the literature, for the treatment of migraine, times per year. Arose in December 2020 with incipient
of hypothyroidism and presence of temporomandibular many analgesic options, specially in recent years, with right eyelid ptosis. In August 2021, she had a headache
joint dysfunction (TMJD). Physical examinations during the increasingly frequent use of tryptans with gradual episode that lasted about 30 days. Vitamin D on June 2021
pain-free periods were normal except for TMJD signs. reduction in the use of ergotamines due their adverse was 31.8 ng/dL. On January, 10th 2022 presented a new
Blood counts, liver, renal and thyroid function were nor- effects. Many patients seeking emergencies have already episode with exacerbation. Presented to the neurologi-
mal, as well as Brain magnetic resonance imaging (MRI). taken many groups of analgesic medications even before cal examination with a medial deviation of the right eye
Still using CBZ, the patient had pain in only the left side get to the Hospital. The use of haloperidol in a series of when looking up vertically and looking centered, right
of the face. Therapy was changed to 75 mg a day mg of seven cases, in an intravenous environment (5 mg) in pupil mydriatic and photoreactive, and right eyelid ptosis
indomethacin and was increased to 100 mg a day, with five of these cases and intramuscular application (5mg) without worsening of the deficit to repetition stimulation.
headache frequency improvement and up to five pain- in two patients between December 2019 and June 2021, is Magnetic Resonance Imaging of the brain on January
free consecutive days. After two months she was admitted discussed. Three patients are male and the other female. 17th without relevant abnormalities. On March 04th
due to recurrence of pain, with the same pattern, however The ages ranging from 28 years to 65 years. In all cases, during clinical reassessment, she reported spontaneous
associated with conjunctival hyperemia and eyelid edema. there was improvement after the use of the substance improvement of the headache. She had symmetrical pupils
Reintroduction of CBZ 400 mg a day in combination haloperidol, although several analgesics had been used but maintained right eyelid ptosis. Intracranial Pressure
with indomethacin resulted in complete improvement. previously showing the effectiveness of haloperidol com- non-invasive monitoring by Brain4 Care on March 30th
Discussion: Paroxysmal Hemicrania-Tic (PH-TIC) syn- pared to other treatments. Final Comments: This study showed normal oscillation of pressure levels, however, the
drome is a rare association between chronic paroxysmal reinforces the findings in the literature that support the P2/P1 ratio altered the change to decubitus, reaching a
hemicrania (CPH) and trigeminal neuralgia (TN). The age use of haloperidol in a dose of 5mg in the treatment of value of 1.8, compatible with intracranial hypertension.
of onset is usually between the fifth and seventh decades migraine crises, especially in “migraine status”, making Magnetic Resonance angiography of the intracranial
of life. Features such as pain of different durations, uni- use of the safety and good results that the substance offers. venous system on April 27th revealed a slight volumetric
lateral involvement of the face and partial response to reduction of the lateral ventricles associated with elon-
indomethacin should alert to the overlap between PH and gation and tortuosity of optic nerves, notably on the left.
Cefaleia A lumbar puncture with opening pressure of 31cm H20
TN. Frequently, no abnormalities are found in laboratory
tests or in brain MRI. CPH was improved with initiation was performed. Ophthalmoscopy of May 19th without
of indomethacin and trigeminal neuralgia with reintro- abnormalities. Optical coherence tomography this day
duction of CBZ. Final Comments: PH-TIC is uncom- showed a borderline reduction of the inferior temporal
mon, however it should be hypothesized in patients with thickness of both eyes and a reduction of the superior
short-lasting headaches associated with facial pain that temporal area of the left eye. On May, 25th started oral
partially improve with carbamazepine or indomethacin. polyvitamin supplement weekly, cholecalciferol weekly,
by case series, over single pharmacotherapy. and acetazolamide twice a day. Discussion: The sixth
cranial nerve is most commonly affected. Third cranial
nerve involvement is distinctly unusual. Final Comments:
Cefaleia Systematic review of cases of ophthalmoplegia in the set-
ting of idiopathic intracranial hypertension published last
year identified only one case presentation of unilateral
oculomotor palsy.

Cefaleia

17
XXX Congresso Brasileiro de Neurologia

TL 1106205 TL 1106287 TL 1106296

ONABOTULINUM TOXIN TYPE A AS AN ADD- SPONTANEOUS PNEUMORRACHIA: A RARE ULTRAFAST DYNAMIC CT MYELOGRAPHY
ON THERAPY IN REFRACTORY TRIGEMINAL CAUSE OF STROKE MIMICS FOR THE IDENTIFICATION OF LEAKAGE
NEURALGIA: PRELIMINARY RESULTS LEVEL IN MULTIPLE MENINGEAL
Carolina Franciely Vitor Miranda, Ana Lucia de
Carvalho Mello, Saulo Ramos Ribeiro, Evelyn DIVERTICULA
Thábata Emanuelle Martins Nunes, Paula Adriele
Santos, Léo Coutinho, Isabela Maria Garcia Pacheco, Mariana Okada, João Brainer, Gustavo Mohamed Ali Hussein, Camila Carneiro Ferreira,
Marcolla, Patricia Aurea Andreucci Martins Bonilha, Kuster, João José Freitas de Carvalho, Jose Carlos Bernardo Corrêa de Almeida Teixeira, Kristofer
Beatriz Cassarotti, Elcio Juliato Piovesan, Hélio Teixeira Garcia, Renan Domingues Ramina, Afonso Aragão, Bruno Camporeze, Luiza
Afonso Ghizoni Teive Gonçalves Fraga, Ludmila Machado Lima
Samaritano Paulista, São Paulo SP, Brazil
Hospital das Clínicas da Universidade Federal do Americas Serviços Médicos/UHG. Rio de Janeiro RJ, Instituto de Neurologia de Curitiba. Curitiba PR,
Paraná. Curitiba PR, Brazil Brazil Brazil
Amil Medicina e Saúde/UHG, São Paulo SP, Brazil
thabatamnunes@gmail.com Américas Serviços Médicos/UHG, São Paulo SP, camilasilcarneiro@gmail.com
Brazil
Background: Trigeminal neuralgia (TN) is a rare but Américas Serviços Médicos/UHG. Fortaleza CE, Case Presentation: A 26 year-old woman with clinical and
often debilitating condition, characterized by paroxys- Brazil neuroimaging criteria for low cerebrospinal fluid (CSF)
mal attacks of sharp pain in one or more branches of pressure headache was submitted to a CT myelography
the trigeminal nerve. Although several pharmacological sauloramosribeiro@gmail.com which identified multiple thoracic meningeal divertic-
options are available for prophylaxis, the treatment of TN ula, but specific site of leakage was not possible due to
is frequently unsatisfactory. Onabotulinum toxin type A Case Presentation: Female patient, 39 years old, was temporal resolution. Ultrafast dynamic CT myelogra-
(OBTA) is proven safe and effective in migraine and cra- admitted to the emergency department of a private phy was then performed and confirmed opacification
nial neuralgias such as occipital neuralgia, representing Hospital in the city of São Paulo, after a severe headache of only a T8-T9 right-side meningeal diverticula in the
a possible therapeutic option in TN. Objectives: This followed by two episodes of syncope and motor deficit initial phases with later opacification of the remaining
study evaluated the effects of OBTA injections in a cohort in the left upper limb. Neurological examination showed diverticula and epidural spaces. Targeted surgical treat-
of patients with refractory TN. Methods: We enrolled motor deficit in the left upper limb without sensitive ment with diverticula coagulation and fat grafting with
patients with confirmed TN referred to our botulinum impairment. She had previous history of bipolar affective fibrin glue resulted in symptoms resolution. Discussion:
toxin outpatient clinic, taking standard oral prophylaxis disorder, chronic anemia, and overweight with previous Spontaneous intracranial hypotension (SIH) seems to be
with partial or no response. The Intensity (estimated bariatric surgery. The patient was submitted to computed related to the structural fragility of the dura mater, which
with the visual analog pain scale) and frequency of pain tomography (CT), arterial angiotomography of the skull promotes, even if due to minor traumas (eg, coughing and
(estimated by the number of days with pain per month) and cervical neck, which showed rare small gaseous foci sneezing), a meningeal rupture. Cases that are refractory
were noted before the start of prophylaxis, with prophy- in the perivertebral soft tissues and extradural site on the to clinical treatment may be submitted to a blood patch
laxis alone, and 3 months after OBTA injections. OBTA left side in the craniovertebral transition, near the foramen or surgical intervention. Only 50% of patients with SIH
dosage and number of injection points, as well as other magnum. Small foci of pneumocephalus were found in improve after the first blood patch. In refractory cases,
demographic and clinical data, were noted. Results: Our the posterior fossa, near the left sphenopetrous fissure. the ideal is to identify the exact point of CSF leakage for
preliminary sample of 20 patients was marked by a pre- Electroneuromyography (ENMG) of four limbs was per- surgical correction. Myelotomography is highlighted here,
dominance of females (n = 11, 55%), with a median age formed, with normal results, and brain and cervical mag- as it can identify meningeal diverticula, which correspond
of 70.5 years (Min 44, Max 87), with idiopathic TN (n = 12, netic resonance imaging (MRI) performed 48 hours after to points of fragility. Ultrafast dynamic CT myelography is
60%). Carbamazepine was the most frequent prophylactic CT showed no more expression of the changes described an option in cases where there is more than one divertic-
(used by 14 patients, 70% of the sample). Patients reported on CT. She was treated with analgesia and was discharged ulum and if you want to identify the one responsible for
pain with a median intensity of 10 (Min 9, Max 10) and with referral to rehabilitation service and outpatient fol- the leak. This technique involved positioning the patient
occurring daily (30 days with pain per month in the entire low-up. Discussion: Spontaneous pneumorrhachia is in Trendelenburg and application of intradural con-
sample) before prophylaxis. Although prophylaxis was rare disease and is characterized by the presence of air trast while the images were obtained. Final Comments:
effective to reduce pain intensity, lowering an average of in the spinal canal, both in the intradural and extradural Ultrafast dynamic CT myelography is a technique that has
3.5 points (p < 0.0001, IC 95% 2 – 5), it was ineffective to compartments. It is usually benign with spontaneous res- been employed mainly in ventral dural ruptures related
reduce the frequency, and the entire sample maintained olution. Our patient was first suspected as having a stroke, to osteophytes, but can also be applied in SIH, besides
daily pain. When compared to prophylaxis alone, OBTA due to the thunderclap headache and focal motor deficit. the conventional dynamic myelography.
+ Prophylaxis further reduced intensity on average by 4.9 CT revealed the diagnosis Final Comments: Spontaneous
points (p < 0.0001, IC 95% 3 – 5.9), and reduced frequency pneumorrhachia is a rare cause of stroke mimics. Cervical
on average by 28 days with pain per month (p = 0.002, IC CT should be carried out in patients with thunderclap Cefaleia
95% 23 – 30). In our sample, 7 patients (35% of the sam- headache and radicular pain in an upper limb.
ple) reported a full response. The mean duration of effect
was 80.75 ± 20.14 days. No side effects were registered in
our sample. Final Comments: Our results suggest that Cefaleia
OBTA is a safe and effective add-on treatment option for
patients experiencing TN refractory to oral prophylaxis.
Although OBTA was particularly effective in reducing the
frequency of painful episodes, further studies are neces-
sary to confirm these findings.

Cefaleia

18
XXX Congresso Brasileiro de Neurologia

TL 1106298 TL 1106301 TL 1106391

SHORT-LASTING UNILATERAL HEADACHE SECONDARY TO MENINGITIS AN UNUSUAL CAUSE OF HEMICRANIAL


NEURALGIFORM HEADACHE ATTACKS WITH DUE TO M. TUBERCULOSIS RESPONSIVE TO HEADACHE: A CASE PRESENTATION
CONJUNCTIVAL INJECTION AND TEARING NERVE BLOCKAGE: A CASE PRESENTATION
Franklin de Castro Alves Neto, Gustavo Rodrigues
(SUNCT) RESPONSIVE TO INTRANASAL Ferreira Gomes, Danyela Martins Bezerra Soares,
Pedro Rodrigues Neves, Laura Motter Rosso, Cesar
LIDOCAINE AND NERVE BLOCKAGE: A CASE Augusto Weschenfelder, Daniel Teixeira dos Santos, Jorge Luiz Brito de Sousa, Rebeca Bessa Mauricio,
PRESENTATION Leonardo Galdino da Silva, Thalis Silva de Oliveira, Késia Sindy Alves Ferreira Pereira, Davi Lopes
Renata Londero Santos, Ingred Pimentel Guimarães, Abel Dantas de
Pedro Rodrigues Neves, Cesar Augusto
Figueiredo Belém, Danilo Nunes Oliveira
Weschenfelder, Daniel Teixeira dos Santos, Thalis Escola de Medicina da Pontifícia Universidade
Silva de Oliveira, Leonardo Galdino da Silva, Laura Católica do Rio Grande do Sul. Porto Alegre RS, Universidade Estadual do Ceará. Fortaleza CE,
Motter Rosso, Wyllians Vendramini Borelli, Gabriel Brazil Brazil
Paulo Mantovani, Raphael Machado Castilhos, Universidade Federal do Rio Grande do Sul. Porto Universidade Federal do Ceará. Fortaleza CE, Brazil
Renata Londero Alegre RS, Brazil
gustavo_r_f_gomes@hotmail.com
Hospital de Clínicas de Porto Alegre. Porto Alegre
Escola de Medicina da Pontifícia Universidade
RS, Brazil
Católica do Rio Grande do Sul. Porto Alegre RS, Case Presentation: A 67-years-old woman presents in
Brazil danieltds1@gmail.com an outpatient clinic with a 4 month history of new onset
Universidade Federal do Rio Grande do Sul. Porto left headache and progressive periorbital pain. Few days
Alegre RS, Brazil Case Presentation: A 49-year-old male patient, with before the consultation, she noticed discrete binocular
Hospital de Clínicas de Porto Alegre. Porto Alegre migraine since 10 years old, seeked medical attention diplopia on rightward gaze. Considering red flags (new
RS, Brazil with a new and distinct headache pattern that initiated onset, age > 40 years, persistent hemicrania) a brain mag-
in the previous two months and had a progressive inten- netic resonance imaging (MRI) was requested. The MRI
danieltds1@gmail.com
sity and frequency. It was described as a high intensity shows a round expansive lesion at the medial corner of
continuous pressure in the right frontal-parietal-occipital the left orbit, well-defined margins, measuring 1,5x1,1x-
Case Presentation: A 75-year-old male patient went
topography, with worsening periods of recurrent stabbing 1,0cm, extraconal, touching the left rectus medialis mus-
to the emergency department due an intense recur-
pain episodes. Initially, a cluster headache diagnosis was cle inferomedially. The lesion had intermediate signal
rent and refractory daily headache that initiated in the
suspected due to possible autonomic features associ- in T1 and high signal in T2 at its internal portion and
previous two months, was described as a stabbing, was
ated and a treatment with a right greater occipital nerve diffusion restriction and post-contrast heterogeneous
exclusively right-sided and had maximal duration of
block, high-flow oxygen and sumatriptan when head- enhancement, predominantly peripherally. Hypotheses of
10 seconds, with recurrence at least every 10 minutes
ache recurred was offered and preventive treatment with Schwannoma or metastasis were considered for the case.
and no refractory period. It was mostly perceived in the
Verapamil was initiated. Also, laboratory and advanced Upon the metastasis hypothesis, new imaging tests were
ophthalmic nerve (V1), with ipsilateral allodynia and
neuroimaging investigation turned out negative. Two performed. A chest CT revealed a 1,8 cm spiky nodule at
autonomic symptoms, such as conjunctival injection,
weeks later, he reported improvement in the right side the right posterior basal segment of the lung and lym-
lacrimation and ptosis. The pain was unresponsive to
pain but described a new headache with the same char- phomegalies compatible with secondary implant, neck CT
acetaminophen and non-steroidal anti-inflammatory
acteristics as before on the left side that didn’t respond showed multiple lymphomegalies, abdominal CT showed
drugs. In his neurological examination, we reproduced
to the prescribed treatment and reported a worsening of hepatic nodules suggestive of secondary implants. Blastic
the headache’s episodes when the right V1 region was
pain when lying down in his bed. A left greater and lesser image at L1 suggestive of secondary implant. The patient
lightly touched. Based on the elevated frequency, short
occipital nerves block, trigger points injections in bilat- was diagnosed with stage 4 non-small cell lung cancer
duration of attacks and presence of ipsilateral autonomic
eral trapezius and left semispinalis were performed and and referred to oncology service. Discussion: Although
features, SUNCT diagnosis was made. A treatment regi-
a lumbar puncture was scheduled. Five days later, the a minority of patients presenting with headache have
men of trochlear, supra-orbital, major and minor occipital
patient had a marked headache improvement, presenting a secondary cause, screening for these cases by ques-
nerve anesthetic blockades using 2% lidocaine without
only 3 episodes in 2 weeks, with a mild intensity. Lumbar tioning and evaluating some red flags can be useful to
vasoconstrictor was made in association with a preven-
puncture revealed a mildly elevated opening pressure (270 reduce costs, false-positives and/or incidental findings
tive treatment with Lamotrigine 100mg twice a day. Also,
mmH2O) with significant elevations in cell and protein provided by routine use of blood tests and neuroimaging.
he was instructed to do a sphenopalatine ganglion block
counts. A PCR test for M. tuberculosis was positive and Considering this purpose, a common mnemonic currently
by slowly dripping 2 mL of intranasal 2% lidocaine in his
treatment for this condition was initiated. Discussion: A used is SNNOOP10. Eye is a rare site of lung cancer metas-
right nose when symptoms recurred. The patient had
minority of headache patients have a secondary headache tasis although metastasis to the eye represents by far the
no pain for at least six months of these treatments, not
disorder and the medical literature promotes red flags to most common form of intraocular and orbital cancer.
needing to use intranasal lidocaine anymore. Discussion:
direct the clinician to initiate a workup plan. Sometimes, A review of 42 case presentation of ocular metastasis in
Trigeminal autonomic cephalalgias comprehend a myriad
we may not proceed to further investigation even with a lung cancer found that the most common lung cancer
of headaches which include the short-lasting unilateral
red flag present if the patient’s headache presents signif- that metastasizes to the eye is adenocarcinoma. In this
neuralgiform headache attacks with cranial autonomic
icant improvement with medical treatment. In this case, study, ocular symptom was the first referred symptom
symptoms (SUNA) and with conjunctival injection and
our patient had significant response to bilateral occipital in 83% (35) cases. The choroid was the most common
tearing (SUNCT). Intravenous lidocaine has been con-
nerve blocks but, nevertheless, further investigation was site for ocular metastasis. Final Comments: In our case,
sistently described as the treatment of choice for acute
warranted because of the presence of recent onset of new recent onset, age above 50 years, neurologic dysfunction
episodes, but there is a risk of severe cardiac side effects
headache and positional headache. Final Comments: and progressive nature warranted further investigation,
(arrhythmias), mainly for older adults. Therefore, they
Neurologists worldwide estimate that 18% of patients which was of utmost importance to the diagnosis. We
can benefit from alternate, less harming and secure treat-
with a headache have a secondary headache disorder. therefore encourage further neurological investigation
ment options such as intranasal lidocaine drip and nerve
This case reinforces that even the improvement of head- of patients presenting with headache red flags in order
blockades in the area corresponding to the pain, like in
ache with occipital nerve blockage or other treatments to better establish the diagnosis.
this case. Final Comments: Our SUNCT patient had a
must not exempt the need to investigate the existence
very satisfactory response of headache after treatment
of a secondary cause of headache, which proved to be
with the simple and relatively safe procedures previously Cefaleia
crucial for this patient.
described, which presented efficient acute pain control
within a large follow-up period. Similar acute treatments
were reported for SUNCT/SUNA with satisfactory results, Cefaleia
but data on this topic are still scarce in the literature, being
based mostly on case presentation and small studies, so
further studies are mandatory in order to corroborate the
findings of this case presentation.

Cefaleia

19
XXX Congresso Brasileiro de Neurologia

TL 1106423 TL 1106487 TL 1106580

PERIPHERAL FACIAL PALSY AS A FALSE TRANSVERSE SINUS ANGIOPLASTY (TSA) SMART SYNDROME – A CASE
LOCALIZING SIGN OF INTRACRANIAL WITH STENT TO REFRACTORY IDIOPATHIC PRESENTATION
HYPERTENSION: A CASE PRESENTATION INTRACRANIAL HYPERTENSION
Luciana Queiroz, Gabriela Romualdo, Danielle
Daniel Alves de Oliveira, Vitor Maia Arca, Arthur Juliana Naback Toniolo, Caroline Darold Vieira, Ellen Lacerda, Raíssa Garibe, Rodrigo Dourado, Leandro
Cesário de Holanda, Victor de Moraes Chagas, Silva de Carvalho, Giulia Maria Ximenes Verdi, Maria Aguiar, Breno Franco
Raphaelly Ribeiro Campos, Bruno Henrique Carneiro Clara Baseio, Matheus Compart Hemerly, Natália
Hospital Felicio Rocho. Belo Horizonte MG, Brazil
Costa Filho, Ana Rosa Santana, Geovane Gomes de Oliveira Silva, Roberto Satler Cetlin, Daniel
Silva, Marcos Eugênio Ramalho Bezerra, Eduardo Giansante Abud, Fabíola Dach luciana-queiroz@hotmail.com
Sousa de Melo
Universidade de São Paulo. Ribeirão Preto SP, Brazil
Case Presentation: P.S.P is a 57 years-old male, with a
Hospital das Clínicas da Universidade Federal de
juliananaback@gmail.com past history of resection of an ependymoblastoma in the
Pernambuco. Recife PE, Brazil
thalamic region requiring adjuvant radiotherapy in 1989.
daniel_oliveira_@live.com Case Presentation: Idiopathic intracranial hypertension In 2005, he initiated a decline in functionality manifested
(IIH) is a relatively uncommon syndrome, but in recent by cognitive complaints, loss of balance and gait distur-
Case Presentation: A 30-year-old female patient pre- years its prevalence has been increasing. Our group ana- bances, with slow and progressive worsening over time. In
sented with acute, pulsatile, progressive, continuous left lyzed data from six patients, mean age of 33.5 years, with the past 4 years, he began to experience throbbing head-
occipitofrontal headache, nausea and vomiting. One refractory IIH treated in a tertiary neurology center. They aches with moderate to severe intensity and preceded in
week later, she started feeling a heavy sensation on right had poor response to pharmacological treatment and were 2 to 3 days by auto-limited episodes of aphasia and gait
hemiface and low vision in the right eye, and after 1 week submitted to a transverse sinus angioplasty (TSA). The instability. In 2021, he was admitted to our Hospital pre-
in the left eye. She denied diplopia She used combined time from symptoms onset to interventionist treatment senting a migraine-like episode, accompanied by aphasia
contraceptives daily. On physical examination, she had varied from 14 days to 4 years. The general technique was and right-sided hemiparesis, with prolonged duration.
low visual acuity of 20/80 in the right eye and 20/60 in transverse sinus stenting but a sigmoid sinus stent was Brain MRI showed T2/FLAIR hyperintensity involving
the left eye; funduscopy showed papilledema; she also also implanted in one of the cases. Three patients had pain the cortex of the temporal and occipital lobes of the left
had right peripheral facial palsy with lagophthalmos relief varying between 1 to 2 days after the procedure with hemisphere, with gyriform gadolinium enhancement,
and Bell’s sign. CT angiography showed filling failure in drugs subsequently withheld. The other three persisted without water diffusion restriction. Videoencephalogram
the left transverse sinus and in the transition between with pain and it was necessary to maintain medications. showed rare epileptiform discharges in the left tempo-
the sigmoid sinus and the jugular bulb, without signs Two of them had an immediate response, but the pain ral region and left-sided bursts of frontal rhythmic delta
of venous infarction. Cerebrospinal fluid showed an returned with the same intensity before the procedure. waves. Resonance angiography and blood analysis was
opening pressure of 50cmH?O and a closing pressure of One of them had headache immediately after the proce- unremarkable. The patient was diagnosed with Stroke-
25cmH?O, with no changes in the CSF laboratory analysis. dure. Discussion: Although the IIH physiopathology did like migraine attacks after radiation therapy syndrome
The diagnosis of intracranial hypertension (HI) secondary not have been completely elucidated and multifactorial (SMART syndrome) and treatment with dexamethasone
to cerebral venous thrombosis (CVT) was defined and full mechanisms are proposed, there is a clear association and analgesics was initiated, with complete resolution of
anticoagulation and acetazolamide were initiated. Thus, with obesity and female gender. Recently, it has been the symptoms in 3 weeks. Discussion: SMART syndrome
an etiological investigation of peripheral facial paralysis observed that transverse sinus stenosis is a prevalent is a rare delayed neurological complication of cranial
was carried out; however, CT of the sinuses and tempo- finding in these patients, but it is not known if it has a radiotherapy, manifesting years after the initial treatment.
ral bones, as well as the MRI of the inner ear, were nor- causal relationship with elevation of intracranial pressure The syndrome usually presents and is diagnosticted with
mal, concluding as a probable cause of a false localizing or if this finding is a consequence of that. The treatment recurrent and often reversible episodes of migraine-like
signal of HI and CVT. Discussion: The pathogenesis of is based on weight loss and drugs that reduce produc- attacks, seizures and/or focal neurological deficits, rep-
false intracranial localization signals usually occurs in tion or increase drainage of cerebrospinal fluid. The aim resenting transitory dysfunction of the irradiated cortical
the context of IH with herniation syndromes, with VI is to reduce intracranial pressure, relieve headache and areas, associated with reversible, transient and unilateral
nerve palsy being more common in 14% of idiopathic avoid visual loss. TSA with stent has been pointed out as cortical gadolinium enhancement associated with abnor-
intracranial hypertension (IIH) cases. The finding of VII an option for refractory cases in which transverse sinus mal T2/FLAIR signal changes of the affected brain region.
nerve palsy is uncommon in HI, in a case series of 140 stenosis with hemodynamic repercussion (pressure gra- Image control after two months can be useful to assess
patients with IIH, only 2 cases had VII nerve palsy with a dient greater than 7mmHg) is confirmed by venography. the resolution of the alterations. Differential diagnosis can
peripheral pattern. As a possible pathophysiology, there Final Comments: In refractory IHH with confirmed dural be mainly tissue necrosis. Treatment consists of manag-
may be compression or stretching of the facial nerve in sinus stenosis, angioplasty with stent placement has been ing symptoms with analgesics, anti-seizure medications
its intratemporal segment close to the facial canal, in suggested as an effective therapy. Published meta-anal- and corticosteroids. Typically, the course of the disease
addition to reduced drainage of the extensive venous yses consider significant symptom relief – improvement is self-limiting. Final Comments: The case presented
plexus that accompanies the nerve in the facial canal. of up to 90% of papilledema, 80% of visual symptoms, illustrates an extremely rare delayed neurological com-
Final Comments: Although rare, the finding of periph- and 80% of headache. Our case series showed symptom plication of cranial radiotherapy. SMART syndrome is
eral facial paralysis may have intracranial hypertension benefits in only 50% of patients. However, in patients a radiologic and clinical entity of importance, since its
as one of the possible etiologies. who responded to therapy, the response was dramatic recognition as a self-limited disease prevents potentially
and allowed to suspend the pharmacological therapy. iatrogenic diagnostic tests pursuing tumor recurrence and
also potentially harmfull treatments.
Cefaleia
Cefaleia
Cefaleia

20
XXX Congresso Brasileiro de Neurologia

TL 1104886 TL 1105133 TL 1105198

DUMBBEL-SHAPED INTRA AND REVERSIBLE BITEMPORAL DANGEROUS HINTS AND NORMAL MRI:
EXTRADURAL HYPOGLOSSAL HEMIHYPOKINETIC PUPIL WITHOUT DON’T FORGET INFERIOR VESTIBULAR
SCHWANNOMA: CLINICAL PRESENTATION HEMIANOPSIA: A NEW CHIASMAL SIGN NEURITIS
AND MICROSURGICAL TREATMENT IN
Jorge Alberto Martins Pentiado Junior, Fernando Fernando Henrique Magri Alves, Alisson Dias
BRAZIL Henrique Magri Alves, Eduardo Yukio Nakamura, Azevedo Souza, Emanuelle Roberta da Silva Aquino,
Stella de Angelis Trivellato, João Lucas Gomes Lucas Ravagnani da Silva, Patricia Approbato Bruna Melo Holanda Cavalcanti, Danilo Martins
Salgado, Marco Antonio Zanini, Mario Wolak Jr, Leon Marques, Natalia Oliveira da Silva, Ricardo Santos Real, Roseli Saraiva Moreira Bittar, Cristiana Borges
Pinheiro, Francisco Moura Neto de Oliveira, Maria Lucia Habib Simão Pereira

Hospital de Clínicas da Faculdade de Medicina de Universidade de São Paulo, São Paulo, São Paulo Hospital das Clínicas da Universidade de São Paulo.
Botucatu – UNESP. Botucatu SP, Brazil SP, Brazil São Paulo SP, Brazil
Universidade de São Paulo. São Paulo SP, Brazil
stella.trivellato@gmail.com Hospital São Lucas. Ribeirão Preto SP, Brazil fernando.h.m.alves@gmail.com
Universidade de São Paulo, Ribeirão Preto, São
Case Presentation: 51-year-old female presented with Paulo SP, Brazil Case Presentation: We describe two patients complaining
history of progressive dizziness for the past 2 years, asso- of sudden dizziness in the emergency department whose
ciated with long date left tinnitus. The patient reported fernando.h.m.alves@gmail.com examination revealed dangerous HINTS but normal image
no nausea, gait disturbance, phonation or swallow diffi- studies. A 52-years-old woman presented with acute ver-
culties. On examination, she was alert and oriented; her Case Presentation: A 38-year-old woman presented with tigo associated with vomiting, tinnitus and severe imbal-
left hemi-tongue was atrophic and showed deviation to a complain of a two-day right retro-orbital pain and right ance. She had left torsional spontaneous and positional
the left side on protrusion. The palate elevation and uvula progressive visual loss. Upon examination, she had a right downbeat and torsional to the left nystagmus and normal
contraction were normal, with vomiting reflex preserved, relative afferent pupillary defect (RAPD) and dyschroma- vestibulo-ocular reflex (VOR). A 66-years-old man also
as well as hearing acuity and cerebellar function tests. topsia, with normal visual field testing (confrontation) presented with acute vertigo, vomiting and imbalance.
MRI images showed a solid extra-axial lesion, homoge- and funduscopic findings. The visual acuity was 0.8 in He had positional downbeat and torsional to the left
neus, with gadolinium enhancement, occupying the left the right eye and 1.0 in the left eye. The automated perim- nystagmus, a normal VOR, felt to the right side with eyes
lateral clivus and petrous bone, with extension to the left etry test (APT) demonstrated a mild superior temporal closed when standing on foam, subjective visual vertical
hypoglossal canal and jugular foramen, and also caudal reduction in sensitivity in the field of the left eye and a was abnormal (2,5º) to the right. Both patients had normal
extension. A cerebral angiography was also performed, superior temporal scotoma with diffuse loss of sensitivity brain MRI and abnormal video-head impulse test (vHIT),
showing left transverse and sigmoid sinuses stenosis, (pseudoconstriction) in the right eye. A more thorough showing low gain (0.37 and 0.3 respectively) of VOR for
with predominant contralateral drainage and absence of pupil examination was performed and it was noted that the right posterior semicircular canal gain of the posterior
tumour blush. After proper investigation, the patient was the pupil finding was not simply a RAPD. When hemifield semicircular canal and corrective saccades, receiving the
submitted to microsurgical resection of the lesion through light stimulation of each eye was performed individually diagnosis of vestibular neuritis of the inferior division of
a far lateral transcondylar approach. During the surgery (while occluding the non-tested eye) with a beam flash- the right vestibular nerve. Discussion: The HINTS has a
it was observed that the tumour eroded the left occipital light, a peculiar pupil response was noted: right nasal high sensitivity (97%) and specificity (95%) in discerning
condyle, which was resected; the tumour had also an hemiretina light stimulation demonstrated feeble pupil central causes of acute vestibular syndromes. Nonetheless,
intradural extension, which could be removed with pres- contraction, whereas stimulation of the temporal hemiret- some peripheral etiologies could mimic central patterns
ervation of the hypoglossal nerve. It was not noticed any ina resulted in better pupil contraction. In the left eye, the in the first evaluation. The isolated unilateral involve-
dural sinus involvement. The dura was by the end of the difference was not as significant as contralaterally, but it ment of the inferior division of the vestibular nerve may
procedure sutured with a galeal patch to cover its defect. was also notable. These findings have neen reversed after manifest as positional downbeat nystagmus with normal
Intraoperative anatomopathological analysis demon- surgical treatment of the lesion. Discussion: In this study, horizontal VOR and must be included in the differential
strated low grade neoplasia suggestive of Schwannoma, we describe a novel chiasmal sign, reporting a patient in diagnosis in a patient with normal MRI. Final Comments:
later confirmed by definite immunohistochemical analy- which a pupillary bitemporal hemihypokinesia was not An acute onset vertigo, dangerous HINTS, with downbeat
sis. In the post-operative care, there was no development accompanied by hemianopsia and the phenomenon was nystagmus and normal brain MRI should remind of ves-
of any new neurological deficits and the patient noticed reversible after the surgical treatment. We hypothesizes tibular neuritis of the inferior division of the vestibular
improvement of dizziness. The tongue symptons from that there would be a separation between the afferent nerve. The vHIT is of great help to confirm the diagnosis.
hypoglossal paresia persisted. During the later 24 months visual fibres and the pupillomotor fibres in the chiasm,
of follow-up, there was no evidence of tumour recurrence being the last ones responsible for the referred chiasmal
sign (like an ‘alarm signal’) and superficially distributed Distúrbio Vestibulares e do Equilíbrio
on clinical examination and MRI images. The first case of
Hypoglossal Schwannoma was described In 1933, with in this anatomical structure, similarly to the parasympa-
about one hundred new descriptions in the international thetic fibers along the third cranial nerve; they would be,
literature since then. The proper treatment consists of therefore, susceptible to extrinsic compressive lesions
surgical resection of the tumour as curative option, being (and reversible after decompression). Final Comments:
the far-lateral transcondylar approach – used in this case We hope that other researchers will be able to address
– of great value. This approach enables the exposition of the main question of our study and assess the accu-
vertebral arteries and low cranial nerves, what facilitates racy of the indicated chiasmal sign in the diagnosis and
the tumour resection with lower risk of damage to physi- screening of patients with chiasmal diseases of different
ological vascular and nervous structures. aetiologies (e.g., neoplastic, demyelinating, vascular,
and inflammatory diseases) by comparing the results of
automated methods (e.g., pupillometry, pupillary cycle,
Distúrbio Vestibulares e do Equilíbrio retinal ganglion cell analysis) and non-automated meth-
ods (flashlight, slit lamp).

Distúrbio Vestibulares e do Equilíbrio

21
XXX Congresso Brasileiro de Neurologia

TL 1105847 TL 1106621 TL 1106631

VERTICAL ONE-AND-A-HALF SYNDROME CERVICAL DYSTONIA ASSOCIATED WITH L2- DYSKINESIAS IN A PATIENT WITH
IN A PATIENT WITH PECHERON ARTERY GLUTARIC ACIDURIA (L2HGA) CEREBROTENDINOUS XANTHOMATOSIS
ISCHEMIA: A CASE PRESENTATION
Isabel Maria Oliveira Macedo Lima, Assíria Leite de Isabel Maria Oliveira Macedo Lima, Bruna Tavares
Aline Moreira Lócio, João Pedro Benati, Beatriz Azevedo Costa, Carlos Eduardo Cordeiro Cavalcante, Falcão, Maria Eduarda de Souza Arêa Leão, Natália
Coutinho, Lara Paiva, Isadora Paiva, Victor Araújo, Gabriel Cipriano Feitosa Oliveira, Kelson James Araújo Nascimento Viana, Marcello Holanda de
Rafaella Iuguetti, Victória Maria, Lia Jereissati, Julio Almeida Andrade, Rairis Barbosa Nascimento, Thiago Assis
Santos Borges Morais, Kelson James Almeida
Universidade Federal do Piauí. Teresina PI, Brazil
Centro Universitário Christus – Unichristus. Universidade Federal do Piauí. Teresina PI, Brazil
isabelmariaoliveiramacedolima@gmail.com Centro Universitário UniFacid. Teresina PI, Brazil
Fortaleza CE, Brazil

aline.locio@hotmail.com Case Presentation: A 38-year-old female patient, daughter isabelmariaoliveiramacedolima@gmail.com


of consanguineous parents, seeks neurological care with
Case Presentation: A 78-year-old female patient, pre- complaints of worsening gait and seizures. During child- Case Presentation: Male, 48 years old, seen at a neu-
sented at the Hospital emergency with approximately hood, she presented learning disabilities and regression rology outpatient clinic, attendant reports total aphasia.
12 hours of sudden onset of diplopia, associated with in developmental milestones, besides frequent falls and Since childhood he refers to retarded neuropsychomo-
dizziness. Neuro-ophthalmological examination showed fainting. From 32 years of age on, he reported repeated tor development, besides low weight and seizures. Sister
no blinking alterations or ptosis. It also revealed torsional episodes of seizures and gradual worsening of gait, requir- has a similar clinical picture. Presents global ataxia, does
nystagmus associated with bilateral upgaze palsy with ing a wheelchair. Recently, he complains of episodes of not assume orthostasis, axial component, dysmetria and
limitation of infraduction on the left. Pupillary reflexes behavioral arrest. On physical examination, he presents decomposition of the upper limbs bilaterally, asymmet-
were hypoactive bilaterally. No facial nerve paresis was cervical dystonia in laterocollis, atetotic movements in rical to the left. Extended arms maneuver: high ampli-
observed. Previous pathological history consisted of type the left upper limb, and stereotyped open-closed move- tude and low frequency tremor, associated with ataxia.
2 diabetes mellitus and systemic arterial hypertension ments in both hands. Magnetic resonance imaging of the He has dyskinetic movement of head, trunk and limbs,
(SAH). Laboratory exams had no apparent abnormali- skull showed leukodystrophy and ventricular dilatation. axial predominance in the neck region and spontaneous
ties. The cardiac examination also had no evident alter- He has a previous diagnosis of Dandy-Walker syndrome. nystagmus. He has nodules in joints, suggestive of ten-
ations. Brain Computerized Tomography and Magnetic Finally, genomic analysis was performed, with identifi- dinous xanthomas. Symmetrical reflexes bilaterally in
Resonance (MR) angiography were normal. Later mag- cation of homozygosity in L2HGDH gene and diagnosis upper limbs and exalted in bilateral patellar, bilateral
netic resonance imaging (MRI) showed a restriction of of L-2-Hydroxyglutaric aciduria. Development: L2HGA is flexor cutaneous-plantar reflex, unresectable glabellar.
the diffusion of water molecules in the thalamic regions an autosomal recessive neurometabolic disorder caused Magnetic resonance imaging (MRI) of the skull showed
and the mesencephalic tegment more evident in the right by a deficiency of L-2-hydroxyglutarate dehydrogenase intra-axial, non-expansive, bilateral, symmetrical lesions
rostral region. The diagnosis was made with the combi- due to homozygous or heterozygous mutations in the in the white matter of the cerebellar hemispheres, associ-
nation of an ophthalmoparetic syndrome suggesting a L2HGDH gene. The symptoms are developmental and ated with hyperintense areas on T2/FLAIR in brainstem
midbrain impairment associated with the MRI results. cognitive retardation, seizures, gait problems, as there and basal nuclei, suggestive of cerebrotendinous xan-
Treatment protocol included dual platelet antiaggrega- are cerebellar findings in symptomatic patients such as thomatosis (CTX) when associated with clinical. Patient
tion with acetylsalicylic acid and clopidogrel. Followed ataxia, dyskinesia presented by the patient. However, does not use levodopa. Discussion: The (CTX) is a rare
by clopido- grel for 21 days with statin association. At acute metabolic decompensation and rapid neurologi- autosomal recessive disease caused by mutations in the
follow-up, partial recovery was achieved after 3 months cal deterioration is usually not common. In patients with CYP27A1 gene, leading to absence of the mitochondrial
in physical therapy. Discussion: An ischemic stroke of L2HGA, worsening gait/balance, altered consciousness sterol 27-hydroxylase, responsible for cholesterol metab-
the aPe can cause stupor, agitation, change in behavior, and seizures, incipient headache may be indicative of olization in the bile synthesis pathway. Thus, there is
aphasia (dominant side), hemineglect (non-dominant brain tumors.Movement disorders are unusual, atetosis accumulation of cholesterol in tissues, including the
side), and diplopia due to the involvement of the mid- is related to loss of neurons and proliferation of astrocytes brain, leading to progressive neurological dysfunction
brain and interstitial nucleus of Cajal (INC), the largest in the caudate and putamen, as well as markedly reduced marked by dementia, epilepsy, hyperreflexia, spasticity,
nucleus of the medial longitudinal fasciculus (MLF). gamma-aminobutyric acid and glutamate decarboxylase movement disorders such as parkinsonism, dyskinesias,
The INC is the neural integrator for vertical eye move- activity. Dystonia and dyskinesia, on the other hand, occur and cerebellar ataxia. In other tissues it causes tendon
ment and is involved in vertical gaze, both with saccadic due to inhibition of neuronal glutamate decarboxylase by xanthomas, atherosclerosis and diarrhea. Cerebellar
generation and the vestibulo-ocular reflex (VOR). Two glutaric acid with decreased biosynthesis of gamma-ami- ataxia and tremor are justified by lesions in the dentate
different types of VOHS have been described in the liter- no-butyric acid. Among the neuroradiological findings, nuclei, extending into the surrounding white matter of
ature. The first: Consists of bilateral paralysis of the gaze cerebral and cerebellar atrophy as the disease progresses. the cerebellar hemispheres. Trunk-members dyskinesia
upwards and monocular paresis of the gaze downwards As for treatment, riboflavin to increase FAD levels and can be explained by neurodegenerative theory, neurotoxic
with injury ipsilateral or contralateral. Paretic look down carnitine as regulator of DNA damage. Final Comments: mechanisms, such as the production of lipid peroxidation,
to the left and injury to the right. This variant has been The authors describe a rare case of toxic accumulation of with excess cholesterol in CTX and free radical formation,
described in thalamomesencephalic lesions, exactly like L-2-hydroxyglutarate causing movement disorder: cervical leading to a higher rate of dopamine neuron loss due to
our patient. The second one: consists of difficulty to look dystonia in laterocollis and atetosis in the upper limbs, lipid accumulation in the substantia nigra. Furthermore,
down in association with monocular elevation paralysis, with impaired quality of life. CTX is a genetic disease, it can be associated with muta-
described in bilateral mesodiencephalic infarctions Final tions in the CYP2D6, the dopamine D2 and D3 receptor
Comments: This report describes a rare type of VOHS genes, and the serotonin 2A and 2C receptor genes that
and brings a new insight on a possible aPe topography Distúrbio Vestibulares e do Equilíbrio cause dyskinesia. Final Comments: CTX with dyskinetic
possibly causing this clinical presentation. and ataxic movements that are unusual in the presenta-
tion of this disease.

Distúrbio Vestibulares e do Equilíbrio


Distúrbio Vestibulares e do Equilíbrio

22
XXX Congresso Brasileiro de Neurologia

TL 1104805 TL 1104809 TL 1104833

UNUSUAL MICROBLEEDS CRITICAL ILLNESS CERVICAL PAIN AND HYPOGLOSSAL PALSY: MOYAMOYA DISEASE PRESENTING
BRAIN MRI OF COVID-19 PATIENT A CERVICAL ARTERIAL DISSECTION SIGN AS CEREBRAL HEMORRHAGE: CASE
PRESENTATION
Vanessa Maia Costa, Marco Aurelio Fraga Borges, Alberto Martins Pina Rodrigues Neto, Gustavo L de
Ana Helena Barbalho Bezerra de Oliveira Freitas Honorio, Carolina Rouanet, Marco Antonio Pedro Thiago Simões, Juliana Oliveira Almeida, João
Dantas Salles Lima, Caio Faria Tardin Vitor Nunes Sobreira Cruz, Allef Roberto Gomes
ING. Goiânia GO, Brazil
Bezerra, Kirsten Araújo Melo, Bruna Acioly Leão,
Hospital das Clínicas da Universidade Federal do
marcofbmed@me.com Alice Cavalcante Almeida Lins, Nayra Roberta Sales
Rio de Janeiro. Rio de Janeiro, RJ. Brazil
Salvador, Patricia Pereira Nunes, Simone Cássia
Case Presentation: Covid-19, initially described as a albertopina1994@gmail.com Silveira Silva
respiratory system’s infection, is currently more and
HGE. Maceió AL, Brazil
more recognized as a multiorganic disease, including Case Presentation: A 42 year old man came to the emer-
neurological manifestations. We are reporting the imag- gency room presenting a sudden neck pain, on the left pedro_thiago22@hotmail.com
ing findings of the rare entity of critical illness-associated side, that had started while he was jogging. Soon after,
cerebral microbleeds in a COVID-19 45 years old woman he felt spams and difficulty moving his tongue inside the Case Presentation: Female, 41 years old, complained
with hypoxic respiratory failure, who was eventually intu- mouth, he denied any Other symptoms. On the physical of sudden headache, vomiting and posterior neck pain
bated and ventilated, prolonged coma. She was tested examination there was no sign of lesion in the pirami- two days ago, evolving with impaired level of conscious-
positive for COVID-19 confirmed by reverse transcriptase dal tract. Soon after being admited, he underwent an ness and sphincter release. Past medical history: obe-
polymerase chain reaction. The purpose of this study is AngioCT of the head and neck, that showed a dissection sity and hypertension. Physical examination: somno-
to describe findings, in the form of cerebral microbleeds of the internal carotid artery, in its nasofaringeal portion, lent, responsive to commands, pupils equal round and
affecting different brain structures, observed in MRIs of where it has close contact with the 4th segment of the reactive to light, hemiplegia and hypoesthesia in the left
critically ill patients. Multiple scattered cerebral microhae- XII cranial nerve, compressing it. Although it is the most lower limb, Kernig and Lasègue’s signs positive. CT and
morrhages diffusely distributed in the juxtacortical white affected cranial nerve in these lesions, 5% of the cases, CT-angiography: newly subarachnoid hemorrhage in the
matter and internal capsule region, sparing the deep and this presentation in uncommon. For any patient with supra and infratentorial cisterns; hematoma in the right
periventricular white matter, basal ganglia, thalami and sudden neck pain, with associated XII nerve palsy it is thalamus and intern capsule filling all the four ventricles;
cortex were seen. Cerebral microbleeds and leukoen- imperative to investigate the possibility of carotid dissec- diffuse parietal irregularity of arteries from carotid sys-
cephalopathy have been described in COVID-19 patients; tion, considering a dissection in the vertebral arteries as tem, with multiples areas of stenosis in filiform flow in
although the mechanism remains unknown, possibilities a differential diagnosis. the distal segments of the middle and anterior cerebral
include endotheliitis with thrombotic microangiopathy, arteries. Cerebral angiography: moyamoya pattern in the
excessive inflammation, prolonged respiratory failure, carotid systems. Discussion: The evidences of the sub-
and hypoxemia. Doença Cerebrovascular arachnoid hemorrhage and distal arterial irregularities
from carotid system suggested vasculitis or vasospasm,
needing complementation with cerebral angiography that
Doença Cerebrovascular showed stenosis of the distal supraclinoid internal carotid
arteries with subocclusion/occlusion of the carotid ‘T’
and hypertrophy of the lenticulostriate arteries and of the
cisternal piercing branches compatible with ‘moyamoya’
pattern. Final Comments: Only 10 percent of the cases
of MoyaMoya Disease (MMD) presents as intracerebral
hemorrhage. The most often initial presenting is the
ischemic stroke. In patients with cerebral hemorrhage
the bleeding occurs in deep structures such as the basal
ganglia, thalamus and/or the ventricular system. Cortical/
subcortical bleeding is less frequent. The patient above
presented cerebrovascular syndrome and images with
a specific pattern compatible with the description from
literature for MMD.

Doença Cerebrovascular

23
XXX Congresso Brasileiro de Neurologia

TL 1105046 TL 1105065 TL 1105083

REVERSIBLE ISCHEMIC NEUROLOGICAL PRES OR NOT PRES?: ATYPICAL MRI OCULAR TILT REACTION AND VERTICAL GAZE
DEFICIT CAUSED BY S-PROTEIN DEFICIENCY LESIONS IN A MALE ADULT WITH PALSY DUE THALAMIC STROKE – A CASE
ASSOCIATED WITH THE PRESENCE HIPERTENSIVE ENCEPHALOPATHY PRESENTATION
OF PATENT FORAMEN OVALE: A CASE
Gustavo Maximiano Alves, Gabriel Rosa Vilela, Fernando Henrique Magri Alves, Gustavo Maximiano
PRESENTATION Frederico F A Alves, Maria Clara Zanon Zotin, Alves, Jorge Alberto Martins Pentiado Junior
Arthur Batista de Freitas, Moacir Pereira Leite Neto, Cristiano Milani, Millene Rodrigues Camilo, Octávio
Marques Pontes-Neto Hospital São Lucas. Ribeirão Preto SP, Brazil
Joyce Maria Leite e Silva, Isabel Monique Leite
Romualdo, Francisco Marcos Bezerra da Cunha Hospital das Clínicas da Faculdade de Medicina de gustavo.g.ma@hotmail.com
Universidade Federal do Cariri. Barbalha CE, Brazil Ribeirão Preto. Ribeirão Preto SP, Brazil
Case Presentation: A 70-year-old-man with uncontrolled
Universidade Regional do CaririIguatu CE, Brazil gustavo.g.ma@hotmail.com hypertension presents at emergency department with
Instituto Centro de Ensino Tecnológico. Campos sudden diplopia and dysarthria, initiated at home 10
Sales CE. Brazil Case Presentation: A 44-year old man with uncontrolled hours late. Neurologic exam showed an upbeat nystag-
arthur.freitas@aluno.ufca.edu.br hypertension presented to the emergency department mus, vertical diplopia, vertical supranuclear gaze palsy
with severely increased blood pressure and altered mental with normal vestibule-ocular reflex, normal horizontal
Case Presentation: K.R.G.R., a 36-year-old female patient status in the past two hours. At admission, he had acute gaze, mild dysarthria, left head tilt (Bielschowsky sign),
presented with sudden aphasia, dyslalia and acalculia. She hypertension (220/110 mmHg) and impaired kidney func- right eye with hypertropia, excyclotorsion of the left eye
had complete remission of these symptoms in about 10 tion. He was uncooperative, agitated and confused. Head wich was in hypotropia and a comitant skew deviation.
hours, but remained verbalizing with paraphasia for more CT without contrast showed only mild effacement of cor- There was no more neurological deficits. Fundoscopic
than a week, and then evolved completely asymptomatic. tical sulci, suggesting edema. A fundoscopic examination exam confirmed the contraversive ocular tilt reaction.
The adopted conduct was, in the first two weeks, treat- showed hypertensive retinopathy with retinal hemorrhage. A brain MRI study showed acute right paramedian-tha-
ment with acetylsalicylic acid 300mg a day, request for A presumptive diagnosis of hypertensive encephalopathy lamic infartation. The etiology was atheroembolism and
investigation tests (electrocardiogram, Holter, transtho- was made and high doses of intravenous nitroprusside patient received aspirin and statin. Discussion: Vertical
racic echocardiogram, transesophageal echocardiogram, were immediately initiated for blood pressure control. gaze palsy with normal vestibule-ocular reflex suggests
Doppler of the carotid and vertebral arteries, chest X-ray Cerebrospinal fluid and electroencephalography were supranuclear lesion and probably located at the mesen-
in PA and profile and cranial MRI) and ambulatory mon- completely normal. After five days, the blood pressure cephalic vertical gaze center. However, this topographic
itoring of blood pressure. Serum tests revealed a protein was well controlled and the patient recovered without any association cannot preserve cranial nerves and at the same
S deficiency and presence of hyperactivated factor VIII, neurologic deficits. Brain MRI performed one-week after time explain a comitant skew deviation, head tilt and the
possibly related to the use of oral contraceptives. The admission symmetric enlargment and increased signal excyclotorsion of the eye in hypotropia, causing a contra-
conclusion is that the patient had suffered a left parietal on T2-weighted images of the basal ganglia and base of versive ocular tilt reaction. There are graviceptives path-
cerebral ischemia, with remitted deficit, caused by an the pons without restricted diffusion. Contrast was not ways responsible for vestibular tonus (vestibular thalamus)
embolism originating from a state of transitory hypercoag- used due to chronic kidney disease. A control MRI after connected with the interstitial nucleus of Cajal, posterior
ulability and favoured by the presence of a patent foramen three-months showed complete resolution of the afore- commissure, interstitial nucleus of medial longitudinal
ovale(PFO), confirmed by echocardiography. The patient mentioned neuroimaging findings. Discussion: This fasciculus and Darkschewitsch nucleus (these structures
started oral anticoagulation with warfarin 5mg a day, and patient showed classical symptoms of posterior revers- are the vertical gaze center located at mesencephalon).
started using an IUD, no longer using oral contraceptives, ible encephalopathy syndrome (PRES), however, atypical These graviceptives pathways are located at thalamic
with normalization of protein S and factor VIII. Afterwards MRI findings led us to reconsider the initial diagnosis and nuclei of ventral posterolateral nucleus and probably
the PFO was surgically occluded, and she started taking contemplate differential. Typically MRI findings of PRES, were lesioned by the right paramedian stroke reported
acetylsalicylic acid 100mg a day. Discussion: Protein S is seen in almost 98% of cases, include symmetric and bilat- in this case. Final Comments: There are so many differ-
a cofactor for the cleavage of coagulation factors Va and eral hyperintense foci in the parieto-occipital subcortical ent clinical presentations of neurovascular syndromes
VIIIa and its deficiency predisposes to the formation of regions on FLAIR/T2-weighted images. The imaging find- of thalamus. The clinical and anatomical correlation is
venous or arterial thrombi, which when moving lead to ings in this case could be classified as a central variant difficult and neurologic exam is crucial to topographic
the possibility of ischemic strokes. When the phenome- PRES, in which lesions are predominantly observed in the diagnosis. This case presentation aims to get familiar to
non lasts beyond 24 hours with subsequent improvement basal ganglia and brainstem, sparing subcortical and cor- neurologists a rare presentation of a common disease,
it is said to be a reversible ischemic neurological deficit. tical regions. Differential diagnosis need to be considered for which rapid diagnostic and treatment are important
Another possible etiology for an ischemia is the persistence in this case and infarction, osmotic demyelination, auto- to better patient outcome.
of communication between the cardiac atria, especially immune diseases, infectious encephalitis and metabolic
when there is considerable size of this communication, encephalopathies were discarded because MRI pattern
with normal laboratory findings and clinical full recov- Doença Cerebrovascular
a large amount of shunt between the chambers and the
presence of an aneurysm or redundant membrane in the ery without deficits using only antihypertensives. Final
interatrial septum. Final Comments: The association Comments: PRES is a rare and dangerous condition in
of diverse etiological pathways may be understood as which rapid diagnosis and treatment are essential. The
being of importance, both for sharing the study, as well diagnosis of atypical PRES may be challenging then the
as for analysing the evolution of this patient profile and condition could be mistaken for other pathologies because
choosing the best conducts to be taken, since treatment the symptoms are nonspecific and the radiologic findings
ends up necessarily developing on two distinct fronts: are rare, especially when lesions are located in central or
anticoagulant drug therapy and surgical intervention. infratentorial regions.

Doença Cerebrovascular Doença Cerebrovascular

24
XXX Congresso Brasileiro de Neurologia

TL 1105115 TL 1105263 TL 1105294

SIMULTANEOUS BILATERAL HYPERTENSIVE INTRACRANIAL DURAL ARTERIOVENOUS OPALSKI’S SYNDROME AND COVID-19: CASE
THALAMIC HEMORRHAGIC – A VERY RARE FISTULA: CASE PRESENTATION PRESENTATION
PRESENTATION
Erick Broder Bichara, Victor Arthur Soares Costa Jacqueline Gomes da Silveira, Laura Helena Esteves
Nalcer Eduardo Canedo, Michel Ferreira Machado, Araújo, Daniel Abreu Santos, Trajano Aguiar Pires Poggianella Santana, Victor Hugo Castro Sá2, Jane
Pablo Nascimento Oliveira, Luiz Fernando Rodrigues Gonçalves, Caio César Molina Silva Lacerda Bahia, Dimitria Fortes de Oliveira Borges,
de Oliveira, Igor Vieira Trehoff, Pedro de Souza Alberlúcio Esquirio Pessoa, Rafael Pallos da Silveira,
EBMSP. Salvador BA, Brazil Samuel Filippe Motta Martins Dias, Pedro Igor
Domingues, Caroline Suemi Ogusuku, Flavio Vieira
Hospital das Clínicas da Faculdade de Medicina de Figueiredo Turíbio, Ismar Andrade da Silveira Neto
Marques Filho, Maria Sheila Guimaraes Rocha
Ribeirão Preto. Ribeirão Preto SP, Brazil
Hospital Santa Marcelina. São Paulo SP, Brazil Hospital São Joaquim/Unimed Franca. Franca SP, Hospital Metropolitano Odilon Behrens. Belo
Brazil Horizonte MG, Brazil
nalcereduardo@hotmail.com Faminas. Belo Horizonte MG, Brazil
victorarthur9@hotmail.com
Case Presentation: A 75-year-old woman, hyperten- jacquelinegomesneuro@gmail.com
sive, diabetic and smoker with irregular treatment, was Case Presentation: Male patient, 40 years old, admit-
found unconscious and with signs of sphincter release at ted with a headache for 1 week, progressive, weighty, Case Presentation: A 64-year-old male patient was admit-
home. She was last seen well at 10 am and was admitted holocranial, associated with bilateral pulsatile tinnitus, ted at the Emergency Department in January 2022 with
at Hospital at 11 pm. In the emergency room she had without suggestive ICH. He progressed to a generalized history of sudden onset severe headache followed by
blood pressure 224/127 mmHg, blood glucose 133 mg/ tonic-clonic seizure with sphincter release followed by vomiting, right side hemiparesis and ipsilateral sensory
dL, SpO2 98%, 3-points Glasgow Coma Scale, miotic loss of consciousness in July/2021. He was diagnosed loss. He didn’t receive thrombolytic therapy and showed
pupils. Her non-contrast computed tomography showed with SAH in the frontal and right temporal regions by progressively improvement of the symptoms. After two
bilateral thalamic hemorrhage. Cranial CT angiography CT and CTA, and failure to fill the sagittal venous sinus days, he developed sudden onset right hemiparesis, dys-
showed no vascular malformations and cranial mag- after contrast administration. He denied any comorbid- arthria, dysphagia and gait instability. The patient had a
netic resonance imaging showed age-matched bilateral ities or medication use. Referred to ICU with no return of background of hypertension and epilepsy. Furthermore,
hemorrhage, in addition to deep cerebral microbleeds, consciousness. A cranial CT was redone one week later the patient had symptoms of cough and fever three days
bilateral periventricular microangiopathy and ischemic revealing the resolution of SAH. Arteriography was indi- previously addition and was diagnosed with COVID-19
lesions with lacunar aspect of indeterminate date. She was cated to search for an aneurysm, however, due to lack of at the Hospital based on an antigen test. Neurological
managed conservatively and at the time of publication clinical control, the patient was not submitted. The patient examination showed right side facial weakness, ipsilat-
of this case she is still in a comatose state with a 7-point improved and was discharged. A hematologist diagnosed eral miosis and touch sensory loss. Right side arm and
Glasgow Coma Scale. Discussion: Hemorrhagic stroke thrombosis and indicated anticoagulant therapy (dabig- leg paresis was present associated with left side pain and
has an estimated prevalence of 12-15% of all strokes. The atran 150mg). After treatment, partial recanalization of temperature loss in trunk and limbs. He also had axial
most common etiologies include amyloid and hyperten- the superior sagittal sinus was presented by CTA with ataxia and gait instability. Characterizing a rare variant
sive angiopathy. Considering hypertensive angiopathy, thrombosis resolution and an improvement of headache, of the lateral medullary syndrome: Opalski`s syndrome.
the main sites of hemorrhage are the basal ganglia, thala- but with tinnitus maintenance. The differential diagno- MRI revealed an acute medullary infarction on the right
mus, pons and cerebellum. Hemorrhage in the thalamus sis was cerebral venous thrombosis or intracranial dural dorsolateral portion. Routine blood tests were normal,
represents less than 25% of hemorrhagic strokes and the arteriovenous fistula (IDAF). New arteriography revealed as were the cerebrospinal fluid, electrocardiogram and
simultaneous involvement of these structures is even the presence of IDAF in the torcula and left transverse/ echocardiogram. CT angiography revealed hypoplasia of
rarer. To date, there are less than 15 case presentations sigmoid venous sinuses, Cognard grade 1, with an absence the right vertebral artery. The patient was managed con-
worldwide of bilateral thalamic hemorrhage. The patho- of venous reflux; coupled with partial venous thrombo- servatively, received secondary prophylaxis with aspirin
physiology is still uncertain but is related to the rupture sis of the superior sagittal and left transverse/sigmoid and statin, physiotherapy and speech therapy at the stroke
of microaneurysms or perforating arteries chronically sinuses. Discussion: IDAFs represent 15% of intracranial unit. He was referred for outpatient follow-up. Discussion:
injured by uncontrolled hypertension. Final Comments: vascular malformations, frequently occurring between Opalski’s syndrome is a rare variant of Wallenberg’s syn-
Our patient had an atypical presentation of bilateral tha- 50-60 (age). They may present in a diverse symptomatic drome. It is usually caused by vertebral artery occlusion,
lamic hemorrhage. It is necessary to exclude important manner, ranging from less aggressive manifestations compromising blood flow to the lateral region of the
differential diagnoses such as cerebral venous thrombosis (e.g., headache and tinnitus) to more aggressive ones medulla, caudal to the pyramids decussation, leading to
with hemorrhagic infarction, vascular malformations and (e.g., progressive cognitive decline) determined by the involvement of corticospinal and spinothalamic tracts,
even ischemic stroke with hemorrhagic transformation venous drainage pattern and the location of the fistula. autonomic fibers and cranial nerves. The classical presen-
for the treatment be performed properly. Some clues that These fistulas mainly occur in the cavernous, transverse, tation is ipsilateral hemiplegia or hemiparesis, dysarthria,
may help in the diagnosis include the spectrum of small and sigmoid sinuses, and the cerebral tent. The main way dysphagia, ipsilateral nystagmus, vertigo, hypoesthesia in
vessel disease such as periventricular microangiopathy, of classifying IDAFs is according to Cognard types I and the ipsilateral hemiface and contralateral sensory loss in
deep cerebral microbleeds and previous ischemic lacu- IIa (benign) and IIb, IIa + b, III, IV and V (malignant). The trunk and limbs. It’s relevant to be familiar with the syn-
nar lesions. We hope that this case presentation can help benign ones drain directly into the venous sinuses, while drome as the myriad and bilateral distribution of symp-
other colleagues in the future who are faced with the same the malignant ones have direct or indirect reflux into the toms could lead to difficulties in topographic localization.
radiological images. cortical veins. CT, CTA, and MRI are the first-line diagnostic It’s also worth mention that the recent COVID infection
tools, but DSA has established itself as the gold standard and hypertension were risk factors for the stroke event.
for a suspected IDAF. Final Comments: The established Final Comments: Thus, in face of sudden ipsilateral
Doença Cerebrovascular relationship between IDAF with thrombosis and the pos- hemiparesis and cross-sensitivity alterations, this syn-
sibility of hemorrhagic transformation therefrom obscures drome should be considered and appropriate treatment
the cause-consequence relationship. should be promptly initiated, considering the impact on
survival and prevention of disabling deficits.

Doença Cerebrovascular
Doença Cerebrovascular

25
XXX Congresso Brasileiro de Neurologia

TL 1105338 TL 1105359 TL 1105362

ALICE IN WONDERLAND SYNDROME DUE TO EPILEPTIC SEIZURES AS STROKE MIMICS HYSTOPLASMA CAPSULATUM: AN UNUSUAL
CHRONIC CEREBRAL VENOUS THROMBOSIS AND CHAMELEONS: A CHALLENGE IN THE ETIOLOGY OF CENTRAL NERVOUS SYSTEM
– A RARE CONDITION EMERGENCY ROOM VASCULITIS
Bárbara Loiola Santos, Gustavo Maximiano Alves, Ana Paula de Góes Louly Bustamante, Caroline Eder Leandro da Silva Dantas, Juliana Naback
Gabriel Rosa Vilela, Fernando Henrique Magri Alves, Santos Eneas, Déborah Castro Ferreira de Oliveira, Toniolo, Gabriela Lopes de Morais, Giulia Maria
Frederico Fernandes Alessio Alves, Octavio Marques Eduardo Bernardo Chaves Neto, Isadora de Castro Ximenes Verdi, Natália Oliveira Silva, Tissiana
Pontes Neto, Millene Rodrigues Camilo Ferreira de Oliveira, Marta Rodrigues de Carvalho, Marques de Haes, Maria Clara Zanon Zotin, Antônio
Nayara da Costa Andrade, Pablo Henrique da Costa Carlos dos Santos, Octavio Marques Pontes-Neto
USP – Ribeirão Preto. Ribeirão Preto SP, Brazil Silva, Hiram Fernandes Soares
Hospital das Clínicas da Faculdade de Medicina de
barbaraloiolas@gmail.com Hospital de Base do Distrito Federal. Brasília DF, Ribeirão Preto. Ribeirão Preto SP, Brazil
Brazil
Case Presentation: A 44-year-old man was admitted to the ederleandro.sdantas@gmail.com
emergency department with sudden seizures and severe anapaulalouly@hotmail.com
headache for 3 days. He had a history of pulsatile tinnitus Case Presentation: A 47-year-old woman who had been
for 2 years. The patient reported intermittent symptoms of Case Presentation: A – J.C.R., a 58 year-old male, was investigating Systemic Lupus Erythematosus (SLE) was
visual illusion lasting about 10 seconds, characterized by admitted with sudden onset global aphasia, without no admitted to the Emergency Room with acute onset right
self-perceived distortions of his body and people’s faces, other neurological deficits. An unprecedented focal motor hemiparesis and hemihypoesthesia. Computed tomogra-
consistent with metamorphopsia. He also described onset seizure, with secondary generalization, was wit- phy scan showed a left thalamic hypodensity compatible
oscillation in the size of objects suggesting micropsia nessed at home. After admission to the emergency room, with a subacute ischemic stroke, along with a lacunar isch-
and macropsia. Physical examination showed proptosis he underwent a cranial tomography, which didn’t show emic lesion in the right internal capsule. Cerebrospinal
of the right eye. Fundoscopy showed no macular, vascular ischemic or hemorrhagic lesions. Intravenous thrombol- fluid (CSF) showed lymphocytic pleocytosis, consumed
and optic nerve changes. Cranial computed tomography ysis was performed. After 24 hours, a new tomography glucose and elevated protein levels. The etiologies consid-
and angiotomography were performed, which suggested still showed no signs of acute ischemic stroke. The patient ered were autoimmune and infectious disorders. Exams to
venous infarction and sinus thrombosis, respectively. The remained with isolated aphasia and no new seizures were rule out the second one were negative. A cranial magnetic
electroencephalogram indicated focal base epileptogen- witnessed. An electroencephalogram showed a periodic resonance imaging (MRI) showed more ischemic lesions
esis of the right occipital lobe due to a structural lesion. pattern between 1-1,5 Hertz compatible with continuum and diffuse arterial narrowing. The rheumatology team
Treatment with 1500mg/day of valproic acid and 20mg/ interictal-ictal. He completely recovered from his language considered active arthritis as well, so an autoimmune
day of clobazam was initiated with complete resolution deficit with antiseizure medication. B – T.M.B.S., a 39 mechanism was considered the most likely. Due to pro-
of metamorphopsia, including macropsia, and micropsia. year-old female, was admitted with a history of unprece- gression of lesions and the potential gravity of this condi-
Later, cerebral arteriography confirmed thrombosis of the dented tonic-clonic generalized seizure with subsequent tion, she was submitted to a course of methylprednisolone
superior sagittal sinus, transverse and sigmoid sinuses transient left hemiparesis, which lasted about 1 hour, and and cyclophosphamide. Her initial response was good
on the right. In addition, a tentorial dural arteriovenous completely remitted. Initial cranial tomography didn’t and she was discharged a few days later. After one week,
fistula nourished by posterior branches of the middle show any alterations and she was sent back to her origi- she started with fever, a novel right-sided oculomotor
meningeal artery and right occipital artery with drain- nal healthcare service for observation. She returned, 48 nerve paresis and mental confusion. Cranial MRI showed
age to cortical veins was visualized. Embolization of the hours later, with left hemiparesis and dysarthria. A new recent ischemic events on the midbrain, pons and right
arteriovenous fistula was performed, and apixaban 10 tomography showed a subacute ischemic lesion in the cerebellar hemisphere. CSF analysis evidentiated wors-
mg/day was started. The patient evolved with complete territory of the right middle cerebral artery. Discussion: ening of the previous findings with a high opening pres-
remission of headache and pulsatile tinnitus. Discussion: This poster reports two cases of relevance for the study sure. A new screening for infectious causes was positive
Metamorphopsia is a misperception of the size of objects of acute ischemic stroke and epilepsy. The presence of for histoplasmosis, with a counterimmunoelectrophore-
or body parts due to migraine, epilepsy, intoxication, unprecedented seizures with focal neurologic deficits sis for Hystoplasma capsulatum titer of 1: 16 on CSF and
or infectious cerebral diseases. They are referred to as may cause diagnostic doubt, as well as in the indication blood, with negative cultures. In view of the hypothesis
Lilliputian hallucinations and were described as Alice of thrombolytic therapy. In the first case, the symptom of of CNS histoplasmosis leading to vasculitis and ischemic
in Wonderland Syndrome (AIWS). In the present case, aphasia in a patient who was actually in non-convulsive strokes, antifungal treatment was started. She developed
the patient exhibited this syndrome due to focal epilep- status epilepticus mimicked an actue ischemic stroke. generalized seizures and progressive somnolence, with
togenesis secondary to a venous infarction caused by a The diagnosis of a “Stroke Mimic” was only made later, needing for orotracheal intubation. During her prolonged
chronic cerebral venous thrombosis. To our knowledge, with the result of the electroencephalogram. In the sec- Hospitalization, she had multiple infectious and clinical
there is only one case of AIWS related to cerebral throm- ond case, a young woman had a transient ischemic attack complications. Subsequent radiological studies and CSF
bosis reported in the literature. Final Comments: AIWS that was mistaken for Todd’s paralysis after a generalized analysis evidentiated poor improvement. Months later, she
is a rare condition caused by severe illness. Therefore, seizure. 48 hours later, her case was found to be a “Stroke was discharged with many neurological sequelae, with a
the early diagnosis is fundamental, and the treatment Chameleon”. Final Comments: Stroke mimics and chame- plan of prolonged oral antifungal treatment. Discussion:
also needs to be directed at underlying diseases. This leons represent a great challenge in every-day neurological CNS vasculitis is rarely caused by SLE. Infectious causes
case presentation aims to familiarize neurologists with practice. Approximately 5% of ischemic strokes present must be remembered due to their prevalence, especially
this uncommon condition. with seizures. Therefore, it is reasonable to proceed with in immunocompromised patients. Our patient had radio-
thrombolytic therapy, in theses cases, if evidence suggests logic findings previously described in CNS vasculitis
that residual impairment is secondary to stroke and not caused by Hystoplasma, as the main involvement of small
Doença Cerebrovascular postictal phenomenon. Current evidence shows that, when vessels of posterior circulation. However, sometimes it
in doubt about a stroke mimic, intravenous thrombolysis is necessary to ponder empirical immunosuppressive
should not be delayed, because the risk of symptomatic treatment because of clinical worsening and sequelae
intracranial hemorrhage, in these cases, is quite low. risks, like in our case.

Doença Cerebrovascular Doença Cerebrovascular

26
XXX Congresso Brasileiro de Neurologia

TL 1105414 TL 1105421 TL 1105450

POST-COVID CEREBRAL VENOUS MARANTIC ENDOCARDITIS LEADING TO LOBAR INTRAPARENCHYMATOUS


THROMBOSIS COMPLICATED TO DURAL STROKE IN AN ONCOLOGIC PATIENT ON FULL HEMORRHAGE SECONDARY TO AMYLOID
ARTERIOVENOUS FISTULA ASSOCIATED TO ANTICOAGULANT DOSE: WHATS LEFT TO ANGIOPATHY IN A YOUNG FEMALE: A CASE
AUDIBLE PULSATILE TINNITUS WITHOUT PREVENT NEW EVENTS? PRESENTATION
STETHOSCOPE
Pedro Vinicius Brito Alves, Victoria Veiga Ribeiro Lara Guimarães Queiroz Silva, Thiago Oscar Goulart,
Fabíola Gondim Medeiros Chaves, Daniel Ribeiro Gonçalves, Paula Baleeiro Rodrigues Silva, Paula José Rosemberg Costa Lima, Thire Baggio Machado
Chaves, Gisele Franco Castro, Daniel Oliveira Fiuza Rodrigues de Medeiros, Nathalia Watanabe, Marazzi, Rui Kleber Vale Martins Filho, Octávio
Almeida, Diego Eduardo Nunes, Francisco Mauricio Luiz Gustavo Brenneisen Santos, Isabela Fonseca Marques Pontes Neto, Millene Rodrigues Camilo,
Maia Neto, Vicente Paulo Afonso Abreu, Amanda Risso, Eduardo Genaro Mutarelli Francisco Antunes Dias, Frederico Fernandes
Souza Chaves Macedo, Clarice Lima Alessio Alves, Carlos Eduardo Massote Fontanini
Hospital Sírio Libanês. São Paulo SP, Brazil
Universidade Nilton Lins. Manaus AM. Brazil Hospital das Clínicas da Faculdade de Medicina de
pvbalves@gmail.com Ribeirão Preto. Ribeirão Preto SP, Brazil
Hospital Adventista de Manaus. Manaus AM, Brazil

danielrchavesam@gmail.com Case Presentation: We present the case of a 79 year-old laragqs@gmail.com


woman on treatment for a metastatic lung adenocarci-
Case Presentation: A.O.S., 36-years-old, attended on noma diagnosed upon bilateral pulmonary thromboem- Case Presentation: This case refers to a 44-years old
09/2020 with a complaint of intense left hemicranial bolism. She was started on apixaban 5mg BID and osim- female patient, who presented sudden paresis of the
headache for 01 week associated to nausea, dizziness ertinib. Six months later, she developed a severe stroke right inferior limb (RIL) associated with frontoparietal
and blurred vision. On 10/2020, after refractoriness to caused by an occlusive thrombus from right ICA to M1. lobar intraparenquimatous hemorrhage in the left cor-
sintomatic treatment, a neuroimage identified cerebral She was submitted to mechanical thrombectomy, leav- ticosubcortical region, and also hypodense areas in the
venous thrombosis (CVT) in the superior sagittal sinus ing a residual M1 occlusion, with partial recanalization. white matter. In the propaedeutic, it was performed Brain
and partial in the transverse and sigmoid sinuses, greater Transthoracic ecocardiogram showed mobile filamen- MRI, it was visualized multiple foci of microbleedings in
on the left. The pacient was treated with enoxaparin full tar wooden images adhered to the right coronary and the cerebral and cerebellar parenchymas, with alteration
anticoagulation and then converted to warfarin for 6 mitral valves, generating moderate reflux. Laboratory of white matter sign and recent hematoma in the high
months. An extensive laboratory etiological investigation workup showed a hemolytic microangiopathic anemia left convexity associated with perivascular spaces. With
was done but it´s normal. He had history of Covid-19 with marked increase in D-dimers levels. Blood cultures these findings in the imaging, compatible with small
infection two months ago. On 04/2021, an image control were negative. She was followed with enoxaparin 1mg/kg vessel disease (SVD), it was widened the investigation
was performed and showed chronic CVT in the middle BID, with no further vascular events. Discussion: One in to possible inflammatory etiologies (vasculitis, inflam-
and posterior thirds of the superior sagittal sinus, and in seven ischemic stroke patients can host occult cancers, matory amyloid angiopathy) with performing of lumbar
the left transverse and sigmoid sinuses. On 07/2021, the with up to 20% of these being discharged as Embolic puncture to liquor study and it was initiated pulsotherapy
case evolved to left pulsatile tinnitus that worsened when Stroke of Undetermined Source (ESUS). Marantic endo- with methylprednisolone for five days. It was not detected
lying down, audible on ipsilateral periauricular ausculta- carditis (ME) is a proposed mechanism leading to stroke any liquor alterations. There was partial recovery of the
tion and sometimes audible without a stethoscope aid. in oncologic patients. It is a rare condition and is strongly deficits and the patient was discharged with ambulatorial
A new cerebral angiography was performed on 09/2021 associated with lung adenocarcinoma. It usually dam- follow-up for etiology investigation. Nearly one year after
and revealed a dural arteriovenous fistula (dAVF) in the ages healthy valves, leading to the formation of high-risk the first event, she presented a new neurologic deficit
sigmoid and left transverse sinuses. It was embolized on embolic thrombi. Permanent anticoagulation is warranted with complete right hemiparesis and right hypoesthe-
11/2021, with tinnitus resolution. Discussion: dAVF can and surgical approaches are reserved for early disease in sia, right homonymous hemianopsia, and aphasia. Brain
be congenital, idiopathic or acquired. When acquired, younger patients. Secondary prophylaxis with new direct Tomography evidenced heterogenous lobar intraparen-
its main etiologies are traumatic brain injury, otomas- oral anticoagulants (DOACS) lacks substantial evidence, quimatous hemorrhage in the high left frontal convexity,
toiditis, CVT, previous craniotomy and pregnancy. It is with some studies citing edoxaban 60mg as a potential and also demonstrated multiple foci of microbleedings
a rare complication of CVT, evolving in only 1% of cases. player. Vitamine K antagonists (VKA) are more accessible, with lobar distribution, characterizing possible cerebral
It is classified into low and high grade. Low-grade ones but lack in patient safety due to interactions with chemo- amyloid angiopathy, by the Boston Criteria. Discussion:
(Borden I; Cognard I and IIa) are usually not indicated for therapy plus higher bleeding rates. Enoxaparin is the most Cerebral Amyloid Angiopathy is a relevant etiology of
surgery, except in cases of secondary glaucoma, refrac- recommended by specialists, targeting final pathways in Lobar Intraparenquimatous Hemorrhage (LIH) and char-
tory orbital pain, and pulsatile tinnitus. High-grade ones the coagulation cascade, but carries inconvenient logistics acterizes as deposits of beta-amyloid in small and medium
(Borden II and III; Cognard IIb, IIa+b, III, IV and V) must and administration. Since no absolute recommendation brain blood vessels, being strongly associated with age.
be treated early due to of bleeding high risk. This case on the topic has been proposed, choosing an anticoagula- Its prevalence is higher in patients older than 65 years
becomes particularly rare due to the high possibility of it tion strategy in these patients envolves social, economic old and, in its occurrence in young ones, the medic must
has being triggered by the post-covid prothrombotic state. and technical issues as well as patient education. Final hypothesize inflammatory and hereditary causes. Final
Final Comments: This is a case of CVT probably related Comments: Despite full dose anticoagulation, our patient Comments: The investigation of LIH in young patients
to a post-covid prothrombotic state, which evolved after still developed a major embolic event, highlighting the must be performed, as this is one of the most common
10 months with dAVF associated to intense pulsatile tin- interface between cancer inflammation and thrombosis. causes of cerebral venous thrombosis, arteriovenous
nitus, resolved after embolization. The case deserves to be Preventing vascular events in oncologic patients poses a malformations, and vasculitis. The occurrence of amyloid
highlighted due to the rarity of its evolution, as well as the true challenge, since their hyper coagulable state seems angiopathy in these patients must have the inflammatory
association with an unusual clinical sign of spontaneously to be multifactorial and dynamic. Future studies should and hereditary origins investigated and the institution of
audible pulsatile tinnitus, without a stethoscope aid. approach better stroke prevention strategies, keeping therapeutic measures, as well as the control of risk fac-
up with the current therapies that are improving cancer tors is of extreme importance to avoid the recurrence of
mortality and morbidity. bleeding, which could implicate in the loss of functionality
Doença Cerebrovascular and worsen the individual life quality.

Doença Cerebrovascular
Doença Cerebrovascular

27
XXX Congresso Brasileiro de Neurologia

TL 1105455 TL 1105461 TL 1105462

THE FUNCTIONAL IMPAIRMENT OF ACUTE OCCLUSION OF CENTRAL RETINAL BILATERAL SYMMETRICAL ISCHEMIC
GERSTMANN SYNDROME AFTER A ARTERY DIAGNOSED BY TRANSCRANIAL STROKE IN A YOUNG PATIENT: CASE
SUBACUTE STROKE: A CASE PRESENTATION DOPPLER (TCD): A CASE PRESENTATION PRESENTATION
Gabrielle Benevides Lima, Ana Raquel Jucá Parente, Thiago Oscar Goulart, Renato Ramon da Cruz, Ellen Gabriela Flor Nimer, Priscila dos Santos Mageste,
Norberto Anizio Ferreira Frota Silva de Carvalho, Lara Guimarães Queiroz Silva, Catarina Sodré de Castro Prado, Isadora Versiani
Octávio Marques Pontes Neto, Rui Kléber Vale Lemos, Viviane Alexandre Silva, Mariana Soares
Universidade de Fortaleza. Fortaleza CE, Brazil Martins Filho, Frederico Fernandes Aléssio Alves, de Freitas Tamy, Matheus Nunes Ferreirinha Leite
anarjparente@gmail.com José Rosemberg Costa Lima, Millene Rodrigues Castro, Raimundo Marcial Brito Neto, Márcia
Camilo, Carlos Eduardo Massoti Fontanini Cristina Antunes Ribas
Case Presentation: Female patient, 42 years old, incom- Hospital das Clínicas da Faculdade de Medicina de Hospital Universitário Antônio Pedro. Niteroi RJ,
plete elementary school, black, obese, with hypertension, Ribeirão Preto. Ribeirão Preto SP, Brazil Brazil
type 2 diabetes mellitus and depression. Family history of
cardiovascular disease, father with a history of 5 previous drthiagogoulart@gmail.com gfnmed1@gmail.com
ischemic events and deceased from acute myocardial
infarction. Followed up at the neurology outpatient clinic Case Presentation: 70-years old female, previously with Case Presentation: A 30-year-old woman with a past
due to sequelae of ischemic stroke. She presented the onset pre-diabetes, systemic arterial hypertension, and hypothy- record of resistant systemic arterial hypertension for 15
of focal symptoms with right hemiparesis, memory loss roidism. Presented acute amaurosis in the right eye, being years, diabetes mellitus type 1 with irregular treatment,
and dysarthria, however, she was managed as an anxiety evaluated by an ophthalmologist, who hypothesized retina heart failure, on therapeutic anticoagulation due to right
crisis in the emergency room. After 3 months, she pre- detachment. On the following day, she was admitted to deep vein thrombosis and seronegative antiphospho-
sented with a sudden severe headache, which was then our service, a tertiary Hospital with an Emergency room lipid antibody syndrome, presented to the emergency
referred to the tertiary center for imaging tests. Cranial and Neurology. In the admission, she presented NIHSS 0. department with a sudden onset of left hemiplegia, which
computed tomography showed a hypodense cortical Ophthalmoscopy demonstrated indirect evidence of cher- resolved spontaneously in less than an hour. A head CT
lesion in the frontal and left parietal lobes. Cranial mag- ry-red spot and applied retina. Brain Angiotomography was performed and displayed no acute alterations. The
netic resonance angiography showed signs of ischemia was performed, which was normal. Transcranial Doppler patient was discharged without being evaluated by the
in the left perirolacnic cortex. The two ischemic events (TCD) with transorbital window was performed, demon- Neurology team. Later that same day, she exhibited a
had an atheroembolic origin. The patient has a deficit in strating Right Central Retinal Artery (R-CRA) with a resid- decreased level of consciousness and bilateral tonic-clonic
spatial and semantic memory and impairment in instru- ual pattern, detecting only isolated systolic peaks, findings seizures, requiring orotracheal intubation and mechanical
mental activities of daily living. For functional rehabili- compatible with occlusion of the central retinal artery ventilation. This time a head CT angiography was ordered
tation, she was referred to our institution. On physical (OCRA). It was initiated with secondary prophylaxis with and revealed an occlusion of the left middle cerebral
examination, patients scored 18 points on the Mini Mental acetylsalicylic acid 100mg per day and simvastatin 40 mg artery and a subocclusion of the right middle cerebral
State Examination, with acalculia, agraphia, digital agno- per day. Exams were performed to investigate the stroke artery. A new head CT confirmed bilateral symmetrical
sia and right-left disorientation, characteristic findings etiology, resulting in the atherothrombotic etiology with hemisphere infarction. Discussion: This is a relevant case
of Gerstmann Syndrome. In the somatic examination, atheromatosis in the carotid bulbs bilaterally, with ste- since it depicts two rare events taking place simultane-
homonymous hemianopia on the right and paresis in nosis of nearly 50%. Discussion: The OCRA is a medical ously: a bilateral symmetrical hemisphere infarction and
the right lower limb grade IV were observed. Discussion: emergency that provokes sudden and painless amauro- the occurrence of an ischemic stroke in a young patient.
Gerstmann Syndrome is characterized by digital agnosia, sis and it must be treated as “Retinian stroke”. TCD with The incidence of stroke in young adults is low, however
right-left disorientation, dysgraphia and acalculia and its transorbital window allows the rapid and reliable detec- recent studies have shown an increase in the past years.
development can be caused by different etiologies, such tion of several signs which suggest its presence and even This rise can be attributed to traditional risk factors found
as neoplasms or stroke. The presence of the symptom tet- the OCRA mechanism, such as the absence of flow in the in ischemic strokes in older adults, such as hypertension,
rad of this condition is rare, so having all the associated CRA and the presence of hyperechoic material in the ret- dyslipidemia, diabetes mellitus and obesity, but also to
symptoms is not necessary for the diagnosis. It is notewor- robulbar circulation of the optic nerve. TCD can detect alcohol abuse, use of illicit drugs, thrombophilias and
thy the persistence of the patient’s functional disability, signs of OCRA, including in cases with unremarkable antiphospholipid antibody syndrome. This case illustrates
mainly related to cognitive functions, even after the work initial ophthalmologic evaluation. It has been evidence a catastrophic vascular event in a young woman with mul-
of the multidisciplinary team for her rehabilitation and high sensibility of this method, and due to this fact, it is tiple comorbidities that could lead both to thrombotic
motor improvement. Final Comments: Recognition of an important tool to diagnose OCRA and investigate the and to embolic events. It is important to acknowledge the
this syndrome is essential to understand which cognitive stroke etiology, which could guide the clinical treatment. occurrence of stroke in the younger population, since its
deficits are impacting the patient’s functionality and to Final Comments: TCD is a relevant diagnostic method dismissal could lead to tragic outcomes such as the one
design a better rehabilitation strategy. in the suspect cases of OCRA, which can have no alter- reported here. If the patient had received proper care
ations in the angiotomography and ophthalmoscopy. when she first presented to the emergency room, with a
The OCRA diagnosis is fundamental to establishing the probable transient ischemic attack, maybe the bilateral
Doença Cerebrovascular acute treatment and definition of secondary prophylaxis. hemisphere infarction could have been avoided. Final
This exam was incorporated into our service in cases and Comments: It’s of great importance to control risk factors
made it possible for the confection of a specific protocol in young adults, who are in productive and reproductive
to attend OCRA cases. age, in order to avoid ischemic events and their disabling
sequels that affect not only the patients but family and
society as well.
Doença Cerebrovascular

Doença Cerebrovascular

28
XXX Congresso Brasileiro de Neurologia

TL 1105477 TL 1105505 TL 1105511

DEEP CEREBRAL VENOUS THROMBOSIS REVERSIBLE POSTERIOR ENCEPHALOPATHY CEREBRAL VENOUS THROMBOSIS
MIMITING COVID-19-RELATED SYNDROME IN A YOUNG PATIENT SECONDARY TO MUTATION IN THE GENE
ENCEPHALITIS: DISCUSSION OF A CASE IN WITH PRIMARY MALIGNANT ARTERIAL PROTHROMBIN IN THE CONTEXT OF
PANDEMIC TIMES HYPERTENSION: CASE PRESENTATION VACCINATION AGAINST COVID-19: A CASE
PRESENTATION
Eduarda Basso Badalotti, Gabriel Angelo Garute Amanda Leticia Andre, Tatiane Lopes Alves de Jesus
Zenatti, Renato Endler Iachinski, Rafaela Luiza Arroyo, Arthur Coelho Moura Marinho, Nathalye Eduardo Mariano Carvalho Silva, Jhonata Gabriel
Altheia Griza, Luiza Orth, Marcia Bernardon, Lucas Fernanda Pedroso Dircksen, Marcelo Simplicio Moura Silva, Arthur Costa Junger, Fábio Pereira
Victoy Guimarães Zengo Carvalho, Lorena Fernandes Kronbauer, Vinicio da Silva Júnior, João Victor da Cunha Silva, Lays
Manella Pimentel, Igor Andrade Vasconcelos, Breno Saraiva Rodrigues Carvalho, Lorena da Silva Viana,
Centro Universitário da Fundacão Assis Gurgacz – William Correa dos Santos, Damacio Ramon Kaimen Luiz Felipe Bezerra de Sousa, Nínivi Daniely Farias
FAG. Cascavel PR. Brazil Maciel Santos, André Camacho Oliveira Araújo
Fundação Hospitalar São Lucas FHSL
Hospital Santa Casa de Londrina. Londrina PR, Universidade Federal do Maranhão. Imperatriz MA,
duda.badalotti@hotmail.com Brazil Brazil
Case Presentation: V.Z, 19 years old, female, ballet dancer. amanda_leeticia@hotmail.com jhonata.moura@discente.ufma.br
Diagnosed by RT-PCR with COVID-19 7 days before arriv-
ing at the Hospital, came up with a holocranial headache. Case Presentation: P.S.B, male, 29 years old, low visual Case Presentation: A 15-year-old woman attended the
One day after, she evolved with apathy and motor apha- acuity 3 months ago, hypertension requiring sodium medical office with a headache without improvement for
sia, not witnessed. At Hospital admission, the examina- nitroprusside in an infusion pump, altered level of con- weeks after 30 days of vaccination against coronavirus
tion revealed a bedridden patient, Glasgow Coma Scale sciousness, and convulsive crisis. Patient with no comor- with the Ad26.COV2.S vaccine. Denied using contracep-
of 8, with muscular strength overall reduced. Her risk bidities. In ophthalmological evaluation, there is evidence tives. Evolved with drowsiness, neck stiffness, abducens
factors for trombotic events were the use of combined of bilateral papilledema, increased vascular tortuosity, nerve paresis, low visual acuity, vomiting, ataxia and mild
oral contraceptive, current infection and recent vacci- bilateral microaneurysms and microhemorrhages, and asymmetric tetraparesis. Optical Coherence Tomography
nation against SARS-CoV-2. The patient was submited visual acuity of hand movement. Initially, skull tomogra- (OCT) showed bilateral papilledema. Cerebral Magnetic
to a Magnetic Nuclear Resonance (MRI) of the brain and phy (CT) presented diffusely hypodensity in white matter Resonance (CMR) and CSF examination without alter-
lumbar puncture for Cerebrospinal Fluid (CSF) collection. and hydrocephalus. In cerebrospinal fluid, presence of ations. Spinal manometry confirmed the Intracranial
The MRI showed a symmetrical T2/Flair hyperintensity protein increase, and alteration of renal function com- hypertension (ICH) (75cmH2O). A relief lumbar punc-
in both thalamus and basal ganglia. Also, sparse foci of patible with chronic kidney disease. This was followed by ture was performed and acetazolamide was started.
diffusion restriction in the supratentorial white matter. hypotheses of diseases of the white substance of inflam- Arteriography showed venous thrombosis of the sigmoid
In SWI weighting, were hypointense foci in the deep matory, infectious, neoplastic or vascular origin. In Cranial and left transverse sinus. The investigation of the alter-
venous structures. With administration of Gadolinium, Magnetic Resonance Imaging (MRI), hypersignal in FLAIR ation of Leiden V factor, antithrombin III, C and S Protein,
failure of filling of venous structures and of the straight in supra and infratentorial white matter, in optic nerves antiphospholipid antibodies was all normal. A positive
sinus were evidenced. The CSF did not show abnormal- and ventricular ectasia. Intracranial arterial angiography result for the prothrombin gene 20210 mutation in het-
ities. Also, D-dimer in blood was 562 ng/mL. Having without alterations. Brain biopsy was also performed, with erozygosity was obtained. Full anticoagulation therapy
Cerebral Venous Thrombosis (CVT) and Encephalitis as a normal result. Serological, infectious and rheumatolog- was performed with 5 mg warfarin (INR between 2 and
differential diagnoses, we started anticoagulation with ical tests negative, and normal abdominal MRI and renal 3) and enoxaparin bridge, associated with topiramate 25
enoxaparin (1mg/kg 12/12h) and pulsotherapy with arteriography. After satisfactory blood pressure control, mg/day. Patient remains in outpatient follow-up, with no
methylprednisolone (1g for 5 days). Ten days after admis- the patient presented complete improvement of mental signs of disease recurrence. Discussion: Cerebral venous
sion, she was discharged from Hospital walking, with confusion, absence of new convulsive crises and partial thrombosis (CVT) is defined as the presence of a thrombus
fluent speech and no sequelae, taking dabigatran 150mg improvement of visual deficit. On cranial MRI performed within a venous sinus, in a superficial or deep intracranial
12/12h. After thirty days of discharge, the MRI showed after one month of Hospitalization, there was a signifi- vein. CVT has a higher incidence in young women, with
an area of sequela gliosis in both thalami. The patient is cant reduction in the lesions. Given the diagnostic exclu- additional risk factors, such as pregnancy, puerperium and
clinically well and remains under outpatient follow-up, sions and patient evolution, the diagnosis of Reversible use of oral contraceptives². Headache is the most com-
without neurological sequelae. She has even returned to Posterior Encephalopathy Syndrome (PRES) was con- mon symptom, but focal neurologic deficits, convulsion
ballet practice. Discussion: COVID-19 is clearly related to firmed. Discussion: The PRES course with altered level episodes and encephalopathies are also evidenced. The
thromboembolic events and neurological manifestations, of consciousness, visual deficits, headache, convulsive most suitable study technique of venous thrombi is CMR
through several mechanisms. CVT is a cerebrovascular crises and focal deficits. There are divergences on the venography. The coagulogram may indicate the presence
disease that can eventually mimic encephalitis, with a pathophysiology, believing that the main point is endo- of conditions that help the pathogenesis of CVT and the
non-specific and progressive clinical scenery, mainly thelial damage with vascular extravasation, resulting in research of the thrombophilic status and mutations is
due to changes in the level of consciousness, as presented edema. It translates as hypodensity on CT and hypersignal helpful. Lumbar puncture is important to rule out men-
by the patient. The diagnosis was only concluded after a on cranial MRI, predominantly in occipital and parietal ingitis cases³. In addition, other conditions such as prior
venous Magnetic Resonance Angiography (MRA), per- lobes, but not exclusively. It may be secondary to severe vaccination for COVID-19 as adenovirus vector-based
formed as soon as the resources were available for this arterial hypertension, chronic kidney disease, sepsis, vaccines are related to thrombotic episodes, through a
purpose. Final Comments: The early approach to the and immunosuppression. In the differential diagnoses mechanism called Vaccine-Induced Thrombocytopenic
patient and the study of possible differential diagnoses we found infections, autoimmune diseases, vasculitis, Thrombosis (VITT)4. Final Comments: It is noteworthy
allowed the medical team to quickly start therapies with malignant diseases and basilar artery thrombosis. The that CVT is an uncommon and reversible condition if diag-
LMWH and pulsotherapy, which contributed to the good treatment does not control the primary etiology. Final nosed and reated properly. Its association with mutations
outcome. The cause of CVT was attributed to COVID-19, Comments: Atypical and/or infratentorial PRES is less in the 20210A gene of prothrombin is well established and
by the fact that the patient had an active infection, added common, but in the face of a classic clinical picture with in relation to vaccination it is important to recognize that
to the other risk factors. a typical triggering factor should be part of the differential the risk of CVT from COVID-19 exceeds that of vaccines.
diagnosis of white matter diseases.

Doença Cerebrovascular Doença Cerebrovascular


Doença Cerebrovascular

29
XXX Congresso Brasileiro de Neurologia

TL 1105552 TL 1105556 TL 1105574

VERTICAL AND HORIZONTAL ONE AND A ISCHEMIC STROKE AS AN INITIAL ANEURISMATIC SUBARACHNOID
HALF SYNDROME: A CURIOUS STROKE MANIFESTATION OF LYMPHOPROLIFERATIVE HEMORRHAGE IN SINGLENTON-MERTEN
PRESENTATION DISEASE: A CASE PRESENTATION OS SYNDROME: A MERE COINCIDENCE?
POLYCYTHEMIA VERA DIAGNOSIS
Mariana Floriano Luiza Piva, Heitor Nunes de Thiago Oscar Goulart, Thire Baggio Machado
Oliveira Sento-Sé Neto, Flávio Vieira Marques Rodrigo de Paiva Bezerra, Milena Fernandes Castro, Marazzi, Victor Augusto Zanesi Maciel, Lara
Filho, Mateus Pradebon Tolentino, Pedro de Souza Pedro Passos Guimaräes, Guilherme Seiti Orikasa Guimarães Queiroz Silva, José Rosemberg Costa
Domingues, Luisa Pacheco Avezum, Lorrane Lima Filho, Millene Rodrigues Camilo, Francisco
Campidelli Arthuzo, Júlian Letícia de Freitas, Pablo Conjunto Hospitalar do Mandaqui, São Paulo SP, Antunes Dias, Rui Kléber do Vale Martins Filho,
Nascimento Oliveira, Michel Ferreira Machado Brazil Otávio Costa Vincenzi, Octávio Marques Pontes-
Neto
Hospital Santa Marcelina. São Paulo SP, Brazil rodbzmd@gmail.com
Hospital das Clínicas da Faculdade de Medicina de
marianaflpiva@gmail.com Case Presentation: A 49-year-old female with uter- Ribeirão Preto. Ribeirão Preto SP, Brazil
ine myomatosis and controlled arterial hypertension.
Case Presentation: A 69-year-old female patient with a Admitted with right middle cerebral artery syndrome – drthiagogoulart@gmail.com
history of hypertension and heart failure was admitted NIHSS 13 and 45-minute therapeutic window; intravenous
with a sudden onset of right hemiparesis, dysarthria, and thrombolysis was contraindicated due to vaginal bleed- Case Presentation: A 24-year-old 12-week-pregnant
diplopia. On neurological examination, she had right ing. Past history of nonspecific symptoms of holocranial woman was admitted to the emergency room presenting
hemiparesis, sparing the face, dysarthria, appendicu- headache, dizziness, and mild asthenia.CT angiography hypertension, a thunderclap headache, neck stiffness, and
lar ataxia of the four limbs, cutaneous-plantar reflex in was not performed to assess the possibility of mechanical torpor. She denied trauma. She had a prior medical history
extension on the right, moderate bilateral ptosis more thrombectomy. Initial investigation: Electrocardiogram of dental and orthopedic anomalies, arthritis, short stat-
prominent on the left. Ocular movements examination in sinus rhythm, transthoracic echocardiogram without ure; aortic valvulopathy, and refractory systemic arterial
demonstrated bilateral upgaze palsy with limitation of any abnormalities of chambers, valves and walls, as well hypertension, in use of four classes of drugs. At the age
infraduction on the left, indicating vertical one and a as systolic and diastolic functions. Control brain CT con- of nineteen, she was diagnosed with Singleton-Merten
half syndrome. For horizontal ocular movements, only firmed Ischemic stroke (IS) showed right temporoparietal Syndrome (SMS) by a molecular test, which evidenced
the right eye could abduct. Also, there was bilateral con- cortico-subcortical hypodensity with CT angiography a heterozygous for a c.992C>T p.Thr331Arg variant in the
jugated leftward palsy, indicating horizontal one and a showing absence of stenoses or plaques in carotid and IFIH1 gene. Until this date, she had no evidence of neu-
half syndrome. Doll’s eye test (oculocephalic reflex) was vertebral arteries; internal carotid, anterior, middle and rologic involvement/disorders. Her cranial computed
absent and the eyes were unable to converge. Brain MRI posterior cerebral arteries, as well as vertebral and basilar tomography (CT) imaging showed modified Fisher IV
showed hypersignal in the midbrain and pons to the left arteries with preserved path, caliber and enhancement. Subarachnoid Hemorrhage (SAH), intraparenchymal
on T2-FLAIR, with water diffusion restriction, indicating a Dynamic assessment with transcranial doppler evidence hemorrhage, and calcification of bilateral basal ganglia.
recent ischemic event. A small right cerebellar lesion with means intracranial vessel velocities in the lower limits Arteriography showed an aneurysmal in the perimarginal
hypersignal in T2-FLAIR and water diffusion restriction of normality for the patient’s age and normal pulsatility artery. The aneurysm was successfully treated with coil
was also present. Discussion: The one-and-a-half hor- indices. In the laboratory investigation observed: 20.3% embolization. Discussion: SMS and Aicardi-Goutieres
izontal syndrome is a well-known and well-described hemoglobin and 59.1% hematocrit. Hematologist evalu- syndrome (AGS) are rare and multisystemic disorders
clinical entity, characterized by complete limitation of ation performed bone marrow biopsy with characteristic caused by a gain-of-function mutation in the IFIH1 gene.
ipsilateral horizontal eye movement and limitation of immunophenotyping of Polycythemia Vera (PV). She Classically, SMS presents involvement of ectoderm tissues,
adduction of the contralateral eye. It occurs due to injury underwent therapeutic phlebotomy with improvement of such as skin and teeth, cardiac involvement, and aortic cal-
to the pontine paramedian reticular formation or the sixth systemic symptoms, hematimetric indexes: Hemoglobin cification, but no neurological involvement was reported.
cranial nerve nucleus and the ipsilateral medial longitu- of 14.6 and Hematocrit of 42% in addition to NIHSS 10 On the other hand, AGS classically presents progressive
dinal fasciculus. The vertical one-and-a-half syndrome and modified Rankin of 2, outpatient with a Hematologist encephalopathy, spastic paraplegia, and calcification of
is a less frequent entity, characterized by complete lim- at discharge in addition to rehabilitation. Discussion: basal ganglia. The variant in the IFIH1 gene identified in
itation of vertical eye movement in one eye and limita- Hematological diseases are responsible for about 1% of IS. this patient was described previously in a family affected
tion of upward or downward vertical movement in the Despite being uncommon, IS can be the initial presenta- by both neurological involvement and features of SMS. An
contralateral eye. Among its causes, the most common tion of lymphoproliferative diseases, with its occurrence extensive literature review was performed and no report
are related to injury to supranuclear structures, such as as the first symptom in 15% or more of patients with this describing intracranial aneurysm was found even when
thalamic nuclei. However, other structures related to type of condition. Final Comments: Despite the fact that there is AGS overlapping occurrence. Final Comments:
the control of the vertical gaze may be involved, such many patients do not present common causes of IS even This case demonstrates SAH in a patient with probable
as reticular formation and pretectal area in the rostral in adults or elderly, we should always pay attention to overlapping syndromes (SMS and AGS). Because the dis-
midbrain, and rostral interstitial nucleus of the medial less frequent etiologies (non-atherosclerotic or non-car- eases are extremely rare, there is still a lack of evidence
longitudinal fasciculus, and interstitial nucleus of Cajal. diogenic) since the etiological definition of stroke is the on whether it is a fortuitous association or intracranial
The most frequent etiology related to both syndromes is starting point of secondary prophylaxis. and recurrence aneurysms are part of the syndrome spectrum.
cerebrovascular disease, followed by demyelinating dis- of brain damage. In this case, the etiological hypothesis
eases and encephalitis. Final Comments: The association was based on a basic laboratory test.
between the horizontal and vertical one and a half syn- Doença Cerebrovascular
dromes is rarely described in the literature. In addition to
changes in ocular movement, our patient also had other Doença Cerebrovascular
neurological findings that are justified by the topography
of the ischaemic lesions.

Doença Cerebrovascular

30
XXX Congresso Brasileiro de Neurologia

TL 1105615 TL 1105646 TL 1105657

CEREBRAL VENOUS THROMBOSIS RECURRENT ISCHEMIC STROKE DUE NON-DECUSSATION OF THE PYRAMIDAL
PRESENTING AS REFRACTORY STATUS TO INTRACRANIAL STENOSIS TREATED TRACT DISCOVERED INCIDENTALLY
EPILEPTICUS IN A PATIENT WITH WITH BALLOON-EXPANDABLE: A CASE IN AN ASYMPTOMATIC PATIENT WITH
COMPLEX ARTERIOVENOUS FISTULA AND PRESENTATION ARTERIOVENOUS MALFORMATION: CASE
PTEN MUTATION SPECTRUM – A CASE PRESENTATION
Thaís Takamura, Luiza Gonçalves Fraga, Bruno
PRESENTATION Camporeze, Camila Carneiro Ferreira, Ludmila Hermes Castro Araujo Junior, André Luiz Guimarães
Eduardo Mesquita Souza, Matheus Alves da Silva, Machado Lima, Vanessa Rizelio, Matheus Kahakura Queiroz, Rafael Andrade Palhares, Débora Ferreira
Rafael Bragança Rodrigues Matias, Barbara Maini Franco Pedro Mendes, Christiane Monteiro Siqueira Campos,
de Carvalho, Feres Eduardo Aparecido Chaddad Victor Hugo Rocha Marussi
Instituto de Neurologia de Curitiba. Curitiba PR,
Neto, Ricardo Souza Abicalaf, Christiane Monteiro Brazil Universidade de Fortaleza. Fortaleza CE, Brazil
de Siqueira Campos, Alex Machado Baeta
Hospital Beneficência Portuguesa de São Paulo,
luizaag.fraga@gmail.com
Beneficencia Portuguesa de São Paulo, São Paulo São Paulo SP, Brazil
SP, Brazil Universidade Nove de Julho, São Paulo SP, Brazil
Case Presentation: A 70 year old female with a history
matheus.alves.123@outlook.com of ischemic stroke one year ago in the territory of mid- hermescaj@edu.unifor.br
dle cerebral artery, currently in using of acetylsalicylic
Case Presentation: A 30 year old patient was admitted to acid and clopidogrel, presented with a new stroke in the Case Presentation: A 47-year-old woman with a 5-month
the Hospital with progressive consciousness impairment. same territory previously affected. Angiography showed headache history underwent neuroimage investigation
She had a previous history of a complex arteriovenous stenosis of 55% of the M1 segment of the right middle with a CT scan that suggested an AVM at the right parietal
fistula (AVFs), undergoing multiple embolizations. Four cerebral artery. Due to the recurrence of ischemic events lobe. She had a clinical past of hypothyroidism denying
hours after admission, she evolved with a refractory status despite the double antiplatelet therapy, an endovascular previous seizures or weakness. A neurological examina-
epilepticus, controlled using multiple drugs and with the treatment was chosen. The initial planning was a balloon tion on admission revealed no focal neurological deficit
need of mechanical ventilation. Her admissional mag- stent, but dur-ing the procedure, it was not possible to and the first brain magnetic resonance imaging (MRI)
netic resonance imaging showed AVF on transverse and place the stent, with only the balloon being per-formed, did not reveal any parenchymal lesions. The patient
sigmoid sinus, also involving the inferior portion of the with good opening of the vessel and evolutionary control underwent cerebral angiography, which revealed AVM
sagittal sinus associated with cerebral venous thrombosis exams with transcrani-al Doppler showing good reper- at the right angular gyrus and a 3-stage embolization
on the basal vein of Rosenthal and sphenoparietal sinus. fusion and even improvement of the structural pattern was indicated. In the postoperative of 3rd embolization
Her complex anatomy made the thrombus inaccessible of the wall of the affected vessel. The patient has evolved session, the patient developed a right motor deficit over
for mechanical thrombectomy. She was anticoagulated well, so far without new recur-rences of ischemic events, the course of a few days. Brain MRI revealed a perilesional
and submitted to four new embolizations, but the venous is still under medical treatment. Discussion: Intracranial cytotoxic edema with restricted-diffusion posteromedi-
hypertension persisted. Surgical venous drainage discon- stenosis (ICS) is associated with a higher risk of ischemic ally to the AVM in the right parietal lobe – ipsilateral to
nection was made, remaining in a minimal state of con- stroke. Although the SAMMPRIS trial clearly demonstrated patient’s motor deficit. A 3.0 T MRI Tractography showed
sciousness. To elucidate the genetic mechanism behind superiority of aggressive risk factor management plus anti- solely unilateral non-crossing corticospinal tract with
the clinical phenotype, an exoma was made, showing a platelet therapy over intracranial stenting, the question no decussation. Discussion: Abnormal pyramidal tract
variant with loss of function on PTEN. She had no family remains regarding opti-mal treatment of those patients decussation is a very rare event that can be seen in ecto-
history of neurological diseases. Discussion: AVFs are who develop recurrent stroke symptoms despite best dermal dysplasias. Non-decussation of the pyramidal tract
usually unique. However, the coexistence of refractory medical management. A post-hoc analysis of SAMMPRIS is observed in several conditions such as Dandy Walker,
and recidivant lesions that progress from the anterior cir- evaluated the impact of hemodynamic markers, such as Joubert, Kallman syndrome and Ichthyosis. There are only
culation to the posterior circulation, as our case showed, the presence of “water-shed” and impaired collateral flow a few case presentations of incidental findings in healthy
raise the question if there dormant channels between on baseline imaging, and rates of recurrent stroke in the patients. We describe a patient who exhibited hemipa-
dural arteries and sinuses, which open when the sinus medical arm. The analysis showed that patients with poor resis ipsilateral to an arteriovenous malformation (AVM)
is occluded and venous pressure is increased or that the collateral flow and baseline strokes, suggesting a hemo- complication after embolization and whose neuroimag-
venous hypertension may cause hypoxia and produc- dynamic rather than an embolic mechanism, are at most ing showed a lack of pyramidal tract decussation. Final
tion of angiogenic factors, that promoted endothelial risk for subsequent stroke with medical therapy alone. This Comments: Pathologies of the pyramidal tract are rare
growth and neoangiogenesis. In our patient, alteration finding may help identify patients who are more likely to and are frequently associated with genetic and congenital
on angiogenic factors like tissue plasminogen activator, benefit from endovascular therapy for severe intracranial disorders such as posterior fossa anomalies or congen-
may be responsible for the multiple dural fistulas, like on stenosis. The optimal way to determine which intracranial ital mirror movements. However, they can be present in
some patients with PTEN mutation. There are few case stenosis is “hemodynamically significant” remains to be healthy, asymptomatic individuals and observed when
presentations on literature that show the correlation of determined. Final Comments: Intracranial stenosis is an a lesion to motor areas causes ipsilateral motor deficits.
patients with Cowden syndrome genotype associated important cause of strokes and therefore needs treatment, Only a few cases have been described in neurologic
with arteriovenous fistulas without other clinical man- especially when symptomatic. Although the overwhelm- asymptomatic individuals. Our patient presented with
ifestations but, until now, many phenotypes are correl- ing majority of patients with ICS should be treated with right-sided hemiparesis ipsilateral to an ischemic lesion
acionated with PTEN mutations like Cowden syndrome best medical therapy, a subset of patients with recurrent after AVM embolization.
and Bannayan-Riley-Ruvalcaba syndrome. Some authors strokes require endovascular revascularization by angio-
suggest predisposition to thrombosis in these patients. plasty with or without stenting.
Final Comments: The FAVD are a subtype of AVFs. Our Doença Cerebrovascular
case displayed an atypical case of complex and large AVFs,
Doença Cerebrovascular
manifesting with refractory status epilepticus secondary to
a cerebral venous thrombosis that caused intense venous
hypertension. The clinical spectrum of PTEN mutations
is heterogeneous, and may involve patients without the
classical sintomatology.

Doença Cerebrovascular

31
XXX Congresso Brasileiro de Neurologia

TL 1105702 TL 1105703 TL 1105769

REVERSIBLE POSTERIOR CAROTID WEB: A RARE FINDING INVOLVED CAROTID WEB: A SUBDIAGNOSED ISCHEMIC
LEUKOENCEPHALOPATHY SYNDROME IN ISCHEMIC STROKE STROKE CAUSE IN YOUNG ADULTS
(PRES): CASE PRESENTATION
Paula Fiuza Rodrigues Medeiros, Pedro Vinícius Isadora Castro Ferreira Oliveira, Déborah Castro
André Teixeira Souza Castro, Ronaldo Maciel Dias, Alves Brito, Luiz Gustavo Brenneisen Santos, Ferreira Oliveira, Caroline Santos Eneas, Nayara
André Gustavo Fonseca Ferreira, Ernane Pires Nathalia Watanabe, Paula Baleeiro Silva, Victoria Costa Andrade, Ana Paula Góes Louly Bustamante,
Maciel, Wagner Afonso Teixeira, Rodrigo Torres Sena Veiga Ribeiro Gonçalves, Eduardo Genaro Mutarelli, Pablo Henrique Costa Silva, Eduardo Bernardo
Pita, Robercon Alves Carmo, Fernanda Sousa Lima, Tarso Adoni Chaves Neto, Felipe Borelli Del Guerra, André
Larissa Lucas Schloika, Carolina Flores Welker Gustavo Fonseca Ferreira, Hiram Fernandes Soares
Hospital Sírio Libanês. São Paulo SP, Brazil
Hospital de Base do Distrito Federal. Brasília DF, Universidade Federal do Piauí. Teresina PI, Brazil
paulafrmedeiros@gmail.com Hospital de Base do Distrito Federal. Brasília DF,
Brazil
Brazil
andretsscastro@gmail.com Case Presentation: A 70-year-old male with medical Escola Superior de Ciências da Saúde – ESCS.
history of arterial hypertension, type 2 diabetes, hyper- Brasília DF, Brazil
Case Presentation: A 37-year-old male patient was lipidemia and metastatic prostate cancer, presented with
brought to our Neurologic Emergency Department with acute onset right-side weakness and global aphasia which castro_isadora@hotmail.com
acute ataxic neuropathy, cortical blindness, vertigo, gaze had started 2 days before the admission in the Hospital
palsy, skew deviation, paresthesia associated with ton- facility. Patient did not receive IV alteplase. CT revealed an Case Presentation: 40-year old male was admitted in the
ic-clonic seizures and impaired consciousness. As a result acute infarct on the left temporoparietal lobe. Computed emergency room with a sudden onset left hemiparesis
of topographical manifestations, a neurovascular syn- tomography angiography (CTA) described a linear struc- and asymmetry of the smile 2.5 hours ago. Previously
drome in the vertebrobasilar arterial territory was hypoth- ture in the posterior portion of the carotid bulb on the hypertensive, alcoholic and ex-smoker. The primary neu-
esized, consequently undergoing an attempt at chemical left side, suggestive of carotid web, partial occlusion of rologic examination showed a right middle cerebral artery
reperfusion. However, after the resolution of the acute M1 segment of the left medial cerebral artery and in M2 (RMCA) syndrome with a NIHSS (National Institutes of
phase, the patient remained under amaurosis bilaterally, e M3 segments as well, without other signs of atheroma- Health Stroke Scale) of 14. A cranial computerized tomog-
and an etiological evaluation was initiated by requesting tosis or stenosis. No evidence of embolism was shown raphy (CT) showed a hyperdensity of the RMCA and an
MRI Neuroimaging and subsequently lumbar puncture on the further investigation. The patient received aspirin ASPECTS of 10. Intravenous thrombolytic therapy with
(LP). Radiological findings include confluent areas of T2 100mg and high-intensity statin therapy associated with Alteplase 0.9mg/kg was administered, since no contra-
hyper signal and posterior cortical lesions when assessed strict control of risk factors, without intervention on the indications were found, with a subsequent NIHSS of 11.
using the FLAIR modality. In addition to DWI sequence, AFMD. In short-term follow-up, patient maintained the Laboratory investigation, electrocardiogram and trans-
we can distinguish vasogenic edema in the brainstem deficits. Discussion: Carotid web is considered an atypical thoracic echocardiogram showed no relevant findings.
and cerebellum. Also, cortical laminar necrosis and the variant of fibromuscular dysplasia (AFMD) and presents An arterial angiotomography (AT) of the cervical vessels
corpus callosum are observed, suggesting the diagnosis itself as an abnormal shelf-like intra-luminal projection found a minor irregularity with a linear aspect in the pos-
of Reversible Posterior Leukoencephalopathy Syndrome of hyperplasia intima in the posterior wall of the proximal terior wall of the right carotid bulb, suggesting a carotid
(PRES). Promptly recognized, we initiated an etiological internal carotid artery, best visualized on CTA or carotid membrane, which was confirmed with an angiography.
investigation regarding possible associated comorbidities angiography. This finding has potential to cause blood An angioplasty with stent of the right internal carotid
with severe blood pressure and biochemical management. stagnation, thromboembolism and distal embolization, artery was opted as treatment. Discussion: The carotid
After 02 weeks, the patient reported regaining visual with ongrowing evidence of stroke association, specially web is a cause of Ischemic Stroke in young adults with no
capacity in low acuity, which he progressively recovered in younger patients, but also on hypercoagulable state, cardiovascular history and no positive investigation for
during Hospitalization. Thenceforth, he remains at our such as malignancy. Previous studies have shown that other cerebrovascular diseases. It is shown as a projection
service for adequate neurologic rehabilitation control. carotid web is found in up to 9.5% of patients <65 years of of linear aspect inside the bulbar portion of the carotid
Discussion: Reversible Posterior Leukoencephalopathy age with anterior circulation stroke of unknown cause. The artery, and is better visualized in the sagittal cuts of the
Syndrome (PRES) is a rare clinical and radiological entity incidence of AFMD in older patients with ischemic stroke CT. It is an acquired vasculopathy, pathologically defined
characterized by acute posterior topographical symp- is unknown. In patients with symptomatic carotid web, as an intimate fibromuscular dysplasia. The ischemic
toms and mostly reversible characteristics when prompt without other attributable causes of stroke, antiplatelet stroke pathogeny resides in the stagnation of flow and
recognition and treatment occur. The pathophysiology therapy is recommended to prevent recurrence. Carotid remote embolization of fibrin-based clots. It can cause
remains unclear, associating endothelial cell dysfunc- stenting or endarterectomy may be considered in refrac- recurrent hemispheric ischemic strokes. Although easily
tion and dysautonomic changes like arteriolar constric- tory cases, based on limited data. Nevertheless, optimal seen in ATs, the carotid web can be missed or misinter-
tion or dilatation, which may lead to a breakdown of the management (medical or interventional) is unknown. preted, as it doesn’t cause a significant hemodynamic
Blood-Brain Barrier (BBB). Also, as observed in MRI Final Comments: The carotid web is a proposed stroke stenosis and can mimic an arterial dissection, non-calci-
scans, intense vasoconstriction should evolve with local mechanism particularly in younger patients without fied arteriosclerotic plaque or an intraluminal thrombus.
hypoperfusion, cytotoxic edema, and cerebral infarction. vascular risk factors. However, it may have an important The diagnostic golden standard is the angiography, with a
The prevention and treatment evolve control of arterial roll in older patients. Future studies involving diagnosis non-invasive option being the AT of the neck. Treatment
hypertension, reduction of immunosuppressive drugs, and and secondary prevention of symptomatic AFMD should options include antiplatelet and anticoagulant therapies,
electrolyte disturbances management. Final Comments: focus on all age spectrum and on the association with both with a high recurrency rate (40-50%). Both carotid
This presentation describes a patient with PRES who fully other risk factors. endarterectomy and angioplasty with stent in the internal
recovered from his respective Focal Neurologic Deficits carotid are definitive treatments. Final Comments: The
(FND), such as cortical blindness. Most cases in this therapeutic decision making in the secondary prevention
syndrome result from case presentation and case series, Doença Cerebrovascular of the ischemic stroke depends on the cause of said stroke,
requiring multicenter trials to define a reliable conclusion. being the vascular study a primary one in the diagnostic
investigation. The carotid web is an undiagnosed and sub
treated entity, making it an important point of discussion
Doença Cerebrovascular among neurological cases.

Doença Cerebrovascular

32
XXX Congresso Brasileiro de Neurologia

TL 1105773 TL 1105807 TL 1105810

AN UNSUAL PRESENTATION OF THORACIC THROMBOLYSIS PERFORMANCE IN A EIGHT AND A HALF SYNDROME: CASE
SPINAL CORD ATROPHY DUE TO SUSAC BRAZILIAN PUBLIC SERVICE PRESENTATION
SYNDROME
Jane Lacerda Bahia, Dimitria Fortes de Oliveira Luíza Alves Monteiro Torreão Villarim, Matheus
Vitor Maia Arca, Ana Rosa Santana, Anne Elizabeth Borges, Ana Luisa Carvalho Santiago, Rafael Pallos Gurgel Saraiva, Rafael de Souza Andrade, Maria
Ferraz de Andrada, Daniel Alves de Oliveira, da Silveira, Jaqueline Gomes da Silveira, Laura Yvone Carlos Formiga de Queiroz, Paulo Antônio
Arthur Cesário de Holanda, Geovane Gomes Silva, Helena Esteves Poggianella Santana, Marilia Farias de Lucena, Mylena Gaudêncio Bezerra,
Raphaelly Ribeiro Campos, Eduardo Sousa de Melo Fontenelle e Silva, Raisa Cristina Teodoro e Silva, Jeanina Cabral Dionizio, Arthur Felipe Barbosa
Joana Luiza Rojo, Alberlucio Esquirio Pessoa Vasconcelos, Francisco Anderson de Sá Carvalho,
Hospital das Clínicas da Universidade Federal de Thiago Medeiros Palmeira de Araújo
Pernambuco. Recife PE, Brazil Hospital Metropolitano Odilon Behrens. Belo
Fundação Altino Ventura FAV Horizonte MG, Brazil Hospital Metropolitano Dom José Maria Pires. João
Pessoa PB, Brazil
vitorm_arca@hotmail.com janelbfarm@gmail.com
luizavillarim@hotmail.com
Case Presentation: A 35-year-old man presented with Background: Stroke is the second most common cause
3 years history of progressive crural paraparesis and uri- of morbimortality worldwide and one of the most import- Case Presentation: Female patient, 52 years old, hyper-
nary incontinence. He had a previous Hospitalization for ant causes of death in Brazil. The ischemic stroke is the tensive, was admitted to the service with a complaint of
cognitive symptoms, headache and seizure. Was observed most common type and its treatment in the acute phase blurred vision and difficulty closing her left eye suddenly
in MRI increased T2 signal in the corpus callosum and aims to restore the cerebral blood supply. This treatment 24 hours ago. She denied other symptoms or previous
lumbar puncture showed an increase protein ( 86 mg/ can be performed by intravenous thrombolysis with episodes. On examination, she had medial rectus muscle
dL) and White cells (9 with 88% neutrophils). Because alteplase and/or mechanical thrombectomy, the latter paresis in the right eye and lateral and medial rectus mus-
of these findings, at this moment, the main hypothesis not being available in most of brazilian public Hospitals. cle paresis in the left eye, facial paralysis of a peripheral
was multiple sclerosis (MS). A treatment for presumed Thrombolysis is currently one of the first line treatments pattern on the left, without further changes. The follow-
primary demyelinantion disease with methylprednis- in patients with acute ischemic stroke within 4,5 hours of ing exams were performed: cranial tomography without
olone was performed with improvement of the mental symptom onset. Available data shows functional improve- alterations, skull magnetic resonance imaging (MRI) with
confusion. Three years later was performed another MRI, ment of patients 3 to 6 months after treatment. The most recent lacunar ischemia in the left pons, normal cranial
showing increased T2/FLAIR signal in the central corpus dreaded complication of thrombolytic therapy is symp- magnetic resonance angiography, neck magnetic reso-
callosus, assuming snowball appearance. At this time he tomatic intracerebral hemorrhage which is associated nance angiography with tapering and parietal irregular-
reported urinary incontinence, erectile dysfunction, sen- with risk factors such as high blood pressure, diabetes ities in the distal portion of the foraminal segment (V2)
sory symptoms and weakness. Physical exam showed a mellitus, advanced age, microangiopathy, atrial fibrilla- of the left vertebral artery at the level of the axis tooth,
crural paraparesis and a spastic gait. Moreover he had an tion and stroke severity. Objective: Reveal data about the without defining dissection areas to the method, normal
ophtalmological examination showing areas of ischaemia benefits and possible complications of the patients treated echocardiogram, normal 24-hour Holter. Dual antiplatelet
demonstrating branch retinal artery occlusion and the with IV thrombolysis in a real life scenario in a brazilian therapy, statin and blood pressure control were started.
MRI images had involvement of central corpus callosum public Hospital, in comparison with the data in the liter- Discussion: Eight-and-a-half syndrome (EHS) is rare, with
and deep grey matter lesions in the basal ganglia. The ature, focusing on hemorrhagic complications. Method: few cases described in the world literature. Its early recog-
image of spinal cord showed a thoracic atrophy with no A prospective observational study was conduted between nition is of great practical importance, as it has varied and
sign enhance and cerebrospinal fluid analysis was nor- November/2019 and April/2020. The sample was calcu- potentially serious etiologies, with different therapeutic
mal. Under these circumstances another acute treatment lated to compare outcomes with the studied population implications. It is characterized by conjugated horizon-
was not performed. The presumptive diagnosis of Susac and the data of the available RCTs. The study included 53 tal gaze palsy, ipsilateral internuclear ophthalmoplegia,
Syndrome was made. Discussion: Susac`s syndrome is sequential patients diagnosed with acute stroke treated and peripheral facial palsy without impairment of taste,
a rare autoimmune disease that affects the microvascu- with thrombolytic therapy. Results: Most of the patients as taste fibers for the anterior 2/3 of the tongue travel
lature. It’s an endoteliopathy that can be manifest with were female and the mean age was 70 years old. The through the intermediate nerve, which has its nucleus
neurological, visual or auditive symptoms. (1) The clas- symptomatic hemorrhagic transformation rate was slightly in the medulla. The syndrome occurs due to a lesion that
sic clinical presentation was described as the triad of higher in our study, as was the rate of worsened NIHSS affects the medial longitudinal fasciculus, the parame-
encephalopathy, sensorineural hearing loss and branch after 24h of treatment, compared with data from the RCTs dian pontine reticular formation and the fasciculus of
retinal artery occlusion. (2) Our patient had bilateral and ECASS II and ECASS III. Moreover, 24,5% of the patients the facial nerve on the same side at the level of the pons.
multiple retinal ischaemia, but no involviment of the optic had other complications such as angioedema, hyperten- The diagnosis is easily missed as it needs a detailed eye
nerve. Although his spinal cord image showed an thoracic sion and seizures and 15,09% of the patients died. Final movement examination. It is mainly caused by stroke,
atrophy, spinal cord involvement was extremely rare in Comments: It was observed that the population in our multiple sclerosis, gliomas, metastases and vascular mal-
Susac’s syndrome. (4) Final Comments: Susac syndrome study was older and had at baseline more comorbidities, formations. Less frequent etiologies include iatrogenesis
is a rare disease and the differential diagnosis includes which are probably the features associated with higher related to tumor resection, neurocysticercosis, systemic
others autoimmune diseases, infectious diseases and odds of complications. In addition, despite the higher lupus erythematosus and myasthenia gravis if isolated
paraneoplastic syndromes. The ocular and vestibule-co- rates of hemorrhagic transformation, the patients in ocular involvement. Final Comments: The clinical rec-
chlear involvement, along with neurological symptoms the study had an improvement in the functional status ognition of EHS is essential for an accurate topographical
were cardinal features of the syndrome, but it could not similar to the comparative studies. All in all, although in diagnosis of pontine tegmentum lesions. Vascular disease
be seen at the same time. Presentation with spinal cord real life settings the rate of complications appears to be and multiple sclerosis are the main differential diagno-
involvement is rare, but have been described and it’s higher than that of RCTs, thrombolysis still leads to better ses, although rarer causes should always be considered.
important to be known or could lead to a misdiagnosed outcomes, and therefore should be performed following MRI is the exam of choice for topographic and etiologic
with other demyelination disease. proper protocols. diagnosis. Treatment depends on the cause.

Doença Cerebrovascular Doença Cerebrovascular Doença Cerebrovascular

33
XXX Congresso Brasileiro de Neurologia

TL 1105836 TL 1105876 TL 1105881

HIV ASSOCIATED MOYAMOYA SYNDROME MOYAMOYA’S SYNDROME RELATED TO HEMOPERICARDIUM: AN UNCOMMON


MANIFESTATION AS AN ACUTE PULMONARY TUBERCULOSIS – CASE COMPLICATION OF THROMBOLYSIS IN
HEMICHOREA PRESENTATION STROKE
Victor Augusto Zanesi Maciel, Anna Letícia de Rodrigo de Paiva Bezerra, Rodrigo Aragão Andrade, Pedro Miguel Mendonça Couto, Breno Gabriel
Moraes Alves, Renan Barros Lopes, Alison Mangolin, Abner Carnizello Souza Rodrigues Queiroz, Luiza Lima Beretta, Wender
Larissa Peres Delgado, José Rosemberg Costa Lima Rodrigues Teodoro, Leticia Silva Alves, Arthur Costa
Filho, Natalia de Oliveira Silva, Roberto Satler Cetlin, Conjunto Hospitalar do Mandaqui, São Paulo SP, Nascimento, Stefano Machado
Tissiana Marques de Haes, Fabíola Dach Brazil
Hospital de Clínicas da Universidade Federal de
Hospital das Clínicas da Faculdade de Medicina de rodbzmd@gmail.com Uberlândia. Uberlândia MG, Brazil
Ribeirão Preto. Ribeirão Preto SP, Brazil
Case Presentation: Female, 19 years old, admitted to the peps_couto@hotmail.com
victor93@gmail.com ER with acute desaturation, and a one month’s history of
fever and cough, in use of rivaroxaban for a 3 weeks diag- Case Presentation: Male 72-year-old patient, with hyper-
Case Presentation: A 24 year-old presented to our nosis of pulmonary embolism; has shown worsening for a tension and a previous hearing loss, started with sudden
Hospital clinic complaining about spontaneous move- week. Neurology has been called to evaluate for an acute confusion and aphasia. He was sequentially refered to the
ments in the left side of her body which began approxi- confusional state, lower limb weakness and respiratory tertiary service due to a condition compatible with stroke,
mately 6 months earlier. Her medical history was notable symptoms. Previously higid, the patient had domicili- presenting a 9 NIHSS on admission. After a brain CT
for congenital HIV infection (undetectable viral load and ary Pulmonary Tuberculosis contact. To the neurologic scan without any evidence of hipodensity and ASPECTS
CD4 count of 506), well controlled epilepsy and migraine. examination: sleepness, lentified, disoriented in time and of 10, thrombolysis was performed. The procedure was
She has been on antiretroviral therapy (ART), amitripty- space, no language impairment, mild dysarthria, muscle uneventful and there was a complete improvement of the
line, valproate and low dose chlorpromazine. She denied grade 1 on left and 3 on the right, and heightened deep deficit. After 2 days of Hospitalization, while waiting the
any other neurological or psychiatric symptoms, use of reflexes. Previous Hospital admission review: Tomography etiologic investigations, the patient started with tachycar-
other drugs or seizures recurrence in the past ten years. showed ischemic areas in the vascular frontier territory, dia (110 bpm) and respiratory distress requiring oxygen
Her level of consciousness was normal. There were ran- the new CT has shown new hypoattenuating cortico-sub- supplementation. A transthoracic echocardiogram was
dom involuntary movements in the left side of her face, cortical areas in the frontal and parietal high convexities performed, which showed significant pericardial effu-
arm and leg in a choreoathetoid phenomenology and of both hemispheres, determining amplitude reduction sion with mild hemodynamic repercussion with right
incorporated to voluntary movements. Distraction had no of some adjacent liquor spaces. AngioCT has shown no atrial collapse during systole. The Cardiac Surgery’s team
influence on the pattern of her movements. Her left limbs thrombus, but an unspecified narrowing in the internal quickly attempted an ultrasound-guided subxiphoid
were hypotonic. CSF profile, bacterial and fungi cultures, carotid. Cerebrospinal Fluid: protein elevated (62mg/ drainage, however, it was unsuccessful. Progressively
erythrocyte sedimentation rate, thyroid function tests, mL), undetectable Mycobacterium tuberculosis PCR. the patient stars with sings of cardiac tamponade, jugu-
glycosylated hemoglobin A1c were unremarkable. Brain Angiography: occlusions and “smoke” aspect collateral lar swelling, drowsiness and hypotension (85x55mmHg),
magnetic resonance imaging (MRI) showed no acute or circulation, suggesting Moyamoya’s disease. Laboratory and urgent surgical approach was performed with peri-
chronic stroke lesions, nor evidence of neuroinfection. The investigation for thrombophilias, falciform anemia and cardial drainage of 400mL of serohematic fluid. After
time-of-flight angiogram was notable for absence of flow other infections has turned negative. One month of treat- the procedure, the patient evolved with hemodynamic
in both middle cerebral arteries, suggesting Moyamoya ment, no cough or dyspnea, with progressive improvement stability, and underwent a new echocardiogram, with
syndrome (MMS). Arterial angiography located the occlu- of the neurologic symptoms. In 60 days, she was evaluated complete improvement of the pericardial effusion. The
sion on both supraclinoid segments of internal carotid in the ambulatory, alert, oriented and muscular strength basic investigation was unremarkable and not show an
arteries with blood flow redirected to a wide collateral grade 4 to the left, still in rehab. The investigation still awaits etiological cause and the patient was discharged for out-
circulation and posterior communicating arteries, con- anticoagulation cessation for complete neurovascular patient follow-up. Discussion: Spontaneous pericardial
firming MMS pattern. Reviewing older exams a contrast CT evaluation. Discussion: Moyamoya in the terminology effusion is a rare complication of thrombolysis in stroke.
scan done 4 years before had no abnormalities, including that refer to the progressive narrowing of a big intracra- It is more common when thrombolysis is performed in
arterial circulation. We found no other etiology to justify nial artery in parallel to multiple collaterals that confer acute myocardial infarction, in which the patients probably
her chorea besides the MMS. The patient received daily the “smoke” to the angiography. It’s called Moyamoya’s had as a pre-existence cardiomyopathy or pericardiopa-
risperidone 2mg with complete resolution of the symp- disease when it has genetic association, and Moyamoya’s thy. Mortality is usually very high, due to rapid evolution
toms. Discussion: MMS is a rare cause of chorea with a Syndrome when related to other clinical entities. A few to cardiac tamponade, but in this case the patient did not
frequency of 3 to 6%, as well incidence of MMD in HIV case presentation relate the syndrome to standard resis- have an immediate complication. However, any sings of
patients. The dilated collateral vessels compress the basal tant tuberculosis treatmen and Neurotuberculosis, cases cardiovascular complications should promptly undergo a
ganglia or can cause local ischemia, which can lead to a with long infection periods, necessary to the disease’s cardiac investigation. Final Comments: As the diagnosis
disruption of normal striatal activity. HIV infection has progress and collateral circulation development. Final is echocardiographic, in any case of hypotension in the
been reported as one of many conditions implicated in Comments: Despite the findings of Moyamoya Syndrome first hours or days of Hospitalization, with no other pos-
the etiopathogenesis of MMS. HIV can cause endothelial and its association with tuberculosis being described in sible clinical causes, an urgent echocardiogram should
damage and also induce proinflammatory states that later few cases in the literature, it is possible that the chronic be performed. And the existence of a cardiac surgery
manifest as stenosis or dilation of the vessel wall that can infectious condition plays a role in the pathophysiology team at the Hospital to perform pericardiocentesis or
occur despite effective ART and stable CD4 counts. Final of the neurovascular condition. pericardiostomy could be what would save the patient.
Comments: Since HIV infection has a well recognised
relation to cerebrovascular disease as well as to move-
ment disorders, we believe that it was a major factor to the Doença Cerebrovascular Doença Cerebrovascular
development of this secondary form of chorea.

Doença Cerebrovascular

34
XXX Congresso Brasileiro de Neurologia

TL 1105905 TL 1105908 TL 1106019

STROKE DUE TO SPONTANEOUS COENZYME Q10 DEFICIENCY MULTIPLE ISCHEMIC STROKES DUE TO
SUBCLAVIAN AND VERTEBRAL ARTERIES LEUKOENCEPHALOPATHY – CASE ADULT-ONSET STILL’S DISEASE: CASE
DISSECTION PRESENTATION PRESENTATION
Arthur da Veiga Kalil Coelho, Beatriz Marques dos Pedro Thiago Simoes Ferreira, Allef Roberto João Paulo Barile, Andreas Batista Schelp,
Santos, Ivan Vilchez Santillán, Andreas Batista Gomes Bezerra, João Vitor Nunes Sobreira Cruz, Fernanda Maria Gonçalves de Sousa Moura, Thiago
Schelp, Marcele Schettini de Almeida, André Luiz Kirsten Araújo Melo, Juliana Oliveira Almeida, Alice Ivan Vilchez Santillan, Andre Lopez Fernandez,
de Rezende Cavalcante Almeida Lins, Bruna Acioly Leão, Nayra Andre Luiz Rezende
Roberta Sales Salvador, Livia leite Goes Gitaí,
Hospital do Servidor Público Estadual de São Paulo. Patrícia Pereira Nunes Hospital do Servidor Público Estadual de São Paulo.
São Paulo SP, Brazil São Paulo SP, Brazil
Hospital Geral do Estado de Alagoas. Maceió AL,
arthurvkc@gmail.com Brazil barile.jpaulo@gmail.com

Case Presentation: A 39-year-old woman suddenly pre- pedro_thiago22@hotmail.com Case Presentation: A 44 year old Woman reports that in
sented rotational vertigo, loss of balance with fall from februrary os 2022 presented sudden change of visual field
standing height, without head trauma, and intense left Case Presentation: Female patient, 42 years old, history with a homonymous right hemianopia. She had previous
temporoparietal pulsatile headache. She used simple of gait incoordination for 03 years and progressive wors- diagnosis of schizophrenia, a stroke in 2019, and Adult-
analgesics and didn’t search for medical service at that ening, in addition to nocturnal enuresis and clonus in onset still’s disease (AOSD) diagnosed in 2020. In the
moment. On the next day, due to the continuation of the the lower limbs, requiring support to walk. There were Hospital admission, the CT scan revealed a recent onset
symptoms, she looked for medical service. She didn’t have no reports of vertigo, diplopia, changes in motricity or ischemic stroke in the left thalamus, occiptotemporal
previous cervical trauma or physical activity before the sensitivity. Personal history: Depressive disorder for 10 gyrus and cuneo, besides a stroke sequel on the right
installation of the symptoms and she referred that it all years; Bilateral cataract; Consanguinity between parents; frontal, temporal, parietal, insula, adjacent to the lateral
started while she was taking a shower. The patient has sibling with psychiatric disorder, bradykinesia, bradypsy- fissure. The head and neck angio tomography revealed a
systemic arterial hypertension, dyslipidemia and diabe- chism and ataxia. On examination: alert, oriented, mild oclusion of the comunicating segment of the right inter-
tes. She didn’t have any smoking or etilism history and in dysarthria and bradypsychism; isochoric and photoreac- nal carotid as well as the A1 segment of the right anterior
her family there was no history of previous stroke. At the tive pupils, with bilateral central opacification; decreased cerebral artery, the right middle cerebral artery and the
admission she presented incoordination on the left side speed of ocular saccades; hypopalesthesia to iliac crests P2 segment of the left posterior cerebral artery (PCA).
of her body and an ataxic gait without other findings. The bilaterally; muscle strength preserved with global bra- Cardiac investigation was done with electrocardiogram,
CT showed hypoattenuation on the posterior and inferior dykinesia; global hyperreflexia, Hoffman and Trommer 24 hour holter, transthoracic and transesophageal echo-
cerebellar vermis and on the left cerebellar hemisphere present bilaterally; inexhaustible bilateral achilleus clonus; cardiogram resulting normal. It was then performed the
compatible with an acute stroke. The CTA demonstrated broad-based and bouncing gait; positive romberg. Mini- cerebral arteriography resulting intracranial vascular
a hypodense semicircular formation partially obliterating mental with a score within the expected level of educa- oclusive disease in two distinct vascular territories (a
the flow of contrast on the left subclavian artery deter- tion (29/30). Cranial MRI with bilateral and symmetrical cronic feature occlusion on the top of the right ICA and
mining a stenosis greater than 50% with extension to the signal alteration of the supratentorial white matter, with a subocclusion on the P2 segment of the left PCA), possi-
ipsilateral vertebral artery. The diagnosis of stroke due to periventricular predominance, with intermingled cystic bly due to an inflamatory component. Pulse therapy was
spontaneous subclavian and vertebral arteries dissection formations, compatible with leukoencephalopathy; it then performed with 1 g per day of methylprednisolone
was given. The patient was discharged in use of warfarin also has T2/FLAIR hypersignal in the caudate nucleus for 5 days, and a posterior introduction of prednisolone
and returned in consultation with almost full resolution and putamen bilaterally. Discussion: The association of 60 mg per day. Discussion: AOSD is an inflamatory dis-
of the symptoms after a few months. Arterial dissection is the progressive clinical findings of ataxia and extrapyra- order, characterized by daily fever, arthritis and evanes-
when a tear occurs in the inner layer of the artery, caus- midal manifestations with the aspects evidenced in the cent rash that occurs in patients older than 16 years old.
ing an intramural hematoma. The stroke due to arterial magnetic resonance suggests a neurodegenerative picture The etiology is unknown with an annual incidence of
dissection is a consequence of the embolism originat- with predominant involvement of the white matter. The approximaly 0,16 cases per 100.000 people. The AOSD is
ing on the inner layer of the vessel or a consequence of history of consanguinity between the parents, in addition a diagnosis of exclusion and can be done on base on the
local hemodynamic compromise due to reduction of the to a similar evolutionary picture in the sibling, point to a clinical features and the typical laboratorial findings fol-
lumen of the vessel. The arteries most commonly affected genetic etiology. During Hospitalization, a genetic panel lowing the yamaguchi diagnostic criteria. Ischemic istroke
are the cervical and this type of lesion is generally due to for leukoencephalopathies was performed on the patient, is a rare complication of this disease, and the explanation
traumatic events, surgical positioning or endovascular which showed probably pathogenic homozygosity in the for this event is the vasculitis pathophysiology. The cor-
procedures. However, a spontaneous subclavian arterial COQ8A gene and an increase in copies (03) of the EIF2B1 ticosteroids are the first line druggs for treatment. Final
dissection causing a stroke is a very rare event, with only gene, with uncertain significance. Final Comments: The Comments: We reported an uncommon case of a young
a few cases described in medical literature, and can be COQ8A gene plays a key role in the biosynthesis of coen- 44 years old woman with previous diagnose of AOSD,
associated with migraine, pregnancy and use of illegal zyme Q10, which acts as an electron carrier in the mito- that had a stroke in different territories, with involvment
substances. The incidence is higher in women between 40 chondrial respiratory chain for increased ATP production of the anterior and posterior circulation, visualized by
and 50 years associated with systemic arterial hyperten- and also acts as an antioxidant and membrane stabilizer. the head and neck angio tomography and arteriography
sion. Although rare, the spontaneous subclavian arterial The phenotypic characteristics reported in a study with corroborating with typical finding of vasculitis. Thus, it’s
dissection is a relevant cause of stroke, specially in young patients with a biallelic alteration in the COQ8A gene importante to be alert that one of the possible complica-
adults, thus clinical suspicion is necessary for a proper include cerebellar ataxia (100% of cases), movement tions of the AOSD is the occurrence of stroke events due
management of the case. Anyway it has a good prognosis disorders, cognitive impairment, epilepsy and neuro- to vasculitis inflamatory process.
even with conservative therapy. psychiatric symptoms (depression, anxiety, psychosis
or impulsive-aggressive behavior). The main therapeu-
tic approach has been coenzyme Q10 supplementation. Doença Cerebrovascular
Doença Cerebrovascular

Doença Cerebrovascular

35
XXX Congresso Brasileiro de Neurologia

TL 1106059 TL 1106085 TL 1106105

COMPLETE BLOOD COUNT IN ANEURYSMAL COMPLETE RESOLUTION OF A CONSECUTIVE HEMORRHAGIC STROKES


SUBARACHNOID HEMORRHAGE SCENARIO: HUGE CAROTID THROMBUS AFTER POSSIBLY CAUSED BY CEREBRAL AMYLOID
POSSIBLE USES OF BLOOD ELEMENTS FOR ANTICOAGULATION IN 48-YEAR-OLD ANGIOPATHY RELATED TO DURAPLASTY
PREDICTING OUTCOMES WOMAN WITH STROKE
Ian Silva Ribeiro, Karla Rafaele Silva Vasconcelos,
Savio Batista dos Reis, Herisson Rodrigues de Sheila Ferreira Pessoa, Murilo Santos de Souza, Larissa Brenda Gonçalves Miná, Sarah Diógenes
Oliveira, Amanda Cacaes Modesto Accioly, Arthur Anderson Rodrigues Brandão de Paiva, Alexandre Alencar, Robson de Sales Ferreira, João Igor Dantas
Antônio Freire Alves, Maurus Marques de Almeida Drayton Maia Barros, Saulo Pimenta Lacerda, Ana Landim, Sylvio Ricardo Gonçalves de Souza Lima,
Holanda Clara Guilherme Rodrigues, Thaina Gonçalves Samuel Ranieri Oliveira Veras, Norberto Anízio
Tolentino de Figueredo, Anselmo de Jesus Santos Ferreira Frota, Fernanda Martins Maia Carvalho
Universidade Federal do Rio de Janeiro. Rio de
Janeiro RJ, Brazil Hospital São Rafael. Salvador BA, Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Universidade Federal da Paraíba. João Pessoa PB,
sheilaferreirapessoa@hotmail.com iansilvarbr@gmail.com
Brazil

heri.md.92@gmail.com Case Presentation: A 48-year-old woman, without previ- Case Presentation: A 44-year-old male patient was admit-
ous comorbidities, admitted by complete right hemipare- ted to our Hospital in March 2022 complaining of head-
Background: Aneurysmal subarachnoid hemorrhage sis, mixed transcortical aphasia and right homonymous ache and left homonymous hemianopsia. In the past two
(SAH) is a worldwide health burden with high fatal- hemianopsia 7 hours after admission, NIHS 7. Imaging years, he had two intraparenchymal hemorrhages, both
ity and permanent disability rates, and variable prog- of the skull at the arrival showed no established ischemic in the right parietooccipital lobe. Computed tomography
nosis. Recent evidence suggests that some biomarkers lesion, however, arterial angiography of the neck showed showed right parietooccipital intraparenchymal hema-
play a critical role in predicting outcomes. Objectives: the presence of a subtraction image inside the left com- toma, left parietooccipital encephalomalacia and signs
We propose a systematic review of literature on recent mon carotid artery with irregular contours reducing the of a previous left occipital craniectomy. Magnetic reso-
advances in blood element biomarkers for aneurysmal lumen of the vessel by up to 90%, suggesting the presence nance imaging confirmed the above findings, also showing
SAH, translating from a physiological point of view to of intra-arterial thrombus. Started full anticoagulation with several corticosubcortical punctate foci of low signal on
their potential usefulness. Methods: Pubmed, Embase, soredic enoxapara at a total dose of 120mg/d. He presented SWI sequence predominating on bilateral occipital lobes
Scopus, Cochrane, and Web of Science databases were a transesophageal echocardiography (ETE) demonstrating indicating microbleeds. Angioresonance did not show
systematically searched to identify studies assessing patent foramen of high conductance. Maintained antico- any abnormalities. These findings support the possible
red blood cell element biomarkers for aneurysmal sub- agulation with Apixaban at Hospital discharge at a total diagnosis of cerebral amyloid angiopathy (CAA) related
arachnoid hemorrhage, following sensibility, and spec- dose of 10mg and after 15 days of treatment, a new con- hemorrhage according to the modified Boston criteria,
ificity results found in each paper published. Results: trol image was performed now with vessel wall protocol excluding the age criteria. The patient had a past medical
Six studies were included. Delayed Cerebral Ischemia with evidence of complete resolution of the thrombus history of head trauma at the age of 7 years, caused by a
(DCI) was predicted by Neutrophil/Lymphocyte ratio and absence of dissection of the vessel wall. Discussion: reaping hook, for which he underwent a neurosurgical
(87.3%/48.4%, and 63%/53%), and Platelet/Lymphocyte common carotid artery occlusion (AOCC) is a rare disease procedure including craniectomy and duraplasty. He had
ratio (55.3%/78.5%). Vasospasm was seen by Platelets and accounts for 1%–5% of all cerebrovascular events. no relevant familiar history of cerebrovascular disease or
(40%/90.5%, and 78%/85%), Lymphocytes (57.9%/83.3%), May present as stroke, transient ischemic attack (ITA), or dementia. Discussion: CAA is commonly found in older
Neutrophils (40%/100%), Leukocytes (50%/100%), with features of cerebral hypoperfusion. Chronic arterial people and in patients with Alzheimer’s disease accompa-
and Combined Platelet and Leukocytes high values occlusions are often attributed to atherosclerosis, while nying cerebrovascular disorders and dementia. Cerebral
(60%/89.5%). Rebleeding in Neutrophil/Lymphocyte other less frequent causes include cardiac embolism, blood vessels affected by CAA are associated with func-
ratio (72.3%/63.3%), and Neutrophil/Lymphocyte ratio Takayasu’s arteritis, post-radiation arteriopathy, syphilis, tional and pathological changes, leading to hemorrhagic
combined with Fisher Grade (39.94%/100%). Poor out- trauma. To date, there is no clear consensus on the best disorders, such as lobar intracerebral hemorrhage and
come in Neutrophil/Lymphocyte ratio (74.5%/69.3%), and treatment in the face of a AOCC and the decisions for cortical micro-hemorrhage. It is hypothesized that amy-
Platelet/Lymphocyte ratio (55.3%/78.5%). Mortality in treatment of these patients are made according to each loid-? (A?), a pathological hallmark of this condition, is
3-months appears predicted by RDW value (80.6%/91.6%). case and experience of the service. Final Comments: transmissible by a similar mechanism to acquired prion
Final Comments: Biomarkers could help in predicting Occlusion of the common carotid artery although rare diseases. Furthermore, besides genetic inheritance, it has
complications in aneurysmal subarachnoid hemorrhage and acute clinical presentations vary from mild to devas- been described the occurrence of early onset A?-CAA
patients. We listed some of the recent advances in this tating, the acute management of these patients remains associated cerebral hemorrhage, several decades after
translational, intriguing, fast-growing field. Although the a major challenge. In our service we report the case of a neurosurgical procedures performed in children involving
knowledge gaps are still significant, some markers are young patient with probable cardioembolic etiology who cadaveric dura mater grafts or neurovascular embolization
demonstrating promising results and could be helping obtained benefit in the resolution of the thrombus with using material derived from cadaveric dura mater. Due to
patients in the near future. the use of direct oral anticoagulants. the early age, temporal association, no family history of
stroke, we hypothesize that the duraplasty may be asso-
ciated with the occurrence of amyloid angiopathy in this
Doença Cerebrovascular Doença Cerebrovascular patient. Final Comments: This case illustrates a possible
cause of early onset CAA and emphasizes that A?-CAA
should be considered in the differential diagnosis even
for young patients with intracerebral hemorrhage and
history of neurosurgery. Furthermore, the exact phys-
iopathology of this condition needs more elucidation
due to the possibility of iatrogenic human transmission.

Doença Cerebrovascular

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XXX Congresso Brasileiro de Neurologia

TL 1106121 TL 1106124 TL 1106125

DIAGNOSTIC CHALLENGE IN A YOUNG BILATERAL SIMULTANEOUS NON-ARTERITIC WYBURN-MASON SYNDROME: A


MAN: DENGUE ENCEPHALITIS OR ANTERIOR ISCHEMIC OPTIC NEUROPATHY IN RARE PHACOMATOSIS WITH MULTIPLE
POST-DENGUE ACUTE DISSEMINATED A ELDERLY AFTER SPINE SURGERY: A CASE ARTERIOVENOUS MALFORMATIONS
ENCEPHALOMYELITIS (ADEM)? PRESENTATION
Livio Leite Barros, Marilia Brito Ricarte, Pedro
Yan da Silva Raposo, Daniel Isoni Martins, Eduardo Daniel Isoni Martins, Yan da Silva Raposo, Carolina Helder Oliveira Junior, Liandra Rayanne Sousa
Antônio Roquim e Silva, Galileu Chagas Lourenço, Sales Carlos, Caroline Nogueira Aguiar, Jéssica de Barbosa, Mariana Michiles Santos Ramos, Wallison
Henrique Oliveri Leite Praça, Laisse Leite Ribeiro, Cássia Marques Leocádio, Renata Cristina Resende, Granjeiro Bringel Silva, Carolina Figueiredo Santos,
Laura Helena Poggianella Esteves, Lucas Bruno Rafael Mattos Tavares, Eduardo Jardel Portela, Manoel Alves Sobreira Neto, Pedro Braga Neto,
Rezende, Marco Andrée Morel Téliz, Rafael Mattos Gustavo Camargos de Toledo Santos, Eduardo Paulo Ribeiro Nóbrega
Tavares Antônio Roquim e Silva
Hospital Universitário Walter Cantídio. Fortaleza
Hospital das Clínicas Samuel Libânio. Pouso Alegre Faculdade Ciências Médicas de Minas Gerais. Belo CE, Brazil
MG, Brazil Horizonte MG, Brazil Universidade de Fortaleza. Fortaleza CE, Brazil
Faculdade Ciências Médicas de Minas Gerais. Belo Hospital das Clínicas Samuel Libânio. Pouso Alegre Hospital universitário Walter Cantídio. Fortaleza
Horizonte MG, Brazil MG, Brazil CE, Brazil
Hospital Vila da Serra. Nova Lima MG, Brazil Hospital Vila da Serra. Nova Lima MG, Brazil
livioleiteb@gmail.com
Hospital Nova Lima. Nova Lima MG, Brazil
yanraposo@yahoo.com.br Hospital Mater Dei Betim-Contagem. Betim MG,
Case Presentation: An 18-year-old male patient, initially
Brazil
Case Presentation: A 25-year-old male patient presented evaluated at the age of 2 for proptosis and visual impair-
a clinical course suggestive of dengue, with a positive yanraposo@yahoo.com.br ment of the right eye. A red supraorbital skin lesion was
NS1 test. With less than 24 hours of symptoms, he began perceived at that moment. There was no family history of
progressive mental confusion, focal seizures, and sub- Case Presentation: A 78 years-old woman, caucasian, neurological disorders and his parents were nonconsan-
sequent sensorium lowering. Brain magnetic resonance a retiree, residing in Belo Horizonte, Minas Gerais State, guineous. Magnetic resonance imaging (MRI) revealed
imaging was performed, which showed foci of diffusion Brazil. Patient with systemic arterial hypertension and an arteriovenous malformation (AVM) involving the right
restriction and hyper signal on T2/FLAIR bilaterally in the right peripheral facial nerve palsy. Prior cataract surgery. cavernous sinus, which was treated with embolization.
brainstem, hippocampus, and thalamus topographies, a Due to spinal stenosis, she was submitted to a thoracic Despite the procedure, proptosis and edema of perior-
pattern suggestive of encephalitis. An electroencephalo- lumbar spinal decompression procedure on March 31th, bital tissue continued to worsen and at the age of 12 he
gram was performed, which showed bursts of slow waves 2022. Performed for nine hours in a prone position without underwent a biopsy of frontal, infra-orbital and periorbital
predominantly frontally bilaterally and rare epileptiform intercurrences. After eight hours, she awoke from anes- subcutaneous tissue which was compatible with a cav-
paroxysms in the parietal topography bilaterally. A lumbar thesia with psychomotor agitation and complaining of ernous angioma. At the age of 18 he was evaluated at our
puncture was performed, which showed only an increase severe pain at the surgical site and reduced bilateral visual institution. On neurologic examination there was marked
in protein (100mg/dL). Treatment of seizures was per- acuity. Progressed to loss of color vision and ultimately proptosis with impairment on abduction, adduction and
formed with Phenytoin and Levetiracetam. Discussed as bilateral amaurosis. She denied other focal symptoms or elevation as well as amaurosis of the right eye. Edema of
a team and opted to perform Immunoglobulin for 5 days, headaches. No reports of mental confusion by assistant periorbital, frontal and zygomatic regions on the right was
with subsequent significant improvement of the condition. staff. Without a history of visual impairment. The patient also seen. Preserved strength and tone, but deep tendon
At Hospital discharge, the patient had improved orienta- presented to the neurological examination with a regular reflexes were brisk, and Hoffman and Babinski signs were
tion and had no focal deficits. Discussion: Dengue is the general condition, good spatial orientation, alertness, present. MRI revealed an expansive formation centered in
main cause of encephalitis with normal cellularity in the Glasgow Coma Scale 15, right corectopia, normal extrinsic the right orbit causing proptosis and compromising conal
cerebrospinal fluid in endemic regions. Although there ocular motricity, hand movement perception half a yard spaces with distortion of eye muscles and optic nerve. The
are no characteristic lesions for dengue encephalitis, the away, right peripheral facial nerve palsy, without menin- lesion extended through the superior orbital fissure into
patient had lesions characteristic for encephalitis in gen- geal irritation signs. Ophthalmoscopy revealed bilateral the right cavernous sinus and to the contralateral orbit.
eral, that is, involvement of the limbic system, in addition papilledema more evident on the left and retinal pigment Discussion: Wyburn-Mason syndrome is a rare, non-he-
to hyperproteinorraquia and a suggestive clinical course. epithelium atrophy, without signs of vasculitis. Magnetic reditary congenital disease, belonging to the group of
Therefore, dengue encephalitis was our main diagnostic Resonance Angiography of the skull revealed moderate neurocutaneous syndromes, with fewer than 100 cases
hypothesis. However, there was also a differential diag- stenosis of the left anterior cerebral artery, hypoplasia reported since its first description in 1937. It usually affects
nosis with ADEM, which is a post-infectious autoimmune of the left vertebral artery, and moderate stenosis of the the orbit, retina and brain, with predominant involve-
encephalitis. Despite differences in pathogenesis and supraclinoid segment internal carotid artery, but with ment of the midbrain. Lesions may also affect the skin,
histological findings, their clinical course and signs in the good distal flow. Magnetic Resonance Imaging of maxilla, jaw, pharynx, oral and nasal cavities. Headache,
imaging tests can be very similar. Therefore, since it was skull without restriction on diffusion-weighted imaging seizures and hemiparesis are some other neurological
not possible to rule out ADEM and given the patient’s or hyperintense signal on T2-weighted FLAIR. There manifestations of the syndrome. Despite an uncertain
clinical severity at the time, we chose to perform an was a gradual spontaneous improvement of the visual etiology, an embryonic defect is believed to result in the
Immunoglobulin infusion for 5 days. Final Comments: deficit. On April 04th, she reported a return to baseline dissemination of vascular lesions originating from the
There is no specific treatment for dengue encephalitis, visual acuity. She had uncorrected visual acuity in both cephalic mesoderm along the migratory route, before
with most patients having a good prognosis. ADEM can be eyes: 20/70 vision. Discussion: Postoperative visual loss the actual migration of vascular cells to their destination.
treated with immunosuppressive medications. Given the following prone spine surgery occurs in from 0.013% to Final Comments: Treatment of AVMs with endovascular
diagnostic doubt, we chose to use Immunoglobulin, and 1% of cases, and the most frequently quoted risk is 0.2%. techniques, surgery and radiosurgery are possibilities,
the patient had a good response and recovery. It is attributed mainly to ischemic optic neuropathy. but conservative treatment may be preferred due to the
The multiple factors contributing to POVL may include; high risk of recurrence. However, bevacizumab, an anti-
prolonged operative times, long-segment spinal instru- VEGF agent, has been shown to contain macular edema
Doença Cerebrovascular mentation, anemia, intraoperative hypotension, greater and improve visual loss in a short follow-up. Therefore,
estimated blood loss (EBL), microvascular pathology, given the rarity of the pathology in question, the present
and decreased percent colloid administration. Final report seeks to add to the literature.
Comments: Laboratory tests revealed no hematimetric
abnormalities and no excessive bleeding or hemody-
namic instability was reported during surgery, however, Doença Cerebrovascular
the operative time was prolonged.

Doença Cerebrovascular

37
XXX Congresso Brasileiro de Neurologia

TL 1106145 TL 1106157 TL 1106162

RECURRENCE OF STROKE IN A PATIENT SEPTIC THROMBOSIS OF THE TRANSVERSE PERIMESENCEPHALIC HEMORRHAGE:


WITH FIBROMUSCULAR DYSPLASIA AND SIGMOID SINUS, DISCUSSION A RARE ENTITY WITH POSSIBLE
ABOUT THE INTRODUCTION OF FULL COMPLICATIONS
Clara Kimie Miyahira, Diogo Lima de Souza Ramos,
Bruna Burigo, Thiago da Cruz Marques, Natalia ANTICOAGULATION: CASE PRESENTATION
Ana Silvia Sobreira Lima Verde, George Nilton Nunes
Figueiredo Miranda, Beatriz Medeiros Correa, Beatriz Medeiros Correa, Vanessa Moraes Rossete, Mendes, Alessandra Braga Cruz Guedes de Morais,
Vanessa Moraes Rossette Clara Kimie Miyahira, Daisi Fritz Jordao, Thiago Sylvio Ricard Gonçalves de Souza Lima, Felipe
da Cruz Marques, Julio Cesar Rodrigues, Natalia Araújo Rocha, Adson Freitas de Lucena, Sarah
Santa Casa de Santos. Santos SP. Brazil
Figueiredo Miranda, Raphael Palomo Barreira, Diógenes Alencar, Karla Rafaele Silva Vasconcelos,
clara.kimie@gmail.com Antonio Carlos de Padua Milagres Larissa Brenda Gonçalves Miná, Fernanda Martins
Maia Carvalho
Case Presentation: A 64-year-old woman, previously Irmandade Santa Casa da Misericórdia de Santos.
Guarujá SP, Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil
hypertensive, woke up with a sudden loss of strength on
the right and dysmetria on the left. She was rescued, but Irmandade Santa Casa da Misericórdia de Santos.
aninhaslv@icloud.com
with a time longer than 4.5 hours, which contraindicated Santos SP. Brazil
the use of thrombolytics for stroke. Basic investigation beamedneuro@gmail.com Case Presentation: A 37-year-old woman, previously
was performed with cranial tomography at admission healthy, was admitted with a 5-day history of occipital and
and after 24 hours, transthoracic echo, carotid and ver- Case Presentation: Septic sigmoid sinus thrombosis is nuchal headache with irradiation to the back, inedit, pro-
tebral Doppler. After normal findings in all exams, the a rare disease and is commonly caused by middle ear gressing to worst intensity in 5 to 10 minutes, with nausea
investigation continued with cranial magnetic resonance, infections, with Staphylococcus aureus being the main associated. It was also evidenced by high blood pressure
which showed T2/FLAIR hypersignal in bridge and left etiologic agent. Clinical manifestations are diverse and (220x130 mmhg). 3 days after the initial symptoms, she
cerebellum with restriction of water molecule to diffu- headache is the most frequent complaint followed by focal had mental confusion and irritability. The neurological
sion. Patient progressed using secondary prophylaxis deficits such as cranial nerve involvement. Radiological examination revealed neck stiffness and parisia of the
with dual antiplatelet therapy. However, 17 days later, investigation and lumbar puncture are important in aid- right lateral rectus. A brain non contrast computerized
she was Hospitalized again, with a worsening of the ing the diagnosis. To discuss the controversial use of full tomography (NCCT) was performed – we found hyper-
same symptoms. A new CT scan 1 day after the new anticoagulation in cases of septic thrombosis, describ- dense images near the mesencephalic cisterns. Then we
condition showed subacute hypodensity in a capsule ing a patient treated at a tertiary Hospital in which it performed a digital subtraction angiography (DSA), which
on the left. Cerebral angiography showed occlusion of was decided not to introduce this therapy. Discussion: was normal. The following day, the patient presented
vertebral arteries and a pattern of multiple parietal irreg- G.S.P.L, 15 years old, male, admitted with a report of otal- worse of mental confusional, papilledema, complete
ularities, extending throughout its course in the“string of gia due to refractory oral antibiotic therapy. Evolved with ophthalmoplegia in the right eye and parisia of the lateral
beads” appearance. Secondary prophylaxis was changed vomiting, loss of appetite, persistent fever and periods rectus in the left eye. A brain magnetic resonance imag-
to anticoagulation with apixaban, in addition to motor of sleepiness. A neurological examination showed rigth ing (MRI) showed multiple areas of restricted diffusion
rehabilitation and outpatient follow-up. Discussion: appendicular ataxia and purulent discharge from the in the occipital lobe, bilateral cerebellar and right front.
Fibromuscular dysplasia (FMD) is a non-inflammatory external auditory canal directly to otoscopy. Performed A MRI angiography with vessel wall was performed and
and non-atherosclerotic disease that courses with arte- cranial tomography with diffuse veiling of the cells of the showed multiple stenotic focus in large vessels (ACID,
rial stenosis, occlusion, aneurysm, dissection and arte- mastoid and the right middle ear and hypodensity of the ACMD, ACPD), and fine enhancement still on their walls.
rial tortuosity, more frequent in young adult women, right cerebellar hemisphere with a nonspecific appear- Based on these findings, and considering the severity and
the internal carotid artery is more frequently affected, ance. Cerebrospinal fluid was collected with pleocytosis aggressiveness of this disease, we treated her for central
histological form more common is fibroelastic dysplasia and hyperproteinorraquia. During Hospitalization, a nervous system (CNS) vasculitis with pulse therapy (meth-
with thickening of the media and muscular layer with Cranial Magnetic Resonance with Angioresonance was ylprednisolone 1 gram for 5 days). After the third day, the
destruction of the elastic lamina. Diagnosis is by vessel performed, which showed opacification of the mastoid patient improved her mental confusion and her ophthal-
study. Genetic predisposition, chronic trauma, stretching cells and tympanic cleft on the right and filling failure moplegia. In order to evaluate if the patient’s improvement
of the involved vessel and hormonal factors are believed in the sigmoid and adjacent transverse sinus, inferring was related to radiologic findings, we therefore submitted
to have been suggested as etiologic agents. The clinical venous thrombosis. Broad-spectrum antibiotic therapy her to another DSA. The findings were related to cerebral
presentations are determined by the affected artery. In was started for a long time and a surgical approach was vasospasm than to CNS vasculitis. 25 days after the initial
this case, the patient presented a recurrent and relapsing scheduled with the otorhinolaryngology team. Final symptoms, we performed a new DSA, which was normal.
symptoms in a short period of time, in two different terri- Comments: When questioned about the use of antico- Her final diagnosis was delayed cerebral ischemia (DCI)
tories, first in the anterior inferior cerebellar artery terri- agulants, the benefit was not superior to the risks related due to a perimesencephalic hemorrhage (PMH). The
tory, and later in the lenticostriate artery territory. Final to this therapy, the use prevents the persistence of the PMH’s clinical features described in some reviews were
Comments: FMD associated with neurovascular events thrombus, but it can facilitate the release of septic emboli headache with instantaneous or more gradual onset,
can be treated with antiplatelet therapy, blood pressure and increase the risk of bleeding. Thus, in the case pre- accompanied by nausea and vomiting. It’s rare, but can
control, or treatment with angioplasty with or without sented, a conservative approach was chosen, based on also present with loss of consciousness, transient focal
stent implantation. However, in this case, antiplatelet antibiotic therapy and neurological surveillance without symptoms or epileptic seizure. Complications are not
therapy was not enough for prevention in a short period the introduction of anticoagulation. common, but can occur hydrocephalus, DCI, rebleeding,
of time, opting for anticoagulation with apixaban, which amnesia and cranial nerve palsies. The diagnoses of PMH
kept the patient stable without new neurovascular events. can be done with a NCCT. MPH with short term com-
Doença Cerebrovascular plications are rare and long-term outcome is excellent.
Although it is a condition that remains underdiagnosed.
Doença Cerebrovascular We understand very few aspects of this condition. But
knowledge about this disease is still under development.

Doença Cerebrovascular

38
XXX Congresso Brasileiro de Neurologia

TL 1106179 TL 1106191 TL 1106228

SPONTANEOUS DISSECTION OF THE UNUSUAL PRESENTATION OF ISCHEMIC STROKE IN A YOUNG PERSON: SLE AND FAS
BASILLAR ARTERY: CASE PRESENTATION STROKE DUE TO STENOSIS OF PERSISTENT ASSOCIATED WITH MULTIPLE EVENTS:
PRIMITIVE HYPOGLOSSAL ARTERY: A CASE A CASE PRESENTATION
Raíssa Barreto Vieira Soares, Dkaion Vilela de Jesus,
Isadora Garcia Carneiro Kriunas Severino, Maria PRESENTATION
Beatriz Medeiros Correa, Vanessa Moraes Rossette,
Ondina Machado Diniz, Alessandra Braga Cruz Gabriella Maria Martins Favero, Samia Talise El Daisi Carolina Fritz Jordao, Julio Cesar Rodrigues,
Guedes de Morais, Vitor Oliveira Machado, Fernando Horr de Moraes, Michelle Zonkowski Ribas, Marcos Clara Kimie Miyahira, Natalia Figueiredo Miranda,
Elias Borges Christiano Lange, Viviane de Hiroki Flumignan Thiago da Cruz Marques, Gustav BOgel, Raphael
Zétola, Valéria Cristina Scavasine, Edison Matos Palomo Barreira, Antonio Carlos Milagres
Santa Casa de Misericórdia de Goiânia. Goiânia GO,
Brazil Novak, Gabriel Abrahão Stoliar, Alana Bacelar
Irmandade Santa Casa da Misericórdia de Santos.
Hospital de Urgências de Goiânia. Goiânia GO, Brazil Limeira Sales, Rebeca Tamara Milan
Santos SP. Brazil
Hospital Geral de Goiânia. Goiânia GO, Brazil Hospital de Clínicas da Universidade Federal do
Hospital Geral de Fortaleza. Fortaleza CE, Brazil beamedneuro@gmail.com
Paraná. Curitiba PR, Brazil
Centro Estadual de Reabilitação e Readaptação.
Goiânia GO, Brazil gabriellammf@yahoo.com.br Case Presentation: Patient 25 years old, with a history
of diagnosed idiopathic dilated cardiomyopathy and a
dkaion@hotmail.com Case Presentation: A 74-year-old man only treated for stroke in July 2021, was admitted to the service with a
benign prostatic hyperplasia underwent elective magnetic decreased level of consciousness, mental confusion and
Case Presentation: Female, 16 years old, history of resonance imaging (MRI) to investigate left hypoacusis right heparesis, preceded by headache with onset of the
migraine, without other comorbidities. She presented with months of evolution. An area of bilateral parieto-oc- previous three days. Cranial CT showed acute cerebel-
intense pain in the cephalic region, evolving immediately cipital diffusion restriction was detected with hypersignal lar hypodensity on the right, with chronic hypodensity
with loss of consciousness, without previous traumatic in fluid-attenuated inversion recovery and hyposignal in in the midbrain and right parietal lobe. In other exams,
brain injury. Therefore, she was admitted to an intensive the apparent diffusion coefficient. The patient was admit- normocytic normochromic anemia, lymphopenia, hypo-
care unit. Brain computed tomography with contrast per- ted without any neurological deficits, but reported a recent albuminemia, proteinuria and microscopic hematuria
formed in the acute phase showed hyperdensity in the episode of vertigo lasting seconds in addition to reduced were evidenced. During complementary investigation,
basilar artery with filling failure after contrast, suggesting a visual acuity. He had a blood pressure of 129/74mmHg right vertebral dissection was evidenced in AngioMR.
thrombus inside it, without hemorrhagic transformation. and capillary blood glucose level of 97mg/dL. Therefore, Taking into account the set of findings, an evaluation of
The patient was admitted by neurology after 14 days of ictus a dual antiplatelet therapy was initiated. During the Rheumatology was requested. Rheumatologic screening
presenting tetrasegmental hypotrophy, reduced tone and investigation, carotid and vertebral Doppler revealed was positive for Lupus with associated Antiphospholipid
complete quadriplegia provided. Tetrasegmental hyper- non-significant carotid stenosis. Electrocardiography Antibody Syndrome (FAN 1: 1280; Anti-SM: positive; anti-
reflexia with pyramidal signs. Tromner and Hoffman are revealed sinus rhythm and Holter monitoring detected cardiolipin IgM and IgG: positive, lupus anticoagulant
present. Plantar cutaneous reflex in extension to the left minimal changes. Transthoracic echocardiography iden- positive). The patient evolved with pre-renal failure, due
and indifferent to the right. Absence of meningeal signs. tified an enlarged left atrium and ventricle with concen- to probable class 3/4 lupus nephritis, acute respiratory
Isophotoreactive pupils. Slowed ocular motricity. Facial tric remodeling. Computed tomography angiography failure of cardiogenic origin, with bilateral transudative
mimicry without alterations, paralysis of the conjugated revealed bilateral vertebral artery (VA) hypoplasia and a pleural effusion. Discussion: About 10% of stroke cases
gaze to the left, nausea reflex absent. She underwent cra- persistent primitive hypoglossal artery (PPHA) originat- occur in people under 45 years, with acquired thrombo-
nial magnetic resonance angiography 25 days after the ing from the right internal carotid artery, giving rise to the philia and autoimmune diseases being the main precipi-
event, showing a caliber reduction of about 50% in the basilar artery, in addition to a hypodense plaque during tating factors. SLE is an important cause, as it is a chronic
middle third of the basilar artery. During Hospitalization, its course in the hypoglossal canal, reinforced by vessel systemic inflammatory disease. About 36% of SLE cases
a negative investigation was performed for vasculitis and wall MRI. Craniocervical junction computed tomography are associated with Antiphospholipid Antibody Syndrome
thrombophilia. Dissection is the loss of structural integrity did not reveal any extrinsic compression. Discussion: (APS), responsible for more than 20% of strokes in young
of the arterial wall, allowing blood to pool between the PPHA is a rare vascular anomaly that is usually detected people and the most frequent cause of acquired throm-
arterial layers. Occlusive disease of the basilar artery most incidentally during cerebral angiography and is generally bophilia. The mechanism of injury is mainly due to alter-
often presents as pons ischemia. The paramedian pontine asymptomatic. It is mainly associated with aneurysms, ations in coagulation, generating a prothrombotic state of
base contains the descending long motor tract and the probably because of the anomalous structure of the ves- hypercoagulability, predisposing to multiple events. Final
cerebellar fibers that cross it. The paramedian tegmen- sel wall, which can expose the basilar trunk to excessive Comments: In the case presented, the patient had SLE
tum contains mainly oculomotor fibers. Altered level of hemodynamic stress. As most patients have hypoplastic and APS associated with a high level of disease activity,
consciousness is an important sign in patients with bas- VA, the blood supply to the posterior circulation travels even in the presence of adequate treatment. A fact that
ilar artery occlusion. They may present with coma when through PPHA. Therefore, in ischemic stroke patients predisposed the patient to present several thrombotic
the bilateral medial pontine tegmentum suffers ischemic with PPHA, both the anterior and posterior circulations events, with evidence of three strokes and bilateral deep
insult. In the case presentation, a cerebrovascular acci- are often involved. We present the case of a patient with vein thrombosis of the lower limbs, even in the presence
dent was evidenced after spontaneous dissection of the PPHA and bilateral hypoplasia of the VA, who presented of full anticoagulation. Evidencing that in these patients
basilar artery, compromising the brainstem, generating with a bilateral oligosymptomatic stroke with a hemo- the main decisive factor is the autoimmune disorder.
Basilar Artery Syndrome characterized by motor and ocu- dynamic component caused by a plaque found in the
lomotor signs and alteration in the level of consciousness. hypoglossal artery, which is an uncommon location of
stenosis. Final Comments: We demonstrate a case of rare Doença Cerebrovascular
anatomic variation communicating the anterior and pos-
Doença Cerebrovascular
terior circulations. In this way, we show the importance of
knowing the anatomical variations in cerebral circulation
to identify possible unusual presentations of stroke and
to choose the best secondary prevention.

Doença Cerebrovascular

39
XXX Congresso Brasileiro de Neurologia

TL 1106229 TL 1106233 TL 1106234

STROKE ASSOCIATION WITH EXTENSIVE SPONTANEOUS BILATERAL CAROTID PACHYMENINGITIS: A CASE PRESENTATION
CENTRAL VENOUS TROMBOSIS IN THE DISSECTION: A RARE PRESENTATION OF
João Igor Dantas Landim, Isabella Nunes Matos,
CONTEXT OF NEUROINFECTION ACUTE ISCHEMIC STROKE Patrícia Pereira de Andrade, Matheus Costa Bessa,
Alessandra Braga Cruz Guedes de Morais, Morgana Lucas Gondim Briand Vieira, Ian Silva Ribeiro, Karla Ian Silva Ribeiro, Robson de Sales Ferreira, Matheus
Feitosa de Queiroga, Pedro Rubens Araujo Carvalho, Rafaele Silva Vasconcelos, Sarah Diógenes Alencar, da Costa Guedes, Fernanda Martins Maia Carvalho,
Sarah Diógenes Alencar, Ana Silvia Sobreira Lima Karoline Ferreira Menezes Mororó, Álissa Ellen Noberto Anizio Ferreira Frota, Déborah Moreira
Verde, Dkaion Vilela de Jesus, Esther Grangeiro Formiga Moura, Daniel Gurgel Fernandes Távora, Rangel
Barreto, Fabricio Oliveira Lima Fernanda Martins Maia Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Hospital Geral de Fortaleza. Fortaleza CE, Brazil Hospital das Clínicas da Faculdade de Medicina de Universidade de Fortaleza. Fortaleza CE, Brazil
Hospital de Urgências de Goiania. Goiânia GO, Brazil Ribeirão Preto. Ribeirão Preto SP, Brazil João igorlandim@hotmail.com
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
alebcgmorais@gmail.com
lucasbriand@hotmail.com Case Presentation: A 48 years old female, with history of
Case Presentation: A 48 old female patient was admitted headache with phenotype of migraine with visual aura.
to the emergency department due to several episodes of Case Presentation: A 57-year-old male patient was In 2005 she developed headache with pattern change,
seizures without recovery of consciousness lasting more referred from an Emergency Care Unit to our Hospital suggestive of intracranial hypertension, and targeted
than 5 minutes. There was no record of previous diseases due to a sudden loss of consciousness associated with propedeutics was performed. On clinical and imaging
besides one Hospitalization due to status epilepticus four headache and high blood pressure at 10: 55 on 15/04/2022. investigation, indirect signs of intracranial hypertension
months earlier, from which she was discharged with val- There was no history of neck pain, trauma, fall or sub- were found on skull MRI such as: partially empty sella
proate 500 mg once a day. Nonetheless, the patient was stance use or abuse. The patient was admitted intubated turcica, enlargement of the perioptic liquoric spaces and
known to live in precarious situation and to be a frequent due to Glasgow Coma Scale < 9. Computed tomography of the cavum of meckel, in addition to regular and diffuse
user of alcohol, tabaco, crack and cocaine. At the admis- was performed, which showed slight left basal nuclei and pachymeningeal thickening at the level of the cerebellar
sion, the patient presented comatose, however without bilateral frontoparietal subcortical hypodensity, with no tentorium, mainly on the right, as well as of the inter-
vital signs alterations. She was stabilized after pharma- bleeding. A new computed tomography was performed hemispheric sickle, in its posterior aspect without a clear
cological measures and was performed intubation for the next day, which showed progress of the initial lesion nodular aspect, a nonspecific finding from the etiological
clinical support. Physical examination revealed left hemi- and a small right frontal cortical hypodensity. The patient point of view, but may be associated with diverse con-
paresis, worse at the upper limb and right eye mydriasis. underwent magnetic resonance imaging that showed DWI ditions, such as pachymeningitis. The CSF was altered,
The Neurological Team was required due to symptoms increased signal and reduced ADC values involving the with increased opening pressure (29 and 30); and inflam-
being presented within time for thrombolytic use in case frontal cortex, frontoparietal white matter, basal nuclei and matory features (cels: 11 (leucus 97%), no macrophages,
of acute stroke. A head computed tomography (CT) scan internal capsule of both hemispheres with hyperintense glucose: 58, prot: 119 ldh: 23). Ophthalmology evalua-
revealed intense cerebral oedema and hyperdensity of the signal on T2/FLAIR, suggesting ischemia. Furthermore it tion with campimetry was without significant changes.
cerebellar tentorium. At CT angiography it was observed showed T2 and DWI high signal crescent sign indicating Rheumatological and serological tests were negative,
absent flow in inferior sagittal sinus, straight sinus and intramural hematoma of both internal carotid arteries except for a high serum IgG 4 value (260.1 – VR: 4-86).
bilateral transverse sinus. Patient was conducted as a and angiography was performed the next day which also Thus, he started treatment for IgG4 hypertrophic pachy-
case of Central Venous Thrombosis and transferred to suggested bilateral carotid dissection and ruled out other meningitis with 1mg/kg/day Prednisone and Azathioprine
the intensive care unit. Patient recovered partially with causes. Discussion: Dissection of vertebral and carotid 2-3mg/kg/day. After starting the medication, there was
persistence of right eye mydriasis and after waking up, arteries are known causes of ischemic stroke and transient a significant improvement in symptoms, but with a side
presented right ptosis. Further investigation revealed on ischemic attack. Dissection can occur due to an external effect attributed to the corticosteroid, which is currently
Magnetic Resonance Restricted diffusion within the right factor, such as trauma, neck manipulation, fall, or due to still being weaned. Discussion: IgG4-related hypertro-
lateral portion of the pons and midbrain, identifying com- an intrinsic vessel disease, such as fibromuscular dyspla- phic pachymeningitis is only one of many possible IgG4-
patible ischemia with clinical findings and pachymenin- sia, or spontaneously. Bilateral carotid dissection is a rare related diseases, it denotes inflammation and thickening
geal enhancement on cerebellar tentorium too. It was also presentation of the phenomenon. In the literature, these of the dura mater and often comes associated with sys-
performed an angiography that observed absent flow in cases were associated with blunt trauma, Eagle syndrome, temic symptoms such as fever, weight loss or abdominal
sinuses and diffuse small venous engorgement leading medication use, Ehlers-Danlos syndrome, and others. pain. Neurological disorders in these patients are often
to the hypothesis of ischemia associated with low venous Spontaneous bilateral carotid dissection is an even rarer associated with nerve or vascular compression, leading
flow. Fluid analysis showed discrete rise of cellular count presentation of neck artery dissection, especially in a to focal deficits, but diffuse inflammation of the dura can
(06 cells with 70% lymphocyte) hyper proteinorachie (66 patient without previous history of vessel disease. Final lead to more diffuse presentations such as headache,
mg/mL) and hypoglycorrhachia (CSF to serum glucose Comments: Spontaneous bilateral carotid dissection is a cervical stiffness, and seizures. Its diagnosis remains a
ratio 0,45). Granulomatous disease such as tuberculosis rare presentation of acute ischemic stroke and can cause great challenge because of the compendium of possible
was raised as a suspicion. On CT scan, the patient had a sudden loss of consciousness due to bilateral infarcts in presentations and its indolent character, which ends up
typical diffuse “tree-in-bud” pattern, consolidations and the brain. This case illustrates this rare presentation and delaying the search for medical assistance by patients.
cavitations. This report intention is to contribute to the the importance of performing a vessel study in a patient Final Comments: The early diagnosis of hypertrophic
differential diagnosis and relevance of neuroinfection with a history suggestive of stroke for the evaluation of pachymeningitis is essential to stop disease progression
causing CVT which can also be responsible for arterial cervical and intracranial vessels. and brain damage. The management of the disease still
ischemia. Association of epidemiological features with is a challenging due to its recent description in 2003, with
unusual differential diagnosis were vital to deliver the a pathophysiological picture not yet fully understood.
right treatment for the patient and could be used as an Doença Cerebrovascular
example of a good clinical practice.
Doença Cerebrovascular

Doença Cerebrovascular

40
XXX Congresso Brasileiro de Neurologia

TL 1106244 TL 1106245 TL 1106246

AN UNUSUAL PRESENTATION OF AN ACUTE HYPERKINETIC MOVEMENTS OF SPONTANEOUS SPINAL EPIDURAL


ISCHEMIC STROKE DUE TO LARGE VESSEL HYPERACUTE ONSET: A CASE HEMATOMA AS A STROKE MIMIC IN TIME
OCLUSION PRESENTATION TREATED WITH RTPA WINDOW FOR THROMBOLYSIS
João Igor Dantas Landim, Matheus Costa Bessa, Mônica Maria Costa do Rêgo Álvares, Kellen Paiva Fernando Cavalcanti de Sá e Benevides Falcão,
Gabriella Bezerra de Figueirêdo, Ian Silva Ribeiro, Fermon, George Nilton Nunes Mendes, Fabrício Rafael Augusto Rosalem, Igor Vilela Brum,
Reed André Siqueira Severo, Fabricío Oliveira Oliveira Lima, Samuel Ranieri Veras, Tamires Guilherme Diogo Silva, Adalberto Studart Neto,
Lima, Fernanda Martins Maia Carvalho, Norberto Ferreira do Carmo, Alberto Jorge Castelo Branco Mateus Mistieri Simabukuro, Luiz Roberto
Anizio Ferreira Frota, Samuel Ranieri Veras, Milena Roque, Walisson Grangeiro Bringel Silva, Déborath Comerlatti
Pitombeira Sales Lúcia Oliveira Diniz, André Borges Ferreira Gomes
Universidade de São Paulo. São Paulo SP, Brazil
Hospital Geral de Fortaleza. Fortaleza CE, Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Universidade de Fortaleza. Fortaleza CE, Brazil f.falcao@hc.fm.usp.br
monicaamancio@gmail.com
João igorlandim@hotmail.com Case Presentation: A 73-year-old woman presented to
Case Presentation: 64-year-old woman, hypertensive, the Emergency Department with right hemiparesis and
Case Presentation: A 58-year-old male with unremark- previously functional (Rankin 0). She had sudden dys- ipsilateral neck pain with sudden onset three hours before
able medical history, was addmited in the emergency arthria, central facial palsy (CFP), and left hemiparesis. arrival. Her past medical history included hypertension
department presenting sudden abnormal movements There was the resolution of the deficits and a clinic pic- and diabetes, but no remarkable history of neurological
in right side limbs. At initial neurologic evaluation, the ture of hyperkinetic movements began. She was admit- disease. Code Stroke was activated. At admission, her
patient showed only hemichorea on the right side, and ted to the neurological emergency with a stroke in the blood pressure was 220/105 mmHg and her blood glucose
his National Institute of Health Stroke Scale (NIHSS) score therapeutic window for thrombolytic treatment and on level was 213 mg/dL. Her National Institutes of Health
was zero. After excluding cerebral hemorrhages in brain physical examination, chorea movements were observed, Stroke Scale score was 8 due to a right-sided hemiple-
CT scan and no contraindications for chemical thrombol- characterized by brief, random and irregular involuntary gia; there was no facial palsy. CT scan showed no signs
ysis, Alteplase was administered. Cranial and cervical CT muscle contractions affecting the left side, associated with of recent ischemic infarction or intracranial hemorrhage.
angiography showed tandem occlusion in the left inter- a slight effacement of the ipsilateral nasolabial fold and CT angiography showed no sign of arterial occlusion,
nal carotid artery and middle cerebral artery. Brain MRI slurred speech. NIHSS: 2 (dysarthria and CFP). Cranial critical stenosis or dissection. As the patient was taken
displayed, an area of restricted diffusion in the left insula. computed tomography: 10 aspects and cranial computed back to the emergency room, her motor deficit improved
He was admitted at Stroke Unit for etiologic evaluation, in tomography angiography: occlusion in the proximal seg- substantially spontaneously, but then got worse again.
addition to acute phase care. There was a fluctuation of ment of the right middle cerebral artery. No dysglycemia. The team decided to proceed with thrombolytic therapy,
deficits in the first 24 hours, nonetheless the patient was After ruling out exclusion criteria, thrombolysis was per- but the time window ran out due to poor blood pressure
discharged after 4 days of Hospitalization with complete formed. A few minutes after the rTPA, she had a resolu- control despite increasing doses of intravenous vasodi-
improvement of abnormal movements. The mechanism tion of the movement disorder. Control image delimited lator drug. The patient was then started on antiplatelet
was defined as athero-embolic with internal carotid artery lesion in Lentiform Nucleus and Insula section on the therapy for secondary prophylaxis. 24 hour follow-up
occlusion, probably chronic due to flow distribution right. The investigation of the mechanism evidenced Atrial CT scan showed no changes from the initial exam. The
pattern evidenced in carotid, vertebral and transcranial Fibrillation as, a probable cardioembolic mechanism. patient performed an MR angiography for persisting
doppler scan. Discussion: Although hemichorea is an The deficits were discharged stable and with outpatient neck pain, which revealed a posterolateral right-sided
uncommon symptom of AVC, studies reveal that vascular follow-up. Discussion: Movement changes can be pri- cervical epidural hematoma. Because her motor deficit
etiology is the most prevalent non-genetic cause among mary (genetic) or secondary to cerebrovascular disease, had remained stable over the last day, a conservative
the various diseases that induce chorea. The results of autoimmune conditions, metabolic causes, neoplasms approach was chosen. Discussion: Spontaneous Spinal
a study published in Neurology showed lesion network (mass effect or paraneoplastic syndromes), iatrogenic Epidural Hematoma (SSEH) represents a rare cause of
mapping to anatomically heterogeneous lesions that can (drug-induced), or trauma. Movement disorders second- spinal cord compression and neurological emergency.
cause hemichorea-hemiballismus, and while the findings ary to stroke are a rare manifestation of cerebrovascular Paraplegia or tetraplegia are the most common presen-
failed to localize a single region, it did show a 92 percent disease. They account for up to 22% of secondary move- tation, while hemiparesis is considered to be a rare fea-
connectivity to the posterolateral putamen, which plays ment disorders and develop in 1-4% of stroke patients. ture, more commonly associated with cervical epidural
a central anatomical and functional role within the basal The main structures affected are the basal ganglia (44%) hematoma. In these rare stroke mimicking presentations,
ganglia-thalamocortical motor loop, emphasizing its and thalamus (37%), although there are case studies that thrombolytic therapy, anticoagulation or treatment with
importance in this unknown pathophysiology. Research also affect the cerebral cortex. Vascular chorea is rarely an antiplatelet agent can lead to hematoma expansion and
indicates that most patients experience complete remis- progressive and may regress spontaneously in about half need for a prompt surgical evacuation. Although clinical
sion of hemichorea-hemiballism within a few days after of patients, but others may persist with symptoms and presentation may be similar to a stroke, previous studies
stroke vascularization. The underlying cause of the clin- need to be treated. Final Comments: Although rare, the have reported persistent neck or back pain and absence
ical finding must be treated, as in the case of the high- initial presentation of acute cerebral ischemia may present of facial weakness as a high index of suspicion of SSEH,
lighted patient, which even without thrombectomy, pre- with hyperkinetic movement disorders and should be an and may help to distinguish from cerebral etiologies. Final
sented full remission post thrombolysis. Final Comments: important differential diagnosis for acute-onset chorea. Comments: This case represents a rare stroke mimic with
A sudden onset hemichorea should be treated as an acute Thus, rapid and decisive action must be performed with potential harm associated with thrombolytic therapy, so
stroke until proven differently. This case supports the an acute phase protocol. SSEH should be always suspected as a differential diagno-
idea that the cortico subcortical transition of the insular sis of ischemic stroke to avoid fatal outcomes.
region has a role in the genesis of aberrant hyperkinetic
movements, as well as a probable functional linkage with Doença Cerebrovascular
the basal ganglia Doença Cerebrovascular

Doença Cerebrovascular

41
XXX Congresso Brasileiro de Neurologia

TL 1106251 TL 1106252 TL 1106262

A CASE OF STROKE IN A PATIENT WITH INTRAVASCULAR LYMPHOMA PRESENTING MANIC EPISODE ASSOCIATED WITH
DOWN SYNDROME ASSOCIATED WITH STROKE AND ISCHEMIC MYELOPATHY ISCHEMIC STROKE: CASE PRESENTATION
MOYAMOYA SYNDROME
Renan Fabri Rosenstein, Mateus Belo, João Paulo Maria Thereza Leitão Mesquita, Priscila Natiele
Adelmar Marinho Neto, Júlio Farias Rangel, Daniel Yoshio da Silva, Francisco Araújo, Luan Marchi, Mauricio Alves, Maria Carolina Rocha Muniz,
Monte de Andrade Gervásio, Yan Gondim de Sousa, Marcos Castello Barbosa, Marcelo Houat de Brito, Mariana Macêdo Militão Mendonça, Gabriela Torres
Miguel Vieira de Almeida Luiz Roberto Comerlatti, Leandro Tavares Lucato, Alves de Carvalho, Nathália Pinheiro Rodrigues, Rian
Mateus Mistieri Simabukuro Vilar Lima, Melina Nunes Teófilo, Victor Elmo Gomes
Hospital Regional do Sertão Central. Fortaleza CE, Santos de Moura, Danilo Nunes Oliveira
Brazil Hospital das Clínicas da Universidade de São Paulo.
Universidade Estadual do Ceará. Fortaleza CE, São Paulo SP, Brazil Universidade de Fortaleza. Fortaleza CE, Brazil
Brazil Instituto do Câncer do Estado de São Paulo, São Hospital de Saúde Mental Professor Frota Pinto.
Universidade Estadual do Ceará. João Pessoa PB, Paulo SP, Brazil Fortaleza CE, Brazil
Brazil Hospital das Clínicas da Faculdade de Medicina da
USP, São Paulo SP, Brazil rianvilar@edu.unifor.br
juliofr04@hotmail.com
renan.rosenstein@hc.fm.usp.br Case Presentation: A 61-year-old man was admitted to
Case Presentation: A patient with a previous diagnosis a psychiatric inpatient center with a manic episode. He
of Down Syndrome (DS) was sent to a stroke referral Case Presentation: A 78-year-old female presented with had a diagnosis of hypertension and a past history of
Hospital ten days after a stroke. At the initial neurological subacute and progressive lower limb weakness and numb- a short-lived mild depressive episode 4 years ago with
examination, he manifested a state of alertness, in addi- ness, urinary retention, confusion and weight loss. Her spontaneous resolution. The patient had been in his
tion to being uncooperative, aphasic, and unresponsive past medical history included hypertension, trigeminal usual state of health until 15 days before the admission,
to commands. He presented facial paralysis, hemipare- neuralgia and meningioma resection eleven years before when he presented mood alteration, labile affect, dis-
sis, and hypoesthesia on the left, indicating a suspected presentation. Upon admission, neurological examination inhibition, grandiose ideas and persecutory delusions,
cerebral infarction. A Cranial Computed Tomography showed impaired attention and executive dysfunction, noted by family and coworkers. At that point, the patient
(CT) showed hypodensity in the right middle cerebral along with asymmetric spastic paraparesis (worse on the was admitted to the inpatient setting. The neurological
artery (MCA) and Ecass 1 transformations. Due to the right leg) and a dermatomal sensory deficit level at T4. exam showed preserved strength, sensitivity, coordina-
ictus having occurred ten days before admission, the Laboratory findings were noticeable for pancytopenia. tion and gait. Laboratory screening tests with serologies
approach remained conservative at the subacute stroke Patient underwent neuroimaging. Brain MRI revealed showed no alterations. The brain magnetic resonance
division. On CT angiography, there were occlusions in M1 multiple areas of T2/FLAIR hyperintensities in addition imaging showed signs of subacute ischemic event in the
segments of MCA and Moyamoya pattern, manifested by with restricted diffusion in the parasagittal gray and white posterior branch of the left internal capsule and mild
stenosis of the left internal carotid artery and apparent matter bilaterally, overlapping with internal and external microangiopathy. A cerebral magnetic resonance angiog-
hypertrophy of the collateral lenticulostriate arteries. watershed zones of the frontal, parietal, and occipital raphy showed no hemodynamically significant stenosis.
The patient evolved clinically and hemodynamically lobes, indicating an ischemic etiology. Spine MRI showed A treatment with lithium 900mg and olanzapine 5mg was
stable, but still alert, uncooperative, and aphasic. After hyperintensities on T2-weighted sequences, predom- started with great improvement. When the patient was
discussion with a neurologist, the patient was discharged inantly in the central and upper segments of thoracic discharged after 3 weeks, he returned to work and kept
from the Hospital with follow-up care in health services. spinal cord, also consistent vascular insult. CSF exam- follow-up on an outpatient basis. Discussion: Mania is
Discussion: Moyamoya disease represents an arteriopathy ination was normal. Because of pancytopenia, a bone characterized by affective disturbances and can manifest
with stenosis in segments of the internal carotid arteries, marrow biopsy was obtained, which confirmed an intra- with various symptoms, including increased talkativeness,
with hypertrophy of collateral vessels. Former character- vascular non-Hodgkin B cell lymphoma. Chemotherapy flight of ideas, racing thoughts, abnormal distractibility
ized as an idiopathic condition, it’s now associated with was initiated afterwards and patient is showing gradual and increased energy. It is reported to be an uncommon
genetic disorders and a higher risk of cerebral infarction, neurologic improvement during follow-up. Discussion: manifestation after stroke. In this setting, its frequency
which is relevant in this case presentation, due to the Intravascular large B cell lymphoma (IVLBCL) is a rare is < 1%, compared with depression and other mood dis-
aggravating factor related to DS. Such an etiological cor- subtype of extranodal large B-cell lymphoma with an orders. Post-stroke mania should be considered in any
relation for arteriopathy resides in the fact that the gene for aggressive course, which may affect multiples organs, manic patient who has concomitant neurological deficits
the alpha chain of collagen VI is located on chromosome including central nervous system. Its main feature resides and is older than expected for the onset of primary mania.
21, as well as the genes of other proteins that interfere with on the fact that tumor cells have a confined proliferation This presentation is most common when lesions occur in
arterial physiology. Moyamoya diagnosis in patients with within the lumen of small and medium-sized vessels, dif- the right cerebral hemisphere, specially in the thalamus
DS occurs at an average age of 9.7 years. The previous ferentiating IVLBCL form other forms of lymphoma. Even and caudate nucleus, although left-sided lesions have
diagnosis helps avoid the commonly observed course though any organ may be involved, skin and CNS are the also been reported. From a review of the literature, it was
of recurrent stroke. Anticoagulant preventive measures most commonly affected sites. Most frequent neurologi- noted that most of the data about this topic focuses on
can also be implemented to reduce complications. In cal symptoms are cognitive impairment (encephalopathy case presentation. Final Comments: The case presented
the report, the patient with DS did not receive follow-up and dementia) and myelopathy with neuroimaging show- is a rare episode of mania after ischemic stroke with
care by health professionals until the age of 31, when he ing infarct-like lesions due to vessel occlusion by tumor unusual involvement of the left internal capsule which
presented with the acute condition of arteriopathy that cells. IVLBCL poses a diagnostic challenge because of its emphasizes the importance of neuroimaging in the first
culminated in stroke, revealing the risks of late observa- protean manifestation and lack of specific laboratory and episode of mania. A longer follow-up is needed to assess
tion of the disease. Final Comments: Due the evidence imaging findings. In addition, PET-CT is usually negative the recurrence of symptoms and, in this case, distinguish
of severe conditions after the correlation between Down because this type of tumor tends to remain intravascular. between causality and correlation. Further studies are
Syndrome, Moyamoya disease and the reported stroke, Final Comments: The importance of this case lies in the required to better understand this association of events.
the greater dissemination of knowledge associated with fact that the combination of concomitant brain and spinal
the case is essential to health professionals is necessary cord scattered infarction with systemic findings (pancy-
to define the appropriate conduct and treatment. topenia and constitutional symptoms) must include this Doença Cerebrovascular
rare, underecognized disease in the differential diagnosis.

Doença Cerebrovascular
Doença Cerebrovascular

42
XXX Congresso Brasileiro de Neurologia

TL 1106319 TL 1106329 TL 1106395

SUBARACHNOID HEMORRHAGE DUE BLUE TOE SYNDROME AND STROKE: A CASE PRESENTATION MEDULLARY
TO SPONTANEOUS RUPTURE OF AN CASE PRESENTATION ON SIMULTANEOUS VASCULAR ACCIDENT DUE TO DISSECTION
ARACHNOID CYST: CASE PRESENTATION CARDIOEMBOLISM OF THE DESCENDING BRANCH OF THE
ABDOMINAL AORTA ARTERY HOLY HOUSE OF
Ana Lucia de Carvalho Mello, Carolina F. V. Miranda, Karen ISabelle Pontes Duran Bottaro, Vanessa
Saulo Ramos RIbeiro, Evelyn Pacheco, Mariana Bernardo Nunes Lepre, Rodrigo Teixeira Pellini, MERCY OF SANTOS
Okada, Daniel C Bezerra, João Jose Freitas de José Elias Makaron Neto, Omar Gurrola Arambula, Vanessa Moraes Rossette, Thiago da Cruz Marques,
Carvalho, José Carlos Teixeira Gomes, Gustavo Heloisa Ferraz Troijo Leite, João Marcelo Borba Natália Figueiredo Miranda, Beatriz Medeiros
Kuster, Renan Domingues Leite, Maria Ludmila Setti Aguiar Moro, Nathalia Corrêa, Raphael Palomo Barreira, Clara Kimie
Raquel Cristaldo Alvarez, Gabriel Pereira Braga Miyahira, Guilherme Rocha Storte, Daisi Carolina
Samaritano Paulista São Paulo SP, Brazil
Américas Serviços Médicos. Rio de Janeiro RJ, Brazil Associação Beneficiente Santa Casa de Campo Fritz Jordão
Amil Assistência Médica, São Paulo SP, Brazil Grande. Campo Grande MS, Brazil Santa Casa da Misericórdia de Santos. Santos SP.
Américas Serviços Médicos. Fortaleza CE, Brazil Brazil
Américas Serviços Médicos, São Paulo SP, Brazil karen_duran_@hotmail.com
vanessarossette7@hotmail.com
sauloramosribeiro@gmail.com Case Presentation: A 76 years old male, with chronic atrial
fibrillation (AF), CHA 2 DS 2 -VASc: 8, was admitted with Case Presentation: A 65 years- old, man, previously dia-
Case Presentation: A 37-year-old female patient seeks sudden homonymous left hemianopsia andmild lefthe- betic and hypertensive. Admitted to the intensive care unit,
medical care at a private Hospital in São Paulo. She was miparesis (NIHSS 2) associated with fixed cyanosis of the with sudden back pain, associated with weakness and
referring sudden, pulsating headache, with photophobia first left pododactile. A diagnosis of acute right posterior paresthesia of the lower limbs. During Hospitalization,
and phonophobia. The neurological examination was cerebral artery occlusion was made concomitance with it evolves with urinary retention, paraplegia and lower
unremarkable. As the only previous medical history, she acute occlusion of the dorsal digital artery.The patient limb anesthesia. On admission, an echocardiogram was
had deep venous thrombosis in 2014, since then she was had previously suspended warfarin treatment30 days performed in atrial fibrillation rhythm with diffuse hypoki-
irregularly using of Warfarin. She underwent CT and MRI before the event. Brain magnetic resonanceshowed a nesia. Magnetic resonance imaging of the skull with areas
of the skull which showed enlargement of the bilateral ret- definite ischemic area in the right occipital, left medial of restriction to diffusion in the occipital, periventricular
rovermian and retrocerebellar cerebrospinal fluid space, occipital, and left cerebellar region. A transesophageal and corona radiate regions and of the thoracic spine with
with thin membranes/septations inside, and heteroge- echocardiogram (TEE) showed a mobile thrombus in the signal alteration in the anterior and central situation of
neous content, with deposition of hemoglobin degradation left atrial appendage (LAAT)measuring approximately the spinal cord, in the T4-T7 region, which correspond
products. Such findings are consistent with a retrovermian 1.1 cm x 0.8 cm. The vascular surgery team recognized to spinal infarction. To proceed with the investigation, a
arachnoid cyst with hemorrhagic content. No abnormal- systemic embolization to the distal arterial bed, and only chest angiotomography was requested, which showed
ities were found in cervical abd intracranial arteries with medical therapy with anticoagulants was recommended. thoracoabdominal aortic dissection (type IIIB), with
angiotomography. The prothrombin time was normal and Abdominal ultrasound revealed discrete atheroma plaques thrombus in the descending aorta and bilateral pericardial
international normalized ratio (INR) was 1. The patient was inthe abdominal aorta without any other signs of embolic and pleural effusion. After the examination findings, the
discharged after 2 days of Hospitalization with improve- eventsat different sites. The warfarin anticoagulation was patient was transferred to a referral Hospital and surgical
ment in the headache. Discussion: Arachnoid cysts are resumed, but the patient presented a labile INR, soanti- correction of the dissection. Discussion: Aortic artery
collections of fluid located between the meningeal mem- coagulation was switched to dabigatran with good evolu- dissection is the rupture of the aortic intima that allows
branes. They are congenital and are formed due to a valve tion. Discussion: AF is a significantcause of systemic and blood to penetrate the aortic wall and promote the dis-
defect in the arachnoid membranes, which facilitate the especially brain embolization.Dislodgment of thrombus continuity of vascular layers. The event may be secondary
passage of cerebrospinal fluid into its interior, leading to from the left atrium into the circulation is one possible to the presence of an aortic aneurysm, characterized by
the formation of the collection. Most arachnoid cysts are mechanism that explainsembolic episodes in atrial fibril- irreversible dilation of the vessel wall, whose predisposing
an incidental finding. About 1 to 2% of the population has lation patients. Althoughblue toe syndrome was initially factors are smoking, hypertension, and atherosclerosis,
a subarachnoid cyst and symptoms can be seen when described in patients with microemboli from proximal congenital, genetic and infectious diseases. When dissec-
the cyst increases in size or bleeds inside the cyst. Thus, atherosclerotic lesions, an annual rate of 0,4%/yr was tion occurs, there is a risk of complications, with ischemic
the case presented is a rare condition. Final Comments: documented in atrial fibrillation patients in antiagrega- phenomena being observed upstream and downstream
The patient presented a headache with a warning sign – tion therapy. Prevention of stroke and systemic embolic of the initial orifice, such as spinal cord ischemia, a rare
tunderclap headache – and was investigated aiming to events had been successfully demonstrated with DOACs complication with significant neurological symptoms
rule out a subarachnoid hemorrhage which is frequently use in AF patients, but in some series, the prevalence of and a high rick of sequel. To report a case of medullary
associated with aneurysm rupture. The neuroimaging LAAT in patientsanticoagulated for at least three weeks vascular event secondary to abdominal aortic dissec-
findings disclosed a rare complication of the arachnoid with DOACs can be as high as 5,8% depending on the TEE tion, as a differential diagnosis of acute flaccid paralysis
cyst. The most important risk factor for arachnoid cyst screening strategy used a similar rate in anticoagulation using data from medical records. Final Comments: The
hemorrhage is trauma. In the case described, there was with warfarin. Final Comments: We describe a simulta- case in question raises the discussion about a rare type
no history of trauma. Another possibility would be the neous blue toe syndrome and ischemic stroke secondary of complication resulting from abdominal aortic dissec-
use of an anticoagulant; however, this association was to multi-source embolic sourcestreatedwith anticoagu- tion, spinal ischemia, which must be remembered and
ruled out with normal prothrombin time. The risk of lation with dabigatran. DOACs use can be an option in investigated in cases of acute flaccid paralysis, aiming
intracystic hemorrhage in people using anticoagulants is cases like these, particularly in patients com labile INR at early diagnosis and treatment making it possible to
unknown and so far there is no contraindication to their or contraindications to warfarin use. reduce neurological sequel.
use in this population.

Doença Cerebrovascular Doença Cerebrovascular


Doença Cerebrovascular

43
XXX Congresso Brasileiro de Neurologia

TL 1106397 TL 1106403 TL 1106427

BOTULINUM TOXIN AS TREATMENT FOR A CASE OF STROKE CORRELATED WITH IMPACT OF COVID-19 PANDEMIC ON THE
VASCULAR HEMICHOREA–HEMIBALLISM: THREE TERRITORY SIGN IN A PATIENT WITH NUMBER OF THROMBOLYSIS PERFORMED
A CASE PRESENTATION TROUSSEAU SYNDROME DURING THE CLOSURE OF THE STROKE UNIT
IN A NEUROLOGICAL REFERRAL CENTER
Daniel Alves de Oliveira, Arthur Cesário de Holanda, Adelmar Marinho Neto, Daniel Monte de Andrade
Vitor Maia Arca, Raphaelly Ribeiro Campos, João Gervásio, Júlio Farias Rangel, Miguel Vieira de Vitor Maia Arca, Maria Eduarda da Costa Brandão
Pedro Matos de Santana, Luísa Couceiro de Almeida, Yan Gondim de Sousa Justino, Monique Evelyn Mendonça do Nascimento,
Albuquerque Macêdo, Ana Rosa Santana, Geovane Pedro Lucas Negromonte Guerra, Ana Dolores
Gomes Silva, Eduardo Sousa de Melo, Clélia Maria Hospital Regional do Sertão Central. Fortaleza CE,
Firmino Santos do Nascimento
Ribeiro Franco Brazil
Universidade Estadual do Ceará. Fortaleza CE, Hospital das Clínicas da Universidade Federal de
Hospital das Clínicas da Universidade Federal de Brazil Pernambuco. Recife PE, Brazil
Pernambuco. Recife PE, Brazil Universidade Estadual do Ceará. João Pessoa PB, Faculdade Pernambucana de Saúde. Recife PE,
Brazil Brazil
daniel_oliveira_@live.com
Hospital da Restauração. Recife PE, Brazil
juliofr04@hotmail.com
Universidade de Pernambuco. Recife PE, Brazil
Case Presentation: A 55-year-old male patient started
with altered sensitivity in the right hemibody seven days Case Presentation: A 78-year-old male patient, hyper- vitorm_arca@hotmail.com
before admission, in addition to abnormal movements tensive and former smoker, was admitted to a tertiary
in the right arm, starting after waking up, with no appar- Hospital with suspected ischemic stroke, presented with Case Presentation: With the Covid-19 pandemic, a new
ent triggers and which improved during sleep. He had a left upper limb plegia and left lower limb paresis for three scenario and a new demand has become a priority in
previous history of unspecified cardiac arrhythmia, with days before the admission, with mild dyslalia and respon- health services globally, causing impact, restructuring
irregular use of propafenone, acetylsalicylic acid and rosu- sive to verbal commands. Cranial Computed Tomography and even closing of stroke units around the world. (1) All
vastatin. Physical examination showed abnormal move- (CT) revealed gliosis and myelin rarefactions, and Cranial these measures had an impact on the care of patients with
ments on the right side, sparing the face and worse in the Magnetic Resonance Imaging (MRI) showed randomly acute stroke, both in developed and developing coun-
proximal part of the right upper limb, with low frequency, distributed foci of ischemic insult in three brain regions. tries. (2) The neurology emergency unit at Hospital da
high amplitude, arrhythmic and erratic, characteristics Four days after admission, the patient reported worsening Restauração in Recife/Pernambuco, one of the services
of hemichorea-hemiballismus. Biochemical and sero- epigastric pain with irradiation to the spine. A CT scan with the highest flow of care for patients with acute stroke
logical tests were normal. Brain MRI showed ischemic of the abdomen/pelvis indicated a primary solid-cystic in the Northeast, also had to readjust to the new scenario
stroke with areas of subacute ischemia in the occipital lesion, involving the body of the pancreas and bilateral imposed. Objective: To observe the impact of the clo-
lobe and left occipitotemporal transition. Drug treatment renal cysts. USG indicated secondary neoplastic liver sure of the stroke unit at Hospital da Restauração in the
was performed with high doses of Haloperidol, Valproic implants. The presence of cerebrovascular lesions in three period from May 2020 to October 2021 and to compare
Acid and Clonazepam without satisfactory clinical con- regions suggested a diagnosis of Trousseau Syndrome the difference in the numbers of thrombolysis performed
trol. Botulinum toxin type A was applied with 300UI to the (TS) associated with a neoplastic condition. The patient before closing (January 2020 to April 2020) and after the
right trapezius muscle and 200UI to the right latissimus remained clinically stable and it was decided to maintain reopening, from November 2021 to April 2022, with the
dorsi muscle, with considerable improvement in move- the clinical treatment of the stroke, and he was assigned numbers of thrombolysis performed in the 18 months in
ments after thirty days and with preservation of strength for a follow-up at the oncology department. Discussion: which the unit remained closed. Methods: An observa-
in all four limbs. Discussion: Hemichorea-hemiballismus TS is characterized as a spectrum of pathophysiological tional, retrospective, cross-sectional study was performed
syndrome is one of the main movement disorders caused disorders associated with neoplastic conditions that can by analyzing the medical records of patients undergoing
after a stroke. The main treatments described are typical cause microthrombi by mucin, P-selectin, and L-selectin chemical thrombolysis, in a period of 28 months, which
and atypical antipsychotics, antiepileptics and presyn- and thrombi induced by the action of tissue factors. corresponded to the period from January 2020 to April
aptic dopamine depletors. The use of botulinum toxin Thrombotic events may precede the diagnosis of visceral 2022 in the neurological emergency of the Hospital da
is well established in some hyperkinetic movement dis- malignancy or arise concomitantly with the tumor, indi- Restauração – Recife. Results: The total number of patients
orders such as dystonia, myoclonus and tics; its use in cating a hypercoagulable state with a tendency to mul- undergoing chemical thrombolysis from January 2020 to
hemichorea-hemiballismus is still poorly studied, with tiple thromboembolisms. Ischemic stroke is character- April 2022 was 153. In 2020, 79 patients were thrombolysed
little evidence found in the literature. Our case presented ized by the occlusion of blood vessels that carry out the and in 2021 there were 42 patients. In these 28 months of
a good response similar to the few cases described in the vascularization of the Nervous System (NS), with active study, 10 months corresponded to the period of operation
literature. Final Comments: The use of botulinum toxin cancer being a risk factor for this clinical condition. In of the unit, with closure in May 2020 and reopening in
in the treatment of hemichorea-hemiballismus can be TS, the excess of embolic signals in patients with active November 2021. The period of operation of the stroke unit
effective in patients who are refractory to drug treatment. neoplasia explains the lesion in three territories associ- corresponded to 35% of the period analyzed. While the
ated with stroke. In the report, the 78-year-old patient stroke unit was open, 60% of the Hospital’s thrombolysis
was diagnosed with solid-cystic lesions in the pancreas were performed throughout the analyzed period. With the
Doença Cerebrovascular with an MRI indicating multiple foci of ischemic insult reopening of the stroke unit in November 2021, 48 patients
and pattern in three territories of cerebrovascular com- have already undergone chemical thrombolysis in the first
promise. Final Comments: Due to the association of TS 4 months of 2022, corresponding to an absolute increase
with a clinical condition of hypercoagulability that favors of 342.8% over the previous year. Final Comments: Stroke
thromboembolic occurrences, it is necessary for health units around the world guarantee better care for patients
professionals to identify the etiology of ischemic stroke with acute stroke phase. (3) They promote better func-
episodes of neoplastic origin more quickly and accu- tionality outcome in all acute-phase patients, regardless
rately. Familiarization with such vascular injuries may of whether they were undergoing thrombolytic therapy.
allow conduct that leads to a better prognosis for patients. (4) In this study, we observed the negative impact on the
amount of chemical thrombolysis performed, at a time
when the Covid 19 pandemic reached the worst rates in
Doença Cerebrovascular the state Pernambuco.

Doença Cerebrovascular

44
XXX Congresso Brasileiro de Neurologia

TL 1106439 TL 1106444 TL 1106447

STROKE MIMIC THROMBOLYSIS IN A OPALSKI SYNDROME IN LEFT BULBAR THROMBOLYSIS IN A LARGE UNRUPTURED
PATIENT WITH PARTIAL ABDOMINAL ISCHEMIC STROKE: CASE PRESENTATION INTRACRANIAL ANEURYSM: A CASE
ANEURYSM THROMBOSIS PRESENTATION
Daniel Alves de Oliveira, Vitor Maia Arca, Arthur
Luiza Alves Monteiro Torreão Villarim, Alex Tiburtino Cesário de Holanda, Raphaelly Ribeiro Campos, Filipe Nolasco de Souza e Silva, Mariana Soares
Meira, Matheus Gurgel Saraiva, Rafael de Souza Ana Rosa Santana, Geovane Gomes Silva, Eduardo Pinheiro, Maria Clara Carvalho Silva de Amorim, Ana
Andrade, Paulo Antônio Farias de Lucena, Mylena Sousa de Melo Flávia Souza Freire da Silva, Karla Oliveira Couto,
Gaudêncio Bezerra, Jeanina Cabral Dionizio, Rayanderson Nunes da Gama, João Gabriel Teixeira
Hospital das Clínicas da Universidade Federal de
Francisco Anderson de Sá Carvalho, Arthur Felipe Mota, Renan Carvalho Castello Branco, Daniel
Pernambuco. Recife PE, Brazil
Barbosa Vasconcelos, Daniel Vicente de Siqueira Lordelo San Martin, Pedro Antonio Pereira de Jesus
Lima Junior daniel_oliveira_@live.com
Hospital Santa Izabel. Salvador BA, Brazil
Hospital Metropolitano Dom José Maria Pires. João Hospital Geral Roberto Santos. Salvador BA, Brazil
Case Presentation: A 50-year-old male patient started ten
Pessoa PB, Brazil
days ago with intense headache in the left frontal region, filipe.nolasco95@gmail.com
Universidade Federal da Paraíba. João Pessoa PB,
tightness, with nausea and vomiting. Symptoms wors-
Brazil
ened after four days, with loss of left lower limb strength Case Presentation: A 17-year-old patient, with a previous
luizavillarim@hotmail.com and vertical diplopia. On physical examination, Horner diagnosis of aneurysm in the right middle cerebral artery
syndrome on the left, with miosis and ptosis; skew devi- (4.7x2.4x1.9 cm), presented to our Hospital complaining of
Case Presentation: MGFM, 73 years old, female, with ation with hypotropia in the left eye; complete left spastic sudden onset of left sided hemiparesis and hemihypoes-
normal pressure hydrocephalus (NPH) using a ventric- hemiparesis predominantly in the left lower limb; alter- thesia (NIHSS 14). On admission, computed tomography
uloperitoneal shunt (PVD), was admitted to the Hospital nating thermal and painful hemihypoesthesia was also hasn’t showed any ischemic parenchymal injury or intra-
due to spontaneous exteriorization of an abdominal observed, alternating with involvement of the left hemiface cranial bleeding. Computed tomographic angiography
catheter. During the investigation, a partially throm- and right limbs. Brain MRI showed an ischemic stroke in has confirmed diffusely irregular aneurysmal dilatation
bosed fusiform aneurysm was observed in the infrarenal the lateral region of the left medulla. Discussion: Opalski throughout its M1 segment of right middle cerebral artery
segment of the abdominal aorta, with an extension of 10 syndrome is a rare variant of lateral bulbar infarction, char- associated with occlusion of the same vessel and the A1
cm and a diameter of 8.0x6.2 cm, with characteristics acterized by the triad of ipsilateral hemiparesis, Babinski segment of anterior cerebral artery. He underwent chem-
of instability. Indicated aortic endoprosthesis. While sign and contralateral hypothermalgesia. Although our ical reperfusion therapy with Tenecteplase 0.25mg/kg,
waiting for the procedure, the patient developed vomit- patient did not have Babinski’s sign, he did have spastic- with excellent outcome, improving the NIHSS to 5 in a
ing and sudden aphasia, presenting a score of 11 on the ity, which corroborates the involvement of the pyramidal few hours. Arteriography confirmed a dissecting aneu-
National Institutes of Health Stroke Scale (NIHSS). Cranial tract. Lateral bulbar lesions may present with diplopia, rysm of right MCA. Patient has maintained neurological
tomography presented an ASPECTS of 10. Intravenous nystagmus, and bias deviation due to involvement of the improvement being discharged with NIHSS 3 and an
chemical thrombolysis with alteplase was performed, vestibular pathways. Final Comments: In cases of stroke elective evaluation of aneurysm approach. Discussion:
the post-procedure NIHSS score was 8, with no other with hemiparesis, diplopia and asymmetric deviation, The major concern with intravenous thrombolytic ther-
complications. Ultrasound study of the cervical arteries with contralateral sensory changes, Opalski’s syndrome apy is the presence of symptomatic intracranial bleeding.
and echocardiogram were normal. Magnetic Resonance due to lateral bulbar involvement should be considered. The presence of aneurysmal formation is not an absolute
Imaging of the Skull did not show an area of previous contraindication for thrombolysis, however, due to the
ischemia, configuring, therefore, a mimic stroke of a increased risk of bleeding, there is a tendency to choose
patient with hydrocephalus of undetermined origin. The Doença Cerebrovascular not to proceed with the therapy. Final Comments: Most
patient was discharged with a NIHSS of 2. Three months guidelines and studies point to a reasonable safety of
later, the endoprosthesis implantation surgery was suc- thrombolysis in aneurysms < 10 mm, but there is still little
cessfully performed. Discussion: Intravenous thrombol- information about aneurysms with larger dimensions.
ysis is associated with an increased risk of intracranial
aneurysm rupture, resulting in intracranial hemorrhage.
The 2018 American Heart Association (AHA) guidelines Doença Cerebrovascular
suggest that thrombolysis is safe in unruptured intra-
cranial aneurysms less than 10 mm in diameter. There is
no recommendation regarding extracranial aneurysms.
Experience with endovascular thrombectomy in patients
with thrombosed intracranial aneurysms causing acute
stroke is also limited. Proposed mechanisms of stroke
related to unruptured aneurysms include thrombosis
with distal embolization, extension of the thrombosis
to the parent artery, or compression of the parent artery
by the aneurysm. The risk of symptomatic intracranial
hemorrhage in people with stroke mimic is low. Final
Comments: It is unclear whether thrombolysis is safe in
extracranial aneurysm cases, future case series may be
useful to answer the question. Furthermore, in cases of
ischemic stroke within the therapeutic window, intrave-
nous thrombolysis should be performed, except in known
contraindications, because even in cases of stroke mimics
the risk of intracranial bleeding is low.

Doença Cerebrovascular

45
XXX Congresso Brasileiro de Neurologia

TL 1106465 TL 1106480 TL 1106516

INTERNAL CAROTID ARTERY DISSECTION IN LEFT ATRIAL APPENDAGE MORPHOLOGY “CAROTID WEB” AN IMPORTANT ETIOLOGY
THE POSTPARTUM PERIOD PRESENTING AS A RISK FACTOR FOR OF STROKE IN YOUNG PEOPLE: CASE
STROKE IN A YOUNG PATIENT IN THE PRESENTATION
Raphaelly Ribeiro Campos, Eduardo Sousa de
Melo, Geovane Gomes Silva, Vitor Maia Arca, Daniel ABSENCE OF ATRIAL FIBRILLATION:
Marcelle Sanjuan Ganem Prado, Keila Narimatsu,
Alves de Oliveira, Arthur Cesário de Holanda, Ana A CASE PRESENTATION Alex Lederman, Annelise Akemi Higa Lee, Darah
Rosa Santana, Luziany Carvalho Araújo, José Luiz Fontes da Silva Assunção, Francisco Tomaz
Gabriella Maria Martins Favero, Samia Talise El
Miranda de Inojosa, Victor de Moraes Chagas Meneses de Oliveira
Horr de Moraes, Michelle Zonkowski Ribas, Marcos
Hospital das Clínicas – HC – UFPE Christiano Lange, Viviane de Hiroki Flumignan Santa Casa de Misericórdia de São Paulo, São Paulo
Zétola, Valéria Cristina Scavasine, César Minoru SP, Brazil
camposraphaelly@gmail.com Toita Koga, Alexandre Henrique Scheibe, Guilherme Hospital Albert Einstein, São Paulo SP, Brazil
Egidio Rocha Scatola, Andre Luiz Canteri
Case Presentation: Caso 1 – A 36 year-old woman, on the cellesanjuan@yahoo.com.br
21st day after vaginal delivery, presenting a sudden episode Hospital de Clínicas da Universidade Federal do
of complete left hemiplegia, hemi-hypoesthesia and tactile Paraná. Curitiba PR, Brazil Case Presentation: J.A.T.T, male, 44 years old, has pro-
and auditory hemineglect. She had moderate-intensity gabriellammf@yahoo.com.br thrombin II mutation. Attended at Albert Einstein Hospital
right hemicranial headache started 12 hours before the on April 21, 2022 with dysarthria lasting 5 minutes one day
event. She was admitted to the emergency department before admission. He denies motor or sensory deficits.
Case Presentation: A 43-year-old woman treated with
1 hour and 17 minutes after the ictus (NIHSS 11), under- On admission, cranial CT and AngioCT identified areas
losartan, aspirin, and simvastatin had a medical history
going a CT scan of the skull with ASPECT 10 and then of recent infarction in the subcentral and right inferior
of stroke two years ago without etiological investigation,
submitted to venous thrombolysis, with no neurological frontal gyrus associated with linear and irregular filling
in addition to using a pacemaker for total atrioventric-
improvement. Subsequently, she performed a new cranial failure adhered to bulb wall/origin of right internal carotid
ular block since the age of 20, without atrial fibrillation
CT showing right frontoparietotemporal hypodensity and artery (ICA), suggestive of carotid membrane with associ-
(AF) documented in previous exams. She arrived at the
extracranial CT angiography showing signs of dissection ated thrombus, typical of “carotid web”. The findings were
emergency department with sudden hemiparesis and
of the right internal carotid artery and irregular proximal confirmed with MRI and AngioMRI with vessel studies that
hypoesthesia on the left side of her body at 10: 30pm.
third on the left internal carotid artery, with focal ecta- showed occlusion of the M4 segment of the right middle
On examination, she had a National Institutes of Health
sia. Case 2 – A 33-year-old woman, on the 14th day after cerebral artery. Angioplasty with stent was performed
Stroke Scale (NIHSS) score of five, a blood pressure of
cesarean delivery, had a sudden episode of complete left on April 24, without complications. The patient evolved
172/85mmHg and a capillary blood glucose level of 98mg/
hemiplegia, hemi-hypoesthesia and hemineglect for all without changes in the neurological examination, using
dL. Cranial computed tomography (CT) performed at 1:
modalities. She had a mild headache with progressive full anticoagulation. Discussion: “Carotid web” (CA-Web)
00am was normal, and thrombolytic therapy was initi-
onset. She was admitted to the emergency department is defined as thin, linear, membrane that extends from
ated at 1: 30am with full recovery of deficits. On subse-
with 5 hours after the ictus (NIHSS 8), with CT of the the posterior aspect of the internal carotid artery bulb
quent examination, she had an elevated N-terminal pro
head was normal. Under investigation, she performed a into lumen, located just beyond the carotid bifurca-
B-type natriuretic peptide (NT-proBNP) of 1379.9pg/mL.
new head CT 48 hours after the event, with right fronto- tion. Histopathologically is characterized by fibroelastic
Carotid and vertebral Doppler findings were unremark-
parietotemporal hypoattenuation and extracranial CT thickening of the arterial intima. Prevalence is estimated
able. Electrocardiography revealed sinus rhythm, and
angiography with signs of bilateral internal carotid artery ranging from 9.4% to 37% in patients with cryptogenic
pacemaker telemetry showed no evidence of arrhythmia
dissection. Discussion: Cervicocerebral arterial dissection stroke. Studies showed average age of 41 years and an
in the previous four months. Transthoracic echocardiog-
is one of the most frequent causes of ischaemic stroke in NIH of 10.5 in patients with this vascular disorder. Thus,
raphy detected an enlarged left atrial appendage (LAA)
young and middle-aged adults. (1) However, postpar- symptomatic CA-Web patients are younger and likely to
and an enlarged left atrium (LA). Transesophageal echo-
tum cervicocerebral arterial dissections are much rarer, have more severe strokes compared to ischemic stroke
cardiography did not reveal any intracavitary thrombi
accounting for only 6% of spontaneous arterial dissection patients in general. Among the non-invasive diagnostic
or vegetation. Cardiac CT angiography confirmed the
in women under the age of 50. Headache and neck pain methods, there is AngioCT which Ca- Web is usually seen
increased LA and LAA, the latter with a non-chicken-wing
are the most common symptoms in postpartum cervical as a thin, smooth, membrane-like intraluminal filling
morphology and without evidence of thrombi inside. The
carotid and vertebral artery dissections, accounting for defect along the posterior wall of the ICA bulb on oblique
patient was discharged after full anticoagulation at the
60% to 90% of cases. (2) The pathophysiology of cervical sagittal images. Treatment is based on secondary preven-
Hospital with prescription of dabigatran, atorvastatin,
artery dissection appears multifactorial, with evidence tion of stroke, as well as an individualized anticoagulation
amlodipine and outpatient follow-up in the neurology and
suggesting environmental and genetic contributions. decision, as the risk of recurrent ischemic events is likely
cardiology departments. Discussion: The LAA originates
(3) Final Comments: We describe two cases of isch- to be higher in antiplatelet therapies. Surgical resection
from the main body of the LA and represents one of the
aemic stroke secondary to carotid artery dissection in can be considered, especially for patients with thera-
major sources of cardiac thrombus formation. Based on
the puerperium. Therefore, severe headache and neck peutic failure, and carotid artery stents are alternative
imaging findings, LAA morphology can be classified into
pain during this period should be rigorously evaluated in to surgical resection, with a lower risk of complications.
simple (chicken-wing morphology – most common and
view of the possibility of a poor prognosis mainly related Final Comments: CA-web is rare variant of fibromuscu-
with lower risk of thrombi) and complex (non-chicken-
to misdiagnosis and delay in treatment. lar dysplasia that affects the carotid artery intima. Recent
wing). Studies have suggested that complex LAA mor-
phology by itself may lead to stroke, even in the absence studies have associated this change as important etiology
of AF, probably because of lower flow velocities compared in young patients with cryptogenic ischemic stroke. The
Doença Cerebrovascular knowledge about radiographic findings, as well as man-
to simple LAA morphology. In addition, there is evidence
that ischemic stroke patients without known AF and with agement in the treatment of this etiology can prevent
elevated NT-proBNP levels may benefit more from antico- recurrence of ischemic vascular accidents, especially in
agulants than antiplatelet agents, explaining this patient the young population.
treatment. Final Comments: We present the case of a
patient without documented AF and with two strokes at
Doença Cerebrovascular
a young age, of probable cardioembolic etiology due to
an LAA of complex morphology.

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46
XXX Congresso Brasileiro de Neurologia

TL 1106522 TL 1106528 TL 1106553

CLINICAL CASE PRESENTATION DISSECTING STURGE WEBER SYNDROME TYPE 3: STROKE IN POSTERIOR CIRCULATION DUE
ANEURYSM OF THE MIDDLE CEREBRAL A CHALLENGING DIAGNOSIS TO ANTERIOR CIRCULATION STENOSIS:
ARTERY IN A THREE-MONTH-OLD INFANT: A CASE PRESENTATION OF VERTEBRAL
José Rosemberg Costa Lima Filho, Victor
CASE PRESENTATION Augusto Zanesi Maciel, Thiago Oscar Goulart, ARTERY ORIGIN IN INTERNAL CAROTID
Luan Monte Barroso, Marina Mayara Pereira, Lara Guimarães Queiroz Silva, Trajano Aguiar Matheus Costa Bessa, Robson de Sales Ferreira,
Cristiano Ribeiro Soares, Felipe Salvagni Pereira, Pires Gonçalves, Thire Baggio Machado Marazzi, George Nilton Nunes Mendes, Barbara Matos
Luis Gustavo Biondi, William Pegoraro Kus Frederico Fernandes Alessio Alves, Rui Kleber Vale Almeida Queiroz, Alessandra Braga Cruz Guedes
Martins Filho, Millene Rodrigues Camilo, Octávio de Morais, Pedro Arnaud Galdino, Ian Silva Ribeiro,
UNINOVAFAPI. Teresina PI, Brazil Marques Pontes Neto João Gabriel Dias Brasiliense Frota, Samuel Ranieri
Escola de Medicina da Pontifícia Universidade Veras, Fabricio Oliveira Lima
Católica do Rio Grande do Sul. Porto Alegre RS, Hospital das Clínicas da Faculdade de Medicina de
Brazil Ribeirão Preto. Ribeirão Preto SP, Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil
victor93@gmail.com Universidade de Fortaleza. Fortaleza CE, Brazil
luanmontebarroso@gmail.com
mcostabessa@gmail.com
Case Presentation: A 3-month-old female infant with a Case Presentation: 19 years-old female with obesity,
report of her first seizure in her life. On examination, he hypothyroidism, insulin resistance, and precocious Case Presentation: A 57 year old female admitted in
was in good general condition, normotensive anterior puberty presented, when at age of 5 years-old, a first epi- the emergency department presenting sudden onset of
fontanelle, without neck stiffness and afebrile. Normal sode of self-limited left hemiparesis following a new-onset left side weakness, drowsiness and vomiting. At neuro-
cardiopulmonary auscultation and abdomen without a headache. At the same age, she presented the first episode logical evaluation, hemiplegia and hemianopia were
palpable mass. Previous history: Late premature (34 weeks of seizure. Since then, she has persisted with a chronic observed. Angiographic study showed right common
and 2 days), vaginal delivery with meconium amniotic headache. Evaluation with brain MRI showed extensive carotid artery stenosis,internal carotid origin of right ver-
fluid. There was a prolonged Hospital stay due to proxi- right parenchymal calcification and venous-capillary tebral artery and right posterior cerebral artery occlusion.
mal jejunal atresia, and she underwent corrective surgery angioma with ependymal drainage. Although the patient Because of the delayed time between symptoms onset
on her 10th day of life. No history of infection or previous has had typical radiological findings for Sturge-Weber and Hospital admission, patient was not submitted to
trauma. Cerebral aneurysm embolization with detachable Syndrome (SWS), she imposed a diagnosis challenge reperfusion therapy with alteplase or endovascular treat-
platinum microcoils was chosen, using a total of 10 units. since she did not have the typical cutaneous port-wine ment. In 24 hours, massive edema caused by posterior
Control angiography showed aneurysmal occlusion with nevus. Annual follow-up MRI demonstrated progression ischemia was shown in a new CT scan. Then, the neu-
preserved filling of distal branches of the right MCA. The of cortical calcifications and increasing venous stasis due rosurgical team performed a craniectomy. Patient was
postoperative evolution was satisfactory, and the patient to venous-capillary malformation. Despite progression, discharged 1 month after Hospitalization, with several
was discharged on the 3rd postoperative day. Discussion: the patient maintains clinical and neurological stability. disabilities. Etiologic investigation was negative for car-
Cerebral aneurysms are rare in pediatric patients. In Discussion: Sturge–Weber syndrome is a neurocutaneous diologic sources. Discussion: The anatomical variations
relation to the adult population, its presentation differs syndrome characterized by a facial port-wine birthmark of cranial and extra cranial arteries are well established
in terms of incidence, size and location. Despite its low (capillary malformation) with abnormal blood vessels in in literature, and its current implication as risk factor for
incidence, 95% of the cases have subarachnoid hemor- the brain and the eye. It is a sporadic developmental dis- ischemic stroke. In this presentationed, the patient had
rhage as the initial manifestation, giving high morbidity order caused by somatic mosaic mutations in the GNAQ a stenosis in anterior circulation origin that had implica-
and mortality to patients. Among the risk factors of the gene. This syndrome is the third most common neurocu- tions in posterior circulation, not having a fetal pattern of
pediatric population, trauma and mycotic aneurysms, in taneous disorder after neurofibromatosis (NF) and tuber- posterior cerebral artery, causing a stroke. The right verte-
terms of acquired pathologies, deserve to be highlighted; ous sclerosis complex (TSC), but it is not associated with a bral artery had it`s origin at distal portion of extracranial
and family history and collagen diseases (Elher-Danlos, greatly increased risk of tumors. SWS exists as a spectrum internal carotid artery. Final Comments: It is very import-
Marfan syndrome, Type I neurofibromatosis and poly- ranging from the isolated brain involvement seen in about ant for the clinical practice of physicians to understand
cystic kidney disease) and congenital pathologies. In 10% of the cases, classified as type 3 SWS, to facial birth- the variability of the human anatomy, as they help with
the presented, we did not identify associated risk fac- mark associated with brain and eye involvement, type 1. the accurate diagnosis process. Rare variations can have
tors. Final Comments: Due to the lower incidence of Seizure is usually the first neurological manifestation in implications in the setting of cerebrovascular diseases.
intracranial aneurysms in the pediatric population, the the first or two years of life. Diagnosis of Sturge-Weber
scientific literature lacks robust evidence pointing to the syndrome is based on typical clinical symptoms and brain
best therapeutic option. Our case aims to contribute to magnetic resonance imaging (MRI) findings characterized Doença Cerebrovascular
the scientific literary scope as evidence of a successful by enhancing dilated leptomeningeal vessels associated
endovascular approach. with enlarged deep draining vessels and a choroid plexus
glomus. Final Comments: This case exemplifies the chal-
lenge of neurological diagnosis of SWS in the absence of
Doença Cerebrovascular port-wine stain facial angioma. In a patient with typical
symptoms such as focal seizure, cognitive impairment,
and metabolic disturbance presenting with radiological
findings, SWS type 3 should always be considered as a
possibility.

Doença Cerebrovascular

47
XXX Congresso Brasileiro de Neurologia

TL 1106570 TL 1106587 TL 1106612

ATYPICAL DISCOVERY OF A CEREBRAL SPONTANEOUS RUPTURED ACUTE ISCHEMIC STROKE IN A YOUNG


DURAL ARTERIOVENOUS FISTULA ARTERIOVENOUS MALFORMATION IN A PATIENT CAUSED BY BINGE DRINKING:
YOUNG PATIENT: A CASE-BASED UPDATE A CASE PRESENTATION
Esther Grangeiro Barreto, Alessandra Braga Cruz
Guedes de Morais, Morgana Feitosa de Queiroga, Gabriel Marinheiro Santos-Bezerra, Luís Eduardo Clauhan Williams Soares dos Santos, Jonata Ribeiro
Pedro Rubens Araújo Carvalho, Sarah Diógenes Oliveira Matos, Amandha Espavier Trés, Yasmin de Sousa, João Eudes Magalhães, Marcílio José
Alencar, George Nilton Nunes Mendes, Ana Silvia Silveira Cavalcante, Airton Ferreira Ponte, David Oliveira Filho, Maria Júnia Lira Silva, Rodrigo André
Sobreira Lima Verde, Dkaion Vilela de Jesus, Elison Lima Silva, Espártaco Moraes Lima Ribeiro, de Souza Araújo
Fabricio Oliveira Lima Paulo Roberto Lacerda Leal
Universidade de Pernambuco. Recife PE, Brazil
Hospital Geral de Fortaleza. Fortaleza CE, Brazil Universidade Estadual do Ceará. Sobral CE, Brazil
clauhanw@gmail.com
alebcgmorais@gmail.com gabrielmarbez@gmail.com
Case Presentation: A previously healthy 40-year-old man
Case Presentation: A 35-year-old male with an acute Case Presentation: A 17-year-old female has presented a from Recife, with a history of occasional binge drinking,
history of recurrent seizures and decreased level of con- history of severe frontoparietal headache for 6 days, refrac- presented sudden hemiparesis with hypoesthesia in the
sciousness associated with high fever of unknown onset. tory to analgesics. After admission, the patient evolved right side of the body after binge drinking. Associated with
He arrived at the emergency department with a Glasgow with worsening headache associated with drowsiness, the condition, he presented transient gait incapacity for
Coma Scale of 3 alongside automatism. Past medical nausea, persistent vomiting and inappetence. There are two minutes. He arrived at the emergency department
history was remarkable for traumatic brain injury ten no reports of comorbidities in the family and her parents in 2 hours with a NIHSS score of 2 points and a normal
years prior to these events and a seven-months his- are not consanguineous. Neurological exam revealed cranial CT scan, with no indication for thrombolysis. A
tory of progressive impaired gait. He was submitted to a mild paresis in left limbs. A skull Computed Tomography double antiplatelet therapy was started with acetylsali-
brain computed tomography (CT) which showed cortical (CT) showed the presence of an extensive frontoparietal cylic acid and clopidogrel. After 24 hours, the weakness
hypodensities, periventricular and symmetrical bilateral hemorrhagic infarction with brain mass effect, indic- in the right side remained, although of lesser intensity,
basal ganglia calcifications, venous engorgement due to ative of intracranial hypertension. In addition, a brain evolving with hyperreflexia and superficial hypoesthesia
an extensive arteriovenous fistula with brainstem involve- Digital Subtraction Angiography (DSA) was performed in the entire right hemibody. The brain MRI revealed an
ment. Lumbar puncture showed a high open pressure, and showed lobar intraparenchymal hemorrhage in the area of ??restriction to water diffusion in the left parietal
normal glucose levels, elevated white blood cell and pro- right temporal opercular region, with a estimated volume cortico-subcortical region associated with signal hyperin-
tein counts. Cerebrospinal fluid cultures were positive for of 48cm³, presenting circulating vasogenic edema and tensity on T2 and FLAIR, compatible with subacute isch-
gram negative cocci. Prolonged EEG showed moderate Spetzler-Martin grade 1. A right temporal arteriovenous emic vascular insult. Spectroscopy showed a reduction in
to severe diffuse symmetrical baseline activity disorgani- malformation was diagnosed. Discussion: Arteriovenous the levels of N-Acetyl aspartate (neuronal marker) and a
zation without epileptiform activity. The patient evolved malformations (AVMs) are the most dangerous of the slight increase in the peak of lipids/ lactate with features
with refractory non-epileptic seizures and healthcare-as- cerebrovascular malformations with the potential to suggestive of necrosis. Autoantibody research revealed
sociated infections and was then submitted to palliative cause intracranial hemorrhages and even epilepsy. The ANA 1: 640 (fine speckled). Serology for HIV, syphilis and
care. Intracranial dural arteriovenous fistulas (DAVFs) are most common clinical presentations are intracranial hepatitis were non-reactive. Magnetic resonance angi-
abnormal connections between dural arteries and dural hemorrhages, most commonly intraparenchymal, but ography showed no evidence of atherosclerosis or other
venous sinuses, meningeal veins or cortical veins and are they can also occur in the intraventricular or subarach- changes. New screening tests were performed for lupus,
responsible for 10-15% of all intracranial arteriovenous noid space. Bleeding into the subarachnoid space is thrombophilia and vasculitis, including a new ANA, with
malformations. It can be related to trauma, dural sinus common in superficial AVMs. In addition, seizures, focal normal results. The echocardiogram and 24h Holter did
thrombosis, venous hypertension, previous craniotomy deficits and headache are common. The Spetzler-Martin not show heart disease. The patient was discharged clini-
or mostly idiopathic aetiology. It is diagnosed preferably scale (1 to 5) classifies the risk of surgical removal of the cally stable after one month of Hospital investigation with
though conventional angiography, but CT and Magnetic AVM according to its size, location in eloquent areas or complete recovery of strength and sensitivity. Discussion:
Resonance (MR) can also be used, although they confer not, and whether deep venous drainage is present. High Ischemic stroke mainly affects people over 65 years of
lower accuracy. After suspicions of DAVFs, additional eval- scores correlate with greater risks of surgical morbidity age, with atherosclerosis as the main etiopathogenesis.
uation with CT or MR angiography is needed to confirm. and neurological deficits. Patient age is an important fac- Ischemic stroke in young individuals, below 45 years of
Most common locations in adults are at the cavernous, tor in the decision to treat brain AVMs, as patients with age, should lead to the investigation of secondary causes
sigmoid and transverse sinuses. Most symptoms start a longer life expectancy appear to accumulate a higher such as infections, autoimmune diseases, genetic condi-
around the fifth and sixth decades of life. The pattern lifetime risk of hemorrhage. Thus, therapy is best suited tions, neoplasms, heart diseases and drug abuse, includ-
and location of the DAVF venous drainage is associated for children and young adults. Brain DSA is essential for ing alcohol and smoking. In this case, the patient started
with its clinical presentation and severity. DAVFs are not treatment planning and postoperative follow-up, as it is the symptoms within 24 hours after the binge drinking
a risk factor for meningitis or other central nervous sys- possible to better obtain the configuration of the nidus, its episode, with no other etiopathogenic causes being evi-
tem (CNS) infections. Gait impairment is more associated relationship with the circulating vessels, and the location denced. Final Comments: In the context of stroke in a
with spinal DAVF or brainstem DAVF. Herein we present a of the draining or efferent portion of the cerebral AVM. young patient, an adequate investigation of secondary
case with a past history of gait impairment and a sudden Final Comments: Spontaneous rupture of arteriovenous causes should be carried out. In this case, alcohol abuse
onset of status epilepticus due to meningitis. Although malformations in young patients is associated with the were notorious as a possible cause of stroke in young peo-
CNS infections are not facilitated by DAVFs, it led to the possibility of performing immediate surgical interventions, ple, and alcohol consumption should be considered in the
discovery of an extensive one in our patient which already which allow lower risks of acute complications. However, primary and secondary prevention of stroke.
compromised cortical regions, which contributed partially these patients with longer life expectancy appear to have
to severity of the case despite treatment. a higher lifetime risk of hemorrhage.
Doença Cerebrovascular

Doença Cerebrovascular Doença Cerebrovascular

48
XXX Congresso Brasileiro de Neurologia

TL 1106616 TL 1106620 TL 1104832

CEREBRAL VASOSPASM: HOW FAR DO THE STROKE MIMIC SECONDARY TO VACICELA MAN IN THE BARREL SYNDROME AS
SEQUELS OF SARS-COV-2 INFECTION GO? ZOSTER VIRUS ENCEPHALITIS A PRESENTATION OF MOTOR NEURON
DISEASE: CASE PRESENTATION
Matheus da Costa Guedes, João Igor Dantas Larissa Brenda Gonçalves Miná, George Nilton
Landim, João Gabriel Dias Brasiliense Frota, Sarah Nunes Mendes, Karla Rafaele Silva Vasconcelos, Pedro Thiago Simões Ferreira, Juliana Oliveira
Diogenes Alencar, Barbara Matos Almeida Queiroz, Sylvio Ricard Gonçalves de Souza Lima, Ana Silvia Almeida, João Vitor Nunes Sobreira Cruz, Allef
Vito Thayson Damasceno dos Santos, Karoline Sobreira Lima Verde, Ian Silva Ribeiro, Sarah Roberto Gomes Bezerra, Kirsten Araujo Melo,
Ferreira Mororo Menezes, Glauber de Menezes Diógenes Alencar, Samuel Ranieri Oliveira Veras, Simone de Cássia Silveira Silva, Patricia Pereira
ferreira, Norberto Anizio Ferreira Frota, Fernanda Fabrício Oliveira Lima Nunes, Fernando Tenório Gameleira, Mariana Cota
Martins Maia de Carvalho Bastos
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Hospital Geral de Fortaleza. Fortaleza CE, Brazil HGE – HUPAA. Maceió AL, Brazil
Universidade de Fortaleza. Fortaleza CE, Brazil laribgm@gmail.com
HUPAA. Maceió AL, Brazil
matheusguedes010@gmail.com Case Presentation: Female, 77 years old, hypertensive pedro_thiago22@hotmail.com
and coronariopathy, searched our service after being
Case Presentation: Female, 22 years-old, referred by found sleepy, disordered and with alteration of speech. Case Presentation: Male, 52 years old, history of fascic-
SAMU, was admitted to the neurological emergency ser- on admission, it was evaluated by the neurology team, ulations and sudden biparesis of the limbs, asymmetric,
vice after an episode of generalized tonic-clonic epileptic which confirmed aphasia. a ct scan of the skull was per- for 3 years, evolving with mild paresis of the LLL with
seizure, lasting about 20 minutes, and drowsiness after the formed, which showed an asymmetry of density between progression to RLL in 10 days, without prejudice on gait,
crisis. She denied sphincter release. On admission, the the insulae, which, together with the sudden focal deficit, associated with dysphonia and intermittent dysphagia.
patient was feverish, pale, drowsy, GCS 14, without focal strengthened the diagnostic hypothesis of hyperacute The condition followed with a gradual worsening of the
deficits. No neck stiffness. Complementary investigation ischemic brain vascular accident, being thrombolytic tetraparesis, with a severe predominance in the upper
showing hypokalemia and leukocytosis. Brain Computed treatment with alteplase and the patient referred to the limbs, with no complaints of sensitivity. Personal back-
Tomography (CT) did not show any changes. Before, she ischemic stroke unit. the following day, the patient was ground: systemic arterial hypertension. On examination:
reported Hospitalization for a month due to a SARS-Cov-2 sleepy and encephalopathy, when ulcerated lesions cov- alert, oriented, dysarthria and dysphonia, isochoric and
infection, requiring mechanical ventilation for 17 days. ered by serohematical crusts and lesional hyperemia photoreactive pupils, plegia and bilateral hypotrophy of
Considering the hypothesis of infectious encephalitis, were noticed, located in the front region on the right, in the upper limbs, generalized hypotonia, with patellar
the patient received empirical treatment with Acyclovir the territory of the ophthalmic branch of the trigeminal hyperreflexia, Babinski’s sign on the right, Hoffman’s sign
and Meropenem. After two days of Hospitalization, she nerve, the diagnostic hypothesis of herpes-zoster being on the left. Neuroaxis imaging exams without alterations.
presented a new episode of generalized tonic-clonic proposed. lumbar puncture performed which evidenced Electroneuromyography (ENMG): findings consistent
epileptic seizure. The investigation proceeded: negative cellularity of 201 (66% lymphocytes and 19% monocytes), with motor neuron disease. Discussion: Clinical signs
serology, no hypovitaminosis, negative COVID swab, protein 31 mg/dl and glucose 52 mg/dl, in addition to mul- of upper and lower motor neuron lesions, with a clear
electroencephalogram without changes. Magnetic res- tiplex pcr confirmed varcella zoster virus infection. the predominance of involvement in the upper limbs, with a
onance imaging (MRI) of the skull with hyperintense mri and angio-mri brain showed lesion with restriction characteristic phenotype of man-in-the-barrel syndrome,
lesions in the occipital region. Positive Cerebrospinal to left parietal diffusion. treatment with acylovir started required the need for an electroneuromyographic study,
Fluid (CSF) Analysis for herpes simplex virus 2, with and in 48 hours the patient evolved with improvement of which showed alterations compatible with sensory pre-
negative bacterioscopy. Electroneuromyography with- symptoms. Discussion: The varicella zoster virus causes ganglionic involvement, showing signs of chronic dener-
out significant changes. The patient evolved with better chickenpox as a primary infection, after which the virus vation and reinnervation in at least two body segments,
clinical improvement. New CSF study: red blood cells: becomes latent in the cranial nerves and dorsal root gan- corroborating the hypothesis of motor neuron disease /
120, leukocytes: 1 cell (neutrophils: 2%, lymphocytes: glia along the neuroaxis. when cell mediated immunity amyotrophic lateral sclerosis (ALS). Final Comments:
93% monocytes: 5%) proteins: 28. glucose: 57, LDH: 27 decreases, the virus may reactivate, travel peripherally to The nomenclature “man in the barrel syndrome” (MBS)
ADA: 0.1, VDRL: negative. A new MRI was performed, cause a skin eruption and peripheral neuropathy (her- was first used in 1983, since then, many cases have been
which showed an increased signal at the cortico-subcor- pes zoster) or, less commonly, it may move centrally and reported, although the vast majority of motor neuron
tical T2 and Flair sequences in bilateral parieto-occipital cause a variety of neurological complications, including pathologies have a classically different etiopathogene-
lobes and right frontal lobe with contrast enhancement. vasculopathy and encephalitis, these can cause irrevers- sis from this description. Still, MBS is not exclusive to
Vessel wall with microconstrictions inferring a reversible ible brain damage. in the case in question, the apparent ALS, as a considerable part of the reports are caused by
cerebral vasospasm pattern. Discussion: Here we present ischemic lesions on mri did not justify the patient’s initial Progressive Spinal Amyotrophy, differential diagnosis that
a case of cerebral vasospasm in a young patient with no symptoms, being treated as stroke mimic secondary to requires neurophysiological evaluation for clarification,
previous comorbidities. It is known that COVID-19 has encephalitis framework by vzv. this agent is susceptible given its lower severity compared to ALS. The patient
been associated with immune dysregulation of the host to various antiviral drugs, but acyclovir is the drug of above presented an atypical clinic of upper limb biplegia,
that can increase the risk of infections such as herpes choice and its early initiation has contributed to avoid- and the ENMG was consistent with the diagnosis of ALS
virus, but this mechanism is still not well understood. In ing serious complications in the case in question. Final manifested with MBS.
addition, Sars-cov-2 has been showing a vast amount of Comments: There is a large quantity of diseases that can
sequelae in those affected, including cerebral vasospasm. behave like stroke mimic, vzv encephalitis is one of them,
Final Comments: The literature shows to an endothelial that should be suspected, especially, when there is clini- Doenças do Neurônio Motor – Esclerose Lateral
inflammation caused by the coronavirus as a contributor cal acute encephalopathy associated with suggestive skin Amiotrófica
to the development of cerebrovascular disease, which lesions. timely treatment can prevent serious sequelae.
includes, in addition to vasospasm, stroke, headaches
and other changes in the central nervous system, and it
may even aggravate other infections. Doença Cerebrovascular

Doença Cerebrovascular

49
XXX Congresso Brasileiro de Neurologia

TL 1106095 TL 1104860 TL 1104862

WHEN OCCAM´S RAZOR ISN´T SHARP: MUSK-RELATED CONGENITAL MYASTHENIC EXPANDING THE SPECTRUM OF TBK1-
ASSOCIATION BETWEEN PROGRESSIVE SYNDROME: FIRST REPORT OF ADULT- RELATED DISORDERS: ADULT-ONSET NON-
MUSCULAR ATROPHY, PIRIDOXIN ONSET CASE IN BRAZIL 5Q SPINAL MUSCULAR ATROPHY
NEUROTOXICITY AND LATE ONSET
Eduardo Claus Constante, Wladimir Bocca Vieira Vinícius Lopes Braga, Igor Melo de Almeidal,
MYASTHENIA GRAVIS de Rezende Pinto, Vinícius Lopes Braga, Karen Eduardo Claus Constante, Wladimir Bocca Vieira de
Francisco de Assis Pinto Cabral Júnior Rabello, Yumie Tanamati, Igor Braga Farias, Bruno de Mattos Rezende Pinto, Ana Carolina dos Santos Jorge, Paulo
Camila Castelo Branco Pupe, Osvaldo José Moreira Lombardi Badia, Roberta Ismael Lacerda Machado, Victor Sgobbi de Souza, Igor Braga Fariasl, Bruno
Nascimento Marcelo Annes, Paulo Victor Sgobbi de Souza, Acary de Mattos Lombardi Badia, Marco Antônio Troccoli
Souza Bulle Oliveira Chieia, Acary Souza Bulle Oliveira
Universidade Federal Fluminense. Niterói RJ, Brazil
UNIFESP – EPM, São Paulo SP, Brazil Escola Paulista de Medicina, Universidade Federal
francisco_cabral@id.uff.br de São Paulo UNIFESP
eduardoconstanteh@hotmail.com
Case Presentation: A 71 years old male, Swiss, started viniciuslopesbraga@live.com
recurrent episodes of cramps on both feet in the last Case Presentation: A 33-year-old man presented with
3 years, progressing to the hands in the next several severe muscular fatigue for more than a year, more pro-
nounced in the evening, associated with double vision Case Presentation: A 36-yo man presented with a slowly
months. The symptoms progressed insidiously to distal progressive history of weakness and painful cramps in his
weakness first in the right foot, then to the left one, within and eyelid ptosis, both initially intermittent, then contin-
uous after six months. Neurologic examination revealed lower limbs since age 22 years. After 8 years, he noted mild
12 months, causing stumbling when walking. He started amyotrophy involving the distal portion of the thighs and
complex B vitamins supplementation by a neurologist dysphonia, bilateral eyelid ptosis with fatigue during
maneuvers, discrete global ophthalmoparesis, bilateral calves, slow gait speed, and waddling gait. Medical and
prescription. He brought an electromyography (EMG) family history were unremarkable. Parental consanguin-
with signs of acute and chronic axonal cervical and lum- facial nerve palsy, and discrete and symmetric palatal
numbness. MRC muscle strength was 4/5 in the prox- ity was not present. Examination disclosed crural flaccid
bosacral motor degeneration. There was also a sensory paraparesis with proximal predominance, absent tendon
axonal polyneuropathy (SAP) in the four limbs. He took imal lower and upper limbs and global hyporeflexia.
Needle EMG showed a myopathic pattern and a deltoid reflexes and bilateral pes cavus. Neurophysiological stud-
gabapentin for chronic low back pain due to L5 herniated ies disclosed symmetric chronic denervation pattern with
vertebral disc. He was told that he had motor neuron dis- muscle biopsy revealed nonspecific mild and chronic
myopathic findings. Initially, we tried pyridostigmine and reinnervation in the lumbosacral myotomes (L3-S1). Brain
ease spectrum (MNDE) after extensive complementary and spine MRI and cerebrospinal fluid analysis were unre-
exams, so he asked for a second opinion. Positive neu- prednisone. LF-RNS showed a pathological decrement
of 18%. His condition progressively worsened until he markable. Muscle MRI disclosed mild fatty replacement of
rological findings on examination at this moment were: the vastus medialis, adductor and focal fatty replacement
nasal voice, Romberg sign, deltoid myofasciculations suffered six episodes of myasthenic crisis within a year,
requiring prolonged mechanical ventilation, IGIV, PLEX, bilaterally in the medial head of the gastrocnemius muscle.
(https: //www.youtube.com/shorts/KSv9yZS4y0Y), absent As clinical and neurophysiological features were sugges-
Achilles reflex, hypoesteshia on the lateral aspect of the and remained refractory even with rituximab. Both anti-
AchR and anti-MUSK were negative. His 29-year-old tive of non-5q spinal muscular atrophy (SMA), especially
right leg with ipsilateral weakness on hallux dorsiflexion. SMA with lower extremity predominance (SMALED), a
There were no signs of upper motor neuron (UMN) dis- sister had a history of eyelid ptosis, neonatal hypotonia,
and an isolated episode of GTC seizure associated with genetic testing with whole-exome sequencing (WES) was
ease. Curiously, patient state fatigue during the physical performed. WES evidenced the heterozygous pathogenic
examination. Due to muscular fatigability, screening dos- hypoxia. At age 22, she began to experience intense and
slowly progressive muscular fatigue, particularly in the variant c.1960-1G>T (a splice acceptor variant) in intron
age of nicotinic acetylcholine (AChR)-binding antibody 18 of the TBK1 gene, leading to a loss of function effect
was requested and showed a title of 0,56 mmol/L (posi- lower limbs, associated with eyelid ptosis. Neurologic
examination revealed appendicular fatigue, eyelid pto- due to protein truncation and activation of nonsense-me-
tive when greater than 0,40). Piridoxin seric levels were diated decay. Thus, we concluded that our patient pre-
high (154,8 µg/L, normal range between 2,8-74,6 µg/L), sis, and discrete ophthalmoparesis without dysphonia
or dysphagia. Genetic testing on both siblings confirmed sented with a new MND phenotype associated with TBK1
but lowered spontaneously to 68,6 µg/L when vitamin B6 gene. Additional testing for 5q SMA (including MLPA and
supplementation was stopped. Patient started prednisone the diagnosis of MUSK-related autosomal recessive con-
genital myasthenic syndrome. NGS-based gene panel Sanger sequencing) and a wide NGS-based gene panel
40mg/dia and pyridostigmine 360mg/dia. Discussion: The test were performed and their results were unremark-
association between myasthenia gravis (MG) and amyo- testing disclosed the presence of compound heterozy-
gous variants c.2125G>A (p.Val709Met) and a deletion able. Discussion: TBK1 (TANK binding kinase-1) gene
trophic lateral sclerosis (ALS) is strong, but its association (12q14.2) is associated with modulation of autophagy and
with the lower motor neuron (LMN) sporadic phenotype of exons 3-4 in the MUSK gene. Discussion: Congenital
myasthenic syndrome (CMS) is a clinic and genetically inflammatory function in neuronal and glial cell types.
(Progressive Muscular Atrophy, PMA) is unknown. PMA TBK1 variants are related to Amyotrophic Lateral Sclerosis
is a controversial entity and represents 3% of all cases of heterogeneous group of inherited neuromuscular disor-
ders, which share impaired neuromuscular transmission (ALS), Frontotemporal Dementia, and susceptibility to
MNDE. One third of patients with PMA develop UMN acute infection-induced herpes simples-specific enceph-
features within 2 years during disease course. The patient due to distinct genetic mechanisms. Autosomal recessive
pattern of inheritance is observed in the MUSK (9q31.3) alopathy type 8. Our patient presented with clinical and
had not palpebral ptosis or diplopia, but fatigability and neurophysiological findings suggestive of long-standing
nasal speech pointed to MG and not a bulbar UMN sign. gene-related CMS. MUSK encodes a post–synaptic pro-
tein involved in endplate maintenance and maturation, lower MND, mimicking features of SMALED syndrome
Sensory ataxia due to B6 toxicity was a bias in the con- and atypical ALS variants. SMALED phenotypes have
text of interpretation of EMG, since sensory nerve action proper function of Rapsyn and AchR, thus acting in the
AchR clustering pathway. Treatment of CMS is not stan- been previously associated with DYNC1H1 and BICD2
potentials (SNAP) are normal in MND. Final Comments: heterozygous pathogenic variants, however with distinct
Neurologists should be aware since muscular weakness dardized because of the small number of patients and
the lack of specific therapeutic trials. Final Comments: muscle imaging findings when compared to our patient.
may be either the common final path symptom or an Final Comments: Clinicians must be aware about the
coexisting condition in both neuromuscular junction Our cases expand the current knowledge about CMS and
provide original information regarding the first geneti- possibility of TBK1 variants as new monogenic basis of
disease and motor neuron disease. pure non-5q SMA and SMALED-like phenotypes with
cally confirmed adult-onset MUSK-related CMS case in
brazilian population. specific muscle imaging pattern of involvement.
Doenças do Neurônio Motor – Esclerose Lateral
Amiotrófica Doenças Neuromusculares
Doenças Neuromusculares

50
XXX Congresso Brasileiro de Neurologia

TL 1105097 TL 1105102 TL 1105154

HYPERTHYROIDISM PRESENTING WITH NECROTIZING MYOPATHY RELATED TO GUILLAÍN-BARRÉ SYNDROME AFTER


HYPOKALEMIC PERIODIC PARALYSIS ARBOVIROSIS – CASE PRESENTATION DENGUE VIRUS INFECTION, A RARE
AND CLUSTER HEADACHE: A CASE NEUROLOGICAL COMPLICATION
Paulo Barbosa Leite Neto, Larissa Clementino Leite
PRESENTATION Sa Carvalho, Victor Fellipe bispo Macedo, Djanino Emanuelle Bianchi da Silva Rocha, Amanda
Carolina Rodrigues Dal Bo, Fernanda Dalgé de Fernandes Silva, Marco Andre Moraes Bernardino, Selvátici dos Santos Dias, Janaina Moraes de
Carvalho, Lucas Felipe Miniello, Marcela Botelho Ana Rosa Melo Correa Lima, Fabiola Lys Medeiros, Araújo, João Pedro Izidoro Gomes, Laura Alonso
de Carvalho, Mariana da Cruz Torquato, Raissa Ligia Rufino Silva, Pedro Nogueira Fontana, Carolina Matheus Montouro, Alex Bertolazzo Quiterio, Caique
Kitaguchi Sakajiri, Rene de Araujo Gleizer Cunha Correia Alberto Dosualdo, Andressa Regina de Mello Galego,
Ricardo Funes Bastos, Gabriel Pina Paiva
Hospital Israelita Albert Einstein, São Paulo SP, Hospital Universitário Oswaldo Cruz. Recife PE,
Brazil Brazil Faculdade de Medicina de Ribeirão Preto. Ribeirão
Faculdade Israelita de Ciências da Saúde Albert Preto SP, Brazil
paulo.lei.te@hotmail.com
Einstein, São Paulo SP, Brazil
m.bianchis@hotmail.com
Hospital Municipal Dr. Moysés Deutsch, São Paulo Case Presentation: A 35-year-old man, 3 weeks after a
SP, Brazil febrile condition with polyarthralgia, evolved with proxi- Case Presentation: A 39-year-old man with a history
carolinardalbo@gmail.com mal lower limb weakness and subsequent progression to of 10 days of fever, arthralgia and rash that evolved with
upper limbs. He denied myalgia, cramps, myoglobinuria, nausea, vomiting and abdominal pain, admitted at the
Case Presentation: A 26-year-old brazilian male, without falls, respiratory complaints, dysphagia or dysphonia. Hospital because of worsening abdominal pain, liver dys-
known comorbidities, presented with acute flaccid tetra- Neurological examination revealed scapular and pelvic function, increase in hematocrit and thrombocytopenia
paresis. He reported an abusive carbohydrate intake the muscle wasting, proximal weakness, hypoactive deep with positive serology for dengue (IgM). At the third day
previous night. He denied strenuous physical exercise, tendon reflexes and waddling gait. Laboratory investi- of Hospitalization, patient started with paresthesia of both
fever, recent trauma, loss of sphincter control, use of med- gation revealed high levels of creatine phosphokinase legs that rapidly progressed, distal weakness, areflexia,
ications, drugs or alcohol. On neurological examination, (CPK: 11,591 UI/L), lactate dehydrogenase (DHL: 1420 bilateral facial paralysis, dysarthrophonia and dysphagia.
he presented flaccid tetraparesis with proximal weakness U/L) and positive IgG serology for chikungunya (Elisa). The analysis of cerebrospinal fluid showed cytological
predominance. Laboratory investigation showed serum Electroneuromyography showed a myopathic pattern. protein dissociation (1 cell and 58 proteins). Considering
potassium 2.89 mEq/L and there was no alteration on neu- Muscle biopsy, performed at right biceps brachialis, the clinical and laboratory features the diagnosis was
roimaging. After treatment with intravenous potassium, revealed several necrotic and regenerating myofibers, with Guillain-Barré Syndrome (GBS). Then, we referred him to
the patient completely recovered his motor strength in scarce perivascular inflammatory infiltrate, several CD68+ the intensive care unit and intravenous immunoglobulin
four hours. During outpatient follow-up, he reported epi- cells, rares CD8+ cells and no CD4+ cells, consistent with (IgIV) for five day was the treatment. At the second day of
sodes of headache described as a left-sided excruciating necrotizing myopathy. The patient was then treated with IgIV he evolved with dysautonomia (heart rate instability
pain, originated in the orbital region, irradiated to supra- prednisone 1mg/kg/day. However, due to increasing CPK and urinary retention) associated with worsening of the
orbital and temporal region, lasting 15 minutes. The pain levels and persistence of weakness, human intravenous bulbar symptoms with necessity to orotracheal intubation.
was associated with ipsilateral ptosis. Results of analysis immunoglobulin (2g/kg in 5 days) was added to treatment. He had clinical complications such as ventilator-associ-
collected during Hospitalization showed TSH (<0.01?UI/ The patient was discharged with prednisone 1mg/kg/day ated pneumonia receiving broad-spectrum antibiotics
mL), T4 (6.11 ng/dL), TRAB (4.7 UI/L). The patient was and methotrexate 10mg per week. After 45 days, in follow and evolved with pseudomembranous colitis because of
diagnosed with Thyrotoxic Hypokalemic Periodic Paralysis up visit, patient showed significant improvement in mus- the antibiotics, but presenting a good treatment response.
(TPP) and met the criteria for cluster headache. He was cle strength, normal gait and normalization of CPK and Given the slow recovery and prolonged Hospitalization,
treated with tiamazol and atenolol for hyperthyroidism. DHL levels. Prednisone was tapered and methotrexate was he underwent tracheostomy and gastrostomy, being dis-
He started prophylactic and symptomatic treatment for maintained. Discussion: Immunemediated necrotizing charged after 60 days of Hospitalization. Discussion: GBS
cluster headache. Five months after Hospital discharge, myopathy is a inflammatory myopathy, characterized by is a rare presentation of dengue infection. The most com-
the patient denied new episodes of acute tetraparesis and proximal muscle weakness, high CPK levels, necrotic and mon neurologic manifestations associated with dengue
episodes of cluster headache. Discussion: TPP is a very regenerating fibers and macrophagy in muscle biopsy, as virus (DENV) infection include headache, encephalop-
rare complication of thyrotoxicosis, more frequent in the well as good response to immune therapy. Necrotizing athy and seizures. Other neurologic syndromes reported
Asian population. It must be considered as a differential myopathy is related to various etiologies, such as use of include acute pure motor weakness, mononeuropathies,
diagnosis of acute tetraparesis. The role of thyroid dys- statins, neoplasms and viral infections. One of the viral polyneuropathies, stroke and transverse myelitis. A cohort
function in the etiology of headache remains uncertain. causes, the chikungunya virus, an alphavirus present study evaluated the increasingly incidence of neurologic
In literature, there are cases of patients with newly diag- throughout the brazilian territory, presents with a febrile complications of DENV and demonstrated that clinical
nosed hyperthyroidism whose headaches remitted after disease with intense arthralgia. Neurological complica- and laboratory parameters like higher body temperature,
treatment of hyperthyroidism. A study found reduced tions include encephalitis and myelitis; there are reports liver dysfunction, increase in hematocrit and throm-
TSH response to thyrotropin-releasing hormone (TRH) of cases with peripheral nervous system involvement, bocytopenia are independent predictors of neurologic
in patients with cluster headache during cluster period, including skeletal muscle. Final Comments: Arbovirosis- complications, those changes were found in our patient
hypothesizing a hypothalamic involvement in the patho- related necrotizing myopathy is a rarely reported entity, what is in agreement with the literature. Final Comments:
genesis. Final Comments: We report this case to empha- and there is still discussion about the best therapeutic The incidence of GBS associated with dengue fever is still
size the importance of the evaluation of thyroid function approach. We reinforce the clinical response with the unknown and in the literature, there are just few isolated
in patients with acute flaccid tetraparesis. Timely recog- use of intravenous intravenous immunoglobulin; how- case presentation. In Brazil, dengue fever is endemic and
nition and treatment of TPP can reduce the risk of severe ever, long-term immunosuppressive therapy remains early recognition of neurological conditions that may be
complications such as respiratory muscle weakness and a recommendation in the follow-up of these patients. associated with this infection and being attention to pre-
arrhythmias. Further studies are needed to clarify the dictors that increases the risk of these complications is
association between hyperthyroidism and cluster head- very important in order to prevent bad outcomes.
Doenças Neuromusculares
ache, but physicians should be aware that there may be a
correlation and consider a thyroid function assessment.
Doenças Neuromusculares

Doenças Neuromusculares

51
XXX Congresso Brasileiro de Neurologia

TL 1105158 TL 1105194 TL 1105212

DISTAL PRESENTATION IN CENTRAL CORE A CASE PRESENTATION OF RECESSIVE TUMOUR NECROSIS FACTOR RECEPTOR-
MYOPATHY CAUSED BY RYR1 MUTATIONS: A MYOTONIA CONGENITA (BECKER’S ASSOCIATED PERIODIC FEVER SYNDROME
CMT-LIKE PHENOTYPE DISEASE) AND EPILEPSY. IS IT CAUSAL OR (TRAPS) IN TWO HYPOTONIC SIBLINGS
CASUAL LINK? WITH AXONAL POLYNEUROPATHY AND
Bruna Moreira Souza Proença, André Macedo
Serafim Silva, Alulin Tácio Quadros Santos Monteiro RECURRENT ABDOMINAL PAIN
Antonio Serpa Amaral Neto, Andre Dias Oliveira,
Fonseca, Clara Gontiijo Camelo, Cristiane Araujo Gisele Espíndola, Priscila Bernardi, Matheus Paulo de Lima Serrano, Samia Calil, Hélvia Bertoldo,
Martins Moreno, Lucas Marenga Arruda Buarque, Marquardt, Lais Bissoli Perino, Fernando Netto Glenda Barros, Igor Farias, Bruno Badia, Paulo Victor
Beatriz Carneiro Gondim Silva, Michelle Abdo Paiva, Zanette, Bruno Montagna Andrade Sgobbi Souza, Wladimir Bocca V R Pinto, Acary
Edmar Zanoteli Souza Bulle Oliveira
Hospital Universitário da UFSC – Florianópolis – SC
Hospital das Clínicas da Faculdade de Medicina da – Brazil Universidade Federal de São Paulo. São Paulo SP,
Universidade de São Paulo. São Paulo SP, Brazil Brazil
amnetomedunir@gmail.com
brunamoreirasp@hotmail.com paulo.lima.serrano@gmail.com
Case Presentation: Male, with normal neuropsychomo-
Case Presentation: A 16-year-old patient, with no con- tor development in early childhood, started at 7 years of Case Presentation: Two siblings, a 10-years-old girl and a
sanguinity in the family, presented with frequent falls age with generalized epileptic seizures. He was treated 7-years-old boy, hypotonic since birth with delayed motor
and tip-toe gait, at the age 2. She developed a distal leg with carbamazepine and phenytoin, evolving with com- milestones. Gait was unbalanced with frequent falls wich
weakness with significant deformities. On examination, plete remission of the attacks in 1 year. At 10 years of age, aggravated after yellow fever vaccination. Family com-
she presented global hyporeflexia, MRC grade 4 in the he developed stiffness, progressive distal and proximal plained of progressive worsening of ambulation. Both
proximal upper and lower limbs. She had a more intense weakness of the upper and lower limbs, initially with had recurrent episodes of vomiting and abdominal pain
distal lower limbs weakness (MRC 3), with deformities stumbling and difficulty running. At the age of 15, he associated with bloody diarrhea. Fever without known
in pes cavus, hammer fingers and aquileus retractions. developed the need for a wheelchair to walk distances source occured frequently. Both complained of chronic
Due to the marked presentation of distal weakness with of more than 100 meters. At 18 years of age, he presents burning pain in lower limbs. During neurological exam-
the described deformities, she was initially diagnosed fatigue and tiredness to chew solid foods. Past history: ination both had signs of a symmetrical sensory motor
with Charcot-Marie-Tooth (CMT) disease and referred Pregnancy and delivery without complications. Family polyneuropathy with areflexia, ataxic gait intensified when
to our center. On investigation, we found normal levels history: no neurological disease. Neurological exam- closing eyes and global hypotonia. C-reactive protein and
of creatine phosphokinase (CK); no evidence of polyneu- ination shows: Myotonic phenomena in hands, mouth erythrocyte sedimentation ratio were highly elevated. MRI
ropathy was seen in neurophysiological studies. Biopsy and tongue, with Warm up sign. Gowers sign present. was unremarkable. Electroneuromyography depicted a
of brachial biceps muscle revealed well delimited cores Hypermobility of the interphalangeal joints of the hands, chronic axonal symmetrical distal sensory motor polyneu-
by the staining of NADH-TR. MRI showed involvement swan-necked fingers. Biceps, deltoids and gastrocnemius ropathy. Whole exome sequencing showed a heterozygous
of paravertebral and posterior compartment of the leg with pseudohypertrophy. Presents facial and masticatory variant in the TNFRSF1A gene, confirming the diagnosis
muscles. The whole exome sequencing showed two mis- muscle weakness, mild weakness for neck flexion. Muscle of Tumour Necrosis Factor Receptor-Associated Periodic
sense likely pathogenic variants in RYR1 (p.Phe41Ser strength grade 4 proximal and distal lower limbs, grade Fever Syndrome (TRAPS). Canakinumab was initiated
and p.Gly4686Asp). Discussion: The RYR1 gene encodes 4 proximal and 3 distal upper limbs. Patellar reflexes 3/4 with complete resolution of the gastrointestinal symp-
the rianodine 1 receptor channel and is the main cause and Achilles 3/4. Sensitivity preserved. Myopathic gait. toms, lower limb pain and marked improvement in gait.
of susceptibility to malignant hyperthermia and a wide Laboratory tests with a slight increase in CK levels (341 Discussion: TRAPS is an autossomal dominant autoinflm-
spectrum of congenital myopathies, including central U/L). Electroencephalogram (2012): epileptic activity matory syndrome caused by variants in the TNFRS1A gene.
core myopathy (CCM). CCM is characterized by central characterized by polyspicules. MRI of the brain (2012): Recurrent episodes of periodic fever, myalgia, arthralgia,
cores on muscle biopsy and clinical features of a con- no changes. Electromyography (2022): diffuse myotonic abdominal pain, periorbital edema and migratory rashes
genital myopathy with autosomal dominant (AD) and discharges. Genetic Test (2022): heterozygous variants of are well known features. Episodes occur either sponta-
recessive (AR) inheritance patterns. The patients present autosomal recessive pattern, CLCN1 chr7: 143,351,593 neously of following a trigger. The neurological manifesta-
predominantly proximal weakness pronounced in the hip G>A c.2596-1G>A and Gene CLCN1 chr7: 143,342,461 tion still need further characterization. Headache, aseptic
girdle with orthopedic complications. On the other hand, T> C. Confirming the diagnosis of Myotonia Congenita meningitis, myositis and neuropathy are described but
classic CMT phenotype reflects length-dependent axo- (MC) recessive disorder (Becker) that is associated with hypotonia and delayed motor milestones has never been
nal degeneration represented by distal sensory loss and moderate to severe myotonia. Studies show evidence that reported to our knowledge. Inflammatory biomarkers are
weakness, deep tendon reflex abnormalities, and skeletal CLCN1 may be a gene that can also cause generalized often elevated. Electroneuromyography should be ordered
deformities. According to an international cohort of 21 genetic epilepsy. This case illustrates a patient diagnosed in all cases to better distinguish the features of myopathy
AR RYR1-related CCM patients, all of them had predom- with Becker’s Autosomal Recessive Myotonia Congenita, and neuropathy that may be present in this group. Early
inantly proximal muscle weakness. Although most had confirmed by genetic testing, associated with epilepsy diagnosis is essential since patients experience signifi-
some involvement of distal muscle groups, distal weakness and hypermobility of the phalanges. Epilepsy is rarely cant improvement with treatment. The knowledge and
was as severe as proximal weakness in only 4. An early described in patients with CM but some studies suggest early recognition is needed for a better depiction of the
exclusively distal phenotype has not been reported in a role of CLCN1 in regulating brain excitability, it may neurological manifestations. Final Comments: TRAPS is
the main studies of CCM. Final Comments: Our patient be that the disorders are linked by this mutation in the an autossomal dominant hereditary autoinflammatory
presented an atypical phenotype for RYR1-related CCM, chloride channel. syndrome, associated with an expanding neurological
with predominantly distal weakness and feet deformities picture in the literature. Our case shows two siblings with
that resembled the CMT phenotype. RYR1 must be con- marked hypotonia, delayed motor milestones, axonal sen-
sidered as a cause of distal myopathies. Pes cavus and Doenças Neuromusculares sory motor polyneuropathy and a marked recovery after
hammer fingers are not specific for CMT.

Doenças Neuromusculares
Doenças Neuromusculares

52
XXX Congresso Brasileiro de Neurologia

TL 1105213 TL 1105221 TL 1105226

FUKUTIN-RELATED LIMB-GIRDLE ANTI-AQUAPORIN 4 ANTIBODY-RELATED CASE PRESENTATION DYSAUTONOMIA IN


DYSTROPHY PRESENTING AS MYOPATHY SPASTIC PARAPLEGIA TYPE 5A (SPG5A)
PSEUDOMETABOLIC MYOPATHY
Paulo Lima Serrano, Samia Calil, Hélvia Bertoldo, Gabriel Pinheiro Martins de Almeida e Souza,
José Pedro Soares Baima, André Macedo Serafim Glenda Barros, Igor Farias, Bruno Badia, Paulo Victor Wladimir Bocca Vieira de Rezende Pinto, Igor Braga
Silva, Michelle Abdo Paiva, Clara Gontijo Camelo, Sgobbi Souza, Wladimir B V R Pinto, Acary Souza Farias, Bruno de Mattos Lombardi Badia, Paulo
Ana Lucila Moreira, Edmar Zanoteli Bulle Oliveira Victor Sgobbi de Souza, Acary Souza Bulle Oliveira

Department of Neurology, Hospital das Clínicas da Universidade Federal de São Paulo. São Paulo SP, Division of Neuromuscular Diseases, Department
Faculdade de Medicina da USP, São Paulo SP, Brazil Brazil of Neurology and Neurosurgery, Escola Paulista
de Medicina, Universidade Federal de São Paulo
jpedrobaima@gmail.com paulo.lima.serrano@gmail.com UNIFESP

Case Presentation: A 35-years-old woman, born from Case Presentation: 47-years-old Woman, presented bilat- gabrielpinheiromas@gmail.com
non-consanguineous parents with a Japanese ancestry, eral optic neuritis in the third decade of life associated with
presented with an episode of myalgia, dark colored urine, longitudinal extensive transverse mielitis. Anti-aquaporin Case Presentation: A 26-years-old woman presented with
high CK levels after volleyball training at age 15- years- 4 antibody was requested and came back positive. Due to slowly progressive weakness and stiffness in her legs, fol-
old. During this episode, CK level was 15.450 U/L. She clinical picture and positive anti-aquaporin 4 the diagnosis lowed by urinary incontinence. She also reported dizziness
had a spontaneously recovery. She then developed pro- of neuromyelitis optica spectrum disorder (NMSD) was when standing with frequent falls and changes in the color
gressive exercise intolerance, mild proximal weakness, made. Chronically complained of xerostomia and xer- of her toes associated with swelling when the weather is
and CK levels remained four times upper limit value. oftalmia and had positive anti-Ro antibody. One year ago, cold. Physical examination revealed persistent painless
Electrodiagnostic studies suggested a myopathic process noticed pain in lower limb muscles, being worse in the toes cyanosis and neurological examination showed cru-
with normal repetitive stimulation. A muscle biopsy of proximal muscles and associated with important fatigue, ral spastic paraparesis, patellar and ankle hyperreflexia,
biceps brachii revealed a mild myopathic pattern with aggravating the gait that was already compromised by the bilateral Babinski sign and distal impairment of all sensory
mitochondrial and glycogen accumulation, suggest- demyelinating outbreaks. Creatinephosphokinase and modalities in lower limbs. Neuroimaging studies were
ing a metabolic myopathy. On a recent visit, physical aldose were highly elevated. Lower limbs MRI depicted unremarkable. Tilt test demonstrated postural hypoten-
examination showed MRC 4 in proximal muscles, axial liposubstitution on the T1 sequencing and edema on STIR sion and urodynamic study reveals detrusor overactivity
weakness, and winged scapula. Cervical weakness was sequencing, in the anterior compartment of the thigh and stress urinary incontinence. Genetic testing identified
also noted. She was unable to get up from lying in the bilaterally and in the medial gastrocnemius on the right, the pathogenic variant c.825T>A (p.Tyr275*) in homo-
examination table. Neuromuscular ultrasound indicated an image pattern suggestive of myopathy. Discussion: zygosity in the CYP7B1 gene associated with hereditary
mild hyper echogenicity (Heckmatt 2) in brachialis and Aquaporin 4 is the most prevalente aquaporin channel in spastic paraplegia 5A (SPG5A). Discussion: The chronic
vastus intermedius, and moderate hyper echogenicity the central nervous system (CNS). Its function is to provid phase of spinal cord injury may present with dysautono-
(Heckmatt 3) in hip adductors and rectus abdominal rapid transport of water, maintaining balance and cellular mia, including urinary dysfunction, orthostatic hypoten-
muscle. Diaphragmatic muscle involvement was also homeostasis. The detection of anti-aquaporin 4 antibody sion, and even impaired regulation of the body extremities
demonstrated. No tongue involvement was seen. A next is an importante hallmark in the diagnosis of NMOSD, a due to loss of vasomotor tone in lower limbs. Neurogenic
generation panel for neuromuscular genes revealed com- well-characterized disease of the CNS. However, aqua- bladder, postural hypotension, sexual dysfunction, and
pound heterozygous pathogenic variants in the Fukutin prin 4 is also expressed outside the CNS, as in the striated decreased sweat output have been associated with dif-
gene (FKTN). DNA segregation confirm the recessive skeletal muscles, and can cause hyperCknemia, myosi- ferent SPG subtypes. However, to our best knowledge, we
pattern. Discussion: FKTN biallelic mutations usually tis with muscle lesions evidenced in muscle byopsy and did not find any case presentation with an association
present as a congenital myopathy, mainly in Japan and is MRI. We present a case of a patient with NMOSD, with between these symptoms and SPG5A. In this case, the
considered quite rare in other populations. Presentation worseneing fatigue and significant myalgia in the lower presence of postural hypotension, urinary incontinence
of limb-girdle manifesting during late childhood is even limbs, elevation of creatinephosphokinase and alsolase, and cold-induced acrocyanosis suggest impairment of the
rarer. Only one case of FKTN related congenital muscu- with myopathic image pattern on MRI of the lower limbs. autonomic nervous system. Final Comments: Our case
lar dystrophy was reported in a Japanese patient living in The condition described is an extra-CNS manifestation expands current knowledge about the association between
Brazil. In a recent national limb-girdle muscle dystrophy of anti-aquaporin 4 positive NMOSD and should be SPG5A and autonomic disturbances. We conclude that
cohort, this gene was not included. As muscle panels are screened for in cases of myalgia, worsening of fatigue and autonomic involvement may be underrecognized in the
becoming more widespread and accessible, FKTN could be gait without evidence of a new demyelinating outbreak. different SPG subtypes.
an answer of previous undiagnosed muscle dystrophy in Final Comments: The association of anti-aquaporin
people with a Japanese ancestry. Final Comments: Brazil 4 antibody with NMOSD is well stablished. Currently
has the larger Japanese community outside Japan, there- recognized, but less explored in clinical practice, is its Doenças Neuromusculares
fore FKTN-related myopathies should be considered not association with myopathy. HyperCKnemia associated
only when facing a congenital myopathy or a limb-girdle with anti-aquaporin 4 antibody should be considered a
phenotype, but also when a pseudometabolic myopathy sign of muscular manifestation of NMOSD. Through this
is present. More studies are needed to identify real fre- case presentation we hope to increase knowledge and
quencies of this rare disorder in our country. interest about muscle involvement in anti-aquaporin 4
positive NMOSD.

Doenças Neuromusculares
Doenças Neuromusculares

53
XXX Congresso Brasileiro de Neurologia

TL 1105283 TL 1105285 TL 1105293

A COMPLEX ASSOCIATION OF CONGENITAL MYASTHENIC SYNDROME DUE FACIAL ONSET SENSORY AND MOTOR
SENSORIMOTOR AXONAL NEUROPATHY, TO GMPPB VARIANTS NEURONOPATHY (FOSMN SYNDROME):
DEAFNESS, EPISODIC WORSENESS, AND A CASE PRESENTATION
Daniel Delgado Seneor, Wladimir Bocca Vieira de
RETINOPATHY: MITCHELL SYNDROME Rezende Pinto, Paulo de Lima Serrano, Roberta Gabriella Corrêa Dousseau, Daniel Thomas Pereira
Samia Rogatis Calil, Wladimir Bocca Vieira de Ismael Lacerda Machado, Rodrigo Galvão Bueno Lopes, Eduardo de Paula Estephan, Pedro Henrique
Rezende Pinto, Igor Braga Farias, Bruno de Mattos Gardona, Beatriz Castro Reis, Igor Braga Farias, Marte Arruda Sampaio, Isabel de Oliveira Santos,
Lombardi Badia, Paulo de Lima Serrano, Glenda Marcelo Annes, Paulo Victor Sgobbi de Souza, Acary Heitor Nunes de Oliveira Sento-Sé Neto, Elaine
Barbosa Barros, Hélvia Bertoldo de Oliveira, Márcio Souza Bulle Oliveira Calumby Teixeira, Lorrane Campidelli Arthuzo, Maria
Luiz Escórcio Bezerra, Paulo Victor Sgobbi Souza, Sheila Guimarães Rocha
Universidade Federal de São Paulo. São Paulo SP,
Acary Souza Bulle Oliveira Brazil Hospital Santa Marcelina. São Paulo SP, Brazil
Universidade Federal de São Paulo. São Paulo SP, danielseneor30@gmail.com gabrielladousseau@gmail.com
Brazil

calil.samia@gmail.com Case Presentation: A 40-year-old brazilian woman pre- Case Presentation: A.M.M., male, 60 years old. This
sented with a long-standing slowly progressive clinical patient was referred for neurological evaluation due to
Case Presentation: A 28-year-old brazilian woman pre- course of muscle fatigue and exercise intolerance since an insidious complaint of left arm paresthesia, weakness
sented with a long-standing clinical course of lower limb age 22 years. After two years of symptom-onset, she also and atrophy since 2003. Electroneuromyography was per-
weakness starting at age 15 years, associated with recurrent noted eyelid ptosis without diplopia and dysgraphia. There formed, demonstrating cervical radiculopathy. He didn’t
and prolonged episodes of numbness and neuropathic was also worsening of muscle weakness at the end of the continue the medical investigation and returned in 2017
pain. Her symptoms evolved and after five years she pre- day and after physical activity. By the age of 36, weakness complaining that the weakness had progressed to his ipsi-
sented with worseness of motor symptoms and sensory worsened, and she could not walk for more than 10 min- lateral face and leg. On neurological examination, upper
loss in the lower limbs, mild motor compromise of the utes. At that time, serum creatine kinase levels were evalu- and lower motor neuron signs were found. In addition,
upper limbs, and bilateral sensorineural hearing loss. ated and disclosed mild elevation (900; 3 times the upper midface hypoesthesia (“onion bulb”) and blink reflex
After almost one decade of symptom-onset, she noted limit value). One year ago, she started diplopia, dyspnea, abnormality were observed. Electroneuromyography
marked worseness of motor symptoms, urinary distur- and dysphagia. Medical history disclosed dyslipidemia. was performed, demonstrating signs of denervation in
bances, severe hearing loss (with the need of bilateral Parental consanguinity was present. Family history dis- bulbar segments and alteration of the blink reflex, sug-
cochlear implants), and progressive unilateral visual closed her sister with similar symptoms. Examination gesting the diagnosis of FOSMN. Discussion: FOSMN is a
loss of the left eye. Medical history disclosed short stat- disclosed mild proximal weakness in the upper and lower rare neurologic syndrome and pathogenesis is uncertain,
ure of unknown etiology. No parental consanguinity was limbs, mild axial muscle weakness. Her nerve conduction but studies suggest that the pathophysiology is more neu-
observed. Examination disclosed distal dominant quad- studies were unremarkable. Needle electromyography dis- rodegenerative than autoimmune. Apparently, FOSMN
riparesis with brisk global reflexes and absent Achillean closed typical chronic myopathic features. Muscle biopsy is most likely to be a TDP-43 proteinopathy within the
tendon reflexes, sensory ataxia, steppage gait, and marked showed nonspecific myopathic findings. A next-gener- ALS-FTD spectrum. The mean age at onset is approxi-
hypoesthesia and hypopalesthesia in the lower limbs. ation sequencing-based multigene panel for inherited mately 55 years with progressive course involving sen-
Neurophysiological studies disclosed axonal sensorim- neuromuscular disorders disclosed the presence of the sory disturbances in trigeminal nerve pathway, bulbar
otor polyneuropathy. Brain MR imaging showed mild pathogenic variant c.95C>T (p.Pro32Leu) and the variant symptoms, upper extremities and, in some cases, to the
periventricular leukoencephalopathy. Optic coherence of uncertain significance c.478G>A (p.Val160Met) in com- lower extremities, as in this case. The diagnostic marker is
tomography showed bilateral thinning of the neurossenso- pound heterozygosity in the gene GMPPB, thus enabling the absence of the blink reflex on EMG in addition to the
rial retina. As riboflavin transporter defect was suspected, the diagnosis of GMPPB-related Congenital Myasthenic presence of denervation that can be found in the bulbar,
oral riboflavina was started, 20 mg per kg, with moderate Syndrome (CMS) associated with myopathic involvement. cervical and thoracic preganglionic segments, although
improvement of motor, visual and hearing complaints. Oral salbutamol and pyridostigmine were started with there are no specific criteria, which makes the diagnosis
NGS-based gene panel testing showed the heterozygous clinical stability of symptoms. Discussion: GMPPB-related difficult. Final Comments: This case presentation empha-
variant c.1384C>T (p.Arg462Cys) in the ACOX1 gene neuromuscular disorders represent Congenital Disorders sizes that it is very important to recognize rare diseases
involved with Mitchell syndrome. Discussion: Palmitoyl of Glycosylation and occur due to variable deficiency of within the spectrum of neuromuscular disorders, such as
acyl-CoA oxidase 1 represents the rate-limiting peroxiso- mannose-1-phosphate beta-guanylyltransferase, which FOSMN, in the context of progressive pain and weakness
mal enzyme involved in the beta-oxidation of fatty acids, is involved with GDP-mannose biosynthesis related to with cranial-caudal progression. However, the clinical
which is related both to loss-of-function mechanisms with multiple glycosylation pathways. It has been previously pictures and pathophysiology of FOSMN have not been
enzyme deficiency due to biallelic variants and to gain-of- associated with autosomal recessive limb-girdle muscu- well established.
function with neurotoxic mechanisms due to heterozygous lar dystrophy type 19 (formerly LGMD2T), Congenital
variant. Mitchell syndrome is an extremely rare progres- Muscular Dystrophy-Dystroglycanopathy type 14 and,
sive inherited neurometabolic and neurodegenerative more recently, with late-onset CMS phenotypes. Some Doenças Neuromusculares
disorder associated with sensorimotor polyneuropathy, of the previously described GMPPB-associated myop-
deafness, and episodes of clinical worseness with central or athies had dysfunction of the neuromuscular junction
peripheral demyelination. Our case description disclosed and mildly raised serum creatine kinase levels. Final
progressive visual loss and moderate response to ribofla- Comments: Clinicians must be aware about the possi-
vin therapy. Final Comments: Clinicians must be aware bility of late-onset presentations of CMS due to GMPPB
about the possibility of facing new clinical presentations variants, which should be considered in cases with myo-
of inherited peroxisomal disorders, such as Mitchell syn- pathic findings in neurophysiological studies and raised
drome, in the clinical context of progressive hearing loss, serum creatine kinase levels.
polyneuropathy, sensory ataxia, visual loss, and acute
or subacute episodes of motor and sensory worseness.
Doenças Neuromusculares

Doenças Neuromusculares

54
XXX Congresso Brasileiro de Neurologia

TL 1105306 TL 1105309 TL 1105342

TUBULAR AGGREGATE MYOPATHY WITH CLINICAL IMPROVEMENT IN ANTI-MUSK INCREASING GENETIC AND PHENOTYPIC
RHABDOMYOLYSIS: AN UNCOMMON MYASTHENIA GRAVIS PATIENT AFTER VARIABILITY OF PRPS1 GENE MUTATIONS: A
PRESENTATION IN ELDERLY MALE THYMECTOMY – A CASE PRESENTATION NEW MUTATION ASSOCIATED TO A COMPLEX
HEREDITARY MOTOR NEUROPATHY
Rafael Braganca Rodrigues Matias, Eduardo Danilo Jorge da Silva, Fabiana Carla dos Santos
Mesquita Souza, Matheus Alves da Silva, Barbara Correia, Sergio Roberto Pereira da Silva Júnior, Gabriela Lopes de Morais, Lucas Gondim Briand
Maini Carvalho, Hennan Salzedas Teixeira, Karlla Isadora Bitencourt Baesso, Marcelo Maroco Vieira, Vanessa Lauanna Lima Silva, Pedro José
Danielle Ferreira Lima, Edmar Zanoteli, Alex Cruzeiro Tomaselli, Rodrigo Siqueira Soares Frezatti, Henry
Machado Baeta Houlden, Mary M Reilly, Wilson Marques Júnior
Hospital Universitário da Universidade Federal de
Beneficencia Portuguesa de São Paulo, São Paulo Juiz de Fora. Juiz de Fora MG, Brazil Hospital das Clínicas da Faculdade de Medicina de
SP, Brazil Ribeirão Preto. Ribeirão Preto SP, Brazil
Universidade de São Paulo. São Paulo SP, Brazil fabi-karlla@hotmail.com
Instituto de Neurologia e Neurociências, Queen
Square, Londres – Grã-Bretanha Reino Unido
matheus.alves.123@outlook.com Case Presentation: Female patient, 32 years old, pre-
sented eyelid ptosis to the right, diplopia, dysphonia gabrielalmorais8@gmail.com
Case Presentation: A 76-year-old male with acute sym- and proximal appendicular weakness in superior mem-
metrical inferior limbs weakness and pain. No previous bers with floating characteristic. She was submitted to Case Presentation: Two male siblings from non-con-
history of muscle weakness. Denied alcohol intake, prior an electroneuromyography with repetitive stimulation sanguineous parents presented in their first decade of
exercise intolerance, fatigue, myoglobinuria, ptosis, or (decremental pattern). The tomography of the chest life with a complex phenotype including delayed motor
pigmenturia, as well as worsening after periods of fast- showed thymic hyperplasia associated to the antirecep- milestones, dysarthria and deafness. Ataxic gait was noted
ing. History of systemic arterial hypertension and dys- tors antibody dosage of acetylcholine (AChR) negative, at age of three in both. Symptoms worsened over the years,
lipidemia. He had been treated with the same dose of but anti-kinase antibody muscle-specific (MuSK) posi- mainly gait, with frequent falls. Their mother presented
simvastatin and antihypertensives. Furthermore, no rel- tive. She initiated the usage of prednisone up to 80mg/ hypoacusia by the age of 54, with no other symptoms.
evant familial history of consanguinity. On examination, day and pyridostigmine reaching 360mg/day with par- Brain MRI revealed cerebellar atrophy. Nerve conduc-
the strength of proximal inferior limbs graded MRC grade tial response. Continued with the symptoms worsening, tion studies revealed motor axonal neuropathy in both
IV. Neoplastic, infectious, rheumatological, and endocri- tachydyspnea and Hospitalization in an intensive care siblings. WES revealed a class 5 variant on the PRPS1 gene:
nology diseases were ruled out. Initial CK = 6.719U/L. unit. Medication adjustment was attempted without any (NM_002764.4) c.894A>C. Hence, a diagnosis within the
Muscle MRI revealed a heterogeneous edema pattern, improvement, besides developing Cushing’s syndrome spectrum of Arts syndrome and PRPS1-related disorders
with predominant proximal impairment. No evidence of secondary to the corticotherapy. Then a thymectomy was was made. Discussion: Arts syndrome is a rare entity
atrophy was detected. Statins were promptly withdrawn. chosen. The patient presented diaphragmatic paralysis described in 1993. Twelve young boys of a family presented
After 5 to 7 days, CPK lowered to normal level and muscle after the thymectomy, and a diaphragmatic plication was sensorineural hypoacusia, ataxia, delayed motor mile-
biopsy confirmed Tubular Aggregates Myopathy. There carried out with clinical improvement. The weaning of stones and tendency to upper respiratory tract infections.
was a complete improvement of muscle weakness after the corticotherapy was initiated. After six months she is Later, an autopsy showed absence of myelinated axons in
30 days. Discussion: Tubular Aggregate Myopathy is a found using exclusively prednisone 5mg/day and without the posterior column of the spinal cord. It is an X-linked
rare disorder of unknown prevalence. The symptoms myasthenic symptoms. Discussion: Myasthenia gravis condition caused by a loss-of-function mutation on the
and severity are widely heterogeneous, and the diagno- (MG) is an autoimmune disorder of the neuromuscular PRPS1 gene leading to decreased enzyme function. Besides
sis is based on a history of progressive loss of strength junction hallmarked by motor fluctuations (fatiguabil- Arts syndrome, mutations in PRPS1 have been associ-
and/or muscle pains, muscle biopsy revealing tubular ity). Treatment options are acetylcholinesterase inhibi- ated with a large spectrum of manifestations, as X-linked
aggregates, and absence of another neuromuscular dis- tors, glucocorticoids, immunoglobulin, plasmapheresis, recessive Charcot-Marie-Tooth disease-5 (CMTX5) and
ease. The Tubular Aggregate Myopathy can present as a immunosuppressives and monoclonal antibodies (eculi- X-linked deafness-1 (DFNX1). Adult female carriers can
pseudo metabolic feature, without progressive weakness, zumab). The antibodies for the diagnosis are anti-AChR, present isolated hypoacusia, as did the mother of our
mimicking the metabolic myopathies. Final Comments: anti-MuSK and anti-protein related to the lipoprotein 4 patients. We found description of 22 pathogenic variants
Tubular Aggregate Myopathy is a rare and an inherited (LRP4). The thymectomy is indicated based on the flaw in this gene, apart from the aforementioned new vari-
disorder and, differently from metabolic myopathies, of the thymic recognition function and negative selection ant. Clinical features of this case showed the presence of
is characterized by slowly progressive proximal muscle of autoreactive T cells – physiopathology implied mech- motor neuropathy, sensorineural hypoacusia and delayed
weakness, cramps and myalgia. Muscle biopsy usually anism of mediated MG AChR (MG-AChR). The thymec- motor milestones, which have been described in some
points to tubular aggregates of variable morphology and tomy is indicated on thymic persistence or on thymoma loss-of-function mutations. Gain-of-function mutations
type II fibers atrophy. presence, when is performed the mediastinal toilet. Not described so far are mostly related to hyperuricemia and
long ago thymectomy was not indicated in anti-MuSK gout arthritis. This is of great importance as a minor study
positive cases, but the last updates do not define thymec- has shown benefit from providing S-adenosylmethionine
Doenças Neuromusculares tomy as a resource or contraindicate. Interestingly, in this (SAM) to some patients. Description of a novel variant in
case, despite thymectomy’s possible inefficacy for anti- the PRPS1 gene may support the impression that according
MuSK positive patients, an unequivocally improvement to the mutation and residual enzyme function, different
and corticoids/pyridostigmine dosage reduction was clinical features are seen. Final Comments: We present a
observed after the thymectomy. Final Comments: The new variant in the PRPS1 gene related to Arts syndrome,
success of the therapeutic response after the thymectomy and a pure motor neuropathy could be a new finding
on an anti-MuSK positive patient puts in discussion the that had not been described so far. Segregation analysis
debate about thymectomy as a therapeutic weapon in suggests an X-linked inheritance. PRPS1 gene is related to
myasthenic anti-MuSK positive patients with persistent nucleotide metabolism and the synthesis of purines, and
thymus/ thymoma. identification of this mutation is of practical relevance as
SAM supplementation may be beneficial to these patients.
Doenças Neuromusculares
Doenças Neuromusculares

55
XXX Congresso Brasileiro de Neurologia

TL 1105358 TL 1105370 TL 1105398

RHABDOMYOLYSIS AND MUSCLE CHRONIC INFLAMMATORY DEMYELINATING JAG2-RELATED LIMB-GIRDLE MUSCULAR


ADAPTATION AFTER WHOLE-BODY POLYNEUROPATHY (CIPD) WITH UNILATERAL DYSTROPHY: FIRST REPORT IN LATIN
ELECTROSTIMULATION (WB-EMS) FACIAL AND HYPOGLOSSAL INVOLVEMENT: AMERICA AND DESCRIPTION OF A NOVEL
A CASE PRESENTATION VARIANT
Michelle Abdo Paiva, Ana Luisa Calich, José Pedro
Soares Baima, Edmar Zanoteli, André Macedo Marina Buldrini Filogonio Seraidarian, Paolla Leidys marina pedrozo, João Eduardo Bastianello,
Serafim Silva Giovanna Rossito Magalhães, Barbara Oliveira Bibiana Thomé, Jefferson Becker, Giordani dos
Paixão, Gabriella Braga da Cunha Silva, Ana Luiza Passos
Department of Neurology, Hospital das Clínicas da
Figueiredo Campos, Arthur Braga Pereira, Rodrigo
Faculdade Medicina da Universidade de São Paulo, Pontificia Universidade Católica de Rio do Sul –
Santiago Gomez
São Paulo SP, Brazil Porto Alegre – RS – Brazil
Hospital Sírio Libanês. São Paulo SP, Brazil Hospital Madre Teresa. Belo Horizonte MG, Brazil
leidyspedrozo@gmail.com
michelle.abdo@hc.fm.usp.br marinabuldrini@hotmail.com
Case Presentation: Two mixed-race siblings, non-con-
Case Presentation: A 25-year-old woman (BMI 22kg/ Case Presentation: A 31-year-old female with a two- sanguineous parents, Rio Grande do Sul, Brazil, referred
m2), without comorbidities, presented with an incidental months history of progressive weakness and paresthesia to our outpatient clinic this year at ages 35 (case 1) and
high creatine kinase (CK) level (29.068 U/L) after starting in both upper limbs, mainly distal, followed by a two-day 24 (case 2). Both had normal neuropsychomotor devel-
whole-body electrostimulation (WB-EMS). She had been right foot numbness. She referred transient and recovered opment up to 24 months and 12 months, respectively,
previously untrained and identified mild myalgia believ- weakness in the right hand three years prior to admission, when they developed progressive proximal weakness
ing it to be expected due to training. There was no urinary with normal exams e no follow-up needed. She had no on 4 limbs and lost the ability to walk at ages 8 and 7.
complaint or muscle weakness.Her neurological exam was comorbidities, antecedent infection or recent immuniza- Evolution of motor disability with increased frailty and
normal. Her renal function was normal and urine sample tion. She underwent Hospitalization and extensive labora- muscle atrophy in 4 limbs for the following 10 years, pre-
one week after the event showed no changes. Muscle MRI tory and radiological work-up. Cerebrospinal fluid analysis dominantly proximal in the lower limbs, associated with
demonstrated small foci of edema in gluteus maximus, vas- revealed high protein content (>90,8mg/dL), normal cell contractures with joint deformities and limited extension.
tus lateralis and intermedius muscles. We proceeded with count (WBC 1/mm3, 100% lymphocytes) and normal glu- Both presented dorsal scoliosis and mild cervical weak-
a complete investigation including full rheumatological cose level (54 mg/dL). Needle electromyography (EMG) ness. Case 1, at age 25 showed significant bulbar weak-
panel, thyroid function, serologies (HIV, hepatitis B and C, and nerve conduction velocity (NCV) study showed dif- ness, with dysphagia and dyspnea at rest with the need
dengue, leptospirosis, toxoplasmosis) and whole-exome fuse, symmetric, sensorimotor, predominantly primary for intermittent bilevel positive airway pressure. Case 2
sequencing which resulted all negative. Most plausible segmental demyelinating polyradiculoneuropathy. No does not present dysphagia or symptomatic dyspnea.
explanation was exertional rhabdomyolysis and we sug- other relevant findings were found. Initial treatment Both maintain support therapy with motor physiother-
gested a return to WB-EMS trainings with weekly serum included intravenous methylprednisolone and immu- apy. The initial investigation included: normal CK, EMG
CK measurement. The high CK level seen after individual noglobulin that shown immediate clinical improvement. showing myopathic pattern and muscle biopsy at ages
WB-EMS session was progressively blunted and normal- Outpatient management identified facial asymmetry two 4 and 8 (case 1) and at age 3 (case 2) with no conclusive
ized after 2 months of regular training. A control muscle months later, besides recurrency of incipient weakness and results. The molecular confirmation of the diagnosis was
MRI five months after regular training showed resolution numbness in extremities. A new curse of medication was only reached in 2022, by means of a next-generation mul-
of muscular edema. Discussion: WB-EMS uses an elec- carried out also with improvement in symptoms and bime- tigene sequencing (NGS) panel, which revealed a hetero-
trical stimulator with several channels that can recruit strial sessions were scheduled. On her next return, she zygous pathogenic variant in the JAG2 gene [T>TGGCCP.
more muscle fibers than normal exercises and attracts complained of difficulty speaking and eating. Neurological His1171ArgFS*9] that promotes amino acid substitution
people for the belief of being more time-effective. The exam evidenced right hypoglossal palsy that determined histidine in code 1171 by Arginine and change in the read-
case illustrates the risks of WB-EMS related to muscle dysarthria and impaired mobility of the bolus within the ing matrix from this point promoting early interruption
injury, especially in physically deconditioned people, mouth. No atrophy or tongue fasciculation were found. of protein translation in case 2.In addiction, case 1 was
and how to manage training-related CK level increase. New laboratory and radiological exams did not show any also tested with another NGS panel for neuromuscular
There are not enough data regarding the prevalence of abnormalities and 45-day interval course of medication conditions (which did not include JAG2), but it resulted
rhabdomyolysis associated with WB-EMS. However, it was restated with no further significant neurological in no relevant variants. Discussion: Autosomal reces-
has been previously reported especially after the first deterioration. Discussion: The usual phenotype of CIDP sive limb-girdle muscular dystrophy-27 (LGMDR27) is
workout session and studies show a drop in CK levels is a sensorimotor disorder with symmetric proximal and caused by pathogenic variants in the Jagged Canonical
after regular training. Our case reinforces that mainte- distal affectation, areflexia and conduction lowing, tem- Notch Ligand 2 (JAG2). It was first described in 2021 in
nance of WB-EMS training may be safe even after a very poral dispersion and/or conduction block. Cranial nerve a single international cohort with no reported cases in
high CK level. Muscle ability to adapt through exercise findings are not common: facial muscle weakness may Latin America. This variant is classified as pathogenic
training is the most probable explanation to justify serum be present in up to 13% and hypoglossal involvement has due to early interruption of protein translation that con-
CK increase attenuation. Final Comments: Patients and been reported in only 0–3.8%. Final Comments: CIDP is tributes to protein dysfunction in the notch signaling
health care professionals must be alert to intense muscle an immune-mediated chronic neuropathy with a wide pathway, which is important in intercellular signaling.
pain and changes in urine color, especially at the begin- spectrum of clinical manifestations, lasting for more Final Comments: This report expands the geographical
ning of training. Clearer protocols are needed to guide than two months. Although cranial nerve manifestation distribution of LGMDR27 to Latin America, describes the
physicians and neuromuscular practitioners to manage is infrequent (7%-16%), we describe this uncommon clinical features of two new cases of this rare condition
these patients as WB-EMS will become even more popu- finding in a patient with facial and hypoglossal involve- and contributes a novel variant affecting the JAG2 gene.
lar. Serial CK measurement may be necessary in subjects ment. Therefore, therapeutic strategies and prognostic
beginning WB-EMS training and an evaluation prior to implications of the presence of cranial neuropathy have
WB-EMS is advised to identify potential individual risks. yet to be determined and active examination to search Doenças Neuromusculares
this affection must be carefully accomplished.
Doenças Neuromusculares
Doenças Neuromusculares

56
XXX Congresso Brasileiro de Neurologia

TL 1105409 TL 1105431 TL 1105448

A DANCER WITH CRAMPS: MOERSCH- NUSINERSEN AND MULTIDISCIPLINARY URBANORUM SPP AND GUILLAIN-BARRÉ
WOLTMAN SYNDROME OR STIFF-PERSON APPROACH IN IMPROVEMENT OF MOTOR SYNDROME, A POSSIBLE ASSOCIATION?
SYNDROME FUNCTION IN SPINAL MUSCULAR ATROPHY
Emily Tainara Miranda Alves, Juliana Cardoso
TYPE 3: A 2 YEARS FOLLOW-UP CASE Fiquene, Aline Geisler, Barbara Jimenez
Laís Bissoli Perini, Fernando Zanette, Matheus
Marquardt, André Dias de Oliveira, Gisele Espíndola, PRESENTATION
Hospital Santa Isabel – Blumenau – SC – Brazil
Fernando Cini Freitas, Katia Lin Elisa Gutman Gouvea, Deborah Santos Sales, Vivian
Pinto de Almeida, André Bertola Vanzan Filho, emiily.taiinara@gmail.com
Universidade Federal de Santa Catarina.
Florianópolis SC, Brazil Mariana Ribeiro Pereira, Clarissa de Araujo Davico,
Rayanne da Silva Souza, Patricia Gomes Pinheiro, Case Presentation: A 57-year-old, white, male patient,
UFSC – Florianópolis SP, Brazil
Hospital Governador Celso Ramos – Florianópolis Carolina Garcia Nuñez Carrijo, Karina Lebeis Pires previously without comorbidities, presented with diar-
rhea lasting four days, with aqueous content, without
– SC – Brazil Universidade Federal do Estado do Rio de Janeiro. pathological products and with improvement after the
laisbperini@hotmail.com Rio de Janeiro RJ, Brazil use of symptomatic. After eight days, he started pares-
Casa Hunter. Rio de Janeiro RJ, Brazil thesia in feet and legs evolving with paraparesis and
Case Presentation: A 30 yo, female, psychologist and karinalebeis1@gmail.com needing support to walk. On admission, the neurological
dancer, previously healthy, non-consanguineous par- examination revealed asymmetric tetraparesis of proxi-
ents, without family history of neurological diseases. In mal predominance and areflex with Medical Research
Case Presentation: Male patient initiated progressive
2018, she noticed limited movement of her left leg and Council (MRC) with a total score of 24/60 and proteino-
weakness at 2-years-old. He was able to walk, but began
hip, especially when dancing and performing “pilates”, cytological dissociation in cerebrospinal fluid. Due to the
to have frequent falls and difficulty climbing stairs. At the
activities that she previously performed without com- neurological clinical condition and laboratory findings,
age of 14 he was referred to genetics due to a slow and pro-
plaints. She had severe painful cramps, associated with the diagnosis of Guillain Barre syndrome was suggested.
gressive worsening. Denied dysphagia, dyspnea, speech
intense stiffness that later migrated to her right leg up to Electroneuromyography was performed with signs of
or sphincter alterations. Upon examination, he presented
the lumbar column. She started physiotherapy with partial acute motor neuropathy, of axonal pattern, compatible
with proximal and symmetric weakness, associated with
improvement of the condition, but it was discontinued with motor axonal form (AMAN). Treatment with intra-
lower limb atrophy and 4-membered areflexia. In 2018,
in August 2020, which led to progressive worsening, now venous human immunoglobulin was then started on the
at the age of 23, he was diagnosed with Spinal Muscular
compromising her upper limbs and leading to fixed cer- third day of Hospitalization at a dose of 0.4kg/kg/day for
Atrophy (SMA) type 3 due to electroneurography and
vical hyperextension and hyperlordosis, confining her to 05 days. During Hospitalization, abdominal distension
genetic examination. He began treatment with nusin-
the wheelchair. She also developed solid-liquid dysphagia, associated with watery diarrhea was observed. Positive
ersen in 2019, along with multidisciplinary follow-up.
dysphonia, and dyspnea on minor exertion. Neurological stool parasitological examination and visualized urbano-
Motor function data were assessed by trained evaluators
examination revealed a fixed semi-flexed position of her rum ssp. with a positive fecal leukocyte test. In addition
using Hammersmith Functional Rating Scale Expanded
lower limbs, lumbar hyperlordosis, and a semi-flexed trunk to the therapeutic approach of the syndrome, treatment
(HFMSE) and Ten-Meter Walking Test (10 MWT), accord-
posture. Strength was grade 2 of the iliopsoas, thigh abduc- for diarrhea secondary to urbanorum spp was also insti-
ing to the international recommendations on SMA stan-
tors, and adductors; grade 4+ of the biceps and quadri- tuted with nitazoxanide for 07 days with improvement of
dard of care, at the onset of treatment and reassessed every
ceps femoris, and grade 5 on the other muscle groups. abdominal distention and reduction of evacuation fre-
six months. After 2 years, the patient increased 5 points
Generalized grade 3+ deep tendon reflexes with bilateral quency. Discussion: The high prevalence of parasites are
in the HFMSE and decreased 7s in the 10 MWT, which,
plantar flexion reflexes. Postural instability and inability sources of constant infections for the brazilian population
according to the literature, defines clinically meaningful
to walk. Baclofen and Pregabalin were administered up to due to its territorial extension and precariousness in terms
changes. Discussion: SMA is an autosomal-recessive
maximal doses, with no response. Clinical improvement of basic sanitation. knowing the new incident parasites,
lower motor neuron disease causing progressive muscu-
was observed only with Clonazepam 2 mg/d, with muscle their pathophysiology, virulence and possible associated
lar atrophy and weakness. It is divided into four subtypes
relaxation, resuming independent gait. An investigation diseases means that there are ways to combat and min-
(0-4) according to age of symptoms onset and achieved
was carried out with serology, rheumatological profile, imize the damage. A more detailed study of Urbanorum
motor landmarks. Nusinersen is an antisense oligonucle-
skull MRI and screening for neoplasms, with all negative spp, its morphological structure and mainly, which drug
otide administered intrathecally, able to increase func-
results. Normal CPK dosage. Serum dosage of Anti-GAD therapy to use will bring uniformity of information and
tional SMN protein levels, modifying SMA natural history.
with result > 2000 IU/ml. Therefore, clinical, laboratory, conduct. Awareness measures and basic hygiene care must
Although approved for any 5q SMA, most studies focus
and therapeutic response led to the final diagnosis of Stiff- be carried out to reduce transmissibility and reduce the
on nusinersen efficacy in infants and children, with data
Person syndrome, which is why electroneuromyography incidence of the parasite. Final Comments: We report the
on adults limited to few observational studies and case
was not performed at the time. Therapy continued with first case of clinical suspicion of a temporal association
series. This case shows clinical improvement in motor
Clonazepam, Prednisone 60 mg/day, Azathioprine 150 between Guillain-Barré syndrome and gastrointestinal
function of a SMA type 3 patient, even with the onset of
mg/day, and due to partial response to therapy, she is now tract disease caused by Urbanrum spp. Thus, seeking to
treatment being twenty years after the onset of symptoms.
awaiting infusion of Human Immunoglobulin. Discussion: alert about the probable relation of the parasite and the
This supports nusinersen efficacy in a long disease dura-
Due to its often prolonged evolution and rarity, the diag- risk of development of GBS, as well as the pathophysio-
tion setting. Furthermore, multidisciplinary management
nosis of Stiff-Person syndrome is still challenging, and logical mechanisms and treatment.
is a well established therapy designed to address the psy-
its diagnostic criteria are not yet well established. Final chosocial challenges of patients with SMA and cannot be
Comments: This hypothesis should be considered in excluded as an adjuvant in this patient’s enhancement.
all patients presenting muscle stiffness associated with Doenças Neuromusculares
Final Comments: In adult SMA, outcome measures
pain and muscle spasms, mainly in the lower back. There have not been as thoroughly standardized and validated.
are several differential diagnoses, and anti-GAD dosage Although it is proven that a longer disease duration has
should be requested for diagnostic aid. a negative impact on treatment efficacy, this report indi-
cates a satisfactory enhancement in unfavorable clinical
conditions. Also, a multidisciplinary approach acts as an
Doenças Neuromusculares
adjuvant for Nursinersen treatment in the improvement
of motor function.

Doenças Neuromusculares

57
XXX Congresso Brasileiro de Neurologia

TL 1105495 TL 1105559 TL 1105565

CHILDHOOD-ONSET MYOTONIC DYSTROPHY FAMILY WITH HSP 75 PHENOTYPE DUE TO LATE ONSET ASYMMETRIC MYOPATHY: A
TYPE 1: A CASE PRESENTATION MAG GENE VARIANT CASE OF NEUTRAL LIPID STORAGE DISEASE
Mariana Ribeiro Pereira, André Bertola Vanzan Glenda Barbosa Barros, Wladimir Bocca Vieira de Mariana Floriano Luiza Piva, José Lopes de
Filho, Elisa Gutman Gouvea, Pedro Ignacio Ferraz Rezende, Ana Carolina dos Santos Jorge, Paulo de Vasconcelos Júnior, Mateus Pradebon Tolentino,
Montenegro de Almeida, Daniel Lucas de Lima Lima Serrano, Helvia Bertoldo de Oliveira, Samia Pedro de Souza Domingues, Luisa Pacheco Avezum,
Silva Santos, Ana Carolina Frinhane Hergoz, Karina Rogatis Calil, Paulo Victor Sgobbi Souza, Igor Braga Kennedy Soares Linhares Silva, Flavio Vieira
Lebeis Pires, Carolina Garcia Nuñez Carrijo, Patricia Farias, Bruno de Mattos Lombardi Badia, Acary Marques Filho, Gleica Maria de Macena Kanawa,
Gomes Pinheiro, Clarissa de Araujo Davico Souza Bulle Oliveira Cristiane de Araújo Martins Moreno, Eduardo de
Paula Estephan
HUGG. Rio de Janeiro RJ, Brazil Universidade Federal de São Paulo. São Paulo SP,
Universidade Federal do Estado do Rio de Janeiro. Brazil Hospital Santa Marcelina. São Paulo SP, Brazil
Rio de Janeiro RJ, Brazil
Casa Hunter. Rio de Janeiro RJ, Brazil ana_carolinajorge@hotmail.com marianaflpiva@gmail.com

pribeiromariana@gmail.com Case Presentation: A 47-years-old man presented with Case Presentation: We present a male patient, 42 years-
a slowly progressive history of weakness in the left lower old, active smoker, without comorbidities. At 38 years of
Case Presentation: Thirteen year old girl with cramps and limb since age 35 years. After 1 year, he noticed involve- age, he developed an asymmetrical proximal weakness
pain on both hands that initiated during early childhood ment of the right lower limb, with gradual worsening of in the upper limbs. Over the next 04 years, there was
and got worse after coronavirus disease 2019 (COVID-19) gait. Subsequently, onset of paresthesia in the feet asso- slowly progressive worsening of weakness in the upper
in november of 2020. She had myotonic phenomena at ciated with episodes of urge urinary incontinence. It has limbs and evolution to the lower limbs. On neurological
both hands and tongue, mild proximal weakness on both alpha-1 antitrypsin deficiency. In the family history, the examination, the patient had asymmetric tetraparesis,
hip flexors and hip extensors, and a myopathic gait. No father had a history of progressive spastic paraparesis, with proximal predominance in the upper limbs and distal
history of neurologic or genetic disorders in the family starting at age 60 years, with recurrent falls associated predominance in the lower limbs, with grade 2 strength
was reported. Laboratory investigation of nutritional defi- with cognitive decline over 7 years and urinary incon- at arm abduction bilaterally, grade 2 for forearm flexion
ciencies, endocrine and other systemic involvement was tinence. He later progressed with dysarthrophonia and and 4 for extension bilaterally, but more prominent on
negative. Electromyography showed myotonic myopathy. dysphagia, and death occurred 14 years after the onset of the right side. Dorsiflexion of the right feet was grade 2,
Genetic testing helped conclude the diagnosis of Myotonic motor symptoms. Parental consanguinity was not related. and 4 on the left. Selective atrophy of the biceps brachii
Dystrophy type 1 (DM1). Discussion: DM1 is the result of The examination disclosed crural paraparesis, global muscle with sparing of the triceps brachii were remark-
a CTG repeat expansion in the 3? untranslated region of hyperreflexia, spasticity in the dorsiflexion of the feet, able. Investigation showed serum creatine kinase of 3.033.
the DM1 protein kinase (DMPK) gene on chromosome hypoesthesia in the left foot and bilateral positive Babinski Electromyography had a pattern of asymmetric myop-
19q13.3. The clinical triad associated with DM1 is myo- sign. Biopse of muscle is unremarkable. A genetic panel athy with proximal predominance, nerve conduction
tonia, distal weakness and early-onset cataracts. Patients was collected, showing a genetic variant of uncertain study was normal. Biceps brachii muscle biopsy showed
may refer to myotonia as cramps, muscle stiffness or significance (with “in silico” prediction of pathogenic- muscle fibers with intracytoplasmic vacuoles and intra-
delayed muscle relaxation. Proeminent pain and proxi- ity) heterozygous mutation in MAG (Myelin Associated cytoplasmic masses, alterations suggestive of metabolic
mal weakness are features most associated with Myotonic Glycoprotein). Thus, we concluded that our family pre- myopathy. A genetic panel was performed and showed a
Dystrophy type 2 (DM2), although case presentation with sented hereditary spastic paraplegia 75 (HSP – 75) with homozygous likely pathogenic variant in PNPLA2, ( c.421-
this presentation in DM1, such as our patient, have been cognitive involvement by the MAG gene. Discussion: 1G>A) consistent with neutral lipid storage disease with
previously reported. Childhood-onset of DM1 is character- Hereditary spastic paraplegias (HSP) are a heterogeneous myopathy (NLSDM). Discussion: Neutral lipid storage
ized by symptoms initiated between after the completion group of neurodegenerative diseases and more than 70 disease with myopathy (NLSDM) is a metabolic myopathy,
of the first year of life to ten years old. A childhood-onset genes have been implicated. Although most variant con- characterized by the presence of triglyceride-containing
is more linked to intellectual impairment and gastrointes- texts occur in an autosomal recessive scenario in HSP75, cytoplasmic droplets in leukocytes and in other tissues,
tinal symptoms, such as diarrhea, constipation and fecal previous descriptions (adult-onset spastic paraparesis, including bone marrow, skin, and muscle. NLSDM is an
incontinence. Common adult features of myotonia do not action tremor in the hands, cognitive decline) with an autosomal recessive muscle disorder caused by mutations
present until early adolescence. Our patient describes that autosomal dominant inheritance pattern have already in the PNPLA2 gene. Usually presents with juvenile-onset
pain and cramps got worse after coronavirus infection in been reported and justify the presented clinical context by (after the first decade) of slowly progressive proximal mus-
2020 and that was what motivated the family to search for patients. Final Comments: Clinicians should be aware of cle weakness with increased serum creatine kinase; distal
medical counseling. At our best knowledge, there are no the possibility of HSP75 presentation by the MAG gene in muscle weakness may also occur. Multisystem involve-
reports of worsening Myotonic Dystrophies after corona- the context of autosomal dominant inheritance. ment with cardiomyopathy, dyslipidemia, hepatomegaly,
virus infection. But patients with DM1 may be at higher and/or diabetes is present in 20 percent or more of cases.
risk for presenting severe acute respiratory syndrome Our patient had a late onset of weakness and absence of
(SARS) associated with COVID-19. Final Comments: Doenças Neuromusculares multisystem involvement, with neurological examination
DM1 is a genetic disorder with onset described between and complementary work up compatible with metabolic
birth and early adulthood associated with three cardinal myopathy due to lipid accumulation. Final Comments:
features (myotonia, weakness and early-onset cataracts). Until the present date of submission of this report, no
Proximal weakness and pain are not the most prominent registry was found for the genetic variant c.421-1G>A on
symptoms in patients with DM1, although previously PNPLA2, making this a probably novel pathogenic variant.
reported. Patients with childhood-onset are expected to Our case study highlights the importance of considering
have a more severe course than those with adult onset, neutral lipid storage disease for patients with late onset
and an early recognition of symptoms may lead to a better asymmetric myopathies.
quality of life in these patients.

Doenças Neuromusculares
Doenças Neuromusculares

58
XXX Congresso Brasileiro de Neurologia

TL 1105569 TL 1105578 TL 1105580

CHARCOT MARIE-TOOTH DISEASE DEMYELINATING NEUROPHYSIOLOGICAL REFRACTORY RE-THYMECTOMY AND


(CMT) SUPERIMPOSED ON CHRONIC FINDINGS IN FAMILY AMYLOID IMMUNOTHERAPY IN MYASTHENIA GRAVIS:
INFLAMMATORY DEMYELINATING POLYNEUROPATHY CASE PRESENTATION
POLYRADICULONEUROPATHY (CIDP) IN A
Larissa Bittencourt de Carvalho, Ligia Rocha André Teixeira Souza Castro, Ronaldo Maciel Dias,
PATIENT WITH A HISTORY OF CHILDHOOD Andrade, Izabela Jardim Rodrigues Pitta, Clarissa Andre Gustavo Fonseca Ferreira, Ernane Pires
POLIOMYELITIS: A CASE PRESENTATION Neves Spitz, Rômulo Heckert, Vital Teixeira Robson, Maciel, Wagner Afonso Teixeira, Rodrigo Torres Sena
Eduardo Rodrigues Davidovich, Salim Lázaro Pita, Robercon Alves Carmo, Fernanda Sousa Lima,
Helen Maia Tavares Andrade, Bruna Franchito
Balassiano, Marcia Rodrigues Jardim Larissa Lucas Schloika, Carolina Flores Welker
Freire, Adriano Marcelo Silva Filho, Lucas Arone
Belmonte Hospital Universitário Pedro Ernesto. Rio de Janeiro Hospital de Base do Distrito Federal.HBDF
RJ, Brazil
Pontifícia Universidade Católica de Campinas. andretsscastro@gmail.com
Hospital Federal Antônio Pedro. Rio de Janeiro RJ,
Campinas SP, Brazil
Brazil
helenmaiatandrade@gmail.com Case Presentation: A 41-year-old patient, female, pre-
laribit@hotmail.com viously healthy, presented to this service in 2017 with a
myasthenic syndrome, referring progressive bulbar mus-
Case Presentation: A 68-year-old male patient presented
Case Presentation: A 69-year-old man presented with culature weakness associated with bilateral ptosis without
weakness in lower limbs, myalgia, paresthesia in the
paraesthesias, significant weight loss, fasciculations and fatigability maneuver, being later diagnosed with myas-
feet and hands, initiated 9 months ago, with progressive
proximal and distal weakness in the four limbs. His clinical thenia gravis (Osserman Classification IIIa). In clinical-ra-
worsening. He denied loss of sphincter control, dyspnea,
picture had begun less than a year ago. Physical examina- diological evaluation (CT), a mass with 60 cm³ of volume
dysphagia and infections preceding the onset of the
tion of the patient showed diffuse arreflexia, thermoalgic was identified in the anterior mediastinum, and subse-
condition. History of childhood poliomyelitis with left
hypoesthesia with dependent length pattern and flaccid quent histopathological analysis of Thymoma (Masaoka
lower limb atrophy. Neurological examination showed
tetraparesis. There was no cranial nerve dysfunction. I) was associated with the positivity of Anti-AChR (1,28).
bilateral pes cavus, hammer toes and lower limbs in a
Electroneuromyography showed polyradiculoneuropathy She has been refractory to traditional therapy methods,
characteristic “inverted champagne bottle” appearance.
with mixed axonal/demyelinating pattern that met crite- like using glucocorticoids, Intravenous Immunoglobulin
The cranial nerves were all intact. Motor examination
ria for chronic inflammatory demyelinating polyradicu- (IVIG), and acetylcholinesterase inhibitors in optimized
showed reduced muscle strength (MRC grades were 4 for
loneuropathy (CIDP). Cerebral spinal fluid did not show dosage, keeping the progression of symptoms and being
lower limbs and 5 for upper limbs) and hyporeflexia in
significant albuminocytological dissociation. The patient at risk of hypercapnic respiratory failure. As a result of
upper and lower limbs. Sensory examination revealed a
underwent intravenous corticosteroid therapy with meth- recent scientific evidence regarding the surgical benefit
decreased vibratory sense in the distal lower and in upper
ylprednisolone, without satisfactory clinical response. of thymectomy in these select patients, she underwent a
limbs. The coordination test assessment showed ataxic gait
He was then submitted to a genetic panel of neuromus- Video-assisted Thoracoscopic Surgery (VATS) to remove
aggravated by closing eyes and dysmetria on the left side.
cular diseases, which identified the Ile127Val mutation, the mass. However, despite IV pulse therapy and the mea-
Cerebrospinal fluid (CSF) revealed albuminocytological
known to be pathogenic for transthyretin family amyloid sures previously adopted, the patient continued to experi-
dissociation (leukocytes: 1,56 cell/mm3 and proteins:
polyneuropathy (TTR-FAP). Discussion: Although the ence progressive clinical worsening, requiring a definitive
116,4 mg/dL). Electroneuromyography demonstrated
typical neurophysiological finding of TTR-FAP is axonal airway proper to hypoxemia and carbon dioxide narcosis.
a chronic demyelinating sensory-motor polyradiculo-
sensory-motor polyneuropathy, it is common to observe Due to the recurrence of the disease, it required immuno-
neuritis associated with a bilateral chronic neuropathic
heterogeneous patterns of presentation in electroneuro- therapy with Rituximab, Cyclophosphamide, and a second
process, without current denervative activity, in bilateral
myography. There are reports in the literature of clinical surgical re-approach for a new removal of the recurrent
myotomes from L2 to S1, consistent with the history of
and nerurophysiological presentations that mimic CIDP. mass. Since then, there has been partial improvement in
childhood poliomyelitis. The patient has been submitted
Demyelination findings are probably secondary to axonal fatigability control through this second investigation of
to monthly pulse therapy with methylprednisolone 1g/day
degeneration. Final Comments: The variability of TTR- ectopic secretion of anti-AchR. Discussion: Myasthenia
for 5 days, with progressive improvement of the condition.
FAP manifestations may delay it’s diagnosis, which may gravis is a neuromuscular transmission disorder result-
Discussion: In the first evaluation, the patient has lower
directly compromise the indication of treatment for these ing from the binding of autoantibodies to components
limbs deformities characteristic of CMT, but due to the
patients. In these cases, knowledge about the neurophys- of the neuromuscular junction (NMJ), most commonly
presence of progressive loss of muscle strength, the CIDP
iological findings of FAP can be used as an auxiliary tool the acetylcholine receptor (AChR). These autoantibodies
hypothesis was raised and corroborated by protein-cyto-
in the differential diagnosis. are directly responsible for the pathological change in the
logical dissociation. Although the coexistence of Charcot-
endplate – being dependent on CD4+ T and B cells. It is
Marie- Tooth disease with CIDP is already established in
characterized by fatigable muscle weakness, with a vari-
the literature, the inflammatory diagnosis superimposed Doenças Neuromusculares able course with a tendency to progress to distal muscles.
on the hereditary neuromuscular process is not always
A particular subpopulation is restricted to ocular and
easy. This case was even more challenging from a clinical
facial manifestations. Other autoantibodies involved are
and electrophysiological perspective because of the his-
anti-MuSK and anti-LRP4, acting on proteins responsible
tory of childhood poliomyelitis. Final Comments: This
for maintaining the NMJ and on the ability to emit action
case presentation highlights the importance of consid-
potentials at the endplate, respectively. Final Comments:
ering clinical exacerbation of CMT due to the possibility
In this presentation, we describe a patient with refractory
of superimposed CIDP. The recognition of superimposed
Myasthenia gravis, leaving the usual pattern of clinical
inflammatory neuropathy, although difficult, is of high
control and suggesting the importance of the thymus in
clinical importance since with specific treatment the
the pathophysiology.
patient’s clinical condition can be improved.

Doenças Neuromusculares Doenças Neuromusculares

59
XXX Congresso Brasileiro de Neurologia

TL 1105585 TL 1105586 TL 1105590

RESISTANT MYASTHENIA GRAVIS WITH RITUXIMAB THERAPY IN THE TREATMENT RECIDIVED THYMOLIPOMA APPROACH
RITUXIMAB AND RE-THYMECTOMY OF STIFF-PERSON SYNDROME: A CASE FOR MYASTHENIA GRAVIS FATIGABILITY
APPROACH: CASE PRESENTATION PRESENTATIONS SERIES CONTROL: CASE PRESENTATION
André Teixeira Souza Castro, Ronaldo Maciel Dias, Mateus Barros Bueno, Maria Cecília Inácio, Clarice André Teixeira Souza Castro, Ronaldo Maciel Dias,
André Gustavo Fonseca Ferreira, Ernane Pires Pereira Sales Oliveira, Isabela Corrêa Samper, André Gustavo Fonseca Ferreira, Ernane Pires
Maciel, Wagner Afonso Teixeira, Rodrigo Torres Sena Laura de Lourdes Cardoso e Silva, Gabriela Tomás Maciel, Wagner Afonso Teixeira, Rodrigo Torres Sena
Pita, Robercon Alves Carmo, Fernanda Sousa Lima, Alves, Leonardo Peixoto Garcia, João Paulo Moreira Pita, Robercon Alves Carmo, Fernanda Sousa Lima,
Larissa Lucas Schloika, Carolina Flores Welker Fernandes, João Augusto Nunes Vitorino, Diogo Larissa Lucas Schloika, Carolina Flores Welker
Fernandes dos Santos
Hospital de Base do Distrito Federal.HBDF Hospital de Base do Distrito Federal.HBDF
Universidade Federal de Uberlândia. Uberlândia
andretsscastro@gmail.com MG, Brazil andretsscastro@gmail.com

Case Presentation: A 32-year-old patient, female, previ- bueno.mateus22@gmail.com Case Presentation: A 26-year-old woman presented to
ously healthy, presented to this service in 2016, implying our Neurologic Emergency Department, referring uni-
bulbar musculature weakness with dysphagia and dys- Case Presentation: Stiff-person syndrome (SPS) is char- lateral ptosis and dysphagia associated with fatigability
arthria. Due to the fatigability pattern of bulbar muscu- acterized by a huge axial and proximal members stiffness of shoulder girdle muscle compartment as a myasthenic
lature, there was a diagnostic suspicion for Myasthenia associated with spasms. Treatment is basically symptom- syndrome was hypothesized. Lately, neurophysiologic
gravis, which was later confirmed by humoral dosage of atic, based on the use of benzodiazepines and baclofen study with Electromyoneurography (EMNG) and humoral
anti-AchR (61) with negative anti-MusK. Subsequently, as the main therapeutic interventions. However, there are analysis of autoantibodies has been positive (anti-AchR
despite optimizing measures adopted, such as Intravenous few studies that describe the benefit of using immunosup- 1,0 nmol/L), suggesting autoimmune Myasthenia gravis
Methylprednisolone (IVMP) pulse therapy, acetylcholin- pressants in this condition. Case 1: A 30-year-old female diagnosis. Despite using Immunomodulators such as
esterase inhibitors, and Intravenous Immunoglobulin patient complained of low back pain radiating to the lower Azathioprine, Glucocorticoid therapy, Therapeutic Plasma
(IVIG), the patient presented progressive clinical wors- limbs and frequent falling episodes. She presented a clau- Exchange (TPE) and Intravenous Immunoglobulin (IVIG)
ening with consequent respiratory failure suitably to dia- dicating gait, hypertonia of the paravertebral, abdominal in addition to pyridostigmine, the patient remained with
phragmatic loss (Osserman Classification V). Therefore, and lower limb muscles. After using benzodiazepines, the the breakthrough symptoms (Osserman Classification IV)
properly to refractoriness of previous measures, spe- patient reported a partial improvement of symptoms, but and specialists proposed a thymectomy while an attempt
cialists recommended a Video-assisted Thoracoscopic maintained hypertonia and mild pain. Anti-GAD serol- to control the ectopic secretion of antibodies. The patient
Surgery (VATS) excision and histopathological anal- ogy showed high titers. With immunoglobulin therapy, underwent the mediastinal mass removal (12 x 10 x 2.5
ysis of the thymus, as it is associated with possible ambulation has improved, but falls and painful stiffness cm) with posterior histopathological analysis of disclosed
humoral hypersecretion and consequent development persisted. She was then treated with infusions of rituximab thymic gland tissues with an abundant mature adipose tis-
of cross-reactions and autoimmune lesions. As a result, every 6 months. After 2 years, she reports improvement sue, suggesting a rare neoplasm thymolipoma, considered
the Immunohistochemical Profile (IP) evidenced thymic of symptoms and life quality, resuming the practices of a possible factor with humoral discharge. After surgical
hyperplasia without atypia. However, after the procedure, their activities of daily living. Case 2: A 43-year-old female excision, there was partial maintenance of symptoms for
the patient remained with muscle fatigability requiring patient complained of pain in the lower limbs, limiting a certain period, with recurrence of the tumor evidenced
clinical control through Azathioprine and immunother- stiffness and recurrent seizures. She presented hypertonia by a CT scan approximately 04 years after the procedure,
apy with Rituximab – since there is no availability of the of paravertebral musculature and high titers of anti-GAD. performing a re-thymectomy proposal through thora-
monoclonal antibody Eculizumab at our disposal. Then, She had already been unsuccessfully treated with immu- coscopy approach for clinical control. Thenceforth, she
we proposed a second approach to investigate ectopic tis- noglobulin. During 2 years she was treated with benzodi- has moderate fatigability with immunotherapy, avoiding
sue with complete resection of pericardial and mediastinal azepines and presented clinical instability with sporadic clinical decompensation such as diaphragmatic loss in
fat. The analysis showed a small aggregate of lymphoid seizures and no important recovery from the symptoms. myasthenic crisis. Discussion: Myasthenia gravis (MG)
tissue in re-thymectomy. Since then, the patient has had After rituximab therapy with infusions every 6 months, is a neuromuscular transmission disorder resulting from
adequate clinical therapy, maintaining optimized doses there was a significant clinical and life quality improve- the binding of autoantibodies to components of the neu-
according to guidelines for fatigability control. Discussion: ment. Case 3: A 46-year-old male patient complained of romuscular junction (NMJ), most commonly the ace-
Myasthenia gravis is a neuromuscular transmission dis- lumbar and lower limbs pain, limitation of movement, tylcholine receptor (AChR). These autoantibodies are
order resulting from the binding of autoantibodies to frequent epileptic seizures. He presented hypertonia of directly responsible for the pathological change in the
components of the neuromuscular junction (NMJ), most the paravertebral muscles and lumbar lordosis. Anti- endplate – being dependent on CD4+ T and B cells. The
commonly the acetylcholine receptor (AChR). These auto- GAD titers showed elevated values. After unsuccessful incidence of Thymolipoma is around 0.12 out of 100.000
antibodies are directly responsible for the pathological immunoglobulin therapy, he underwent infusions of rit- cases per year, including 34 documented cases reporting
change in the endplate – being dependent on CD4+ T uximab interspersed over 6 months. There was a recovery the association between Myasthenia gravis and this spe-
and B cells. Multicentric trials recommend a sternotomy of motricity and stiffness and complete cessation of pain, cific tumor. The exact mechanisms remain unclear; how-
approach with en bloc resection of all mediastinal tissue. contributing to functional improvement and resumption ever, there is guidance for en bloc resection of all anterior
This occurs due to microscopic thymic components that in the practice of their work activities. Discussion: Most mediastinal tissue in order to symptomatic control. Final
are not macroscopically identifiable. A focused and con- common type of SPS is associated with the presence of Comments: In this presentation, we describe a patient
servative approach remains in question. Final Comments: anti-GAD impairing the GABA function. Rituximab is a with refractory Myasthenia gravis, leaving the usual pattern
In this presentation, we describe a patient with resistant monoclonal antibody against CD20, expressed on the of clinical control and suggesting the importance of the
Myasthenia gravis, leaving the usual pattern of clinical surface of B cells. Its use in SPS is still a recent approach. thymus in the pathophysiology. However, the complete
control and suggesting the importance of the thymus in Final Comments: Rituximab may be a benefit drug option remission rate of symptoms needs further studies for a
the pathophysiology. in SPS treatment. However, these reports show partial reliable conclusion.
results, requiring long-term follow-up.

Doenças Neuromusculares Doenças Neuromusculares


Doenças Neuromusculares

60
XXX Congresso Brasileiro de Neurologia

TL 1105602 TL 1105642 TL 1105750

VAMP1-RELATED PRESYNAPTIC CENTRONUCLEAR MYOPATHY: CASE PROGRESSIVE ENCEPHALOMYELITIS


CONGENITAL MYASTHENIC SYNDROME PRESENTATION WITH RIGIDITY AND MYOCLONUS (PERM)
MIMICKING SEPSIS IN STIFF-PERSON
Alulin Tácio Quadros Santos Monteiro Fonseca, Antonio Serpa Amaral Neto, Matheus Marquardt,
Eduardo P Stephan, Pedro Henrique Marte de Lais Bissoli Perino, Fernando Netto Zanette, Priscila SYNDROME
Arruda Sampaio, José Marcos Vieira Albuquerque Bernardi, Gisele Espíndola Gustavo Manginelli Lamas, Jessica Daniele
Filho, Acary Souza Bulle Oliveira Marques, Lucas Melo Teixeira Branco
HU-UFSC – Florianópolis – SC – Brazil
Universidade Federal de São Paulo. São Paulo SP, Universidade Estadual de Campinas. Campinas SP,
Brazil amnetomedunir@gmail.com
Brazil
USP, São Paulo SP, Brazil
Case Presentation: Female, 47-year-old, previously mlamas.gustavo@gmail.com
josemarcosvieirafilho2@gmail.com healthy, started at age 23 with a condition of distal, pro-
gressive and mild weakness in the lower limbs, with Case Presentation: A 65-year-old female patient, pre-
Case Presentation: 21-year-old male, resident in a long- stumbling and falls, but without limitations in activities of viously diagnosed with anti-GAD positive Stiff Person
stay institution for patients with cerebral palsy since the daily living. After the age of 40, she began to have difficulty Syndrome (SPS), on treatment with monthly corticoste-
age of 5 months. Abandoned by his mother and with no climbing stairs and getting up from chairs, in addition to roid cycles, was seen due to a complaint of pustular skin
information about his father or family history. There are being unable to carry bags or purses anymore. No report of lesions after completion of the last methylpredisolone
no reports of problems during pregnancy or childbirth. At bulbar symptoms. Past history: no comorbidities. Family infusion. She was treated with topical corticosteroid
5 months of age, clinical evaluation showed microcephaly history: mother and younger brother (40 years old) with and anti-histaminic agents, but with no improvement
(-2.5 SD), global hypotonia, with decreased spontaneous similar neurological picture. Neurological examination was observed. About three weeks later, she developed
movement of limbs, proximal and distal, with semiflex- shows mild eyelid ptosis, especially on the right. facial high fever, confusion, prostration, and worsening of
ion of the wrists. CK was normal. The patient needed diparesis. Divergent strabismus evident in the right eye. generalized muscle stiffness. Physical examination upon
nocturnal BiPAP and exclusive G-tube feeding.Initial Muscle strength by the Medical Research Council Scale admission evinced significant limb stiffness and to dif-
diagnosis was congenital myopathy. Muscle biopsy was demonstrating grade 4 in the upper and lower limbs fuse papular lesions in the pelvic region, dorsum, and
performed at 3 years of age and showed increased variation distally, Grade 3 in foot dorsiflexion and grade 4 in flex- buttocks, with associated hyperemia. She was admit-
in fiber size, type I and type II fiber atrophy, with rounded ion. Grade 4 quadriceps and bilateral gluteus medius. ted with a hypothesis of sepsis of cutaneous focus, and
fibers and slight increase in endomysial connective tis- Muscle hypotonia. Grade 1 deep reflexes globally except empirical antibiotic therapy was initiated. Laboratory
sue. In an evaluation at 11 years of age, hypotonia and for abolished Achilles reflexes, bilateral Achilles tendon tests showed worsening of the renal function and mild
global muscle hypotrophy were observed, with proximal retraction, bilateral cutaneous-plantar flexion reflex. leukocytosis. The patient evolved with generalized ton-
and distal muscle weakness (MRC grade 3 and grade 2, No winged scapula. Absence of myotonic phenomena. ic-clonic seizures, worsening of hyperthermia, presenting
respectively). Deep tendon reflexes were absent. There Sensitivity preserved. Myopathic gait, not walking on a temperature of 41 Celsius degree, despite the use of high
were contractures on the knees, wrists, fourth and fifth tiptoes and heels. Laboratory tests demonstrate CPK at dose antipyretics, in parallel with decreased level of con-
fingers, with thoracic kyphosis, everted ankles, and flat normal levels. ENMG (2021): signs of primary muscle fiber sciousness, requiring orotracheal intubation. Antibiotic
feet. The face had a myopathic appearance with a high- involvement, associated with signs of active denervation therapy was optimized to cover central nervous system
arched palate. Ophthalmoparesis was predominant in in most motor units examined, predominantly proximal infection, but all infectious screening with blood cultures,
vertical gaze but became global with the progression of and lower limbs.Genetic Testing: Identified heterozygous cerebrospinal fluid analysis, and chest and abdominal
the condition. Dysarthria was severe with barely intelligi- pathogenic variant DNM2 c.1393C>T (p. Arg465Trp). imaging tests were negative. White blood cell count and
ble speech. Molecular panel for neuromuscular diseases Discussion: Mutations in the DNM2 gene lead to con- C-reactive protein were within normal limits, although
was performed at 19 years of age which demonstrated genital centronuclear myopathy (CCM) and two forms of he persisted with hyperthermia maintained between 39
a homozygous nonsense variant in the VAMP1 gene Charcot-Marie-Tooth (CMT) in the autosomal dominant and 40 degrees. Considering infectious causes excluded
(c.97C>T; p.Arg33*). Electromyography (EMG) demon- form. NCM manifests as a disorder of progressive deteri- as the etiology of the decompensation, it was decided
strated increased insertional activity, with fibrillations oration and delays in motor milestones, facial and gen- to schedule immunotherapy treatment considering the
and positive sharp waves, as well as markedly polyphasic eralized muscle weakness, ptosis, and ophthalmoplegia. diagnosis of Progressive Encephalomyelitis with Rigidity
motor unit action potentials, with reduced recruitment Some patients also develop pes cavus before experiencing and Myoclonus (PERM), and the patient underwent
and interference pattern. The above findings suggested a symptomatic muscle weakness. CNM patients with DNM2 plasmapheresis with marked and clinical improvement.
severe motor neuropathy, but a muscle ultrasound showed mutations had mild axonal neuropathy, which may or Final Comments: PERM is a rare SPS spectrum disorder
a hyperechogenic pattern, without marked muscle atro- may not have reduced motor conduction velocities. Final characterized by severe prognosis and high mortality.
phy, suggesting that a severe neuromuscular dysfunction Comments: This case illustrates a patient with late-onset Clinical manifestation include level of consciousness
may be responsible for the loss of CMAP amplitude and and slowly progressive Centronuclear Myopathy, associ- impairment, dysautonomia and brainstem dysfunction
EMG changes. Pyridostigmine was then prescribed up to ated with pes cavus and global hyporeflexia, with normal along with typical SPS phenomenom. Diagnosing PERM
a dose of 300 mg/day. Improvement after the drug treat- levels of CPK, ENMG with myopathic pattern and con- might be challenging, since some of these manifestations
ment appeared to be slight, with a mild improvement in firmed by genetic testing. may overlap with other more commom diseases, such as
the movement of the upper limbs and voice turned out a infections. Neurologists should be aware of PERM as it is
little louder. VAMP1-associated presynaptic CMS is a rare a potentially treatable condition; patients often improve
form of disease from a group that is already uncommon. Doenças Neuromusculares
with aggressive immunotherapy.
All cases appear to be consistent with severe congenital
muscle weakness and hypotonia, with facial and bulbar
involvement, and early feeding difficulties. Some patients Doenças Neuromusculares
responds to pyridostigmine.

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XXX Congresso Brasileiro de Neurologia

TL 1105751 TL 1105756 TL 1105789

CASE PRESENTATION BILATERAL PROGRESSIVE MUSCLE WEAKNESS AND INCLUSION BODY MYOPATHY AND
GASTROCNEMIUS MYOSITIS ASSOCIATED DYSPHAGIA IN AN ELDERLY PATIENT: FRONTOTEMPORAL DEMENTIA DUE TO VCP
WITH INFLUENZA-LIKE SYMPTOMS CHLOROQUINE MYOPATHY GENE MUTATION: CASE PRESENTATION
Rafaela Luiza Altheia Griza, Renato Endler Iachinski, Matheus Compart Hemerly, Larissa Peres Delgado, Victor Fellipe Bispo Macedo, Larissa Clementino
Gabriel Angelo Garute Zenatti, Angelo Marcelo Beatriz Gioppo Betini, Helder de Lima Ribeiro, Andre Leite Sá Carvalho, Paulo Barbosa Leite Neto, Marco
Wosniacki Filho, Eduarda Basso Badalotti, Lucas Cleriston Jose dos Santos, Ana Beatriz Gonçalves André Moraes Bernardino, Djanino Fernandes
Victoy Guimarães Zengo, Marcia Bernadon, Vinicius Zanovello, Wilson Marques Junior, Claudia Ferreira Silva, Cacia Caroline Carvalho Silva, Pedro Nogueira
Slongo Buhler, Maria Eduarda Dall’Oglio Whitaker da Rosa Sobreira Fontana, Fabíola Lys Medeiros, Paulo roberto Brito-
Marques, Carolina Cunha Correia
Fundação Hospitalar São Lucas. Cascavel PR. Brazil Hospital das Clínicas da Faculdade de Medicina de
Ribeirão Preto. Ribeirão Preto SP, Brazil Hospital Universitário Oswaldo Cruz, Universidade
rlagriza@gmail.com de Penambuco. Recife PE, Brazil
matheuscompart@hotmail.com
Case Presentation: The patient is a 34 year old male of victorfmacedo@gmail.com
previous good health. It was his second day of symptoms Case Presentation: A 70 years-old woman sought medi-
when he went to the doctor complaining of headache, cal care due to unintentional weight loss of 10 to 15kg in Case Presentation: A 51-years-old female presented with
sore throat, body ache and fever. The oropharynx had a 2 months. There was progressive weakness, dysphagia progressive muscular weakness for 10 years. She got worse
diffuse hyperemia and exudates at the tonsillar crypts. and dysphonia. She had been treated for gastric cancer from weakness in the past year and started complaining
The patient was discharged with Amoxicillin for 7 days, and arthritis and was under the use of chloroquine, nor- of forgetfulness, anomia and periphrases. She developed
symptomatic medication and diagnosed with acute ton- triptyline, desvenlafaxine and a combined formula of apraxia for daily activities, frequent falls and joint pain.
sillitis. Three days later he returned with loss of strength in indomethacin, prednisone and famotidine. Neurological Her sister was diagnosed with Paget’s disease of bone at
arms and legs, also feeling better from the odynophagia examination disclosed generalized muscle wasting and age 20, and has language and behavioral changes. Her
and body pains. At the neurological examination nothing weakness, with marked involvement of proximal, axial father died at advanced age with proximal weakness and
pathological was found just as the cranial CT. The patient and bulbar muscles. CK was elevated. Investigation for behavioral changes without confirmed diagnosis. On
was Hospitalized and a lumbar puncture was performed, paraneoplastic syndrome was normal. She was admitted physical examination, the patient was thin, bald and dis-
the liquor analysis had non pathological signs. All the lab- to our Hospital to receive clinical and nutritional care and oriented in time and place. There was wasting of trapezius
oratorial routine exams didn’t show any abnormalities. complete the investigation. The EMG showed increased and deltoid muscles, proximal quadriparesis (MRC IV),
On the second day of Hospitalization an increase of the spontaneous activity at rest and findings consistent with normal tonus and reflexes, abolished plantar responses,
pain was noticed on bilateral calf muscles. Prednisone a myopathic pattern. The muscle biopsy showed a vac- difficulty in arising from chair, waddling gait and brisk
was initiated, 60mg/day, more exams were requested, uolar myopathy with autophagic vacuoles. A hypothesis facial reflexes. Coordination and sensibility were normal.
and the discrepancy was on CPK levels (1.166,4) and ANF of toxic myopathy due to chronic chloroquine use was MMSE: 10 points (11 years/schooling), poor reading and
(anti-nuclear factor) titulation 1/160. The day after the made. Medication was discontinued and, although in figure interpretation, hesitant and reduced fluency, ano-
patient was dismissed without any neurological deficits intensive care, the patient evolved with multiple clinical mia, digital agnosia, acalculia, apathy, disinhibition and
and complaints, the final diagnosis was bilateral gastroc- complications and death. Discussion: Chloroquine is a executive changes. Laboratory data showed normal CPK,
nemius myositis. Discussion: Our case was probably an drug used worldwide for treatment of malaria, and as aldolase and cardiological screening. EMG showed prox-
IAM (Influenza-Associated Myositis), that disease was an immunomodulator to treat a variety of inflammatory imal myopathic pattern in all limbs; brain MRI demon-
defined as follows: Virologically proven influenza or disorders. It has a high affinity to some cellular compart- strated brain atrophy, mostly in left temporal lobe. In
influenza-like illness plus clinical evidence for localized ments, resulting in abnormalities of lysosomal function muscle biopsy, there was marked variability in fibers size,
myalgia plus elevation of serum creatine phosphokinase and autophagy, as chloroquine remains sequestered increased connective endomysial and perimysial tissue,
(CPK) or abnormal muscle biopsy. Acute myositis has into those organelles after chemical modifications due endomysial inflammatory reaction, necrotic fibers and
been reported as an pediatric exclusive disease, normally to the acid pH. The cumulative effect can be deleterious rimmed vacuoles. Immunohistochemistry was positive
with symptoms like painful oedema of leg muscles in the to skeletal muscle, which can have a spectrum of clinical for CD4 and CD8. The Next-Generation Sequencing panel
days following a viral illness. Calf muscles were the only presentations. There is a predilection for axial, proximal showed a pathogenic heterozygous mutation in exon 5 of
muscles affected in the cases reported in the past. The limb, bulbar, respiratory, and cardiac muscles. The mor- VCP gene [c.463C>A; p.(Arg155Ser)], also present in her
incidence is higher in boys, muscle symptoms usually bidity is high, even after drug discontinuation, although sister. Thus, it was consistent with inclusion body mio-
begin 3 days following influenza symptom onset, with some patients may improve. In our case, there was a rapid sitis (IBM) with frontotemporal dementia. Discussion:
pain in one or both calves and difficulty walking being evolution to multiple organ dysfunction with respiratory The mutation on VCP gene (valosin containing protein)
the most common complaints. Among cases of IAM, in failure secondary to her severe clinical condition. Final includes a group of hereditary diseases that can cause
? of cases, the calf muscles were affected alone, and ? of Comments: The skeletal muscle is a highly vascularized myopathy, frontotemporal dementia and bone disease,
cases occurred in multiple muscular groups in addition tissue and, thus, susceptible to the toxic effects of vari- as well as others neurodegenerative diseases. It shares
to the calf muscles. Treatment is symptomatic, as myositis ous substances, including a variety of therapeutic drugs. the same pathophysiology of protein aggregates with
is self-limited, usually resolving within a mean of 3 days. Although rare, chloroquine myopathy can result in marked ubiquinin-proteossome system disfunction and autoph-
Final Comments: Our case of IAM behaved differently morbidity and potential mortality if not accurately and agy. This hereditary type of IBM can be associated with
from previous published reports, specially because of the promptly diagnosed. The nonspecific clinical manifesta- the presence of inflammatory infiltrate in muscle biopsy,
age of the patient reported. So, it is a study that expands tions, specially when occurring in the elderly population, being the presence of rimmed vacuoles of great diagnos-
the definition for the general population, being not an may contribute to a misleading diagnosis. In our patient, tic contribution. Final Comments: The recognition of
exclusive pediatric disease and should be on the list of the muscle biopsy was a crucial tool to characterize the the particularities of this multisystemic proteinopathy,
differential diagnosis on muscular disorders. skeletal muscle involvement and attribute chloroquine a group of autosomal dominant diseases with variable
toxicity as a preponderant factor in her muscle disease. penetrance and phenotypes, allow improving the treat-
ment and follow-up of patients and promoting genetic
Doenças Neuromusculares guidance to their relatives.
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62
XXX Congresso Brasileiro de Neurologia

TL 1105820 TL 1106066 TL 1106092

PARANEOPLASTIC MYOPATHY WITH INCLUSION BODY MYOSITIS ASSOCIATED MOTOR FUNCTIONAL FOLLOW-UP OF TWO
PHENOTYPIC MANIFESTATION OF WITH SJOGREN’S SYNDROME: A NEW SPINAL MUSCULAR ATROPHY PATIENTS
OCULOPHARYNGEAL MUSCULAR MYOPATHY DESCRIPTION? TREATED WITH GENE THERAPY
DYSTROPHY (OPMD): A CASE PRESENTATION
Isadora Santos Ferreira, Natália Merten Athayde, Graziela Jorge Polido, Rodrigo de Holanda
Pedro Rodrigues Neves, Antonella Brun de Carvalho, Ana Marina Dutra, Roseli Corazzini, Alzira Alves de Mendonça, Edmar Zanoteli
Taís Michele Werle, Vitoria Pimentel, Marco Antonio Siqueira Carvalho
Department of Neurology, Faculty of Medicine,
Eduardo Koff, Erica Rizon, Jefferson Becker
Hospital Israelita Albert Einstein, São Paulo SP, University of São Paulo, São Paulo SP, Brazil
Escola de Medicina da Pontifícia Universidade Brazil
Centro Universitário FMABC, São Paulo SP, Brazil grazielapolido@gmail.com
Católica do Rio Grande do Sul. Porto Alegre RS,
Brazil
isadora.sferreira@gmail.com Case Presentation: The first case is a male, with SMA
Serviço de Neurologia do Hospital São Lucas – Porto
type II receiving GT at age of 7 years and 11 months olg,
Alegre – RS – Brazil
Case Presentation: A 47-year-old man presented with and with 8 years and 3 months old at time of last evalua-
pedro.neves@edu.pucrs.br polyarthralgia associated with knee and ankle joints tion. The patient had already been using nusinersen for
swelling 4 years prior to evaluation. A year later, he noticed 5 years. Before treatments, the patient was evolving with
Case Presentation: A 53-year-old male, dyslipidemic, progressive weakness of upper and lower limbs, with loss of motor functions such as rolling. At the beginning of
former smoker and drinker patient presented a walking difficulty raising the arms and climbing stairs. He also nusinersen treatment he scored 14 points on the HFMSE
difficulty and left ptosis, which progressed bilaterally, referred trouble in writing, reaching out for things and scale, and after 5 years of treatment (before receiving
dysphonia, dysphagia for liquids and fever. Physical picking up objects. Neurological examination revealed GT) he scored 24 points. The child continued to roll over
examination revealed paralysis of the lateral rectus and myopathic gait, atrophy of the deltoids and pectorals, and became able to move from a sitting to a lying posi-
medial rectus muscles; paresis of the oblique muscles; facial weakness, proximal and distal muscle weakness tion with the use of nusinersen. After 3 months of GT he
grade 3 strength for cervical flexion and grade 4 for lat- in the upper and lower limbs. Deep tendon reflexes were increased the score to 26 points and started to assume
eralization; dysmetria in the lower limbs; hyporeflexia; decreased in the upper limbs and normal in the lower the crawling posture and became more agile in the motor
absent cutaneous-plantar reflex on the right side and in limbs. Cranial nerve, sensitivity, cerebellum and cogni- functions that he had already acquired. He had gains in
flexion on the left side; grade 2 gynecomastia. ENMG tion tests were normal. The creatine kinase (CK) was 3,077 basic activities of daily living such as dressing. The sec-
showed neurophysiological findings of myopathy. Thigh U/L and serum aldolase level was 32 (normal, < 7,6 U/L). ond case is one of the first brazilian children, a female
MRI revealed mild liposuction of the quadriceps femoris Erythrocyte sedimentation rate was elevated. Anti-cN1A with SMA type I, to receive GT at 1 year and 11 months
and gluteus maximus muscles. Skull MRI showed marked and Anti-SSA/Ro antibodies were positive. Complement old, and is currently 3 years and 8 months old. With the
atrophy of the masticatory muscles. Lumbar puncture levels and serum levels of thyroid hormones were within previous use of nusinersen, she acquired head control,
showed a CSF without alteration. The rheumatology team normal ranges. Negative autoantibodies included: anti- ability to sit without support and to roll over. Regarding
evaluated him and ruled out the hypothesis of inflamma- nuclear antibodies, anti-SSB/La, anti-Sm, anti-DNAh, motor assesments in scales, the patient scored 08 to
tory origin because the clinical (no response to cortico- anti-SRP, anti-RNP, anti-SCL-70, anti-RNA polymerase III. 16 points on HFMSE after 3 months of GT, and scored
steroids) and imaging exams (no inflammatory signs on Lower limb MRI showed muscle edema with fatty partial 25 points after 20 months of treatment. Currently, she
thigh MRI) were not compatible. After weeks, the patient replacement and atrophy. Electromyography demon- walks with hip support and stands with support for a few
started to manifest lymphadenomegaly: bilateral cervical strated a pattern of proximal and distal weakness in both minutes. Discussion: Children with 5q-Spinal Muscular
and left supraclavicular. According to the neck CT, the upper and down extremities associated with spontaneous Atrophy (SMA) have predominantly axial muscle weak-
cervical one was suggestive of neoplasia, and according activity. Repetitive stimulation test was normal. Muscle ness, which directly impacts the acquisition of motor
to the pathological examination, the supraclavicular one biopsy was consistent with Inclusion Body Myositis (IBM). milestones. To demonstrate motor improvment of two
was a metastasis of an epidermoid carcinoma. Based An association between IBM and Sjogren’s Syndrome children with SMA, one was among the first patients to
on the picture evolution, the main diagnostic hypoth- (SS) was considered and the patient was started on oral receive a single dose of gene therapy (GT) in Brazil and
esis was an occult primary site epidermoid carcinoma prednisone (1mg/kg daily). After two weeks with corti- another was one of the oldest in the world to receive the
and paraneoplastic myopathy syndrome. Discussion: costeroid, he showed partial clinical improvement thus therapy. Assessment of motor functions using the CHOP-
Paraneoplastic syndromes are rare disorders triggered we indicated methylprednisolone pulse therapy (1g IV/ INTEND (Children’s Hospital of Philadelphia Infant Test
by an altered immune system response to a non-meta- daily for 5 days). At 1-month follow-up, the patient showed of Neuromuscular Disorders) and HFMSE (Hammersmith
static neoplasm and most commonly occur in patients improvement in muscle strength, specially of proximal Functional Motor Scale Expanded for SMA) motor scales
who are not known to have cancer. The symptoms of upper extremities. Discussion: Inclusion Body Myositis before GT and after 3 and 20 months of treatment, respec-
neuromuscular paraneoplastic syndromes may mimic and its histopathological features were first described tively. Final Comments: patients treated with GT showed
common neurological conditions, such as Myasthenia in 1967. An association between IBM and SS was pro- improvement in motor function even if they were treated
gravis syndrome. But, the patient of this case showed an posed fifteen years later, in 1982. The pathogenesis of this previously with nusinersen.
oculopharyngeal muscular dystrophy (OPMD), which is association is unclear. Recent studies suggest for disease
a myopathy characterized by slowly progressive ptosis, concurrence a shared association with T-cell granular
dysphagia, and proximal limb weakness with myopathic lymphocyte leukemia. Even though sporadic IBM is Doenças Neuromusculares
involvement of other muscles, particularly of the limb considered treatment-refractory, studies have reported
girdles, starting in the legs. Final Comments: OPMD is response to immunotherapy when this combination is
a very rare phenotypic manifestation of paraneoplastic present, likewise our case. Final Comments: Faced with
syndromes, even in cases of neuromuscular paraneoplastic the diagnosis of IBM we suggest that SS should be investi-
syndrome with myopathy. Once the patient had a family gated, as patients with this association may benefit from
history of similar condition and lung cancer, he was kept immunosuppressive therapy.
under investigation for some weeks until the diagnosis
of neoplasia. After that, treatment with Carboplatin and
Paclitaxel was instituted with good response. Doenças Neuromusculares

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63
XXX Congresso Brasileiro de Neurologia

TL 1106093 TL 1106122 TL 1106172

MOTOR IMPROVEMENTS OF AN ADULT SMA LMOD3-RELATED NEMALINE MYOPATHY HEART FAILURE IN LMNA-RELATED EMERY
TYPE 3 PATIENT TREATED WITH RISDIPLAM (NEM10): NEW INSIGHTS BASED IN TWO DREIFUSS MUSCULAR DYSTROPHY
BRAZILIAN CASES
Graziela Jorge Polido, Rodrigo de Holanda Cláudia Suemi Kamoi Kay, Alessandra Filpo, Gel
Mendonça, Eduardo Vital de Carvalho, Edmar Thaissa Gianolla Arnaut Correia, Roberta Caramico Roberto Marmitt Berardi, Augusto Arrebola Presoto,
Zanoteli Pinto, Natália Merten Athayde, Ana Marina Dutra, Otto Jesus Hernandez Fustes, Paula Raquel do Vale
Roseli Corazzini, Paulo Breinis, Alzira Alves de Pascoal Rodrigues, Renata Dal-Prá Ducci, Lineu
Department of Neurology, Faculty of Medicine, César Werneck, Paulo José Lorenzoni, Rosana
Siqueira Carvalho
University of São Paulo, São Paulo SP, Brazil Hermínia Scola
Centro Universitário FMABC . Santo André SP, Brazil
grazielapolido@gmail.com Universidade Federal do Paraná. Curitiba PR, Brazil
thaissa.correia@aluno.fmabc.net
Background: Spinal Muscular Atrophy (SMA) type 3 in alessandrafilpo@gmail.com
adulthood is still a challenge in terms of treatment response Case Presentation: A – Male, 5 months old, polyhydram-
due to disease duration, previous spine fusion, and some nios and reduced fetal movement, born with generalized Case Presentation: A 26-year-old female patient was
cases may benefit from oral drug treatment. Objective: To hypotonia, cyanosis, respiratory distress, and irregular admitted to the Cardiovascular Intensive Care Unit due
describe motor improvements of a patient with SMA type 3 breathing. Submitted to orotracheal intubation, trache- to acute shortness of breath and hypoxia at rest, reporting
treated with Risdiplam for 6 months. Methods: Evaluation ostomy and gastrostomy. Inspection: elongated and hypo- exertional dyspnea over the past years. She was accom-
of motor function using Hammersmith Functional Motor tonic face, bilateral palpebral ptosis, adducted thumb and panied at the Neuromuscular Disorders outpatient clinic
Scale expanded for SMA (HFMSE) before started treatment fifth finger, flaccid and areflex tetraparesis. Received pyr- due to a past medical history of worsening muscle weak-
and after 6 months of drug use. Results and Discussion: idostigmine (20mg/kg), evolving with increased mobility ness and contractures beginning at the age of 3. These
The patient is a 38 year old female with SMA type 3 (homo- in the shoulder girdle, trunk, and extremities of the upper complaints were associated progressively with inability
zygous deletion in SMN1 gene and with 3 SMN2 copies), limbs. No side effects were observed. B – male, 19 years to walk without support and dysphagia. Her birth and
who lost her gait at 11 years of age and was submmited to old, son of consanguineous parents, hypotonia since birth, family history, as well as her developmental milestones,
surgery for scoliosis when she was 13. Before treatment delay in motor milestones, mild dysarthria, elongated and were unremarkable. Examination revealed weakness
she was able to sit unsupported, transfer independently, hypotonic face, bilateral palpebral ptosis, generalized amy- affecting the proximal arms and distal legs with sparing
and is able to roll over. She has shortened hip extension, otrophy and muscle weakness with distal predominance, of facial muscles, associated with scoliosis and contrac-
knee extension and dorsal flexion and she never used adducted thumb, and steppage gait. Muscle Biopsy showed tures involving elbow and heel. Cardiorespiratory exam
orthotics. She started using Risdiplam since November nemaline bodies. EMG in both cases revealed a myopathic showed bilateral jugular vein distension and an irregular
27, 2021 and scored 26 pontis in HFMSE. After 6 months pattern. Repetitive stimulation identified a decrement of pulse. A through diagnostic work-up was conducted and
treatment the patient gained 7 points in the same scale. 18.8% in case B. Received pyridostigmine with a subjec- a diagnosis of acute decompensated heart failure (HF)
The gains were as follows: item 3, is now able to take both tive improvement. Molecular analysis identified in both with reduced ejection fraction was made, associated with
upper limbs above the ear line; items 6, 7, 8 and 9, which cases 2 different variants (homozygosis) in LMOD3 gene. atrial fibrillation and bilateral pleural effusion. Laboratory
assess rolling, and now she is able to roll without the help Discussion: Nemaline myopathy (NM) is a congenital assessment disclosed a serum creatine kinase level of 478
of the upper limbs; item 11, she is able to remain in the myopathy defined by the presence of rod-like structures, IU/L. Previous investigation was remarkable for a muscle
prone position, with support on the forearms and cervi- called nemaline bodies, in myofibers, caused by 13 genes. biopsy with myopathic features and immunohistochemis-
cal extension; item 12, is now able to, in prone, perform The LMOD3 is associated with a severe birth-onset form of try revealing deficiency of every tested protein (dystrofin,
cervical extension with upper limbs in an intermediate NM (NEM10). We describe the first two brazilian cases of sarcoglycan, dysferlin, destroglycan and emerin). A genetic
position (elbows at shoulder height). After using started NEM10. Out of 34 cases reported in the literature, 5 reached analysis was performed, disclosing one pathogenic variant
treatment, patient alds presented an improvement in adulthood. Our Case A has a severe form with a non-sense in LMNA gene [c.83G>A (p.Arg28Gln)] shortly after the
fatigue, more agility in everyday life, such as for transfers, variant (c.154del), previously described in a Portuguese patient’s death due to refractory respiratory insufficiency,
and is now able to pack her body in the car without help. family. Case 2 has a mild form, with a missense variant confirming a diagnosis of autosomal dominant Emery-
Final Comments: In this specific case, even though the (c.1194G>C) not previously described. There seems to be Dreifuss muscular dystrophy (EDMD). Discussion: EDMD
patient was an adult and had a previous spine surgery, a genotype-phenotype correlation in NEM10, based on commonly results from a defect of one or more proteins
treatment with Risdiplam was effective in improving motor the type of consequence of the variant and the position of comprising the cell nuclear envelope. The classic clinical
function, such as transfers and fatigue. the amino acid in the protein, determining a more severe triad consists of early contractures, progressive muscle
(case A) or milder (case B) phenotype. The presence of weakness and cardiac abnormalities. The two most com-
decrement is a classic finding of neuromuscular junction mon associated genes are EMD and LMNA. Mutations
Doenças Neuromusculares disease (NMJD), however, it has also been described in in LMNA most typically display an autosomal dominant
subtypes of congenital myopathy. So, these patients may inheritance pattern and de novo mutations are common.
have a good response to acetylcholinesterase inhibitors. Mostly, these patients have a more severe disease course,
Final Comments: Our cases are the first NEM10 cases in and the incidence of cardiac involvement increases in an
the literature with a partial response to pyridostigmine. age-dependent manner. LMNA-related cardiomyopathy
The use of pyridostigmine in NM was reported in one also carries a substantial risk of end-stage HF develop-
case related to KLHL40, with significant improvement. ment, as seen in our patient. Final Comments: Despite
Therefore, we should consider using pyridostigmine in its rareness, early diagnosis of LMNA-related muscular
NM as an alternative therapy. dystrophy is important for the detection of potentially fatal
cardiac abnormalities. Thus, routine cardiac surveillance
may prevent mortality in these young patients.
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64
XXX Congresso Brasileiro de Neurologia

TL 1106198 TL 1106206 TL 1106218

IMMUNE-MEDIATED ANTI-SRP STIFF PERSON SYNDROME, PARTIAL GUILLAIN-BARRÉ SYNDROME FOLLOWING


NECROTIZING MYOPATHY – A CASE VARIANT, IN A 12-YEAR-OLD PATIENT –A ACUTE DENGUE INFECTION: A CASE
PRESENTATION CASE PRESENTATION PRESENTATION
Amabile Rodrigues Alves, Mariana Matos Mayer, José Elias Makaron Neto, Gabriel Pereira Braga, Sarah Diógenes Alencar, Sara Arcanjo Bringel,
Gabriela da Silva Cremonese, Ivanio Alves Pereira Larissa Emile Paulo, João Marcelo Borba Leite, Ana Silvia Sobreira Lima Verde, Alessandra Braga
Heloisa Ferraz Troijo, Nathalia Raquel Cristaldo Cruz Guedes de Morais, Matheus Costa Bessa,
Universidade do Sul de Santa Catarina – Palhoça – Alvarez, Omar Gurrola Arambula, Karen Isabelle Karla Rafaele Silva Vasconcelos, João Gabriel
SC – Brazil Pontes Duran Bottaro Dias Brasiliense Frota, Karoline Ferreira Mororó
gabicremonese@hotmail.com Menezes, Fernanda Martins Maia Carvalho,
Santa Casa de Campo Grande. Campo Grande MS, Norberto Anízio Ferreira Frota
Brazil
Case Presentation: A 51 years old female started 6 months Hospital Geral de Fortaleza. Fortaleza CE, Brazil
ago with muscle weakness in the upper and lower limbs netinhoneto94@gmail.com
and difficulty to raise the arms. She had dysphagia, alo- sarah_diogenes@hotmail.com
pecia, paresthesias in hands, and lost weight in the last 3 Case Presentation: A.S.S, 12 years old healthy male,
months. Physical examination showed proximal muscle reports burning back pain and lack of control of the lower Case Presentation: In February 2022, a 20 year old woman
strength grade of 3 in upper and lower limbs. The labo- limbs, unable to move them. Evolved with sustained mus- was admitted with a 19-day history of ascending tetra-
ratory tests revealed abnormal high levels of CK (4.309), cle contraction of the left hemiabdome and left lower paresis and paresthesia. 30 days after admission, she
LDH (948) and aldolase (13). ANA test, anti-JO1, anti- limb. He denies urinary or gastrointestinal alterations or presented with cough, nasal congestion, odinophage,
SSA, anti-SSB, anti-SM and anti-RNP were negative. concomitant and previous infectious symptoms. He also and tested negative for Covid-19. Upon Hospital admis-
The autoantibody recognizing the SRP was positive. The denies recent vaccinations. On physical examination, sion, the patient was unable to walk without help. She
electromyography showed myopathic pattern and the spastic paresis of the left abdomen and left lower limb, was conscious, well oriented and had normal vital signs.
MRI of the thighs presented signs of myopathy with pronator deviation of the left foot, and a left hemiparetic Neurological examination revealed global hypotonia,
mild edema of the semitendinosus, hamstring, biceps espastic gait. Magnetic resonance imaging of all central arreflexy and severe reduction of strength in the bilateral
femoris, tensor muscles of the fascia lata, diffuse muscle nervous system was not remarkable except for the dubi- lower limbs. In upper limbs, she had hyporelexia, moder-
hypotrophy. Mild peripheral uptake by contrast medium ous enhancement of some lumbar roots inside the dural ate lower limb weakness bilaterally, and global involve-
and mild edema. The muscle biopsy revealed necrotic sac, which may be related to radiculitis. Non-reactive ment of deep sensitivity, her neck muscle strength was
fibers, sometimes surrounded by myophagocytes. A antiGAD, non-reactive serology, unavailable anti-MOG reduced and she had right peripheral facial palsy. Initial
significant variation in the size of muscle fibers was also and aldolase 13,3. Eletroneuromiography (ENMG) showed laboratory exams were unremarkable. Cerebrospinal fluid
seen, with several regenerated fibers and capillaries with persistent and continuous muscle action potentials in the study (CSF) showed protein-cytological dissociation and
mural thickening. Necrotic fibers and a few lymphocytes rectus abdominis muscles, and the muscles of the left Electroneuromyography (ENMG) revealed a demyelin-
dispersed throughout the endomysium, in addition to lower limb without relaxation. The patient was treated ating polyradiculoneuropathy pattern. The patient was
the scarce sarcolemmal expression of MHC-I. She was with muscle relaxants, benzodiazepines, and physical treated with 5 cycles of plasmapheresis. By the end of
treated initially with prednisone 60mg/day, but after a therapy. Discussion: Stiff person syndrome (SPS) is a rare the cycles she was able to walk without help. This patient
bad response to the glucocorticoid she received immu- entity characterized by painful muscle stiffness associated was diagnosed with Guillain Barre Syndrome (GBS) in its
noglobulin human (IGIV) monthly during one year and with muscle spasms1. It is a rare neuromuscular disorder acute inflammatory demyelinating polyneuropathy form
rituximab each 6 months. Discussion: This report aims that affects about one to two cases per million, primarily (AIDP). Additionally, she was tested for dengue-specific
to expose a rare case of inflamatory myopathy, character- affecting adults aged 20 to 50 years, with women being IgM in the CSF, providing the association of GBS AIDP
ized by typical laboratory and clinical features including affected about two to three times more than men. It is with dengue fever (DF). Symptoms of GBS may begin
rapidly progressive muscle weakness, elevated muscle infrequent to occur during childhood1,2. The association a few weeks after a febrile infection. Though there are
enzymes and characteristic histopathology of muscle of autoantibodies against glutamic acid decarboxylase well established preceding infective agents, arbovirus
biopsies. The immune-mediated necrotizing myopathy (anti-GAD) is found in about 60 to 80% of cases. It can as aetiological factors for GBS are uncommon. Studies
(IMNM) represents 20 to 30% of cases of inflammatory be subdivided into three clinical forms: Classic, Partial show that the annual incidence of GBS after arbovirus
myopathy and is distinguished from other subtypes by and Paraneoplastic Variant.Comments: The exposed infection is 0.24 cases per 100 thousand inhabitants.
the existence of necrosis of muscle fibers and absence case shows us a rare syndrome in a patient with atypical Arbovirus infections are frequent, but they rarely involve
of inflammatory infiltrate. Final Comments: IMNM, a epidemiology and an even more unusual presentation. the nervous system. In this patient case, the presence of
type of autoimmune myopathy that has three different Furthermore, the lack of association with autoantibodies a positive dengue-specific IgM in CSF, following neuro-
serologically subtypes: Anti-SRP, anti-HMGCR and anti- makes this case even more unusual, making the diagnosis logical manifestations, patterns of ENMG and the typical
body-negative IMNM myopathy. In histology these dis- possible only with ENMG. CSF findings, supported the association of GBS and DF.
eases have similar characteristics: myofiber necrosis in Although GBS associated with DF is a rare condition, it’s a
muscle biopsy, but the different subtypes have different serious neurological complication that remains underdiag-
risk factors, cancer risks, extramuscular manifestations Doenças Neuromusculares nosed. Strategies for notification and investigation should
and prognosis. These differences indicate that those are be implemented. Our findings highlight the importance
three different diseases, such as distinct pathological of further studies to better understand this condition and
mechanisms and may respond to a variety of therapeu- raise awareness amongst clinicians.
tics modalities. More data on disease pathogenesis and
optimal treatment of each subtype of IMNM are needed.
Doenças Neuromusculares

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65
XXX Congresso Brasileiro de Neurologia

TL 1106227 TL 1106257 TL 1106257

CERVICAL SPONDYLOTIC AMYOTROPHY: A ADENOSINE DEAMINASE 2 ADENOSINE DEAMINASE 2


RARE CAUSE OF FOCAL PERIPHERAL NERVE DEFICIENCY SYNDROME (DADA2): DEFICIENCY SYNDROME (DADA2):
HYPEREXCITABILITY SYNDROME A CASE PRESENTATION OF A RARE A CASE PRESENTATION OF A RARE
AUTOINFLAMMATORY NEUROLOGICAL AUTOINFLAMMATORY NEUROLOGICAL
Lucas Assis Santos
DISEASE DISEASE
Universidade Estadual de Campinas. Campinas, SP,
Brazil Gabriela da Silva Cremonese, Nathalia Tomazoni Gabriela da Silva Cremonese, Nathalia Tomazoni
Silva, Maria Luiza Benevides, Ivanio Alves Pereira Silva, Maria Luiza Benevides, Ivanio Alves Pereira
lucasdsouza9413@gmail.com
Universidade do Sul de Santa Catarina – Palhoça – Universidade do Sul de Santa Catarina – Palhoça –
Case Presentation: A 47 year-old man presented to the SC – Brazil SC – Brazil
emergency department reporting involuntary movements Universidade Estadual de Campinas, São Paulo SP, Universidade Estadual de Campinas, São Paulo SP,
and weakness in the right upper limb in the previous 28 Brazil Brazil
days. These symptoms were associated with paresthesia gabicremonese@hotmail.com gabicremonese@hotmail.com
and neuropathic pain. Neurological examination showed
myokymia, fasciculation and distal weakness in the right Case Presentation: 35-year-old woman, born in south- Case Presentation: 35-year-old woman, born in south-
upper limb, a claw hand, atrophy of the hypothenar emi- ern Brazil, presented with livedo reticularis, reactive C ern Brazil, presented with livedo reticularis, reactive C
nence, and asymmetrical response of the right finger flex- protein (RCP) constantly elevated (30-50mg/dL), and protein (RCP) constantly elevated (30-50mg/dL), and
ion reflex. A magnetic resonance imaging of the cervical mononeuritis multiplex. In addition, she had recurrent mononeuritis multiplex. In addition, she had recurrent
spine showed a C5-C6 spondylotic compression of the spi- unexplained fever, and anemia since childhood. At 22 unexplained fever, and anemia since childhood. At 22
nal cord with a “snake eye sign”. Electroneuromyography years old, she was Hospital admitted due to a third cra- years old, she was Hospital admitted due to a third cra-
showed an axonal compromise of the preganglionic por- nial nerve impairment, probably caused by an ischemic nial nerve impairment, probably caused by an ischemic
tion of the C7-C8 myotomes. Myokymia, fasciculation and event. She received anticoagulants for a couple of months, event. She received anticoagulants for a couple of months,
pain had a significant improvement with carbamazepine. which were discontinued by her neurologist due to a nor- which were discontinued by her neurologist due to a nor-
Discussion: Cervical spondylotic amyotrophy is charac- mal cranial MRI. In her family history, she had a 5-year- mal cranial MRI. In her family history, she had a 5-year-
terized by predominantly motor symptoms related to com- old sister, who also had livedo reticularis, and died due old sister, who also had livedo reticularis, and died due
pression of the ventral neural root or injury to the anterior to cerebral aneurysms, and alveolar hemorrhage. The to cerebral aneurysms, and alveolar hemorrhage. The
horn due to cervical spondylosis. The intrinsic injury to the complementary investigation showed low levels of IgM, complementary investigation showed low levels of IgM,
anterior horn can be related to insufficient blood supply, and negative tests for ANA, rheumatoid factor, ANCA, and negative tests for ANA, rheumatoid factor, ANCA,
which was demonstrated by high intensity signal in the cryoglobulin, and antiphospholipid antibody. The sero- cryoglobulin, and antiphospholipid antibody. The sero-
spinal cord, the so-called snake-eye sign. Peripheral nerve logical tests for infectious diseases were also negative logical tests for infectious diseases were also negative
hyperexcitability syndrome is characterized by cramps, (VDRL, HIV, hepatitis B, and C testing). The thoracic and (VDRL, HIV, hepatitis B, and C testing). The thoracic and
fasciculations, neuromyotonia and myokymia. PNHS is abdominal magnetic resonance angiography discarded abdominal magnetic resonance angiography discarded
usually associated with autoimmune disorders such as vessel stenosis or aneurysms. Clinical and complemen- vessel stenosis or aneurysms. Clinical and complemen-
in the presence of anti-CASPR2 autoantibodies, but can tary exam data were compatible with medium vessel tary exam data were compatible with medium vessel
also occur in channelopathies, amyotrophic lateral scle- vasculitis, suggesting a polyarteritis nodosa phenotype. vasculitis, suggesting a polyarteritis nodosa phenotype.
rosis, and, rarely, cervical spondylotic amyotrophy. Final She was treated with corticosteroids, methotrexate, and She was treated with corticosteroids, methotrexate, and
Comments: Focal PNHS should include cervical spondy- tocilizumab. Even under treatment, she presented with tocilizumab. Even under treatment, she presented with
lotic amyotrophy in its differential diagnosis. an acute ischemic stroke located in the mesencephalon. an acute ischemic stroke located in the mesencephalon.
Her clinical syndrome, associated with a positive familiar Her clinical syndrome, associated with a positive familiar
Doenças Neuromusculares history, makes the diagnosis of DADA2 plausible, and the history, makes the diagnosis of DADA2 plausible, and the
next step would be the molecular test. Considering this next step would be the molecular test. Considering this
hypothesis, anti-TNF drug adalimumab was prescribed, hypothesis, anti-TNF drug adalimumab was prescribed,
and oral anticoagulant warfarin was immediately stopped. and oral anticoagulant warfarin was immediately stopped.
Discussion: Adenosine deaminase-2 deficiency (DADA-2) Discussion: Adenosine deaminase-2 deficiency (DADA-2)
is described as an autoimmune disease caused by muta- is described as an autoimmune disease caused by muta-
tions in the CECR1 (ADA-2) gene. Vasculopathy involv- tions in the CECR1 (ADA-2) gene. Vasculopathy involv-
ing small and medium-sized vessels is one of the main ing small and medium-sized vessels is one of the main
clinical manifestations of DADA-2. It is one of the rare clinical manifestations of DADA-2. It is one of the rare
causes of stroke in young people, associated with varied causes of stroke in young people, associated with varied
systemic manifestations, such as polyarteritis nodosa systemic manifestations, such as polyarteritis nodosa
(PAN), livedo reticularis, ulcerations of the extremities, (PAN), livedo reticularis, ulcerations of the extremities,
arthritis, recurrent infections, and peripheral neuropathy. arthritis, recurrent infections, and peripheral neuropathy.
Immunosuppressive drugs can prevent further attacks Immunosuppressive drugs can prevent further attacks
and morbidity; on the other hand, anticoagulants and and morbidity; on the other hand, anticoagulants and
antiplatelets are not recommended. Final Comments: antiplatelets are not recommended. Final Comments:
The reported case highlights the importance of consid- The reported case highlights the importance of consid-
ering DADA-2 as a differential diagnosis in patients with ering DADA-2 as a differential diagnosis in patients with
recurrent neurological deficits at a young age, associ- recurrent neurological deficits at a young age, associ-
ated with systemic manifestations, especially regarding ated with systemic manifestations, especially regarding
prompt treatment. prompt treatment.

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XXX Congresso Brasileiro de Neurologia

TL 1106274 TL 1106302 TL 1106347

LAMINOPATHY PRESENTING AUTOPHAGIC CONGENITAL FIBER TYPE DISPROPORTION A RARE CASE OF EARLY-ONSET
VACUOLAR HISTOPATHOLOGY DUE TO HOMOZYGOUS HACD1 MUTATION: A INCLUSION BODY MYOSITIS (IBM):
CASE PRESENTATION A CASE PRESENTATION
Bruna Moreira Souza Proença, André Macedo
Serafim Silva, Cristiane Araujo Martins Moreno, Jessica Blanc Leite Oliveira, Felipe Franco da Graça, Caroline Corrêa Santos, Rubson Soares Rocha,
Clara Gontijo Camelo, Beatriz Carneiro Gondim Alexandre Motta Mece, Rafaella do Rosário Tacla, Matheus Corrêa Santos, Luiza Silva Antunes
Silva, Lucas Marenga Arruda Buarque, Edmar Anamarli Nucci, Marcondes Cavalcante França Jr
Zanoteli Hospital Geral Roberto Santos. Salvador BA, Brazil
Hospital de Clínicas da Unicamp. Campinas SP,
Hospital das Clínicas da Faculdade de Medicina da Brazil line_correa12@hotmail.com
Universidade de São Paulo. São Paulo SP, Brazil
jessicablancleite@gmail.com Case Presentation: Female, 37 years old, from Salvador/
brunamoreirasp@hotmail.com Bahia, with crural paraparesis for 10 years. She perceived
Case Presentation: Congenital myopathies are a broad difficulty in climbing stairs and today she walks with bilat-
Case Presentation: A 17-year-old patient, with no con- group of pathologies whose symptoms include hypoto- eral support. In the last two years, she also presented upper
sanguinity and normal motor development in childhood nia, proximal weakness, distinct phenotypic features, limbs weakness, distal, more evident when trying to open
presented with difficulty in walking at the age 10, with gait and wide variation in severity. Its histological findings pots. Hypotrophy of the quadriceps and on the ventral
on tiptoe. On examination, he had distal weakness MRC are important and helped in its classification. Currently, surface of the forearms was identified, as well as reduced
grade 3 for anterior tibialis and finger extensors and MRC however, with the advent and easier access to genetic muscle strength in leg extension and foot dorsiflexion and
4 for gastrocnemius. Deep tendon reflexes were hypoac- testing by next generation sequencing (NGS), knowledge also weakness in the fingers. Myopathic gait pattern. A CPK
tive and Achilles abolished. Contractures were observed about the pathophysiological bases of these diseases has (Creatine Phosphokinase) of 164 U/L (normal < 145) was
in cervical, elbows and Achilles tendons, characterizing expanded greatly. We report the case of a 66-year-old male measured. Electroneuromyography of the lower limbs
Emery-Dreifuss phenotype. On investigation, we found patient whose symptoms started at birth with hypotonia with myopathic pattern. Muscle biopsy revealed rimmed
elevated CK levels (1901 U/L). EMG showed myopathic and, subsequently, gait delay (walked independently vacuoles, expression of the MHC-I complex in sarcoplas-
changes on distal muscles. Muscle biopsy revealed dystro- at 7 years of age). After childhood there was stability of mic membranes, immunophenotypic staining of mem-
phic myopathic histological pattern and abundant rimmed symptoms, with slight worsening after the fifth decade, brane attack complex in sarcoplasmic membranes and
vacuoles with autophagic characteristics. Additional evalu- when we started to follow him up. In the initial assess- capillaries, presence of CD8+ lymphocytes and presence
ation revealed no cardiopathy nor respiratory damage. The ment proximal weakness, mainly in the lower limbs, of P62 aggregates in focal areas of the endomysium and
whole-exome sequencing showed a likely pathogenic vari- winged scapula and myopathic gait were noticed. There sarcoplasm.The clinical suspicion was of Inclusion Body
ant in LMNA (c.946A>C;p.Lys316Gln) Discussion: Variants were frequent falls, and difficulty climbing stairs. There Myositis. The treatment started with prednisone 1mg/kg/
in heterozygosis in the LMNA can cause a wide spectrum were no bulbar symptoms, sensibility or coordination day for 90 days, with no clinical response. The choice of not
of clinical manifestations known as laminopathy. There impairment, myotonic phenomenon, joint hypermobil- introducing any other immunosuppressants was made.
may be distal muscle weakness, with phenotype similar ity, cicatrization impairment or skin lesions. The patient’s Discussion: Inclusion Body Myositis (IBM), an inflamma-
to Emery–Dreifuss muscular dystrophy, or due to periph- parents were consanguineous (first degree cousins), and tory myopathy, is a rare, slowly evolving disease common
eral nerve involvement, resembling Charcot-Marie-Tooth there was a history of premature death of a sister from an in males over 50 years of age 1,2,3. Clinical manifestations
phenotype. Other patients present a congenital muscular uninvestigated neuromuscular disease. Aldolase and cre- include weakness in the quadriceps femoris muscle and
dystrophy with severe axial involvement or, even, isolated atine phosphokinase levels were normal. Further investi- ankle dorsiflexors, resulting in difficulty in walking and
cardiomyopathy. Described myopathological findings gation with nerve conduction study and electromyography climbing stairs, as well as in the wrist and finger flexors
in skeletal muscle laminopathies typically manifest as showed a proximal myopathic pattern and muscle biopsy 3,4,7,8,9. CPK (Creatine Phosphokinase) can be normal
nonspecific myopathic changes or dystrophic features. sampling the left biceps brachii showed congenital fiber or increased. Anti-cN1A antibody dosage provides good
Altered autophagy accompanied by rimmed vacuoles type disproportion. No necrosis, interstitial fibrosis or specificity. On electroneuromyography, it is characteristic
is a common denominator of many muscle diseases. inflammatory infiltrate were found. Echocardiogram and the presence of fibrillation and positive acute waves and
When it is combined with protein aggregates the defini- electrocardiogram showed no abnormalities. The patient increased polyphasic motor unit potentials 6,7,8,9. On
tion is of myofibrillar myopathy (MFM). Classical MFM was subsequently tested with a broad genetic NGS panel muscle biopsy, the gold standard exam, it was verified
pathology in skeletal muscle laminopathies has been for neuromuscular diseases, and a homozygous mutation the presence of endomysial infiltrate of mononuclear
reported in a few patients, however isolated autophagic in the HACD1 gene (c.373_375+2del) was found (classi- cells: CD8+ T lymphocytes and macrophages. A small
vacuolar myopathy has not been described associated fied as probably pathogenic according to ACMG criteria). number of myofibrils presented marginated vacuoles,
to LMNA. It was speculated that the mutated lamin A/C Additionally, a heterozygous pathogenic mutation in the a very characteristic finding in IBM 3,6,8,9. Evidence
may disrupt nuclear lamina integrity, unanchor desmin COL6A2 gene was identified. As mentioned above, how- indicates that patients with IBM respond poorly to cor-
from the nuclear envelope and cause disorganization of ever, the patient did not have phenotypic characteristics ticosteroid therapy. Other immunosuppressants can be
myofibrils linked to desmin. This process could lead to compatible with mutations in collagen 6 gene.To date, tried in those patients who respond to corticosteroids
MFM pathology. Final Comments: Vacuolar myopathies few cases of myopathy caused by HACD1 mutations have 10,11,12,13,14. Final Comments: IBM remains a rare
remain diagnostically challenging. Our case highlights been described worldwide and, as far as we are aware, and challenging entity. This is the case presentation of
the occurrence of this pathologic finding in patients with this is the first case of HACD1 mutation related myopa- a patient who, despite being an unusual age group, had
LMNA-related myopathy. We suggest including patho- thy reported in Brazil. By reporting this case, we expect to clinical and pathological findings suggestive of IBM and
genic variants in LMNA as a causative gene in the groups contribute to expanding the knowledge of this congenital unresponsiveness to corticosteroid therapy.
of vacuolar and myofibrillar myopathies. Identification of myopathy and bring it to attention as a possible cause of
this condition means a high risk of ventricular arrhyth- congenital myopathy in our country.
mias in these patients and its importance is to prevent Doenças Neuromusculares
lethal cardiac events.
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XXX Congresso Brasileiro de Neurologia

TL 1106362 TL 1106363 TL 1106377

BRUNS-GARLAND SYNDROME: PREVIOUSLY UNPUBLISHED HOMOZYGOUS NEW TREATMENTS, COMBINATION THERAPY


CASE PRESENTATION CHRNA1 C.997C>T VARIANT ASSOCIATED AND VENTILATORY CHANGES: REPORT
WITH RELATIVELY MILD CONGENITAL OF TWO CASES OF SPINAL MUSCULAR
Daiane Magalhaes
MYASTHENIC SYNDROME (CMS): A CASE- ATROPHY UNDER TREATMENT
Hospital Risoleta Tolentino Neves. Belo Horizonte REPORT
MG, Brazil Eduardo Vital de Carvalho, Rodrigo Holanda
Vítor Guimarães Corrêa, Fabrício Castro de Borba, Mendonça, Graziela Jorge Polido, Edmar Zanoteli
daiane.magalhaes@ymail.com Karen Fernanda Alves, Adilson Luciano Caleffi,
Neurology Department, Faculdade de Medicina,
Thiago de Souza Rosa, Paulo Schneider Wolmer,
Case Presentation: Female patient, 72 years old, hyper- Universidade de São Paulo, São Paulo SP, Brazil
Anamarli Nucci, Marcondes Cavalcante França
tensive, dyslipidemic, obese, diabetic, seeks care for pain Junior duvital1983@gmail.com
that started in the left ankle, 20 days of evolution, with
burning, shock and paresthesia, with ascending progres- Universidade Estadual de Campinas. Campinas SP,
Background: Spinal muscular atrophy (SMA) is charac-
sion and that reached the lumbar spine on the fifth day of Brazil
terized by progressive muscle weakness and respiratory
evolution. She developed paresis in the entire left lower vitorg@unicamp.br involvement. Recently, disease-modifying drugs in associ-
limb, initially with leg extension, but culminating in an ation with multidisciplinary management have increased
inability to flex, abduct, and adduct the thigh, grade 2 leg survival. A small group of patients have received therapies
Case Presentation: We present a 51-year-old male patient,
strength, and grade 1 thigh strength. Bilateral hyporeflexia. in combination, such as the use of gene therapy (GT) fol-
referred to the neuromuscular unit with history of mild
Initial suspicion of spondylosis neuropathy neurosur- lowed by nusinersen. However, the effects of this approach
transient neonatal bulbar weakness, presenting with
gery. Lumbosacral spine CT showing circumferential in terms of efficacy and safety are not well established.
worsening limb and bulbar weakness after 30 years of
disc bulges between L3-L4 and L4-L5, with epidural fat Objective: The aim of this study is to present the ventilatory
age. Family and past medical history were unremark-
effacement and determining impressions on the ventral changes in two patients with SMA type 1. The respiratory
able except for consanguineous parents. Previous NCS
surface of the thecal sac, with narrowing of the conjuga- functional measures collected were forced vital capacity
and needle EMG revealed some evidence of post-syn-
tion foramina in L4-L5 and possible involvement of the (FVC) and thoracic development was measured by chest
aptic neuromuscular junction dysfunction with diffuse
L4 roots. Cervicothoracic myelotomography without cirtometry (CC). Case 1 – Male patient, SMA type 1, who
myopathic motor unit potentials. Anti-AChR and anti-
obstruction to contrast passage. Absence of spinal cord started nusinersen at 5 months and received GT at 1 year
MuSK results were negative. The patient was initially
or radicular conflicts on MRI of the lumbar and thoracic and 7 months old. He continued using oligonucleotide
diagnosed with seronegative auto-immune myasthenia
spine. She evolved to partial improvement and, on the antisense after GT. The patient underwent 18 assessments
gravis and received various immunosuppressant therapies
seventh day of Hospitalization, she had grade 3 thigh at 30-day intervals. In the first evaluation the child was
and pyridostigmine with partial response. Nevertheless,
strength and neuropathic pain relieved with gabapentin 1 year and 7 months old, he presented FVC of 300 ml
from 30-50 years of age, the patient underwent multiple
and nortriptyline. The neurology team was called, which and CC of 48 cm of axillary line and 50 cm of xiphoid
Hospitalizations due to respiratory infections, with suc-
found recent involuntary weight loss. Report of improved process. After 6 months, FVC was 240 ml and CC was
cessful recovery. Genetic testing revealed homozygous
median mononeuropathy. Head CT without changes. 49cm of axillary line and 50cm of xiphoid process. After
c.997C>T variant resulting in a change p.Arg333Trp. In
Lumbar puncture with 113 proteins, 100 nucleated cells 12 months, FVC was 250 ml and CC was 51 cm of axillary
silico prediction tools showed potentially deleterious
(100% lymphocytes), normal glucose, no pathogens. line and 52 cm of xiphoid process, and after 18 months,
value, and was reported as probably pathogenic based on
Electroneuromyography: left lumbar radiculoplexoneu- FVC was 290ml and CC was 55 cm of axillary line and 56
previous fast-channel CMS description associated with
ropathy, marked recent axonal sensory-motor involvement cm of xiphoid process. Case 2 – Male patient, SMA type
compound heterozygous for that variant. After CMS diag-
and active signs of denervation. Glycated hemoglobin 1 who started nusinersen at 4 months and received GT
nosis, the patient was kept in rehabilitation and waned
8.5% (poor glycemic control). Effective insulin therapy at 1 year 4 months, then nusinersena was stopped. The
immunotherapy. By the time of this report, the patient
was introduced. Discharged after 21 days, complete reso- patient underwent 10 assessments at 30-day intervals.
partially recovered motor functions, walks unaided and
lution of pain, walking with support. Discussion: Diabetic In the first evaluation the child was 1 year and 7 months
sustains improvement with pyridostigmine and ephedrine.
amyotrophy has an annual incidence of 4.2/100,000 and old, he presented FVC of 150ml and CC of 44cm of axil-
Discussion: CMS englobes a variety of genetically inher-
affects 1% of diabetics. Called Bruns-Garland Syndrome lary line and 48 cm of xiphoid process. After 3 months,
ited neuromuscular junction disorders. The most com-
or proximal diabetic neuropathy, it is installed, probably, FVC was 200 ml and CC was 48cm of axillary line and 50
mon form of CMS is post-synaptic and can be primarily
due to an ischemic lesion caused by microvasculitis. It cm of xiphoid process. After 06 months, FVC was 220ml
due to decrease in its subunit’s expression or by altering
appears as acute pain, proximal or distal, asymmetric and and CC was 49 cm of axillary line and 51 cm of xiphoid
the channel’s kinetic. CHRNA1 mutations causing CMS
focal, followed by weakness in the proximal lower limb, process, and after 09 months, FVC was 220 ml and CC
are rare, and generally severe at early onset. The present
autonomic dysfunction and weight loss. Final Comments: was 46 cm of axillary line and 50 cm of xiphoid process.
report describes a patient with relatively mild clinical
No treatment is proven to be effective. Data on immu- Final Comments: CC increased throughout the evalua-
features harboring a c.997C>T homozygous variant in
nosuppressive therapies are limited and controversial. tions, demonstrating satisfactory growth of the thoracic
CHRNA1 which was only previously reported in a patient
with compound heterozygosity. This variant prevalence cage. During the evaluation period, there were no serious
is approximately 5 in 105 and is probably associated with respiratory complications. Ventilatory support has been
Doenças Neuromusculares
autosomal recessive CMS. In summary, this case expands maintained only at night. The two cases maintained venti-
our knowledge of the complex genotype-phenotype cor- latory management through air stacking techniques, and
relation of CMS with demonstration of a homozygous mechanical assistance for cough. Thus, specific therapies
mutation expected to cause a very severe phenotype but for SMA, either alone or in combination, have been shown
instead originating a mild adult-onset phenotype. Final to be effective in improving respiratory function in SMA.
Comments: Since the only previous report of the same
variant was compound heterozygous, and definition of
kinetic disturbances and receptor deficiency requires Doenças Neuromusculares
functional proof, we acknowledge the benefit of further
histological and advanced neurophysiological testing.

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XXX Congresso Brasileiro de Neurologia

TL 1106388 TL 1106404 TL 1106412

MOLECULAR CHARACTERIZATION OF POLYRADICULONEURITIS BY VARICELLA SPINAL MUSCULAR ATROPHY WITH


CONGENITAL MYASTHENIC SYNDROMES ZOSTER VIRUS (VZV) WITHOUT CUTANEOUS PREDOMINANT LOWER EXTREMITY TYPE I
IN REFERENCE OUTPATIENT CLINIC, IN INVOLVEMENT IN A IMMUNOCOMPETENT (SMALED1) WITH NO SIGNS OTHER THAN
SALVADOR, BAHIA MAN: A CASE-REPORT PURE MOTOR SYMPTOMS
Mariana Soares Pinheiro, Ana Flávia Souza Freire da Ray Almeida da Silva Rocha, Thales Pardini Emanuela Coriolano Fidelix, Mário Emílio Teixeira
Silva Silva, Henrique Nascimento Dourado, Marcela Fagundes, Eder Leandro da Silva Dantas, Gabriela Dourado Júnior
Câmara Machado Costa Lopes de Morais, Ellen Silva de Carvalho, Larissa
Peres Delgado, Charles Maroly Lessa Mantovani, Onofre Lopes University Hospital, Universidade
Neurology Department – Hospital Santa Izabel. Trajano Aguiar Pires Gonçalves, Pedro José Federal do Rio Grandeo do Norte. Natal RN, Brazil
Salvador BA, Brazil Tomaselli, Wilson Marques Júnior Department of Integrated Medicine, Onofre Lopes
Escola Bahiana de Medicina e Saúde Pública. University Hospital, Universidade Federal do Rio
Salvador BA, Brazil Hospital das Clínicas da Faculdade de Medicina de Grandeo do Norte. Natal RN, Brazil
Escola Bahiana de Medicina e Saúde Pública – Ribeirão Preto. Ribeirão Preto SP, Brazil
Salvador – AP – Brazil efidelix@gmail.com
rasr.5@hotmail.com
nanapinheiro18@gmail.com Case Presentation: 5-year-old boy, with a history of
Case Presentation: A 62-year-old healthy man presented delayed motor development. It evolved with proxi-
Case Presentation: JGL, fem., 6y, frequent falls since 2y, with a 3-week history of progressive severe low back mal weakness in the lower limbs (Gowers signal pos-
evolved with difficulty to walk and to stand up. MS 4/5 pain and weakness. One week after pain onset, he noted itive), hyperreflexia, atrophy in the legs and lordosis.
distal and 3/5 proximal, axial hypotonia and areflexia. progressive proximal weakness in lower limbs and left Electroneuromyography showed chronic signs of dener-
Gait with adducted feet and tendency to fall. Genetic facial palsy. He had no fever, weight loss, choking or vation with normal sensory conduction. The father, not
testing (GT): DOK7 gene mutation. Improvement with recent travel history. He was evaluated twenty days after genetically tested, has atrophy in his legs. A large DNA
Albuterol. RSS, fem., 47y, ptosis, diplopia, fluctuating the symptoms onset and his neurologic examination panel of neuromuscular diseases (NGS) detected the
weakness of distal and cervical predominance. EMG: mild showed an MRC grading system (right/left) of: hip flexion heterozygous pathogenic variant, chr14: 101.986.017 C>T
neuromuscular junction (NMJ) disorder. GT: mutation 2/2, hip extension 2/3, knee flexion 4-/4, knee extension (or alternatively c.1792C>T – ENST00000360184) in the
in the CHRND, DOK7 and COL1 genes. Improvement 4/4, ankle dorsiflexion 5/5 and plantar flexion 5/5, along DYNC1H1 gene. Considering the clinical and electrophys-
with Albuterol and Fluoxetine. AJS, fem., 36y, weakness with mild weakness in the distal upper limbs (UL). Deep iological data and the pathogenic variant, this is a case of
in both LL with frequent falls since birth. Between 11-27y tendon reflexes were absent in the LL and normal in the SMALED1 due to a pathogenic variant of the DYNC1H1
used an unremembered medication with improvement. UL. Sensory examination reveled abnormal response to gene. Discussion: Spinal Muscular Atrophies (SMA) cor-
At 27y, evolved with fluctuating weakness in both UL, pain and temperature senstionss in the medial aspects of respond to a group of hereditary diseases caused by loss
cramps and fasciculations after cesarean section. MS 2/5 both legs. Vibration was absent on the ankles. There was or abnormal development of lower motor neurons. The
proximal and 4/5 distal. EMG: decremental pattern and a left-sided facial palsy House-Brackman (HB) grade III. major cause is SMA associated with homozygous dele-
postsynaptic impairment of NMJ. Reduced vital capac- No skin lesions were seen. Sensory and motor nerve con- tion of exons 7 and 8 of the SMN1 gene on chromosome
ity. GT: mutation in the DOK7 gene. Improvement with duction studies were normal in both UL and LL. Needle 5q. Other causes, called non-5q SMA, are less frequent
Albuterol and Fluoxetine. JMS, fem., 34y, fatiguing global examination showed diffuse acute denervation affecting and poorly recognized. One of these is SMALED1 (Spinal
weakness, bilateral ptosis and NPMD delay since birth. the lumbosacral segment bilaterally. Brain MRI showed muscular atrophy lower extremity predominant; OMIM
Consanguineous parents and three family generations bilateral facial neuritis, worse on the left. CSF revealed #158600), of autosomal dominant inheritance, associ-
with intellectual deficit and epilepsy. Presents facial lymphocytic pleocytosis, elevated protein and a CSF/ ated with the DYNC1H1 gene (OMIM #600112), with a
dysmorphia, bilateral semiptosis, paresis of VI CN, MS blood glucose ratio of 53%. Spinal MRI had diffuse lep- less aggressive evolution than 5q SMA. This gene lead to
3/5 proximal and 4/5 distal. EMG: postsynaptic involve- tomeningeal thickening and enhancement of thoracic two human motor neuropathies, SMALED and axonal
ment of JNM. Suspected of congenital myasthenia (CM), and lumbar nerve roots. He was treated with high doses Charcot-Marie-Tooth (CMT) disease. Final Comments:
with improvement with Albuterol but not Mestinom. GT: of oral steroids, with improvement of weakness and pain The phenotype SMALED1 show childhood onset, lower
mutation in the CHRNE gene. Discussion: CM is a rare in the lower limbs. Laboratory results evidenced elevated limb weakness, associated with arthrogryposis and cog-
and difficult to diagnose entity usually seen in childhood, levels of IgG for Varicella Zoster Virus (VZV) in blood and nitive impairment. There is great clinical variability, with
but may be insidious/present late. Common symptoms a 1: 8 titer for IgG VZV in CSF. Discussion: We present a cases of adult onset (after 18 years) and purely motor
include fluctuating muscle weakness, bulbar symptoms, rare manifestation of VZV infection with no skin lesions and proximal cases, simulating a myopathy, as in the
ophthalmoparesis and ptosis. The most common associ- in an immunocompetent patient. VZV remains latent in case described.
ated gene mutations are: CHRN, DOK7, RAPSN. CHRN the sensory ganglia of the peripheral nervous system after
encodes the subunits of the ACh receptor and the mutation a primary infection and can reactivate. Usually, during
deforms it, being able to cause two separate syndromes reactivation, there are papulovesicular skin lesions and Doenças Neuromusculares
depending on the affected subunit, as seen in RSS’s and neuropathic pain. Some patients show signs of segmental
JMS’s cases. DOK7 is an adaptive protein bound to MuSK. radiculopathy, but polyradiculitis as this patient presented
Its mutation can affect breathing since birth and cause is a very rare manifestation. VZV is also a relevant cause of
weakness and ptosis throughout life. There is marked cranial neuropathy, and the trigeminal and facial nerves
improvement with Albuterol or Ephedrine, as seen in are the most frequently affected. Patients often recover
the cases. Final Comments: Despite rare, CM should from motor deficits. Final Comments: The presence of
be suspected when the clinical history and therapeutic polyradiculoneuritis in immunocompromised patients
response isn’t similar to the usual response for Myasthenia should include VZV infection as a differential diagnosis.
Gravis. Genetic testing helps to guide the treatment and In the absence of immunosuppression and a character-
dictate the prognosis, modifying the quality and lifestyle istic skin lesion, diagnosis is challenging and laboratory
of the patient and their relatives. It also helps to identify tests are essential.
the most common mutated genes and new mutations
that can contribute to future diagnoses.
Doenças Neuromusculares

Doenças Neuromusculares

69
XXX Congresso Brasileiro de Neurologia

TL 1106419 TL 1106460 TL 1106554

CENTRONUCLEAR MYOPATHY: A CASE USE OF INSPIRATORY MUSCLE TRAINING IN CONGENITAL MYASTHENIC SYNDROME
PRESENTATION A PATIENT WITH AMYOTROPHIC LATERAL WITH DISTAL MYOPATHIC PHENOTYPE
SCLEROSIS: A CASE PRESENTATION
Herisson Rodrigues de Oliveira, Camilla Vanessa Natália Virgínia de Oliveira Ambrósio, Bruna Queiróz
Araujo Soares, Julia de Melo Nunes, Carolina de Aline Xavier Frota, Deborah Santos Sales, Carolina Vieira, Marcelo Sobrinho Mendonça, Thiago Cardoso
Cunha Correia, Isabella Araujo Mota Fernandes Garcia nunez Carrijo, Karina Lebeis Pires Vale

Universidade Federal da Paraiba. João Pessoa PB, Fiocruz. Rio de Janeiro RJ, Brazil Hospital e Maternidade Therezinha de Jesus – Juiz
Brazil Universidade Federal do Estado do Rio de Janeiro. de Fora – MG – Brazil
Departamento de Neurologia do Hospital Rio de Janeiro RJ, Brazil Hospital e Maternidade Therezinha de Jesus – Juiz
Universitário Lauro Wanderley. João Pessoa PB, de Fora – BA – Brazil
Brazil karinalebeis1@gmail.com
nvoambrosio@gmail.com
heri.md.92@gmail.com Case Presentation: Amyotrophic lateral sclerosis (ALS)
is a disease of the central nervous system characterized Case Presentation: A 50-year-old male with bipolar
Case Presentation: M. S, 48 years old, female, living in by a progressive degeneration of upper and lower motor disorder was evaluated due to a progressively-worsen-
João Pessoa, admitted with a complaint of muscle weak- neurons. Respiratory muscle weakness is commonly ing weakness that began 34 years ago. Weakness was
ness with progressive worsening since the age of 20. She observed in these patients, as well as a progressive reduc- predominantly distal and asymmetrical (right > left),
reported difficulty walking, running and climbing stairs tion in lung function, leading to complications such as involving his upper-limbs and causing fine motor activities
associated with falls, fatigue, significant dysphagia for alveolar hypoventilation and impairment of the cough impairment. He evolved with neck extensor weakness and
solids and difficulty holding objects, with progression of reflex. Despite the known effects of inspiratory muscle dysphagia. Physical examination revealed predominantly
symptoms over the years. The patient reports that limb training (IMT), there are few studies on the benefits of distal and right-sided weakness in his upper-limbs with
weakness intensified during and after pregnancy at age 32. this therapy in neuromuscular diseases. Therefore, this bilateral atrophy of thenar, hypothenar and interosseous
She is apathetic, and reports nocturnal awakenings and work aims to demonstrate the results obtained through muscles. EMG showed proximal and distal post-synaptic
frequent choking. On physical examination, she has proxi- a pulmonary rehabilitation program with IMT protocol, neuromuscular junction disease associated with distal
mal tetraparesis, grade 3 muscle strength in iliopsoas, del- associated with a multidisciplinary approach in patients upper-limb myopathy. Family history revealed a similar
toids and biceps, and grade 4 in the other muscle groups; with ALS. Methods: 38 years old patient, with a clinical condition in his paternal grandmother, father and two
global areflexia; positive Gowers sign, myopathic lifting diagnosis of ALS for 2 years, followed in the same period daughters. Genetic test yielded Congenital Myasthenic
and falling, limiting limb movement. In October 2020, in a pulmonary rehabilitation program twice a week, Syndrome (CMS) related to a mutation in the CHRNA 1
she underwent an echocardiogram, which showed mild period: 30 to 40 min, protocol: IMT with Powerbreathe® gene. Discussion: CMSs include a group of neuromuscular
tricuspid valve regurgitation. In addition, she has a family Plus Medic in interval exercises, with load 30% of MIP; diseases of genetic origin with variable genotype and phe-
history of muscle weakness, a great-grandmother and two Expiratory exercises with EMST 150® load of 30cmH2O; notype. Clinically, patients present fatigability, transient
sisters, and the biopsy of a sister is compatible with an Pulmonary hyperinflation exercises with ambu®. Non- or permanent weakness of the extrinsic ocular, facial, bul-
autosomal dominant centronuclear myopathy. She denies invasive ventilation (NIV) was not used during pulmo- bar, trunk, respiratory or limb muscles. Symptoms usually
consanguinity between her parents. Polysomnography, nary rehabilitation, only used once a day and during the start in childhood and may be present from birth or more
electroneuromyography (ENMG) and muscle biopsy were night. Results: For a year, the patient increased inspiratory rarely in adolescence. The CMS 1A is related to a mutation
requested. ENMG showed a predominantly proximal strength by 30 cmH2O, reaching MIP -140cmH2O in this in the CHRNA1 gene and has an autosomal dominant
myopathic pattern. The histological result was compati- period and maintained the expiratory strength without character, but with variable penetrance and expression,
ble with autosomal dominant centronuclear myopathy. losses or gains at +110cmH2O. Regarding pulmonary explaining the different phenotypes. Mutations occur in
Polysomnography showed 1 episode of desaturation. The function during this period, the patient showed a slight the acetylcholine receptor (AChR) subunits causing a
patient was referred for neurorehabilitation in order to decrease in FEV1 (99 – 93%) and FVC (94 – 86%) with no delay of the opening time of the acetylcholine ion channel
receive multidisciplinary care, in addition to receiving pattern of pulmonary restriction or obstruction. There was that are located at the neuromuscular junction. CMS 1A
genetic counseling. Discussion: Centronuclear myopathy an improvement in the subjective sensation of exertion, should be suspected in patients of any age who present
is a congenital muscle disorder with a heterogeneous clin- where the resting BORG reduced from 3 to 0. It is possible weakness and fatigability of the scapular, cervical and
ical presentation, which usually involves muscle fatigue, to conclude that IMT can be a feasible therapy and bring dorsal forearm (wrist and finger extensors) muscle groups
muscle atrophy and varied cardiorespiratory involvement, benefits to the patient with neuromuscular disease, how- in which there is no response to the use of cholinesterase
characterized histologically by the prominence of muscle ever, more studies in the area are necessary. inhibitors or the worsening of symptoms. The diagnosis
fibers with centralized nuclei. The patient in the pres- is based on the symptomatology and on the electroneu-
ent case presentation a progressive worsening of symp- romyography findings that demonstrate a decrease in the
toms, emphasizing a decline in strength after pregnancy. Doenças Neuromusculares repetitive stimulus; the dosage of anti-AChR antibody can
Furthermore, the presence of dysphagia, with episodes of also be performed. Final Comments: CMS with myopathic
postprandial choking, was also reported. Even with this phenotype is a rare presentation, with only a few cases
situation, she is completely independent for daily activ- described in the literature with different phenotypes in
ities, with maintenance of quality of life, with multidisci- the same family. Our patient had the distal myopathic
plinary follow-up. Final Comments: Finally, the present phenotype while his two daughters had CMS of the slow
case has its relevance in reporting a case of centronuclear channel type with mutation in the same gene.
myopathy, a rare disease with an incidence of 1: 100,000
live births, with the presence of atypical findings, as well
as the association of the patient with worsening of symp- Doenças Neuromusculares
toms after pregnancy.

Doenças Neuromusculares

70
XXX Congresso Brasileiro de Neurologia

TL 1106561 TL 1106572 TL 1106585

GUILLÁIN BARRÉ SYNDROME: AN ATYPICAL THE IMPORTANCE OF DIAGNOSING POMPE CONGENITAL MYOPATHY WITH MYBPC1
PRESENTATION DISEASE AMONG NEUROMUSCULAR GENE MUTATION ASSOCIATED WITH
DISEASES: A CASE PRESENTATION MUSCULAR ACTIVITY TREMOR-LIKE
João Pedro Cotrim Rodrigues, Fábia Lais Cotrim
Fernandes, Rodrigo Defensor Meira, Renata Cotrim Victoria Faustino Silva Reis, Murilo Lopes Coelho, Marcelo Sobrinho Mendonça, Thiago Cardoso Vale,
Rodrigues Juliana Silva Almeida Magalhães, Marcela Camara Natália Virgínia Oliveira Ambrósio, Bruna Queiróz
Machado Costa, Daise Larissa Ribeiro França, Vieira
Universidade Federal do Tocantins, UFT. Palmas TO,
Adriana Virgínia Barros Faiçal, Adriele Ribeiro
Brazil Serviço de Neurologia do Hospital e Maternidade
França, Gabriel Vianna Pereira Aragão, Lara Cordeiro
Hospital Nova Aliança – Guanambi – BA – Brazil Therezinha de Jesus – Juiz de Fora – MG – Brazil
Magalhães, Henrique Nascimento Dourado
Faculdades Integradas Padrão, FIP Guanambi –
Guanambi – BA – Brazil Escola Bahiana de Medicina e Saúde Pública. marcelocmjf@hotmail.com
Universidade Federal do Tocantins. Palmas TO, Salvador BA, Brazil
Brazil Hospital Martagão Gesteira. Salvador BA, Brazil Case Presentation: A 10-year-old boy, born with cleft
palate that was corrected at age 1.5, with a history of
João.cotrim@mail.uft.edu.br victoriareis20.2@Bahiana.edu.br delayed language development, presented for assessment
of tremor. Relatives inform the presence of postural and
Case Presentation: A 50-year-old man, hypertensive and Case Presentation: TRA, male, 3 years and 7 months old. action tremor since birth. Patient complained of tremor
pre-diabetic, reported polyarthralgia, low-grade fever, At 2, he started having abdominal pain and alteration in interfering with his daily living activities associated with
adynamia and prostration with gradual worsening for 9 the transaminases. After an investigation with a gastro- myalgia affecting his lower-limbs, particularly when
days. On the fifth day of evolution, he presented voiding pediatrician, without a clear diagnosis, a neuromuscular running. On physical examination, it was noted proxi-
effort and burning pain on the extremities, followed by condition was suspected, and the patient was referred to mally-predominant tetraparesis and postural and action
clear urinary retention, requiring relief probing, intestinal a neuropediatrician. The mother reported that, from 1 tremor. EMG showed notably proximal myopathy without
constipation, in addition to progressive ascending and year and 6 months old, the child began to show difficulty complex repetitive or myotonic discharges. Surface elec-
symmetrical paraparesis. The neurological examination to climb stairs, symptoms that were not valued. At 2 and tromyographic study of tremor, with a channel recording
showed mild facial diparesis and dysphonia, grade 4 3 months, he began to have difficulty walking, dyspha- the flexor and another one recording the extensor muscles
paraparesis and hyporeflexia in the lower limbs, with- gia, fatigue on moderate and big efforts and nocturnal of forearms, showed continuous muscular activity in the
out sensory level. 24 hours later, he already had grade hypoventilation. The CPK level was 2175, and molecular extensor muscles during wrist extension, with no bursts
2 paraparesis. CSF with no cells and 76 mg/dL of pro- tests for spinal muscular atrophy, Steinert’s myotonic (typical sign of tremor). Brain MRI was normal. There
tein. Arboviruses serologies were non-reactive, as was dystrophy and facioscapulohumeral muscular dystro- was no family history of neurological diseases. Given the
PCR COVID. Performed treatment with immunoglobu- phy were negative. Electroneuromyography revealed clinical picture suggestive of myopathy, a neuromuscular
lin 400 mg/kg/day for 5 days (starting on the 11th day) altered motor response. Neuromuscular Disease Panel panel was ordered and the genetic test confirmed MYBPC1
in an intensive care unit, without the need for mechan- was performed: 2 acid alpha-glucosidase (GAA) variants gene mutation, thus, a congenital myopathy. Discussion:
ical ventilation, where he developed non-dialytic acute in heterozygous pathogenic were found, which confirms Congenital myopathies are a group of inherited muscle
kidney injury, but completed the proposed regimen. the diagnosis of Pompe disease, with an alpha-glycosi- disorders clinically characterized by hypotonia and weak-
Electroneuromyography revealed axonal active senso- dase dosage of 0.26. In addition, the patient has Autistic ness. It usually begins at birth and has a static or slowly
rimotor polyradiculoneuritis (AMSAN), predominantly Spectrum Disorder. Discussion: Pompe disease has an progressive clinical course. Diagnosis and classification
in the lower limbs. For differential diagnosis with myelo- incidence of 1: 40,000 live births, being a lysosomal stor- of congenital myopathies has historically been based on
radiculopathy, spinal MRIs with contrast were requested, age disease caused by a mutation in the gene encoding clinical features and histopathology. Nevertheless, recent
which showed no relevant changes. Discussion: GBS is GAA, leading to intracellular glycogen accumulation. advances in genetics have changed diagnostic practice.
an acute autoimmune polyradiculoneuritis that primarily The disease is characterized by muscle weakness, hypo- The gene MYBPC1 encodes a member of the myosin-bind-
affects the myelin of the proximal portion of peripheral tonia, dysphagia, and respiratory failure, cardiomyopathy, ing protein C Family (sMyBP-C), a modular sarcomeric
nerves in an acute/subacute manner. Its most common among other nonspecific symptoms. It can be classified protein that exerts structural and regulatory roles through
etiology is post-infectious, but less important precipitat- in Infantile Onset Pompe Disease (IOPD) and Late Onset dynamic interactions with actin and myosin filaments of
ing factors are surgery, immunization and pregnancy. The Pompe Disease (LOPD). IOPD symptoms appear up to the muscle. This protein has three isoforms: cardiac, fast
diagnosis is clinical, complemented by laboratory and 12 months of age, with associated heart disease. In the skeletal and slow skeletal. The main roles attributed to the
electrophysiological tests, and two criteria are essential, late form, symptoms appear after 12 months, but are less MyBP-C family are the stabilization of thick filaments and
such as progressive weakness of more than one limb or severe. The diagnosis is made by the GAA enzyme dosage, the regulation of the cross-bridge cycle. Recent studies
cranial muscles of varying degrees, and hyporeflexia followed by a molecular test. The treatment is carried out demonstrate that the genesis of tremor may be associated
and distal areflexia with varying degrees of hyporeflexia by the enzyme replacement. The introduction of enzyme with mutation in some gene positions; this hypothesis is
proximal. In this case, the patient demonstrated atypical replacement in patients diagnosed with Pompe, in 2006, supported by the formation of cross-bridges by the sar-
manifestations of GBS, with urinary retention/constipa- changed the prognosis of the disease, as it stabilized the comeric protein. Final Comments: Constant advances
tion and fever at the beginning of the condition, leading damage caused by the intracellular accumulation of gly- in the field of genetics have led to a significant increase
to the imperative exclusion of other differential diagnoses, cogen. Therefore, with early diagnosis, it is possible to in knowledge of the broad phenotypic spectrum, includ-
which did not delay the start of treatment. Given the rapid quickly institute the appropriate therapeutic measures, ing overlapping phenotypes, of congenital myopathies.
evolution, and late search for specialized medical care, ensuring a better quality of life and preventing irrevers-
the prognosis was unfavorable. Final Comments: GBS is a ible lesions. Final Comments: Pompe disease, although
disease whose incidence has increased dramatically in the rare, should be considered as a differential diagnosis in Doenças Neuromusculares
COVID era, and its complications can lead to high degrees pediatric patients with neuromuscular symptoms. Since
of disability and, in some cases, death. Identification of it has a disease-modifying therapy, an early diagnosis
GBS and early initiation of specific treatment are essential can significantly change the patient’s prognosis. There
to reduce recovery time and minimize sequelae. is no evidence of a relationship between Pompe disease
and Autism.
Doenças Neuromusculares
Doenças Neuromusculares

71
XXX Congresso Brasileiro de Neurologia

TL 1106596 TL 1106597 TL 1106610

PURELY BULBAR MYASTHENIA GRAVIS: A RECURRENCE OF BREAST NEOPLASM IN CASE PRESENTATION ATIPICAL LATE ONSET
CASE PRESENTATION MULTIPLE SCLEROSIS: CASE PRESENTATION POMPE DISEASE IN A 36 YEAR PATIENT
WITH SPASTIC PARAPARESIS
Natália Virgínia de Oliveira Ambrósio, Bruna Camila de Araújo Toscano, Louisy Carvalho Araújo,
Queiróz Vieira, Laís Ciribelli Yamaguchi, Laura Fernando de Paiva Melo Neto, Raquel Minervino de Edson Júnior Gonçalves Bechara, Annelise Akemi
Altomare Fonseca Campos, Jéssica Cristina Silveira Carvalho Sobrinha, Rayana Tavares de Queiroz, Davi Higa Lee, Felipe Teijeiro Cabral, Luiza Helena
Damasceno, Bruno de Magalhães Barbosa Leite, Veloso Guerra, Bianca Etelvina Santos de Oliveira Chuque Medina, Keila Narimatsu, Francisco Tomaz
Daniel Teixeira Martins Schettini, Fabiana Veloso Meneses de Oliveira, Rubens José Gagliardi
Ferreira, Marcelo Sobrinho Mendonça, Thiago Centro Universitário de João Pessoa – Unipê. João
Cardoso Vale Pessoa PB, Brazil Hospital Santa Casa Misericórdia de São Paulo, São
Fundação Centro Integrado de Apoio à Pessoa com Paulo SP, Brazil
Hospital e Maternidade Therezinha de Jesus – Juiz Deficiência FUNAD
de Fora – MG – Brazil a_akehiga@yahoo.com.br
camilatoscanno@gmail.com
nvoambrosio@gmail.com Case Presentation: Male, 36 years old, smoker 1 pack of
Case Presentation: Female, 54 years old, with Relapsing- cigarette per day for 20 years, without previous comorbid-
Case Presentation: A 69-year-old female, previously Remitting Multiple Sclerosis, diagnosed in February 2013, ities, with cosanguinous parents (cousins). Patient refers
hypertensive, was evaluated for sudden dysphagia with she used Betainferon 1a and is currently using Glatiramer lower limb paresis, initially on the right distal, with pro-
mild dysarthria and dysphonia. Neurological examina- Acetate. In 2017, she presented a breast nodule in the left gressive worsening and spasticity, started three years ago.
tion also showed hypoesthesia of the right hemiface. breast, identified as Invasive Ductal Carcinoma, undergo- Refers 8 kg loss of weight in the period. He denies alteration
She spontaneously recovered 24 hours before admission ing neoadjuvant treatment with chemotherapy and sub- of upper limbs. Report six months ago, beginning of dys-
to Hospital. There was no alcohol or drug use history. sequent excision, which occurred without intercurrences phagia for liquids and dysphonia. Neurological physical
Laboratory tests and cranial CT were normal, but electro- or complications. Later, in 2022, she presented a nodule examination shows dysphonic speech, muscle strength
cardiogram showed an atrial fibrillation rhythm. Within in the right breast, and a biopsy was performed and iden- grade IV- in lower limbs with spastic rigidity, exalted reflex
48 hours of admission, the symptoms returned, with tified as Invasive Ductal Carcinoma, with estrogen and in lower limbs without signs of pyramidal release and a
sudden severe dysphagia, which worsened throughout progesterone receptor positive and HER2 negative, being spastic gait. Serological tests for HIV and syphilis are neg-
the day, requiring the use of a nasoenteral tube. She was in clinical follow-up and therapeutic management with ative. Brain Magnetic resonance imaging (MRI) without
urgently submitted to a new cranial CT with intracranial hormone therapy. Discussion: Multiple Sclerosis (MS) is alterations and cervical, thoracic and lumbar spine MRI
and cervical CT angiography, but both were normal. Brain an autoimmune disease of a degenerative nature, which with normal spinal Cord sign. Electromyoneurography
MRI was then performed, but was also normal. We ended leads to demyelination of the Central Nervous System. (ENMG) with Carpal tunnel syndrome. Positive genetic
up ruling out a vascular cause and pursued a therapeutic Regarding the link with neoplasms, in a cohort study, it test for Pompe disease. Discussion: Pompe disease (PD),
test with pyridostigmine 60 mg and oral corticosteroid. A was found that the presence of MS is associated with an or glycogen storage disease type II, is a rare inherited
positive response was rapidly seen during the first hours increase in all-cause mortality, but it was not associated autosomal recessive disease caused by a deficiency in
after administration, evolving with progressive dysphagia with changes in neoplasm specific survival. In patients acid alpha-glucosidase (GAA) enzyme. The late-onset
improvement. She tested negative for muscle-specific with MS, longer diagnostic intervals may occur if the phenotype results from an incomplete GAA deficiency,
thyrokinase and acetylcholine receptor binding antibod- symptoms of neoplasms were wrongly attributed to the which appears later in childhood, in adolescence or in
ies. Electroneuromyography with repetitive stimulation underlying disease or if the cancer symptoms were less adulthood. It is characterized by a slowly progressive
and chest tomography came out normal. Discussion: relevant than other health problems. In addition, MS myopathy. The diagnosis of late-onset Pompe disease
Myasthenia gravis is an autoimmune disease of the post- related disability, which increases with age, as well as the (LOPD) is still challenging and often quite delayed. It
synaptic neuromuscular junction characterized by fluc- occurrence of neoplasms, can make access to diagnostic is not unusual for patients to go undiagnosed for many
tuating weakness involving ocular, bulbar, limb and/or tests, such as mammography, difficult. Thus, the lower rate years as screening for PD is often overlooked or delayed
respiratory muscles. The disease occurs at any age, with of cancer detection through screening in these patients in adults. LOPD it is usually milder than the infantile-on-
an early peak in the 2nd and 3rd decades and a late peak compromises disease control, negatively affecting sur- set forms of this disorder and is less likely to involve the
between the 6th and 8th decades. Diagnosis is based on vival. Final Comments: Studies show that patients with heart. Most individuals experience progressive muscle
investigation of the clinical history and typical examination MS do not appear to have an increased risk of developing weakness, especially in the legs and the trunk, including
findings. More than 50% of patients have isolated ocular neoplasms. However, some have suggested an increased the muscles that control breathing. The treatment incluid
symptoms of ptosis and/or diplopia and only about 15% risk not only of breast cancer, but also of the CNS, urinary enzyme replacement therapy. A drug called alglucosidase
of patients have bulbar symptoms. Final Comments: tract and nasopharynx. Commonly, these observations alfa is given intravenously. It may help reduce the buildup
This is a case presentation of a 69-year-old hypertensive are based on a small number of cases, which makes these of glycogen inside cells, and slow the progression of the
woman who suddenly started the condition with isolated estimates inaccurate. Thus, understanding the pathways disease. Final Comments: The present case of a patient
bulbar symptoms, without involvement of other muscle to early detection of breast cancer is critical to develop- with adult-onset progressive spastic paraparesis with a
groups, mimicking a posterior circulation stroke/tran- ing and planning interventions to improve outcomes for positive genetic test for pompe’s disease. A clinical mani-
sient ischemic attack. Although the initial hypothesis was people with MS and cancer. festation not commonly seen in late-onset Pompe disease.
a vascular condition, the fatigability presented led to the
suspicion of myasthenia. The case highlights the impor-
tance of differential diagnosis of sudden neurological Doenças Neuromusculares Doenças Neuromusculares
events, particularly involving bulbar symptoms, which
should raise suspicion for myasthenia gravis.

Doenças Neuromusculares

72
XXX Congresso Brasileiro de Neurologia

TL 1106615 TL 1104889 TL 1105393

FAMILY AMYLOIDOTIC POLYNEUROPATHY: A OPHTHALMIC HERPES-ZOSTER AFTER USE OF SPINAL ELECTRODE IN A PATIENT
RARE DISEASE INCREASINGLY COMMON VACCINATION AGAINST COVID19: CASE WITH VASCULITIC NEUROPATHY
PRESENTATION
Fábia Lais Cotrim Fernandes, João Pedro Cotrim Beatriz Oliveira Cardoso, Adria Melissa Silva
Rodrigues, Rodrigo Defensor Meira, Renata Cotrim Antônio Rodrigo Lima Alves, Beatriz Maria Andreoli Campos, Marjorie Bindá Leite, Nabil Abuchahin,
Rodrigues, Carlindo Malheiros Castro Marques Fellini, Silvio Marcelo Silva Fernandes, Lorena Franklin Reis
Martins Antunes, Gustavo Leite Antunes, Gisele
Hospital Nova Aliança, Guanambi – Guanambi – BA Faculdade Metropolitana de Manaus. Manaus AM,
Tezolin Menezes, Emerson Oliveira Lise
– Brazil Brazil
Universidade Federal do Tocantins, UFT. Palmas TO, Faculdade Metropolitana de Manaus. Manaus AM,
Brazil beatrizocco@outlook.com
Brazil
Faculdades Integradas Padrão, FIP Guanambi – Centro Universitário Fundação Assis Gurgacz.
Guanambi – BA – Brazil Case Presentation: Female patient, 23-year-old, reports
Cascavel PR. Brazil
Clínica Sagrada Saúde, Guanambi – Guanambi – BA sudden onset of petechiae on the lower limbs, from the
Hopsital Beneficente Portuguesa de Manaus.
– Brazil knee to the foot, which bilateral and apparently symmetri-
Manaus AM, Brazil
cal character. The lesions disappeared after a month, when
Universidade Nilton Lins. Manaus AM, Brazil
João.cotrim@mail.uft.edu.br pain appeared in the same location. After consultation
rodrigo.wings@gmail.com with specialists, the patient underwent electroneuromy-
Case Presentation: A 76-year-old man, from Guanambi- ography, which revealed a distal symmetrical pattern and
BA, born in Cansanção-BA, with kidney disease and heart Case Presentation: The patient: Female, 59 years old. No received a multidisciplinary diagnosis of vasculitic neurop-
disease, with an implantable cardioverter-defibrillator, noteworthy medical history, including denying Varicella athy. The rheumatological treatment partially improved
with symmetrical paresthesia and dysesthesia in gloves infection during childhood. On 04/13/2021, she received the condition, but the intensity and frequency of pain
and boots, in addition to dysautonomia with episodes the first dose of the vaccine against COVID-19 (Coronavac) increased after a while. An evaluation with a doctor, pain
of syncope, which had been worsening for about 1 year. without post-vaccination reactions. On 05/07/2021, she specialist, was requested to perform a spinal electrode test
On neurologic examination, moderate symmetrical distal received the second dose of the Coronavac vaccine, with no in the thoracolumbar region. There was a good response
hypopalesthesia and gait of small steps with wide base adverse reactions until 05/14/2021, when she noticed the with the test, decreasing more than 50% on the pain scale,
without support. Four-limb ENMG (26/04/2022) showed appearance of vesicobullous lesions with an erythematous without intolerable side effects, and so it was decided to
chronic polyneuropathy with axonal predominance in the base associated with ocular edema, electric shock-like pain implant the electrode and the generator permanently.
lower limbs, distal and proximal involvement, symmet- and local hyperesthesia, evidenced in the course of the V1 The patient is in the seventh month of follow-up, with
rical, moderate to severe. The transthoracic echocardio- branch of the trigeminal nerve, being clinically diagnosed pain intensity around 3, on the Visual Analogue Scale
gram showed significant biatrial enlargement, absence of as ophthalmic herpes-zoster. Treatment with Acyclovir (VAS), with improved quality of life. Discussion: Vasculitic
intracavitary thrombi, diastolic dysfunction, significant left was started, but hyperesthesia persisted. Discussion: neuropathy is characterized by the submission of a vessel
ventricular hypertrophy, minimal pericardial effusion and Trigeminal neuralgia is characterized by paroxysms of by an infiltrate of cells due to inflammation, generating
mild to moderate mitral regurgitation. Myocardial pyro- excruciating pain in the V nerve. Among the infectious ischemia and injury to peripheral nerves. The diagnosis
phosphate scintigraphy (26/04/22): strongly suggestive of causes, herpes zoster (HZ) should be considered. After is made by biopsy of the suspected nerve and its treat-
amyloidosis. The panel for transthyretin-linked heredi- primary infection with Varicella, the virus becomes qui- ment is done with immunosuppressants. However, this
tary amyloidosis revealed susceptibility to amyloidosis, escent in cranial sensory nerve ganglia and spinal dorsal. condition is still poorly documented, since only 31 results
variant val142Ile. The Fabry test was negative. Tafamidis Regarding vaccination against COVID-19 until the present were found in the search for MeSH Terms (vasculitic) AND
meglumine 80 mg/day was requested for his treatment. writing of this article, case presentation were observed in (neuropathy) in the Pubmed database, making this issue
The investigation of first-degree relatives (ongoing) has Europe. No case presentation were found on the South a real challenge. The importance of spinal electrical stim-
so far revealed two asymptomatic susceptible cases. American continent. A systematic review entitled ‘‘Can ulation in the treatment of neuropathic pain refractory
Discussion: Familial Amyloid Polyneuropathy (FAP) is SARS-CoV-2 vaccine increase the risk of reactivation of to pharmacotherapy has become clear. Its mechanism of
a rare multisystem disease characterized by an autoso- Varicella zoster?’’ showed a relationship between HZ action works according to the pain gate theory, in which
mal dominant transmission, in which the mutant form development and mRNA vaccination against COVID- the posterior horn of the spinal cord controls the passage of
of transthyretin (TTR) is synthesized in the liver. More 19. A total of 54 cases were analyzed. The median period stimuli by activating larger-diameter fibers and inhibiting
commonly, FAP presents in clinical conditions of varying between the development of HZ and vaccination against the passage of stimuli from smaller-diameter ones. Final
forms and intensities that mix familial amyloidotic poly- COVID-19 was 7 days. 68.27% of HZ cases were reported Comments: Therefore, although vasculitic neuropathy is
neuropathy and familial amyloidotic cardiomyopathy. by mRNA vaccine. Thirty-six patients 36/45 developed HZ not among the most evident pains, this work addresses
The diagnosis is clinical and based on the presence of after the initial dose of COVID-19 vaccine among those the issue between it and the treatment of chronic pain
the genetic mutation in TTR. In the case in question, the who received the mRNA vaccine. This led to the hypoth- through the use of spinal electrode, having in this report
changes were compatible with FAP, whose variant val142Ile esis that the disease, with physical stress, could trigger a good response to treatment. Considering that the elec-
is predominantly associated with cardiomyopathy, renal HZ. Functional impairment of T lymphocytes could reac- troneuromyography of the disease reveals axonal loss, It
dysfunction and polyneuropathy. Final Comments: The tivate varicella zoster virus. Another study titled “Phase I/ is possible that just the rheumatologic treatment, alone,
diagnosis of FAP at a pre-clinical stage has an important II study of COVID-19 RNA vaccine BNT162b1 in adults” is not sufficient for sensory recovery. Therefore, it is nec-
epidemiological value, aiming at mapping the mutation on Nature, showed a transient decrease in lymphocyte essary to resort to complementary therapies to alleviate
in the community. In addition, the early initiation of counts observed within a few days after vaccination and the patient’s suffering and generate greater functionality.
treatment is related to the symptomatic improvement of return to baseline within 6-8 days on all participants. Thus,
the patient, minimizing the evolution of the disease. The associating this post-vaccination lymphocytopenia and
dissemination of information and accessibility to genetic the Varicella virus reactivation, precisely when there is a Dor
tests has led to an increase in the incidence of FAP, which drop in CD4+ and CD8+ levels to be a plausible justifi-
becomes a possible etiology in the face of polyneuropa- cation. Final Comments: The report a case in which HZ
thies without a specific cause. 7 developed days after vaccination against COVID-19 in
a patient without comorbidities. Consider the report of
importance to the scientific community.
Doenças Neuromusculares

Dor

73
XXX Congresso Brasileiro de Neurologia

TL 1105783 TL 1105869 TL 1105895

SUCCESSFUL TREATMENT WITH SPINAL ACUTE PAINFUL DIABETIC NEUROPATHY: PULSED RADIOFREQUENCY FOR POST-
CORD STIMULATION IN A PATIENT WITH PRESENTATION OF A TREATMENT-INDUCED COVID OCCIPITAL NEURALGIA: A CASE
TESTICULAR TORSION: CASE PRESENTATION CASE PRESENTATION
Steffany Protásio Moura de Oliveira, Giovana de Raimundo Marcial de Brito Neto, Gustavo Silva Luís Felipe Ferreira Marques, Luiz Severo Bem
Oliveira Sarubi, Hitesh Babani, Denner Matheus Schafascheck, Renata Cavalvanti Eiras, Luiza Aguiar Júnior, Nilson Batista Lemos, Otávio da Cunha
Ribeiro Nascimento, Ornella Aquino da Silva, Lima, Priscila Santos Mageste, Mariana Soares de Ferreira Neto, Joaquim Fechine de Alencar Neto,
Franklin Reis Freitas Tamy, Isadora Versiani de Lemos, Matheus Hildo Rocha Cirne de Azevedo Filho, Júlio Augusto
Nunes Ferreirinha Leite de Castro, Camila Castelo Lustosa, Arthur Oliveira Lira, Maria Luísa Rocha,
Fametro. Manaus AM, Brazil Branco Pupe, Osvaldo José Moreira do Nascimento Luis Bandeira Alves Neto
Hospital Adventista de Manaus. Manaus AM, Brazil
Universidade Federal Fluminense. Niteroi RJ, Brazil Universidade do Estado de Mato Grosso – Cáceres
steffany.protasio@gmail.com – MT – Brazil
marcialbrito@live.com Universidade Federal de Pernambuco. Recife PE,
Case Presentation: Chronic testicular pain is defined Brazil. Recife PE, Brazil
as constant or intermittent pain in the testicle lasting Case Presentation: Female, 19 years old, presented in Centro Universitário Unifacisa. Campina Grande PB,
three months or more, which generates impairment in October 2021 to the neurology service with a picture of Brazil
patients’ daily activities. For those who do not respond burning pain in the lower limbs. History of DM1, diag- Universidade Católica de Pernambuco. Recife PE,
to conservative treatment and nerve blocks in the spinal, nosed when he developed diabetic ketoacidosis in 2018; Brazil
a good option is to use electrode implants. We can state Treated with insulin and oriented on the management Departamento de Neurocirurgia do Hospital da
that peripheral neuropathic pain is a type of chronic pain of the disease. Follow-up was irregular in the first two Restauração. Recife PE, Brazil
that occurs when there is injury or damage to the somato- years, with no attendance at follow-up appointments. Centro Universitário de Brasília. Brasília DF, Brazil
sensory system, which persists for longer than 3 months Readmitted to the endocrinology department in March Universidade de Pernambuco. Recife PE, Brazil
or remains for more than 1 month after the resolution of 2021; She weighed 70kg and had an HbA1C index of
an acute tissue injury. In this case presentation, we will 15.6%. She was oriented and showed no changes in the luisfelipe.marques585@gmail.com
describe a young patient who presented testicular torsion level of consciousness. Returns in September 2021, with
and evolved with peripheral neuropathic pain, undergoing 55kg, after strict metabolic control. HbA1C of 6.9%, with Case Presentation: Patient, 45 years old, male, history
treatment with implantation of a spinal cord stimulation intense pain, characterized by numbness and a burning of infection by Covid-19, was attended at an outpatient
electrode. With this report, we want to describe the quali- sensation involving bilateral feet. Her thyroid profile neurosurgery service complaining of pain in the occip-
tative outcome of the patient with chronic testicular pain was within normal limits, as was vitamin B12. She was ital and cervical region, of strong intensity, paroxysmal,
treated with a spinal electrode. medicated with 60mg/day of morphine and referred burning type. On physical examination, he had more
to the neurology department for investigation. A nerve marked paresthesia on the right and a positive Tinel’s
conduction study suggested bilateral axonal predomi- sign on the right. Test blockade of the greater and lesser
Dor nant sensory polyneuropathy of the lower limbs. Upper occipital nerves with anesthetic solution showed improve-
limb nerve conduction studies were normal. The cause ment in the condition for 4 days. From the diagnosis of
of the neuropathy was diagnosed as treatment-induced post-Covid-19 Occipital Neuralgia, he underwent neu-
neuropathy (TIN). Treatment was started with opioid romodulation therapy with pulsed radiofrequency in
dose reduction and gradual introduction of amitripty- the C2 dorsal root ganglion, at a temperature of 42 °C,
line and gabapentin. In the control consultations, he for 3 minutes, with repetition after an interval of 3 min-
showed good evolution, despite gradually lower doses utes. The procedure was uneventful, with complete res-
of medication. Discussion: Acute painful diabetic neu- olution of pain and Hospital discharge on the same day.
ropathy (APDN) is rare and seen in both type 1 and type Discussion: In regard to chronic neuropathic pain, there
2. The incidence of APDN is not known and is probably are reports of it being triggered by psychological stress-
less than 1 in 1,000 diabetic patients. APDN can manifest ors, viral infection or as a result of Hospitalization in the
as treatment-induced neuropathy (TIN) and diabetic intensive care unit. There are post-viral immunological
neuropathic cachexia (DNC). Studies have shown that reactions capable of promoting an environment condu-
it can occur after rapid glycemic control with insulin, cive to the establishment of neurological complications.
as in the case presented here, and in patients using oral Neural virulence would likely be linked to violations of
antidiabetic drugs. A set of diagnostic criteria for TIN is the blood-brain barrier, either by endothelial invasion
proposed, including: (1) acute onset of neuropathic pain through the binding of the virus’s Spike protein to the
or autonomic symptoms, (2) a decrease in HbA1c level of angiotensin-2-converting enzyme, or trans-synaptically
more than 2% at 3 months, and (3) onset of neuropathic through the invasion of the olfactory nerves, or even an
pain and /or autonomic symptoms within 8 weeks after increase in the permeability of the barrier caused by the
the decrease in HbA1c level. Pharmacological treatment release of cytokines resulting from the systemic inflam-
should be performed in conjunction with the holistic matory process as a reactional response. Pulsed radiofre-
management of the patient. Final Comments: Physicians’ quency consists of radiofrequency emission by a catheter
awareness of this rare and completely reversible condition through an electrode, positioned close to a nociceptive
is necessary to ensure proper treatment and adherence pathway, in short pulses, leaving intervals between emis-
to glycemic control. sions in order to allow the affected tissue to cool. The area
affected can be well defined, being restricted to the nerves
responsible for transmitting and modulating painful sen-
Dor sations. In the case of occipital neuralgia, the electrode
implant is placed in the C2 ganglion, where greater and
lesser occipital nerves emerge, thus allowing pulsed and
non-continuous waves to induce a low-intensity elec-
tric field around these nerves, aiming to modulate and
regulate the activity of A-delta fibers and C fibers. Final
Comments: Occipital neuralgia implies a careful investi-
gation and carries a vast possibility of treatments, among
them, pulsed radiofrequency neuromodulation acquires
notoriety along with its promising results.

Dor

74
XXX Congresso Brasileiro de Neurologia

TL 1105913 TL 1106479 TL 1105320

NASOPHARYNGEAL POLYP: AN UNUSUAL CANNABIDIOL: NEW PERSPECTIVES FOR PRIMARY CENTRAL NERVOUS SYSTEM
PRESENTATION TREATMENT OF POST-MASTECTOMY PAIN LYMPHOMA: THE CHALLENGES
SYNDROME, A CASE-REPORT OF DIAGNOSTIC WORKUP – CASE
Tiago César Costa da Silva, Herisson Rodrigues de
Oliveira, André Luiz Pinto Fabricio Ribeiro, Marcílio PRESENTATION
Anédia Dourado, Ana Rafaela Soares Vale, Filipe
Ferreira de Paiva Filho, Maurus Marques de Almeida Augusto Silva Leite, Lorena Alves Mendes, Mariana Kaliny Oliveira Peixoto, Paulo Santiago de Morais
Holanda Costa Nascimento Queiroz, Antônio Souza Andrade Brito, Diana Pessoa Silva Freire, Maria Weryca
Filh de Souza Belo Silva, Regina Taís da Silva, Rafael
Universidade Federal da Paraíba, Faculdade de
Medicina. João Pessoa PB, Brazil Santiago Araujo, Manoel Moreira Neto, Joanisson
União Metropolitana de Educação e Cultura.
Hospital Universitário Lauro Wanderley, Rubens Gomes Diniz, Heitor Caetano dos Santos,
Salvador BA, Brazil
Universidade Federal da Paraíba. João Pessoa PB, Mario Emilio Teixeira Dourado Junior
Escola Bahiana de Medicina e Saúde Pública.
Brazil Salvador BA, Brazil UFRN – Natal – RN – Brazil
Universidade Salvador. Salvador BA, Brazil
tiagocesarcs@hotmail.com kalinyop355@gmail.com
Fundação de Neurologia e Neurocirurgia – Instituto
do Cérebro. Salvador BA, Brazil
Case Presentation: J.I.R.B., a 17-year-old male student Case Presentation: A 16-year-old male patient devel-
with a history of pharyngeal pain, nocturnal nasal obstruc- anavale19.2@Bahiana.edu.br oped hypersomnia, dysarthria, left hemiparesis, loss of
tion for 2 months, associated with a complaint of throat sphincter control and seizures for 4 months. He received
swelling for 1 month. He denied dysphagia, rhinorrhea, Case Presentation: J.C.A female, 46 years old, presents acyclovir, followed by pulse therapy with methylprednis-
headache and allergic symptoms. He was not asthmatic. to the neurology outpatient clinic with a complaint of olone, showing clinical improvement. After 2 months, he
Oroscopy revealed a large polypoid lesion protruding from disabling pain in the hemithorax and right upper limbs evolved with cognitive deficit, axial and appendicular
the oropharynx. On dynamic inspection, the soft, but not after mastectomy and worsening with subsequent ipsi- ataxia, and diparesis. He received another pulse therapy,
friable, lesion invaded the oral cavity, occupying almost lateral radiotherapy in 2018. The patient reports that the with no response and showed progressive impairment
the entire oropharynx. Magnetic resonance imaging pain interferes with the quality of her sleep and limits her of ambulation. During the Immunoglobulin infusion,
revealed a bulky lesion with cystic components, hyper- daily activities. It is characterized as arrowheads, without he developed clinical complications. Under intensive
intense on T1-weighted and hypointense on T2-weighted irradiation, constant, but with frequent exacerbations, 10 care, he performed plasmapheresis, with no improve-
images, of approximately 8.0 cm in its largest diameter out of 10 on the pain scale, accompanied by paresthesia ment. Autoimmune markers were negative. The search
on the sagittal section, extending from the nasopharynx and loss of strength in the affected limb. A directed phys- for neoplastic cells in the cerebrospinal fluid (CSF) was
to the oropharynx, reaching the region of the epiglottis. ical examination was not performed because the patient inconclusive. Infectious screening was negative, as was
Endonasal and oral endoscopic resection was indicated, was intolerant to touch and movement. She takes several extraneural neoplastic screening. Spinal resonance was
and the polyp was dissected and removed from the naso- medications, including vitamin B and D, clonazepam, normal and brain resonance showed multiple supra
pharynx, which, because of its size, was removed through dipyrone, pregabalin, amitriptyline, and methadone, and infratentorial lesions, with moderate mass effect,
the oral cavity. The procedure was uneventful during or without improvement. She has a history of intestinal, post-contrast enhancement and moderate water diffusion
after surgery, and he was discharged after 48 hours. The rectal, and breast cancer, the latter still active. She was restriction, with a nodular lesion in the pineal; proton
anatomopathological study revealed an inflammatory diagnosed with post-mastectomy pain syndrome (PMPS), spectroscopy suggested cell proliferation. Brain biopsy
polyp, with intense interstitial edema and an inflammatory maintained on previously prescribed medications, and favored the diagnosis of lymphoproliferative disease.
infiltrate rich in eosinophils, with no signs of malignancy. introduced cannabidiol 250mg per serving. After 7 days Immunohistochemistry described high-grade B-cell lym-
The patient has been followed up for ten months with no using 3 drops a day, the patient reported improvement, phoma of the central nervous system. With the diagnostic
recurrence and no pharyngeal, nasal or oral complaints. with pain 8 out of 10. In the following 7 days he started confirmation, she underwent systemic chemotherapy,
Discussion: Primary non-neoplastic inflammatory polyps taking 7 drops a day with the same concentration, with showing slight clinical and radiological improvement, but
of the nasopharynx are rare, representing 4 to 6% of nasal significant improvement, reporting pain 5 out of 10, it was interrupted by infectious complications, which led
polyps. There is still no evidence about its pathogenesis, which allowed the reduction of the methadone dose. to death. Discussion: Primary central nervous system lym-
but it is believed that both the adaptive and innate immune Discussion: PMPS is a chronic neuropathic pain, result- phoma is a rare and aggressive non-Hodgkin lymphoma
response could be involved. The present case is of great ing from direct injury or scar tissue formation of plexuses that may involve the brain, leptomeninges, spinal cord or
importance because of its rarity, the site of involvement and nerves ipsilateral to the mastectomy, and may be eyes. Approximately 90% are of the diffuse large B-cell lym-
of the benign inflammatory polyp, as well as the size of aggravated by local radiation-based therapies such as phoma type. Congenital or acquired immunodeficiency
the lesion. This rare pathology should be added to the list radiotherapy. Nerve injury results in inflammatory pro- is the main risk factor. In immunocompetent patients,
of differential diagnoses of nasopharyngeal mass lesions cesses that favor ectopic impulses and neuronal hyper- the mean age at diagnosis is 53 to 57 years. Neurological
between nasoangiofibroma and nasopharyngeal carci- excitability, and thus neuropathic pain. Thus, due to its symptoms tend to overlap with systemic ones. Diagnosis
noma. Clinically, there is a benign course of this lesion anti-inflammatory potential and ability to modulate pain requires neuroimaging, CSF analysis and brain biopsy.
despite the extensive volume. Therefore, regarding neu- pathways and dopamine release, which interferes with the Treatment involves radiotherapy and chemotherapy.
rological manifestations, literature confirms that acute response to pain, it is possible to understand the choice of Final Comments: The purpose of this report is to describe
sinus inflammatory damages can possibly result in nerve cannabidiol as a therapeutic measure for the condition, a case of Primary central nervous system lymphoma, a
hypersensitivity Final Comments: In the approach of considered refractory to conventional therapy, as well as rare disease in a young and immunocompetent patient,
nasopharyngeal and oropharyngeal masses in young and the response presented by the patient after years without emphasizing the importance of its recognition among
adult patients, primary inflammatory polyps are among any improvement. Final Comments: In this perspective, the differential diagnoses, and the consequent search for
the diagnoses to be considered. Despite the small num- despite the findings that speak in favor of the therapeutic timely therapy, providing greater survival.
ber of cases, the radiological and pathological features of potential of cannabidiol, it is important to emphasize the
this lesion should be remembered, minimizing the risk need for more studies to deepen the knowledge on the
of being misdiagnosed. subject in question. Educação Médica

Dor Dor

75
XXX Congresso Brasileiro de Neurologia

TL 1105366 TL 1105818 TL 1105047

CENTRAL RETINAL ARTERY OCCLUSION: GROUP ACTIVITIES FOR PARKINSON’S STROKE OR SEIZURE: A CHALLENGING CASE
POINT OF CARE TECHNOLOGY, TELESTROKE PATIENTS IN THE COUNTRYSIDE OF BRAZIL OF PROLONGED TODD PARESIS
AND INDIRECT OPHTHALMOSCOPY – A SUCCESSFUL EXPERIENCE
Ana Letícia Fornari Caprara, Jamir Pitton Rissardo,
TECHNIQUES George Vasconcelos Calheiros de Oliveira Costa,
Tiago Camilo Eisemberg Alvarenga, Patrick Silva
Lucas Bruno Rezende, Beatriz Gioppo Betini, Viana, Isabela Cristina Soares Cordeiro, Deiziani Paulo Gilberto Medeiros Jauris, Fernando von Bock
Fernando Henrique Magri Alves, Patrícia Approbato Cristina Valadares Silva, Juliana Aparecida Braga Bolli, Juliana Oliveira Freitas Silveira
Marques, Lucas Ravagnani da Silva, Eduardo Yukio Cruz
Universidade Federal de Santa Maria. Santa Maria
Nakamura, Jorge Alberto Martins Pentiado Junior RS, Brazil
UNIFUNCESI – Itabira – MG – Brazil
Hospital das Clínicas da Universidade Federal de Hospital Universitário de Santa Maria . Santa Maria
tiagocea@hotmail.com RS, Brazil
Minas Gerais. Belo Horizonte MG, Brazil
Hospital das Clínicas da Faculdade de Medicina julianaofs@yahoo.com.br
Case Presentation: The present work intends to describe
de Ribeirão Preto da Universidade de São Paulo.
the creation and strengthening of an association of patients
Ribeirão Preto SP, Brazil Case Presentation: A 32-y-old male with a history of
with Parkinson disease (PD) in a medium-sized city in
Universidade de São Paulo. São Paulo SP, Brazil remittent epilepsy without current use of antiepileptic
Brazil. In September 2021, it started a partnership with
Hospital São Lucas. Ribeirão Preto SP, Brazil drugs, traumatic brain injury with amaurosis in the left
a local college with 6 patients and the neurologist who
lucasbrunorezende@hotmail.com led the cases. The initial objective of the partnership was eye, smoking, and abstinence from cocaine presented
to encourage the socialization and practice of physical to our ER with left-sided weakness, mild dysarthria and
Case Presentation: An 83-year-old man presented to the activities in those patients, in addition to reducing the loss of nasolabial fold at the left with 30 min of onset. CT
Emergency Department (ED) due to sudden non-painful stigmas that accompany the disease. In May 2022, the scan at the admission presented no signs of bleeding.
left eye monocular vision loss 15h after he was last known group had grown to 50 participants, activities twice a week, His initial NIHSS was 21. He had no contraindications
well. His neurological examination was notable for a left conducted by students from the 9th and 10th periods of to thrombolysis and alteplase was administered without
eye relative afferent pupillary defect and a left eye visual physiotherapy. The strategies are set up with stimuli for complications. After thrombolysis, NIHSS remained the
acuity of light perception at 30 cm with no other focal neu- gait training, cognitive strategies to improve transfers, same. On the next day, the patient remained with hemi-
rological deficits. He was immediately taken to Radiology exercises for balance, mobility training joints and mus- paresis and a motor examination showed weakness on
on stroke code and, during transport, mydriatic drops cle strength to implement physical capacity. Low cost his left side (2/5, Medical Research Council [MRC] scale).
were instilled on his eyes. Approximately 20 mintues after materials used are dumbbells, balls, shin sticks, cones, Brain MRI was performed and showed no alterations.
first contact with Neurology, an indirect ophtalmoscopy walking tracks with obstacles. In addition to routine activ- Transthoracic echocardiogram and doppler ultrasound
done after neuroimaging with a 20 diopter lens and a ities, the group counts on the participation of guests in imaging of the carotids and vertebral arteries were normal.
smartphone camera confirmed a left eye central retinal lectures that explore other aspects of the parkinsonian’s Blood count, autoimmune disease biomarkers, and met-
artery occlusion (CRAO). Discussion: Most Hospitals do life. Discussion: Parkinson disease is a neurodegenerative abolic panel were without alterations. By day 5 of admis-
not have an ophtalmologist on call 24h a day and formal disease characterized by bradykinesia and hypokinesia sion, the patient had an improvement in his symptoms
specialized consultations can result in treatment delays combined with rest tremor and/or rigidity. In addition but remained with weakness on his left side (4/5 on his
in time sensitive situations such as stroke. It is essential to these typical motor features, patients with PD may left leg and 2/5 on his left arm). Prolonged Todd’s pare-
neurologists in EDs are capable of perfoming good qual- experience nonmotor symptoms related to the disease sis was considered and an EEG showed disorganization,
ity fundoscopies. Although extensively advocated by the itself or to the medications used to treat it. Those patients asymmetry, hyporeactivity, and slowing of background
neurology education community as mandatory part of require broad-based management including patient and activity, compatible with mild-moderate toxic-metabolic
training, many neurologists are uncomfortable with the family education, support group services, general wellness encephalopathy, with a background asymmetry possibly
use of direct ophtalmoscopes. In addition, the 15° fundus maintenance, exercise, and nutrition. Education is essen- related to a post-ictal seizure state. On day 11 of admis-
view amplitude of direct ophtalmoscopy is very useful tial in order to provide the patient and family with some sion, the patient was asymptomatic and was started on
for optic disc pathology detection, but could potentially understanding and control over the disorder. Education valproic acid 250 mg t.i.d., discharged home, and referred
miss CRAO, in which optic discs are spared. Our proposed can combat the stigma and misinformation that often sur- to the Neurology outpatient clinic. Discussion: Todd
technique circumvents these drawbacks with a much round the disorder. Regular exercise is very important in paresis, also known as Todd paralysis or postictal hemi-
wider, 360° view of the eye fundus, without adding a sig- those patients, because it promotes a feeling of physical paresis, was initially described in 1849 and is associated
nificant amount of time to the care of patients on acute and mental wellbeing. Exercise may help to slow motor with weakness or paralysis in part or all of the body after
stroke codes, since mydriatic drops can be used during progression, improve nonmotor symptoms, and alleviate a seizure. Since stroke is the main differential diagnosis
the neurological exam or while transporting patients to some secondary orthopedic effects of rigidity and flexed in patients presenting with weakness in the ER, this may
imaging. Furthermore, when the on call neurologist is posture. Patients are at risk for social isolation, depres- lead to misdiagnosis and unnecessary treatments. In fact,
not sure of the correct interpretation of the eye fundus, sion is one of the most common psychiatric disturbances in one series, this was the most common non-stroke cause
images can be sent via secure instataneous communi- seen. Final Comments: With a low-value investment, it is of referral to a stroke unit. However, Todd paresis tends to
cantion channels, such as mobile phone applications, possible to offer quality of life and dignity to patients with be a regressive deficit with symptoms that do not exceed
for discussion with fellow and more experienced col- Parkinson’s Disease in medium-sized cities. We believe 36h, and only rarely do symptoms last beyond this period
leagues, ensuring tailored care delivery as in telestroke. that centers like this one should be stimulated by the of time. Final Comments: In this way, this is a crucial dif-
As limitations of our work, we emphasize some patients interior of Brazil, improving the quality of life of patients ferential diagnosis in the ER and a thorough evaluation
have absolute contra-indications to the use of mydriatic and allowing higher quality studies by uniting patients. involving medical history, physical examination, clinical
drops, the 15 minute delay it takes for pupillary dilation, parameters, neuroimaging, and EEG is often necessary
the price of indirect ophtalmoscopy lens and the need to establish the diagnosis, particularly when atypical
for staff training.Final Comments: CRAO diagnosis can Educação Médica presentations such as prolonged Todd paresis take place.
only be established by good eye fundus exam. Indirect
ophtalmoscopy with smarthphone camera is a power-
Epilepsia
ful mean of rapidly making this diagnosis in the ED and
broadening access to emergency stroke reperfusion ther-
apy in select eligible patients.

Educação Médica

76
XXX Congresso Brasileiro de Neurologia

TL 1105087 TL 1105088 TL 1105090

STATUS EPILEPTICUS IN A PATIENT TUBEROUS SCLEROSIS OR BOURNEVILLE- LEVETIRACETAM-INDUCED RENAL FAILURE:


WITH ANOREXIA NERVOSA-RELATED PRINGLE DISEASE: CASE SERIES AND CASE PRESENTATION AND LITERATURE
WERNICKE-KORSAKOFF SYNDROME: CASE LITERATURE REVIEW REVIEW
PRESENTATION
Paula Köhler Carpilovsky, Fabio Pascotto de Oliveira, Paula Köhler Carpilovisky, Fernando von Bock Bolli,
Mariusi Glasenapp dos Santos, Maria Eduarda Fernando von Bock Bolli, George Vasconcelos Daniel Melchiades da Silva, George Vasconcelos
Mercado Vargas, George Vasconcelos Calheiros Calheiros de Oliveira Costa, Paulo Gilberto Medeiros Calheiros de Oliveira Costa, Juliana Oliveira Freitas
de Oliveira Costa, Paulo Gilberto Medeiros Jauris, Jauris, Juliana Oliveira Freitas Silveira Silveira
Fernando von Bock Bolli, Juliana Oliveira Freitas
Universidade Franciscana . Santa Maria RS, Brazil Universidade Franciscana . Santa Maria RS, Brazil
Silveira
Hospital Universitário de Santa Maria . Santa Maria Hospital Universitário de Santa Maria . Santa Maria
Universidade Federal de Santa Maria. Santa Maria RS, Brazil RS, Brazil
RS, Brazil Hospital de Caridade Astrogildo de Azevedo . Santa
julianaofs@yahoo.com.br Maria RS, Brazil
Hospital Universitário de Santa Maria . Santa Maria
RS, Brazil
Case Presentation: CASE 1: A 58-y-old male with intellec- julianaofs@yahoo.com.br
julianaofs@yahoo.com.br tual disability, epilepsy, renal angiomyolipomas, dupuy-
tren contracture in hands, facial angiofibromas and ungual Case Presentation: A 24-y-old male patient with a history
Case Presentation: A 42-year-old female underwent fibroma in the nail matrix. CT scan showed cortical atrophy of epilepsy since the age of 16, in regular use of antiepi-
exploratory laparotomy due to acute intestinal obstruction, and periventricular calcifications (calcified tuberes). EEG leptic drugs, was brought to our ER due to a new seizure.
and nineteen days later lost consciousness. She had been with epileptiform activity in anterior regions bilaterally. He had been using oxcarbazepine since the beginning of
severely depressed and anorexic for over four months by CASE 2: A 33-y-old female with epilepsy, renal angio- treatment. The EEG showed frequent slow wave paroxysms
that time. There was no report of previous seizures, other myolipomas and facial angiofibromas. Her grandfather, and slow wave peaks of 3-6 Hz. Levetiracetam had been
neurological disorders, smoking or regular alcohol use. father and cousin had the same signs and symptoms. recently added with good seizure control. Normal neuro-
Physical examination revealed pupillary hippus, vertical Brain MRI showed subcortical tubers in both cerebral logical exam. Brain MRI showed no changes. Laboratory
and horizontal nistagmus, tachyarrhythmia, irregular hemispheres and a small subependymal nodule. The tests showed high levels of creatinine and he progressed to
breathing pattern and bilateral focal neurological signs genetic testing showed alteration in the TSC2 gene. CASE acute renal failure. During Hospitalization, a renal biopsy
– particularly on the left – findings suggestive of focal 3: A 35-y-old male with epilepsy, intellectual disability, was performed, which showed acute tubular nephritis.
status epilepticus, which was successfully treated with IV facial angiofibromas and hypopigmented macules on Levetiracetam was then discontinued and valproic acid
midazolam. The hypothesis of status epilepticus compli- the back. CT scan showed two cortical hamartomas and started, with progressive improvement in renal function.
cating Wernicke-korsakoff syndrome (WKS) was raised. subependymal nodules. CASE 4: A 36-y-old male with He was discharged from Hospital after a few days and with-
MRI displayed several T2/Flair hyperintense areas and epilepsy, intellectual disability, facial angiofibromas, out new epileptic seizures. Discussion: Levetiracetam is
“faint” hyperintense DWI areas in the mammilary bod- shagreen patches and hypopigmented macules on the a broad-spectrum anticonvulsant, being one of the most
ies, thalamus, parieto-occipital zones and cerebellum, as back. Discussion: Tuberous Sclerosis (TS), also known as used drugs in its class. This drug has minimal pharmaco-
well as gyriform oedema within the telencephalic sites. Bourneville-Pringle disease, a rare autosomal dominant kinetic interactions due to the fact that it does not bind to
In addition, a medium right-sided parieto-occipital area phacomatosis or cutaneous syndromes, was first describe plasma proteins and is independent of the cytochrome
of DWI hyperintensity (with corresponding ADC map in 1862 by von Recklinghausen. Posteriorly, Bourneville p450 system. Thus, its main side effects, already reported
hypointensity) was found. The radiological findings were and Pringle used the term Tuberous Sclerosis. In 1905, in the literature, are dizziness, headache, asthenia, drows-
suggestive of peri-ictal epileptic state and WKS + sec- Vogt characterized the clinical triad of mental retar- iness and rarely adverse behavioral effects. However, this
ondary parieto-occipital stroke. EEG, performed three dation, seizures and characteristics skin lesions. TS is anticonvulsant, due to its water-soluble properties and
days after seizure control was obtained with phenytoin originates from mutations in the TSC1 and TSC2 tumor low molecular weight, is largely eliminated by the kid-
and levetiracetam, was compatible with toxic-metabolic suppressor genes located on chromosomes 9q34 and neys and may, although rarely, cause repercussions in
encephalopathy and focal slowing on the right posterior 16p13.3, respectively. The TSC1 gene is responsible for this system. There are still few cases relating the use of
cerebral quadrant due to stroke. Discussion: WKS is a encoding a protein called hamartin and TSC2 for tuberin. levetiracetam and acute renal failure, with the first case
disorder caused by thiamine defficiency, usually related TS affects both genders equally with a great diversity of described being that of a 17-year-old girl who developed
to binge alcohol drinking, or, alternatively, to anorexia manifestations (cutaneous, renal, pulmonary, cardiac, acute renal failure and interstitial nephritis ten days after
nervosa, hepatic disfunction, hyperthyroidism or hyper- and neurological). The diagnosis is basically clinical. All starting treatment with the drug. Thus, the case described
emesis gravidarum. The recommended daily thiamine patients described have epilepsy, the main neurological in this work is one of the few described in the literature,
intake is supplied by a normal healthy diet. However, manifestation of TS, occurring in up to 90% of the cases, and only one of the three existing cases found involving
malnourished people undergoing medium-large surger- with seizures usually starting in the first three years of life. a male patient. As observed by previous descriptions, the
ies (which demand a good storage of B1), are especially All patients have renal angiomyolipomas which usually age range is variable, having affected patients from 17 to
prone to this condition. Nevertheless, associated focal affects about 80% of patients. All of them presented facial 45 years old. Final Comments: The association of renal
cortical lesions are rather rare, and among these cases angiofibromas and also cortical tubers. Although genetic failure and levetiracetam is rare, being of fundamental
70% are non-alcohol-related and 30% present seizures testing was performed in only one patient, the diagno- importance to recognize it for a better approach. In addi-
at some point of clinical follow-up. Final Comments: sis can be made in the other cases based on the clinical tion, it is worth mentioning that the use of this medica-
we have reported an atypical evolution of anorexia ner- and radiological presentation. Complementary exams, tion, like any other, must be well evaluated, taking into
vosa-related WKS, which led to permanent multifocal like CT and MRI, are important to detect cortical tuber- account all possible risks, benefits and adverse effects.
encephalic damage following status epilepticus. Although osities. Final Comments: TS is a rare disease. Its early
its atypical course here, WKS should be considered – as a recognition is of great importance for a better treatment
standard of care – in every single patient at the emergency and follow-up, with multidisciplinary team. Epilepsia
room with reduced conscious level. WKS must always be
a “don’t miss” diagnosis, and, as soon as possible, treated
with thiamine. Therefore, the burden of permanent brain Epilepsia
damage is usually minimal.

Epilepsia

77
XXX Congresso Brasileiro de Neurologia

TL 1105321 TL 1105555 TL 1105767

DUPUYTREN’S CONTRACTURE SECONDARY FEBRILE INFECTION-RELATED EPILEPSY CORTICAL DYSPLASIA AND ANTI-GAD
TO CHRONIC USE OF PHENOBARBITAL: CASE SYNDROME (FIRES) AS A CAUSE OF SUPER- ENCEPHALITIS IN A PATIENT WITH
PRESENTATION AND LITERATURE REVIEW REFRACTORY STATUS EPILEPTICUS: A CASE REFRACTORY MESIAL TEMPORAL LOBE
PRESENTATION EPILEPSY: CASE PRESENTATION
Sarah Cerillo Lopes, Amélia Limongi Zambon, Ana
Claudia Piccolo Jéssica Daniele Marques, Giovana Barros e Silva Ana Clara Guilherme Rodrigues, Sheila Ferreira
Ribeiro, Aline de Fátima Dias, Alexandra Seide Pessoa, Thainá Gonçalves Tolentino de Figueiredo,
Universidade Municipal de São Caetano do Sul –
Cardoso, Warlisson Fonseca Pinheiro, Raisa do Val Anselmo de Jesus Santos, Laís Maria Gomes de
São Caetano do Sul – SP – Brazil
Roso, Tania Aparecida Marchiori de Oliveira Cardoso, Brito Ventura, Victor Mascarenhas de Andrade
xsarahcerillo@gmail.com Clarissa Lin Yasuda, Fernando Cendes Souza, Aroldo Luiz da Silva Bacellar

Universidade Estadual de Campinas. Campinas SP, Hospital São Rafael. Salvador BA, Brazil
Case Presentation: Male, Caucasian, 65 years old, retired
Brazil
(rural area resident), attended the neurology outpatient clariguilherme@gmail.com
clinic in October 2021 and showing pain and difficulty jessica.dani.marques@gmail.com
moving the fingers of both hands. He observed nodules Case Presentation: Female patient, with type 1 DM
and hardened tendons, with progressive installation in Case Presentation: A previously healthy 14-year-old and hypothyroidism, with non-perceptive seizures since
the last 3 years, with worsening in the last 6 months. He female was brought to the emergency department with 14 years old. Deja Vu aura, oro-masticatory and motor
had epilepsy for 26 years: focal motor seizures with bilat- focal to bilateral tonic-clonic seizures that started abruptly automatism were described. She used to have 3-6 epi-
eralization, and focal impaired seizures, with epilepsy at the day of admission. There was a report of odynophagia, sodes/day. An investigation was performed with brain
secondary to neurocysticercosis treated with praziquantel headache and fever over the past five days. She persisted MRI, evidencing right mesial temporal cortical dysplasia,
in the year 1996, with subsequent ischemic stroke in the with recurrent seizures despite first-line treatment, as well and video-EEG with right temporal ictal semiology but
year 2000, and status epilepticus, secondary to middle as continuous infusion of sedatives. After therapeutic left temporal electrographic onset. Seizures were refrac-
cerebral artery vasculitis due to active perivascular cys- optimization, including the administration of six classes tory to treatment with 1200mg/day carbamazepine. PET
ticercosis, treated with Prednisone. The patient evolved of antiepileptic drugs, the patient maintained recurrence compatible with bilateral temporal hypometabolism,
with control of epileptic seizures through the use of pheno- of seizures. The electroencephalogram (EEG) showed a worse on the right, neuropsychological evaluation with
barbital 200 mg/ day and carbamazepine 600 /day in the severe disturbance of the background activity, with a visuospatial difficulty. Given the suspicion of Cortical
last 22 years. Examination of hands: palmar contracture, burst-suppression pattern. After a through clinical and Dysplasia, a surgical approach was chosen, with a right
passive extension deficits of the 3 fingers (2º, 3º e 4º) with laboratory evaluation, the hypothesis of an autoimmune temporal lobectomy performed. Pathology compatible
retraction and fibrosis of the flexor tendons and nodules. encephalitis was raised, and the patient was started on with type IB cortical dysplasia. After surgery, she was
Abdominal ultrasound examinations were performed, in immunosuppressive steroid therapy (methylpredniso- seizure-free and drug-free for 2 years, when she had a
addition broad laboratory evaluation, and they were all lone). The clinical response was satisfactory, however, recurrence of seizures with the same ictal pattern. Even
normal, excluding secondary causes such as collagen dis- after the third day of therapy, the infusion was interrupted after adjusting the antiepileptic medications, there was no
eases, and additional family’s history of Dupuytre’s (CD) due to a severe respiratory infection. Following the inter- satisfactory control of the seizures. In view of the endocrine
contracture, being defined a diagnosis of CD secondary ruption of therapy, a deterioration of the EEG pattern was disease, the possibility of associated anti-GAD encepha-
to the use of phenobarbital. Discussion: Dupuytren’s observed, as well as clinical recurrence of focal seizures, litis was raised, with documented antibody positivity in
contracture (CD) is a fibroproliferative condition involv- requiring an increase of the intravenous sedatives. Given the CSF, in addition to the raising of serum titers in the
ing the superficial palmar fascia, leading to a progressive the respiratory infection, intravenous immunoglobu- period seizure recurrence. Immunotherapy with IVIG was
and irreversible flexion of the fingers. Dupuytren’s (CD) lin was administered (total dose of 2g/Kg). The patient started for 5 days in 2021 with marked seizure frequency
contracture might have a higher incidence in patients remained without recurrence of clinical seizures, but reduction. Discussion: Active neuroinflammation and
with epilepsy. In literature, there are different opinions EEG worsening was maintained. Therefore, barbiturate cell injury seems to play a common pathogenic role in
regarding the phenobarbital, a common antiepileptic coma with thiopental was started, with no satisfactory epilepsies. The increased prevalence of autoantibodies
drug, and its effective role in the genesis and development response for 48 hours. After adequate treatment of the in epilepsy reinforces that immune dysregulation partic-
of CD. We believe that phenobarbital causes a dose and infection, a second immunosuppressive steroid therapy ipates in this process. As in temporal lobe epilepsy and
time-dipendent profibrotic effect. The CD’s aetiology of was started (methylprednisolone 1g/day for five days). cortical dysplasias, in immune-mediated encephalitis,
some aspects is still unclear. Many factors are already The patient, then, presented clinical control of seizures there is microglial activation, providing evidence of an
known to be implicated, and there are different opinions and improvement of the electroencephalographic pattern, immune response, in addition to astroglial activation,
regarding the association with the phenobarbital used for with gradual reduction of sedatives. Discussion: Status which accompanies the progression of cortical damage
the antiepileptic treatment, and its effective role in the epilepticus in previously healthy individuals is known as and probably participates in the inflammatory process.
genesis and development of Dupuytren’s contracture. new-onset refractory status epilepticus (NORSE). When Among patients with epilepsy, 2.58% of them had a pos-
Final Comments: Our case presentation demonstrate it affects young patients with a history of febrile infection itive anti-GAD antibody. The correlation between focal
that phenobarbital cause a dose and time-dipendent preceding the seizure by two weeks to twenty-four hours, dysplasia and anti-GAD encephalitis seems to raise the
profibrotic effect and must to be involved in the genesis the condition is known as febrile infection-related epi- idea that an autoimmune response caused by dysmorphic
of Dupuytren’s contracture. Progressive withdrawal of lepsy syndrome (FIRES). It is a rare condition, with an neurons contributes to the pathogenesis of the intense
phenobarbital and introduction of Valproate was per- estimated incidence of one per million. Final Comments: inflammatory response. Outcome: Epilepsies that have
formed, and he was referred for surgical treatment by The case presented demonstrates the difficulty of clinical autoimmunity as an etiology go beyond this spectrum
an orthopedist. management of FIRES, due to the high refractoriness to and it is increasingly important to recognize them as
standardized treatments for status epilepticus. potentially treatable with immune modulation. Even
with scarce evidence, patients with focal epilepsies and
Epilepsia autoantibodies may benefit from immunotherapy even
Epilepsia after surgical intervention.

Epilepsia

78
XXX Congresso Brasileiro de Neurologia

TL 1106064 TL 1106106 TL 1106111

THE ELECTROENCEPHALOGRAM IN THE FOCAL AWARE STATUS EPILEPTICUS ATYPICAL ROLANDIC EPILEPSY AND
RESEARCH OF PROGRESSIVE MYOCLONIC IN PATIENT WITH STROKE-LIKE LANGUAGE DELAY ASSOCIATED WITH
EPILEPSY MIGRAINE ATTACKS AFTER RADIATION NR4A2 AND GRIN2A
THERAPY (SMART) SYNDROME: A CASE
Tatheane Couto de Vasconcelos, Angela Rodrigues Julieta Sobreira Goes, Marielza Fernandez Veiga,
Gifoni, Ana Karoline da Costa Monteiro, Mariana PRESENTATION Tatiane Chagas
Carvalho Vasconcelos, Norma Martins de Menezes Julia Loripe Guimaraes, Guilherme Carvalhal Ribas,
Morais, Samir Camara Magalhaes, Fernanda Paiva UNEB – Salvador – CE – Brazil
Luis Otavio Caboclo CLININ. Salvador BA, Brazil
Pereira Honorio
Faculdade Israelita de Ciências da Saúde Albert ESTIMULOS. Salvador BA, Brazil
Universidade Federal do Ceará. Fortaleza CE, Brazil Einstein, São Paulo SP, Brazil julieta.goes@gmail.com
tatheanecv@gmail.com Hospital Israelita Albert Einstein, São Paulo SP,
Brazil Case Presentation: M.A.B, female, 4 years old, normal
Case Presentation: A previously healthy 8-year-old luisotaviocaboclo@gmail.com pregnancy and delivery, non-consanguineous parents. In
patient, with adequate neuropsychomotor development, the first year of life, she had motor development delay, and
started a generalized tonic-clonic crisis (TCGC), afebrile, Case Presentation: A 56-year-old woman was admitted at 2 years old, her language was incomprehensible, and
during sleep in October 2021, associated with the presence with a three-day history of complex visual hallucinations. presented inappropriate social behaviors; At age 3, she
of sudden movements contraction of limbs, described as She had previous history of right temporo-occipital grade started with focal seizures during sleep. Dysmorphic fea-
“shocks”. He underwent a cranial tomography without III ependymoma, diagnosed 10 years before, and treated tures were absent; Good social interaction, but impaired
alterations, remained asymptomatic for two weeks. He with surgery and radiotherapy. Repeated neuroimaging attention; Low expressive linguistic with predominantly
evolved with weekly frequency TCG crisis, with sudden exams showed no signs of tumor recurrence. She reported gestural/verbal speech. Initiates a conversation, as it
movements (myoclonus) daily and started to present neither epileptic seizures nor use of antiseizure med- presents difficulties in understanding. The orofacial struc-
behavioral changes. Two months after the onset of the ications (ASM). On admission, she reported recurrent tures presented low posture, tone, mobility of the lips and
condition, the patient showed gait and speech distur- episodes of complex visual hallucinations, described as cheeks; First EEG: independent projection epileptiform
bances and had daily epileptic seizures. Neurological altered depth perception and image distortion, associated activity in the centrotemporal regions activated by sleep;
examination: fluctuating alertness, expression aphasia, with left lateral gaze deviation, without impairment of The use of oxcarbazepine, but the attacks increased in
slow gaze tracking. Myoclonus on the face, incomplete consciousness. Episodes occurred several times a day and frequency; Control EEG: frequent epileptiform activity
trunk support. Limb coordination: dyskinesia.Balance/ lasted up to an hour. On admission, neurological exam- and associated with slowing of baseline activity in the left
gait: gait with a support, discreetly dyskinetic and on ination revealed left homonymous hemianopsia, with no cerebral hemisphere. MRI normal; Clobazam was associ-
tiptoe. Exams: CSF: clear;0 leukocytes;2 red blood cells; other abnormalities. Magnetic resonance imaging (MRI) ated with oxcarbazepine, with no improvement in the sei-
proteins 26; Cranial MRI: multifocal signal alteration of the head demonstrated cortical gyriform enhancement zures and later levetiracetam, but the seizures continued
affecting the subcortical white matter of both cerebral surrounding the surgical site in right temporo-parie- to occur sporadically. The last seizure was 4 months ago.
hemispheres, without cytotoxic edema or intramyelinic to-occipital region, virtually pathognomonic of stroke- Genetic analysis by next-generation sequencing identi-
edema. EEG: abnormal, due to diffuse disorganization like migraine attacks after radiation therapy (SMART) fied: In heterozygosity, in the NR4A2 gene, a variant pro-
of sleep activity, with asymmetry, worse in the left hemi- syndrome. Continuous electroencephalogram (cEEG) moting the substitution of the amino acid isoleucine. The
sphere. Generalized, polyphasic, high-amplitude periodic monitoring showed repeated focal epileptic seizures, NR4A2 gene encodes a transcription factor that is highly
activity at 3- to 6-second intervals. After the EEG result, with ictal onset over posterior regions of the right hemi- expressed in critical regions for language development
the hypothesis of subacute sclerosing panencephalitis due sphere. Clinically, the patient referred visual symptoms; and the normal formation of the dopaminergic pathway.
to measles (characteristic clinical picture, typical EEG) in some of the seizures, she had eye and head deviation Autosomal dominant inheritance. In heterozygosity in
was made, and a new CSF puncture showed negative to the left. Seizures lasted up to 77 minutes, leading to the GRIN2A gene, a variant that promotes the substitu-
immunology for measles IgM and IgG 1: 32 and protein diagnosis of focal status epilepticus (SE) without impair- tion of the amino acid valine for leucine; The treatment
electropheresis with the presence of multiple bands in the ment of consciousness. She was treated with intravenous was modified, for a gradual withdrawal of oxcarbaze-
gamma region (81.3%). Discussion: Subacute sclerosing lacosamide plus oral clobazam, which led to seizure ces- pine and maintenance of levetiracetam and clobazam,
panencephalitis (SSPE) is a progressive and fatal neuro- sation. MRI was repeated one month later and showed without new crises.
degenerative encephalitis caused by the persistence of complete resolution of previous findings. A new EEG
measles virus in the central nervous system (CNS). In the demonstrated absence of epileptiform abnormalities.
state of Ceará, there were 916 confirmed cases of measles Epilepsia
Discussion: SMART syndrome is a rare reversible syn-
between December 2013 and September 2015. The only drome that occurs as a late effect of brain radiotherapy.
concrete strategy against measles and its complications is Classical features include migraine-like headaches, focal
prevention through vaccination. The COVID-19 pandemic neurologic signs and/or seizures, associated with typical
has left a dangerous immunity gap due to the suspension imaging findings. Episodes last days to weeks and usually
of measles awareness campaigns and delay in immuniza- occur years or decades after radiotherapy. We report a case
tion, leading to medical concern about the risk of further of a patient with SMART syndrome who presented with
measles outbreaks in the coming years. Final Comments: focal aware SE with visual symptoms, which is an atyp-
In this case, we highlight the electroencephalogram as a ical presentation of this syndrome. After treatment with
fundamental exam for the diagnosis, showing the periodic ASM symptoms regressed, as well as MRI abnormalities,
complexes with a long interval, thus directing the inves- demonstrating the reversible character of the syndrome.
tigation in the CSF to confirm the pathology. Final Comments: Albeit rare, SMART syndrome should be
considered in patients with history of brain radiotherapy
and typical clinical and neuroimaging findings.
Epilepsia

Epilepsia

79
XXX Congresso Brasileiro de Neurologia

TL 1106471 TL 1106589 TL 1104966

PHENYTOIN-INDUCED RHABDOMYOLYSIS TRANSVERSE MYELITIS ASSOCIATED NEUROLOGICAL ALTERATIONS RELATED


WITH NEUROLISTERIOSIS: A CASE-BASED TO VITAMIN B12 DEFICIENCY: A CASE
Davi Bravo Huguinim Légora, Alexandre Venturi,
Matheus Gonçalves Maia, Diogo Haddad Santos, UPDATE PRESENTATION
Yngrid Dieguez Ferreira, Emerson Gisoldi, Sophia Matheus Brasil Câmara Monteiro, Luis Eduardo Arthur Batista de Freitas, Moacir Pereira Leite Neto,
Bravo Huguinim Légora Oliveira Matos, Paulo Roberto Matos Neto, André Joyce Maria Leite e Silva, Isabel Monique Leite
Irmandade Santa Casa de Misericódia de São Paulo, Felipe Oliveira Fernandes, José Anchieta Rodrigues Romualdo, Susyane Ribeiro Beserra
São Paulo SP, Brazil Filho, David Elison Lima Silva, Regina Coeli Carvalho
Porto Carneiro, Espartaco Moraes Lima Ribeiro, Universidade Federal do Cariri. Barbalha CE, Brazil
Irmandade da Santa Casa de Misericórdia de São Universidade Regional do CaririIguatu CE, Brazil
Paulo, São Paulo SP, Brazil Paulo Roberto Lacerda Leal
Instituto Centro de Ensino Tecnológico. Campos
Multivix. Cachoeiro de Itapemirim ES, Brazil Universidade Estadual do Ceará. Sobral CE, Brazil Sales CE. Brazil
davibravohl2@gmail.com Instituto Federal de Educação, Ciência e Tecnologia
matheus.monteiro987@gmail.com do Ceará – Crato – CE – Brazil
Case Presentation: A 41-year-old male patient, previously Case Presentation: A 68-year-old man was admitted with arthur.freitas@aluno.ufca.edu.br
hypertensive and diabetic, presented a sudden onset of a case of suspected chronic lymphocytic leukemia, due
two reentrant generalized tonic-clonic seizures, with- to leukocytosis with a lymphocytic predominance. He Case Presentation: A 56-year-old male patient, in
out regaining consciousness between them, with a total started investigation for further treatment in 2020, los- February 2012, presented a neurological condition show-
duration of about 10 minutes. In the initial evaluation in ing follow-up in the pandemic. 2 years after, the patient ing irritability, paranoid delusions, evocation amnesia
the emergency room, was in a typical post-ictal period, reported two episodes of falls. In the first, he reported a and apraxia when dressing and performing personal
and initial assistance was provided and a loading dose fall from a “water truck”, with pain and swelling in the hygiene. Simultaneously, paresthesia and dysbasia in the
of phenytoin with 20 mg/kg, was performed, and later left knee, but without trauma or excoriation. A week after lower limbs began due to imbalances of insidious onset.
maintained medication at a dose of 100 mg every eight this event, he fell off the hammock reporting severe pain On examination, cognitive screening tests were initially
hours. In laboratory tests on admission, the patient had in the lumbar region, being medicated, but always with unrealisable, and there was diffuse arreflexia, hypoesthesia
Creatine phosphokinase (CK) of 311 U/L, creatinine 1.3 the pain returning after the medication ceased its effects. in “boot” and ataxic gait. Laboratory evaluation demon-
mg/dL. During Hospitalization, an acute curve of CpK From the second fall, on the following day, the condition strated normal blood count, electrolytes, transaminases,
elevation was observed over the days, withpeak observed evolved with paresis and hypoesthesia in the left lower nitrogen scores, folic acid, ESR and blood glucose levels,
on the fourth day of Hospitalization, in association with limb. On this same day, evolving with involvement in TSH 12.99 mUI/L, free T4 0.87 ng/dL, VDRL and anti-
Acute Kidney Injury (AKI), with creatinine 5.7 mg/dL. In the right lower limb. He was referred to the reference HIV 1 and 2 not reactive, serum vitamin B12 50.0 pg/ml.
view of the expressive increase, in large proportion, in the Hospital in the region, already with a condition of grade Magnetic resonance imaging of the skull showed a small
context of a stable patient, without new crises, and with- 1 paraplegia and loss of sensitivity at the T10-T11 level. cystic lesion in the right basal ganglia and mild leukoara-
out complaints, a hypothesis of association was raised For investigation of the condition, a lumbar puncture was iosis. The patient started parenteral use of a compound
the causal link between the rhabdomyolysis condition performed, the analysis of cerebrospinal fluid showed a of B complex vitamins – 5000 U, intramuscularly, once a
and the administration of phenytoin, with the drug being yellow and turbid aspect. Then, antimicrobial treatment week, for three months, associated with quetiapine, 50mg/
changed to Levetiracetam on fourth day of Hospitalization was performed with subsequent confirmation of infection day, and evolved with memory, behavioral disorders and
(CK 20199 U/L). After this event, an immediate progres- by Listeria monocytogenes. After imaging tests, myelitis balance improvement as well as functional autonomy,
sive drop in serum creatine phosphokinase levels was was suspected, which was associated with the bacte- however remaining with a slight sensation of numbness
observed, so that and 24h after phenytoin suspension rial infection. Discussion: Listeria monocytogenes is a in the feet. Discussion: Vitamin B12 deficiency usually
went to 14000. Discussion: The causal association between gram-positive bacteria commonly transmitted by inges- causes megaloblastic anemia, which may be absent in
phenytoin and rhabdomyolysis is considered rare, its first tion of poorly washed or undercooked food, via the fecal- one third of cases, associated with neurological symp-
report being in 1976. In some cases, the manifestation and oral route. This type of bacteria has a certain affinity with toms, due to vascular alterations associated with the
in a context of hypersensitivity syndrome, with other clin- the central nervous system, affecting mostly neonates, elevation of homocysteine, or alterations in methylation
ical and laboratory findings such as eosinophilia and skin pregnant women, elderly, and immunocompromised reactions, affecting myelin. Such alterations compromise
rash, however, there are reports as being the only clinical patients. Neurolisteriosis can occur in two ways, through axons of the ascending tracts of the posterior funiculus,
manifestation, still considered with uncertain patho- a retrograde neural (associated with rhombencephalitis) descending pyramidal tracts and peripheral nerves. The
physiology. In these cases, the diagnosis is a challenge, or a hematogenous route (resulting in meningoencepha- treatment of the disease is carried out through parenteral
because rhabdomyolysis is a frequent finding in seizures. litis). Neurolisteriosis is an uncommon occurrence, but replacement of the vitamin, with a maximum improve-
However, in view of being a complication often associated its association with myelitis resulting in a spinal cord ment generally occurring in the first six months, and its
with acute kidney injury, it is important to evaluate dif- syndrome is rare, even in cases with risk factors, such as early initiation is of great importance to avoid possible
ferential diagnoses that require rapid management. Final possible immunosuppression caused by leukemia in the neurological sequelae. Final Comments: This scenario,
Comments: In this context, attention should be paid to present case, with meningeal involvement being more consisting of an amnesic and behavioral, apraxia, ataxia
this little-known adverse effect of the drug. Thus, acute common. Final Comments: Neurolisteriosis is a rare and sensory cognitive syndrome, representing subacute
elevations to unusual CK values, progressive worsening event which may be associated with myelitis, and such an dementia, sensory ataxia and sensory polyneuropathy,
of AKI, and refractoriness to clinical measures to treat association should be suspected, especially in patients at has, in many cases, a secondary relation to vitamin B12
the condition should be considered warning signs for risk for infection. Its treatment should be initiated quickly, deficiency, based on its serum dosage, with the possibility
phenytoin-induced rhabdomyolysis. Since the response due to its high mortality rate. of associated problems, such as the existence of a subclini-
in these cases was observed soon after drug withdrawal, cal hypothyroidism, which was also verified in the patient.
as seen in the case in question.
Líquido cefalorraquiano
Miscelânea
Epilepsia

80
XXX Congresso Brasileiro de Neurologia

TL 1104970 TL 1104978 TL 1104995

INADVERTENT INTRATHECAL TRANEXAMIC EXTENSIVE LEUKOENCEPHALOPATHY FROM OTOLARYNGOLOGY TO


ACID ADMINISTRATION: A MISTAKE WITH SECONDARY TO VITAMIN B12 DEFICIENCY: HAEMATOLOGY: A BOND CHAINED BY
POTENTIAL CATASTROPHIC OUTCOME CASE PRESENTATION NEUROLOGY
Matheus Compart Hemerly, Anna Leticia de Pedro Thiago Simoes Ferreira, João Vitor Nunes João Vitor Ribeiro dos Santos, Thamilee Pizzatto,
Moraes Alves, Larissa Peres Delgado, Ellen Silva de Sobreira Cruz, Juliana Oliveira de Almeida Almeida, Abraao Iuri Medeiros Angelim, Mariana Spitz,
Carvalho, Natalia de Oliveira Silva, Andressa Gomes Allef Roberto Gomes Bezerra, Kirsten Araújo Melo, Marcelo Cagy, Jamison Menezes de Souza, Ana
Niederauer, Fabiola Dach Alice Cavalcante Almeida Lins, Bruna Acioly Leão, Carolina Andorinho de Freitas Ferreira
Nayra Roberta Sales Salvador, Fernando Tenório
Hospital das Clínicas da Faculdade de Medicina de Gameleira, Patrícia Pereira Nunes Hospital Federal dos Servidores do Estado. Rio de
Ribeirão Preto. Ribeirão Preto SP, Brazil Janeiro RJ, Brazil
HGE. Maceió AL, Brazil
matheuscompart@hotmail.com jvriber@gmail.com
pedro_thiago22@hotmail.com
Case Presentation: A 20-years-old pregnant woman was Case Presentation: A 49-year-old man presenting a grow-
submitted to spinal anesthesia for an elective cesarean Case Presentation: A 43-year-old male patient with a ing left neck mass was under etietiological investigation
procedure at another Hospital. She referred no anesthetic history of sudden cervical pain irradiating to the lower by otorhinolaryngology for two months before he became
effect after administration of 15 mg bupivacaine, so an limbs followed by paraplegia 11 years before, with a slight bedridden. Upon neurological evaluation, he had upper
additional dose of 15 mg was used. The procedure was fin- improvement of motor déficit, remained restricted to bed limbs paralysis, lower limbs paresis, urinary retention
ished as usual, however, about 30 minutes later, the patient for 2 years, when he presented spontaneous recovery of and sensitive level at T10 dermatome. Diagnostic work
presented seizures refractory to the first approach with functionality, without complaints for the following 2 years up disclosed chronic anemia and a hypodense cervical
diazepam, and progressed to clinical status epilepticus when he acutely presented symmetrical paraparesis with mass associated to bilateral pleural thickening and left
(SE). There was no history of headache or hypertension episodes of daily kinesiogenic painful dystonia in the pleural effusion on chest CT. The Queckenstedt’s manoeu-
during the pregnancy. Her prenatal was unremarkable. lower limbs with clonus, associated to paresthesia with vre was positive for spinal block and the CSF analysis
After reviewing all the infused drugs, the anesthetist a sensory level not clearly defined in the T5 topography. revealed significant hyperproteinorachia. Spine MRI
noted that tranexamic acid was wrongly administered The condition has remained stable with no improvement unveiled incursion of the cervical mass into the spinal
during the anesthetic procedure due to the first ampoule in weakness up to the present day. Personal history: canal through the neural foramen from C2 to C7, com-
being wrongly relabed. She was sedated, submitted to SAH. On examination: alert, oriented, mild intellectual pressing the cervical and dorsal cord. Surgical biopsy of
orotracheal intubation, and transferred to our Intensive impairment, isochoric and photoreagent pupils, parapa- the mass was conclusive for nodular sclerosing Hodgkin
Care Unit. At the admission in our Hospital, a new gen- resis (grade 1), spastic tone, normotrophic, superficial lymphoma. Steroids had been previously prescribed and
eralized tonic-clonic seizure was observed, so it was hypoesthesia at T5, distal hypopalesthesia with patellar had led to some motor improvement. Chemotherapy was
treated according to SE protocol. The electroencephalo- hyperreflexia, Babinski sign on the right and bilateral initiated and further progress was achieved, especially
gram demonstrated a burst-suppression pattern due to Hoffman sign. Mini mental with score below the value for upper limbs segments and for left lower limb. After
sedatives. Sedation was progressively reduced until the for the degree of education (27/30). Serum vitamin B12 four months of follow-up, however, trunk balance is still
patient woke up. She was discharged from the Hospital dosage <148, with macrocytic and hyperchromic red blood unsatisfying. Discussion: Hodgkin’s Lymphoma (HL) is
some weeks later without cognitive sequels, using leve- cells, without anemia; MRI of the neuroaxis showing a hematological malignancy that represents 0.5 to 1% of
tiracetam, which withdrawal was done at our outpatient confluent punctiform foci in subcortical and deep white adult cancers. Spinal cord compression (SCC) is usually
clinic. Discussion: Tranexamic acid is an antifibrinolytic matter, U-shaped cortical fibers and diffuse medullary a late complication by lymphoma. Moreover, it is three
agent indicated in an obstetric scenario to control post- atrophy, with hypersignal on T2 and STIR in the posterior times more frequent in non-Hodgkin’s disease. In HL, it
partum hemorrhage. Frequently anesthesiologists have cord at the level of D2 to D5, suggestive of vitamin B12 occurs only in 5% of cases. In the present report, there
this medication in their medicine box. Its adverse effects deficiency. Discussion: The combined clinical signs of is probably a direct spread from cervical lymph nodes,
may vary from severe back pain and muscle spasm to pyramidal syndrome, with clear predominance of lower although the cervical spine tends to be the least involved
hemodynamic instability, ventricular tachycardia, seizures limb involvement, sensory changes with spinal cord segment. The epidural mass infiltrated the spinal canal
and SE. There is no antidote to this drug, so the recom- sensory level suggestive of myelopathy, and quantified via the left neural foramen and spared the vertebral bod-
mended management is to treat complications. Cephalo cognitive decline, necessitated the need for CNS imag- ies. A retrospective study carried out between 1987-2002
Spinal Fluid (CSF) lavage, a technique that consists of ing study that showed extensive leukoencephalopathy gathered only 12 patients (11 males) with SCC, of which
10-20mL CSF drainage and infusing the same amount pattern and signal enhancement of the posterior spinal half with vertebral body destruction and only two with
of preservative-free normal saline, is indicated in some cord, which, together with the finding of serum hypovi- cervical compression. Cervical involvement is advanta-
cases. Although there are only a few studies considering taminosis B12 and gastric atrophy on AGE, strengthened geous in terms of time for diagnosis, since the mass may
this procedure, none of them were performed in patients the hypothesis of specific metabolic myelopathy. Final be promptly noticed and the diagnosis procedures are
with inadvertent intrathecal administration of tranexamic Comments: Vitamin B12 is directly linked to neuropsy- favored. Differential diagnosis includes: non-Hodgkin’s
acid. Final Comments: Despite doubtless advances in chiatric functions due to its participation in DNA synthesis lymphoma, squamous cell carcinoma,tuberculosis and
patient health security, mistakes with catastrophic dis- and neuronal myelination, and, thus, its deficiency can eosinophilic granuloma. Final Comments: We present
closures still occur. Even with many preventive strategies, cause alterations in the central and peripheral nervous a case of unusual manifestation of Hodgkin lymphoma,
the wrongly relabelled drug at the Hospital pharmacy in system. The above patient presented an atypical clinical that exemplified the need of multidisciplinary integra-
association with the similar aspects of bupivacaine and picture, with spastic paraparesis and fluctuating painful tion in order to establish the diagnosis. The neurological
tranexamic acid ampoules contributed to that. Thus, the dystonias, with a surge pattern, extensive radiological propaedeutic was able to predict the large extension of
relabel practice should be reviewed. involvement, suppressed serum dosage of this substrate, SCC and supported the celerity in the investigation and
and a definite digestive disabsorptive etiology, consis- intervention.
tent with the diagnosis of cyanocobalamin deficiency
Miscelânea encephalomyelopathy.
Miscelânea

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81
XXX Congresso Brasileiro de Neurologia

TL 1105016 TL 1105081 TL 1105118

DIFFUSE LARGE B CELL LYMPHOMA WITH ASEPTIC MENINGOENCEPHALITIS AND WERNICKE’S ENCEPHALOPATHY
SECONDARY CENTRAL NERVOUS SYSTEM PERIPHERAL NEUROPATHY – A RARE AND POLYNEUROPATHY FOLLOWING
WITH ATYPICAL PRESENTATION ASSOCIATION OF NEUROSARCOIDOSIS INTRAGASTRIC BALLOON PLACEMENT: A
CASE PRESENTATION
Andre Lopes Valente, Heloisa Lopes Cohim Moreira, Marcelo Tohoru Nakamura, Gloria Maria Shtini
Marcela Santarelli Casella, Adrialdo Jose Santos, Souza, Ingrid Pereira Marques, Roberto Pereira Giovana Barros e Silva Ribeiro, João Vitor Gerdulli
Fabiano Moulin de Morais Santos, Alberto Martins Pina Rodrigues, Rita Cássia Tamanini, Marco Tulio Medina Montoya, Marcio Luiz
Leite Fernandes, Marcos Martins Silva, Lucas Figueredo Balthazar
Universidade Federal de São Paulo. São Paulo SP, Leroux Ricchezza, Caio Faria Tardin, Nathalia Borges
Brazil Carmo Universidade Estadual de Campinas. Campinas SP,
Brazil
andrevalentelop@gmail.com Universidade Federal do Rio de Janeiro. Rio de
Janeiro RJ, Brazil giovanabsribeiro@gmail.com
Case Presentation: A 67-year-old man presented with 20
days of mental confusion, emotional apathy, dysphagia, marcttnakka@gmail.com Case Presentation: A 42-year-old woman, with a pre-
followed by decreased level of consciousness. Previously, vious medical history of hypertension, was brought to
he had a weight loss of 10 kg in 3 months. He had not Case Presentation: A 67-year-old female, began symp- the emergency department with an acute confusional
fever, night sweats or headache, and had no relevant toms of muscular weakness on both legs in June 2021 state that started five days prior to admission. No other
comorbidities. At examination, he had multiple cervical that evolved to an ataxia of gait on a two months period. symptoms were reported. The patient was submitted to
lymphadenopathy, spontaneous eye opening, localized At Hospital admission, she had sensitive ataxia, hypor- an intragastric balloon (IGB) placement four months
painful stimulus, no verbal response. MRC grade 3 in reflexia and mild weakness on lower limbs. There were before the beginning of symptoms, with significant emesis
all limbs, global hyperreflexia and frontal release signs neither cranial nerve palsies nor other abnormalities. After following the procedure and retrieval of the IGB a week
were present. Brain MRI demonstrated multiple con- five days of Hospitalization, she presented generalized prior to admission. During the physical examination,
trasting juxtacortical, hemispherical and bilateral nodular seizures and stupor, requiring mechanical ventilatory upbeat nystagmus and clinical signs of polyneuropathy
images, sometimes with diffusion restriction, as well as support. The liquor was clear with an open pressure of 15 in the lower extremities were noticed. Concomitant to a
in the hypothalamic-chiasmatic region, medial thalamus, cmH20, a protein level of 150 mg/dl, 74 mg/dl Glucose, thorough clinical and imaging evaluation, including the
pontomesencephalic transition and right cerebellar hemi- and 14/mm³ white cell count (78% lymphocytes, 22% investigation, initial treatment and exclusion of potential
sphere. There are also areas of discontinuous contracting monocytes); VDRL, test for M. tuberculosis and PCR causes, such as infectious and autoimmune encephali-
delineating the ependymal surface of the lateral and III for multiple viruses were all negative and no bacteria or tis, the patient was treated with intravenous thiamine,
ventricles. It is associated with hypersignal on T2/FLAIR of fungus were isolated on cultures. Brain and spinal cord which was followed by oral replacement. The diagnosis of
the adjacent brain parenchyma, determining compression MRI were normal. Chest Computed Tomography showed Wernicke’s encephalopathy was made clinically, although
of regional cerebrospinal fluid (CSF). CSF examination bilateral hilar and paratracheal adenomegaly.There was a specific imaging finding (mammillary body hemorrhage)
showed cell count of 7,6/mm, protein 178 mg/dl, lactate non-necrotizing granulomatous lymphadenitis on lymph was observed. She presented significant improvement
32 mg/dl, glucose 63 mg/dl and was negative infectious node biopsia. These elements allowed us to diagnose neu- regarding the cognitive impairment, as well as partial
screening and oncotic citology. He had negative serolo- rosarcoidosis (NS). She treated with methylprednisolone improvement of the ocular and motor symptoms. The
gies. Incisional biopsy was performed in cervical lymph 1g/day i.v. for 5 days followed by oral prednisone 5 mg/day patient is under physical therapy for rehabilitation and
node, compatible with Diffuse Large B-cell Lymphomas until discharge and two antiseiuzure drugs for epilepsy oral replacement of thiamine. Discussion: Wernicke’s
(DLBCLs). Discussion: Isolated central nervous system control. Neurological improvement was achieved along encephalopathy is a potentially severe acute neurological
(CNS) relapse involving the brain parenchyma is a rare the first week with the patient going out the ventilator condition caused by thiamine (B1) deficiency. Although
complication of non-Hodgkin lymphoma. Secondary CNS and becoming ambulatory with almost normal gait after typically described as a consequence of chronic alcohol
involvement (SCNSL) affects 5% of patients with DLBCL. thirty days. Homewer, she developed osteomyelitis and abuse, malabsorptive weight loss surgeries are emerging
Cranial nerve signs, headache, mental status and person- died after clinical complications. Discussion: Sarcoidosis causes. Restrictive procedures, such as intragastric bal-
ality changes were the most frequent manifestations. The is a multisystem inflammatory disorder characterized loon positioning, are not commonly associated with the
neuroimaging characteristics may mimic other neurologic by noncaseating granulomas in various organ systems, condition and scarce reports are present in the literature.
disorders. The immunocompetent patients with primary mainly the lungs and the lymphatic system. NS occurs Here, we describe the case of a patient that was admitted
CNS lymphoma (PCNSL) presents uniformly enhancing in about 5–10% of patients with sarcoidosis and 50% of with an acute confusional state and was diagnosed with
mass lesions without necrosis. Intraparenchymal mass and patients suspected of NS presents neurologic symptoms Wernicke’s encephalopathy after IGB placement. Given the
perilesional edema are the most common neuroradiolog- as first manifestation. Cranial neuropathy is the most potential severity of the condition and necessity of early
ical findings suggesting a SCNSL. SCNSL predominantly common manifestation (mainly facial nerves) and is seen treatment to prevent major neurological deficits, clinicians
presented with multiple lesions, in contrast to PCNSL, in 50–70% of cases. Peripheral nerve involvement occurs should be aware of this condition. Final Comments: The
while infratentorial and brainstem involvement were in 10% and brain and spinal cord lesions are less prev- case presented demonstrates a possible, although rare,
rare in SCNSL. In our case, multiple nodulary lesions can alent. Here we report an even rarer presentation of NS: cause of Wernicke’s encephalopathy and polyneuropathy.
mimic neurotuberculosis, nocardiosis and fungal infec- aseptic meningitis of chronic evolution associated with
tions. Final Comments: The suspicion of CSF lymphoma peripheral neuropathy. Glucocorticoids are considered
is a challenge in patients without previous diagnosis of to be first-line therapy. It is associated with good efficacy Miscelânea
aggressive lymphoma. Atypical MRI and CSF features and rapid clinical response. Severe presentations may
make it difficult to confirm diagnosis. In this scenario, require pulse-dose IV methylprednisolone, followed by a
invasive strategies and investigating the primary site prolonged oral glucocorticoid tapering. Final Comments:
could be necessary. This case illustrates a rare presentation of neurosarcoidosis
that deserves adequate recognition and prompt treatment
to avoid neurological sequelae or even the patient’s death.
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82
XXX Congresso Brasileiro de Neurologia

TL 1105121 TL 1105142 TL 1105146

POST-TRANSPLANT LYMPHOPROLIFERATIVE LATE DIAGNOSIS OF SPINAL CORD LIPOMA: CENTRAL NERVOUS SYSTEM INFILTRATION
DISORDER MYELORADICULOPATHY IN A A CASE-BASED UPDATE ON MULTIPLE MYELOMA: TWO CASE
KIDNEY TRANSPLANT RECIPIENT PRESENTATIONS OF A RARE CLINICAL
Luís Eduardo Oliveira Matos, Airton Ferreira
Ponte, André Felipe Oliveira Fernandes, Gabriel Sá PRESENTATION
Tácio Luis Cavalcante Coradine, Lucas de Oliveira
Cantaruti Guida, Adrialdo José Santos, Daniel Figueiredo, Daniela Remontti, Espártaco Moraes Matheus Alves da Silva, Barbara Maini de Carvalho,
Moreira Gabay, Vinícius Lopes Braga, João Victor Lima Ribeiro, Keven Ferreira Ponte, Paulo Roberto Rafael Bragança Rodrigues Matias, Eduardo
Cabral Correia Férrer, André Lopes Valente, Daniel Lacerda Leal Mesquita de Souza, Hennan Salzedas Teixeira,
Natalio Gauss Yankelevich Breno Moreno Gusmão, Victor Hugo Rocha Marussi,
Universidade Estadual do Ceará. Sobral CE, Brazil
Alex Machado Baeta
Universidade Federal de São Paulo. São Paulo SP,
luisematos@icloud.com
Brazil Beneficencia Portuguesa de São Paulo, São Paulo
Case Presentation: A 20-year-old woman is admitted SP, Brazil
tacio_coradine@hotmail.com
with progressive paraparesis started two months ago. matheus.alves.123@outlook.com
Case Presentation: 56-year-old Woman presented with Magnetic Resonance Imaging (MRI) showed an expansive
weakness in the right leg and urinary retention. She had intradural lesion, with thoracic spinal compression (T7- Case Presentation: A – A 64 years old patient presented
undergone living-donor kidney transplantation 21 years T8), hyperintense in T1/T2 and hypointense in T2 with to the emergency department complaining of holocra-
before for end-stage renal disease, use prednisone and fat saturation (T2 FAT). In an axial view, the spinal canal nial headache, unsteady gait and drowsiness. She had a
mycophenolate mofetil (MMF). Started right lower back was affected in >75% of its area. Biopsy revealed sections history of multiple myeloma inactive for four years after
pain and evolved with muscle weakness of the right leg, of mesenchymal neoplasia composed of mature adipose an autologous stem cell transplantation presented to
worse in distal regions, associated with numbness and cells, confirming the diagnosis of myelolipoma. In the sur- the emergency department. Her neurological examina-
tingling. In two weeks she was unable to walk and desen- gical procedure, partial resection was performed due to tion showed proximal muscle weakness associated with
volved urinary retention. Physical examination revealed the wide adherence of nervous structures, with significant exacerbation of all tendinous reflexes, demanding us to
right lower extremity paresis and reduced sensation to pain spinal decompression. There was complete neurologi- a complementary investigation with cranial neuroimage.
and touch below L1 level. Magnetic resonance imaging cal improvement, without post-surgical complications. The magnetic resonance imaging showed leptomenin-
(MRI) revealed cauda equina enhancement and a nodular Discussion: Non-dysraphic spinal cord lipomas com- geal, ependymal, supra and infratentorial lesions with
formation on left nucleo capsular location. Cerebrospinal pound less than 1% of spinal cord lesions. The diagnosis involvement of cranial nerves (subclinical at the moment)
fluid (CSF) analysis presented with lynphomonocytic is usually made in children, without sex predilection. In and parenchymal micronodular lesions. There were also
pleocytosis (50 cels), high protein (539 mg/dL) and lactate this work, is presented a late diagnosis case of spinal cord lesions in the cervical level of the spinal cord associated
(82 mg/dL), reduced glucose (9 mg/dl), PCR Epstein-Barr lipoma. Clinical, imaging and surgical discussions are with diffuse leptomeningeal impregnation, affecting the
virus (EBV) positive and immunophenotyping with mature also done. Myelolipoma is a rare non-dysraphic spinal cervical, thoracic, lumbar regions and the cauda equina
clonal B lymphocytes. At this point, we made a diagnosis cord tumor, infrequently diagnosed in adults. The most roots. The lumbar puncture showed monoclonal plas-
of Post-transplant lymphoproliferative disorder (PTLD) accepted hypothesis of its origin determines an associa- mocytes. Rescue chemotherapy was attempted, with-
myeloradiculopathy. MMF was suspended and started tion with defects in neurulation during embryogenesis. out therapeutic response. She died 5 months after the
chemotherapy (MADIT), Dexamethasone and Rituximab. Intramedullary lipoma patients usually present neuro- recurrence. B – A 58-year-old female patient diagnosed
After four months was observed an important reduction logical deficits secondary to mass effect, having a subtle with multiple myeloma for 4 years since admission, with
of de nodular lesion and normal CSF. Patient had partial clinical presentation, slowly progressive myelopathy and previous autologous bone marrow transplant presented
improvement on muscle weakness. Discussion: PTLD a rapid neurological dysfunction after a long period of to the Hospital with vertigo and headache. The magnetic
is a rare disorder associated to solid organ, stem cell, or indolent tumor growth. The clinical manifestations include resonance imaging showed lytic lesions on parietal bones
bone marrow transplantation. Pathogenesis is presumed pain, paraparesis and paraesthesias, varying according to with intense gadolinium uptake associated with signif-
to be associated with impairment in the cellular immunity, the lesion location and size, commonly the cervical-tho- icant temporal leptomeningeal thickening, inferring
leading to proliferation of the lymphoid system. EBV is a racic region. MRI is the most sensitive exam for diagnosis, invasion of the central nervous system by the underlying
relevant factor that plays causative and prognostic roles. with hyperintense lesions in T1 being characteristics of disease. The initial cerebrospinal fluid puncture had 27
The disease have a bimodal pattern of incidence, with a lipomas, confirmed by sequences with suppression of the cells, 100% with neoplasic features. Rescue chemotherapy
peak in the first and a second occurring after 7-10 years. fat signal. Treatment ranges from conservative methods, was performed, however the patient died 5 months after
Central nervous system (CNS) PTLD is considered a rare such as diet therapy, to aggressive surgical remove of the diagnosis. Discussion: Multiple myeloma is a hemato-
form of the disease, affects mostly supratentorial regions tumor. The main objective of surgery is to decompress logical disorder caused by abnormal plasmocyte clones
of CNS, specially the basal ganglia, and involvement of the adjacent structures, since lipomas cannot be completely with production of monoclonal antibodies. The disease
spinal cord is rare. Diagnostic confirmation is performed resected. The prognosis of surgically treated patients is can cause some neurological symptoms secondary to
with biochemical and cytological analysis of blood, CSF satisfactory, including pain reduction and neurological peripheral nervous system disease, amyloidosis and
and tissue from biopsy (gold standard). Patterns of alter- improvement. Tumor remissions have not been reported. spinal cord accometiment. However, while some onco-
ations in MRI may support suspicion. The treatment is Final Comments: Although rare, especially in adults, logical diseases more commonly presents with brain
focused in reduction of immunosuppression as the first spinal cord lipomas must be known, as these tumors are metastasis or leptomeningeal invasion, these are rare
step, radiotherapy and Rituximab with high response and well detected on MRI, their surgical approach is effective on myeloma multiple patients. Invasion of the central
chemotherapy as the standard option. Final Comments: and, if left untreated, they evolve with sudden neurolog- nervous system by multiple myeloma denotes a poor
CNS-PTLD representes a potential disabling cause, with ical worsening. prognosis, with some studies demonstrating an average
specific investigation and treatment. Therefore, it shows survival of 3 months, similar to the patients presented
the relevance of this discussion in the era of transplan- in this case. Final Comments: These case are rare and
tation and immunosuppression. Miscelânea
severe presentations of multiple myeloma. Knowledge
of such manifestations and detailed investigation allows
an accurate management.
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83
XXX Congresso Brasileiro de Neurologia

TL 1105150 TL 1105207 TL 1105237

POST-RADIOTHERAPY UNILATERAL OSMOTIC MYELINOLYSIS ASSOCIATED PROLONGED UNILATERAL MYDRIASIS


HYPOGLOSSAL NEUROPATHY: A CASE WITH PSYCHOGENIC POLYDIPSIA: CASE SECONDARY TO CHRONIC IPRATROPIUM
PRESENTATION PRESENTATION DOUGLAS MARQUES BROMIDE NEBULIZATION IN THE
ZARATINI, FRANKLIN TERTULINO FREITAS, OUTPATIENT SETTING: CASE PRESENTATION
Bruna Gutierres Gambirasio, Wardislau Ferreira,
Pedro Barbosa Oliveira, Thiago Yoshinaga Tonholo ALEX SOARES SOUZA SOUZA
Arthur Cesário de Holanda, Vitor Maia Arca, Ana
Silva, José Luiz Pedroso, Orlando Graziani Povoas Universidade Federal Rural do Semi-Árido – Rosa Santana, Bruno Henrique Carneiro Costa Filho,
Barsottini Mossoró – RN – Brazil Daniel Alves de Oliveira, Raphaelly Ribeiro Campos,
Universidade do Estado do Rio Grande do Norte – Geovane Gomes Silva, Victor de Moraes Chagas,
Escola Paulista de Medicina – UNIFESP, São Paulo
Mossoró – RN – Brazil Eduardo Sousa de Melo
SP, Brazil
douglaszaratini@hotmail.com Hospital das Clínicas da Universidade Federal de
brunagambirasio@hotmail.com
Pernambuco. Recife PE, Brazil
Case Presentation: A 54-year-old man had development Case Presentation: Patient with psychogenic polydip-
arthur.c.holanda@gmail.com
of involuntary movements of his tongue started one month sia evolving with hyponatremia, correction of serum
before the first neurologic evaluation. Those movements sodium levels, and consequent episode of altered level
Case Presentation: A 42-year-old female was evaluated
were intermittent occurring around ten times a day. One of consciousness. Years later, magnetic resonance imag-
at our Neurology service due to blurred vision and dilated
year prior, he presented an airway obstruction that led him ing showed features of extrapontine myelinolysis. Such
pupil in the right eye for three years. There were no pain,
to tracheostomy and the diagnosis of invasive squamous lesions were distributed exclusively in the corticosubcor-
diplopia, nor other associated symptoms. Visual com-
cell carcinoma at the right amygdala. He underwent 35 tical regions, mainly in the temporal region of the right
plaints and pupil diameter both fluctuated throughout
sessions of radiotherapy and was considered cured. His hemisphere, but also affecting the right parietal region
the months, without correlation to any factors. She had
medical history was also relevant for systemic hyperten- and the left temporal region. Discussion: Osmotic myeli-
hypertension, pre-diabetes, and asthma. Her current
sion, chronic kidney disease (due to systemic hyperten- nolysis (OM) is classified into 3 groups: central pontine
medications were losartan and inhalatory combination
sion) and hepatitis C. He was a former smoker, quitting myelinolysis (CPM) without extrapontine involvement,
of formoterol and budesonide, the latter started last
the habit about 30 years prior. At neurologic examination, extrapontine myelinolysis (EPM) without the involvement
month. She had been using salbutamol and ipratropium
tongue fasciculation was observed along with right sided of pontine sites, and a form of central pontine myelino-
bromide nebulization since childhood; five years ago,
deviation at protrusion. A CT brain scan excluded cancer lysis with extrapontine involvement (CPM+EPM). The
frequency increased to 8-hourly courses every day. She
recurrence. An electroneuromyography was performed extrapontine form affects regions such as the putamen,
had suspended its use two weeks before consultation, as
and disclosed chronic axonal involvement of the hypo- thalamus, caudate nucleus, external capsule, and, more
asthmatic symptoms were improving, and subsequently
glossal nerve, with the presence of fasciculation, probably rarely, corticosubcortical regions. Isolated cases of cor-
perceived a discrete pupillary reduction. At examina-
related to actinic neuropathy. Low-dose carbamazepine ticosubcortical involvement are extremely rare in the
tion, there was right mydriasis with absent direct and
treatment was started and by the dose of 200mg twice literature. The clinical presentation of patients affected
consensual photomotor reflex (Figure 1). Visual acuity,
a day, the patient had a complete improvement of the by this disease is quite variable, and the use of magnetic
visual field, direct ophthalmoscopy, and extrinsic ocular
fasciculations. Radiation-induced lower cranial nerve resonance imaging is extremely important to confirm
motricity were unremarkable, as well as the remaining
neuropathy is a rare complication of radiotherapy. It usu- the diagnosis. This pattern of exclusive corticosubcor-
neurological examination. She had been evaluated by
ally starts around five years after radiation and bilateral tical involvement was identified and well analyzed in
several physicians, and submitted to ophthalmological
involvement has been described. The most affected nerve an autopsy study, but it lacks reports on its radiological
tests, laboratory work-up, and neuroimaging studies (i.e.,
is the hypoglossal, due to its long and tortuous course features. Final Comments: As the clinical presentation
computed tomography and magnetic resonance imaging
from the brainstem to the tongue. The most important of OM is nonspecific and the radiological aspect can be
with angiography), without any findings. Ipratropium sus-
risk factor for lower cranial nerve neuropathy is a large characteristic, knowing the different imaging patterns is
pension was reinforced. Discussion: Some reports have
total radiation dose (more than 70Gy). No treatment of paramount importance to narrow the differential diag-
established the relationship between unilateral mydri-
has already been proved as effective, but there are some nosis for the various professionals who may face this type
asis and nebulized ipratropium bromide in intensive
case presentation on positive outcomes with high-dose of disease, such as neurologists, psychiatrists, emergency
care patients. It acts topically in the pupillary sphincter
steroids. There is also some data about the use of car- physicians, intensivists, among others. It is hoped that this
muscle by blocking cholinergic stimulation. Ill-fitting face
bamazepine as a membrane-stabilizing drug that may case presentation will provide an expansion of knowl-
masks, especially in patients with facial asymmetries, are
reduce nerve hyperexcitability. Our case presentation edge about this rare condition, generating an eventual
attributed as the major cause for unilateral presentation.
an earlier lower cranial nerve neuropathy due to radio- diagnostic and therapeutic optimization for future cases.
Inadequate self-medication and chronic misuse of face
therapy with a very good outcome with low dose of car- masks could explain this finding in the outpatient setting.
bamazepine. Cancer patients are surviving much longer Miscelânea Physical examination, complementary work-up, and fol-
nowadays, and it is extremely important to diagnose the low-up are essential for differential diagnosis, especially
treatment-related complications, firstly because of their with Adie’s pupil. Pilocarpine test is also helpful. In most
impact on the patients’ quality of life and secondly they reports, exposure is short and recovery fast after suspen-
may shorten patients life spam. sion. As the patient had five years of continuous expo-
sure, a longer time to resolution could be expected. Final
Comments: The present report stresses the importance
Miscelânea of an extensive review of patient history, especially in an
already over investigated patient. As far as we know, this
is the first case in which chronic exposure to ipratropium
bromide in the outpatient setting caused a prolonged
unilateral mydriasis.

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84
XXX Congresso Brasileiro de Neurologia

TL 1105272 TL 1105299 TL 1105301

UNRESECTABLE HIGH-GRADE PONTINE HEMATOMYELIA MIMICKING DESMOPLASTIC MEDULLOBLASTOMA IN AN


GLIOMA IN AN ADOLESCENT: A CASE-BASED LONGITUDINALLY EXTENSIVE MYELITIS: ADULT PATIENT: CASE PRESENTATION
UPDATE A CASE PRESENTATION
Rian Vilar Lima, Maria Carolina Rocha Muniz, Maria
João Valdêncio Silva, Luís Eduardo Oliveira Matos, Amanda Loureiro de Toledo Troian, Paulo Filho Thereza Leitão Mesquita, Gabriela Oliveira Muniz,
Matheus Brasil Câmara Monteiro, Paulo Roberto Soares Marcelino, Vanessa de Freitas Moreira, Yasmine Portela Velez Portela Velez, Mariana
Matos Neto, Daniela Remontti, Espártaco Moraes Déborah Inayara Mendes Tenório de Albuquerque, Macêdo Militão Mendonça, Francisca Adna Almeida
Lima Ribeiro, Gerardo Cristino Filho, Paulo Roberto Andreas Batista Schelp, Sephora Sabrina Candido de Oliveira, Denise Nunes Oliveira, Juliana Carneiro
Lacerda Leal de Almeida, Davi Vargas Freitas Teixeira, Larissa Melo, Giuliano Ferreira Morgantetti
Baccoli de Souza, Ana Karoline Basler, Herval
Universidade Estadual do Ceará. Sobral CE, Brazil Universidade de Fortaleza. Fortaleza CE, Brazil
Ribeiro Soares Neto
Laboratório Nordd Patologia. Fortaleza CE, Brazil
João valdencio@gmail.com Hospital do Servidor Público Estadual de São Paulo.
rianvilar@edu.unifor.br
São Paulo SP, Brazil
Case Presentation: A 14-years-old female has presented
a history of sporadic, frontal headache, triggered by amanda_lpms@hotmail.com Case Presentation: A 28-year-old female patient under-
movements, associated with vomiting, photophobia and went a biopsy of a spinal cord (SP) and cerebellar tumor.
osmophobia for 3 years. There are no reports of similar ill- Case Presentation: A 54-year-old woman, admitted in Grossly, the fragments from both sites were brownish,
nesses in the family and her parents are not consanguine- 2016 due to sudden severe neck pain with no history of firm and irregular. The histopathological examination
ous. The patient developed a visual clouding 7 months ago, trauma, that radiated to the left side of the body, asso- of the two tumors showed an undifferentiated neoplasm
which evolved to persistent diplopia and macular edema 2 ciated with decreased ipsilateral strength and sensitiv- with cells with hyperchromatic nuclei and high biphasic
months after. The symptoms had important intensification ity. On admission, she had hemiparesis, hyperreflexia cellularity, ranging from compact to loose. An immuno-
4 months ago, requiring Hospitalization. On admission, with Babinski´s sign and hypoesthesia of left hemibody. histochemical panel was performed with synaptophysin,
she had an episode of convulsive crisis and sensorium Spinal MRI showed T2/FLAIR C1-T2 hyperintense lesion, chromogranin, enolase, CD45, TTF1, PAX5, CD99, GFAP
lowering, so orotracheal intubation was performed. Skull with extensive vasogenic edema, mild hyposignal on and KI67 cell proliferation index (KI67-CPI). The results
Magnetic Resonance Imaging (MRI) showed an expansive, T1-weighted gradient-echo (GRE) inside the spinal cord showed positivity for enolase and focal positivity for syn-
infiltrative lesion with imprecise limits, with an epicenter and gadolinium-enhancement near the spinal canal. aptophysin and chromogranin. The KI67-CPI was 70%,
in the pons and extension to the midbrain, medulla and CSF analysis with 15 leukocytes (lymphomononuclear being lower in nodules. Discussion: Medulloblastoma
cerebellar peduncles, with a compressive effect on the cells predominance), 135 proteins, 79 glucose and 11275 (MB) is a primitive neuroectodermal tumor. Although
fourth ventricle and involvement of the basilar artery. In red blood cells (puncture accident). Anti-AQP4 antibody MB is the most common brain neoplasm in children, it
the evolution, convulsive episodes were installed again for was negative, as well as other infectious and inflamma- is very rare in adults, with an estimated incidence of 0,6
a week, and the neurosurgical evaluation did not indicate tory screening tests. The condition was attributed to cases per million per year. Normally, it emerges in poste-
the possibility of surgical resection or biopsy, thus being a seronegative longitudinally extensive myelitis with rior fossa structures, specially the cerebellum, and tends
transferred to the palliative radiotherapy unit. Discussion: probable inflammatory etiology and started 5 days of to disseminate through the cerebrospinal fluid pathways,
Gliomas represent about 24% of primary tumors of the high-dose steroid treatment, followed by 5 sessions of being especially prone to leptomeningeal spread. Rarely,
central nervous system, 80% of which are malignant. In plasma exchange, with no improvement in symptoms. other malignant tumors with small cell morphology can
general, they are histologically classified into low grade She was maintained with prednisone and azathioprine, be found, including small cell glioblastoma, which can
(I and II) and high grade (III – anaplastic astrocytomas/ suspended 3 years later due to myelotoxicity. In 2019, typically be excluded by immunohistochemistry, using
anaplastic oligodendrogliomas and IV – glioblastoma), new MRI scans showed hyposignal on GRE sequence in lineage markers or entity specific stains. The desmoplastic
in addition to molecular criteria. Clinical aspects depend the subcortical region of the left inferior parietal lobe, variant is a subtype of MB with medium to high risk. Our
on tumor location or compressive effect and may include thalamus, bulbo medullary transition and in the C1-T1 case presents the rare manifestation of a desmoplastic MB
focal neurological deficit, headache, nausea, vomiting, spinal cord, suggesting chronic hemorrhage. Idiopathic in cerebellum and SP in the same patient. Intramedullary
seizures, intracranial hypertension and visual changes. hematomyelia was the hypothesis made and the patient spinal dissemination is rare, with very few cases in the
Contrast-enhanced MRI is the imaging exam of choice, was kept without immunosuppressive, remaining stable available literature. We find no previous scientific report of
showing heterogeneous gadolinium enhancement, with until today. Discussion: Hematomyelia is a hematoma in a desmoplastic MB in SP, but there are seven documented
non-enhanced signal changes in the T2 images (infiltrative the spinal cord. There are few published case presenta- cases of small cells MB in SP, with two of them appearing
tumor), in addition to edema. Evidence of hemorrhage tion about vascular myelopathy caused by this entity. The simultaneously to an extramedullary component, as in our
or restricted diffusion may also be seen. Confirmation etiology can be traumatic (most common) or non-trau- report. Final Comments: In adulthood, MB has a higher
comes from the pathological examination of the resection matic. Idiopathic hematomyelia is the term used when proportion of desmoplastic histological features, different
or biopsy. Treatment depends on the degree of cancer, spontaneous intraspinal bleeding occurs without a clear proliferation rates and a tendency for late relapses. The
ranging from chemotherapy, radiotherapy and surgical etiology. Myelopathy occurs due to the mass effect caused therapeutic role of adjuvant chemotherapy in adults is
resection. Cases where the brainstem is largely involved by the hematoma, that acutely obstruct the cerebrospinal still not entirely known due to a shortage of prospective
are rare and unfeasible for resection, remaining palliative fluid and blood flow within the spinal cord. The symptoms studies. Surgical resection plays a central role in treatment
care. Final Comments: Early diagnosis and treatment presented are intense and sudden pain in the neck and and should be regarded individually, according to the sta-
of high-grade gliomas remains a challenge, especially in back, evolving rapidly (in hours to days) to myelopathic tus of the patient’s disease. Thus, further studies on the
centers with limited financial resources. Being aware of deficits. Recent studies have shown that patients initially occurrence of MB in adults and on SP involvement are
the presentations of these tumors is necessary for proper diagnosed with idiopathic transverse myelopathy often needed, in order to better understand prognostic factors
and rapid management. have alternative diagnoses, with vascular etiology being and the best treatment options.
one of the most common. Final Comments: Recognize
that hematomyelia can cause extensive spinal cord injury
Miscelânea and can mimic an autoimmune longitudinally extensive Miscelânea
myelitis is of fundamental importance, allowing for prompt
recognition and management for better outcomes.

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85
XXX Congresso Brasileiro de Neurologia

TL 1105318 TL 1105340 TL 1105347

CEREBELLAR SUBEPENDYMOMA IN AN FOIX-CHAVANY-MARIE SYNDROME: A CASE INTRADURAL DISC HERNIA: RARE


ADULT PATIENT: CASE PRESENTATION PRESENTATION CONDITION
Maria Carolina Rocha Muniz, Rian Vilar Lima, Gabriella Corrêa Dousseau, Sônia Maria Dozzi Aline Lourdes Pasqualli, Amanda Nassur Corrêa
Nathália Pinheiro Rodrigues, Gabriela Torres Alves Brucki, Caroline Suemi Ogusuku, Lorrane Campidelli Leite, Ana Carolina Graciano, Chelin Auswaldt
de Carvalho, Stella Maria Macêdo, José Samuel Arthuzo, José Lopes Vasconcelos Júnior, Elaine Steclan, Arlindo Américo de Oliveira, Michael
Pereira Filgueira, Priscila Natiele Mauricio Alves, Calumby Teixeira, Camila Rodrigues Nepomuceno, Ricardo Lang, Ivo Knut Anders Junior, Leonard
Juliana Carneiro Melo, Denise Nunes Oliveira, Maria Sheila Guimarães Rocha Rocha Fonceca de Brito
Giuliano Ferreira Morgantetti
Hospital Santa Marcelina. São Paulo SP, Brazil Faculdade Estácio. Jaraguá do Sul SC, Brazil
Universidade de Fortaleza. Fortaleza CE, Brazil Universidade do Contestado. Mafra SC, Brazil
Laboratório Nordd Patologia. Fortaleza CE, Brazil gabrielladousseau@gmail.com NEUROMAX; Hospital Universitário Santa Terezinha
– Joaçaba – SC – Brazil
rianvilar@edu.unifor.br Case Presentation: J.J.S, male, 61 years old. This patient Hospital São Vicente de Paulo – Mafra SC, Brazil
was referred with an insidious condition of vocal alter- Hospital São José. Jaraguá do Sul SC,Brazil
Case Presentation: A 29-year-old male patient underwent ation, difficulty in swallowing and sporadic choking since
a biopsy of a cerebellar tumor. Grossly, the fragments childhood. From the age of 40, he noticed an intensifica- chelinsteclan@gmail.com
obtained were irregular, brown and soft. Microscopically, tion of symptoms, presenting lower and slurred speech
circumscribed glial neoplasm was observed, formed by associated with significant dysphagia for solids and liq- Case Presentation: This is a case presentation with an
clustered nuclei, without atypia in the midst of occasional uids. Neurological examination showed involvement of investigative and descriptive methodology about clinical
microcysts with fibrinoid content. No mitotic or necrotic multiple cranial nerves with hypoesthesia and diparesis case of intradural disc hernia, a rare condition that brings
figures were identified. The association between clinical facial, reduction of the nausea reflex, dysphagia, dys- a lot of information that can enrich the scientific commu-
aspects, macroscopic features and microscopic findings arthrophonia and paresis of the tongue musculature. nity – A 65-year-old male patient with a history of low back
defined the diagnosis of subependymoma, a grade I Despite the paralysis of the orofacial voluntary muscles, pain without clinical improvement. Lumbar arthrodesis
tumor according to the 2021 World Health Organization they are preserved when evoked by emotion, such as the was performed (L3-4-5-S1). Over the months, he evolved
(WHO) classification. Discussion: Subependymomas are act of smiling. He also had mild cognitive impairment, with disease of the adjacent level. Neuroimaging exams
rare, well-circumscribed, slow-growing and often asymp- as evidenced by cognitive tests. On MRI, bilateral atro- such as Magnetic Resonance Imaging (MRI) suggested dis-
tomatic masses with an ependymal origin that are most phy was observed in the opercular regions. Discussion: ease of the adjacent level with extruded hernia. Extensive
commonly found in the ventricular system, usually in Foix-Chavany-Marie syndrome, also known as opercular arthrodesis was then performed. The disease evolved
the fourth or lateral ventricle. Its rarity is reflected in the syndrome, occurs due to bilateral cortical injury in the with a degenerative behavior, with disease of the adjacent
scarce number of reports in the scientific literature. They anterior opercular region. It is a rare suprabulbar palsy level and later with junctional kyphosis and paraparesis
represent 0.2 to 0.7% of all intracerebral tumors and can condition with dissociation in voluntary and sponta- – requiring a new surgery to understand the arthrodesis
manifest with cerebrospinal fluid obstruction, causing neous (emotional) movements. The clinical presenta- again, in which a transoperative diagnosis of intradu-
increased intracranial pressure. The intraparenchymal tion reflects bilateral paralysis of voluntary movements ral hernia was made. Microneurosurgical treatment by
site is very rare and other tumor locations include the of muscles innervated by NNCC V, VII, IX, X and XII, as arthrodesis was then performed, spinal decompression
septum pellucidum, foramen of Monro and, less com- in this case. However, the initial involvement of impair- with wide laminectomy and intradural hernia resection
monly, the spinal cord. Most tumors are found incidentally ment in chewing, tongue movement and speech apraxia, was performed. Postoperatively, he showed significant
at autopsy. Symptomatic subependymomas most often but without language impairment, stands out. The main improvement, returning to ambulation. He later evolved
affect middle-aged patients and may rarely manifest with cause is vascular, but cases caused by neuroinfections, with T8-9 spondylodiscitis that culminated in myelopathy.
intratumoral hemorrhage or seizures. Surgical exploration traumatic brain injury, neuroinflammatory, demyelin- Intradural disc herniation is a rare complication: 0.04%.
and biopsy are necessary to distinguish the appropriate ating and neurodegenerative diseases have already been In this way, presenting and discussing the diagnostic
treatment. Usually, tumor excision and radiotherapy are described. On MRI, asymmetric cortical atrophy is seen, (neuroimaging exam) and therapeutic (neurosurgical)
strongly recommended and often corrective, with tumor predominantly in the lower frontal region, especially in procedures associated with the unusual epidemiological
recurrence being quite rare. Final Comments: Our case the opercular region, as in this case. Final Comments: picture enriches the literature and medical discussion.
intends to register this rare case of subependymoma, This case shows that progressive loss of orofacial volun- There is a higher prevalence in males, mainly affecting
recommend more studies to understand the possible tary motor skills and apraxia of speech with preservation the lumbar region. The diagnosis of the disease is diffi-
origin of this tumor and highlight this possibility for the of emotionally evoked movements are the hallmarks of cult, with several differentials in the neuroimaging, and
diagnostic range in clinical picture of dizziness, empha- this rare syndrome. The exact diagnosis of the cause is not immediate surgery is essential to prevent the progression
sizing the importance of histopathology in defining the always possible and the treatment involves a multidisci- of the pathology and minimize the patient’s discomfort,
best course of action. plinary team. Due to the significant involvement of the as well as post-surgical neurofunctional follow-up for
facial muscles, malnutrition is the main consequence and rehabilitation. The present report discusses a lumbar
alternative feeding methods are necessary. degenerative disease that, after clinical and intraoper-
Miscelânea ative investigation, was diagnosed as an intradural disc
herniation, a rare and difficult to diagnose condition.
Miscelânea Therefore, the data presented tend to contribute to the
collection of clinical history of the disease, enabling the
possibility of early and effective diagnoses.

Miscelânea

86
XXX Congresso Brasileiro de Neurologia

TL 1105349 TL 1105377 TL 1105378

NEUROCUTANEOUS MELANOSIS WITH CERVICAL SPONDYLOTIC MYELOPATHY ISOLATED THIRD CRANIAL NERVE PALSY
CENTRAL NERVOUS SYSTEM INVASION MIMICKING SUBACUTE COMBINED WITH PUPIL SPARING IN PITUITARY
DEGENERATION: A CASE PRESENTATION APOPLEXY – CASE PRESENTATION
Guilherme Wandall, Sheila Wayszceyk, Chelin
Steclan, Arlindo Américo de Oliveira, Michael Gabriel Saboia de Araújo Torres, João Cláudio da Paula Baleeiro Rodrigues Silva, Victoria Veiga
Ricardo Lang, Gabriel Hoher Peres, Leonard Rocha Costa Urbano, João Gabriel Mansano de Oliveira, Ribeiro Gonçalves, Pedro Vinicius Brito Alves,
Fonseca de Brito, Filipe Laurindo Cabral Igor Vilela Brum, Filipe Tupinambá Di Pace, Márcia Paula Fiuza Rodrigues Medeiros, Nathalia Wanabe,
Rúbia Rodrigues Gonçalves, Ida Fortini, Luiz Isabela Fonseca Risso, Jorge Fernando de Miranda
FURB – Universidade Regional de Blumenau –
Henrique Martins Castro Pereira, Mauricio Silva Teixeira, Luiz Gustavo
Blumenau – SC – Brazil
Brenneisen Santos
Faculdade Estácio. Jaraguá do Sul SC, Brazil Hospital das Clínicas da Universidade de São Paulo.
Universidade do Contestado. Mafra SC, Brazil São Paulo SP, Brazil Hospital Sírio Libanês. São Paulo SP, Brazil
NEUROMAX; Hospital Universitário Santa Terezinha
– Joaçaba – SC – Brazil gabriel.fla15@hotmail.com paula.baleeiro1@gmail.com
Hospital São Vicente de Paulo – Mafra SC, Brazil
Hospital São José. Jaraguá do Sul SC,Brazil Case Presentation: A 60-year-old woman with rheuma- Case Presentation: We present the case of 54-year-old
toid arthritis presented with a 1-year history of difficulty man with a 5-day-history of sudden onset, severe, pul-
chelinsteclan@gmail.com holding objects due to numbness and weakness of the satile headache that was worse in the left retroocular
upper limbs. Three months before admission, the symp- region and associated with nausea and vomiting. Two
Case Presentation: An 18-year-old female patient with toms progressed to the lower limbs, and she required days after the onset, he developed binocular diplopia and
comorbid cutaneous melanosis was admitted to the support to walk. On physical examination, there was a left eyelid ptosis. Physical examination showed complete
emergency room reporting headaches for more than two spastic tetraparesis with extensor plantars and decreased isolated left third cranial nerve (3CN) palsy but the pupil
months. After neuroimaging findings, acute hydrocepha- vibratory and position sense in all limbs. The patient also was spared. Head MRI showed a 2.1x1.4x2.1cm pituitary
lus was observed and an emergency external ventricular experienced a prominent increase in limb weakness with adenoma, without significant cavernous sinus compres-
shunt was performed. Therefore, endoscopic third ventric- neck flexion (McArdle sign). The cervical MRI revealed sion. He also had normal TSH with low free-T4 level; low
ulostomy approach was performed for arachnoid biopsy spinal canal stenosis at the level of C2-C8 and symmetric prolactin and testosterone levels; and normal ACTH and
of the pre-pontine cistern, followed by septostomy. In the bilateral high signal within the dorsal columns (inverted cortisol levels. Cerebrospinal fluid had 13 white blood
biopsied pathological analyses, the presence of melano- “V” sign). Serum vitamin B12 was 243 pg/ml (normal cells/mm3 and 116mg/dL protein. Based on the clinical
cytic cells was then confirmed. In the immediate postop- 180–500 pg/ml) and serum methylmalonic acid was 0.13 history and complementary exams we made a diagnosis
erative period, there was clinical improvement, however, umol/L (normal < 0.40 umol/mL). Flexion–extension of pituitary apoplexy (PA). He was transferred to neurosur-
six months later there was decompensation of condition, radiographs revealed aggravation of cervical misalignment gery. Discussion: PA is a rare medical emergency caused
with reduced visual acuity, with failure of the third ven- with neck flexion. A standard anterior cervical discectomy by abrupt hemorrhaging and/or infarction of the pituitary
triculostomy and the need for a new bypass – however, was performed and there was a marked improvement in gland, generally due to a pituitary adenoma. Sudden and
due to the presence of disseminated skin lesions in the symptoms at 1-month follow-up. The typical MRI finding severe headache is the main symptom and can be associ-
abdominal region, it was then ventriculoatrial shunt was of subacute combined degeneration (SCD) is a symmetric ated with ocular palsy in 52% of the cases. The third 3CN
performed by puncture, resulting in clinical improvement. bilateral high signal of the dorsal columns that begin in is often the first and the most affected cranial nerve, and
One year after this last event, the patient developed tet- the upper thoracic region, with ascending or descending is impaired in 50% of the cases, but isolated 3CN palsy is
raparesis with plural predominance. The entire central progression. The lateral corticospinal tracts and lateral a rare manifestation. Complete nerve palsy characterized
nervous system was then reinvestigated, where several spinothalamic tract may also be involved. On the other by ptosis, impaired pupillary constriction and ophthal-
syringomyelia and spinal cord injuries were identified. hand, MRI of spondylotic myelopathy reveals a circum- moparesis is the most common clinical presentation, but
After that, the patient entered palliative and exclusive ferential enhancement sparing spinal cord gray matter pupil sparing may occur in 11% of cases of isolated 3NC
care, however, thirty days later, she died. Neurocutaneous on axial images, usually located beneath the site of max- palsy. Although complete 3CN palsy with pupil sparing
melanosis is a rare syndrome that is associated with a imum stenosis (pancake-like sign). We describe a case of is typical of ischemic lesions, compressive lesions may
cutaneous manifestation and even more rarely invades compressive myelopathy mimicking clinic and radiologic spare the pupil when the pressure of the lesion is evenly
the central nervous system. This condition can evolve in a characteristics of SCD. In addition, our patient also pre- distributed and allows the relatively pressure-resistant,
benign or malignant form, therefore, when presented early sented with McArdle sign, which is considered highly smaller-caliber pupillomotor fibers to escape injury; alter-
in life, it is associated with a high mortality rate. Although specific for the diagnosis of multiple sclerosis (MS). In natively, the lesion may compress only the inferior por-
skin lesions pose a risk of melanoma, the most likely our patient, the normal level of methylmalonic acid and tion of the nerve, sparing the dorsal pupillomotor fibers.
source of morbidity and mortality comes from “benign” the rapid improvement after surgery ruled out SCD. The In addition, several mechanisms have been proposed to
melanocytic proliferation to central nervous system, as clinical features were not compatible with MS. This This explain the occurrence of 3CN palsy in PA, such as direct
presented here. Common neurologic manifestations usu- is a case presentation shows that spondylotic myelopathy invasion of the cavernous sinus; compression without
ally arise in the first few years of life, however, continued can have a great spectrum of clinical and radiographic invasion, since the nerve is located horizontally in the
melanocytic proliferation can lead to other findings, as features and can mimic conditions such as SCD. same plane as the pituitary gland in the lateral wall of the
discussed here. This way, the present report discussed cavernous sinus; and compression of the vasa nervorum
the clinical history of a young woman with the rare form of the nerve due to increased pressure. In our opinion, the
of neurocutaneous melanosis with evolution to the brain Miscelânea last mechanism may also explain the sparing of superfi-
and spinal cord central nervous system. The description cial pupillomotor fibers. Final comments: PA should be
of the clinical history of rare conditions enriches knowl- considered an important differential diagnosis in patients
edge and makes it possible to guide other diagnostic and presenting with headache and 3CN palsy, even if isolated
therapeutic approaches. and with pupil sparing.

Miscelânea Miscelânea

87
XXX Congresso Brasileiro de Neurologia

TL 1105379 TL 1105387 TL 1105429

SPHENOCLIVAL INTRAOSSEOUS LESION METHOTREXATE NEUROTOXICITY WITH BRAIN METASTASIS DUE TO


MIMICKING ACUTE OPTIC NEURITIS: A CASE ATYPICAL RADIOLOGIC FINDINGS RETROPERITONEAL LEIOMYOSARCOMA:
PRESENTATION A CASE PRESENTATION
Anna Letícia de Moraes Alves, Juliana Naback
Felipe Rodrigues Marques Ferreira, Beatriz Pereira Toniolo, Victor Augusto Zanesi Maciel, Vanessa Leonardo de Sousa Bernardes, Ingrid Caroline
Rios, Murilo Reverendo Duarte, Telio Diego Cantalice Lauanna Lima Silva, Gustavo Maximiano Alves, Freitas Barboza, Paula Baleeiro Rodrigues Silva,
de Paula, Rubson Soares Rocha, Pedro Antonio Natália de Oliveira Silva, Tissiana Marques de Haes, Andreia Martini Pazini, Gabriel Novaes Rezende
Pereira de Jesus, Gustavo Balthazar da Silveira Fabíola Dach Batistella, Adrialdo José Santos
Carvalho
Hospital das Clínicas da Faculdade de Medicina de Universidade Federal de São Paulo. São Paulo SP,
Hospital Geral Roberto Santos. Salvador BA, Brazil Ribeirão Preto. Ribeirão Preto SP, Brazil Brazil
Hospital Sírio-Libanês, São Paulo SP, Brazil
bprios3@gmail.com moraes.annaleticia@gmail.com
leosb@hotmail.ca
Case Presentation: This 17 year old patient was referred Case Presentation: A 18 year old man with infiltration
to the Emergency Room for Neurological evaluation due of the central nervous system (CNS) by T-cell acute lym- Case Presentation: We present the case of a 53-year-old
to acute painful right eye vision loss, involuntary ipsilat- phoid leukemia was treated with intrathecal chemotherapy man with biopsy-proven retroperitoneal leiomyosar-
eral eyelid movements and pulsatile occipital pain. Pain (ITC) with dexamethasone, cytarabine and methotrexate coma (LMS) and pulmonary metastasis. Chemotherapy
was also triggered by eye movement. It was obtained pain (MTX). Two weeks later, severe lumbar arachnoiditis was started, with stability of the abdominal lesion and
relief with good response to simple analgesics, despite her was diagnosed, so ITC was interrupted and an Ommaya regression of the lung metastasis. Nine months after the
past medical history of Migraine. Additionally, physical reservoir was implanted. After 10 days, he developed diagnosis, he presented with progressive headache and
examination revealed decreased sensitivity at right V1 aphasia and apathy. Brain magnetic resonance imaging an episode of epileptic seizure. Head MRI showed meta-
trigeminal branch and ipsilateral reduced visual acuity. (MRI) revealed white matter hyperintensities in T2/FLAIR static brain lesion in the right parietal lobe. He underwent
Brain and Orbits MRI showed T1 and T2 hyperintensity (fluid attenuated inversion recovery)-weighted images in surgical resection followed by stereotactic radiosurgery
in the medium clivus, with signs of bone destruction and both frontal lobes, in corpus callosum and around the (total dose of 2000 cGy) and pathological examination
without gadolinium enhancement or DWI restriction. A Ommaya reservoir, with peripheral and irregular gadolin- confirmed LMS metastasis. Control head MRI was per-
contrast Brain CT was also performed which showed no ium enhancement and areas of diffusion-water restriction. formed 1 month after radiosurgery and showed a new
signs of vascular stenosis or venous thrombosis, even Leukemia infiltration and reservoir-associated infection right frontal lesion, which was also managed with stereo-
though some bone destruction could be noticed. Given the were considered as possible diagnoses. CSF analysis had tactic radiosurgery (total dose 3000 cGy). After a period
pain improvement, our patient was referred for follow-up no neoplastic cells and cultures were negative. Despite of 6 months of clinical and radiological stability, a new
at the clinic and possibly elective byopsy. Discussion: the achievement of hematologic recovery at the end of lesion close to the 3rd ventricle appeared, the previous
Depending on the tumor growth direction and speed, that chemotherapy phase, he persisted with neurologic brain lesions increased, and the systemic disease pro-
infiltrative sphenoclival lesions can mimick acute optic deficits. A few days later he was treated for clinical status gressed. He evolved with global functional decline, and
neuritis, including intraosseous lipoma as a distintictive epilepticus and a new brain MRI showed progression palliative care was then instituted. Discussion: Sarcoma
diagnosis to be considered. Although byopsy was not yet of the lesions. Spectroscopy had a pattern suggestive of refers to a cancer that arises from cells of mesenchymal
performed, MRI seems to be very useful for evaluating neoplastic infiltration, but considering the normal CSF origin while LMS is a rare histological subtype that origi-
this condition. Final Comments: Intraosseous lipoma analysis and the systemic remission of leukemia it was nates from smooth muscle. It is the second most common
is a hamartoma rarely manifested in the sphenoclival improbable. So, a brain biopsy was performed. Fragments soft-tissue sarcoma to affect the retroperitoneum. Sarcoma
topography and often asymptomatic. MRI can be very of deep frontal lesions showed edema, necrosis and hysti- metastasis to the central nervous system is uncommon,
suggestive, however byopsy is essential to properly diag- ocitary inflammatory reactional areas, without lymphoid occurring in 1-8% of the cases, with LMS being the most
nose and manage this condition. atypical infiltrates. Histokymic fungi methods tests and JC common subtype. Among LMS of all sites, the retroper-
virus search were negative. The morphology observed was itoneal LMS has the worst prognosis and about 80% –
compatible with MTX neurotoxicity. Given the temporal 87% of these patients die within 5 years. LMS responds
Miscelânea relationship of the lesions with the drug administration, poorly to chemotherapy and radiation, being considered
although there were some atypical MRI findings for this a resistant cancer; thus the best results come from early
condition, MTX was withheld for 2 months. Gradually, surgery, when feasible. Retroperitoneal LMS is often fatal
he had neurological improvement with complete res- owing to local recurrence and distant metastases. As our
olution of aphasia. Follow up MRI showed a decrease knowledge of retroperitoneal LMS brain metastasis is
of lesions. Discussion: MTX leukoencephalopathy is based on a few cases reports, the clinical management
rare and occurs in about 2 to 3,8% with MTX high dose and adjuvant therapy of theses tumors remain based on
treatments. It is commonly transient and improves with individualized decision-making. Final Comments: This
cessation of the drug. A subsequent MTX dose after the is one of the few cases described in the literature of brain
resolution of the acute phase is habitually well tolerated. metastasis from retroperitoneal LMS treated with surgical
Final Comments: MTX neurotoxicity typically presents as resection and stereotactic radiosurgery and, to the best of
peripheral neuropathy, but it is a main differential diag- our knowledge, the only case described with documenta-
nosis in CNS lesions in leukemia patients receiving ITC, tion of the treatment of the disease progression. Reports
particularly if neuroinfection and secondary neoplastic like this aim to contribute to the development of a body of
invasion were excluded. evidence that will guide the management of similar cases.

Miscelânea Miscelânea

88
XXX Congresso Brasileiro de Neurologia

TL 1105472 TL 1105503 TL 1105525

PROGRESSIVE MULTIFOCAL PRIMARY SARCOMA OF THE CENTRAL NEUROPLASTICITY ON DYKE DAVIDOFF


LEUKOENCEPHALOPATHY: A CASE NERVOUS SYSTEM – CASE PRESENTATION MASSON SYNDROME: A CASE
PRESENTATION IN A IMMUNOCOMPETENT PRESENTATION
Andréia Martini Pazini, Murillo Silva Catito, Thays
PATIENT Neri Andrade, João Norberto Stavale, Gabriel Bruna Andrade de Oliveira, Carolina Rocha da
Alana Strucker Barbosa, Bruno Eiji Nakano, Eduardo Novaes de Rezende Batistella, Adrialdo José Santos Silveira Brügger Cardoso, Natália da Costa Chiote
Silveira Marques Branco, Isabela Badan Fernandes, Pinheiro
Universidade Federal de São Paulo. São Paulo SP,
Gabriel Flamarin Cavasana, Paula Carolina Grande Brazil Instituto de Neurologia da Universidade Federal
Nakazato, Beatriz Cassarotti, Isabela Silva Souza,
do Rio de Janeiro – INDC/UFRJ. Rio de Janeiro RJ,
Lucas Oliveira Pinto Bertholdi, Fábio Araujo Pereira andreiampazini@gmail.com
Brazil
Faculdade de Medicina de Marilia – Marilia – SP – Case Presentation: A 28-years-old woman, presented carolinarbrugger@gmail.com
Brazil with headache in the right frontotemporal region start-
bruno.nakano1@gmail.com ing in July 2019, evolving with visual acuity changes, Case Presentation: A 57-year-old woman diagnosed
dizziness and paresthesia in the left upper limb (LLE). with Dyke Davidoff Masson Syndrome presented with
Case Presentation: A 23-years-old men, consanguine- Neurological examination showed only bilateral pap- cognitive impairment and secondary epilepsy since her
ous parents, present vertigo and left visual cloudiness in illedema. Magnetic Resonance Imaging (MRI) of the childhood. Orphan of both parents as a young infant, her
November 2020 evolving in 07 days with paraesthesia and skull showed a right frontoparietal dural lesion measur- prenatal history is therefore unknown.The patient lives
weakness in the right hand and dizziness. Pulse therapy ing 5.1x4.4x3.8cm, heterogeneous with T1 hyposignal, in a public psychiatry institution, where therapy, medi-
was done for 05 days without improvement. He evolved intense contrast enhancement and increased rCBV inside. cal assistance, artistic and ludic activities are offered. The
with incoordination, severe dysarthria, right hemiplegia. She underwent surgical resection in September 2019 radiological findings evidenced an important unilateral
CSF and serology were performed without changes; MRI with no residual lesion. Histopathological examination atrophy of the left cerebral hemisphere and an ipsilateral
of the skull that showed defects of semiovarian centers, identified features suggestive of high-grade fusocellular skull hypertrophy. Despite significant brain damage,
bilateral periatrial, posterior horns of lateral skulls, sple- sarcoma, with immunohistochemistry demonstrating a the patient maintained only cognitive impairment and
nium of the corpus callosum and cerebellar with hypo- Ki-67: Positive 50%. During the planning of radiotherapy, controlled seizures by the regular use of antiepileptic
signal in T1 and hypersignal in T2/FLAIR in the white the patient progressed with recurrence of complaints, drugs, being independent for daily living activities. Her
substance of the cerebral tissue. Immunoglobulin was neuroimaging showed disease progression, and a new neurological examination showed Wartenberg’s sign
performed without improvement of the condition, with surgical resection was performed in January 2020. The and hipotrophy in the right upper limb and reduction of
disease progression. Investigation of the immune sys- patient underwent adjuvant three-dimensional conformal motion in the same limb during gait.This favorable clin-
tem without alterations, positive JC virus, stereotactic radiotherapy (dose 60Gy). It evolves with recurrence of ical outcome may be explained by the innate capacity of
biopsy and anatomopathological examination charac- headache and paresis in LLE, in addition to focal motor neuroplasticity. Discussion: The Dyke-Davidoff-Masson
terizing Progressive Multifocal Leukoencephalopathy perceptual onset crisis. Cranial MRI showed progression Syndrome is defined as an atrophy or hypoplasia of one
(PML). Despite therapy, the patient passed away within of the lesion in the same topography. She underwent a cerebral hemisphere following a prior fetal or childhood
7 months. Discussion: PML is a central demyelinating third procedure in June 2021, with wide resection, but insult (1). The etiopathogenesis is not clear, but it could be
disease, related to JC virus reactivation, usually occuring with a residual lesion. Stereotactic radiosurgery was per- either a vascular insult during intrauterine life or acquired
in immunodepressed patients and less usual in immuno- formed in a residual lesion (dose 25Gy) in September 2021. causes like trauma, infection, vascular abnormalities and
competent patients. The physiopathology is based on the During clinical follow-up, a protuberance in the surgical intracranial hemorrhage in the perinatal period or shortly
oligodendrocytes and astrocytes lesions of the subcorti- wound was identified and MRI of the skull confirmed the thereafter (2). Clinical features depend on the extent of
cal hemispheric white matter and cerebellar peduncles, progression of the disease with exteriorization through brain injury and include hemiparesis or hemiplegia, sei-
and may also affect a gray matter of the basal ganglia. the frontal bone. A new surgical approach was performed zures, mental retardation or learning disability, speech or
The prognosis depends on variables such as the degree of in January 2022 with lesion resection, in addition to sub- language disorders and facial asymmetry (2). Diagnosis
contrast enhancement on neuroimaging, CD4 T lympho- dural empyema drainage and surgical wound cleaning. is settled by clinical history, neurological examination
cyte count and neurological recovery. Final Comments: Antibiotic therapy was started, but the patient evolved and radiologic findings. Treatment is symptomatic and
In the case under analysis, there was a peculiarity of the unfavorably and died in March 2022. Final Comments: aimed at controlling seizures and improving quality of
pathology affecting a previously healthy individual, with Primary sarcoma of the central nervous system (CNS) life (3). Some patients evolve with minimum neurologic
no history of Acquired Immunodeficiency Syndrome or is a rare neoplasm. It has been hypothesized that these impairment considering the extension of the brain dam-
use of medications that could adjust his immunological neoplasms originate from a pluripotent mesenchymal age. It could be explained by the nervous system’s ability
competence, making the diagnosis even more challeng- lineage within the dura or leptomeninges, extending to change its activity in response to stimuli by reorganizing
ing. Restoration of the adaptive immune system is the into the CNS parenchyma. Surgical resection along with its structure, functions, or connections after injuries (4).
main objective given the fact that the disease still has no chemotherapy and radiation have historically been the The basis of neural plasticity derives from synaptic plas-
specific treatment. cornerstone treatment strategy for CNS sarcoma. Future ticity that refers to changes in the strength of neurotrans-
studies should be focused to understand the histopatho- mission induced by activity experienced by the synapse
logical subtypes of primary CNS sarcoma to use different in the past (5). Final Comments: This report illustrates
Miscelânea chemotherapeutic agents which be helpful to give patients a rare syndrome that has a wide spectrum of cognitive
long survival and best choice of treatment. disabilities affecting multiple domains. We defend that
neuroplasticity has a key role on its prognost,making it
possible for one part of the brain to take up the functions
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89
XXX Congresso Brasileiro de Neurologia

TL 1105542 TL 1105597 TL 1105604

BELL’S PALSY AFTER CORONAVAC POSTERIOR REVERSIBLE ENCEPHALOPHATY PEDUNCULAR HALLUCINOSIS SECONDARY
VACCINATION: A CASE PRESENTATION SYNDROME (PRES): A CASE PRESENTATION TO CYCLOSPORINE PONTINE MYELINOLYSIS
IN A MALE PATIENT FROM THE NORTH’S
Eduarda Kotlinsky Weber, Jorge Ernesto Miyazaki Alison Mangolin, Victor Augusto Zanesi Maciel, Ellen
Araújo, Marina Scop Medeiros, Cristiano Schaffer INNER Silva de Carvalho, Beatriz Gioppo Betini, Roberto
Aguzzoli, Wyllians Vendramini Borelli, Tatiane Pedro Thiago de Cristo Rojas Cabral, Paulo Hermes Satler Cetlin, Natália de Oliveira Silva, Fabiola Dach
Morgana da Silva, Antonella Brun de Carvalho, Lima Amaral, Nise Alessandra de Carvalho Sousa,
Marina Musse Bernardes, Matheus Padão Schuster, FMRP Universidade de São Paulo. Ribeirão Preto
Luiziane Maria da Silva Alves, Flavia da Costa SP, Brazil
Lucas Porcello Schilling Mourão, Leonardo Rodrigues Leopoldo de Menezes,
Escola de Medicina da Pontifícia Universidade Wesley Lopes da Silva, Camille Albuquerque alison11235@gmail.com
Católica do Rio Grande do Sul. Porto Alegre RS, Rodrigues Chirano, Rafael Cavalcanti Fernandes
Brazil Rocha, Talísia Nascimento Vianez Case Presentation: A 54 years- old female patient,
Departamento de Neurologia do Hospital São Lucas Hospitalized in our service with a diagnosis of acute
Universidade Federal do Amazonas. Manaus AM, myeloid leukemia, presented with vivid and complex visual
da Escola de Medicina da Pontifícia Universidade Brazil
Católica do Rio Grande do Sul. Porto Alegre RS, hallucinations consisting mostly of her family members,
Brazil phla.med@uea.edu.br but with size distortions. She had recently undergone a
Global Brain Health Institute, Memory and Aging stem cell transplantation and was under immunosup-
Center, University of California San Francisco – Case Presentation: E. B. D. S., a 50-year-old, brown pressive treatment with cyclosporine. She had a normal
Estados Unidos male patient, born and resident in the interior of the neurological exam and denied previous similar episodes
Serviço de Neurologia do Hospital de Clínicas de Amazonas, was admitted in July 2021 with a history of of hallucinations of any kind. Her MRI showed diffuse T2
Porto Alegre. Porto Alegre RS, Brazil generalized tonic-clonic seizure preceded by occipital and Flair hyperintensities in pons, suggestive of osmotic
pulsating headache events and sudden loss of visual demyelination syndrome. She had normal laboratory
eduardakweber@hotmail.com acuity without pain on eye mobilization. He was referred exams, including serum glucose and electrolytes levels.
to the capital emergency department with psychomotor Due to her immunosuppressive status, CSF was also
Case Presentation: Patient is a 66 year old woman, diag- agitation and mental confusion after more than 48 hours obtained for investigation of cellularity, biochemistry,
nosed with systemic arterial hypertension and type II of the ictus. Brain Magnetic Ressonance Imaging (MRI) cultures, VDRL, Xpert MTB/RIF and viral PCRs (HSV 1
diabetes, with blood glucose under control. She received was performed and showed signal alteration of the sub- and 2, EBV, HIV). The results were unremarkable. There
the first dose of the CoronaVac COVID-19 vaccine on cortical white matter bilaterally in the occipital lobes, was no history of ethillism or other metabolic disorder
April 1st, 2021, and the second dose on April 22nd of the which indicated the possibility of reversible posterior that could result in the MRI findings. Three days before
same year. Four days after receiving the second dose, the encephalopathy (PRES). Previous commemoratives of the onset of symptoms her serum cyclosporine level
patient reports waking up with left side labial deviation, note included Systemic Arterial Hypertension (SAH) that was supratherapeutic. Therefore, the hypothesis of an
along with a painful sensation in her right eye. In addition, was difficult to control, diagnosed during Hospitalization, osmotic demyelination syndrome induced by cyclospo-
she exhibited muscle weakness on the right side of her and diabetic nephropathy. After discharge in August, the rine intoxication was made. Our patient had an atypical
face, causing difficulties in chewing food and swallowing patient was oriented to drug treatment combined with presentation, due to the lack of motor, cerebellar or cra-
liquids, which poured out of her mouth. Furthermore, the outpatient neurological and nephrological follow-up; nial nerve involvement, but presenting only with com-
patient states hardship in articulating words. She seeked a however, geographic and socioeconomic obstacles inter- plex visual hallucinations, compatible with peduncular
neurological evaluation on the same day, where peripheral rupted follow-up’s consistency. Discussion: PRES is a hallucinosis. After a change in her immunosuppressive
facial palsy House-Brackmann V was noted. No further clinical-radiological entity associated with a wide array therapy and cyclosporine discontinuation, the patient
abnormalities were found in the evaluation, and no signs of clinical presentations including visual disturbances, evolved with progressive improvement of symptoms.
of Herpes infection were noted in her ear. A brain MRI was headache, seizures and altered mental status.There is Discussion: Peduncular hallucinosis (PH) is a rare clinical
performed, with no acute alterations being revealed. She no single mechanism that explains the development of syndrome with dream-like visual hallucinations intruding
received a prescription of 60mg of Predinisone daily during PRES in all cases, and multiple nonexclusive mechanisms normal consciousness, most commonly occurring after
ten days, together with physiotherapy. In a few months of likely contribute. Hypertension is the most common pre- a midbrain or diencephalic lesion. The pathogenesis is
treatment, the patient had a complete recovery of facial cipitating factor, with endothelial dysfunction playing an explained by a disconnection between the ascending
sensibility and motor function. Discussion: Peripheral important role. The cerebral imaging abnormalities are reticular activating system and retinogeniculocalcarine
facial palsy or Bell’s palsy is an acute mononeuropathy often symmetric and predominate in the posterior white pathways, particularly at the level of the thalamus. PH
that leads to paresis or paralysis of the upper and lower matter. The combination of suggestive clinical manifesta- occurs mostly after ischaemic infarction and tumoral
face. Several etiologies for Bell’s Palsy have been reported, tions and radiological criteria establishes the diagnosis of lesions. Its occurrence after a imunossupressor induced
such as thermal shock, ischemia, infection and post-vac- PRES. Final Comments: The national incidence for Pres is osmotic demyelination syndrome is rare, with very few
cination immune trigger reactions. However, there are still uncertain. Searching main databases, there is an evident cases reported until now. Final Comments: Complex
few studies on the relationship between peripheral facial discrepancy in the described cases’ scarcity in the north visual hallucinations in an immunocompromised patient
paralysis and vaccination for COVID-19, especially with compared to other regions. The interior is a needness’ have a broad differential diagnosis and may require mul-
the CoronaVac vaccine. A Chinese study compared the portrait enhanced by obstacles from lack of integration tiple complementary exams. We describe a case of PH
risk of developing peripheral facial palsy between two beetween services which provide proper diagnosis, treat- secondary to cyclosporine-related pontine myelinolysis,
different COVID-19 vaccines: Pfizer and CoronaVac. The ment, and especially follow-up care for this neurological which is potentially reversible with rapid identification
study found an Odds Ratio of 2.385 for CoronaVac and disorder, as in this case presentation. and discontinuation of the drug.
1.755 for Pfizer – the latter, however, was not statistically
significant (CI=0.886–3.77 and p=0.11), not having a
significantly higher relative risk than the control group. Miscelânea Miscelânea
Therefore, the study concluded that there is an increased
risk of developing Bell’s Palsy with Coronavac. Final
Comments: Peripheral facial paralysis can be one of the
adverse events of vaccination for COVID-19. However, the
risk of developing these effects is minimal and does not
outweigh the benefit of getting vaccinated.

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90
XXX Congresso Brasileiro de Neurologia

TL 1105606 TL 1105618 TL 1105624

FAHR SYNDROME SECONDARY MYELOPATHY INDUCED BY VITAMIN CASE PRESENTATION PATIENT WITH
TO HYPOPARATHYROIDISM: CASE B12 DEFICIENCY IN THE PRESENCE OF INTRACRANIAL HYPERTENSION
PRESENTATION PERNICIOUS ANEMIA: CASE PRESENTATION SECONDARY TO THE USE OF USTEKINUMAB
Amanda Leticia Andre, Tatiane Lopes Alves de Jesus Amanda Leticia Andre, Tatiane Lopes Alves de Jesus Ludmila Machado Lima, Bruno Camporeze, Luiza
Arroyo, Arthur Coelho Moura Marinho, Nathalye Arroyo, Arthur Coelho Moura Marinho, Nathalye Gonçalves Fraga, Camila Carneiro Ferreira, Thais
Fernanda Pedroso Dircksen, Marcelo Simplicio Fernanda Pedroso Dircksen, Marcelo Simplico Takamura, Izadora Celant Miranda da Silva, Amanda
Carvalho, Lorena Fernandes Kronbauer, Damacio Carvalho, Lorena Fernandes Kronbauer, Damacio Batista Machado, Matheus Pedro Wasem
Ramon Kaimen Maciel Ramon Kaimen Maciel
Instituto de Neurologia de Curitiba. Curitiba PR,
Hospital Santa Casa de Londrina. Londrina PR, Hospital Santa Casa de Londrina. Londrina PR, Brazil
Brazil Brazil
ludlima@lwmail.com.br
amanda_leeticia@hotmail.com amanda_leeticia@hotmail.com
Case Presentation: Patient, 36 years old, male, with a
Case Presentation: A. J.M., 68 years old, history of falls Case Presentation: I.F.W, female, 65 years old, with dia- previous diagnosis of Psoriasis and HIV infection. On
associated with vertigo and syncope for a year. Also, betes mellitus and hypothyroidism, with no history of 05/18/2022 he woke up with a central scotoma in his right
report of behavioral change, visual hallucinations and alcoholism or gastrointestinal complaint. She started a eye and a temporal one on the left. He had episodes of
aggressiveness for eight months, receiving a diagnosis progressive picture of paraparesis, hypoesthesia below blurred vision in the morning during the week prior to the
of schizophrenia. No other comorbidities. At physical T10, urinary and fecal retention more than 30 days ago. reported symptoms, in addition to episodes of headache
examination, he was oriented in time and space, but On physical examination, she was lethargic, a force pre- in recent months and tinnitus of mild intensity and spon-
unattentive, with difficulty in understanding commands served in the upper limbs and reduced in the lower limbs. taneous resolution. Last dose of ustekinumab for psoriasis
and bradykinesia. Globally preserved strength and sen- Hypoesthesia at T10 level, paresthesia and dysesthesia in treatment on 05/06/2022. Subjected to retinal mapping
sitivity. Presence of bilateral dysmetry with ataxic gait.In feet and hands bilaterally, in addition to hypopalestinian that showed papilledema, worse on the right. In brain res-
Computed Tomography (CT) and Magnetic Resonance and proprioception abolished in lower limbs.Normal CSF, onance, no changes were seen. Laboratory tests showed
(MR) of the skull the presence of gross calcifications in serological tests for syphilis, HIV, HTLV and normal liver reagent serum FTA-ABS, VDRL 1: 2048 and undetectable
base nuclei, supra and infratentorial white substance and function, blood count with pancytopenia, normal iron Viral Load. A diagnosis of syphilis was made and treat-
bridge. Normal CSF and laboratory tests with low total and folic acid profile, and vitamin B12 < 50 mg/dL. High ment with Intravenous Crystalline Penicillin was initiated.
calcium (CA) corrected by albumin, reduced parathy- digestive endoscopy with biopsy confirming pernicious He was subjected to cerebrospinal fluid puncture with
roid (PTH) (value: 4), 25-hydroxyvitamin D and normal anemia. Normal magnetic resonance imaging (MR) of opening pressure of 36 cmH20 and result of non-reactive
urinary CA. Normal serological and rheumatoid exams, the skull and cervical spine, and in the dorsal column, VDRL, FTA ABS IgM and IgG in the cerebrospinal fluid.
abdominal and chest CT without special features. Cervical the presence of hypersignal in the posterior portion of the Treatment with acetazolamide was started. The patient
ultrasound with reduced thyroid size.In the face of the spinal cord at T10 and T11 levels, considering subacute evolved with significant improvement in the described
clinic and examinations, the patient presented criteria degeneration due to vitamin B12 deficiency. In view of visual symptoms. Discussion: The signs and symptoms
for diagnosis of hypoparathyroidism and Fahr Syndrome. the predominant clinical picture of a posterior cord, the of intracranial hypertension include headache, transient
Calcium replacement and symptomatic medications were diagnosis of myelopathy due to vitamin B12 deficiency visual blurring, amaurosis, papilledema, and diplopia sec-
performed, maintaining a stable neurological and neu- was established. Parenteral replacement was initiated ondary to sixth cranial nerve palsy. Through the analysis
ropsychiatric condition. Discussion: Fahr Syndrome is with a slight improvement up to discharge, considering of the clinical condition presented, associating it with the
a rare and intractable neurodegenerative condition. It is the time of evolution and involvement of nervous fibers. results of the complementary exams, as well as the exclu-
cured with neuropsychiatric, cognitive and other mani- Discussion: Vitamin B12 deficiency is caused by bad gas- sion of other causal factors for intracranial hypertension,
festations such as motor symptoms, ataxia and aphasia. trointestinal absorption, insufficient diet or genetic defects, a strong correlation between the situation presented by
Among its causes, we included hypoparathyroidism, and anemia is pernicious due to the absence of intrinsic the patient with the use of ustekinumab is suggested.This
pseudoparathyroidism, vasculitis and hereditary dis- factors. There are several neurological manifestations, is a monoclonal antibody, which binds to the p40 sub-
eases. Hypoparathyroidism results from deficient pro- including myelopathy, neuropathy, cognitive alterations unit of IL-12 and IL-23 so that they are unable to bind to
duction of PTH, resulting in inefficient mobilization of and others. The motor deficit results from involvement of their receptors, reducing T cell-mediated inflammation
bone calcium and inadequate renal resorption, leading pyramidal pathways associating the hypoPalestinian and in psoriasis. The most common adverse reactions are
to ectopic calcifications in various tissues, which, when batiesthesia due to posterior twonal involvement, and the nasopharyngitis, headache and upper respiratory tract
intracerebral, characterizes the syndrome, in addition to paresthesias due to polyneuropathy, usually axonal pat- infection. Limited data is available on its potential neu-
serum hypocalcemia.The calcium deposit in the central tern.Such a vitamin is a coenzyme in metabolic functions, rological adverse effects. Final Comments: Intracranial
nervous system starts in the blood vessel wall and peri- and neuronal damage occurs by accumulation of methyl- hypertension associated with the use of ustekinumab is
vascular space and extends slowly to involve the whole malonyl coenzyme A and increase of methylmalonic acid a rare condition. We emphasize the relevance of knowing
neurone. Progressive calcification generates tissue damage (MMA) and homocysteine, compromising normal myelin the medications in use by the patient in order to make a
and deposits of minerals. There is a predilection for basal synthesis. The diagnosis is made by the clinic, imaging differential diagnosis in patients with clinical manifes-
ganglia, also reaching other areas. There is no specific examination and vitamin B12 and/or homocysteine or tations of intracranial hypertension without anatomical
treatment for the Syndrome, and it is only symptomatic. urinary MMA dosage. Final Comments: Neurological alterations. Therefore, performing a complete anamne-
Final Comments: The Fahr Syndrome is rare, but an clinical presentation with definition of sensitive level of sis and careful ophthalmological examination is essen-
important differential diagnosis in cases of intracranial B12 deficiency is rare and of worse prognosis, and it is tial to obtain an accurate diagnosis and the adoption of
calcifications, considering the need to discover the pri- essential to suspect myelopathy cases, since early iden- appropriate therapy.
mary etiology for adequate treatment and orientation on tification with immediate treatment may result in better
the neurodegenerative character of the disease. outcomes for the patient.
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91
XXX Congresso Brasileiro de Neurologia

TL 1105626 TL 1105628 TL 1105633

CASE PRESENTATION AN ISOLATED HERPES SIMPLEX ENCEPHALITIS IN CLINICAL FEATURES OF A LARGE BRAZILIAN
PROGRESSIVE PARANEOPLASTIC PATIENTS WITH MALIGNANT GLIOMA COHORT OF PATIENTS WITH MELKERSSON-
MYELOPATHY ASSOCIATED WITH PRIMARY ROSENTHAL SYNDROME
Thays Neri Andrade, Adrialdo José Santos, Gabriel
KIDNEY NEOPLASMS Novaes de Rezende Batistella, Murillo Silva Catito, Hélvia Bertoldo de Oliveira, Wladimir Bocca Vieira
Lorena Praia de Souza Bezerra, Paulo Hermes Leonardo de Sousa Bernardes, Ana Luiza Jácome de Rezende Pinto, Igor Braga Farias, Paulo de Lima
Lima Amaral, Nise Alessandra de Carvalho Sousa, Franca Campos Serrano Roberta Correa Ribeiro, Isabela Danziato
Luiziane Maria da Silva Alves, Flavia da Costa Fernandes, Glenda Barbosa Barros, Samia Rogatis
Universidade Federal de São Paulo. São Paulo SP,
Mourão, Leonardo Rodrigues Leopoldo de Menezes, Calil, Paulo Victor Sgobbi Souza, Acary Souza Bulle
Brazil
Wesley Lopes da Silva, Ingrid Demosthenes Oliveira
Unipê. João Pessoa PB, Brazil
Wanzileu, Camille Albuquerque Rodrigues Chirano,
Division of Neuromuscular Diseases, Department of
Giselle Benevides Monteiro Ferreira thays.neri@hotmail.com
Neurology and Neurosurgery, Federal University of
Universidade Federal do Amazonas. Manaus AM, São Paulo UNIFESP
Case Presentation: APS, male, 30 years old, began with
Brazil strabismus and horizontal diplopia, headache, nausea, helviabertoldo@gmail.com
phla.med@uea.edu.br vomiting,dizziness, weakness and hypoesthesia in the left
side, evolving with difficulty walking, dysphagia, dysarthria Background: Melkersson-Rosenthal syndrome (MRS)
Case Presentation: Patient R.R.S, 70 years old, male, three months ago. Neurological exam showed alternating is a rare and underdiagnosed neurological condition,
started in August 2021 with clinical symptoms of monopa- syndrome characterized by a lesion in VI cranial nerve characterized by recurrent facial palsy, orofacial edema,
resis in the distal region of the right lower limb. The initial (NC) on the right, complete hemiparesis provided on the and fissured tongue, starting during the second or third
condition of distal paraparesis evolved, in a period of 3 left and hemihypoesthesia on the left. MRI evidenced het- decade of life. Its pathophysiological basis is still widely
months, ascending to proximal involvement of the lower erogeneous expansive/infiltrative lesion, with hypersig- unknown. Objective: Our objective was to characterize
limbs – being unable to walk in November 2021. In January nal on T2/FLAIR, focus of contrast uptake in the central clinical and epidemiological features of a brazilian cohort
2022 it evolved with urinary and fecal incontinence, portion, diffusely affecting the pons, extending inferiorly of patients with MRS. Methods: We performed a retro-
being indicated by the Urology team indwelling urinary to the medulla oblongata and right superior and middle spective observational study reviewing medical records
catheter. No history of spinal cord trauma was reported. cerebellar peduncles, compression of the fourth ventricle. from patients with MRS diagnosis from an initial sample
In his past pathological history, he has a history of vacci- Considering the diagnosis of Diffuse Midline Glioma, was of 1550 patients followed-up in a brazilian Neuromuscular
nation for COVID19 (Astrazeneca) in May 2021, Benign prescribed Stupp Protocol. One month later, the patient Reference Center. Results: We report 16 patients with MRS
Prostatic Hyperplasia, Pulmonary Tuberculosis at age 20. developed fever, brad psychism and tonic-clonic seizures. (7 women, 9 men), with age at diagnosis ranging from 20 to
During the reported period was made diagnostic investi- MRI with punctate cerebellar hemorrhages, thrombocyto- 84 years. All patients presented with peripheral facial nerve
gation with third parties, not being defined the etiology. penia (92,000), cerebrospinal fluid (CSF) with glucose 48, palsy as their first sign, starting at age 12 to 50 years, and
Neurological examination revealed a spinal cord syn- protein 106, leukocytes 53 (predominance of lymphocytes each episode lasting from 10 to 50 days. Fifteen patients
drome, due to spastic paraplegia associated with hypoes- and monocytes), negative cultures and DNA of Herpes presented with recurrent facial palsy, including one case
thesia and crural apalesthesia up to T8 level. Admitted for Virus 1 was detected in CSF. After 10 days of Acyclovir intra- with 28 episodes during 55 years of disease course. Two
diagnostic investigation, Neuroaxis Magnetic Resonance venously, the patient improved the level of consciousness. cases presented with synchronous bilateral peripheral
(MRI) was performed – an area of signal alteration in the Maintained follow-up, used adjuvant with TMZ, however facial nerve palsy. Six patients presented with the three
central aspect of the spinal cord of D5-11 was evidenced, there was tumor growth during the fourth cycle, being typical features (orofacial edema, fissured tongue, and
apparently without associated expansive effect, compat- currently in palliative care. Discussion: Herpes simplex facial palsy), while six presented with fissured tongue,
ible with longitudinally extensive myelitis. In addition, encephalitis (HSE) is a very severe infection of the cen- twelve with labial edema, and eleven with facial and orbital
Cerebrospinal Fluid was collected and analysis performed tral nervous system, affecting immunocompetent in most edema. Mischer’s granulomatous cheilitis was identified
(including TRM-TB) – with no changes. Request comple- cases. Perhaps, nowadays, it has been more commonly in four cases. We also identified: episodic dysgeusia (n=7),
mentary imaging exams, being evidenced in abdominal HSV encephalitis in immune-suppressed patients than is hypoacusis or hyperacusis (n=3), peripheral neuropathy
magnetic resonance imaging solid heterogeneous lesions currently recognized. Some studies suggest an increased (n=1), and other cranial nerve involvement (n=4). Facial
in the right kidney, with a suspicious appearance for neo- risk in patients with cancer and indicate that chemother- spasm or myokymia in almost 33% of cases and Marin
plastic involvement. On contrast-enhanced MRI of the apy and steroid administration could even induce HSE Amat synkinesis in 12,5% of cases. First clinical suspicion
skull, nodular lesions suggestive of secondary implants without brain irradiation by disrupting host immunity², included recurrent Bell palsy (n=11), multiple cranial
were visualized. Discussion: Due to the clinical picture promoting HSV reactivation. Other studies imply some neuropathy (n=1), neurosarcoidosis (n=1), Guillain-Barré
reported and the imaging tests analyzed, the diagnostic specific vulnerability in use of TMZ upregulates a pro- syndrome (n=1), multiple sclerosis (n=1), and ischemic
hypothesis was of isolated progressive paraneoplas- tein that results in promoting viral replication, cell death stroke (n=1). Three patients had a familial history of facial
tic myelopathy – with kidney neoplasia being the third and tissue injury1. One of the difficulties to identify HSE palsy. Medical history disclosed: varicella infection during
most common etiology. As a change in this pathology, condition is moderate symptoms in these patients and a childhood (n=11), chronic rhinitis (n=6), autoimmune
they were present in the reported patient T2-weighted cluster of alternative etiologies, like systemic infection, thyroiditis (n=3). Diagnostic approach disclosed: 75%
signal hyperintensity, with involvement that extends to cancer progression, metabolic diseases. Final Comments: of cases with normal neuroimaging studies; 100% with
more than three vertebral segments – associated with a Otherwise the co-occurrence of HSE and glioma is rare and normal cerebrospinal fluid analysis; a large NGS gene
picture of progressive myopathy and evidence of primary the benefits of TMZ chemoradiation overtake the risks in panel for neuropathies was performed in seven cases
neoplasm, Graus criteria can be used during diagnostic malignant glioma, HSE can happen during the standard and was unremarkable. Final Comments: MRS may be
investigation. Final Comments: The isolated progressive treatment. So, in case of suspicion, the treatment should associated with previous autoimmune and autoinflam-
paraneoplastic myelopathy still present as a very rare and be promptly started to avoid more damage. matory conditions. It is unlikely that a monogenic basis
poorly described, requiring further studies on the disease. exists for MRS, despite a predisposing genetic risk basis
being highly suggestive.
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92
XXX Congresso Brasileiro de Neurologia

TL 1105635 TL 1105699 TL 1105748

MULTIPLE CRANIAL NERVE PALSIES ATYPICAL CLINICAL PRESENTATION OF A DURAL ARTERIOVENOUS FISTULA AS A
SECONDARY TO SPHENOID SINUS PRIMARY NERVOUS SYSTEM GERMINOMA CAUSE OF MYELOPATHY
MUCOCELE: A CASE PRESENTATION IN A 32 YEARS OLD MAN – A CASE
Matheus Gurgel Saraiva, Mylena Gaudencio
PRESENTATION Bezerra, Francisco Anderson de Sá Carvalho, Paulo
Jéssica Daniele Marques, Giovana Barros e Silva
Ribeiro, João Vitor Sabino, Bianca Gomes Wanderley, Paulo Henrique Martinelli Oliveira, Lucas Silva Antonio Farias de Lucena, Maria Yvone Carlos
Gustavo Manginelli Lamas, Thomas Viera de Paula, Dias, Raderi Luiz Cardoso dos Santos, Indianara Formiga de Queiroz, Daniel Vicente de Siqueira Lima
Augusto Celso Scarparo Amato Filho, Alfredo Keila Pastório, Lorena Dias Araújo, Francine de Júnior, Luiza Alves Monteiro Torreão Villarim, Rafael
Damasceno Paula Roberto Domingos, Rafael de Almeida, Sayuri de Souza Andrade, Rafael Gonçalves Duarte Cunha,
Aparecida Hirayama, Raquel Mattos Filgueiras Thiago Medeiros Palmeira de Araujo
Universidade Estadual de Campinas. Campinas SP,
Brazil Hospital Municipal Dr. José de Carvalho Florence. Hospital Metropolitano Dom José Maria Pires. João
São José dos Campos SP, Brazil Pessoa PB, Brazil
jessica.dani.marques@gmail.com
paulohmoliveira96@gmail.com matheus_gurgel_pb@hotmail.com
Case Presentation: An 83-year-old male, with previous
medical history of hypertension, reported frontal head- Case Presentation: F.L.G, 32 years old, male, admitted Case Presentation: A 52-year-old male patient, without
ache with nausea and vomiting of sudden onset, lasting reporting amaurosis in the left eye, of acute onset, three comorbidities, presented crural motor deficits with insidi-
48 hours, that evolved with bilateral eyelid ptosis, restric- months ago. He presented new visual loss in the right eye, ous onset about 2 years ago. He had had a car accident one
tion of eye movement and worsening of visual acuity. with progressive worsening in 1 month. In addition, the month before the onset of the alterations. Upon admission,
The ocular motricity deficit progressed with complete patient reported 6 kilograms weight loss in three months the patient presented crural paraparesis, grade III to the
bilateral ophthalmoplegia. On physical examination, and dyschromatopsia since childhood. In the neurological left and grade IV to the right, associated with elastic hyper-
the following findings were observed: non-photoreactive exam there was dysosmia to the right side, inferolateral tonia and exalted patellar and Achilles reflexes. Spinal
mydriatic pupils, complete palsy of III, IV and VI cranial quadrantopsia in the right eye and amaurosis in the left resonance imaging showed increased volume with diffuse
nerves bilaterally, with absent oculocephalic reflex. There eye; besides that, it was identified atrophic papilla on the intramedullary hypersignal at T2/STIR, associated with
were no significant visual fields changes and fundoscopic left eye and afferent pupillary defect and papilledema in serpinous congestive dilatation of the intradural pial veins
examination showed no abnormalities. Facial sensitivity the right eye. Magnetic Resonance Imaging showed a cystic surrounding the thoracic medullary cord, predominantly
and motricity, as well as corneal reflex, were preserved. lesion in the pellucid septum and right nucleocapsular from the level of D6 to D12, including a lesser degree of
Cranial MRI showed an expansive lesion, with marked lesion. This patient was submitted to an endoscopic sur- edema of the medullary cone, suggestive of venous conges-
spontaneous hyperintensity on T1-weighted images and gery and biopsy showed a germinoma OCT-4 (MRQ-10), tive myelopathy secondary to dural spinal arteriovenous
a fine peripheral contrast-enhancement, centered on the PLAP (8A9), CD 117 (c-kit), Ki-67 (MIB-1) positive (60%). fistula (DASF). Subsequently, a spinal cord angiography
topography of the sphenoid sinuses, measuring approx- Discussion: Primary germinomas are rare malignant brain was performed, which confirmed the dorsal DASF at the
imately 4.2 × 3.4 × 2.8 cm. The lesion determined com- tumors with usual location in the pineal and supraselar level of the left D8 type 1 intervertebral foramen, and its
pression and cranial displacement of the pituitary gland regions. Its prevalence is approximately 0,2-1,7% of all embolization with adhesive embolic fluid. Anatomical
and stalk and was in close proximity with the intracranial primary intracranial tumors, most common among male. occlusion of the dural arteriovenous malformation was
internal carotid arteries, without signs of vascular invasion. The reason why germinomas occur is poorly understood obtained. Discussion: Spinal vascular malformations may
Laboratory evaluation demonstrated leukocytosis, with but it is secondary to changes during embryogenic devel- be arterial or venous, corresponding to rare lesions, with
no significant change in pituitary function. The patient opment. These tumors mainly are diagnosed until the an incidence of approximately 4% of spinal cord masses.
was, then, diagnosed with sphenoid mucocele. He under- second decade of life. The treatment involves radiation They constitute a quite heterogeneous group, the main
went endoscopic endonasal surgery, with evidence of a therapy or combination with craniospinal chemotherapy lesions being arteriovenous malformations (AVMs), DASF,
purulent collection in the sphenoid sinus. After surgical if the tumor is not metastatic. The case presented above spinal hemangiomas, cavernous angiomas and aneu-
drainage, the patient evolved with gradual improvement has an unexpected clinical presentation, once the most rysms. DASF is the most common (70%), being a direct
of the neurological deficits, maintaining only bilateral ocu- common are headache, vommiting, ataxia, somnolence communication between a radiculomingeal artery directly
lar abduction deficit and right eye adduction restriction. and eye movement abnormalities. Final Comments: This feeding a radicular vein; it causes venous congestion due
Discussion: Sphenoid sinus mucoceles are uncommon case is important to be discussed between neurologists to increased pressure in the venous system and consti-
lesions, representing only 1-2% of all paranasal sinus because its atypical signs and symptoms could mimic tutes a low-flow lesion. They are much more common in
mucoceles. The condition may cause severe neurological other exclusively neurological conditions, like inflam- men than in women and occur mainly between the fifth
sequelae, including amaurosis and intracranial spread of atory central nervous system diseases. Besides that, the and eighth decade of life, with the thoracolumbar region
the infection, if there is a delay in diagnosis or treatment. age of the disease, in the reported case, is different than being the most affected. Final Comments: DASF is a rare
Symptoms are nonspecific and result from direct mechan- expected, what could delay the diagnosis. From that it cause of myelopathy but potentially treatable if diagnosed
ical pressure on adjacent structures or nerve involvement is important to neurologists recognize this condition, early. A case was shown that due to the prolonged time
in the inflammatory process. The most common signs and despite of unlikely epidemiology and clinical presenta- between the onset of symptoms and adequate treatment
symptoms are headache, visual loss, and III and VI cra- tion, to make early diagnosis and improve survival rate the patient had little clinical response.
nial nerves palsies. Final Comments: The case presented once initiated the treatment.
demonstrates a rare condition that may evolve with mul-
tiple cranial nerves palsies, and should be considered as a Miscelânea
differential diagnosis when identifying expansive lesions Miscelânea
in the topography of the sphenoid sinuses.

Miscelânea

93
XXX Congresso Brasileiro de Neurologia

TL 1105765 TL 1105779 TL 1105798

ACUTE HEMORRHAGIC STROKE AS A SUBACUTE COMBINED DEGENERATION IN A CEREBRAL BILATERAL MONOCULAR


TRIGGER FOR MYXEDEMA COMA: A CASE VEGAN YOUNG WOMAN: THE IMPORTANCE POLYOPIA: AN UNUSUAL PRESENTATION OF
PRESENTATION OF A CORRECT TREATMENT AN ISCHAEMIC STROKE
Giovana Barros e Silva Ribeiro, Nadson Bruno Serra Davi Teixeira Urzêdo Queiroz, Ianka Cristina Ernesto, Lucas Gondim Briand Vieira, Victor Augusto Zanesi
Santos, Sabrina Vechini Gouvêa, Guilherme Vassoler Henrique Amancio Ferreira, João Victor Lage Maciel, Alison Mangolin, Gabriela Lopes de Morais,
Baldasso, Fernanda Garcia Callegari, Deny Glauber Guerra, Andre Filipe Luchi Rodrigues, Bianca Gomes Anna Letícia de Moraes Alves, Rui Kleber do Vale
Pereira Mazzoni, Sarah Teixeira Camargos, Breno Franco Martins Filho, Octávio Marques Pontes Neto
Silveira Fernandes, Rodrigo Santiago Gomez
Universidade Estadual de Campinas. Campinas SP, Hospital das Clínicas da Faculdade de Medicina de
Brazil Hospital das Clínicas da UFMG. Belo Horizonte MG, Ribeirão Preto. Ribeirão Preto SP, Brazil
Brazil
giovanabsribeiro@gmail.com victor93@gmail.com
davituq@gmail.com
Case Presentation: A 66-year-old woman, with a previous Case Presentation: A 58-year-old man presented to our
medical history of diabetes, hypertension and hypothy- Case Presentation: A 31 year-old woman presented emergency unit with a history of a sudden low visual acuity
roidism, was brought to the emergency department due with numbness and complete loss of vibratory sensitiv- 2 days prior to the admission followed by a polyopia up to
to loss of consciousness nine hours prior to admission. ity and proprioception in her legs and arms, significantly four images in both eyes, in all 9 positions of gaze which
At the initial evaluation, a left central facial palsy and left decreased temperature and pain sensitivity at T12 level, did not improve with monocular vision. His medical his-
hemiplegia were observed, as well as severe hypertension. as well as an ataxic gait and postural instability, which tory included type 2 diabetes and ischemic stroke with a
The initial non-contrast head CT showed an acute bleed- had been present for over a year. When the symptoms mild left hemiparesis. The patient had an otherwise normal
ing in the right nucleocapsular and frontal regions, with started, she sought medical care. Exams then revealed ophthalmological exam, except for visual acuity of 20/40
areas of subarachnoid hemorrhage. After initial evaluation low levels of vitamin B12 and she received three doses in the left eye and 20/30 in the right eye, besides two hard
and emergency management, including endotracheal of 5.000UI parenteral cyanocobalamin. However, the exudates in the right eye at the direct fundoscopy. Visual
intubation and infusion of intravenous vasopressors, the symptoms worsened over time and she was referred field testing was unreliable due to the fluctuating diplopia.
patient was transferred to the intensive care unit. She was to our service. Electroneuromyography performed at On examination, he had a mild sequelae left hemiparesis
maintained under continuous venous infusion of seda- admission showed pure sensitive axonal polyneuropa- and simultanagnosia. The patient elaborated better that his
tives, with the following need of neuromuscular blocking thy; magnetic resonance imaging of her spine suggested double vision involved persisting images in his visual field,
agents, due to frequent patient-ventilator asynchrony subacute combined degeneration from C2 to T12; labora- indicating palinopsia. The remainder of the neurological
despite optimization of ventilatory parameters. Over the tory findings were normal, including vitamin B12 levels. exam was normal. A CT scan showed chronic watershed
following days, after empiric treatment of ventilator-asso- Since the patient was vegan and had never taken vitamin right frontoparietooccipitall hypodensities and subacute
ciated pneumonia was started, she developed bradycardia supplements, the main hypothesis remained incorrectly watershed left parietooccipital hypodensities, later con-
and hypertension, but the non-contrast head CT showed treated vitamin B12 deficiency. Therefore, she was pre- firmed the same infarct areas by magnetic resonance
stability of the initial findings, without worsening of the scribed 5.000UI of intramuscular cyanocobalamin every imaging. The patient was diagnosed with a new ischemic
cerebral edema. The patient, then, developed anasarca, week for four weeks and then one dose per month. The stroke and started dual antiplatelet therapy. His clinical
worsening of the ventilation parameters and hypoxemia patient returned for a new evaluation two months after presentation was attributed to cerebral polyopia with
(P/F ratio of 98). At the occasion, a CT pulmonary angio- Hospital discharge. Her gait had improved, along with palinopsia. Discussion: Diplopia is a frequent complaint
gram was performed, but showed no signals of pulmonary proprioception and vibratory sensitivity in the lower in the neurology clinic. Unilateral monocular diplopia is
thromboembolism. The laboratory workup revealed a limbs. Her neuropathic pain was well controlled with secondary to intraocular pathology in most cases. Bilateral
TSH of 55,90 ?UI/mL, FT4 < 0,10 ng/dL and total triiodo- 150mg pregabalin. Results of her gastric endoscopy were monocular diplopia can occur due to an intraocular pro-
thyronine (T3) < 26 ng/dL. The patient was, then, started normal and there was no evidence of autoimmune disease cess, but that’s unusual to happen simultaneously in both
on steroids (hydrocortisone) and levothyroxine, with that could hamper vitamin B12 absorption. Discussion: eyes. Polyopia is the visualization of multiple images, often
significant improvement of the ventilatory parameters Vitamin B12 deficiency prevalence vary among different due to an intraocular or a functional disorder. Regardless,
over the following days, in addition to resolution of the populations. Some conditions may be associated with it, it can be a rare manifestation of cortical and subcortical
bradycardia. Discussion: Myxedema coma is a severe like reduced intake of animal products and strict vegan ischemic stroke at the non-dominant or bilateral occipital
and life-threatening endocrine emergency that occurs diet. The clinical manifestations can range from hema- lobe and even in the dominant parietal lobe. Palinopsia
when physiological adaptations to untreated hypothy- tological findings (anemia) to neuropsychiatric changes is the persistent visualization of visual images after the
roidism are overwhelmed by an acute precipitant. The (mood fluctuations, insomnia, cognitive slowing, ataxia, stimulus ceased and occur due to cortical non-dominant
most common triggers to the condition are hypother- sensitivity disturbances and weakness). The neurological occipital lesions. Final Comments: Bilateral monocular
mia and infection, while other described precipitants findings can exist even without hematological alterations. polyopia is a rare manifestation of ischemic stroke. In
are medications, surgery, GI bleeding and myocardial Patients with neurological abnormalities may benefit the presence of this symptom, a complete evaluation of
infarction. Although uncommon, acute hemorrhagic from more aggressive cyanocobalamin replacement, higher order visual function is mandatory, such as visual
stroke is a possible precipitating event and the condition starting with weekly parenteral replacement for at least agnosia, simultagnosia and palinopsia. We report an illus-
should be early diagnosed for prompt initial treatment. four weeks and then once per month. It is important as trative case of watershed ischemic stroke manifesting as
Final Comments: The case presented demonstrates a rare well to investigate the ethology. Final Comments: Despite polyopia and palinopsia associated with a simultagnosia.
association of acute hemorrhagic stroke and myxedema being a common ethology for neurological abnormali-
coma. Given the severity of the condition, early suspicion ties, this case presentation demonstrates how harmful
should be made for prompt initiation of the treatment. vitamin B12 deficiency can be if not properly addressed. Miscelânea

Miscelânea Miscelânea

94
XXX Congresso Brasileiro de Neurologia

TL 1105839 TL 1105864 TL 1105865

WERNICKE ENCEPHALOPATHY: A LIFE- CASE PRESENTATION SEROTONIN FIRST CASE OF OCULAR CONTRAPULSION
THREATENING CONDITION, PASSIVE TO SYNDROME ASSOCIATED WITH FOLLOWED BY IPSIPULSION IN
REVERSIBILITY IN THE FACE OF EARLY MEDICATIONS WITH A SECONDARY WALLENBERG SYNDROME
CLINICAL RECOGNITION SEROTONERGIC EFFECT
Beatriz Beatriz Vieira Coutinho, Aline Lócio, Lia
Caroline Santos Eneas, Déborah Castro Ferreira Natana Rangel da Silva Ribeiro, Paula Tomiko Jeiressati, João Pedro Benati, Victoria Fontenele,
de Oliveira, Isadora de Castro Ferreira de Oliveira, Uchida, Fernanda Nicoli Broch, Mariana Severo Lara Paiva, Rafaela Iughetti, Victor Araújo, Isadora
Nayara da Costa Andrade, Ana Paula de Goes Louly Debastiani, Manuella Giusti Fin, Gabriela Klein Ponte, Júlio Santos
Bustamante, Eduardo Bernardo Chaves Neto, Herwig, Artur Vestena Rossato, Lara Willers Lobato,
Centro Universitário Christus – Unichristus.
Pablo Henrique da Costa Silva, Marta Rodrigues Pedro Lorenzo Neves da Silva, Marlise Castro
Fortaleza CE, Brazil
de Carvalho, Ronaldo Maciel Dias, André Gustavo Ribeiro
Unichristus – Forteza – CE – Brazil
Fonseca Ferreira
Universidade Federal de Ciências da Saúde de Porto
beatrizvlc1616@gmail.com
Hospital de Base do Distrito Federal. Brasília DF, Alegre – UFCSPA – Porto Alegre – RS – Brazil
Brazil
natanarangel@gmail.com Case Presentation: A 51-year-old man presented with
Universidade Federal do Piauí. Teresina PI, Brazil
a sudden rotatory dizziness, unsteady gait, and numb-
dra.carolineneas@gmail.com Case Presentation: A 31-year-old male patient with a ness in the left hemibody. He showed left palate paresis,
diagnosis of obsessive compulsive disorder, undergoing incoordenation, horizontal jerk nystagmus with left fast
Case Presentation: Patient, male, 43 years old, chronic knee pain investigation for two months, was referred to fase, and ocular contrapulsion evoked by eye closure.
alcoholic, presented a fall from his own height, evolving the emergency room due to myalgia and tremors of the Magnetic Resonance Imagingresonance imaging (MRI)
with ataxic gait and visual clouding. After the 4th day, extremities. He was on daily use of fluvoxamine 100mg, Flairflair showed left cerebellar hyperintensity. Diffusion-
he presented worsening of symptoms with acute mental quetiapine 100mg and clomipramine 112.5mg increased Weighted Imaging (DWI)/Apparent Diffusion Coefficient
confusion, vertigo syndrome, headache and vomiting. On for 3 weeks in addition to pregabalin and tramadol on (ADC) showed infarction in the left lateral medulla. After
neurological examination: Vigil with spatial disorientation, demand. During evaluation, tachycardia, hyperthermia, 7 days of symptoms, he reported intermittent hiccups
Mini Exam of the Mental State : 18 points, Clock test: hit diaphoresis, global hypertonicity and hyperreflexia were treated with chlorpromazine. The ocular exam at this time
the circle, missed orders of numbers; Vertical Nystagmus also present. Anxiety and restlessness quickly progressed revealed ocular lateropulsion ipsilateral to the lesion (ipsi-
at rest and horizontal at the lateral view on both sides; to lethargy. Laboratory tests showed an increase in serum pulsion) Discussion: Wallenberg syndrome, also called
Skew Deviation; tremor of the extremities, ataxic gait, levels of creatine phosphokinase (CPK), characterizing lateral medullar syndrome, is caused by the infarct in the
dysbasis with fall to the Romberg maneuver without rhabdomyolysis. Computed tomography of brain and territory of posterior inferior cerebellar artery. Its clinical
latency, with preferential side to the right, and vibratory cerebrospinal fluid examination showed no changes. signs and symptoms include vertigo, nystagmus, diplopia,
hypoesthesiav in the left foot. Among the tests, LDH 594; Treatment involved discontinuation of offending agents, ipsilateral Horner syndrome, facial ruddiness and dry
GGT 902; TGP 696; TGO 642; RT PCR SARs-COV2 positive. supportive therapy and use of benzodiazepine, in addi- skin, dysphonia, dysphagia, dysarthria, ipsilateral loss
Skull MRI showed hypersignal in nipple bodies and in the tion to monitoring for declining CPK levels. Discussion: of gag reflex, ipsilateral ataxia, ipsilateral impaired taste,
bilateral medial thalamic region, in FLAIR. Treatment Patients with serotonin syndrome can deteriorate rapidly ipsilateral facial pain and paresthesia, decreased ipsilat-
performed with intramuscular (IM) thiamine for 7 days, and require intensive care. This syndrome occurs due to a eral blink reflex, contralateral hypoalgesia and thermoa-
progressing with significant improvement in ataxia and progression of serotonergic toxicity based on an increase in naesthesia in the trunk and limbs; and ipsilateral facial
partial improvement of nystagmus, but maintained the its concentration levels. Conditions that can alter serotonin hypoalgesia and thermoanaesthesia. Final Comments:
mental confusion. He was discharged from Hospital, with regulation include therapeutic doses, drug interactions, To our knowledge, this semiologic feature of alternating
oral thiamine prescription, for 24 months. Discussion: intentional or unintentional overdoses, and overlapping lateropulsion has not been previously described in the
Wernicke’s encephalopathy (WE), thiamine deficiency, transitions between medications. Therefore, it can be literature until now. The scientific study of atypical clin-
should be part of the differential diagnoses of acute/ avoided with careful titration of drugs and also observing ical manifestations can help medical care to identify the
subgued ataxic syndromes, especially in the context of the possibility of interaction with drug classes that do not syndrome early and to better manage patients.
chronic alcoholism. The patient in the case described indicate their serotonergic action in their nomenclature
has 3 of the 4 CAINE criteria for clinical diagnosis (acute (e. g. opioids or antiepileptic drugs), especially in poly-
encephalopathy; oculomotor dysfunction and ataxic gait). pharmacy. Final Comments: Serotonin syndrome is Miscelânea
Laboratory tests show changes in the markers of liver and characterized by a triad of autonomic dysfunction, neu-
canalicular injury, secondary to the chronicity of alcohol romuscular excitation and altered mental status caused
consumption. Although neuroimaging is not essential by high levels of this neurotransmitter in the central and
for the diagnosis, its important to the exclusion of other peripheral nervous systems. With the substantial increase
diagnoses, and corroborates the hypothesis of WE: acute in the use of antidepressants worldwide, serotonin syn-
diencephalic and periventricular lesions in the III and drome has become common in recent decades. Due to
IV ventricles, and atrophy of nipple bodies are specifics its severity and high correlation with elevated levels of
findings. In the suspicion of the diagnosis, patients should the serotonin, it can and should be avoided.
receive immediate administration of thiamine, preferably
intravenously. Given the unavailability in the service, it
had been performed IM. Final Comments: The acute Miscelânea
evolution of mental confusion or ataxia should have WE
as a differential diagnosis, considering the risk factors for
vitamin B1 deficiency. Despite being a clinical diagnosis,
it continues to be underdiagnosed, even though it is a
treatable and reversible neurological emergency, early
non-recognition can result in unfavorable outcomes:
coma and death.

Miscelânea

95
XXX Congresso Brasileiro de Neurologia

TL 1105906 TL 1105907 TL 1106020

A RARE CASE OF EXPANDING VIRCHOW PINEOCYTOMA PRESENTING AS A WERNICKE’S ENCEPHALOPATHY DUE TO


ROBIN SPACES MIMICKING CYSTIC TUMOR PITUITARY ADENOMA IN A YOUNG WOMAN: ACUTE PANCREATITIS
AND CAUSING HYDROCEPHALUS A CASE-BASED UPDATE
Amanda Gadelha Prysthon, Felipe Oliveira Costa
Pedro Alberto Diogenes Saldanha Pontes, Franklin Gabriel Marinheiro Santos-Bezerra, Luís Eduardo
Hospital do Subúrbio. Salvador BA, Brazil
Freitas Tertulino, Douglas Marques Zaratini Oliveira Matos, Mateus Brasil Câmara Monteiro,
José Anchieta Rodrigues-Filho, David Elison Lima amandaprysthon@uol.com.br
UFERSA – Mossoró – RN – Brazil Silva, Espártaco Moraes Lima Ribeiro, Gerardo
freitasfranklin@hotmail.com Cristino-Filho, Paulo Roberto Lacerda Leal Case Presentation: N.L.S, 19 years old, is reported to
the ER, in Salvador – BA, on 04/23/2021, complaining of
Universidade Estadual do Ceará. Sobral CE, Brazil
Case Presentation: A 57 year women presented with pro- epigastric pain and vomiting for 30 days, with worsening
gressive imbalance and right hemiparesis progressing in gabrielmarbez@gmail.com in the last 24h of admission. She carried an outpatient
three months. Chikungunya infection 3 years before was ultrasound suggestive of acute cholecystitis. Therefore,
reported. Magnetic resonance imaging (MRI) showed Case Presentation: A 18-year-old female patient had a the possibility of acute biliary pancreatitis was consid-
cystic lesions in the midbrain and obstructive hydro- history of progressive frontal headache for about 1 year. ered. Diet restriction and partial parenteral nutrition
cephalus. The features suggested abnormal dilatation of The patient evolved with bilateral visual loss 1 month were started, with maintenance of nausea and vomit-
perivascular spaces. The lesion was partially resected, with ago, accompanied by vertigo, galactorrhea, amenorrhea ing, being then escalated to total parenteral nutrition on
good evolution of symptoms with three-year postopera- and paraparesis, with greater intensity in the left limb. 04/27. On 05/04, she developed fever, tachycardia with
tive follow-up. Discussion: Perivascular enlargements, Neurological exam revealed bilateral absence of direct leukocytosis. The assistent team prescribed Meropenem
or Virchow-Robin spaces (VRS), are seen on MR images and consensual photomotor reflexes. Brain Magnetic and Teicoplanina. Abdominal CT shows slightly hyper-
as small foci that are isointense to the CSF, like small Resonance Imaging (MRI) showed the presence of an attenuating content inside the gallbladder. On 09/05,
cystic cavities that usually surround the small arteries expansive lesion around the pineal gland, manifested she evolved with a lowered level of consciousness, GCE
and arterioles at the level of basal ganglia, the anterior with T1 hyposignal, T1 contrast-enhanced hypersignal, 8, being subjected to orotracheal intubation for airway
perforated substance and the thalamic-mesencephalic and low signal foci on T2 GRE. This lesion extends to the protection. The neurology team is called in to assess the
junction. Small Virchow-Robin spaces appear in all age quadrigeminal plate, left fornix, interior of the fourth condition. Findings of ataxia, multidirectional ophthalmo-
groups. Age, arterial hypertension, dementia, and the ventricle, left lateral ventricle and quadrigeminal cistern, paresis, nystagmus, tetraparesis and global hyperreflexia
incidental white matter, lesions were found to be signifi- causing tonsillar hernia and obstructive hydrocephalus were identified. Two cranial CT were performed without
cantly associated with large Virchow-Robin spaces In a of the sides of the ventricles and the third ventricle. After acute changes. Cranial MRI was performed, which showed
retrospective study of MR imaging. Typically, they have neurosurgical evaluation, a right ventriculoperitoneal hypersignal on T2/FLAIR and in the diffusion sequence,
no mass effect and are asymptomatic, but in our patient shunt was performed to resolve intracranial hypertension affecting the medial and posterior portion of the thalami
and in other studies showed that the perivascular spaces and collection of cerebrospinal fluid for histopathological bilaterally and symmetrically. Such findings are superim-
can expande more than usual, acting as space-occupying study, which confirmed the diagnosis of pineocytoma. posable to those observed in Wernicke’s encephalopathy.
lesions. Poirier et al reported the first case of expanding Discussion: Tumors of the pineal region are relatively The patient received IV thiamine replacement in 05/14,
lacunae in a 54-year-old woman with dementia. Surgical rare, accounting for about 0.4-1% of intracranial tumors. presenting an almost complete reversal of the clinical pre-
treatment for the hydrocephalus has produced different These tumors are generally divided into pineocytoma sentation. Discussion: Wernicke’s encephalopathy (WE)
results; Ono et al reported a case wherein cystectomy (grade I), intermediately differentiating pineal paren- is a rare condition associated with thiamine deficiency,
combined with a third ventricle-to-peritoneum shunt chyma tumors (grade II or III), papillary tumor of the characterized by neurological symptoms such as ataxia,
improved the symptoms and decreased the size of both pineal region (grade II or III), and pineoblastoma (grade nystagmus, tremors, paresis and cognitive impairment.
ventricles and cysts. Final Comments: Virchow-Robin IV). Pineocytomas on MRI are usually smaller than <3 cm, Although very associated with chronic alcoholism, it is
spaces are typically considered incidental but can be well defined, iso or hypointense on T1 and hyperintense present in 12.5% of the autopsies of addicts, the condition
symptomatic in rare cases. The typical scan character- on T2. They usually do not seed cerebrospinal fluid and is also associated with situations of malabsorption, poor
istic and location of Virchow Robin spaces can help to have slow growth associated with a good prognosis. The dietary intake, increased metabolic requirement or in sit-
differentiate from cystic mass lesions. However, despite signs and symptoms depend on the tumor grade and its uations of accentuated loss of thiamine (hemodialysis).
advanced MRI techniques, sometimes it is still difficult to location, being mainly due to cranial hypertension due However, other less common etiologies are described,
differentiate VRS from some cystic brain tumors. to obstructive hydrocephalus secondary to compression such as prolonged fasting associated with other patholo-
of the midbrain roof. In this sense, vomiting, behavioral gies, as acute pancreatitis. The diagnosis of WE is difficult
changes and visual disturbances are also observed, which to confirm, and if untreated, patients progress to coma
Miscelânea can characterize Parinaud’s syndrome. Galactorrhea and and death. Fortunately, IV administration of thiamine is
amenorrhea are not usually associated with pineocyto- safe, simple, inexpensive, and effective. Diagnostic tests,
mas. However, they may be present rarely when there is therefore, should not delay treatment. Final Comments:
coexistence with pituitary adenomas in bifocal tumors. It is essential to prevent thiamine deficiency in cases
of acute pancretitis, as well as early recognition of the
clinical picture.
Miscelânea

Miscelânea

96
XXX Congresso Brasileiro de Neurologia

TL 1106042 TL 1106053 TL 1106161

CLIPPERS: CASE PRESENTATION METASTATIC CERVICAL CARCINOMA WITH MEDULAR COMPRESSION SYNDROME OF
EVOLUTION TO ENCEPHALIC MENINGEAL METASTATIC ORIGIN
Debora Leticia Correia, Thamilyn Yoshizaki
Saruwatari, Willian Medina Guimaraes, Sebastiao CARCINOMATOSIS
Raíssa Barreto Vieira Soares, Dkaion Vilela de Jesus,
Carlos de Sousa Oliveira, Maria Teresa Fernandes Carolina Matté Dagostini, Sabrina Busnello, Alessandra Braga Cruz Guedes de Morais, Isadora
Castilho Garcia, Guilherme Menezes Mescolotti Nathalia Luiza Mattes Heck, Aline Caldart Tregnago Garcia Carneiro Kriunas Severino, Lara Cristina
Rocha Alvarenga, Fernando Elias Borges
Hospital Regional de Presidente Prudente. Universidade de Caxias do Sul. Caxias do Sul RS,
Presidente Prudente SP, Brazil Brazil Santa Casa de Misericórdia de Goiânia. Goiânia GO,
Centro de Patologia Médica – Caxias do Sul – RS – Brazil
deboraleticiacorreia@gmail.com
Brazil Hospital de Urgências de Goiânia. Goiânia GO, Brazil
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Case Presentation: M.S.M., male, 33 years old, was admit- carolmdagostini@hotmail.com Hospital Geral de Goiânia. Goiânia GO, Brazil
ted in January 2022 with a report of gait impairment for 4
years, worsening in the last year, associated with imbal- Case Presentation: Female patient, 38 years old, smoker, dkaion@hotmail.com
ance. He also presented, in the last month, blurred vision, diagnosed with stage IIIB squamous cell carcinoma of the
diplopia, and slurred speech. On physical examination, he cervix in 2019, underwent radiotherapy until February Case Presentation: CES, male, 62 years old, painter,
presented with a global strength grade of 4+, all reflexes 2020 and after started brachytherapy. During the fol- smoker, alcoholic, previously functional and indepen-
exalted, and ataxic gait. At the time of admission, a new low-up, the patient presented progression of the dis- dent. He reported low back pain for a month. Paresthesia
brain MRI was made with pons lesions, with a salt and ease to the peritoneum, lung, ribs and dorsal spine, and in the lower limbs that evolved a day after to symmetrical
pepper aspect, suspected of inflammatory disease, being underwent to palliative chemotherapy with carboplatin paraplegia associated with significant change in sensitiv-
opted for pulse therapy with corticosteroids and plasma- and plalaclitaxel. In September 2020, had a Hospital ity and urinary retention. Admitted to neurology already
pheresis, with clinical and radiological improvement. admission due to severe holocranial headache, fatigue, with deficit. Neurological examination showed paraple-
Discussion: Chronic lymphocytic inflammation with dysuria, diarrhea, pain in the lumbar region and episodes gia, urinary and fecal retention, bilateral biceps, patellar
steroid-responsive pontine perivascular enhancement of fever. Cranioencephalic MRI revealed the presence and achilles hyperreflexia. Tromner and Hoffman present
is an inflammatory disease of the central nervous system of multiple contrast-enhanced nodular lesions in the bilaterally. Abdominal skin reflex absent. Plantar cuta-
defined in 2010 by Pittock, who described the case of 8 skullcap, compatible with metastatic implants, some neous reflex in extension on the left limb. Apalesthesia,
patients with similar clinical, radiological, and pathologi- causing erosion of the inner bone plate, with thickening thermal, tactile and painful anesthesia with a T6 level.
cal features who were responsive to corticosteroids. For the and diffuse pachymeningeal enhancement, more evi- Magnetic resonance imaging of the brain, cervical and
diagnosis of CLIPPERS is necessary that the patient pres- dent in the right frontal region, which could be related thoracic spine were performed, which showed foci of signal
ents clinical alterations like progressive subacute ataxia to pachymeningeal carcinomatosis. Also, the presence of alteration in the facets and right pedicle of C3, vertebral
and diplopia and symptoms related to brainstem involve- minute nodular images with hypersignal in FLAIR and bodies of C6 and C7, with a secondary neoplastic aspect.
ment. Associated with MRI alteration with multiple salt weak contrast enhancement, located along the leaves of Multiple lesions diffusely distributed in the thoracic ver-
and pepper sign in pons, with or without propagation to the middle third of the right cerebellar hemisphere, may tebrae, especially long expansive and infiltrative lesions
cerebellar peduncles and cerebellum. Histopathologically be related to leptomeningeal or intraparenchymal corti- involving the vertebral bodies and posterior elements
presenting a perivascular lymphohistiocytic infiltrate of co-subcortical implants. In addition, it was detected small from T4 to T8, with an important soft tissue component
the white matter, with a predominance of CD3 and CD4 hyperintense T2/FLAIR images in the supratentorial white at the T5-T6 level. T5-T6 decompressive laminectomy
lymphocytes. It´s possible to make a definitive diagno- matter, with no expansive effect, diffusion restriction or was performed with biopsy of the surgical resection and
sis only with clinical and image characteristics, if other contrast enhancement. Other imaging exams revealed the malignant epithelial neoplasia with clear cell components
etiologies are excluded. Final Comments: CLIPPERS is extent of metastatic involvement to the liver, adrenals and was visualized. An abdominal tomography was also per-
a rare disease with a high morbidity rate, with a high risk mandible. Treatment was ceased due to terminality of the formed, which showed a suspicious-looking lesion for
of complications and disability when not diagnosed and patient, and was kept in exclusive comfort. The patient primary urothelial neoplasm in the right ureter. An inves-
treated at the right time, so it is important to know the died a few days later. Discussion: There are less than 22 tigation was also performed for prostate and lung cancer,
pathology and make a diagnosis to offer the best treat- articles published in the literature that describe menin- both negative. Informed consent was obtained from the
ment to the patient. geal carcinomatosis related to gynecological cancers. It is patient for a case presentation Spinal cord compression
estimated that the incidence of meningeal carcinomato- syndrome is a condition that can be caused by different
sis in patients with cervical cancer is 0.03%. Patients with etiologies that cause spinal canal stenosis. It may be the
Miscelânea initial manifestation of 20% of metastatic neoplasms.
meningeal carcinomatosis have a poor prognosis due to
the advanced stage of the primary tumor. Literature data The characteristic manifestations of this syndrome are
indicate that the median survival after diagnosis ranges relatively symmetrical motor deficits, urinary retention
from 10 days to 26 weeks. Final Comments: Meningeal or incontinence, loss of sensation, and includes signs of
carcinomatosis related to cervical carcinoma is rare. hyperreflexia. Some tumors tend to metastasize to spe-
However, this pathology should be considered in patients cific regions of the spine. Renal, gastrointestinal or pros-
with primary gynecological tumors who present a clinical tate tumors generate metastases to the lower regions of
picture suggestive of CNS involvement, since palliative the thoracic spine and lumbosacral spine. The patient
therapies can be instituted to improve the patient’s quality in question had a spinal cord compression syndrome,
of life and delay neurological deterioration. mainly in the thoracic region, with a primary site in the
urinary system.

Miscelânea
Miscelânea

97
XXX Congresso Brasileiro de Neurologia

TL 1106237 TL 1106283 TL 1106326

NEUROSARCOIDOSIS PRESENTING IDIOPATHIC POLYARTERITIS NODOSA AND A RARE CASE OF PARANEOPLASTIC


AS “ACUTE” NORMAL PRESSURE NEUROLOGICAL MANIFESTATIONS: CASE LONGITUDINALLY EXTENSIVE
HYDROCEPHALUS: A CASE PRESENTATION PRESENTATION MYELOPATHY, SECONDARY TO MALIGNANT
PHEOCHROMOCYTOMA
Giovana Barros e Silva Ribeiro, João Vitor Gerdulli Amanda Leticia Andre, Tatiane Lopes Alves de Jesus
Tamanini, Marco Tulio Medina Montoya, Izadora Arroyo, Arthur Coelho Moura Marinho, Nathalye Eduardo Bernardo Chaves Neto, Marta Rodrigues
Vieira Bolzani Lopes Lima, Ana Carolina Piauilino Fernanda Pedroso Dircksen, Marcelo Simplicio de Carvalho, Ana Paula de Góes Louly Bustamante,
Santos Falcão, Sabrina Vechini Gouvêa, Marcio Luiz Carvalho, Lorena Fernandes Kronbauer, Breno Pablo Henrique da Costa Silva, Caroline Santos
Figueredo Balthazar William Correa dos Santos, Walton Luiz Del Tedesco, Eneas, Nayara da Costa Andrade, Déborah de Castro
Damacio Ramon Kaimen Maciel Ferreira de Oliveira, Isadora de Castro Ferreira de
Universidade Estadual de Campinas. Campinas SP,
Oliveira, André Gustavo Fonseca Ferreira, Hiram
Brazil Irmandade Santa Casa de Londrina. Londrina PR,
Fernandes Soares
Brazil
giovanabsribeiro@gmail.com
Hospital de Base do Distrito Federal. Brasília DF,
amanda_leeticia@hotmail.com
Brazil
Case Presentation: A 68-year-old woman, with a previous
medical history of diabetes, hypertension and hypothy- Case Presentation: RBR, 53 years old, with intense and eduardochavesmed@gmail.com
roidism, was brought to the emergency department with pulsating headache, vertigo, tinnitus, hearing loss on the
complaints of bilateral leg weakness, cognitive impairment left, low bilateral visual acuity and behavioral change for Case Presentation: Male patient, 60 years old, with
and urinary incontinence that started twenty days prior to 7 months. Past history of an episode of joint pain, fever hemophilia type A diagnosed at the age of 13 years old
admission. The patient also reported unmeasured daily and nonspecific hepatitis for 4 years, without diagno- and HCV diagnosed in 1983, with cure control in 2012.
fever over the preceding two weeks, a significant uninten- sis and using corticosteroids since then, in addition to 50 days ago from admission, he noticed painless inguinal
tional weight loss and anosmia over the past two years. At arterial hypertension and dyslipidemia. Neurological lymph nodes. After 15 days, he began dysesthesia in the
the initial evaluation, a mild loss of strength in the lower examination was lethargic, strength and sensitivity were cervical region and upper limbs. He evolved with severe
limbs and significant gait apraxia were observed. Initial preserved, grade 3 osteotendinous reflexes globally, low neck pain, dorsal irradiation, worsening of movement
head CT showed dilatation of supratentorial ventricular bilateral visual acuity, without other changes. On general and motor symptoms, associated with nausea, vomit-
system and transependymal effusion of cerebrospinal physical examination, the presence of hyperemic and scaly ing and hiccups. 10 days before admission, he started
fluid (CSF). CSF analysis demonstrated a cellularity of lesions on the lower limbs. Cranial MRI showed lesions sensory-motor symptoms in the lower limbs predomi-
12 leukocytes/mm3 (100% lymphocytes), increased total with hypersignal on T2/FLAIR in the right superior tem- nant asymmetric to the left and, after 4 days,presented
protein (63mg/dL) and a low glucose level (CSF/plasma poral gyrus, ipsilateral superior parietal lobe, with a gran- constipation and urinary incontinence. After 5 days, he
ratio of 52%). The MRI showed signs of leptomenin- ulomatous aspect, suggesting an inflammatory nature. In presented restriction on ambulation and loss of muscle
geal and choroid plexus inflammation, with obstructive cerebrospinal fluid, lymphocytic predominant pleocytosis, strength in the lower limbs and was Hospitalized. On
hydrocephalus and intracranial hypertension. Following normal glucose and protein, and negative culture. Also, admission neurological examination showed grade V
the investigation, a chest CT demonstrated paratracheal HIV, Syphilis, Hepatitis and other viral infections were strength in the upper limbs, grade III in right lower lim-
and subcarinal lymphadenopathy and a biopsy was per- excluded. In rheumatological and vascular research, b,grade II in left lower limb (preserved toe dorsiflexion,
formed. The fungal, bacterial and mycobacterial cultures an increase in ESR, with no other changes. Finally, in a grade IV plantar flexion of the toes).Babinski sign present
of CSF and lymph node were negative and pathologic biopsy of the lower limb lesion, evidence of mixed inflam- bilaterally. Laboratory tests showed: LDH 795; Anti-HVC
examination of the lymph node revealed noncaseating matory infiltrate, lymphocytic predominance with foci of 166. Normal rheumatological tests. CSF: colorless, cell 1,
granulomas without evidence of malignancy. A diagno- aggression in the vascular wall, with thickened central proteins 119 mg/dl; missing oligoclonal bands, anti-Aqua-
sis of neurosarcoidosis with obstructive hydrocephalus medium-sized vessels, with fibrosis, surrounded by the porin 4 negative. Total abdominal CT: Retroperitoneal
was made and the patient was started on steroids (1mg/ inflammatory infiltrate, diagnosing polyarteritis nodosa and pelvic lymph node enlargement.Nodule in the body
Kg/day of prednisone). After the beginning of therapy, (PAN). Discussion: PAN is a vasculitis of medium-sized of the left adrenal gland. Neuroaxis MRI: Capture annular
the patient presented significant improvement of the arteries and rarely of small vessels. It may be idiopathic or lesion, encapsulated with perilesional edema in the left
symptoms, returned to walk independently and there secondary to hepatitis and malignancies. Of multisystem frontotemporal region. Cervical spinal cord injury with
was no need of neurosurgical procedures to drain the CSF. involvement, it can cause renal, coronary and gastrointes- irregular contrast uptake, bulb-sacral enhancement.
Discussion: Sarcoidosis is a multisystemic granulomatous tinal artery disease, in addition to neurological disease, Biopsy left inguinal lymph node tissue with immunohis-
disorder characterized by the formation of noncaseat- coursing with mononeuritis, peripheral neuropathy and tochemistry: malignant, metastatic pheochromocytoma.
ing granuloma in various organ systems. Neurological involvement of the central nervous system, more rarely. During Hospitalization, he evolved with unprecedented
involvement may result in peripheral or central nervous In the disease, the occlusion or rupture of the inflamed quadriparesis and seizures, being submitted to orotracheal
system (CNS) manifestations. Gait disorders and cognitive arteries generates ischemia or hemorrhage. Its diagnosis intubation and transferred to the ICU. It was decided to
decline are less common forms of presentation. Here, we is clinical associated with rheumatological exams of exclu- perform pulse therapy with methylprednisolone 1g/5 days,
report an unusual presentation of the disease. A patient sion, serological exams to identify possible causes, and skin with no clinical response. Discussion: Paraneoplastic
with a previously preserved functionality presented a rap- biopsy demonstrating infiltrate of inflammatory cells in longitudinally extensive myelopathy is a rare event, more
idly progressive cognitive impairment, gait apraxia and vessels and fibrinoid necrosis, in addition to angiography associated with neoplasms of bones, liver, lymph nodes
urinary incontinence. Given the challenging diagnosis that may show saccular aneurysms or stenosis in blood and lung. Literary evidence of malignant pheochromo-
of the case and association with obstructive hydroceph- vessels. Final Comments: The association of systemic cytoma with intramedullary involvement is scarce and
alus, clinicians should be aware of this condition. Final and neurological vascular diseases is common. Thus, it is constitutes a diagnostic and therapeutic challenge. In
Comments: The case presented demonstrates an unusual important to correlate neurological manifestations with this case, in addition to extensive spinal cord involve-
association of neurosarcoidosis and a rapidly progressive those of other organs, as in the present case, being able ment, brain involvement is evident. Final Comments:
presentation of cognitive impairment, gait apraxia and to direct and reach decisive diagnoses. The case in question is notable for the rarity of the clini-
urinary incontinence. cal presentation. The unfavorable evolution is due to the
extent of the lesion and concomitant brain involvement,
Miscelânea with no previous reports of association with malignant
Miscelânea pheochromocytoma in the literature.

Miscelânea

98
XXX Congresso Brasileiro de Neurologia

TL 1106357 TL 1106370 TL 1106407

SMART SYNDROME: A CASE PRESENTATION EARLY INFLAMMATORY CHANGES IN THE MELKERSSON-ROSENTHAL SYNDROME: A
BRAINSTEM AFTER RADIOSURGERY: A CASE CASE PRESENTATION
Bruna Gutierres Gambirasio, Leonardo de Sousa
Bernardes, Andreia Martini Pazini, Thays Neri PRESENTATION
Patrick Emanuell Mesquita Sousa Santos, Pedro
Andrade, Murillo Silva Catito, Gabriel Novaes de Ana Clara Guilherme Rodrigues, Anderson Machry Pozzobon, Helio Aquaroni Farão Gomes,
Rezende Batistella, Adrialdo José Santos Rodrigues Brandão de Paiva, Sheila Ferreira Pessoa, Ana Beatriz Marangoni Baston, Tarcísio Nunes
Thainá Gonçalves Tolentino de Figueiredo, Anselmo Alvarenga, João Vitor Mortari Lisboa, Igor Oliveira
Escola Paulista de Medicina – UNIFESP, São Paulo
de Jesus Santos, Aroldo Luiz da Silva Bacellar Fonseca, Danielle Patricia Borges Margato,
SP, Brazil
Fernando Coronetti Gomes Rocha
brunagambirasio@hotmail.com Hospital São Rafael. Salvador BA, Brazil
Hospital de Clínicas da Faculdade de Medicina de
clariguilherme@gmail.com Botucatu – UNESP. Botucatu SP, Brazil
Case Presentation: A 34-year-old man had a progressively
developed headache and focal seizures. CT brain scan Case Presentation: A 36-year-old woman, without previ- pedro_machry_pozzobon@hotmail.com
disclosed a left frontal mass, with gadolinium enhance- ous comorbidities, was diagnosed with a right petroclival
ment. The patient underwent a successful subtotal lesion meningioma, who underwent radiosurgery. 30 days after Case Presentation: A 44-years-old woman presented to
resection, followed by whole-brain radiotherapy for 3 the procedure, she was admitted with initial complaints our service due repeted episodies of right Bell’s palsy. The
months (60Gy). Pathological analysis revealed an ana- of vertigo, followed by sensory changes on left hemibody begin of symptoms was five years ago, with right facial
plastic oligoastrocytoma, according to the 3rd WHO and diplopia. On neurological examination, there were nerve palsy diagnosed as Bell’s plasy and treated with pred-
classification of central nervous system tumors. Due to right horizontal gaze-evoked nystagmus, with binocular nisone and aciclovir, showing gradual improvement. One
great post-radiotherapy brain MRI status, the patient was diplopia, right sensorineural hypoacusia, paresis of the year later, she developed facial disesteshya in right face,
put under conservative treatment, with control images palate elevation on the left, and asymmetry on shoulder associated with right facial edema. In the following 3 years,
every six months. The four following brain MRIs revealed elevation, worse on the left. Motor strength was preserved she had two more episodes of facial nerve palsy, associated
important gadolinium enhancement in addition to cortical on the four limbs. There was tactile and painful hypoes- with ipsilateral facial edema and tongue fissures, referred
gyriform enhancement, although the patient remained thesia on the left hemibody, with deep senses preserved. to our service. Brain MRI were normal, and laboratory
asymptomatic. Two years after the initial diagnosis, the There was no axial or appendicular ataxia, but a shift to tests showed reagent antinuclear antibody (1/320), with
patient presented sudden transient right-sided weakness. the left on Fukuda gait. CSF disclosed a slight increase no other biomarkers present and serological tests for the
A new brain MRI showed diffuse gadolinium enhance- in cellularity and protein. MRI showed a hypersignal infectious diasease negative. CSF showed 16 cells, 65 pro-
ment. The patient underwent a second resection and tissue on T2-weighted images of the brainstem and cerebellar teins, with negative infectious tests. Salivary gland biopsy
histopathology revealed radiation necrosis, with no signs peduncle. Pulse therapy was performed with 1g of met- unchanged. The hypothesis of recurrent facial paralysis
of neoplastic disease. He also started a daily headache of hyprednisolone for 05 days, with significant improvement due to Melkersson-Rosenthal Syndrome was raised and
pulsating quality, in association with nausea. Migraine in symptoms. Discussion: Inflammatory changes in the treatment with corticosteroids (prednisone 60mg per
prophylaxis was started, along with steroids therapy, with brainstem admit a wide differential diagnosis and may day) for two weeks was performed, without recorrence
great symptom control. Steroid therapy was necessary for be challenging. Imaging changes on MRI are common of symptoms. Discussion: Melkersson–Rosenthal syn-
the two following years, and the patient presented new after CNS radiation and can be seen as a hypersignal on drome (MRS) is a rare syndrome with unclear etiology,
clinical recurrences and worsening brain image in every T2-weighted images. However, only about 10% of patients comprised of the triad of recurrent facial nerve paralysis,
withdrawal attempt. According to Black’s criteria, the are symptomatic and presented with cranial neuropathies, nonpitting orofacial edema and fissured tongue (lingua
patient was diagnosed with stroke-like migraine attacks headache, hemorrhages, edema, epileptic seizures, radio- plicata. All the three features are present in 20–75% of
after radiation therapy (SMART) syndrome. Discussion: necrosis, among others. Radiation complications can be cases. Other neurological manifestations may be seen in
SMART syndrome is a rare late complication of radiother- acute (radiation encephalopathy), subacute (transient MRS, like migraine, facial paresthesias, tinnitus, dizziness,
apy and usually presents 12 years after the treatment. demyelination) or chronic. Radionecrosis, the most com- hypogeusia and pharyngeal neuralgias. Non-neurological
Classic symptoms are migraine attacks and spell focal mon chronic complication, especially in the brainstem, symptoms include uveitis, diverticulitis, and ulcerative
deficits, such as aphasia and hemiplegia, although some is rare and atypical presentations are even less frequent. colitis. The investigation must be exclude systemic pathol-
patients may experience seizures. Some patients also may Usually, radionecrosis occurs about 6 months to 3 years ogies, mainly sarcoidosis, Crohn’s disease, tuberculosis,
recur from clinical deficits. Risk factors are not clearly after treatment. First-line therapy is with corticosteroids. herpes and other infectious diseases. Biopsy of a region
defined but higher radiation doses may increase the Final Comments: Acute complications related to radio- with orofacial edema demonstrating noncaseating granu-
chances of developing SMART syndrome. Pathophysiology therapy are uncommon. Later complications, such as lomas supports the diagnosis of MRS. There is no specific
is still poorly understood, but some authors believe that radionecrosis, occur in about 23.1% of cases, in the first treatment for MRS. Therapy with corticosteroids leads to
radiation leads to vascular damage, which causes endo- year up to decades after treatment. Attention is required improvement in 50–80% of patients and reduced relapse
thelial dysfunction. Brain MRI is not always specific, and for this presentation reported here, in view of its wide frequency by 60–75%. However, randomized trials are
distinction from tumor progression may be difficult. No differential diagnosis and favorable prognosis after treat- lacking and frequency, dosing or duration are not estab-
treatment has been established, but some cases report the ment with corticosteroids. lished. Other options described are NSAIDs, antibiotics
use of steroids and antiplatelet agents. Final Comments: (like doxycycline and dapsone) and immunosuppressants
Our case presentations an early development of SMART (methotrexate, infliximab). Intralesional triamcinolone
syndrome. It is a rare complication of growing importance, Miscelânea acetonide injection also are used in local edema. Final
especially because cancer patients are surviving much Comments: MRS is a cause of recurrent facial nerve palsy,
longer, and the symptoms can impact on quality of life. and etiologies such as multiple sclerosis and infections
should be investigated. In idiopathic cases, steroids are
often used. More studies are needed to understand and
Miscelânea manage these cases.

Miscelânea

99
XXX Congresso Brasileiro de Neurologia

TL 1106433 TL 1106441 TL 1106459

ADULT DIFFUSE HIGH-GRADE BRAINSTEM REVIEWING AND RECALLING THE KEY VITAMIN B12 DEFICIENCY ASSOCIATED
GLIOMA INDUCED BY BRAIN RADIOTHERAPY ROLE OF NEUROIMAGING IN WERNICKE WITH ATYPICAL BRAIN MRI FINDINGS
IN CHILDHOOD, A CASE PRESENTATION ENCEPHALOPATHY
Isabel Oliveira Santos, Camila Rodrigues
Matheus Gonçalves Maia, Amanda Alencar Carolina Ferreira Colaço, Camila Lorenzini Tessaro, Nepomuceno, Gabriella Corrêa Dousseau, Heitor
Cavalcanti Carneiro Cunha, Augusto Obuti Saito Felipe Trevisan Matos Novak, Caio César Diniz Nunes Oliveira Sento-Sé Neto, Filipe Brito
Disserol Porto, Lorrane Campidelli Arthuzo, Pedro Souza
Santa Casa de Misericórdia de São Paulo, São Paulo Domingues, Sônia Maria Dozzi Brucki, Maria Sheila
SP, Brazil Hospital de Clínicas da Universidade Federal do Guimarães Rocha, Júlian Letícia Freitas
Camargo Cancer Center, São Paulo SP, Brazil Paraná. Curitiba PR, Brazil
Casa de Saúde Santa Marcelina. São Paulo SP,
matheusgmaia1@gmail.com carolinafcolaco@gmail.com Brazil

Case Presentation: 33-year-old female, in 2002, at age 13, Case Presentation: AMC, 21 years old, 8 weeks preg- isabelo.Santos.1@gmail.com
was diagnosed with pineal dysgerminoma. The patient nant, previously healthy. Admitted to the gynecological
underwent surgical resection of the lesion, followed by emergency care due to repeated vomiting, being diag- Case Presentation: A 68-year-old woman was referred to
chemotherapy and radiotherapy. In 2003, this patient nosed with hyperemesis gravidarum. On the third day our Hospital complaining of progressive and symmetri-
had tumor recurrence and was submitted to a new sur- of Hospitalization, the neurology team was called due cal paraparesis, paresthesia, and gait imbalance over the
gical resection followed by chemotherapy and reirradi- to mental status change. The neurological examination last five months, currently confined to a wheelchair. She
ation. After achieving complete response, the patient revealed the presence of confusion, multidirectional oph- had no previous medical history or noteworthy dietary
remained in clinical follow-up for 18 years. In May 2020, thalmoparesis and ataxia with significant gait alteration. restrictions. On neurological examination, she had severe
she evolved with altered mental status associated with It was decided to measure serum vitamin B1 (thiamine) lower limb weakness, spasticity, and generalized brisk
diplopia and oculomotor nerve paresis. Cranial MRI was and request magnetic resonance imaging (MRI) and anal- deep tendon reflexes with a bilateral Babinsky sign. The
performed and showed an expansive lesion in the right ysis of the cerebrospinal fluid. The diagnosis of Wernicke patient also had reduced light touch and vibration sense
thalamo-mesencephalic region with contrast enhance- encephalopathy was confirmed due to the finding of vita- in both legs and upper limbs. Laboratory testing revealed
ment. In July 2020, a biopsy of the midbrain lesion was min B1 deficiency and, mainly, the presence of hypersig- reduced levels of B12 vitamin and on brain MRI it was
performed and confirmed the diagnosis of high-grade nal of the mammillary bodies and lesions of the medial noticed FLAIR and T2 hyperintensities in periventricu-
glioma. Immunohistochemical analysis demonstrated thalamus and of the walls of the third ventricle on MRI. lar and centrum semiovale white matter, bilateral globus
positivity for expression of GFAP, ATRX and Ki67 (40% The patient showed significant clinical improvement after pallidus, cerebral and middle cerebellar peduncles, and
of the tumor cells), while the expression of IDH1, CD34, thiamine replacement. Discussion: Wernicke encepha- in the brainstem. The described lesions had no diffusion
neurofilament, synaptophysin, p53 and H3K27M were lopathy can occur as a rare complication of hyperemesis restriction or contrast enhancement. These findings were
negative. Diffuse high-grade glioma, H3-wildtype and gravidarum. MRI plays an important role in elucidating therefore compatible with vitamin B12 deficiency and
IDH-wildtype was the definitive diagnosis. Radiotherapy the diagnosis as it presents classic alterations, with the the patient was referred for further investigation in our
was performed initially and later this patient had disease mammillary bodies, medial thalamus and periventricular outpatient clinic. Discussion: Vitamin B12 deficiency
progression and received temozolamide. Currently, this region of the third ventricle being the typically affected can be responsible for hematological, gastrointestinal,
patient is in palliative care. Discussion: Aside from genetic regions. Because it is an acute neurological disease that psychiatric, and neurological symptoms. The causes of
factors, the main risk factor already established for the results from thiamine deficiency, the prognosis of patients depletion are numerous and include malabsorption due to
development of glioma is previous exposure to ionizing depends on the time from the onset of symptoms and GI tract abnormalities, autoimmune diseases, and dietary
radiation of the central nervous system (CNS), as occurs the start of supplementation of this vitamin. When the restrictions. The most common neurological manifesta-
from therapeutic radiation therapy for childhood brain diagnosis and treatment are delayed, Korsakoff psycho- tions are paresthesia, progressive symmetrical weakness
tumors or leukemia. The interval between irradiation sis or death may occur. Final Comments: Neuroimaging and gait disturbances. The disease may progress to spastic
and glioma development varies from five years to several plays a central role in the early diagnosis of this prevalent paraplegia with cognitive and mood alterations. Spinal
decades. In this clinical case, the etiology of the glioma is pathology, which can be reported in up to 36% of patients cord MRI often shows the altered signal in its lateral and
probably related to the brain radiotherapy that this patient with acute mental confusion and lead to significant neu- posterior columns. Less commonly there are brain white
underwent for the treatment of pineal dysgerminoma rological morbidity if not properly managed. matter and optic nerve involvement. Bilateral globus pal-
during childhood. Final Comments: Radiation induced lidus lesions are an even rarer presentation, described in
glioma is relatively rare but well described in the litera- the presented case. Treatment is based on vitamin B12
ture. Unfortunately, radiation-induced gliomas promote Miscelânea supplementation and neuropsychiatric improvement can
high morbidity and mortality in patients. In this way, the be seen after months, however, some neurologic findings
rational use of brain radiotherapy especially in children may be irreversible. Final Comments: In this case there
and the development of new technologies that reduce the are classical neurological signs compatible with vitamin
risk of second brain neoplasms induced by radiotherapy B12 deficiency such as combined subacute degeneration,
is extremely important. hyperreflexia and peripheral neuropathy. Nevertheless,
we present uncommon imaging finding poorly reported
in the literature, more specifically bilateral hyperinten-
Miscelânea sities in the globus pallidus.

Miscelânea

100
XXX Congresso Brasileiro de Neurologia

TL 1106461 TL 1106500 TL 1106512

ISCHEMIC MYELOPATHY AND A RARE CASE OF ALCOHOL-INDUCED REPORT OF FORAMEN MAGNUM


HEMATOMYELIA: A CASE PRESENTATION OSMOTIC DEMYELINATION SYNDROME MENINGIOMA
João Gabriel Dias Brasiliense Frota, Sarah Diogenes Isadora Castro Ferreira Oliveira, Déborah Castro Alexandre Federisi, Alice Berto Canova, Larissa
Alencar, Matheus da Costa Guedes, Gabriela Joca Ferreira Oliveira, Pablo Henrique da Costa Silva, Maria Davoglio, Tomásia Oliveira de Holanda
Martins, Milena Sales Pitombeira, Francisco José Caroline Santos Eneas, Nayara da Costa Andrade, Monteiro Frezatti, Rodrigo Siqueira Soares Frezatti,
Arruda Mont’Alverne, Ana Vitória Gabriel Diógenes, Ana Paula de Góes Louly Bustamante, Eduardo Iverson Silva Correia
Fernanda Martins Maia Bernardo Chaves Neto, Wagner Afonso Teixeira,
André Gustavo Fonseca Ferreira, Ronaldo Maciel Centro Universitário Estácio Ribeirão Preto. Ribeirão
Hospital Geral de Fortaleza. Fortaleza CE, Brazil Dias Preto SP, Brazil
Universidade de Fortaleza. Fortaleza CE, Brazil
Universidade Federal do Piauí. Teresina PI, Brazil alexandre_federisi@hotmail.com
João gfrota@gmail.com Hospital de Base do Distrito Federal. Brasília DF,
Brazil Case Presentation: Patient, female, 43 years old, eval-
Case Presentation: A 28-year-old woman presented uated with a complaint of neck pain and headache that
upper thoracic spine pain after supposed trauma in a castro_isadora@hotmail.com started seven months before. Her neck pain got worse after
soccer game, evolving after 03 days with paresthesia 3 months, leading her to use a lot of analgesics, without
of hands and tongue with evolution in 40 minutes to Case Presentation: Male, 53 years old, is admitted to the improvement. She was evaluated in other service and a
tetraparesis, lowered level of consciousness and acute emergency room with a subacute picture of dysarthria, brain CT was performed, without alterations. The con-
respiratory failure requiring airway support.The patient central facial paralysis on the left and hemiparesis on dition evolved progressively with pain and weakness in
underwent a study of the cerebrospinal fluid (CSF) which the left with evolution of one week. The clinical condi- the Right Upper Limb (RUL) and Left Upper Limb (LUL),
showed pleocytosis with lymphocytic and neutrophilic tion began a day after an episode of alcoholic libation. in addition to low back pain, pain like shocks in the Left
predominance associated with normal levels of protein Previously hypertensive, he made continuous use of tel- Upper Limb (LLL) and difficulty in evacuating. On phys-
and glucose. The diagnostic investigation was comple- misartan + amlodipine and chlorthalidone. According ical examination, cranial nerve pair testing was obtained
mented with magnetic resonance imaging (MRI) of the to family members, he consumed alcohol excessively on without abnormalities. The strength examination (using
skull and cervical spine, which presented longitudinally weekends, and after family deconjuncture this consump- MRC Scale graduation) showed grade 4 in RUL and grade
extensive myelopathy in addition to internal hemorrhagic tion was exacerbated. In the neurological examination, 5 in LUL, grade 4 proximal in Right Lower Limb (RLL)
foci and presence of hematomyelia in the pons and in he was vigil, oriented in time and space, with severe dys- and grade 5 distal in RLL and LLL. She had hypoesthesia
the anterior portion of the cervical spine. A hypothesis arthria, facial paralysis of central pattern to the left, devi- on the right side of the body and her deep osteotendin
of acute longitudinally extensive transverse rhomboen- ation of the uvula to the left, deviation of the ispilateral reflexes were exalted with bilateral Babinski sign. Brain
cephalomyelitis of probable immune-mediated etiology soft palate. He evolved with an asymmetric tetraparesis, and cervical MRI were requested, revealing an expansive
was raised and pulse therapy was started with 1g meth- with degree muscle strength (DMS) 2 in the left dimidium lesion in the craniocervical transition, compressing the
ylprednisolone for 05 days, and, due to an unsatisfactory and DMS 4 in the right upper limb and DMS 2 in the right spinal cord and the medulla, suggestive of a foramen mag-
response, complemented with 05 sessions of plasmapher- lower limb. Hoffman is present bilaterally. Slight tactile num meningioma prompting neurosurgery evaluation.
esis, with imaging improvement afterwards, but without and painful hypoesthesia on the left. Right dysmetria in A complete tumor ressection was performed, leading to
clinical response. She underwent cervical and thoracic the index-nose test. The cerebrospinal fluid was clear and a great clinical improvement. Discussion: Meningiomas
spinal cord angiography that showed dissection of the colorless, with an opening pressure of 22cmH2O, with originating from the foramen magnum (FMM) are uncom-
left vertebral artery in the V2 cervical segment and failure normal cellularity, normal protein and glucose value. mon, being diagnosed in only 1.8% to 3.2% of intracra-
of opacification of the anterior spinal artery in the pro- Contrast-enhanced nuclear magnetic resonance imaging nial meningiomas, with a higher prevalence in females.
jection of the odontoid process suggestive of occlusion. (MRI) of the skull revealed the presence of a lesion in the They present a slow growth pattern with insidious clinical
We reached the conclusion of an ischemic lesion with brainstem, especially in a pons characteristic of pontine symptoms and late diagnosis. They are classified as ven-
hemorrhagic transformation, secondary to dissection myenolysis, with hyposignal on T1-weighted sequences, tral, ventrolateral and dorsal, which is essential for the
of the left vertebral artery and probable occlusion of the hypersignal on T2-weighted sequences and Flair, mea- conduct in an integrated manner. The clinic of patients
anterior spinal artery. Discussion: Hematomyelia is a suring 3.1x3x2cm, bilaterally without circum edema. with foramen magnum tumors is diverse, encompassing
very rare intraparenchymal spinal cord hemorrhage. Intense contrast enhancement. Other similar lesions in headache, neck pain, upper limb pain, weakness and low
The most common cause is trauma, followed by vascular splenium of the corpus callosum on the right and right back pain. Magnetic resonance imaging (MRI) is the gold
malformation, coagulopathy, neoplasm, and iatrogenic occipital lobe. Discussion: The most common cause of standard test for diagnosis. Surgical treatment is indicated
procedures. A hemorrhagic transformation after spinal Osmotic Demyelination Syndrome (ODS) is the very rapid for growing and symptomatic tumors. Final Comments:
cord infarction can sometimes occur, caused by mech- correction of chronic hyponatremia. Cases of alcohol-re- The FMM has a variable clinical presentation, making its
anisms such as arterial dissection, atherosclerosis and lated non-hyponatraemic osmotic demyelination result diagnosis challenging. The physical examination can be
fibrocartilaginous embolism. Patients may present with from glycogen depletion and thiamine deficiency that an important clue when bilateral signs of upper motor
acute severe neck or back pain at onset followed by rapid limit the ability to adapt brain to any osmotic stress. The neuron lesion are present, as it is an important differen-
severe myelopathy deficits The hematomyelia treatment diagnosis is initially suspected from clinical symptoms tial diagnosis of spastic paraparesis. Clinicians must be
consists of cervical or thoracic laminectomy followed by and is confirmed by magnetic resonance imaging of the aware that asymmetric sensory abnormalities, as seen in
durotomy and myelotomy, after which the hematoma cav- brain. Final Comments: The authors describe a rare this case, can happen as a result of non-uniform tumor
ity is evacuated and irrigated. Final Comments: Vascular case of pontine and extra-pontine ODS not associated growth and partial compression of spinotalamic tract.
myelopathies are important clinical entities due to its acute with hyponatremia. The causal factor in question was
and progressive course. A quick diagnosis can potentially alcohol libation and the clinical diagnosis was possible
result in a life-saving therapy and prevent lasting neuro- from the clinical condition and the exclusion of other Miscelânea
logic damage and death. differential diagnoses, as well as the performance of the
neuroimaging examination, which allowed the identifi-
cation of the lesions.
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XXX Congresso Brasileiro de Neurologia

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INCOMPLETE CAUDA EQUINA SYNDROME DENTATE NUCLEUS HYPERINTENSITY: AN CASE PRESENTATION PATIENT, 24
SECONDARY TO TARLOV CYST HEMORRHAGE IMPORTANT DIAGNOSTIC CLUE IN A WOMAN YEAR, WITH VOGT KOYANAGI HARADA
– CASE PRESENTATION WITH ENCEPHALOPATHY (VKH) INCOMPLETE SYNDROME AND
RADIOLOGICALLY ISOLATED SYNDROME
Késia Sindy Alves Ferreira Pereira, Ingred Pimentel Gabriela Lopes de Morais, Gustavo Maximiano
Guimarães, Davi Lopes Santos, Franklin de Castro Alves, Roberto Satler Cetlin, Natalia Oliveira da (RIS)
Alves Neto, Danyela Martins Bezerra Soares, Silva, Fabíola Dach Annelise Akemi Higa Lee, Marcelle Sanjuan Ganem
Gustavo Rodrigues Ferreira Gomes, Lucas Alverne Prado, Keila Narimatsu, Alexandre Teruya, Eunice
Freitas de Albuquerque, Enrico Pinheiro de Oliveira, Hospital das Clínicas da Faculdade de Medicina de
Ribeirão Preto. Ribeirão Preto SP, Brazil Higuchi
Stelio da Conceição Araújo Filho, Gilnard Caminha
de Menezes Aguiar gabrielalmorais8@gmail.com Hospital Santa Casa de Misericórdia de São Paulo,
São Paulo SP, Brazil
Universidade Estadual do Ceará. Fortaleza CE, Hospital Moriah, São Paulo SP, Brazil
Brazil Case Presentation: A 56-year-old woman with invasive
Hospital Geral de Fortaleza. Fortaleza CE, Brazil ductal carcinoma was admitted to the gynaecology depart- a_akehiga@yahoo.com.br
Instituto Doutor José Frota. Fortaleza CE, Brazil ment with a 2-week history of somnolence, weakness,
mental confusion and reduced fluency of speech. Two Case Presentation: Female, 24 years old, previously
kesia.sindy@aluno.uece.br months before onset of current symptoms, she had been healthy, presented in december 2021 an acute poste-
Hospitalized due to a febrile neutropenia, herpes-zoster rior uveitis associated with serous retinal detachment,
Case Presentation: A 37-year-old male patient presented and a local infected large ulcer on her right hemithorax. tinnitus and vertigo. Ophthalmological evaluation was
sudden onset of severe sacralgia (VAS 9), with paresthesia Because of this skin lesion, topic metronidazole was performed with suspected Vogt Koyanagui Harada (VKH)
and allodynia in the right buttock, which radiated to the prescribed. About two weeks later, she started with the syndrome, treatment with intravenous methylpredniso-
posterior aspect of the thigh, making it impossible for referred symptoms. Metabolic causes were discharged and lone was initially with partial response. It remained with-
him to sit down. Five days later, he progressed to urinary the only alteration was a pleural effusion with unproven out maintenance treatment. Two month later she was
and fecal retention and erectile dysfunction. There was malignancy involvement. Neurologic evaluation was with worse ocular complaints, binocular involvement,
no decrease in strength, coordination, or other changes requested and a severe encephalopathy without focal that didn’t get better after oral corticoid administration.
in the neurological examination. MRI of the lumbosacral deficits was diagnosed. Brain magnetic resonance imag- Magnetic resonance imaging (MRI) orbits showed thick-
spine and pelvis showed multiple tubular images in sacral ing (MRI) revealed T2/FLAIR hyperintensities in dentate ening and enhancement of the posterior contour of the
projections with hypersignal on T2 and hyposignal on T1, nuclei and white matter nonspecific lesions. Cerebrospinal right eyeball (suggestive of VKH) and brain MRI exhibit
suggesting multiple Tarlov cysts (Perineural Cyst – PC), fluid was normal and electroencephalogram showed non- hypersignal areas in white matter mainly periventricular
in addition to pelvic and perianal varicose veins, edema specific findings. The diagnosis of metronidazole induced as well as infratentorial in FLAIR and T2 sequences and
adjacent to the coccyx and fluid in the pelvis. An area of encephalopathy was made because of history taking and post-gadolinium enhancement of two lesion (compli-
50x25 mm was detected in pre-sacral projection at the S4 MRI patterns. Hence, the antibiotic was immediately sus- ant with criteria for Radiologically isolated syndrome
level, with hyposignal on T2, isosignal on T1 with periph- pended. Discussion: Encephalopathy may be caused by (RIS)). No oligoclonal bands were detected. The patient
eral areas of hypersignal, without restriction to diffusion, toxic, metabolic, infectious and autoimmune mechanisms. had no cutaneos lesions. Discussion: VKH syndrome is
indicating hemorrhage in a PC with compression of the Metronidazole-induced brain toxicity can cause altered a chronic disorder characterized by inflammation of the
cauda equina. Before hemorrhage, the patient was being mental status, seizures and cerebellar ataxia with a wide uvea, meninges, auditory system, and integumentary sys-
treated for hemorrhoidal disease with the drug combi- variation of doses and time of use. In up to 93% of cases tem. It may present clinical and/or radiological that can
nation diosmin and hesperidin for two months, possi- of this intoxication, MRI shows bilateral T2/FLAIR hyper- mimic autoimmune demyelinating diseases of the central
bly contributing to the bleeding of the PC. The patient intensities in dentate nuclei, which is a very specific pat- nervous system (CNS) such as Multiple Sclerosis (MS).
underwent conservative treatment, with neuropathic tern. The radiologic differential must be taken with inborn Briefly the revised diagnostic criteria for VKH include:
pain and anxiety control with duloxetine, pregabalin, errors of metabolism, totally discharged in the presented 1-absence of penetrating ocular trauma; 2-absence of
chlorpromazine and clonazepam. After 30 days there was case because of the patient’s past medical history. Hence, evidence of concomitant eye disease; 3-bilateral uveitis;
an important spontaneous reduction of the cyst to 20x10 we assume that the extent of the ulcer being treated with 4- neurological and auditory manifestation; 5- cutaneous
mm, with improvement in excretory functions, erectile topic metronidazole had great cutaneous absorption and alteration. We described a case of incomplete VKH dis-
dysfunction and pain. Discussion: Tarlov cyst consists of within some weeks the antibiotic intoxication led to the ease (based on diagnostic criteria) in which the brain MRI
the formation of saccular dilatations filled with cerebro- current symptoms. In such cases, the antibiotic must be demonstrated demyelinating white matter abnormalities
spinal fluid, located in the nerve sheaths of the posterior promptly suspended and most patients show mental compatible with the revised 2017 McDonald Criteria for
roots of the spinal nerves, mainly the sacral roots. It has a status improvement. Final Comments: The aim of this dissemination in space. In the absence of clinical demy-
prevalence of 1-4%, with asymptomatic clinical course, case presentation is to present an uncommon diagno- elinating episodes it was classified as a RIS. Besides the
being symptomatic in less than 1% of cases, due to pres- sis in which MRI pattern recognition and a well-taken treatment and prognosis for both conditions are differ-
sure from the cyst on the nerve roots, caused by trauma, anamnesis are essential to the final diagnosis. The faster ent, the early and accurate diagnosis is very important to
ischemia, inflammation, or hemorrhage, the latter being the metronidazole suspension, the better the patient’s avoid complications of delayed diagnosis or unnecessary
rare, with few cases reported in the literature. Symptoms prognosis. Neurologists must be familiarized with this medications. However, differential diagnosis of the com-
can be back pain, sensory and motor radiculopathy, cause of encephalopathy. bination of uveitis and CNS demyelination may include a
changes in excretory functions, dyspareunia and sexual systemic or CNS vasculitis, systemic lupus erythematosus,
dysfunction, indicating the need for clinical or surgical Behcet’s disease, sarcoidosis, and paraneoplastic disor-
treatment. Final Comments: This case highlights a rare Miscelânea
ders. More studies in the area need to be carried out. Final
complication of incomplete cauda equina syndrome Comments: A rare case presentation of incomplete VKH
secondary to Tarlov cyst hemorrhage, after treatment of disease in which the brain MRI demonstrated demyelin-
hemorrhoidal disease, demonstrating the need to include ating white matter abnormalities compatible with RIS.
PC in lumbosacral compressive etiologies, for rapid diag-
nosis, appropriate therapeutic intervention, and reversal
of neurological symptoms. Miscelânea

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102
XXX Congresso Brasileiro de Neurologia

TL 1106583 TL 1106602 TL 1106613

NEURO-ONCOLOGICAL PERSPECTIVE IN A CASE PRESENTATION WILSON’ S DISEASE OPTICAL NEUROTOXICITY BY TOLUENE


PATIENT WITH BONE METASTASIS FROM ON A 15-YEAR-OLD BOY WITH SEVERE
Natália Virgínia de Oliveira Ambrósio, Laís Ciribelli
FOLLICULAR THYROID CARCINOMA NEUROLOGICAL IMPAIRMENT AND RARE Yamaguchi, Bruna Queiróz Vieira, Jéssica Cristina
CORPUS CALLOSUM INVOLVEMENT ON Silveira Damasceno, Laura Altomare Fonseca
Rodrigo Alves Ataíde, Erlan Pércio Lopes Rufino,
Emily de Carvalho Batista, George Gláucio Carneiro BRAIN MRI Campos, Bruno de Magalhães Barbosa Leite, Daniel
Leão de Guimarães Filho, Ana Letícia Lacerda Paiva, Teixeira Martins Schettini, Fabiana Veloso Ferreira,
Marcela Santarelli Casella, Jose Marcos Vieira de
Victoria Maria Fontenele Costa, Fernando Augusto Fernanda Groppo Nogueira Herculano, Thiago
Albuquerque Filho, Alulin Tacio Quadros de Monteiro
Pacífico, Abraão Wagner Pessoa Ximenes Cardoso Vale
Fonseca, Marcelo de Melo Aragão, Ricardo S Pinho
Universidade Federal de Pernambuco. Recife PE, Hospital e Maternidade Therezinha de Jesus – Juiz
Universidade Federal de São Paulo. São Paulo SP,
Brazil de Fora – MG – Brazil
Brazil
Real Hospital Português de Beneficência. Recife nvoambrosio@gmail.com
PE, Brazil marcela.santarelli@hotmail.com
Faculdade de Medicina de Olinda – Olinda – PE – Case Presentation: A 32-year-old male, illicit drug user,
Brazil Case Presentation: A 15-year-old boy presented with a
was evaluated for bilateral visual loss after inhaling large
6-month history of progressive loss of dexterity in his right
rodrigoataidemed@gmail.com amounts of thinner and accidently ingesting the product,
hand, manifested by difficulty writing and also dysarthria,
in addition to touch his eyes with the product’s dirty hands.
disphagya, generalized feeling of weakness and difficulty
Case Presentation: A 31-year-old female patient under- A day after the event, he developed bilateral intense retro-
walking, dragging his right leg in the past months, slowness
went a total thyroidectomy 5 years ago for thyroid tumor cular pain that worsened with eye movement, associated
and shaking of hands. He was under thrombocytopenia on
resection. Recently, she complained of back pain, having with photophobia and diffuse myalgia. He evolved with
investigation. His parents are non-consanguineous and
a palpable mass at the site. Hypoesthesia was also noted significant loss of visual acuity, remaining with only light
he has four older siblings and five other half siblings, all
in the right T10 dermatome. 4 months before the neuro- perception. He was assessed by an ophthalmologist who
of them with no medical problems. He was disoriented in
logical consultation, she performed a CT scan in which did not find ocular or retinal changes. Eight days later the
time and place. He also had hypomimia, micrographia,
an osteolytic expansive lesion was observed on T10 in the accident, he was then referred to this neurology service
dysarthria, rigidity and bradykinesia on four limbs and
proximal portion to the right costal arch. Then an MRI was with non-reactive mydriatic pupils, bilateral amaurosis,
also dystonia on both hands and cervical muscles. Lab
requested, in which a large tumor was found, reaching with only light perception. Cranial CT was normal and
investigations showed leukopenia with lymphopenia,
the vertebral body of T10 and extending to the right tho- CSF showed high protein levels. He was subsequently
thrombocytopenia, mild elevated GGT and elevated
racic cavity, so a giant cell tumor was suspected. Thus a diagnosed with a bilateral optic nerve damage due to
AST levels. His liver function was impaired, with pro-
biopsy of that tumor was requested and the result was optic neurotoxicity caused by toluene present in thinner.
longed RNI, normal albumin and bilirubin levels, but
compatible with follicular thyroid carcinoma metastasis. Discussion: Thinner is a solvent for paints and varnishes,
abnormal abdominal ultrasound, demonstrating chronic
Then, tumor resection surgery was performed, including used in automobiles paint and body shops. Its main com-
parenchymal liver disease and splenomegaly. Endoscopy
T10 corpectomy and arthodesis with pedicle screws from pound is toluene, a fat-soluble which is rapidly absorbed
showed esophageal varices without red spots. Serology
T7 to L1. Besides, the patient is being followed up with by the lipid-rich CNS, its primary target. Its distribution in
such HIV, syphilis, HBsAg and HCV Ab were negative or
oncology and is awaiting screenig tests, but the scintig- the body and excretion are quite rapid. All the absorbed
normal. Ophthalmologic examinations were suggestive of
raphy did not show any other bone lesions. Discussion: compound is almost completely eliminated from the body
Kayser-Fleischer rings on the initial stage. On laboratory
Thyroid cancer can evolve to its distant metastic state, 18 to 20 hours after exposure. Acute manifestations of
low concentrations of serum ceruloplasmin (2.9 mg/dL,
such as bone metastases. In a hematogenous route, a toluene toxicity include euphoria, headache, weakness,
normal 20–63), low serum copper (<0.25?g/ dL, normal
carninoma can lead to bone metastasis, with this malig- vision disturbances, tremors, confusion, ataxia, paralysis,
80–140) and increased daily urinary copper excretion (157
nancy occurring in 2-13% of cases of DTC (Differentiated epileptic crisis, narcosis, and death. In addition, toluene
?g/24 h, normal 0–60). Gene sequencing later identified
Thyroid Cancer). This specific type of metastasis is more optic neuropathy may occur, which leads to a slow and
ATP7B c.3061-12T>A (intronic) / ATP7B deletion (exon
prevalent in follicular cancer (7-28%) than in papillary progressive decline in visual acuity. The initial appearance
7), confirming Wilson’s Disease. MRI of the brain showed
cancer (1.4-7%). Bone metastases represent a frequent of the optic disc is usually normal, but there is gradual optic
bilateral and symmetric T2 hyperintensity in basal ganglia
complication, specially in follicular thyroid carcinoma, atrophy. Final Comments: Hippuric acid is a biological
(especially putamen), mid-pons, cerebellar peduncles,
and cause pain, fractures and spinal cord compression, indicator of exposure to toluene, excreted in the urine.
thalamus, which are typical regions of involvement for
also is observed hypoesthesia in some dermatomes, so the However, some authors claim that there is no diagnostic
WD, but also showed and evident hyperintensity in the
patient’s quality of life is severely reduced. The diagnosis value in its dosage, as it is a normal constituent of urine.
splenium of corpus callosum, an uncommon finding in the
is established by correlating clinical suspicion with neu- Thus, after discussing the condition with the toxicology
disease. The patient was started on biperiden 2mg 8/8h for
roimaging, such as whole-body and skeletal MRI, which team of a reference Hospital, it was decided not to mea-
symptomatic control and we prescribed D-Penicillamine
are essential to locate and assess the extent of lesions. As sure this marker, since the main suspicion would remain,
as a treatment plan. Parkinson-like syndrome is the most
an adjuvant treatment, radioiodine therapy is indicated to even with a negative test. A fat-suppressed MRI of the skull
common neurologic presentation of Wilson Disease, espe-
destroy malignant thyroid tumor cells left over from total and orbits were performed, showing discreet tortuosity
cially in adolescents. On Brain MRI the most common
thyroidectomy. Thus, it eliminates locoregional microme- of optic nerves and peri sheath fluid at the insertion of
finding is T2 hyperintensity in putamen and involvement
tastases and distant metastases. Final Comments: Since the eyeball, suggestive of neuritis. He underwent pulse
of other basal ganglia, pons, thalamus and the midbrain.
scintigraphy excludes the possibility of another bone therapy for 5 days with methylprednisolone, without
Midbrain involvement led to the classic “face of the giant
lesion, the patient has a good prognosis. According to the clinical improvement.
panda sign”. Is it uncommon the involvement of frontal,
neuro-oncological literature, complete remission reaches parietal, temporal lobes, cerebellum and corpus callosum,
20% of clinical cases of bone metastases resulting from the last being the rarest site of lesion. Corpus callosum Miscelânea
follicular thyroid carcinoma, reiterating the matter of involvement was associated with more severe neurological
radioiodine therapy as an adjuvant treatment. Finally, a and psychiatric dysfunction, more extensive brain lesions
clinical improvement in the patient under analysis could and longer course of disease.
already be observed, as the hypoesthesia of the T10 der-
matme was reversed.
Miscelânea
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XXX Congresso Brasileiro de Neurologia

TL 1106633 TL 1106562 TL 1104697

LEPTOMENINGEAL NEOPLASTIC EPIDEMIOLOGICAL ANALYSIS OF PATIENTS CASE PRESENTATION LEFT FRONTAL EXTRA
SYNDROME: A CASE PRESENTATION WITH DIFFUSE GLIOMAS TREATED IN AXIAL TUMOR CAUSED BY EPITHELIAL
PARAÍBA NEOPLASM OF THYROID FOLLICULAR
Daniel Vicente de Siqueira Lima Junior, Matheus
Gurgel Saraiva, Luiza Alves Monteiro Torreao ORIGIN
Herisson Rodrigues de Oliveira, Savio Daniel Freire
Villarim, Jeanina Cabral Dionizio, Arthur Felipe de Albuquerque Figueiredo, Camilla Vanessa Araújo Gabriella Cruz Goebel, Isabela Reis Manzoli, Diego
Barbosa Vasconcelos, Mylena Gaudencio Bezerra, Soares, Tiago César Costa da Silva, Maurus Marques Bezerra Soares, Ícaro Tavares Sanches, Jhoney
Francisco Anderson de Sa Carvalho de Almeida Holanda Francieis Feitosa
Hospital Metropolitano Dom Jose Maria Pires – Universidade Federal da Paraiba. João Pessoa PB, Unifacimed – Cacoal – RO – Brazil
Santa Rita – PB – Brazil Brazil
Departamento de Neurologia e Neurocirurgia gabriellacruz1011@outlook.com
daniel.med.upe@gmail.com
do Hospital Universitário Lauro Wanderley. João
Pessoa PB, Brazil Case Presentation: From antiquity to the present day,
Case Presentation: E.D.S; female, 65yo, white, smoker (50 tumors have always aroused curiosity in the field of medi-
pack-years) with consumptive syndrome and report of cog- heri.md.92@gmail.com cine and instigated science in an obsession to understand
nitive decline, behavioral and gait disorders, with a slowly its mechanism of action and seek its cure. Neoplasms were
progressive course plus a recent episode of exacerbation Background: Diffuse glioma (DG) is the most frequent initially described around 2600 years before Christ in an
and possible diagnosis of Normal pressure hydroceph- and aggressive type of malignant tumor of the Central Egyptian papyrus containing reports of 48 cases of the
alus (NPH). The patient presented, in the last 5 months, Nervous System with high morbidity and mortality. disease carried out through the studies of the Egyptian
worsening of the symptoms associated with headache, Epidemiological data on patients with diffuse gliomas physician Imhotep. It was only centuries later, with the
evolving with dependence for activities of daily living. In in Brazil are scarce. Objective: To characterize the epi- Renaissance movement and the beginning of the mod-
addition, he presented hearing deficit with a unilateral demiological profile of patients with gliomas in Paraíba. ern age in the 18th century, that the first anatomical and
sensorineural pattern on the right, in addition to central Method: This is a cross-sectional, retrospective observa- histopathological descriptions were made thanks to the
vestibular dysfunction and left third cranial nerve pare- tional study that included participants of both genders, development of microscopy and the work of the German
sis. The patient sought care when she was Hospitalized over 18 years of age, with DG diagnosed and treated in physician Virchow and the Belgian anatomist Andreas
and tonic muscle spasms were observed with associ- João Pessoa. Results: The sample consisted of 28 patients, Versalius. In addition, with the advance of the scien-
ated loss of consciousness, in addition to an increase in with a mean age of 59 years. The most relevant symptom tific revolution in the 19th century, factors related to the
serum inflammatory and infectious markers and in the at diagnosis was seizure (46.15%), followed by head- heredity of tumors as well as their compression of genetic
cerebrospinal fluid (Hyperproteinorrhaquia with hypo- ache (23%), progressive forgetfulness (23%), aphasia aspects were elucidated through analytical observations
glycorrhachia and slightly increased cellularity; BAAR (12%), progressive visual loss (8%), and hemiparesis (8%). and categorical writings developed by the Austrian monk
and GeneXpert negative). Magnetic resonance imaging Astrocytomas were the most prevalent type of umor (16 Gregor Mendel and later applied to the study of the genetic
showed leptomeningeal enhancement, mild dilatation of patients), with grade 4 being the most prevalent of them. basis. Modern. Since then, with the advent of technology,
the supra and infratentorial ventricular system, associated There were 5 glioblastomas multiform (GBM) diagnosed, hundreds of scientists have embarked on a mad dash
with signs suggestive of transependymal CSF transudation all in males, while in astrocytomas, only 37.5% were males. to improve new techniques and innovative treatments
and ventriculitis. Computed tomography of the abdomen, The mean KPS scale was 81.15 with only 19.2% (5 patients) that allow us to understand and treat the mechanisms
pelvis and neck were performed in order to promote having KPS below 70, and the mean overall survival was of the disease. In this context, epithelial neoplasms are
neoplastic screening, however the results were normal. 41 months. Patients with KPS greater than 85 had better known to be tumors that can be classified as benign or
Cervical ultrassound showed lymph nodes increased in overall survival (57.8 months) than patients with KPS less malignant, originating from a disordered proliferation of
number in left cervical chains. A leptomeningeal biopsy than 85 (36.4 months). When we associated age and overall follicular cells in tissues influenced by thyroid hormones.
was performed and the result consisted of infiltration by survival, we noticed that patients younger than 45 years Benign follicular nodules are the most common types
atypical cells with the presence of KI67 marker in 100% had a mean overall survival of 105 months, while patients presented, which contain in their cytological analysis
of the cells of interest. The patient had an unfavorable aged 45 years or older had a mean overall survival of 30.9 variable proportions of colloid and benign-looking fol-
outcome and died within 1 month. Discussion: Despite a months. None of the five patients with GBM survived licular cells arranged as macrofollicles or macrofollicle
clinical course suggestive of NPH, the history of smoking after 5 years (mean overall survival of 10 months). As for fragments in some tissues of the body. Therefore, in the
and the consumptive syndrome, associated with diffuse Astrocytomas, 37.5% of patients were alive after 5 years (6 Case Presentation: in question, a 50-year-old female
meningeal manifestation and characteristic cerebrospi- patients, with a mean overall survival of 46.9 months). As patient was identified with the presence of a left frontal
nal fluid profile, showed a probable neoplastic syndrome for the other gliomas, 2 of the 5 patients were alive after 5 extraaxial tumor caused by an epithelial neoplasm of
in the central nervous system, associated with the KI67 years (median overall survival 74.7 months). Discussion: thyroid follicular origin.
marker, which is more frequently expressed in upper respi- Current literature highlights that patients with older ages
ratory tract epidermoid carcinomas. Final Comments: The (>45 years) have a worse prognosis than younger patients.
presence of hypoglycorrhachia in the diagnostic context In addition, KPS <85 is also an important predictor of Neurofisiologia Clínica
of meningoencephalitis should expand the possibilities unfavorable outcomes, and may increase mortality. In
of differential diagnosis, with neoplastic diseases among contrast, low-grade gliomas (1 and 2) reduce mortality
the hypotheses. In the case in question, the histochem- by up to 22%. In GBMs, the 5-year survival after diagno-
ical finding allowed the correct diagnosis of meningeal sis is less than 5%. Final Comments: The data brought by
carcinomatosis, with the central nervous system being an this study corroborate the current literature, in addition
uncommon site for metastatic implantation of squamous to reinforcing the influence of age, KPS at diagnosis and
cell carcinomas of the upper respiratory tract. histological type as prognostic factors, assisting in deci-
sion making by health professionals.
Miscelânea
Neuroepidemiologia

104
XXX Congresso Brasileiro de Neurologia

TL 1104975 TL 1105119 TL 1105148

PARRY-ROMBERG SYNDROME – CASE RABIES ENCEPHALITIS: FOLLOW-UP WITH AUTONOMIC NEUROPATHY FOLLOWING
PRESENTATION ELECTROENCEPHALOGRAM COVID-19 VACCINATION
Carla Beatriz Jales da Silva, Larissa Russo Ramos, Marcillio Holanda Bezerra, Camila Cristina Bastos Rafael Ragazzi de Moraes, Matheus dos Santos
Mariana Jacob de Castro, Rafaela Sousa Severo, Silva Raposo, Rejane Karla Santana Albuquerque, do Nascimento Carvalho, Edson Gabriel de Lima
Antônio de Matos Lima Neto Andrea Caroline Cavalcante, Carlos Alexandre Lopes, Rebeca Montenegro de Lacerda, Maria Luísa
Twardowschy Granja de Araújo, Pedro Henrique Almeida Fraiman,
Unifamaz. Belém PA, Brazil Fernanda Thaysa Avelino dos Santos
Hospital Ophir Loyola. Belém PA, Brazil Hospital Universitário da Universidade Federal do
Maranhão -UFMA – São Luis – MA – Brazil Universidade Estadual de Ciências da Saúde de
carlajales1507@gmail.com Hospital Universitário da Universidade Federal do Alagoas. Maceió AL, Brazil
Maranhão -UFMA – São Luís – MA – Brazil Centro Universitário Tiradentes. Maceió AL, Brazil
Case Presentation: A 25-year-old female referred to the PUC – PR. Curitiba PR, Brazil Universidade Federal de São Paulo. São Paulo SP,
vascular neurology outpatient clinic after hemorrhagic Brazil
stroke in July 2021. She complained of daily pulsatile marcillio.holanda@hotmail.com Neuro Intensiva. Maceió AL, Brazil
headache associated with nausea and left hemicranial
location. Besides progressive deformity in the right hemi- Case Presentation: A 2-years-old boy from the interior rafardm3@gmail.com
face. Physical examination: good general condition, con- of Maranhão was referred for suspected encephalitis. He
scious and oriented, afebrile, acyanotic, anicteric and had a history of fox scratch on the left leg without rabies Case Presentation: Patient, male, 24 years, presented in
eupneic. Besides left hemiparesis and deformities in the prophylaxis 2 months ago of symptoms onset (fever, emergency service following a 4-day history of intense
skull, this one being hypotrophy in the right hemiface. irritability, spasms and incoordination). The diagnosis abdominal pain with associated nausea, after the first
Being requested Magnetic Resonance Imaging of skull of rabies was made through splitte and PCR test. CSF dose of a mRNA COVID-19 vaccine. Upon interroga-
and subsequently having the confirmation of the diag- (9th day) and brain MRI (15th day) were initially nor- tion, he stated that he had not defecated for 2 days and
nosis of Parry-Romberg Syndrome. Discussion: The mal. The CSF (16th day) showed WBC 106; PMN: 76% reported having difficulties urinating.On physical exam-
Parry-Romberg Syndrome (PPR) or progressive facial Prot: 117; Glucose: 35. Serial evaluations were performed ination, he was alert, with normoactive reflexes, without
hemiatrophy is a disease of unknown origin, usually with electroencephalogram (EEG), which showed: The appendicular and facial motor deficits, and apparent
develops in the first or second decade of life, prevailing EEG was performed under continuous sedation on the hypoesthesia in his left lower limb, with normal deep
female gender, most often affects only one hemiface, as 15th, 18th and 22nd day.; 15th day: Diffuse slowing with sensitivity.In a later evaluation, he reported sporadic
in the patient’s report. It is manifested by a craniofacial the presence of frontocentral spindles; 18th day: Burst- urinary retention, requiring effort to initiate diuresis.
atrophy that can affect part or all facial structures from suppression pattern, consisting of delta and theta activity He denied nausea. Mentioned intestinal colic with diar-
skin, muscle, fat, bone tissue, trigeminal nerve and even with frontal accentuation and maximum interburst inter- rhea and hypotension, reporting episodes of dizziness
the brain parenchyma (PRADO et al., 2011). The SPR can val of up to 5 seconds; 22nd day: Activity suppression. when standing in orthostasis. Laboratory tests showed
affect the central nervous system, among the neurolog- Patient evolved with the absence of brain stem reflexes; no changes.Electroneuromyography showed absence of
ical manifestations, headache and seizure disorders are 32nd day: Burst-suppression. Its bursts were constituted plantar cutaneous reflex in the right foot region, which
the most common. Neuropathies involving several cra- of rhythmic theta activity (5 to 7 Hz) in the frontocen- is compatible with suspicion of autonomic neuropathy.
nial nerves (the third, fifth, sixth and seventh), vascular tral region, with a lack of alpha and beta rhythms. The After 10 days from the onset of symptoms, the patient
inflammation and destruction of the bone surrounding the maximum interburst interval was 10 seconds. 38th day: evolved with clinical improvement and normal, pain-free
trigeminal nerve causes secondary trigeminal neuralgia in Activity suppression and occasional bursts of rhythmic urination. Discussion: Autonomic neuropathy is a con-
these patients, manifesting with chronic facial pain that is theta activity in the left frontocentral region. The last brain dition where the nervous connections of the autonomic
usually resistant to treatment (ARIF et al., 2020) Computed MRI showed: areas of diffuse hyperintensity in the cor- nervous system are affected, causing denervation that
tomography and MRI are methods for diagnosing CNS tex and basal ganglia on T2 and Flair without expression has repercussions on various body systems, such as the
changes, with the characteristic findings being: white in the diffusion sequence. The patient died on the 51st gastrointestinal, urinary and cardiovascular. There are
matter hypersignal on T2 and FLAIR, leptomeningeal day of symptoms onset. Discussion: Rabies encepha- several associated etiologies, as well as different patho-
enhancement, and brain atrophy, being found on the litis is caused by a Lyssavirus. It is transmitted through physiologies. The current literature already has published
patient’s skull MRI. The differential diagnosis includes the bite or scratch of mammals carrying the disease. case studies associating the COVID-19 mRNA vaccine with
diseases that course with cerebral hemiatrophy, such as Despite reports of survivors, the mortality of the disease autonomic dysfunction. However, most cases mention
Rasmussen’s encephalitis and Sturge-Weber syndrome, is close to 100%. There are descriptions of multiple EEG Guillain-Barré Syndrome, an autoimmune disease, in
but in these we did not observe the typical hemifacial patterns of this encephalitis, such as: slowing, periodic which autonomic neuropathy is a secondary complication,
changes found in Parry-Romberg syndrome (PAULA et al., discharges, isoelectric pattern mimicking brain death, not the main symptom. Primary autonomic dysfunctions
2014). Final Comments: The reported case and exposed among others. None of them are specific to the disease. can occur as an autoimmune complication of viral infec-
publications bring a rather rare diagnosis. As the patient’s In the present case, it was possible to follow, through the tions, including being associated with the post-COVID-19
facial involvement has not yet significantly affected the electroencephalogram, the catastrophic evolution of the syndrome itself. As an adverse post-vaccination effect,
quality of life of the patient, the professionals responsi- disease, being observed patterns of progressive hypome- it has already been reported in studies after immuniza-
ble for the case did not prescribe a surgical approach. tabolism, as respectively: diffuse slowing, burst-suppres- tion with the quadrivalent HPV vaccine, but with regard
sion and activity suppression. The EEG is not indicated to COVID-19 vaccines, so far only two cases have been
for the diagnosis of brain death in this population. Final reported worldwide, one with a recombinant vaccine and
Neurofisiologia Clínica Comments: In rabies encephalitis, there are no specific another with a mRNA vaccine. Final Comments: The
findings on EEG. It is used as a non-invasive method at the patient’s clinical history together with the complementary
bedside to identify subclinical seizures, monitor the level exams performed confirmed the diagnosis of autonomic
of sedation and follow-up the patient’s clinical course. neuropathy, which by the chronology of the facts can
be attributed to a rare complication of the vaccination.
Furthermore, the authors believe that further studies on
Neurofisiologia Clínica the comorbidities of vaccination for COVID-19 will may
explain its complete pathophysiology.

Neurofisiologia Clínica

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XXX Congresso Brasileiro de Neurologia

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MYOPATHY MISS DIAGNOSED AS ABDOMINAL PAIN AND FLACCID THE IMPORTANCE OF


AMYOTROPHIC LATERAL SCLEROSIS FOR TETRAPARESIS IN A YOUNG ALCOHOLIC ELECTRONEUROMYOGRAPHY IN THE
SEVEN YEARS – DIAGNOSTIC CHALLENGES MAN: AN ATYPICAL CASE OF ACUTE DIFFERENTIAL DIAGNOSIS OF ACUTE
OF NEUROMUSCULAR DISEASE INTERMITTENT PORPHYRIA (AIP) FLACCID PARALYSIS
Fernanda Michely Nicoli, Marcelo Teixeira Machado, Karla Rafaele Silva Vasconcelos, Hermany Arthur Felipe Barbosa Vasconcelos, Matheus
Amilton Komnitski Júnior, Aluísio Cláudio Mentor Capistrano Freitas, Victor Vitalino Elias, Ian Silva Gurgel Saraiva, Rafael de Souza Andrade, Juliana
Neves de Couto Melo Júnior Ribeiro, Larissa Brenda Gonçalves Miná, Sylvio Magalhães Leite, Paulo Antônio Farias Lucena,
Ricard Gonçalves de Souza Lima, Sarah Diógenes Mylena Gaudêncio Bezerra, Jeanina Cabral Dionizio,
EMG LAB. Curitiba PR, Brazil Alencar, Sarah de Sousa Magalhaes, Ana Sílvia Daniel Vicente de Siqueira Lima Junior, Luiza Alves
Reumatologista. Curitiba PR, Brazil Sobreira Lima Verde, Cleonísio Leite Rodrigues Monteiro Torreão Villarim, Gêneton Romualdo de
fernanda_nicoli@hotmail.com França
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Universidade de Fortaleza. Fortaleza CE, Brazil Secretaria Estadual de Saúde da Paraíba. João
Case Presentation: Male, 56 years old, was referred Pessoa PB, Brazil
to EMG lab by a rheumatologist for evaluation of his rafaele_vasconcelos@hotmail.com
Amyotrophic Lateral Sclerosis (ALS). He arrived with arthurbarcelos_@hotmail.com
complaints of weakness and sadness because he received Case Presentation: A 38-year-old man is admitted in
a diagnostic seven years ago. Since the diagnostic, he was January-2022 with worsening abdominal pain that started Case Presentation: A 53-year-old hypertensive pharma-
expecting his physical decline, as said by his attendant. It 4 months ago. The pain was triggered by alcohol inges- cist reports that 2 months ago he began insidiously with
slowly evolves. That moment was the first time some doc- tion, associated with abdominal distension, nausea and bilateral paresthesia associated with mild brachial dipa-
tor asked him to repeat the electromyography (EMG) test vomiting. He was Hospitalized. He realized a computed resis. In the last 2 weeks before admission, patient reports
after the first two diagnostic tests. Physical exam: Medical tomography (CT) scan of the abdomen, which showed worsening of the condition with paresis in the 4 limbs, pre-
research council (MRC) strength in the triceps and the distension of the loops, without obstruction. He was pre- dominantly brachial and proximal. In the evaluation, the
quadriceps was IV, He had prominent atrophy of facial, viously healthy, had been drinking for 10 years, denied patient was alert, without alterations in the cranial nerves,
arms and legs muscles. Sensory examination was mark- smoking and illicit drugs, hadn’t had a similar episode reduced strength in the upper limbs (Grade I) and in the
edly normal. The two previous EMG reports demonstrated nor family history. No urinary changes. He had seizures, proximal segment of the lower limbs (Grade III) and with
prominent fibrillations (Fibs) and positive sharp waves psychomotor agitation, dysphagia, dysphonia, dipa- areflexia. In the laboratory analysis, the cerebrospinal fluid
(PSWs) with increased motor unit potentials (MUPs) and resis, signs of dysautonomia and progressive proximal showed cellularity of 38 (90% lymphomononuclear), pro-
with a final statement of motor neuron disease. We per- paraparesis, progressing to areflexic flaccid tetraparesis. teins of 36 and glucose 70; in serum tests, the only change
formed a new EMG that showed Fibs and PSWs. Interesting Normal brain CT, cerebrospinal fluid (CSF) study with 3 was VDRL reagent in the titer of 1/128, when FTA-Abs was
that was no high amplitude MUP, instead, we are able, erythrocytes, 2 cells, glucose 85, proteins 95, LDH 25 and requested (which later turned out to be negative). In the
with quantitative EMG, to show a great number of small electroneuromyography (ENMG): a predominantly sub- imaging evaluation, cranial and cervical spine resonance
amplitude and short duration (SASD) MUPs, consistent acute motor axonal polyneuropathy of the four limbs with was performed without significant changes. Therefore,
with active myopathy. Muscle biopsy show inflammatory F waves and reflex normal H. He evolved with respiratory Electroneuromyography (ENMG) was requested and the
infiltrate. Corticosteroids were started and the patient failure, was intubated, and plasmapheresis was initiated, condition was initially managed as neurosyphilis, until
slowly evolves with significant recovery of symptoms. as it was not possible to exclude an acute polyradiculo- there was no therapeutic response and ENMG proved to be
Discussion: The ALS diagnostic is a dramatic report to neuropathy. The hypothesis of AIP was also raised, and a sensory-motor syndrome with a demyelinating pattern.
be given to any patient. The findings of widespread active the urinary dosage of porphobilinogen (UDPBG) and Based on the hypothesis of Guillain-Barré Syndrome, he
denervation and chronic reinnervation in three different genetic tests were positive. Discussion: Acute hepatic underwent treatment with Human Immunoglobulin and
corporal segments were easily obtained in this case. The porphyrias (AHP) are those in which enzyme deficiency showed significant improvement in the condition, with
normal sensory examination also corroborates for the occurs in the liver, with AIP being the most common. AHP improvement in sensitivity and strength in all segments.
establishment of the diagnosis of ALS. High amplitude mainly affects the central and peripheral nervous system Discussion: The use of Electroneuromyography as an
and long duration MUPs also were there. The patient by increasing the production of neurotoxic precursors auxiliary tool in the diagnosis of neurological diseases is
stayed waiting for his slow decline and it was a surprise (delta-aminolevulinic acid and porphobilinogen). It is already a reality. However, ENMG has been shown to be
when he shows up with seven years of ALS history walk- an acute predominantly motor axonal neuropathy sim- particularly useful in the diagnostic distinction between
ing with some difficulty but without any assistive device ilar to Guillain-Barré syndrome (GBS), which usually acute flaccid paralysis, being a fundamental element for
and speaking normally. To us, it was an easy diagnostic occurs after muscle pain and weakness commonly pre- more adequate therapeutic guidance. Final Comments:
as the patient still has widespread positive sharp waves ceded by abdominal pain and psychiatric disorders that In the same way that complementary exams can, on the
with fibrillation potentials, small amplitude short duration can progress to quadriplegia and death. Alcoholism, sex, one hand, help the diagnosis, on the other hand, they
MUPs and no HALD MUPs. Many reasons can throw us negative family history, and protein-cytologic dissocia- can delay it due to numerous diagnostic possibilities that
in the wrong diagnostic direction in this case. In Brazil, tion in the patient’s CSF made the diagnosis challenging. remain open. However, the ENMG, when in correlation
in our lab, we see too many HALD without reasonable Final Comments: ENMG may be helpful in differentiat- with the clinic, has been shown to be a valuable element
explanation in a way that we don’t have seen in the USA. ing GBS from porphyric neuropathy with GBS showing in the therapeutic decision
As in this case, the true cause of weakness was hidden absent H reflex and F waves or with prolonged latency,
under HALD MUPs. Final Comments: We encourage the in contrast to the relative preservation of the H reflex and
routine use of quantitative EMG techniques to discover F wave in porphyric neuropathy, as well as prolonged Neurofisiologia Clínica
and quantify the SASD that otherwise can pass masked distal latency and block. conduction block on GBS with
as seen in this case. normal latency and without conduction block on AHP.
The definitive diagnosis of AIP is made with DUPBG and
genetic testing, both positive in our case.
Neurofisiologia Clínica

Neurofisiologia Clínica

106
XXX Congresso Brasileiro de Neurologia

TL 1106413 TL 1106453 TL 1104807

HEREDITARY AXONAL MOTOR NEUROPATHY SEIZURE CONTROL INDUCED BY TACTILE LABRUNE SYNDROME: CASE PRESENTATION
DUE TO DYNC1H1 GENE MUTATION, NEW STIMULI, NOT ALWAYS A CONVERSION OF AN RARE GENETIC ENCEPHALOPATHY
FORM OF MARIE TOOTH CHARCOT DISEASE DISORDER
Victor Evangelista, Alberto Martins Pina Rodrigues
Yasmim Nadime José Frigo, Hendrick Henrique João Igor Dantas Landim, Matheus Costa Bessa, Neto, Caio Faria Tardin, Beatriz de Moraes Rivera,
Fernandes Gramasco, Mayara Apolinário Januzzi, Ian Silva Ribeiro, Matheus da Costa Guedes, Lécio Thais Dacache
Ana Flávia Andrade Lemos, Natalia de Castro Fim Figueira Pinto, Fernanda Martins Maia Carvalho,
Hospital das Clínicas da Universidade Federal do
Nakao Tiago Pinho Feijo, Jose Hortencio dos Santos Neto,
Rio de Janeiro. Rio de Janeiro, RJ. Brazil
Norberto Anizio Ferreira Frota
UNESP – Botucatu – SP – Brazil
albertopina1994@gmail.com
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
mim.nadime@gmail.com
João igorlandim@hotmail.com Case Presentation: Our patient is a young man with a
Case Presentation: C.A.A, male, 53 years old, hypertensive normal development and previously healthy until 30 years
and asthmatic. He reports adequate neuropsychomotor Case Presentation: A 50 years old male, with previous old, when he presented a clinical picture of a subacute
development and at 6 years of age he began to have dif- medical history of systemic arterial hypertension and intracranial hypertension characterized by progressive
ficulty walking, due to distal weakness in the lower limbs diabetes mellitus, presented a 20 days onset of parox- headaches, vomiting, gait difficulties and papilledema. At
and the presence of left tendon retraction. At age 18 he ysmal abnormal movements at left side limbs, with 5 the time, he was seen by a neurosurgery team at another
walked with unilateral support. Referred for evaluation minutes duration, and ataxia in between episodes. At Hospital, and after going through a CT scan and a brain
at age 49 years after performing electroneuromyography neurological evaluation, a complex movement disor- MRI, was suspected to have an intracranial tumor of the
with the presence of neurogenic motor unit potentials in der was observed, with predominantly hemiballism, right frontal lobe. He underwent surgery for drainage of
the soleus and tibialis anterior muscles, with anterior tip but clonic-tonic paroxysmal seizures, with 2 minutes the lesion and a biopsy was performed, that later came
potentials and absence of current denervatory activity. A duration and EEG electric ictal activity, were observed. inconclusive. He responded well to the procedure and
genetic test was performed for progressive spinal amyot- These seizures ceased clinically and electrographically was relieved of his symptoms. 2 months later, he came
rophy and showed no deletions in the SMN1 gene. Thus, with tactile stimulus. Excluding structural and other to our Hospital after having two episodes of a focal to
the investigation was continued with hereditary neurop- metabolic causes after normal brain MRI, CSF analysis generalized tonic clonic seizure. While doing the workup
athy panel and evidenced a heterozygous mutation in the and blood tests, chemical control of blood glucose with for the seizure disorder, our team reviewed the images
DYNC1H1 gene (OMIN* 600112). This variant is associated insulin showed improvement in clinical symptoms. Patient and noted an aspect of diffuse leukoencephalopathy
in this case with axonal type 2O Charcot-Marie-Tooth was discharged and in follow-up was asymptomatic. with cysts, and asked our pathology team to look at the
disease. Referred to motor rehabilitation, with improve- Discussion: Separately, seizures and hemiballism are an biopsy, noting and chronic gliosis with Rosenthal fibers.
ment in ambulation afterwards. Thus, the investigation uncommon complication of non cetotic hyperglicemic The patient underwent and genetic testing that excluded
was continued with the hereditary neuropathy panel and states. In this case presentation, we showed a very rare COATS syndrome. Overall, the findings were consistent
evidenced a heterozygous mutation in the DYNC1H1 complex movement disorder, with components of both with Labrune Syndrome, an extremely rare form of genetic
gene (OMIN* 600112). This variant is associated in this clincal conditions. More importantly,although pathophi- encephalopathy. The patient remained asymptomatic
case with axonal type 2O Charcot-Marie-Tooth disease. siologicaly explained in few studies, with cases in which with seizures controlled by low doses of antiepileptic
Referred to motor rehabilitation, with improvement in seizure cessation was obteined by auditive stimulus, both drugs for 7 years, when he again presented with a clinical
ambulation. Discussion: Hereditary neuropathy encom- clinically and eletrographically, with tactile stimulus have picture of intracranial hypertension and imaging exams
passes a group of genetic disorders with a phenotypic no case presentation, being the presented above unique. revealed formation of new cysts over the cerebral hemi-
spectrum that ranges from oligosymptomatic to severe Final Comments: Although rare, hyperglycemia induced spheres, cerebellum and brainstem. The neurosurgery
disability. Charcot-Marie-Tooth disease (CMT) is the seizures and movement disorders must be in differential team at our Hospital decided to place a ventriculoperi-
most common form of hereditary neuropathy, and it diagnosis, especially because they can be easily treated. toneal shunt this time, for which the patient responded
may exclusively affect peripheral nerves and produce Furthermore, more data about seizures inhibitor effects well. At the same time, the family agreed to do genetic
symptoms of their dysfunctions. Final Comments: The of sensorial stimulus are needed and possibly supports testing specific for Labrune Syndrome, which confirmed
advancement of genetic testing allowed new mutations the development of new therapies. the diagnosis. Labrune Syndrome is a rare genetic disor-
to be discovered or those that were previously not consid- der that is characterized by the triad of encephalopathy,
ered pathogenic to become later. In the case described, it brain cysts and calcifications. It was described first by
was discovered that the patient’s variant could manifest Neurofisiologia Clínica Labrune et al. in 1996, and was for a while though to be
with the Charcot-Marie phenotype or spinal muscular related to the syndrome of cerebroretinal microangiopathy
atrophy predominantly in the lower limbs type 1. Thus, with calcifications and brain cysts, but later the genetic
given the clinical picture and the neurological examina- mutation specific for Labrune was discovered to be the
tion, it was associated with Charcot-Marie, this is a new SNORD 118 mutation, and these patients, as our own, do
form of the disease. not have retinal telangiectasias as would be expected in
the COATS syndrome. Due to its rarity, there are no large
studies that give information about natural history, life
Neurofisiologia Clínica expectancy and prognosis of the syndrome.

Neurogenética

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XXX Congresso Brasileiro de Neurologia

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LATE-ONSET ATYPICAL PARKINSONISM AND ADCY5 RELATED DYSKINESIA PRESENTING A CASE OF ADULT-ONSET LEUKODYSTROPHY
MOTOR NEURONOPATHY RELATED TO POLG AS DYSTONIA-PLUS RESPONSIVE TO OF RARE UNDERLYING AETIOLOGY: THE
GENE VARIANT CAFFEINE IMPORTANCE OF EXPANDING TOOLS FOR
EARLIER DIAGNOSIS
Wardislau Ferreira, Carina da Silveira Massaro, Igor Melo de Almeida, Vinícius Lopes Braga,
Wladimir Bocca Vieira de Rezende Pinto, Vinicius Wladimir Bocca Vieira de Rezende Pinto, Paulo Maria Fiorot Dalla, Leila Delorme Goldschmidt,
Lopes Braga, Eduardo Claus Constante, Igor Braga Victor Sgobbi de Souza, Acary Souza Bulle Oliveira André Poton, Marcelo Cagy, Mariana Spitz, Ana
Farias, Roberta Ismael Lacerda, Paulo Victor Sgobbi Carolina Andorinho de Freitas Ferreira
de Souza, Acary Souza Bulle Oliveira Universidade Federal de São Paulo. São Paulo SP,
Brazil Hospital Federal dos Servidores do Estado. Rio de
Division of Neuromuscular Diseases, Department Janeiro RJ, Brazil
of Neurology and Neurosurgery, Escola Paulista igoralmeida@id.uff.br
de Medicina, Universidade Federal de São Paulo mariafd_1@hotmail.com
UNIFESP Case Presentation: We present a case of a 40 years old
male with early childhood onset dystonia, paroxysmal Case Presentation: A 43-year-old male developed demen-
wardislau@hotmail.com worsening of varying frequency triggered by fasting and tia in the previous three years. He presented with dys-
improved by coffee consumption, important orofacial arthria, dysphasia, speech and ideomotor apraxia and,
Case Presentation: A 60-year-old patient presented dyskinesia and normal cognitive function, neuroimaging mainly, gait apraxia, which was followed by knee walking.
with a 4-year-history of stiffness and slowing of the left and laboratory screenings. This suggestive story brought On examination there was paratonia, global hyperre-
upper limb, and shortly afterwards in the left leg. He also up the suspicion of a ADCY5 related dyskinesia, confirmed flexia despite preserved strength. MRI disclosed diffuse
developed neck stiffness, urinary retention, and weight by genetic testing and with excellent response to caffeine cortical atrophy with unspecific bilateral white matter
loss of 10kgs. The patient had no sleep complaints, REM with clonazepam. Discussion: ADCY5 related dyskinesia hypersignal, suggestive of advanced leukodystrophy
sleep behavior disorder, cognitive complaints, or anos- is a newly recognised heterogeneous entity with various (LKD). Electroneuromyography was unremarkable, as
mia. Divergent strabismus in the right eye was present phenotypes caused by many different pathogenic variants well as, infectious and metabolic screening. CSF revealed
since childhood. Neurological examination showed dys- in the ADCY5 gene in chromosome 3q21-3p21. Clinically hyperproteinorrachia. Genetic investigation from sali-
arthria, exotropia of the right eye, and facial hypomimia. it is characterized by hyperkinetic movement disorder of vary sample identified one likely pathogenic mutation
Asymmetric bradykinesia more pronounced on the left early childhood onset including choreoathetosis, myoc- in ETFDH gene, associated to autosomal recessive multi-
side. Festinating gait with significant postural instability lonus and dystonia with paroxysmal worsening related to ple-acyl-CoA dehydrogenase deficiency (MADD). Dietary
and camptocormia without ataxia. Treatment was ini- sleep, waking up, fatigue, physiological or psychological orientation and supplementation with riboflavin were
tiated with levodopa 1000mg and pramipexole 1.5mg stresses, little to no progression and important facial dys- initiated. Discussion: MADD impairs the riboflavin-de-
daily with partial clinical response and wearing off after kinesia. The majority of cases have normal neuroimaging, pendent electron transfer system in mitochondria. This
1h30min. Cranial CT disclosed punctiform calcification normal cognitive function and unrevealing laboratory genetic mutation compromises the fatty acid oxidation,
of the basal ganglia. Brain and spinal cord MRI showed finds. Some cases are associated with axial hypotonia, particularly branched chain ones, and the amino acids
marked calcification in the basal ganglia. DaT-SPECT painful dystonias, hyperreflexia and proeminent nocturnal metabolism. Therefore, treatment recommendations
showed asymmetric reception of the radiotracer right paroxysms. This phenotype suggests the R418W mutation include: protein and fat-restricted diet every 4 h; conju-
less than the left side. Electrodiagnostic studies showed as the cause. The two most frequent variants (first the gation of toxic metabolites with L-carnitine and glycine
chronic preganglionic involvement in C6-T1 bilaterally R418W and second the A726T) both occur at conserved reposition; supplementation with riboflavin; besides
and L4-S1 bilaterally. NGS-based gene panel testing was residues in the catalytic pocket of the protein, probably the promising replacement of endogenous ketone body
performed, showing the heterozygous variant c.678G>C resulting in gain of function. Many therapies have been with sodium D,L-3-hydroxybutyrate Clinical spectrum
(p.Gln226His) in the POLG gene. Discussion: The human tried in those patients, with conflicting data on response. varies widely depending on the degree of hypoketotic
Polymerase Gamma (POLG) gene encodes the mito- Treatment with caffeine has been proposed by many hypoglycaemia, metabolic acidosis and defective protein
chondrial DNA polymerase responsible for replication authors. The rationale behind this relies on the antago- metabolism. It ranges from lethal malformation to adult
and repair of the mitochondrial DNA. In adults, the most nism of the A2A adenosine receptor by caffeine counter- onset, throughout a myriad of complications: hepatic dys-
common movement disorder is parkinsonism, occurring balancing the theoretical gain of function of ADCY5. There function, cardio/myopathy, neurodevelopmental delay
in up to 43% of the patients. POLG-related parkinsonism are reports in literature of autosomal dominant heritage, and LKD. In late-onset MADD, it is more prominent the
has an earlier age of onset, at about 40 years and is ini- autosomal recessive cases with a more severe phenotype, neuromuscular phenotypes, with demyelination directed
tially asymmetric clinically and in imaging. Tang et al. de novo mutations, mosaicisms and even possible dual to peripheral nervous system. On the other hand, LKD in
described Q226H POLG variant as potentially pathogenic. diagnosis. The most prevalent seems to be the de novo adults shelters others inherited and acquired disorders
Different from what is seen in the literature our patient mutation in R418W.? Final Comments: This report helps to (e.g.vascular, toxic, degenerative or inflammatory) without
had a late-onset parkinsonism and poor response to consolidate the phenotype of ADCY5-related-dyskinesia, precise genotype–phenotype correlation. Symmetrical
levodopa. Nevertheless, our patient had early postural to describe a new case of R418W as a causative variant pyramidal findings and varying degrees of cognitive
instability and camptocormia, that can be the presenting and adds evidence to the safety and efficacy of caffeine impairment are common. Final Comments: Adult-onset
sign in patients with POLG variant, sphincter dysfunction, in association use with benzodiazepines. LKD requires a puzzling investigation, considering the
asymmetric DAT-SPECT findings, and neuronopathy on differential diagnosis of the underlying pathology. The
electrodiagnostic studies. These findings led to the dif- prevalence is lower than in childhood and specific radio-
ferential diagnosis of a genetic metabolic cause for par- Neurogenética logical/clinical markers may be missing. Our case illus-
kinsonism, and the diagnosis of POLG-related disorder trates MADD leading to adult-onset LKD, while it would
was made. Final Comments: Our case further expands be expected milder findings at this age. Expanding the
our knowledge regarding POLG gene variants and pheno- access to ancillary exams might widen the etiological
types, as a highly heterogeneous spectrum, and shows the panel for young-onset dementia and guide therapeuti-
importance considering it in patients with parkinsonism. cal interventions.

Neurogenética Neurogenética

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XXX Congresso Brasileiro de Neurologia

TL 1105073 TL 1105082 TL 1105171

NEUROFIBROMATOSIS TYPE 1: MULTIPLE CONGENITAL ANOMALIES MELAS SYNDROME &NDASH; MOTHER AND
CASE PRESENTATION HYPOTONIA SEIZURES SYNDROME (MCAHS) DAUGHTER WITH RARE MUTATION (3291) IN
– MULTIPLE GENES, ONE PHENOTYPE? A MITOCHONDRIAL DNA
Maria Laura Caetano Tonhon
CASE SERIES
Nathalye Fernanda Pedroso Dircksen, Arthur Coelho
Centro Universitário Claretiano. Rio Claro SP, Brazil
Rebeca Bessa Maurício, Jorge Luiz de Brito de Moura Marinho, Amanda Leticia Andre, Tatiane
marialaura.ct18@gmail.com Souza, Miguel Vieira de Almeida Chiara Gübel Arroyo Lopes Alves de Jesus, Marcelo Simplicio
Portugal, Antônio Alves Sobreira Neto, Samuel Carvalho, Lorena Fernandes Kronbauer, Damacio
Case Presentation: Neurofibromatosis is a neurocutane- Cavalcante Marinho, Davi Lopes Santos, Maria Ramon Kaimen Maciel
ous syndrome characterized by tumors in the central or de Fátima de Menezes Guimarães, Carolina de
Irmandade da Santa Casa de Londrina. Londrina
peripheral nervous system and in the skin, and can be of Figueiredo Santos, Paulo Ribeiro Nóbrega
PR, Brazil
three types: 1 (NF1), 2 (NF2), and Schwannomatosis. NF1
is the most common type, also called Von Recklinghausen’s Universidade Estadual do Ceará UECE
n.athalye@hotmail.com
disease, and is a rare genetic disorder with vertical autoso- Universidade de Fortaleza. Fortaleza CE, Brazil
mal dominant transmission and an estimated incidence Hospital Infantil Albert Sabin HIAS
Case Presentation: BVSM, 11 years old, in early childhood
of 1: 2500-3000 live births. It is caused by a de novo or Hospital Universitário Walter Cantídio HUWC
difficulty in gaining weight and median school perfor-
inherited loss-of-function mutation in the neurofibromin rebecabessam@gmail.com mance due to inattention. At 9 years of age, he started to
1 gene, located in band 17q11.2, and encodes the tumor have epilepsy associated with severe metabolic acidosis.
suppressor neurofibromin. The diagnosis is clinical, and Case Presentation: We report 3 patients from two unre- After 1 year, the patient evolved with visual scotomas,
there are seven criteria for recognition, two of which are lated families with Multiple Congenital-Anomalies- headache, vomiting, daily seizures, right hemiparesis,
sufficient for that purpose: at least six café-au-lait spots, Hypotonia Seizures Syndrome (MCAHS) due to pathogenic fatigue and palpitations. At the time, she underwent tests
larger than 5 mm in pubertal period or larger than 15 mm variants in different genes and discuss phenotype-gen- that showed an increase in lactate, magnetic resonance
post-pubertal; at least two neurofibromas or one or more otype associations. Case 1: A 26-year-old woman with a imaging (MRI) of the skull with ischemic stroke (IS) and
plexiform neurofibromas; axillary or groin freckles; optic history of myoclonus in both hands during breastfeeding, echocardiogram with the presence of dilated cardiomy-
glioma; two or more Lisch nodules; sphenoid dysplasia, generalized seizures, delayed milestones, hypotonia and opathy. Genetic tests were requested to investigate the
dysplasia, or enlargement of the cortex of long bones; dysmorphisms. She also had recurrent flu and pneumonia underlying pathology, and a mutation was found in the
first-degree relative with NF1. The objective of this paper because of an immunodeficiency. Parents were not related mitochondrial gene MT-TL1, variant c.3291T>C in het-
is to describe the case of a patient with neurofibromatosis but both were of Lebanese descent. Case 2: Female patient, eroplasmy (71%), closing the diagnosis for MELAS. The
type 1 who died due to the appearance of neurofibromas sibling of Case 1, died 6 years ago, was diagnosed with patient died in a few months from congestive heart failure.
in internal organs. Female, 8 years old, diagnosed with tetralogy of Fallot and had a similar history of generalized Due to the mitrochondropathy, a genetic investigation
neurofibromatosis at birth because her father was a carrier seizures, myoclonus, global delayed milestones, dysmor- of the mother was guided, and the same mutation was
of the disease. She developed nodules only internally, but phisms, hypotonia, urinary infections and recurrent flu found (heteroplasmy in 32%). She was asymptomatic and
in vital regions, so she underwent seven surgeries until and pneumonia also because of an immunodeficiency. recently had an unprecedented seizure. Discussion: In
she was 8 years old. She was diagnosed with a nodule in Whole exome sequencing in both sisters disclosed two MELAS syndrome (mitochondrial encephalopathy, lac-
the mediastinum at that age, and 3 to 4 months later in pathogenic variants in heterozygosis (Chr20: 44.047.933 tic acidosis and stroke) there is a genetic mutation that
the throat, which was impossible to remove. She began A>G and Chr20: 44.047.955 C>T) in PIGT (OMIM* 610272). compromises the mitochondrial DNA, being exclusively
to lose speech and have recurrent respiratory infections, Case 3: 9-year-old male, presented with a history of epi- maternally transmitted. Mitochondria generate ATP, and
and was Hospitalized at least eight times with pneumo- lepsy, delayed milestones, gastroesophageal reflux disease their dysfunctions lead to energy insufficiency for various
nia. She was placed in the ICU, placed on non-invasive and myoclonus since birth. He also exhibited obstruc- organs. The clinical diagnosis is based on the presence of
mechanical ventilation, and required respiratory phys- tive sleep apnea syndrome. Immunodeficiency was not IS (<40 years), encephalopathy (convulsions/dementia),
iotherapy to try to maintain airway patency. She was on present. Brain Magnetic Resonance Imaging showed mitochondrial myopathy (lactic acidosis and/or irregu-
drug therapy with traditional corticoids, antibiotics, and cortical and cerebellar atrophy. Whole exome sequenc- lar red fibers), criteria found in the patient in the case in
analgesics. She had café au lait spots and Lisch nodules. ing revealed a missense variant in homozygosity (Chr18: question. The disease can lead to other symptoms such
Eight months after the appearance of these nodules, a 62.157.747 G>A) in PIGN (OMIM* 606097). Discussion: as headache, vomiting, dementia, deafness, diabetes,
brain nodule was discovered, also impossible to remove, MCAHS is a heterogeneous autosomal recessive disorder short stature and cardiomyopathy. About 80% of cases
all benign but located in vital organs. Afterwards, he characterized by global developmental delay, hypotonia result from leucine tRNA mutation, with m.3243A>G
began to present frequent headaches, important visual and early-onset seizures, as well as variable congenital being the most common. The c.3291T>C variant, present
alterations and convulsive crises, dying about a year and anomalies in urinary, cardiac and gastrointestinal sys- in the patient, is found in <1% of cases. MRI lesions are
a half after the discovery of the nodules. It is important tems. MCAHS has also been classified under the more not classic, restricted to the cortex and subcortical white
to highlight that, even though the disease was inherited encompassing term of “glycosylphosphatidylinositol bio- matter. In spectroscopy there is a reduction of n-acetylas-
from the father, the patient had a greater evolution due to synthesis defects” (GPIBD). It can result from variants in partate and accumulation of lactate. Treatment is symp-
the appearance of the nodules being in internal organs. different genes (PIGN, PIGA, PIGT, PIGQ), and MCAHS1 tomatic and with a multidisciplinary team. Antioxides and
and MCAHS3 are related to PIGN and PIGT mutations cofactors can be administered with the aim of improving
respectively. Despite some information in the literature quality of life and reducing the risk of complications. Final
Neurogenética Comments: MELAS presents different phenotypes in the
about the clinical spectrum, it is still unclear if the phe-
notypes are really similar between the different gene same mutation, making it a diagnostic challenge. Still
mutations. Immunodeficiency was the atypical finding intractable and with devastating consequences as in the
in our two cases of mutation in PIGT, which can suggest case described, it is expected that in future research new
some particularities related to the different genes. Final medications can bring better quality of life to patients.
Comments: Immunodeficiency is a symptom not yet
described in patients with PIGT mutation and, despite
the visible similarities between our 3 cases, further stud- Neurogenética
ies may demonstrate phenotypic specificities in different
patients with MCAHS/GPIBD.

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CPEO DUE TO TK2 MUTATION, A RARE AND A CASE PRESENTATION ON THE DIAGNOSIS GENETIC LEUKOENCEPHALOPATHY: A
ORPHAN DISEASE – CASE PRESENTATION OF NEURONAL CEROID LIPOFUSCINOSIS DIAGNOSTIC CHALLENGE
TYPE 2 (CLN2) AND ITS FOLLOW-UP IN A
Emanuelle Bianchi da Silva Rocha, Alex Bertolazzo Roberta Monteiro de Souza, Marcela de Moares
Quiterio, Janaina Moraes de Araújo, Laura Alonso FEMALE PATIENT IN THE STATE OF CEARÁ Serpa, Lucas Leroux de Richezza, Nathália Borges
Matheus Montouro, João Pedro Izidoro Gomes, Humberto Lucca Andrade Moreira, Maria Denise Carmo, Vinicius Pinheiro Soares, Luiz Felipe Rocha
Rodrigo Fellipe Rodrigues, Maria da Penha Fernandes Carvalho de Andrade, Vytor Alves de Vasconcellos
Ananias Morita, Erica Nogueira Coelho, João Aris Lavor, Ellaine Doris Fernandes Carvalho, Vitória
Kouyoumdjian, Eduardo de Paula Estephan Institute of Neurology- Federal University of Rio de
Maria Torres Peixoto Janeiro, Rio de Janeiro, BR. Rio de Janeiro RJ, Brazil
Faculdade de Medicina de Ribeirão Preto. Ribeirão Universidade Estadual do Ceará. Fortaleza CE,
Preto SP, Brazil robertamonteiro02@hotmail.com
Brazil
m.bianchis@hotmail.com GenClinics. Fortaleza CE, Brazil Case Presentation: C. A. S., male, 35 years old, presented
Centro Universitário Christus. Fortaleza CE, Brazil in 2014 with depressive symptoms, developing visuospatial
Case Presentation: female patient, 32 years old, previously hubert1231@outlook.com and executive impairment, as well as behavioral disorder,
healthy, with proximal limb weakness since childhood, after a few months. He presented worsening of cognition
described as incapacity to jump, difficulty to run and Case Presentation: Female patient, 15 years old. With over the years. In 2019 he developed gait ataxia with early
frequent falls. In adulthood, she had progression of the normal neuropsychomotor development up to 5 years old, falls, becoming wheelchair bound within few months. He
weakness associated with pain in proximal limbs. There when she began to have seizures. A computer tomography became totally dependent for daily life activities since
were no diplopia, dysphagia, cervical weakness or sys- scan of the skull was requested, which was normal. The then. In 2021 he developed dysarthria and dysphagia.
temic complaints. No fluctuation of symptoms was noted. patient progressed with limb tremors, ataxia, pyramidal There was no consanguinity nor similar cases in the family.
Patient denied similar cases in family and consanguinity. syndrome, and speech difficulties. At 9 years of age, she Physical exam: awake, bulbar dysarthria, disconnected
Neurological examination showed bilateral, symmetrical had difficulty walking and loss of gait; the cranial reso- words, spastic tretraparesis, bilateral Babinski sign, dys-
and proximally appendicular weakness with myopathic nance showed alterations in the white matter and signs of metria in four limbs. Brain MRI showed diffuse conflu-
gait associated with facial diparesis and paresis of the cerebral cortico-subcortical and pontine-mesencephalic ent white matter T2/FLAIR hyperintensity and cortical
bilaterally superior rectus muscles. Complementary inves- atrophy. In 2016, after full exome sequencing, the diag- atrophy. The possibility of leukodystrophy was raised.
tigation showed a slight increase in hepatic transaminases nosis of Neuronal Ceroid Lipofuscinosis Type 2 (NCL Genetic testing showed heterozygous mutations in the
associated with significant increase in CK (1.341 UI/L) and 2) was confirmed. In 2018, the patient started using the mitochondrial gene alanyl-tRNA synthetase 2 (AARS2).
lactate levels. Electroneuromyography evidenced polypha- drug Brineura. At age 12, in 2019, she scored 1 on The The patient passed away in April 2022. Discussion: The
sic motor unit potentials with reduced amplitude and short Hamburg Motor and Language and CLN2 Clinical Rating diagnostic evaluation of genetic leukoencephalopathy
duration in the right deltoid muscle. Muscle biopsy showed Scale (motor=0 + language=1), in 2020, she scored 3 includes screening test that should be performed to rule
marked variation in the diameter of fibers, single or mul- (motor=1 + language=2). Discussion: NLC is a group of out the main causes: biochemical for inborn errors of
tiples internal nuclei, some degenerating fibers and evi- neurodegenerative diseases with 4 types of variation and metabolism, spinal fluid analysis, magnetic resonance of
dence of post-necrosis regeneration, derangement of the clinical and pathological classification according to age at the brain and spinal cord, and genetic testing, if possible.
intermyofibrillar cytoarchitecture without inflammatory onset of neurological symptoms, neuroradiological and Diseases associated with mitochondrial aminoacyl-tRNA
infiltrates and also substantial number of “ragged-red” and pathological findings. However, the final distinction is synthetase proteins (mt-aaRSs) enzymes are a group of
“ragged-blue” fibers with COX-negative and fiber hyper- genetic. Among them, the late infantile variant or Jansky- genetic leukoencephalopathies associated mainly with
intensity in the NADH-TR and SDH reactions. Genetic Bielschowsky disease starts between 2 and 4 years of age, three specific clinical diseases: aspartyl-tRNA synthe-
panel revealed a homozygous pathogenic variant in the with rapid progression. NCL2 occurs as a result of the defi- tase (DARS2), mitochondrial glutamate tRNA synthetase
TK2 gene (c.323C>T, p.Thr108Met). Discussion: TK2 is a ciency of the lysosomal enzyme tripeptidyl-peptidase 1 (EARS2) and AARS2. [1]. AARS2 is associated with a rare
nuclear gene that encodes the mitochondrial thymidine (TPP1) when the gene encoding the protein expresses an autosomal recessive leukodystrophy. In adults the most
kinase (TK), an enzyme involved in the maintenance of error. The usual findings are myoclonus, seizures, ataxia, prevalent phenotype, first described in 2014, is charac-
the deoxynucleotides of the mitochondria and crucial in difficulty walking, or progressive loss of pronunciation of terized by premature ovarian failure in women and pro-
the biosynthesis of the mitochondrial genome. Bi-allelic words. Encephalic volumetric reduction causes a decrease gressive leukodystrophy [2]. The age of onset is usually
mutations in the TK2 gene relates with diverse clinical in the levels of intelligence, phonation, and motor func- around 25 years, with cognitive decline, neuropsychiatric
presentations, mainly with a severe myopathy, which is tion. Speech and motor function disorders are related to changes, pyramidal and extrapyramidal signs, ataxia and
rapidly progressive, or with chronic progressive external the reduction in pons and bulb diameters, with cerebellar neuropathy [2,3]. Some cases have been described with
ophthalmoplegia (CPEO) associated with limb muscle atrophy being the most prominent factor. In the patient, developmental delay or motor problems in childhood [2].
weakness and dysphagia, both with premature death. manifestations ranging from seizures to changes in white Final Comments: This case presentation illustrates the
Although not approved by FDA yet, deoxynucleosides matter and signs of cerebral cortico-subcortical and pon- diagnostic challenge presented by the primary leukodys-
therapis for TK2 deficiency are under research with posi- tomesencephalic atrophy were found. Final Comments: trophies, which is only possible with a genetic study, unfor-
tive results, mainly in early-onset cases. Final Comments: We present a case of CLN2, a very rare disease, occurring tunately difficult to obtain in our country. The possibility of
In clinical practice, from molecular point of view, CPEO in 6: 100,000 inhabitants. Effective management and identifying a specific mutation related to leukodystrophy
is a diagnostic challenging. This case presentation illus- treatment of this condition require an early diagnosis. is the first step that will allow the development of targeted
trates that testing CPEO in genetic panels is worthwhile. Therefore, we bring this case to encourage the investi- therapeutic treatment for this group of diseases, that are
Considering the tragic natural history of the disease, it is gation of CLN2 disease in children presenting seizures, currently associated with high morbidity and mortality.
essential to identify the TK2 patients, not only for diagno- loss of motor and cognitive function, or speech delay
sis, but also to offer these promising therapies. without a diagnosis.
Neurogenética

Neurogenética Neurogenética

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TL 1105247 TL 1105344 TL 1105382

HICKAM’S DICTUM ON A CASE OF ARGINASE 1 DEFICIENCY: AN IMPORTANT CANTU SYNDROME AS A DIFFERENTIAL


PROGRESSIVE WEAKNESS AND MIMICKING OF EARLY-ONSET HEREDITARY DIAGNOSIS OF DEPOSIT DISEASES: A CASE
HYPERMOBILITY: A DOUBLE GENETIC HIT SPASTIC PARAPLEGIA PRESENTATION
DISORDER
Gabriella Corrêa Dousseau, Julian Letícia Freitas, Isadora Constantini Sorares Andrade, Klesia
Louise Makarem, Rodrigo Siqueira Soares Frezatti, Cristiane Araújo Martins Moreno, Maria Sheila Adaynny Rodrigues, Victor Hugo Souza Silva Gomes,
Pedro José Tomaselli, Mary M Reilly, Wilson Marques Guimarães Rocha Beatriz Fulador, Heloise Helena Siqueira
Junior
Hospital Santa Marcelina. São Paulo SP, Brazil Hospital Geral Universitário. Cuiabá MT, Brazil
Hospital das Clínicas da Faculdade de Medicina de
gabrielladousseau@gmail.com klesia.adaynny@gmail.com
Ribeirão Preto. Ribeirão Preto SP, Brazil
Department of Neuromuscular Diseases, UCL Queen
Case Presentation: A 19 years-old male patient, born Case Presentation: The patient is a female child, who
Square Institute of Neurology, London, UK – Grã-
from consanguineous parents, presented with an insidi- sought consultation with a neurologist at 1 year and 8
Bretanha Reino Unido
ous complaint of progressive weakness and frequent falls months of age due to delay in neuropsychomotor devel-
frezattirodrigo@hotmail.com since the age of nine. At fourteen he needed bilateral assis- opment and excessive irritability. She showed features
tance to walk, and at eighteen he already needed the use of thick hair up to the forehead and increased body hair,
Case Presentation: Son of consanguineous parents, of a wheelchair. His mother highlighted that the patient prominent mouth with full lips, macroglossia, coarse face,
a 14-year-old male patient presented with slowly pro- never liked meat and that he had a lack of appetite and wide nasal bridge; also had a history of surgically corrected
gressive, symmetrical, distal predominant neurogenic frequent vomiting since childhood. She has also noticed congenital heart disease (patent ductus arteriosus) at 9
weakness since he was 1-year-old. He also experienced that he has greater learning difficulties, is more childlike months old and a hypertrophic cardiomyopathy of the
striking joint hypermobility and spinal deformities. His than people of the same age, and is more irritable and left heart ventricle. The hypothesis of lysosomal storage
whole exome sequencing (WES) first revealed a homo- anxious. Neurological examination shows MRC grade disorder was raised and a genetic testing was requested.
zygous class 4 variant at the IGHMBP2 gene (c.2796delC, II weakness and severe spasticity in the lower limbs, in The result was an alteration in the ABCC9 gene, indicative
p.Cys932TrpfsTer46), related to distal hereditary motor addition to being unable to stand in an orthostatic posture. of Cantu Syndrome. Patient was referred to orthopedist
neuropathy (dHMN) type VI and CMT2S. This variant, Brain MRI showed diffuse cortical and cerebellar atrophy. for the investigation of osteochondrodysplasia, speech
however, failed to explain the hypermobility. Therefore, Whole-exome sequencing revealed a homozygous patho- therapy and physiotherapy, and advised to keep follow-up
a thorough search at the WES was conducted, revealing genic variant mutation p.Arg308Gln in the ARG1 gene. with a cardiologist. Discussion: Cantu Syndrome is a rare
a second class 4 homozygous variant at the ALDH18A1 Discussion: Arginase-1 deficiency (OMIM #207800) is a disease, with little more than 100 cases described in the
gene (c.121 C>T, p.Arg41Cys) associated with cutis laxa, rare autosomal recessive genetic disorder caused by muta- literature, caused by alteration in two genes, ABCC9 and
and thus elucidating the clinical picture. Discussion: tions in the ARG1 gene, resulting in partial or complete KCNJ8. It is inherited in an autosomal dominant manner
Occam’s razor is a heuristic tool much applied in medi- loss of enzyme function that affects the liver-based urea and is proposed as a potassium channelopathy. Clinical
cine. It values simplicity, asserting that multiple symptoms cycle. ARG1-deficient patients mainly exhibit hyperargi- features include face coarse with bushy eyebrows, prom-
should have a common explanation. Hickam’s dictum, on ninemia with spastic paraparesis, progressive neurolog- inent supraorbital ridges, anteverted nostrils, prominent
the other hand, states that concomitant symptoms can ical and intellectual impairment, and persistent growth mouth with full lips, macroglossia, wide nasal bridge,
be due to different reasons. In this case, we demonstrate retardation. Early symptoms include irritability, global long and wide philtrum, hypertrichosis with generalized
the antitheses. When in face of a pathogenic variant that developmental delay, failure to thrive, recurrent vomiting, increase in body hair, cardiomegaly, patent ductus arteri-
only partially explained the clinical scenario, we decided feeding/ protein aversion, and anorexia. Spastic parapare- osus requiring correction, mild speech delay, broad ribs,
to further investigate by expanding molecular analysis. sis with onset in early childhood is the most obvious sign skull thick, scoliosis, flaring of the metaphyses. The patient
This led to the discovery of a second pathogenic variant, of the disease. Brain imaging may reveal cerebral atrophy in the clinical case has the main features of the disease.
in a double-hit genetic disorder. The clinical evaluation and cerebellar atrophy may also occur. The ARG1 gene sits Differential diagnoses of the disease includes Beckwith-
remains sovereign to the point of directing the reasoning on chromosome 6 (6q23) composed of eight exons and Wiedemann syndrome, Zimmermann-Laband, dilated
and defining whether the eventual finding is sufficient for there are at least 43 potentially disease-causing variants in and hypertrophic cardiomyopathy, mucolipidosis III
the diagnosis or if we need to look for a second explana- ARG1 with the majority being missense/nonsense muta- and mucopolysaccharidosis (types I, II and IV), the lat-
tion, invoking Hickam’s dictum. Final Comments: The tions and small deletions. In this case, a rare homozygous ter one being the patient’s initial diagnostic hypothesis.
implementation of the next-generation sequencing (NGS) missense mutation was identified. Approximately half of Final Comments: So far, there is no treatment for Cantu
technics in clinical practice changed the molecular assess- the reported subjects are homozygous with a moderate Syndrome, however the manifestations can be managed
ment and improved patient and family care. However, the number of cases arising from consanguineous relation, individually, such as follow-up with a cardiologist and
massive information obtained with these tests is still not as in this case. Final Comments: This case shows that correction of correctable changes; dermatological treat-
fully manageable. We emphasize the importance of inter- progressive loss of mental and motor skills, increasingly ment for hypertrichosis; surgical correction of possible
preting genetic findings in light of clinical manifestations. more severe spasticity, and pyramidal tract signs are the bone changes; monitoring of DNPM in children with
We also stress that Occam’s razor is not always superior hallmarks of the disease caused by ARG1 deficiency, and delay, among others. There is great potential in thera-
to Hickam’s dictum. In genetic conditions, when a strong this is an important differential diagnosis of childhood-on- peutic options with future clinical studies.
pathogenic variant does not explain the full clinical picture, set hereditary spastic paraplegia.
the possibility of a double-hit genetic disorder should be
considered. In such cases, a judicious clinical analysis is Neurogenética
essential to shape the genetic data search. Neurogenética

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DEVELOPMENT DELAY, IMPAIRED GROWTH, AN ATYPICAL PRESENTATION OF LESCH- CLINICAL RESPONSE TO SELUMETINIB:
DYSMORPHIC FACIES, AND AXONAL NYHAN SYNDROME MIMICKING INHERITED CASE PRESENTATION
NEUROPATHY (DIGFAN) WITH EPILEPSY: NEUROTRANSMITTER METABOLISM DEFECT
Ligia Henriques Coronatto, Enzo Barnabé Monteiro,
CASE PRESENTATION WITH A DOPA-RESPONSIVE PHENOTYPE Natália Silva Fernandes, Gabriel Novaes de Rezende
Marina Nolasco Manhaes Gomes Barreto, Danielle Pedro Barbosa Oliveira, Wladimir Bocca Vieira de Batistella, Luiza Ramos de Freitas, Adrialdo José
Carolay Guzman Souza, Marcela de Moraes Serpa, Rezende Pinto, Paulo de Lima Serrano, Roberta Santos
Roberta Monteiro de Souza, Luiz Felipe Rocha Ismael Lacerda Machado, Igor Braga Farias, Anna Universidade Federal de São Paulo. São Paulo SP,
Vasconcellos Beatriz Perdigão Cordeiro, Maisa Vieira da Silva Brazil
Malta, Bruna Gutierres Gambirásio, Paulo Victor UNAERP. Ribeirão Preto SP, Brazil
Institute of Neurology, Federal University of Rio de Sgobbi de Souza, Acary Souza Bulle Oliveira
Janeiro- BR. Rio de Janeiro RJ, Brazil liscorona@hotmail.com
Universidade Federal de São Paulo. São Paulo SP,
marinanolasco3@gmail.com Brazil Case Presentation: Neurofibromatosis type 1 (NF1),
Case Presentation: LSG, 31 years-old man, presented pedro_barbosa36@hotmail.com neurofibromatosis type 2 (NF2) and schwannomatosis
development delay (motor and cognitive), short stature, are tumor suppressor syndromes characterized by mul-
dysmorphic facial features, deafness and epilepsy since Case Presentation: A 10-month-old brazilian male infant tiple nervous system tumors, which manifest in different
childhood. The brother had motor and intellectual impair- presented with irritability, generalized hypotonia and locations depending on their genetic etiology. NF1 affects
ment and his father progressive ataxia related to SCA 2. global developmental delay since the neonatal period. approximately 1: 3000 individuals and is a tumor caused
Neurologic exam revealed: short stature, facial dysmor- Since age 4 months, he started with paroxysmal events, by mutations of the NF1 gene on chromosome 17q11.2.
phism (figure 1), hearing loss, incomprehensible sounds, tonic hyperextension of upper limbs several times a The treatment of NF1 consists of surgical excision of
comprehends some basic commands with gesture, no day. Afterwards, he started having apneic spells, vom- cutaneous neurofibromas (CNFs) when symptomatic.
pyramidal nor cerebellar signs were documented. Brain iting, and diaphoresis. Examination at age 1 year and 3 Surgical removal of plexiform neurofibromas (PNFs)
MRI revealed white matter hyperintensities on T2 and months disclosed axial hypotonia, no ability to sit without are not always possible due their diffuse and multiple
FLAIR, brain and cerebellar atrophy and thinning of the support, dystonic posture of neck, arms, and legs, along nerve roots involvement. The recent advance is the FDA
brainstem. Auditory evoked potential showed bilateral with choreoathetoid movements in hands and feet. CSF approval of the MEK inhibitor selumetinib (Koselugo™)
involvement with peripheral topography. The genetic analysis and lab tests were unremarkable, including nor- for the treatment of children with symptomatic, inopera-
test showed a pathogenic variant c.79G>A in the MORC2 mal serum uric acid levels. As an inherited disorder of ble PNF based on a trial that demonstrated 72% response
gene. Discussion: MORC2 is a gene that through its ATPase monoamine metabolism was suspected, a levodopa trial rate in tumor volume shrinkage by ? 20% in NF1 children
activity is essential for epigenetic silencing. Mutation in was started with marked improvement of dystonic and with PNFs. However, this is not curative as no subjects
this gene seems to cause a hyperactivation of silencing autonomic disturbances. NGS-based multigene panel for had complete tumor disappearance and tumors regrew
in neural cells, impairing neurological development [1]. movement disorders disclosed the pathogenic missense particularly after dose reduction or cessation due to
These mutations have been reported most in Charcot- variant c.233T>C (p.Leu78Pro) in hemizygosity in the toxic effects, such as diarrhea, weight gain, paronychia,
Marie-Tooth type 2Z and DIGFAN syndrome [2,3]. DIGFAN HPRT1 gene (Xq26.2-q26.3), establishing the diagnosis skin ulceration, and elevated creatinine level. This case
syndrome is characterized by development delay, short of Lesch-Nyhan syndrome (LNS). Five months after the presentation is about a patient with NF1 diagnosed at
stature, hypotonia, cognitive impairment, dysmorphic genetic diagnosis, the patient presented with the typical 14 years old in 2008 who started compassionate use of
facies, and axonal neuropathy. This syndrome can also so-called self-injurious behavior. Discussion: Although Selumetinib 80 mg/day (divided into two doses a day)
present hearing loss and brain atrophy [4,5]. In the litera- classically considered in the differential diagnosis of ear- in august 2020 due to clinical and functional worsening
ture, patients with the c.79G>A variant are associated with ly-onset presentation of complex movement disorders, at this time. He had difficulty swallowing due to neurofi-
developmental delay, growth retardation and abnormal LNS is rarely considered for further diagnostic work-up bromas in cervical region, also had inability to walk, was
brain MRI, as in our case. Epilepsy is not usually associ- after normal serum and urinary screening for uric acid able to move only on a wheelchair, and difficulty in evac-
ated with DIGFAN syndrome in MORC2 mutation as in levels. Even in the absence of abnormal serum biomark- uating and urinating, using a urinary catheter and laxative
our case, being a rare manifestation of this phenotype. ers and the typical automutilation features, LNS diagnosis medications continuously. Neuroaxis resonance showed
Final Comments: Epilepsy should be considered as a must be kept in the diagnostic work-up of highly suggestive countless nodular images in the subcutaneous tissue of
manifestation of MORC2 spectrum (DIGFAN syndrome), neurological phenotypes. This case highlights the impor- the scalp, compatible with neurofibroma, sometimes
depending on the variant. Some variant exerts a more tance of performing large NGS-based multigene panels confluent, predominance in the occipital regions. Other
intense action on neural silencing, resulting in more severe to screen complex phenotypes of suspected inherited similar lesions can be seen in the deep cervical spaces.
phenotypes related to central nervous system that could neurometabolic disorders, including LNS and inherited Countless PNFs involving all intervertebral foramina eval-
be involved in epileptic manifestation as in our case. [4]. disorders of neurotransmitters metabolism. Marked very uated, confluent in the paravertebral regions, sometimes
early-onset autonomic disturbances were present in this forming masses. Intradural and extramedullary lesions
patient and presented with a levodopa-responsive pattern, within the vertebral canal stand out, the largest at the
Neurogenética as well as the dystonic involvement. Final Comments: level of C1 and C2, compressing the spinal cord, without
Infancy or neonatal-onset cases of global developmen- altering its signal. After starting Selumetinib the patient
tal delay, generalized hypotonia, early-onset paroxysmal had a significant reduction in neurofibromas, and signif-
events resembling dystonic posturing, and dysautonomia icant clinical improvement. At the moment, the patient
must include LNS in the diagnostic work-up. Levodopa- is able to walk with the help of a walker, has sphincter
responsive cases should aware clinicians about LNS as a control and improved dysphagia. The case is maintaining
differential diagnosis. follow-up in the neuro-oncology sector at UNIFESP, with
stable clinical status.

Neurogenética
Neurogenética

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XXX Congresso Brasileiro de Neurologia

TL 1105540 TL 1105541 TL 1105547

DOUBLE TROUBLE: COEXISTENCE OF FULLY SPINAL MUSCULAR ATROPHY TYPE 2 – USE MORC2 RELATED NEUROPATHY ASSOCIATED
PENETRANT CAG REPEAT EXPANSIONS IN OF NUSINERSENE IN ADULT PATIENT WITH AUTONOMIC DYSFUNCTION
THE ATXN3 AND ATXN7 GENES
Nathalye Fernanda Pedroso Dircksen, Arthur Isabela Silva Souza, Caroline Lourenço de Medeiros,
João Nicoli Ferreira dos Santos, Pedro José Coelho Moura Marinho, Tatiane Arroyo Lopes Alves João Nicoli Ferreira dos Santos, Rodrigo Siqueira
Tomaselli, Rodrigo Siqueira Soares Frezatti, Caroline de Jesus, Marcelo Simplicio Carvalho, Lorena Soares Frezatti, Pedro Jose Tomaselli, Wilson
Lourenço de Medeiros, Isabela Silva Souza, Wilson Fernandes Kronbauer, Amanda Leticia Andre, Marques Júnior
Marques Júnior Damacio Ramon Kaimen Maciel
Faculdade de Medicina de Ribeirão Preto. Ribeirão
Hospital das Clínicas da Faculdade de Medicina de Irmandade da Santa Casa de Londrina. Londrina Preto SP, Brazil
Ribeirão Preto. Ribeirão Preto SP, Brazil PR, Brazil
isabelawollp@hotmail.com
drJoão nicoli@gmail.com n.athalye@hotmail.com
Case Presentation: A 27-year-old female, borned from
Case Presentation: A 16-year-old young man presented Case Presentation: VLO, male, 23 years old, at 6 months non-consanguineous and healthy parents. She presented
with a 5-year history of slowly progressive imbalance and of age, started to develop muscle weakness, evidenced by in her first decade of life with distal and proximal asym-
visual impairment. On neurological examination there was the delay in developmental milestones. At 2 years of age, metrical weakness, sensory loss, generalized arreflexia.
cerebellar ataxia, loss of visual acuity, macular degenera- he was able to walk with support, when the hypothesis She also presented cognitive impairment. Nerve conduc-
tion, slow saccades, ophthalmoparesis, upper motor neu- of Spinal Muscular Atrophy (SMA) type 2 was raised. tion studies revealed sensory and motor axonal neurop-
ron signs and mild cognitive impairment. Interestingly, which showed chronic denervation and a DNA study with athy. WES revealed a class 5 variant in the MORC2 gene
there was a positive medical history of an ataxic disease mutation of the SMN 1 chromosome 5q gene (absence of (NM_001303257.2: c.754C>T ; p.Arg252Trp) confirming
with male-to-male transmission on both paternal and exons 7 and 8). Since then, there has been a progressive the diagnosis of CMT2Z. Sanger sequencing of her parents
maternal sides in up to six consecutive generations. Brain worsening of symptoms. In recent years, he developed confirmed it was a de novo variant. At 26 years old, she
MRI demonstrated mild cerebellar atrophy. Target analysis dysphagia and dyspnea, especially during sleep, requir- reported episodes of recurrent abdominal pain, early satia-
for CAG expansions through PCR with fragment analysis ing CPAP. In 2021, he started treatment with intrathecal tion, bloating, nausea, vomiting, constipation and progres-
detected 59 repeats in the SCA3 gene and 57 repeats in Nusinersena at a dose of 2 mg, with the first three doses sive weight loss. Blood pressure standing and lying, as well
the SCA7 gene. Discussion: The Spinocerebellar Ataxias administered at 14-day intervals, the fourth dose after as heart rate due valsalva maneuver were normal. Gastric
are heterogeneous group of autosomal dominant neuro- 30 days of the third, following treatment with mainte- emptying scintigraphy showed that gastric emptying time
degenerative diseases characterized by progressive cere- nance doses every 4 months. During therapy, an improve- T1/2 was greater than 30 minutes, with gastric retention
bellar ataxia, different genetic mutations and frequently ment in the strength of limbs and patient autonomy was of about 70% of the content at the end of the study, con-
associated a further signs and symptoms. SCA3 is caused observed, demonstrated by the increase in the SCORE firming gastrointenstinal specific autonomic dysfunction.
by a monoallelic CAG trinucleotide repeat expansion in the of the HFMSE scale (Hammersmith functional motor Discussion: Charcot-Marie-Tooth disease type 2Z is a rare
gene coding for the cytoplasmic protein ataxin-3 (ATXN3) scale expanded), from 10 to 37 points. Discussion: SMA form of autosomal dominant axonal neuropathy. Clinical
located on chromosome 14. Normal individuals have up is an autosomal recessive genetic disease with mutation findings include sensory impairment, distal and proximal
to 44 glutamine repeats and complete penetrance of the in the SMN1 (motoneuron survival 1- the gene product weakness, distal atrophy and muscle cramps. Also, there
disease is commonly seen in individuals with more than is a key protein for RNA division that is reduced in SMA), are reports of global hypotonia, including MORC2-related
52 repeats. Age of onset usually occurs in the 4th decade inhibiting functional SMN protein production and lead- disorder in the differential of non-51 SMA. Additionally,
of life, and correlated with the CAG repeat size. SCA7 is ing to degeneration of anterior horn cells in the spinal intellectual disability, hearing loss, microcephaly, pyrami-
caused by a heterozygous CAG repeat expansion in the cord, which results in progressive proximal weakness dal signs and brain atrophy have been reported. MORC2
gene encoding ataxin-7 (ATXN7) located on chromosome with varying degrees of muscle atrophy and paralysis of protein regulates DNA transcription and is important for
3. Pathogenic fully penetrant expansions have 37-460 the respiratory muscles. It presents a wide spectrum of cytoplasmatic function. In a literature review, dysautono-
repeats. Disease spectrum may vary from an aggressive clinical severity, and can be classified according to the mia is not reported in patients with CMT2Z, but it is well
early-childhood ataxia with failure to thrive, rapidly age of onset of symptoms and the developmental mile- established the secondary involvement of MORC2 in
deterioration and early death or as a progressive ado- stones reached. Recently, disease-modifying therapies mitochondrial function and maintenance. Additionally,
lescent/adult form. Both forms have visual impairment. have emerged, such as Nusinersene, a nonsense oligo- there is a Leigh-Like case previously reported sin asso-
As observed in SCA3 there is a correlation between CAG nucleotide that increases the amount of complete SMN ciation to MORC2. Final Comments: We report a case of
repeat size and disease severity and age of onset. Final protein, thereby reducing mortality and improving the CMT2Z caused by a de novo MORC2 gene mutation in a
Comments: The presence of more than one SCA mutation patient’s respiratory and motor function. Its use is ben- patient presenting with gastrointestinal isolated dysau-
in a single individual is rare. The age of onset, the presence eficial at any stage of the disease. In children, mainly, tonomia. We hypothesized that this mutation may affect
of macular degeneration with visual impairment suggests with little application in adults. Final Comments: The mitochondiral machinery. Further data studies are nec-
SCA7 is contributing to his clinical picture. This patient advent of new treatments for AME has highlighted the essary to elucidate the relationship between mutation in
and his family also may provide unique opportunities to importance of early diagnosis, allowing pre-symptom- MORC2 gene and dysautonomia.
study the interactions of two genetic mutations and their atic treatment and resulting in better quality of life for
effect on clinical expression of disease. Furthermore, find- patients. More studies are needed, especially those that
ing two genetic mutations in a patient may have important include the adult population with the disease, where a Neurogenética
implications regarding counseling on genetic risk, genetic good response is also observed.
testing, and disease prognosis.
Neurogenética
Neurogenética

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XXX Congresso Brasileiro de Neurologia

TL 1105781 TL 1105844 TL 1105867

WHEN A HINT TURNS INTO A HINT – A RARE STURGE WEBER SYNDROME IN ADULT: A EXPANDING THE PHENOTYPIC SPECTRUM
CASE OF AN AXONAL SENSORY-MOTOR CASE PRESENTATION IN OF HEREDITARY SPASTIC PARAPLEGIA
NEUROPATHY WITH NEUROMYOTONIA TYPE 6 (SPG6): A CASE PRESENTATION OF
Tatiane Arroyo Lopes Alves de Jesus, Amanda Leticia
Andre, Arthur Coelho Moura Marinho, Nathalye SPASTICITY, EPILEPSY, MOTOR AXONAL
Vanessa Lauanna Lima Silva, Gabriela Lopes de
Morais, Victor Augusto Zanesi Maciel, Rodrigo Fernanda Pedroso Dircksen, Marcelo Simplicio NEUROPATHY, AND DISATROPOPHONIA
Siqueira Soares Frezatti, Pedro José Tomaselli, Mary Carvalho, Lorena Fernandes Kronbauer, Damacio
Maria Clara Baseio, Ellen Silva Carvalho, Rodrigo
Reilly, Wilson Marques Junior Ramon Kaimen-Maciel
S Frezatti, Pedro José Tomaselli, Wilson Marques
Hospital das Clínicas da Faculdade de Medicina de Irmandade Santa Casa de Londrina. Londrina PR, Junior
Ribeirão Preto. Ribeirão Preto SP, Brazil Brazil
Hospital das Clínicas da Faculdade de Medicina de
UCL Queen Square Institute of Neurology – Grã- Irmandade Santa Casa de Londrina, Neuroclinica
Ribeirão Preto. Ribeirão Preto SP, Brazil
Bretanha Reino Unido Londrina. Londrina PR, Brazil
e_len12@hotmail.com
vanessalauanna@gmail.com arroyotatiane@gmail.com
Case Presentation: A 70-years-old man presented with
Case Presentation: A 17-year-old woman born from Case Presentation: Patient FB, 41 years after entering the
a long standing history of complex hereditary disorder
consanguineous parents presented to our neurogenetic service complaining of left hemiparesis (strength grade
characterized by spastic paraplegia, epilepsy and axo-
outpatient clinic with a history of progressive unsteady 4-). The same refers that since childhood (5 months – 5
nal motor peripheral neuropathy. In the last decade,
gait since early childhood. She had normal milestones years) he presents recurrent episodes of reported deficit,
he observed that his voice became laborious, leading to
achievement and no significant medical history until the which improve with the use of phenytoin, causing delay in
disartria and hypophonia. Otorhinolaryngological eval-
age of 6-year-old when she began to have frequent falls. his motor development. He denies other comorbidities or
uation revealed vocal fold paresis, which was attributed
For the subsequent years, she had worsening of symptoms neurological pathologies in the family. In addition to the
to the neuromuscular disease after excluding acquired
with slowly progressive muscle weakness and sensory deficit, diagnosed as a focal crisis, a cutaneous vascular
conditions. Brain and spinal cord MRI showed T2 hyper-
loss, especially at distal parts of the limbs. The patient was malformation on the right hemiface, compatible with a
signal along the corticospinal tract. The SPG6 diagnosis
actively asked about difficulties in releasing grip after a flaming nevus, called attention on physical examination.
was confirmed by an exome sequencing that identified a
strong voluntary hand contraction and reported distress Due to this clinical finding and previous history, a brain
missense variant, (NM_144599.5: c.316G>A, p.Gly106Arg),
doing this task since the beginning of the symptoms. In resonance imaging (MRI) was performed, which showed
within the microtubule binding site domain of the NIPA1
the neurological examination she had winged scapula, volumetric reduction in the right parietal hemisphere and
gene. This variant was classified as class 5 according to
absent Achilles tendon reflexes, pinprick and vibration cortical leptomeningeal uptake compatible with anomaly
ACMG criteria, which implies it has known functional
sense impairment with a length-dependent pattern and a of the cortical venous system, prominence of varicose lep-
consequences. Sanger sequencing showed this variant was
slap gait with distal lower limbs weakness combined with tomeningeal veins and prominence of the brain-related
also present in his affected son. Discussion: Mutations
fasciculations and action myotonia. Nerve conduction choroid plexus. trigeminal angiomatous. The seizures
in the NIPA1 gene have been associated with autosomal
studies revealed an axonal motor-greater-than-sensory were controlled with phenytoin, a drug that the patient
dominant SPG6, which is a progressive neurodegenera-
polyneuropathy and needle monopolar examination received when he was discharged, without any focal defi-
tive disorder. Although most cases present with a pure
revealed increased insertional activity with myotonia cits. Scheduled outpatient follow-up for seizure control
HSP phenotype, there has been some descriptions of
and neuromyotonia (discharges of 90 to 120 Hz). The and follow-up. Discussion: Sturge Weber syndrome is a
complex cases, with variable clinical pictures. About 23%
patient’s parents also performed nerve conduction and rare condition with an incidence of 1/50,000 to 1/230,000
of all published cases report a complex phenotype, with
needle studies with normal results. On complementary live births. It is explained by the abnormal persistence of
epilepsy found in about 10% and a sensory and motor
investigation, no cardiologic or pulmonary involvement the embryonic vascular system which is located around
length-dependent peripheral neuropathy in only 5,5% of
was detected. Initial molecular analysis ruled out MPZ, the cephalic region of the neural tube. Its most common
them. Disartria, hypophonia and a distal motor neuropa-
PMP22, GJB1, GDAP1 mutations. An American College of clinical presentation is the vascular dermato alteration
thy have not been described, according to the best of our
Medical Genetics and Genomics (ACMG) class 5 homozy- manifested in the face, in addition to seizures. Less pre-
knowledge. Final Comments: We presented an unusual
gous missense variant on histidine triad nucleotide bind- sented per patient, in addition to classic change seen on
case of SPG6 with a complex phenotype including epi-
ing protein 1 (HINT1) gene (c.110G>C,p.Arg37Pro) was MRI of the skull. Final Comments: The management of
lepsy, axonal motor peripheral neuropathy, disartria and
found leading to the diagnosis of autosomal recessive neu- patients with this syndrome shows challenges due to its
hypophonia in a patient harboring a pathogenic variant
romyotonia and axonal neuropathy (NMAN). Discussion: rarity and general medical ignorance about it. Its treat-
in NIPA1 gene, which expands the phenotypic spectrum
An early-onset (mostly around the age of 10-years-old) ment aims to control epilepsy and decrease visual defi-
of NIPA1-related disorders.
axonal form of motor-predominant peripheral neurop- cits. In view of the benefit of the correct treatment, it is
athy associated with neuromyotonia has been linked to important to disseminate knowledge about this pathology
loss of functional HINT1 gene. About 70-80% of patients in the medical and academic environment, prioritizing Neurogenética
present with neuromyotonia, which is a clinical hallmark investigation with an image exam which demonstrates
for the diagnosis. Our case brings to light a classical pre- lesions characteristic of the disease.
sentation of a rare autosomal recessive mutation gene that
produces a neuropathy within the Charcot–Marie–Tooth
(CMT) spectrum. Final Comments: Despite the increas- Neurogenética
ing diagnosis of HINT1 patients world-wide, it remains a
diagnostic challenge to assess the pathogenicity of novel
variants. This reinforces the importance to recognize the
clinical syndrome allowing for request exome sequencing,
identify molecular etiology and define treatment strate-
gies of this CMT subtype.

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XXX Congresso Brasileiro de Neurologia

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ADULT-ONSET MELAS: WHAT IS THE BEST SYNDROME 47, XYY: A CASE PRESENTATION MILLER-DIEKER SYNDROM: A CASE-BASED
WAY TO INVESTIGATE? FROM A NEUROPSYCHIATRIC PERSPECTIVE UPDATE
Giuliano Cunha, Marcus Vinícius Abatti, MAriana Breno Henrique Gomes da Costa, Artur Pereira Matheus Brasil Câmara Monteiro, Luís Eduardo
Motta, Renata Guimarães, Henrique MOhr, Helena de França Medeiros, Caio Ferreira de Lima Silva, Oliveira Matos, Amandha Espavier Trés, Gabriel
Fussiger Domingos Sávio Amorim de Souza Dias Guimarães, Marinheiro Santos Bezerra, Espártaco Moraes Lima
Matheus de Lima Cavalcanti, Paulo Eduardo Sales Ribeiro, Paulo Roberto Lacerda Leal, Regina Coeli
Hospital Mãe de Deus – Porto Alegre – RS – Brazil Chalegre, Zenilda Gueiros Silvestre, Elder Machado Carvalho Porto Carneiro
FEEVALE – Porto Alegre – RS – Brazil Leite
Universidade Estadual do Ceará. Sobral CE, Brazil
helena.fussiger@gmail.com Universidade de Pernambuco. Garanhuns PE, Brazil
matheus.monteiro987@gmail.com
Case Presentation: Female patient, 41 years old, with a brenohgcosta@gmail.com
previous history of non-migraine headache and cramps Case Presentation: A 5-year-old female presents with
during exercise, presented, at the age of 36, a sudden Case Presentation: Male, 15 years old, had his first con- significant delay in neuropsychomotor development,
change in consciousness associated with reduced visual sultation approximately 6 years ago due to complaints first observed at 3 months of age, and refractory epilep-
acuity on the right, auditory and visual hallucinations, from his parents regarding psychomotor agitation, het- tic seizures, despite multiple regimens with anti-seizure
with complete recovery in two days. Here, she was diag- eroaggressiveness, hypoprosexia, and a possible ton- drugs. Previous physiological history revealed gestational
nosed with a psychiatric illness. After four years, she had a ic-clonic and generalized ictus pattern at 5 years of age bleeding until the 4th month of pregnancy. Neurological
new episode, but now with associated right hemiparesis. without tongue biting and apparent triggers. He arrives exam revealed adequate visual contact and social smile,
Within two months, she recovered, but remained with on chlorpromazine, risperidone, biperidene, and valproic but no language, and significant axial and appendicular
some cognitive sequelae (mainly memory and executive acid. Drug therapy is corrected to risperidone, carbamaz- hypotonia, with absence of cervical tone. Morphological
dysfunction). After months, she had a new crisis, now with epine, and clonazepam. Physical examination revealed examination revealed the presence of microcephaly,
left hemiparesis, which was initially treated as herpetic macrotia, gait with right lateral-everted foot, plagioceph- divergent strabismus to the right, epicanthus, ocular
encephalitis. His exams revealed an increase in CPK, an aly due to early fusion of the right lambdoid suture, and hypertelorism, flat nasal base, small and upturned nose,
increase in CSF lactate (despite normal cellularity and absence seizures. The psychomotor symptoms improved palpebral fissures turned down and prominent forehead.
proteinorrhea) and cranial resonance with extensive significantly with continued use of the prescribed therapy, Computed Tomography and Magnetic Resonance Imaging
DWI / FLAIR lesion in the occipital, temporal and frontal but hetero-aggressiveness persisted. In complementary of the skull were performed, which showed lissencephaly
regions on the right, not respecting vascular territory and exams, cranial computed tomography (CT) scan showed and agiria. A molecular study to search for microdeletion
without marked hyposignal in the ADC map, in addition to encephalic hypotrophy with malformed gyrus dysmor- syndromes was performed, which detected deletions
lactate peak spectroscopy. Panel of autoantibodies, elec- phisms; electroencephalogram (EEG) showed negative in the METTL16 and PAFAH1B1 genes of chromosome
troneuromyography, echocardiogram, CT of the abdomen, spikes in temporal lobes and negative sharp wave asym- 17p13.3. Discussion: Miller-Dieker syndrome (MDS) is
thorax and pelvis without alterations. Due to the clinical metry and the karyogram confirmed the diagnosis by a rare genetic disease that occurs due to depletions in
suspicion of mitochondrial disease, a molecular study was resulting in a 47, XYY genotype. Discussion: Jacobs’ syn- chromosome 17p13.3. It is characterized by lissenceph-
carried out, collected by oral swab, aimed at the variants drome or 47, XYY consists of an aneuploidy of the male aly, craniofacial alterations, growth retardation, delay
most associated with MELAS. This showed the presence, sex chromosome. Typically, this nosological entity usually in neuropsychomotor development and it is common
in heteroplasmy (in 36% of the readings performed), of presents in association with cognitive, behavioral, and to present refractory seizures. Lissencephaly associated
the pathogenic variant m.3243A>G in the MT-TL1 gene, motor alterations. Van Rijn, in a recent systematic review, with MDS tends to be more severe when compared to
the most associated with this syndrome, confirming the highlighted the prevalence of ASD and ADHD symptoms other diseases of chromosome 17p13.3, being classified
patient’s diagnosis. Discussion: Adult-onset MELAS, in XYY patients in most cases. Executive functions, such as grade 1 lissencephaly (agiria), also distinguished by
especially after the age of 20 years, is considered very as working memory, cognitive flexibility, and inhibitory its craniofacial alterations. The genetic deletion of MDS
uncommon, which leads to a delay in diagnosis by being control, also suffer greater impairment compared to the usually occurs between the PAFAH1B1 and YWHAE genes,
confused with isolated psychiatric conditions or other general population. The cognitive alterations caused by with the PAFAH1B1 isolated deletion resulting in classic
causes of encephalitis. In cases where clinical suspicion is syndrome 47, XYY are also linked to neuroanatomical lissencephaly. In this case, there was a deletion of two
form, it is initially recommended to evaluate the variants alterations caused by the overexpression of homologous genes between these chromosomes, which configures a
in the MT-TL1 gene. The m.3243A>G variant is the one and holandric regions of the Y chromosome. The NLGN4Y MDS case. There was also a deletion of METTL16 gene
most associated with MELAS syndrome, and the degree gene, which is related to autistic spectrum symptoms that succeeds the PAFAH1B1, and, despite being associ-
of heteroplasmy in oral swab collection is in agreement and social responsiveness is an example of these over- ated with the MDS, the exact effects on the progression or
with what has been previously reported. If it were low, expressed genes. Final Comments: The understanding severity of the syndrome are not elucidated. It is known,
due to the high clinical suspicion, one possibility would of anatomical, physiological and cognitive alterations however, that it may be associated with hepatocellular
be to perform the analysis on epithelial cells by urine allows a better elucidation of the relationship between the carcinoma. Final Comments: MDS is rare and has a poor
collection, since it is the sample that shows the highest Y chromosome and neuropsychomotor development. A prognosis, with a reduced life expectancy in most cases.
percentages of heteroplasmy, among those of easy access. striking feature of Jacobs syndrome are the behavioral and Its suspicion is very important when there is a typical
Final Comments: In adult-onset forms, diagnostic delay cognitive changes present in carriers; such changes are phenotype associated with refractory seizures, requiring
of MELAS is common. In addition, collection by blood or closely connected to neuroanatomical variations triggered evidence of lissencephaly by imaging.
oral swab is the most performed, although urinary anal- by genetic reasons. This disorder, therefore, manifests
ysis has a higher diagnostic yield. itself as a peculiar intersection of genetic, neurological,
and psychiatric factors, thus being a fertile field for the Neurogenética
development of research that integrates several areas of
Neurogenética medical knowledge.

Neurogenética

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XXX Congresso Brasileiro de Neurologia

TL 1105904 TL 1106067 TL 1106107

SUSCEPTIBILITY TO INFECTION-INDUCED RECESSIVE TAY-SACHS DISEASE: A BRAINSTEM ISCHEMIC SYNDROME IN


ACUTE ENCEPHALOPATHY (IIAE1/ANE1) CLINICAL CASE STUDY JUVENILE NEUROFIBROMATOSIS TYPE 2:
TRIGGERD BY COVID-19 CASE PRESENTATION
Isabela Pires Porto Santarém Pinto, Ana Beatriz
Helena Fussiger, Bruna Klein da Costa Vieira Pacheco, Thiago Parreira Guzzo Ian Silva Ribeiro, Karla Rafaele Silva Vasconcelos,
Sarah Diógenes Alencar, João Igor Dantas Landim,
Hospital São Lucas da Escola de Medicina da Unesc / Uninassau – Vilhena – RO – Brazil
Matheus da Costa Guedes, Ana Silvia Sobreira Lima
Pontifícia Universidade Católica do Rio Grande do Verde, Gabrielle Brito Bezerra Mendes, Álissa Elen
isabela.porto27@gmail.com
Sul. Porto Alegre RS, Brazil Formiga Moura, Karoline Ferreira Menezes Mororó,
Hospital São Lucas da Escola de Medicina da Fernanda Martins Maia Carvalho
Case Presentation: Male patient, 3 years and 8 months
Pontifícia Universidade Católica do Rio Grande do
old, born in Brazil, son of parents with no history of
Sul. Porto Alegre RS, Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil
consanguinity, pregnancy discovered at 5 months, with
Universidade de Fortaleza. Fortaleza CE, Brazil
helena.fussiger@gmail.com prenatal care and delivery without complications. The
patient has a family history of a first cousin with the dis- iansilvarbr@gmail.com
Case Presentation: A 3-year-old male patient had a first ease. History of developmental regression from baseline
episode of epileptic seizure at the age of 1 year, 2 days to 2 years, with progression to global ataxia. Cranial mag- Case Presentation: An 18-year-old male patient admitted
after the onset of fever. After this episode, he had several netic resonance imaging showed signal alteration with to our Hospital complaining of intense headache, with
Hospitalizations for epileptic seizures, always preceded atrophic effect affecting the white matter adjacent to spontaneous relief after three days, evolving then with
by fever, and in the last one he had status epilepticus the trigones and posterior horns of the lateral ventricles, left hemiparesis that worsened during the first week and
and required sedation. His cerebrospinal fluid showed hypersignal and volumetric reduction of the inner and with posterior gradual improvement. Physical examina-
increased proteinorrachia (122), 6 red blood cells and posterior surface of the thalamus. Fundoscopy showed tion demonstrates left spastic hemiparesis with hyper-
10 cells (69% macrophages) and MRI of the Skull showed cherry red spots in the macular area bilaterally. Genetic reflexia and right third cranial nerve palsy (congenital).
hypersignal on T2 FLAIR temporal poles, external capsule, panel showing homozygous pathogenic mutation of the Ectoscopy showed multiple fibroelastic skin nodules and
insula and posterior trigone, bilaterally. Normal interictal HEXA gene and deficiency of oxidative phosphorylation left crystalline lens opacity. Patient brought a magnetic
electroencephalogram. In 1 year, when he had COVID, he by mutation in the PNPT1 gene compatible with the resonance (MRI) (Jul-15, 2021) that showed a focus of T2/
started with severe epileptic encephalopathy, requiring diagnosis of Tay-Sachs disease, with Sodium Valproate FLAIR and DWI hyperintense signal with reduced ADC
ICU admission. New new MRI showed extensive white being prescribed for the onset of crises. At 2 years and values, contrast ring enhancement on T1 and SWI slight
matter lesions on T2 FLAIR. After treatment with pulse 11 months, the patient had preserved transitive praxis, hypointense signal localized in right midbrain and pons
therapy with corticosteroids and tocilizumab, he had a global ataxia, axis instability without preferential fall, measuring 2x2,5 cm indicating hemorrhagic-ischemic
response. When he started with fever again, he made broad-based gait and little movement of the upper limbs, injury or neoplastic nature lesion (glioma). Subsequent
early use of steroids, preventing further deterioration. exalted global osteotendinous reflexes with reduced axial MRI (Ago-06, 2021) showed extension of the lesion to
His father was diagnosed with encephalitis at age 4. After tone, and preserved trophism. At 3 years and 5 months, right internal capsule persisting the same patterns of
Exome sequencing, found a probably pathogenic vari- he developed seizures that had not been contained with signal. Stroke mechanism was extensively investigated
ant in heterozygosis in the RANBP2 gene c.1754C>T: p. prescribed medication since the time of diagnosis. The with no relevant findings. Because of the possibility of
(Thr585Met), suggesting the diagnosis of Susceptibility to electroencephalogram showed changes with generalized neoplastic nature of the lesion, patient underwent MR
Infection-Induced Acute Encephalopathy (IIAE1/ANE1), epileptiform activity and carbamazepine was prescribed, spectroscopy and perfusion (Oct-10, 2021) in which it
an autosomal dominant disease. Segregation analysis with a 90% regression in the number of seizures after two was noticeable volume reduction of the initial lesion,
showed that her father is also a carrier of the variant. days of medication. Discussion: Tay-Sachs disease is a rare persistent restricted diffusion in addition of multiples
Discussion: The most frequent clinical presentation of genetic disease transmitted from parents to children. It contrast enhanced nodules of cranial nerves and cervical
IIAE1/ANE1 is characterized by three stages: prodromal is caused by the absence of an enzyme that helps break roots. The spectroscopy study showed discreet increase
(febrile viral infection), acute (encephalopathy, on aver- down fatty substances called gangliosides, which reach of choline and lactate indicating ischemia. CBV perfu-
age 3 days after the beginning of the prodromal phase, toxic levels in the brain and spinal cord and affect nerve sion MR values were normal. This set of findings may
which may be accompanied by epileptic seizures and cell function. Signs and symptoms of the disease usu- suggest the diagnosis of brainstem ischemic syndrome
focal neurological signs). and recovery. Episodes can be ally begin at 3 to 6 months of age. Having a reduction in in juvenile neurofibromatosis type 2 (NF2). Discussion:
recurring. MRI findings are characterized by T2/FLAIR muscle development and atrophy, evolving, over time, to NF2 is an autosomal-dominant inherited tumor-prone
hypersignal in the thalamus and brainstem. The outcome seizures, loss of vision and hearing, paralysis and other disorder, predisposing the occurrence of meningiomas,
ranges from complete recovery to persistent deficits major problems. Tay-sachs disease results in mortality ependymomas and specially schwannomas. Patients are
and death. Several infectious triggers have already been within a few years of life. Final Comments: This case study also characterized by skin abnormalities, juvenile cata-
reported, but here we report one of the first triggered by reveals the importance of clinical and imaging follow-up ract, retinal hamartoma and scoliosis. Case series studies
COVID-19. Early high doses of corticosteroids seem to of patients with diseases whose prognosis is reserved, identified progressive brainstem ischemia as a rare pre-
be the best treatment. IL6 (tocilizumab) and TNF alpha as this, associated with adequate therapy, brings results sentation of NF2 occurring in young patients. These cases
inhibitors appear to play a role, but there are very few that in the short term provide a better quality of life for had no obvious reason for stroke like occlusion of a visi-
studies to date. Final Comments: Although rare, the the patient and family. ble larger vessel, especially not in the vertebral or basilar
investigation of genes associated with susceptibility to arteries. Thus, a microvascular affection is the most likely
Acute Encephalopathy should be investigated in recurrent cause and the midbrain/brainstem area seems to be the
cases triggered by infectious triggers, as early treatment Neurogenética predilection site for these ischemic events in NF2. Final
with corticosteroids or immunomodulators seems to Comments: We presented a case of progressive brainstem
change the outcome of patients. ischemia with persistent restricted diffusion on MRI after
3 months, representing a challenge diagnosis due to its
rarity and differential diagnosis with neoplastic lesions.
Neurogenética

Neurogenética

116
XXX Congresso Brasileiro de Neurologia

TL 1106177 TL 1106256 TL 1106349

POMPE DISEASE (PD) IN A PATIENT WITH AUTOSOMAL DOMINANT HEREDITARY ADULT-ONSET LEUKOENCEPHALOPATHIES:
ACROMEGALY PREVIOUSLY DIAGNOSED AS SPASTIC PARAPLEGIA WITH PURE A DIAGNOSTIC CHALLENGE
POLYMYOSITIS – A CASE PRESENTATION PHENOTYPE AND EARLY ONSET CAUSED BY
Vanessa Freitas Moreira, Déborah Inayara Mendes
VARIANT IN THE KIF1A GENE Tenório Albuquerque, Davi Vargas Freitas Teixeira,
Jorge Luiz de Brito de Souza, Rebeca Bessa
Maurício, Pedro Helder de Oliveira Junior, Davi Lopes Helena Helena Fussiger Andreas Batista Schelp, Amanda Freitas Alves,
Santos, Késia Sindy Alves Ferreira Pereira, Ingred Sephora Sabrina Candido Almeida, Erick Dupont,
Pimentel Guimarães, Vitória Maria Torres Peixoto, Universidade FEEVALE – Porto Alegre – RS – Brazil Roberta Arb Saba Rodrigues Pinto
Pedro Braga Neto, Manoel Alves Sobreira Neto,
helena.fussiger@gmail.com IAMSPE, São Paulo SP, Brazil
Paulo Ribeiro Nóbrega
Case Presentation: A 14-year-old male patient started vf_moreira@hotmail.com
Universidade Estadual Ceará. Fortaleza CE, Brazil
Universidade Estadual do Ceará. Fortaleza CE, walking on tiptoe at one and a half years of age. These
symptoms evolved progressively. On current physical Case Presentation: Male patient, 58 years old, hyperten-
Brazil
examination, he has grade IV strength in dorsiflexion sive, with a history of colon cancer in 2011 (treated with
Hospital Universitário Walter Cantídio. Fortaleza
of the feet and interosseous in the hands, patellar and resection and chemotherapy) and hypogonadotropic
CE, Brazil
achilleus hyperreflexia, bilateral Achilles clonus, bilat- hypogonadism. He began with attention difficulties in
jorge.brito@aluno.uece.br eral Babinski sign and reduced vibratory sensitivity. Mild September/2021, which worsened over the next months
hyperreflexia in upper limbs. He also has low-amplitude, with cognitive-behavioral changes like aggressiveness,
Case Presentation: A 46-year-old male patient, sixth high-frequency postural and kinetic tremor. His parents irritability and apathy, sleep disturbances, memory dis-
son of non-consanguineous parents and with no similar are not consanguineous and there are no similar cases function and difficulties to perform daily tasks (paying
family history, was previously diagnosed with acromegaly in the family. He performed investigation of sporadic bills on the phone, financial controlling). On neuro-
(enlargement of nose, hands and ears, mandibular prog- and metabolic causes that showed no changes, as well logic examination, the patient only presented cognitive
nathism and deep voice) due to a pituitary adenoma. Six as MRI of the skull and spinal cord. We opted for genetic changes, with pronounced executive dysfunction. He was
years ago he started to have proximal muscle weakness. investigation with a diagnosis of pure Hereditary Spastic then Hospitalized for investigation. Cerebrospinal fluid
Muscle biopsy revealed mild muscle atrophy with a lym- Paraplegia (HSP), as an isolated case, through a panel of examination showed slightly elevated protein level, and
phocyte infiltrate, suggestive of an inflammatory myop- genes. In this, the probably pathogenic variant was found brain MRI showed symmetric frontal white matter hyper-
athy. He was diagnosed with polymyositis and treated in the KIF1A gene c.1021A>G (p.Thr341Ala), in heterozy- intensity with spared subcortical U fibers on FLAIR images,
with prednisone, with little response. One year later gosity. This was also evaluated in their parents, who did besides no contrast enhancement and no restricted diffu-
he was forwarded to a neurology outpatient clinic with not have the mutation. Thus, the diagnosis of SPG30, pure sion foci. Metabolic, infectious, and serologic tests were
progressive fatigue, difficulty standing up and low back form, autosomal dominant (AD), with early onset, was normal. The patient underwent further investigation,
pain. Symptoms have become worse over time. The last concluded. Discussion: Hereditary Spastic Paraparesis including genetic testing to Adult Leukodystrophies, which
examination showed grade 4 muscle weakness globally is a heterogeneous group of rare neurogenetic disorders revealed heterozygosity for the GBE1 gene. Discussion:
and marked hypotrophy of proximal muscles, including caused by variants in more than 80 genes, with all forms Leukodystrophies are hereditary disorders that present
pectorals, associated with a myopathic gait. Deep tendon of inheritance already described. They are divided into with progressive white matter dysmyelination or defects
reflexes were hypoactive globally. Plantar responses were pure or complicated forms, according to the clinical pre- of myelin biosynthesis and metabolism. The pattern
indifferent. Cranial nerves, sphincter function and bulbar sentation. SPG30, caused by variants in the KIF1A gene, of white matter involvement is essential to narrowing
muscles were preserved. He had elevated creatine kinase which encodes a motor protein expressed exclusively in the diagnosis process. Although rare, it is an important
(327 IU/L). A next-generation sequencing panel for neuro- brain tissue and transports cargoes from the neuronal differential diagnosis to be considered in patients with
muscular disorders was performed and showed two GAA body to the periphery of neurites. In addition to the two cognitive decline. Even with advances in imaging and
pathogenic variants (c.-32-13T>G – intronic; and Exon 18 forms of inheritance, AD conditions have already been genetic testing, about 40% of patients remain undiag-
deletion). Enzymatic testing for acid alpha-glucosidase described as pure in some families and as complicated nosed. Mutations in the GBE1 gene is associated with
was 0,64 (reference range – 4.57 ± 1.37 umol/L/h), which in others, and also as a cause of polyneuropathy, some- Adult Polyglucosan Body Disease, an autosomal reces-
confirmed Pompe disease (PD). Discussion: PD is still times making it difficult to confirm the pathogenicity of sive disease that presents as neurodegenerative condition
underdiagnosed in Brazil and may affect people of all the variant. Few cases with this phenotype have been with involvement of the central and peripheral nervous
ages with varying degrees of severity, usually presenting described in the brazilian population. Final Comments: system. Final Comments: Leukoencephalopathy disor-
as a limb-girdle myopathy, predominantly in lower limbs. A better understanding of the variants found in the brazil- ders represent a diagnostic challenge for the neurologist,
Symptoms such as dysarthria, dysphagia, scoliosis, pain, ian population and, consequently, which forms of HSP we requiring an extensive investigation, including infectious,
fatigue and sphincter involvement can also be found. Mild find here, helps in the interpretation of molecular results immune-mediated, degenerative, vascular, metabolic, and
PD is common in late-onset patients. Because of its similar and even in the creation of more directive gene panels, neoplastic process, in addition to genetic causes. This last
presentation, PD can be mistaken for polymyositis, which depending on the origin of the patient. In addition, better group constitutes the major diagnostic challenge, mainly
also exhibits a progressive proximal weakness pattern. This understanding the changes caused by mutations in the due to the availability of diagnostic resources in health
patient had a biopsy reportedly compatible with polymy- KIF1A gene further broadens the spectrum of diseases services. For this reason, a systematic approach to the
ositis, but search for serum antibodies was negative and associated with it. investigation of these patients is mandatory.
corticosteroid therapy didn’t show any improvement. In
those situations, testing for genetic myopathies might
reveal alternative diagnoses of potentially treatable con- Neurogenética Neurogenética
ditions, such as PD. Final Comments: To the best of our
knowledge, this is the first case of PD in a patient with a
typical presentation of acromegaly. Here we also high-
light the recognition of Pompe disease as an important
differential diagnosis of polymyositis. This knowledge can
reduce the delay in etiologic determination and might
reveal potentially treatable conditions.

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XXX Congresso Brasileiro de Neurologia

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EFFECTS OF LONG-TERM TREATMENT IN A A RARE CAUSE OF NEURODEVELOPMENTAL MORC2 VARIANT PRESENTING WITH
PATIENT WITH ISOVALERIC ACIDEMIA DISORDER WITH INVOLUNTARY DEVELOPMENTAL DELAY, DYSMORPHIC
MOVEMENTS: GNAO1 ENCEPHALOPATHY FACIES, POLYDACTYLY, AND MICROCEPHALY
Vitória Maria Torres Peixoto, Gustavo Rodrigues
Ferreira Gomes, Aline Campos Fontenele Rodrigues, Gustavo Rodrigues Ferreira Gomes, Vitória Maria Vitória Maria Torres Peixoto, Gustavo Rodrigues
Erlane Marques Ribeiro, Marcelo de Almeida Torres Peixoto, Aline Campos Fontenele Rodrigues, Ferreira Gomes, Aline Campos Fontenele Rodrigues,
Pinheiro, Tamiris Carneiro Mariano, André Luiz Erlane Marques Ribeiro, Marcelo de Almeida Erlane Marques Ribeiro, Marcelo de Almeida
Santos Pessoa Pinheiro, Tamiris Carneiro Mariano, André Luiz Pinheiro, Tamiris Carneiro Mariano, André Luiz
Santos Pessoa Santos Pessoa
Universidade Estadual do Ceará UECE
Hospital Infantil Albert Sabin HIAS Universidade Estadual do Ceará. Fortaleza CE, Universidade Estadual do Ceará UECE
Brazil Hospital Infantil Albert Sabin HIAS
vitoriamtp12@gmail.com
HIAS. Fortaleza CE, Brazil
vitoriamtp12@gmail.com
Case Presentation: An 11-year-old female patient, daugh- gustavo_r_f_gomes@hotmail.com
ter of consanguineous parents, was born with no compli- Case Presentation: 12-year-old male patient, nonconsan-
cations during pregnancy, childbirth or puerperium. At Case Presentation: A 6 years old girl born from vaginal guineous parents, born at term, with cesarean delivery,
the age of 3 years, she required Hospital admission with delivery, at term, by the age of 8 months started with invol- no complications during pregnancy, childbirth or puer-
a history of recurrent vomiting, fever, metabolic acidosis, untary hyperkinetic jerk movements most notably in the perium. At 9 months, neuropsychomotor development
lethargy, and diarrhea. It was also reported neuropsycho- hands, and jaw, especially when anxious, with progression (NPDM) delay and dysmorphic facies were observed. He
motor development delay. The patient was suspected to of symptoms. Crawling and sitting at the age of 2 years old also presented preaxial polydactyly, which was resolved. At
have encephalitis, which led to treatment with ceftriaxone, and first words at the age of 5. At the age of 6, the patient 12 months, he had an episode of febrile seizure. Posteriorly,
aciclovir and vancomycin, but remained a concern for was admitted to the current service and parents reported it was also verified intellectual disability and hyperac-
underlying metabolic disorder. During Hospitalization, anxious behavior and worsening of the chewing move- tivity. During etiological investigation, brain Magnetic
the patient presented one convulsive seizure, beginning ments and uncoordinated arm movements. Neurological Resonance Imaging (MRI) revealed thin corpus callosum.
phenobarbital therapy. Plasma amino acids and urine examination showed axial hypotonia, compromising trunk Cytogenetic tests were performed, with suspicion of Fragile
for organic acids were performed to investigate meta- sustentation, sialorrhea, uncoordinated crawling,chewing X Syndrome, and identified no alterations. Besides the
bolic disorders. The metabolic studies revealed a marked movements, Babinski sign and achilles tendon shortening. NPDM delay, and dysmorphic facies, the neurological
excretion of isovalerylglycine, along with a moderate A full exome sequencing was performed, demonstrat- examination was notable for generalized hyporeflexia,
excretion of 3-hydroxy-isovaleric acid. The diagnosis of ing, in heterozygous, the variant chr16: 56.351.397 A>T normal strength, microcephaly, and digitigrade gait. An
Isovaleric Acidemia (IVA) was made in the same year. (or alternatively c.737A>T – ENST00000262493), which analysis of the exome sequencing was solicited, which
The genetic examination revealed, in homozygous, the promotes que substitution of the glutamate amino-acid showed, in heterozygous, the variant hr22: 30.958.684
variant p.Arg414Trp C>T in the IVD gene (OMIM 607036). by a valine on the codon 246 (p.Glu246Val) in GNAO1 C>T (or alternatively c.79G>A – ENST00000397641) in
The patient was placed on a protein-restricted diet and (OMIM 139311). This variant has never been described the MORC2 (OMIM 616661) gene, which promotes the
use of L-carnitine 100 mg/kg/day therapy. Eight years in the medical literature. Discussion: GNAO1 encodes substitution of the glutamate amino acid by lysine at the
later, she made significant progress on speech and motor the alpha subunit of the Go subclass of heterotrimeric 27th codon (p.Glu27Lys). This is a rare variant, and was
development, with improvement on strength, gait, and guanine nucleotide-binding proteins (G proteins), which described in the medical literature associated with devel-
academic performance. Discussion: IVA is a rare inborn is expressed in the brain and has an important role in the opmental delay, dysmorphic facies, and microcephaly.
error of leucine metabolism caused by biallelic pathogenic regulation of neuronal excitability and neurotransmis- Discussion: Microrchidia CW-type zinc finger protein 2
variant in the isovaleryl CoA dehydrogenase (IVD) gene. sion. Heterozygous mutations in GNAO1 are associated (MORC2) encodes a DNA-dependent ATPase involved
The accumulation of isovaleric acid, toxic to the central with a wide range of manifestations, from Developmental in epigenetic silencing through chromatin modification,
nervous system, is related to the phenotypic abnormalities and Epileptic Encephalopathy-17 (DEE17) to signifi- necessary for chromatin remodeling, DNA repair, and
in this disorder. Patients may present an acute neonatal cant Neurodevelopmental Disorder with Involuntary transcriptional regulation. Heterozygous variants in the
form of the disease, leading to massive metabolic acido- Movements (NEDIM). Patients with NEDIM may present MORC2 are associated with Charcot-Marie-Tooth disease
sis and rapid death in the neonatal period, or a chronic with severe choreoathetosis, developmental delay, and type 2Z; and developmental delay, impaired growth, dys-
intermittent form, with retarded psychomotor develop- hypotonia. The hyperkinetic movements were often exac- morphic facies, and axonal neuropathy (DIGFAN syn-
ment, aversion to dietary protein, hyperammonemia, and erbated by specific triggers, such as voluntary movement, drome). DIGFAN syndrome is an autosomal dominant
pernicious vomiting. If a patient is not treated on time, the intercurrent illnesses, high ambient temperature, and neurodevelopmental disorder, associated with de novo
patient may proceed to severe acidosis, coma, and death. specific emotions, such as anxiety, a phenotype similar mutations. Besides the mentioned characteristics, the
Therapy consisting of L-carnitine supplementation and to that of the patient described herein. Final Comments: patients may also present delayed acquisition of motor
protein restriction may be sufficient for effective manage- Our study reports a brazilian patient with NEDIM, con- skills, gait abnormalities, hyporeflexia, and abnormalities
ment of IVA, starting as soon as possible after birth. Final firming the association between the GNAO1 p.Glu246Val on brain imaging, a phenotype similar to that of the patient
Comments: Our results demonstrate the neurocognitive pathogenic variant and this phenotype, which has not described herein. Final Comments: Our study presents a
improvement of long-term treatment with L-carnitine been described before in the literature. Therefore, we 12-year-old male with a rare MORC2 p.Glu27Lys hetero-
supplementation and protein restriction in our patient encourage that GNAO1 pathogenic variants should be zygous pathogenic variant. We highlight the importance
with IVA. We encourage the analysis for this disorder taken into consideration when investigating patients of MORC2 variants analysis in front of patients with global
when investigating patients with recurrent vomiting and with unexplained early-onset hyperkinetic movement developmental delay, short stature, microcephaly, and
lethargy, born from consanguineous marriage. disorders and neurodevelopmental delay. variable dysmorphic facies.

Neurogenética Neurogenética Neurogenética

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PHENOTYPIC VARIABILITY IN 3 SIBLINGS MULTIPLE ABDOMINAL ANEURYSMS IN AN NOVEL SH3TC2 GENE MUTATION IN A
WITH CLCN1 ASSOCIATED BECKER’S ASYMPTOMATIC LAMA2 MYOPATHY AND BRAZILIAN INDIVIDUAL WITH CHARCOT-
MYOTONIA CONGENITA LEUKOENCEPHALOPATHY MARIE-TOOTH DISEASE TYPE 4C
Rebeca Bessa Maurício, Jorge Luiz de Brito de Jorge Luiz de Brito de Souza, Rebeca Bessa Graziella Aguiar Santos Faria, Maria Sheila
Souza, Vitória Maria Torres Peixoto, Antonio Andrei Maurício, Dánton Campos de Queiroz, Gustavo Guimarães Rocha, Pedro Henrique Marte de Arruda
da Silva Sena, Lorenzo Marinho Morais, Gustavo Rodrigues Gomes, Antônio Andrei Sena, Tito Bastos Sampaio, Cristiane de Araujo Martins Moreno,
Rodrigues Ferreira Gomes, Tito Bastos Siqueira Soares, Lorenzo Marinho Morais, José Daniel Castro, Eduardo de Paula Estephan
Soares, Manoel Alves Sobreira Neto, Pedro Braga Pedro Braga Neto, Paulo Ribeiro Nóbrega
Neto, Paulo Ribeiro Nóbrega Departamento de Neurologia – Hospital Santa
Universidade Estadual do Ceará. Fortaleza CE, Marcelina, São Paulo SP, Brazil
Universidade Estadual do Ceará UECE Brazil Departamento de Neurologia – HC/FMUSP, São
Hospital Universitário Walter Cantídio HUWC Universidade Federal do Ceará. Fortaleza CE, Brazil Paulo SP, Brazil
Hospital Universitário Walter Cantídio. Fortaleza Departamento de Neurologia – Hospital Santa
rebecabessam@gmail.com CE, Brazil Marcelina e HC/FMUSP, São Paulo SP, Brazil

Case Presentation: Three affected siblings whose parents jorge.brito@aluno.uece.br graziasf@hotmail.com


and grandparents were consanguineous. The parents had
3 affected and 7 non-affected children. Case 1: A 40-year- Case Presentation: A 50-year-old female, hypertensive, Case Presentation: A 42-year-old brazilian male with
old woman presented with a history of muscle cramps, daughter of non-consanguineous parents, presented a history of hypertension was admitted to our medical
difficulty standing-up after being seated for a long period, with acute pain at the upper left abdominal quadrant. department due to progressive numbness and weak-
stiffness in legs while walking, dysphonia and sporadic Abdominal ultrasound revealed left non-obstructive ness in the lower extremities. The patient described his
transfer dysphagia. She exhibited pronounced muscular renal lithiasis. Computed tomography scan incidentally motor performance as relatively poor compared to his
hypertrophy and generalized myotonic phenomenon showed multiple saccular aneurysms close to the renal peers since childhood. At age 39, he noticed decreased
(GMP) at examination, was able to walk unassisted and hilum and to the splenic hilum, in addition to expressive sensation in his feet, causing difficulty in wearing shoes
did not need medication for myotonic phenomenon. volumetric loss of paravertebral and lumbar dorsal spinal and walking. He slowly developed progressive weakness
Electroneuromyography (EMG) showed diffuse myo- muscle bellies. Her creatine phosphokinase (CPK) was of lower limbs, unsteadiness, and clumsiness at the age
tonic discharges. Case 2: A 48-year-old male presented 359 IU/L. A next generation sequencing panel for neuro- of 41. He is the son of a consanguineous couple and has a
with GMP and complaints of dysphagia for liquids, diffi- muscular disorders revealed two pathogenic variants in sister with similar symptoms. Physical exam was remark-
culty climbing stairs and brushing his teeth. He reported LAMA2: c.2461A>C (p.Thr&21Pro). Pulmonary function able for pes planus deformity, stork legs, and scoliosis.
weakness since childhood and had frequent falls and tests disclosed only mild obstructive respiratory disorder. Neurological examination revealed slowing of saccades,
needed intermittent assistance when walking. Myotonic Brain MRI revealed extensive involvement of the white hypoacusis, occasional tongue fasciculations, and mild
phenomenon did not respond well to carbamazepine or matter of the cerebral hemispheres, mainly in the deep facial diplegia. He had weakness of the proximal (4+/5)
phenytoin. EMG demonstrated non-dystrophic myotonic regions of the brain, confluent and bilateral, compatible and distal lower limbs (4-/5) with relatively preserved
syndrome. Case 3: A 51-year-old male with a history of with leukoencephalopathy. Discussion: LAMA2-Related muscle strength of the upper limbs. Sensation of vibra-
muscle cramps, dysphagia and dysphonia, muscle stiffness Muscular Dystrophy (LAMA2-MD) is part of a group of tion, light touch, and pinprick were markedly reduced in
with difficulty running and standing-up after being seated muscle disorders called Congenital Muscular Dystrophies both lower extremities. Hyporeflexia, a broad-based gait,
for a long period. Examination showed GMP, hyporeflexia (CMD) and the clinical manifestations represent a contin- and a positive Romberg sign were also noted. iochemical
and myoclonus in upper and lower limbs. Myotonic phe- uous spectrum, varying from a severe congenital muscular analysis showed elevated serum creatine kinase (1278
nomenon was well controlled with phenytoin 200mg/day. dystrophy to milder late-onset LAMA2-MD. Late-onset U/L) and neuron-specific enolase (10 ?g/L). His com-
None of the siblings reported exacerbation of myotonic LAMA2-MD usually presents with proximal muscle weak- plete blood cell count, electrolytes, HbA1C, Vitamin B12,
phenomenon by cold. The 3 siblings were submitted ness (not present in our case) and delayed motor mile- folic acid, liver, thyroid, and kidney function tests were
to a genetic panel for neuromuscular disorders which stones (but independent ambulation can be achieved). all unremarkable. Autoimmune panel, serum tumor
revealed a CLCN1 c.1886T>C variant in homozygosity. A leukoencephalopathy is also present and is an import- markers, and cerebrospinal fluid analysis disclosed no
Discussion: Myotonia Congenita is an inherited disorder ant finding to suggest this diagnosis. Reported atypical abnormalities. Electrophysiological studies indicated a
caused by biallelic pathogenic variants in CLCN1, which findings include seizures, rigid spine syndrome with joint symmetrical, length-dependent, demyelinating senso-
encodes chloride channel ClC-1 expressed predominantly contractures, progressive respiratory insufficiency and rimotor polyneuropathy with axonal loss and markedly
in skeletal muscles. These variants cause muscle hyper- cardiomyopathy with or without conduction defect, but reduced motor conduction velocity. Genetic testing rev-
excitability and delayed muscle relaxation. The disease aneurysms were not yet mentioned. LAMA2 pathogenic eled a novel homozygous pathogenic variant, c.1144G>T
usually presents in childhood but has a highly variable variants can lead to defects in laminin alpha-2 protein, p.Glu382*, in SH3TC2. Discussion: Our patient presented
phenotype even in patients with the same variants, such also called merosin. As merosin is a subunit of laminin with the typical features of Charcot-Marie-Tooth disease
as in this family, where one patient had muscle hypertro- and laminin isophormes are expressed in a lot of tissues, type 4C (CMT4C), including demyelinating neuropathy,
phy and mild myotonic phenomenon without disability including endothelial and perivascular basement mem- foot deformity, scoliosis, and cranial nerve involvement.
while her brother had intense, drug-resistant myotonic branes, we might observe vascular abnormalities as the We identified a new mutation in SH3TC2, predicted to
phenomenon leading to falls, difficulty walking and quality aneurysms described in this case. Final Comments: Here be disease causing since it results in a very premature
of life impairment. Final Comments: Our study reports we report a case not yet described, as far as we know, of stop codon at amino acid position 382 and a shortened
three patients with the same pathogenic variant pre- multiple abdominal aneurysms in a patient diagnosed with protein with a potentially abnormal function. The iden-
senting with different phenotypes. There’s still a lack of muscular dystrophy and leukoencephalopathy related to tified variant is absent in 141000 control chromosomes
description on the multitude of repercussions related to LAMA2. Although there is a lack of literature regarding from gnomAD; it has never been reported in the medical
chloride channel alterations. We therefore encourage an association of LAMA2 with aneurysms, we hypothe- literature or in the ClinVar database. Final Comments: In
more research regarding this topic in order to unveil the size that alterations in laminin could interfere with vessel conclusion, we present a brazilian patient with CMT4C
potential repercussions of this condition. wall architecture. associated with a novel mutation of SH3TC2. This case
highlights the importance of considering rare diagnoses
in the new generation sequencing era.
Neurogenética Neurogenética

Neurogenética

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XXX Congresso Brasileiro de Neurologia

TL 1106567 TL 1106598 TL 1106622

PHENOTYPIC VARIABILITY IN TWO ANALYSIS AND THE IMPORTANCE OF THE CEREBELLAR ATAXIA ASSOCIATED WITH
SIBLINGS WITH UNVERRICHT-LUNDBORG DIFFERENTIAL DIAGNOSIS BETWEEN LANGERHANS-CELL HISTIOCYTOSIS:
DISEASE: JUVENILE MYOCLONIC EPILEPSY KENNEDY’S DISEASE AND AMYOTROPHIC A CASE PRESENTATION
PHENOTYPE X CLASSIC PHENOTYPE LATERAL SCLEROSIS
Jorge Luiz de Brito de Souza, Franklin de Castro
Jorge Luiz de Brito de Souza, Rebeca Bessa Isabel Maria Oliveira Macedo Lima, Pedro Henrique Alves Neto, Rebeca Bessa Maurício, Pedro Lucas
Maurício, Késia Sindy Alves Ferreira Pereira, Ingred Ximenes Ramalho Barros, Luma Rodrigues da Silva, de Souza Barroso, Davi Lopes Santos, Samuel
Pimentel Guimarães, Miguel Vieira de Almeida, Assiria Leite de Azevedo Costa, Hitalo Roberto Cavalcante Marinho, Danyela Martins Bezerra
Antônio Alves Sobreira Neto, Chiara Gübel Portugal, Araújo Coêlho, Joana Clara Oliveira Macedo Lima, Soares, Manoel Alves Sobreira Neto, Pedro Braga
Pedro Braga Neto, Manoel Alves Sobreira Neto, Kelson James Almeida Neta, Paulo Ribeiro Nóbrega
Paulo Ribeiro Nóbrega
Universidade Federal do Piauí. Teresina PI, Brazil Universidade Estadual do Ceará UECE
Universidade Estadual do Ceará UECE Universidade Estadual do Piauí. Teresina PI, Brazil Hospital Universitário Walter Cantídio HUWC
Hospital Universitário Walter Cantídio HUWC Universidade Estadual do Ceará. Fortaleza CE,
isabelmariaoliveiramacedolima@gmail.com Brazil
jorge.brito@aluno.uece.br Hospital Universitário Walter Cantídio. Fortaleza
Case Presentation: Male, 57 years old felt weakness in CE, Brazil
Case Presentation: Here, we present two siblings with the right upper limb in rural work, dropping the instru-
consanguineous parents (first-degree cousins) and an ment used five years ago. Three years ago he presented jorge.brito@aluno.uece.br
uncle with a history of epilepsy. Case 1: A 28-year-old difficulty in flexion of the right index finger and atrophy
female with a history of myoclonic epilepsy since she was of the right upper limb, diagnosed with ALS. The symp- Case Presentation: A 36-year-old male patient suffered
10-years-old that became worse over time. She had normal toms started on the right side and progressed to the 2 episodes of skull fracture related to low kinetic energy
psychomotor development until 11-years-old. Physical left side. He also reports polyuria, urgency, cries easily, accidents at the age of 3. When he was 4 years old, con-
examination demonstrated global ataxia, intense action imbalance and falling, night sweats and coldness in the sidering the imaging alterations and skull bones fragility,
myoclonus and psychotic symptoms. Genetic testing upper limbs. History of 10kg loss and use of Riluzol 50mg. a bone biopsy was performed and suggested Langerhans-
revealed homozygous variant for the dodecamer expan- On physical exam he presented grade 3 strength deficit Cell Histiocytosis, which was confirmed with immunohis-
sion in the cystatin B (CTSB) gene, which confirmed the in the four limbs, fasciculations in the right upper limb, tochemistry. The patient was submitted to chemotherapy
clinical diagnosis of Unverricht-Lundborg Disease (ULD). upper back and tongue that presented atrophied right for 1 year and 6 months with complete remission. When
She scored 21/30 in the Mini-mental state examination muscles, interosseous atrophy of hands, hyporeflexia and he was 12 years old, gait ataxia, urinary volume increase
(MMSE) and 28 in the Scale for the Assessment and Rating staggering gait. Discussion: Spinal and bulbar muscular and cognitive delay started. At 14 years, ataxia stabilized.
of Ataxia (SARA). Brain MRI disclosed diffuse cerebral and atrophy (SBMA) or Kennedy’s disease, is a rare, X-linked, He was restricted to a wheelchair due to gait impairment.
cerebellar atrophy. Case 2: Brother of Case 1, 23-year-old, inherited lower motor neuron disease. It is characterized Cerebellar atrophy was identified by Magnetic Resonance
who developed tonic-clonic seizures and occasional myoc- by progressive muscle weakness. An expanded trinucle- Imaging (MRI). Diabetes Insipidus (DI) was diagnosed
lonus that started at 11-years-old. Psychomotor develop- otide repeat (CAG > 37) in the androgen receptor gene, and therapy with desmopressin was introduced. During
ment was normal. On examination he didn’t exhibit action which encodes glutamine, is the responsible mutation. the following years, the patient presented pseudobulbar
myoclonus. Coordination tests and gait were preserved. Toxicity of this mutant protein affects both motor neu- affect, lower limbs myoclonus, dysphagia and dysarthria.
He scored 30/30 in the MMSE and 29/30 in the Montreal rons and muscles. The main symptoms are weakness in An MRI of the spine when he was 33 years old revealed T2
Cognitive Assessment (MOCA). Electroencephalography the lower limbs, more proximal than distal, hyporeflexia, hypersignal in the lateral funiculi of the whole spinal cord,
showed sparse paroxysms of generalized spike-wave and fasciculations, and muscle atrophy. Non-motor symptoms more prominent in the thoracic segment. A skull MRI when
polyspike-wave epileptiform activity, with normal baseline include dysphagia, dysarthria, testicular atrophy, Brugada he was 35 years old showed midbrain and pontocerebel-
activity. Brain Magnetic Resonance Imaging was normal. syndrome, uriary disorders, and dementia due to reduced lar atrophy with central pontine hypersignal and marked
Discussion: Unverricht-Lundborg Disease (ULD) is a rare, frontal brain volume. Amyotrophic Lateral Sclerosis is the globus pallidus T2 hyposignal, in addition to focuses
disabling and severe genetic condition associated with main differential diagnosis and is ruled out by the absence probably representing hemosiderin or calcifications at
pathogenic variants in CSTB. ULD is considered the most of hyperreflexia and sensory changes. Early diagnosis is the temporo-occipital transitions and a slight increase in
common form of Progressive Myoclonus Epilepsy (PME). important because the prognosis is related to the risk of the supratentorial cerebrospinal fluid spaces. Discussion:
In our cases, the sister expressed a classic ULD phenotype weakness-related falls and bronchoaspiration, and sudden Langerhans-cell histiocytosis (LCH) is considered the most
while the brother presented a relatively mild phenotype, death from Brugada Syndrome. Final Comments: SBMA common histiocytic disorder and is associated with an
which can be mistaken for similar conditions as Juvenile is not only a lower motor neuron disease, but a complex abnormal accumulation of immature histiocytes in some
Myoclonic Epilepsy (JME). The relative intensity of the disorder affecting different systems, including the central parts of the body, usually bone, skin, lungs and pituitary.
various symptoms and the speed at which the disease pro- nervous system. The differential diagnosis with (ALS) is Diabetes insipidus (DI) is the most common neurological
gresses can vary, even within the same family. Therefore, of utmost importance, since the age of presentation of manifestation, but cerebellar ataxia has been reported as
it isn’t possible to assume that the brother’s disease will SBMA is 30-40 years and the patient is already in a very an extremely rare presentation and may mimic an autoso-
progress in a similar way to his sister’s condition. Final advanced picture at diagnosis. mal recessive ataxia. The mechanisms leading to cerebellar
Comments: In this report we aimed to describe the first ataxia in Lagerhans-cell histiocytosis are still unclear. In
case of a JME-like phenotype and a classic ULD phenotype our case, DI and ataxia presented almost simultaneously,
in siblings. This highlights the possibility of searching for Neurogenética and the patient also exhibited pyramidal syndrome and a
ULD in some cases of families with clinical diagnosis of pontine gliosis that may be related to osmotic demyelin-
juvenile myoclonic epilepsy. ation events. Final Comments: Ataxia is a rare finding in
LCH with a still unclear pathogenesis. It is possible that
in some cases neurological complications of this disease
Neurogenética are related to osmotic demyelination episodes caused by
diabetes insipidus.

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XXX Congresso Brasileiro de Neurologia

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MARBURG MARIANT OF MULTIPLE HASHIMOTO ENCEPHALOPATHY: INTRACRANIAL HYPERTENSION AS A


SCLEROSIS AFTER COVID-19 SUCESSFULLY DIAGNOSTIC CHALLENGE RARE NEUROLOGICAL PRESENTATION
TREATED WITH BETAINTERFERON 1A OF SJÖGREN’S SYNDROME: A CASE
Fábio Henrique Limonte, Lívia Maria Ribeiro Pitaro,
Heitor Cherulli Resende PRESENTATION
Luísa Diógenes Queiroz, Júlio Cesar dos Santos,
Euldes Mendes Junior, Leandro Oliveira, Paulo Maria Lucia V Pimentel, Carlos Otavio Brandão,
FACERES – São José do Rio Preto – SP – Brazil
Marcelino, Samuel Rabelo, Fábio Lima Júnior Washington Bianchi, Dante Bianchi, Bárbara
Hospital Maternidade Nossa Senhora Aparecida –
Caldas Novas – GO – Brazil Sampaio Souza Dias, Frederico Del Duca Frazao,
Faculdade de Medicina do Centro Universitário
Laís Felix Teieira da Cunha, Pollyana Marcela Silva
Christus, UNICHRISTUS. Fortaleza CE, Brazil
ly-pitaro@hotmail.com de Farias, Raquel Quimas Molina Costa
Universidade Federal da Paraíba, João Pessoal PB,
Brazil Santa Casa de Misericórdia do Rio de Janeiro. Rio de
Case Presentation: Woman, 49 years old, with hypothy-
Universidade Federal de Minas Gerais. Belo Janeiro RJ, Brazil
roidism and fibromyalgia. The first symptom reported by
Horizonte MG, Brazil NEUROLIFE. Rio de Janeiro RJ, Brazil
her husband was crisis of anxiety that in a monthwas more
Universidade Federal de São Paulo. São Paulo SP,
frequent and associated with hallucinations. In 2 months, basampaio__@hotmail.com
Brazil
he noticed changes in memory with temporo-spatial dis-
luisadiogenessss@gmail.com orientation and difficulty in recognizing family members. Case Presentation: A healthy, young female patient
Involuntary tremors in the upper limbs of myoclonus were presented a sudden short-term loss of consciousness.
Case Presentation: The Marburg variant of MS is a rare observed more frequently during sleep. During a 9-month In the next day she complained of moderate headache
variant that leads to an severe clinical course, with a high diagnostic investigation,the patient underwent a psychi- that worsened in supine position and emesis. It evolved
rate of mortality or severe residual deficits and unclear atrist’s Hospital being treated as a psychotic break. She with bilateral diplopia and convergent strabismus. Brain
pathophysiology. A 20-year old female patient, presented was Hospitalized with the hypothesis of limbic enceph- MRI showed signs of intracranial hypertension (ICH). Her
at the Hospital emergency with left inferior limb paresis alitis after neurologic evaluation. The tests revealed; MRI examination revealed good general condition, gait devi-
and visual blurring. The neurologic exam showed com- of the brain with alteration signal in the hippocampus ation to the right in the Fukuda’s Test, star gait with eyes
plete and proportionate left hemiparesis with pyramidal bilaterally, electroencephalogram with record of electro- closed and bilateral paresis of the VI cranial nerve. Pupils
signs and clonus, loss of proprioception and vibration in graphic crisis and presence of paroxysm in the previous were isocoric, photoreactive, visual acuity was normal, as
lower limbs, tactile and painful hypoesthesia on the left temporal regions bilaterally. Serum tests identified anti- it was the fundoscopy. Lumbar puncture revealed proteins
side. This report describes a rare case of Marburg variant TPO: 940. Markers for autoimmune encephalitis were all 41, glucose 40, negative anti-AQP4, anti-MOG andOCB,
associated with COVID-19 infection. negative and the possibility of paraneoplastic syndrome and intrathecal synthesis of IgA and IgM antibodies, indi-
was ruled out. Pulse therapy with methylpredinisone was cating blood-brain barrier dysfunction, negative IgG, and
performed and lamotriginewas prescribed. The patient intrathecal production of kappa free light chain (Kappa
Neuroimunologia showed significant clinical improvement. Discussion: index 7.23), and neurofilaments = 4852. Laboratory tests
Hashimoto encephalopathy (HE) is a term used to describe showed VHS 34, anti-RO 240, anti-LA 174. She was referred
a rare encephalopathy, of presumed autoimmune origin, to the Rrheumatology Service for Sjögren’s Syndrome
associated with Hashimoto’s thyroiditis. It is included (SS) investigation. Because of complaints of dysphagia
in a group of nonvasculitic autoimmune inflammatory and xerostomy during this period, salivary gland biopsy
meningoencephalopathies. The patient described pre- was performed, and the findings were compatible with
sented all the characteristic symptoms: behavioral and SS. She was treated with corticosteroids, acetazolamide,
psychiatric symptoms, movement disorders and convul- azathioprine, hydroxychloroquine and lubricating eye
sions. The way symptoms are on set up is typically acute or drops, and all symptoms resolved. Discussion: ICH is a
subacute which can become chronic. Final Comments: clinical syndrome characterized by increased intracranial
Behavioral complaints, hallucinations, accompanied by pressure without brain or cerebrospinal fluid lesions that
memory deficits and language disorders, are often found justify it, maybe explained by recent discoveries of the
at an early stage of HE and may be confused with psychi- glymphatic and lymphatic systems of the brain. It mainly
atric disorders. Common differential diagnoses should be affects overweight or obese women. Although female, the
made with Creutzfeldt-Jakob disease, rapidly progressive patient in this case was eutrophic. SS is a rheumathologic
dementias and limbic encephalitis. disease with 8.5 to 70% of the patients presenting with
neurological complains. The diagnosis of SS is based on
clinical grounds and laboratory findings with positivity
Neuroimunologia
of anti-Ro and anti-La in 52% and 34% of the patients
respectively. There are few cases described where ICH
was the primary symptom of SS. In our case, the CSF
analysis revealed a high Kappa index and neurofilaments,
important biomarkers related to important neuroaxonal
damage. Kappa index and neurofilaments are promising
biomarkers for the diagnosis, prognosis and evaluation
of the disease progression in immunomediated compro-
mise of the Central Nervous System. Final Comments:
Although rare, neurological manifestation of SS may be
the first symptom of the disease. Among the neurologi-
cal manifestations described, ICH is even less frequent.

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XXX Congresso Brasileiro de Neurologia

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DIAGNOSTIC CHALLENGES IN ANTI-NMDAR LYMPHOMA ASSOCIATED WITH FINGOLIMOD DIFFERENTIAL DIAGNOSIS IN


ENCEPHALITIS: A CASE PRESENTATION TREATMENT: FIRST REPORT OF ADULT- DEMYELINATING DISEASES: A CASE
ONSET CASE IN BRAZIL PRESENTATION
João Vitor Mortari Lisboa, Isaac Pantaleão Souza,
Pedro Machry Pozzobon, Patrick Emanuell Mesquita Eduardo Claus Constante, Felipe Toscano Lins de Maria Lúcia Vellutini Pimentel, Ana Beatriz da
Sousa Santos, Itamar Meireles Andrade Santos, Menezes, Vinícius Lopes Braga, Wardislau Ferreira, Costa Lucas, Bárbara Sampaio de Souza Dias,
Eduardo Abrão Spinola Rezk, Camila Bonfanti Carina da Silveira Massaro, Jéssica Monique Cícera Simplicio Machado Leandro, Frederico Del
Baima, Laura Cardia Gomes Lopes Dias Alencar, Inara Taís de Almeida, Otavio Cesar Duca Frazão, Gabriel Rivadavia de Farias Amaral,
Carvalho Guimarães Baiocchi, Denis Bernardi Kathleen Torres Tenorio Monteiro, Leticia Fêzer
Unesp – Botucatu – SP – Brazil
Bichuetti, Enedina Maria Lobato Oliveira Mansur, Raquel Quimas Molina da Costa, Rodrigo
João vmlisboa@outlook.com Moreira Pereira
UNIFESP – EPM, São Paulo SP, Brazil
PUC-RJ/SCMRJ. Rio de Janeiro RJ, Brazil
Case Presentation: A 29-year-old woman, lawyer, no eduardoconstanteh@hotmail.com
comorbidities or psychiatric history, presented with bia_lucas@hotmail.com
agitation and insomnia. In few days, there was wors- Case Presentation: A 31-year-old white woman had
ening of symptoms, associated with delusional speech been suffering from relapsing-remitting multiple scle- Case Presentation: A 46 years old female patient came
and executive dysfunction. Present to psychiatry aval- rosis (RRMS) for more than 8 years. She had been tak- to our Neurology Service in 2003 with decreased visual
iation, with TAB diagnoses, Olanzapine started, but no ing fingolimod properly for one and a half years when acuity in her left eye, which evolved to amaurosis after 7
improvement. After three weeks, the patient needs help she developed subacute behavioral changes, dysarthria, days. Brain MRI revealed two hyperintense signs on T2
for basic ativities. She presented perioral moviments and right numbness, and hemiparesis. The general laboratory and FLAIR in the semioval center, without post-contrast
unexplained coma, with Hospitalar addimission. The showed 696 lymphocytes. As if she had relapsed, she enhancement, and hyper signal in the left optic nerve on
MRI, CSF, laboratory tests for infection and metabolismo received intravenous (IV) methylprednisolone with no the STIR sequence. She was treated with pulse therapy
were normal. Acyclovir started due to suspected herpetic significant improvement. Four months later, a repeat MRI and partially improved. She returned after a few months
encephalitis. The patient was transferred to a tertiary scan showed new lesions with gadolinium enhancement complaining of low visual acuity in her right eye. A cer-
center, anti-NMDA encephalitis was suggested and a in the posterior white matter and brainstem, in addition to vical MRI revealed contrast-enhanced lesions in C2-C3,
sample of CSF and serum was sent for antibody testing. meningeal enhancement in the frontal lobe. Fingolimod CSF test was positive for oligoclonal bands (OCB) and a
Screening imaging and markers excluded teratoma or was discontinued because of possible progressive mul- serum aquaporin-4 antibody was negative. She was treated
other occult neoplasms. Empirical treatment was started tifocal leukoencephalopathy. After its discontinuation, with three cycles of pulsetherapy and began Interferon
with methylprednisolone 1g daily for five days, followed two months later, a comprehensive malignancy workup ß1-a 44mcg, being episode free for several years, even
by five sessions of phasmapheresis and rituximab. The failed, including normal CTs, serous immunofixation and though routine brain MRI revealed new lesions in 2009
tests has been found positive for NMDAr antibodies, and beta-2 microglobulin, a cervical lymph node biopsy, and and 2011. In 2020, at the age of 61, she complained of
the pacient remains in intensive care for clinical support. a brain biopsy with typical MS findings. Subsequent CSF paresthesia in her left inferior limb, which ascended to
Discussion: Autoimmune encephalitis is caused by the examination revealed 16 cells, and immunophenotyping the dorsal region. Neurologic examination revealed crural
production of antibodies against neuronal autoantigens. revealed 3% non-neoplastic clonal plasma cells. Given paraparesis and posterior cord syndrome. A new cervical
It affects mostly young women, presenting with acute or this atypical evolution, our team of experts concluded a and dorsal MRI showed an extensive hypointense lesion
subacute psychiatric symptoms, seizures, cognitive defi- diagnosis of B-cell lymphoma during immunosuppres- on T1, and hyperintense on T2, with peripheral contrast
cits, coma, and autonomic dysfunction. Often psychiatric sion in advanced RRMS due to immune reconstitution. enhancement involving the central and posterior por-
symptoms are the initial symptoms and these patients are She received IV rituximab and IV methylprednisolone and tions of the spinal cord, with an expansive aspect from C5
mistakenly treated as primary psychosis or mood disor- had significant improvement. Discussion: Fingolimod is a to D1, surrounded by edema from C2 to D3. A new CSF
ders, leading to diagnostic delay and worse prognosis. The sphingosine-1-phosphate (S1P) receptor agonist and the study demonstrated positive OCB, positive aquaporin-4
association of anti-NMDAr encephalitis with neoplasms first oral agent approved for the treatment of RRMS. After antibody in CSF and serum, and non-reactive anti-MOG.
is large, and investigation should be extensive, focusing phosphorylation in vivo, the drug acts as an S1P receptor A new pulsetherapy and azathioprine were initiated after
on ovarian teratoma. Treatment primarily involves inten- modulator that binds with high affinity to four of the five the denial of rituximab by her healthcare plan, with a
sive care associated with immunosuppressive therapy. known S1P receptors, retaining lymphocytes in lymph good response to treatment. Discussion: Neuromyelitis
The first line constitutes corticoids, immunoglobulin or nodes and preventing their egress into the peripheral optica (NMO) is a demyelinating disease with severe and
plasmapheresis. The second line of treatment is done in circulation. As a result, the infiltration of autoreactive lym- more disabling outbreaks than multiple sclerosis (MS).
selected cases, usually in patients with late diagnosis or phocytes into the CNS is reduced. It is believed that due In this reported case, the patient had neuroimaging stud-
in those with no association with tumor, being Rituximab to this mechanism, fingolimod may alleviate and reduce ies suggestive of MS, with positive OCB in the CSF and
the drug of choice. For refractory cases, Bortezomib is the disease burden of lymphoma, increase the efficacy of negative anti-aquaporin-4 antibody in the serum. After
third line. The prognosis is generally satisfactory, however, other treatment options, and improve the function of the 16 years without any recurrent episodes, or significant
because it is a long-stay disease in the ICU, it is suscep- immune system in destroying neoplastic cells. Therefore, gain of lesion load, she presented with a new severe bout
tible to several clinical and neurological complications the drug can be used not only to treat lymphoma, but also with posterior cord ataxia and crural paraparesia, with
throughout treatment. Final Comments: Anti-NMDA to control and prevent disease recurrence in patients in evidence of extensive myelitis on cervical-dorsal MRI.
encephaltis should be considered as a diagnostic pos- remission. Final Comments: To our knowledge, this Final Comments: As a highly sensitive test, aquaporin-4
sibility in cases of subacute psychiatric alterations with is the first report of fingolimod-associated lymphoma antibody has gained prominence to differentiate MS from
neurological symptoms, and empiric treatment having to confined to the CNS in Brazil. It is a reminder that immu- NMO, increasing the diagnostic accuracy of NMO to 90%
be considered for better therapeutic results. nosuppressive drugs have a full spectrum of side effects, of cases, when associated with clinical criteria.Therefore,
not just in infectious diseases. Both solid tumors and in diagnostic doubt aquporine-4 antibody must be tested
hematologic cancers should be considered as possible more than once.The differential diagnosis between these
Neuroimunologia causes of deterioration. two entities is essential and requires specific treatments.

Neuroimunologia Neuroimunologia

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XXX Congresso Brasileiro de Neurologia

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STEROID-RESPONSIVE PONTINE ATYPICAL PRESENTATION OF ACUTE STEROID-RESPONSIVE ENCEPHALOPATHY


PERIVASCULAR ENHANCEMENT DUE TO DISSEMINATED ENCEPHALOMYELITIS ASSOCIATED WITH AUTOIMMUNE
CHRONIC LYMPHOCYTIC INFLAMMATION (ADEM) IN ADULT AFTER SARS-COV-2 THYROIDITIS (SREAT): A SEVERE CASE IN A
(CLIPPERS) IN A YOUNG PATIENT AS INFECTION YOUNG WOMAN
A DIFFERENTIAL DIAGNOSIS WITH
João Vitor Mortari Lisboa, Isaac Pantaleão Souza, Eduardo Silveira Marques Branco, Paula Carolina
INFLAMMATORY CENTRAL NERVOUS Pedro Machry Pozzobon, Patrick Emanuell Mesquita Grande Nakazato, Luiz Domingos Mendes Melges,
SYSTEM DISEASES: A UNCOMMUN REPORT Sousa Santos, Itamar Meireles Andrade Santos, Isabela Badan Fernandes, Bruno Eiji Nakano,
CASE Eduardo Abrão Spinola Rezk, Danielle Patrícia Gabriel Flamarin Cavasana, Rodrigo Batista Irikura,
Borges Margato, Fernando Coronetti Gomes Rocha, Sarah de Souza Chinelato, Bruna Santos Silva,
Victor Hugo Souza Silva Gomes, Klesia Adaynny
Laura Cardia Gomes Lopes Alana Strucker Barbosa
Rodrigues, Bianca Barbosa Araldi, Bruno Ludivig
Vieira, Isadora Constatini Soares Andrade, Beatriz Unesp – Botucatu – SP – Brazil Faculdade de Medicina de Marília – FAMEMA –
Fulador, Lucas Cruz Farias, Heloise Helena Siqueira Marília – SP – Brazil
João vmlisboa@outlook.com
Hospital Geral Universitário. Cuiabá MT, Brazil pcgnakazato@gmail.com
Universidade de Cuiabá. Cuiabá MT, Brazil Case Presentation: A 31-years-old man, gardener, pre-
viously obese and hypertensive, presented with binocu- Case Presentation: A 39-years-old woman, previously
victorhhugo2005@gmail.com
lar diplopia associated with SARS-Cov-2 infection. After healthy, presented in the Emergency department with
two weeks, he presented gait abnormalities and nauseas tonic-clonic seizures, followed by refractory status epi-
Case Presentation: M.L.N.P, female, 22 y, admitted to
with progressive worsening of symptoms, and onset of lepticus requiring induction of barbituric coma to achieve
a neurology ER reporting a holocranial headache asso-
peripheral facial paralysis. The patient was admitted to seizures control using standard antiseizure drugs in high
ciated with 3 weeks of intermittent rotational vertigo,
Emergency Department for investigation, and the neu- doses. Initial blood test and serologies were normal.
diplopia and strabismus. On physical exam observed
rological examination showed right dysmetria, cerebellar Thyroid function tests showed TSH: 7,98, free T4: 0,81,
sixth cranial nerve ophthalmoplegia leading to right eye
gait, horizontal nystagmus with fast phase to the right, Anti-thyroglobulin: 37,4 and Anti-thyroid-peroxidase:
convergent strabismus and horizontal nystagmus, rapid
and right peripheral facial palsy. CT in admission was 97,6. Cerebrospinal fluid cell count, protein and glucose
phase to the left side, ataxia in left hemibody with dys-
normal. Extensive laboratory tests were normal. CSF has were normal. CSF VDRL and Criptococcal antigen were
metria and ataxic gait. Hypothesis of CNS inflammatory
been showed protein 83mg/dL and 2 cells. MRI showed negative. Cultures for bacteria, fungi and BAAR were
disease was evented and brain MRI with positive signal
in FLAIR and T2 a periaqueductal hypersignal at mid- negative. Brain magnetic resonance imaging revealed in
anomaly in the right nucleocapsular region, extending
brain-bridge transition, in the posterior region of the FLAIR/T2 sequences increased signal in hippocampus
to midbrain, pons, middle cerebellar peduncles and left
midbrain and cerebellar vermis. On T1 contrast, there bilaterally, extending to hypothalamus, with swealling
cerebellar hemisphere, with punctate enhancement was
was gadolinium enhancement. Neoplastic screening features, exhibiting cortical diffusion restriction. The
identified, suggesting CLIPPERS diagnosis. Other labs
revealed no signs of tumor. Then the patient was treated patient received human immunoglobulin, remaining
and supplementary tests were normal results. Performed
with intravenous methylprednisolone 1000mg/d each for with consciousness impairment, orofacial and lower
intravenous therapy with methylprednisolone 1g/d for 5
5 consecutives days. After 5 days, the neurological exam- limbs mioclonus at the end of immunotherapy. Infusion
days and improvement mainly strabismus was identified.
ination showed gait and cerebellar tests normal, with therapy with methylprednisolone were indicated. After
Prednisone 20mg daily was prescribed, in accordance with
persistence of mild facial palsy. New brain MRI showed pulse therapy, the patient showed improvement of con-
literature findings just until Rituximab as a Corticosteroid-
significant improvement in aspect of the lesion, with mild sciousness level and the mioclonus ceased. However,
Sparing Therapy (CST) starts. Outpatient care 30 days
hypersignal in cerebellar vermis. Discussion: ADEM is a within ten days, the seizures recurred, associated with
later, demonstrated no neurological deficits, with remis-
rare demyelinating disease more commonly seen in chil- drowsiness. Prednisone 40mg daily associated with anti-
sion of previous symptoms and new MRI identified no
dren, presenting clinical heterogenic, generally causing seizures drugs gradually improved the clinical picture. She
signs perivascular enhancement. CST was introduced
encephalopathy and multifocal deficits. Several studies was discharged within six days, lucid, with mild-cognitive
4 months later, without adverse events or new episodes
reported an increased incidence of ADEM after SARS- impairment and normal thyroid function tests. She is cur-
of inflammatory outbreaks at last 6 month. Discussion:
CoV-2 epidemics. In the typical presentation of ADEM, rently in physical rehabilitation, with regular follow ups.
CLIPPERS has become a crucial differential diagnosis of
neurological symptoms develop 7–14 days following an Discussion: Previouslly named Hashimoto’s encephalop-
CSN immune-mediated diseases. Its pathophysiology,
infection and may involve headache, emesis, behavior athy, Steroid Responsive Encefalopathy associated with
characterized by autoreactive lymphocytes-mediated
alterations and coma. The case presented showed dysme- autoimune thyreoiditis is a rare disease, associated with
perivascular inflammation, remains unclear. In Brazil,
tria, cerebellar gait, nauseas and facial palsy. ADEM CSF antithyroid antibodies. Women tend to be more affected
only few cases of female patients with this syndrome
examination reveals inflammatory findings, consisting than men and the mean age of onset is 51 years. It may
has been reported since it classically affects men, with a
of elevated protein levels and lymphocytic pleocytosis. comprise neuropsychiatric signs, such as progressive
wide range of age distribution. This clinical case is rele-
Isolate increased protein level was present in up 40% of cognitive impairment, disturbance of consciousness,
vant to brazilian neurological may the use CST, so far, has
ADEM linked to SARS-Cov-2 in some case series. A few seizures, psychosis, behavioral changes, hallucinations,
been scarely described. Final Comments: Diagnosis of
atypical presentations can be seen in some cases, and the myoclonus, involuntary movements, language barrier
CLIPPERS was established using clinical and complemen-
differential diagnosis with multiple sclerosis (MS) remains and ataxia. This wide range of clinical findings leads to
tary criteria, described by Pittock et al, and treatment is
challenging due to the overlap in the clinical presenta- a underrecognition of the syndrome. The pathophysi-
successfully corelated by symptoms remission. This case
tion. In ADEM, the lesions can be seen in the brainstem ology, as well as the relationship between Hashimoto
might contributes to the knowledge that CLIPPERS is a
and cerebellum, resulting in symptoms that are like those thyroiditis remains unclear. SREAT has a pronounced
differential diagnosis of immune-mediated CNS diseases,
found in the present case. Final Comments: The varied response to corticotherapy, the first-line treatment. Final
understanding follow-up and therapy used with positive
clinical manifestations caused by SARS-Cov-2 infection Comments: The relevance of this case presentation lies
results. Cases estimeteds in the world is uncertain and a
in the nervus system have been constantly updated. In on the fact that SREAT is a rare and severe, but treatable
literature review founded about 40 cases, predominant in
this context, coronavirus infection can be seen as a trig- syndrome. In order to avoid and ease complications that
older men, where reports on female patients is uncommon.
ger to ADEM. could jeopardize the patient’s recovery, the early diagnosis
and treatment are decisive.
Neuroimunologia Neuroimunologia
Neuroimunologia

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XXX Congresso Brasileiro de Neurologia

TL 1105169 TL 1105183 TL 1105216

EXTENSIVE SPINAL WALLERIAN SEVERE GUILLAIN-BARRÉ SYNDROME HASHIMOTO’S ENCEPHALOPATHY: CASE


DEGENERATION IN A PARANEOPLASTIC DURING RECENT DENGUE OUTBREAK IN PRESENTATION AND LITERATURE REVIEW
GANGLIONOPATHY CASE THE SOUTH OF BRAZIL OF A RARE ENTITY
Thamilee Pizzatto, Mariana Spitz, Roberto Queiroz Giuseppe Dick Bonato, Erica Garbin Rizon, Leticia Anna Laura Lima Larcipretti, Fernando Cotrim
dos Santos, João Vitor Ribeiro dos Santos, Abraão Bassani Devens, Marco Antonio Koff, Douglas Gomes, Ana Carolina Santiago Dias Barbosa,
Iuri Medeiros Angelim, Marcelo Cagy, Ana Carolina Kazutoshi Sato Matheus Resende Costa Pimenta, Ana Luisa
Andorinho de Freitas Ferreira Carvalho Santiago, Jane Lacerda Bahia, Rafael
Hospital São Lucas da Pontifícia Universidade Pallos da Silveira, Dimitria Fortes de Oliveira Borges,
Hospital Federal dos Servidores do Estado do Rio de Católica do Rio Grande do Sul – Porto Alegre – RS Kélia Rejane Santiago Dias
Janeiro. Rio de Janeiro RJ, Brazil – Brazil
Hospital Federal dos Servidores do Estado do Rio School of Medicine, Federal University of Ouro Preto
de Janeiro, Hospital Pedro Ernesto/UERJ. Rio de g.d.bonato@hotmail.com – Ouro Preto – MG – Brazil
Janeiro RJ, Brazil Faculty of Medicine, Federal University of Minas
Hospital Federal dos Servidores do Estado do Rio Case Presentation: Fourteen days before admission, a Gerais. Belo Horizonte MG, Brazil
de Janeiro, Americas Medical City. Rio de Janeiro 62-year-old woman was diagnosed with dengue virus Faculty of Medical Sciences of Minas Gerais. Belo
RJ, Brazil infection. She had typical clinical features – high fever, Horizonte MG, Brazil
Hospital Federal dos Servidores do Estado do Rio retro-orbital pain, headache, muscle pain and cutaneous Department of Neurology, Odilon Behrens Hospital.
de Janeiro, Hospital São Lucas-RJ. Rio de Janeiro rash – and positivity to NS1 antigen. She recovered well Belo Horizonte MG, Brazil
RJ, Brazil and remained asymptomatic for 5 days. However, 2 days Department of Neurosurgery, João XXIII Hospital.
before Hospital admission, the patient presented with Belo Horizonte MG, Brazil
anandorinho@hotmail.com progressive paraparesis and lower limbs paresthesia.
At the first neurological exam at the emergency room, anna.larcipretti@aluno.ufop.edu.br
Case Presentation: A 52-year-old woman presented she had distal lower limb weakness (MRC = 4) and walk
a three-month history of weight loss and incoordina- impairment. In less than 24 hours, she rapidly evolved to Case Presentation: A 62-year-old man presenting altered
tion that turned her bedridden. She was a longstanding global paresis (MRC = 1-2) associated with diminished mental status and a history of four seizure episodes in the
smoker without comorbidities. Neurological evaluation upper limbs reflexes, lower limbs areflexia, dysphagia past 2 months, along with aggravated neuropsychological
unveiled severe appendicular and axial sensitive ataxia, and urinary retention. After ICU admission, she required symptoms, such as anxiety, delirium, and depression.
uncomfortable paresthesia, despite preserved strength. orotracheal intubation and mechanical ventilation due Previous history of steroid-responsive encephalopathy,
Previous electroneuromyography endorsed the hypoth- to ventilation distress. Sudden changes in heart rate suspected Hashimoto’s encephalopathy (HE). He was
esis of ganglionopathy. Etiologic investigation disclosed and blood pressure suggested autonomic dysfunction. treated with prednisone 3 months prior current admission,
a small cell lung cancer (SCLC) and the final diagnosis Cerebrospinal fluid analysis revealed protein elevation with a gradual withdrawal. Magnetic resonance imaging
was a paraneoplastic syndrome. Intravenous pulse steroid (111 mg/dl) and normal cell counts. Due to the probable (MRI) report was unremarkable, serology tests positive
therapy was prescribed without symptomatic change, and Guillain-Barre Syndrome (GBS) diagnosis, the patient for Anti-TPO antibodies, EEG not available. HE suspicion
the patient was referred to Oncology. After 2 months of underwent 7 plasmapheresis sessions on alternate days. remained. Pulse corticosteroid therapy was administered,
chemotherapy, surveillance exams revealed lung response During ICU period, she had other complications such as previous treatment reinstated. Discussion: HE is a rare
though the appearance of dorsal spine hyperintensity, neuropathic pain, ventilation associated pneumonia and and controversial disease encompassing a heterogeneous
not seen in the first MRI. Neurological examination was urinary tract infection. She received intense physical and neurological and psychological presentation. Diagnosis of
unchanged. Patient was submitted to radiotherapy and a ventilatory rehabilitation. The first sign of clinical improve- HE is a clinical challenge since there isn’t a well-defined
3rd MRI still showed the hyperintensity. Nine months after ment was the resolution of dysautonomic symptoms. On criteria and the pathophysiological mechanisms are still
the treatment beginning, the oncologic disease remains the 12th day, she started to recover spontaneous move- unclear. Literature review presents neither a gold-stan-
under control without neurological gain. Discussion: ments from axial muscles. Tracheostomy was removed dard test for diagnosis nor a reference value for serology
Paraneoplastic ganglionopathy is most associated to on day 24. After 46 days, she was discharged with partial exams, although elevated anti-TPO seems to be the hall-
SCLC indeed. The main antibodies involved are: anti-Hu, recovery, being able to stand and walk with assistance, mark of HE. Other case presentation presented highly
anti-CRMP5 and anti-amphiphysin. MRI dorsal column and partial diuresis control. She was referred to outpatient heterogeneous patient profiles with long-ranging age
hyperintensities have been reported in ganglionopathy, a rehabilitation and neurological follow-up. Discussion: groups, mostly female, and diverse clinical presentations,
differential diagnosis for tabes dorsalis, vitamin B12 and Dengue virus infection was very uncommon in the Rio varying from acute to insidious onset. Studies showed
copper deficiency and nitrous oxide toxicity. In gangli- Grande do Sul state, but recent outbreaks and spread of the relapse and remitting course in 50% of the patients.
onopathy, it has been reported a precocious emergence Aedes aegypti vector changed the disease epidemiology. Most common treatment is still corticosteroid adminis-
of this spine hypersignal, besides a radiological improve- Fast evolving neurological manifestations of dengue are tration, but intravenous immunoglobulin, methotrexate,
ment after immunomodulatory therapy. However, in rare and may be overlooked by general practitioners. case mycophenolate, and azathioprine have been used with
most cases, MRI finding represents irreversible damage presentation of GBS after dengue virus infection are scarce. variable efficacies. Final Comments: Thus, it is important
due to gliotic injury. This latter scenario reinforces the Management of severe GBS requires quick diagnosis with to raise awareness of HE since it’s a neglected differential
physiology basis of Wallerian degeneration (WD) started rapid treatment, ICU support and intensive rehabilitation diagnosis for various neuropsychological symptoms and
from dorsal root ganglia (DRG), pseudobipolar neurons to reduce disease related morbidity and mortality. Final early intervention can define prognosis. Furthermore,
that ramify into the peripheral branch and the central Comments: Dengue virus infection should be considered the relapse after corticosteroids treatment emphasizes
branch which carries the somatosensory information to in the diagnostic workup of triggers for GBS, including in the need of therapeutic alternatives, which demands a
the spinal cord. Although the underlying cause has been regions with previous low prevalence. Plasmapheresis is better understanding of its pathophysiology.
addressed, the lesion of the DRG, namely the proximal cell an alternative treatment when human immunoglobulin
body, triggered a cascade to distal axonal degeneration. is not available.
Therefore, first MRI was unremarkable but the following Neuroimunologia
exams testified a permanent finding. Final Comments:
Our case highlights WD in central afferent tracts, sec- Neuroimunologia
ondary to disrupted synapse at the dorsal-horn. The
persistence of the novel hyperintensity and the absent
recovery from sensitive ataxia supports MRI as a tool
for chronicity ascertainment. Moreover, MRI was able
to illustrate the pathogenesis behind the natural history
of ganglionopathy.

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MYELITIS AS THE FIRST OVERLAP OF MILLER FISHER SYNDROME PAROXYSMAL DYSTONIA AS ATYPICAL
NEUROPSYCHIATRIC MANIFESTATION IN (MFS) WITH BICKERSTAFF SYNDROME (BS) INITIAL MANIFESTATION OF MULTIPLE
A SYSTEMIC LUPUS ERYTHEMATOSUS SCLEROSIS
Indianara Keila Pastorio, Lucas Oliveira Pinto
PATIENT – RARE CASE PRESENTATION Bertholdi, Lorena Dias Araújo, Raquel Mattos Paulo Filho Soares Marcelino, Amanda Loureiro
João Pedro Izidoro Gomes, Emanuelle Bianchi da Filgueiras, Sayuri Aparecida Hirayama, Francine de Toledo Troian, Andreas Batista Schelp, João
Silva Rocha, Janaina Moraes de Araújo, Rodrigo Paula Roberto Domingos, Rafael Almeida, Paulo Paulo Barile, Vanessa de Freitas Moreira, Isabela
Fellipe Rodrigues, Laura Alonso Matheus Montouro, Henrique Martinelli Oliveira, Raderi Luiz Cardoso de Almeida Stella, Sephora Sabrina Candido de
Alex Bertolazzo Quiterio, Gabriel Pina Paiva, Ricardo Santos, Lucas Silva Dias Almeida, Ana Karoline Basler, Davi Vargas Freitas
Funes Bastos, Andressa Regina de Mello Galego, Teixeira, Herval Ribeiro Soares Neto
Hospital Municipal José de Carvalho Florence – São
Pamela Regina Henning Jose dos campos – SP – Brazil IAMSPE, São Paulo SP, Brazil
Faculdade de Medicina de São José do Rio Preto – Hospital das Clínicas da Faculdade de Medicina de
Ribeirão Preto. Ribeirão Preto SP, Brazil paulomed7@gmail.com
FAMERP – Sao Jose do Rio Preto – SP – Brazil
Faculdade de Medicina de São José do Rio Preto – indi_kp@hotmail.com Case Presentation: A 14-year-old male with autism
FAMERP – São José do Rio Preto – SP – Brazil
spectrum disorder started with gait disturbance and
João _pedro300@hotmail.com Case Presentation: A 15-year-old male patient presented vertigo, evolving with tingling in his hands and feet, in
with subacute weakness of the lower limbs associated addition to several daily episodes of sustained and pain-
Case Presentation: M.C.J.S., 30 years old, female, diag- with incoordination within 1 week, with a 48-hour pro- ful flexion of the left upper limb, lasting 1-2 minutes. On
nosed with Systemic Lupus Erythematosus (SLE) at the gression to impaired gait, axial instability and diplopia. examination, he had dystonic posture in the left hand
age of 20, presenting, over 10 years, cutaneous, joint, He denied fever and diarrhea but reported having a flu- and forearm, hyperactive patellar reflex, and bilateral
hematological and renal activity. During this period, she like syndrome three weeks before the current condition. clonus. No changes in cranial nerves, strength, sensi-
received treatment with hydroxychloroquine and pred- On examination: hypotonia and exalted osteotendinous tivity and coordination. Therapy with carbamazepine
nisone. In June 2021, she started a prodromal condition reflexes with bilateral Babinski, and bilateral ophthal- showed progressive improvement of dystonic spasms.
flu-like associated with paresthesias, distal weakness in moparesis was noted. On the day after admission, the Investigation demonstrated CSF with oligoclonal type 2
the lower limbs and urinary incontinence. After 48 hours, patient developed ebrious dysarthria, dysphagia, inef- bands and cranial resonance with T2/FLAIR hypersignal
she evolved with worsening of the legs weakness, being fective cough and decreased level of consciousness, in the juxtacortical, periventricular, calloseptal and bul-
unable to stand and walk without assistance, and in less and orotracheal intubation was chosen. After 1 week of bar paramedian interface and gadolinium enhancement
than 24 hours progressed to flaccid tetraparesis. Analysis of sedation withdrawal, he presented some improvement in in the corpus callosum. He was diagnosed with Multiple
the cerebrospinal fluid showed pleocytosis with lympho- the sensorium, proximal grade 0 and distal limbs grade Sclerosis (MS) and treated with steroid pulse therapy fol-
cytic predominance, hyperproteinorraquia and normal IV strength, sustained bilateral ophthalmoparesis, facial lowed by plasma exchange. At discharge, the patient was
levels of glucose and lactate faced with the suspicion of diparesis, absent cough reflex, associated with dysautono- asymptomatic, with complete improvement of dystonia.
autoimmune inflammatory myelopathy. In addition, we mia with pressure variability and of heart rate.Tomography Discussion: Paroxysmal symptoms in MS are a heteroge-
noticed a high value of anti-double-stranded DNA anti- (CT) and Magnetic Resonance of the skull performed: neous group of motor, sensory, cerebellar, or brainstem
body 400 U/ml, indicating high disease activity. Magnetic no alterations; cerebrospinal fluid (CSF) with protein of changes, characterized by repetitive, stereotyped and
resonance evidenced hypersignal lesions, with a diffuse 23mg/dL, glucose: 118mg/dL; leukocytes: 3, Oligoclonal short episodes that can last from days to months. The
and irregular distribution, longitudinally from the medulla bands absent. Electroencephalogram without changes. incidence is 1.6 to 17% in MS cases, of which 24% are the
oblongata transition to the level of the conus medullaris, Laboratory tests/cultures/serologies without alterations. initial manifestations. The most important are dyskinesia,
with a slight swelling effect and without contrast enhance- Chest CT: normal. Based on the above, the suspicion myokymia, lhermitte sign, trigeminal neuralgia, vertigo,
ment. She was treated with high-dose intravenous meth- of SMF superimposed with SB was raised. He received diplopia and ataxia. Paroxysmal dystonia is an involun-
ylprednisolone with little initial improvement, showing a treatment with plasmapheresis in 4 sessions on alternate tary, abrupt and also stereotyped movement. It may be
better therapeutic response after plasmapheresis. During days, being interrupted after motor worsening in the left painful or not, kinesiogenic or not, focal or generalized.
follow-up, 6 months later, she was able to walk unassisted, side and evidence of intraparenchymal hematoma with Although not completely understood, it is speculated that
maintained temporary treatment with cyclophosphamide, hemoventricle and midline deviation on a new cranial CT, this phenomenon may occur due to erratic transmission
but due to the potential nephrotoxic damage, she started evolving with complications and death. Discussion: MFS of ectopic nerve impulses by partially demyelinated axons.
using rituximab with excellent results. As additional data, is a variant of Guillain-Barre syndrome characterized by a These may present a dysfunction in ion channels, which
anti-aquaporin-4 antibody (AQP4) was measured with a subacute, monophasic onset and the classic triad of oph- generates neural desensitization to stress factors. This is
negative result. Discussion: the occurrence of myelitis thalmoplegia, ataxia and areflexia. If there is progressive also strengthened by its good therapeutic response to
in SLE is estimated at 1.2%. In cases where signs of gray impairment of the level of consciousness and/or associ- anticonvulsant drugs. Furthermore, its location occurs
matter predominate, high disease activity was observed ated hyperreflexia, we recognize brainstem encephalitis, due to lesions in the motor pathway, such as in the poste-
by high levels of anti-double-stranded DNA antibodies, or BS. Sharing similarities in the autoimmune mecha- rior horn of the internal capsule, basal ganglia, brainstem
while when white matter disorders predominate there nism, both syndromes are associated with anti-GQ1b and cervical spinal cord. We report a case of a patient
is an association with anti-AQP4 positivity, indicating, antibodies, which are highly specific for diagnosis. One who presented with a first onset of MS associated with
in fact, a great possibility of overlap with neuromyelitis of the main limitations of this case was the unavailability dystonic spasm, with dramatic improvement of dystonia
optica spectrum disorder (NMOSD). Final Comments: of dosing this antibody. Both intravenous immunoglobu- after the introduction of carbamazepine. Final Comments:
although uncommon, promptly diagnosing and treating lin and plasmapheresis have been used in the treatment. Paroxysmal symptoms are uncommon in MS, posing a
SLE myelitis can prevent severe neurological damage. Also, Final Comments: The overlap of SMF with SB is rare. It is diagnostic challenge. Recognition as a possible manifes-
it is important to pay attention to the differential diagnosis important to remember the need for attention in clinical tation of MS is of fundamental importance, as it can avoid
with other autoimmune demylinating diseases such as practice for possible diagnose because the prognosis is delay in diagnosis and provide adequate early treatment.
NMOSD, which can coexist with SLE and be responsible generally good and most patients recover well when the
for the involvement of the spinal cord and not the activity syndrome is quickly recognized and treated.
of the underlying systemic disease. Neuroimunologia

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VOGT-KOYANAGI-HARADA SYNDROME CASE PRESENTATION POST-COVID19 ACUTE TRANSVERSE


AFTER SARS-COV 2 VACCINE: CASE ENCEPHALOMYELORRADICULITIS OF MYELITIS TREATED WITH PLASMA
PRESENTATION UNKNOWN ETIOLOGY IN A YOUNG PATIENT EXCHANGE AND RITUXIMAB: A CASE
PRESENTATION
Beatriz Pereira Rios, Luiz Filipe Alves Matos, Felipe Bárbara Oliveira Paixão, Marina Buldrini Filogonio
Rodrigues Marques Ferreira, Caroline Correa Seraidarian, Paolla Giovanna Rossito de Magalhães, Davi Vargas Freitas Teixeira, Andreas Batista Schelp,
Santos, Camilla Souza Fonseca, Paula Queiroz Gabriella Braga da Cunha Silva, Maira Cardoso Déborah Inayara Mendes Tenório de Albuquerque,
Alves, Pedro Antonio Pereira de Jesus Aspahan, Ana Luiza Figueiredo Campos, Arthur Vanessa de Freitas Moreira, Alexia Carneiro de
Braga Pereira, Daiane Magalhães Almeida, Amanda Loureiro de Toledo Troian, Luciana
Hospital GERAL ROBERTO Santos. Salvador BA,
Zelante Ambiel Magalhães, Beatriz Marques dos
Brazil Hospital Madre Teresa. Belo Horizonte MG, Brazil
Santos, Érico Induzzi Borges, Herval Ribeiro Soares
bprios3@gmail.com barbara_paixao@hotmail.com Neto

Hospital do Servidor Público Estadual de São Paulo.


Case Presentation: A 29-year-old female patient, who Case Presentation: 49 year old male patient with con-
São Paulo SP, Brazil
was vaccinated against SARS-COV-2 (Pfizer), sought a trolled hypertension was Hospitalized with a first-time
neurological emergency service 24 days after vaccination seizure. Three months before Hospitalization he pre- davivft@hotmail.com
due to a severe, progressive headache, mainly in the right sented a persistent low back pain which progressed to
temporal region, with subsequent bilateral involvement an intermittent left hemiparesis. Those symptoms were Case Presentation: 51-year-old woman with severe obe-
associated with phonophobia, photophobia, nausea and previously assigned to a C4-C5 disc herniation. Magnetic sity and no other medical history, admitted with dyspnea
dizziness. She also reported visual scotomas, tearing of resonance imaging showed an irregular lesion in the and anosmia for 5 days. SARS-COV-2 pneumonia was
the eyes and intense bilateral conjunctival hyperemia, right temporal lobe causing mass effect in addition to confirmed by RT-PCR. The patient developed respira-
with the development of blurred vision and diplopia. an extensive cervicothoracic myelopathy with contrast tory distress within 24 hours and required mechanical
She was Hospitalized and in the initial neurological eval- enhancement and a diffuse leptomeningitis affecting ventilation for 4 days, without hemodynamic instability.
uation showed a reduction in bilateral visual acuity, not even cauda equina roots. The cerebrospinal fluid (CSF) After pulmonary recovery the patient noticed difficulty
accompanied by changes in extrinsic ocular muscles or sample had 50 cells/microL, with a lymphocytic predom- in standing, received discharge in a wheelchair. At home,
direct pupillary changes. Other focal deficits were also not inance, 663 mg/dL proteins and glucose consumption. progressively worsens motor function, not being able to
detected. She underwent imaging studies of the skull and Oncocytic cells and the antineuronal antibody panel were support her trunk associated with bladder dysfunction
initial orbits, which showed no changes. She also under- negative. Adenosine deaminase (ADA) at CSF was 1 U/L, and lower limb paresthesia. Readmitted 15 days after, the
went lumbar puncture, which showed lymphomonocytic not suggestive of tuberculous meningitis. Oligoclonal first neurological examination described severe muscle
pleocytosis, with manometry within the normal range. An bands were present in both CSF and serum suggestive weakness of lower limbs, feet drop and mild involvement
ophthalmologic evaluation was performed, which showed of a systemic inflammatory process. The CSF immuno- of intrinsic hands muscles; global deep tendon absence
bilateral conjunctival hyperemia with ciliary injection and histochemistry and rheumatologic/infectious workup and babinski sign bilaterally; large and small fiber anes-
posterior synechiae of almost 360 degrees. The funcoscopy were negative. Angiotensin-converting enzyme (ACE), thesia below T12 level. Spine MRI concluded transverse
examination revealed cloudy media, pink papilla with clear anti-myelin oligodendrocyte glycoprotein (MOG) and myelitis from C7 to T12; CSF’s routine was normal and
edges, sensorineural retinal detachment in the macular anti-aquaporin-4 (AQP4) were non-reactive. Chest and RT-PCR for SARS-COV-2 and Herpesviruses went neg-
region, presence of nodules in the middle periphery. papil- abdomen tomographies showed no neoplasms. Biopsy of ative. Investigation for neuroimmune and rheumato-
litis, with Vogt-Koyanagi-Harada syndrome as the main the temporal lesion resulted in nonspecific meningoen- logic markers, serological status, onconeural antibodies,
suspect. Discussion: Vogt-Koyanagi-Harada syndrome cephalitis, with no signs of malignancy. The CSF metag- hypovitaminosis and metabolic causes were negative.
(VKH) is an inflammatory and autoimmune condition enomic sample is still without a definitive result. Patient Methylprednisolone 1g for seven days was administered
characterized by panuveitis, serous retinal detachment was referred for a external ventricular drain implanta- with poor motor outcome. Afterwards, seven plasma
and extraocular manifestations (auditory, integumentary tion for intracranial pressure monitoring. In addition, exchange sessions followed by Rituximab 2g total dose
and central nervous system). Its autoimmunity mecha- empirical plasma exchange was started. However, it was led to substantial improvement. At deinstitutionalization
nism may be associated with viral infections as well as not possible to observe the patient’s response since he the patient regained bladder and trunk control with poor
vaccines. In the current scenario of mass vaccination progressed with a septic shock due a Staphylococcus lower limb mobility. At three-month follow-up the patient
due to the SARS-CoV2 pandemic, this association has haemolyticus bloodstream infection. By the sixth day could sustain her legs; at sixth month she could walk with
also been evidenced, but with mechanisms still poorly after admission brain death was certified. Discussion: crutches. Nine months after discharge she could walk
described. The diagnosis of VKH is essentially clinical This case presented itself as a challenging clinical case without assistance. Discussion: Acute Transverse Myelitis
and can be confirmed with imaging tests. Treatment in a previously functional young patient with multiple (ATM) is a rare and acquired neuro-immune disorder char-
consists of the use of immunosuppressants, preferably active central nervous system lesions. Considering the acterized by perivascular lymphomononuclear infiltration
started early to avoid the emergence of complications, main hypotesis, inflammatory disease and neoplasm, of spinal cord affecting white and gray matter, explaining
mainly neuro-ophthalmological. Final Comments: In several procedures were performed, especially MOG, sensitive, motor and autonomic dysfunctions. Besides
the context of a pandemic and in the course of a global anti-neuronal antibody panel, CSF immunohistochem- it’s difficult to assuredly exclude differential diagnosis,
mass vaccination campaign, it is important to recognize istry and metagenomic sample. Some of these exams are post-viral infection immune-mediated TM is a main etiol-
possible complications associated with vaccination against still not easily available in medical practice and may be ogy, classically associated with the human Herpesviruses.
COVID-19. However, to date, these risks are low and the extremelly important for some etiological diagnosis. Final Nethertheless, as described in other reports, immune-me-
benefits of vaccination outweigh any side effects. Comments: Neurological cases without etiological defi- diated TM is an emerging complication of SARS-COV-2
nition occur occasionally in medical practice. Therefore, infection, which by analogy, has been treated how other
it is important for neurologists to share experiences for viruses justifying an immunosuppressive therapy. Final
Neuroimunologia the establishment of new approaches in daily practice. Comments: Albeit evidence is needed to guide decision
through post-COVID19 TM, this case corroborates for an
combined aggressive immune-therapy after a suitable
Neuroimunologia investigation for severe presentations with high rate of
disability and complications.

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THE IMPORTANCE OF DYNAMIC MAGNETIC GUILLAIN BARRÉ SEVERE SYNDROME (GBS) ANTI-LEUCINE-RICH GLIOMA-INACTIVATED
RESONANCE IMAGING IN THE DIAGNOSIS PROBABLY ASSOCIATED WITH VACCINATION 1 ENCEPHALITIS: A CASE SERIE
OF CERVICAL MYELOPATHIES: A CASE FOR COVID-19
Anna Letícia de Moraes Alves, Alison Mangolin,
PRESENTATION Victor Augusto Zanesi Maciel, Vanessa Lauanna
Indianara Keila Pastorio, Lucas Oliveira Pinto
Lucas Bruno Rezende, Beatriz Gioppo Betini, Bertholdi, Lorena Dias Araujo, Sayuri Aparecida Lima Silva, Natália de Oliveira Silva, Roberto Satler
Guilherme Robles Chaparim, Patrícia Approbato Hirayama, Francine Paula Roberto Domingos, Rafael Cetlin, Fabíola Dach
Marques, Lucas Ravagnani da Silva, Eduardo Yukio Almeida, Paulo Henrique Martinelli Oliveira, Lucas
Hospital das Clínica da Faculdade de Medicina
Nakamura, Jorge Alberto Martins Pentiado Junior Silva Dias, Raderi Luiz Cardoso Santos, Raquel
de Ribeirão Preto – Universidade de São Paulo.
Mattos Filgueiras
Hospital das Clínicas da Universidade Federal de Ribeirão Preto SP, Brazil
Minas Gerais. Belo Horizonte MG, Brazil Hospital Municipal José de Carvalho Florence – São
moraes.annaleticia@gmail.com
Hospital das Clínicas da Faculdade de Medicina Jose dos Campos – SP – Brazil
de Ribeirão Preto da Universidade de São Paulo. Hospital das Clínicas da Faculdade de Medicina de
Case Presentation: Three eldery patients, mean age of
Ribeirão Preto SP, Brazil Ribeirão Preto – Ribeirao Preto – SP – Brazil
67 years old, two women and one man, presented to our
Hospital São Lucas. Ribeirão Preto SP, Brazil Hospital clinic due to subacute faciobrachial dystonic sei-
indi_kp@hotmail.com
lucasbrunorezende@hotmail.com zures (FBDS) as main symptoms. FBDS were bilateral and
Case Presentation: Female patient, 60 years old, hyper- high frequency (mean sixty episodes per day). One patient
Case Presentation: A 62 year old man was admitted to our tensive, presented insidious lower limb (LL) weakness also had dystonic seizures in legs. Mild chorea was noticed
service due to weakness in lower limbs and unsteadiness. associated with tactile-painful hypoesthesia. In five days, in one woman concurrently. Executive dysfunction and
Symptoms began 3 months before, with pain and numb- there was a progression of weakness with impairment of short-term memory loss were noted in all of them, leading
ness in his hands, evolving to bilateral and symmetrical walking, which motivated the visit to the Hospital. She to rapidly progressive dementia phenotype in two. The
weakness in lower limbs. The weakness became worse denied fever, diarrhea, or respiratory infection symptoms man also showed visual hallucinations, behavior changes,
4 days before admission, after receiving the 2nd dose of preceding her current condition, but a vaccine refer- depressed humor and hyponatremia (120 mmol/L),
Covid-19 vaccine. There was no history of trauma, consti- ence for COVID-19 (Astrazeneca) fifteen days earlier. On which prohibited the initial management of FBDS with
tutional nor sphincters complaints. On neurologic exam- examination: hypotonia in the LL, reflex absence glob- carbamazepine (CBZ). Electroencephalogram showed
ination, he showed a global MRC strength grade 5, except ally, grade IV strength in the upper limbs (UL), grade III disorganized background activity with bifrontal slowing
for 4+ on hip flexion and 4+ on hip abduction. There were proximal, grade II distal in the LL, and peripheral facial in both women. The Magnetic resonance images evinced
signs of bilateral pyramidal release in lower limbs. He had diparesis. Performed cranial CT with no alterations and white matter hyperintensities presumed of vascular origin
a reduced proprioception and doubtful touch and pain cerebrospinal fluid (CSF) with protein of 141mg / dL, glu- in totality, besides hippocampal volume reduction in the
hypoesthesia at T12 and T4 level, respectively, and he cose: 80mg / dL; WBC: 0. Screening for nasopharyngeal woman with worse cognitive status. Search to occult neo-
was unable to stand without assistance. He had sensory SARS-CoV-2 using rapid SWAB test, negative. Based on plasm was negative to all. There were no cerebrospinal
ataxia in all limbs, worse in his legs. Results of laboratory the above, the suspicion of SGB was raised. Intravenous fluid abnormalities besides anti-LGI1 antibody positive,
exams and CSF were unremarkable. The cervical spine immunoglobulin infusion (IVIG) was started, followed excepting the last patient whose testing is still in progress.
MRI showed an nonspecific T2 signal heterogeneity at by clinical worsening of the degree of strength (LL and Metilprednisolone was the first line therapy to all, added
C4-C5. Based on the findings, the hypothesis of a post UL grade 0) and presence of dysautonomia (sustained to plasmapheresis (PLEX) in two. CBZ and levetiracetam
vaccinal inflammatory myelopathy was made and the tachycardia and arterial hypertension). After the end of associated with immunotherapy were essential to achieve
patient went under pulse therapy with 1g methylpred- IVIG, the patient established dysphagia, dysphonia, and FBDS full control in all patients in the mean period of 1
nisolone for 5 days, without objective improvement. dyspnea. Due to the clinical deterioration, we opted for month. Micofenolato and prednisone were the choices for
Because of the atypical presentation of an inflammatory a new IVIG cycle, with an additional 1g / Kg being a total long term immunosuppression. Cognitive functions were
mielitis and for the differential diagnosis of compressive dose, divided into three days. There was an improvement improved in all cases. Discussion: Anti-LGI1 encephalitis
spondylotic myelopathy (CSM), a dynamic cervical spine in swallowing, dyspnea, upper limb strength (proximal mainly affects eldery (median age 60 years) who present
MRI was requested. The exam revealed a canal stenosis at grade II, distal grade III), and reversal of dysautono- with seizures, cognitive and behavioral disorders. FBDS
C4-C5. The patient underwent laminectomy, spinal cord mia. Discussion: GBS is an acute inflammatory disease are very specific for the disease otherwise presenting in
decompression and posterolateral cervical arthrodesis of the peripheral nerves that commonly presents with a minority of cases. Usually there is no EEG correlation
from C3 to C6. The procedure was uneventful and he progressive symmetrical weakness and areflexia, result- with FBDS. Moreover, about 60-65% of patients develop
evolved with progressive improvement of symptoms. ing in respiratory failure. Annual report of 1 to 2 cases / hyponatremia, which is a clue to the diagnosis. Treatment
Discussion: Cervical spondylotic myelopathy is the most 100,000 inhabitants and peak between 20-40 years, being consists in immunotherapy with corticosteroids, IV immu-
common spinal cord disorder in the elderly. Diagnosis is somewhat more common in men. It can result from an noglobulins or plasmapheresis. Final Comments: This
based on clinical signs and symptoms associated with a immune response that occurs 3-6 weeks after a previous case series adds knowledge of brazilian and local epide-
radiological exam. The spinal cord is made under dynamic acute illness, infection, or trigger in 50% of cases. Still, it miology of this rare cause of rapidly progressive demen-
changes during flexion and extension. These changes can also be associated with immunization, surgery, and tia, especially since patients presented with the classical
lead to biomechanical stress with movement, which trauma, for example. A relationship between COVID-19 clinical features of the disease had a great response to
may aggravate damage in patients with critical cervical infection and many neuropathies, including GBS, has treatment. This reinforces the importance to recognize
stenosis. Normal MRI is unable to detect those changes. been reported. However, we still do not have full knowl- the clinical syndrome allowing for promptly request
Therefore, the dynamic MRI has a higher sensibility for edge of GBS related to immunization against COVID-19. antibody testing and initiate the immunotherapy even
the detection of CSM. Final Comments: This case shows Final Comments: It is essential to remember the need before antibody results are available.
a patient with a typical presentation of subacute cervical to pay attention in clinical practice to GBS causes in a
of CSM, but no significant changes in MRI. The diagnosis scenario of such a recent and devastating infection as
COVID-19 and its newly discovered vaccines. Extensive Neuroimunologia
was only confirmed after a dynamic cervical spine MRI.
Hence, dynamic MRI is an additional image resource studies are needed.
able to provide useful information when there is a high
clinical suspicion for CSM and the standard radiological
exam is inconclusive. Neuroimunologia

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MOG ASSOCIATED DISEASE FOLLOWING DIAGNÓSTICO DE NEUROMIELITE ÓPTICA, MULTIPLE SCLEROSIS AFTER ANTI-NMDAR
HERPES 7 INFECTION: A CASE EM PACIENTE COM MIASTENIA GRAVIS ENCEPHALITIS: CASE PRESENTATION AND
PRESENTATION REVIEW OF THE LITERATURE
Ricardo Eberhart Ribeiro da Silva, Elder Yanaze
Thainá Gonçalves Tolentino de Figueiredo, Laís Oda, Lucas Casimiro de Oliveira, Amanda Loureiro Alexandre Coelho Marques, Pedro Victor de Castro
Maria Gomes de Brito Ventura, Murilo Santos de de Toledo Troian, Thaís Laura Almada da Fonseca, Silva, Mariana Driemeier Cardoso, Bruna de Freitas
Souza, Ana Clara Guilherme Rodrigues, Sheila Pedro Lucas Kuibida Belleze, Janaina Alves Leite Dias, João Henrique Fregadolli Ferreira, Fábio Fieni
Ferreira Pessoa, Anselmo de Jesus Santos Matos Toso, Lívia Almeida Dutra

Hospital São Rafael. Salvador BA, Brazil HRMS. Campo Grande MS, Brazil Hospital Albert Einstein, São Paulo SP, Brazil

thainatolentino@hotmail.com ricardo.cmcg@hotmail.com acmarques.neuro@yahoo.com.br

Case Presentation: A 34-year-old woman, on the 4th Case Presentation: G.C.D.S, started at 29 years of age, Case Presentation: A 19-years-old woman presented
postoperative day of video surgery due to ruptured ecto- floating weakness in arms and legs, prevailing in the prox- with seizures in June/2019. Brain MRI and EEG were
pic pregnancy, was Hospitalized with a CSF suggestive of imal muscles, associated with intermitent diplopia and unmarkable and she received levetiracetam. After 30 days
viral meningitis with a positive HHV-7 PCR and acyclovir ptosis. Reported worsening of symptoms with stress and she developed depression, aphasia, anosognosia, apathy,
was started. Evolved with a somnolence, associated with warm weather. Electroneuromyography with repetitive dyscognitive seizures, sleep disorder and recurrent vom-
ampicillin for 21 days and a brain MRI was performed nervous stimulation at 3 hz showed a 25.4% decrease in iting. Physical examination disclosed temporal disorien-
with a finding of diffuse hyperintensity on T2/flair in the amplitude; she was then diagnosed with myasthenia gravis tation, mild ataxic gait, tremor and incoordination in left
brainstem without contrast enhancement, associated with (MG), and started treatment with pyridostigmine, azathi- limbs. New EEG showed epileptiform discharges in right
lesions in the hippocampus and in the internal and poste- oprine and prednisone and thymectomy was performed. temporal lobe. CSF showed cells 11 cells/mm3, protein 23
rior capsule/right lentiform nucleus, coexisting with dif- Evolved with good response to treatment with control of mg/dL, glucose 68 mg/dL and positive BOC. Brain MRI
fuse leptomeningeal enhancement, with a fine and regular symptoms; 10 years later, during an interruption of aza- showed T2/FLAIR hyperintense lesions in subcortical
pattern. She developed urinary retention. Three days after thioprine use, she noticed recurrent paresthesias in the WM in left temporo-occiptal lobe and left precuneus
discharge, she was diagnosed with a optic neuritis. A new arms and developed sudden visual acuity loss of the left associated with SWI hypossinal areas that could repre-
CSF analysis revealed just a lymphocytic pleocytosis 53 eye; fundoscopy with optic disk with low-defined mar- sent hemosiderin deposition. Anti-NMDAR antibody was
cells/mm3 and no OCBs. She was readmitted with a classic gins and pale color suggestive of optic neuritis; Treatment detected in serum and CSF. The patient received IVIG and
clinic of an optic neuritis on the right (20/40). All types of with pulsotherapy with methylprednisone 1g for 5 days MPIV with improvement. After 16 months she became
blood tests were requested, with no changes. Therefore, in Hospital was performed, with partial improvement impulsive, irritated, with difficulties in planning com-
she was treated with IV 5 grams methylprednisolone, and of visual acuity; Brain MRI: smooth signal change and plex activities. Brain MRI showed multiple demyelinating
a new MRI revealed findings of involvement of the right apparent enhancement by contrast of the left optic nerve. lesions in brainstem, with leptomeningeal enhancement.
optic nerve, in addition to signal hyperintensity on the side Cervical MRI: focus of signal change in the vertebral cord, CSF showed 6 cells, protein 26mg/dl, glucose 56 ml/dL,
of the pons on cranial MRI associated with the finding on most evident at the bulb and at the level of C2, C4 and C5, BOC +. Anti-MOG, anti-APQ4 and anti-NMDAR were
spinal MRI with anomalous gadolinium enhancement at without significant enhancement by contrast; dosage of negative. EEG showed rare epileptiform discharges in
the level of the conus medullaris, suspicious findings of an anti-aquaporin 4 IgG: reagent 1: 20; receiving a diagno- right frontoparietal lobe. According to 2017 MacDonald
autoimmune nature due to MOGAD. Foscarnet was started sis of neuromyelitis optic (NMO); Discussion: MG and criteria she was diagnosed with Multiple Sclerosis (MS).
for 14 days and on the Hospital discharge, she presented NMO are immune-mediated disorders with low preva- HLA-DRB1*15: 01, HLA-DQA1*01: 02/06: 02 were nega-
resolution of all symptoms. Positive result of serum anti- lence in the general population. MG is a neuromuscular tive. She was treated with MPIV, and received rituximab.
mog antibodies. Primary infection with HHV-7 can occur disorder associated with the presence of anti–acetylcho- In After 22 months she developed dizziness, nausea, mild
in adults which can lead to different manifestations such line receptor antibodies (Achr-Ab) and characterised by upper limb ataxia, pallor of optic disc with bilateral visual
as encephalomyelitis, however studies show that cases of fluctuating muscle weakness. NMO is an inflammatory acuities of 20/50. EDSS was 2,0. Brain MRI showed new
secondary optic neuritis are uncommon. Onset of demy- disease of the central nervous system that mainly affects infratentorial and supratentorial demyelinating lesions.
elinating diseases, such as multiple sclerosis, due to EBV the spinal cord and optic nerve, and is associated with Alemtuzumab was indicated. Discussion: We report a
infection have already been scientifically proven and presence of anti–aquaporin-4 antibodies (AQP4-AB) in patient that developed MS after anti-NMDAR encepha-
the occurrence of MOGAD spectrum diseases have also 60%-80% of patients. Both AQP4-AB-positive NMO and litis, a rare association. An overlap between anti-NMDAR
been described after reports of infectious mononucleosis Achr-AB-positive MG are associated with other autoim- encephalitis and neuro-inflammatory disorders, mainly
by EBV and VZV infection. Case presentation showing mune diseases and autoantibodies, both organ-specific with NMOSD and MOGAD was described in a large series.
an association between MOGAD and HHV-7 infection and systemic. Despite the rarity of MG and NMO, several However, association with MS is limited to only a few
are rare. However, due to molecular similarity between cases and small series of patients with both disorders have case-reports. Hypothesis for such association include:
virus from the herpes family to EBV, it can be suggested been reported over the years. Aqp4-NMO is recognized medication-induced autoimmunity, exposed epitopes
that the herpes virus family may be triggering agents of to be associated with other autoimmune manifestations during disease course, or EBV trigger. Final Comments:
autoimmunity. Therefore, in the rare clinical presentation in about 25%–50% of cases, both organ-specific and sys- In conclusion, recurrence of neurological symptoms in
of MOGAD spectrum disease possibly triggered by an temic. Final Comments: Clinicians should have a low anti-NMDAR encephalitis, the presence of unspecific
infection caused by HHV-7, the description of this case threshold to screen for comorbid autoimmune disease in brain MRI lesions at first attack and BOC + should trigger
is very important for this etiology to be considered in patients with MG and NMO, in which non-invasive testing additional investigation and raise the possibility of MS.
the differential diagnosis of an infectious condition that for antibodies is specific and allows for early treatment A question is whether the overlap between anti-NMDAR
evolves with demyelination. and better outcomes. encephalitis and the demyelinating disorders occurs by
chance or whether they may be mechanistically linked

Neuroimunologia Neuroimunologia
Neuroimunologia

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XXX Congresso Brasileiro de Neurologia

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COMBINED CENTRAL AND PERIPHERAL AUTOIMUNE ENCEPHALITIS AFTER 1ST GANGLIONOPATHY AND LONGITUDINALLY
DEMYELINATION: A CASE PRESENTATION DOSE OF PFIZER COVID-19 VACCINE EXTENSIVE MYELOPATHY ASSOCIATED
IN A 16-YEAR-OLD PATIENT: CASE WITH SJOGREN’S SYNDROME, IN A
Mariana Ribeiro Pereira, Luana Ribeiro Silveira,
André Bertola Vanzan Filho, Karina Lebeis Pires, PRESENTATION PATIENT WITH A BACKGROUND OF POST-
Alexandre Hofke Alamy, Arthur Fernandes Cortez, VACCINATION POLYMYELITIS: A CASE
Victória Rosas Marques, João Pedro Moreira
Claudia Cristina Ferreira Vasconcelos Guilherme, Danilo Issa Mitozo Veras, Emmanuel PRESENTATION
HUGG. Rio de Janeiro RJ, Brazil Avelino de Lima, Cecília Tizatto Barroso, Wendell Larissa Clementino Leite de Sa Carvalho, Marco
Universidade Federal do Estado do Rio de Janeiro. Silveira de Souza, Daniel Sampaio e Souza, João Andre de Moraes Bernardino, Jonata Ribeiro de
Rio de Janeiro RJ, Brazil Victor da Costa Nunes, Vanise Campos Gomes Sousa, Paulo Barbosa Leite Neto, Victor Fellipe
Amaral, Tales de Oliveira Júnior Bispo Macêdo, Djanino Fernandes da Silva, João
pribeiromariana@gmail.com Eudes Magalhães, Aline Ranzolin, Fabiola Lys de
Universidade do Estado do Amazonas. Manaus AM,
Brazil Medeiros
Case Presentation: Our patient is a forty-six year old
woman, with a previous 9 year diagnosis of Multiple Universidade Federal do Amazonas. Manaus AM, Hospital Universitário Oswaldo Cruz da
Sclerosis (MS) and using intramuscular interferon beta-1a, Brazil Universidade de Pernambuco. Recife PE, Brazil
a disease-modifying therapy. She has been asymptom- Fundação Hospital Adriano Jorge. Manaus AM, Hospital Universitário Oswaldo Cruz, Universidade
atic for 3 years, until she started to report numbness Brazil de Pernambuco – Recife CE – Brazil
and weakness beginning at her feet and knees ascend- vrm.med19@uea.edu.br clementinolari@gmail.com
ing to upper limbs symmetrically over the course of 48
hours. She denied a previous history of gastrointesti- Case Presentation: A 16-year-old male patient, from a Case Presentation: In March 2020, arthralgias, peri-
nal or respiratory symptoms. At examination, she pre- countryside city in Amazonas, was referred to our service oral numbness, difficulty to speech, voice, swallowing,
sented with grade 2 and 3 muscular strength on lower reporting sudden onset of paresthesia and paraparesis in walking and forgetfulness, developed in a 44-years-
and upper limbs, respectively, associated with hypoto- the lower limbs, associated with chest tremors, dyspnea old woman. November/2020, she had COVID-19, and
nia and areflexia. We also observed a sensory level at T4 and anterograde amnesia one day after receiving his 1st trunk/limbs weakness, with abnormal hand movements
and Lhermitte sign. Cerebrospinal fluid showed albu- dose of Pfizer COVID-19 vaccine. When admitted to the appeared. In childhood, she presented post-vaccina-
min-cytologic dissociation and nerve conduction stud- Hospital, he had an GCS of 14, spasms in the abdominal tion poliomyelitis (atrophy in the right leg). June/2021,
ies (NCS) presented the findings of acute sensory-motor and thoracic muscles and plastic hypertonia in the left Neurological exam: Alertness, tachycardia, tachy-
demyelinating polyradiculoneuropathy, characteristics upper limb. After admission, he presented with gener- pnea, and hypotension. Performed simple commands,
of Guillain-Barrè Syndrome (GBS). Spinal cord Magnetic alized myoclonic seizures without loss of consciousness reduced fluency. Hypomimic face, dysphonia, dysar-
Resonance Imaging (MRI) presented a T2 high-intensity associated with constant high-pitched tinnitus bilater- thria. Equine right foot and fasciculations in the right
lesion from C1 to C5. We opted to treat the patient with ally and his neurological examination showed grade 3 leg. Global hypotonia. Force graded MRC 2. Reflexes
intravenous Methylprednisolone for five days. She started reflexes in right biceps and patellar, left brachioradialis abolished. Indifferent plantar responses. Apalesthesia
to improve significantly and was walking again 5 weeks and triceps bilaterally, in addition to hypoesthesia in from C3. Normal superficial sensibility. Choreic and
after the first symptoms initiated. Discussion: The co-oc- the left hemibody and bilateral nystagmus. The patient dystonic movements in face and upper limbs. Brain
curence of MS and GBS is rare, with less than 20 cases was treated with phenytoin, which was later replaced by MRI-hypersignal in deep white matter, pons and gliosis
described around the world until now. Demyelinating sodium valproate, associated with diazepam and empiric in the right temporal pole. Cervical MRI-hypersignal
diseases involving both central nervous system (CNS) and acyclovir. Given the information presented, the hypothesis region C3-T1(posterior). Electroneuromyography- lower
peripheral nervous system (PNS) are usually etiologically of autoimmune encephalitis was raised. Due to logistical motoneuron pathology with a sensory ganglionopathy in
heterogeneous. Recently, autoantibodies against nodal difficulties caused by the pandemic, the patient underwent four limbs. Polysomnography revealed significant oxygen
and paranodal proteins were discovered (NF186, NF155, only a cranial magnetic resonance imaging 18 days after desaturation. FAN 1: 320 and anti-Ro >240. Schirmer’s
CNTN1 and CASPR1) and associated chronic and acute admission, where no changes were evidenced. After an test was positive to xerophthalmia. Anti-aquaporin-4
demyelinating diseases. Combined Central and Peripheral increase in the frequency of seizures, he was referred to negative. Initially, methylprednisolone (1g/5days) was
Demyelination (CCPD) is defined fulfilling of the following the ICU for treatment with plasmapheresis. One month performed, without improvement, followed by immu-
criteria: a presentation of T2 high-signal intensity lesions after his admission, he was discharged using levetirac- noglobulin (0.4mg/Kg/5days), without major motor
in the brain, optic nerves, or spinal cord upon MRI, or etam and the case was followed up in our outpatient gains. Subsequently, she underwent cyclophosphamide
abnormalities on visual-evoked potentials (VEPs), and service. Discussion: Autoimmune encephalitis presents (750mg/m2/day-3months), with interaction, swallow-
conduction delay, conduction block, temporal dispersion as a range of acute neurological symptoms, which can ing and abnormal movements improved, but continued
or F-wave abnormalities according to NCS. This condition include headaches, altered mental status, seizures and with force graded MRC 2. Then, Rituximab was realized
is highly sensitive to intravenous corticosteroids, with an autonomic and movement disorders, and can or can not (1000mg-15/15days), with recovery of cervical and trunk
outcome of symptom recovery of 83,3%. Our patient pre- be associated with a paraneoplastic syndrome. Its treat- support. She is in homecare, with speech-physical thera-
sented both criteria to CNS and PNS involvement simul- ment is based on immunosuppressive therapies such as pies, using nighttime beeper, and waiting for Rituximabe
taneously and a significant response to treatment with steroids, immunoglobulins or plasmapheresis, favoring (2nd cycle). Ten months Hospitalized, there were com-
intravenous Methylprednisolone. Final Comments: We the patient’s prognosis in case of an early diagnosis. Final plications, requiring ICU, due to dysautonomia, acquired
hope to shed light on the rare association between CNS Comments: In this case, we bring a severe and rare pre- tuberculosis, respiratory infections, which were circum-
and PNS demyelinating diseases and encourage a better sentation of post-vaccination encephalitis. It is essential to vented. Discussion: Sjögren’s syndrome (SS) is a chronic
investigation of ascending acute sensitive symptoms in shed light on such adverse reactions, not to question the autoimmune inflammatory disease with exocrine gland
patients with a previous diagnosis of MS. safety of the vaccine campaign, but to show that treating involvement and extraglandular disease features, affecting
them effectively can enable complete patient rehabilita- peripheral and central nervous systems. Autonomic neu-
tion, in addition to contributing to the formation of more ropathy cause difficult control of the symptoms. Myelitis
Neuroimunologia
accurate epidemiological evidence about neurological leading a diagnosis of NMO spectrum. The importance
disorders related to COVID-19 vaccination. of distinguishing NMO and SS is important for different
treatments and prognosis. Final Comments: Neuro-
Sjogren is a defiant illness to immunology, pathophys-
Neuroimunologia
iology and possible complications inherent to this rare
disease. Advances in disease-modifying therapies and
early interventions can greatly improve the prognosis.

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PARANEOPLASTIC NEUROMYELITIS NEUROMYELITIS OPTICA &NDASH; TONIC CASE PRESENTATION OF AUTOIMMUNE


OPTICA SPECTRUM DISORDER: A CASE SPASMS AS ASSOCIATED CLINICAL ANTI-NMDA ENCEPHALITIS IN
PRESENTATION MANIFESTATION ADOLESCENTS IN THE STATE OF RIO
GRANDE DO SUL
Luiza Barbosa de Oliveira, Isadora Lyrio Stábille, Nathalye Fernanda Pedroso Dircksen, Arthur Coelho
Fernando Lopes de Sousa, Ruth Maria Ribeiro Moura Marinho, Amanda Leticia Andre, Tatiane Fabio Pacheco Martins – Passo Fundo – RS – Brazil,
Guerra, Camilla Ribeiro de Oliveira, Renata Coelho Arroyo Lopes Alves de Jesus, Marcelo Simplicio Daniel Lima Varela, Alan Christimann Fröhlich,
Chaves Gaspar, Thiago Vieira Sasse, Bruna Essado Carvalho, Lorena Fernandes Kronbauer, Damacio Bruna Constantino Rech, Ricardo Lorenzato
Nascimento, Alex Eduardo da Silva, Lilian Vieira de Ramon Kaimen Maciel Bortoluz, Daniela Ragnini, Vitor Picanço Lima
Sousa Oliveira Gomes, Raphaell Souza, Guilherme Michelon
Irmandade da Santa Casa de Londrina. Londrina
Universidade Federal do Triângulo Mineiro UFTM PR, Brazil Hospital de Clínicas de Passo Fundo – Serviço de
Irmandade da Santa Casa de Londrina – Irmandade Neurologia e Neurocirurgia SNN
luizaboliveira@outlook.com.br da Santa Casa de Londrina – PR – Brazil Hospital de Clínicas de Passo Fundo, Serviço de
Neurologia e Neurocirurgia – SNN – Passo Fundo –
Case Presentation: A 53-year-old female patient was n.athalye@hotmail.com
RS – Brazil
admited to our Hospital with back pain history, progressive
spastic paraparesis, with urinary incontinence and con- Case Presentation: DNG, female, 26 years old, with inap- fpachecosm@gmail.com
stipation, being diagnosed with acute transverse myelitis. petence, asthenia and paresthesia in the lower limbs,
Her magnetic resonance imaging showed longitudinally started 40 days after cesarean section and with progres- Case Presentation: Patient GCS, female, 18 years old, in
extensive spinal cord lesions; and her capillary electro- sive worsening. She had painful tonic limb spasms of July 2020 started to present behavioral changes, hypersex-
phoresis (CE) showed polyclonal gammopathy (PG). She short duration provoked by voluntary movements and ualization, depression, anxiety, crying crises, emotional
received high-dose intravenous methylprednisolone with emotional factors. Former alcoholic/addict for 6 months, lability, refractory to psychiatric treatment. In April 2021,
satisfactory results. During follow-up, she tested positive she reported reduced visual acuity for 4 years after first the exacerbation of the symptoms evolved, followed by
for AQP4-IgG, fulfilling criteria for Neuromyelitis optica pregnancy, with partial improvement. On physical exam- language alteration, agitation, aggressiveness, visual hallu-
spectrum disorder (NMOSD), and was discharged with ination, in addition to spasms, there was dysesthesia and cinations, and seizures, evolving to status epilepticus. On
Azathioprine (AZT) plus prednisone. After 4 months, she paresis of limbs, hyperreflexia, bilateral reduced visual further investigation she presented small area of hypersig-
was Hospitalized again for cellulitis and infectious ulcers. acuity with diffuse left papilla atrophy. Magnetic resonance nal in T2W and FLAIR in bilateral mesial temporal region
Her complete blood count showed pancytopenia that was imaging (MRI) of the orbits showed atrophy and hyper- topography, no pathological enhancement on skull MRI
associated with the use of AZT, therefore, the medication signal in the left optic nerve, suggesting inflammatory and pleocytosis with 69 cells, exclusive intrathecal oligo-
was suspended. The patient progressed with worsening optic neuropathy, cervical spine MRI with hypersignal in clonal bands and presence of anti-NMD-receptor anti-
cytopenias. Another CE disclosed monoclonal gammopa- T2 bulbomedullary, in C2-C3 and C6-C7, corresponding bodies in cerebrospinal fluid. She was initially approached
thy (MG), and a myelogram was performed, which showed to demyelinating lesion, increased CSF of proteins and with acyclovir, pulse therapy in another service, with no
60% of clonal plasma cells in the bone marrow. As she was positive anti-aquaporin 4 antibodies (anti-AQP4). Pulse response. We followed treatment with immunoglobulin,
diagnosed with Multiple Myeloma (MM), with IgA/Kappa therapy with 1g Methylprednisolone was performed for again without response, with ongoing epilepsy and orofa-
monoclonal protein, isolated Kappa light chain and beta-2 5 days and 40 mg of prednisone was maintained with cial dyskinesias. Therapy with Rituximab was subsequently
microglobulin, AZT was discontinued and myeloma treat- gradual weaning. There was a good response with com- followed, resulting in severe pharmacoderma in the con-
ment started with Cyclophosphamide, Bortezomib and plete recovery. Currently on Azathioprine 50mg 2 times dition of Toxic Epidermal Necrolysis (TEN), combined
Dexamethasone (CYBORD protocol). Discussion: Studies a day. For the treatment of spasms, we opted for the to secondary complications such as multidrug-resistant
report a link between autoimmune diseases accompanied use of benzodiazepine and carbamazepine, with good infections, that greatly increased morbidity. At the first
by diagnosis of MG. A study described 6 cases of paraneo- control. Discussion: Neuromyelitis optica (NMO) is an opportunity of clinical stabilization, cyclophosphamide
plastic NMOSD. In his results 5 patients were older than 50 autoimmune inflammatory disease of the central ner- therapy was proceeded, obtaining good response in neu-
years old, and 4 had positive AQP4 antibodies. The median vous system (CNS), characterized by simultaneous or ropsychiatric manifestations. The patient maintained
time between NMOSD and cancer was 12 months. A case consecutive cases of optic neuritis and transverse myeli- the rehabilitation with a slow and gradual recovery, and
series reported a 58-year-old female with visual defects tis. The majority (80%) is caused by IgG autoantibodies today she has returned to her family, walks without help,
and acute transverse myelitis, with a concomitant finding anti AQP4– a protein that forms a water channel in CNS. and eats orally. Discussion: In this case, we discuss the
of MG in CE. Similarly to our case, there were no lytic bone The clinical manifestations are diverse, being normally importance of autoimmune encephalitis among the dif-
lesions,hypercalcemia and renal impairment. Generally, present the reduction of visual acuity and paresis/pares- ferential diagnoses of neuropsychiatric conditions, its
patients with prolonged autoimmune diseases have PG. thesia in limbs with sensitive level. Tonic spasms are not diagnosis delay, mainly due to the onset of psychiatric
Therefore, CE with protein immunofixation and free light common and are characterized by sudden onset of tonic manifestations and consequently delay in treatment.
chain quantification is adequate for MG detection. The postures, short duration, triggered by voluntary move- We also present the reality of a committed neurology
pathogenesis involving inflammatory states and clonal ments, sensory stimulation or hyperventilation, with or service in the northern interior of the state of Rio Grande
transformation is still unclear. Polyclonal proliferation without pain and are due to axonal irritability due to the do Sul. The N-methyl-D-aspartate (NMDA) anti-receptor
may progress to monoclonal proliferation of B cells and release of inflammatory mediators. On MRI there is hyper- encephalitis first described in 2007 is the most common,
induce MG. Possibly, there is involvement of dysregulated signal in the optic nerve and spinal cord, in addition to and children and young adults should always be consid-
cytokines. Final Comments: We suggest that MG should anti-AQP4 in blood/liquor. Treatment is with pulsether- ered. The research of these receptors in the cerebrospinal
be ruled out for patients with prolonged autoimmune apy, followed by immunosuppressive medication. Tonic fluid confirms the diagnosis, since brain images may be
diseases. There is probably a direct mechanism between spasms respond well to carbamazepine. Final Comments: normal. The therapeutic approach consisting of immu-
cancer cells and development of NMOSD, however, fur- NMO is a disease with multiple clinical manifestations, nosuppression should be initiated as early as possible to
ther studies are required. sometimes presenting atypical symptoms as in the case improve the outcome. The possibility of adverse reactions
above. It is up to the physician to make an early diagnosis to potent and consolidated medications should always be
for appropriate treatment, seeking control of symptoms taken into consideration.
Neuroimunologia and a favorable outcome.

Neuroimunologia
Neuroimunologia

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PARANEOPLASTIC ENCEPHALOMIELITIS MYELIN OLIGODENDROCYTE GLYCOPROTEIN HYPERSOMNOLENCE AND


DUE TO ANTI-HU ANTIBODIES: A CASE ANTIBODY-ASSOCIATED DISEASE ENCEPHALOPATHY AS A MANIFESTATION
PRESENTATION (MOGAD) WITH PNS INVOLVEMENT: A CASE OF NEUROMYELITIS OPTICA: A CASE
PRESENTATION PRESENTATION
Alison Mangolin, Victor Augusto Zanesi Maciel,
Lucas Goldim Briand Vieira, Rodrigo Siqueira Soares Paulo Hermes Lima Amaral, Nise Alessandra de Luiza Gonçalves Fraga, Thaís Takamura, Ludmila
Frezatti, Pedro Jose Tomaselli, André Cleriston Jose Carvalho Sousa, Rafael Cavalcanti Fernandes Machado Lima, Camila Carneiro Ferreira, Bruno
dos Santos, Wilson Marques Junior Rocha, Luiziane Maria da Silva Alves, Flavia da Camporeze, Pedro Cougo Samueli, Henry Koiti Sato,
Costa Mourão, Leonardo Rodrigues Leopoldo Matheus Pedro Wasem
FMRP Universidade de São Paulo. Ribeirão Preto
de Menezes, Wesley Lopes da Silva, Ingrid
SP, Brazil Instituto de Neurologia de Curitiba. Curitiba PR,
Demosthenes Wanzileu, Camille Albuquerque
fmrp Universidade de São Paulo. Ribeirão Preto SP, Brazil
Rodrigues Chirano, Talísia Nascimento Vianez
Brazil
Universidade Federal do Amazonas. Manaus AM, luizaag.fraga@gmail.com
alison11235@gmail.com
Brazil
Case Presentation: Female, 77 years old, hyperten-
Case Presentation: A 75-year-old man, with multiple phla.med@uea.edu.br sive. In January 2021, she started presenting behavioral
cardiovascular risk factors and a previous history of heavy changes, difficulty looking up and binocular horizontal
smoking, presented to our clinic due to a forty-five days Case Presentation: GLF, male, 13 years old. The patient diplopia. The medical team performed brain MRI which
history of progressive right hemiparesis and hemipares- came for consultation in June 2020. At the age of three, presented a slight expansive effect on the mesial portions
thesia. On neurologic examination he had normal cranial he had a generalized tonic-clonic febrile seizure with of the thalamus, hypothalamus and midbrain. After being
nerve evaluation. Motor examination revealed a right post-ictal drowsiness. From then on, a difficulty in school Hospitalized for investigation, the patient evolved to an
hemiparesis with upper motor signs (DTR were patholog- learning was noticed. At the age of 7, he evolved with bilat- altered level of consciousness, with severe drowsiness. She
ical and cutaneous response was extensor). Pinprick was eral visual impairment with pain at eye mobilization. Two was submitted to a lumbar puncture and a non-reactive
reduced distally and vibration was severely impaired with years later, he started with distal weakness and frequent anti-AQP4 test. Part of the liquor was sent to Vienna. On
positive Romberg sign. Brain MRI showed previous lacu- falls. He had no family history. The patient was oriented, 02/02, a stereotaxic biopsy was performed due to suspi-
nar infarcts and no abnormalities in diffusion-weighted but with childish behavior for age. Mini-mental state cion of a tumoral lesion. Treatment with IV pulse therapy
imaging. The patient had no signs of infection whatso- exam: 22/30. He also presented flaccid tone and global was started. On 02/08 she had reentrant seizures and one
ever. The CSF analysis showed normal cell count with hypotrophy associated with symmetrical tetraparesis week after started IV immunoglobulin. On 02/25 she was
sligtly increased protein. Nerve conduction studies were predominantly crural. Deep tendon reflexes and cuta- discharged from Hospital with partial improvement in
normal and needle examination revealed chronic neuro- neous-plantar reflex were absent. Cerebellar gait with her consciousness level. The next day she returned due
genic features. EEG revealed diffuse desorganized back- dysdiadochokinesia and dysmetria was evident. Distal to worsening of the drowsiness. In a new examination, a
ground with no epileptic discharges. After three weeks hypopalesthesia was noticed. Visual acuity: 20/300 in reduction of previously altered areas in the thalamus was
of Hospitalization, the patient’s level of consciousness the left eye and 20/200 in the right eye. Fundoscopy seen. A new therapeutic attempt was performed, includ-
got progressively worse and orotracheal intubation was showed bilateral optic nerve atrophy. Considering the ing Cyclophosphamide 1g IV, evolving with a significant
needed. In the setting of sensory ataxia, progressive phenotype of presentation, findings in complementary improvement in the level of consciousness, but still pre-
encephalopathy, without focal injury on neuroimaging exams and epidemiological profile, we chose to measure senting periods of drowsiness and cognitive impairment.
or signs of infection in an elderly patient, a paraneoplastic the anti-MOG IgG, with a positive result, corroborating a On 03/26, a result of anti-AQP4 from Vienna was sent
panel was ordered, revealing anti-Hu antibodies in CSF diagnosis of Myelin oligodendrocyte glycoprotein anti- back with a reactive result. Discussion: Neuromyelitis
and sera. The patient underwent neoplastic screening and body-associated disease (MOGAD) with PNS involvement, Optica (NMO) is characterized by the presence of anti-
a pulmonary nodule was identified. Unfortunately, before manifested in our patient as polyradiculoneuropathy. bodies called Aquain 4 present in the hematoencephalic
it was possible to biopsy the pulmonar mass, he died from Discussion: MOGAD is an immune-mediated demye- barrier. It has predominantly female occurrences and the
infectious complications. Discussion: Anti-Hu antibodies linating inflammatory disorder of the central nervous first symptoms occur between the 3rd and 4th decades
is part of the onconeurals antibodies. The anti-Hu para- system predominantly affecting the optic nerves, brain, of life. The most common clinical manifestations include
neoplastic neurologic syndrome may present as sensory and spinal cord. The disease has a predilection for chil- visual impairment and bilateral motor symptoms due
neuronopathy, chronic gastrointestinal pseudoobstruc- dren. Oligodendrocyte myelin glycoprotein (MOG) is a to optic neuritis and transverse myelitis. There are other
tion, encephalomyelitis, and limbic encephalitis. When protein on the surface of oligodendrocytes that has his- NMO spectrum syndromes, including an encephalopathy
anti-Hu is identifiable in blood tests, cancer is almost torically been considered as a potential antibody target characterized by other levels of consciousness syndromes
always found. being small cell lung cancer the most in multiple sclerosis, until in 2007 it was described as a and seizures. The diagnoses are based on the presence of
common. Our patient presented risk factors for oncologic different disease phenotype. According to a database AQP4 antibody associated with at least one outbreak of
disease to his age, previous smoking history, and clini- review (PUBMED), there are a few studies that corrobo- clinical syndromes. One can still have the diagnosis with-
cal course. Paraneoplastic syndromes are a differential rate the occurrence of peripheral impairment associated out the presence of the antibody along with two clinical
diagnosis in these cases and investigation of this cause with MOGAD, present in 7% of MOGAD patients in an outbreaks and the presence of extensive lesions in the
is required. Final Comments: Paraneoplastic neurologic Australian cohort. There are some hypotheses to justify optic nerve, spinal cord or brain stem. Final Comments:
syndromes can affect any part of the nervous system. This the concomitant central and peripheral demyelination In patients with positive AQP4 antibody, it is possible to
group of rare disorder is becoming more prevalent as the processes: one of the most accepted claims that MOG make the diagnosis of NMO even with atypical clinical
population age and with new paraneoplastic panel being is found in an isoform that can be released into the CSF, presentations. Our patient presented from the beginning
affordable. The neurologist must include these group of triggering or “spreading” autoimmunity when drained the typical symptoms of NMO Encephalopathy in addition
disorder in the differential diagnosis, specially when the into the PNS. Final Comments: The presence of central to radiological changes, but the diagnosis was concluded
patient has risk factors for neoplasm. demyelination syndrome and SNP involvement may be after the second AQP4-AB test with a positive result.
associated with MOGAD and may respond to immuno-
therapy, and should not discourage the search for MOGAD
Neuroimunologia in patients with an appropriate clinical setting. Neuroimunologia

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CASE PRESENTATION OF A 73-YEAR-OLD CASE PRESENTATION OF A 59-YEAR-OLD OPHTHALMOPARESIS SECONDARY TO IGG4-


PATIENT WITH INCLUSION BODY MYOSITIS PATIENT WITH TRANSVERSE MYELITIS, RELATED DISEASE – A CASE PRESENTATION
EVOLVING WITH BRADYCARDIA AFTER CEREBELLAR SYNDROME AND ADEM AFTER
Paulo Henrique Martinelli Oliveira, Lucas Silva
VACCINE FOR COVID VACCINE FOR COVID IN BRAZILIAN AMAZON Dias, Raderi Luiz Cardoso dos Santos, Indianara
João Victor Oliveira de Melo, João Pedro Moreira João Victor Oliveira de Melo, Cecilia Tizatto Keila Pastório, Lorena Dias Araújo, Francine de
Guilherme, Victória Rosas Marques, Emmanuel Barroso, Orizon Hilario Inocente, João Pedro Paula Roberto Domingos, Rafael de Almeida, Sayuri
Avelino de Lima, Lucas Bentes Rodrigues, Daniel Moreira Guilherme, Maria Clara Paulino Campos, Aparecida Hirayama, Raquel Mattos Filgueiras
Sampaio e Souza, Bruda Guimarães Dutra, Tales de Bruna Guimarães Dutra, Neyde Alegre de Souza Hospital Municipal Dr. José de Carvalho Florence.
Oliveira Júnior, Vanise Campos Gomes Amaral Cavalcante, Tales de Oliveira Júnior, Vanise Campos São José dos Campos SP, Brazil
Gomes Amaral
Universidade Federal do Amazonas. Manaus AM, paulohmoliveira96@gmail.com
Brazil Universidade Federal do Amazonas. Manaus AM,
Universidade do Estado do Amazonas. Manaus AM, Brazil Case Presentation: A.L.O.S, female, 61 years old. The
Brazil Universidade do Estado do Amazonas. Manaus AM, patient related binocular diplopia, ophtalmoparesis –
Fundação Hospital Adriano Jorge. Manaus AM, Brazil there was no horizontal movement and limited vertical
Brazil Fundação Hospital Adriano Jorge. Manaus AM, movement – and fixed semiptosis in the left eye that
Brazil started 3 days before the medical care. The patient expe-
jvictor71@yahoo.com.br
jvictor71@yahoo.com.br rienced similar symptoms two years ago, wich solved
Case Presentation: A 73-year-old male patient, from spontaneously. Previous diseases were osteoporosis,
Manaus, Amazonas, previously diagnosed with Inclusion Case Presentation: A 59-year-old patient from Manaus, dilispidemia and systemic arterial hypertension. The
Body Myositis (IBM) confirmed in 2017 by the mus- Amazonas, seeks emergency care at an emergency Magnetic Resonance Imaging showed expansive/infiltra-
cle biopsy method, which presented an inflammatory Hospital after the fifth day of AstraZeneca vaccine for tive formation compromising the left lateral rectus mus-
myopathic histological pattern with a large amount of COVID-19, presenting progressive, symmetrical and culature and a lesion with the same sign in the temporal
COX- negative. The patient was taking prednisone 5mg/ ascending loss of strength in the lower limbs with 3 days musculature in the same side. Cerebrospinal fluid anal-
day, ramipril 10mg/day, hydrochlorothiazide 25mg/day of evolution, without other associated symptoms, deny- ysis was normal. Lateral rectus muscle biopsy confirmed
and glicazinde 60mg/day at home. On the twenty-sixth ing diabetes, systemic arterial hypertension, alcohol IgG4-related disease. The patient started corticotherapy
day after the third dose of Pfizer vaccine for covid-19, the consumption or smoking. Upon admission, physical (prednisone 40 mg/ per day). Discussion: IgG4-related
patient appears for an outpatient follow-up consultation examination revealed a Glasgow Coma Scale 15, isochoric disease is an imunomediated disease characterized by
for underlying disease and for investigation of complain- and photoreactive pupils, no signs of meningeal irritation, enlargement of the organs and lymphoplasmtyc infil-
ing of lipothymia and vertigo, denying any other sign of grade 5 muscle strength in the upper limbs and grade 0 in tration. It is a rare condition that affect 2,2 to 100000
infection. During the evaluation, dyspnea was verified in the lower limbs according to MRC, areflexia in the lower people. Extraocular muscle involvement in this disease
MRC Class 3 and, in physical examination, a heart rate limbs and decrease of superficial and deep sensitivity on has been related but with a poor description of the eye
of 34 bpm was verified. Due to the condition, the patient them. Initially, the patient was admitted to the Intensive movement. The literature describes IgG4-related disease
was immediately advised by our neurology team to go Care Unit (ICU), staying for 11 days for investigation for eye movement muscles involvement as a condition that
to the emergency Hospital. In the emergency room, an Guillain-Barré Syndrome (GBS) and for pulse therapy with is associated to pain, proptosis and ophtamoplegia. The
electrocardiogram (ECG) was performed and a Complete methylprednizolone 1000mg IV for 5 days, but without case presented above had a different clinical presentation
Heart Block (CHB) was identified and the implantation improvement of symptoms. He was later transferred to because semiptosis and sever ophtalmopharesis that is
of a temporary transvenous pacemaker was chosen as our teaching Hospital, where a new neurological assess- uncommon in IgG4-related disease; also, there was no
an emergency therapeutic approach, later, a permanent ment was performed, identifying persistence of the pre- eye pain. Final Comments: IgG4-related disease usu-
pacemaker was implanted. Patient was Hospitalized for vious findings, in addition to the presence of horizontal ally appear as a condition with normal ocular motility.
21 days in an Intensive Care Unit (ICU) for follow-up and choreic movements of the head, intention tremor in the Once there are lots of other diseases that compromisse
procedures, today he has relief from dyspnea and lipo- upper limbs, significant dysmetria, worse on the left, nys- eye movement muscles this case repor is important to
thymia. Discussion: Cardiovascular damage is a known tagmus and without alteration of cranial nerves. During neurologists to show na unexpectd clinical presentation
comorbidity of myopathies and can evolve with func- Hospitalization, two biochemical evaluations of CSF and call atention to the need to make a correct diagnosis
tional and conduction limitations. For example, cases were performed, but with unspecific results. The patient despite of na atypical presentation.
of atrioventricular conduction delay have already been evolved with complications of cutaneous, urinary and
reported in a patient with IBM and, similarly, cases of pulmonary infections and died of sepsis after 110 days of
Neuroimunologia
CHB after vaccination for COVID-19 have already been Hospitalization. Discussion: Acute disseminated enceph-
reported. However, the causes of CHB are vast, making it alomyelitis (ADEM) is a rare inflammatory disease of
difficult, in some cases, to identify its etiology. Thereby, autoimmune etiology that may have a presentation sim-
the interconnection between vaccination, CHB and IBM ilar to GBS. In the current context, there are already a few
can be arduous. Final Comments: In this case, we pres- reports that point to cases similar to the one we present,
ent a possible correlation between the Pfizer vaccine for showing a possible correlation between the vaccine for
covid-19 and abnormal electrical conduction in a patient COVID-19 and ADEM. Final Comments: In this case,
affected by IBM. However, some limitations should be we present a possible and rare correlation between the
mentioned: despite the temporal correlation between the Astrazeneca vaccine for COVID-19 and ADEM. That said,
vaccine dose and the onset of symptoms being suggestive the present report seeks to encourage more discussions
of inference and the existence of subsidies for connection about the possible complications of vaccination without,
with the baseline diagnosis, tests were not performed to however, questioning its safety, since adverse effects are
directly prove the correlation between the three factors. extremely rare.

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CASE PRESENTATION ACUTE ISCHEMIC A REMARKABLE RADIOLOGICAL TITLE: PAI-1 INHIBITOR GENE 4G5G
STROKE IN BEHÇET’S DISEASE IMPROVEMENT IN A PATIENT WITH NMOSD POLYMORPHISM IN THE CONTEXT OF
RECURRENT STROKE AS A DIFFERENTIAL
Isabela Badan Fernandes, Gabriel Flamarin Gustav Barbosa Falcão, Kézia Souza Pinheiro, João
Cavasana, Bruno Eiji Nakano, Alana Strucker Pedro Moraes Miossi, José Guilherme Martins Lima, DIAGNOSIS OF RELAPSING-REMITTING
Barbosa, Eduardo Silveira Marques Branco, Paula Rodrigo Leite Marinho, Gabriel Marim Roni, Arthur MULTIPLE SCLEROSIS
Carolina Grande Nakazato, Luiz Henrique Soares Prando Barros, Marcos Rosa Jr, Bruno Batitucci
Claudia Cristina Ferreira Vasconcelos, Andre Bertola
Santos Stefano, Fábio de Araújo Pereira, Natalia Castrillo, Vera Lúcia Ferreira Vieira
Vanzan Filho, Marilza Campos de Magalhães
Samadello Melges, Luiz Domingos Mendes Melges
UFES – Vitória – ES – Brazil
HUGG-UNIRIO/EBSERH. Rio de Janeiro RJ, Brazil
Faculdade de Medicina de Marília – Marília – SP –
Brazil gustav.falcao@edu.ufes.br
cfvas@hotmail.com
bruno.nakano1@gmail.com Case Presentation: A 43-year-old woman presented to
Case Presentation: A – Woman, 40 years old, in 2017 had
the neurology department quadriplegic, with hypoes-
a visual blurring in the left eye upon waking up, and her
Case Presentation: A 16-year-old male patient, previously thesia below the C4 level, bilateral impaired vision, no
brain CT scan suggested stroke. Due to further motor defi-
healthy, presented a sudden onset of severe headache alterations in other cranial nerves and bilateral Babinski
cit, brain MRI was performed and were observed multiple
associated with paresis of the right hemibody. Undergoing sign. She had a history of similar symptoms 7 months
hyperintense focal lesions on T2/FLAIR in white matter,
Hospitalization in a highly complex local referral Hospital before, but was not examined at that time. The dosage of
one of them enhanced contrast. CSF analysis, cervical
for treatment and investigation. During the Hospital stay, vitamin B12 was 88 pg/mL, the AQP4-IgG was negative,
and dorsal spinal cord MRIs were normal. A screening
he develops a new condition of left hemiparesis, sud- MOG-IgG was not available, and other laboratory results
for microvascular disease, autoimmune diseases, bacte-
denly. In both events, areas of cerebral ischemia were were non-significant. Lumbar puncture was not performed
rial/viral infections and hereditary and acquired throm-
evidenced, confirmed by magnetic resonance imaging, because the patient was in full anti-coagulation. A mag-
bophilia were negative. In 2019, she presented motor
in different locations. After active investigation, patient netic resonance imaging (MRI) of the spine evidenced
aphasia and a new brain MRI showed a left frontal isch-
and mother confirmed a personal history of recurrent areas of hyperintensity on T2-weighted sequences from
emic lesion. In 2020 it evolved transient stroke episodes.
oral ulcers, episodes of ulcers in the penile region, and the area postrema to the conus medullaris, compatible
An investigation for thrombophilia causes, a mutation
ulcerated skin lesions. Complementary exams: Skull with longitudinally extensive transverse myelitis. The
in the polymorphism in the PAI-1 gene, 4G allele, was
Resonance: Temporo-parietal signal alteration on the patient met the diagnostic criteria for neuromyelitis optica
detected. B – A woman, 26-year-old in December 2020
right represented by hypersignal on T2 and FLAIR which spectrum disorders (NMOSD) without AQP4-IgG, and a
had sequential generalized motor seizures, a brain CT
may correspond to an ischemic event. Entry labs exams course of pulse therapy with methylprednisolone was
scan showed right cortical and subcortical hypodensity
(2/16/2022): ESR 150, Bilirubin 2.4, TGO 38, TGP 51, prescribed, as well as one plasma exchange therapy, and
with signs of subarachnoid bleeding. He remained with
Reticulocytes 2.5%, DHL 519. Pathergy test was positive. vitamin B12 replacement. She used oral prednisone until
mild right hemiparesis. A control MRI showed areas of
Patient submitted to Hospital pulse therapy treatment azathioprine was available. Five years after this episode,
right parietal encephalomalacia, with hemosiderin res-
with 1g/day of Methylprednisolone for five days, associ- the patient returned to the neurology department for a
idues, and multiple small hyperintense focal lesions on
ated with full anticoagulation, evolving with stability of follow-up consultation. She was in a good general state,
T2 and Flair in white matter, some of them with appar-
the condition. Patient referred for outpatient follow-up no longer in need of a wheelchair, with a spastic paretic
ent enhancement by gadolinium, suggesting demye-
after Hospital discharge, with oral corticosteroid main- gait, no alterations in cranial nerves, muscular strength
lination. The CSF analysis showed positive oligoclonal
tenance, therapeutic anticoagulation and immunosup- of 4/5 in the upper limbs and 3/5 in the lower limbs,
bands. Cervical and dorsal spinal cord MRI, visual and
pressive therapy planning. Discussion: Behçet’s Disease and hypoesthesia below T10 level. Her EDSS was 5,5. A
somatosensory evoked potentials were normal. An inves-
is an autoimmune syndrome, and among the systemic new MRI of the spinal cord showed a great radiological
tigation for polymorphic alteration of the PAI-1 inhibitor
vascular diseases, is notable for its ability to involve improvement, with only small areas of hyperintensity
gene, configured as 4G5G, was detected. Discussion: In
blood vessels of all sizes. Affects young adults aged 20 to at the cervical spine. The reported case shows a patient
PAI-1, the 4G allele, suppresses fibrinolysis by inhibiting
40 years and the most common clinical features are the diagnosed with NMOSD with an important clinical and
tPA and uPA, plasminogen activating enzymes, thereby
presence of recurrent and painful mucocutaneous ulcers, radiological improvement after 5 years of evolution. The
suppressing plasmin formation. Normal polymorphism is
painful urogenital lesions and various skin lesions. The initial manifestation on MRI, with a centrally located lesion
5G/5G. The 4G5G and 4G4G polymorphic presentations
neurological impairment of the syndrome occurs in less on axial imaging, a large cord expansion and post-contrast
are associated with cardiovascular thrombotic conditions
than 10% of patients and more commonly seen in men enhancement, was compatible with the general presenta-
and cerebral infarctions The clinical relevance of PAI-1
and the diagnosis is made on the basis of clinical findings. tion of NMOSD. While vitamin B12 deficiency could be a
has been documented in several clinical and laboratory
The goal of treatment is to promptly suppress inflamma- confounding factor, radiological findings do not support
studies (1,2) Cases of Multiple Sclerosis, an inflammatory
tory exacerbations and recurrences to avoid irreversible the hypothesis, although it could enhance symptoms.
disease, may include fibrinolysis disorders in its patho-
organ damage, and immunosuppressive medications Different studies discuss the link between AQP4-IgG,
genesis.(3) The increased presence of the PAI-1 inhibitor,
are mainly indicated. Final Comments: Despite being MOG-IgG and the disease prognosis, with better outcomes
through polymorphism with 4G alleles, can destabilize the
rare, the neurological impairment in Behçet syndrome for patients who are AQP4-IgG-seronegative and MOG-
clinical picture, inducing infarctions and ischemia. Final
has high rates of morbidity and mortality, given the vast IgG-seropositive. In the present case, MOG-IgG was not
Comments: the participation of these polymorphisms may
clinical involvement of this pathology. Considering that tested, which weakened the ability to completely inter-
have contributed to the neurological conditions presented
the diagnosis of the disease is clinical, a careful anamne- pret the clinical observations. Nevertheless, a remarkable
by the patients and may be a confounding or intensifying
sis and the direct search for complications caused by this reduction of the lesions extent on MRI and a less likely
factor in the development of Multiple Sclerosis and other
disease are essential for the treatment to be established chance of relapse are seen in AQP4-seronegative cases,
diseases that course with thrombosis.
and the systemic damage to be stopped. as in this case.

Neuroimunologia
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CASE PRESENTATION OF STEROID ACUTE DISSEMINATE ENCEPHALOMYELITIS PARANEOPLASTIC NEUROMYELITIS


RESPONSIVE ENCEPHALOPATHY AFTER PFIZER VACCINE: CASE OPTICA SPECTRUM DISORDER WITH
ASSOCIATED WITH AUTOIMMUNE PRESENTATION ANTI-AQUAPORIN-4 ASSOCIATED WITH
THYROIDITIS (SREAT) SQUAMOUS CELL CARCINOMA OF THE LUNG
Wendell Silveira de Souza, Bruna Guimarães Dutra,
Flavia Costa Mourão, Ingrid Demosthenes Wanzileu, Danilo Issa Mitozo Veras, Daniel Sampaio e Souza, João Victor Lage Guerra, Henrique Amancio Ferreira,
Paulo Hermes Lima Amaral, Leonardo Rodrigues João Pedro Moreira Guilherme, João Victor Oliveira Andre Felipe Lucchi Rodrigues, Bianca Gomes
Leopoldo Menezes, Wesley Lopes Silva, Luiziane de Melo, Neyde Alegre de Souza Cavalcante, Ada Mazzoni, Davi Teixeira Urezêdo Queiroz, Rodrigo
Maria Silva Alves, Camille Albuquerque Rodrigues Virginia Alves Rodrigues, Vanise Campos Gomes Santiago Gomez, Breno Franco Silveira Fernandes
Chirano, Pablo Vinicius Silveira Feitoza Amaral, Tales de Oliveira Junior
Hospital das Clínicas da UFMG. Belo Horizonte MG,
Hospital Universitário Getúlio Vargas. Manaus AM, Universidade Federal do Amazonas. Manaus AM, Brazil
Brazil Brazil
Universidade do Estado do Amazonas. Manaus AM, João.lageguerra@hotmail.com
ingridwanzileu85@gmail.com Brazil
Fundação Hospital Adriano Jorge – Manaus – BA Case Presentation: A 61-year-old man with a past medical
Case Presentation: Patient S.C.D.S., 51 years old, female, – Brazil history of liver transplantation in 2001 due to alcoholic cir-
started in May 2020 with gait ataxia with postural insta- rhosis was admitted for untreatable hiccups and vomiting,
bility determining falls. Report of incoordination to han- wendell_sdesouza@hotmail.com which had started 14 days earlier. Initial imaging showed
dle objects, hand tremor when performing movements. a left lung apex mass. A biopsy was made and the patient
Later, difficulty in balancing oneself in an orthostatic Case Presentation: A 44-year-old male patient from was diagnosed with squamous cell lung carcinoma. One
position, even at rest. There was progressive worsening Manaus, Amazonas was admitted in our Hospital with a month after the first symptoms, the patient developed
over the subsequent month, requiring support to walk. history of upper limbs paraparesis starting on April 21, paraparesis, upper right limb paresis, complete anesthe-
Neurological examination showed cerebellar syndrome 10 days after Pfizer COVID vaccine (it was his 3rd dose; sia of the lower limbs, urinary and fecal retention. During
with ataxic gait, dysmetria, dysdiadochokinesia, Romberg the 1st and 2nd doses were Coronavac), which progres- the following 24 hours, the symptoms worsened and he
sign, Stewart-Holmes test, and moderate frequency and sively affected his lower limbs associated with stiff neck, became paraplegic. Brain and spine MRI showed a longi-
amplitude intention tremor. Complementary tests showed nausea and vomiting. He evolved with dysphagia, dysar- tudinally extensive transverse myelitis, with high T2 signal
thyroid hormones (TSH and free T4) within the normal thria, urinary retention and severe tetraparesis, looking involving C5 to T11 segments, along with high T2 signal
range but high Anti – T.P.O. and Anti – Thyroglobulin. Skull for an emergency service on may 15, where he performed within the area postrema. CSF analysis were as follows:
M.R.I. without alterations. Given the findings, the diag- a cranial CT which showed right frontal hypodensity and protein 472 mg/dl, 124 cells (81% neutrophils), with no
nosis of steroid-responsive encephalopathy associated enlargement of the right temporal horn of the lateral other relevant findings. Anti-aquaporin 4 antibodies were
with autoimmune thyroiditis (S.R.E.A.T.) was proposed. ventricle, being later referred to the neurology Intensive detected on a blood sample. The patient was not tested for
The patient started pulse therapy with methylpredniso- Care service for better investigation. CSF collection did onconeural antibodies due to unavailability. The patient
lone with excellent therapeutic response. In outpatient not show infectious processes. Neurological examination was started on high dose EV corticosteroids (methylpred-
follow-up, cerebellar syndrome improved, especially in revealed grade 2 strength tetraparesis in the proximal lower nisolone 1g for five days), without any improvement. A
gait, with the return of the ability to walk without support. limbs and plegia distally, while the upper limbs had 1- second treatment attempt with a five-day course of plasma
SRAT characteristically presents as an encephalopathy in strength proximally and 1+ distally. Spastic tetraparesis exchange therapy was also unsuccessful. During Hospital
which seizures, movement disorders, and psychosis are associated with clonus in superior and lower limbs was stay, the patient developed sepsis and was intubated. Due
common findings. Rarely, the disease is predominantly also found. Reduced protopathic touch globally. Also to respiratory muscular weakness, ventilator weaning was
expressed as cerebellar ataxia, as presented in the case bilateral vertical nystagmus and paresthesia in the left challenging. After a prolonged ICU stay, the patient died.
described. The criteria for diagnosis of this condition were hemiface, without other alterations. In view of the imag- Discussion: Paraneoplastic neuromyelitis optica is a rare
established with the elevation of anti-thyroid peroxidase ing findings in association with clinical manifestations, condition, with an estimated incidence of less than 4 per
(anti-TPO) and anti-thyroglobulin (anti-Tg) antibodies. the diagnosis of Acute Disseminated Encephalomyelitis cent of all NMOSD cases. It is more often associated with
Patients usually have an excellent response to immuno- (ADEM) was confirmed. After the condition worsened, lung and breast adenocarcinoma. However, other cancer
therapies, including steroids, as corroborated in the case due to dyspnea and hypoxemic respiratory insufficiency, types can be involved. The mean age of onset is 55 years.
we describe. Considering the atypical clinical presentation the patient was referred to the ICU where he underwent Brainstem lesions and longitudinally extensive transverse
of the predominantly cerebellar S.R.A.T., the relevance of endotracheal intubation and, to date, 7 plasmapheresis myelitis are the most common presentations, and some
the case is reinforced. sessions. Now, he is in a lethargic state, with sepsis due studies suggest cancer screening in these patients. Final
to urinary tract infection. Discussion: ADEM is a rare Comments: Although the condition is rare, it is important
disease that can be associated with other neurological for the neurologist to recognize paraneoplastic NMOSD
Neuroimunologia diseases, such as Guillain-Barré syndrome or isolated as a cause of demyelinating disease in cancer patients,
encephalitis. It is a diagnosis of exclusion and, therefore, but also as a red flag for possible occult tumors in older
imaging tests and CSF collection are essential. In the clini- patients who develop neuromyelitis optica, especially
cal aspects, several signs of encephalopathy can be found, those over the age of 55.
such as headache, ataxias, hemiparesis and cranial nerve
alterations. Treatment revolves around immunosuppres-
sion, typically with human immunoglobulin, steroids, Neuroimunologia
and, in severe cases, plasmapheresis. Final Comments:
The case presented shows a rare case of ADEM after the
3rd dose of the vaccine against COVID-19. The present
report does not aim to question the safety of vaccines,
since adverse effects of this severity are rare, but rather
to share this specific case and, in this way, foster discus-
sions about the preparation of health professionals to
deal with such a situation.

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VOUGT-KOYANAGI-HARADA DISEASE, A DIAGNOSIS OF AUTOIMMUNE CEREBELLITIS MARBURG VARIANT OF MULTIPLE


RARE ETIOLOGY OF STERILE MENINGITIS – DETECTED BY 18 F-FDG-PET/CT AFTER SCLEROSIS AFTER COVID-19
A CASE PRESENTATION SARS-COV-2 INFECTION SUCCESSFULLY TREATED WITH
BETAINTERFERON 1A – A CASE
Jane Lacerda Bahia, Rafael Pallos da Silveira, Ana João Gabriel Pacetti Capobianco, Gabriela Soares
Luisa Carvalho Santiago, Dimitria Fortes de Oliveira Correia PRESENTATION
Borges, Glaucus Fernando Vieira Nunes, Jaqueline Elizeu Alves Herbster, Júlio César Claudino dos
Gomes da Silveira, Joana Luiza Rojo, Laura Helena Pontifícia Universidade Católica de Minas Gerais.
Poços de Caldas MG, Brazil Santos, Enzo Lima Alcântara Parente, Aston Alves
Esteves Poggianella Santana, Sandra Lucia Dias, de Freitas, Euldes Mendes Júnior, Paulo Filho
Leandro Santos Franco de Aguiar FAMEMA – MARILIA – SP – Brazil
Soares Marcelino, Samuel Ramos Rabelo, Idna Lara
Hospital Metropolitano Odilon Behrens. Belo João 20_03@hotmail.com Sena de Goes
Horizonte MG, Brazil Centro Universitário Christus – Unichristus.
Case Presentation: A 57-year-old man presented with
janelbfarm@gmail.com Sars-Cov-2 infection in March 2020 with severe viral pneu- Fortaleza CE, Brazil
monia, without the need for mechanical ventilation. He elizeuherbster@gmail.com
Case Presentation: A 16 year old Venezuelan boy with no evolved after 2 weeks with gait ataxia, dysmetria, dysdia-
previous comorbidities, who was admitted at the emer- dochokinesia, vertigo with cerebellar features, cerebellar Case Presentation: A 20-year-old female patient pre-
gency room presenting headache associated with bilateral tremor and bilateral vertical nystagmus. Cranial magnetic viously healthy had a mild Covid-19 one month before
conjunctival hyperemia and reduced visual acuity, limited resonance imaging was performed, which did not show neurological symptoms. Four days before admission,
to light perception. The signs and symptoms presented any acute changes. Lumbar puncture was performed to she started with paresthesia in the inferior left limb. The
have been developing during the course of 14 days. There collect cerebrospinal fluid that showed no changes. Brain neurological examination showed complete left hemi-
were no other neurological findings. A head computed PET-CT with 18F-FDG was performed, which showed paresis and painful hypoesthesia on the left side. She
tomography scan accused a diffuse enlargement of the anomalous hyperconcentration of the radioisotope in was submitted to a brain magnetic resonance imaging
ventricular system, suggesting hydrocephalus, and there cerebellar tonsils and cerebellar vermis. Hypothesized (MRI) that evidenced hyperintense subcortical lesions
was an altered signal inside both orbits compatible with post-COVID-19 autoimmune cerebellitis. Pulse therapy on T2/FLAIR and hypointense on T1. She received a
retinal detachment. In addition, the analysis of the cere- with methylprednisolone 1g/day was performed for 5 five days course of methylprednisolone 1g with partial
brospinal fluid revealed pleocytosis, with a monocytes days, with considerable improvement in symptoms. He improvement of the symptoms and she was discharged
predominance. The patient underwent neurosurgery is discharged from the Hospital with improvement of gait to ambulatorial following. After one month, the patient
and a ventriculoperitoneal shunt had been installed. The symptoms and improvement of nystagmus. After 15 days, went back to emergency with focal seizures in the face
ophthalmologic evaluation pointed out a serous panu- improvement of dysdiadochokinesia and dysmetria, main- and worsening of left hemiparesis. The new MRI showed
veitis. The hypothesis of Vogt–Koyanagi–Harada disease taining mild vertigo. After 2 months of pulse therapy, the an increase of hyperintense T2/FLAIR lesions on the
has been raised and the patient was treated with a 5-day patient was asymptomatic from a neurological point of right frontotemporal. Due to the fast and aggressive pre-
course of methylprednisolone followed by oral gluco- view. Discussion: The diagnosis of encephalitis/cerebe- sentation with neurological deterioration and failure of
corticoid and ophthalmological follow-up. Discussion: litis using the PET-CT technique with 18-F-FDG infusion initial therapy, the possibility of Marburg’s variant of MS
Vogt–Koyanagi–Harada (VKH) disease is an important is effective in cases with normal CSF results described in was suggested. It was decided to start beta interferon 1a
and rare cause of sterile meningitis. It is a systemic auto- the literature. Reports of autoimmune cerebellitis after with 44 mcg three times a week. After one month of the
immune disorder that target pigmented tissues of the COVID-19 infection are described in the literature with new therapy, the patient presented an improvement of
body, affecting particularly the eyes, but also the skin, inconclusive results regarding the use of methylprednis- symptoms, reduction of lesions in MRI. Discussion: The
meninges and inner ear either, resulting in neurological olone or immunoglobulin. Final Comments: The report Marburg variant is a particular form of MS. It is known
symptoms such as headache, cranial nerves palsy and on the use of PET with 18-F-FDG for the diagnosis of this results in myelin sheath instability that contributes
tinnitus. The prevalence of VKH varies in different pop- autoimmune involvement of the central nervous system to the formation of demyelinating plates. Generally, the
ulations in the world, accounting for 2 to 4% of all uve- appears to be a promising alternative with increasing use. injuries show a widespread and inflammatory aspect.
itis in Brazil. The diagnosis is based on clinical findings Considering the possible relationship between Sars-Cov-2 The neuroimaging generally reveal multiple extensive
and must follow the criteria proposed by the American infection and autoimmune cerebellitis, it is necessary to lesions, involving the white matter. Our case presentation
Uveitis Society, including bilateral ocular involvement publicize the method used to the scientific community in showed an evolution of neurological injuries in a healthy
with no history of penetrating ocular trauma or surgery, order to facilitate future prospective studies. patient, except history of Covid-19, possibly a trigger of
along with neurological or integumentary findings. The this demyelinating disease. The first-line therapy is the use
goal of treatment is to suppress intraocular inflammation of glucocorticoids in high doses, and the second-line is
and prevent potential visual impairment, therefore early Neuroimunologia
intravenous immunoglobulin and plasmapheresis. After
diagnosis and treatment are essential. Final Comments: the progression of deficits, we decided to treat with beta-
It’s important sharing cases like this since it is a relatively interferon 1a, which stops that progression, improved
rare diagnosis of sterile meningitis in clinical practice, neurological symptoms and promoted regression of
hence often underdiagnosed. It is vital that physicians brain lesions. Although this medication is not commonly
pay attention on the ophthalmological manifestations indicated for this form of disease, it shows the immune
associated since they are the most frequent ones and the factor in the Marburg variant and its potential to respond
base of the diagnostic criteria. Once more, it is of utmost to immunomodulatory drugs. Final Comments: The
importance the fast diagnosis followed by and early and Marburg variant of MS requires a high level of clinical
aggressive treatment with systemic corticosteroid to suspicion and knowledge about different MS presenta-
reduce subsequent recurrences and avoid complete and tions. We present a peculiar case because a correlation
irreversible visual loss. with Covid-19 made this a possible trigger and the good
response with beta interferon 1a showed a possible drug
to consider in management of this disease.
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ADULT AUTOSOMAL LEUKODYSTROPHY: EIGHT-AND-A-HALF SYNDROME AS A MARBURG VARIANT OF MULTIPLE


RARE FAMILY CASE, WITH DYSAUTONOMIA RARE INITIAL PRESENTATION OF MULTIPLE SCLEROSIS AFTER COVID-19 SUCESSFULLY
PRECEDING ATAXIA ESCLEROSIS (MS) TREATED WITH BETAINTERFERON 1A – A
CASE PRESENTATION
Tatiane Arroyo Lopes Alves de Jesus, Amanda Leticia Erick Dupont, Arthur da Veiga Kalil Coelho, Davi
André, Arthur Coelho Moura Marinho, Marcelo Vargas Freitas Teixeira, Marcella Canato Toloi, Micael Porto Portela Lima, Idna Lara Goes de Sena,
Simplicio Carvalho, Nathalye Fernanda Pedroso Andreas Batista Schelp, Déborah Inayara Mendes Mikaio de Sousa Jucá, Julio Cesar Claudino dos
Dircksen, Lorena Fernandes Kronbauer, Damacio Tenório de Albuquerque, Sephora Sabrina Cândido Santos, Victor Oliveira Araújo, Paulo Filho Soares
Ramon Kaimen-Maciel de Almeida, Herval Ribeiro Soares Neto, Christian Marcelino, Samuel Ramos Rabelo, Fabio Antonio
Ricardo Ibsen Serra de Lima Junior, Vitor Gil Gonçalves Teixeira,
Irmandade Santa Casa de Londrina. Londrina PR,
Roberto Rocha Eisele Rocha Eisele
Brazil Hospital do Servidor Público Estadual de São Paulo.
Irmandade Santa Casa de Londrina, Neuroclinica São Paulo SP, Brazil Centro Universitário Christus, Unichristus. Fortaleza
Londrina. Londrina PR, Brazil CE, Brazil
dupont.erick@gmail.com
Universidade Federal do Piaui – Palmas – PI – Brazil
arroyotatiane@gmail.com
Faculdades Integradas do Norte de Minas – Montes
Case Presentation: A 22-year-old male with no previous
Carlos – MG – Brazil
Case Presentation: A family in which 4 of its members medical history is admitted with a new onset strabismus
Universidade Federal da Paraíba. João Pessoa PA,
were diagnosed with autosomal dominant adult leuko- and double vision, both installed three days prior to the
Brazil
dystrophy with dysautonomia (LADA) was studied in our consultation. He had no history of trauma, head or ocular
service: 1. PJ, first child, onset of symptoms with pain in pain, vision loss or systemic symptoms. Ophthalmology micaelppl1@gmail.com
the lower limbs (LL) in 2012 at age 42, evolving with loss was the first specialty evaluating the patient, concluding
of strength in the lower limbs and ataxia in 2016. He cur- no alterations in their field. Neurological examination Case Presentation: A 20-year-old female patient previ-
rently has hoarseness, urinary retention, pain and ataxia showed an ocular misalignment at primary gaze, with right ously healthy had a history of mild covid-19 one month
in the lower limbs. On MRI: hypersignal in the supraten- eye exotropia, right conjugate gaze palsy, impairment of before neurological symptoms. Four days before admission
torial, periventricular white matter, with preservation of right eye adduction and complete right facial nerve palsy. she started with paresthesia in the inferior left limb, evolv-
the “U” fibers, internal capsule, trigeminal nerve nucleus Remaining neurological assessment was preserved. Initial ing with paresis and visual blurring. Neurological exam-
and midbrain course, cerebellum, cerebellar pedun- blood investigation tests were negative for inflammatory ination showed complete left hemiparesis with pyramidal
cles with bulbar pyramid extension. 2. AF, second child, markers, serological status and rheumatological panel. signs and clonus, loss of proprioception and vibration
started in 2010 at age 40 with a complaint of generalized The Cerebrospinal fluid analysis showed 1 leukocyte/ in lower limbs, tactile and painful hypoesthesia on the
loss of strength and ataxia with progressive worsening in mm³ and 24mg/dL proteins. Magnetic resonance imaging left side, slightly scanning speech and hemiparetic gait.
ambulation and urinary retention. The current physical identified several T1-weighted hypointense images with no Differential diagnosis (DD) was with infectious, inflam-
examination shows hypoesthesia in the right hemibody, gadolinium enhancement compatible with “black-holes” matory and rheumatologic tests, all negative. Magnetic
in addition to dysmetria in the lower limbs. On magnetic in periventricular white matter, as long as T1-weighted resonance imaging (MRI) showed diffuse hyperintense
resonance imaging (MRI): symmetrical hypersignal in hyperintense lesions on cervical spinal cord and right subcortical lesions on T2/FLAIR and hypointense on T1,
white matter of the semiovais and periventricular centers, facial colliculus site at the pons, also with no gadolinium involving periventricular white matter and brainstem.
cerebellar peduncle, critical spinal path and medullary enhancement. Patient was treated for MS outbreak with Methylprednisolone was initiated, with partial improve-
bobus transition. 3. AA, third child, individual already Methylprednisolone 1g IV for five days with complete res- ment of the symptoms. After one month, the patient had
in death secondary to a car accident, siblings reported olution of diplopia and partial recovery of the eye move- focal seizures in the face and worsening of left hemipa-
that he had dysmetria in the lower limbs, was diagnosed ments and strabismus. As disease modifying therapy, the resis, with MRI revealing increase of hyperintense T2/
through imaging tests, but did not maintain medical fol- patient was put under Natalizumab schedule. MS is an FLAIR lesions on the right frontotemporal and parietal
low-up. 4. CA, mother started symptoms at 40 years old, autoimmune demyelinating disease whose lesions can lobes, pons and cerebellar regions. DD was tumecfative
with frequent falls, at 56 years old, need for a wheelchair, potentially cause irreversible disabilities when untreated. demyelination or tumor. The family didn’t agree about
intestinal and urinary dysautonomia, dying after 14 years. Among these, neuronal pathways to ocular extrinsic motil- the brain biopsy. The possibility of Marburg’s variant
FA and PJ presented. The two brothers present in cervical ity are commonly affected. Nevertheless, the presentation of MS was suggested. Interferon beta therapy was ini-
MRI, medullary thinning and hypersignal. Discussion: of eight-and-a-half syndrome is a very rare and intriguing tiated three times a week. After one month, the patient
Autosomal dominant leukodystrophy in adults with dysau- presentation where a single lesion compromises three presented improvement of symptoms and reduction of
tonomia is a rare condition to the point that its prevalence pons structures: medial longitudinal fasciculus, leading lesions in MRI, with continuous improvement in the fol-
is unknown. It is secondary to the accumulation of lam- to homolateral eye adduction deficit (called internuclear lowing months. Marburg variant is a form of MS that has
ina b1 proteins, by genetic defect 5q23 Lmnb1, leads to ophthalmoplegia); abducens nuclei, impairing homolat- a single-phase course and can lead to death in weeks to
demyelination with astrocyte dysfunction and oligoden- eral eye abduction and fascicular facial nerve fibers at months after the initial presentation, an uncommon out-
droglia preservation. The disease has a slow evolution the genu site where they arch behind abducens nerve, come in the general MS patients. The distribution of the
in about 18 years, with paraparesis, spastic tetraparesis clinically presented as “periferic” pattern facial palsy lesions can be exactly as MS, though, in this variant, they
and finally pseudobulbar palsy, leading to death. Clinic besides being a central lesion. In our case we illustrate spread to diverse brain areas, even including the brain-
in which the mentioned family presents, in addition to how the knowledge of anatomical pathways is crucial to stem. Differential diagnoses, such as tumor, infectious,
MRI. Final Comments: In the literature, only one case is clinical neurology, useful for identifying patterns which granulomatous, and vasculitic lesions must be excluded.
presented in which dysautonomia complained of ataxia, help correct diagnosis and treatments. Despite continu- Neuroimaging alterations are not specific, involving the
which, like the aforementioned family, alternates the ous improvement, MS remains a challenge to clinicians white matter of the hemispheres and brainstem. Our
classic order of symptoms. Which makes the reported and neurologists because of its extensive variety of initial report showed evolution of neurological injuries in a
case even more rare presentations, sometimes making the correct diagnosis patient with no previous conditions, except history of
a true hardship. covid-19, possibly a trigger of the disease. The patient
was treated with doses of steroids with partial response.
Neuroimunologia After the progression of deficits, treatment with betainter-
Neuroimunologia feron 1-a was proposed, regressing symptoms and brain
lesions. We present Marburg variant of MS its correlation
with covid-19 as a possible trigger and the good response
with beta-interferon.

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MIOCLONUS-OPSOCLONUS-ATAXIA DEPERSONALIZATION AS A SYMPTOM OF A CADASIL AND/OR MULTIPLE SCLEROSIS? A


SYNDROME POST COVID-19 INFECTION: PARIETO-OCCIPITAL LESION IN A PATIENT CASE OF DIFFICULT DIAGNOSIS
CASE PRESENTATION WITH RELAPSING REMITTING MULTIPLE
Laura Tietzmann Grevet, Pedro Olavo Montenegro
SCLEROSIS: A CASE PRESENTATION Stolzmann Barreto, Vitória Pimentel da Silva,
Eduardo Mariano Carvalho Silva, Luiz Felipe
Bezerra de Sousa, Ana Carolina Nascimento de Carolina Braga Moura, Luiza Aguiar Lima, Ana Luiza Taís Michele Werle, Stella Duarte Pinto, Giordani
Sousa, Arthur Costa Junger, Fábio Pereira da Silva Utrine Pimentel, Matheus Nunes Ferreirinha Leite Rodrigues dos Passos, Jefferson Becker
Júnior, Jhonata Gabriel Moura Silva, Lays Saraiva Castro, Isadora Versiane Lemos, Raimundo Marcial Escola de Medicina da Pontifícia Universidade
Rodrigues Carvalho, Lorena da Silva Viana, Nínivi Brito Neto, Priscila Santos Mageste, Mariana Soares Católica do Rio Grande do Sul. Porto Alegre RS,
Daniely Farias Santos, Albina Karolyne Diniz Freitas Tamy, Gutemberg Augusto Cruz Santos Brazil
Mariano
Universidade Federal Fluminense. Niterói RJ, Brazil Serviço de Neurologia do Hospital São Lucas – Porto
Universidade Federal do Maranhão. Imperatriz MA, Alegre – RS – Brazil
Brazil carolinabragamr@gmail.com
laura.grevet@edu.pucrs.br
jhonata.moura@discente.ufma.br Case Presentation: In june 2021, a 52-year-old female
with relapsing remitting multiple sclerosis (MS) since the Case Presentation: A 48-year-old female was referred
Case Presentation: A 65-year-old man attended the out- age of 14 with left hemiplegia, attended to an outpatient to our clinic for investigation of possible MS, which had
patient clinic referring tremors for 30 days. The condition follow-up with a history of 4 monthly episodes of temporal started 3 years earlier with an episode of dysarthria and
progressed with dizziness, malaise, vomiting, aphasia, disorientation and disorganized thinking in the past few right lower limb weakness. MRI showed focal and con-
opsoclonus and myoclonus, which were presented 9 years, after one of the MS outbreaks. Each episode begins fluent T2-hyperintense lesions in the subcortical and
days after confirmed Covid-19 infection. Denies vertigo, by waking up; lasts throughout the day; ends at bedtime. periventricular white matter as well as infratentorial
seizures and history of cerebral vascular accident (CVA). While in this situation, the patient describes that she regions and cervical and thoracic spinal cord. Since she
Neurological examination showed bilateral dysmetria, watches her body from above, also, she cannot perform lacked evidence of dissemination in time, a diagnosis
dysdiadochokinesia in left upper limb and involuntary a sequence of tasks properly, also she notices a change of high-risk clinically isolated syndrome was made and
coarse movement with chorea and athetosis, in addition in processing speed, characterizing a depersonalization she was started on glatiramer acetate. Over the next year,
to muscle contraction. The cerebral magnetic resonance disorder. The patient underwent a new brain MRI that there were new symptoms accompanied by gadolini-
revealed global atrophy and microangiopathy. Requested showed a focal T2 hypersignal in the subcortical white um-enhancing brain lesions. At this point she fulfilled
electroencephalogram (EEG), which showed normal matter of the right frontal lobe present in a previous exam the diagnostic criteria for MS and was treated with I.V.
activity with the presence of rapid eye movement arti- and a new lesion in the right parieto-occipital subcortical methylprednisolone, with improvement. She was switched
facts, in addition to laboratory tests for paraneoplasia white matter next to the posterior horn of the lateral ven- to natalizumab, which effectively controlled MS activ-
and other possible etiologies screening (CA 19.9, antigen tricle with hypersignal in T2 and FLAIR and no gadolin- ity, and five years later to fingolimod due to increasing
carcinoembryonic (ACE), ESR, PCR, occult blood test, ium enhancement that might temporarily correspond to titers of serum JCV antibodies, but later on developed
ammonia, ANA, anti-beta2-glycoprotein, homocysteine, the onset of the current symptom. Discussion: Cognitive insidious worsening of physical and cognitive disability
vitamin B12 and vitamin E dosage, folic acid, Pil, anticar- impairments and MS are often related, suggesting they are (EDSS 5.5), suggestive of secondary progressive MS, as
diolipin, ALT and AST, AP and GGT, HbsAg, anti-HCV, associated with the demyelination process. This finding well as epilepsy. She had no personal or family history of
Elisa HIV, VDRL and FTA-ABS). The results did not show is more common in patients with progressive MS. Most migraine, stroke ou psychiatric conditions. At age 62 years,
paraneoplasms or infections, thus closing the diagnosis cases report deficits in episodic memory and processing no-cost testing for genetic leukoencephalopathies became
of Kinsbourne Syndrome. A lumbar puncture was made speed, although symptoms such as verbal fluency, visu- available for our site and revealed one likely pathogenic
to evaluate the cerebrospinal fluid in addition to a high ospatial analysis, difficulty in multitasking and in word variant in NOTCH3, the gene associated with autosomal
dose methylprednisolone pulse therapy. At the return finding may occur. These declines often begin early in dominant cerebral arteriopathy with subcortical infarcts
visit, the patient showed a significant improvement in the disease, being one of the major concerns of the patients and leukoencephalopathy (CADASIL). In retrospect, her
clinical status. Discussion: The Kinsboume Syndrome or once they are diagnosed. Besides cognition, psychiatric MRI scans were assessed as compatible not only with
Opsoclonus Myoclonus Ataxia syndrome is a rare neu- symptoms are also reported in some MS phenotypes, MS but also with CADASIL, due to involvement of the
roinflammatory disorder with a variable combination of including psychosis, depersonalization. Despite the link external capsule and temporal lobes bilaterally. Genetic
opsoclonus, myoclonus, and ataxia¹. The etiology may be between the relapse and the depersonalization, it is more counseling was performed, management of vascular risk
paraneoplastic, such as for neoblastoma, parainfectious frequently seen the association between MS and cognitive factors was intensified, and she was kept on fingolimod.
(after viral or bacterial infections), toxic/metabolic or disorders. Also, neuropsychiatric disorders appeared in Discussion: CADASIL is a monogenic disease associated
idiopathic². The exact pathogenesis is not fully known, a bigger percentage associated with frontal lobe lesions. with recurrent small-vessel ischaemic strokes, migraine,
however, in recent years, the emergence of neurologi- Final Comments: Cognitive disorders and behavioral and dementia. As CADASIL often has a relapsing-remit-
cal disorders, such as Kinsbourne syndrome itself, after symptoms may be associated with multiple sclerosis out- ting presentation and widespread cerebral and brainstem
COVID-19 infection has been reported in several studies¹. breaks and represent an important factor to the patient’s hyperintense white matter lesions, misdiagnosis with MS
The syndrome is believed to be an inflammatory phe- quality of life. In this patient, the parieto-occipital lesion in can occur. However, in this case, features such as gado-
nomenon mediated by the autoimmune system, being the new MRI correlates with symptom onset, suggesting linium-enhancing and spinal cord lesions and response
secondary to the Sars-Cov 2 infection². Final Comments: a possible correlation between that region and deper- to steroids were rather suggestive of MS. We found simi-
Although it is a rare disease, the diagnosis of Kinsbourne sonalization. Although not described before, it could be lar cases in the literature, indicating that the association
syndrome by the neurologist, in the present case, was ver- attributed to the spatial recognition function of the parietal between MS and CADASIL may be more than casual.
ified from the permanence of sequelae, mainly related to lobe, prompting more studies regarding the correlation Final Comments: Our case highlights that CADASIL may
motricity and persistence of opsoclonus, as reported in of depersonalization and its topography. mimic or coexist with MS, yet the relationship between
the literature, in addition to the exclusion of occult neu- these conditions is yet to be determined. Genetic testing
roblastoma and other conditions. for leukoencephalopathies may prove useful for selected
Neuroimunologia patients with MS.

Neuroimunologia
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POST COVID-19 VACCINE-ASSOCIATED ANTI-HU SYNDROME WITH SIMULTANEOUS POST COVID-19 STEROID-RESPONSIVE
ACUTE MYELORADICULONEUROPATHY MOTOR, SENSITIVE AND AUTONOMIC ENCEPHALOPATHY ASSOCIATED WITH
RESPONSIVE TO PLASMAPHERESIS NEURONOPATHY: A CASE PRESENTATION AUTOIMMUNE THYROIDITIS (SREAT)
PRESENTING AS A NEW ONSET
José Wagner Leonel Tavares-Júnior, Pedro Braga- Sara Terrim, Beatriz Carneiro Gondim Silva, Roger
Neto Santana Araujo, Ida Fortini, Marcia Rubia Rodrigues REFRACTORY STATUS EPILEPTICUS (NORSE)
Gonçalves, Guilherme Diogo Silva, Artur Martins RESPONSIVE TO TREATMENT
Universidade Federal do Ceará. Fortaleza CE, Brazil Novaes Coutinho, Luiz Henrique Martins Castro,
José Wagner Leonel Tavares-Júnior, Pedro Braga-
wagnerleoneljr@gmail.com Mateus Mistieri Simabukuro
Neto, Manoel Alves Sobreira-Neto
Hospital das Clínicas da FMUSP, São Paulo SP,
Case Presentation: A 31-year-old male patient pre- Universidade Federal do Ceará. Fortaleza CE, Brazil
Brazil
sented with acute tetraparesis and urinary retention wagnerleoneljr@gmail.com
one day after the first dose of the Covid-19 vaccine. sterrim.sara@gmail.com
Covid-19 PCR (polymerase chain reaction) was negative,
Case Presentation: Neurological symptoms after Covid-19
and IgM (Immunoglobulin M) Covid-19 was reactive. Case Presentation: A 67 year-old male with a medical
are common, as seizures.1,2 In addition, refractory status
Electromyography showed asymmetric motor-sensory history of prostate cancer, with no evidence of active
epilepticus has also been described after Covid-19.3 This
axonal polyneuropathy. Cervical MRI (Magnetic reso- disease in the last year, started numbness in the right leg
article aims to describe a patient with steroid-responsive
nance imaging) revealed a longitudinally extensive cer- and left hand in May 2019, which progressed to paresthe-
encephalopathy associated with autoimmune thyroiditis
vical transverse myelitis. Immunoglobulin for five days sias and paresis in the four limbs (asymmetrical, worse
(SREAT) after Covid-19, presenting as a new onset refrac-
improved arms strength. Methylprednisolone for five in MSE), reporting difficulty picking up objects, writing
tory status epilepticus (NORSE), with good response ther-
days was started without improvement. He also under- and walking. In July 2020, he began to present episodes of
apy. Discussion: Our patient presented with NORSE.7
went plasmapheresis with improvement. Blood tests and seizures reported as a cephalic version, automatisms and
The most common etiologies of NORSE were ruled out,
cerebrospinal fluid were performed, excluding autoim- unmotivated laughter. Neurological examination showed
such as structural damage, vascular, infectious, toxic and
mune diseases, other infections, and neuromyelitis optica. diffuse paresthesia and areflexia with diminished vibra-
metabolic causes. Markers for autoimmune encephalitis
He returned walking unassisted after 60 days with mild tion and proprioception sensitivity in addition to sensory
were negative. However, thyroid autoantibodies raised the
hypoesthesia in his left foot and mild urinary retention. ataxia. Also, prominent and asymmetric weakness was
hypothesis of SREAT. The diagnosis of SREAT is currently a
Previous reports with similar and worse outcomes were evidenced on the left hand, with signs of muscular atro-
diagnosis of exclusion. The clinical picture usually involves
reported after the Covid-19 vaccine, after Covid-19, and phy and grade two strength. Pupils were symmetric with
encephalopathy, cognitive impairment, tremor, and other
other viral infections1,2. Our report presents one of the no photomotor response. Nerve conduction study and
symptoms (such as seizures).5 The patient in the present
earliest cases described after vaccination; however, it has electromyography showed absence of diffuse sensitive
case met criteria for this disease.8 The treatment of this
already been registered at a similar time3. Such cases responses and chronic motor commitment of left C8-T1
condition involves immunotherapy with methylpredniso-
usually occur with extended latency periods, probably roots, suggesting motor root or anterior horn lesion. Brain
lone and/or immunoglobulin.5 The relationship between
by SARS-CoV-2 antigens in the Covid-19 vaccine or its MRI was normal, and spine axial T2-weighted sequences
Covid-19 and the subsequent appearance of SRAT may
chimpanzee adenovirus adjuvant. These antigens could showed hyperintense lesions in the dorsal columns, com-
be due to the autoimmunity triggered by SARS-CoV-2, as
cause myelitis by immune mechanisms2. patible with sensory ganglionopathy. Further investigation
already described in other conditions (referencia NMDA
revealed positive serum anti-Hu antibodies. Neoplastic
covid). The presentation of SREAT with NORSE is rare
screening with chest and abdomen CT and whole-body
Neuroimunologia and, after Covid-19, it is unprecedented.9 The treatment
PET-CT were negative. The patient was treated with intra-
of NORSE, in turn, involves the control of seizures, with
venous methylprednisolone 1g/d per five days during three
anticonvulsants and anesthetic drugs.10 In addition,
consecutive months, intravenous cyclophosphamide for
immunotherapy should always be used, attempted when
three months, and five plasmapheresis sessions for each
autoimmune causes are part of the diagnostic hypothesis.8
month during two consecutive months, but showed no
Although other causes were investigated and excluded, the
improvement. In the follow-up, patient presented consti-
relationship between Covid-19 and SRAT may have been
pation and PET-CT showed important gastroparesis. New
a fortuitous finding. The present case showed improve-
neoplasia was not identified until two years after the first
ment after the attempt with the addition of ketamine to
symptoms. Discussion: Anti-Hu-syndrome occurs when
propofol and midazolam aiming at suppression burst
there is a dysfunction of the peripheral, autonomic and
with inter-surge intervals longer than 5 seconds, for 48
central nervous systems caused by an immune disorder.
hours and after immunotherapy with IVIG and MPIV.
Small cell lung cancer is found in more than 90% of cancer.
Final Comments: Our case illustrates an unprecedented
Anti-Hu syndrome may present with neuropathy, gangli-
case of NORSE by SREAT after Covid-19 with excellent
onopathy, Iimbic encephalitis, brainstem encephalopa-
therapeutic response.
thy, opsoclonus-myoclo¬nus, paraneoplastic cerebellar
degeneration or myelopathy. We report a case of anti-Hu
syndrome presenting simultaneous evidence of motor, Neuroimunologia
sensitive and autonomic neuropathy, with no neopla-
sia identified. Motor commitment was prominent with
extension of the lesion to anterior horn. Final Comments:
Anti-Hu antibodies should be tested in patients with
simultaneous resentation of motor, sensitive and auto-
nomic neuronopathy.

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ANTI-MOG DISEASE A DIFFERENTIAL ACUTE NECROTIZING ENCEPHALOPATHY: FLAIR-HYPERINTENSE LESIONS IN ANTI-


DIAGNOSIS TO BE CONSIDERED CASE PRESENTATION MOG-ASSOCIATED ENCEPHALITIS WITH
SEIZURES (FLAMES): A MISUNDERSTOOD
Tiago Abreu Tempone Paula Oliveira Pinto, Rafael Saideles, Bruna Klein
Costa, Fernanda Silveira Quadros, Helena Tairine ENTITY WITH DIAGNOSTIC LIMITATIONS
Unimed. Poços de Caldas MG, Brazil Fussiger Ana Silvia Sobreira Lima Verde, Alissa Elen Formiga
tiagoabreu.med@hotmail.com Escola de Medicina da Pontifícia Universidade Moura, Gustavo Mesquita de Oliveira, Sylvio Ricard
Católica do Rio Grande do Sul. Porto Alegre RS, Gonçalves de Souza Lima, Karla Rafaele Silva
Case Presentation: Patient G.A.M.C.R, 13-year-old, black Brazil Vasconcelos, Sarah Diogenes Alencar, Larissa
woman. In December 2018 suffered a sudden event of Escola de Medicina da Pontifícia Universidade Brenda Gonçalves Miná, Mariana Saraiva Bezerra
amaurosis in the right eye followed by mild spontaneous Católica do Rio Grande do Sul. Porto Alegre RS, Alves, Norberto Anízio Ferreira Frota, Fernanda
improvement. Visual acuity 20-800 in the right eye and Brazil Martins Maia Carvalho
20-20 in the left eye. With papilla pallor at direct fundos- Santa Casa de Misericórdia de Porto Alegre. Porto
copy. Under the hypothesis of acute inflammatory optic Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Alegre RS, Brazil
neuritis, cerebrospinal fluid collection and magnetic res- aninhaslv@icloud.com
onance of the brain were performed. Received treatment paulinhaoliveirapinto@gmail.com
with methylprednisolone 1g/day for 5 days. A extensive Case Presentation: A 73-year-old male was admitted in a
propaedeutics for etiology of optic neuritis revealed MRI Case Presentation: We describe a case of acute necrotizing tertiary Hospital due to progressive weakness in the right
without alterations with absence of monoclonal peaks or encephalopathy in a 3-year-old boy. His father has been arm and later in the right leg, over three days, along with
oligoclonal bands in cerebrospinal fluid. Magnetic res- previously diagnosed with encephalitis at age four. The dysarthria and temporospatial disorientation. Was initially
onance imaging without criteria for multiple sclerosis. patient case started with epileptic seizures time-locked approached as an acute ischemic stroke with associated
Negative anti-AQP4. Under the chance of isolated optic to several viral infections. The first seizure happened at dementia, and, after Hospital discharge, about one week
neuritis or spectrum of optic neuromyelitis, after treat- 1 year, two days after the onset of fever. He followed up from the start of the symptoms, he started to present focal
ment with methylprednisolone the visual acuity was for with several Hospitalizations for convulsive crises, always aware clonic seizures in the right side. At admission, he was
20-200 in the right eye, remaining 20-20 in the left eye. preceded by fever. He was diagnosed with febrile seizures. globally aphasic, with frontal release signs and right spastic
Another test for anti-mog and anti-AQP4 was tried at At the age of 2 years and 6 months, he was affected by hemiplegia. Brain magnetic resonance imaging (MRI) did
the moment. However, on 06-01-22, the patient seeks an COVID 19, presenting a status epilepticus concomitant not reveal ischemic stroke signs, but retrospective analy-
emergency complaining of sudden low visual acuity, pain with the infection, requiring intubation. Treated with sis of previous MRI showed left parieto-occipital cortical
on eye movement with 20-800 in the right eye again and methylprednisolone for five days. Brain MRI with ven- and bilateral insula hyperintensities on fluid attenuated
20-20 in the left eye. But complaining of loss of definition tricular dilatation, signs of cytotoxic edema and cortical inversion recovery (FLAIR) with cortical post-gadolinium
for colors in both eyes. Direct fundoscopy with bilateral necrosis. Ventriculoperitoneal shunt was performed, enhancement, with notable progression. CSF analysis did
pallor. Made new therapy with methylprednisolone 1g a and immunoglobulin was sequentially administered for not show inflammatory markers and Multiplex PCR was
day for 5 days. The patient brings the result of the new test 5 days. Sequencing of the exome was performed showing negative. Due to the symptoms progression and radiologic
test for anti-AQP4 with non-reagent value but brings the a probably pathogenic variant in the RANBP2, suggesting features, an hypothesis of FLAIR hyperintense cortical
result of the positive anti-mog test. After pulse therapy the diagnosis of Infection-Induced Acute Encephalopathy lesions in MOG associated Encephalitis with Seizures
there is improvement for color vision and visual acuity Susceptibility (IIA1/ANE1). It was then decided to per- (FLAMES) was made. The patient was then treated with
returns to the previous level with visual acuity 20-20 in form treatment with Tocilizumab, subsequently better high dose intravenous steroids and albumin plasmapher-
the left eye and 20-200 in the right eye. Star using azathi- controling symptoms, culminating in extubation 4 days esis, with an excellent response, being discharged after
oprine with gradual dose escalation reaching 150mg-day after the infusion. The patient is currently 3 years and two weeks speaking and walking without help. After one
and is maintained prednisone 10 mg day. Since then, the 9 months old, remains stable, without new infections, month, the patient is taking azathioprine and on cor-
disease is kept under control. Discussion: In this case, relapsing intermittently with drop attacks. There is no ticosteroids tapering, still able to walk independently
we discuss the importance of considering differential motor deficit, only cognitive. Discussion: The disease and speak with only a small latency of speech response.
diagnostics beyond the spectrum of optic neuromyelitis hallmark is the presence of a viral infection related to Myelin oligodendrocyte glycoprotein (MOG) antibody
in cases of severe optic neuritis. In this sense, anti-mog seizures, evidencing multiple symmetrical lesions due associated disease (MOGAD) is an inflammatory afflic-
disease has proved very relevant, especially in cases where to edema and necrosis in the thalamus, cerebellum, tion of the central nervous system (CNS), and similar
there is severe optic neuritis but with specific examination brainstem and white matter. The etiology arises from the cases were described with FLAMES (4). Clinical features
of negative anti-AQP4. Final Comments: Severe optic uncontrolled release of cytokines during a febrile illness. include seizures, headache, fever and cortical symptoms
neuritis can be highly disabling and elucidation aiming There are a variety of viroses that has been already iden- referable to the FLAMES location, and 95% reported cases
at the best treatment of the conditions involves knowing tified and related to ANE, Influenza A is the most com- at least two of these four findings (5). Although initially
the diagnostic hypotheses, considering them and well monly related disease trigger. Most cases are sporadic, described as a unilateral cortical encephalitis, bilateral
testing them. Anti-mog disease should be remembered but familial cases are associated with mutation in the cortical involvement and possible meningeal inflam-
in this context. gene RANBP2. The clinical presentation is characterized mation could indicate a broader disease spectrum. The
by three stages: prodromal (febrile viral infection), acute syndrome is highly steroid-responsive (1). FLAMES is a
(encephalopathy, on average 3 days after the beginning of rare condition that remains underdiagnosed. Although, we
Neuroimunologia the prodromal phase) and recovery. After recovery, brain still have a lot of limitations to diagnoses, considering the
lesions may resolve completely or result in atrophy, or unavailability and high prices of the tests. Recognition of
hemosiderin deposition. Final Comments: The patient this distinct clinico-radiographic syndrome may facilitate
had the classic clinical presentation, confirmed with the prompt diagnosis and treatment. Therefore, the reported
exome, despite the absence of the most frequent radio- cases suggest excellent response to adequate treatment.
logical alteration. The purpose of this report is to draw
attention to potentially serious complications in patients
with these comorbidities. Neuroimunologia

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DYSARTHRIA-CLUMSY HAND SYNDROME: NEUROLOGICAL COMPLICATIONS IN ANTI-GABA-AR POSITIVE ANTIBODY


AN ATYPICAL PRESENTATION OF MULTIPLE LYMPHOHEMATOPOIETIC LINEAGE ENCEPHALITIS AS A RECURRING
SCLEROSIS RELAPSE NEOPLASMS: REPORTS ON CRANIAL ENCEPHALITIS ASSOCIATED WITH
NERVE INFILTRATION AND ITS DIAGNOSTIC THYMOMA
Flávia Timbó Albuquerque, Alexandre Bussinger
Lopes, Caio Vinicius Barroso de Lima, Enedina Maria CHALLENGE
Rafael Gomes Costa, Guilherme Diogo Silva,
Monteiro Lobato Lara Cordeiro Magalhães, Henrique Nascimento Leandro Tavares Lucato, Nathalia Rossoni Ronchi,
Dourado, Joanna Sousa Fonsênca Santana, Gabriel Márcia Rubia Rodrigues Gonçalves, Ida Fortini, Luiz
Universidade Federal de São Paulo. São Paulo SP,
Vianna Pereira Aragão, Hugo Oliveira Polito Barreto, Henrique Martins Castro, Ricardo Nitrini, Mateus
Brazil
Victoria Faustino Silva Reis, Fernanda Souza Mistieri Simabukuro
flaviatalbuquerque@gmail.com Gracílio Silva, Ana Rafaela Soares Vale, Juliana
Hospital das Clínicas da Faculdade de Medicina da
Martini Oliveira, Daniel Rocha Paranhos
Universidade de São Paulo. São Paulo SP, Brazil
Case Presentation: A 39-years-old woman with a history
Escola Bahiana de Medicina e Saúde Pública. Hospital das Clínicas da Faculdade de Medicina da
of hypothyroidism presented in August 2020 with progres-
Salvador BA, Brazil Universidade de São Paulo. São Paulo SP, Brazil
sive slurred speech. The following day she noticed weak-
ness in her right hand and was evaluated at the emergency Hospital Geral Roberto Santos HGRS
rgc0711@gmail.com
room. She was three weeks pregnant according to obstetric laramagalhaes19.2@Bahiana.edu.br
ultrasound. Neurological examination revealed mild weak- Case Presentation: A 53-year-old otherwise healthy
ness of the right upper limb and discrete dysarthria. The Case Presentation: CASE 1: LPS, male, 46y. Presented with female presented with a six-month history of cogni-
remainder of the examination was normal. Brain magnetic pain in the lumbar region for 6 months with irradiation to tive and behavioral symptoms. Six months before, she
resonance imaging (MRI) revealed a tumefactive lesion the left lower limb and bilateral inguinal lymphadenopa- presented difficulty in planning and performing multi-
on the right corona radiata and rare foci of T2/FLAIR high thy. Laboratorial investigation showed leukocytosis with ple-step household tasks and progressive apathy. After
signal lesions in the white matter, suggesting a demye- left shift. Immunophenotyping (IPT) suggestive of chronic two months, she manifested persecutory and religious
linating disease. Cerebrospinal fluid was normal except myeloproliferative disease. During Hospitalization, devel- delirious thoughts, sexual disinhibition and irritability.
for the presence of type 2 oligoclonal bands. Due to her oped Peripheral Facial Palsy (PFP), being managed as After another two months, she presented recurring focal
pregnancy and improvement of symptoms, she did not Bell’s Palsy (BP) while CNS infiltration was investigated. onset seizures, starting with a speech disturbance and
receive any treatment initially, and she had an incomplete CASE 2: ACDS, male, 70y. Presented with melena, enter- quickly evolving to a generalized tonic-clonic episode.
recovery. Six months after delivery, the MRI was repeated orrhagia, hepatomegaly and mobile painless lymphade- Neurological exam revealed a frontal syndrome character-
and showed a new mass lesion involving the left internal nopathy. IPT suggestive of chronic lymphoproliferative B ized by apathy, social disinhibition and executive cognitive
capsule. She decided to breastfeed, and a DMD was ini- cells disease. During Hospitalization, evolved with ptosis impairment. Brain magnetic resonance imaging showed
tiated a year later. Discussion: Multiple Sclerosis (MS) is of the right eyelid, hypotropia and esophoria. Discussion: multifocal, non-enhancing, cortico subcortical T2/FLAIR
the most common demyelinating disease of the central We report the investigation of two patients diagnosed signal abnormalities predominantly on frontal and tem-
nervous system, and its typical presentations include with neoplasm of the lymphohematopoietic lineage poral lobes. Cerebrospinal fluid study demonstrated the
optic neuritis, internuclear ophthalmoplegia, and myelitis. that developed cranial nerve (CN) palsy. Case 1 reports presence of oligoclonal bands despite normal cell and
Stroke-like presentation of MS is uncommon, with a few a patient with chronic myeloid leukemia that develops protein counts. A chest computed tomography revealed
cases described in the literature. Dysarthria-clumsy hand PFP, a rare finding. Thus, BP was raised as a possible a thymoma that was surgically removed. Autoantibody
syndrome (DCHS) is typically a lacunar syndrome charac- diagnosis. Contrast-enhanced MRI of the brain showed panel confirmed the diagnosis of anti-GABA-A receptor
terized by unilateral pyramidal signs and speech distur- thickening of the 7th CN suggestive of leptomeningeal encephalitis. The patient was treated with pulse therapy
bance. The most frequently described sites of lesions are metastasis, a finding also seen in BP. Patient was trans- using methylprednisolone and plasmapheresis followed
the internal capsule, corona radiata and pons. Our patient ferred without being able to perform specific LCR analysis. by cyclophosphamide, obtaining good seizure control.
also had a tumefactive demyelinating lesion (TDL), a rare Case 2 presents a patient with lymphoma that develops Six months later the disease relapsed presenting this
form of MS relapse. Typical pathological findings of TDLs an incomplete 3rd CN palsy. Contrast-enhanced MRI of time also with hemiparesis. Rituximab was administered,
include abundant foamy macrophages containing myelin the brain showed no significant changes. CSF analysis seizure control was once more obtained but the patient
debris, reactive astrogliosis, relative axonal preservation, did not present with neoplastic cells, despite hypoglycor- remains to this day with important behavioral symptoms.
and variable perivascular and parenchymal lymphocytic rhachia and increased protein. Reports suggest that such Discussion: Anti-GABA-Ar encephalitis can be one of
inflammation. The TDL could be explained by a procoag- findings do not rule out the cranial nerve involvement. the thymoma associated paraneoplastic encephalitis.
ulant state that represents an increased innate immune Final Comments: Neurological involvement can occur Seizures, cognitive impairment, decreased level of con-
response, probably needful to compensate for reduced during the natural history of hematological neoplasms, sciousness, altered behavior and movement disorders
cell-mediated immunity inherent to the pregnant state. especially in leukemia and lymphoma. Despite several are the most frequent symptoms. Distinctive multifocal
Final Comments: There is no description in literature clinical presentations, cranial nerve impairment is the cortical-subcortical T2/FLAIR MR abnormalities provide
of MS relapse causing DCHS, but the lesion’s location most common and more easily noticeable of the extra-he- an important clue to the diagnosis, since they rarely occur
seen on MRI and the patient’s symptoms are compatible matological manifestations. Even so, its diagnosis is not in other autoimmune encephalitis. The disorder is severe
with the syndrome. This atypical case shows that MS can simple, as diagnostic confirmation requires invasive and but most patients respond to treatment. However, up to a
present in various forms. In this context, the systematic difficult-to-access complementary investigations, which third of patients may relapse and recurrent disease may
approach is fundamental to diagnosing and expanding makes the confirmation a challenge. Therefore, even occur even after surgical removal of the thymoma. Final
the knowledge about this disease. though CN infiltration by hematological neoplasms is Comments: Anti-GABA-Ar positive encephalitis should be
rare, if a CN impairment appears in a patient with positive considered in patients with rapidly progressive cognitive
neoplastic history, it is worth investigating. The investi- decline, particularly in the setting of thymoma. Relapse
Neuroimunologia may occur even after the removal of the associated tumor.
gation should include imaging and cerebrospinal fluid
study. Sometimes the diagnosis is presumptive and it is
plausible to believe that the challenge of confirming the
diagnosis is the probable cause of its low epidemiology. Neuroimunologia

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PRIMARILY PROGRESSIVE MULTIPLE AMYLOID-? RELATED ANGIITIS AS A SUSAC SYNDROME: DO WE NEED THE
SCLEROSIS WITH LATE PRESENTATION NEUROCOVID MANIFESTATION? TRIAD?
Dkaion Vilela de Jesus, Alessandra Braga Cruz Alessandra Braga Cruz Guedes de Morais, Matheus Mônica Maria Costa do Rêgo Álvares, Milena Sales
Guedes de Morais, Maria Clara Alvarenga Rodrigues, da Costa Guedes, Matheus Costa Bessa, George Pitombeira, André Borges Ferreira Gomes, Daniel
Caroline Barra Souza Santana Milhome, Raissa Nilton Nunes Mendes, Sarah Diogenes Alencar, Ana Gurgel Fernandes Távora, Gabriela Joca Martins,
Barreto Vieira Soares, Jessica de Medeiros Silvia Sobreira Lima Verde, Morgana Feitosa de Fernanda Martins Maia Carvalho, Norberto Anízio
Carpaneda, Vitor Oliveira Machado, Fernando Elias Queiroga, Pedro Rubens Araujo Carvalho, Fernanda Ferreira Frota, Glauber de Menezes Ferreira, João
Borges Martins Maia Carvalho, Norberto Anizio Ferreira Igor Dantas Landim, João Gabriel Dias Brasiliense
Frota Frota
Hospital de Urgências de Goiânia. Goiânia GO, Brazil
Hospital Gera de Fortaleza. Fortaleza CE, Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Universidade Evangélica de Goiás – Anápolis – GO Universidade de Fortaleza. Fortaleza CE, Brazil
– Brazil alebcgmorais@gmail.com
Santa Casa de Misericórdia de Goiânia. Goiânia GO, monicaamancio@gmail.com
Brazil Case Presentation: A previously independent 75-years-
Universidade de Rio Verde. Goiânia GO, Brazil old woman was admitted in April of 2021 to a neurological Case Presentation: A 35-year-old male presented with
Centro Estadual de Reabilitação e Readaptação Dr. department in the city of Fortaleza, Brazil. She presented rapid onset headache and visual disturbance in may
Henrique Santillo. Goiânia GO, Brazil a three week report of progressive speech impairment, 2022. The symptoms evolved in two days with worse in
Hospital Geral de Goiânia. Goiânia GO, Brazil difficult interaction and comprehension associated with the intensity of the headache, associated with vomiting,
right hemiparesis and sphincter incontinence.She had dizziness and imbalance. Physical examination revealed
dkaion@hotmail.com an unremarkable medical history. Upon neurological disorientation, confusion, amnesia and executive dysfunc-
examination, it was additionally observed mutism, hyper- tion. The Mini mental state examination (MMSE) score
Case Presentation: Male patient, 67 years old, without reflexia, clonus and babinski sign. On further work up, a was 14. Cerebrospinal fluid analysis showed lymphocytic
comorbidities, five years ago he started a postural imbal- magnetic resonance imaging (MRI) exhibited numerous pleocytosis, hyperproteinorrhachia and normal glucose
ance, weakness of the lower limbs and falls. Four months microbleeds on SWI surrounded by vasogenic edema on levels. Brain MRI disclosed supratentorial and infratento-
ago, he presented: mental confusion; apathy; attention T2 sequences. RT-PCR test for COVID-19 was revealed rial white matter lesions with high signal on T2-weighted
alteration; excessive sleepiness; depressed mood, dyspha- to be positive.She had been experiencing mild dyspnea imaging. Snowball lesions in the corpus callosum, lep-
gia; major dysarthria; urinary incontinence and became one week prior to first evaluation. Cerebrospinal Fluid tomeningeal and linear contrast enhancement were also
bedridden. At Hospital admission, on neurological exam- Evaluation revealed normal opening pressure, 40 RBC/ observed. Retinal angiography with fluorescein showed
ination, he was disoriented and uncooperative, dysar- mm, 2 WBC/mm, Protein level of 126 mg/dl, Glucose 80 peripheral vasculitis and areas of peripheral arterial
thric, with emotional lability, tetrasegmental hypotrophy, mg/dl. It was negative pathogens. After initial approach the occlusion in both eyes. Audiometry was normal. After
elastic hypertonia and hyperreflexia with signs of pyra- diagnosis was considered to be probably ABRA (Amyloid-? reasonable exclusion of alternative causes, the diagnosis
midal release, reduced strength, worse in lower limbs. In Related Angiitis), she was submitted to therapy with a 5 of Susac Syndrome was made. Intravenous methylpred-
the imaging exams, the patient presented characteristic day course of methylprednisolone evolving with progres- nisolone for 5 days was started followed by Prednisone
lesions of multiple sclerosis, comparing magnetic reso- sive clinical improvement. Was discharged in a few days 1mg/kg/day and Azathioprine for maintenance theraphy.
nance imaging of the brain performed in a period of one and was followed at the outpatient care. She remained After the acute treatment, the patient presented marked
year, the disease activity is visible, demonstrated by the at follow up with motor aphasia and a slight upper right cognitive improvement in the MMSE (22), semantic flu-
contrast enhancement in some the lesion areas. Multiple limb weakness with relatively independence requiring ency and clock drawing-test. Discussion: Susac syndrome
lesions were defined in the periventricular white matter, little assistance to daily routine. Discussion: Neurological is a rare pathology, believed to be caused by autoimmune
subcortical, corona radiata, in the left portion of the pons symptoms associated with COVID?19 have been described microvessels vasculitis of the brain, retina, and inner ear.
and in the right cerebral peduncle, most with a perivenular with manifold aetiologies, such as ischaemic stroke, These occlusions lead to the clinical triad: encephalopathy,
aspect (Dawson’s fingers), some with contrast enhance- hemorrhagic encephalopathy and others. The largest visual disorders and hearing deficits. The occurrence of
ment. In the cerebral spinal fluid: leukocytes: 2, proteins: published autopsy series of COVID?19 neuropathology two features of the classical triad suggests the diagnosis
100; albumin: 56.2 / gamma: 15.7 / total proteins: 118 / reported microthrombi and acute hemorrhagic infarction of incomplete susac. In a review study with 304 patients,
presence of suggestive band in the gamma region; IgG: in a significant number of patients, while another more only 13% presented the triad at the clinical onset. In
17.2 / IgM: 163; Serum protein immunofixation: IgG: 907 recent study found evidence of lymphocytic encephalitis the disease course, in up to 21 weeks, 85% presented
/ IgM: 159. No monoclonal bands were detected. Serum and meningitis. Endotheliitis of the brain and extraneural the complete triad. Final Comments: Despite being a
albumin 3.67g/dL CSF IgG index was 1.24. The investi- organs has been shown in Sars?CoV?infected patients. rare disease, a better understanding of the syndrome
gation of neoplasms was performed, and did not show Amyloid-beta related angiitis is a predominantly gran- is needed. Neurologist should be aware that basing the
lesions. The patient and the family were oriented about ulomatous angio-destructive inflammatory mediated diagnosis solely on the presence of complete triad may
the prognosis of the disease and was improved the clini- disease affecting leptomeningeal and cortical vessels not be appropriate on the clinical onset of this disease.
cal support. Multiple sclerosis (MS) is the most common characterized by meningeal lymphocytosis and abun- Once excluded alternative diagnosis and in the presence
immune-mediated inflammatory demyelinating disease dant amyloid-beta deposition within the vessel walls. of suggestive radiological features of Susac, immunossu-
of the central nervous system, the mean age of MS onset The pathophysiology of ABRA is not fully understood. pression treatment should be immediately considered
ranges from 28 to 31 years; clinically it becomes apparent One theory suggests an immunologic response toward in patients with incomplete syndrome in order to avoid
between the ages of 15 to 45. Clinical onset rarely occurs amyloid-beta, resulting in leptomeningeal and paren- further disability.
in the first years of life or after the seventh decade. In only chymal inflammation. Reports of rare cases such as this
about 5% of cases, MS is diagnosed after age 50. There one of amyloid-? related Angiitis associated with COVID
is growing concern of a more progressive form of MS in infection, may help to clarify roles of immune respon- Neuroimunologia
late-onset cases. Unlike adult-onset MS, the first presen- se,its triggers and incite further questions in the scien-
tation of the late form is usually motor dysfunction. This tific community.
case presentation exemplifies what we see in the literature,
although it is uncommon, late presentation, after 50 years
of age, occurs, and has some characteristics such as the Neuroimunologia
onset with a motor condition. It is important to emphasize
unusual presentations so that we do not fail to recognize
them and manage to treat them earlier.

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GUILLAIN-BARRÉ SYNDROME AFTER BICKERSTAFF BRAINSTEM ENCEPHALITIS A MYELIN OLIGODENDROCYTE


COVID-19 VACCINE: CASE PRESENTATION CASE PRESENTATION GLYCOPROTEIN ANTIBODY-ASSOCIATED
AND LITERATURE REVIEW DISEASE (MOGAD) AFTER RECENT COVID-19
Tiago Abreu Tempone
INFECTION AND COVID-19 VACCINATION: A
Valdecir Boeno Spenazato Júnior, Maycon Melo
Lopes, Isadora Munik Oliveira Ferreira, Gabriela de
Hospital Santa Lúcia. Poços de Caldas MG, Brazil CASE PRESENTATION
Moura Camargo, Gabriel de Moraes Mangas tiagoabreu.med@hotmail.com Hiago Antunis Silva, Patrícia Marques Mendes, Yan
Universidade do Vale do Sapucaí. Pouso Alegre MG, da Silva Raposo, Ilana Werneck Augsten, Maycon
Case Presentation: Bickerstaff brainstem encephalitis Melo Lopes
Brazil
(BBE) is a rare post-infectious neurological disease defined
Universidade Federal Fluminense. Niterói RJ, Brazil Hospital das Clínicas Samuel Libânio. Pouso Alegre
by the triad of ophthalmoplegia, ataxia and decreased con-
juniorspenazato@hotmail.com sciousness. It is considered to be a variant of Miller Fisher MG, Brazil
syndrome. Discussion: We present the case of the patient hiagoantunis@gmail.com
Case Presentation: We report the case of a male patient, T.A.O., 40 years old, female, admitted to the aforemen-
50 years old, who started an acute condition of sensory tioned nosocomium on 11-24-21 with flaccid quadriple-
Case Presentation: Female patient, 53 years old, previ-
and motor loss of progressive ascending evolution, asso- gia and sensorium lowering. Aphasia. Currently, patient
ously diabetic, hypertensive, and dyslipidemic; with a
ciated with low back pain, global areflexia, evolving with with no history of previous illness. But she had a history
clinical picture of gradual visual loss installation of central
peripheral facial palsy on the right side, 10 days after the of using oral contraceptives and smoking. Initially, under
predominance in the left eye (LE), pain at ocular mobili-
administration of the first dose of the AstraZeneca lab- suspicion of a cerebrovascular event, she was submitted
zation in the LE, and cephalea. On physical examination,
oratory vaccine against COVID-19. The patient had no to imaging tests and, due to acute respiratory failure, she
she presented visual acuity (VA) of counting fingers in the
infections or other vaccinations prior to the neurologi- was intubated and kept under intensive care. After brain
LE, presence of a relative afferent pupillary defect in the
cal condition. Clinical examination and complementary and cervical imaging exams did not show signs of vascu-
LE, papilledema in the LE on dilated eye examination, and
investigation findings, including cerebrospinal fluid (CSF) lar disease, blood tests were collected with serology for
remaining neurological examination without alterations.
and electroneuromyography (ENMG), were consistent virus as well as cerebrospinal fluid collection, this one,
Symptoms began 22 days after the first dose of the COVID-
with the diagnosis of Guillain-Barré syndrome (GBS). without testing for viral serology. After a detailed inter-
19 vaccine (AstraZeneca) and 15 days after COVID-19
GBS is an acquired acute inflammatory demyelinating view with the family members it was reported, 15 days
infection with bilateral mild-moderate lung impairment.
polyradiculoneuropathy that commonly occurs after respi- before the beginning of the events, episode of diarrhea
She was admitted to the Neurology Department, with lab-
ratory or gastrointestinal viral infections, immunizations with malaise lasting 24h. This fact reinforced the hypoth-
oratory tests presenting serologies for COVID-19 IgM and
or surgical interventions, which precede neurological esis of post-infectious encephalitis. In this intercourse
IgG Reagents, increased PCR, other general tests without
symptoms by 5 days to 4 weeks. Its etiology is not fully the patient was receiving anti-viral following treatment
alterations, infectious serologies without acute conditions,
understood, but there is evidence that it is mediated by with human immunoglobulin for 5 days. Physical exam-
negative rheumatological tests, and liquor with a normal
autoimmune mechanisms with self-tolerance suppression ination revealed. Facial diplegia, complete internal and
result. Magnetic resonance imaging (MRI) of the orbits
by molecular mimicry. The symptoms of this syndrome external ophthalmoplegia. Flaccid tetraplegia. Chart,
shows signs of optic neuritis on the left, with paramag-
are wide-ranging, present in a wide variety of ways, and which according to medical literature, can be confused
netic contrast enhancement, without extension for optic
its diagnosis is made based on clinical suspicion, asso- with brain death. In fact, electroencephalogram was
chiasm. The MRI of the brain, cervical and thoracic spine
ciated with neurophysiological confirmation and CSF performed that showed wakefulness activity, without
was performed without any relevant changes. Opted for
examination. Treatment consists of immunotherapy with pathological graphoelements. In fact, electroencepha-
methylprednisolone 1 gram/day for 5 days, and the patient
immunoglobulin or plasma exchange, supportive care logram was performed that showed wakefulness activ-
evolved with partial recovery of VA in the LE (20/200). Due
and associated clinical complications. The case presented ity, without pathological graphoelements. The results
to atypical optic neuritis, serum tests of antiaquaporin 4
and the literature review show that there is a correlation of viral serologies showed positivity for acute reaction
antibody with negative result and anti-MOG antibody
between vaccines against SARS-Cov-2 and the occurrence to cytomegalovirus. Reinforcing the diagnosis. Slowly,
with positive result were requested. Was performed a
of Guillain-Barré Syndrome. after treatment with immunoglobulin, physiotherapeu-
visual evoked potential evidencing demyelinating process
tic, nutritional and pressure wound prevention care, the
in the pre-chiasmatic visual pathways of the LE. In view
patient recovered from diplegia. Improvement of ophthal-
of the findings, a diagnosis of Myelin Oligodendrocyte
Neuroimunologia moplegia, flaccid tetraplegia. With slow improvement of
Glycoprotein Antibody-associated Disease (MOGAD)
ventilatory support. Six months after the beginning of the
was made, and subsequently, azathioprine was pre-
condition and treatment, the patient maintains tetrapa-
scribed associated with corticosteroid therapy, without
resis with improvement of aphasia, without the need for
new neurological events and with progressive improve-
ventilatory support, improvement of diplegia gradually
ment of the visual picture. Final Comments: The afore-
recovering to basic activities such as feeding and verbal
mentioned patient presented a picture of optic neuritis
communication. Final Comments: Bickerstaff brainstem
in the LE with diagnostic possibilities of post-vaccina-
encephalitis may even be confused with brain death. This
tion etiology, post-infectious COVID-19 or first event of
requires a lot of holistic care.
demyelinating disease. The visual evoked potential with
signs of demyelinating optic neuritis and the positivity
Neuroimunologia of the anti-MOG antibody contributed to the diagnosis
of MOGAD and probably the previous infection and/or
previous vaccination worked as triggers for the manifes-
tation of the autoimmune disease.

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RELAPSE OF ANTI-NMDAR ENCEPHALITIS VOGT-KOYANAGI-HARADA SYNDROME, AN PULMONARY EMBOLISM IN A PATIENT


IN COVID-19 INFECTION: A CASE IMPORTANT DIFERENCIAL DIAGNOSIS WITH MULTIPLE SCLEROSIS DURING THE
PRESENTATION TREATMENT WITH TERIFLUNOMIDE: A CASE
Victor Vitalino Elias, Leonardo Mesquita Costa, Igor
Barbosa Ferreira da Silva, Sylvio Ricard Gonçalves PRESENTATION
Luíza Alves Corazza, Kennedy Soares Linhares,
Mariana Floriano Luíza Piva, Caroline Suemi de Souza Lima, Karla Rafaele Silva Vasconcelos, Thiago de Souza Rosa, Giovana Barros E Silva
Ogusuku, Isabel Lopes de Oliveira, João Fellipe Matheus Andrighetti Rossi, Lara Albuquerque de Ribeiro, Lucas de Melo Teixeira Branco, Thomas
Santos Tatsch, Julian Letícia de Freitas, Paula Brito, Karoline Ferreira Mororó Menezes, Lucas Vieira de Paula, Vítor Guimarães Corrêa, Alfredo
Camila Alves Pereira de Assis Matos, Sonia Maria Silvestre Mendes, Fernanda Martins Maia Carvalho Damasceno
Dozzi Brucki, Maria Sheila Guimarães Rocha
Hospital Geral de Fortaleza HGF Universidade Estadual de Campinas. Campinas SP,
Hospital Santa Marcelina. São Paulo SP, Brazil Universidade de Fortaleza. Fortaleza CE, Brazil Brazil
Hospital Gera de Fortaleza HGF
luucorazza@gmail.com thiago_rosa94@hotmail.com
victorel2@icloud.com
Case Presentation: A 19-year-old male presented with Case Presentation: A 44-year-old woman, former smoker,
visual and auditory hallucinations, insomnia, confusion, Case Presentation: Female, 44 years old attended at was previously diagnosed with relapsing-remitting mul-
and mood oscillations, as well as three episodes of fever Neurology service with 15 days of bitemporal, throb- tiple sclerosis (RRMS) and had been receiving interferon
in the week prior. A day before Hospital entry, he had four bing headache, intensity 8/10, associated with nausea, beta-1a over the past five years, with no signs of disease
episodes of stereotyped movements on the left side of his vomiting, hearing loss (HL), low visual acuity (VA) and activity since the beginning of treatment. Due to the
body, as well as sialorrhea, sweating and visual hallucina- pain on eye movement in the left eye (LE). After 7 days, development of flu-like symptoms and poor adherence
tions, lasting 9 minutes. At examination, he was afebrile, she evolved with low VA in the right eye (RE), with loss to the medication, she was switched to teriflunomide
agitated, disoriented, without focal deficits or meningeal of color saturation and presence of central stochoma. (TFN). After one month of treatment with TFN, the patient
signs. He had been diagnosed with anti-NMDAR enceph- She reported a history of previous, mild, perimenstrual reported a new-onset right-sided lumbar pain and dys-
alitis in our service (confirmed by serum antibody dosage) headache and no ophthalmologic complaints. Denied pnea. At the occasion, chest CT angiography showed
in 2014, when he exhibited severe agitation, autonomic other symptoms, trauma, use of medications, allergies or signs of embolism in the inferior segmental pulmonary
signs, chorea, and status epilepticus, having undergone Hospitalizations. On examination: preserved eye move- arteries, as well as pulmonary infarction in the right lower
treatment with two courses of methylprednisolone 1g, one ments. VA of the RE: counting fingers (CF) 50 cm. VA of lobe. The patient was, then, started on rivaroxaban and
course of immunoglobulin 1g, followed by 1g of cyclophos- LE: CF 30 cm. Reduced photomotor reflex bilaterally, and presented significant improvement of the symptoms
phamide, having remitted completely of the symptoms at biomicroscopy with thin keratic precipitates and posterior over the following weeks. TFN was discontinued and the
discharge. He was on carbamazepine 200mg 8/8h from uveitis (2+/4). Bilateral optic disc edema, with vascular patient maintained the initial Expanded Disability Status
2014 to 2020, having suspended the medication himself, tortuosity and bilateral serous retinal detachment. Further Scale (EDSS) of 2.5. Echocardiogram showed no signs
with no epileptic events until the day prior to admission. investigation showed: lumbar puncture with opening of pulmonary hypertension. Discussion: TFN is an oral
He underwent diagnostic testing with unremarkable head pressure 23 cmH2O (and 1 leukocyte, non-reactive VDRL, immunomodulator and a disease modifying therapy for
CT, MRI, and lumbar puncture. His SARS-CoV-2 PCR test total proteins 25); Normal magnetic resonance imaging patients with RRMS. Although liver toxicity is the most fre-
was positive, and sequential EEGs showed diffuse slowing, (MRI); Mixed HL audiometry and orbital magnetic res- quent concern during the treatment with this medication,
without epileptic activity. Considering an anti-NMDAR onance imaging with signs of chorioretinitis. In addition scarce reports of pulmonary embolism (PE) are present
encephalitis relapse triggered by COVID-19 infection, he to negative serological tests and negative antiaquaporin 4 in the literature. Thrombotic and thromboembolic events
was treated with two courses of methylprednisolone 1g research. Discussion: Vogt-Koyanagi-Harada Syndrome have been described in clinical trials evaluating TFN for
for five days, two days of cyclophosphamide (1g/d), and (VKHS) is a systemic autoimmune disease that mainly RRMS, with the group receiving TFN having a slightly,
one course of immunoglobulin (0,4mg/kg/d) for five days affects melanocyte-rich structures such as the retina, but not significantly higher, incidence compared to the
and kept on 30mg/d of prednisolone after discharge, with inner ear and central nervous system. It contains 4 distinct placebo group (1.7% vs. 1%). Here, we report the case of
progressive tapering. At the time of discharge, the patient clinical stages: prodromal, uveitic, chronic and recurrent. a patient that developed PE approximately one month
had mild executive dysfunction and echolalia, and was The previously healthy patient sought care due to unilat- after the beginning of treatment with TFN. Despite the
oriented to return for 6 monthly courses of cyclophos- eral eye pain, which developed bilaterally after 7 days, non-significant higher incidence, given the potential
phamide. Discussion: COVID-19 has been described as associated with other ocular symptoms and HL. MRI rule association between the use of TFN and development of
trigger to autoimmune or autoinflammatory conditions; out tumors or signs of increased intracranial pressure PE, clinicians should be aware of this serious condition in
anti-NMDAR encephalitis is a multistage acute autoim- and demyelinating diseases. Addicionaly, acute worsen the follow-up of this set of patients. Final Comments: The
mune disease, progressing from psychiatric symptoms of HL, along with serous retinal detachments bilaterally case presented demonstrates a rare association between
to memory disturbances, dyskinesias, seizures, and cata- and posterior uveitis. And a negative viral serology and the treatment of RRMS with teriflunomide and the devel-
tonia. Several cases of anti-NMDAR encephalitis associ- anti-aquaporin 4 narrows criteria for VKHS. Treatment opment of pulmonary embolism. Although liver toxicity is
ated to the infection by the novel coronavirus have been with 5 days of 1g methylprednisolone intravenous was the most frequent concern, scarce reports of PE are present
reported, many confirmed by SARS-CoV2 PCR and anti- made with significant outcome, showed by improve- in the literature and should be considered as a differen-
NMDAR dosage in CSF/Serum. Given our patient response ment of VA and retinal detachments at OCT, along with tial diagnosis when evaluating a patient with new-onset
to treatment and unremarkable laboratory and image, no more headache episodes. Final Comments: As a rare respiratory symptoms during the treatment with TFN.
we believe this might be the case. Final Comments: The syndrome, the diagnosis of VKHS requires a complete
coronavirus pandemic gave rise to neurological manifes- anamnesis and a good physical examination, associated
tations, including triggering of Anti-NMDAR encephalitis with complementary tests, such as OCT and serological Neuroimunologia
or their relapses, which should be considered in the dif- tests, to aid diagnosis.
ferential diagnosis of patients in this context.
Neuroimunologia
Neuroimunologia

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NEUROMYELITIS OPTICA SPECTRUM CASE PRESENTATION BELLY-DANCER CENTRAL NERVOUS SYSTEM INVOLVEMENT
DISORDER (NMOSD) ASSOCIATED WITH SYNDROME IN A PATIENT WITH IN SJOGREN’S SYNDROME: A DIAGNOSTIC
PRIMARY ANTIPHOSPHOLIPID SYNDROME: NEUROMYELITIS OPTICA SPECTRUM CHALLENGE
A CASE PRESENTATION DISORDER
Larissa Brenda Gonçalves Miná, Karla Rafaele Silva
Jonata Ribeiro de Sousa, Marcílio José de Oliveira Felipe Teijeiro Cabral, Annelise Akemi Higa Lee, Vasconcelos, Karoline Ferreira Mororó Menezes,
Filho, Rodrigo André de Souza Araújo, Maria Júnia Bruno Cassis Antunes Rodrigues, Marcelo Tognato Álissa Elen Formiga Moura, Sylvio Ricard Gonçalves
Lira e Silva, Clauham Williams Soares dos Santos, Ximenes, Luiza Helena Chuque Medina, Rafael de Souza Lima, Fernanda Martins Maia Carvalho,
Aléx de Novaes Batista, Fabíola Lys de Medeiros, Paterno Castello, Emerson Gisoldi, Maria Fernandes Matheus Costa Bessa, Norberto Anízio Ferreira
João Eudes Magalhães Mendes Frota, Glauber de Meneze Ferreira, João Igor Landim

Universidade de Pernambuco. Recife PE, Brazil Hospital Santa Casa de Misericórdia de São Paulo, Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Hospital Universitário Oswaldo Cruz da São Paulo SP, Brazil
laribgm@gmail.com
Universidade de Pernambuco. Recife PE, Brazil
ftcabral@gmail.com
jsousastmiec@gmail.com Case Presentation: A 26-year-old woman, previously
Case Presentation: Female, 46 years old, diagnosed with healthy, started in april 2021 with intermittent pulsatile
Case Presentation: A previously healthy woman aged Neuromyelitis Optica Spectrum Disorder (AQP4 positive) headache, which months later was associated with pro-
25 years reported that one and a half years ago, left-sided in 2010, with episodes of inflammation characterized by gressive sensorineural hearing loss on the right. after 4
paresthesia up to her breast evolving to the right side in optic neuritis, left hemiparesis and hemiparesthesia. She months, she presented episodes of rotational vertigo,
a few days, followed by painful sensations in both legs, was treated with methotrexate, azathioprine and, after a with subacute onset, ataxia and paresthesia in the left
urinary retention, and bilateral weakness limiting her to new attack, rituximab was initiated until now. The last epi- foot and right hand, when she was admitted for inves-
walk. She was diagnosed with a deep vein thrombosis sode was in July 2020, characterized by parestesia below T8 tigation. magnetic resonance imaging (mri) of the brain
in the left leg and received oral anticoagulation for six vertebral level. In January 2021, she reported abdominal was performed, which revealed multifocal lesions with
months. She was able to walk after 30 days without treat- involuntary movements, radiating to the dorsal region at post-contrast enhancement, affecting the basal nucleus,
ment. One month later, she presented a sudden visual the thoracolumbar transition. It was interpreted as bilat- subcortical white matter, cerebellum and pericerebel-
loss in the right eye with progressive improvement after eral internal and external oblique muscle contraction. On lar leptomeninges. she had spontaneous improvement
15 days. After five months, a mild episode of paresthesia electroneuromyography, spontaneous discharges of action of symptoms, being discharged for follow-up investi-
and weakness in both legs, with spontaneous improved potentials of arrhythmic activity and variable frequency gation as an outpatient. however, after 02 months, she
after a few days. On neurologic exam during a new severe was found, suggestive of myokymia at rest in the rectus evolved with pain in the topography of the right parotid
episode revealed clinical signs of acute transverse tho- abdominis muscles and more markedly in the left oblique. gland, progressive low visual acuity on the right (acuity:
racic myelitis with no muscle strength in both legs and Considering the diagnostic hypothesis of Belly-dancer 20/200), with pain and no tears on the same eye. she
anesthesia up to T4 spinal level. Intermittent bladder Syndrome, levetiracetam 250mg 12/12h was started, with was hospitalized again, performing a new brain mri,
catheterization was required (high urinary residue). no satisfactory response either when associated with car- which showed regression of the previous lesions with-
Cranial nerves were normal, including visual function. bamazepine 200mg 12/12h and gabapentin 300mg 8/8h. out contrast enhancement. a tear gland biopsy revealed
Spinal cord images showed signs of myelitis affecting Opted for the application of botulinum toxin in the left a typical pattern of sjögren’s syndrome. she started pulse
C7-T1 and T8-T9 levels with slight contrast enhancement. rectus abdominis and left external oblique with partial therapy with methylprednisolone 1 g/day for 5 days and
The cerebrospinal fluid analysis showed mildly elevated improvement of movements. Discussion: Belly-Dancer the patient regressed from the visual deficit. Discussion:
cell count and protein level with no signs of infections. Syndrome or diaphragmatic flutter is a rare condition Sjögren’s syndrome (ss) is an autoimmune disease that
Oligoclonal bands were not done. There was a positive that involves repetitive and involuntary movements of affects the exocrine glands and, when primary, is asso-
aquaporin-4 antibodies test (1: 80). Oral anticoagulation the abdominal wall muscles. The probable etiologies are ciated with extraglandular manifestations. neurological
was indicated again due to lupus anticoagulant positivity. drug (levodopa, biperiden, quetiapine), postoperative impairment occurs in about 25% of cases, with periph-
Visual evoked potential showed signs of visual pathway abdominal surgery, encephalitis, stroke, osmotic demy- eral manifestations being well documented in up to 20%
dysfunction in both eyes. She received five days of intra- elination, metabolic changes and trauma. Such changes of patients with ss, unlike central alterations, which are
venous methylprednisolone followed by seven sections lead to abnormal excitation of the phrenic nerve, via the rarely described. central involvement usually precedes
of plasmapheresis. Muscle strength improved to grade central nervous system or along the nerve. Another pro- the classic symptoms of sicca syndrome, in addition to
3 in both legs, and she recovered truncal and bladder posed mechanism is irritation of the diaphragm muscle the fact that antibodies (anti-ro/anti-la) are not altered
control. She is currently receiving initial doses of ritux- itself. Diagnosis can be made with ultrasound, thoracic in most cases at the beginning of the disease and the
imab. Discussion: Recurrent transverse myelitis, optic videofluoroscopy, or electromyography. Supposed treat- central symptoms can manifest in a relapsing-remitting
neuropathy, and positive aquaporin-4 antibody indicate ment includes phenytoin, carbamazepine, clonazepam, manner with asymptomatic periods, delaying diagnosis
NMOSD in this patient. Previous deep venous thrombosis fluoxetine, gabapentin and valproic acid. The use of botu- and treatment. Final Comments: The case demonstrates
in association with a lupus anticoagulant are features of linum toxin is one of the most recent therapies. In general, that despite the predominantly central pattern of involve-
the antiphospholipid syndrome (APS). The association treatment attempts have been largely ineffective. Final ment, without symptoms and without serological markers
between NMOSD and primary APS is rare, although Comments: The present case of Belly-Dancer Syndrome of ss, this patient should be periodically reassessed for the
autoantibodies related to APS had been often reported in in a patient with Neuromyelitis Optica Spectrum Disorder, possibility of being ss, due to its diversity of presentations
patients with NMOSD. Pathophysiological mechanisms may be associated with the last episode inflammation of and special evolutionary characteristics, requiring time
are complex, and both diseases may overlap, affecting the disease. The syndrome is little known and that more and high level of suspicion to close the diagnosis and
the nervous system. Cooccurrence of both conditions descriptions of cases and treatments are needed to pro- direct the treatment in time to reduce morbidities and
increases morbidity and unfavorable outcomes. Final vide a better evaluation of treatment. control the disease.
Comments: NMOSD is a severe disease, and comorbidity
with rheumatological diseases may occur and worsen the
prognosis. Further studies are necessary to understand Neuroimunologia Neuroimunologia
this relationship.

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54-YEAR-OLD MALE DIAGNOSED NEUROLOGICAL SYMPTOMS AS INITIAL A NEUROMYELITIS OPTICA CASE FOLLOWED
WITH ACUTE DISSEMINATED MANIFESTATION IN A PATIENT WITH BEHÇET FIRST-DOSE ASTRAZENECA COVID-19
ENCEPHALOMYELITIS (ADEM) AFTER SYNDROME VACCINATION
RECEIVING A DOSE OF ASTRAZENECA
Hendrick Henrique Fernandes Gramasco, Yasmim Mayara Apolinário Januzzi, Yasmin Jose Nadime,
COVID-19 VACCINE Nadime José Frigo, Ana Flávia Andrade Lemos, Bruno Rodrigues de Souza, Danielle Patrícia Borges,
Mariana Michiles Santos Ramos, Liandra Rayanne Mayara Apolinario Januzzi, Danielle Patricia Borges Hendrick Gramasco, Fernando Coronetti
de Sousa Barbosa, Lívio Leite Barros, Pedro Heder Margato, Fernando Coronetti Gomes da Rocha
UNESP-FMB – Botucatu – SP – Brazil
Oliveira Jr, Jose Daniel Vieira Castro, Samuel Ranieri Faculdade de Medicina de Botucatu – UNESP –
Oliveira Veras, Rafaela Feitosa Aguiar, Paulo Ribeiro Botucatu – SP – Brazil mayjanuzzi@gmail.com
Nóbrega
gramasco.hendrick@gmail.com Case Presentation: R.S, female, 64 years old, with systemic
Hospital Universitário Walter Cantidio. Fortaleza arterial hypertension, diabetes mellitus, hypothyroidism
CE, Brazil Case Presentation: Male patient, 44 years old, no comor- and smoker. First dose of Astrazeneca vaccine performed
Hospital Regional do Sertão Central. Fortaleza CE, bidities. He started dysphagia for 3 months and, then, on 05/05/2021. On 05/23/2021, she presented with bladder
Brazil complete right paresis of III cranial nerve, dysarthria retention associated with paraparesis and hypoesthesia of
marianamichiles116@gmail.com and bilateral dysmetria with gradual worsening. On the lower limbs. On 05/28/2021 was Hospitalized with a
admission, the presented foud were described, in addi- hypothesis of Transverse Myelitis, performed pulse ther-
Case Presentation: 54-year-old male farmer, from north- tion with mouth ulcers refractory of oral treatment. An apy with Methylprednisolone 1000mg for 5 days. Data:
east Brazil, previously healthy, presented with headache investigation was carried out with CSF suggestive of an Oligoclonal Banding (presence of identical IgG bands
and non-measured fever 2 days before neurological symp- inflammatory process, negative serology and neuroim- in CSF and serum), CSF (Cell 281 [98% lymphocytes],pt
toms. patient received a dose of astrazeneca vaccine aging with hypersignal on T2/FLAIR in the brainstem 94, glyc 59), negative anti aquaporin, non-reactive ADA,
against sars-cov-2, and on the following day, he presented in the rostral region of the medulla, midbrain and left serology negative (syphilis;toxoplasmosis;varicella zos-
with worsening of headache and left-sided weakness. thalamus, besides positive pathergy test and biopsies of ter;herpes simplex and CMV). On 07/2021 performed MRI
hours later, confusion, altered level of consciousness mucocutaneous and genital lesions with positive findings. of the brain and total spine, which showed longitudinally
and seizures. magnetic resonance imaging (mri) revealed Thus, given the central, chronic and progressive neuro- extensive myelitis without gadolinium enhancement, with-
white-matter t2/flair hyperintense plaques and diffusion logical condition, with involvement of the brainstem and out evidence of involvement of optic nerves, CSF (normal
weighted (dwi) alterations on right occipital, temporal, important sequelae, treatment with methylprednisolone range) and serology (without changes). Hospital discharge
frontal and parietal lobes, as well as basal ganglia. on a 1000 mg/day for 3 days was chosen, followed by main- with neurological examination presenting no visual defi-
second mri study 3 days later, lesions progressed in size tenance with azathioprine at a dose of 2 mg/kg/day. The cits and paraparesis and hypoesthesia with T10 level. On
and new lesions were seen on the left side. electroenceph- patient evolved with stagnation of sequelae and did not 10/2021 she presented with optic neuritis in the left eye
alogram displayed focal slowing on both right and left present new neurological manifestations, remaining which corroborates the diagnosis of Neuromyelitis optica.
frontal and temporal lobes, focal epileptiform discharges with cerebellar symptoms and persistence of paresis Discussion: Neuromyelitis optica (NMO) and neuromy-
on right frontal lobe and disruption of base activity. during of the III cranial nerve. Discussion: Behçet’s syndrome elitis optica spectrum disorders (NMOSD) are inflamma-
the next days, patient developed acute kidney injury. is a rare disease whose main characteristics are mouth tory disorders of the central nervous system characterized
he also needed ventilatory support and was transferred ulcers and systemic manifestations that are mostly due by severe immune-mediated demyelination associated
to an intensive care unit. he was submitted to therapy to vasculitis. Final Comments: Although rare, Behçet’s with predominant axonal injury of the optic nerves and
with antibiotics for bacterial meningitis and acyclovir, as syndrome should be included in the differential diagnosis spinal cord (may arise in different chronologies). Final
well as methylprednisolone in high doses. a progressive of patients with progressive cerebellar syndrome, asso- Comments: We found in the literature cases descrip-
improvement was seen on level of consciousness. there ciated with the absence of demyelinating or neoplastic tions of patients who developed transverse myelitis (TM)
was parcial recovery of cognition and motor symptoms lesions, with negative infectious screening and CSF sug- after receiving the Oxford/AstraZeneca vaccine (contains
patient no longer needed renal replacement therapy. he gestive of an inflammatory process. Active research for chimpanzee adenovirus antigen as an adjuvant) and only
continues to receive anti-epilepsy medications with com- dermatological changes in the patient’s clinical history four cases of NMO following COVID-19 vaccination (of a
plete control of seizures. after treatment, cerebral spinal and physical examination should be carried out, as the different type than AstraZeneca). Immunization can lead
fluid (csf ) analysis showed cell count 1, protein 59.1 and delay in treatment can lead to the irreversibility of neu- TM through different mechanisms such as a cross-reaction
glucose 98. viral and bacterial panel was negative for all rological sequelae. between foreign antigens and self-antigens, overactivation
pathogens on essay. pcr for sars-cov-2 on csf came back of antigen presenting cells and the subsequent autoim-
negative. Discussion: This patient was diagnosed with mune response, and polyclonal or bystander B cell activa-
Neuroimunologia tion that can lead to cytokine synthesis and activation of
adem after receiving a dose of astrazeneca covid-19 vac-
cine. this case figures among rare possible side effects of a autoreactive T cells. In our case it could be hypothesized
recently released vaccine. however, further observation is that the cytokine storm, leads to cell lysis and release of
required to establish causality, since this patient reported AQP4 molecules and might trigger humoral response in
prodromic symptoms even before receiving the dose. other individuals with genetic susceptibility to autoimmune
demyelinating cns diseases, such as multiple sclerosis, dis- eases. The role of adjuvants as contributing factors to
infectious aetiologies, vasculitis, cerebral venous throm- the inflammatory and immune adverse vaccine reaction
bosis and posterior reversible encephalopathy syndrome should be emphasized and studied.
should be considered in the differential diagnosis for this
case. Final Comments: Hopefully, this case presentation
Neuroimunologia
will help scientific community in face of urgent and recent
findings concerning covid-19 pandemics.

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MOG ANTIBODY-ASSOCIATED DISEASE POST COVID-19 VACCINATION RELAPSING ANTI-NMDA RECEPTOR


(MOGAD) AFTER VACCINATION WITH AD26. NEUROMYELITIS OPTICA SPECTRUM ENCEPHALITIS: CASE PRESENTATION
COV2.S DISORDER: CASE PRESENTATION
Heitor Caetano Santos, Paulo Santiago Brito, Gabriel
Kézia de Souza Pinheiro, Gustav Barbosa Falcão, Rafael Saideles, Paula Oliveira Pinto, Luana Miler Carvalho Oliveira, Armando Diógenes Diógenes,
Ryann Pancieri Paseto, Daniel Escobar Bueno Ghani, Andressa Gomes Niederauer Joanisson Gomes Diniz, Kaliny Oliveira Peixoto,
Peixoto, Bruno Batitucci Castrillo Maria Souza Silva
Escola de Medicina da Pontifícia Universidade
Universidade Federal do Espírito Santo – Vitória – Católica do Rio Grande do Sul. Porto Alegre RS, UFRN – Natal – RN – Brazil
ES – Brazil Brazil
heitorcaetano.10.hc@gmail.com
USP – Ribeirão Preto. Ribeirão Preto SP, Brazil
kezia.pinheiro@edu.ufes.br
paulinhaoliveirapinto@gmail.com Case Presentation: A previously healthy 16-year-old
Case Presentation: A previously healthy 37-year-old female patient presented with an episode of tonic seizures.
woman presented to the Hospital with a 7-day history Case Presentation: A 40 years old woman presented to After 05 days, she evolved with syncope. One month later,
of progressive worsening eye pain, headache, blurry the emergency service with left-sided hemiparesis and she had seizures characterized by ocular supraversion,
vision of the left eye, urinary retention, numbness in both bilateral numbness in arms and legs progressive over four followed by loss of consciousness, limb hypertonia, and
hands, weakness in both legs, bilateral Babinski sign and days. The patient didn’t have history of recent infection dis- drooling. She progressed with behavioral changes, visual
Lhermitte sign. She had received one dose of the Ad26. eases, she received the first dose of CORONAVAC vaccine hallucinations, gait and language alterations. In addition,
COV2.S vaccine against SARS-CoV-2 13 days before the 12 days before the beginning of symptoms. Previously, this she had psychomotor agitation, urinary and fecal incon-
onset of symptoms. Laboratory findings included a positive patient had been diagnosed with Neuromyelitis Optica tinence, and oromandibular dyskinesias. Cerebrospinal
MOG-Ab, negative AQP4-Ab, an elevated CSF leukocyte Spectrum Disorders (NMOSD) after episodes of unilateral fluid analysis showed pleocytosis. Brain MRI was normal.
cell counts and protein levels, and a positive antinuclear optic neuritis, in 2012, and a longitudinal extensive trans- Tomographic screening of neoplasms was negative. The
antibody. Other results were non-significant. A magnetic verse myelitis, in 2013. At that time, she had a negative electroencephalogram was suggestive of nonspecific
resonance imaging (MRI) of the brain and the spine anti-aquaporin-4 (anti-AQP4). Since the last attack she encephalopathy. In view of the subacute onset of psy-
revealed multiple hyperintense lesions on the cerebral had been using Azathioprine for recurrence prevention. chiatric symptoms, associated with new focal neurolog-
cortex, basal ganglia, and from the C5-level to the conus In the current Hospitalization, MRI showed a diffuse thin- ical findings, the presence of epileptic seizures and CSF
medullaris in FLAIR and T2-weighted images, with no ning of the right optic pathway and, in cervical spinal cord, pleocytosis, the patient fulfilled diagnostic criteria for
post-contrast enhancement. The patient received high- longitudinally extensive was identified at levels from C1 autoimmune encephalitis. She performed an immuno-
dose IV steroids, 5 sessions of plasma exchange therapy, to C5, associated with an area of contrast enhancement logical panel on CSF, which confirmed the diagnosis of
and oral steroids. She had a complete remission of the at C3 and C4, suggestive of an active inflammatory/demy- anti-NMDA receptor encephalitis. During Hospitalization,
visual symptoms after the treatment, and was discharged elinating lesion. A new longitudinally extensive cervical she underwent pulse therapy with immunoglobulin and
in need of a walking aid, with an EDSS of 6,5, and in use myelitis was then confirmed. Treatment was performed methylprednisolone, showing a significant improve-
of monthly intravenous immunoglobulin therapy (IVIg). with methylprednisolone and plasmapheresis. The patient ment in her condition. After 4 years, she evolved with
Eleven months after the episode, the patient was in a good was discharged from Hospital in good general condition. disease recurrence, characterized by agitation, aggres-
general state, with only a mild urinary retention. Her In a recent outpatient visit, the patient had complete sion, disorientation, behavioral alteration, visual hal-
EDSS was 3,0. A new MRI showed an almost complete remission of this deficits. In this consultation, azathioprine lucinations, and generalized tonic-clonic seizures. She
resolution of the previous lesions, and MOG-Ab dosage was replaced by rituximab due to therapeutic failure. This underwent pulse therapy with methylprednisolone and
was negative. She had received IVIg for 6 months and now case presentation highlights the temporal relationship immunoglobulin again, showing complete remission.
was initiating Rituximab. Cases of Myelin oligodendro- between the realization of the vaccine for SARS-Cov2 and Discussion: Anti-NMDA autoimmune encephalitis is
cyte glycoprotein (MOG) antibody-associated disorder an outbreak of NMOSD, aiming at the discussion of pos- an inflammatory condition of the brain triggered by the
(MOGAD) developed after COVID-19 vaccination have sible causality between the vaccine and the occurrence production of antibodies against the NMDA receptor on
been described. Although molecular mimicry between of relapses in patients with autoimmune diseases. SARS- the neuronal cell surface. It predominantly affects young
SARS-CoV-2 spike proteins and MOG is unclear, analyses CoV-2 infection has been associated with the development women. The initial symptom is usually neuropsychiatric,
of the cross-reactions between host and microbial pro- of autoimmune processes. Molecular mimicry has been followed by cognitive dysfunction, movement disorders,
teins show a potential correlation between CNS inflam- suggested as a potential mechanism for these associa- decreased level of consciousness, autonomic dysfunction,
mation and demyelinating diseases. In the reported case, tions. Some studies showed that antibodies against the and hypoventilation. Epileptic seizures tend to occur at
the patient developed MOGAD 13 days after receiving an spike protein S1 of SARS-CoV-2 had high affinity against any time during the illness. MRI is altered in about a third
adenovirus vector-based vaccine against SARS-CoV-2, specific human tissue proteins. As this is the same viral of patients. The CSF is altered in about 80% of cases. EEG
which is considered sufficient time for the creation of protein that the vaccine mRNA codes for, it is plausible reveals abnormal findings in around 90% of patients.
autoantibodies. MOG-Ab-associated disease is related that these vaccines may unmask or flare-up autoimmune Although it is a disease better known as monophasic,
to better outcomes than AQP4-Ab-associated disease, diseases in predisposed patients. Whether there exists a some patients can evolve with recurrence of symptoms.
although MOGAD has a higher relapse rate of optic neu- causal relationship between COVID-19 vaccination and Final Comments: The present report aims to describe a
ritis. Patients who remain seropositive after treatment the flare-up of autoimmune diseases it remains to be case of relapsing anti-NMDA encephalitis, characterizing
seem to present more relapses. Therefore, a prospective determined. Only long-term follow-up of large cohorts an atypical evolution of the disease.
follow-up of MOG-Ab titres is important for the prognosis of patients receiving the vaccine will answer this ques-
evaluation. This case presents a patient with MOGAD post tion. Until then, healthcare providers are encouraged to
Ad26.COV2.S vaccination with an unusual presentation remain vigilant about potential side effects that will likely Neuroimunologia
and excellent recovery. emerge with the spreading of vaccination.

Neuroimunologia Neuroimunologia

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NEUROMYELITIS OPTICA SPECTRUM REVERSIBLE CEREBELLAR MAGNETIC YOUNG PATIENT DIAGNOSED WITH CRION
DISORDER: A CASE PRESENTATION WITH RESONANCE IMAGING ABNORMALITY WITH VISUAL IMPROVEMENT AFTER USE
THE SAME ONSET IN IDENTICAL TWINS AND ATAXIA AS A FIRST MANIFESTATION OF HUMAN IMMUNOGLOBULIN: A CASE
IN A PATIENT WITH SYSTEMIC LUPUS PRESENTATION
Matheus da Costa Guedes, João Gabriel Dias
Brasiliense Frota, Matheus Costa Bessa, Gabrielle ERYTHEMATOSUS
Vitor Maia Arca, Bruno Henrique Carneiro Costa
Benevides Lima, Ivna Lacerda Pereira Nobrega, Karla Oliveira Couto, Thiago Gonçalves Fukuda, Filho, Igor de Oliveira, Arthur Cesário de Holanda,
Lucas Silvestre Mendes, Gabriela Joca Martins, Jose Maria Clara Carvalho Silva de Amorin, Pedro José Daniel Alves de Oliveira, Raphaelly Ribeiro Campos,
Artur Costa D’almeida, Karoline Ferreira Mororo da Silva Júnior, Ian Felipe Barbosa Souza, Filipe Ana Rosa Santana, Alvaro Jose Porto Moreira,
Menezes, Fernanda Martins Maia de Carvalho Nolasco de Souza e Silva, Mariana Soares Pinheiro, Eduardo Sousa de Melo Melo, Jose Luiz de Mirando
Ana Flávia Souza Freire da Silva, Rayanderson Coelho Inojosa
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Universidade de Fortaleza. Fortaleza CE, Brazil. Nunes da Gama, João Gabriel Teixeira Mota
Hospital das Clínicas da Universidade Federal de
Fortaleza CE, Brazil Hospital Santa Izabel. Salvador BA, Brazil Pernambuco. Recife PE, Brazil
matheusguedes010@gmail.com karla.oliveira.couto@gmail.com vitorm_arca@hotmail.com

Case Presentation: We describe the case of identical Case Presentation: A 36-year-old woman, admitted to the Case Presentation: A 26-year-old female patient with
twins sisters with neuromyelitis optica spectrum disorder emergency department complaining cognitive changes, low visual acuity and photophobia in the left eye. The
(NMOSD) with antiquaporine 4 antibodies (AQP4-Ab) in gait imbalance, progressive incoordination, asthenia condition evolved progressively, with the appearance
positive, within a two years difference in the onset of and weight loss started 3 months ago. At the neurological of pain on ocular mobilization 2 days after the onset of
symptoms between them. Sister 1: 19-years-old, with examination, the patient presented disorientation with symptoms. Condition occurred acutely, already waking
back and neck pain, lower limb weakness and sensory inappropriate behavior, gait ataxia and bilateral dysmetria up with visual changes. The patient had several similar
loss and evoluted with urinary retention, persistent head- and dysdiadokokinesia. Initial investigations showed the episodes in both eyes since the age of 14, with a milder
ache and ocular pain with transitory loss of vision in both presence of normochromic normocytic anemia, with pos- condition, with no limitation of daily activities in most
eyes. Spinal magnetic resonance imaging (SMRI) revealed itive direct coombs. The FLAIR in brain MRI revealed high episodes. The current episode it is the second with an
T2/FLAIR hyperintensity at the level of C3-C7 and L1-S1. signal intensity in the cerebellum symmetrically and bilat- impact on daily activities. In the first severe episode, she
Cerebrospinal fluid (CSF) examination showed a cellu- erally and the DWI showed restricted diffusion. The study underwent pulse therapy with methylprednisonole and
lar reaction of 499 WBC/mm³, Protein level of 71 mg/dl, of CSF that had inflammatory characteristics with 35 cells her symptoms completely improved. In an ophthalmo-
Glucose 40 mg/dl. The AQP4-Ab test was positive (1: 80). (85%monocytes), proteins 58 mg/dl and glucose 50 mg/dl, logical evaluation, an important change in the visual field
Methylprednisolone intravenous pulse therapy (IVMP) the CSF culture did not reveal any signs of infection; the of the left eye was observed by the campimetry. MRI of
associated with immunoglobulin worked to bring a clinical culture was negative at 72 h, cytomegalovirus PCR, human the brain was performed, with a slight hypersignal on T2
improvement of the symptoms, and the empiric main- herpesvirus 1 e 2, varicella-zoster virus and Epstein–Barr in the left optic nerve. Cerebrospinal fluid was normal
tenance regiment were made with Rituximab after the virus PCR were all negative. Serological profile for hep- Requested rheumatological tests, anti aquaporin 4, and
change of Azathioprine due it resulted in hepatotoxicity. atitis B, hepatitis C and human immunodeficiency virus anti MOG research, all negative. The patient was again
Sister 2 20-year-old, that two months prior to her clinic visit were negative. Neoplastic screening with tomography was submitted to pulse therapy with methylprednisolone,
also experienced back pain, paresthesia in upper limbs and negative. Autoimmune profile, however, was positive for without a satisfactory clinical response. Next step was
ascendent hypoesthesia localized in the intermammillary antinuclear antibody (1: 640 titers, speckled pattern), anti- intravenous immunoglobulin infusion, and a significant
region and evoluted with weakness of her legs, paraplegia DNA antibody (1: 40 titer) and anti-Sm antibody reagent positive response of improvement in the visual acuity was
and sphincter defect. On neurologic examination revealed with consumed C3, C4 and CH50. Systemic lupus ery- observed. In this context, in view of the clinical mani-
meningeal irritation, and confirmed the sensory loss and thematosus, unequivocally fulfilling the Systemic Lupus festation and the complementary exams, the diagnosis
paraplegia. SMRI evidenced T2 hypersignal at the level of International Collaborating Clinics classification criteria, of Chronic Relapsing Inflammatory Optic Neuropathy
T10 and longitudinally extended hypersignal in the cer- was made and corticosteroid treatment was started with (CRION) was made. Discussion: CRION is a rare form of
vical column. CSF revealed 8 WBC/mm, Protein level of prednisone 60 mg per day, after a week of follow-up the autoimmune optic neuropathy. Is characterized by vision
98 mg/dl, Glucose 54mg/dl. The AQP4-Ab test was also patient has a completely improved of ataxia and the brain loss and pain, and the occurrence of symptoms for more
positive. IVMP associated with Plasma exchange was MRI abnormality. Discussion: Neuropsychiatric mani- than one episode. MRI shows contrast enhancement in
a success and the empiric maintenance regimen was festation is commonly found in patients with SLE, it can the optic nerve, and a good response to corticosteroid
made with Rituximab. Discussion: NMOSD is an group manifest as changes in both the central and peripheral therapy is expected. Absence or suspension of treatment
of demyelinating syndromes characterized by IgG auto- nervous systems. The most common manifestations are can lead to recurrence of symptoms, with possible irrevers-
antibodies directed against aquaporin-4 causing severe cognitive dysfunction, headache, seizures, and psychiatric ible visual loss. An atypical aspect of the case described
attacks in the optic nerve and/or spinal cord, leading to conditions; aseptic meningitis, stroke, encephalopathy, is the therapeutic response only with the use of human
blindness, dysautonomic symptoms and paralysis. Most movement disorders, and myelopathy are also seen. Immunoglobulin. Kalish at el. described a series of CRION
cases of NMOSD are sporadic, but some familial reports Cerebellar involvement in SLE is estimated at less than 2% cases with a favorable outcome in selected cases treated
were reviewed, with a prevalence of 0,8%. So, in this case, of cases and more rarely as the first clinical manifestation. with venous immunoglobulin. Final Comments: Patients
we observe twin sisters whose onset of symptoms were We present a patient that had a cerebellar syndrome and with CRION are a diagnostic challenge. It is important for
similar and started within a two years difference between MRI abnormalities as the first presentation of SLE. The neurologists to maintain clinical suspicion. Initiation of
them and had to receive the second line attack therapy diagnosis of inflammatory cerebellitis associated with early appropriate therapy may promote a better clinical
with rituximab due to the good response from her sister. SLE was made supported by mild inflammatory changes response and a better functional outcome. The treatment
Final Comments: The treatment refractoriness of the sis- in the CSF, clinical changes and abnormalities in neuro- of acute CRION events with human immunoglobulin is
ters evidence that there may be a prognostic relationship imaging rapidly reversible. Cerebellar degeneration due not the first therapeutic option, but as described in the
influenced by genetics, especially in the improvement of to SLE is not common. literature, it becomes a viable option, especially in cases
symptoms after acute treatment. of first-line refractoriness.

Neuroimunologia
Neuroimunologia Neuroimunologia

147
XXX Congresso Brasileiro de Neurologia

TL 1106599 TL 1106637 TL 1104891

AN ILLUSTRATIVE PHENOTYPE IN ANTI-MOG CASE PRESENTATION PSEUDOTUMORAL MILLER FISHER SYNDROME: CRACK USER
ASSOCIATED MENINGOENCEPHALITIS MULTIPLE SCLEROSIS LESION ASSOCIATED RECURRENCE
WITH DISCONTINUOUS FINGOLIMOD
Juliana Naback Toniolo, Thales Pardini Fagundes, Andréia Canello, Maria Francisca Moro Longo, Luisa
Anna Letícia de Moraes Alves, Lucas Gondim Briand TREATMENT Zanetti, Fabiana Romancini, Felipe William Dias
Vieira, Trajano Aguiar Pires Gonçalves, Ana Beatriz Edson Junior Gonçalves Bechara, Annelise Akemi Silva, Dara Lucas Alburquerque, Jordana Villar,
Gonçalves Zanovello, Beatriz Gioppo Betini, Rui Higa Lee, Angelo Chelotti Duarte, Antônio José Pietro Domit
Kleber do Vale Martins Filho, Vanessa Daccach Rocha, Maria Fernanda Mendes
Marques, Katharina Vieira Messias UNIVILLE, Joinville SC, Brazil
Hospital Santa Casa Misericórdia de São Paulo, São Hospital Municipal São José. Joinville SC, Brazil
Hospital das Clínicas da Faculdade de Medicina de Paulo SP, Brazil
Ribeirão Preto. Ribeirão Preto SP, Brazil canello.deia@gmail.com
a_akehiga@yahoo.com.br
moraes.annaleticia@gmail.com Case Presentation: A 54-year-old man sought Hospital
Case Presentation: Female, 26 years old, diagnosed care due to low fever, productive cough, urinary retention,
Case Presentation: A 23-year-old male admitted to our with a Relapsing-Remitting Multiple Sclerosis (RRMS) in low back pain, visual alteration, and diffuse paresthesia
Hospital with a headache three weeks after his first dose 2018 with na episode of right central visual field deficit. for 2 days. Miller Fisher Syndrome (MFS) was diagnosed
of vaccination against SARS-CoV-2. One week later, he was She reported two previous episodes in 2012 and 2017, of and treated with immunoglobulin. Crack user. Irregular
admitted due to generalized tonic-clonic seizure, evolv- lower limb paresthesia with progressive improvement and treatment of hepatitis C. On examination, patient in regu-
ing with fever, encephalopathy, bilateral low visual acuity complete remission within thirty days. In May 2021, she lar general condition, pain facies, eupneic, photoreactive
and left hemiparesis. Cerebrospinal fluid (CSF) examina- had na episode of paresis in the left side and left visual isochoric pupils, extrinsic ocular muscles hampered in the
tion showed pleocytosis (lymphocyte predominant) and deficit, involvement of VII, IX and X cranial nerves ipsilat- left and bilateral lower gazes, reduced overall strength with
elevated protein. Broad-spectrum antimicrobials were eral. She reported have stayed in February and April 2021, difficulty in overcoming resistance, areflexia in lower limbs
promptly initiated, but specific exams for infectious dis- without fingolimod. No infectious processes or previous and hyporeflexia in the upper limbs, appendicular ataxia,
eases were all negative. His first brain magnetic resonance vaccination. Neurological examination showed complete other aspects of the neurological examination without
imaging (MRI) was normal. He had mental status worsen- hemiparesis with predominance of the left brachial, AV abnormalities. Admission exams for infectious screening
ing and a new MRI demonstrated cortical and subcortical 20/80 on the left and 20/30 on the right, deviation of without changes. Lumbar puncture was performed, clear
hyperintensity on the T2-weighted image and FLAIR in the uvula on the right and tongue deviation on the left. and colorless CSF, glucose 61, protein 114, 1 cell/mm³,
the right frontotemporal region. He was readmitted to EDSS: 8.0 at the time of admission. Complementary tests: 100% MNM, gram-negative, ink negative, lactate 1.7, VDRL
our Hospital, where the antimicrobial treatment was sus- cerebrospinal fluid (CSF): 01 cell, proteins: 40, glucose: negative. Cranial CT angiography without acute injuries.
pended and methylprednisolone and plasma exchange 53, BOC present. Other exams without changes. Brain The patient’s general condition worsened, evolving with
were prescribed, considering the possibility of a demy- magnetic resonance imaging (MRI): tumefactive right vomiting, plethoric facies, worsening of left lumbar pain,
elinating disorder, with optimal response. A new MRI nucleocapsar hypersignal with perilesional edema and dyspnea with supplemental oxygen therapy, and need for
showed frontotemporal cortical and subcortical lesions signs of acute supratentorial inflammatory activity. She urinary catheterization. A 2019 electroneuromyography
bilaterally, in the thalamus and brainstem, with areas of received pulse therapy with dexamethasone (200mg/ exam showed an old and partially reinnervated demye-
subcortical enhancement. Oligoclonal bands in CSF and day) for 12 consecutive days with visual and motor defict linating and axonal process in the upper and lower limbs;
anti- myelin oligodendrocyte glycoprotein (MOG) in the improvement: left eye AV (20/40), EDSS: 04. Discussion: signs of diffuse neuropathy involving cranial nerves with
serum were positive. Discussion: MOG antibody-associ- Multiple Sclerosis (MS) is the main chronic demyelinat- signs of old reinnervation, no evidence of active disease.
ated disease (MOGAD) is an inflammatory disease of the ing inflammatory disease of the Central Nervous System Diagnosed with a relapse of Miller Fisher Syndrome, the
central nervous system with many described phenotypes (CNS). Injuries to myelin, oligodendrocytes, and axons, to patient started with intravenous immunoglobulin for five
still in characterization, including meningoencephalitis. varying degrees, are caused by an autoimmune response. days. After clinical and neurological improvement, he was
Final Comments: We presented a case of MOGAD with Abrupt discontinuation of fingolimod is reported to cause discharged from the Hospital. Discussion: Considered a
meningeal involvement, which occurs in less than 10% a rebound effect, with increased inflammatory activity, rare variant of Guillain-Barré (GBS), MFS is a multifocal
of adults. Vaccination may be a risk factor for MOGAD severe flare-ups, and eventually pseudotumor manifes- neuropathy characterized by the clinical triad of ophthal-
attacks. Some findings of our patient are more specific for tations within 4-12 weeks. Treatment is challenging and moplegia, ataxia, and areflexia. The recurrence of MFS
MOGAD, like leptomeningeal and thalamic involvement. pulse therapy with corticosteroids is considered an alter- episodes is extremely rare, with only 37 cases reported
native to treat rebound after discontinuation of fingoli- in the literature until 2016. This fact, associated with
mod. Final Comments: Although a pseudotumoral lesion the chronic use of crack by the patient, brings curiosity
Neuroimunologia concerning the recurrence of FMS itself. A liquor disso-
in MS is always a challenge, in this case presentation its
appearance was due to the abrupt discontinuation of fin- ciation appears in 90% of cases as in the case presented.
golimod. Unplanned discontinuation of this medication, The third cranial nerve is frequently the most affected.?-
caused by interruption of supply, is a risk for MS patients. Final Comments: Clinical history and complementary
The rapid recognition of this manifestation is essential for exams, associated with improvement of the condition
the institution of the correct therapy. with immunoglobulin, strengthen the diagnosis of FMS.
The use of crack, considered a risk factor, associated with
liver disease and previous acute respiratory infection, may
Neuroimunologia have favored recurrence.

Neuroinfecção

148
XXX Congresso Brasileiro de Neurologia

TL 1104949 TL 1105005 TL 1105076

MITOCHONDRIAL MYOPATHY MANIFESTED HERPHETIC ENCEPHALITIS WITH FLU-LIKE VARICELLA ZOSTER MYELOPATHY
AFTER ZIKA VIRUS VIRAL INFECTION: CASE SYNDROME IN A COVID-19 PANDEMIC UNMASKED BY A IMMUNE RECONSTITUTION
PRESENTATION CONTEXT: A CASE PRESENTATION INFLAMMATORY SYNDROME: A CASE
PRESENTATION
Maria Clara Pontello, José Geraldo Speciali, Luciana Bruna Guimarães Dutra, João Pedro Moreira
Siuves Couto Guilherme, Zuriel Rodrigues Seixas Nunes – Gabriel Saboia de Araújo Torres, Cristiani Rocha
Manaus – BA – Brazil, Pedro Thiago de Cristo Rojas Lima Cruz, Filipe Tupinambá Di Pace, Igor Vilela
Universidade Federal de Ouro Preto. Belo Horizonte Cabral, Roberta Sena Graupner, Cecília Tizatto Brum, Lucas Assis Santos de Souza, Bruno
MG, Brazil Barroso, João Victor Oliveira de Melo, Daniel Buzaglo Fukelmann Guedes, Hélio Rodrigues Gomes,
Universidade de São Paulo. Ribeirão Preto SP, Brazil Gonçalves, Vanise Campos Gomes Amaral, Tales de Marcia Rubia Rodrigues Gonçalves, Ida Fortini, Luiz
mariaclarapontellobl@gmail.com Oliveira Júnior Henrique Martins Castro
Universidade Federal do Amazonas. Manaus AM, Hospital das Clínicas da Universidade de São Paulo.
Case Presentation: Female patient, 49 years old, report Brazil São Paulo SP, Brazil
that for 5 years she has been progressing with urinary Universidade do Estado do Amazonas. Manaus AM,
incontinence, sleep apnea using Continuous Positive Brazil gabriel.fla15@hotmail.com
Airway Pressure (CPAP), dysphagia, generalized fatigue, Faculdade Metropolitana de Manaus FAMETRO
severe pain intensity in lower and upper limbs, mild hear- Fundação Hospital Adriano Jorge FHAJ Case Presentation: A 57-year-old woman living with
ing loss, decreased vocal tone and constipation. Activities HIV and with previous poor adherence to treatment
likebrush the hair, going up and down stairs and walking brunagdutraa@gmail.com started regular use of antiretroviral drugs in December
short distances have become obstacles due to fatigue and 2021 after pneumocystosis. In January 2022, she started a
pain. She said it all started after Zika virus infection in 2016 Case Presentation: An 60-years-old previously healthy right frontal throbbing headache and vertigo. Afterwards,
(confirmed by IgG antibody). She denied any similar family women, from a countryside city of Amazonas, was referred she developed paresthesia, progressive crural parapare-
history. After analyzing the case, two possible differential to our neurologic service with a family-reported history of sis, urinary urge incontinence, right hearing loss with
diagnoses for the case were presented: mitochondrial flu-like symptoms such as fever and coryza, and both ret- pulsatile tinnitus. The neurologic examination showed
myopathy and myasthenia gravis and, complementary rograde and anterograde amnesia, starting 45 days before spastic crural paraparesis, hypoaesthesia and decrease
exams were requested. The electroneuromyography per- her first consult and, a week before, psychomotor agitation vibratory sense at the T8 level, a left horizontal torsional
formed in association with the repetitive stimulation test and disorientation in time, space, situation and person. nystagmus and an abnormal right head impulse test. The
revealed the presence of some action potentials of the Her neurological examination showed GCS scoring 9, with cerebrospinal fluid analysis showed a lymphomononu-
motor unit, from the examined proximal muscles, with isochoric photoreactive pupils, no signs of meningeal irri- clear pleocytosis and a positive Varicella-Zoster RT-PCR.
myopathic characteristics. The lactic acid at rest was 5.8 mg tation, no alterations in the cranial nerves and no motor The magnetic resonance imaging (MRI) demonstrated
/ dl and lactic acid after 15 minutes of walking was 22.2 mg deficit. She had bilateral patellar hyperreflexia, left upper T2-FLAIR right vestibulocochlear nerve hyperintense
/ dl showing an increase of 3.83 times. The acetylcholine limb spasticity, and ipsilateral Babinski and Hoffman signs signal and spinal cord hyperintensity at the T1-T2 and
anti-receptor antibody was not found. With the history and were present. She was Hospitalized and tested positive T4 levels. We started treatment with acyclovir for 21
clinical examination associated with the complementary for COVID-19 and, during the Hospitalization, evolved days, with just mild neurologic improvement. During
exams, it was possible to infer as a probable diagnosis of with lowered level of consciousness, seizures and global the investigation, an important increase in the CD4 lym-
mitochondrial myopathy. Treatment with coenzyme Q10 aphasia. CT showed no signs of hypodensity or bleeding. phocyte count was found (12,2 cell/mm3 in December
at a daily dose of 800 mg was instituted and the patient After discussion, the neurologic team started empirically to 300 cell/mm3 in February). Immune reconstitution
presented a complete remission of the vast majority of antibiotic therapy and intravenous Acyclovir and, after 5 inflammatory syndrome (IRIS) was suspected, and a 1
symptoms using the coenzyme for 5 months. Discussion days of Hospitalization, PCR in cerebrospinal fluid was g intravenous methylprednisolone pulse therapy was
Just as infection by the Zika virus has been linked to the requested showing HSV-1, and a MRI presenting signal prescribed for 5 days with a significant improvement of
occurrence of neurological manifestations, other viruses changes with a slight swelling effect and a component of bladder symptoms, gait, and proprioception. She was dis-
have also been shown to have similar actions. Reports in breakdown of the blood-brain barrier, suggestive of a viral charged from the Hospital walking with no support. IRIS
the literature prove the relationship between arbovirus inflammatory process. Patient evolved with improvement consists of an increase in T-cell lymphocyte counts result-
infection and the involvement of the nervous system. In compared to admission and referred for outpatient fol- ing in a dysregulated immune response against infecting
the case presented, the Zika virus was associated with a low-up. Discussion: HSV encephalitis representes one of pathogens and the host, concomitantly. The incidence of
neurological manifestation different from those reported the most devastating forms of encephalitis. Its incidence this condition changes according to the pathogen, being
so far in the available literature, mitochondrial myopathy. accounts for up to 20% of all identified viral encephalities. more common in tuberculosis and Cryptococcosis. Our
The relationship can be established since even before the Little is known about the influence of SARS-Cov-2 infection patient presented an increase of CD4 lymphocyte count
arbovirus infection, the patient denies any symptoms of on the CNS and its neurological alterations. Otherwise, associated with new neurological symptoms and abnor-
mitochondrial dysfunction. However, it is not possible to some authors state that a proinflammatory status and mal imaging exams and evolved with mild improvement
say whether viral infection was the triggering factor of the release of cytokines are the key to manifest other viral after adequate treatment of the Varicella-Zoster virus. As
dysfunction or the environmental activator of the muta- encephalitis, specially the ones caused by reactivation of immune reconstitution inflammatory syndrome was sus-
tion, with the appearance of clinical manifestations. Final virus from the Herpesviridae family. Kahwagi et al (2022) pected, steroid treatment was prescribed to reconstitute
Comments: Knowledge of the pathogenic mechanisms reported a case of coinfection including encephalitis the blood-brain barrier and decrease the response of T
involved in Zika virus infection and its consequences for caused by Varicella-Zoster virus confirmed by lumbar lymphocytes. Afterwards, a great clinical improvement
adults is still very limited. puncture associated with a swab test positive for COVID- was noticed. There is no previous report of a Varicella
19, reinforcing participation of the new coronavirus in Zoster myelopathy unmasked in this scenario and with
dysregulation of immune system and neuroinflammation. a great response to steroids, resembling the importance
Neuroinfecção Final Comments: The case raises greater vigilance in typ- of the recognition of this important condition.
ical neurological conditions of encephalitis that present
SARS-CoV-2 co-infection, because, despite the outcome
of this correlation isn´t fully established, it is known that Neuroinfecção
the pro-inflammatory state sustained by the coronavirus
performs a favorable condition for HSV-1 reactivation.

Neuroinfecção

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XXX Congresso Brasileiro de Neurologia

TL 1105078 TL 1105084 TL 1105092

NEUROBORRELIOSIS, MANTAINING A HIGH ACCIDENTAL TETANUS OF ATYPICAL DENGUE-ASSOCIATED DEMYELINATING


SUSPITION: CASE PRESENTATION EVOLUTION, A CASE PRESENTATION LESIONS – CASE PRESENTATION
Luiz Gustavo Brenneisen Santos, Victoria Veiga PEDRO ANGELO LUZINI GONDIM, SUZANA NOVAIS Tiago Camilo Eisemberg Alvarenga, Rafaella
Ribeiro Gonçalves, Paula Fiuza Rodrigues Medeiros, TEIXEIRA OLIVEIRA, ISABELA LUZINI GONDIM Albuquerque Lopes, Raissa Domingues de Simoni
Nathalia Watanabe, Pedro Vinicius Brito Alves, Paula Silveira, Cinthia Grisolia Barbosa, Ronaldo Duarte
Baleeiro Rodrigues Silva, Mauricio Silva Teixeira HUGOL – GOIÂNIA – GO – Brazil Figueiredo, Isabela Cristina Soares Cordeiro,
UNIRV – GOIÂNIA – GO – Brazil Deiziani Cristina Valadares Silva, Igor Camilo
Hospital Sirio-Libanês, São Paulo SP, Brazil Eisemberg Alvarenga, Camila Dias Martins
suzananovais.novais@gmail.com
l.gustavo1201@hotmail.com CIN – Centro Itabirano de Neurociências – Itabira –
Case Presentation: A man, 48 years old, rural worker, 210 MG – Brazil
Case Presentation: A previously healthy 38-year-old cm tall, hypertensive and obese, presented for seven days Hospital Nossa Senhora das Dores – Itabira – MG
woman presented to an emergency care in São Paulo intense pain in the left gastrocnemius.On examination, – Brazil
-Brazil due to severe holocranial headache, nausea and fixed left gastrocnemius muscle contracture unleashed CIN – Centro Itabirano de Neurociências – itabira –
vomiting for the last 7 days. During initial evaluation, she by light and sound stimulus. Normoreflexia, normoes- MG – Brazil
was agitated and disoriented in time and space, but had thesia. Homans sign absent. Trismus/dysphagia absents. Hospital Nossa Senhora das Dores de Itabira –
otherwise normal physical and neurological exams. She Presented two punctate lesions consolidated on the sole Itabira – MG – Brazil
denied fever and skin rash and had no signs of meningeal of the left foot caused by barbed wire fence, one with 30
irritation. As for epidemiologic history, she lived in Geneva days long and the other with 15 days long. No ecographic tiagocea@hotmail.com
and was recently vacationing in the USA (Kansas and evidence of deep vein thrombosis, nor phlogistic signs and
Washington) before coming to Brazil. Initial tests showed no signs of clinical severity. Two doses of the dT vaccine, Case Presentation: Male patient, 21 years old, presented
mild neutrophilic leukocytosis and a positive ?-hCG test. which last one applied 25 years ago.Three visits to the with myalgia, headache and fever for 3 days and spon-
Inflammatory and rheumatologic tests were normal and Emergency Room in the last 20 days for trismus as the taneous resolution. After 7 days of the resolution of the
there were no organ disfunctions. Brain MRI showed main complaint, in addition to sardonic laughter, when previous condition, he started to have paresthesia in the
only non-specific FLAIR hyperintensity in some cortical diazepam being administered for “ansiety”. Therefore, lower limbs, evolving in a subacute way with urinary reten-
sulci. CSF analysis showed a slightly elevated opening patient classified as Tetanus grade I (according to the 1956 tion. Referred to the emergency service with progressive
pressure 21cmH2O, 38 cells with lymphocitic predomi- Ablett scale)in local phenotype.Treatment with antibiotic worsening of strength in the upper limbs after 24 hours.
nance, 65mg/dL protein, normal glucose and somewhat therapy, muscle relaxation, analgesia was made, admin- After admission, the patient began to present diplopia
elevated ADA 4,8mg/dL and lactate 22mg/dL. Serologies istration of equine tetanus-antibody and a 3rd dose of dT. and excessive sleepiness. In the initial exams, the only
for toxoplasma, syphilis, herpes family and bacteriolog- He was evolved with improvement of muscle spasms after laboratory alteration that called attention was a serology
ical, micobacteriological and fungal test were negative. antibodies inoculation, however, remained with muscle for Dengue (positive IgM and IgG). CSF analysis showed
Nevertheless, due to clinical suspicious of encephalitis, IV pain and required intensive support after showing signs pleocytosis (112 cells with lymphocytic predominance)
acyclovir and ampicillin were introduced empirically. After of Respiratory Failure. After discharge from the ICU, he and protein analysis (194mg/dl, presence of a suggestive
5 days, due to symptomatic maintenance, further analysis had remission of the initial symptoms. Discussion: Recent band in the gamma region). Magnetic resonance imaging
of the CSF was conducted with a negative herpes-family data from the WHO show an increase in tetanus notifi- of the Thoracic Spine showed a hyperintense centromedul-
PCR and a positive ELISA IgM for Borreliosis. Ampicillin cations, a preventable disease and compulsory notifica- lary lesion on T2/stir, affecting practically its entire length
and acyclovir were discontinued and intravenous ceftri- tion in Brazil. In view of that, it is a case that starts with with spots enhancement by gadolinium. Brain MRI with
axone was introduced and maintained for 28 days with symptoms of generalized disease and developed to a signs of infratentorial and supratentorial lesions, without
progressive symptomatic improvement. Discussion: Lyme local clinic. This scenario is against with the mechanism gadolinium enhancement. Lesions were not characteristic
disease is a tick-borne illness, endemic in the USA and of retrograde axonal transport of tetanospasmin, which of ADEM. It was decided to perform pulse steroid therapy
some parts of Europe. It usually starts within a month of implies diffuse involvement, in addition of questioning the with Methylprednisolone 1g for 5 days, with progressive
the tick bite by presenting with characteristic skin rash bioavailability of the toxin due to the size of the patient. improvement and complete resolution of the condition.
and constitutional symptoms. After a few months, neu- Additionally, there is a low clinical suspicion for intoxi- Discussion: The Dengue virus can affect the neurolog-
rologic findings may follow, being the most commom cation by strychnine, since there was no relaxation of the ical system directly by viral invasion or indirectly with
presentations cranial nerve palsies, radiculopathy, lym- muscles between spasms or maxillary involvement, and immune-mediated reactions. Complications may arise
phocytic meningitis and, rarely, encephalomyelitis. For also for conditions such as drug withdrawal or hypocal- during a feverish period or during convalescence, and
the diagnosis, ELISA and Westernblotting essays for B. cemia due to the epidemiology and personal history not may manifest as Encephalitis, Stroke, Myalgia, Guillain
burgdoferi s.s. antigens are used. As for the treatment, suggestive. Final Comments: Early diagnosis and treat- Barré Syndrome, Myositis, Hypokalemic Paralysis, Myelitis
being the patient pregnant, she was unable to receive ment are ratified in this scenario and invite specialists to and Acute disseminated encephalomyelitis (ADEM). In
oral doxycycline. In these cases possible options are IV propose a new clinical presentation scale that increases the case above, the manifestations started 7 days after the
cefotaxime, G penicillin or ceftriaxone, which was the sensitivity for atypical cases of tetanus. febrile period, suggesting a molecular mimicry reaction
therapy of choice in this case. Final Comments: In this between the pathogen and the host’s myelin. There are
study we present an unusual case in our country, with few cases in the literature relating longitudinally exten-
non-specific symptoms. Serologic testing combined to Neuroinfecção sive myelitis associated with dengue, with emphasis on
an epidemiologic exposure were key for the diagnosis, a study in the Amazon region of 51 cases, with favorable
reinforcing the importance of extensive questioning of evolution and recovery of deficits in 95% of patients.
travel history during anamnesis. Other cases correlate dengue with brain involvement,
most cases occurred during the recovery phase of uncom-
plicated dengue. Final Comments: Dengue is the most
Neuroinfecção prevalent arbovirus in the world, and Brazil recorded an
increase of 113.7% in the first months of 2022 compared to
the previous year. Consequently, they link an alert to the
neurological manifestations associated with this infection
that can be serious and potentially fatal.

Neuroinfecção

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XXX Congresso Brasileiro de Neurologia

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CERVICAL INFECTIOUS SPONDYLODISCITIS RHOMBENCEPHALITIS SECONDARY TO UNUSUAL ETIOLOGY OF TRANSVERSE


AS A DIFFERENTIAL DIAGNOSIS OF ACUTE BRAIN ABSCESS AS A DIFFERENTIAL MYELITIS: CASE PRESENTATION
FLACCID TETRAPARESIS DIAGNOSIS IN SUBACUTE TETRAPARESIS IN
Maria Lúcia Vellutini Pimentel, Ana Beatriz da Costa
A IMMUNOCOMPROMISED PATIENT Lucas, Cícera Simplicio Machado Leandro, Frederico
João Vitor Mortari Lisboa, Isaac Pantaleão Souza,
Pedro Machry Pozzobon, Patrick Emanuell Mesquita João Vitor Mortari Lisboa, Isaac Pantaleão Souza, Del Duca Frazão, Gabriel Rivadavia de Farias
Sousa Santos, Itamar Meireles Andrade Santos, Pedro Machry Pozzobon, Patrick Emanuell Mesquita Amaral, Kathleen Torres Tenorio Monteiro, Lucas
Eduardo Abrão Spinola Rezk, Laura Cardia Gomes Sousa Santos, Itamar Meireles Andrade Santos, Vergara Cury, Rodrigo Moreira Pereira, Vanessa Gil
Lopes Eduardo Abrão Spinola Rezk, Ana Flávia Andrade Humberto dos Santos, Leticia Fêzer Mansur
Lemos, Laura Cardia Gomes Lopes PUC-RJ/SCMRJ. Rio de Janeiro RJ, Brazil
Unesp – Botucatu – SP – Brazil
Unesp – Botucatu – SP – Brazil PUC-RJ/SCMRJ. Rio de Janeiro RJ, Brazil
João vmlisboa@outlook.com
João vmlisboa@outlook.com bia_lucas@hotmail.com
Case Presentation: A 72-years-old man, previously hyper-
tensive and alcoholic, presented to the emergency room Case Presentation: R.M, 48 years old, male, with a pre- Case Presentation: L.S.M., male, 18 YO, was admitted to a
with a five-day history of shortness of breath, cough, fever, vious history of untreated HIV infection. Started vomit- Hospital with bilateral paresthesia in the plantar regions,
muscle pain and four limbs weakness. Neurological exam- ing, diarrhea, abdominal pain, headache and hiccups in ascending to the lower limbs, associated with sphincter
ination demonstrated tetraparesis, hypotonia in four limbs, March/2022. After 2 weeks, initiates progressive tetra- incontinence, in June 2021. The brain MRI showed focci of
areflexia in upper limbs and hyporeflexia in lower limbs. paresis, starting with the lower limbs and ascending to high-signal on T2/ FLAIR, in the subcortical white matter,
Laboratory demonstrated leukocytosis. Blood culture was the upper limbs, preventing ambulation, associated with variable in size and shape, some with gadolinium enhance-
positive for methicillin-sensitive SA. Cerebral spinal fluid urinary incontinence, dysphagia, paresthesias that pre- ment; the cervical spine MRI showed nonspecific focal
analysis revealed important protein elevation, no glucose dominated in the lower limbs and unintentional weight areas of high-signal on T2. The cerebro spinal fluid (CSF)
consummation and cell count within reference range. At loss (8 kg in 2 months). During the initial evaluation, was clear, with 04 cells mm³ (10% polymorphonuclear,
arrival of patient, the MRI was not available, and among tetraparesis was demonstrated associated with hyper- 90% mononuclear); negative bacterioscopy, glucose 64
the differential diagnosis, the main hypothesis was of reflexia. The evaluation of the cranial nerves showed a mg/dl; protein 28.4 mg/dl; negative culture. Blood VDRL
Guillian-Barré syndrome. Extended investigation with deficit to lift the palate bilaterally, as well as the inability was 1/32. He was treated with benzathine penicillin for 3
cervical MRI realized as soon as available, showed at to protrude the tongue. Brain CT was normal, as well as weeks and pulse therapy with methylprednisolone for 5
FLAIR and T2, inflammatory hypersignal at C3-C4 left CSF test. Systemic investigation also was negative. Two days, with no improvement in the symptoms. He was seen
facet joint and three isolated collections. The third forma- days after the initial evaluation, the patient had an epi- in our Service, in July 2021, and new tests were requested
tion was causing vertebral foramen stenosis with hyper- sode of fever, respiratory failure, and was intubated and that showed VDRL 1/32; Vitamin B12 241, HIV negative,
signal alterations at the adjacent spinal cord compatible started on cefepime. Three days later, the patient started HTLV negative, Hepatitis B and C negative. A new CSF
with compressive myelopathy. All three formations were to experience significant clinical improvement in the tet- study on 07/27/21 showed 6 cels/mm3, 29 protein mg/
suggestive of abscess, due bacterial spondylodiscitis. The raparesis, as well as in the lower cranial nerves. Brain MRI dl, 80 lymphocytes, glucose 66 mg/dl, VDRL negative,
patient was treated with intravenous oxacillin, with clinical was performed after clinical stabilization, demonstrating a FTAabs IgG positive, IgM negative, Kappa index 3.7 (nor-
improvement and regression of the masses at a posterior nodular lesion on T1-weighted gadolinium enhancement mal < 2.9), OCB negative, anti-NMO negative, anti-MOG
control MRI. Discussion: Spondylodiscitis refers to the in the medulla, with T2 hyperintensity in the medulla up negative. He was treated with crystalline penicillin for
infectious process that affects the vertebral body, the pos- to C5 level in spinal cord. Due to clinical improvement, 14 days and pulse therapy again for 5 days with signifi-
terior vertebral arch, and the intervertebral disc. Installed the hypothesis of brain abscess is considered, and antibi- cant improvement of the symptoms. In October, a new
in an insidious way and has a slow evolution, usually otic therapy is maintained for 6 weeks. A new brain MRI VDRL test was performed: 1/16. Discussion: Syphilis is
manifesting as fever, local pain, changes in sensitivity and was performed 3 weeks later, demonstrating a significant a sexually transmitted disease caused by the Treponema
strength in limbs, and may present as a radiculopathy as reduction in the lesion as well as the associated edema. pallidum. It affects 12 million people a year worldwide.
well as a spinal syndrome due to the involvement of the After 6 weeks, the patient was discharged from the Hospital If untreated, the disease can progress up to the tertiary
spinal canal. The physical examination of uncooperative with resolution of symptoms. Discussion: Brain abscess form, which affects the central nervous system (CNS)
patients can make it difficult to discriminate sensitivity does not usually occur in the brainstem region, and the and can be presented from its different clinical types:
changes such as, for example, sensory level in the context involvement in this location is associated with significant meningovascular, general paresis and tabes dorsalis.
of spinal cord syndrome. In this context of flaccid tetra- morbidity and mortality. The most likely etiology of a bac- Transverse myelitis has different etiologies, such as auto-
paresis, as in the case above, the acute demyelinating terial brain abscess in immunocompromised patients is immune diseases, CNS infections, hypovitaminosis B12
polyradiculopathy syndrome is an important differential Listeria monocytogenes, and the response to beta-lactam and demyelinating diseases Syphilitic myelitis is a form
diagnosis. This protein-cellular dissociation in the cere- makes this the main hypothesis for our case. Nocardia spp of meningo-vascular syphilis confined to the spinal cord.
brospinal fluid, uncommon in peri-medullary infections is also an important agent, but its clinical course occurs The diagnosis sometimes is challenging and comprises
that usually progress with an increased number of cells in a more insidious way. Final Comments: The immuno- not only blood tests, but also MRI findings, such as “the
in the CSF, was notorious confounding factor, alongside compromised patient can be affected by several serious flip-flop sign” or “the candle guttering appearance sign”
to the limb flaccid paresis found in the acute spinal cord insults, often without the typical manifestations, as we and CSF test. MRI can sometimes be essential for a cor-
injury. Final Comments: The acute flaccid tetraparesis demonstrate a patient with absence of fever and headache rect diagnosis. Final Comments: This case describes
has a large number of differential diagnoses ang usually in the initial condition. Thus, brain abscess should always an uncommon presentation of syphilis as a transverse
demands early complementary exams to the correct eval- be a differential diagnosis in the context of tetraparesis myelitis. Syphilis is a differential diagnosis to look after
uation of the lesion, especially in the ER. associated with signs of upper motor neurons, especially in transverse myelitis cases specially because of good
in immunocompromised patients clinical response to the therapy.

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Neuroinfecção Neuroinfecção

151
XXX Congresso Brasileiro de Neurologia

TL 1105136 TL 1105151 TL 1105208

NEUROTUBERCULOSIS IN AN HOLOCORD SPINAL EPIDURAL ABSCESS – NEUROBORRELIOSIS, A RARE CONDITION


IMMUNOCOMPETENT PATIENT: A CASE- A RARE CASE PRESENTATION IN BRAZIL – A CASE PRESENTATION
BASED UPDATE GABRIELA DA SILVA CREMONESE, NATHALIA
Emanuelle Bianchi da Silva Rocha, Amanda Selvátici
dos Santos Dias, Janaina Moraes de Araújo, João TOMAZONI SILVA, IVANIO ALVES PEREIRA
Luís Eduardo Oliveira Matos, João Valdêncio Silva,
Yasmin Silveira Cavalcante, Amandha Espavier Pedro Izidoro Gomes, Caique Alberto Dosualdo, Universidade do Sul de Santa Catarina – Palhoça –
Trés, Nickolas Souza Silva, Espártaco Moraes Guilherme Vedovato Vilela de Salis, Andressa Regina SC – Brazil
Lima Ribeiro, Keven Ferreira Ponte, Paulo Roberto de Mello Galego, Gabriel Pina Paiva, Ricardo Funes
Lacerda Leal Bastos, Fábio de Nazaré Oliveira gabicremonese@hotmail.com

Universidade Estadual do Ceará. Sobral CE, Brazil Faculdade de Medicina de Ribeirão Preto. Ribeirão Case Presentation: A 37-year-old, male pacient presented
Preto SP, Brazil weakness in the lower limbs that was initially attributed
luisematos@icloud.com
m.bianchis@hotmail.com to ciatalgia associated with muscle shortening since
2019. Due to the worsening of the condition with low
Case Presentation: A 35-year-old man was admitted 1
Case Presentation: male healthy patient, 56 years old, back pain and cramps, he sought out other profession-
month ago with paraparesis and thunderclap headache.
refers back pain started 9 days ago irradiated to lower als who diagnosed him with axial spondyloarthritis and
He reports a chronic condition of nausea, vomiting, diar-
limbs and perineum with urinary hesitancy and vomit started treatments with non-steroidal anti-inflammato-
rhea, recurrent fever and monoparesis in the left lower
without fever. First diagnosis thought was nephrolithiasis, ries and sulfasalazine. More than 2 years after the onset
limb for 3 months. A skull Magnetic Resonance Imaging
excluded after normal abdomen computed tomography of symptoms, consultations with some specialists and
(MRI) was performed, showing important hydrocephalus
(CT). The following day, started with weakness in the right progressive worsening of the condition, with associated
with diffuse nodules in the leptomeninges. A ventricu-
lower limb (mainly in quadriceps), constipation and uri- headache and memory loss, the patient underwent an
loperitoneal shunt was installed, with partial neurolog-
nary retention. CT of the thoracic and lumbar region was electroneuromyography that showed axonal neuropathy
ical improvement. There was a condition worsening 2
normal. After two days patient evolved with somnolence, of the posterior tibial nerves and denervation in activity,
weeks ago, with extension to a tetraplegia associated with
flaccid paraplegia, absence of deep sensitivity on lower and a test of ELISA confirmed with Western Blotting for
ascending paresthesia to the T9-T10 level. Neurological
limbs, peripheral facial palsy in the right, dysautonomia positive Borrelia burgdorferi in different laboratories of
examination revealed global areflexia, central nystagmus,
and anisocoria. Cerebrospinal fluid showed pleocytosis excellence. Treatment was started with 28 days of ceftriax-
right peripheral facial palsy and partial neck stiffness.
(neutrophil and lymphocyte predominance), hyperpro- one and continued for two more months with doxycycline.
Laboratory tests showed negative bacterioscopy, blood
teinorraquia and important glucose consumption with Discussion: As evidenced in the case, the clinical mani-
cultures, sputum smear microscopy, rapid tests for HIV
lactate level of 9.9. Magnetic resonance evidenced anoma- festations of brazilian neuroborreliosis – Baggio-Yoshinari
and VDRL. Chest computed tomography (CT) revealed
lous leptomeningeal enhancement of the cerebellum and Syndrome – can mimic multiple diseases and are poorly
no lung lesions, and T2 FLAIR MRI of cervical-thoracic
skull base cisterns; thickening and anomalous enhance- described so far. Consequently, neuroborreliosis can be
spine and skull showed hypersignal in broad sites of tel-
ment of the arachnoid and diffusely involving the entire overlooked by the clinician, especially in non-endemic
encephalon, brainstem, cerebellum, and C3-T8 spinal
spinal cord, including cauda equina; laminar collection areas, and has characteristics of recurrence and a tendency
cord. Cerebrospinal fluid (CSF) examination revealed
with peripheral contrast enhancement extensive on the to cause chronic neurological and joint manifestations,
cellularity of 60/mm3, proteins of 146 mg/dl, glucose of
epidural posterior region from T1 to L2 with greater thick- especially if antibiotic treatment is instituted late. Final
28 mg/dl, negative VDRL and bacterioscopy, no fungal
ness in the lumbar. We started empirical treatment with Comments: This is a challenging diagnostic zoonosis,
growth on India ink stain, and adenosine deaminase
ceftriaxone and meropenem plus dexamethasone 4 mg with clinical symptoms suggestive of other pathologies
(ADA) of 26 U/L, which confirmed the diagnosis of neu-
every six hour. Neurosurgery team indicated conservative and serological tests of limited sensitivity and specificities.
rotuberculosis. A regimen of rifampicin, isoniazid, pyr-
management. Serologies for HIV, syphilis and hepatitis Therefore, further studies on the neurological involvement
azinamide and ethambutol was started, to which the
and PCR for M. tuberculosis in CSF were negative. All CSF of this syndrome are essential to bring attention to this
patient has been evolving with paraparesis improve-
and blood cultures (bacteria, fungi and mycobacterium) differential diagnosis for an effective and early treatment.
ment. Discussion: Tuberculosis (TB) is an infectious dis-
ease caused by Mycobacterium tuberculosis that mainly were negative. On the next day, patient worsened con-
affects lungs, but can also affect the meninges, causing sciousness level requiring orotracheal intubation. After Neuroinfecção
neurotuberculosis (3% of TB cases in seronegative HIV eight days of antibiotic CSF had a significant improvement.
patients). There is slower subacute evolution than other We treated the patient during forty days, with mild neuro-
meningitis. Clinical response to treatment is excellent logical improvement in consciousness level and quadri-
when it is diagnosed before irreversible neurological plegia sequelae. During Hospitalization patient presented
damage, so empiric therapy should be initiated imme- many complications (secondary bacterial infections,
diately in any patient with neurological abnormalities, acute renal failure and upper gastrointestinal bleeding)
CSF with low glucose concentration, high protein, lym- progressing to death 44 days after Hospital admission.
phocytic pleocytosis and if TB is suspected. Higher ADA Discussion: Until now less than twenty cases of holocord
levels in CSF are useful in diagnosis. Hydrocephalus and spinal epidural abscess (SEA) had been published and it is
basal meningeal thickening are common findings in CT more common in immunocompromised. The commonly
or MRI. Treatment is initially composed by a four-drug symptoms are fever, back pain and neurological deficit
regimen including isoniazid, rifampicin, pyrazinamide and the mortality rate is about 15%. Here we present an
and ethambutol daily for 2 months. Final Comments: immunocompetent man that had a bad evolution. Final
The diagnosis of neurotuberculosis in immunocompetent Comments: SEA can mimic other medical conditions, but
patients can be challenging and lead to high mortality if have a high morbidity and mortality, so it is essential to
not diagnosed soon. be attentive to avoid delaying diagnosis and treatment.

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152
XXX Congresso Brasileiro de Neurologia

TL 1105235 TL 1105256 TL 1105363

OPTIC NEURORETINITIS IN AN HIV- NEUROCRYPTOCOCCOSIS AND MENINGEAL CARCINOMATOSIS MIMICKING


SEROPOSITIVE PATIENT: CASE NEUROSARCOIDOSIS: SIMULTANEOUS A PSYCHIATRIC CONDITION: CASE
PRESENTATION PRESENTATION IN AN IMMUNOCOMPETENT PRESENTATION
PATIENT
Ana Claudia Piccolo, Maria Aparecida Ferraz, Luisa Anderson Matheus de Lima Barbosa, Lucas Luan
Almeida Fonseca, Marina Rossi Oliveira, Priscila Maria Weryca de Souza Belo Silva, Diana Pessoa Gonçalves Barros Leal, Carolina Queiroz Feijó, Arieny
Souza Schinzari, Vanessa Ferreira Baldino, Ana Silva Freire, Regina Taís da Silva, Alberto Pereira Karen Santos Lima, Leonardo Halley Carvalho
Karolina Santana Arruda, Daniela Midori Kamada, Firmino Filho, Tulio Francisco de Vasconcelos Silva, Pimentel
Frank Gregory Cavalcante Silva, Leticia Souza Agábio Diógenes Pessoa Neto, Heitor Caetano dos
Borges da Silva Unifacid. Teresina PI, Brazil
Santos, Arthur Felipe Barbosa Vasconcelos, Kaliny
UESPI. Teresina PI, Brazil
Oliveira Peixoto, Joanisson Rubens Gomes Diniz
Hospital MUNICIPAL PROF.DR ALIPIO CORREA
NETTO, São Paulo SP, Brazil andersonbarbosa7570@outlook.com
Universidade Federal do Rio Grande do Norte –
Hospital Universitário Onofre Lopes – Natal – RN
luisaalmeidafonseca02@gmail.com Case Presentation: A 45-year-old previously healthy man,
– Brazil
who works in an accounting office, comes to the doctor
Hospital Metropolitano Dom José Maria Pires. João
Case Presentation: Male, 27 years old, white, initially with a complaint of sudden disorientation, delusions, and
Pessoa PB, Brazil
searched an outpatient clinic due to painless and non-pru- vomiting for 20 days. The confused mental state caused
ritic erythematous macules on the soles of the feet and mariaweryca@hotmail.com dependence on others for all activities for one week, then,
palms, being diagnosed with syphilis, and later as a car- he partially improved his state for five days and worsened
rier of the human immunodeficiency virus (AIDS)/HIV, Case Presentation: C.A.J.S. Male, 52 years, underwent again. He reported a daily headache of strong intensity.
and treatment was started for both diseases (first line for restrictive diet (loss of 20 kilos in 3 months), evolving with He denies seizures, allergies, or comorbidities. His fam-
AIDS, and for secondary syphilis according to the pro- depressive symptoms associated with the neurological ily brought him to the consultation, directing him to a
tocol of the brazilian Institute of Health. After 30 days, framework of paraparesia, abasia, astasia, urinary and psychiatric condition due to behavioral change, and for
he began to complain of floaters in the right eye (OD), fecal incontinence, constipation and hypoesthesia in having a family history (mother) of severe depression. He
in addition to fixed scintillating scotomas in the left eye lower limbs, being restricted to the bed. After psychiatric denies alcoholism or smoking. On physical examination,
(LE), painless and referred to a secondary Hospital for sus- treatment with antidepressants and antipsychotics had the patient does not cooperate, obeys simple commands
pected neurosyphilis. On examination of the eye fundus improved mood and he returned to wander, but only with with difficulty, does not obey complex commands, appears
(FO), papilla edema and signs of retinitis in the RE were support. Then there was sudden worsening of strength unaware of his surroundings, strength preserved, vivid and
observed. During etiological investigation, brain magnetic and equilibrium, being Hospitalized for neurological symmetrical reflexes, cutaneous-plantar reflex in bilateral
resonance imaging and cerebrospinal fluid (CSF) were framework. In lumbar puncture was verified the presence flexion, isochoric and photoreactive pupils, preserved
normal (negative VDRL). A diagnosis of syphilis neurore- of cryptococcus by the direct research of fungi and per- extrinsic ocular motricity, mimicry symmetrical face.
tinitis was defined and the patient received intravenous formed treatment with antifungal for 42 days, with discreet During the investigation, magnetic resonance imaging
crystalline penicillin for 14 days, with total regression of improvement. Concomitant to the previous findings a (MRI) revealed hypersignal in the sulci of the cerebral
symptoms. Discussion: Acquired syphilis can be classified chest tomography was performed to evoluate pulmonary hemispheres, associated with gadolinium enhancement
as primary, secondary and tertiary, being more frequent compromise by the, being evidenced mediastinal lymph in a leptomeningeal pattern. Cerebrospinal fluid (CSF)
and more severe among HIV carriers, and in this case, it node in which anatomopathological suggested the pres- revealed the presence of neoplastic cells. After screening
may present clinical manifestations of two stages simulta- ence of sarcoidosis, raising the idea of neurosarchoidosis for neoplasms, non-small cell lung carcinoma was iden-
neously. T. pallidum is invasive and can affect the central in coexistence with neurocryptococcosis. Subsequently, tified. Discussion: Meningeal carcinomatosis (MC) is a
nervous system at any stage of infection. Neurosyphilis is pulse therapy was instituted with methylprednisolone and rare neurological complication characterized by multifo-
more frequent in HIV carriers, especially in those with- the patient presented substantial improvement of symp- cal invasion of the meninges by neoplastic cells. Breast,
out antiretroviral therapy. Ocular syphilis, on the other toms. Discussion: Neurocryptococcosis is a serious and lung, and melanoma tumors are the main ones respon-
hand, is an uncommon form and early manifestation of usually fatal infection caused by two species: Cryptococcus sible for the cases. About 5% of all cancer patients have
syphilis, at any stage of the disease, and can be confused neoformans and Cryptococcus gattii. In general, it presents meningeal carcinomatosis, lung adenocarcinoma is the
with optic neuritis and neurosyphilis. Uveitis and optic as cryptococcal meningitis and affects immunocompro- one that most often affects the meninges when referring
neuritis are common to all patients with ocular syphilis, mised individuals, with typical symptoms of meningeal to non-small cell cancer. In the present case, the patient
but some studies suggest that HIV-infected patients tend infection: headache, lowered level of consciousness and presented behavioral changes associated with bradypsy-
to have acute posterior uveitis, and particularly poste- cerebrospinal fluid changes. Neurosarcoidosis (NS) is chia and headache. MC is a late event in the evolution of
rior chorioretinitis, as we observed in this patient. Final a granulomatous disease of unknown etiology, whose the neoplasm and can manifest with the involvement of
Comments: Ocular syphilis has different clinical spec- main manifestations are leptomeningitis, cranial nerve spinal roots, cranial nerves, and/or lesions of the cerebral
tra and can manifest at any stage of the disease, thus, it involvement, hypothalamic dysfunction and intraparen- hemispheres. The diagnosis is based on clinical findings,
should be part of the differential diagnoses of retinitis, chymal masses, corresponding to 5% of the presentations neuroimaging, and CSF analysis. It has an unfavorable
optic neuritis and especially neuroretinitis in HIV positive of sarcoidosis. In the case described above, we have prognosis, survival is 3 to 6 months in patients undergoing
patients. Early diagnosis and treatment of neuroretinitis a patient presenting with nonspecific systemic mani- treatment. Final Comments: This clinical case is unusual
will prevent permanent visual loss. festations and significant impairment of sensory and due to the low frequency of MC and the presence of neu-
motor functions, in addition to associated psychiatric ropsychiatric manifestations in the absence of a previous
symptoms, with simultaneous presentation of diseases. diagnosis of neoplasia. In addition, behavioral changes
Neuroinfecção Final Comments: Neurocryptococcosis is a rare fungal may be a rare and early sign of meningeal carcinomatosis.
disease in immunocompetent patients. This case stands
out for its concomitance with another uncommon dis-
ease: neurosarcoidosis, in a previously healthy patient, Neuroinfecção
enabling differential diagnoses of rare combinations with
a favorable outcome.

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153
XXX Congresso Brasileiro de Neurologia

TL 1105367 TL 1105394 TL 1105397

DENGUE AND NEUROLOGICAL SYMPTOMS: CENTRAL NERVOUS SYSTEM INVOLVEMENT PSEUDOTUMORAL NEUROCYSTICERCOSIS
AN ATYPICAL REPORT BY GUILLAIN BARRÉ IN COMPLICATED RHINOSINUSITIS: CASE
Leticia Yabushita Rigoti, Keila Maroli, Samyra Soligo
AFTER DENGUE PRESENTATION Rovani, Vicente Albuquerque Maranhão
Marcelo Simplicio Carvalho, Amanda Leticia André, Igor Fortunato da Silva, Fernanda Gonçalves de UNIOESTE – Francisco Beltrão – PR – Brazil
Tatiane Arroyo Lopes Alves Jesus, Arthur Coelho Andrade, Camila Moreira Carvalho Dias, Helena
Moura Marinho, Nathalye Fernanda Pedrosa Machado Galhardo, Isadora Martins Garcia vicmaranhao@hotmail.com
Dircksen, Lorena Fernandes Kronbauer, Damacio
Ramon Kaimen Maciel Universidade José do Rosário Vellano – Passos – Case Presentation: Woman, 38 years old, no comor-
MG – Brazil bidities, is forwarded to Neurology due to recurrent ton-
Santa Casa de Londrina. Londrina PR, Brazil Santa Casa de Misericórdia de Passos – Passos – ic-clonic seizures on the right, and secondary general-
Santa Casa Londrina / Neuroclinica. Londrina PR, MG – Brazil ization, with sudden onset one month ago. The patient
Brazil lives on a rural property, having agricultural and swine
igor.fortunato@aluno.unifenas.br
marcelo.neurolog@gmail.com farming activities. In the examination, does not present
Case Presentation: Male, 16 years old, illicit drug user, focal signals, however, shows impairment in attention,
Case Presentation: a 52-year-old female patient, 17 days symptoms of headache, mental confusion, bilateral eye- fixation memory, and basic math operations. Image
before the arrival of the service, with asthenia, cutane- lid edema, eye pain, blurred vision and fever after upper exam was realized, and brain MRI evidenced a single
ous rash in thorax and bros, with diagnosis of dengue, airway infection. Exams showing leukocytosis and cere- cystic lesion, easily identified, with 5 cm in the largest
evolved 11 days later with hypoesthesia and paresis of brospinal fluid indicative of bacterial meningitis. Broad- diameter, homogeneous and well delimited, with a liquor-
lower limbs (MMII) with ascence to upper limbs (MMSS), spectrum antibiotic therapy was started. Pulse therapy like component in all MRI sequences, without contrast
subsequently, the patient presented bilateral facial pare- with methylprednisone was also performed to help reduce capture, in the left parietal lobe with mass effect. It was
sis and constipation. The patient presented Lucidity and proptosis and visual symptoms. Magnetic Resonance initiated carbamazepine, albendazole, and dexameth-
well-oriented, with bilateral peripheral facial paralysis, Imaging (MRI) of the brain was requested, which showed asone,and, after, valproic acid, without modification of
with paresis and hyporeflexia of predominance in llll, intracranial collection starting in the medial temporal the condition, remaining symptomatic and with the same
also presented hyperalgesia of them. lumbar puncture region adjacent to the cavernous sinus and extending to seizures. Parietal craniotomy was realized to aspirate the
showed protein dissociation and cellularity, with 10 mm3 the posterior fossa, bypassing the right cerebellar hemi- cystic lesion, obtaining thereabout 80 ml of clear liquid,
of leukocytes, 95% leukocytes, 540.4 meq/l of protein and sphere, suggestive of subdural empyema. He underwent and capsule and scolex exeresis. In the follow-up, after
72 mg/dl of glucose, with cervical resonance and cranial neurosurgical drainage by posterior fossa craniotomy, on eyear, there has been regression on the image exams,
tomography without alterations and other general labora- without complications. Post-surgical control MRI showed showing residual encephalomalacia area, remission of
tory tests within the normal range. performed as human thrombosis of the right sigmoid sinus, probable intraorbital the seizures and the cognitive deficit, and the anticonvul-
immunoglobulin treatment 5 g for 5 days. Before discharge, collection on the right, in addition to reduced proptosis sants were suspended after three years without attacks.
a new cerebrospinal fluid was performed, showing a fall and peri-orbital soft tissue edema, and it was decided to Discussion: Neurocysticercosis is an important cause of
in the protein, 350 meq/l and leukocytes, 4 mm3. After maintain antibiotic therapy and start anticoagulation. epilepsy in developing countries, with behavior changes
Hospital discharge, the patient presented improvement After a month of antibiotic therapy, a new orbital MRI beyond the increased intracranial pressure. Even as the
of the picture with recovery of the strength pattern and showed an abscess next to the superior rectus muscle, presented case, cerebral lesions are commonly found in
improvement of facial paralysis. Discussion: Guillain and ophthalmic surgery was chosen for drainage, which brain imaging exams, due to the installation of the para-
barré syndrome (sgb) is often associated with infections occurred without intercurrences. After almost two months site in brain tissue and the immune reaction produced.
by campylobacter jejuni, cytomegalovirus, Epstein–Barr of Hospitalization and with clinical improvement, the The treatment, occasionally, is a challenge for the profes-
virus, mycoplasma pneumonia, hiv and the presence of patient was discharged with mild restriction of right sional, needing clinical approach with antiparasitic and
association between arboviroses and sgb is well docu- orbit eye movements, being followed up as an outpatient. anticonvulsants, besides the surgical approach. In the
mented, mainly in relation to zika virus, however cases Discussion: the patient in question presented rare and case in question, all the forms of treatment were made,
related to dengue. where only about 5% of the patients serious complications of rhinosinusitis, both ophthalmic aiming at the remission of the parasite and the symp-
present with associated neurological symptoms, they and intracranial. Anatomical factors that explain this toms. Final Comments: This case evidences a rare form
are rare. The symptoms are closely related to infection infectious dissemination are: close relationship between of neurocysticercosis of giant brain cyst, with the differ-
where antigens stimulate the demyelination process, the orbital contents and the ethmoidal labyrinth, which ential diagnosis of neoplastic lesions. The treatment of
where most of the symptoms such as ascending paral- is separated from the orbit by the papyraceous lamina; this patient was effective, with efficient results. Thereby,
ysis, facial paralysis and areflexia. The only treatment the existence of a large number of sutures and neurovas- it is necessary to pay attention to the symptoms and the
with recognized and recommended clinical response is cular foramina in the medial wall of the orbit; the optic disease incidence in our region, aiming at the improve-
the application of human immunoglobulin, with a good canal which is closely adjacent to the posterior ethmoid ment of the patient’s clinical condition.
response, and can be followed after application with pred- and sphenoid sinuses; and valved ophthalmic veins that
nisone, but with studies with inconclusive results, in the communicate with vessels inside the orbit and directly
Neuroinfecção
case presented, the patient had a clinical and laboratory with the cavernous sinus, presenting a direct blood flow,
condition compatible with the sgb, with a clear indica- facilitating hematogenous dissemination. The presence of
tion for immunoglobulin treatment. Final Comments: eyelid edema and headache are warning symptoms for a
The report shows a case of association between dengue more attentive investigation of rhinosinusitis, in addition
infection and neurological symptoms, in which case the to intravenous antibiotic therapy. Final Comments: the
development of sgb is a rare presentation, with a good importance of anatomical knowledge is emphasized for
response to immunoglobulin treatment. understanding the routes of spread of facial and intra-
cranial infections.

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Neuroinfecção

154
XXX Congresso Brasileiro de Neurologia

TL 1105402 TL 1105403 TL 1105419

SYPHILITIC OPTIC NEURITIS SPONDYLODISCITIS ASSOCIATED WITH SPINAL TUBERCULOSIS MIMICKING A


PSOAS ABSCESS COMPRESSIVE PARAVERTEBRAL TUMOR: A
Vitor Teixeira Maito, Vitor Hugo Ferreira Candido,
Samira Soligo Rovani, Vicente Albuquerque CASE-BASED UPDATE
Fernanda Mara Alves, Hellen Camila Marafon,
Maranhão Samyra Soligo Rovani, Vicente Albuquerque Paulo Roberto Matos Neto, Luís Eduardo Oliveira
Maranhão Matos, Matheus Brasil Câmara Monteiro, Lucas
UNIOESTE – Francisco Beltrão – PR – Brazil
Antonissen Lima Verde, David Elison Lima Silva,
UNIOESTE – Francisco Beltrão – PR – Brazil
vicmaranhao@hotmail.com Espártaco Moraes Lima Ribeiro, Gerardo Cristino
vicmaranhao@hotmail.com Filho, Paulo Roberto Lacerda Leal
Case Presentation: A 46 years old male, with type 2
diabetes mellitus. He reports sudden decrease in the Universidade Estadual do Ceará. Sobral CE, Brazil
Case Presentation: 42-year-old male, smoker, with hep-
visual field in the right eye without other associated atitis B. One year ago,suffered injury from trampling.Six paulor30@alu.ufc.br
complaints. After 20 days without improvement, referred months ago, presented insidious, diffuse and daily low
tot he Ophthalmology service, who found a decrease in back pain with progressive worsening relatedwith par- Case Presentation: A 32-year-old immunocompetent
visual acuity and papilledema on funduscopy, without esthesia in perineum, buttocks, thighs and feet.Ten days male patient developed low back pain that, after 7 months,
impairment in oculomotricity and normal neurological ago, reported decreased strength in the lower limbs and evolved with paresthesia of the left lower limb (LL), fol-
exam. In the investigation, with normal laboratory tests, impaired gait. He denies fever and weight loss. The physi- lowed by paraparesis, paraparesthesia, and sphincter
non-reactive viral serology, VDRL (+); FTA-Abs IgM(-) cal examination revealed left paravertebral hypertonia in incontinence. Neurological exam revealed T10 sensory
IgG (+), negative autoantibodies, CSF without pleocytosis L3-L4, pain on distal mobilization in bed, anti-algicand level, muscle strength grade 2/5 in limbs, patellar and
and VDRL (-), requested brain MRI, normal, and orbits paretic gait and ortho support. Proximal strength in right aquileu reflexes grade 4/4, Babinski’s and switchblade
with hypersignal in the optic nerve in the orbital región lower limb 5/5 and distal 0/5, proximal strength in left sign present, and diffuse clonus. Magnetic resonance
on theright. He reports abaut 3 years ago. a popular lesión lower limb 4/5 and distal 1/5, hypoactive and symmet- imaging (MRI) of the spine was performed, which sug-
on the penis, painless, disappearing later, and intermit- rical osteotendinous reflexes, hypoesthesia in the bilat- gested extensive spinal neoplasia, with involvement
tent erythematous cutaneous lesions on the extremities, eral plantar region. The lumbar spine and abdominal of the vertebral body on T4 and significant spinal cord
without medical consultation. He remained in the ward tomography (CT) showed L3-L4 spondylodiscitis, volu- compression. A laminectomy was performed, raising
for 14 days under treatment with Ceftriaxone – HICC minous heterogeneous fluid collection in the left psoas the possibility of tuberculosis (TB) vertebral. The regi-
guidance. After discharge, follow-up at an ophthalmic out- muscle with intrasomatic extension in L2 that reaches men for TB was started for 2 months, until the diagnosis
patient clinic with a marked reduction in eye symptoms. spinal canal compressing nerve roots. The laboratory tests was confirmed with a positive sputum test for BK, and
Discussion: Syphilis can affect the CNS at any stage of its were normal and serological tests VDRL, HIV and IGRA the regimen was extended to 12 months. On the 11th
natural history. Early neurosyphilis, less common, appears were negative. Broad-spectrum antibiotics were started. postoperative day, the anatomopathological examina-
soon after syphilitic infection, and ocular involvement may Ultrasound-guided percutaneous lumbar puncture was tion revealed chronic granulomatous osteomyelitis with
be the first manifestation. As in the case described, in the performed with aspiration of 100 ml of purulent fluid that a tuberculoid pattern, without malignancy criterion. After
presence of isolated inflammatory optic neuritis, there is was sent for culture and showed no growth of bacteria. 5 months, he was readmitted with multiple T1 to T6 lytic
no clinical finding or specific test that differentiates the The CT control presented abscess remission and reduc- lesions and extensive subcutaneous collection, consistent
syphilis lesion from other causes of optic neuritis. Optic tion of material in the spinal canal, besides improving the with an infectious process. Drainage was performed and
nerve involvement can be unilateral or bilateral, leading pain and gait. At discharge, antibiotic therapy was main- antibiotic therapy was started. He had a new infectious
to a decrease in visual acuity, with evidence of optic peri- tained with clindamycin and ciprofloxacin, putti vest and condition 2 months after discharge, requiring another
neuritis, papillitis, neuroretinitis, retrobulbar neuritis and return for follow-up of spondylodiscitis. Discussion: With decompressive laminectomy, drainage of the collection
papilledema and the need to perform a lumbar puncture unspecific symptoms, low back pain due to spondylodis- and an antibiotic regimen for 42 days. Since then, it has
in all cases of neurological or ophthalmological symptoms citis may present with fever, claudication and prostration. evolved with significant neurological improvement, with
and the immediately indicated treatment with antibiot- Psoas abscess, secondary to infectious hematogenous gains in strength and physiological sphincter functions.
ictherapy. If treatment is not instituted properly, cranial lesions, tuberculosis, diabetes, tumors, and trauma, was Discussion: Responsible for 1-2% of TB cases, spinal
nerve injuries can be permanent. Final Comments: The the etiology of disc infection in our case.The diagnosis involvement, more common in immunocompromised
case shows an atypical manifestation of neurosyphilis. In was confirmed by the history, neurological evaluation and patients, results from hematogenous dissemination, with
this way, we emphasize the importance of paying attention imaging tests, with CT being the gold standard.Although the thoracolumbar junction being the most affected site.
to non-specific neuro-ophthalmological disorders and we did not conclusively identify the origin of the abscess, The manifestations are nonspecific, insidious and progres-
calling the attention to this disease that, unfortunately, an association with the trauma previously reported is valid. sive, in order to mimic many other clinical conditions,
still high prevalent in the population. The treatmentwas based on abscess drainage, antibiotic such as paravertebral tumors themselves, which makes
therapy, and lumbar immobilization for follow-up. Final the diagnosis a challenge. MRI is the best imaging test for
Comments: We would like to emphasize the importance this extrapulmonary form of TB, but it is worth noting that
Neuroinfecção of performing an abdominal CTto the diagnosis of spon- radiological tests are not always capable of making this
dylodiscitis caused by the psoas abscess. This condition diagnosis. As a result, the anatomopathological study is
presents with a complex and little-known diagnosis, the gold standard. Treatment lasts 12 months, while the
related with several other pathologies and which, in our prognosis depends, in part, on the degree of established
case, triggered a favorable outcome. neurological impairment. Final Comments: It is import-
ant to be aware of the different presentations of spinal TB,
which can often mimic a malignancy, in order to avoid
Neuroinfecção diagnosis and treatment delay.

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TL 1105453 TL 1105469 TL 1105531

CEREBRAL SCHISTOSOMIASIS: NA HUMAN HERPESVIRUS 7 (HHV-7) POST-COVID LEUKOENCEPHALOPATHY


IMPORTANTE DIFFERENTIAL DIAGNOSIS OF ENCEPHALITIS: A CASE PRESENTATION WITHOUT SEVERE HYPOXIA WITH EARLY
BRAIN TUMOR TREATMENT
Lécio Aragão Souza Morais, Bernardo Gratival
Matheus Gurgel Saraiva, Arthur Felipe Barbosa Gouvea Costa, Lucas Abner Alves, Marcos Baruch Lázaro de Lima, Paulo Eduardo Mestrinelli Carrilho,
Vasconcelos, Rafael de Souza Andrade, Maria Portela Filho, Gessica Almeida Vasconcelos, Liliane João Gabriel Strauch, Rodrigo Bianchi Zancanaro
Yvone Carlos Formiga de Queiroz, Tatheane Couto Rodrigues Soares, Daniele Santos Andrade, Catarina
de Vasconcelos, Daiane Pereira de Farias, Daniel Ester Gomes Menezes, Paula Cely da Silva Torres, HUOP. Cascavel PR. Brazil
Vicente de Siqueira Lima Junior, Luiza Alves Matheus Santana do Rosário UNITOM. Cascavel PR. Brazil
Monteiro Torreão Villarim, Paulo Antonio Farias de pemcarrilho@gmail.com
UNEB. Salvador BA, Brazil
Lucena, Mylena Gaudencio Bezerra
EBMSP. Salvador BA, Brazil
Hospital Metropolitano Dom José Maria Pires. João Case Presentation: A 44-year-old male patient sought
marcosbaruch05@outlook.com medical evaluation due to concentration and memory defi-
Pessoa PB, Brazil
cits initiated three months before He also referred nausea,
matheus_gurgel_pb@hotmail.com Case Presentation: Female, 15 year-old, black, previously excessive irritability, dizziness, and a moderate-intensity
healthy and immunocompetent, presented to the emer- headache in the period. His medical history disclosed a
Case Presentation: A 24-year oldpatient, without comor- gency room with a history of fever, skin rash, headache, previuous mild covid-19 about 5 months before, con-
bidities, started to present in March 2021 with seizures. diarrhea, feet edema and polyarthritis in January/2022. firmed by polymerase chain reaction (PCR). The patient
He was admitted to the servisse with a neurological On the neurological examination, she had drowsiness, did not present hypoxia or great respiratory symptoms
examination without alterations, but when performing mental confusion, psychomotor agitation, memory alter- in the period that needed further care. He had taken, 1
a cranial tomography, hypodensity was observed in the ation, generalized motor epileptic seizures, visual field month before the infection, the first dose of Astrazeneca
right parieto-occipital region, being Hospitalized due to alterations, drooling, urinary incontinence, and objective vaccine and the second dose, 3 months after the disease.
the initial clinical suspicion of a brain tumor, lateron a muscle weakness. A syndromic diagnosis of encephalitis There was no evidence of drug and alcohol abuse, intox-
cranial resonance that showed a swollen lesion involv- was given, and venous acyclovir was prescribed, with no ication with exogen substances, radiation exposition or
ing the subcortical White matter. Of the right superior improvement of the neurological condition. She was then chemotherapy. Physical and mental examination did not
frontal gyrus, characterized by hypersignalonT2/FLAIR referred to a tertiary Hospital facility. On admission, no show any alteration during tests. An encephalic Magnetic
sequences and with tiny nodular foci with hyposignal and changes on the segmental physical examination were Resonance Imaging (MRI) revealed coalescent hyper-
intense post-gadolinium enhancement, without diffusion present. In the neurological examination, she had a intense signal abnormalities in white matter of corona
restriction. In the evaluation of the cérebro spinal fluid, Glasgow Coma Scale of 9 points, mental confusion, mem- radiata, periventricular, frontoparietal and subcortical
there were no changes in cellularity, proteins and glucose, ory changes and global aphasia. Visual acuity examina- temporal lobes in both hemispheres. In addition, in T2/
and it was not possible to perform immunology due to tion, strength, sensitivity, and reflex were not able to be FLAIR sequence, small oval hyperintense abnormalities
unavailability in the service. Based on the findings of the performed. There were no changes in pupillary reflex and after contrast in both thalami, cerebellum’s white mat-
imaging exam, the possibility of cerebral schistosomiasis ocular motility. She was then admitted to the intensive ter, pons, amygdala, and dentate nucleus. There were no
was raised and a biopsy was performed, which showed the care unit and mechanical ventilation was started. Head hippocampi alterations. Blood count, cerebrospinal fluid
presence of Schistosoma mansoni eggs. Treatment with CT, MRI and EEG were normal. Serum and cerebrospinal analysis and serologies were performed, showing no alter-
praziquantel and corticosteroid was performed. Patient fluid (CSF) samples were negative for zika and chikun- ations or plausible causes for the findings. Considering
evolves with neurological examination without alter- gunya viruses and indeterminate for dengue fever virus by the possible causes and the exclusion of the majority
ations and with out new episodes of seizures. Discussion: PCR method. Multiplex PCR, an exam to simultaneously of plausible causes, the diagnostic was given as Acute
Schistosomiasis of the central nervous system (CNS) is identify human adenovirus, cytomegalovirus, enterovirus, Diffuse Leukoencephalopathy secondary to Covid-19.
relatively rare, being called neuroschistosomiasis (NE). erythrovirus B19, human herpesvirus 7 (HHV-7), human Based on previous reports, it was initiated treatment with
It occurs mainly in the spinal cord, accounting for about parechovirus, human herpesvirus 6, Epstein-Barr virus, corticotherapy. After 10 days of treatment, the patient
6 to 13.4% of myelopathies in endemic areas. Intracranial herpes simplex virus 1, herpes simplex virus 3 and vari- referred great improvement of symptoms, with remission
localization, notably in the cerebral hemispheres, is much cella-zoster virus, was performed and positive in the CSF of dizziness. This pathology is mostly related to microhe-
rarer. NE can be clinically manifested by meningo myelo- for HHV-7 and negative for the others tested. Discussion: morrhages, severe hypoxia and, during the pandemic,
radiculitis, myelitis, radiculitis, or by the cerebralor med- Primary HHV-7 infection usually occurs during childhood associated with long term Hospitalization and mechan-
ullary pseudotumor form. The most common presenting and presents mainly as a sudden exanthema, with a later ical ventilation. In this case, the patient showed demye-
symptoms are cognitive impairment, headache, seizures, latency state and possible reactivation in immunodefi- linating patterns associated with a rare complication of
and focal neurologic deficits. Final Comments: NE with ciency. Final Comments: However, although rare, cases sars-cov2 infection with direct lesions of the nervous cells
cerebral involvementis very rare, but it should Always be of encephalitis and post-infectious autoimmune encepha- of the brain. The diagnostic of this pathology and timely
suspected in patients with images suggestive of tumors litis related to HHV-7 in immunocompetent patients have treatment is difficult, considering negative findings in
and who live in endemic areas. Early and adequate treat- already been reported in the literature. This possibility physical and mental exams as well as laboratorial testing.
ment is of Paramount importance to minimize CNS tissue should be considered when the main causes of enceph- Most patients do not have access to MRI and probably will
damage and prevent chronic sequelae. alitis are ruled out. In such cases, multiplex-PCR should not receive an appropriate diagnostic. The probability of
be performed, allowing for a rapid and comprehensive certain medical care is lowered even more if examinators
etiological diagnosis. restrict the occurrence of the pathology only in critical ill
Neuroinfecção patients with long exposure to hypoxia.
Neuroinfecção
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XXX Congresso Brasileiro de Neurologia

TL 1105534 TL 1105551 TL 1105570

ACUTE DISSEMINATED SEPTIC THROMBOPHLEBITIS OF ONGOING CENTRAL NERVOUS SYSTEM


ENCEPHALOMYELITIS (ADEM) AFTER THE CAVERNOUS SINUS AFTER A DEMYELINATING MULTIPLE ACTIVE LESIONS
COVID-19 INFECTION: CASE PRESENTATION HYMENOPTERA ACCIDENT: POST MRNA VACCINE: CLINICAL AND SERUM
A CASE PRESENTATION BIOMARKER MONITORING
Anderson Matheus de Lima Barbosa, Gilberto José
de Melo Neto, Bruna Tavares Falcão, João Pedro Igor Fortunato da Silva, Renato Ortolani Marcondes Jéssica Vasques Raposo-Vedovi, Andreza Salvio
Rosal Miranda, Elizeu Pereira dos Santos Neto de Castro, Iara Proença Xavier, Juliana Marilia Lemos, Renan Amphilophio Fernandes, Larissa
Pereira de Oliveira, Argel Gava de Jesus Araujo Duarte, Milene Teixeira Ataíde, Vinicius
Unifacid. Teresina PI, Brazil
Gabriel Coutinho Faria, Valeria Coelho Santa Rita
Hospital São Marcos. Teresina PI, Brazil Universidade José do Rosário Vellano – Passos – Pereira, Assuncion Martinez Liem, João Gabriel Dib
MG – Brazil Farinhas, Soniza Vieira Alves-Leon
andersonbarbosa7570@outlook.com
Hospital Universitário Alzira Velano – Alfenas – MG
– Brazil Universidade Federal do Estado do Rio de Janeiro.
Case Presentation: Woman, 56 years old, with a previ-
Rio de Janeiro RJ, Brazil
ous history of infection by covid-19 for a month. Denies igor.fortunato@aluno.unifenas.br Universidade Federal do Rio de Janeiro. Rio de
smoking, alcoholism or drug addiction, works in an
Janeiro RJ, Brazil
accounting office, goes to the emergency service with a Case Presentation: A 23 year old male patient, previously
report that 24 hours ago she started left hemihypoesthesia healthy, was admitted to the emergency department jessicavasquesr@gmail.com
and left rhyme deviation, with no additional complaints. reporting an insect bite on the nasal dorsum 05 days ago.
On neurological physical examination, the patient has Since then he had pain, edema and hyperemia in bilateral Case Presentation: During COVID-19 pandemia, the
right peripheral facial palsy and left hemihypoesthesia. peri-orbital region, worse to the left, followed by decreased central nervous system (CNS) involvement was associated
During the investigation of the case, computed tomog- general condition, myalgia, headache, fever, neck stiffness with a more severe outcome. In face of these cases, the
raphy did not show any changes. Brainstem audiometry and mental confusion. Laboratory tests showed signs of emerging challenges are the differential diagnosis with
(BERA) showed bilateral retrocochlear dysfunction. CSF bacterial meningitis, and treatment with Ceftriaxone was inflammatory demyelinating diseases, mainly the multi-
with a slight increase in protein. Magnetic resonance initiated with improvement of the meningeal picture. ple sclerosis (MS). Patients with intrathecal synthesis of
imaging (MRI) showed a right paramedian image at the Computed tomography (CT) of the skull showed signs immunoglobulin G (IgG) and oligoclonal bandas (OCB)
medulla-pontine transition, without expansive or atrophic of thrombosis of the transverse sinus, sigmoid and left during the first demyelinating event distributed in the
features. Raising the hypothesis of acute disseminated internal jugular vein, confirmed by magnetic resonance space, excluding other etiologies, fulfill diagnostic criteria
encephalomyelitis (ADEM). We started pulse therapy imaging of the brain. Anticoagulation therapy was started of MS allowing us initiate treatment in the more favorable
with methylprednisolone 1g/day for 05 days. The patient with warfarin. Arteriography showed a carotid-cavernous therapeutic window. Otherwise, vaccines can trigger
evolved in good general condition, eupneic, phasic, con- fistula, but it was decided to maintain anticoagulation immune response, probably in individuals with genetic
scious and oriented; also reports improvement in the due to extensive central venous thrombosis. Besides the susceptibility, and relapsing events need, in specific cases,
sensation of paresthesia in the left side and of facial paral- neurological picture, cavitary lesions were found on chest to be addressed with modifying drug therapies (DMD).
ysis. Discussion: Acute disseminated encephalomyelitis CT and bronchoscopy was requested for investigation, Besides clinical and MRI activity serum biomarkers,
(ADEM) is an inflammatory demyelinating condition of the however the result of the biopsy showed only signs of light chain neurofilament (NfL) can contribute to deci-
central nervous system, which is usually caused by viral inflammation with inconclusive result. After suspension sion making. Here, we presented a case of a 19-years-old
infections, however, it can also be triggered by vaccines of anticoagulation 24 hours before the bronchoscopy, female that has been followed up since December 2021,
or not have an apparent cause. Although its incidence the patient evolved with motor aphasia and cranial CT with neurological exams registers, EDSS, neuroimaging,
is not fully known, it is known that it is an uncommon showed foci of ischemia. After returning to anticoagula- cerebrospinal fluid and plasma analysis. Single molecule
disease in adults, occurring more frequently in children. tion, there was improvement of neurological deficits. After array (SIMOA) was used to analyze neurofilament light
The classic picture of ADEM occurs, on average, 26 days stabilization and absence of active infectious signs, the level chain (NfL) during the last 6 months. Fifteen days
after an infectious condition, manifesting itself with patient was referred to vascular neurosurgery for fistula after mRNA COVID-19 vaccine, she presented paresthesia
acute multifocal neurological symptoms, often evolving repair. However, a new pre-surgical arteriography showed in four limbs, bladder incontinence, appendicular ataxia,
with vertiginous deterioration. Most patients have motor total and spontaneous correction of the fistula through visual impairment and mental confuse state. Cranium
and/or sensory deficits, which may be accompanied by thrombosis of its path and also revascularization of central MRI showed multiple white and grey matter lesions,
other signs and symptoms such as headache, malaise, venous thrombosis. Final Comments: Septic cavernous some of them with mass effect, brainstem and spinal
ataxia, aphasia and nystagmus. The diagnosis is made sinus thrombosis is an uncommon and serious compli- cord lesions, various with gadolinium enhancement.
from clinical presentation and history, neuroimaging and cation of facial infections, and occurs when the infected CSF showed 4 cells, protein 38mg%, no viral, fungus or
CSF analysis. The prognosis of the patients with ADEM areas are close to the nose because this region is drained bacterial identification, and Reiber and Felgenhauer
is mostly favorable, with total or almost complete recov- by the ophthalmic veins. About 20 to 30% of cases have diagram showing intrathecal synthesis of Kappa free
ery after approximately 6 months of presentation. Final fatal outcomes. The importance of this report lies in the chain. For the last six months, she remained with active
Comments: Thus, this clinical case is uncommon due to rarity of the case associated with the singularity of the ongoing disease, presented new MRI lesions, several
the low frequency of ADEM after infections, especially in event of spontaneous fistula correction. Hospitalization events, receiving intravenous methyl-
adults. In addition, this is a rare manifestation resulting prednisolone (IVMPN) treatment and plasmapheresis,
from COVID-19 infection, and it can be a condition that leading to a partial improvement. In december-13-2021,
leaves sequelae. Neuroinfecção NfL level was 247.94pg/mL; in January-17-2022, 216.24pg/
mL and, in March-07-2022, 271.38pg/mL. The NfL level
is correlated with ongoing disease, new MRI lesions and
Neuroinfecção the EDSS score. In May 2022, we initiated treatment with
Natalizumab. Acute demyelinating events post vaccine are
generally monophasic. This patient fulfills aggressive MS
criteria and differential diagnosis with recurrent ADEM
here is a challenge to manage treatment. In this context,
NfL level, besides clinical and MRI activity contributed
to our decision to treatment onset.

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XXX Congresso Brasileiro de Neurologia

TL 1105581 TL 1105647 TL 1105668

COMMUNITY-ACQUIRED PSEUDOMONAS CRANIAL NERVE INVOLVEMENT BY NEUROFILAMENT LIGHT CHAIN (NFL),


AERUGINOSA MENINGOENCEPHALITIS: A NEUROSYPHYLIS GLIAL FIBRILLARY ACIDIC PROTEIN
CASE PRESENTATION (GFAP), UBIQUITIN CARBOXY-TERMINAL
Déborah Inayara Mendes Tenório de Albuquerque,
Vanessa de Freitas Moreira, Andreas Batista Schelp, HYDROLASE L1 (UCH-L1) ANDTAU PROTEIN
Luiza de Lima Beretta, Stefano Machado, Pedro
Miguel Mendonça Couto, Breno Gabriel Rodrigues Davi Vargas Freitas Teixeira, Amanda Loureiro de LEVELS IN A SEVERE COVID 19 PATIENT
Queiroz, Letícia da Silva Alves, Wender Rodrigues Toledo Troian, Cledson Gonçalves Ventura, Alexia WITH DOUBLE HOSPITALIZATION AND
Teodoro, Arthur Costa Nascimento, Rogerio Rizo Carneiro de Almeida, Beatriz Marques dos Santos, FAVORABLE OUTCOME
Morales, Diogo Fernandes dos Santos Roberta Arb Saba Rodrigues Pinto, Erick Dupont
Helena Ferreira França, Elisa Gutman Gouvea,
Universidade Federal de Uberlândia. Uberlândia Hospital do Servidor Público Estadual de São Paulo. Andreza Salvio Lemos, Jessica Vasques Raposo-
MG, Brazil São Paulo SP, Brazil Vedovi, Renan Amphilophio Fernandes, Larissa
Araujo Duarte, Carlos Henrique Ferreira Ramos,
luizaaberetta@gmail.com deborah.albuquerque@icloud.com Carlos Henrique Ferreira Ramos Filho, Luciane
Almeida Amado-Leon, Soniza Vieira Alves-Leon
Case Presentation: A 66-year old woman with type 2 Case Presentation: A 67 yo female patient presented with
diabetes mellitus, chronic obstructive pulmonary disease right peripheral facial paralysis, followed by scotomas in Universidade Federal do Estado do Rio de Janeiro.
(COPD) and chronic sinusitis was admitted to our institu- the left eye (LE) and decreased visual acuity. On evalua- Rio de Janeiro RJ, Brazil
tion presenting with a 8-day history of continuous holocra- tion, she presented ocular pain and anterior uveitis, low Hospital Universitário Clementino Fraga Filho –
nial headache, nauseas and vomiting, without fever. She visual acuity (20/100) and relative afferent pupillary defect Universidade Federal do Rio de Janeiro. Rio de
evolved with disorientation and seizures. Computed in the LE, with scotomas in the left temporal hemifield, Janeiro RJ, Brazil
tomography of the brain was normal and cerebrospinal marked papillitis and bilateral red desaturation. There Universidade Estadual do Rio de Janeiro. Rio de
fluid (CSF) showed pleocitosis with a lymphocytic pre- was also mild peripheral facial paralysis and right sen- Janeiro RJ, Brazil
dominance. Ceftriaxone, ampicillin and acyclovir were sorineural hearing loss. She also had erythematous, scaly Fundação Oswaldo Cruz. Rio de Janeiro RJ, Brazil
started empirically promptly. Pseudomonas aeruginosa and diffuse infiltrative plaques and papules in the lower
limbs, hypogastric, paravertebral and inframammary helena.faf33@gmail.com
was identified in CSF culture, and the antibiotic regi-
men was modified for cefepime, oxacillin and metro- regions. The serum Veneral Disease Research Laboratory
(VDRL) test was positive, with a titer of 1/512, and the Case Presentation: Coronavirus disease 19 (COVID-19) is
nidazole. Cranial magnetic resonance showed bilateral
Microparticle Chemiluminescence for Syphilis (CMIA) an infectious disease caused by SARS-CoV-2 (Severe Acute
frontal and sphenoidal sinusopathy, discontinuity of the
also had a positive result, of 24.21. In the cerebrospinal Respiratory Syndrome related to Coronavirus 2). Most
sphenoid roof, without cerebrospinal fluid insinuation,
fluid, mild pleocytosis was observed, with 9 cells/dL, SARS-COV-2 infected people experience mild self-limiting
purulent collections in posterior ventricles, empyema in
normal total protein (12 mg/dL), reagent FTA-ABS test respiratory illness. Some, however, become seriously ill
the left temporal and occipital regions. A new CSF three
and negative VDRL. Anatomopathological result of skin and require medical attention. Here we present the case
days later maintained culture with Pseudomonas aeru-
biopsy pointed lymphohistiocytic dermatitis, suggesting of a 71-years-old male patient with comorbidities (chronic
ginosa, so meropenem and ciprofloxacin were started.
treponematosis. Brain Magnetic Resonance demonstrated arterial hypertension, diabetes mellitus, previous stroke),
Antibiotic treatment lasted eight weeks, with a control
enhancement in the auditory canals, greater on the left. and previous history of admission in Intensive Care Unit
lumbar puncture without bacteria growth. Sphenoid
The patient was treated with intravenous crystalline pen- (ICU), intubation and coma due to COVID-19 in 2020.
and ethmoid sinusotomy was performed by the otorhi-
icillin G for 14 days, with improvement of the skin lesions One year later, in July 2021, he had been admitted to the
nolaryngology team. She was discharged after one month
and visual acuity. Discussion: Neurosyphilis can occur Clementino Fraga Filho University Hospital ICU for the
with mild paresthesia on the left hemiface, for outpatient
at any stage of the disease, as the bacterium Treponema same reason, when also presented myoclonic seizure;
follow-up. Discussion: Gram-negative bacilli are an
pallidum spreads throughout the body early in the infec- at that time, he was intubated for 2 weeks. Positive PCR
uncommon etiology of community-acquired meningitis
tion, including the central nervous system. The patient for COVID-19 confirmed a second infection. Cranium
in adults, with the majority of the cases occurring in neo-
in question had involvement of the cranial nerves most Computed Tomography (September 2021) showed no
nates or infants. Escherichia coli accounts for the most
commonly reported in cases of neurosyphilis (II, VII acute changes but diffuse atrophy. MRI wasn’t possible
cases, but there are less frequent pathogens described,
and VIII), at the secondary stage of the disease. Syphilis at the time. During the second ICU admission (2021), his
such as Pseudomonas aeruginosa, Acinetobacter and
diagnosis demands a positive treponemal test and a pos- blood sample was collected, so we could investigate neu-
Enterobacter. Non-hematogenous bacterial invasion of
itive non-treponemal test, while neurosyphilis diagnosis rological and immunological biomarkers (Neurofilament
CSF occurs in situations of compromised integrity of the
requires a compatible clinical picture and cerebrospinal light chain – NFL; Glial fibrillary acidic protein – GFAP;
blood-brain barrier, such as neurosurgery, mastoiditis,
fluid. CSF in neurosyphilis is marked by pleocytosis and Ubiquitin carboxy-terminal hydrolase L1 – UCH-L1; TAU
sinusitis and malformations. We described a rare case
hyperproteinorraquia. CSF’s VDRL is highly specific with a protein; Interleukin-22 – IL-22), those were measured
of an elderly patient who has been diagnosed with com-
sensitivity between 30-70%, therefore, it does not exclude using SIMOA (SIngle MOlecule Array), an ultrasensitive
munity-acquired Pseudomonas aeruginosa meningoen-
the diagnosis. CSF treponemal test is highly sensitive technique for biomarkers quantification in peripheral
cephalitis and a medical history of chronic sinusitis and
but not very specific for the disease. Final Comments: blood. The patient showed the following levels for these
COPD. This patient presented with headache and confu-
Neurosyphilis is a manifestation that can occur at any biomarkers: NFL- 131,23pg/mL; GFAP- 615,12pg/mL;
sional state, the last may be the only clinical feature in the
stage of the disease, however, it is treatable and, if diag- UCHL1- 115,24pg/mL; TAU- 1,36pg/mL; IL-22- 385.71pg/
elderly.Final Comments: Pseudomonas aeruginosa is a
nosed and treated early, serious sequelae can be avoided. mL. Most of these values are considered high, when com-
rare cause of meningoencephalitis and has a significant
pared to age-matched control group, with the reference
morbidity and mortality. Further studies are needed to
values of GFAP- 192,75pg/mL; NfL- 17,52pg/mL; Tau-
investigate if it is more common in patients with COPD
and chronic sinusitis than in the general population due Neuroinfecção 1,89pg/mL; UCHL1- 41,56pg/mL and IL-22- 7.49pg/mL.
The only biomarker considered at normal levels was TAU
to the bacterial colonization in this profile of patients.
protein, in agreement with expected due to his preserved
memory and absence of dementia even after the second
Neuroinfecção COVID-19 event. However, the GFAP, NfL, and UCHL1
were significantly higher when compared to the control.
These are important markers of neuroinflammation and
may be related to the myoclonic seizure and coma. It is
important to highlight that he presented high IL-22 levels
and that this IL has been considered a protective factor
during SARS-COV-2 infection, and probably an import-
ant biomarker for this favorable outcome. These intense
variations together with the clinical picture of the patient
may be an indication of the relevance of these biomarkers
for the progression and outcome of COVID-19.

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XXX Congresso Brasileiro de Neurologia

TL 1105688 TL 1105718 TL 1105724

MIMICS OF NERVOUS SYSTEM CHAGAS TUBERCULOUS PACHYMENINGITIS SPASTIC PARAPARESIS IN A 45-YEAR-OLD


DISEASE REACTIVATION IN HEART PRESENTING AS MOTOR NEURON DISEASE WOMAN POST MILD SARS-COV-2 INFECTION
TRANSPLANTATION PATIENTS
Andre Lopes Valente, Daniel Natalio Gauss Thomas Vieira de Paula, Lucas Scárdua Silva,
Rebecca Ranzani Martins, Lucas Assis Santos Yankelevich, Filipe Pereira Sarmento, Marianna Vinicius de Oliveira Boldrini, Bárbara Juarez Amorim,
Souza, Iori Rodrigues Junqueira, Helio Rodrigues Pinheiro Moraes de Moraes, Tacio Luis Cavalcante Clarissa Lin Yasuda, Lucas de Melo Teixeira Branco
Gomes Coradine, Lucas de Oliveira Cantarutti Guida,
Sandro Luis de Andrade Matas Universidade Estadual de Campinas. Campinas SP,
Hospital das Clínicas da Universidade de São Paulo. Brazil
São Paulo SP, Brazil Universidade Federal de São Paulo. São Paulo SP,
Brazil thomasdepaula92@gmail.com
rebecca.rmartins@hc.fm.usp.br
andrevalentelop@gmail.com Case Presentation: We present the case of a 45-year-
Case Presentation: A 48-year old male underwent heart old woman without previous comorbidities, who sought
transplantation for heart failure secondary to Chagas Case Presentation: A 63-year-old man presented with the emergency unit of our university Hospital with a
disease. On the second postoperative day, he had a ton- bilateral frontoparietal headache that progressed within 6 1-month history of asymmetrical paresthesia in lower
ic-clonic seizure and developed hemiparesis and aphasia. months to generalized weakness, hoarseness, dysphagia, limbs, worse in the right side, progressing to both supe-
CT revealed superior sagittal sinus thrombosis, venous left hemifacial neuralgic pain, unintentional weight loss rior limbs after a few days. She had a recent diagnosis
infarct and subarachnoid hemorrhage. Despite ade- of 20kg and no history of febrile illness. On examination, of SARS-CoV-2 infection, confirmed through a positive
quate anticoagulation, anti-seizure drugs adjustments there was cranial nerve palsies of nerves V, VII, VIII, IX, X, rt-PCR performed ten days before the Hospital admis-
and hemorrhage reabsorption, seizures and neurologi- XI XII on right side and V on left side; tongue and upper sion, with mild respiratory symptoms. Along the following
cal deficit persisted. MRI revealed a necrohemorrhagic limb fasciculation, distal amyotrophy in upper limbs and week, she presented ascending weakness (worse on the
lesion suggestive of chagoma and a hypoplastic superior global hyperreflexia. Brain MRI showed pachymeningeal right side), spasticity and weakness in the lower limbs
sagittal sinus. Another 48-year old male with a history of thickening in the left middle cranial and posterior fossa, with necessity of a wheelchair and marked constipation.
Chagas disease started, 24 days after heart transplanta- nodular lesions with low T2 signal in cerebellar vermis Physical examination at admission evinced: asymmetrical
tion, a confusional state in the absence of clinical findings and right cerebellar hemisphere with growth towards spastic paraparesis with brisk reflexes, ankle clonus and
that could justify a delirium followed by seizure two days cerebelomedullary cistern and fourth ventricle. Cervical Babinski sign, diminished sense of vibration and pro-
later. Initial cerebrospinal fluid (CSF) analysis was nor- spine MRI was normal. Cerebrospinal fluid examination prioception in lower limbs and absence of sensitive level.
mal and CT scan suggested posterior reversible enceph- showed pleocytosis of 52/mm3 with lymphocytic pre- We admitted her to the neurology ward, proceeded with
alopathy syndrome (PRES). Tacrolimus was suspended dominance, hyperproteinorraquia and no glucose con- brain/whole spine magnetic resonance imaging (MRI)
and improvement was noticed afterwards. He was later sumption. Ziehl-Neelsen, India ink, Gram stain and Xpert and cerebrospinal fluid (CSF) analysis. The MRI did not
readmitted confused and hemiparetic. Repeated CT MTB negative. Serologies for syphilis and HIV negative. present any significant alterations and the CSF showed a
showcased a new lesion with imprecise limits, vasogenic Electroneuromyography (EMG) presented chronic and mild lymphocytic pleocytosis that didn’t remain in further
edema, cortical necrosis and hemorrhage indicative of subacute denervation of preganglionic origin of bulbar, evaluation. Specific autoimmune panels and infectious
chagoma. T. cruzi was identified in both CSF analyses. cervical, thoracic and lumbosacral myotomes, without investigations were negative. The hypothesis of autoim-
Discussion: Central nervous system (CNS) reactivation spontaneous activity. Based on clinical and paraclini- mune myelopathy was made and she received high dose
in a rare complication of heart transplantation in Chagas cal data, empiric treatment for tuberculosis was started. (5g) methylprednisolone for five consecutive days, with
disease. It is manifested as meningoencephalitis (altered Diagnosis was confirmed by bronchoalveolar lavage with poor response. After multiple follow-up MRI scans (1,
mental status, seizures, focal deficits) or tumor-like lesions a positive Xpert MTB/RIF test. Despite therapy, patient 2, 5 and 6 months from initial symptoms) and a electro-
called chagomas (causing seizure, focal deficits). Single presented clinical complications and died after 2 months. neuromyography with unremarkable findings, we made
or multiple necrotic-hemorrhagic nodular lesions in Discussion: Tuberculous hypertrophic pachymeningitis the diagnosis of MRI-negative post-COVID myelopathy.
white matter can be seen in neuroimage. Diagnosis is usually presents with cranial signs and symptoms such as Discussion: After COVID-19 pandemic, there have been
usually made by a combination of clinical symptoms and headache and cranial nerve palsies, or spinal symptoms rare reports of inflammatory myelopathy after SARS-CoV-2
detection of the parasite in blood, CSF or cerebral tissue such as radicular pain. Reported patient showed progres- infection. These patients typically had asymptomatic or
biopsy. Treatment is recommended in asymptomatic or sive cranial nerve palsies and on brain MRI, the extensive mild-moderate COVID-19 disease and absence of radio-
symptomatic patients. Benznidazole is the first line of dural thickening involving the skull base explains all the logic evidence of structural spinal cord abnormality, which
treatment in the recommended dose of 10-15mg/kg/day, cranial nerve involvement. He also had signs of upper differs from previous reports that showed myelopathy in
even though there are few studies on its interaction with and lower motor neuron involvement without radicular Hospitalized patients with severe COVID-19 disease and
immunosuppressive drugs. Although there is a relevant pain, sensory deficits or sphincter complaints, which is needs of critical care. This is a recently described entity,
risk of chagas reactivation, transplant outcomes of end- not typical of tuberculous myeloradiculopathy. On cer- with only a few reports and unknown pathophysiology
stage Chagas cardiac disease are usually similar to other vical spine MRI there was no thickening or enhancement and prognosis. Up to date there is no effective treatment
transplantation indications. Final Comments: We pres- of meninges, but EMG indicated symmetric pre-gangli- described, except rehabilitation. Final Comments: This
ent two heart transplantation neurological complications onic involvement of nerves, probably secondary to a case adds with prior reports and reinforces the need to
that were first diagnosed as central venous thrombosis radiculopathy only of the anterior root. Our case shows increase clinical suspicion in presentations such as this
and PRES, but during follow-up were recognized as CNS that this topography should be considered in patients in which there is a compatible epidemiological history
manifestation of Chagas disease. This report highlights who presents with motor neuron disease and have some and absence of MRI findings and raises the urge for more
potential clinical and radiologic findings and the impor- alarming features such as new onset headache. Final studies to better comprehension of pathophysiology and
tance of Chagas disease reactivation as a mimic of other Comments: We presented a case of tuberculous pachy- possible therapies.
neurological diseases. meningitis with extensive cranial nerve involvement and
moto neuron symptoms.
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MENINGITIS AND SPINAL ARACHNOIDITIS DISSEMINATED HISTOPLASMOSIS FIRST REPORT OF HEPATITIS C PATIENT
NEUROTUBERCULOSIS PRESENTATION: THE AS A DIAGNOSTIC CHALLENGE IN AN WITH NEUROMYELIT OPTIC SPECTRUM
CHALLENGE OF DIAGNOSIS IMMUNOSUPPRESSED PATIENT DISORDER ANTI-AQP4 ANTIBODIES
(+) AND REMARKABLE HIGH LEVEL OF
Henrique Amancio Ferreira, Andre Filipe Lucchi Catarina Sodré Castro Prado, Isadora Versiani,
Rodrigues, Bianca Gomes Mazzoni, Davi Teixeira Matheus Nunes Ferreirinha Leite Castro, Gihana NEUROFILAMENT LIGHT CHAIN
Urezêdo Queiroz, João Victor Lage Guerra, Elienay Larissa Soares, Mariana Soares Freitas Tamy, Andreza Salvio Lemos, Larissa Araujo Duarte,
Cassio Oliveira, Rodrigo Santiago Gomez, Breno Priscila Santos Mageste, Raimundo Marcial Brito Milene Ataíde Teixeira, Jéssica Vasques Raposo-
Franco Silveira Fernandes Neto, Camila Pupe Vedovi, Renan Amphilophio Fernandes, João Gabriel
Hospital das Clínicas da UFMG. Belo Horizonte MG, Universidade Federal Fluminense. Niterói RJ, Brazil Dib Farinhas, Valeria Coelho Santa Rita Pereira,
Brazil Assuncion Martinez Liem, Roberto de Andrade
isadora.versiani@gmail.com Medronho, Soniza Vieira Alves-Leon
henrique3p@hotmail.com
Case Presentation: A 38-year-old female patient with a Universidade Federal do Estado do Rio de Janeiro,
Case Presentation: A previously healthy 43-year-old history of systemic lupus erythematosus presented with Universidade Federal do Rio de Janeiro. Rio de
man, who worked as a sand extractor in river benches, spastic paraparesis, cognitive dysfunction and urinary Janeiro RJ, Brazil
was admitted to the Emergency Department, presenting incontinence. She underwent head CT and MRI, which andrezaslemos@gmail.com
with paraparesis and urinary retention. He had previously showed communicating hydrocephalus. CSF analysis
sought medical care due to fever and headache four days revealed monocytic pleocytosis (45 nc/µl), hypoglycor- Case Presentation: Hepatitis-related viruses usually stim-
earlier. Both cranial and thoracolumbar computerized rhachia (12 mg/dL), significant increase in proteins (733 ulate extra hepatic manifestations in most of the cases. An
tomography scan were normal. Cerebrospinal fluid (CSF) mg/dL) and was negative for cryptococcus, geneXpert, important representative of these viruses is the hepatitis
revealed the following: Glucose <20mg/dL, 96 white blood VDRL and bacterial and fungi cultures. Chest and abdo- C virus (HCV), which is also associated to chronic silent
cells/mm³ (WBC) – 75% lymphocytes, protein 536mg/dL, men CT evidenced calcified perihilar and abdominal infections that can last several years before a diagnosis,
negative nucleic acid amplification test (NAAT) for tuber- lymph nodes, most indicative of tuberculosis (TB) or sar- leading to cirrhosis and hepatocellular carcinoma. Our
culosis (TB), negative Ziehl–Neelsen staining and nega- coidosis. After full workup and multiple blood and CSF aim here is to present a rare phenotype of extra hepatic
tive direct microscopy for acid-fast bacilli. Extra neural tests, TB was ruled out and the patient was treated with manifestation involving nervous system, the neuromyelitis
sites of TB disease were excluded after screening exams corticosteroids and cyclophosphamide, with no improve- optical spectrum disorder (NMOSD), and the correlation
as well as neoplastic, inflammatory and other infectious ment. Despite regular immunosuppression, she contin- with plasma biomarkers level of neurodegeneration. We
diseases, including HIV. Magnetic resonance imaging was ued to deteriorate. New CSF analysis kept same previous identified a 68-years-old female patient who has been
performed and revealed thick diffuse leptomeningeal pattern. Blood radial immunodiffusion was positive for diagnosed for the hepatitis C in 2014, with a sustained
enhancement, ischemic infarcts in basal ganglia, obstruc- Histoplasma capsulatum. Liposomal amphotericin B was high viral load along the years (~10 UI/mL). In 2021, she
tive hydrocephalus and longitudinal extensive transverse started, followed by oral itraconazole. After three months received the diagnosis of NMOSD, while presenting HCV
myelitis, from T8 to T11. Due to high suspicion, standard of treatment, she presents with mild cognitive deficit and detectable in serum and symptoms related to this viral
anti-tuberculous therapy was prescribed for presumed discrete gait disturbance. Discussion: Histoplasma cap- infection. By the time, her blood sample was collected
TB. During treatment, six CSF samples were collected, sulatum incidence rate has increased with the advance of and evaluated for anti-MOG (Myelin-Oligodendrocyte
showing progressive decrease of Glucose, protein and immunosuppressive therapies in recent years, especially in Glycoprotein) and anti-AQP4 (AQuaPorin 4) antibodies
WBC levels. NAAT, direct microscopy and staining were endemic areas. Up to 10% of patients develop disseminated detection by CBA (Cell-Based Assay), which showed pos-
negative in all samples. Despite medical assistance, patient histoplasmosis via hematogenous dissemination, leading itivity for anti-AQP4 antibodies. Neuroimaging showed
died seven weeks after symptoms onset. Two weeks after to meningitis, associated vasculitis, focal brain or spinal extensive lesion involving of brainstem and extensive
his death, Mycobacterium tuberculosis was detected in lesions, or hydrocephalus. Diagnostic tools are mostly central spinal cord lesion from cervical to l dorsal level.
CSF cultures. Discussion: Central nervous system (CNS) nonspecific, and diagnosis relies on strong clinical sus- During the onset of NMOSD, she was also presented uri-
involvement is considered one of the most severe mani- picion, which may be a challenge. For our patient, it came nary infection, hemorrhagic gastritis and high HCV viral
festations of extra-pulmonary TB. Immunosuppression is up after ruling out TB, sarcoidosis and lupus hydroceph- load, followed by several Hospitalization events, with
an important risk factor. For instance, HIV/AIDS patients alus. CSF analysis usually shows monocytic pleocytosis, active disease since then. For a better understanding of this
have a five-fold increase in the likelihood of having CNS elevated protein and low glucose. CSF culture has poor case, we evaluated neurofilament light chain (NfL) levels
involvement. Despite being highly sensitive and spe- sensitivity, and antigen and antibody testing in CSF are using SIMOA (Single Molecule Array). The plasma NfL
cific, negative NAAT or any other test should not exclude not widely available in our scenario. However, urine and level in 2022 was 365.51 pg/mL (SD=48.39) that compared
the diagnosis. The present case presentation is relevant blood antigen testing are available, sensitive and highly with matched age control group was very high (~11.73
because the rapid TB test, NAAT, which is meant to speed specific. Pharmacologic treatment should be maintained pg/mL). This result corroborates the severity findings for
up diagnosis and to guide a better clinical approach, was, for at least 1 year, until CSF profile is resolved. If possible, patients in neuroinflammatory and neurodegenerative
in six samples, negative, despite the high suspicious for it is ideal to stop immunosuppressive therapies. Relapses processes associated to viral trigger and can contribute
mycobacterium disease. Final Comments: Definitive are frequent and may require prolonged treatment. Final to better understanding the large spectrum of NMOSD.
diagnosis of CNS TB is difficult and may take weeks. This Comments: Untreated histoplasmosis is associated with The NfL levels can contribute as biomarker for neuroin-
case shows the importance of valuing all the clinical con- high morbidity and mortality rates. Diagnosis is critical flammatory and neurodegenerative disease, including
ditions that suggest neurotuberculosis when approaching for adequate treatment implementation and better out- those associated to viral trigger. Here, we show the first
a patient. Despite the availability of modern and rapid comes. It is even more important to accurately diagnose case of HCV patient positive for anti-AQP4 antibodies,
molecular tests, diagnosis should not rely solely on them, fungal infection in patients with autoimmune diseases, reinforcing that there is a wide neuromyelitis spectrum
and treatment should not be postponed. since treatment plans are opposite. disorders associated to AQP-4 antibody identified after
different viral trigger, suggesting a probable relationship
between viral infections and neurological manifestations.
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BILATERAL THROMBOSIS OF THE MIDDLE PROGRESSIVE MULTIFOCAL NEUROLOGIC MANIFESTATION CAUSED BY


CEREBRAL ARTERY BY NEUROSYPHILIS: LEUKOENCEPHALOPATHY: AN ATYPICAL WEST NILE VIRUS: A CASE PRESENTATION
AVCI PRESENTATION
Nathalia Watanabe, Guilherme Silva Soares, Luiz
Tatiane Arroyo Lopes Alves de Jesus, Amanda Leticia Pedro de Souza Domingues, Mariana Mendes Gustavo Breneissen Santos, Paula Fiuza Rodrigues
Andre, Arthur Coelho Moura Marinho, Nathalye Bergamini, Mariana Floriano Luiza Piva, Nalcer de Medeiros, Pedro Vinicius Brito Alves, Victoria
Fernanda Pedroso Dircksen, Marcelo Simplicio Eduardo Canedo de Aguiar, Mateus Pradebon Veiga Ribeiro Gonçalves, Paula Baleeiro Rodrigues
Carvalho, Lorena Fernandes Kronbauer, Damacio Tolentino, Kennedy Soares Linhares Silva, Luisa Silva, Mauricio Silva Teixeira
Ramon Kaimen-Maciel Pacheco Avezum, Flávio Vieira Marques Filho, Maria
Hospital Sírio-Libanês, São Paulo SP, Brazil
Sheila Guimarães Rocha, Júlian Letícia de Freitas
Irmandade Santa Casa de Londrina. Londrina PR,
nathalia.watanabe@hotmail.com
Brazil Hospital Santa Marcelina. São Paulo SP, Brazil

arroyotatiane@gmail.com marianaflpiva@gmail.com Case Presentation: A 39 years old male, physician, trav-


eled to Egypt recently, where he presented odynophagia,
Case Presentation: Male DC 61 years, complaint of right Case Presentation: A 45-year-old woman, with no known myalgia, painful cervical lymphadenopathy, diffuse rash
hemiparesis, dysarthria and sudden onset confusion 2 comorbidities, presents to our service with progressive and fever. Symptoms were self-limited for a period of
days ago. With progressive improvement, without alter- onset of weakness in her right upper and lower limb. 15 days. Approximately two weeks after the episode, he
ation in the neurological exam already in 1 care. Initially, a Concomitantly, she developed tingling in her right hemi- started feeling burning pain in the scalp, in the left pari-
protocol was performed for ischemic stroke (ICAVs) with face and dysarthria. Neurological examination showed etal region, associated with dysesthesia. It evolved with
skull resonance: ischemia sequelae in the left temporal right peripheral facial nerve palsy, soft right palatal palsy, pain in the bilateral pectoral region with progression to
and parietal lobe and left postcentral gyrus transthoracic in addition to complete and proportionate hemiparesis the back at T4 sensory level, radiating to the thoracoab-
echocardiogram and Doppler ultrasound of carotid and on the right, with signs of ipsilateral pyramidal release. At dominal region on the right to the superior iliac crest and
vertebral arteries without significant alterations. In lab- investigation, anti-HIV 1/2 reagent serology was found. later to the right leg. He denied weakness, mental confu-
oratory investigation: fta abs reagent and vdrl 1/64. The CT brain scan showed subcortical hypoattenuation in pre sion and dysautonomic symptoms. Cervical ultrasound
study was carried out on cerebrospinal fluid: Leukocytes and postcentral gyrus on the left. Brain MRI had evidence showed lymph nodes with a reaction pattern. Screening
70, neutrophils 2, lymphocytes 98, glucose 54 and proteins of hypointense signal on T1 and hypersignal in T2-FLAIR with viral serologies was started and MRI of the spine
75.2. In the face of an etiological finding (neurosyphilis at the topography of the pons and cerebellar peduncles, and brain was performed, which showed areas with
causing vascular injury), a patient submitted to intra- as well as in the supratentorial topography mentioned hypersignal on T2 in the spinal cord extending from T8
cranial arterial angio resonance (MMR), showing: Distal above at head CT, involving subcortical U-fibers, and to T11 without anomalous enhancement. CSF was col-
subocclusive stenosis of M1 in right MCA, parietal irregu- without water diffusion restriction or contrast enhance- lected with opening pressure of 30cmH2O; 2 cells/m2
larities in M1 segment of left MCA and M2 bilaterally, and ment. The possibility of progressive multifocal leukoen- lymphocytic predominance; proteins 70mg/dL; ADA
parietal irregularities with moderate stenosis in P2 to the cephalopathy (PML) was then raised, with confirmation 2mg/dL. Positive serum West Nile IgM test. After con-
right. RMIA confirms the etiological hypothesis, due to its after positive JC Virus research in cerebrospinal fluid. firmation of the diagnosis, the patient received 4 days of
classic pattern of arteritis by syphilis (Heubner’s arteritis). Discussion: PML is a progressive demyelinating disease metriprednisolone 1g/day with satisfactory improvement
DC was treated with crystalline penicillin for 14 days and of the Central Nervous System (CNS) caused by the JC of symptoms. Discussion: West Nile fever is caused by the
kept asymptomatic on discharge. The patient unknown Virus in immunocompromised patients. Among the West Nile virus of the flavivirus class and has been one
the diagnosis of syphilis, and it was impossible to deter- conditions most associated with this disease, the best of the major emerging causes of encephalitis in the US.
mine the incubation period and showed comorbidities, known is HIV infection, in the AIDS phase, however cases Transmission occurs mainly through the bite of infected
only smoking. Discussion: Syphilis, sexually transmitted, of PML have already been described in other contexts of Culex mosquitoes, but vertical transmission and blood
if untreated can reach its tertiary form reaching the central immunodeficiency, such as cancer, organ transplantation, transfusion have already been described. Although most
nervous system. DC presents the classic type of menin- and autoimmune diseases. Concerning the diagnosis, in individuals who contract the virus are asymptomatic,
geal neurosyphilis. It has mainly manifested the AVCI addition to the clinical history, the patients present, on symptoms such as fever, rash and myalgia are part of the
secondary to vasculitis caused by treponema pallidum. head CT with asymmetric areas of hypoattenuation in clinical apresentation. Neuroinvasion usually occurs in
The agent causes tapering of the vessel affected by fibro- white matter. On MRI, it characteristically presents with less than 1% of those infected and is characterized by asep-
elastic proliferation of the intima, narrowing of the media hypointensity on T1 and hyperintensity on T2-FLAIR in tic meningitis, meningoencephalitis, acute flaccid paresis,
and fibrosis associated with adventitia alterations, with white matter, as well as the absence of water restriction rhomboencephalitis, myelopathy, polyneuropathy, or
preference for medium and large caliber arteries. There diffusion and contrast enhancement, typically in supra- radiculopathies. The form of neurological involvement
is appearance and collateral circulation, which explains tentorial topographies. The diagnosis is confirmed by the varies according to the seasonality and location of the epi-
clinical practice not compatible with image findings. As positivity of JC Virus in cerebrospinal fluid of suspected demic. CSF usually shows pleocytosis with a lymphocytic
shown in the report, a poor clinic for an exuberant image. patients. Final Comments: Our patient had an atypical pattern, normal glucose, high protein levels (>900mg%).
Final Comments: Approximately 30% of patients without presentation of PML, with infratentorial lesions. It is nec- West Nile virus IgM titers tend to remain high even after
adequate treatment for neurosyphilis will present CNS essary to consider this diagnosis in the face of an adequate acute infection. Final Comments: In Brazil, West Nile
involvement, them 70% are asymptomatic. Associated clinical context. Despite the atypical presentation, there Fever is not a common cause of myelopathies, and other
with a non-exuberant clinic and the growing number are similar cases described in the literature, particularly diagnoses, such as arboviruses, are primarily considered.
of syphilis cases, with 72.8 cases/100,000 inhabitants in in the context of immunodeficiencies other than those In the aforementioned case, the epidemiology retrieved
Brazil, it is always necessary to continue the investigation associated with HIV. We hope that this case presentation during the anamnesis was essential for the investigation
of these infections in cns, using the vdrl test as proposed can help other colleagues in the future who are faced with and conclusion of the proper diagnosis.
by the Ministry of Health as a screening the same radiological images.
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POST-VARICELLA FOCAL CEREBRAL MENINGOEPITHELIAL MENINGIOMA NEUROCRYPTOCOCCOSIS PRESENTED AS


ARTERIOPATHY: A RARE CAUSE OF ASSOCIATED WITH TUBERCULOSIS: A CASE ACUTE MOTOR AXONAL NEUROPATHY: AN
ISCHEMIC STROKE PRESENTATION ATYPICAL MANIFESTATION
Maria Paula Carvalho Azevedo, Camila Caroso Camilla Vanessa Araújo Soares, Herisson Rodrigues Pedro Miguel Mendonça Couto, Breno Gabriel
Lobo, Thiago de Souza Rosa, Lucas de Melo de Oliveira, Andressa Gabriella Duarte de Queiroz, Rodrigues Queiroz, Luiza Lima Berreta, Stefano
Teixeira Branco, Gustavo Manginelli Lamas, Jessica Marcílio Ferreira de Paiva Filho, Maurus Marques de Machado, Arthur Costa Nascimento, Diogo
Daniele Marques, Thomas Vieira de Paula, Amanda Almeida Holanda Fernandes Santos, Wender Rodrigues Teodoro,
Michelucci dos Santos, Felipe Franco da Graça, Leticia Silva Alves
Tânia Aparecida Marchiori de Oliveira Cardoso Federal University of Paraíba, Faculty of Medicine.
João Pessoa PB, Brazil Hospital de Clinicas da Universidade Federal de
Universidade Estadual de Campinas. Campinas SP, Federal University of Paraíba, Faculty of Medicine. Uberlândia. Uberlândia MG, Brazil
Brazil João Pessoa PB, Brazil
Department of Internal Medicine, Hospital peps_couto@hotmail.com
camilaloboc@gmail.com Universitário Lauro Wanderley, Federal University of
Rio de Janeiro. Rio de Janeiro RJ, Brazil Case Presentation: A 33-year-old man, who worked as
Case Presentation: A 15-years-old male patient pre- air conditioning technician, attended to the emergency
sented to the emergency department complaining of camilla.vanessa46@gmail.com department with progressive tetraparesis, ptosis in the
sudden-onset left hemiparesis since the previous day. left eye, hearing loss and sleepiness, associated with a
He also complained about left hemibody paresthesias Case Presentation: A 79-year-old woman admitted to the 2-weeks history of malaise, headache, lumbar and cer-
one week before admission. There was no prior history of Hospital with a history of seizures and difficulty speaking. vical pain. Physical exam revealed arterial hypertension
illness, vaccinations, travel or trauma.However, the par- She reports thirteen years of infrequent headaches, no (164/88 mmHg) and tachycardia (116 bpm), with no fever
ents said that the patient had contact with the sister who seizures or auditory symptoms and a lesion suggestive and a normal cardiovascular auscultation. The patient
had chickenpox months before the event. Neurological of meningioma confirmed via computed tomography, presented with flaccid dysarthria, anisocoric pupils (left
examination revealed complete and disproportionate also reporting having been oligonsymptomatic for 11 3mm and right 2mm), with direct and consensual reflex
left hemiparesis, predominantly in the left upper limb, years with only mild leukopenia. Three years ago she preserved, left-ptosis and impaired adduction and upward
with pyramidal signs. The patient underwent computed presented frontal alopecia and after a new CT scan of the gaze of left eye. At the motor exames there was tetrapare-
tomography, which did not initially reveal any abnormal- skull without contrast that showed an oval lesion, isoat- sis, with MRC score of the right arm of 4, left arm 4- and
ities, but cranial magnetic resonance revealed signs of tenuated in relation to the cerebral cortex with a regular 3 of both legs accompanying global areflexia. CT brain
ischemia in right basal ganglia and internal capsule and contour, measuring 2x1.5 cm in diameter, of extra-axial scan was normal and cerebrospinal fluid (CSF) showed
Vessel wall evaluation revealed signs of focal vasculitis left frontal parrasagittal location in direct contact with pleocytosis (1350 cells/mm³) and hyperproteinorrha-
in the M1 topography of the right middle cerebral artery. the isolateral superior frontal gyrus without perilesional chia (215 mg/dL), suggesting an infectious process, the
The patient underwent lumbar puncture, which revealed edema. In January 2020 he had a seizure with loss of opening pressure was 54 cmH20. Gram staining was
CSF pleocytosis with a predominance of lymphomono- voice for about five minutes and two months later he positive for encapsulated yeast, and cultures growth of
nuclear cells, serology for Varicella Zoster reagent in the developed a second seizure with the same clinical man- Cryptococcus gatti. VDRL and HIV, HTLV, B and C virus
CSF (IgG 1: 8) and in the blood (IgG > 5000 and IgM <0.20). ifestation. Magnetic resonance imaging showed an oval serology were negative. Electromyography confirmed the
The treatment consisted of high doses of corticosteroid lesion located in the left frontal parasagittal region with presence of predominant motor acute axonal neuropathy
therapy (1g of methylprednisolone per day for 5 days) in a diameter of 2.5x2.0x2.0 cm and significant perilesional (AMAN), an Guillain-Barre Syndrome (GBS) variant. It
addition to Acyclovir (800 mg/kg for 14 days) due to evi- edema. After 6 months, in July of the same year, the patient was promptly initialized treatment with immunoglobulin
dence of possible underlying Varicella Zoster infection. presented six crises during a period of four days, and (IVIG), amphotericin B and fluconazole. With the treat-
In evolutionary control imaging, complete resolution of the surgical treatment of tumor excision was indicated. ment there was improvement of the strength, however
the findings suggestive of vasculitis in ACM D described Discussion: Intracranial tuberculosis has several forms, he developed complications of the cryptococcal menin-
above was noted. Discussion: Focal cerebral arteriopathy including tuberculoma and tuberculous abscesses, which goencephalitis with refractory intracranial hypertension
consists of a vasculitis that affects a specific topography of often mimic a meningioma radiologically, requiring (a ventricular shunt was installed), severe hipoacusia
the cerebral circulation, usually affecting the distal portion histopathological and immunohistochemical examina- and visual impairment. Discussion: GBS is an aberrant
of the internal carotid artery and proximal to the middle tion to confirm the diagnosis. The present paper reports reaction of the immune system, in genetically suscep-
cerebral artery. Despite several described causes, varicella a case of coexistence between a meningoendothelial tible individuals, triggered by typically gastrointestinal
infection is one of the main causes in pediatric patients or meningioma associated with multiple epithelioid gran- infection (Campylobacter jejuni). The exact mechanism
those close to this age group. Vascular inflammation can ulomas with foci of caseous necrosis, possibly being the of the autoimmune reaction is not yet clarified. The asso-
occur in a para-infectious context or even months after first case presented in anatomopathological images with ciation with fungal infection is very rare, there are very
the chickenpox condition. This case demonstrates the immunohistochemistry of this association of a tumor few reports of this association. It is not clear if this case
importance of investigating not only the patient’s history, with colonization by Koch’s bacillus. Final Comments: was classic Guillain-Barre Syndrome, or a rare manifes-
but also the history of indirect contact with the virus. Final This case study reported a meningothelial meningioma tation of cryptococcus infection as acute motor neurop-
Comments: focal cerebral arteriopathy is a rare cause of associated with multiple epithelioid granulomas with foci athy. Final Comments: This case highlighted a very rare
stroke that should be kept in mind in young patients and of caseous necrosis, thus being one of the first cases with manifestation of neurocryptococcosis, and despite the
investigation of varicella infection should be performed this rare association and that in the anatomopathologi- fungal infection IVIG was introduced to treat the AMAN
especially in the younger and pediatric population cal examination with immunohistochemistry shows the with relative success. Further studies are needed to clearly
presence of Koch’s bacillus. demonstrate this association.

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PARKINSONISM SECUNDARY TO SIMULTANEOUS OCULAR AND OTOLOGIC MYELITIS POST DENGUE


NEUROINFECTION: A FUNCTIONAL SYPHILIS IN A HIV-INFECTED PATIENT: A
Marcelo Simplicio Carvalho, Amanda Leticia André,
LOBECTOMY BY NEUROCRYTOCOCCOSIS CASE PRESENTATION Tatiane Arroyo Lopes Alves de Jesus, Arthur Coelho
Déborah Castro Ferreira de Oliveira, Pablo Henrique Giulia Murillo Wollmann, Caroline Figueiredo Moura Marinho, Nathalye Fernanda Pedroso
da Costa Silva, Isadora de Castro Ferreira de da Silva, João Pedro Ribeiro Baptista, Gustavo Dircksen, Lorena Ferndandes Kronbauer, Damacio
Oliveira, Hiram Fernandes Soares, Ana Paula de Figueiredo da Silva, Sabrina Hafemann Loz, Rodrigo Ramon Kaimen Maciel
Góes Louly Bustamante, Nayara da Costa Andrade, Harger, Alexandre Luiz Longo Santa Casa de Londrina. Londrina PR, Brazil
Eduardo Bernardo Chaves Neto, Caroline Santos
Eneas, Wagner Afonso Teixeira, André Gustavo Department of Medicine, University of the Region of marcelo.neurolog@gmail.com
Fonseca Ferreira Joinville UNIVILLE
Neurology Service, Internal Medicine Department, Case Presentation: A male, 32-year-old patient was
Hospital de Base do Distrito Federal. Brasília DF, Hospital Municipal São José – Joinville – SC – Brazil admitted to the service due to a history of tetra paresthesia
Brazil initiated three days before, progressing with lower limb
Universidade Federal do Piauí. Teresina PI, Brazil giuliamw@gmail.com
paresis and urinary retention and inability to walk. Patients
Escola Superior de Ciências da Saúde. Brasília DF, denied symptoms such as fever, itching or myalgia. He had
Brazil Case Presentation: A 53-year-old male patient presented
with bilateral rapidly progressive painless visual loss for no other comorbidities or continued medication. At the
deborah_castro22@hotmail.com nine days, associated with bilateral hearing loss, was patient’s examination, she presented a preserved level of
admitted in our emergency service. The comorbidities consciousness, hypoesthesia at L4 level and dysesthesia
Case Presentation: A previously healthy 39 year-old were HIV infection with a CD4 count of 217 cells/mm3 at T5 level and bilateral paresis of the lower limbs. In ini-
patient started with progressive emotional lability, loss and undetectable viral load. The neurological examination tial laboratory tests presented plaquetopenia, rapid test
of appetite and hyporexia, associated with a depressive findings an important reduction of the visual acuity with for Dengue positive, Liquor initially had leukocytes 99,
status 2 years ago. The case was initially associated with light perception and vision of counting fingers bilateral. lymphocytes 89, neutrophils 11, glucose 83 and proteins
functional limitation. 12 months ago the initial symptoms Also a papilledema bilateral with normal photomotor 118.3, lactate 3.3 and the absence of oligoclonal bands,
worsened, and intermittent holocranial headaches with reflex and isochoric pupils. A hearing loss and a tinnitus anti-MOG and anti-aquaporin 4. No changes to initial skull
tremors, slow and clumsy member movements were complaint were present on admission, however no specific tomography. Then, a column resonance was requested,
associated. They were admitted in a specialized service tests were performed. No pathological findings were found which evidenced a C6-C7 signal alteration and in the
for investigation. A parkinsonian syndrome was detected on cranial CT. CSF analysis demonstrated white cell count entire dorsal column. MRI of the skull and orbits with-
in the physical examination: plastic hypertonism, brady- of 4 cels/uL, appropriate level of glucose, protein levels 87 out alteration. Then initiated pulse therapy with methyl-
kinesia, with rest and postural bilateral, symmetric, high mg/dL and a non treponemic test (VDRL) negative. Due prednisolone 1g for 5 days, the patient presented gradual
frequency and low amplitude tremors of the extremities. to the optic neuritis hypothesis, a brain and orbital MRI improvement of symptoms, with improvement of sensi-
Hypomimia, cognitive loss with an amnestic component was requested and no abnormalities were identified. An tivity and strength of lower limbs. Discussion: Transverse
and bradypsiquism were also found. Routine laboratorial ophthalmological evaluation was made with the retinal myelitis is a clinical picture of loss of strength, autonomic
investigation showed no findings. HIV, syphilis and hepati- mapping showing an elevated and hyperemic optic disc and sensory dysfunction, and its relation to dengue is
tis serologies were negative, with normal levels of TSH and with blurred edges 360 degrees, especially in the left rare. Neurological manifestations resulting from dengue,
T4. CSF: OP 19 cmH20 – Erythrocytes 250; white cells 100 eye. A mild macular edema, secondary to papilledema, are atypical, compared to other arboviruses, and within
(Lymph 86 | Neutro 15 | Mon 9), Glu 42; Ptn 262; negative was present, normal blood vessels and no exudative or these manifestations are the most frequent encephalitis
acid-fast stain; negative Gram stain; general culture results hemorrhagic lesions were present. To assess the etiol- and Guillain Barré syndrome. The diagnosis is based on
were also negative. A rapid test for Cryptococcus neofor- ogy, the laboratory was screened for infectious agents the patient’s clinical, resonance exams and liquid anal-
mans was positive. Cranial MRI showed a T1 hypointen- such as toxoplasmosis and cytomegalovirus, all of which ysis, and the presentation of the condition can appear
sity signal and a T2 and FLAIR hyperintensity signal in the were negative. The serum treponemal test was reactive about 4 days after classic symptoms of dengue, such as
bilateral nuclear-capsular region, somewhat symmetric. and the VDRL result was 1: 128. Based on the presence fever and myalgia. In this specific case, the patient did not
There was also a post-contrast enhancement of these of ocular and otological involvement the hypothesis of present classic symptoms resulting from dengue, and the
multiple confluent nodular points, with compromise of simultaneous ocular syphilis and otosyphilis was made. neurological condition was the only manifestation of the
the ependyma of the lateral ventricles, III and IV ventri- The patient received intravenous therapy with ceftriaxone disease. The treatment proposed in the literature is pulse
cles and the basal cisternal leptomeningeal layer. With for 14 days, aqueous crystalline penicillin G was unavail- therapy with methylprednisolone for 5 days, which was
the diagnose of neurocryptococcosis in mind, treatment able. A gradual improvement of signs and symptoms were performed in the case with adequate response to medi-
started with Amphotericin B, Fluconazole and inicial fase presented with partial recovery of visual acuity and resolu- cation. Final Comments: Post-dengue myelitis is one of
corticostheroids, which were later associated with Prolopa tion of otological complaints. The patient was discharged the rare neurological manifestations of this arbovirus,
and Levetiracetam, with substantial improvement of the for outpatient follow-up. Discussion: CNS involvement with a small sample described in the literature, and it is
bradykinesia and bradypsiquism. Ambulatorial follow-up can occur at any stage of syphilis. Among patients with important to report these cases to better understand the
is being made to this day. Final Comments: Secundary secondary syphilis, 18% may have neurological symp- pathological mechanisms related to dengue.
parkinsonian syndromes are frequently associated with toms. Syphilis has a wide variety of clinical forms, it can
vascular diseases or drug usage, but other differential affect the ocular or auditory level. Only one previous case
Neuroinfecção
diagnoses include post-cranial cerebral trauma, neu- found in the literature presents a clinic similar to this
roinfections, encephalitis, external intoxications and one.Syphilis affecting the auditory system is an uncom-
multiple sclerosis. This particular case stood out because mon but underdiagnosed condition. Final Comments:
of the low frequency of Parkinson syndrome secondary Although rare, this report is of great value to review the
to a neuroinfection; specially a non-HIV infected patient importance of differential diagnosis in neuroinfectology.
suffering from neurocryptococcosis. Ethics Committee No: 5397799

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163
XXX Congresso Brasileiro de Neurologia

TL 1106134 TL 1106167 TL 1106170

ATYPICAL PRESENTATION OF SEVERITY OF MYELITIS-ASSOCIATED CEREBRAL VENOUS THROMBOSIS (CVT)


NEUROSYPHILIS AS STROKE-LIKE RHOMBOENCEPHALITIS IN AN AND ITS INTERFACE WITH ARBOVIROSES
SYNDROME – A CASE PRESENTATION IMMUNOSUPPRESSED PATIENT
Iury Hélder Santos Dantas, Iony Santana Santos
Gabriel Vianna Pereira Aragão, Aurea Maria Lago Beatriz Medeiros Correa, Vanessa Moraes Rossette, Dantas, Roberta Lessa Ribeiro, Fernanda Martins
Novais, Ana Rafaela Soares Vale, Ana Beatriz Bruna Burigo, Ana Paula Almeida Campos, Clara Maia Carvalho
Oliveira Andrade, Hugo Oliveira Polito Barreto, Kimie Miyahira, Natalia Figueiredo Miranda, Thiago
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Lara Cordeiro Magalhães, Henrique Nascimento da Cruz Marques, Antonio Carlos de Padua Milagres,
Universidade Federal do RIO GRANDE do NORTE –
Dourado, Daniel Rocha Paranhos, João Felipe Raphael Palomo Barreira
CAICÓ – RN – Brazil
Pereira Câncio, Rubson Soares Rocha
Irmandade Santa Casa da Misericórdia de Santos.
iuryhelder@hotmail.com
Escola Bahiana de Medicina e Saúde Pública. Santos SP. Brazil
Salvador BA, Brazil
beamedneuro@gmail.com Case Presentation: A 50-year-old, female, previously
Hospital Geral Roberto Santos HGRS
healthy sought medical attention due to progressive,
gabrielaragao18.2@Bahiana.edu.br Case Presentation: Rhomboencephalitis is rare, poten- squeezing, diffuse headache associated with persistent
tially serious and fatal, affecting the hindbrain (pons, fever (38°C), arthralgia and myalgia. Given the suspicion
Case Presentation: J.C.O, male, 53y, admitted with sud- medulla and cerebellum). The pathology has varied etiol- of arbovirus, the patient was transferred to a special-
den left hemiparesis, being referenced to the Neurology ogies such as infectious, autoimmune and paraneoplastic. ized service in Fortaleza. The admission exams revealed
referral Hospital for thrombolysis window protocol. Upon It may be associated with myelitis, causing motor, sensory platelets of 20.000 mm³, positive PCR for dengue and
admission, he had an 11 NIHSS score with temporo-spatial and autonomic changes. The clinic is characterized by positive IgM and negative IgG serology. However, during
disorientation, mild facial palsy and dysarthria, paresis prodromes of fever, vomiting, and headache, followed by Hospitalization, headache intensity worsened, associated
with hypoesthesia on left hemibody and bilateral amau- neurological manifestations such as cranial nerve pare- with nausea, vomiting, visual blurring and unprecedented
rosis. He reported optic neuritis diagnosis when he pre- sis, paresthesia, and sphincter dysfunction.To report the tonic-clonic seizures in 4 limbs (post-ictal lasting 30
sented bilateral amaurosis in 2013 and carried out an case of a patient with Human Immunodeficiency Virus minutes). Due to clinical worsening, underwent Cranial
incomplete investigation at the time, by his own choice. (HIV) without regular treatment, diagnosed with myeli- Resonance and Angioresonance (arterial and venous
In view of the situation, the case was then conducted as tis-associated rhomboencephalitis, by obtaining data phases) which revealed venous thrombosis in the trans-
a Cerebrovascular Accident (CVA), but the patient was from medical records and reviewing the literature on the verse sinus and left sigmoid sinus. Absence of changes in
not thrombolysed due to a contraindication related to the subject. Discussion: R.A.C, 37 years old, admitted to the the electroencephalogram, lumbar puncture with opening
time of admission. Etiological investigation exams were intensive care unit in April 2020, with a report of vertigo pressure of 25 cmH2O, 10 cells (90% of lymphocytes), no
performed, showing normal results. Serology with VDRL and paresthesia of the left lower limb with 7 days of evo- changes in glucose, increase in proteins: 60 mg/dL. It is
and rapid test came back positive. The patient was then lution progressing with crural paraparesis and urinary useful to point out that on this occasion the platelets were
submitted to cerebrospinal fluid puncture, which showed retention. He has a history of using anabolic steroids, hep- 175.000 mm³. Hematological and rheumatological exam-
increased cellularity of 24 and reactive VDRL. During atitis B, presenting CD4 lymphocyte 48 and a viral load of inations were normal. It was decided to start anticoagu-
Hospitalization, the family reported that the patient had 32,000 copies. On admission cranial tomography, he had lation with low-molecular-weight heparin and later start
been experiencing markedly cognitive impairment with extensive hypodensity in the subcortical region, cerebro- warfarin 5 mg/day. The patient was discharged without
memory loss, behavior and psychological changes. He spinal fluid puncture with pleocytosis, proteinorrachia complaints, with oral anticoagulation and follow-up at
was treated with Crystalline Penicillin according to the and reagent serology for herpes zoster and cytomegalo- the CVT outpatient clinic. Discussion: Cerebral venous
protocol and on the 14th day of treatment showed motor, virus. A contrast-enhanced magnetic resonance imaging thrombosis (CVT) is a rare and often underdiagnosed
sensory and cognitive improvement, being able to walk was performed, revealing irregular uptake nodules at condition. It presents with a wide spectrum of signals and
and carry out his activities without assistance, main- the transition of the middle cerebellar peduncles with with high variability. Headache is present in most cases.
taining the previous amaurosis. Discussion: Syphilis is the hemispheres and at the bulbopontine transition, in The presence of papilledema, vomiting, blurred vision,
a sexually transmitted infection caused by the bacterium addition to poorly defined hypersignal in the left hippo- alteration of the VI cranial nerve, diplopia, focal deficits,
Treponema pallidum. It is estimated the incidence of 12 campus, parahippocampal gyrus and ipsilateral inferior seizures and changes in consciousness are highlighted.
million cases per year, more than 90% being in develop- temporal, suggesting rhomboencephalitis. The MRI of the Hematological and rheumatological exams as well as the
ing countries. Neurosyphilis occurs in less than 10% of cervical and thoracic spine showed hypersignal typical study of vessels with angioresonance are essential for the
cases, and its most commonly observed form presents of myelitis. Treated with pulse therapy associated with diagnosis. In this approach it is necessary to be attentive to
with neuropsychiatric changes. Only 3% of cases are broad-spectrum, antifungal and antiviral antimicrobials in the interface of arboviruses, an example of dengue, with its
associated with CVA, due to an infiltrative inflammatory an intensive care setting with partial improvement of the repercussions on the Central Nervous System in order to
process, most commonly of the middle cerebral artery. It condition. After 28 days of Hospitalization, he presented better understand the behavior of the disease. CVT is an
is thought that the patient’s previous optic neuritis may worsening and sepsis of pulmonary focus evolving to uncommon complication in dengue. Final Comments:
have been an even more uncommon manifestation of death. Final Comments: The case presented raises the It is essential to be aware of the alarm signals (red flags)
neurosyphilis. Final Comments: This case illustrates an discussion about the involvement of immunosuppressed as a form of suspected secondary causes of headache.
atypical manifestations of neurosyphilis. It is important patients by a pathology little reported in this population, In this context cerebral venous thrombosis constitutes
to remember that this condition can be a primary etiology in relation to other pathologies that affect the central ner- an important differential diagnosis, especially in women
for stroke, especially in patients under 50 years of age, as vous system in these individuals, evolving with a severe with persistent headache in the context of arboviroses.
well as for optic neuritis. Therefore, a detailed evaluation form, rhomboencephalitis associated with myelitis, a
is essential in patients with initial investigation for normal potentially fatal, like the picture described.
stroke or atypical natural history. Once the diagnosis is Neuroinfecção
made, treatment can prevent an unfavorable evolution
and provide a significant clinical improvement. Neuroinfecção

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164
XXX Congresso Brasileiro de Neurologia

TL 1106187 TL 1106239 TL 1106264

ATYPICAL NEUROTUBERCULOSIS LONGITUDINAL EXTENSIVE TRANSVERSE NEUROCRYPTOCOCCOSIS IN A YOUNG


IN AN ADOLESCENT WITH MYELITIS CAUSED BY SCHISTOSOMIASIS: A IMMUNOCOMPETENT PATIENT: A CASE-
CLINICAL PRESENTATION OF CASE PRESENTATION BASED UPDATE
MENINGOENCEPHALOMYELITIS FORM: A
Clauhan Williams Soares dos Santos, Débora de João Valdêncio Silva, Luís Eduardo Oliveira Matos,
CASE PRESENTATION Moura Muniz, Débora Mendes Braun, João Gabriel Paulo Roberto Matos Neto, Lucas Antonissen Lima
Marco André Moraes Bernardino, Djanino Fernades Lucena de barros, Luiza Carvalho de Paula, Ellen Verde, David Elison Lima Silva, Espártaco Moraes
Silva, Paulo Barbosa Leite Neto, Victor Fellipe Bispo Kosminsky, Alice Rodrigues Pimentel Correia, Yan Lima Ribeiro, Keven Ferreira Ponte, Paulo Roberto
Macedo, Larissa Clementino Leite Sá Carvalho, João Pereira Rocha, João Eudes Magalhães, Ana Rosa Lacerda Leal
Eudes Magalhães, Fabiola Lys Medeiros, Elzeleide Melo Correa Lima
Universidade Estadual do Ceará. Sobral CE, Brazil
Souza Vasconcelos Faculdade de ciências médicas da Universidade de
Pernambuco. Recife PE, Brazil João valdencio@gmail.com
Hospital Universitário Oswaldo Cruz. Recife PE,
Brazil Faculdade Pernambucana de Saúde. Recife PE,
Brazil Case Presentation: A 19-years-old female presented 3
marconerimorais@hotmail.com months ago an episode of an intense occipital pulsatile
clauhanw@gmail.com headache with irradiation to the frontal region, associated
Case Presentation: A previously helalthy, 17-year-old with vomiting, photophobia, phonophobia, right arm
woman, with a complete vaccination schedule, in april Case Presentation: A previously healthy 47-year-old man paresthesia and dysarthria. After 20 days, she evolved
2021, presented acute onset of moderate-intensity head- presented with a history of diarrhea associated with low with a return of this condition, with additional lower limb
ache evolving after 2 days to reduced consciousness. back pain since January 2022. He evolved in one month paraparesis, fever and low back pain, which demanded
Neurological exam: regular general condition, drowsy, with bilateral leg paresthesia and later spastic parapare- Hospitalization for 4 days. After partial improvement of
uncooperative, and contacting. Presence of meningeal sis. He also presented urinary urgency and incontinence, the condition, she was discharged, still unable to walk.
irritation. Ophthalmoplegia in left eye abduction and intestinal constipation and erectile dysfunction. In the At her home, she evolved with worsening headache and
right eye with nystagmoid jerks. Right central facial. Right neurological examination there were hyperreflexia in low back pain, also appearing diplopia and left convergent
hypotonia and left elastic hypertonia (Ashworth-2). Muscle the upper and lower limbs (more overresponsive in the strabismus. Admitted to the emergency, a skull Computed
strength-MRC-2, bilateral. Hypoactive deep reflexes glob- inferior limbs), bilaterally positive Hoffman and Babinski Tomography and Magnetic Resonance Imaging were per-
ally, bilateral Babinski. Empirical treatment was insti- responses, bilateral spinal automatism, and bilateral formed, both without alterations. The cerebrospinal fluid
tuted immediately for bacterial, viral meningoenceph- paretic gait. In addition, there was decreased superficial (CSF) analysis showed hypoglycorrhachia and hyperpro-
alitis, corticosteroid, and therapy for neurotuberculosis. sensation up to the T7 level, but no alterations in deep sen- teinorrhachia, then antibiotic therapy was performed for
Rheumatological tests and serum angiotensin-converting sation. The MRI of the spine revealed an extensive lesion, 12 days, without improvement. A new lumbar puncture
enzyme negatives. Cerebrospinal fluid (pleocytosis-lym- more evident in the mid-segment of T1-T7, and affecting was performed, which showed cells suggestive of crypto-
phomononuclear, hyperproteinorrachy and hypoglycor- mainly the central spinal cord denoting probable myelitis. coccosis, in the strong suspicion of this fungus, multiplex
rhachia), negative results to gram, fungus culture and Active infection by Schistosoma mansoni was evidenced Polymerase Chain Reaction (PCR) was requested, which
tuberculosis genexpert. Brain MRI with hypersignal of in a kato-katz stool parasitological examination. The CSF confirmed the infection by Cryptococcus Neoformans/
left nucleus-capsular; thickening and enhancement of analysis showed increased cellularity including eosino- Gattii. Discussion: Neurocryptococcosis is a fungal infec-
supra and infratentorial meningeal planes; prominent phils and protein level with positive anti-Schistosoma tion caused by two species of Cryptococcus, C. neoformans
supratentorial ventricles; focal nodular with peripheral serology. ANA, HIV/HTLV serology, VDRL, rheumatoid and C.gattii. It represents an important opportunistic
enhancement in right insula. Cervicothoracic and lum- factor, anti RO, anti LA, and vitamin B12 level were unre- infection related to AIDS, being uncommon in immu-
bosacral MRI revealed thickening and enhancement of markable, and anti-aquaporin-4 was negative. He received nocompetent patients. The clinical presentation includes
posterior leptomeningeal in D2 and D8; hypersignal D4 intravenous therapy with 1g methylprednisolone for 5 signs and symptoms such as headache, fever, nauseas/
to D7 (edema), left enhancement of descending nerve days and oral praziquantel. He progressively improved vomiting, cranial neuropathies, mental disorder, lethargy,
root of S1(inflammatory neuropathy); increased dimen- and regained gait after treatment. Discussion: We pres- diplopia, intracranial hypertension and signs of meningeal
sions and leptomeningeal nodular foci enhancement in ent a case of neuroschistosomiasis as the cause of spastic irritation. The CSF analysis can include elevated white
C7 and D11 segments. Normal transesophageal echocar- paraparesis with superficial sensory loss up to thoracic cell count, with lymphocyte predominance, elevated CSF
diogram. Subsequently, COXCIP drug-induced hepatitis level, and sphincter dysautonomic syndrome. The spinal protein, and low CSF glucose. The definitive diagnosis is
with suspension, and an alternative tuberculostatic RIPE cord lesion was not only high, but also longitudinally made by isolation of Cryptococcus or direct detection
regimen was replaced, being reintroduced drug by drug extensive, which is uncommon. Myeloradiculitis is the of the fungus by means of India ink staining of the CSF.
each month, for 12 months. The patient presented motor most common extraintestinal manifestation of schistoso- Other forms of diagnosis include histopathology, sero-
improvement (MRC-4) after 6 months. Maintain outpatient miasis. It is caused by the presence of the parasite egg or logical and molecular methods. Amphotericin B deoxy-
follow-up, with motor rehabilitation. Discussion: Central the adult worms that migrate through the venous plexus cholate and flucytosine is the main treatment for severe
Nervous System Tuberculosis is 1% of all tuberculosis of Batson to the spinal cord and induce a focal granulo- cryptococcal infection, including meningoencephalitis.
cases and half of these involve the spine. Intramedullary matous reaction. Due to its anatomy, the most frequent Liposomal amphotericin B is a second option with less
involvement is rare, usually expressed in radiculomyelitis, locations are the lower thoracic and lumbar regions of the nephrotoxicity, and other antifungals or combinations
transverse myelitis, intraspinal granulomas, or anterior spinal cord, unlike the presented case. Final Comments: can be used in particular cases. Final Comments: Early
spinal artery thrombosis. PCR test for M. Tuberculosis In the context of thoracic, longitudinal extensive spinal diagnosis of neurocryptococcosis in immunocompetent
has sensitivity from 56% to 90% and specificity from 88% cord lesions, it is important that schistosomal myelora- patients can be challenging. Being aware of this infection
to 100%. Final Comments: As already documented and diculitis should be included in the differential diagnosis, in this group of patients is necessary for rapid and appro-
supported by case series, it is essential, especially in especially in endemic areas. priate management.
endemic areas, to consider causes of infectious mycobac-
terial etiology, particularly tuberculosis, in the differen-
Neuroinfecção Neuroinfecção
tial diagnosis of radiculomyelitis, even if the etiological
evidence is not confirmed.

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XXX Congresso Brasileiro de Neurologia

TL 1106311 TL 1106312 TL 1106337

ONE MORE CASE PRESENTATION BRAIN FUNCTIONAL CONNECTIVITY IS NEUROCYSTICERCOSIS WITH EXTENSIVE
OF A PATIENT WITH COVID-19 AND SEVERELY DISRUPTED IN THE PRESENCE CERVICAL MYELITIS: CASE PRESENTATION
ENCEPHALOPATHY ASSOCIATED WITH OF ANXIETY AND DEPRESSION SYMPTOMS
Ellen Silva Carvalho, Maria Clara Baseio, Renan
POSSIBLE LANCE-ADAMS SYNDROME AFTER MILD COVID-19 INFECTION Barros Lopes, Caroline Darold Vieira, Túlio Maranhão
WITHOUT CARDIAC ARREST Neto, Beatriz Gioppo Betini, Ray Almeida Silva
Beatriz Amorim da Costa, Lucas Scárdua Silva,
Matheus Henrique Nogueira, Rafael João Batista, Rocha, Tissiana Marques Haes
Bruno Miceli Gonzalez Nogueira, Cláudia Cecília da
Silva Rêgo, Maria Emilia Cosenza Andraus, Valéria ítalo Karmann Aventurato, Vítor Guimarães Corrêa, Hospital das Clínicas da Faculdade de Medicina de
Coelho Santa Rita Pereira, Tiago Silva Aguiar, Jorge Fernando Cendes, Maria Julia Mendes, Clarissa Lin Ribeirão Preto. Ribeirão Preto SP, Brazil
Paes Barreto Marcondes de Souza, Soniza Vieira Yasuda, Brunno Machado de Campos
Alves Leon e_len12@hotmail.com
Universidade Estadual de Campinas. Campinas SP,
Translational Neurosciences Laboratory, Federal Brazil Case Presentation: A 59-year-old woman was diagnosed
University of the State of Rio de Janeiro. Rio de with epilepsy secondary to neurocysticercosis (NCC) at
beatriz.amorim@email.com
Janeiro RJ, Brazil seven years old. Despite the use of albendazole, she devel-
Translational Neurosciences Laboratory, Federal oped cysticercal meningitis that was responsive to dexa-
Background: Although most individuals with post-COVID
University of the State of Rio de Janeiro, and methasone, but recurred upon withdrawal. The chronic
syndrome present symptoms of anxiety and depression,
Clementino Fraga Filho University Hospital, Federal corticotherapy resulted in aseptic necrosis of knees. Then,
little is known about the associated brain dysfunction. So
University of Rio de Janeiro, Neurology Department, obstructive hydrocephalus occurred due to a suprachias-
far, fewer studies have analyzed brain connectivity abnor-
Epilepsy Center. Rio de Janeiro RJ, Brazil matic cyst, requiring ventriculoperitoneal shunt and cyst
malities in post-covid syndrome. Objective: To investigate
Clementino Fraga Filho University Hospital, Federal removal. One year later, she had an ischemic stroke due
the impact of symptoms of anxiety and depression on
University of Rio de Janeiro, Neurology Department, to cysticercotic vasculitis, confirmed by arteriography.
functional brain connectivity (FC) after mild COVID-19
Epilepsy Center. Rio de Janeiro RJ, Brazil After 16 years, she presented with asymmetrical tetra-
infection, analyzing 12 large-scale brain networks (NW)
Translational Neurosciences Laboratory, Federal paresis, fecal and urinary incontinence and neuropathic
with resting-state fMRI (rs-FMRI). Methods: We analyzed
University of the State of Rio de Janeiro, and pain. Neuroimaging was suggestive of cervicomedullary
154 post-covid subjects who had a mild infection and con-
Clementino Fraga Filho University Hospital, transition cysts and active diffuse myelitis. Quarterly cor-
firmed RT-PCR test and 90 healthy controls. All individuals
Federal University of Rio de Janeiro, Neurosurgery ticosteroid pulse therapy was started, reducing pain and
were evaluated with BDI (Beck Depression Inventory) and
Department, Epilepsy Center. Rio de Janeiro RJ, stabilizing the motor symptoms. However, the condition
BAI (Beck anxiety inventory) for quantification of symp-
Brazil worsened three years later, not responding to corticother-
toms. Patients were divided into asymptomatic-group
bmicgonza@gmail.com (ASYMP-Group, no symptoms of depression or anxiety) apy, when treatment was changed to oral methotrexate
(84 individuals, 54 women, median age of 36 years), and (MTX) 10mg per week, and then modified to the subcu-
DOUBLE-group (combined symptoms of anxiety and taneous presentation to minimize gastric intolerance.
Case Presentation: A diabetic, hypertensive, with heart
depression) (70 subjects, 55 women, median age of 41 Six years ago, BIPAP use was started due to severe sleep
and chronic renal disease and ex-smoker 78-year-old man
years).All individuals underwent rs-fMRI scan (along with apnea and restrictive lung disease secondary to myelitis.
was admitted to the emergency department with acute
3D T1-weighted) on a 3T scanner. Each group was com- Currently, epilepsy, neuropathic pain and motor symp-
dyspnea, sweating and fever. A chest CT scan revealed
pared to a specific control group paired for age and sex. toms are stable, but the patient is partially dependent
hypo-expanded lungs, atelectasis signs in the bilateral
Images were analyzed with UF2C/SPM12 (http: //www. on a wheelchair and needs 10mg of MTX every 21 days,
lower lung lobes, right pleural effusion and cardiomegaly.
lni.hc.unicamp.br/aoo/uf2c/) running on MATLAB2019b. besides carbamazepine, gabapentin and venlafaxine for
A new chest CT showed diffuse and bilateral ground-glass
The protocol includes steps of pre-processing (normal- pain control. Discussion: NCC is a disease that presents
opacities and a nasopharyngeal swab RT-PCR for SARS-
ization and smoothing) and extraction of time series variable symptoms, being epilepsy the main one, followed
CoV-2 was positive. The patient evolved with lowering of
from 70 Regions of Interest (ROIs) (from 12 resting-state by focal neurological deficit, intracranial hypertension
consciousness level associated with left upper limb rapid
networks: Anterior and Posterior Salience, Basal Ganglia, and cognitive deficit. Only 2.7% of cases have spinal cord
and repetitive movements described as myoclonus. The
Dorsal and Ventral Default Mode, Left and Right Executive involvement and most of these cases present as radicular
electroencephalogram (EEG) showed a diffuse slowing,
Control, Auditory, Visual, Language, Sensorimotor and pain, sensory and motor deficits. Severe parenchymal or
in the theta/delta range, and periods of generalized atten-
Visuospatial/Dorsal Attention) used to construct Pearson’s subarachnoid NCC may require long-term immunosup-
uation confirming an encephalopathy pattern. There is
Cross-Correlation Matrix. Results were considered signif- pression, with a steroid-sparing drug, such as methotrex-
no previous cardiac arrest. The patient died a few weeks
icant after correction for multiple comparisons at p<0.05. ate and etanercept, as prolonged use of corticosteroids
later, after clinical worsening and complications of his
Results: While the DOUBLE-group exhibited a wide- can have serious side effects. Final Comments: Serious
renal condition. Discussion: We report a rare case of
spread, bilateral pattern of hyperconnectivity involving manifestations may result from NCC and myelitis is a rare
COVID-19 infected patient with severe hypoxia evolving
the 12 networks and most of 70 ROIs, the ASYMP-group associated condition. Immunosuppression can stabilize
with decrease of consciousness level and focal myoclo-
showed fewer alterations, with hyperconnectivity involving the course of this condition.
nus. There are several studies in the literature showing
the occurrence of de novo epileptic seizures in SARS- only five networks (posterior salience, ventral and dor-
CoV-2 infection, which was not confirmed in this case sal DMN, and right and left executive control) and nine
Neuroinfecção
(DELORME et al., 2020; DIXON et al., 2020; SOMANI et ROIs. (p<0.05). Final Comments: Our results suggest a
al., 2020). On the other hand, Lance-Adams syndrome is severe pattern of abnormal cerebral functional connec-
associated with severe hypoxemic states of different eti- tivity, mainly in individuals with persisting symptoms
ologies and seems to be reported in very few COVID-19 of anxiety and depression. These abnormalities may be
patients (MUDDASSIR et al., 2021). Final Comments: The associated with cognitive dysfunction in post-covid syn-
present case is representative about the severe outcome drome. Symptomatic treatment should be available for
associated to nervous system involvement and includes these individuals to improve their quality of life.
the COVID-19 hypoxemic state among the differential
diagnosis of Lance-Adams syndrome without previous
cardiac arrest. Biological mechanism associated with Neuroinfecção
the severe outcome is still a challenge to be understood
and this report may contribute adding up to a few cases
describing so far.

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166
XXX Congresso Brasileiro de Neurologia

TL 1106365 TL 1106389 TL 1106450

RECURRENT NEUROTUBERCULOSIS CNS (CENTRAL NERVOUS SYSTEM) NEUROLOGICAL MELIOIDOSIS: A CASE


WITH RIFAMPICIN RESISTANCE IN PARACOCCIDIOIDOMYCOSIS AS A PRESENTATION
IMMUNOCOMPETENT PATIENT: A CASE DIFFERENTIAL DIAGNOSIS OF IGG4 –
Sylvio Ricard Gonçalves Souza Lima, Karoline
PRESENTATION DISEASE RELATED : CASE PRESENTATION Ferreira Mororó Menezes, Ana Silvia Sobreira Lima
Larissa Clementino Leite Sá Carvalho, Djanino Paulyane Thalita Miranda Gomes, Paulo Pereira Verde, Karla Rafaele Silva Vasconcelos, Larissa
Fernandes Silva, Marco André Moraes Bernardino, Crhisto, Raquel Vassao Araújo, Antonio Pereira Brenda Gonaçalves Miná, Sarah Diogenes Alencar,
Paulo Barbosa Leite Neto, Victor Fellipe Bispo Gomes Neto Victor Vitalino Elias, Deborah Moreira Rangel,
Macêdo, João Eudes Magalhães, Fabiola Lys Fernanda Martins Maia Carvalho, Norberto Anizio
Medeiros, Ana Rosa Melo Correa Lima Santa Casa de Belo Horizonte. Belo Horizonte MG, Ferreira Frota
Brazil
Hospital Universitário Oswaldo Cruz da Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Universidade de Pernambuco. Recife PE, Brazil paulyanetmg@gmail.com
sylviorgs@gmail.com
clementinolari@gmail.com Case Presentation: Male, 38 years old, healthy, moto-
boy,from Belo Horizonte,MG.In Mid 2020,he presented FCase Presentation: Male, 54 years old, with SAH and
Case Presentation: Male, 38 years old, previously healthy, headache,xeroftalma and perceived two small bilateral DM 2, on 05/10/2021 she started inappetence, nau-
in 2018, presented progressive headache, vomiting and cervical nodules in addition to intermittent paresthesias sea and muscle fatigue. She evolved 7 days later with
fever. After 4 months, diplopia and syncope. On exam, in fingers,hands and face and seizures.He also had type unmeasured fever, drowsiness and the appearance of a
generalized lymphadenomegaly and vertical conjugate B symptoms(weight loss, frequent sweating and fever painful nodular lesion on the scalp.; after three days she
gaze limited. Viral hepatitis, syphilis and HIV serologic spikes).He looked for a neurologist who requested an had temporo-spatial disorientation and generalized ton-
tests were negative. Cerebrospinal fluid (CSF) showed MRI,EEG and referred him to Hospital.He received pulse ic-clonic seizures. Attended at the HGF emergency room,
inflammatory pattern with increased opening pressure, therapy with1g methylprednisolone for five days, with brain MRI showed changes in tissues from the face to the
and immunological tests to syphilis, cytomegalovirus, partial improvement in his neurological condition.A sin- galea, intradiploeal and subdural collections, irregular
Epstein-Barr, cultures for fungi and mycobacteria were gle dose of IV cyclophosphamide(1g)was prescribed.The pachymeningeal enhancement, T2/FLAIR hypersignal
negative. Brain MRI showed solid hypointense nodular laboratory showed an increase in IgG4(above 8000),with and diffusion restriction in the left parietal lobe suggestive
images, enhancement in the frontal and bilateral tempo- a brain MRI report suggesting Igg4-related disease.The of cerebritis, MR angiography with thrombosis sigmoid
ral regions. Lung and brain biopsies were inconclusive. Rheumatology evaluation ruled out other rheumatologic sinus and CSF study 1182 red blood cells, 9 leukocytes
Empirical treatment for tuberculous, fungal meningoen- diseases.The biopsy of cervical lymph node revealed (neutrophils 77%, lymphocytes 22%), proteins 49mg/
cephalitis, nocardiosis, and pyogenic brain abscess was paracoccidioidomycosis(PCM), and treatment with dl, glucose 55mg/dl, LDH 41, ADA 0.1 and oncotic cells,
started. Antituberculosis drugs were used for 12 months amphotericin B deoxylate was initiated.After treatment, fungi, BAAR, VDRL and negative cultures. She worsened
and maintenance doses of fluconazole and prednisone, there was significant clinical and radiological improve- clinically, requiring mechanical ventilation in the ICU. The
with improvement of diplopia and headache. After 2-years, ment,with a drop in serum IgG4 levels. Discussion: The pathogen Burkholderia pseudomallei was isolated from
he presented again with pain and right axillary lymph node IgG4-related disease has various clinical manifestations, three blood culture samples and treated with meropenem
enlargement, anatomopathology realized, suggested gan- with possible involvement of the pancreatobiliary tract, for 8 weeks. In the eradication treatment, he developed
glionar tuberculosis, and no other changes in new radio- retroperitoneum, head and neck organsand glands. It pancytopenia secondary to the use of sulfamethoxaz-
logical exams. The rapid molecular test for tuberculosis could mimic neoplastic, inflammatory and infectious ole-trimetropine. He was discharged with negative blood
(TRM-TB) was positive with no resistance to Rifampicin conditions.Histopathology remains the key to diagnosis cultures for outpatient treatment with amoxicillin-clavu-
and antituberculosis drugs and corticotherapy was reini- because reliable biomarkers are lacking.IgG4-related lanate. Melioidosis is caused by the gran-negative bacillus
tiated. Primary immunodeficiencies were searched, with disease is treated with glucocorticoids but can even lead Burkholderia pseudomallei, responsible for 89,000 annual
no abnormalities. At the end of treatment, corticotherapy to death if not recognized.However, to get accurate diag- deaths worldwide. In Ceará, the first case was described
was discontinued; however, diplopia, visual blurriness, nosis of IgG4-related disease, it is mandatory to exclude in 2003. In 2005 it became a notifiable disease. The infec-
dizziness, and headache returned. New brain MRI showed infectious, neoplastic and rheumatologic diseases. On tion occurs by inhalation/inoculation of the pathogen; is
active mesencephalic lesions and MRI-BT of a CSF sample the other side, PCM, a systemic infection caused by the confirmed by culture or PCR. In the neurological form,
positive for rifampicin resistance. Then, alternative regi- fungus Paracoccidioides braziliensis, affects individuals extracranial lesions occur, may involve contiguous sites,
men for rifampicin-resistant neurotuberculosis with RIPE in all age groups.The lung is the most affected organ, sub or extradural collections, brain micro or macroab-
regimen, Levofloxacin and Clarythromicin, and adjust- and it may present on the CNS.Santos et al,2012, eval- scesses, cranial/spinal osteomyelitis, leptomeningitis,
ment of prednisone were chosen, with improvement of uated the levels of IgG, IgG 1, IgG 2, IgG 3 and IgG4 in meningonencephalitis, brainstem encephalitis with cra-
symptoms and outpatient follow-up. Discussion: Central 54 patients with PCM during and after treatment.Only nial nerve palsy, myelitis and dural venous sinus throm-
nervous system tuberculosis is an infrequent extrapulmo- the igg4 subtype showed a negative correlation between bosis. Risk factors such as DM2, alcohol, COPD, chronic
nary manifestation. Prior to antituberculostatic therapy, its levels and clinical improvement during treatment. kidney disease can lead to a worse outcome. Intensive
this condition was uniformly fatal and still results in high Overall, 65% of patients showed reactivity against IgG 4 treatment varies from 14 days to 8 weeks according to
morbidity and mortality today. The main risk factors are when Mexo antigen was used and this reactivity decreased severity, with carbapenems or ceftazidime. Eradication
associated with compromised immune system; therefore, over the course of treatment.The antigen can be used in treatment lasts up to 12 weeks, using trimethoprim-sulfa-
its occurrence in immunocompetent patients is rare. High the measurement of total igg levels and its subclasses to methoxazole to prevent recrudescence. Doxycycline and
clinical suspicion and early treatment are associated with monitor patients during treatment. Final Comments: it’s amoxicillin-clavulanate are options. In endemic areas, it
better outcomes. Final Comments: Neurotuberculosis imperative to identify and exclude differential diagnosis is crucial to maintain high clinical suspicion in cases in
remains a challenge diagnostic, especially, in immuno- on suspecting IgG4-related disease,due its lack of other which the image suggests the possibility of a diagnosis
competent patients, the high sensitivity and specificity biomarkers and the clinical and laboratory charachteri- of the neurological form, helping the rapid institution
of diagnostic tests are important tools, however, in some ustics may be mimicked by other pathologies. of therapy due to the risk of severe and fatal evolution.
cases, it remains a diagnostic and therapeutic challenge.

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Neuroinfecção

167
XXX Congresso Brasileiro de Neurologia

TL 1106466 TL 1106478 TL 1106493

A INVASIVE INFECTION BY STREPTOCOCCUS REPORT OF THREE CASES OF PATIENTS LIMBIC ENCEPHALITIS POS COVID: A CASE
AGALACTIAE CAUSING MENINGITIS OF WITH PROBABLE CREUTZFELDT-JAKOB PRESENTATION
ATYPICAL PRESENTATION DISEASE IN A PERIOD OF SIX MONTHS IN
Matheus da Costa Guedes, Barbara Matos Almeida
THE SAME REGION OF BRAZIL Queiroz, João Gabriel Dias Brasiliense Frota, Robson
Larissa Brenda Gonçalves Miná, Karla Rafaele Silva
Vasconcelos, Karoline Ferreira Mororó Menezes, Lorena Ohrana Braz Prudente, Marta Rodrigues de de Sales Ferreira, Juliane Lima Fonseca Brayner,
Álissa Elen Formiga Moura, João Igor Landim, Sylvio Carvalho, Beatriz Schmid Dal Berto, Pablo Henrique Karoline Ferreira Mororo Menezes, Glauber de
Ricard Gonçalves de Souza Lima, Ana Silvia Sobreira da Costa Silva, Ronaldo Maciel Dias, Maciel Eduardo Menezes Ferreira, Norberto Anizio Ferreira Frota,
Lima Verde, Noberto Anízio Ferreira Frota, Fernanda de Pontes Tiago Pinho Feijo, Fernanda Martins Maia de
Martins Maia, Glauber de Menezes Ferreira Carvalho
Hospital de Base do Distrito Federal. Brasília DF,
Hospital Geral de Fortaleza. Fortaleza CE, Brazil Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Hospital de Base do Distrito Federal. Brazilia DF, Universidade de Fortaleza. Fortaleza CE, Brazil.
laribgm@gmail.com Fortaleza CE, Brazil
Brazil
Case Presentation: A 33-Year-Old Man, Previously marta_rodrigues12@hotmail.com matheusguedes010@gmail.com
Healthy, Evolved With Severe, Progressive, Persistent
Headache Associated With Vomiting, Photo And Case Presentation: A 54-year-old woman presented with Case Presentation: A 57-years-old woman, hyperten-
Phonophobia And 03 Episodes Of Fever (38-39ºc). Five psychiatric disorder with persecutory delusions, evolving sive, was admitted to the neurologic emergency with a
Days After The Onset Of The Condition, He Developed with focal epileptic seizures for 20 days. On admission, 10-day history of flu-like symptoms. After 24 hours at
Binocular Diplopia When Looking To The Right Side myoclonus was evident in the upper and lower limbs and admission showed lowering the level of consciousness.
And Worsening Of The Headache Intensity. An Skull spasticity in the lower limbs. LCR cultures were negative, Upon neurological exam: spatial disorientation, mem-
Angio-Ct Realized In Another Service That Showed VDRL unreactive, oligogloconal bands absent and 14-3-3 ory loss,irrelevant speech, Babinski sign bilateral and
Indirect Signs Of Intracranial Hypertension And Then protein search present. EEG unchanged. MRI of the skull, Brudzinski sign. SWAB COVID-19: positive. Cerebrospinal
He Sought Medical Care In Our Service After 15 Days signs of restriction and sparse cortical diffusion in the Fluid(CSF) Analysis: pleocytosis: 400 (63% lymphocytes),
Of Onset Of Symptoms. A Lumbar Puncture Was frontal and parietal lobes, and in the striatum on the right. glucose 86mg/dL, protein: 36mg/dL, ADA 0,1, LDH 49,
Performed, Opening Pressure Of 37 Cmh²O, 1.649 Cells A 57-year-old man presented with progressive cognitive PCR multiplex PCR: negative.Therefore, treatment with
(59% Neutrophils), Protein Of 135 Mg/Dl And Glucose impairment associated with depression, two months ago. Acyclovir and Ampicillin, but without improvement after
Of 39 Mg/Dl. The Sample Was Analyzed By Molecular On admission, hypovigil, temporo-spatial disorientation, 14 days of therapy. Furthermore, electroencephalogram
Test Which Detected Streptococcus Agalactiae. Mri Of psychomotor retardation, impaired short-term memory, (EEG) revealed non-convulsive status epilepticus despite
Her Brain Demonstrated Various Focus Of Restriction To myoclonus in upper and lower limbs and face, spastic- phenytoin. Thus, new CSF analysis were performed with
Diffusion In High Frontopparietal Convexity. The Patient ity of the limbs. CSF negative for encephalitis. EEG with panel collection for limbic encephalitis research negative.
Was Treated With Ceftriaxone 4g/Day For 14 Days And diffuse slowing of background activity. Cranial MRI with Also performed magnetic ressonance (MRI) revealed
Evolved With Total Improvement Of Symptoms. Repeated bilateral striated cortex and bilateral lentiform nucleus. A limbic encephalitis pattern. Due to clinical and radio-
Lumbar Puncture After 10 Days Of Treatment: Opening 68-year-old man presented with episodes of spatial dis- logical worsening, considered post covid encephalitis.
Pressure Of 24 Cmh²O, 160 Cells (78% Lymphocytes orientation for a month and a half, evolving with mental Hence, we started treatment with pulse therapy with
And 9% Monocytes), With 46mg/Dl Protein And 52 confusion associated with visual hallucinations, being methylprednisolone 1g for 5 days and de immunoglob-
Mg/Dl Glycorrhachia. Discussion: This Clinical Case admitted to psychiatry with a hypothesis of delirium. ulin for 5 days. She was discharged from Hospital with
Demonstrates An Adult Patient With Atypical Presentation Assessed by Neurology due to spontaneous myoclonus in partial improvement in behavior and interaction with
Of Bacterial Meningitis With An Unusual Etiologic Agent the face and upper limbs. The EEG marked disorganized the environment. Discussion: The Limbic Encephalitis
For The Age Group, Streptococcus Agalactiae (Group B brain electrical activity for age with generalized periodic is a severe inflammatory disease that affects the limbic
Streptococcus, Gbs). Gbs Is A Normal Floral Bacterium discharges. CSF with 14-3-3 protein search present. MRI system. It may have infectious, inflammatory or autoim-
That Colonizes The Gastrointestinal And Genitourinary of the skull signs of diffusion restriction involving the mune etiology. The diagnosis is realized with clinical, EEG,
Tracts, It Is The Main Cause Of Puerperal And Neonatal caudate and putamen bilaterally and symmetrically. The CSF analysis, Serum antibody dosage and neuroimaging.
Infections, Including Meningitis. In Adults, Gbs Meningitis three cases evolved in less than 30 days of Hospitalization The clinical is varied,including involvement of memory,
Is Relatively Rare, Accounting For 0.3 To 4.3% Of All Cases with akinetic mutism, and death from bronchoaspiration cognition,behavioral change, seizures, hallucinations,
Of Bacterial Meningitis. Gbs Meningitis In Adults Usually pneumonia. Discussion: Creutzfeldt-Jakob disease (CJD) confusion and agitation. The neurological manifestations
Occurs In Patients With Underlying Conditions Or In is a rare, rapidly progressive neurodegenerative disease, of COVID-19 including acute encephalopathies, stroke.
Pregnancy/Puerperium, Diabetes Mellitus Is The Most whose diagnosis is based on clinical, radiological, elec- Therefore, Immune Mediated Encephalitis post COVID
Associated Comorbidity, During Hospitalization The troencephalographic or cerebrospinal fluid findings, 19 is the possível cause of encephalitis in this case. Final
Patient Was Found To Be Pre-Diabetic. Domingos Et Al which makes its diagnosis a challenge due to the avail- Comments: COVID 19 and Its complications that need
Revealed That 86% Of The Patients Had Comorbidities ability of exams and the low rate of clinical suspicion. to be studied. The limbic encephalitis as a neurological
And 50% Had A Distant Focus Of Infection. The Clinical Final Comments: Despite the rarity, the three cases were complication of COVID 19 should be recognized and
Picture Of Meningitis By Gbs And The Csf Findings Do identified with a hypothesis of probable CJD, in a period studied as the new etiology.
Not Differ From Other Bacterial Meningitis, However of only six months in the same region of Brazil, with no
Our Case Revealed Itself With 15 Days Of Evolution And relationship of kinship and no report of possible acqui-
Signs Of Intracranial Hypertension. Although Gbs Are 4 Neuroinfecção
sition of an iatrogenic form.
To 10 Times Less Susceptible To Penicillin, Penicillin Is
Still The First Choice, As Is Ceftriaxone. Final Comments:
With The Rate Of Invasive Disease Per Gbs In Adults Neuroinfecção
Increasing, The Physician Must Be Prepared To Recognize
And Initiate Effective Treatment To Avoid Complications
Of The Disease.

Neuroinfecção

168
XXX Congresso Brasileiro de Neurologia

TL 1106547 TL 1106563 TL 1106603

CRYPTOCOCCOSIS AS A CAUSE OF SUCCESSFUL APPROACH: A CASE FUNGAL BRAIN ABSCESS AS


PARAINFECTIOUS CEREBRAL VASCULITIS PRESENTATION OF A PERIAMYGDENTAL A DIFFERENTIAL DIAGNOSIS
ABSCESS WITH SPINAL CORD OF NEUROINFECTION IN
Filipe Nolasco de Souza e Silva, Ana Flávia Souza
Freire da Silva, Mariana Soares Pinheiro, Lucas COMPRESSION IMMUNOCOMPETETENS PATIENTS
Vergne Cheade Lins, Julia Cordeiro Braga, Marcel Rafael Zieglitz Santos, Luana Jessica da Silva Arthur Felipe Barbosa Vasconcelos, Juliana
Leal Ribeiro, Rubson Soares Rocha, Daniel Santana Pontes, Felipe Oliveira Costa, Ingredy Piton Barreto, Magalhães Leite, Matheus Gurgel Saraiva, Francisco
Farias, Renan Carvalho Castello Branco, Luciana Aline Cortes da Silva Moreira, Diego de Oliveira Anderson de Sá Carvalho, José Joaquim Laurindo
Barberino Rocha Ximenes Souza, Claudia do Nascimento Gomes, Sirlene Filho, Mylena Gaudêncio Bezerra, Jeanina Cabral
Hospital Santa Izabel. Salvador BA, Brazil Mendes Borges, Thamyris Vilar Correia, Luiz Carlos Dionizio, Luíza Alves Monteiro Torreão Villarim,
Passos Daniel Vicente de Siqueira Lima Junior, Paulo
filipe.nolasco95@gmail.com Antonio Farias Lucena
Hospital Ana Nery. Salvador BA, Brazil
Case Presentation: Female, 56 years old, with sudden Secretaria Estadual da Saúde da Paraíba. João
rafaelzsan@gmail.com Pessoa PB, Brazil
onset of difficulty opening the left eye associated with dip-
lopia. She reports a history of severe holocranial headache Case Presentation: L.R.N.O., a 60-year-old male, hyper- arthurbarcelos_@hotmail.com
that started 5 days ago. Known comorbidities: Systemic tensive and decompensated diabetic, was admitted in
lupus erythematosus and antiphospholipid antibody syn- July 2021 to the emergency department with ataxia, ery- Case Presentation: Patient in 56 years old, hypertensive,
drome, using Marevan 5mg/day and prednisone 10mg/ thematous patches in upper and lower limbs, edema in presented changes cognitive and behavioral, followed
day. Physical examination: paresis of left eye adduction, large joints associated with odynophagia, and fever. The by progressive impairment of the level of consciousness
left eyelid ptosis, nuchal rigidity. Computed tomography patient was referred to the Hospital unit for cardiology with a gradual evolution in 3 weeks, associated with fever,
of the brain at admission without alterations. Cranial and rheumatology support, and purulent collections were deficits neurological focal and march slowed down. At
MRI showed an acute ischemic vascular insult in the left identified on the topography of the right shoulder and admission, appeared lethargic, ECG 10, no abnormalities
midbrain. CSF study revealed: 240 cells (predominance left elbow. Cervical tomography showed retropharyngeal in the examination of the cranial nerves and with motor
of mononuclear cells); glucose: 13; proteins: 192; Positive collection extending posteriorly to the left paravertebral deficit on the left and without stiffness in back of neck.
China ink; positive latex test for c. neoformans. In view space. An etiologic investigation and antibiotic therapy The Cranial nuclear magnetic resonance (MRI) showed
of the diagnosis of neurocryptococcosis, cerebral infarc- were initiated. In September, the patient developed acute multiple lesions dispersed in the brain parenchyma, more
tion secondary to parainfectious cryptococcal vasculitis tetraparesis and intense cervicalgia and was referred to evident in the nucleocapsular, with annular enhancement
was suspected and therapy was initiated. Discussion: the neurosurgery department, where neck MRI showed after intravenous infusion of the contrast, associate The
Immunosuppressive conditions increase the risk of CNS maintenance of the images, with invasion into the verte- edema vasogenic of parenchyma brain adjacent, and
infections. CNS vasculitis associated with cryptococcal bral canal and compression of the anterior aspect of the restriction of peripheral diffusion the lesions, presenting
infection is rare, with clinical manifestations of cerebral spinal cord from C1 to C3, causing signs of myelopathy. a discrete amount of exudative material in the occipital
infarctions in the internal capsule, thalamus and basal He underwent laminectomy of C1 and drainage of epi- horns of the lateral ventricles, suggestive of of ventriculi-
ganglia. The clinical course has a subacute or chronic dural empyema in that service. After changing antibiotic tis. Treatment was started for bacterial coverage initially
onset, which can usually lead to delays in diagnosis and treatment in which he was admitted to Vancomycin and with ceftriaxone and Oxacillin, then in Meropenem and
adequate treatment. Radiological examinations such as CT Cefepime he progressed to the improvement of strength vancomycin, for the suspected brain abscess. The liquor
and magnetic resonance imaging (MRI) contribute to the in all 4 limbs, cervicalgia, and headache. Despite cultures collection not performed due to injuries with edema
visualization of infarcted areas, emphasizing the value of collected, the infectious agent was not identified. Deep cerebral and Detour in line average. THE investigation
angiography with evidence of vasculitis. The diagnosis is neck space infections are suppurative infectious processes for HIV and immunosuppression were negative. Despite
confirmed through confirmation in the cerebrospinal fluid that can cause abscesses and affect deep visceral spaces of antibacterial therapy, patient remained with clinical and
with nanquin ink and culture. Treatment should be insti- the neck such as the parapharyngeal space and the danger laboratory tests worsening, and it was decided to start in
tuted as soon as possible, aiming to reduce morbidity and space. A common etiology is the spread of oropharyngeal therapy with amphotericin B thinking in abscess fungal,
mortality from the disease. Final Comments: Although infections, such as odontogenic infections and tonsillitis, although gives renal dysfunction presented by ampho-
rare, it is important to know this presentation in neuro- through fascial planes. Deep cervical infections can be fatal tericin B, patient evolved with improvement of the clinic
cryptococcosis, since early diagnosis and management due to jugular vein thrombosis, airway obstruction, medi- status, being discharged from the intensive care unit. At
provide a reduction in patient morbidity and mortality. astinitis, and sepsis. This paper aims to report a successful Hospital discharge, the patient is alert, no language defi-
case of a periamygdala abscess with deep cervical dissem- cit, no motor deficit, mild gait slowing, without stiffness.
ination, resulting in tetraparesis and a dismal prognosis. Control cranial MRI examination after Hospital discharge
Neuroinfecção
The evolution of antibiotic therapy was responsible for a evidence decrease of edema and of injuries presented.
decrease in the incidence of deep cervical space abscesses. Discussion: Fungal brain abscess is a life-threatening
However, in the last decade, the number of this type of condition, more common in immunocompromised indi-
abscesses has increased. The patient follows this trend viduals, but with increasing reports in the literature in
and reveals systemic comorbidities of diabetes mellitus immunocompetent patients. This case highlights the
without disease control. Therefore, although tonsillitis is importance and need for a higher level of suspicion and
a common infection, it can generate important and fatal sensitive diagnostic methods to optimize therapy and
complications. In this paper we present a case presenta- improve case outcomes. Final Comments: Despite of
tion with significant neurological involvement, such as limitations of service for realization of liquor analysis,
epidural empyema, with severe headache, tetraparesis, the importance of evaluating images that suggest fungal
and cervicalgia, giving a dismal prognosis. However, the abscess were important for outcome positive of case.
patient had a positive prognosis.

Neuroinfecção
Neuroinfecção

169
XXX Congresso Brasileiro de Neurologia

TL 1106632 TL 1106635 TL 1105522

THE REFRACTORY TREATMENT IN A LEGIONNAIRE’S DISEASE ASSOCIATED – POSTERIOR REVERSIBLE ENCEPHALOPATHY


PATIENT WITH SYPHILITIC UVEITIS: A CASE SEVERE ENCEPHALOPATHY SYNDROME AFTER CHRONIC OBSTRUCTIVE
PRESENTATION PULMONARY DISEASE : A CASE
Marcela Santarelli Casella, Victor Cardoso de
Faria, Andre Lopes Valente, Jose Marcos Vieira PRESENTATION
Matheus da Costa Guedes, João Igor Dantas
Landim, Robson de Sales Ferreira, Gabrielle Albuquerque Filho, Jose Luiz Pedroso, Orlando Daniel Sampaio Souza, Wendell Silveira de Souza,
Benevides Lima, Arthur Holanda Moreira, Karoline Graziani Povoas Barsottini João Victor Oliveira de Melo, Victória Rosas
Ferreira Mororo Menezes, Matheus Andreghetti Marques, Bruna Guimarães Dutra, Danilo Issa
Universidade Federal de São Paulo. São Paulo SP,
Rossi, Flavia de Paiva Santos Rolim, Sarah Diogenes Mitozo Veras, Neyde Alegre de Souza Cavalcante,
Brazil
Alencar, Fernanda Martins Maia de Carvalho João Pedro Moreira Guilherme, Vanise Campos
marcela.santarelli@hotmail.com Gomes Amaral, Tales Oliveira Junior
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Universidade de Fortaleza. Fortaleza CE, Brazil Universidade Estadual do Amazonas. Manaus AM,
Case Presentation: A 57-year-old brazilian man pre-
sented to the emergency department with a 3-day history Brazil
nosbordesales@gmail.com
of diarrhea and hiccups. On the next day, he developed Universidade Federal do Amazonas. Manaus AM,
incoordination followed by change in speech and vom- Brazil
Case Presentation: A 23 year old man was admitted in
iting episodes. Symptoms worsened, until he was unable Fundação Hospital Adriano Jorge. Manaus AM,
emergency with progressive bilateral vision impairment,
to stand. While waiting for medical attention, he became Brazil
which started one month ago with ocular hyperemia and
dark spots. Also reported history of adynamia, hyporexia confused, reporting delusions and hallucinations. He had a dss.med18@uea.edu.br
and fever. Upon neurological exam: pupils with light-near past history of smoking, cocaine use and moderate alcohol
dissociation, fundoscopy: bilateral posterior synechiae consumption. On examination, he was drowsy, disoriented Case Presentation: ERS, male, 61 years old, born in
and right eye with optic nerve with infiltrative-appear- in space and time, dysarthric, apparent restriction of ver- Itacoatiara, Amazonas. He entered the emergency depart-
ing papilledema. The laboratory tests revealed a vene- tical upward gaze bilaterally. He had astasia and abasia. In ment on January 20, 2022 being immediatelly intubated
real disease research laboratory test (VDRL) 1: 1024 the emergency room, he became agitated and developed and sedated. His family tells a history of chronic obstruc-
and HIV reagent, confirmed by two positive samples. tachycardia, tachypnea, fever (40 C) and was intubated tive pulmonary disease (COPD) with exacerbation in the
Cerebrospinal fluid (CSF) analysis showed non-reactive and sent to ICU. Blood screening revealed elevated CK of last 2 weeks causing respiratory failure due to communi-
VDRL and increased protein. A diagnosis of neurosyphilis 18.306 (normal: 39-308), thrombocytopenia high creati- ty-acquired pneumonia. He had systemic arterial hyper-
was made. Treatment with crystalline penicillin and eye nine levels of 1,55, hyponatremia of 124, hypokalemia of tension and heart failure. Somatoscopy showed normal
drops (corticoid and tropicamide) was instituted. After 21 3,3, low ionized calcium of 1,08, mild elevated ALT/AST, color, hydrated, anicteric, acyanotic, and afebrile. Physical
days of crystalline penicillin with persistent lesions in the elevated troponin of 50 and elevated CRP of 244. Chest examination revealed edema of the lower limbs and the
eye, and evidence of capture contrast in basal ganglia in TC showed an extensive consolidation affecting almost respiratory system showed diffusely abolished breath
cranial magnetic resonance (MRI), empirical treatment the entire right lower lobe and minimal bilateral pleural sounds, with no other complaints. On January 27, the
for toxoplasmosis neuroretinitis was discussed and sul- effusion. CSF studies revealed 9 leukocytes (88% lym- patient had a seizure being medicated with phenytoin
fadiazine was prescribed to treat neurotoxoplasmosis. phocytes, 12% monocytes), 5 erythrocytes, glucose of and, later, escalated with an association of valproate,
The patient was later reassessed by the ophthalmologist, 88 mg/dL, lactate of 27,5. Culture and viral panel were phenobarbital and clonazepam. On this same day, a
showing improvement in the retinal edema. Discussion: negative. Brain MRI was normal. Hydratation for rhabdo- cranial tomography (CT) was performed, which showed
Syphilitic uveitis comprises less than 1-2% of all uveitis myolysis, Ceftriaxone and metronidazole were the initial hypodense lesions in the posterior fossa, in addition to
cases, however an increase in the number of cases over treatment. Because of lack of response Levofloxacin was partial emptying of the sella turcica, which correlated with
the past decade was observed. The manifestations are started with positive response. Positive Legionella Urine intracranial hypertension. In view of the clinical findings
diminished visual acuity, blindness, optic neuropathy, confirmed Legionnaire’s disease. After recovering, he was and the imaging exam, posterior reversible encephalop-
interstitial keratitis and it can occur at any state of natu- discharged home but with residual ataxia and dysarthria athy syndrome (PRES) due to COPD was observed and
ral history of untreated syphilis. In the present case, even in improvement. Legionnaire’s disease is an infection in the same period the patient complicated with a sub-
with CSF non-reactive, the signs of active uveitis and the caused by Legionella pneumonia that usually manifests arachnoid hemorrhage which was reabsorbed later. Lastly
VDRL titulation suggested neurosyphilis. Neuroretinitis is with pneumonia. Extrapulmonary manifestations are Patient evolved to death due to cardiac arrest due to the
a phenotype of syphilis uveitis and is described as a clin- rare, but meningitis, encephalopathy, which can be from lack of possibility of long-term treatment. Discussion:
ical triad of unilateral vision loss accompanied by optic mild to coma, cerebellar dysfunction and focal deficits. PRES is a neurotoxic event which results a fluid extrava-
disc edema and the formation of a characteristic macular Brain MRI can present with hyperintensities at multiple sation marked often by the presence of vasogenic edema
star. Pacientes should test for HIV infection either, because localizations CSF findings are normal in the vast major- in the parieto-occipital region in CT or magnetic reso-
the co-infection are frequent. A few cases of neuroretinitis ity of patients, raising the possibility that Legionella does nance imaging (RMI) with clinical features like altered
by Toxoplasma gondii have been reported, however the not directly affect the central nervous system. Therefore, mental status, headache, seizures and vision loss. This
presence of hiperdense lesions in basal ganglia in MRI pathophysiology is uncertain and it is unknown whether syndrome is due mainly from secondary chronic diseases
associated with lack of improvement of ocular lesions sup- the mechanism is an immune, toxin-mediated or a direct such as hypertension. In addition, it may be complicated
port the treatment choice for neurotoxoplasmosis. Final damage by the bacteria.The patient was followed and per- by subarachnoid hemorrhage which, despite its not fully
Comments: Immunosuppression by HIV may contribute sisted with ataxia and dysarthria, in slow recovery three understood mechanism, progresses in most cases with
to the appearance of opportunistic infections with atypi- months after discharge. bleeding reabsorption within a few days. Finally, due to
cal involvement, such as neuroretinitis in toxoplasmosis the reversible behavior of PRES, improvement in the con-
and syphilitic uveitis. For the adequate management of dition generally occurs after controlling the underlying
these patients a thorough etiological investigation should Neuroinfecção
disease. Comentários finais. In this case, we present a
be performed, as well as the observation of improvement curious and rare manifestation of PRES correlated with
after treatment. COPD exacerbation. In patient care, all possible measures
to be used in the service were adopted, however, some
limitations can be mentioned, such as the lack of long-
Neuroinfecção term therapeutic possibilities, which culminated in the
patient’s death. In this context, further discussions on
future advances and research in the field are necessary.

Neurointensivismo

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XXX Congresso Brasileiro de Neurologia

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CASE PRESENTATION ACUTE CANDIDA ALBICANS AND ADULT ONSET OPSOCLONUS-MYOCLONUS-


CEREBROVASCULAR EVENT WITH COVID-19 STENOTROPHOMONAS MALTOPHILIA: ATAXIA SYNDROME WITH SEIZURE AND
INFECTION, A RARE AND SERIOUS ATYPICAL CASES OF MENINGITIS IN A RELATED TO EPSTEIN BARR VIRUS
COMPLICATION NEUROINTENSIVE UNIT
Micael Porto Portela Lima, Euldes Mendes Júnior,
Isadora Lyrio Stábille, Luiza Barbosa de Oliveira, Raimundo Marcial de Brito Neto, Naiade Andrade Mikaio de Sousa Jucá, Júlio César Claudino dos
Luiza Carolina Moreira Marcolino, Thiago Vieira Couto, Catarina Sodre de Castro Prado, Viviane Santos, Idna Lara Goes de Sena, Ricardo Felipe
Sasse, Renata Coelho Chaves Gaspar, Ruth Maria Alexandre da Silva, Rodrigo Queiroz Silveira dos Santos, Tonyato Fernandes Ribeiro Maia, Fábio
Ribeiro Guerra, Camilla Ribeiro de Oliveira, Alex Antonio Serra de Lima Júnior, Leandro Freitas
Eduardo da Silva Universidade Federal Fluminense. Niteroi RJ, Brazil Oliveira
Complexo Hospitalar de Niterói – Niteroi – RJ –
Universidade Federal do Triângulo Mineiro UFTM Brazil Centro Universitário Christus – Unichristus.
Fortaleza CE, Brazil
luizaboliveira@outlook.com.br marcialbrito@live.com Universidade Federal de Minas Gerais. Belo
Horizonte MG, Brazil
Case Presentation: A 60-year-old male patient, diabetic, Case Presentation: 1. Female, 85 years old, previously Universidade Federal da Paraíba. João Pessoa PA,
hypertensive and previously vaccinated with one dose of hypertensive and diabetic, presented, on July 15, 2021, Brazil
COVID-19 vaccine ( ChAdOx1), was isolated at home for HSA-fisher 3, being admitted to the Neurointensive Unit. Santa Casa de Monte Carlos – Monte Carlos – MG
COVID-19, with a positive antigen test and first symptoms He evolved with hydrocephalus and intracranial hyperten- – Brazil
13 days before admission. He was admitted presenting sion. On July 16, an external ventricular shunt was placed Universidade Federal da Paraíba. Belém PA, Brazil
aphasia and right hemiplegia, with no respiratory dis- with an intracranial pressure monitor. On the third post- Universidade Federal de São Paulo. São Paulo SP,
tress. His cranial computed tomography (CT) had an operative day, he showed fever and worsening inflamma- Brazil
extensive commitment at the territory of irrigation of tory patterns. In the CSF study, it had an opening pressure
the middle cerebral artery (MCA). Another finding was of 20cmH2O, with 18773/mm 3 cells (75% neutrophils), micaelppl1@gmail.com
internal carotid and MCA occlusion in CT angiography. 181 mg/dL protein, 20 mg/dL glucose. CSF culture, with
Chest CT showed a 40% parenquimal commitment with growth of Gram negative Stenotrophomonas malthopilia Case Presentation: A 49 year-old-man previously healthy
ground-glass opacification, and bronchopneumonia. sensitive to levofloxacin and tigecycline. After one month started with fever, headache, malaise, swallow pain and
He was treated with antibiotics, corticotherapy. During of treatment, he died, despite the medication; 2. A 34-year- was diagnosed with pharyngitis and treated with azitro-
Hospitalization he presented with delirium and acute old male with no previous comorbidities is admitted to the micin and amoxicillin clavulanate, without fever resolu-
respiratory distress, afterward, thromboembolism was Neurointensive Unit to investigate a seizure. The patient tion. Twelve days after initial symptoms, he had a gen-
diagnosed by CT angiography. Then, mechanical venti- had no history of epilepsy. It was reported that, a week eralized tremor and presented a tonic-clonic seizure. It
lation was needed for 6 days. The patient had a 2 months ago, he had started with a mild headache and tremors of was described anxiety and irritability of the patient. On
stay in Hospital, due to urinary tract infection, persistent the extremities, which was followed by a fever measured neurological examination, he had generalized tremor
fever and pseudomembranous colitis. Finally, he was between 38-39°C and blurred vision. In emergence, kernig with head and axial titubation, GCS 15, dysarthria, ocular
discharged with no oxygen supplementation and was and brudzinski signs are present. Laboratory tests and motricity with saccades intrusions and opsoclonus, cere-
prescribed anticoagulation. Moreover, he maintained his magnetic resonance imaging of the skull were performed, bellar ataxia, tremor with kinetic, postural and intention
motor impairment and improved his aphasia. Discussion: without alterations. In neurointensive care, liquor was characteristics and myoclonus. He was started on acy-
COVID-19 is known as a proinflammatory state associ- collected, which showed PCR detecting Candida albi- clovir. Brain MRI was normal. CSF with pleocytosis and
ated with thrombophilia. There is an increased risk of cans. He was then treated with liposomal amphotericin high protein. CBC had leukocytosis with lymphocytosis.
thrombotic events, even though stroke is considered B and fluconazole. About two weeks later, the patient’s HIV, HBsAg, VDRL and HCV serologies were negative.
rare. The incidence of ischemic stroke associated with symptoms improved significantly and he was discharged Cytomegalovirus (CMV) IgG serology was positive and IgM
COVID-19 patients has ranged from 0.4 to 2.7 percent. A from the Hospital. Discussion: Meningitis caused by S. negative. HSV was positive for IgM and IgG. Opsoclonus
study showed that COVID-19 patients were more likely maltophilia is rare, with only 18 cases reported so far. Myoclonus Ataxia Syndrome (OMS) diagnosis was given.
to have a cryptogenic stroke. Outcomes were worse, Populations at risk for S. maltophilia meningitis are typi- Methylprednisolone was given, with significant clinical
including longer length of stay, intensive care unit care cally immunocompromised patients, those who have had improvement. The case presented all four symptoms of the
(ICU) stay, greater rate of neurological worsening, and neurosurgical procedures, premature infants, and patients syndrome: opsoclonus, myoclonus of trunk and extrem-
in-Hospital death. In comparison, our patient had a requiring prolonged Hospitalization. Multi-agent resis- ities, step ataxic, and behavioral change. In adults, there
long length stay, with ICU care needed. Nevertheless, tance is a hallmark of S. maltophilia. Regarding the fungal is a strong paraneoplasic association, in particular with
our patient had a atherothrombotic event and evolved meningitis described, its prevalence began to decrease small cell lung carcinomas, breast and ovarian neoplasia,
with a neurological improvement. Similar to our case, in the last decade, while the frequency of detection of C. leading us to perform CT screening of chest, abdomen and
another article demonstrated the association between albicans species gradually increased. It presents manifes- pelvis, all negative. Brain MRI was made, also normal. Also
ischemic stroke and pulmonary embolism. Besides,the tations similar to those of bacterial meningitis, making relevant in adults are infections, being the most related
median time between the onset of COVID-19 symptoms the initial use of antibacterials not uncommon. It has a EBV, CMV, HCV, HIV, HSV, post-streptococcal. In our case,
and diagnosis of stroke was 2 weeks. Final Comments: good prognosis after the institution of the correct ther- the onset was compatible with infectious mononucleosis.
Stroke appears to be a rare but serious event associated apy. Final Comments: Although these atypical menin- The presence of IgM and IgG antibodies for HSV is prob-
with COVID-19 patients. Considering the poor outcome gitis remain rare in the population, physicians should be ably a cross reaction with EBV. Echocardiogram shown
described in literature, our patients, despite all complica- aware of them as possible meningitis-causing organisms a mild pericardial effusion. A remarkable characteristic
tions, had improvement of his conditions. However, the for better therapeutic follow-up, even in the absence of is the seizure. We didn’t find any case presentation of
characteristics of COVID-19 patients with stroke vary in the their known risk factors. OMS in literature associated with tonic-clonic seizure.
current literature, therefore more description is needed. The investigation of the patient rule out encephalitis.
Our explanation for the seizure was a cortical disfunction
Neurointensivismo without patterns of encephalitis. Thus, our case presen-
Neurointensivismo tation is the unique to literature with this specific type
of presentation. OMS is a rare entity with difficult diag-
nosis. Recurrence is common, and there are few studies
on prognosis of this population. In our case, a detailed
history and neurological examination was crucial to sus-
pect OMS, using opsoclonus as an important feature to
accurate diagnosing. We ruled out tumors and stabilished
early treatment, resulting in good clinical improvement.

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XXX Congresso Brasileiro de Neurologia

TL 1105901 TL 1106155 TL 1105026

POSTERIOR REVERSIBLE ENCEPHALOPATHY OCULAR MOTILITY IN THE DIAGNOSIS OF VERY EARLY-ONSET BEHAVIORAL VARIANT
SYNDROME ASSOCIATED WITH POST- COMA FRONTOTEMPORAL DEMENTIA ASSOCIATED
VACCINATION GUILLAIN-BARRÉ SYNDROME, WITH FUS GENE IDENTIFIED VARIANT: A
Erika Christina Silva, Marcus Vinicius Della Coletta,
WOULD IT BE POSSIBLE? Augusto Carvalho Bezerra, Silas Fernandes Avelar CASE PRESENTATION
Rodrigo de Paiva Bezerra Junior Marina Scop Medeiros, Cristiano Schaffer Aguzzoli,
Hospital Universitário Francisca Mendes. Manaus Rodrigo Rosa de Stefani, Júlia Patatt, Eduarda
Hospital Alemão Oswaldo Cruz. São Paulo SP, Brazil Kotlinsky Weber, Antonella Brum de Carvalho,
AM, Brazil
rodbzmd@gmail.com Universidade do Estado do Amazonas. Manaus AM, Laura Gomes Pereira, Renata Diefenthaler Campos,
Brazil Rodrigo Twardowski Scherer, Lucas Porcello
Case Presentation: 70-year-old female with hyperten- Schilling
sion, obesity and anxiety, taking atenolol and citalo- erika_csilva@outlook.com
Hospital São Lucas da PUCRS, Instituto do Cérebro
pram. Admitted with weakness after 7 days of receiving do RS – Porto Alegre – RS – Brazil
the Janssen-COVID19 vaccine. She evolved with hyper- Case Presentation: A 52-year-old male, obese, hyperten-
sive and non-dialytic chronic renal patient underwent Global Brain Health Institute, Memory and Aging
active delirium, in addition to paraparesis and hypore- Center, University of California San Francisco UCSF
flexia that were difficult to assess, admitted to the ICU for coronary artery bypass graft surgery and wide endar-
terectomy, with sixty-five minutes of cardiopulmonary Escola de Medicina da PUCRS PUCRS, Instituto do
delirium control. Head CT with signs of subarachnoid Cérebro do RS – Porto Alegre – RS – Brazil
hemorrhage (SAH) in cortical sulci in the frontal right bypass (CPB) and forty-five minutes of aortic clamping
time, without intercurrences. He progressed in the post- Escola de Medicina da PUCRS, Instituto do Cérebro
convexities. Cerebral angiography initially without any do RS – Porto Alegre – RS – Brazil
vascular. She had been presenting with tachycardia and operative period with weaning from vasoactive drugs,
hypertension despite treatment. She presented worsen- maintenance of renal function and reduction of medias- marina.scop.medeiros@gmail.com
ing of the motor condition and after 7 days of control of tinal drain output. Sedation was turned off 48 hours after
the delirium. Brain MRI was performed showing hyper- the surgical procedure, without awakening or adequate Case Presentation: A 24 year-old male developed pro-
signal in T2/FLAIR cortico-subcortical in the gyri of the respiratory driving after five days. On physical examina- gressive neuropsychiatric symptoms following a mild
convex encephalic convexities, symmetrical, notably tion, the patient remained in a comatose state (Glasgow depressive episode, with compulsive behavior, hyper-
frontoparieto and occipital, without restriction on diffu- coma scale 3/15), with mild alternating conjugate ocular orality, disinhibition and, finally, psychotic symptoms
sion; suggesting as posterior reversible encephalopathy deviations. A cranial tomography was requested, which such as delusions and psychomotor agitation, leading
syndrome (PRES). The RCVS2 score was 5. Nimodipine showed an increase in the density of the basal ganglia to a psychiatric Hospitalization nearly 10 months after
was started, and increased intracranial artery velocities symmetrically and of the thalamus. In the intensive care symptom onset. The patient was initially diagnosed with
were observed on transcranial Doppler. After 2 weeks, unit, ocular signs may have prognostic, diagnostic and schizophrenia and received several treatment attempts,
presented with sudden cortical blindness; then submitted therapeutic value. Changes such as ptosis, nystagmus and including neuroleptics, SSRIs, electroconvulsive ther-
to arteriography showing diffuse vasospasm in addition pupillary dilation have different diagnostic values and can apy and empiric high-dose corticosteroids, all of which
to areas of subocclusion in P3 left segments, and received help in a faster and more adequate management of the were ineffective. He was discharged after 6 months. The
intra-arterial milrinone. For 15 days, she had continuous case. In postoperative cardiac surgery units, especially in patient progressed with severe cognitive and functional
milrinone and noradrenaline and nimodipine until the procedures with cardiopulmonary bypass, cerebrovascu- decline within months. At 26 years old, he was referred
vasospasm improved. Control MRI showed areas of right lar accidents (CVA) should always be included in the list to our outpatient clinic. On examination, he was globally
frontal, bilateral parieto-occipital and right cerebellar of differential diagnoses, since up to 14% of patients can aphasic and only vocalized a repetitive grunt. He had
subacute/chronic ischemic events. CSF with protein-cy- develop CVA, as well as intraoperative hypoxic lesions. frontal release signs, mild generalized muscular atrophy
tological dissociation, was submitted to plasmapheresis, The roving conjugate eye is a slow, conjugated, lateral and and paratonia. Of note, he did not have motor neuron
due to a post-vaccination Guillain-Barré Syndrome (GBS) symmetrical movement, characteristic of coma and sug- disease. The patient had 11 years of schooling and nor-
confirmed by an electroneuromyography study. Evolved gestive of ischemic or metabolic lesions. Imaging exams mal childhood development. He had no history of CNS
with improvement of the delirium, treating neuropathic may present without significant changes, which does not infections or trauma, nor previous psychiatric disorders.
pain and motor rehabilitating. Discussion: Recognition exclude brain injury. In the case in question, despite the Family history included a paternal uncle with schizophre-
of non-aneurysmal SAH causes is still a challenge for the reduced CPB time and the absence of significant meta- nia. Extensive investigation ruled out systemic diseases.
clinician. Systemic assessment of the patient and not just bolic changes, the risk factor of obesity and the need for Neuroimaging performed 1-2 years after symptom onset
the neurovascular condition is extremely important to endarterectomy may have led to small ischemic lesions, showed severe atrophy in the hippocampus and head
recognize the PRES and its possible triggering factors; in addition to cardiopulmonary bypass, which constitutes of the caudate bilaterally. Genetic testing identified a
despite uncertainties regarding the pathophysiology of a risk factor isolated for hypoxic and ischemic injuries. variant of uncertain significance (VUS) in the FUS gene:
a minority of cases evolving with unfavorable outcomes. Ophthalmological examination is an important step in p.Gly229_Gly231dup. Discussion: This is a case of very
Final Comments: The association of GBS with a post-vac- the physical examination of patients in intensive care early-onset behavioral variant frontotemporal degenera-
cination condition is already well established in the litera- and should be a widespread knowledge among attend- tion (bvFTD) clinical phenotype with an unusual rapidly
ture, but with few reported cases of association with PRES ing physicians. Although there are imaging tests that progressive course. The clinical history and neuroimaging
of severe evolution. Despite the use of medication known complement and guide, sometimes they become insuf- support a hypothesis of FUS pathology, which might be
to be associated with some antidepressant medications; ficient in the diagnosis, and the clinical examination is an related to the found variant. The fact that the same VUS
its association with GBS is poorly described. indispensable tool for better management of the patient. was found in the patient’s asymptomatic 58 year-old father
raises uncertainty for establishing this relation, although
most FUS gene mutations show incomplete penetrance.
Neurointensivismo Neurointensivismo
Importantly, the identical VUS has been reported with
essential tremor and ALS. Other FUS gene variants have
been reported, but without pathological confirmation. If
neuropathology demonstrates FUS protein deposition,
this could be the first case of FUS mutation potentially
causing FTLD-FUS pathology. Final Comments: FTLD-
FUS pathology must be suspected in sporadic, early-on-
set and rapidly progressive bvFTD. The found VUS could
represent a novel FUS gene mutation related to bvFTD,
although additional evidence is needed to determine its
pathogenicity.

Neurologia Cognitiva e do Envelhecimento

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“DYSMETRIA OF THOUGHT”: CEREBELLAR ATYPICAL DEMENTIA WITH LEWY BODIES: A AGRAMMATIC PRIMARY PROGRESSIVE
STROKE AS A POSSIBLE CAUSE OF CASE PRESENTATION APHASIA DUE TO GNR MUTATION: A CASE
STRATEGIC INFARCT DEMENTIA PRESENTATION
Tereza Brenda Clementino de Freitas, Maria Clara
Patrick Emanuell Mesquita Sousa Santos, Ana Barbosa de Oliveira, Leila Gabriele Nunes Silva, Rodrigo Twardowski Scherer, Fernando Tonon
Beatriz Marangoni Baston, Pedro Machry Pozzobon, Vinícius de Noronha Barboza, Murilo Tomaz Rocha, Schneider, Marina Scop Medeiros, Cristiano
Tarcísio Nunes Alvarenga, João Vitor Mortari Lisboa, Vítor Silveira Reis Canêdo, Eloisa Alves Viana, Schaffer Aguzzoli – Estados Unidos, Wyllians José
Igor Oliveira Fonseca, Rodrigo Bazan, Igor Lima Gabriel Maciel Morais, Samila Marissa Pinheiro Vendramini Borelli, Julia Patatt, Matheus Padão
Teixeira Gomes Schuster, Jorge Ernesto Miyazaki Araujo, Laura
Gomes Pereira, Lucas Porcello Schilling
Hospital de Clínicas da Faculdade de Medicina de Universidade Federal Rural do Semi-Árido. Mossoró
Botucatu – UNESP. Botucatu SP, Brazil RN, Brazil Escola de Medicina – Pontifícia Universidade
Católica do Rio Grande do Sul PUCRS
pedro_machry_pozzobon@hotmail.com terezabrenda13@gmail.com
Global Brain Health Institute, Mamory and Aging
Center, University of California San Francisco UCSF
Case Presentation: A 71-years-old woman, 8 years of Case Presentation: Female, 65-year-old, refers restless
Instituto do Cérebro do Rio Grande do Sul InsCer
schooling, presented to Hospital with intense vertigo, sleep, visual hallucinations and sporadic forgetfulness
InsCer
vomiting and headache. Brain CT showed cerebellar for 1 year. Family reports spatial disorientation, with an
(CB) infarct in vermis and CB right hemisphere, diag- intensification of the hallucinatory condition for 1 month, rodrigotscherer@hotmail.com
nosed CB stroke. After discharge, the patient developed period in which persecutory delusions were presented.
a non-progressive cognitive decline with amnestic com- Developed, after administration of risperidone, difficulty Case Presentation: Male, 70 years old, caucasian, aircraft
plaints, with executive, attention and social cognition in motor control and, after replacing it with aripiprazole, mechanic and 11 years of schooling. Patient sought care
declines. After 9 months, she presented to our service to evoluted with worsening of disorientation and delusions. for behavioral and speech changes that had been progress-
cognitive investigation. The neurological examination Her education attainment level was college and the Mini- ing for one year. Patient was recently sadder, quieter, with
showed right skew deviation and horizontal nystagmus, Mental State Examination (MMSE) scored 20, suggesting decreased social interaction and functional decline. He’d
besides right dysmetria and dysdiadochokinesia. In cog- cognitive impairment. Brain magnetic resonance imag- previously no neurological symptoms. His mother died
nitive tests, she scored 22/30 in the Mini Mental State ing showed rare foci of microangiopathy, as well as small at the age 86 due to dementia without specific diagnosis.
Examination; 19/30 in the Montreal Cognitive Assessment, foci of gliosis in the right thalamus, with no evidence of His older brother is diagnosed with cortico-basal syn-
with Clinical Dementia Rating Scale (CDR) score equal parenchymal atrophy. Fludeoxyglucose positron emis- drome and his younger brother has no diagnosis but he
to 1 and Frontal Assessment Battery scale score of 14/18. sion tomography (FDG-PET) demonstrated bilateral has similar symptoms to the patient. Neuropsychological
In the Schmahmann Syndrome Scale, a specific office parieto-occipital hypometabolism, with preservation assessment was notable for significant deficits in the
to evaluation of cognitive impairment in CB lesions, from medial portions of occipital lobes. Discussion: Boston Naming Test (-3.8 SD), MOCA test (7/30), along
the patient scored 52/120 points, with fail in 9/10 tests. This report describes a case of dementia of Lewy bodies with impaired comprehension of complex sentences, non
Laboratory tests were unremarkable. Brain MRI showed (DLB). History establishes cognitive changes impacting fluent speech and altered memory, executive function and
increased T2/FLAIR signal in area of previous CB stroke. function consistent with dementia, a required criterion, attention. Brain MRI showed atrophy especially in the left
A diagnosis of probable cerebellar vascular cognitive with early deficits in executive function and visuoper- frontal lobe. A genetic investigation found the GRN T272fs
decline was made. Discussion: The cerebellar cognitive ceptual ability. Plus, the patient presents core features of mutation (exon 8, c.813_816del), establishing a genetically
affective syndrome (CCAS), was described first in 1998, DLB: fluctuating cognition, rapid eye movement (REM) confirmed case of non-fluent variant frontotemporal
also named Schmahmann’s syndrome, is characterized sleep behavior disorder and recurrent visual hallucina- dementia (nfvFTD). Discussion: Frontotemporal demen-
by declines in executive functions, linguistic processing, tions, meeting criteria for probable DLB, established tia (FTD) is a clinical and pathologically heterogeneous
spatial cognition, and affect regulation. The “dysmetria by the presence of two or more core clinical features or syndrome characterized by behavioral, language and
of thought hypothesis” attributes the cerebellar-related one core clinical feature plus one indicative biomarker. executive abnormalities. The spectrum of presentation
cognitive declines to changes in pathways connecting Antipsychotic hypersensitivity and systematized delu- includes behavioral variant frontotemporal dementia
the CB with the limbic circuitry, prefrontal and parietal sions are supportive clinical features. Further testing can (bvFTD), non-fluent variant primary progressive apha-
association cortices. Part of patients with CB lesions and be helpful to establish the diagnosis, and the presence of sia (nfvPPA) and semantic variant primary progressive
cognitive complaints present normal ranges in tests as occipital cortex hypometabolism on FDG-PET imaging aphasia (svPPA). Unlike the clinical syndrome, the term
MoCA and MMSE. Thus, in 2018 was published the CCAS is an essential tool in differentiation between DLB and frontotemporal lobar degeneration (FTLD) is reserved to
Scale, that recently in 2022 was translate and validated Alzheimer’s disease, the latter with typically temporopari- describe pathological changes of the disease that encom-
to Portuguese. In our case, we used the validated ver- etal hypometabolism. The parieto-occipital pattern seen in passes the syndrome. So FTD is characterized by a disorder
sion of CCAS scale and we detected significant decline this case, when aligned with the characteristical patient’s with distinct clinical phenotypes that can coexist and are
in CB functions associated to a related subtle decline in clinic, suggests an atypical image of DLB. Treatment is associated with multiple neuropathological substrates.
functional daily activity, based on CDR scale, indicating symptomatic to address cognitive, motor and other non- NfvPPA is characterized by degeneration of frontal lan-
probable dementia. In addition, symptoms were reported motor symptoms, and involves avoiding medications that guage networks in the brain. It usually manifests in late
to have started soon after acute CB injury and there was may exacerbate symptoms, besides palliative care. Final middle age with minor difficulties with speech but with
no significant report of progressive worsening of these Comments: DLB is diagnosed in the context of dementia preserved executive and cognitive functions that, with dis-
symptoms. Final Comments: Cerebellar stroke can be with clinical core features of DLB on history and physical ease progression, are also impaired. In etiological terms,
a cause of vascular cognitive impairment, which in this examination, with or without indicative biomarker. DLB it should be noted that mutations in MAPT, C9ORF72 and
case fulfills the criteria for the diagnosis of a dementia leads to a decline in functional outcome and quality of GRN are the main causes of FTLD, accounting for 30% of
syndrome, thus also fulfilling the criteria for the diagnosis life, requiring a multidisciplinary approach. the cases. Besides, the most common GRN mutation is
of dementia due to strategic infarctions. T272fs, with a known founder effect in Italy, where the
patient’s family is descended. Final Comments: PPA is
Neurologia Cognitiva e do Envelhecimento an uncommon and underdiagnosed disease often mis-
Neurologia Cognitiva e do Envelhecimento diagnosed with Alzheimer’s. It’s important to highlight
that genetic investigation has a main role to confirm
diagnosis, allowing better therapeutic approach and even
genetic counseling.

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POSTERIOR CORTICAL ATROPHY: VARIANT NEUROPSYCHOLOGICAL ALTERATIONS AND CREUTZFELD JAKOB DISEASE AND COVID
OF ALZHEIMER’S DISEASE RELATED PET/CT ABNORMALITIES AFTER COVID-19 19: A CASE PRESENTATION
TO PSEN1 MUTATION MANIFESTING AS INFECTION: CASE PRESENTATION
Alex Bertolazzo Quiterio, Emanuelle Bianchi da Silva
RAPIDLY PROGRESSIVE DEMENTIA Rocha, Janaina Moraes de Araujo, Laura Alonso
Felipe Cesar Gomes Andrade, Amanda Sabino Pinho
Laís Bissoli Perini, Fernando Zanette, Matheus Sales, Carolina Medina Duarte, Bruno Paulo Teles Matheus Montouro, Rodrigo Fellipe Rodrigues, João
Marquardt, Gabriel de Deus Vieira, Eduardo de Chaves, Ricardo Augusto Machado Silva, Laise Pedro Izidoro Gomes, Amanda Selvátici dos Santos
Novaes Costa Bergamaschi, Ricardo Góes Freitas, Cristina de Oliveira Rego Andrade Dias, Andressa Regina Galego, Fabio de Nazaré
Marcia Tatsch Cavagnollo, Gisele Espíndola, Kátia Oliveira, Gabriel Pina Paiva
Hospital da Restauração Governador Paulo Guerra.
Lin Faculdade de Medicina de Ribeirão Preto da
Recife PE, Brazil
Universidade Federal de Santa Catarina. Faculdade Pernambucana de Saúde. Recife PE, Universidade de São Paulo. Ribeirão Preto SP, Brazil
Florianópolis SC, Brazil Brazil
alex.bertolazzo@gmail.com
Instituto de Medicina Integral Prof. Fernando
laisbperini@hotmail.com Figueira. Recife PE, Brazil
Case Presentation: We describe a case of a male patient,
Íntegrus Cérebro e Comportamento Integrados.
Case Presentation: A 44yo right-handed female artisan, 48-years-old, whose started 20 days after the diagnosis of
Recife PE, Brazil
with university degree, was brought to emergency care due coronavirus disease 2019 (COVID 19) with progressive
to a history of approximately 1 year of progressive memory amandasabinopinho14@gmail.com fatigue, binocular diplopia, headache and slowing of
deficit (losing objects, messages and appointments), which thought. After a few weeks, he began to experience dif-
rapidly evolved in 6 months to prominent visuospatial Case Presentation: A 75-year-old woman, diabetic, less ficulties in walking, with ataxia and incoordination. The
impairment preventing her to drive her car, and dyspraxia than 4 years of schooling, no past or family history of symptoms has a dramatic evolution over three months
hindering her to paint or performing manual tasks and to dementia or psychiatric disorder, presented with sudden with onset of diffuse myoclonus, significant language and
carry on her work, significant global dysphasia, and uri- behavioral change after COVID-19 infection in April of movement impairment and finally evolved to akinetic mut-
nary incontinence, leading to severe functional limitation. 2021. Throughout the infection, the patient had fever, ism. Complementary investigation whit brain magnetic
In the month before admission, she no longer handled anosmia, with no need of Hospitalization. After 14 days, resonance imaging (MRI) showed restricted diffusion in
money or was able to cook. On neurological examination, exhibited infantilized behavior, dysarthria and did not the cortical and subcortical posterior regions and deep
she scored 5 out of 30 points on the MMSE. Insight and recognize her family members, mistaking her daugh- nuclei gray matter (left caudade nuclei). The electroen-
social adequacy were preserved. The patient was disori- ter for her own mother. The Addenbrooke’s Cognitive cephalogram (EEG) revealed intermittent generalized
ented in time and space, ideomotor apraxia was observed, Examination-Revised (ACE-R) and Neupsilin were admin- periodic discharges (GPD) and diffuse-symmetric slow-
as lack of verbal fluency with anomia, and inability to istered, revealing executive dysfunction, visuospatial ing. The cerebrospinal fluid (CSF) analysis and the 14-3-3
repeat complex sentences. Also, an ocular apraxia was impairment, deficits in working memory, language and research were negative. In view of the findings, we agree
noticed. Tendon reflexes were symmetric and no frontal sustained attention. Patient remains stable after one- with the Crutzfeld-Jacob disease hypothesis, supported
releasing signs were observed. Gait was normal. Magnetic year follow-up. Cerebral Magnetic Resonance Imaging by University of Edinburgh diagnostic criteria (2017) to
Resonance Imaging (MRI) of the brain showed diffuse (MR) with MR angiography, CSF analysis and laboratory probable sporadic Jakob-Creutzfeldt disease. Patient
brain reduction, with marked bilateral parietal atrophy. studies identified no abnormalities. Positron Emission complicated with bronchoaspiration pneumonia with
Genetic testing detected a pathogenic variant in the PSEN1 Tomography (PET/CT), after F18-FDG injection, showed needing of mechanical ventilation. Nowadays he persist
gene. She was treated with donepezil up to 10mg daily. Her discrete diffuse cortical hypometabolism, with bilateral in akinetic mutism with tracheostomy and gastrostomy.
deceased mother had a history of early-onset dementia frontal, posterior temporal, bilateral parietal, precuneus Discussion: Prion diseases are a heterogeneous group of
without a confirmed diagnosis. Discussion: The patient’s and cingulate gyrus predominance, not suggestive of vas- diseases caused by prions –abnormally shaped proteins,
clinical manifestation, with accelerated evolution and cular dementia. Discussion: Cognitive impairment is a and it can be acquired, hereditary or sporadic form. CJD
dramatic impairment of functionality, draws attention complication of COVID-19, that usually initiates during is a more common form of prion disease that affects
to a rapidly progressive dementia, whose main causes the diasease’s acute or subacute phase and improves humans and courses with rapidly progressive dementia
include prion diseases (Creutzfeldt-Jakob), infectious, a few months afterwards. Memory and attention defi- associated with behavioral changes, ataxia, extrapyrami-
inflammatory and neurodegenerative diseases, such as cits are the most common symptoms. In this case, the dal features and myoclonus. Its diagnosis is based on the
corticobasal degeneration, dementia with Lewy bodies impairment persists after a one-year course and, unlike clinical history and interpretation of the clinical features,
and atypical Alzheimer’s disease. The proposed diagnosis late-onset Alzheimer Disease (AD), exhibits disexecutive CSF, EEG and MRI, in addition to the genetic tests sup-
was posterior cortical atrophy (PCA), an uncommon type predominance. Identification of vascular and inflamma- ported by polymerase chain reaction (PCR-RT). There
of dementia that is often neglected or subdiagnosed. Final tory lesions in MR images has assisted in comprehend- are some reports of patients who developed CJD with
Comments: Posterior cortical atrophy (PCA) or progres- ing its pathophysiology, however they were not present a temporal relationship to COVID 19 that were initially
sive posterior cortical dysfunction syndrome (PPCD) is a in this patient’s exams. On the other hand, it needs to be interpreted as a coincidence because the rare incidence
rare but disabling dementia syndrome. This progressive clarified whether COVID-19 can influence the develop- of CJD and the COVID 19 pandemic that we have faced in
dementia initially presents with visual disturbances and ment of dementias, such as AD, accelerating its course, recent years. Although, there are descriptions of simulta-
is characterized by visuospatial and visual perceptual as it is suggested in this case. Cerebral PET and SPECT neous clinical presentations of COVID-19 and CJD, that
impairment, visual agnosia, with a later onset of cognitive findings, despite the variety of possibilities, always show, can indicate some correlated mechanism between them.
decline generally associated with typical Alzheimer’s dis- after COVID-19, hypometabolism with frontoparietal or Final Comments: There is still no definitive explanation
ease. Furthermore, PPCD can sometimes be mistakenly bilateral frontal predominance. The recent finding that for the relationship between COVID 19 and CJD, but it is
attributed to ophthalmological disorders. Its diagnosis SARS-CoV-2 infection induces hypometabolism in brain questioned whether the inflammatory cascade caused
remains challenging, with differential diagnoses includ- areas that are affected by neurodegenerative diseases will by the coronavirus infection could accelerate or even
ing rapidly progressive dementias. contribute to better comprehend the neuropsychologi- precipitate the neurodegeneration characteristic of CJD.
cal profiles in mixed cases of COVID-19 and AD. Final
Comments: It was presented probably new dementia
Neurologia Cognitiva e do Envelhecimento syndrome characterized by the sudden beginning, pro- Neurologia Cognitiva e do Envelhecimento
longed and fluctuating course after COVID-19.

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A “VARIANT” IN THE DIAGNOSIS OF FAHR’S SYNDROME: A CASE PRESENTATION THE IMPORTANCE OF BIOMARKER TO
SPORADIC CREUTZFELDT-JAKOB DISEASE IDENTIFY THE ETIOLOGY OF POSTERIOR
Ygor Roberto Ramos Gonçalves Soares, Emanuelly
Nogueira Gomes de Arruda, Maria Eduarda de CORTICAL ATROPHY SPECTRUM: A CASE
Marcela de Moraes Serpa, Lucas Leroux de
Ricchezza, Roberta Monteiro de Souza, Nathalia Oliveira Fernandes, Gloria Jeane de Oliveira PRESENTATION
Borges Carmo, Vinicius Pinheiro Soares, Danielle Fernandes
Tatiane Morgana da Silva, Marina Scop Medeiros,
Guzman, Abelardo de Queiroz Araújo, Luiz Felipe Cristiano Schaffer Aguzzoli, San Francisco, USA –
Faculdade de Ciências Médicas da Paraíba – Afya.
Rocha Vasconcellos Estados Unidos, Wyllians José Vendramini Borell,
João Pessoa PB, Brazil
Universidade Federal do Rio de Janeiro. Rio de Centro Universitário de João Pessoa – UNIPÊ. João Fernando Tonon Schneider, Rodrigo Twardowski
Janeiro RJ, Brazil Pessoa PB, Brazil Scherer, Eduarda Kotlinsky Weber, Marina Musse
Espaço Saúde – Clínica especializada – Cajazeiras Bernardes, Lucas Porcello Schilling
marcela.serpa@yahoo.com.br – PB – Brazil
Hospital São Lucas da PUCRS, Instituto do Cérebro
Case Presentation: M.P.J,?, 73 years old, presented with ygorroberto1996@gmail.com do RS – Porto Alegre – RS – Brazil
a history of progressive gait ataxia since Jan 2021, evolv- Global Brain Health Institute, Memory and Aging
ing with hypophonia, memory loss, executive dysfunc- Case Presentation: M. M. V, male, 76 years old, presented Center, University of California San Francisco UCSF
tion, agitation and dysphagia over a few months. In July symptoms consistent with parkinsonian syndrome and Hospital de Clínicas de Porto Alegre. Porto Alegre
2021 the patient became wheelchair bound and totally convulsive symptoms associated with fever for two years. RS, Brazil
dependent to perform daily activities. Exam: echolalia, Later he progressed with worsening of motor symptoms, Escola de Medicina da Pontifícia Universidade
did not follow commands, pupils were miotic and poorly presenting chorea and neuropsychiatric alterations such Católica do Rio Grande do Sul. Porto Alegre RS,
responsive to light. Marked ataxia, unable to stand. Action as irritability, excessive anxiety, and mental confusion. Brazil
tremor, myoclonus, mild paratonia and brisk reflexes in One year ago, after a seizure event, the patient presented Escola de Medicina da PUCRS, Instituto do Cérebro
the upper limbs. Brain MRI showed restricted diffusion decreased level of consciousness, was admitted to the do RS – Porto Alegre – RS – Brazil
in the left frontoparietal cortex, bilateral pulvinar and Hospital, and requested a CT scan. It revealed calci- marina.scop.medeiros@gmail.com
caudate nuclei, with the “double hockey stick sign”. 14-3-3 fications affecting the basal ganglia, dentate nuclei of
protein testing in CSF was negative. EEG was nonspe- the cerebellum, and white matter bilaterally, suggestive
Case Presentation: A 66-year-old female patient with a
cific. Investigation for other causes was unremarkable. A of Fahr’s disease. Laboratory tests were requested and
Law degree was evaluated at an outpatient clinic due to
diagnosis of probable sporadic Creutzfeldt-Jakob disease demonstrated hypocalcemia, vitamin B12 deficiency,
a series of cognitive deficits for the past 2 years: difficulty
(sCJD) was made. The patient died within 13 months of and low PTH levels. The patient started his therapy during
reading, spatial perception impairment and environmen-
symptoms onset. Discussion: sCJD is a fatal neurode- Hospitalization with supplementation of calcitriol, cal-
tal agnosia. In the past year, she would forget the pass-
generative disorder. Diagnosis is made through clinical cium carbonate, and cholecalciferol. After two months
word for her credit card, developed agraphia, apraxia of
presentation and at least one supporting test: periodic of Hospitalization, clinical treatment was supplemented
dressing, acalculia and impaired perception of objects.
sharp wave complexes in EEG; elevated 14-3-3 protein with quetiapine, phenytoin, levodopa, biperiden, and
The ophthalmological clinical evaluation was unremark-
in the CSF (not sensitive nor specific). In 2009 the cri- zolpidem, demonstrating significant improvement in neu-
able. She more recently has been repeating stories and
teria were updated to include typical MRI findings. The ropsychiatric and motor symptoms. Discussion: Fahr’s
had difficulty establishing a line of reasoning within them.
patient in question filled all clinical criteria for probable disease is a rare neurodegenerative disorder characterized
On physical examination, she had no pathological signs,
sCJD. Despite negative CSF protein 14-3-3 and EEG, by idiopathic, symmetrical, and bilateral calcification of
except for simultanagnosia. In the Addenbrooke test, she
she had one positive supporting test, an altered MRI, the basal ganglia. Symptoms include movement disorders,
was unable to perform the task of counting dots without
showing the “double hockey stick sign”, reported to be with parkinsonian syndrome being the most common, in
pointing at them. In the MoCA test, she achieved a score
up to 100% specific for the variant form of the disease. addition to seizures, severe headache, and neuropsychi-
of 9 and performed notably worse in visual tasks. Brain
Although this sign has been rarely seen in the sporadic atric changes. Treatment aims to control symptoms and
MRI showed parieto-occipital cortical atrophy mainly
form. Correlation with others MRI findings suggestive improve quality of life. Final Comments: The literature
on the left hemisphere. In the neuropsychological eval-
of sDCJ could be useful in the differentiation of vDCJ is still outdated when it comes to how to manage these
uation, difficulties in verbal and semantic fluency and in
such as: pulvinar involvement with lower signal intensity patients, and studies are needed to control the symptoms
visuomotor coordination were observed, as well as exec-
than putamen and caudate best seen in DWI compared and improve the patient’s quality of life since it is an irre-
utive dysfunction. Brain SPECT showed hypoperfusion
to FLAIR and prominent cortical diffusion restriction versible condition and there is no definitive treatment
in frontotemporal and parietal regions of the left cerebral
[5]. Final Comments: This case illustrates how a patient according to the guidelines.
hemisphere, extending to the hippocampus and poste-
with probable sDCJ may present as a mimic of a much rior cingulate, with a nonspecific scintigraphic appear-
rarer and epidemiologically concerning form of the dis- ance. Finally, the PET-CT with Florbetaben-F18 indicated
ease, variant CJD (vCJD), with an MRI sign reported to be Neurologia Cognitiva e do Envelhecimento
accumulation of b-amyloid plaques predominantly in the
highly specific to it. Thus, clinicians should be wary when prefrontal, orbitofrontal, parietal, temporal, cingulate and
encountering “classical” clinical or imaging findings, and occipital regions. Discussion: In 1988, Frank Benson and
remember to consider the full picture in context. In this colleagues described five unusual patients with progres-
case, the patient did not fill the criteria for vCJD besides sive visual difficulties that they termed posterior cortical
the MRI finding – she did not live in a risk area, did not atrophy (PCA). Subsequent autopsies of cases of posterior
have predominant psychiatric symptoms and was not in cortical atrophy showed a predominance of Alzheimer’s
the age group most commonly affected. [1] neuropathology, but other diagnoses such as prion disease
and Lewy body dementia were also reported. Currently, we
can use biomarkers that help diagnose the etiology of PCA.
Neurologia Cognitiva e do Envelhecimento
This case, the patient had clinical criteria for PCA, that
could be related to Alzheimer’s disease through PET-CT
performed with Florbetaben-F18. Final Comments: The
use of functional neuroimaging as a biomarker, especially
for amyloid deposition, is a helpful tool for identifying the
etiology within the spectrum of PCA. Consequently, new
clinical trails will be able to investigate atypical pheno-
types within the same etiology. Getting to a more specific
diagnosis using biological markers is the first step towards
finding effective targeted therapies for PCA patients.

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XXX Congresso Brasileiro de Neurologia

TL 1105641 TL 1105726 TL 1105754

ANAPLASTIC ASTROCYTOMA CAUSING WHITE MATTER HYPERINTENSITIES IN A CASE PRESENTATION LATE PRESENTATION
RAPIDLY PROGRESSIVE DEMENTIA – A CASE SPORADIC CASE OF SEMANTIC VARIANT CREUTZFELDT-JACOB DISEASE
PRESENTATION PRIMARY PROGRESSIVE APHASIA: A CLUE
Marcella Canato Toloi, Andreas Batista Schelp,
TO GRN GENOTYPE Déborah Inayara Mendes Tenório de Albuquerque,
Lorena Dias Araújo, Indianara Keila Pastorio, Rafael
de Almeida, Paulo Henrique Martinelli Oliveira, Anna Beatriz Perdigão Cordeiro, Caio Vinicius Kássia Braga Canzian, Isabela de Almeida Stella,
Lucas Silva Dias, Sayuri Aparecida Hirayama, Barroso de Lima, Pedro Barbosa Oliveira, Flavio Bruno Rezende Trindade Borges, Fernanda Maria
Francine de Paula Roberto Domingos, Raderi Luiz Moura Rezende Filho, Paulo Henrique Ferreira Gonçalves de Sousa Moura, Amanda Freitas Alves,
Cardoso dos Santos, Raquel Mattos Filgueiras Bertolucci Andre Lopes Fernandez, Roberta Arb Saba

HMJCF. São José dos Campos SP, Brazil Universidade Federal de São Paulo. São Paulo SP, Hospital do Servidor Público Estadual de São Paulo.
Brazil São Paulo SP, Brazil
lorena.d.a25@hotmail.com
annabeatrizpc@gmail.com marcella.toloi@gmail.com
Case Presentation: Patient M.B.S., 50 years old, male,
hypertensive. Two months ago he started with memory Case Presentation: A previously healthy 66-year-old Case Presentation: An 88-year-old female patient, pre-
and executive function alterations, with an impact on his woman presented with a four-year history of progressive viously functional, presented with gait disturbance, pro-
functionality and his work. In addition, with severe left difficulty in communication. The patient’s chief com- gressive involuntary movements, dysphagia, dysarthria
hemicranial headache, stabbing type, lasting 2 minutes, plaint was a “very bad voice”. She initially had occasional and altered cognitive status with an evolution of only 3
intermittent, without photophobia, phonophobia, nausea problems in understanding single words and often asked months. In the evaluation, the patient presented with eye
or vomiting. After 2 months, he presented with seizure of for sentences to be repeated. Gradual worsening of the opening to verbal stimuli, non-contacting, non-respon-
unknown onset with evolution to generalized tonic-clonic, symptoms led to severe impairment in comprehension. sive to commands, in akinetic mutism, with spasticity and
without associated fever. No weight loss. Physical exam- She eventually stopped reading and praying. There were myoclonus in the 4 limbs, in addition to signs of frontal
ination revealed mixed aphasia of motor predominance, no memory, orientation or behavioral problems. There release. In view of the rapid cognitive decline and loss
with phonemic paraphasia and agraphia. No change in was no family history of neuropsychiatric disorders. On of important functionality associated with myoclonus,
strength, sensitivity or coordination. Cranial resonance the neurological examination, she had no signs of motor investigation for rapidly progressive dementias (RPD)
with T2/FLAIR hypersignal affecting the caudate nucleus, neuron involvement, nor parkinsonism. She exhibited was initiated. Cranial magnetic resonance imaging (MRI)
putamen, globus pallidus, hippocampus and left insula, aphasia with severe loss of fluency, very poor object showed areas of restriction to diffusion in the cortical
as well as cortical surface of the frontal and homolateral naming and single-word comprehension combined with region, bilateral, predominantly on the left, in addition
parietal regions, with mass effect, without DWI restriction surface dyslexia. The patient scored 14 points at the Mini- to an electroencephalogram with a periodic sharp-wave
and without gadolinium enhancement. Ascopy with cho- Mental State Examination (MMSE) (16 years of educa- complexes, filling in, after ruling out other causes of PRD,
line peak and n-acetyl-aspartate reduction. Biopsy showed tion), 10/20 points at the Boston Naming Test and 6 and diagnostic criteria for Creutzfeldt-Jakob disease (CJD).
grade III anaplastic astrocytoma. Descompressive cra- 3 points at Verbal Fluency Test for animals and fruits, Discussion: CJD belongs to a group of degenerative
niectomy and radiotherapy were performed. Discussion: respectively. Brain magnetic resonance imaging (MRI) diseases caused by infectious protein particles (prions),
Analyses of the global disease burden have shown that revealed asymmetric left temporal atrophy, prominent left which are pathogenic forms caused by the conversion
43.8 million people lived with dementia. Assuming that parietal atrophy and white matter hyperintensities. The of normal prion protein into its abnormal isoform. This
a substantial percentage of these patients presented with clinical diagnosis was semantic variant primary progres- process is replicated exponentially, which explains the
rapidly progressive dementias (RPD), it is evident the sive aphasia (svPPA). A next generation sequencing panel rapid development of the disease. Clinical diagnosis is
importance to consider superimposed reversible condi- for dementias identified a pathogenic variant in GRN gene based on a set of symptoms and complementary exams,
tions in the differential diagnosis of RPD.Besides para- (c.1252C>T – p.Arg418*). Discussion: The hallmark of the including CSF, EEG and MRI. The classic phenotype is
neoplastic immunemediated encephalopathies, primary Primary Progressive Aphasias (PPA) is the insidious dete- rapidly progressive dementia with behavioral changes,
CNS neoplasias are also potential causes of RPD. These rioration of language, with relative preservation of other ataxia (usually in gait), extrapyramidal signs and myoc-
neoplasias can affect cortical and subcortical structures cognitive functions. This group of disorders is clinically lonus. The EEG demonstrates periodic complexes of
through space occupation, infiltration and destruction of heterogeneous, and comprises at least three subtypes: sharp waves, whereas the MRI may present with diffu-
brain tissue, secondary edema or disturbance of CSF cir- nonfluent, semantic and logopenic. SvPPA is characterized sion restriction in the cortex or in the basal ganglia. CSF
culation resulting in intracranial hypertension. However, by anomia associated with loss of semantic knowledge, is usually normal and the usefulness of biomarkers such
treatment options that prolong survival and might improve and severe impairment in single-word comprehension. as 14-3-3 is still controversial due to the variability of sen-
cognitive performance are becoming available. Final Familial cases of svPPA have been linked to pathogenic sitivity and specificity. The disease is inevitably fatal, with
Comments: In RPD, the chances of treatment success variants in GRN gene, which encodes the protein pro- an average survival of 6 months, as there is no specific
are better the earlier the diagnosis is made. Mainly when granulin. However, sporadic cases of svPPA harboring treatment to date. Final Comments: CDJ affects many
potentially treatable pathologies are identified. pathogenic variants in this gene are extremely rare. Our areas of the brain, so it can present itself as a multiform
patient had a clinical phenotype consistent with svPPA, and it can also mimic, particularly early on, many other
and no family history. White matter hyperintensities, in neurological and psychiatric conditions, which can delay
Neurologia Cognitiva e do Envelhecimento combination with asymmetric temporoparietal atrophy diagnosis. However, despite having no specific treatment,
have raised the suspicion for a GRN genotype, which was it is highly stigmatizing, and its suspicion and diagnosis
subsequently confirmed by the molecular study. Final are important for a better management of symptoms.
Comments: GRN genotype is rare among sporadic svPPA
cases, but white matter hyperintensities and asymmetric
Neurologia Cognitiva e do Envelhecimento
temporoparietal atrophy should reduce the threshold for
molecular investigation.

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XXX Congresso Brasileiro de Neurologia

TL 1105793 TL 1105816 TL 1106058

RAPIDLY PROGRESSIVE DEMENTIA IN A LATE PRESENTATION OF THE CEREBELLAR COGNITIVE AFFECTIVE


A PATIENT WITH NON-HEMORRHAGIC FRONTOTEMPORAL DEMENTIA (FTD) SYNDROME AS MANIFESTATION OF
CEREBRAL AMYLOID ANGIOPATHY: A CASE ASSOCIATED WITH PSYCHIATRIC ISCHEMIC STROKE: CASE PRESENTATION
PRESENTATION SYMPTOMS: A CASE PRESENTATION
Thiago da Cruz Marques, Vanessa Moraes Rosette,
Bárbara Maini de Carvalho, Matheus Alves da Silva, Bruna Guimarães Dutra, Wendell Silveira de Souza, Clara Kimie Miyahira, Natalia Figueiredo Miranda,
Eduardo Mesquita de Souza, Rafael Bragança João Pedro Moreira Guilherme, Neyde Alegre de Beatriz Medeiros Correa
Rodrigues Matias, Hennan Teixeira Salzedas, Souza Cavalcante, Talisia Nascimento Vianez
Santa Casa de Santos. Santos SP. Brazil
Chistiane Monteiro de Siqueira Campos, Carmen
Lucia Penteado Lancellotti, Alex Machado Baeta Universidade Federal do Amazonas. Manaus AM,
cmarques.thiago@gmail.com
Brazil
A Beneficencia Portuguesa de São Paulo, São Paulo Universidade do Estado do Amazonas. Manaus AM,
Case Presentation: The cerebellum is the control center
SP, Brazil Brazil
for voluntary movements and coordination contributing
Santa Casa de São Paulo, São Paulo SP, Brazil
brunagdutraa@gmail.com to planning, execution and modulation of movements.
barbaramaini@outlook.com.br However, research reveals a wider role of the cerebel-
Case Presentation: A 73-years-old patient, male, lum in cognitive, emotional, and behavioral functions
Case Presentation: A 84 year-old male patient with a from Manaus, Amazonas, arrived to our service with because of widespread cerebellar connections to the cor-
history of follicular low grade non-hodgkin’s lymphoma a long-standing sleep disorder, and about 2 years ago tex and associated areas. Clinically, Cerebellar Cognitive
treated, evolving for two months with loss of self-care, the clinical condition was associated with anterograde Affective Syndrome (CACS), also known as Schmahmann’s
significant difficulty in walking, total dependence for amnesia, in addition to episodes of disorientation and Syndrome, is characterized by language deficits, visual
daily life activities associated with cognitive impairment, mental confusion, according to his wife. He progressively and spatial executive functions, and personality changes
especially recent memory. He denied urinary inconti- evolved with behavioral changes, characterized by agita- seen in patients with focal cerebellar lesions. Some studies
nence. Infectious, paraneoplastic, immune-mediated tion, akathisia, constant speech of persecutory content with maps of lesions with clinical diagnosis in patients
processes were ruled out. MRI of the brain showed diffuse and worsening of sleep disorders, followed by episodes of with CACS and normal motor performance suggest the
encephalic volumetric reduction, without lobar predom- visual hallucination. He denies a history of neurological posterior cerebellar injury involving the lobes VII and
inance. Hyperintense lesions on T2/Flair in the white or psychiatric diseases in the family, and at the time of the VIII, being a risk factor for cognitive deficits. We report
matter of the cerebral hemispheres, without expansive first consult was using Risperidone and Rivastigmine. On the case of a male patient, 82 years old, admitted to a
character and a tendency to confluence in a periventricular the neurological examination was slightly disoriented in semi-intensive therapy presenting with a delta time greater
situation, suggestive of moderate degree microangiopathy, time, space, person and situation. CGS 14, restless, with than 5 hours with speech impaired and understanding,
as well as enlarged perivascular spaces, mainly affecting constant talk of self-destructive content, echolalia and inappropriate laughter and presence of logorrhea in the
the basal ganglia, this finding being a marker of small akathisia. Osteotendinous reflexes abolished in upper eyes of his relatives (wife and son). Patient referred for
vessel disease. For diagnostic clarification a brain biopsy limbs, Patellar present, Achilles absent and Glabellar tap Hospitalization to the care of neurology as a diagnostic
was performed with characteristic findings of amyloid was fatiguable. Cognitive tests were distorted, with MMSE hypothesis of Cerebral Vascular Accident. Neurological
angiopathy with an inflammatory pattern. Treatment was score of 15 out of 30 and clock test score of 1. MRI showed physical exams show a conscious and cooperative elderly
performed with methylprednisolone 1g daily for 3 days moderate atrophy, without hippocampal predominance man with cautious gait, reduced grammatical speech, lack
with significant improvement in the patient’s quality of life and without signal changes or ventricular dilatation, lead- of fluency and inadequate understanding and laughter;
Discussion: Cerebral amyloid angiopathy (CAA) is char- ing to the probable diagnosis of Frontotemporal dementia tests for coordination (nose finger test, heel test and
acterized by acellular thickening of the walls of small and (FTD). After discussion by the neurologic team, Periciazine dysdiadochokinesia) were normal, sensory and motor
medium arteries due to accumulation of amyloid. CAA and Olanzapine were introduced and Risperidone was systems within normality, cranial nerves without alter-
is often asymptomatic but usually manifests as demen- suspended. In the following months, patient returned with ations. Mental functions reveal impaired attention and
tia and vascular syndromes, and there is an important support of the clinical picture, in addition to the appear- concentration, recent memory, and altered perception.
association between CAA and spontaneous intracerebral ance of essential tremor in upper limbs, maintenance of MRI Brain showed a peripheral intraparenchymal focus
hemorrhage. In this sense, we present a case of non-hem- sleep disorder and he became dependent on family mem- in the right cerebellar hemisphere presenting restriction
orrhagic CAA, with an inflammatory pattern, presenting bers for basic-life activities around 6 months since the first to the diffusion of water in the Ecoplanar sequence, with
as rapidly progressive dementia. Histologically analyzed consult, and Citalopram was added to the prescription for a weak hypersignal in the T2 / FLAIR sequence. After
lesions can be of amyloidoma, CAA with primary angiitis symptomatic control. The follow-up continued at our ser- extensive diagnostic and exclusionary investigation, the
of the central nervous system (SPAC) and, as in this case, vice to assess the progression of the cognitive-behavioral diagnosis of Cerebellar Vascular Accident with cogni-
they consisted of isolated CAA. Its pathogenesis is not very status, and he died at home in 2022 after approximately tive manifestation and without motor impairment was
clear, but amyloid deposition is accompanied by inflam- 3 years of follow-up. Discussion: The case reveals a late instituted. The Cerebellar Cognitive Affective Syndrome
mation or edema, in contrast to the usual presentation of presentation of FTD, since the most affected age group is conceptualized by Schmahmann and Sherman, seen in
patients with CAA, involving acute intracerebral hemor- under 65 years, and helps to emphasize that the presence a patient with cerebellar damage, was clinically prom-
rhage. Final Comments: The literature emphasizes the of this condition in older ages should not be ignored. The inent in patients with cerebellar posterior lobe disease
diagnosis of CAA and its association with hemorrhages presence of hallucinations and sleep disorders are not as and worms, and there may be several causes of cerebellar
and dementia, however rare cases of non-hemorrhagic common as behavioral and language changes, fact that damage, such as viral infections and drugs, but no specific
brain lesions are associated with CAA, as exposed above. may have directed examiners to hypothesis such as Lewy- etiology can be detected in our patient, which evidenced
Little information is available regarding their treatment. body dementia in this case. Final Comments: Healthcare cause of ischemic vascular etiology of cerebellar damage.
Non-hemorrhagic AAC has shown a satisfactory response providers should order routine tests and deep examination
to corticosteroid treatment in patients with evidence of to rule out other conditions that cause similar symptoms
inflammation, as we also evidenced in our case. Thus, and advised on the reserved prognosis of this disease. Neurologia Cognitiva e do Envelhecimento
although rare, it should be considered in the differential
diagnosis of lesions of the central nervous system.
Neurologia Cognitiva e do Envelhecimento

Neurologia Cognitiva e do Envelhecimento

177
XXX Congresso Brasileiro de Neurologia

TL 1106268 TL 1106291 TL 1106315

LEUKOENCEPHALOPATHY WITH ADULT-ONSET LEUKOENCEPHALOPATHY CREUTZFELDT-JAKOB DISEASE


EVANESCENT WHITE MATTER PRESENTING WITH AXONAL SPHEROIDS AND PIGMENTED HEIDENHAIN SUBTYPE: CASE
WITH SUBCORTICAL DEMENTIA, A CASE GLIA PRESENTATION
PRESENTATION
Roberta Lessa Ribeiro, Iury Hélder Santos Dantas, Vitor Picanço Lima Gomes, Fabio Pacheco Martins,
Matheus Gonçalves Maia, Diogo Haddad Santos, Morgana Feitosa de Queiroga, Ian Silva Ribeiro, Daniela Ragnini, Ricardo Lorenzato Bortoluz,
Davi Bravo Huguinim Légora, Yngrid Dieguez Ferreira Norberto Anízio Ferreira Frota Guilherme Michelon, Giovanni Gosch Berton, Maria
Carolina Cardoso da Silva, Daniel Lima Varela, Alan
Santa Casa de Misericórdia de São Paulo, São Paulo Hospital Geral de Fortaleza. Fortaleza CE, Brazil Christimann Fröhlich, Bruna Constantino Rech
SP, Brazil
robertalessaribeiro@hotmail.com Universidade Federal Fronteira Sul / Hospital de
matheusgmaia1@gmail.com Clínicas de Passo Fundo – Passo Fundo – RS –
Case Presentation: A 45-year-old man consulted his Brazil
Case Presentation: A 48-year aged woman was attended doctor with a year’s onset of memory problems and Serviço Neurologia e Neurocirurgia / Hospital de
at the SCMSP Cognitive and Behavioral Neurology outpa- behavioral changes. He worked as a delivery man for a Clínicas de Passo Fundo – Passo Fundo – RS –
tient clinic in May 2020. She was graduated on high school company and began to mix up his deliveries and get lost Brazil
and a had a job as a supermarket cashier. She failed 1 year in previously known streets. His wife also noticed he
at high school and was fired on one occasion when she was forgetting to pay the bills, a task he had always done fpachecosm@gmail.com
argued with a customer. On 2017 she had an isolated epi- without problem, and that he was using the money for
sode of tonic-clonic seizure, evolving with a progressive other purposes. Because these problems were getting Case Presentation: J.T.M., female, 67, alcoholic. Social
picture of incoordination of gait and cognitive processing worse and affecting the life of the patient and his family, isolation begins and evolves with a bilateral visual defi-
speed impairment. She also had chronic visual blurring the wife chose to take him for a medical examination. cit, abdominal pain, and headache. She presented with
and early menopause at age 45. On neurological examina- He underwent cognitive testing and his results were dental septicemia. At examination: miotic pupils, pho-
tion, she was inattentive, disoriented in time and space, indicative of mild cognitive impairment, especially in toreactive pupils, upper limbs with spasticity, parato-
with optic atrophy and cerebellar ataxia. She presented the memory and visual-spatial areas. Screening tests nia, and myoclonus. She didn’t interact and didn’t obey
significant impairment on cognitive scales MMSE: 18/30 were ordered. His blood tests were normal and the CSF commands. In addition, patellar hyperreflexia and bilat-
and functionality scales, KATZ: 6/6 and LAWTON: 18/27. showed only a slight increase in total protein. The MRI eral flexion on plantar reflex. Evolution with gait ataxia,
She underwent neuropsychological assessment, which revealed atrophy and white matter lesions in the frontal changes in motor coordination, ideomotor and speech
showed a 50 QI, severe orientation and recent memory lobe of the cerebrum and around the lateral ventricles, apraxia, and behavioral changes were present. During
impairment, significant emotional lability and executive making those appear enlarged; a thinning of the anterior Hospitalization, she evolved with bilateral extension
disfunction, little initiative and flexibility, and a lot of corpus callosum; and small calcifications in the white at plantar reflex. 14.3.3 protein was dosed due to the
confabulation. Neuroimaging revealed cystic degener- matter around the semi-oval centers, radiated crowns, hypothesis of spongiform encephalopathy. Its result
ation of the white matter of both cerebral hemispheres and knee of the corpus callosum. These findings sug- showed a value of 33,712 (0-19,999). There has been an
with extension to the U-fibers and diffusion-symmetric gested Adult-Onset Leukoencephalopathy with Axonal intense investigation of differential diagnoses. The patient
curvilinear periventricular restriction. She underwent Spheroids and Pigmented Glia (ALSP) and a genetic test began to have anticipated complications and progressed
genetic testing, which revealed homozygosity in the was done for further research. A Variant of Uncertain to support. Discussion: Regarding CJD, about 80 to 85%
EIF2B5 gene, with a p.Arg113His mutation. Discussion: Significance, c.2522A>G (p.Tyr841Cys), was identified in of cases are caused by the sporadic form, with prevalence
LEWB is an autosomal recessive, chronic and progres- the colony-stimulating factor-1 receptor (CSF1R) gene, in the elderly population, between the 5th and 6th decade
sive leukodystrophy caused by mutation in any of the 5 which is associated with autosomal dominant hereditary of life, with no sex differentiation and absence of distinct
genes encoding eIF2B. It typically presents with ataxia, diffuse leukoencephalopathy with spheroids (HDLS) and transmission patterns. In Heidenhain subtype CJD, the
spasticity and optic atrophy that typically follow an initial pigmentary orthochromatic leukodystrophy (POLD). Once Parieto-Occipital cortex is affected, affecting the primary
stressor event. On adults, a case series with 16 patients both disorders were linked to CSF1R gene variants, they visual area and causing image changes such as cortical
(Labauge et. Al, 2009) showed cognitive decline in 8 sub- became known as ALSP. Treatment was initiated with a hyperintensities on T2/FLAIR MRI and basal ganglia
jects, in addition to spasticity and/or cerebellar syndrome, cholinesterase inhibitor and antipsychotic drugs, with a (striatum and thalamus). The electroencephalography
seizures, dementia, psychiatric symptoms, and ovarian partial response from the patient, mostly in behavioral (EEG) shows periodic, three-phase acute waves, predom-
failure (Ovarioleukodystrophy). The correlation between symptoms. Non-pharmacological approaches were also inantly in the posterior areas. 14.3.3 protein can also be
LSBE and its neuropsychiatric presentations has already established. The family was oriented about the diagnosis, dosed from the cerebrospinal fluid. Prognosis is one year
been studied by authors such as Van der Knaap, 1997, in and the disease’s progressive nature, and they were sent to of life after the onset of symptoms. The classic presenta-
a report of 9 children with diffuse cystic degeneration of genetic counseling. ALSP is a rare, progressive neurolog- tion of this variant involves cortical blindness capable of
BS, relatively preserving the cerebral cortex; Prass, 2001, ical disease that causes leukodystrophy, forming lesions generating various types of visual changes, visual field
in a report of 1 patient with presenile dementia syndrome; in certain brain areas due to disease-causing variants in restriction, hallucinations, and visuospatial recognition.
Van der Knaap, 2004, in a report of 1 case of a 25-year-old the CSF1R gene. It is estimated to occur in 10 to 25% of Neuropsychiatric symptoms culminated in agitation and
woman who presented paranoid behavior, evolving with adult-onset leukodystrophies. Lesions of the white matter aggression. The symptoms progressed in a short time (60
a decline in cognitive functions. Final Comments: Our lead to major changes in personality, cognition, and mus- days) until presenting akinetic mutism, typical of the dis-
patient presented with a subcortical dementia profile cle function. There are currently no approved treatments ease. Measurement of 14.3.3 protein in the CSF, among
with spatial disorientation, slow cognitive processing, for ALSP and current treatment options do not reverse the presence of at least two supportive clinical criteria,
attentional impairment, dysnomia, visuoconstructive brain damage but instead are meant to manage symptoms. and the association with progressive dementia were cru-
disorder with preserved recognition and cortical function cial for defining a probable diagnostic hypothesis. Final
Comments: The signs and symptoms described in the
Neurologia Cognitiva e do Envelhecimento literature were compatible with those presented in this
Neurologia Cognitiva e do Envelhecimento case, representing the Heidenhain subtype of sCJD. There
is no specific treatment. Currently, the basis of the thera-
peutic approach is on supportive measures.

Neurologia Cognitiva e do Envelhecimento

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XXX Congresso Brasileiro de Neurologia

TL 1106502 TL 1106557 TL 1105116

AMYLOID-β–RELATED ANGIITIS: A CASE CREUTZFELDT-JAKOB DISEASE AND MULTIPLE SCLEROSIS IN A 13-YEAR-OLD


PRESENTATION THE CHALLENGES OF THE DIAGNOSTIC GIRL
INVESTIGATION: A CASE PRESENTATION
Matheus Costa Bessa, Barbara Matos de Almeida Aline Haag, Chelin Auswaldt Steclan, Rafaela
Queiroz, Karoline Ferreira Mororó Menezes, Joanisson Rubens Gomes Diniz, Kaliny Oliveira Hammerschimidt, Stefan Moreira, Diogo Pasquali
Matheus da Costa Guedes, Alissa Elen Formiga Peixoto, Heitor Caetano Santos, Maria Weryca Souza Nones, Oscar Nelson Reimman Junior, Filipe
Moura, Glauber de Menezes Ferreira, Pamella Belo Silva, Gabriel Carvalho Oliveira Cruz, Armando Laurindo Cabral, Gabriel Hoher Peres, Michael
Christine de Sousa Munhoz, Robson de Sales Carlos Diógenes Júnior, Paulo Santiago Morais Brito, Ricardo Lang, Leonard Rocha Fonseca de Brito
Ferreira, Norberto Anizio Ferreira Frota, Fernanda Rodrigo Alencar e Silva
Martins Maia Carvalho Universidade do Contestado – NUPESC – Mafra –
Hospital Universitário Onofre Lopes – Natal – RN SC – Brazil
Hospital Geral de Fortaleza. Fortaleza CE, Brazil – Brazil Universidade do Contestado – NUPESC e
NEUROMAX – Mafra – SC – Brazil
mcostabessa@gmail.com rubensdiniz08@gmail.com NEUROMAX; São Vicente de Paulo/Mafra-SC –
Mafra – SC – Brazil
Case Presentation: 76 years old female, previously diag- Case Presentation: A previously healthy 68 yo male NEUROMAX; Universidade do Contestado; São
nosed with systemic arterial hypertension and diabetes initiated symptoms of imbalance and gait disturbance, Vicente de Paulo/Mafra-SC – Mafra – SC – Brazil
mellitus 2, presented episodic falls, chronically pro- followed by anterograde amnesia and impaired func- NEUROMAX e Hospital Santo Antônio/Blumenau-SC
gressing to cognitive impairment, due mainly to apraxia tionality. In the following weeks, he progressed with – Blumenau – SC – Brazil
and loss of motor functionality, without rigidity or other decreased verbal fluency and social interaction, wors- NEUROMAX; Universidade do Contestado –
parkinsonian symptoms. After 2 months of worsening of ening of gait and became completely dependent to per- NUPESC; e Hospital São Vicente de Paulo/Mafra-SC
symptoms, patient had new seizure, with tonic posture form daily activities. A month after the initial symptoms – Mafra – SC – Brazil
in four limbs, leading to seek medical care. On admis- he was Hospitalized with akinetic mutism, myoclonus, NEUROMAX e Hospital Santo Antônio/Blumenau-SC
sion,cognitive assessment showed ideomotor apraxia, severe dysphagia, and dystonic upper limb posture. The – Blumenau e Jaraguá do Sul – SC – Brazil
but, due to reduced level of consciouness, the exam was initial investigation with electroencephalogram (EEG)
jeopardized. the patient underwent a non-contrast CT and brain magnetic resonance imaging (MRI) showed chelinsteclan@gmail.com
scan, which demonstrated subcortical diffuse hipoden- no characteristic signs of the disease, despite the study of
sity. For further investigation, brain MRI was performed, cerebrospinal fluid (CSF) showing the presence of 14.3.3 Case Presentation: Female, 13 years old. Asthma was
revealing T2 and FLAIR hyperintensity of subcortical white protein. The third performed EEG showed signs of diffuse mentioned as the only pre-existing disease. Reported
matter and SWI several microbleeds in right parietal lobe. encephalopathy and periodic generalized discharges that that three months ago had started with paresthesia and
Besides, serology, biochemical exams and cerebrospinal infer a high propensity to epileptic seizures, suggesting the loss of sensitivity in both lower limbs, but in the last
fluid did not present any alterations. Diagnosis of amyloid possibility of prion disease. A posterior brain MRI showed month he remained only in the LLL, which culminated
angeitis was proposed.High dose IV methylprednisolone cortical restricted diffusion and FLAIR hyperintensity on in the difficulty to extend and flexing the thigh, leg and
was started, with significant imprevement of cognitive occipital lobes, head of caudate nucleus and putamen left foot, this being the only reason for seeking the health
symptoms, however, after 4 days, the patient had a sei- and DWI/ADC abnormalities in temporo-parietal cortex. service. Presented negative Brudzinski and Kering signs,
zure, wth worsening of awareness. After two weeks of Signal abnormalities were subtle on the previous MRIs isophotoreactive pupils and no cognitive alterations;
Hospitalization, patient was discharged with mild to mod- but became more pronounced with the progress of the grade II strength in the LLL, weak contractions, produc-
erate improvement of cognitive function, but locomotion disease as well as the cerebral atrophy. Given the patient’s ing movement with elimination of gravity; and degree V
was disabled. 60-day re-evaluation at neurological clinic clinical condition, associated with typical EEG and MRI of strength in the RLL, in addition with presence of vari-
showed betterment of walking and cognitive impairment. and the protein 14.3.3 on CSF, the patient had diagnostic cose veins in the medial region of the thigh. Underwent
Discussion: Cerebral amyloid angeitis, a rare condition, criteria for probable sporadic Creutzfeldt-Jakob Disease complete blood count, EchoDoppler of the LLL and
given the evidence of 50 cases in the literature, linked to (sCJD). He was discharged after four months in Hospital, carotid arteries, echocardiogram and cerebrospinal fluid
aging and resulting from the pathogenic mechanism of taking anticonvulsants to control myoclonus. Discussion: examination, speech-language analysis and pediatric
autoimmunity to ?-amyloid, insoluble nature, which are Prion diseases are rare and fatal degenerative diseases evaluation – normal results. However, initial CT showed
deposited in cerebral vessels, causing a local inflamma- of the central nervous system, with an estimated annual small hypodense area in left frontal lobe with an aspect
tion with eventual fibrinoid necrosis and, later, cerebral incidence of 1 to 1.5 cases per million inhabitants. They unspecific to method. Brain MRI showed focal areas with
infarction, being demonstrated on MRI a hyperintensity can occur sporadically, genetically or acquired. SCJD is signal alteration in white matter bilaterally, compromis-
and cortical/subcortical microhemorrhages. Symptoms the most common form of them and its median survival ing periventricular, juxtacortical and optical radiation
include altered mental status, focal neurological deficits is about six months. Although clinically variable, neuro- topography bilaterally, without contrast enhancement.
and seizures which, in a complete analysis of the clinical psychiatric decline, cerebellar manifestations, myoclonus, Extending the scan, MRI of thoracic spine showed char-
case, may lead to a diagnosis of amyloid angeitis. Final and extrapyramidal signs are present in most cases. MRI acteristic lesion of recent inflammatory activity, leading
Comments: Amyloid angiitis is a difficult pathology to has a diagnostic accuracy of 97%. EEG sensitivity increases to sclerosing demyelinating disease (MS) hypothesis,
diagnose and staging due to its clinical presentation, in more advanced cases, requiring repetition throughout which was confirmed by focal lesion identification with
which matches other etiologies, usually being a differ- the clinical course. The sensitivity and specificity of the enhancement through the contrast at the T5 vertebral
ential diagnosis. Finally, even in the face of published 14.3.3 protein is variable. CSF prion detection is the gold body. Pulse therapy with synthetic steroids was already
studies, it is essential to conduct further research on this standard test for lifelong diagnosis. Treatment consists of started and this follow-up was necessary on an ongoing
subject, since there is little current research, besides the clinical support. Final Comments: This report describes basis. Closed diagnosis of MS-with recurrent outbreak,
lack of probable diagnostic measures. a rare disease, emphasizing the importance of persisting re-presenting at two-month intervals. Etiology remained
in the complementary investigation, even in the face of unknown. According to DATASUS, state of SC is in 2nd
uncharacteristic initial tests. place in relation to cases of disease in the southern region,
Neurologia Cognitiva e do Envelhecimento having that, information pertinent to this situation in
young patients, exposing the diagnostic protocol, can
Neurologia Cognitiva e do Envelhecimento help more effective therapeutic outcomes. Therefore,
rapid diagnostic, management and treatment of young
patients with MS is increased through detailed anam-
nesis and neuroimaging. As a is chronic inflammatory
disease of an autoimmune nature, adequate treatment
increases and improves the patient’s quality/expectation
of life. Knowledge of the epidemiological profile associ-
ated with a detailed neurological anamnesis supported
by neuroimaging exams can reduce the interval between
diagnostic confirmation and treatment, defining a more
accurate and decisive prognosis.

Neurologia Infantil

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XXX Congresso Brasileiro de Neurologia

TL 1105441 TL 1105575 TL 1105689

INFANTILE NEUROAXONAL DYSTROPHY: REPORT OF A RARE CASE OF LEUKOENCEPHALOPATHY WITH VANISHING


REPORT OF TWO SISTERS PONTOCEREBELLAR HYPOPLASIA TYPE WHITE MATTER: A CASE PRESENTATION
6 IN A CHILD IN THE AMAZON REGION OF
Raissa Kaori Ikeda, Rebeca Aiko Ikeda, Gabriel Beatriz Santana Borges, Juliana de Castro Naves
Queiroz de Abreu, Júlia Evaristo Figueiredo NORTHERN BRAZIL Peixoto, Luiz Alves Ferreira Filho, Thais Bonfim
Malheiros, Marcela Tiezzi Saporito, Vanessa Maria Leandro Araújo Costa, Paula Carolina Brabo Monte, Teixeira, Maria das Graças Nunes Brasil, Delson
de Sousa, Juliana da Silva Soares, Leticia Alves Vívian Garcia da Cunha, Priscila Altoé Echer, Rafaela Jose da Silva, Gabriel Rocha Santos Knorst, Denise
Bastos, Giulia Matiusso Zumbano, Paulo Scatulin de Souza Dias Neves, Reja Syanne de Aguiar Ribeiro, Sisterolli Diniz, Isabela Louise Caldeira Silva, Gabriel
Gerritsen Plaggert Rejane Sambrana Trelha Montenegro Ribeiro da Silva

Centro Universitário São Camilo, São Paulo SP, Universidade Federal do Pará. Belém PA, Brazil Hospital das Clínicas – Universidade Federal de
Brazil Fundação Santa Casa de Misericórdia do Pará. Goiás. Goiânia GO, Brazil
Universidade Nove de Julho – São Bernardo do Belém PA, Brazil Hospital das Clínicas – Universidade Federal de
Campo – SP – Brazil Hospital Abelardo Santos. Belém PA, Brazil Goiá. Goiânia GO, Brazil
Hospital das Clínincas – Universidade Federal de
raissa_ikeda@outlook.com leandrocosta.dr@gmail.com Goipas – Goiânia – gO – Brazil

Case Presentation: We present a case presentation of Case Presentation: EGM, female, 2 years old, present- bia.borges7897@gmail.com
sisters with infantile neuroaxonal dystrophy (INAD), one ing with recurrent and uncontrolled seizures since
of them was diagnosed. Both are daughters of non-con- 4 days of life, in addition to spasms and myoclonus. Case Presentation: CRS, male, 2 years and 2 months, from
sanguineous parents and there were no complications Neuropsychomotor and hypersecretive delay. Cranial Goiânia-GO. A previously healthy child, 15 months of age,
during pregnancy and childbirth. Case 1: female, 7 years Computed Tomography showed prominence of the ret- daughter of first cousins, after receiving a booster vaccine
and 7 months, the onset of symptoms at 9 months, with rocerebellar cerebrospinal fluid space in the posterior (triple viral vaccine, dTP, hepatitis A, OPV) developed
difficulty in crawling, convergent strabismus, and hyper- fossa, suggesting the possibility of megacisterna magna. high fever, pain in the lower limbs, edema and hyperemia
opia. Progressive worsening of symptoms: at 2 and a half He had frequent seizures during Hospitalization. The at the injection site. He evolved with progressive tetra-
years she developed loss of cephalic support and at 5 years diagnostic hypothesis of Pontocerebellar Hypoplasia paresis with difficulty sitting and supporting the trunk,
started clonus in all four limbs. Physical examination was then raised. The Exome Post Expanded Panel, from inability to walk, spastic hypertonia in four limbs, pres-
without interaction with the environment, the neck was in the DNA extracted from nasal swab, was requested. In ence of abnormal reflexes, macrocephaly and seizures.
hyperextension, without cephalic support. She had nystag- the molecular analysis by exome sequencing, two het- A genetic test was performed showing EIF2B5 alteration
mus, temporal optic nerve pallor, muscle atrophy, reduced erozygous variants were identified in the RARS2 gene, and cranial magnetic resonance imaging suggestive of leu-
strength, and generalized spastic hypertonia. MRI revealed position chr6: 87,589,923, T > C variation, concluding the coencephalopathy with vanishing White matter (VWM).
changes in cerebellar volume and subtle hyper signal on diagnosis of Pontocerebellar Hypoplasia Type IV, asso- Discussion: VWM, also known as diffuse central myel-
T2 and FLAIR. Molecular analysis by exome sequencing ciated with Double Hemiparesis, Epilepsy and Global inopathy and Cree leukoencephalopathy, is a chronic
was performed and two heterozygous variants in the Delay in the Neuropsychomotor Development. For better and progressive white matter disorder exacerbated by
PLA2G6 gene were identified. The association of exams quality of life and refractoriness of epileptic seizures, the infection, head trauma or other stresses. These stresses
with the clinical picture confirmed the diagnosis. Case 2: drugs OXCARBAMAZEPINE, PHENYTOIN, DIAZEPAN, can trigger the first symptoms of the disease or worsen
female, 3 years and 10 months, the onset of symptoms at PHENOBARBITAL, LEVETIRACETAM, CLOBAZAM were existing symptoms and can cause affected individuals to
2 years, with difficulty in walking. Progressive worsening scheduled, in addition to general support. Due to neu- become lethargic or comatose. The inheritance of VWM
of the condition resulting in motor incoordination and rodegenerative dysphagia, a gastrostomy was made to is autosomal recessive. Mutations in any of the five genes
speech impairment, and inability to walk and sit without maintain the diet. Genetic counseling and general guide- encoding the eukaryotic translation initiation factor EIF2B
support. Physical examination with interaction with the lines were carried out. The patient was referred for out- subunits are the cause of VWM and its phenotypic vari-
environment, nystagmus, convergent strabismus and patient, multidisciplinary follow-up and rehabilitation. ants. The partial loss of EIF2B function makes it more
no changes in the optic disc. She had muscle atrophy, Discussion: Pontocerebellar Hypoplasia (HPC) is a group difficult for the body’s cells to regulate protein synthesis
generalized hypotonia, and appendicular and axial dys- of rare, hereditary, progressive neurodegenerative disor- and cope with changing conditions and stress. Glial cells
metria. Absence of tremors and clonus. Laboratory tests ders that start in utero. It is a heterogeneous group of rare appear to have a selective vulnerability to decreased eIF2B
without changes. The patient awaits exome performance neurodegenerative diseases caused by genetic mutations activity, which explains the predominant involvement of
to clarify the diagnosis but receives the same treatment and characterized by progressive atrophy of various parts white matter in the brain. The most distinctive clinical
as her sister. Discussion: INAD is an autosomal recessive of the brain, cerebellum or brain stem. Seven subtypes feature of VWM is progressive neurological deteriora-
neurodegenerative pathology associated with mutation of of mutations have already been identified, and in all of tion, often with prominent ataxia and spasticity. There
PLA2G6 gene. It is rare and has a poor prognosis, whose them there are characteristics of severe cognitive and are five clinical subtypes of VWM based on age of onset.
symptoms are marked by regression of neuropsychomotor motor deficiencies. When known, these disorders are Patients with an earlier age of onset have a more severe
development and neurological impairment, gait disorders, inherited in an autosomal recessive manner. Treatment course and decline more rapidly. Prevalence is unknown.
extrapyramidal symptoms, and ophthalmological abnor- for all types is only symptomatic and supportive, as the Although it is a rare disease, it is believed to be one of the
malities, with rapid and progressive evolution, usually prognosis is of death during early childhood in most of the most common hereditary diseases that affect the white
fatal. Both cases have characteristics like those found in few cases described in the literature, and this was estab- matter. Final Comments: The findings in the literature
the literature: onset of symptoms (average of 14 months, lished for the patient in question. Final Comments: The are compatible with the clinical picture of the patient
with a range of 12-22), characteristic clinical picture and reported case and cited publications raise the complex described here. Elucidating the clinical course of VWM
progressive worsening, no type of complications during situation that is the described disease and that despite will be useful to facilitate early diagnosis and avoid spe-
pregnancy and childbirth, and non-consanguineous par- the poor prognosis and the absence of specific treatment, cific situations that could trigger the deterioration of the
ents. Final Comments: Even presenting similar charac- the correct diagnosis and stabilization of a symptomatic disease, as well as to better understand the pathogenesis
teristics to other reports, the cases bring important data and supportive therapy can improve the quality of life of and provide subsidies for possible therapeutic targets and
that aggregate INAD study, in search of better prognosis patients. patients and family members. treatment strategies in the future.
and conduct.

Neurologia Infantil Neurologia Infantil


Neurologia Infantil

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XXX Congresso Brasileiro de Neurologia

TL 1105752 TL 1105899 TL 1105937

MUTIPLE UNUSUAL CONDITIONS AUTISM SPECTRUM DISORDER (ASD) SÍNDROME DE RETT: RELATO DE CASO
ASSOCIATED WITH CEREBRAL PALSY IN A ASSOCIATED WITH HIPPOCAMPAL SOBRE AS COMPLICAÇÕES DE UMA DOENÇA
PRETERM WAS BORN IN BAHIA SCLEROSIS AND MESIAL TEMPORAL LOBE NEURODEGENERATIVA RARA
EPILEPSY
Ítalo Barros Andrade, Juliana de Oliveira Nunes, Gabrielle Miranda Magalhães Pinto, Isabelle Diniz
Beatriz Souza Martins, Álvaro Luiz dos Santos Breno Henrique Gomes da Costa, Zenilda Gueiros Melo, Maria Eduarda Teixeira Benício, Déborah
Araujo, Samuel Luca Rocha Pinheiro Silvestre, Artur Pereira de França Medeiros, Elder Araújo Leitão, Filipe Caetano Siqueira, Fabiane
Machado Leite Elpídio de Sá, José Lucivan Miranda
Faculdade Santo Agostingo. Vitória da Conquista
BA, Brazil Universidade de Pernambuco. Garanhuns PE, Brazil Universidade Federal do Ceará. Fortaleza CE, Brazil
UFBA. Vitória da Conquista BA, Brazil Universidade Federal do Cariri. Barbalha CE, Brazil
brenohgcosta@gmail.com
vc_italo@hotmail.com gabriellemmp1@hotmail.com
Case Presentation: Female, 4 years old, had her first con-
Case Presentation: A 3-year-old male was diagnosed sultation 1 year ago due to epileptic seizures and difficul- Case Presentation: EDMA is a 4 years and 11 months old
with epileptic encephalopathy, cerebral palsy, autism, ties in cognitive development. The characteristic episodes female, born by Cesarean section. Her mother presented
Autosomal Recessive Deafness 77 and identification of focal seizures started at 9 months of age, the last one with asthma and fibromyalgia while pregnant. When
of genetic variants (CAXNA1C, FKTN, SLC19A3), with occurring when the patient was 2 years old. Anamnesis: EDMA was 6 months old, she started presenting myoc-
delayed neuropsychomotor development. According to antisocial behavior, echolalia, and stereotypes. She had lonic-atonic seizures. At 16 months, she was diagnosed
the mother’s report, during pregnancy, she had constant previously used depakene (suspended due to jaundice with Autism Spectrum Disorder (ASD). At 17 months,
symptoms of severe headache (10/10), agitation, failure observed in the physical exam) and valproate. Valproate EDMA began to walk, but, after 3 months, she presented
to maintain sleep, a seizure episode that was treated with was replaced by carbamazepine, and clonazepam was with ataxic gait. At 23 months, a molecular study for Rett
carbamazepine in the second trimester, preeclampsia, introduced to avoid possible withdrawal crises. In the Syndrome (RS) was realized, detecting a variance of uncer-
hematemesis and gestational diabetes. In the prenatal return visit, in regular use of the medications, the absence tain meaning of heterozygosity on the gene MECP2. When
exams, the patient was, in most cases, without intrauter- crisis did not reoccur, but the cognitive and behavioral she turned 2 years old, she presented Neuropsychomotor
ine movements and at other times with heart arrhythmia. symptoms, compatible with the autistic spectrum, still developmental delay (NDD), manual stereotypies, global
Therefore, an emergency caesarian section was performed persistent. Magnetic resonance imaging showed right hip- hypotonia, aggressiveness and intractable epilepsy. An
at 34 weeks in the state of Bahia. The patient was uncon- pocampal hypotrophy with hypersignal on T2, suggestive electroencephalography evidenced suggestive findings
scious and cyanotic, required orotracheal intubation and of medial hippocampal sclerosis. Electroencephalogram of diffuse encephalopathy. 2 months later, a MRI exhib-
an exchange transfusion, was diagnosed with epilepsy in (EEG) showed slow-wave spike complexes of 5 to 6 Hz in ited discrete encefalic sulci and fissures global emphasis.
the first days of the ICU, the transfontanellar ultrasound central and parietal regions intermittently with slowing At 3 years and 11 months, EDMA underwent a gastrec-
examination identified grade 3 of peri-intraventricular of regional rhythms and negative spikes in right temporal tomy and, at 4 years and 1 month, she had an episode of
hemorrhage, in addition to early neonatal sepsis. After (T4) and left central (C3). There is also excessive slow and bronchoaspiration during a seizure, which mostly occur
his condition improved, he became jaundiced and was fast activity without Alpha-Theta predominance, typical during her sleep, with an interval of 2 days between epi-
treated with phototherapy. At 7 months, the first case of of the patient’s age range. The patient is on a combined sodes. Discussion: this case describes complications
coma, when the mother noticed a cyanotic appearance, follow-up with speech therapy, psychopedagogy and related to RS, a rare neurodegenerative disease caused
leading to the child’s second Hospitalization. On return occupational therapy. Discussion: Medial temporal lobe by a mutation on the X chromosome gene MECP2. This
to the ICU, with oxygen saturation of 60%, Glasgow exam epilepsy is commonly manifested by focal seizures, with condition affects neurodevelopment and the patient
of 4, magnetic resonance imaging of the skull was per- EEG manifestations as spike-like epileptiform activity and usually has a typical development until 6 months, when
formed and results were observed with cerebral atrophy sharp wave, which may be followed by slow wave, with symptoms, such as NDD, smaller brain growth, sleep
and increase in the interpeduncular cistern. At two years maximum electronegativity in leads T3-F7/T4-F8, espe- disorders, global hypotonia, irritability, intractable epi-
for age, he had 3 episodes of cardiorespiratory arrest. The cially in sphenoidal leads, having already been reported lepsy, gait dysfunction, disfagia and gastroesophageal
electrocardiogram performed for investigation showed by Andrade-Valença (2006) in concomitance with hip- reflux. Furthermore, this progressive encephalopathy,
right atrial and biventricular overload. One year later, pocampal sclerosis. However, the association of these by presenting absence of protein 2 (MeCP2) methyl-cyto-
he was Hospitalized due to the presence of urine calcu- pathologies with the ASD is an expensive record in the kine ligand, generates a discrete encefalic atrophy, since
lus and severe UTI, grade 3 phlebitis in the lower limb, literature. In the meantime, besides the patient in ques- MeCP2 is important for its adequate development. In
pericardial effusion, constipation and convulsion, with tion having clinical imaging and exams proving the three addition, the presence of manual stereotypies, similar to
the latter having been treated with diazepam and phe- conditions, the diagnosis of these conditions was made handwashing, and lower social and environmental inter-
nytoin. Holter confirmed ectopic atrial rhythm. Currently, early. Thus, therapy with carbamazepine and clonazepam est may lead to the equivocate diagnosis of ASD at early
on an outpatient basis, he is stable, using topiramate 50 was efficient in controlling the epileptic seizures. As for stages of the disease. A gastrectomy is also an important
mg, phenobarbital drops, risperidone 1 mg, clobazam 10 the behavioral changes, it is expected that with advancing strategy for preventing bronchoaspiration caused pneu-
mg, levetiracetam 100 mg/ml, pericyazine 1 mg/drop and age and multiprofessional intervention they will evolve monias, some of the most prevalent causes of death in
biotin 10 mg/ml, all via gastrostomy tube. This case por- with improvement. Final Comments: The case is unique SR. Final Comments: in face of RS complications, more
trays the consequences of common underlying genetic because of the diversity of symptoms stemming from the studies on this encephalopathy are necessary, since the
predispositions and deleterious events in the prenatal combination of two distinct diagnoses that complement correct diagnosis and multiprofissional intervention can
and neonatal periods in a patient with cerebral palsy, each other in the patient’s clinical picture. The therapy contribute to the improvement of social abilities and to
which is already a condition associated with an increased used in the case, along with the multiprofessional fol- the attempt of preserving motor skills.
risk for the occurrence of other medical comorbidities low-up has had good results.
that interfere with the quality of life of the patient and
caregiving family. Neurologia Infantil
Neurologia Infantil

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TL 1106148 TL 1106308 TL 1106484

BILATERAL OPEN LIP SCHIZENCEPHALY, PAEDIATRIC-ONSET OF HTLV-1 ASSOCIATED WEST SYNDROME ASSOCIATED WITH
A CORTICAL MALFORMATION: A CASE TROPICAL SPASTIC PARAPARESIS, DUE AUTOSOMAL DOMINANT INTELLECTUAL
PRESENTATION TO VERTICAL TRANSMISSION – A CASE DEVELOPMENT DISORDER 13: CASE
PRESENTATION PRESENTATION
Ana Beatriz Vieira Pacheco, Isabela Pires Porto
Santarém Pinto, Thiago Parreira Guzzo Sarah Diógenes Alencar, Paula Caracas Barreto, Luan Monte Barroso, Marina Mayara Pereira,
Ana Letícia Vieira de Oliveira Pinheiro, Alessssandra Cristiano Ribeiro Soares, Denise Maria Menezes
Unesc / Uninassau – Vilhena – RO – Brazil
Braga Cruz Guedes de Morais, Ian da Silva Ribeiro, Cury
isabela.porto27@gmail.com Ana Silvia Sobreira Lima Verde, Sylvio Ricard
Gonçalves de Souza Lima, Karoline Ferreira Mororó UNINOVAFAPI. Teresina PI, Brazil
Menezes, Fernanda Martins Maia Carvalho Uninovafapi. Teresina PI, Brazil
Case Presentation: A 3-year-old female patient was diag-
UNINOVAFAPI. Teresina PI, Brazil
nosed with bilateral open-lip schizencephaly at 4 months
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
of age. The pregnancy was discovered at 6 months, with luanmontebarroso@gmail.com
Hospital Universitário Walter Cantidio HUWC
placental abruption and loss of amniotic fluid. The patient
Escola de Saúde Publica ESP
was delivered by cesarean section, without complications. Case Presentation: Infant, 8 months and 13 days old,
Hospital Geral de Fortaleza HGF
At 20 days, she had the first seizure and the second at 2 at 5 months of age, he started to have partial myoclonic
months, when the investigation was started. At 4 months, sarah_diogenes@hotmail.com seizures that presented with the upper limbs in repetitive
the patient was diagnosed with open-lip Schizencephaly extension movements. Initially, the seizures lasted for
through tomography, with the presence of three clefts that Case Presentation: M.L.F.O, 13 years old, female, previ- seconds and had a frequency of 2 to 3 episodes per day.
communicate the ventricular system to the surface of the ously diagnosed with neurogenic bladder, started at age An electroencephalogram (EEG) was performed, which
pia mater, showing clefts in the temporal region on the 10 with an insidious condition of mechanical arthralgia showed a pattern of hypsarrhythmia, and West Syndrome
right, and in the frontal and temporal lobes. on the left, with in the right knee, associated with difficulty in walking (WS) was diagnosed due to the EEG pattern, spasms and
initiation of carbamazepine, but without resolution, being and non-progressive or ascending asymmetrical lower developmental delay. His first Hospitalization was at 7
Hospitalized in a pediatric ICU with ventilatory support limb paresis. Due to a family history of dermatopolymy- months, when he had a crisis that lasted more than 5
due to repeated crises. After changing the medication, the ositis in a first-degree relative, she was admitted to the minutes. During Hospitalization, he performed several
condition stabilized for a period, with the use of phenyt- Hospital for investigation of inflammatory myopathies complementary exams to investigate the etiology of the
oin, valproic acid and phenorbarbital. In this year, 2022, in a pediatric rheumatoid department. On examination, condition, EEG, magnetic resonance and exome. This con-
there was a return of seizures, associated with a drop in hyperreflexia was identified in the lower limbs with an firmed Autosomal Dominant Intellectual Development
oxygen saturation, carbamazepine was prescribed again increased reflex area on the left, inexhaustible clonus Disorder 13, which presents with neuropsychomotor
and neuleptil was started, with the medications already on the left, grade IV strength distally in the left lower developmental delay, epilepsy, cortical changes in CNS
prescribed. On physical examination, the patient has a limb and paretic gait on the left, with no other clinical migration and hypotonia. She currently performs thera-
delay in neuropsychomotor development, does not hold findings. Initial tests showed CPK 211, TGO 24, TGP 17, pies with valproic acid, gabapentin, levetiracetam, nitraz-
her head, does not sit without support, does not walk and DLH 249, non-reactive Anti-Jo1. During investigation of epam and ACTH for the treatment of epilepsy and WS.
does not speak, but has preserved speech understanding. infectious causes, the presence of antibodies to HTLV-1 Discussion: The WS is the most common encephalop-
Currently, the condition has improved with the medica- was detected by Western Blot, with the presence of GD21, athy in the first two years of life, consisting of: epileptic
tions in use and he is undergoing continuous medical p19, p24 and rgp 46-I bands. Electroneuromyography of spasms, hypsarrhythmia on encephalography, and delay
follow-up. Discussion: Schizencephaly is a rare malfor- the lower limbs and resonance of the right thigh did not or arrest in neuropsychomotor development. In this
mation of the cerebral cortex (1.48/100,000 births) that show any changes, however spinal resonance showed aspect, the diagnosis of WS is made by combining clin-
can be open or closed, bilateral or not. The etiology is still apparent thinning of the spinal cord of the dorsal segment. ical characteristics and electroencephalographic (EEG)
not well understood, as well as the period of embryonic The exclusion of acute infectious, metabolic, and demye- findings. The most prevalent characteristic EEG finding is
development that is involved in the development of the linating pathologies and the positivity of the serology for a hypsarrhythmic pattern. In addition to the EEG, imag-
malformation. The clinical manifestations of the disease the HTLV-1 virus led to the diagnosis of tropical spastic ing tests and genetic tests are necessary for the etiology
are associated with the degree to which the brain area paraparesis, pyramidal motor sequelae caused by HTLV of the syndrome. The patient’s exome report indicates
was affected, ranging from individuals who lead a normal infection, resulting from vertical transmission, whether via pathogenic variants in the DYNC1H1 gene, which would
life without manifestations, to severe cases of neurolog- breastfeeding, transplacental or birth canal, considering correlate with the presented phenotype of Autosomal
ical impairment, with epilepsy being the most common maternal serology also positive for this virus. Discussion: Dominant Intellectual Developmental Disorder 13, clin-
manifestation. Final Comments: This study brings the The human T cell lymphotropic virus type I (HTLV-1) was ically characterized by intellectual disability, behavioral
symptomatic management of a cortical malformation, the first retrovirus isolated in humans, in a patient with changes, epilepsy, central nervous system abnormalities
showing the importance of continued monitoring so that cutaneous lymphoma. Currently, its prevalence in Brazil and variable dysmorphisms. Final Comments: The SW
there is an improvement in the quality of life of the child is 0.47%, and its manifestation as early-onset myelopathy constitutes 2% of all childhood epilepsies, being, there-
and parents, reducing the damage caused by clinical has been described in 27 cases in the country. According fore, a rare syndrome and despite the clinical signs, it is
manifestations and allowing the child to have a better to Bittencort et al, in a study carried out in Salvador, the essential to perform imaging tests for diagnosis. It is a
life. as close to normal as possible. infection is acquired mainly through breastfeeding, being pathology with a poor prognosis, as it has no cure, evolving
the main form of vertical transmission, and then through largely into difficult-to-control epilepsy associated with
sexual intercourse. Final Comments: Spastic paraparesis delayed neuropsychomotor development. Therefore,
Neurologia Infantil is a severe and disabling myelopathy, with a chronic and early diagnosis is necessary to start pharmacological
progressive presentation, involving the pyramidal tract, treatment and strict follow-up with a multidisciplinary
with proximal leg weakness, low back pain and neurogenic team, including speech therapy, physical therapy and
bladder. When it begins in childhood, it is manifested by neurologist to control the crises.
pyramidal signs followed by gait disturbances and sensory
symptoms. The evolution is usually rapidly progressive,
making early diagnosis essential. Neurologia Infantil

Neurologia Infantil

182
XXX Congresso Brasileiro de Neurologia

TL 1106605 TL 1106630 TL 1105323

ATYPICAL OCULO-CEREBRO-RENAL MACROCRANIA, PHENOTYPIC ALTERATIONS ACUTE CEREBRAL THROMBOSIS CAUSED BY


PRESENTATION – A REPORT CASE OF AND INTELLECTUAL DEFICIT: A CASE FLOW DIVERSION STENT AND RESISTANCE
LOWE’S SYNDROME PRESENTATION OF SOTOS SYNDROME TO TICAGRELOR – CASE PRESENTATION
Victoria Faustino Silva Reis, Lara Cordeiro Victoria Faustino Silva Reis, Henrique Nascimento Carla Beatriz Jales da Silva, Mariana Jacob de
Magalhães, Gabriel Vianna Pereira Aragão, Murilo Dourado, Fernanda Souza Gracílio Silva, Samantha Castro, Rafaela Sousa Severo, Antônio de Matos
Lopes Coelho, Samantha Lopes Oliveira, Iana Maciel Lopes Oliveira, Murilo Lopes Coelho, Iana Maciel Lima Neto
Silva Souza, Camilo Vieira Santos, Julia Monteiro silva Souza, Juliana Silva Almeida Magalhães,
Barros Pereira Carvalho, Juliana Silva Almeida Camilo Vieira Santos, Julia Monteiro Barros Pereira Unifamaz. Belém PA, Brazil
Magalhães, Sâmara Pinto Vasconcelos Carvalho, Sâmara Pinto Vasconcelos carlajales1507@gmail.com
Escola Bahiana de Medicina e Saúde Pública. Escola Bahiana de Medicina e Saúde Pública.
Salvador BA, Brazil Salvador BA, Brazil Case Presentation: A 52-year-old female presented to the
Hospital Martagão Gesteira. Salvador BA, Brazil Hospital Martagão Gesteira. Salvador BA, Brazil interventional neurology team for treatment of saccular
aneurysm located in the ophthalmic segment of the left
victoriareis20.2@Bahiana.edu.br victoriareis20.2@Bahiana.edu.br internal carotid artery. As comorbidities she reported hav-
ing Systemic Arterial Hypertension (SAH). She reported
Case Presentation: JJS, male, 10 months old. Born by vag- Case Presentation: AOD, male, 9yo. Genitor reports using losartan 50mg and two weeks before the procedure
inal delivery, APGAR 9/9, presented significant hypoglyce- asymmetric skull growth of the infant at 3 months of age. the neurologist prescribed prasugrel and acetylsalicylic
mia and respiratory distress in the ward, and was referred Background: TPAL 1-0-0-1, HC: 37cm at birth, delayed acid 100mg. Cerebral angiography was requested for treat-
to the ICU, where he stayed Hospitalized for 15 days. After NPMD (first words at 1y2m), no family history of neuro- ment of the aneurysm. In the cerebral angiography a flow
ICU discharge, he was admitted to the maternity ward logical disease. He reports a moderate to strong and pul- diversion stent was positioned in the left middle cerebral
due to frequent regurgitations. During Hospitalization, satile holocranial headache that worsens with physical artery. The final control showed incomplete opacifica-
regression of the sucking reflex and global hypotonia were activity and noise. Neurological examination: turricephaly, tion of the internal carotid due to platelet aggregation,
observed, and he was referred to a neuropediatrician for HC: 62cm, symmetrical typical facies with micrognathia, denoting subsequent stenosis. Balloon angioplasty was
evaluation for SMA investigation, with negative molecu- hypertelorism, distant teeth, atypical gait, speech with- performed, but was unsuccessful. It was necessary to
lar results for neuromuscular diseases. During the multi- out impairment, sensitivity and tone preserved, MF 5/5 use a hyperglide balloon, showing improvement of the
professional evaluation, congenital bilateral cataract and globally, osteotendinous reflexes 2/4 globally, symmet- left internal carotid artery, but presenting occlusion of
renal tubular acidosis were diagnosed; serologies came rical, without pyramidal signs, no changes in the cranial the upper trunk of the left M1 associated with the avas-
back negative. Thus, Lowe’s Syndrome (oculo-cerebro-re- nerves. Segmental examination: syndactyly between the cular area. A Trevo stent progression was performed in
nal syndrome) was investigated and diagnosed. Patient 2nd and 3rd left podotactiles, increase of the 2nd right the same catheter, released in the m3-m2 segment of the
evolved with neuropsychomotor developmental delay podotactile, presence of multiple surgical scars from left middle cerebral, and thrombus was removed. Being
(NPDM), visceromegaly, disphagia, requiring gastros- lipoma’s removal and multiple verrucous lesions in the requested Computed Tomography of the skull for fol-
tomy. Neurological examination: pupils punctiform and cervical and inguinal region. The suspicion of hydro- low-up. Discussion: Taking into consideration that the
leukocoria, nystagmus, global hypotonia and preserved cephalus was ruled out after skull MRI, which showed patient in question takes the platelet antiaggregant tica-
cutaneos extension. Discussion: Lowe’s syndrome has an macrocrania, multiple enlargements of the perivascu- grelor 90 mg, it is necessary to point out some information
incidence of 1: 500,000 live births, being a disease clinically lar spaces in the subcortical regions, especially in the about it. Ticagrelor is a platelet antiaggregant medication
characterized by congenital cataract, renal tubular aci- frontoparietal region, cystic lesions in the bilateral fron- that acts differently from clopidogrel and prasugrel. The
dosis and changes in the central nervous system, such as to-temporo-parietal and periventricular left white matter. 3 drugs have the same mechanism of action, which is to
neuromuscular symptoms, intellectual disability, epilepsy Clinical presentation compatible with Sotos Syndrome. inhibit the platelet receptor P2Y12, but while the thieno-
and neurodevelopmental delay. Lowe’s is a disease with Discussion: Sotos Syndrome is clinically characterized by pyridines act irreversibly, ticagrelor has a reversible effect
an X-linked inheritance pattern, and it’s associated with macrocephaly, phenotypic changes, intellectual deficit, (LI et al., 2022). Based on the case of the patient in ques-
mutations in the OCRL gene, a gene involved in multiple autistic spectrum disorder, hyperextensibility of joints, tion and according to a study published by the American
intracellular processes involving endocytic trafficking scoliosis and epilepsy. It is a genetic disease marked by Journal of Neuroradiology, several factors can influence
and actin’s skeleton dynamics. Lowe’s Syndrome can be a pathogenic variant in the heterozygosity in the NSD1 intracranial aneurysm thrombosis, such as: flow-diverter
attributed to a de novo variant in about ? of the cases. So gene, and most mutations are de novo, as we could see in stenting, which causes reduced blood flow. However, due
far, there is no treatment for this genetic condition, but the presented case, in which there is no family history of to the lack of detailed measurements of intra-aneurysmal
symptomatic treatment such as cataract surgery and similar neurological conditions. The diagnosis is made by flow, its real impact remains uncertain (BRINA, 2019).
physical therapy can be performed to preserve existing identifying the mutation in a genetic panel. So far, there Final Comments: The reported case and exposed pub-
motor function. Final Comments: Lowe’s Syndrome is a is no treatment for the genetic condition, but symptom- lications bring the discussion succession of infrequent
rare condition, predominantly affecting boys. The pres- atic treatment can be done, such as the performance of events. The patient used prasugrel and acetylsalicylic
ent case is compatible for the condition, with expected adjunct therapies like speech and physical therapy. The acid within two weeks prior to the angiography and at
clinical history and presentation. It is suggested that fur- literature has not yet demonstrated an association with the time of the procedure was administered ticagrelor.
ther investigation with genetic testing be performed and migraine or the presence of lipomas. Final Comments: Conduct after placement of a flow diversion stent in the
follow-up of this child with therapies and interventions The present cause is highly suggestive for the condition, left middle cerebral artery caused cerebral thrombosis,
aimed at a better prognosis. with compatible clinical history and presentation. Further which was resolved with thrombectomy and the use of
investigation with genetic testing is suggested for full tirofiban hydrochloride.
diagnoses confirmation. Besides that, follow-up of this
Neurologia Infantil child with therapies and interventions for a better prog-
nosis are suggested. Neurologia Intervencionista

Neurologia Infantil

183
XXX Congresso Brasileiro de Neurologia

TL 1106072 TL 1105130 TL 1105182

CEREBRAL ISCHEMIA DUE TO EARLY DIAGNOSIS OF HEREDITARY PERIPHERAL POLYNEUROPATHY


SPONTANEOUS DISSECTING ANEURYSM OF AMYLOIDOSIS AFTER COVID-19 INFECTION: A CASE
THE ANTERIOR CEREBRAL ARTERY TREATED PRESENTATION
Ligia Rocha Andrade, Salim Lazaro Balassiano,
WITH A FLOW DIVERTER STENT SILK VISTA Eduardo Barbosa, Eduardo Davidovich, Marcia Luís Felipe Ferreira Marques, Luiz Severo Bem
BABY Rodrigues Jardim, Izabela Rodrigues Pitta, Ana Júnior, Nilson Batista Lemos, Otávio da Cunha
Siquara, Clarissa Neves Spitz, Robson Vital, Larissa Ferreira Neto, Joaquim Fechine de Alencar Neto,
Ian Felipe Barbosa Souza, Márcio Azevedo de
Bittencourt Carvalgo Hildo Rocha Cirne de Azevedo Filho, Júlio Augusto
Andrade
Lustosa, Arthur Oliveira Lira, Maria Luísa Rocha,
Hospital universitario pedro ernesto. Rio de Janeiro
Santa Casa de Misericórdia da Bahia – Hospital Luis Bandeira Alves Neto
RJ, Brazil
Santa Izabel. Salvador BA, Brazil
Hospital universitario pedro ernesto – niteroi – RJ Universidade do Estado de Mato Grosso – Cáceres
ibarbosa_10@hotmail.com – Brazil – MT – Brazil
Universidade Federal de Pernambuco. Recife PE,
ligiaandradee@gmail.com
Case Presentation: A 68-year-old man admitted with Brazil
sudden onset of weakness in his right leg, with no other Centro Universitário Unifacisa. Campina Grande PB,
Case Presentation: Asymptomatic individuals carrying
neurological symptoms. He did not report headache. There Brazil
the hereditary amyloidosis gene are screened and submit-
were no illnesses in his medical history. Head computed Universidade Católica de Pernambuco. Recife PE,
ted to annual assessments in our service and it include a
tomography did not show ischemia or hemorrhage, while Brazil
neurological examination, a questionnaire on symptoms
CT angiotomography revealed aneurysmal dilatation of Departamento de Neurocirurgia do Hospital da
of dysautonomia, complementary tests that include elec-
left anterior cerebral artery (ACA), partially thrombosed. Restauração. Recife PE, Brazil
troneuromyography and screening for dysautonomia, such
Subsequent cranial magnetic resonance imaging showed Centro Universitário de Brasília. Brasília DF, Brazil
as Quantative Sensory Test(QST), heart rate variability
acute ischemia in the left ACA territory. Digital subtrac- Universidade de Pernambuco. Recife PE, Brazil
HRV) and reflex sympathetic response (RSR). We iden-
tion angiography confirmed aneurysmal dilatation in
tified abnormal findings in two young women carriers of luisfelipe.marques585@gmail.com
the A1-A2 transition of the left ACA measuring 10 x 7 x
the hereditary amyloidosis transthyretin. A 32-year-old
6 mm, with a wide implantation base and the presence
female carrier of Phe84Leu gene presented symptoms of Case Presentation: Patient, 42 years old, male, no comor-
of a small stenosis in the post-aneurysmal segment,
dysautonomia (blurred vision, dry eyes, increased sweat- bidities, attended at an outpatient neurosurgery service
suggesting a dissecting etiology. Initially, he was treated
ing in her hands, orthostatic intolerance and constipation). complaining of burning pain in the plantar region of both
with dual antiplatelet therapy. After 14 days of ictus, he
The electroneuromyography showed mild carpal tunnel feet, a condition that started after 3 months of mild infec-
underwent endovascular treatment with the flow diverter
syndrome bilateral. The reflex-sympathetic response was tion of the airways by COVID-19. On physical examination,
stent (FDS) Silk Vista Baby – SVB (Balt, Montmorency,
abolished in the four limbs. She was then submitted to bilateral symmetrical paraparesis was identified, and a
France), without complications. Patient was discharged
a salivary gland biopsy which revealed deposit of amy- grade 3 strength rating for plantar flexion and dorsiflexion
2 days later with minimal right leg weakness, modified
loid material. Her father was the index case and at the and for hip flexion-extension. Was also identified change
Rankin Scale 2. Discussion: Spontaneous dissections of
age of 64 he started a condition of polyneuropathy and in orthostatism and in the stimulation of ambulation,
intracranial arteries are rare and there are few reports of
dysautonomia, receiving a late diagnosis and treatment with balance deficit due to alteration in proprioception.
spontaneous dissections of ACA, whose optimal treatment
was not possible, dying from disease complications. The Paresthesia in both heels and distal phalanx of the hal-
remains uncertain. Endovascular treatment of ACA dis-
second case was a 22-year-old female carrier of Val50met lux in both feet. Also reported intense pain in both lower
section has been reported as a feasible alternative, espe-
gene. She had reflex sympathetic response abolished in limbs, graded in 10 on the visual analogue pain scale. The
cially in patients in whom subarachnoid hemorrhage is
the 4 segments and the quantative sensory test (QST), current clinical condition directly affects their work and
the main clinical presentation. However, in the reported
revealed warm detection and warm pain thresholds were daily life activities. Was reported previous use of opioids
cases of ACA dissection with ischemia alone, conserva-
higher and the cold detection and cold pain thresholds to control symptoms, being refractory to treatment and
tive treatment showed good outcome in most cases. FDS
were smaller when compared to a sex and age matched without improvement. With the progressive worsening
were initially designed and constructed for the treatment
healthy control. She also underwent salivary gland biopsy, of the clinical condition and considering that the patient
of aneurysms that could not be easily treated using more
which also confirmed the deposition of amyloid material has a significant kinetic-functional deficit, treatment with
conventional endovascular approaches. The SVB is the
in the tissue. Her aunt was the index case and presented antidepressants and anticonvulsants at optimized doses
first FDS that can be delivered via a 0.017 inch microca-
polyneuropathy and dysautonomia at the age of 38, and and spinal electrode treatment was indicated for neuro-
theter and is designed to target aneurysms of the distal
treatment was not possible because it was in an advanced pathic pain. Discussion The post-COVID-19 syndrome
vasculature for which existing FDS are not optimized.
stage. Discussion: Hereditary amyloidosis transthyretin brought with it peripheral polyneuropathy. One of the
By implanting the densely woven mesh in the parent
is a rare, autosomal dominant systemic disease caused main symptoms reported by patients who suffer from this
vessel, the aneurysm neck is covered and blood flow is
by extracellular deposition of insoluble amyloid fibrils syndrome is pain, which can be associated with several
directed away from the aneurysm orifice. Despite the lack
formed by the mutated transthyretin protein. The clin- other systemic disorders. The wide range of neurologi-
of studies, SVB has achieved sufficient occlusion rates of
ical presentation and the age of onset varies. The dis- cal manifestations resulting from the immune response
intracranial aneurysms originating from peripheral seg-
ease is often rapidly progressive, so an early diagnosis is to the virus has led to studies on the pathophysiology of
ments, which are comparable to conventional FDS with
important for a an effectively treatment. Final Comments: this it. The main mechanism associated with the onset
acceptably low complication rates. Final Comments:
Systematic and regular monitoring of an asymptomatic of this varied condition is associated with the invasion
Dissecting aneurysm is a rare cause of cerebral ischemia
carrier is necessary to detect early signs of hereditary of the blood-brain barrier by the virus, in addition to the
in the ACA territory and patients tend to have a good clin-
amyloidosis transthyretin. systemic pro-inflammatory action due to the release of
ical outcome, as in the case presented. The SVB is a new
promising device as a possible treatment. numerous cytokines. Peripheral polyneuropathies in
patients post-COVID-19 are frequent and associated not
Neuropatias Periféricas only with toxicity resulting from immunological aspects,
Neurologia Intervencionista but also due to the neurotoxic action of the main drugs
used to combat the disease. It was noted that COVID-19
does not have a direct influence on the peripheral nerve
branches, and no infectious neuropathy caused by this
virus were established. Final Comments In view of the
cases already reported in the literature and the frequency
of neurological complaints after COVID-19 infection, there
is a relevance of these studies for future clarifications on
the subject and treatment measures for it.

Neuropatias Periféricas

184
XXX Congresso Brasileiro de Neurologia

TL 1105189 TL 1105209 TL 1105223

NEUROPATIA RADIAL SECUNDÁRIA À POEMS SYNDROME, ALTHOUGH RARE, COVID-19 INDUCED NEUROPATHY
PRÁTICA DE CROSSFIT PRESENTS A CONSTELLATION OF CLINICAL
Tereza Brenda Clementino de Freitas, Vítor Silveira
FINDINGS THAT ALLOWS ITS EARLY Reis Canêdo, Murilo Tomaz Rocha, Leila Gabriele
Silvya Nery Bernardino, Isabel Nery Bernardino
de Souza, Regina Nery Bernardino de Souza, DIAGNOSIS – A CASE PRESENTATION Nunes Silva, Vinícius de Noronha Barboza, Eloisa
Paulo Ricardo Andrade de Medeiros, Kalil Ibrahim Alves Viana, Maria Clara Barbosa de Oliveira, Gabriel
Gabriela da Silva Cremonese, Ivanio Alves Pereira
Mansur, Haldson Cesar Barbosa Neto, Tainá Montini Maciel Morais, Samila Marissa Pinheiro Gomes
Zampolli, Fernando Henrique Morais de Souza Universidade do Sul de Santa Catarina. Palhoça SC,
Universidade Federal Rural do Semi-Árido. Mossoró
Brazil
Hospital Getúlio Vargas. Recife PE, Brazil RN, Brazil
Universidade de Pernambuco. Recife PE, Brazil gabicremonese@hotmail.com
terezabrenda13@gmail.com
Hospital da Restauração. Recife PE, Brazil
Case Presentation: A 55-year-old male patient pre-
s-nery@hotmail.com Case Presentation: Male patient, 35 years old, hiper-
sented acrocyanosis with intense pain in the hands and
tensive and obese, was addimited in ICU by COVID-19
paresthesia in boot-glove pattern associated with a sig-
Case Presentation: Patient GDL, male, 29 years old, pre- infection, spending 14 days intubated and 7 days in the
nificant weight loss, asthenia and hyporexia, in 3 years of
viously healthy, reports that after a rope climbing CrossFit ward. While in the Hospital, the patient started to feel
evolution. On clinical examination, in addition to acro-
activity on 12/01/21, presented with left humerus fracture, lower limb weakness, paresthesia and neuropathic pain,
cyanosis, he had telangiectasias on the upper body and
resulting in wrist fall (Strength 0/5) and elbow extension which until the day he was discharged the symptoms
face, bilateral gynecomastia, palpable hepatomegaly,
déficit (Strength 1/5). Submitted to surgery for fracture were assigned to be related by both collateral effects of
global hyporeflexia, 4+ muscle strength in the extremi-
correction with application of locking plate on 03/12/21, medications administered and the time he spent at the
ties, reduced pain sensitivity in the lower limbs, reduced
without complications. After the procedure, the patient ICU itself. Already at home the lower limb weakness got
vibration in the upper and lower limbs. Complementary
persisted with clinical presentation. An electroneuro- worse, especially in the proximal musculature of the right
exams showed a polycythemia and monoclonal peak
myography done in 02/01/22 shows accentuated fibers leg (iliopsoas and quadriceps). Thus, was solicited an
between beta 2 and gamma globulin fractions, a chronic
impairment of radial nerve, probably located above the electroneuromyography that showed serious injury and
inflammatory demyelinating pattern polyneuropathy and
spiral sulcus, with present denervation and no signs of prominent denervation of both right femoral and left sci-
osteosclerotic and lytic areas in the body of the sternum
reinnervation. Patient is currently being followed up atic nerve, in addition to sensory axonal polyneuropathy
and osteosclerotic lesions in the skull. A bone marrow
with physical therapy with a strength grade of 1/5 for left in the lower limbs, post COVID-19. Therefore treatment
aspirate was performed, which revealed 9% of plasma
wrist extension and 2/5 for ipsilateral elbow extension. was started with pulse therapy of 1g methylprednisolone
cells and in the sternal biopsy bone marrow hypocel-
Discussion: Crossfit is a program developed to promote for 5 days and pregabalin with progression up to 150mg
lularity with discrete polyclonal reactive plasmacytosis.
the improvement of physical conditioning with exercise 12/12h. Upon return, the patient reports improvement
The set of such findings allowed the final diagnosis of
variability and high intensity¹. It has gained a lot of pop- in neuropathic pain and paresthesia, but maintains a
POEMS Syndrome, an Osteosclerotic Multiple Myeloma.
ularity and several studies have emerged with the pur- motor deficit in the lower limbs. The electroneuromyog-
Treatment was based on cycles of dexamethasone, diso-
pose of highlighting the physical benefits and answering raphy was repeated, which showed signs of femoral and
dium pamidronate, amitriptyline and gabapentin for neu-
questions about safety in its practice, mainly related to the sciatic reinnervation, but still with severe injury in these
ropathic pain management, followed by a VTD protocol
higher prevalence of injuries.² Feito et al evaluated groups nerves. Discussion: COVID-19 is a disease with tropism
(Bortezomib+Doxorubicin+Thalidomide). Discussion:
of Crossfit practitioners for 04 years and showed injuries for the respiratory system, but as a reflection of serious
This report aims to expose a rare case, with an estimated
to the shoulders (39%), back (36%), knees (15%), elbows infections by SARS-COV-2, patients can develop neuro-
prevalence of 0.3 per 100,000 people, which had an evo-
(12%) and wrists (11%) were the most common³. Costa et logical deficits, such as motor and sensory ones. Among
lution of more than 3 years, and with a constellation of
al revealed that among injuries associated with Crossfit, the main pathophysiological mechanisms hypotheses
clinical presentations that may have made early diagnosis
mild/moderate ones represented 89.1% of the cases, are: ischemic phenomena; development of autoimmu-
difficulties. POEMS Syndrome should be analyzed as a
while severe/very severe injuries 10.9% and injuries that nity; and the neuropathy of the critically ill patient. Thus,
differential diagnosis of several diseases, such as polyneu-
required surgical treatment represented 2.6%?. Humeral the development of neuropathy as a symptom of COVID-
ropathies, osteosclerotic lesions, plasma cell dyscrasias,
stress fractures have been described in activities with 19 is reinforced by the temporal relationship with the
neoplasms, infections and vasculitis. Final Comments:
excessive use of the upper extremities and weight-lifting?, onset of the disease and by the absence of other factors
The case in question portrays that the identification of
aspects present in Crossfit practices. Since there is a close that could predispose to this condition, such as diabetes
the disease can be a challenge, but a good anamnesis and
anatomical relationship between the radial nerve and the mellitus or Guillain-Barré syndrome. Final Comments:
physical examination followed by appropriate tests can
humerus and the fact that it promotes motor innervation A rare case of neurological manifestation of COVID-19
distinguish this Syndrome from its differential diagnoses.
of the triceps and wrist, with wrist drop being the hallmark was presented here, which differs from the pattern of
of a radial nerve injury?, the context and consequences of neuropathies caused by other viruses, which usually have
the patient’s injury significantly reflectthe findings found Neuropatias Periféricas a distal polyneuropathy pattern with sensory predomi-
in literature. Final Comments: Although Crossfit’s rising nance. Thus, it is important for health care professionals
popularity demonstrates an interest in physical exercise, to be alert for manifestations similar to those described
its practice without guidance can result in serious harm to in this case, as well as other possible neurological man-
the body. The episode described brought consequences ifestations caused by SARS-COV-2. Finally, treatment
that affect the patient’s life as a whole due to the current with pulse therapy of methylprednisolone, pregabalin,
coexistence with a neuromotor dysfunction of indefinite and physical therapy showed some degree of efficacy in
duration, shining a light on the importance of discussions improving the condition.
about the safety of the practice.
Neuropatias Periféricas
Neuropatias Periféricas

185
XXX Congresso Brasileiro de Neurologia

TL 1105224 TL 1105255 TL 1105277

INTERMEDIATE CHARCOT-MARIE-TOOTH ASSOCIATION OF TWO DISEASES AND CHARCOT-MARIE-TOOTH TYPE 1A AND


DISEASE TYPE C: A CASE PRESENTATION THEIR CLINICAL MANIFESTATIONS: POEMS SYNDROME: WHEN TO INVESTIGATE
CHARCOT-MARIE-TOOTH AND HEREDITARY ACQUIRED CAUSES IN HEREDITARY
Tereza Brenda Clementino de Freitas, Vinícius de
Noronha Barboza, Maria Clara Barbosa de Oliveira, TRANSTHYRETIN AMYLOIDOSIS NEUROPATHIES
Leila Gabriele Nunes Silva, Murilo Tomaz Rocha, ligia rocha andrade, Salim Lazaro Balassiano, José Rosemberg Costa Lima Filho, Trajano Aguiar
Vítor Silveira Reis Canêdo, Eloisa Alves Viana, Eduardo Barbosa, Eduardo Davidovich, Marcia Pires Gonçalves, João Marcus de Lima Brito Alves,
Gabriel Maciel Morais, Samila Marissa Pinheiro rodrigues jardim, Izabela Rodrigues Pitta, Ana Túlio Maranhão Neto, Giulia Maria Ximenes Verdi
Gomes Siquara, Clarissa Neves Spitz, Larissa Bittencourt Pinto, Caroline Lavgne Moreira, Camila Derminio
Universidade Federal Rural do Semi-Árido. Mossoró Carvalho, Robson Vital Donadel, Rodrigo Siqueira Soares Frezatti, Pedro
RN, Brazil José Tomaselli, Wilson Marques Junior
Hospital Universitário Pedro Ernesto. Rio de Janeiro
terezabrenda13@gmail.com RJ, Brazil Faculdade de Medicina de Ribeirão Preto da
Universidade de São Paulo. Ribeirão Preto SP, Brazil
ligiaandradee@gmail.com
Case Presentation: A 31-year-old male with a history trajanoapg@gmail.com
of progressive muscle weakness in four limbs for more Case Presentation: A 41-year-old man presented a his-
than 20 years. He has 2 sisters and 1 maternal uncle tory of difficulty walking since he was 5 years old, with Case Presentation: The proband was diagnosed at age 40
with a similar condition. He denied dysphagia, respira- stumbling and frequent falls. The condition remained with CMT1A. Her symptoms were mild and slowly pro-
tory complaints, or continuous use of medications. On stable until 5 years ago, when he noticed a progressive gressive, as expected for most CMT1A. At age of 56, her
examination, muscle strength was assessed in proximal worsening of gait, with muscle weakness and atrophy in walk deteriorated fast, she started with difficulty to climb
and distal grade IV in all four limbs and deep tendon the upper and lower limbs. On neurological examination, stairs and frequent falls, associated with positive sensory
reflexes were hypoactive globally. Artresthesic and vibra- he had decreased thermal and pain sensitivity in the lower symptoms. Additionally, she lost about 17 kg in 8 months.
tory sensitivities were reduced in the lower limbs, while limbs and hands, in addition to decreased vibratory sen- On examination, first came to our attention the changes in
the superficial was reduced in the four limbs at the 10 Hz sitivity in the lower limbs He had grade 3 hand strength her skin color. She also had hepatosplenomegaly, lymph
monofilament test. He had Gowers maneuver and gait in the lower limbs (MRC scale) and generalized areflexia. adenomegaly, papilledema, and generalized edema. NCS
with weakness of the gluteal muscles. There was no calf In addition, the patient had signs of dysautonomia, such revealed a marked worsening in the velocities. A mono-
pseudohypertrophy, nervous hypertrophy or myotonia. as erectile dysfunction, orthostatic intolerance, and pal- clonal IgA lambda peak wasdetected. Lytic lesions were
Electroneuromyography (ENMG) revealed a moderate pitations.Electroneuromyography revealed severe sen- found, and bone marrow biopsy revealed 25% monoclonal
reduction in motor and sensory conduction velocities sorimotor polyneuropathy. Genetic testing revealed two plasma cells. Based on clinical and laboratory findings, a
in the four limbs, with F waves abolished in ulnar and genetic mutations, PMP22 causing Charcot-Marie-Tooth diagnosis of POEMS syndrome was made. Systemic che-
tibial nerves. In electromyography, voluntary contrac- disease and Val142Ile, a gene responsible for hereditary motherapy followed by autologous bone marrow trans-
tion showed signs of chronic reinnervation with moder- transthyretin amyloidosis. Discussion: Charcot-Marie- plantation was performed. She evolved with dramatic
ate rarefaction of motor units in the proximal muscles of Tooth disease is a group of hereditary diseases that man- improvement of her motor and sensory symptoms. In the
upper limbs, and marked rarefaction in distal muscles ifests itself in adolescence or early adulthood, with loss setting of an inherited neuropathy of slow progression, a
of upper limbs and in proximal and distal muscles of of sensitivity, weakness, deformity and muscle atrophy sudden clinical decline and a rapid progression, should
lower limbs, describing a primarily demyelinating, sen- mainly in the lower limbs, with dysautonomic manifesta- raise attention for an associated condition. In the case we
sory-motor, symmetrical, chronic polyneuropathy, with tions not being common. Hereditary amyloidosis courses present, the systemic manifestations and the laboratory
proximal and distal involvement, worse in the lower with progressive polyneuropathy, often associated with investigations permitted a fast diagnosis and treatment
limbs. Vitamin B12, folic acid, infectious disease serology, dysautonomia, also causing multisystem symptoms such of POEMS syndrome. Significant changes in the natural
metabolic panel, aldolase, ANA and anti-acetylcholine as gastrointestinal impairment, cardiomyopathy, nephrop- history of an inherited neuropathy should be considered
tests were normal. CPK was increased (990U/L). Genetic athy, or ocular deposition. The genetic test provided the as a red flag for an associated condition CMT1A is the
testing confirmed the diagnosis of intermediate type C opportunity to diagnose two genes that cause peripheral commonest type of hereditary neuropathy, presenting
Charcot-Marie-Tooth Disease (CMT) by mutation in the neuropathy, with hereditary amyloidosis being a poten- as a slowly progressive length-dependent sensory and
PLEKHG5 gene. Discussion: CMT is a group of genetic tially treatable disease depending on the stage. As the motor neuropathy, areflexia, pes cavus and uniform
diseases that present as chronic and progressive motor patient was still able to walk with bilateral support, pati- reduction of NCV. POEMS is a paraneoplastic disorder
and sensory neuropathy. The CMT1 and CMT2 subtypes siran was indicated and he is awaiting treatment Final of plasma cells frequently associated to a sensory and
are the most frequent and represent the demyelinating Comments: it is important to be aware to atypical signs motor demyelinating neuropathy. Distinction of both
and axonal forms, respectively. The most common clini- of a disease looking for other diagnostic alternatives or diseases is important, as early treatment significantly
cal presentation is distal weakness, atrophy, and sensory even concomitant diseases as was this case. changes POEMS prognosis.
loss in a boot-and-glove pattern. In the present case, the
patient has proximal weakness and intermediate motor
nerve conduction velocity. The genetic test allowed the Neuropatias Periféricas Neuropatias Periféricas
diagnosis of the intermediate type C form, an uncommon
subtype characterized by mixed, axonal and demyelin-
ating involvement. Final Comments: CMT is described
as the most common hereditary neuromuscular disease,
and the intermediate type C form, a rare variant of the
disease, was identified in this report. In many cases, the
diagnosis still focuses on ENMG, but genetic testing is
critical for confirmation.

Neuropatias Periféricas

186
XXX Congresso Brasileiro de Neurologia

TL 1105296 TL 1105314 TL 1105326

GASTROINTESTINAL DYSMOTILITY AS CANVAS: EXPANDING THE MULTIPLE MONONEURITIS SECONDARY TO


THE LEADING COMPLAINTS IN SENSORY DIFFERENTIAL DIAGNOSIS OF SENSORY VASCULITIS AS A DIFFERENTIAL DIAGNOSIS
NEURONOPATHY PATIENTS POLYNEUROPATHIES/NEURONOPATHIES: OF THE GUILLAIN-BARRÉ SYNDROME
POSITIVE SENSORY MANIFESTATIONS AS
Rafaella do Rosario Tacla, Gustavo Manginelli Rodrigo Oliveira Arakaki, Rebeca Montenegro
Lamas, Thomas Vieira de Paula, Lucas de Melo THE MAIN COMPLAINT Lacerda, Maria Eduarda Almeida Cavalcanti, Maria
Teixeira Branco, Alberto Rolim Muro Martinez, Juliana Bruneli Secchin Algemiro, Valeska Julio Vitória Menezes Coutinho, Fernanda Thaysa Avelino
Marcondes Cavalcante França Jr Forza, Grazieli Canal, Kelmer Mozer Moro, Manoella Santos

Universidade Estadual de Campinas. Campinas SP, Guerra Albuquerque Bueno, Waldemar Carlos Centro Universitário Tiradentes. Maceió AL, Brazil
Brazil Barros Algemiro, Pedro José Tomaselli, Wilson
Marques Júnior rodrigooarakaki@hotmail.com
taclarafaella@gmail.com
Faculdade Brasileira de Cachoeiro, Instituto de Case Presentation: A 46-year-old female patient with a
Case Presentation: A – A 43-year-old male patient Neurologia, Neurocirurgia e Comportamento, history of burning pain and paresthesia on the left foot and
reported a 2-month history of paresthesia on calves. Hospital Evangélico de Cachoeiro de Itapemirim. progressing to the right foot. Subsequently, the worsening
Examination demonstrated asymmetric loss of vibra- Cachoeiro de Itapemirim ES, Brazil of sensory complaints, besides motor involvement, with
tion and position sense (including pseudoathetosis), Hospital Evangélico de Cachoeiro de Itapemirim. ascending progression of symptoms and involvement
Romberg’s sign, diffuse hyporeflexia, and tabetic gait. Cachoeiro de Itapemirim ES, Brazil of the upper limbs. In the physical exam, edema and
The patient developed constipation followed by acute Faculdade Brasileira de Cachoeiro. Cachoeiro de ecchymosis on the lower limbs; hypoperfused podotac-
abdominal pain. Intestinal pseudo-obstruction was diag- Itapemirim ES, Brazil tiles; predominantly distal flaccid tetraparesis, with an
nosed, and an exploratory laparotomy was performed but Faculdade de Medicina de Ribeirão Preto – asymmetrical pattern; normoactive deep tendinopathic
no mechanical obstruction was found. B – A 58-year-old Universidade de São Paulo. Ribeirão Preto SP, Brazil reflexes, except for abolished right Achilles; and apales-
woman reported pain and paresthesia in her right hand Instituto de Neurologia, Neurocirurgia e thesia on the right podotactiles, suggesting the hypothesis
initiated 8 years before, with gait imbalance. Neurological Comportamento. Cachoeiro de Itapemirim ES, Brazil of Guillain-Barré Syndrome and was maid guided pulse
evaluation showed diffuse loss of position and vibration vforzaj@gmail.com therapy with immunoglobulin without clinical improve-
sense, Romberg’s sign, and global areflexia. She also ment. In the investigation, serologies were negative, lab-
reported urinary incontinence and severe constipation Case Presentation: A 38 year male patient started with oratory evaluation of suspected vasculitis and connec-
with onset in the previous 4 years. HTLV-1 serology was severe paresthesia in the feet that interfered with sleep tive tissue was reagent for rheumatoid factor, ANA, and
positive. C – A 53-year-old female patient was admitted and daily activities. Over the years, there was a progres- lupus anticoagulant, polyclonal gammopathy detected
for investigation of dysphagia associated with post-meal sive worsening, with involvement of the lower limbs up by serum protein electrophoresis. CSF did not present
vomiting and paresthesia in the upper limbs for the past 6 to the knees, and more recently, after 6 years of evolu- alterations. Electroneuromyography disclosed severe
months. Neurological examination showed pseudoathe- tion, of the hands and forearms. There is also a report asymmetrical sensory-motor axonal neuropathy and signs
tosis and sensory ataxia in the upper limbs, tactile and of chronic cough. This is a previously healthy patient, of denervation. Lumbar spine magnetic resonance didn’t
painful hypoesthesia in the four limbs, diffuse hypopales- that also complained of reflux and rhinitis and no other present alterations. Diagnosis of multiple mononeuritis
thesia, and diffuse hyporeflexia. A diagnosis of achalasia comorbidities. No family history of peripheral neurop- secondary to systemic vasculitis was suspected, and
was made and was surgically corrected. NCS and EMG athy. On neurological examination, the patient had pulse therapy with methylprednisolone was initiated,
of all three patients demonstrated a severe reduction in preserved muscle strength, decreased osteotendinous with an improvement of peripheral nervous symptoms.
SNAPs and preserved motor conduction, compatible reflexes globally, tactile, thermal and painful hypoes- The patient evolved during Hospitalization with cardiac
with the SN diagnosis. Discussion: The frequency and thesia up to the bilateral ankles and distal hypopales- and central nervous system involvement, and died due to
pattern of dysautonomic features in SN are still poorly thesia. Electroneuromyography revealed a length-de- the severity of the disease. Discussion: The mononeuritis
understood, despite being remarkably present in clinical pendent sensory neuropathy/neuronopathy. The patient multiplex clinical presentation is linked to progressive
practice. A study performed on patients with non-para- was extensively investigated and, due to the severity of motor and sensory deficits through the distribution of
neoplastic SN demonstrated the involvement of various his symptoms, he repeatedly sought different health specific peripheral nerves, with irregular and asymmet-
subdomains of the autonomic nervous system (ANS), services. Autoimmune, infectious, metabolic diseases rical characteristics. Ordinarily, it’s caused by vasculitis,
with about half of the patients presenting orthostatic and research for amyloidosis, which were normal, were due to an inflammatory process that results in multifo-
hypotension and three-quarters with cardiovascular excluded. The exome showed no pathogenic variants. The cal ischemia or peripheral nerve infarction. It presents a
autonomic neuropathy. From the 12 cases of intestinal CANVAS search revealed a bilateral AAGGG expansion predilection for the extremities of lower limbs and has
pseudo-obstruction associated with SN reported in lit- at the RFC1 gene. The propaedeutics of vestibular tests acute and subacute rhythms. The acute mononeuritis
erature, only 3 were idiopathic. Autopsy studies of the and brain MRI showed no alterations. Discussion: The multiplex being similar to the Guillain-Barré Syndrome.
patients revealed severe damage of myenteric plexus. The typical cerebellar ataxia, neuropathy and vestibular are- The early diagnosis and proper treatment can prevent
patients discussed here differ from the other reports since flexia syndrome (CANVAS) is a progressive neurologic comorbidities and reduce mortality, namely to reduce
they are non-paraneoplastic forms of SN. Furthermore, the disease characterized by imbalance, sensory neurop- the damage and consequently improve the quality of life.
association between SN and achalasia is also quite rare in athy and, occasionally, chronic cough and autonomic Final Comments: Guillain-Barré has a clinical presenta-
the literature, with few cases reported.Final Comments: dysfunction. Its etiopathogenesis is associated with a tion similar to acute-onset multiple mononeuritis. Thus,
Gastrointestinal dysautonomia leading to dysmotility is biallelic (AAGGG) expansion in an intronic region of the it is important to evaluate the differential diagnoses of the
a substantial burden to patients with SN. Understanding RFC gene. Recently, the phenotypic spectrum associated cases with acute evolution which can have high mortality
the involvement of the ANS in SN is of special interest with this mutation has been expanded and more limited if there is delay in diagnosis and treatment.
both for earlier diagnosis of atypical presentations and phenotypes involving predominantly or exclusively one of
for improvement in quality of life. the systems involved in balance control were described.
Neuropatias Periféricas
Our patient complained essentially of a neuropathy and
retrospectively we identified cough. As that pathogenic
Neuropatias Periféricas
RFC1 AAGGG repeat expansions cannot be routinely
detected by sequence-based multigene panels or exome
sequencing, we need to be aware for this condition to
order the appropriate genetic test. Final Comments:
Sensory neuropathy/neuronopathy is a challenge in clin-
ical practice, especially when the most common acquired
diseases are ruled out. Our case shows that incomplete
and phenotypes of CANVAS syndrome should be con-
sidered in this scenario.

Neuropatias Periféricas

187
XXX Congresso Brasileiro de Neurologia

TL 1105348 TL 1105423 TL 1105432

WILSON’S DISEASE WITH PERIPHERAL EXPANDING THE DIFFERENTIAL DIAGNOSIS NEUROPATIA COMPRESSIVA DO NERVO
NERVOUS SYSTEM INVOLVEMENT: ATYPICAL OF POLYNEUROPATHIES: AN ATYPICAL RADIAL POR PRÓTESE DE SILICONE
PRESENTATION OF A COPPER DEPOSIT MANIFESTATION AFTER GASTRIC BYPASS
Silvya Nery Bernardino, Isabel Nery Bernardino de
DISEASE Souza, Regina Nery Bernardino de Souza, Paulo
Rairis Barbosa Nascimento, Paulo Filho Soares
Rairis Barbosa Nascimento, Paulo Filho Soares Marcelino, Lucas Samuel da Silva Araújo, Raimundo Ricardo Andrade de Medeiros, Haldson Cesar
Marcelino, Lucas Samuel da Silva Araújo, Raimundo Nonato Marques Filho, Ademir Aragão Moura Barbosa Neto, Kalil Ibrahim Mansur, Tainá Montini
Nonato Marques Filho, Ademir Aragão Moura Zampolli, Fernando Henrique Morais de Souza
Hospital Universitário da Universidade Federal do
Hospital Universitário da Universidade Federal do Piauí. Teresina PI, Brazil Hospital Getulio Vargas. Recife PE, Brazil
Piauí. Teresina PI, Brazil IAMSPE. São Paulo SP, Brazil Universidade de Pernambuco. Recife PE, Brazil
IAMSPE, São Paulo SP, Brazil Universidade Federal do Piauí. Teresina PI, Brazil Hospital da Restauração. Recife PE, Brazil
Universidade Federal do Piauí. Teresina PI, Brazil s-nery@hotmail.com
marquesnonato97@gmail.com
marquesnonato97@gmail.com
Case Presentation: Woman, 26 years old, started to Case Presentation: MFV, 26 years old, female, past bar-
Case Presentation: Male, 21 years old, known to have experience several episodes of nausea and vomiting 15 iatric surgery on 01/2010, current plastic surgery for
Wilson’s Disease for 1 year. Two months after admission, days after performing a Roux-en-Y gastric bypass, due to placement of silicone prostheses (breasts, gluteus, calves,
he started to lose strength in the lower limbs after starting grade III obesity. During this period, she did not ingest arms: biceps and triceps) on 10/2010. The patient evolved
treatment with D-penicillamine. On examination of the prescribed vitamin supplementation. After two months, with loss of motor strength in left wrist extension/left wrist
lower limbs, he presented proximal grade IV and distal she developed weakness of the lower limbs, with difficulty drop (0/5 strength) with preservation of elbow extension.
grade II strength, in addition to areflexia. He also had walking without assistance. She was admitted to the neu- An MRI was performed, identifying the well-positioned
hypopalesthesia and hypoesthesia distal. Examination rology service with symmetrical grade III strength in the prostheses, except for one in the left arm, which main-
of upper limb motricity, cranial nerves and coordination lower limbs, in addition to burning-type paresthesias in tains close contact with the radial nerve between the
showed no changes. The slit lamp examination showed the distal extremities of the four limbs. Complementary triceps heads. Furthermore an electroneuromyography
the presence of Kayser-Fleischer rings and the cerebro- investigation of cerebrospinal fluid, serology and vitamin on 09/01/2010 which showed a severe, but partial axo-
spinal fluid did not show protein-cytological dissociation. B12 were normal. Homocysteine levels were elevated. nal mononeuropathy of the left radial nerve in the arm
Electroneuromyography showed marked distal axonal The neurophysiological study showed polyneuropathy (axonotmesis). She underwent surgery to remove the
polyneuropathy, predominantly motor, and a diagnostic with an accentuated sensory and moderate motor axo- prostheses on 11/2010, in which the anatomical integ-
hypothesis of a polyneuropathy of metabolic/toxic cause nal pattern in the lower limbs. A presumptive diagnosis rity of the nerve was identified with a weak response to
was raised due to the use of medication. The treatment of deficiency polyneuropathy was performed and vitamin intraoperative electrical stimulation. There was full func-
instituted was the exchange of the copper chelator in B complex and folic acid replacement was performed. tional recovery after 1 year of rehabilitation. Discussion:
addition to symptomatic medications for peripheral The patient evolved with considerable improvement, Silicone implants have been used in Medicine for over
polyneuropathy, resulting in improved motor strength. walking without assistance after 2 months. Discussion: 60 years. Historically, interest in aesthetic procedures to
Discussion: Wilson’s disease is an autosomal reces- Bariatric procedures are an alternative to the treatment alter body contour has increased greatly during the 1980s
sive condition caused by a mutation in the ATP7B gene, of morbid obesity, offering benefits in relation to weight due to the popularization of body building as a sport and
responsible for copper metabolism. Its global prevalence reduction and management of associated comorbidities. the fitness culture¹. Since the beginning of the century,
is estimated at 1/10000 to 1/30000. About ? of patients However, they can contribute to the evolution with import- due to the increasing accessibility of information about
have neurological manifestations, which are mainly rep- ant neurological complications, due to the reduction of the procedures a new rising wave of demand for silicone
resented by movement disorders, such as dystonic, ataxic the absorption surface, the decrease in the production of implants for body contouring has been installed¹. In par-
and parkinsonian syndromes, with peripheral nerve intrinsic factor and gastric juice by the parietal cells, in ticular, prostheses for previously unexplored areas are
involvement being a rare event. The disease associated addition to the possibility of dietary restriction associated gaining more and more space, as is the case of implants
with polyneuropathy is generally characterized by distal with events of vomiting in the postoperative period. This to increase the biceps and triceps. Despite the relative
and symmetrical sensory and/or motor involvement. leads to vitamin and mineral deficiencies, such as the scarcity of data and publications?, the most expected com-
Furthermore, drugs such as D-penicillamine may be commonly described vitamins B1, B6, B9, B12, A, D, E, plications in upper limb implants include local infection,
associated with polyneuropathies, inferring a toxic cause folate and copper. Neurological complications may arise seroma, implant extrusion, asymmetry, compartment
for this condition in the context of Wilson’s disease. The after 3 to 20 months of the procedure and involve nerves, syndrome, muscle and nerve damage, with neuropraxia
patient under study, despite the diagnosis of Wilson’s dis- spinal cord and brain, as in polyneuropathies, myelop- being the most common immediate complication¹³. In
ease 1 year ago, presented symptoms after the introduc- athies and cognitive syndrome. Treatment consists of agreement with the reports in the literature, the present
tion of D-penicillamine therapy. It is not yet possible to vitamin replacement, with a strong role in remyelination case presented neuropathy as a result of the implant per-
establish a precise cause and effect relationship with the and brain glucose metabolism, which may contribute to formed in the posterior region of the arm. The triceps is
initiation of this drug, but this highlights the importance the gradual improvement or clinical stabilization of the innervated by the radial nerve. Radial nerve damage can
of new studies aimed at evaluating this condition. Final patient. Final Comments: Polyneuropathy is a possible occur either from intraoperative damage during dissec-
Comments: In the context of symptoms compatible with neurological complication of bariatric procedures. The tion of the muscle or from compression by positioning
peripheral neuropathy in patients with Wilson’s disease, urgent approach with correction of nutritional disorders the prosthesis. Final Comments: Currently available data
it is important to consider storage disease itself as etiol- is mandatory to avoid the irreversibility of the condition. on complication rates in cosmetic procedures for upper
ogies, as well as the use of copper chelating medications limb implants are scarce and in small samples, ranging
such as D-penicillamine. from 26.7%² to 47.6%? – the latter encompassing only
Neuropatias Periféricas biceps implants. Further studies about complications of
implants are necessary.
Neuropatias Periféricas
Neuropatias Periféricas

188
XXX Congresso Brasileiro de Neurologia

TL 1105475 TL 1105516 TL 1105535

MULTIPLE MONONEURITIS SECONDARY TO LATE-ONSET CHARCOT-MARIE-TOOTH PIDC CASE AFTER VACCINATION FOR
RHEUMATOID VASCULITIS DISEASE WITH LEUKOENCEPHALOPATHY COVID-19 IN A PATIENT FOLLOWED UP AT
DUE TO MFN2 GENE VARIANT HOSPITAL UNIVERSITÁRIO GETÚLIO VARGAS
Rebeca Montenegro Lacerda, Rodrigo Oliveira
Arakaki, Fernanda Thaysa Avelino Santos AND AMBULATÓRIO ARAÚJO LIMA
Ana Carolina dos Santos Jorge, Wladimir Bocca
Vieira de Rezende Pinto, Glenda Barbosa Barros, Luiziane Maria Silva Alves, Ingrid Demosthenes
Centro Universitário Tiradentes. Maceió AL, Brazil
Paulo de Lima Serrano, Igor Braga Farias, Bruno Wanzileu, Paulo Hermes Lira Amaral, Leonardo
rebecalacerdaaa@hotmail.com Mattos Lombardi Badia, Marcio Luiz Escorcio Rodrigues Leopoldo Menezes, Wesley Lopes
Bezerra, Paulo Victor Sggobi de Souza, Acary Souza Silva, Flavia Costa Mourão, Camille Albuquerque
Case Presentation: A 60-year-old male patient, diabetic, Bulle Oliveira Rodrigues Chirano, Maria Lourdes Souza Galvão,
hypertensive and rheumatoid arthritis without treatment, Nise Alessandra Carvalho Sousa
Universidade Federal de São Paulo. São Paulo SP,
started about 2 months ago with multiple cutaneous
Brazil Hospital Universitário Getúlio Vargas. Manaus AM,
lesions on lower members without pruritic purpuric
appearance with dissemination from upper limbs and Brazil
ana_carolinajorge@hotmail.com
face. Beside that, the patient presents pain and pares- ingridwanzileu85@gmail.com
thesia on the plantar region. In the physical exam it was Case Presentation: A 73-year-old Austrian man presented
observed: ulnar deviation of the fingers on symmetrical with a long-standing clinical course of 20 years of slowly Case Presentation: Vaccines against COVID-19 have
hands with bone prominences on metacarpophalangeal progressive unsteady gait, imbalance, tingling in the legs mainly been associated with adverse events of little clin-
joints. Neurological examination: oriented, tactile and and hands, and complained about difficulty in speech ical importance. The profile of these side effects is still
painful hypoesthesia on the left lower limb, strength grade and hand tremors for 5 years and the need of unilateral undetermined and more serious adverse events have
2 on the left lower limb and grade 3 on the right lower limb support for ambulation for 2 years. Medical history dis- been reported in a minority of cases. One such event is
and upper limbs with global hyporeflexia. The serologies closed: bilateral cataracts and glaucoma surgery; chronic immune-mediated inflammatory polyneuropathy. There
for HIV, hepatitis B and C were negative with evidence of migraine with aura; left sinusectomy at age 35; chronic are some reports in the literature of cases of acute-onset
thrombocytosis, reactive rheumatoid factor (1280 UI), allergic rhinitis; three episodes of motor aphasia lasting inflammatory polyradiculoneuropathy in patients after
no-reactive FAN, decreased C4 dosage and normal C3 about 30 minutes; generalized tonic-clonic seizures at 46 COVID-19 infection and vaccination. Although little
dosage. The electroneuromyography showed a peripheral and 56 years; progressive hearing loss since age 58 years; described in the literature, CIDP has already been reported
sensory-motor involvement, of axonal nature, asymmet- chronic diarrhea; moderate hepatic steatosis. Family his- in the post-vaccination period for influenza, tetanus and
rical involvement, subacute, acute grade, compatible with tory was unremarkable. Examination disclosed diffuse other common vaccines. We describe the case of a 52-year-
multiple mononeuritis. A diagnosis of rheumatoid arthri- palatal tremor, thoracic hyperkyphotic posture, slight old patient, who performed the first dose of vaccination
tis complicated with rheumatoid vasculitis was made. saccades decomposition at horizontal and conjugate for Covid-19 (Astrazeneca) on 06/12/2021 and, on June 16,
Pulse therapy was initiated with methylprednisolone for gaze, reduced deep tendon reflexes, flaccid quadripa- 2021, started tingling in his hands and feet. The following
5 days and associated with gabapentin for neuropathic resis (predominantly distal); hypopalesthesia, painful day, he developed weakness in the lower limbs and, one
pain treatment. Patient evolved with improvement of skin hypoesthesia, and distal-proximal thermal gradient in day later, he was no longer able to walk. He was taken to
lesions, strength of the lower limbs, plantar and hands the lower limbs. Electroneuromyography disclosed signs the emergency room, submitted to Hospital admission on
paresthesia after the pulse therapy. Opted for Hospital of severe axonal sensorimotor polyneuropathy, pre- the third day of illness; during this Hospitalization, ther-
discharge and maintenance treatment with rituximab. dominantly sensory, distal. Brain MR imaging disclosed apy with human immunoglobulin (1.65 mg/kg dose) was
Discussion: The rheumatoid arthritis is a chronic and diffuse leukoencephalopathy. Muscle biopsy showed performed, with partial improvement of symptoms. After
systemic inflammatory disease progressive that involves chronic nonspecific neurogenic amyotrophy (10% of COX- three weeks of Hospital discharge, he developed dysphagia
joints, but also can show extra-articular manifestations, negative muscle fibers). Cerebrospinal fluid analysis was and respiratory distress, being Hospitalized again in the
such as vasculitis. Rheumatoid vasculitis is a rare and unremarkable. Audiometry showed moderate bilateral emergency room on 08/03/2021. He was transferred to
serious complication that must have an early diagnosis, sensorineural hearing loss. Genetic testing for Fabry’s HUGV for follow-up with Neurology on 08/07/2021. On
given the great interference in the evolution, treatment disease was negative. NGS-based multigene panel for 08/13/2021, an electroneuromyography of the four limbs
and prognosis of the base disease. The peripheral nervous neuropathies disclosed the definitely pathogenic missense was performed, which showed sensory and motor neu-
system is affected in 50% of the cases. The worst progno- variant c.2119C>T (p.Arg707Trp) in single heterozygosity ropathy, axonal, symmetrical, with denervation of very
sis of multiple mononeuritis occur with acute installa- in the MFN2 gene. Discussion: MFN2 (mitofusin 2) gene serious intensity and intense signs of activity. During this
tion, axonal degeneration and presence of extensive and (1p36.22) is associated with mediating mitochondrial Hospitalization, he performed seven sessions of plasma-
severe angiitis. Final Comments: Rheumatoid vasculitis fusion and fission. MFN2 variants are the most common pheresis, observing significant clinical improvement.
is a complication rare and severe of rheumatoid arthri- cause of axonal CMT, generally presenting with optic atro- Two weeks after Hospital discharge, the weakness wors-
tis, that can be associated with others comorbidities, in phy, hearing loss, vocal cord paralysis, severe early-onset ened, initially in the upper limbs and later in the lower
especial the acute multiple mononeuritis that needs an axonal neuropathy (SEOAN) and even spasticity. Most limbs, and he was Hospitalized again on 10/14/2021 for
aggressive early treatment for the reduction of the mor- cases have onset during the infancy, childhood, adoles- clinical compensation, when immunoglobulin therapy
bidity and mortality. cence, or early adulthood, however our patient presented was performed at a dose of 2 g/kg and this therapy was
with late-onset compromise, as well as with palatal tremor, maintained. monthly for six months, with significant
kyphosis, seizures, and leukoencephalopathy, which are improvement. Due to the clinical characteristics, ruling
Neuropatias Periféricas not the most typical signs associated with MFN2 patho- out other diagnoses and the form of disease progression,
genic variants. Final Comments: Genetic screening for the diagnostic hypothesis of CIDP was proposed. In view of
MFN2 variants in late-onset neuropathies with complex the above, it can be seen that there is temporality between
clinical pictures is important in the diagnostic work-up, vaccination and the onset of symptoms presented by the
including cases with marked leukoencephalopathy. patient. The adverse effects of vaccines for Covid-19 are
still being known, so it is essential to publish this report
as a form of contribution to the scientific community.
Neuropatias Periféricas

Neuropatias Periféricas

189
XXX Congresso Brasileiro de Neurologia

TL 1105553 TL 1105560 TL 1105717

CASE PRESENTATION OF NON- ADULT-ONSET CHARCOT-MARIE-TOOTH ACUTE AUTONOMIC NEUROPATHY POST


SYSTEMIC VASCULITIC NEUROPATHY DISEASE DUE TO SAMD9L GENE VARIANT COVID19 VACCINE: A CASE PRESENTATION
WITH IMPROVEMENT AFTER THERAPY
Ana Carolina dos Santos Jorge, Wladimir Bocca Eder Leandro da Silva Dantas, João Marcus de Lima
WITH METHYLPREDNISOLONE AND Vieira de Rezende, Vinícius Lopes Braga, Paulo Brito Alves, Trajano Gonçalves, Jose Rosemberg
CYCLOPHOSPHAMIDE de Lima Serrano, Igor Braga Farias, Bruno Mattos Lima Filho, Túlio Maranhão Neto, Giulia Maria
Lombardi Badia, Fabricio Porto do Nascimento, Ximenes Verdi, Caroline Lavigne Moreira, Millene
Luiziane Maria Silva Alves, Ingrid Demosthenes
Marcio Luiz Escorcio Bezerra, Paulo Victor Sggobi de Rodrigues Camilo, Pedro José Tomaselli, Wilson
Wanzileu, Paulo Hermes Lima Amaral, Leonardo
Souza, Acary Souza Bulle Oliveira Marques Junior
Rodrigues Leopoldo Menezes, Wesley Lopes
Silva, Flavia Costa Mourão, Camille Albuquerque Universidade Federal de São Paulo. São Paulo SP, Hospital das Clínicas da Faculdade de Medicina de
Rodrigues Chirano, Pablo Vinicius Silveira Feitoza, Brazil Ribeirão Preto. Ribeirão Preto SP, Brazil
Nise Alessandra Carvalho Sousa
ana_carolinajorge@hotmail.com ederleandro.sdantas@gmail.com
Hospital Universitário Getúlio Vargas. Manaus AM,
Brazil Case Presentation: A 49-year-old brazilian woman pre- Case Presentation: A 26-years old woman presented with
ingridwanzileu85@gmail.com sented with lower limb weakness for 8 years, associated a four-days history of recurrent postprandial vomiting,
with tingling and sensory disturbance in both hands diarrhea, headache and fainting. Her symptoms started
and feet. Medical history disclosed chronic diarrhea, within 24 hours after the second dose of COVID-19 vac-
Case Presentation: Vasculitic neuropathies are a hetero-
self-limited episodes of blurred vision and abdominal cine AstraZeneca/Oxford. Her symptoms were mild at
geneous and rare group of autoimmune disorders of the
pain mimicking appendicitis. Family history was unre- presentation, including headache, lightheadedness and
peripheral nerves. In primary vasculitic neuropathies,
markable. Her cognitive function and general develop- vomiting. Then she had a syncope while taking shower
there is no clinical evidence of involvement of other organs.
ment were normal. Examination revealed inability to and started experiencing recurrent episodes of palpi-
They usually have a multifocal distribution and develop
perform tandem gait, positive Romberg test, pes cavus, tation and dizziness. On neurological examination her
acutely or subacutely. The most commonly affected nerves
diplopia during bilateral horizontal gaze. Reduced deep gait was normal. She was able to walk on heels and toes.
are the common peroneal nerve in the leg and the ulnar
tendon reflexes in upper limbs and brisk reflexes in lower Romberg sign was absent. Coordination was normal.
nerve in the arm. Electromyography reveals a predomi-
limbs. Paraparesis (predominantly distal). Muscle biopsy There was an anisocoria and light-near reflex dissocia-
nantly axonal, asymmetric sensory and motor neuropathy
disclosed mild subsarcolemmal mitochondrial prolifer- tion. Pinprick evoked pain, tactile and thermal sensation
(multiple mononeuropathies). Immunosuppression is the
ation and nonspecific chronic neurogenic amyotrophy. was abnormal throughout, but worse on the left side.
cornerstone of disease-modifying therapy. We describe
Nerve conduction studies disclosed distal and uniform Strength was normal in all muscles. Deep tendon reflexes
the case of a 37-year-old patient with tingling paresthesia,
demyelinating sensorimotor polyneuropathy. Brain MR were normal. There were no upper motor neuron signs.
shock-like pain, and paresis in the right hand, presenting
imaging disclosed mild leukoencephalopathy, mild cer- Her blood pressure lying was 100x60 mmHg and seated,
after two weeks a similar picture in the topography of the
ebellar atrophy, and the hot-cross bun sign. Laboratory 76x50mmHg. She did not tolerate standing. Tilt test was
left foot. She is initially evaluated by orthopedics and
tests revealed normal values. Whole-exome sequenc- performed and at the 3rd minute her heart rate was 180
rheumatology for carpal tunnel syndrome. Neurological
ing disclosed a heterozygous pathogenic variant in the bpm, she had near fainting symptons, causing it to be
evaluation characterized multiple mononeuropathies. In
SAMD9L gene. Discussion: Variants in Sterile Alpha Motif interrupted. CSF was normal at the 10th day of symptoms.
a complementary investigation, asymmetric sensory and
Domain-Contain Protein 9-Like (SAMD9L) gene have been Brain MRI was unremarkable. NCS performed at days 10
motor neuropathy of axonal nature and with signs of activ-
associated with ataxia-pancytopenia syndrome (ATXPC), and 20 were within normal limits. Small-fibre evaluation
ity was confirmed, considering the possibility of vasculitic
spinocerebellar ataxia type 49, myelodysplasia and leuke- revealed decreased RR variability. QST was abnormal.
neuropathy. It was carried out an extensive investigation
mia syndrome with monosomy 7, and SAMD9L-associated Sympathetic skin response to sound stimulus was absent
of the causes of systemic vasculitis, with negative results.
autoinflammatory disease. The main clinical presentation in the upper limbs. Considering her clinical presenta-
The biopsy of the left sural nerve performed revealed a
in our case is a late-onset inherited demyelinating neurop- tion and the electrophysiological abnormalities found, a
normal histological and immunohistochemical appear-
athy, mimicking features of Charcot-Marie-Tooth (CMT) diagnosis of acute autonomic neuropathy was made. She
ance. It was decided to perform therapy with methylpred-
disease. Despite the presence of mild cerebellar atrophy, was treated with high dose of IV methylprednisolone and
nisolone and cyclophosphamide due: first, to worsening
our patient presented mainly with sensory ataxia and her IgIV with excellent response. She was discharged after 2
of sensory and motor function, and second, a sustained
other clinical features resulted from chronic neuropathic weeks with mild tachycardia. Discussion: Acute auto-
increase in inflammatory tests. Excellent response with
compromise. Final Comments: Our report provides new nomic neuropathy (AAN) is an extremely rare syndrome
this treatment has been obtained. The importance of this
evidence linking SAMD9L variants to primary peripheral and may manifest with abnormalities in cardiovascular
case presentation lies in the fact that non-systemic vascu-
nerve compromise, expanding the clinical spectrum asso- (orthostatic hypotension), gastrointestinal (anorexia or
litic neuropathy is a challenging diagnosis. Usually, it is
ciated with this gene. SAMD9L variants must be included early satiety) and urogenital systems (erectile dysfunc-
hard to perform a nerve biopsy. The adequate response
in the differential diagnosis of late-onset demyelinating tion), dry eyes and mouth, and also visual impairment
to the instituted therapy reaffirms the relevance of the
CMT phenotypes. due to disturbance of pupils constriction. AAN is often
clinical evaluation to determine the beginning of the
immune mediated. The vast majority of cases have good
therapy, despite the negativity to the performed biopsy.
prognosis. It is known mild abnormalities on autonomic
Neuropatias Periféricas system are common following COVID-19 infection. This
Neuropatias Periféricas is the second case presentation that associates COVID-19
vaccine and AAN. Final Comments: The case we present,
describing the development of acute autonomic neurop-
athy after COVID-19 vaccination and favorable response
possibly associated with IVIg and corticosteroids, supports
previous reports of this association.

Neuropatias Periféricas

190
XXX Congresso Brasileiro de Neurologia

TL 1105749 TL 1105768 TL 1105778

GUILLAIN-BARRÉ SYNDROME POST AUTOSSOMAL DOMINANT IFIH1-DISORDER EOSINOPHILIC GRANULOMATOSIS


VACCINE: A CASE PRESENTATION ASSOCIATED WITH SENSORIMOTOR WITH POLYANGIITIS AS A CAUSE OF
POLYNEUROPATHY POLYNEURRADICULOPATHY
Leonardo Rodrigues Leopoldo Menezes, Paulo
Hermes Lima Amaral, Wesley Lopes Silva, Flavia Clara Gontijo Camelo, Cristiane de Araújo Martins Matheus Gurgel Saraiva, Jeanina Cabral Dionizio,
Costa Mourão, Luiziane Maria Silva Alves, Ingrid Moreno, Alulin Tácio Quadros Monteiro Fonseca, Artur Felipe Barbosa Vasconcelos, Davi Veloso
Demosthenes Wanzileu, Camille Albuquerque Pedro Henrique Marte de Arruda Sampaio, André Guerra, Daniel Vicente de Siqueira Lima Junior,
Rodrigues Chirano, Nise Alessandra Carvalho Macedo Serafim da Silva, Marcos Vinícius Oliveira Luiza Alves Monteiro Torreão Villarim, Juliana
Sousa, Maria Lourdes Souza Galvão, Talísia Marques, Edmar Zanoteli Magalhães Leite, Rafael de Souza Andrade, Rafael
Nascimento Vianez Gonçalves Duarte Cunha, Daiane Pereira de Farias
Department of Neurology, Faculdade de Medicina
Hospital Universitário Getúlio Vargas. Manaus AM, da Universidade de São Paulo FMUSP Hospital Metropolitano Dom José Maria Pires. João
Brazil Pessoa PB, Brazil
mvom13@gmail.com
ingridwanzileu85@gmail.com matheus_gurgel_pb@hotmail.com
Case Presentation: The IFIH1 gene encodes a cytoplas-
Case Presentation: A 23 year old, male patient on March mic receptor for viral double-stranded RNA, that activates Case Presentation: Patient 57 years old, male, with a his-
6th started to feel paresthesia in both hands with later type I interferon signaling and antiviral response. Gain- tory of asthma and no other comorbidities, with a report of
progression to his feet at night. After two days, he pro- of-function mutations in IFIH1 are related to increased COVID in JUNE 2021 evolving about 2 weeks later with pal-
gressed with dysphagia and quadriparesis and worsen- and inappropriate exposure to type I interferon resulting pable purpuric lesions in the lower limbs associated with
ing of paresthesia, later being admitted in an emergency in a distinct set of inborn errors of immunity, Aicardi- initial crural sensory-motor deficit insidious and progres-
room and being transferred within 01 day to an ICU of Goutieres, and Singleton-Merten syndromes (SMS). SMS sive character. On admission, he had facial diparesis and
a tertiary Hospital. He denied any infections prior to his is a rare genetic disease characterized by calcifications in areflex quadriparesis (grade IV-). In the clinical investiga-
symptoms and said that he was vaccinated with his third the aorta and cardiac valves, delayed primary tooth erup- tion, CSF collection was performed, which showed 05 cells
dose of SARS COV 2 vaccine (Astrazeneca) within 14 days tion, dysregulated mineralization, teeth loss, osteoporosis, and 35 proteins, electroneuromyography that came with
before his symptoms. On physical examination, he pre- dysmorphisms, muscle weakness, psoriasis, and recurrent suggestive signs of acute immune-mediated inflammatory
sented flaccid quadriparesis with abolished reflexes in infections. This is the first description of polyneuropathy polyneuroradiculopathy and axonal nature (AMSAN).
lower limbs and diminished in upper limbs with facial in an autosomal dominant IFIH1-Disorder presenting with Laboratory tests showed significant eosinophilia (> 5000),
diplegia and bulbar palsy, with no sensitive impairments. incomplete Singleton-Merten syndrome.A male patient by positive rheumatoid factor and anti-DNA, P-ANCA 1/40,
Electromyogram was performed on the fourth day of the age of 2 started presenting joint retractions, hypodon- protein electrophoresis with increased gamma globulin
onset, with no significantly findings.CSF was analyzed tia, fatigue, exercise intolerance, and large joint pain. He and alpha-1-globulin. Such findings close the diagnosis
and showed albuminocytologic dissociation. MR showed had severe bone fractures following minor traumas. At the of Eosinophilic Granulomatosis with Polyangiitis (GEP).
no abnormalities. He was treated with IVIG for 05 days age of 12, he was diagnosed with psoriasis. Clinical exam- Imaging examinations showed pansinusopathy. During
and two days after the IVIG course he developed respi- ination revealed hypodontia, broad forehead, anteverted Hospitalization, he underwent immunoglobulin for 5
ratory insufficiency and disautonomia and had cardiac ears, malformation of helix and goblin, and trophic alter- days followed by pulse therapy with methylprednisolone,
respiratory arrest during rapid sequence intubation. He ation of skin. We observed distal weakness and retractions. evolving with excellent clinical response and absence of
was resurrected after eleven cycles and after two days he Lower limb deep tendon reflexes were absent and upper eosinophilia. He is discharged with azathioprine and
developed diffuse brain swelling and elevated ICP, being limb reflexes were hypoactive. There was hypoesthesia prednisone and remains asymptomatic in outpatient fol-
monitored with an intraventricular ICP device, and after in the lower limbs up to the knee and in fingertips in the low-up. Discussion: PEG, formerly called Churg-Strauss
one week he developed refractoriness to clinical treatment, upper limbs, associated with reduced vibration sense. disease, is a systemic necrotizing vasculitis of small and
being submitted to an EVD. He died two days after. The Cognition was normal. Brain MRI showed foci of calcifi- medium-sized vessels characterized by extravascular
Guillain-Barré syndrome is an acute autoimmune disorder cation in the left frontal radiated crown. Echocardiogram, granulomas, eosinophilia and eosinophilic tissue infil-
after infection or rarely to immunization in susceptible EKG, and CSF analysis were normal. Bone densitometry trate. The annual incidence is estimated at 1 to 3 cases
individuals. Autoimmune antibodies that recognize gan- revealed severe osteoporosis. Nerve conduction studies in 1000,000 people. The mean age of onset is 48 years,
gliosides are found in a large proportion of GBS patients showed a length-dependent sensorimotor polyneuropa- with a slight predilection for females. According to the
and are thought to contribute to the ultimate pathology thy. The nerve ultrasound was normal. Muscle ultrasound American College of Rheumatology criteria, of the 6 pos-
by inducing complement-mediated axonal injury and showed neurogenic changes in lower limbs sparing the sible clinical criteria (asthma, eosinophilia greater than
demyelination.1 The first case of GBS following COVID- rectus femoris, and tibialis anterior. Upper limb muscles 10%, mononeuropathy or polyneuropathy, non-fixed
19 vaccination was reported in February 2021 in the USA were normal, as were paraspinal and respiratory muscles. pulmonary infiltrate, paranasal sinuses abnormality,
in an elderly female who presented 2 weeks after the first Whole exome sequencing revealed a heterozygous muta- biopsy with extravascular eosinophils) the patient had 5,
dose of the vaccine.2 Also, it has been reported GBS syn- tion in the IFIH1 gene that was not previously described confirming a case of PEG. Final Comments: We present
drome after SARS COV2 infection, with approximately and is absent from population databases. We classified a case of a rare cause of polyneuropathy that had a good
ten days between viral infection and the development the variant presented here as likely pathogenic accord- response after adequate therapy, showing the importance
of GBS syndrome.3 One narrative review showed nine ing to the ACMG criteria. This case confirms that IFIH1 of including it among the hypotheses when the patient
articles reporting 18 cases of SARS COV2 Vaccine asso- gain-of-function mutations cause a spectrum of diseases. presents any of the suggestive symptoms for PEG, as it
ciated GBS Syndrome4. We described a male patient of The sparing of the tibialis anterior muscle is of particu- was diagnosed with early intervention, provides a sig-
23 year old who developed a flacid arreflex quadriparesis lar interest, as it is a commonly affected muscle in other nificant improvement in the patient’s quality of life and
and bulbar palsy with albuminocytologic dissociation hereditary polyneuropathies and this pattern may help in disease prognosis.
within 14 days of vaccination with Astrazeneca, who had the differential diagnosis. Finally, polyneuropathy could
refractoriness to IVIG and died after complications from play a role in patients’ phenotype.
cardiac arrest. This report is importante due to the rarity Neuropatias Periféricas
of GBS induced by vaccination.
Neuropatias Periféricas

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191
XXX Congresso Brasileiro de Neurologia

TL 1105787 TL 1105805 TL 1105824

LEFT BRACHIAL PLEXOPATHY AS A SEVERE IMMUNE NEUROPATHY WITH AN INFLAMMATORY LUMBAR


CONSEQUENCE OF THE NEUROLOGICAL RESPIRATORY DISTRESS AND LOCKED-IN RADICULOPLEXOPATHY POS SEVERE
SEQUELAE OF COVID-19 SYNDROME COVID-19 INFECTION ASSOCIATED WITH
HETEROTOPIC OSSIFICATION
Albano Mayer Junior, Renato Endler Iachinski, Thais Fresneda Felice, Ellen Silva de Carvalho, Maria
Pietra Baggio Peixoto de Oliveira, Marcia Bernardon, Clara Baseio, Carolina Lavigne Moreira, Rodrigo S Camila Pupe, Flavia Costa, Wendell Bernardes,
Vinicius Slongo Bühler, Gabriel Angelo Garute Frezatti, Andre Cleriston Jose dos Santos, Pedro Osvaldo Nascimento, Arthur Xavier
Zenatti, Lucas Victoy Guimarães Zengo Jose Tomaselli, Wilson Merques Junior
Universidade Federal Fluminense. Niterói RJ, Brazil
Centro Universitário da Fundação Assis Gurgacz HCFMRP-USP – RIBEIRAO PRETO – SP – Brazil DASA. Rio de Janeiro RJ, Brazil
FAG Hospital das Clínicas da Faculdade de Medicina de Centro Integrado de Reabilitação em Dor. Rio de
Fundação Hospitalar São Lucas FHSL Ribeirão Preto. Ribeirão Preto SP, Brazil Janeiro RJ, Brazil
albanomayer2003@hotmail.com thaisffelice@gmail.com arthurfxavier@gmail.com

Case Presentation: Male patient, 46 years old, diagnosed Case Presentation: A 58-years-old man, presented with Case Presentation: Neurological complications due to
with COVID-19, remained Hospitalized in the ICU for tingling and needles in both hands, progressed to weak- covid-19 infection have been linked to severe cases of
38 days, and after being discharged, he presented with ness. Over 5 weeks his symptoms progressed to the lower COVID-19 infection since the onset of the pandemic.
flaccid paresis in MSE, loss of strength and sensitiv- limbs, and he also complains of difficulty to go up and However, the clinical and pathophysiological character-
ity and pain. The patient underwent an electroneuro- down stairs. In a few months, he was unable to walk with- ization of peripheral nervous system involvement is still
myography (ENMG) which indicated a plexopathy in out support. On neurological examination he presented unclear. We describe a case presentation of a 57 years old
the lower left trunk and a cranial Magnetic Resonance with proximal and distal asymmetrical weakness, areflexia man, previously healthy, who presented an asymmetric
(RNM), which showed abnormalities in right semi-oval and sensory impairment distaly. CSF was normal. NCS lumbar radiculoplexitis after a severe presentation of
center. Subsequently, a plexus MRI was performed, which revealed a sensory and motor demyelinating polyradic- COVID-19 infection responsive to corticotherapy. In
demonstrated a regular diffuse thickening of the left bra- uloneuropathy. A hypothesis of CIDP was made and he December of 2020, the patient was admitted to the Hospital
chial plexus. Dorene TABS 75mg, Benerva and FST were was put on high doses of intravenous methylprednisolone with a respiratory failure related to covid-19 infection. He
prescribed. After 5 months, there was improvement in for 5 consecutive days. Despite that he rapidly deterio- was intubated and placed on ECHMO for 26 days. During
paresis, sensitivity, maintaining difficulty in moving the rated, and he was treated with IvIG with no response. He this time, the patient had 3 secondary infections, multiple
MSE. Discussion: The brachial plexus is a complex ner- evolved with respiratory insufficiency requiring invasive blood transfusions, and drug-induced hepatitis. He was
vous structure, and is formed by the roots C5, C6, C7, C8 mechanical ventilation. In addition, he had bilateral eyelid tracheostomized and in February of 2021, after sedation
and T1, and which can also receive contributions from ptosis and restriction of eye movement in all positions. A was removed, the patient was quadriplegic and arreflexia,
C4 and T2, and is a critical region of the peripheral ner- bedside test with pyridostigmine was performed, with a and the diagnosis of critical ill polyneuropathy was made.
vous system, due to the fragility of its anatomical region slight improvement in ptosis in one eye. Then, PLASMEX In the following months, the patient was under rehabili-
and its relationship with neighboring mobile structures. was done and he started to recover. Based on the poor tation, but no motor improve was noted on the left foot.
Commonly, brachial plexus injuries are divided into: response to treatment IgG-4 nodo-paranodopathy was In September 2021 came to our peripheral neuropathy
open, closed, perinatal paralysis and after radiation. considered and rituximab was done, and he is improving. center. At physical examination, the patient presented a
Closed injuries can be further classified into supra, infra Discussion: CIDP is an acquired immune inflammatory plegia on the left foot with areflexia on left Achilles ten-
and subclavicular. The symptomatology presented by the polyneuropathy characterized by a progression over at don. The nerve conduction study and the EMG showed
patient is wide, as it is closely related to the type of nerve least 2 months with a progressive or relapsing course. an important partial axonal involvement of the left tibial
that was injured, whether it is sensory, motor or mixed. If Our case had several unusual features that raised the and peroneal nerves with few functioning motor units.
it is a lesion of an exclusively motor nerve, the symptom- possibility of a different diagnosis, including onset of Partial axonal involvement of the right peroneal nerve
atology will be a paralysis of the musculature innervated weakness from upper limbs, cranial neuropathy, poor Lumbosacral MRI with neurography showed signs of
by it. If it is a lesion of an exclusively sensory nerve, the response to IVIg and methylprednisolone. The incidence inflammation in the roots of L4 to S1 and hyperintensity
symptomatology will be a loss of sensation. And if it is of ventilatory failure in CIDP is very rare (1-9%), its mech- in T2 STIR with gadolinium enhancement bilaterally, but
a lesion of a mixed nerve, the symptoms presented by anism is believed to be via phrenic nerve demyelination. more intensive on the left, with extension to left sciatic
the patient will be characteristic of sensory and motor Cranial neuropathy, ptosis and ophthalmoplegia are not nerve. A heterotopic ossification was identified between
losses. To make the diagnosis, the patient’s history and common in patients with CIDP, raising the possibility of the left gluteus minimums muscle. Heterotopic ossifi-
neurological examination must be taken into account. an overlap of myasthenic syndrome. Final Comments: cation on left iliac cortical bone related to the previous
Electroneuromyography is an essential test to confirm We identified a patient with a distinct phenotype char- Hospitalization period with edema located in the deep
the diagnosis, and imaging tests may also be requested. acterized by severe sensorimotor neuropathy presenting muscle fibers of the left gluteus minimums. Inflammatory
In general, in less severe injuries, the nerve can heal on with tetraplegia, cranial nerve involvement, respiratory edema affecting the soft tissues between the iliotibial
its own, and physical therapy or occupational therapy is failure and locked-in syndrome and poor response to tract and the greater femoral trochanter bilaterally, as
usually indicated. In more severe injuries, surgical inter- steroids and IvIG. There are some case presentation of well as the insertional portions of the gluteus minimus
vention is usually required. Final Comments: Brachial anti–pan-neurofascin-associated neuropathy with sim- and medius, being slightly more evident on the right, may
plexus injury is a common pathology that compromises ilar phenotype. Unfortunately, we were not able to test correspond to a change related to friction/pressure by
the individual in numerous spheres, such as social and specific antibodies. decubitus. Signs of neuropathy/neuritis of L4 to S1 bilat-
economic. Therefore, it must be recognized early in order erally more evident on the left. Final Comments: Severe
to reduce the risk of sequelae that this injury can cause. cases of COVID-19 infection can lead to an inflammatory
Neuropatias Periféricas presentation of the peripheral nervous system as a radic-
uloplexitis presented here.
Neuropatias Periféricas

Neuropatias Periféricas

192
XXX Congresso Brasileiro de Neurologia

TL 1105831 TL 1105850 TL 1106084

CSF PATTERN, SEVERE PROTEINORRACHI, MEDIAN NERVE ABSCESS DUE TO LEPROSY POLR3B VARIANT CAUSING AN AXONAL
IN GUILLAIN BARRÉ SYNDROME AFTER REACTION AFTER COVID-19 IMMUNIZATION: CHARCOT-MARIE-TOOTH DISEASE
SARS-COV-2 VACCINE, ASTRAZENECA – A CASE PRESENTATION
José Pedro Soares Baima, Pedro Henrique Marte
REPORT IN 3 CASES de Arruda Sampaio, Rodrigo de Holanda Mendonça,
Leonardo Peixoto Garcia, Isabella Sabião Borges,
Tatiane Arroyo Lopes Aves de Jesus, Amanda Letícia Gabriela Tomás Alves, João Augusto Nunes Vitorino, Cristiane Araújo Martins Moreno, Edmar Zanoteli
Andre, Arthur Coelho Moura Marinho, Nathalye Maria Cecília Inácio, Marcela Araújo de Oliveira
Hospital das Clínicas da Faculdade de Medicina da
Fernanda Pedroso Dircksen, Marcelo Simplicio Santana, Isadora Costa Celestino, Andrea de
Universidade de São Paulo. São Paulo SP, Brazil
Carvalho, Lorena Fernandes Kronbauer, Damacio Martino Luppi, Isabela Maria Bernardes Goulart,
Ramon Kaimen-Maciel Diogo Fernandes dos Santos jpedrobaima@gmail.com
Irmandade Santa Casa de Londrina. Londrina PR, Universidade Federal de Uberlândia. Uberlândia
Case Presentation: An 18-years-old male patient pre-
Brazil MG, Brazil
sented with a history of daily vomiting, delayed puberty,
arroyotatiane@gmail.com peixotogarcialeo@gmail.com and weakness. He complained of gastrointestinal tract
symptoms since his first year of life. Frequent falls were
Case Presentation: 1. RF, 22 years old, ten days after the Case Presentation: A 58-year-old woman, previously noted when learning to walk. At age seven, he could no
3rd dose of astrazeneca vaccine, develops bilateral par- hypertensive, diagnosed with dimorphic-virchowian longer handle small objects, and was unable to climb
esthesia in hands and feet, the following day the deficit leprosy in 2017, underwent multidrug therapy (MDT) stairs. The patient also complained of painful paresthesia.
increases to limbs. After 4 days, there was loss of strength for 12 months with multibacillary MB regimen, but had On physical examination, muscle atrophy was perceived,
in proximal limbs, hypoesthesia in the tongue and mouth to go through a new treatment cycle due to therapeutic strength in opposition had a MRC grade 4 in proximal
and dysphagia. In cerebrospinal fluid (CSF): protein 476 failure at the end of MDT. After 19 months of this sec- muscles and MRC grade 0 in distal muscles. Deep tendon
and 1 leukocyte. After 2 days, the deficit worsened with ond treatment approach, the patient presented with reflexes were absent. Pinprick and vibration were reduced
new 938 protein orrachia. 2. R, 52 years old, 1 week after fever, chills, intense arthralgia, edema, and paresthesia in upper and lower extremities. Electrodiagnostic studies
the first dose of astrazeneca, developed paresis in the right in the limbs, besides significant pain in hands and feet (EDX) revealed a severe axonal sensory-motor polyneu-
lower limb, on the 8th day he presented the same com- and cutaneous erythema nodosum leprosum. A senso- ropathy. Median and ulnar motor conductions studies
plaint in the left lower limbs, both ascending, in addition ry-motor evaluation revealed hyposthesia and paresis, were within normal range. Neuromuscular ultrasound
to bilateral upper limbs. On the 10th day, a vesical and especially in the left hand. No systemic co-infections showed neurogenic changes in distal muscles. Whole-
nasoenteral tube was needed. In lcr: 453.9 proteins and 1 were identified. However, the patient reported that she exome sequencing identified an heterozygous de novo
leukocyte. 3. FD, 56 years old, ten days after the 3rd dose received the Oxford/Covishield vaccine against COVID- mutation in POLR3B – chr12: 106.444.552 G&gt;A (p.Ar-
of astrazeneca vaccine, he develops paresthesia in the 19 10 days before the onset of the condition. Upper and g682Lys). A final diagnosis of Charcot-Marie-Tooth type
hands and feet and 1 day after the deficit ascends to the lower limb peripheral nerve ultrasonography identified 2 (axonal) was made. Discussion: Polymerase RNA 3 B
legs bilaterally. It evolves with loss of strength in feet and marked thickening of the left median nerve. Doppler anal- is the largest subunit of RNA polymerase III, an enzyme
legs, in addition to urinary retention. In lcr: 1 leukocyte, ysis demonstrated the presence of flow associated with a involved in transcription of small non-conding RNA.
306.6 proteins. All patients had MRIs of the skull and cer- small hypoechoic image measuring 0.5x0.3 centimeters Classical disease descriptions were related bi-allelic
vical spine, without changes and also without complaints near the perineurium, compatible with an abscess, in mutations causing leukodystrophy. In 2021 this gene was
of recent viral infection. In addition to an electroneuro- addition to thickening of the tibial, left fibular, and left associated with peripheral neuropathy. To our knowledge,
myography study demonstrating demyelinating impair- ulnar nerves. Discussion: This report discusses a case only nine patients presenting with this pathogenic variant
ment. They also have a severe course with the need to of a type 2 reaction with neural abscess formation in a were described worldwide (six in original publication, two
repeat human immunoglobulin monthly. Discussion: The patient being treated for dimorphic-virchowian leprosy in Japan, and one in China). There are no descriptions of
vaccine in question is composed of deficient replication after receiving Oxford/Covishield immunization. Immune this variant causing neuropathy in Latin America. Although
of the adenovirus in chimpanzees, which stimulates the reactions can occur due to an immunological imbal- all cases reported had a EDX with predominantly demy-
S glycoprotein of COVID 19, producing T cells and anti- ance in leprosy. These reactions are divided into type 1, elinating features, the case here presented is of a clear
bodies, which generates a molecular mimicry generating or reverse reaction, and type 2, or erythema nodosum axonal phenotype, as demonstrated by EDX and nerve
the attack on the myelin sheath, which culminates in guil- leprosum and are acute and potentially serious and can ultrasound. Delayed puberty was present in our patient,
lain-barré syndrome ( GBS). In all the cases presented, constitute a medical emergency due to their morbidity like classical leukodystrophy type. Other features such as
a closed diagnosis and clinical, cerebrospinal fluid and and the worsening of already existing damage. There are abnormal dentition and ataxia were not present. Final
Enmg criteria. A common fact that draws attention is the some risk factors related to the triggering of those reactions Comments: Despite widespread use of Next Generation
high increase in protein, creating a CSF pattern for GBS such as co-infections and vaccines. The pathophysiology Sequencing in Hereditary Neuropathies, many patients
post-ChAdOx 1 vaccine, not yet evidenced in the literature. of leprosy reaction type 2 is still not completely under- remain without a final genetic diagnosis. This number
Final Comments: Even though it is a rare occurrence, stood, but previous studies show an intense inflammation is even larger in axonal types. Newly described variants
post-AstraZeneca vaccine GBS, totaling 27 cases in Brazil mediated by humoral immune response, with increased associated with disease as the one here presented are of
until 07/2021, being included as an adverse event in the levels of TNF-?. These same mechanisms are possibly great interest in improving diagnostic rates and expand-
immunization package insert. Given this fact, added to involved in the immune response against COVID-19 ing phenotypes.
the importance of vaccination, since it reduces about 80% and may be related to this post-vaccine reaction episode.
Hospitalizations in the elderly. It is extremely important Final Comments: The reported case serves as an alert to Neuropatias Periféricas
to create a pattern for this new etiology of GBS, studying the possible consequences of the pandemic of COVID-
not only a new cerebrospinal fluid pattern, seen in an 19 for leprosy patients. It is necessary to ensure access to
important proteinorachie report, but also clinical and immunization for these patients, with increased vigilance
electroneuromyography. after immunization, especially those with multibacillary
forms and recurrent leprosy reactions.

Neuropatias Periféricas
Neuropatias Periféricas

193
XXX Congresso Brasileiro de Neurologia

TL 1106181 TL 1106506 TL 1106537

MYASTHENIA GRAVIS IN ASSOCIATION POEMS SYNDROME: CASE PRESENTATION MILLER FISHER SYNDROME (MFS)
WITH GUILLAIN BARRE SYNDROME: A CASE ASSOCIATED WITH HEPATITIS B VIRUS (HBV)
Bárbara Pina Aiello, Caio Faria Tardim, Gloria Maria
PRESENTATION Schitini de Souza, Roberto Pereira Santos, Alberto INFECTION: A CASE PRESENTATION
Caroline Meneses Resende, Pedro Mafra de Martins Pina Rodrigues Neto, Kalec Thiago Simonek Matheus Costa Bessa, Barbara Matos Almeida
Andrade, Miclecio Luiz da Silva, Ellen Dayanne de Moraes, Marcus Vinícius Rocha Monteiro, Marco Queiroz, Karoline Ferreira Mororó Menezes, Sarah
Barros Silva, Deryc Cleyner Bastos Piones, Lucas Antonio Sales Dantas de Lima, Gabriel Salim Saud Diogenes Alencar, Pedro Roa Appio, Hermany
Nascimento Monteiro, Max Luiz Mendes Ramires de Oliveira, Bruno Castelo Branco Rodrigues Capistrano Freitas, João Igor Dantas Landim,
Filho, Analuiza Silva Tenório Luna Sarmento Universidade Federal do Rio de Janeiro. Rio de Cleonisio Leite Rodrigues, Norberto Anizio Ferreira
Janeiro RJ, Brazil Frota, Fernanda Martins Maia Carvalho
Universidade Federal de Alagoas. Maceió AL, Brazil
drabarbarapina@gmail.com Hospital Geral de Fortaleza. Fortaleza CE, Brazil
caroline.resende@famed.ufal.br Universidade de Fortaleza. Fortaleza CE, Brazil

Case Presentation: A 48-year-old male was diagnosed Case Presentation: A 70-year-old woman was admitted to mcostabessa@gmail.com
with Myasthenia Gravis (MG) five years ago, when pre- our Hospital with a 2-year history of complaints of lower
senting paresis in the all four limbs, eyelid ptosis and limb paresthesia, imbalance, ascending paresis to the Case Presentation: A 55-year-old female presenting an
difficulty in swallowing, symptoms currently controlled upper limbs culminating in bedridden and weight loss of acute onset of bilateral ptosis, ophthalmoparesis, bilat-
with the use of pyridostigmine bromide and corticosteroid 30 kg. Physical examination revealed hippocratic facies, eral fixed mydriasis, limb ataxia and neck pain. Serum
therapy. Concomitantly, thymoma was also detected and diffuse muscular atrophy, severe bilateral hand contrac- serology demonstrated B virus antigen.A ENMG and CSF
treated with thymectomy, radiotherapy and chemother- ture, non-painful hepatomegaly and lower limb edema analysis were normal in the first week Because of the acute
apy, getting complete remission. In April 2022, the patient with acrocyanosis. Neurological examination showe distal onset and pupil involvement, anti botulinum toxin serum
sought an emergency care unit (ECU) due to fever and weakness, generalized hyporeflexia and pallhypesthesia, was administered. However, in 3 days, the patient still
diffuse joint pain, being diagnosed with Chikungunya hypoesthesia of the legs. Laboratory tests results showed presented worsening symptoms with new onset bulbar
and medicated with analgesics. After a week, he evolved a normal blood count and renal function, proteinuria signs. Another CSF analysis identified protein-cytologic
with paresis in the lower limbs, difficulty in locomotion, and elevated TSH and VEGF. Electroneuromyography dissociation. Plasmapheresis was started suspecting MFS.
and paresthesias in the feet and hands, looking again revealed sensorimotor peripheral polyneuropathy with a Five sessions were performed and, after that, the patient
for the ECU, where he was medicated with betameth- demylinating pattern with axonal loss and Lumbar punc- improved from symptoms and remained in Hospital for
asone. However, he evolved into urinary incontinence ture had albuminocytological dissociation. Computer dysautonomia surveillance. Treatment with tenofovir was
and progression of paresthesia to the trunk and upper tomography showed bilateral pleural effusions, ascitis also initiated for Hepatitis B, considering the patient’s
limbs, being referred to the State General Hospital, where and osteosclerotic lesions at the vertebral body of L3, L4, clinical condition was an extrahepatic manifestation.
he was diagnosed with Guillain B arré syndrome (GBS), and sacroiliac. Further investigation exhibited protein Due to improvement of dysautonomia and neurological
treated with plasmapheresis for 10 days. He currently electrophoresis with a distortion in gamma zone and symptoms, the patient was discharged from Hospital,
has grade 5 strength in all limbs and preserved tactile, immunofixation elevated free light chains. Bone marrow after another ENMG, that showed typical signs of Miller
painful, and vibration sensitivities, only complaining biopsy showed a reactive and hypercellular marrow with Fisher Syndrome. Discussion: In MFS there is an acute
of diffuse paresthesias. Discussion: GBS and MG are interstitial plasmocytosis and polyclonal free light chain polyneuritis, characterized by a triad of areflexia, ophthal-
autoimmune neurological diseases. MG is caused by the elevation. Discussion: POEMS syndrome is definided moplegia and ataxia. These last two signs were present in
action of anti-acetylcholine receptor antibodies, causing by the presence of a monoclonal plasma cell disorder, the patient in question. Anti-ganglioside antibodies are
neuromuscular junction dysfunction and culminating peripheral neuropathy, and one or more of the following found in CSF analysis of patients with MFS in more than
in fluctuating muscle weakness. MG is often associated features: osteosclerotic myeloma, Castleman disease, 90% of cases. This anti-GQ1b inflicts immune-mediated
with thymomas, as described in the case, especially in the increased levels of serum vascular endothelial growth damage on target tissue found in cranial nerves III, IV
presence of positive antibody to AChR (AChR-MG). GBS factor (VEGF), organomegaly, endocrinopathy, edema, and VI, which causes the characteristic ophthalmoplegia.
is an acute inflammatory polyneuropathy, caused by the typical skin changes, and papilledema. The pathogenesis Plasmapheresis, and intravenous human immunoglobu-
action of autoantibodies that act against myelin proteins of syndrome is not well understood, VEGF is the cytokine lin are first line treatments. There are few case-reports of
or against components of peripheral nerve axons, often that correlates best with disease activity. Our patient Hepatitis B Virus and MFS association in literature, with
triggered by infections, in this case Chikungunya. It is exhibited most of the diagnostic criteria for POEMS. The no evidence of direct cause-effect correlation. However,
estimated that the frequency of co-occurrence of MG and most disabling feature of POEMS is the demyelinating Guillain Barre syndrome has been reported as extra
GBS, as in the case in question, is less than 1 in 10 billion. neuropathy, frequently misdiagnosed as CIDP (chronic hepatic manifestation of hepatitis B, with viral antigen
Final Comments: Although comorbidity of GBS and MG inflammatory demyelinating polyradicuneuropathy). present in CSF and nerve biopsy. Final Comments: The
is extremely rare, early recognition of this combination of A typical POEMS neuropathy is a symmetrical, senso- relative rarity of MFS and the variety of diagnoses make
inflammation of peripheral nerves and the dysfunction of rimotor, length-dependent painful neuropathy, which it difficult to plan and implement procedures and treat-
neuromuscular junction is of great importance for both progresses proximally over time. Treatment is aimed at ments. It is assumed that the anti-GQ1b antibody is the
initial treatment and a better prognosis. the clonal hematologic disease and the cytokine drive mediator of such syndrome. Thus, early recognition and
and supportive care for the neurological and endocrine prompt plasmapheresis therapy can decrease the severity
consequences of the disease. Final Comments: The aim and duration of the disease. Furthermore, there is still a
Neuropatias Periféricas of this study was to report a case of POEMS syndrome, need for unpublished treatments that are evidenced in
emphasizing the importance of this condition, which, terms of their effectiveness and availability.
although rare, it should be part of the differential diag-
nosis of polyneuropathy.
Neuropatias Periféricas
Neuropatias Periféricas

194
XXX Congresso Brasileiro de Neurologia

TL 1106617 TL 1105639 TL 1106379

DEVELOPMENT OF NEUROMA SECONDARY THE IMPACT OF INTRAOPERATIVE EFFECT OF NON-INVASIVE


TO DECOMPRESSIVE SURGERY FOR CARPAL NEUROPHYSIOLOGICAL MONITORING NEUROMODULATION ON LANGUAGE
TUNNEL SYNDROME ON THE POST-SURGICAL CLINICAL REHABILITATION IN ACUTE STROKE
REPERCUSSION OF AN EPENDYMOMA: A
Gustavo Carvalho Nogueira, Victoria Maria Costa, Marta Maria da Silva Lira-Batista, Paulo Filho
Fernando Augusto Pacifico, Rodrigo Alves Altaíde, CASE PRESENTATION Soares Marcelino, Fabíola Mara de Castro Araújo,
Jônatas Wesley Ferreira, Levi Lucena Diógenes, Maria Luiza de Sousa Andrade, Jackson Daniel Marcello Holanda de Andrade, Júlia Rachel Ferreira
André César Soáres, Erlan Pércio Rufino Sousa Silva, Jóshua Matheus de Sousa Leda, João Meneses, Rogério da Silva Batista, Tibério Silva
Vitor Monteiro Santos, Lia Leal Laurini, Renan Borges dos Santos
Faculdade de Medicina de Olinda, Olinda, PE, Brazil.
– Olinda – PE – Brazil Camarço do Lago Veloso, Kairo Igor Freitas de HU-UFPI. Teresina PI, Brazil
Faculdade de Medicina de Olinda – Olinda – PE – Aquino, Ayllan Pabllo Viana Nunes Pereira, Maria IAMSPE, São Paulo SP, Brazil
Brazil Eduarda de Sousa Vieira, Letícia Farias Oliveira IESVAP – PARNAIBA – PI – Brazil
Universidade Federal de Pernambuco, Recife, PE, Centro Universitário Uninovafapi. Teresina PI, Brazil IFPI. Teresina PI, Brazil
Brazil – Olinda – PE – Brazil Hospital Universitário da Universidade Federal do
Centro Universitário Maurício de Nassau, Recife, PE, fgamarthalb@gmail.com
Piauí. Teresina PI, Brazil
Brazil – Olinda – PE – Brazil Universidade Federal do Piauí. Teresina PI, Brazil
Hospital Real Português de Beneficência. Recife Case Presentation: A 58-year-old male patient in the late
PE, Brazil malu.sousa.andrade@hotmail.com postoperative period of mitral valve replacement associ-
ated with infective endocarditis. It evolved suddenly with
victoriafontenelecosta@gmail.com Case Presentation: Patient, male, 59 years old, in 2019, motor and language alterations (onset of symptoms 14
started with neck pain along with paresthesia in an irra- days prior to Hospital admission date). On examination,
Case Presentation: A 42-year-old female with a previous diated band for the upper limbs (UL), muscle strength NIHSS 16 (right hemiplegia and motor aphasia). Brain
diagnosis of carpal tunnel syndrome and two months of in the upper limbs grade IV and normal coordination. imaging showed hypodensity in the left middle cerebral
surgical decompression evolution median nerve endo- Cervical magnetic resonance imaging showed an intra- artery territory associated with ECASS 2 hemorrhagic
scopically. According to the information collected, during medullary expansive lesion at the level of C4-C5 with transformation. The patient was clinically managed,
the procedure (CIS) there was a possible cutting injury a neoplastic appearance, ependymoma. Surgery was and rehabilitation measures were initiated while in the
to the aforementioned nerve. After detecting the lesion, performed through posterior cervical access with lam- Hospital. There were 4 sessions of Transcranial Direct
the physician would have made a longitudinal incision inectomy and fixation. Direct stimulation of medullary Current Stimulation – tDCS (20 minutes/session; 2mA;
in the region for exploration, followed by primary raphy tissue was also used for precise location of the physio- 35cm2)+ 8 sessions of speech therapy. Semantic and pho-
of the nerve. In the late postoperative period, the patient logical midline and determination of the best myelotomy nological processing were prioritized in a functional com-
evolved with intense pain and paralysis of the muscles site. There was complete resection of the lesion during municational context. After the interventions (discharge
of the median nerve territory. The physical examina- microsurgery with the use of intraoperative neurophysi- from Hospital), an improvement in fluency, access to
tion showed limited flexion and abduction of the wrist, ological monitoring, which demonstrated importance in the lexicon, and spontaneous and written language was
weakness in thumb opposition, painful palpation of the the functional and clinical outcome of the patient, given observed, affecting mood, adherence to other opportu-
region and positive Tinel’s sign. A MRI of the wrist was the sensory, which is potential more than expected, and nistic therapies (psychology and physical therapy), and
requested, which showed a neuroma on the topography without alterations in motor potentials. Postoperatively, a reduction in the NIHSS to 12. Discussion: Changes in
of the median nerve with a probable total lesion. The the patient presented ataxic gait, hypoesthesia in the lower neuroplasticity and cortical excitabilitymay contribute
patient underwent microsurgery to expose the lesion limbs (LL), mild paresthesia on the right side, and altered to regulating neural activity. Both could be modified by
and exeresis of the neuroma, with subsequent sural nerve proprioception. Two months after surgery, the patient applying direct electrical current in the sensorimotor
grafting to try to improve pain and return movement of progressed with significant motor improvement, without cortex, with results dependent on the type and modality
the muscles innervated by the affected nerve. One year worsening muscle strength. The sensory deficit reduced of the polarity of the current, and it seffect would last for
postoperatively, the patient evolved with a functional significantly and gradually in postoperative evaluations. days after the end of the stimulation. To describe lan-
hand, grade 4 strength in the muscles innervated by the At six months after the surgery, there were no more com- guage skills achieved from Hospital bedside language
median nerve and significant pain improvement (VAS 1). plaints. Discussion: Among the main subtypes of glio- rehabilitation in acutestroke. The cascade effect on ther-
Discussion: Intraoperative complications of carpal tun- mas, ependymomas stand out, accounting for 60 to 80% apeutic improvement has been well documented in the
nel syndrome treatment can occur during superficial of the cases. They are the most common intramedullary literature; however, the use of tdcs in the acute phase of
skin dissection or ligament release. Endoscopic decom- tumors in adults, whose peak age varies between 30 and stroke isstill controversial, despite several studies having
pression surgeries of the nerve in question, can lead to 40 years. Due to the intramedullary location, the patient’s presented consistent data.In the Hospital environment,
the development of certain complications. The median symptoms resulted from progressive myelopathy due to it is highly relevant to provide therapies thatshorten
nerve, having both motor and sensory fibers can have a compression of normal neural pathways. Intraoperative rehabilitation since most patients come from accessible
variable pattern of deficit. Lesions repaired with neural neurophysiological monitoring (IONM), used in surgery, is health networks(health prevention). Final Comments:
raphy, can evolve with damage to the internal structure performed using techniques for analyzing the integrity of The semantic and phonological networks were improved
of the nerve fiber, allowing fascicular escape and disorga- the neural system through somatosensory evoked poten- by using tDCS in the Hospital environment, providing
nized nerve repair of axons in the circulating tissue. Added tials (SEPs), motor evoked potentials (MEP), D-wave and an opportunity to enhance mood and adherence to the
to this, there may be deposition of fibroblasts, schawann electroneuromyography. This technology has been the proposed multidisciplinary therapies.
cells, which usually evolve with neuroma formation. After crucial factor in the post-surgical clinical repercussions,
neuroma formation internal neurolysis may be indicated, as it helps in the real-time feedback of critical pathways,
with subsequent nerve autograft to supply the motor Neurorreabilitação
preventing neurological deficits, enabling a rapid clini-
and sensory needs related to the median nerve damage. cal evolution, in the absence of sequel, accelerating its
Final Comments: The case presented and the publica- functional status, as it happened with the patient. Final
tions raised bring to light the discussion of the therapy Comments: Neurophysiological monitoring is a crucial
of a complex situation that is postoperative neuroma factor to avoid nervous involvement with postoperative
and show that although adopted in a minority of cases, motor and sensory repercussions, this been, a factor for
when well performed and in properly selected patients, reducing the risk of sequelae in the patient’s clinic.
internal neurolysis associated with autogenous graft is
able to obtain satisfactory and lasting results regarding
symptomatic relief. Neurorreabilitação

Neuropatias Periféricas

195
XXX Congresso Brasileiro de Neurologia

TL 1105373 TL 1104934 TL 1105878

IMPRESSIONS FROM MEDICAL STUDENTS ARTERY OF PERCHERON TERRITORY HYPERSOMNIA, BEHAVIORAL AND
ON THE IMPACT OF THE COVID-19 PANDEMIC INFARCT: AN UNUSUAL CAUSE OF COGNITIVE ALTERATIONS: REPORT OF
ON SLEEP AND QUALITY OF LIFE EXCESSIVE DAYTIME SLEEPINESS KLEINE LEVIN SYNDROME
Anderson Matheus de Lima Barbosa, Lucas Luan Vinícius Lopes Braga, Eduardo Claus Constante, Raimundo Marcial de Brito Neto, Viviane
Gonçalves Barros Leal, Carolina Queiroz Feijo, Carina da Silveira Massaro, Wardislau Ferreira, Alexandre da Silva, Renata Cavalcanti Eiras, Ilana
Thiago Assis Borges Morais, Maria Eduarda Fernando Morgadinho Santos Coelho Cwajgenberg, Marco Antonio Araujo Leite
de Souza Arêa Leão, Leonardo Halley Carvalho
Pimentel Escola Paulista de Medicina – Universidade Federal Universidade Federal Fluminense. Niteroi RJ, Brazil
de São Paulo, São Paulo SP, Brazil
Unifacid. Teresina PI, Brazil marcialbrito@live.com
UESPI. Teresina PI, Brazil viniciuslopesbraga@live.com
Case Presentation: R.C.C., 29 years old, started episodic
andersonbarbosa7570@outlook.com Case Presentation: A 23yo female comes to the emergency hypersomnolence at age 15. Sleep attacks lasted between
department complaining of excessive daytime sleepiness days to three weeks. The first was preceded by bacterial
Background: The new Coronavirus, known as SARS- (EDS) starting in the last three days. Before, the patient pharyngitis. During these periods, he only slept and only
CoV-2, had its first case recorded in Wuhan, China in went to bed around 11 pm, slept at 1: 30 am, and woke fed or ingested liquids when he was awakened. During
December 2019. However, there are already more than up at 8: 00 am. Currently, the patient sleeps around 18 to awakenings, aggressive behavior, disinhibition, polypha-
500 million confirmed cases of COVID-19 in 220 countries 20 hours per day. The patient refers one episode of visual gia, visual and auditory hallucinations, as well as memory
around the world, with about 6 million deaths due to the hallucination three days ago. After that, permanent mild changes were observed. There were no mood disorders,
novel coronavirus. There are many repercussions gener- dizziness and sleep attacks. Her Epworth sleepiness scale no sleep disturbances, substance abuse, or previous
ated by the pandemic established as a result of this virus, was 14/21. The neurology exam was normal, except by sub- developmental delays. The first physical examination
especially consequences for people’s quality of life and jectively drowsy but aroused easily to voice. The general performed after the third episode of hypersomnia was
sleep. Medical students are a target group of studies on labs, cerebrospinal fluid, serum tests, rheumatological and normal. However, there was a history of spontaneously
quality of life and sleep due to the stress and pressure they hypercoagulability panels were normal. Axial FLAIR and appearing hematomas on the skin. During this period,
go through during academic life. Objectives: To evaluate T2-weighted MRI show symmetrical hyperintense lesions magnetic resonance imaging of the brain, examination
the impact generated by the COVID-19 pandemic on the on the paramedial thalamus with abnormal restriction of cerebrospinal fluid, digital electroencephalogram and
quality of life and sleep of medical students in Teresina-PI of water diffusion on DWI and a low ADC. The findings polysomnography were performed, with normal results.
through their impressions. Methods: This is an observa- above confirmed the diagnosis of an acute infarction Other complementary tests were also requested. There
tional, quantitative, and cross-sectional study, performed of the artery of Percheron (AOP). Discussion: EDS is a was a change in the white series in the blood count. Bone
by filling out an online form by medical students and sent common complaint in neurologic practice and has a marrow biopsy showed changes consistent with chronic
through social networks, from June to December 2021. wide spectrum of differential diagnoses. We describe an myeloid leukemia (CML). The intensity and frequency of
Results: A sample of 198 participants was obtained, most unusual cause of EDS caused by stroke in the bilateral hypersomnia episodes, which reached four in a single year,
of the participants were female (66%), with an average age thalamus. Thalamus is a large egg-shaped mass of grey decreased over the years. The last crisis was in 2020, last-
of 23 years, most of the students were in the last two years of matter situated at the core of the diencephalon and lies ing 1 week. Thus, the diagnosis of Kleine-Levin Syndrome
medical school (56.5%) and were from a public university between the midbrain and forebrain, acting as a central (KLS) was made. Discussion: KLS is a rare sleep disorder,
(64%). Less than half (40%) of respondents said they had hub. The medial thalamus is involved in memory and is characterized by recurrent episodes of hypersomnia that
a good quality of sleep. When students compared poor essential for regulating arousal because it represents the last from days to weeks associated, during the period
sleep quality before and during the COVID-19 pandemic superior extension of the midbrain reticular activating of awakening, with transient cognitive and behavioral
there was a 13% increase. Regarding quality of life, 70% of system. Bilateral lesions of the medial thalamus can cause changes. The frequency and intensity eventually decay
respondents said they had been negatively impacted. In hypersomnia and decrease consciousness. Paramedian over 8 to 12 years, and may become extinct. The estimated
addition, most students classified as negative the impact arteries usually supply the medial thalamus. AOP is prevalence is 1-5: 1,000,000 of cases, being more common
generated by the COVID-19 pandemic on sleep, academic a rare anatomical variation presented in 4-12% of the in men. The average age of starting SKL is 16 years. The
performance, continuity of their studies, family income, population when a single arterial trunk originating from pathophysiology is still unknown. Autoimmune disorders,
physical and mental health. On the other hand, the vast the posterior cerebral artery irrigates the paramedian among others, appear as candidates for precipitating
majority of students (71.7%) reported improvement in regions of the thalamus and, sometimes, the rostral mid- factors of the syndrome. However, we did not find CML
family relationships. Final Comments: COVID-19 has brain. Bilateral paramedian thalamic infarcts account for among them in the scientific literature. The diagnosis is
significantly impacted in a negative form many areas approximately 0.1% to 2% of all ischemic strokes and 27% based on criteria established by the 3rd International
related to the quality of life and sleep of medical students. of thalamic infarcts. People with AOP infarct have vertical Classification of Sleep Disorders (ICSD). Because it is a
Perhaps due to the measures of distancing, interruption of gaze paresis (65%), neuropsychiatric symptoms, coma rare syndrome, there are no clinical trials and treatment
various activities and uncertainty about their continuity. (42%), and memory impairment (58%). The EDS was the guidelines. Currently, exclusively symptomatic therapy
initial complaint in our patient and improved gradually is chosen. Final Comments: In addition to the rarity of
after two months. Final Comments: Clinicians must be the syndrome, the never mentioned coexistence with a
Neurossonologia aware of AOP’s infarction as a differential diagnosis of lymphoproliferative disease and the occurrence of CML
EDS, especially in the presence of sudden EDS onset or in an unusual age group may provide new insights into
neuropsychological and ophthalmological signs. the pathophysiology of the disease.

Sono Sono

196
XXX Congresso Brasileiro de Neurologia

TL 1106305 TL 1106328 TL 1106414

HYPNIC HEADACHE AND COVID-19 WHOLE-GENOME ANALYSIS OF SLEEP PATERNS AMONG PATIENTS WITH
PANDEMIC: IS IT A COINCIDENCE? MONOZYGOTIC TWINS DISCORDANT FOR CEREBROTENDINOUS XANTHOMATOSIS
TYPE 1 NARCOLEPSY
José Luis Lima Neto, Wardislau Ferreira, Cristina Alissa Elen Formiga Moura, Davi Farias de Araujo,
Frange, Fernando Morgadinho Coelho João H. C. Campos, Ana C. R. Aguilar, Fernando Anderson Moura Bernardes, Davi Augusto Batista
Antoneli, Giselle Truzzi, Marcelo R. S. Briones, Sá Araújo, Paulo Ribeiro Nóbrega, Pedro Braga Neto,
Universidade Federal de São Paulo. São Paulo SP, Manoel Alves Sobreira Neto
Renata C. Ferreira, Fernando M S Coelho
Brazil
Universidade Federal de São Paulo. São Paulo SP, Universidade Federal do Ceará. Fortaleza CE, Brazil
fernandomorgadinho@hotmail.com
Brazil
alissamoura@gmail.com
Case Presentation: Hypnic headache (HH) is character- fernandomorgadinho@hotmail.com
ized by attacks that occur only during sleep with wakening. Case Presentation: Two adult patients with CTX were
Patients with HH complain of pain between fifteen minutes Case Presentation: Narcolepsy type 1 (NT1) is a rare and evaluated with sleep anamnesis, physical exam and full-
to four hours, bilateral, and varies from mild to moderate chronic neurological disease characterized by sudden night type 1 polysomnogram. Case 1: J.R.A, male, 42 years
intensity. The pathophysiology is poorly understood, but sleep attacks, overwhelming daytime drowsiness, and old, presents generalized epilepsy and neuropsychomotor
circadian disruption can be involved. We describe a case cataplexy. To contribute to the understanding of NT1 development delay since childhood. He had cataracts in
of HH during the COVID-19 pandemic. A 22-year-old genetic basis, here we describe a whole-genome analy- his left eye, ataxia and tendon xanthomas. C.M.C, female,
patient from São Paulo complains of headaches exclu- sis of a monozygotic twin pair discordant for NT1. Our 32 years old, presents alterations in neuropsychomotor
sively during sleep (at night and napping), without auto- whole-genome analysis revealed that both twins have development in childhood, in addition to diarrhea. She
nomic symptoms, that started during the quarantine in identical pathogenic mutations in NT1 associated genes had bilateral visual acuity impairment, ataxia, pyramidal
the first wave of the COVID-19 pandemic in Brazil. The (such as HLA-DQB1*06: 02: 01, HLA-DRB1*11: 01: 02/*15: signs and tendon xanthomas. Both patients had genetic
patient had significant circadian disruption during that 03: 01) and therefore the affected twin has the expected confirmation. Sleep events were scored according to stan-
period of life due to the pandemic. Neurological exams, clinical manifestation while the unaffected twin has an dard clinical criteria according to the American Academy
magnetic resonance imaging, magnetic resonance angi- unexpected phenotype. This suggests that the unaffected of Sleep Medicine guidelines. The patients reported no
ography of the brain, and sleep studies were regular. He twin might have protective alleles outside the HLA com- specific sleep complaint or daytime consequence. Physical
had three PCR tests negative for SARS-CoV-2 and no flu plex. The unaffected twin has significantly more frameshift examination of those subjects revealed cranial deformities
symptoms. Treatment with lithium carbonate 450mg at mutations as compared to the affected twin (108 versus including high-arched palate and maxillary hypoplasia. No
night led to the remission of the pain attacks. Circadian 75) and mutations that affect stop codons (61 versus 5 in soft tissue abnormalities where identified. Patient 1 poly-
rhythms interfere in many aspects of physiology and stop gain, 26 versus 2 in start lost). Frameshift mutations somnography showed reduced sleep efficiency (37.2%),
pathophysiology of many biological systems and head- and stop codon mutations are generally associated with high apnea hypopnea index (AHI) (15.5events/h), high
aches. The association between the sleep patterns caused loss-of-function effects and protective alleles are almost periodic leg movement index (PLMi)(77.4 events/h) and
by the restrictions during the COVID19 pandemic can always loss-of-function rare alleles. Also, overrepresen- presence of alpha-delta pattern in NREM sleep. Patient
influence the incidence of a few kinds of headaches such tation analysis of genes containing variants with poten- 2 exam showed reduced sleep efficiency (80%), normal
as HH. The disrupted sleep and circadian rhythm could tial clinical relevance in the unaffected twin shows that AHI (2.2 events/h) and presence of alpha-delta pattern.
be contributed to the beginning of HH. Indeed, the med- most mutations are in genes related to immune regu- Discussion: Cerebrotendinous xanthomatosis (CTX) is
ication that adjusted the circadian cycle was effective. lation function, Golgi apparatus, MHC, and olfactory a rare autosomal-recessive lipid storage disease caused
This case highlights the possible impact of the COVID-19 receptor. These results support the notion that NT1 has by pathogenic variants in CYP27A1, which lead to defi-
pandemic and the lockdown on the incidence of HH and an immunological basis but that protective mutations in ciency of the mitochondrial enzyme, sterol 27-hydroxylase,
other headaches due to sleep and circadian disruption. non-HLA alleles might interfere with the expression of resulting in the accumulation of cholestanol in the serum
the NT1 phenotype and consequently with the clinical and many organs. Clinical presentation is variable and
manifestation of the disease. Discordant monozygotic characterized by cholestasis, refractory diarrhea, juvenile
Sono twin studies provide a unique opportunity to observe the cataracts, tendon xanthomas, osteoporosis, coronary heart
effects of very rare alleles that might not be picked up in disease, and progressive neuropsychiatric disturbances.
populational studies. Neuropsychiatric abnormalities include intellectual dis-
ability or dementia, psychiatric symptoms, cerebellar
signs, pyramidal signs, progressive myelopathy, peripheral
Sono neuropathy, extrapyramidal manifestations, and seizures.
To our knowledge, sleep disorders have not been studied
in these patients. Final Comments: Patients with CTX may
present higher prevalence of sleep disorders when com-
pared with the general population. The absence of sleep
complains could be related to an overlap of symptoms
with other neuropsychiatric disorders. Other studies with
higher number of patients are necessary to understand
sleep disorders in these patients.

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XXX Congresso Brasileiro de Neurologia

TL 1106472 TL 1106601 TL 1104981

CENTRAL NERVOUS SYSTEM REFRACTORY HYPERSOMNOLENCE IN A ACERULOPLASMINEMIA: A SEVERE


GANGLIOGLIOMA PRESENTING WITH PATIENT WITH MULTIPLE AUTOIMMUNE NEUROMETABOLIC DISORDER
SEVERE CENTRAL AND OBSTRUCTIVE SLEEP MORBIDITIES AND PSYCHIATRIC DISORDER
Carina Silveira Massaro, Wardislau Ferreira,
APNEA IN A CHILD: A CASE PRESENTATION Filipe Pereira Sarmento, José Marcos Vieira de
Maria Júlia Figueiró Reis, Fernando Morgadinho
Douglas Sousa Soares, Karla Carlos, Lorena Raulik Santos Coelho Albuquerque Filho, Vinícius Lopes Braga, Eduardo
Cyrino, Katrine Freitas Valeriano, Mariana Campos Claus Constante, Henrique Ballalai Ferraz
Universidade Federal de São Paulo. São Paulo SP,
Gubeissi, Ricardo Silva Pinho, Gilmar Fernandes Universidade Federal de São Paulo. São Paulo SP,
Brazil
Prado Brazil
julia_figueiro@hotmail.com
Universidade Federal de São Paulo. São Paulo SP, ca_massaro@hotmail.com
Brazil
Case Presentation: 35-year-old female presenting with
douglas.sousa.soares@gmail.com excessive daytime sleepiness (EDS), insomnia, bruxism, Case Presentation: 59-year-old woman presented with
snoring, fatigue, and fluctuating muscular weakness. She recent onset depressed mood and emotional lability.
Case Presentation: we report the case of a four-year- also complained of periods of hypersomnia that lasted for After 6 months, forgetfulness, impairment of activities
old girl who has experienced, according to her mother, 3 to 7 days, without food compulsion, hypersexuality, or and speech difficulties were observed, followed by gait
snoring, breathing pauses lasting for 8-10 seconds, and worsening of cognitive impairment. There was also wors- imbalance. On examination, she was dysarthric, disori-
a breathing pattern suggestive of Cheyne-Stokes ventila- ening of muscular weakness in events of acute distress, ented in time, and also had saccadic intrusions, rigidity,
tion during sleep since she was two years old. At the age but without complaints of sleep attacks, hallucinations, oral dyskinetic movements, cervical dystonia, and ataxic
of three, she presented headaches, vomiting and mental or sleep paralysis. She had the additional diagnosis of gait. Investigation included complete blood counts, liver,
confusion, being diagnosed with Grade I ganglioglioma Mast Cell Activation Syndrome (MCAS), Ehlers-Danlos thyroid and kidney function tests, HIV, syphilis and hep-
in brainstem and cerebellum. She was submitted to tumor Syndrome subtype 3, chronic migraine, depression, atitis serologies; and they were unremarkable. Additional
resection and maintained under vinblastine chemother- Asperger syndrome, and a history of thyroidectomy fol- tests revealed elevated ferritin levels (2540ng/ml), low
apy. The sleep complaints got progressively worse, and lowed by radioactive iodine therapy due to papillary car- serum iron (27 µg/dL), reduced plasmatic ceruloplasmin
she was referred to evaluation in the Neurology Sleep cinoma. She also referred to anasarca, drowsiness, and and low urinary copper. Brain MRI showed T2 hypointen-
Medicine service. This child was obese (BMI = 37.6 kg/ generalized muscle weakness in extremes of climate or sity and SWI low signal on basal ganglia, thalamus and
m²), usually sleeping from 11 p.m. to 9 a.m., waking up acute distress and with consumption of histamine-rich dentate nucleus. NGS-based gene panel testing was
multiple times to urinate, but without any complaints foods. On physical examination, she had retrognathia performed showing the pathogenic variant c.1149G>A
of daytime sleepiness. She also had a small oral cavity and ogival palate, and there was also unilateral sustained (p.Tr383) as well as a variant of uncertain significance
(Mallampati 4, Brodsky tonsil scale 3, and an ogival pal- palpebral ptosis. Muscular fatigability tests were positive (VUS) c.31118T>C (p.Cys1040Arg) in the ceruloplasmin
ate). Polysomnography (PSG) evidenced severe sleep for diplopia and led to discrete appendicular weakness. (CP) gene, in heterozygosity. The VUS is expected to dis-
apnea [Apnea Hypopnea Index (AHI) = 84.1 events/h] Polysomnography showed moderate obstructive sleep rupt CP protein function, although not yet reported on
with both obstructive (AHI = 13.3 events/h) and central apnea (apnea-hypopnea index of 21 events per hour) CP-related conditions. Despite not being able to determine
(AHI = 70.8 events/h) components. Baseline SatO2 was and periodic limb movement syndrome (periodic limb if the CP variants are in cis or in trans, our final diagnosis
90% and it decreased until 68% during apnea episodes. The movement of 49 per hour). Multiple latency sleep tests was aceruloplasminemia (ACP). The patient initiated the
central sleep apnea (CSA) showed up in the second half showed no sleep-onset REM period and average sleep treatment with deferoxamine combined with fresh frozen
of the night, and Cheyne-Stokes breathing was observed. latency of 19 minutes. Electroneuromyography with sin- plasma (FFP) which controlled the disease progression.
CPAP titration was effective to stop obstructive sleep gle fiber was compatible with myasthenia gravis, and the Discussion: ACP is an adult-onset autosomal recessive dis-
apneas at the pressure of 8cm of H2O, yet central apneas autonomic study was suggestive of autonomic neuropa- order caused by homozygous or compound heterozygous
remained. In this second PSG, CSA also appeared in the thy of tiny fibers. Thoracic computerized tomography did mutations in the CP gene (responsible for iron homeosta-
second part of the night. Discussion: This case is about not demonstrate thymoma. Laboratory workup showed sis). This mutation can cause systemic and cerebral iron
a rare condition, associated to severe central (also rare in iron deficiency and a positive dosage of anti-acetylcho- overload. The clinical features are diabetes, retinopathy,
children) and obstructive sleep apnea, not to mention the line receptor antibody. Renal and thyroid function and liver disease and progressive neurological symptoms:
Cheyne-Stokes breathing. The prevalence of CSA is 4-6% dosage of vitamin B12 were normal. She sustained EDS movements disorders and cognitive dysfunction. An early
in children with underlying conditions (using a cutoff > (Epworth sleepiness scale of 12/24) after treatment with sign of the disease is an atypical anemia with microcy-
5 events/h). Brainstem lesions may lead to several sleep CPAP, intravenous iron supplementation, and use of lis- tosis, low transferrin saturation and hyperferritinemia.
modifications, such as reduced sleep efficiency, excessive dexamfetamine, venlafaxine, trazodone, escitalopram, Brain MRI can show dentate nuclei of cerebellum, basal
daytime sleepiness, decreased stage 3 and REM sleep, and aripiprazole, coenzyme Q10, levothyroxine, and zolp- ganglia and thalamus with diffuse hypointensity in T2
narcolepsy symptoms. These lesions might cause alveolar idem. In the following steps, we will look for the preva- and SWI. The diagnosis of ACP is established with typical
hypoventilation, having an impact on sleep, which may lence of the HLA-DQB1*0602 allele and hypocretin level clinical findings and the identification of pathogenic CP
be severe enough to cause respiratory insufficiency and in cerebrospinal fluid. Final Comments: The differential gene variants. The treatment is focused on iron chelators
require mechanical ventilation, especially when lesions diagnosis of EDS is complex and multifactorial. Proper therapy (ICT). ICT is more effective in reducing systemic
extend to the medulla. Brainstem lesions are a rare yet workup is essential for a better clinical approach, although iron overload but less effective on neurological symptoms
possible cause of CSA and the prevalence of central ner- polytherapy is not always sufficient to entirely manage and it can be combined with FFP administration. Final
vous system (CNS) tumors is higher in individuals with the symptoms. Comments: ACP is a severe neurometabolic disorder
CSA when compared to controls. Final Comments: The that needs a prompt diagnosis and therapy to prevent
presence of brainstem lesions increases the risk of multiple neurological complications.
sleep pathologies. CSA is a rare condition in children and Sono
CNS tumors should be investigated as a possible cause.
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XXX Congresso Brasileiro de Neurologia

TL 1105061 TL 1105185 TL 1105195

CLINICAL REASONING IN THE DIAGNOSIS A TEENAGER WITH CHOREA AND TOOTH PARKINSON’S DISEASE EMERGED DURING
OF MULTIPLE SYSTEM ATROPHY: A CASE AGENESIS: A CASE PRESENTATION ACUTE COVID-19 ILLNESS:
PRESENTATION A CASE PRESENTATION
Patrícia Aurea Andreucci Martins Bonilha, Thábata
Lia Leal Laurini, Tibério Silva Borges dos Santos, Emanuelle Martins Nunes, Beatriz Cassarotti, Leo Fernando Tonon Schneider, Marina Scop Medeiros,
Denise Maria Meneses Cury Portela, Matheus Coutinho, Helio Afonso Ghizoni Teive Marina Musse Bernardes, Renata Diefenthaeler
Rodrigues Corrêa, Natália Rebeca Alves de Araújo, Campos, Rodrigo Twardowski Scherer, Yuri Ferreira
Universidade Federal do Paraná. Curitiba PR, Brazil
Luana Larisse de Sousa Araújo, Maria Luiza de Felloni Borges, Gustavo Chatkin, Jaderson Costa da
Sousa Andrade, Maria Alice Silva Vasconcelos, patriciambonilha@hotmail.com Costa, Lucas Porcello Schilling
Francisco Sales da Silva Júnior, Thiago Assis Borges
Morais Escola de Medicina da Pontifícia Universidade
Case Presentation: A 16 year old female patient began
Católica do Rio Grande do Sul. Porto Alegre RS,
7 years earlier with involuntary movements in superior
Centro Universitário Uninovafapi. Teresina PI, Brazil Brazil
limbs and trunk, being medicated with Clonazepam and
Hospital Universitário da Universidade Federal do Instituto do Cérebro do Rio Grande do Sul. Porto
Topiramate, but with no improvement of the symptoms.
Piauí. Teresina PI, Brazil Alegre RS, Brazil
Previously use of Levothyroxine and SSRI, there was no
Universidade Federal do Piauí. Teresina PI, Brazil Escola de Medicina – Pontifícia Universidade
history of delivery complications or abnormal neuro-
Universidade Estadual do Piauí. Teresina PI, Brazil Católica do Rio Grande do Sul e Instituto do Cérebro
development. She had no family history of movement
do Rio Grande do Sul – Porto Alegre – RS – Brazil
lialeal02@gmail.com disorders or other neurologic diseases. The neurologic
exam showed gait ataxia, myoclonus on upper limbs, fernando_tonon@hotmail.com
Case Presentation: A 53-year-old female complained generalized chorea and partial tooth agenesis. At this
of sudden and temporary loss of consciousness epi- point, a trial with Levodopa three times daily was initiated Case Presentation: A 47-year-old female was evaluated
sodes after assuming the orthostasis position at the first and the symptoms ameliorated. She underwent several in an outpatient setting with a complaint of asymmet-
admission. 2 years later, she had worsening symptoms, complementary investigations, such as brain MRI, EEG rical rigidity, rest tremor and slowness. She had been
associated with dysarthria and insidious paresis of the and blood laboratory tests, which were all unremark- Hospitalized two months prior due to mild COVID-19,
right side. Currently, she had a clinical deterioration, with able. At last, whole exome sequencing was performed notably with anosmia and ageusia. The motor symp-
multiple fainting spells; severe dysarthria; insomnia and and the results pointed microdeletion on chromosome toms were noticed by the patient on the third day of
urinary incontinence. Upon examination, she was alert 14 (14q.13.3q.21.1), with 5,4Mb, heterozygosis and vari- Hospitalization, with progressive worsening. She has a
and oriented; had dysmetria, movement decomposi- ation on copy numbers, therefore, involving the following medical history of chronic constipation and was taking
tion, hypermetric saccade and nystagmus; right-sided genes: NKX2-1 and PAX-9. Hence, this patient was diag- multivitamins and pantoprazole. She lives in an urban area
hemiparesis; plastic hypertonia; bradykinesia 3+/4+. nosed with hereditary benign chorea within NKX2-1 spec- and works at a Hospital laundry. She declined psychiatric
Tilt test with positive result of type 3 confirmed postural trum disorder and, notably, the tooth agenesis was due or cognitive problems. Her family history was negative
hypotension and brain MRI showed tenuous T2/Flair the mutation on PAX-9 gene, both located in proximity. for neurological disorders. On MDS-UPDRS part III she
hyperintensity in the topography of the median raphe of Discussion: The mutation on NKX2-1 gene is related to scored 37 points, presenting bradykinesia grade 4 in the
the pons. Follow-up neuroimaging showed a “Hot cross systemic manifestations, such as benign hereditary cho- right upper limb (LUL), grade 3 in the right lower limb
burn” signal, cerebellar volumetric reduction and bilat- rea, congenital hypothyroidism, and neonatal respiratory (RUL) and grade 1 in LUL. She had unilateral grade 2 rest
eral putaminal atrophy. In the clinical course, Levodopa distress (also known as brain-lung-thyroid syndrome). tremor in RUL, bilateral postural and intention tremor,
was used, with an unsatisfactory response and a possible Additionally, the PAX-9 gene mutation, associated to and unilateral grade 2 rigidity in LUL. The rest of the neu-
extrapyramidal reaction, reinforcing the probable diag- hypodontia, is not expected to be involved, though, our rological exam was unremarkable. Laboratory assessment
nosis of Multiple System Atrophy (MSA). In addition, patient presents molar tooth agenesis and we concluded and brain MRI were normal. Functional study with SPECT
she used fludrocortisone and had no clinical response to that this phenotypic presentation is due the proximity with TRODAT showed bilateral putamen reduced capta-
baclofen. Discussion: MSA is a neurodegenerative disor- between the NKX2-1 and PAX-9 genes on chromosome 14. tion, with right and left basal ganglia/occipital relation of
der that affects approximately 2 to 5 people per 100,000 Final Comments: PAX-9 mutation was not expected with 0.39 and 0.58, respectively (reference value: 1.98 ± 0.24).
population and has an adult-onset with a mean age of NKX-2’s, as both doesn’t have any phenotypic or genetic She was started on levodopa/benserazide 100/25mg
55-60 years old. Although the cause of MSA is unknown, association. But, in this particular case, genetic sequenc- TID. After 1 month, she noticed an improvement with a
the described mechanism accounts for the abnormal ing showed that one mutation may lead to subsequently slight objective reduction of the MDS-UPDRS part III to
accumulation of ?-synuclein mostly in oligodendroglial alterations in other genes located nearby. Furthermore, 34 points (8.1% decrease). An increase in medication was
cells. The clinical features include autonomic failure, par- it is of great importance to remind that benign heredi- considered, but the patient did not desire dose modifica-
kinsonism and ataxia. MSA has two motor phenotypes: tary chorea is the only form of chorea that improves with tion. Discussion: Numerous neurological symptoms have
parkinsonian and cerebellar (MSA-C). This patient has an Levodopa treatment, contrarily with other choreas, which been described in acute and long COVID-19. Among the
MSA-C phenotype, presenting as gait ataxia, limb ataxia, worsen with this medication. theories involving the invasion of the CNS, there are the
ataxic dysarthria and cerebellar disturbances of eye move- hematogenous route and axonal transport through the
ments. A poor response to levodopa is highlighted as a olfactory neuroepithelium. To date, only three COVID-19-
characteristic that differentiates Parkinson’s disease from Transtornos do Movimento related parkinsonism cases have been reported. Same as
MSA, as well as cerebellar and autonomic signs. MRI may our patient, two of them presented hyposmia. The SPECT
reveal putamen, pons and middle cerebellar penducles with TRODAT was also abnormal in all three cases, and the
atrophy, as was described in the case here. The “Hot Cross motor symptom onset ranged between 10 and 32 days after
Burn”, a transverse and anteroposterior T2 hyperintensity PCR-confirmed diagnosis. Final Comments: Although a
in the pons region, is a characteristic sign result of the latent hypokinetic rigid syndrome may be unmasked in
pontocerebellar fibers degeneration. Final Comments: the context of great stress or infection, possibly by deplet-
MSA is a rare and severe disease, critical clinical reasoning ing compensatory mechanisms, the acute presentation
associated with neuroimaging is extremely important for during viral illness may implicate a more direct causal
early diagnosis, since there is a symptomatological cor- relation. Thus, this case could represent either one of
relation mainly with idiopathic Parkinson disease and secondary parkinsonism, with a more benign prognosis,
other atypical parkinsonian syndromes. or of a neurodegeneration exacerbated or even triggered
by COVID-19. Follow-up will help differentiate between
the two scenarios.
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XXX Congresso Brasileiro de Neurologia

TL 1105315 TL 1105408 TL 1105447

PARANEOPLASTIC HEMICHOREA ORTHOSTATIC TREMOR PLUS ASSOCIATED POTOMANIA AS SYMPTOM IN WILSON’S


ASSOCIATED WITH SMALL CELL LUNG WITH PARKINSONISM: A CASE DISEASE: A CASE PRESENTATION
CARCINOMA PRESENTATION
Tamires Cristina Martins de Vasconcelos, Nadja
Beatriz Marques dos Santos, Alexia Carneiro de João Eduardo Bastianello, Bibiana Thomé, Leidys Layane Gomes Santiago, Camila Orrico Peixoto,
Almeida, Larissa Baccoli de Souza, Deborah Inayara Marina Pedrozo, Bruno Fraiman, Sheila Trentin Clariana Nascimento de Oliveira, Iris Montaño
Mendes Tenorio de Albuquerque, Vanessa de Freitas Castellón, Roberta Borges Gomes Kauark
Moreira, Davi Vargas Freitas Teixeira, Andreas Escola de Medicina da Pontifícia Universidade
Católica do Rio Grande do Sul. Porto Alegre RS, Universidade Federal da Bahia. Salvador BA, Brazil
Batista Schelp, Roberta Arb Saba Rodrigues Pinto
Brazil
nadjalayane@hotmail.com
Hospital do Servidor Público Estadual de São Paulo.
São Paulo SP, Brazil João.bastianello@gmail.com
Case Presentation: It is a report of a 22-year-old male
bmarques09@gmail.com Case Presentation: Female patient, 79 years old, hyper- patient who, since March 2020, has evolved with depressed
tensive, family history of Parkinson’s disease. Five years mood, apathy, social isolation and speech reduction,
Case Presentation: A 69-year-old male, with a previous ago, she started presenting tremors in the lower limbs only anterograde amnesia and dyscalculia. In one month, he
history of hypertension and smoking, and no relevant when in orthostatic position. It progressed until last year, presented slowing of speech and hypophony, progressively
family history, presented with a report of dry cough with when it evolved to difficulty in standing. Four months ago, worsening with dysphagia, imbalance and tremors, espe-
progressive worsening over several months, evolving she started treatment with Primidone 100mg/day, with- cially in the lower limbs. He was restricted to the bed in
with mild dyspnea on exertion. He was admitted to the out significant improvement. She denied symptoms of November 2020. No comorbidities or similar family history
Pulmonology service for investigation, and a lung nod- REM sleep behavior disorder, anosmia, or constipation. and non-consanguineous parents. On physical examina-
ule was detected on chest CT. During Hospitalization, On physical examination, high-frequency tremor was tion, a palpable liver and free tube. He had depressed mood
neurological evaluation was requested due to a painless observed in orthostatic position in all four limbs, more and bradypsychism; alert, oriented, with micrograph,
involuntary movement in the right lower limb, which significant in lower limbs, grade 2 bradykinesia on the without other language changes. He presented hypoph-
had started about 2 months before. ??On physical neu- upper limbs and 1 on the right lower limb, rigidity grade ony and the kayser-fleischer rings were clear, associated
rological examination, the patient had right hemichorea 1 on the right and 2 on the left, reduced arm swing during with parkinsonism and cognitive impairment. He was
with no other abnormalities. He underwent an extensive ambulation and postural instability. Previous MRI and diagnosed with Wilson’s disease and started treatment,
etiological investigation; a brain MRI showed no signifi- ENMG were normal. Based on the patient’s clinic, the diag- progressing with potomania, with intense fluid intake
cant abnormalities, laboratory tests (including blood glu- nosis of orthostatic tremor (OT) was defined. The patient and vomiting. Psychiatric symptoms in Wilson’s disease
cose, thyroid function, infectious serology, rheumatology showed a good response to the increase of Primidone occur in almost 100%, but potomania are less common,
screening tests) were also performed with normal results. to 250mg/day and subsequently started treatment with with no cases reported in the disease, so far.
He underwent biopsy of the pulmonary nodule, and small- Levodopa 200mg/day for Parkinsonism. Discussion: In
cell lung cancer was detected. Symptomatic treatment with this report, the patient presented a movement disorder
a haloperidol was initiated, with partial improvement of characterized by tremor while in an orthostatic position Transtornos do Movimento
the condition. Discussion: Chorea is characterized by associated with parkinsonism, suggesting a diagnosis of
involuntary random movements, erratic and migratory, OT-plus. OT is a rare condition of unknown origin that
with an abrupt onset, large and short duration, which usually occurs in the lower limbs and occasionally involves
are repeated with variable intensity. Immune-mediated the trunk and abdomen. The tremor is characterized by its
chorea encompasses several etiologies including parane- high-frequency and low-amplitude. It begins immediately
oplastic, post-infectious, and idiopathic. Paraneoplastic or a few minutes after assuming orthostatic position and
chorea (PC) is a rare entity, caused by nerve tissue dam- disappears or decreases with ambulation or rest. When
age by the effects of onconeural autoimmune factors, associated with another neurological disease, in this case
mediated by T cells. PC is more common around adult parkinsonism, it is called OT-Plus. Other neurological
age, and small cell lung carcinoma (SCLC) is the most manifestations reported in the literature are Parkinson’s
prevalent association. Chorea may predate the discovery disease, dyskinesias, neuropathies and others. Final
of the neoplasm by months to years and may present in Comments: In order to make the OT diagnosis, muscle
conjunction with other movement disorders and enceph- recording performed by surface electrodes while stand-
alopathy. The antibodies most commonly identified in ing is essential, since high-frequency tremor 13 to 18 Hz
PC are CRMP-5 (CV2) and ANNA-1 (Hu) IgG. In SCLC, is characteristic. It is important to emphasize that the
approximately 70% are associated with CRMP-5. Tests conventional ENMG of the lower limbs does not present
being false negatives in a minority of cases, which may significant changes. Regarding treatment, drugs known
imply discouraging neoplastic screening in less classic to be effective in reducing tremors, such as primidone
cases. The expected brain MRI finding is basal nuclei hyp- and propranolol, should be considered. Levodopa has
osignal on T1-weighted sequences and hypersignal on T2/ also shown a modest response to OT-Plus associated with
FLAIR. The result and prognosis depend on the diagnosis parkinsonism, suggesting involvement of the dopaminer-
and early initiation of therapy. Final Comments: Although gic system. Among the differential diagnoses, it is worth
rare, a paraneoplastic etiology must be considered as a mentioning other causes of postural instability, such as
differential diagnosis in patients presenting with chorea. Parkinson’s disease, ataxias and other types of tremor.
Screening for neoplastic conditions should be performed,
and early treatment for the underlying malignancy may
help decrease morbimortality. Transtornos do Movimento

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MYOCLONUS-DYSTONIA OF PROBABLE COVID-19-ASSOCIATED MYOCLONIC CASE PRESENTATION HEMICHOREA


GENETIC ETIOLOGY: CASE PRESENTATION ENCEPHALOPATHY – REPORT OF A IN A PATIENT WITH NON-KETOTIC
WITH MOTOR SYMPTOMS RESPONSIVE TO 79-YEAR-OLD PATIENT HYPEROSMOLAR HYPERGLYCEMIC STATE
THE USE OF LEVODOPA AFTER KIDNEY TRANSPLANTATION
João Pedro Moreira Guilherme, Vanise Campos
Tarcísio Nunes Alvarenga, Pedro Machry Pozzobon, Gomes Amaral, Adriano Robert Marcião, Tales Laís Elizabete Favaretto, Giovanna Testa Brustolin,
Ana Beatriz Marangoni Baston, Patrick Emanuell Oliveira Junior, Victoria Rosas Marques, Marcus Fernanda Nicoli Broch, Daniel Fortes Amarante,
Mesquita Sousa Santos, João Vitor Mortari Lisboa, Vinicius Della Coletta Fernando Kowacs, Carlos Roberto de Mello Rieder
Igor Oliveira Fonseca, Igor Lima Teixeira
Universidade do Estado do Amazonas. Manaus AM, Santa Casa de Misericórdia de Porto Alegre – Porto
Hospital de Clínicas da Faculdade de Medicina de Brazil Alegre – RS – Brazil
Botucatu – UNESP. Botucatu SP, Brazil Universidade Nilton Lins. Manaus AM, Brazil
Fundação Hospital Adriano Jorge. Manaus AM, laisfavaretto@hotmail.com
pedro_machry_pozzobon@hotmail.com Brazil
Case Presentation: A 76-year-old woman was admitted
Case Presentation: Female, 21 years-old, without rele- mcolletta@uea.edu.br to the emergency room with involuntary movements in
vant family history, present to our service of movement the left hemibody that started suddenly four days ago.
disorders due tremor. Around 7-years-old, she had trem- Case Presentation: A 79-year-old male patient with Medical history revealed diabetes mellitus since 2005,
ors exacerbated in moments of mood changes and it was RT-PCR confirmed COVID developed jerky movements systemic arterial hypertension and renal transplantation
common to drop objects, especially in morning. After ten in his trunk, face, and limbs on the eighth day of symp- 5 months before admission, after nephropathy due to the
years the symptoms were more evident in the left upper toms, preventing him from walking. He developed dysar- underlying diseases. Pre-transplantation tests showed
limb and interfered with her daily life, and since then thria and dysphagia, which led to the need for a feeding 6% glycated hemoglobin in oral hypoglycemic use, but
there has been progression to the contralateral upper tube. Fourteen days after COVID symptoms started, he after transplantation there was decompensation of gly-
and lower limbs. In addition, she initiated movements was transferred to the Hospital, for supplemental oxy- cemia, attributed to the use of corticosteroids, with the
described as “muscle spasms”, which were often painful. gen, without intubation. The neurologic examination need for insulin therapy. On neurological examination,
In our evaluation, she had increased tone and trophism showed mild somnolence, temporal disorientation, and she presented continuous choreoathetoid movements in
in the left sternocleidomastoid and trapezius and in the generalized myoclonus, with positive and negative jerks, the distal region of the upper and lower limbs on the left.
extensor and flexor muscles of upper limbs. Stereotyped predominantly involving all four limbs, trunk, and proxi- She had a note of blood glucose tests, most of them above
involuntary movements more intense on the left in upper mal musculature. CSF analysis and brain MRI were both 250mg/dL. Glycated hemoglobin was 11.6% and venous
limbs, of low frequency and high amplitude, less evident unremarkable. Under the hypothesis of COVID-associated blood gas was normal. Brain MRI showed mild T1 hyper-
on lower limbs, bilateral oromandibular dyskinesia. Ultra- myoclonus, methylprednisolone (MP) 1000mg/day for five signal in the globus pallidus and putamen on the right.
rapid movements, especially in shoulders and arms. No days was used, with mild improvement of dysphagia and, The diagnosis of hemichorea secondary to a non-ketotic
other alterations on neurological examination. MRI and to a lesser extent, dysarthria. Immunoglobulin IV 0,4g/ hyperosmolar hyperglycemic state was established and
laboratorial tests were normal. Electroneuromyography Kg/day for five days was used with improvement in walk- the patient was managed with intensive diabetes control
showed postural and intention tremor, bilateral and ing, confusion, and myoclonus. He was discharged with and haloperidol 2.5mg orally twice daily. There was signif-
asymmetric, predominance in the left upper limb and Levetiracetam, Clonazepam, and Valproate. He gradually icant improvement in the following days and the patient
frequency of 6Hz. Subsequently, Levodopa+Benserazide improved, weaning off his medications over the following was discharged with almost no symptoms. Discussion:
100/25mg tree doses daily was started, with substantial months. He was released from the outpatient service with- This case describes the clinical presentation and imag-
improvement in symptoms. Discussion: Myoclonus- out any prescription, remaining without myoclonus and ing findings of hemichorea in a non-ketotic hyperosmo-
dystonia (M-D) is an autosomal-dominant movement with complete recovery of motor and cognitive function. lar hyperglycemic state. This condition is relatively rare,
disorder caused by mutations in Epsilon-Sarcoglycan Discussion: Myoclonus is a condition with multiple eti- with an estimated prevalence of 1 in 100,000 people, and
(SGCE) gene, located on chromosome 7q21. The onset ologies and topographies, most commonly secondary to it occurs more frequently in women with uncontrolled
of symptoms usually occurs in the first decade of life. It medical illness. SARS-Cov-2 potential to cause myoclonus type II diabetes. Brain MRI typically demonstrates a
manifests with myoclonus that most often affect the trunk, has been explored in recent reports and probably shares hyperintense signal in the putamen and caudate nuclei
cervical region and upper limbs; and dystonia also in the mechanisms with other viruses under the hypothesis of on T1-weighted images, usually more prominent on the
upper limbs. There is a description of improvement in an immune-mediated condition. Other myoclonus causes side contralateral to the hemichorea. Proposed etiologies
motor symptoms after alcohol libation. The diagnostic were extensively sought but were dismissed. The patient include petechial hemorrhage, temporary ischemia (with
criteria proposed by Grünewald et al. (2008) classify M-D, didn’t undergo severe hypoxia nor use myoclonus-induc- or without hyperviscosity), or decreased synthesis of
according to the presence of family history and classic ing drugs, and there were no metabolic disorders. Final GABA and acetylcholine secondary to metabolic changes.
symptoms, as Definitive, Probable or Possible. Drugs that Comments: In this case, we present an unusual manifes- Treatment is based on blood glucose control, but chorea
should be considered for treatment are benzodiazepines tation of a severe generalized myoclonic encephalopathy may continue for months after hyperglycemia resolves
and anticholinergics, including in combination. Several due to COVID. Some limitations should be cited: despite and require specific medication. Final Comments: The
other drugs need more robust studies. Final Comments: the significant improvement, it’s unclear whether this present case illustrates a condition that, although rare, is
M-D’s definitive diagnosis is limited by unavailability of was a result of immunotherapy or the natural evolution treatable and, therefore, must be recognized. A thorough
genomic sequencing in most services. There is still a lack of this poorly understood disease; finally, EEG and EMG physical examination and compatible clinical history and
of therapies that change the prognosis of a disease that weren’t performed due to logistical difficulties aggravated imaging are essential tools for diagnosing and treating
leads to significant functional impairment. And, in this by the pandemic. metabolic chorea. In this case of symptoms secondary to
sense, Levodopa may appear as a viable option, some- diabetes decompensation due to corticosteroid use after
thing little described in the literature. transplantation, glycemic control and central dopaminer-
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XXX Congresso Brasileiro de Neurologia

TL 1105619 TL 1105629 TL 1105710

PARKINSON’S DISEASE ASSOCIATED WITH WILSON’S DISEASE: PROGRESSIVE CORTICOBASAL DEGENERATION


PARK7 MUTATION: CASE PRESENTATION OF DYSTONIC PRESENTATION DIAGNOSTIC CHALLENGES IN A PATIENT
CLINICAL ONSET AT 20-YEARS-OLD WITH WERNICKE-MANN’S POSITION
Kássia Braga Canzian, Larissa Baccoli de Souza,
Bruno Camporeze, Camila Carneiro Ferreira, Luiza Vanessa de Freitas Moreira, Guilherme Cristianini Matheus Nunes Ferreirinha Leite de Castro, Priscila
Gonçalves Fraga, Ludmila Machado Lima, Thaís Baldivia, Marcella Canato Toloi, Isabela de Almeida dos Santos Mageste, Mariana Soares de Freitas
Takamura, Elora Sampaio Lourenço, Marcela Stella, Roberta Arb Saba, Sônia Maria Cesar de Tamy, Catarina Sodre de Castro Prado, Gustavo Silva
Ferreira Cordellini Azevedo Silva Schafascheck, Raimundo Marcial de Brito Neto,
Isadora Versiani de Lemos, Viviane Alexandre Silva,
Instituto de Neurologia de Curitiba. Curitiba PR, Hospital do Servidor Público Estadual de São Paulo.
Jano Alves de Souza
Brazil São Paulo SP, Brazil
Universidade Federal Fluminense. Niterói RJ, Brazil
camilasilcarneiro@gmail.com kassiacanzian@gmail.com
matheus118@hotmail.com
Case Presentation: Female patient, at 20 years, presented Case Presentation: A 16-year-old male patient, previ-
with rest tremor in right hand, evolving during 5 years ously healthy, presented with involuntary movements Case Presentation: A 52 year-old male with a prior history
with continuous tremor, worse in right side, associated that started 2 months ago in the 5th finger of the left hand, of hypertension and well controlled epilepsy was admit-
with walking difficult. Approximately 2 years ago, the later compromising the entire hand. He had non-painful ted to the outpatient neurology department with severe
patient refers the onset of vocal disturb. The patient was sustained contractions, that occurs at rest, in action and pain and rigidity in the right upper limb. The symptoms
admitted in our service in 2022 presenting spastic and worsened with stress. One month after the onset of the had an insidious onset and gradual progression over the
hypophonic speech, severe bradykinesia and postural condition, he developed cervical tremor, facial contrac- course of 2 years, assuming a Wernicke-Mann position.
instability associated with high-frequency, moderate tions and speech alteration. There were no family history The patient reported depressive mood, irritability and
amplitude tremor, with right hand intention tremor and for neurological diseases. On neurological exam he had self-injurious behavior mainly against his right arm. On
bilateral kinetic tremors of the hands. The patient was sub- mild hypotonia, dystonia of the left upper limb, cervical neurological examination, hemiplegic gait, allodynia in
mitted to neuroimaging investigation of brain and cervical and facial, cervical tremor that worsened with rotation the right upper limb, weakness and hypertonia in the
spine, as well as metabolic screening, with no pathological of the head to the right, and alteration in speech due to right side were observed. Evaluation of the affected limb
findings. The patient was submitted to Levodopa over- muscle contraction (dystonia) of the facial/orobuccal mus- was limited by the patient’s pain. Brain MRI was normal
load test, showing objective improvement in symptoms, cles. Ophthalmological evaluation showed the presence without noticeable vascular lesions that could explain the
and subsequentely to genetic investigation, that showed of Kayser-Fleischer rings. MRI revealed hypersignal in motor symptoms. He was started on citalopram and was
PARK7 mutation. Initial treatment with levodopa at 400mg the head of the caudate nucleus and putamen bilaterally. referred to psychiatry that suspected of conversion disor-
daily developed orofacial dyskinesias, so long-acting Electroneuromyography showed dystonic movements der. Over the next years, he presented with worsening of
Pramipexol was started, with significant improvement of the left upper limb. Laboratory tests: hepatic function the cognitive symptoms with memory impairment, ideo-
of bradykinesia, vocalization and postural instability. without alterations, ceruloplasmin < 9 (21-53mg/dl), motor apraxia and mild dysarthria and developed loss of
Discussion: Although it is known that the majority of serum copper: 32 (70-150 µg/dl). Discussion: Wilson’s balance and bradykinesia in the four limbs, unresponsive
PD is a combination of complex genetic susceptibility disease is an autosomal recessive genetic disorder of to Levodopa. A new MRI showed mild corpus callosum
and environmental factors, around 5–10% of PD may be copper metabolism, resulting from the mutation in the atrophy, leading to the hypothesis of corticobasal degen-
attributed to monogenic forms. Mutations in the PARK7 ATP7B gene that encodes ceruloplasmin (transmembrane eration (CBD). Discussion: CBD is a rare neurodegener-
gene are a rare cause of autosomal recessive PD, with at copper transport protein), leading to the accumulation ative disease characterized by intracellular accumulation
least 20 mutations in its gene identified. The majority of the metal in the liver, brain, cutaneous tissues, as well of misfolded tau proteins which presents as an asymmetric
(83%) of PARK7-linked PD cases have a clinical onset as in the cornea (Kayser-Fleischer rings). Typically man- rigid-akinetic parkinsonism with dystonia and cortical
between the second and third decades of life, whereas ifesting by early adulthood, characterized by tremor, dys- deficits. Frontal behavioral-spatial syndrome, nonflu-
4% have late onset and 13% have juvenile onset, and the tonia, chorea, parkinsonism, cerebellar and psychiatric ent/agrammatic variant of primary progressive aphasia
youngest youngest case presented was in a 5-year-old symptons, liver disease (tends to be seen in early-onset and progressive supranuclear palsy (PSP) syndrome are
patient. Clinically, PARK7 mutations has been described presentations). MRI shows hypersignal on T2 and hyp- also possible phenotypes of this disorder. Similarly, mul-
as a levodopa-responsive parkinsonism with early or even osignal on T1 in the basal ganglia, thalamus, midbrain tiple etiologies may mimic its symptoms, namely PSP,
juvenile onset and slow progression. In contrast to others (face of the giant panda sign), pons and cerebellum. Alzheimer disease, Pick disease and dementia with Lewy
neurodegenerative diseases that manifests itself at such Laboratory tests show reduced levels of ceruloplasmin, bodies. This overlap represents a challenge to the diagnosis
a young age, autosomal recessive PD caused by PRKN, normal or increased serum copper, urinary copper >100 of CBD with an antemortem prediction in only 25 to 56%
PINK1, and PARK7 mutations typically combines young g/24h. The gold standard for diagnosis is liver biopsy. of cases. Also, as seen in this case, symptoms like dystonia
age at onset with very slow progression, as illustrated Treatment is based on drugs that chelate or reduce cop- and pain may hinder the assessment of apraxia and bra-
by our current case. Final Comments: Although gene per absorption. Final Comments: In this case, the patient dykinesia. In this context, new biomarkers are important
mutations in PD are rare, atypical cases in young patients showed predominantly neurological involvement, with to distinguish similar etiologies. Unfortunately, their high
with adequate diagnosis implies in significant recovery the presence of Kayser-fleishcer rings without hepatic/ cost is impracticable for use in the general population,
in quality of life. Furthermore, studies of the genetic and psychiatric involvement. Progressive dystonic condition especially in developing countries. Final Comments:
neuropathological features of rare genetic forms of it has was observed with early diagnosis based on laboratory and Misdiagnosing CBD is frequent and can lead to harmful
been described PARK7 as potentially key role in new ther- neuroimaging alterations. Early diagnosis is important, and unnecessary interventions. Even without a specific
apeutic targets and interventions. and appropriate treatment should be promptly initiated treatment, the prognostic perspective to the patient and its
to prevent progressive and irreversible damage. family is invaluable. Also, it is important for neurologists
to be able to distinguish CBD from its mimics because of
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XXX Congresso Brasileiro de Neurologia

TL 1105716 TL 1105740 TL 1105744

EXCESSIVE SMILE CAN BE WILSON DISEASE TETRABENAZINE USE IN TREATMENT OF ATYPICAL PRESENTATION OF THE CHOREA
MOVEMENT DISORDERS IN ANTI-NMDAR ASSOCIATED WITH PRIME DIAGNOSIS OF
Guilherme Machado Vieira, José Guilherme Martins
Lima, Gabriela de Paula Abranches, Luis Henrique ENCEPHALITIS – A CASE PRESENTATION DIABETES: A CASE PRESENTATION
Sunderhus de Oliveira, Gabriel Marim Roni, Gustav Paula Fiuza Rodrigues Medeiros, Victoria Veiga Luana Larisse de Sousa Araújo, Denise Maria
Barbosa Falcão, Kezia de Souza Pinheiro, Raphael Ribeiro Gonçalves, Paula Baleeiro Rodrigues Silva, Meneses Cury Portela, Gustavo Sousa Noleto,
de Paula Doyle Maia, Mariana Lacerda Reis Grenfell Pedro Vinicius Brito Alves, Luiz Gustavo Brenneisen Natália Rebeca Alves de Araújo, Lia Leal Laurini,
Universidade Federal do Espírito Santo – Vitória – Santos, Nathalia Watanabe, Eduardo Genaro Antônio Levi Farias Borba, Silmara Ferreira de
ES – Brazil Mutarelli, Tarso Adoni Oliveira, Anna Beatriz Feitosa Couto, Maria Luiza de
Centro Universitário Multivix – Vitória – ES – Brazil Sousa Andrade, Maria Alice Silva Vasconcelos
Hospital Sírio Libanês. São Paulo SP, Brazil
gmachado98@outlook.com UESPI. Teresina PI, Brazil
paulafrmedeiros@gmail.com Centro Universitário Uninovafapi. Teresina PI, Brazil
Case Presentation: A 22-year-old male patient with no Hospital Universitário da Universidade Federal do
Case Presentation: 14-year-old female presented with Piauí. Teresina PI, Brazil
previous disease with a 6-month history of deep sadness, acute behavioral changes, confusion, excessive worry
anhedonia and recurrent crying episodes. Three months Centro Universitário Uninovafapi. Teresina PI, Brazil
and short-term memory impairment. Later on, she exhib- Centro Universitário UniFacid. Teresina PI, Brazil
ago, he presented with dysarthria, dysphonia and dys- ited manual automatisms, fever and seizures. CSF exam
phagia, losing 6 kg since then. Neurological examination showed 75 cells with lymphomonomorfic predominance lu123luanalarisse@hotmail.com
evidenced an excessive and maintained smile, despite and normal glucose. Intravenous acyclovir was prescribed.
the depressive symptoms, associated with difficulty in She was intubated after an episode of bronchoaspiration Case Presentation: A 71-year-old female patient pres-
opening the mouth, mild instability of gait and bradyki- and still showed continuous ocular, facial and limb dyski- ents with choric movements in the left hemibody that
nesia predominantly on the left side, without rigidity or nesias and dystonic posture, tachycardia and fever after has initiated 3 years before after a hyperglycemic episode
tremor. Magnetic resonance evidenced extensive signal sedation. She was diagnosed with encephalitis anti-NMDA diagnosed as Diabetes Mellitus (DM). This diagnosis of
alteration spreading through thalamus, pons, putamen, based on typical clinical presentation and positive CSF DM happened after an episode of impaired strenght of the
caudate nuclei and a questionably alteration of globus antibodies. Brain MRI with mild hypersignal in FLAIR and lower limbs, mental disorder and whole body paresthesia,
pallidus, in addition to volumetric loss of basal nuclei contrast enhancement in encephalic cortical sulci. FDG- that has led her to seek for an emergency servicewhere it
and cerebellum. Serum ceruloplasmin was 3,6 mg/dL PET scan showed diffuse asymmetry of cerebral metabolic was diagnosed the hyperglycemic condition (522mg/l).
(normal: 20,0 – 60,0 mg/dL) and urine copper was 184,8 activity, with increased metabolism in the basal frontal Head CT scan and the other laboratorial exams showed
(normal: < 80 mcg/dL). Slit-lamp examination revealed cortex specially on the right hemisphere and marked no changes. She was Hospitalized for one week and after
Kayser-Fleischer ring and the diagnosis of Wilson Disease deficit of metabolism in the bilateral occipital cortex. a glycemic control she maintained outpatient treatment
was established. Discussion: This report highlights that Electroencephalogram showed extreme delta brush pat- with metformin. Despite of the treatment of the high
excessive smile can be the major clue to Wilson Disease tern, without epileptic paroxysms. Tumor screening with serum glucosis, the patient maintained choric movement
(WD) at initial presentation. The association of this sign pelvic MRI and full body pet scan was negative. Treatment lasting more than three years. She used Aripiprazole, that
with depression, bulbar symptoms, bradykinesia and with methylprednisolone 1g/daily for 5 days and intrave- was switched to Risperidona and after that it was noticed
unstable gait in a young patient lead to the hypothesis of nous immunoglobulin (IVIG) was initiated. Dyskinesias, significant improvement of chorea. Discussion: The cho-
WD that was confirmed with laboratorial tests and oph- dystonic posture and dysautonomia persisted. There rea is a hyperkinetic movement disturb presenting rapid
thalmological examination. Abnormal face expression was no symptomatic response to oral benzodiazepines, and unpredicted muscle contractions. It affects mainly
is a common presentation of dystonia in WD, but can ketamine or clonidine relying on IV benzodiazepines. the distal portion of the limbs, besides the face and the
vary in its appearance, ranging from classical presenta- Initiation of tetrabenazine made significant improvement torso and can be classified as hereditary, as in Huntington
tions to more unspecific cases, where a smile can lead of movement disorders, which resurface upon drug half- Disease, or acquired, when associated with other etiologies
to a differential diagnosis with psychiatric diseases or time clearance. Discussion: Anti-NMDAr encephalitis is as traumatic brain injuries, drugs and metabolic changes
pseudobulbar palsy.The classic manifestations of facial characterized by limbic encephalitis, fever, seizures and (hyperglycemia among others). According to literature,
dystonia in WD are “risus sardonicus”, a forced smile due dysautonomia. Movement disorders are present in 75% metabolic features appears as second main etiology of
to dystonic spasm of the risorius muscle, and “vacuous of young children and 95% of adults, appearing as oral, acute chorea, present in 30% of all cases. Generally there
smile”, an open-mouth smile due to dystonic dropped facial or lingual dyskinesias, dystonia, chorea, athetosis, is slow remission of the chorea after glycemic control
jaw. Excessive smiling, elicited to trivial stimuli is also myorhythmia, opisthotonus, ballismus, blepharospasm and abnormal movements may last for more than one
described. Recognize these signs is important, not only and oculogyric crisis. Immunotherapy generally improves year. Final Comments: This case shows the association
for diagnosis, but also because it interferes with eating, this movement disorders. Benzodiazepines, valproate, hal- between an acute choric event after hyperglycemia that
speech and increase mortality of the patients, needing operidol, ketamine and propofol have been reported for remains even after glycemic stabilization, reinforcing the
correct management. Final Comments: Identify the myr- symptomatic treatment for refractory cases. Tetrabenazine non-remission of the symptoms acutely after the etiolog-
iad of signs and symptoms of WD is essential, once it is a is a reversible inhibitor of the human vesicular monoamine ical treatment. Furthermore, it is relevant to perform an
potentially treatable disorder, especially if early diagnosed. transporter type 2 (VMAT-2) with scarce data about use in adequate etiological investigation in order to have early
The facial manifestations include “risus sardonicus”, “vac- anti-NMDA encephalitis. The case presented presented recognition of the cause and prompt treatment avoiding
uous smile” or excessive smile. In the context of a young time-related and dose-related improvement of dyskine- additional complications.
patient and associated with suggestive symptoms, these sias and dystonia with tetrabenazine. Final Comments:
signs must lead to the appropriate investigation. Tetrabenazine is shown as a possible alternative symp-
tomatic treatment to high doses of benzodiazepines Transtornos do Movimento
with less adverse effects in movement disorders of anti-
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NMDAr encephalitis.

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XXX Congresso Brasileiro de Neurologia

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WILSON DISEASE AFTER A TRAUMATIC ACUTE DYSKINESIA AND STROKE IN A MACHADO-JOSEPH DISEASE AGGRAVATED
INJURY: A CASE PRESENTATION PATIENT WITH THYMIC CARCINOMA BY HIV THERAPY ABANDONED AND
COVID-19 INFECTION: CASE PRESENTATION
Victor Fellipe Bispo Macedo, Djanino Fernandes Pollyana Marcela Silva de Farias, Kathleen Torres
Silva, Paulo Barbosa Leite Neto, Marco André Tenório Monteiro, Letícia Pires Ottoni, Marcos Victor Fellipe Bispo Macedo, Larissa Clementino
Moraes Bernardino, Larissa Clementino Leite Sá Ravi Cerqueira Ferreira, Yuri Silva Macedo, Camila Leite Sá Carvalho, Djanino Fernandes Silva,
Carvalho, João Eudes Magalhães, Ana Rosa Melo Castelo Branco Pupe, Ana Carolina Andorinho de Paulo Barbosa Leite Neto, Marco André Moraes
Correa Lima, Elzeleide Souza Vasconcelos, Fabíola Freitas Bernardino, João Eudes Magalhães, Ana Rosa Melo
Lys Medeiros Correa Lima, Elzeleide Souza Vasconcelos, Carlos
Pontifícia Universidade Católica do Rio de Janeiro.
Frederico Leite Souza Lima, Fabíola Lys Medeiros
Hospital Universitário Oswaldo Cruz, Universidade Rio de Janeiro RJ, Brazil
de Penambuco. Recife PE, Brazil Hospital São Lucas Copacabana. Rio de Janeiro RJ, Hospital Universitário Oswaldo Cruz, Universidade
Brazil de Penambuco. Recife PE, Brazil
victorfmacedo@gmail.com Universidade Federal Fluminense. Rio de Janeiro
RJ, Brazil victorfmacedo@gmail.com
Case Presentation: A 23-years-old male suffered trau- Hospital Federal dos Servidores do Estado
matic injury at 2019, he was beaten until he passed out Case Presentation: 45 years-old female, presented with
needing to go to an emergency room and after a month med.pollyanasilvadefarias@gmail.com incoordination and drunken gait for 15 years, with pro-
presented a symmetric, progressive and slowly weakness gressive worsen along the years. She has positive familiar
in lower limbs, movement disorders in hands and, pos- Case Presentation: An 80-year-old woman under chemo- history of spinocerebellar atrophy type 3 (SCA-3) with pos-
teriorly, dysphagia, dysarthria and loss of trunk control, therapy for thymic carcinoma and no history of movement itive genetic test in father and sister. Her genetic test for
being restricted to bed after 3 years of the disease onset. disorder (MD) or antipsychotic use evolved with fever SCA-3 was also positive. After multiple pharmacological
Parental consanguinity (1th degree cousins) and similar and focal seizure at home. Once admitted at emergency attempts, reached the symptomatic control with risperi-
family history negative. Had abuse of marihuana, cocaine room, she presented a secondarily generalized seizure that done, baclofen, buspiron and clonazepam, restoring her
and crack. On physical examination, alert, thin, exophoria led her to orotracheal intubation. Phenytoin was started functionality for daily basic activities. On September 2021,
(left eye), symmetric reduced lift of soft palate, dysarthria in parallel to piperacillin/ tazobactam (for pneumonia) she has evolved with worsen of the ataxic condition and
and dysphagia. Symmetric cogwheel hypertonia on upper and acyclovir (for viral encephalitis). She was extubated weight loss, even with regular medication use, becoming
limbs and spasticity (Ashworth 1) on lower limbs. Force- and upon evaluation on 3 rd day she was torporous, bedridden. Thus, she was admitted for investigation and
MRC V. Present axial face reflexes, normal osteotendinous obeyed simple commands despite plegic right hand and clinical compensation. On neurological exam alert, com-
reflexes and bilateral Babinski sign. Exhaustible Achilles had orolingual dyskinesia and choreic movements of municative, thin, horizontal gaze decomposition, severe
Clonus. Apraxic and ataxic gait. Index-nose maneuver lower limbs. MRI disclosed left frontal leptomeningeal dysphagia, severe hight amplitude tremor in limbs and
with bilateral movement decomposition. Dystonia and hypersignal with frontoparietal cortical hypersignal and head, movement decomposition, dysmetria, dysdiado-
myoclonus in hands were presented. Eyes inspection restricted DWI in hand motor area. CSF revealed 373 cells chokinesis, scant speech and positive Babinski torso-thigh
with dark brown halo on irises. Brain MRI revealed supra (73% lymphomononuclear), 102mg/dL protein and no test. She had symptoms of a respiratory infection, with
and infratentorial atrophy, hypersignal foci (T2-weighted neoplastic cells. On 4 th day, CSF PCR and hemoculture laboratory positive for COVID-19 e HIV infections. She
sequence) in frontal periventricular white matter, subinsu- were positive for Listeria monocytogenes and ampicil- claimed to know of HIV infection since 2011 and aban-
lar, claustrum, cerebral peduncles, midbrain tegmentun, lin was started. The hyperkinetic movements remitted doned the treatment nearly 12 months before the ataxic
pons and middle cerebelar peduncles, diffusion restric- in the 2 nd week of treatment but right hand remained decompensation. Brain RMI ruled out opportunistic
tion on splenium of corpus collossum and hemoglobin plegic. Patient recovered conscious level but died after 3 infeccion in CNS and showed moderate atrophy of pons,
breakdown products on caudate and lentiform nucleus. months of internment. Discussion: Listeriosis incidence cerebellum and middle cerebelar peduncles, with T2/
Laboratory showed reduced ceruloplasmin level (5mg/ is around 3-6 cases/million/year globally, although the FLAIR hypersignal in the same location. After an initial
dL) and elevated 24 hour urinary copper (239.7µg/24h). prevalence is higher in the elderly and immunocom- improvement of the respiratory infection, she evolved
Liver ultrasound showed steatosis and eye evaluation con- promised patients, and the mortality rate is about 30% with cardiorespiratory arrest in pulseless VT for 26 min-
firmed Kayser-Fleisher ring. Thus, it was consistent with whether neurological involvement. In a Chinese system- utes, returned in coma vigil and died after a few weeks
Wilson’s disease (WD) and was started D-penicilamine atic review, dyskinesia was mentioned only in 3 cases in the ICU. Discussion: The SCA 3 is the prototype of
with good tolerance. Discussion: WD is a autosomal of 163 non-perinatal patients with listeriosis. In other autossomic dominant spinocerebellar atrophies. It stems
recessive inheritance disease due a mutation on ATP7B review, just 10 from 139 MRI lesions showed basal gan- from the CAG expansion in the ATXN3 gene in chromo-
gene on cromossome 1, leading to an accumulation of glia affection, and no dyskinesia was cited. We report an some 14. There is no specific treatment, and symptomatic
copper in the body, including the central nervous sys- elderly oncologic patient with meningoencephalitis and management being performed. The decompensation is
tem. Triggers are not usually found, but recently has been no clear radiological substrate for hyperkinetic MD that common in systemic abnormalities, such infection. Final
described WD onset after traumatic injury. In our opin- evolved with acute orolingual dyskinesias and LL chorea Comments: We emphasize that atypical exacerbation of
ion, it is difficult to separate the direct effects of trauma that remitted during ampicillin employment. Moreover, spinocerebellar atrophy disorder is linked principally to
from those resulting from neurodegenerative processes, the ischemia endorses the role of meningoencephalitis infections, and their control is essential in the prognosis
but some degenerative diseases are being initiated after in vasculitic thrombotic infarction. Differential diagnosis and survival of patients with neurodegenerative diseases.
trauma, without a clear explanation. Final Comments: would be a paraneoplastic syndrome (PS). An example of
The recognition of this relationship between traumatic possible PS is anti-NMDA encephalitis that may present
injury and WD onset is still poorly described. We alert with dyskinesia but is not associated to thymic neoplasms Transtornos do Movimento
that early diagnostic recognition and treatment for WD, (TN). Besides, thymic carcinomas, unlike thymoma, is not
is essential for the best outcome of the patient, even with related to PS and dyskinesia was solved after ampicillin
unlikely triggers and diagnostic confounders. use. Final Comments: We present a patient with thymic
carcinoma undergoing chemotherapy who developed
dyskinesia secondary to a neuroinfection. Despite MRI
Transtornos do Movimento findings being isolated to frontal lobe, the MD allied to the
cortical stroke suggest a diffuse vasculitis due to Listeria
meningoencephalitis.

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XXX Congresso Brasileiro de Neurologia

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GPI DBS IN PATIENT WITH DYT28 NBIA-5 WITH WDR45 GENE MUTATION – CASE OF GENERALIZED MYOCLONIAS
ASSOCIATED WITH KLIPPEL-FEIL CLINICAL AND NEUROIMAGE FINDINGS CAUSED BY SEVERE HYPONATREMIA
SYNDROME: A ONE YEAR FOLLOW-UP CASE INDUCED BY TOPIRAMATE
Deise Andrade Melo, Sandro Adriano Souza Lima
PRESENTATION Junior, Diana V. Brito, Maria Eduarda Alencar Santos, Assíria Leite de Azevedo Costa, Carlos Eduardo
Denise Maria Meneses Cury Portela, Natália Rebeca João Pedro Moreira Guilherme, Victoria Rosas Cordeiro Cavalcante, Isadora de Castro Ferreira de
Alves de Araújo, Gustavo Sousa Noleto, Matheus Marques, Marcus Vinicius Della Coletta Oliveira, Joana Clara Oliveira Macedo Lima, Kelson
Rodrigues Côrrea, Marcos Vinícius Bertoldo Gomes, James Almeida
Universidade Federal do Amazonas. Manaus AM,
Luana Larisse de Sousa Araújo, Janine Lemos Mel Brazil Universidade Federal do Piauí. Teresina PI, Brazil
Lobo Jofili Lopes, Lais Machado de Oliveira, Carlos Universidade do Estado do Amazonas. Manaus AM, UESPI. Teresina PI, Brazil
Daniel Miranda Costa, Francisco José Alencar Brazil
Universidade Nilton Lins. Manaus AM, Brazil assirialeite@hotmail.com
Centro Universitário Uninovafapi. Teresina PI, Brazil
Universidade Estadual do Piauí – UESPI. Teresina mcolletta@uea.edu.br Case Presentation: Female, 73 years old, rural worker,
PI, Brazil
admitted to the movement disorders clinic for investi-
Hospital Universitário da Universidade Federal do Case Presentation: We report the case of a female 33 years gation of generalized and progressive myoclonus since
Piauí – HU UFPI. Teresina PI, Brazil old who developed seizures at eight months of age, being 2016 with cognitive decline, and worsening 1 year ago.
Universidade Federal do Piauí. Teresina PI, Brazil treated with anticonvulsants suspended at one year and The movements started in the foot or hand and spreading
Departamento de Neurologia, Instituto Norman eight months of life. In the past pathological history, she to the other limbs, sparing the face. It started only in the
Fixel para doenças neurológicas, Universidade da had delayed development, gait, and speech. At school, pre- left upper and lower limbs, but progressed to the right
Flórida – Estados Unidos sented cognitive development delay without other motor hemibody. The myoclonias lasted up to 3 days, including
Divisão de Neurologia, Departamento de Medicina, alterations. At 32, she complained of sleep disorders and during sleep, but with decreased intensity. She had type
Universidade de Alberta – Canadá significant bilateral parkinsonism, treated as a Dandy- 2 diabetes mellitus and systemic arterial hypertension
Hospital Infantil Lucídio Portela. Teresina PI, Brazil Walker variant with parkinsonism. Initially, she had an and was using topiramate, chlorthalidone, valsartan+hy-
nataliarebeca2607@gmail.com excellent response to levodopa. In the following months, drochlorothiazide, metformin, biperiden, acetylsalicylic
she developed headaches, psychiatric involvement with acid, and simvastatin. On neurological examination, she
Case Presentation: A 14-year-old female with history depressive mood, and a worsening of the neurodegenera- presented bilateral tabetic gait, inability to perform tan-
of congenital scoliosis, Klippel–Feil Syndrome (KFS), tive condition with an intensification of parkinsonism, cer- dem gait, and bilateral anarthesia. Dysmetria and decom-
Sprengel’s anomaly and generalized dystonia status vical dystonia, cognitive deterioration, and disturbances in position in the index-nasal and index-finger in the upper
post bilateral Globus Pallidus Internus (GPi) Deep Brain sleep cycles. The Complete Exome sequencing identified limbs were also evident. Ideative and ideomotor apraxia
Stimulation (DBS). Patient was diagnosed with congen- a heterozygous mutation in the WDR45 gene, confirm- and visuospatial dysfunction. During the investigation,
ital scoliosis, KFS and Sprengel’s anomaly at age 1. At ing the diagnosis of neurodegeneration with brain iron severe hyponatremia (serum Na of 104) was found, and
age 6, she developed focal left lower extremity dystonia, accumulation type 5 (NBIA5). The patient was submitted the patient became drowsy; intravenous NaCl 3% replace-
that progressed to trunk, both upper extremities and to a brain MRI that was considered normal. However, a ment was performed. The main diagnostic hypothesis
oromandibular region over the years. Patient was poste- posterior neuroimaging analysis revealed a hypersignal was a syndrome of inadequate ADH secretion (SIADH)
riorly diagnosed at age 11 with KMT2B-related dystonia on T1 and hyposignal on T2 in the substantia nigra, typ- caused by topiramate. A liquor analysis revealed an acel-
(DYT28) associated to KFS. It was confirmed by genetic ical findings of NBIA5. The patient still has progressive lular pattern, with normal glucose and protein. During
testing that revealed microdeletion on chromosome 19 balance worsening, difficulty in gait, cognitive decline, the investigation of the myoclonus picture, the electro-
between the 19q13.11q13.12 bands, consistent with the parkinsonism, and cervical dystonia. Discussion: NBAI5 encephalogram (EEG) results were awake and drowsy
cytogenetic location of KMT2B (OMIM 606834). She was is a group of inherited neurodegenerative diseases char- without abnormalities and the magnetic resonance imag-
whel-chair bound for most part of her day, presented with acterized by iron deposition in the basal ganglia. Patients ing (MRI) of the skull showed no relevant alterations. At
severe spasmodic dysphonia and responded partially to with mutations in the WRD45 gene are characterized by clinic follow-up, after correction of hyponatremia and
levodopa. She underwent bilateral GPi DBS at age 13 and delayed development in early childhood, mainly in lan- suspension of topiramate, there was improvement of
returned to our clinic for a 1-year postoperative follow guage and motor skills, followed by a period of stability movements. Discussion: SIADH has many etiologies,
up, presenting a 22% improvement in the Burke-Fahn- and later neurodegenerative manifestations. As in the through physiological, pathological or drug alterations.
Marsden Dystonia Rating Scale when compared to pre literature, the patient showed an excellent response to In particular, oxcarbazepine and carbamazepine act in
operative assessment, especially in neck, trunk and lower levodopa in the initial adulthood period, but these effects part by increasing sensitivity to ADH. Similarly, it was
extremities. We also noticed a significant improvement were of short duration. NBIA5 syndrome still lacks data observed in a control study that older patients taking the
on gait, which was reflected in a subjective improvement in the literature on the subject. Final Comments: Based anticonvulsants valproate, phenytoin, and topiramate had
of functionality and quality of life (QoL). Discussion: on the case presented and the literature review, it is sug- an increased risk of Hospitalization for hyponatremia, as
Mutations in KMT2B were recently associated with an gested that NBIA5 be inserted as a differential diagnosis observed in this case. Therefore, hyponatremia should
early-onset, progressive and often complicated dystonia for neurological disorders in patients with developmental be noted as a possible etiology of metabolic myoclonus,
(DYT28), commonly presenting with an initial lower-limb delay in childhood and parkinsonism in early adulthood. among others such as hepatic encephalopathy and ure-
dystonia that progresses to generalized dystonia. Among We present the typical neuroimage findings that must mia. Final Comments: Authors describe a rare case of
generalized early-onset dystonias, DYT28 may account bring to mind this rare diagnosis. SIADH caused by topiramate, a drug poorly correlated
for up to 10% of the cases. DBS is indicated for patients as a cause of hyponatremia in the medical literature.
with generalized or segmental dystonia, refractory to Hyponatremia resulting from this disorder can lead to
Transtornos do Movimento severe consequences, such as movement disorders, which
drug treatments. This surgery improves about 42% of the
clinical conditions in DYT-KMT2B cases, significantly incapacitate for daily activities.
improving motor symptoms. KFS is a congenital syndrome
marked by the fusion of two or more vertebrae. It may be
associated with other disorders, but the co-occurrence of Transtornos do Movimento
typical KMT2B-related dystonia has not been reported
in association with KFS to our knowledge. This case was
previously presented by the authors and now we present
the post DBS follow-up. Final Comments: This is a patient
status post GPi DBS as stated in the literature, there was a
relevant improvement on patient’s symptoms, especially
on gait. More studies are necessary to better understand
the benefits of this procedure and to get broader and
more concrete results.

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XXX Congresso Brasileiro de Neurologia

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FUNCTIONAL TIC SIMILAR TO TOURETTE’S NEUROLOGICAL WILSON DISEASE IN A LATE CLINICAL MANIFESTATION OF DANDY-
SYNDROME ASSOCIATED WITH DIGITAL YOUNG ADULT: A CASE PRESENTATION WALKER SYNDROME: CASE PRESENTATION
MEDIA CONSUMPTION DURING THE
Laryssa Garcia de Almeida, Yan da Silva Raposo, Tiago Abreu Tempone
COVID-19 PANDEMIC Ilana Werneck Augsten, Hiago Antunis Silva, Patrícia
Marques Mendes Policlínica Central Andradas-MG – Andradas – MG
Rodrigo André de Souza Araújo, Elzeleide Souza – Brazil
de Vasconcelos, Alex de Novais Batista, Ewerton Hospital das Clínicas Samuel Libânio. Pouso Alegre
Emmanuel Soares Silva, Marcílio José de Oliveira MG, Brazil tiagoabreu.med@hotmail.com
Filho, Maria Júnia Lira e Silva, Clauhan Williams
Soares dos Santos, Jonata Ribeiro de Sousa yanraposo@yahoo.com.br Case Presentation: The Dandy-Walkér malformation was
described by Virchow in 1863 and Fusari in 1891. However
Hospital Universitário Oswaldo Cruz – UPE. Recife Case Presentation: A 28-years-old male, mixed race, it was Dandy and Taggart and Walker who described the
PE, Brazil bricklayer, residing in Paraisópolis, Minas Gerais State, clinical and radiological elements necessary for the per-
rodrigo__andre@hotmail.com Brazil. History of alcoholism and use of narcotics. On fect characterization of the syndrome. This is an embry-
December 3rd, 2021, the patient suffered the first gener- onic defect in the posterior fossa with absence from of
Case Presentation: An 15-years-old woman, started body alized clonic tonic seizure while sleeping. Magnetic res- the 4th ventricle, agenesis of the lower part of the cere-
movements six months before the admition in the neu- onance imaging (MRI) of the brain this month detected bellar vermis, very high situation of the lateral sinus and
rology service, after emotional stress with acutely present a brain tumor. Levetiracetam was orally initiated on cerebellum tent, and dilation of the ventricular system.
complex and diverse movements. After one month, she December 21st. He was diagnosed with Covid 19 on A case of late manifestation of the disease is reported.
started nonspecific vocalizations, such as whistling and February 03rd, 2022, but with a benign evolution. The wife Female patient, 44 years old, complaining of insidious
coprolalia, which worsen with anxiety and presence of noticed from February 9th speech and gait disturbance, impairment of evolution of more than 15 years with pro-
self- and hetero-aggressive movements. Such movements difficulty mobilizing the right hand, and urinary inconti- gressive ataxia, dysarthria, scant speech, incoordination.
can be suggested and get worse if suppressed. She started nence. On February 16th, the anticonvulsant was switched Patient with incomplete higher education. Discussion:
risperidone and sertraline. Important consumption of to Phenytoin orally and associated with Dexamethasone Dandy-Walker Syndrome is a rare clinical and radiologi-
digital media. Cranial MRI and electroencephalogram orally. Cerebrospinal fluid (CSF) on March 21th revealed cal disease that most commonly is the subject of study in
did not show any changes that would justify the con- a cell count of 0 units; glucose 89 mg/dL, lactate 15.7 mg/ the pediatric age group. However, this case is presented
dition by secondary causes. Discussion: Tics are sud- dL, gram without staining bacteria, and CSF culture with- with a patient who arrived at the university until the last
den, rapid and repetitive movements or sounds that are out bacterial growth. He was Hospitalized on March 24th year of advocacy. Because the cerebellum is a vital part of
preceded by an urge sensation and can be temporarily for social reasons. The patient presented to the neurolog- movement, balance and body coordination, many people
suppressed. Motor tics can be clonic, dystonic or tonic ical examination risus sardonicus and right-hand flexor with Dandy-Walker syndrome have problems with their
movements and vocal tics are projected mostly as irrele- dystonia and ataxic gait. Magnetic resonance imaging voluntary muscle movements and coordination. They
vant sounds. The tic can be considered complex with more (MRI) of the brain on April 01st, 2022 showed bilateral may also experience difficulties with their motor skills,
elaborate movements like kicks, copropraxia, speech of and symmetrical involvement of the basal ganglia and mood, and behavior, and may have limited intellectual
words or phrases, with coprolalia and echolalia. Tourette’s crus posterius, in addition to the midbrain and pons, and development. About half of people with Dandy-Walker
Syndrome (TS) occur mostly in the pediatric population cerebellar peduncles. T2 FLAIR demonstrates a focus on syndrome have a below-average IQ. Despite the classic
and males. Characterized as motor tics and one or more diffusion restriction without significant expansive effect or symptoms, the 44-year-old G.M.O. patient was evaluated
verbal tics present at some point in the disease. The pan- paramagnetic contrast enhancement. Laboratory workup with a complaint of insidious ataxia, cognitive impair-
demic caused by Covid-19, there has been an worldwide on March 24th revealed total serum copper of 24.6 mg/ ment, nystagmus, incoordination and scanted speech. He
increase in cases of people between 12 and 25 years old, dL, serum ceruloplasmin of 7.0 mg/dL, and, finally, a dropped out of college a few months before completing
mostly female, with a rapid onset and development of 24-hours urine copper test of 187.4 mg/dL which con- it. It observed losses in the performance of its domestic
complex motor or vocal tic-like behaviors, causing emer- firmed the diagnosis of Wilson disease. Penicillamine activities. When, already in his fifth decade of life, he
gency department visits, which was named of Functional was started at low bose, however, he is evolving with neu- sought medical assistance. Magnetic resonance imaging
Tic-like Behaviors (FTLBs). A despite the geographic diver- rological worsening. Discussion: Incipient neurological examination of the brain, at the time of the first visits in
sity, most cases shared common denominators, such as symptoms are subtle and nonspecific and begin on aver- December 2019, revealed Dandy-Walker variant in the
exposure to specific content on online media platforms age between 20 and 40 years. Neurological impairment posterior fossa with limited development of the cerebellar
such as TikTok and YouTube. Associated with the content occurs about a decade after liver failure and, therefore, vermis, right and left side of the cerebellum, enlargement
of TS and FTLBs. As main differences between TS and are signs of advanced disease. Clinical worsening may of the fourth ventricle, development of a large, cyst-like
FTLBs is an absence of progression from initially simple occur after starting Penicillamine. Final Comments: formation at the base of the skull, where the brain stem
to complex tics; less suppression capacity and presence It is a rare disease whose epidemiology in Brazil lacks and cerebelus are located. Final Comments: For most
of psychosocial stressors such as tension between par- data and complementary tests have reduced specificity. people, the signs and symptoms of Dandy-Walker syn-
ents or family members, intensified by the lockdown, in Diagnosis at an advanced stage of the disease compro- drome are evident at birth or in the first year, but 10 to
addition to a higher prevalence with anxiety disorder or mises the neurological prognosis. 20% of individuals may not develop symptoms until late
depression. Final Comments: Therefore, attention to the childhood or early adulthood.
symptoms presented and the chronology of the disease
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are important, in order to avoid the automatic diagnosis
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of TS in patients with motor and verbal tics and also con-
sider the presence of a functional disorder. In these last
cases, modifying inappropriate social media exposures,
psychotherapy, and addressing psychiatric disorders
such as anxiety and depression can all be considered.

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BROWNELL-OPPENHEIMER VARIANT OF ACUTE CHOREA ASSOCIATED WITH STATUS DYSTONICUS IN WILSON DISEASE
SPORADIC CREUTZFELDT- JAKOB DISEASE: THYROTOXICOSIS
Júlian Letícia Freitas, Iara Amorim, Luiza Corazza,
A CASE PRESENTATION IN RECIFE João F Tasch, Paula Camila Alves, Maria Sheila
João Igor Dantas Landim, Lívia Carvalho Figueira,
Victor Fellipe Bispo Macedo, Paulo Barbosa Leite Alessandra Braga Cruz Guedes de Morais, Ian Silva Rocha
Neto, Marco André Moraes Bernardino, Djanino Ribeiro, Morgana Feitosa de Queiroga, Matheus
Hospital Santa Marcelina. São Paulo SP, Brazil
Fernandes Silva, Larissa Clementino Leite Sá Costa Bessa, Noberto Anizio Ferreira Frota,
Carvalho, João Eudes Magalhães, Ana Rosa Melo Fernanda Martins Maia Carvalho, Antonia Rosivalda julian.leticia.freitas@gmail.com
Correa Lima, Elzeleide Souza Vasconcelos, Mylana Teixeira Marinho, Flavia de Paiva Santos Rolim
Dandara Pereira Gama, Fabíola Lys Medeiros Case Presentation: We describe a young woman, born
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
from consanguineous parents, who presented at the age
Hospital Universitário Oswaldo Cruz da Universidade de Fortaleza. Fortaleza CE, Brazil
of 23 years with a 9-month past of anxiety and depression
Universidade de Pernambuco. Recife PE, Brazil and a 2-month history of left upper limb dystonia. She
João igorlandim@hotmail.com
victorfmacedo@gmail.com progressed rapidly and severe generalized dystonia. She
Case Presentation: A 30 year-old with a three-month was admitted to the emergency room with status dyston-
Case Presentation: 61 years-old female, farmer, presented history of progressive generalized hyperkinectics move- icus (video). Laboratory investigation showed elevated
with progressive weight loss (nearly 20 kg in 3 months), ment. She had a prior history of severe anxiety, followed creatine kinase, hepatic dysfunction, and low serum level
ataxic gait, imbalance and falls in October 2020, that asso- by a diagnosis of depression with psychotic symptoms. of ceruloplasmin. Brain MRI demonstrated a bilateral
ciated along 3 months with symmetric tremor in upper The condition persisted even after medical prescription of T2-W hyperintensity in basal ganglia, pons, and midbrain
limbs, visual hallucinations (with animals), behavior antipsychotics. Due to significant clinical deterioration and (figure). Whole-exome sequencing revealed a homozy-
change, persecutory delusion, incoordination, aphasia loss of functionality, the family sought neurological care. gous pathogenic variant in the ATP7B gene, c.547C>T:
non-fluent, urinary incontinence and dysphasia. In the On admission, was agitated, hypervigilant, disoriented, p.(Arg183Trp). Initially, management of dystonic status
end of this period, she became confined to bed and her tearful, feverish, tachycardic and presenting generalized was performed with orotracheal intubation, high doses
family sought medical care, being transferred to a tertiary choreoathetotic movement. She also had exophthalmos. of intravenous benzodiazepine associated with baclofen,
neurology service to investigate encephalitis of unknown After much questioning, family members reported that one amantadine, and gabapentin via enteral tube. Regarding
etiology. Relatives deny infectious symptoms before the year prior to admission, she also had unmeasured hyper- a poor response to the therapy instituted so far, it was
disease onset neither similar cases with closed relatives. thermia episodes, sweating, hair loss, asthenia, significant decided to implement intermittent intrathecal baclofen,
On physical examination, alert, no interaction with the weight loss and low amplitude tremor of the extremities. but the treatment had to be interrupted due to infection of
environment, pupilar reflex slowed down, cogwheel ECG showed atrial fibrillation. Burch and Wartofsky index the central nervous system. Therefore, we chose to perform
hypertonia and spasticity in upper limbs, associated with scored 75 points. Initial exams revealed TSH: < 0.01 and unilateral campotomy as an adjuvant treatment of status
Gegenhalten paratonia. Presence of axial face reflexes, T4L > 6. Thyrotoxic crisis was diagnosed and the patient dystonicus. Discussion: Wilson disease (OMIM #277900)
osteotendinous reflexes increased and indifferent cutane- was treated with propylthiouracil, beta-blockers, and corti- is an autosomal recessive inherited disorder of copper
ous-plantar reflex. Myoclonic tremor (postural and Kinect) costeroids. She also received low doses of haloperidol and metabolism, caused by a mutation in the copper-trans-
in upper limbs and head. Startle reflex. Laboratory without clonazepam to symptomatic control of chorea. Subsequent porting gene ATP7B. The disease can manifest as chronic
abnormalities. Cerebral MRI showed hypersignal in fron- examinations showed TRAB > 40. There were positive liver disease, a progressive neurologic illness, or a psy-
tal cortex, caudate nucleus head, putamen, medial wall rheumatologic markers, but no target organ damage, so chiatric disorder. Movement disorder is one of the main
of the III ventricle and pulvinar of the thalamus (hockey immunosuppressants were not performed. The patient symptoms of neurologic manifestation and dystonia is a
stick sign), with DWI restriction. EEG showed diffuse experienced substantial and progressive improvement of common feature of WD. Dystonia can be focal, multifocal,
disorganization of the base rhythm. CSF showed mild choreic movements after normalization of thyroid func- segmental, or generalized. Status dystonicus is rare and is
pleocytosis, with normal glucose and protein. With the tion. At the follow-up evaluation choreic movements had commonly associated d-penicilamine introduction, but
exclusion of the most common causes of encephalitis, the completely disappeared Discussion: We report a case of Teive et al suggest that this presentation may be part of
main diagnostic hypothesis was Creutzfeldt-Jakob disease a young female patient, with no previous comorbidities, the natural history of this disorder. Final Comments: We
(CJD). Thus, 14-3-3 protein was measured in the CSF, with who presented to the Hospital with generalized choreic report a case of Wilson disease with a rare presentation
positive result. After about a month of Hospitalization, movements due to thyrotoxicosis. She was further diag- of status dystonicus without any treatment introduction.
the patient died of aspiration pneumonia. Discussion: nosed with Graves disease. Chorea is described as a very
CJD is the prototype of prion diseases, with rapid pro- rare manifestation, present in less than 2% cases of thy-
rotoxicosis. Mechanism of chorea in these cases is still Transtornos do Movimento
gression and leading to death in less than a year of onset
the disease. The Brownell and Oppenheimer form starts poorly established. Previous reported cases suggest that
with cerebellar ataxia and dementia during the course it might be related to dopamine effect on basal ganglia,
of the disease. EEG findings of this variant usually reveal and to functional modification of adrenergic receptors.
absence of periodic sharp-wave complexes. Most cases Association with autoimmunity has also been described
are sporadic, as in the case presented, but acquired and Final Comments: In conclusion, we enforce the impor-
genetic forms have already been described. It’s an exclu- tance of considering hyperthyroidism as a main differen-
sion diagnosis and there is no specific treatment. Final tial diagnosis of acute chorea. This etiology is identifiable
Comments: Prion disease must always be considered using simple laboratory tests and has a good response to
in case of rapidly progressive dementia without defined drug treatment. Further studies are needed to elucidate
diagnosis, especially when associated to myoclonus and pathophysiology of chorea related to hyperthyroidism
eventually, ataxia. We emphasize that early diagnosis and autoimmunity
and identification of people in contact with the patient is
essential to prevent the spread of the disease.
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XXX Congresso Brasileiro de Neurologia

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SPORADIC ADULT-ONSET ATAXIA IN A CAPN1 MUTATION ASSOCIATED PAROXYSMAL NON-KINESIGENIC


PATIENT WITH PLATYBASIA AND BASILAR WITH SPASTIC PARAPLEGIA, A RARE DYSKINESIA (PNKD): CASE PRESENTATION
INVAGINATION PROGRESSIVE GAIT DISORDER WITH FEW
Carla Lenita Coelho Siqueira, Gabriel Palomares
REPORTS IN LITERATURE Garcia, Isadora de Oliveira Cavalcante, Lisiane
Conte A Talita, Patrícia Áurea Andreucci Martins
Bonilha, Helio Afonso Ghizoni Teive Luiz Eduardo Novis, João Vitor M Lisboa, Patrícia Seguti Ferreira, José Ribamar Pereira Neto
Aurea Andreucci Martins Bonilha, Thabata Hospital Universitário de Brasília. Brasília DF, Brazil
Universidade Federal do Paraná. Curitiba PR, Brazil
Emanuelle Martins Nunes, Beatriz Casarotti, Leo Universidade de Brasília. Brasília DF, Brazil
patriciambonilha@hotmail.com Coutinho, Francisco Manoel Branco Germiniani, Hospital de Base de Brasília. Brasília DF, Brazil
Helio Afonso Ghizoni Teive
Case Presentation: A 77-year-old man, white, born in carlalenita.med@gmail.com
Universidade Federal do Paraná. Curitiba PR, Brazil
Alagoas, with a negative family history of neurological
disease, came to our observation complaining of gait patriciambonilha@hotmail.com Case Presentation: MCD, 4 months. Parents report that
disturbances since 2020. His medical history included at 2 months of age, their child has been presenting dys-
systemic arterial hypertension, a depressive disorder Case Presentation: Two young patients attended our tonic episodes characterized by forced semiflexion of
and benign prostatic hyperplasia. He reported that his clinic presenting difficulties to walk at the same age, 30 the left upper limb, elevation and hyperextension of the
complaints began after a fall from a height in 1982, when years old, with progressive worsening. A woman, with a right upper limb (posture in swordsmanship), accompa-
he suffered head trauma and that resulted in dizziness history of two ischemic strokes, the first at 24, and later nied by hypertonia of the lower limbs. During episodes,
with progressive worsening associated with gait imbal- with 40 years of age, struggles to walk at 30 years of age, the patient presents pain, facial redness and groaning,
ance. Physical examination revealed enlargement of the associated with weakness in the lower limbs, hyperto- and at the end of the crisis, she demonstrates crying and
anteroposterior skull diameter and downbeat nystagmus, nia, signs of pyramidal release and ataxia. Neuroimaging irritability. Episodes are usually triggered when placed
ataxia, intention tremor, and wide-based gait. MRI showed showed only an area of previous ischemic stroke at the in the supine position and during the child’s sleep. Its
evidence of platybasia characterized by a Welcker basal right frontoparietoinsular region. She had no similar family beginning was concomitant with the use of orthosis
angle of 149.1º. The tip of the odontoid process is located history or consanguinity but is of German descent. She for aesthetic correction of positional plagiocephaly at
approximately 19 mm from the Chamberlain line, indi- improved symptons with thiozidine. The second case, 2 months of age. Exome and EEG without alterations.
cating basilar invagination (BI). Discussion: Platybasia, a man, started the symptoms with difficulty in walking, Female, born at 38 weeks and 5 days, vaginally, without
defined by a Welcher basilar angle greater than 140°, is progressivly worsening, associated with dysarthria, global gestational or neonatal complications. A 30-year-old
the flattening of the skull base and may occur as a single spasticity, ataxia, significant postural instability and club father has a history of right temporal lobe epilepsy with
finding or in association with other forms of skull base or foot. Neuroimaging showed atrophy of the brainstem and spontaneous remission with oxcarbazepine use. Autistic
craniovertebral deformity. Basilar impression (second- cerebellum. He had No pathological antecedents and maternal cousin – with tetrasomy of chromosome 15. At
ary/acquired) and BI (primary) occur when the mastoid had Portuguese ancestry. The man has family members 4 months of age, oxcarbazepine 0.1 ml twice a day was
process of C2 invades the foramen magnum and causes with confirmed disease, a sister who started symptoms started – increasing weekly to 0.5 ml 12/12h – 10 mg/kg/
compression of the brainstem and upper cervical spine. at 20 years old (homozygous) and another asymptom- day. The patient evolved within a month with an 80%
The top of the mastoid process typically lies below the atic sister (heterozygous). Both cases were diagnosed decrease in the number of events with oxcarbazepine use.
Chamberlain line (from the hard palate to the opisthion) after genetic testing, confirming the CAPN1 mutation. Discussion: PNKD is part of a range of presentations of
and is abnormal if it protrudes more than 5 mm above this Discussion: CAPN1 is a gene involved in protein cod- dyskinetic movement disorders. The typical presentation
line. Platybasia alone does not usually cause symptoms ing and related to nervous system development and the of this condition involves involuntary movements, result-
unless it is associated with BI. When it is present, the main integrin pathway, also associated with autophagy-lysos- ing from sustained or intermittent muscle contractions.
symptoms are paraparesis, quadriparesis, ataxia, nys- somal pathway in cerebral ischemia scenario. Structural Triggers that can start an attack are caffeine use, alcohol
tagmus, dysphagia and dyspnea. The cause of BI is more alterations in this gene are associated with Autosomal use, stress, or a specific position. Because it is a rare con-
commonly associated with congenital anomalies such as Recessive Spastic Paraplegia 76, spasticity and possi- dition, it is poorly studied and its pathophysiology is still
Klippel-Feil syndrome. Basilar impression is more com- bly with neuronal damage secondary to impairment in not understood. Theories involve disinhibition of cer-
monly associated with conditions that cause softening of autophagic flux during an cerebral ischemia. Autosomal tain brain regions, such as the basal ganglia, inadequate
the bone, including Paget’s disease, hyperparathyroidism recessive spastic paraplegia spastic type 76 usually begins GABAergic response in regions related to fine motor skills,
and osteogenesis imperfecta. Remarkably, trauma was a in adulthood with slow progression, leading to spasticity structural changes in the brain, and are often associated
precipitating factor for symptoms in patients with BI, as and hyperreflexia, as well as dysarthria. Other findings with genetic alterations. There is debate as to whether
in our patient. BI is a progressively debilitating disease that may be found are foot deformities (clubfoot), plan- these types of dyskinesia can be categorized as having
that can lead to severe neurologic deficits and death if tar extension reflex and ataxia. Final Comments: The an epileptogenic etiology, since in most cases there is no
left untreated. Treatment is usually surgical and includes finding of gait disturbances with progressive worsening electroencephalographic alteration. Treatment usually
differential decompression followed by posterior stabi- in youth or early adulthood should raise as a suspicion, consists of the use of anticonvulsants, with carbamazepine
lization. Final Comments: Our goal is to emphasize the among other etiologies, of genetic diseases. Often seen being the drug of choice. Final Comments: The patient
importance of careful clinical observation, in this case, as sporadic forms of spastic paraplegia, only with genetic had an early onset of dyskinetic condition which is trig-
the apparent platybasia and the evaluation of the char- testing is a correct diagnosis possible. gered by the supine position. There is a family history of
acteristics of this rare disease, allowing early diagnosis epilepsy and autism, but the exome did not find known
and offering the best possible treatment. alterations. Laboratory tests and EEG without changes.
Transtornos do Movimento The good response to the use of oxcarbazepine guided
the therapy in this case.
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XXX Congresso Brasileiro de Neurologia

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PROPRIOSPINAL MYOCLONUS POST- NEUROACANTHOCYTOSIS WITH PALIDOTOMY IN PARKINSON’S SURGERY: IS


SARS-COV-2 INFECTION AND EXPOSITION PSYCHIATRIC ONSET: A CASE THERE STILL A PLACE?
TO COVID-19 VACCINATION: CAUSAL PRESENTATION
Renata Maia de Medeiros Falcão, Emilly Vieira
RELATIONSHIP OR COINCIDENCE? Zuza Alencar, Arissa Crispim Maximo Lima, Rafaela
Barbara Matos Almeida Queiroz, Matheus Costa
Jonata Ribeiro de Sousa, Marco André de Moraes Bessa, Karoline Ferreira Mororo Menezes, Alissa Trindade do O Caminha, Diego Silveira Oliveira,
Bernardino, Djanino Fernandes da Silva, Paulo Elen Formiga Moura, Sarah Diogenes Alencar, Fernando de Paiva Melo Neto, Geovanna Maia da
Barbosa Leite Neto, Cristiano Sobral de Carvalho, Patricia Pereira de Andrade, Flavia Paiva Rolim, Nóbrega Araujo, Stelly Vieira Zuza, Camila Crispim
Otávio Gomes Lins, João Eudes Magalhães, Fabíola Norberto Anizio Ferreira Frota, Fernanda Martins Queiroz, Francisco Neuton de Oliveira Magalhaes
Lys de Medeiros, Ana Rosa Melo Correia Lima Maia
Centro Universitário de João Pessoa – UNIPÊ. João
Universidade de Pernambuco. Recife PE, Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil Pessoa PB, Brazil
SOS Neuro Recife. Recife PE, Brazil Universidade de Fortaleza. Fortaleza CE, Brazil Centro Universitário de João Pessoa – UNIPÊ. João
Hospital Universitário Oswaldo Cruz da Pessoa PB, Brazil
barbaramatosaq@gmail.com Centro Universitário de João Pessoa – UNIPÊ – João
Universidade de Pernambuco. Recife PE, Brazil
Pessoa – PR – Brazil
jsousastmiec@gmail.com A 12 years old female, previously healthy, presented pro- UNIFIP – PATOS – PB – Brazil
gressive neuropsychiatric symptoms, characterized by psy- Universidade Federal de BRASÍLIA. Brasília DF,
Case Presentation: A previously healthy 34-year-old chotic behavior. Antipsychotic medications were started Brazil
man presented with episodes of focal spasms affecting and patient had complications of its uses, like malignant
the right shoulder and causing right-sided flexion of the neuroleptic syndrome extrapyramidal syndrome. Evolved renata__maia@hotmail.com
trunk along with abdominal muscle contractions evolving with choreoathetotic movements disorders and postural
with progressive worsening for 20 days. He observed that instability, being referred to the neurological department Case Presentation: Patient, 48 years old, female, resident
the abnormal movements increase with stressful events in a tertiary Hospital.First neurological evaluation showed and born in Ibiara-PB, presented in 2011 symptoms of
and disappear when he was sleeping. Thirty days before, cognitve impairment, with jeopardize in comprehen- movement disorder with tremor at rest in the right upper
he presented flu symptoms associated with anosmia and sion, temporospatial disorientation, bizarre behavior limb, being diagnosed with Parkinson’s Disease (PD).
ageusia that recovered in seven days. He did not search (allotriophagy), aggressive posture, bradykinesia and In 2020, she had worsening symptoms with disabling
for medical assistance and received Aztrazeneca COVID- rigidity. Tardive dyskinesia and Schizophrenia were the dyskinesias due to levodopa therapy. Pallidotomy was
19 vaccination 15 days after improvement. Magnetic first hypothesis due to prolonged use of neuroleptic med- indicated with instant improvement of symptoms and no
resonance images of the spinal cord, laboratory tests ications (10 years). No improvement of symptoms were side effects. Discussion: PD is a chronic, progressive and
including autoantibodies, and neoplastic screening were observed, albeit optimized therapeutic drugs were pre- degenerative disease of the Central Nervous System (CNS),
unremarkable. SARS-CoV-2 IgG antibodies were positive. scribed. Further investigation was made, with brain MRI with different clinical manifestations, resulting from the
Electromyography showed motor unit potential bursts showing only hippocampal atrophy. Serum copper level deficit in the production of dopamine by the neurons of
between 50-150ms without rhythmic pattern indicating and ceruloplasmin were normal. Peripheral blood analysis the substantia nigra. The most effective pharmacological
myoclonus in the right-side trunk flexor musculature that showed acanthocytes. Discussion: Neuroacanthocytosis therapy is with levodopa, aimed at maintaining normal
changed with body positioning. During polymyography, is a rare, late onset genetic disorder in which progressive levels of dopamine in the CNS. However, its long-term use
the electroencephalogram showed no epileptic parox- neurological decline associates with acanthocytes (spicu- can cause adverse effects, such as fluctuations in motor
ysms before or after myoclonus, and the Bereitschafts lated red blood cells) in the blood, being prevalent in less response and the development of involuntary movements,
evoked potential was not obtained. The patient received than one case per one million. The family history, in most classified as levodopa-induced dyskinesia. Currently, two
benzodiazepines associated with anticonvulsants and cases, is positive, with the main cause being abetalipopro- surgical methods are used for the treatment of dyskine-
improved.Discussion: The clinical features and the elec- teinemia, an autosomal recessive disease. Common signs sia: pallidotomy with ablation of the internal globus pal-
trodiagnosis were indicative of propriospinal myoclonus, and symptoms besides chorea are stereotypic gestures, lidus and deep brain stimulation (DBS), the latter being
which is a rare condition characterized by repetitive, often epileptic seizures, cognitive deficit, amyotrophy, absence the most used because neuromodulation is reversible
arrhythmic spasms in the flexor muscles of the trunk, of osteotendinous reflexes, high serum creatine kinase and adjustable. However, the choice of method must be
hips, and knees following a regular pattern. It is possible level, feeding dystonia, and self-mutilation by biting the based on careful clinical analysis and socioeconomic
that a spinal cord generator causes this kind of abnormal lips and tongue. Neuroacanthocytosis was attributed to conditions. This patient lives in a small city in the interior
movement. Diagnosis is challenging due to the possibility several mutations in the CHAC (chorea-acanthocytosis) of the state of Paraíba, about 500km from the capital, and
of a functional movement disorder, elevated frequency gene, which was later renamed as VPS13A and mapped has difficulty in transport for clinical follow-up. She had
of normal images, and a broad range of triggers (such as on chromosome 9q21.2. Final Comments: Studies and disabling dyskinesia for more than 50% of the day. The
viral infections and drugs). There are no biomarkers for research on Neuroacanthocytosis are scarce and, when treatment indicated was the ablative technique (palidot-
this disorder. Among all post-COVID-19 movement dis- present, old. This condition is still poorly reported in the omy) because it is the one that suits the patient’s profile,
orders previously published, two-thirds are reported as medical literature, and is therefore underdiagnosed. There as it is safe, low cost, effective and does not require adjust-
myoclonus, including one case associated with mRNA is a need for further research on the subject in order to ments such as neuromodulation. After surgery, dyskinesia
vaccination. It is possible that they occur due to either improve the diagnosis and treatment of these patients. totally regressed (UPDRS-part III pre: 65; 3 years later: 27).
neuronal hyperexcitability or post-viral autoimmune syn- Final Comments: The therapeutic management of PD is
drome. There is one case presentation of propriospinal based on a holistic view of the patient, which involves, in
myoclonus post-SARS-CoV-2 infection, but not after any Transtornos do Movimento addition to their clinic, their socioeconomic conditions.
COVID-19 vaccination. Final Comments: Propriospinal Pallidotomy is a surgical procedure that offers excellent
myoclonus is a rare movement disorder. Relationship results, being indicated for patients who have difficulty
with immune triggers such as SARS-Cov-2 infection or in maintaining clinical follow-up due to inaccessibility
COVID-19 vaccines may contribute to the pathophysio- to large centers.
logical knowledge of the disorder, especially when there
are no other usual causes.
Transtornos do Movimento

Transtornos do Movimento

209
XXX Congresso Brasileiro de Neurologia

TL 1105381 TL 1105481 TL 1106054

TRAUMATIC PNEUMORRHACHIS: A CASE CHRONIC EPIDURAL HEMATOMA WITH TRAUMATIC RETROCLIVAL TRAUMATIC
PRESENTATION IGOR FORTUNATO DA SILVA, ATYPICAL EVOLUTION POST TRAUMATIC SUBDURAL HEMATOMA DUE TO
FERNANDA GONÇALVES DE ANDRADE, BRAIN INJURY: CASE PRESENTATION GUNSHOT WOUND TO THE SKULL: A CASE
FERNANDA AQUINO DE OLIVEIRA, HYASMIN PRESENTATION AND LITERATURE REVIEW
Amanda de Araújo Cravo, Klaus Manoel Melo
MARIA OLIVEIRA CARVALHO Cavalcante, Labibe Manoela Melo Cavalcante, Carolina Matté Dagostini, Sabrina Busnello, Alberto
Universidade José do Rosário Vellano – Passos – Cícero José Pacheco Lins, José Augusto Rodrigues Chitolina Nesello, Eduardo Anzolin, Luciano Silveira
MG – Brazil Flores Basso
Santa Casa de Misericórdia de Passos – Passos – Centro Universitário Tiradentes. Maceió AL, Brazil Universidade de Caxias do Sul. Caxias do Sul RS,
MG – Brazil Hospital Memorial Arthur Ramos. Maceió AL, Brazil Brazil
igor.fortunato@aluno.unifenas.br Universidade Federal de Ciências da Saúde de Porto
amanda.cravo@souunit.com.br
Alegre – Porto Alegre – RS – Brazil
Case Presentation: male, 25 years old, illicit drug user, Hospital Cristo Redentor – Porto Alegre – RS –
Case Presentation: Patient, male, 27 years old, with hemi-
victim of a beating, complaining of difficulty moving the Brazil
paresis and grade 4 motor strength – on the right, with a
left lower limb (LLL) after a stab wound in the right lateral history of severe traumatic brain injury (TBI) and neuro- carolmdagostini@hotmail.com
dorsal region at the level of the thoracolumbar transition. surgical approach with left decompressive craniectomy
He was conscious and oriented, incomplete deficit in and later cranioplasty, at age 19. A control cranial tomog- Case Presentation: A 48-year-old male patient was admit-
the LLL with strength grade 4 in hip flexion, grade 2 in raphy revealed a passive epidural hematoma of resorption, ted to the emergency room with a gunshot wound to the
leg flexion and extension, grade 4 in plantar dorsiflexion which was chosen for a conservative approach. During skull. At baseline, he had 14 points on the Glasgow Coma
and grade 5 in plantar flexion; Left patellar reflex absent the subsequent 7 years, there was no clinical worsening, Scale (GCS), left foot paresis, and two open gunshot
and decreased Achilles deficit, partial sensory deficit in but with gradual growth of the hematoma evidenced on wounds to the frontal bone. Computed tomography (CT)
L1 to L4 dermatomes on the left, without motor or sen- CT. In 2022, CT showed a heterogeneous left frontopari- revealed projectile fragments entering the right frontal
sory deficits on the right. Computed tomography (CT) of etotemporal formation, predominantly hyperattenuat- region, traveling in the left parietal and frontotemporal
the spine shows subarachnoid pneumorrhachis between ing, measuring 8.7 x 8.6 x 6.3, with a midline deviation regions and lodged in the left frontoparietal region. A
T12 and L1, without fracture. Magnetic Resonance (MRI) of 1.8 cm, dilatation of the ventricular system and CSF subdural hematoma was observed posterior to the clival
shows an indolinear lesion and contrast uptake with a tra- transudation, suggestive of Chronic Epidural Hematoma ramp, with greater volume on the right, with a thickness
jectory starting in the skin on the right, right paravertebral (CEH). A reapproach was decided, with preoperative MRI of up to 0.6 cm. He underwent frontal craniotomy with
obliquely in the midline at the level of the L1 direction, showing a lesion measuring 9.3 x 9.3 x 7.2 cm. During the debridement and duraplasty. Subdural HRC was treated
signs of rupture and direction of the T12-L1 ligamentum procedure, the presence of fibrosis in the dura mater was conservatively. Postoperatively, he had a 15-point GCS and
flavum and foci of hypersignal in the neural roots close noted, as well as a heterogeneous hematoma with no persistence of grade 1 paresis in the left foot. Discussion:
to the left conus medullaris, compatible with traumatic apparent origin of the bleeding, with an aspect of “hard Retroclival hematomas (RCH) are rare, representing 0.3%
radiculopathy. There were no signs of meningitis. In the clay” next to the “motor oil” aspect. In the postoperative of acute extra-axial hematomas, and form a subgroup
outpatient follow-up after 1 month, the patient was able period, the patient evolved with a bulging wound. surgery of extra-axial posterior fossa hemorrhage. They can be
to walk without assistance, with partial recovery in some and exteriorization of serous secretion, without exudate classified as subdural, blood up to the lower border of C3
patients. Discussion: Pneumorrhachis was described or foci of bleeding, with a diagnostic hypothesis of dural and absence of blood anterior to the transverse ligament,
for the first time in 1977, and it can have different causes cerebrospinal fluid fistula. Discussion: The precise defi- or extradural, presence of blood loss at the attachment
(iatrogenic, after neurosurgery, spontaneous, infectious, nition of CEH is not clearly defined, it is known, however, of the tectal membrane to the body of C2. The classifi-
etc.), with a traumatic cause being a rare condition. that it is more common in young men, associated with TBI cation relates to neurological deficits, subsequent com-
Its diagnosis is mainly made by CT. In the presence of and the source of bleeding is usually venous. Most reports plications, and management of the patient’s conditions.
neurological deficits and with a history of trauma, even state that only 15% of patients did not present clinical The most common clinical presentation is unilateral or
without identification of spinal fractures, an MRI investi- signs suggestive of CEH, as in this case. It is understood bilateral abducens nerve palsy, as well as severe head-
gation should be followed to better determine the lesion. that a cerebrospinal fluid redistribution in the lateral ache simulating subarachnoid hemorrhage and nerve
The neurological examination is always essential for the ventricles could allow the creation of space for the hema- involvement by direct compression or contusion. Most
correct topographical diagnosis and for the appropriate toma to grow, not leading to clinical involvement, which reports are epidural hematomas associated with trau-
request of complementary exams. The case in question may be the anatomopathological basis of this report. In matic brain injury in pediatric patients. We found only 31
had a difficult initial correlation between the right back addition, most cases have a mean time between CT and reported cases of subdural RCH in adults, none of which
injury and the left lower limb deficit. Final Comments: A diagnosis. months, with few descriptions with more than were caused by a firearm. Final Comments: Traumatic
careful examination of the back in search of injuries is part years after the TBI, in this case the evolution of 8 years. posterior fossa hematomas are particularly rare condi-
of polytrauma care. Even without obvious external signs, In addition, the literature describes that CEH is isodense, tions. The conservative approach with immobilization
or unlikely mechanisms, there may be spinal injuries that corroborating the predominantly liquid presentation of is sufficient in most cases.
are not initially identified. The topographic diagnosis of CEH on surgical inspection, but with little association
neurological injuries is often challenging. with the “hard clay” appearance. Final Comments: We
present an CEH with a poorly documented chronological Traumatismo cranioencefálico
Traumatismo cranioencefálico evolution and whose pathophysiology has not yet been
elucidated. Its heterogeneous appearance, together with
postoperative evolution, raises the diagnostic hypothesis
of a dural CSF fistula.

Traumatismo cranioencefálico

210
XXX Congresso Brasileiro de Neurologia

TRABALHO CIENTÍFICO

211
XXX Congresso Brasileiro de Neurologia

TL 1104711 TL 1105070 TL 1105166

THE INTERSECTION BETWEEN CEREBELLAR SPINOCEREBELLAR ATAXIAS IN THE STATE SPINOCEREBELLAR ATAXIA TYPE 6 IS NOT
ATAXIA AND NEUROPATHY: A PROPOSED OF ALAGOAS A PURE CEREBELLAR DISEASE: DATA FROM
CLASSIFICATION AND A DIAGNOSTIC SPINAL CORD IMAGING
Débora Beserra Vilar Moraes, Tácio Luís Cavalcante
APPROACH Coradine, Everton Vieira Lopes Silva, Patrícia Lúcia Lucas de Melo Teixera Branco, Breno Kazuo
Cristina Saade Jaques, Marcio Luiz Escorcio- Silva Sampaio Leite, Nicolas Rodrigues Araújo, Massuyama, Gustavo Manginelli Lamas, Camila
Bezerra, José Luiz Pedroso, Orlando Graziani Povoas Wilson Marques Jr, Lívia Leite Góes Gitaí, Vitor Caroso Lobo, Maria Paula Carvalho Azevedo,
Barsottini Tumas Carelis Gonzáles-Salazar, Orlando Graziani Povoas
Barsottini, José Luiz Pedrozo, Thiago Junqueira
Universidade Federal de São Paulo. São Paulo SP, IAN – MACEIO – AL – Brazil
Ribeiro de Rezende, Marcondes Cavalcante França
Brazil Faculdade de Medicina da Universidade Federal de
Junior
Alagoas. Maceió AL, Brazil
cristina.sjaques@gmail.com Faculdade de Medicina de Ribeirão Preto da Universidade Estadual de Campinas. Campinas SP,
Universidade de São Paulo. Ribeirão Preto SP, Brazil Brazil
Background: Neuropathy is a common associated feature Universidade Federal de São Paulo. São Paulo SP,
of different types of genetic or sporadic cerebellar atax- liviagitai@gmail.com
Brazil
ias. The pattern of peripheral nerve involvement and its
associated clinical features can be an invaluable aspect Background: Spinocerebellar ataxias (SCAs) are a group of lucasmtbranco@gmail.com
for narrowing the etiologic diagnosis in the investigation autosomal dominant ataxias characterized by dysfunction
of cerebellar ataxias. Objective: In this review, we discuss of the cerebellum and its connections besides additional Background: Spinocerebellar ataxia type 6 (SCA6) is a
the differential diagnosis of the intersection between manifestations, such as pyramidal and extrapyramidal rare autosomal dominantly inherited disorder caused
peripheral nerve and cerebellar involvement, and clas- signs, peripheral neuropathy, visual impairment and epi- by abnormal CAG trinucleotide repeat expansions in the
sify them in accordance with the predominant features. lepsy. SCAs have an average worldwide prevalence of 2.7 CACNA1A gene (19p13.13). SCA6 is considered a proto-
Genetics, clinical features, neuroimaging, and neuro- cases per 100,000 individuals and great ethinc and geo- type of pure cerebellar ataxia, with late onset and slowly
physiologic characteristics are discussed. Furthermore, graphic variability. In Brazil, epidemiological studies are progressive course, but extracerebellar findings have been
a diagnostic approach for cerebellar ataxia with neu- based on data obtained mostly at neurogenetic reference described. The pathological hallmark of SCA6 is cerebel-
ropathy is proposed according to the different clinical centers in south and southeast regions and some regions lum and inferior olive atrophy, but degeneration has also
characteristics. Methods: This is an Educational and are still underrepresented. Objective: The present study been found on brainstem nuclei and spinal cord in autopsy
Descriptive review with the aim of medical education for aimed to identify and characterize SCAs in the state of studies. Previous MRI studies found cerebellar damage
the approach to the patients with cerebellar ataxia and Alagoas in order to contribute to the epidemiological and in SCA6 cohorts, but little is known regarding spinal cord
neuropathy. Conclusion: The diagnostic approach to the clinical profile of SCAs and to stimulate the development imaging changes in these patients. Objective: To assess
patient with cerebellar ataxia with neuropathy requires a of strategies to detect people with SCA in regions without spinal cord involvement through MRI and its clinical cor-
detailed medical history, phenotyping, characterization epidemiological data. Methods: From June 2018 to August relates in a SCA6 cohort. Methods: We enrolled 17 SCA6
of disease progression and family history. Neuroimaging 2021, we carried out active search strategies for people patients with genetic confirmation, but 4 patients were
features and the neurophysiological findings play pivotal with a diagnosis or clinical suspicion of SCA and applied excluded due to compressive myelopathy. They under-
roles in defining the diagnosis. Establishing an organized a clinical and molecular evaluation protocol that included went clinical evaluation, including SARA, INAS and ICARS
classification method for the disorders based on the clin- clinical interview, heredogram construction, neurological scales, and MRI examination on a 3T Phillips scanner at
ical features may be very helpful, and could be divided as exam, ataxia and functional scales and molecular analysis the same day. Thirteen age and sex-matched healthy con-
those with predominant cerebellar features, predominant for SCAs type 1, 3, 6, 7,8,10, 12, 17 and DPRLA. Results: As trols were also assessed. The Spinal Cord Toolbox (SCT)
neuropathic feature, or conditions with both cerebellar a result, 73 people with SCA were identified, configuring a was employed to measure spinal cord area and eccen-
ataxia and neuropathy. Second, determining the mode minimum estimated prevalence of 2.17 cases per 100,000 tricity in C1-T2 levels and white matter tracts integrity
of inheritance is critical on cerebellar ataxias: autosomal inhabitants, distributed as 75.3% patients with SCA3, by assessing diffusion parameters, which are fractional
dominant and recessive cerebellar ataxias, mitochondrial 15.1% with SCA7, 6.8% with SCA1 and 2.7% with SCA2. anisotropy (FA), axial diffusivity (AD), radial diffusivity
or sporadic types. Third, one must carefully assess neu- SCA3 and SCA7 analysis showed age at onset and clinical (RD) and mean diffusivity (MD), in C2-C5 levels. Imaging
rophysiologic findings in order to better characterize the features congruent with other studies; predominance of data were compared between SCA6 and controls groups
predominant pattern of involvement: damage location, SCA3 subphenotype 2; gait alterations as the main initial through a Mann-Whitney U test, employing age and sex
mechanism of lesion (axonal or demyelinating), motor, manifestation and, in people with SCA7, accompanied as covariates. Bonferroni correction for multiple com-
sensory or sensory motor compromise, large or small by reduced visual capacity; inverse correlation between parisons was performed. Correlations between imaging
fibers, and autonomic system abnormalities. age at onset and number of expanded CAG; positive and clinical data were assessed through Spearman’s rank
correlation between severity and disability scales and correlation coefficients. Results: Mean age of the patients
duration of illness; and that most people affected are not and disease duration were 68,1±7,0 and 15,3±6,9 years,
Ataxias engaged in a rehabilitation program and there are large respectively. Mean SARA score was 15,0±6,9. SCA6 group
numbers of asymptomatic people at risk of developing had increased AD in left tectospinal tract (p=0.023), left
SCA. Conclusion: Therefore, this study show that sim- lateral vestibulospinal tract (p=0.027) and left ventral cor-
ple collaborative strategies can enhance the detection ticospinal tract (p=0.045), increased MD in right lateral
capacity of SCA families and demonstrate that Alagoas vestibulospinal tract (p=0.032) and left tectospinal tract
has a minimum estimated prevalence of SCA similar to (p=0.019), and increased RD in right lateral vestibulospi-
the world average prevalence. nal tract (p=0.032). These MRI parameters did not cor-
relate with clinical data but, in an exploratory approach,
left ventral corticospinal tract RD were directly related to
Ataxias INAS scale (p=0.011, r=0.699). Conclusion: These findings
indicate that not only the cerebellum, but also the spinal
cord is involved in SCA6. Quantitative spinal cord MRI
might be a useful biomarker in SCA6.

Ataxias

212
XXX Congresso Brasileiro de Neurologia

TL 1105284 TL 1105785 TL 1106076

NIGROESTRIATAL DYSFUNCTION IS ATROPHY OF CRANIAL NERVES V AND VIII IS NUTRITIONAL ASSESSMENT OF PATIENTS
FREQUENT IN RFC-1 RELATED DISORDER A SPECIFIC NEURORADIOLOGICAL SIGN OF WITH HEREDITARY ATAXIAS IN NORTHEAST
RFC1-RELATED DISORDER BRAZIL: A CASE-CONTROL STUDY
Maria P A Carvalho, Camila C Lobo, Lucas M T
Branco, Gabriel S Schimitti, Paula C A A P Matos, Camila Caroso Lobo, Guilherme Soares Oliveira Camila Gonçalves Monteiro Carvalho, Pedro Braga
Alberto R M Martinez, Barbara J Amorim, Orlando G Wertheimer, Lucas Melo Teixeira Branco, Thiago J R Neto, Paulo Ribeiro Nobrega, Déborah Moreira
P Barsottini, Jose L Pedroso, Marcodes C França Jr Rezende, Fabiano Reis, Paula Camila A A P Matos, Rangel, Stheffane Suzane Oliveira Scott, Danyela
Orlando G P Barsottini, José Luiz Pedroso, Wilson Martins Bezerra Soares
Universidade Estadual de Campinas. Campinas SP,
Marques jr, Marcondes C França Jr
Brazil Universidade Estadual do Ceará. Fortaleza CE,
Universidade Federal de São Paulo. São Paulo SP, Universidade Estadual de Campinas. Campinas SP, Brazil
Brazil Brazil Universidade Federal do Ceará. Fortaleza CE, Brazil
Universidade Federal de São Paulo. São Paulo SP,
paulacarvalhoazevedo@hotmail.com camila_gmonteiro9@hotmail.com
Brazil
Universidade de São Paulo. Ribeirão Preto SP, Brazil
Background: RFC1/CANVAS syndrome is a rare adult-on- Background: Hereditary Ataxias (HAs) comprise a wide
set heredodegenerative disorder caused by biallelic camilaloboc@gmail.com spectrum of genetically determined neurodegenerative
AAAAG RFC1 expansions and characterized primarily diseases with progressive ataxia as the main symptom. Few
by cerebellar ataxia, sensory neuronopathy (ganglion- Background: The Cerebellar Ataxia, Neuropathy, and studies have evaluated nutritional profile in HA patients.
opathy), as well as bilateral vestibular hypofunction. Vestibular Areflexia Syndrome (CANVAS) related to RFC1 Objective: To evaluate eating habits and nutritional status
Parkinsonism is now recognized as an additional fea- is an important cause of late-onset progressive ataxia. In of a population of patients with HA in Northeastern Brazil.
ture in the disease, however no systematic evaluation clinical practice, it may be difficult to differentiate it from We also analyzed the association of these findings with
of nigrostriatal dopaminergic function has been pub- other degenerative ataxias, such as spinocerebellar ataxias clinical and genetic profiles of this population. Methods:
lished so far. Objective: To describe striatal dopamine (SCA) and multiple system atrophy cerebellar type (MSA- We performed an observational case-control study of 2
transporter (DAT) density in a sample of RFC1/CANVAS C) due to its clinical interface. Thinning of cranial nerves V reference centers in neurogenetic diseases in Fortaleza,
patients using dopaminergic transporter brain scintig- and VIII has been lately reported in MRI scans of patients a city located in Northeast Brazil. Body composition was
raphy with 99mTc-TRODAT-1 (DAT imaging). Methods: with RFC1-related disorder, but its diagnostic specificity is assessed with bio-impedance analysis and dietary intake
This is an observational, single-center study conducted not yet clear. Objective: To assess the usefulness of cranial was estimated with a validated questionnaire (24-hour
in a tertiary referral Hospital, which analyzed 10 patients nerves V and VIII imaging to differentiate RFC1-related dietary recall). Results: The final sample consisted of
with molecular confirmation of RFC1/CANVAS. Disease disorder from SCA and MSA-C. Methods: Thirty-seven 76 participants, 38 patients with hereditary ataxias and
severity was assessed with the SARA scale. Each subject patients with late-onset ataxia were enrolled, including 38 controls. Mean body mass index (BMI) was lower in
was carefully evaluated for the presence of parkinsonian 9 individuals with RFC1-related disorder, 23 with SCAs HA compared to controls (p=0,032). Hereditary ataxia
features. DAT imaging was acquired and reconstructed in (types 2,3 and 6) and 5 with MSA-C defined by clinical patients showed lower protein intake, higher frequency
the transverse, coronal and sagittal planes 4 hours after criteria. Nine healthy controls were also assessed. All sub- of dysphagia and higher incidence of nausea and diar-
venous injection of 99mTc-TRODAT-1. An experienced jects underwent MRI scans on a 3T Philips scanner and rhea. The difference in average estimated caloric intake
nuclear physician performed the visual analysis of all clinical evaluation on the same day. We acquired axial did not reach statistical significance. Disease severity
images. Quantification was performed drawing region T2-weighted MRI with thin slices (balance sequence) for measured by SARA scale was not associated with BMI,
of interest (ROI) in the striatum, putamen and caudate qualitative assessment of V and VIII cranial nerves. Images nor was ataxia subtype (autosomal dominant x non-auto-
bilaterally. The specific uptake (mean uptake ratio) of were reviewed by a neuroradiologist, blinded to patient somal dominant ataxias). Conclusion: Hereditary ataxia
each region was calculated using occipital region uptake and clinical data, to classify these nerves as atrophic or patients have lower BMI compared to healthy controls.
as the reference. Results: Patients had a mean age of 63 ± normal. We analyzed the frequencies of combined and iso- There was no difference in this cohort between dominant
9.2 years, and there were 7 women. The mean SARA score lated atrophy of nerves V and VIII for each subject. These or non-dominant ataxia regarding BMI. Weight loss may
was 16.9 ± 5.8. Seven patients had abnormal DAT imaging frequencies were compared between groups using Fisher be a common finding among hereditary ataxias and may
results , characterized by reduced uptake in left putamen exact test. Level of significance was set at 0.05. Results: affect quality of life in these patients.
(100%), right putamen (85.7%), left caudate (71.4%) and Mean age of patients with RFC1-related disorder, SCA
right caudate (71.4%). Four patients (57.1%) had bilat- and MSA were 66.7±7.3, 56.3±16.6 and 64.4±13.2 years,
eral striatum abnormalities. Parkinsonism was noticed respectively. Atrophy of cranial nerves V (CNV) and VIII Ataxias
in 3/7 patients, all of which had abnormal DAT scans. (CNVIII) were significantly more frequent in the RFC1
Interestingly, four subjects had reduced DAT imaging group when compared to SCA (CNV: p<0.001; CNVIII:
uptake, but no clinical signs of parkinsonism. Conclusion: p<0.001), MSA (CNV: p<0.001; CNVIII: p=0.008) and con-
Nigrostriatal dysfunction is frequent in RFC1/CANVAS, trols (CNV: p<0.001; CNVIII: p=0.008). In an exploratory
suggesting that disease pathology is more widespread than sub-analysis assessing each SCA subtype, simultaneous
previously thought. Additionally, the fact that patients atrophy of both nerves was also more frequent in the
without parkinsonism also had abnormal DAT imaging CANVAS group than in SCA2 (p=0.035), SCA3 (p=0.015)
results might suggest that DAT imaging changes occur and SCA6 (p=0.009) subgroups. Conclusion: MRI eval-
early along the disease course. These results have practi- uation of cranial nerves V and VIII using a dedicated
cal therapeutic relevance and suggest that dopaminergic sequence is an easy-to-use tool that helps to distinguish
agents might be useful at least for a subgroup of patients RFC1-related disorder from other late-onset degenerative
with RFC1-related disorder. ataxias. This new neuroradiological sign should be sought
in the routine evaluation of ataxias.
Ataxias
Ataxias

213
XXX Congresso Brasileiro de Neurologia

TL 1106564 TL 1106656 TL 1104999

THE IMPORTANCE TEVALUATION OF QUALITY TRANSLATION, CROSS?CULTURAL DIALYSIS-RELATED HEADACHE:


OF LIFE WITH THE USE OF VIRTUAL REALITY ADAPTATION, AND VALIDATION TO PREVALENCE AND CLINICAL FEATURES
IN PATIENTS WITH SPINOCEREBELLAR BRAZILIAN PORTUGUESE OF THE IN HEMODIALYSIS PATIENTS AND AFTER
ATAXIA CEREBELLAR COGNITIVE AFECTIVE/ KIDNEY TRANSPLANTATION
SCHMAHMANN SYNDROME SCALE
Bianca Simone Zeigelboim, Maria Izabel Rodrigues Bruno Teixeira Gomes, Alberto Luiz Cunha Costa,
Severiano, Geslaine Janaina B. Santos, Hélio Stephanie Suzanne de Oliveira Scott, Jose Luiz Marilda Mazzali
Afonso G Teive, Maria Renata José, Bianca Lopes Pedroso, Victor Vitalino Elias, Paulo Ribeiro Nobrega,
Cavalcante Leão, Cristiano Miranda Araújo, Jessica Faculdade de Ciências Médicas da UNICAMP.
Emmanuelle Silva Tavares Sobreira, Maria Thereza
Maliski, Francisco Manoel Branco Germiniani Campinas SP, Brazil
Drumond Gama, Marcela Patricia de Almeida,
Norberto Anizio Ferreira Frota, Orlando Graziani brunogomes465@gmail.com
UTP. Curitiba PR, Brazil
Povoas Barsottini, Pedro Braga-Neto
Universidade Federal do Paraná. Curitiba PR, Brazil
Background: Headache is a common symptom, occur-
Universidade Federal do Ceará. Fortaleza CE, Brazil
frankgerminiani@hotmail.com ring in up to 70% of patients on hemodialysis. Dialysis
Universidade Federal de São Paulo. São Paulo SP,
headache is defined by the International Classification
Brazil
Background: Spinocerebellar ataxias (SCAs) are a hetero- of Headache Disorders (ICHD-3) as a headache with no
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
geneous group of neurodegenerative diseases, featuring specific characteristics, occurring during and caused by
Universidade de Fortaleza. Fortaleza CE, Brazil
the presence of progressive cerebellar ataxia, whose early hemodialysis, and which resolves spontaneously within 72
clinical symptoms are balance and motor coordination stephaniesscott@hotmail.com hours after the session has ended. There is no consensus
decline and ocular disorders. Objective: To evaluate the on its pathophysiology or triggering factors. Objective:
living standard of patients with SCAs, by applying the Background: The Cerebellar Cognitive Afective Syndrome To evaluate the prevalence, clinical features and factors
Vestibular Disorders Activities of Daily Living Scale (VADL) (CCAS) is characterized by defcits in executive functions, associated with dialysis headache in patients with chronic
and Activities-specific Balance Confidence Scale (ABC) language processing, spatial orientation, and afect regu- kidney disease on renal replacement therapy. Methods:
questionnaires, before and after rehabilitation with vir- lation in patients with cerebellar disease. The symptoms Cross-sectional observational study with a quantitative
tual reality (ViR). Methods: An uncontrolled clinical trial can occur isolated or along with motor and coordina- approach. Participants were divided into two groups: (1) 25
study was conducted with 28 patients suffering from SCA tion symptoms. Objective: The aim of our study was to patients on hemodialysis and (2) 25 patients on early post
(mean of 41.6 years, ± of 16.9 years), underwent anam- translate and culturally adapt the CCAS scale to brazil- kidney transplantation. All participants were interviewed
nesis, otolaryngologic examination, vestibular screening, ian Portuguese and validate the scale in our population. with a structured questionnaire, the Hospital Anxiety and
and the application of protocols applied before and after Methods: We performed a cross-sectional study with Depression Scale, and the Epworth Sleepiness Scale. Blood
rehabilitation with ViR. Result: The most reported otoneu- patients with primary and secondary ataxia. The study pressure, weight, urea, glucose and electrolytes were eval-
rological complaints in the anamnesis were: imbalance included 111 individuals, aged between 20 and 80 years, uated before and after one hemodialysis session. Control
(85.7%), fall (28.5%), dizziness (17.8%), diplopia (10.7%), of both genders, 20 without cognitive and/or afective groups were classified from the headache-free patients in
and tremor (7.1%); The vestibular exam was altered in complaints who participated in the pre-test phase, 40 each group. Results: In group (1), 8 patients (32%) were
64.3% of the cases; The comparison Results: between with cerebellar disease (hereditary/neurodegenerative diagnosed with dialysis headache, predominantly pulsa-
the evaluation time (T1, T2 and T3) and the dimensions ataxia or acquired/secondary cerebellar ataxia), and 51 tile (n=6, 75%), accompanied by photophobia (n=6, 75%),
(functional, locomotion and instrumental) from the VADL healthy controls with no evidence of cognitive impair- phonophobia (n=4, 50%) and nausea or vomiting (n=6,
did not show significant differences (1st assessment ment and no afective symptoms matched for sex, age, 75%), with a mean pain score of 7.75 ± 1.58. Individuals
before the start of rehabilitation designated T1, after 10 and educational level. The scale was translated, cultur- with headache had higher scores for anxiety (7.00 ± 3.93 vs.
rehabilitation sessions, considered T2 and, at the end of ally adapted, and validated. Statistical analysis of the data 3.82 ± 3.23, p=0.03) and sleepiness (9.13 ± 3.94 vs. 4.76 ± 3,
20 rehabilitation sessions, called T3);The results from the was performed, with association tests, mean comparison, 85, p=0.01). Dialysis headache was associated with lower
ABC revealed that there was not significant differences and ROC curve analysis. Results: In the association test, pre-dialysis serum calcium (p=0.01), higher pre-dialysis
when compared with the evaluation time (T1, T2 and T3); the items Semantic Fluency (P= >0.999) and Phonemic systolic (p=0.02) and diastolic (p=0.02) blood pressure.
The result between the correlation of the VADL and ABC Fluency (P= 0.386) did not present a statistically signifi- There was no correlation between headache and varia-
questionnaires showed significant Results: in all cases. cant difference when comparing the group of patients to tions in serum urea levels. In group (2), 5 patients (20%)
The correlation between the ages and disease length the control group. The scale’s internal reliability, assessed were diagnosed with dialysis headache, predominantly
with the VADL and ABC questionnaires was significant by Cronbach’s alpha, was 0.752, demonstrating a good pulsatile (n=4, 80%), accompanied by nausea or vomiting
between the age and the instrumental dimension of the degree of reliability and consistency between the scale’s (n=4, 80%), aggravated by routine physical activity (n=3,
VADL questionnaire, in the T3 assessment. The correlation subitems. Based on the analysis of the ROC Curve, optimal 60%), with a mean pain score of 8 ± 1.41. Individuals with
between the disease length and the VADL questionnaire cutoff values were found for each subitem of the scale. headache had higher sleepiness scores (9.20 ± 4.32 vs. 4.80
was significant in all cases; The comparison of the ViR Conclusion: The translated and adapted scale has good ± 4.51, p=0.029) and younger age (38.93 ± 14.43 vs. 54.02 ±
result (T1 to T2) showed significant difference for all of the internal consistency, is reproducible, has good reliability, 8.31, p=0.03). Conclusion: Headache is common among
applied games, except for the ski slalom. The comparison and has the potential to be a reliable tool for screening patients on hemodialysis, it has migraine-like features,
of the ViR result (T1 to T3) showed significant difference cognitive symptoms in patients with cerebellar disease. and it is associated with increased anxiety, increased
for all of the applied games. Conclusion: We can point sleepiness, and higher pre-dialysis blood pressure levels.
out a direct improvement of the living standard, reflected
by the reduction of falls, better balance and march, con- Ataxias
tributing to a higher self-confidence in patients in daily Cefaleia
activities. We also point out the importance of ViR as the
symptomatology in this kind of population improved.

Ataxias

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XXX Congresso Brasileiro de Neurologia

TL 1105053 TL 1105302 TL 1105305

TELEMEDICINE ON THE TREATMENT OF THE EFFICACY OF GREATER OCCIPITAL USE OF MONOCLONAL ANTIBODIES IN
CHRONIC HEADACHE: A SYSTEMATIC NERVE BLOCK FOR THE TREATMENT OF PATIENTS WITH MIGRAINE: SUBJECTIVE
REVIEW MIGRAINE: A SUBJECTIVE EVALUATION AND ASSESSMENT OF USE AND IMPROVEMENT
REPORT OF 31 PATIENTS WITH THESE DRUGS INTRODUCTION
Tamires Vasconcelos, Bernardo Rodrigues, Juliana
Melo, Larissa Monteiro, Nadja Santiago, Camila MIGRAINE IS ONE OF THE MOST FREQUENT
Vinicius Slongo Buhler , Luiza Orth , Lucas Victoy
Orrico, Cristina Salles, Carolina Orge, Ailton Melo Guimarães Zengo , Eduarda Basso Badalotti , DISEASES IN NEUROLOGICAL CLINICAL
Angelo Marcelo Wosniacki Filho , Márcia Bernardon PRACTICE, WITH ECONOMIC AND SOCIAL
Universidade Federal da Bahia. Salvador BA, Brazil EFFECTS FOR PAT
, Gabriel Angelo Garute Zenatti , Renato Endler
University of Connecticut , United States
Iachinski , Rafaela Luiza Altheia Griza , Pietra Baggio
Escola Bahiana de Medicina e Saúde Pública. Pietra Baggio Peixoto Oliveira, Márcia Bernardon,
Peixoto de Oliveira
Salvador BA, Brazil Albano Mayer Junior, Renato Endler Iachinski ,
Universidade do Estado da Bahia. Salvador BA, Centro Universitário da Fundação Assis Gurgacz Gabriel Angelo Garute Zenatti , Vinicius Slongo
Brazil FAG Bühler, Lucas Victoy Guimarães Zengo
Fundação Hospitalar São Lucas FHSL
asm@ufba.br Centro Universitário da Fundação Assis Gurgacz
viniciusslongobuhler@hotmail.com Fundação Hospitalar São Lucas FHSL
Background: Chronic headaches, such as migraines
and tension-type headaches, are among the most com- pietrabaggio@hotmail.com
Background: Migraine is the most common type of pri-
mon disorders in the world, but access to specialists for mary headache in outpatient clinics, even in the emer-
adequate follow-up is not yet feasible in all locations, Background: Migraine is one of the most frequent dis-
gency room. With their debilitating crises that often end
especially in rural areas. Telemedicine/teleconsultation eases in neurological clinical practice, with economic
up invalidating the individual in their full work activity.
is a form of evaluation that can be used for several neuro- and social effects for patients and the health system.
An example of a treatment that has been extensively stud-
logical disorders, including headaches, allowing a greater The disease treatment is costly and those who suffer
ied today is the occipital nerve block, which has shown to
reach of health care. Objective: our goal was to evaluate from migraine are affected in multiple domains. The
be quite promising. The greater occipital nerve (GON) is
the effectiveness of telemedicine/teleconsultation visits prophylactic treatment uses antidepressants, anticonvul-
responsible for the cutaneous sensory innervation of the
in the treatment of chronic headaches compared to a tra- sants, triptans, among other drugs. They are safe drugs
occipital and suboccipital regions. Recent research has
ditional visits. Method: A systematic review of literature, but have low efficacy, after all, 35% of patients – treated
shown that it also actively participates in brain nociceptive
using PubMed, MEDLINE, Cochrane, Lilacs, and Scielo with Triptains – overgoing randomized clinical trials did
modulation, acting directly, together with the trigeminal
databases, yielded 262 articles, of which 9 studies were not improve, and 40% of them experienced side effects.
nerve, on secondary nociceptive neurons located at the
included for analysis. Results: a total of 1,834 patients were In an attempt to chance the outcome of the scenario,
level of the brainstem. Objectives: Evaluate the efficacy
evaluated, mostly involving women and with migraines alternative therapies, such as immunological therapies,
of GON-block in the treatment of migraine. Method:
sufferers. They also included children, adolescents, and with use of Monoclonal Antibodies (mAb) such as Ajovy,
The model of this study was a survey research, involving
adults. Four studies were conducted during the corona- Emigality and Pasurta, intend to improve the perspectives
patients diagnosed with migraine. These patients were
virus pandemic. All studies showed a high patient sat- for patients and direct the investments of the health system
treated with GON-block. The instrument of data collection
isfaction rate with teleconsultation, whether adults or in efficient treatments. Objective: To carry out a subjective
comprehends a questionnaire made through telephone
children with their families. It also showed a tendency evaluation with patients treated at the Proneuro Clinic, in
calls, and was prepared with the aid of two sources: the
to reduce the number of headache-days per month, the the city of Cascavel-PR, who used mAb for the treatment
medical records of the reference unit and relevant data
use of analgesics, as well as the frequency and duration of migraine, verifying if there was an improvement in the
from other research in the area. Results: The sample size
of pain. Conclusion: telemedicine was found to be an clinical condition with the new therapy. Method: This is
presented a number (n) of 31 participants diagnosed
effective and viable form of follow-up for patients with a subjective assessment with the application of a ques-
with migraine, with a predominance of female patients
chronic headaches. tionnaire, carried out through calls, clarifying relevant
(93.5%, n = 29). Concerning the age, the average was 38
information about the outcomes of immune therapy.
years. In the survey, 93.5% (n = 29) reported pain relief
Results: The sample consisted of 68 individuals diag-
after the procedure. Among these, they were asked to
Cefaleia rate verbally their average pain relief on a scale ranging
nosed with migraine, treated by the same neurologist,
who were instructed to use mAb. 34 did not answer the
from 0 to 10. The average on the headache relief scale
calls, 14 never underwent the treatment due to the high
was 7.5, and 77.4% (n = 24) reported a relief greater than
cost and 20 used mAb and answered the questionnaire.
6 points. The onset time for symptom relief was less than
The results show that 70% of the collaborating patients
48 hours for 45.1% (n = 14), with an average duration of
resorted to use the immunological therapy because the
54 days. In the sample, 25.8% (n = 8) reported side effects,
previous treatments did not present good efficacy. 30%
that include dizziness, holocranial headache and edema.
made the substitution due to side effects such as pares-
Finally, 37 participants answered that they would do the
thesia, xerostomia and dizziness. Also, it was found that
procedure again. Conclusion: Greater occipital nerve
70% of patients who used mAb had a reduction in the
block has proven to be an effective treatment method
frequency of migraine attacks and 85% reported relief in
when properly indicated. However, further randomized
intensity. However, 30% pointed out that the frequency
controlled trials are needed to better assess the evidence
remained the same and the intensity in 15% of them also
of efficacy in the treatment of migraine.
did not change. Conclusion: The result with the mAb
has a statistically significant effect when compared to
Cefaleia conventional treatment. However, low adherence, due
to the high cost, prevents the therapy from being applied
globally so that the disease can have reasonable control
in the majority of the population.

Cefaleia

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XXX Congresso Brasileiro de Neurologia

TL 1105490 TL 1105499 TL 1105548

BOTULINUM TOXIN AS A PREVENTIVE MONOCLONAL ANTI-CGRP ANTIBODIES AS A THE INFLUENCE OF THE USE OF
TREATMENT FOR MIGRAINES TREATMENT FOR MIGRAINE PERSONAL PROTECTIVE EQUIPMENT ON
PREVIOUS HEADACHES OF HEALTHCARE
Italo Felipe Cury, Anderson Moura Bernardes, Italo Felipe Cury, Anderson Moura Bernardes,
Anderssany Moura Bernardes Anderssany Moura Bernardes PROFESSIONALS EXPOSED TO THE
COVID-19 PANDEMIC
Universidade de Gurupi. Gurupi TO, Brazil Universidade de Gurupi. Gurupi TO, Brazil
Universidade Federal do Piaui. Teresina PI, Brazil Universidade Federal do Piaui. Teresina PI, Brazil Júlia Rabêlo Evangelista, Ana Letícia Batista Leal
Universidade de Gurupi. Gurupi TO, Brazil Barbosa, Lara Samanta Barbosa Ribeiro, João Pedro
italofelipe.cury@gmail.com Rosal Miranda, Arieny Karen Santos Lima, Ana
italofelipe.cury@gmail.com Beatriz Luz Barradas Coutinho, Laís Fortes Hidd
Background: Migraine is a disease that affects about 12% Vasconcellos, Carlos Eduardo Cordeiro Cavalcante,
of the world population. This headache can be character- Background: Migraine is a highly debilitating pathology Kelson James Almeida
ized as intense and often debilitating, resulting in impair- capable of causing a loss of ability to work and social life.
ments in work capacity, in social life and generating large However, only about 15% of patients who need prophy- Unifacid. Teresina PI, Brazil
expenses for health services. As a result, prophylactic lactic follow-up do so properly, the vast majority due to Universidade Estadual do Piauí. Teresina PI, Brazil
treatments for chronic migraines began to gain greater absence/efficacy or as a result of the secondary effects of Universidade Federal do Piauí. Teresina PI, Brazil
value and adherence among those affected by this disease. the prescribed medications. In recent years, the develop- juliarabeloevangelista@gmail.com
Among the wide range of possible treatments, botulinum ment of a new family of drugs, anti-calcitonin gene-related
toxin has been gaining prominence in the preventive peptide (CGRP) monoclonal antibodies, paves the way for
Background: As a result of the COVID-19 pandemic, the
treatment of chronic migraines for more than a decade, more effective treatment of migraine migraines. This drug
use of personal protective equipment (PPE) has become
although its mechanism of action is gradually being under- is based on blocking the CGRP pathway of action, which
indispensable for the safety. However, the impact of PPE
stood. Objective: To systematically evaluate advances in tends to have its levels increased during crises. Objective:
use on previously diagnosed headaches of healthcare pro-
the use of botulinum toxin as a preventive treatment for To systematically evaluate advances in the treatment of
fessionals is uncertain. Objective: To analyze the influence
migraine, considering improvements in quality of life and migraine based on the use of monoclonal antibodies
of the use of PPE on previous headaches of health profes-
considering its cost-benefits for the patient. Methods: This related to the anti-calcitonin gene. Methods: This is a
sionals exposed to the COVID-19 pandemic. Method: This
is a systematic literature review based on data extraction systematic literature review based on data extraction in
was a non-interventional, quantitative, observational, and
in indexes such as PUBMED and MEDLINE. Publications indexes such as PUBMED and MEDLINE. Publications
cross-sectional study. Thus, the Snowball method was used
from 2018 to 2022 were considered, in English and with between 2018 and 2022, in English and with the fol-
for recruitment and an online questionnaire was applied
the following descriptors: Botulinum Toxin, Migraine and lowing descriptors: CGRP, monoclonal antibodies and
to the participants. In addition, the Kolmogorov-Smirnoff
Treatments. Results: Since the approval of botulinum Migraine, were considered. Results: CGRP is a neuro-
test was used to define data normality and Fisher’s exact
toxin as a preventive treatment for migraine, the results peptide synthesized at multiple sites in the central and
test served to correlate categorical variables. Headaches
of the various studies carried out have demonstrated its peripheral nervous system and its role was noted from
were classified according to diagnostic criteria present in
strong efficacy, as it leads to a significant decrease in the the observation of its elevation during moments of cri-
the International Classification of Headache Disorders
number of hours in crises that the patient was affected in ses and also in the induction of crises in patients who
(ICHD-3). Results: The study showed that 20.6% of the
the month. In addition, patients who used this preventive underwent amino acid injection. The evaluation of CGRP
participants had headaches diagnosed before, of which
treatment showed a positive result after about 2 months inhibition has emerged as an effective method of treating
70% had migraine headaches and 30% had tension-type
of medication use and quickly showed improvement in migraine and studies have shown a significant reduction
headaches. Previous headaches, which are more common
quality of life, in work and social activities. Finally, the in the number of attacks in patients who used medication.
in women, had a significant difference between genders
studies point to a continuity in the clinical improvement Thus, the primary goals of the treatments with mono-
(p<0.001). The difference between the types of headaches
after treatment, since the effects of the toxin remained clonal antibodies were significantly achieved, since the
presented was not significant (p=0.5698). Most profession-
over time. Conclusion: Preventive treatments for chronic patients pointed to a reduction in the number of attacks
als (67.5%) had changes in the pain pattern when using
migraine seek to reduce the number of hours and amount in the month. Conclusion: Treatment with anti-CGRP
PPE during the pandemic, of which 45% had migraine and
of attacks per month that each patient is affected. From monoclonal antibodies appears as a positive aspect in
22.5% had tension-type headaches, with no statistically
this perspective, botulinum toxin gains prominence for the treatment of migraine attacks and of course there are
significant difference between the change in the pattern
guaranteeing both possibilities, in addition to offering a still some barriers to be faced, such as the high cost and
and the classification (p=0.7158). The increased frequency
lower cost than the new monoclonal medications. This the lack of research on long-term treatment or relapses.
of crises was the main alteration mentioned by 60% of the
presented relationship guarantees the outstanding local However, this new line of research managed to guarantee
professionals with previous headaches, although there was
botulinum toxin in the field of preventive medicine for significant results in the treatment with a reduction in
no statistical significance (p=0.9899). Furthermore, 40%
these situations. the number of seizures per month in the vast majority of
of the professionals with previous headaches reported an
patients and thus allowed an improvement in the quality
increase in the duration of the crises, without statistical
of their personal and social life.
Cefaleia significance (p=4898). The analysis of the participants’
perception, who reported an increase in the frequency
Cefaleia of crises, regarding the relationship between the types
of PPE and this change showed statistical significance
(p<0.040) for the use of masks. There was also statistical
significance in the perception of the relationship between
increased crisis duration and each type of PPE (p=0.0402).
Conclusion: A change was observed in most patients who
reported previous headaches, in the pattern of crises, in
terms of pain and frequency, with the use of PPE during
the pandemic. Most patients considered the association
between the types of PPE and these changes as very likely.

Cefaleia

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XXX Congresso Brasileiro de Neurologia

TL 1105653 TL 1105696 TL 1105961

PREVALENCE OF EXTERNAL COMPRESSION THE ASSOCIATION BETWEEN HEADACHE RELATION BETWEEN TENSIONAL HEADACHE
HEADACHE CAUSED BY USE OF PERSONAL AND ANXIETY DURING THE COVID-19 AND GENERALIZED ANXIETY DISORDER
PROTECTIVE EQUIPMENT IN HEALTHCARE PANDEMIC IN THE SOUTHEAST REGION OF
Bruna Gonçalves Dantas de Almeida, Beatriz
WORKERS EXPOSED TO THE COVID-19 BRAZIL Barrozo Gonzalez de Oliveira
PANDEMIC
Beatriz Carvalho Soares, Cristina Pessoa de Queiroz UNICEUMA. São Luís MA, Brazil
Bruna Tavares Falcão, Maria Eduarda de Souza Arêa Faria Góes, Ana Carolina Godoi Medrado, Ana
Leão, Raimundo Nonato Marques Filho, Gabriel Beatriz Garcez de Oliveira, Bruna Cavalcante de brunaalmeidag@icloud.com
Nunes Dantas, Ana Letícia Batista Leal Barbosa, Sousa, Crissia Pitanga Malta, Daniel Meohas de
Ana Beatriz Luz Barradas Coutinho, Laís Fortes Hidd Souza Lima, Isadora Baldissara da Rocha Pitta, Background: Headaches are considered an important
Vasconcellos, Carlos Eduardo Cordeiro Cavalcante, Louise Ferreira Nascimento Pestana da Costa, public health problem and there are several phycological
Kelson James Almeida Milena Pereira Ribeiro da Silva factors linked to the manifestation of this symptom that is
related, especially to anxiety. With that in mind, it became
Centro Universitário Unifacid. Teresina PI, Brazil Universidade Estadual do Rio de Janeiro. Rio de essential to study the connection involving these two fac-
Universidade Federal do Piauí. Teresina PI, Brazil Janeiro RJ, Brazil tors in an attempt to explain this phenomenon and how
bruvares@gmail.com biacarvalhosoares@gmail.com to proceed with the treatment of this patiente. Objective:
The objective of this study is to relate the incidence of ten-
Background: Headaches have scientifically established sion- type headache in patients with Generalized Anxiety
Background: External compression headache is classified
tryggers, among it the psychosocial factors are of great Disorder, as well as its manifestation. Methods: This is
as a primary headache resulting from compression of the
medical importance. So, comorbidities like psychoso- a systematic review, conducted on Scielo and Google
soft tissues lining the skull. Pain is caused when periph-
cial stress and anxiety can act primary cephalea. Due to Scholar platforms. Were selected articles in portuguese,
eral nociceptors are stimulated due to some tissue lesion.
changes in the social dynamics during COVID-19 pan- published in the last twenty years with the descriptors
In this perspective, with the use of protective equipment
demic, debates in social media and studies about mental “stress”, “headache” and “anxiety”. Results: Tension- type
covering the skull in times of the COVID-19 pandemic, it
disorders have been received a great scientific interest. headache refers to a mild to moderate pain that is usually
is evident the possibility of the development of headache
Objective: The aim of this study is to investigate an asso- described as a feeling of a tight band around the head. It
in health professionals, due to the prolonged use of these
ciation between headaches and anxiety during the COVID is also the most common kind of headache presented in
accessories. Objective: To determine the prevalence of
19 pandemic, analyzing new and pre existing scenarios – clinics, expressed specially by pericranial muscular spasm
external compression headache caused by the use of
and therefore aggravated – of cephalus and mental disor- and important myofascial pain. Several factors can cause
face shields and other personal protective equipment
ders. Methods: This is a cross-sectional, retrospective and a tension headache, especially stressful situations that
(PPE) in healthcare workers exposed to the pandemic of
descriptive study with a quantitative approach. The data predispose to anxious episodes, like work problems, study
COVID-19. Methods: This was a non-intervention, quan-
was collected through an online form, on GoogleDocs, issues, and financial trouble. In this sense, it is clear that
titative approach, observational, cross-sectional study.
“Headache Prevalences during the Pandemic” between individuals who present classic anxiety symptoms, such
202 participants completed an online questionnaire,
september/2020 to january/2021.The inclusion criteria are: as muscle tension, fatigue and palpitation, usually also
after approval by the local Research Ethics Committee. Of
over 18 years of age, signing the Free Informed Consent have headache as a form of manifestation of an anxious
these, 8 were excluded because they used a cloth mask.
Term (FICT) available online. Exclusion criteria were par- state like a way of “alert” of an anxiety crisis, mainly in indi-
ICHD3 criteria were used to confirm the presence of pre-
ticipants who did not fully complete the questionnaire or viduals with Generalized Anxiety Disorder. Conclusion:
existing headache and external compression headache.
who live outside Brazil. To carry out the analysis of the Therefore, it is a possible to affirme that there is a strong
Kolmogorov-Smirnoff’s test and Ficher’s exact test were
variables, sociodemographic data were considered – sex, connection between episodes of tension-type headache
applied using SPSS software. Results: 1949 healthcare
age, ethnicity, and place of residence; as well as clinical in individuals who manifest anxious episodes frequently.
professionals were included in the sample, which com-
data – previous diagnosis of headache, symptoms or Thus, neglecting this association can result in an ineffec-
prised 71.6% (139) women and 28.4% (55) men. 79.4%
previous diagnosis of anxiety, the impact of social isola- tive treatment and it could also contribute to a decrease
(154) of the professionals were between 22-40 years of
tion with the headache, among others. Statistical proce- in the patient’s life quality.
age and 20.6% (40) were between 41-64 years of age. It
was observed that 20.6% (40) had headaches diagnosed dures are descriptive measures, frequency tables, mean
before the pandemic. Headaches prevalence associated and standard deviation. Results: In the present study,
Cefaleia
with PPE use was high (81%). Regarding new headaches, 59.3% of the volunteers (162, of which 147 were from the
62% (49) could be classified as external compression Southeast) reported headaches. The most prevalent was
headache, with 34 women. When calculating the prev- pulsatile headache, affecting 56.17% of the participants.
alence of external compression headache in relation to Regarding location of pain, most participants reported
the study sample, a value of 25.2% was obtained. To 15 bilateral pain (69.13%) and frontal headache was the most
of the 49 professionals (30.6%), headache was not asso- prevalent (51.85%). It was found that 70.7% (193) of the
ciated with any symptoms. Furthermore, the frequency participants state that there was a worsening of anxiety
of painful episodes was 4 to 7 days per month for most of symptoms during the COVID-19 pandemic and 36.3%
them (32.6%). Conclusion: Prevalence of external com- (99) report that they simultaneously observed worsening
pression headache caused by PPE use was high in the of headaches. In addition, stress was listed as an import-
study population, reaffirming the clinical characteristics ant headache trigger throughout the study. Conclusion:
of previous studies, and may have associated symptoms. The present study indicates the relation between anxiety
There was no significant difference between the types of and headaches, besides an increase of anxiety during the
PPE and the prevalence of the occurrence of external COVID-19 pandemic. Thus, the correlation of these data
compression headache, and a change in the preexisting proves, considering its prevalence and great impact on
headache pattern was also reported. patients’ quality of life, the importance of the Unified
Health System (SUS) to study and prioritize headaches
and mental disorders.
Cefaleia

Cefaleia

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XXX Congresso Brasileiro de Neurologia

TL 1106049 TL 1106139 TL 1106222

PREVALENCE AND IMPACT OF MIGRAINE IMPROVING EMERGENCY DEPARTMENT MIGRAINE HEADACHE ASSOCIATION WITH
AMONG HEALTH SCIENCES STUDENTS: A HEADACHE TREATMENT: THE VIRTUAL CARDIOVASCULAR AND CEREBROVASCULAR
CROSS-SECTIONAL STUDY HEADACHE TRAINING PROGRAM (VHTP) DISEASES
Carolina Matté Dagostini, Murillo Cesar Gionedis, Marcio Nattan Portes Souza, Marcelo Calderaro, Luisa Diogenes Queiroz, Júlio César Claudino dos
Arthur Aguzzoli, Yan de Assunção Bicca, Matheus João José Freitas de Carvalho, Evelyn Pacheco, Santos, Isadora Mônica Ponte de Oliveira, Leda
Machado Rech, Asdrubal Falavigna Renan Domingues, Gustavo Kuster, Cesar Villela, Maria Cavalcante Viana, Lêda Maria Veloso Bastos,
Tatiana Brandão, Saulo Ramos Ribeiro, Daniel C Maria Clara Borges Girão Peixoto, Rafaella Iughetti
Universidade de Caxias do Sul. Caxias do Sul RS, Bezerra da Costa, Victor Oliveira Araújo, Micael Porto Portela
Brazil Lima, Tiago Antoniol
Hospital Samaritano Higienópolis / UHG
carolmdagostini@hotmail.com Américas Serviços Médicos / UHG – Fortaleza – SP Centro Universitário Christus – Unichristus.
– Brazil Fortaleza CE, Brazil
Background: It has been shown that migraine has a Amil Assistência Médica / UHG Faculdade de Medicina, Centro Universitário
significant presence and negative impact on students’ Samaritano Botafogo. Rio de Janeiro RJ, Brazil Christus, UNICHRISTUS. Universidade Federal
quality of life and academic achievement. Health Sciences Américas Serviços Médicos / UHG. Rio de Janeiro de São Paulo. Laboratório de Neurociências,
Students are exposed to specific types of risk factors that RJ, Brazil Departamento de Neurologia e Neurocirurgia,
may contribute to the prevalence of migraine. Objective: Universidade Federal de São Paulo. Fortaleza CE,
This study aims to evaluate the presence and persistence marcionattan@gmail.com Brazil
of migraine in students of health services and its impacts. Centro Universitário Governador Ozanam Coelho.
Methods: This cross-sectional study was carried out at Background: Headache is among the most common Ubá MG, Brazil
the University of Caxias do Sul, Brazil. A web-based sur- causes of visits to the Emergency Department (ED), and
vey between the second semester of 2020 and the first migraine is the most common identifiable diagnosis in luisadiogenessss@gmail.com
semester of 2021 was sent to students of health sciences’ this context. The diagnosis of migraine is clinical, and neu-
course. The survey contained questions regarding demo- roimaging should not be performed routinely unless an Background: The migraine (MI) is one of the most com-
graphics, lifestyle, headache aspects and the ID-Migraine. alarm sign is identified in the history or physical examina- mon neurologic disorder, represented by recurring head-
The ID-Migraine™ was a questionnaire proposed in 2003 tion. Currently, the first-line treatment of acute migraine ache episodes associated with motor and somatosen-
as a way to identify migraine in outpatient primary care attacks should be performed with analgesics, non-steroid sory transitory disturbances, and is an important cause
and was found to be a valid and reliable screening instru- anti-inflammatory drugs, triptans, and antidopaminergic of incapacitation in the word. In this scenario, several
ment. It was the tool chosen to assess the prevalence of medications. Second-line treatment includes corticoids, studies propose the correlation between MI, specially
Migraine among the students. A multivariate model was magnesium, and nerve blocks. Opioids should be avoided with aura, and cerebrovascular diseases (CVAs) and car-
used to investigate potential associations with migraine for the treatment of migraine attacks2. Despite these rec- diovascular diseases (CVDs), due to pathophysiological
and disability reported. Results: The survey was responded ommendations, we identified a high use of opioids and aspects in common. However, the mechanisms of asso-
by 504 students. The majority (90.1%; N=379) of students a high rate of neuroimaging exams in the emergency ciation between these diseases are not well enlightened,
had headaches in the last three months. According to the department of a tertiary Hospital in Brazil. Objective: which difficults a better approach for the patients. The
ID-Migraine, 71.9% (N=350/454) of the students pre- To evaluate the impact of a virtual headache training study aims to contribute for a better understanding of
sented with migraine. To manage their headaches, 80.8% program (VHTP) for healthcare providers at the ED of the association between migraine and CVAs and CVDs.
(N=367/454) of the respondents had resorted to the use a tertiary Hospital in improving headache treatment. This article presents a narrative review of the literature
of pharmacotherapy. Only 20.9% (N=77/367) of them had Method: We evaluated preliminary data including all visits based on the analysis of scientific articles published from
these medications prescribed by a physician. There was to the ED with the final diagnosis of migraine (CID G43) 2012 to 2022, in PubMed and Science Direct, on MI and
no significant association (p<0.05) regarding both func- and unspecified headache (CID R51). The intervention its association with cardiovascular and cerebrovascular
tional impairment and migraine with semester, hours of consisted of an asynchronous virtual training program diseases. Two criterias were applied, titles that did not
work per day, time spent at the university per week and of 90 minutes, aimed to review the clinical presentation mention the thematic association between Migraine
time spent in physical exercises per day. Hours of study of the primary headaches, the alarm signs for secondary and CVDs/CVAs and abstracts that did not address the
were significant (p<0.05) on functional impairment. headaches, and an evidence-based treatment protocol theme migraine and cardiovascular and cerebrovascular
Conclusion: Migraine prevalence among health Science for primary headaches. Data were collected for 14 weeks diseases. After the application of the exclusion criterias,
students is high compared to other studies. The popula- before and 12 weeks after intervention. The primary end- was obtained in total, 50 articles remained. MI is a very
tion studied used self-medication to treat their pain. There point was the reduction in the rate of opioids prescription. frequent neurological disorder, being reported as one of
was no difference in functional impairment and migraine Secondary endpoints were the reduction in neuroimag- the most disabling diseases in the world, affecting the
when we consider the year of graduation, hours of work ing examens in the ED, reduction of inpatient admission quality of life and social functioning of the patients. It is
per day, time spent at the university per week and time rate, and duration of inpatient treatment. Results: we characterized by a pathological brain state that alters the
spent in physical exercises per day. Hours of study were included 87 headache visits in the pre-VHTP group, and patterns of neuronal activity, which can be accompanied
an important triggering factor for pain. 96 in the post-VHTP group. The opioid prescription for by the presence of aura, generating pain associated with
headache patients was reduced in 75.1% (p = 0.005). There several motor and somatosensory manifestations that
was no significant difference of the secondary outcomes. appear both prior to the moment of headache and after
Cefaleia Neuroimaging was performed for 55.4% vs 41.8% (p = the end of the pain crisis. In addition to these clinical asso-
0.16), mean length of ED visit was 249.3 vs 167.3 minutes ciations, MI can be related to an increased risk of devel-
(p = 0.23), inpatient admission rate was 5.5% vs 0.8% (p oping cardiovascular and cerebrovascular diseases, due
= 0.08) and length of inpatient admission was 2 vs 1 day to its path physiology related to endothelial dysfunction,
(p = 0.32). Conclusion: The Virtual Headache Training cortex spreading depression and increased coagulability
Program successfully improved headache treatment, markers and the inflammatory response, which can result
reducing the opioid prescription by 75.1%. Limitations: in vasospasms and consequent hypoperfusion of some
This is a single-center retrospective study with prelim- regions. This study discussed the relation between MI,
inary data from a small number of cases and a short especially with aura, and cerebrovascular and cardiovas-
period of observation. cular pathologies. Overall, it was evidenced that there is
an association of mechanisms between these disorders.
In this context, there is an urgent need for more specific
Cefaleia research and dissemination of data on the association of
Migraine and vascular heart and neurological diseases.

Cefaleia

218
XXX Congresso Brasileiro de Neurologia

TL 1106224 TL 1106442 TL 1106481

OVERVIEW OF HOSPITALIZATIONS DUE TO THE INCREASING NUMBER OF HEADACHES THE RELATIONSHIP BETWEEN TENSION-
HEADACHE IN BRAZIL FROM 2016 TO 2022 IN THE CONTEXT OF INFOXICATION AND IN TYPE HEADACHE (TTH) AND ANXIETY
THE POST-PANDEMIC WORLD DISORDER: A LITERATURE REVIEW
Bianca Berardo Pessoa Zirpoli, Daniel Cavalcanti
Sena, Maria Valeska Berardo Pessoa de Souza, Júlia Leite Xavier Bertrand, Beatriz Barrozo Alessandra Monteiro Camapum, Beatriz Barrozo
Maria Eduarda Iglesias Berardo Pessoa de Souza, Gonzalez Oliveira, Vitória de Mendes Sá Lazera, Gonzalez Oliveira, Juliana Lago de Araújo, Gabriel
Patryck Andrew Ribeiro de Melo Pontes Enzo Derick Guterres Oliveira, Juliana Lago de Pereira Sousa, Dayana Dourado de Oliveira
Araújo, Rita de Cássia Miranda
Universidade Católica de Pernambuco. Recife PE, CEUMA – São Luís – MA – Brazil
Brazil CEUMA – São Luís – MA – Brazil
Prefeitura do Recife. Recife PE, Brazil bbgo2000@yahoo.com.br
Faculdade Pernambucana de Saúde. Recife PE, bbgo2000@yahoo.com.br
Brazil Background: A headache is a pain with cephalic involve-
Background: At the current Digital Age, access to infor- ment, the most common neurological symptom and a
berardobianca@gmail.com mation is constant, whether through large communica- very frequent medical complaint. Anxiety worsens the
tion devices or through smartphones. In the context of perception of pain, intensifies it, increases its duration,
Background: Headache is a common cause of medical the Covid-19 pandemic, this problem has gotten worse, and, by an escalation mechanism, further increases the
consultation, not only in primary care but also in special- with not only the disclosure of necessary data, but also level of previous anxiety. In several clinical and epide-
ist neurology outpatient clinics. Despite most of Covid- the spread of misinformation, causing emotional and miological studies, a comorbidity especially focused on
19 symptoms being respiratory, there was an increase in mental overload. Given this, an increase in anxious and among patients with TTH is anxiety. Objective: To pres-
symptoms in the central and peripheral nervous system, depressive symptoms was noted. In addition, there were ent relevant information about the relationship of anxiety
including anosmia, ageusia and headache, possibly via neurological symptoms, among which the most common symptoms among TTH sufferers. Material and Methods:
direct viral injury, immunological mechanisms and the is headache, with emphasis on primary ones. Such head- This is a literature review that allows a broad approach to
angiotensin-converting enzyme 2 (ACE2) receptor, and aches occur without the usual clinical or laboratory tests tension-type headache and its relationship with anxiety,
hypoxia. It is estimated that with the COVID-19 pandemic showing any etiology, as in, for example, migraine. In these providing knowledge and incorporation of the applicability
there has been a five-fold increase in the incidence of cases, brain neurochemical disorders occur, involving definition of concepts, results: and studies. The collection
headache in the affected regions. There has also been neurotransmitter imbalance. In this bias, genetic and began in May 2022, so that a survey of data and studies
described a possible new daily persistent headache in the environmental factors act in these disorders. The present relevant to the research in question was carried out. The
aftermath of COVID-19. Therefore, it is fundamental to look study will address the environmental factor contribution. textual search was restricted to scientific publications
at the epidemiological profile of the most affected groups. Objective: To associate the impacts of disproportionate from the Scientific Electronic Library Online (SCIELO)
Methods: This consists of an ecological and descriptive information overload with the increase in the number of and PUBMED databases during the month of May of this
study of the Hospitalizations in Brazil, according to age headaches in the post-pandemic context. Methods: This year, using publications from the last 10 years, through the
and gender, from January 2016 to April 2022, for which is a literary review, in which research published in both following descriptors: “tension-type headache” and “anx-
data collection was obtained via the Health Information English and Portuguese between the years 2019-2022 on iety”. Original articles indexed in the period between 2012
(TAB- NET) and the SUS Hospital Information System Scielo, Google Scholar and PubMed were used. The fol- to 2022 were included. Results: Seven articles were found
(SIH / SUS). Results: During the past 7 years in Brazil lowing descriptors were used: “Headache”, “Information” in the initial search, and after analyzing the abstracts, 4
there were 61.324 admissions for headache, while the and “Covid-19”, with 5 articles selected. Results: Based on papers remained for this review. Among the primary head-
region with the highest prevalence was the Southeast, these articles it can be seen that, with the increase in the aches, tension-type headache stands out, characterized
with a total of 18.910 cases (30,84%), and subsequently use of electronic devices and with the excess of informa- by light or moderate compressive pain sensations and its
the Northeastern region, in which there were 18.346 tion available, incidence of headaches in the population in relation with anxiety disorder. It is subclassified accord-
cases (29,91%). The region with the lower incidence was all age groups has been increasing. Within the spectrum ing to its duration into infrequent episodic, constant, and
the Central-West, with only 3.138 cases (5,11%). The age of studying the cognitive function of the elderly, even chronic. Its pathophysiology is complex and involves
group with most cases was from 20 to 49 years, with the before the covid-19 pandemic, anxiety and depression peripheral and central pain mechanisms, and it must be
highest number of cases from 30 to 39 years, in both gen- were already shown to be associated with headache. differentiated from other types of headache. And through
ders (11.457 cases, of which 7.910 were female). There was However, this situation was intensified during the intox- the analyzed studies we concluded that the patient diag-
a predominance of females, representing 40.073 cases ication resulted from Covid-19 and headaches in elderly nosed with anxiety has a higher frequency of occurrence
(65,35%). From 2016 to 2021, there was an increase of people who have access to information proved to be an of headaches when compared to those without mental
4,64% in the number of Hospitalizations, with a progressive important health marker. In teenagers, excessive use of disorders. The characteristics of headache, frequency,
increase until 2019, the year with the highest incidence. computers was considered a risk factor for the develop- intensity, duration of crises were more aggravated in
Conclusion: Hence, women from 20-39 were the most ment of headache, especially in females. Furthermore, patients with anxiety. This disorder is preventable, with
affected, and the region with most Hospitalizations was there was a high prevalence of primary headache among changes in habits in order to reduce or eliminate trigger-
Southeast. In this case, it is fundamental to focus on the them, migraine being the most prevalent type, followed ing factors. Conclusion: Tension headache and anxiety,
availability of headache treatments especially in primary by tension-type headache. Conclusion: In this context, although recurrent, is underdiagnosed, usually due to
care, while also making policies and approaches with contributing factors are the excessive use of electronic lack of knowledge by general practitioners, a fact that may
individualized multidisciplinary planning, psychologi- devices and the information overload to which people result in the adoption of inappropriate treatment plans
cal resources and universalization of the access to such were exposed during the Covid-19 pandemic, causing and cause impairment in the quality of life of patients.
treatments. It is also important to make further studies to an immense emotional and mental damage and, finally,
assess the real incidence of such cases, even before the reflecting negatively on neurological health.
need of Hospitalization. Cefaleia

Cefaleia
Cefaleia

219
XXX Congresso Brasileiro de Neurologia

TL 1106652 TL 1106654 TL 1106655

“NOT OTHERWISE SPECIFIED HEADACHE” IN OCCURRENCE OF SILENT LACUNAR ANALYSIS OF CLINICAL AND VOLUMETRIC
EMERGENCY DEPARTMENT: AN ANALYSIS INFARCTS DETECTED BY VOLUMETRIC PARAMETERS OF WHITE MATTER LESIONS
OF 149,603 VISITS TO NINE UPA’S 24H IN ANALYSES OF MAGNETIC RESONANCE IDENTIFIED BY MAGNETIC RESONANCE
FORTALEZA IMAGING IN WOMEN WITH MIGRAINE IMAGING IN WOMEN WITH MIGRAINE
Flávio Clemente Deulefeu, Virgínia Angelica Silveira Fabíola Dach, Deborah Bevilaqua-Grossi, Gabriela Fabíola Dach, Debora Bevilaqua-Grossi, Carina
Reis, Fernanda Gadelha Severino, Jamille Soares Carvalho, Carina Pinheiro, Antônio Carlos dos Pinheiro, Nicoly Maciel, Gabriela Carvalho, Antônio
Moreira Alves, João José Freitas de Carvalho Santos, Nicoly Maciel, Natália de Oliveira Silva Carlos dos Santos, Natália de Oliveira Silva

Instituto de Saúde e Gestão Hospitalar. Fortaleza Faculdade de Medicina de Ribeirão Preto. Ribeirão Faculdade de Medicina de Ribeirão Preto. Ribeirão
CE, Brazil Preto SP, Brazil Preto SP, Brazil
Unichristus School of Medicine. Fortaleza CE, Brazil
fabioladach@yahoo.com.br fabioladach@yahoo.com.br
jjcarvalho@gmail.com
Background: The development of neuroimaging tech- Background: Migraine is a very prevalent primary head-
Background: Headache is one of the most common neuro- niques aroused interest in structural brain changes associ- ache characterized by attacks of pulsatile, unilateral pain,
logical symptoms and is almost a universal experience that ated with migraine. Silent lacunar infarcts (SLI) are related associated with nausea, photo and phonophobia. It is
requires medical attention on many occasions. Patients to cerebral microvascular disease and frequently found in believed that repeated headache episodes throughout life
often seek medical advice for headaches in primary or neuroimaging studies in migraine. Objective: To describe may cause structural changes in the central nervous sys-
secondary health care systems. In these scenarios, estab- the occurrence of ILS in different groups of migraineurs tem, especially in those patients with a high frequency of
lishing the correct diagnosis of headache and referring and to characterize them in terms of location. Methods: attacks and with aura subtype. White matter lesions (WML)
further treatment to a specialist is essential. Hundreds of Sixty patients were selected, without cerebrovascular risk are the most reported structural changes in neuroimaging
low-complexity Emergency Care Units (called UPAs 24h) factors, from the headache clinic of a tertiary neurology studies. Objective: This work uses volumetry and vox-
were opened throughout the country in the last decade. center. The volunteers were equally allocated into four el-based morphometry (VBM) techniques to analyze WML
Working 24/7, many UPA 24 h are one of the preferred groups according to the third International Classification in migraineurs. Methods: Sixty volunteers from a tertiary
places by patients to treat acute headaches. Objective: of Headache Disorders: Episodic migraine without aura headache center were selected and divided equally into
This study aims to evaluate the frequency of the “Not (MoA), Episodic migraine with aura (MA),Chronic four groups: episodic migraine without aura (MoA), epi-
otherwise specified headache diagnoses” in patients who migraine (CM) and control group (CG). All participants sodic migraine with aura (MA), chronic migraine (CM) and
seek UPA’s 24-h for headache Methods: We evaluated the were submitted in a 3T MRI scan with volumetric proto- controls (CG). The participants performed 3.0T magnetic
ED charts of 149,603 visits motivated by headache to nine cols. The acquired images were analyzed by two indepen- resonance imaging (MRI). WML variables were compared
UPAs 24h in Fortaleza, Ceará, from January 2017 to April dent neuroradiologists. Results: ILS were detected in only between groups and about the clinical characteristics of
2022. In addition to the demographic data, the care flow three volunteers, two of them from the MoA group and one the sample. Results: There were no differences in WML
chart, the MTS classification, the times of each step of care from the MA group; no ILS were identified in the MC and variables between groups. There was a positive weak cor-
(from risk classification to discharge or death), and the CG. Only one lesion was identified in each patient. Two relation between age and the number and total volume
final diagnosis coded by the attendant physician using the SLI were located in the frontal lobe and the other one in of WMLs, which persisted in the comparison categorized
International Classification of Diseases (ICD-10). Data the occipital lobe. No infratentorial SLI were identified by size and location by brain lobe, except for the occipital
were analyzed using statistical software, and a p <0.05 in our study. Conclusion: the occurrence of ILS was low lobe. Disease duration was positively correlated with the
was considered for significance. Results: The 149,603 in the sample, and presented in the anterior circulation. number and total volume of WML, but when the variable
consultations, which represented 3,5% of the total, were Prospective studies are needed to determine whether was controlled by age; the correlation was significant only
made by men and women (72 and 28% respectively) with migraine is related to a higher risk of SLI formation. for the insular lobe. Aura frequency was relevant for the
38.2 (± 14.6) years of age, on average. Regarding the diag- frontal and temporal lobe WMLs. There was no statisti-
nosis, only 25,121 (16.8%) patients received the diagnosis cally significant correlation with attack frequency, pain
of migraine, and 4,671 (3.1%) were diagnosed with “other Cefaleia intensity and use of prophylactic medication. Conclusion:
cephalic algic syndromes”. The remaining 119,811 (80.1%) The type of migraine does not influence the formation of
had the not otherwise specified headache discharge LSBs. Number and volume of WML were correlated with
diagnosis in their charts. Conclusion: The UPA´s 24 h insula, and the presence of frontal and temporal lobe
represents an important care channel for patients with WML were correlated with aura frequency.
acute headaches; however, most patients in this setting
do not receive the correct diagnosis, which undoubtedly
contributes to inadequate treatment and prognosis. A Cefaleia
training program for the health professionals who work
there is urgently needed to improve the care of thousands
of headache patients.

Cefaleia

220
XXX Congresso Brasileiro de Neurologia

TL 1105292 TL 1106590 TL 1104942

THE PREVALENCE OF VESTIBULAR BALANCE REHABILITATION WITH VIRTUAL ASSESSMENT OF THE PRESENCE OF
SYNDROMES IN A BRAZILIAN TERTIARY REALITY IN PATIENTS WITH HEREDITARY INDICATORS OF ATRIAL HEART DISEASE
CENTER SPASTIC PARAPLEGIA: A PRELIMINARY IN PATIENTS WITH ISCHEMIC STROKE
STUDY WITHOUT PREVIOUSLY DIAGNOSED ATRIAL
Fernando Henrique Magri Alves, Emanuelle Roberta
da Silva Aquino, Cristiana Borges Pereira FIBRILLATION
Bianca Simone Zeigelboim, Maria Izabel Rodrigues
Severiano, Geslaine Janaina B. Santos, Hélio Afonso Caroliny Trevisan Teixeira, Mayara Silva Marques,
Hospital das Clínicas, USP SP
G. Teive, Maria Renata José, Cristiano Miranda Julia Maria Rodruigues da Rocha, João Brainer
fernando.h.m.alves@gmail.com Araújo, Bianca Lopes Cavalcante Leão, Francisco Clares Andrade, Vanessa Rizelio, Kristel Beck
Manoel Branco Germiniani Merida
Background: Vestibular symptoms are common com-
UTP. Curitiba PR, Brazil Instituto de Neurologia de Curitiba. Curitiba PR,
plaints and sometimes challenging to discriminate among
Universidade Federal do Paraná. Curitiba PR, Brazil Brazil
different syndromes and etiologies. A great number of
these patients are referred to the neurologist, who must be Caroliny. Curitiba PR, Brazil
frankgerminiani@hotmail.com
able to determine whether there is a central or peripheral Caroliny. Curitiba PR, Brazil
cause for the problem, as the management and therapy Universidade Federal de São Paulo. São Paulo SP,
Background: Hereditary spastic paraplegia is a heteroge-
are very distinct in each situation. Objectives: The goal Brazil
neous group of hereditary degenerative diseases involv-
of this study was to quantify the most frequent vestibular ing the disorder of a single gene and is characterized by carol._teixeira@hotmail.com
syndromes in a tertiary dizziness reference department the progressive retrograde degeneration of fibers in the
Methods: Data of 1649 patients between 1999 and 2022 spinal cord. Among the symptoms, vestibulopathy is one Background: Ischemic Stroke may have paroxysmal
were evaluated retrospectively in our institution. These of the causes of discomfort and a decrease in quality of atrial fibrillation as a potential risk factor, which existed
data were analyzed and the diagnoses of these most fre- life. Objectives: Determine the benefits of vestibular before the ischemic event, but remained undiagnosed
quent vestibular syndromes were established by anam- rehabilitation involving virtual reality by comparing pre even with the etiological investigation. Objective: To
nesis, physical examination, and vestibular tests and intervention and post intervention assessments in indi- study whether the presence of markers of atrial heart
images when necessary. Results: The most common viduals with hereditary spastic paraplegia. Methods: disease in patients with stroke has a statistical correla-
syndromes were: benign paroxysmal positioning vertigo Randomized controlled clinical trial from the Rebec tion with the identification of AF at follow-up. Methods:
(BPPV) (24.2%), central vertigo (12.7%), migraine (8.6%), platform, were evaluated 40 patients. The interventions Retrospective longitudinal study with evaluation of data
perceptual postural persistent dizziness (PPPD) (7.8%), included vestibular rehabilitation with virtual reality using in electronic medical records of patients Hospitalized
Menière disease (6.5%), clinical non-neurological causes the Wii® console, Wii-Remote and Wii Balance Board and for stroke from 2014 to 2021. A total of 872 patients with
(7.4%), vestibular neuritis (4.9%), other causes (26.7%), pre- and postintervention assessments. Group I included complete data in medical records and without known AF
and undetermined (1.2%). Discussion: Our findings are in twenty volunteers who performed balance games. Group were analyzed. The markers of atriopathies studied were
similar to the literature, and show that the most frequent II included twenty volunteers who performed balance LA size, reduced ejection fraction, presence of intraven-
causes of vertigo can be treated and have good prognosis. games and muscle strength games. The sessions were tricular septal fibrosis, interatrial septal aneurysm or atrial
A small difference in number of some diagnosis may have performed twice a week for 10 weeks (total: 20 sessions). appendage thrombus identified on echocardiography.
different reasons such as publications new diagnostic Results: This study provides an assessment of the effec- Using Holter, we analyzed the presence of arrhythmias,
criteria for vestibular migraine, and changes the referral tiveness of a virtual reality vestibular rehabilitation pro- as well as the presence of extrasystoles. We also tabulated
system in our institution. Conclusion: The knowledge- gram in decreasing the progression of the imbalance data such as age, image topography, main comorbidi-
ment of the epidemiology in a reference dizziness depart- (above 50%) in the hereditary spastic paraplegia, and ties and failure to use antithrombotics in the population
ment is important for the best planning and conducting this treatment can be personalized and affordable. It is studied. Results: We identified that LA greater than or
those patients. The early diagnosis and most adequate observed that the improvement was more evident in the equal to 42 mm, age greater than or equal to 70 years,
treatment are critical since these syndromes may have a group that performed the muscle strength exercises con- the presence of septal aneurysm and NHISS greater than
good prognosis when correctly handled. comitantly (56.4%) .Conclusion: The study contributed to or equal to 6 at admission are variables with statistical
the reduction the progressive effect of hereditary spastic significance associated with the risk of developing atrial
paraplegia and serve as an alternative treatment option fibrillation in the follow-up. With these variables, it was
Distúrbio Vestibulares e do Equilíbrio that is accessible and inexpensive. possible to structure a risk score for the diagnosis of AF
in the follow-up, in which the patient who scores equal
to or more than 2 points has a risk of AF in the follow-up
Distúrbio Vestibulares e do Equilíbrio 5 times greater. Conclusion: Based on the present study,
we concluded that in the sample of stroke patients from
the Instituto de Neurologia de Curitiba, without known
AF before the event, the presence of markers of atrial heart
disease showed a positive correlation with the detection
of AF at follow-up. In addition, it was possible to apply
an AF risk score which could be validated in larger sam-
ples in future studies.

Doença Cerebrovascular

221
XXX Congresso Brasileiro de Neurologia

TL 1105123 TL 1105134 TL 1105168

CHARACTERIZATION AND FUNCTIONALITY FUNCTIONAL BIOMARKERS OF TRUNK AND BILATERAL THALAMIC INFARCTION
OF INDIVIDUALS POST STROKE OF THE LIMB CONTROL AND QUALITY OF LIFE IN PRESENTED AS A LOSS OF CONSCIOUSNESS
NORTHERN PLATEAU OF SANTA CATARINA: POST-STROKE PATIENTS
Larissa Baccoli de Souza, Beatriz Marques dos
ASSISTED IN STROKE UNIT Santos, Jasson José Moscon Neto, Alexia Carneiro
Chelin Auswaldt Steclan, Karine Haag, Leonardo
Adriélle da Costa, Oscar Nelson Reimann Junior, Trindade Buffara, Aline Haag, Ivana da Rosa Ieskik, de Almeida, Hyago Casimiro Mendes de Oliveira,
Daniele Peres, Jonathan Wei Ting Wen Liu, Leonardo Jaqueline Sueli Horodeski, Oscar Nelson Reimman Amanda Loureiro de Toledo, André Luiz de Rezende,
Trindade Buffara, Stephanie Lindner, Arlindo Junior, Stefan Moreira, Diogo Pasquali Nones, Sônia Maria César de Azevedo Silva
Américo de Oliveira, Diogo Pasquali Nones, Chelin Michael Ricardo Lang
Instituto de Assistência Médica ao Servidor do
Auswaldt Steclan, Michael Ricardo Lang Estado de São Paulo – IAMSPE
Universidade do Contestado. Mafra SC, Brazil
Universidade do Sul de Santa Catarina Unisul NEUROMAX; Hospital São Vicente de Paulo – Mafra/
larissabaccoli@hotmail.com
NEUROMAX; Hospital São Vicente de Paulo – Mafra/ SC – Mafra – SC – Brazil
SC – Mafra – SC – Brazil NEUROMAX; Hospital São Vicente de Paulo – Mafra/
Case Presentation: A 62-year-old female presented in the
Centro Universitário Avantis – Balneário Camboriú SC; Universidade do Contestado – NUPESC, Mafra/
emergency department with sudden loss of consciousness.
– SC – Brazil SC. – Mafra – SC – Brazil
At admission, the patient was conscious but confused.
Hospital Universitário Cajuru. Curitiba PR, Brazil She had clinical history of hypertension. On examination,
chelinsteclan@gmail.com
Universidade do Contestado – NUPESC – Mafra – she was somnolent, and the rest of the physical examina-
SC – Brazil tion was unremarkable. Her electrocardiogram showed
Background: Stroke has a great impact on the lives of many
NEUROMAX; Hospital Universitário Santa Terezinha a sinus rhythm. Echocardiogram reported concentric
individuals. The identification of post-stroke biomarkers
– Joaçaba/SC – Joaçaba – SC – Brazil remodeling of the left ventricle. A non-contrast CT scan
is important to assume functional independence and the
Universidade do Contestado – NUPESC; revealed a hypoattenuation in the medial portion of both
impact of the disease on the patient, family and society.
NEUROMAX. – Mafra – SC – Brazil thalami, corroborated by MRI. Angio-CT demonstrated
The present study aimed to characterize the functional
chelinsteclan@gmail.com capacity of post-stroke patients, evaluated by the applica- stenosis in the distal V4 segment of the left vertebral
tion of functional scales for the trunk (Trust Control Test, artery. Discussion: The thalamus is part of the dienceph-
Background: Stroke is known as the most disabling TCT) and upper limb (Frenchay Arm Scale), in addition to alon and is the relay center of the brain, retransmitting
chronic disease, affecting not only the individual, but scale for the assessment of quality of life after stroke using information between the cerebral cortex and subcortical
also his family and society as a whole, mainly caused the Specific Quality of Life Scale for stroke (EQVE-AVE). structures. The inferolateral, polar, paramedian, and pos-
by the significant reduction in the individual’s level of This study was carried out with patients treated in stroke terior choroidal arteries are the main arteries that supply
independence.Therefore, the objective was to charac- unitat that is reference in the Planalto Norte in the state of blood to the thalamus. There is considerable variation in
terize post-stroke individuals Hospitalized in U-stroke, Santa Catarina, including total of 20 patients, evaluated in the territory supplied by each artery. Gerard Percheron
regional reference for the North Plateau of state os SC in period of six months (out of a total of 48 months of study). described four variants of paramedian artery that supply
terms of epidemiological, clinical and functional profile. Therefore, the mean age range was 67±17.6 years, 55% the mediodorsal and intralaminar thalamic nuclei and
Prospective, observational and longitudinal study carried were female, and the mean NIHSS for the first 24 hours occasionally the upper brainstem. One of the variants is
out between Nov/2019 to Dec/2020. With individuals ?18 was 9.7. It was evidenced as a predominant risk factor for the artery of Percheron. It arises from P1 segment of the
years old; post-stroke confirmed by CT or MRI. Individuals SAH (90%), sedentary lifestyle (90%) and DM (45%). As posterior cerebral artery as a single artery and supplies
with other diseases and conditions that could lead to for functional capacity by TCT, the Results: show great blood to the bilateral paramedian thalamic nuclei and
motor deficits or functional changes other than the stroke variability, obtaining an average of 51.9±36.03. In this test, occasionally the rostral mesencephalon. Occlusion of
were excluded. Sociodemographic data, ICTUS and ther- the examinee is proposed to perform 4 maneuvers depen- the artery of Percheron can lead to a bilateral parame-
apeutic window were analyzed. The TOAST scale, NIHSS, dent on the skeleton, muscle and axial nerves, responsible dian thalamic infarct. It can present with a variety of
Functional Independence Measure (MIF) and Modified for stability and postural integrity – the main disabilities clinical manifestations such as an altered mental status,
Rankin Scale (ERm) were also applied at discharge. ERm present were: ability to balance while sitting on the edge increased somnolence, memory impairment, a vertical
was also measured at 30 and 60 days after stroke, using of the bed with the feet off the floor; activity of rolling to gaze palsy, aphasia, hemiplegia, movement disorder,
the brazilian and validated version of the interview via the compromised side and rolling to the healthy side. In cerebellar ataxia and even coma. Conclusion: Due to
telephone contact. Sample composed of 44 patients; 45 the Frenchay Arm Scale, the mean score was 2.4±2.27 the large number of possible differential diagnoses, vigi-
to 92 years old; 79.5% elderly (>61 years), 16% 46-60 years, points (from 0 to 5 points), which reflects in a heteroge- lance impairment poses a common diagnostic challenge.
4.5% 31-45 years and no young adults (21-30 years). 56.8% neous sample regarding the capacity of proximal control The occlusion of the artery of Perhceron, an anatomical
were male and 88.6% self-declared white. 96% had modifi- of the upper limb and manual dexterity. On the analysis variation whereby both paramedian arteries arise from
able risk factors; 79% hypertensive (only 73% undergoing of quality of life, through EQVE-AVE, an average value of a common P1, often leads to a bilateral paramedian tha-
treatment); 22.7% with Atrial Fibrillation (only 4.5% using 125.3±48.8 points were obtained, which reflects their sig- lamic infarction. Thalamic infarcts account for 11% of
anticoagulants); 48% with a previous history of stroke/ nificant impairment – on this, the most affected domains vertebrobasilar infarcts, of which 4-18% are due to the
transitory stroke without sequelae; 84% considered sed- were Mood, Self-care and Social Role; the least affected artery of Percheron occlusion and only 0.6% of cerebral
entary. Only 9% were eligible for thrombolytic therapy. In were work, energy and family roles. Thus, the present study infarctions are bilateral thalamic. In a case with no obvi-
tasks of daily living according to the total FIM: they were points to a positive correlation on functional findings and ous cause for sudden loss of consciousness, occlusion of
predominantly dependent (complete, 54%; and moderate, post-discharge reflexes, mainly in emotional and social the artery of Percheron should be considered as one of
39%). Considering the motor FIM classification, the levels aspects, not showing significant loss in relation to work the differentials. The severity of vigilance impairment is
of functionality were 70% complete dependent and 23% (possibly because the majority are already out of the job significantly more pronounced in bilateral paramedian
moderate. As for the functional evolution by Rankin, at the market). Interestingly, energy and disposition were also infarction than in unilateral lesions.
time of discharge, 82% of the individuals were dependent. less affected. Thus, the important correlation between
After thirty days, 57% were able to walk independently and the loss of trunk and limb functionality and quality of
life in relation to mood and self-care is highlighted here. Doença Cerebrovascular
seven died. After sixty days, 27% had negligible disability;
29% moderate disability; 23% reported severe disability
and one died. The findings point to important correlation
between the prevalence of modifiable risk factors and Doença Cerebrovascular
functional disability even after Hospital discharge. This
scenario reflects on the population of the Planalto Norte
Catarinense and the necessary health care measures,
thus enabling the design of strategic measures that aim
to meet the characteristics of the population.

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IMPACT OF COVID-19 ON ACUTE STROKE NEUROPSYCHIATRIC DISORDERS IN POST- EPIDEMIOLOGICAL AND CLINICAL
CARE STROKE PATIENTS CHARACTERISTICS OF ANEURYSMAL
SUBARACHNOID HEMORRHAGE PATIENTS
Renann Pirola, Marcele Almeida, Bárbara Silva da Eduardo Antonio Rosa, Viviane de Hiroki Flumignan
Fonseca, Adriana B Conforto Zetola, Marco Antonio do Socorro Marques Ribeiro IN A BRAZILIAN TERTIARY NEUROINTENSIVE
Bessa CRITICAL CARE UNIT
Hospital das Clínicas da Faculdade de Medicina da
Universidade de São Paulo. São Paulo SP, Brazil Universidade Federal do Paraná. Curitiba PR, Brazil Marta Rodrigues Carvalho, Beatriz Schmidt Dal
Berto, Amelia Maia Rodrigues, Lorena Ohrana Braz
adriana.conforto@gmail.com viviane.zetola@gmail.com Prudente, Edmilson Leal Bastos Moura, Flávio Faria
Costa Pereira
Background: The COVID-19 pandemic had impact on Background: Stroke patients have a high prevalence
stroke care around the world, but middle and low-income of neuropsychiatric disorders. Post-stroke depres- Hospital de Base do Distrito Federal. Brasília DF,
countries may have struggled more to adapt to the rapid sion, for instance, can affect around a third of these Brazil
changes in workflow because of its more fragile health care patients, in addition to the occurrence of anxiety disor- marta_rodrigues12@hotmail.com
systems. At Hospital das Clínicas/University of São Paulo, ders, post-traumatic stress disorder, bipolar disorder and
major changes were made in the stroke workflow after the others. However, psychiatric diagnosis and treatment is
Background: Subarachnoid hemorrhage (SAH) is a rare
onset of the pandemic. Objectives: To assess changes in still limited. Objectives: We aimed to identify the preva-
and life-threatening cerebrovascular disease. Ruptured
metrics of acute ischemic stroke care at a large-volume lence of neuropsychiatric disorders in patients previously
aneurysms are the most common cause of atraumatic
Hospital, before and after the pandemic. Methods: In diagnosed with stroke and compare commonly used
SAH. It is one of the main medical emergencies associ-
this prospective, observational study we compared key depression and anxiety assessment scales to a structured
ated with high rates of morbidity and mortality and years
performance measures collected from the University of interview, in order to assess their reliability in this popu-
lived with disability. In Brazil epidemiology of aneurys-
São Paulo Stroke Data Base, one year before and one year lation. Methods: We evaluated ischemic stroke patients.
mal SAH remains unknown. Objectives: To evaluate
after the report of the first cases of COVID-19 in São Paulo. Sociodemographic, health data and information on brain
epidemiological and clinical data and outcomes such as
Results: We analyzed 1.584 stroke admissions: 797 from lesion location were collected and the Mini-International
mortality, time of mechanical ventilation of aneurysmal
March 16, 2019 to March 15, 2020 and 787 from March 16, Neuropsychiatric Interview (M.I.N.I) was applied for diag-
subarachnoid hemorrhage patients interned in a ter-
2020 to March 15, 2021. There were no statistically signifi- nosis of neuropsychiatric disorders. The Beck (BDI-II and
tiary neurointensive critical care unit. Methods: This is a
cant differences between patients admitted during the two BAI) and Hamilton (HAM-D, HAM-A) depression and anx-
cross-sectional study. Patients with SAH admitted to the
periods regarding age, sex or NIH Stroke Scale (NIHSS) iety scales were then applied, at same day, for comparison
neurointensive critical care unit (neuroICU) in a tertiary
scores. Before the pandemic, 132 procedures of intrave- and analysis. Results: Of 81 included participants, 45.7%
care public Hospital were eligible. The inclusion criteria
nous thrombolysis (IVT) and 29 mechanical thrombecto- were diagnosed with at least one neuropsychiatric disor-
were a minimum stay in the intensive care unit (ICU) of
mies (MT) were performed and afterwards, 110 IVT and 19 der. Post-stroke depression had the highest prevalence
24 h, due to subarachnoid hemorrhage from a nontrau-
MT. There was no statistically significant difference in the (35.8%), followed by anxiety disorders (34.6%), post-trau-
matic spontaneously ruptured cerebral aneurysm, and
monthly average between the one-year periods for stroke matic stress disorder (7.4%), obsessive-compulsive disor-
Hospital admission within 24 h after the onset of symp-
admissions, IVT or MT. After the pandemic, there was an der (6.2%), and other (4.93%). Only 14.8% of participants
toms. Results: Our population was mainly composed
increase in ictus-to-admission and door-to-imaging (DTI) had a psychiatric diagnosis at the moment of the inter-
by women (67.2%). The most reported symptoms were
times compared to before, but there were no statistically view. Brain lesion location was not statistically relevant.
syncope (26%), isolated headache (23.2%), headache
significant differences regarding door-to-needle time The depression rating scales BDI-II and HAM-D had an
and syncope (16.3%). Twenty-eight percent presented
(DTN) or door-to-puncture (DTP) times. Conclusion: accuracy of 97% and 96%, respectively, when compared
Hunt Hess Scale grade II and 62.1% presented Fisher
In the largest medical center from Latin America, despite to M.I.N.I as the gold standard. The anxiety rating scales,
scale grade 4. Sixty-eight percent required mechanical
significant increases in delays in the interval from onset of BAI and HAM-A, accuracy of 91% and 92%, respectively.
ventilation, 48.6% presented vasospasm, 5.1% presented
symptoms of stroke to admission, as well as from admis- Conclusion: The prevalence of neuropsychiatric disorders
rebleeding, 36.7% acute hypertensive hydrocephalus,
sion to performance of CT scans, median DTN times did in post stroke patients is high, being underdiagnosed and
40.7% died. Females presented higher mean of age than
not significantly change and remained below 45 minutes. undertreated. The Beck and Hamilton depression and
males (women: 57.21±12.20 years; men: 51.34±12.54
Median DTP times did not significantly change, possibly anxiety scales can be used in this population with good
years; p=0.03) and also had the longest waiting time
due to the limited power, given the relatively low number accuracy and reliability, improving screening, diagnosis
for an intensive care unit bed (women: 6.12±6.71 days;
of thrombectomy procedures performed. Training of the and follow-up of these patients.
4.21±3.56 days; p=0.01). When stratified by sex there
stroke team and maintenance of high volumes of patients
was no statistical significance among outcomes such as
may have contributed to the lack of significant difference
Doença Cerebrovascular rebleeding, vasospasm, delayed cerebral ischemia, acute
in key performance metrics.
hypertensive hydrocephalus, death. When stratified by
type of discharge, death was statistically associated with
Doença Cerebrovascular vasospasm (OR 2.138 p=0.01) and acute hypertensive
hydrocephalus (OR 2.136 p=0.01). Conclusion: This study
can address relevant data about epidemiological and clin-
ical factors involving subarachnoid hemorrhage patients.
As reported in literature the majority of patients were
female. Percentages of complications are similar from
the previous reported. It is noteworthy that women spent
more time waiting for a specialized bed.

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HEADACHE AT THE CHRONIC STAGE OF EPIDEMIOLOGICAL ASPECTS OF PATIENTS UNUSUAL PRESENTATIONS OF SUDDEN
CEREBRAL VENOUS THROMBOSIS AFFLICTED BY STROKE IN REFERRAL FOCAL NEUROLOGICAL DEFICIT: “STROKE
SERVICES OF THE PAJEÚ MICROREGION IN CHAMELEONS”
Alexandre Bossoni, Mario Peres, Ida Fortini, Adriana
Bastos Conforto THE STATE OF PERNAMBUCO
Bianca Maranhão Vieira, Carlos Gonzaga Melo
Lílian Karine Machado Souza, Joab Lins Serafim, Filho, Victória de Menezes Sá Lazeira, Enzo Derick
Hospital das Clínicas da Faculdade de Medicina da
Ariel Lucas Medeiros Silva, Amanda Karla Alves Guterres Oliveira, Juliana Lago de Araújo, Dayana
Universidade de São Paulo. São Paulo SP, Brazil
Gomes Silva, Laiza Paula Candido Melo, Debora Dourado de Oliveira Costa
HIAE
Rayssa Siqueira Silva, José Jefferson Silva
UNICEUMA. São Luís MA, Brazil
adriana.conforto@gmail.com Cavalcanti Lins, Paulo Victor Machado Souza, Rita
Cassia Oliveira Angelo, Americo Danuzio Pereira biancamavi2000@gmail.com
Background: Headache is the most frequent symptom Oliveira
of cerebral venous thrombosis (CVT) but there is limited Background: Stroke is a medical emergency, present with
information about the frequency and phenotype of head- Universidade de Pernambuco. Serra Talhada PE,
sudden onset focal neurologic disorder and it may lead to
ache, weeks to months after CVT (post-CVT headache, Brazil
severe disability or death in worse case scenarios. There
PCH). Objective: To assess the frequency, characteristics Universidade Federal de Campina Grande – Campus
are false-negatives when the clinical sign suggests other
and predictors of PCH. Methods: In this cross-sectional Campina Grande. Campina Grande PB, Brazil
disorder conditions. These are known as “stroke chame-
study, the frequency and characteristics of PCH were lilian.souza@upe.br leons”. Objective: Trace the unusual presentations of sud-
assessed in CVT survivors. Patients were interviewed den focal neurological deficit caused by stroke capable of
between 6 months and 5 years after the CVT diagnosis. Background: The profile of patients afflicted by stroke dismissing the diagnostic suspicion and the appropriate
Clinical and imaging characteristics at the time of CVT differs between countries and geographical regions. An treatment. Method: This study is a literature review. The
diagnosis, as well as history of headache prior to CVT area’s demographic and socioeconomic aspects have a chosen database was PUBMED, with keywords “stroke
were compared in subjects with (GroupPCH) and with- direct impact on stroke determinant risk factors,whose chameleon” and “stroke”. Inclusion criteria were reviews,
out PCH (Groupcontrol). Results: Subjects (n=100; 82% identification is necessary for the development of pri- systematic reviews, clinical trials and meta-analysis with
women) were assessed, on average, at 1.1 ± 1.6 years mary and secondary prevention strategies. In Brazil, the free full text available, published between 2017 and 2022.
of follow-up. PCH was present in 59% of the patients, availability of epidemiological data is low, which reflects Results: The common presenting symptoms include
had phenotypes of tension-type-like headache in 31/59 the reality of regions with higher sociodemographic dizziness/vertigo without trigger symptoms, nausea/
(52.6%) and of migraine-like headache, in 16/59 (27.1%). indexes. In Pernambuco, most of all in the “Sertão do vomiting, confusion, decreased level of consciousness,
History of primary headache prior to CVT was significantly Pajeú” region, there aren’t any available studies with this altered mental status, generalized weakness, and severe
more common (OR: 6.4; 95% CI: 1.7-36.3) in GroupPCH outline. Objective: Trace the epidemiological profile of hypertension. In addition, it is presented with wrist drop
(33.9%) than in Groupcontrol (7.3%). Conclusion: PCH patients with stroke admitted into the emergency services syndrome purely motor or sensorimotor with hypoto-
was present in more than half of the patients. History of of “Serra Talhada/PE”, reference for 17 cities of the Pajeú nia, decreased myotatic reflexes and normal superficial
prior headache may be a risk factor for PCH. Prospective microregion. Method: Descriptive, transversal study. reflexes during the acute phase of stroke in precentral
studies are required to confirm these findings and deter- The instruments used were the Individual Registration gyrus. Futhermore, transient global amnesia, hyperki-
mine mechanisms, as well as interventions for prevention File of SUS and Evaluation File elaborated according to netic movement disorders defined as abnormal, repeti-
and treatment of PCH. the protocol of the brazilian Society of Cerebrovascular tive, involuntary movements rarely occur in acute stroke
Diseases. Results: The sample consisted of 114 partic- presentations. Among these, are the Alien limb syndrome,
ipants with male predominance (n=64; 56,1%), black consisting of involuntary movements and foreign-sense
Doença Cerebrovascular of limb, the Anton’s syndrome, the denial of blindness
self-declaration (n=86; 75,5%) and higher distribution in
the 61-80 age group (n=57; 50%). Most from Pernambuco and confabulation of visual sensory information, and the
(n=109; 95,6%), with 56,1% (n=64) from Serra Talhada/PE. Gerstmann syndrome consists of a tetrad of symptoms:
Around 48% (n=55) were farmers; 36,8% (n=42) had no agraphia, acalculia, finger agnosia, and right–left con-
schooling and 42,1% (n=48) had incomplete elementary fusion. A few stroke chameleon cases are diagnosed as
school. The main comorbidities observed were hyperten- psychiatric diseases. Conclusion: It appears that detailed
sion (n=93; 81,6%) and diabetes (n=42; 36,8%); despite history taking, comprehensive neurological examination,
that, 74,5% (n= 85) did not follow the diet. As for the life and appropriate imaging studies are important to identify
habits, approximately 30% (n=34) smoke; 12% (n=14) the clinic mainfestation and, when acute onset symptoms
consume alcoholic beverages 3 times a week and 87% are present, stroke should be considered.
(n=99) are sedentary. The alteration on the conscious-
ness level was light at 41,2% (n=47) and the neurological
compromise was severe at 41,2% (n=47). The predominant Doença Cerebrovascular
stroke type was ischemic (n=101; 88,6%) and the etiology
undetermined at 45,6% (n=52). Conclusion: The study
made it possible to trace the epidemiological profile of
the patients attended at the Hospitals of reference of the
“Sertão do Pajeú”, identifying the predominance of the
elderly, males and black people, reaffirming some of the
main risk factors for stroke, such as hypertension, a sed-
entary lifestyle and bad dietary control of comorbidities.
The knowledge of stroke determinant characteristics in
this group shows the importance of the development of
prevention strategies directed at the region’s profile for
control over the main modifiable risk factors.

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EIGHT YEARS OF EXPERIENCE WITH PREDICTORS OF HEMORRHAGIC STROKE ETIOLOGIES IN PATIENTS WITH
STROKE PROTOCOL IN A HOSPITAL IN THE TRANSFORMATION IN ISCHEMIC STROKE COVID-19
COUNTRYSIDE OF MINAS GERAIS PATIENTS: EXPERIENCE OF A BRAZILIAN
Iago Navas Perissinotti, Marcele Schettini de
TERTIARY HOSPITAL Almeida, Giovanna Mattos Ferreira, Barbara da Silva
Paulo Prado Vasconcelos, Igor Fortunato da Silva,
Dayane Menezes Terra, Roberta Bessa Veloso Silva, Mayara Silva Marques, Caroliny Trevisan Teixeira, Fonseca, Felipe Borelli Del Guerra, Gisela Tinone,
Luiza Barbosa Brito, Camila Moreira Carvalho Dias, Vanessa Rizelio, João Brainer Clares Andrade, Adriana Bastos Conforto
Rodrigo Bitencourt Ribeiro, Caio Fagundes Godinho, Daniel Wallbach Peruffo, Raissa Kaori Ikeda, Kristel Universidade de São Paulo. São Paulo SP, Brazil
João Henrique Orsi Neto, Maria Luísa Marcão Larisa Back Merida
Tavares iagonavas@gmail.com
Instituto de Neurologia de Curitiba. Curitiba PR,
Santa Casa de Misericórdia de Passos – Passos – Brazil Background: Patients with severe COVID-19 may have an
MG – Brazil Universidade Federal de São Paulo. São Paulo SP, increased risk of venous and arterial thrombotic events,
Universidade José do Rosário Vellano – Passos – Brazil including ischemic stroke (IS). It has been hypothesized
MG – Brazil Centro Universitário São Camilo that the infection by SARS-CoV-2 alone may be a risk
Universidade José do Rosário Vellano – Alfenas –
maysmarques@gmail.com factor for IS, particularly in young subjects. We designed
MG – Brazil
an observational retrospective cohort to compare stroke
Universidade do Estado de Minas Gerais – Passos
Background: Hemorrhagic transformation (HT) is one of etiologies in patients with IS, with (ISCOVID+) or without
– MG – Brazil
the most common stroke-related complications. Predictors (ISCOVID-) SARS-CoV-2 infection, defined by molecular
igor.fortunato@aluno.unifenas.br of this complication are still unclear in the literature and detection of SARS-CoV-2 before the stroke, or within 15
understanding them may be a resource of unique clinical days after stroke or detection of serum antibodies before or
Background: Stroke is the second most frequent cause of value to predict this dreaded condition. Objective: Identify within 30 days after the IS. The primary outcome was the
death in Brazil, and the main cause of disability. The use predictive factors for HT in patients with ischemic stroke stroke etiology according to the Causative Classification
of care protocol guides the development of best practices submitted or not to reperfusion therapies (RTs). Methods: System for Ischemic Stroke (CCS) algorithm. In addition,
in health work processes, and can directly impact the We analyzed retrospectively data from all patients admit- two subgroups were determined for the category of Other
indicators related to stroke. Objective: To express data ted to a South-brazilian Stroke Center, from 2017 to 2019. uncommon Causes (OC): iatrogenic causes (OC-IC) and
and evaluate the evolution of quality indicators related to Results: 821 patients were included in the analyzed sam- Other uncommon Causes excluding iatrogenic (OC-EI). A
stroke over 8 years after the adoption of care protocols by a ple. The prevalence of any HT was 9.7%. Thrombolysis total of 212 patients were included (160 in ISCOVID+ and
Hospital in the countryside of Minas Gerais, which serves with thrombectomy (n=12) was independently associated 42 in ISCOVID-). Cardio-aortic embolism (CE) was the
approximately 400,000 inhabitants. Method: Descriptive, with TH (OR 4.7, 95% CI 1.4-16.1, p=0.013). Thrombolysis most common etiology in the two groups. Two patients in
cross-sectional, documentary study that used second- with tPA was not associated with TH (p=0.1), in contrast the ISCOVID+ group but none in the ISCOVID-group had
ary data collected from the medical records of patients to performing thrombectomy alone (n=12/805, 1.5%). In unexplained thrombosis of the common carotid artery.
diagnosed with stroke during the years 2014 and 2021. general, TH was not related to a worse clinical outcome There were independent associations between younger
Results: In the analysis, the chi-square test was used, at (mRS3-6) in adjusted analysis. Conclusion: The value of age (RR 0.91, 95% CI 0.87 – 0.95) as well as COVID-19
the nominal level of 5% of significance, which indicated a low ASPECTS score, high admission NIHSS, and cardioem- infection (RR 4.08; CI 95% 1.03 – 16.14) and IS due to
significant difference in all data presented. In 8 years, 2,608 bolic etiology were possible predictors for HT in patients OC-EI. Classic stroke etiologies accounted for most of
cases of stroke were managed, of which 2,083 (79.87%) who did not undergo rt-PA thrombolysis. the cases of IS and concomitant COVID-19 infection, but
were of ischemic type and 525 (20.13%) were hemor- large-artery thrombosis was more common in infected
rhagic; of the ischemic strokes, 142 were thrombolysed than in non-infected patients.
and another 165 were admitted in a timely manner for Doença Cerebrovascular
thrombolysis, but this was not performed due to contra-
indications to the use of thrombolytics or failure in care. Doença Cerebrovascular
The number of thrombolysis performed in this period of
time were, respectively: 4, 18, 15, 23, 13, 18, 24, and 27.
Of the patients who arrived at the Hospital less than 4.5
hours after the onset of symptoms, the percentage that
had cranial computerized tomography (CCT) performed
in less than 25 minutes between 2018 and 2021 were,
respectively: 55.9, 69.4, 62.2, and 76.9. Of all the stroke
cases, 601 (23.04%) evolved to in-Hospital death, the
others were discharged. Stroke mortality rates between
2014 and 2021 in percentages were, respectively: 23.4,
24.4, 23.5, 18.9, 21.5, 21.4, 26.0, and 24.2. Conclusion:
The adoption of the protocol makes it possible to observe
progress in quality of service indicators, such as CCT
execution time and number of thrombolysis performed.
There is also a progressive drop in mortality between 2014
and 2019, but this rise again, reaching peaks in the years
of the Covid-19 pandemic, probably mortality increased
in this period due to the absence of 1st and 2nd preven-
tion, which made strokes more frequent and more severe
during this period. Therefore, it is stated that the use of
protocols organizes the service and directly impacts stroke
mortality. Remembering that the real improvement in the
indicators comes in health policies in all areas of health.

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ANALYSIS OF THE FUNCTIONAL CLINICAL AND EPIDEMIOLOGICAL PROFILE LACUNAR STROKE AS A PREDICTOR OF
CHARACTERISTICS OF THE PATENT OF MEDICAL CARE IN ACUTE STROKE AT THE MORE FAVORABLE OUTCOMES: THE
FORAMEN OVAL AND DIAGNOSTIC EMERGENCY ROOM AT AMECOR HOSPITAL RELEVANCE OF BAMFORD CLASSIFICATION
ACCURACY USING CONTRASTED (CUIABÁ-MT) WHEN EVALUATING ISCHEMIC BRAIN
TRANSCRANIAL DOPPLER EVENTS AND PROGNOSTIC PREDICTION
Beatriz Fulador, Victor Hugo Souza Silva Gomes,
Rebeca Teixeira Costa, Viviane de HIROKI Klesia Adaynny Rodrigues, Isadora Constatini Joab Lins Serafim, Lílian Karine Machado Souza,
FLUMIGNAN ZÉTOLA, Marcos Christiano Lange, Soares Andrade, Bianca Barbosa Araldi, Bruno Laiza Paula Candido Melo, Amanda Karla Alves
Gabriela Caetano Lopes Martins, Gabriel Pereira Ludvig Vieira, Leticia Pereira Scolari, Maria Gomes Silva, Debora Rayssa Siqueira Silva,
Braga, Rodrigo Bazan Fernanda Silva Ferreira, Alzira Nobuko Nishiyama, Ariel Lucas Medeiros Silva, Jose Jefferson Silva
Heloise Helena Siqueira Cavancanti Lins, Rita Cassia Oliveira Angela,
Hospital das Clínicas da Universidade Federal do Americo Danuzio Pereira Oliveira
Paraná. Curitiba PR, Brazil Hospital Geral Universitário. Cuiabá MT, Brazil
Hospital das clínicas da Universidade Federal do Hospital AMECOR. Cuiabá MT, Brazil Universidade de Pernambuco. Serra Talhada PE,
Paraná. Curitiba PR, Brazil Brazil
victorhhugo2005@gmail.com
Hospital Universitário Maria Aparecida Pedrossian.
Campo Grande MS, Brazil joab.serafim@upe.br
Background: Stroke is divided into two categories: isch-
Faculdade de Medicina de Botucatu – Botucatu –
emic (iCVA) and hemorrhagic (hCVA). The iCVA is caused Background: The occlusion of perforating arteries is
SP – Brazil
by the interruption of blood flow to a specific brain area, often the cause of a ischemic stroke and it appears as a
rebecaateixeira@hotmail.com having as main causes thrombosis, embolism and systemic syndrome called lacunar syndrome (LACS). That descrip-
hypoperfusion. The hCVA is the presence of intracranial tion is made based on Bamford classification and its
Background: Patent foramen ovale (PFO) is an inter- bleeding, due to parenchymal or subarachnoid hem- use can help handling a patient and in their prognostic
atrial embryonic remnant that results in right-to-left orrhage. Ischemic CVA is the most common, however, evaluation. Objective: To investigate possible relations
circulation (RLC) communication, therefore associated hemorrhage CVA is associated with a higher mortality between lacunar and non-lacunar syndromes of a isch-
with paradoxical embolism events. It is associated with rate. Both can cause complications and need for differ- emic stroke, the severity of neurological deficit, and the
cases of stroke of undetermined source in young people, ent approaches in the acute phase. Objectives: Describe early functional result in patients admitted in the emer-
although it is present in about 30% of the general popula- the clinical-epidemiological profile of patients treated gency room. Method: This is a descriptive, analytic and
tion and its incidental finding in complementary exams for acute stroke in a reference service in Cuiabá-MT. transversal study. The variables that were analyzed were
is common. The causal relationship has been explored in Methods: This is a descriptive observational study with obtained by the use of the National Institutes of Health
many studies, and the search for high-risk markers is the retrospective data collection from patients diagnosed Stroke Scale (NIHSS) instruments, the Bamford classifi-
subject of numerous meta-analyses. Contrast-enhanced with acute stroke. Results: In the year of 2021, 48 patients cation, the modified Rankin Scale (mRS) and the Barthel
transcranial Doppler (cTCD) is considered the screening presented suspected clinical features of acute stroke, and Index. Results: The sample had 103 patients, most of them
test of choice because it is a low-cost, non-invasive diag- the stroke protocol was open; 35% of these patients were being men (n = 57; 55.3%) between ages 51-80 (n = 69;
nostic tool with high sensitivity and specificity. Methods: aged between 61 and 80 years, 54% were male and 46% 67%). The LACS was seen in 26.2% (n = 27) of the patients
The database of the Neurosonology Laboratory of the were female. Of the 46 stroke protocols opened, 24% were and it showed a significant correlation (? = 0.46; p < 0.001)
Hospital de Clínicas of the Federal University of Paraná confirmed as iCVA and 8% as hCVA, and 56% were dis- with better functional results (mRS). There was a greater
was used, with a review of information from 901 patients carded cases. Stroke protocols were more opened in July sampling distribution in light incapacity levels (22.2%)
from January 2015 to May 2019. Of the total, 701 patients (16.6%) and August (16.6%). In relation to the time from and insignificant incapacity (29.6%) when compared to
who underwent cTCD were included and 551 who had a ictus until arrival at the referral service, 62% were admit- non-lacunar syndromes, which had a higher percentage
complementary investigation with transthoracic echocar- ted between 0 and 4.5 hours (thrombolysis window), 21% in moderate to severe incapacity level (29%) and severe
diography (TTE) or contrast transesophageal echocardi- between 4.5 and 12 hours, 12% between 12 and 24 hours incapacity level (29%). The analysis of the independence
ography (c-TEE). The functional pattern by the cTCD fol- and 5% after 24 hours. Of the cases confirmed as iCVA of a patient during their daily activies showed a significant
lowed criteria previously published in the Latin American that entered in the window for thrombolysis, 20% had correlation between the LACS and the Barthel Index (?
Consensus. Results: Our study showed a significant indication for thrombolytic therapy, being successfully = 0.42; p < 0.001). It was possible to highlight higher per-
difference in the “curtain” pattern at rest in the cerebro- performed in approximately 3 hours after the ictus. The centage numbers on total dependence (17.8%) and on
vascular disease group when compared to migraine with median door-to-imaging time was 1h and 23m, while the severe dependence (49.3%) in patients with non-lacunar
aura and incidental finding groups. In the evaluation of median door-to-needle time was 01h and 24m. The mean syndromes. No case of total dependence was found on
the subgroup that performed the cTCD and c-TEE, we Hospital stay time was 5 days. There was no registered patients with LACS. Among those with severe neurolog-
found a high sensitivity and specificity of the cTCD when deaths in patients with iCAV. Conclusion: Thrombolytic ical damage (NIHSS ? 16), 95% of them (n = 37) showed
compared with the gold standard exam. Conclusion: The therapy when properly indicated provides effective results non-lacunar syndromes. The analysis of the correlation
“curtain” rest pattern resulted in the greatest association and the analysis of this series demonstrates that adequate between LACS and NIHSS showed a significant number
of functional risk for the event related to cerebrovascular information is needed for greater agility in the care of the (? = -0.32; P < 0.001), so that the bigger the severity of the
disease and can be considered a high-risk characteristic. acute stroke phase in the reference unit. deficit the smaller was the distribution of patients with
Comparison of diagnostic tests for RLC resulted in a sen- that stroke subtype. Conclusion: Lacunar Syndrome has
sitivity of 98.6% and a specificity of 91.3%, with a PPV of a correlation with functional and independent results
96% and a NPV of 96.9%. Doença Cerebrovascular higher than non-lacunar syndrome does. Moreover, the
neurological damage in patients with LACS tends to be
lighter. That result reveals the importance of the right
Doença Cerebrovascular classification of the ischemic stroke as well as allows the
establishment of parameters in order to better guide a
patient’s rehabilitation.

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XXX Congresso Brasileiro de Neurologia

TL 1105388 TL 1105422 TL 1105435

THE RELATION BETWEEN THE SEVERITY DIAGNOSTIC CONSIDERATIONS IN CERVICAL AN EPIDEMIOLOGICAL ANALYSIS OF THE
OF THE NEUROLOGICAL DEFICIENCY AND AND INTRACRANIAL ARTERIAL DISSECTIONS ENDOVASCULAR VS MICROSURGERY
THE FUNCTIONAL DEPENDENCY DEGREE IN THROUGH A CASE SERIES STUDY TREATMENT OF INTRACRANIAL
PATIENTS DURING THE ACUTE PHASE OF A ANEURYSMS BETWEEN THE BRAZILIAN
Pedro Vinicius Brito Alves, Anderson Machado
STROKE Benassi, Victoria Veiga Ribeiro Gonçalves, Paula REGIONS
Joab Lins Serafim, Jose Jefferson Silva Cavalcanti Baleeiro Rodrigues Silva Luciano de Albuquerque Mota, David Augusto
Lins, Lílian Karine Machado Souza, Amanda Karla Hospital Sírio-Libanês Batista Sá Araújo, Geovanni Guimarães Bandeira,
Alves Gomes Silva, Debora Rayssa Siqueira Silva, Pedro Lucas Grangeiro Batista Sá Araújo, Lucas
Laiza Paula Candido Melo, Ariel Lucas Medeiros pvbalves@gmail.com Soares Radkte, Rodrigo Mariano Ribeiro, Arnaldo
Silva, Americo Danuzio Pereira Oliveira, Rita Cassia Ribeiro de Arruda, José Arnaldo Motta de Arruda
Oliveira Angelo Background: Cervical and intracranial vessels dissections
are often misdiagnosed given their symptoms hetero- Universidade Federal do Ceará. Fortaleza CE, Brazil
Universidade de Pernambuco. Serra Talhada PE, geneity. Headache, partial Horner Syndrome and focal Instituto Dr. José Frota. Fortaleza CE, Brazil
Brazil deficits are important clinical features. Objective: Report lucianodealbuquerquemota@hotmail.com
joab.serafim@upe.br epidemiological and clinical features of 6 patients diag-
nosed with arterial dissections in a neurology emergency Background: The advances of the technology for the
Background: The ischemic stroke is the second most department. Method: We have retrospectively studied endovascular treatment for intracranial has brought a
cause of people with some sort of physical impairment. six cases of cervical and intracranial arterial dissections change in the approach for this pathology. Therefore,
The severity of the initial neurological deficiency is a and extracted information regarding age, symptoms, vac- the choice for course of treatment in these lesions have
relevant predictor of a post-stroke functional prognosis. cine status, covid-19 infection and radiological aspects been a topic of recent discussions. Objective: Compare,
Objective: To analyse the correlation between the neu- of the dissections. Resultados: Medium age of onset was through a prevalence analysis, the endovascular and
rological deficiency and the functional dependency in 43 years-old. Five patients had headaches, in which two the microsurgical treatment of intracranial aneurysms
victims of a stroke during the periods of: admission to the cases were occipital/cervical pain, one orgasm-associ- among the brazilian regions in the last decade (2012-
Hospital, Hospital discharge and 3 months post Hospital ated headache, one headache plus transient expression 2021), whilst comparing cost and mortality rate (MR).
discharge. Method: Longitudinal study done in the XI aphasia and one new-onset bilateral frontal headache. Methods: Epidemiological, retrospective, descriptive
Gerência Regional de Saúde de Pernambuco between New headache pattern was the main red flag for further study, carried out with data obtained from the Hospital
September/2021 and April/2022. The instruments of rea- investigation. Unresponsiveness to usual analgesics led Information System (SIH/SUS) and from the Institute for
search were the National Institutes of Health Stroke Scale to the diagnosis in a patient with migraine. Direct trauma Applied Economic Research (IPEA). From these data, the
(NIHSS), which was used on admission (T0) and Hospital caused an extensive carotid bulb dissection complicated average dollar cost per procedure was calculated. We also
discharge (T1); and the Barthel Modified index, which with distal embolization and ipsilateral M1 occlusion compared both treatments for intracranial aneurysms
was used on Hospital discharge (T1) and 3 months after in a patient. The mean time to diagnosis was 4.8 days. over and under 15 mm. Results: In the period analyzed,
Hospital discharge (T2). Results: 43 patients participated, Diagnosis was achieved with angiotomography or mag- 32.010 intracranial aneurysms were treated. When treat-
most of them had a ischemic stroke (88%), men (53%), age netic resonance angiography. Anterior circulation was ing aneurysms >15mm the embolization technique was,
between 51-80 (63%) and blacks (72%). Medical record stricken in 2 cases, with a carotid bulb dissection related throughout the regions, the most utilized, had the lowest
of hypertension (86%), sedentary lifestyle (88%) and a to trauma and a bilateral carotid spontaneous dissection. MR but was also the most expensive. The biggest aver-
non-regulated diet (65%). The median of the NIHSS T0 Extracranial vertebral dissections were seen in 3 cases, plus age cost gap between approaches was in the northeast
was 13 [4-22.5]. It shows a moderate neurological defi- 1 case of intracranial V4 dissection. Four patients had had region (108%) and the smallest in the north with 59%.
ciency. The median of the NIHSS T1 was 6 [1-18], which respiratory viral infections or vaccine shots within 30 days The north region had the most difference in MR (8,49%)
shows a light neurological deficiency. There was a signif- of symptoms onset. Five patients were discharged with and the central-west region had the least (1,68%). The
icant statistic difference (p=0.001) between the scores of no disability. No deaths occurred. Discussion: Cervical choice of approach had its biggest contrast in the north-
the NIHSS T0 and T1. The evaluation of the functionality arterial dissections are present in up to 20% of strokes in east region with 76%, and smallest in the north region
showed a severe level of dependency with a median of young patients. Intimal damage or vasa vasorum rupture (11%). Regarding the course of treatment for intracranial
20 [10;5-43] on Bathel (T1) to the detriment of the sever- are proposed mechanisms. Hypertension, migraines, aneurysms <15 mm, the north region was the only one
ity of the neurological deficiency reduction. After three recent infections and direct trauma are established risk to have a higher MR in endovascular treatment, and to
months at home, there was a slight Barthel (T2) reduc- factors for arterial dissections, which usually occur after showcase a higher average cost with surgery but was also
tion to 17 [0-43.5], however, the severe dependency level carotid bifurcations. Conclusion: This case series high- the region with the highest percentage of this approach
didn’t change. We could also see a significant correlation lights important pitfalls in diagnosing arterial dissections: (89%). The north region had the smallest average cost
between the NIHSS scores (T0) and Barthel’s(T1) (? = -0.77; (1) unresponsive and new occipital or cervical headaches variance (6%), with the southeast having the biggest
p < 0.001); and between NIHSS (T0) and Barthel (T2) (? = prompt further investigation with contrasted images. (2) (74%), and had the biggest difference in course of treat-
-0.65; p < 0.001). Plus, there was a significant correlation Infections and vaccines may cause transient endothelial ment (78%) while the central-west region has the smallest
(? = 0.79; p < 0.001) between the scores of Barthel T1 and inflammation, weakening the arterial wall, with further (19%) both favoring the surgery route. The MR showed its
T2. Conclusion: More severe levels of neurological defi- risk of dissection. (3) Neurologic focal deficits arising from largest variation in the southeast region (6,62%) with its
ciency verified by the NIHSS during the acute phase of a both anterior and posterior circulations can be subtle and smallest in the central-west with 1,21%. Conclusion: We
stroke grant a worse expectation of a functional prognosis should be carefully searched in patients presenting with conclude that although its higher cost, the endovascular
in a three month period. Therefore, the relevance of ther- high-risk headaches. treatment for aneurysms >15 mm is already more utilized
apeutic interventions directed towards the reduction of in all the regions. While the approach for aneurysms <15
the severity of the neurological condition and the inten- mm still varies between the regions, with both the north
Doença Cerebrovascular
sification of Hospital rehabilitation should be reiterated. and central-west regions having the lowest percentage
for this treatment. Furthermore, we affirm that further
studies are needed to evaluate these regional differences
Doença Cerebrovascular in Brazil more deeply.

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TL 1105464 TL 1105498 TL 1105500

CEREBRAL AND CERVICAL ARTERY VALIDATION OF AN ARTIFICIAL INVESTIGATION OF PLASMA BIOMARKERS


DISSECTION: 6 YEARS CLINICAL REVIEW INTELLIGENCE IN THE PREDICTION OF IN CASES OF CAVERNOMA-RELATED
STROKE RECURRENCE: PRELIMINARY EPILEPSY
Thaís Takamura, Luiza Gonçalves Fraga, Camila
Carneiro Ferreira, Bruno Camporeze, Ludmila RESULTS
Vinicius Gabriel Coutinho Costa, Ane Caroline
Machado Lima, Vanessa Rizelio, Matheus Kahakura Rodrigo Mesquita de Vasconcelos, Luana Karoline Chales de Carvalho Pires, Jéssica Vasques Raposo,
Franco Pedro Castro Silva, Clarice Cristina Cunha de Souza, Renan Amphilophio Fernandes, Gustavo Fontoura
Dennise Lanna Barbosa Costa, Wagner Rodrigues Galvão, Fabrícia Fontes Lima, Jorge Paes Barreto
Instituto de Neurologia de Curitiba. Curitiba PR,
Galvão, Renata Viana Brígido de Moura Jucá, Ramon Marcondes de Souza, Soniza Vieira Alves-Leon
Brazil
Távora Viana, Lidiane Andrea Oliveira Lima, Carlos
Instituto de Ciências Biomédicas/UFRJ. Rio de
luizaag.fraga@gmail.com Mauricio Jaborandy de Mattos Dourado Júnior
Janeiro RJ, Brazil
Instituto Federal de Educação, Ciência e Tecnologia Instituto Biomédico/UNIRIO. Rio de Janeiro RJ,
Background: Arterial dissections are rare, despite being
do Ceará. Fortaleza CE, Brazil Brazil
a common cause of stroke in the young; they occur upon
Universidade Federal do Ceará. Fortaleza CE, Brazil Hospital Universitário Clementino Fraga Filho/UFRJ.
lesion of the arterial wall, allowing formation of intramu-
Rio de Janeiro RJ, Brazil
ral hematoma. Aneurysm or hematoma formation and lidianelima848@ufc.br Instituto de Biologia. Rio de Janeiro RJ, Brazil
vessel dilatation may causes local and result in stenosis
Faculdade de Medicina/UFRJ. Rio de Janeiro RJ,
or occlusion, which can lead to cerebral ischemia. The Background: Stroke remains the main cause of death Brazil
optimal treatment of dissection remains a challenge due in Ceará (Brazil). The high number of deaths in the state
to limitations of rapid definitive diagnosis, low incidence, reflects the large incidence of primary and recurrent vgcoutinho@outlook.com
low recurrence rate, and marked variation in patient char- strokes. With the advances in digital health, technologies
acteristics. Objective: Evaluate the profiles and outcomes such as Artificial Intelligence (AI) can help the develop- Cerebral cavernous malformations (CCM) are a type
of patients with cerebral or cervical artery dissections, ment of prediction studies. Machine Learning, an area vascular disease found in the central nervous system.
as well as the main symptoms and characteristics of the of AI, is defined as the ability of a machine to learn “by CCM are triggered by mutations in different genes, called
affected vessel, in patients treated in a brazilian tertiary itself” through database analysis and perform predictions, CCM1, CCM2 and CCM3, whose loss of function mod-
care Hospital. Methods: Retrospective, single-center such as the recurrence of a stroke in a timely manner with ifies the endothelial cytoskeleton and results in lesion.
observational study evaluating patients between January minimal error. Objective: To validate an artificial intelli- Although most cases are asymptomatic, the increased
2016 and December 2021. Data were collected from med- gence that helps predict the recurrence of stroke. Method: vascular permeability and fragility at the site can trig-
ical records; patient characteristics were analyzed (sex The database used for training the predictive algorithm ger cavernoma-related epilepsy (CRE). Currently, there
and age, initial symptoms, risk factors, history of trauma, consolidated the records of 571 individuals residing in are not clinically established or user-friendly biomark-
pre-vious comorbidities). Imaging methods data include Ceará. It was built from three databases, with 127 records ers to determine the likelihood of this complication.
location of affected vessel and treatment. Results: 87 acquired with the Neurofunctional Physiotherapy League Furthermore, most available studies have used symp-
patients were screened with intra or extracranial arterial of the Federal University of Ceará database; 66 records tomatic hemorrhages in cavernous angioma (CASH ) as
dissection, with female predominance of 60.9% and mean by a survey from the Fortaleza General Hospital; and 378 the only primary endpoint, with CRE being considered a
age of 40.9 years. 28 cases occurred in the internal carotid records, from a population database, by an internet form minor comorbity for CCM patients. In this context, from
artery, 17 on the left, with 53 in vertebral arteries, 22 on survey. The variables included in the model were: occur- a cohort of 128 patients with CCM, we tried to identify
the left; a higher incidence of cases in the V2 and V3 seg- rence of stroke, sex, age, marital status, hypertension, possible plasma biomarkers for CRE. The disease was
ments (19 and 26, respectively) was seen; 31 cases were heart disease, type of work, local of residence, diabetes classified as familial or sporadic according to the distri-
multisegmentar. The main symptoms were headache and mellitus, tobacco use, alcohol use, and depression/anxi- bution of lesions in SWI sequence, with familial being
neck pain, in 57 and 37 cases respectively; there were also ety. Results: These are preliminary results of an ongoing synonymous to the presentation of multiple lesions in this
clinical presentation with nausea (12 cases), dizziness (12), study. Of the 571, 237 people had a primary stroke and method. From this, the division between the groups was
stroke (19), Horner’s syndrome (9) and visual alterations 51 of those had a recurrence. The algorithm with the best made using clinical history and complementary exams.
(9). Only 36.7% of the cases had a history of trauma, 19.5% predictive performance was the Light Boosting Gradient CRE was defined with documented epileptic syndrome
were using hormonal therapy, 8% had some coagulopa- Model (LBGM) with the highest accuracy of 90.0% and arising in CCM topography. We selected and obtained
thy after etiological investigation. All cases were initially an AUC (Area Under the Curve) of 96.5%. The higher the plasma samples from 20 patients (8 asymptomatic and
investigated with AngioCT or AngioMRI and 55% under- accuracy and the AUC, the more positive model’s accu- 12 with CRE diagnosis). Vascular Endothelial Growth
went angiography to confirm the diagnosis. Patients were racy. Among the variables, those with high influence Fator (VEGF) and Thrombomodulin proteins, linked
treated with warfarin; 4 patients were thrombolysed and 3 scores on the stroke recurrence were: 1st stroke (80.1), to vasculogenesis and coagulation, respectively, were
underwent angioplasty. Conclusion: Arterial dissections heart disease (76.2), age > 50 years (48.5), Hypertension selected for analysis from a pre-designed panel of mol-
are an important cause of stroke in young patients and do (46.9), BMI > 24 (18.4), Diabetes mellitus (12.5), Alcohol ecules of interest. Both proteins were measured in our
not always evolve with a typical clinical manifestation, so use (8.5), Depression/Anxiety (5.18), urban residents samples by ELISA assays. Patients diagnosed with asymp-
they should be suspected with minor symptoms, such as (4.77), married (3.03), retired (2.81), male (0.61) and tomatic CCM had a mean level of 49.45 pg/mL for VEGF
headache and neck pain, in order to be treated early and smoking (0.26). Individuals after the first stroke frequently and 482.88 pg/mL for thrombomodulin, while patients
prevent stroke. Treatment with anticoagulation seems stopped smoking, this shows that the change in this habit diagnosed with CRE had 24.45pg/mL and 441.57 pg/mL
to have good efficacy, but it is still the subject of several makes it less explicative in the model. Conclusion: The AI for VEGF and thrombomodulin, respectively. A decrease
studies, given the advance in new therapies. algorithm is valid and has accuracy in stroke recurrence in VEGF was observed in patients with CRE when com-
prediction models, with LBGM, a modern machine learn- pared to asymptomatic patients, with statistical signifi-
ing algorithm, being the most accurate. According to the cance (P<0.05). Disruption of VEGF expression has been
Doença Cerebrovascular widely studied in CCM disease and models. Low levels
analysis, the first stroke makes the individual susceptible
to a second stroke, and the change in modifiable factors of this molecule are known to alter blood-brain barrier
may be related to a lower influence of recurrence of stroke. permeability and induce progression of CCM lesions.
Interestingly, decreased plasma VEGF has been described
as a predictor of disease severity. This pilot study opens
Doença Cerebrovascular a path for VEGF as potential target in future studies on
CCM biomarkers and justifies the understanding of CRE
as a distinguishable clinical entity, while larger samples
for a more meaningful analysis are still needed.

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TL 1105508 TL 1105537 TL 1105594

HOSPITALIZATION AND MAIN MODIFIABLE EPIDEMIOLOGY OF CEREBROVASCULAR REDE CUIDAVC APP: DEVELOPMENT OF
RISK FACTORS OF STROKE IN THE STATE OF ACCIDENT IN PATIENTS WITHOUT A MOBILE APPLICATION FOR STROKE
CEARÁ (BRAZIL): VIGITEL DATA COMORBIDITIES: A DESCRIPTIVE ANALYSIS PREVENTION
Luana Karoline Castro Silva, Dennise Lanna Sheila Wayszceyk, Leandro José Haas, Wesley Wagner Rodrigues Galvão, Ismael Lima Rocha,
Barbosa Costa, Cristian Douglas Dantas de Sousa, Severino, Guilherme Wandall, Wallace Mees, Clarice Cristina Cunha de Souza, Luana Karoline
Wagner Rodrigues Galvão, Renata Viana Brígido de Bernardo Przysiezny, João Pedro Domingos, Castro Silva, Francisco Hanlly da Silva, Renata
Moura Jucá, Ramon Távora Viana, Paula da Cruz Jacqueline Dulce Yumi Sanches, Pietra Cani Viana Brígido de Moura Jucá, Ramon Távora Viana,
Peniche, Christina Danielli Coelho de Morais Faria, Linzmeier, Gabriela Scheidt Christina Danielli Coelho de Morais Faria, George
Lidiane Andrea Oliveira Lima André Pereira Thé, Lidiane Andréa Oliveira Lima
Universidade Regional de Blumenau. Blumenau SC,
Universidade Federal do Ceará. Fortaleza CE, Brazil Brazil Master Program in Physiotherapy and Functioning,
Universidade Federal de Minas Gerais. Belo Federal University of Ceará. Fortaleza CE, Brazil
Horizonte MG, Brazil sheila.wayszceyk@yahoo.com.br Department of Electrical Engineering, Federal
University of Ceará. Fortaleza CE, Brazil
lidianelima848@ufc.br Cerebrovascular accident (CVA) is a sudden neurological Physiotherapy Department, Federal University of
deficit caused by ischemia or hemorrhage of the central Ceará. Fortaleza CE, Brazil
Background: Stroke is the leading cause of death in nervous system, with a predominance in females, aged Physical Therapy Department, Physical education,
Ceará (Brazil) as well has continued with high rates over 50 years, with the middle cerebral artery being the Physical therapy and occupational therapy school,
of Hospitalization. Since 90% of stroke cases can be most affected. Among the risk factors, the highest is Federal University of Minas Gerais. Belo Horizonte
avoided by preventing stroke risk factors, understanding systemic arterial hypertension. In addition, smoking, MG, Brazil
the health and risk profile of this population is necessary. accumulation of abdominal fat, a diet rich in fats and Department of Teleinformatics Engineering, Federal
Objectives: To describe the rate of Hospitalization of carbohydrates, dyslipidemia, diabetes, heart disease, and University of Ceará. Fortaleza CE, Brazil
stroke and its modifiable risk factors in the state of Ceará previous vascular events are included. Stroke patients
(Brazil) between the years 2020 and 2021. Methods: may present with some signs such as aphasia, dizziness, lidianelima848@ufc.br
The number of Hospitalizations for stroke in the state of headache with no known cause, decreased or loss of con-
Ceará to calculate its incidence rate was provided by the sciousness, nausea or vomiting, and motor deficits. The Background: The fast-growing stroke burden in the world
website of DATASUS-tabnet and the brazilian Institute aim of the study is to analyze the epidemiology of stroke suggests that current healthcare is failing to reduce modi-
of Geography and Statistics (IBGE). In addition, spe- in patients who did not have associated comorbidities. fiable risk factors in the population. Mobile apps became
cific data regarding the main modifiable risk factors for The study consists of a descriptive quantitative analysis of part of our everyday life may offer an opportunity to
stroke (Systemic Arterial Hypertension, Diabetes mellitus, patients treated at the Neurosurgery Service of Hospital improve healthy behaviors and potentially be useful for
alcohol consumption, smoking, physical inactivity and Santa Isabel, in Blumenau, Santa Catarina, between the stroke prevention. Objective: To describe the development
overweight) were obtained from the surveillance system periods of 2016 and 2020. From a group of 27 patients, 7 of the Rede CuidAVC App as new digital technology to
of risk and protective factors for chronic diseases by tele- patients were selected who did not have comorbidities improve long-term health behaviors and trace the stroke
phone survey (VIGITEL). Results: The incidence rate of associated with the vascular condition. Of the 7 patients survivors’ needs according to their residence location.
Hospitalization per 100,000 inhabitants was 76.12 in 2021, selected, the variables selected for frequency analysis Methods: The App development took four stages to be
a higher number than in 2020 (71.93). In the same period, were sex, age, symptoms, stroke characteristics, proce- concluded: 1. conceptual modeling; 2. navigation project;
there was an increase in the frequencies of risk factors for dure, and comorbidities (SAH, diabetes, dyslipidemia, 3. abstract design of the interface and 4. Implementation.
stroke: Systemic Arterial Hypertension [2020 (21.4%); 2021 smoking, previous stroke, or previous TIA). Of the patients The design was based on criteria for Primary Stroke/CVD
(22.8%)], Diabetes Mellitus [2020 (7.3%); 2021 (9.0%)], and analyzed, 71.4% were female and 28.6% were male, and of Prevention Digital Tools: Scientific evidence; Target popu-
regular alcohol consumption [2020 (17.6%); 2021 (18.3%)]. these 57.1% were between the 5th and 6th decade of life, lation and purpose of the tool; Scalability; Interface, usabil-
Active smoking was the only factor that decreased its fre- 14.3% in the 8th decade of life, 14.3% in the fourth decade ity, and privacy protection; Interactivity and Engagement.
quency, from 7.6% to a total of 7.2% of smokers. Despite of life, and 14.3% in the 2nd decade of life. Regarding the Results: The first stage of Conceptual modeling includes
this, levels of exercise and quality diet, protective factors for symptoms presented, 71.4% had some motor deficit, 57.1% what is recommended as evidence-based preventive rec-
stroke, showed lower values in 2021, with less than half of of the patients had headaches, 57.1% had aphasia, 57.1% ommendations; Target population and purpose of the tool
individuals following the recommended level of adequate had altered level of consciousness, 14.3% had dizziness with general information about stroke; and Interactivity
physical activity of 150 minutes per week [2020 (45.9 %); and 14 had 3% nausea. As for the characteristics of the and engagement with alerts and reminders with at least
2021 (35.3%)]. Approximately 65.7% of these individuals lesion, 85.6% of the patients had a stroke after endovas- one motivational technique. At this stage, the seven (7)
were totally sedentary. In addition, most of the population cular procedures and 14.3% of the patients had a sponta- main risk factors were defined according to the American
keeping overweight or obese [2020 (82.2%); 2021 (82.9%)]. neous stroke. 71.4% of patients had lesions in the middle Heart Association and included a self-reported question-
Conclusion: The high number of Hospitalizations for cerebral artery, 14.3% in the anterior cerebral artery, and naire approaching the main modifiable risk factors. Based
stroke, and the incidence of risk factors suggest the need 14.3% in the cavernous segment of the internal carotid on the user responses, recommendations for changes in
to implement effective actions to control stroke in Ceará. artery. Of these injuries, 71.4% occurred on the left side lifestyle habits and how to manage the risk factors will
The still elevated numbers of common modifiable risk and 28.6% on the right side. In 71.4% of the patients, the be sent to users. The App also complies with scalability
factors for stroke and the unwanted decline of protec- mechanism of approach was chemical fibrinolysis and by being designed to run on a computer, smartphones,
tive factors prompt the emergence of new public health in 28.6% it was mechanical thrombectomy. None of the and internet browsers. The second and third stages,
approaches to stroke prevention. patients had previous treatment or comorbidities. Thus, Navigation project and Abstract interface design, applies
it is clear that the patients, even without presenting risk to the Interface, usability, and privacy recommendation.
factors, met the epidemiological data that characterize In those stages, the concern was to provide a clean, sim-
Doença Cerebrovascular stroke in the literature. ple, and easy-to-use interface. For this, the applications
were divided into screens: Home; Address registration;
Cardiovascular Health profile and make an appointment
Doença Cerebrovascular (for Stroke individuals users with motor disorders) for an
evaluation with the neurological team of physiotherapists
of the Federal University of Ceará. The fourth stage is still
in progress with validation and implementation studies
to be carried out with health professionals and users.
Conclusion: The Rede CuidAVC App has the potential to
reduce modifiable risk factors and better manage access
to post-stroke rehabilitation in the state.

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XXX Congresso Brasileiro de Neurologia

TL 1105605 TL 1105630 TL 1105645

WHAT CAUSES THE INCREASED FREQUENCY PROFILE OF PATIENTS ADMITTED TO A DEATHS FROM STROKE IN THE STATE OF
OF ISCHEMIC CEREBROVASCULAR STROKE UNIT OF A TERTIARY HOSPITAL CEARÁ IN THE PANDEMIC (2020-2022) AND
ACCIDENT CASES IN PATIENTS INFECTED AND OUTCOMES AFTER A 3-MONTH POST- PRE-PANDEMIC (2017-2019) PERIOD: AN
WITH SARS-COV-2 VIRUS? A SYSTEMATIC DISCHARGE FOLLOW-UP ECOLOGICAL TIME SERIES STUDY
REVIEW
Nadson Bruno Serra Santos, Lucas Silva Scardua, Mariana Carmo Freitas, Anna Klara Bremer Moreira,
Marco Antonio Martins Barbosa Lucas de Melo Teixeira Branco, Gustavo Manginelli Lucas Frederico Silveira de Amorim, Thiago Barbosa
Lamas, Lenise Valler, Mariana Alemeida Vidal, Vivas
Universidade de Fortaleza. Fortaleza CE, Brazil Guilherme Menezes Mescolotte, Maycon Melo
Lopes, Alessandro Augusto Viana Oliveira Lopes, UNIME. Lauro de Freitas BA, Brazil
marco@edu.unifor.br
Wagner Mauad Avelar mariana.hfreitas@outlook.com
Background: The hypercoagulant characteristic of Covid- Universidade Estadual de Campinas. Campinas SP,
19 is a factor of high impact in the lives of affected patients. Brazil Background: The stroke is a neurological emergency
The syndrome hypercoagulation promotes cases of isch- in which there is an obstruction in the oxygenation to
emic stroke, which occurs when there is blockage or rup- nadson_bruno@hotmail.com the brain. It consists of an alteration in the blood flow
ture of a cerebral artery, even after the passage of high in the nervous tissue that can be caused by ischemia,
waves of contamination, however, specific studies of Background: Stroke is a major public health issue that when there is an obstruction of blood vessels, or hem-
the characteristic are still insufficient, making treatment leads to elevated mortality and morbidity. Specialized orrhage, when there is a rupture of the vessel. This inter-
and prevention of the problem inefficient. Objective: To Hospital care and standardized protocols are crucial in ruption in the blood flow causes death of nerve cells and
analyze the causes of the prevalence of ischemic stroke minimizing disability and intraHospital complications. damage functions in the body, according to the injured
cases in patients contaminated by the SARS-CoV-2 virus. Stroke Units (SU) provide the best multi-professional care, brain region. Objective: To analyze the variation in the
Method: The results established by incidence studies leading to reduced Hospital stay length and adverse out- number of deaths from stroke in the state of Ceará in the
published from the year 2017 to 2022 with the keywords: comes for these patients. Objective: To describe the profile pandemic period (2020-2022) and pre-pandemic period
“SARS-CoV-2”, “Covid-19”, “Ischemia” and “Stroke” in the of inpatients of a SU in a Tertiary Hospital and functional (2017-2019). Methods: This is an ecological time-series
MEDLINE and PUBMED databases were selected and outcome after a period of 3-month follow-up post-dis- study that analyzed the number of deaths from stroke in
analyzed for this systematic review, excluding publica- charge. Methods: This is a single-center, retrospective the state of Ceará from March 2020 to March 2022 and
tions that portrayed patients who were contaminated study of inpatients admitted to a SU in a Tertiary Center compared with the pre-pandemic period, January 2017 to
with the SARS-CoV-2 virus only after presenting ischemic during 2020, which received standardized multi-profes- January 2019. The data were collected through the Tabnet
conditions and publications that portrayed cases of isch- sional health care. Fifty-nine individuals with hemorrhagic Platform (DATASUS) and analyzed according to age group,
emia that were not classified as cerebral ischemia. Their or ischemic stroke were enrolled. The following data were sex, metropolitan region, year and month of occurrence
characteristics and results were analyzed to determine assessed: chemical thrombolysis with Alteplase (rtPA), and color/race. Results: In metropolitan regions, there
the probable reasons for the increase in cases of ischemic thrombolytic door-to-needle time (DNT), the prevalence was an increase in the incidence of cases, except for the
stroke in infected persons. Results: Studies have stated of major adverse outcomes due to rtPA, NIH Stroke Scale Cariri region, which had a decrease of 13.0%. The analy-
that cases of ischemic stroke in patients infected with (NIHSS) at Hospital admission, NIHSS at SU admission, sis according to the gender variable revealed an increase
the SARS-CoV-2 virus are more common in patients with NIHSS at discharge length of Hospital stay, the prevalence in the total number of cases, however, in the race/color
severe infections and in patients with preexisting cases of of adverse outcomes during the Hospital stay, functional variable, only mixed race expressed an increase in the
ischemic risks. Research also shows that cases of stroke status through modified Rankin Scale (mRS) at Hospital number of cases. When analyzing the age group, people
accompanied by Covid-19 are associated with cases of discharge and after 3 months of discharge, through a aged 10 to 29 years and 80 years or older had a lower
endotheliopathy, systemic inflammatory responses, and a remote assessment by telephone call. Results: Mean age mortality rate in the pandemic period, while those aged
high Von Willebrand Factor. Conclusion: The increase in was 65,7±18 years, and 52% were women. Six patients 30 to 79 years had a higher mortality rate. When analyz-
ischemic stroke cases is shown to be related to the hyper- (10,1%) were diagnosed with hemorrhagic stroke and ing the data by month, it was found that from January to
coagulant characteristic of the SARS-CoV-2 infection, a 53 (89,9%) with ischemic stroke, which included 6 indi- July was presented a drop in the number of cases, and the
characteristic influenced by points such as a high Von viduals with wake-up stroke. Among the ischemic stroke numbers from July to December continued to increase.
Willebrand Factor. Despite the presentation of a number group, 19 (35,8%) received rtPA, with a mean DNT of 47 Conclusion: The public health dynamics during the Covid-
of probable motivations, there is no study to guarantee +18 minutes, and none had hemorrhagic transformation 19 pandemic reflected on the prevalence and prognosis of
that new factors may not have a major influence on the or anaphylaxis. Median NIHSS was 7 at Hospital admis- various diaseases, as well as stroke. In the state of Ceará,
increase in cases. New studies are also needed to evalu- sion, 3 at SU admission, and 2 at discharge. Regarding there was a 5.9% increase in cases in the pandemic period.
ate if there is a predominance of cerebral ischemia or a adverse outcomes during the Hospital stay, 1 patient (1,6%) The proportionally greater increase found, 44.0% in brown
homogeneous distribution among other types of ischemia deceased, 8 (13,5%) had pneumonia, none had DVP or people in the color/race variable, may be related to the
due to the influence of the virus and its characteristics. pressure ulcers. The mean length of Hospital stay was 7,5. fact that these people are the majority of those who are in
Mean mRS at Hospital discharge was 02. After a 3-month situations of social vulnerability. The results of the study
period post-discharge, 44 patients were reassessed and 30 require further research to investigate the real impact
Doença Cerebrovascular (68,2%) individuals had mRS 0-2, 8 (18,2%) had mRS 3-5 of the pandemic on stroke mortality, since it may have
and 6 (13,6%) had mRS 6 by any cause. Discussion: SU and various influences.
standardized multi-professional health care might bene-
fit stroke patients and reduce the length of Hospital stay
and adverse outcomes. Our data regarding a brazilian SU Doença Cerebrovascular
stroke are in alignment with previous international data.

Doença Cerebrovascular

230
XXX Congresso Brasileiro de Neurologia

TL 1105655 TL 1105658 TL 1105672

CARDIAC MAGNETIC RESONANCE IMAGING OVERVIEW OF VASCULAR NEUROSURGERY INCREASED FOVEAL AVASCULAR ZONE IN
ABNORMALITIES ARE ASSOCIATED WITH PROCEDURES IN BRAZIL BEFORE AND CHAGAS DISEASE REINFORCES VASCULAR-
STROKE RECURRENCE IN PATIENTS WITH AFTER THE COVID-19 PANDEMIC: AN MEDIATED HYPOTHESIS: A CROSS-
EMBOLIC STROKE OF UNDETERMINED ECOLOGICAL TIME-SERIES STUDY SECTIONAL STUDY
SOURCE: A PROSPECTIVE COHORT STUDY
Maria Clara Oliveira Valente, Camilla Silva Dantas Pedro Fernandes Abbade, Débora Nalígia Moraes
Pedro Fernandes Abbade, Leila Souza Brito Santos, Mendes Lima, Thiago Barbosa Vivas, Mariana Carmo Luna, Paula Vieira Pereira, Isabella Reis Vieira,
Victor Luis Peixoto Pereira Botelho, Carita Victora Freitas, Lays Katharina Assis Coppieters, Lázaro Eric Aguiar Wittlich, Júlia Barreto De Farias, Lucy
Carvalho de Santana, Júlia Barreto Farias, Israela Freire Silva Filho, Anna Klara Bremer Moreira, Lucas Rodrigues-Ribeiro, André Barbosa Castelo Branco,
Souza Brito Santos, Jorge Andion Torreão, Pedro Frederico Silveira Amorim Roque Aras, Jamary Oliveira-Filho
Antônio Pereira de Jesus, Lidianne Ramos Neri,
UNIME. Lauro de Freitas BA, Brazil Universidade Federal da Bahia. Salvador BA, Brazil
Jamary Oliveira-Filho
UNINOVAFAPI. Teresina PI, Brazil OftalmoDiagnose. Salvador BA, Brazil
Universidade Federal da Bahia. Salvador BA, Brazil
mariana.hfreitas@outlook.com pfabbade@gmail.com
Hospital Santa Izabel. Salvador BA, Brazil

pfabbade@gmail.com Background: Vascular neurosurgery comprises a wide Background: Chagas Disease (CD) is associated with
range of procedures for the treatment of various health cerebral atrophy, silent cerebral microembolism, stroke,
Background: An unknown fraction of embolic strokes problems, which include brain arteriovenous malforma- and cognitive dysfunction in patients with heart failure
of undetermined source (ESUS) are thought to be cardi- tion, ischemic and hemorrhagic strokes, and cerebral (HF), all independently of HF severity. Two theories were
oembolic. Previously published baseline data from this aneurysms. The Covid-19 pandemic created a global health proposed to explain these associations: microvascular
cohort showed that cardiac magnetic resonance imaging crisis and had an overall impact on health care, delaying damage and/or neurodegeneration. The vascular and
(CMRI) abnormalities are present in over one-quarter of the scheduling of elective treatments and reducing the nerve fiber layers of the retina are assessable non-inva-
ESUS patients, especially in patients with coronary artery demand for emergency care. It is not known, however, sively using Optical Coherence Tomography (OCT) and
disease or Chagas disease. However, it is not known if if the pandemic has affected more complex procedures, Optical Coherence Tomography Angiography (OCT-A),
these CMRI abnormalities can affect the incidence of such as vascular neurosurgery. Objective: to analyze the which may be useful to understand if the pathophysiol-
stroke recurrence. Objectives: To identify the associa- frequency and variation of vascular neurosurgery proce- ogy of CD is vascular- or neurodegenerative-mediated.
tion between an abnormal CMRI and stroke recurrence. dures in Brazil, from 2012 to 2021. Methods: This is an Objective: To investigate the association between CD and
Methods: Prospective cohort study. Participants were ? 18 ecological time-series study that used secondary data abnormalities detected in OCT and OCT-A. Methods:
years old, with previous ESUS according to the Causative from the SUS’s Hospital Information System. Vascular Cross-sectional study from a prospective cohort of indi-
Classification of Stroke. CMRI was performed at baseline, neurosurgery procedures registered from 2012 to 2021 viduals aged ? 18 years, fulfilling Framingham criteria for
between 2012-2016. An abnormal CMRI was defined as in Brazil were analyzed. The study’s main outcome was HF. CD was defined by a positive ELISA or immunofluo-
the presence of wall fibrosis, wall edema, intracardiac the evolution of the surgery, and the data were analyzed rescence test performed in duplicate. Exclusion criteria
thrombus or left ventricle aneurysm. Follow-up occurred according to the region of residence. The mortality rate was were diabetes mellitus, pregnancy, glaucoma, previously
every three months, using the Questionnaire for Verifying calculated according to the total number of procedures diagnosed retinal diseases, or stroke. We performed OCT
Stroke-Free Status and neurologic evaluation with neuro- performed, as a percentage. The prevalence of events and OCT-A in patients between 2020-2022 and excluded
imaging to identify stroke recurrence. Survival analysis was was estimated according to occurrence per 1,000,000 tests with exam quality <50%. We conducted a univari-
performed for the primary outcome. Results: Follow-up inhabitants. Results: 22,053 vascular neurosurgeries ate analysis considering the mean of both eyes. Results:
was obtained from 30 (61%) out of 49 normal CMRI and 13 were performed in the period, of which 2,361 resulted We included 32 patients with CD and 20 without CD.
(77%) out of 17 abnormal CMRI patients. Baseline clinical in deaths. The mean (± SD) of procedures performed in Baseline clinical characteristics were similar for both
characteristics were similar for both groups except for a 2020 and 2021 (pandemic period) was 41% lower than groups except for a higher frequency of females (88% vs
higher frequency of Chagas disease among patients with between 2012 and 2019 (1,260 ± 170; 2,170 ± 904, respec- 50%) and higher ejection fraction (mean 65.5% ± 9.2 vs
abnormal CMRI (46% versus 17%). Mean age was 52 ± tively). The analysis of the year-on-year percentage change mean 41.0% ± 19.5) among patients with CD. In OCT, the
15 and 23 (53,3%) were female. No patient had coronary reveals that the largest changes were recorded between foveal thickness was lower in CD patients (mean 230 µm
artery disease. Mean follow-up time was 75 ± 21 months 2014 and 2015 (-26%) and 2020 and 2021 (-17%). The ± 18.1) compared to those without CD (mean 241 µm ±
for abnormal CMRI and 81 ± 24 months for normal CMRI. total number of procedures oscillated by -65% in the 24.7), but not statistically significant (p=0.13). No differ-
Stroke recurrence incidence was 39.8 (IC95% 8-116) per period. The mortality rate was around 9%, with the year ence was noticed in the thickness of retinal nerve fiber
1000 patient-years if CMRI was abnormal and 5 (IC95% 2021 having the highest mortality in the period (14%). or ganglion cell layers. Regarding OCT-A, the superficial
0-28) per 1000 patient-years if CMRI was normal (log- The South region had the highest mean (± SD) number plexus foveal vessel density was lower in CD patients
rank test: p=0.04). Regarding health status during fol- of surgeries/1,000,000 inhab. (13 ± 7) and the Northeast (mean 12.9% ± 5.10) compared to those without CD
low-up, anticoagulation was started in 5 (38%) patients region the smallest (8 ± 4). Conclusion: the number of (mean 16.6% ± 4.23), with p=0.02. The deep plexus foveal
with abnormal CMRI and 1 patient (3%) with normal vascular neurosurgeries procedures performed in Brazil vessel density in CD (mean 26.7% ± 7.10) was lower than
CMRI (p=0.03). We noticed no difference in emergency decreased from 2012 to 2021 and has shown a sharp drop in patients without CD (mean 30.8% ± 6.21) but not with
department visits or atrial fibrillation diagnosis during in the years 2015 and 2021. The comparison between the statistical significance (p=0.05). There was no difference
follow-up. Conclusion: An abnormal CMRI is associated period before and after the Covid-19 pandemic revealed in vessel density considering the whole image, perifoveal
with higher stroke recurrence in ESUS patients, suggesting that, in the latter, the average of procedures was lesser. or papillary regions. Conclusion: Chagas disease was
that this method may be useful to further stratify stroke The mortality rate was higher in 2021. More studies should associated with decreased foveal vascular density, but
risk in patients with ESUS. be carried out to confirm the hypothesis that the Covid- no changes in nerve fiber or ganglion cell layers of the
19 pandemic changed the care dynamics in the vascular retina. This finding is common to microvascular retinop-
neurosurgery scenario and influenced the prognosis of athies, suggesting that CD may damage microcirculation
Doença Cerebrovascular the procedures performed. beyond neuroimaging-detected damage with tests such
as magnetic resonance imaging.

Doença Cerebrovascular
Doença Cerebrovascular

231
XXX Congresso Brasileiro de Neurologia

TL 1105684 TL 1105725 TL 1105852

HOSPITAL ADMISSIONS FOR STROKE AND ASSESSMENT OF HOSPITALIZATIONS FOR ANALYSIS OF DOOR-TO-CT AND DOOR-
STROKE IN ADULTS BEFORE AND DURING TIA IN THE UNIFIED HEALTH SYSTEM FROM TO-NEEDLE TIME IN PATIENTS WITH
THE COVID-19 PANDEMIC. AN ANALYSIS OF 2017 TO 2022 SUSPECTED STROKE AT THE CLINICAL
DATASUS HOSPITAL OF THE FEDERAL UNIVERSISTY
Aline Rabelo Rodrigues, Louise Bruns Willrich,
Bernardo Caetano Novaes, Iago Alvino Cordeiro, OF MINAS GERAIS, FROM 2018 TO 2021
Aline Rabelo Rodrigues, Daniella Rocha Soares,
Gustavo Sales França, Ana Maria dos Santos Luana Frizzo, Ana Maria dos Santos Oliveira, Davi Teixeira Urzêdo Queiroz, Ianka Cristina Ernesto,
Oliveira, Luana Frizzo, Iago Alvino Cordeiro, Bernardo Gustavo Sales França, Daniella Rocha Soares, Henrique Amancio Ferreira, João Victor Lage
Caetano Novaes, Louise Bruns Willrich, Marcos Marcos Christiano Lange Guerra, Bianca Gomes Mazzoni, André Filipe Lucchi
Christiano Lange Rodrigues, Sarah Teixeira Camargos, Breno Franco
Universidade Federal de Jataí. Jataí GO, Brazil
Universidade Regional de Blumenau. Blumenau SC, Silveira Fernandes, Rodrigo Santiago Gomez, Elisa
Universidade Federal de Jataí. Jataí GO, Brazil
Brazil de Paula França Resende
Universidade do Estado de Mato Grosso – CUIABÁ
– MT – Brazil Centro Universitário de Volta Redonda – Volta Hospital das Clínicas da Universidade Federal de
Centro Universitário de Pato Branco – Pato Branco Redonda – RJ – Brazil Minas Gerais. Belo Horizonte MG, Brazil
– PR – Brazil Universidade do Estado de Mato Grosso – CUIABÁ
Centro Universitário de Volta Redonda – Volta – MT – Brazil davituq@gmail.com
Redonda – RJ – Brazil Centro Universitário de Pato Branco – Pato Branco
Universidade Regional de Blumenau. Blumenau SC, – PR – Brazil Background: Stroke is one of the most important causes
Brazil CHC, UFPR/EBSERH. Curitiba PR, Brazil of morbimortality around the world. Trombolysis can
CHC, UFPR/EBSERH. Curitiba PR, Brazil improve patients outcomes, but time is crucial: When the
aline_rabelo@discente.ufj.edu.br patient gets to the emergency room, a brain computed
aline_rabelo@discente.ufj.edu.br tomography (CT) must be performed in 25 minutes and
Background: Transient ischemic attack (TIA) is an import- the intravenous (IV) trombolysis (first-line therapy) must
Background: Over the course of the COVID-19 pan- ant risk factor for ischemic stroke. Objective: To deter- be iniciated no more than 4.5 hours after the symptons
demic, numerous evidences have demonstrated a close mine the frequency of Hospitalizations for TIA in the onset and within one hour (60min) after admission.
relationship between severe acute respiratory syndrome 2 different federative regions of Brazil in a period of 05 Objective: To evaluate the latency to perform brain CT
(SARS-CoV-2) and the development of serious neurolog- years. Methods: Through the database of the Ministry of (door-to-CT time, DTC) and to iniciate IV trombolysis
ical manifestations, such as stroke. Given that Brazil was Health, the Hospital Information System of the SUS (SIH/ (door-to-needle time, DTN) in patients with suspected
one of the countries most affected by the pandemic, an SUS), the records of transient ischemic strokes (ICD-10 stroke at the Clinical Hospital of the Federal University
analysis of the variation in the number of Hospitalizations G45) of patients Hospitalized between January 2017 and of Minas Gerais, and to identify possibles flaws in the
for stroke before and during the pandemic period is January were selected. 2022, according to SUS Hospital management of these patients. Methods: We analyzed
essential. Objective: To carry out an investigative analysis Morbidity and place of residence in Brazil. Being strat- medical records of patients admitted with suspected
of changes in the epidemiological profile regarding the ified in the federative regions: North, Northeast, South, stroke, from 2018 to 2021. We also invited professionals
number of Hospitalizations for stroke in Brazil during the Southeast and Midwest. The analysis was performed per involved in stroke care (neurologists and nurses, both
pandemic in view of the pre-pandemic period. Methods: 100,0000 inhabitants. Results: 10,1447 cases were iden- in the emergency room and in the intensive care unit –
Epidemiological, retrospective, cross-sectional and quan- tified, 38.2% in the Southeast region, 26.7% in the South ICU) to fill forms containing questions about the initial
titative study, which collected data from the Department region, 20.8% in the Northeast region, 8.6% in the North management of stroke. Results: A total of 947 patients
of Informatics of the Unified Health System (DATASUS) region and 5.4% in the Midwest region. When analyzed per were selected. 63 were submitted to IV tromboloysis. The
regarding the occurrence of Hospitalizations for stroke 100,000 inhabitants, the southern region had the highest average DTC time and DTN time were 22min and 81min,
in Brazil in adults aged between 20 and 39 years, in a prevalence, 0.089/100,000 inhabitants, followed by the 17min and 63min, 12min and 56min, 19min and 62min
period of 24 months before the SARS-CoV-2 pandemic North region (0.046/100,000 inhabitants), the Southeast in 2018, 2019, 2020 e 2021, respectively. In addition, 30
compared to 24 months after its onset. The study included region (0.043/100,000 inhabitants), the Northeast region professionals answered the questions, among these, 12
Hospitalizations for strokes not specified as ischemic (0.037/100,000 inhabitants) and the Midwest region. neurologists and ICU physicians, 16 nurses and 2 others
(istroke) or hemorrhagic (hstroke) (ICD-10 I64), transient (0.033/100,000 inhabitants). Conclusion: according to professionals (clincal pharmacy and radiology techni-
ischemic strokes (ICD-10 G45) and cerebrovascular syn- the analysis of DATASUS, the rates of Hospitalization cian). 20% answered correctly about maximum DTC
dromes (ICD-10 G46). variables studied were: number of for TIA in the SUS occur with higher prevalence in the time, 46,7% about maximum DTN time and 70% about
Hospitalizations, age group, sex and color/race. Statistical southern region compared to other regions of the country. maximum time after symptoms onset to iniciate IV trom-
analysis was performed using the Excel program, using the bolysis. Also, 17 professionals have reported problems
Collective Health measures present in descriptive statis- with high demand in the emergency room and 22 with
tics. Results: 18,733 Hospitalizations were observed during Doença Cerebrovascular
team comunication. Conclusion: Despite achieving the
the pre-pandemic period and 17,394 during the pandemic goal of performing a CT within 25min or less, the time to
period. In the male population, there was a variation from iniciate the IV trombolysis after patient admission had
8,367 to 7,956 and in the female population, from 10,366 to not been reached. Furthermore, the percentages of cor-
9,438. In adults aged 20 to 29 years, the variation was from rect answers indicate the need of professionals education
5,508 to 5,111 and in the age group from 30 to 39 years, it update and to optimize the Hospital protocols in order
was from 13,225 to 12,283 The only variable that showed to minimize complaints about team comunication and
an increase was in the indigenous population, with an professionals overload.
increase of 5.55 %, however the number of reported cases
was small, from 18 cases before the pandemic to 19 cases
during the pandemic period. Conclusion: In general, as Doença Cerebrovascular
in the group analyses, there were no changes regarding
the number of Hospitalizations comparing the pre-pan-
demic period with the first two years of the pandemic by
SARS-COV-2 in SUS through DATASUS.

Doença Cerebrovascular

232
XXX Congresso Brasileiro de Neurologia

TL 1105875 TL 1106028 TL 1106040

RECANALIZATION THERAPIES IN ACUTE NEURORADIOLOGICAL MARKERS OF SHORT-TERM EFFICACY OUTCOMES OF


ISCHEMIC STROKE: THE EXPERIENCE OF A VASCULAR COGNITIVE IMPAIRMENT AFTER TENECTEPLASE VERSUS ALTEPLASE FOR
BRAZILIAN UNIVERSITY HOSPITAL STROKE ACUTE ISCHEMIC STROKE: META-ANALYSIS
OF 5 RANDOMIZED TRIALS
Kamila Santos Ferreira, Millene Rodrigues Camilo, Letícia Escorse Requião, Murilo Santos de Souza
Pedro Telles Cougo-Pinto, Francisco Antunes Dias, Letícia Escorse Requião, Roberto Santos de Oliveira
Rui Kleber Martins Filho, Luiz Henrique Castro- Escola Bahiana de Medicina e Saúde Pública.
Júnior, Lorena Silva dos Reis, Ana Flávia Barbosa de
Afonso, Daniel Giansante Abud, Octavio Marques Salvador BA, Brazil
Castro, Beatriz do Nascimento Garcia Moreno, Luisa
Pontes Neto Hospital Cardiopulmonar. Salvador BA, Brazil
Rodrigues Cordeiro, Davi Jorge Fontoura Solla
Hospital das Clínicas da Faculdade de Medicina de leticiarequiao17.2@Bahiana.edu.br
Escola Bahiana de Medicina e Saúde Pública.
Ribeirão Preto. Ribeirão Preto SP, Brazil Salvador BA, Brazil
Faculdade de Medicina de Ribeirão Preto (USP). Background: Cognitive vascular impairment (CCV) is a
Escola Bahiana de Meicina e Saúde Pública.
Ribeirão Preto SP, Brazil frequent, but overlooked, possible consequence of stroke.
Salvador BA, Brazil
The most relevant markers in Magnetic Resonance Imaging
Hospital das Clínicas da Universidade de São Paulo.
ksferreira@hcrp.usp.br (MRI) seem to be, among others, strategic location, sever-
São Paulo SP, Brazil
ity of white matter changes, as well as the degree of atro-
Background: Stroke is the second leading cause of death phy of the medial temporal lobe. Objective: To assess leticiarequiao17.2@Bahiana.edu.br
and disability in the world. Ischemic stroke (IS) corre- the relationship between stroke and CCV using markers
sponds to 85% of cases and its treatment reduces sequelae from MRI. Methods: Systematic review of observational Background: Tenecteplase (TNK) has been shown to be
and mortality. However, recanalization therapies for stroke studies published between 2005 and 2020. The search was non-inferior to Alteplase (ALT) for long-term efficacy and
are available only to a small portion of the population carried out in the PubMed and SciELO databases with the safety outcomes. Whether this also applies to short-term
and there are few data in the brazilian literature on this keywords “stroke”, “MRI”, “Vascular cognitive impairment”. efficacy outcomes such as early clinical improvement and
topic. Objectives: To evaluate the clinical characteristics The PRISMA check-list was used to guide this review. recanalization is unknown. Objective: Compare TNK
of patients undergoing recanalization therapy treated at Results: 8 studies were selected. “Event location” was the and ALT regarding the short-term efficacy outcomes
a public University Hospital in Ribeirão Preto; to identify marker in MRI of the skull most frequently considered (7 early neurological improvement and recanalization.
the predictors of in-Hospital mortality and symptomatic studies). It proved to be a statistically significant marker Methods: The Preferred Reporting Items for Systematic
hemorrhagic transformation (HTs) and good functional (p <0.05) for the prediction of CCV in 6 out 7 studies. Reviews and Meta-Analyses PRISMA was used to con-
outcome using the Modified Rankin Scale (mRs) 0-2 at 75% of the studies included in this review evaluated the duct a meta-analysis, adapted to noninferiority analysis.
90 days after therapy was instituted. Methods: A review relationship between the presence of “hyperintensity in The primary outcome was early (24-72 hours) neurolog-
of the medical records of patients with acute stroke who the white matter” at MRI and CCV. However, this marker ical improvement, defined as either Nation Institutes of
underwent endovenous thrombolysis and/or endovas- was shown to be statistically significant in 50% of these Health Stroke Scale (NIHSS) score 0 or reduction of at
cular treatment between the years 2001 to 2017 was per- studies. Conclusion: A review that brought together the least 8 points compared to baseline. Recanalization was
formed. Results: There were 869 patients treated, most assessment of a wide range of possible neuroradiological a secondary outcome. The noninferiority margin was set
of them male (57%), mean age of 66.5±13.1 years, median predictors of CVD after stroke had not been carried out at 6.5%. Results: Search strategy yielded 5 randomized
NIHSS 15 and 65.7% with proximal occlusion. The rate of so far. Yet it would be particularly useful to evaluate the clinical trials (1585 patients: 828 TNK, 757 ALT). Across all
intravenous thrombolysis was 87.5% and endovascular markers in a more homogeneous way in a study with a trials, mean age was 70.8, 58.8% were men, mean baseline
treatment was 28.7%. Mean admission-needle time was larger sample size. NIHSS was 7 and mean onset-to-treatment time was 148
57 minutes [35-70] and ictus-needle time was 204 min- minutes. Patients in intervention group received TNK at
utes [150-248]. HTs occurred in 5.9% of patients, in-Hos- doses of 0.1mg/kg (6.8%), 0.25mg/kg (24.6%), and 0.4mg/
pital death in 16.8% and the median mRs after 90 days Doença Cerebrovascular kg (68.6%), while all ALT patients received 0.9mg/kg. In
was 3. The predictors of good functional outcome (0-2) random-effects meta-analysis, TNK was non-inferior to
were age and NIHSS; for Hospital mortality, in addition ALT for the primary outcome early major neurological
to these, diabetes mellitus, proximal occlusion and HTs; improvement (risk difference 8% in favor of TNK, 95% CI
for HTs, NIHSS, proximal occlusion and blood glucose. 1-15%). Recanalization was also non-inferior for the TNK
Conclusion: The profile of patients with acute ischemic compared to the ALT group (risk difference 9% in favor of
stroke is predominantly male, in the sixth decade of life, TNK, 95% CI -6% to 23%). Fixed-effects models yielded
with several comorbidities, admitted within 2.5 hours of similarly non-inferior results and signaled for a possible
symptom onset, high blood pressure and blood glucose TNK superiority for both early neurological improvement
levels, in addition to severe and severe symptoms. high and recanalization. Conclusion: TNK is non-inferior to
rate of proximal occlusion. The HT rate was similar to ALT at the short-term efficacy outcomes early neurolog-
those reported in international and national studies. The ical improvement and recanalization.
shorter treatment time had an impact on the functional
outcome, supported by previous evidence. The present
study presents the largest series of patients treated with Doença Cerebrovascular
thrombolysis and/or thrombectomy in Brazil, through-
out a historical serie, showing the safety of recanalization
therapies and less chance of sequelae, with 40% of patients
having a good functional outcome.

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XXX Congresso Brasileiro de Neurologia

TL 1106065 TL 1106141 TL 1106156

PROFILE OF PATIENTS HOSPITALIZED CEREBRAL VENOUS THROMBOSIS AN UPDATED NON SYSTEMATIC REVIEW ON
FOR VENOUS THROMBOSIS CEREBRAL IN SECONDARY TO THE MUTATION IN THE CEREBRAL AMYLOID ANGIOPATHY (CAA)
NORTHEASTERN BRAZIL PROTHROMBIN GENE IN THE CONTEXT OF
Ana Júlia Trierweiler Vieira, Breno Rampeloti, Luiza
VACCINATION AGAINST COVID-19: CASE Ferreira Gomes da Silva, Giulia Murillo Wollmann,
Ana Karoline da Costa Monteiro, Paulo Filho
Soares Marcelino, Tatheane Couto de Vasconcelos, PRESENTATION Carolina Haveroth Lara, Giuliana Moro, Gustavo
Marx Lima de Barros Araújo, Ana Karine da Costa Manhaguanha, Mateus Andres Colussi, Vinicius Biff,
Eduardo Mariano Carvalho Silva, Jhonata Gabriel
Monteiro Marcus Vinicius Magno Gonçalves
Moura Silva, Arthur Costa Junger, Fábio Pereira
Universidade Federal do Piauí. Teresina PI, Brazil da Silva Júnior, João Victor da Cunha Silva, Lays Universidade da Região de Joinville. Joinville SC,
Universidade Federal do Ceará. Fortaleza CE, Brazil Saraiva Rodrigues Carvalho, Lorena da Silva Viana, Brazil
Luiz Felipe Bezerra de Sousa, Nínivi Daniely Farias
karolmonteiro2006@hotmail.com Santos, André Camacho Oliveira Araújo giuliamw@gmail.com

Background: Cerebral venous thrombosis (CVT) is a Universidade Federal do Maranhão. Imperatriz MA, Background: Cerebral amyloid angiopathy (CAA) is
rare disorder and presents a high risk of morbidity and Brazil described as the accumulation of amyloid fibrils on the
mortality. The signs and symptoms can be diverse, which eduardo.neurologista@gmail.com walls of blood vessels of the parenchyma of the central
makes this condition a diagnostic challenge. Objective: nervous system (CNS) and leptomeninges. Due to abnor-
to characterize the clinical and epidemiological profile of mal production or impaired clearance of the amyloid beta
Case Presentation: A 15-year-old woman came to the
patients diagnosed with cerebral venous thrombosis at a protein, structural changes in the vessels are lead, causing
consultation with a progressive headache for 6 weeks, after
University Hospital in Teresina. Methods: This is a retro- ischemic lesions and intracerebral hemorrhages (ICH)
30 days of vaccination against the coronavirus with the
spective, cross-sectional and descriptive study, carried out owing to small vessels fragility. Objectives: To describe
Ad26.COV2 vaccine. S. She Denied use of contraceptives.
through the evaluation of electronic medical records of the pathophysiology, criteria diagnosis and progression
She evolved with drowsiness, neck stiffness, abducens
patients admitted to the Neurology Service of a University of CAA. Methods: Non systematic review in the Pubmed,
nerve paresis, low visual acuity (BAV), vomiting, ataxia
Hospital in Teresina/PI, from May to July 2021. Results: Lilacs and Scielo databases, selecting articles in english,
and mild asymmetric tetraparesis. Optical Coherence
The sample consisted of 13 medical records. Most CVT published from 2020 to 2022. Results: The pathophysio-
Tomography (OCT) showed bilateral papilledema. Brain
patients were female (69.2%), with a mean age of 39.2 years. logical progression of CAA remains uncertain, but has a
Magnetic Resonance (MRI) and CSF examination without
Headache (92%) was the most common clinical manifes- strong contribution of a genetic component (APOE 2 and
alterations. Spinal manometry confirmed Intracranial
tation. The use of oral contraceptives was the risk factor 4), related to changes in the astrocitarian reactivity and
Hypertension (ICH) (75cmH2O). A relief lumbar punc-
most associated with CVT, with 46%. The mean length of deficit of solubility factors. In addition, the impairment
ture was performed and acetazolamide was started.
stay observed was 18.1 days. Conclusion: Despite being of perivascular spaces on the blood-brain barrier (BBB)
Arteriography showed Venous Thrombosis of the sigmoid
a potentially serious disease, no death was observed in or lymphatic system, accumulation of amyloid beta pro-
sinus and left transverse. The investigation of alteration of
the study population, which may be linked to adequate teins, in the integrity of BBB by the loss of proteins on tight
factor V Leiden, antithrombin III, Protein C and S, anti-
diagnosis and timely treatment, capable of reflecting on joints, leads to inflammatory effects by oxidative stress,
phospholipid antibodies were normal. A positive result
the successful outcome of patients with CVT. which alters the permeability of the BBB and promotes
was obtained for the mutation of the prothrombin gene
toxicity and changes in vessel structure. Currently, the
20210A in heterozygosity. Full anticoagulation was per-
management of CAA remains focused in the prevention
formed with 5 mg warfarin (INR between 2 and 3) and
Doença Cerebrovascular of recurrent ICH episodes, and the protocols for diag-
bridging with enoxaparin, associated with topiramate 25
nosis still lack padronization – there is no consensus in
mg/day. The patient remains under outpatient follow-up,
the meanings of CAA’s neuropathology. Also, a defin-
with no signs of disease recurrence. Discussion: Cerebral
itive diagnosis can only be confirmed via postmortem
venous thrombosis (CVT) is defined as the presence of a
examination of the brain. Thus, to identify possible CAA
thrombus within a venous sinus, superficial intracranial
patients, the Boston criteria was developed as a combi-
vein or deep intracranial vein. CVT has a higher incidence
nation of clinical, pathological, and radiographic criteria
in young women, with additional risk factors, such as
that uses MRI findings, classifying the results in “possible”
pregnancy, puerperium and use of oral contraceptives².
and “likely”. According this criteria, the analyzed aspects
Headache is the most common symptom, but focal neu-
of the MRI are strictly lobar cerebral microhemorrhages
rologic deficits, seizure episodes, and encephalopathies
(small cerebral hemorrhages restricted to the cortical and
are also evidenced. Digital subtraction angiography is
subcortical regions of the brain), and superficial corti-
indicated for inconclusive cases. The coagulogram may
cal siderosis (deposition of blood degradation products
indicate the presence of conditions that aid in the patho-
in the cortical sulci over the convexity of the cerebral
genesis of CVT and the search for thrombophilic status
hemispheres). The use of this criteria, in synthesis, can
and mutations is useful. Lumbar puncture is important
improve the chances of obtaining a diagnosis during the
to rule out meningitis³. In addition, other conditions such
patient’s lifetime. Conclusion: The poor understanding
as previous vaccination for COVID-19 should be investi-
of the disease mechanisms reflects the lack of therapies
gated, as vaccines based on adenovirus vectors are related
able to limit the disease progression. The combination
to thrombotic episodes, through a mechanism called
between experimental results and clinical data from large
Vaccine-Induced Thrombocytopenic Thrombosis (VITT).
observational studies are mandatory to its understanding.
Conclusion: It is noteworthy that CVT is an uncommon
and reversible condition if properly diagnosed and treated.
Its association with mutations in the prothrombin gene Doença Cerebrovascular
20210A is well established and in relation to vaccination it
is important to recognize that the risk of CVT by COVID-19
in the general population outweighs the risk of vaccines.

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XXX Congresso Brasileiro de Neurologia

TL 1106208 TL 1106215 TL 1106220

CHAGAS’ DISEASE AND ISCHEMIC STROKE: MORTALITY AND FUNCTIONAL STATUS PALLIATIVE CARE IN PATIENTS
A STUDY OF ASSOCIATED FACTORS FIVE YEARS AFTER SURVIVING A STROKE: HOSPITALIZED FOR STROKE: AN 8-MONTH
A PROSPECTIVE COHORT STUDY OF 1.005 TIME FRAME DURING THE SARSCOV-2
Eduardo Sousa de Melo, Arthur Cesário de Holanda,
Wilson Alves de Oliveira Júnior, Luciana Patrizia PATIENTS IN FORTALEZA, NORTHEAST, PANDEMIC
Alves de Andrade-Valença BRAZIL
Luciana Oliveira Neves, Clarissa Rocha Montenegro,
Hospital das Clínicas da Universidade Federal de João José Freitas de Carvalho, Amanda Souza Igor Bessa Santiago, João Renato Figueiredo Souza,
Pernambuco. Recife PE, Brazil Moreira, Carolina Murad Regadas, Marina Murad Benevides José Silva Santos, Sthefany Lorrany
Casa de Chagas do Pronto-Socorro Cardiológico Regadas, Rebeca Holanda Nunes Nepomuceno Santos, Rafaela Silva Félix, Karla
Universitário de Pernambuco PROCAPE Maria Carneiro Rolim
Unichristus School of Medicine. Fortaleza CE, Brazil
arthur.c.holanda@gmail.com Hospital São Carlos. Fortaleza CE, Brazil
jjcarvalho@gmail.com Universidade de Fortaleza. Fortaleza CE, Brazil
Background: Chagas’ disease is an independent risk fac- dra.luciananeves@gmail.com
Background: Stroke is among the leading causes of death
tor for stroke. The management and prevention of these
and the second most common cause for disability-adjusted
cases are based on incomplete information or extrapo- Background: Since March 2020, Brazil has been expe-
life years globally. Over the past decades, mortality related
lations of knowledge about other stroke etiologies. The riencing the waves of the pandemic caused by the
to stroke has decreased globally. The same, however, is not
occurrence of strokes without cardiac involvement in SARSCOV-2 virus. Stroke is an important cause of mor-
observed regarding impacts on personal, family, social
Chagas’ disease has also been documented. Objective: tality and morbidity in the country and in the world,
and productive life aspects due to the ensuing disabilities,
The present study aims to verify the risk factors (clinical and therefore it is imperative to consider Palliative Care
which remain high. Knowing the survival and disability
and laboratory) for the occurrence of stroke in patients in cerebrovascular diseases. During the pandemic, it
rates after a stroke helps evaluate the performance of the
with Chagas’ disease. Methods: This is a cross-sectional was reported that patients were afraid to look for emer-
line of care from primary prevention to pre-Hospital and
study that included patients seen at the Chagas Disease gencies with symptoms of stroke for fear of contracting
Hospital care in the acute setting to rehabilitation and
outpatient clinic in the Cardiovascular Emergency Room COVID-19. Objective: To describe Hospitalizations for
secondary prevention. In addition, it permits having a
of Pernambuco and the Neurovascular Outpatient Clinic stroke from October 2021 to May 2022; describe the cases
better understanding of the evolution of the disease and
at Hospital São Paulo – Federal University of São Paulo. of COVID-19 in these patients during Hospitalization;
the effects of therapeutic interventions. Objective: To
Demographic, clinical and complementary cardiological describe the outcome of these Hospitalized patients;
study the mortality and functional status five years after
exams (echocardiogram, electrocardiography, Holter- describe epidemiological variables of patients (age and
surviving a stroke in Fortaleza, Northeast Brazil. Methods:
electrocardiography), in addition to those related to the sex). Method: Cross-sectional, observational, quantita-
This prospective cohort study was carried out in Fortaleza
investigation of stroke and its mechanisms were analyzed. tive study in patients admitted through the emergency
to analyze patients’ survival and functional state five years
It was defined as significant p <0.05. The local research department of a private Hospital from October 2021 to
after a stroke. A translated and culturally adapted brazilian
ethics committee of each participating center approved May 2022. Inclusion criteria were patients Hospitalized
Portuguese version of the Modified Rankin Scale (mRS)
this study. Results: The sample consisted of 678 patients, for ischemic, hemorrhagic stroke and Cerebral Venous
validated to be used over the telephone was adopted to
72 (10.6%) had a confirmed stroke. The mean age of the Thrombosis (CVT). Patients Hospitalized for other cere-
interview 2,214 patients who survived having a stroke
patients was 62 years old (SD = ± 12.3) ranging from 14 brovascular syndromes, as well as those who had a stroke
between February 3 and December 31, 2014. Results:
to 94 years old, mostly women 67.6% and born in the after Hospitalization, were excluded. Data were col-
From the 3,052 patients investigated by the State Stroke
state of Pernambuco (82%). The variables that showed a lected from the patients’ medical records and analyzed
Registry, 838 (27.5%) died while Hospitalized. Of these
statistically significant association with stroke were: sex, using Excel and SPSS programs. The study is part of the
2,214 survivors, 1,209 patients were excluded (27 refused
congestive heart failure, alcoholism, not-sinus electrocar- research project approved by the Hospital’s CEP -CAE
to participate, 217 did not answer the phone calls, and 961
diogram, left bundle branch block, right bundle branch 48835021.6.0000.5043. Results: 75 patients were studied,
changed their telephone number). The remaining 1,005
block, anterosuperior divisional block, atrial fibrillation, 60 of which were diagnosed with Ischemic Stroke, 14 with
had a mean age of 66.8 ± 14.8 years. After five years, 347
left ventricular ejection fraction, and use of oral anti- Hemorrhagic Stroke and 1 with CVT. The mean age of
(34.5%) patients had died; 124 (12.3%) did not present any
coagulants (p <0.05). Variables with p?0.20, except for the patients was 75.8 years and 60% were female. 86.66%
deficit or disability; 116 (11.5%) presented some deficit
drugs that are a consequence and not a cause of stroke, were discharged from Hospital, 3 remained Hospitalized
but no disability, and 88 (8.7%) presented deficits that
were included in the multivariate analysis, with heart until the end of the clipping and 7 of them died. Of all
were considered severe disabilities and were bedridden.
failure, atrial fibrillation, right bundle branch block and patients, 6.66% were diagnosed with COVID-19 during
Conclusion: Our data show that five years after surviving
anterosuperior divisional block being the risk factors that Hospitalization. 11 patients were indicated for palliative
a stroke, approximately one-third of patients are indepen-
remained significant. Conclusion: Although risk factors care, and of these patients, 54.54% died, 18.18% were dis-
dent, one-third rely on assistance for their everyday lives,
for atheromatosis such as diabetes, dyslipidemia, obe- charged from the Hospital and 27.27% were Hospitalized
and one-third are deceased. These numbers are aligned
sity, smoking and alcoholism are present in patients with by the end of data collection. Conclusion: Most patients
with other studies. Age above 50 years, previous stroke,
stroke and Chagas disease, the variables related to heart were Hospitalized for ischemic stroke, aged over 70 years
and atrial fibrillation were strong poor prognostic predic-
disease are the most related to the occurrence of stroke. and were discharged from Hospital. Of the 75 patients, 5
tors, though obesity and dyslipidemia were paradoxically
associated with better prognoses. cases were found with a positive COVID-19 test and of the
7 deaths, 6 entered the Hospital’s palliative care line. The
Doença Cerebrovascular
study is relevant in assessing the importance of the stroke
Doença Cerebrovascular itself, even in the face of the pandemic. The authors also
emphasize the importance of Palliative Care for stroke
patients. There is a need for further analysis on the sub-
ject, in order to approach the patient and his family in
the most appropriate and comprehensive way possible.

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XXX Congresso Brasileiro de Neurologia

TL 1106249 TL 1106261 TL 1106267

MACHINE LEARNING APPROACH IN TELECARE IN NURSING CONSULTATION IMPACT OF DELIRIUM DURATION ON STROKE
PREDICTION OF NASOENTERAL TUBE IN TO STROKE PATIENTS USING WARFARIN: OUTCOMES
ACUTE ISCHEMIC STROKE EXPERIENCE REPORT
Gabriel Souza Suzart, Iva Taiuan Fialho Silva,
Maria Sheila Rocha, Michel Ferreira Machado, Samia Jardelle Costa de Freitas Maniva, Nair Catarina Secundino, Gabriel Praxedes, Maria
Percilia Cardoso, Pablo Nascimento Oliveira, Paola Assunta Antônia Corso Câmara, Vera Lúcia nogueira Eduarda Vassoler, Thaís Barbosa, Milena Fernandes,
Marques da Silva, Geovana Veronezi Augusto, Júlian Ramos, Deborath Lúcia de Oliveira Diniz Tayla Samanta Silva dos Santos, Silas Paulo Lima
Letícia Freitas, Luiz Fernando Oliveira, Luiz Dalfior Jr de Souza, Pedro Antonio Pereira de Jesus
Universidade Federal do Ceará. Fortaleza CE, Brazil
Hospital Santa Marcelina. São Paulo SP, Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil Universidade Federal da Bahia. Salvador BA, Brazil
Hospital Geral Roberto Santos. Salvador BA, Brazil
julian.leticia.freitas@gmail.com samia.jardelle@ufc.br
gabriel.suzart@ufba.br
Background: Dysphagia occurs in about 20 to 50% of acute With The COVID-19 pandemic, health services gad to
stroke patients, and it may persist longer than six months make adjustments in their routines to harm patient care. Background: Delirium is a common complication after
after stroke. Nasoenteral tube feeding (NTF) must be judi- Thus, medical and nursing telecare was incorporated as stroke and its occurrence is associated with worse out-
ciously prescribed to avoid dysphagia complications and, a strategy to combat the pandemic. This study aimed to comes in this population. Longer delirium duration
at the same time, prevent its unnecessary usage, which report the experience of telecare in nursing consultation seems to be associated with higher cognitive impairment
is not free of adverse events. Objective: Our main aim to stroke patients using warfarin. This was a descriptive in critical illness. However, literature is scarce in studies
was to state independent predictive factors associated study, the type of experience report. The scenario was the investigating its association with death and functional
with dysphagia and nasoenteral tube feeding. Besides, neurology outpatient clinic of a tertiary public Hospital, dependence in stroke patients. Objective: We aimed to
we aimed to develop one prediction model for nasoen- located in the city of Fortaleza, Ceará. Results a nurse and evaluate the impact of delirium duration on stroke prog-
teral tube feeding through a machine learning modeling a neurologist implemented telecare for patients using nosis. Methods: Prospective cohort of stroke patients with
approach. Methods: This is a prospective cohort study. All warfarin in September 2020, through synchronous con- symptom onset within 72 hours before research admis-
consecutive ischemic acute stroke patients were included. sultation, on Tuesdays, in the afternoon. The average num- sion. Delirium was diagnosed by Confusion Assessment
All patients had at least one phonoaudiological evalua- ber of attendance/day was 25. The neurology outpatient Method in an Intensive Care Unit and coma was defined
tion for dysphagia screening. Data analyzed included clinic was equipped with devices, which allowed real time by Richmond Agitation-Sedation Scale of minus 4 (respon-
age, sex, Glasgow coma scale, NIHSS, Aspects score, video calls with users, and the health professionals were sive only to physical stimulus) or minus 5 (unresponsive
Seattle comorbid index, Rankin scale, previous and at trained to use telecare system. The patients were previously to physical stimulus). Delirium duration measurement
discharge, TOAST classification for stroke subtypes, pres- scheduled, by phone call, and on the scheduled day, they was adjusted by considering as the main predictor the
ence of major stroke risk factors and data from CT scan started the consultation via WhatsApp, waiting in a virtual number of days alive free of delirium or coma (DFDC)
of all patients. Results: This research studied 1101 acute waiting room for care with the nurse. During the nursing after admission. We calculated delirium/coma-free days
stroke patients. Twenty-eight percent of stroke patients consultation, the main activities developed were: patient/ (DCFDs) as follows: DCFDs = 30 – days of delirium –
went through nasoenteral tube feeding. They were older family welcoming; health status assessment/complaints/ days of coma – days deceased. We did not require that
(p<0.001), had a more severe stroke (p<0.001), and pre- complications; monitoring of protrombina time, whose delirium/coma-free days occur consecutively. Our main
sented consciousness disturbance and dysarthria more examination file was sent by the patient; and registration outcome was functional dependence or death (mRS>2),
frequently (p<0.001 for both measures). These findings in the follow-up form; adjustment of the dose of warfa- evaluated by modified Rankin Scale, at 90 days post-ictus.
predicted independently nasoenteral tube feeding in rin, according to the outpatient anticoagulation protocol Results: Three hundred sixty three patients were enrolled.
acute stroke. The decision tree model disclosed a sensi- adopted in the service; return consultation schedule; edu- Delirium was diagnosed in 85 (23,4) patients, who pre-
tivity of 75% and specificity of 87%, with 84% accuracy for cational guidance on care related to the use of warfarin sented a median number of 25 (19,5-29,0) days alive with-
predicting nasoenteral tube feeding. On the other hand, and registration of the telecare in the patient´s medical out delirium or coma. Patients who developed delirium
the artificial neural network predicted 83% accurately, record. In case of clinical complications, the patient was and died or became functionally dependent in long term
disclosing a sensitivity of 70% and specificity of 86%. An referred to teleconsultation with a neurologist via system. had fewer DFDC [median (IQR); 25 (17,2-28) vs 29 (26-
application for clinical decision support was developed Telecare proved to be a useful tool that continued to be 29) days, p<0,001]. Multivariate analysis showed DFDC
based on the decision tree model, which disclosed bet- requested by patients, even with the return of face-to-face (odds ratio, 0,80 [IC 95%, 0,75-0,86]) as an independent
ter sensitivity and accuracy for nasoenteral tube feeding care. Patients who preferred this modality of care were predictor of mRS > 2 in 90 days, as well as age, sex, Charlson
prediction. Conclusion: Dysphagia occurred in one third those who with physical or financial problems or residents Comorbidity Index, National Institutes of Health Stroke
of cases. Older age, stroke severity, dysarthria, reduced in other cities. The nursing teleconsultation allowed the Scale on admission, thrombolytic treatment and infection
conscious level at onset independently predict the need simultaneous interactivity of the nurse with the patient occurrence. Conclusion: A first episode of delirium after
for nasoenteral tube. The tree decision model is a accurate using warfarin, contributing to the continuity of care and stroke is usually not the only one, being followed by other
tool for predicting NTF in acute ischemic stroke patients. minimize health problems resulting from the treatment. days of delirium or coma. Longer duration of delirium is
associated with higher risk of death and worse functional
outcome in this population. Delirium duration seems to
Doença Cerebrovascular Doença Cerebrovascular be a parameter of significant clinical relevance, being a
valid outcome to be adopted in clinical trials of delirium
prevention or mitigation. To our knowledge, it is the first
study to investigate prospectively the effect of delirium
duration on stroke functional outcome.

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XXX Congresso Brasileiro de Neurologia

TL 1106272 TL 1106293 TL 1106307

MILD CAROTID STENOSIS FREQUENCY IN CEREBRAL OXIDATIVE STRESS IN SYSTEMIC CAUSES FOR NOT PERFORMING
PATIENTS WITH EMBOLIC ISCHEMIC STROKE INFECTION AFTER ISCHEMIC STROKE INTRAVENOUS THROMBOLYSIS FOR
OF UNDETERMINED SOURCE (ESUS) ISCHEMIC STROKE PATIENTS IN A HOSPITAL
Larissa Silva Joaquim, Beatriz Steiner, Lucineia
Gainski Danielski, Fernanda Gava, Richard Simon WITH A CERTIFIED STROKE PROGRAM
Lucy Rodrigues-Ribeiro, Julia Barreto de Farias,
Pedro Fernandes Abbade, Eric Aguiar Wittlich, Victor Machado, Brenno Farias, Khiany Mathias, Jaqueline Saulo Ramos Ribeiro, Ana Lucia Carvalho Mello,
Luis Peixoto Pereira Botelho, Maria Eduarda Lisbôa Silva Generoso, Rafael Mariano Bitencourt, Fabricia Carolina F V Miranda, Evelyn Pacheco, Mariana
Marques, Israela Souza Brito Santos, Carita Victoria Petronilho Okada, Daniel C Bezerra, João Jose Freitas de
Carvalho de Santana, Isabella Reis Vieira, Jamary Carvalho, Jose Carlos Teixeira Gomes, Gustavo
UNISUL – Tubarão – SC – Brazil
Oliveira-Filho Kuster, Renan Domingues
UNESC – Criciúma – SC – Brazil
Universidade Federal da Bahia. Salvador BA, Brazil Américas Serviços Médicos/UHG. Rio de Janeiro RJ,
larij2010@hotmail.com
Brazil
lucyrribeiro@hotmail.com
Background: Ischemic Stroke (AVCi) represents 87% of Samaritano Paulista/UHG
all stroke, and in Brazil already reached 222.773 thousand Amil Assistência Médica
Background: Etiological classifications define carotid
individuals. AVCi has a greater effect in women than in Américas Serviços Médicos/UHG. Fortaleza CE,
stenosis ?50% as a source of ischemic stroke. However,
men, then women have more events and are less likely Brazil
some studies showed a higher frequency of mild carotid
to recover. The pathophusiological mechanisms of AVCi Samaritano Paulista
stenosis (<50%) ipsilateral to Embolic Ischemic Stroke of
determine evenst suc as neuroinflammation and oxidative Américas Serviços Médicos/UHG
Undetermined Source (ESUS), as well as high-risk features,
such as intraplaque hemorrhage, thrombus and ulceration. stress, leading the individual to a systemic imune suppres- sauloramosribeiro@gmail.com
Objectives: To investigate the frequency of mild carotid sion, being sepsis one of them. The role of oxidative stress
stenosis and to assess whether it occurs more frequently associated with inflammatory conditions is well character- Background: Thrombolytic treatment (TT) reduces the
ipsilateral than contralateral to cerebral infarction in ized, event that can be observed in brain áreas such as the morbidity and mortality of ischemic stroke when per-
ESUS. Methods: Cross-sectional study based on medical prefrontal cortex and hippocampus. Objective: To evaluate formed within 4.5 hours after stroke symptoms. Therefore,
records of adult (?18 years) ESUS patients from a stroke the relationship between AVCi and sepsis through oxida- it is crucial to expand the performance of this treatment.
outpatient clinic. ESUS was defined as imaging-confirmed tive parameters in female rats submetted to the animal Objective: To understand the reasons for not performing
non-lacunar stroke; absence of ?50% stenosis on arteries model of AVCi and sepsis. Methods: Female Wistar rats TT in ischemic stroke patients. Method: We retrospec-
that supply the area of ischemia; and no cardioembolic aged 60 days were randomized into groups: SHAM+SHAM, tively evaluated the database of the stroke program of a
or other rare specific sources of stroke. The frequencies SHAM+MCAO, SHAM+CLP, MCAO+CLP, and submitted private Hospital in the city of SP over one year. All data
of mild carotid stenosis and side of cerebral infarction on day 0 to Middle Cerebral Artery Occlusion (MCAO) from patients admitted to the program during this period
were compared using Fisher’s exact test. Results: A total and on day 7 submitted to Cecal Ligation and Perforation were analyzed. Patients that were transferred from other
of 55 participants with ESUS were enrolled. The mean (CLP) technique, suffering painless death after 24 hours services were excluded from this analysis. The percentage
age was 61±11.3 years and 28 (50.9%) were women. Mild and the hippocampus and prefrontal cortex were removed of patients undergoing TT and the exclusion reasons were
carotid disease was present in 27 individuals (49.1%) and for biochemical analysis. Result: We observed that sep- analyzed, based on the criteria of non-eligibility for this
this group was significantly older (65.4±9.3 vs 56.8±12.2, sis exacerbated reactive oxygen and nitrogen species in treatment. Results: Data from 272 patients were analyzed.
p<0.001). Among these, 21 (77.8%) had bilateral mild animals that suffered AVCi as well as increased sensitivity Of these, 137 (59%) were included in the protocol (ischemic
carotid stenosis and unilateral cerebral infarction and of lipid peroxidation in sepsis. The brain damage caused and hemorrhage stroke, TIA, subarachnoid hemorrhage).
two (7.4%) had bilateral stenosis and bilateral infarcts. Of by AVCi induces protein oxidation and the association Ninety-five patients (69%) were confirmed as having isch-
patients with unilateral stenosis, two (7.4%) had contra- of two conditions was effective to potentiate a decrease emic stroke (69%). Of these, 40 patients were excluded
lateral and one (3.7%) had ipsilateral infarct. One (3.7%) of antioxidant enzyme catalase decrease. Conclusion: because they had been transferred from other services.
participant presented with unilateral stenosis and bilateral Sepsis can potentiate brain oxidative stress in female Of the 55 patients included in the analysis, 8 (14%) were
cerebral infarction. There was no difference of mild carotid rats undergoing AVCi. submitted to TT. Of the 47 (86%) who did not receive TT,
stenosis frequency when comparing ipsilateral, contra- the causes were: ischemic stroke occurring outside the
lateral or bilateral infarct groups (p=0.456). Considering therapeutic window (75%), large ischemic stroke area on
carotids as individual observations, we evaluated 110 Doença Cerebrovascular
arrival or previous stroke that occurred less than 3 months
carotids, 50 of them presenting mild stenosis, with 27 ago (2%), postoperative period of recent major surgery (5
(54.0%) ipsilateral to stroke and 23 (46.0%) contralateral %), use of oral anticoagulants (7%), and ischemic stroke
to cerebral infarction, with no significantly association with multiple comorbidities (12%). Conclusion: We found
(p=0.848). Conclusion: In our sample, assessment of that the most important challenge to increase the percent-
stenosis laterality is not sufficient to suggest an associa- age of cases treated with TT is to reduce the arrival time
tion with stroke, given the large volume of patients with of patients to the health service. Educations actions for
bilateral stenosis. The evaluation of plaque high-risk fea- the population are crucial in this regard.
tures by means of exams such as carotid plaque MRI is a
resource to be studied.
Doença Cerebrovascular

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XXX Congresso Brasileiro de Neurologia

TL 1106320 TL 1106348 TL 1106360

CEREBRAL VENOUS THROMBOSIS IN TIMES LESION MAPPING IN PATIENTS WITH TELENEUROLOGY CARE IN ACUTE
OF PANDEMIC SARS-COV-2: NEUROCOVID STROKE SUBMITTED TO TRANSCRANIAL CEREBROVASCULAR DISEASE
STUDY CASE SERIES DIRECT CURRENT STIMULATION FOR
Saulo Ramos Ribeiro, Miguel Rossi Picanço,
UNILATERAL SPATIAL NEGLECT Wanderson Rebello Teixeira, Valério Carvalho Jr,
Marta Rodrigues de Carvalho, Felipe von Glehn da
Silva, Marcia Silva Santos Neiva, Cristiane Campello Mayara Apolinário Januzzi, Gustavo Luvizutto, Júlio João José de Freitas Carvalho, Renan Domingues,
Bresani Salvi, Clarice Neuenschwander Lins de Cesar Moreira, Rodrigo Bazan, Luiz Eduardo Betting Daniel C Bezerra, Gustavo Kuster, José Luiz
Morais Fonseca, Maria de Fatima Pessoa Militao de Carneiro, Evelyn Pacheco
Albuquerque, Maria Cynthia Braga, Jurandy Júnior UNESP-FMB Botucatu – Botucatu – SP – Brazil
UNESP-FMB – Botucatu – SP – Brazil Américas Serviços Médicos. Rio de Janeiro RJ, Brazil
Ferraz de Magalhães, Maria Lucia Brito Ferreira,
Pró Cardíaco/UHG. Rio de Janeiro RJ, Brazil
Lucas Luiz Vieira
mayjanuzzi@gmail.com Américas Serviços Médicos/UHG. Fortaleza CE,
Universidade de Brasília. Brasília DF, Brazil Brazil
Hospital Universitário de Brasília. Brasília DF, Brazil Background: Transcranial direct current stimulation Amil Assistência Médica/UHG
Fundação Oswaldo Cruz. Recife PE, Brazil (tDCS) may be useful in the management of patients
with stroke and unilateral spatial neglect. In a previous sauloramosribeiro@gmail.com
Laboratório Central de Brasília. Brasília DF, Brazil
investigation we described a better recovery of patients
marta_rodrigues12@hotmail.com submitted to anodal tDCS. Objective: Use neuroim- Background: Stroke treatment is extremely effective, but
aging to access structural predictors of the outcomes its benefit is time-dependent, making it challenging to
Background: Cerebral venous thrombosis (CVT) is a rare those patients submitted to tDCS. Methods: Patients with achieve the goals established by the guidelines. The neurol-
cause of stroke associated with significant morbidity and stroke and unilateral spatial neglect were randomized in ogist participation is essential for the best clinical decision
mortality. COVID-19 began in 2019, when an outbreak of 3 groups (anodal tDCS [group 1] cathodal tDCS [group and teleneurology has been used to increase the access
atypical pneumonia occurred in the city of Wuhan (China), 2] and control [group 3]). High resolution computerized of these cases to specialized management. Objective:
and the etiologic agent was identified as a novel human tomography scans in the sub-acute phase of the stroke Demonstrate the results of a private teleneurology net-
pathogen from the family Coronaviridae, called Severe were obtained. Lesions were semi-automatically seg- work. Methods: We evaluated patients admitted to the
Acute Respiratory Syndrome Coronavirus type 2 (SARS- mented and normalized. Extension of the lesions based database from January 2021 to April 2022 from a private
CoV-2). As the pandemic progressed, cases of stroke in of regions of interest (ROIs) were extracted using the Atlas network of Hospitals located in 2 brazilian states(SP and
young patients without cardiovascular risk factors were of Anatomical Labeling. Only gray matter of right hemi- RJ), totaling 15 Hospitals that use teleneurology. Several
reported and new cases of CVT associated with both the sphere was evaluated, and the total area analyzed was interventions were carried out to improve the indicators
infection and vaccination were also described. Objective: 642.745mm3. Statistical analysis was conducted using in previous years, such as: continuous training, monthly
To describe the clinical, epidemiological, and laboratory chi-square test. Results: For group 1 (n=8, mean age 66 meetings, managed protocol. The KPI evaluated were:
characteristics of patients with cerebral venous throm- years), extension of damage obtained was 194.241mm3 Median door-to-image time (mPI); median door-to-needle
bosis admitted to a public referral Hospital. Methods: (30% of all ROIs) centered in the amygdala; for group 2 (mPA); venous thrombolysis rate (txTV), mortality rate (tx
This is a case series study nested within the multicenter (n=8, mean age 61), 79.100mm3 (12% of all ROIs) cen- mort) and median length of stay (mTI). Results: A total of
case-control entitled NeuroCOVID: Association of SARS- tered in the amygdala and for group 3 (n=7, mean age 2081 patients were admitted during the study period, with
CoV-2 with the occurrence, prognosis and pathogenesis 68) 49.789mm3 (8% of all ROIs) centered in the insula. mPI=24min; mPA=68min; txTV=15%; txmort=7%; mTI=4
of cerebrovascular diseases, funded by the Inova Fiocruz Group 1 was statistically different when compared to days. Conclusion: About 4000 patients are included in the
Program (Fundação Oswaldo Cruz – COVID-19 Emergency group 2 and 3 (p=0.001 and p<0.001). There was no dif- database annually, and approximately 1800 patients with a
Fund: VPPCB-005 FIO-20-2-22). Results: Over a 1-year ference between group 2 and 3 (p=0.9). Conclusion: A stroke diagnosis confirmed. Telemedicine provides access
period of data collection, 19 patients included had a clin- larger area of damage was observed in patients with stroke to vascular neurology expertise for Hospitals without it,
ical and radiological diagnosis of CVT. It is evident that submitted to anodal tDCS. Despite the increased area of and its use has increased rapidly in recent years. Access
84.2% of the sample was composed of women and 42.1% damage compared to the other groups these individuals to such technology demonstrates an improvement in the
self-declared as brown. Only 26.3% and 5.3% reported presented a better outcome indicating that this approach KPIs of the acute phase of stroke. The results founded are
having hypertension and diabetes mellitus, respectively. may be useful in these patients. congruent with previous studies, showing that teleneu-
Approximately 62.5% of women used contraceptives. Only rology in a private network is an important support and
2 patients reported a diagnosis of COVID-19 in the 30 measures of continuous improvement of the process
days preceding Hospitalization. Headache was the most Doença Cerebrovascular are fundamental.
reported symptom (89.5%), followed by seizures (26.3%).
Approximately 63.2% had venous infarction and 52.6%
had an associated hemorrhagic event. Thirty-six percent Doença Cerebrovascular
received covid19 vaccination with pfizer; 21.1% received
astrazeneca. The minimum, median, and maximum times
between vaccination and the occurrence of CVT were, in
days: 22, 75, and 159, respectively. Two patients (10.5%)
were RT-PCR positive for COVID19 on admission; 63.2%
were IgA positive for COVID19 and 73.7% were IgG posi-
tive for COVID19. The minimum, median, and maximum
number of platelets were: 158.000, 221.350 and 382.500/
mm3. Conclusion: Our study highlights the clinical and
laboratory characteristics of patients with CVT in pan-
demic COVID19. The main limitation is that this is a small
case series from a single center.

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PROFILE ASSESSMENT OF MOYAMOYA POST-STROKE DYSPHAGIA IN PATIENTS HAS THE IMPACT OF CEREBROVASCULAR
SYNDROME IN PATIENTS FROM THE UNDERGOING THROMBOLYTIC TREATMENT: DISEASES CHANGED DURING THE COVID-19
NORTHEAST: CASE SERIES A RETROSPECTIVE COHORT PANDEMIC?
Barbara Matos Almeida Queiroz, Matheus Costa Isaac Rêgo Purificação, Felipe Oliveira Costa, Luciana Oliveira Neves, Clarissa Rocha Montenegro,
Bessa, Karoline Ferreira Mororo Menezes, Alissa Fernanda Souza Gracílio Silva Igor Bessa Santiago, João Renato Figueiredo Souza,
Elen Formiga Moura, Matheus da Costa Guedes, Benevides José Silva Santos, Sthefany Lorrany
Vitoria Flexa Ribeiro, Francisco Jose Arruda Escola Bahiana de Medicina e Saúde Pública. Nepomuceno Santos, Rafaela Silva Félix
Mont’alverne, Norberto Anizio Ferreira Frota, Salvador BA, Brazil
Fernanda Martins Maia Hospital do Subúrbio. Salvador BA, Brazil Hospital São Carlos. Fortaleza CE, Brazil

Hospital Geral de Fortaleza. Fortaleza CE, Brazil fernandasilva19.2@Bahiana.edu.br dra.luciananeves@gmail.com


Universidade de Fortaleza. Fortaleza CE, Brazil
Background: For the treatment of ischemic stroke in its Background: Stroke, both ischemic and hemorrhagic,
barbaramatosaq@gmail.com acute phase, the use of intravenous thrombolysis with is an important cause of morbidity and mortality world-
Alteplase (rt-PA) is currently recommended, as it is capa- wide. Due to the SARS-COV-2 pandemic, patients were
Background: MoyaMoya syndrome is an uncommon cere- ble of reducing the functional deficits and the severity of afraid to seek medical care for fear of contracting COVID
bral vasculopathy worldwide and rare in South America, the lesion. However, despite the advances in care and while health professionals were, amid the search for beds
in which reduced blood flow in the internal carotid artery reduction of functional deficits in stroke patients, dys- to care for all patients with respiratory symptoms, alerts
promotes compensatory angiogenesis. Known risk fac- phagia is a common dysfunction. Nevertheless, there for the association of COVID with stroke, in addition to
tors associated are systemic arterial hypertension, type 2 are few studies related to thrombolytic treatment and strict precautions so that patients do not contract COVID
diabetes and Down Syndrome. Objectives: Describe clin- its effects on dysphagia, with divergent results regarding after Hospitalization. Objective: To describe the number
ical-epidemiologic profile of 7 patients with MoyaMoya the benefits of this drug on the severity and incidence of of patients Hospitalized for ischemic stroke, hemorrhagic
Syndrome Methods: Report of seven cases of MoyaMoya dysphagia. Objective: To analyze dysphagia at Hospital stroke and cerebral venous thrombosis (CVT) and their
Syndrome in a tertiary neurology department Results: discharge after stroke in patients undergoing and not respective outcomes from March 2019 to February 2022.
In our series, all patients were female, in an age range of undergoing thrombolytic treatment. Method: A retrospec- To describe the number of patients who were diagnosed
18-49 years. All of them had sudden onset of focal neu- tive cohort was carried out in a stroke referral Hospital with COVID-19 in the period of March from 2020 to
rological symptoms, which prompted the investigation. in Salvador -BA, in which patients were divided into two February 2022. To assess whether there was a difference
Three patients had ischemic stroke, with varied Bamford groups: submitted and not submitted to thrombolytic in the incidence of cerebrovascular diseases during the
Classification of anterior circulation syndrome. Four treatment. This research was submitted to and approved COVID-19 pandemic in a private Hospital in Fortaleza.
patients had hemorrhagic events, one with association by the Research Ethics Committee. The patient’s socio- Methods: The study design was cross-sectional, obser-
of Subarachnoid Acute Hemorrhage (SAH) and Subdural demographic and clinical characteristics were analyzed, vational, descriptive, evaluating patients Hospitalized
Hemorrhage, one withTetraventricular Hemorrhage and and the incidence and severity of dysphagia between the for ischemic stroke, hemorrhagic stroke and CVT from
two Frontal Lobar Intraparenchymal Hemorrhage. The groups were compared. The Dysphagia Outcome and March 2019 to February 2022 who had a diagnosis of
angiographic study of the patients showed Suzuki-Takaku Severity Scale (DOSS) was used to analyze dysphagia. COVID after their Hospitalization. Patients who did not
stages of 3 and 4. Two patients had neurosurgery indica- Patients with some degree of dysphagia (DOSS ? 5) or no have a stroke confirmed by imaging or clinical findings
tion, but one refused and another died in anesthesical dysphagia (DOSS>5) were considered. Data were analyzed were excluded from the study. Data were collected from
procedure. Two patients had brain death because of using descriptive statistics and compared by parametric the patients’ medical records. The study is an arm of the
hemorrhage. The other patients remain in neurologic or nonparametric tests, when appropriate. Moreover, a approved research project at the Hospital’s CEP. Results:
department clinic follow-up. Conclusion: Moya Moya univariate analysis was performed for the outcome dys- From March 2019 to February 2020, the year in which the
Syndrome is rare in our population, with high potential phagia at Hospital discharge and the variables that were pandemic had not started, 85 patients were Hospitalized,
to provoke cerebrovascular events, with disabilities and significant (p<0.05), were taken to logistic regression mean age 75 years, 53% female, 74% with stroke, 26%
death. There is low evidence of pharmacologic treatment, to assess whether the thrombolytic is an independent stroke, 14% death. From March 2020 to February 2021,
being surgery the main strategy of approach in these protective factor. Results: A total of 120 thrombolyzed 95 patients were Hospitalized, mean age 72 years, 55%
cases. Therefore, we recommend furthering studies of and 117 nonthrombolyzed patients were included in the female, 77% stroke, 17% stroke, 6% CVT, 15.7% death, 3.1%
patogenesis, prognosis and treatment of this disease, to final analysis. Dysphagia at Hospital discharge affected COVID+. From March 2021 to February 2022, 90 patients
minimize complications. 23(19.2%) thrombolyzed and 41(35%) nonthrombolyzed were Hospitalized, mean age 72 years, 55% female, 79%
patients (p=0.006). There was no difference regarding the stroke, 6% stroke, 5% CVT, 14.4% death, 10% COVID +.
severity of dysphagia between the groups (p=0.158). For Conclusion: The number of Hospitalizations for stroke
Doença Cerebrovascular multivariate analysis, thrombolysis was an independent was not impacted in the 24 months of the pandemic.
protective factor for dysphagia at Hospital discharge [OR: However, an increase in the incidence of CVT related to
0.29 (CI: 0.08-0.95); p=0.042]. Conclusion: The incidence COVID+ patients was observed in most cases, which cor-
of dysphagia at Hospital discharge was significantly higher roborates the association found in the literature. Regarding
in nonthrombolyzed patients, with no effect on dysphagia contamination by COVID after admission, there is no evi-
severity. Furthermore, thrombolysis was an independent dence that patients Hospitalized for stroke contracted it.
protective factor for dysphagia at Hospital discharge.

Doença Cerebrovascular
Doença Cerebrovascular

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TL 1106492 TL 1106496 TL 1106520

RECOGNIZING AND REACTING TO A THE COMPLICATIONS OF PATIENTS THE STROKE AND ISCHEMIC HEART
HYPOTHETICAL STROKE SCENARIO IN HOSPITALIZED IN STROKE UNIT CARE OF DISEASE DISCONNECTION: THE NUMBERS
BRAZIL’S NORTHEAST THE HOSPITAL IN PERNAMBUCO FROM CEARÁ, BRAZIL
Mario Luciano Melo Silva Junior, Giuliana Maria Monique Evelyn Mendonça do Nascimento, Ana João José Freitas de Carvalho, Fabricio Oliveira Lima
Morais Gonzalez, Neila Clediane Sousa Menezes, Dolores Firmino Santos do Nascimento, Vitor Maia
Roberto Carlos Sousa Alves Junior, Yuri Almeida ARCA, Pedro Lucas Negromonte Guerra, Maria Unichristus School of Medicine. Fortaleza CE, Brazil
Oliveira, Caline Almeida Barbosa, Rômulo Martins Eduarda da Costa Brandão Justino Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Ferreira Santos, Rasec Kayan Oliveira Santos, jjcarvalho@gmail.com
Marcos Vinícius de Souza Vilanova Hospital da RESTAURAÇÃO de PERNAMBUCO.
Recife PE, Brazil
Universidade Federal de Pernambuco. Recife PE, Hospital das Clínicas da Universidade Federal de Background: Stroke and ischemic heart disease (IHD)
Brazil Pernambuco. Recife PE, Brazil are Brazil’s leading causes of death. However, mortality
Universidade Ceuma – São Luiz – MA – Brazil Faculdade Pernambucana de Saúde. Recife PE, rates have declined for both diseases since the 70s. This
Uninassau. Recife PE, Brazil Brazil has been more pronounced in regions with higher socio-
Centro Universitário Uninovafapi. Teresina PI, Brazil economic status (mainly in the southeast and south),
Universidade Federal de Campina Grande. Campina moniqueemnascimento@gmail.com probably due to better control of risk factors, access to
Grande PB, Brazil health care services, and treatment of hypertension and
Universidade Federal do Oeste da Bahia – Barreiras Background: Stroke is a condition characterized by diabetes through national government programs. There
– BA – Brazil reduced or absent blood flow to a certain area of ??the are very few epidemiological studies on stroke and IHD
Centro Universitário CESMAC. Maceió AL, Brazil brain. According to its etiology, it can be classified as mortality trends in regions with lower socioeconomic
ischemic, when caused by the occlusion of an intracra- status, such as the Northeast. Objective: This study aimed
mariomelosjr@gmail.com nial or extracranial artery. Stroke can also be classified as to investigate the trend of mortality rates from stroke and
hemorrhagic, when caused by the rupture of intracranial IHD in Ceará, a state with low socioeconomic status in
Background: Recognizing the alarming signs of stroke is vessels due to conditions such as arteriovenous malfor- Northeast Brazil, from 2009 to 2019. Methods: The mor-
the first step to starting a proper treatment reaction chain. mations or aneurysms. Among the types of stroke, the one tality data were obtained from the brazilian Mortality
Objectives: We aimed to assess the frequency of awareness with the highest incidence is ischemic, which globally Information System made available by the National Health
of the laypeople on recognition and reaction to stroke. reached the mark of 7.2 million cases in 2019, which rep- Ministry. The mortality rate estimates, expressed as the
Methods: Cross-sectional survey-based study, which data resented 62% of the total. In second place is intracranial number of deaths per 100 000 inhabitants, were cal-
was collected in 2020, in twelve cities of Brazil’s Northeast. hemorrhage responsible for 3.4 million cases of stroke or culated for each year—population estimates derived
The participants were exposed to a stroke case study and 28% of the total, followed by subarachnoid hemorrhage from the brazilian Institute of Geography and Statistics
then responded to an open-ended semi-structured ques- with 1.2 million cases in 2019 and approximately 10% of (IBGE). The proportion of deaths caused by stroke and
tionnaire to evaluate their stroke knowledge. Results: A the total. Despite the severity of the disease, there have ischemic heart disease was also calculated, and statistical
total of 1,475 subjects were enrolled in this study (52.6% been many advances in the matter of treatment, espe- significance was tested. Results: A consistent decline in
women, 36.2±15.3 years old). 1,220/1,475 (82.7%) recog- cially for ischemic stroke, for which, in eligible patients, stroke mortality was observed from 2009 to 2019 (10,0%,
nized the situation as a stroke; 1,148/1,475 (77.8%) would intravenous thrombolysis or mechanical thrombectomy 9,9%, 9,5%, 9,2%, 8,9%, 8,3%, 8,5%, 8,7%, 8,2%, 7,5% and
react to it by taking the patient to the emergency room. Of can be performed. Objective: To describe the complica- 7,5%; p<0.001) but the coronary heart disease mortality
those who recognized the stroke, 19.9% (243/1,220) did tions of patients from the stroke unit care of “Hospital da increased consistently over the years studied (8,0%, 7,7%,
not take an appropriate reaction to it, meaning a recog- Restauração” in the months of April and May 2022, based 8,1%, 8,5%, 8,0%, 8,1%, 8,4%, 8,8%, 8,5%, 9,6% and 9,5%;
nition-reaction gap. Conclusion: The frequency of stroke on the service quality indicator questionnaires. Methods: p<0.001). Conclusion: Stroke is no longer the leading
recognition and reaction to it were acceptable, but could An observational, retrospective and cross-sectional study cause of death among non-communicable diseases in
be improved. The recognition-reaction gap needs to be was carried out on patients admitted to the stroke unit care Ceará, overtaken by ischemic heart disease, which has
addressed by specific campaigns focusing on time-sen- of Hospital da Restauração in Pernambuco in the months shown a consistent increase in recent years. Population-
sitivity aspects for successful stroke outcomes. of April and March 2022. Results: The study observed based epidemiological studies are necessary to clarify
that of the 66 patients who were Hospitalized during the why mortality rates exhibit such a different tendency in
period, 4 had pressure ulcers with lesion stages ranging diseases that share most risk factors.
Doença Cerebrovascular from 2 to 3. In addition, 15 patients used antibiotics to treat
infection in the period, with most used antibiotics ceftri-
axone, which was used in 6 cases and clindamycin, used Doença Cerebrovascular
in 2 cases. Finally, another complication identified was
the use of nutritional devices (11 cases), mainly a naso-
enteral tube, which was used by 9 patients, and a urinary
catheter (1 patient). Conclusion: The study showed that
approximately ¼ of the patients had some complication
post stroke. Some measures can be taken to avoid them,
for example, the follow-up with speech therapists, aiming
to reduce the use of nasoenteral tube, use of protocols to
avoid bronchoaspiration, reducing the use of antibiotics
and change of decubitus to avoid pressure ulcers.

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TL 1106523 TL 1106558 TL 1106573

DIFFERENT NAMES FOR STROKE IN NURSING CONSULTATION TO STROKE EPIDEMIOLOGICAL AND CLINICAL
BRAZIL’S NORTHEAST PATIENTS USING WARFARIN IN A INDICATORS OF A TYPE III EMERGENCY
NEUROLOGY OUTPATIENT CLINIC CARE CENTER FOR STROKE IN JUIZ DE FORA
Mário Luciano Mélo Silva Júnior, Ana Gabriella
Camelo Oliveira, Weslley Medeiros Gois, Matheus (MINAS GERAIS)
Samia Jardelle Costa de Freitas Maniva, Nair
Franco Andrade Oliveira, Lourdes Maria Dantas de Assunta Antônia Corso Câmara, Vera Lúcia Nogueira Natália Virgínia de Oliveira Ambrósio, Jéssica
Góis, Lucas Pereira Ferreira, Marcos Vinícius de Ramos, Waldélia Maria Santos Monteiro, Deborath Cristina Silveira Damasceno, Laura Altomare
Souza Vilanova Lúcia de Oliveira Diniz Fonseca Campos, Laís Ciribelli Yamaguchi, Bruna
Universidade Federal de Pernambuco. Recife PE, Queiróz Vieira, Bruno de Magalhães Barbosa Leite,
Universidade Federal do Ceará. Fortaleza CE, Brazil
Brazil Daniel Teixeira Martins Schettini, Fabiana Veloso
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Universidade Federal de Sergipe – Lagarto – SE – Ferreira, Thiago Cardoso Vale
Brazil samia.jardelle@ufc.br
Hospital e Maternidade Therezinha de Jesus – Juiz
Escola Bahiana de Medicina e Saúde Pública. de Fora – MG – Brazil
Salvador BA, Brazil The outpatient nursing consultation consists of a strategy
Universidade Potiguar – Natal – RN – Brazil of resolutive and quality care, supported by law and exclu- nvoambrosio@gmail.com
Uninassau. Recife PE, Brazil sive to the nurse. In the care of patients, using warfarin is
show to be a tool of great value, because they need close Background: Stroke is one of the leading causes of morbid-
mariomelosjr@gmail.com follow-up, given the specificities of the treatment, such as ity and mortality in Brazil and in the world. Thrombolytic
drug interaction, food and the need for correction in the therapy and stroke units were important advances to
Background: There are different names for stroke in dose of the anticoagulant. This study aimed to report the patients suffering from stroke. The Stroke Unit of the ref-
Brazil, but it is not clear if those names mean the same experience of the nursing consultation to stroke patients erence stroke center in Juiz de Fora has a multidisciplinary
idea of disease. Objectives: To assess the different words using warfarin. This was a descriptive study, the type team to assist stroke patients 24 hours a day, and it serves
for stroke in Brazil and both intended response to it and of experience report. The scenario was the outpatient the entire macro-region of Juiz de Fora, in Minas Gerais
symptoms associated. Methods: Cross-sectional study clinic of a tertiary public Hospital, located in the city of state, exclusively within the scope of “Sistema Unico
enrolling healthy individuals from urban areas in twelve Fortaleza, Ceará. The nursing consultation aimed at this de Saúde” (SUS). Objective: To study epidemiological
cities in all nine states of Northeast Brazil for an open- target audience began in 2020 and took place on Tuesdays, and clinical indicators of a stroke care center in Juiz de
ended survey. We presented a typical clinical case of a in the afternoon. The service was performed by a nurse, Fora (Minas Gerais), Brazil. Method: Medical records
stroke (an elderly who had sudden onset of hemiparaly- in two moments. Initially, a face-to-face care occurred of patients admitted to the emergency department and
sis and slurred speech) and asked “what is happening?”. and then telemedicine care intended for patients with referred to the Hospital from September 2020 to March
Results: From 1,475 interviewed individuals, 1,220 (82,7%) difficulty in physical mobility or residents in other cities. 2022 were retrospectively reviewed. Results: A total of 732
recognized the scenario as a stroke, and they used 4 words The main activities developed by the nurse were: patient/ individuals (371 [50.8%] male, mean age 67 years) with a
to identify the situation as a stroke, which were “AVC”, family welcoming; health status assessment/complaints/ predictive diagnosis of stroke were referred to the Hospital
“derrame”, “trombose” and “aneurisma”. There were signif- complications; monitoring of protrombina time and reg- from September 2020 to March 2022 and their medical
icant differences among them concerning demographic, istration in the follow-up form; adjustment of the dose charts were reviewed. With regards to the comorbidities,
economic, educational and geographical aspects. The of warfarin, according to the outpatient anticoagulation 560 (76.6%) had arterial hypertension and 212 (29.0%)
group who stated “AVC” was younger, had more school protocol adopted in the neurology outpatient clinic and were diabetics. Regarding lifestyle habits, 385 (52.7%)
years and lived in capitals. “Derrame” was cited often return consultation schedule; educational guidance on patients were smokers and 428 (58.5%) were alcohol
in Bahia and in the countryside cities; this group was care related to the use of warfarin, by providing an infor- consumers. Mean symptom onset occurred 691 minutes
made mostly of Blacks and Mullatos. “Trombose” was mation leaflet; referral to the neurologist in case of clinical (11.5 hours) before admission. Mean NIHSS score value
mentioned only in 5 states (Paraíba, Ceará, Maranhão, complications and registration in the patient´s medical was 8.0 points. Thrombolysis was performed in only 57
Piauí and Rio Grande do Norte), other states did not record. The nursing consultation proved to be beneficial (7.9%) of patients. Cranial CT scans were normal in 264
cite this name at all. Conclusion: There are at least four to patients using warfarin, as it allows systematized and (36.2%) of patients. 423 (57.8%) scans showed ischemic
words for stroke in Portuguese (“AVC”, “derrame”, “trom- individualized actions, which contributes to minimize stroke while 45 (6%) showed hemorrhagic stroke. The
bose” and “aneurisma”). Stroke campaigns should apply health problems resulting from the treatment. average time the patients stayed in the Hospital was 11
those alternative names to improve the compression of days. At discharge, the average NIHSS was 4.0 points
the general population. and the mean RANKIN scale score was 3.0 points. 543
Doença Cerebrovascular (74.2%) were discharged with platelet antiaggregation,
633 (86.5%) with statin prescription. Among Hospitalized
Doença Cerebrovascular
patients, 610 (83.4%), received deep vein thrombosis
prophylaxis in the first 48hrs. At Hospital discharge, 579
(79.1%) patients received prescription of a prophylactic
and rehabilitation therapy plan. The total mortality rate
in 10 months of the study period was 71 (9.7%) and the
mean number of deaths was 7.1 deaths, ranging from 4
(0.55%) deaths in September 2021 to 10 (1.36%) deaths
in July 2021. Conclusion: Thrombolysis eligibility rate
was rather similar to other stroke reference centers in
Brazil. We also observed a long time between the onset
of symptoms and arrival at the service, which was the
main barrier to performing thrombolysis, due to loss of
the therapeutic window.

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PANORAMA OF HOSPITALIZATIONS AND IMPACT OF IN-HOSPITAL ACUTE KIDNEY AMYOTROPHIC LATERAL SCLEROSIS
DEATHS ATTRIBUTABLE TO STROKE IN INJURY ON DEATH OR FUNCTIONAL ATTENDED IN A REFERENCE CENTER IN THE
BRAZIL FROM 2016 TO 2022 DEPENDENCY AFTER STROKE CITY OF RIO DE JANEIRO
Bianca Berardo Pessoa Zirpoli, Daniel Cavalcanti Gabriel Souza Suzart, Thaís Barbosa de Oliveira, Rômulo Cerqueira Heckert dos Santos, Larissa
Sena, Maria Valeska Berardo Pessoa de Souza, Vinicius Leal Borges da Cruz, Milena Fernandes de Bittencourt de Carvalho, Ligia Rocha Andrade,
Maria Eduarda Iglesias Berardo de Souza, Patryck Oliveira, Maria Eduarda Messias Vassoler, Ivã Taiuan Clarissa Neves Spitz, Salim Lázaro Balassiano,
Andrew Ribeiro de Melo Pontes Fialho Silva, Tayla Samanta Silva dos Santos, Pedro Robson Teixeira Vital, Márcia Maria Jardim
Antônio Pereira de Jesus Rodrigues
Universidade Católica de Pernambuco. Recife PE,
Brazil Hospital Geral Roberto Santos. Salvador BA, Brazil Hospital Universitário Pedro Ernesto UERJ. Rio de
Prefeitura do Recife. Recife PE, Brazil Janeiro RJ, Brazil
Faculdade Pernambucana de Saúde. Recife PE, thaisbo@ufba.br
Brazil romulochsantos@gmail.com
Background: Renal dysfunction and acute kidney injury
berardobianca@gmail.com (AKI) have been identified as a risk factor to worse out- Background: Amyotrophic lateral sclerosis (ALS) is,
comes in the general population of intensive care units. to date, one of the incurable progressive neurolog-
Background: Cerebrovascular diseases are the second According to previous studies, AKI has been a frequent ical diseases with the worst clinical prognosis. The
cause of death in the world, and stroke a leading cause complication after an acute cerebrovascular event, with an Neuromuscular Diseases Service of the Pedro Ernesto
of long-term disability. In the United States (US) in 3% of overall prevalence around 11.6%. Objective: Our aim is to University Hospital (HUPE/UERJ) is a reference center
males and 2% it led to disability, and caused 6.6 million evaluate the impact of AKI during Hospitalization on stroke that receives patients referred from the primary and
deaths in 2019. Early treatment is critical, but there are patients. Methods: Prospective cohort of stroke patients secondary care levels of the SUS, which provides the
several factors that lead to disparities, while in the US with symptom onset within 72 hours before research meeting of rarer and more complex neurological cases,
whites have higher proportional rates of access to treat- admission. AKI was diagnosed by the occurrence of an such as ALS. Objective: To carry out a retrospective epi-
ments, worldwide the differences are of overall access to increase in the serum creatinine level by approximately demiological study on confirmed cases of ALS treated at
quality health care. Methods: An ecological and descrip- 0.4 mg/dl in less than 72 hours during Hospitalization for the Neuromuscular Diseases Outpatient Clinic at HUPE/
tive study of the Hospitalizations due to Stroke in Brazil, ischemic or hemorrhagic stroke. Our main outcome was UERJ between 2004 and 2022 to identify the locoregional
from January 2016 to April 2022, for which data collection functional dependence or death, evaluated by modified profile of the patients. Methods: Review of care records
was obtained via the Health Information (TAB- NET) and Rankin Scale (mRS), at 90 days post-ictus. Results: Two of patients with suspected or confirmed cases of ALS and
the SUS Hospital Information System (SIH/SUS). Results: hundred and fifty one patients were enrolled. AKI was treated at the Neuromuscular Diseases Outpatient Clinic
During the past 7 years in Brazil there were 127.956 stroke diagnosed in 78 (37.1) patients. The frequency of death of HUPE/UERJ between 12/17/2004 (first record found)
admissions. The region with the highest incidence was the or functional dependence (mRS> 2) was lower among and 05/31/2022, identifying and organizing information
Southeast, with a total of 48.861 cases (38,18%), followed those who were not diagnosed with acute kidney injury related to gender, age, etiology and clinical phenotype
by the Southern region with 35.453 cases. The region with [U = 4.523; 38 (48.7) vs 40 (51.3) cases, p=0.033]. However, of ALS cataloged cases. Results: Ninety-five suspected
the lower incidence was the Central-West, with only 7.119 multivariate analysis failed to show the occurrence of AKI cases of ALS were identified in the 17 years and 5-month
cases (5,56%). There was a predominance of males, rep- as independent predictor of mRS > 2 in 90 days (odds interval considered, of which, after reviewing the consul-
resenting 66.612 cases (52,05%). The age group with most ratio, 1.19 [IC 95%, 1.13-1.25]), which was observed to tations, 16 cases were discarded, making a total of 79 ALS
cases was from 70 to 79 years, with 33.272 cases (26%). variables as age, sex, National Institutes of Health Stroke records. In this series, 45 were men (57%), and 34 were
There were a total of 10.020 deaths due to stroke, with the Scale on admission, type of stroke, pre-ictus mRS score women (43%). The vast majority of cases were idiopathic
highest incidence in patients with 80 years or more (4.023 and infection occurence. Conclusion: Our study suggests (76 cases, 96% of the total), while only 3 cases showed
cases, 40,14%). From 2016 to 2021, there was a decrease of that, although in-Hospital acute kidney injury may be genetic/family inheritance (4%). The most affected age
10,97% in the number of Hospitalizations, with a fluctua- associated with worse outcomes in stroke patients, we group was 50-69 years-old (46 cases, 58%), with a mean
tion of incidence. While 2019 was the year with the highest were not able to describe it as an independent predic- age of 55 years-old, and the most common phenotype was
incidence of Hospitalizations, the year with most deaths tor. That conclusion indicates there may be unknown classic ALS (65 cases, 82%), although were found 4 bulbar
due to stroke was 2016, with 1.755 cases, followed by a variables, which our data was not able to detect, that can ALS, 4 cases of progressive muscular atrophy, 2 cases of
decrease up until 2020, with the lowest cases 1.469, and influence this association. primary lateral sclerosis, 2 cases of monomelic atrophy,
a new increase in 2021 with 1.555 cases. When it came to 1 flail-arm syndrome and 1 Mills variant. Conclusion:
racial differences, patients with the highest Hospitalization The data collected on a locoregional scale, despite the
rates for Stroke where whites, with 38.270 cases (41,9%), Doença Cerebrovascular small number of patients considered (because it is a
followed by browns, blacks, yellows, with the lowest inci- rare disease), are in line with the epidemiological char-
dence among indigenous people, and deaths followed the acteristics of ALS already demonstrated in other studies
proportions of Hospitalizations, with the highest among of national and international scope, showing a predom-
whites 4.057 and lowest among indigenous people, 6. inance of men, sporadic cases as opposed to hereditary
Conclusion: Hence, white men from 70-79 were the most cases, and average age at onset of the typical condition
affected, having the highest amount of Hospitalizations from the sixth decade of life.
and deaths, and the region with most Hospitalizations
was Southeast. It is crucial to focus on primary preven-
tion, especially in primary care, along with educational Doenças do Neurônio Motor – Esclerose Lateral
programs towards the population, so that they may seek Amiotrófica
medical attention sooner, while also making policies and
approaches to improve access to medical care.

Doença Cerebrovascular

242
XXX Congresso Brasileiro de Neurologia

TL 1105456 TL 1105601 TL 1106062

MOTOR CORTEX HYPOINTENSITY IN ANALYSIS OF TELEMONITORING AND A GEOGRAPHICAL STUDY ON AMYOTROPHIC


SUSCETIBILITY WEIGHTED IMAGING: A EARLY INDICATION OF HOME NONINVASIVE LATERAL SCLEROSIS IN RIO GRANDE DO
RADIOLOGIC BIOMARKER OF AMYOTROPHIC MECHANICAL VENTILATION IN PATIENTS NORTE, BRAZIL, FROM 2005 TO 2018
LATERAL SCLEROSIS? WITH AMYOTROPHIC LATERAL SCLEROSIS
Emanuela Coriolano Fidelix, Roberto Teodoro Gurgel
FOLLOWED UP IN A TERTIARY CARE de Oliveira, Glauciane Costa Santana, Maria de
Jessica Santos de Souza Rocha, Luciano Chaves
Rocha, Ricardo Rogério Mendes, Nathalia Barros HOSPITAL Jesus Gonçalves, Guilherme Augusto de Freitas
Ferreira Fregonezi, Sancha Helena de Lima Vale, Lucia Leite
William Nicoleti Turazza da Silva, Maria Fernanda
Lais, Mário Emílio Teixeira Dourado Júnior
Hospital Ophir loyola. Belém PA, Brazil Prado Rosa, Ana Elizabeth Cunha Guimarães
de Almeida, Daniela Name Chaul Vidigal, Laerte Hospital Universitário Onofre Lopes da Universidade
ssouza.jeh@gmail.com Honorato Borges Junior, Vinicius Pafume de Oliveira, Federal do Rio Grandeo do Norte. Natal RN, Brazil
Isabella Cristina Guimarães de Almeida, Mateus Universidade Federal do Rio Grandeo do Norte.
Amyotrophic lateral sclerosis (ALS) is a neurodegenera- Barros Bueno, Glauber Mota Pacheco, Diogo Natal RN, Brazil
tive disease of the central nervous system characterized Fernandes dos Santos
by progressive loss of upper (UMN) and lower motor efidelix@gmail.com
neurons that results in loss of voluntary movement con- Universidade Federal de Uberlândia. Uberlândia
trol. The diagnosis of definite ALS is based on clinical or MG, Brazil Background: Amyotrophic lateral sclerosis (ALS) is a
electrophysiological evidence of lesions in the two afore Instituição ResMed. Uberlândia MG, Brazil rare and fatal neurodegenerative disorder with variable
mentioned motor segments. The presence of hypointensity Centro Universitário do Triângulo. Uberlândia MG, incidence and prevalence worldwide. However, clini-
in the motor cortex in the magnetic susceptibility sequence Brazil cal-epidemiological studies on ALS are scarce in Brazil.
(SWI) may indicate injury to the UMN. The objective was bueno.mateus22@gmail.com Objective: This study investigated whether ALS incidence
to verify the presence of hypointensity in the motor cor- had uniform spatial distribution in a population-based
tex in SWI of brain magnetic resonance imaging (MRI) in cluster analysis in Rio Grande do Norte state (Brazil), from
Background: Amyotrophic lateral sclerosis (ALS) is a rare
patients diagnosed with ALS, its correlation with clinical 2005 to 2018. Methods: New cases of ALS were identified
neurodegenerative disease that invariably results in sig-
characteristics and also the association with corticospi- in a database of the ALS multidisciplinary care centre of
nificant ventilatory impairment. In this sense, respiratory
nal tract (CST) radiologic findings in the magnetization the Onofre Lopes University Hospital in Natal (Rio Grande
function monitoring is essential, including the early indi-
sequence transfer (MT) of the same patients. This is a do Norte, Brazil). Approaches were based on incidence
cation of noninvasive ventilation (NIV), although there is
descriptive study that evaluated data from 20 patients with (empirical Bayes estimator and Moran’s I analysis) and
still no consensus on the ideal moment of its indication
ALS and 20 control subjects. The exams were performed cluster analyses (Moran scatter plot and spatial cor-
or how to promote satisfactory adherence and adapta-
at the same imaging center and analyzed by the same relogram). Results: A total of 177 patients (59% males)
tion during its use. Objective: To describe the effects of
radiologist with experience in neuroimaging. Clinical participated in the study; mean age of ALS onset was
NIV telemonitoring in patients with ALS followed up in
data were collected from medical records. Statistical 57 years. Mean annual incidence of ALS was 0.3769 per
a tertiary Hospital in Brazil. Methods: NIV was indicated
analysis was performed using Bioestat 5.0 software. The 100,000 inhabitants (95% confidence interval of 0.0889),
for ALS patients with forced vital capacity (FVC) lower
statistical significance of the variables was verified using higher in males than in females (0.4516 per 100,000 vs.
than 70% (late indication) or with FVC higher than 70%
the T test and the linear regression test, with alpha levels 0.3044 per 100,000). According to spatial statistics, patients
(early indication). The following variables were observed
of 0.01 and 0.05, respectively. Clinical variables did not were homogeneously distributed throughout the studied
during 9 months of telemonitoring: hours of use, leak,
statistically influence the presence of hypointensity in the area. Conclusion: A low estimate was observed com-
apnea hypopnea index (AHI), respiratory rate, minute
motor cortex of ALS patients, however in control group pared with other populations. Results did not indicate
volume, and trigger. Results: 22 patients with a definitive
there was a statistical significance of older age in the pres- areas of increased risk or significant spatial geographic
diagnosis of ALS were included, with a predominance
ence of hypointensity. When comparing the presence of dependence, suggesting a random ALS incidence in Rio
of males (68%; 15/22) with a mean age of 60.8 (±9.25)
hypointensity in ALS patients and healthy individuals, Grande do Norte.
years. 40.9% (9/22) underwent endoscopic gastrostomy.
this finding occurred especially in ALS subjects. There was 22.2% (5/22) had FVC over 70. 22.2% (5/22) had a fall of
also a statistical influence of the CST hyperintensity of the more than 10% between sitting and lying position, none
MT on the presence of hypointensity in the motor cortex Doenças do Neurônio Motor – Esclerose Lateral
had satO2 less than 93.8%. The ALS Functional Rating Amiotrófica
in SWI. It was concluded that the presence of hypointen- Scale (ALS-FRS-S) had a mean of 22.38 at inclusion and
sity in the motor cortex of ALS patients is not influenced 24.5 at month 9. In the first month of telemonitoring, we
by clinical variables despite being more frequent in ALS observed: 3.4 (±2.0) hours of daily compliance, AHI of 6.7
patients than in healthy individuals. Age can influence (±2.2) events/hour, leak of 3.15 (±14.4), RR of 25.5 (±5.7).
the presence of hypointensity in healthy individuals, 56.25% had poor adherence, defined as usage time of less
but in the ALS group there was no such relationship, than 4 hours. Comparing the first and ninth month of
which may represent neurodegeneration caused by the telemonitoring, we observed a reduction in the follow-
disease itself. Some patients with hypointensity at SWI ing parameters: AHI by 11%, leakage by 74%, RR by 21%,
in the cortex also presented hyperintensity in the CST MV by 25% and firing by 38%. In this period, there was an
at MT, finding that suggest a possible to use the SWI as increase of 117% in the time of daily use. Conclusion: The
another tool for detecting lesions in the UMN and thus increasing use of new technologies has made it possible
help in ALS diagnosis. to offer better quality in the performance of health care
activities. In this sense, telemonitoring is a tool capable
of providing information about diagnosis, prescription
Doenças do Neurônio Motor – Esclerose Lateral
and treatment that contributes to an increasingly ear-
Amiotrófica
lier indication for NIV. In addition, it makes it possible
to improve care, allowing the identification of problems
and complications not observed in periodic evaluations
in determining appropriate solutions and interventions.

Doenças do Neurônio Motor – Esclerose Lateral


Amiotrófica

243
XXX Congresso Brasileiro de Neurologia

TL 1106132 TL 1106185 TL 1106204

AMYOTROPHIC LATERAL SCLEROSIS TYPE UNVEILING CONFLICTING OPINIONS ABOUT IS COGNITIVE RESERVE A PREDICTOR OF
8 HAS LESS COGNITIVE DEFICITS AND AUTONOMY, END-OF-LIFE DECISIONS AND THE CLINICAL OUTCOME OF AMYOTROPHIC
DISTINCT BEHAVIOURAL PROFILE THAN THE MEDICAL MANAGEMENT OF AMYOTROPHIC LATERAL SCLEROSIS? A LONGITUDINAL
SPORADIC FORM: A COMPARATIVE STUDY LATERAL SCLEROSIS (ALS): RESULTS FROM STUDY
A SURVEY IN 3 CITIES FROM THE STATE OF
Cássia de Alcântara, Marcelo Maroco Cruzeiro, Émerson Costa, Mariana Asmar Alencar, Caroline
Marcondes Cavalcante França Jr, Mariana Asmar CEARÁ Martins de Araújo, Cássia de Alcântara, Leonardo
Alencar, Antônio Jaeger, Caroline Martins de Araújo, José Lopes Tabatinga Neto, Guilherme Nobre Cruz de Souza
Natália Araújo Sundfeld da Gama, Sarah Teixeira Nogueira, Luciana Ferreira Xavier, Lucas Eduardo
Camargos, Leonardo Cruz de Souza Universidade Federal de Minas Gerais. Belo
Lucena Cardoso, Marina Vasconcelos Sampaio, Horizonte MG, Brazil
Universidade Federal de Minas Gerais. Belo Ana Flávia Café, Renato Evando Moreira Filho, José
Horizonte MG, Brazil Marcelino Aragão Fernandes, Francisco Marcos leocruzsouza@hotmail.com
Universidade Federal de Juiz de Fora. Juiz de Fora Bezerra da Cunha, Francisco de Assis Aquino
MG, Brazil Gondim Background: Amyotrophic Lateral Sclerosis (ALS) is a
Universidade Estadual de Campinas. Campinas SP, neurodegenerative disease with motor neuron involve-
Universidade Federal do Ceará. Fortaleza CE, Brazil ment and non-motor symptoms, such as cognitive and
Brazil Centro Universitário Christus – Unichristus. behavioral deficits. It is unclear whether cognitive reserve
leocruzsouza@hotmail.com Fortaleza CE, Brazil has a protective factor in the rate of functional decline in
Universidade Regional do Cariri, Juazeiro do Norte patients with sporadic ALS (sALS). Objective: To inves-
Objective: To compare the cognitive performance and the CE, Brazil tigate the effect of cognitive reserve on the clinical out-
behavioural profile of patients with sporadic Amyotrophic zz.neto@hotmail.com come of sALS patients. Methods: This longitudinal study
Lateral Sclerosis (sALS) and patients with ALS type 8 included patients with the diagnosis of probable or definite
(ALS8), a familial form of ALS caused by mutations on Background: ALS is an irreversible neurodegenerative ALS according to Awaji criteria, between March/2018 and
VAPB gene. Methods: We included 29 symptomatic ALS8 disorder. End-of-life decisions vary according to cultural March/2020. Patients underwent regular clinical assess-
patients (17 men; median age = 49 years-old), 20 sALS perspectives about the limits of medical treatment. In ments, including the ALS Functional Rating Scale-Revised
(12 men; median age = 55 years-old) and 30 healthy con- Brazil, assisted suicide and euthanasia are illegal and the (ALSFRS-R). Patients were evaluated at the baseline, 6
trols (16 men; median age = 50 years-old), matched for boundaries of patient’s autonomy for decisions are not months, 12 months and 18 months after inclusion. We
sex, age, and educational level. Participants underwent precise. Objective: To evaluate the opinions of the edu- considered years of education (schooling) as a proxy
a neuropsychological assessment focused on executive cated general population about different medical decisions of cognitive reserve. Correlation and linear regression
functions, visual memory, and facial emotion recognition. related to management of ALS patients. Method: Presential analysis were performed to test the association between
Behavioural and psychiatric symptoms were investigated written opinion survey conducted with unidentified vol- schooling and ALSFRS decline. Results: We included 55
with the Hospital Anxiety and Depression Scale and unteers (minimum education: complete High school) individuals (31 male) with sALS (mean age=55.3 years,
the Cambridge Behavioural Inventory. Results: Clinical after signing informed consent with demographic data mean schooling =7.16 years and median disease duration
groups (sALS and ALS8) had lower global cognitive effi- collection, pre-test teaching about the subject and 4 case = 2.1 years). Eighteen patients died during the follow-up.
ciency than controls, and also impaired performance in simulations. Results: 510 volunteers, age 28.9+12.6 years The mean (±SD, number of patients) for ALSFRS-R scores
cognitive flexibility and inhibitory control. Verbal fluency (range 18-89), 50.7% female, 18.5% married. 45.4% were were, respectively: 31.22 (±10.7, 55), 28.2 (±11, 48), 24.8
deficits were not found in ALS8 patients, but they seem to for-, 35.7% against euthanasia. 71.8% were for-, 11.5% (±10,4, 41) and 22.2 (±10.1, 37), at baseline, 6, 12 and
have impairments in working memory subcomponents. against orthothanasia. 15.2 % were for-, 63.8% against dys- 18 months. Multivariate analyses including ALSFRS-R
On the psychiatric and behavioural aspects, apathy was thanasia. In situation 1 (ALS patient requested the medi- variation, schooling, disease duration, age and spinal/
shown to be frequent in both clinical groups. ALS8 patients cal team not to undergo gastrostomy that would increase bulbar onset, found that disease duration was positively
presented more eating changes and more stereotypical life expectancy from 3 to 36 months): 17.1% considered correlated with ALSFRS-R decline (B=0.73, p<0.05). The
behaviours. Conclusion: Overall, sALS and ALS8 had sim- this suicide, 64.1% orthothanasia, 7.1% euthanasia done other variables did not show association with disease pro-
ilar deficits in most cognitive domains, but ALS8 patients by family and 2.6% murder by either physician/family. If gression. Conclusion: In this series, schooling was not a
exhibit milder degree of executive dysfunction than sALS, the ALS patient was restricted to a wheelchair (situation predictor of clinical decline in sALS. More studies, with
besides distinctive pattern of behavioural changes. ALS8 2), only 5.9% considered refusal suicide but 5.5 and 5.7 larger samples, are warranted to investigate the influence
has a specific cognitive-behavioural profile, which should considered murder by family or physician, 5.9% medical of cognitive reserve in the clinical outcome of ALS patients.
be considered in the clinical care of patients. malpractice. However, if the same patient suffered from
severe depression (under treatment) and required gas-
Doenças do Neurônio Motor – Esclerose Lateral
trostomy and tracheostomy but declined (situation 3):
Doenças do Neurônio Motor – Esclerose Lateral Amiotrófica
47.2% considered this suicide, 53.8% orthothanasia, 18.4%
Amiotrófica
euthanasia, 9.1 and 5.8% murder by family or physician,
20.7% failure to provide adequate medical assistance,
12.9% medical malpractice. In situation 4, a 40-year-
old patient who progressed to “vegetative state” after
cardiac arrest had previously informed that she would
refuse CPR/aggressive treatments if severely disabled.
Following her wishes, her family refused to allow hemo-
dialysis. The patient died. 6.9% considered this suicide,
74.3% considered orthothanasia, 19.3% euthanasia, 14.1%
considered failure to provide adequate medical assistance.
Conclusion: In this educated general population from 3
cities of Ceará (Fortaleza, Crajubar, Sobral), after prior
teaching, there is great confusion about physician’s role,
patient’s autonomy/rights, euthanasia and end-of-life
decisions. A large % of respondents considered accept-
ing patient’s autonomy the same as allowance of suicide,
euthanasia, murder or malpractice.

Doenças do Neurônio Motor – Esclerose Lateral


Amiotrófica

244
XXX Congresso Brasileiro de Neurologia

TL 1106263 TL 1106384 TL 1106426

CLINICAL DECISION SUPPORT SYSTEM PROPOSAL OF AN INTEROPERABLE THE PROTOCOL AND THE IMPLEMENTATION
APPLIED TO AMYOTROPHIC LATERAL ELECTRONIC HEALTH RECORD FOR THE STEPS OF THE BRAZILIAN AMYOTROPHIC
SCLEROSIS PROGNOSIS MULTIDISCIPLINARY FOLLOW-UP OF LATERAL SCLEROSIS REGISTRY
PATIENTS WITH AMYOTROPHIC LATERAL
Fabiano Papaiz, Mário Emílio Dourado, Ricardo Emanuela Coriolano Fidelix, Igor Braga Farias,
Alexsandro de Medeiros Valentim, Antonio Higor SCLEROSIS Daniele Montenegro da Silva Barros,Danilo Alves
Freire de Morais, Anna Paula Paranhos Miranda Ingridy Marina Pierre Barbalho, Hebert Falcão Pinto Nagem, Ricardo Alexsandro de Medeiros
Covaleski, Marcela Câmara Machado Costa, Isaac Sales, Aleika Lwiza Alves Fonsêca, Guilherme Pablo Valentim, Mário Emílio Teixeira Dourado Júnior
Holanda Mendes Maia, Francisco Marcos Bezerra de Santana Maciel, Felipe Ricardo dos Santos
da Cunha, Daniele Montenegro da Silva Barros, Joel Universidade Federal do Rio Grande do Norte. Natal
Fernandes, Danilo Alves Pinto Nagem, Mário Emílio RN, Brazil
Perdiz Arrais Teixeira Dourado Júnior, Ricardo Alexsandro de
Universidade Federal do Rio Grande do Norte. Natal Medeiros Valentim efidelix@gmail.com
RN, Brazil Universidade Federal do Rio Grande do Norte. Natal
Hospital das Clínicas da Universidade Federal de Background: ALS is a rare disease. Recent studies have
RN, Brazil reported the incidence of ALS between 0.6 and 3.8 per
Pernambuco. Recife PE, Brazil
Fundação Bahiana para Desenvolvimento das ingridy.marina@lais.huol.ufrn.br 100,000 person-years, with possible geographic variation.
Ciências. Salvador BA, Brazil In Brazil, its incidence and prevalence and the distribution
Hospital Geral de Fortaleza. Fortaleza CE, Brazil Amyotrophic Lateral Sclerosis (ALS) is characterized as of cases in the national territory are unknown. In recent
Hospital Universitário Walter Cantídio da a rare neurodegenerative disease with an unknown cure. years, the public health importance of rare diseases has
Universidade Federal do Cariri. Barbalha CE, Brazil Due to its irreversible progression and complexity, it is been recognized. The use of a population-based registry,
CISUC / DEI / Universidade de Coimbra, Portugal essential that patients diagnosed with ALS are followed allowing the collection of information on all individuals
up by a multidisciplinary team. By involving different diagnosed with ALS, allows a reliable estimate of the inci-
fabianopapaiz@gmail.com professionals in the health care of a patient with ALS, dence and prevalence of the disease, in addition to other
it is essential to consider the use of a platform capable data that can be analyzed. Objectives: Our mission is to
Background: Amyotrophic Lateral Sclerosis (ALS) is a of collecting and sharing information by each specialty, describe the study protocol and the steps to implement the
rare and incurable disease that affects the neurons of building a concise and cohesive clinical history to assist ALS registry in Brazil. Methods: The brazilian ALS registry
the human motor system. The communication between the multidisciplinary team in decision-making. It is in this began in 2022, with a retrospective and prospective col-
the brain and muscles is gradually interrupted, leading perspective that electronic health records arise. This tool lection of cases, as a multicenter observational study of a
patients to paralysis and death. The average life expec- stores processable information about patients’ health, population-based cohort. A web platform was developed
tancy is 3-5 years after symptoms onset, and the world- allowing the sharing of this information to the multidis- by the Laboratory of Technological Innovation in Health
wide incidence is about 1.9 cases per 100,000 individuals ciplinary team safely and efficiently. Due to the evolution (LAIS) to collect data on new and monitored cases of ALS
per year. ALS is clinically heterogeneous, with different of technological resources, these tools have more func- in different regions of Brazil. Demographic variables such
symptoms and disease progression among its patients, tionalities and, consequently, more significant importance as age at onset, ethnicity, sex, and family history of ALS
making it challenging to perform prognoses (e.g., survival in the health area. In addition to containing information and FTD will be collected. This data can be added by the
time and disease progression). Research using Machine that allows communication between the entire medical physician (neurologist or neurophysiologist) or by the
Learning (ML) algorithms has been applied to improve team, the electronic health record can provide continu- patient (self-registry). Six months later, the physician
the prognosis of diseases. ML could extract information ity of clinical care, gathering important information that, will be invited to update the patients’ clinical situation.
from the training data, transform it into knowledge, and when analyzed, can contribute to the diagnosis and con- All participating centers have authorization from the cor-
use it to solve different categories of problems. Thus, it is trol of the evolution of diseases and adequate treatment. responding research ethics committee. Results: The ALS
crucial to collect patient data to perform relevant studies Given this context, the present work proposes develop- registry was successfully implemented. Different centers
and create ML solutions to help physicians in their daily ing a platform for collecting and storing structured data throughout the brazilian territory have already contributed
work, e.g., developing a Clinical Decision Support System related to patients with ALS. The main objective is to to the registration platform and data from ALS patients
(CDSS). Objectives: The primary objective is to develop present a blockchain-based architecture for developing have already been collected. Os dados serão analisados
a CDSS to assist brazilian health workers in their tasks a specific electronic health record for ALS patients. Given no final de 2022. Conclusion: We reached consensus on
related to ALS prognosis. The proposed CDSS should the need for information exchange, this architecture will a central dataset and data sharing processes established
provide helpful information about prognosis prediction, be interoperable, following the Health Level 7 (HL7) stan- with various partners to address the need for high quality
including the survival time, the disease stage, and the dards with the Fast Healthcare Interoperability Resources information on the epidemiology of ALS in Brazil. In this
moment when respiratory or nutritional support will be (FHIR) specification, in accordance with the standards of way, we can plan health planning interventions, support
needed. Consequently, we intend to create a brazilian the National Health Data Network (RNDS, its Portuguese decision-making processes regarding the diagnosis and
ALS Prognosis Database to support future research in this acronym) and adherent to the General Data Protection evolution of the disease.
field. Methods: This study is clinical, prospective, obser- Law. It is expected that this architecture will provide
vational, and longitudinal. Data will be collected from greater integration between the professionals who make
patients followed by brazilian Research Centers, which Doenças do Neurônio Motor – Esclerose Lateral
up the multidisciplinary team, guaranteeing security,
include demographics, clinical, laboratory, functional, Amiotrófica
privacy, and confidentiality in managing information in
and respiratory biomarkers. The data collected will be the electronic health record for ALS. Once developed,
processed by ML algorithms to learn and create models this electronic health record will store essential data that
to provide the target predictions defined. Results: A CDSS can be used to plan health interventions, support deci-
will be developed to allow the health workers to register sion-making processes, and follow up in more detail on
information and obtain the prognosis predictions about the evolution of ALS in the patients.
their patients. The system will comprise the Electronic
Medical Record and the Prognosis Dashboard modules.
Conclusion: This research aims to develop a CDSS to help Doenças do Neurônio Motor – Esclerose Lateral
physicians in their ALS clinical practice. This system will Amiotrófica
allow health workers to register and monitor their ALS
patients. We expect that the proposed CDSS can provide
valuable information to the health workers about prog-
nosis predictions and that it could represent a valuable
tool for knowledge dissemination among all interested
health workers and researchers in this area.

Doenças do Neurônio Motor – Esclerose Lateral


Amiotrófica

245
XXX Congresso Brasileiro de Neurologia

TL 1105009 TL 1105031 TL 1105048

THE COMMUNICATION OF THE DIAGNOSIS MYOTONIC DYSTROPHY TYPE 1: A DISEASE NEUROIMAGING, GENETIC AND CLINICAL
OF SPINAL MUSCULAR ATROPHY IN WITH MULTIPLES OPHTHALMOLOGIC CORRELATIONS IN LAMA2- CONGENITAL
ENDOGAMOUS X NON-ENDOGAMOUS FINDINGS MUSCULAR DYSTROPHY
REGIONS
Stefanie Hitomi de Nishi Lee, Ellen Yukie Fukuda Clara Gontijo Camelo, Mariana Cunha Artilheiro,
Isabella Araujo Mota Fernandes, Renata Oliveira Chiovatto, Priscila Alves Nascimento, Maria Beatriz Cristiane Araújo Martins Moreno, Sueli Fazio
Almeida Menezes, Guilhermina Rego Santos Elias Daher, Fabiana Jallad Sallum, Juliana Ferraciolli, André Macedo Serafim Silva, Tatiana
Yumi Kinjo, Maiara Kanematsu, David Feder, Roseli Ribeiro Fernandes, Leandro Tavares Lucato, Antônio
iversidade do Porto. João Pessoa PB, Brazil Corazzini, Alzira Alves de Siqueira Carvalho José Rocha, Umbertina Conti Reed, Edmar Zanoteli
UFRN. João Pessoa PB, Brazil
Universidade do Porto – Portugal Faculdade de Medicina do ABC Universidade de São Paulo. São Paulo SP, Brazil
Universidade Federal de São Paulo. São Paulo SP,
isabella_mota@yahoo.com.br stefanielee14@gmail.com Brazil

Background: The communication of the diagnosis of a Background: Several ocular abnormalities have been docu- claragc@gmail.com
rare, genetic and neurodegenerative disease may leave mented to occur in patients with myotonic dystrophy type 1.
sequelae in the lives of those involved. Objective: To Objective: To evaluate the frequency of these abnormal- Background: LAMA2-Congenital muscular dystrophy
compare the communication of the diagnosis of spinal ities and to alert the importance of multidisciplinarity for (CMD) is the most common cause of CMD. LAMA2 gene
muscular atrophy (SMA) in endogamous x non-endoga- better patient management. Methods: All participants codifies a protein named laminin alpha-2 (merosin),
mous regions. Methods: A cross-sectional study obtained underwent a thorough ophthalmologic examination, which binds to alpha-dystroglycan and is expressed in
by means of a questionnaire containing the Impact of including visual acuity assessment, slit-lamp biomicros- skeletal muscle fibers, and also in the brain. Objective:
Event Scale–Revised, sent to volunteers with SMA or their copy, ocular motility, dynamic refraction, and fundus Characterize motor, genetic and neuroimaging changes,
families. Results: The sample consisted of 100 volunteers, examination in addition to optical coherence tomogra- and in a large cohort of CMD-LAMA2. Methods: Patients
83% female, and aged 36.65 (±10,19) years old and homo- phy, a complementary exam that is fundamental for ret- were divided according to motor severity, and, for each
geneous for the variables of sex, age, color, education, inal evaluation, since many of these patients may have group, comparisons were made among motor, genetic
religion and SMA subtype (1,2,3 and 4) between groups. low visual acuity unrelated only to the presence of cata- and central nervous system findings. Results: The study
The endogamous regions, despite being economically racts. Results: We evaluated 68 eyes of 34 patients aged cohort consisted of 56 patients. (1)Three patients (5,3%)
less favored, showed more satisfaction and inclusion in 3 to 71 years (mean 42.7 ± 16.9), 17 of which were men. had the severe congenital form and were not able to sit
medical care and health services, with less trauma and IOP was reduced in 20 eyes (29,4%). We found cataract without support.(2) Forty-three (76,8%) patients had the
signs of post-traumatic stress disorder related to the time or positivity for surgery in 31 eyes (45,6%) and ptosis in classic congenital form and were able to sit unassisted.
of SMA diagnosis. Conclusion: the positive impact of 40 (58,8%). Myopia in 36 eyes (52,9 %), hyperopia in 16 (3) Five patients (8,9%) had the mild congenital form and
AME communication in endogamous regions resulted eyes (23,5%), ocular motility limitations in 14 eyes (20,6 were able to walk; among them, two patients who had
in less emotional damage, favoring free and informed %), blepharitis in 12 eyes (17,6 %), epiretinal membrane acquired independent gait at four and five years of age
decision-making. in 24 eyes (35,3 %) and pigmentary retinopathy in 13 eyes became wheelchair dependent at age of eight years. (4)
(19,1 %). Conclusion: Cataract and ptosis are frequent in Five patients (8,9%) had the limb girdle muscular dystro-
DM1 and associated to motor dysfunction. An expressive phy form and acquired gait within the expected age. 40
Doenças Neuromusculares reduction of intraocular pressure was observed in our different mutations were found, 26 variants were novel.
patients as well. A finding described in the literature that All three patients with the severe congenital form had null
was not found in our patients was lamellar macular holes variants in both alleles. 36 (83,7 %) patients with the clas-
and foveal schisis. However, we observed an important sic congenital form presented null variants in both alleles
and relatively common alteration that was an irregular- and 7 (16,3 %) had one null variant with one missense or
ity and pigmentary changes in the choriocapillary/RPE inframe variant. Among 5 patients with mild congenital
complex which could only be analyzed and visualized form, 3 had one missense variant plus one null or CNV
with the advent of the spectral domain optical coherence variant, and two had two null variants. The patients with
tomography technology. We report an increased preva- 2 null variants were those who lost ambulation. Among
lence of epiretinal membrane in the myotonic dystrophy patients able to walk, there was a higher frequency of
type 1 group. Therefore, it is important to emphasize the missense variants than null variants (p<0.05). Among
importance of the inclusion of different specialties in mul- the three patients with severe congenital form, one had
tisystem diseases like DM1, once that brings benefits to associated cortical malformation. Cortical malforma-
both patients, in their diagnosis and follow-up, as about tions were also found in 8 (18,6%) patients with the clas-
the quality of life and prognosis, once again ophthalmo- sic congenital form. Among patients who were able to
logical changes above found have treatment. acquire gait none had cortical malformation. All patients
who presented cortical malformations had null variants
in both alleles. Cortical malformations were: occipital
Doenças Neuromusculares polymicrogyria, temporal polymicrogyria, occipital lis-
sencephaly-pachygyria and white matter changes. They
were more frequently seen in patients with mutations
located in the LG domains, region that binds Laminin-211
to alpha dystroglycan (p<0.05). Conclusion: The most
severe motor presentations are related to cortical mal-
formations and null variants, and to variants present in
the LG domain of LAMA2 gene.

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XXX Congresso Brasileiro de Neurologia

TL 1105049 TL 1105176 TL 1105178

PSEUDOMETABOLIC SYMPTOMS PHENOTIPICAL PATTERNS OF CONGENITAL MOLECULAR ANALYSIS OF PATIENTS WITH


IN CONGENITAL AND DYSTROPHIC MYASTHENIC SYNDROMES CLINICAL SUSPICION OF ACUTE HEPATIC
MYOPATHIES PORPHYRIA FROM A TERTIARY CENTER IN
Eduardo de Paula Estephan, Antonio Alberto
Zambon, Carlos Otto Heise, Cristiane Araújo Martins BRAZIL
Clara Gontijo Camelo, Alulin Tácio Quadros Monteiro
Fonseca, Cristiane Araújo Martins Moreno, Karlla Moreno, André Macedo Serafim Silva, Umbertina Michelle Abdo Paiva, Andre Macedo Serafim Silva,
Danielle Ferreira Lima, André Macedo Serafim Conti Reed, João Aris Kouyoumdjian, Maria da Rodrigo de Holanda Mendonça, José Pedro Soares
Silva, Rodrigo Holanda Mendonça, Marco Antônio Penha Ananias Morita, Edmar Zanoteli Baima, Beatriz Carneiro Gondim Silva, Cristiane de
Veloso Albuquerque, Umbertina Conti Reed, Edmar Araujo Martins Moreno, Antonio Alberto Zambon,
Faculdade de Medicina da Universidade de São
Zanoteli Angelina Maria Martins Lino, Edmar Zanoteli
Paulo. São Paulo SP, Brazil
Universidade de São Paulo. São Paulo SP, Brazil Faculdade de Medicina de Ribeirão Preto. Ribeirão Hospital das Clínicas da Faculdade de Medicina da
Universidade Federal de São Paulo. São Paulo SP, Preto SP, Brazil Universidade de São Paulo. São Paulo SP, Brazil
Brazil
eduardo.estephan@gmail.com michelle.abdo@hc.fm.usp.br
claragc@gmail.com
Congenital myasthenic syndromes (CMS) comprise a Background: Acute hepatic porphyrias (AHP) consist in
Background: Metabolic myopathies comprise a diverse rare group of genetic diseases with phenotype that can acute intermittent porphyria (AIP), hereditary copropor-
group of muscle disorders that result in impaired energy be difficult to be distinguished between their selves. In phyria (HCP), variegate porphyria (VP) and porphyria
production due to defects in glycogen, lipid, or mito- previous study we found that a pure ocular syndrome due to delta-aminolevulinic acid deficiency dehydratase
chondrial metabolism. Most of the patients have exer- is not a usual phenotype of CMS, and that usually the (ALAD). Data from USA indicate that the most common
cise intolerance, pain, muscle cramp, myoglobinuria, phenotype is predominantly limb weakness or predom- is AIP, followed by VP and HCP. Epidemiological data
variation in CK levels, with or without progressive muscle inantly axial weakness (impairment of bulbar, neck and/ from Brazil are scarce due to lack of available specific
weakness. Structural myopathies can also present with or respiratory muscles). This study verifies which genes biochemical exams and genetic studies. Objectives: To
symptoms triggered by exercise, and thus mimic the are related to each pattern of CMS phenotype in a large describe epidemiological and genetic profile of patients
metabolic myopathies, being referred to as the pseudo- CMS cohort with genetically confirmed cases. The clini- with AHP in a tertiary center in Brazil. Methods: We
metabolic myopathies. To date, there is only few articles cal and molecular characteristics of a large cohort of 92 included patients based on clinical history: recurrent
about pseudometabolic symptoms in structural myopa- patients with suspected CMS were analyzed. Data from abdominal pain, altered colored urine and peripheral
thies, and the actual frequency and genetic correlations clinical evaluations and ancillary exams were collected or central nervous system manifestation. We reviewed
is not yet well established in large cohorts. Objective: and blood samples were submitted to new generation medical records and evaluated all patients. We performed
identify and genetically characterize patients with pseu- sequencing. After molecular results, 66 patients were con- a next generation sequencing panel using a commercial
dometabolic symptoms and to identify the frequency of sidered confirmed cases of CMS. These confirmed cases kit. Results: We identified 34 suspected patients from 26
this symptoms amongst patients with RYR1-myopathies were stratified into three categories: predominantly limb different families. Age varied from 18 to 80, median age
Methods: Observational study conducted from 2020 weakness; predominantly axial weakness and generalized of 43 years. Majority of patients were female (25/34).
to April 2022 at Hospital das Clínicas da Faculdade de weakness. CHRNE was the defective gene in 36 patients, 21 patients identified as being white and 13 patients as
Medicina da Universidade de São Paulo. São Paulo SP, and was followed by DOK7 (8), RAPSN (5), COL13A1 (4), brown. A total of 26 patients underwent genetic testing.
Brazil. Among 1310 patients evaluated, 30 (2,3%) had COLQ (4), GFPT1 (2), GMPPB (2), CHRNA1 (1 receptor AIP was diagnosed in seven patients from four families,
symptoms resembling metabolic myopathies. Inclusion deficiency, 1 slow channel) SCN4A (n=1), CHAT (n=1), VP was diagnosed in nine patients from five families. Two
criteria was the presence of at least one of the following MUSK (1). Comparison between gene related cases was patients had inconclusive tests and eight patients had a
four typical metabolic symptoms, being the most import- done with Fisher’s test. Twenty-nine cases were consid- negative panel. Regarding AIP, each family had a differ-
ant complaint: (1) exercise intolerance, (2) muscle pain, ered as predominantly limb weakness: 19 CHRNE (52% ent pathogenic mutation: 973C>T (p.Arg325*); c518G>A
(3) muscle cramps, (4) myoglobinuria, with or without of CHRNE cases), four DOK7 (50%), two RAPSN (40%), (p.Arg173Gln); deletion of exon 1; and a splice donor vari-
muscle weakness. Exclusion criteria: patients whose most two GFPT1 (100%), one GMPPB (50%) and one COLQ ant (c.912+2T>C). For VP, four families shared the same
important complaint/symptom was weakness, patients (25%). Predominantly axial weakness was considered to missense variant in exon 6, c503G>A (p.Arg168His), and
with inconclusive genetic test or genetic test confirming a be the phenotypical pattern in 14 cases: four DOK7 (50% one patient had a deletion in exon 5-6. Five out of nine
metabolic myopathy. Results: From 30 selected patients, of DOK7 cases), four COL13A1 (100%), two RAPSN (40%), patients with VP identified as brown, and four out of seven
twelve (40%) had genetic confirmation of structural myop- two COLQ (50%), one MUSK and one SCN4A. 23 cases with AIP, as white. We also identified seven cases of patho-
athy. Four patients had RYR1-myopathy (33,3%), three were considered as generalized weakness: 17 CHRNE (48% genic intronic variant for erythropoietic porphyria (c.315-
patients had Dystrophinopathy (25%), two siblings had of CHRNE cases), one RAPSN (20%), one COLQ (25%), 1 48T>C), in six of them associated with AHP. Conclusion:
Gamma-sarcoglycanopathy (16,6%), and the other three GMPPB (50%), 1 CHRNA1 (receptor deficiency), 1 slow Our data suggest that VP is more common in our popu-
patients had FKRP, ANO5 and COL6-related myopathy, channel, 1 CHAT. CHRNE cases were significantly not lation than previously reported. This may be related to
respectively. All patients had exercise intolerance and related to predominant axial weakness (p=0.0001), and our country’s history and colonization. VP originated in
muscle pain, five (41,7%) patients had muscle cramps and DOK7 cases were not related to generalized weakness South Africa, whereas AIP originated in Scandinavia and
four (33,3%) had myoglobinuria Conclusion: Our cohort (p= 0.0430). COL13A1 cases were related to predomi- Northern Europe. More studies are necessary to confirm
highlight the need to consider structural myopathies in nant axial weakness (p=0.0014). In a cohort larger then this epidemiological pattern.
the differential diagnosis of patients who present with our previous, we still find pure ocular symptoms not a
complaints suggestive of a metabolic myopathy, partic- phenotype typical of CMS, once no CMS cases had this
ularly when metabolic studies have been unrevealing. phenotype. Predominant axial weakness seems to be the Doenças Neuromusculares
It also highlight the need to consider not only genes of less frequent of the three patterns verified, and is related
muscular dystrophy, but genes of structural myopathy, to COL13A1 cases, but it is not expected as a CHRNE
specially RYR1-related myopathy. phenotype. Similarly, generalized weakness is probably
a rare presentation of DOK7 CMS.

Doenças Neuromusculares
Doenças Neuromusculares

247
XXX Congresso Brasileiro de Neurologia

TL 1105206 TL 1105230 TL 1105239

MUSCLE ULTRASOUND CHANGES CLINICAL-EPIDEMIOLOGICAL PROFILE AND GENOTYPIC AND PHENOTYPIC SPECTRUM
CORRELATE WITH RESPIRATORY MUSCLES FACTORS ASSOCIATED WITH PROGNOSIS IN OF FIG4 VARIANTS IN CHARCOT MARIE
INVOLVEMENT AND FUNCTIONAL PATIENTS WITH MYASTHENIA GRAVIS IN A TOOTH DISEASE
IMPAIRMENT IN SPINAL MUSCULAR REFERENCE HOSPITAL IN NORTHEASTERN
Eduardo Boiteux Uchôa Cavalcanti, Savana Camilla
ATROPHY BRAZIL Lima Santos, Pedro José Tomaselli, Wilson Marques
Ana Lucila Moreira, Rodrigo Holanda Mendonça, Lara de Menezes Andrade, Maria Lucia Brito Junior, Osvaldo José Moreira Nascimento
Graziela Jorge Polido, Marcos Castello Barbosa Ferreira, Lidiane Alves Macedo, Alvaro Jose Porto Rede SARAH – Unidade Lago Norte. Brasília DF,
Oliveira, Andre Macedo Serafim Silva, Edmar Moreira, Maria Iris de Moraes Machado Brazil
Zanoteli Rede SARAH – Unidade Brasília. Brasília DF, Brazil
Hospital da Restauração. Recife PE, Brazil
Hospital das Clínicas da Universidade de São Paulo. Hospital das Clínicas da Faculdade de Medicina
São Paulo SP, Brazil laradma@hotmail.com de Ribeirão Preto – Universidade de São Paulo.
Ribeirão Preto SP, Brazil
ana.lucila.moreira@hotmail.com Background: Myasthenia gravis (MG) is an acquired Faculdade de Medicina de Ribeirão Preto –
chronic autoimmune, neuromuscular disorder due to Universidade de São Paulo. Ribeirão Preto SP, Brazil
Background: Although new therapies modified the course autoantibodies formed against the relevant post-synaptic Universidade Federal Fluminense. Niterói RJ, Brazil
of Spinal Muscle Atrophy (SMA), these treatments are membrane proteins at the neuromuscular junction syn-
costly and clinical trials have been substantially based apsis. According to the Myasthenia Gravis Foundation eduardouchoarj@hotmail.com
on clinical assessments. Reliable techniques to evaluate of American Clinical Classification (MGFA), the severity
disease progression and therapy response could support varies from mild weakness limited to the ocular muscles Charcot Marie Tooth disease (CMT) type 4J is a rare
more robust clinical trials. Here we investigated ultra- to generalized limb or axial muscles and bulbar weakness. inherited peripheral neuropathy, affecting less the 1% of
sound patterns of muscle involvement in different types This classification is designed to identify subgroups of patients with CMT. It is caused by biallelic disease causing
of SMA and their correlation with functional status to patients who share distinct clinical features or severity of variants in FIG4 gene, with patients presenting a variable
determine the validity of this non-expensive, and easy- disease that may indicate different prognoses or responses phenotype and onset ranging from early childhood to
to-use assessment. Objective: We examined 41 patients to therapy. Objective: This study aims to identify potential adulthood. According to the literature, most individuals
with SMA (types 1 to 4) and 46 age-, and gender-matched clinical predictors for the prognosis of MG, comparing typically have both proximal and distal asymmetric mus-
healthy controls using B-mode ultrasound (whole muscle groups with mild-moderate disease (MGFA I to III) and cle weakness of the upper and lower limbs. In addition
method) for grey scale analysis (GSA), area (biceps bra- with severe disease (MGFA IV and V). Methods: Within to the CMT phenotype, pathogenic variants in FIG4 gene
chii and rectus femoris) and diaphragm thickening ratio. 88 patients followed at the Neuroimmunology Service have also been related to motor neuron disase, bilateral
Methods: Functional scales were applied to patients only. from January 2006 to December 2021, this retrospective temporooccipital polymicrogyria, and Yunis?Varon syn-
We analyzed ultrasound abnormalities in specific clinical study included 79 patients whose medical records were drome. Here, we describe the clinical, genetic, neuro-
subtypes and correlated findings with functional status. available. The diagnosis of MG was based on a combi- physiological and other ancillary data from 3 unrelated
Also, we analyzed if it’s possible to predict respiratory nation of clinical, and laboratory criteria, and electro- individuals with CMT4J evaluated at two referral centers
involvement using GSA. Results: Compared to controls, myography. Results: Regarding gender, 47 (59.5%) were for neuromuscular diseases. All patients have early-onset
patients had reduced muscle area and increased mean female, corresponding to an overall female-male ratio of disease, with symptoms beginning before age 5 years,
GSA with correlation with the SMA severity, and the GSA 1.46: 1. In the clinical presentation, generalized weakness and ENMG exam with a demyelinating pattern. One of
of their intercostal muscles differed more in relation to predominated (48.1%), followed by exclusively ocular the patients started to use a wheelchair as a mobility
the controls, as compared to differences in the diaphragm symptoms, (40.5%), and less frequently by the involve- aid at the age of 43 and the other 2 patients maintain an
histograms. Biceps brachii GSA was correlated with dia- ment of the bulbar muscles (11.4%). As for the myasthenic independent gait. Two patients who underwent brain
phragm and intercostals GSA (p=0.019 and 0.008), and crisis, 24 (20.4%) patients had at least one episode during MRI did not have significant radiological findings. One
linear regression showed that it could predict respiratory follow-up, and for 9 of them, this was the initial clinical of the patients had an abnormal a pulmonary function
muscle involvement (R=0.456 and 0.489 respectively). presentation. When comparing the group with MGFA test with FVC: 50% and FEV1: 54%, indicating a restrictive
Discussion: Muscles are affected on ultrasound in SMA classification ranging from I to III, to the group in classes pulmonary pattern. Spinal radiography evaluation showed
patients with correlation with SMA severity. Biceps brachii IV to V, it was shown that greater severity was associated mild scoliosis in two individuals. Genetic test revealed the
GSA can possibly be used to predict respiratory involve- with the bulbar form of MG presentation, and more fre- variant c.122T>C (p.Ile41Thr) in a homozygous state in
ment in SMA patients because it is correlated with dia- quent positivity of anti-AChR antibodies, plus the greater the FIG4 gene in all three patients. Despite being a rare
phragm and intercostal muscle involvement. frequency of thymoma and thymic hyperplasia. All these variant of CMT, patients with disease causing variants in
associations reached statistical significance. Conclusion: FIG4 usually have more severe forms, including potential
In summary, this study showed that initial presentation of respiratory involvement. Accurate genetic diagnosis is
Doenças Neuromusculares the bulbar form, positivity for Anti-AChR, and association very important for appropriate genetic and reproductive
with thymic alterations are prognostic factors for MG. counseling of patients.

Doenças Neuromusculares Doenças Neuromusculares

248
XXX Congresso Brasileiro de Neurologia

TL 1105312 TL 1105325 TL 1105443

CHARACTERIZATION OF THE RESPIRATORY PERIOPERATIVE NURSING CARE CAREGIVER OVERLOAD IN THE CONTEXT OF
PROFILE OF PATIENTS WITH SPINAL MEDIATING THE CARE OF MYASTHENIC RARE NEUROMUSCULAR DISEASES
MUSCULAR ATROPHY IN A TERCIARY PATIENTS SUBMITTED TO TIMECTOMY: AN
Clarissa de Araujo Davico, Patricia Gomes Pinheiro,
CENTER INTEGRATIVE REVIEW Mariana Ribeiro Pereira, Carolina Garcia Nuñez
Eduardo Vital de Carvalho, Rodrigo Holanda Lucas de Souza Oliveira, Illymack Canedo Ferreira Carrijo, Karina Lebeis Pires, André Bertola Vanzan
Mendonça, Graziela Jorge Polido, Edmar Zanoteli de Araujo Filho, Elisa Gutman Gouvea, Pedro Ignacio Ferraz
Montenegro de Almeida, Rayanne da Silva Souza,
Faculdade de Medicina da Universidade de São Pontifícia Universidade Católica de Campinas. Stephanie de Freitas Canelhas
Paulo. São Paulo SP, Brazil Campinas SP, Brazil
Casa Hunter. Rio de Janeiro RJ, Brazil
duvital1983@gmail.com lucasenfoncologia@gmail.com Universidade Federal do Estado do Rio de Janeiro.
Rio de Janeiro RJ, Brazil
Background: Spinal muscular atrophy (SMA) is a disease The pathology of Myasthenia gravis (MG) is of idio-
of motor neurons due to mutations in the SMN1 gene. pathic cause, resulting in alteration in the neuromus- patricia.pinheiro7@yahoo.com.br
Respiratory involvement is the main cause of death in cular junction by the presence of Anti-Acetylcholine
SMA and respiratory care should be introduced early in receptor (AChRs), preventing the physiological action Background: Rare neuromuscular diseases are chronic
disease course. Knowledge about the respiratory clinical of this chemical mediator resulting in several clinical degenerative, presenting, in some cases, a motor and/or
profile is necessary to ensure the appropriate ventilatory forms of this disease: neonatal MG, congenital MG, MG bulbar deficit. The patient may evolute with physical and
management in SMA patients. Objective: To present of drug origin and autoimmune MG. Objectives: This psychological impairment, thus becoming dependent on
the respiratory clinical profile of SMA patients followed study aimed to highlight the importance of perioper- a caregiver, which impacts the life of the patient and his
up at a tertiary center. Methods: Descriptive and retro- ative nursing care as a mediator in the qualification of companion. In view of this, the caregiver may present an
spective study carried out from July 2021 to June 2022. care to be provided in myasthenic patients submitted overload in the face of the new demands that arose with
Data was collected from medical records of respiratory to thimectomy. Methods: This is an integrative review the illness of their loved one. Objectives: The objective of
physiotherapy assessments, such as use of invasive and bibliographic study that resulted in an active search this study is to identify the presence of burden in caregivers
non-invasive ventilation support, forced vital capacity in the VHL Regional Portal and Scielo Research Portal of patients with rare neuromuscular diseases. Methods:
(FVC), peak cough flow (PCF), oxygen saturation, heart with selected titles between 2008 and 2021. Thirty pub- Cross-sectional and descriptive study, with selected rare
rate, chest deformity and scoliosis. Results: A total of 45 lications that met the proposed objectives were selected neuromuscular diseases. As inclusion criteria, having
SMA patients were included, 27 (60%) male and 18 (40%) and grouped, categorized, synthesized, and described in rare neuromuscular disease and over 18 years of age.
female. Mean age was 12.9±9.5 years, mean height was thematic axes according to the similarity of the subjects We excluded patients whose medical conditions made
1.39±0.26 m, and mean weight was 38.2±22.43 kg. Most treated. Results: In the analyzed articles, it was observed it impossible to understand or perform the tests (such
patiens (49%) were SMA type 2, 36% were type 3 and 14% that the patient who needs myasthenia presents general- as acute confusional state, aphasia, reduced level of
were type 1. Only 11% had recurrent respiratory infections, ized weakness and, in this condition, besides the ocular consciousness, dementia, and sensory deficits). The fol-
13% had symptoms of alveolar hypoventilation, 0.04% had muscles, there is an impairment of the muscles of the lowing clinical instruments were applied to caregivers of
a paradoxical pattern and 13% complained of dyspnea. limbs and the trunk, that may affect the muscles of the patients: form with sociodemographic factors; ZARIT scale
Three (6%) patients had a history of weight loss and 13 oropharynx and muscles involved with breathing, making (caregiver burden assessment) and FACIT (Functional
(29%) had dysphagia. Regarding the use of ventilation sup- the use of the Perioperative Nursing Care Systematization Assessment of Chronic Illness Therapy-Spiritual Well-
port, 14 (31%) use non-invasive and 4 (8%) use invasive. (PNCS) a valuable tool in the dynamization of nursing care Being/FACIT-Sp). The functional independence mea-
The mean FVC was 1.81±1.45 l, with 24% of patients with through the three phases of the surgical experience: the surement (FIM) and activities of daily living (BARTHEL)
severe restrictive ventilatory impairment, 6% with moder- immediate and mediate preoperative, intra- or transop- scales were applied to the patients, in addition to the
ate restrictive impairment, 11% with mild restriction and erative and immediate postoperative. Conclusion: It is PHQ-9 (Patient Health Questionnaire-9) to screen for
37% of the patients had no decrease in FVC. The mean concluded that the knowledge about MG aligned to PNCS major depressive episodes (MDE). Results: 43 caregiv-
PCF was 250.27±139.98 l/min. Mean oxygen saturation will corroborate with the delineation of actions involving ers were included in the study; 28 patients (65.11%) were
was 97±0.70%, heart rate was 94±15 beats per minute, the care planning to be directed to the patient, from clinical female and age was 42 ±18.4 years. ZARIT Scale was 14.0
respiratory rate was 20±5 breaths per minute and 55% of judgment supporting evidence of signs and symptoms. (9.2-22.5), being a mild classification to caregiver over-
the patients had chest deformities. Conclusion: Through load, and the total score of FACIT-Sp was 33 (28.0-37.0).
an evaluation of ventilatory clinical parameters, it was These results are correlated with caregivers of a group of
possible to classify and identify the degree respiratory Doenças Neuromusculares patients who have 77 (55.2-111.5) of measure of functional
involvement SMA patients. It is remarkable that severe independence, with modified dependency up to 25% and
ventilatory restriction is the most frequent finding. Such PHQ-9: 9 (5.0-13.0), with mild depression. However, this
information will be useful for planning the respiratory sample shows severe dependence for activities of daily
management and care of these patients. living which showed in Barthel Scale with 42 (25.0-78.7).
Conclusion: The assessment of caregiver burden was
mild in the studied sample of caregivers of our patients.
Doenças Neuromusculares This sample, despite presenting with severe dependence
for activities of daily living, shows functional depen-
dence modification of up to 25% and low probability of
depression, besides the FACIT Spiritual Well-Being of
the caregivers. It is possible that happens psychological
biases when caregivers, especially family members, are
questioned about their position as caregivers.

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XXX Congresso Brasileiro de Neurologia

TL 1105468 TL 1105473 TL 1105485

EVALUATION OF DYSPHAGIA, SARCOPENIA PREVALENCE OF DYSARTHRIA, DYSPHAGIA ASSESSMENT OF EXECUTIVE FUNCTIONS


AND PULMONARY FUNCTION IN PATIENTS AND ITS CORRELATION TO TONGUE IN PATIENTS WITH AMYOTROPHIC LATERAL
WITH RARE NEUROMUSCULAR DISEASES STRENGTH AND ENDURANCE IN A CASE SCLEROSIS
SERIES OF PATIENTS WITH POMPE DISEASE
Rayanne da Silva Souza, Vívian Pinto de Almeida, Patricia Gomes Pinheiro, Stephanie de Freitas
Ana Carolina Frinhane Herzog, Daniel Lucas de Lima Deborah Santos Sales, Vívian Pinto de Almeida, Canelhas, Vívian Pinto de Almeida, Carolina Garcia
Silva Santos, Stephanie de Freitas Canelhas, Karina Ana Carolina Frinhane Herzog, Karina Lebeis Pires, Nuñez Carrijo, Karina Lebeis Pires, Clarissa de
Lebeis Pires, Deborah Santos Sales, Patricia Gomes Mariana Ribeiro Pereira, Elisa Gutman Gouvea, Araujo Davico, Deborah Santos Sales, Rayanne da
Pinheiro, Clarissa de Araujo Davico, Pedro Ignacio Pedro Ignacio Ferraz Montenegro de Almeida, Daniel Silva Souza, André Bertola Vanzan Filho, Mariana
Ferraz Montenegro de Almeida Lucas de Lima Silva Santos, Stephanie de Freitas Ribeiro Pereira
Canelhas, Carolina Garcia Nuñez Carrijo
Casa Hunter. Rio de Janeiro RJ, Brazil Casa Hunter. Rio de Janeiro RJ, Brazil
Universidade Federal do Estado do Rio de Janeiro. Universidade Federal do Estado do Rio de Janeiro. Universidade Federal do Estado do Rio de Janeiro.
Rio de Janeiro RJ, Brazil Rio de Janeiro RJ, Brazil Rio de Janeiro RJ, Brazil
Casa Hunter. Rio de Janeiro RJ, Brazil
clarissadavico@hotmail.com patricia.pinheiro7@yahoo.com.br
clarissadavico@hotmail.com
Background: Neuromuscular diseases (NMDs) affect Background: Rare neuromuscular diseases are chronic
the Central Nervous System (CNS) and the Peripheral Background: Pompe’s disease is defined by the deficiency degenerative, presenting, in some cases, a motor and/or
Nervous System (PNS). They can be chronic, progressive of acid alpha-glucosidase (GAA) of autosomal-dominant bulbar deficit. Some studies mention a significant presence
and degenerative. Dysphagia is characterized by changes hereditary inheritance that leads to the gradual and sys- of executive dysfunctions in patients with Amyotrophic
in one or more phases of swallowing biomechanics, pre- temic accumulation of glycogen inside the lysosomes Lateral Sclerosis (ALS). The Five Digit Test (FDT) aims
venting adequate safe, efficient and comfortable oral and generates a picture of progressive muscle weakness, to measure mental processing speed and the ability to
intake. It can cause important functional damage to the cardiac disorders, insufficiency breathing and a host of direct and switch their attentional control, demanding
individual, such as pneumonias, malnutrition, dehydra- other symptoms. Based on the clinical analysis of patients executive function skills such as response inhibition
tion and mortality. Another frequent manifestation is sar- affected by the disease, the involvement of the orofacial and cognitive flexibility. Objectives: The objective of
copenia, which is defined as the natural and progressive anatomy and its musculature was observed, leading to this study is to describe executive functions in patients
loss of muscle mass and strength, whether peripheral the condition of tongue strength deficit that can have an with ALS with a multilingual and non-reading test like
or respiratory. Objective: The objective of this study is outcome of dysphagia and dysarthria. Objective: report FDT. Methods: Cross-sectional and descriptive study,
to evaluate these manifestations in a group of patients the prevalence of dysarthria, dysphagia and its correlation in which patients treated at an outpatient clinic for rare
from an outpatient clinic for rare neuromuscular dis- to tongue strength and endurance in a Serie of patients neuromuscular diseases were selected. As inclusion crite-
eases, followed by a multidisciplinary team, using the with Pompe disease. Methods: Seven patients were eval- ria, having rare neuromuscular disease and over 18 years
“Eating Assessment Tool – EAT-10, in the classification uated at a neuromuscular rare disease outclinic from a of age. We excluded patients whose medical conditions
of the risk of dysphagia. Methods: The following instru- university Hospital of Rio de Janeiro, from March 2020 made it impossible to understand or perform the tests
ments were applied: sociodemographic and clinical data; to December 2021. Tongue strength and endurance was (such as acute confusional state, aphasia, reduced level of
Eating Assessment Tool (EAT-10) scale; Speech and voice evaluated using the Iowa Oral Performance Instrument consciousness, dementia, and sensory deficits). The FDT
assessment; Assessment of strength and resistance of (IOPI). Speech intelligibility was assessed from spon- test was applied to assess executive functions in patients
lips and tongue measured by Iowa Oral Performance taneous speech. Dysarthria was characterized as mild, with ALS. All percentiles below the 25th are considered
Instrument (IOPI); Nutritional assessment, including moderate or severe according to the screening of the 5 “changed”, given that scores below the 25th percentile
calf circumference and handgrip strength measurement; motor bases of speech. Dysphagia was evaluated using indicate mild difficulties in executive functioning and
Maximum inspiratory and expiratory pressure and peak the water swallowing test. Tongue strength and endurance processing speed, even without necessarily having clin-
expiratory flow values. Results: Seventy-two patients were was evaluated using the Iowa oral performance instrument ical significance, and scores below the 5th percentile are
analyzed, 38.88% men and 61.11% women. Fifty – nine (IOPI). Results: The average age was 48.42 ± 14.92 years. more indicative of prominent deficits. Results: A total of
patients with ALS, twenty-three with SMA. According to 42.9% were female (3) and 57.1 male (4). The prevalence 16 patients were included in the study; 10 patients (62.5%)
EAT-10, it was classified patients into risk or not risk for of dysarthria was 42.9 % (3), being characterized as mild in were male and mean (SD) age was 56 (11.04) years. More
dysphagia. In the risk group, age was 62.0 ± 15.39 years. all patients. Speech intelligibility was presented in 42.9% than half of patients showed changes to FDT decoding
BMI, calf circumference and hand grip were respectively (3) of the sample. The prevalence of dysphagia was 28.6 and retrieving (75% and 68.75% respectively). However,
25.10 ± 11 Kg/m2, 36.0 ± 3.3 cm and 9.8 ± 10.22 kpa. The (2). The average strength and resistance of the tongue was the control processes are 56.25% not changed in Response
PImax and the PEmax were 40.00 ± 29.62 cmH20 and 31.00 low, when compared to the reference values ??for age (28 Inhibition and 62.5% not changed in cognitive flexibility.
± 31.82 cmH20. Peak flow value and the maximum time ± 11,07 kPa; 10,71 ± 8,03 kPa respectively). The means of Conclusion: Patients with ALS can manifest executive
of sustained phonation (TMF) were 160.0 ± 103.3 L/min tongue strength and resistance were significantly lower dysfunction severe enough to classify them as cognitively
and 6.46 ± 9.66 s. The maximum peak of tongue tip and in the groups of patients with dysarthria, dysphagia and impaired. However, in this sample, despite the automatic
dorsum were, respectively, 17.50 ± 13.56 kPa and 17.00 ± impaired speech intelligibility. Conclusion: Low tongue processes (reading and counting) being altered, the inhi-
12.20 kPa. Finally, the endurance strength result was 9.00 strength and resistance are related to the increased preva- bition and cognitive flexibility, which are control processes
± 7.19 s. Values ??from the group without risk of dyspha- lence of dysarthria, dysphagia and impairment on speech and depend on executive function, responses remained
gia were higher, but no significant differences when the intelligibility, which demonstrates the importance of preserved. Furthermore, more studies are needed to better
groups were compared. Conclusion: The importance of including these measurements in the screening of patients understand and monitor executive functions in patients
a multidisciplinary team in the follow-up of patients with with pompe’s disease. with ALS, as well as discuss an assessment more adapted
neuromuscular diseases in order to promote compre- to the progression of the disease.
hensive care is highlighted. This study presented partial
results, requiring an expanded analysis to better assess Doenças Neuromusculares
the outcomes of rare conditions that affect the quality of Doenças Neuromusculares
life of these individuals.

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XXX Congresso Brasileiro de Neurologia

TL 1105491 TL 1105504 TL 1105564

THE AVALGLUCOSIDASE ALFA PHASE 3 AVALGLUCOSIDASE ALFA IMPROVES DIAGNOSTIC YIELD OF WHOLE-EXOME
COMET TRIAL IN LATE-ONSET POMPE HEALTH-RELATED QUALITY OF LIFE (HRQOL) SEQUENCING AND GENETIC PANEL
DISEASE PATIENTS: EFFICACY AND SAFETY IN PATIENTS WITH LATE-ONSET POMPE IN ADULT BRAZILIAN PATIENTS WITH
RESULTS AFTER 97 WEEKS DISEASE (LOPD) VS. ALGLUCOSIDASE ALFA: MYOPATHY
PATIENT-REPORTED OUTCOME MEASURES
Paulo Victor Sgobbi de Souza, on behalf of the Alexandre Motta Mecê, Lucas de Melo Teixeira
COMET Study Group (PROMS) FROM THE PHASE 3 COMET TRIAL Branco, Anamarli Nucci, Marcondes Cavalcante
Paulo Victor Sgobbi de Souza, MD, on behalf of the França Júnior
Universidade Federal de São Paulo. São Paulo SP,
Brazil COMET Study Group Universidade Estadual de Campinas. Campinas SP,
Universidade Federal de São Paulo. São Paulo SP, Brazil
pvsgobbi@gmail.com
Brazil amottamece@gmail.com
Avalglucosidase alfa, a recombinant human GAA enzyme pvsgobbi@gmail.com
replacement therapy with increased mannose-6-phos- Background: Myopathies in adults have multiple eti-
phate content for increased cellular uptake, is approved The phase 3 COMET trial (NCT02782741) assessed ologies, but a significant proportion of cases are due to
in the United States for late-onset Pompe disease (LOPD) PROMs in treatment-naïve adult LOPD patients receiv- genetic variants in more than 200 known genes. Whole-
patients ?1 year of age and in Japan for all Pompe dis- ing avalglucosidase alfa (AVAL, n=51) or alglucosidase exome sequencing (WES) and genetic panels (GP) have
ease patients (Nexviazyme(TM), Sanofi Genzyme, alfa (ALGLU, n=49). PROMs comprised generic mea- been employed as a diagnostic test in this setting with
Cambridge, MA). During the 49-week, double-blinded sures (12-item Short-Form Health Survey [SF-12], Patient variable success rates. Factors such as age, ethnic back-
primary-analysis period (PAP) of the Phase 3 COMET Global Impression of Change [PGIC]), EuroQoL-5D-5L ground and phenotypic characterization of patients seem
trial (NCT02782741) comparing avalglucosidase alfa [EQ-5D-5L]), and LOPD-specific measures (Pompe to influence in both accuracy for myopathies. Herein, we
with alglucosidase alfa, avalglucosidase alfa resulted Disease Symptom Scale [PDSS], Pompe Disease Impact investigated the diagnostic yield of WES and GP in a cohort
in greater improvements in forced vital capacity (FVC), Scale [PDIS], and Rasch-Built Pompe-Specific Activity of adult patients with myopathy coming from a popula-
6-Minute Walk Test (6MWT), and other outcomes and a [R-PAct]) that cover various domains: breathing, mobil- tion with mixed ethnic background (Brazil). Objective:
more favorable safety profile than alglucosidase alfa in ity, fatigue, disease-related symptoms, upper extrem- to determine the accuracy of WES and commercial GP in
treatment-naïve LOPD participants. During the open-la- ity weakness, pain, morning headache, daily activities, a population of patients from a reference neuromuscular
bel extension treatment period (ETP), 51/51 participants physical functioning, and mental health. Change from center with suspected genetic myopathy. Methods: 106
receiving avalglucosidase alfa during the PAP continued baseline to Week 49 was analyzed using mixed models patients attending a reference neuromuscular center were
this treatment and 44/49 receiving alglucosidase alfa for repeated measures. AVAL was numerically better than recruited. Diagnosis for all of them remained elusive after
during the PAP switched to avalglucosidase alfa. Changes ALGLU across all domains except for pain/discomfort physical examination, muscle biopsy, electrophysiology
(LS mean [SE]) from Baseline at Week 97 are reported. (EQ-5D-5L) and mood (PDIS). Between-group difference and lab tests. WES was performed following standard
Changes in FVC %predicted were +2.65 (1.05) for aval- was nominally significant (p-value <0.05) for PGIC ques- procedures for 30 patients, while GP was performed for
glucosidase alfa PAP participants and +0.36 (1.12) for tions: daily activities (least squares mean difference vs. 76. We identified variants labeled as pathogenic or likely
alglucosidase alfa PAP participants. Changes in 6MWT ALGLU (95% confidence interval [CI]): 0.57 [0.08, 1.06]), pathogenic following ACMG guidelines. Then, diagnos-
distance (meters) were +18.60 (12.01) for avalglucosidase disease-related symptoms (0.78 [0.31, 1.25]), mobility tic yield of WES and GP were determined for the whole
alfa PAP participants versus +4.56 (12.44) for alglucosi- (0.65 [0.18,1.12]); and EQ-5D mobility (–0.31 [–0.62, 0.00]). cohort and for subgroups defined according to the major
dase alfa PAP participants. Similar trends occurred in Post-hoc responder analyses numerically favored AVAL phenotypes. Results: Mean age of patients was 38 years
other Week-97 outcomes. Treatment-emergent adverse vs. ALGLU for all PROMs. Proportion of responders was and there were 43% men. Definite genetic diagnosis
events (AEs) during the ETP occurred in 49 (96.1%) and nominally significantly greater (p<0.05) for all PGIC ques- was found in 12 from 30 patients in WES group (40%
42 (95.5%) participants from the avalglucosidase alfa tions (daily activities [62.0% vs. 37.0%], disease-related accuracy), while 36 from 76 in the genetic panel group
and alglucosidase alfa PAP groups, respectively. Five symptoms [74.0% vs. 49.0%], ability to breathe [66.0% vs. (47%), leading to a diagnostic yield of 45% in this cohort.
participants discontinued during the ETP by Week 97 41.0%] and mobility [66.0% vs. 29.0%]), EQ-5D-5L (usual Pathogenic variants were found at TRAPPC11, SCN4A,
due to AEs (ocular hyperemia, erythema, urticaria, respi- activities [54.0% vs. 30.6%]), and PDSS (shortness of breath ACADS, COL6A2, SGCB, CAPN3, SGCB, POLG, TWNK,
ratory distress, acute myocardial infarction, pancreatic [26.0% vs. 4.1%], overall fatigue [22.0% vs. 2.0%], fatigue/ RYR1, TRIM32, TTN, EMD, SLC25A4, TPM3, DOK7, DMD,
adenocarcinoma). Twenty-two participants had treat- pain [18.0% vs. 4.1%], and morning headache [22.0% vs. ANO5, CHRNE, GAA, ADSSL1, CLCN1, PYGM, PNPLA,
ment-emergent serious AEs during the ETP. Patients who 4.1%]). Cumulative distribution functions showed a clear HACD1, NEB, DPAGT1, MYH7, FKTN. Conclusion: WES
switched from alglucosidase alfa to avalglucosidase alfa separation in favor of AVAL for full range of thresholds in and GP are useful diagnostic test for adult patients with
presented no safety- or immunogenicity-related con- PDSS (shortness of breath, overall fatigue, and fatigue/ undefined myopathy. In this mixed ethnic background
cerns. Overall results demonstrate sustained treatment pain) and PDIS (difficulty in performing daily activities). cohort, diagnostic yield was 45% and variants in multiple
effect for improvements observed with avalglucosidase These LOPD-specific PROMs provide further support for genes were identified.
alfa during the PAP and stabilization of treatment effect greater therapeutic benefit of AVAL over ALGLU, includ-
after switching from alglucosidase alfa to avalglucosidase ing the most relevant LOPD domains – mobility, usual
alfa over 97 weeks, supporting long-term maintenance of Doenças Neuromusculares
activities, and breathing. This dataset confirms mean-
outcomes and persistence of avalglucosidase alfa’s effect. ingful impact of AVAL on LOPD patient’s quality of life.

Doenças Neuromusculares Doenças Neuromusculares

251
XXX Congresso Brasileiro de Neurologia

TL 1105650 TL 1105706 TL 1105713

FHL1-RELATED MYOPATHY IS ASSOCIATED PROFILE OF RESPIRATORY CHAIN ENZYME HISTOPATHOLOGICAL FINDINGS OF


TO INFLAMMATORY FINDINGS IN SKELETAL ACTIVITY IN PATIENTS WITH EXERCISE RYANODINE RECEPTOR 1 (RYR1)-RELATED
MUSCLE HISTOLOGY INTOLERANCE AND MILD CHANGES AT MYOPATHIES IN A COHORT FROM A
MUSCLE BIOPSY TERTIARY NEUROMUSCULAR CENTER
Lucas Marenga de Arruda Buarque, André Macedo
Serafim Silva, Clara Gontijo Camelo, Alulin Tácio Elmano Henrique Torres de Carvalho, Raissa Gontijo Beatriz Carneiro Gondim Silva, André Macedo
Quadros Santos Monteiro Fonseca, Cristiane Araujo Cabral de Souza, Silvia Helena Andrião Escarso, Serafim da Silva, Alulin Tácio Quadros Santos
Martins Moreno, Rodrigo Holanda Mendonça, Bruna Margleice Marinho Vieira Rocha, Cláudia Ferreira da Monteiro Fonseca, Clara Gontijo Camelo, Cristiane
Moreira de Souza Proença, Beatriz Carneiro Gondim Rosa Sobreira de Araújo Martins Moreno, Lucas Marenga Buarque,
Silva, Edmar Zanoteli Bruna Moreira Proença, Edmar Zanoteli
Faculdade de Medicina de Ribeirão Preto da
Faculdade de Medicina do Hospital das Clínicas da Universidade de São Paulo. Ribeirão Preto SP, Brazil Hospital das Clínicas da Universidade de São Paulo.
Universidade de São Paulo São Paulo SP, Brazil
raissagont@gmail.com
l.marengabuarque@gmail.com beatrizcgondim@gmail.com
Background: Exercise intolerance is a nonspecific term
Background: FHL1 (Four-and-a-Half-LIM domains 1) used to describe disproportionate fatigue, myalgia and/ Background: Ryanodine receptor 1(RYR1)-related myop-
is a protein involved in signaling processes, differenti- or cramping as a consequence of muscular exertion. athy presents with a variety of phenotypes characterized
ation and maintenance of structural cellular elements. Although it is a common complaint, a specific etiologic by early onset of symptoms such as hypotonia, delayed
Mutations in FHL1 can lead to a myopathy with myofibril- diagnosis is rarely achieved, even after comprehensive motor development, proximal muscle weakness, ptosis
lar derangement and intracytoplasmic bodies, known as investigation. Objective: In the present study we aim to and/or ophthalmoparesis, facial weakness, thoracic defor-
reducing body myopathy (RBM). RBM is characterized by define the profile of respiratory chain enzyme activity in mities, and spinal involvement. Among the congenital
progressive weakness in childhood and the presence of skeletal muscle of patients with exercise intolerance and myopathies, RYR1-related myopathy is the most frequently
cytoplasmic aggregates that reduce nitro-blue-tetrazolium mild and nonspecific changes at muscle biopsy. Methods: diagnosed which includes a heterogeneous spectrum
(NBT) in muscle fibers. In addition to RBM, other phe- One hundred and three adult patients with long lasting of histopathological and clinical subtypes followed by
notypes are described, such as Emery-Dreifuss, X-linked unexplained exercise intolerance were included in the variable disease severity. Objectives: To describe the
scapuloperoneal myopathy and hypertrophic cardiomy- study. They were followed at the outpatient clinic of our epidemiological and histopathological findings on mus-
opathy. We recently described a case of RBM presenting University Hospital and were subjected to muscle biopsy cle biopsy of a case series with RYR1-related myophaties
as inflammatory myopathy. However, there is no data in the course of diagnostic investigation. Inclusion criteria: in a tertiary referral center. Methods: We retrospectively
systematically investigating inflammatory findings in 18 years old or above; chronic (2 years or more) myalgia selected patients who had a clinical history of proximal
muscle biopsy in FHL1-related myopathy. Objective: To and/or fatigue in relation to exercise; non-specific find- myopathy or congenital myopathy and a positive genetic
evaluate inflammatory findings in skeletal muscle histol- ings in the muscle biopsy. Exclusion criteria: insufficient test for the RYR1 gene. We evaluated clinical features and
ogy of patients with FHL1-related myopathy. Methods: muscle sample size for enzyme assays. Respiratory chain histological aspects. The evaluation of muscle biopsies
We retrospectively identified patients with FHL1-related activities of complexes I, II, III, II+III and IV were mea- included grading the fiber variability, nuclei internaliza-
myopathy in a tertiary neuromuscular center. We reviewed sured in whole muscle homogenates. Citrate synthase tion, presence of type 1 or type 2 atrophy, intermyofibril-
medical records to clinical description. We evaluate his- activity was measured to estimate the mitochondrial lar disorganization, presence of cores and presence of
topathological findings of muscle biopsy of the patients mass, which was used to correct enzyme activity. Results: necrosis. Results: Thirty-one patients with RYR1-related
using the following stains: hematoxylin and eosin, mod- Activity of respiratory chain enzymes was unremarkable myopathy were included. Age varied from 4 to 62 years old
ified Gömöri’s trichrome, NADH-tetrazolium reductase, in 70% of patients. However, 16% of patients presented a (median age of 17). Most patients (61.3%) were male,. The
succinate dehydrogenase, cytochrome-c-oxidase, acid complex I deficiency (mean 0.38 and SD 0.29 micromol/ most common phenotype was classical congenital myop-
phosphatase, NBT and immunohistochemistry using com- min/g tissue; when corrected for citrate synthase activity). athy (25/31), representing 80%, followed by later-onset
mercial antibodies against desmin, CD4, CD8, CD68 and Another 9% of patients presented isolated deficiency of congenital myopathy with 9.6%. One patient presented
major histocompatibility complex type I (MHC-I). Results: complexes II (mean 0.48 and SD 0.34), III (mean 0.72 and with rhabdomyolysis phenotype and one patient with
Five patients with FHL1-related myopathy were clinically SD 0.25) or IV (mean 1.12 and SD 0.3). In 5% of patients, a distal myopathy. Ptosis was reported by 22.5% of the
evaluated. Two patients (one male and one female) pre- there was a combined deficiency of complexes I, III and patients and a history of malignant hyperthermia in one
sented the classical RBM phenotype, and three females IV. Conclusion: Although skeletal muscle biopsy with patient. Regarding histological findings, central core pat-
presented an X-linked scapuloperoneal myopathy. All routine histochemical studies frequently show mild and tern appeared in five cases,. The centronuclear pattern
patients presented increased serum CK over 10x the UNL. nonspecific changes in patients with exercise intolerance, occurred in six patients, as well as dystrophic pattern was
Histopathological analysis from all patients showed intra- respiratory chain enzyme assays may contribute to a better seen in other six ones. Importantly, one patient showed
cytoplasmic desmin-positive aggregates, NBT-reducing understanding of the pathophysiology of exercise intoler- abundant necrosis on muscle biopsy, in addition to the
bodies, necrotic fibers and intense increased acid phos- ance in a significant portion of those patients. presence of intracytoplasmic cores. Discussion: We pres-
phatase activity. Moderate endomysial CD4+, CD68+ cell ent a large cohort of patients with genetically confirmed
infiltrates, in addition to milder CD8+ infiltrates, with mild RYR1-related myopathy. Histopathological findings var-
to moderate increase in MHC-I staining was observed Doenças Neuromusculares ied, as did phenotypes. Most patients had dystrophic or
in all patients. Conclusion: FHL1-related myopathy is centronuclear patterns. An interesting finding was the
associated with intense inflammatory findings in skele- presence of abundant fiber necrosis in one patient, which
tal muscle histology. Some cases can be misdiagnosed as is not a characteristic usually described in these patients.
inflammatory myopathy. Curiously, anti-FHL1 antibodies The broad spectrum of RYR1-related myopathy is not only
was previously described in 25% of patients with idiopathic clinical but also histological.
inflammatory myopathy, which indicate that FHL1 can
be an epitope able to induce an inflammatory response.
Doenças Neuromusculares

Doenças Neuromusculares

252
XXX Congresso Brasileiro de Neurologia

TL 1105739 TL 1105743 TL 1105842

THE RELATION BETWEEN MYASTHENIA PEDIATRIC NEUROMUSCULAR HOSPITALIZATIONS FOR TREATMENT


GRAVIS AND COGNITION: A REVIEW OF DISORDERS IN A TERTIARY HOSPITAL AT OF ACUTE DEMYELINATING
LITERATURE NORTHEASTERN BRAZIL POLYRADICULONEURITIS AND THE
PANDEMIC: A QUANTITATIVE STUDY
Mateus Pradebon Tolentino, Mariana Floriano Luiza Djanino Fernandes Silva, Elton Spirty Rodrigues
Piva, Daniel Thomas Pereira Lopes, Eduardo de Silva, Larissa Clementino Leite de Sá Carvalho, Mariana Oliveira Salamargo, Lukas Santos Freire,
Paula Estephan, Pedro Henrique Marte de Arruda Marco André de Moraes Bernardino, Victor Fellipe Guilherme Rocha Cardoso, Rebeca Lima de Almeida
Sampaio, Cristiane de Araújo Martins Moreno, Bispo Macêdo, Paulo Barbosa Leite Neto, Pedro Santos, Victor Sousa Campos, Giovana Andrade de
Sonia Maria Dozzi Brucki, Ivy Liger Riso, Flávio Vieira Nogueira Fontana, Carolina Cunha Correia, Fabíola Oliveira, Erica Otoni Pereira Miranda, Gabriel Meira
Marques Filho, Hugo Salomão Grangeiro Furtado Lys Medeiros Xavier, Lucas Silva Santos, Philip George Glass
Mirô Andrade
Hospital Universitário Oswaldo Cruz da
Casa de Saúde Santa Marcelina. São Paulo SP, Universidade de Pernambuco. Recife PE, Brazil UESB. Vitória da Conquista BA, Brazil
Brazil Universidade de Pernambuco. Recife PE, Brazil FASA. Vitória da Conquista BA, Brazil
mateus_pradebon@hotmail.com djanino@gmail.com marianaoliveira.uesb@gmail.com

Background: Myasthenia Gravis (MG) is an autoimmune Background: Neuromuscular disorders (NMD) are diag- Background: Acute immune-mediated polyneuropathies,
chronic disease that involves neuromuscular junction nosed across the lifespan and create many challenges also called Guillain-Barré syndrome (GBS), is one of the
generating muscle weakness and fatigability. In addition especially with infants, children, and adolescents. NMD main causes of acquired weakness. The pathogenesis
to peripheral muscle involvement, cognitive impairment typically cause muscle weakness, and usually, the symp- occurs when an immune reaction, in response to a pre-
has been hypothesized for a long time. Controversial toms slowly get worse over time. Objective: To evaluate vious infection, cross-reacts with shared epitopes in the
results about the impact of MG in cognitive functions the epidemiological, clinical, and diagnostic profile of peripheral nerve. This process generates a clinical con-
are present in varied studies. Objective: To evaluate the pediatric patients with neuromuscular diseases. Method: dition characterized by symmetric and progressive limb
literature regarding relation between MG and cognitive A retrospective cross-sectional study with consultation weakness, sensory symptoms and dysautonomia. Some
dysfunction. Methods: An extensive literature review was of outpatient records of child and adolescent popula- cases require immediate Hospitalization, since among
carried out based on analysis and selection of articles and tion symptomatic for neuromuscular diseases treated the main complications is bulbar weakness, which can
publications found on MEDLINE, SCIELO, LILACS and at a specialized referral center at a university Hospital lead to respiratory failure. Several cases of GBS associ-
grey literature. We included in this review studies refer- in northeastern Brazil. The statistical study was descrip- ated with COVID-19 have been reported in the literature,
ring to congenital myasthenia to access myasthenic syn- tive of the variables analyzed. Results: We evaluated 77 although no direct causal relationship has been estab-
dromes. Results: In this search, 415 records were found, patients (60% male), age at onset of symptoms 1day of lished. Objective: This article aims to verify if there was an
corresponding to 101 different studies selected. About 20 life-15 years. Affected age group: <2 years=26 (34%, 65% increase in the number of GBS diagnoses in Brazil during
studies compared cognitive impairment between patients men; and <6months 65.4%); 3-6years=25 (32.4%, 60%men); the pandemic, looking for a possible causal correlation
with myasthenia and healthy control. The majority of those 7-10 years= 19 (24.6%, 52.6% men); and >11 years=7 (9%, with COVID-19. Methods: This is a descriptive quantita-
studies demonstrated correlation between myasthenia 44.4% men). Diagnoses were grouped into: Myopathies= tive study, conducted in the database of the Ministério
and cognitive impairment. The main domains affected 45 patients, 58.4% (Mitochondrial 22.2%; Congenital 18%; da Saúde, DATASUS, in the Hospital Procedures section
were language, visuospatial function, information pro- Metabolic 6.6%; Inflammatory 6.6%; Duchenne Dystrophy (SIH/SUS) with data on Hospitalizations for treatment of
cessing and memory. Conclusion: Despite the fact that (DMD) 15.6%; Sarcoglycanopathies 11.1%; LAMA2- 11.1%; acute demyelinating polyradiculoneuritis referring to the
MG is traditionally seen as a purely motor disease, there Emery-Dreifuss 4.4%; Becker 2.2%; Collagen 6- 2.2%); period from January 2017 to December 2021. The values
are evidences suggesting it is related to cognitive dys- Motor Neuron Diseases= 14 patients, 18.2% (Spinal mus- were categorized by year of attendance. Data analysis
functions in multiples domains. Beside that, it is associ- cular atrophy – SMA-1 50%; SMA-2 7.2%; SMA-3 28.5%; was performed in Microsoft Excel version 2019 program.
ated with more neuropsychiatrist symptoms and state of others 14.3%); Neuropathies=11.7% (Charcot Marie Results: A reduction in the number of Hospitalizations
fatigue. Further studies are needed to evaluate the impact Tooth- CMT 77.8%); Myoneural Junction Disease= 7.8% for treatment of acute polyradiculoneuritis was observed
of cognitive impairment in myasthenic patients and the (Myasthenia Gravis (MG) 83.3%; Myasthenia congenital during the years 2020 to 2021, characterized by a total of
pathophysiological mechanisms. 16.7%); Plexopathy= 3 (3.8%, Traumatic 66.7%; Parsonage 3740, in relation to the amount found from March 2018 to
Turner 33.3%). Muscle involvement occurred in 11.7% February 2020, 4362 Hospitalizations, with the year 2020
eye (66.7% MG), 28.5% face (32% SMA), 31.2% swallow- having the lowest Hospitalization rate, 21.7%, compared
Doenças Neuromusculares ing (29% SMA), 19.5% neck (46.7% SMA), 72.7% upper to the total of the period analyzed. About the reduction
limbs, 84.4% lower limbs. Respiratory involvement 26% of expected cases compared to the real data, it is hypoth-
(SMA 40%; LAMA2 20%). Impairment in walking 53.2% esized that there was a decrease in the number of beds
(SMA 29.2%; Dystrophies 19.5%; CMT 14.6%). Intellectual available for treatment of GBS cases due to the resettle-
Deficit 10.4% (DMD 50%). Heart disease 6.5% (DMD 40%). ment for severe cases of covid. Also, one can speculate
Conclusion: Our study showed that neuromuscular dis- about an underreporting of this pathology and, finally, the
eases begin more frequently in boys, infants (especially, safety protocols during the period avoided transmission
younger than 6 months) and preschoolers. More than half routes for etiological agents of GBS, possibly decreasing
of patients have Myopathies (mainly, mitochondrial). The its incidence. Conclusion: The data presented point to a
most affected muscle groups were those of 4 members, lower incidence of GBS in the period. Future studies will be
mainly the lower ones. However, SMA patients were fre- necessary to better understand the relationship between
quently affected by the face, swallowing, neck, breathing COVID-19 and acute polyradiculoneuritis, which was
and walking, being a greater gravity. Thus, understanding found to be unassociated by the present study. The lack
our pediatric population with neuromuscular diseases, of specific data for GBS case diagnosis in the DATASUS
accurate diagnosis, correct therapeutic and better prog- system was a limitation of the study.
nosis can be performed.

Doenças Neuromusculares
Doenças Neuromusculares

253
XXX Congresso Brasileiro de Neurologia

TL 1105879 TL 1106082 TL 1106120

EPIDEMIOLOGICAL PROFILE OF 494 SENSORY NEURONOPATHY IN MULTIPLE ANALYSIS OF THE GENETIC AND
PATIENTS WITH NEUROMUSCULAR DISEASE ACYL-COENZYME A DEHYDROGENASE EPIDEMIOLOGICAL PROFILE OF PATIENTS
IN A BRAZILIAN UNIVERSITY CENTER DEFICIENCY (MADD) WITH LIMB-GIRDLE MUSCULAR DYSTROPHY
IN REFERENCE CENTERS FROM
Thiago Duque Pinheiro, Isadora Versiani, Renata José Pedro Soares Baima, André Macedo Serafim
Eiras, Eduardo Davidovich, Bruno Coutinho, Camila Silva, Rodrigo de Holanda Mendonça, Fernando PERNAMBUCO
Pupe, Osvaldo Nascimento Freua, Michelle Abdo Paiva, Edmar Zanoteli Rodrigo Nobre de Novais, Anna Paula Paranhos
Universidade Federal Fluminense. Niterói RJ, Brazil Hospital das Clínicas da Faculdade de Medicina da Miranda Covaleski, Eduardo Souza de Melo, Álvaro
Universidade de São Paulo. São Paulo SP, Brazil José Porto Moreira, José Luiz de Miranda Coelho
isadora.versiani@gmail.com Inojosa, Magda de Melo Freire
jpedrobaima@gmail.com
Background: It is of utmost importance to seek cross Universidade Federal de Pernambuco. Recife PE,
border collaboration and common ground to facilitate Background: Multiple acyl-CoA dehydrogenase defi- Brazil
disease’s diagnosis and management. Nonetheless, little ciency (MADD) is a recessive disorder due to ETFA, ETFB, Universidade de São Paulo. Ribeirão Preto SP, Brazil
effort has been made regarding epidemiological profile of and ETFDH pathogenic variants. Typical late-onset pre- rodrigonobredenovais@gmail.com
neuromuscular disorders. Objective: This report analyses sentation includes exercise intolerance, cyclical vomiting,
the epidemiological profile of 494 patients that presented and progressive muscle weakness. Only a few reports of Background: Limb-girdle muscular dystrophies (LGMD)
for consultation in our university Hospital, a tertiary care sensory neuronopathy were associated with MADD, with are the fourth leading cause of genetically generated
center for neuromuscular patients in Brazil. Methods: Data variable improvement after riboflavin supplementation. muscle weakness. LGMDs form a heterogeneous group
was collected from April 2015 to April 2022. The patients Objectives: We aim to describe MADD-associated sensory of diseases, which includes hereditary pathologies that
were stratified according to topography – (1) nerve; (2) ganglionopathy from a cohort of MADD patients in a ter- preferentially affect the proximal muscles of the pelvic
muscle; (3) motor neuron; (4) neuromuscular junction tiary neuromuscular center. Methods: This retrospective and scapular girdle. There are currently 29 subtypes,
– and etiology. Results: The most prevalent disorder was study presents clinical, molecular and response charac- the vast majority of which are autosomal recessive. As
neuropathy (314 cases, 63,5%), followed by neuromus- teristics after treatment. Results: We identified five cases it is a rare pathology, prevalence data are limited. There
cular junction disorders (73 cases, 14,1%), myopathy (53 with myopathy and genetically confirmed MADD. Three is a lack of studies that evaluate the epidemiological
cases, 11,8%) and motor neuron disease (40 cases, 8%). patients manifested with sensory neuronopathy. All these and genetic profile of LGMDs in the brazilian northeast
Among patients with neuropathy, 276 (87,8%) cases were cases were female, aged 34, 67 and 74. Physical examina- region. Objective: Evaluate the epidemiological and
related to acquired etiologies and 38 (12,25%) to hered- tion was remarkable for severe sensory ataxia and global genetic aspects of patients with suspected limb-girdle
itary causes. In the subgroup of acquired neuropathies, weakness. Two patients were unable to walk, complained muscular dystrophy who underwent a molecular panel
major etiologies were inflammatory in 97 cases (35,2%), of fluctuating hoarseness, and had a dropped head on at two referral centers in Pernambuco. Methods: This is a
metabolic in 48 (15,3%), infectious in 35 (11,1%), toxic examination. Electrodiagnostic studies were consistent cross-sectional observational study. Data were collected
in 4 (1,1%) and there were 17 cases with more than one with a ganglionopathy in all cases, but two patients also from the medical records of patients treated in two cen-
etiology hypothesized, 34 cases with other etiologies had a superimposed myopathy. Serum CK levels were high ters from Pernambuco. Demographic data such as age,
(nutritional, post-surgical, compressive or traumatic) in only one patient. Muscle MRI of the three patients had gender and origin were analyzed, as well as the results of
and 39 (14,13%) cases with no etiology defined (cur- fat substitution or edema. Acylcarnitine profiles were col- the molecular panels performed by the patients. Results:
rently under investigation). Conclusion: The majority of lected in two patients, with only one test consistent with Information was collected from the medical records of 37
neuromuscular disease in our cohort was represented MADD. Muscle biopsies of all patients showed lipidic patients, 17 had their diagnosis confirmed, the majority
by nerve disorders and the inflammatory etiology was droplets and mild mitochondrial disfunction. A next-gen- being sarcoglycanopathies (70.6%), especially alpha-sar-
the most frequent. Since this study is limited to only one eration sequencing panel for myopathies led to different coglycanopathy with 35.5% of all LGMD subtypes found.
center, and little effort in specialized literature has been heterozygous ETFDH pathogenic variants in the patients. The distribution by age and sex was similar in the group
made to set common referral patterns, selection bias and Riboflavin, coenzyme Q10, and L-carnitine were supple- of 17 patients diagnosed with LGMD, with a higher prev-
information bias may affect the interpretation of the data. mented in all patients with a significant response in motor alence of alpha-sarcoglycanopathy among those younger
Nonetheless, this study contributes for further analysis function, and variable response in sensory symptoms. than 18 years (66.7%), with a higher number of women
including topographic and etiological epidemiology of One patient regained the capacity to walk. Conclusion: in this subtype (88.3%) being also observed. The origin
neuromuscular diseases and calls out for national as well MADD, a curable myopathy, is often missed in its typi- of 14 of the 17 diagnosed patients was found, the major-
as cross-border collaboration to proceed a standardized cal presentation. An atypical presentation as presented ity (four) were from Recife-PE. All patients underwent a
approach to neuromuscular diagnosis. here is even more ignored, and probably missed. Despite molecular panel, a total of 52 different mutations were
extensive investigations, many sensory ganglionopathies observed, being 53.8% pathogenic variants, 17.3% prob-
remain idiopathic, therefore, we propose MADD should ably pathogenic variants and 28.8% variants of uncer-
Doenças Neuromusculares be in differential diagnosis of sensory neuronopathy, tain significance (VUS). Regarding the pathogenic and
especially with the phenotype here described. All three probably pathogenic variants, most were found in the
patients had a heterozygous variant, and we could not SGCA gene and in CAPN3. The most common mutation
conclude whether there is a dominant inheritance related was c.229C>T in the SGCA gene (61.5% of the variants in
to this atypical manifestation, or the second variant was this gene). Among the VUS, most corresponded to DYSF
not found. In author’s experience, the motor function gene mutations with 33.3%, followed by FKRP and CAPN3
has a better response than sensory symptoms, but fur- with 20% each. Conclusion: Despite presenting a small
ther studies and phenotype characterization are needed. population sample, the study presented relevant data
on the genetic and epidemiological profile of LGMDs in
Pernambuco. The results presented can promote stud-
Doenças Neuromusculares
ies, in addition to strengthening general population and
professional health knowledge.

Doenças Neuromusculares

254
XXX Congresso Brasileiro de Neurologia

TL 1106133 TL 1106175 TL 1106189

THE EVALUATION OF A SECOND CYCLE PHENOTYPE-GENOTYPE CORRELATION CLINICAL AND MOLECULAR SPECTRUM OF
OF INTRAVENOUS IMMUNOGLOBULIN IN DESMINOPATHY: VARIANT LOCATION IN MYOFIBRILLAR AND DISTAL MYOPATHIES
FOR PATIENTS WITH GUILLAIN-BARRÉ DES GENE DETERMINES THE SEVERITY OF
André Macedo Serafim Silva, Cristiane Araújo
SYNDROME ON MECHANICAL VENTILATION PHENOTYPE AND THE MORPHOLOGY OF Martins Moreno, Rodrigo Holanda Mendonça,
INTRACYTOPLASMIC AGGREGATES Eduardo Estephan, Clara Gontijo Camelo, Eliene
Emanuela Coriolano Fidelix, Roberto Teodoro
Gurgel de Oliveira, Vinicius Furtado da Silva Castro, Dutra Campos, Marcela Alcântara Machado, Leslie
André Macedo Serafim Silva, Patrícia Rodrigo,
Agabio Diógenes Pessoa Neto, Mário Emílio Teixeira Domenici Kulikowski, Acary Souza Bulle Oliveira,
Cristiane Araújo Martins Moreno, Rodrigo Holanda
Dourado Júnior Edmar Zanoteli
Mendonça, Eduardo Estephan, Clara Gontijo
Camelo, Eliene Dutra Campos, Leslie Domenici Faculdade de Medicina da Universidade de São
Hospital Universitário Onofre Lopes da Universidade
Kulikowski, Montse Olivé, Edmar Zanoteli Paulo. São Paulo SP, Brazil
Federal do Rio Grandeo do Norte. Natal RN, Brazil
Escola Bahiana de Medicina e Saúde Pública.
Faculdade de Medicina da Universidade de São
efidelix@gmail.com Salvador BA, Brazil
Paulo. São Paulo SP, Brazil
Universidade Federal de São Paulo. São Paulo SP,
IDIBELL-Hospital Universitaria de Bellvitge –
Background: Approximately 25% of patients with Guillain- Brazil
Espanha
Barré syndrome (GBS) do not respond to the first cycle
of intravenous immunoglobulin (IVIg) infusion and are macedosera@gmail.com
macedosera@gmail.com
offered a second cycle. There are a few studies on the
effectiveness of this second infusion. Objectives: To Background: Distal myopathies (DMs) are a group of
Background: Desmin (DES) is the main intermediate fil-
evaluate the effectiveness of the second cycle of IVIg in muscle diseases featuring a predominance of weakness
ament in the muscle, arranged in a tridimensional struc-
individuals with GBS who required mechanical ventila- in the distal extremities, while myofibrillar myopathies
ture that connects myofibrils to each other as well as to
tion in the acute phase. Methods: We performed a retro- (MFMs) are skeletal and cardiac muscle diseases with
the nuclei, the sarcolemma and organelles. Pathogenic
spective review of the database of patients with GBS who Z-disk protein aggregates and myofibril disarray. Both
variants in DES cause desminopathy, a disorder affect-
required mechanical ventilation, between the years 1994 conditions may present with predominant distal weak-
ing skeletal muscles and the heart. It is characterized by
to 2018, aged > 6 years, with Brighton level 1 or 2 and who ness and demonstrate a vacuolar histology, confound-
a spectrum of phenotypes and arrangements of intracy-
received 1 or 2 cycles of IVIg, in the state of Rio Grande ing clinical classification. Objectives: To characterize
toplasmic aggregates. Objectives: We aimed to analyze
do Norte. Demographic, clinical and outcome data up patients with DMs and MFMs, comparing both groups
the clinical features and the morphology and distribu-
to 6 months were compared. Results: 74 individuals and classifying their genetic subtypes. Methods: This
tion of desmin aggregates in the skeletal muscle biopsies
from a sample of 350 (21.4%) were selected. 59 (79.7%) observational study identified patients at a tertiary neu-
of patients with desminopathy, and to correlate these
received 1 cycle IVIg and 15 (20.3%) received 2 cycles romuscular center with MFM and DM diagnoses. Patients
findings with genetic characteristics. Methods: This is a
IVIg. There was no difference between age (34 x 39 years; underwent a next-generation sequencing panel, followed
retrospective study that included patients with molecu-
median; p=0.746) and sex (p=0.5501). There was also no by whole-exome sequencing. Results: We included 54
larly confirmed desminopathy from two national referral
difference between nadir (8 x 6 days; p=0.3979), facial patients from 42 families: 34 patients (23 families) with
centers (Brazil and Spain). Results: Thirty patients from
paralysis (p=1,000), sensitive alterations (p=0.2207), pres- MFMs, and 20 patients (19 families) with DMs. Among the
20 unrelated families were included. The mean age of dis-
ence of dysautonomia (p=0.1932), presence of anti-GM1 MFM families, variants in DES were the main cause (n=8),
ease onset was 25 years old. Cardiopathy was present in
(p=1.000) and demyelinating and axonal electrophysio- followed by TTN (n=4), FHL1 (n=3), FLNC (n=2), BAG3
86.7% of them, and in 14 patients, the cardiac symptoms
logical subtypes (p=0.1241). The 1-cycle IVIg group had (n=2), MYOT (n=1) and HNRNPA2B1 (n=1). Among the
preceded musculoskeletal weakness. From 21 available
less diarrhea (p=0.0002) and more proximal weakness DM families, DYSF (n=6) and GNE (n=4) were the most
biopsies, two distinct patterns of desmin aggregates were
(p=0.0051). The 2-cycles IVIg group had more global frequent disease-causative genes, followed by MYH7
identified: subsarcolemmal well-demarcated aggregates
weakness (p=0.0141) and tracheostomy (p=0.0077). The (n=2), NEB (n=2), TTN (n=1), ANO5 (n=1), RYR1 (n=1),
(in eight patients) and aggregates with diffuse and poorly
ability to walk at 6 months was similar (48X13 cases; and DNM2 (n=1). The severity of motor impairment
delimited borders (in 13 patients). Pathogenic variants
p=1,000). However, the median time to walk was shorter occurred in a similar proportion between distal myopa-
in the 1B segment and the tail domain presented cardi-
in the 1-cycle IVIg group (31 x 90 days; p=0.0093), as was thy and myofibrillar myopathy, being more pronounced
opathy and the proximal distribution of weakness at the
the time to gain 1 point on the Hughes disability scores in patients with variants in the BAG3, FHL1, MYH7 and
onset of symptoms more frequently when compared with
(14 x 40, p=0.0004), the time on mechanical ventilation ( GNE. Myofibrillar myopathies had a higher proportion and
patients with variants in other segments of the desmin.
13 x 34; p = 0.0001) and the days of Hospitalization (43 x severity of cardiorespiratory impairment. DES myopathy
All patients with mutations in the 1B segment presented
59.5; p = 0.0014). Conclusion: This observational study was related to more frequent cardiopathy. BAG3 myopathy
the well-demarcated desmin aggregate pattern, and no
did not observe better results after a second cycle of IVIg was an early and severe subgroup with axial contractures,
patients with variants in other segments showed this his-
in GBS under mechanical ventilation. The absence of a cardiac and respiratory impairment. Child FHL1 patients
tological arrangement. Conclusion: We suggest a pheno-
history of diarrhea and the presence of proximal weak- presented with inflammatory histology, and adult female
type-genotype correlation that associates the presence of
ness were significantly present in the 1-cycle IVIg group, presented a scapuloperoneal phenotype. Miyoshi pheno-
variants in the 1B segment and the tail protein domain
suggesting a good prognostic marker. type was seen in dysferlinopathy and anoctaminopathy.
with a more prominent cardiac presentation and a proxi-
GNE myopathy, in turn, presented with involvement of
mal-onset weakness distribution. Additionally, we found
the anterior leg muscles. Some genes related to congenital
a morphotype-genotype correlation, with variants in the
Doenças Neuromusculares 1B segment causing well-demarcated subsarcolemmal
myopathies were also causes of DMs (TTN, RYR1, DNM2)
and were confounded with hereditary neuropathy during
desmin aggregate formation. These findings may be use-
the first years of disease, although we only noted concom-
ful for identifying more severe patients, thus optimizing
itant peripheral nerve involvement in patients with BAG3
clinical care in this subgroup.
myopathy. Conclusion: DMs and MFMs comprise het-
erogeneous groups, but their phenotypic characteristics
Doenças Neuromusculares allow relative categorization into subgroups. Recognizing
these myopathies is important due to their potential sever-
ity, early cardiac complications, and respiratory failure,
which can lead to preventable death.

Doenças Neuromusculares

255
XXX Congresso Brasileiro de Neurologia

TL 1106195 TL 1106231 TL 1106270

USEFULNESS OF ULTRASOUND IN COMPARING HORMONAL EFFECT IN EFFECT OF THE COVID-19 PANDEMIC


DETECTING MUSCLE INVOLVEMENT SYMPTOMS IN MYASTHENIA GRAVIS AND ON PATIENTS WITH INHERITED
PATTERN IN POMPE DISEASE CONGENITAL MYASTHENIC SYNDROMES NEUROMUSCULAR DISORDERS
André Macedo Serafim Silva, Ana Lucila Moreira, José Pedro Soares Baima, Eduardo de Paula Cristiane Araujo Martins Moreno, Clara Gontijo
Edmar Zanoteli Estephan, Ana Flávia Pincerzo Pouza, Antônio Camelo, Pedro Henrique Marte de Arruda Sampaio,
Alberto Zambon, Edmar Zanoteli Alulin Tacio Quadro Santos Monteiro Fonseca,
Faculdade de Medicina da Universidade de São Eduardo de Paula Estephan, André Macedo Serafim
Paulo. São Paulo SP, Brazil Hospital das Clínicas da Faculdade de Medicina da Silva, Renann Nunes Pirola, Marcos Vinícius Oliveira
Universidade de São Paulo Marques, Karlla Danielle Ferreira Lima, Edmar
macedosera@gmail.com
jpedrobaima@gmail.com Zanoteli
Background: Pompe disease is a treatable autosomal Hospital das Clínicas da Faculdade de Medicina da
recessive disorder caused by deficiency of acid alfa-glu- Background: Fluctuations due to hormonal alterations Universidade de São Paulo
cosidase. Two classical phenotypes are described: the are known features of myasthenic syndromes. In auto-
infantile and the late-onset forms. Among late-onset immune myasthenia gravis a reasonable explanation of mvom13@gmail.com
Pompe disease, most patients present with a limb-gir- this phenomenon is hormonal influence in autoimmune
dle weakness pattern, although axial involvement is response, as usually seen in other autoimmune disorders. Background: The COVID-19 pandemic has brought sub-
common. Muscle ultrasound may evaluate clinical and Congenital myasthenic patients also experience these stantial challenges to current practices in treating hered-
subclinical involvement of muscles, identifying some fluctuations, but their syndrome is not associated with itary neuromuscular disorders (hNMDs). However, this
particular distribution pattern, and acting as adjunct to autoimmune mechanisms. Therefore, the influence of infection has not been the only concern for these patients.
the physical exam. Objective: To describe the pattern of symptoms might be due to other mechanisms of neu- Social distancing has compromised multidisciplinary
muscle involvement by ultrasound, including the tongue, romuscular junction impairment. Objective: Evaluate assistance and physical activity and has brought about
paravertebral and diaphragm muscles in patients with whether there are differences in menstrual period, preg- several mental health issues. We presented a follow-up
Pompe disease. Methods: Eight patients with molecular nancy, or menopause in patients with congenital myas- on 363 patients with hNMDs at a brazilian tertiary center
diagnosis of Pompe disease were clinically assessed and thenic syndromes and autoimmune myasthenia gravis. during the peak of the COVID-19 pandemic. Objective:
underwent muscle ultrasound using the setting for mus- Methods: data of patients were all obtained by phone call We aimed to show the frequency and severity of SARS-
cle evaluation with a fixed gain (Sonosite Edge, Fujifilm) and outpatients visits, and then compared between two CoV-2 infection among hNMD patients and to demon-
with a 7-15MHz transducer. The followings segments were groups by Fisher’s test. Results: A total of 34 participants strate the effects of the pandemic on life habits, disease
assessed: tongue, upper and lower limb (mostly for prox- with autoimmune myasthenia gravis (22 seropositivity for progression, and multidisciplinary supportive care sta-
imal muscles), abdomen, diaphragm and paravertebral anti-acetylcholine antibody was present in 22 patients, and tus. Methods: Three hundred and sixty-three patients
muscles. For the diaphragm we measured the thickness two for anti-Musk) and 27 participants with congenital (58% male and 42% female) were followed for three
during expiration and forced inspiration. All ultrasound myasthenic syndromes (15 CHRNE, 2 COL13A1, 2 GFPT1, months through three teleconsultations during the peak
images were obtained and scored based on Heckmatt’s 2 GMPPB, 1 RAPSN, 1 COLQ, 2 DOK7, 1 CHRNA1, 1 MUSK) of the COVID-19 pandemic in Brazil. Results: There were
rating scale. Results: Five from eight patients were male. were interviewed. Mean age was 42.8, MG-ADL median decreases in the numbers of patients who underwent
The mean age was 42 years old, with 10 years of disease score was 4.7 and pregnancy rate 1.5, in the autoimmune physical, respiratory, and speech therapies. For several
duration. Only one patient complaint of dysphagia and group. In the congenital group the mean age was 34.6, patients, their appetite (33%) and sleep habits (25%)
five reported dyspnea. The most involved and weakest MG-ADL median score 5.1 and pregnancy rate 1.5. There changed. Physical exercises and therapies were inter-
muscles were abdominal (mean MRC 2), gluteus maxi- were no statistical differences of worsening rates during rupted for most of the patients. They reported new-on-
mus (mean MRC 2) and gluteus medius (mean MRC 3), menstrual period and anticonception medications or set/worsening of fatigue (17%), pain (17%), contractions
and adductors (mean MRC 2.5). On ultrasound the most improvement after menopause. Pregnancy in patients (14%) and scoliosis (7%). Irritability and sleep, weight and
affected muscles were abdomen, vastus intermedius, with congenital myasthenia resulted in improvement in appetite changes, and especially diminished appetite
adductors and lumbar paravertebral muscles, scaled as 55% of patients compared to 70% of worsening symptoms and weight loss, were more frequent in the group that
Heckmatt 3 in virtually all patients. We also observed a in myasthenia gravis. Congenital myasthenic patients reported disease worsening. There was a low COVID-19
pattern of brachialis more involved than biceps brachii, were significantly more likely to have symptoms improved contamination rate (0.8%), and all infected patients had
and vastus intermedius more affected than rectus femoris during pregnancy compared with autoimmune patients a mild presentation. Conclusion: The isolation by itself
in all patients. Five patients had imaging involvement of the (p = 0.018). Conclusion: Most results showed that no was protective from a COVID-19 infection perspective.
tongue without any complaint of dysphagia. Considering statistical differences could be observed in patients with However, this isolation might also trigger a complex sce-
diaphragm, seven patients had high echointensity, and congenital myasthenia or myasthenia gravis. An excep- nario with life habit changes that are associated with an
all patients with Heckmatt 3 had a thickness ratio (con- tion was the improvement in symptoms during pregnancy unfavorable course for the NMD.
sidering inspiration and expiration thickness) lower than in congenital myasthenic women. This finding suggests
30%, which was considerably lower than in Heckmatt 2 that hormonal changes related to pregnancy may favor
patients (which had a thickness ratio between 30 and neuromuscular junction function but may also have an Doenças Neuromusculares
60%). Conclusion: Muscle ultrasound was able to detect unfavorable effect on immunological attack to neuro-
a recurrent pattern of muscle compromise in all patients. muscular junction. These conflicting events would have a
Subclinical involvement of the tongue and diaphragm neutral, or even deleterious resulting effect on symptoms
may help early disease recognition and predict potential of autoimmune myasthenic patients. On the other hand,
respiratory complication. congenital patients, who do not present immunological
disorder, would be subject only to the beneficial effects.

Doenças Neuromusculares
Doenças Neuromusculares

256
XXX Congresso Brasileiro de Neurologia

TL 1106271 TL 1106289 TL 1106300

SEROPATHOLOGICAL CORRELATION IN ROLE OF MUSCLE BIOPSY IN GENETIC ERA A UNIVERSITY NEUROMUSCULAR CENTER
PATIENTS WITH INFLAMMATORY MYOPATHY: EXPERIENCE IN CHRONIC AUTOIMMUNE
Marcílio José Oliveira Filho, Lígia Rufino Silva, Pedro
A COHORT STUDY Nogueira Fontana, Carolina Cunha Correia DEMYELINATING POLYNEUROPATHY
DIAGNOSTIC WORKUP
Vinicius Gomes Silva, Pedro Nogueira Fontana, Universidade de Pernambuco. Recife PE, Brazil
Natalia Merten Athayde, Roseli Corazzini, Ana Isadora Versiani, Matheus Nunes Ferreirinha Leite
Marina Dutra, Carolina Cunha Correia, Helen Maia oliveirafilho.m@uol.com.br Castro, Mariana Soares Freitas Tamy, Priscila
Tavares Andrade, Paula Fratini, David Feder, Alzira Santos Mageste, Raimundo Marcial Brito Neto,
Alves Siqueira Carvalho Background: Innovations in genetic testing have revolu- Carolina Braga, Bruno Coutinho, Camila Pupe,
tionized the diagnosis of hereditary myopathies. However, Osvaldo Nascimento
Centro Universitário FMABC . Santo André SP, Brazil the detection of variants of uncertain significance makes
Hospital Universitário Oswaldo Cruz. Recife PE, the interpretation of some cases difficult. In addition, Universidade Federal Fluminense. Niterói RJ, Brazil
Brazil hereditary myopathies can mimic acquired diseases that
Pontifícia Universidade Católica de Campinas. isadora.versiani@gmail.com
have specific treatment, such as inflammatory myopa-
Campinas SP, Brazil thies. Thus, muscle biopsy may have a defining role in the
Background: Chronic autoimmune neuropathies are
vinicius.gomes@outlook.com.br diagnosis of myopathies. Here we present three cases in
a heterogeneous spectrum of disorders with distinct
which the muscle biopsy allowed a diagnostic conclusion
pathophysiology, phenotypes and treatment. This group
Background: Muscle biopsy have been considered the by complementing or guiding the genetic evaluation. Case
accounts for both economic and quality of life burden.
gold standard pattern for the diagnosis of idiopathic 1: female infant with neonatal hypotonia and delayed
Misdiagnosis remains common, especially for chronic
inflammatory myopathies (IIM). Myositis-specific anti- motor development. NGS panel revealed heterozygous
autoimmune demyelinating polyneuropathy (CIDP)
bodies (MSA) and myositis-associated antibodies (MAA) VUS in the RYR1 gene. The biopsy showed signs of neu-
variants, which is why diagnostic criteria have recently
have been shown to play an important role in diagnosis rogenic disease. New genetic testing revealed homozy-
been reviewed. Widespread interest in immune neurop-
and prognosis. Methods: Describe a cohort of IIM, cor- gous pathogenic variant in the SMN1 gene, concluding
athies relies on the fact that they are treatable conditions.
relating histopathological and immunohistochemical with the diagnosis of spinal muscular atrophy. Case 2: 4 year
Objective: An analysis of our university Hospital cohort of
serological findings (MSA and MAA). Results: Out of 44 old boy with neonatal hypotonia, muscle weakness, dys-
patients presenting with CIDP variants, aiming to contrib-
patients, 40 tested for MSA or MAA and 38 were underwent phagia and respiratory distress. Exome sequencing found
ute to diagnostic workup debate. Methods: A cohort of 46
muscle biopsy, as the following: 15 (39%) immune-me- no pathogenic variants that would justify the clinical
suspected CIDP patients was identified in our neuromus-
diated necrotizing myopathy (IMNM), 8 (21%) dermato- picture. Muscle biopsy showed myotubular myopathy.
cular outpatient clinic, after thorough chart review from
myositis (DM), 4 (10.5%) perimysial myopathy (PMM), A microarray detected a deletion in MTM1gene, which
2018 to June/2022. Patients underwent analysis based on
3 (7.9%) immune-mediated myopathy (IMM), 2 (5.2%) causes X-linked myotubular myopathy. Case 3: 12 year old
EFNS/PNS diagnostic criteria. Results: Out of 46 suspected
lipid-storage myopathy (LSM), 5 (13%) other myopathies male, with frequent falls, gait alteration and progressive
acquired demyelinating neuropathy patients, 9 fulfilled
and 1 (2.6%) inclusion body myositis. A total of 25 cases muscle weakness. Genetic investigation of symptomatic
criteria for multifocal motor neuropathy and 2 were diag-
(62.5%) were seropositive for the following antibodies: 6 relatives did not clarify the diagnosis. In muscle biopsy,
nosed with metabolic neuropathy, and were all excluded
(24%) anti-SRP, 5 (20%) antiHMGCR, 3 (12%) anti-Ro52, several reducing bodies were identified. Genetic testing
from further analysis. Out of 35 remaining patients, 14 are
2 (8%) anti-Jo1, 2 (8%) anti-Mi2, 2 (8%) anti-Ku, 1 (4%) detected a deletion in the FHL1 gene, which causes, in
female and 21 male. 12 patients presented with relapsing
anti-Jo1 + anti-Ro52, 1 (4%) anti-SRP + anti-Ro52, 1 (4%) addition to reducing body myopathy, Emery-Dreifuss
forms, 3 progressive, 11 secondary progressive, 8 clinical
anti-SAE1 + anti-Ro52 and 1 (4%) anti-SAE1 + anti-Ro52 muscular dystrophy type 6, X-linked myopathy with
remission and 1 still unidentified. According to 2021 ENFS/
+ anti-Ku. Among the IMNM, 5 (33.3%) were antiSRP postural muscle atrophy and X-linked scapuloperoneal
PNS electrodiagnostic criteria, 20 patients were classified
positive (+), 3 (20%) anti-HMGCR+, 1 (6.6%) anti-Ku+, 1 myopathy. Discussion: The case 1 shows limitations of
as definite CIDP and 7 as possible CIDP. 8 did not fulfill
(6.6%) anti-Ro52+ and 1 (6.6%) anti-SRP+ and anti-Ro52+, the exome as a tool for genetic diagnosis, as the reading
criteria. Twenty three patients presented with typical
(sensitivity: 60%). The DM subtypes 1 (12.5%) tested posi- depth can be variable and sometimes insufficient. In
CIDP, 2 with multifocal CIDP, 1 with focal CIDP and 1
tive for anti-Mi2, 1 (12.5%) anti-SRP, 1 (12.5%) anti-NXP2, case 2, the exome did not detect the pathogenic variant
with sensory CIDP. Median time for diagnosis after initial
1 (12.5%) anti-SAE1 and anti-Ro52 and 1 (12.5%) anti- because it is a copy number variation; the biopsy directed
symptoms was 53 months. Conclusion: Accurate diagnosis
SAE1 and anti-Ku + anti-Ro52. Regarding the 4 cases of the microarray to the region of the X chromosome where
and full understanding of different clinical presentations
PMM, 2 (50%) were positive for anti-Jo1 and 1 (25%) for the suspected gene is located, confirming the diagnosis. In
guide therapeutic management and prognosis. They range
anti-Mi2. Among IMM cases, 1 (33.3%) was positive for case 3, the final diagnosis was guided by the histopatho-
from multifocal to diffuse, from sensory to motor, from
anti-Ku and 1 (33.3%) for anti-Jo1 and anti-Ro52. In LSM, logical finding, which suggested the causative gene; this
benign to severe and from relapsing to progressive forms.
1 (50%) tested positive for anti-HMGCR. Conclusion: The gene, however, can cause different myopathies, and it is
Out of our cohort of clinically suspected and treatment
positive test for MAA and MSA occurred in 62.5% of our only possible to define the type through muscle biopsy.
responsive patients, 77.14% fulfilled criteria for possible
sample, demonstrating sensitivity above that reported Conclusion: In genetic era, muscle biopsy may play a
diagnosis of CIDP, which reinforces importance of sup-
in the literature (50%). Anti-HMGCR and anti-SRP anti- role in diagnosing hereditary myopathies when genetic
portive criteria when diagnosis is uncertain. Sensibility
bodies were highly specific and sensitive for IMNM, testing is inconclusive.
may be reduced for focal and sensory forms, besides severe
while anti-Jo1 showed a correlation with PMM. On the cases with axonal loss. Most frequent clinical course was
other hand, anti-Ro52 proved to be the less specific. Lipid relapsing. 85.2% of patients showed typical presentations.
Doenças Neuromusculares
accumulation in anti-HMGCR myopathy was probably a CIDP diagnosis remains a challenge, which is evidenced
secondary finding. The positive case for anti-Mi2 in 1 case by the prolonged time lapse between initial symptoms
of PMM points out to the possibility of DM diagnosis in this and definite diagnosis.
subgroup. The presence of MAA in the nonspecific IMM
group highlights the value of testing theses autoantibodies
in cases only suggestive of a immune process. Overall, the Doenças Neuromusculares
future will decide how important these antibodies are to
replace or not the muscle biopsy in IIM.

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257
XXX Congresso Brasileiro de Neurologia

TL 1106352 TL 1106380 TL 1106408

STRUTUCTURAL DAMAGE IN GENETIC PANEL TESTING’S DIAGNOSTIC ELECTROPHYSIOLOGICAL FINDINGS IN A


DYSTROPHINOPATHIES: A MULTIMODAL ACCURACY FOR HEREDITARY SAMPLE OF GENETICALLY CONFIRMED
NEUROIMAGING STUDY NEUROMUSCULAR DISEASES IN HOSPITAL BRAZILIAN CITIZENS WITH CANVAS
UNIVERSITÁRIO ANTÔNIO PEDRO
Mariana Rabelo de Brito, Thiago Junqueira R. Manoella Guerra de Albuquerque Bueno, Pedro José
Rezende, Cristina Iwabe, Gabriella Conte, Anamarli Lorena Izabel Esteves Adolphsson, Larissa Lorrhan Tomaselli, Fernanda Barbosa Figueiredo, Paula
Nucci, Fernando Cendes, Marcondes Cavalcante Santos Neves, Helen Maria de Azevedo, Camila Camila Alves, Orlando Barsottini, Alberto Rolim
França Jr. Branco Pupe, Oswaldo Nascimento Muro Martinez, Gabirel Schmitt, José Luiz Pedroso,
Marcondes Cavalcante França Junior, Wilson
Universidade Estadual de Campinas. Campinas SP, Universidade Federal Fluminense. Niterói RJ, Brazil Marques Junior
Brazil
lorenaadolphsson@id.uff.br Hospital das Clínicas da Faculdade de Medicina
mariana_rabelo_@hotmail.com de Ribeirão Preto da Universidade de São Paulo.
Background: Neuromuscular disorders (ND) represent Ribeirão Preto SP, Brazil
Background: Dystrophinopathy is a X-linked recessive an important fraction of neurological diseases. They affect Faculdade de Medicina de Ribeirão Preto da
progressive neuromuscular disorder characterezed by the peripheral nervous system, which comprehends a Universidade de São Paulo. Ribeirão Preto SP, Brazil
muscle weakness. Dystrophinoapthies are also character- vast spectrum of syndromes and etiologies. In this regard, Universidade Federal de São Paulo. São Paulo SP,
ized by central nervous system damage, but pattern and hereditary NDs are characterized by an early age manifes- Brazil
extent of such damage are not yet clear. For this reason, we tation, family history and progressive loss. Furthermore, Universidade Estadual de Campinas. Campinas SP,
designed a cross-sectional multimodal MRI-based study the diagnosis is challenging and of exclusion, naturally Brazil
to address this point. Methods: Mean age of patients and requiring a detailed investigation and complementary
disease duration were 12 and 8, respectively. We enrolled exams, such as and beyond genetic sequencing and elec- manoellagab@gmail.com
50 patients (40 duchenne and 10 becker) and 40 age-sex tromyography. Owing to the diagnostic’s difficulty, the
matched controls. All subjects underwent MRI in a 3T genetics tests are value resources when it comes to iden- Cerebellar ataxia, neuropathy and vestibular areflexia
device to assess gray (GM) and white matter (WM). To tifying the disease, allowing a better understanding and syndrome (CANVAS) is a late-onset, slowly progressive
evaluate the cerebral and cerebellar cortices, we used orientation of the patient’s symptoms and outcomes. This neurological disorder. Despite recently described, its
measures from FreeSurfer. DTI-multiatlas was used to ongoing study aims to specify the most correlated factors etiopathogenesis is already in the process of elucidation
investigate microstructural-abnormalities in cerebral WM. with the test’s positivity. In addition, a decisive algorithm due to the identification of a biallelic intronic AAGGG
All analyses were corrected for multiple comparisons. for the genetic approach will be established, along with repeat expansion in the RFC1 gene as the genetic cause
Results: Group analyses showed WM microestructural the patient’s sample characterization. To that end, this is of the disease. Meanwhile, electrophysiological findings
abnormalieties at bilateral fornix, being characterized by an observational retrospective and descriptive current are still to be completely described as patients receive
increased axial, medial and radial difussivities, with large study related to genetic test’s accuracy. Patients included their definitely diagnosis. Based on these findings, this
effect sizes (>0.8) for all significant diffusion parameters. will have hereditary ND’s suspicion, age over 18 years and study evaluated nerve conduction study and electro-
We also found a negative correlation between axial dif- other etiologie excluded. In total, 54 patients matched myography findings of genetically confirmed CANVAS
fusivity and Motor Functional Measure scale (r=-0.338, the previous criteria and preliminary Results: show that patients from a single brazilian center. In order to genet-
p=0.022) of such structure. Regarding volumetric anal- 51% are women and 29,6% are between 53-62 years. The ically identify this expansion, flanking polymerase chain
ysis, patients showed volumetric reduction of both left hereditary ND was identified in 33% of the patients, yet reaction (PCR) and repeat-primed PCRs (RP-PCR) for the
thalamus and cerebellar cortex. Conclusion: Patients a Charcot-Marie-Tooth phenotype was most prevalent pathogenic allele expansion configuration in RFC1 were
with dystrophinopathies are characterized by structural with mutation of the PMP22, DNM2 and SH3TC2 genes. performed in patients with at least one clinical feature of
damage at fornix, thalamus and cerebellar cortex. Such Conclusion: Therefore, genetic testing is an important the Syndrome. If flanking PCR did not show any prod-
structural signature helps us to understand some phe- instrument for hereditary ND’s diagnosis and disease uct, the DNA sample would undergo RP-PCR. Biallelic
notypic traits in this disease. Our results also shed some specification. In consequence, the patient experiences AAGGG expansion was found in nineteen patients of the
light in the dystrophinopathy pathogenesis and highlight an improvement in the orientation and outcome’s antic- sample group (18%). Subsequently, confirmed CANVAS
potential neuroimaging marker for therapeutic trials. ipation, besides an elementary comfort in knowing one’s patients were submitted to electrophysiological studies
disorder. However, genetic panel testing doesn’t ensure (nerve conduction study and electromyography). Fifteen
precise diagnosis and, as a result, a wide range of patients out of the nineteen confirmed cases were submitted to
Doenças Neuromusculares continues without an answer. For this reason, studying electrophysiological evaluation. Only one patient had
those tests accuracy is mandatory, highlighting hotspots a normal electrophysiological study and clinically pre-
in need of improvement. sented with cerebellar ataxia related symptoms alone. Six
patients (40%) had motor involvement associated with
neuropathy. Notably, eight patients (53%) had exclusively
Doenças Neuromusculares sensory involvement and primarily axonal deterioration
for most cases. Additionally, 6 patients were submitted
to the blink test during the exam and 3 (50%) had the R2
reflex impaired. With regards to autonomic evaluation,
four patients were evaluated for cutaneous response
for electric, respiratory and sound stimuli. One of these
patients showed diminished response to the electric
stimulus. Ultimately this study aims to contribute to the
understanding of the electrophysiological findings of the
CANVAS patients in the brazilian population.

Doenças Neuromusculares

258
XXX Congresso Brasileiro de Neurologia

TL 1106411 TL 1106416 TL 1106424

CLINICAL MANIFESTATION OF NEBULIN- CORRELATION BETWEEN AGE, TRIPLE-FURROW TONGUE ATROPHY IN


ASSOCIATED NEMALINE MYOPATHY RESPIRATORY AND MOTOR FUNCTION NEMALINE MYOPATHY: A NOVEL FINDING
IN CHILDREN WITH LAMA2 CONGENITAL THAT CHANGES MANAGEMENT CARE
Cristiane Araujo Martins Moreno, Mariana Cunha
Artilheiro, Alulin Tacio Quadros Santos Monteiro MUSCULAR DYSTROPHY
Cristiane Araujo Martins Moreno, Clara Gontijo
Fonseca, Clara Gontijo Camelo, Andre Macedo Mariana Cunha Artilheiro, Clara Gontijo Camelo, Camelo, Alulin Tacio Quadros Santos Fonseca,
Serafim Silva, Luiz Dalfior Junior, Umbertina Conti Tatiana Ribeiro Fernandes, Michelle Abdo Paiva, André Macedo Serafim Silva, Luiz Dalfior Junior,
Reed, Edmar Zanoteli Cristiane de Araujo Martins Moreno, André Macedo Umbertina Conti Reed, Edmar Zanoteli
Universidade de São Paulo. São Paulo SP, Brazil Serafim Silva, Umbertina Conti Reed, Edmar
Universidade de São Paulo. São Paulo SP, Brazil
Zanoteli
Hospital Santa Marcelina. São Paulo SP, Brazil
cristianeammoreno@gmail.com
Hospital das Clínicas da Faculdade de Medicina da
cristianeammoreno@gmail.com
Background: Nemaline myopathy (NM) is a genetically Universidade de São Paulo. São Paulo SP, Brazil
heterogeneous inherited myopathy related with at least michelle.abdo@hc.fm.usp.br Background: Inherited Nemaline myopathy (NM) is
twelve genes while pathogenic variants in NEB gene are a clinically and genetically heterogeneous myopathy.
the most common genetical cause. The clinical spectrum Background: LAMA2 Natural history studies have been Orofacial and bulbar muscles are commonly involved in
of NM caused by NEB pathogenic variants (NM-NEB) is increasing in the last years due to the new era of emerging NM patients. Objective: Previous data showed that one
very broad ranging from mild to severe presentations gene therapy. In this type of congenital muscular dystro- third of patients needed feeding tube and that dysphagia
manifesting with generalized weakness, respiratory and phy, the age is associated with poor respiratory and motor might lead to recurrent pulmonary. However there is a
bulbar involvement. Currently there is not enough data function. A better understanding of how age relates to the lack of data characterizing such a common comorbid-
regarding the progression disease. Objective: Here we course of respiratory and motor aspects can contribute ity. Methods: We evaluated patients with NM molecu-
present a mutational and phenotypic spectrum of 33 NM to multidisciplinar management and treatment for this larly and/or histologically diagnosed and stratified them
patients caused by NEB variants (NM-NEB) classified children. This study aimed to investigate the relationship according to swallow function using the Neuromuscular
according to age groups and the use of ventilatory sup- between age, respiratory and motor function in LAMA2 Disease Swallowing Status Scale (NdSSS). Results: 48
port. Methods: Clinical and genetic data from NM-NEB children. Methods: Eighteen children (11.4±5.8 years; 11 patients were seen, 33 were caused by NEB, 10 by ACTA1,
patients followed in one specialized center were collected male\7 female; 7 with nocturnal noninvasive ventilation) 2 by TPM2 variants and 3 had no defined genetic cause. All
through regular consultations. Thirty-three patients were had the forced vital capacity percentage (FVC) and cough patients presented high arched palate and most of them
evaluated regarding motor, bulbar and respiratory func- peak flow (CPF) measured by spirometer and peak flow had facial weakness, less pronounced in the two patients
tion. Results: 32% of the NM-NEB patients used a G-tube, meter, respectively, and the motor function evaluated by with childhood onset. Neonatal dysphagia and failure to
65% were able to walk unsupported and 45% did not used three domains and total score of Motor Function Measure thrive was referred by 68% of the patients, half of them
ventilatory support (VS). Scoliosis and dysphagia were (MFM) – 32. The Shapiro?Wilk test demonstrated that improved in the next few years of life. At the evaluation,
more common among patients that used VS. Comparing the data were non-normally distributed. The relation- around 30% used feeding device, but 20% additional,
the patients grouped by age we found that proportionally, ships between respiratory and motor functions and also despite eating by mouth, needed to adapt their food and
older patients had more scoliosis and respiratory dysfunc- between age and respiratory and motor functions were take supplements. Comparing ACTA1 with NEB patients,
tion than younger groups suggesting the progression of the tested by Spearman correlation test. The correlation dysphagia was more common among the ACTA1 group.
disease in these domains. In addition to that, we showed coefficients were classified as strong (r?0.70), moderate We described a novel finding which is tongue atrophy in
that ventilatory support use was associated with scoliosis (0.40 ? r < 0.70), or weak (r ?0.40). Results: FVC strongly a triple furrow pattern. The tongue atrophy was present
and dysphagia. Discussion: NM-NEB is a very debilitat- correlated to MFM-D2 (axial and proximal function) 50% of the accessed patients, but it was more frequent in
ing disease. There is an association between scoliosis and and moderately correlated to MFM-D1 (standing and the dysphagic patients (83%). Discussion: Dysphagia is
respiratory dysfunction while patients using VS have more transfers function) and to MFM-total. No correlation was a very common disability caused by NM myopathy and
often scoliosis than the no-VS group. Grouping patients found between CPF and motor function items, but CPF is present in around 50% of the patients, being more fre-
by age suggested disease stability in motor and swallow was moderately correlated to age. Age strongly correlated quent in patients with ACTA1 variants. Tongue atrophy
function but a progression in respiratory dysfunction and to MFM-D2 and moderately correlated to MFM-D1 and is a novel finding which is associated with dysphagia and
skeletal deformities. All these observations are relevant to MFM-total. Conclusion: A good pulmonary function can help us to stratify patients needing a more detailed
in the management care of NM-patients. (FVC) was positively correlated to high motor function to bulbar assessment.
perform axial\proximal, standing and transfers activities.
Interestingly, CFP was not correlated to motor function,
Doenças Neuromusculares Doenças Neuromusculares
but was negatively correlated to age. Addictionaly, age was
negatively correlated to axial\proximal and total motor
function in LAMA2 children

Doenças Neuromusculares

259
XXX Congresso Brasileiro de Neurologia

TL 1106436 TL 1106445 TL 1106458

MOLECULAR INVESTIGATION USING NGS MITOCHONDRIAL MYOPATHY CAUSED BY PHYSICAL EXERCISE IN PATIENTS WITH
FOR INHERITED MYOPATHIES : EXPERIENCE TK2 DEFICIENCY DEMYELINATING DISEASES
OF A BRAZILIAN TERCIARY CENTER
Cristiane Araujo Martins Moreno, Clara Gontijo Louisy Carvalho Araújo, Fernando de Paiva Melo
Cristiane Araujo Martins Moreno, Andre Macedo Camelo, Valeria Nogueira Tobias Granja, Elizabeth Neto, Camila de Araújo Toscano, Raquel Minervino
Serafim Silva, Clara Gontijo Camelo, Alulin Tácio Silveira-Lucas, Mariana Cunha Artilheiro, Umbertina de Carvalho Sobrinha, Rayana Tavares de Queiroz,
Quadros Santos Monteiro Fonseca, Eduardo de Conti Reed, Edmar Zanoteli Davi Veloso Guerra, Bianca Etelvina Santos de
Paula Estephan, Pedro Henrique Marte de Arruda Oliveira
Universidade de São Paulo. São Paulo SP, Brazil
Sampaio, Umbertina Conti Reed, Edmar Zanoteli
Hospital Geral do Estado de Alagoas. Maceió AL, Centro Universitário de João Pessoa. João Pessoa
Universidade de São Paulo. São Paulo SP, Brazil Brazil PB, Brazil
Hospital Moinhos de Vento. Porto Alegre RS, Brazil Fundação Centro Integrado de Apoio à Pessoa com
cristianeammoreno@gmail.com Deficiência FUNAD
cristianeammoreno@gmail.com
Background: Inherited myopathies represent a large and louisyaraujo@gmail.com
variable group of disorders. The age of onset ranges from Background: The nuclear gene TK2 encodes the mito-
neonatal presentations to late-adult forms. Most of the chondrial thymidine kinase, an enzyme involved in the Background: People with demyelinating diseases, such as
conditions can be caused by multiple genes, making the phosphorylation of deoxycytidine and deoxythymidine Multiple Sclerosis (MS) and Neuromyelitis Optica (NMO),
unique gene testing unfeasible. Advances in next gen- nucleosides. Biallelic TK2 mutations lead to mitochondrial despite the evidence of benefits, have reduced the prac-
eration sequencing techniques and the participation of DNA instability and are associated with a wide clinical tice of physical exercises, given the fear of worsening the
industry in providing no-cost panels has made this genetic spectrum of disease (TK2d). Current classification includes symptoms. This increases physical disability and worsens
evaluation a new reality in Brazil. Objectives: Our aim is to infantile (? 1 year), childhood- (1-12 years), and late-onset stability, in addition to reducing muscle strength, mobil-
present the experience of the use of these technologies in (?12 years) forms. These forms differ for progression and ity and quality of life. In this context, some studies have
a terciary center and present the gene distribution among life expectancy. Deoxynucleosides reposition were shown demonstrated beneficial results of aerobic training on
the main groups of inherited myopathies: congenital to be effective to disease controls in a compassionate brain gray matter volume and on the white matter tract
muscular dystrophy (CMD), congenital myopathy (CM), use. Objectives: Here we report the clinical findings and unit, in addition to greater functional connectivity of the
congenital myasthenia (CMS), mitochondrial myopathies mutational spectrum of a series of brazilian cases pre- hippocampus and cortex. Objectives: To analyze the prac-
(MiM), metabolic myopathies (MeM), myofibrilar and dis- senting infantile and childhood-onset TK2d. This report tice of physical exercise by patients with demyelinating
tal myopathies(M_DM), limb-girdle muscular dystrophy characterized patients with TK2-deficiency enhancing diseases in the State of Paraíba. Methods: A retrospective,
(LGMD). For analysis purposes we included distrophi- the importance of a clinical recognition of patients with cross-sectional study carried out by reviewing the medical
nopatly patients with the LGMD. Results: We evaluated a severe, potentially lethal, and potentially treatable con- records of 115 patients, selected in a non-probabilistic
427 patients and performed panel for 249 patients and dition. Methods: Patients with TK2d were evaluated in manner and for convenience, with demyelinating dis-
whole exome sequencing for 178 patients presenting a Terciary Center. We presented clinical and molecular eases in a Multiple Sclerosis center, using the variables
diagnostic rates of 69% for the exomes and 66% for the findings from 8 patients (6 families) harboring pathogenic sex, age, Body Mass Index (BMI), diagnosis, frequency
panels. We identified a genetic cause for the condition variants in TK2 gene. Results: Four patients presented the and category of physical exercise. Results: Among the
in 68% of patients with a hipotesis of canalopathy, 90% early-onset infantile form. Of those, three passed away still study participants, a distribution of 101 (87%) patients
of the CMD, 76% of the MC, 72% of the CMS, 16% of the in their first decade of life. P5 was 2 years old in the last with MS and 14 (13%) with NMO was observed. There was
MiM, 58% of the MeM, 46% of the M_DM and 71% of the evaluation, he had normal motor development until the a predominance of females (70%). In terms of age group,
LGMD. Discussion: We showed the performance of NGS age 11 months, when a weakness started. After 5 months 3% belonged to the age group under 16 years old, 9% from
technologies among many highly curated patients for a of the beginning of symptoms he had lost his gait, head 16 to 25 years old, 34% from 26 to 35 years old, 31% from
particular phenotype. We had higher diagnostic rates support and started on night-time non-invasive ventila- 36 to 45 years old; 10% from 46 to 55 years old; 10% from
for several groups of disorders such as CM, CMD, CMS tory support at age of 18. At 23 months he was hypotonic, 56 to 65 years old and 3% from those over 65 years old.
and LGMD than previously reported in the literature. We had generalized weakness and was requiring ventilation Regarding BMI, 3% had a thin BMI, 49% were eutrophic,
linked this finding to a continuous vigilance among new also for a couple of hours during the day. Five patients 32% were overweight and 16% were obese. Before the
genes in the literature and to the importance of the clini- presented the childhood form (mean of age average of 32 diagnosis of MS or NMO, 43% rarely practiced physical
cal phenotype stratification for the positive results. In the +- 8 years old). Despite the slow disease progression, all of exercise and 27% at least 5 days a week. After diagnosis,
opposite direction, we had lower diagnostic rates for MiM them noticed first symptoms in their first decade of life, it was observed that 24% of the patients increased the fre-
than previously reported, but mtDNA variants were not they had difficulties gaining weight and frequent episodes quency of physical exercises, 29% decreased it and 47%
yet checked. Conclusion: NGS has facilitated the diag- of diarrhea. They had severe proximal and axial weakness maintained the frequency. Before the diagnosis, 12% prac-
nosis in the inherited myopathies but requires a careful and started on NIVS around 20 years old. Four of them ticed Tension and Aerobic Exercises (TAE); 42% Aerobic
assessment of its limitations. A detailed clinical assess- used NIVS night-time only and one used for 15 hours/day. Exercise (AE); 18% Tension Exercises (TE); 28% did not
ment is very important to provide high diagnostic rates. Conclusion: With the advent of a specific treatment and practice activities. After diagnosis, 20% practice TAE; 41%
given the severity of TK2d, clinicians need to know how AE; 18% TE; 21% do not practice activities. Conclusion:
to recognize this disorder. The mortality rates are high A predominance of patients between 26 and 45 years
Doenças Neuromusculares mainly among the infantile presentation; however, child- (65%) and the presence of 48% of participants with an
hood-onset are also a debilitating condition. Ptosis and overweight or obese BMI is observed. Regarding the prac-
hypoacusis were not found in our series. But all patients tice of physical exercise, it is noted that 76% decreased
had proximal and axial weakness, global hypotrophy, or maintained the frequency, in addition, an increase of
dysphagia and respiratory involvement. 8% was observed in TAE practitioners and a decrease of
7% in individuals who do not practice physical exercise.

Doenças Neuromusculares
Doenças Neuromusculares

260
XXX Congresso Brasileiro de Neurologia

TL 1106469 TL 1106542 TL 1106551

LATER DIAGNOSIS OF DUCHENNE LONG-TERM FOLLOW-UP OF SMA TYPE 1 SAFETY AND EFFICACY OF GENE THERAPY
MUSCULAR DYSTROPHY IN BRAZILIAN TREATMENT WITH NUSINERSEN: A SINGLE- FOR PATIENTS WITH SPINAL MUSCULAR
PATIENTS: WHERE COULD WE IMPROVE? CENTER EXPERIENCE ATROPHY: A REAL-LIFE STUDY IN A
BRAZILIAN COHORT
Marco Antonio Veloso Albuquerque, Karlla Danielle Rodrigo Holanda Mendonca, Graziela Jorge Polido,
Ferreia Lima, Umbertina Conti Reed, Edmar Zanoteli Ciro Matsui Jr, Umbertina Conti Reed, Edmar Rodrigo Holanda Mendonça, Adriana Banzzatto
Zanoteli Ortega, Ciro Matsui Jr, Luis Fernando Grossklauss,
Hospital das Clínicas da Faculdade de Medicina da
Elizabeth Lemos Silveira Lucas, Edmar Zanoteli
Universidade de São Paulo. São Paulo SP, Brazil Faculdade de Medicina da Universidade de São
Paulo. São Paulo SP, Brazil Faculdade de Medicina da Universidade de São
karlla.dfl@gmail.com
Paulo. São Paulo SP, Brazil
holandamendonca28@gmail.com
Hospital Pequeno Príncipe. Curitiba PR, Brazil
Background: Duchenne muscular dystrophy (DMD) is
Hospital Samaritano Higienópolis
caused by a mutation in the dystrophin geneand is the Background: Spinal Muscular Atrophy (SMA) is a genetic
Hospital Infantil Sabará
most common form of childhood-onset muscular dys- motor neuron disease caused by mutations in the SMN1
Hospital Moinhos do Vento
trophy affecting approximately 1 in 3500 newborn boys. (Survival Motor Neuron) gene, which leads to hypoto-
The disease is progressive and most patients exhibit nia, muscle weakness and respiratory involvement. Its holandamendonca28@gmail.com
signs of muscle weakness before 6 years. Despite all the most severe form, SMA type 1, starts before 6 months of
advances in management and treatment of DMD over life and has a high mortality due to ventilatory failure. Background: Spinal Muscular Atrophy (SMA) is a genetic
the last decades, the mean age at diagnosis of DMD has Nusinersen, the first approved treatment for SMA, is an motor neuron disease caused by mutations in the SMN1
been reported to be around the age of 4.5-5 years in sev- antisense oligonucleotide for intrathecal use, which leads (Survival Motor Neuron) gene, which leads to hypoto-
eral countries with a delay of about 2 years between the to greater survival and gain in motor acquisitions. Studies nia and muscle weakness with high mortality related to
first symptoms are noted, and the diagnosis. Objective: on the safety and efficacy of long-term treatment are still respiratory involvement. Gene therapy (GT) (onasem-
This retrospective study had objective to investigate the scarce. Objective: To present long-term results (4 years of nogeno aberpavovec) for SMA, through an adeno-asso-
age at diagnosis of disease in a group of brazilian patients follow-up) in SMA type 1 patients under treatment with ciated viral vector 9 (AAV9) was recently approved in our
followed in a tertiary center. Results: We identified 122 Nusinersen. Methods: We followed a total of 24 patients, country, but its safety and efficacy outside the context
brazilian boys with phenotype/genotype compatible with all with SMA type 1 (20 patients with 2 copies of SMN2). of clinical trials is still poorly understood. Objective: To
DMD in the last 7 years (2014-2021). The most common The patients were evaluated by the functional scale CHOP- present early results regarding safety and efficacy in SMA
mutational event was represented by intragenic deletions, INTEND (The Children’s Hospital of Philadelphia Infant patients treated with GT. Methods: We followed a total
accounting for 58% (71/122) of all mutations. Duplications Test of Neuromuscular Disorders) and in relation to gain of 33 patients treated with GT for SMA from 6 months to
accounted for 14% and 20% of patients had a point muta- of motor milestones (head control, sitting with or without 1 year of treatment. The patients were evaluated by the
tion (including 12/122 with nonsense mutation). In 7 boys support, standing and walking), in addition to assessment functional scales CHOP-INTEND (The Children’s Hospital
(5%) was found an intronic mutation and in 2 the muscle of survival and use of permanent ventilation (PV) and of Philadelphia Infant Test of Neuromuscular Disorders)
biopsy confirmed absence or near absence of dystrophin feeding route. Results: Twelve patients were female, only and in relation to gain of motor milestones. In addition,
in the muscle. The mean age at onset of the disease was 11 patients (45.8%) started treatment before 12 months of assessment of survival and use of continuous ventilation
3,3 years. Frequent falls were the more frequent symptoms illness. 22 patients (91.6%) were already using gastrostomy (CV) was performed and also data regarding transaminase
(55/122) at initial clinical presentation. The mean age at at the beginning of treatment. After 4 years of follow-up, elevation, liver function, hematological data, elevation
diagnosis was 6,9 years. The age of diagnosis was defined 22 (91.6%) patients were alive, two deaths occurred: one of troponin and duration of corticosteroid use. Results:
as the age of molecular test was realized, confirming the after gene therapy and the other after respiratory failure. 33 patients were included, 26 SMA type 1 and 7 SMA
disease. Steroid therapy was initiated in 120/122 patients Two patients received gene therapy but continued to use type 2. The mean age at dosing was 18.5 months (14.0 –
(prednisolone 36/120 or deflazacort 84/120). The mean Nusinersen (combined therapy). Eight patients gained 23.2), with a mean weight of 9.9 kg (8.3 kg). – 16.3) and
age at start treatment with steroid was 7,3 years. Thirty- some motor milestone, all of them started treatment 28 patients (87.5%) were using nusinersen previously.
seven (30%) lost the capacity to walk until the last evalua- before 12 months disease. The greatest gains in CHOP- After 1 year of treatment 32 patients (96.9%) were alive,
tion. The mean age of lost was 10 years. In all patients, CK INTEND occurred up to 24 months of treatment, and after 7 patients (21.2%) remained on CV (>16h/day) versus
levels were markedly increased in the early stages of the this period, the scores tended to stabilize, without further 11 (33.3%) patients at dosing. Regarding the gain in the
disease. Conclusion: In this group of brazilian patients gains. 19 patients (79.1%) were already using PV (>16h/ CHOP-INTEND score, the mean baseline score was 30.50
with DMD, an important delay in diagnosis was observed, day) at the beginning of treatment and 15 patients were (19.50, 40.75) to 46 (40.00, 52.00) at 6 months and to 56
which led to a delay in the beginning of steroid therapy, using PV after 4 years of treatment. Even in those patients (50.00, 58.00) points at 12 months. Regarding motor mile-
when compared to the onset of initial symptom. This late who were on PV, there was a reduction in the duration of stones, from those with SMA type 1, nine patients (42.9%)
onset of therapy is probably related to an earlier age of ventilation use and an improvement in the management sat and four patients (19%) stood with support, and three
loss of capacity to walk observed in this group. Despite of airway secretion. Conclusion: Nusinersen showed con- patients acquired gait with support among SMA type 2. In
the availability of access to molecular testing, we still tinuous benefit over 4 years of treatment, bringing motor terms of safety, the highest transaminase peak occurred
observed difficult in recognizing the disease, which may improvement mainly within the first 2 years of treatment in weeks 3 and 6 after infusion. Only 10 patients (30.3%)
be improved with wider dosage of serum CK in patients and maintaining motor function acquired at 4 years. Only had transaminase levels similar to baseline at week 8. 15
with motor/global development delay and weakness. patients who started treatment before 12 months of illness patients (45.4%) had thrombocytopenia in the first week
gained some motor milestone. No new adverse events and one patient met criteria for thrombotic microangi-
were reported in this long-term follow-up. opathy. The mean time of prednisolone use was 105 days
Doenças Neuromusculares (60.0 – 122.2). Conclusion: GT is effective in real life but
with the potential for serious adverse events. There is a
Doenças Neuromusculares need for strict monitoring of transaminases, platelets and
troponin and the occurrence of liver damage beyond 2
months of drug use, especially in patients with an older
profile than in clinical studies.

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261
XXX Congresso Brasileiro de Neurologia

TL 1104633 TL 1105627 TL 1105784

NEUROPLASTICITY INVOLVED IN THE BOTULINUM NEUROTOXIN TYPE A FOR FIBROMYALGIA IMPACT WORSENED DURING
REVERSAL OF NEUROPATHIC PAIN INDUCED TINNITUS MANAGEMENT IN PATIENTS WITH THE COVID-19 PANDEMIC
BY MOTOR CORTEX STIMULATION TEMPOROMANDIBULAR DISORDERS: A
João Pedro Sá Lins, Yago Eduardo Frezza Soliz,
DOUBLE-BLIND RANDOMIZED CONTROLLED Manoel Vieira, Ítalo França, Gerson Pereira Alves,
Cristiane Cagnoni Ramos, Fernanda Cardoso,
Thierry Salmon, Carlos Eduardo Malavasi Bruno TRIAL Eduardo dos Santos Paiva

Instituto Albert Einstein Cristina Salles, Miguel Meira e Cruz, Bernardo Universidade Federal do Paraná. Curitiba PR, Brazil
FMVZ USP Rodrigues, Mieszko Wieckiewicz, Tharcilia Azevêdo, Universidade de Brasília. Brasília DF, Brazil
Instituto Pau Brazil Juliana Melo, Carolina Orge, Ailton Melo Universidade Federal do Piauí. Teresina PI, Brazil

cris.cagnoni@gmail.com Bahiana School of Medicine and Public Health. jpsalins@gmail.com


Salvador BA, Brazil
Background: The motor cortex stimulation (MCS) have University of Lisbon – Portugal Background: Pain is an unpleasant experience modulated
been widely used in clinical medicine as a tool for pain Unniversity of Connecticut – Estados Unidos by sensorial, emotional, cognitive and social components.
control, used in patients with different pain syndromes, Wroclaw Medical University – Polônia Fibromyalgia is a chronic pain syndrome whose mani-
mainly of a chronic nature, which do not respond satisfac- Federal University of Bahia. Salvador BA, Brazil festation is influenced by these factors that were aggra-
torily to any type of conventional analgesia. Preliminary asm@ufba.br vated in the context of fear, uncertainty and restrictions
studies, obtained by our group, have demonstrated that of COVID-19 pandemic. This study aimed to evaluate if
MCS reverts the neuropathic pain in rats, inhibiting neu- the impact of fibromyalgia worsened during this period.
Background: Tinnitus is a common otological symptom
rons of the ventral posterior lateral and medial thalamus Methods: This is a longitudinal retrospective analysis of
eventually leading to the development of serious physical
nuclei and dorsal horn spinal cord, and activating neurons the database of Fibromyalgia Impact Questionnaire (FIQR)
and emotional disorders. Although uncertainties remains
of the anterior cingulate cortex and central and basolateral scores collected in the visits in the Fibromyalgia Outpatient
about pathophysiological mechanisms, the high preva-
nuclei of the amygdala. Despite this evidence, the mecha- Clinic of Hospital de Clínicas da Universidade Federal
lence of such complaint and the relationship to temporo-
nisms involved in that nociceptive modulation are not yet do Paraná (HC-UFPR) in Curitiba, Brazil. Two analytical
mandibular disorders make this and important topic on
understood. Objective: The aim of this research project approaches were done. The analysis of monthly evolution
clinical medicine. Treatment is challenging and currently
was to evaluate the neuroplasticity induced by MCS in of FIQR medians indicates the pattern of disease man-
there are no approved drugs. Objective: To evaluate effi-
a persistent peripheral neuropathic pain model in rats, ifestation during pandemic in the months of pandemic
cacy and safety of BoNT-A injection in the masticatory
investigating the participation of glial cells and of canna- and compared to the months of three previous years. The
muscles to treat tinnitus intensity compared to 2% lido-
binoids involved with the activation and inhibition of the hypothesis testing if there was an intrinsic variation of the
caine and 0.9% saline solution in patients with TMD and
nociceptive response, respectively. Methods: involved parameter was done in an analysis of four periods between
tinnitus. Methods: A double-blind, placebo-controlled,
induction of neuropathic pain by chronic constriction March, 16 and March, 15 of the following year. Results: In
randomized controlled trial (RCT) involved 96 patients
injury of the sciatic nerve, implantation of transdural descriptive analysis of monthly variation of FIQR median,
who were diagnosed with TMD and tinnitus. The Fonseca
electrodes over the primary motor cortex and evalua- registers of 326 patients were analysed, and a worsening of
Anamnestic Index (FAI) was assessed to characterize
tion of the nociceptive response in tests of mechanical this parameter was observed after March, 2020, when the
the severity of TMD symptoms in patients. The protocol
hyperalgesia and allodynia. Immunoblotting and immu- pandemic was officially declared in Curitiba. This trend
included evaluation using the Visual Analog Scale (VAS),
nohistochemical assays were performed to detected glial was also observed in November, 2020 when COVID-19
Tinnitus Handicap Inventory (THI), and Survey of Pain
fibrillary acidic protein (GFAP, an astrocyte marker), OX-42 epidemiologic indices worsened again in Paraná State.
Attitudes—brief version (SOPA-brief). Patients in all the 3
(a microglial marker) and cannabinoid receptor type 1 22 patients were included in hypothesis testing if there
groups received injections in bilateral masseters and tem-
(CB1) in spinal cord obtained from sham rats or animals was a FIQR score variation in pandemic compared to
poralis muscles, according to the American Pain Society
with neuropathic pain, stimulated or not. In animals with previous periods, a p-value <0.05 was observed only in
criteria. Insulin needles and a 1-mL syringe (Ultra Fine
MCS reversed neuropathic pain, a decrease of microglial comparison to 19-20 (p=0.026). In comparison to 17-18
Syringe 1 mL 6 ´ 0.25; UNIQMED ) were used for injec-
activation was seen, as well as increased CB1 immunola- and 18-19, the p-values obtained were 0.101 e 0.065,
tion. Group A received 100 IU of onabotulinumtoxin A
bel in the spinal cord, compared to unstimulated animals respectively. However, the confidence interval analysis
(Botox®) diluted in 2 mL of 0.9% saline solution; group B
with neuropathic pain. The data obtained suggest that demonstrated a trend of relative worsening of FIQR scores
received 2 mL of 2% lidocaine (without vasoconstrictor);
MCS inhibits spinal microglia, which contribute to the in pandemic comparing to all previous periods: 17-18 (CI
and group C received 2 mL of 0.9% saline solution. The
induction of nociceptive processes. Also, the cortical stim- 95% = [-1.55; + 13.48]), 18-19 (CI 95% = [-0.48; +12.36])
dose was equally divided and administered in the right
ulation induces the activation of CB1, which is important and 19-20 (CI 95% = [+0.97; +16.25]). Conclusion: This
and left sides. Results: Myofascial pain was found in 92
for the modulation of pain sensitivity. Conclusion: These retrospective analysis of FIQR scores registers collected
patients (92.7% of TMD patients). Myofascial pain with
results will contribute to a better understanding of the in HC-UFPR can indicate a trend of worsening of fibro-
limited opening was found in 71 patients (71.7%), disc
role of the motor cortex in the modulation of neuropathic myalgia impact in life of patients during the pandemic
displacement with reduction in 51 patients (51.5%), and
pain and to an improvement of therapeutic strategies to compared to previous periods.
osteoarthritis or osteoarthrosis in only 17 patients (17.2%).
treat persistent pain. Evaluation using THI after 30 days of the treatment showed
a significant improvement in tinnitus symptoms in the Dor
treated group [ F (2.94) = 6.498; p < 0.001]. The post hoc
Dor
least significant difference test showed that the average
THI score measured on the 30th day of the treatment in
BONT-A group differed from that of the lidocaine group
(27.9 ± 4.6 vs 22.5 ± 4.3; p < 0.001) and the saline solution
group (27.9 ± 4.6 vs 23.4 ± 3.8; p < 0.001). Conclusion: This
study demonstrated that BoNT-A is effective in treating
tinnitus in TMD patients. However, further studies are
necessary to understand better the longterm effects of
BoNT-A on tinnitus.

Dor

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XXX Congresso Brasileiro de Neurologia

TL 1105165 TL 1105527 TL 1105533

STROKE ATTENTION JOURNEY: RESEARCH, NEUROANATOMIC DISSECTION OF CORPSES PRACTICAL CLASSES IN NEUROANATOMY:
TEACHING AND EXTENSION IN THE NORTH AS A STUDY MATERIAL IN A MEDICINE THE USE OF STUDY SCRIPT
PLATEAU OF SANTA CATARINA COURSE
Sheila Wayszceyk, Mary Anne Pasta de Amorim
Michael Ricardo Lang, Renan Carneiro, Ivana da Sheila Wayszceyk, Mary Anne Pasta de Amorim
Universidade Regional de Blumenau. Blumenau SC,
Rosa Iesbik, Aline Haag, Leonardo Neidorf, Jaqueline
Universidade Regional de Blumenau. Blumenau SC, Brazil
Sueli Horodeski, Oscar Nelson Reimann Junior,
Jucimara Rosauro, Diogo Pasquali Nones, Chelin Brazil
sheila.wayszceyk@yahoo.com.br
Auswaldt Steclan sheila.wayszceyk@yahoo.com.br
Background: Neuroanatomy is the study of the struc-
NEUROMAX; Hospital São Vicente de Paulo. Mafra
Background: The study of neuroanatomy is extremely tures of the central and peripheral nervous systems. Its
SC, Brazil
important in the medical course, both for academics who study constitutes a mandatory subject in many health
Universidade do Contestado. Mafra SC, Brazil
wish to pursue neurology and for the training of general courses, introducing concepts and structural knowledge
Hospital São Vicente de Paulo. Mafra SC, Brazil
practitioners. Teaching-learning Methods: that focus on essential for the understanding of areas of activity. Due
NEUROMAX. Mafra SC, Brazil
the development of practical skills of the academic, such to the complexity and extensive program content, anat-
chelinsteclan@gmail.com as the dissection of human brains for the study of neuro- omy teachers need pedagogical resources that facilitate
anatomy, facilitate the acquisition of detailed knowledge the understanding of the academics in their practical
Background: Stroke is the second most deadly disease of human anatomy. Objective: The aim of this work is to classes. A resource that helps and guides students in their
in the world, however, with 80% of the causes considered describe and demonstrate the dissections performed on learning process, inside and outside the laboratory, are
preventable because they are modifiable factors and the parts of human brains intended for the neuroanatomi- the scripts in practical classes, leading students to build
time between the recognition of the signs and medical cal study of the Medicine course. Literature data refer- their own knowledge. Objective: The aim of the study is
care is crucial for the chance of saving lives. Having this, ring to neuroanatomical dissection were reviewed and to describe the use of study guides in the Laboratory of
in a continuous way and permanent education, already in parts of human brains from the Anatomy Laboratory of Human Anatomy as a method of practical study of neuro-
its second version (1st in 2019 and 2nd in 2021), the Stroke the Universidade Regional de Blumenau were photo- anatomy by the medical course of the Regional University
Attention Journey in the North Plateau of Santa Catarina graphed. Dissection constitutes a selection of fundamen- of Blumenau – FURB. The scripts of FURB academics and
has been carried out. Characterized by a partnership tal techniques for the preparation of the piece that will the curriculum in which it is inserted were used, accord-
between a Hospital institution, educational institution and be the subject of study and is an efficient method for the ing to the description of the Methods: of the laboratory
private institutions providing health services. Objective: learning of human anatomy by medical students. At the professors. At the Laboratory and Human Anatomy of
The objective of the actions linked to this journey is med- Laboratory of Human Anatomy of the Regional University FURB – Universidade Regional de Blumenau, practical
ical and health education, promoting the achievement of of Blumenau, medical students have the opportunity to neuroanatomy classes are carried out using a script of
recognition at different levels of care focused on stroke have contact with parts of the nervous system of human practical classes, which includes a list of the nomenclature
risk factors and stroke patient care. For this, technical and cadavers, which are dissected and prepared for visual- of the structures to be studied by academics and images
professional lectures, courses and community actions were ization of the anatomical structures, at different levels of that point to the location from them. This guide is previ-
held in favor of knowledge about the risk factors for stroke, depth and relationship with other structures, also pre- ously made available to academics so that they can seek
what is stroke, signs of a stroke, diagnosis and possible senting serial cuts, which are similar to cuts performed information about the structures and become familiar
treatments and prognosis, as well as, how the post-stroke in imaging exams, thus demonstrating the connections with the anatomical terms. In the first practical classes,
patient rehabilitation. The actions were aimed at different between them and the basis of neuroscience to be used the structures contained in the scripts are demonstrated
age groups, from early childhood education to gradu- when future professionals. The dissection process allows in the anatomical pieces, initially in synthetic models, by
ate studies. Professionals and academics from different the student to transform the organ into a puzzle that has means of numbered pins, which correspond to the struc-
areas made a great contribution, covering the modality several ways to assemble and consequently absorb the tures listed in the anatomical pieces. This first contact
of research, teaching and extension. For this, in raising content to be learned. Unlike the study only in synthetic, serves for academics to observe the location and rela-
awareness of the proposal, data from regional surveys plaster or plastic models, neuroanatomical parts in cadav- tionship between the structures. In the following classes,
were used, which show that patients with suspected and ers allow a three-dimensional view of the nervous system, the professors present the structures to the academics in
later confirmed stroke were mostly transferred to U-AVC fundamental for the formation of the basic neuroanatomy the human natural parts, making a relationship between
by ambulances (65.9%), and only 25% is performed by of the future doctor. With the dissection and preparation the anatomical structure and its concept, function, rela-
SAMU. In addition, those who arrived outside the thera- of natural pieces, it is also possible for the academic to tionship and location, integrating theoretical and prac-
peutic window were justified due to the failure to identify recognize anatomical variations, a source of practical tical content. Thus, it was demonstrated that the human
the stroke by family members (34%) and delay during the learning, encouraging the student to actively participate anatomy scripts in practical classes are effective tools
regulation/transfer from the municipalities to the U-AVC in the search for knowing such variations and relating in the learning process in Neuroanatomy, directing the
(18%). In this scope, more than 5700 people were reached them to possible pathological processes. Thus, the use of student to understand and fix knowledge and facilitating
with a frequency computed directly, estimating a greater human cadavers in the study of neuroanatomy in med- the teaching-learning process.
indirect reach. There are numerous reports and data that ical universities proves to be a differential for academic
show the permeability of the proposal in the different training, providing an adequate and efficient method for
target communities. Continuing health education is the learning the nervous system. Educação Médica
focus of different world organizations in favor of health
promotion. This action shows the multi and inter-profes-
sional interaction, aimed at the different spheres of edu- Educação Médica
cation and community. It is demonstrated here not only
the scope of this action, but also the Objectives: behind
it, as they bring direct benefits to the community and to
the regional health market.

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XXX Congresso Brasileiro de Neurologia

TL 1105567 TL 1105600 TL 1106192

DESCRIBING HORNER SYNDROME: AN ANALYSIS OF THE COMMUNICATION EXTENSION PROJECTS AS A STRATEGY TO


ILLUSTRATED GUIDE QUALITY OF THE DIAGNOSIS OF SPINAL STROKE PREVENTION
MUSCULAR ATROPHY IN THE VIEW OF
Giordanno Santana Mazza, Giuliano da Paz Oliveira Davi Lopes Santos, Ingred Pimentel Guimarães,
PATIENTS AND FAMILY MEMBERS Késia Sindy Pereira, Tito Bastos Soares, Lorenzo
Universidade Federal do Delta do Parnaíba. Marinho Morais, Antônio Alves Neto, Miguel Vieira
Parnaíba PI, Brazil Isabella Araujo Mota Fernandes, Vivian Romanholi
Coria, Renata Oliveira Menezes, Guilhermina Rego de Almeida, Samuel Cavalcante Marinho, Antônio
gsmazzaufpi@gmail.com Andrei Sena, Pedro Braga Neto
Universidade do Porto. João Pessoa PB, Brazil
Universidade Federal do Rio Grandeo do Norte. Universidade Estadual do Ceará. Eusébio CE, Brazil
Background: Horner Syndrome (HS) is classically char- Universidade Estadual do Ceará. Fortaleza CE,
acterized by a clinical triad: miosis/anisocoria, ptosis Natal RN, Brazil
Universidade do Porto – Portugal Brazil
and facial anhidrosis. Its pathophysiology is related to the UNIFESP
interruption of the oculosympathetic pathway; however, isabella_mota@yahoo.com.br
the mechanisms that lead to the onset of the syndrome are david.lopes@aluno.uece.br
diverse, and some are even potentially fatal, highlighting Background: The communication of the diagnosis of a
the importance of its early identification. Objective: The genetic and neurodegenerative disease, such as Spinal Background: In medical education, students are usually
aim of this study was to elucidate the main anatomical, Muscular Atrophy (SMA), requires a transmission cen- invited to participate, throughout their training, in activ-
etiopathogenic and clinical aspects of this neurological tered on the patient and or on the family, giving auton- ities that bring them closer to the community, in exercise
condition, based on original illustrations. Method: In order omy to those involved and strengthening the millenary of developing empathy. In the academic environment,
to synthesize the central aspects of the anatomy, clinical doctor-patient binomial. Objective: To analyze, from extension projects offer students the opportunity to be
and pathophysiological aspects of Horner syndrome, the perspective of patients and family members, the in closer contact with a particular medical condition
original illustrations of own authorship were developed. quality of disclosure of the SMA diagnosis. Methods: of great relevance to the community. In these projects,
In addition, neuroradiological examinations and pho- Semi-structured interview, via telecall, whose analysis students can get in touch with people outside academic
tographs of patients with the syndrome were included. was carried out by systematic condensation of the text, enviroment and disseminate essential information that
Results: Although uncommon, it is important to know summarized in four thematic axes. Results: Fifty-seven is often little known. In this context, the Interest Group of
how to recognize, diagnose and properly investigate volunteers from all regions of Brazil reported positive Neurosciences of the State University of Ceará (Neuruece)
patients with HS. Understanding the anatomy involved and negative experiences about clarifying the diagnosis, applies an extension project named “health education in
in the sympathetic innervation of the eye is fundamental communicating the prognosis, affective memory related stroke in primary care and in social networks”. Objective:
to both understanding the pathophysiology of the syn- to the event, in addition to advice to doctors. Discussion: Perform educational strategies relates to stroke preven-
drome and screening for its possible causes. The oculo- The individualized, clear, honest and welcoming trans- tion in patients and/or population with very high risk for
sympathetic pathway involves three neurons: first order mission, emphasizing positive aspects, in the presence stroke in the context of Primary Health Care. Method:
(central), second order (preganglionic) and third order of family members and with the possibility of continued This project aims to enlighten the population about the
(postganglionic). The etiologies of HS include infections, monitoring, were important to meet the communication risk factors for stroke, the importance of detection of its
neoplasms, vasculopathies and even iatrogenic lesions. demands. Among the barriers found, the lack of empathy, symptoms as well as immediate intervention to avoid
Besides the clinical picture composed of the clinical the estimation of life time and the absence of guidance and sequelae. The target audience consists of hypertensive
triad, pharmacological and neuroradiological tests are follow-up stand out. Conclusion: Failures in the transmis- individuals at high or very high risk for stroke followed
often indicated for the diagnostic investigation of HS. sion of the SMA diagnosis, in the perception of patients up in Primary Health Care Units of Fortaleza (CE), as
Conclusion: We present an illustrated guide with tar- and families, alert about the need for improvement and well as its family members, companions and community
geted information on the anatomy, clinical picture and adequacy of communication techniques and protocols, for health agents. In addition, educational posts on aspects
most important complementary tests in the context of the application of ethical principles in medical practice. involving stroke are elaborated and posted, both for the
HS, in order to provide a didactic guide on this syndrome target audience and for the general public that has access
for neurologists and the general medical community. to the profiles of Neuruece’s social networks. Results:
Educação Médica The health promotion action in the Primary Health Care
Units reached 104 people who participated in training
Educação Médica on the main characteristics of stroke, such as: risk fac-
tors, main symptoms, how to identify a stroke and when
to call emergency services. In addition, 2 materials were
posted on the following topics: “what is a stroke?” and
“main signs and symptoms of stroke”, which gathered an
average of 98 likes and 93 shares. In total, 1118 accounts
from different cities were reached (Fortaleza (CE), São
Paulo (SP), Maracanaú (CE) and Eusébio (CE)); 87 visits
to the @neuruece profile and 34 new followers gained,
currently totaling 2550. Conclusion: The impacts of these
educational strategies on the participants can prevent
new cases of stroke or reduce complications resulting
from the sequelae of people already affected by the dis-
ease. Neuruece’s social networks may impact positively
the current knowledge of the population about stroke.

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XXX Congresso Brasileiro de Neurologia

TL 1106238 TL 1106385 TL 1106437

CREATING SHORT VIDEOS AS AN ACTIVE “FORMANDO SINAPSES”: INSTAGRAM AS A MARKETING, SOCIAL MEDIA AND
LEARNING TOOL FOR NEUROANATOMICAL TOOL FOR TEACHING NEUROSCIENCE, AN NEUROLOGY: RECOGNITION OF ETHICAL
STUDIES EXPERIENCE REPORT ASPECTS BY NEUROLOGISTS
Bianca Berardo Pessoa Zirpoli, Daniel Cavalcanti Ingred Pimentel Guimarães, Davi Lopes Santos, Jonathas Silva Farias, Rebeca Lima de Almeida
Sena, Patryck Andrew Ribeiro de Melo Pontes, Maria Késia Sindy Alves Ferreira Pereira, Vitória Maria Santos, Mariana Oliveira Salamargo, Pedro Dantas
Eduarda Iglesias Berardo de Souza, Maria Valeska Torres Peixoto, Franklin de Castro Alves Neto, Oliveira, Philip George Glass Andrade
Berardo Pessoa de Souza, Luís Daniel Nóbrega Antonio Andrei da Silva Sena, Jorge Luiz de Brito
Santos, Mariana Soares Barros de Andrade, Shalom de Souza, Chiara Gübel Portugal, Danyela Martins Universidade Estadual do SUDOESTE da Bahia.
Pôrto de Oliveira Assis Bezerra Soares, Pedro Braga Neto Vitória da Conquista BA, Brazil
Universidade Federal de SERGIPE – Aracaju – SE
Universidade Católica de Pernambuco. Recife PE, Universidade Estadual do Ceará. Fortaleza CE, – Brazil
Brazil Brazil
Faculdade Pernambucana de Saúde. Recife PE, rebeca_lima23@outlook.com
Brazil ingredpguimaraes@gmail.com
Prefeitura do Recife. Recife PE, Brazil Background: The control of information in the face of
Background: Digital technologies have been consolidating a universe surrounded by easy access to the media is a
berardobianca@gmail.com themselves as important educational forms in the teaching challenge found in all areas. In Medicine, it is evident that
and learning process. Among these technologies, social the role of disseminating scientific content, dictated as
Background: Despite the traditional predisposition to networks have become important tools for the dissemi- medical advertising, must exclusively follow ethical prin-
favor textual instruction, research has been carried out nation of educational content and for the democratiza- ciples of primarily educational orientation, guaranteeing
since the 1980s suggesting the importance of image- tion of knowledge, with Instagram being one of the main the dissociability of merely commercial practices from
based education. Among these lines, Levin, Anglin and platforms used for this. Thus, it is important to explore the production of content with an instructive content.
Carney proposed the five functions of textual illustra- this social media as a potential neuroscience teaching Objective: To analyze the posts made by neurologists and
tions: Decorative; Representational; Transformational, network. Objective: To report the use of Instagram as quantify the nonconformities carried out in opposition to
facilitating the memorization of crucial information; a tool for teaching neurosciences. Method: This is an the guidelines of the Federal Council of Medicina (FCM).
Organizational; and Interpretation, in order to contrib- experience report on the actions carried out by medical Method: This is a cross-sectional and quantitative study
ute to the understanding of the information. Seeing the students in the university extension project “Formando carried out on the social network Instagram, through
importance of new media and exposure to images for Sinapses”, from February 2022 to June 2022. 8 educa- the search for an anonymous profile with a disabled
learning, as well as first-hand contact with the subject, tional publications were carried out in the thematic axes: locator. The descriptors used were: neuro, neurology,
short videos were chosen to be made as a means to learn “Neuroinformação” (posts containing information about headache, dementia, parkinson, migraine, stroke, epi-
Neuroanatomy, a crucial subject for medical education, important matters for Neurology), “Neuroartes” (posts lepsy and dystonia; postings from January to May 2022
also as an active learning tool. Methods: A Bibliographic relating artistic expressions to Neurology), “Dicionário in each neurologist profile were analyzed, among the first
Narrative Review was carried out on the different ways de Neuro” (explanatory post on neurological terms), 50 posts found in the “most relevant” and “recent” tags.
of learning, memory functioning, use of illustrations and “Neuroimagem” (explanatory post on neuroradiology The variables were related to ethical infractions (before
active teaching methods , as well as a text-book approach signs) and “Tópicos em Neurologia” (explanatory video and after photos, self-promotion, sensationalism, praise
for the contact with the subject (Neuroanatomy), followed on essential topics in Neurology). The publications were from third parties, gifts and sweepstakes, prizes with no
by note-taking on the subject. Structured scripts were cre- aimed at the public with the aim of disseminating infor- scientific value, photos with patients, announcement of
ated for the voiceover and the images, in order to build a mation on neurosciences. The posts were made using exclusive technique), recommendations (educational
narrative from individual photograms, featuring an ani- PowerPoint and Photopea and the videos were made using posts, disclosure of the registry expertise, contact disclo-
mation. The scripts were directed to the construction of the cell phone camera. All publications were posted on sure) and not recommendations (work selfies, personal
short scenes, favoring memorization (transformational), the Instagram profile @neuruece and the feedbacks were posts). Results: 63 public profiles were found through
the organization of information in a coherent way (orga- accounted and analyzed for using a spreadsheet created the selected descriptors, showing that about 50.8% con-
nizational), leading up to understanding (interpretation). in Excel. Results: The 8 posts published had the follow- tained at least one infraction, with selfies being pointed
The photographs were taken using a semi-professional ing distribution: 4 “Neuroinformação”, 1 “Neuroartes”, 1 out as the most prevalent in 73% of the total cases, fol-
camera, Canon T5i and a tripod, as well as a Smartphone “Dicionário de Neuro”, 1 “Neuroimagem” and 1 “Tópicos lowed by self-promotion and company advertisements.
Apple SE. Editing was performed using the Final Cut Pro em Neurologia”. The analysis by Excel accounted for an private institutions, and only 14.3% had no infractions.
program. Results: Two (02) short animation films of 03 average of 76 likes, 366 shares and 374 views on the video. Regarding non-recommendations, personal posts prevail
to 06 minutes were made, with animations alongside In total, 3471 accounts from different cities were reached in 69.8% of the profiles. In addition, when dealing with
the voiceover explanation for easy understanding of the (Fortaleza (CE), São Paulo (SP), Sobral (CE), Caucaia (CE) recommendations, only 42.9% of physicians strictly follow
contents regarding the function and naming of the cranial and Maracanaú (CE)); 1018 visits to the @neuruece profile what has been proposed. Conclusion: The study pointed
nerves, as well as another video explaining encephalic and 47 new followers were gained, currently totaling 2562 out the great disparity between the norms presented by
gyri and sulci. Conclusion: Experiments carried out followers. Conclusion: University extensions projects may the FCM and the infractions presented in the surveyed
on the performance of students with different teaching contribute to production and dissemination of scientific profiles. Furthermore, by understanding the basic premise
methods showed that students with access to multimedia knowledge to society. The use of Instagram as an educa- of caring for the individual, current Medicine presents the
had the greatest success, being a playful and at the same tional platform made it possible for more people to be continuous need for the dissemination of evidence-based
time efficient way to expand the student’s contact with reached with the knowledge disseminated. We believe that knowledge and the review of norms, highlighting the limits
the subject and then to consolidate the knowledge, espe- the project “Formando Sinapses” significantly increase of medical advertising in the face of the vulnerability in
cially if the Student is involved during the preparation of the diffusion of neuroscience knowledge to our students. which the general population is inserted.
the study material itself.

Educação Médica Educação Médica


Educação Médica

265
XXX Congresso Brasileiro de Neurologia

TL 1106499 TL 1106606 TL 1106651

DEVELOPMENT OF AN ONLINE COURSE AS SIMULATION TRAINING HELPS IN LEARNING THE USAGE OF OSCE IN NEUROLOGY
A TOOL TO TEACH DEMENTIA MANAGEMENT THE NEW BRAZILIAN BRAIN DEATH AS A METHOD TO EVALUATE CLINICAL
FOR PRIMARY HEALTH CARE DOCTORS PROTOCOL? KNOWLEDGE AND TECHNIQUE IN MEDICAL
STUDENTS
Samia Thabida de Oliveira Rabelo, Matheus Costa Livia Freitas, Maria de Oliveira, Roger Martins,
Bessa, Sarah Diogenes Alencar, Robson de Sales Norberto Frota, Fernanda Maia, Manoel Sobreira- Analuiza Silva Tenório Luna Sarmento, Renata
Ferreira, Barbara Matos Almeida Queiroz, Norberto Neto Alicya Alves dos Santos, Rafaela Volpini Medeiros,
Anizio Ferreira Frota, Fernanda Martins Maia Wellisson Rodrigues Silva, Caroline Meneses
Carvalho, Antonia Rosivalda Teixeira Marinho Universidade de Fortaleza. Fortaleza CE, Brazil
Resende, Ellen Dayanne Barros Silva, Lucas
Universidade Federal do Ceará. Fortaleza CE, Brazil
Nascimento Monteiro, Deryc Cleyner Piones Bastos,
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
manoelsobreira@yahoo.com.br Max Luiz Mendes Ramires Filho
samiathabida@gmail.com
Universidade Federal de Alagoas. Maceió AL, Brazil
Background: In 2017, the Federal Medical Council
Background: Due to the progressive aging of the pop- released the new Brain Death (BD) protocol with many caroline.resende@famed.ufal.br
ulation and the exponential increase in the number of modifications compared to the last one. According to the
elder people, it became urgent that health professionals protocol, the training course should be theoretical-prac- Background: oral and written exams allow for a traditional
are able to manage the main diseases that affect this pop- tical, however the resolution hasn’t detailed how was evaluation of clinical knowledge and skills in medicine,
ulation, especially dementia in its most varied etiologies. this practical activities. Simulation-based teaching has failing to gauge both technique and emotional skills in the
Objectives: This work aimed to build a virtual educational been increased use in medical education and could be students. The objective Structured Clinical Examination,
technology in the form of a basic course in the man- part of practical program. Objectives: to verify if the BD known as OSCE, is vastly used to supplement written
agement of elderly patients with dementia for Primary simulation training during the medical course modifies exams, allowing students the chance to practice medical
Health Care physicians, using an active methodological the learning about the new BD protocol. Methods: This skills (such as taking patient’s history, going through the
proposal. Methods: This is a methodological study. The is a cross-sectional analytical study, performed at the physical exam and formulating diagnosis) in a controlled
construction of the tool was divided into 3 phases. In University of Fortaleza, Brazil, from March to December of space. In the Federal University of Alagoas’ Medical School
December 2021, the first phase of the study began with 2018. Forty-five, fourth-year medical students composed (FAMED/UFAL), the OSCE is implemented during the
a review of the literature in health databases.The next the sample. The students were divided into three groups, 6th semester with the sole purpose of evaluating stu-
phase consisted in the elaboration of the course project Group A – students who participated in theoretical class dent’s technique and clinical thinking in the subject of
with the pedagogical planning. In the third phase, the and simulated station; Group B – students who partici- Neurology. Objective: To report the neurologists’, assistant
educational technology for the dementia management pated only in theoretical class. Group C – students who did students’ and 6th semester students’ experience with the
course was developed with an active methodological not engaged in any activity. Results: Group A has scored OSCE’s development and implementation in FAMED/
proposal using strategies such as storytelling, role play, a mean of 8.13 ± 1.01, group B has scored 6.49 ± 1.52, and UFAL. Method: The OSCE happened in FAMED/UFAL’s
interactive activities (multiple choice questions, clinical group C has scored 5.54 ± 1.83 (p = 0.000). A significant dif- Abilities Laboratory in the rotation format; four stations
cases, images, videos, quizzes, memory game) and short ference between the groups in the prerequisite (p=0.004) were displayed with clinical cases addressing typical topics
expository video classes. The material prepared was then and the clinical examination step (p=0.003) was found, in neurology that were discussed during classes. Accessory
inserted and made available in a virtual environment with with the biggest mistakes in Groups B and C. Conclusion: devices were used to heighten the students’ immersion,
publication of the material on the Moodle platform at the the BD simulated stations developed contributed signifi- such as videos, audio and actors. 1 minute was reserved
electronic address: “cursodeemencia.net”. Conclusion: cantly to the learning of the new brazilian BD protocol, for clinical cases’ initial reading and 4 minutes for the
It is believed that the course developed is an adequate for the fourth-year medical students. cases’ resolution, making each rotation lasting 20 min-
strategy for the improvement of health professionals in utes. Results: both assistant students and 6th semester
primary care, whose potential to change the health situa- students gave positive feedback to the neurology depart-
tion of the population is still underused and it is expected Educação Médica ment about the OSCE. Evaluated students shared great
that it will result in the improvement of care provided to interest in the possibility of conjoining theoretical and
the elderly population under your care. practical skills and the capacity to reinforce the knowl-
edge passed on by the professors. The practice of both
physical exam and clinical thinking were the highlights
Educação Médica for the evaluated students, with most of them considering
themselves ready to face neurological cases in real life.
Conclusion: underestimated by most subjects in medical
schools of Brazil, OSCE allows for a better implementation
and evaluation of clinical and emotional skills, theoretical
knowledge and practical technique in a controlled space,
better preparing students for their future as professionals.
Therefore, expansion of the OSCE method is needed for
other clinical subjects in brazilian medical schools, as a
way to improve learning, reinforce skills that can not be
gauged during written and oral exams and better prepare
students for their future as doctors.

Educação Médica

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XXX Congresso Brasileiro de Neurologia

TL 1105018 TL 1105072 TL 1105103

TEMPORAL LOBE EPILEPSY WITH MESIAL THE INCIDENCE OF NEPHROLITHIASIS IN EVALUATION OF ADHERENCE TO
HIPPOCAMPAL SCLEROSIS (TLE-HS): PATIENTS ON TOPIRAMATE THERAPY: A TREATMENT OF PATIENTS WITH EPILEPSY
LITERATURE REVIEW LITERATURE REVIEW
Sthéfani Spricigo Portilho, Heloise Helena Silveira
Wagner Gonçalves Horta, Mauro Souza Horta Maria Laura Caetano Tonhon Borges, Katia Lin, Juliane Salter Dalbem, Mariana
dos Santos Lunardi
Universidade de Pernambuco. Recife PE, Brazil Centro Universitário Claretiano. Rio Claro SP, Brazil
Clinica de Neurologia. São Lourenço MG, Brazil Universidade Federal de Santa Catarina.
marialaura.ct18@gmail.com Florianópolis SC, Brazil
wghorta@gmail.com Universidade Federal do Mato Grosso. Cuiabá MT,
Epilepsy is a chronic disease related to the nervous sys- Brazil
Background: Temporal Lobe Epilepsy (TLE) is the most tem, in which the brain oscillates between moments of
common focal epilepsy in adults. Hippocampal sclerosis stability and instability, in the latter presenting epileptic sthefanispricigoportilho@gmail.com
is associated with TLE and is present in 70-80% of cases. seizures. In order for the individual to control the inci-
This is an epileptic syndrome commonly associated with dence of seizures, there are antiepileptic drugs (AEDs), Background: Epileptic seizure is characterized by the
drug-resistant epilepsies, and represents one of the most among them Topiramate (TPM), which acts by blocking transient occurrence of signs and/or symptoms about
frequent indications for surgery for epilepsy.Objective: To voltage-dependent sodium channels, improves chloride the excessive and synchronous neuronal activity in the
understand the current scenario of TLE-HS.Methods: This influx, weakly inhibits carbonic anhydrase, potentiates the brain. The factors that affect the quality of life of chronic
is an integrative literature review with descriptive charac- GABA receptor, and blocks the AMPA receptor. Among the disease patients is medication. Drugs will only be effec-
ter, of articles indexed in the Medical Literature Analysis adverse effects manifested in patients taking long-term tive if taken regularly. Objectives: Compare adherence to
And Retrieval System Online MEDLINE/Pubmed, Latin PMT, there is the incidence of nephrolithiasis. The objec- treatment of patients with epilepsy and patients with other
American and Caribbean Literature in Health Sciences tive of this study is to describe the occurrence of kidney chronic diseases. Methods: Cross?sectional observational
LILACS, and Scientic databases Electronic Library Online stones in individuals, especially epileptic patients, who are controlled study, with application of a questionnaire to
(SciELO), researched in the period between october 2021 treated with the drug TPM, based on research in PubMed a group of 100 patients diagnosed with epilepsy and 50
and March 2022. Results: Initially, 136 articles were found and SciELO databases. The research showed that, despite controls, who were patients with chronic diseases use
in the databases, which after reading, 32 articles were having a low incidence rate, some patients on long-term medication. Results: By the controlled partial correla-
selected in the research that corresponded to the pro- therapy with the drug may develop renal lithiasis. However, tion, there was a positive and strong correlation between
posed objective. Conclusion: Attention to the treatment in most of the reported cases, discontinuation of the drug the scores of the Morisky tests and the scores of the BMQ
of mesial temporal lobe epilepsy associated with hippo- improved the symptoms and the stones disappeared. It Regimen and BMQ Memories test regarding age, disease
campal sclerosis remains of special interest due to its high was also possible to notice a certain incredulity described duration and scholarity in years. Morislky’s test and BMQ
prevalence and frequent resistance to antiepileptic drugs. by older authors about this adverse effect, in which many Regimen score (r = 0.317; p < 0.05) and BMQ Recall score
Thus, surgery should be considered in these patients. The did not consider relevant the relationship between renal (r = 0.383; p < 0.05) correlated strongly and positively
preoperative evaluation is responsible for determining the lithiasis and the use of TPM. In contrast, more current when controlling for the number of focal and bilateral
brain area where the crises originate and the precaution studies emphasized the relationship as something signif- seizures. The effect about having or not having epilepsy
of a safe resection without postoperative sequelae. It is a icant. One study from 2002 does not report the incidence using the Mann-Whitney test shows that the diagnosis
multidisciplinary process that involves the performance of nephrolithiasis, but, the duration of treatment of the interferes with the Morisky test score (U = 1,917.00; p <
of structural tests, functional tests, video-EEG monitoring individuals who participated in the study ranged from 20 0.05) and on the BMQ – Regimen score (U = 1,959.00; p <
and psychosocial assessment. The main data to consider to 1143 days, a time that may be insufficient to determine 0.05). Conclusion: Patients with epilepsy demonstrated
are magnetic resonance, interpreted in the context of clin- the risk of kidney stones, since this is an effect provided greater adherence to treatment the oldest, disease dura-
ical data and EEG. Surgical treatment for drug-resistant by long-term therapy. Finally, one study tried to associate tion, education level and better control of symptoms.
mesial temporal lobe epilepsy with hippocampal sclerosis the use of the drug with a ketogenic diet, which proved to
(ELT-HS) is the most effective alternative, although the be contraindicated because the ketogenic diet provides
literature reports seizure recurrence in 30% of patients a significant reduction in HCO3- levels, increasing met- Epilepsia
treated surgically. Among the standard techniques for abolic acidosis, a predisposing factor for the occurrence
patients refractory to clinical treatment, the anterior tem- of kidney stones. It can be concluded that nephrolithiasis
poral lobectomy (ATL) stands out. The analyzed studies may have a great impact on the patient’s quality of life, and
demonstrate to be the most effective treatment for the may result in life-threatening complications such as sepsis
control of crises. This technique consists of removing the and renal failure. Knowing the predisposing factors for
anterior portion of the temporal lobe, removing struc- its occurrence in each individual is of utmost importance
tures such as the amygdala, hippocampus and temporal when prescribing drugs that inhibit carbonic anhydrase,
neocortex. The ATL surgical technique is a consolidated such as Topiramate, in order to provide quality of life and
and effective procedure, with minimal mortality after the longevity to them, especially to patients with epilepsy.
surgical procedure. The surgical treatment of TLE-HS has
been shown to be effective for the complete resolution of
crises in most patients. Epilepsia

Epilepsia

267
XXX Congresso Brasileiro de Neurologia

TL 1105122 TL 1105124 TL 1105215

LEVEL OF CONFIDENCE OF HEALTHCARE INJURIES CAUSED BY FUNCTIONAL PROTEOMIC ANALYSIS OF SYNAPTOSOMES


PROVIDERS IN DIAGNOSING AND TREATING SEIZURES: A SURVEY OF PROVIDERS’ ISOLATED FROM BRAIN TISSUE OF PATIENTS
FUNCTIONAL SEIZURES IN BRAZIL: A PERCEPTION WITH MESIAL TEMPORAL LOBE EPILEPSY
CROSS-SECTIONAL STUDY
Adriana Boschi Moreira, Giullia Victória Froehner, Amanda Morato do Canto, Jaqueline Cruz Geraldis,
Adriana Boschi Moreira, Giullia Victória Froehner, André Enoch Knochenhauer, Rachel Schlindwein- Fabio Rogério, Marina K M Alvim, Clarissa S
André Enoch Knochenhauer, Rachel Schlindwein- Zanini, Marcelo Liborio Schwarzbold, Kette Dualibi Yasuda, Enrico Ghizoni, Helder Tedeschi, Matthew L
Zanini, Marcelo Liborio Schwarzbold, Kette Dualibi Ramos Valente, Katia Lin MacDonald, Fernando Cendes, Iscia Lopes-Cendes
Ramos Valente, Katia Lin
Universidade Federal de Santa Catarina. FCM-UNICAMP. Campinas SP, Brazil
Universidade Federal de Santa Catarina. Florianópolis SC, Brazil University of Pittsburgh – Estados Unidos
Florianópolis SC, Brazil Hospital das Clínicas da Faculdade de Medicina da
Universidade de São Paulo amanda.morato.canto@gmail.com
Hospital das Clínicas da Faculdade de Medicina da
Universidade de São Paulo
adriana.moreira.psicologia@gmail.com Background: The synapses are electric, and chemical
adriana.moreira.psicologia@gmail.com communications between the neurons and synapto-
Background: Functional seizures (FS) commonly pro- somes are membrane vesicles that contain the synaptic
Background: Functional seizures (FS) are a common duce physical injuries but the stigma that injuries rarely components. The study of synaptosomes can give clues
differential diagnosis of epilepsy. However, not all occur during FS persists among providers. Objective: To about synaptic transmission and its abnormalities since
FS-involved healthcare providers (HCP) are confident describe providers’ perception of the occurrence of injuries they contain all the machinery involved in the release,
in diagnosing and treating this disorder. Objective: To as a result of functional seizures. Methods: Professionals reuptake, and storage of neurotransmitters. In addition,
compare the level of confidence of HCP in diagnosing from the health sector involved in the care of patients with synaptic proteins are possible therapeutic targets in many
and treating FS in Brazil. Methods: Neurologists, psychi- FS answered to an online questionnaire, publicized via neurological diseases. Mesial temporal lobe epilepsy
atrists, and psychologists who work in the health sector email, social media and professional organizations. The (MTLE) is the most common type of focal epilepsy in
respond to an online questionnaire. Researchers publi- closed-ended question “In your opinion, can an adult with adults. It presents a high proportion of patients who do
cized the link to the survey via email and social media. The FS be injured during an event?” was rated on a 5-point not respond to antiseizure medication (ASM) treatment.
level of confidence in diagnosing and treating PNES were scale (1 = never; 2 = rarely; 3 = occasionally; 4 = often; 5 = However, patients with MTLE who are pharmacoresistant
questioned separately and self-rated on a 5-point scale always). Kruskal-Wallis H test was used to compare pro- to treatment with ASM may benefit from epilepsy surgery.
(1 = none; 2 = low; 3 = medium; 4 = high; 5 = very high). viders’ perception at the alfa level of.05. Data were col- Objective: To characterize the protein content of synapto-
Kruskal-Wallis test was used to compare the confidence lected during the first semester of 2022. Results: A total somes isolated from tissue of patients with pharmacore-
level distribution across categories of HCP (? =.05). Data of 515 healthcare providers (mean age of 39.4 ± 11.4 yrs sistant MTLE using mass spectrometry-based proteomics
were collected from February 22 to May 4, 2022. Results: [range 22-75 yrs]; 68% female) answered to the survey: Methods: We analyzed the synaptosomes isolated from
A total of 515 HCP (mean age of 39.4 ± 11.4 yrs [range 190 neurologists (37%), 82 psychiatrists (16%), and 243 brain tissue obtained from epilepsy surgery (hippocam-
22-75 yrs]; 68% female) respond to the survey: 190 neu- psychologists (47%). The respondents represented the pus and temporal lobe) of patients with MTLE and com-
rologists (37%), 82 psychiatrists (16%), and 243 psycholo- public and private healthcare sectors from all geographi- pared it to normal tissue. We isolated the synaptosomes
gists (47%). The respondents represented the public and cal regions of Brazil. The perception of most neurologists using the Syn-PER reagent, extracted and digested the
private healthcare sectors from all geographical regions (53%), psychiatrists (59%), and psychologists (58%) was proteins using the S-Trap columns, and labeled them
of Brazil. Fifty-four (28%) respondents were neurologists that an adult with FS can occasionally be injured when with TMT11-plex. Data was acquired using a Thermo
with expertise in epilepsy, 54% were psychologists with having a seizure. However, neurologists and psychia- ScientificTM Orbitrap EclipseTM TribridTM coupled to
expertise in clinical psychology, and 26% were psychiatrists trists tended to perceive the occurrence of injuries as less a Dionex UPLC system. We used the ProteomeDiscoverer
with expertise in psychotherapy. Almost all neurologists frequently than psychologists (H(2) = 34.628, p = 0.001, and R software for bioinformatics analysis. Results: We
(94%) and psychiatrists (96%) have diagnosed or treated mean rank 215.31 vs 210.91 vs 281.67, respectively): none identified 1,890 proteins, 7,521 peptides and 18,601 PSMs.
at least one adult patient with FS. In contrast, a minority of psychologists respond “never” to the occurrence of Our foldchange (FC) analysis revealed some critical
(43%) of psychologists reported experience with these injuries, in contrast to 1% of neurologists and 1% of psy- proteins that may be relevant in the epilepsy context.
patients. Psychologists’ level of training in FS was lower chiatrists. Thirty-three percent of neurologists and 30% Some of them are Synapsin-3 (FC=104), Abl interactor 1
than psychiatrists and neurologists (Mdn 2 points vs. 3 of psychiatrists responded “rarely” for the occurrence of (FC=147), Sodium-driven chloride bicarbonate exchanger
pts vs. 4 pts, respectively) (p<.01). Also, psychologists’ injuries, compared to 14% of psychologists. The option (FC=37.8), and Outer mitochondrial transmembrane helix
level of confidence in diagnosing FS in adults was lower “often” was selected by 13% of neurologists, 10% of psy- translocase (FC=51). The expression of these proteins was
than psychiatrists and neurologists (Mdn 2 pts vs. 3 pts chiatrists, and 24% psychologists. None of neurologists higher in the tissue of patients when compared to con-
vs. 3 pts, respectively) (p<.01). The level of confidence in and psychiatrists respond “always”, contrasting to 4% of trols. Conclusion: Our study explores for the first time
treating FS in adults differed among psychologists, neu- psychologists. Conclusion: A significant percentage of the synaptosome proteome of patients with MTLE. We
rologists, and psychiatrists, who had the highest level of providers underestimate the occurrence of injuries caused identified novel proteins that may have a role in disease
confidence (Mdn 2 pts vs. 3 pts vs. 3.5 pts, respectively) by functional seizures. mechanisms, or they may contribute to biological pro-
(p<.01). Conclusion: Psychologists’ confidence in diag- cesses leading to pharmacoresistance in MTLE.
nosing and treating FS in adults is lower than neurologists
and psychiatrists. Since psychological treatment is the Epilepsia
treatment of choice for FS, patients will likely face diffi- Epilepsia
culties in Brazil accessing psychologists with expertise.

Epilepsia

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XXX Congresso Brasileiro de Neurologia

TL 1105276 TL 1105361 TL 1105407

ROBUST CHANGES IN GENOMIC COSTS AND CASE FATALITY RATES OF THE WNT PATHWAY ALTERATION IN DISPLASIC
METHYLATION FOUND IN MESIAL HOSPITAL TREATMENT OF EPILEPSY IN THE CORTICAL TISSUE COMPARED WITH NO
TEMPORAL LOBE EPILEPSY STATE OF BAHIA, BRAZIL LESION ADJACENT TISSUE OF PACIENTES
WITH FCD AND REFRACTORY EPILEPSY
Jaqueline Geraldis, Danielle Bruno, Welliton Souza, Marcos Baruch Portela Filho, Jefferson Heber
Amanda Morato do Canto, Marina Alvin, Fabio Marques Fontes Júnior, Gleif Farias Leite, Catarina Marco Antonio Koff, Daniel Marinowick, Sofia
Rogerio, Clarissa Yassuda, Benilton Carvalho, Ester Gomes Menezes, Lécio Aragão Souza Morais, Azevedo, Gabriele Zanirati, Fernando Xavier, Fabio
Fernando Cendes, Iscia Lopes-Cendes Liliane Rodrigues Soares, Daniele Santos Andrade, Jean Varella, Eliseu Paglioli Neto, Andre Palmini,
Paula Cely da Silva Torres, Matheus Santana do Denise Machado, Jaderson Costa da Costa
Universidade Estadual de Campinas. Campinas SP, Rosário
Brazil Pontifícia Universidade Católica do RS. Porto Alegre
UNEB. Salvador BA, Brazil RS, Brazil
jaquelinegeraldis@gmail.com UNIME. Salvador BA, Brazil Instituto do Cérebro do RS. Porto Alegre RS, Brazil
Hospital Sao Lucas da Escola de Medicina da
Background: Mesial temporal lobe epilepsy with hippo- marcosbaruch05@outlook.com
Pontifícia Universidade Católica do Rio Grande do
campal sclerosis (MTLE+HS) is the most frequent and
Sul. Porto Alegre RS, Brazil
severe epilepsy in adults. When patients with MTLE+HS Background: Epilepsy is one of the most common neu-
do not respond to clinical treatment, one may consider rological disorders, characterized by a predisposition marco_koff@hotmail.com
epilepsy surgery. The tissue resected by surgery has been to the occurrence of unprovoked seizures. Worldwide,
extensively studied, and we and others have demonstrated there are about 50 million people living with epilepsy and Background: Focal cortical dysplasia (FCD) is a malfor-
abnormal gene expression in MTLE+HS. Gene expression 125,000 deaths related to the disease per year, of which mation of the cortical development that cause medical
is complex and regulated by many molecular mecha- 80% occur in low- and middle-income countries. Due refractory seizures and the only treatment may be surgical
nisms, including epigenetic changes. Therefore, we aim to its high prevalence and chronicity, epilepsy Results: resection of the affected area of the brain. People affected
to investigate whether epigenetic changes determined in high costs for healthcare systems and patients. Thus, by FCD may present seizures of variable severity since
by DNA methylation are present in brain tissue resected we aimed to evaluate the frequency of Hospitalizations, childhood. The physiopathology of the disease is not yet
from patients with MTLE+HS. Methods: We performed costs and deaths related to the public Hospital care of understood, however it is known that several genes alter-
whole-genome bisulfite sequencing to analyze DNA epilepsy in the state of Bahia, Brazil. Methods: Ecological ations may play their role. The WNT/?-catenin pathway is
from 11 brain samples obtained by epilepsy surgery from study, carried out with data from SIH/SUS, extracted associated with cell transformation and migration and for
patients with medically refractory MTLE+HS. We com- from the DATASUS platform. The period from January this reason may be crucial for understanding FCD’s aeti-
pared these to data obtained from samples of four autopsy 2017 to December 2021 was considered. The ICD G40 ology. The aim of this study was to explore genes related
controls (hippocampus and dentate gyrus). Samples from was the selection parameter used. Data was collected to the WNT/?-catenin pathway in patients with FCD type
patients were divided into two groups: i) patients with on the number of Hospitalizations, total cost, mean cost II. Methods: Dysplastic tissue and tissue adjacent to the
less than 20 years of disease duration (G1 – n = 5); and per Hospitalization, mean length of Hospital stay (LOS), primary dysplastic lesion of patients with FCD type II
ii) patients with more than 20 years of disease duration deaths, and case fatality rate (CFR) according to the vari- were obtained from two patients who underwent surgi-
(G2 – n = 6). Methylome was performed in an Illumina® ables gender, race and age group. Results: In the 5 year cal treatment. The analysis of the relative expression of
platform, and differentially methylated regions (DMRs) period, there were 16472 Hospitalizations for epilepsy in genes was performed by a qRT-PCR array containing 84
were identified. We conducted two by two comparisons Bahia. The total sum spent was R$ 12,833,908.38, and the genes related to the WNT pathway. Results: In patient
between data from patients and controls and between mean cost per Hospitalization was R$ 766.57. The mean 1, the analysis showed a difference in the expression of
the two groups of patients. Furthermore, we catalog the LOS was 6.3 days and there were 650 deaths, with a CFR seven genes, demonstrating an increase in AXIN2, FRAT2,
putative genes located in the DMRs. Results: Overall of 3.9%. Males were Hospitalized more frequently than FZD9, KREMENI and PP2R1A genes and a reduction in
hypermethylation was observed in tissue from patients. females (59.5% vs. 40.5%), with similar costs (R$ 802.75 CSNK1G3 and PPP2CA genes in dysplastic tissue. In
Also, DMRs had a heterogeneous distribution throughout vs. 713.40). Patients of mixed race were the majority patient 2, the analysis showed increased expression of
the genome, and these were usually observed in more (54.9%), followed by whites (3.5%) and blacks (3.4%). CSNK1A1, FZD4 and PPP2CA genes, as well as reduced
than one region, depending on their length, but introns Compared to other race groups, the mean cost for black of CTNNBIP1 gene in dysplastic tissue. Conclusion:
and promoters had increased DMRs compared to other patients was the highest (R$ 2392.33), as were the mean Dysregulation in the expression of genes that control the
genomic regions. We identified many genes in the DMRs LOS (25.1 days) and the CFR (4.49%). Patients aged 1 to 4 receptors of the WNT pathway keeps it in an inactivated
for the comparisons performed (6,371 in G1 × Controls; years were the most frequently Hospitalized, correspond- state. Therefore, a eventual manipulation of this pathway
5,548 in G2 x Control; and 4,088 for G1 x G2). Conclusion: ing to 19.9% ??of the total. The age group between 0 and 1 focusing on its activation may influence molecular man-
Currently, DNA methylation can be analyzed using high year had the highest mean cost (R$ 1216.54). The highest ifestations underlying the epileptogenic status in injured
throughput methods to determine its distribution in the CFRs were observed in patients aged 70 to 79 years (8.3%) brain tissue, which could act as a therapeutic alternative
entire human genome, the so-called methylome. This anal- and in patients that were 80 years old or more (13.4%). to FCD type II. The WNT/ ?-catenin signaling pathway is
ysis gives a complete overview of gene regulation medi- Conclusion: Black patients’ Hospitalizations resulted in crucial for the control of embryonic development, which
ated by methylation. We found remarkable changes in the higher mean cost, longer LOS, and higher CFR, which may takes place through the regulation of cell differentiation,
methylome of the brain tissue in patients with MTLE+HS, be related to inadequate access to primary health care to migration and proliferation, and apoptosis process.
with thousands of DMRs in patients. These were related to treat the underlying disease, but also to low income and
many candidate genes potentially involved in the molecu- deficient social support to maintain post-discharge care
lar mechanisms underlying MTLE+HS. Furthermore, our amongst this population. Infancy epileptic syndromes Epilepsia
results indicate that the molecular abnormalities present are more difficult to control, resulting in more frequent
in HS change over time. Hospitalizations and higher costs. Elderly patients have
more comorbidities, as well as an inherent physiological
fragility, which implies greater fatality.
Epilepsia

Epilepsia

269
XXX Congresso Brasileiro de Neurologia

TL 1105610 TL 1105871 TL 1106009

EVALUATION OF ACCIDENTS OCCURRENCE ANALYSIS OF THE COST OF KNOWLEDGE ABOUT SUDEP: WHAT
IN PATIENTS WITH TEMPORAL LOBE HOSPITALIZATIONS FOR EPILEPSY PEOPLE WITH EPILEPSY AND FAMILIES
EPILEPSY: COMPARISON BETWEEN ACCORDING TO REGION AND AVERAGE IN BRAZIL THINK ABOUT COUNSELING? IS
SURGICAL AND CLINICAL TREATMENTS LENGTH OF STAY IT DIFFERENT FROM OTHER COUNTRIES?
WHEN, HOW, WHO AND WHAT MIGHT
Aline de Fátima Dias, Leticia Franceschet Ribeiro, Victor Sousa Campos, Lucas Silva Santos, Gabriel
Rafael Batista João, Lucas Scárdua Silva, Marina Meira Xavier, Rebeca Lima de Almeida Santos, Lukas CHANGE AFTER INFORMATION
Koutsodontis Machado Alvim, Fernando Cendes, Santos Freire, Giovana Andrade de Oliveira, Erica Lecio Figueira Pinto, Amanda Cristina Mosini, Maria
Clarissa Lin Yasuda Otoni Pereira Miranda, Guilherme Rocha Cardoso, Alice Araujo de Moraes Mello Susemihl, Laura Maria
Mariana Oliveira Salamargo, Philip George Glass de Figueiredo Ferreira Guilhoto Guilhoto
Universidade Estadual de Campinas. Campinas SP, Andrade
Brazil Hospital das Clínicas – USP
FASAVIC. Vitória da Conquista BA, Brazil Escola Paulista de Medicina, Universidade Federal
alinedef.dias@yahoo.com.br UESB. Vitória da Conquista BA, Brazil de São Paulo; Associação Brasileira de Epilepsia
FASA. Vitória da Conquista BA, Brazil Associação Brasileira de Epilepsia
Background: Patients with Epilepsy have a greater risk
of accidents, which associates with high morbidity. The victorfasa@outlook.com lecio.figueira@hc.fm.usp.br
causes of frequent accidents are multifactorial, including
falls without previous warning related to seizures, inca- Background: Epilepsy is defined as a reversible brain Background: Sudden unexpected death in epilepsy
pacity of using primitive protective reflexes because of disorder characterized by the persistent predisposition (SUDEP) is the second leading cause of death in people
the unawareness during seizures, drugs´ side effects (gait of the brain to generate spontaneous epileptic seizures. with epilepsy (PWE), affecting 12% of those with drug
disturbances, disequilibrium and cognitive impairment). Objective: Relate the cost of Hospitalization for epi- resistant seizures. The concept that SUDEP counseling
Mesial Temporal Lobe Epilepsy is recognized for being lepsy treatment with the type of epilepsy, geographic would cause fear, anxiety and finally not change outcome
pharmacoresistant and, therefore, there will be a greater region and in-Hospital length of stay. Method: A quan- evolved due to recognition of effective prevention mea-
risk of accidents. Although the surgical treatment is recog- titative, descriptive, ecological study was carried out, sures. Nevertheless, worldwide awareness is still insuffi-
nized as effective in yielding seizure control, few studies with data available on DATASUS, an online platform by cient, despite counseling is advised by international soci-
have evaluated the impact of surgery on the frequency the Ministry of Health. The data chosen between 2011 eties, such as American. Academy of Neurology, American
of accidents. Objectives: To compare the frequency of to 2021 were based on the ICD-10 with federation unit; Epilepsy Society and National Institute for Health and
accidents in patients with Temporal Lobe Epilepsy under- total number of Hospitalizations; total cost; mean value of Care Excellence – UK. Objective: Assess the knowledge,
going clinical and surgical treatments. Methods: This Hospitalizations; days of stay; and average length of stay. preferences and attitudes of PWE, family members and
is a retrospective, non-randomized study. Data were As public data and without identification of the individuals, caregivers (FC) about SUDEP in Brazil. Method: An online
collected from medical records of individuals from out- there was no need for approval from an ethics committee. questionnaire approved by ethics committee was avail-
patient Epilepsy Clinics of the University of Campinas Results: In the evaluation of Hospitalizations in Brazil able on social media of brazilian Epilepsy Association.
(UNICAMP). Patients were divided into two groups: during 2011 to 2021, traumatic brain injury was the most Results: 533 complete questionnaires (311 PWE; 222 by
clinical and surgical. We searched for data related to falls, prevalent neurological cause, followed by epilepsy. This FC on behalf of patients), median age 33.5 (+9.64) and
burnings, fractures, head trauma and car accidents. We one represents 561.953 Hospitalizations with an annual 18.8 (+15.03) years, respectively, most female (84,9% PWE;
performed chi-square and Fisher’s exact tests to analyze average of 51.086 and a 4,5% variation. Their mean value 57.2% FC). Mental/intellectual or physical disability was
categorical variables and survival analysis (Kaplan Meier) was R$ 753,60, with an increase of 49% between the first declared by 15% (PWE) and 32.4% (FC), active seizures by
to evaluate the time between the beginning of follow-up and the last year of data, and the average length of stay 61.1% and 71.6%, and polytherapy by 49.8% and 64.4%.
and the event´s occurrence. The comparison was made was 5,8 days. The region with most Hospitalizations was Tonic clonic seizures were reported in the last 12 months
using Log Rank and Breslow tests. Results: The clinical the Southeast (43%). For the total and average costs by 56.9% and 81.7%. 48.6% PWE and 61.3% FC heard about
group included 242 patients, while the surgical group had per Hospitalization compared to the total number, the SUDEP, rarely by clinicians (11.3% and 16.2%). No coun-
197 patients. Accidents were more frequent in the clinical southeast continues to lead with 53%, such as about seling by physicians were available in 89.6% and 76.4%,
group (37 [15.3%]) when compared to the surgical one (9 the average length of stay with 7,1 days, followed by the despite most wanted to know (76.5% and 77.9%), at the
[4,6%]) [p<0,001]. During the follow up (median interval Northeast, North, Midwest and South. When comparing first consultation/diagnosis (34.1% and 41.2%) or during
of 17 years [DP 6.78] in the clinical group; 8.6 years [DP Hospitalizations for diseases of the nervous system, epi- early follow-up (32% and 21.3%). Neurologists and epi-
5.41] in the surgical group), 95.4% of the surgical patients lepsy only has higher mean value than migraine, multi- leptologists should be responsible for the information
were free of accidents, while in the clinical group, 84.7% ple sclerosis and nerve disorders. Conclusion: With the according to PWE (81.4%) and FC (82.9%). Most PWE
[p=0.02]. Falls were more frequent in the clinical group (90 analysis of the total number of Hospitalizations by region, and FC (96%) reported that they would change behaviors
[37.2%]) than in the surgical one (15 [7.6%]) [p<0.001]. The a concentration of the number in brazilian metropolises after being informed about SUDEP risk such as accept-
survival analyses confirmed that over time, falls occurred is seen, which can be related not only to the population ing devices to detect seizures, new treatments, lifestyle
in 36% of the clinical group compared to 8% in the sur- density and number of stressors, but also to the major changes and better adherence to them. Fear and sadness
gical group [p<0,001], which can act as a risk factor for supply of Hospitals that assist these patients and avoid were the most common reported feelings, but despite
major accidents. Discussion: Accidents can be frequent in underdiagnosis. This fact shows the average cost and that, >80% responders believed that counselling should
people with epilepsy. Surgical treatment in pharmacore- days of Hospitalization by region, except for the south- be done to all. Conclusion: PWE and FC in Brazil want to
sistant Temporal Lobe Epilepsy may have an important ern region, which has a higher cost of services per day of know about SUDEP, by neurologists, at the diagnosis or
role in accidents prevention. Therefore, surgery should Hospitalization in the region. In addition, the low vari- soon. They consider that treatment and behavior changes
be considered during clinical analyzes of these patients ation in Hospitalizations over the years is a response to would potentially be used to prevent this issue. Our data
because of its significant impact on their quality of life. their etiologies, since unlike having specific causes such suggest low adherence to international recommendations,
as status epilepticus or first seizure, epilepsy not con- and brazilian neurologists should review their practices
trolled by non-adherence to drug treatment is the main based on patient and family preferences, in addition to
Epilepsia cause of Hospitalizations which tends to reverse with the the possibility of preventing SUDEP.
improvement of outpatient care.

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CLINICAL AND NEUROPHYSIOLOGICAL ANALYSIS OF SEIZURE CONTROL AND COMORBITIES ASSOCIATED WITH EPILEPSY
EVALUATION OF EPILEPSY IN PATIENTS RISK FACTORS FOR REFRACTORINESS IN IN PEDIATRIC POPULATION
WITH CONGENITAL ZIKA SYNDROME PEOPLE WITH EPILEPSY IN AN ISOLATED
Djanino Fernandes Silva, Paulo Barbosa Leite
INDIGENOUS POPULATION IN PARÁ, BRAZIL Neto, Marco Andre Moraes Bernardino, Larissa
Mariana Braatz Krueger, Marcelo Rodrigues
Masruha, Samir Câmara Magalhães, Angela Marcos Manoel Honorato, Jonata Ribeiro de Sousa, Clementino Leite Sá Carvalho, Victor Fellipe Bispo
Rodrigues Gifoni, André Luiz Santos Pessoa, Manoel Carine Luri Fukase, Renata Maria de Carvalho Macêdo, João Eudes Magalhães, Fabíola Lys
Alves Sobreira Neto Cremaschi, Fernando Morgadinho Coelho Medeiros

Hospital Infantil Albert Sabin / Universidade Federal Universidade de Pernambuco. Recife PE, Brazil Hospital Universitário Oswaldo Cruz da
de São Paulo. Fortaleza CE, Brazil Universidade do Estado do Pará. Santarém PA, Universidade de Pernambuco. Recife PE, Brazil
Universidade Federal de São Paulo. São Paulo SP, Brazil djanino@gmail.com
Brazil Departamento de Neurologia e Neurocirurgia –
Universidade Federal do Ceará. Fortaleza CE, Brazil Universidade de São Paulo. São Paulo SP, Brazil Background: The presence of comorbidities epilepsy in
Hospital Infantil Albert Sabin. Fortaleza CE, Brazil
jsousastmiec@gmail.com the pediatric population is routinely evaluated in the fol-
mari_bk@hotmail.com low-up, in such a way that the associated clinical condition
Background: The prevalence of epilepsy is variable, can affect quality of life, response to treatment or even
Background: Congenital Zika virus syndrome (CZVS) is being higher in developing countries. Rates of 57/1000 mortality. Proper diagnosis and treatment of comorbidities
a pattern of congenital anomalies associated with ZIKV were found within the Guaymi indigenous in Panama can influence the appropriate control and management of
infection during pregnancy. The expanded spectrum and 18,6/1000 within the Bakairi in Mato Grosso, central epilepsy. Objectives: To assess the prevalence and types
of this anomaly includes neurological and non-neu- Brazil, but there was no study in isolated populations, of comorbidities (intellectual disability, migraine, behav-
rological malformations, with epilepsy being of great wich may have peculiar life habits and genetic features. ioral changes and sleep Disorders); Analize the associ-
prominence. There is wide variation in descriptions of Objectives: To evaluate risk factors, clinical and electro- ation of comorbidities with types of seizures and use of
epilepsy, between 9-88.3% of patients. The hypothesis encephalographic characteristics and the evolution of anti-seizures medications. Methods: Retrospective study,
of this investigation is that CZVS courses with epilepsy, people with epilepsy in a geographically isolated indig- carried out in the Child Neurology outpatient sector of
at a higher frequency than previously documented, with enous population. Methods: Retrospective, historical a University Hospital from April 2020 to May 2022. Data
alterations possibly documented through a 12-hour video cohort study. It used the medical records of patients seen were distributed in frequency, and the chi-square tests
electroencephalogram (VEEG) study. Objectives: The aim from 2003 to 2018 at the neurology outpatient clinic in of independence were performed to verify the associa-
of this study is to describe the characteristics of epilepsy the city of Oriximiná-PA, a reference for 2,503 Waiwai tion between variables and Fisher’s exact test, using IBM
in SCZV, through clinical and electrographic evaluation. tribe’s indigenous people. They are unmixed and inhabit SPSS software, and adopted significance of 5%. Results:
Methods: The evaluation was performed using a 12-hour a forest reserve in the Amazon. There were 25 patients The sample consisted of 282 patients with epilepsy (50.7%
video electroencephalogram, at the Hospital Universitário with epilepsy among the 118 indigenous treated. Clinical male; 49.3% female). The most prevalent age group, when
Walter Cantídio, and the characteristics of epilepsy were aspects, electroencefalogram (EEG) and neuroimaging the disease started, was older than 11 years (31.2%), fol-
evaluated using a questionnaire. This is a non-random- exams, family history, comorbidities, the treatment and lowed by those younger than 2 years (30.9%). Focal sei-
ized, cross-sectional study with consecutive inclusion, the response were studied. A survival analysis was per- zures in 77.7% of the sample, indeterminate seizures in
and the inclusion criteria were: confirmed SCZV and formed, whose outcome was seizure control. Results: The 11.3% and generalized seizures in 11.3%. Some degree
follow-up at the Hospital Infantil Albert Sabin. Results: occurrence of epilepsy diagnosis in the Waiwai population of intellectual disability represented 60% of the sample,
Twelve patients with CZVS were evaluated, with a mean was 10/1000, most cases started in childhood (68%), with and significant association with indeterminate epilepsy
age of 4.94 years, eight males, all grade V (transported in no difference regarding gender. The number of seizures (53.1%; p=0.007). Temporal lobe seizures ocurred in 21.6%,
a manual wheelchair) in the gross motor function classi- before the first consultation was high in 44%, family his- with seizures with behavioral arrest in 67.2%; the frontal
fication (GMFCS) and eleven (91.6%), with microcephaly tory was reported in 24%, perinatal hypoxia and febrile crisis corresponded to 12%. In addition, the occurrence
at birth. All patients in the sample had epilepsy, with a seizures in 20% each. There was intellectual disability in of temporal lobe crisis was associated with some degree
mean age of onset of epileptic seizures of 12.4 months. The one-fifth of the patients. Neurological examination and of intellectual deficit (62.3%) with good correlation with
most commonly observed seizures were: focal seizures neuropsychomotor development were abnormal in one the severity of the deficit (p<0.001). The presence of
(91.6%), followed by generalized tonic seizures, in 66.6% third. EEG showed abnormalities in 68% of the patients, frontal seizures was more associated with the use of two
and gelastic seizures, in 25% of the sample. Three patients with a predominance of focal epileptiform discharges or more medications (50%) compared to the group with
had, between two years and four years, reflex crises when (44%). Focal epilepsies were 64% and probably genetic other seizures (26.7%; p=0.021). Patients with migraine
positioned in the supine position. Polytherapy was used in and unknown etiologies were prominent. Treatment made up 34% of the sample, being more frequent in the
75% and monotherapy in 16.6%. As for the electroenceph- controlled 72% of patients (monotherapy in 64%). The non-frontal seizures group (p=0.017) or using more med-
alographic characteristics, all patients had disorganized analysis of seizure control over time showed, through ications (p=0.006). Behavior disorders were observed in
background activity. Interictal epileptiform paroxysms Kaplan-Meier estimation curves and Cox and Weibull 21% of the sample, with a higher prevalence in the group
were present in 91.6%, of which 66.67% were activated by regression models, that the factors most related to refracto- that used 2 or more medications (p=0.026). Conclusion:
sleep. It was detected in 66.67% of electrographic seizures riness with observation time of 24 months were abnormal The association of many common psychiatric and somatic
and in 41.67% of electrical sleep disorders. Conclusion: neurological examination and the presence of a family conditions is increased in epilepsy. The use of 2 or more
The frequency of epilepsy in the population of children history (p=0.001). Epilepsy classification and age had not anti-seizures medications should reflect not only dificult
with confirmed CZVS is very high (100% of patients), statistical significance for the outcome (Log-rank=0.2 and in epilepsy control. These findings may have implications
with varied and often refractory semiology, including 0.7, respectively). Conclusion: Unlike other indigenous in diagnosis and management of epilepsy and coexisting
subclinical and sleep-activated, being important longer peoples described in the literature, the Waiwai have a low conditions.
and periodic electrographic evaluation. incidence of epilepsy. Therapeutic results were similar to
the general population.
Epilepsia
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LOW BONE MINERAL DENSITY OCCURS BILATERAL, UNILATERAL AND ABSENT THE IMPACT OF PHARMACORESISTANT
IN MEN WITH EPILEPSY, REGARDLESS OF HIPPOCAMPAL ATROPHY EXHIBIT SEIZURES ON GRAY MATTER ATROPHY IN
YOUNG AGE OR SURGICAL TREATMENT DIFFERENT GRAPH THEORY PATTERNS IN GENERALIZED GENETIC EPILEPSY
TEMPORAL LOBE EPILEPSY
Jéssica Daniele Marques, Aline de Fátima Dias, Ricardo Brioschi, Gabriel Ferri Baltazar, Lucas
Lucas Scárdua Silva, Rafael Batista João, Marina Lucas Scárdua-Silva, Ítalo Karmann Aventurato, Scárdua Silva, Marina Machado Alvim, Rafael João,
Koutsdontis Machado Alvim, Ricardo Brioschi, Raphael Fernandes Casseb, Brunno Machado de Clarissa Lin Yasuda, Fernando Cendes
Gabriel Ferri Baltazar, Carlos Montovani Guerreiro, Campos, Fernando Cendes, Clarissa Lin Yasuda
Fernando Cendes, Clarissa Lin Yasuda Universidade Estadual de Campinas. Campinas SP,
Universidade Estadual de Campinas. Campinas SP, Brazil
Universidade Estadual de Campinas. Campinas SP, Brazil
Brazil ricardo_brioschi@hotmail.com
scardua@unicamp.br
jessica.dani.marques@gmail.com Background: Genetic generalized epilepsies (GGEs) cor-
Background: Although temporal lobe epilepsy (TLE) with respond to 20-25% of all epilepsies. They are genetically
Background: Although some studies showed increased unilateral hippocampal atrophy (HA) has been exten- determined disorders with subtle encephalic alterations
risk of developing metabolic bone disease in people with sively investigated, fewer studies evaluated the impact affecting the white and gray matter (GM), as demon-
epilepsy, little is known about the multifactorial under- of bilateral HA and the absence of HA on the efficiency strated by modern quantitative neuroimaging techniques.
pinning mechanisms, especially in men. Aspects of bone of brain connectivity and networks. Objectives: To inves- Alterations involving the thalamo-cortical connections are
metabolism such as vitamin D, calcium homeostasis and tigate brain connectivity and network properties with well described; however, few studies have investigated the
bone turnover markers may be negatively impacted by the graph theory Methods: in TLE patients according to the impact of pharmacoresistant seizures on gray matter atro-
use of antiseizure drugs (mainly the enzyme-inducers). laterality of HA (RIGHT-HA, LEFT-HA, BILATERAL-HA, phy (GMA) in patients with GGEs. Objective: To investigate
Objective: To evaluate the bone mineral density (BMD) NEGATIVE-HA). Methods: We analyzed 216 3T MRI rest- gray matter atrophy in Generalized Genetic Epilepsy (GGE)
in men with epilepsy and correlate it with clinical data ing-state images of TLE patients followed at the University patients according to seizure control. Methods: Fifty-
such as age, type of seizure and anti-seizure drugs (ASD). of Campinas (median age: 48 y.o.; 122 women): 92 with six patients with GGE from UNICAMP’s Epilepsy Clinic
Methods: We evaluated 505 men previously diagnosed LEFT-HA, 70 with RIGHT-HA, 25 with BILATERAL-HA were recruited: 33 Seizure-Persistent ([SZ-Persistent]
with epilepsy and exposed (minimum of five years of and 29 NEGATIVE-TLE. Groups were paired with healthy patients with current seizures during the previous year
exposure) to previous or current antiseizure drugs (phe- controls in terms of age (p>0.05) and sex (p>0.05). We of MRI acquisition, 21 women, median of 30 years); 23
nobarbital, phenytoin, carbamazepine and valproic acid), used the CONN connectivity toolbox/MATLAB 2019 to Seizure-Free ([SZ-Free] free of any type of seizures for at
followed at UNICAMP-Brazil in 2021. We identified 178 preprocess and extract graph theory measures (global effi- least one year before MRI, 17 women, median 34 years),
patients with BMD analysis (median age range, 50.5 ciency (G-eff ), average path length (PATH), degree/cost compared to 63 healthy controls (43 women, median
(21-86 years). Individuals were split into two groups (DEGREE), betweenness centrality (BC), local efficiency 34 years) matched for age (p=0.51) and sex (p=0.83).
(young-group [21-49 years], 90 individuals; older group (L-eff) and clustering coefficient (CC), after a whole-brain High-resolution 3T T1-weighted scans were firstly seg-
[50-86 years], 89 subjects). The BMD test evaluated t-score analysis. We considered a cost of 0.15 (including only the mented on CAT12/SPM12/MATLAB 2019 software (http:
indexes from the femoral neck, whole femur and lumbar 15% more statistically significant results:) and reported //www.neuro.uni-jena.de/cat12-html/cat.html). Then,
spine. Osteopenia was defined with a t-score of -1.0 to -2.4; p-values<0.05. Results: The LEFT-HA and RIGHT-HA pre- voxel-based morphometry (VBM) and surface-based
osteoporosis, with T-scores lower than -2.5. Clinical data sented a trend to higher measures of global connectivity morphometry (SBM) were performed to search for areas
were extracted from medical records and analyzed with (G-Con), local connectivity (L-Con), and centrality in the of gray matter volume and cortical thickness reduction
SPSS22. We performed chi-square tests for categorical brain (higher G-eff, DEGREE, BC, L-eff, CC and lower with an ANOVA-test (between the SZ-Persistent group
variables. Results: BMD was reduced in 107/179 men PATH) and a trend to lower G-Con, L-Con and centrality and SZ-Free groups compared to controls) on SPM12.
(59.7%). High-levels of bone disease were identified in in the cerebellum. The LEFT-HA and RIGHT-HA pre- We used age, sex and intracranial volume as covariates
both young and older patients (p=0.09): young-group sented higher DEGREE in the ipsilateral hippocampus for the VBM analysis and sex and age for the SBM analy-
[37/90 normal (41.1%), 40/90 osteopenia (44.4%), 13/90 and higher L-eff /CC in the contralateral hippocampus. sis. Clinical data were analyzed with SPSS20. The results
osteoporosis (14.4%)]; older-group [35/89 normal (39.3%), The NEGATIVE-TLE group showed a trend to lower G-eff from MRI analyses were reported with p <0.05, corrected
30/89 osteopenia (33.7%), 24/89 osteoporosis (26.9%)]. and higher average PATH, DEGREE and CC in the brain. for multiple comparisons. Results: We identified cortical
As expected, the osteoporosis group (20.6% of patients) The BILATERAL-HA presented a trend to higher L-eff / thickness atrophy only in the SZ-Persistent group, local-
was older than the osteopenia group (p=0.011). About CC in the brain. Conclusion: All groups presented a trend ized at the right precentral gyrus, while no alteration was
90% of patients with abnormal BMD had focal epilepsy. of higher local connectivity to the detriment of the G-eff. observed in the SZ-Free. Regarding the analyses of corti-
Unfortunately, 33/53 (62%) operated patients presented While the unilateral groups showed higher connectivity cal volume, we verified atrophy of the left thalamus in the
abnormal BMD. Conclusion: We observed elevated levels levels in the brain (with higher ipsilateral hippocampal SZ-Persistent group and the left precuneus region in the
of bone disease (regardless of the young age and surgical G-Con and probable compensatory higher contralateral SZ-Free group. Conclusion: As previously demonstrated
treatment) in men with epilepsy exposed to ASD. results hippocampal L-Con) and disconnection in the cerebel- in the literature, our results indicate that thalamo-frontal
Our results suggest that ASD exposure is associated with lum, the NEGATIVE-TLE presented concordantly higher networks are vulnerable to persistent seizures in GGE, with
early BMD reduction, which evolves into osteopenia and L-Con and lower G-Con both in the brain and cerebel- significant gray matter atrophy. However, we also find that
osteoporosis. BMD evaluation in patients with epilepsy lum. BILATERAL-HA did not exhibit any other specific different degrees and locations of GM abnormalities are
and appropriate treatment may be necessary to reduce patterns. Overall, cerebral G-eff is reduced in all TLE related to seizure control, which may have implications
the risk of fractures and related comorbidities. The selec- groups, regardless of the presence of HA. The presence of for the understanding of the physiopathology of the GGE.
tion of appropriate ASD is of extreme importance to avoid unilateral HA adds specific dysfunction in bilateral hemi-
bone disease in people with epilepsy, who usually require spheric connectivity, which may yield poor performance
long-term treatment. of the global brain connectivity. Epilepsia

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HOW FRANKLIN D. ROOSEVELT’S DIAGNOSIS FRIEDRICH NIETZSCHE COLLAPSE: PROFESSOR CHARCOT’S GENEALOGY
INFLUENCED THE DEVELOPMENT OF NEUROSYPHILIS OR FRONTOTEMPORAL
Helio Afonso Ghizoni Teive, Leo Coutinho, Gustavo
POLIOMYELITIS VACCINE DEMENTIA? Leite Franklin, Patrícia Áurea Andreucci Martins
Davi Lopes Santos, Ingred Pimentel Guimarães, Ingred Pimentel Guimarães, Késia Sindy Alves Bonilha, Thabata Emanuelle Martins Nunes, Beatriz
Késia Sindy Pereira, Chiara Gübel Portugal, Danyela Ferreira Pereira, Davi Lopes Santos, Franklin de Cassarotti, Carlos Henrique Ferreira Camargo,
Martins, Antônio Andrei Sena, Jorge Luiz Souza, Castro Aves Neto, Vitória Maria Torres Peixoto, Olivier Walusinski
Rebeca Bessa Maurício, Franklin de Castro Neto, Antônio Andrei da Silva Sena, Chiara Gübel Portugal, Universidade Federal do Paraná. Curitiba PR, Brazil
Pedro Braga Neto Danyela Martins Bezerra Soares, Jorge Luiz de Brito PUCPR. Curitiba PR, Brazil
de Souza, Pedro Braga Neto Private Practice – França
Universidade Estadual do Ceará. Eusébio CE, Brazil
Universidade Estadual do Ceará. Fortaleza CE, Universidade Estadual do Ceará. Fortaleza CE, patriciambonilha@hotmail.com
Brazil Brazil
UNIFESP Background: Professor Jean-Martin Charcot is widely
ingredpguimaraes@gmail.com
david.lopes@aluno.uece.br recognized as one of the most influential physicians of the
Background: Friedrich Nietzsche was a German phi- 19th century. Usually described as having an austere and
Background: Poliomyelitis is an acute infectious disease losopher who influenced all Western philosophy during reserved personality, his family not only influenced his
caused by an enterovirus that invades the central nervous the 19th century. His death occurred in 1900, but it all personality but also helped tailor his career. Objectives:
system and damages the anterior horn cells of the spinal started with a psychotic episode called “Turin Collapse” This work discusses Charcot’s genealogy and the role
cord and brainstem. Progress to lower motor neuron cell in 1899. In this episode, Nietzsche embraced the neck of played by this family in defining his personality and
death leads to disruption of motor units and subsequent a horse that was being whipped, unconsciously repro- advancing his career. Methods: We reviewed historical
muscle weakness or complete paralysis. Poliomyelitis ducing a scene from the book “Crime and Punishment” evidence concerning Charcot’s genealogy and his famil-
already existed long time ago, but only when Franklin D. by Dostoevsky. After that, he was declining physically ial relationships. Results: Born on November 29, 1825,
Roosevelt was diagnosed with the disease that the United and mentally, dying a year after this episode. After sev- in Paris, France, Charcot was the eldest son of Simon-
States mobilized to try to eradicate it. He was an American eral attempts at diagnosis, the main hypothesis is that he Pierre Charcot, a carriage builder, and Jeanne -Georgette
lawyer and politician who served as the president of the had neurosyphilis, a disease that can cause dementia in Saussier. His mother, Jeanne, passed away when he was
United States from 1933 until his death in 1945. He was a more advanced stage of syphilis. However, new diag- 14. Jean-Martin Charcot married in 1864, at the age of
elected to four presidential terms, being the longest serv- noses have recently been raised such as Frontotemporal 38, to Madame Victoire-Augustine Laurent, who was 29
ing president. Objective: Describe Franklin D. Roosevelt’s Dementia (FTD). Objective: To raise questions about years old (born in 1834). She had one daughter from her
illness and the increase the research to develop a vaccine the diagnosis of Nietzsche’s death. Methods: Analysis first marriage, Marie Charlotte Thérèse Durvis, who was
against poliomyelitis in the United States. Method: A com- of Nietzsche’s death and the new diagnoses suggested, raised from the age of 10 by Jean-Martin Charcot and
prehensive, critical and objective analysis of the current based on a narrative review of the literature. Results: In Victorie-Augustine. The couple had a couple of children:
knowledge about poliomyelitis and Franklin D. Roosevelt 1889, Nietzsche was admitted to a psychiatric Hospital in Jeanne Marie Amélie Claudine Charcot, and Jean-Baptiste
disease by means of a narrative literature review. Results: Basel, with loss of cognitive abilities, including language Charcot. Jeanne married Sir Alfred Joseph Charles Pierre
In August 1921, Roosevelt was swimming with his son, and and episodes of aggression and disinhibition. Due to this, Edwards, but divorced soon after, and married in second
the next day he started to fell weakness in his left leg. He the philosopher was diagnosed with “General Paralysis of nuptials with Sir Arthur Georg Hendry. Jean-Baptiste
also reported fever and a severe pain in his legs and lower the Insane” (GPI), the parenchymal form of neurosyph- Charcot, a physician, trained in Neurology under the
back. After 3 days, the politician had loss of sensitivity of ilis. Symptomatic neurosyphilis can be divided into two supervision of his father, abandoned his neurological
the lower limbs. The professor Robert Lovett of Havard main types: meningovascular and parenchymal, the latter medical career years after Professor Charcot’s death,
confirmed the diagnosis of poliomyelitis. His diagnois being the most similar to Nietzsche’s condition, starting becoming a famous maritime explorer, known worldwide
was essential to change the attention to public health. with mood and personality disorders and worsening with as Commander Charcot. Jean-Baptiste Charcot had a rela-
After the issue entered the national political agenda, the the deterioration of higher mental functions. Although tionship with Miss Céline Ducassé and had a daughter
US tried to develop a vaccine against the disease. In 1933, Nietzsche’s condition was compatible with a diagnosis of named Marie-Louise (Marion Charcot). He married for
Roosevelt created a commission to research infantile GPI, many of the symptoms observed are not specific to the first time in the year 1896, to Miss Jeanne Léopoldine
paralysis that in 1938 became the National Foundation for GPI and are compatible with a variety of neurological con- Hugo, daughter of the famous writer Victor Hugo, who
Infantile Paralysis (NFIP), whose awareness campaigns ditions. Due to this and other symptoms such as migraine had been married to Mr. Léon Daudet, son of the writers
described polio as the greatest threat to public health. The and stroke episodes, new diagnoses have been proposed, Alphonse Daudet and Julia Daudet, in the year 1891, and
NFIP has funded many studies that sought to develop a such as retrobulbar meningioma, CADASIL, MELAS and divorced in 1895. Jean-Baptiste Charcot and Jeanne Hugo
vaccine for polio. In 1952, Jonas Salk, funded by the NFIP, FTD. The FTD hypothesis was suggested due to the onset Charcot, divorced in 1905, after Jean-Baptiste Charcot’s
completed a study to create a vaccine and wanted to test of the clinical condition of early decline in social interper- trip to Antarctica, on the grounds of desertion. After that
it in children. 90% of respondents allowed their children sonal conduct, in personal hygiene, emotional blunting, Jean-Baptiste Charcot, remarried in the year 1907, to Miss
to participate in the study, which involved 1,5 million hyperorality, behavior compulsions, perseverative and Marguerite Cléry. Conclusion: Professor Charcot’s early
people. In 1953, the University of Michigan announced stereotyped behavior and mood with inappropriate joc- life was marked by the premature loss of his mother and
that the vaccine was safe and effective against the dis- ularity, symptoms noted on his admissions. Conclusion: his brother Jean-Eugene. Although these events appar-
ease. Conclusion: Roosevelt’s diagnosis of poliomyeli- Even after more than 100 years of Nietzsche’s death, there ently did not take any toll on his career, they might have
tis changed the American society’s look to the disease are still questions about what caused it. When analyzing contributed to his reserved personality. His marriage to
by stimulating the improvement of research through the its history, contradictions, and uncertainties about the a widow from the aristocracy provided him with a quick
creation of specific institutions that increased resources diagnosis of neurosyphilis are perceived, contributing social ascent, as he came from a modest family.
to develop a safe and effective vaccine. to the emergence of other diagnostic hypotheses, such
as Frontotemporal Dementia.
História da neurologia
História da neurologia
História da neurologia

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CHARCOT AND MIGRAINES: A PERSONAL THE HISTORY OF NEUROLOGY IN MACBETH AND CREUTZFELDT-JAKOB
RELATIONSHIP PERNAMBUCO: A HISTORIOGRAPHICAL DISEASE: THE FIRST RECORD OF HUMAN
OVERVIEW IN PERNAMBUCO NEWSPAPERS PRION DISEASE
Beatriz Cassarotti, Patrícia Áurea Andreucci Martins
Bonilha, Thábata Emanuelle Martins Nunes, Olivier Daniel Vicente de Siqueira Lima Junior, Elder Késia Sindy Alves Ferreira Pereira, Ingred Pimentel
Walusinski, Léo Coutinho, Carlos Henrique Ferreira Machado Leite, Pedro Antonio Medeiros de Oliveira, Guimarães, Davi Lopes Santos, Gustavo Rodrigues
Camargo, Hélio Afonso Ghizoni Teive Giovanna Maria Feitoza Barbosa dos Santos, Ferreira Gomes, Jorge Luiz de Brito de Souza,
Zenilda Gueiros Silvestre, Lucas Brandao Holanda Franklin de Castro Alves Neto, Danyela Martins
Hospital de Clínicas da Universidade Federal do
da Silva Bezerra Soares, Pedro Braga Neto
Paraná. Curitiba PR, Brazil
Private practice, Brou – França Universidade de Pernambuco. Garanhuns PE, Brazil Universidade Estadual do Ceará. Fortaleza CE,
Hospital de Clínicas da Universidade Federal do FACISA. Campina Grande PB, Brazil Brazil
Paraná. Curitiba PR, Brazil
daniel.med.upe@gmail.com kesia.sindy@aluno.uece.br
beatriz_cassarotti@yahoo.com.br
Background: Neurology is a relatively recent specialty Background: William Shakespeare, born in 1564, was an
Background: Professor Jean-Martin Charcot is widely in brazilian Medical History. Therefore, it is necessary English poet, playwright and actor, considered one of the
recognized as one of the most influential physicians of to study the steps taken towards the status quo of this greatest figures in world literature. After more than four
the 19th century. As head of the Neurology service of the specialty. However, analyzing its entire history is a work centuries of his death, his works continue to be the subject
Salpêtrière, Charcot developed a reputation as both a cli- that goes beyond its framework and for this study it was of discussions, due to the more than 712 medical refer-
nician and a professor. In his Leçons du Mardi, Charcot chosen to analyze the past and present of the Neurology ences described in the symptoms of his characters, when
presented patient-guided discussions on neurological in Pernambuco due to its relevance in the medical field. the writer did not attend university. These symptoms fulfill
diseases, presenting the audience with his appraisal of Objective: To make a general historical overview of the the diagnostic criteria of several pathologies, especially
the medical literature and his practical experiences on the History of Neurology in Pernambuco. Methods: A bib- neurological disorders such as parkinsonism, dementia,
subject. Although few of these discussions approached liographic survey was carried out with the files of the epilepsy, sleep disorders, headache, paralysis and human
headache disorders, as a migraine sufferer Charcot was Hemeroteca Digital Brasileira of available digitalized bra- prion disease. His references to neurological syndromes
able to present his insights uniquely. Objectives: This zilian newspapers in the year 2020. Results: Analyzing the inspired Jean-Martin Charcot, in the 19th century, to use
work discusses Charcot’s lecture on migraine and his historical process of Neurology in Brazil means to under- his literature in neurological teaching sessions. One of the
personal experiences on the subject. Methods: We pres- stand, above all, the links between it and Psychiatry, like outstanding works is the Shakespearean tragedy Macbeth,
ent historical evidence concerning Charcot’s lecture on Charcot who described the current Multiple Sclerosis at in which it is believed that there is a possible description
migraine, originally presented on November 22, 1887, the same time that he was interested in hysteria. As evi- of Creutzfeldt-Jakob disease, through the protagonist’s
including excerpts and original drawings representing dence, the first brazilian book that broke the paradigmatic symptoms in a soliloquy, this being the first record of the
his visual auras. Results: Charcot presented the case of model of “Psychiatry of the brain” was published in 1878 human variant of prion disease, three centuries earlier of
a 37-year-old teacher who presented a 12-year-history of (Lições sobre as moléstias do systema nervoso feitas na the official record. Objective: To describe the symptoms
“seizures” accompanied by limb paresthesias and speech Faculdade de Medicina do Rio de Janeiro). In a simple portrayed in Macbeth about human prion disease and
impairment. Although the patient reported that she never search in the records of newspapers in Pernambuco, the the importance of literature in the development of neu-
lost consciousness, she presented visual phenomenology term “neurology” was first used on May 14, 1913, when rological clinical thinking. Methods: Clinical, critical and
that included scotoma and line of fortification, sometimes a neurologist at the Hospital of Philadelphia showed objective analysis of the work Macbeth, through a narrative
followed by severe headaches. Charcot exposed his own brazilian doctors a 6km extension of films about clini- literature review. Results: Human prion disease is a fatal
experiences as a migraine sufferer, describing the visual cal cinematography, a practice that was not commonly spongiform encephalitis caused by a mutation that trans-
auras: “I know the shape of this picture for myself from applied in Brazil. The newspaper reports: “Certainly who- forms the PrPC protein into PrPSc, which is deposited in
having felt it, but the description comes from an astron- ever sees them not only assists the phases of an etiology tissues, causing dementia, neuromuscular symptoms such
omer […] Sometimes the scotoma presents only the line but also the development, the march of the morbid entity as ataxia and myoclonus, and psychiatric manifestations.
of fortification of yellow tints, sometimes red and green and can thus draw much more valuable conclusions In Macbeth, the protagonist presents a rapid cognitive
tones, and inside the luminous zone, which remained [...]”. Neurology in Pernambuco was initially linked to deterioration, also presenting involuntary movements,
dark, you can see a kind of smoke, vapor more or less Psychiatry. It became an independent specialty due to such as myoclonus, visual and auditory hallucinations,
thick, vibrating in a way reminiscent of what is seen in the the influence of the chair of neurology at the Faculdade and insomnia, symptoms of Creutzfeldt-Jakob disease.
atmosphere immediately above a working stove. The initial de Medicina – UFPE, with the outstanding participation Another event that confirms the diagnostic hypothesis is
very narrow circle gradually widens and finally ends up of Jarbas Pernambucano. Later, with Manoel Caetano de the description of the ingestion of necromantic infusions
disappearing, a new phenomenon succeeds it, hemiopia, Barros, Jarbas Pernambucano’s successor, we have the that contained a variety of human and animal organs, such
which is to say that by looking someone in the face, we expansion of the field of neurosurgery. Subsequently, the as neural tissues, which is a known form of transmission
do not see distinctly half of his figure. From this moment, area of ??Neuroradiology grew, with Glenystane Holanda. of infectious prions, which occurred prior to the onset of
pain is felt in the affected eye and above. Gradually the Conclusion: The history of Neurology in Pernambuco had Macbeth’s symptoms. After this event, Macbeth claims to
ocular phenomena disappear but the pain persists, and a long trajectory to become what it is today. Understanding have a strange illness. Conclusion: Shakespearean litera-
vomiting may occur.” Conclusion: As a professor and a the sequence of steps that have been taken up to the ture reveals itself, in this way, not only as a literary object
migraine sufferer, Charcot offered a unique perspective present day makes the neurologist beyond a mere exter- of entertainment, but as a vehicle of medical knowledge,
on migraine phenomenology. Although none of these nal observer of the phenomena to become an actor and which precedes official pathological records and which,
descriptions were new at the time, the richness of detail participant in the story, as the links of a chain which con- in this case, contributes to the neurological teaching and
provided by Charcot certainly paints a picture of the expe- tribute to its steadiness improvement of the clinical view.
riences our migraine patients go through.

História da neurologia História da neurologia


História da neurologia

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CHANGES IN CEREBROSPINAL FLUID FROM KAPPA INDEX AND OLIGOCLONAL BANDS CEREBROSPINAL FLUID (CSF) CULTURE
COVID-19 DETECTION IN CEREBROSPINAL FLUID RESULTS IN 47,473 CSF SAMPLES WITH
SAMPLES OF PATIENTS WITH SUSPECTED NORMAL CELL COUNT AND BIOCHEMICAL
Fernando Tenório Travassos, Luis Felippe Barros
Silva Pereira Ribeiro, Fernanda Martins Maia, MULTIPLE SCLEROSIS (MS) RESULTS
Marcelo Morais Valença Renan Barros Domingues, Fernando Brunale, Márcio Renan Domingues, Fernando Brunale, Márcio Vega,
Laboratório Fernando Travassos. Recife PE, Brazil Vega, Carlos Giafferi, Daiane Salomão, Sabrina Carlos Giafferi, Daiane Salomão, Sabrina Bonfante,
Hospital Pelopidas Silveira. Recife PE, Brazil Bonfante, Carlos Senne Carlos Senne
Hospital Geral de Fortaleza. Fortaleza CE, Brazil Senne Liquor Diagnóstico Senne Liquor Diagnóstico
Universidade Federal de Pernambuco. Recife PE,
Brazil contato@renandomingues.med.br contato@renandomingues.med.br

fernandottravassos@gmail.com Background: The evaluation of the intrathecal production Background: Some studies have shown there is a low
of IgG can be useful in the evaluation of MS suspected probability of positive microbiological results in patients
Background: The new coronavirus (SARS-CoV-2) cases. The most important test to verify the existence of with suspected meningitis/encephalitis and with normal
appeared in Wuhan, China, and quickly evolved into a intrathecal IgG production is the detection of oligoclonal CSF cell count. However, there is not yet a clear recom-
pandemic. There have been reports of neurological man- bands (OCBs) in cerebrospinal fluid (CSF) and serum. mendation of not performing CSF culture in such cases.
ifestations associated with COVID-19. These manifesta- Recent studies have reported that measuring CSF IgG Objective: To retrospectively analyze all the CSF culture
tions include mild symptoms, such as headache, fatigue, free light chains (FLC), especially the Kappa Index, is results in samples obtained from patients with suspected
hypogeusia and hyposmia; and severe manifestations, comparable with OCBs as markers of IgG synthesis. It meningitis and/or encephalitis sent to a specialized labo-
such as encephalitis, encephalopathy, Guillain-Barré is still unknown if diagnostic sensitivity be increased by ratory over a period of 3 years. Method: Culture results for
syndrome (GBS) and stroke. Cerebrospinal fluid (CSF) performing these two methods in parallel. Objective: bacteria, fungi, and mycobacteria in patients diagnosed
analysis is essential in making diagnoses of infections The aim of this study was to evaluate the results of the or suspected of having meningitis and/or encephallitis
of the central nervous system (CNS), once it can provide Kappa index and the oligoclonal bands (BOCs) in sam- were analyzed. CSF general analysis was considered
information about the inflammatory response and can ples from patients with suspected multiple sclerosis altered when at least one of the following were identified:
identify the etiological agent. The aim of this study is to (MS). Method: Patients with suspected MS underwent global count above 3 cells/mm3, protein above 45 mg/
assess the profile of patients with suspected and confirmed cerebrospinal fluid (CSF) analysis. Intrathecal synthe- dL, glucose below 50 mg/dL, lactate above 19.6 mg/dL.
COVID-19 for and verify the cytobiochemical changes in sis of IgG was evaluated with BOCs, IgG index, Reiber Results: Five hundred and nine (509) culture samples
CSF. Methods: It is a cross-sectional, retrospective and nomogram, and kappa index. Results: One hundred and (1.07%) were positive. CSF general analysis was altered
multicenter study. Patients who were selected had sus- fifty-three serum and CSF samples were included, 70 of in 495 (97.25%) and normal in 14 (2.75%) of them. In
pected or confirmed COVID-19. Lumbar punctures were them had positive CSF BOC. Among the BOC+ cases, 44 nine cases the identified bacteria were considered a
performed from May 2020 to April 2021 at the following (77.14%) were also positive with the Reiber nomogram, sample contamination. In 5 there was a positive result
locations: Hospital Português, Hospital Esperança Recife, 48 (68.5%) were also positive with the IgG index, and 68 for Cryptococcus. Conclusion: Our data suggest that
Hospital da Unimed, Hospital Memorial São José, Hospital (97.1%) were also positive with the kappa index. Among except in cases of cryptococcal meningitis, in which the
Santa Joana Recife and Hospital Esperança Olinda. All the patients without BOCs, 5 (6.02%) cases had a positive CSF culture may remain positive after CSF normalization
punctures were requested by the attending physician Reiber, six had a positive IgG index, and seven (8.4%) had and is a therapy parameter, to perform a microbiological
to assess neurological disease. The CSF samples were a positive kappa index. Conclusion: The Kappa index evaluation in CSF samples with normal cell and biochem-
analyzed at the Fernando Travassos Laboratory. Data was the method with the highest association with BOCs, ical parameters is not justified.
collection is being carried out in the medical records. The confirming that this method is at least as sensitive as the
research project for the master’s degree was accepted by detection of BOCs. Positive kappa results found among
the UFPE ethics committee. Results: The total number cases without BOCs may be false positives but may also Líquido cefalorraquiano
of patients was 116. Men represented 55.17% of the sam- indicate false negative results with BOC. Therefore, it is
ple. The mean age was 49,874 years, which ranged from possible that kappa index can help to diagnose cases of
10 days to 95 years. The main reasons for requesting the MS without detection of BOCs.
CSF analysis were: headache (32.4%), encephalitis (25.9%)
and lowered level of consciousness (17.65%). During the
procedure, 61.7% patients were alert and 32.2% were in a Líquido cefalorraquiano
coma. Almost all collections were performed in the lum-
bar/lying position, except for 1 patient who was seated
and another patient who sent the sample. The mean ini-
tial pressure was 19.9 cmH20. The average global white
cell count was 34.38 mm³, in only 3 patients there was
the presence of neutrophils. The mean total protein was
72.4 mg/dL. and in only 1 patient had glucose consumed.
Conclusion: Analysis of cerebrospinal fluid is important to
understand how SARS-CoV-2 acts on the nervous system.

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RARE CASE OF PANCREATIC CANCER WITH EVALUATION THROUGH THE STOP-BANG TELECONSULTATIONS IN NEUROLOGY
LEPTOMENINGEAL CARCINOMATOSIS QUESTIONNAIRE FOR THE RISK OF DEVELOPED AND IMPLEMENTED DURING
OBSTRUCTIVE SLEEP APNEA AMONG THE COVID-19 PANDEMIC IN THE BRAZILIAN
Fernando Tenório Travassos, Heloisy Maria Nunes
Galvão, Mariana Santaliz de Godoy Moreno, Patrick MILITARY POLICE OF AMAZONAS UNIVERSAL HEALTH SYSTEM
Ramon dos Santos Leal, Luis Felipe Ferreira Victor Rodrigo Felix, Anelisa Anelisa Itinose, Andrea Emanuelle Roberta da Silva Aquino, Daniela
Carvalho, Rodrigo Medeiros Barbosa Arruda Lira Oliveira, Matheus Acioly Muniz Teixeira, Carlos Laranja Rodrigues, Carlos Eduardo Batista, Josué
Laboratório Fernando Travassos. Recife PE, Brazil Mauricio Oliveira de Almeida Basso, Sabrina Gadenz, Kevin Kim, Daniela Pachito,
Hopsital Pelópidas Silveira. Recife PE, Brazil Stephan Sperling, Soraya Camargo Ito Süffert,
Universidade do Estado do Amazonas. Manaus AM, Carlos Eduardo Mantese
IMIP. Recife PE, Brazil Brazil
fernandottravassos@gmail.com Hospital Sírio Libanês. São Paulo SP, Brazil
acrmauri33@gmail.com Hospital Alemão Oswaldo Cruz. São Paulo SP, Brazil
Background: Leptomeningeal arcinomatosis (LC) occurs Hospital Moinhos de Vento. Porto Alegre RS, Brazil
Background: Obstructive sleep apnea (OSA) is a sleep Hospital do Coração. São Paulo SP, Brazil
in 5-8% of cases with solid tumors such as lung, breast disorder caused by total or partial cessation during sleep,
and melanoma. LC of pancreas adenocarcinoma is rare. associated with air hypoxia and recurrent awakenings; emanurob@gmail.com
We report a case of unresectable pancreatic cancer that Objectives: To evaluate the risk of sleep apnea and sleep
progressed to LC, despite maintaining good oncological, quality among the military police of Amazonas; Methods: Background: Teleconsultation gain strength in Brazil as
clinical and radiological disease control, as well as a signif- We carried out a cross-sectional observational study from an urgent response to the COVID-19 pandemic, aiming
icant reduction in plasma tumor markers, with no evidence August 2019 to January 2020 with about ninety military at providing alternative strategies of care in a context of
of local progression. Case Presentation: Male patient, 58 police officers from the military police of the state of overburdened health systems. Ever since Brazil’s Unified
years old, followed up at the Oncology Department of Amazonas (PMAM).The study was approved by CEP- Health System (Sistema Único de Saúde, SUS) conception,
IMIP (Instituto de Medicina Integral Professor Fernando UEA (CAAE: 13042019.0.0000.5016).Data were analyzed primary health care (PHC) was designed to be the main
Figueira), in Recife/PE, due to metastatic pancreatic ade- by the R software.The accepted level of significance was form of access to the health services, to facilitate a struc-
nocarcinoma to the liver, lymph nodes and osteoblastic 5%. 47.8% (N=43) of military police officers are at high risk tured and coordinated care. Despite these efforts, there
lesions in several vertebral bodies, more evident in L1, for OSA, 50% (N=45) have poor sleep quality according to are still important gaps between primary and specialized
without lesions in other organs, since February 2021. In the MSQ questionnaire and 41.1% (N=37) have excessive care due to the fragmentation of healthcare networks and
May 2022, he presented with acute left hemiparesis and sleepiness A significant association was found between to the expressive demand for consultations in secondary
dysarthria. The cranial resonance showed 6 lesions sug- the presence of snoring and HAS (p=0.005) and an asso- and tertiary care, ultimately resulting in long waiting
gestive of secondary neoplastic implants and the presence ciation between poor sleep quality (MSQ) with anxiety lists for many medical specialties, including neurology.
of meningeal enhancement. The result of the analysis and depression (p=0.001 and p=0.003). Conclusion: In In 2018, the Ministry of Health of Brazil started a project
of the cerebrospinal fluid was 27 cells/mm³ with 80% our study, we observed that military police officers have in partnership with private Hospitals aiming to improve
of lymphocytes and 20% monocytes, proteins: 560mg/ poor sleep quality, excessive sleepiness, and snoring, in efficiency in referring cases from PHC to specialized care,
dL, glucose: 10mg/dL. and there was the presence of addition to being overweight, which may be associated employing telehealth strategies. To minimize the impact
neoplastic cells. Some of them were shaped like signet with the presence of OSA. Public health policies aimed of the pandemic on the access of patients to specialized
rings. Conclusion: Particularly, in patients with pancre- at this population are necessary. care, the project expanded its activities by initiating the
atic cancer, the development of central nervous system offer of teleconsultations. Objective: The aim of this study
metastases is rare is rare (occurring in approximately 0.3% is to characterize the teleconsultations in neurology car-
of cases). This case is remarkable in two ways. First, only 15 Miscelânea ried out by this project in Recife, capital of Pernambuco, a
cases of LC in pancreatic cancer have been published so state in northeastern Brazil. Methods: Retrospective study
far. Second, neoplastic cells were seen in the first lumbar evaluating teleconsultations in neurology that happened
puncture. The diagnosis of LC is difficult in most patients between May to September 2020. Teleconsultation was
because this requires the detection of malignant cells on performed preferably in the video modality. Conditions
CSF cytology. They are detected in the initial CSF sample eligible for teleconsultation were headache, epilepsy,
in only 50% of patients with LC. Repeat CSF analysis has and cerebrovascular disorders. Results: A total of 243
been found to improve the yield to 90%. teleconsultations were analyzed, of which 76.95% was
a first appointment. In 48.97% of cases, the teleconsul-
tation represented the first opportunity for the patient
Líquido cefalorraquiano
to be consulted with the specialist. Among cases of first
appointment, 20.16% were further referred to a face-
to-face consultation and 21.81% could be redirected to
primary health care. Headache disorders were the most
predominant clinical conditions. Conclusion: The imple-
mentation and development of telemedicine in neurology
during the Covid-19 pandemic represented an opportunity
to assess the value of having teleconsultations added along
the line of care from primary care to a medical specialty,
promoting the coordination of care across different levels
of complexity of care in the health system and improving
access to specialized care.

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DEVELOPMENTAL MILESTONES POSSIBLE RELATIONSHIP BETWEEN FAT MASS CHARACTERIZATION IN POST-


EVALUATIONS AND BRAIN MEASUREMENTS PRENATAL EXPOSURE TO THE LARVICIDE STROKE INDIVIDUALS
IN RATS EXPOSED TO THE LARVICIDE PYRIPROXYFEN AND DEFICITS IN MOTOR
Sacha Fuchs dos Santos, Jaqueline Sueli Horodeski,
PIRIPROXIFEM DURING THE GESTATIONAL FUNCTION AND IN TIBIALIS ANTERIOR Daniel Rogério Petreça, Oscar Nelson Reimann
PERIOD MUSCLE STRUCTURE IN RATS Junior, Diogo Pasquali Nones, Stefan Moreira,
Stephanie Lindner, Arlindo Américo de Oliveira,
Nicole Jansen Rabello, Katriane Endiel Pereira, Nicole Jansen Rabello, Gabrielle Batista de Aguiar,
Chelin Auswaldt Steclan, Michael Ricardo Lang
Bianca Villanova, Gabrielle Batista de Aguiar, Katriane Endiel Pereira, Bianca Villanova, Rafaela
Rafaela Maria Moresco, Marcelo Alves de Souza, Maria Moresco, Marcelo Alves de Souza, Aline Universidade do Contestado – NUPESC. Mafra SC.
Aline Barbosa Macedo, Lígia Aline Centenaro Barbosa Macedo, Lígia Aline Centenaro Brazil
Ministério da Saúde, Secretaria de Atenção Primária
Universidade Estadual do Oeste do Paraná. Universidade Estadual do Oeste do Paraná.
à Saúde
Cascavel PR, Brazil Cascavel PR, Brazil
NEUROMAX; Hospital São Vicente de Paulo. Mafra
Universidade Federal do Paraná. Toledo PR, Brazil Universidade Federal do Paraná. Toledo PR, Brazil
SC, Brazil
nijanrab@gmail.com nijanrab@gmail.com NEUROMAX; Hospital Universitário Santa Terezinha.
Joaçaba SC, Brazil
Background: In 2015, there was an increase in the occur- Background: Pyriproxyfen is a pesticide used in Brazil
chelinsteclan@gmail.com
rence of microcephaly in Brazil, which was related to to control the Aedes aegypt mosquitoes. However, this
maternal contamination by Zika virus during pregnancy. pesticide has a similar molecular structure to the retinoic
Eighty percent of the risk factors for the development of
However, there are inconsistencies between the Zika acid, a vitamin A derivative that regulates neurogenesis,
stroke are modifiable, with obesity being among the top
maternal infections rates in relation to microcephaly cases. skeletal striated muscle development and expression of
five – In the northern plateau of Santa Catarina, it one is
Thus, other causes that may be related to the occurrence myogenic specification genes. In this sense, it is postu-
among the three most prevalent. With this in mind, present
of this malformation have been studied, including the lated that exposure of pregnant women to this larvicide
study aimed to characterize and correlate in a pioneering
exposure of pregnant to pesticides. The pyriproxyfen is a could interfere with retinoic acid signaling, impairing the
way the body mass index(BMI) and percentage of fat mass
pesticide used to control the Aedes aegypti mosquitoes, motor function and muscle tissue structure. Objective:
in affected individuals and bedridden by stroke, during
the vector of Zika virus. This pesticide is structurally similar This study aimed to verify if the prenatal exposition to
immediate Hospitalization. For that, data such as: sex;
to retinoic acid, a derived from vitamin A, involved in the pyriproxyfen can produce motor deficits, as well as dam-
BMI; age; physical activity level; leg length(right, cm);
control of neurogenesis during embryonic development. age to the muscular tissue of the offspring. Methods: Male
and fat mass(%). For weight, self-reported values by the
OBJETIVE: This study evaluated if prenatal exposure to Wistar rat pups were divided in 3 groups: Negative con-
patient and/or caregiver were assumed, justified because
pyriproxyfen can affect the neonatal development and trol (CT-) – offspring of mothers who drunk water during
100% of the population was bedridden or unresponsive
brain measurements in rats. Methods: Wistar rat pups pregnancy; Positive control (CT+) – offspring of mothers
– for the same reason, height was predicted according
were divided into negative control group (offspring of who drunk water containing 2 g/L of vitamin A during
to Chumlea et al.(1988), using knee height as an anthro-
mothers that drink water during pregnancy, CT-); posi- pregnancy and Pyriproxyfen (PIR) – offspring of mothers
pometric measure. BMI was calculated and classified
tive control group (offspring of mothers that drink water who drunk water containing 0.002 g/L of Sumilarv during
according to WHO recommended criteria. Fat mass was
containing 2 g/L of vitamin A during pregnancy, CT+) and pregnancy, a pesticide that uses pyriproxyfen as the active
estimated using the tetrapolar electrical bioimpedance
pyriproxyfen group (offspring of mothers that drink water ingredient. The gait quality of the experimental animals
method(Maltron, model BF906)– considering a desirable
containing 0.002 g/L of Sumilarv during pregnancy, a pes- was evaluated using Basso, Beattie and Bresnahan scale
level for men when ?15%, borderline from 15.1-24.9% and
ticide that uses pyriproxyfen as the active ingredient, PIR). (1996). On the 40th postnatal day, animals were euth-
elevated ?25%; and for women, desirable ?23%, border-
From the 1st to the 21st postnatal day, the developmental anized and the tibialis anterior muscle was dissected.
line 23.1-31.9% and high ?32%. The sample refers to the
milestones of pups were evaluated. At the 45th day of age, Samples were stained with hematoxylin and eosin for
three-month period of collection in the Unit-stroke in the
animals were euthanized and measurements of the brain analysis of the peripheral nucleus/muscle fiber ratio,
northern plateau of SC; with a mean age of 70±11.1 years,
volume, the brain and cerebral hemisphere length, the capillary/muscle fiber ratio and cross-sectional area of
and 57.1% of the individuals were female. The mean BMI
cerebellar vermis length, the maximum brain and cere- muscle fibers. Results: In one animal from the PIR and
found was 23.36±5.36 (normal) of the according to the
bellar width were performed. Results: Neonatal develop- one from the CT+ group it was observed external rotation
considered criteria. However, the population presented
mental assessments showed that PIR group performed of the hindlimbs. Deficits in motor coordination were also
an average of 35.18±7.65% of fat mass, which is consid-
latter the forelimb grasping, but carried out early the observed in one animal from the PIR and in four animals
ered high for both sexes. When the indices were statisti-
negative geotaxis task compared to CT- and CT+ groups. from the CT+ group. However, the BBB score was simi-
cally analyzed in relation to the distribution of means by
CT+ group exhibited the audio startle response latter than lar between CT-, CT+ and PIR groups. The peripheral
sex, there were no significant differences between them
the other groups. However, the eyelids opening and the nucleus/muscle fiber ratio and cross-sectional area of
or in relation to the mean, however, higher coefficients
hindlimb placing response were performed early by the muscle fibers was also similar in the three experimental
of variation were found for females. Interestingly, 100%
CT+ group. A reduction in the maximum brain width was groups studied. On the other hand, capillary/muscle fiber
of population self-reported being sedentary. These data
observed in PIR and CT+ groups when compared to CT- ratio was lower in the CT+ and PIR compared to the CT-
demonstrate the discrepancy between the qualification
one. Conclusion: The changes in neonatal developmental group. Conclusion: The motor and structure damage of
that the BMI and bioimpedance measures generate,
milestones observed in the PIR group were not the same the muscle observed in this study were subtle, which may
pointing out here that in immediate post-stroke popula-
as those seen in the CT+ group, showing that this pesticide be related to the administration of pyriproxyfen and reti-
tion with a normal mean BMI, where obesity would not
does not appear to interfere with retinoic acid signaling noic acid having been performed on demand. This method
be characterized as a prevalent risk factor, there were
during embryogenesis. However, pyriproxyfen affected of administration was used to mimic what occurs when
quantitative findings of the percentage of fat mass beyond
the acquisition of part of the developmental milestones pyriproxyfen is added to drinking water tanks to control
the limits considered high, regardless of sex. Therefore,
evaluated and reduced the maximum brain width of the the Aedes Aegypt. More studies are needed to confirm
it is proposed that other studies, with a larger sampled
offspring, suggesting that prenatal exposition to this pes- whether maternal exposure to pyriproxyfen produces
population, be carried out to highlight the importance of
ticide can produce neurotoxic effects. more severe deleterious effects on the offspring.
the anthropometric characterization of fat mass as a tool
for analyzing a risk factor for stroke, as well as its reliable
Miscelânea Miscelânea characterization and tracking.

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INVESTIGATION BETWEEN A MOZART MEDICAL CANNABIS: SOCIAL HISTORY OPSOCLONUS-MIOCLONUS-ATAXIA POST-


SONATA EFFECT ON CONTEXTUAL MEMORY CONTEXT AND CURRENT APPLICATIONS IN INFECTIOUS COVID-19 SYNDROME: CASE
EXTINCTION NEUROLOGY – A REVIEW PRESENTATION
Lelis do Vale Miranda, Amanda Di Gesu, Jully Henrique Nascimento Dourado, Victoria Reis, Lara Eduardo Mariano Carvalho Silva, Albina Karolyne
Gabriela Silva Souza, Ericles Willy Machado de Cordeiro Magalhães, Hugo Oliveira Polito Barreto, Diniz Mariano, Luiz Felipe Bezerra de Sousa, Ana
Oliveira, César Renato Sartori, Luciano Magalhaes Gabriel Vianna Pereira Aragão, Ana Rafaela Soares Carolina Nascimento de Sousa, Arthur Costa Junger,
Vitorino, Clarissa Maria Ferreira Trzesniak, Rodolfo Vale, Fernanda Souza Gracílio Silva, Daniel Rocha Fábio Pereira da Silva Júnior, Jhonata Gabriel Moura
Souza de Faria Paranhos, Télio Diego Cantalice, Pedro Antônio Silva, Lays Saraiva Rodrigues Carvalho, Lorena da
Pereira Jesus Silva Viana, Nínivi Daniely Farias Santos
Faculdade de Medicina de Itajubá. Itajubá MG,
Brazil Escola Bahiana de Medicina e Saúde Pública. Universidade Federal do Maranhão. Imperatriz MA,
Universidade Estadual de Campinas. Campinas SP, Salvador BA, Brazil Brazil
Brazil Hospital Geral Roberto Santos HGRS Neuroprime Neurologia. Imperatriz MA, Brazil

lelistp@hotmail.com henriquendourado@gmail.com eduardo.neurologista@gmail.com

Background: Memory consists in the capacity of storing Background: Cannabis is a plant genus native to Case Presentation: A 65-year-old man came to the out-
and recalling information acquired through experiences. south-central Asia that includes three different species: patient clinic reporting tremors for 30 days. The condition
After consolidated it can be kept, if repeatedly evoked, Cannabis sativa, Cannabis indica and Cannabis rudera- progressed with dizziness, feeling of malaise, vomiting,
or extinguished. Memory extinction is as important as lis. More than a hundred compounds with physiological scant speech, opsoclonus and myoclonus, which were
its formation, since it demonstrates the capacity of sup- action have already been isolated from the plant, including presented nine days after the mild coronavirus disease
pressing thoughts and/or behaviors no longer needed. cannabidiol and tetrahydrocannabinol. The discovery of (COVID-19) was confirmed. He denies vertigo, convul-
This process does not imply experience elimination, but these compounds allowed the subsequent discovery of a sive crisis and history of cerebrovascular accident (CVA).
it’s ressignification. Despite the known music beneficial vast biological system called the endocannabinoid system Neurological examination showed bilateral dysmetria, dys-
effects on the brain, especially Mozart’s K448 sonata, few (ECS), which promotes several interactions between dif- diadochokinesia, dyssynergia, myoclonus, truncal ataxia,
is known about its potential on memory extinction. Aims: ferent systems and organs. With this new knowledge, new astasia and abasia. Brain Magnetic Resonance Imaging
Investigate Mozart’s K448 sonata effects on contextual drugs were created aiming at the treatment of all sorts of (MRI) revealed mild global atrophy and microangiopathy.
memory extinction in mice. Methods: The project was conditions, mainly neurological, psychiatric and gastric. An electroencephalogram (EEG) was requested, which
accomplished with 41 C57BL/6J mice, divided into G1: Investigations of the safety and efficacy of these drugs showed normal baseline activity with the presence of
Mozart (n=14), G2: Ambience (n=14) and G3: Control are still at its beginning, even though its commercializa- multidirectional rapid eye movement artifacts, in addition
(n=13). Only the first group was exposed to music from 9: tion and use in different diseases are already a reality. to laboratory tests to screen for paraneoplasia and possi-
00 pm to 7: 00 am throughout the project, since intrauter- Objective: To carry out a comprehensive literature review bles etiologies (CA 19.9, carcinoembryonic antigen, ESR,
ine life. Groups 2 and 3, however, were only exposed to on the use of medical cannabis in various neurological CRP, occult blood test, ANA, homocysteine, vitamin B12,
ambient noise. From the 50th to the 54th day, the animals diseases, discussing its social history context and eluci- vitamin E, folic acid, negative antiphospholipid antibod-
underwent Habituation, in order to control behavioral dating the applicability of cannabinoids and their results ies, liver enzimes, hepatitis B virus, Hepatitis C virus, HIV
interferences in response to the testing ambient differ- in the light of current scientific evidence. Methods: A Elisa, VDRL and FTA-ABS, chest and abdomen tomogra-
ences. On the 55th day the Aversive Training was per- literature review was performed in 2021 using the MeSH phy). Results: The results did not show paraneoplasms
formed with groups 1 and 2. On the 82nd day all groups terms “Neurology” and “medical cannabis” in PubMED. or other infections, thus concluding the diagnosis of
began Extinction Test, which lasted five consecutive days. Clinical trials, systematic review and meta-analyses were Kinsbourne Syndrome. Lumbar puncture was performed
All groups then underwent Recall Test on the 107th day. included, without restrictions on date of publication to assess cerebrospinal fluid (CSF), cultures for bacteria,
The procedures were recorded for analysis. Repeated and language. Results: Thirty-eight articles were found, fungi and tuberculosis were negative and pulse therapy
measures ANOVA Test was used to analyze Extinction from which twenty were analyzed for the writing of this with high-dose methylprednisolone. The patient showed
Text and One-Way ANOVA Test to Recall Test. Values of work. Among the twenty articles selected, four were complete improvement of the clinical picture. Discussion:
p?0,05 were considered significant. Results: The statis- meta-analyses, nine were systematic reviews and seven Kinsbourne Syndrome or Opsoclonus Myoclonus Ataxia
tical analysis demonstrated that the fear extinction pro- were clinical trials. As for the diseases studied by each Syndrome is a rare neuroinflammatory disease with a vari-
cess varies amongst groups, despite the reduction of the paper, three were about movement disorders, three about able combination of opsoclonus, myoclonus and ataxia¹.
freezing time for all of them during the tests. In general pain, three about multiple sclerosis, ten about epilepsy The etiology can be paraneoplastic, such as neoblastoma,
G1’s freezing time was longer than G2’s. On the first day of and one about various diseases. Conclusion: This work parainfectious, after viral or bacterial infections, toxic and
extinction G1’s freezing time was longer than G2’s e G3’s, found little evidence in current medical literature that metabolic or idiopathic. The exact pathogenesis is not
whilst on the second day it was only longer than G3’s. At supports cannabis-based treatment for neurological fully known, however, in recent years, the emergence of
last, on the fifth day, G1’s freezing time was shorter than conditions, except in specific epileptic syndromes and neurological disorders, such as Kinsbourne syndrome
G2’s. Amongst the second and third day of extinction, G1’s in spasticity in the context of multiple sclerosis, both itself, after COVID-19 infection has been reported in
freezing time reduced more than G3’s, whilst amongst the which have been approved in some parts of the world several studies¹. It is believed that the syndrome may be
fourth and the fifth day, G1 upholded its freezing time, and are already in use. However, the use for Parkinson’s an inflammatory phenomenon mediated by the autoim-
whilst G2’s extended. On recall, G1’s freezing time was disease and chronic pain still needs further evidence. mune system, secondary to infection by the coronavirus².
longer than G3’s. All analysis were statistically significant Therefore, the main critics and caveats of this study are Conclusion: Despite being a rare disease, the diagnosis of
(p?0,05) Conclusion: It was noted that mice exposed to that the irrational use of medical cannabis may promote Kinsbourne syndrome by the neurologist, in the present
Mozart’s K448 sonata were more reactive to aversive stim- short and long-term harm to patients and their families case, was verified from the neurological physical exam-
ulus. However, they exhibited faster contextual memory and that this drug may end in a new historical ostracism ination, mainly related to motricity and persistence of
of fear extinction. because of political and economic reasons. opsoclonus, as reported in the literature, in addition to
a broad diagnosis differential.

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Miscelânea

278
XXX Congresso Brasileiro de Neurologia

TL 1106152 TL 1106223 TL 1106226

PREVALENCE OF CERVICAL POSTURAL SAFETY AND EFFECTIVENESS OF COVID19 OPTICAL COMPRESSION CHANGES IN
ALTERATIONS IN OCULOMOTOR VACCINATION IN MULTIPLE SCLEROSIS PATIENTS WITH SELAR INJURY: A TERTIARY
DISTURBANCES PATIENTS – INTERIM ANALYSIS OF A MULTI- HOSPITAL STUDY
CENTER STUDY
Luciano Barroso de Albuquerque Filho, Sara Sarah Diógenes Alencar, Gabriela Sanford
Diógenes Peixoto de Medeiros, Leandro Freitas Pedro Luiz Lopes, Francisco Bruno Santana da Guimarães Caminha de Oliveira, Ana Vitória Gabriel
Oliveira, Júlio César Claudino dos Santos Costa, Igor Bessa Santiago, Avelino Missialdes Diógenes, José Ítalo Soares Mota, Nayara Queiroz
Dutra Júnior, Gabriela Joca Martins, Paulo Ribeiro Cardoso Pinto, Alessandra Braga Cruz Guedes
Centro Universitário Christus – Unichristus. de Morais, Matheus Costa Bessa, João Gabriel
Nóbrega
Fortaleza CE, Brazil Dias Brasiliense Frota, Karoline Ferreira Mororó
Faculdade de Medicina, Centro Universitário Facisa, Hospital Universitário Walter Cantídio. Fortaleza Menezes, Fernanda Martins Maia Carvalho
UNIFACISA. Campina Grande PB, Brazil CE, Brazil
Universidade Federal de São Paulo. São Paulo SP, Hospital Geral de Fortaleza. Fortaleza CE, Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Brazil Universidade de Fortaleza. Fortaleza CE, Brazil
pedroluizlopes2@hotmail.com
lucianoalbuquerque1991@gmail.com sarah_diogenes@hotmail.com
Background: Multiple sclerosis (MS) is the most common
Background: The human mechanism of adjustment the immune-mediated demyelinating disease of the central Background: Sellar tumors are classified according to
balance and the posture involves a reflex relation between nervous system. COVID-19 infected more than 535 million size, presence of compressive symptoms and functionality.
the oculomotor system and the superior cervical, which people worldwide. The most promising solution remains When the lesion is large and has a suprasellar extension,
Results: from the information coming from the vestibular an effective vaccine. Given the autoimmune condition it can compress the optic chiasm and may evolve with
and ocular systems. In the deficient posture, there is an of MS, questions have arisen as to whether vaccination ophthalmological changes. Optical coherence tomog-
abnormal relation between various body parts, resulting for COVID-19 confers effective immunity or whether raphy (OCT) can determine the thickness of the nerve
in excessive solicitation of the supporting elements and it could represent a trigger for EM attacks. Objective: fiber layer (NFL) and its division by quadrants, being of
in the decrease of the perfect balance of the body over Report the clinical features and possible adverse effects significant relevance in chiasmatic compressions due to
its supporting foundation. Among the cervical postural of COVID-19 vaccination in patients with MS. Describe its predilection for the nasal region of the optic nerve, and
problems there is the torticollis, describes the condition clinical events in patients diagnosed with MS that are its correlation with data already classically obtained by
of a cephalic deviation in relation to the torso. The oculo- subsequent to vaccination. Show possible changes in campimetry. Objective: The present study aims to eval-
motor disturbance, which can cause torticollis, is known functional classification after vaccine exposure. Method: uate the changes in optical compression of patients with
as squint. This pathology is an extraocular musculature Cross-sectional clinical study. Applied questionnaire a sellar lesion, using visual computerized campimetry
dysfunction, congenital or acquired, which has high directed to patients diagnosed with MS of patients fol- (VCC) and OCT. Methods: This is a longitudinal analytical
prevalence in the population, varying from 2% to 4%. lowed up at the Hospital Universitario Walter Cantídio observational study whose design is a retrospective cohort.
Objectives: This research aims to evaluate the prevalence and Hospital Geral de Fortaleza. Data collected between By reviewing the attendance records, information was
of cervical postural alterations in patients with oculomo- January and February 2022 includes age,gender,vaccine collected regarding demographic and clinical data, data
tor disturbance, through eye motricity tests and postural used,side effects,number of MS attacks,clinical form of from the magnetic nuclear resonance of the sellar region
exams, showing the correlation between both patholo- MS,EDSS,COVID infection. Statistical analysis was descrip- and the eye examination.The research was approved by
gies. Method: The inclusive criteria to being a part of the tive. Continuous variables were described by their means the Research Ethics Committee of the Hospital, comply-
research were: patients between the ages of four and fifty and standard deviations (SD). Results: Total of 13 partic- ing with ethical principles. Results: Seven patients were
years old, carriers of oculomotor alteration and acquired ipants,10 female and 3 male. Mean age of 43.7 years,SD selected for the study, with ophthalmological assessment
cervical postural alterations. As the exclusive criteria, of 16.9.Predominance of complete higher education (5 before and after treatment. Unilateral hemianopsia was
carrier patients of congenital postural disturbs which participants) and born in Fortaleza (6 participants).Only the most common symptom, present in 57.1% of patients
present profound amblyopia, monocular or subnormal 1 patient had not been vaccinated with any dose and,of (n = 4), followed by bitemporal hemianopsia and head-
vision. Results: The obtained results demonstrated that the the 12 vaccinated,only 1 had not taken the booster dose. ache. All cases (n = 7) had suprasellar extension and com-
oculomotor disturbers carrying individuals also presented Vaccines applied in immunization: BNT162b1,ChAdOx1 pression of the optic chiasm and only 14.3% (n = 1) of the
postural alteration, the presence of several dysfunctions on and COVID-19 Adsorbed Vaccine (inactivated).4 patients cases had parasellar extension. In the analysis through
segments responsible for posture in individuals with lim- reported mild side-effects, all after ChAdOx1 vaccine.2 VCC, the mean of Mean Deviation (MD) was -21.03 dB
itations, paresis and paralysis in the ocular muscles. This vaccinated patients had COVID-19 after vaccination. ± 6.27 (-24.42 dB) in pre-treatment and 15.29 dB ± 10.32
study demonstrated the it was reported a really frequent No MS attack after any dose of vaccine.All of them use (-21, 51 dB) in post treatment. In optic nerve analysis
vicious head position condition is the eye adjustment for or have already used some disease-modifying drug,but through OCT, the mean NFL thickness was 73.86µm ±
the purpose of compensating for a squint. Beside this, the 1 does not currently use it.4 participants had attacks in 22.88 (7550µm) in the pre-treatment and 70.42 (69.50µm)
literature is still scarce, in this perspective it is clear the the last 2 years (1 unvaccinated patient with 3 attacks;1 in the post. Those who showed better thickness of the
need for further research and publications to clarify the patient with 3 attacks-only 1 attack after vaccination; MD fiber layer in the pre-tratment, also showed better
association of the two pathologies. Conclusion: The pres- and 2 patients with 1 attack before the beginning of the values in the recovery of campimetry and visual acuity.
ent study conclude that patients who carry oculomotores vaccination).The EDSS remained unchanged for all par- Patients with better NFL thickness on OCT also had the
disturbes, also present cervical postural alterations, which ticipants, except for one with a change in EDSS from 1 best results on MD in post treatment. Conclusion: These
demonstrate a meaningful percentual results to alter- to 3 due its psychotic condition (psychosis with urinary data reinforce the importance of a complete ophthalmo-
ations on the segments responsible to the maintenance incontinence). Conclusion: There was no triggering of logic evaluation as a means of determining the possibil-
of postural balance. Therefore, these patients should be new clinical MS attacks in patients vaccinated and no ity of visual improvement in the treatment of pituitary
referred to specialists for a postural assessment and, if worsening of EDSS attributable to vaccination.Side effects tumors, in addition to demonstrating its effectiveness in
possible, undergo postural reeducation treatment and to vaccination were present only after ChAdOx1 vacci- decompressing the optic pathway, especially the chiasm.
treatment of the extrinsic eye muscles. nation,with side effects already reported in the general It also suggests that the OCT should be used as a tool to
population in phase III studies. assess the patient’s visual prognosis.

Miscelânea
Miscelânea Miscelânea

279
XXX Congresso Brasileiro de Neurologia

TL 1106577 TL 1104977 TL 1105034

ROLE OF THE NERVOUS SYSTEM IN THE CLINICAL AND EPIDEMIOLOGICAL PROFILE RELATIONSHIP BETWEEN PARKINSON’S
DEVELOPMENT OF LANGUAGE AND OF PATIENTS DIAGNOSED WITH MULTIPLE DISEASE AND DEMENTIA IN A CITY IN THE
COMMUNICATION SCLEROSIS IN CASCAVEL, PARANÁ, BRAZIL INTERIOR OF MINAS GERAIS
Bruno Ricardo Leite Barboza, Tayane Moura Martins Lucas Victoy Guimarães Zengo , Luiza Orth , Igor Fortunato da Silva, Gérsika Bitencourt Santos,
Vinicius Slongo Bühler , Márcia Bernardon , Pietra Guiomar Nascimento de Oliveira
Universidade Federal do Pará. Altamira PA, Brazil Baggio Peixoto de Oliveira , Renato Endler Iachinski
, Rafaela Luiza Altheia Griza , Eduarda Basso Universidade José do Rosário Vellano. Alfenas MG,
bruno.leite1@hotmail.com Brazil
Badalotti , Gabriel Angelo Garute Zenatti , Angelo
Background: Language is essential for communication Marcelo Wosniacki Filho igor.fortunato@aluno.unifenas.br
between human beings, being responsible for the trans- Centro Universitário da Fundação Assis Gurgacz
mission of sounds, symbols, gestures and words. Several FAG Background: Parkinson’s Disease (PD) is the second most
organs and systems work together in the development of Fundação Hospitalar São Lucas FHSL common neurodegenerative disease in the general pop-
speech, writing and body expression, such as the visual ulation, after Alzheimer’s Disease (AD), with Parkinson’s
and auditory system, cerebral cortex. Objective: To under- lvgzengo@minha.fag.edu.br Disease Dementia (PDD) being a well-established com-
stand the mechanism of action of the nervous system plication of PD. About 25 to 30% of patients with PD
in language development. Material and Methods: This Background: Multiple sclerosis is a chronic autoimmune develop PDD, and the incidence of dementia in patients
is a descriptive study, through a bibliographic review, disease of the brain and spinal cord in which inflamma- with PD is about six times higher when compared to the
carried out through books and scientific articles pub- tion, demyelination and axonal loss occur from the early general population, which is cumulative with advancing
lished between 2018 and 2022 available on the Scientific stages of the disease. The onset of multiple sclerosis has age. Objective: To quantitatively correlate dementia as a
Electronic Library Online (SciELO), Latin American and been typically observed in individuals aged from 20 to 40 complication of Parkinson’s disease in a city in the coun-
Caribbean Literature in Science platforms. da Saúde years, with the female predominance. Objectives: The aim tryside area of Minas Gerais, in addition to describing the
(LILACS) available in full in Portuguese, English and of the present study was to describe the clinical-epide- clinical-epidemiological profile of these patients. Method:
Spanish versions. The keyword “neurophysiology” was miological profile of patients with multiple sclerosis who Descriptive, cross-sectional, documentary study, which
used. 34 results were found, of which 5 works were selected were diagnosed and followed at a local neurology service. used secondary data collected from 56 medical records
to compose the bibliographic review. Results: Studies Methods: The model of this study was observational and of patients diagnosed with PD from two general neurol-
show that language and communication skills are the retrospective descriptive, involving patients diagnosed ogy outpatient clinics in Alfenas and who underwent
result of the combination of the performance of specific with multiple sclerosis in the last two years and followed consultation during the years 2020 and 2021. Results: Of
areas in the cerebral cortex: Broca’s, Wernicke’s, angular in a neurology service in the city of Cascavel, Paraná (PR). the 56 medical records reviewed, 31 had some degree of
gyrus and part of the motor cortex. Broca’s area, located The epidemiological variables were gender, age, clinical dementia on clinical examination, but 14 patients were
close to the lateral sulcus, is responsible for the articu- presentation, patient age at diagnosis, patient age at excluded from the study because they did not meet the
lation and motor coordination of the pronunciation of onset, predominant symptoms, use of disease-modifying criteria for diagnosing PDD or had other clinical entities
words. Wernicke’s area, located in the temporal lobe, drugs and EDSS (expanded disability status scale). The besides PD that could justify dementia. Therefore, of the
provides understanding and meaning for the language clinical presentation was defined in the following cate- 42 patients selected, 17 had dementia with probable
used in communication, as does the area of ??the angular gories: relapsing-remitting, secondary progressive and cause of PD (40.48%). Furthermore, of these 42 patients,
gyrus, which makes language intelligible through reading. primary progressive. Results: The sample size presented 25 were men and 17 women. Of the 17 patients with
In addition, there is also the participation of the motor a number (n) of 39 participants diagnosed with multiple PDD, the mean age was 70.86 years, with a mean time of
cortex – which is divided into primary, pre-motor and sclerosis, with a predominance (79%, n = 31) of female diagnosis of PD of 5.14 years, 9 were women and 8 men;
supplementary areas, which enables speech vocalization patients (female: male ratio = 4: 1). The raw prevalence of of the 25 who did not have dementia, the mean age was
and dominant body expression in non-verbal language. multiple sclerosis was determined to be 11.6 per 100.000. 67.32 years, with a mean time of diagnosis of PD of 4.25
These areas work in complex neural networks together Concerning the age, the average was 36 years and 69% (n years, with 8 females and 17 males. Of the motor signs and
with sensory and memory information for the execution = 27) were aged between 20 to 40 years. The relapsing-re- symptoms, some degree of bradykinesia was observed in
of the different types of language used in communication mitting clinical form was predominant (87%, n = 34), fol- 42, plastic rigidity in 40, postural instability in 32, tremor
and send the information to the skeletal muscles, mainly lowed by primary progressive form (7%, n = 3) and only at rest in 30, and difficulty in swallowing in 10 patients.
through the corticospinal tract and the innervation from one case of secondary progressive form. The average of Other frequent diagnoses in these patients were: arterial
the brainstem. Conclusion: The neural circuitry involving the EDSS score was 1. At the time of diagnosis they had hypertension in 22, insomnia in 17, depressive disorder
the cerebral cortex and the conduction pathways make it a median age of 32 years and at the first onset they had in 13, hypothyroidism and REM sleep behavior disorder
possible to maintain the high complexity of the patterns a median age of 27 years. As initial signs and symptoms, in 12, anxiety disorder, hallucinations and excessive day-
of the skills in question, and the involvement of lesions in 35% (n = 14) reported paresthesia, 28% (n = 11) reported time sleepiness in 9, diabetes in 7, heart disease in 6 and
these places interferes with the quality of communication. hemiparesis, 23% (n = 9) reported dizziness, 20% (n = 8) arrhythmia in 4. Conclusion: The proportion of patients
reported diplopia or blurred vision, 7% (n = 3) had disme- with PPD found in this study (40.48%) is in line with the
tria. Other symptoms include urinary urgency, holocra- global average. Resting tremor is the motor symptom that
Miscelânea nial headache and syncope. In the sample, 76% (n = 30) best responds to pharmacological therapy, and difficulty
use some disease-modifying drug. Among these, 43% (n in swallowing is common in these patients.
= 13) use dimethyl fumarate, 20% (n = 6) use glatiramer
acetate, 16% (n = 5) use natalizumab. Other medications
include ocrelizumab, interferon beta-1a, teriflunomide Neuroepidemiologia
and fingolimod. Conclusion: The clinical-epidemio-
logical profile and prevalence of multiple sclerosis in
Cascavel (PR) is considered average in accordance with
most brazilian studies.

Neuroepidemiologia

280
XXX Congresso Brasileiro de Neurologia

TL 1105054 TL 1105096 TL 1105125

EPIDEMIOLOGICAL PROFILE OF LEPROSY IN STROKE PROFILE AND CARE DURING THE THROMBOLYTIC THERAPY IN THE STROKE
AN AMAZON HEALTH REGION COVID-19 PANDEMIC: THE JOINVILLE’S UNIT OF A HOSPITAL IN NORTHEASTERN
FIRST YEAR EXPERIENCE BRAZIL – EXPERIENCE IN THE LAST 5 YEARS
Leandro Araújo Costa, Márcio Alex Reis Câmara,
Claudio Joaquim Borba Pinheiro, Greice de Lemos Felipe Ibiapina Reis, Pedro Silva Correa Magalhães, Maria Eduarda Costa Brandão Justino, Pedro Lucas
Cardoso Costa, Silvio Henrique dos Reis Junior, Henrique Diegoli, Juliana Safanelli, Vivian Nagel, Negromonte Guerra, Vitor Maia Arca, Monique
Izaura Maria Vieira Cayres Vallinoto Alexandre Luiz Longo, Carla Heloisa Cabral Moro, Evelyn Mendonça do Nascimento, Ana Dolores
Marcos Christiano Lange, Viviane Hiroki Flumignan Firmino Santos do Nascimento
Universidade Federal do Pará. Belém PA, Brazil
Zétola
Instituto Federal do Pará. Tucuruí PA, Brazil Faculdade Pernambucana de Saúde. Recife PE,
Universidade do Estado do Pará. Belém PA, Brazil UNIVILLE, Joinville SC, Brazil Brazil
Universidade do Estado do Pará. Tucuruí PA, Brazil Hospital Municipal São José. Joinville SC, Brazil Univesidade Federal de Pernambuco. Recife PE,
Universidade Federal do Paraná. Curitiba PR, Brazil Brazil
leandrocosta.dr@gmail.com
Hospital das Clínicas da Universidade Federal de
reisfelipeir@gmail.com Pernambuco. Recife PE, Brazil
Background: Leprosy is a chronic, granulomatous, infec-
Hospital da Restauração. Recife PE, Brazil
tious disease, caused by the bacterium Mycobacterium Background: The SARS-CoV-2 coronavirus pandemic
leprae, also called Hansen’s bacillus, an alcohol-acid resis- (COVID-19) has become one of the hardest challenges meduardacbj@gmail.com
tant bacillus, which has the characteristic of being obliga- nowadays. Public managers and health teams have con-
tory intracellular, demonstrating a peculiarity of predilec- cerned about social restrictions, lack of resources and a Background: In Brazil, the incidence of ischemic stroke
tion for skin cells and cells. peripheral nerves, which settles possible negative impact on incidence and quality of ranges from 105 to 137 per 100,000 inhabitants (1). It is
in the infected person’s body and can multiply. By 2005, care for Stroke patients. Objective: We aim to investigate the second cause of death and the leading cause of per-
leprosy elimination had been achieved in all countries the impact of COVID-19 on incidence and management manent disability in the adult population. Thus, reper-
except: Angola, Brazil, India, Madagascar, Mozambique, of Transient Ischemic Attack (TIA), Stroke and its sub- fusion treatment is a goal to be achieved in patients with
Nepal, Central African Republic, Democratic Republic of types in Joinville, Brazil. Methods: We did a prospective acute ischemic stroke (2) and, stroke units have a funda-
Congo and Tanzania. Brazil has an average detection rate of population-based stroke registry of all first-ever cases of mental role related to the organized and fast care of such
12.2 cases/100,000 inhabitants, being classified as having Transient Ischemic Attack (TIA), Stroke and its subtypes individuals (3). However, the pandemic of the disease
a high burden for the disease and, in the world ranking, admitted to health services in Joinville since the begin- by coronavirus brought with it barriers for reperfusion
as the 2nd country with the highest number of new cases ning of restrictions due to COVID-19 (defined as March, therapies in acute stroke, such as the closing of several
registered in the world, second only to India. Objectives: 2020) and the same period one year before. A comparative stroke units around the country (4). In this study we will
To trace the epidemiological profile of the leprosy in the analysis was made between these two periods (12 months present the profile of cerebral ischemic patients under-
11th Health Region of Pará, from 2010 to 2020. Method: before versus 12 months after COVID-19 outbreak). The going thrombolysis in the stroke unit of a large Hospital
This is a cross-sectional, descriptive, retrospective epi- patients’profile, incidences of TIA, stroke and its sub- in the Northeast of Brazil in the last 5 years. Objective:
demiological study with a quantitative approach, based types, severity, reperfusion therapies, in-Hospital stay To describe the evolution of thrombolytic therapy in
on secondary data collections, reported in the System and workout and in-Hospital lethality in these periods the stroke unit of the Hospital da Restauração over the
of Notifiable Diseases Information (SINAN), from 2010 were compared. Results: The analysis showed a signif- past 5 years. Methods: We conducted a cross-sectional,
to 2021. The data were organized in Excel spreadsheets icant incidence reduction of TIAs (32,8%; p=0,002). The observational, retrospective study that included patients
and statistically analyzed in Bioestat 5.3. Results: During rate of thrombolysis (IV) and trombectomy (MT) did not with ischemic stroke undergoing chemical thrombolysis
the period from 2010 to 2020, 9,863 cases of leprosy were change. The intervals from admission to IV/MT were sim- in the period from June 2018 to May 2022 admitted to
reported in the 11th Health Region of Pará, with 7,966 new ilar in both periods. Patients with ischemic stroke caused the neurological emergency of Hospital da Restauração,
cases, with an average of 896 notifications/year. Despite by atrial fibrilation had their in-Hospital stay abbreviated Recife, Pernambuco. Results: 269 patients underwent
the large decline in the annual detection rate of leprosy during the pandemic. Workout investigation was similar chemical thrombolysis in the period studied. There was
in the region, based on the first and last year of the survey before and during the pandemic, with an increase in a predominance of males (51.7%) and a mean age of 65
(2010 – 2020), the region is still in the Very High Endemicity number of Cranial Tomography (p=0.02), transtoracic years. Our study, therefore, reaffirms the profile found
profile, with 29 cases per 100,000 inhabitants/year. The ecocardiogram (p=0.001), chest X-rays (p<0.001) and in the literature (5). Despite the male predominance,
profile of leprosy cases in the region is predominantly Transcranial Dopplers (p <0.001). During COVID-19 pan- in 2021 and in the first five months of 2022 there was a
male (62.4%), between 15 and 50 years old (60.6%), brown demics, in-Hospital lethality did not change. Conclusion: female predominance of 57.1% and 56.8%, respectively.
(67.8%) and with a low level of education (73.9%). Mostly We concluded that COVID-19 Pandemic in Joinville was Prior to the closure of the stroke unit due to the COVID-
Multibacillary (68%) and Dimorphic (57%), reflecting associated with lower TIA incidence, without negative 19 pandemic, from June/2018 to April/2020, 174 patients
high percentages of disabilities (27%) and new cases influence over quality of care, workout investigation and were thrombolyzed. In contrast, we noticed a sharp drop
in children under 15 years of age (12.6%). Conclusion: in-Hospital management of stroke patients. Studies are in the number of patients undergoing reperfusion ther-
It is concluded that leprosy is a public health problem in needed to understand the real reasons for this epidemio- apy in the period when the unit was deactivated in May
the region, with high rates of disability, under 15 years- logical behavior. We believe that fast and strategic response 2020 to the end of November 2021, totaling 30 patients.
old and hidden cases, requiring interventions in various to unusual pandemic challenges can explain good results After reopening, we had a significant increase and by
spectrums, such as active casework, better monitoring e minor hazards in stroke prevention and caring. May 2022, 65 patients were thrombolyzed. Conclusion:
of the treatment, training of the professionals about the Our study showed a significant increase in the number
disease and its attributions, as well as health education of patients undergoing thrombolysis over the years. The
for the population on the subject. Neuroepidemiologia closure of the stroke unit resulted in a decline in the
number of patients treated in the period of the COVID-
19 pandemic. With this, it is shown the importance of the
Neuroepidemiologia units for a better assistance to patients who need care in
the acute phase of stroke.

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IDIOPATHIC INTRACRANIAL VIRAL MENINGITIS: AN EPIDEMIOLOGICAL EPIDEMIOLOGIC ASSESMENT OF


HYPERTENSION MORTALITY IN BRAZIL: AN ANALYSIS IN ONE DECADE IN BRAZIL WITH HOSPITALIZATION RATES FOR
EPIDEMIOLOGICAL ANALYSIS THE IMPACT OF THE COVID-19 PANDEMIC MENINGOCOCCAL DISEASE IN BRAZIL
Luciano de Albuquerque Mota, Isabelle Diniz Melo, Luciano de Albuquerque Mota, Isabelle Diniz Melo, Sophia Costa Vasconcelos, David Augusto Batista
Gabriel Magalhães Santos, Caio Oliveira Cavalcante, Gabriel Magalhães Santos, Caio Oliveira Cavalcante, Sá Araújo, Pedro Lucas Grangeiro de Sá Barreto
Luís Eduardo de Medeiros e Silva, Rodrigo Becco Luís Eduardo de Medeiros e Silva, Rodrigo Becco Lima, Emanuel Assis Bertolino Martins Gomes,
de Souza de Souza Lucas Soares Radtke, Carolina de Figueiredo
Santos, Paulo Ribeiro Nóbrega
Universidade Federal do Ceará. Fortaleza CE, Brazil Universidade Federal do Ceará. Fortaleza CE, Brazil
Hospital Universitário Walter Cantídeo. Fortaleza Hospital Universitário Walter Cantídio. Fortaleza Universidade Federal do Ceará. Fortaleza CE, Brazil
CE, Brazil CE, Brazil Hospital Infantil Albert Sabin. Fortaleza CE, Brazil

lucianodealbuquerquemota@hotmail.com lucianodealbuquerquemota@hotmail.com sophia.costava@gmail.com

Background: Idiopathic Intracranial Hypertension is Background: Accordingly, to the brazilian Ministry of Background: Neisseria meningitidis is an important cause
an unexplained increase of intracranial pressure, which Health between 2007 and 2020 more than 265 thousand of bacterial meningitis and sepsis worldwide, with twelve
is defined by the relationship between the cranial com- cases of meningitis were confirmed. Viral meningitis recognised serogroups that can lead to meningococcal
ponents (brain, cerebrospinal fluid, and blood) and its was responsible for 45,9% of the confirmed cases. The infection. This disease represents a signicant burden to
volume, a constant. Objective: To compare, through a social isolation of the COVID-19 could have affected society due to its high morbidity and mortality rates, thus
prevalence analysis, the mortality of Idiopathic Intracranial this scenario. Objective: Analyze the latest epidemio- it remains relevant to discuss its epidemiology. Objective:
Hypertension among the brazilian regions in the one logical findings of viral meningitis among the brazilian The aim of this study was to evaluate the annual variation
decade (2011-2020). Methods: Epidemiological, retro- regions in the last decade (2012-2021) and the impact of of Hospitalization rates (HR) for meningoccocal disease
spective, descriptive study, carried out with data obtained the COVID-19 pandemic. Methods: Epidemiological, by age, in Brazil, from 2011 to 2020. Methods: Data were
from the Mortality Information System (SIM/SUS) and retrospective, descriptive study, carried out with data obtained from the Hospital Information System (SIH/SUS)
data published by the brazilian Institute of Geography obtained from the Hospital Information System (SIH/ and from the brazilian Institute of Geography and Statistics
and Statistics (IBGE). From these data, the number of SUS) and data published by the brazilian Institute of (IBGE), both of which are governmental datasets fed with
deaths per million people of each brazilian region per Geography and Statistics (IBGE). From these data, the health reports and demographic information, respectively.
year of the period was calculated. For a more elaborate number of Hospitalizations per million people of each Using the number of Hospitalizations and deaths due to
analysis, the variables age group of patients and gender brazilian region per year of the period was calculated. menincoccocal infection as well as population projec-
were considered. Results: In 2011, the national aver- For a more elaborate analysis, the variables, age group tions, it was possible to estimate the yearly Hospitalization
age of deaths per million from Idiopathic Intracranial of patients, gender, value of Hospital services, average rates for each age group. A temporal analysis was then
Hypertension in Brazil was 0,63. The Southern Region length of stay and the mortality rate per year of care were performed using a joinpoint regression model, by means
featured the lowest mortality rate, with 0,47 deaths per considered. Results: In 2012, the Hospitalization rate for of which it was obtained the Annual Percent Changes
million, and the Northern Region featured the highest viral meningitis per million people was 20,70 in Brazil. (APC) with a 95% confidence interval (CI). Results: In
rate, with 0,68. Furthermore, males were proven more The Southern region had the highest rate (24,92) and the referred period, a total of 16,984 Hospitalizations for
vulnerable than females, spanning 57% of total deaths the Northern region had the lowest (9,44). The national meningococcal disease were registered in the country. In
that year. Regarding age group, in 2011, deaths were more cost per admission was R$ 892,78, and the 1-4 years old 2011, Brazil presented a Hospitalization rate of 18,8 per
prevalent in the groups ranging from 20-29 and 50-59 years age group was the most affected, representing 19% of the one million inhabitants. Regarding the general population,
old, while they were less prevalent in the group ranging Hospitalizations. Males represented most admissions the HR presented a decreasing trend the following years,
from 10-14. In 2020, the national average of deaths per (58%), and the average duration of Hospital stay was with an APC of -16,8 (CI95%= -19,6;-13,8). Interestingly,
million was 0,40, representing a 37% drop when compared 7,2 days with a mortality rate of 3,04. By the end of 2021, if na inflection point is established in 2015, it is observed
to the beginning, in which the Mid-Western Region had Hospitalization rate per million was 4,95, representing a a more accentuated decline from 2011 to 2015 than from
the biggest decrease (71%), and the Northeastern Region 76% decrease. The Southern region continued with the 2015 to 2020. Hospitalization rates have also been observed
had the lowest decrease (17%). In that regard, the region highest rate, 8,26, while the Northeastern region had the to follow a lowering tendency for each age group anal-
with the biggest mortality rate is the Northeastern, with lowest rate with 3,02. The average cost per admission in ysed. The greatest Annual Percent Change was found for
0,58, while the lowest rate goes to the Mid-Western Region, 2021 was R$ 1341,33, 50% more expensive than in 2012. the age range from 5 to 19 years old, with an APC of -21,29
with 0,18. In relation to gender, the male population had Moreover, the below 1 year old age group was the most (CI95%= -25,6;-16,5). In contrast, the age group of 60 or
a reduction of 29% while the female had a reduction of affected, while the age group of 5-9 years old had the older displayed the smallest APC (APC= -9,63; CI95%=
33%. Moreover, the age group of 20-29 years old had the highest variation, with a decrease of 88%. The male sex -12,2; -7,0). The age group from 0 to 4 years old held
biggest decrease in that period (76%), and the age group remained the most affected, with 54%. Besides, the aver- the highest Hospitalization rates troughout the period,
of 50-59 years old, besides having a reduction of 24%, kept age duration of Hospital stay in 2021 was 8 days, and the with a HR of 12,86 per million at the end of the observed
the highest average, with 20,6 deaths. Conclusion: This national mortality rate was 5,88, 93% higher than in 2012. period. Conclusion: The results of this study showed that
analysis allowed a better understanding about the impact Conclusion: This study made it possible to identify the the Hospitalization rate for menincococcal disease has
of Idiopathic Intracranial Hypertension in Brazil, since, population most affected by viral meningitis in Brazil, and been decreasing steadily in the past decade. Nevertheless,
despite an important decrease in mortality rate during the changes sustained in the last decade. Moreover, the this disease remains very relevant, particularly among
the period of 2011-2020, keeps being a relevant cause of impact of the COVID-19 pandemic and its forced social younger age groups, pointing to the necessity of constant
death among the population, mostly affecting the males, isolation should be further analyzed since this study improvement in regards to its control and prevention.
the age group between 50-59 years old and the country’s demonstrated a big decrease of the Hospitalization rate
Northeastern Region. but an associated rise in mortality and length of stay.
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MORTALITY FROM GUILLAIN-BARRÉ PROFILE OF PATIENTS ADMITTED TO A PREVALENCE AND CLINICAL ASPECTS
SYNDROME IN BRAZIL: A TEN-YEAR STROKE UNIT IN NORTHEASTERN BRAZIL ASSOCIATED WITH COGNITIVE DECLINE
RETROSPECTIVE ANALYSIS AND ITS QUALITY INDICATORS IN THE ELDERLY IN THE INTERIOR OF THE
AMAZON
Emanuel de Assis Bertulino Martins Gomes, Pedro Maria Eduarda da Costa Brandão Justino, Pedro
Lucas Grangeiro de Sá Barreto Lima, Fernando Lucas Negromonte Guerra, Vitor Maia Arca, Lohana Guimarães Souza, Luciane Aparecida
Degani Vazquez, Luciano de Albuquerque Mota, Monique Evelyn Mendonça do Nascimento, Ana Gonçalves Manganelli, Calila Oliveira Alves, Brenda
Lucas Soares Radtke, Maria Suelly Nogueira Dolores Firmino Santos do Nascimento Santana Almeida, Murilo Sousa Ramos, Tiótrefis
Pinheiro, Geovanni Guimarães Bandeira, Francisco Gomes Fernandes, Renato Campos Freire Júnior,
Matheus Oliveira de Carvalho, Pedro Helder de Faculdade Pernambucana de Saúde. Recife PE,
Grasiely Faccin Borges
Oliveira Junior Brazil
Universidade Federal de Pernambuco. Recife PE, Universidade Federal do Sul da Bahia. Teixeira de
Universidade Federal do Ceará. Fortaleza CE, Brazil Brazil Freitas BA, Brazil
Hospital Universitário Walter Cantídio. Fortaleza Hospital das Clínicas da Universidade Federal de Universidade Federal do Amazonas. Manaus AM,
CE, Brazil Pernambuco. Recife PE, Brazil Brazil
Hospital da Restauração. Recife PE, Brazil
emanueldeassis@alu.ufc.br lohana.souza@cja.ufsb.edu.br
meduardacbj@gmail.com
Guillain-Barré Syndrome (GBS) is a polyradiculoneuropa- Background: Dementia represents a worldwide pub-
thy of autoimmune etiology, considered the leading cause Background: Stroke is one of the leading causes of mor- lic health problem, due to the extent and severity of
of acute flaccid paralysis in the world. This syndrome is tality and morbidity worldwide (1). To improve this sce- its functional, psychological and social manifestations,
characterized by ascending motor and sensory symp- nario, it is necessary to evaluate reliable data to improve both for the elderly and their families. Advanced age, low
toms, also presenting varied clinical forms in part of the the standards of Hospital care. Ordinance No. 665, pub- level of schooling and socioeconomic conditions have
patients, such as Miller Fisher syndrome and Bickerstaff lished in 2012 by the Ministry of Health, provides for the a great impact on the cognitive decline of the elderly.
encephalitis, whose presentations include cranial nerves implementation of stroke units, as well as the inclusion Objective: To analyze the prevalence and clinical aspects
involvement, altered coordination, and involvement of of quality indicators to monitor their management (2). associated with cognitive decline in the elderly in the
the level of consciousness. This study aims to evaluate Objective: To describe the characteristics of patients interior of Amazonas. Method: This is an observational,
the mortality from Guillain-Barré Syndrome during the admitted to the stroke unit during the implementation cross-sectional population-based study, approved by the
last decade (2011-2020) in Brazil, comparing descriptive of quality indicators in the service. Method: We con- Research Ethics Committee (CAAE nº 249.098/2013) and
variables: region, age group and gender. The analysis was ducted a cross-sectional, observational, retrospective carried out in Coari/AM. After performing the sample
conducted through an epidemiological, retrospective, study that included patients admitted to the stroke unit calculation, cluster sampling was performed. Inclusion
descriptive and cross-sectional study, carried out with of the Hospital da Restauração, in Recife, Pernambuco, criteria were: 60 years or older and resident in the urban
information released by the Mortality Information System during the period from March 2022 to the beginning of area of ??Coari. In the data collection, a socioeconomic,
(SIM/DATASUS) and the brazilian Institute of Geography May 2022. Results: The studied stroke unit has 12 beds, demographic, activities of daily living, cognitive status,
and Statistics (IBGE). From these data, mortality per 1 one of which is permanently available for venous throm- depression, health status and independence measure were
million inhabitants of each region per year was calculated bolysis. All beds are monitored, there is availability of applied. Data were tabulated in electronic spreadsheets
and adjusted for the descriptive variables. In the decade daily physiotherapy and speech therapy assessment; and analyzed using Jamovi software. Results: The study
analyzed, 1875 deaths from GBS were recorded in Brazil, nurse 24h/7 days and neurologist available for evolution was carried out with 274 elderly people, 63.1% female,
of which 47.4% occurred in the Southeast region. In 2011, and reassessments, as well as full time acute treatment. with a mean of 71.6 years (SD 8.03) and a mean of 3.01
the country had an mortality rate of 0.7 deaths per million In the period studied, there were 123 Hospitalizations, years (SD: 4.03) of study. Most had a family income of 1
inhabitants for this syndrome, with the Southeast region of which 54.5% were for ischemic stroke (n=67) and 4.9% to 2 minimum wages (62.9%) and did not have caregiv-
having the highest mortality rate (1), and the North region, were TIA (n=6), and the rest were for HCVA. There was a ers (90.4%). Regarding the perception of health, 55.1%
the lowest (0.4). In 2020, an average of 1 death per mil- predominance of females (52.8%) and a mean age of 68 reported regular health and 24.8% good health. Among the
lion inhabitants was observed, showing an increase of years. The proportion of patients treated with reperfu- reported comorbidities, Systemic Arterial Hypertension
approximately 40% compared to 2011. At the end of the sion therapy was 14.6% (n=18). The mean score on the (48.2%), heart disease (17.2%), Diabetes Mellitus (16.1%),
decade, the Midwest region had the highest mortality rate NIH stroke scale was 12.6 (SD: ± 8.4). All patients were Stroke (12.8%), dementia (5.5%) prevailed. ) and cancer
per million inhabitants (1.6), representing an increase of evaluated by physical and speech therapy. 40.7% of the (2.2%). About 31.2% had mild depression on the Geriatric
149% compared to 2011, while the Northeast region had patients admitted to the stroke unit had as a reason for Depression scale, 30.2% had mild cognitive loss on the
the lowest rate (0.7), corresponding to an increase of 36% discharge the transfer to other sectors of the Hospital, Mini Mental State Examination (MMSE), and 28.1 had
compared to 2011. Regarding age group, people aged and only 1 patient died during Hospitalization. Many moderate cognitive loss. Both obtained, respectively,
over 50 years accounted for 40% of the deaths caused by other data were collected, however, as it is a pilot project an average of 5.50 points (SD: 4.57) and 23.3 points (SD:
GBS. Moreover, the average annual number of deaths per for the implementation of indicators, the collection was 4.62). The MMSE showed a moderate negative correlation
million inhabitants was 0.7 for women and 1.14 for men, incomplete, mainly due to the doctors and nurses team with knowing how to read and write (Pearson’s r -0.501;
an average annual difference of 62.8% between the two not filling out the form. Conclusion: This study allowed p <0.001). Regarding the performance in Instrumental
groups, there was also a difference of 56.5% between the corroborating the importance of the adequate filling of Activities of Daily Living according to the Lawton scale,
number of deaths accumulated by each of the genders quality indicators in the stroke unit by the multiprofes- 34% have mild dependence, with an average of 24.9
in this period. Conclusion: This study provided a better sional team, as a validated instrument to guide the care (SD: 2.84). Conclusion: It is observed in the study that
understanding about the mortality of the Guillain-Barré provided to patients with cerebral ischemia. the elderly presented moderate to mild cognitive loss,
Syndrome in Brazil, showing particularities in the profile mild depression and mild dependence. These results:
of patients and the heterogeneity of mortality rates among are similar to those found in other regions of the country.
different regions. However, it was not possible to identify Neuroepidemiologia
complete data about the incidence of GBS, limiting the
scope of the study. Neuroepidemiologia

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PREVALENCE AND CLINICAL ASPECTS OF MULTIPLE SCLEROSIS MORTALITY IN EPIDEMIOLOGICAL PROFILE OF CONGENITAL
COGNITIVE DECLINE IN INSTITUTIONALIZED BRAZIL: A RETROSPECTIVE ANALYSIS FROM ZIKA VIRUS SYNDROME (SCZ) IN CEARÁ
ELDERLY IN THE INTERIOR OF BAHIA 2016 TO 2020 BETWEEN 2015 AND 2021: A CRITICAL VIEW
ON THE NOTIFICATIONS
Lohana Guimarães Souza, Calila Oliveira Alves, Rodrigo Mariano Ribeiro, José Arnaldo Mota
Vinicius Teixeira Bravim, Caroline da Silva Meira, Arruda, Anna Melissa Noronha Oliveira, Sophia Mariana Carmo Freitas, Lays Katharina Assis
Beatriz Rihs Matos Tavares, Grasiely Faccin Borges, Costa Vasconcelos, Luciano de Albuquerque Mota, Coppieters, Lázaro Freire Silva Filho, Thiago Barbosa
Ana Paula Pessoa de Oliveira Emanuel de Assis Bertulino Martins Gomes, Lucas Vivas
Soares Radtke, Fernando Degani Vazquez, Maria
Universidade Federal do Sul da Bahia. Teixeira de Suelly Nogueira Pinheiro, Pedro Vitor Ferreira UNIME. Lauro de Freitas BA, Brazil
Freitas BA, Brazil UNINOVAFAPI. Teresina PI, Brazil
Universidade Federal do Ceará. Fortaleza CE, Brazil
lohana.souza@cja.ufsb.edu.br mariana.hfreitas@outlook.com
rodrigo.marianoribeiro@hotmail.com
Background: Aging, although inherent to life, is cor- Background: Zika Virus is transmitted by the Aedes
related to cognitive decline and establishment of comor- Background: Multiple Sclerosis (MS) is an inflammatory aegypti mosquito and its infection has as its main symp-
bidities. This causes problems, such as forgetfulness; and chronic immune-mediated disease, being the most toms myalgia, headache, and fever. During pregnancy,
disturbances in attention, concentration and reasoning; common primary demyelinating pathology of the cen- contamination by the Zika virus carries a high risk of
physical dysfunctions; dependency; and institutional- tral nervous system. It presents a high diversity of motor developing a congenital malformation called microceph-
ization. Objective: To assess the prevalence of cognitive and sensory clinical manifestations, with a progressive aly, characterized by Congenital Zika Virus Syndrome
decline in institutionalized elderly people in a city in degenerative evolution, and usually affects young and (SCZ), in which a newborn (NB) is born with a head cir-
the interior of Bahia. Method: This is a cross-sectional, middle-aged adults. Objective: The aim of this study was cumference smaller than expected (32 cm). Objective:
observational and descriptive study, approved by the CEP, to assess MS mortality rates in Brazil over the five years To delineate the epidemiological profile of SCZ in Ceará
through CAAE No. 92726218.0.0000.8467 and carried out 2016-2020, with a comparative analysis of the descriptive between the years 2015 and 2021, identifying the risk
in a long-term care institution for elderly people (LTCI) in variables sex, age, and region. Methods: The research was factors of this condition. Methods: This is a descriptive
Teixeira de Freitas/BA. It included elderly people of the carried out through an epidemiological, retrospective, epidemiological study that used secondary data from
age of 60 years old or older living in the LTCI for at least descriptive, and cross-sectional study of information Tabnet, being tabulated in Microsoft Excel®, according
6 months. Elderly people with severe mental disorders available at SIM/DATASUS and IBGE. Subsequently, to notifications of suspected cases of SCZ (DATASUS).
were excluded. Questionnaire about sociodemographic annual mortality per million inhabitants was calculated Results: In the period analyzed, 924 cases of SCZ were
and health aspects was applied, and the Mini Mental State by region of the country, with subsequent adjustment notified. The most affected maternal ethnicities were
Examination (MMSE) for cognitive assessment. The sam- of the descriptive variables sex and age. Results: There brown (71%) and uninformed or ignored (23.38%). The
ple is made up of 28 elderly people, which is higher than were 1518 deaths from MS during the 2016-2020 five- average age group of the most affected mothers was 20
the estimated population for a confidence level of 95% year period, among which 922 (60.74%) were female to 29 years (42.1%), with the two least affected being 45
and sampling error of 10%. Data were tabulated in spread- deaths, while 596 (39.26%) were male. In addition, there to 49 (0.32%) and under 15 years (0.65%). The gestational
sheets and analyzed using the Jamovi software using the was a predominance of deaths in the Southeast region, age at which the most significant congenital abnormal-
Spearman test, considering p<0.05. Results: Most of the with a total of 695 (45.78%). However, in the analysis of ity was detected was >27 weeks (3rd trimester) (66.99%).
elderly were men (60.71%); browns (50%); coming from the number of deaths per million inhabitants, the South Among the congenital alterations identified between
the interior of Bahia (100%); aged between 70-79 years region, with an average of 2.13 deaths/million inhab., 2015 and 2021, exclusive microcephaly was the most
(39.28%), ±76.5 years (SD: 10.6); institutionalized for at presented the highest rate over the five years of analy- prevalent (63.43; 79.43%), followed by microcephaly with
least 3 years (39.28%); without a partner (89.28%); with sis. In sequence, in descending order, are the Southeast Central Nervous System (CNS) alterations (8.86; 11.09%).
no or only one child (60.71%) and no schooling (57.14%). region (1.58 deaths/million inhab.), Midwest (1.55 deaths/ Almost all cases of SCZ had no detection of concomitant
Systemic Arterial Hypertension was the most prevalent million inhab.), Northeast (1.03 deaths/million inhab.) neurological impairment (910; 98.4%). In the year 2016
pathology (60.71%), followed by Brain Stroke sequelae and, finally, in the North (0.95 deaths/million inhab.). (449 cases), there was the most intense underreporting
(32.14%) and low visual acuity (21.42%). It was found that It is worth noting that the mortality profile has changed frequency by etiology, because there is an amount of
89.28% of the sample showed cognitive decline. The main little over the years of work analysis. Furthermore, in the unreported, ignored and/or unknown etiology in 372
correlation identified concerns age, being moderately analysis of mortality by age, the age range younger than cases (82.85%). From the studied period, 2018 to 2021,
negative (Spearman’s r = -0.424 and p=0.027). The most 19 years old presented the lowest number of deaths in the the underreporting of microcephaly cases became even
compromised domains, respectively, were ‘registration’ period. From adulthood onwards, the constant growth in more exorbitant, as 94.66% fell into the unknown etiol-
and ‘attention and calculation’, with r equivalent to 0.283 mortality was remarkable until reaching the peak, in the ogy. The percentage difference between the genders of
(p=0.034) and 0.394 (p=0.042), respectively, expressing range of 50 to 59 years, with a total of 383 deaths from MS NB with microcephaly in the analyzed period is 15.91%,
moderate correlation with cognitive decline. On the other (25.23%), followed by the age cut from 60 to 69 years, with with males being the most affected (55.74%). However, in
hand, the ‘memories’ and ‘spatial orientation’ domains 342 deaths (22.52%), with a subsequent decline in rates 4.44% of cases, there was no information, or the gender
showed less commitment, with r values equal to 0.615 with advancing age. Conclusion: Given the data analyzed, of the NB was ignored. Conclusion: It is not possible to
(p=<0.001) and 0.510 (p=0.07). Conclusion: It was verified it was clear that the South region had the highest mortality delineate precisely the epidemiological profile of the SCZ
a degree of cognitive impairment in a significant portion of per million inhabitants, followed by the Southeast region. given the intense underreporting, among them: etiology
institutionalized elderly, corroborating national studies on In addition, mortality from MS in women was substantially and ethnicity, being the most important in the diagnostic
the subject. Investigations are necessary to develop assis- higher than mortality in men. Finally, the age group that context. There was a relevant distinction between gen-
tance resources to stimulate the affected cognitive areas. comprises the end of adulthood and the beginning of the ders, which hypothesizes that males are more exposed
elderly was the one with the highest number of deaths. to the condition.
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EPIDEMIOLOGICAL PROFILE OF ACUTE TEMPORAL TRENDS OF THE TRENDS OF THE MORTALITY RATE OF
FLACID PARALYSIS IN BRAZIL: 2015-2020 HOSPITALIZATION RATE OF TRAUMATIC BRAIN INJURY IN BRAZIL
MENINGOCOCCAL INFECTION IN BRAZIL BETWEEN 2011-2021
Jhonata Gabriel Moura Silva, Ana Carolina
Nascimento de Sousa, Arthur Costa Junger, Fábio BETWEEN 2011 AND 2021
Gabriel Montenegro Ribeiro da Silva Cavalcante,
Pereira da Silva Júnior, João Victor da Cunha Silva, Gabriel Montenegro Ribeiro da Silva Cavalcante, Ana Clara Tavares de Melo, Giovana Carvalho de
Lays Saraiva Rodrigues Carvalho, Lorena da Silva Átila de Oliveira Melo, Laysa Moreira Campos Costa, Resende, Beatriz Caldas Gonçalves, Gabriel Souza
Viana, Luiz Felipe Bezerra de Sousa, Nínivi Daniely Vinícius Pereira de Abreu, Marcos Paulo do Carmo Dias Cardoso, Júlia Rodrigues Picazo Rigueiral,
Farias Santos, Eduardo Mariano Carvalho Silva Assunção, Railson Cipriano Regis, Thiago Martins de Carolina Daher de Alencar Neves, João Vitor Gomes
Abreu, João Felipe da Silva Mielke, Beatriz Santana da Silva, Beatriz Santana Borges
Universidade Federal do Maranhão. Imperatriz MA,
Brazil Borges
Universidade Federal de Goiás. Goiânia GO, Brazil
Universidade Federal de Goiás. Goiânia GO, Brazil UniEvangélica. Goiânia GO, Brazil
jhonata.moura@discente.ufma.br
Universidade Federal de Jataí. Goiânia GO, Brazil Universidade Federal de Goiás. Goiânia GO, Brazil
Background: Guillain-Barré syndrome (GBS) is a acute gabriel.montenegro@discente.ufg.br gabriel.montenegro@discente.ufg.br
inflammatory polyneuropathy, currently considered the
most frequent cause of acute flaccid paralysis (AFP) in chil- Background: Meningitis is one of the most feared issues Background: Traumatic Brain Injury (TBI) is a disorder
dren after polio eradication in developed countries. It is in the medical community. The infection by the Neisseria caused by a sudden and external assault responsible for
characterized by acute paralysis with arreflexia and liquor meningitides may be sudden and affect mostly young and either anatomical lesions or functional impairment of
albuminocytological dissociation, being it a neurological children under 5 years old. It is endemic in Brazil, with the brain. TBI is a common cause of disability and death,
emergency. The differential diagnosis of GBS offers a wide an incidence of about 1.8 cases per 100,000 inhabitants, with a significant impact on Brazil’s public health system
range of possibilities, among them stands out spinal canal with lethality reaching 18-20% of the infected. The clini- and its policies. Objective: To analyze the mortality rate
compression, transverse myelitis, botulism and cerebellar cal features are alarming and the efficiency of protective due to TBI among the brazilian population, from 2011
ataxia¹. Objective: To characterize the epidemiological measures may be monitored through the number of to 2021, through data collected from the Informatics
cases profile of notified acute flaccid paralysis between in Hospitalizations in the national territory, which is usually, Department of the Unified Health System (DataSUS).
Brazil 2015 and 2020. Methods: The data were extracted underreported. Objectives: analyze the data available on Methods: A retrospective, longitudinal, observational
from Notifiable Diseases Information System (SINAN) the DataSUS platform about the Hospitalizations by men- and analytic study. Intracranial trauma deaths (ICD-10
and encompassed the period from 2015 to 2020. Results: ingitis in Brazil and provide epidemiological information S06) data were obtained from the Mortality Information
Between 2015 and 2020, 2711 patients with AFP were noti- about this health issue. Methods: A retrospective, longi- System (SIM) and the population data from the brazilian
fied in Brazil, and 2018 had the higher number of notifi- tudinal, observational and analytic study. The number of Institute of Geography and Statistics (IBGE). The number
cations (520/19.18%). The Northeast region showed the meningitis Hospitalizations (CID 10-A36) were obtained of deaths from 2011 to 2021 was stratified by age group,
highest number of cases (1047/38.62%) and the Midwest from the Sistema de Informações sobre Mortalidade (SIM) and the mortality rate (MR) was calculated per 100,000
was the region with the lowest cases of AFP (171/6.3%). and the population data from IBGE. That includes the inhabitants. Age-specific MR trends were determined by
As for age, 122 (4.5%) were under 1 year old, 966 (35.63%) number of Hospitalizations from 2011 to 2021, stratified joinpoint regression. The dependent variable was the nat-
were between 1 and 4 years, 805 were between 5 and 9 by region. The Hospitalisation rate (HR) was calculated ural logarithmic transformation of the MR, and the year of
years and 818 were between 10 and 14 years. No patients per 100.000 inhabitants. The HR trends of each region analysis was the regression variable. The Average Annual
over fifteen years was notified. In relation to gender, the during the study time were determined by segmented lin- Percent Changes (AAPC) were obtained with a confidence
male gender had the highest prevalence with 1545 cases ear regression (joinpoint regression), with the dependent interval of 95%. Results: The AAPC in mortality rate due
(56.99%). The Brown color/race exceeded the other races. variable the natural logarithmic transformation of the HR to TBI registered in Brazil was -0.7% (95%CI: -1.2,-01;
None cases were classified as “Corfirmed Wild Polivirus”. and the year for the regression variable. Thus, the Annual P<0.1), indicating a statistically significant reduction in
Conclusion: It was found that AFP still occurs frequently Percent Changes (APCs) were obtained with a confidence the number of deaths due to intracranial trauma from 2011
in Brazil, especially in areas with lower socioeconomic interval of 95% (CI95%). Results: Hence, HR by meningitis to 2021. Analysis of age-specific trends in MR explains the
status, in male children and in black race people. There in Brazil from 2011 to 2021 was reduced nationwide and apparent controversial results observed in Brazil. From
was no poliovirus cases detected. in all regions, evaluated individually through data strat- 2014 to 2021, the age group 15-19 years revealed a statis-
ification. In Brazil the results of simple linear regression tically significant decline in MR, with an AAPC of -8.9%
point to an annual reduction of 17% (APC = -17.0), with (95%CI: -11.3,-6.4; P<0.1). A different trend was observed
Neuroepidemiologia in the age group 70-79 years, in which a statistically insig-
a p-value <0.1, with a confidence interval (IC) = -19.7 to
-14.3, which shows the assessment was statistically sig- nificant AAPC was recorded in both periods of analysis:
nificative. Concerning the rates by region, the North had an increase of 1.6% (95%CI: -1.0,4.3; P<0.1) from 2011 to
APC = -6.4 until 2019, in which rates began to reduce by 2016, and a reduction of -2.1% (95%CI: -4.4,0.2; P<0.1)
approximately 43% per year (APC = -43) until 2021. The from 2016 onwards. The age group 70-79 years showed
Northeast had APC = -14.8. In the Central-West region, stable rates of intracranial trauma mortality over the
there was a decrease from 2011 to 2021, with an aver- ten years analyzed. The age group of individuals over 80
age APC = -14.5 in the HR. The analysis of the Southeast years old indicated a statistically significant increase in
shows an APC= -20.8 for the same period, the highest the MR due to TBI only from 2011 to 2016, with an AAPC
of all brazilian regions. Finally, in the South, there was of 4.2% (95%CI: 1.0,7.4; P<0.1). Conclusion: Analysis of
a continuous reduction from 2011 to 2021 APC= -13.9. data systems such as DataSUS is relevant in establishing
Conclusion: In summary, the Hospitalizations due to policies to prevent TBI in age groups with higher inci-
meningitis nationwide had a significant reduction. The dence. Identifying its symptoms, as well as correlating
Southeast region had the highest HR reduction with a its signs with imaging exams, is essential for adequate
decrease of 20.8% per year. treatment decisions.

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EPIDEMIOLOGICAL PROFILE OF STROKE IN TEMPORAL TRENDS ANALYSIS OF THE DECREASING ON WAITING TIME TO START
CHILDREN AND ADOLESCENTS IN BRAZIL MORTALITY RATE DUE TO CEREBRAL TREATMENT FOR MALIGNANT BRAIN
BETWEEN 2012 AND 2021 INFARCTION IN BRAZIL BETWEEN 2011-2021 NEOPLASM DURING THE PANDEMIC IN
BRAZIL
Elvis Oliveira Fonseca, Catarina Ester Gomes Gabriel Montenegro Ribeiro da Silva Cavalcante,
Menezes, Paulo Henrique Rodrigues e Rodrigues, Vitória Gabrielle Castilho dos Santos, Miguel Alexandre Saboia Augusto Borges Filho, Rian Vilar
Nelson Felipe Venas de Jesus Cândido da Costa Júnior, Carolina Gabriela Divino Lima, Antonio Valterno Nogueira Pinheiro Filho,
Soares Gioia, Caio Rodrigues Gomes Dias, Lorenzo Lia Camurça Costa, Júlia Silva Pinheiro Firmino,
Universidade Salvador UNIFACS Fernades Alves, Lucio Hakkinen dos Santos Rosa Matheus Maia Gonçalves Bringel Correia, Wirna
Universidade do Estado da Bahia. Salvador BA, Costa, Beatriz Santana Borges Queiroz Belém Moreira
Brazil
UniFTC Salvador. Salvador BA, Brazil Universidade Federal de Goiás. Goiânia GO, Brazil Universidade de Fortaleza. Fortaleza CE, Brazil
Universidade Estadual de Goiás. Goiânia GO, Brazil
catarina.gomes.menezes@gmail.com Unifemes. Goiânia GO, Brazil alexandrenf@edu.unifor.br
UniRV – Goiás – GO – Brazil
Background: Stroke among pediatric patients is a rare Background: The priority of care given to SARS-COV-2
event, but when it occurs it is severe and has the poten- gabriel.montenegro@discente.ufg.br patients during the COVID-19 pandemic has configured
tial to leave sequelae that compromise neuropsychomo- itself as a challenge to the care provided in oncologic treat-
tor development, leading to low schooling, reduced life Background: Cerebral infarct results from ischemic ments. Thus, studies highlight the change in the surgical
expectancy, incapacity to work and burdening the health necrosis caused by an interruption or reduction of the burden as well as in the time pattern of brain tumor treat-
and social security systems. Still, the rarity of this event blood supply of the damaged area. According to the ments. Thus, it is necessary to understand the impact of
can make it difficult to suspect of, delaying diagnosis World Health Organization, 6 million people die annually the pandemic on the waiting time for the treatment of this
and treatment. Objective: To describe the epidemio- from cerebrovascular diseases, corresponding to 11% of disease. Objective: Analyze whether there was a change
logical profile of stroke in children aged 0 to 19 years in all deaths, becoming the second major cause of annual in the waiting time for brain tumor treatment during the
Brazil. Methods: Observational, retrospective, cross-sec- mortality. Objective: To analyse the MR due to Cerebral pandemic of COVID-19. Method: This is an ecological
tional and descriptive study carried out with data from infarction among the brazilian population. Methods: A computational program-based time-series study. The
the Hospital Morbidity System (SIH/SUS – DATASUS) retrospective, longitudinal, observational and analytic Health Information System (TABNET) database was used,
from the years 2012 to 2021 considering the number study. The number of brain infarction deaths (CID-10 relying on health workers to input data accurately, thus
of Hospitalizations due to stroke in Brazil according to 163) where obtained from the Sistema de Informações having the corresponding limitations arising from it. Two
place of residence. The variables analyzed were sex, race, sobre Mortalidade (SIM) and the populational data from spreadsheets were generated to look for diagnosis and the
mortality rate and year of Hospitalization. Data analysis IBGE. That includes the number of deaths between 2011 treatment start date for malignant brain neoplasm in dif-
was performed using the software Microsoft Office Excel® and 2021, stratified by region. The mortality rate (MR) ferent ranges. The first is from January 2018 to December
2016. Results: Based on the data collected, there was was calculated per 100.000 inhabitants. The MR trends 2019, being the pre-COVID period. However, the TABNET
a total of 7031 Hospitalizations due to childhood and of each region during the study time were determined by system generated it until February of 2020 due to some
adolescent stroke in Brazil between 2012 and 2021, with segmented linear regression (joinpoint regression), with treatments only being started in this period. The second
São Paulo being the state with the most Hospitalizations the dependent variable the natural logarithmic transfor- spreadsheet was generated with data from January 2020
(19.69%), followed by Minas Gerais (11%), Bahia (8.6%) mation of the MR and the year for regression variable. The to March 2022 (during COVID). After that, a script written
and Pernambuco (8.5%). Among the states with fewer Annual Percent Changes (APCs) were obtained with a in Python was built to calculate the time from diagnosis
Hospitalizations are Sergipe (0.73%) and Ceará (3.88%). confidence interval of 95% (CI95%). Results: 31857 cases to the start of the treatment, providing weighted average
The epidemiological profile of Hospitalizations nationally of stroke were notified in Brazil between 2011-2021, with and standard deviation of this time for each spread-
was composed, above all, by females (50.23%), brown 2019 and 10-21 being the years with the highest incidence. sheet. Results: In the pre-COVID period, on average,
people (36.90%), without identification (31.67%) and The average annual mortality presents 1,4 events for every there was a 2.56 months interval from the diagnosis to
white people (25.44%). In the temporal analysis of the 100000 people. The progression of MR in the brazilian the start of the treatment with a 4.61-month standard
last decade, the year with the most Hospitalizations was population revealed a rising tendency: APC=4,36%year deviation (SD) and 7140 patients. During the pandemic,
2014 with 731 Hospitalizations and the year with the few- (CI 95%= 3,4-5,3 P<0,05). The Southeast was the only the month interval from the diagnosis to the start of the
est Hospitalizations was 2020 with 637 Hospitalizations. one that presented a rising tendency during the whole treatment was 1.58 with a SD of 2.43 months and a total
The national mortality rate was 8.03%, with the states period (APC 7,6%/year, CI 95% 5,2-10,1 p<0,05), although of 6696 patients. This result of 1 month less wait during
with the highest mortality rate being Sergipe (21.15%), the progression of the deaths could only be identified pandemics is truly surprising due to the tendency of
Ceará (17.39%) and Tocantins (15.38%), while in São between 2014 and 2021 (APC = 9,76%/year, CI 95% 7,8- postponing surgical treatments, necessary in most cases
Paulo the mortality rate was 4.59%; 7.06% in Minas Gerais; 11,8 p<0,001). The South region presented a significant of brain neoplasm. Although our study is not capable of
10.42% in Bahia and 8.51% in Pernambuco. Discussion progression until 2017 (APC 5,4% CI 95% 1,8-9,1 p=0,01), point the cause of this reduction we elected some possi-
and Conclusion: The mortality rate was higher in states while analysing the whole period, the absolute evolution ble reasons: lower demand in the health system due to
with few Hospitalizations, as in the case of Ceará and of deaths doesn’t show a significant difference (p>0,05). fear of covid, lower number of elective surgeries provid-
Sergipe, suggesting disparities in access to health services, Similar to what happened to the North region, with an ing beds for the not-elective ones, and efforts to remove
especially the high-complexity services responsible for increasing tendency only during 2011 and 1019 (APC 7,5%/ immunosuppressed patients, like oncologic ones, as
handling these cases, which when associated with the year CI 95% 1,8-13,5 p=0,017). The trends were stationary quickly as possible from Hospital settings. Conclusion:
limitations of therapeutic approaches for stroke within for Center-West and Northeast during the whole period It’s unclear if the reduction of the time from diagnosis to
this age group points to the need for further studies to without significant difference. Conclusion: Two types treatment has improved survival or life quality of patients.
expand management of stroke in children and adolescents. of epidemiologic patterns in the MR for cerebral infarcts Therefore, further studies are needed to understand this
in Brazil are observed, a stationary and progressive evo- complex situation of neuro-oncology during pandemics.
lution. In Center-West and Northeast regions follow the
Neuroepidemiologia stationary pattern, while the South, Southeast and North
regions follow the progressive and significant profiles. Neuroepidemiologia

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EPIDEMIOLOGICAL PROFILE OF ACUTE GENERAL NEUROLOGY OUTPATIENT CLINIC: EPIDEMIOLOGICAL PROFILE OF NEONATES
FLACID PARALYSIS IN BRAZIL: 2015-2020 EPIDEMIOLOGICAL PROFILE BORN WITH CONGENITAL HYDROCEPHALUS
IN BRAZIL: ANALYSIS OF A DECADE
Eduardo Mariano Carvalho Silva, Jhonata Gabriel Tiago Abreu Tempone, Angela Bernardino, Raísa
Moura Silva, Ana Carolina Nascimento de Sousa, Laisner Fregonezi Pedro Lucas Grangeiro Sá Barreto Lima, David
Arthur Costa Junger, Fábio Pereira da Silva Júnior, Augusto Batista Sá Araújo, Sophia Costa
João Victor Cunha Silva, Lays Saraiva Rodrigues Hospital Municipal de Poços de Caldas-MG. Poços
Vasconcelos, Fernando Degani Vazquez, Maria
Carvalho, Lorena Silva Viana, Luiz Felipe Bezerra de de Caldas MG, Brazil
Suelly Nogueira Pinheiro, Carolina Figueiredo
Sousa, Nínivi Daniely Farias Santos Hospital Municipal. Poços de Caldas MG, Brazil
Santos, Samir Câmara Magalhães, Paulo Ribeiro
tiagoabreu.med@hotmail.com Nóbrega
Universidade Federal do Maranhão. Imperatriz MA,
Brazil Universidade Federal do Ceará. Fortaleza CE, Brazil
Background: The present study is intended to identify
Hospital Infantil Albert Sabin. Fortaleza CE, Brazil
eduardo.neurologista@gmail.com the profile of neurology events in a specific period of time
Hospital Universitário Walter Cantídio. Fortaleza
in a general neurology outpatient clinic of the brazilian
CE, Brazil
Background: Guillain-Barré syndrome (GBS) is an acute public health system. In fact, it is a frequent question
inflammatory polyneuropathy, currently considered the during the training of the neurologist about the prev- padro.pe@hotmail.com
most frequent cause of acute flaccid paralysis (AFP) in alence of the main diseases in the consultations in the
children, after the eradication of poliomyelitis in devel- neurological area. Thus, we seek to trace this reality by Congenital hydrocephalus(CH) is the condition in which
oped countries. It is characterized by acute paralysis with surveying the various diagnoses in neurology in outpa- there’s an abnormal build-up of cerebrospinal fluid in the
areflexia and albuminocytological dissociation in the CSF, tient care, elucidating questions about the field of work cerebral ventricles or the subarachnoid space, resulting
being a neurological emergency. The differential diag- itself. Objective: To identify the profile of care in general in possible impairment of both physical and mental
nosis of GBS offers a wide range of possibilities, among neurological clinics in a given period of time, elucidating development of children born with it. This study aims to
them spinal canal compression, transverse myelitis, bot- the most and/or less commonly identified diseases and, describe the epidemiological profile of newborns with
ulism and cerebellar ataxia. Objective: To characterize consequently, the profile of work in the neurological field congenital hydrocephalus (ICD-10 Q03) in Brazil from
the epidemiological profile of cases notified in Brazil of in the outpatient environment of the unified health system. 2011 to 2020. Data from the Brazil Live Birth Information
acute flaccid paralysis. Methods: The data were extracted Method: The neurological diagnoses were collected in a System (Sistema de Informações sobre Nascidos Vivos/
from the Notifiable Diseases Information System (SINAN) neurology outpatient clinic of the public health system SINASC) were used. This study descriptively evidenced
and encompassed the period from 2015 to 2020. Results: in a Municipal Hospital of the health network of the city the prevalence of congenital hydrocephalus, gender dis-
Between 2015 and 2020, 2711 patients with AFP were noti- of Poços de Caldas-MG, covering the period from April tribution of cases, type of delivery, weight, and asphyxia
fied in Brazil, and 2018 had the higher number of notifi- 2016 to April 2017. In this way, it was possible to trace the scores in the first and fifth minutes of life in the period
cations (520/ 19.18%). The Northeast region showed the profile of disease groups in general neurology care in the from 2011 to 2020. Prevalence analysis also included the
highest number of cases (1047/38.62%) and the Midwest public health system. Results: A total of 1236 persons were assessment of annual percent change in the period. In
was the region with the lowest cases of AFP (171/6.3%). evaluated from April 2016 to April 2017, 1043 male patients, parallel, independent T-tests were used to compare the
As for age, 122 (4.5%) were under 1 year old, 966 (35.63%) corresponding to 84.38% of the patients in this outpatient group of neonates with CH and those born alive without
were between 1 and 4 years, 805 were between 5 and 9 clinic in this period. Such care includes: headache: 215 the condition. In the period from 2011 to 2020, a preva-
years and 818 were between 10 and 14 years. None patient (17.39%), epilepsies: 180 (14.56%), dementia syndromes: lence of 26,52 cases per 100.000 births (7699 births with
over fifteen years was notified. In relation to gender, the 171 (13.83%), cervical-dorsal-lumbar discopathies: 140 CH) was observed, with its peak incidence occurring in
male gender had the highest prevalence with 1545 cases (11.32%), neurovascular diseases: 120 (9.70%), move- the year 2011 with 32,2 per 100.000 births. The Average
(56.99%). The Brown color/race exceeded the other races. ment disorders: 102 (8.25%), peripheral nerve diseases: Annual Percent Change was -3,6%, with a decrease of
None cases were classified as “Corfirmed Wild Polivirus”. 64 (5.17%), vestibular disorders: 48 (3.88%), spinal trauma 18% in prevalence from 2011 to 2020. Approximately
Conclusion: It was found that AFP still occurs frequently spinal cord and cranioencephalic: 41 (3.31%), mood dis- 54% of the newborns with congenital hydrocephalus
in Brazil, especially in regions with lower socioeconomic orders: 35 (2.83%), central nervous system neoplasms: 25 were male and 88,5% were born from cesarean delivery.
status, in male children and in brown people. There were (2.02%), CNS demyelinating diseases: 23 (1.86%), neuro- 74,4% of the neonates with CH presented more than 2500g
no reported cases of poliovirus. muscular diseases: 15 (1.21%), sleep disorders: 13 (1.05%), at birth, while 6,7% of them presented less than 1500g.
neuro-infectious diseases: 12 (0.97%), other diagnoses: Regarding asphyxia scores, only 29% of newborns with
32 (2.58%). Conclusion: An interesting epidemiological CH presented moderate to severe asphyxia (APGAR score
Neuroepidemiologia data can be seen with the majority of male patients in this 0-4) at 1 minute, and 11% at five minutes from birth. In
study. It is possible to link this fact to diseases related to the comparison between the CH group and the neonates
the occupational aspect (cervico-dorso-lumbar discop- without the condition, the first ones presented with higher
athies, spinal cord and cranioencephalic trauma). On the rates of moderate to severe asphyxia at both 1 minute (
other hand, aspects already known in the literature are p < 0,001; 95% CI 25,2-28,2%) and 5 minutes (p < 0,001;
confirmed, such as the high prevalence of headaches and 95% CI 9,5-11,9%), of cesarean delivery (p < 0,001; 95%
epilepsies, now objective ly explained. CI 32,7-35,1%) and of very low weight at birth (p < 0,001;
4,5-6%). It was also observed that the group of newborns
with CH had a higher male-to-female ratio compared to
Neuroepidemiologia the general population (p < 0,001; 95% CI 7,4-17,6%).
Conclusion: This study presented the profile of congenital
hydrocephalus in Brazil over the last ten years, showing a
reduction in the prevalence of the disease, along with the
characteristics of the newborns affected. The comparative
analysis also showed that CH neonates are more likely to
present with indicators of poor prognosis.

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INCIDENCE OF MIGRAINE AND CEPHALIC INCIDENCE OF TRANSIENT ISCHEMIC MORTALITY BY CEREBRAL


PAIN SYNDROMES IN PEDIATRIC PATIENTS ATTACK BEFORE AND AFTER THE PANDEMIC CRYPTOCOCCOSIS IN BRAZIL: A FIVE-YEAR
IN BRAZIL RETROSPECTIVE ANALYSIS
Valdecir Boeno Spenazato Junior, Matheus
Valdecir Boeno Spenazato Júnior, Matheus Fernando Manzolli Ballestero, Aline Rabelo Lucas Soares Radtke, Emanuel de Assis Bertulino
Fernando Manzolli Ballestero, Gabriel Bagarolo Rodrigues, Gabriel Bagarolo Petronilho, Enzo Martins Gomes, Luciano de Albuquerque Mota,
Petronilho, Daniela Alves Gulhote, Ana Monize Lustosa Campos, Carolina Carmona Pinheiro Francisco Matheus Oliveira de Carvalho, Geovanni
Ribeiro Fonseca, Danielly Maximino da Rocha, Machado, Juan Felipe Galvão da Silva, Álvaro Alves Guimarães Bandeira, Rodrigo Mariano Ribeiro,
Arthur Santos da Silva, Maria Fernanda Grael Tinós, de Sá Júnior, Gabriel de Moraes Mangas, André Pedro Helder de Oliveira Junior
Izabella Rocha Lobo, Bruno Barco Fukuda Fonseca Taufner
Universidade Federal do Ceará. Fortaleza CE, Brazil
Universidade do Vale do Sapucai. Pouso Alegre MG, Universidade do Vale do Sapucai. Pouso Alegre MG, Hospital Universitário Walter Cantídio. Fortaleza
Brazil Brazil CE, Brazil
Universidade Federal de São Carlos, São Carlos SP, Universidade Federal de São Carlos, São Carlos SP,
Brazil Brazil lucasradtke20@gmail.com
Centro Universitário Fundação Assis Gurgacz Universidade Federal de Jataí. Jataí GO, Brazil
Pontíficia Universidade Católica de Campinas. Centro Universitário Fundação Assis Gurgacz Cryptococcosis is a fungal disease that manifests as a
Campinas SP, Brazil Universidade de Fortaleza. Fortaleza CE, Brazil chronic meningoencephalitis in its neurological form,
Universidade Tiradentes. Aracajú SE, Brazil Universidade Federal Fluminense. Rio de Janeiro having unispecific symptoms, such as headache, fever,
Centro Universidade Fundação Assis Gurgacz RJ, Brazil cranial nerves paralysis, sensory, motor and memory
Universidade Federal do Tocantins. Palmas TO, Universidade Federal do Paraná. Curitiba PR, Brazil deficits. This disease usually occurs in immunosuppressed
Brazil patients, where Cryptococcus neoformans is the main
juniorspenazato@hotmail.com agent. This research aims to trace the epidemiological
Universidade do Oeste Paulista. Presidente
Prudente SP, Brazil profile of mortality by cerebral cryptococcosis in Brazil,
COVID-19 is an infectious disease caused by SARS-CoV-2. comparing the data from the 5 national regions from 2016
Faculdade Técnico Educacional Souza Marques. Rio
The most important clinical manifestation is the respira- to 2020. A cross-sectional, descriptive, and retrospec-
de Janeiro RJ, Brazil
tory one, although, an increase in neurological features and tive study was conducted using data from the Mortality
Pontifícia Universidade Católica do Paraná. Curitiba
cerebrovascular disorders have been reported as increased Information System (SIM/Datasus) and from the bra-
PR, Brazil
risk of stroke and transient ischemic attacks (TIA). This zilian Institute of Geography and Statistics (IBGE). We
juniorspenazato@hotmail.com study aims to evaluate the change in the incidence of TIA calculated the mortality rate per 10 million inhabitants
regarding to the coronavirus pandemic context expe- in Brazil and in the five regions individually for each year
Migraine is classified as a type of recurrent primary head- rienced in Brazil. This is a cross-sectional, quantitative analyzed. Furthermore, the profile of mortality by age and
ache, characterized by recurrent attacks of pulsating head- epidemiological study, with analysis of data collected by gender was also accessed. 419 deaths were recorded. The
ache, preceded by photophobia, phonophobia, nausea the SUS Department of Informatics (DATASUS). In 2020 national average mortality rate per 10 million inhabitants
and/or vomiting. It is the most common acute headache in and 2021, there was a significant reduction in cases when per year was 4.01, with the North Region presenting the
children. Although its etiology is still uncertain, the family compared to 2018 and 2019, with an average reduction highest average (8.03) and the Northeast Region the lowest
history is positive in most patients. The aim of this study of 15%. The average number of cases in the last two years (2.96). In 2016, Brazil presented 3.59 deaths per 10 million
was to analyze the incidence of migraine and other pain analyzed was close to 18,261 cases. The region with the inhabitants. The North Region had presented the highest
syndromes in pediatric patients, around brazilian regions, highest incidence of cases over the years analyzed was the average with 7.34, and the Northeast Region the lowest
between January 2017 and February 2022. Quantitative Southeast region, representing 30,829 cases, followed by with 1.93. By the end of 2020, Brazil showed a mortality
data was extracted from the Ministry of Health’s database the South region with a total of 20,308 cases. North and rate of 3.87, with a 7.8% growth compared to the begin-
(DATASUS) through the SUS Hospital Information System Midwest were the least incident regions, representing ning of the period. The Northeast region remained with
(SIH/SUS). It was decided to include patients aged from 10 6,392 and 4,492 cases, respectively. Although there is an the lowest mortality (2.09), with an increase of 8.22%, and
to 14 years, in all brazilian federations and both genders association between COVID-19 and an increase in the the Southeast region showed the highest mortality (4.83),
without distinction. The search was made by selecting risk of cerebrovascular events, such as TIA, the number with an increase of 26.42%, which was the second-high-
the options provided in the system: ‘’Migraine and other of cases did not increase in the years 2020 and 2021, est increase recorded. The most expressive increase was
cephalic pain syndromes’, from January 2017 to February which may reflect both underreporting and overload of 40.84%, which occurred in the South Region. There was
2022. Between January 2017 and February 2022, there were the health care system during the pandemic. a higher number of deaths caused by cerebral cryptococ-
2,609 Hospitalized children. In descending order of inci- cosis among men (69,45%) than women. Moreover, the
dence, there was the Southeast region (35.87%), Northeast 30 to 59 age group was the most affected, concentrating
(28.74%), South (22.23%), Central-West (6.89%) and North Neuroepidemiologia the majority (58.47%) of deaths. Conclusion: This study
(6.24%). Among the years analyzed, 2019 stood out as the allowed a better understanding of the mortality by cerebral
year with the highest number of cases and 2020 the year cryptococcosis in Brazil, which showed a growth trend in
with the lowest number of cases. It was found that the the country. A significant interregional discrepancy was
Southeast region has the highest incidence of migraines observed, with the South and Southeast regions show-
and cephalic pain syndromes in childhood. Therefore, ing the highest growth rates. It is questioned that this
there is a clear need for preventive activities in these age disparity may be associated with several factors, such
groups and better preparation of health professionals with as underreporting and endemicity of the pathology, but
regard to a fast management in emergencies. further studies are needed to elucidate the relevance of
these factors. Moreover, it was possible to analyze the age
range and the most affected gender.
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A PANORAMA OF NEUROLOGICAL EPIDEMIOLOGICAL PROFILE OF CHANGES IN STROKE HOSPITALIZATION


EMERGENCIES DURING COVID-19 MENINGOENCEPHALIC TUBERCULOSIS IN EPIDEMIOLOGY DURING PANDEMICS IN
PANDEMIC IN BRAZIL BRAZIL: 2011 – 2021 BRAZIL
Rian Vilar Lima, Alexandre Saboia Augusto Borges Arthur Costa Junger, Ana Carolina Nascimento de Wirna Queiroz Belém Moreira, Rian Vilar Lima, Dara
Filho, Lia Camurça Costa, Júlia Silva Pinheiro Sousa, Fábio Pereira da Silva Júnior, João Victor Maria de Sá Bonfim, Maria Rita Sousa Soares,
Firmino, Antônio Valterno Nogueira Pinheiro Filho, da Cunha Silva, Jhonata Gabriel Moura Silva, Lays Luana Lima Barroso, Maria Clara Apolônio Pinheiro,
Matheus Maia Gonçalves Bringel Correia, Igor Saraiva Rodrigues Carvalho, Lorena da Silva Viana, Sarah Almeida Sales de Oliveira, Afonso Henrique
Castelo Branco Fontenele Costa, Pedro Henrique Luiz Felipe Bezerra de Sousa, Nínivi Daniely Farias Costa de Oliveira, Ana Vitória Costa de Oliveira, Júlia
Araújo Marques, Velko Veras Pereira de Matos Filho, Santos, Eduardo Mariano Carvalho Silva Silva Pinheiro Firmino
Ana Lara Vieira Soares
Universidade Federal do Maranhão. Imperatriz MA, Universidade de Fortaleza. Fortaleza CE, Brazil
Universidade de Fortaleza. Fortaleza CE, Brazil Brazil
rianvilar@edu.unifor.br
alexandrenf@edu.unifor.br jhonata.moura@discente.ufma.br
Background: According to the literature it is possible
Background: Population aging changed the demographic Background: According to the World Health Organization to elencate 3 main alterations caused by pandemic on
profile of the brazilian population, generating an epi- (WHO), approximately 10 million cases of tuberculosis stroke epidemiology: possibility of stroke as a compli-
demiological transition. Hence, there was an increase (TB) are recorded worldwide every year, being it the cation of COVID-19, decreased number of admission
in the incidence of neurological emergencies such as infectious disease that kills the most young people and of stroke patients and difficulties to maintain full range
stroke, meningitis, epilepsy, and headache. COVID-19 adults, surpassing HIV/AIDS, witch is one of the main of stroke treatment services in periods of worsening in
changed those dynamics, making it necessary to under- risk factors for developing extrapulmonary TB forms. In the pandemic scenario. These alterations were seen in
stand the new panorama of neurological emergencies. Brazil, there were approximately 69,000 new cases and most brazilian services, but there are some particular-
Objective: Analyze how the panorama of neurological 4,500 deaths of the disease in 2020¹. Tuberculous men- ities of the stroke epidemiology on pandemics in the
emergencies has changed with COVID-19, in respect to ingitis or meningoencephalic tuberculosis (MT) is a type country that remain in need of investigation.Objective:
Hospitalizations, deaths, and mortality rates. Method: This of extrapulmonary tuberculosis that develops in the ner- This study aims to understand epidemiology of stroke
is an ecological time-series study, which used the Health vous system due to the dissemination of M. tuberculosis Hospitalizations in Brazil before and during pandemics
Information System database (TABNET), made available in the subarachnoid space, being the worst form of the and compare it with the global findings. Methods: This
by the Department of Informatics of the Unified Health disease. Objective: To characterize the epidemiological is an ecological time series study, which used the Health
System (DATASUS). In the SUS Hospital Information profile of Meningocephalic Tuberculosis cases notified in Information System database (TABNET), made available
System (SIH/SUS), Hospitalizations, deaths, and mor- Brazil from 2011 to 2021. Methods: This is a quantitative by the Department of Informatics of the Unified Health
tality rates of the major neurological emergencies in the observational study of the analytical cross-sectional and System (DATASUS). In the SUS Hospital Information
country (stroke, meningitis, epilepsy, and headaches) were retrospective type developed from information obtained System (SIH/SUS), information about Hospitalizations
collected, using secondary data from the public domain, from the records of the Notifiable Diseases Information due to stroke were collected in two periods of time, the
dispensing ethical appreciation. The range was from System (SINAN). Results: Between 2011 and In 2021, first (pre-pandemics) ranging from november 2017 to
02/2020 (beginning of COVID-19 in Brazil) to 04/2022. there were 9,221 confirmed cases of MT in Brazil, the january 2020 and the second (during pandemics), from
Results: For strokes, Hospitalizations peaked 10/2021, peak being in 2018. More cases were recorded in the february 2020 to april 2022. The data were divided by bra-
with 16951 cases and an average (avg) of 14272.85, and a state of São Paulo with 2,544 notifications (27.56%), fol- zilian region and transferred for the software “Microsoft
linear trendline shows a slightly decreasing trend (SDT); lowed by Rio Grande do Sul (12.5%) and Pernambuco Excel” to be quantitatively analyzed. Results: A reduction
for deaths, a peak of 2759 in 07/2021 with an avg of 2157.3, (7.27%). Roraima was the only state that did not regis- of 9463 (3,4%) strokes was noticided when comparing the
SDT; mortality rates peaked with 17.33 in 03/2021, avg of ter any cases in the last 11 years. The most affected age 2 years and 2 months pre-covid period with the pandemic
15.06, and SDT. For meningitis, Hospitalizations peaked group was between 20 and 39 years old (4,023/43.62%), period, confirming the global tendency of less interna-
03/2020, with 556 cases and an avg of 369.67, SDT; for under five years old kids presented an incidence of only tions. Another finding was that the average Hospitalization
deaths, a peak of 62 on 01/2022 with an avg of 32.74, 1.91% with 177 registered cases. About genders, there time decreased from 7,13 days, with a standard deviation
SDT; mortality rates peaked at 12.55 on 01/2022, avg of was a higher frequency in males. 248 expressed other (SD) of 0,51, in pre-covid to 6,83 days, with a SD of 0,43,
8.83, and slightly increasing trend (SIT). For epilepsy, extrapulmonary forms of TB, mainly being the pleural currently. Before pandemics the linear trendline already
Hospitalizations peaked 12/2021, with 5189 cases and an (64/25.8%) and miliary forms (50/2.16%). 4,735 (51.35%) shows a slightly decreasing tendency (R^2=0,27) that
avg of 4199.70, SIT; for deaths, a peak of 153 on 06/2021 of the reported patients had HIV/AIDS co-infection at became even lower (R^2=0,12), but it is still decreasing
with an avg of 110.63, SIT; mortality rates peaked at 3.28 on the time of diagnosis. 36.13% evolved with cure, 9.45% in pandemics. The lowest Hospitalizations time averages
01/2022, avg of 2.63, SIT. For headaches, Hospitalizations left the treatment and 9.22% died. Conclusion: Finally, (6,2 and 6,7 days) happened simultaneously to the peaks
peaked 02/2020, with 1000 cases and an avg of 683.40, SDT. it was observed that the Meningoencephalic tuberculosis of Hospitalizations due to COVID-19 in march and april
For headaches, only Hospitalization data was available. is more common in males, among adults and young peo- of 2020 and 2021. Conclusion: COVID-19 pandemics
Conclusion: Strokes had a SDT in all analyzed factors. For ple, and that the incidence in children under 5 years old altered the epidemiology of Hospitalizations due to stroke
meningitis, mortality rates increased. Epilepsy increased in has decreased, probably in response to good coverage of in Brazil. The ideal post-stroke Hospitalization time is still
all factors that were analyzed. The rate of Hospitalizations BCG vaccination at birth. There was a significant reduc- unclear, but the reasons for this tendency of decreasing
due to headaches slightly decreased. These results show tion in the number of deaths in relation to the previous this time in pandemics can be seen as an effort to remove
that meningitis was deadlier and that epilepsy cases century, but it was not possible to find data in SINAN of the patient’s from Hospital as quickly as possible due to
may require more attention. A hypothesis would be that the frequency of sequelae, which limits the analysis of the risk of contracting COVID-19. Although, it is not clear
patients chose to wait longer to receive proper care due the clinical outcome of the cases. if this tendency, benefits patients, therefore more studies
to the pandemic. More studies are necessary to better are needed to clarify this point.
understand these dynamics.
Neuroepidemiologia
Neuroepidemiologia
Neuroepidemiologia

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TL 1106571 TL 1106581 TL 1106608

LOWER CRANIAL NERVE INVOLVEMENT IN CLINICAL-EPIDEMIOLOGICAL PROFILE NEUROLOGICAL CAUSES OF CHILD


PATIENTS WITH BASILAR INVAGINATION: A OF PATIENTS WITH SPONTANEOUS MORTALITY IN 10 YEARS, HAS SOMETHING
SERIES OF 65 CASES CEREBROSPINAL FLUID FISTULA CHANGED?
ASSOCIATED WITH IDIOPATHIC
Herisson Rodrigues de Oliveira, Camilla Vanessa Manuela Cavalcante Coling Lima, Letícia Pinheiro
Araújo Soares, Tiago César Costa da Silva, Renan INTRACRANIAL HYPERTENSION IN A Pontes, Rian Vilar Lima, Pedro Arnaud Galdino,
Furtado de Almeida Mendes, Maurus Marques de UNIVERSITY HOSPITAL Nathália Pinheiro Rodrigues, Víctor Queirós
Almeida Holanda Calheiros Campelo Maia
Walisson Grangeiro Bringel Silva, Paulo Ribeiro
Universidade Federal da Paraíba. João Pessoa PB, Nóbrega, Pedro Lucas Grangeiro de Sá Barreto Lima, Universidade de Fortaleza. Fortaleza CE, Brazil
Brazil Pedro Braga Neto, Manoel Alves Sobreira Neto, Davi
Universitário Lauro Wanderley da Universidade Farias de Araújo, Rodrigo Becco de Souza, Livio Leite rianvilar@edu.unifor.br
Federal da Paraíba. João Pessoa PB, Brazil Barros, Pedro Helder de Oliveira Júnior
Background: Child mortality (CM) is a relevant indicator
camilla.vanessa46@gmail.com Universidade Federal do Ceará. Fortaleza CE, Brazil that analyzes the living conditions of a country because
Faculdade de Medicina, Universidade Federal do its high values ??are alarming, and the national panorama
Background: Basilar invagination (BI) corresponds to a Ceará, Fortaleza. Fortaleza CE, Brazil is precarious, both from a social and economic point of
bony malformation that flattens the skull base over the walissong@hotmail.com view. In Brazil, defects in the nervous system comprised
cervical spine, displacing the odontoid process through 3,5% of infant deaths in the last ten years. Encephalitis
the foramen magnum and compressing brainstem, cer- and meningitis are the dominant viral infections related
Background: This descriptive and retrospective study
ebellum, and cervical spinal cord structures. Lesions to the central nervous system that affect children and
aims to describe the analysis of medical records of patients
in this region can imply alterations of the last cranial that, when aggravated, currently confer a high risk of
enrolled in a university Hospital diagnosed with cere-
nerves. Objective: Describe the cranial nerve changes mortality. Objective: The project aims to analyze the sit-
brospinal fluid (CSF) fistula resulting from intracranial
in people who have basilar invagination. Methods: This uation of CM resulting from neurological causes in the
hypertension (ICH). Methods: Data from patients treated
is a descriptive, retrospective and quantitative study. last 10 years, observing aspects such as the avoidability of
at Hospital Universitário Walter Cantídio at Ceará state,
The sample was composed of 65 medical records with deaths and using the TABNET tabulator for this analysis,
Brazil were collected. This study descriptively evidenced
diagnosis of IB and information regarding the time of which generates information linked to the Unified Health
the clinical and epidemiological data of individuals with
pre-surgical evaluation seen between 1996 to 2020. Data System (SUS). Methods: This is an ecological time-series
the studied condition. The inclusion criteria used were
were collected with presurgical changes of the last 6 study, which used the Health Information System data-
adult patients with a diagnosis of spontaneous nasal CSF
cranial nerves. Results: In 65 medical records, 35 % of base (TABNET), made available by the Department of
fistula. A positive history of skull surgery or head trauma
cases with reduced sensitivity of the nauseous reflex, 35 Informatics of the Unified Health System (DATASUS). In
was used as an exclusion criterion to define the sponta-
% with rhinolalia, 34 % with decreased soft palate reflex, the SUS Hospital Information System (SIH/SUS), informa-
neous fistula. The present study represents the partial
19 % with soft palate paralysis, 15 % with nasal reflux, tion about infant (children with less than 1 year) deaths
results of the major research, as all the patients with the
07 % with prognathism and 03 % with retrognathism due to nervous system diseases (chapter VI of ICD-10)
disease are yet to be accessed. Results: All these patients
were evidenced. Dysphagia was also evidenced in 32 % was collected. The period considered ranges from janu-
were diagnosed with the support of both magnetic reso-
of the cases and dysarthria in 10 % of them. As for other ary/2010 to december/2020 and the data were divided by
nance imaging (MRI) and computerized tomography and
anatomical changes in the craniofacial region, 7 % had brazilian region. All the information was transferred for the
2 of them were submitted to lumbar puncture to evaluate
hypoglossal nerve damage. Discussion: It was found that software “Microsoft Excel” to be quantitatively analyzed.
the presence of high opening pressure. Only one patient
100% of the population studied presented some type of This work used secondary data from the public domain,
received a proper diagnosis of idiopathic intracranial
Orofacial and Cervical Myofunctional Disorder (OOFM), therefore dispensing ethical appreciation. Results: In the
hypertension from the attending physician. The mean age
showing lesions of the VIII to the XII cranial nerves. The southeast region of Brazil, a significantly higher decrease
at diagnosis was 36,5 (ages of 26, 28, 57 and 35 at the first
decrease in nausea and soft palate reflexes occurred in in the number of CM related to neurological causes was
presentation) and all the patients were female. The main
a significant number of cases, and in the literature it is observed (R^2=0,79) in comparison to other regions of
clinical manifestations of the disease were nasal CSF leak
one of the most present symptoms both before and after the country, although all of them had fewer reported
and headache, present in all four patients. Other signs and
surgery. Nasal reflux impairment, soft palate paralysis, deaths at the end of the the timeframe in comparison to
symptoms observed were paresthesia (1 patient), ear pain
hypotonia, deficit in sensitivity and mobility of the oro- the beginning (average of 35,8 less deaths). 34% of death
(1 patient), dizziness (1 patient), tinnitus (1 patient), and
facial muscles are also commonly described. Rhinolalia causes were considered evitable, with 3% being reducible
epistaxis (1 patient). Half of the patients had recurrent
and dysarthria present, are not evidenced in the litera- by immunization actions and 97% by adequate treatment
meningitis at some point in their disease, while 1 of them
ture. There was a higher number of disorders related to and diagnosis, among these only epilepsy had an increase
progressed with seizures. The four patients were submit-
phonoarticulatory organs (OFA’s), and a prevalence of (50%) in the number of fatalities. Conclusion: This study
ted to surgical procedures, but only one of them evolved
alterations related to dysphagia. Conclusion: The results provides insights regarding neurological causes of child
without relapse. The absence of symptoms was achieved
suggest a relationship between IB and the lower cranial mortality as well as their epidemiological rates. Although
only with the use of acetazolamide in the remaining three
nerves involvement, being the disorders related to the the total deaths had decreased in the chosen timeframe,
patients, with one of them requiring the setup of a ven-
phonoarticulatory organs prevalent. Thus, preoperative the panorama appears to have changed in the later years
triculoperitoneal shunt. The mean time to relapse after
evaluation by a speech therapy professional and evalua- with decreasing importance of meningitis and special
surgical procedure was 2 months. Conclusion: This study
tion of the permanence or not of these alterations in the attention needing to be given to epilepsy.
presented a partial profile of patients with spontaneous
postoperative period is indicated.
nasal CSF fistula in a brazilian teaching Hospital, showing
the demography of individuals with the disease, as well as Neuroepidemiologia
Neuroepidemiologia the difficulty in treating and recognizing this condition,
and a high relapse rate. The apparent success of the use
of pharmacological agents in the treatment opens new
possibilities for use and further research.

Neuroepidemiologia

290
XXX Congresso Brasileiro de Neurologia

TL 1106623 TL 1106624 TL 1104831

MENINGITIS PANORAMA IN 10 YEARS EPIDEMIOLOGICAL PROFILE OF RESEARCH OF THE RELATIONSHIP


MENINGOENCEPHALIC TUBERCULOSIS IN BETWEEN MOZART’S SONATA K. 448 AND
Nathália Pinheiro Rodrigues, Pedro Arnaud Galdino,
Rian Vilar Lima, Víctor Queirós Calheiros Campelo BRAZIL: 2011-2021 THE EXTINCTION OF THE MEMORY OF FEAR
Maia, Letícia Pinheiro Pontes, Manuela Cavalcante TO SOUND
Eduardo Mariano Carvalho Silva, Arthur Costa
Coling Lima Junger, Fábio Pereira da Silva Júnior, João Victor Fernanda Ribeiro Guida, Isadora Loiola Franco,
Universidade de Fortaleza. Fortaleza CE, Brazil da Cunha Silva, Jhonata Gabriel Moura Silva, Lays Andresa Aline Silva Trindade, Ana Claudia Bellini
Saraiva Rodrigues Carvalho, Lorena Silva Viana, Vasconcelos, Clarissa Ferreira Trzesniak, Cesar
rianvilar@edu.unifor.br Luiz Felipe Bezerra de Sousa, Nínivi Daniely Farias Renato Sartori, Rodolfo Souza Faria
Santos, Ana Carolina Nascimento Sousa
Background: Meningitis consists of an inflammation of FMIT. Itajubá MG, Brazil
the meninges, which are the lining and protective layers Universidade Federal do Maranhão. Imperatriz MA, Universidade Estadual de Campinas. Campinas SP,
of the human brain. This condition can cause sudden high Brazil Brazil
fever, severe headache, emesis and stiffness in the back of eduardo.neurologista@gmail.com fguidafmit@gmail.com
the neck. This disease can be caused by viruses, bacteria or
fungi. Currently, it is possible to be immunized against it. Background: According to the World Health Organization Background: Memory is the process of storing and evok-
In Brazil, meningitis is a notifiable disease, which allows (WHO), approximately 10 million cases of tuberculosis ing information learned from previous experiences. The
the accompaniment of this pathology over the years and (TB) are registered every year in the world. Main risk memory process is divided into phases. Extinction is the
how it affects the country’s population. Objective: The factors for the development of extrapulmonary forms. In final phase, in which previously established information is
project aims to analyze the situation regarding deaths Brazil, in 2020, approximately 69,000 new cases and 4,500 suppressed, starting with a new learning process. Music,
related to meningitis in the last 10 years, by observing deaths from the disease were reported. ¹ Tuberculous men- (especially Mozart’s Sonata K. 448) has several effects on
aspects such as possibility of avoiding death and utilizing, ingitis or meningoencephalic tuberculosis (MT) is a type the brain, due to its neuronal plasticity. Although there are
for this analysis, the TABNET tabulator, responsible for of extrapulmonary tuberculosis that develops in the ner- studies showing positive effects of Mozart´s sonatas on
generating information vinculated to SUS. Methods: This vous system, due to the dissemination of M. tuberculosis short-term memory, few investigate its effect on memory
is an ecological time series study, which used the Health in the subarachnoid space, being the most severe form of extinction. Objective: To analyze the exposure to Mozart’s
Information System database (TABNET), made available the disease. Objective: characterizing the epidemiological Sonata K. 448 in the extinction of fear memory to sound
by the Department of Informatics of the Unified Health profile of cases of Meningocephalic Tuberculosis reported in mice. Methods: Twenty C57BL/6J mice were used,
System (DATASUS). In the SUS Hospital Information in Brazil from 2011 to 2021. Methods: this is an analytical, divided into: G1: Mozart (n=5), G2: Ambience (n=8) and
System (SIH/SUS), information about meningitis was cross-sectional, retrospective, quantitative observational G3: Control (n=7). The G1 group was the only one exposed
collected. Were considered the variables “year of the study developed from information obtained from the to music from the intrauterine life onwards, from 9: 00
first symptom”, deaths and evolution. The period of time records of the Notifiable Diseases Information System pm to 7: 00 am, throughout the project. The other groups
ranges from January/2012 to January/2022 and the data (SINAN). Results: Between 2011 and 2021, there were remained exposed only to ambient sound until the end
were divided by brazilian region, state and city. All the 9,221 confirmed cases of MD in Brazil, the peak being in of the experiment. On the 50th to 54th days, the animals
information was transferred for the software “Microsoft 2018. More cases were recorded in the state of São Paulo were submitted to the Habituation of the Arena, which
Excel” to be quantitatively analyzed. This work used sec- with 2,544 notifications (27.56%), followed by Rio Grande aims to control the interference of behaviors related to the
ondary data of public domain, therefore dispensing eth- do Sul (12.5%) and Pernambuco (7.27%). Roraima was novelty of the experimental environment during training.
ical appreciation. Results: Between the years 2010 and the only state to register no cases in the last 11 years. On the 55th day, the Aversive Training was performed
2022, there were 196741 confirmed and reported cases of The most affected age group was from 20 to 39 years old only with the G1 and G2 groups. On the 56th day, the
meningitis in Brazil, with an emphasis on the southeast (4,023/43.62%), children under five had an incidence of Extinction Test started for 13 consecutive days with the
region, with 106063 of those. There have been fluctuations only 1.91%, with 177 registered cases. Regarding gender, three groups. On the 81st day, all groups were submitted
in the regional curves, reaching a peak of 21807 cases there was a higher frequency in males. 248 had other forms to the Recall Test. The tests were videotaped for analysis.
in 2012. Since then, the curve has declined (R^2=0,71). of extrapulmonary TB, mainly the pleural (64/25.8%) and The Extinction Test and the Recording Test were analyzed
Considering only complete years, 2021 had the lowest miliary (50/2.16%) forms. 4,735 (51.35%) of the reported by the repeated measures and one-way ANOVA tests,
number of confirmed and reported cases, which was patients had HIV/AIDS co-infection at the time of diagno- respectively. Values of p?0.05 were considered significant.
6223. As for the Federative Units, from 2010 to 2022, São sis. 36.13% evolved with cure, 9.45% with abandonment Results: From the statistical analysis, it was shown that the
Paulo was the state that had the highest number of con- and 9.22% with death. Conclusion: It was observed that fear extinction process was different between the groups
firmed and reported cases of meningitis, totaling 79747, approximately half of the cases are concentrated in the over time. Regarding the Extinction Test, the Mozart group
representing 40,53% of the total in Brazil in the same states of São Paulo, Rio Grande do Norte and Pernambuco; showed a significantly higher percentage of freezing than
period. Conclusion: This study provides data that reveal Meningoencephalic Tuberculosis is more common in the others, especially on the first day (p?0.05). In addi-
the decline in confirmed and reported cases of meningitis males, among young adults and that the incidence in tion, freezing reduction was faster for this same group.
in Brazil. The highest percentage in the analyzed period children under 5 years of age has decreased, possibly in Finally, in the Recall Test, the freezing behavior in the
was in the Southeast region (53.91%), mainly in São Paulo response to good coverage of BCG vaccination at birth. Mozart group was significantly higher than in the other
(40.53%). Future information must be gathered and ana- There was a very significant reduction in deaths compared groups (p?0.05). Conclusion: Our results suggest that
lyzed to accompany the development of this scenario. to the previous century. mice exposed to music – Mozart’s Sonata K. 448 – had a
positive effect on fear memory, since music accelerated
Neuroepidemiologia the extinction of fear memory to sound.
Neuroepidemiologia

Neurofisiologia Clínica

291
XXX Congresso Brasileiro de Neurologia

TL 1104979 TL 1105335 TL 1106600

DYSAUTONOMIA IN RFC1-RELATED ELECTRODIAGNOSTIC CHARACTERISTICS OF MOTOR UNIT NUMBER ESTIMATION IN


DISORDER: CLINICAL AND SENSORY GANGLIONOPATHIES PATIENTS WITH SPINAL MUSCULAR
NEUROPHYSIOLOGICAL EVALUATION ATROPHY USING THE CMAP SCAN
Nícolas Ruan dos Santos Cavalcante, Antonio Edvan
Camelo Filho, José Pedro Soares Baima, Halisson TECHNIQUE
Gabriel Schmitt, Fabrício Diniz de Lima, Paula
Camila A.A.P. Matos, Alberto R.M. Martinez, Carelis Flamini Arantes, Alberto Andrade de Mello, Carlos Felipe Barbosa Magalhaes, Rodrigo Holanda
González-Salazar, Anamarli Nucci, Wilson Marques Otto Heise Mendonça, Edmar Zanoteli
Jr, Orlando Graziani P. Barsottini, José Luiz Pedroso,
Department of Neurology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São
Marcondes Cavalcante França Jr
Faculdade de Medicina da Universidade de São Paulo. São Paulo SP, Brazil
Universidade Estadual de Campinas. Campinas SP, Paulo
Brazil holandamendonca28@gmail.com
nicolas.cavalcante@hc.fm.usp.br
Universidade Federal de São Paulo. São Paulo SP,
Brazil Background: 5q-Spinal Muscular Atrophy (SMA) is one
Background: the term sensory ganglionopathy refers to of the most prevalent neuromuscular diseases in our
Universidade de São Paulo. Ribeirão Preto SP, Brazil
the pathological process in which there is involvement of country, and still an important cause of lethality, due to
gabrielschmittt@gmail.com the sensory component, both central and peripheral. In genetic disease, in its most severe forms. From a genetic
most cases patients will have preserved strength despite point of view, it is already known that the number of
Objectives: to determine the frequency and pattern of sensory involvement. To recognize this pathology, asso- copies of the SMN2 gene drastically influences the phe-
autonomic complaints in patients with RFC1-related dis- ciated with malignancies and other potentially treatable notype in an inverse relationship with the severity of the
order. An additional goal was to characterize and quantify diseases, allows the patient to undergo specific treatments. disease. Several studies show the reduction of motor
the involvement of cardiac and sudomotor branches of The differentiation from polyneuropathies is important unit counts by different techniques. A new Motor Unit
the autonomic nervous system. Methods: We enrolled 16 and nerve conduction studies are particularly useful, since Number Estimation (MUNE) technique described in
subjects with biallelic RFC1 (AAGGG)n expansions and the distribution of ganglionopathy is not length-dependent 2016 by Bostock, MScanfit (CMAP Scan MUNE), uses
16 age and sex-matched healthy controls that underwent and often asymmetrical. Goals: describe the electrodiag- a mathematical model that considers the stimulus-re-
comprehensive clinical and neurophysiological evalua- nostic (EDX) findings on a series of patients cases with sponse curve of the compound muscle action potential
tion. SCOPA-AUT score was used to assess autonomic ganglionopathy, obtained in the NCS/EMG laboratory. (CMAP) to estimate the number of motor units. Studies
symptoms. Electrophysiological testing included assess- Methods: retrospective study based on EDX exams from have shown that this technique has greater sensitivity
ment of heart rate variability and quantitative sudomotor the Hospital das Clínicas of the University of São Paulo than other conventional techniques (MUNIX and MPS),
axon reflex test (QSART). Between-group comparisons in the last 8 years. Electrophysiological criteria similar in addition to not requiring voluntary activation, facilitat-
were assessed using non-parametric tests. Results: In to those used by Camdessanche were applied. Sensory ing its use in children or patients with marked weakness.
the patient group, there were 9 men/7 women and the changes were considered length-dependent if the sural/ Objectives: The present study aims to evaluate the use-
median age was 60.5 years. SCOPA-AUT scores were sig- radial ratio was below 20% bilaterally. Sensory asymmetry fulness of Mscanfit in patients with SMA at the Hospital
nificantly higher in the RFC1 group compared to controls. was considered when we observed a side-by-side differ- das Clinicas-FMUSP neuromuscular diseases outpatient
Major differences were noticed for gastrointestinal, uri- ence above 50% of the sensory potential amplitude. The clinic compared to other techniques (MUNIX and CMAP
nary and cardiovascular subscores. In neurophysiology, following data were evaluated: sex, age, etiology, presence amplitude). Methods: Forty-seven patients with SMA
there was resting tachycardia combined with abnormal of ataxia and characteristics obtained from EDX: sensory were evaluated, CMAP scan values were obtained with
responses during Valsalva maneuver and deep breathing and motor conduction amplitudes and velocities, late surface electrodes on the abductor pollicis brevis (APB)
among patients. QSART responses were also significantly responses and assessment of the blink reflex. Results: 27 and abductor digiti minimi (ADM) muscles. MUNIX values
reduced in the RFC1 group, especially in the lower limbs. patients were identified between November 2014 and April were obtained in the same muscles, for comparison, of 40
Conclusion: Autonomic dysfunction is frequent, clinically 2022. There was a predominance of females 77% (20). The collaborative patients for the technique. In 8 patients, the
relevant and involves multiple domains in RFC1-related age of the patients ranged from 11 to 86 years (mean: 53.5). same exams were performed with one year of follow-up.
disorder. Patients have both sympathetic and parasym- About 88% of the cases (26) met Candessanche criteria for Results: Seven patients were SMA type 1, 25 patients were
pathetic involvement. From a topographical perspective, ganglionopathy. We observed asymmetry in the sensory SMA type 2 and 15 were SMA type 3. Mean CMAP scan
this condition is characterized by a small fiber (possibly action potentials in 53% (14) of the cases. Six cases (23%) MUNE values correlated inversely with disease severity,
length-dependent) autonomic axonopathy. had a length dependent distribution according to sural/ with patients with SMA type 1 having lower values while
radial ratio. There was a predominance of Sjögren’s syn- those with patients with type 3 SMA have higher MUNE
drome in 34% (9) and undetermined etiology in 34% (9), values. Among the patients who were controlled at 1 year
Neurofisiologia Clínica neoplasms in 27% (7), celiac disease in 7% (2), in addition of follow-up, only 1 patient was SMA type 1, being treated
to isolated cases of other etiologies. The H reflex was not with nusinersen, and the MUNE values obtained by the
obtained in 88% (23) of the cases. The blink reflex was CMAP scan were the same after 1 year of follow-up. The
altered in 75% of the cases, but the test was done in only remaining seven patients who underwent the technique
8 cases. Conclusion: Our cases were compatible with were SMA type 2 or 3 and the values did not differ signifi-
Candessanche criteria. It was also possible to observe cantly between the two exams in relation to the use or not
non-length dependence, predominance of this pathol- of disease-modifying therapy. Conclusion: CMAP Scan
ogy in females and motor involvement in paraneoplastic can be used to count motor units in patients with spinal
etiologies. An important finding is that sural/radial ratio muscular atrophy. More ongoing studies should assess
may not differentiate sensory ganglionopathy from poly- its usefulness as a biomarker of disease progression and
neuropathy in isolation. treatment response parameter.

Neurofisiologia Clínica Neurofisiologia Clínica

292
XXX Congresso Brasileiro de Neurologia

TL 1105167 TL 1105186 TL 1105288

NON 5Q SMA: A BRAZILIAN COHORT STUDY CLINICAL VARIABILITY IN A LARGE LONG-TERM CARDIAC MAGNETIC
GERSTMANN-STRÄUSSLER-SCHEINKER RESONANCE IMAGING STUDY IN
Rodrigo Siqueira Soares Frezatti, Pedro José
Tomaselli, Mary M Reilly, Wilson Marques Junior (P.P102L) KINDRED FROM BRAZILIAN FRIEDREICH’S ATAXIA
NORTHEAST
Hospital das Clínicas de Ribeirão Preto. Ribeirão Thiago JR Rezende, Alberto RM Martinez, Mariana
Preto SP, Brazil Marcos Vinícius Oliveira Marques,, André Macedo R de Brito, Luis M Silva, Lucas MT Branco, Camila
Department of Neuromuscular Diseases, UCL Serafim da Silva,, Cristiane de Araujo Martins C Lobo, Fernando Cendes, Michael Jerosch-Herold,
Queen Square Institute of Neurology, London, United Moreno,, Edmar Zanoteli, Otávio R Coelho-Filho, Marcondes C França
Kingdon. Universidade Estadual de Campinas. Campinas SP,
Faculdade de Medicina da Universidade de São
frezattirodrigo@hotmail.com Paulo. São Paulo SP, Brazil Brazil
Harvard Medical School – Estados Unidos
mvom13@gmail.com
Spinal muscular atrophy (SMA) describes a group of thiago.jrezende@gmail.com
hereditary motor neuron disorders, characterized by
Gerstmann-Sträussler-Scheinker syndrome (GSS) is a rare
progressive muscle weakness due to the degeneration of Background: Friedreich’s ataxia (FRDA) is an autoso-
autosomal dominant hereditary prion disease caused
the anterior horn cells. This process is a consequence of mal recessively inherited disorder related to abnormally
by a mutation at codon 102 at PRNP protein gene. The
structural alterations of the lower motor neurons, 95% of expanded GAA repeat in intron 1 of FXN gene (9q21.11),
disease has high penetrance, and symptoms may vary
the time caused by loss-of-function SMN1 mutations on and characterized by cerebellar ataxia, spasticity and are-
among affected individuals. The most common pre-
chromosome 5. Recently, with the advance of next gen- flexia of lower limbs and loss of position and vibration
sentations are ataxia, dementia, spasticity, nystagmus,
eration sequencing, several potentially causative genes sense. The disease is also related to cardiomyopathy in
and behavioral abnormalities. This study describes the
outside the chromosome 5 have been recognized. This about two thirds of individuals, and heart failure is the
phenotypical variability within kindred from Bahia state
subgroup of wide phenotypic variability and inheritance main cause of death in these patients. There is a lack of
(brazilian northeast). We used neurologic examination
pattern, in which proximal muscle weakness predomi- biomarkers for clinical care and assessment in clini-
to map clinical symptoms and Addenbrooke’s Cognitive
nates, is known as non-5q spinal muscular atrophy. We cal trials, and previous data might suggest that cardiac
Examination-Revised to assess cognition. We used clinical
sought to describe the clinical and neurogenetic spectrum magnetic resonance imaging (cMRI) might be sensitive
history and genetic panels to map 26 affected individuals,
of non 5q proximal SMA patients in a brazilian cohort for early damage and clinical follow-up. Objective: The
7 of whom are still alive. In our cohort, we have individuals
of patients. Cases from a neuromuscular clinic were present study aimed to assess the usefulness of cMRI as
in different stages of the disease, ranging from pre-symp-
reviewed. Patients with proximal neurogenic weakness a clinical marker in FRDA. Methods: Eighteen healthy
tomatic to bedridden. We also identified 28 individuals at
(SMN1 testing negative) or those with clinical evaluation controls and 42 FRDA patients were included. cMRI was
risk of having the mutation who declined genetic testing
suggestive of motor neuronopathy were included. So far performed in a 3T Phillips scanner. Among patients, 17 also
or could not be tested yet. The age of onset was between
95 patients from 48 families were identified, 45 (48%) of underwent a 5 year-interval repeat cMRI. The following
the 3rd and 5th decade of life. Some individuals might
them, male. Eleven of the patients (12%) started symptoms imaging data were assessed: left ventricle ejection fraction
have started even earlier if we considered their history
at birth, 50 patients (53%) had from 1 to 30 years and 30 (LVEF), mass and volumes, late gadolinium enhancement
of neuropsychiatric symptoms. Gait abnormalities, broad
(32%) had more than 30 years when symptoms started. The (LGE), extracellular volume fraction (ECV) and intracellu-
base, and unsteadiness were the first signal observed in
inheritance pattern was autosomal dominant in 51 (53%), lar water-lifetime (?ic). The FRDA rating scale (FARS) was
all the patients evaluated. During disease progression,
recessive in 9 (10%), X-linked in one (1%), mitochondrial determined to quantify neurological decline. An ANCOVA
ataxia and spasticity become prominent. The Babinski
in 2 (2%) and sporadic in 32 (33%). Molecular analysis is test, using age, sex and follow-up time as covariates, was
sign is present very early in those patients. On the other
ongoing and, so far, 52 (55%) have a definitive diagnosis. performed between groups, and also a Pearson correlation
hand, cognitive symptoms occurred much later in disease
The phenotype is complex in 71 patients (75%) and many coefficient to determine relationship between clinical and
evolution. All the deceased individuals presented ataxia
associated features have been described: disautonomia, imaging data. Results: The FRDA group had increased LV
at some moment in their disease progression, along with
piramidalism, ophtalmoplegia, scoliosis, artrogriposis, volumes and thickness, ECV and ?i, with preserved LVEF,
spasticity and loss of ambulation. Neurosensorial deafness
cognitive impairment, sensory neuropathy, ataxia, among when compared to controls in cross-sectional analyses.
was observed in 2 out of 7 alive individuals and reported
others. Non-5q SMA is genetically diverse, and many Nine patients (21,9%) had epicardial LGE. FARS scores
in five other deceased individuals, contrasting with other
genes will be novel. Our cohort will be fully genetically did correlate LV volumes. Follow-up analyses evinced
cohorts in which deafness was reported in less than 2%
investigated using next-generation sequencing techniques an increase of the LV diastolic volume and decrease of
of individuals. MRI was normal in most patients; some
with targeted panels and/or whole exome sequencing. So the LV volume/mass ratio, but no significant LV decline.
presented mild cerebellar atrophy later in the disease. The
far, the results highlights this heterogeneity of genes and Also, 5 additional patients presented epicardial LGE.
causes of death were infection, complications related to
associated features. Conclusion: FRDA patients have an expansion of the
neurodegeneration and immobility, and suicide. Since
its first description in 1992, much has been studied in myocardial interstitium and an increase in cardiomyo-
prion protein and its related diseases. Studies of natu- cyte size, with increased LV volumes and thickness, and
Neurogenética
ral history are important to a better understanding of a preserved LVEF. The cMRI might be useful not only for
disease progression. They may play an important role in cardiac assessment but for general clinical evaluation in
knowing other neurodegenerative disorders with similar FRDA since LV volumes correlate with ataxia severity. The
progression patterns and pathophysiological substrates. follow-up analyses indicate a switch from a hypertrophic
Therefore, there is still much to be investigated in this cardiomyopathy phenotype towards a dilated cardiomy-
field. Genetic counseling is very important to reduce the opathy phenotype, suggesting progressive heart damage.
number of affected individuals in the family. Thus, our data suggest that cMRI might be a useful bio-
marker in FRDA.

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TL 1105371 TL 1105545 TL 1105588

GASTROINTESTINAL TRACT RFC1-RELATED DISORDER: IN VIVO MUTATIONAL SCREENING IN BRAZILIAN


MANIFESTATIONS IN PATIENTS WITH EVALUATION OF SPINAL CORD DAMAGE PATIENTS WITH ACUTE INTERMITTENT
A DIAGNOSIS OF FABRY’S DISEASE IN PORPHYRIA DISEASE
Thiago J R Rezende, Gabriel S Schimitt, Fabricio
FOLLOW-UP CARE BY A NEUROLOGY TEAM D de Lima, Paula Camila A A P Matos, Lucas M T Bianca Mara Andrade, Ivan Augusto Lorena, Pedro
IN CUIABA Branco, Alberto R M Martinez, José Luiz Pedroso, Jose Tomaselli, Rodrigo Soares Frezatti, Wilson
Orlando G P Barsottini, Wilson Marques, Marcondes Marques Junior
Klesia Adaynny Rodrigues, Victor Hugo Souza Silva
C França
Gomes, Heloise Helena Siqueira, Bianca Barbosa Faculdade de Medicina de Ribeirão Preto. Ribeirão
Araldi, Isadora Constantini Soares Andrade, Beatriz Universidade Estadual de Campinas. Campinas SP, Preto SP, Brazil
Fulador, Lucas Cruz Farias, Andressa Gabrielle da Brazil
Silva Universidade Federal de São Paulo. São Paulo SP, bianca.mara@usp.br
Brazil
Hospital Geral Universitário. Cuiabá MT, Brazil Background: Acute Intermittent Porphyria (AIP) is a rare,
Universidade de São Paulo. Ribeirão Preto SP, Brazil
klesia.adaynny@gmail.com genetically determined neurometabolic disorder with an
thiago.jrezende@gmail.com autosomal dominant inheritance pattern and reduced
Background: Fabry disease (FD) is an inborn metabolic penetrance. Pathogenic variants in the HMBS gene lead
Background: RFC1-related disorder is a recently described to a partial deficiency of the enzyme hydroxymethylbi-
error linked to the X-chromosome, affecting the GLA
neurodegenerative condition related to biallelic intronic lane synthase, also called porphobilinogen deaminase
gene, characterized by a lysosomal storage disorder of
expansions in RFC1, leading to CANVAS syndrome. This (PBDG), the third enzyme in the heme group biosyn-
the globotriaosylceramide (Gb3). Its pathophysiology is
condition is typically characterized by a clinical triad of thetic pathway. Deficiency in HMBS production leads
based on the absence or dysfunction of alpha-galactosi-
cerebellar ataxia, sensory neuronopathy and vestibular to the accumulation of heme precursors, wich are toxic
dase, with protein accumulation in a variety of cells in the
areflexia, but also with a broad phenotypic spectrum, to cells. During puberty, the disease manifests itself and
body, with cytotoxic, pro-inflammatory and pro-fibrotic
including dopa-responsive parkinsonism, spasmodic is characterized by intermittent neurovisceral attacks,
effects. Likewise, the gastrointestinal tract (GIT) is affected,
cough and dysautonomia. Cerebellar and encephalic MRI including severe abdominal pain, peripheral neuropathy,
with deposit primarily in the autonomic intestinal ganglia,
changes have been described in this disease, but little is and psychiatric manifestations. The combined prevalence
generating intestinal dysmotility, and associated mani-
known regarding spinal cord involvement. Objectives: of acute porphyrias is 5 cases per 100000. The brazilian
festations. It is considered a rare disease, predominantly
The present study aims to determine spinal cord alter- epidemiology is unknow. In a study carried on 2002 it was
diagnosed in men, affecting one in every 117,000 live
ations in RFC1-related disorder and its clinical correlates. found seven mutations and in a 2015 study it was found
births. Objective: The aim is to describe specific manifes-
Methods: Seventeen subjects with biallelic RFC1 (AAGGG) nine variants. Objectives: To evaluate the prevalence of
tations of the GIT, such as abdominal pain, nausea, and
n expansions and 11 age and sex-matched healthy con- genetic variants in the HMBS gene in a brazilian cohort
constipation, in patients with confirmed FD followed up
trols were enrolled between 2019 and 2021. All individ- of patients admitted to a tertiary Hospital presenting with
at a neurology outpatient clinic. Methods: A systematic
uals underwent multimodal MRI spinal cord acquisi- an acute neuropathy associated with abdominal pain,
literature review was conducted based on the PUBMED
tions in a 3T Philips Achieva scanner and patients were psychiatric manifestations, or laboratory abnormali-
and UPTODATE databases using keywords as “Fabry dis-
evaluated including SARA scores and time from ataxia ties suggestive of AIP. Methods: Forty-nine consecutive
ease”, “Gastrointestinal manifestations” and “Neurological
onset (disease duration) to quantify disease severity. The probands were tested, of which thirty-four were women.
Disorders” to compare data acquired from standardized
Spinal Cord Toolbox (SCT) was used to assess spinal cord Sanger sequencing from all HMBS exons was performed.
GIT symptoms questionnaires that were regularly applied.
cross-sectional area (CSA), eccentricity (ECC), spinal cord Variants detected were classified according to ACMG cri-
Results: Six patients diagnosed with FD were interviewed
gray matter area and diffusivity parameters of fasciculus teria Results: A pathogenic/likely pathogenic variant was
of whom 2 were men and 4 women, aged between 37 and
cuneatus, fasciculus gracilis and lateral corticospinal tract found in twelve probands. Segregation analysis revealed
66 with a mean age of 49 years, presenting a mean time of
across C2-C5 levels. Between-group comparisons were additional seven carriers,of wich three had clinical mani-
diagnosis of 7.6 years; of these 5 (74 %) were on enzyme
assessed using Mann-Whitney U test, employing age and festation. Eight variants found according to (NM_000190.4/
replacement therapy (ERT) with alpha-galactosidase. It
gender as covariate. A Bonferroni test correction was ENST00000652429.1) were: three missense alterations
was observed that 100% of the patients had some gastro-
used to control for multiple comparisons. Correlations (c.346C>T probably pathogenic; c.517C>T pathogenic;
intestinal manifestation in their lives, dyspepsia being
between MRI findings and clinical data were assessed c.77G>A probably pathogenic), two nonsense alterations
the main symptom, which remains a lifelong symptom
through Spearman correlation test. Results: RFC1 group (c.973C>T pathogenic; c.912_913insT pathogenic); two
in 34% of them with a frequency that varies from weekly
had significant spinal cord CSA reduction along all cervi- splice site alterations (c.652-1G>A pathogenic; 499-1G>A
to daily, appearing after main meals. In addition, 16% of
cal levels, increased ECC in lower cervical levels and gray pathogenic) and one frameshift variant (c.854_855insC
the interviewed who had not yet started ERT reported
matter atrophy when compared to controls. Regarding pathogenic). The remaining thirty-seven probands had
nausea and vomiting, as well as abdominal cramps since
white matter alterations, diffusivity abnormalities were no variants, the remaining for genes are being tested for
childhood, lasting approximately a week, with a recurrence
found only in the dorsal columns. There was no significant this cohort. Conclusion: In this study, the molecular
of every two months. Along the years, this slowly evolved
correlation between clinical data and imaging parameters. diagnosis was possible in 24.5% of probands tested with
to daily cramps associated with constipation at the age of
Conclusion: RFC1-related disorder patients have promi- a clinical diagnosis of AIP. We found the most frequent
39, with a 4-day stool interval. Conclusion: In sum, it has
nent gray matter atrophy and white matter degeneration abnormality (c.973C>T), a nonsense pathogenic variant
been observed that the use of ERT is important to prevent
in cervical spine, mostly on dorsal columns, showing that observed in five unrelated individuals. We are now using
progression of various manifestations, in particular GIT
spinal cord damage is a hallmark of this disease. More a next-generation sequencing methodogy to search for
disorders, caused by FD that threaten patients’ lives and
studies are needed to assess the usefulness of these find- the remaining genes associated with acute porphyria
well-being. In addition, only patients who had not been
ings as clinical biomarkers.
on ERT progressed to more severe symptoms compared
to those that were on it.
Neurogenética
Neurogenética
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XXX Congresso Brasileiro de Neurologia

TL 1105595 TL 1105695 TL 1105714

CARCINOGENIC EVALUATION OF DISTAL HEREDITARY MOTOR BIALLELIC PATHOGENIC VARIANTS IN


LISDEXAMFETAMINE DIMESYLATE NEUROPATHY: CLINICAL AND MOLECULAR VACCINIA RELATED KINASE-1 GENE IS A
THROUGH BIOASSAY WITH DROSOPHILA CHARACTERIZATION OF A BRAZILIAN CAUSE OF MOTOR NEURONOPATHY WITH
MELANOGASTER POPULATION UPPER MOTOR NEURON SIGNS
André Teixeira Souza Castro, Caíque Martins Mortati Pedro José Tomaselli, Rodrigo Siqueira Frezatti, Pedro José Tomaselli, Diogo F dos Santos, Rodrigo S
Silva, Priscila Capelari Orsolin Fernanda Figueiredo Barbosa, Silmara Gouvea, Frezatti, Alexander M Rossor, Matilde Laura, Julia C
André Cleriston Jose dos Santos, Carolina Lavigne Blake, Jasper Morrow, Henry Houlden, Mary M Reilly,
Centro Universitário de Patos de Minas – Patos de Moreira, Henry Houlden, Stephen Zuchner, Mary M Wilson Marques Jr
Minas – MG – Brazil Reilly, Wilson Marques Jr
Hospital das Clínicas da Faculdade de Medicina de
andretsscastro@gmail.com Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto. Ribeirão Preto SP, Brazil
Ribeirão Preto. Ribeirão Preto SP, Brazil Universidade Federal de Uberlândia. Uberlândia
Lisdexamfetamine Dimesylate is a psychotropic drug used Faculdade de Medicina de Ribeirão Preto. Ribeirão MG, Brazil
as first-line therapy for Attention Deficit Hyperactivity Preto SP, Brazil NINN, Queen Square. United Kingdon
Disorder. The lack of bio-safety studies on this compound HCFMRP. Ribeirão Preto SP, Brazil Faculdade de Medicina de Ribeirão Preto. Ribeirão
demonstrates the need to verify long-term adverse effects. NINN, Queen Square. United Kingdon Preto SP, Brazil
This study aimed to evaluate the carcinogenic effect of University of Miami, United States
this amphetamine through the Epithelial Tumor Test pjtomas@alumni.usp.br
(ETT) in Drosophila melanogaster. This test uses the pjtomas@alumni.usp.br
interpretation of malignant phenotypes in the body seg- Background: Recessive mutations in VRK1 (human vac-
ments of Drosophila, being used to study the mechanisms Background: The distal hereditary motor neuropathy cinia-related kinase 1) gene have been reported in a small
involved in tumor formation and the assessment of drug (dHMN) is a rare form of hereditary neuropathy present- number of patients with spinal muscular atrophy with
biosafety. ETT is used to assess the carcinogenic and ing with a length-dependent motor / predominant motor pontocerebellar hypoplasia (SMA-PCH). More recently,
anticarcinogenic effects of various chemical compounds neuropathy. Additional features as upper motor neuron a pure axonal neuropathy has been reported in associa-
since about 70% of cancer-causing genes have homologs signs (UMN), respiratory distress, vocal cord involvement tion with VRK1 mutations. Objective: To describe clini-
in Drosophilas, including the wts gene homologous to may be seen, and together with the inheritance pattern cal, neurophysiological and image findings in cohort of
human LATS1. In the present study, D. melanogaster are routinely used for clinical classification. Objectives: patients with VRK1-related disorder. Methods: Clinical,
larvae from the crossing of wts/TM3 and mwh/mwh To describe the clinical and neurogenetic spectrum of 127 MRI and electrophysiological data were collected in pre-
mutant strains were treated with different concentrations dHMN proband / familie’s diagnosed in reference centre defined preform from patients with with genetic diagno-
of Lisdexamfetamine Dimesylate (0.125; 0,25; 0,5; 1.0 and for neuromuscular disorders. Methods: 148 patients from sis. Results: Six affected patients from 5 different families
2.0 mM). Doxorubicin was used as a positive control, 127 families were included (85 males; mean age 33.7y, were included. Four were female, and there was history of
and for the negative control, ultrapure water was used. range 2-78y). Cases were genetically investigated using consanguinity in just one family. All cases presented in the
The results show a significant increase (p<0.05) in tumor next-generation sequencing technics – targeted panel first decade of live with difficulty walking or frequent falls,
frequencies in individuals treated with concentrations and / or WES. WES was performed in 25 probands. Target and no patient had sensory abnormalities. One patient
of 0,5; 1.0 and 2.0 mM of Lisdexamfetamine Dimesylate panel with 27 genes (AAAS, AARS, BICD2, BSCL2, DCTN1, presented with fasciculation, and all patients presented
when compared to the negative control suggesting a DYNC1H1, DYNC1H1, FBLN5, FBXO38, GARS, GBE1, with brisk reflexes. Brain and spinal MRI was normal in
dose-dependent carcinogenic effect in D. melanogaster. HINT1, HSPB1, HSPB3, HSPB8, IGHMBP2, KIF1A, LAS1L, all cases. Involvement of the posterior compartment of
However, this assay contrasts with some previous studies MYH14, PLEKHG5, REEP1, SETX, SLC52A1, SLC52A2, the leg was greater then the anterior compartment in four
for carcinogenic evaluation of the active ingredient d-am- SLC52A3, TRPV4, VRK1) Sanger sequencing was per- cases detected by clinical examination and confirmed by
phetamine. Carcinogenicity studies indicated negative formed for segregation analysis. Results: The inheritance lower limb MRI. One case has a homozygous missense
results upon continuous administration of this compound. pattern observed was autosomal recessive in 20, dominant variant and other cases were compound heterozygous
Nonetheless, the authors cited various capacities of the in 18 and sporadic in the remaining cases. Ten cases had carring one missense variant in association with a loss
active principle in indirectly inducing the formation of also UMN signs, 19 had some proximal involvement, 6 had of function mutation. When available, analysis of paren-
neoplasms. This is due to the substance’s adverse effects, upper limb predominance and 1 had vocal cord involve- tal DNA showed the mutations were on separate alleles.
such as increased body temperature and anorexia, causing ment. A class 4 or 5 variant was identified in 26% and a VUS Discussion: This study expands the clinical spectrum of
loss of adipose tissue, increasing the incidence of ade- in 20% of proband / families. BSCL2 and DNAJB2 were VRK1-related disorders, and suggests that mutations in
nomas in the testicles, and myeloproliferative diseases. the most frequent cause dHMN in our cohort, another VRK1 should be considered in patients with motor neu-
Therefore, it can be concluded that Lisdexamfetamine 13 genes have one or two proband. Conclusion: Distal ropathy. We propose that missense mutations in VRK1
Dimesylate had a carcinogenic effect in vivo analysis hereditary motor neuropathies encompass a rare neu- manifest a milder phenotype compared to nonsense
model with D. melanogaster, but further investigations ropathy group. Several causative genes have been iden- mutation characterized by a combination of upper and
involving other biological models in vivo and in vitro are tified in the brazilian cohort, but only a few were found in lower motor neuron signs mostly confined to the lower
needed to verify drug safety in humans. more than one proband/family. The spectrum of dHMN limbs, with normal brain development.
genes differs considerably between populations. We did
not found any predominant causative gene. Additional
Neurogenética studies are needed to better determine the genetic pro- Neurogenética
file of our population. It is expected that additional testes
for the cases underwent the panel analysis may increase
our positivity rate and may give a more accurate genetic
picture from our cases.

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TL 1105797 TL 1105837 TL 1106372

HYPOTHALAMIC STRUCTURAL ALTERATIONS CLINICAL AND ELECTROPHYSIOLOGICAL GENETIC COUNSELING AND


IN HUNTINGTON’S DISEASE PATIENTS FEATURES OF A BRAZILIAN COHORT OF EMPOWERMENT IN WOMEN WITH
CHARCOT-MARIE-TOOTH TYPE 4C (CMT4C) PATHOGENIC VARIANTS ASSOCIATED WITH
Yves Glauber Silva Santos, Marcondes Cavalcante
França Jr, Thiago Junqueira Ribeiro Rezende DUCHENNE AND BECKER MUSCULAR
Ellen Silva Carvalho, Maria Clara Baseio, Renan
Barros Lopes, Fernanda B Figueiredo, Marcus DYSTROPHY
Hospital de Clínicas da UNICAMP. Campinas SP,
Vinícius Gomes, Rodrigo S Frezatti, Pedro José Alice Maria Luderitz Hoefel, Bruna Faria Rosa, Cesar
Brazil
Tomaselli, Mary M Reilly, Stephan Zuchner, Wilson Augusto Weschenfelder, Jonas Alex Morales Saute
Hospital de Clínicas UNICAMP. Campinas SP, Brazil
Marques Junior
Hospital de Clíncas da UNICAMP. Campinas SP,
Hospital de Clínicas de Porto Alegre
Brazil Hospital das Clínicas da Faculdade de Medicina de
Ribeirão Preto. Ribeirão Preto SP, Brazil caweschenfelder@hcpa.edu.br
yves.glauber2@gmail.com
National Hospital for Neurology and Neurosurgery.
United Kingdon Background: In recent years, with the emergence of
Background: Huntington’s Disease (HD) is a genetically
University of Miami, United States next-generation sequencing and advanced therapies
determined neurodegenerative disorder, with dominant
for Duchenne muscular dystrophy (DMD), access to
autossomic transmission, provoked by unstable trinucleo- e_len12@hotmail.com genetic testing for diagnostic confirmation and detec-
tide repeat expansion in the huntingtine gene. In addition
tion of mutations amenable to specific therapies has
to the classic features of involuntary movements, cogni- Background: Charcot-Marie-Tooth (CMT) is a clinical and been expanded in Brazil. Such programs favor genetic
tive decline and neuropsychiatric disorders, non motor genetically heterogeneous group of inherited neuropa- testing of boys with DMD and Becker muscular dystro-
symptons are frequently reported. Hypothalamic involve- thies with an estimated prevalence of 1 in 2500 individ- phy (BMD) suspicion, with little attention being given
ment might explain these findings. Objective: Assess uals. CMT4 is a group of demyelinating neuropathy with to women testing. Objectives: to evaluate the process of
strutural hypothalamic involvement in Huntignton’s autosomal recessive inheritance. To date, there are 11 genetic counseling and empowerment of genetic infor-
Disease patients. Methods: Magnetic ressonance with causative genes. CMT4C is caused by pathogenic variants mation for women at risk of carrying variants related to
volumetric acquisition was performed in 35 HD patients in SH3TC2. It usually presents in the first decade of life DMD/BMD in Brazil. Methods: a cross-sectional study
and 35 healthy controls paired by age and sex. The hypo- with distal muscle weakness, pes cavus, difficulty walking was carried-out, in which an online questionnaire was
thalamus was segmented utilizing a Deep Learning based and slow nerve conduction velocities, additional features applied through Google Forms with the application of the
network. Volume and texture was compared between include scoliosis. Objective: We aim to describe the clin- Genomic Outcome Scale (GOS) that evaluates empower-
the two groups. Results: There was no significant differ- ical and electrophysiological features of a case series of ment of genetic information. The main inclusion criteria
ence in hypothalamic volume between HD patients and CMT4C from a tertiary Hospital. Methods: Clinical and were being female and being at risk of carrying or being
healthy controls. However, there was significant differ- electrophysiological data were retrospectively collected known to carry variants that cause DMD/BMD. Results:
ence between texture parameters between the two groups from clinical records. Seven patients from five different Ninety-five participants were included, with a mean age
(p=0,048). Conclusion: We didn’t detected hypothalamic families with molecular diagnosis were included. Results: of 41.6 years. In 72.6% of the cases, genetic testing was
atrophy in HD patients, however, there is indirect evidence Four out seven were female. The most frequently present- offered, with 60% of them reporting having performed
of microstructural alterations that might justify the non ing symptoms were frequent falls and imbalance (42,8%). the test and of those who did not, 87.3% mentioned they
motor features observed in Huntington’s Disease patients. In five patients symptoms started in the first decade. would have liked to perform it. 63.2% said they received
Other symptoms were described, as feet deformities, genetic counseling and 68% decided not to have children
distal weakness, distal paresthesia, scoliosis, difficulty after being diagnosed as a carrier. The mean GOS score
Neurogenética walking and difficulty to run. Deep tendon reflexes were was 20.03 (4.1) points. There was a difference in the GOS
absent or reduced throughout, and vibratory sensation regarding schooling (p=0.021), with greater empowerment
was abnormal on the lower limb in all patients. Nerve of women with complete higher education compared to
conduction studies were characterized by patchy sen- those with complete elementary school or lower education
sory and motor reduction of the conduction velocity with (p=0.017). Per capita family income correlated with GOS
at least one nerve presenting with temporal dispersion (R=0.338, p=0.008), with higher income being associated
(TD). Conclusion: Biallelic pathogenic variants in the with greater empowerment, as well as women who par-
gene SH3TC2 are a known cause of demyelinating neu- ticipated in patient associations had higher GOS scores
ropathy, being the most common subtype of CMT4 in (2.72 points, 95% CI -0.90 to 4.55, p=0.004). Conclusion:
several populations. TD indicates desynchronization of Our research found that most of the women interviewed
components CMAP due to different rates of conduction. have genetic empowerment to deal with the diagnosis of
Recently, nerve pathology from SH3TC2-knockout ani- their children; however, they do not have it to deal with
mal models revealed loss of internodal architecture that the prevention and care of dystrophies in themselves.
could ultimately cause TD. Suggests an important role of It is necessary to instruct and refer these women about
this gene in peripheral nerve maintenance, which may the manifestation of DMDs in their bodies, in order to
be a frequent finding in patients with CMT4C that may prevent complications, and so that they can obtain a
differentiate it from other CMT subtypes. better quality of life.

Neurogenética Neurogenética

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XXX Congresso Brasileiro de Neurologia

TL 1106575 TL 1104939 TL 1105001

IMPACT OF RELIGIOSITY ON THE HEALTH OF PROFESSIONAL TRAINING PROGRAM FOR ANTI-LGI1 ENCEPHALITIS: A CASE SERIES
CHILDREN WITH DIFFERENT LEVELS OF ASD PEOPLE WITH MULTIPLE SCLEROSIS FROM BRAZIL
IN AN ASSOCIATION FROM PIAUÍ
Gisele Regina Barboza, Gecila Aragão Santos, Pedro Victor de Castro Silva, Marina Driemeier
Isabel Maria Oliveira Macedo Lima, Matheus Priscila Silva Santos, Alice Estevo Dias Cardoso, João Henrique Fregadolli Ferreira, Bruna
Oliveira de Brito, Matheus Rodrigues Corrêa, Assiria Freitas Dias, Alexandre Coelho Marques, Fabio Fieni
Associação Brasileira de Esclerose Múltipla – ABEM Toso, Lívia Almeida Dutra
Leite de Azevedo Costa, Hitalo Roberto de Araújo
Coêlho, Kelson James Almeida alice.estevo@abem.org.br Faculdade Israelita de Ciências da Saúde Albert
Universidade Federal Piauí. Teresina PI, Brazil Einstein
Background: Multiple Sclerosis (MS) is an autoimmune Hospital Israelita Albert Einstein
Universidade Estadual do Piauí. Teresina PI, Brazil
disease that affects the nervous system of people of work-
isabelmariaoliveiramacedolima@gmail.com ing age, with an impact on employability. Objectives: pvcsilva1@gmail.com
To present a professional skills development program
Background: Spirituality and religiosity interfere with the for people with MS. Methods: The free program was Background: Anti-LGI1 antibody causes an autoim-
health-disease process and patient longevity. In general, developed through a partnership between two brazilian mune encephalitis characterized by seizures of variable
the impact is positive and associated with protection entities, one focused on the rehabilitation of people with types, cognitive impairment, limbic encephalitis, and
against diseases and better quality of life (MISHRA, 2017). MS and other focused on training and promoting profes- rarely Morvan syndrome. Faciobrachial dystonic seizures
Spirituality, which may or may not involve religious prac- sional development. Seven remote meetings were orga- (FBDS) are the hallmark of the disease, occurring in 47%
tices and groups, shows great potential for coping with nized and publicized throughout the national territory, of cases. Anti-LGI1 encephalitis is more common in men,
stressful experiences (MARTÍNEZ, 2014). As an example, which addressed the topics of emotional intelligence, is rarely paraneoplastic, and responds well to steroids.
spirituality can be used as an ally in the traditional treat- marketing, entrepreneurship, finance, business idea Data from developing countries are lacking. Objective:
ment of epilepsy and other neurological disorders, such and company formalization. All those with MS, as well To describe epidemiologic and clinical features of a case
as autistic spectrum disorder (ASD) (VANCINI, 2016). as their relatives and caregivers, who expressed interest, series of anti-LGI1 encephalitis Methods: We reviewed
Thus, it is essential to establish the impact of spirituality were included. Results: 755 registrations were obtained charts of patients with anti-LGI1 antibodies from BrAIN
and religiosity, also, on the quality of life and development from people with MS, residing in 22 brazilian states. Of (brazilian Autoimmune Encephalitis Network) database
of patients with ASD. Objectives: to correlate the impact these, 418 (55.36%) regularly participated in the meet- from 2017 to 2022. Information on clinical, epidemiologi-
that religiosity causes on the health of children of differ- ings, completed the program and received a Certificate cal, and treatment features was compiled. Results: Of the
ent levels with ASD. Methods: Cross-sectional, analytical of Participation. All received didactic material and had 130 patients with AIE, we found 13 (10%) patients with
study with a qualitative approach. The sample is composed a technical team for the application of classes, lives and anti-LGI1 antibodies. Most were female (n=9, 65%), with
of 42 parents whose children have been diagnosed with calls. The Training Program promoted entrepreneurship, a mean age of 61,9 ± 13,2 years. Two patients had myas-
some degree of ASD and who attend the Associação de increased self-knowledge, enabled a greater network of thenia gravis (MG), thymoma, and developed Morvan
Pais e Amigos dos Excepcionais (APAE) in Teresina. The contacts, awakened competitiveness in business and syndrome with anti-CASPR2 antibodies; one had breast
research instruments chosen were the Duke Religiosity guided financial credit. Conclusion: The professional skills cancer. All the patients met the criteria for possible auto-
Scale (DUREL) and the Childhood Autism Rating Scale development program for people with MS is a pioneer in immune encephalitis, with 2 patients with criteria for
(CARS). DUREL (HGK), contains 5 questions that capture Brazil and was successfully implemented as a result of the limbic encephalitis (15%). The most frequent symptoms
3 of the dimensions of religiosity that are most closely partnership. Participants acquired knowledge both to were behavioural changes (n=13, 100%), epilepsy (76%,
related to health outcomes: organizational, non-organi- optimize their preserved skills and to develop new ones n=10), memory disturbances (69%, n=9), impaired level
zational, and intrinsic religiosity, in which the first two are for the professional market. of consciousness (38%, n=5), language disturbances
related to indicators of physical and mental health and (23%, n=3) and autonomic stability (15%, n=2). Reported
social support. The CARS is done through 15 questions abnormal facial movements were: FBDS (n=3, 23%), and
related to different aspects of the child’s life, so that it is Neuroimunologia other types in 4 (30%) including tremor, myokymia, and
possible to diagnose and define the degree of his or her dyskinesia. We observed variable taxonomy in describ-
ASD. After obtaining the data from the questionnaire, the ing Morvan Syndrome and abnormal facial movements.
data were filled in a Microsoft Excel spreadsheet and later Pleocytosis was found in 3 (23%) patients and abnormal
analyzed using Spearman software. Results: The grouped EEG findings in 12 (92%). Approximately 92% had abnor-
data were analyzed using Spearman’s correlation tests. mal MRI, and of those 53% (n=7) with mesial temporal
The correlation coefficients of the CARS with the DUREL T2/FLAIR hyperintensities – 4 of them with this find-
scale were: RO and RNO (-0.043; p= 0.788) and RI (0.169; ing happened bilaterally. Conclusion: We report vari-
p=0.314). Therefore, there was no significant association able findings amongst brazilian patients with anti-LGI1
between the autism scale score and intrinsic religiosity encephalitis: they were more commonly female, older,
nor with organizational and non-organizational religiosity. and with a higher frequency of abnormal CSF when
Conclusion: The study showed no association between compared to prior series. We found limbic encephalitis
the two variables analyzed, evidencing the complexity phenotype in only 15%, and prior reports indicate 90%.
of the factors linked to the psychosocial and familial Patients with Morvan syndrome and thymoma may har-
course of Autism. bour anti-LGI1 antibodies. Although our data need to
be confirmed in larger brazilian cohorts, our findings
may indicate the occurrence of genetic variability in the
Neurogenética disease. Nevertheless, our results indicate the need for
neurology training in recognizing anti-LGI1 encephalitis.

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TL 1105040 TL 1105112 TL 1105269

SAFETY AND EFFICACY OF INEBILIZUMAB LOWER GLOBUS PALLIDUS VOLUME NEUROFILAMENT LIGHT CHAIN LEVELS
IN NMOSD OVER A MEAN TREATMENT IN MULTIPLE SCLEROSIS PATIENTS DURING RELAPSING PHASES OF MULTIPLE
DURATION OF 3.2 YEARS: END OF STUDY ASSOCIATED WITH DISEASE DURATION: A SCLEROSIS AND NEUROMIELITE OPTIC
DATA FROM THE N-MOMENTUM TRIAL POSSIBLE DISTINCT NEURODEGENERATIVE SPECTRUM DISORDER
PROCESS
Bruce AC Cree, Jeffrey L Bennett, Brian G Larissa Araujo Duarte, Andreza Salvio Lemos,
Weinshenker, Dean M Wingerchuk, Friedemann Luciano Chaves Rocha, Jéssica Santos Souza Milene Teixeira Ataíde, Renan Amphilophio
Paul, Ho Jin Kim, Sean J Pittock, Dewei She, Daniel Rocha, Ricardo Mendes Rogério, Hideraldo Luis Fernandes, João Gabriel Dib Farinhas, Vinicius
Cimbora, Ivo Marguti Souza Cabeça Gabriel Coutinho Costa, Valeria Coelho Santa
Rita Pereira, Assuncion Martinez Liem, Márcia
UCSF Weill Institute for Neurosciences, Department Hospital Ophir Loyola. Belém PA, Brazil Waddington Cruz, Soniza Vieira Alves-Leon
of Neurology, University of California San Francisco,
San Francisco, CA, USA – Estados Unidos lcrocha1992@hotmail.com Universidade Federal do Rio de Janeiro. Rio de
University of Colorado School of Medicine, Anschutz Janeiro RJ, Brazil
Medical Campus, University of Colorado, Aurora, CO, Background: Multiple Sclerosis (MS) is a chronic inflam- Universidade Federal do Estado do Rio de Janeiro.
USA – Estados Unidos matory disease of the central nervous system, character- Rio de Janeiro RJ, Brazil
University of Virginia, Charlottesville, VA, USA – ized essentially by white matter lesions, or plaques, and
Estados Unidos acute relapses with focal neurological deficits. However, larissa.ad@hotmail.com
Department of Neurology, Mayo Clinic, Scottsdale, a chronic disease course with neurological and radio-
AZ, USA – Estados Unidos logical deterioration has been described with limited Background: Multiple Sclerosis (MS) and Neuromyelitis
Experimental and Clinical Research Center, Max biomarkers and predictors. Objective: The present study Optic Spectrum Disorders (NMOSD) are autoimmune dis-
Delbrück Center for Molecular Medicine, Charité aimed to evaluate volumetric analyzes of magnetic res- eases that present different morbidity. NMOSD patients
Universitätsmedizin Berlin, Berlin, Germany – sonance imaging in multiple sclerosis (MS) patients with reach higher levels of EDSS and lethality rate in a shorter
Alemanha the puporse to stabilish associations or predictors of poor interval of time when comparing with MS, due to more
Research Institute and Hospital of National Cancer outcome/severity of this disease in a reference center in aggressive relapsing. brazilian population is characterized
Center, Goyang, Republic of Korea – Coréia do Sul Pará state. Methods: a descriptive and transversal study by high miscegenation and ethnicity is also associated to
Department of Multiple Sclerosis and Autoimmune with patients’ records evaluation and usage of imaging disease severity. Serum Neurofilament light chain (NfL)
Neurology, Mayo Clinic, Rochester, MN, USA – of magnetic ressonance to generate volumetric data level is a noninvasive toll to measure neurodegeneration.
Estados Unidos in VolBrain platform. Thirteen patients with MS were Objective: Measuring Serum Neurofilament Light Chain
Horizon Therapeutics plc, Gaithersburg, MD, USA – evaluated and fifteen imagings of healthy control sub- level during relapsing phases of MS and NMOSD patients.
Estados Unidos jects paired by age were obtained from online databank. Methods: We include five of 60 NMOSD patients, nine of
Horizon Therapeutics, Brazil Results: the brain volume and other diverse structures, 250 MS patients during relapsing phase and 24 healthy
in cm3, showed significant reduced values in MS patients control matched by age. Plasma samples were underwent
imarguti@horizontherapeutics.com compared to healthy controls, with relative preservation by single molecule array (SIMOA) to investigate NfL level
of cortical frontal structures and normal appearing white according to the manufacturer’s instructions, with limit of
Background: Inebilizumab is approved in the USA and matter. None volumetric variable correlated with age, age detection 0.05 pg/mL. Results: Among NMOSD patients,
Japan for aquaporin 4 immunoglobulin (Ig)G seroposi- at diagnosis and disability (EDSS). The Globus Pallidus all were female with mean age of 44.4 years, and among
tive neuromyelitis optica spectrum disorder (NMOSD). volume was the only structure that showed strong cor- MS patients six were female with mean age of 42.8 years.
Objective: To report final safety and efficacy data from the relation and significant association in regression analyses Healthy control mean age was 40.5 years. NfL level MS
N-MOmentum trial of inebilizumab in NMOSD. Methods: with disease duration, showing the dependency between patients was 26.99 pg/mL, NfL level in NMOSD patients
Participants with NMOSD (aged 18+, EDSS score of ?8, these two variables. Conclusion: this study concludes was 244.64 pg/mL and in healthy control was 6.77 pg/
recent history of attacks) were randomized 3: 1 to ine- that, just as reported in scientific literature, MS patients mL. Conclusion: Significant difference between MS and
bilizumab or placebo monotherapy for 28 weeks or up showed diminished volume in diverse brain structures, NMOSD NfL levels (p < 0.01) seem to be in according to
to attack occurrence; the randomized controlled period suggesting a neurodegenerative process with relative more severity pathophysiology of NMOSD and reinforce
(RCP). Primary outcome was time to adjudicated attack. sparing of frontal and normal appearing white matter the necessity of aggressive treatment to prevent and treat
Participants could then enter the inebilizumab open label that occurs independently of disability. Yet, the Globus autoimmune diseases associated to neurodegenerative
period (OLP). Final study data are presented, including Pallidus volume, in isolation, what diverges from scientific process. Yet, our NfL results in brazilian NMOSD patients
attack risk and safety outcomes. Results: Of the 230 par- literature, could be a potential biomarker of disease pro- can possible be associated to the population ethnicity and
ticipants randomized and dosed, 216 (93.9%) entered and gression, though, more studies are necessary to stabilish need to be repeated in a larger number of patients and
174 (80.6%) completed the OLP. In the RCP, 87.0% were better relationship between these variables. population background.
attack free with inebilizumab and 59.9% with placebo
(72.8% risk reduction, p<0.001). In the OLP, 87.7% were
attack-free in those continuing inebilizumab and 83.4% Neuroimunologia Neuroimunologia
in those switched from placebo. Regardless of randomiza-
tion, 225 participants received inebilizumab. Mean (SD)
treatment duration was 3.2 (1.4) years; 36.8% were treated
for >4 years (maximum of 5.5 years). Total exposure was
730.36 person-years (py) with an annualized attack rate
of 0.092; 40/63 (63.5%) attacks occurred in the first year.
Treatment-emergent adverse events (AE) were reported
by 89 (39.6%) participants, most frequently urinary tract
infection (26.2%), nasopharyngitis (20.9%) and arthral-
gia (17.3%). Infusion-related reactions with inebilizumab
occurred in 28 (12.9%) participants (rate per 100-py: whole
study, 11.1; RCP, 37.6). The rate (95% confidence interval)
of infections per 100-py did not increase with continued
treatment: year 1, 116.3 (102.4-131.6); year 2, 68.1 (57.2-
80.6); year 3, 61.9 (50.3-75.5); year 4, 55.1 (41.7-71.4). 105
participants had transient low IgG (<700 mg/dL) during
treatment, but no correlations were found between the
worst IgG, IgM or IgA levels recorded and the occurrence
of any infection or an infection grade 3 (Fisher exact test,
all p>0.05). Three trial participants died: one from com-
plications of NMOSD attack, one from a CNS event of
unclear etiology and one due to COVID-19, after 9, 224
and 1225 days of inebilizumab treatment, respectively.
Conclusion: During the 5.5 years of N-MOmentum,
the risk of attack in participants receiving inebilizumab
remained low with no evidence of unexpected serious
adverse events, including serious infection.

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TL 1105297 TL 1105412 TL 1105479

BRAZILIAN AUTOIMMUNE ENCEPHALITIS SERUM PRESENCE OF ANTINUCLEAR RADIOMICS IN THE STUDY OF SPINAL CORD
NETWORK (BRAIN) STUDY: ANTIBODY ANTIBODIES IN NMOSD PATIENTS RELATES INJURIES CAUSED BY MULTIPLE SCLEROSIS,
PROFILE, CLINICAL CHARACTERISTICS TO AQP4-IGG REACTIVITY MNOSD AND HTLV-I VIRUS INFECTION
FROM A NATIONAL WIDE NETWORK
Marcos Baruch Portela Filho, Tayla Samanta Silva Rodrigo Queiroz Silveira, Viviane Tavares Carvalho
Fabio Fieni Toso, Bruna Freitas Dias, Mariana dos Santos, Ivã Taiuan Fialho Silva, Fernanda Crelier, Priscila dos Santos Mageste, Ursula
Driemeier Cardoso, Pedro Vitor de Castro Silva, João Ferreira de Abreu, Evelin Duarte Serpa, Débora Medeiros Araujo de Matos, Felipe Schmidt,
Henrique Fregadolli Ferreira, Alexandre Coelho Lopes dos Santos, Juliana de Alencar Fontes, Gutemberg Augusto Cruz dos Santos, Augusto
Marques, Livia Almeida Dutra Catarina Ester Gomes Menezes, Lécio Aragão Souza Righetti Vieira Ferreira de Araujo, Fernanda
Morais, Thiago Gonçalves Fukuda Rueda, Jefferson Becker, Osvaldo José Moreira do
Hospital Israelita Albert Einstein Nascimento
UNEB. Salvador BA, Brazil
fabiofieni@yahoo.com.br Universidade Federal da Bahia. Salvador BA, Brazil Universidade Federal Fluminense. Niterói RJ, Brazil
Universidade do Estado do Rio de Janeiro. Rio de
Background: Autoimmune encephalitis (AIE) is a group marcosbaruch05@outlook.com Janeiro RJ, Brazil
of inflammatory diseases characterized by prominent Universidade Federal Fluminense / IDOMED –
neuropsychiatric symptoms associated with neuroglial. Background: Neuromyelitis optica spectrum disorder Niterói – RJ – Brazil
Most common AIE is anti-NMDAR encephalitis, followed (NMOSD) is an autoimmune and demyelinating dis- Escola de Medicina da Pontifícia Universidade
by anti-lgI1 and anti-CASPR2. Data on clinical features ease of the central nervous system (CNS), whose patho- Católica do Rio Grande do Sul. Porto Alegre RS,
from developing countries are lacking. Objectives: To physiology is related to the anti-aquaporin 4 autoanti- Brazil
describe the clinical and epidemiological profile of AIE body (AQP4-IgG). Patients with NMOSD tend to present
in the brazilian population and to verify the sensibility of with concurrent autoimmune diseases, such as systemic rqsilveira92@gmail.com
the criteria methods brazilian Autoimmune Encephalitis lupus erythematosus (SLE), Sjögren’s syndrome (SS) and
Network (BrAIN) is a prospective study on AIE involving Hashimoto’s thyroiditis (HT). These patients may also Background: Radiomics is an emerging field that uses
17 centers from all brazilian regions, in partnership with express autoimmunity biomarkers, such as antinuclear Artificial Intelligence for medical research. Radiological
the Medical University of Vienna. Inclusion criteria are antibodies (ANA), even without a given diagnosis of other imaging exams can be statistically analyzed in a way
possible AIE according to Graus and agreement with autoimmune diseases. Therefore, we aimed to evaluate that allows extracting multiple graphical elements not
informed consent. Demographic data, clinical presenta- the influence of the serum presence of ANA on clinical visible in routine assessments and transforming them
tion, presence of neoplasia, and treatment were compiled and prognostic aspects of NMOSD patients. Methods: into numerical data. It is a method that extracts a large
by the attending physician using REDCap. CSF analysis, Cross-sectional study carried out in a public outpatient number of features from medical images using data-char-
EEG and brain MRI were also recorded. CSF and serum clinic (Ambulatório Magalhães Neto, HUPES-UFBA) in acterization algorithms. The main steps of radiomics
samples were tested using TBA for intraneuronal and sur- Salvador, Brazil. Clinical and demographic data were analysis include image collection as well as reconstruc-
face antigens, followed by CBA or immunoblot. Results: collected using a standardized questionnaire. Laboratory tion, segmentation of the region of interest (ROI), feature
We included 547 patients and tested 512 for ANeA, which and MRI results were obtained through a retrospective extraction as well as quantification, and establishment
402 (78,5%) fulfilled the probable AIE criteria and 103 search of medical records. EDSS, annualized relapse rate of the predictive as well as prognostic models. Through
(25.6%) were confirmed as seropositive AIE. The following (ARR) and progression index (EDSS/disease time) were this technology, it is possible to analyze the heterogene-
ANeA were found anti-NMDAR (n=53, 51%), anti-MOG used as prognostic parameters. Results: 106 patients were ity of tissues and lesions at the cellular level, helping to
(n=12,11.5%), anti-GAD (n=10, 9.6%), anti-LGI1(n=7, enrolled, of which 85 (80.2%) were female and 84 (79.2%) distinguish pathophysiological processes in a non-inva-
n=6.7%), anti-Caspr2 (n=4, 3.8%), anti-AMPAR (n=4, 3.8%), were afrodescendants. The mean age at disease onset was sive way. Compared to biopsy studies, in radiomics, the
anti-GABA-BR (n=3, 2.9%), anti-glyR (n=3, 2.9%). In pedi- 36.5 (±13.6) years. Sixteen (15.6%) patients were diagnosed whole tissue or the whole lesion is available for analysis,
atric population, it was detected only two antibodies: 21 with a concomitant autoimmune disease, the most fre- and not just a short sample. In this way, radiomics plays
anti-NMDAR (75%) and 7 anti-MOG (25%). Sensitivity of quent being HT (6.8%) and SLE (3.8%). 41 patients had an important role in daily practice, once it can distinguish
clinical criteria for possible AIE was 94%, and Specificity documented ANA results ANA was positive in 23 (52.3%) diseases that eventually look alike radiologically, but hold
was 26%; Negative predictive value was 95%. Predictable patients. Serum presence of ANA was related to reactivity completely different pathophysiology and treatments. In
variables of AIE in pediatric population were decreased for AQP4-Ig with a trend towards statistical significance our case, the diseases under study are MS, NMOSD, and
level of consciousness (p=0.042), chorea (p=0.008), and (85.7% vs 57.1%; p=0.089). ANA positive patients had a HTLV-I virus, a common infectious disease in our coun-
dystonia (p=0.018) and in adults, movement (p=0.016) and higher frequency of concomitant autoimmune diseases, try. Objectives: Our objectiveis to use the technology of
memory impairment (p=0.042). Conclusion: Frequency of although without statistical significance (28,6 vs 14,3; radiomics to analyze, in a non-invasive way, spinal cord
antibodies in AIE vary among populations, and we found p=0.406). There were no significant differences between injuries caused by Multiple Sclerosis, MNOSD and HTLV-I
unexpected high rates of anti-glycine and anti-MOG. In the groups regarding age at onset (39,4 ± 8,4 vs 38,5 ± 17,0; virus infection. Thus, it is possible to identify the structure
our population sensitivitty for AIE clinical criteria is low p=0.871), sex (85,7% vs 82,1%; p=1.000), initial clinical and components of the lesions, helping to distinguish the
(26%) with high negative predictive value. Clinical vari- core syndrome (p=1.000 for transverse myelitis; p=1.000 different medical conditions and guiding to adequate
ables that could help better predict AIE in children were for optic neuritis), EDSS (4,5±2,4 vs 5,2±2,8; p=0.424), ARR treatment, as well as accompanying the structural dif-
decreased level of consciousness, chorea and dystonia. (1,6±1,6 vs 2,0±1,8; p=0.749) or progression index (4,9±5,6 ferences during treatment or surveillance. The need for
Among adults, movement and memory.Future scores vs 3,7±5,5; p=0.938). Conclusion: The serum presence a consistent number of patients for the study brings to us
for better AIE prediction should consider specific clin- of ANA was not related to specific clinical syndromes at a necessary inclusion of patients from different Centers
ical presentation. disease onset nor to a worse prognosis in patients with in Brazil, thus comprising a multicenter study. Methods:
NMOSD. An interrelationship between ANA and AQP4- This is an ongoing study in which patients enrolled are
IgG was observed, ratifying the humoral autoimmunity those in whom there is a spinal lesion with common
Neuroimunologia predisposition in NMOSD patients. HT and SLE were characteristics for MS, NMOSD, or HTLV-1 disease and
the most prevalent diseases concurrent with NMOSD who will be submitted under radiological investigation
in our sample. with the aid of radiomics technology to differentiate in a
non-invasive and unequivocal way these three conditions.
Conclusion: This study will allow us to demonstrate the
Neuroimunologia great potential of radiomics in the diagnosis and monitor-
ing of neurological diseases. We invite neurologists from
other centers to take place in this project.

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TL 1105494 TL 1105566 TL 1105607

HETEROGENEOUS PHENOTYPES OF THE VARIABILITY OF SUBACUTE ONSET NEUROFILAMENT LIGHT CHAIN LEVELS
INFLAMMATORY DEMYELINATING DISEASES HTLV-1 MYELOPATHY AS BIOMARKER OF SEVERITY ACCORDING
AND TGF-B1 AS POTENTIAL BIOMARKER OF TO THE PRESENCE OF ANTI-AQP-4 IN
João Pedro Moraes Miossi, Gabriel Marim Roni,
DISEASE PROGRESSION Luis Henrique Sunderhus de Oliveira, Guilherme NEUROMYELITIS SPECTRUM DISORDER
Machado Vieira, José Guilherme Martins Lima, PATIENTS
Vinicius Gabriel Coutinho Costa, Larisse Araújo
Duarte, Sheila Espírito Santo Araújo, Valéria Coelho Rodrigo Leite Marinho, Gustav Barbosa Falcão,
Renan Amphilophio Fernandes, Larissa Araújo
Santa Rita, Asunción Martinez Liem, Flávia Carvalho Kézia de Souza Pinheiro, Victória Pagani Samora
Duarte, Milene Teixeira Ataíde, Andreza Salvio
Alcantara Gomes, Soniza Vieira Alves-Leon Sousa, Paula Zago Melo Dias
Lemos, Jéssica Vasques Raposo-Vedovi, João
Universidade Federal do Espírito Santo – Vitória – Gabriel Dib Farinhas, Vinicius Gabriel Coutinho
Instituto de Ciências Biomédicas/UFRJ. Rio de
ES – Brazil Costa, Valéria Coelho Santa Rita Pereira, Assuncion
Janeiro RJ, Brazil
Hospital Universitário Cassiano Antonio Moraes – Martinez Liem, Soniza Vieira Alves-Leon
Faculdade de Medicina/UFRJ. Rio de Janeiro RJ,
Brazil Vitória – ES – Brazil
Universidade Federal do Estado do Rio de Janeiro.
UENF – Campos dos Goytacazes – RJ – Brazil João pedromiossi@gmail.com Rio de Janeiro RJ, Brazil
Hospital Universitário Clementino Fraga Filho/UFRJ. Universidade Federal do Rio de Janeiro. Rio de
Rio de Janeiro RJ, Brazil HTLV-1 infects around 10-20 million people worldwide Janeiro RJ, Brazil
vgcoutinho@outlook.com and despite its prevalence only a small fraction of the renanyfernandes@gmail.com
infected individuals ever manifest HTLV-1 related pathol-
Idiopathic inflammatory demyelinating diseases ogy. The most common neurological manifestation of
Neuromyelitis Optic Spectrum Disorder (NMOSD) is a
(IIDDs) are a group of Central Nervous System disor- HTLV-1 is the tropical spastic paraparesis, a chronic spastic
severe Central Nervous System (CNS) inflammatory and
ders which have white matter lesions as common feature. paraparesis of insidious onset. The clinical manifestations
autoimmune disease, preferentially affecting the spinal
Neuroimaging and molecular assays led to the establish- include spasticity and/or hyperreflexia of lower extremi-
cord and optic nerves. Advances in diagnostic criteria
ment of more discernible diagnosis in this field, such ties, urinary disturbances and lower extremity weakness.
have allowed greater diagnostic sensitivity and specific-
as multiple sclerosis (MS), neuromyelitis optica spec- Radiologically, brain white matter lesions and mild atrophy
ity and the inclusion of patients without antiaquaporin-4
trum disorders (NMOSD) and MOG associated diseases of the spinal cord can be observed. Symptomatic treat-
antibody (AQP-4 ab). Neurofilament light chain (NfL)
(MOGAD), each with their own clinical course and ther- ment remains the mainstay of therapy. To report 3 cases
is an important biomarker of neurological damage in a
apeutic interventions. In this work, we aim to determine of subacute onset myelopathy due to HTLV-1 infection.
wide spectrum of neurological diseases. NfL levels may
the clinical profile and disease course in a population of A retrospective study was performed on 3 patients with
contribute to evaluate disease severity. Here, we analised
patients from an IIDDs reference center. To explore new subacute onset myelopathy, positive serology for HTLV-1
samples from a coorte of 59 follow-up NMOSD patients
tools for course evaluation, we used in vitro assays to pro- and absence of other causes of acute myelopathy. The
in treatment at HUCFF Demyelinating Diseases outpa-
pose potential markers. In preliminary analysis, we had 60 clinical history, physical examination, CSF, MRI, treat-
tient Clinic. All these patients match the 2015 diagnostic
patients enrolled. From those, 46 patients were diagnosed ment and disclosure of the cases were all reviewed. All 3
criteria for NMOSD, and they underwent anamnesis,
with MS, being 43 with relapse-remitting (RRMS), 2 with patients were male, the age ranged between 29-41 years
laboratory tests and neuroimaging. They were divided
secondary progressive disease (SPMS) and 1 with primary old. They were admitted to our Hospital with a history
into 2 groups: anti-AQP4 positive and anti-AQP4 nega-
progressive (PPMS). 8 presented with NMOSD, 1 with of paraparesis, difficulty in walking and urinary distur-
tive. This anti-aquaporine-4 status was achieved by CBA
optic neuritis, 1 with acute disseminated encephalomy- bances and/or constipation over the last 2-5 months. The
(Cell-Based Assay), prior to the beginning of NfL tests.
elitis and 3 with a clinically isolated syndrome. Another spinal MRI showed no significant alteration in one case,
To evaluate the neuronflammation targeting NfL as bio-
patient was still with diagnosis pending. We determined one presented as longitudinally extensive myelitis with
marker, we collected blood samples by venipuncture in
59,7% of patients presented with a multifocal disease as swollen and high T2 signal in the cervical and thoracic
EDTA tubes and plasma was isolated. Furthermore, we
early as clinical onset, with sensitive (44,%), pyiramidal spinal cord and the other with discrete degenerative
used the NfL kit for SIMOA (Single Molecule Array), an
(30,4%) and brainstem (23,2%) being the most commit- alterations in cervical and lumbar spine, in addition to
ultrasensitive immunoassay, for detecting e quantifying
ted functional systems. And although 60% of patients got small foci of signal alterations in the high thoracic region.
this neurological biomarker in plasma. We observed
an EDSS equal or higher than 3,0 during onset, 77,8% CSF examination revealed pleocytosis in all cases range
that the proportion of patients with positive anti-AQP4+
got total remission, even if 35,2% didn´t get any medical between 15-28 leukocytes, with a lymphocytic-mononu-
(65.7%) was higher than negative ones (34,21%). Also,
care. At time of current assessment, though, 25% percent clear pattern. All three patients had a positive serology for
during relapse NMOSD, they presented an increase in
already got to that level. From these, 6 (10% from total) HTLV-1, and one was positive since childhood but had
NfL levels when compared to non-relapse patients and
were equal or higher than 6,0. Investigating which CNS no symptoms until 2 months before admission. The two
healthy controls (p< 0.001). Independently of relapse
molecules could be used for disease evaluation on those patients with abnormal MRI had negative AQP-4 IgG and
moment, AQP-4 ab (+) patients present significant higher
patients, we performed in vitro assays: astrocytes from one negative anti-MOG. Two patients received cycles of
level of NfL compared with AQP-4 ab (-) (p <0,05). NfL
healthy mice, cells linked to inflammation and synaptic pulse therapy with IV mPSL with only insignificant or no
high level may represent not only a biomarker of active
regulation, were cultured and treated with myelin proteins. improvement at all. One of these patients was still sub-
disease, but also an important biomarker for a more severe
While MOG exerted little response on those cells, ?24 hour mitted to 5 plasmapheresis without any improvement.
and neurodegenerative course in AQP-4 ab (+) patients.
treatment of cultures with Nogo-A led to defective expres- These two patients maintained urinary incontinence,
sion of pro-synaptogenic factors. Among them, we found erectile dysfunction and constipation, and, from these,
a nearly 30% reduction in TGF-B1, a cytokine that in CNS one became totally plegic in inferior limbs. The patient Neuroimunologia
exerts a neuroprotector effect, being currently considered which did not receive the mPSL treatment, had mild
both as therapeutic target and biomarker for an array of improvement of the symptoms. Despite an uncommon
neurodegenerative diseases. Thus, here we identified presentation, HTLV-1 can cause subacute paraparesia and
TGF-B1 as a player in demyelination synaptopathy, with thus, represents a differential diagnosis for neuromyelitis
potential for investigation as a biomarker in IIDDs. New optica and other inflammatory myelitis.
assays are still needed to functionally relate that mole-
cule to different stages of disease progression, helping to
Neuroimunologia
better define its clinical employability.

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FACTORS AGGRAVATING QUALITY OF LIFE IN FACTORS RELATED TO JC VIRUS ANTI-NMDA AUTOIMMUNE ENCEPHALITIS:
AUTOIMMUNE DISEASES OF THE CENTRAL SEROPREVALENCE AND SEROCONVERSION DESCRIPTIVE ANALYSIS OF 75 PATIENTS
NERVOUS SYSTEM: A COMPARATIVE IN PATIENTS TREATED WITH NATALIZUMAB FROM BRAIN DATABASE
STUDY BETWEEN NEUROMYELITIS OPTIC
Lidiane Alves de Macedo Souza, Maria Lúcia Brito Marina Driemeier Cardoso, Pedro Victor de Castro
SPECTRUM DISEASES (NMOSD) AND Ferreira, Lara de Menezes Andrade, Maria Iris Silva, João Henrique Fregadolli Ferreira, Bruna de
MULTIPLE SCLEROSIS (MS) Moraes Machado, Ávaro Jose Porto Moreira Freitas Dias, Alexandre Coelho Marques, Fabio Fieni
Toso, Livia Almeida Dutra
Tayla Samanta Silva dos Santos, Ivã Taiuan Fialho Hospital da Restauração. Recife PE, Brazil
Silva, Marcos Baruch Portela Filho, Juliana de Hospital Israelita Albert Einstein
Alencar Fontes, Fernanda Ferreira de Abreu, Silas lidianemacedohr@gmail.com
Paulo Lima de Souza, Gabriel Souza Suzart, Vinicius pvcsilva1@gmail.com
Leal Borges da Cruz, Thiago Gonçalves Fukuda, Background: Natalizumab (NTZ) is a monoclonal anti-
Jamary Oliveira Filho body effective in treatment of relapsing-remitting multi- Background: Anti-NMDAR encephalitis is the most com-
ple sclerosis (RRMS), however high in title the antibody mon type of autoimmune encephalitis (AIE), and clinical
Complexo Hospitalar Universitário Professor Edgard against the John Cunningham virus (JCV), the antibody picture involves prodromic symptoms followed by a neu-
Santos – UFBA. Salvador BA, Brazil can often limit the drug use due to the risk of develop- ropsychiatric syndrome. Patients present acute behavioral
Universidade do Estado da Bahia – UNEB. Salvador ing progressive multifocal leukoencephalopathy (PML). changes, epilepsy, memory impairment, reduced level
BA, Brazil Objective: This retrospective study has the objective of of consciousness movement disorders, language distur-
taylasamanta@gmail.com evaluating the relationship between sero-prevalence and bances and autonomic instability. Pediatric population
epidemiological factors, previous therapy used and treat- (<13 years old) usually present seizures and movement
ment time in the MS population studied. Methods: Anti- disorders, while adult patients tend to have behavioral
Background: Neuromyelitis optical spectrum diseases
JCV levels were evaluated in 30 patients diagnosed with changes. Data from the brazilian population are lacking.
(NMOSD) and multiple sclerosis (MS) are autoimmune
multiple sclerosis and followed up at a treatment center Objective: To describe clinical and epidemiological profile
diseases of the central nervous system (CNS) that establish
in the city of Recife, and with known serological status at from 75 anti-NMDAR encephalitis patients and to com-
a chronic inflammatory and demyelinating process. The
the initiation of treatment, and who kept follow-up in that pare with prior reports. Method: Chart of patients with
limitations caused by the active disease, or the sequelae,
service. All patients studied had no symptoms suggestive of anti-NMDAR antibodies from BrAIN (brazilian autoim-
can negatively impact the quality of life, in addition
PML and were on Natalizumab monotherapy. Serum tests mune encephalitis network) database from 2017 to 2022
to causing disorders such as depression and anxiety.
were performed using the enzyme-linked immunosorbent were reviewed. Information on clinical, epidemiological
Comparing the quality of life between the diseases is a
method (ELISA) at the initiation of treatment, followed by and treatment were compiled. Patients were divided in
point to be explored, as the comparative studies carried
annual control tests. Results: The majority (73.4%), were two age groups: pediatric (younger than 13) and adult
out differ both in terms of the presence of differences
female. The highest percentage of patients (36.6%) were (13 or older). Results: Of the 130 patients with AIE, we
between groups of diseases and the severity of the factors.
over 50 years old at the time of the survey. Most patients found 75 (58%) patients with anti-NMDAR antibodies.
Objective: To compare the quality of life in patients with
(46%) were dark-skinned. Baseline positivity was 16.6%, Approximately 69% were female, mean age was 13,8 ±10,1
NMOSD or MS, as well as to determine aggravating factors.
and overall seroconversion was 23%. Of the baseline pos- years. There were 28 (37%) pediatric patients. Thirty-five
Methods: Cross-sectional, single-center study conducted
itive patients, 100% had been using immunosuppressants (47%) patients presented prodromes, most commonly
with patients who comply with the 2015 International
for a period greater than 2 years. In the group of patients headache (n=17, 23%;), fever (n=17,23%;) and nausea/
Consensus criteria for the diagnosis of NMOSD or the
who seroconverted during treatment, 76% had also used vomiting (n=10, 13%;). The most reported symptoms
2017 McDonald criteria for the diagnosis of MS. The
immunosuppressants previously. The most used immu- were behavioral, psychiatric, and cognitive changes (99%;
quality of life was quantified by the EQ-5D Quality of Life
nosuppressant was interferon beta 1a (INF-?1a) 22 or 44 n=74), epilepsy (85%; n=64) and movement disorders
Questionnaire. Depression and anxiety were measured
?g subcutaneously three times a week. It not possible to (81%; n=61). Most common symptoms in adult and pedi-
by the Hospital Anxiety and Depression Scale (HAD).
have a relationship between seroprevalence and increas- atric population were movement disorders. Pleocytosis
Fatigue was measured by the Modified Fatigue Impact
ing age, as also no relationship was identified between was reported in 56%. Abnormal EEG was present in 87%
Scale (MFIS). The degree of disability was expressed by
the time of exposure to medication and seropositivity. (n=65) and 18% (n=12) had extreme delta brush. Thirty-
the Expanded Disability Status Scale (EDSS). Results: 229
Conclusion: There was variation in seroconversion rates two patients (43%) had abnormal brain MRI, ten of them
patients were enrolled, of which 69 (30.1%) were diagnosed
in the different cohorts studied, but the rate found in this were children. Two patients reported neoplasia, one
with NMOSD and 160 (69.9%) with MS. The mean quality
study is like those found in European and American coun- ovarian teratoma and the other a pulmonary mass under
of life by EQ-5D was 0.59 (SD ± 0.23). Forty-three patients
tries. There is a strong correlation between seroprevalence investigation. Methylprednisolone was the most common
(18.8%) were classified as having probable symptoms for
and previous use of immunosuppressants. None of the first line treatment (n=54, 52%) followed by intravenous
anxiety and thirty-four (14.8%) as having probable symp-
patients in the group developed PML. For patients with immunoglobulin (n=35, 47%). Conclusion: In Brazil, anti-
toms for depression. One hundred forty-four patients
anti JC titers above 1.5 associated with infusion time lon- NMDAR encephalitis follows the typical clinical picture,
(62.9%) reported symptoms compatible with fatigue on
ger than two years, a modification of the interval between being more frequent among young females. We found a
the MIFS scale. Patients with NMOSD had worse quality
infusions was defined. lower association with neoplasm in our population when
of life [0.51 (± 0.23) vs 0.62 (± 0.22); p = 0.001]. We did not
compared to other series.
detect any difference between anxiety symptoms [7.60 (±
4.60) vs 7.60 (± 4.60); p = 0.978], depressive symptoms [6.7 Neuroimunologia
(± 4.90) vs 6.1 (± 4.10); p = 0.353] and fatigue [43.8 (± 17.3) Neuroimunologia
vs 43.5 (±19.9); p = 0.916]. In multivariate analysis, higher
scores on the EDSS, MFIS, older age, and employment
status were independently associated with worse qual-
ity of life. There was no association between diagnosis of
NMOSD and quality of life. Conclusion: NMOSD and MS
have similar impacts in quality of life after adjusting for
disease severity. However, disease severity is clearly worse
in NMOSD when compared MS, secondarily impacting
quality of life.

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CLINICAL DIFFERENCES AMONG CHILDREN COMPARISON OF VISUAL ACUITY RECOVERY FATIGUE IN MULTIPLE SCLEROSIS PATIENTS
WITH SERONEGATIVE NORSE AND ANTI- IN 3-,5-, AND 7-DAY SCHEDULES OF
Tayla Samanta Silva dos Santos, Alex de Cerqueira
NMDA ENCEPHALITIS INTRAVENOUS METHYLPREDNISOLONE IN Silveira Figueiredo, Antonio Lázaro Santos Soares
PATIENTS WITH OPTIC NEURITIS: A CASE- Junior, Vinicius Leal Borges da Cruz, João Paste
João Henrique Fregadolli Ferreira, Bruna Freitas
Dias, Marina Driemeier Cardoso, Pedro Victor Castro CONTROL STUDY Silva, Silas Paulo Lima de Souza, Thais Barbosa
Silva, Alexandre Coelho Marques, Fabio Fieni Toso, de Oliveira, Milena Fernandes de Oliveira, Thiago
Fernando Cavalcanti de Sá e Benevides Falcão,
Lívia Almeida Dutra Gonçalves Fukuda, Pedro Antonio Pereira de Jesus
Sara Terrim, Cleuber Esteves Chaves, Maria Alice
Hospital Israelita Albert Einstein Pimentel Falcão, Guilherme Diogo Silva Complexo Hospitalar Universitário Professor Edgard
Santos – UFBA. Salvador BA, Brazil
jhfregadolli@gmail.com Universidade de São Paulo. São Paulo SP, Brazil
taylasamanta@gmail.com
f.falcao@hc.fm.usp.br
Background: NORSE patients often fulfill the criteria for
possible AIE, nonetheless nearly half of the cases are cryp- Background: Multiple Sclerosis (MS) is an inflammatory
Background: Intravenous methylprednisolone (IVMP)
togenic. NORSE secondary to anti-NMDAR encephalitis demyelinating disease that affects about 2.3 million people
is the main therapy for optic neuritis to accelerate and
have different treatment options and response rates, and worldwide. Fatigue is characterized by lack of physical and
improve final visual outcomes. However, systematic
data comparing both diseases are scarce. Objective: To mental energy, and can be of primary cause as a result
reviews demonstrated studies used different schedules
compare clinical and paraclinical characteristics of pedi- of the underlying demyelinating disease or of second-
of IVMP 1 g/day and the optimal duration of IVMP is
atric patients with NORSE and anti-NMDAR. Methods: ary cause to comorbid conditions. Among the various
unknown. Objective: We aimed to investigate if 3-day,
Chart of patients ? 13 years with NORSE that fulfill the AIE impairments to the quality of life of patients with MS, this
5-day, and 7-day schedules of IVMP could lead to dif-
criteria included in BrAIN (brazilian autoimmune enceph- is one of the most frequent and debilitating conditions.
ferent recovery of visual outcomes. Methods: We per-
alitis network) database from 2017 to 2022 were reviewed. Objective: This study aims to evaluate the impact of fatigue
formed a case-control study in a tertiary center in São
Information on clinical, epidemiological and treatment on patients monitored in a reference outpatient clinic
Paulo, Brazil. We selected from a registry of consecutive
data were compiled. NORSE was defined as new-onset for Demyelinating Diseases. Methods: Cross-sectional,
patients with optic neuritis, patients that received 7-day
refractory status epilepticus in patients without active epi- single-center study conducted with patients meeting the
and 3-day schedules of IVMP 1 g/day. We chose controls
lepsy or other preexisting relevant neurological disorder, 2017 McDonald criteria for the diagnosis of MS. Fatigue
with 5-day schedules of IVMP, matching the groups with
according to 2018 consensus. Seronegative NORSE were was measured by the Modified Fatigue Impact Scale
a propensity score for significant prognostic variables
compared to a cohort of pediatric anti-NMDAR, using (MFIS), with the patient being classified with fatigue at
in optic neuritis – age, baseline severity of disease, the
T-test and Chi square tests. Statistical significance was scores ? 38 and high-impact fatigue ? 59. The degree of
presence of anti-AQP4 antibody, the time from symp-
set at p<0,05. Results: We found 19 patients with NORSE, disability was expressed by the Expanded Disability Status
tom onset to IVMP, and the co-intervention with plasma
and 5 of them that had AIE antibodies identified were Scale (EDSS). Quality of life was quantified by the EQ-5D
exchange. Our primary outcome was the percentage of
excluded. The remaining 14 patients constituted the pedi- Quality of Life Questionnaire. Depression and anxiety
patients with incomplete recovery of the visual acuity at
atric NORSE cohort. There were no differences between were measured by the Hospital Anxiety and Depression
6-12 months, using the Rosembaum Chart. As sensitivity
groups regarding female gender (NORSE: 36% x NMDA: Scale (HAD). Results: 195 patients were included with a
analyses, we tested if the results were consistent using
56%, p=0.23) or age (NORSE: 6.64 ± 2.87 x NMDA: 5.62 ± mean age of 39.9 (±11.7), most female (71.8%). The clini-
an estimated LogMar scale and evaluating two different
3.27, p=0.3). Prodromal symptoms were more frequent in cal forms in the sample were relapsing-remitting (RRMS)
timepoints: the discharge and one month after symptom
NORSE (NORSE: 93% x NMDA: 52%, p=0.009), of which and primarily progressive (PPMS), with the majority
onset. Results: We screened 107 patients for eligibility.
the most common was fever (NORSE: 71% x NMDA: 37%, being RRMS (90.1%). The average MFIS in these patients
We found 24 patients that received 7-day schedule of
p=0.04). NORSE patients presented less frequently with was 44.8 (±21.1). Most patients (65.1%) achieved scores
IVMP and 11 patients that received 3-day schedule of
behavioral, psychiatric, and cognitive changes (NORSE: for presence of fatigue, of which 43.3% had high-impact
IVMP. After exclusion of patients due to missing of sig-
50% x NMDA: 96%, p=0.0005) and movement disorders fatigue. Patients with fatigue had worse quality of life [0.49
nificant data in confounding variables, we matched 22
(NORSE: 64% x NMDA: 93%, p=0.02), like orofacial dys- (±0.20) vs 0.80 (±0.17); p< 0.001], major functional disabil-
patients with 7-day with 22 patients with 5-day IVMP and
kinesia (NORSE: 29% x NMDA: 44%, p=0.035), dystonia ity [3.4 (±2.4) vs 1.4 (±1.9); p< 0.001], higher number of
11 patients with 3-day with 11 patients with 5-day IVMP
(NORSE: 0% x NMDA: 56%, p=0.0005) and chorea (NORSE: bouts [3.7 (±4.0) vs 2.4 (±1.8); p= 0.014], higher HAD scale
schedules. We found that the percentage of patients with
0% x NMDA: 37%, p=0.0009). NORSE patients also showed scores for anxious symptoms [9.3 (±4.6) vs 5.4 (±3.7); p<
incomplete visual acuity recovery, adjusted by prognostic
less frequent CSF pleocytosis, defined as >5cels/mm³ 0.001] and depressive symptoms [7.5 (±4.0) vs 4.0 (±3.1);
variables, was similar at 6-12 months when we compared
(NORSE: 21% x NMDA: 52%, p = 0.059), and normal brain p< 0.001]. We detected no difference related to length of
3-day versus 5-day and 7-day versus 5-day schedules. Our
MRI (NORSE: 21% x NMDA: 64%, p = 0.009). Conclusion: illness [10.2 (±7.5) vs 8.8 (±6.3); p= 0.201], and age [40.6
results remained in different scale (LogMAR) and time-
We found that NORSE patients have more frequently pro- (±11.2) vs 38.6 (±12.4); p= 0.263]. Conclusion: Fatigue is
points (at discharge and one month after symptom onset).
dromal symptoms. It’s possible that the high frequency a common symptom in MS patients and manifests itself
Conclusion: Shorter IVMP 1 g/day schedules appears to
of orofacial dyskinesia in NORSE patients might be result mainly in those with major disease severity and more
be as effective as longer schedules in the recovery of visual
of misinterpretation of focal seizures. Moreover, move- psychiatric symptoms. It is still a poorly measured con-
acuity in patients with optic neuritis. We should consider
ment disorders, behavioral and cognitive changes were dition in clinical practice, delaying access to available
3-day instead of 5-day schedules for mild cases and 5-day
more common in anti-NMDAR, indicating that cognitive treatments. For being a cross-sectional study, it cannot
instead of 7-day schedules for severe cases.
symptoms may be a clue in differentiating anti-NMDAR state the primary or secondary origin of fatigue. Therefore,
from seronegative NORSE. prospective studies are needed to clarify how fatigue and
Neuroimunologia comorbid conditions are truly related in these patients.

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COMPARATIVE ANALYSIS OF THE PROFILE CLINICAL AND EPIDEMIOLOGICAL PROFILE EPIDEMIOLOGICAL, CLINICAL AND
OF HOSPITALIZATIONS AND DEATHS FROM OF PATIENTS DIAGNOSED WITH MULTIPLE THERAPEUTIC PROFILE OF PROGRESSIVE
MULTIPLE SCLEROSIS IN THE PRE AND SCLEROSIS IN A SINGLE CENTER FORMS OF MULTIPLE SCLEROSIS FOLLOWED
INTRA PANDEMIC PERIOD IN BRAZIL UP AT A BASED REFERENCE CENTER IN THE
Luis Henrique Sunderhus de Oliveira, Rodrigo Leite
Marinho, Victória Pagani Samora Sousa, Arthur STATE OF CEARÁ
Victor Arthur Soares Costa Araújo, Erick Broder
Bichara, Daniel Abreu Santos, Trajano Aguiar Pires Prando de Barros, Guilherme Machado Vieira, João Iury Hélder Santos Dantas, Ivna Lacerda Pereira
Gonçalves Pedro Moraes Miossi, José Guilherme Martins Lima, Nóbrega, Igor Bessa Santiago, Lucas Silvestre
Gabriel Marim Roni, Bruno Batitucci Castrillo, Vera Mendes, Gabriela Joca Martins, José Artur Costa
EBMSP. Salvador BA, Brazil Lúcia Ferreira Vieira D’almeida, Milena Sales Pitombeira
Hospital das Clínicas da Faculdade de Medicina de
Ribeirão Preto. Ribeirão Preto SP, Brazil Universidade Federal do Espírito Santo – Vitória – Hospital Geral de Fortaleza. Fortaleza CE, Brazil
ES – Brazil
victorarthur9@hotmail.com iuryhelder@hotmail.com
sunluis01@gmail.com
Background: Multiple Sclerosis (MS) is the most preva- Background: Multiple Sclerosis (MS) is an immune-medi-
lent chronic inflammatory disease of the CNS, and those Background: Multiple Sclerosis (MS) is a Central Nervous ated, chronic inflammatory disease of the Central Nervous
with the disease require periodic specialized follow-up. System demyelinating disease with a varying clinical pre- System (CNS), characterized by multifocal areas of demy-
The COVID-19 pandemic has had an impact on this pop- sentation. Some studies have statistically validated an elination in the brain and spinal cord, with infiltration of
ulation on access to health care and the outcome of the epidemiological profile regarding prevalence between inflammatory cells, reactive gliosis and axonal degen-
disease. Objective: Comparatively describe the profile of genders, age of onset, initial symptoms and clinical clas- eration. There is a knowledge gap in understanding the
Hospitalizations and deaths related to MS in Brazil in the sification. These studies, however, applied older criteria disease, particularly the progressive forms in different
pre (Apr/2019-Jan/2020) and intra (Feb/2020-Nov/2020) for classification and diagnostic. There is also a common regions of the country, such as the Northeast. Objective:
pandemic period by COVID-19. Methods: Descriptive and clinical perception that MS is more frequent among white To report the profile of progressive MS at a reference center
retrospective study carried out from DataSUS between people. In this context, we investigated the epidemiological in Ceará. Methods: This observational and cross-sectional
Apr/19 and Nov/20. The variables analyzed were number profile of MS patients in a single reference center clinic study was conducted through a review of medical records
of Hospitalizations, age range, character of care, gender, of demyelinating diseases. Objectives: Comparatively and interviews at a Reference Center for Demyelinating
race/color, and deaths. Results: In pre-pandemic, with a evaluate the clinical and epidemiological profile of MS Diseases in the city of Fortaleza, Ceará. The population
total of 3,864 Hospitalizations, there was a peak in Jan/20 patients in a reference center clinic of demyelinating was composed of patients with the progressive forms of
(518) and the lowest value in Dec/19 (208). Of 1551 in diseases. Methods: Three articles published on Brain Multiple Sclerosis, registered in that center, whose diagno-
capital cities, 1074 occurred in São Paulo. 30-39 years were chosen for comparison. We reviewed the electronic ses meet the criteria of McDonald 2017. Results: From 283
old led with 1180 cases and 5-9 years was the lowest (4). records of patients seen or prescribed in this clinic at patients regularly followed with MS diagnosis according
There were 1872 urgent and 1992 elective admissions. least once within the last year (May 17th, 2021 – May to the 2017 McDonald criteria, 36 (12.7%) had progressive
There were 1286 male and 2578 female Hospitalizations 17th, 2022), assessing the following parameters: gender phenotype, 20 (56%) secondary progressive and 16 (44%)
(2.004: 1). By color/race, there were 2374 white and 895 – male or female; age of onset – years; skin color – white, primary progressive. From those 36 patients currently
brown people. There were 41 deaths in the country, 7 in black, brown, yellow or indigenous; initial symptoms – followed up, 25 (69,4%) were women, 19 (52,8%) were
capitals. The 50-59 age group led (13 deaths). 25 deaths isolated optic neuritis (ION), isolated brainstem/cerebel- Caucasian, 18 (50%) were high educated and the major-
occurred in men and 16 in women (1.56: 1). By color/race, lum dysfunction (IBCD), isolated long tracts dysfunction ity lived in Fortaleza 25 (69.4%). The peak incidence was
22 deaths were in whites and 12 in browns. In intra-pan- (ILTD) or combined dysfunction (CD); clinical classifi- in the third decade of life and the peak prevalence was
demic, with a total of 3105 Hospitalizations (19.64% less), cation (Lublin’s 2013 classification) – Clinically Isolated between the 4th and 5th decades. The median length of
there was a peak in Apr/20 (498). Of 1272 in capitals, 815 Syndrome (CIS), Relapsing-Remitting Multiple Sclerosis illness is 193 months. It is important noting that patients
occurred in São Paulo. 30-39 years old led with 947 cases (RRMS), Secondary Progressive Multiple Sclerosis (SPMS) are many with an undiagnosed disease for years. In turn,
and 5-9 years was the lowest (2). There were 1640 urgent or Primary Progressive Multiple Sclerosis (PPMS). We the follow-up time in a tertiary service, when most of them
admissions (12.39% less) and 1465 electives (26.45% excluded from the study cases which did not meet the had a closed diagnosis, is 61 months. Regarding disabil-
less). In Apr/20 (498 Hospitalizations), 350 were elective McDonald’s 2017 diagnostic criteria for MS, and those ity, 27.8% already used support in the first assessment
(70.29 %). On Sep/20, of the 190 Hospitalizations, 170 without defined initial symptoms. Cases were organized (EDSS 6.0), and in the last assessment, considering the
were urgent (89.47%). There were 1003 men and 2102 accordingly into Remitting Onset (RRMS and SPMS) or last EDSS recorded, the percentage of patients in need of
women notified (2.09: 1). By color/race, there were 1933 Progressive Onset (PPMS), as they were in the reference support almost doubled: 52.8%. Regarding the first dis-
white and 724 brown people. There were 21 deaths in the studies. All data was organized and individually compared ease-modifying drugs (DMD), there was a prevalence of
country (48.78% less), 5 in capital cities. 40-49 and 50-59 between the three articles before mentioned. Results: In interferons, considering that it was the most used class in
years old presented 5 deaths each. 8 deaths occurred in the RRMS subgroup (46 of 54 cases) we found a 2.83: 1 the 1990s. Considering the current DMD, there is a preva-
men and 13 in women (1.625: 1). By color/race, there were F/M Ratio; a 30.24 years mean age (11.85 SD; 1,748 SE); lence of monoclonal antibodies (47%), with Natalizumab
11 deaths in whites and 7 in browns. Conclusion: White a 26 years median age, and a comparable proportion of (25%) and Ocrelizumab (22%). In a comparative analysis,
women aged 30-39 form the population most affected by initial symptoms – 17.79% ION, 30.43% IBCD, 41.30% there is a drop in the use of interferons (8%). Conclusion:
MS in Brazil in the pre-pandemic period, while in deaths, ILTD, 10.87% CD. The comparison of other subgroups Understanding the epidemiological, clinical and thera-
white men aged 50-59. In the intra-pandemic period, was hindered by a lack of cases. The skin color of all cases peutic profile of MS patients with a progressive phenotype
there was a reduction in Hospitalizations, but the epide- pooled was: 31 brown (57,40%); 18 white (33,33%); 5 is essential to guide the propaedeutic reasoning, define
miologic profile regarding sex, race, and age remained black (9,26%). Conclusion: Despite the small case pool, conduct and restructure the dynamics of health services.
the same. In deaths, however, white women aged 40-59 our subpopulation matches the large cohorts’ epidemi-
were the most affected. ological profile, except for the skin color, which could be
explained due to brazilian miscegenation. Neuroimunologia

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AUTOIMMUNE ENCEPHALITIS: HOW TO THE ASSOCIATION OF DIFFERENT ACUTE CLINICAL SIGNIFICANCE OF THE CEREBRAL
RECOGNIZE? MANIFESTATIONS OF MS ON QUALITY OF LESION’S SIDE IN ISCHEMIC STROKE
LIFE AND FUNCTIONAL OUTCOME
Bibiana Mayer, Chadi Emil Adamo Milena Fernandes de Oliveira, Gabriel Souza Suzart,
Alex Cerqueira Silveira Figueiredo, Antônio Lázaro Paulo Atila Castro Carvalho de Jesus, Tayla Samanta
Universidade de Ribeirão Preto- UNAERP. Ribeirão Silva dos Santos, Rodrigo Almeida Magalhães
Santos Soares Júnior, Tayla Samanta Silva dos
Preto SP, Brazil Oliveira, Thais Barbosa de Oliveira, Maria Eduarda
Santos, Vinicius Leal Borges da Cruz, Silas Paulo
Universidade de São Paulo- Universidade de São Messias Vassoler, Vinicius Leal Borges da Cruz,
Lima de Souza, Gabriel Souza Suzart, João Paste
Paulo. Ribeirão Preto SP, Brazil Antonio Carlos Dias Andrade, Pedro Antonio Pereira
Silva, Leonardo Guimarães Melo, Thiago Gonçalves
bibiana_mayer@hotmail.com Fukuda, Pedro Antonio Pereira de Jesus de Jesus

Universidade Federal da Bahia. Salvador BA, Brazil HGRS. Salvador BA, Brazil
Autoimmune encephalitis (AS) comprises a group of
immune-mediated inflammatory diseases of the brain alexcsf@ufba.br milenafo@ufba.br
parenchyma, usually lasting less than 3 months. A mono-
phasic course is common in AS by surface antibodies; a Background: Multiple sclerosis (MS) is an inflammatory Background: Stroke is a cerebrovascular disease that can
progressive course can occur in paraneoplastic enceph- demyelinating disease that affects about 2.3 million people affect both hemispheres and cause functional dependence.
alitis – such as paraneoplastic cerebellar degeneration worldwide, with a direct impact on quality of life, causing The structural and functional symmetry of the right and
(anti-Yo) –, hence the importance of screening for neo- an important degree of disability. The first manifestations left sides of the brain is not absolute. Some differences in
plasms. The objective is to elucidate diagnostic forms of the disease can be subdivided into optic neuritis, with the two hemispheres have already been demonstrated.
and the importance of a detailed history and physical visual blurring or blindness; myelitis, with paresis, ple- The left side of the brain is more associated with the
examination in the management of these patients. After gia, hypoesthesia, and sphincter incontinence; trunk ??language, logic and problem solving, while the right
clinical suspicion, investigation with magnetic resonance syndrome, with ataxia and cranial nerve syndrome; and side is associated with emotions, empathy and visuo-
imaging (MRI) of the brain and analysis of cerebrospinal mixed syndrome, when symptoms from more than one spatial tasks. Furthermore, about 90% of individuals have
fluid (CSF) is usually carried out. It is known that the pres- group are present. Objective: The present study aims to the right hand as dominant, which is controlled by the
ence of bilateral limbic encephalitis is the only isolated analyze the relationship of the first clinical manifesta- contralateral motor cortex. Structural and physiological
MRI finding that is sufficient for a definitive diagnosis, tion on the quality of life and functional outcome of MS changes are involved in this differentiation, for example,
within the correct clinical context, even in the absence of patients. Methods: Cross-sectional study, conducted there is an increase in the length of the central sulcus in
measured antibodies. All other suggestive MRI patterns with patients diagnosed with MS according to McDonald the left hemisphere for right-handers and in the right for
(cortical/subcortical, striatal, diencephalic, brainstem, 2017 criteria and followed in a neuroimmunology refer- left-handers demonstrated in some studies. Objective:
encephalomyelitis, and meningoencephalitis) require ral service in Bahia from July 2021 to May 2022. Quality Evaluate possible changes in the functional outcome
antibody positivity. Furthermore, the electroencepha- of life was quantified based on the EQ-5D Quality of Life of right-handed patients who had an ischemic stroke
logram (EEG) provides evidence of focal or multifocal Questionnaire and functional outcome was rated by the in the right hemisphere compared to the left. Methods:
brain abnormality when MRI is negative. AS is the leading Extended Disability Status Scale (EDSS). Individuals were Prospective cohort with patients from the Stroke Intensive
cause of refractory de novo status epilepticus (NORSE) classified into 4 groups according to the first manifesta- Care Unit of Hospital Geral Roberto Santos. Patients were
and suggestive findings include focal deceleration/sei- tion of MS: optic neuritis, myelitis, trunk syndrome, and analyzed in relation to their handedness, a question asked
zures and periodic lateralized discharges (Delta Brush). mixed syndrome. Results: 212 patients participated in directly to the patient or family members. Right-handed
In addition, all patients with suspected AE require lumbar this study, of which 59(27.8%) were male and 153(72.2%) patients were kept for analysis, while left-handed patients
puncture (LP), unless contraindicated. In the analysis of were female. The mean EDSS was 2.62(±2.4) and the mean were excluded due to their reduced number. The affected
the CSF, usually performed after MRI, the presentation of EQ-5D 0.6 (±0.24). Regarding the first clinical manifes- hemisphere was collected from medical records. For the
an inflammatory character may be the only abnormality tation, we obtained: nine in optic neuritis (4.2%); thirty outcome, functional dependence (mRS>2) was evalu-
found, serving as an indication for empirical immunother- in myelitis (14.2%); thirteen in trunk syndrome (6.1%) ated using the modified Rankin scale performed 90 days
apy, after ruling out infection. Common findings include: and one hundred and sixty in mixed syndrome (75.5%). post-ictus. Patients with previous stroke and who died
mild-to-moderate lymphocytic pleocytosis (commonly The Kruskal-wallis test showed that there is an effect of were excluded from the analysis. Result: Of the 132 patients
20-200 cells, can be as high as 900 cells), hyperproteinorra- the different syndromic manifestations on the EDSS at analyzed, 69 (52.3%) were male with an average age of 58.1
quia, and sometimes an elevated IgG index. These findings, admission [X²(3) = 12.8; p<0.01] but not on the EQ5D (±40.5), those with stroke in the right hemisphere had a
in the setting of negative infectious and oncotic cytology score [X²(3)=7.7; p=0.052]. Pairwise comparison showed higher risk of functional dependence (42.3% vs 27.8%,
studies, support an immune-mediated etiology, but do differences between the median EDSS scores of the mixed RR=1.52). With the chi-square test, there was no statistical
not differentiate AS from other immune-mediated condi- syndrome and trunk syndrome groups [2(IQR 1-4) vs significance in the association of the affected hemisphere
tions, hence the need for clinical correlation. In addition, 0(IQR 0-1); p=0.03], but not between the other groups. and the functional outcome (p=0.088). Conclusion: The
CSF and serum antinuclear antibody (NAA) testing and Conclusion: The different acute MS conditions have sim- percentage of patients with functional dependence was
monitoring of the sodium level (showing hyponatremia ilar impact on the quality of life of patients, but interfere higher in patients with stroke in the right hemisphere, but
in certain subtypes, such as LGI-1 antibody AS) may differently in the functional capacity of these individuals. the p-value didn’t show statistical significance. Patients
increase sensitivity. Thus, it is observed that the wide This important divergence between the groups with trunk with ischemic stroke in the dominant hemisphere may be
diagnostic approach and early identification are bases syndrome and mixed syndrome can be interpreted by the more likely to develop functional difficulties. The dom-
for diligent treatment, maximizing the chances of full fact that the EDSS is a scale that refers to the functional inant hand is used for activities that require precision,
recovery of the patient. capacity, especially regarding mobility, therefore, the such as writing. Mild functional independence can lead
most affected are those with mixed syndrome and the to disabling deficits in patients pos-stroke.
least affected are those with symptoms related to the
Neuroimunologia trunk syndrome.
Neuroimunologia

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PREDICTION MODEL FOR THE DIFFERENTIAL REPORT OF MULTIPLE SCLEROSIS STUDY OF MULTIPLE SCLEROSIS BY
DIAGNOSIS OF MOG-IGG ASSOCIATED HOSPITALIZATIONS IN BRAZIL BETWEEN FOURIER-TRANSFORM INFRARED
DISEASE AND MULTIPLE SCLEROSIS JANUARY 2010 AND DECEMBER 2020 VIBRATIONAL SPECTROSCOPY TECHNIQUE
AT FIRST CENTRAL NERVOUS SYSTEM
Ana Rafaela Soares Vale, Fernanda Souza Gracílio Raí dos Santos Santiago, Marcia Helena Cassago
DEMYELINATING EPISODE IN PAEDIATRIC Silva, Isaac Rêgo Purificação, Henrique Nascimento Nascimento, Leonardo Barbosa Leal, Bruno
PATIENTS Dourado, Victoria Faustino Silva Reis, Lara Cordeiro Batitucci Castrillo, Paula Zago Melo Dias, Paulo
Magalhães, Hugo Oliveira Polito Barreto, Gabriel Roberto Figueiras, Wanderson Romão, Valerio
Bruna Klein da Costa, Rafael Canani Sommer,
Vianna Pereira Aragão Garrone Barauna, Lívia Carla de Melo Rodrigues
Fernanda Silveira de Quadros, Marlise de Castro
Ribeiro, Renata Barbosa Paolilo, José Albino da Paz, Escola Bahiana de Medicina e Saúde Pública. Universidade Federal do Espírito Santo – UFES –
Manuela de Oliveira Fragomeni, Vanessa Fragoso, Salvador BA, Brazil Vitória – ES – Brazil
Maria Lucia Brito, Douglas Kazutoshi Sato Hospital Universitário Cassiano Antonio Moraes –
anavale19.2@Bahiana.edu.br Vitória – ES – Brazil
Pontifícia Universidade Católica do RS
Santa Casa de Misericórdia de Porto Alegre Background: Multiple Sclerosis (MS) is the leading auto- raisantiaago@gmail.com
Hospital das Clínicas da Faculdade de Medicina da immune, inflammatory, demyelinating, chronic disease
Universidade de São Paulo of the central nervous system. MS affects about 2.3 mil- Background: Multiple Sclerosis (MS) is a chronic,
Hospital da Criança de Brasília José de Alencar. lion people worldwide and is the most common cause immune-mediated, inflammatory and degenerative dis-
Brasília DF, Brazil of nontraumatic disability in young adults. It can cause a ease characterized by demyelinating lesions of the central
Hospital da Restauração. Recife PE, Brazil progressive neurological dysfunction in this population, nervous system. The development of new Methods: that
Pontificia Universidade Católica do Rio Grande do leading to significant functional and economic damage. can extract biochemical information from biological fluids
Sul The disease presents itself through outbreaks, which lead and promote an effective and rapid diagnosis is of great
brunakleinc@gmail.com to frequent Hospitalizations and consequent import- importance to improve patient management and treat-
ant socioeconomic implications. Objective: Describe ment. The vibrational infrared spectroscopy technique
the epidemiological profile of Hospitalized patients for with Fourier transform (ATR-FTIR) has been approached
Background: Anti-myelin oligodendrocyte associated
MS. Methods: A descriptive epidemiologic study was as an alternative for the identification of biomarkers and
disease (MOGAD) is more frequent in pediatric patients
carried out with data collected from the Departamento diagnosis in several areas. The present study aims to dis-
than in adults. In international cohorts about 30% of pedi-
de Informática do Sistema Único de Saúde (DATASUS) tinguish individuals with MS from the control group by
atric patients with central nervous system inflammatory
system, in which the report of Hospital admissions for ATR-FTIR spectra through pattern recognition (PR). The
diseases have MOGAD. Since MOG-IgG testing is not yet
MS between January 2010 and December 2020 was eval- sample consisted of serum obtained from fifteen control
widely available worldwide, the identification of patients
uated. The Hospitalizations were analyzed by age group, subjects (mean age 35.0±13.7; 11 females) and fifteen sub-
that might benefit from testing at first presentation is
ethnicity, region, care scheme, gender, total value, and jects with relapsing-remitting MS (mean age 32.0±11.7;
recommended specially in low-income countries. Also,
number of days of stay. Patient mortality rate, deaths 10 females), from the Neurology outpatient clinic of the
the correct differential diagnosis of MOGAD and multi-
and the predominant age group were also described. The Cassiano Antonio Moraes University Hospital, following
ple sclerosis (MS) prevents unnecessary and potentially
average was calculated, along with the standard deviation the 2017 McDonald’s criteria. The EDSS score in subjects
harmful treatments. Objective: Our aim is to propose a
(SD) to describe the continuous variables and the rela- with MS ranged from 1 to 7 (mean 1.8±1.5) and the number
predictive score based on the clinical characteristics at
tive frequency for the categorical variables. Since this is of outbreaks from 1 – 8 (mean 3± 1.5). FTIR spectra were
first clinical attack for the differential diagnosis between
a public domain database, submission to the Research obtained from 10 uL pipetted onto an aluminum plate and
MOGAD and MS in pediatric patients. Methods: This
Ethics Committee was not necessary. Results: There dried for at least 2 hours in triplicate. The spectra were
is a nested case-control study of patients ?18 years of a
were 30,768 Hospitalizations. Most were white (58.26%), preprocessed and normalized to selected regions. For PR,
brazilian pediatric cohort of 6 reference centres in neuro-
women (69.7%), between 30 and 39y (29.6%) and in the Principal Component Analysis (PCA) and Unsupervised
immunology in 5 states. We selected patients that tested
southeast region of the country (64.98%). About the care Random Forest (URF) Methods: were used. Using the PCA
positive for MOG-IgG at the incidental inflammatory attack
regimen, 70.3% were ignored at Hospital admission; how- method, it was possible to observe a tendency to distin-
and those diagnosed with MS at the first year of follow-up
ever, there was no significant difference between public guish between the groups, but with a greater number of
in the study. The clinical characteristics at first presen-
and private. The total value of Hospitalizations in these combinations up to the main component 4 (total spec-
tation were evaluated identifying those more strongly
10 years was R$1,757,360.311 (SD: R$714,199.89), with trum 89.22%; high number of waves 98.68%; fingerprint
associated with MOGAD. Maximum likelihood or exact
the mean price per Hospitalization being R$686.19 (SD: 86.46% ). Then, the URF method was performed, where
logistic regressions were used to investigate associations
R$129.44). With the total number of deaths in this time it was possible to distinguish samples from the EM and
of clinical characteristics and the diagnosis of MOGAD
frame being 328 individuals, the year with the highest control groups both in the regions of high number of waves
or MS. The cut-off for the predictive score was estimated
incidence was 2019 (N: 51), with an average of 27.5 patient (48.35%), fingerprint (47.84%) and total spectral (62.86%)
based on the receiver operating characteristic (ROC) anal-
deaths/year. The average mortality rate is 1.14% with a total with 3 main components. Combinations of regions (high
ysis. Results: We found that younger age at presentation
of 0.92% over 10 years. The predominant age group in the number of waves and fingerprint) by the URF method
(1 point), male sex (1 point), bilateral optic neuritis (1
deaths was 50-59 years (19.51%). Conclusion: The data were performed, with a distinction between the samples
point) and multifocal presentation with encephalopathy
of patients Hospitalized for MS match with the epidemi- (50.42%). Conclusion: The results showed a distinction
or isolated optic neuritis (1 point) were associated with
ology for the disease itself. Over the years, the number of between the two groups, which suggests the presence of
MOGAD and contributed for the composite score. Having ?
Hospitalizations remained high, making MS a high-cost structures in the data capable of biologically distinguish
2 points in our proposed clinical composite score has 80%
disease, both because it affects an active population between MS and the control group. This indicates a high
sensitivity (95% CI, 0.56-0.93) and 66% specificity (95%
and because of the total costs of Hospitalizations due to potential of the ATR-FTIR technique associated with
CI, 0.35-0.89) for the diagnosis of MOGAD. Conclusion:
its progressive characteristics and frequent outbreaks. machine learning methods for the study and diagnosis
Combined clinical and demographic characteristics at
of MS. Ethical approval: (CAAE n.44387521.8.0000.5071).
first attack might be used to guide serologic testing for
MOG-IgG and help to differentiate pediatric MS from Neuroimunologia
MOGAD. The score might support treatment decisions Neuroimunologia
and optimize the use of health resources.

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CORRELATION BETWEEN THE DEGREE OF NEUROLOGICAL COMPLICATIONS POST- THERAPEUTIC INERTIA AND FUNCTIONAL
DISABILITY AND DEPRESSIVE AND ANXIOUS VACCINATION AGAINST SARS-COV-2: A CASE DISABILITY IN MULTIPLE SCLEROSIS
SYMPTOMS IN MULTIPLE SCLEROSIS SERIES FROM NORTHEASTERN BRAZIL
Antonio Lazaro Santos Soares Junior, Alex de
Vinícius Leal Borges da Cruz, Antônio Lazaro Santos Renata Santos Almeida, Cristiane Campello Bresani Cerqueira Silveira Figueiredo, Tayla Samanta Silva
Soares Júnior, Alex de Cerqueira Silveira Figueiredo, Salvi, Lara de Menezes Andrade, Marcelo Ataíde de dos Santos, Silas Paulo Lima de Souza, João Paste
Tayla Samanta Silva dos Santos, Pedro Antonio Lima, Eduardo R. Pessoa de Aquino, Jurandy Júnior Silva, Gabriel Souza Suzart, Vinicius Leal Borges
Pereira de Jesus Ferraz de Magalhães, Clarice Neuenschwander Lins da Cruz, Maria Eduarda Messias Vassoler, Pedro
de Morais Fonseca, Ailton de Souza Silva, Victor de Antonio Pereira de Jesus, Thiago Gonçalves Fukuda
Universidade Federal da Bahia. Salvador BA, Brazil Menezes Carvalho Filho, Maria Lúcia Brito Ferreira
Universidade Federal da Bahia. Salvador BA, Brazil
cruz.vinicius@ufba.br Instituto Aggeu Magalhães/Fiocruz-PE. Recife PE,
antoniolssj@ufba.br
Brazil
Background: Multiple Sclerosis (MS) is a chronic multi- Hospital da Restauração. Recife PE, Brazil
factorial disease characterized by inflammation, demy- Background: Multiple sclerosis (MS) is an autoim-
LACEN-PE. Recife PE, Brazil
elination and gliosis mainly in the white matter of the mune demyelinating neurological disease that develops
Universidade Católica de Pernambuco. Recife PE,
Central Nervous System (CNS). Among these individuals, a chronic degenerative and inflammatory condition. After
Brazil
depression and anxiety are quite common disorders, the the onset of symptoms, it can take time before diagnosis
former condition affecting up to 54% of patients, and can laradma@hotmail.com and initiation of treatment. This therapeutic inertia can
be caused and aggravated by several factors. Objective: To have deleterious impacts on the individual’s functional
evaluate the association between the degree of disability Background: The SARS-CoV-2 virus caused the coronavi- capacity. Objective: To analyze the association between
and depressive and anxious symptoms in patients with rus disease pandemic in mid-2020 with millions of deaths, therapeutic inertia time and functional disability in MS.
MS. Method: Cross-sectional, single-center study con- which led to an urgency in the development of vaccines Methods: Cross-sectional, single-center study conducted
ducted with patients diagnosed with multiple sclerosis against the virus. Neurological complications resulting with patients diagnosed with multiple sclerosis according
according to the 2017 McDonald criteria. The degree from vaccination against SARS-CoV-2 are increasingly to the 2017 McDonald criteria. The time of therapeutic
of disability was assessed using the Expanded Kurtzke evident worldwide and require further clinical investi- inertia was calculated based on the dates of symptom
Disability Status Scale (EDSS). Depressive and anxious gation. Vaccination-related neurological manifestations onset and start of pharmacological treatment for MS. The
symptoms, on the other hand, were assessed using the are rare but already reported for other vaccines, such as degree of disability was expressed according to the EDSS.
Hospital Anxiety and Depression Scale (HAD), which Guillain-Barré syndrome after influenza vaccination. The sequelae were grouped into syndromes, namely optic
assesses the preexistence of anxiety and depression sep- Objective: To describe the clinical presentation of six- neuritis syndrome, transverse myelitis syndrome, brain-
arately through 14 questions. The score for each modality teen cases of patients exhibiting neurological manifes- stem syndrome, mixed syndrome, and other symptoms.
ranges from 0-21, where: 0-7 unlikely, 8-11 possible, 12-21 tation after SARS-CoV-2 vaccination. Methods: This is a Results: 187 patients were admitted, of whom 20 (10.7%)
likely. Results: We evaluated 196 patients, of which 70.5% case series report nested within the brazilian multicenter were diagnosed with primarily progressive multiple scle-
were diagnosed as unlikely, 16.7% as possible and 12.9% case-control NeuroCOVID study (Fiocruz-PE). Sixteen rosis (PPMS) and 154 (82.4%) with relapsing remitting
as probable for depression. And for anxiety 50.5% had adult patients with neurological manifestations referred multiple sclerosis (RRMS). The mean time to therapeutic
unlikely diagnosis, 31.5% possible diagnosis and 18.6% to Hospital da Restauração (Recife, Pernambuco, Brazil) inertia was 31.96 (SD±45.56) months, with 110 (58.8%)
probable diagnosis. The mean EDSS among the partici- in 2021 were studied. Physical examination, imaging test patients with inertia less than 32 months and 56 (29.9%)
pants was 2.60(+- 2.39). Kruskal-wallis test was performed (when necessary), and laboratory tests were performed. patients with inertia greater than or equal to 32 months.
between the groups categorized by HAD scale previously All the patients (or legal representatives) signed the study The median EDSS score was 2 (IQR 1-4). Regarding the
mentioned and there was difference of median EDSS consent form. Results: Most of the patients were of the presence of sequelae: 1 (0.5%) was classified with optic
between depression groups[X²(3) = 9.58; p<0.01] and female gender (10/16) and the median age was 63.5 years neuritis syndrome, 10 (5.3%) with transverse myelitis syn-
Pairwise comparison showed the difference was found (mean ± SD = 58.25 ± 21.03 years). Of the sixteen patients, drome, 2 (1.1%) with brainstem syndrome, 154 (82.4%)
between Improbable-Probable groups[p adjusted<0.05]. eleven patients were vaccinated with an adenoviral vec- with mixed syndrome and 13 (7%) with other symptoms.
Whereas among anxious symptoms, no difference in tor-based vaccine encoding the spike glycoprotein of 7 (3.7%) patients had no sequelae. Time of therapeutic
degree of disability was found between the groups [X²(3) SARS CoV-2 (ChAdOx1 nCov-19, Oxford/AstraZeneca/ inertia greater than or equal to 32 months was associ-
= 1.23; p>0.05] Conclusion: Through the results of the Fiocruz), and six individuals received the inactivated ated with higher EDSS scores [median 4 (IQR 1-6) vs 1.75
study, it is possible to conclude that there is a difference virus-based vaccine (Sinovac/Coronavac/Butantan). (IQR 0-3.5); p=0.001] and these more often had EMPP
in the degree of disability between patients with a proba- The delirium-associated encephalopathy was the major (x²=11.402, 70.6% vs 29.5%, p=0.001). Conclusion: Greater
ble diagnosis of depression and patients with an unlikely neurological manifestation among the patients (4/16), therapeutic inertia was associated with more impaired
diagnosis. However, it is not possible to state that there followed by optic neuritis (3/16), and headache/migraine functional capacity and a clinical form with worse dis-
is any difference in the degree of disability regarding the (2/16). Other neurologic complications were benign par- ease progression. Thus, possible factors that delay the
anxious symptoms of patients with multiple sclerosis. oxysmal vertigo, ischemic stroke, encephalitis, Guillain- initiation of pharmacological therapy should be inves-
Barré syndrome, myasthenia gravis, sixth nerve neuritis, tigated in clinical practice, considering their impacts on
and transverse myelitis. The interval between the vaccine the natural history of the disease. Prospective studies
Neuroimunologia dose and the onset of the symptoms varied from zero to can better explore this relationship and the possibilities
ninety-five days. These cases had no other cause associ- of intervention on therapeutic inertia in MS patients.
ated with the neurological diagnosis and were reported to
the government surveillance system. Moreover, our study
team is part of the Expert Committee on Adverse Events Neuroimunologia
of the COVID-19 vaccine of the Pernambuco State Health
Department. The notification and evaluation of these
cases are of great importance for a better understanding
of the clinical picture and management of the patients.
Financial Support: Fiocruz-PE.

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ADVERSE REACTIONS TO COVID-19 THE PRESENCE OF DEPRESSIVE AND/OR PREVALENCE OF DEPRESSION IN PATIENTS
VACCINES IN PATIENTS WITH ANXIOUS SYMPTOMS IS CORRELATED WITH WITH RELAPSING REMITTING OR PRIMARY
DEMYELINATING DISEASES FATIGUE AND ITS INTENSITY IN PATIENTS PROGRESSIVE MULTIPLE SCLEROSIS
WITH MULTIPLE SCLEROSIS
Jairo Aragão Perez, Antonio Marcelo Moreira, Milena Fernandes de Oliveira, Tayla Samanta Silva
Fernando de Paiva Melo Neto, Luis Felipe Dantas Vinicius Leal Borges da Cruz, silas paulo lima de dos Santos, Paulo Atila Castro Carvalho de Jesus,
da Nóbrega Machado, Milton Cabral de Melo Pinto, souza, juliana de alencar fontes, Maria Eduarda Maria Eduarda Messias Vassoler, Silas Paulo Lima
Nivaldo Alves Calado Neto, Davi Veloso Guerra, Messias Vassoler, Antônio Lazaro Santos Soares de Souza, Juliana de Alencar Fontes, Vinicius Leal
Bianca Etelvina dos Santos de Oliveira Júnior, Alex de Cerqueira Silveira Figueiredo, Tayla Borges da Cruz, Thais Barbosa de Oliveira, Thiago
Samanta Silva dos Santos, Gabriel Souza Suzart, Gonçalves Fukuda, Pedro Antonio Pereira de Jesus
UNIPÊ. João Pessoa PB, Brazil
Thaís Barbosa de Oliveira, Pedro Antonio Pereira de
Centro Universitário de João Pessoa – UNIPÊ. João HUPES. Salvador BA, Brazil
Jesus
Pessoa PB, Brazil
Fundação Centro Integrado de Apoio ao Portador de milenafo@ufba.br
Universidade Federal da Bahia. Salvador BA, Brazil
Deficiência – FUNAD. João Pessoa PB, Brazil
cruz.vinicius@ufba.br Background: Multiple sclerosis is a demyelinating auto-
jairoaragao2000@hotmail.com immune disease of the central nervous system (CNS). May
Background: Multiple sclerosis (MS) is an inflammatory cause visual, motor, cognitive and dysautonomic symp-
Background: Vaccine efficacy is heterogeneous and can disease that affects the central nervous system and is the toms. The main clinical presentations are relapsing-re-
be modified by multiple external factors; it is noteworthy leading cause of disability in young people in developed mitting (RRMS) or primary-progressive (PPMS). Due to
that, although vaccines have demonstrated high efficacy or countries. MS significantly affects the quality of life of the debilitating symptoms and the unpredictability of new
effectiveness in preventing Hospitalizations and deaths, all patients and has great importance for public health. outbreaks, in addition to the neurological dysfunctions
of them have some adverse event. Objectives: To analyze Further, the pathophysiology is complex and the clini- associated with the condition, MS patients are suscepti-
the occurrence of adverse reactions to COVID-19 vaccines cal presentation is heterogeneous, where fatigue is the ble to depression, which can compromise the individual’s
in patients with demyelinating diseases. Methods: A retro- most prevalent symptom among patients with the dis- quality of life and prognosis. Objective: To compare the
spective, cross-sectional study carried out by reviewing the ease, being significantly experienced in up to 92% of sick prevalence of depression in patients with RRMS or PPMS.
medical records of 115 patients, selected in a non-proba- patients. Worldwide researchers have already tried to Methods: Cross-sectional study, single-center, conducted
bilistic manner and for convenience, with demyelinating investigate the impact of fatigue on the quality of life of with multiple sclerosis patients at the Magalhães Neto
diseases at the Multiple Sclerosis Reference Center of those patients, however, especially in Brazil, there’s still a ambulatory in Salvador from august 2021 to may 2022.
the State of Paraíba, using the variables sex, age, diag- gap. Objective: To evaluate impact of fatigue in depressive Patients were asked about their clinical history, whether
nosis, COVID-19 vaccination, and adverse reactions after and anxiety symptoms.in MS patients. Methods: A pro- they had a diagnosis of depression were also measured
COVID-19 vaccination. Results: Among the 115 medical spective study was conducted with consecutive patients by the Hospital Anxiety and Depression Scale (HADS).
records analyzed, there was a predominance of females who met McDonalds diagnostic criteria. Clinical and Results: Of the 178 patients that were enrolled, 89.3% (n
(70.43%), in addition, a distribution of 101 (87.82%) epidemiological characteristics were described. Fatigue – 159) had RRMS, 10.6% (n – 19) had PPMS. Of the 159
patients with Multiple Sclerosis (MS) and 14 (12.12%) was measured with Modified Fatigue Impact Scale (MFIS) RRMS patients, 22,6% (n – 36) had depression, 76,7%
with Neuromyelitis Optica (NMO). In categorization by and depressive and anxiety symptoms with the Hospital (n – 122) did not. Of the 19 PPMS patients, 47,3% (n – 9)
age group, 3% belonged to the age group under 16 years Anxiety and Depression Scale (HADS). Patients were sep- had depression, 52.6% (n – 10) did not. The HADS was
old, 9% from 16 to 25 years old, 34% from 26 to 35 years arated in six categories: absence of fatigue (MFIS score applied in 105 RRMS patients. 74.2% (n – 78) had a normal
old, 31% from 36 to 45 years old; 10% from 46 to 55 years 0-38) and fatigue clinically significant (MFIS score 39-84), value of 0-7, 8.6% (n – 9) had a borderline value of 8-10
old; 10% from 56 to 65 years old and 3% from those over 65 probable depression (HAD-depression 12-21), improba- and 17.14% (n – 18) had an abnormal value of 11-16. The
years old. Regarding the vaccination of COVID-19, 100% ble depression (HAD-depression 0-12), probable anxiety HADS was applied in 15 PPMS patients. 60% (n – 9) had
performed the first dose, 99% the second dose and 74% (HAD-anxiety 12-21) and improbable anxiety (HAD- a normal value, 20% (n – 3) had a borderline value and
the third dose. Regarding vaccine reactions, reactions anxiety 0-12). The descriptive analysis was performed 20% (n – 3) had an abnormal value. Conclusion: Patients
were reported in 49% of patients, with 36% reporting through clinical information, their frequencies, means with PPMS had a higher prevalence of depression and
body pain, 30% pain at the injection site, 16% fever, 16% and standard deviations. The inferential analysis was per- abnormal values ??on the HADS compared to patients
headache, 11% chills, 9% worsening of MS symptoms, 5% formed using Pearson’s chi-square test and its respective with RRMS. The patient with PPMS presents progressive
fatigue, 4% nausea, 2% vomiting and diarrhea. Among odds ratio. Results: 204 patients were included in study. worsening, with the accumulation of neurological deficits
the patients who had vaccine reactions, 11% had NMO The frequency among men was 58 (28.4%) and women over time, in addition, there are few medications avail-
and 89% had MS. Conclusion: The effectiveness of vac- was 145 (71.1%). The mean of age was 39,78 ± 11,89 years. able for these patients, leading to a higher occurrence of
cination varies according to the vaccine applied, and The Pearson Chi-square test demonstrated that there is an depression in this group.
may present vaccine reactions, despite being extremely association between the presence of depressive symptoms
effective in preventing deaths resulting from infection by among patients with clinically significant fatigue [X²(1)
SARS-CoV-2. We can observe that no patient had serious 13,494; p<0,001] with 10,010 of odds ratio (CI 95%: 2,3- Neuroimunologia
adverse reactions. Thus, vaccination against Sars-COV 2 43,47) and also anxiety symptoms [X²(1) 11,803; p<0,001]
is extremely important, since the benefits will be greater with 4,546 of odds ratio (CI 95% 1,81-11,41). Conclusion:
than the harm, as observed in the study. the study concludes that there is a correlation between
the presence of clinically significant fatigue and the pres-
ence of important depressive and anxious symptoms in
Neuroimunologia patients with MS. In addition, it was noticed that patients
with significant fatigue are 10,01 times more likely to have
major depressive symptoms and 4,54 times more likely
to have major anxiety symptoms.

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ACUTE TRANSVERSE MYELITIS ASSOCIATED REGION IN THE AMAZON HAS HIGH RATES NEGLECTED TROPICAL DISEASES AND
WITH VACCINATION AGAINST COVID-19: A OF HIDDEN PREVALENCE OF LEPROSY AND THEIR NEUROLOGICAL MANIFESTATIONS:
SYSTEMATIC REVIEW OF THE LITERATURE HIGH CASES WITH DISABILITIES IMPORTANT DIFFERENTIAL DIAGNOSES FOR
NEUROLOGY
Paulyane Thalita Miranda Gomes, Raquel Vassão Leandro Araújo Costa, Márcio Alex Reis Câmara,
Araujo, Paulo Pereira Christo, Antonio Pereira Izaura Maria Vieira Cayres Vallinoto, Claudio Anna Laura Lima Larcipretti, Eduarda de Pellegrin,
Gomes Neto Joaquim Borba Pinheiro Fernando Cotrim Gomes, Breno José Alencar Pires
Barbosa
Santa Casade Belo Horizonte. Belo Horizonte MG, Universidade Federal do Pará. Belém PA, Brazil
Brazil Instituto Federal do Pará. Belém PA, Brazil Universidade Federal de Ouro Preto – Ouro Preto –
MG – Brazil
paulyanetmg@gmail.com leandrocosta.dr@gmail.com
Universidade Luterana do Brazil – Canoas – RS –
Brazil
Background: Longitudinallyextensive transverse myelitis Background: The method to estimate the hidden preva-
Universidade Federal de Minas Gerais. Belo
(LETM) is a rare complication of different vaccines. Due lence of leprosy, was proposed by Gil Suáres and Lombardi,
Horizonte MG, Brazil
to the pandemic,several COVID-19 vaccines have been in 1997, and is used and suggested by the Pan American
Universidade Federal de Pernambuco. Recife PE,
used worldwide. Among the reported side effects,LETM Health Organization (PAHO) and used by the WHO (2016),
Brazil
deserves to be highlighted, being a condition with high it is based on the percentage of cases with some incapac-
morbidity.Objective: To conduct a systematic review of the ity in the diagnosis (in relation to those evaluated), and anna.larcipretti@aluno.ufop.edu.br
literature on transverse myelitis associated with vaccina- applied to the total of new cases in the last 05 years of the
tion against Covid-19.Method: A systematic review of the research. At the end, the hidden prevalence is added to the Background: Neglected tropical diseases (NTDs) consist
literature was carried out, that included articles published new cases and the estimated real prevalence is obtained. of a group of 20 infectious diseases caused by bacteria,
between March 2021 and January 2022. Initially, 86 arti- Objectives: Evaluate the hidden prevalence of leprosy in helminths, protozoa, and viruses, affecting more than one
cles were found. After applying the inclusion and exclu- the 11th Health Region of Pará, Brazil, between 2010 and billion people worldwide, mostly in the context of social
sion criteria, we encountered 21 publications. Results: 2020. Method: This is a cross-sectional and retrospective disparities. Many of these pathologies cause systemic
There were a total of 23 cases, with the highest number in study, with data from the information system of notifica- repercussions, including a wide variety of neurological
Germany, followed by Japan. The remaining cases were tion disputes in Brazil. The data were organized in work- manifestations. Objective: To investigate the frequency
reported in Italy, United States, China, United Kingdom, sheet and analyzed according to the methods proposed and types of neurological involvement among NTDs in
Malaysia, Iran, Brazil, Portugal, Turkey, India. The vaccines by Gil Suáres and Lombardi (1997), and recommended Brazil from January 2012 to December 2021 according
involved were: AZD1222AstraZeneca, ModernRNA-1273, by the World Health Organization and the Pan-American to government databases. Methods: This is an epidemi-
the BNT162b2 vaccine,BBIBP-CorV andAd 26.COV2.S, Health Organization. Results: The 11th Health Region of ological, descriptive, and retrospective study. Data were
Johnson and Johnson.34.7% of the cases occurred in Pará was classified in 2010 as a hyperendemic area, with a extracted from the SUS Hospital Information System (SIH/
female patients and 47.8% in male Hypertension and detection rate of 90/100.000 inhabitants. Over the years, SUS). In addition, a review of the literature on the neuro-
diabetes were the most commonly present. The interval this rate has decreased, and in 2020 it was 29/100.000 logical manifestations of NTDs was performed. Results:
between vaccination and the LETM ranged from 24 hours inhabitants, where much of this decrease may be related to The study revealed 507,469 Hospitalizations by NTDs in
to three weeks. The most common radiological findings the Covid-19 pandemic. However, even with a significant Brazil between 2012 and 2021, with the Northeast and
on MRI were: extensive involvement of the cervical cord decrease in cases (p<0.05), the region is classified in the Southeast regions being the most affected. The number
up to the thoracic spine, involvement of the cervical and parameter of very high edemicity, with high rates of cases of deaths in this period was 5,232, with higher burden
thoracic cord separately.Among the alterations found in in children under 15 years old (11.3%) and some degree among adults and those living in the Southeast region. The
the CSF, mild hyperproteinorraquia up to above 500mg/ of disability (27%). Regarding occult leprosy, 792 cases most relevant NTDs in the brazilian context were Dengue,
dL with unaltered cellularity were reported. Among the were not diagnosed in the study region, thus suggesting Leprosy, Visceral Leishmaniasis, Helminthiasis, and
clinical manifestations, tetraparesis, paraparesis or para- an occult prevalence of 8.19/10,000 inhabitants. In other Trypanosomiasis. Neurological manifestations of varying
plegia were reported, with or without paresthesia, sensory words, it can be said that 23% of the cases were not diag- degrees of severity and pattern of involvement have been
level and urinary sphincter dysfunction. The majority of nosed and, consequently, went untreated, maintaining described, including damage to the peripheral nervous
patients received pulse therapy with methylprednisolone the active cycle of leprosy in the region. Conclusion: It is system (plexopathy, Guillain-Barré syndrome, polyneu-
1g/day, from three to five consecutive days. In 26% of concluded that the 11th Health Region of Pará has a high ropathies, etc.) and central nervous system (encepha-
cases, there was additional therapy with plasmapheresis. rate of leprosy in children under 15 years of age, and in lopathy, meningoencephalitis, stroke, epilepsy, etc.) The
In one case, the patient spontaneously improved. In five this region it is a very disabling disease with many hidden frequencies of the manifestations were not described in
cases reported there was complete remission of symp- cases, keeping the transmission cycle active, resulting in the SIH/SUS database. These findings are in accordance
toms, in three cases the clinical outcome after treatment a region with difficult to control leprosy. with our review of the literature, which emphasizes han-
was not reported. And in the remaining cases there was seniasis as an important cause of peripheral neuropathy
partial improvement. In only one case, the patient died. in low and middle income countries, as well as neurocys-
Conclusion: Vaccines against COVID-19 have a significant Neuroinfecção ticercosis as a significant cause of epilepsy in south-east-
impact on morbidity and mortality, being indispensable ern countries like Brazil. Conclusion: The neurological
in the fight against the pandemic. Post-vaccination LETM forms of NTDs contribute substantially to their morbidity
is a rare event, but early recognition is important as treat- and mortality, with heterogeneous manifestations being
ment can reduce long-term disability. reported in the present survey. The frequencies of the
findings found were not described, which points to the
need for more research in the area and for improvements
Neuroimunologia in the NTDs notification systems.

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XXX Congresso Brasileiro de Neurologia

TL 1105449 TL 1105510 TL 1105530

HOSPITALIZATION FOR MENINGITIS IN THE DETAILED CLINICAL EVALUATION SERUM LEVELS OF NEUROFILAMENT
NORTHEAST REGION OF BRAZIL IN THE PRE- IN ASYMPTOMATIC SEROPOSITIVE LIGHT CHAIN, GLIAL FIBRILLARY ACIDIC
PANDEMIC AND PANDEMIC PERIOD (2018- HOUSEHOLD CONTACTS OF PATIENTS PROTEIN, UBIQUITIN CARBOXY-TERMINAL
2021): AN ECOLOGICAL TIME SERIES STUDY DIAGNOSED WITH LEPROSY – THE HYDROLASE L1 AND TAU PROTEIN IN
IMPORTANCE OF THE NEUROLOGIST IN COVID-19 INFECTED PATIENTS DURING THE
Mariana Carmo Freitas, Júlia Dantas de Deus,
Thiago Barbosa Vivas EARLY DIAGNOSIS PANDEMIC FIRST WAVE: EARLY NERVOUS
SYSTEM INVOLVEMENT AND SEVERE
UNIME. Lauro de Freitas BA, Brazil João Paulo Moreira Fernandes, Thales Junqueira
Oliveira, Laura de Lourdes Cardoso e Silva, Glauber OUTCOME
mariana.hfreitas@outlook.com Mota Pacheco, Mateus Barros Bueno, João Augusto Elisa Gouvea Gutman, Helena França Alcaraz,
Nunes Vitorino, Iago Resende Carvalho, Agnes Laura Andreza Salvio Lemos, Jessica Vasquez Raposo-
Background: The meningitis refers to an inflammatory Silva Neres, Isabela Maria Bernardes Goulart, Diogo Vedovi, Renan Amphilophilo Fernandes, Larissa
process in the meninges. It can be caused by an infec- Fernandes dos Santos Araujo Duarte, Carlos Henrique Ferreira Ramos,
tious etiology, whose pathological agents are bacteria, Carlos Henrique Ferreira Ramos Filho, Luciane
viruses and fungi; as well as it can be a non-infectious Universidade Federal de Uberlândia. Uberlândia
MG, Brazil Almeida Amado-Leon, Soniza Vieira Alves-Leon
etiology, being triggered by trauma, drugs and other
mechanisms. The meningitis has the highest morbid- João paulomoreira2012@gmail.com Universidade Federal do Estado do Rio de Janeiro.
ity and mortality rates among central nervous system Rio de Janeiro RJ, Brazil
infections, and is therefore considered a severe pub- Background: The long incubation period of leprosy, its Universidade Federal do Rio de Janeiro. Rio de
lic health problem. Objective: To analyze the variation insidious signs and symptoms produce difficulties in its Janeiro RJ, Brazil
in the number of Hospitalization for meningitis in the diagnosis and correct clinical classification. The early rec- Fundação Oswaldo Cruz. Rio de Janeiro RJ, Brazil
Northeast of Brazil during the pre-pandemic (2018-2019) ognition of neural involvement in leprosy, especially in gutman811@gmail.com
and pandemic period (2020-2021). Methods: This is an household contacts with subclinical infection, in which
ecological time-series study that estimated the number the classic clinical and laboratory findings of the disease SARS-CoV-2 infection can lead to highly heterogeneous
of Hospitalizations and Hospital detection rate of men- are, by definition, absent, represents a major challenge in clinical outcomes, ranging from no or mild symptoms to
ingitis using data from the SUS’s Hospital Information clinical practice. Objective: To characterize the clinical, severe cases with need for life support in intensive care
System (SIA/SUS). The data were obtained from the molecular, serological and neurophysiological aspects in units (ICU) and death risk. The search for predictors able
Tabnet Platform (DATASUS), being analyzed according the early diagnosis of leprosy neuropathy in household to forecast disease severity at patient admission to direct
to the variables age, sex, color/race, comparing those contacts with subclinical infection (positive anti-PGL1 clinical choices would be beneficial to set priorities and
findings in the pre-pandemic and pandemic period. ELISA serology). Method: 361 asymptomatic seropositive optimize interventions. Central nervous system bio-
Results: 2,756 Hospitalizations were registered in this household contacts (SPHC), defined as subclinical infec- markers might reflect early multi-organ involvement of
period, with a decrease of 0.2 in incidence per 10,000 tion, followed up in a national reference center for leprosy Coronavirus Disease 19 (COVID-19), typical of patients
inhabitants, between 2020 and 2021. Hospitalizations in Brazil, from 2016 to 2020, were recruited. All individ- with more severe disease evolution. Neurofilament light
for meningitis due to parasitic infections represented uals underwent a clinical, laboratory and neurophysio- chain (NfL) is a protein of central and peripheral neu-
0.6% of all causes, showing a 21% drop in the number of logical evaluation. Results: 361 SPHC were evaluated, rons validated as a nervous system damage biomarker
Hospitalizations corresponding to this etiology in the years with a mean age of 35.7 years and female predominance in a variety of neurological diseases. Glial fibrillary acidic
2020 to 2021. Other causes of meningitis were responsi- (66.2%; 239/361). The qPCR analysis was positive in 35.5% protein (GFAP) is highly expressed in astrocytes and
ble for 114 Hospitalizations, which 46% occurred in the (128/361) in dermal scrapings and in 25.8% (85/361) in increasingly used as a serum biomarker of astrocytic acti-
pandemic period. Men, people under 70 years of age and skin biopsy of SPHC. In electroneuromyographic eval- vation/injury. Ubiquitin carboxy-terminal hydrolase L1
people of mixed race represented the largest number of uation, 23.5% (93/361) of SPHC showed signs of neural (UCH-L1) is a neuronal specific protein frequently used
Hospitalizations, corresponding to 56.09% (1,546), 96.84% involvement, with an average of 2.1 compromised nerves for indicating brain damage induced by viral infections.
(2,669) and 58.27% (1,606), respectively. Conclusion: per SPHC. 62.3% (53/93) showed a mononeuropathy pat- Tau protein is mostly expressed by neurons, implicated
The lower number of Hospitalizations for meningitis, tern on electroneuromyography (ENMG). The group of in the pathogenesis of brain damage, especially in neu-
may have been a reflection of the underreporting that SPHC with altered ENMG had a higher qPCR positivity rodegenerative diseases. This study aims to investigate
occurred during the years 2020 and 2021 as a result of the of dermal scrapings (47.0%; 40/85) when compared to whether NfL, GFAP, UCH-L1 and Tau protein provide
attention paid to cases of Covid-19. Besides, the viral form the group with normal ENMG (31.9%; 88/276) (p=0.01), non-redundant prognostic value to COVID-19 severity.
of meningitis is the most prevalent in Brazil. Therefore, conferring a greater chance of neural involvement (OR, So, the objective of this study is to investigate these bio-
infections by SARS-Cov-2 could justify the large number 1.8; p=0.02). Conclusion: The annual monitoring of SPHC markers (NfL, GFAP, UCH-L1 and Tau) in the plasma of
of Hospitalizations in the pandemic period. The lower showed a considerable prevalence of peripheral nerve patients with severe and mild COVID-19. An observa-
rates of meningitis due to parasitic infection may have impairment assessed by ENMG, favoring the early initia- tional study with 106 participants, comprising 73 patients
been a consequence of the unrestricted use of antipara- tion of treatment. It is essential the role of neurologists in with COVID-19 admitted to intensive care unit (ICU), 9
sitic drugs for prophylactic purposes of Covid-19 during the control protocols of this endemic, favoring the break- COVID-19 patients with mild symptoms and 24 healthy
the last two years analyzed. Considering the importance ing of the transmission chain of the disease. controls. Blood samples were taken from all participants
of meningitis, the results obtained in the present analy-
and Single molecule array (SIMOA) was used to analyze
sis denote the need for more research to investigate and
NfL, GFAP, UCH-L1, and total Tau. The ICU participants
deepen the associations between the different variables Neuroinfecção
were collected at the moment of the admission in the
of the condition.
ICU and the COVID-19 outpatients at the beginning of
symptoms. Graph prism was used to compare results of
Neuroinfecção the ICU group, outpatients and healthy controls, in each
protein. In our findings, NfL, GFAP, UCH-L1, and total tau
levels were significantly increased in patients with severe
or critical COVID-19 compared to outpatients (p<0.0001)
and controls (p <0.0001), independently of nervous sys-
tem involvement. No relevant difference was observed
between outpatients and control groups. These results
indicate the early involvement of the nervous system
measured by serum neurodegeneration biomarkers as an
important risk factor in prognosis of COVID-19 outcome.

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XXX Congresso Brasileiro de Neurologia

TL 1105532 TL 1105583 TL 1105631

FREQUÊNCIA DE NEUROCISTICERCOSE IMPACT OF COVID-19 ON VIRAL NEUROLOGIC MANIFESTATIONS IN


EM DOENTES ADMITIDOS NO HOSPITAL ENCEPHALITIS CASES IN BRAZIL PATIENTS WITH ACUTE CHAGAS DISEASE
AMÉRICO BOAVIDA DE 2012-2014 TREATED WITH BENZNIDAZOLE
Rebeca Lima de Almeida Santos, Erica Otoni Pereira
Nadjeda Madjessy Morais Leite Ferreira, Martinho Miranda, Giovana Andrade de Oliveira, Lukas Santos Maria Júnia Lira e Silva, Filipe Prohaska Batista,
Luemba, Job Monteiro Chilembo Jama-António Freire, Guilherme Rocha Cardoso, Mariana Oliveira Demetrius Montenegro, Ana Rosa Melo Correa Lima,
Salamargo, Gabriel Meira Xavier, Victor Sousa Eduardo Sousa de Melo
Faculdade de Medicina UAN – Angola Campos, Lucas Silva Santos, Philip George Glass
Faculdade de Medicina da Universidade Agostinho Andrade Universidade de Pernambuco, Hospital Universitário
Neto – Angola Oswaldo Cruz. Recife PE, Brazil
UESB. Vitória da Conquista BA, Brazil Universidade Federal de Pernambuco, Unidade
jobmonteiro2011@hotmail.com FASA. Vitória da Conquista BA, Brazil de Neurologia e Neurocirurgia, Departamento de
Neuropsiquiatria. Recife PE, Brazil
Background: Neurocysticercosis (NCC) is a worldwide rebeca_lima23@outlook.com
public health problem, being the most common par- lirajunia@gmail.com
asitic infection of the Central Nervous System (CNS), Background: At the imminence of the COVID-19 pan-
caused by the larval form of Taenia solium, the cysticer- demic, it was found that the pathogen has an infectious Background: Chagas disease (CD) is a serious public
cus cellulosae. It is the most frequent cause of epilepsy potential of neuroinvasion and neurotropism, resulting in health problem and more than 70% of transmission in
worldwide and has a lethality rate ranging from 16.4% high rates of morbidity and mortality. In this context, its Brazil occurs by consumption of foods contaminated.
to 25.9% in regions considered endemic, which include association with neurological conditions emerges, such Neurologic manifestations in the acute phase are rare,
countries in Southern Africa, Asia, South and Central as viral encephalitis, whose manifestation is mediated with a predominance of headache. Common side effects
America, mainly affecting rural communities in devel- by a diffuse inflammatory process and affects mainly the of Benznidazole include headache and peripheral neu-
oping countries. Objective: To describe the Frequency of extremes of age and immunosuppressed patients. In addi- ropathy. Given the infrequency of neurologic findings
Neurocysticercosis in patients admitted to the Neurology tion, the etiology also covers enteroviruses, arboviruses, associated with acute CD, it is important to differentiate
Service of Hospital Américo Boavida from 2012 to 2014. herpesviruses and adenovirus. Objective: To verify if between manifestations associated with the disease itself
Methods: Retrospective descriptive study, the sample there was an increase in the diagnosis of viral encephalitis or the imidazole treatment. Objective: Describe the neu-
selection was by the non-probabilistic method for con- during the pandemic. Method: This is an analytical obser- rologic manifestations in patients with acute CD treated
venience, where all the Clinical Processes of patients vational ecological study based on data on viral enceph- with Benznidazole. Method: Cross-sectional observational
admitted to the Neurology service of Hospital Américo alitis obtained from the Hospital Morbidity System (SIH/ study with 32 patients treated at a referral Hospital for
Boavida in the period from 2012 to 2014, of both sexes, SUS) of DATASUS. The period analyzed was from March infectious and parasitic diseases in the city of Recife-PE
with aged over 18 years who were accompanied by com- 2018 to February 2022, using the following variables: Year/ between May and July 2019 during an outbreak in the
puted tomography of the skull. Results: 923 clinical files month of processing, age group 2 and Hospitalizations. backlands of the state. Previous, post-infection and post-
were analyzed, of which 31 (3.3%) presented calcifica- Microsoft Excel version 2204 was also used to analyze the use antiparasitic neurologic symptoms were evaluated,
tions suggestive of NCC. Of these (51.6%) were female. results, using graphs. Results: During the study period, such as headache, tremor, drowsiness, dyskinesia, syn-
The age of the patients ranged from 25 to 85 years, with 7754 cases of Hospitalization were recorded, of which cope, or pre-syncope and sensory complaints. The project
a predominance of the age group from 66 to 75 years 4578 occurred in the period before the pandemic and was approved by the local Ethics Committee under No.
(25.8%); (74.2%) of the patients were single, and most 3176 during the pandemic, showing a reduction of 18%. 2,107,217. Results: Mean age was 30.7 years (range: 9-56
of them lived in the municipality of Rangel (25.8%), fol- In this context, there was a prevalence in the age group years), 50% female. From a personal history, 15 patients
lowed by the municipality of Cacuaco (22.6%). Regarding of 0-9 years, with 1581 and 1124 cases in the pre- and (46.8%) had a headache, predominantly migraine (60%),
the number of calcifications in the tomographic findings, post-pandemic period, respectively. Thus, it is inferred 6 (18.7%) drowsiness, 3 (3.3%) postural tremor in upper
most had 1-2 calcifications (46.7%). Headache (32,3%) that the observed results contradict what was expected, or lower limbs and 2 (6.2%) paresthesia. After infection,
and epileptic seizures (25,8%) were the most frequent as they portray a reduction in cases. Such a reduction 26 (81.2%) had a new headache, mostly with a migraine
symptoms. As for the form and stage of the cysts, (96.8%) may be related to the adoption of biosecurity measures pattern (76.9%). With the introduction of the antiparasitic
of the patients had the cysts in the calcification stage and to mitigate the contagion and prevention of COVID-19, drug at a dose of 200-300 mg/day, 7 patients (21.8%) had a
only one (3.2%) in the active stage. Conclusion: In this also acting to reduce the spread of other viruses that can new headache with a migraine pattern in 5 cases (71.4%)
study, the frequency of Neurocysticercosis is 3.3%, which cause the pathology. In addition, broad-spectrum vacci- and 17 (53.1%) had tremor in their majority of postural
we can consider high. The female gender was more repre- nation for SARS-CoV-2, which began in January 2021 in character (82.3%) and may affect upper and lower limbs
sentative; The Municipalities of Rangel and Cacuaco had Brazil, and other immunization campaigns have a direct and head. Different sensory complaints were also reported:
higher numbers of cases of NCC; Headaches and seizures impact on reducing viral encephalitis. Conclusion: It was 7 cases of hypoesthesia (21.8%), 3 of pain (9.3%) and 7
were the most frequent symptoms. found that there was a reduction in the number of cases of paresthesia (21.8%), all symptoms predominantly in
of viral encephalitis during the COVID-19 pandemic in the upper limbs. A 10-year-old child, after 300 mg/day
Brazil. This was possibly due to biosecurity measures and of Benznidazole, developed dyskinesia on the seventh
Neuroinfecção widespread vaccination. In addition, age is an important day of treatment. Dose reduction to 150 mg/day led to
determinant in the diagnostic profile of viral encephalitis full recovery. Conclusion: Acute CD is infrequent and
in the country. However, the lack of data on Coronavirus neurologic manifestations may be a consequence of the
viral encephalitis and the possibility of underdiagnosis infection itself or treatment. Headache and tremor were
and underreporting were limiting. In this regard, there is the predominant findings, the latter being associated
a lack of studies at the national level that better charac- with Benznidazole use.
terize the relationship of the virus as a potential agent of
the pathology in question.
Neuroinfecção

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310
XXX Congresso Brasileiro de Neurologia

TL 1105697 TL 1105698 TL 1105719

ENCEPHALITIS ASSOCIATED WITH NEUROLOGICAL MANIFESTATIONS OF LONG- EFFICACY OF THE PHOTOBIOMODULATION


PARVOVIRUS B19 INFECTION: A CASE TERM COVID-19 IN PATIENTS WITH SARS- THERAPY ON RECOVERY OF OLFACTORY
PRESENTATION COV-2 INFECTION: DESCRIPTIVE STUDY AND GUSTATORY DYSFUNCTION IN
PATIENTS WITH LONG-COVID-19. A SERIE OF
Catarina Ester Gomes Menezes, Ester Costa Lima, Mariana Beiral Hammerle, Deborah Santos Sales,
Paula Cely da Silva Torres, Marcos Baruch Portela Patrícia Gomes Pinheiro, Rayanne da Silva Souza, CASES
Filho, Lilliane Rodrigues Soares, Daniele Santos Karina Lebeis Pires, Ana Carolina Frinhane Herzog, Deborah Santos Sales, Mariana Beiral Hammerle,
Andrade, Lécio Aragão Souza Morais, Jefferson Daniel Lucas de Lima Silva Santos, Débora Viana Patrícia Gomes Pinheiro, Daniel Lucas de Lima Silva
Heber Marques Fontes Júnior, Alice Santos Freitas, Elisa Gutman Gouvea, Claudia Cristina Santos, Ana Carolina Frinhane Herzog, Clarissa
Mascarenhas, Mateus Santana do Rosário Ferreira Vasconcelos de Araujo Davico, Rayanne da Silva Souza, Débora
Universidade do Estado da Bahia. Salvador BA, Universidade Federal do Estado do Rio de Janeiro. Viana Freitas, Karina Lebeis Pires, Cláudia Cristina
Brazil Rio de Janeiro RJ, Brazil Ferreira Vasconcelos
União Metropolitana de Educação e Cultura UNIME Universidade Federal do Estado do Rio de Janeiro.
marianabeiral@gmail.com
catarina.gomes.menezes@gmail.com Rio de Janeiro RJ, Brazil
Background: The clinical scenery of COVID-19 was ini- deborahsales.fono@gmail.com
Case Presentation: A 62-year-old female patient was tially described as a respiratory infection with possible
admitted to the emergency department with epigastric severe and systemic manifestations. Studies point to Background: Smell and taste disorders are frequent symp-
pain, hypoactivity, fever, chills, nausea, arthralgia and chronic complications that affect neurological system of toms during acute COVID-19 and may persist long after the
decreased consciousness for 2 days. Two days later, she patients infected with the virus. Complaints may appear resolution of the initial phase. Many studies have reported
evolved with headache, vomiting, decreased level of con- after the period of acute infection, with persistent and persistence of these symptoms more than 6 months after
sciousness and tonic-clonic seizure. Petechial rash, pap- prolonged neurological manifestations being perceived. the onset, with female patients more likely to report per-
ules, livedo reticularis, hyperemia and jaundice were also Objectives: To investigate, in a population sample from sistent symptoms. Photobiomodulation Therapy (PBMT)
present. Head MRI and EEG were normal. Cerebrospinal the State of RJ, the frequency of different types of neu- represents a new and emerging therapeutic modality for
fluid (CSF) analysis revealed xanthochromia, hyperpro- rological manifestations after COVID-19 infection and the treatment of olfactory impairment after COVID-19,
teinorrachia (98 mg/dL), normal glucose (66 mg/dL), 21 their medium and long-term outcomes.Methods: Cross- as it is able to modulate inflammatory processes and
cells/m3 (69% neutrophils; 10% macrophages). Diagnosis sectional descriptive study carried out in an outpatient improve tissue healing in general. Objectives: Report a
of meningoencephalitis was made. Empirical treatment clinic for post-COVID-19 neurological manifestations series of cases using PBMT for the management of COVID-
with ceftriaxone, ampicillin and acyclovir was initiated. of an university Hospital in the city of Rio de Janeiro. A 19-related olfactory and taste impairment. Methods: Six
Serologic tests for Zika, Dengue, CHIKV, HIV, VDRL and total of 233 patients were analyzed from September 2020 females (average: 46,6 years ± 13,7) and one male patient
hepatitis were negative. Acute renal failure, septic shock, to April 2022 using socio demographic questionnaires to were seen at the post-COVID-19 Outpatient Clinic of a
leukocytosis (27.350), thrombocytopenia developed rap- collect clinical-epidemiological data, including gender, University Hospital in Rio de Janeiro, Brazil, from march
idly, and the patient was transferred to the ICU. She woke age, comorbidities, time of onset of symptoms, most to December 2021. COVID-19 infection was confirmed by
up 12 days later, with delirium, diffuse weakness and mild prevalent complaints in the acute phase, Hospitalization, biomolecular assay (RTPCR) and all patients had anosmia
right VI and VII cranial nerve palsy. New CSF was collected complications and possible neurological manifestations and ageusia since infection. Data about the exact initi-
which remained xanthochromic and showed hyperpro- of long-term COVID-19 in patients with SARS COV-2 ation and duration of all signs and symptoms related to
teinorrachia (138 mg/dL), glucose of 83 mg/dL and 23 infection.Results: Among the 233 patients evaluated, COVID-19 was collected. Olfactory and gustatory func-
cells/m3 (77% lymphocytes; 18% macrophages). XGEN 182 were women and 51 were men. In addition, the mean tion was evaluated separately using visual analog scales
MULTI N9 PCR was performed on CSF for Varicella zos- age was 33.97 years (RI: 18 to 72) and 45.92% of them had (VAS) ranging from 0 (normal smell/taste) to 10 (com-
ter, HSV 1, 2, 6 and 7, CMV, EBV, enterovirus, adenovirus, comorbidities, the most prevalent being obesity (20.06%), plete anosmia/ ageusia). Scale was applied at the first
parechovirus and parvovirus (PVB19) and only PVB19 followed by systemic arterial hypertension (18.45% ) and clinical appointment (baseline) and after the end of the
was positive. EBV was detectable (296 IU/mL) by PCR in diabetes mellitus (10.42%). The mean time between symp- treatment. Two questions related to quality of life were
serum. Six days later, the CSF showed reduced cellularity tom onset and assessment was 7.45 months (RI: 1 to 25). applied before and after treatment. An intranasal PBMT
(4 cells/m3) and proteinorrachia (92 mg/dL). There was The main symptoms in the acute phase of the disease protocol was applied using Laser DUO® (MM Optics Ltda
improvement on neurological examination with nor- were, respectively, fatigue (79.39%), anosmia (68.66%), São Carlos, SP, Brazil) at 660 nm, on contact mode, with
mal cognition and mild generalized weakness. She was headache (67.38%) and ageusia (64.37%). In addition, 100 mW of power, and 8J of energy in each nasal mucosa
then referred for physical rehabilitation. After 2 months 93.13% did not need Hospitalization. It is noteworthy that and 6J in ten points of the tongue. A total of sixteen laser
she returned to clinical office with normal neurological 78.54% of the sample remained with some symptoms in sessions, twice a week and with a 48-hour interval were
examination. Conclusion: The most frequent clinical pre- the chronic phase of the disease, namely chronic fatigue done. The paired t test was used to compare the pre-
sentation of PVB19 infection is erythema infectiosum, a (43.20%), cognitive deficit (42.06%), anosmia/hyposmia and post-treatment VAS means. Results: All patients
self-limited disease which is very common in children. (38.19%), ageusia /hypogeusia (33.04%) the most relevant. completed the protocol (16 sessions). On average, the
Encephalitis due to PVB19 infection is uncommon and Conclusion: The prevalence of the neurological condi- olfactory and gustatory VAS scale scores after treatment
should be considered in cases of encephalitis whose main tion in the acute phase of the disease was remarkable in (media =3,66, standard error =0,55; media =8,09, stand
etiologies have been ruled out. When available, multi- the results presented. It is also worth mentioning that the error = 0,85 respectively) was lower than the pre-treatment
plex-PCR should be performed, as it allows for a faster main symptoms throughout the period after infection by score (media =8,00, stand error = 0,85; media =3,50, stand
and wider etiological diagnosis. the virus were predominantly neurological, with the most error = 0,42 respectively), t (5)= 6,50, p < 0,01; t (5) = 5,89,
frequent being fatigue, anosmia, ageusia and cognitive p<0,01. Patients also reported an improvement in quality
deficit. Thus, it is evident the need for more studies that of life after treatment. Conclusion: Given the lack of infor-
Neuroinfecção can improve the biopsychosocial impact that these late mation about the available treatments for olfactory and
manifestations can have on the patient’s life. gustatory dysfunctions in patients with long-term covid,
PMB appears to be a promising therapeutic modality.
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NEUROPSYCHIATRIC DISORDERS OF PREVALENCE OF SPEECH-LANGUAGE CAUSE AND EFFECT RELATIONSHIP


COVID-19 AFTER MILD SARS-COV-2 SYMPTOMS RESULTING FROM COVID-19 BETWEEN PARKINSON’S DISESE AND
INFECTION OVER TIME IN A 2-YEAR CROSS-SECTIONAL COVID-19
STUDY IN RIO DE JANEIRO
Mariana Beiral Hammerle, Deborah Santos Sales, Beatriz Barrozo Gonzalez Oliveira, Bruna Gonçalves
Patricia Gomes Pinheiro, Karina Lebeis Pires, Deborah Santos Sales, Mariana Beiral Hammerle, Dantas de Almeida, Maria Isabella Farias de Araújo,
Clarissa de Araujo Davico, Rayanne da Silva Souza, Patrícia Gomes Pinheiro Gomes Pinheiro, Ana Ana Clara Mota Gonçalo, Ana Beatriz Matos de
Daniel Lucas de Lima Silva Santos, Elisa Gutman Carolina Frinhane Herzog, Rayanne da Silva Souza, Souza, Vitoria de Menezes Sá Lazera, Júlia Leite
Gouvea, Carolina Garcia Nuñez Carrijo, Claudia Pedro Ignacio Ferraz Montenegro de Almeida, Xavier Bertrand
Cristina Ferreira Vasconcelos Nathalie Souza de Andrade, Elisa Gutman Gouvea,
CEUMA – São Luís – MA – Brazil
Karina Lebeis Pires, Cláudia Cristina Ferreira
Universidade Federal do Estado do Rio de Janeiro.
Vasconcelos bbgo2000@yahoo.com.br
Rio de Janeiro RJ, Brazil
Universidade Federal do Estado do Rio de Janeiro.
marianabeiral@gmail.com Background: Parkinson`s disease (PD) is one of the
Rio de Janeiro RJ, Brazil
most common neurodegenerative disorders, affecting
UNIRO. Rio de Janeiro RJ, Brazil
Background: COVID-19, a disease caused by the SARS- dopaminergic neurons in the susbtantia nigra. Patients
CoV-2 virus, started in December 2019 in Wuhan, China. deborahsales.fono@gmail.com can present motor and neuropsychiatric complications,
It is a disease that affects multiple systems, including besides sleep disorders and sensory abnormalities. The
neuropsychiatric manifestations such as anxiety, depres- Background: The emergence of the disease known as Braak hypothesis suggest that PD may originate from an
sion, fatigue and sleep disorders. These disorders may COVID-19, caused by the virus SARS-CoV-2, was reported external pathogen, whom would enter the body through
persist after the acute phase, leading to impairment of in China in 2019. It spreads rapidly and devastatingly the nasal cavity or the vagal nerve at the gut. This would
cognition and life quality. Objectives: To analyze the around the world, infecting people in mild, moderate and help link the neurological complications that followed
prevalence of persistent neuropsychiatric manifestations severe ways. It is known that the virus uses the receptors COVID-19, mainly the worsening of symptoms of PD
(fatigue, depression and anxiety) after primary COVID-19 of the enzyme angiotensin 2 (ACE2), which is present in and your appearance in patients who never had it before.
infection. Methods: This is a descriptive cross-sectional various organs of the human body (lungs, brain, kidneys, Objective: This work includes evidence of the relationship
study carried out in a cohort of 146 patients who had heart), as an entry into cells, allows us to understand between apparition of PD and the worsening of its symp-
mild COVID-19 infection. Patients were seen at the Post- the diversity of symptoms of this infection. Although toms after COVID-19. Methods: This study consists of a
COVID-19 Outpatient Clinic of a university Hospital in Rio the main symptoms of this respiratory syndrome are literature review of articles in English and Portuguese in
de Janeiro, Brazil, from September 2020 to May 2022. Tests cough, fever, dyspnea, which are marked factors for the PUBMED and GOOGLE SCHOLAR databases, published
were performed to screen for neuropsychiatric symptoms, severity of the condition, other speech and language between 2020 and 2022 with the following descriptors:
including the fatigue severity scale (FSS), Hospital Anxiety symptoms can occur, such as: dysphonia, dysphagia, Parkinson`s disease, COVID-19. Results: The studies
and Depression Scale (HADS), in addition to surveys to hypoacusis and chemosensory changes over time, as analysed showed that close to 34% of COVID-19 survi-
collect socio demographic data. The HADS classifies markers of symptoms of COVID-19. Objectives: Identify vors received a neurological or psychiatric diagnosis in
patients into unlikely, possible and probable diagnoses the prevalence of speech-language symptoms resulting the six months that followed their infection. This occurs
of depression and/or anxiety. Results: Tests performed from COVID-19 infection over time. Methods: This is a because COVID-19 may have a neurodegenerative func-
an average of 6.95 (RI: 1 – 24) months after COVID-19 2-year-long cross-sectional, quantitative and descriptive tion, due to cytokine storm, triggering an autoimmune
infection. The patients average age was 22 (RI: 19 – 71). study. Patients with CoVID-19 infection by PCR, rapid reaction, brain damage and making it more priming to
75.34% of patients were female. 47.26% of patients have test or serology, of both genders, living in the State of Rio other neurotoxic processes. This also happened in the
previous comorbidities. 54.79% of patients reported pres- de Janeiro, selected by convenience, participated in the Spanish-flu pandemic, in which there was a wave of new
ence of subjective cognitive deficit after COVID-19 infec- study. To determine the prevalence of speech-language cases of encephalitis lethargica, while in the COVID-19
tion. Per the evaluation of the FSS, 71.91% of the patients symptoms, the questionnaire was designed by the author pandemic, non-motor symptoms in PD patients were
presented fatigue after infection by COVID-19. Among and applied electronically through Google Forms. It is exacerbated or make them appear as an early state of PD
the evaluated patients, 19.86% had probable depression, composed of 10 open and closed questions, classified in other patients. The two main attack zones of COVID-
with the same value for possible depression. 30.13% of the according to the presence of the symptom and its duration, 19 are the nasal cavity and the gut. In the first one, the
patients had probable anxiety, while 20.54% had possible focusing on the evaluation of the symptomatologic report. virus induces neuroinflamtion of the olfactory bulb and
anxiety. Conclusion: The scales used showed that patients Statistical analysis was performed using SPSS 22.0 version. reduces neural stem cells, causing anosmia. Meanwhile
with mild COVID-19 infection had frequent complaints Results: A total of 403 patients were included in the study. in the second, the patient may present alteration in the
of chronic fatigue, anxiety and depression. In addition, Median age was 37 (interquartile range (IQR) 19-77) years; microbiota, causing dysbiosis, dysphasia and constipa-
more than half of the patients reported some component 328 patients (81,4%) were female. Olfactory and Gustatory tion. This, as said in the Braak hypothesis, is similar to
of subjective cognitive deficit, which may be related to Dysfunction were the most frequently reported, followed the clinical presentation of PD, which can explain the
the referred alterations. Further investigations and eval- by vocal and cognition impairment (80,9%, 73,7%, 28,0% link between this two, seeing the COVID-19 as a trigger
uations are needed regarding the origin of the reported and 23,8% respectively). The median duration of ageusia to future complications and it it’s not being explored in
complaints, since the period of confinement and social and anosmia were 58 (IQR 1-695) and 76 (IQR 1-695) days, enough studies to make it relevant. Conclusion: It is of
isolation can contribute to the emergence of neuropsy- respectively. Median duration of cognitive impairment was utmost importance to bring this group to the fore, pro-
chiatric complaints evidenced in the study. 173 (IQR 5-820) days. Standing out 18,4, 14,1% and 11,7% ducing more evidence of the correlation between PD
of the sample that kept Gustatory, Olfactory and Cognitive and COVID-19 by monitoring patients with neurological
Dysfunction for more than 3 months. Conclusion: The diseases after. This will allow the medical community to
Neuroinfecção prevalence of speech-language symptoms resulting from prepare itself for the upcoming of new cases creating a
COVID-19 infection is high, highlighting the changes in proper conduct and treatment method.
smell and taste with varied durability. More studies are
needed, especially regarding the impact of these changes
on the quality of life of patients. Neuroinfecção

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COVID-19 NEUROLOGICAL MANIFESTATIONS MOLECULAR MIMICRY AS ONE OF THE NEUROLOGICAL AFFECTS IN THE PEDIATRIC
IN PATIENTS WITH INFLAMMATORY BOWEL POSSIBLE MECHANISMS ASSOCIATED WITH POPULATION INFECTED BY SARS-COV-2 IN
DISEASE TREATED IN A TERTIARY HOSPITAL PHENOTYPES OF DEMYELINIZING EVENTS MARANHÃO
IN CEARÁ IN PATIENTS INFECTED WITH ZIKA VIRUS
Ana Paula Costa Linhares, Victória de Menezes Sá
Isabelle Carvalho Gonçalves, Guilherme Nobre Laíse Carolina França, Fabrícia Lima Fontes- Lazera, Kaline dos Santos Kishishita Castro, Bianca
Nogueira, João Pedro Barros Façanha, Pedro Dantas, Renan Amphilophio Fernandes, Andreza de Melo Ferro, Melissa dos Santos Costa, Karla
Robson Sousa Vieira, Lilian Kriger Ramos de Salvio Lemos, Jéssica Vasques Raposo-Vedovi, Vanessa Morais Lima, Fabricio Silva Pessoa, Juliana
Carvalho, Lucas Eduardo Lucena Cardoso, Osvaldo José Moreira do Nascimento, Fernanda Lago de Araújo, Eliza Maria da Costa Brito Lacerda
Marcellus Henrique Loiola Ponte de Souza, Lúcia Cristina Rueda Lopes, Alice Laschuk Herlinger,
UNICEUMA. São Luís MA, Brazil
Libanez Bessa Campelo Braga, Antônio Miguel Renato Santana de Aguiar, Soniza Vieira Alves-Leon
Furtado Leitão, Francisco de Assis Aquino Gondim viclazera@hotmail.com
Universidade Federal Fluminense. Rio de Janeiro
Universidade Federal do Ceará. Fortaleza CE, Brazil RJ, Brazil
Background: In 2019, the SARS-CoV-2 coronavirus was
Universidade Estadual do Rio de Janeiro. Rio de
isabellecarvalho1@gmail.com identified. It is important to conduct studies reporting
Janeiro RJ, Brazil
the behavior of this virus in Maranhão in the pediatric
Universidade Federal do Estado do Rio de Janeiro.
Background: COVID-19 has been frequently associated population since the epidemiological profile may be dif-
Rio de Janeiro RJ, Brazil
with a wide range of neurological disorders. However, it ferent from the rest of the world. Objective: To delineate
is unknown whether COVID-19 exacerbates neurological andrezaslemos@gmail.com the clinical epidemiological profile of neonates, children
symptoms in patients with Inflammatory Bowel Disease and adolescents infected with SARS-CoV-2 in the state of
(IBD). Objective: To identify possible worsening of gas- Evidences observed during the Zika Vírus (ZikV) epidemic Maranhão. Methods: This research was approved with
trointestinal and neurological manifestations in patients in Brazil indicate a strong link between the Zika infection CEP 4.315.245. It constitutes a cross-sectional observa-
with IBD due to COVID-19 infection. Methods: Research and neurological complications. Acute myelitis, optic neu- tional study from March 2020 to August 2021. Secondary
was carried out in 146 patients from the gastroenterology ritis, polyneuropathy and encephalomyelitis that mimic data from neonates, children and adolescents affected by
outpatient clinic, from June 2020 to June 2022, in a tertiary inflammatory idiopathic demyelination disorders (IIDD) SARS-CoV-2 in Maranhão were used. The analysis was
Hospital in Ceará. Evaluation was conducted in person or of the Central Nervous System (CNS) after ZikV infection performed using descriptive statistics. 260 data were
by phone and addressed, among other aspects, whether have been reported. The present study aims to investigate collected, 99 with incomplete information were excluded
the patient was infected by COVID-19, if there was worsen- the possible occurrence of molecular mimicry between and 161 were selected for analysis. Results: Regarding
ing of IBD symptoms and if there was neurological mani- ZikV antigens and Multiple Sclerosis (MS) autoantigens, neurological involvement, 82.61% (n=133) did not present
festation. Results: Among 146 patients interviewed during the most frequent IIDD of the CNS. A retrospective cohort and 17.39% (n=28) expressed some of the analyzed types.
a 2 year period (28 in 2020; 36 in 2021; 82 in 2022), 78 had study with 305 patients admitted due to suspected arbo- Neurological involvement was considered as the presence
Crohn’s disease and 68 had ulcerative colitis. Of this total, virus infection in 3 university Hospitals in Rio de Janeiro of at least one: sign and symptom, neuropathy, neuroim-
36 patients reported COVID-19 infection with positive test was performed. All patients were submitted to neurolog- aging, treatment, neurological coinfection. Regarding
confirmation, comprising about 24% of the sample, with ical examination and biological sample was collected for neurological signs and symptoms, 87.58% (n=141) did not
16.6% of the patients responding that there was a wors- serologic and molecular diagnostic by a multidisciplinary and 12.42% (n=20) did. Among the signs and symptoms,
ening of IBD symptoms during COVID. Regarding neu- team. Bioinformatics tools were used to analyze the pep- 4.35% (n=7) each presented with seizure and decreased
rological symptoms, 72.2% of patients reported headache tides shared between ZikV antigens and MS autoantigens level of consciousness. In addition, 3.11% (n=5) presented
during COVID infection. In comparison, among patients and their conformation. Of 305 patients, twenty-six were with headache, 1.86% (n=3) with encephalitis and motor
who didn’t have COVID with positive test confirmation, positive for ZikV and 4 presented IDD pattern found in loss, respectively. 1.24% (n=2) presented anosmia, hypo-
about 57% of patients reported headache.Other neuro- MS cases. Sequence homology comparisons by bioinfor- tonia and stiffening of the limbs, each. Also, 0.62% (n=1)
logical symptoms reported by patients affected by COVID matics approach between NS5 ZikV and PLP MS protein presented hyponosmia, decreased reflexes, dysgeusia,
were anosmia (47.2%), ageusia (47.2%), tingling (22.2%), revealed a homology of 5/6 consecutive amino acids and arreflexia, respectively. No patient presented age-
numbness (19.4%) and disorientation (5.5%). Surprisingly, (CSSVPV/CSAVPV) with 83% identity. Analysis of the 3D usia, hypogeusia and neuralgia. Regarding neuropathy
no patient reported syncopal spell during COVID-19 structures revealed a similar conformation with alpha (comorbidity), 93.79% (n=151) did not and 6.21% (n=10)
infection. Conclusion: The results indicate that 83.3 % of helix presentation in a perfect align, which can deduce did. Regarding the type of neuropathy, 2.48% (n=4) had
patients who claim to have COVID had at least one of the a molecular mimicry. Molecular mimicry between NS5 encephalopathy and epilepsy, respectively, 1.86% (n=3)
aforementioned neurological symptoms. Furthermore, Zika virus antigen and PLP MS autoantigens emerge as cerebral palsy, 1.24% (n=2) hydrocephalus, and 0.62%
the prevalence of neurological manifestations of these a possible mechanism for IDD spectrum in genetically (n=1) myelomeningocele. As for the type of neurological
patients with IBD during SARS-COV-2 infection is in line susceptible individuals. treatment, 93.79% (n=151) did not perform and 6.21 (n=10)
with the literature. In this sense, it is relevant to point out did. In addition, 98.14% (n=158) did not have neurolog-
that the percentage of patients with headache was higher ical coinfection and 1.86% (n=3) did. Regarding the type
in the group of patients with COVID than in the uninfected Neuroinfecção of neurological coinfection, 1.24% (n=2) had PVD infec-
group. Finally, the patients with GI worsening also had tion and 0.62% (n=1) had meningitis and brain abscess,
neurological exacerbation. respectively. Regarding the use of neuroimaging, 99.38%
n=160) did not perform it, with only 0.62% (n=1) perform-
ing it. Conclusion: Most patients had no neurological
Neuroinfecção involvement, the most frequent being the presence of
signs and symptoms.

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CEREBELLAR SCHISTOSOMIASIS: AN HOSPITALIZATIONS FOR VIRAL REDUCED WORKABILITY ASSOCIATED


UNUSUAL PRESENTATION ENCEPHALITIS: AN ANALYSIS OF THE WITH INCREASED LEVELS OF FATIGUE AND
EPIDEMIOLOGICAL PROFILE IN BRAZIL IN ANXIETY IN PATIENTS WITH POST-COVID
Bianca Gomes Mazzoni, André Filipe Lucchi
Rodrigues, Thales Fernandes Potenciano, Renata THE YEARS 2019 TO 2021 SYNDROME
Cristina Resende, Henrique Amancio Ferreira, Davi Francisco Matheus Oliveira de Carvalho, Rodrigo Gabriel Monteiro Salvador, Mateus Henrique
Teixeira Urzêdo Queiroz, João Victor Lage Guerra, Mariano Ribeiro, Fernando Degani Vazquez, Maria Nougueira, Lucas Scardua Silva, Italo Karmann
Breno Franco Silveira Fernandes, Rodrigo Santiago Suelly Nogueira Pinheiro, Pedro Vitor Ferreira, Aventurato, Fernando Cendes, Márcia Bandini,
Gomez Luciano de Albuquerque Mota, Pedro Helder de Antonio de cesare Del Nero, Clarissa Lin Yasuda
Hospital das Clínicas da Universidade Federal de Oliveira Junior
Universidade Estadual de Campinas. Campinas SP,
Minas Gerais. Belo Horizonte MG, Brazil Universidade Federal do Ceará. Fortaleza CE, Brazil Brazil
biancagmazzoni@gmail.com Hospital Universitário Walter Cantídio. Fortaleza
CE, Brazil gabrielSalvador1906@gmail.com

Case Presentation: A previously healthy 23 year-old fmatheus.oc@gmail.com Background: Although the neuropsychiatric symptoms
man from a known endemic area for schistosomiasis of fatigue, depression, anxiety and sleepiness are part of
was admitted at the Neurology Emergency Department. Background: Infectious encephalitis is defined as a group the post-covid syndrome, little is known about their neg-
Two months before admission, he developed severe of diseases characterized by an inflammatory process of ative impact on work capacity. Here we quantified these
daily headache, which was refractory to common anal- the brain caused by microorganisms, especially viruses, symptoms with validated questionnaires and analyzed
gesics. Moreover, he complained about weight loss. One with evidence of neurological dysfunction. The clinical the relationship with reduced work capacity. Objective:
month later, he presented with a newly onset cerebel- presentation may involve acute fever, headache, altered To investigate the impact of persistent neuropsychiatric
lar ataxia, more pronounced on the right side. Initial consciousness and focal neurological deficits, with high symptoms (and cognitive dysfunctions) on the occupa-
Magnetic resonance imaging (MRI) revealed high T2 morbidity and mortality. Objective: To analyze the epide- tional vulnerability of survivors in a homogeneous group
signal areas with a slight expansive effect in the right miology of Hospitalizations for viral encephalitis in the SUS of bank workers. Materials and Methods: We analyzed
aspect of the medulla; right cerebellar hemisphere and in Brazil between the years 2019 and 2021. Methods: This 626 individual bank workers diagnosed with confirmed
pons, and meningeal enhancement. Cerebrospinal fluid research is a descriptive, observational, cross-sectional COVID19 (Hospitalized (17%) and non-Hospitalized
(CSF) analysis showed normal opening pressure, slight study of Hospitalizations for viral encephalitis in SUS (83%)). The information was extracted from the database
pleocytosis (16 cells) 75% lymphocytes, protein 35mg/dL. between the years 2019 and 2021. Data were collected in of a follow-up study. Patients answered the “Chalder
CSF Serologies for tuberculosis, cryptococcosis, Syphilis, the DATASUS database, with investigation of the variables: Fatigue Scale (CFQ)”, “Epworth Sleepiness Scale (ESS)”,
HIV and cultures were negative. Computed tomography region, age group, gender, number of Hospitalizations, “Hospital Anxiety and Depression scale (HADS)” and
of the chest and abdomen showed only nonspecific alter- total value, mortality rate and days of stay. Results: There “Work Ability Index (WAI)”. We used SPSS22 for statis-
ations. MRI Spectroscopy suggested a pattern compatible were 5741 Hospitalizations in the period, with 54% men tical analysis with Chi-Square tests for analyses of pro-
with inflammatory disease in detriment of a tumor lesion. and 46% women, with predominance in the Northeast portions. Results: Our patients presented average age 42
An inflammatory disease hypothesis was made and the (41.9%). As for age, the cases occurred more in the range years (22 to 66 years old), sex (53.5% female), education
patient was discharged with oral corticosteroids. However, between 1 and 4 years (16.8%) and less in the range of 80 (48.9% complete undergraduate, 35.6% graduate and
some weeks later, he developed a right peripheral facial years or more (2.3%). The total number of Hospitalizations 8,1% complete high school), vaccination (83.4%) and
palsy and he was readmitted to the Hospital. A new MRI in the first year was 2436, being higher than the following function at work (74.6% contact with the public and 25%
showed progression; Enhancement of right VII and VIII years, which had 1689 and 1616 cases. The total costs were administrative work). With an average interval of 200 days
cranial nerves and a recent ischemic event on the left lat- R$11.7M, with higher spending in the Northeast region between diagnosis and online interview, subjects SELF-
eral aspect of the pons. Finally, a cerebellar biopsy was (40.9%). The average total amounts per Hospitalization reported several symptoms, including related fatigue
performed and revelead a Schistosomal granulomatous were R$1913, R$2103, R$2153, respectively, in the years (37%), anxiety (36%), depression (17%) and cognitive
cerebellitis. The patient was treated with Praziquantel 2019, 2020, and 2021. The average mortality rate was 5.4%, dysfunction (29%). Approximately 20% reported no post-
and high dose glucocorticoids with partial improvement. with higher rates in those aged 60 to 69 years (10.5%), 70 COVID symptoms at the interview. The quantification of
Discussion: Schistosoma mansoni is endemic to many to 79 years (10.3%), and 80 years or older (18.4%). The symptoms with questionnaires revealed excessive som-
brazilian states. Its eggs may be found throughout the number of days of stay was highest in the 1- to 4-year age nolence (ESS) (45,1%), fatigue (CFQ) (79,2%), symptoms
Central nervous system (CNS). They have been reported group, with 9121 total days, and lowest in the 80 years and of anxiety (HADS) (60,6%) and symptoms of depression
into the brain, cerebellum, leptomeninges and choroid older populations, with 1470 days. Conclusion: Based (HADS) (65,9%). In addition, the comparison of symp-
plexus. However, the most common presentation is the on the data, we noted a significant difference in the total toms between the group with normal WAI (37.5%) and
schistosomal myeloradiculopathy. CNS lesions may cause number of Hospitalizations throughout the years of the reduced WAI (62.5% of participants) revealed high levels
cerebral small vessels endarteritis with fibrinoid necrosis, period, which makes it possible to suggest interference of excessive sleepiness (25% without somnolence versus
or immunological reactions secondary to toxin release from the pandemic, requiring further studies on the 56%; p<0.05), fatigue (54% no fatigue versus 93.2% with
from the granuloma, leading to astrocytic proliferation, subject from now on. It is also observed that, despite the fatigue; p<0.05), symptoms of depression (18% versus
neuronal necrosis and demyelination. Conclusion: This lower number of Hospitalizations among the elderly, this 68.2%; p<0.05) and anxiety (30% versus 76.8%, p<0, 05).
case shows the challenge of an atypical presentation of group had the highest mortality rates in the period, so as Discussion and Conclusion: Our results reveal a negative
neuroschistosomiasis, which could have been easily mis- to suspect the importance of age during Hospitalization impact of neuropsychiatric symptoms on reduced work
diagnosed as a noninfectious inflammatory or neoplastic and early diagnosis. Finally, it is worth mentioning the ability months after the acute infection. These findings
disease. A high index of suspicion is necessary, mainly in high financial investments evaluated, raising the impor- point to the urgent need to provide specific treatment
those patients coming from endemic areas, and histolog- tance of more extensive research capable of assessing for patients to minimize the individual burden and the
ical diagnosis remains crucial. both direct and indirect costs resulting from severe cases economic loss.
of viral encephalitis in Brazil.
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ATYPICAL NEUROLOGICAL MANIFESTATIONS FREQUENCY OF EPSTEIN-BARR VIRUS EVALUATIVE STUDY OF THE MOST
POST-COVID-19: A CASE SERIES (EBV) AND HUMAN HERPESVIRUS 6 (HHV-6) PREVALENT INFECTIOUS AGENTS
INFECTION IN PATIENTS WITH RELAPSING- ASSOCIATED WITH MENINGITIS/
Thaís de Maria Frota Vasconcelos, Paulo Ribeiro
Nóbrega, José Daniel Vieira de Castro, Moysés REMITTING MULTIPLE SCLEROSIS ENCEPHALITIS BEFORE AND DURING THE
Loiola Ponte de Souza, Pedro Braga Neto, Manoel COVID-19 PANDEMIC. EXPERIENCE IN THE
Jéssica Gonçalves Pereira, Luciane Almeida Amado
Alves Sobreira Neto Leon, Nathalia Alves Araújo de Almeida, Jéssica CITY OF RIO DE JANEIRO
Hospital Universitário Walter Cantídio – Vasques Raposo-Vedovi, Fabri?cia Lima Fontes- Léo Freitas Correa, Ana Luiza Lavrado Diblasi,
Universidade Federal do Ceará. Fortaleza CE, Brazil Dantas, João Gabriel Dib Farinhas, Vale?ria Vale?ria Patricia Provenzano Leal, Cristiane Casanova,
Coelho Santa Rita Pereira, Soniza V Alves-Leon, Celina de Oliveira, Ricardo Canuto Benesi, Marcus
thaisvasconcelos53@gmail.com Vanessa Salete de Paula Tulius Teixeira Silva, Carlos Otavio Brandao

Background: SARS-COV-2 infection has affected millions FIOCRUZ. Rio de Janeiro RJ, Brazil Neurolife. Rio de Janeiro RJ, Brazil
of individuals to date. This disease has variable manifes- Universidade Federal do Estado do Rio de Janeiro. FIOCRUZ. Rio de Janeiro RJ, Brazil
tations from an asymptomatic condition up to a rapidly Rio de Janeiro RJ, Brazil
Universidade Federal do Rio de Janeiro. Rio de marcustulius@uol.com.br
evolving severe. Moreover, this infection may lead to
multisystemic involvement, including the central and Janeiro RJ, Brazil
peripheral nervous systems. We report below three cases Fiocruz. Rio de Janeiro RJ, Brazil Infectious meningitis/encephalitis (EM) are pathological
conditions associated with high rates of morbidity/mortal-
of atypical neurological manifestations post- COVID-19 jessica-gpereira@hotmail.com ity, where the examination of the cerebrospinal fluid (CSF)
and their clinical follow-up. Case Presentation: 27-years-
is essential in the diagnosis. In 2020 and 2021, the world
old male patient, previously healthy, who had symptoms Multiple sclerosis (MS) is a chronic neuroinflammatory was faced with a pandemic caused by the SARS-CoV2 virus.
of COVID-19 confirmed by oropharyngeal and nasopha- and neurodegenerative disease of the central nervous We selected CSF samples in 2 different periods: Before the
ryngeal swab tests. On the tenth day of symptoms, the system (CNS). The etiology of MS is not well understood, pandemic and during the pandemic. CSF samples col-
patient started to experience acute paraparesis, urinary but it’s likely one of the genetic and environmental factors. lected were sent to the Neurolife Laboratory immediately
retention, constipation, and hypoesthesia, up to T4 level. Approximately 85% of patients have relapsing-remitting after lumbar puncture for diagnostic investigation. CSF
The second patient is a 50-years-old male, previously MS (RRMS), while 10-15% have primary progressive MS was analyzed by conventional and molecular tests. The
healthy, who had symptoms of the flu-like syndrome. (PPMS). Epstein–Barr virus (EBV) and Human herpesvirus screening of the panels was performed according to the
The diagnosis of COVID-19 infection was confirmed by 6 (HHV-6), members of the human Herpesviridae family, clinical indication, epidemiology and morbid history of
oropharyngeal and nasopharyngeal swab test. On the are strong candidates for representing the macroenviron- the patients. In 2019, 737 PCR tests/month were performed
tenth day of symptoms, the patient started to experience mental factors associated with MS pathogenesis. Antigenic for the main pathogens related to meningitis/encephalitis.
paraparesis, urinary incontinence, and hypoesthesia up mimicry of EBV involving B-cells has been implicate in In 2020, this monthly average was lower (8%) due to the
to T6 level. The neuroimaging and cerebrospinal fluid MS risk factors and concomitance of EBV and HHV-6 reflection of the pandemic. It is worth mentioning that
(CSF) analysis of both patients confirmed acute transverse latent infection has been associated to inflammatory MS from April onwards the increase in PCR requests for SARS-
myelitis, after COVID-19 infection. High-dose corticoste- cascade. To verify the possible role of EBV and HHV-6 CoV-2 in CSF was also perceptible, including becoming
roid therapy was started, and both patients showed rapid as triggering or aggravating factors in RRMS and PPMS, among the top 5 of the PCRs most requested by the medical
recovery from their deficits. The third patient is a 45-year- we compare their frequency in blood samples collected community. Although the number of PCR tests requests
old male patient who presented an 8-month history of from 166 MS patients. The presence of herpes DNA was has returned to the equivalent in the period before the
progressive gait disorder and cognitive impairment after searched by real-time PCR (qPCR). The frequency of EBV pandemic, the lockdown schemes and restrictions due
being Hospitalized for SARS-CoV-2 infection. Magnetic and HHV-6 in MS patients were 1.8% (3/166) and 8.9% to the pandemic showed a drop in the numbers of infec-
resonance imaging (MRI) was compatible with NPH. A (14/166), respectively. Among the positive patients, 100% tious agents reported in cases of meningitis/encephalitis.
spinal tap test was positive and there was a progressive (3/3) EBV and 85.8% (12/14) HHV-6 are RRMS and 14.4% A reduction from 354 positive cases (in 2019) to 124 (in
improvement after shunting, with complete resolution (2/14) HHV-6 are PPMS. About clinical phenotype of these 2020) and 141 (in 2021) can be observed. This is evident
of symptoms after 30 days of follow-up. Conclusion: patients, incomplete multifocal myelitis, and optic neuri- when looking at positive Enterovirus cases in 2019 com-
We reported two cases of patients who developed acute tis were the main CNS manifestations. These are the first pared to the pandemic years. The numbers have dropped
transverse myelitis after COVID-19 infection that had a data about concomitant infection of these viruses in MS by almost 80%. In 2019, there were 151 patients, while in
good and rapid response to high doses of methylprednis- patients from Brazil. Up to date, our findings confirm a 2020 there were 19 and in 2021 there were 24 patients. The
olone, unlike the previous reports. The differences in the higher prevalence in female with MS and a high frequency top 5 pathogens found in 2019 were Enterovirus (151),
doses and the treatment time may explain this. The third of EBV and HHV-6 in RRMS patients. Herpes simplex 2 (34), Streptococcus Pneumoniae (34),
patient is a previously healthy patient who developed
Varicella Zoster (23) and Neisseriae Meningitidis (17). In
NPH two months after the COVID19 infection. Given
2020 and 2021, the top 5 were, respectively, Enterovirus
the significance of this condition as a cause of reversible Neuroinfecção
(19) and (24), Varicella Zoster (18) and (26), Streptococcus
dementia, it is important to consider the possibility of a
Pneumoniae (16) and (6), Herpes simplex 2 (8) and ( 12)
causal association with COVID19.
and Neisseriae Meningitidis (2) and (5).Conclusion: It is
evident that the decrease in the movement of people was
Neuroinfecção reflected in the drop in cases of meningitis/encephalitis
throughout the pandemic.

Neuroinfecção

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XXX Congresso Brasileiro de Neurologia

TL 1106353 TL 1106491 TL 1106540

GREY MATTER ATROPHY ACCORDING TO HYPEROXIA BY SHORT-TERM PROMOTES BRAIN MAGNETIC RESONANCE IMAGING
FOUR COVID-19 STRAINS: FRONTAL LOBE OXIDATIVE DAMAGE AND MITOCHONDRIAL (MRI) FINDINGS IN PATIENTS WITH
AND LIMBIC ATROPHY ARE COMMON TO DYSFUNCTION IN RAT BRAIN INFECTIOUS CEREBROSPINAL FLUID (CSF):
DIFFERENT STRAINS A SINGLE-CENTER EXPERIENCE FROM
Taís Luise Denicol, Richard Simon Machado,
Leonardo Tenfen, Larissa Joaquim, Mariana A TERTIARY CARE HOSPITAL IN NITEROI,
Lucas Scárdua-Silva, Beatriz Amorim da Costa, Vítor
Guimarães Corrêa, Rafael Batista João, Mateus Pacheco de Oliveira, Mariella Reinol da Silva, BRAZIL
Henrique Nogueira, Ítalo Karmann Aventurato, Lucinéia Gainski Danielski, Gislaine Tezza Rezin,
Fernanda Cristina Rueda Lopes, Luis Alcides
Marina Koutsodontis Machado Alvim, Mariana Fernanda Frederico Gava, Fabricia Petronilho
Quevedo Canete, Marcus Tulius Teixeira da Silva,
Rabelo de Brito, Fernando Cendes, Clarissa Lin Monica Ferreira Caramalho, Alair Augusto Sarmet
Department of Medicine, University of South Santa
Yasuda Santos
Catarina – Tubarão – SC – Brazil
Universidade Estadual de Campinas. Campinas SP, Laboratory of Experimental Neurology, Graduate
Complexo Hospitalar de Niterói – Niterói – RJ –
Brazil Program in Health Sciences, University of Southern
Brazil
Santa Catarina – Criciuma – SC – Brazil
scardua@unicamp.br frueda81@hotmail.com
taisdenicol@hotmail.com
Background: The presence of neuropsychiatric symp- Central nervous system (CNS) infections are a significant
toms in the post-COVID syndrome seems frequent and Background: Oxygen (O2) is an essential molecule for
cause of mortality and morbidity world-wide. Infectious
debilitating and represents a major public health concern. aerobic life, being the basis of several metabolic reactions.
diseases of the CNS include a wide spectrum of infections
Unfortunately, neither the physiopathology nor the under- Oxygen therapy is used as a therapeutic protocol to pre-
caused by various pathogens affecting one or more of
lying cerebral alterations are understood. Objectives: To vent or treat hypoxia. However, a high inspired fraction
these components. Despite advances in medicine, the
investigate structural brain alterations (gray matter atro- of O2 (FIO2) promotes hyperoxia, a harmful condition
accurate diagnosis of a specific disease of CNS infections
phy) associated with post-COVID syndrome in patients for the central nervous system (CNS). Objectives: The
has proved to be very challenging, as its clinical variable
with different strains of SARS-CoV-2. Methods: We ana- present study evaluated parameters of oxidative stress and
severity and radiological presentations are very general
lyzed 3T T1 MRI images of 300 post-COVID patients mitochondrial dysfunction in the brain of rats exposed
and nonspecific. In most cases, the etiological diagnosis
followed at the University of Campinas (median age: to different FIO2 for a short period. Method: Male Wistar
is only confirmed with the investigation of the pathogen
40 y.o.; 209 women): 188 infected with the Alpha strain, rats were exposed to hyperoxia (FIO2 40% and 60%) com-
in the cerebrospinal fluid. This study aimed to know and
29 infected with the P.2-Gamma strain, 61 infected with pared to the control group (FIO2 21%) during 2 hours.
describe, through the cases, the MRI findings in patients
the P.1+P.1.*-Gamma strain, 22 infected with the Delta Oxidative damage in lipids and proteins, the activity of
diagnosed with infectious diseases through the inves-
strain and 90 healthy controls scanned during the pan- the antioxidant enzyme catalase (CAT), nitrite/nitrate
tigation of the pathogen in the cerebrospinal fluid and
demic. The determination of strain was according to the (N/N) concentration, neutrophilic infiltration, and mito-
followed up in a tertiary Hospital in Niteroi-Brazil, from
Fiocruz database (http: //www.genomahcov.fiocruz.br/ chondrial respiratory chain enzymes were determined
December 2019 to March 2022. Among the 17 patients
dashboard/), which exhibits the brazilian SARS-CoV-2 in the hippocampus, striatum, cerebellum, cortex, and
studied, the most common etiologic agent was varicella
predominant strains by intervals. We used the CAT12 prefrontal cortex after 120 minutes of exposure. Results:
zoster virus (5 cases). Other etiological agents found in
toolbox/ SPM12/MATLAB 2019 to perform Voxel-Based The animals exposed to hyperoxia showed increased lipid
the study were Epstein Barr virus, herpes virus type 1, her-
Morphometry (VBM) analysis (to detect grey matter peroxidation, formation of carbonyl proteins, N/N con-
pes virus type 2, Candida spp, Pseudomonas aeruginosa,
atrophy (GMA)) and statistical analyses. We reported centration, and neutrophilic infiltration in some brain
Klebsiella pneumoniae and Enterovirus. what was most
p-values<0.001, we used sex, age and total intracranial regions, like hippocampus, striatum, and cerebellum
observed were images suggestive of diffusion restriction
volume as covariates and corrected the results for multiple being the most affected. Furthermore, CAT activity and
with an infarct pattern, some being a confounding factor,
comparisons. Results: Overall, all four strains presented activity of mitochondrial enzyme complexes were also
due to the fact that the CNS infection was still unknown
GMA atrophy of bilateral anterior cingula and ventrome- altered after exposure to hyperoxia. Conclusion: rats
at the time of the first MRI. We can then conclude that in
dial frontal lobes. In addition, Strain P.2-Gamma showed exposed to FIO2 > 21% by 2 hours showed increase in
critically ill patients, with suspected CNS infection, the
GMA of the right cuneus and left superior occipital gyrus. oxidative stress parameters and mitochondrial dysfunc-
imaging appearance may appear as recent infarctions.
The P.1+P.1.*-Gamma Strain presented more widespread tion in brain structures.
atrophy, mainly in the right angular gyrus, fusiform gyrus,
frontal operculum and precuneus and left frontal oper- Neuroinfecção
culum and precuneus. Interestingly, the Alpha and Delta Neuroinfecção
Strains revealed exclusive frontal lobe GMA. Conclusion:
Despite the vaccination and some differences, in acute
presentation, all four groups presented GMA of THE ante-
rior cingula and ventromedial frontal area. Although dif-
ferent strains may show individual variations, post-COVID
syndrome seems to exhibit some commonalities of GMA.
These findings suggest similarities in terms of cerebral
injury and physiopathology across different SARS-CoV-2
strains. Given the location of GMA, these areas may be
somehow associated with the highly prevalent neuropsy-
chiatric symptoms (dysexecutive syndrome, depression
and anxiety) in post-COVID syndrome. Further research
in this area is necessary to correlate with neuropsychiatric
dysfunction and to detect therapeutic targets.

Neuroinfecção

316
XXX Congresso Brasileiro de Neurologia

TL 1106595 TL 1106604 TL 1106607

CHARACTERIZATION OF INDIVIDUALS PREVALENCE OF ANOSMIA IN LONG-COVID ABSENCE OF STRAIN-SPECIFIC EFFECTS ON


AFFECTED BY POST-SURGICAL INFECTION PATIENTS: A COHORT OF 221 PATIENTS COGNITIVE DYSFUNCTION AFTER COVID-19:
IN THE NERVOUS SYSTEM IN A REFERRAL ANALYSES OF 454 SUBJECTS
Danilo Nunes Oliveira, Jose Wagner Leonel Tavares
HOSPITAL Junior, Safira de Brito Gaspar, Álissa Ellen Formiga Ítalo Karmann Aventurato, Mateus Henrique
Luan Monte Barroso, Marina Mayara Pereira, Moura, Manoel Alves Neto Sobreira, Pedro Braga- Nogueira, Lucas Scardua Silva, Rafael Baptista
Cristiano Ribeiro Soares, Carolina Barcha Santos, Neto João, José Flávio Becchelli, Alan Ferreira dos
Matheus Fernando Manzolini Santos, Leila Camila Santos Silva, Mariana Rabelo
Universidade Federal do Ceará. Fortaleza CE, Brazil
de Brito, Fernando Cendes, Clarissa Lin Yasuda
UNINOVAFAPI. Teresina PI, Brazil danilonunesoliveira@gmail.com
Universidade de Araraquara – Araraquara – PI – Universidade Estadual de Campinas. Campinas SP,
Brazil Brazil
Background: Olfactory dysfunction (OD), including anos-
Universidade Federal de São Carlos, São Carlos SP, mia and hyposmia is a frequent symptom among upper italo.kar.av@gmail.com
Brazil respiratory symptoms in COVID-19 patients. Long-COVID
luanmontebarroso@gmail.com refers to the persistence of symptoms beyond the initial Background: Long-term effects on cognitive function
phase of infection. Of note OD is a frequent symptom in are present even after mild COVID-19 infection, leading
Background: Surgical procedures in the nervous system Long-Covid. The suggested pathophysiology of hyposmia the WHO to coin the term “long COVID”. Recent reports
have a number of possible complications. In this context, and anosmia in Covid-19 involves the injury caused by suggest that the viral strain plays a role in determining
the development of a surgical site infection (SSI) is capa- SARS-CoV-2 to nasal mucosa and olfactory bulb, con- long-COVID symptoms. Objective: This study aims to
ble of causing high morbidity and mortality, psychoso- sidering its high expression of angiotensin converting evaluate the effect of the presumed viral strain on differ-
cial impacts on the life of the infirm and their families, enzyme 2 (ACE2), as the virus has a high neurotropism for ent cognitive domains. Methods: Data from UNICAMP’s
in addition to increasing treatment costs for the health it. Objective: To analyze quantitatively the epidemiology NeuroCOVID cohort was used in the study. The date of
institution. The literature related to SSI in neurosurgery of OD as a long-term effect of coronavirus infection in a the positive test was used as a proxy to separate subjects
is scarce. Objective: To detect the incidence of SSI, pre- cohort of patients. Methods: We performed a prospective in different presumed strain groups according to the
disposing factors and, finally, to evaluate the forms of cohort study with patients followed in a post-COVID neu- prevalent strain Fiocruz database (http: //www.genomah-
prevention. Method: This is a descriptive, cross-sectional rological outpatient clinic. Patients were recruited based cov.fiocruz.br) Subjects were evaluated with a neuro-
and retrospective study to assess the incidence of SSI on positive COVID-19 PCR and post-COVID neurologi- psychological battery consisting of the following tests:
in neurosurgery at a referral Hospital in the interior of cal symptoms. Patients were evaluated by neurological phonetic fluency (FAS), semantic fluency (SF: animals),
the state of São Paulo. Secondary data contained in the examination, Addenbrooke Cognitive Scale (ACE-R) and logic memory (immediate and late recall), Rey-Osterrieth
medical records of patients undergoing neurosurgical laboratory tests for APOE genotyping. OD was evaluated complex figure test (copy and recall), colored trails test
interventions at Hospital Santa Casa de Araraquara-SP, by clinical complaint and simple olfactory examination. A and B, 9-hole peg test (dominant and nondominant
a tertiary referral Hospital for elective and emergency The duration of the OD was estimated in basis of clinical hand) and the five digits test (reading, counting, choice,
procedures, from 2017 to 2020, were used. RESULT: We history and day of evaluation. Results: The final sample alternation, inhibition and flexibility), comprising 16
obtained an incidence of SSI in neurosurgeries of 6.5%, consisted of 221 patients. Nearly half of patients (N =101; cognitive measures. We converted Test scores to Z-scores
in 651 surgeries, mainly wall infection (35.9%) and ven- 45.7%) had OD during COVID-19 infection. More than a using normative tables for sex, age and education. The
triculitis/meningitis (35.9%). The mean age of affected half of these patients (N = 65; 64.34%) persisted with OD MANOVA test was initially used to test for strain effects
individuals was 53.8 years and 79.5% of patients who for an average of 6.3?3.8 months. Subjects with persistent on any cognitive domain, followed by one-way ANOVA
developed infection had comorbidities. Elective surgeries OD had 40.6?13.3 years and those without persisten OD tests for each domain. Results: 454 subjects (15 years of
and clean procedures had a higher frequency of infection had 48.8?14.2 years (p<0.01). Gender (p=0.54), ACER education; 88 days after diagnosis) were divided into 5
than non-elective and potentially contaminated proce- score (p=0.26), allele frequency in Apolipoprotein epsilon presumed strain groups: original strain (247), original+P2
dures (53.8%). Trauma and hemorrhage interventions genotype test (p=0.30) showed no significant difference (36), P2 (86), gamma+P2 (50) and delta (6). Data for all
showed a greater presence of SSI compared to other between groups. Conclusion: Persistent of OD is a com- tests were available for 141 subjects. One-way MANOVA
types of surgeries; 53.8% of SSIs were diagnosed during mon clinical manifestation in patients with COVID-19. in this subset showed significant strain effects (p=0.046).
the outpatient follow-up of the patient; 66.7% of the acts Our study identified that younger age is a risk factor for Nonetheless, post-hoc tests showed no strain effects on
used some type of drain or graft device. S. aureus was the persistent olfactory dysfunction. It should be noted that any of the cognitive tests. SF showed a marginally signif-
most frequently isolated pathogen. The study showed patients with dementia and elderly patients may not have icant effect (n=392, p=0.056) due to better performances
an incidence of infection similar to other national and brought this complaint to the consultation, raising the age in the original+P2 and P2 strains. FDT reading subtest was
developing country studies, but with a higher incidence of this group of participants. marginally significant (n=323, p=0.073) due to better test
in elective surgeries, which may be related to the lack of performances in the P2 and delta presumed strain groups.
institutional protocols in the postoperative period and Discussion: although a global test effect was observed,
Neuroinfecção individual test data could not show significant effects of
neurosurgical dressings.Conclusion: The investigation
demonstrated the need to develop institutional protocols the presumed strain. Marginal significance in semantic
that indicate the adoption of pre, intra and post-surgical fluency and FDT reading subtest mainly accounted by
measures, such as: use of intranasal mupirocin, metic- presumed P2 strain subjects’ better performances, sug-
ulous aseptic technique and exchange of gloves before gesting P2-related post-COVID syndrome may relatively
the placement of prostheses, as well as the maintenance spare processing speed. Regardless of the absence of
of prolonged outpatient follow-up and patient guidance strain-specific effects, post-COVID subjects in the cohort
on surgical wound care. showed significant cognitive deficits, which may impair
workability and quality of life.

Neuroinfecção
Neuroinfecção

317
XXX Congresso Brasileiro de Neurologia

TL 1106114 TL 1106569 TL 1104780

GLASGOW SCALE: WHAT IS IT AND HOW TO THE COGNITIVE IMPACT OF POST-INTENSIVE IMPACT OF THE SARS-COV-2 PANDEMIC
USE IT? CARE SYNDROME: A REVIEW OF RECENT ON ALZHEIMER’S DISEASE PATIENTS: A
PERSPECTIVES RETROSPECTIVE AND EVOLUTIONARY
Bibiana Mayer, Chadi Emil Adamo
ANALYSIS OF COGNITIVE ASPECTS
Monique Evelyn Mendonça do Nascimento
Universidade de Ribeirão Preto – UNAERP. Ribeirão
Preto SP, Brazil Christian Max Uchôa Leite, Norberto Anízio Ferreira
Hospital da Restauração de Pernambuco. Recife
Universidade de São Paulo Universidade de São Frota, Flávia de Paiva Santos Rolim, Vitor Carneiro
PE, Brazil
Paulo. Ribeirão Preto SP, Brazil de Vasconcelos Gama, João Lucas A Morais,
moniqueemnascimento@gmail.com Amanda Gonçalves Moura
bibiana_mayer@hotmail.com
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Background: Mortality from critical illness has decreased
The Glasgow Coma Scale (GCS) is widely used to assess in recent decades. Intensive care unit survivors experi- christiannmax@hotmail.com
and calculate the patient’s level of consciousness, due to ence cognitive impairments and mental health problems,
the simplicity of the scoring system and rapid application referred to as part of post-intensive care syndrome (PICS). Alzheimer’s Disease(AD) is a neurodegenerative disor-
at the bedside. The objective is to outline a strategy that Objective: In this review, we summarize recent data der that manifests as deterioration of multiple cognitive
combines the main indicators of severity in traumatic brain on PICS cognitive impairment and its new challenges. domains. These patients are at greater risk of Covid-19
injury (TBI) in a simple scale. There are three criteria: eye Methods: This is a literature review article that studied and suffering with the pandemic, which can lead to accel-
opening (up to 4 points), verbal response (up to 5 points) patients in the Intensive Care Unit. The data search used erated functional and cognitive impairment. Thus, it is
and motor response (up to 6 points). Added together, they LILACS, SciELO and Cochrane databases. Results: In cur- necessary to understand the clinical and social impact
give a score between 3 and 15. Eye opening, when spon- rent studies, questionnaires such as the Short-Memory of the SARS-Cov-2 pandemic in this group. The aim of
taneous, takes 4 points. If not, commands are requested, Questionnaire (SMQ) were used. Cognitive impairment the study was to describe the evolution of cognitive and
such as calling the patient by name, setting the score to occurs in approximately 40% of ICU survivors within 3-6 functional performance of AD patients from 2018-22. A
3. Without response, a painful stimulus is applied (supra months of critical illness and persists for at least 1 year in retrospective cohort of 77 AD patients treated at a ter-
orbital notch pressure or trapezius pinching), receiving some patients². Areas commonly affected are attention, tiary Hospital in Fortaleza was carried out from 2018
2 points. A minimum score of 1 indicates that there are concentration, memory, mental processing speed, and to 2022. Cognitive(cross-sectional and retrospective)
no openings. To assess the verbal response, one asks, for executive functions. Among them, the most commonly assessment was performed with the Mini Mental State
example: “what month is it in?”, “what is your name?”. affected domains are memory and executive functions. Examination(MMSE), Semantic Verbal Fluency(SVF)
Responding, the maximum score is given. Otherwise, the Risk factors for cognitive impairment after admission to and Clock Drawing Test(CDT) and functional assess-
extent of their verbal skills is assessed. Sentences can be the Intensive Care Unit are preexisting cognitive dysfunc- ment with the Clinical Dementia Rating Scale(CDR) and
coherent and complete, but with confusion about time, tion, delirium, sepsis, hypoxia, and invasive mechanical Functional Staging Scale(FAST), in addition to clinical
place or person (4 points). Inappropriate use or disorga- ventilation. It has been seen that patients face the diffi- aspects, caregiver stress, satisfaction with telemedicine
nized words refers to audible, random and out of context culty of returning to their original lives due to disabilities and social isolation. We used MMSE and FAST 2 and 3
words (3 points). The patient may make noises or moans, that persist for up to years after discharge from the ICU.¹ [(1-17) and (1-7), respectively, discrete numerical vari-
without forming any words (2 points). If there is no audible Also, it was seen that bundles like ABCDEF can help to ables] to define significant impairment. The evolution of
response, the score is minimal. For the motor response, prevent PICS. Conclusion: Survivors of critical illness are the data in relation to the beginning of the pandemic was
those who fit the maximum score obey commands such at high risk of developing post-intensive care syndrome. recorded as: before(2018-19), during(2020-21/22), and in
as: “close your hands”, “show your tongue”. If negative, These findings highlight the importance of adequate post- the transition(2019-20). The three annual evolutions were
the pain response is evaluated with the aforementioned ICU care planning to diagnose and treat this population. then compared to each other. The results showed that in
stimuli. In this case, the patient can locate it correctly four years of outpatient follow-up, there was a functional
(5 points); flexing or withdrawing a limb away from the deterioration, assessed by an increase in CDR [1(0.5-2) to
pain stimulus (4 points); perform adduction and inter- Neurointensivismo 3(2-3)], FAST 2 [4(3-5) to 8(5-11)] and FAST 3 [4(3-5) to
nal rotation of the upper limbs (3 points)- “decortica- 6(5-7)](p<0.05). The last two configured significant func-
tion posture” (suggesting damage above the level of the tional impairment. There was an increase in the prevalence
red nucleus)- or even, extend upper and lower limbs (2 of anticholinesterase, anti-NMDA and antipsychotic use
points)- “decerebration posture”. With no response, the (36.5% to 74.3%; 20.3% to 45.9%; 18.9% to 44.6%, respec-
patient receives 1 point. The GCS is just one part of the tively) and its side effects. We found an increase in the
neurological system exam. It is also important to assess prevalence of urge incontinence (1.4% to 37.8%) and of
pupillary reactivity, brainstem reflexes, tendon reflexes, neuropsychiatric symptoms such as anxiety (35.1 to 58.1%)
signs of meningismus, and plantar skin response. Together, and insomnia (17.6 to 40.5%). Cognitive worsening was
they can help localize the neurological lesion. Even in the evidenced through the MMSE, SVF and CDT scores (15
regular observations of an inpatient, attention is paid to to 11; 6 to 4.5; and 2 to 0, respectively;p<0.05), the first of
the trend of GCS scores, since an abrupt decrease may which configured significant cognitive impairment. There
represent neurological deterioration, requiring urgent was an increase in caregiver stress (10.8 to 74.3%). It was
additional evaluation. possible to conclude that AD patients showed significant
cognitive and functional impairment after the beginning
of the pandemic. The follow-up of these patients is nec-
Neurointensivismo
essary in order to better describe the long-term extent of
the effects of the pandemic in this group.

Neurologia Cognitiva e do Envelhecimento

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XXX Congresso Brasileiro de Neurologia

TL 1105029 TL 1105056 TL 1105062

EFFECTS OF THE MOZART’S SONATA K448 CHRONIC STRESS MEDIATORS IN PATIENTS CAUSES OF RAPIDLY PROGRESSIVE
ON THE EXTINCTION OF AVERSIVE MEMORY WITH MILD COGNITIVE IMPAIRMENT: A DEMENTIA: A MULTICENTRIC STUDY IN
TRANSVERSAL ANALYSIS OF THE BRAZILIAN TERTIARY MEDICAL CENTERS OF SANTA
Mateus Lopes de Lima Falsarella, Kelly Christina
da Mota, Clarissa Maria Ferreira Trzesniak, Rodolfo MEMORY AND AGING STUDY CATARINA
Souza de Faria, Cesar Sartori Breno José Alencar Pires Barbosa, Maria Clara Eduardo de Novaes Costa Bergamaschi, Adaucto
Faculdade de Medicina de Itajubá. Itajubá MG, Ferreira Jesus, Maira Okada de Oliveira, Isabella Wanderley da Nóbrega Júnior, Edson Pillotto Duarte,
Brazil Avollio, Luciana Casimiro, Juliana Nery Souza- Matheus Souza Steglich, Manuela Aparecida
Universidade Estadual de Campinas. Campinas SP, Talarico, Sonia Maria Dozzi Brucki Kloeppel, Gabriel Martins Rodrigues, Iara de Sousa
Brazil Coelho, Myllan Eiroa Feitosa, Katia Lin
Universidade Federal de Pernambuco. Recife PE,
mateus.falsarella@gmail.com Brazil Universidade Federal de Santa Catarina.
USP Florianópolis SC, Brazil
Background: Memory is the ability to acquire, to store and University of Iowa – Estados Unidos Hospital Regional Homero de Miranda Gomes –
to recall information. Memory can be classified as short- Florianópolis – SC – Brazil
brenojb@gmail.com Instituto de Psiquiatria de Santa Catarina – São
term and long-term memory. It is known that music has
an effect on almost the entire brain and its neural sub- José – SC – Brazil
Background: Several studies suggest that chronic stress Hospital Governador Celso Ramos – Florianópolis
systems. In addition, it seems to play a crucial role in the and persistently high levels of cortisol are related to cog-
treatment of phobias and post-traumatic stress disorder. – SC – Brazil
nitive dysfunction and neurodegenerative processes.
There is evidence of positive effects of Mozart’s Sonata The brazilian Memory and Aging Study (BRAMS) is a ed.brg_@hotmail.com
K448 on short-term memory. However, little is known research initiative for the longitudinal follow-up of indi-
about the effect of memory extinction to the environment. viduals in a tertiary center specialized memory outpa- Background: rapidly progressive dementia (RPD) is
Objective: To investigate the influence of Mozart’s Sonata tient clinic. Objectives: to investigate the relationship characterized by accelerated cognitive decline leading to
K448 in the extinction of the aversive memory. Methods: between markers of chronic stress and cognitive status functional impairment. There is no clear accepted defi-
Twenty mice were used, divided into three groups: G1: in BRAMS, making comparisons between the control, nition of RPD, with most studies using a time frame of 1
Mozart (n=5), G2: Ambience(n=8) and G3: Control (n=7). subjective cognitive decline (SCD) and mild cognitive to 2 years from symptom onset to dementia. There are
Only the G1 group was exposed to music from intrauterine impairment (MCI) groups (amnestic, non-amnestic, single many causes of RPD, but few studies have examined the
life onwards, from 9: 00 pm to 7: 00 am. From days 50 to domain or multiple domains). Secondarily to correlate epidemiology of different RPD etiologies. Objectives: this
54, the animals were submitted to Habituation. On the the markers with the amyloid status of the participants. study aimed to identify the relative frequency of different
55th day, the Aversive Training was performed only with Methods: observational study of the cross-sectional type causes of RPD among inpatients from four tertiary medical
G1 and G2 groups. On the 56th day, the Extinction Test with a subgroup analysis of the BRAMS cohort, in which centers in Florianópolis, Santa Catarina, Southern Brazil.
started and was repeated for 12 consecutive days for the individuals aged > 60 years who had access to medical, This is an ongoing study and we report its preliminary
three groups. On the 81st day, all groups were submitted neuropsychological and amyloid PET assessment. The results. Methods: this is a cross-sectional retrospective
to the Recall Test. The tests were recorded for analysis. The present analysis seeks to measure the allostatic load study. Medical records of patients admitted from 2001
Extinction Test and the Recall Test were analyzed by the index (ALi) through anthropometric, endocrinological, to 2020 and registered under ICD-10 codes potentially
repeated measures ANOVA and one-way ANOVA tests cardiovascular, metabolic and inflammatory markers of linked to RPD were identified and searched for RPD cases.
respectively, with values of p?0.05 being considered sig- chronic stress, correlating the variables with cognitive sta- RPD was defined as any disorder fulfilling the National
nificant. Results: The Mozart group had higher freezing tus and amyloid status. Results: Between the years 2018 Institute on Aging – Alzheimer’s Association criteria for
time in the Extinction Test when compared to the other and 2021, 77 participants were evaluated in the present dementia whose course from symptom onset to dementia
groups (p<0.05), especially on the first 3 days. In addition, protocol, of which 65 met the inclusion criteria. The mean was less than or equal to 2 years. Patients diagnosed with
the decrease in freezing time was greater for the Mozart age was 70.2 (+ 6.01) years, with 72% being female with a primary psychiatric disorder or delirium were excluded.
group in relation to the other groups (p<0.05). Finally, 11 years of schooling (interquartile range 7 – 15). There The etiological RPD diagnoses registered at Hospitalization
the Mozart group showed a significant increase in freez- were 42 subjects classified as MCI, 15 as SCD and 8 as were reported. Each medical record was reviewed and
ing time compared to the other two groups in the Recall controls. There was a significant difference between HDL the authors reclassified the RPD etiology according to
Test. Conclusion: Mice submitted to Mozart’s Sonata measurements (55.6 in the MCI group vs. 66 in the SCD established diagnostic criteria. Results: 4714 medical
K448 during pregnancy had a longer freezing time at the group vs. 69 in the control group, p = 0.04), waist/hip ratio records were identified. As for May 2022, 2502 medical
beginning of the Extinction tests, with a decrease in this (0.94 in the MCI group vs. 0, 88 in the SCD group, p = 0.03) records have been analyzed, and 68 RPD patients were
freezing time over the days. Therefore, music seems to and also for the measurement of the ALi in percentage found, 46 (67.6%) of which were male. Mean age of onset
play a positive effect on short-term memory as well as value considering the extremes of cortisol (ALi % corti- was 65.5 years (SD=15.3), and mean time from onset to
on the extinction of aversive memory, since it causes this sol p<12.5 or p>87.5) (36.9% in the MCI group vs. 27.2% functional impairment was 222.3 days (SD=210.1). At
memory to be more rapidly extinguished. in the SCD group, p = 0.04). In the multivariate analysis, Hospitalization, the RPD diagnoses were: mixed demen-
age, education and economic class played a moderating tia (MD; n=16; 23.5%), vascular dementia (VD; n=15;
role. Conclusion: The present work probably represents 22.1%), nonprion neurodegenerative disorders (NPND;
Neurologia Cognitiva e do Envelhecimento
one of the first research initiatives to address measures n=9; 13.2%), autoimmune encephalopathies (AIE; n=5;
of chronic stress beyond cortisol in elderly participants 7.4%), neoplasms (NEO; n=4; 5.9%), prion diseases (PRD;
diagnosed with SCD and MCI, with increased markers n=3; 4.4%), carential diseases (CD; n=3; 4.4%), infectious
in the MCI group. Due to the cross-sectional nature of diseases (ID; n=3; 4.4%), toxic-metabolic dementia (TMD;
the study, the associations found do not allow inferring n=1; 1.5%) and genetic diseases (GD; n=1; 1.5%). No cause
causality between the measures, but they can be better was registered in 8 cases (11.8%). The revised diagnoses
explored in the longitudinal follow-up of the participants. were: NPND (n=17; 25.0%), VD (n=17; 25.0%), MD (n=8;
11.8%), AIE (n=6; 8.8%), TMD (n=4; 5.9%), NEO (n=4;
5.9%), ID (n=4; 5.9%), PRD (n=2; 2.9%), post-traumatic
Neurologia Cognitiva e do Envelhecimento dementia (n=2; 2.9%), CD (n=2; 2.9%), GD (n=1; 1.5%) and
normal pressure hydrocephalus (n=1; 1.5%). Conclusion:
the etiologies of RPD are many and tertiary center studies
may help clarify their epidemiology and clinical profile.

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XXX Congresso Brasileiro de Neurologia

TL 1105227 TL 1105384 TL 1105413

EVALUATION OF MEEM AND CDR IN AVAILABILITY AND ACCESS OF PALLIATIVE AEROBIC EXERCISE AND IMPROVEMENT
ELDERLY WITH AND WITHOUT DEMENTIA CARE IN ELDERLY WITH ALZHEIMER’S OF QUALITY OF LIFE IN PEOPLE WITH
FOLLOWED AT A HOMEOPATHY SERVICE IN DISEASE IN THE PUBLIC HEALTH SYSTEM: A ALZHEIMER’S DEMENTIA: SYSTEMATIC
RIO BRANCO, ACRE REVIEW OF THE LITERATURE REVIEW
Kauan Alves Sousa Madruga, Luiz Fernando Melo Giovana de Oliveira Sarubi, Hitesh Babani, Adria Lohana Guimarães Souza, Beatriz Rihs Matos
Lima, Leonardo Matos Santos, Juliene de Oliveira Melissa Silva Campos, Anne Larissa Silva Campos, Tavares, Brenda Santana Almeida, Murilo Sousa
Marques, Carlos Antônio de Arroxelas Silva, Ana Karolinne Cruz Cavalcante, Caroline Souza dos Ramos, Alexandre Gonzaga dos Anjos, Luciane
Milagros Leopoldina Clavijo Velazquez, Mônica da Anjos Aparecida Gonçalves Manganelli, Maria Laura de
Silva-Nunes Almeida Alves, Calila Oliveira Alves, Jane Mary de
Fametro. Manaus AM, Brazil Medeiros Guimarães, Grasiely Faccin Borges
Universidade Federal do Acre – Rio Branco – AC – Instituto Vincité. Manaus AM, Brazil
Brazil Universidade Federal do Sul da Bahia. Teixeira de
Universidade Federal de São Carlos, São Carlos SP, sarubigiovana@gmail.com Freitas BA, Brazil
Brazil Universidade de São Paulo. São Paulo SP, Brazil
Background: Dementia syndromes are among the leading
kauanmbr@gmail.com causes of death and have similar symptom control chal- lohana.souza@cja.ufsb.edu.br
lenges as cancer patients. However, dementias tend to
Background: Dementia is a clinical condition in which receive less attention in palliative care planning. According Background: Alzheimer’s dementia (AD) corresponds
there is a significant cognitive decline in relation to a pre- to the brazilian Alzheimer’s Association (ABRAZ, 2010), to 90% of dementia cases in the elderly, and is expressed
vious level, causing difficulties in occupational, social and there are approximately 1.2 million elderly people with by functional, cognitive and behavioral impairment and
domestic life. The Mini Mental State Examination (MMSE) Alzheimer’s Disease (AD). AD is characterized by a pro- consequent reduction in life quality. Because it is asso-
and the Clinical Dementia Rating (CDR) are useful tools longed course of progressive disabilities that bring a sig- ciated, among other things, with low levels of physical
that can be applied to patients with dementia for screening nificant responsibility to the public health system and exercise, they can lead to a positive effect on the many
and staging purposes, respectively. Objective: Evaluation families.Aims: Describe the availability and access of losses related to the disease. Objective: To evaluate evi-
and comparation of the results of the MMSE and CDR in palliative care for elderly Alzheimer’s patients using the dence of aerobic exercise efficacy in improving life quality
elderly patients with and without dementia, undergoing SUS(brazilian Unic health system).Methods: A litera- of people with AD. Method: A systematic review was per-
complementary homeopathic treatment. Methods: Study ture review was developed in three steps: Development formed, following the PRISMA check-list, in the SciELO,
participants were 43 elderly under follow-up for cogni- of the research question, search for scientific articles in PubMed, VHL, Medline, Cinahl, PEDro and Cochrane
tive or motor complaints, with and without dementia, the Pubmed database, and critical analysis of included databases, using descriptors. Randomized clinical trials
at Hospital do Idoso – Fundação Hospitalar do Acre. articles. The search was conducted in March 2022, and with the variables AD, aerobic physical exercises and
Dementia diagnoses were made according to well-defined articles between 2012 and 2022 were selected, for a total life quality were included. The methodological quality
etiological criteria. Participants were interviewed with of 30 articles, of which 8 were used. Results: AD is one was evaluated with the JADAD scale and the data were
individual and socioeconomic questionnaires, cognitive of the types of dementia that received the worst qual- analyzed with descriptive statistics. Results: Six articles
assessment by the MMSE and assessment of dementia ity of end-of-life medical care. It is noted that it is not were evaluated, published between 2009 and 2020. The
severity using the CDR. Data were entered and analyzed established as a routine of care in this group of patients sample consisted of 314 elderly people of both sexes,
using the SPSS 20 program. Results: The most frequent even though the recognition of terminality is proposed between 65-80 years. The studies obtained an average
diagnoses of dementia were frontotemporal dementia, as a fundamental prerequisite for better end-of-life care of 4.5 points on the JADAD quality scale. The diagnostic
Alzheimer’s dementia and vascular dementia. The results in demented patients. Effective care management for criteria were established by the DSM IV and the National
of the CDR were in agreement with those of the MMSE in demented patients can be provided from specialized Institute of Neurologic and Communicative Disorders.
24 patients with dementia and 3 patients without demen- centers to complement usual care and mitigate the grow- About 66.6% of the investigations reported mild demen-
tia. Of 16 patients classified as “questionable dementia” by ing social and economic burden of dementia. Although, tia, 33.3% moderate and 33.3% did not report the degree.
the CDR, 10 were classified as “dementia” by the MMSE it is not recognized as a protocol within the healthcare There are reports of Chronic Noncommunicable Diseases
and 6 were classified as “no dementia” by the MMSE. If system that provides outpatient care as well as, there is in 33.3% of the investigations. The presence/severity of
we consider as having dementia confirmed by the CDR no unit and facility to which patients and their families AD was analyzed by the Mini Mental State Examination,
only scores from 1 to 3, the agreement rate between the can be referred for support and better care planning. and in relation to life quality, the Quality of Lyfe-AD was
CDR and the MMSE is 76.74%. Discussion: The MMSE Conclusion: Neuropaliative care should be available on used, both used in 66.6% of the studies in the pre-test and
result worsens as the CDR staging levels increase, which the public health system(SUS) and offered to all patients post-test. Regarding the intervention, 50% of the studies
is in agreement with other studies. The data also point to who need it, regardless of diagnosis, in order to screen analyzed walking and 50% aerobic exercises of strength,
a strong influence of the level of education on the MMSE, for psychosocial factors that worsen the disease in addi- endurance, balance and flexibility, 83.4% with mild to
an association that is already well described in the litera- tion to promote better symptom control and recognize moderate intensity and 16.6% with moderate to high.
ture. In addition, in the direct comparison of the MMSE caregiver exhaustion. On average, the programs lasted from 8-12 weeks, rang-
with the degrees of the CDR, there is interference, even in ing from 2-5 weekly sessions, the most common being 3
patients without dementia, in attention, calculation and days/week (50%), and 41.3 minutes/session (±11.17). It
visuospatial ability, suggesting that these domains can be Neurologia Cognitiva e do Envelhecimento was found that 83.3% of the interventions were able to
affected early even in patients with complaints that are positively modulate the dependent variable Quality of
limited to deficits in memory. Conclusion: The results life, evidencing medium-term observation of the effects
of the present study suggest that the mean MMSE scores of aerobic exercises on the improvement of cognitive
follow a pattern of agreement in relation to the staging and functional function. Conclusion: In elderly with AD,
degrees recommended by the CDR and tend to remain aerobic physical exercises led to changes in quality of life
regular in returns, with some exceptions that can be and improvement in physical and cognitive performance.
explained by the progression of the disease, by the effect However, further investigations are needed comparing
of medications and by external factors. interventions of high relevance in a larger sample size.

Neurologia Cognitiva e do Envelhecimento Neurologia Cognitiva e do Envelhecimento

320
XXX Congresso Brasileiro de Neurologia

TL 1105501 TL 1105536 TL 1105562

ADUCANUMAB AS A NEW THERAPEUTIC MOLECULAR MECHANISMS IN DIAGNOSTIC DISCLOSURE OF ALZHEIMER’S


POSSIBILITY FOR ALZHEIMER’S DISEASE NEURODEGENERATION AND PROTEIN DISEASE IN BRAZIL: CURRENT PRACTICE
ACCUMULATION: A REVIEW OF PHYSICIANS WHO DIAGNOSE AND TREAT
Italo Felipe Cury, Anderson Moura Bernardes,
Anderssany Moura Bernardes DEMENTIA
Daniel Delgado Seneor, Luiz José Malta Gaia
Ferreira Júnior, Wladimir Bocca Vieira de Rezende Vitor Santos de Souza, Sofia Brunchport Guazzelli,
Universidade de Gurupi. Gurupi TO, Brazil
Pinto, Roberta Corrêa Ribeiro, Paulo de Lima Serano, Leonardo Cardoso Cruz, Marina Cunha Serafini,
Universidade Federal do Piaui. Teresina PI, Brazil
Rodrigo Galvão Bueno Gardona, Beatriz Castro Reis, Rafael Felipe Silva Rodrigues, Elisa de Paula França
italofelipe.cury@gmail.com Igor Braga Farias, Paulo Victor Sgobbi de Souza, Resende, Leonardo Cruz de Souza, Maira Tonidandel
Acay Souza Bulle Oliveira Barbosa, Paulo Caramelli
Background: Alzheimer’s disease (AD) corresponds to
Universidade Federal de São Paulo. São Paulo SP, Universidade Federal de Minas de Gerais. Belo
a cognitive and functional decline associated with age,
Brazil Horizonte MG, Brazil
specific neuropathology and in its earliest stages it can
be characterized by a deficit in the ability to encode and Hospital das Clínicas da Universidade Federal de
danielseneor30@gmail.com
store memories. da is characterized by deposition of Minas Gerais. Belo Horizonte MG, Brazil
amyloid-? (A?) plaques and neurofibrillary tangles in the Background: The abnormal accumulation of proteins is vitorsantosdesouza@outlook.com
brain, accompanied by neurodegeneration. Regarding a common feature of neurodegenerative diseases. Novel
therapeutic options, there is still a relevant search for information regarding the mechanism of aggregation Background: Dementia is a syndrome characterized by
improvements in the treatment of this disease, and for include dysfunction of proteins involved in membrane- cognitive and functional decline. In Brazil, the estimated
that, the path that leads to the use of anti-amyloid agents less organelles formation, which have a chemical struc- number of individuals with dementia in 2019 was 1.7
showed significant efficacy in clinical trials. Agents with ture that is prone to aggregation. Once assembled, these million, with an estimate that it will reach 3.7 million in
potential for short-term approval meet these criteria: the aggregates spread in the central nervous system through 2050. Legal, ethical and moral issues permeate diagnostic
injectable antibodies, aducanumab, gantenerumab, and a prion-like mechanism that involves the exportation of disclosure of Alzheimer’s disease (AD), the main cause of
BAN 2401, and an oral small-molecule agent, ALZ-801. seeding nuclei to nearby neurons. Objective: This work dementia, to patients and families. The right to know the
Objective: To analyze the new therapeutic possibilities in aims to review the main protein misfolding mechanisms diagnosis, the autonomy of those involved and the princi-
the treatment of Alzheimer’s Disease and to understand involved in neurodegeneration, as well as to explain the ples of non-maleficence and beneficence are mixed with
the relevance of this new therapy for patients affected by recent prion-like spreading of such misfolding patterns values and attitudes ??that often do not correlate with bio-
this disease. Methods: This is a systematic literature review throughout the central nervous system. Moreover, this ethical and legal ones. Objective: To investigate the current
based on data extraction in indexes such as PUBMED review dives into chemical structure particularities of practice of physicians who diagnose and treat dementia
and MEDLINE. Publications from 2018 to 2022 were some common proteins identified in neurodegener- in Brazil. Methods: Observational cross-sectional study.
considered, in English and with the following descrip- ative diseases. Methods: Articles produced between Electronic survey was sent to specialists who are members
tors: Aducanumab. Alzheimer’s. Neurology. Results: 2010 and 2022 were searched using keywords related to of the national medical societies of Geriatrics, Neurology
Alzheimer’s disease currently ranks as the most common the field of study. The selection of included studies was and Psychiatry (psychogeriatricians). Results: During 41
form of neurodegenerative disease, and its symptomatic based on whether the papers’ theme was neurodegener- days (5/9/2022-6/18/2022), 218 responses were obtained.
treatment is able to offer only a modest and measur- ation, protein aggregation and/or prion-like mechanism Of the total, 52.8% are men, whose main medical special-
able effect on cognition. Regarding the aforementioned of spreading. Results: The reviewed studies showed ties are Neurology (58.9%), Geriatrics (30.7%), Psychiatry
anti-amyloid agents, Aducanumab showed significant that the mechanisms of protein aggregation seem to be (6.9%), Internal Medicine (1.4%), others (2.1%). These
efficacy in clinical and biomarker results o aducanumab closely related to membraneless organelles dysfunction. professionals are aged between 26 and 84 years (mean =
atua ligando-se e reduzindo o A? solúvel e insolúvel de Such organelles have important physiological functions. 46.2 years). The majority (58.7%) of the participants have
maneira dependente da dose e com cerca de um ano de However, under certain conditions, they might form been following patients with AD for more than 10 years.
infusões intravenosas mensais de aducanumab foi capaz irreversible solid aggregates, whose toxicity is related to Concerning the disclosure of the diagnosis of AD, only
de reduzir a A? cerebral, o que resulta em uma desacel- neuronal dysfunction and death. The studied proteins 16.5% have the habit of always revealing it, 50.9% affirm
eração do declínio clínico medido. Conclusion: In this have low complexity domains that predispose them to that they usually do it, 20.6% sometimes, and 11.9% rarely.
way, it is noticed that new possibilities for the treatment of misfold, with a particular chemical structure identified. Despite this, 73.4% of physicians believe that patients, in
AD appear on the horizon and create new hopes to guar- Moreover, we discussed the prion-like mechanism the- general, want to know their diagnosis. The reasons why
antee a better quality of life and who knows even evolve ory for neurodegeneration spreading, allowing novel professionals decide to reveal or not the diagnosis of AD
to a clinical decline of this pathology. Evidently, there is approaches to this group of diseases. This theory states to their patients are related to the patient’s desire to know
still a long way to go, mainly due to the complexity of the that transference of prionic nuclei may occur among it, the opinions of the patient’s family about the disclosure,
pathophysiological factors of this comorbidity, but the neural cells, which would induce protein misfolding and the severity of dementia, which were indicated by
possibility of an effective treatment opens up new hopes. and aggregation in naive cells. Conclusion: Abnormal 73.9%, 62.4% and 56.4% of the participants, respectively.
accumulation of protein aggregates in neurodegenerative On the other hand, 96.3% of physicians would like to
disorders has been extensively studied in the last years. know their diagnosis, if they were diagnosed with AD.
Neurologia Cognitiva e do Envelhecimento Although intrinsically disordered regions have been Of these, 50.9% would like to be better prepared for the
evolutionarily selected for the assembly membraneless future. Conclusion: The diagnostic disclosure of AD by
organelles, they are related to toxic protein aggregation specialized physicians is still not very common in Brazil,
within neurons. Furthermore, the prion-like spreading which is related to several reasons. More studies and ini-
mechanism of such aggregates seems to play a role in tiatives are necessary to discuss the benefits and harms
the dissemination of neurodegeneration. Identifying that diagnostic disclosure can bring and to better under-
the mechanisms and structures involved in this process stand the demands of patients and families.
might contribute to the development of new therapeutic
approaches against such disorders.
Neurologia Cognitiva e do Envelhecimento

Neurologia Cognitiva e do Envelhecimento

321
XXX Congresso Brasileiro de Neurologia

TL 1105623 TL 1105636 TL 1105660

COGNITIVE COMPLAINTS ASSESSMENT AND ASSESSMENT OF QUALITY OF LIFE AFTER USING AN EXTERNAL CONTROL TO
NEUROPSYCHIATRIC DISORDERS AFTER MILD COVID-19 INFECTION CONTEXTUALIZE EFFICACY DATA FROM
MILD COVID-19 INFECTION PATIENTS WITH PRODROMAL AND MILD
Patricia Gomes Pinheiro, Mariana Beiral Hammerle,
Deborah Santos Sales, Débora Viana Freitas, Karina ALZHEIMER’S DISEASE TREATED WITH
Patricia Gomes Pinheiro, Mariana Beiral Hammerle,
Deborah Santos Sales, Carolina Garcia Nuñez Lebeis Pires, Clarissa de Araujo Davico, Nathalie GANTENERUMAB IN SCARLET ROAD
Carrijo, Karina Lebeis Pires, Clarissa de Araujo Souza de Andrade, Carolina Garcia Nuñez Carrijo, AND MARGUERITE ROAD OPEN-LABEL
Davico, Ana Carolina Frinhane Herzog, Daniel Lucas Pedro Ignacio Ferraz Montenegro de Almeida, EXTENSION STUDIES
de Lima Silva Santos, Elisa Gutman Gouvea, Cláudia Cláudia Cristina Ferreira Vasconcelos
Márcia Lorena Fagundes Chaves, Paul Delmar,
Cristina Ferreira Vasconcelos
UNIRIO-HUGG. Rio de Janeiro RJ, Brazil Zhiyue Huang, Anuja Neve, Gregory Klein, Geoffrey A
UNIRIO-HUGG. Rio de Janeiro RJ, Brazil Universidade Federal do Estado do Rio de Janeiro. Kerchner, Paulo Fontoura, Monika Baudler, Rachelle
Universidade Federal do Estado do Rio de Janeiro. Rio de Janeiro RJ, Brazil S Doody
Rio de Janeiro RJ, Brazil
patricia.pinheiro7@yahoo.com.br UFRGS
patricia.pinheiro7@yahoo.com.br S. Hoffmann Lá Roche LTDA – Suíça
Background: The incidence of post-COVID-19 sequelae is S. Hoffmann Lá Roche LTDA – China
Background: There are an increasing number of reports estimated between 10 and 35%. The COVID-19 infection
affects people of all ages, generates a disease burden that mchaves@hcpa.edu.br
of persistent and prolonged effects after the acute phase
of COVID-19. Damage to the CNS can have a long-term can reduce work skills, cause social, psychological and
neuropsychiatric challenges. These long?term symptoms Gantenerumab, a human monoclonal antibody tar-
negative impact on cognitive function, daily functioning,
could cause a significant effect on the Post-COVID-19 qual- geting aggregated beta-amyloid (Abeta), is a poten-
and quality of life, even several months after recovery
ity of life. Objectives: Evaluate the quality of life of patients tial disease-modifying treatment for early (prodro-
from COVID-19. Reports of acute cognitive complications,
after being affected by COVID-19, which domains are most mal-to-mild) Alzheimer’s disease (AD). SCarlet RoAD
such as attention and dysexecutive symptoms, are also
prevalent in Short Form-36 (SF36) Scale and the presence (SR) (NCT01224106) and Marguerite RoAD (MR)
emerging. Objectives: To analyze cognitive impairment
of anxiety, depression, fatigue, chronic headache, chronic (NCT02051608) were two Phase III trials with open-la-
related to long term COVID-19 and its correlation with
myalgia, ageusia and olfactory disorders after infection. bel extensions (OLEs), during which participants were
anxiety, depression, and fatigue in patients with mild
Methods: This is a cross-sectional study performed in a treated with up to 1,200 mg/month of subcutaneous gan-
COVID-19. Methods: This is a cross-sectional study per-
cohort of 143 patients with COVID-19. These patients were tenerumab for up to 5 years. This work aims to evaluate
formed in a cohort of 127 patients with COVID-19. These
regularly followed up from September 2020 to September the gantenerumab treatment effect during the SR and MR
patients were regularly followed up from September 2020
2021. Tests to screen for symptoms included the SF36 uncontrolled OLE studies by using an external control
to September 2021. Tests to screen for neuropsychiatric
which range from 0 to 100, fatigue severity scale (FSS), group based on the Alzheimer’s Disease Neuroimaging
symptoms included Mini-Mental State Exam 2 (MMSE-
Hospital Anxiety and Depression Scale (HADS) and yes Initiative (ADNI) cohort. Participants from the ADNI
2), Symbol Digit Modalities Test (SDMT), and Hospital
and no questionnaires. Results: Median age was 41 (inter- studies were weighted using the inverse probability of
Anxiety and Depression Scale (HADS). Results: Tests were
quartile range (IQR 29-50) years; 108 patients (75,5%) were treatment weighting method to make their demograph-
performed a median of seven months after COVID-19
female. In the assessment of quality of life by the SF-36, the ics and baseline characteristics more comparable to
infection, with the test application time varying between
domain most affected was the physical aspects, with an the patients in the OLE stages of the pooled SR and MR
one and 18 months. SDMT was abnormal in 22%, and the
average of 41. Then, the domain’s vitality and emotional studies. In the weighted sample, the treatment effects
BV version of MMSE-2 was abnormal in 16.5%, while the
aspects showed low performance, with an average of 45 of gantenerumab, per change from the OLE Baseline in
EV and SV versions were normal. There were significant
each one. The best scored domain was the functional CDR-SB, ADAS-Cog13, MMSE at Weeks 104 and 156 were
positive correlations between the versions of the MMSE-2
capacity, with an average of 67 points. More than a half investigated by a mixed-effect model of repeated measure.
and SDMT (p< 001 for all tests). In contrast, there were
of the sample reported cognitive impairment (55,9%) Participants (N = 164) from the SR and MR OLE studies
significant negative correlations between the different
and a minority reported chronic headache (23.1%) and and 1,218 participants from ADNI were selected. After
versions of the MMSE-2 and SDMT with depression (r=-
myalgia (11.2%). Just 35.7% complained of chronic fatigue, weighting, the demographics and baseline characteristics
2.88, p<0.001 and r=-0.397, p<0.001, respectively) and
but 71.3% scored in the FSS with the presence of fatigue. were comparable between the two populations. Relative
anxiety (r=-0.175, p=0.049 and r=-0.198, p=0.026, respec-
Regarding screening for depression and anxiety, 40.6% reductions between the pooled participants in SR and
tively). There was a significant negative correlation with
and 52.5% scored positively, respectively. Conclusion: MR OLE studies and weighted ADNI participants were
SDMT (r=-0.212, p=.017), but not with MMSE-2 (r=-0.149,
The prevalence of fatigue, anxiety and reported cognitive found for CDR-SB and ADAS-Cog13 at weeks 104 and
p=0.094). However, the presence of a greater degree of
impairment resulting from COVID-19 infection is high. 156, with greater reductions in SR and MR participants.
these neuropsychiatric conditions correlated little with
The aspects of quality of life deserve attention which are Conclusion: When comparing participants in the SR and
worse performance on the test. Conclusion: Even in those
observed in low scores in SF-36. More studies are needed, MR OLE studies to a matched ADNI control group, a slower
with a mild presentation, cognitive impairment complaints
especially regarding the causes of impact quality of life progression was observed with gantenerumab treatment.
were frequent in patients with COVID-19. Screening tests,
such as SDMT, helped confirm changes in the attentional and possible causes correlated.
domain and processing speed, especially in patients with Neurologia Cognitiva e do Envelhecimento
cognitive impairment complaints after infection. Although
manifestations such as fatigue, depression, and anxiety Neurologia Cognitiva e do Envelhecimento
were frequent in the post COVID-19 phase, especially in
those with complaints of cognitive deficits, these three
conditions that are known to contribute to cognitive
impairment showed little correlation with worse perfor-
mance on rapid screening tests.

Neurologia Cognitiva e do Envelhecimento

322
XXX Congresso Brasileiro de Neurologia

TL 1105665 TL 1105723 TL 1105747

NEUROFILAMENT LIGHT CHAIN, ?-AMYLOID ASSESSMENT OF MESIAL TEMPORAL LOBE POST-STROKE COGNITIVE ASSESSMENT
40, ?-AMYLOID 42 AND TAU LEVELS IN ATROPHY USING THE MEDIAL TEMPORAL IN PATIENTS FROM A SPECIALIZED
PARKINSON’S AND CHAGAS DISEASE LOBE ATROPHY SCORE AND HIPPOCAMPAL OUTPATIENT CLINIC
VOLUMETRY IN THE BRAZILIAN POPULATION
Andreza Salvio Lemos, Larissa Araujo Duarte, Clariana Nascimento de Oliveira, Camila Orrico
Roberta Borges Gomes Kauark, Lucy Rodrigues- Ariane Lima Fernandes, Debora Beserra Vilar, Vitor Peixoto, Tamires Vasconcelos, Nadja Santiago, Iris
Ribeiro, Eric Aguiar Wittlich, Israela Souza Brito Tumas Montaño, Jamary Oliveira Filho
Santos, Carita Victoria Carvalho de Santana,
Luciana Mattos Barros Oliveira, Soniza Vieira Alves- Faculdade de Medicina de Ribeirão Preto Universidade Federal da Bahia. Salvador BA, Brazil
Leon, Jamary Oliveira-Filho Universidade do Estado de São Paulo – Ribeirão
camilaorrico@gmail.com
Preto – Sp – Brazil
Universidade Federal do Rio de Janeiro. Rio de
Janeiro RJ, Brazil arilimafernandes@gmail.com Background: The occurrence of cognitive impairment
Universidade Federal da Bahia. Salvador BA, Brazil after stroke is already studied in the literature. 50% of stroke
Background: The reference studies to determine cut-off survivors will have some degree of cognitive impairment,
lucyrribeiro@hotmail.com parameter for biomarkers of neurodegeneration, such of these approximately 66.7% will have mild cognitive
as the medial temporal lobe atrophy (MTA) score and impairment (MCC). Some risk factors, such as the NIHSS,
Background: The use of serum biomarkers as hippocampal volumetry, in Alzheimer’s disease (AD), are associated with greater impairment. Objective: To
Neurofilament Light Chain[NfL], ?-amyloid40[?A40], were performed in high-income countries. There are assess the prevalence and predictors of cognitive dysfunc-
?-amyloid42[?A42] and Tau protein have been adressed few studies in low-income countries, and it was not tion in a stroke outpatient clinic in a reference Hospital
for diagnosis risk and prognostic assessment of neuro- found previous national research to establish the cut-off in Salvador – Bahia. Method. This is a cross-sectional,
logic disorders, as marker of neurodegeneration. It is parameter for the brazilian population. Objective: To descriptive and analytical study carried out through data
known that Chagas Disease (CD) is associated to stroke establish diagnostic criteria for the MTA score and hip- collection via medical records at the stroke outpatient
and cognitive impairment independently of cardiac dis- pocampal volumetry based on the brazilian population clinic located at the Professor Edgard Santos University
ease severity. In this context, some of serum biomarkers and to determine if there is a correlation between these Hospital Complex of the Federal University of Bahia –
may be altered in CD. Objectives: To determine if serum two techniques for measuring the hippocampus. Method: Professor Francisco Magalhães Neto Outpatient Clinic,
NfL, ?A40, ?A42 and Tau levels are altered in patients with The study is composed of 149 participants (73 controls, of patients treated during the period of January 2021
CD when compared to controls and Parkinson’s Disease 44 with mild cognitive im-pairment (MCI) and 32 with to October 2021. Among the variables analyzed are:
(PD). Methods: Cross-sectional study of adult (? 18 years) probable early-stage AD from NAPNA and Foss co-hort. Presence of systemic arterial hypertension, Diabetes
patients from a heart failure (HF) and movement disor- The automatic measurement of the hippcampal volume Mellitus, acute coronary disease, current smoking, type of
ders outpatient clinics. CD and control patients fulfilled was performed (FreeSurfer software version 6.0) and two stroke if ischemic or hemorrhagic, use of antihypertensive
Framingham criteria for HF; and had no history of stroke, independent observers assigned the MTA score. Results: drugs and which class, Pre-stroke rankin, NIHSS Stroke
recent thrombotic event or neurodegenerative diseases. Based on the AUC-ROC, it was proposed as the cut-off Scale, Anxiety and Depression Scale (HAD) and Mini
CD was defined by a positive ELISA serologic test. The value for the individuals aged < 75 years: MTAmean ?2 Mental State Examination (MMSE) scores and Results:
PD group fulfilled UK Bank criteria for PD; and had no (sensitivity (Sens: ): 61% and specificity (Spec): 85%) and of complementary exams which can be cranial tomog-
dementia or parkinsonism of any other etiologies. NfL, MTAworst ? 3 (Sens: 47% and Spec: 82%) whilst for those ? raphy or cranial resonance through which the location
?A40, ?A42 and Tau were analyzed by single molecule 75 years: MTAmean ?3 (Sens: 64% and Spec: 91%) and the of the stroke will be identified.Result. There was a 71.4%
array (SIMOA) technique. Multivariable analysis was MTAworst was not statistically significant. The cut-off by prevalence of post-stroke cognitive impairment and the
performed using linear regression adjusted by age and the AUC-ROC of volumetry showed statistical significance most important predictor was low education and prior
sex. Results: NfL was obtained from 22 patients with CD, only in the mean volume of those aged < 75 years being cognition. Conclusion: There is a high prevalence of post-
51 with PD and 24 controls. The median NfL was 7.73pg/ 3430mm³ (Sens: 56% and Spec: 88%). Considering the stroke cognitive impairment. Thus, it is important to act
ml in CD, 18.84 in PD and 8.81 in controls. NfL concen- 95% percentile of controls, the proposed normality value on early diagnosis and rehabilitation.
tration adjusted for age/sex was 13.54pg/ml higher in sugessted for indivi-duals < 75 years: a score of 0 and 1 of
PD compared to controls (p=0.006), but not different in the MTAworst and a mean volume of the hip-pocampal
CD group comparing to controls (p=0.24). ?A40 was ana- > 3013mm³ and for those ? 75years: a score of 0 to 2 of Neurologia Cognitiva e do Envelhecimento
lyzed in 11 patients with CD, 50 with PD and 6 controls. the MTA worst and a mean volume of the hippocampal
Median ?A40 was 0.73pg/ml in CD, 209.8 in PD and 3.01 > 3115mm³. The premorbid volume of the left hippo-
in controls. Adjusted ?A40 concentration was 207.07pg/ campus is lower than the right (VL = 3893mm³ vs. VR
ml higher in PD compared to controls (p<0.001) and there = 3729mm³, D de Co-hen´s D = 0.73, p <0.001). There is
was no difference of ?A40 levels in CD patients (p=0.77). moderate correlation (RS = -0.496, p<0.001) between mean
Regarding ?A42, were 14 samples of CD, 50 of PD and 5 hippocampal volumetry and MTA Scale score. Moderate
of controls. The median was 0.41pg/ml in CD, 10.29 in PD inter-observer agreement (Kappa (k) = 0.578) and sub-
and 0.75 in controls. Adjusted ?A42 was 9.54pg/ml higher stantial, nearly perfect intra-observer agreement were
in PD comparing to control (p<0.001). CD had no effect obtained from the radiologist (k = 0.674) and neurologist
on ?A42 values (p=0.70). Finally, Tau presented median of (k = 0.871) respectively. Conclusion: Considering the diag-
0.72pg/ml in CD, 2.17 in PD and 0.48 in controls, with 20 nostic performance of the Scheltens Scale, we suggested
samples in CD, 50 in PD and 15 in control. In the adjusted to use a MTAmean ?2 (Sens: 61% and Spec: 85%) in those
analysis, CD and PD had no effect on protein Tau levels aged < 75 ye-ars and a MTAmean ?3 (Sens: 64% and Spec:
comparing to controls. Conclusion: NfL, ?A40 and ?A42 91%) in those aged ? 75 years. Mo-reover, the MTA cut-
were increased in PD when compared to controls. None of off score of the brazilian population was slightly higher
the serum biomarkers were different in CD compared to than the one described on the literature and new studies
controls, suggesting that CD may affect cognition through with larger samples are needed to confirm these findings.
different pathways. The normality values based on the healthy popula-tion
studied were similar to those described in the literature.
Neurologia Cognitiva e do Envelhecimento
Neurologia Cognitiva e do Envelhecimento

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TL 1105772 TL 1105776 TL 1105851

EPIDEMIOLOGICAL PROFILE OF PATIENTS CLINICAL AND EPIDEMIOLOGICAL PROFILE ALCOHOL AS A RISK FACTOR FOR DEMENTIA
HOSPITALIZED FOR ALZHEIMER’S DISEASE OF PATIENTS DIAGNOSED WITH TRANSIENT SYNDROMES
IN BRAZIL OVER THE LAST 10 YEARS GLOBAL AMNESIA IN CASCAVEL, PARANÁ,
Andersanny Moura Bernardes, Italo Felipe Cury,
BRAZIL Anderson Moura Bernardes, Anderlanny Moura
Erick Broder Bichara, Victor Arthur Soares Costa
Araújo Luiza Orth , Gabriel Angelo Garute Zenatti , Renato Bernardes
Endler Iachinski , Eduarda Basso Badalotti , Marcia UNIRG – Gurupi – TO – Brazil
EBMSP. Salvador BA, Brazil
Bernardon , Lucas Victoy Guimarães Zengo , Vinicius Universidade Federal do Piauí. Teresina PI, Brazil
victorarthur9@hotmail.com Slongo Bühler
andersannybernardes@gmail.com
Centro Universitário da Fundação Assis Gurgacz
Background: Alzheimer’s disease (AD) is a progressive
FAG Background: Alcoholism is a chronic disorder character-
neurodegenerative disorder, which is manifested by cog-
Fundação Hospitalar São Lucas FHSL ized by long periods of abstinence associated with periods
nitive degradation. From a neuropathological point of
view, the brain of individuals presents diffuse cortical luizaorth@hotmail.com of alcohol consumption relapses. It is already known that
atrophy, neuronal loss, an excessive number of senile exposure to excess alcoholic beverages for a long period
plaques, and neurofibrillary tangles. Knowledge of the Background: Transient global amnesia (TGA) is a neu- will lead to brain damage, such as reduced concentra-
epidemiological panorama of patients Hospitalized for rological syndrome defined by sudden and severe alter- tion, recent memory and can lead to pathologies such as
this neurological pathology is of fundamental importance ation of recent memory, with resolution within hours. dementia. Dementia is a persistent cognitive syndrome,
since it is the main and most common representative of During the event, the patient has impaired memory of with decreased functionality and changes in at least two
the class of dementia, as well as being essential for the recent events, as well as becoming repetitive. However, cognitive domains. Under this question, many studies
implementation of public policies and more effective despite the temporo-spatial disorientation, the other have done analyzes on the effects of chronic alcohol use
social measures in the population-target. Since it is still a cognitive abilities remain preserved. Deficits tend to and the brain damage that may be related. Because of
recent post-COVID period, we have some greater care to resolve within 24 hours and the prognosis is favorable these studies, several pathways for this damage have been
be taken regarding this population, whereas this period in most cases. Although there is no specific treatment, suggested, including the neurotoxic effects of thiamine,
has further worsened their vulnerability and mortality. it is necessary to treat any cardiovascular risk factors ethanol, and acetaldehyde deficiency. Objetivo: Avaliar
In addition to cognitive changes, motor abnormalities that may be present. MRI can reveal a focus of hyperin- a relação entre o consumo inadequado de álcool e sua
may also be observed in AD patients. Gait disturbances, tensity in the hippocampus through diffusion-weighted relação com a progressão das Síndromes Demências,
decreased limb strength, and changes in postural control sequence (DWI). Objective: The objective of this study is investigando a quantidade e os limites entre o benefício
may be present in the initial phases of dementia or even to identify the epidemiological profile of patients who had e o dano no consumo de álcool. Methods: This is a sys-
in the pre-clinical stage of AD, thus referring to a greater TGA and were treated at a private neurology clinic in the tematic literature review based on data extraction in
need for support, especially from March 2020, due to the city of Cascavel (PR), from 2014 to 2022. Methods: This indexes such as PUBMED and MEDLINE. Publications
social distancing produced, and thus more unreported is an observational, retrospective and descriptive study between 2018 and 2022 were considered, in the English
or notified cases already in urgency. Objective: Analysis involving patients who presented transient global amne- language and with the following descriptors: Alcohol,
of the epidemiological profile of patients who required sia during the period of 2014 to 2022 and were treated at Dementia, Neurology. Results: The beginning of the dis-
Hospitalization for Alzheimer’s disease, between 2012 and a private neurology clinic in Cascavel, Paraná. The vari- cussion about the positive or negative effects of alcohol
2021, in Brazil. Methods: Quantitative and retrospective ables analyzed were sex, age, risk factors, initial symptom, is based on the lack of basic guidelines on the amount
study, performed using secondary data from DATASUS approximate duration of the symptom and changes in and frequency of alcohol intake that would be adequate
on the monthly/annual frequency of admissions related magnetic resonance imaging. Results: 19 (n =19) patients to guarantee health benefits or harm. To fill this gap, the
to AD from July/2012 to June/2021. Sex, race/color, age diagnosed with transient global amnesia were identified, research cites important points about the levels of alco-
range, the character of care, mean Hospitalization rate, with a predominance of females (78,94%, n = 15). The age hol consumption, and indicates that light to moderate
and deaths were analyzed. Results: 12246 admissions had a median of 64 years. The main symptom mentioned consumption of alcohol does not imply a high risk for
were reported, with 2382 belonging to capitals. Of the total, was “forgetfulness”, followed by “becoming repetitive” and causes of dementia, however when this intake goes to a
we have 7969 female and 4277 male Hospitalizations, the “confusion”. The duration of the symptoms had a median high level, the risks of dementia have already been shown
majority being white (5294 cases). The most affected age of approximately 10 hours. The most lasting symptom to be significantly higher. 8 drinks per week for women
group is 80 years and over (7073), with a peak in 2019 (932). had 24 hours and the most fast had three hours. MR, in and 15 drinks per week for men is found to be signifi-
Out of these, 9957 were urgent and 2289 elective cases. most cases, dia not show any alteration. In a few cases cantly associated with a consistently high trajectory of
The average spending was R$1832.23, with men gener- (n = 4), microangiopathy was evidenced. The comor- cognitive function and a lower rate of cognitive decline.
ating higher expenses (R$1921.28 versus R$1784.43), as bidities related to the patients were hypertension (n = Conclusion: Notably, the relationship between alcohol
well as elective care (R$5157.06 versus R$1067.89). The 9), Dyslipidemia (n = 3), and less often, hypothyroidism, and progression of dementia syndromes is related to the
total number of deaths was 551, led by São Paulo (185). hyperthyroidism, migraine, diabetes melitus, Anxiety, level of ethanol intake, in which low to moderate intakes
Conclusion: It is concluded that white women, 80 years old rheumatoid arthritis and arrhythmia. Only two patients present a lower risk for progression of brain damage than
and over, living in the country and under emergency care had a repeat episode of amnesia. The neurological exam people with high alcohol intakes.
are the group with the greatest vulnerability to this disease. was normal in almost all patients, except by one, who had
tremor at rest. Conclusion: Transient global amnesia is a
Neurologia Cognitiva e do Envelhecimento
syndrome whose pathophysiology is not fully understood.
Neurologia Cognitiva e do Envelhecimento Its evolution tends to be benign, and there are usually no
recurrences. It is highly related to cardiovascular risk fac-
tors, especially hypertension. More studies are needed to
understand its development.

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TL 1105856 TL 1105890 TL 1105914

PARKINSON’S DISEASE AND PSYCHOSIS: A A REVIEW OF THE LITERATURE ABOUT IMPACT OF THE SARS-COV-2 PANDEMIC
SYSTEMATIC REVIEW DEMENTIA IN AGED PATIENTS POST-COVID ON ALZHEIMER’S DISEASE PATIENTS: A
19 INFECTION RETROSPECTIVE AND EVOLUTIONARY
Andersanny Moura Bernardes, Anderson Moura
Bernardes, Italo Felipe Cury, Anderlanny Moura ANALYSIS OF COGNITIVE AND FUNCTIONAL
Ana Karolinne Cruz Cavalcante, Anne Larissa Silva
Bernardes Campos, Ádria Melissa Silva Campos, Giovana de ASPECTS
Oliveira Sarubi, Hitesh Babani, Sileno Fortes Filho Christian Max Uchôa Leite, Norberto Anízio Ferreira
Unirg – GURUPI – TO – Brazil
Universidade Federal do Piauí. Teresina PI, Brazil Centro Universitário Fametro. Manaus AM, Brazil Frota, Flávia de Paiva Santos Rolim
UNIRG – GURUPI – TO – Brazil Centro Universitário FAMETRO. Manaus AM, Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil
andersannybernardes@gmail.com karolcavalcantec@gmail.com christiannmax@hotmail.com
Background: Parkinson’s disease (PD) is a loss of dopa- Background: With the pandemic, frail elderly people, Background: Patients with Alzheimer’s Disease(AD)
minergic neurons in the substantia nigra present in the with various comorbidities and more susceptible to hav- are at greater risk not only of contracting COVID-19,
midbrain and in the development of Lewy corpuscles. The ing complications related to infection, were targeted with but of increased physical and psychological suffering
pathophysiology of PD may be related to environmental demanding restrictions and with delays in socializing. from the pandemic and greater vulnerability to accel-
factors (aging, family factors) and genetics. Diagnosis Therefore, there is a hypothesis of cognitive worsening erated functional and cognitive decline. Thus, it is nec-
is based primarily on clinical findings and can be char- in the elderly with dementia, as well as increased mani- essary to understand the clinical and social impact of
acterized by motor and non-motor symptoms, classi- festation of behavioral and psychiatric symptoms. Aims: the SARS-Cov-2 pandemic in this group. Objective: The
cally resting tremor, rigidity, bradykinesia, and hunched Analyze the impacts related to cognition in the elderly in objective was to evaluate an evolution of the cognitive
posture. When related to non-motor factors, PD can be the Covid-19 pandemic. Methods: A literature review was and functional performance of AD patients from 2018-
associated with depression, dementia, autonomic dys- developed in three steps: Development of the research 22. Methods: A retrospective cohort of 74 AD patients
function and psychosis. Parkinson’s disease psychosis question, search for scientific articles in the Pubmed treated at a tertiary Hospital in Fortaleza was carried
(PDP) encompasses minor phenomena such as illusions, database, and critical analysis of included articles. The out, from 2018-22. Cognitive assessments were carried
hallucinations, and non-visual delusions and can com- search was conducted in March 2022, and articles between out, longitudinally and retrospectively, with the Mini-
monly be associated with poorer quality of life. Objective: 2019 and 2022 were selected, for a total of 34 articles were Mental State Examination(MMSE), Semantic Verbal
To analyze the presence of Psychosis in patients with obtained of which 9 were used. Of these, 7 were cohort Fluency(SVF) and Clock Design Test(CDT); with the
Parkinson’s Disease, to point out ways to improve these studies and 2 cross-sectional studies. Results: The effect Clinical Dementia Rating Scale(CDR) and Functional
symptoms and consequently guarantee a better quality on the cognition of the elderly in isolation is evident, due Assessment Staging(FAST), in addition to assessments
of life for both the patient. Methods: This is a systematic to reduced contact with family and society, and restricted that sought to characterize neuropsychiatric symptoms,
literature review based on data extraction in indexes access to health services during the pandemic, making medication use, social isolation, caregiver stress and sat-
such as PUBMED and MEDLINE. Publications between it difficult to monitor cognitive disorders. Studies on the isfaction with telemedicine care. We used MMSE, FAST
2018 and 2022 were considered, in languages and with elderly have shown cognitive worsening and increased 2(1-17) and 3(1-7) to define significant decline; the last
the following descriptors: Parkinson disease, neurology, behavioral changes such as depression, apathy, anxiety, two being, respectively, numerical encodings of the FAST
psychosis. Results: The presence of symptoms of psy- irritability, sleep disturbance, and agitation. Conclusion: categorical variables. The evolution of the data in rela-
chosis in patients with PD are indicative of a worsening More studies are needed to better understand the wors- tion to the beginning of the pandemic was recorded as:
in quality of life, because this symptom is related to sleep ening of dementia post-covid. Furthermore, care alter- before(2018-19),after(2020-22) and during the transition
disorders, cognitive impairment, depression and, conse- natives for monitoring the elderly with dementia, such to the beginning of the pandemic(2019-20). The annual
quently, with the advancement of the disease’s pathology. as telemedicine, should be improved. evolution was then compared: 2018-19x2019-20x2020-22.
Because of this multifactorial relationship, the treatment
Results: We evidenced that in four years of follow-up there
of PDP requires investigation of the triggering symptoms,
was a functional deterioration, disproportionately between
reducing or discontinuing measurements that can induce Neurologia Cognitiva e do Envelhecimento
2019-2020; assessed by increasing the CDR from 1 to 2;
psychosis, and starting with non-pharmacological treat-
increase in FAST 2 from 4,5 to 6,5 and in FAST 3 from 4,5
ments. As for pharmacological medications that can pre-
to 6; p<0.05.The last two configured significant functional
vent this condition, clozapine remains the most indicated
decline. There was an increase in the prevalence, over the
drug for the treatment of PDP. As for pharmacological
four years, of the use of anticholinesterases, anti-NMDA
medications that can prevent this condition, clozapine
and antipsychotics(36,5x74%; 20x46%; 19x45%, respec-
remains the most indicated drug for the treatment of PDP.
tively) and their side effects. We also found a higher
Conclusion: It is concluded that the way to treat psychosis
prevalence of urge-incontinence(1,4x38%) and of some
in patients with PD involves the need to ensure quality
neuropsychiatric symptoms, such as anxiety symptom-
of life for patients. As there is no complete cure for the
s(35x58%) and insomnia(18x40,5%). Cognitive worsening
condition, what becomes important in these conditions
was evidenced, disproportionately between 2019-2020;
is to delay the evolution of the pathogenesis so that the
through the MMSE, SVF and CDT(15x11; 6x4,5; and 2x0,
patient has a longer and better survival.
respectively; p<0,05), the first of which configured signif-
icant cognitive decline. There was a significant increase
Neurologia Cognitiva e do Envelhecimento in caregiver stress(11x74%). Conclusion: Patients with
AD showed significant cognitive and functional decline
after the beginning of the pandemic.

Neurologia Cognitiva e do Envelhecimento

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TL 1105933 TL 1105935 TL 1105949

ACOMETIMENTO NEUROLÓGICO E FRANK’S SIGN AS A PREDICTOR OF ANALYSIS OF THE EPIDEMIOLOGICAL


COGNITIVO NA COVID-19: CARACTERIZAÇÃO CARDIOVASCULAR DISEASE AND COGNITIVE PROFILE OF HOSPITALIZATIONS IN PUBLIC
CLÍNICA E EPIDEMIOLÓGICA IMPAIRMENT IN AN ELDERLY POPULATION: HEALTH SYSTEM OF BRAZIL FOR DEMENTIA
A CROSS-SECTIONAL STUDY BETWEEN 2019 AND 2021
José Wagner Leonel Tavares-Júnior, Danilo Nunes
Oliveira, Manoel Alves Sobreira-Neto, Pedro Braga- Rubén David dos Reis Zuniga, Roseli Corazzini, Francisco Matheus Oliveira de Carvalho, Pedro
Neto Izadora Fonseca Zaiden Soares, Alzira Alves de Lucas Grangeiro de Sá Barreto Lima, Lucas Soares
Siqueira Carvalho Radtke, Emanuel de Assis Bertulino Martins
Universidade Federal do Ceará. Fortaleza CE, Brazil
Gomes, David Augusto Batista Sá Araújo, Geovanni
FMABC . Santo André SP, Brazil Guimarães Bandeira, Pedro Helder de Oliveira
wagnerleoneljr@gmail.com
rubencito1231@hotmail.com Junior
Background: COVID-19 neurological manifestations were
Universidade Federal do Ceará. Fortaleza CE, Brazil
demonstrated during the pandemic, including cognitive Background: Frank’s sign consists of a diagonal crease Hospital Universitário Walter Cantídio. Fortaleza
impairment. Objectives: To determine the prevalence of in the earlobe that extends from the tragus to the inferi- CE, Brazil
cognitive and behavioral complaints (such as dementia, or-posterior edge of the auricle. First described as a pre-
MCI or SCD) in a outpatient sample with recent SARS- dictor of cardiovascular diseases (CVDs), it was related to fmatheus.oc@gmail.com
COV2 infection. Specific: Evaluate the association of atherosclerosis, being later considered an independent
cognitive impairment with the presence of the polymor- cardiovascular risk factor and associated with cerebro- Background: Dementia is a clinical syndrome charac-
phism found in the APOE gene and with respiratory dis- vascular events and cognitive impairment. Objective: terized by gradual cognitive decline, involving at least
ease Methods: Observational, longitudinal, prospective To assess the prevalence of Frank’s sign in a general two cognitive domains, associated with impaired func-
clinical study. Inclusion criteria: patients with confirmed population aged 60 years or older regardless of the pres- tionality. Dementias usually have a progressive course,
Covid-19. Patients are evaluated in an outpatient clinic. ence of cardiovascular risk factors and to evaluate the which leads to disability in different timing and degrees,
They are evaluated through a standardized attendance presence of this sign as a predictive factor of CVDs and and is associated with infectious and vascular com-
record, with somatic and cognitive neurological assess- cognitive impairment (CI). Method: Through a conve- plications, which often lead these patients to Hospital
ment. Cognitive assessment involves the application of nience sampling, we randomly recruited patients aged services. Objective: To analyze the epidemiological
cognitive (ACER, MMSE and CDR), functional (Pfeffer) 60 years or older, not Hospitalized, from health services profile of Hospitalizations for dementia in the SUS in
and psychiatric (GDS or Beck) screening instruments, not related to cardiovascular affections from ABC Region Brazil between the years 2019 and 2021. Methods: The
in addition to subsequent extensive neuropsychological and Itapecerica da Serra, in Greater São Paulo, who had research is an observational and cross-sectional study of
assessment. In addition, APOE polymorphism is analysed. their ears photographed in profile, responded to either the Hospitalizations for dementia in the SUS in the years 2019
Preliminary Results: To date, 225 patients were evaluated. Mini Mental State Examination or the Montreal Cognitive to 2021. The collection was carried out in the DATASUS
The average age is 46.5 years, with 65.4% female, 79.16% Assessment depending on years of education, and a ques- database, with investigation of the variables: region,
with 8 or more years of schooling, in addition to 57.5% of tionnaire with epidemiological and clinical variables. age group, gender, number of Hospitalizations, total
the sample with cognitive complaints. Conclusion: The This study was conducted according to the Strengthening value, mortality rate and days of stay. Results: In the
Results: so far in our study demonstrate that cognitive the Reporting of Observational Studies in Epidemiology period, there were 7908 Hospitalizations with 56% of
complaints are frequent in patients even in the chronic (STROBE) Statement guidelines. Results: We evaluated cases occurring in the Southeast. The largest shares of
phase of the disease. 500 individuals, wich 57% were women; 57% were white, Hospitalizations were between 60 and 69 years (16.7%),
39% were black and 4% were asian; 55% had between 60 70 and 79 years (23%), and more than 80 years (26.2%).
to 69 years; 64% had the sign; Frank’s sign was associated The number of Hospitalizations was highest in the first
Neurologia Cognitiva e do Envelhecimento with older people (p=0.015), white population (p<0.001), year, with 2963 cases, while it was lower in the following
women (p<0.01), dyslipidemia (p=0.044) and had a ten- year with 2421 cases and 2524 in the third. The average
dency of association with hypertension (p=0.058), while values per Hospitalization were R$5381, R$6323, and
there was no association with CI neither cardiovascular R$5847 in 2019, 2020, and 2021, respectively. Total costs
events as myocardial infarction or stroke. Conclusion: were R$46M, mostly represented by the 40 to 49 year old
Frank’s sign prevalence is more frequently found in white age group, whose expenses were R$12.1M. The average
elderly women and doesn’t seem to be a good predictor mortality rate was 11.2% for the period, with higher rates
of CVDs neither CI, however, it showed effect with car- in the Southeast. The group between 40 and 49 years had
diovascular risk factors. Prospective studies should be more days of Hospitalization in each year, with 26.7% of
taken to assure whether this sign is a marker for CVDs the total, followed by the group over 80 years, which had
in this population. 16.8% of the days. Conclusion: From the analysis of the
number and mortality rate of cases, it is suggested that
the long-term complications of dementias, which culmi-
Neurologia Cognitiva e do Envelhecimento nate in the Hospitalization of these patients, are a major
health problem, associated with high total costs and
values per Hospitalization still higher than the average
monthly income for the period, and further studies are
needed to measure the impact of the condition in the
country. Furthermore, the preponderant regions were the
Southeast and South, despite the population of the latter
being smaller than the Northeast in numbers. Interestingly,
it was shown that the groups with more Hospitalizations
did not correspond to higher total expenditures or length
of stay, even when analyzing the year individually.

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TL 1105988 TL 1106068 TL 1106075

RECRUITMENT ANALYSIS BY MEDIA IN A KNOWLEDGE AND ATTITUDES ABOUT INFLUENCE OF DEPRESSION,


STUDY OF SUBJECTIVE COGNITIVE DECLINE DEMENTIA OF PRIMARY CARE PHYSICIANS INDEPENDENCE IN ACTIVITIES OF DAILY
OF RIO GRANDE DO SUL: PRELIMINARY LIVING AND CLINICAL VARIABLES ON THE
Victória Tizeli Souza, Simone da Mota, Bruno de
Marchi, Carolina Rodrigues Formoso, Gabriela Paz, ANALYSIS QUALITY OF LIFE OF ELDERLY PEOPLE WITH
Haniel Bispo de Souza, Lucas Bastos Beltrame, TYPE 2 DIABETES MELLITUS
Daiana Perin, Laura Ferraz, Marcelo Rodrigues
Samuel Masao Suwa, Wesley Slaviero, Raphael Gonçalves, Márcia Lorena Fagundes Chaves, Marta Rodrigues de Carvalho, Alexandra Rubim
Machado Castilhos Raphael Machado Castilhos Camara-Sete, Maria Rita Carvalho Garbi Novaes,
Serviço de Neurologia, Hospital de Clínicas de Porto Alfredo Nicodemos da Cruz Santana
Programa de Pós-graduação em Ciências Médicas,
Alegre Universidade Federal do Rio Grande do Sul Instituto Hospital de Base. Brasília DF, Brazil
Faculdade de Medicina, Universidade Federal do Secretaria Estadual de Saúde do Estado do Rio Secretaria de Estado de Saúde do Distrito Federal.
Rio Grande do Sul Grande do Sul Brasília DF, Brazil
raphael.castilhos@gmail.com Programa de Pós-graduação em Epidemiologia, Escola Superior de Ciências da Saúde. Brasília DF,
Universidade Federal do Rio Grande do Sul Brazil
Background: Subjective Cognitive Decline (SCD) is con- Serviço de Neurologia, Hospital de Clínicas de Porto
Alegre marta_rodrigues12@hotmail.com
ceptualized as cognitive complaints in cognitively unim-
paired (CU) individuals and is associated with progressive raphael.castilhos@gmail.com Background: Considering the currently increasing num-
cognitive impairments. Recruiting these individuals can
ber of elderly individuals in society, it is necessary to
be challenging, especially because of the low education Background: Knowing the knowledge and attitudes about emphasize that these individuals deserve to grow older
levels and limited access to information by a portion of dementia of primary care physicians (PCP) in the state of with autonomy and good quality of life (QoL). Objective:
the brazilian population. Objective: We aimed to analyze Rio Grande do Sul (RS) is essential for the establishment To identify the factors, especially modifiable ones, that
the initial recruitment process through media in an ongo- of educational measures. Objectives: To assess knowledge affect the quality of life (QoL) of elderly individuals with
ing study about SCD at the Hospital de Clínicas de Porto attitudes about dementia among PCP in RS. Methods: A type 2 diabetes mellitus (EIDM2). Method: A cross-sec-
Alegre (HCPA). Methods: We analyzed the recruitment form was sent by e-mail to the physicians of primary care tional study evaluated EIDM2 with emphasis on QoL,
process through electronic (HCPA website and Twitter) in RS. We collected sociodemographic data, volume of sociodemographic and clinical data, including depression
and written local media of the participants in the study patients with dementia treated and perception of diffi- (geriatric depression scale 15), family functionality/dys-
“Subjective Cognitive Decline and Cognitive Reserve: a culties in caring for these patients. A previously validated function (APGAR questionnaire), activities of daily living
multimodal approach”. This study evaluated CU individu- questionnaire was sent: “Quiz on Knowledge and Attitudes (KATZ index). Multiple linear regression was performed
als aged 65+ years-old, with educational levels < 5 or >10 in Dementia”. In addition to the descriptive analysis and to assess the impact of factors on the five domains of QoL
years and with cognitive complaints. Exclusion criteria comparison of data between the health macro-regions of (WHOQOL-BREF). Results: Two hundred and fifty-three
were the presence of uncontrolled psychiatric or clinical the state, we performed principal components analysis EIDM2 were assessed for possible study enrollment. The
illness, cerebrovascular disease, and the use of psychotro- in the results of the attitude quiz. Results: From March to mean age was of 69.04 (± 6.52) years; the mean diagnostic
pic drugs. The recruitment was carried out exclusively by April 2022, 147 PCP (from 135 municipalities) responded time of DM2 was 15.49 (± 9.86) years; the highest mean
phone calls in two phases. In the first one, the exclusion to the form. About half were women (51%; n=75) and the score (on WHOQOL-bref) was in “social relationships”do-
criteria were applied and, in the second, the following median age (interquartile range – IQR) was 35 (30-42) main of 70.00 (± 15.92); and the lowest mean score in was
scales were performed: Modified Telephone Interview for years-old. Most worked in Family Health Strategy teams “physical”domain of 60.74 (± 16.92). 67.9% of the individ-
Cognitive Status (TICS-M); Generalized Anxiety Disorder (93.9%) and the time as a physician was 7 (4-14) years. uals were females; 88.9% of the elderly individuals con-
Assessment (GAD-2); Geriatric Depression Scale; and Most had training in Family and Community Medicine sidered religion to be important; 48.8% of them said that
the Subjective Cognitive Decline Scale. Results: From (40.8%) or had no specialization (40.1%). The Missioneira they were not financially self-sufficient; 72.2% had their
February to May 2022, 169 individuals presented inter- (Northwest) region (21.8%) was the one with the most self-perceived health status as NOT good; 82.5% received
est in participating in the study. Most were women (74%; responses and the South region (7.5%) had the least. polypharmacy; 40.1% of them had high HbA1c levels;
n=125), with a median age of 71 years-old [interquartile Most physicians see less than 5 patients with suspected 86.9% of the elderly reported absent family dysfunctions;
range (IQR): 67-74], a median of 11 years of formal school- dementia per month (71.4%) and 27.2% said refer less 71.4% showed absent dependence for activities of daily
ing (IQR: 8.5-16), and most lived in Porto Alegre (71.6%; than 5 patients/month to tertiary care. The main reasons living; 71.4% of them had absent depression. Factors that
n=121). Most of the exclusions in phone call 1 were due for referral were difficulty in drug management (35.4%) positively affected QoL were: absent depression (in all
to clinical illness (14.8%; n=25), cerebrovascular disease and behavioral difficulties (29.9%). The Mini Mental State five domains), absent family dysfunction (in 3 domains),
(5.9%; n=10), and off-target education (4.7%; n=8). The Examination was the most used test, both as a screening and absent dependence on activities of daily living (ADL)
majority (62.7%; n=106/169) went to phone call 2 and, (65.3%) and in clinical suspicion (79.6%). Less than half (in 3 domains). Conclusion: The modifiable factors that
at the moment, 78 individuals were assessed. Of these, of the physicians perform cognitive screening (47.6%), influenced QoL of EIDM2 were depression and family
most were excluded because presented a TICS-M score mainly because of the patient’s complaint (66%) and the functionality. Furthermore, to our knowledge, this is the
below cut-off (44.9%; n=35/78) and absence of consistent presence of a family history of dementia (38.8%). The mean first study to show the importance of family functionality
cognitive complaints (23%; n=18/78). Conclusion: Most percentage of correct answers in the knowledge quiz was in QoL of EIDM2. Although the most feasible approach
individuals recruited by electronic and written media were 42.9%, with no difference between the regions of RS. In the to improve QoL is probably to intervene in the above
women with a high educational level. The main causes of attitude quiz, the principal component analysis identified mentioned modifiable factors, it needs to be assessed
exclusion were cognitive decline and the absence of con- 2 factors: 1) frankly positive attitudes towards patients by other prospective studies.
sistent cognitive complaints. This assessment reveals the with dementia and 2) speed in revealing the diagnosis,
difficulty in recruiting SCD individuals through media and but with a greater tendency towards referral. Conclusion:
indicates the need for alternative recruitment methods Knowledge about dementia is low among PCP in RS; how- Neurologia Cognitiva e do Envelhecimento
Neurologia Cognitiva e do Envelhecimento ever, most have positive attitudes towards these patients.
Increasing the sample size may provide information about
differences between regions in the state.

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TL 1106081 TL 1106110 TL 1106144

COGNITIVE DECLINE AND POSSIBLE ANALYSIS OF THE DISTRIBUTION OF DRUGS SUBJECTIVE COGNITIVE DECLINE AND
BIOMARKERS IN RHEUMATOID ARTHITIS: A FOR DEMENTIA DUE TO ALZHEIMER’S COGNITIVE RESERVE PROJECT: PROTOCOL
SYSTEMATIC REVIEW DISEASE IN THE STATE OF RIO GRANDE DO AND PRELIMINARY RESULTS
SUL
Daniela Cabral Sousa, Samuel Brito Almeida, Jarbas Manuella Edler Zandoná Giordani, Ana Letícia
Sá Roriz Filho, Thiago Holanda Freitas, Pedro Braga Ana Laura Brandi, Maísa de Marco, Analuiza Amorim de Albuquerque, Matheus Strelow, Wyllians
Neto Camozzato, Bárbara Krug, Paulo Dorneles Picon, Vendramini Borelli, Giovanna Carello-Collar, Victória
Márcia Lorena Fagundes Chaves, Raphael Machado Tizelli Souza, Maila Rossato Holz, Eduardo Zimmer,
Universidade Federal do Ceará. Fortaleza CE, Brazil Márcia Lorena Fagundes Chaves, Raphael Machado
Castilhos
Centro Universitário Christus. Fortaleza CE, Brazil de Castilhos
Faculdade de Medicina, Universidade Federal do
danicsousa12@gmail.com Universidade Federal do Rio Grande do Sul
Rio Grande do Sul
Programa de Pós-Graduação em Medicina: Ciências Hospital de Clínicas de Porto Alegre
Background: Cognitive dysfunction is a frequent com- Escola de Medicina da Pontifícia Universidade
Médicas, Universidade Federal do Rio Grande do Sul
plaint in patients with autoimmune disease and associ- Católica do Rio Grande do Sul. Porto Alegre RS,
Departamento de Psiquiatria, Universidade Federal
ated with impaired quality of life and loss of functionality. Brazil
de Ciências da Saúde de Porto Alegre
During the last years, a growing number of studies have
Secretaria Estadual de Saúde, Rio Grande do Sul
investigated the link between cognitive dysfunction and manuella.edler.zandona@gmail.com
Hospital de Clínicas de Porto Alegre
Rheumatoid Arthritis (RA), highlighting the potential
Programa de Pós-Graduação em Medicina: Ciências
pathogenic role of several clinical, psychological, and Background: Subjective Cognitive Decline (SCD) is a
Médicas, Universidade Federal do Rio Grande do
biological factors. Objective: Considering the scarce condition characterized by consistent cognitive com-
Sul / Serviço de Neurologia, Hospital de Clínicas de
literature on neuropsychiatric manifestations in RA, plaint in cognitively unimpaired (CU) individuals and
Porto Alegre
as well as the lack of serological markers that can help is associated with progressive cognitive impairment,
in clinical practice, we aimed to investigate serological ana__brandi@hotmail.com especially in the context of Alzheimer’s disease (AD).
and CSF biomarkers in humans, and its association with Cognitive Reserve is the capacity to maintain cognitive
cognitive dysfunction in patients with RA. Methods: We Background: Drugs for dementia due to Alzheimer’s abilities despite pathological damage. Studying the rela-
performed a systematic review using PRISMA protocol. A disease (AD) have been part of the SUS Specialized tionship between these two concepts is fundamental to
systematic search was conducted in the PubMed/Medline, Pharmaceutical Care Component (SPCC) since 2005 understand the early stages of AD. Objectives: We aimed
Embase, LILACS, Scopus and Google Scholar databases and are provided free of charge as long as the request to show the study protocol and the preliminary Results:
from inception up to November 2021.The inclusion cri- for these drugs meets the criteria of the national guide- of the study. Methods: CU individuals with > 65 years
teria for studies were defined based on the participants lines. Assessing the profile of this distribution can reveal old with cognitive complaints and with less than 5 or
involved, type of exposure, type of comparison group, inequalities in the supply of these drugs and, indirectly, more than 10 years of formal education were recruited.
outcome of interest and study design. Results: Five origi- in the quality of care for Alzheimer’s patients. Objectives: Exclusion criteria were the previous diagnosis of demen-
nal studies were included, which provided data from 428 To evaluate the prescription pattern and the profile of tia, uncontrolled neuropsychiatric/clinical illness, or
participants. The studies included plasma proteins, auto- patients who receive prescription of medication for AD cerebrovascular disease. The assessment was performed
antibodies, cytokines, interleukins, neurotrophic factors in the state of RS. Methods: All approved requests for exclusively by phone calls. It consisted of a brief cogni-
and lymphocyte subpopulations. Among plasma proteins, donepezil, rivastigmine, galantamine, and memantine for tive (Modified Telephone Interview for Cognitive Status
SHH was increased and TTR was reduced in patients with the SPCC in the state of RS between July and November – TICS-M) and anxiety/depression screenings, in addi-
mild cognitive impairment; Anti -MBP and Anti-MOG 2021 were evaluated. Patients’ sociodemographic data, tion to the SCD-scale. Individuals recruited were then
negatively correlated with memory, executive function scores on the Mini Mental State Examination (MMSE) evaluated in person with an extensive cognitive battery.
and attention. S100? negatively correlated with memory and Clinical Dementia Rating Scale (CDR), type and dose Blood, cerebrospinal fluid, and saliva were collected to
and executive functions; some lymphocyte subpopula- of the prescribed medication, municipality and health measure AD biomarkers (?-amyloid-42, total/phosphor-
tions positively correlated with attention, memory, and macro-region of origin were obtained. In addition, the ylated tau), APOE genotyping and magnetic resonance
executive functions. IL-2, IL-4, IL-6 and TNF human development index (HDI) and the prescription imaging were also performed. Results: Between March
index (patients/100,000 population) of each macro-re- and April 2022, 11 patients were included (90.1%, n=10/11
gion were associated. Results: In the second half of 2021, were female). Their median interquartile range (IQR) age
Neurologia Cognitiva e do Envelhecimento 2382 AD patients were receiving some specific AD med- was 70.5 (68.3-75) years old, with a median of 16 years of
ication in RS. Most were female (65.5%) and the median formal education for those in the high education group
age (interquartile range – IQR) was 79 (73.5-84) years. and 4 years for those with low education. The median
Most patients had education less than 8 years (n=1266; TICS-M and SCD scores at the recruitment stages were 25
71.7%). The median MMSE score was 15 (12-18) and most (IQR 20.8-26.5) and 8.5 (IQR 7-9), respectively. The main
had CDR 2 (47.5%) or 1 (34.8%). In most municipalities, SCD-plus criteria reported were age at onset of SCD ? 60
339 (68.2%), there were patients with some prescrip- years old (100%, n=11/11) and concerns associated with
tion. The macro-regions “Serra” (37/100,000) and Norte SCD (72.7%, n=8/11). Clinical Dementia Rating (CDR)
(33/100,000) had higher prescription indices and the Sul global score was 0 in most patients (81.8%, n=9/11) – only
region had the lowest (7/100,000). There was a strong cor- 2 scored 0.5 (18.2%). In the cognitive battery, deficits were
relation between prescription index and HDI (Rho=0.877; observed mainly in the Digit Span Memory Test (45.5%,
p<0.009, Spearman). The most prescribed drugs were n=5/11) and in the Five Digit Test (up to 54.5%, n=6/11).
donepezil (n=1014; 42.6%) and rivastigmine (n=691; Conclusion: Most of the patients included had the diag-
29%), and 464 (19.5%) patients were using memantine nosis of SCD, showing that the telephone screening might
association. The “Centro-Oeste” macro-region showed be effective. The expansion of the sample may help us
a higher frequency of patients with CDR=2 (61.2%) and better understand the impact of cognitive reserve on the
a higher frequency of rivastigmine prescriptions (42.1%) early stages of AD.
compared to other regions. Conclusion: The analysis of
the profile of patients with AD who receive medication
for the disease in the state of RS reveals some inequality, Neurologia Cognitiva e do Envelhecimento
with less developed regions showing lower frequency
of prescription, and prescription for patients in more
advanced stages.

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328
XXX Congresso Brasileiro de Neurologia

TL 1106197 TL 1106216 TL 1106219

IMPACT OF THE SARS-COV-2 PANDEMIC IMPACT OF THE SARS-COV-2 PANDEMIC ON IMPACT OF THE SARS-COV-2 PANDEMIC ON
ON ALZHEIMER’S DISEASE PATIENTS: A PATIENTS WITH ALZHEIMER’S DISEASE: PATIENTS WITH ALZHEIMER’S DISEASE:
RETROSPECTIVE AND EVOLUTIONARY A RETROSPECTIVE AND EVOLUTIONARY A RETROSPECTIVE AND EVOLUTIONARY
ANALYSIS OF COGNITIVE AND FUNCTIONAL ANALYSIS OF THE DEGREE OF ANALYSIS OF NEUROPSYCHIATRIC ASPECTS
ASPECTS SATISFACTION WITH TELECARE
Christian Max Uchôa Leite, Arthur Holanda Moreira,
Christian Max Uchôa Leite, Arthur Holanda Moreira, Christian Max Uchôa Leite, Arthur Holanda Moreira, Bruno Diógenes Iepsen, Norberto Anízio Ferreira
Bruno Diógenes Iepsen, Norberto Anízio Ferreira Bruno Diógenes Iepsen, Flávia de Paiva Santos Frota, Flávia de Paiva Santos Rolim
Frota, Flávia de Paiva Santos Rolim Rolim, Norberto Anízio Ferreira Frota
Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Hospital Geral de Fortaleza. Fortaleza CE, Brazil Hospital Geral de Fortaleza. Fortaleza CE, Brazil Universidade de Fortaleza. Fortaleza CE, Brazil
Universidade de Fortaleza. Fortaleza CE, Brazil
aholanda427@gmail.com aholanda427@gmail.com
aholanda427@gmail.com
Background: Alzheimer’s Disease (AD) is a neurodegen- Background: Alzheimer’s Disease (AD) is a neurodegen-
erative disorder that manifests as deterioration of multiple Background: Telemedicine allows Alzheimer’s Disease erative disorder that manifests as a variety of neuropsy-
cognitive domains and impairment of activities of daily (AD) patients to overcome barriers of access to health chiatric symptoms. Such patients are at greater risk not
living, for example. Such patients are at greater risk not care services and increases accessibility for people with only of contracting COVID-19, but of increased physical
only of contracting COVID-19, but greater vulnerability to cognitive and functional impairments. Objective: This and psychological suffering from the pandemic. Several
accelerated functional and cognitive decline. Objective: study aims to evaluate the feasibility of telehealth activ- studies carried out during this period showed the neg-
The objective was to evaluate an evolution of the cognitive ity in the follow-up of patients with AD, focusing on the ative biopsychosocial consequences of isolation mea-
and functional performance of AD patients from 2018 to level of satisfaction with telemedicine care. Methods: A sures. Objective: Evaluate neuropsychiatric conditions
2022. Methods: A retrospective cohort of 74 AD patients retrospective cohort of 74 AD patients treated at a ter- in AD outpatients in Tertiary Hospital during covid-19
treated at a tertiary Hospital in Fortaleza was carried tiary Hospital in Fortaleza was carried out, from 2018 pandemic from 2018 to 2022. Methods: A retrospective
out, from 2018 to February 2022. Cognitive assessments to February 2022. Throughout the cohort of the present cohort of 74 AD patients treated at a tertiary Hospital in
were carried out, longitudinally and retrospectively, with study, questions were asked about telemedicine satisfac- Fortaleza was carried out, from 2018 to February 2022.
the Mini-Mental State Examination (MMSE), Semantic tion. Results: Telemedicine was adopted as a modality Assessments were performed that sought to identify and
Verbal Fluency (SVF) and Clock Design Test (CDT); with in consultations for many patients during the pandemic. characterize neuropsychiatric symptoms. The evolution
the Clinical Dementia Rating Scale (CDR) and Functional Throughout the cohort of the present study, we observed of the data in relation to the beginning of the pandemic
Assessment Staging (FAST). We used MMSE, FAST 2 that 35 patients were seen at some point (2020-2021/22) was recorded as: before (2018 to 2019), after (2020 to
(1-17) and 3 (1-7) to define significant decline; the last by telemedicine; and that 39 had exclusively face-to-face 2021/22) and during the transition to the beginning of the
two being, respectively, numerical encodings of the FAST consultations. In between the 39 patients treated by tele- pandemic (2019 to 2020). The annual evolution was then
categorical variables. The evolution of the data in rela- medicine, 29 (83%) patients, caregivers or family members compared: 2018 to 2019 versus 2020 to 2021/22; 2018 to
tion to the beginning of the pandemic was recorded as: interviewed reported having enjoyed the service and that 2019 versus 2019 to 2020; and 2019 to 2020 versus 2020
before (2018 to 2019), after (2020 to 2021/22) and during it was satisfactory to their demands. Regarding the scores to 2021/22. Results: We observed, when comparing 2018
the transition to the beginning of the pandemic (2019 to for telemedicine consultations, 81% of the interviewees with 2021/22, an increase in all neuropsychiatric symp-
2020). The annual evolution was then compared: 2018 to assigned a grade of 8 or higher: 29% rated the service with toms evaluated. Hallucinations ranged from 14.9% to
2019 versus 2020 to 2021/22; 2018 to 2019 versus 2019 to a grade 10 (ten), 23% with 9 (nine) and 29% with 8 (eight). 35.1%; depressive symptoms from 20.3% to 37.8%; and
2020; and 2019 to 2020 versus 2020 to 2021/22. Results: However, 3% of respondents assigned grade 0 (zero), 3%, anxious symptoms from 35.1% to 58.1%. The two most
We evidenced that in four years of follow-up there was a grade 4 (four), 9%, grade 5 (five) and 6%, grade 7 (seven). prevalent neuropsychiatric symptoms in 2021/22 were
functional deterioration, disproportionately between 2019 Conclusion: This telehealth intervention promoted high anxious (58.1%) and depressive (37.8%) symptoms. As
and 2020; assessed by increasing the CDR from 1 (0,5-2) levels of satisfaction during the isolation of the pandemic. for the sleep changes, we observed an increase, between
to 2 (1-3); increase in FAST 2 from 4,5 (3-6) to 6,5 (4-9) 2018-2021/22, in insomnia (from 17.6% to 40.5%); and
and in FAST 3 from 4,5 (3-6) to 6 (4-6); p<0.05. The last hypersomnolence (from 0% to 13.5%). Conclusion: The
two configured significant functional decline. Cognitive Neurologia Cognitiva e do Envelhecimento social isolation provided by the covid-19 pandemic led to
worsening was evidenced, disproportionately between the worsening of neuropsychiatric disorders.
2019 and 2020; through the MMSE, SVF and CDT scores
(15 versus 11; 6 versus 4,5; and 2 versus 0, respectively;
p<0,05), the first of which configured significant cognitive Neurologia Cognitiva e do Envelhecimento
decline. Conclusion: Patients with AD showed significant
cognitive and functional decline after the beginning of
the pandemic. The follow-up of these patients is neces-
sary, aiming to improve the long-term extension of the
effects of the pandemic in this known vulnerable group.

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XXX Congresso Brasileiro de Neurologia

TL 1106241 TL 1106259 TL 1106318

COVID19 AND COGNITIVE IMPAIRMENT – A THE ADDENBROOKE’S COGNITIVE VALIDATION OF THE ALTERNATIVE VERSION
FOLLOW-UP STUDY OF A CLINICAL HOSPITAL EXAMINATION–REVISED (ACE-R) IN THE OF THE BRAZILIAN MONTREAL COGNITIVE
IN SÃO PAULO DIAGNOSIS OF ALZHEIMER’S DISEASE: A ASSESSMENT (MOCA-BR): PILOT STUDY
NEW WAY TO EXPLORE ITS DIAGNOSTIC
Raphael Ribeiro Spera, Raphael de Luca e Tuma, Valmir Vicente Filho, Alana Gomes Pecorari, Paulo
Júlia Chartouni Rodrigues, Satiko Andrezza Takano PROPERTIES Henrique Ferreira Bertolucci, Viviane de Hiroki
Peixoto, Eliane Correa Miotto, Artur Martins Novaes Viviane Amaral-Carvalho, Thais Bento Lima-Silva, Flumignan Zetola
Coutinho, Sônia Maria Dozzi Brucki Luciano Inácio Mariano, Leonardo Cruz de Souza, Universidade Federal do Paraná. Curitiba PR, Brazil
Hospital das Clínicas da Faculdade de Medicina da Henrique Cerqueira Guimarães, Valéria Santoro Universidade Federal de São Paulo. São Paulo SP,
Universidade de São Paulo. São Paulo SP, Brazil Bahia, Ricardo Nitrini, Maira Tonidandel Barbosa, Brazil
Mônica Sanches Yassuda, Paulo Caramelli
raphaelspera@gmail.com valmirvicentef@gmail.com
Faculdade de Medicina da Universidade de São
Background: Post-COVID19 syndrome is a condition Paulo Background: The current version of the brazilian Montreal
characterized by signs and symptoms that occur within 3 Universidade Federal de Minas Gerais. Belo Cognitive Assessment (MoCA-BR) did not have a reliable
months of the onset of COVID19 and that last for at least Horizonte MG, Brazil cross-cultural adaptation to brazilian Portuguese. In pre-
2 months. In the past 2 years, cognitive impairment has Faculdade de Medicina da Universidade Federal de vious stages of this study, the Alternative Version of the
frequently been associated with COVID19 with descrip- Minas Gerais. Belo Horizonte MG, Brazil MoCA-BR was developed, with changes in the sections:
tions of attentional, executive, memory, and language vivianeamaralc@hotmail.com Memory and Delayed Recall, Language and Naming.
disorders. Many studies have assessed these cognitive Objectives: to verify the influence of cross-cultural adap-
disturbances using online and telephone tests, often Background: The ACE-R is an accurate and brief cogni- tation on the performance of cognitive tools, and the accu-
in isolated interviews. Moreover, some morphological tive battery for the detection of mild dementia, especially racy of the Alternative Version of the MoCA-BR. Methods:
and metabolic changes have been described in MRI and for the discrimination between Alzheimer’s disease (AD) a pilot, prospective, longitudinal and analytical study.
FDG PET-CT, respectively. There is no formal treatment and frontotemporal dementia. The aim of this study was Both versions of the test were applied in a randomized
for this condition, and more advanced knowledge in this to develop a new logarithm based on discriminative and cognitively healthy population, between 18 and 60
important research in this field is needed. Objectives: To items of the ACE-R combined with relevant demographic years, within a medium interval of 54,56 days between
characterize the cognitive profile of this population pre- characteristics that may interfere on cognition. Method: the questionnaires. Results: out of 104 participants, 70
and post-rehabilitation, including neuroimage patterns The ACE-R was administered to 102 patients with mild were included (64.3% female, 40.2 years). The alternative
and the effect of cognitive rehabilitation. Methods: We dementia due to probable AD and 161 controls from two version obtained superior performances in the naming
selected patients less than 65 years of age through social brazilian research centers. All individuals were submit- domain (p < 0.001), and in the adapted sentence in the
media with a history of COVID19, with at least 3 or more ted to the Mattis Dementia Rating Scale (DRS) and the language domain (p = 0.003). There was no significant
months of symptom and no more than 6 months, no rel- ACE-R. The performance of the patients was compared difference in the delayed recall domain. The alterna-
evant structural brain lesions, no history of neurodegen- and analyzed. Mokken scaling analysis was applied to tive version showed good internal consistency, with a
erative disease, important cognitive issues or psychiatric identify the latent trait on the AD Group. Multivariate Cronbach’s alpha of 0.75. The cut-off point suggested by
conditions and currently living in the city of São Paulo logistic regression and ROC curve analysis were carried the study is 27 points, with sensitivity and specificity of
or nearby regions. They were evaluated in person or by out. Result: Mean ± SD total scores in the ACE-R were 91.3% and 79.2%, respectively. Conclusion: Cultural fac-
remote video call 3 times by a neurologist (0, 3, and 6 70.2 ± 10.8 in AD and 85.1 ± 8.2 in controls. AD Mokken tors affect the accuracy of cognitive tests, and adaptation
months) with specific cognitive battery tests and ques- ACE-R (AMokACE-R) comprises 12 items measuring the is essential for their use in different countries.
tionnaires and then by a neuropsychologist for evaluation same latent concept. Logistic regression with cross-val-
and training. Finally, some patients underwent neuroim- idation pointed that AMokACE-R + Years of education +
aging. Results: 39 patients performed the first baseline Neurologia Cognitiva e do Envelhecimento
ACE-R items Orientation (time) and Memory (name and
evaluation and 14 completed the 6-month follow-up, 34 address recall) share importance as independent variables
completed or were in cognitive rehabilitation, and 19 (p<0.05). The proposed logarithm reached an area under
patients with underwent functional and structural neu- the curve of 0.968, with 93% sensitivity, 94% specificity,
roimaging (FDG PET CT/MRI). This sample has a mean 90% PPV and 95% NPV. Conclusion: The new logarithm
age of 46.2 (SD11.4) years, 53% of females, 13.4 (SD3.4) using the ACE-R items achieved high diagnostic accuracy
years of education, 18% of hypertension, 6.2% diabetes, in identifying mild AD versus controls. Furthermore, the
15.6% dyslipidemia, 15.1% stroke/TIA, and 25% obesity. final ROC curves showed the superiority of the model
59% of the assessments were face-to-face, 41% by tele- proposed in relation to the analysis of the subscales indi-
medicine. The cognitive evaluation depicted: 90% had vidually, as the literature previously reported. Further
memory complaints, MMSE of 26.6 (SD2.7), delayed analysis in larger samples, with biomarkers or pathological
recall of 9.2 (SD1.6) out 10; semantic verbal fluency of confirmation, are necessary to confirm these findings.
16.5 (SD5.1), FAS fluency test 27 (SD12.2), clock drawing
test of 8.8 (SD2.2), and Boston naming test of 13.1 (SD2.4)
out 15. Conclusion: Post-COVID19 syndrome and cog- Neurologia Cognitiva e do Envelhecimento
nitive impairment are relatively common, and we need
prospective studies in our population and other low and
middle-income countries.

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XXX Congresso Brasileiro de Neurologia

TL 1106336 TL 1106364 TL 1106386

DEMENTIA SYNDROMES AS NEGLECTED THE INTERFACE BETWEEN SOCIAL THE USE OF THE COGNITIVE CHANGE
DISEASES COGNITION AND NEUROPSYCHIATRIC QUESTIONNAIRE BY COMMUNITY HEALTH
SYMPTOMS IN MULTIPLE SCLEROSIS: A WORKERS AS DEMENTIA SCREENING
Raul Raposo Pereira Feitosa, Fernanda Martins
Maia Carvalho, Esther Alencar Araripe Falcão NEUROPSYCHOLOGICAL INVESTIGATION METHOD
Feitosa, Norberto Anizio Ferreira Frota Maíra Cardoso Aspahan, Paulo Pereira Christo, Raul Raposo Feitosa, Fernanda Martins Maia
Universidade de Fortaleza. Fortaleza CE, Brazil Leonardo Cruz de Souza Carvalho, Esther Alencar Araripe Falcao Feitosa,
Norberto Anizio Ferreira Frota
raulrpfeitosa@yahoo.com.br Universidade Federal de Minas Gerais e Hospital
Madre Teresa. Belo Horizonte MG, Brazil Universidade de Fortaleza. Fortaleza CE, Brazil
Background: The neglected diseases are classically infec- Universidade Federal de Minas Gerais. Belo
Horizonte MG, Brazil raulrpfeitosa@yahoo.com.br
tious diseases. However, in the health care of elderly
patients, we observe that cognition and functionality are maiaspahan@gmail.com Background: Brazil has a public health system that uses
not routinely evaluated. Aging brings with it an increased family medicine as main strategy and the Community
risk of cognitive decline, moreover, elderly and low-in- Background: Multiple sclerosis (MS) manifests with motor Health Workers play a very important role. The Cognitive
come populations in cities in the interior of northeastern deficits and non-motor symptoms, such as cognitive Change Questionnaire (CCQ) was a tool developed in
Brazil would still be exposed to other risk factors such as decline. One of the cognitive domains affected in MS is Brazil to evaluate cognitive changes in a simple way
low education, poor control of chronic diseases and poor social cognition (SC), which refers to cognitive processes to allow a triage for dementia patients. Objective: To
diet in childhood. In this context, the absence of a routine related to the perception, understanding, and mental evaluate the applicability of the CCQ by Community
assessment of this population could be reflected in a large inference, and is essential to interpersonal exchanges Health Workers (CHW) as a screening tool for dementia
number of patients with the disease and lack of diagnosis Studies of SC in MS are still limited and the influence of in a community. Method: This study was carried out in
of adequate treatments. Objective: The objective of this depression, fatigue, and apathy on SC performance is Pindoretma-CE, a 20.000 habitants town in the proximi-
study was to evaluate the proportion of elderly people who unclear. Objective: To investigate the profile of patients ties of Fortaleza. Community health workers were trained
would have dementia, but without a diagnosis and previ- with relapsing-remitting MS (RR-MS) in SC tests, and on basic dementia principles and how to apply the CCQ.
ous treatment of the condition. Methods: Patients aged 60 to explore correlations of SC measures with depres- Patients assisted by 3 health care units were invited to
years or older from the municipality of Pindoretama-CE sion, fatigue and apathy. Methods: This cross-sectional participate in the study. The CHW was applied by the
were evaluated. Patients in the coverage area of 3 health study included included two groups: 20 patients with CCQ to people aged 60 or over individuals during routine
units were invited to participate in the study. They were stable RR-MS (mean age = 40.15±10.15, mean school- home visits. Questionnaires were evaluated afterwards
evaluated by a neurologist with expertise in cognitive ing 15.35±1.98, 4 men/16 women, mean EDSS score = by a neurologist with expertise in cognitive neurology.
neurology. This assessment consisted of a structured 2.5±1.5) and 20 healthy controls (mean age = 40.2±10.17, Patients were than recruited and accessed by the same
medical interview, neurological and cognitive examina- mean schooling 16.3±1.9, 4men/16 women). RR-MS neurologist using a structured medical interview, neu-
tion, and functionality tests. After evaluation, the data patients had no relapses in the last 90 days and no other rological and cognitive examination, and functionality
were discussed with at least two neurologists, to define neurological diseases. All participants underwent the tests. The batteries used were: mini mental state exam,
whether or not the case would be a dementia syndrome. Beck Depression Inventory, Apathy Scale, Fatigue Scale Brief cognitive battery, Dementia Rating Scale, Pfeffer’s
Results: A total of 213 patients were evaluated; 42 patients (MFIS), Brief International Cognitive Assessment for MS Functional Activities Questionnaire, Geriatric Depression
were identified as having dementia syndrome. Of this (BICAMS), Hayling test, and semantic (animal) fluency. Scale 15-item version. After evaluation, data were dis-
total, only 2 patients had a previous medical diagnosis SC was assessed with the Reading the Mind in the Eyes cussed with at least two neurologists, to define whether
of dementia and some treatment directed to the condi- test (RMET), and the mini-version of the Social Emotional or not the case would be classified as dementia syndrome.
tion. Conclusion: The low recognition and treatment of Assessment (mini-SEA), which is composed by the facial Result: A total of 213 patients were evaluated; 42 patients
dementia syndromes shows a negligence of the health emotion recognition test (FERT) and the modified Faux- were identified as having dementia syndrome. Statistical
care system regarding this condition. Pas test (mFP), which assesses mentalizing. Results: analysis comparing the score obtained in the test with the
Groups did not differ on age, sex and educational level. probability of the patient having or not having dementia
Compared to controls, RR-MS patients had lower perfor- is still in progress. Conclusion: Application of the QCC
Neurologia Cognitiva e do Envelhecimento
mance on semantic fluency (p<0.014) and on working by CWH is a possible screening tool for dementia in small
memory (p<0.011), but not on episodyc memory. Patients communities. Additional data regarding accuracy are
had higher score on the Apathy Scale (p<0.004), but not on needed before this strategy can be used in clinical practice.
depression scale. Regarding SC scores, patients underper-
formed on the FERT (p<0.024) and in the mFP(p<0.016),
but not in the RMET. SC measures did not correlate with Neurologia Cognitiva e do Envelhecimento
depression, apathy of fatigue. Conclusion: Patients with
RR-MS present SC deficits, which seem independent of
neuropsychiatric symptoms. More studies are warranted
to investigate the interface between SC deficits and psy-
chiatric symptoms in MS.

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TL 1106410 TL 1106451 TL 1104772

THE INTER-RELATION BETWEEN GUT COGNITIVE AND BEHAVIORAL CHANGES NEUROBIOLOGY IN AUTISM SPECTRUM
MICROBIOTA AND NEURODEGENERATIVE RELATED TO COVID-19 IN PATIENTS DISORDER (ASD)
DISORDERS IN ELDERLY EVALUATED AT A REFERENCE CENTER IN
Lelia Lage Toto, Silvyo Giffoni
S&ATILDE;O PAULO, BRAZIL
Cynthia von Paumgartten Ribeiro Almeida,
Universidade Estadual de Campinas. Campinas SP,
Paola Maria Barros Diógenes Pessoa, Caroline Flavia Esper Dahy, Aline Moura Brasil Matos, Thais Brazil
Farias Barreto de Moura, Elidivane Martins de Malta Romano, Rosa Maria Nascimento Marcusso,
Freitas Soares, Laiane de Alencar Macedo, Júlia Tatiane Assone, Camila Malta Romano, Augusto ltoto77@gmail.com
Albuquerque Feitosa, Viviane de Sousa Oliveira, César Penalva de Oliveira, Jerusa Smid
Geridice Lorna Andrade Moraes Background: Autism spectrum disorder (ASD) is a dis-
Instituto de Infectologia Emílio Ribas order of the neurodevelopment associated with commu-
Universidade de Fortaleza. Fortaleza CE, Brazil Faculdade de Medicina, Universidade de São Paulo nication deficits and repetitive behavior characterized
Faculdade de Medicina, Instituto de Medicina by a large and atypical variation in the connectivity of
pd-pessoa@hotmail.com
Tropical, Universidade de São Paulo cortical and subcortical brain ligation. Objective: Study
Universidade Federal de São Paulo. São Paulo SP, the neurobiological changes found in the patients with
Background: The gut microbiota has a central role in the
Brazil ASD in childhood and adolescence through reports in the
regulation of immune, endocrine, and neural pathways.
Faculdade de Medicina, Instituto de Medicina literature. Method- Systematic review of the literature on
Recent studies suggest that gut microbiota dysbiosis
Tropical, Universidade de São Paulo the neurobiological aspects of ASD. Pubmed was used as
has influence in the development of neurodegenerative
and neuropsychiatric diseases. The alteration in the gut neuro.flaviaesper@gmail.com a database, with descriptors such as neurobiology, autism,
microbiota during the aging process associated with genetics, neurochemistry, neurotransmitters, magnetic
inflammation and microglia activation can boost the Background: Central nervous system involvement asso- resonance imaging, neurophysiology. The inclusion crite-
risk of neurodegenerative disorders, like Alzheimer’s ciated with Coronavirus Disease 2019 (COVID-19) has ria were bibliographic materials available in English and
and Parkinson’s disease. Objectives: Identify what the been reported, including cognitive impairment, even Portuguese. Findings: It were identified 649 article with
scientific literature says about the relation between the in patients with mild COVID-19. Objectives: To assess the use of keyword listed above. After the initial analysis,
gut microbiota and neurodegenerative diseases in elderly. cognitive decline related to the SARS-CoV-2 infection the articles were selected. One of the articles studied 662
Methods: A systematic review was performed in PubMed in patients with neurological disease after COVID-19. patients showing that differene areas of the brain have
and Scielo databases using the keywords (“Microbiota” Methods: Longitudinal prospective study developed increased cortical thickness, with the region being the most
AND “Neurodegeneration” AND “Aged”). Articles with to compare the cognitive performance of patients after affect upper temporal gyre. The second most affected area
free access in Portuguese or in English published between COVID-19 based on cognitive complaints. Patients aged would be the middle occipital gyre. One study demon-
January/2018 and May/2022 were included in the study. between 18 and 65 years without previous neurological strated increased connectivity in the sensitive thalamic
Articles out of context, repeated and editorials were disease were included in the study and illiterate people area. Other text claims that the increase on cortical thick-
excluded. It was found 30 articles and analyzed 15. Results: were excluded. Beck’s anxiety and depression question- ness may be related to the severity of the clinic of the case.
It was analyzed 8 transverse studies, 6 systematic reviews naires were applied, also the Addenbrooke´s Cognitive Pathological changes are more frequent in non-treated,
and 1 observational study. 6 of them approached the Examination-Revised (ACE-R) and the Trail Making Test suggestion that treatment in childhood has a beneficial
relation between gut microbiota and Parkinson’s Disease (TMT), parts A and B. Results: Sixty one patients were effect on the brain development. The treated individuals
(PD), 5 presented the association among dysbiosis and evaluated, 41 women (67.21%) and 20 men. The mean may present reversal of the changes showing gains in brain
Alzheimer’s Disease (AD) and 4 discussed the influence of age of all patients was 46.36 years (standard deviation – fuction. Conclusion: Neurobiological changes, especially
gut microbiota and neurological alterations in elderly. The SD 9.92) and median of 46 years. In relation to schooling, structural anatomical changes reported in most of eht
AD studies showed that dysbiosis causes a hypersensitivity mean of 13.93 years (SD 4.06) and median of 16 years. reviewed articles,done in studies with a large number of
of neurons which can amplify the immune response and Forty-four patients had cognitive complaint (72.13%), patients and in different muldisciplinary centers, point
accelerate degeneration via inflammation and release of 21 (47.72%) of them the only complaint. Regarding the out lines of research and study for our environment. The
cytokines. Other studies investigated the benefits of pro- time between the first symptom of COVID-19 and the need for early treatment/rehabilitation was highlighted,
biotics and its contribution for the prevention of neuro- onset of the neurological symptom, mean of 39.52 days since there is evidence in the literature of good results,
degeneration; however, its response depends on dietary (SD 40.7) and median of 29 days. Among all patients, the including reversal of anatomical changes, which other-
habits, comorbidities, probiotic dosage, composition, mean score of Beck’s anxiety and depression question- wise consolidate in adulthood
and intake frequency. Studies showed that elderly with naires was, respectively, 16.72 (SD 10.05) and 13.31 (SD
DP and severe gastrointestinal symptoms due to dysbi- 6.97), and median of 15 and 12, which indicates mild to
osis suffered more cognitive impairment as alterations Neurologia Infantil
moderate degrees of anxiety and depression. The results
of dopamine and motricity. One study also implies that of ACE-R were altered in 41 patients (67.21%) – 30/44
alterations in the oral microbiome have correlation with (68.18%) patients with a cognitive complaint and 11/17
DP. This is relevant to future therapeutic approaches since (61.11%) patients without this complaint (p = 0.07798).
its management is easier than genetics and gut microbiota. The score on the mini-mental state examination (MMSE)
Conclusion: The findings suggested that dysbiosis can was also altered in 41 patients. In the TMT part A, score
amplify inflammatory responses contributing to neuro- altered in 39 patients (63.93%) – 10 had lower average
degeneration. However, the microbiota-gut-brain axis is performance, 8 borderline and 21 severe performance.
not fully comprehended yet. It is necessary to have more In the TMT part B, score altered in 43 patients (70.49%)
research about it in order to better understand the thera- – 4 had lower average, 3 borderline and 36 severe per-
peutic use of prebiotics and probiotics, its prognostic and formance. No patient had superior performance on any
its possible use as prevention in the future. part of the test. Two patients could not take the TMT’s
due to motor limitation because of motor sequelae of
COVID-19. Conclusion: Cognitive complaint was not a
Neurologia Cognitiva e do Envelhecimento predictor of cognitive decline, and the cognitive decline
was observed in most patients, in the MMSE, ACE-R and
TMT assessments cognitive decline.

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TL 1105010 TL 1105164 TL 1105181

THE PAINFUL MISSION OF COMMUNICATING AUDIOVISUAL OVERSTIMULATION THE RELATIONSHIP BETWEEN


THE DIAGNOSIS OF SPINAL MUSCULAR PROMOTES ANXIOGENIC-LIKE BEHAVIOR IN THE NEUROPSYCHOMOTOR AND
ATROPHY TO CHILDREN A SEX-DEPENDENT MANNER IN MICE NEURODEVELOPMENTAL CHARACTERISTICS
IN 18 TO 47 MONTH-OLD CHILDREN
Isabella Araujo Mota Fernandes, Renata Oliveira Gabrielle Brito Bezerra Mendes, Eduarda Rabêlo
Almeida Menezes, Guilhermina Rego Lima, Liêver Moura de Oliveira, Jessica Layanne Maria Eduarda Pasculli Marinheiro, Júlia Neves
de Sousa Lima, Gerlânia de Oliveira Leite, Adriana Barbosa, Jacy Perissinoto, Ellen Osborn, Beatriz
Universidade do Porto. João Pessoa PB, Brazil Rolim Campos Servilha Brocchi
UFRN. João Pessoa PB, Brazil
Universidade do Porto – Portugal Universidade de Fortaleza. Fortaleza CE, Brazil Pontifícia Universidade Católica de Campinas.
Campinas SP, Brazil
isabella_mota@yahoo.com.br gabriellemendes04@edu.unifor.br
Universidade Federal de São Paulo. São Paulo SP,
Brazil
Background: The trinomial relationship between the Background: Excessive use of screens is known to be
doctor-child-legal guardian is essential in the process of related to increased incidence of mental disorders in chil- dudapmarinheiro@gmail.com
a disease communicating and its prognosis. Objectives: dren and adolescents. It has been demonstrated that mice
To analyze the communication of the diagnosis of spinal subjected to audiovisual overstimulation show behaviors Background: the knowledge of the health professionals
muscular atrophy (SMA) by this trinomial relationship. compatible with Attention Deficit Hyperactivity Disorder. of neuropsychomotor and neurodevelopmental skills
Methods: Relatives of SMA patients answered a ques- Objectives: The aim of this study is to analyze whether of children is essential for their follow-up. Objective:
tionnaire containing Objective: questions and the Event there is a relationship between the sex of the animal and to investigate and associate the neuropsychomotor and
Impact Scale – Revised. Results: The sample consisted of the anxious behavior of animals subjected to audiovisual neurodevelopmental skills of 18 to 47 month-old children.
50 volunteers, 94% of whom were female. 48% reported overstimulation. Methods: Swiss mice (20 – 30 g), male and Methods: this research is a transversal study of the rela-
psychological sequelae related to the moment of diagno- female, 21 days old, were used. For 42 days, the animals tionship between neuropsychomotor and neurodevel-
sis, having a direct correlation when the communication were placed in a room containing sound from children’s opmental characteristics. It included 30 parents whose
was made by them. 22% have a high risk of post-traumatic television programs at a volume maintained below 70 dB, children are typical and aged from 18 to 47 months-old
stress, relating the feeling of unpreparedness in com- the level used in acoustic stress models. The animals were followed by a Pediatrics Service. As materials, the NPDC
municating with the child. Conclusion: It was identified divided into groups (n = 6 each) as follows: control males (Neuropsychomotor Development Checklist) and the
that the failure in communication is the main factor for and control females – animals without stimulation, but M-CHAT (Modified Checklist for Austism in Toddlers) were
negative repercussions with self-report of psychological taken daily from the maintenance vivarium and kept in used and both are composed by dichotomous questions.
sequelae, in addition to the high risk for post-traumatic a separate room for 2 or 6 h daily. Stimulated males and The participants were divided into two groups. The first
stress syndrome. stimulated females – animals with daily stimulation for represents the children who presented risk on M-CHAT
2 or 6 h. After 10 days of rest, the animals were subjected and the second those who did not present risk. Descriptive
to the elevated cross maze test. Results: There was no percentages and statistics analysis, Mann-Whitney Test
Neurologia Infantil difference in the behavior of male and female animals (as statistical significance p<0.05), were used to analyze
stimulated for 2 h daily. However, females overstimulated the results. Results: The neurodevelopmental risk group
for 6 h daily, stayed longer in the open arm (*p<0.05) and included six (20%) of the total number of participants (30).
produced more feces pellets (**p<0.01) when compared This group did not achieve the expected neurodevelop-
to the non-stimulated group (naive). The overstimulated mental stages such as directing their gaze by 6 months-
male animals showed increased locomotor activity char- old (33%); crawling by 9 months-old (33%); inviting other
acterized by more entries into the open arm (*p<0.05) children or adults to play by 18 months-old (50%); point-
compared to the non-stimulated group. Conclusion: ing named parts of their own body by 12 months-old
The higher preference for the open arm suggests anx- (66%) and using phrases with two or three words (66%).
ious behavior, inferring impulsivity content. The results Those two last skills were statistically signicant (p=0.007
obtained point out that overstimulated females seem to and p=0.048, respectively). On the other hand, the sec-
show a higher propensity to take risks. ond group, which was not at neurodevelopmental risk,
presented neuropsychomotor skills below expected.
However, the aspects were different if compared to the
Neurologia Infantil risk group, such as sitting without support by 6 months-
old (20%); speaking isolated words by 14 months-old
(25%) and mispronouncing speech sounds (33%). This
group without risk also had some unexpected similar
characteristics. Four children (16%) had not crawled by
9 months-old; six (25%) had not invited other children
or adults to play; almost eight (33%) of them mispro-
nounced speech sounds; six (25%) had not understood
simple stories and all of them had pointed named parts
of their own body by 12 months-old. Conclusion: Both
groups underperformed on neuropsychomotor tasks and
the ability of pointing named parts of their own body by
12 months-old got an important statistical significance.
Thus, the neurodevelopment and neuropsychomotor
follow-up is essential for typical children.

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TL 1105557 TL 1105704 TL 1105809

MOTION SICKNESS IN CHILDREN AND BENEFITS OF APPLYING MUSIC THERAPY CLINICAL-EPIDEMIOLOGICAL PROFILE
ADOLESCENTS WITH NEUROLOGIC TO CHILDREN WITH AUTISM SPECTRUM OF EPILEPSY IN PATIENTS FOLLOWED-
COMORBIDITIES DISORDER: A SYSTEMATIC REVIEW UP SINCE CHILDHOOD IN A UNIVERSITY
HOSPITAL
Larissa Clementino Leite de Sa Carvalho, Gabriel Gabriela Arruda Navarro Tavares, Davi Arnaud, Ana
Silva Chaves, Natália Diane Ricarte Lins Freitas Carolina Araújo Falcão, Letícia Fontenele Texeira, Djanino Fernandes Silva, Paulo Barbosa Leite
Assis, Olavo Santa Cruz Valadares Neto, Paulo Nicolas Andrade Moreira, Letícia Bezerra de Neto, Marco Andre Moraes Bernardino, Larissa
Barbosa Leite Neto, Djanino Fernandes da Silva, Oliveira, Amanda Tomaz Rodrigues, Frederico Carlos Clementino Leite Sá Carvalho, Victor Fellipe Bispo
Victor Fellipe Bispo Macêdo, Marco Andre de Sousa Arnaud Macêdo, Hellysson Phyllipe Firmino Cavalcanti, João
Moraes Bernardino, João Eudes Magalhães, Fabiola Eudes Magalhães, Fabíola Lys Medeiros
Lys de Medeiros Universidade de Fortaleza. Fortaleza CE, Brazil
Hospital Universitário Oswaldo Cruz da
Hospital Universitario Oswaldo Cruz, Universidade gabiarruda8@hotmil.com
Universidade de Pernambuco. Recife PE, Brazil
de Pernambuco. Recife PE, Brazil
Centro Universitario Mauricio de Nassau. Recife PE, Background: Music therapy is a therapeutic modality djanino@gmail.com
Brazil that consists of the integration between music and health
with the Objective: of providing intellectual development, Background: Epilepsy is a neurological condition with
clementinolari@gmail.com communication and well-being, assisting in socialization high prevalence in neuropediatric care, requiring lifelong
factors to improve the quality of life of the individual. drug treatment in most cases, in addition to multidisci-
Background: Motion sickness or intolerance to move- Therefore, several studies have analyzed the use of music plinary team support. The individual with epilepsy may
ments is the occurrence of signs or symptoms of conflict therapy as a therapeutic intervention in the treatment of present neurobiological consequences such as cognitive,
in the sensory perception of stimuli from the proprio- children with Autism Spectrum Disorder (ASD), since in behavioral, and social disorders, causing great impact on
ceptive and vestibular sys-tems. Results: in a number this clinical condition there is a loss in social interaction the patient’s quality of life. Objectives: To describe the
of symptoms including nausea, vomiting, dizziness and and communication. Objective: To identify the main clinical and epidemiological characteristics and the profile
heada-che. The literature on motion sickness in adults benefits of music therapy in the treatment of children of drug use of epileptic patients followed up as outpatients
is extensive due to its medical mission for military and with autism. Method: This is a systematic review, which in a specialized care service. Methods: Retrospective
space travel. In contrast, the pediatric population has not used the following descriptors: “music therapy”, “child”, Cross-sectional study, with consultation of outpatient
been well studied. This is surprising given that the preva- “autism spectrum disorder” with search in the databases: records of child and adolescent population with epilepsy
lence of motion sickness in children to be higher than in Medline, EBSCO, LILACS and SciELO. The inclusion cri- in a university Hospital in Northeastern Brazil. Relative
adults. The difficulty of studies in this area and a lack of teria were articles in Portuguese, English and Spanish; full and absolute frequency distribution tables were used for
understanding this illness’s etiology and its comorbidi- articles with the theme in question; articles published and descriptive data analysis. The Chi-square test for inde-
ties can impact the course of the disease. An example of indexed in the last 5 years in the mentioned databases. pendence and Fisher’s exact test were applied to analyze
this are some neurological diseases that can be etiolog- The exclusion criteria were review articles and articles that the existence of association between variables. The sig-
ically correlated as an aggrava-ting the development of were not related to the theme. Results: 21 references that nificance level was 5% (p<0.05%), using IBM SPSS 20.0
motion sickness. Objective: To evaluate the occurrence met the criteria of this research were selected. Of these, software. Results: The medical records of 282 patients
and characteristics of motion sickness in neuropediat- 20 articles evidenced the value of music and its role as with epilepsy (143 males and 139 females), aged 2 to 19
ric patients with followed up in a university Hospital in a therapeutic resource in children with ASD and 1 was years, were analyzed. About the age of onset of epilepsy,
northeastern of Brazil. Method: It is an observational, indifferent to the results presented. Those that presented the most prevalent age group was those older than 11
analytical, cross-sectional study. All sex patients, younger positive results evaluated the application of music ther- years (31.2%), followed by those younger than 2 years
than 18-years-old, with a previous diagnosis of a neuro- apy in the medium and long term and the benefits pre- (30.9%), schoolchildren between 6 and 10 years repre-
logical disease, regularly followed up at neuropediatrics sented were the development of new social interactions, sented 23.4% of the sample, and preschoolers between
service were included. Preg-nant women and patients improvement in the behavioral pattern of isolation, and 3 and 5 years corresponded to 14.5%. As for the clinical
with severe systemic diseases were not selected. Data improvement in communication skills. The study that did features of epilepsy, 77.7% had focal epileptic seizures,
were collected through digital forms to analize: socio- not show significant results evaluated the social affect and 11.3% had indeterminate seizures, and 11.3% had gen-
demographic factors, predictable diagno-ses, suscepti- the parents’ perception of social response and concluded eralized seizures. The recorded electroencephalogram of
ble to motion sickness and presence of headache. The that there was no considerable improvement. Conclusion: these patients showed alterations in 68% of the sample,
Motion sickness sus-ceptibility questionnaire (MSSQ) Based on the analysis of the studies presented, it can be with focal alterations being the most observed finding
was Applied. The study had ethics committee approval. concluded that the application of music therapy acts pos- (28.4%). Some comorbidities investigated were migraine
Results: The preliminary sample contains 70 patients, itively in the areas of socialization, communication, and (34%) with and without aura, tension-type headache (2%),
35% of these presented motion sickness; 34% of patients language. Playing with instruments is a way to interact behavior disorders (20.9%), and sleep disorders (8.5%).
are within the autistic spectrum (ASD) and of these 15.4% with others and establish strong social bonds, being one Regarding antiepileptic drugs use, 73.3% were monother-
had motion sickness; 12% of patients have a diagnosis of the most important aspects of this practice. The bene- apy, while 23.3% were taking two drugs, and only 3.5%
of attention deficit hyperactivity disor-der (ADHD, and fits are not immediate, but in the long term they promote were taking three drugs; and no significant difference was
30%of these had motion sickness; 20% of the sample has emotional satisfaction, break the patterns of social isola- observed between males and females for these variables
epilepsy, of which 30% had motion sickness; From the tion and facilitate self-expression. cited. Conclusion: As a chronic neurological condition,
sample, 15% of migraine patients and of these 55% have age of onset of epilepsy is more prevalent in teenagers
motion sickness. Conclusion: The preliminary findings of and infants, with a higher incidence of focal seizures.
our research demonstrated a high prevalence of motion Neurologia Infantil We also observed that migraine is the main comorbid-
sickness in children and adolescentes with neu-ropedi- ity diagnosed in these patients. Even in a referral center
atric disorders. Even children with behavioral disorders, for epilepsy care, a large proportion of patients are well
motion sickness could be verified by the parents’ percep- controlled with monotherapy.
tion through the specific questionnaire (MSSQ). Our study
also corroborates the literature on associative aspects of
motion sickness with a previous diagnosis of migraine. Neurologia Infantil

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TL 1105814 TL 1105823 TL 1105826

DETERMINANT VARIABLES OF THE EVEROLIMUS IN THE TREATMENT OF ASSOCIATION BETWEEN SELF-MUTILATION


ABSENCE OF VERBAL LANGUAGE IN REFRACTORY EPILEPSY IN TUBEROUS AND CONSTIPATION IN PATIENTS WITH
PATIENTS WITH AUTISTIC SPECTRUM SCLEROSIS COMPLEX: A CASE SERIES AUTISM SPECTRUM DISORDER
DISORDER
Catarina Ester Gomes Menezes, Marcos Baruch João Pedro Rosal Miranda, Bruna Tavares Falcão,
Gilberto José de Melo Neto, Maria Eduarda de Portela Filho, Lécio Aragão Souza Morais, Daniele Ângelo Antônio Lins Cavalcante, Maria Eduarda de
Souza Arêa Leão, Giovana da Rocha Leal Dias, Santos Andrade, Paula Cely da Silva Torres, Lilliane Souza Arêa Leão, Gabriel Nunes Dantas, Kelson
Gabriel Nunes Dantas, Kelson James Almeida Rodrigues Soares, Jefferson Heber Marques Fontes James Almeida
Júnior, Julieta Sobreira Goes
FACID-WYDEN. Teresina PI, Brazil Unifacid. Teresina PI, Brazil
UNINOVAFAPI. Teresina PI, Brazil Universidade do Estado da Bahia. Salvador BA, Universidade Federal do Piauí. Teresina PI, Brazil
Brazil UFPI e Unifacid. Teresina PI, Brazil
gjdemeloneto@outlook.com União Metropolitana de Educação e Cultura UNIME
jp-rosalmiranda@hotmail.com
Background: Autistic spectrum disorder (ASD) is a neu- catarina.gomes.menezes@gmail.com
rological disorder caused by the interaction of genetic Background: Self-injurious behavior (SIB) is defined as
and environmental factors. The individual presents atyp- Case Presentation: Patient 1: A female infant was diag- any intentional action that generates direct aggression to
ical evolution in developmental milestones, behavioral nosed with tuberous sclerosis complex (TSC) at 7 months one’s own body without conscious intention of suicide or
changes and difficulty in communication and social of age after initiating generalized seizures associated exposure, as in the case of tattoos and piercings. SIB is
interaction. Thus, it is noticeable, with about 5% to 50% with developmental delay and stereotyped behaviors. considered a psychiatric disorder that is common in chil-
of cases, that the first skill to present delay is language, EEG showed generalized and focal epileptiform poten- dren with Autism Spectrum Disorder (ASD). In addition,
becoming a predictor of ASD. Objective: To evaluate the tials and ictal activity from epileptic spasms. Brain MRI gastrointestinal symptoms, such as functional constipation
determinant variables for the absence of verbal language identified cortical dysplasias and subependymal nod- (FBC), are also associated with mental and behavioral
at 3 years of age, and to describe their neuropsychomotor ules. The echocardiogram showed 2 cardiac rhabdomy- health in children with ASD. Objectives: To investigate
development. Methods: This is a documentary, observa- omas and the exome revealed a deletion of 18 base pairs the existence of an association between CIF and SIB in a
tional, cross-sectional, retrospective, descriptive research in exon 41 of the TSC2 gene in heterozygosity (chr16: patient with ASD. Methods: This was a transversal study
with a quantitative approach. The following were ana- g.2088293_2088310del; depth: 133x). First-line treatment of applied, observational and exploratory nature with a
lyzed: medical records collected in a protocol form from for epilepsy in TSC was started with vigabatrin, but after quantitative and descriptive approach. For this, we used
patients diagnosed with ASD, according to the DSM-V an initial improvement period, seizures intensified, and a questionnaire applied to patients with ASD in a care
criteria, from a specialized institution in Teresina-PI, social interaction worsened. Treatment with everolimus and rehabilitation institution. Therefore, the inclusion
in the period between 2014 and 2019, after approval by was then started, and the patient evolved with a substan- criteria according to the DSM-V were used. In addition,
the local Research Ethics Committee (CEP). The demo- tial reduction of seizures, improved social and visual the FASM scales and the Rome IV criteria were applied
graphic variables used were age and gender, the clinical interaction and began to walk. Today, almost two years for the diagnosis of BIS and ICF, respectively, and Fisher’s
variables were neo and perinatal antecedents, neuropsy- after starting treatment, the patient is in use of everoli- Exact Test was used to assess the association between
chomotor developmental milestones, associated neuro- mus and oxcarbazepine and has good seizure control, these variables. Results: A total of 74 patients with ASD
psychiatric pathologies and disorders, and medications in with age appropriate development and without severe were obtained, with a predominance of men, with 59
use. The Denver Scale was used to assess neuropsycho- everolimus side effects. Patient 2: A 3-month-old male cases (79.7%). Mean age was not statistically significant
motor milestones, data was organized using Microsoft® infant presented with clonic seizures in both eyelids, left enough to be considered a predictor variable for SIB or
Office Excel, and statistical analysis using the Biostatic hemiface and left arm. Brain MRI showed subependymal ClF. As for the level of education of the responsible family
5.0. Results: In the present study, 90 medical records nodules in both lateral ventricles and cortical tubers in member, there was a prevalence in patients whose par-
of patients diagnosed with Autism Spectrum Disorder the middle gyri of both frontal lobes. EEG revealed epi- ents had a higher level of education, the most prevalent
(ASD) were analyzed, with a mean age of 11.65 years, of leptiform potentials in the left occipital region. A hetero- being those with complete high school and incomplete
which 20 (22.22%) were female, while 70 (77, 78%) were zygous missense variant was identified in the TSC2 gene college education, about 33.8% (25) of cases, followed by
male. It was possible to verify in this analysis, in isolation, (TSC Complex Subunit 2, OMIM*191092). Treatment was those with complete college education, with 29.7% (22).
that the delay in verbal language was more prevalent in started with vigabatrin. Due to inefficient seizure control, To clinically stratify ASD, the CARS was used, in which
those who were born by cesarean section (p= 0.00018). levetiracetam was associated, followed by the introduction 70.3% of participants were classified as mild/intermedi-
Conclusion: The determining variable for the absence of of oxcarbamazepine. However, the patient continued pre- ate. Additionally, the prevalence of ICF was 37.8% and
verbal language in patients with ASD at 3 years of age was senting 2 seizures weekly. After 5 months without adequate of SIB 37.8%, with no statistical association between
the mode of delivery, especially when correlated alone, symptom control, everolimus was introduced, and seizure them (p of 0.769). Conclusion: Thus, it was noted that
demonstrating a greater tendency to language delay in remission was achieved. Currently, about ten months after there was a high prevalence of SIB and ICF in the study
those who were born by cesarean section, even in the everolimus introduction, the patient remains seizure free sample. There was no association between CIF and SIB
absence of complications. and has adequate development for his age. Conclusion: in patients with ASD.
TSC is a multisystem genetic disease in which epilepsy is
a frequent manifestation and is often difficult to control.
Neurologia Infantil Everolimus is an mTOR inhibitor drug, with evidence Neurologia Infantil
suggesting benefits of its use in treating epilepsy in TSC.
Inadequate control of seizures appears to play a crucial
role in the cognitive and behavioral decline in patients
with TSC, reiterating the importance of early diagnosis
and treatment to improve the neurocognitive prognosis
in these patients.

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DENTAL APPROACH IN PATIENTS WITH WEST SYNDROME: A LITERARY REVIEW FETAL ALCOHOL SYNDROME (FAS):
MICROCEPHALY BY ZIKA VIRUS CONSEQUENCES FOR THE FETUS AND
Andersanny Moura Bernardes, Italo Felipe Cury,
Anderson Moura Bernardes, Anderlanny Moura IMPAIRMENTS IN CHILD DEVELOPMENT
Andryel de Morais Gomes, Júlia de Melo Nunes,
Camilla Vanessa Araujo Soares, Éric Moreira Bernardes Juliana Lago de Araújo, Victória de Menezes
Menezes, Pedro Nascimento Araújo Brito, Raquel Sá Lazera, Fernanda Lago de Araújo Borges,
UNIRG – Gurupi – TO – Brazil
Barbosa de Menezes, Lucas Emmanuel Freitas Alessandra Monteiro Camapum, Matheus Carreiro,
UFPI – Teresina – PI – Brazil
Mendes, Luiz Felipe Nogueira de Figueiredo Lobo, Rita de Cássia Miranda
Wilton Wilney Nascimento Padilha, Juliana Sousa andersannybernardes@gmail.com
Soares de Araújo UNICEUMA. São Luís MA, Brazil
Background: West Syndrome (WS) was first described by UFMA – São Luís – MA – Brazil
Universidade Federal da Paraíba. João Pessoa PB,
William James West (1793–1848) in 1841 and classically viclazera@hotmail.com
Brazil
corresponds to an epileptic syndrome characterized by
camilla.vanessa46@gmail.com repetitive spasms and a pattern of hypsarrhythmia that Background: Alcohol is the most consumed psychoac-
result in a delay in neuropsychomotor development. tive drug worldwide. Its metabolization happens more
Background: Microcephaly is characterized by a neuro- This pathology has been progressively studied and can slowly in women, taking into account their body structure,
logical disease in which the child presents a decrease in often be named as infantile spasm, based on the most increasing the risk of pathologies associated with its con-
brain diameter, causing motor sequelae, speech delay, relevant initial clinical event. Although its causes are sumption, even if it is moderate. However, the demand for
behavioral disorders and craniofacial development. These unknown, in recent years new advances in this disorder alcohol by women has become an issue of public health
characteristics also affect the child’s oral health, because have been made, and point to predisposing factors for concern, especially when it occurs during pregnancy,
most of them present oral changes such as: periodontal the disease: hypoglycemia, lack of oxygen to the brain causing harmful effects to the fetus during pregnancy,
diseases, dental caries, malocclusion, micrognathia, during childbirth, brain malformation, prematurity, sep- postpartum, and during the child’s development, which
delayed dental eruption, dysphagia, bruxism and dental sis, stroke, infections and genetic. Objective: To system- is called FAS (Fetal Alcohol Syndrome). Children with
trauma that may have a direct and negative impact on atically demonstrate the West Syndrome questioning FAS have characteristic facial alterations, prenatal and/or
their health. Objective: In this sense, the present study its complexities and peculiarities and what makes it a postnatal growth restriction, and structural and/or func-
has as its main objective to analyze the craniofacial and rare and complex syndrome. Methods: This is a system- tional abnormalities of the central nervous system (CNS).
dental alterations in patients with microcephaly. Method: atic literature review based on the extraction of data in They may present other congenital malformations, learn-
This is a cross-sectional observational study with patients indexes such as PUBMED and MEDLINE. Publications ing and memory difficulties, and functional, emotional,
with microcephaly treated in Paraíba, which used a struc- from 2018 to 2022, in English, were considered, with and behavioral problems. Currently, alcohol is consid-
tured questionnaire divided into two parts: the first with the following descriptors: West syndrome, Neurologia, ered the most common fetal teratogenic agent, but it is
questions regarding socioeconomic data and the second Epilepsy, Infantile spasms. Results: Overreliance on the still neglected by many health professionals. Objective:
regarding data related to the object of study. The data term hypsarrhythmia is particularly problematic, since This study aims to relate alcohol consumption during
was collected through the Google form and then tabu- many children who experience infantile spasms will not pregnancy with fetal neurological malformation, as well
lated by Excel. Results: 48 children were attended, 54% have this classic pattern. Presence or absence on the as to address ways to prevent this situation. Methods:
female and 46% male. 47% of the public examined were pre-treatment EEG has no impact on treatment response, This is a scientific study that allows a broad approach to
between 5 and 6 years old, with a family income of one risk of subsequent seizures, or later developmental out- fetal alcohol syndrome. The textual search was restricted
to two minimum wages, making up 79% of the sample. come. The hypsarrhythmia requirement in the criteria for to scientific publications from the SCIELO and PUBMED
All participants had significant neurological alterations, West syndrome is unsupported by documentary evidence databases during the month of May of this year, using
presenting seizures, spasms, motor deficits, and cognitive and is overly restrictive. Thus, almost all babies with the publications from the last 13 years with the following
deficits. Regarding craniofacial alterations, the following infantile spasms type of seizure also have West syndrome. descriptors: Pregnant Women; Alcohol; Consequences for
were identified: lack of lip seal, tongue projection, and Conclusion: It can be concluded that many advances the fetus. Original articles indexed in the period between
palate alterations. Regarding oral health, 49% visited the have been made, however there are still many issues to 2009 and 2021 were included and articles that do not have
dentist once a month, and the reason for the visit was the be resolved about SW, which suggests steps forward. As such descriptors and are not within the delimited period
need for treatment and follow-up. 90% use the public ser- a result, the diagnosis of West syndrome should not be were excluded. Results: The harmful effects of alcohol
vice, 57% report difficulty in finding a specialized dentist, made in a restrictive manner and even though the defin- consumption during pregnancy were confirmed and iden-
91% of caregivers reported difficulty in performing oral ing triad of the disease can be considered, the absence of tified, especially regarding its consequences for the fetus
hygiene, for reasons of limited mouth opening, reflux, agi- a criterion should not exclude the diagnosis, so it should and impairments in child development throughout life.
tation and aggressiveness, 88% use a fluoride toothbrush not interfere in the planned treatment for the disease. In addition, ways to prevent this worrying issue for public
and toothpaste for hygiene. Of the total sample, 32% of health were addressed. Conclusion: Alcohol consumption
patients had no need for treatment and 68% had some by pregnant women can lead to irreversible neurological
need for treatment such as: prophylaxis, tartar removal, Neurologia Infantil
consequences for the fetus, postpartum, and throughout
fillings and extractions. Conclusion: This work provided a the child’s development. This psychoactive drug is consid-
better understanding of craniofacial and dental changes ered the most common fetal teratogenic agent, becoming
in patients with microcephaly, a topic little explored in a major public health problem. However, this situation is
research, in order to collaborate to improve the provi- preventable if the woman abstains from drinking alcohol
sion of care for these patients and promote quality of life. before conception and during pregnancy. In addition, they
need to be routinely screened by health professionals for
alcohol use during prenatal care.
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AUTISM AND COGNITIVE IMPAIRMENT IMPACT OF THE COVID-19 PANDEMIC IN DESCRIPTIVE ANALYSIS OF THE
IN DYSTROPHIC AND STRUCTURAL CHILDREN WITH AUTISTIC SPECTRUM CORRELATION BETWEEN THE PRENATAL
MYOPATHIES: ARE WE MISSING OUT? DISORDER AND THE OCCURRENCE OF ANOMALIES IN
THE NERVOUS SYSTEM IN THE PERIOD OF
Clara Gontijo Camelo, Cristiane Araújo Martins Bianca Berardo Pessoa Zirpoli, Daniel Cavalcanti
Moreno, André Macedo Silva, Alulin Tácio Quadros Sena, Patryck Andrew Ribeiro de Melo Pontes, Maria 20 YEARS
Monteiro Fonseca, Umbertina Conti Reed, Edmar Valeska Berardo Pessoa de Souza, Erideise Gurgel Renata Pinheiro Martins de Melo, Sônia Renata
Zanoteli da Costa, Ana Cristina Montenegro, Maria Eduarda Pinheiro Martins
Iglesias Berardo de Souza, João Batista Guerra
Universidade de São Paulo. São Paulo SP, Brazil Barretto Neto Universidade Federal do Ceará. Fortaleza CE, Brazil
Universidade Federal de São Paulo. São Paulo SP, Empresa Brasileira de Serviços Hospitalares
Brazil Universidade Católica de Pernambuco. Recife PE, EBSERH
Brazil
claragc@gmail.com Prefeitura do Recife. Recife PE, Brazil renatapinheiro2003@hotmail.com
Universidade Federal de Pernambuco. Recife PE,
Background: Myopathies represent a wide spectrum Brazil Background: Congenital anomalies are the fourth leading
of heterogeneous diseases characterized mainly by the Faculdade Pernambucana de Saúde. Recife PE, cause of neonatal death and 21% of these malformations
abnormal structure and functioning of skeletal muscle. Brazil are related to the nervous system. In this scenario, despite
Due to the pathogenesis of myopathies being diverse and all advances in obstetrics and improvements in prenatal
several systems being affected, neurodevelopmental disor- berardobianca@gmail.com care for pregnant women, the prevention of mortality in
ders may also occur. However, amongst the genetic myop- neonates due to defects in the neurological tract remains a
athies, intellectual disability (ID) and autism spectrum Background: Autistic Spectrum Disorder (ASD) comprises challenge to be overcome. Objective: Analyze the proper
disorder (ASD) has been mostly described in Duchenne difficulties in social interaction and communication, as performance of prenatal care, correlated to the occurrence
Muscular Dystrophy, Alpha-dystroglicanopathies and well as restrictive patterns and repetitive behavior. In of deaths from neurological defects in neonates in Brazil
Myotonic Dystrophy, but not in other structural and dys- the current pandemic, usual care, routine and activities from 1999 to 2019. Methods: Descriptive analysis, obtained
trophic myopathies. Objective: Describe three patients directed to children with ASD changed due to preven- from the DATASUS platform, of the frequency of prenatal
with dystrophic and structural myopathies with ID and tive measures such as social distancing. With that, these care, the frequency of births with congenital anomalies
ASD. Methods: Description of three patients with ACTA1- children and their caregivers went through great stress, and how many of these disorders are related to defects in
myopathy and LAMA2-congenital muscular dystrophy reflecting on different aspects of their lives. Goal: To assess the nervous system and mortality rate in children under 1
with ID and ASD and literature review. Results: Patient the impact of the COVID-19 pandemic on children with year old due to neurological diseases. Results: From 1999
1 was a 7 years old male with hypotonia, proximal muscle ASD and their caregivers. Methods: A review was made to 2019, in Brazil, 702 neonates died due to defects in the
weakness, myopathic face and muscle biopsy with nema- utilizing the Preferred Reporting Items for Systematic nervous system and 3,443,087 women did not perform
line rods, who presented with hemizygous pathogenic Reviews and Meta-Analyses (PRISMA) of published stud- prenatal care or did it inappropriately. In addition, 30,734
variant in ACTA1 (p.Gly57Arg). He was non-verbal, had ies that assess changes in the well-being of children with children, whose mother didn’t perform prenatal care, or it
stereotyped movements, poorly functioning play, poor ASD and their families during the Pandemic around the was performed inappropriately, were born with a certain
social interaction and didn’t comprehend simple com- world and in Brazil, ranking each article according their congenital anomaly, which represents 21% more than
mands. Patient 2 was an 8 years old boy with congenital Risk of Bias, upon which only the “Good” and “Regular” those who had congenital disorders, but prenatal care
hypotonia, with maximum motor ability to sit without articles were utilized. Results: 161 articles were found was being performed. Furthermore, of a total of 170,392
support, that presented with myopathic face and multi- upon inicial search, of which 17 were selected. Amid the cases of births with anomalies, 32% were characterized
ples joint contractures. He had two pathogenic variants in findings, there was an increase in symptoms related to by neurological defects. Conclusion: It is possible to cor-
LAMA2 (p.Tyr121* and Leu1581Profs*5), and spoke only ASD, greater difficulty in managing emotions, and the relate the inadequate performance of prenatal and the
few words, poorly functioning play, poor social interac- absence of school support was directly associated with occurrence of congenital defects, such as disorders in the
tion and only comprehended simple commands. Patient an increase in behavioral problems. There was also a nervous system, because, with the medical attendance,
3 was an eight years old girl, who presented with congen- reduction in physical activities, increased screen time the pregnant woman can be treated for a certain pathol-
ital hypotonia, muscle proximal weakness, but was able and changes in sleep habits. In most cases, the main ogies and can be orientated to have some precautions,
to walk unassisted, she also presented scoliosis. She had caregiver was female, and among caregivers occurred such as administrate folate (vitamin B9), which prevents
two pathogenic variants in LAMA2 (P.Cys1079Arg and an increase in their burden and their fears surrounding neural tube malformations. This guidance is essential to
exons 2-9 deletion). She spoke just simple phrases, had the pandemic, along with stress and mental health det- avoid harmful consequences to the fetus and to prevent
poorly functioning play, poor social interaction, wasn’t riments, such as an increase in anxiety, depression and the occurrence of premature birth, which can impair the
able to read or write and had stereotyped movements. less time for self care. Conclusion: The pandemic can maturational processes of the brain, leading to anatomi-
Both patients with LAMA2-CMD had white matter hyper- enhance inequalities for children in vulnerable groups, cal changes, functional deficits, that can perpetuate into
signal in T2 and FLAIR at Brain MRI, but none of them so it is essential to support the well-being of ASD chil- adolescence and adulthood, or death. In addition, in case
presented cortical malformation or epilepsy. All three dren and their families as we emerge in this new way of the fetus is already with the disturb, the prenatal allows
patients scored for ASD in the Child Autism Rating Scale living, focusing on making policies and approaches with to early diagnosis and treat the condition, increasing the
(CARS) and presented signs of ID. Conclusion: Given the individualized multidisciplinary planning, psychological survival percentage. In resume, performing prenatal care
gap in the literature regarding cognitive defects within resources and increased services for families in order to correctly, is essential for the prevention or the successful
the context of myopathies, we have presented patients facilitate reintegration and ensure the development of treatment of defects in the nervous system in neonates and
with genetical myopathies associated ASD and ID and the children. It is also important to make further studies for the reduction of mortality in children under 1 year old.
provided insight for clinicians handling these patients. to assess the effects of the pandemic.
As it may appear in a greater proportion of patients, early
screening and multidisciplinary programs is necessary Neurologia Infantil
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NEONATAL NEAR-MISS OF NEONATES BORN EPIDEMIOLOGICAL PROFILE OF DRUGS ASPECTS OF THE QUALITY OF LIFE OF
WITH CONGENITAL HYDROCEPHALUS IN USED IN PEDIATRIC NEUROLOGY CARE FAMILIES CARING FOR PATIENTS WITH
BRAZIL: ANALYSIS OF A DECADE OF PATIENTS WITH AUTISTIC SPECTRUM REFRACTORY EPILEPSY
DISORDER (ASD) AT A REFERENCE
Pedro Lucas Grangeiro Sá Barreto Lima, David Carla Lenita Coelho Siqueira, Isadora de Oliveira
Augusto Batista Sá Araújo, Sophia Costa HOSPITAL IN BRASÍLIA Cavalcante, Stefâni Soares dos Anjos, Lisiane Seguti
Vasconcelos, Lucas Soares Radtke, Carolina Carla Lenita Coelho Siqueira, Maria Luíza Marinho Ferreira, José Ribamar Pereira Neto
Figueiredo Santos, Paulo Ribeiro Nóbrega, Samir de Sá de Paula Lima, Gabriel Haiek Fernandes,
Câmara Magalhães Hospital Universitário de Brasília. Brasília DF, Brazil
Lisiane Seguti Ferreira, José Ribamar Pereira Neto Universidade de Brasília. Brasília DF, Brazil
Universidade Federal do Ceará. Fortaleza CE, Brazil Hospital Universitário de Brasília. Brasília DF, Brazil Hospital de Base do DF. Brasília DF, Brazil
Hospital Infantil Albert Sabin. Fortaleza CE, Brazil Universidade de Brasília. Brasília DF, Brazil
Hospital Universitário Walter Cantídio. Fortaleza carlalenita.med@gmail.com
Hospital de Base do DF. Brasília DF, Brazil
CE, Brazil
carlalenita.med@gmail.com Background: Adaptive family resources such as com-
padro.pe@hotmail.com munication, dominance, social support and financial
Background: Autism Spectrum Disorder (ASD) is a term well-being are associated with good social adaptability
Congenital hydrocephalus (CH) is the variety of hydro- used to describe a range of neurodevelopmental disorders in patients with epilepsy. However, to date few studies
cephalus present at birth and is related to impairment characterized by persistent deficits in communication were carried out to assess the impact of the presence of
in the cognitive and motor development of the children and social interaction and by the presence of repetitive a patient on the functioning of the family. In the case of
affected. In the context of this disease, neonatal near-miss or restricted behaviors and/or interests. Its prevalence refractory epilepsies, the impact is even more significant,
(NNM) is a tool to access the profile of these newborns was estimated, in 2012, between 1 and 5%, in devel- it is a stressful process which requires emotional mastery,
and evaluate the care offered to them. This study aims to oped countries, and it is increasing in recent years. Due physical integrity, as well as time, dedication, and finan-
describe and analyze the neonatal near-misses among to its impact on patients’ quality of life, it is crucial to cial resources. Objective: To assess the quality of life of
neonates born with congenital hydrocephalus (ICD-10 apply an appropriate and individualized treatment that family members who care for children with refractory
Q03) in Brazil from 2011 to 2020. Data from the Brazil Live encompasses behavioral and pharmacological interven- epilepsy. In addition, to present the characteristics of
Birth Information System (Sistema de Informações sobre tions (which do not treat ASD itself, but possible related the family’s social, economic and emotional conditions,
Nascidos Vivos/SINASC) were used. NNM was defined by symptoms, such as agitation and irritability). Objective: limitations, compromised by refractory epilepsy, evaluat-
having one or more of very low weight at birth (<1500g), To characterize the pharmacological treatment profile ing the expectations, anxieties, and fears of the parents in
asphyxia at 5 minutes from birth (APGAR at fifth-minute used by the pediatric neurology service of the Reference relation to the risks of Hospitalization, death and regard-
score ?7), or less than 32 gestational weeks at birth. A Hospital for patients with ASD. Method: A retrospective, ing the academic and professional future of the child.
descriptive analysis of the data was made and the trends descriptive, cross-sectional cohort study, using data col- Method: Descriptive study with a qualitative approach
in both near-miss numbers and their ratio to neonatal lected from the medical records of patients diagnosed carried out remotely with the caregivers of children/ado-
mortality were evidenced from 2011 to 2020. This trend with ASD followed at the Pediatric Neurology service lescents with refractory epilepsy, who are followed up in
included a regression model to describe the behavior of of the Reference Hospital. Results: The medical records a specialized outpatient clinic of a University Hospital in
the data along the time. In parallel, independent T-tests of 109 patients with ASD were evaluated, among which the Federal District. For data collection, a semi-structured
were used to compare the NNM-related variables between there was 1 loss of follow-up. Of the patients evaluated, form was used and an individual interview was carried
the group of neonates with CH and the live births in gen- 19.2% were using antidepressants (n=21) and 45.8% were out. Data collection was carried out through telephone
eral. From 2011 to 2020, a total of 2550 NNM cases were using antipsychotics (n=50). Among antidepressants, contact. Results: Four families participated in the study,
observed among the 7699 newborns with CH, resulting in the most used was fluoxetine, used by 52.3% (n=11) of aged between 20 and 42 years. In this study, it was found
a NNM rate of 331 cases per 1000 live births. 2013 was the patients using antidepressants, followed by sertraline that the children and adolescents participating in this
year with the highest NNM rate with a total of 380 NNM (47%). Regarding antipsychotics, risperidone was the most study perform most of their daily activities with the help
per 1000LB with CH, followed by 2012 (328 per 1000LB) used, representing 74% of this group (n=37), followed by of their family members, thus requiring greater support
and 2015 (335 per 1000LB). During the period, the NNMR pericyazine (14%), aripiprazole (8%) and haldol (6%). 8 and dedication from their caregivers. In addition, it was
trends presented two different behaviors, with an annual additional patients that had used risperidone in the past revealed that family members feel partially satisfied with
percent change (APC) of +1,84% from 2011 to 2013, and were identified, but had quit due to lack of response to their quality of life, this is due to several factors such as:
with an APC of -2,18% from 2013 to 2020, resulting in a the medication or side effects. Other medications such as lack of help from others to manage the child/adolescent,
total reduction of 7,85% in the ten years. Comparing the cannabidiol, methylphenidate and anti-epileptics were insufficient family income, collection, debt, overload,
CH group and the general neonates, the first group pre- being used by 19 patients. Conclusion: Pharmacological family conflicts, impact on marital life, feelings of guilt
sented a higher NNM rate (p < 0,001; 95% CI 279-317). interventions in autism are reserved for particular sit- and unemployment, which cause anxiety, hopelessness
Also, there was a higher NNM to mortality ratio (p < 0,05; uations in ASD and should have their use adjusted to and depression in family members. Conclusion: Caring
95% CI 0,10-0,62) in the neonates with CH, indicating a the needs of each patient. The main medications used for people with chronic neurological disorders has a
better success of care for the CH near-misses than for in the service analyzed were antipsychotics, especially negative impact on the family structure, causing psycho-
general near-misses, hence there are more cases of NNM risperidone, the first drug approved by the Food and logical stress due to the need for adaptations that are not
per overall deaths. Conclusion: This study applied the Drug Administration (FDA) to treat irritability related to always achieved. In this way, care must be analyzed, not
neonatal near-miss concept to analyze the live births autism, followed by antidepressants. Other pharmacolog- only from the perspective of the sick child, but extended
with congenital hydrocephalus in Brazil, presenting a ical treatments were implemented to control conditions to the entire family nucleus.
change of behavior in trends in the last 7 years. A com- such as agitation and insomnia, especially after a lack of
parative analysis showed that CH neonates present with response to the most commonly used medications, as a
more cases of NNM, but at the same time present with a Neurologia Infantil
way of individualizing treatment.
higher rate of NNM to mortality, indicating a recovery of
the newborns from the conditions of birth.
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MUSCLE ULTRASOUND AS A TOOL FOR KINSBOURNE SYNDROME: A CASE ANGELMAN SYNDROME: THE SEARCH FOR
RESPIRATORY ASSESSMENT IN PATIENTS PRESENTATION BRAIN KNOWLEDGE
WITH LAMA2-MD
Djanino Fernandes Silva, Hellysson Phyllipe Luan Monte Barroso, Cristiano Ribeiro Soares,
Clara Gontijo Camelo, Ana Lucila Moreira, Pedro Firmino Cavalcanti, Jeddson Rêgo Nascimento, Marina Mayara Pereira, Carina Maranga, Tiago
Augusto Sampaio Rocha Filho, Mariana Cunha Vanessa Asfura Pinto Ribeiro, Larissa Clementino Fernandes, Evguenia Bekman, Simão Teixeira Rocha
Artilheiro, Cristiane de Araujo Martins Moreno, Leite Sa Carvalho, Victor Felippe Bispo Macedo,
Paulo Barbosa Leite Neto, Marco Andre Moraes UNINOVAFAPI. Teresina PI, Brazil
Tatiana Ribeiro Fernandes, André Macedo Serafim
Silva, Umbertina Conti Reed, Edmar Zanoteli Bernardino, Fabiola Lys Medeiros, Adélia Maria luanmontebarroso@gmail.com
Miranda Henriques-Souza
Universidade de São Paulo. São Paulo SP, Brazil
Hospital Universitário Oswaldo Cruz da Background: Angelman Syndrome (AS) is a neurodevel-
claragc@gmail.com Universidade de Pernambuco. Recife PE, Brazil opmental disorder, considered incurable, characterized
Hospital da Restauração, SES. Recife PE, Brazil by ataxia, intellectual disability, speech impairment, sei-
Background: LAMA2-muscular dystrophy(LAMA2-MD) zures, autistic behavior, hyperactivity and happy behav-
is an autosomal recessive disease,and the most common djanino@gmail.com ior, caused by the loss of function of the UBE3A mater-
form of congenital muscular dystrophy (CMD).Most of nally inherited region of the chromosome 15q11-13 and
the patients develop a form of disease characterized by Case Presentation: Male infant, 1 year old and 10 months, diagnosis is usually made between 1 and 4 years of age.
inability to achieve walking capacity,multiple joint defor- third child of non-consanguineous parents, 2 healthy For a consensus criterion for diagnosing AS, the clinical
mities, respiratory insufficiency, and some degree of dys- older sisters and mother with 2 abortions, presenting features of individuals are divided by their percentage of
phagia.However, there is a gravity spectrum.There are still with aggressive behavior 3 days after immunization frequencies in the syndrome: consistent features, frequent
no adequate biomarkers to assess disease progression, with inactived polio vaccine and Hepatitis B vaccine features, associated features. Objective: To carry out a
and muscle ultrasound(US) can be a useful tool and also in December 2021. Progression to ataxic gait and dys- survey of current research on the definition of the profile
complement the assessment of respiratory and swallow- phagia after 15 days, with myoclonic movements of the of cases related to Angelman syndrome. Methods: This is
ing function.Objective: Evaluate, through muscular US, upper limbs and opsoclonus after 30 days. Admitted to a bibliographic review study, extracted from the PUBMED
the function of the respiratory muscles, tongue muscles the emergency department in January 2022, submit- and The Febs Journal database, using an understanding
and correlate them with respiratory function, degree of ted pelvis, abdomen and chest CT, Cerebrospinal fluid of the brain areas affected in AS. Results: Identified by
dysphagia, disease severity and age.Methods: 10 patients and brain MRI without alterations. Underwent 5 days noted physicians and researchers, Angelman Syndrome
with genetically confirmed LAMA2-MD were divided of pulse therapy with methylprednisolone at 30mg/kg/ is understood to be caused by the loss of the maternal
according to motor severity and evaluated.US of tongue, day, with no change. Then performed IVIG at 2g/kg in 5 copy of the UBE3A gene in the region of chromosome
respiratory and paravertebral muscles were made. For days, ceasing opsoclonus and improving gait. Myoclonus 15q11-13. To diagnose AS, the clinical characteristics of
muscles comparable to bone echo,the 4-point Heckmatt was controlled by Clonazepam. Discharged after 14 days individuals are divided into percentage of frequencies in
scale was used, for the others the classifications were but did not maintain the corticosteroid therapy at home. the syndrome. As consistent features (100%), all patients
hypoechoic, slightly hyperechoic, or very hyperechoic. May 2022, he presented recurrence of opsoclonus and with AS have ataxia, functionally severe developmental
Patients underwent respiratory function assessment and worsening of ataxic gait. Admission exam: Regular gen- delay, frequent laughter/smiling associated with hyper-
underwent neuromuscular disease swallowing status scale eral condition; irritated; unintelligible speech; trophism, motor behavior, and speech impairment with no or min-
(NdSSS).Results: Two patients had severe presentation tone and strength preserved. Exacerbated deep reflexes imal use of words, but greater use of non-verbal commu-
and were not able to sit without support.They presented (grade 3), globally. Exalted axial face reflexes. Did not nication. In the frequent features, 80% of AS cases show a
geniohyoid and genioglossus muscles very hyperechoic, load weight on the lower limbs, making gait assessment small head circumference associated with microcephaly,
had level 3 NdSSS dysphagia and required gastrostomy. difficult. Coordination with dysmetria and global decom- electroencephalogram abnormalities, and frequent and
They both had very affected external oblique, internal position. A new course of IVIG was performed, with no severe seizures during childhood that decrease with age
oblique and transverse muscles, but presented normal change to the clinical presentation. Then, a new course but may persist into adulthood. Associated features in
diaphragm, with normal thickening.They had altered of pulse therapy with methylprednisolone was realized 20 to 80% of patients with AS include obesity, scoliosis,
polysomnography.4 patients had classic disease presenta- with global improvement, except for gait ataxia, in a constipation, abnormal sleep cycles, wide mouth, squint,
tion, with maximum motor ability to sit without support, lesser degree. A new screening for occult neuroblastoma hypopigmented skin, attraction to water, and many oth-
and were under 6 years old.They presented geniohyoid was performed, with normal CT of the pelvis, chest and ers. Not all features need to be present for a diagnosis
and genioglossus muscles slightly hyperechoic, affected abdomen. Discussion: Kinsbourne syndrome is a rare of AS, but it is mandatory that patients exhibit the four
external oblique muscle with normal internal oblique and inflammatory pathology, with no clear etiology. Up to consistent features. Conclusion: It is believed that under-
transverse muscles and normal diaphragm, with normal 50% of cases are paraneoplastic, with neuroblastomas standing about AS in the brain will only be achieved with
thickening.They all had total lung capacity (TLC) above being identified in the majority of them. Despite the a faithful recapitulation of brain development processes
50% and level 7 NdSSS.Other 3 patients had classic dis- extensive search for neuroblastoma at this patient, no in emerging 3D models.
ease presentation but were older than twelve years old, tumor processes were identified. He is still awaiting to
who presented geniohyoid and genioglossus muscles perform full-body scintigraphy, in order to complete this
highly hyperechoic, affected external oblique, internal search. Conclusion: Early recognition of Kinsbourne syn- Neurologia Infantil
oblique and transverse muscles, with normal diaphragm, drome enables early treatment, leading to faster clinical
with normal thickening.They all had TLC below 35% and improvement, with less cognitive sequelae. There is no
level 7 NdSSS.Conclusion: Muscle US can be used as a consensus as to which immunomodulatory treatment
tool to evaluate disease progression and contribute to to use, which varies according to the response of each
the assessment of respiratory function and dysphagia patient. The absence of neuroblastoma at the time of the
in LAMA2-MD. clinical occurrence of the syndrome does not definitively
rule out its presence, requiring a new periodic evaluation.

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TL 1106515 TL 1106538 TL 1105459

BIOMARKERS OF BRAIN ELECTRICAL EVALUATION OF CHILD DEVELOPMENT PREOPERATIVE ENDOVASCULAR


ACTIVITY IN INDIVIDUALS WITH AUTISTIC OF CHILDREN WITH MICROCEPHALY BY EMBOLIZATION OF GLOMUS JUGULARE
SPECTRUM DISORDER CONGENITAL ZIKA VIRUS SYNDROME TUMORS: A RETROSPECTIVE CASE SERIES
OF 22 EMBOLIZATIONS IN 20 PATIENTS
Leandro Araújo Costa, Mariana Cardoso Costa da Camilla Vanessa Araújo Soares, Elidianne Layanne
Silva, Gustavo Maximiano Alves, Ana Karla Jansen Medeiros de Araújo, Pedro Nascimento Araujo Brito, Thaís Takamura, Luiza Gonçalves Fraga, Matheus
de Amorim Larissa Pereira Costa, Maria Eduarda Bezerra Sales Kahakura Franco Pedro, Ricardo Ramina, Murilo
Queiroz, Sâmela Laura da Silva Barbalho, Isabella Sousa Meneses, André Giacomelli Leal
Universidade Federal do Pará. Belém PA, Brazil Rodrigues Cordeiro, Julia de Melo Nunes, Juliana
Universidade de São Paulo. São Paulo SP, Brazil Sousa Soares de Araujo, Suellen Marinho Andrade Instituto de Neurologia de Curitiba. Curitiba PR,
Brazil
leandrocosta.dr@gmail.com Federal University of Paraíba, Faculty of Medicine.
João Pessoa PB, Brazil luizaag.fraga@gmail.com
Autism Spectrum Disorder (ASD) is a neurodevelop-
mental disorder characterized by neurological disorders, camilla.vanessa46@gmail.com Background: Glomus jugulare tumors are rare, highly
which includes communication deficit, decreased social vascularized though slow-growing skull base tumors
interaction, behavioral pattern, restricted and repetitive Background: Microcephaly is a congenital malforma- that originate from paraganglion cells of the neural crest.
activities and interests. The diagnosis of ASD is clinical tion in which there is inadequate brain development. Its The approach for these lesions remains controversial,
and based on the Diagnostic and Statistical Manual of causes are multiple, among them: the exposure of the neurosurgeons are often faced with challenges due to
Mental Disorders (DSM-5). This defines ASD as the entities mother to the Zika virus during pregnancy. Children may the rich vascularization of the lesions as well as to the
Asperger’s Syndrome, Autistic Disorder and Generalized have severe neurological impairment with the presence intimate relationship with neural and vascular struc-
Developmental Disorder not otherwise specified, which of brain abnormalities evidenced by intracranial calcifi- tures. To minimize the risks for the patient and ensure
include Childhood Disintegrative Disorder and Rett’s cations related to cell death, ocular abnormalities, par- total resection, a combined approach is ideal. Objective:
Syndrome. Due to the difficult diagnosis, numerous studies ticularly microphthalmia, sleep disturbances, excessive Evaluate the profiles and outcomes of patients submitted
with electroencephalogram (EEG) exams in autistic indi- irritability to touch, and epilepsy. In view of the import- to preoperative jugular glomus embolization in a brazil-
viduals have shown interesting results in the search for a ant neuro-repercussions, children need to be monitored ian tertiary care Hospital. Methods: The present study
biomarker of this condition. In this work, carried out by and evaluated regarding their development. Objective: is a retrospective analysis of a series of paraganglioma
analyzing the EEG record in autistic individuals (N = 22) in To evaluate the child development of children with con- embolizations between January 2008 and December
comparison to the control group (N = 8), it was found that genital microcephaly caused by the Zika virus. Method: 2019. The embolization was performed through super
in the TEA group there was a lower oscillation power of the A cross-sectional observational study was carried out, selective catheterization of feeder arteries between 3
low frequency waves (alpha, delta and theta) in relation assessing child development using the Ages & Stages to 5 days prior the surgical procedure; feeders from the
to the control group, and in alpha and theta this finding Questionnaires 3rd Edition (ASQ3 – BR) instrument. This external carotid artery and internal carotid artery were
occurred in the whole brain. Along with this, there was a study was approved by the ethics committee of the Federal embolized with gelatin foam, polyvinyl alcohol foam, or
slight predominance of the gamma oscillation power in University of Paraíba (CAAE: 42852621.0.0000.5188) and polyvynil alcohol particles, embolic spheres, Bead Block,
the TEA group in relation to the healthy group, another conducted according to the criteria of Resolution No. and ethylene-vinyl alcohol copolymer, or EVOH. The aim
fact was the inverse relationship between the oscillation 466/2012 of the National Health Council. Nineteen chil- of the treatment was radical and complete tumor removal
power of the low frequency waves (alpha, theta and delta) dren of both sexes were included in the study. Results: No in one surgical procedure with preservation of the cranial
and the power of range oscillation. child scored higher or close to the ASQ3 cut-off score. An nerves. Results: In the period studied 22 paraganglioma
important part of the sample showed severe impairment embolizations were performed in 20 patients, 11 of them
in all domains evaluated, scoring zero in the following had already been submitted to previous neurosurgical
Neurologia Infantil skills: communication (84.16%), broad motor (73.64%), procedures. There were 11 women and 9 men, with a
fine motor (89.42%), problem solving (84.16%) and per- mean age of 51.25 years old. All patients underwent both
sonal skills. and social (84.16%). Conclusion: The results embolization and surgery afterwards. Hearing loss was
of this study can provide evidence that alerts about the the most common symptom (50%), while 40% of patients
importance of continuous monitoring of these children by had tinnitus, 30% had dysphagia, 25% had facial paraly-
a multidisciplinary team, creating therapeutic proposals sis, 20% had hoarseness, and 10% had diplopia. The most
that stimulate the patient’s development, reducing dam- common vessel involved was the ascending pharyngeal
age and promoting quality of life. artery (90%), followed by the posterior auricular artery
(15%), the internal maxillary artery or the occipital artery
(10% each), and the superficial temporal or the lingual
Neurologia Infantil arteries (6% each). The embolization was deemed total
in 13 procedures, partial in 4 procedures, and was not
reported in the remaining 5. Only one patient had involve-
ment of the internal carotid artery. No complications
from embolization were recorded. Conclusion: Glomus
jugulare tumors are complex lesions that demand a mul-
tidisciplinary approach to ensure satisfactory resection
with preservation of lower cranial nerves. Preoperative
embolization is essential to ensure one-stage operation.

Neurologia Intervencionista

340
XXX Congresso Brasileiro de Neurologia

TL 1105466 TL 1105222 TL 1105278

FLOW DIVERTER ENDOLUMINAL DEVICE MONONEUROPATHY MULTIPLEX: A 100- FOLLOW-UP OF INDIVIDUALS WITH
(FRED) FOR THE TREATMENT OF CASE SERIES HEREDITARY TRANSTHYRETIN
INTRACRANIAL ANEURYSMS: 6 YEARS AMYLOIDOSIS
José Pedro Soares Baima, Angelina Maria Martins
CLINICAL RESULTS Lino, André Silva Franco, André Macedo Serafim Ligia Rocha Andrade, Salim Lazaro Balassiano,
Thaís Takamura, Luiza Gonçalves Fraga, Ana Silva, Nícolas Ruan dos Santos Cavalcante, Edmar eduardo barbosa, Eduardo Davidovich, Marcia
Carolina Felipe Silva, Lorena Maria Dering, Camila Zanoteli, Carlos Otto Heise rodrigues jardim, Izabela Rodrigues Pitta, Ana
Carneiro Ferreira, Bruno Camporeze, Matheus Siquara, Clarissa neves spitz, Larissa Bittencourt
Hospital das Clínicas da Faculdade de Medicina da
Kahakura Franco Pedro, Joel Fernando Sanabria Carvalho, Robson Vital
Universidade de São Paulo
Duarte, Murilo Sousa Meneses, André Giacomelli
Hospital Universitario Pedro Ernesto. Rio de Janeiro
Leal jpedrobaima@gmail.com
RJ, Brazil
Instituto de Neurologia de Curitiba. Curitiba PR, Background: Mononeuropathy multiplex is a group of ligiaandradee@gmail.com
Brazil uncommon disorders. Definition is involvement of two or
luizaag.fraga@gmail.com more noncontiguous peripheral nerves. National studies Background: Hereditary transthyretin amyloidosis is
are needed to understand local epidemiology, as numer- caused by a genetic mutation that leads to deposition of
Background: Flow diverter are one of several models ous causes have been identified. Methods: We retrospec- the malformed transthyretin protein in various organs
of metallic stents used in intracranial aneurysms (IAs) tively reviewed data of patients with an electrodiagnostic and tissues. Symptoms include peripheral neuropathy,
embolization. They show an advantage over endosaccu- diagnosis of mononeuropathy multiplex. Etiology was cardiac, renal, ocular, gastrointestinal involvement and
lar coiling since they treat the weakened arterial wall by divided as systemic vasculitis, isolated peripheral nerve carpal tunnel syndrome. Autonomic function is often
providing a scaffold for new endo-thelial cell growth to vasculitis, chronic inflammatory neuropathy, infection undervalued and is often compromised from the onset
occur. The FRED (Flow Diverter Endoluminal Device) is (including leprosy), genetic, neoplastic disorders, and of symptoms resulting in cardiac conduction defects,
a model of flow diverter that stands out for being a two- undetermined. Electrodiagnostic pattern was classi- orthostatic hypotension, urinary retention, sweating
layer braided spiral that helps to reconstruct the blood fied as axonal, demyelinating, intermediated (mixed), abnormalities, and sexual dysfunction. Genetic testing
vessel wall. Objective: Report the experience of the use of and associated pattern (clearly demyelinating lesions in in pre-symptomatic individuals is now widely available
the FRED dispositive in the endovascular treatment of IAs some nerves and axonal lesion in others). When avail- and may provide a proper follow-up. In clinical suspicion
and evaluate the safety and efficacy of this intervention. able, tissue biopsy was also reviewed. Results: A total of and altered complementary exams, the diagnosis must
Methods: This retrospective and single-center observa- 100 patients were included – mean age was 47,4 years, 48 be confirmed through tissue biopsy showing the deposit
tional study evaluates the use of FRED or FRED Jr in the were male. Most frequent etiology was systemic vascu- of amyloid material. Objectives: to make an early diag-
endovascular treatment of IAs from July 2016 to April 2022. litis with 51 patients. Among these, lupus was the most nosis of amyloidosis in asymptomatic carriers Methods:
Patients included were older than 18 years, and treated common diagnosis, but rarer vasculitis that are seldom Asymptomatic individuals with hereditary amyloidosis
with a FRED or FRED Jr dispositive; and were excluded associated with mononeuritis multiplex were also found. from the Neuromuscular Diseases of Neurology outpa-
if the implant did not occur for some complication or if Isolated peripheral vasculitis was seen in three patients. tient clinic of the Pedro Ernesto University Hospital in
they didn’t perform a control cerebral angiography after Leprosy was the most frequent infection with 28 patients, Rio de Janeiro are included in the study. A semi-annual
the procedure. Dual antiplatelet therapy (acetylsalicylic only two presenting with pure neural leprosy. Chronic follow-up is carried out and individuals are periodically
acid and clopidogrel) was managed 5 days before and 6 inflammatory neuropathy was represented by three cases submitted to neurological and neurophysiological exam-
months after the procedure and acetylsalicylic acid was of multifocal CIDP and 03 cases of multifocal motor neu- ination, evaluation of small fiber neuropathy (through QST
used for another 6 months. Data were collected from the ropathy. Genetic was represented by two cases of HNPP. (quantative sensory testing), sympathetic-reflex response,
medical records: sex and age, number and location of IAs, EDX was consistent with an axonal pattern in 70 cases, dysautonomia questionnaire, heart rate variability) and
complications, morbidity and level of IA occlusion – fol- demyelinating in 12, intermediated in 13, associated cardiological evaluation. Results: 10 individuals are being
lowing the O’Kelly Marotta (OKM) classification scale. in five. Entrapment neuropathies pointed towards an followed up and two individuals have developed signs and
All intraprocedural complications were reported. The infectious etiology. A total of 17 nerve biopsies, 44 skin symptoms of the disease, confirming the deposit of amy-
follow-up of patients was carried out 6 and/or 12 months biopsies, three salivary gland biopsies, and seven renal loid material through salivary gland biopsy. A 32-year-old
after the procedure with a digital subtraction angiography. biopsies were available to evaluation. Histology strongly woman with dysautonomia and bilateral carpal tunnel
Results: Between July 2016 and April 2022, 191 patients suggested vasculitis in 33 biopsies, and 19 biopsies were syndrome has the Phe84Leu mutation and the index case
were screened, which 31 met exclusion criteria. 135 diagnostic of leprosy. Conclusion: Facing a mononeurop- was her father, who had a late diagnosis and died due to
patients were female. The mean age was 53 years. A total athy multiplex, systemic vasculitis is the most frequent complications from the disease. A 22- year- old woman
of 198 AIs were treated – 178 of them in the internal carotid diagnosis. Very rare vasculitis types should also be con- with Val50Met mutation presented small fiber neuropa-
artery, mainly in the ophthalmic segment. Following the siderate. In our population, leprosy should always be a thy, with abolition of the reflex sympathetic response in
OKM scale for occlusion of IAs, 61% fulfilled category D; concern in asymmetric neuropathy. Although a diagnostic the 4 limbs and QST abnormalities when compared to a
while C, B and A were observed in, respectively, 11%, challenge, an etiology can be clearly identified in majority healthy control matched by sex and age. The index case
7% and 6%. 28 patients had not undergone control angi- of patients. Non neural tissue biopsies can be very helpful was his maternal aunt who died at age 38 due to dysau-
ography at the time of the study, but were not excluded with much less distress. tonomia. Conclusion: hereditary amyloidosis can be
because there was still time for follow-up. The morbidity difficult to diagnose, since its clinical manifestations are
rate was 16% (mRS = 1, mainly headache). No deaths were quite heterogeneous. An early diagnosis is essential for
Neuropatias Periféricas an effective treatment. Monitoring asymptomatic individ-
reported. Conclusion: IAs are more common in females
after 50 years old. In this study, the occlusion rate was uals is very important for early detection of the disease.
seen in 70% of the cases, a value that can still increase as
follow-ups are concluded. Thus, high efficacy in the use of
FRED in the endovascular treat-ment of IAs is suggested. Neuropatias Periféricas

Neurologia Intervencionista

341
XXX Congresso Brasileiro de Neurologia

TL 1105307 TL 1105355 TL 1105406

ULTRASOUND ABNORMALITIES OF THE POEMS SYNDROME: A BRAZILIAN CASE AVALIAÇÃO DA FORÇA PROXIMAL, DA
MEDIAN NERVE IN LEPROSY NEUROPATHY SERIES MARCHA, EQUILÍBRIO, DA FUNCIONALIDADE
AND CARPAL TUNNEL SYNDROME: HOW TO E DA QUALIDADE DE VIDA EM
Trajano Aguiar Pires Gonçalves, José Rosemberg
DIFFERENTIATE THESE CONDITIONS? Costa Lima Filho, João Marcus de Lima Brito Alves, PACIENTES COM POLIRRADICULOPATIA
Camila Derminio Donadel, Caroline Lavigne Moreira, DESMIELINIZANTE INFLAMATÓRIA CRÔNICA
Iago Resende Carvalho, Agnes Laura Silva Neres,
Isabela Corrêa Samper, Laura de Lourdes Cardoso Manoella Guerra de Albuquerque Bueno, André
Rouse Barbosa Pereira, Larissa Kozow Westin,
e Silva, Douglas Eulálio Antunes, Pedro Henrique Cleriston José Santos, Rodrigo Siqueira Soares
Carlos Bruno Nogueira, Camila Castelo Branco
Sirotheau Corrêa Alves, Andrea de Martino Luppi, Frezatti, Pedro José Tomaselli, Wilson Marques
Pupe, Osvaldo José Moreira Nascimento
Wilson Marques Júnior, Isabela Maria Bernardes Junior
Goulart, Diogo Fernandes dos Santos Universidade Federal Fluminense. Niterói RJ, Brazil
Faculdade de Medicina de Ribeirão Preto da
Universidade Federal de Uberlândia. Uberlândia Universidade de São Paulo. Ribeirão Preto SP, Brazil rousepereira@yahoo.com.br
MG, Brazil trajanoapg@gmail.com
Universidade de São Paulo. Ribeirão Preto SP, Brazil Chronic Inflammatory Demyelinating Polyradiculo-
neuropathy (CIDP) is an acquired disorder of the
iago.r.carvalho@ufu.br Background: Polyneuropathy, organomegaly, endocr-
Peripheral Nervous System (PNS) where the myelin sheath
inopathy, monoclonal-protein and skin changes (POEMS)
is affected. Its etiology is still unknown and in most cases
Background: Leprosy is a chronic infectious disease is a rare paraneoplastic syndrome. It may be difficult to
its evolution is progressive and symmetrical, with proxi-
caused by Mycobacterium leprae which primarily involves diagnose, specialty in developing countries facing spe-
mal and distal involvement of the upper and lower limbs,
the nerves and the skin. Its clinical forms are defined by cialist’s shortage. In this study we sought to describe
causing changes in gait, balance, functionality and qual-
the host immune response and bacillary load, resulting clinical, laboratory and electrophysiological characteris-
ity of life. Several assessment scales have been proposed
in a wide clinical spectrum. Currently, nerve assess- tics found in 19 patients diagnosed in a brazilian referral
aiming to improve patient follow-up and management of
ment in leprosy relies mainly on clinical evaluation and center. Objective: to describe the clinical and laboratory
secondary complications. The aim of this study was to
nerve conduction studies, but although neurophysiology characteristics of POEMS syndrome in a brazilian terciary
evaluate the impact of proximal lower limb strength on
provides detailed information about the dysfunction of center. Methods: POEMS diagnosis was based according
gait, balance, functionality and consequently in quality
affected nerves, it does not reveal anatomic changes, to current diagnostic criteria. Clinical data was retrospec-
of life in patients with CIDP. Scales that aid in the clinical
such as thickening and fascicular pattern changes. High- tively collected. Results: Fourteen out of 19 patients were
assessment of gait, balance, functionality, muscle func-
resolution ultrasonography (US) is an additional technique male (73.7%). The mean age at onset was 48,6 years old
tion and quality of life were used, such as: Dynamic Gait
that permits the examination of multiple nerve trunks (range 38 to 66) and the mean time for diagnosis was
Index (DGI), Performace Oriented Mobility Assessment
over a long course in a few minutes and is considered an 13,7 months (range 5 to 48). Nerve conduction studies
(POMA); Modified Rankin Scale (mRS), Medical Research
accessible and reasonably precise method. Objective: from all patients revealed a sensitive-motor demyelin-
Council (MRC) and The Medical Outcomes Study 36-item
This study aims to compare the sites of median nerve ating neuropathy. Six patients underwent nerve biopsy,
Short-Form Health Survey (SF-36) respectively, all in
enlargement in patients with leprosy and carpal tunnel and none had inflammatory infiltrate. Papilledema was
their brazilian versions. Seven patients participated in
syndrome (CTS). Methods: During the year 2021, were present in 7 patients (36,8%). All cases had albuminocy-
the research, most of them males (71.4%), mean age of
included in this study 15 leprosy patients and 14 patients tological dissociation. A lambda monoclonal component
48 years (median 41 years). According to the MRC, the
diagnosed with CST, totaling 27 nerves in each group. All was detected in serum from all cases, but clonal plasma
patients had proximal muscle weakness (hip flexors)
patients underwent a multisegmental US session of the cells on bone marrow biopsy were found in only 8 cases.
ranging in score from 3 to 4 (mean 3.57) and distal cru-
median nerves, performed by an expert radiologist. The Bone lesions was found in 18 cases (94,7%). Sixteen (84%)
ral muscle weakness ranging from 2 to 4 (mean 3.29).
median nerve was scanned at the carpal tunnel (Mt) and had typical skin changes. Endocrinopathy was present
According to the DGI scale, all participants were at risk of
4 cm above the carpal tunnel (Mpt). The measurements of in 17 cases (89,9%), lymphadenomegaly in 6 (31,5%),
falling with a score equal to or below 19 points (mean of
the cross-sectional area (CSA) and morphological char- splenomegaly in 9 (47%), hepatomegaly in 6 (31,5%) and
17.43). According to the POMA scale, one patient (14.2%)
acteristics such as echogenicity and fascicular pattern thrombocytosis in 13 (68,4%), respectively. Two cases had
had a high risk of falls with a score of 19, five participants
were assessed at these two points. We also calculated Castleman’s disease. Conclusion: POEMS syndrome is a
(71.6%) had a moderate risk of falls, with scores between
the absolute difference between CSAs of the nerve in rare condition. Our data suggest that the prevalence of
20 and 24, and only one participant (14.2%) had no risk
the Mt and Mpt (?Mtpt = Mt – Mpt). Results: CSA of the endocrinopathy and bone lesions in our cohort were more
of falls, with a score of 25. The average among individu-
median nerve was increased in leprosy (13.4; ±4.4) and frequently found than expected, and may be related to a
als was 22.71 points on the POMA scale.It was observed
CTS (12.4; ±4.6) nerves at the Mt, but with no signifi- longer time of disease duration leading.
that the physical aspects obtained the lowest score in the
cant difference. 100% (27/27) of the nerves with leprosy average of the population under study in relation to the
showed some morphological abnormality at the Mt point components of the SF-36, impacting more significantly
Neuropatias Periféricas
and only 40.7% (11/27) of the CTS group (p=0.0042). on the sample. It can be concluded from this study that
CSA was higher among nerves with leprosy (17.0; ±6.7) proximal and distal paresis in the lower limbs in individ-
as compared to CTS (6.6; ±1.3) at Mpt (< 0.0001). 100% uals diagnosed with typical CIDP affects normal gait and
(27/27) of the nerves with leprosy showed some morpho- balance patterns, according to the DGI and POMA scales,
logical abnormality at the Mpt point and none of the CTS and these scales can be a risk predictor of falls in this
group. The ?Mtpt in leprosy nerves was -3.6 (±6.5) and population.It was also observed that functionality in this
5.7 (±4.0) in the CTS (p< 0.0001). A measure of the ?Mtpt population, despite being a chronic and progressive dis-
? 2.2 indicates the diagnosis of CTS with a sensitivity of ease, is not severely affected, according to the mRS scale.
100% and specificity of 96.3%. Conclusion: Median nerve
enlargement in leprosy using US seems to be more prox-
imal than in carpal tunnel syndrome (CTS), which might Neuropatias Periféricas
be an important discriminating sign.

Neuropatias Periféricas

342
XXX Congresso Brasileiro de Neurologia

TL 1105509 TL 1105599 TL 1105746

A LONG-TERM FOLLOW-UP OF BRAZILIAN EPIDEMIOLOGICAL AND CLINICAL SEVERE CHRONIC RECURRENT


PATIENTS WITH TRANSTHYRETIN FAMILIAL CHARACTERISTICS OF GUILLAIN-BARRÉ DEMYELINATING POLYNEUROPATHY AFTER
AMYLOID POLYNEUROPATHY AFTER LIVER SYNDROME CASES TREATED AT A PUBLIC A SINGLE DOSE OF CHADOX1-S NCOV-19: A
TRANSPLANT UNIVERSITY HOSPITAL IN BRAZIL BETWEEN CASE PRESENTATION
2014-2020
Caroline Lourenço de Medeiros, Isabela Silva Souza, Lilliane Rodrigues Soares, Daniele Santos Andrade,
João Nicoli Ferreira dos Santos, Carolina Lavigne Leonardo Peixoto Garcia, Agnes Laura Silva Felipe Edson Couto dos Santos, Karollyne de
Moreira, Rodrigo Siqueira Soares Frezatti, Marcus Neres, Iago Resende Carvalho, João Paulo Moreira Castro Santos, Catarina Ester Gomes Menezes,
Vinicius Simões, Pedro José Tomaselli, Wilson Fernandes, Mateus Barros Bueno, Clarice Pereira Marcos Baruch Portela Filho, Paula Cely da Silva
Marques Júnior Sales Oliveira, Maria Cecília Inácio, Gabriele Reis Torres, Lécio Aragão Souza Morais, Jefferson Heber
dos Santos, Maria Teresa Mamere de Almeida, Diogo Marques Fontes Júnior, Mateus Santana do Rosário
Hospital das Clínicas da Faculdade de Medicina
Fernandes dos Santos
de Ribeirão Preto – Universidade de São Paulo. Universidade do Estado da Bahia. Salvador BA,
Ribeirão Preto SP, Brazil Universidade Federal de Uberlândia. Uberlândia Brazil
MG, Brazil União Metropolitana de Educação e Cultura UNIME
carolinelmedeiros@gmail.com
peixotogarcialeo@gmail.com catarina.gomes.menezes@gmail.com
Background: Transthyretin familial amyloid polyneu-
ropathy (ATTRv-pn) is an inherited neuropathy, with Background: Guillain-Barré syndrome (GBS) is the most Case Presentation: Female, 33 years old, was vaccinated
over 140 mutations identified in the transthyretin (TTR) common acute inflammatory neuropathy, which causes with a single dose of ChAdOx1-S nCOV-19 (Fiocruz-
gene. ATTRv-pn is a severe length-dependent sensorim- sensitive and motor symptoms, and can also be associ- AstraZeneca) at 02/02/2022. She was admitted to an emer-
otor/autonomic neuropathy, which if untreated, leads to ated with dysautonomia. In Brazil, there are few epide- gency unit on 02/26/2021 with complaints of paresthesia in
death in approximately 10 years (y). The liver produces miological and systematized regional studies about the palate and limbs. CSF analysis showed: glucose of 52 mg/
98% of the TTR circulating in the blood, and it was thought syndrome. Objective: The aim of the present study is to dL; proteins 72 mg/dL; 1 cell/mm3; and C. jejuni IgG ELISA
that liver transplant (LT) would stop disease progression. evaluate the epidemiological, clinical, and neurophysi- indeterminate levels (0.93). Electroneuromyography
However, subsequent studies showed that the Results: ological aspects of patients with GBS, treated at a public (ENMG) identified acute motor and sensory demye-
were not good in late-onset patients (? 50y), those who university Hospital, between 2014 and 2020. Methods: linating polyneuropathy on all four limbs. She evolved
were malnourished, with advanced disease and/or had It is a retrospective study made by the analysis of 64 within 2 days with distal flaccid tetraparesis, dyspho-
non-V50M mutations. It proved to be very effective in medical charts of patients diagnosed with GBS, between nia, dysphagia and dyspnea. At this point, a diagnosis of
controlling the neuropathic manifestations, but less effec- January 2014 and December 2020. Results: The major- Guillain-Barré Syndrome (GBS) was made. Intravenous
tive in controlling cardiac manifestations. Objective: To ity of the patients are men (70,3%), with an average age human immunoglobulin (IVIG) was started 3 days after
describe the long term follow up of a series of ATTRv of 39,1/±23,3. 79,7% of the patients claim to have had symptom onset. Five days after IVIG, she developed facial
brazilian patients with polyneuropathy who underwent an infection before the symptoms, especially gastroin- diplegia, worsening of tetraparesis, and dysautonomia.
LT. Method: We have reviewed the medical records of all testinal (51.0%), respiratory (25.5%), and arboviruses A new IVIG infusion was performed 6 days after the first
patients (8) with ATTRv-pn who underwent LT and were (13.7%). Only 3.1% had a recent vaccination. Among the one, with improvement of limb paresis. In march 2021 she
followed in our institution. The diagnosis was made by clinical forms of GBS, the most common was the classic was readmitted with worsening of the facial diplegia, and
genetic testing. We have used the Composite Autonomic sensory-motor form (70.3%) followed by the pure motor plasmapheresis was instituted with good discussion. In
Symptom Score 31 (COMPASS 31) for quantitative mea- form (23.4%). The pharyngeal-cervical-brachial (3.1%), april 2021 she returned with worsening dysphonia and
sure of autonomic symptoms. Results: All patients had paraparetic (1.6%), and Miller-Fisher (1.6%) forms were paresis, with weak positivity to c-ANCA. Thus, suspicion
the ATTRv-pn V50M variant. The mean age at symptoms less commonly observed. Regarding the physical exam- was changed to ANCA-related vasculitis associated with
onset was 31,2y. All patients had neuropathic pain before ination at admission, all cases presented muscle weak- polyneuropathy. She was then treated with methylpred-
LT, and 2 of them experienced complete pain relief after ness. Areflexia or hyporeflexia was present in 89.1% of the nisolone and cyclophosphamid, evolving with worsen-
it. All patients have clinical dysautonomia and the mean patients. Other clinical findings observed: sensory ataxia ing of clinical state with need of mechanical ventilation.
of the last COMPASS 31 was 52,6. 50% have confirmed (48.4%), pain (46.9%), hypopalesthesia (43.8%), hypoes- A new plasmapheresis and rituximab were performed,
cardiomyopathy; 37,5% are still under investigation and thesia (40.6%), paresthesia/dysesthesia (39.1%). 34.4% of with a good clinical response. She was readmitted in May
12,5% do not have it. 87,5% have some arrhythmia or the patients had involvement of some cranial nerve and 2021 with worsening weakness and treated with plasma-
conduction-system disease and 50% needed pacemaker 23.4% had facial paresis. Dysautonomic symptoms such pheresis and IVIG. Specific antibodies were negative for
implantation between 2002 and 2022. The mean time from as tachycardia, dyspnea, and urinary retention/intestinal systemic vasculitis, and a diagnosis of recurrent chronic
symptoms onset to diagnosis was 2y and from symptoms constipation have been observed in 23.4%, 17.2%, and demyelinating polyneuropathy (CIDP) was made. It was
onset to LT was 5,37y. 6 patients are fully ambulants – 9.4%, respectively. In 25.0% of the cases, there was an opted for a monthly maintenance of IVIG from 08/2021
Coutinho stage (CS) 1 -, 1 patient needs one walking aid asymmetric distribution of sensory abnormalities and in until the present time. Currently, the patient is stable
– CS 2 – and 1 patient became wheelchair-bound – CS 3 the assessment of motor function, there was a proximal with preserved muscle strength. Final comments: CIDP
– in the last 2y. The mean time from symptoms onset to predominance in 84.4% and an asymmetric distribution is characterized by autoimmune, inflammatory, demy-
the last clinical evaluation (2022) was 18,2y (8-23y), and in 40.6%. The AMAN and PIDA neurophysiological pat- elinating lesions affecting peripheral nerves and nerve
from LT to the last clinical evaluation (2022) was 13,5y terns were the most commonly observed, both present roots, with a chronic or remitting/recurrent course of
(6-18y). Conclusion: Our patients have most of the fac- in 40.3% of patients who underwent electroneuromyog- neurological disability and is treatable with immuno-
tors for good prognosis cited in the literature, as V50M raphy. The time between symptoms onset and admission modulatory therapies. Neurological complications have
mutation, early-onset and early-stage disease when they was 14.2/±13.0 days, with a Hospital stay of 11.3/±11.4. been reported after COVID-19 vaccination and C. jejuni
underwent LT. The 20-y patient survival rate was 62,5%, 15.6% required ICU admission and 12.5% developed infection. Longitudinal follow-up confirms the diagnosis
higher than the average in the literature (55,3%). The respiratory failure and required mechanical ventilation. of CIDP; the causal relationship with COVID-19 vaccina-
mean time from symptoms onset to diagnosis was lower Conclusion: In the context of public health, it is important tion is possible considering other similar case presenta-
than it is in Brazil (5,9y). to recognize the clinical and epidemiological aspects of tions on literature. The absence of diarrhea and the weak
the GBS cases, to elaborate more efficient diagnosis and antibody reactivity against C. jejuni decrease the chance
treatment strategies. of this agent as etiology.
Neuropatias Periféricas

Neuropatias Periféricas Neuropatias Periféricas

343
XXX Congresso Brasileiro de Neurologia

TL 1105799 TL 1105873 TL 1106004

GUILLAIN-BARRÉ SYNDROME AFTER CORRELATION OF PERFIL DOS PACIENTES PORTADORES DE


COVID-19 VACCINATION: CASE SERIES ELECTRONEUROMIOGRAPHIC FINDINGS IN NEUROPATIA PERIFÉRICA DO MEMBRO
LEPROSY, ACCORDING TO THEIR CLINICAL SUPERIOR ACOMPANHADOS EM UM
Guilherme Machado Vieira, Ciro Vespasiano
Coutinho Tavares, Gabriela de Paula Abranches, FORMS HOSPITAL DA CIDADE DO RECIFE
José Dias de Assis Neto, Luis Henrique Sunderhus Leonardo Peixoto Garcia, Isabella Sabião Borges, Maria Ercilia Lima Barreiro
de Oliveira, João Pedro Moraes Miossi, Rodrigo Leite Douglas Eulálio Antunes, Isabela Maria Bernardes
Marinho, Kézia de Souza Pinheiro, Gustav Barbosa Goulart, Diogo Fernandes dos Santos Faculdade integradas do Recife. Recife PE, Brazil
Falcão, Mariana Lacerda Reis Grenfell
Universidade Federal de Uberlândia. Uberlândia maria.ercilialimabarreiro@gmail.com
Universidade Federal do Espírito Santo – Vitória – MG, Brazil
ES – Brazil Background: The peripheral neuropathy of upper limb
Centro Universitário Multivix – Vitória – ES – Brazil peixotogarcialeo@gmail.com functional bring great damage, interfering with the qual-
Hospital Universitário Cassiano Antônio Moraes – ity of life and socioeconomic conditions of individuals.
Vitória – ES – Brazil Background: Leprosy is the most frequent infectious Objective: To observe the prevalence of peripheral nerve
cause of peripheral neuropathy in the world and can lead lesions of the upper limb in a public service of the city of
gmachado98@outlook.com to sequelae and physical disabilities that perpetuate the Recife, nothing in the prevalence of the type of disease
prejudice and stigma linked to the condition, especially or type of trauma that affect those individuals more.
Background: Guillain-Barré syndrome (GBS) is an acute due to the delay in diagnosis. Objective: To describe the Methods: A cross-sectional study of descriptive and
or subacute immune-mediated polyradiculoneuropathy electroneuromyographic findings in patients with leprosy, analytical, with composite sample of 71 patients in the
generally preceded by an infectious trigger. The first link according to their clinical forms. Methods: The study is a neurosurgery clinic of the Hospital of the Restoration-
between GBS and vaccines was an 8-fold increase in inci- retrospective observational analysis of medical records Recife. Taking as a criterion for inclusion, patients with
dence of GBS in receivers of a Swine influenza vaccine in of patients with leprosy, from a Tertiary Referral Center, traumatic peripheral nerve lesions and / or compres-
1976. Recently, MMR, HPV, MCV4 and Influenza vaccines between the years 2014 and 2021, to correlate their clinical, sive upper limb, and exclusion of patients with obstetric
were pointed as possible triggers, but this association is epidemiological, laboratory and electroneuromyographic brachial palsy, leprosy and peripheral polyneuropathy.
questionable. During the coronavirus (COVID-19) pan- findings. Results: Among the 513 medical records ana- Results: Average age of patients: 43, 5 ? 16, 0 years, the
demic, cases of GBS preceded by COVID-19 vaccination lyzed, 71.7% patients (368/513) had neural thickening, most common disease in women was a mononeurophaty
have been reported, especially associated with viral vec- 73.3% (376/513) had sensory symptoms, 49.3% (253/513) of the median nerve in the wrist (91, 2%, n= 31), in males
tor-based vaccines. Objective: To describe cases of GBS had motor symptoms and 50.5 % (259/513) had leprosy the traumatic brachial plexus injury, with the etiolog-
diagnosed within 6 weeks after COVID-19 vaccination reaction, being 34.9% (179/513) type I and 15.6% (80/513) ical mechanism motorcycle accident (59, 5%, n = 22).
during the mass vaccination period. Methods: We per- type II. Regarding electroneuromyographic characteris- Conclusion: Was obtained knowledge of the prevalence of
formed a retrospective review of all patients diagnosed tics, the most affected sensory nerve was the superficial peripheral neuropathies in a reference service in the city
with GBS within 6 weeks after receiving COVID-19 vacci- fibular nerve (15.5%), while the most affected motor of Recife, as an incentive for greater integration between
nation admitted at our service from January 19th, 2021 to nerve was the ulnar (12.6%). Regarding neural involve- the interdisciplinary team and greater involvement of the
August 22nd, 2021. Exclusion criteria was the presence of ment, 126 patients had normal electroneuromyogra- physiotherapist in the rehabilitation of these patients.
other probable triggers during this period, including doc- phy (ENMG) and, among the 387 with abnormalities in
umented COVID-19 infection. Collected data include gen- ENMG, 13.2% (51/387) had mononeuropathy (MN) and
der, age, vaccine received, time between vaccination and 86.8% (336/387) had multiple mononeuropathy (MMN). Neuropatias Periféricas
symptoms onset (latency), neurological manifestations, Among the patients with the indeterminate (I) form
cerebrospinal fluid analyses, electrophysiological study, (4.9%), 8% had abnormal ENMG and none had sensory/
treatment and outcome at Hospital discharge. Results: motor axonal impairment. In the primary neural leprosy
During the studied period, 7 patients met the inclusion (PNL) (20.3%), 96.2% had abnormal ENMG, being 88%
criteria (6 men and 1 woman) with an average age of 53,4 with MMN and 6.9 (± 5.3) abnormal nerve/patient. In the
years old. 6 received ChAdOx1-S vaccine (AstraZeneca) tuberculoid (T) form (1%), 80% had abnormal ENMG,
and 1 received Ad26.COV2.S vaccine (Janssen). Except for 50% with MMN and 3.0 (±2.1) abnormal nerve/patient.
one patient, who developed symptoms 1 day after immu- In the borderline-tuberculoid (BT) form (49.3%), 68%
nization, all other patients had a latency time between 12 had altered ENMG, 80.8% had MNM and mean number
and 23 days. We identify that 5 of 6 patients who underwent of altered nerves was 5.9 (± 5.2). In the borderline-dimor-
lumbar puncture had an albuminocytological dissociation. phic (BD) form (6.2%), 78.1% had altered ENMG, being
Among the 5 patients who underwent electrophysiological 96% with MMN and 8.7(± 5.5) abnormal nerve/patient.
study, 3 met the criteria for the variant of AIDP, 1 met bor- In the dimorpho-virchowian (DV) form (7.2%), 91.9% had
derline criteria for AIDP and 1 had a pattern compatible altered ENMG, all of whom had MNM, with 10.1 (±4.1)
with AMSAN. Clinical presentation was heterogeneous, abnormal nerve/patient. Among lepromatous (LL) form
requiring individualized treatment. The patients received (11.1%), 98.2% had altered ENMG, with 98.2% MNM and
intravenous immunoglobulin, plasma exchange or both. 7.8 (± 4.3) abnormal nerve/patient. Axonal involvement
The 3 non-severe patients were discharged with mild to was more frequent in the PNL, DT, DV and LL. Conclusion:
moderate paresis and among the 4 patients that required Leprosy is a spectral disease, characterized by a balance
intensive care, one was discharged to rehabilitation with between host immunity and bacillary load. Therefore,
tetraparesis and 3 were transferred to other services. the impairment and neurophysiological characteristics
Conclusion: This study reported cases in which there are distinct and may vary according to the clinical form.
was temporal association between COVID-19 vaccines
and development of GBS. Further studies are required
to establish if there is a causal relation. Neuropatias Periféricas

Neuropatias Periféricas

344
XXX Congresso Brasileiro de Neurologia

TL 1106097 TL 1106255 TL 1106452

EVALUATE, IDENTIFY, INTERVENE: CLINICAL AND GENOTYPICAL SPECTRUM OF TEMPORAL ASSOCIATION OF GUILLAIN-
PRACTICAL REFLECTIONS FOR THE HEREDITARY MOTOR NEUROPATHIES BARRÉ SYNDROME WITH COVID-19
IMPROVEMENT OF HEALTH INDICATORS VACCINATION
Pedro Henrique Marte de Arruda Sampaio, Alulin
IN THE TREATMENT OF GUILLAIN-BARRÉ Tacio Quadros Santos Monteiro Fonseca, Cristiane Barbara Matos Almeida Queiroz, Matheus Costa
SYNDROME de Araujo Martins Moreno, Clara Gontijo Camelo, Bessa, Karoline Ferreira Mororo Menezes, Hermany
André Macedo Serafim Silva, Rodrigo Holanda Capistrano Freitas, Raynrich Kevin Assis Lima,
Francisco de Assis Pinto Cabral Júnior Rabello,
Mendonça, Edmar Zanoteli Alissa Elen Formiga Moura, Robson de Sales
Camila Castelo Branco Pupe, Mauro Eduardo Jurno,
Osvaldo J M Nascimento Ferreira, Norberto Anizio Ferreira Frota, Cleonisio
Department of Neurology, Faculdade de Medicina
Leite Rodrigues, Fernanda Martins Maia
da Universidade de São Paulo
Universidade Federal Fluminense. Niterói RJ, Brazil
Fundação Hospitalar do Estado de Minas Gerais – Hospital Geral de Fortaleza. Fortaleza CE, Brazil
phsampaio@gmail.com
Barbacena – MG – Brazil Universidade de Fortaleza – Fortaleza – BA – Brazil

francisco_cabral@id.uff.br Background: The hereditary motor neuropathies (HMN), barbaramatosaq@gmail.com


also known as distal spinal muscular atrophies, are genet-
ically diverse group of diseases with predominantly distal Background: Guillain-Barré Syndrome(GBS) is an
Background: Guillain-Barré syndrome (GBS) affects about
lower motor neuron symptoms. More than twenty genes acquired autoimmune disorder in which nerves myelin
100,000 new patients worldwide every year. Mortality
have been identified, with up to 60% of patients achiev- sheath is lost, especially the peripheral ones, leading
from this disease can range from 3 to 10%, depending, in
ing a definite genetic diagnosis. Clinically, the HMN lie to varying degrees of progressive and ascending motor
terms of modifiable factors, on access to adequate health
on a clinical spectrum alongside axonal Charcot-Marie- weakness. The trigger is usually associated with respira-
care, which can be problematic in low income regions.
Tooth disease and hereditary spastic paraplegias. The tory and gastrointestinal infections, and, rarely, immu-
Objetive. To determine the mortality rate, clinical compli-
HMN can be further divided into different clinical phe- nization. Objectives: To report a possible association of
cations profile, length of Hospital stay, time to initiation of
notypes, such as classical distal HMN, scapuloperoneal Guillain-Barre Syndrome to COVID vaccination. Methods:
pharmacological treatment and presence of side effects
HMN (SPHMN) and spinal muscular atrophy with lower Description of 7 cases with post-vaccinal acute polyradic-
in Barbacena´s health care region (251630 population).
extremity predominance (SMALED). Objective: In this uloneurophaty. Results: In our series of seven cases of
Results: 33 patients were included in the analysis. The
ongoing study, we describe the clinical and genotypical post-vaccinal COVID GBS, we observed no gender dis-
mortality rate due to GBS was 6%. The rate of clinical com-
findings of patients with HMN in a tertiary healthcare parities, 3 males and 4 females, in an age range of 18-50
plications during Hospital stay was 48,5% (n=16), mainly
service. Methods: We included 22 patients with clinical years old. All vaccines were ChAdOx1-S[recombinant],
infection/sepsis (n=8, 24,2%), cardiac arrhythmias (n=5,
and electrophysiological diagnosis of HMN. We performed with 6 patients presenting symptoms after first dose and
15,2%), intestinal constipation (n=5, 15,2%) and decubi-
whole exome sequencing on 8 patients and a genetic panel 1 patient after second dose. One patient had had H1N1
tus ulcers (n=3, 9,1%). All patients with GBS were treated
for hereditary neuropathies on 7 patients, and 6 others vaccine a week before the sars-cov-2 vaccine first dose.
with immunoglobulin (IgIV). About 78,8% (n=26) started
await genetic testing. Clinical evaluation included pheno- Ascending paraparesia began 15 days post vaccine, evolv-
IgIV within 2 weeks of symptom onset, and 21,2% (n=7)
type and other neurological findings, such as pyramidal ing to tetraparesia in 6 patients. Two patients had respi-
between 2 and 4 weeks of disease presentation. There was
signs. Variant classification was in accordance with ACMG ratory insufficiency, needing mechanical ventilation. All
no treatment-related fluctuation and no need for retreat-
criteria. Results: Of the 22 patients, 15 were males and 7 patients had celular-proteic dissociation in CSF analysis,
ment with IgIV in any patient. Only 6,1% (n=2) of patients
were females. Classical distal HMN was the most common with proteinorraquia ranging from 92mg/d to 578mg/dl.
had IgIV side effects, both of then had headache and one
phenotype, with 17 patients. Of those, one patient pre- Electroneuromyography showed a demyelinating pattern
of then had also diaphoresis and sinusal tachycardia. No
sented with dHMN and myokymia, with an inconclusive in five cases, but 3 were associated with secondary axo-
patient was treated with plasmapheresis. Most patients
genetic panel that included HINT1 gene. Another patient nal degeneration.Other two patients showed an axonal
were Hospitalized for up to 2 weeks (45,5%). A small pro-
had a dHMN with upper limb predominance (Type V), pattern. Conclusion: The described cases had a temporal
portion (12,1%) was Hospitalized for more than 30 days.
split hands and lower limb pyramidal signs, with a variant association of GBS with ChAdOx1-S[recombinant] vac-
Conclusion: Although the length of Hospital stay was
of uncertain significance (VUS) in the GARS gene. Other cination. In literature, the interval between trigger and
considered adequate in most cases (up to 2 weeks) and
genes associated with dHMN were VCP, SETX and HSPB1. symptoms can vary, with some studies reporting 6 weeks
access to first-line treatment (IgIV) was excellent, the rate
Three patients presented with a SPHMN, with distal lower onset after exposure.In our series of cases, mean time of
of clinical complications during Hospital stay was high,
limb weakness and prominent scapular winging, two symptoms onset was after 15 days of vaccination. The
specially infections/sepsis and arrhythmias, wich points
with a pathogenic variant on TRPV4 gene and another massive global program of sars cov2 vaccine provoked a
to the need of protocols for better prevention of noso-
with pyramidal signs and a probable pathogenic variant surge of neurological complications, including GBS cas-
comial infection, frequent evaluation of the swallowing
on BICD2 gene. Two patients had a SMALED phenotype, es,a phenomenon well described in literature.However,
capacity to avoid bronchoaspiration pneumonia, rotation
with probable pathogenic variants in DYNC1H1 in one this association is rare and the vaccination remains the
of decubitus to avoid ulcers, routine cardiac monitoring
patient and BICD2 in another. Of the 15 patients with most effective method of COVID-19 prevention.
and prompt treatment of dysautonomic complications.
genetic analysis, 11 had a conclusive genetic diagnosis.
Non-ischemic chest pain due to IgIV would be an uncom-
Conclusion: There are few epidemiological studies on
mom side effect (0,1-0,9%) of the medication, according
HMN, and to our knowledge, no systematic study has Neuropatias Periféricas
to manufacturer. But in this study sample, this symptom
been conducted in Brazil. Interestingly, we found bial-
was present in 6,1% of patients. The authors had a good
lelic variants in VCP gene causing distal HMN, as well
experience in the treatment of this side effect by reduction
as a patient with SPSMA with a BICD2 variant, a gene
of IgIV flow to a minimum of 0,5ml/kg/hour.
normally associated with SMALED phenotype. Our study
demonstrates the diverse clinical picture of the HMN.
Neuropatias Periféricas
Neuropatias Periféricas

345
XXX Congresso Brasileiro de Neurologia

TL 1106477 TL 1106556 TL 1104844

EVALUATION OF QUALITY OF LIFE IN MORTALITY PROFILE OF PATIENTS WITH THE EFFECTIVENESS OF THE
PATIENTS WITH FAMILIAL AMYLOID INFLAMMATORY POLYNEUROPATHY IN NEUROMODULATION TECHNIQUE USE IN
POLYNEUROPATHY BRAZIL BETWEEN JANUARY 2010 AND BRAZILIAN HOSPITALS: A SYSTEMATIC
DECEMBER 2020 REVIEW
João Gustavo dos Anjos Morais Oliveira, Marcela
Câmara Machado Costa Fernanda Souza Gracílio Silva, Ana Rafaela Soares Marco Antonio Martins Barbosa, Leo Araújo Oliveira,
Vale, Isaac Rêgo Purificação, Henrique Nascimento Érica Dapont Moura, Sarah Gurgel Ponte, Frederico
Escola Bahiana de Medicina e Saúde Pública.
Dourado, Victória Faustino Reis, Lara Cordeiro Carlos Arnaud
Salvador BA, Brazil
Magalhães, Hugo Oliveira Polito Barreto, Gabriel
Vianna Pereira Aragão, Filipe Augusto Silva Leite Universidade de Fortaleza. Fortaleza CE, Brazil
jogusdamo@gmail.com
Escola Bahiana de Medicina e Saúde Pública. marco@edu.unifor.br
Background: Amyloidosis are a group of diseases caused
Salvador BA, Brazil
by deposition of amyloid fibrils. A subgroup of those Background: The effectiveness of the neuromodulation
pathologies is associated with a gene mutation in a pro- anavale19.2@Bahiana.edu.br technique leaves doubts that prevent its proper use. The
tein called Transthyretin, which may cause a series of process consists of the use of stimuli, such as electrical
affections, including a neurological manifestation, called Background: Inflammatory polyneuropathy is clinically or chemical, to trigger the central or peripheral nervous
Familial Amyloid Polyneuropathy (FAP). FAP is a rare, marked by progressive symptoms of weakness affecting system, improving its function in the human body. Having
progressive and uncurable disease, capable of causing the distal and proximal segments with consequent loss its first reports only in the 20th century, the technique is
great impact in patients’ quality of life. Objectives: To of functional capacity. In addition, these patients develop recent, and for this reason its effectiveness is discussed by
evaluate quality of life in patients with Familial Amyloid global loss of reflexes and sensitivity, especially vibratory many in the scientific community, making it of little use in
Polyneuropathy; to characterize the sample regarding bio- and positional. Due to its autoimmune characteristic brazilian Hospitals. Objective: To clarify the effectiveness
logical and sociodemographic variables. Methods: This that generates an inflammatory response on the neu- of the Neuromodulation Technique in the human body
is an observational, transversal, analytical and descrip- rons, the treatment is performed with immunoglobulin, through its use and results in brazilian Hospitals. Methods:
tive study, based on primary data from patients with immunosuppression, and glucocorticoids, generating This systematic review used scientific articles containing
confirmed diagnosis of FAP, recruited by convenience stability and even remission. However, as the disease the keywords: “Neuromodulation” and “Transcutaneous
at an ambulatory in Salvador, Bahia, and collected in progresses, the patient may develop respiratory failure, Electrical Nerve Stimulation”. The search was made in
the year of 2021. There were collected sociodemographic a major cause of death in this etiology even with thera- the databases: PubMed, MedLine and ScienceDirect,
and biological variables and six scores generated by the peutic progress. Objective: Describe the epidemiological in the month of May 2022. The review had, as inclusion
Norfolk QOL-DN questionnaire. Quantitative data were profile of mortality for Inflammatory polyneuropathy. criteria, publications in the last 5 years, in Portuguese
analysed and expressed in means/medians, according Methods: This descriptive study was carried out using and English, that directly addressed the theme. Articles
to their distribution, as well as standard deviations/ data from the Departamento de Informática do Sistema with foreign analyses, articles with no direct relation to
interquartile range; and qualitative data were analysed Único de Saúde (DATASUS), in which the Inflammatory the theme, and systematic reviews were excluded. Case
and expressed in relative and absolute values. Results: Polyneuropathy mortality profile between January 2010 presentation and randomized clinical trials were evalu-
In the period between July/2021 and December/2021, and December 2020 was evaluated. The mortality during ated, with their results being analyzed to determine the
there were collected data from 16 patients, with a mean this period was analyzed by education, age group, month effectiveness of the technique. Results: 20 references met
age of 56,19 years (± 15,94 years) and 9 (56,3%) of them of death, federation region, ethnicity, and gender. In the the criteria of this search. Transcutaneous Electrical Nerve
were male. From those, 6 (37,5%) have not declared their description of these categorical variables, their relative Stimulation (TENS) showed potential for improvement
race; 5 (31,3%) were brown; 3 (18,8%) were black and 2 frequencies (%) will be calculated. Since this is a public in cognitive functions and in reducing symptoms such
(12,5%) were white. The autonomic neuropathy score, domain database, submission to the Research Ethics as depression, although research is needed on custom-
large-fiber neuropathy score and total affected quality of Committee was not necessary. Results: The total num- ized TENS protocols. Preliminarily, TENS was shown to
life score had means, respectively, of 4,19 (± 4,23), 30 (± ber of deaths in the 10-year period was 2283 individuals. be safe, even during pregnancy; however, in the case of
28,4) and 64,81 (± 61,32). The sample was divided in two There was a predominance of deaths in the school years treatments such as laryngeal diadochokinesis, the tech-
age groups, by the mean age (56,19), older or youger, and between 8 and 11 years (22.56%), in the months of January nique showed inefficiency. Conclusion: Neuromodulation
it was applied Student’s t-test, which has demonstrated (9.15%) and November (9.15%), in the age group between shows efficiency in pathological treatment and in the pro-
that the previously cited scores has a similar mean – total 60 and 69 years (20.59%), male (60.7%) and white (54%). motion of natural characteristics such as vocal variety. It
affected quality of life score (t(11,2) = 0,651; p = 0,523); Conclusion: There is a congruence of the mortality profile is noted, however, that in cases such as the treatment of
autonomic neuropathy score (t(10,4) = 0,296; p = 0,773); with the epidemiology of the disease. However, even with laryngeal diadochokinesis, the use of the technique has
large-fiber neuropathy score (t(14) = 0,344; p = 0,736). The the treatment already established and the possibility of not shown efficiency, and traditional treatment is prefer-
small-fiber neuropathy score, the symptoms score and remission, the number of deaths from inflammatory poly- able. Neuromodulation is a favorable option for several
the daily-life activities score had medians, respectively, neuropathy is still high. The higher incidence of mortality situations, but not all, being necessary to evaluate what
of 3 (interquartile range: 0;8,75), 10 (interquartile range: in patients with less education and older age suggest the is the expected result and its comparison with the result
4,25;22), 2 (interquartile range: 0;15,25). Conclusion: lack of access to treatment as one of the causes for this of classical treatments. Considering the low amount of
In our sample, all patients had quality of life prejudice. fact. Thus, new studies are needed to solidify the litera- studies in the area, new researches should be done to
Despite of being a chronic and progressive disease, it ture about the motivations for maintaining high levels of prove the efficacy of the neurological technique accord-
was not found any difference at quality of life decrease deaths in an already well known disease. ing to the sought result.
with age progression.

Neuropatias Periféricas Neurorreabilitação


Neuropatias Periféricas

346
XXX Congresso Brasileiro de Neurologia

TL 1104936 TL 1104937 TL 1104938

MULTIPLE SCLEROSIS IN DETAIL: EBOOK FATIGUE, BALANCE AND GAIT OF PEOPLE TRANSCRANIAL MAGNETIC STIMULATION
FOR PATIENTS WITH MULTIPLE SCLEROSIS AFTER FOR DEPRESSIVE SYMPTOMS IN PATIENTS
TREATMENT WITH TRANSCRANIAL WITH MULTIPLE SCLEROSIS
Guilherme Sciascia Olival, Helena Burock, Lucas
Musa, Alice Estevo Dias MAGNETIC STIMULATION ASSOCIATED WITH
Mauricio Ossamu Bando, Juliana Rhein Telles, Alice
PHYSICAL THERAPY: A PILOT STUDY Estevo Dias, Bruna Helena Sciarinni, Demetrios
Associação Brasileira de Esclerose Múltipla – ABEM
Juliana Rhein Telles, Bruna Bruna Sciarinni, Chiuratto Agourakis Agourakis, Giovanna Paula
alice.estevo@abem.org.br Mauricio Ossamu Bando, Demetrios Chiuratto Vidigal, Andre Santos Caetano, Carlos Mello
Agourakis, Giovanna Paula Vidigal, Andre Santos Monteiro, Talita Dias Silva
Background: Multiple Sclerosis (MS) is a chronic neu- Caetano, Carlos Mello Monteiro, Alice Estevo Dias, Associação Brasileira de Esclerose Múltipla – ABEM
roinflammatory and neurodegenerative disease that is a Talita Dias Silva – São Paulo – SC – Brazil
leading cause of neurological disability in young adults.
Associação Brasileira de Esclerose Múltipla – ABEM Universidade Cidade de São Paulo
Educational materials are important for health promotion,
Universidade Cidade de São Paulo Escola de Artes, Ciências e Humanidades da
increasing knowledge about the disease and improving
Escola de Artes, Ciências e Humanidades da Universidade de São Paulo
quality of life. Objective: Analyze and improve the read-
ability and adequacy of educational materials for patients Universidade de São Paulo alice.estevo@abem.org.br
and relatives with Multiple Sclerosis. Methods: Thirteen alice.estevo@abem.org.br
original educational materials in flyers format written by Background: Multiple sclerosis (MS) is a chronic disease
a multidisciplinary team over 37 years were reviewed by a of the central nervous system that affects the brain and
Background: Multiple Sclerosis (MS) is a demyelinating
neurologist. Three MS experts then compared the original spinal cord. Up to 50% do MS patients experience depres-
autoimmune disease that causes CNS damage and dis-
and revised materials using the Suitability Assessment of sive disorders. Transcranial Magnetic Stimulation (TMS)
abling symptoms. Transcranial Magnetic Stimulation
Materials (SAM) instrument, a grading scale used to evalu- has been used for depression. Objectives: To evaluate
(TMS) has benefits in the rehabilitation process when
ate suitability of health information materials for patients. the effect of TMS on depressive symptoms in patients
combined with therapeutic interventions. Objective: To
The SAM criteria were each given 0, 1, or 2 points based with MS. Methods: A quantitative study was performed
evaluate the influence of TMS combined with physiother-
on adequacy of the materials to address each criterion, with 20 people diagnosed with multiple sclerosis (13
apy on the balance, gait and fatigue of people with MS.
with 0 indicating a “not suitable” rating, 1 indicating an relapsing-remitting, 4 primary-progressive and 3 second-
Methods: Participated of this study 20 people with MS,
“adequate” rating, and 2 indicating a “superior” rating. The ary-progressive), aged between 33 and 68 years (Mean =
14 women and 6 men, aged 33 to 68 years (SD 50,0) and
total SAM score for each flyer was calculated as the sum of 50.0, SD = 10.6 years), 6 men (30%) and 14 women (70%),
Kurtze Expanded Disability Status Scale (EDSS) between
earned points divided by the number of possible points. with EDSS score from 0 to 6.5 (Mean = 4.6, SD = 1.9), time
0 and 6,5. The study was done at brazilian Association
The final SAM score was reported as a percentage, with of diagnostic between 1 and 24 years (Mean = 10.0, SD =
of Multiple Sclerosis. The protocol consisted of 10 TMS
70-100 % indicating superior material, 40-69 % adequate 7.2 years). The protocol consisted of 10 interventions by
sessions and 6 physiotherapy sessions with a protocol
material, and 0-39 % unsuitable material. Afterwards, six TMS (primary motor cortex (Cz): 50 pulses per time, 30
for lower limb strength training and static and dynamic
MS patients and two family members were interviewed to trains, 20seconds of interval, totaling 1500 pulses at 90%
balance. Participants were randomized and divided into
discuss the revised materials, the feedback was analyzed of resting motor threshold and left dorsolateral prefrontal
2 groups: Group I) with real stimulus, so that 10 received
qualitatively. Finally, the revised flyers were converted cortex (F3): 10Hz, 50 pulses per train, 40 trains, 20 seconds
the TMS stimulus (primary motor cortex (Cz): 50 pulses
into an eBook. Pearson correlation was calculated to interval, totaling 2000 pulses at 110% of resting threshold)
per time, 30 trains, 20seconds of interval, totaling 1500
determine inter-observer correlation between SAM scores during 10 consecutive working days. For evaluation, an
pulses at 90% of resting motor threshold and left dorso-
assigned by the three evaluators. Paired t test was used interview was conducted to collect data and applied the
lateral prefrontal cortex (F3): 10Hz, 50 pulses per train, 40
for analysis of SAM scores between original and revised BDI (Beck Depressions Inventory) at the beginning and
trains, 20 seconds interval, totaling 2000 pulses at 110%
patient education materials. Results: It was observed at the end of the stimulations. Results: It was observed
of resting threshold), and Group II) sham, that received
that no specialist evaluated the original or revised flyers that 30% of the participants presented depression. It
the application without TMS stimulation (10 patients). All
as unsuitable; however, the revised flyers represent a was found that 65% of participants had improvement in
underwent physiotherapy. The Berg Balance Scale, Timed
significant improvement in total SAM score compared symptoms of depression. However, among those who had
up and Go Test (TUG) and Modified Fatigue Impact Scale
to the original flyers. Specifically, the mean (± standard depression, 83.3% had improved symptoms. Conclusion:
(MFIS) was applied before the combined interventions
deviation) SAM score improved from 60?±?7% (adequate) Depression is a substantial health problem in need of novel
and after 60 days. Results: It was found that 70% of partic-
for the original versions (n?=?13) to 88?±?4% (superior) and effective treatment strategies. TMS is a non-invasive
ipants showed improvement in balance, 20% a decrease
for the revised flyers (n?=?13) (p?<?0.001). Conclusion: and safe treatment. This preliminary study suggests that
and 10% a plateau on Berg Balance Scale. At the Timed up
The eBook was produced based on the needs of target TMS has potential for treating depression in people with
and Go Test, it was found 70% of improvement and 30% of
audience of the educational action, in understandable MS. The promising results encourage the acquisition of
decrease in performance. At the fatigue evaluation with
language and presentation. The material can be considered additional evidence.
Modified Fatigue Impact Scale, in Cognitive domain, it was
as a facilitator or support for conceptual and perceptual
found 90% decrease in fatigue values and 10% increase.
change and enrichment of skills or attitudes.
In the physical domain, it was analyzed that 65% had an
Neurorreabilitação
improvement in the indexes, 25% a worsening and 10%
Neurorreabilitação remained the same. In the psychosocial domain, 70%
showed a decrease in fatigue levels, 20% an increase and
10% remained unchanged. Conclusion: This preliminary
study suggests that TMS associated with physiotherapy
has great potential in the rehabilitation of balance, gait
and fatigue on people with MS. The promising results
encourage the acquisition of additional evidence.

Neurorreabilitação

347
XXX Congresso Brasileiro de Neurologia

TL 1105033 TL 1105036 TL 1105089

ACCESS TO REHABILITATION AFTER STROKE SUPERVISED, HOME-BASED, ELECTROMYOGRAPHIC PROFILE OF THE


IN BRAZIL (AREA STUDY): PRELIMINARY REAL-TIME VIDEOCONFERENCING OROPHARYNGEAL SWALLOWING DYNAMICS
ANALYSIS TELEREHABILITATION PRESERVES IN INDIVIDUALS WITH PARKINSON’S
PERCEPTION OF SOME CLINICAL ASPECTS DISEASE
Roberta Oliveira Cacho, Carla Heloisa Cabral Moro,
Rodrigo Bazan, Suzete Nascimento Farias da IN PEOPLE WITH PARKINSON’S DISEASE: A
Janayna Trench, Patricia Pinho, Renata Scarpel,
Guarda, Elen Beatriz Pinto, Kelson James Almeida, RETROSPECTIVE STUDY Bernardo Rodrigues, Irenio Gomes, Juliana Melo,
Tatiana Souza Ribeiro, Cesar Minelli, Adriana Bastos Ailton Melo, Ana Caline Nobrega
Erica Tardelli, Erika Okamoto, Fernanda Botta
Conforto, AReA study group
Tarallo Rogatto, Kátia Tanaka, Filipe Almeida, Acácio Universidade Federal da Bahia . Salvador BA, Brazil
Universidade Federal do Rio Grande do Norte, Moreira-Neto, Egberto Reis Barbosa, Carla Silva- State University of Bahia. Salvador BA, Brazil
Faculdade de Ciências da Saúde do Trairi – Santa Batista University of Connecticut – Estados Unidos
Cruz – RN – Brazil Bahiana School of Medicine and Public Health.
Associação Brazil Parkinson
Hospital Municipal de São José – Joinville – SC – Salvador BA, Brazil
Universidade de São Paulo. São Paulo SP, Brazil
Brazil
Faculdade de Medicina de Botucatu, Unesp – presidencia@parkinson.org.br asm@ufba.br
Botucatu – SP – Brazil
Universidade Federal da Bahia . Salvador BA, Brazil Background: Clinical worsening in motor symptoms Objective: to determine whether the electromyographic
Universidade Federal do Piauí UFPI and quality of life in people with Parkinson’s disease profile of the suprahyoid muscle group and of the masse-
Universidade Federal do Rio Grande do Norte UFRN (PD) has been observed during the COVID-19 pandemic. ter muscles are modified according to the oropharyngeal
Hospital Carlos Fernando Malzoni, Instituto “Você Real-time videoconferencing telerehabilitation (RTT) has swallowing dynamics in individuals with Parkinson’s
sem AVC” – Matão – SP – Brazil been recommended to mitigate the self-reported clini- disease (PD). Methods: 30 subjects were assessed using
Hospital de Clínicas da Universidade de São Paulo e cal worsening in people with PD in a safe way, as people videofluoroscopic swallow study (VFSS) and surface
Hospital Israelita Albert Einstein are able to access remote rehabilitation services in their electromyogram (EMG) of the masseter and suprahy-
Nada a declarar own homes. However, if long-term RTT can mitigate oid muscle groups during the swallowing of different
self-reported clinical worsening in PD is still unclear. food consistencies, and volumes. Results: Subjects of
ro_fisio1@hotmail.com both sexes, older than 50 years, presented a decrease
Objective: To compare retrospectively the effects of 10
months of supervised, home-based, RTT and usual care in the electric potential (EP) of the evaluated muscles
The objective of this study is to investigate the Access to in all impaired biomechanics of the oral and pharyn-
without exercise training (UC) on self-reported posture,
Rehabilitation After discharge from public Hospitals in geal stages, except for the laryngeal penetration, where
walking, bradykinesia, and quality of life in individuals
Brazil (AReA study), up to six months after stroke. This the EP was increased during the ingestion of the liquid
with PD. Methods: People with PD who performed either
is a cross-sectional multicenter study setting in eighteen sample. Muscle EP was reduced more frequently during
RTT (n=20) or UC (n=20) were retrospectively assessed
public health centers. We presented a preliminary anal- the ingestion of semi-liquid and thickened liquid. There
before pandemic (February to March 2020) and during
ysis of the first 100 participants (convenience sample) was a strong positive correlation, statistically significant,
pandemic (February 2021). People with PD participated
from seven centers collected between January, 2020 and between the duration of contraction of the evaluated mus-
in 60 min, group (6-10 per group) exercise sessions 2-3
January, 2022. Subjects within six months to 1 year post- cles and the duration of the oral transit time; moderate
days per week for 10 months (April 2020 to January 2021).
stroke answered a survey about access to rehabilitation positive correlation, statistically significant, between the
Exercises included: dual tasking, gait training, lunges,
during the first 6 months after discharge. The primary duration of contraction of the suprahyoid muscle group
dance, balance training and functional skill training. Items
outcome is the percentage of patients who had access and the pharyngeal transit time; weak positive correlation
from UPDRS-III (posture, walking, and bradykinesia) and
to any physical therapy session after Hospital discharge with no statistical significance between the duration of
PD Questionnaire (PDQ-39) scores were assessed before
within the first six months after stroke onset. The secondary contraction of the muscle pairs evaluated and the dura-
and during pandemic by blinded evaluators. Effect size
outcomes are referral to physical therapy after Hospital tion of the pharyngeal stage. Conclusion: the EP of the
(ES) and Confidence Interval (CI) were calculated for
discharge; the percentages of patients who had access masseter and suprahyoid muscle groups are modified
within-group (before vs. during) and between-groups
to multidisciplinary team after discharge; the percent- according to the presence of oral stage and pharyngeal
(during) comparisons. Results: There were no between-
age of patients who received health instructions until six stage swallowing disorders of certain consistencies and
group differences in any variable before pandemic. ES
months after stroke; patients´ and families´ preferences volumes, in patients with PD. Furthermore, oral transit
and CI for within-group comparison showed that RTT
about the rehabilitation process. In this preliminary anal- times, duration of the pharyngeal stage, and pharyngeal
improves perception of bradykinesia, preserves perception
ysis, the median age was 63 years-old [54,5: 70] and 50% transition were correlated with the contraction duration
of walking and quality of life and worsens perception of
of the patients were women. They answered the survey of the masseter muscles and suprahyoid muscle group.
posture. In addition, UC worsens perception of all eval-
with a median time of 8 months [7: 10] post-stroke and
uated aspects. ES and CI for between-group comparison
92% had ischemic strokes. The median NIHSS was 2 [1:
showed that RTT is more effective than UC in preserving
4,25] and the Modified Rankin Scale (mRS) 2 [1: 3]. Most Neurorreabilitação
perception of walking, bradykinesia, and quality of life.
participants (57%) reported that they had no access to
Conclusion: Supervised, long-term RTT at home improves
Physical Therapy within the first 6 months post-stroke.
perception in bradykinesia and preserves perception of
In addition, 97% did not have access to physiatrists, 77%
walking and quality of life, although RTT worsens percep-
to speech therapists, 93% to psychologist, 83% to occupa-
tion of posture, which is linked to fall in people with PD.
tional therapist and 80% to nutritionist. Sixty-four subjects
related that they were referred to a rehabilitation center,
and Physical Therapy (37%) was the most cited, with a Neurorreabilitação
wait time of four weeks for the first visit. These preliminary
findings indicate a gap in rehabilitation after stroke but
may have been influenced by the pandemic. The study is
ongoing. *AReA study group: Suellen Mary Marinho dos
Santos Andrade, Lenise Valler, Gustavo José Luvizutto,
Maria Elisa Pimentel Piemonte, Renata Viana Brígido
de Moura Jucá, Octávio Marques Pontes Neto, Beatriz
Neves, Luana Miranda.

Neurorreabilitação

348
XXX Congresso Brasileiro de Neurologia

TL 1105095 TL 1105177 TL 1105245

ELECTROMIOGRAPHIC PROFILE OF COMPARISON BETWEEN EFFECTS PERCEPTION OF PHYSICAL THERAPISTS


MASSETER AND SUPRAHYIOID MUSCLES IN OF REPETITIVE PERIPHERAL NERVE ON THE USE OF SELF-MANAGEMENT
PEOPLE WITH PARKINSON DISEASE STIMULATION ON UPPER LIMB MOTOR PROGRAMS FOR PHYSICAL ACTIVITY IN
PERFORMANCE IN THE SUBACUTE AND POST-STROKE PATIENTS
Janayna Trench, Irenio Gomes, Bernardo Rodrigues,
Juliana Melo, Ailton Melo, Ana Caline Nobrega CHRONIC PHASES AFTER STROKE
Clebeson Azevêdo Nogueira, Nayara Karina Ferreira
Suzana Bleckmann Reis, Victoria Coelho Teixeira, Pereira Tatsch, Adriano Araújo Carvalho, Enio Walker
Federal University of Bahia. Salvador BA, Brazil
Júlia Suzana Zaiden, Débora Santos Minkovicius, Azevedo Cacho, Roberta Oliveira Cacho
University of Connecticut – Estados Unidos
Bahiana School of Medicine and Public Health. Thiago Voigt Gava, Suzete Farias da Guarda
Universidade Federal do Rio Grande do Norte,
Salvador BA, Brazil Nascimento, Isadora Oliveira Santos, Antônio
Faculdade de Ciências da Saúde do Trairi – Santa
Fernando Machado, Gisele Sampaio Silva, Adriana
Cruz – RN – Brazil
asm@ufba.br Bastos Conforto
ro_fisio1@hotmail.com
Objective: to characterize the electromyographic find- Hospital Israelita Albert Einstein
ings of the masseter and suprahyoid muscle group in Hospital das Clínicas – FMUSP
Stroke represents the second worldwide leading cause
maximal voluntary isometric contraction (MVIC) and Hospital São Rafael. Salvador BA, Brazil
of death and the third leading cause of physical disabil-
during swallowing of different food volumes and consis- suzana.reis@ymail.com ity. As a result of the long-term damage generated, the
tencies, in individuals with Parkinson’s Disease (PD) and rehabilitation guidelines for stroke recommend that
healthy ones, who composed the Group Comparison (GC). Background: Repetitive peripheral nerve stimulation these patients be taught to self-manage their sequelae.
Method: thirty individuals with PD and thirty healthy ones (RPNS) is a promising neuromodulation intervention Self-management is a comprehensive concept that can
were evaluated through the Surface Electromyography to improve upper limb motor performance in stroke. be used in the management of behavioral aspects such
(EMG), where the mean values of the electrical potential Stimulation intensities higher than the sensory threshold as physical activity. This study aimed to characterize the
(EP) of the masseter and suprahyoid muscle group were (suprasensory) were associated with benefits compared knowledge of brazilian physical therapists about the appli-
obtained in MVIC and during the swallowing function. to stimulation intensities below the sensory threshold cation of self-management programs to increase physical
Results: it was observed that the EP of the masseter and (subsensory) or no stimulation, in patients in the chronic activity in the routine of post-stroke patients. The sample
suprahyoid muscle group was significantly lower in the phase after stroke. There is limited information about consisted of physical therapists who had already treated
Parkinson’s Disease Group (PDG) in MVIC, and during the effects of different intensities of RPNS, early after stroke patients, who knew the concept of self-manage-
the swallowing of all consistencies and volumes evalu- stroke. Objective: To compare the effects of suprasen- ment and its applicability to increase physical activity
ated. The duration of muscular contraction was higher in sory and subsensory RPNS of the median nerve, on per- in these patients. The answers were collected through
individuals with PD, but with no statistical significance. In formance of the paretic upper limb in the subacute (< 6 an online questionnaire with closed alternatives. Five
addition, muscular EP tends to reduce and the duration of months) and chronic (> 6 months) phases after stroke. questions were answered using the Likert scale (1 to 5).
muscular contraction tends to increase, according to the Methods: This is an ongoing, multicenter, randomized, The score of this scale was used to measure the impor-
Hoehn and Yahr stages. Conclusion: The EP of the mas- parallel-group, proof-of principle clinical trial with blinded tance attributed by respondents to questions related to
seter and suprahyoid muscle group is reduced in MVIC assessment of outcomes. Patients are randomized to the use of self-management. The analysis was carried out
and during the swallowing function in individuals with PD 2-hour suprasensory or subsensory RPNS in the subacute regarding the mean and standard deviation, to determine,
(Groupsuprasubac and Groupsubsubac) or chronic phases respectively, the degree of importance and agreement
(Groupsuprachronic and Groupsubchronic) after stroke. between the professionals’ opinions. Four questions were
Neurorreabilitação analyzed regarding the correlation between the choice of
In all groups, motor training is performed after RPNS. The
primary outcome is the improvement in performance in methods and the frequency of use of self-management.
the Jebsen-Taylor Test (JTT), assessed before and after Of 56 respondents, 29 participants were within the cri-
RPNS, as well as after training. Descriptive analyses of teria to compose the sample. Not knowing the concept
the preliminary results are shown. Statistical tests to of self-management was the reason for the elimination
compare differences within and across groups will only of 19 respondents. These professionals claimed to use
be performed at the end of the study. PRELIMINARY self-management half of the time (44.83%) or almost
Results: Until March 2022, 39 of 72 volunteers were always (51.72%). The main limiting criteria for self-man-
included. At baseline, motor performance was simi- agement were cognitive impairment (96.55%) and physical
lar in Groupsuprasubac (n=7) and Groupsubsubac activity tolerance (100%). About the resources to assess
(n=8), but worse in Groupsubchronic (n=7) than in the patient during the physical activity program, the more
Groupsuprachronic (n=13). Improvement in JTT scores technological means, such as the use of accelerometers,
occurred in 28.5% of the patients in Groupsuprasubac, 75% were less acceptable among professionals (17.24%) and
of the Groupsubsubac,, 61.7% of the Groupsuprachronic, fatigue was the most cited (100%). Thirteen minutes,
and 57.1% of the Groupsubchronic. After training, per- three times a week and twelve weeks were the protocol
formance improved in all patients. Changes in per- indicated for the most participants on how to organize a
formance after stimulation were (in seconds, average self-management program. brazilian physical therapists
± standard deviation; positive values indicate better generally agree with the literature, except for some points
performance): -18.4±35.3, Groupsuprasubac; 8.5±8.5, of divergence about inclusion criteria in the program and
Groupsubsubac; 4.8±11.3, Groupsuprachronic; 5.5±16, the use of technologies for monitoring patients. These
Groupsubchronic. Improvements in performance after results can guide a future self-management protocol for
training were: 3.5±24.5, Groupsuprasubac; 19.7±12.7, physical activity in the post-stroke brazilian population.
Groupsubsubac; 17.9±11.6, Groupsuprachronic; 23.7±16.0,
Groupsubchronic. Conclusion: Although preliminary,
these results suggest that suprasensory RPNS may be Neurorreabilitação
beneficial to patients in the chronic, but not in the sub-
acute phase after stroke. The study is currently recruit-
ing patients.

Neurorreabilitação

349
XXX Congresso Brasileiro de Neurologia

TL 1105267 TL 1105279 TL 1105391

PHARYNGEAL SWALLOWING RESPONSE IN TREATMENT OF UPPER LIMB PARESIS WITH EFFECTS OF THE PERSONAL STROKE
INDIVIDUALS AFTER ISCHEMIC STROKE: A REPETITIVE PERIPHERAL NERVE SENSORY PROGRAM ON FUNCTIONAL INDEPENDENCE
PILOT STUDY STIMULATION AND MOTOR TRAINING: ONE AND RISK FACTOR SELF-MANAGEMENT
MONTH POST-TREATMENT FOLLOW-UP
Jasiel da Silva, Kristel Larisa Back Merida, Mayara Ana Beatriz Cavalcante Carvalho, Adna Karolinne
Silva Marques, Maria Cristina de Alencar Nunes, Adriana Bastos Conforto, Tiago Voigt Gava, Nathália Fonseca, Letícia Silva Costa, Italo Darlan Soares
Viviane Flumignan Zétola Helena Vieira Ribeiro Costa, Emilly Holanda Bezerra, Maria Laizy Ribeiro
Alves, Weybkenedy José Oliveira Santos, Monalisa
Universidade Federal do Paraná. Curitiba PR, Brazil Hospital das Clínicas da Faculdade de Medicina da Silva França, Clebeson Azevêdo Nogueira, Roberta
Hospital Santa Cruz. Curitiba PR, Brazil Universidade de São Paulo Oliveira Cacho
maysmarques@gmail.com adriana.conforto@gmail.com Universidade Federal do Rio Grande do Norte,
Faculdade de Ciências da Saúde do Trairi – Santa
Background: The onset site of the Pharyngeal Swallowing Background: In a randomized, placebo-controlled, Cruz – RN – Brazil
Response (PSR) provides information about the sensory proof-of-principle clinical trial, active or sham repeti-
motor model of swallowing. Absence or longer duration tive peripheral sensory stimulation (RPSS) were delivered ro_fisio1@hotmail.com
of this process is common in the post-stroke population. as add-on interventions to task-specific training (TST)
Objective: To identify the brain injury and the location and functional electrical stimulation (FES) to 38 sub- The Personal Stroke program is based on the social cog-
of the PSR in post-stroke individuals. Methods: Cross- jects with moderate to severe motor upper limb impair- nition theory and aims to induce long term behavioral
sectional, prospective, quantitative study. Twelve patients ment in the chronic phase after stroke, three times per changes, proposing an intervention centered on the indi-
with mean age 68.58 (±10.07), 5 women and 7 men, were week, for six weeks. Increases in grasp and pinch force vidual and on the community. The objective of this study
evaluated. Included subjects ? 60 years old; confirmed were significantly greater in the active than in the sham was to analyze the effects of the Personal Stroke program
ischemic stroke, responsive to perform videofluoros- group. The per-protocol subgroup (25/38; 66%) only on improving the level of functional independence and
copy (VDF). Pathologies that could cause dysphagia were included patients who complied with at least 80% of the risk factor management in post-stroke individuals. This is
excluded. During VDF, swallowing of 10 ml of extremely interventions. Outcomes were assessed in 19/25 (24%) a single-arm, non-randomized, uncontrolled clinical trial
thickened food consistency was observed. The VDF and of the per-protocol subgroup, one month post-treat- with a non-probabilistic sample. Individuals with a clinical
stroke images were analyzed by two speech pathologists ment. Objective: To investigate whether gains in pinch diagnosis of stroke, up to 18 years-old and who had access
and two neurologists. Lesion location was subdivided and grasp strength remained in subjects of the per-pro- to the internet were admitted to the study. Those who
into: tentorial/supratentorial; cortical/subcortical and/or tocol subgroup, followed up to one month after the end had severe cognitive impairment assessed by the Mini-
deep; right and/or left sides. A 4-point scale was used to of treatment. Methods: Ten subjects were in the active Mental State Examination (MMSE), clinically important
identify the location of the PSR: 0 – posterior mandibular and nine in the sham group. Within-group changes in musculoskeletal or neurological conditions and who were
angle; 1 – epiglottic vallecula 2 – hypopharynx 3 – piri- performance (post-treatment versus 1 month later) were bedridden were excluded. Individuals were evaluated at
form recesses. Results: Five patients with PSR in posterior compared with the Wilcoxon test. Between-group differ- baseline (A1) and 6 months after (A2), using the following
mandibular angle, 3 (25.0%) supratentorial lesion; 1 (8.3%) ences in changes in performance were compared with the instruments: Functional Independence Measure (FIM),
supratentorial/infratentorial and 1 (8.3%) infratentorial. Mann-Whitney test. Results: There were no statistically Modified Rankin Scale (mRS) and Stroke Riskometer. Data
Five patients with PSR in epiglottic vallecula, 4 (33.3%) significant differences in characteristics or baseline per- were analyzed using the mean, standard deviation (sd)
supratentorial lesion, 1 (8.3%) supratentorial/infratento- formance between the two groups (Table 1). There were no and frequency. Seven individuals were included in the
rial. Two patients with PSR in piriform recesses, 1 (8.3%) significant within-group differences between performance study, all female, literate, with a mean age of 38.8 years-old
supratentorial/infratentorial and 1 (8.3%) infratentorial. after treatment and one month later (Table 2). There were ( 9.1 sd), a single vascular event, with the most prevalent
PSR and cortical/subcortical lesion: five patients had PSR no significant between-group differences in changes in ischemic etiology (71.4%) and with a mean injury time of
in the posterior mandibular angle, 1 (8.3%) deep lesion; 2 performance after treatment, compared to one month 30.2 months. The Riskometer results show a 1.6% reduc-
(16.7) cortical/deep; 1 (8.3%) cortical/subcortical and 1 later (grasp, p=0.447; pinch, p=0.094). Conclusion: Gains tion in the average 10 years risk of a new stroke after the
(8.3%) cortical. Five patients had PSR in epiglottic vallec- in performance observed after treatment were retained intervention, (A1: 16 (0.64) and A2: 14.4 (4)), while the 5
ula, 1 (8.3%) deep lesion; 1 (8.3%) cortical/deep; 2 (16.7%) at 1 month in the active and sham groups. These results years risk increased by 0.41% (A1: 10.59 (0.3) and A2: 11
cortical/subcortical and 1 (8.3%) cortical. Two patients should be interpreted with caution due to the limited (0.83)). The FIM scores had an increase of 12.2% in the
with PSR in piriform recesses, 1 (8.3%) deep lesion and 1 power of this subgroup analysis. average of the general score (A1: 104.5 (9.7) and A2: 116.7
(8.3%) cortical. PSR and laterality of the lesion: five patients (7)) and in the mRs was observed a reduction of 0.5 (A1:
with LRI in the posterior mandibular angle, 3 (25.0%) 2.6 (0.8) and A2: 2.1 (0.9)). The Personal Stroke program
lesion on the left; 1 (8.3%) right; 1 (8.3%) bilateral. Five Neurorreabilitação was not able to produce an important reduction in the
patients with PSR in epiglottic vallecula, 1(8.3%) lesion self-management of risk factors but could promote bet-
left and 4 (33.3%) right. The PSR in piriform recesses, 2 ter outcomes in the functional independence measures.
(16.7%) both with left lesion. Conclusion: This pilot study
allowed us to identify the site of the PSR and the location
of the encephalic lesion. A larger sample would be needed Neurorreabilitação
to understand the relationship between lesion location
and its impact on swallowing physiology.

Neurorreabilitação

350
XXX Congresso Brasileiro de Neurologia

TL 1105465 TL 1105692 TL 1105763

WHICH ELECTRICAL MUSCLE STIMULATION BIOLOGICAL PERCEPTION OF HUMAN CONVENTIONAL SPEECH THERAPY
INCREASES CYCLICAL PERFORMANCE IN MOVEMENT IN INDIVIDUALS WITH COMBINED WITH FUNCTIONAL ELECTRICAL
HEMIPLEGIC PATIENTS? AN EXPERIMENTAL PARKINSON’S DISEASE: A CROSS- STIMULATION IN PATIENTS WITH
STUDY SECTIONAL STUDY DYSPHAGIA AFTER ACUTE CVA
Wagner Rodrigues Galvão, Larissa Almeida Maia, Denise Soares Araújo, Marcella Cabral de Oliveira, Klayne Cunha Matos, Vanessa Fernandes de
Luana Karoline Castro Silva, Marcos Manoel Sousa Rodrigo Lopes Barreto, Vaneza Mirele Gomes dos Oliveira, Paula Luanna Carvalho de Oliveira, Fabíola
Silva, Renata Viana Brígido de Moura Jucá, Emerson Santos, Roberta de Oliveira Cacho, Pedrina Célia Aureliano Carvalho, Maria Renata Matos de
Fachin Martins, Henrique Resende Martins, George Brasil, Enio Walker Azevedo Cacho Mesquita, Fernanda Martins Maia Carvalho, Mateus
André Pereira Thé, Ramon Távora Viana, Lidiane Alves Teixeira, Clara Maria Limaverde Oliveira, Pedro
Andréa Oliveira Lima UFRN – Santa Cruz – RN – Brazil Braga Neto
UFRN – Natal – RN – Brazil
Master Program in Physiotherapy and Functioning, IFRN Santa Cruz – Santa Cruz – RN – Brazil Secretaria de Saúde do Ceará. Fortaleza CE, Brazil
Federal University of Ceará. Fortaleza CE, Brazil Universidade Federal do Ceará. Fortaleza CE, Brazil
Physiotherapy Department, Federal University of eniowalker@gmail.com Hospital Geral de Fortaleza. Fortaleza CE, Brazil
Ceará. Fortaleza CE, Brazil Centro Universitário Christus – Unichristus.
Department of Electrical Engineering, Federal Parkinson’s disease (PD) is a multifactorial neurological Fortaleza CE, Brazil
University of Ceará. Fortaleza CE, Brazil disorder, with progressive degeneration and impairment
Centre of Research in Assistive Technology, School of voluntary motor control. Action-observation could be mariarenatamatos@hotmail.com
of Physical Therapy, Faculty of Ceilândia, University an effective way to learn specific motor skills, but in PD
of Brasília. Brasília DF, Brazil the execution of movement and biological perception of Background: CVA is the main cause of neurogenic oro-
Graduate Program in Electrical Engineering, Federal human movements could be affected by the difficulty to pharyngeal dysphagia. Electrostimulation has been used
University of Minas Gerais. Belo Horizonte MG, planning motor actions. The aim of this cross-sectional as a therapeutic tool in these cases. However, few studies
Brazil study was to evaluate the biological perception of human prove its efficacy. We evaluated the effect of functional
Department of Teleinformatics Engineering, Federal movement in individuals with PD (PDG, n=5), comparing electrostimulation complementing standard speech ther-
University of Ceará. Fortaleza CE, Brazil it with a control group of healthy individuals (CG, n=5). apy in patients with dysphagia after stroke in a stroke unit.
Edinburgh Handedness Inventory (EHI) and Mini Mental Objectives: To evaluate the effect of short-term functional
lidianelima848@ufc.br State Examination (MMSE) were used to characterized electrostimulation in a CVA unit to complement standard
both groups. PDG and CG were evaluated in a single ses- speech therapy in dysphagic patients after acute CVA and
Background: FES-assisted cycling is an intervention used sion of 30 minutes by a Human Movement Perception Task, to indicate the benefits of different dysphagia techniques
in the rehabilitation of post-stroke hemiplegic individuals. in which the primary outcome measure was the number rehabilitation as standard therapy and electrotherapy.
However, it is necessary to establish electrostimulation of correct answers (NCA) in each movement perception Methods: It is a randomized clinical trial, divided into
parameters for the optimal effect of the intervention. task and the verbal reaction time (VRT). As a secondary an intervention group (IG) (n = 16) and a control group
Objective: To investigate the different conditions of FES- outcome measure, the Hoehn and Yahr Scale (H&Y) and (CG) (n = 17). Data collection occurred from July 2017 to
assisted cycling in the motor and cardiorespiratory per- section 3 of the Unified Parkinson’s Disease Assessment July 2020. The study included patients with ischemic CVA
formance of hemiplegic individuals and to understand Scale (UPDRS) were used only in PDG. Statistical anal- who had oropharyngeal dysphagia and required enteral
the perception, credibility, and expectation of participants ysis was performed using the BioEstat 5.3, using Mann- feeding. Individuals with degenerative neurological dis-
in each condition. Methods: These are preliminary data Whitney test and Spearman correlation. No statistical eases, neoplasia, pacemaker, cochlear implant, febrile
of an ongoing experimental study. The volunteers with difference (p>0.05) was found when comparing the NCA state, and pregnancy were excluded. Patients underwent
stroke were recruited from the local community and the of each task among the groups. A negative correlation (rs: video endoscopy of deglutition (VED), an initial evaluation
measures of cycling were collected under four differ- – 0,91; p=0.0304) was found between the H&Y and NCA of the epidemiological and socioeconomic profile, a dys-
ent experimental conditions applied in a randomized for kick, long jump, sit to stand and riding a bike tasks. phagia risk assessment protocol (DRAP), and a functional
sequence on the same day. Three different conditions (C1, Further studies should be carried out based on the H&Y oral intake scale (FOIS). Informed consent was obtained,
C2, and C3) used electrostimulation in the quadriceps and classification and its impact on the perception of human and all methods were performed under the relevant
hamstrings muscles. The C1 added electrostimulation to movement in this population. This study showed that guidelines and regulations (Declaration of Helsinki). The
the tibialis anterior while the C2 to the gastrocnemius. In there were no impairments in the perception of human study was approved under number 2,388,931 and previ-
these first two conditions, the pedal used enables ankle movement by individuals with Parkinson’s Disease. This ously registered at ClinicalTrials.gov (NCT03649295) and
movement, while C3 blocks ankle movement. A fourth study is ongoing. the brazilian Registry of Clinical Trials (RBR-56QK5J).
control setup (C4) was performed without any electro- Results: There was a significant difference in FOIS scores
stimulation. For each condition, motor performance was at follow-up for both groups. Both groups also tended
measured by the mean rotation per minute (RPM), car- Neurorreabilitação to have improved levels of dysphagia as measured by
diorespiratory performance was assessed by mean heart FEES, although they were not statistically significant.
rate (HR) and at the end, the participant completed the Improvement in feeding occurred in both groups. No
Credibility and Expectation Questionnaire (QCE). The significant differences were detected between the groups
individuals’ perception was analyzed through the mode before and after intervention by DRAP scores. Electrical
of the answers to each item of the QCE. Descriptive and stimulation showed no additional benefits beyond stan-
inferential statistics were performed applying MANOVA. dard therapy when comparing results between groups.
Result: Five individuals (59.4 ± 8.7) years old; 60% male; Conclusion: Based on the present study results there was
50 weeks after an ischemic stroke) were evaluated. The a significant difference in FOIS level improvements of the
prevalence of hemiplegia was in the left limb [60%] and patients in both the intervention group and the placebo
the level of disability measured by the Modified Rankin group, with no significant difference between the groups.
Scale was rated as one individual with severe disability Electrical stimulation protocol use for five days in the
(Rankin 4), 3 with moderate disability (Rankin 3), and one acute phase of ischemic CVA did not generate additional
with mild disability (Rankin 2). Related to the conditions, benefits beyond conventional therapy and the variables
there was no difference between the four setups regard- analyzed improved in both groups.
ing RPM and HR measurements [Pillai’s Trace=0.993; F
(6.32)=0.459; p=0.833]. Nevertheless C1, C2 and C3 were
considered more logical and promising by the partici- Neurorreabilitação
pants and in addition, the C3 was considered the most
comfortable. Conclusion: It was observed that, by the
individual perception, the setup C3 has greater potential
to improve motor and cardiorespiratory performance.
However, these results should be interpreted with caution
until the study is completed.

Neurorreabilitação

351
XXX Congresso Brasileiro de Neurologia

TL 1106008 TL 1106023 TL 1106057

DOES NON-INVASIVE BRAIN STIMULATION EFFECTS OF NON-INVASIVE BRAIN VESTIBULAR REHABILITATION ON


IMPROVE FUNCTIONAL LOCOMOTION IN STIMULATION (NIBS) ON VESTIBULOPATHY POSTURAL BALANCE AND INSTABILITY IN
PEOPLE WITH MULTIPLE SCLEROSIS? A DISORDERS: A SYSTEMATIC REVIEW PATIENTS WITH PARKINSON´S DISEASE: A
SYSTEMATIC REVIEW AND META-ANALYSIS SYSTEMATIC REVIEW
Bruno Henrique de Souza Fonseca, Pedro Henrique
Bruno Henrique de Souza Fonseca, Eduardo de Sousa de Andrade, Otávio Borges, Jessica Mariana Carla Marineli Saraiva do Amaral, Samuel Brito de
Moura Neto, Gustavo José Luvizutto de Aquino Miranda, Rodrigo Bazan, Gustavo José Almeida, Renata Parente de Almeida, Simony Lira do
Luvizutto Nascimento, Pedro Braga-Neto
Universidade Federal do Triângulo Mineiro. Uberaba
MG, Brazil Universidade Federal do Triângulo Mineiro. Pirapora Universidade Federal do Ceará. Fortaleza CE, Brazil
MG, Brazil Universidade de Fortaleza. Fortaleza CE, Brazil
brunohenrifisio@gmail.com Universidade Estadual de São Paulo – Botucatu –
SP – Brazil marinelicarla@gmail.com
Background: Multiple sclerosis (MS) is a neurodegen- Universidade Federal do Triângulo Mineiro. Uberaba
erative disease that is the leading cause of progressive MG, Brazil Background: Instability and postural imbalance impair
functional impairments in working-age people. MS symp- the quality of life of Parkinson’s disease patients (PD).
toms can vary from sensorimotor to cognitive dysfunc- brunohenrifisio@gmail.com Previous studies already described vestibular system
tion, affecting activities of daily living and affecting the connections to these functions, suggesting involvement
ambulation. Treating the walking disabilities associated Background: Vestibular dysfunction (VD) is a dysfunc- of vestibular nuclei, the vestibulo-espinhal lateral sys-
with MS can delay its dysfunction throughout the dis- tion which affects the vestibular system causing dizziness tem, and the participation of basal ganglia in the recep-
ease course. Objective: This review aimed to analyze and tinnitus. The physical therapy treatment for VD is tion of vestibular stimuli. We hypothesize that vestibular
the effects of non-invasive brain stimulation (NIBS) on based on exercises that can suppress the symptoms, such rehabilitation may contribute to the treatment of PD
functional locomotion in people with multiple sclerosis as adaptation, habituation, and substitution exercises. through central neuroplasticity mechanisms such as
(PwMS). Methods: The search strategy was based on the Objective: New types of treatments have emerged, such adaptation, habituation, and substitution. Objective:
PICO process (transcranial direct current stimulation; any as non-invasive brain stimulation (NIBS), to treat chronic Investigate the efficacy of vestibular rehabilitation in
control group: gait, walking, or ambulation). Online data- VD. Considering that NIBS is a promising approach to postural instability and balance in patients with DP, in
bases such as PubMed, CINAHL, Web of Science, Embase, reduce VD symptoms, this review was aimed to ana- comparison to other routine interventions or care, using
PsycINFO, and Lilacs were searched up to January 2022. lyze the effects of NIBS in patients with VD. Methods: randomised clinical trials. Methods: The electronic data
Two review authors independently assessed the risk of We adhered to the methods described in the Cochrane bases used were PubMed/Medline, Embase, Scopus, and
bias in the included studies, and we used the Grading Handbook for Intervention Reviews. The eligibility crite- the Physical Therapy Evidence Database (PEDro). The
of Recommendations Assessment, Development, and ria were as follows: (a) individuals with vestibulopathy system review was registered in PROSPERO with the ID
Evaluation methods to rate the certainty of the evidence having clinical and neuroimaging; (b) Interventions: CRD42020210185, in accordance with PRISMA recom-
for each included study. A meta-analysis was performed non-invasive brain stimulation, this intervention com- mendations. The first stage of data extraction was carried
by pooling the appropriate data using RevMan version prised transcranial direct current stimulation (tDCS) and out independently by two revisors, with any discrepancies
5.3. Results: One study included a qualitative synthe- repetitive transcranial magnetic stimulation (rTMS); (c) being resolved by a third. The studies included had no
sis and three a meta-analysis. In the meta-analysis, an Control: any comparison or sham; and (d) Outcomes: restrictions on publication dates or languages, and the
improvement in the stride length (mean of differences: dizziness and balance. We included randomized con- most recent update occurred in October of 2021. Results:
0.19; 95% CI: 0.07 to 0.31; I² 77%; p = 0.002) was found in trolled trials and non-randomized studies from July 2004 109 of the 440 searches were duplicated, and 303 were
the experimental group. Conclusion: Transcranial direct to February 2020. We searched the PubMed, CINAHL, removed after reading the title and summary, leaving
current stimulation as a NIBS associated with aerobic and Web of Science, Scopus, Cochrane, and Ovid databases. 28 candidates for full text reading. Twenty of these were
resistance exercise can improve stride length in PwMS. Two pairs of reviewers independently screened all titles excluded due to discordance, and four were annotated by
and abstracts. Two authors assessed the risk of bias for a third reviewer due to discrepancies, leaving four studies
each study using the criteria outlined in the Cochrane for systemic review. One study was conducted in Turkey,
Neurorreabilitação Handbook for Systematic Reviews of Interventions and one in Ireland, and two in Italy; the Hoehn and Yahr scale
used the Grading of Recommendations Assessment, ranged from 1 to 4, and the average PD duration ranged
Development and Evaluation (GRADE) Methods to rate from 3.3 to 10.3 years. Interventions occurred in all studies
the certainty of the evidence for each outcome. Results: in the clinical state “on.” The frequency of interventions
We identified 136 studies and included two studies. Both ranged from one to three times per week, for a total of
studies used tDCS application. One study used anodal six years and eight weeks, with each session lasting 40 to
cerebellar tDCS or sham at 2 mA for 25 min associated 60 minutes. The Berg Balance Scale was described in all
with vestibular rehabilitation therapy (VRT), and the the studies, the Activities-Specific Balance Confidence
other study used anodal tDCS over the left dorsolateral scale and the Dynamic Gait Index in two of them, and
prefrontal cortex (F3) for 25–30 min associated with VRT the others had only one description. The majority of the
at home. Both studies showed clinical improvement in the outcomes in the four systemic review studies showed
Dizziness Handicap Inventory (DHI), State-Trait Anxiety statistically significant improvements in postural stability
Inventory (STAI), Activities-Specific Balance Confidence and balance after vestibular rehabilitation. Conclusion:
(ABC), and Self-Rating Depression Scale (SDS) scores. Vestibular rehabilitation is another type of therapy that can
Both studies presented higher-quality evidence on the help patients with DP improve their balance and stability.
GRADE scale and a low risk of bias. Conclusion: Based on
two studies, anodal tDCS over F3 or the cerebellum asso-
ciated with VRT improved chronic vestibular symptoms. Neurorreabilitação

Neurorreabilitação

352
XXX Congresso Brasileiro de Neurologia

TL 1106094 TL 1106367 TL 1106369

ADHERENCE TO MOTOR REHABILITATION SYSTEMATIC REVIEW BETWEEN APPRAXI®: THE ARTIFICIAL INTELLIGENCE-
CAN INFLUENCE THE MOTOR TRAJECTORIES NEUROMODULATION, EXECUTIVE BASED SPEECH TRAINING APP
OF SMA PATIENTS TREATED WITH FUNCTIONS AND OBESITY: IS THERE A
Marta Maria da Silva Lira-Batista, Rogério da Silva
NUSINERSEN RELATIONSHIP? Batista, Valéria Oliveira Costa, Romero Antonio
Graziela Jorge Polido, Rodrigo de Holanda Marta Maria da Silva Lira-Batista, Marcela Lima Ramos de Mendonça, Bruno Mendes de Carvalho
Mendonça, Edmar Zanoteli Silagi, Gleydyson Wesley Freire Lima, Jaynara Keylla Castelo Branco, Dalla Cristiane Sampaio de Sousa
Moreira da Silva, Maria Rita Carvalho Silva, Rogério Paulo, Temistocles Carvalho Zwang, Otilio Paulo da
Faculdade de Medicina da Universidade de São da Silva Batista, Ricardo Galhardoni, Maria Do Silva Neto, Ana Claudia Galvão Xavier, Marcela Lima
Paulo. São Paulo SP, Brazil Carmo de Carvalho E Martins Silagi
grazielapolido@gmail.com HU-UFPI. Teresina PI, Brazil HU-UFPI. Teresina PI, Brazil
Universidade Federal de São Paulo. São Paulo SP, IFPI. Teresina PI, Brazil
Background: Patients with 5q-Spinal Muscular Atrophy Brazil NEUROCENTRO. Teresina PI, Brazil
(SMA) should always undergo treatment with physi- PPGAN-UFPI. Teresina PI, Brazil Universidade Federal de São Paulo. São Paulo SP,
cal therapists in the motor area, but in many cases this FAESPI. Teresina PI, Brazil Brazil
happens only after the Background: of drug treatment, IFPI. Teresina PI, Brazil
but not every increase in the adherence of rehabilitation fgamarthalb@gmail.com
UNICID
can be beneficial. Objective: To evaluate the effects of Universidade Federal do Piauí. Teresina PI, Brazil
changing the frequency and increasing adherence of Background: Verbal apraxia is the inability to program
motor physical therapy before or after the background fgamarthalb@gmail.com verbal movements voluntarily. However, rehabilitation
of nusinersen. Methods: Twenty-eight patients with should aim at active learning. Many applications with
SMA type 1, 2 and 3 were evaluated using the CHOP- Background: The brain is more sensitive to oxidative auxiliary subjects in the health area are being increasingly
INTEND (Children’s Hospital of Philadelphia Infant Test stress than other organs. Oxidative stress and inflamma- explored. However, very few speeches and language reha-
of Neuromuscular Disorders) and HFMSE (Expanded tory processes can negatively affect cognitive functions. bilitation exist, and they are in Other languages (English
Hammersmith Functional Motor Scale for SMA) motor The cumulative effect can potentially impact deteriora- and Russian), whose phonology is entirely different. It
function scales. A questionnaire was carried out to iden- tion, especially of executive functions. Objective: to ver- should be noted that public access to speech rehabilita-
tify the frequency and adherence of motor rehabilitation ify the results: of neuromodulation on the performance tion and the number of professionals are restricted, and
before and after starting treatment with nusinersen, with a of executive functions when applied to overweight or the therapeutic process is lengthy. Objective: Develop
12-month follow-up. Discussion: In this study it is possi- individuals with obesity. Methods: This is a systematic an application to assist in the speech rehabilitation of
ble to see that those patients who started physical therapy review article based on articles selected from PubMed, people with verbal apraxia. Methods: A team of speech
only after drug treatment with an exaggerated increase in ScienceDirect, BIREME, and Web of Science databases. therapists with experience in the rehabilitation of acquired
rehabilitation time did not present motor gains, as well as The search was performed using the following combi- neurological disorders created the stimulus network with
those patients who reduced the time of physical therapy. nation of descriptors: (“problem solving” OR “executive varying degrees of complexity. This network of stimuli was
The greatest gains in the scores on motor scales were pre- function” OR memory) AND (tDCS OR TMS) AND obe- computaionally absorbed through Artificial Intelligence
sented by patients who had previously performed motor sity. Only controlled and/or randomized clinical trials techniques for IOS® and Android® technologies. The pro-
physical therapy for about 3 hours a week, and for those published in any language between 2011 and May 31th, gram was developed for minimally literate individuals
who performed at least 1 hour of motor physical ther- 2022, were analyzed using the PICOS strategy. The study with minimally functioning hearing and vision, as it has
apy per week and increased by up to 2 hours on average. was registered in the International Prospective Register written, acoustic and visual stimuli that must be repeated
Disease modifying treatment will not achieve its best of Systematic Reviews (PROSPERO) under registration and their degree of accuracy of the articulatory precision
result if there is no adequate rehabilitation associated number CRD42.021.261.770. Results: Using the descrip- captured. The program has four interaction modules: sus-
with medication. Conclusion: It is important to evaluate tors mentioned, 763 articles were identified in the data- tained vowels, phonemes, sequences, and phrases. Each
the frequency and adherence of baseline rehabilitation bases defined for the search. After selecting and analyzing one will have three levels of complexity. In each module,
before starting drug treatment. Therefore, it is possible the works found, 08 articles were selected. The number there will also be subgroups of skills worked on. Among the
to plan a change in physical therapies frequency after of people included in the eight selected studies was 301 various modules, the ‘report’ stands out, which informs
treatment, thus avoiding fatigue and risk of motor loss. participants. The majority were female (235 participants), the professional quantitatively and qualitatively about the
with an average of 30.1 participants per study. The global speaker’s errors. The research was approved by the CEP/
nutritional status varied between low weight and grade 3 IFPI/CONEP under the opinion number 5,111,083 and is
Neurorreabilitação obesity, by the selected studies of 28.1 kg/m2. Regarding currently in the implementation stage. The registration
the type of stimulation modality, the proportion between filing with the National Institute of Industrial Property is
non-invasive neuromodulation strategies as a form of still on request. Results: During the 13 months after the
treatment was 75% (n=6) with tDCS and 25% (n=2) with research was installed, the conceptual design was awarded
EMT. Regarding the relationship between age and per- two research funding notices (CATALISA-ICT/Sebrae® and
formance of executive functions, a study with older par- IFES). For validation of the prototype, 127 adults native to
ticipants demonstrated the go/no-go task and the reduc- the Portuguese language participated, distributed in the
tion of food desire. Differently, in a survey with young states of São Paulo, Rio de Janeiro, Maranhão, Ceará, and
patients, an increase in the consumption of calories and Piauí. In this second stage, people with apraxia are invited
a worse performance in the flanker paradigm task were to participate. Conclusion: It is expected that daily speech
demonstrated. It should be noted that both used the training will reduce the number of sessions for monitoring
same target (anodic at F3) but in different modalities: the Speech-Language Pathologist and increase the turn-
electrical and magnetic, respectively. Furthermore, it over of patients who will be seen, allowing better access
improved the performance of the executive functions of to health at the tertiary level (rehabilitation).
the participants, especially in the groups of subjects with
overweight or obese. Conclusion: The use of non-invasive
Neurorreabilitação
neuromodulation has consistently reduced food cravings,
especially for sweet foods, and fast food, and at the same
time improved executive functions.

Neurorreabilitação

353
XXX Congresso Brasileiro de Neurologia

TL 1106435 TL 1106440 TL 1106490

EFFECT OF ROBOT-ASSISTED TRAINING PHYSICAL ACTIVITY AT HOME IS ABLE TO PHYSICAL ACTIVITY INTERRUPTION DURING
ON UNILATERAL SPATIAL NEGLECT AFTER REDUCE THE WORSENING IN QUALITY OF COVID-19 PANDEMIC AGGRAVATES THE
STROKE: SYSTEMATIC REVIEW AND META- LIFE IN PARKINSON’S DISEASE DURING WORSENING IN QUALITY OF LIFE OF PEOPLE
ANALYSIS OF RANDOMIZED CONTROLLED COVID-19 PANDEMIC WITH PARKINSON’S DISEASE
TRIALS
Isaíra A P S Nascimento, Carla Silva-Batista, Daniel Isaíra A P S Nascimento, Carla Silva-Batista,
Rodrigo Bazan, Bruno Henrique de Souza Fonseca, Boari Coelho, Renato C F Júnior, K R Nobrega Daniel B Coelho, Renato C F Júnior, Kátia Richelma
Jessica Mariana de Aquino Miranda, Helio Rubens Barbosa, An Raquel Lorena Lindquist Almeida, Hugo Nóbrega Barbosa, Ana Raquel Lorena Lindquist
de Carvalho Nunes, Silméia Garcia Zanati Bazan, Sanchez, Clynton L Correa, Maria Elisa P Piemonte, Almeida, Hugo Sanchez, Clynton L Correa, Adriana
Gustavo José Luvizutto Adriana Vera Guimarães Israel Vera Guimarães Israel, Maria Elisa P Piemonte

UNESP – Botucatu – SP – Brazil Universidade de São Paulo. São Paulo SP, Brazil Universidade de São Paulo. São Paulo SP, Brazil
UFTM. Uberaba MG, Brazil Universidade Federal do ABC – SÃO BERNARDO do Universidade do ABC – São Bernardo do Campo –
CAMPO – SP – Brazil SP – Brazil
brunohenrifisio@gmail.com Universidade Federal do Amazonas. Manaus AM, Universidade Federal do Amazonas. Manaus AM,
Brazil Brazil
Background: Several studies have shown that robotic Universidade Federal – Natal Salvador – RN – Brazil Universidade Federal – Santarém Macapá – PA –
devices can effectively improve motor function in stroke Universidade Federal de Jataí. Jataí GO, Brazil Brazil
patients through limb activation. However, the effects of Universidade Federal do Rio de Janeiro. Rio de Universidade Federal – Natal Salvador – RN – Brazil
robot-assisted therapy on perceptual deficits after stroke Janeiro RJ, Brazil Universidade Federal do Jataí. Jataí GO, Brazil
is unclear. Objective: This review aimed to evaluate the Universidade Federal – Florianópolis SC, Brazil Univesidade Federal do Rio de Janeiro. Rio de
effectiveness of robotic limb activation in patients with Janeiro RJ, Brazil
unilateral spatial neglect (USN) after stroke. Methods: isaira@usp.br Universidade Federal – Florianópolis SC, Brazil
A literature search was performed using MEDLINE,
EMBASE, CENTRAL, CINAHL, and LILACS databases Background: The physical distancing, due to COVID isaira@usp.br
without language restrictions. Randomized controlled 19 pandemic, causes an abrupt interruption in physical
trials (RCTs) and quasi-RCTs of robot-assisted therapy activity. The health services in all the world had to face Background: Reduced physical activity can collaborate to
for USN after stroke were selected. Two reviewers inde- the challenge to offer remote health to promote physical accentuate the motor and non-motor changes experienced
pendently assessed the risk of bias and certainty of the activity at home to avoid the general worsening of dis- in daily life by people with PD. Objective: To investigate
evidence of the included studies. Results: A total of 630 ease impairments. However, the efficacy of remote care the impact of interruption in physical activities due to
studies were identified, including five studies for qual- is little known yet. Objective: To compare the effects of physical distancing during the COVID-19 pandemic on
itative synthesis and four meta-analyses. The results of physical activity (PA) at home with and without remote the motor and non-motor aspects of daily life experi-
RCTs comparing robotic limb activation with a control supervision on the motor and non-motor aspects of ences of people living with Parkinson’s disease in Brazil.
group suggested an improvement in the degree of USN daily life experiences of people living with Parkinson’s Methods: 400 individuals with confirmed PD diagnosis
measured by the line bisection test (standardized mean disease during the physical distancing due to COVID-19 mean age of 67.2 years (SD=9.5), in stage 1 – 3 of disease
difference [SMD], -0.64; 95% confidence interval [CI], -1.13 pandemic. Methods: 478 individuals with PD mean age evolution according to Hoehn and Yahr classification,
to -0.15; p = 0.01). There were no differences between the of 67.6 years (SD=9.5), in stage 1 – 3 of disease evolution living in 14 different cities representative of 5 different
groups in the motor-free visual perception test 3rd edition according to Hoehn and Yahr classification, living in 14 geo-economic regions, participated in the present study.
(SMD, 0.27; 95% CI, -0.25-0.79; p = 0.31), star cancellation different cities representative of 5 different geoeconomic After presenting the informed consent form, and express-
test (SMD, 0.26; 95% CI, -0.42-0.94; p = 0.54), Albert’s test regions, participated in this study. Participants were asked ing agreement to participate in the study, participants
(SMD, -0.67; 95% CI, -2.01-0.66; p = 0.32), and Catherine to answer, through telephone interviews, a questionnaire were asked to answer, through telephone interviews, a
Bergego Scale (SMD, -0.81; 95% CI, -2.07-0.45; p = 0.21). about: (1) general information; (2) socio-economic status; previously established questionnaire that investigates:
Conclusion: The study demonstrated that limb activation (3) information associated with PD; (4) quality of life; (5) (1) general information; (2) socio-economic status; (3)
through robotic therapy can improve midline perception. health conditions associated with COVID-19; (6) emo- information associated with PD; (4) quality of life; (5)
However, there was no impact on tasks assessing visual tional health condition; (7) level of physical activity; (8) health conditions related to COVID-19; (6) emotional
scanning, functionality, or activities of daily living. remote health care; (9) cognitive health conditions and health condition; (7) fear of falling; (8) level of physical
(10) motor and non-motor aspects of daily life experience. activity; (9) cognitive health conditions and (10) motor
Participants were divided into three groups, according to and non-motor aspects of daily life experience. The par-
Neurorreabilitação their self-reported physical activity at home: no PA group ticipants were divided into two groups, according to their
(NPA) (150 participants), PA with no-remote supervision self-reported physical activity profile: no-interruption in
group (PANS) (225 participants) and PA with remote PA group (NIPA) with 236 participants, and interruption
supervision group (PAS) (103 participants). Comparison in PA group (IPA), with 164 participants. The compar-
in motor and non-motor self-reported worsening between ison in motor and non-motor self-reported worsening
two groups was analyzed by ANOVA one-way. Results: between two groups was analyzed by ANOVA one-way.
ANOVA showed a significant statistical difference between Results: The groups were comparable in clinical aspects.
the groups for quality of life (F2, 475=3.57, p=.02, ES=0.78), The ANOVA showed a significant statistical difference
but not for motor and non-motor aspects of daily life expe- between the groups for the quality of life and motor aspects
rience. The Tukey post-hoc confirmed that NPA reported of daily life experience (p<.05), but not for motor aspects
more severe worsening in quality of life than PANS and of daily life experience. The Tukey post-hoc confirmed
PAS (p<.05). Conclusion: The PA at home, regardless of that NIPA reported less severe worsening in the quality
remote supervision, reduced the self-reported worsening of life and motor aspects of daily life experience than IPA.
quality of life in people living with PD during the physical Conclusion: The interruption in PA due to physical dis-
distancing. However, it is not able to alleviate the nega- tancing during the COVID19 pandemic promoted more
tive impact of interruption in health care on motor and severe worsening and motor aspects and quality of life
non-motor alterations associated with PD. in people living with PD in Brazil. Future studies should
investigate feasible and effective strategies to guarantee
PA maintenance at home, probably by telehealth.
Neurorreabilitação

Neurorreabilitação

354
XXX Congresso Brasileiro de Neurologia

TL 1106503 TL 1106517 TL 1104824

PHYSICAL ACTIVITY REDUCES THE INFLUENCE OF NON-INVASIVE CLINICAL-EPIDEMIOLOGICAL PROFILE OF


WORSENING IN MENTAL-HEALTH AND NEUROMODULATION ON FOOD INHIBITION, PATIENTS TREATED FOR SLEEP DISORDERS
QUALITY-OF-LIFE IN PEOPLE LIVING WITH EXECUTIVE FUNCTIONS AND BIOCHEMICAL IN A SPECIALIZED OUTPATIENT CLINIC OF
PARKINSON’S DISEASE DURING THE PARAMETERS OF INFLAMMATION AND THE PUBLIC HEALTH SYSTEM
COVID-19 PANDEMIC OXIDATIVE STRESS
Emerson Oliveira Lise, Rodrigo Bindá de Magalhães
Isaíra A P S Nascimento, Carla Silva-Batista, Marta Maria da Silva Lira-Batista, Marcela Lima Loiola, João Bosco Lopes Botelho, Juliana Costa dos
Daniel B Coelho, Renato C F Júnior, Kátia Richelma Silagi, Ricardo Galhardoni, Rogério da Silva Batista, Santos, Moisés de Oliveira Medeiros, Luan Felipe
Nóbrega Barbosa, Ana Raquel Lorena Lindquist Jaynara Keylla Moreira da Silva, Maria Rita Carvalho de Souza Cardoso, Maria Paula batalha Costa, Ana
Almeida, Hugo Sanchez, Clynton L Correa, Adriana Silva, Maria Do Carmo de Carvalho E Martins Carla Campelo Duarte, Carlos Maurício de Almeida,
Vera Guimarães Israel, Maria Elisa P Piemonte Diego Monteiro de Carvalho
HU-UFPI. Teresina PI, Brazil
Universidade de São Paulo. São Paulo SP, Brazil Universidade Federal de São Paulo. São Paulo SP, Fametro. Manaus AM, Brazil
Universidade Federal do ABC – São Bernardo do Brazil Fundação Hospital Adriano Jorge/FAPEAM. Manaus
Campo – SP – Brazil UNICID AM, Brazil
Universidade Federal do Amazonas. Manaus AM, IFPI. Teresina PI, Brazil Universidade do Estado do Amazonas. Manaus AM,
Brazil PPGAN. Teresina PI, Brazil Brazil
Universidade Federal – Santarém Macapá – PA – FAESPI. Teresina PI, Brazil
emersonlisee1@gmail.com
Brazil Universidade Federal do Piauí. Teresina PI, Brazil
Universidade Federal – Natal Salvador – RN – Brazil
fgamarthalb@gmail.com Sleep is a special physiological state that occurs cyclically,
Universidade Federal do Jataí. Jataí GO, Brazil
and its disorders affect quality of life and lead to metabolic
Universidade Federal do Rio de Janeiro. Rio de
Background: The various pathologies that occur with and cardiovascular problems. The third edition of the inter-
Janeiro RJ, Brazil
the metabolic disorder promote chronic inflammation, national classification of sleep disorders (ICSD-3) classi-
isaira@usp.br leading to impairment of the integrity of the blood-brain fies them as insomnia, sleep-related breathing disorders,
barrier, which may affect cognitive functions. Objective: To central disorders of hypersomnolence, circadian rhythm
Background: Anxiety and depression are common correlate markers of antioxidant activity, inflammation and of sleep-wake disorders, parasomnias and sleep-related
non-motor alterations in PD. Increased levels of stress executive functions with tDCS in healthcare professionals. movement disorders. This study aimed to discover the
during the COVID-19 pandemic may have several short- Methods: randomized, parallel, double-blind clinical trial. clinical and epidemiological profile of patients treated
term as well as long-term adverse consequences for this The target population consisted of health professionals in the specialized sleep disorders outpatient clinic of the
population. Reduced physical activity during the pan- from the clinical staff of a university Hospital. The following public otolaryngology and cervical-facial surgery service in
demic may contribute to increased psychological stress, instruments were applied: Intense Food Cravings – Trait the city of Manaus, AM, Brazil, between January 2019 and
thereby further aggravates the worsening in quality of life. and State questionnaires, brazilian Craving-Related Food May 2021. The data of the analyzed sample were obtained
Objective: To investigate the impact of interruption in Inventory, Three-Factor Eating Questionnaire, digit span from 72 medical records, in which it was observed that
physical activities due to physical distancing during the test; Stroop Test of Colors and Words, Victoria version; sleep disorders had a higher prevalence in the female
COVID-19 pandemic on the mental health condition and Trial Making Test). Determinations of thiobarbituric acid sex, and predominated in the age group of between 51-60
quality of life in people living with Parkinson’s disease. reactive species (TBARS) and lipid peroxidation biomark- years. Regarding life habits, both sexes presented a link
Methods: 463 individuals with confirmed PD diagnosis ers were also performed. Results: A statistically significant with alcohol consumption (32.26%), tobacco use (11.1%)
mean age of 67.2 years (SD=9.5), in stage 1 – 3 of disease reduction was found in QDIC-T values ??(p<0.0074**); and were overweight (73.69%). Regarding the use of med-
evolution according to Hoehn and Yahr classification, FCI-Br (p<0.0005**); Digit span (forward order: p<0.007**; ication, 44.68% of patients reported using some type of
living in 14 different cities representative of 5 different reverse order: p<0.01**); TSCP (conditions 1: p<0.003**; hypnotic drug. In the item comorbidities, systemic arterial
geo-economic regions, participated in the present study. 2: p<0.006** and 3: p<0.008**); Trail Making Test (A: 0.05* hypertension (SAH) was noted in 44.62% of the subjects
After presenting the informed consent form, and express- and B: 0.007**) in the tDCS group when compared to the analyzed. The most prevalent diagnosis was obstructive
ing agreement to participate in the study, participants sham group. In this research, we observed that the appli- sleep apnea syndrome (45.5%), which is consistent with
were asked to answer, through telephone interviews, a cation of tDCS triggered statistically significant changes in: the finding that 35.59% of the individuals had class IV
previously established questionnaire that investigates: women with a mean age predominantly between 31 and Mallampati scoring. Statistically, none of these variables
(1) general information; (2) socio-economic status; (3) 40 years; middle-class income; high level of education; were shown to interfere with sleep quality when evaluated
information associated with PD; (4) quality of life wors- overweight and without previous infection by Covid-19; by means of the chi-square test. Finally, it was concluded
ening; (5) health conditions related to COVID-19; (6) regarding: traits of food cravings, especially for sweets, that, in this specialized service, most of the consultations
mental health worsening; (7) fear of falling; (8) level of fatty foods and traditional meal, executive functions. involve overweight women and that obstructive sleep
physical activity; (9) cognitive health conditions and (10) Conclusion: The use of tDCS influences the reduction of apnea is the most prevalent disorder.
motor and non-motor aspects of daily life experience. The food cravings and the reduction of reaction time and the
participants were divided into two groups, according to processing of executive functions, although there are no
their self-reported physical activity profile: PA group (PAG) significant changes in brain bioenergetics to the point of Neurossonologia
with 327 participants, and no PA group (NPAG), with 136 impacting the biochemical parameters of inflammation.
participants. The comparison of the mental health wors-
ening and quality of life worsening between two groups
was analyzed by ANOVA one-way.Results: The groups Neurorreabilitação
were comparable in clinical aspects. The ANOVA showed a
significant statistical difference between the groups for the
mental health worsening; (F1, 461=5.95, p=.015, ES=.78),
and quality of life worsening (F1, 461=8.1, p=.004, ES=.81).
The Tukey post-hoc confirmed a more severe worsening in
the mental health condition and quality of life for NPAG
than PAG. Besides, the worsening in mental health and
in quality of life was directly correlated (R=.49; p<.0001).
Conclusion: The interruption in PA due to physical dis-
tancing during the COVID19 pandemic promoted more
severe worsening in mental health conditions and quality
of life in people living with PD.

Neurorreabilitação

355
XXX Congresso Brasileiro de Neurologia

TL 1105921 TL 1104955 TL 1105497

ASSESSMENT OF CONCORDANCE EVALUATION OF SLEEP QUALITY IN INSOMNIA: AN ANALYSIS OF ITS


BETWEEN ULTRA-PORTABLE ULTRASOUND HOSPITALIZED PATIENTS IN A HOSPITAL IN PATHOPHYSIOLOGY, CLINICAL TREATMENTS
EQUIPMENT AND A TRADITIONAL ONE FOR SOUTHERN SANTA CATARINA AND RELATED COMORBIDITIES
THE DIAGNOSIS OF PARKINSON’S DISEASE
Paulo Henrique Fabiano Zamora, Gregorio das Italo Felipe Cury, Anderson Moura Bernardes,
BY TRANSCRANIAL SONOGRAPHY Neves Meurer, Bianca Ferreira, Laura Goedel Anderssany Moura Bernardes
Maria Alice da Silva Paes, Denise Hack Nicaretta, Santos, Lucas Michelon Tietböhl, Aline Vieira
Scarlatelli Lima UNIRG- Universidade de GURUPI – GURUPI-
Regina Maria Papais Alvarenga, Ana Lucia de Zuma TOCANTINS – TO – Brazil
Rosso, Rodrigo Tavares Brisson, Rita de Cassia Leite Universidade do Sul de Santa Catarina – tubarão – Universidade Federal do Piaui. Teresina PI, Brazil
Fernandes SC – Brazil Universidade de Gurupi. Gurupi TO, Brazil
Universidade Federal do Estado do Rio de Janeiro. Universidade do Sul de Santa Catarina – Tubarão –
SC – Brazil italofelipe.cury@gmail.com
Rio de Janeiro RJ, Brazil
Universidade Federal do Rio de Janeiro. Rio de paulinho.zamora@hotmail.com Background: Insomnia is characterized by difficulty
Janeiro RJ, Brazil in the quality of sleep, whether to initiate or maintain
maspmed@gmail.com Background: Despite the importance of sleep for sleep, which leads to substantial suffering and daytime
health and recovering of Hospitalized patients, the impairment, being more common in elderly people. The
Background: The diagnosis of Parkinson’s disease (PD) is Hospitalization period requires changes in routines that pathophysiology of insomnia is associated with sleep-
clinical. Transcranial sonography (TCS) is used to assist can be harmful to the sleep quality of patients. Objective: wake regulation through wakefulness promoters such
in the diagnosis of PD, revealing an increase in echoge- This study aims to compare intra and out-of-Hospital sleep as orexin, norepinephrine, and histamine and sleep-pro-
nicity of the substantia nigra (SN) with an accuracy of quality, indicating the associated factors with worse sleep ducing chemicals such as GABA, adenosine, melatonin,
80-90%. With the advance in technology, ultrasound (US) quality in Hospitalized patients and their possible clinical and prostaglandin. Insomnia, therefore, results from
equipment is becoming ever more portable, allowing the repercussions. Methods: this is a cross-sectional study increased orexin-mediated neuronal firing and inhibi-
use of this method even in places with difficult access to conducted with 252 patients from a tertiary Hospital. In tion of sleep-promoting areas. In this way, the diagnosis
traditional systems. There are still no studies to determine addition to searching medical records and conducting and treatment of insomnia depends on a clinical history
the accuracy of TCS performed on ultra-portable (UP) interviews to identify clinical characteristics and factors that analyzes precipitating factors, behavioral issues and
equipment within the field of PD. Objective: To verify that interfere with sleep quality, the Pittsburgh Sleep environmental factors that can lead to poor sleep quality.
the accuracy of the Lumify (Philips Medical Systems) Quality Index (PSQI) was used to assess sleep quality Objective: To highlight the pathophysiology, associated
UP equipment by differentiating data from healthy con- in home and Hospital environment. Results: There was comorbidities and therapeutic strategies for treating
trol subjects and from patients with PD and assessing a worsening in the components of duration, efficiency insomnia. Methods: This is a systematic literature review
the concordance between measures obtained from this and subjective quality of sleep during Hospitalization, in based on data extraction in indexes such as PUBMED and
equipment and traditional equipment. Methods: In this addition to greater use of sleep-inducing medications and MEDLINE. Publications between 2018 and 2022, in English
cross-sectional study, TCS was performed on 129 sub- more complaints of daytime dysfunction when compared and with the following descriptors: Insomnia, Neurology,
jects, between September 2019 and July 2021, separated to the same components of the PSQI before Hospitalization Treatment, were considered. Results: Insomnia disorder
into two groups, one with healthy controls and the other (p<0.05). There was also an average reduction of 57 min- goes beyond an individual complaint of poor sleep or
with PD in the Outpatient Clinics for PD at the Hospital utes in total sleep time during Hospitalization (7h52min vs. misperception of sleep status, and there is evidence that
Universitário Gaffrée e Guinle da Universidade Federal do 6h55min; p<0.05). The main disturbing factors reported by insomnia in adolescents can be associated with changes
Estado do Rio de Janeiro / Empresa Brasileira de Serviços patients were associated with the Hospital environment, in several biosystems, including functional cortical alter-
Hospitalares or in the Clinic for Movement Disorders at such as interventions by Hospital staff and noise. Worse in ations and systemic inflammation. Insomnia disorder
the Hospital Universitário Clementino Fraga Filho da inpatients sleep quality was also associated with presence may also be associated with depression, hypertension,
Universidade Federal do Rio de Janeiro. The tests were of hypertension, diabetes and difficulty sleeping before type 2 diabetes, gastroesophageal reflux disease, and
performed by insonation of the temporal bone window admission (p<0,05). There was no association of sleep asthma. The treatment of insomnia symptoms can be
(TW) of healthy individuals and patients with PD. The quantity and quality with blood pressure and blood glu- done through cognitive behavioral therapies and through
parameters obtained in accordance with protocols estab- cose levels. Conclusion: Sleep in Hospital environment this already achieving very positive results, at the same
lished were: assessment of the quality of the TW and the was considerably worse than home environment and the time they can be done through drug therapy that acts on
size of the third ventricle. In addition to the measurement sleep disturbing factors were predominantly associated GABA-A receptors exerting sedative, anxiolytic, muscle
of the mesencephalon area and the SN hyperechogenicity, with the Hospital environment. relaxant and hypnotic effects. Conclusion: In this way,
which, in the UP equipment, were performed by auto- it is important that physicians recognize and administer
matic measurement of an ellipse and, in the traditional the treatment of insomnia to enable the reduction of mor-
Sono bidity related to it, which can guarantee the return of the
one, by manual delineation of its borders. Results: The
accuracy of the UP equipment for the diagnosis of PD quality of life that is lost, reduce daytime fatigue and the
was 93%, with a sensitivity of 98.3% and specificity of predictive risks for other pathologies.
86.7%, with a cut-off point of the SN area >0.20 cm². The
highest concordance was the assessment of the quality
Sono
of the TW (Kappa index of 100% for the right TW and 83%
for the left). The SN measurements presented with higher
indices of concordance: ICC= 90% for the right SN and
93% for the left. The highest concordance between the
systems was the measurement of the third ventricle (ICC
96%). Conclusion: The UP ultrasound is an accurate tool
for PD diagnosis, and could be a valuable, practical, fast,
and great potential bedside method.

Neurossonologia

356
XXX Congresso Brasileiro de Neurologia

TL 1105587 TL 1105589 TL 1105613

SLEEP-WAKE CYCLE PATTERN IN BLIND IMPACT OF THE COVID-19 PANDEMIC WOULD HEMIFACIAL SPASM BE A
PEOPLE LIVING IN LOW LATITUDE AREAS ON NONRESTORATIVE SLEEP PATTERN MOTIVATING FACTOR FOR POOR SLEEP
(NATAL-RN-BRAZIL) IN COLLEGE STUDENTS AT A PRIVATE QUALITY?
UNIVERSITY IN RECIFE
Antonio Handerson Lima Aguiar, Karen Pugliane, Paloma Peter Travassos Zaidan, Paulo Barbosa
Natalia Dipiere, John Fontenele Araújo Julia Renata Pinto Correia Melo, Maria Alice Leite Neto, Victor Fellipe Bispo Macedo, Larissa
Mendonça Gomes Cavalcanti, Renata Silva Sá, Clementino Leite Sa Carvalho, Marco Andre Moraes
Universidade Federal do Rio Grande do Norte. Natal Bernardino, Djanino Fernandes Silva, Fabiola Lys
Djanino Fernandes Silva, Larissa Clementino Leite
RN, Brazil Medeiros, Clelia Maria Ribeiro Franco
Sa Carvalho, Marco Andre Moraes Bernardino, Victor
handerson.aguiar.076@ufrn.edu.br Fellipe Bispo Macedo, Paulo Barbosa Leite Neto,
Hospital Universitário Oswaldo Cruz – UPE. Recife
Fabiola Lys Medieros
PE, Brazil
Background: It is present in the current literature that
Centro Universitário Maurício de Nassau
blind individuals have sleep disorders related to circa- paulo.lei.te@hotmail.com
UNINASSAU
dian rhythmicity. These studies have been guiding, on a
Hospital Universitário Oswaldo Cruz – UPE. Recife
global, scale therapeutic approaches aimed at the blind. Background: Hemifacial spasm (HFS) is a movement dis-
PE, Brazil
However, such studies were carried out in high latitude order characterized by clonic spasms, rarely tonic, involv-
regions, so it is necessary to evaluate the pattern of the paulo.lei.te@hotmail.com ing the muscles of facial mimicry, especially the orbicularis
sleep-wake cycle in blind people in low latitude areas, with of the eye and mouth of one of the hemifaces, with episodes
little variation in the photoperiod. Objective: Characterize Background: Because of the peak incidence of the new of blinking, eye closure and involuntary contractions of the
sleep and rhythm of rest activity in blind people living in coronavirus, the university routine was completely mod- mouth. HFS usually causes great aesthetic and functional
a low latitude region (Natal/RN). Methods: Participated ified. Absence of group conviviality can result in psycho- discomfort in the affected muscles, compromising qual-
in the study 19 total blindness people, living in the city of social implications, impairing restful sleep and reflecting ity of life and mood. Sleep is a biological function in the
Natal. The parameters of the sleep-wake cycle were char- on poor academic development. Therefore, if sleep is maintenance of quality of life, and the existence of sleep
acterized by the actimetry technique (Actrust – Condor not restorative, the difficulty maintenance of deep sleep complaints in patients with HFS and possible correlations
Instruments) that was used for 20 days. The analyses were and muscle relaxation is associated with a set of adverse between these disorders motivated this study. Objectives:
done in the ActStudio software and were quantified sleep symptoms, such as physical, mental and emotional dis- To analyze the clinical-epidemiological characteristics of
parameters and non-parametric variables of the rhythm turbances, among these, mood swings, memory consoli- individuals with HFS; to assess sleep quality, insomnia
of activity and rest (IV = intraday variability; IS = inter- dation and learning. Objectives: To report the incidence symptoms and/or mood changes in these patients; to
day stability; M10 = 10 hours of higher activity; L5 = five of the impact of non-restorative sleep. To investigate relate possible associations between the variables and
hours of lower activity and RA = relative amplitude). 3 comorbidities effects on sleep and learning consequences. sleep impairment. Methods: Observational cross-sectional
participants were excluded due misuse of the actimeter. Methods: Cross-sectional study, observational, descrip- study that analyzed demographic variables, mood, sleep
Project was approved by CEP – UFRN. Results: The blind tive, retrospective, epidemiological quantitative. It was quality, presence and severity of insomnia symptoms in
volunteers in this study had a sleep duration of 377.7min carried out by interviewing 204 students from a private patients with HFS, followed up at the university center in
(6.28 hours) ± 42 min, a sleep latency of 6 ± 11 min and university in Recife, through a semi-structured ques- northeastern Brazil, using questionnaires. The validated
an average sleep efficiency of 84.8% ± 6.1%. We found tionnaire placed on the Google Forms platform. Results: instruments were applied: Pittsburgh Sleep Quality Index
that 68% of volunteers have a sleep time recommended The sample was 204 college students distributed across (PSQI), Insomnia Severity Index (IGI), Hospital Anxiety
by the National Sleep Foundation (NSF), 12.5% have health (74%), humanities (22.5%) and exact (3.5%) areas. and Depression Scale (HAD) to assess mood (depression
adequate sleep and 18% have sleep deficit. The time to Significant changes in sleep quality after onset of COVID- and/or anxiety). Statistical analysis evaluated the associ-
go to bed was 23: 30 ± 98 min and the time to get up was 19 pandemic was felt by 73.5%. Comparing results between ation between the variables and sleep (chi-square test).
7: 24 ± 102 min. For the non-parametric variables of the before and during COVID-19 period and the pandemic Results: 29 patients participated in the study, 21 women
rhythm of activity and rest, an average of IV of 0.72 ± 0.18 period, 72.1% of the students interviewed began to experi- (72.4%) and 8 men (27.6%), aged between 46 and 82
and the SI of 0.4 ± 0.09 were found. This shows that the ence anxiety symptoms. Furthermore, 64.9% experienced years (mean 63.76). 86% of the sample (20 women and 5
blind subjects presented a little fragmented sleep and intensification of symptoms of anxiety. Begining depres- men) had poor sleep quality by the PSQI, and 58.6% (16
low stability of the circadian rhythm. The average value sive symptoms after the pandemic in 45.6%, while 35.2% women and 1 man) had insomnia by the IGI. Mood dis-
of M10 was 124 ± 46, starting at 08: 11 min ± 123 min and reported intensification of depressive symptoms. The order (depression and/or anxiety) in 58.6% (15 women
for L5 17.3 ± 8, starting at 02: 25 ± 118 min. The AR value anxiety and depressive symptons were related as percep- and 2 men). Statistical analysis revealed a significant
of 0.64 ± 0.3. Conclusion: The majority (68%) of blinds tion of the individual. Regarding waking up, 75% reported association between sleep and the following variables:
in our study had a sleep duration and a sleep efficiency feeling fatigued and unwell. Moreover, regarding the sleep sex, age group, BMI and mood disorder. HFS duration,
above 85%. The subjects expressed a nocturnal sleep pattern interfering in daily functioning, 73% affirmed only showed statistical significance, on the IGI scale. The
pattern, as observed by L5 and M10 value and interval, fatigue during the day; 60.3% had mood changes; 49.5% only factor that did not show statistical significance was
signaling an absence of desynchronization and suggesting memory changes; 74.5% felt interference in concentra- the interval of botulinum toxin application. Conclusion:
a greater influence of non-photic factors on synchroniza- tion, and 53.4% in the learning capacity. The sleep quality The study showed that patients with hemifacial spasm
tion. Thus, further studies are needed to evaluate which affects 72.1% in the educational part; 40.2% in the social have a higher risk of impaired quality of life. Among all
and how these non-photic factors are synchronizing the part; 64.2% in the emotional part; 62.7% in the physical non-motor aspects, this study showed the prevalence of
sleep-wake cycle. part. Conclusion: Our study showed that there was an sleep disorder among patients with HFS, and so, places
impairment in the quality of sleep in college students, this disease as an additional risk in sleep impairment,
which may have impacted on the emergence or wors- but not isolated.
Sono ening of anxiety symptoms, and consequently impacted
their learning and social context. Understanding these
consequences of sleep disturbances and quality of life in Sono
the pandemic period, we can emphasize the importance
of intall a specialized psychopedagogical care program
by the university institution.

Sono

357
XXX Congresso Brasileiro de Neurologia

TL 1105862 TL 1105892 TL 1105945

EXCESSIVE DAYTIME SLEEPINESS: A PROSPECTIVE ANALYSIS OF SLEEP CENTRAL HYPERSOMNIA AND CHRONIC
SYMPTOM OF NARCOLEPSY AND AND MEMORY QUALITY AMONG MEDICAL INSOMNIA: EXPANDING THE SPECTRUM
OBSTRUCTIVE SLEEP APNEA STUDENTS AT AN AMAZONAS UNIVERSITY OF SLEEP DISORDERS IN LONG COVID
FROM THE GLYMPHATIC HYPOTHESIS SYNDROME – A PROSPECTIVE COHORT
Andersanny Moura Bernardes, Italo Felipe Cury,
Anderson Moura Bernardes, Anderlanny Moura STUDY
João Luiz Silva Botelho Albuquerque Cunha, Victoria
Bernardes Rosas Marques, João Victor da Costa Nunes, Ana Alissa Elen Formiga Moura, Danilo Nunes Oliveira,
Carolina Silveira Oliveira, Filipe Barroso Nascimento, Danielle. Mesquita Torres, José Wagner Leonel
UNIRG – GURUPI – TO – Brazil
Danilo Issa Mitozo Veras, Neyde Alegre Cavalcante, Tavares Junior, Paulo Ribeiro Nobrega, Pedro Braga
UFPI – TERESINA – TO – Brazil
Pedro Thiago de Cristo Rojas, João Pedro Moreira Neto, Manoel Alves Sobreira Neto
andersannybernardes@gmail.com Guilherme, Carlos Mauricio Oliveira
Hospital Universitário Walter Cantídeo. Fortaleza
Universidade do Estado do Amazonas. Manaus AM, CE, Brazil
Background: Excessive daytime sleepiness (EDS) is
Brazil
defined as the difficulty in staying awake and alert during alissamoura@gmail.com
Universidade Federal do Amazonas. Manaus AM,
the main wakefulness shift. It is characterized by sudden
Brazil
attacks of sleep, even though there is an attempt to stay Background: Long-onset COVID syndrome has been
awake, and is related to the main symptoms of narco- vrm.med19@uea.edu.br described in patients with COVID-19 infection with per-
lepsy and Obstructive Sleep Apnea (OSA). Narcolepsy sistence of symptoms or development of sequelae beyond
is a neurological disease of genetic, autoimmune origin Background: With the glymphatic system discovery, 4 weeks after the onset of acute symptoms, a medium-
and consists of five main symptoms: EDS, cataplexy, sleep responsible for ‘cleaning’ toxic waste from nervous tis- and long-term consequence of COVID-19. This syndrome
paralysis, hypnotic hallucinations and disturbed night sue during sleep, the emergence of neurodegenerative can affect up to 32% of affected individuals. Objective:
sleep. In contrast, OSA is a narrowing of the upper air- diseases related to memory from a dysfunction in this The present study aimed to characterize and evaluate
ways that can result in poor ventilation during sleep, and system has been discussed. Objective: The goal was the prevalence of sleep symptoms in patients with long
is characterized by EDS, nocturnal snoring, and nocturnal to evaluate the correlation between sleep quality and COVID syndrome. Methods: A total of 207 patients with
awakenings with interruption of breathing. Objective: memory in medical students at an Amazonas university post-COVID symptoms were evaluated through clini-
To systematically analyze how excessive daytime sleep- during a semester, and to observe how other factors (like cal evaluation with a neurologist, cognitive and sleep
iness is a fundamental symptom for both diseases and the pandemic) could interfere in this process. Method: test and specific exams in the subgroup complaining of
demonstrate the importance of an effective diagnosis This was a prospective observational study approved by excessive sleepiness (ES). Results: Among 189 patients
in the treatment of these pathologies. Methods: This is a the Ethics Committee in 2021 under statement of No. included in the long COVID sample, 48 (25.3%) had
systematic literature review based on data extraction in 4.978.487, with the exclusion criterion being that stu- sleep-related symptoms. Insomnia was reported by 42
indexes such as PUBMED and MEDLINE. Publications dents under the age of 18 could not participate. It was patients (22.2%), and excessive sleepiness was reported
between 2018 and 2022 were considered, in English and conducted using the Mini-Sleep Questionnaire (MSQ) to by 6 patients (3.17%). Four patients with ES were eval-
with the following descriptors: Excessive daytime sleepi- assess sleep quality, Epworth Sleepiness Questionnaire uated with polysomnography and test, multiple sleep
ness, Narcolepsy, Obstructive sleep apnea. Results: EDS to assess the degree of daytime sleepiness, Prospective latencies test, and Actigraphic data. Of a total of 4 patients
can be found in patients who have both pathologies or who and Retrospective Memory Questionnaire (PRMQ-10) to with ES, 3 fulfilled the clinical criteria for central hyper-
have only one of them, for this reason, adequate clinical assess retrospective and prospective memory, with the somnia, and one reported symptoms of ES that resolved
characterization is the starting point for determining the following cut-off points 25, 10 and 20 respectively, and a spontaneously after 6 months without intervention. We
diagnosis. The diagnosis of both disorders must use tools self-reported sleep diary for two weeks. Results: A total of observed a high prevalence of cognitive complaints in
that analyze sleep quality, situations in which drowsiness 132 academics were included, 77 (58,38%) female and 55 these patients. Discussion: This study demonstrated a
is expressed and associated symptoms: snoring, hallu- (41,67%) male, the means (standard deviations) obtained high prevalence of sleep symptoms in a cohort of long-
cinations, cataplexy. In addition, polysomnography can from the PRMQ-10, MSQ and Epworth tests were 26,44 term COVID-19 patients with persistent sleep disorders
be used to analyze the quality of sleep. Finally, in cases (6,43); 32,34 (8,88) and 10,58 (4,14) respectively. From the up to 18 months after infection. Insomnia was the most
where there is evidence of both diseases simultaneously, sample, 59 students were able to complete the sleep diary frequent sleep disorder in these patients. To the best of
it is important to be aware that the therapeutic options are during the requested time: the total sleep mean was 6,84 our knowledge, we have reported the first cases of central
based on the signs and symptoms that weigh more heav- hours (0,78). A high degree of memory failure complaints hypersomnia after COVID. One of our patients fulfilled
ily on the therapeutic options. Conclusion: The proper were evidenced in 114 participants (86.36%), excessive clinical and polysomnographic criteria for narcolepsy.
diagnosis of the cause of EDS goes far beyond the return daytime sleepiness in 74 (56.06%) and sleep alterations in All patients with ES responded to wakefulness-promoting
of the patient’s quality of life and the guarantee of daily 114 (86.36%), and significant correlations at p<0.05 level agents. We have reported three patients with documented
social activities. As a result, when there is a complaint of between anxiety and depressed states at the time of data central hypersomnia after COVID-19. All 3 patients were
EDS, the follow-up and clinical analysis must be cautious collection; anxiety and worsening memory failure com- young and had no sleep disorders prior to COVID infec-
so that the treatment can adequately identify the disorder plaints during the pandemic period and worsening sleep tion, suggesting a strong association with SARS-CoV-2
that affects the patient and help him/her. and memory complaints during the pandemic; between infection. To our knowledge, this is the first report of this
PRMQ-10 memory failure complaints and MSQ sleep association in the literature. In addition to the possibility
changes, and between the amount of sleep and better of direct viral infection, hypersomnia after COVID might
Sono results on the PRMQ-10. Conclusion: Sleeping changes, be related to immune and inflammatory hyperactivation
daytime sleepiness, and memory failure were significantly resulting in hypothalamic infiltration by CD4+ and CD8+
observed among the students, evidencing the direct cor- T cells leading to neuronal damage. Conclusion: Sleep
relation between sleep and memory, since studies show disorders are common in patients with long COVID syn-
that neurotoxic proteins can be deposited in the nervous drome, particularly insomnia. Chronic insomnia and
tissue even after a poor sleep day, thus being a possible central hypersomnia may expand the spectrum of post-
explanation for the correlation found between the MSQ COVID sleep disturbances.
and the PRMQ-10, i.e., the association between important
sleep changes with high complaints of memory failure.
Sono

Sono

358
XXX Congresso Brasileiro de Neurologia

TL 1105948 TL 1106048 TL 1106078

CANNABIDIOL FOR RAPID EYE MOVEMENT SLEEP HABITS IN THE SLEEPING HAMMOCK THE RELATION BETWEEN THE CHIARI
SLEEP BEHAVIOR DISORDER IN A TERTIARY HOSPITAL IN THE STATE OF MALFORMATION AND SLEEP APNEA
CEARÁ
Carlos Mauricio Oliveira de Almeida, Manuelina M.C. Guilherme Belo Carvalhêdo, Victória de Menezes Sá
Brito, Nayanne B Bosaipo, Angela V Pimentel, Vitor Danielle Mesquita Torres, Alissa Elen Moura Lazera, Pedro Durante Junqueira, Juliana Lago de
Tumas, Antônio W Zuardi, Jose A.S Crippa, Jaime E.C Formiga, Patricia Gomes Damasceno, Pedro Braga Araújo, Júlia Leite Xavier Bertrand, Dayana Dourado
Hallak, Alan Luiz Eckeli Neto, Manoel Alves Sobreira Neto de Oliveira Costa

Universidade Estadual do Amazonas. Manaus AM, Hospital Universitário Walter Cantidio. Fortaleza UNICEUMA. São Luís MA, Brazil
Brazil CE, Brazil UFMA – São Luís – MA – Brazil
Universidade de São Paulo. Ribeirão Preto SP, Brazil HUWC. Fortaleza CE, Brazil
Universidade de São Paulo – Manaus – SP – Brazil viclazera@hotmail.com
daniellemesquita@hotmail.com
acrmauri33@gmail.com Background: Chiari malformation occurs when the skull
Background: The hammock is an item that has followed base and the area of the upper part of the spine are not
Background: Cannabidiol (CBD) is one of the main the evolution of society, since the indigenous reality, and properly formed, causing an inappropriate bulge of the
non-psychoactive components of Cannabis sativa and has spread to the other inhabitants, in a way that involves cerebellum through the foramen magnum. This syndrome,
may represent an alternative route for treating REM sleep a complex diversity of values, tastes and everything else by affecting the central nervous system, allows the devel-
behavior disorder (RBD). Methods: a phase II/III, dou- that forms the national identity (1). Several benefits of the opment of respiratory syndromes. The most noteworthy
ble-blind, placebo-controlled clinical trial was conducted hammock have already been reported in the literature (2). of these is sleep apnea, characterized by noises and inter-
in 33 patients with RBD associated with Parkinson’s dis- The type 1 polysomnography (PSG) test is the gold stan- ruptions in breathing during sleep, gradually decreasing
ease (PD). Patients were randomized to CBD in doses of 75 dard method for studying sleep-associated parameters blood oxygenation and, consequently, increasing blood
to 300mg or matched capsules placebo and were followed (3). It is not known whether there is a difference between pressure and decreasing cerebral blood flow. Therefore,
up for 14 weeks. Primary outcomes were the frequency of the sleep assessment data obtained using PSG type 1 laboratory tests, such as polysomnography, are necessary
nights with RBD, CGI-I, and CGI-S. Results: CBD showed performed in bed and in the hammock. Objectives: To to confirm the diagnosis of sleep apnea and magnetic
no difference in relation to placebo for primary outcomes. analyze sleep habits in the hammock sleeping and percep- resonance imaging to diagnose the type 1 Chiari malfor-
Regarding secondary outcomes, we observed a significant tions of patients in a sleep service of a tertiary Hospital in mation, also requiring neurosurgery to treat the Arnold-
improvement in average sleep satisfaction from the 4th to the state of Ceará. Methods: We perform a observational Chiari syndrome. Objectives: The present study aims to
8th week in the CBD versus placebo group with p=0.049 cross-sectional study. The parameters of sleep preferences analyze the relation between Arnold-Chiari Syndrome
and p=0.038, respectively. Conclusion: CBD, as an adjunct and habits were evaluated by a questionnaire applied to and the breathing disorder Sleep Apnea. Methods: It has
therapy, showed no reduction in RBD manifestation. An patients at the sleep service of a large tertiary Hospital been accomplished a bibliographical research indexed in
improvement in sleep satisfaction with doses of 75-150mg in the city of Fortaleza-Ce. Results: The final sample the databases SciELO, PubMed and Google Acadêmico,
has been noted. consisted of 100 patients. There was a predominance of studies published in the years of 2010 to 2018, using
of females (67%). The mean age of participants was 51 the following descriptors: “Apnea”, “Chiari” and “Sleep”.
years. Most of then came from the capital (Fortaleza). Results: By the selection of three different articles it was
Sono 33% of participants prefer to sleep in a hammock for possible to infer that Chiari Malformation type 1 (MCI)
different reasons. The most cited reasons were: greater is a congenital condition in which the cerebellum enters
comfort, milder temperature, being able to swing. 73% the spinal canal because of a malformation of the skull.
of that who preferred hammock came from the interior This syndrome is strongly linked to respiratory disorders,
of the state. 45% of the participants prefer to sleep in a evidencing Central Sleep Apnea, because of the proximity
diagonal position on the hammock. During childhood, of the respiratory structures of the brain and the struc-
59% of the participants preferred to sleep in a hammock. tures involved in the Chiari Malformation. As a direct
44% reported that at least one parent prefers to sleep in consequence, there is a possible lack of oxygenation in
a hammock. 70% of the patients have children. Of these, the brain and very poor sleep quality. Conclusion: It is
20% reported that their children sleeps in a hammock. The evident, therefore, that polysomnography, a test realized
most common diagnosis was Obstructive Sleep Apnea to analyze the quality of sleep by measuring brain waves,
(42%). 14% patients reported that snoring improves when breathing and the amount of oxygen in the blood, becomes
they sleep in a hammock. 27% of patients reported hav- an ally in the identification of Sleep Apnea. While MRI is
ing a greater feeling of rest when sleeping in a hammock. essential for the identification of Chiari type 1 malfor-
Conclusion: The habit of sleeping in a hammock is part mation. Both decompression surgery, for the treatment
of our people’s culture. Performing the PSG type 1 test in of the malformation, as well as servoventilation for the
a hammock can provide more reliable data on the sleep treatment of apnea, are indicated. Thereby, as a direct
conditions of individuals who sleep in a hammock. There consequence, there is a reduction in the symptoms of
may be a difference between the PSG data performed on sleep-disordered breathing.
the bed and on the hammock. Larger studies comparing
the performance of the exam in the two environments are
necessary for a better analysis. Sono

Sono

359
XXX Congresso Brasileiro de Neurologia

TL 1106100 TL 1106273 TL 1106309

BLEPHAROSPASM PATIENTS AFTER ARTIFICIAL INTELLIGENCE AS A VALIDATION OF THE BRAZILIAN


BOTULINUM TOXIN– SLEEP APPROACH MONITORING STRATEGY FOR INDIVIDUALS PORTUGUESE VERSION OF STANFORD
WITH OBSTRUCTIVE SLEEP APNEA TREATED CATAPLEXY QUESTIONNAIRE
Silvia Cordova Lestingi, Lenise Jihe Kim, Bruno Silva
Gonçalves, Sônia Maria Azevedo Silva, Henrique WITH POSITIVE AIRWAY PRESSURE – A
Ana Carolina Dias Gomes, Lucio Huebra Pimentel
Ballalai Ferraz, Fernando Morgadinho Coelho, PROPOSAL TO THE PUBLIC HEALTH SYSTEM Filho, Ana Carolina Rodrigues Aguilar, Giselle de
Vanderci Borges IN THE STATE OF CEARÁ Martin Truzzi, Sergio Tufik, Fernando Morgadinho
Coelho
Universidade Federal de São Paulo. São Paulo SP, Camila Ferreira Leite, Manoel Alves Sobreira-
Brazil Neto, Nícolas Fonteles Leite, Renê Ripardo Calixto, Universidade Federal de São Paulo. São Paulo SP,
Universidade de São Paulo. São Paulo SP, Brazil Mateus Machado Costa, Kettleyn Alves Paiva, Brazil
Mikaely Lima Melo, Adriano José Xavier Santiago,
silvia_lestingi@yahoo.com Samir Câmara Magalhães, Auzuir Ripardo fernandomorgadinho@hotmail.com
Alexandria
Background: Blepharospasm is a focal dystonia that Background: Cataplexy is the most specific REM clinical
affects the orbicularis oculi muscles. The interest in non- Universidade Federal do Ceará. Fortaleza CE, Brazil manifestation of narcolepsy, and it is characterized by a
motor symptoms is due to their impact on quality of life. IFCE. Fortaleza CE, Brazil sudden and involuntary episode of loss of muscle tone
OBJETIVE: We evaluated the frequency of sleep disorders UFC-EBSERH. Fortaleza CE, Brazil during wakefulness. Cataplexy cannot be easily recognized
and circadian rhythm in a sample of brazilian blepharo- IFCE – Canindé – CE – Brazil when clinical features are atypical or when the physician
spasm patients.Methods: A total of 51 patients, who met is unfamiliar with its characteristics. The unstructured
camilafleite@ufc.br
the clinical criteria for blepharospasm, evaluated by two clinical interview is the only diagnostic method most
specialists in movement disorders, were recruited from common, and the diagnosis by targeted questionnaire
Background: Obstructive sleep apnea (OSA) is character-
the outpatient clinic for movement disorders of two ref- might help identify cataplexy. Methods: Stanford cataplexy
ized by recurrent episodes of partial or total upper airway
erence centers in the city of São Paulo. The selected 13 questionnaire is a self-applied 51-question questionnaire.
obstruction, with interruptions of the sleep cycle, often
patients were evaluated from 13 days before to 13 days after This validation consisted of an initial translation and
associated with intermittent hypoxic/hypercapnic stress.
using botulinum toxin. They were interviewed, underwent back-translation from English into brazilian Portuguese
The prevalence of OSA is high and is strongly associated
physical examination and actigraphy, and completed of the questionnaire followed by a pilot study including
with cerebrovascular and cardiovascular events. Its gold
sleep diaries.Results: After using botulinum toxin, the 10 participants for the cultural adaptation of the scale.
standard treatment is performed through the applica-
group that reported sleep improvement exhibited a 50% The application of the final version to 155 different partici-
tion of positive airway pressure. This treatment, when
decrease in sleep latency. There was no change in restless pants, with 35 narcoleptic patients, was made. Results: 155
properly instituted, is cost-effective. In the State of Ceará,
leg syndrome or circadian rhythm. Patients who reported consecutive patients, aged 18-85 years old, completed the
four sleep-specialized services assist and follow patients
no sleep improvement after using botulinum toxin pre- questionnaire. Cultural adaptations were made to a better
undergoing treatment with positive airway pressure. The
sented poorer synchronization of the light-dark cycle. comprehension of patients. The brazilian version of the
positive pressure equipment used for the treatment is pro-
Conclusion: Blepharospasm patients have poor sleep Stanford cataplexy questionnaire showed similar results
vided to patients through the State Health Department.
quality. About 50% of the patients had sleep improve- to those depicted in the original version. Conclusion: The
Objective: To develop a system that is applied to the pub-
ment after using botulinum toxin. The synchronization brazilian Portuguese version of the Stanford cataplexy
lic health system (SUS – Sistema Único de Saúde) for the
of the light-dark cycle may influence this improvement. questionnaire showed to be a valid tool for assessing the
management and monitoring of positive airway pressure
patients with suspicion of having cataplectic events and
users who are followed up in specialized sleep services
has a potential impact on clinical decisions.
Sono and, from this product, generate a bigdata that allows
offering solutions in the sphere of management, clinical
follow-up and science based on information generated Sono
by artificial intelligence models. Methods: The developed
system uses cloud computing techniques to interact with
the user through conventional browsers and the acquired
data are stored in the cloud, forming a bigdata. Artificial
intelligence techniques will be used to extract information
that enhances the treatment of patients with OSA, both
individually and collectively. Results: The platform is
under construction, based on the research of the necessary
variables and constructs. An entity-relationship diagram
was produced and integrated into the construction pro-
cess. The combined variables for the artificial intelligence
strategy are in the adjustment phase. It is expected that
the developed program will make it possible to expand
the follow-up of patients followed up in specialized sleep
services, enabling better communication between ser-
vices from the public health system, as well as providing
information on the effectiveness of therapy to the State.

Sono

360
XXX Congresso Brasileiro de Neurologia

TL 1106313 TL 1106316 TL 1106330

APPLICATION OF THE RAPID EYE BRAIN COMPLIANCE BEHAVIOR DURING BAD DREAMS IN NARCOLEPSY TYPE 1
MOVEMENT SLEEP BEHAVIOR DISORDER SLEEP – INITIAL ANALYSIS OF A PILOT
Lucio Huebra Pimentel Filho, Cristina Frange,
SCREENING QUESTIONNAIRE IN PATIENTS STUDY Fernando M S Coelho
WITH NARCOLEPSY
Karla Carlos, Tainã Paulo Zanata Trombetta, Douglas Universidade Federal de São Paulo. São Paulo SP,
Giselle Truzzi, Maria Julia Figueiró Reis, Sergio Tufik, Sousa Soares, Davi Dantas Muniz, Ana Flávia Brazil
Maria Fernanda Naufel, Fernando Morgadinho Machado Pitz, Gustavo Henrique Frigieri1, Gilmar
Coelho Fernandes Prado fernandomorgadinho@hotmail.com

Universidade Federal de São Paulo. São Paulo SP, Departamento de Neurologia e Neurocirurgia da Background: Narcolepsy can present with different com-
Brazil Escola Paulista de Medicina da UNIFESP binations of severity and frequency of sleep paralysis,
Brain4care®, Scientific Director sleep-related hallucinations, and cataplexy. Symptoms
fernandomorgadinho@hotmail.com
ka.carlos1@hotmail.com related to REM sleep can happen frequently, such as
lucid dreams, vivid dreams, epic dreams, and nightmares.
Background: REM sleep behavior disorder is charac-
Background. Intracranial compliance represents the cra- Objective: The objective is to highlight the increased
terized by the loss of muscle atony during REM sleep
nium’s ability to tolerate an increase in volume without a frequency of bad dreams in patients with type 1 narco-
associated with abnormal behaviors, often with dream
corresponding increase in intracranial pressure. Waves lepsy, their impact on sleep quality, and possible factors
enactments, which can even cause bodily harm to the
pulses obtained by non-invasive method of monitoring associated with this increased frequency. Methods: We
patient and their bed partner. It is considered a REM sleep
intracranial pressure curves is a validated and more prac- evaluated 52 patients diagnosed with type 1 narcolepsy
parasomnia and can be idiopathic or associated with
tical form to evaluate brain compliance. Nonetheless, little according to the ICSD3 diagnostic criteria. They under-
other diseases, including narcolepsy. When associated
is known about the impact of brain compliance on sleep. went clinical evaluation and application of the Pittsburgh
with narcolepsy, it usually presents an earlier onset and
During the wake state and REM-sleep period, the brain Sleep Quality Rating Scale, Epworth Sleepiness Scale, and
milder symptoms than when it is associated with other
metabolism is high and also the need for blood. Once Narcolepsy Severity Scale. In addition to clinical data, data
diseases, often going unnoticed by patients and health
the intracranial pressure is associated to blood volume, from complementary diagnostic tests and medications in
professionals. Objective: To evaluate signs and symp-
we hypothesized that the compliance during wakeful- use were also recorded. Results: Mean age of patients was
toms of REM sleep behavior disorder in patients with
ness and REM-sleep is higher than in N2 and N3. Our 37.62 ± 11.974, with a minimum of 18 and a maximum
narcolepsy through the application of a questionnaire.
Objective: is to evaluate the behavior of brain compliance of 70. Females corresponded for 59.6%. More than half
Methods: In the study, the Rapid Eye Movement Sleep
during sleep stages and wakefulness, through pulses mor- of the patients, 55.8% had bad dreams more than once a
Behavior Disorder Screening Questionnaire was applied
phology (P1, P2, P3) of intracranial pressure and P2/P1 week in the month of the interview, 15.4% had them less
to patients with type 1 (N1) and type 2 (N2) narcolepsy.
ratio. Methods: The assessment of brain compliance was than once a week and 28.8% had none in the last month.
All patients evaluated had excessive daytime sleepiness
performed using the non-invasive intracranial pressure There was no correlation between the frequency of symp-
and had a previous diagnosis of the disease through poly-
(NICP) method through the Brain4care® device overnight toms related to bad dreams and the narcolepsy severity
somnography and the multiple sleep latency test. Subjects
simultaneously with the performance of type I polysom- scale (person 13.785, p = 0.130). Regarding symptom-
were classified into N1 and N2 according to the result of
nography (Alice 6, Phillips Respironics®). We evaluated atic scales, the prevalence of poor sleep was associated
the cerebrospinal fluid hypocretin dosage, which was per-
brain compliance data in the first 10 minutes of each of with the Pittsburgh sleep quality index score (F 5.278; p=
formed in all patients studied. Results: 71 adult individ-
the following situations: wakefulness (V), sleep N2 (N2), 0.003), but without association with the Epworth sleepi-
uals were evaluated, 45 of whom had N1 and 26 had N2.
sleep N3 (N3) and REM (REM). Results: The patient did ness scale (F0.616; p = 0.608). When correlating with the
The score considered positive for the questionnaire was
not complain in using the Brain4Care device through- use of medications, there was no correlation between
= or > 5. The mean score was 8.276 (SD: 2.801) in patients
out the night. We obtained the following P2/P1 ratios: the use of stimulants (person 1.192; p=0.755) or anti-
with N1 and 6.368 (SD: 4.669) in patients with N2. It was
V= 1.29; N2=1.21; N3=1.17 and in REM=1.30. The P2/P1 cataplectics (person 5.120; p= 0.163). There was also no
observed that there was a higher score on the question-
ratio was higher in REM when compared to NREM sleep individual difference between each type of stimulant in
naire in patients with lower levels of hypocretin, and in
(p > 0.001); higher in REM when compared to N3 sleep use (person 7.591; p=0.576). The use of different anticat-
the N1 group, the score result was significantly positive
(p > 0.001). The behavior of the P2/P1 ratio was similar aplectic antidepressants correlated differently with the
(p=0.00331). Conclusion: It was observed that patients
when comparing wakefulness and REM. Discussion: The prevalence of bad dreams (person 25.919; p=0.011), with
with narcolepsy have high scores when responding to the
main finding of this pilot study was that the P2/P1 ratio tricyclic showing a negative association with a higher fre-
Rapid Eye Movement Sleep Behavior Disorder Screening
increased during the night’s sleep, being higher during quency of bad dreams and selective serotonin reuptake
Questionnaire. Despite being a known comorbidity in
wakefulness and REM sleep. We observed a decrease in inhibitors having a positive association with the absence
people with narcolepsy, this research shows the symp-
brain compliance in REM sleep. Although still an isolated of bad dreams. Conclusion: Oneiric symptoms are fre-
toms of REM sleep behavior disorder are more important
study, this is an interesting finding, as brain metabolism quent in type 1 narcoleptic population. The use of anti-
than usually considered in clinical practice.
increases during REM sleep, equivalent to what is observed depressants with an anti-cataplectic effect may help to
in wakefulness. However, brain compliance in REM sleep control these symptoms.
Sono is quite low, similar to conditions leading to intracranial
hypertension. One possible cause of the increase in the
Sono
P2/P1 ratio may be related to the presence of many hypo-
pneas that occurred during REM sleep. The evaluation of
brain compliance behavior in patients with sleep-disor-
dered breathing will be carried out in the future, which
will allow us to better understand the impact of obstruc-
tive sleep apnea in that particular situation. A relevant
aspect in this study is the potential use of the variable
brain compliance in the polysomnography set up, adding
information with impact on clinical outcomes involving
OSAS. Conclusion: Brain compliance appears to be asso-
ciated with sleep stages and is progressively reduced from
wakefulness to N3 sleep. In this case brain compliance
is no different in wakefulness and REM. The decrease in
brain compliance in REM sleep may be associated with
severe obstructive sleep apnea.

Sono

361
XXX Congresso Brasileiro de Neurologia

TL 1106489 TL 1106495 TL 1106541

CONTINUOUS MONITORING OF BRAIN BIOCHEMICAL ANALYSIS, BODY THE IMPORTANCE OF SLEEP FOR
COMPLIANCE DURING SLEEP: EXPANDING COMPOSITION AND SLEEP QUALITY IN SUCCESSFUL NEUROREHABILITATION
THE SPECTRUM OF POLYSOMNOGRAPHIC HEALTH PROFESSIONALS: IS THERE A AFTER STROKE
VARIABLES RELATIONSHIP?
Cristina Frange, Brian James Murray, Fernando M
Karla Carlos, Douglas Sousa Soares, Tainã Paulo Marta Maria da Silva Lira-Batista, Júlia Rachel S Coelho
Zanata Trombetta, Davi Dantas Muniz, Camila Ferreira Meneses, Gleydyson Wesley Freire Lima,
Universidade Federal de São Paulo. São Paulo SP,
Coelho Carneiro, Ana Flávia Machado Pitz, Gustavo Jaynara Keylla Moreira da Silva, Maria Rita Carvalho
Brazil
Henrique Frigieri, Gilmar Fernandes Prado Silva, Rogério da Silva Batista, Maria do Carmo de
UofT – Canadá
Carvalho e Martins
Escola Paulista de Medicina – UNIFESP
fernandomorgadinho@hotmail.com
Brain4care HU-UFPI. Teresina PI, Brazil
IESVAP – PARNAIBA – RN – Brazil
ka.carlos1@hotmail.com Background: Proper sleep has important clinical implica-
PPGAN-UFPI. Teresina PI, Brazil
tions for the learning and re-learning of movements and
FAESPI. Teresina PI, Brazil
Background: Based on the principle of detecting small activities of daily life for optimal stroke rehabilitation. Both
IFPI. Teresina PI, Brazil
deformations of the skull caused by changes in intracranial sleep and rehabilitation are critical for many activities that
Universidade Federal do Piauí. Teresina PI, Brazil
pressure, a non-invasive monitoring method was devel- may need to be learned or re-learned following stroke, and
oped. The aim of our study was to evaluate the possibility fgamarthalb@gmail.com for functionality in everyday life. In this context, sleep is
of performing continuous monitoring of brain compliance essential in neurorehabilitation practice as it supports
throughout the night, through pulse morphology (P1, P2, Background: Food cravings are regularly experienced by neuroplasticity, memory, and learning. Objective: We
P3) of intracranial pressure synchronized to the other individuals with and without eating disorders, especially aimed to systematically review the literature to answer
physiological variables routinely addressed in a type I for palatable, highly processed foods and high levels of if sleep (including naps) matters as a contributing fac-
sleep study. Methods: After assembling the patient to carbohydrates, refined sugars, and/or fat. The relationship tor for neurorehabilitation in patients after stroke in
perform the polysomnography, we positioned the device between changes in nutritional status, cardiovascular, adults. Methods: A systematic review was performed
on her head. The patient was instructed to call us if there neurodegenerative, metabolic-inflammatory diseases, and on clinical trials investigating the influence of sleep on
was any discomfort regarding the use of the device. The increased oxidative stress are widely established; however, post-stroke neurorehabilitation. The PRISMA guidelines
assessment of brain compliance was performed by the there is still little evidence for the analysis of sleep quality. were followed. Seven investigations were included in this
non-invasive intracranial pressure (NICP) method through Objective: To outline the profile of nutritional status, food review. Data were extracted and assessed for quality and
the Brain4care® device throughout the night. Sleep assess- desire, biochemical markers of inflammation, and sleep risk of bias. We looked at any neurorehabilitation setting,
ment was performed using type I polysomnography (Alice quality in health professionals at a university Hospital. compared sleep versus no sleep in rehabilitation, and
6 – Phillips Respironics® device). We used the AASM scor- Methods: This pilot study was cross-sectional, quantita- explored these factors in stroke patients versus healthy
ing rules to analyze the sleep study. We superimposed the tive, and prospective. The target population consisted of individuals. Results: This systematic review found that
graph representing the variation of the P2/P1 ratio with health professionals from the Hospital clinical staff with published neurorehabilitation interventions included
the hypnogram. Results: The patient remained overnight no previous neurological complaints. Questionnaires were nighttime sleep and long naps (? 90mins after interven-
with the sensor on her head. It did not move at any time applied to measure the Intense Desires for Food – Trait tion) as the intervention improved motor consolidation
during the monitoring. The recording duration was 5h and State questionnaires; inflammation was recorded in patients following stroke. Sleep should be encouraged
44min 42s. The useful pulses for evaluation were in the with sociodemographic and body composition data from in the treatment of stroke to maximize outcomes. After a
line above 60%. Only 6 minutes of the exam could not be bioimpedance. The Pittsburgh Sleep Quality Index and stroke, sleep disorders of any kind (e.g., OSA, insomnia,
analyzed due to the presence of artifacts interfering with the Epworth Sleepiness Scale were used for sleep assess- circadian rhythm disorders) can negatively influence
the P1 and P2 waves. The P2/P1 variation graph was ade- ment. Results: Statistically significant correlations were neuroplasticity recovery and physical rehabilitation.
quately superimposed on the hypnogram. In the morning, found between inflammation, sleep quality, phase angle, Conclusion: The available data suggest that sleep should
the patient reported no complaints related to device use. and body fat percentage. It is inferred that moderate food be considered in the treatment plan for successfully tar-
The signals recorded by the polysomnography equipment cravings are related to poor sleep quality, a biochemical geted physiotherapy to optimize cognitive and motor
were not disturbed by the Brain4Care device. Results: In profile compatible with inflammation in health profes- learning. Sleep hygiene and therapies to improve sleep,
this pilot study, we were able to demonstrate the feasibility sionals. Conclusion: Overweight or obesity, increased both quality and quantity are imperative in stroke treat-
of including a sensor aimed at assessing brain compliance oxidative stress, poor sleep quality and excessive day ments as they modulate rehabilitation outcomes.
and performing continuous monitoring of its variation sleeping are present in the profile of healthcare profes-
during sleep. The data shown here are part of a large sionals, regardless of the work shift.To establish cause and
project that involves analyzing the impact of obstructive effect, we suggest longitudinal studies assessing these Sono
sleep apnea on brain compliance. The data plotted on relationships comparatively in the long term.
the hypnogram allowed a visual analysis of the P2/P1
ratio curve, which behaved as expected, considering the
data that we have already obtained in the different sleep Sono
stages of monitoring this patient. Conclusion: It was pos-
sible to monitor overnight brain compliance through the
non-invasive device Brain4Care. The Brain4Care device
did not determine interference in the signal captured for
performing the polysomnography, preserving the quality
of both assessments.

Sono

362
XXX Congresso Brasileiro de Neurologia

TL 1106578 TL 1106586 TL 1106618

RESTLESS LEG SYNDROME/WILLIS-EKBOM PROPOSED PROTOCOL FOR DISPENSING CHRONONUTRITION AND NARCOLEPSY:
DISEASE (RLS/WED) AND QUALITY OF LIFE POSITIVE AIRWAY PRESSURE EQUIPMENT A BEHAVIORAL APPROACH TO SYMPTOM
IN HEMODIALYSIS PATIENTS FOR USERS OF THE BRAZILIAN UNIFIED MANAGEMENT
HEALTH SYSTEM IN THE STATE OF CEARÁ
Caroline Meneses Resende, Patrícia Pereira Nunes, Maria Júlia Figueiró Reis, Giselle de Martin
Ellen Dayanne Barros Silva, Lucas Nascimento WITH SLEEP-DISORDERED BREATHING Truzzi, Maria Fernanda Soares Naufel, Fernando
Monteiro, Deryc Cleyner Piones Bastos, Max Luiz Oscar Loiola Alencar Nerto, Camila Ferreira Leite, Morgadinho Santos Coelho
Mendes Ramires Filho, Rayane Leite da Silva, Tiago Alan Eckeli, Alessandra Calland, Samir Câmara,
Gomes de Andrade, Flávio Teles de Farias Filho, Lívia Universidade Federal de São Paulo. São Paulo SP,
Isabella Matos, Ivan Guerra Freitas, Lia Belchior Brazil
Leite Goes Gitaí Nery, Patricia Damasceno, Manoel Alves Sobreira
Universidade Federal de Alagoas. Maceió AL, Brazil Neto julia_figueiro@hotmail.com

caroline.resende@famed.ufal.br Centro Universitário Christus – Unichristus. Background: Although still incipient, there is an increas-
Fortaleza CE, Brazil ing body of data suggesting association between circa-
Background: Chronic kidney disease (CKD) is associ- Universidade Federal do Ceará. Fortaleza CE, Brazil dian nutritional preferences and obesity. Some studies
ated with significant morbidity and mortality and can USP – Ribeirão Preto – CE – Brazil demonstrated that concomitance between food intake and
negatively impact on many aspects of health and well- HGCC. Fortaleza CE, Brazil elevated levels of melatonin in bloodstream would predis-
being. Restless Leg Syndrome/Willis-Ekbom disease Hosp Messejana. Fortaleza CE, Brazil pose the individual to a higher insulin resistance, higher
(RLS/WED) is one of the most common and unrecog- manoelsobreira@yahoo.com.br glucose levels and an increase in body mass index (BMI).
nized sleep disorders in CKD patients on hemodialysis It is widely known that obesity is correlated to a higher risk
and is associated to worse quality of life. Objective: The Objective: This paper aims to propose a protocol to assist of developing sleep disorders, such as obstructive sleep
current study aimed to investigate the prevalence of RLS/ and standardize the dispensing of positive pressure equip- apnea (OSA), which increments excessive daytime sleepi-
WED in patients undergoing hemodialysis, as well as ment for patients with sleep-disordered breathing (SDB) ness (EDS) and conversely decreases quality of life. Also, it
the association with quality of life domains. Methods: A in the brazilian Unified Health System (SUS) of the State is known that patients diagnosed with narcolepsy tend to
total of 236 patients undergoing hemodialysis from three of Ceará. Methods: Methodological study for the devel- present with obesity and a sleep phase delay. In addition,
local dialysis units were enrolled from February to June opment of a protocol for the dispensing of positive airway it has been demonstrated that patients with narcolepsy
2022. RLS/WED was identified using a single screening pressure (PAP) equipment to be implemented into the tend to have altered bone mineral metabolism. A study
question (‘When you try to relax in the evening or sleep SUS, meeting the demands of the State of Ceará. The pro- using an animal model showed that the use of modafinil, a
at night, do you ever have unpleasant, restless feelings in tocol was developed from an integrative literature review common drug used in the management of EDS, is associ-
your legs that can be relieved by walking or movement?’) and is grounded on the opinion of experts. The chosen ated with a reduction in bone mass. According to another
and chronic persistent form was defined as symptoms strategy was to apply electronic surveys made available study, circadian disruption plus sleep restriction would
at least twice a week. Quality of life was evaluated using to the consulted experts on two occasions. Results: The also predispose to disruption in bone homeostasis. There
the the Kidney Disease QoL Short Form (KDQOL-SF) by experts have defined the recommended PAP equipment, are still no studies that investigate the association between
face-to-face interview. Results: The prevalence of RLS/ as well as the best way to organize the dispensing of circadian nutritional preferences and the elevated BMI
WED in hemodialysis patients was 30.1%. About 20.4% those devices by the SUS according to the local needs. observed in patients diagnosed with narcolepsy, nor the
had chronic persistent form. Patients with RLS/WED, Discussion: The creation of this protocol aims to decrease reduction of bone mass in this population, to the extent
compared to non-RLS/WED patients, had significantly the time needed to make PAP available for patients with of our knowledge. This is the aim of our study. Methods:
worse KDQOL-SF scores across multiple domains as sleep SDB, which would improve the treatment efficacy and We have selected 33 patients diagnosed with type 1 and
(p=0.003), quality of social interaction (p=0.02), cognitive reduce costs for the public sector. 18 patients diagnosed with type 2 narcolepsy, based on
function (p=0.001), burden of kidney disease (p=0.025), a hypocretin measurement in cerebrospinal fluid pre-
effects of kidney disease (p=0.01), symptoms (p=0.01), viously performed. We intend to use data from medical
physical functioning (p=0.01), social function (p=0.04), Sono records to evaluate the times and nutritional values of
and emotional role (0.02). Conclusion: Therefore, this the main courses and assess their correlation to anthro-
study show that RLS/WED is a common disorder in pometric endpoints. Also, we intend to compare bone
hemodialysis patients and is related to worse perception mass and nutritional intake of micronutrients between
of quality of life in several of its domains. these patients, evaluating the potential of these measure-
ments of becoming biomarkers of disease. Conclusion:
Narcolepsy is commonly associated with an important
Sono decrease in quality of life, in part because of its morbidity
with obesity and OSA. Defining a behavioral approach to
the treatment of obesity, based on nutritional counseling,
would optimize symptoms control in these patients. In
addition, the definition of a decreased bone mass as a part
of the pathophysiology of this disease – and a possible
biomarker – wound allow faster and cheaper diagnosis,
also favoring a better management of bone homeostasis
in clinical follow-up.

Sono

363
XXX Congresso Brasileiro de Neurologia

TL 1106638 TL 1106646 TL 1106647

CHRONOTYPE AND HEMODIALYSIS- ASSESSMENT OF SLEEP AND QUALITY OF IS RESTLESS LEGS SYNDROME IN
RELATED SOCIAL JET LAG IN CHRONIC LIFE IN SCHOOL CHILDREN PARKINSON DISEASE PATIENTS
KIDNEY DISEASE PATIENTS ASSOCIATED WITH ANY SPECIFIC FACTOR?
Carla de Oliveira Carletti, Karla Carlos, Gilmar
Patrícia Nunes Ribeiro, Caroline Meneses Resende, Fernandes do Prado Manoel Alves Sobreira-Neto, Emmanuelle Sobreira,
Ellen Dayanne Silva, Rayane Leite da Silva, Lucas Marcio Pena-Pereira, Marcos Nisihara Chagas,
Universidade Federal de São Paulo. São Paulo SP,
Nascimento Monteiro, Deryc Cleyner Piones Bastos, Carlos Maurício de Almeida, Regina Fernandes, Vitor
Brazil
Max Luiz Mendes Ramires Filho, Tiago Gomes de Tumas, Alan Eckeli
Andrade, Lívia Leite Goes Gitaí, Flávio Teles de carlacarletti28@yahoo.com.br
Farias Filho Universidade Federal do Ceará. Fortaleza CE, Brazil
Universidade de São Paulo. Ribeirão Preto SP, Brazil
Background: Sleep plays an important role for the con-
Universidade Federal de Alagoas. Maceió AL, Brazil USP
struction of the development of children, children and
caroline.resende@famed.ufal.br adolescents, having a great influence on the develop- manoelsobreira@yahoo.com.br
ment of this population, affective and social. Objective:
Background: Chronodisruption refers to the chronic dis- The objective of this study was to evaluate sleep and Background: Restless legs syndrome or Willis-Ekbom
ruption of circadian rhythms leading to disease. Alterations quality of school life in a public school in the city of São disease is a disorder characterized by unpleasant sensa-
of circadian rhythms have been observed in CKD patients Paulo. Method: Sleep assessment was performed using tions associated with the need to mobilize the lower limbs.
and there is accumulating evidence that some of them may the Sleep Disturbance Scale for Children (SDSC) with 26 In Parkinson disease patients, restless legs syndrome is
adversely affect health. Social jet lag is a term describing items, divided into 6 subscales: Disorders of initiating and associated with worse quality of life and excessive sleep-
misalignment between social and biological times con- Maintaining Sleep (DIMS), Sleep-disordered breathing iness. Regarding other factors, results of different studies
ventionally opposing work days to free days. CKD patients (SDB), disorders of arousal (DA), Sleep–wake transition are controversial. Objective: To determine the factors
in hemodialysis are distributed into treatment shifts that disorders (SWTD), Disorders of excessive somnolence associated with the restless legs syndrome presence in
configure a commitment that occurs three times a week. (DOES) and sleep hyperhydrosis (SHY). The SDSC was Parkinson disease patients. Methods: A cross-sectional
Objective: The current study aimed to investigate if there answered by the parents. The quality of life assessment was study was conducted in 88 consecutive Parkinson dis-
is a hemodialysis-related social Jet Lag, to assess chrono- performed using the Pediatric Quality of Life Inventory ease patients from the outpatient clinic for 21 months.
type in hemodialysis patients and if there is inconsistency (PedsQL), by both children and parents. Results: The Participants underwent a clinical interview, assessment
between the chronotype and the dialysis shift. Methods: SDSC was answered by the parents of 22 5-year-old chil- based on standardized scales (Epworth Sleepiness Scale,
A total of 194 patients undergoing hemodialysis from dren, 16 boys (73%) and 6 girls (27%). The average score Parkinson Disease Questionnaire – 39, Pittsburgh Sleep
three local dialysis units were enrolled from February to on SDSC was 45,59±11,86.The DIMS had a higher mean Quality Index, International Restless Legs Syndrome
June 2022. Chronotype was estimated using Morning- (13.27±2.95) when compared to the other subscales (SDB: Study Group rating scale), and video-polysomnography.
Eveningness Questionnaire (MEQ) and a modified version 4.72±2.69; DA: 4.22±1.59; SWTD: 10.72±3, 23; DOES: Results: Out of the 88 participants, 25 had restless legs
of Munich Chronotype Questionnaire (MCQT) was used 8.54±2.69 and SHY: 4.09±2.31. Parents and 17 children, 13 syndrome. In the multivariate analysis, restless legs syn-
to estimate hemodialysis-related social Jet Lag. Results: boys (76%) and 4 girls (24%) responded to the PedsQL™. drome in Parkinson disease has been associated with the
Hemodialysis related jet lag was 48.1 ± 1.4 min (range There was no difference between the total quality of life symptom of smell loss and quality of sleep and life. In the
0 – 4: 57) and was inversely correlated to age (r=-0.216, score between the children’s self-report and the parents’ univariate analysis, restless legs syndrome in Parkinson
p=0.01). Chronotype was distributed as 2.1% moderately perception (76.89±16.30 Vs. 84.84±7.78; p=0.051). Only in disease has occurred more frequently in women with
evening, 31.4% intermediate, 39.2% moderately morn- the physical capacity domain of the PedsQL™ there was a higher frequency of insomnia, constipation, and anos-
ing and 27.3% definitely morning. Forty-four patients difference between the children’s self-report compared mia than in the group without restless legs syndrome.
(22.7%) were in morning shift treatment, 96 (49.5%) in to the parents’ perception (84.76±13.98 Vs. 95.21±4.56; Conclusion: Restless legs syndrome is a prevalent con-
afternoon shift and 54 (27.8%) in night shift treatment. p<0.001). Discussion: We observed that the schoolchil- dition in patients with Parkinson disease and is associ-
Inconsistency between shift treatment and chronotype dren’s sleep was compromised by the mean score on the ated with specific characteristics in this group of patients.
was detected in 38 (19.6%) and was related to higher MEQ SDSC. DIMS are frequent complaints, as it is a transition
scores (p<0.001). Conclusion: Therefore, this study seems period in which children prepare to experience the school
to indicate that there may be a hemodialysis-related social routine, presenting anxious behaviors and, consequently, Sono
jet lag, that morning chronotype is the most frequent in impairing the quality of their sleep. Despite this finding,
hemodialysis patients and higher score in MEQ is related there was no impairment in the quality of life of the chil-
to inconsistency between shift treatment and chronotype. dren evaluated. In the physical capacity domain, the chil-
dren’s self-report score was lower than the parents’ score.
Studies show that the information provided by parents
Sono and/or guardians is not always equivalent to that reported
by children. Conclusion: The children evaluated showed
impaired sleep, with DIMS being the most prevalent. The
child’s self-report about their health is equivalent to the
perception of parents/guardians.

Sono

364
XXX Congresso Brasileiro de Neurologia

TL 1106649 TL 1106653 TL 1106657

SLEEP DISORDERS IN CONGENITAL ZIKA TRANSLATION, TRANSCULTURAL SLEEP MEDICINE ON INSTAGRAM: FASHION
VÍRUS SYNDROME ADAPTATION, AND VALIDATION OF THE DANCES AND NONSENSE
BRAZILIAN PORTUGUESE VERSION OF THE
Mariana Braatz Krueger, Alissa Elen Formiga Moura, Beatriz Astun Giuberti, Karla Carlos, Gilmar
Danielle Mesquita, Samir Camara Magalhães, OBSTRUCTIVE SLEEP APNEA KNOWLEDGE Fernandes Prado
André Pessoa, Patrícia Gomes Damasceno, Marcelo AND ATTITUDES (OSAKA) QUESTIONNAIRE
Rodrigues Masruha, Manoel Alves Sobreira-Neto Escola Paulista de Medicina – UNIFESP
Camila Ferreira Leite, Francisca Soraya Lima Silva,
Universidade Federal de São Paulo. Fortaleza CE, Manoel Alves Sobreira-Neto beatrizastungg@gmail.com
Brazil
Universidade Federal do Ceará. Fortaleza CE, Brazil Background: With the advancement of social networks we
Universidade Federal do Ceará. Fortaleza CE, Brazil
Hospital Infantil Albert Sabin. Fortaleza CE, Brazil camilafleite@ufc.br have a new profession: the “digital influencer”. No formal
Universidade Federal de São Paulo. São Paulo SP, training or specializations are required, just communica-
Brazil tion skills and understanding of the target audience. In the
The knowledge and attitude of physicians regarding the
context of the digital world, it is very common for health
identification and treatment of patients with obstruc-
mari_bk@hotmail.com professionals to abandon their technical behavior based
tive sleep apnea can be assessed through the OSAKA
upon science knowledge, adopting a common language
(Obstructive Sleep Apnea Knowledge and Attitudes)
Background: Congenital Zika virus syndrome (CZVS) and a banal attitude, associating themselves with their
questionnaire. This questionnaire was developed in 2003,
sleep characteristics in patients are still poorly stud- target audience. Objective: The aim of this study was to
and the University of Washington holds the intellectual
ied, with only one study in which the Brief Infant Sleep analyze the Instagram profiles of sleep medicine profes-
property of the instrument. In Brazil, the current literature
Questionnaire (BISQ) was carried out, that shows that sionals. Method: We did an Instagram search using only
does not present any validated instrument that specifi-
34.1% of CZVS were defined as poor sleepers. No previous the word “sleep”. We access the searched accounts and
cally assesses the medical knowledge of OSA. Objective:
study performed electrophysiological assessment of sleep. only analyzed professional profiles. Results: We found
This study aimed to analyze the psychometric properties
Objective: The aim of this study was to clinically charac- 78 professional profiles. Of these, 12 otolaryngologists,
of the translated and cross-culturally adapted version of
terize sleep disorders in a case series of CZVS patients, 12 physiotherapists, 10 sleep consultants, 9 psycholo-
the OSAKA (Obstructive Sleep Apnea Knowledge and
correlating these findings with disease features and poly- gists, 8 neurologists, 5 pulmonologists, 3 psychiatrists, 3
Attitudes) questionnaire in the brazilian Portuguese lan-
somnographic (PSG) results. Methods: This is a non-ran- pediatricians, 2 dentists, 2 sleep educators, 1 sleep biol-
guage. Methods: The OSAKA instrument was translated
domized, cross-sectional study with consecutive inclu- ogist/consultant, 1 biomedical doctor, 1 cardiologist, 1
by two independent translators, and the back-trans-
sion of confirmed CZVS. The evaluation was performed scientist, 1 doula, 1 nurse, 1 coach, 1 speech therapist,
lated conciliated version was presented and approved
with PSG and the characteristics of sleep were evaluated 1 mentor/sleep therapist, 1 multiprofessional group, 1
by Washington University, which holds the intellectual
using Sleep Disturbance Scale for Children (SDSC) and psychoanalyst and 1 sleep specialist. Fifty-eight profiles
property for the OSAKA questionnaire. A total of 176 phy-
BISQ. Results: Ten patients were evaluated, with a mean present a marketing pattern focused on their professional
sicians from different specialties electronically completed
age of 4,6 years, seven male (7/10). Eighty percent of the performance; 17 profiles focused on diseases; 13 advertise
the OSAKA instrument and the ASKME (Assessment of
patients present a SDSC score more than 39, therefore, their courses and 3 profiles their mentorships, 1 profile a
Sleep Knowledge in Medical Education) questionnaire,
demonstrate sleep disturbances and 20% clinically rele- webinar and 1 profile a podcast. The most frequent publi-
which was used as an auxiliary instrument to analyse the
vant. The BISQ sleep pattern analysis showed that 10% cations addressed were: 42% sleep in general, 26% infant
construct validity. Results: The items from the knowledge
of children were defined as poor sleepers, 10% had more sleep, 13% apnea and CPAP, 8% insomnia and 5% snor-
and attitudes domains presented acceptable internal con-
than 3 wakes per night and 80% had nocturnal wakefulness ing. Regarding the content of the publications, 44 profiles
sistency values, with McDonald’s omega coefficients (?)
period more than 1 hour. Polysomnography evaluation: (56%) have an informative/technical pattern, 19 (24%)
of 0.70 and 0.73, respectively. The OSAKA questionnaire
Two patients were not evaluated in the polysomnography have an informative/sensitizing pattern and 20 profiles
showed a moderate correlation with the ASKME instru-
due to technical difficulties (exam duration and was no (26%) have a sensitizing content. Considering the quality
ment (r = 0.60, p < 0.001) and excellent retest reliability,
signal capture in the nasal cannula). Mild sleep apnea of the posts, 68% have a homemade pattern and 33% have
with an intraclass correlation coefficient of 0.81. There
was detected in 5 patients and moderate in 2 patients, of a professional pattern. Conclusion: Advertising in the
were differences in knowledge between the medical spe-
these 2 with central and obstructive apnea. Mild desat- area of sleep medicine involves a heterogeneous group
cialties (p < 0.001). Regarding attitudes, most respondents
uration in 5 patients and severe in 1 patient. Increase in of people and professionals. Most of them, without the
considered obstructive sleep apnoea and its diagnosis to
sleep latency was documented in 5 children, normal in proper formal training required by the area, in addition
be important and felt confident in identifying it, but the
2 and reduction in 1. Presence of periodic movement of to offering treatments, offer courses always aiming at a
same majority did not feel confident in treating the dis-
members, with a PLM index of 29.8/h, in one patient. All good financial return. The way they present themselves is
ease. Conclusion: The OSAKA instrument, as a translated
showed reduced REM sleep, which was not detected in 1 typical of current times when they are equal in expressions
and cross-culturally adapted brazilian Portuguese ver-
patient. Conclusion: The frequency of sleep disorders in to young influencers, centered on their own opinions and
sion, presented psychometric properties with adequate
the population of children with confirmed CZVS is very not on traditional technical-scientific standards. In addi-
reliability and validity.
high, being important the evaluation through polysom- tion, they refer to themselves with a varied and unofficial
nography in this population. titles and measure their value by the number of followers
Sono and interaction with the public.

Sono
Sono

365
XXX Congresso Brasileiro de Neurologia

TL 1104998 TL 1105051 TL 1105085

EFFECTS OF TELEHEALTH ON SLEEP AND FACTORS THAT NEGATIVELY IMPACT THE INDICATORS OF TELEMEDICINE FEASIBILITY
PHYSICAL ACTIVITY LEVEL IN PATIENTS SEXUAL FUNCTION OF MEN LIVING WITH IN PARKINSON’S DISEASE PATIENTS
WITH PARKINSON’S DISEASE DURING THE PARKINSON’S DISEASE DURING THE COVID-19 PANDEMIC
PANDEMIC
Bruno Rafael Antunes de Souza, Kátia Cirilo Costa Gustavo Neves Pinto, Brenda Matheus Nogueira de
Letícia Brasil Gradvohl, Brenda Matheus Nogueira Nóbrega, Bruno Eron Almeida da Silva, Raíssa Lima, Camile Feijó de Andrade, Maria Eduarda Pinto
de Lima, Miriam Lindsay Silva Marques, Maria Amorim Gonçalves, Maria Elisa Pimentel Piemonte Marinheiro, Gabrielle Brito Bezerra Mendes, Letícia
Eduarda Pinto Marinheiro, Maria Luiza Miranda Brasil Gradvohl, Miriam Lindsay Silva Marques,
Universidade de São Paulo/USP Vlademir Carneiro Gomes, Pedro Braga Neto,
Carneiro, Arthur Holanda Moreira, Nara Fontenele
Universidade Federal do Amapá – Macapá – AP – Danielle Pessoa Lima
Dias Porto, Vlademir Carneiro Gomes, Pedro Braga
Brazil
Neto, Danielle Pessoa Lima
Universidade de Fortaleza. Fortaleza CE, Brazil
bruno_souza@usp.br Universidade Federal do Ceará. Fortaleza CE, Brazil
Universidade de Fortaleza. Fortaleza CE, Brazil
Universidade Federal do Ceará. Fortaleza CE, Brazil Universidade Federal do Ceará; Universidade
Background: Among the non-motor alteration associ- Estadual do Ceará. Fortaleza CE, Brazil
Universidade Estadual do Ceará. Fortaleza CE,
ated with Parkinson’s disease, sexual dysfunction is less
Brazil
discussed and, consequently, less known. Satisfactory gustavoneves@edu.unifor.br
gustavoneves@edu.unifor.br sexual function has been associated with better quality
of life even in elderlies. Therefore, it is imperative to know Background: During the COVID-19 pandemic, access
Background: Telemedicine allows Parkinson’s Disease which motor and non-motor alterations negatively affect to health services was limited by government measures
(PD) patients to overcome physical barriers of access to sexual function in order to find new approaches to improve to combat the spread of the virus. Consequently, care for
health care services and increases accessibility for people this vital function. Objective: To investigate the impact of other diseases was impaired, such as Parkinson’s disease
with mobility impairments. Studies have shown similar motor, non-motor function, and relationship quality on (PD). Telemedicine was adopted as a way to make health
effectiveness of virtual consultations versus face-to-face sexual function in men living with PD. Methods: Thirty- care viable in the face of the lockdown state. Objective: To
appointments for the management of PD symptoms. three men with a confirmed diagnosis of Idiopathic PD assess feasibility indicators of a telehealth intervention for
Objective: This study aims to evaluate the feasibility for 6.69 years (SD 4.39); mean age of 54 years (SD=9.98), patients with PD, during the pandemic isolation period
of telehealth activity in the follow-up of patients with in stage 1 – 3 of disease evolution according to Hoehn in 2020. Methods: The sample consisted of 107 patients
PD, focusing on its effects on sleep and physical activity and Yahr classification, without dementia, participated in from the Movement Disorders outpatient clinic of a ter-
level. Methods: This study was performed at a Neurology the present study. After presenting the informed consent tiary Hospital from May 1 to December 31, 2020. Eligibility
Outpatient Clinic in Fortaleza, from May 1st to December form and expressing agreement to participate in the study, criteria were: diagnosis of PD, face-to-face consultation
31st, 2020. Inclusion criteria were PD diagnosis and con- participants were asked to answer, through telephone in the previous 12 months, access to necessary commu-
sultation in the clinic in the last 12 months and exclusion interviews, a previously established questionnaire that nication and caregiver availability in cases of dementia.
criteria were non-adherence to consultations and lack of investigates: (1) general information; (2) socio-economic If eligible, they were invited to attend a medical appoint-
technology for communication. All participants signed status; (3) information associated with PD; (4) mental and ment on WhatsApp© video calls. Recruitment, attendance,
a consent form. During the study, patients participated cognitive health; (5) motor and non-motor aspects of technical problems and acceptance rates were calculated
in a teleconsultation, answered to 3 questionnaires- daily life experience assessed by sections I and II of MDS- as indicators of feasibility. Acceptance was assessed by a
Pittsburgh PSQI Quality Index; Epworth Sleepiness Scale; UPDRS; (6) International Index of Erectile Function (IIEF), questionnaire with 10 questions about: feasibility, sense
and International Physical Activity Questionnaire- Short and Male Sexual Quotient (MSQ); (7) Dyadic Adjustment of security, satisfaction, effectiveness and future use of
Form (IPAQ-SF)- and received health education advice, Scale (DAS). The statistical analysis tested the correlation the intervention, with a visual analogue scale. Results:
such as on healthy eating, sleep hygiene, fall prevention between total scores in the IIEF, MSQ and age, disease We had 61.3% recruitment and 90.7% attendance rates.
and encouragement on doing physical activity at least evolution, I -II MDS-UPDRS scores and DAS. Results: Physicians perceived technical difficulties in 11.2% of con-
30 minutes 3 times per week. After 15 days, calls were There was no correlation between age, disease evolution, sultations. The low recruitment rate was mainly due to the
made to reinforce the recommendations and, after 45 the severity of non-motor alterations, relationship quality, high proportion of patients who changed their telephone
days, the 3 questionnaires were reapplied. Results: A and sexual function. However, there was a statistically sig- number and could not be reached. The inability of outpa-
total of 107 patients participated, 58.9% were male. The nificant correlation between MDS-UPDRS section II and tient clinics to obtain registration data, the unavailability
mean age was 65 and mean disease duration was 10 years. sexual function (R=-.50; p<.01). Significant correlations of technology and the absence of a caregiver were other
8.6% of the patients were attending physical therapy ses- can be observed in multi-domains of sexual function. problems found. Acceptance, in general, was higher than
sions; 25.7% were engaged in physical activity at least 30 Conclusion: For men living with PD, the disability level 80%, with the exception of a question about effectiveness
min 3 times per week; and 36.5% needed walking aids. expressed by motor experiences of daily living is the main in improving the symptoms of the disease, which had an
Perceived functional decline and worsening parkinso- factor for the quality of sexual function. Then, interven- average rate of 75%, suggesting that a single consultation
nian symptoms during the pandemic were reported for tions to improve motor function and reduce disability in is insufficient to resolve all patient complaints, espe-
32.7% of the patients. Baseline IPAQ-SF classification was daily living activities may help the men living with PD cially those that require a multidisciplinary approach.
as follows: 41.4% sedentary; 10.3% insufficiently active A; reach or keep a satisfactory sexual function. Participants were referred to rehabilitation services, but
9.2% insufficiently active B; 34.5% active; and 4.6% very in most cases there was no attendance due to the few
active. There were improvements in three PSQI com- public rehabilitation centers available. Conclusion: This
ponents: perceived sleep duration, sleep disorders and Transtornos do Movimento is probably the first study to assess the feasibility of tele-
daytime sleep disturbance with an average of 1.7, 2.3 and medicine on WhatsApp© in the care of patients with PD
1.7 respectively. As for the level of physical activity, there in the brazilian public health system. The results showed
was an increase in the number of days of walking and the good acceptance and attendance rates for the strategy,
number of days of moderate activities for at least 10 con- but more studies with a control group and intervention
tinuous minutes. Conclusion: This telehealth interven- effect outcomes are needed.
tion promoted positive effects on sleep and on physical
activity level of PD patients and may have greater benefits
in longitudinal application. Transtornos do Movimento

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XXX Congresso Brasileiro de Neurologia

TL 1105107 TL 1105145 TL 1105236

APPLICATION OF SARC-F TO SARCOPENIA DESCRIPTION OF CLINICAL CONDITIONS, ANALYSIS OF UPPER LIMB STRENGTH IN
SCREENING IN PATIENTS WITH PHYSICAL ACTIVITY LEVEL, DAYTIME PATIENTS WITH PARKINSON’S DISEASE: A
PARKINSON’S DISEASE IN A SPECIALIZED SLEEPINESS AND SLEEP QUALITY IN DESCRIPTIVE CROSS-SECTIONAL STUDY
OUTPATIENT CLINIC IN FORTALEZA PATIENTS WITH PD DURING THE COVID-19
Luísa Bedê Bruno, Gabrielle Brito Bezerra Mendes,
PANDEMIC Sofia Gomes Correia, Camila Ximenes Feitosa,
Gabrielle Brito Bezerra Mendes, Beatriz Rodrigues
Neri, Cicero Gilmário Alves Pereira de Lima Filho, Arthur Holanda Moreira, Gustavo Neves Pinto, Cícero Gilmário Alves Pereira de Lima Filho, Vitória
Sofia Gomes Correia, Luísa Bedê Bruno, Elisa Camile Feijó de Andrade, Gabrielle Brito Bezerra Maria Torres Peixoto, Beatriz Rodrigues Neri,
Tavares Diogo de Siqueira, José Ítalo da Silva Mendes, Nara Fontenele Dias Porto, Maria Eduarda Danielle Pessoa Lima, Pedro Braga Neto, Vlademir
Damasceno Gomes Gomes, Maria Luiza Miranda Pinto Marinheiro, Maria Luiza Miranda Carneiro, Carneiro Gomes
Carneiro, Pedro Braga Neto, Danielle Pessoa Lima Vlademir Carneiro Gomes, Pedro Braga Neto, Universidade de Fortaleza. Fortaleza CE, Brazil
Danielle Pessoa Lima Universidade Estadual do Ceará. Fortaleza CE,
Universidade de Fortaleza. Fortaleza CE, Brazil
Universidade Estadual do Ceará. Fortaleza CE, Universidade de Fortaleza. Fortaleza CE, Brazil Brazil
Brazil Universidade Federal do Ceará. Fortaleza CE, Brazil UECE, Universidade Federal do Ceará. Fortaleza CE,
Universidade Federal do Ceará. Fortaleza CE, Brazil Universidade Federal do Ceará; Universidade Brazil
Estadual do Ceará. Fortaleza CE, Brazil Universidade Federal do Ceará. Fortaleza CE, Brazil
gabriellemendes04@edu.unifor.br
gustavoneves@edu.unifor.br gabriellemendes04@edu.unifor.br
Background: Parkinson’s disease (PD) is characterized
by progressive and disabling neurodegeneration. Among Background: Many patients with Parkinson’s disease Background: Parkinson’s disease (PD) is a neurode-
the disorders present in PD, musculoskeletal alterations, (PD) had health care impaired due to physical barriers generative disorder caused by disruption of the dopa-
such as sarcopenia, added to the decline in functional and sanitary conditions imposed by the Government to minergic system. The muscle weakness is one of the
capacity, can favor fall events and reduced functionality contain the virus spread. As a result, there were reports main complaints reported by patients with PD, given the
and quality of life of PD patients. Objective: Describe data of worsening of motor and non-motor symptoms in impacts on day-to-day activities. The clinical diagnosis
obtained in screening for sarcopenia in patients with PD. these patients during the pandemic period. Objective: of PD is based primarily on motor features, that usually
Methods: A descriptive observational study was carried Describe clinical conditions, physical activity level, day- starts in the upper limbs. The involvement of handgrip
out, applying the SARC-F Questionnaire in 125 patients time sleepiness and sleep quality in PD outpatients Tertiary strength can be considered as an intrinsic characteristic
with PD in a specialized outpatient clinic of a tertiary Hospital Movement Disorders during covid-19 pan- of PD, besides being correlated with the motor symptom
Hospital in Fortaleza. The questionnaire evaluates the demic. Methods: The study was carried out in the period severity of the disorder. Objective: Correlate upper limb
patient’s perception of their limitations in the following from May 1st to December 31st, 2020. Eligibility criteria strength to the disability in patients with PD. Method:
conditions: strength to lift and carry 4.5 kg; ability to walk included diagnosis of PD and face-to-face attendance in Descriptive cross-sectional study with PD patients from
around a room; get up from a chair; climb a flight of stairs the previous 12 months and the criteria for exclusion were the Movement Disorders outpatient clinic of a tertiary
and the number of falls in the last year. Results greater than non-adherence to consultations and lack of technology Hospital carried out from September/2020 to May/2022.
or equal to 4 mean probable sarcopenia. Only patients with for communication, unavailability of the caregiver in Demographic, clinical data and upper limb strength mea-
Hoehn & Yahr (HY) 1 to 3 were included and patients with case of patient with dementia. During the study, patients surements were collected using the SAEHAN handgrip test
any other disabling diseases were excluded. Results: A participated in a teleconsultation and responded to 3 with dynamometer according to the Southampton pro-
total of 125 patients were analyzed, 72 (57.6%) male, with questionnaires: Pittsburgh Sleep Quality Index (PSQI), tocol and the cutoff suggested by the Revised European
a mean age of 66.16 years. Of the total, the vast majority Epworth Sleepiness Scale (ESS) and International Physical Consensus on Sarcopenia (2019) of <27kg for men and
(42.4%) had HY of 2.5. Furthermore, 64 (51.2%) scored Activity Short Form Questionnaire (IPAQ-SF). Results: <16kg for women as determinants of weakness. PD stag-
SARC-F positive, of which 33 (51.6%) were female and 107 patients participated, being 58.9% male, with a mean ing was assessed using the modified Hoehn&Yahr (HY)
31 (48.4%) were male, and 61 (48.8%) scored negative, of age of 65.7 (± 17.1) years and mean duration of disease of scale and functionality using the Schwab-England (SE)
which 20 (33.3%) were female and 40 (66.7%) were male. 10 (± 6.7) years. The most comorbidities prevalent were scale. Eligibility criteria were mild to moderate PD (HY
Moreover, among the patients with positive SARC-F, 19 arterial hypertension (44.9%); depression (32.7%); Type 2 1-3), no other neurological diseases with motor impair-
(29.7%) used some walking aid and 45 (70.3%) did not. diabetes (24.3%); dyslipidemia (23.4%) and osteoporosis ment, no chronic diseases with acute instability and no
Among the patients with negative SARC-F, 7 (11.5%) used (15%). In the IPAQ-SF there was 41.4% sedentary; 10.3% health conditions that compromise mobility. Results:
some walking aid and 54 (88.5%) did not. Finally, among insufficiently active-A; 9.2% insufficiently active-B; 34.5% The sample consisted of 125 patients, of which 72 (57.6%)
the total number of patients, 52 (41.6%) practiced physical active and 4.6% very active; that is, 51.7% of the sample were men and 53 (42.4%), women; 56.8% had a family
activity for at least 30 minutes 3 times a week, of which with a significant reduction in physical activity. The PSQI history of PD, 53.6% had dyskinesia, 43.2% reported FOG,
16 (30.8%) had positive SARC-F and 36 (69.2%) negative, and ESS results were 12.1(± 3.6) and 11.2 (± 5.9), respec- 20.8% needed walking aids and 41.6% were physically
and 73 (58.4%) did not practice physical activity, of which tively, showing poor sleep quality and increased daytime active. The comorbidities were: SAH (47.2%), depression
48 (65.8%) had positive SARC-F and 25 (34.2%) negative. sleepiness. Conclusion: During the pandemic, the level (17%), PLD (16%) and DM2 (11.2%). Twenty-five patients
Conclusion: There is still few data in the literature that dis- of physical activity was unsatisfactory, with a predomi- (20%) had reduced palm strength, with an average age
criminate regarding the difference of sarcopenia screening nance of a sedentary lifestyle, as well as poor sleep quality of 64.4 years in the group with normal examination and
in patients with PD, making more studies necessary for and daytime sleepiness. It is not possible to infer whether 71.1 years in the group with alterations. Of these, 12
an adequacy of this analysis for this profile of patients. the quarantine has aggravated pre-existing conditions or were women and 13, men; 12 patients (48%) had SAH, 2
caused these problems, since it is a cross-sectional study, (8%) DM2, 3 (12%) PLD, and 9 (36%) depression. In the
but the results are worrying because they represent a group with weakness in the upper limbs, the prevalent
Transtornos do Movimento greater risk of sarcopenia, falls, functional impairment HY was 3, in 13 patients (52%); 3 (12%) had HY=2 and 9
and worsening of quality of life. (36%) had HY=2.5. Conclusion: Study carried out with
brazilian elderly had 28.2% dynapenic, compared to the
20% recorded in this analysis in patients with PD and a
Transtornos do Movimento mean age of 71.1 years. Despite the correlation between
the decreased upper limb strength in PD and a greater
impairment of manual dexterity, it is important that fur-
ther studies are carried out in this area.

Transtornos do Movimento

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XXX Congresso Brasileiro de Neurologia

TL 1105244 TL 1105410 TL 1105452

ANTHROPOMETRIC PROFILE OF PARKINSON’S DISEASE IS RELATED TO HIGH A STUDY OF THE ASSOCIATION BETWEEN
PARKINSONIANS FOLLOWED UP AT A EXPOSURE TO HOUSEHOLD INSECTICIDES RELIGIOSITY AND DEPRESSION IN PATIENTS
REFERENCE CENTER IN FORTALEZA – CE IN BELÉM, PARÁ WITH PARKINSON’S DISEASE
Cícero Gilmário Alves Pereira de Lima Filho, Luísa Dafne Dalledone Moura, Vanderci Borges, Henrique Carlos Eduardo Cordeiro Cavalcante, Matheus
Bedê Bruno, Gabrielle Brito Bezerra Mendes, José Ballalai Ferraz, Ignacio F Mata, Cyrus P Zabetian, Rodrigues Corrêa, Kelson James Almeida
Ítalo da Silva Damasceno Gomes, Vitória Maria Vitor Tumas, Bruno Lopes Santos-Lobato
Torres Peixoto, Camila Ximenes Feitosa, Pedro Universidade Federal do Piauí. Teresina PI, Brazil
Braga Neto, Danielle Pessoa Lima, Pauliana Alencar Laboratório de Neuropatologia Experimental,
Universidade Federal do Pará. Belém PA, Brazil matheuscorrea990@gmail.com
Monteiro, Elisa Tavares Diogo de Siqueira
Departamento de Neurologia, Universidade Federal
Universidade Estadual do Ceará. Fortaleza CE, de São Paulo Background: Neuropsychiatric comorbidities are com-
Brazil Lerner Research Institute, Cleveland Clinic – mon in Parkinson’s Disease (PD) and impact patients’
Universidade de Fortaleza. Fortaleza CE, Brazil Estados Unidos quality of life. In parallel, religiosity is associated with the
Universidade Federal do Ceará. Fortaleza CE, Brazil Department of Neurology, University of Washington improvement of depressive and anxious symptoms in the
– Estados Unidos general population. However, in other countries, the rates
gabriellemendes04@edu.unifor.br Faculdade de Medicina de Ribeirão Preto, of religiosity among people with PD reported significantly
Universidade de São Paulo. Ribeirão Preto SP, Brazil lower levels of religiousness than did age-matched con-
Background: Parkinson’s disease (PD) is a progressive trols. That possibly happens because, in addition to the
neurodegenerative disorder. PD is known as a move- daf.dalledone@gmail.com motor impairment that limits the experience of religious
ment disorder with three cardinal signs: tremor, rigidity activities, depression, the main neuropsychiatric comor-
and bradykinesia. Weight changes and decreased muscle Background: Parkinson’s Disease (PD) is a neurode- bidity, may influence the religious practices and beliefs
strength are also common in patients with PD. Describing generative disease that does not have a known cause of these patients. Objectives: To assess the level of reli-
anthropometric measurements and Body Mass Index but is strongly affected by environmental factors for its giosity in patients with PD and verify an association with
(BMI) in patients with Parkinson’s disease can improve evolution, such as the extended exposure to household depression and quality of life indices. Methods: Patients
interventions aimed at this public. Objective: The aim is insecticides (HI). The HI, such as the pyrethroids, are with PD diagnosed according to the 2015 MDS criteria
describe the anthropometrics measurements of patients used in residences for insect control, especially mos- were included in this study. We excluded cases with the
with Parkinson’s Disease in a reference center in Ceará, quitos carrying arboviruses. Objective: To evaluate the hypothesis of atypical parkinsonism. Religiosity was
Brazil. Method: this study has a cross-sectional and association between exposure to HI and the development assessed using the Duke University Religiosity Index, with
descriptive design. A sample of 125 eligible participants of PD in three brazilian cities. Methods: This is a clinical, 3 components: organizational religiosity (OR), non-orga-
were included in the study, after signing the consent form. observational, and case-control study. Participants were nizational religiosity (NOR) and intrinsic religiosity (IR).
Data collection took place at the Walter Cantídio University enrolled from three cities: Belém/PA (BEL), Ribeirão Preto/ Beck Depression Index (BDI) and PDQ 39 evaluated
Hospital, in Fortaleza, Ceará, Brazil, from February 8, 2021 SP (RP), and São Paulo/SP (SP), and were submitted to depression and quality of life indices, respectively. The
to April 18, 2022. The inclusion criteria were: having a the survey “Latin American Research Consortium on cutoff point for moderate depressive symptoms was BDI
diagnosis of Parkinson’s Disease according to the English the Genetics of Parkinson’s Disease” (LARGE-PD). The > 19. The Spearman test was used to assess the correlation
Brain Bank diagnostic criteria; Age >40 years; stages 1, statistical analysis was done with the Chi-square test and between the variables. This study obtained approval from
2, and 3 of the Hoehn and Yahr scale. Participants with Mann-Whitney test. Results: 373 patients with PD (BEL: the local ethics committee approval. Results: Twenty-
dementia syndrome, according to MSD-V; chronic dis- 134; RP: 150; SP: 89) and 215 controls (BEL: 94; RP: 97; eight patients were included, among which 3 had mod-
eases with acute exacerbation; active cancer or infection SP: 24) were assessed. The median age of patients and erate depressive symptoms. The correlation coefficient
were excluded. The participants’s data were included in the controls was 59 and 53 years old, respectively. In BEL, between OR/NOR with the other variables was 0.308 for
Research Electronic Data Capture (REDCap) platform, in the exposure to HI between patients (73,8%) and controls BDI (p=0.111); 0.218 for PDQ 39 (p=0.264) and 0.243 for
compliance with the data protection law. Results: 72 men (70,2%) was more common than in RP and SP (p<0,01; UPDRS III (p=0.212). As for the relationship between
(57.6%) and 53 women (42.4%) with Parkinson’s disease RP – patients: 48%; controls: 51,5%; SP – patients: 35,9%; RI and the other scales, the value found was -0.348 for
made up the sample, with a mean age of 65 years. Most controls: 37,5%), but there were no differences in the BDI (p=0.069); -0.443 for PDQ 39 (p=0.018) and -0.407
patients were married (69.6%), did not practice physical exposure to HI between patients and controls in any of for UPDRS III (p=0.032). Furthermore, the relationships
activity (58.4%), did not use alcohol (88%) and did not the cities. In BEL, the majority of individuals had high between PDQ 39 and IDB (0.513; p=0.005) and PDQ 39 and
smoke (97.6%). Comorbidities included hypertension exposure to HI (exposure > 30 days/year) (patients: 69,6%; UPDRS III (0.614; p<0.001) were analyzed. Conclusion:
(47.2%), dyslipidemia (16%), depression (13.6%), diabetes controls: 42,4%; p<0,0001) compared with the other cit- Only IR showed a significant correlation with PDQ 39 and
(11.2%), osteoporosis (8%) and generalized anxiety disor- ies (high exposure: RP – patients: 15,2%, controls: 10%, UPDRS III. Intrinsic religiosity was weakly associated with
der (7.2%). Regarding BMI, 51 patients were overweight p=0,56; SP – patients: 31,2%, controls: 3,3%, p = 0,77), and better levels of quality of life and less motor impairment.
(men, 31 [44.9%]; women, 20 [38.5%]), 40 were normal patients with PD had greater proportion of high exposure Although not significant, a trend towards fewer depressive
weight (men, 23 [33.3%]; women, 17 [32.7%]), 17 were to HI, compared with controls (p = 0,001). The overall symptoms was observed in patients with a higher degree
underweight (men, 9 [13%]; women, 8 [15.4%]) and 13 median exposure time was 15 years, with no differences of intrinsic religiosity. There was also a significant associa-
were obese (men, 6 [8.7%] and women, 7 [13.5%]). BMI of between patients and controls. Conclusion: In BEL, an tion between quality of life with a lower degree of depres-
four patients was absent. Calf circumference was altered Amazon brazilian city with major exposure to HI, there sive symptoms and a lower degree of motor impairment.
(<31cm) in 25 patients (20%). The Waist/Hip Ratio was was an association between high exposure to HI and the
altered in 48 patients (men, 40 [56%]; women, 8 [15%]), development of PD. High exposure to HI in BEL might
with missing data in one participant. Conclusion: these be related to the Amazon environment, which promotes Transtornos do Movimento
measures may highlight the need for greater attention higher insect proliferation. Despite the weakness of the
from the health system in relation to the treatment and study (selection bias, memory bias, small sample, ret-
monitoring of patients with Parkinson’s disease to improve rospective study), these results provide support to new
their quality of life. studies that explore the association between PD and HI,
mainly in less developed areas of the world.

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Transtornos do Movimento

368
XXX Congresso Brasileiro de Neurologia

TL 1105471 TL 1105487 TL 1105648

CLINICAL AND GENETIC ANALYSIS OF A GENETIC ANALYSIS OF A COHORT OF ASSESSMENT OF UPPER LIMBS
POPULATION WITH HUNTINGTON’S DISEASE IDIOPATHIC PARKINSON’S DISEASE IN IMPAIRMENTS IN PARKINSON’S DISEASE: A
IN AMAZONAS AMAZONAS SYSTEMATIC REVIEW
Marcus Vinicius Della Coletta, Giselle Benevides Maria Eduarda Alencar Santos, Deise Andrade Melo, Rubia Rodrigues, Beatriz Santos, Rubens G Cury,
Ferreira, Diana V. Brito, Cleiton Fantin, Deise Sandro Adriano Souza Lima Junior, Diana V. Brito, Egberto R Barbosa, Tamine Capato
Andrade Melo, Sandro Adriano Souza Lima Junior, Giselle Benevides Ferreira, Carlos Mauricio Oliveira
Maria Eduarda Alencar Santos, Carlos Henrique Almeida, Marcus Vinicius Della Coletta Universidade de São Paulo. São Paulo SP, Brazil
Ferreira Camargo, Helio Afonso Ghizoni Teive taminec@yahoo.com.br
Universidade Nilton Lins. Manaus AM, Brazil
Universidade do Estado do Amazonas. Manaus AM, Universidade Federal do Amazonas. Manaus AM,
Brazil Brazil Background: Progressive reduction of upper limbs func-
UFAM. Manaus AM, Brazil Universidade Federal do Paraná. Manaus AM, Brazil tional capacity is common in Parkinson’s disease (PD).
Universidade Federal do Amazonas. Manaus AM, Universidade do Estado do Amazonas. Manaus AM, In addition, upper limb freezing (FOUL) episodes can be
Brazil Brazil very disabling during daily life activities. Only a few tests
Universidade Nilton Lins. Manaus AM, Brazil for assessing upper limbs (UL) in PD are available. And a
Universidade Federal do Paraná. Curitiba PR, Brazil mcolletta@uea.edu.br standard upper limbs functional capacity assessment in
PD is unknown. Objective: To identify the frequent out-
mcolletta@uea.edu.br Background: In the last 15 years, studies on Idiopathic comes measurements to assess UL functional capacity in
Parkinson’s Disease (PD) have advanced considerably, PD. Methods: We reviewed systematically the literature
Background: Huntington’s disease (HD) is a heredi- gaining knowledge about the genetic forms. However, published from February 2010 to February 2021 accord-
tary neurodegenerative disorder. The epidemiology of complete information on the impact of these mutations, ing to PRISMA guidelines. We analyzed only published
HD is still lacking in information covering all regions of especially LRRK2 and GBA, on the clinical aspects of studies in English from a systematic review in PubMed,
Brazil. The first publication, bringing genetic aspects of 33 these patients is still lacking. Objective: To determine using the following keywords: “upper limbs” OR “upper
patients from 16 families with HD in Amazonas (AM), was the frequency of LRRK2 and GBA mutations in a popu- extremity” and “functional capacity” and “Parkinson’s
published by our research group in 2020 (BRITO, 2020). lation of PD patients in Amazonas and to determine the disease”[All Fields])). [MeSH Terms]). This study was
These initial data showed that the incidence of HD in BF influence of mutations on the clinical course of patients. registered at PROSPERO CRD 42021254486. Results:
and the specific clinical aspects of this population should Method: A cross-sectional cohort study with a multicenter We found initially a total of 408 studies, 396 in PubMed
be better studied and included in the brazilian series. character was performed to analyze the genetic forms of and 12 in other sources. According to the inclusion and
Objectives: To analyze HD’s prevalence, phenotypic and ILD. Blood samples were collected from 57 patients and exclusion criteria, 40 studies published in English were
genotypic profile in a reference center for movement dis- initially analyzed for mutations in the GBA and LRRK2 included in this review. All quantitative study designs,
orders in the North region of Brazil. Identify possible HD genes, and 68 other genes were subsequently analyzed if including systematic reviews published from February
transmission clusters due to geographic characteristics. the first analysis was negative. The clinical data was eval- 2010 were also included in this review. Studies that don’t
Describe the phenotypic characteristics of the popu- uated, comparing the evolutionary aspects of the disease mention PD and that don’t present UL assessments were
lation and correlate them with the genotypic findings. between carriers and non-carriers of mutations. Results: excluded. No studies were found to assess UL functional
Method: prospective observational study with analysis After analyzing 57 patients, 2 cases with a mutation in the capacity in PD. The most common upper limbs outcome
of patients seen at a referral center. Genetic testing for GBA gene (3.5%) and 3 cases with LRRK2 mutation (5.2%) measures found in the studies were: (i) to assess sever-
Huntington’s disease and clinical follow-up of patients were found. For the analysis of clinical data, we had access ity and progression of PD motor symptoms (tremor and
and family groups. Results: The results: presented here to 31 patients, of which 2 were carriers of LRRK2 mutation bradykinesia): UPDRS-III (n=17), MDS-UPDRS (n=14),
correspond to 55 patients with suspected HD, 38 of which and 1 of GBA mutation. Patients’ ages ranged from 32 to NFOG-Q (n=6), Nine Hole Peg Test (n=5), MOCA (n=4),
were effectively genetically tested and included in this 89 years (mean = 58.6 years). Age at onset of symptoms Purdue Pegboard Test (n=4), Fahn-Tolosa-Marin Tremor
analysis. Distribution by sex was 50% (19 H and 19 F). ranged from 27 to 81 years (mean = 51 years). Among Rating Scale (n=2); (ii) to assess manual dexterity: Nine
The mean age of symptom onset was 33 years (7 to 62 patients with the LRRK2 mutation, the mean age at onset Hole Peg Test (n=5) and Purdue Pegboard Test (n=4); (iii)
years), with a mean size of the expansions found at 48 of symptoms was 56 years, and in the GBA mutation, it to provoke and assess FOUL Spiral test (n=2) Funnel test
repetitions (39 to 84). There was a positive correlation (r= was 49 years. Regarding the cognitive pattern, 19 of the (n=2); (iv) Use of technology such as sensors (n=3), app
0.8) between the size of the expansions and the age onset 28 patients without mutations had moderate to severe or digital platform (n=3). Discussion: Currently, phys-
of motor manifestations. Parkinsonism was a frequent cognitive decline (2 to 4 in the UPDRS), less intense in iotherapy guidelines for PD provide no standard recom-
manifestation (79%), moderate to severe in 65% of cases. LRRK2 carriers (one patient with a score of 1 and a patient mendations to assess UL functional capacity. Only a few
Chorea was found in 60% of patients, moderate to severe with 3) and mild in the patient GBA carrier, with a score studies have shown that it is possible to assess tremors,
in 50%. Conclusion: Although considered a rare disease, of 1. The presence of hallucinations was less frequent in bradykinesia, and manual dexterity. There is a lack of a
an initial survey detected 19 families with Huntington’s carriers of mutations than in non-carriers, with 1 case in standard UL functional capacity assessment. FOUL can
disease in Amazonas, with great phenotypic and geno- LRRK2 mutation, against 11 cases in non-carriers. Sleep be objective ly assessed by evaluating the spiral-draw-
typic variability. The continuity of the study should bring disturbances were more frequent and intense in those ing or funnel tasks, but only a few studies reported these
comprehensive epidemiological data on the presence of without the mutation (26 cases), against none in GBA assessments. Conclusion: Current evidence is insufficient
clusters of the disease in the brazilian Amazon region. mutation and one moderate case in LRRK2. Conclusion: to recommend standard and effective outcome measures
The genetic analysis of this population showed the pres- to assess upper limb functional capacity in PD. Further
ence of 5 mutation carriers in the 57 patients studied research is needed.
Transtornos do Movimento (8.7%). In the clinical analysis, patients without mutations
showed greater intensity of cognitive complications, hal-
lucinations, and sleep disorders. The small study sample Transtornos do Movimento
limits the broadening of the results.

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369
XXX Congresso Brasileiro de Neurologia

TL 1105682 TL 1105731 TL 1105870

SEXUAL FUNCTION IN WOMEN WITH THERE IS AN ASSOCIATION BETWEEN ASSESSMENT OF QUALITY OF LIFE IN
PARKINSON’S DISEASE COGNITIVE DEFICIT AND RELIGIOSITY IN PATIENTS WITH PARKINSON’S DISEASE: A
PATIENTS WITH PARKINSON’S DISEASE? TERTIARY CARE CENTER EXPERIENCE IN
Katia Cirilo Costa Nóbrega, Raquel Taumaturgo Dias
Brito, Maria Elisa Pimentel Piemonte NORTHEASTERN BRAZIL
Carlos Eduardo Cordeiro Cavalcante, Matheus
Rodrigues Corrêa, Kelson James Almeida Natália Rebeca Alves de Araújo, Denise Maria
Universidade de São Paulo. São Paulo SP, Brazil
Meneses Cury Portela, Luciana Tolstenko Nogueira,
Universidade Federal do Piauí. Teresina PI, Brazil
cirilo_katia@yahoo.com.br Luana Larisse de Sousa Araújo, Lia Leal Laurini, Ana
matheuscorrea990@gmail.com Raquel Batista de Carvalho, Gustavo Sousa Noleto,
Background: Impairment of sexual function in Parkinson’s Antonio Levi Farias Borba, Janine Lemos Mel Lobo
Disease (PD) is common and problematic, with many of Background: Cognitive decline makes up the natural his- Jofili Lopes
its physical and mental aspects interrupted throughout tory of Parkinson’s Disease (PD), especially in advanced
the disease. Among the non-motor changes associated Universidade Estadual do Piauí – UESPI. Teresina
stages. In this context, religiosity may be related to the
with PD, sexual function is less discussed and, conse- PI, Brazil
status of cognition and executive functions, such as cog-
quently, less known. Satisfactory sexual function has been Centro Universitário Uninovafapi. Teresina PI, Brazil
nitive flexibility, which are usually also impacted in PD.
associated with better quality of life. Objective: To assess Universidade Federal do Piauí. Teresina PI, Brazil
Thus, it is essential to investigate the degree of cognitive
sexual performance and the couple’s level of adjustment Hospital Universitário da Universidade Federal do
impairment and its possible associations with religios-
in women with PD. Methods: Forty-seven women with a Piauí HU-UFPI
ity. Therefore, the literature confirms the applicability of
confirmed diagnosis of idiopathic PD and age ranging from Unifacid. Teresina PI, Brazil
the MOCA for cognitive assessment even in populations
38 to 68 years, stage 1 – 3 of disease progression according Departamento de Neurologia, Instituto Norman
with lower levels of education. Objectives: Analyze the
to the Hoehn and Yahr classification, without dementia, Fixel para doenças neurológicas, Universidade da
degree of cognitive impairment and verify its possible
participated in the study. After submitting the Free and Flórida – Estados Unidos
association with religiosity, quality of life and depressive
Informed Consent Term and agreeing to participate in the symptoms in patients diagnosed with PD. Methods: The nataliarebeca2607@gmail.com
study, the participants were asked to answer a previously MOCA and the Duke University Religiosity Index (DUREL)
established questionnaire through telephone contact. The were applied to patients diagnosed with PD according Background: Motor and non-motor symptoms in
following were investigated: (1) general information; (2) to the 2015 MDS criteria. Patients with a hypothesis of Parkinson’s Disease (PD) usually progress and cause
socioeconomic status; (3) information associated with atypical parkinsonism were excluded. DUREL was eval- physical, mental and social impairment, affecting the
the PD; (4) mental and cognitive health; (5) motor and uated according to its 3 components: organizational individual’s level of functionality and patients’ quality of
non-motor aspects of daily life experience assessed by religiosity (OR), non-organizational religiosity (RNO) life (QoL). Objective: To evaluate and to describe QoL
sections I and II of the MDS-UPDRS; (6) Sexual quotient and intrinsic religiosity (IR). Diagnostic of dementia or in PD patients. Method: Between May 2021 and January
– female version (QS-VF); (7) Dyadic Adjustment Scale mild cognitive impairment based on the total test score, 2022, a cross-sectional study was done at a Movement
(DAS). Statistical analysis tested the correlation between according to cutoff points in the literature. In addition, the Disorders clinic in Teresina/PI. QoL assessment was done
total sexual performance scores assessed by QS-VF and Beck Depression Inventory (BDI) and the PDQ 39 were using the Parkinson’s Disease Questionnaire 39 (PDQ39)
DAS. Results: There was a statistically significant cor- applied to assess depression and the degree of quality in brazilian Portuguese version. We also analyzed medical
relation between sexual performance assessed by QS of life in these patients, respectively. The Spearman test records to obtain demographics, HOEHN and YAHR (HY)
– VF and DAS mainly for values (p < 0.00), behavior (p was used to assess the correlation between the variables. Scale scores and Levodopa Equivalent Daily Dose (LEDD).
< 0.00), tired for sex (p < 0.00) and happiness (p < 0.01). This study obtained approval from the local ethics com- Patients were categorized according their age range. Data
Conclusion: For women living with PD, the quality of mittee approval. Results: Twenty-eight patients, aged was analyzed with RStudio software. Regression test was
the marital relationship is the main factor for the qual- between 48 and 76 years, were included. Among these, performed with disease duration and PDQ-39 domains,
ity of sexual performance. Therefore, seeking strategies 10 were diagnosed with dementia (MOCA < 17.5) and 9 considering p<0.05 as significant. Results: During the
with the objective: of intervening and helping couples with mild cognitive impairment (MOCA < 22.5). Among period, data were collected from 93 patients, most of
to achieve or maintain a good marital relationship is of patients with dementia, 50% did not have any level of whom were male (63.4%) and aged between 65 and 74
fundamental importance to improve the sexual function/ education. The Spearman test was calculated and the years (41.9%). As for the duration of the disease, most had
sexual performance of this population. correlation coefficient of the MOCA with the other eval- between 5 and 10 years of evolution (44.1%) and the LEDD
uated scales was: DUREL – RO and RNO (0.347; p=0.71), was between 350 and 700 (33.3%). The mean LEDD of the
RI (0.06; p=0.764), BDI (0.063; p=0.748), PDQ – 39 (-0.063; general population of our patients was 677.37 (± 372.07).
Transtornos do Movimento p=0.749) and UPDRS III (-0.241, p=0.217). Conclusion: On the HY Scale, most were between stages 1 and 3 (91.4%).
Approximately 32% of the sample was classified as having Regarding the PDQ-39, 4 out of the 8 domains had means
a mild cognitive impairment and 36% as having dementia. above 50, and therefore, a worse perception of quality of
No significant association was found between cognition, life, namely Mobility (71.93/±30.24), Body discomfort
religiosity, quality of life or depression. There was also (70.60/±23.71), Activity of daily living (ADL) (62.95/±29,
no significant association between cognition and motor 24) and Emotional well-being (54.25/±29.63). The domain
impairment in these patients. with the lowest score in the general population of this study
was Social Support (34.41/±13.91). When comparing the
averages of men and women separately, the domains with
Transtornos do Movimento the greatest scores were also Mobility, Body Discomfort,
ADL and Emotional Well-being. However, in all these
domains, women had greater scores when compared to
men. In addition, after regression test, there was statisti-
cal significance in the Mobility (p=0.023), ADL (p=0.016)
and Communication (p=0.006) domains in patients with
more than 20 years of disease duration. Conclusion: It
was noted that both motor and non-motor aspects may
be related to a worse perception of QoL by these patients.
In addition, other individual characteristics, such as sex,
lead to different perspectives on quality of life. Thus, it is
necessary to direct integrated care strategies aimed at the
support and treatment of people with Parkinson’s Disease,
taking into account their individualities.

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370
XXX Congresso Brasileiro de Neurologia

TL 1105922 TL 1106061 TL 1106129

WHY SLOTHS ARE SO SLOTH? IS THE NEURAL BASIS OF FACIAL EMOTION THE EFFICACY OF DEEP BRAIN
THE BRADYPUS A PHYSIOLOGICAL RECOGNITION IN PROGRESSIVE STIMULATION IN THE SUBTHALAMIC
PARKINSONIAN ANIMAL MODEL? SUPRANUCLEAR PALSY: A NEUROIMAGING NUCLEUS IN ISOLATED DYSTONIA
STUDY
Carlos Frederico Leite Souza-Lima Júlian Letícia Freitas, Iara Amorim, Luiza Corazza,
Bárbara Caroline Dias Faria, Paulo Caramelli, Thiago João F Tasch, Paula C Alves, Maria Sheila Rocha
Hospital Universitario Oswaldo Cruz. Recife PE,
de Oliveira Maciel, Elisa de Paula França Resende,
Brazil Hospital Santa Marcelina. São Paulo SP, Brazil
Luciano Inácio Mariano, Antônio Lúcio Teixeira,
carlosfredericolima@gmail.com Sarah Teixeira Camargos, Francisco Costa Cardoso, julian.leticia.freitas@gmail.com
Leonardo Cruz de Souza
Background: There is no natural animal model for Background: DBS is a safe and effective therapy for
Universidade Federal de Minas Gerais. Belo
Parkinson’s disease, only experimental animal models patients with isolated (inherited or idiopathic) and med-
Horizonte MG, Brazil
that do not help in understanding the pathophysiol- ically refractory dystonia in both generalized and severe
Universidade Federal do Rio de Janeiro. Rio de
ogy and natural evolution of the disease. The Bradypus focal and segmental dystonia. Subthalamic nucleus DBS
Janeiro RJ, Brazil
Variegatus (Common Sloth) is a tropical forest animal for dystonia publications has been limited to small series
UT Health – Estados Unidos
(atlantic forest) that has the unique behavior of slowness and case presentation. Therefore, the objective of this
and bradykinesia being called a Sloth. To this day, it is not leocruzsouza@hotmail.com systematic review was to provide clinical evidence of the
known why it was so slow (including study of physiology, efficacy of DBS in the STN in isolated dystonia. Methods:
pathology, hormone dosages, etc.). Objective: to describe Background: Deficits in facial emotion recognition have For this article, we performed a comprehensive search in
and correlate the characteristics of motor behavior and been described in Progressive Supranuclear Palsy (PSP). databases for articles published from 1987 to 2022. We
physiology of the Bradypus Variegatus with Parkinson’s However, the neural basis of these deficits remains uncer- included patients with primary dystonia, focal, segmental,
disease, discussing the possibility of being a physiologi- tain. Objectives: To investigate the correlations between or generalized, who underwent DBS placement in STN
cally determined “Parkinsonian” animal model. Method: the Facial Emotion Recognition Test (FERT) and measures and had no prior brain lesioning or brain surgeries. Data
data collection based on a literature review on the study of cortical and subcortical volume in patients with PSP. collected included demographics, clinical features of dys-
of the Common Sloth and its bradykinesia. Animal neu- Methods: 21 patients with PSP (age = 68.0 ± 7.4 years) tonia, adverse events, stimulation parameters, and quality
roanatomy, neurophysiology and animal behavior stud- and 20 healthy controls (age = 67.5 ± 10.8 years) under- of life scales. Results: The literature search resulting in
ies, with special attention to data related to Parkinson’s went cognitive assessment, including the FERT, which the identification of 20 relevant articles corresponding to
disease. Results: Bradypus has several “Parkinsonian” is composed of 35 images from Ekman’s portfolio, with 14 distinct studies and reporting 114 cases. There were 75
features. The most common is bradykinesia. However, 7 different emotions (happiness, sadness, fear, disgust, women (65,7%) and 39 men. The mean age of onset was
paradoxical kinesis is also exist (agile when threatened. surprise, anger, and neutral). All participants underwent 37.2 ± 19.1 years (± SD) and the duration of dystonia was
It has quick strikes with its claw in threats and fights. brain MRI (3T). We conducted two neuroimaging analyses: 9.1 ± 8.8 years (± SD). They were 40 focal dystonia, 44 seg-
Hypomimia with reduced blinking and facial expression, 1) subcortical structures were segmented with Freesurfer mental dystonia, and 30 generalized dystonia. The mean
smell too. Predominance of flexor tone (flexor muscles software and volumes were extracted for analyses. We follow-up time was 12.8 months (range 3-33 months). The
are antigravity), with posture of flexor rigidity most of the explored correlations (Spearman’s test) between sub- BFMDRS movements and TWSTRS scores were signifi-
time. Slow transit colon with a frequency of 1 evacuation cortical nuclei volumes (putamen, caudate, amygdala; cantly lower in pos operatives values (p < 0.0001), with
per 10 days. Slow responses to painful stimulus. In sum- corrected for total intracranial volume) and FERT scores a mean decrease of 55.3% (CI 48.97-61.6). In total, 20
mary, 1) marked bradykinesia 2) Paradoxical kinesis 3) in PSP patients; 2) voxel-based morphometry (VBM) was patients experience an improvement of more than 80%
very slow intestinal transit 4) Sialorrhea 5) facial hypo- performed with FSL software, exploring correlations over the preoperative score. Subitem analysis revealed a
mimia with reduced blinking 6) reduced sense of smell between gray matter density and FERT scores within the 61.4% score for focal dystonia, 47.8% in segmental dys-
7) predominance of postural flexor tone with clear flexor PSP group. Age, sex and disease duration were considered tonia, and 57.5% in generalized dystonia. The BFMDRS
hypertonia are common signs of Bradypus and also to as covariates. A threshold of 50 contiguous voxels was disability scale scores had also improvement (p < 000.1),
parkinsonian patients. These characteristics are useful in adopted, corrected at family-wise error (FWE) at the p<0.05 with mean reduction of 43.9% (p = 0.0057). The quality of
their animal lifestyle (they lives in trees) and this “hypo- threshold, for all analyses. Results: PSP and controls did life was assessed in only five studies using SF-36 scores.
kinetic-hypertonic extrapyramidal status” favors them to not differ on age and schooling. PSP patients performed The patients’ quality of life was improved significantly
remain in the trees, even during sleep, without fatigue. worse than controls (p < 0.001) on the FERT (total score, (p < 0.0001) in every subitem of the scale. Discussion:
Conclusion: This is the first description in the literature disgust, anger, sadness and neutral). Compared to con- This meta-analysis represents the current data on DBS in
that correlates Bradypus with Parkinson’s disease and trols, PSP had lower volumes of brainstem (p < 0.001), STN in primary dystonia. Our findings support sustained
places it as the first natural animal parkinson’s model, caudate (p < 0.001), putamen (p < 0.001), and frontal improvement in motor function, measured by absolute
not from a pathological point of view, but with a natural atrophy. Within PSP patients, surprise score correlated and percentage changes in the BFMDRS and TWSTRS
and sucessful “Parkinsonian status”. to right (p < 0.001, rho = 0.72) and left (p < 0.003, rho = scores from baseline. We also observed an improved
0.66) amygdala; disgust correlated to right (p < 0.02, rho ability to perform activities of daily living and quality of
= 0.54) and left (p < 0.03, rho = 0.52) amygdala; neutral life access through BFMDRS disability and SF-36 score.
Transtornos do Movimento score correlated to right (p < 0.01, rho = 0.55) and left (p
< 0.0001, rho = 0.74) putamen and right (p < 0.008, rho =
0.6) and left (p < 0.001, rho = 0.7) amygdala. VBM analy- Transtornos do Movimento
ses yielded significant direct correlations between frontal
pole and scores of disgust, sadness, anger, and surprise.
Disgust also directly correlated with insula. Conclusion:
Subcortical nuclei and frontoinsular regions play a role
in the recognition of facial emotions in PSP, suggesting
that a complex fronto-subcortical network participate in
emotional processing in the disease.

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TL 1106190 TL 1106217 TL 1106248

SOCIAL COGNITION IN PARKINSON’S THE EFFECTS OF HIGH-SPEED RESISTANCE MOVEMENT DISORDERS AFTER ACUTE
DISEASE AND SUPRANUCLEAR PARALYSIS: TRAINING IN PARKINSON’S DISEASE STROKE: A LARGE PROSPECTIVE COHORT
A NEUROPSYCHOLOGICAL INVESTIGATION PATIENTS ON MOBILITY: A SYSTEMATIC STROKE STUDY
REVIEW AND META-ANALYSIS
Marina Isoni Martins, Francisco Costa Cardoso, Luiza Corazza, João F Tasch, Júlian Letícia Freitas,
Paulo Caramelli, Luciano Inácio Mariano, Natalia Samuel Brito de Almeida, Emmanuelle Silva Tavares Iara Amorim, Paula Camila Alves, Henrique Alves
Pessoa Rocha, Antônio Jaeger, Antônio Lúcio Sobreira, Danielle Pessoa Lima, Janine de Carvalho Bezerra, Maria Sheila Rocha
Teixeira, Vítor Tumas, Sarah Teixeira Camargos, Bonfandini, Manoel Alves Sobreira-Neto, Thiago
Leonardo Cruz de Souza Hospital Santa Marcelina. São Paulo SP, Brazil
Holanda Freitas, Simony Lira do Nascimento, Pedro
Braga-Neto julian.leticia.freitas@gmail.com
Universidade Federal de Minas Gerais. Belo
Horizonte MG, Brazil Universidade Federal do Ceará. Fortaleza CE, Brazil
UT Health – Estados Unidos Background: Post-stroke movement disorders (PSMD)
Universidade de São Paulo. Ribeirão Preto SP, Brazil samuelbrito.ef@gmail.com can occur either acutely or in the first year following
a stroke. Abnormal movements after a stroke may be
leocruzsouza@hotmail.com Background: The loss of strength in PD patients has an either hypo or hyperkinetic, but involuntary hyperkinetic
impact on their quality of life. Common activities like get- movements are more frequently observed during stroke
Background: Patients with Parkinson’s disease (PD) ting up a chair, as well as all daily life activities become follow-up. PSMD is uncommon, with a known preva-
and Progressive Supranuclear Palsy (PSP) have deficits difficult. Moreover, muscle weakness, postural deficits lence of about 1% and an incidence of 0.08% per year,
in mentalizing and facial emotion recognition, two core and decreased mobility have been related to increased highlighting the rarity of PSMD. Most basal ganglia and
functions of social cognition (SC). There is scarce data risk of falls in PD patients. Objective: The aim of this thalamic lesions did not result in movement disorders,
regarding the direct comparison between PD and PSP systematic review and meta-analysis is to compare the suggesting that stroke location may not predict PSMD.
patients in SC measures. Method: We included three efficacy of high-speed resistance training (HSRT) versus Also, PSMD may be associated with both ischemic and
groups of participants: PD (n=18, mean age = 65.2±9.8 control or another form of physical activity intervention hemorrhagic strokes. Few studies concern PSMD prev-
years, 9 men), PSP (n=20, mean age = 67.7±6.8 years, 8 to improve mobility in Parkinson’s Disease (PD) patients. alence in a designed prospective cohort. Objective: We
men) and healthy controls (n=23, mean age =64.7±10 years, Data sources: We performed a systematic literature search aimed at stating the prevalence of acute post-stroke move-
8 men). Groups were matched for age, sex and education. through electronic databases including MEDLINE via ment disorders (PSMD) in a large prospective cohort of
Participants underwent clinical and neuropsychological PubMed, LILACS via BVS, Embase via Elsevier and PEDro acute stroke patients. Methods: We designed an open,
exams, including the Mini-Mental State Exam (MMSE), databases up to September 2021. Methods: We included prospective, one-year, single-center stroke cohort to fol-
executive tests (Frontal Assessment Battery [FAB], spans, randomized clinical trials with patients with PD, HSRT as low all acute cerebrovascular disorders searching for any
phonemic fluency, Hayling) and the mini-version of the the intervention, compared to a control group or another movement disorders during Hospitalization and after dis-
Social and Emotional Assessment (Mini-SEA), which is type of physical activity. Standardized mean difference charge. PSMD was the primary outcome, and death was
composed of the facial emotion recognition test (FERT) (SMD) with 95% confidence interval (CI) were calculated the main competitive outcome. Our study included all
and the modified faux-pas (mFP) test, which assesses based on the inverse of variance method. Results: The acute stroke patients admitted to the Hospital from March
mentalizing. Multivariate analyses considered executive literature search identified a total of 7631 results among 2021 to February 2022. Besides the demographic data, we
performance and apathy as covariates. Results: Compared which 4 studies were selected for the meta-analysis pre- captured clinical and neurological data: NIH stroke scale,
to controls, PD and PSP patients had lower cognitive effi- senting moderate to high methods quality. The meta-anal- modified Rankin scale, Glasgow coma scale, and abnor-
ciency (MMSE). PD and PSP groups did not differ on the ysis showed a significant difference on mobility outcome mal involuntary movement scale. We also evaluated the
MMSE. PSP had lower score than PD patients and con- in favor of HSRT compared to a control group (SMD = first CT scan of all patients for stroke localization and type
trols in the FAB. PD patients scored lower than controls 0.42; 95%CI [0.00;0.83], p= 0.049). Conclusion: HSRT determinations. Results: We included 858 acute stroke
in the mFP, but not in the FERT. PSP patients performed may improve mobility in PD patients when compared to patients. There were four acute PSMD patients in this
worse than controls in the mFP and in the FERT. PD and a control or minimal intervention group. HSRT seems to cohort, yielding a prevalence of 0.5%. Table-1 discloses
PSP groups did not differ in the FERT, but PSP performed be a good approach for resistance training to enhance all demographic data. In all PSMD cases, the stroke was
worse than PD in the mFP test. These differences were mobility in PD individuals. ischemic. Two patients revealed thalamic lesions (2.4%
maintained when controlling for executive dysfunction of thalamic stroke patients), and two had striatal isch-
and apathy. The FERT and the mFP distinguished PSP from emic strokes (2.1% of striatal stroke patients). Movement
PD patients with 89% and 67% accuracies, respectively. Transtornos do Movimento disorder occurred in the first 24h after ischemia in all
Conclusion: The assessment of SC may contribute to the cases. Concerning the abnormal movement phenotype,
differential diagnosis between PD and PSP. all were hyperkinetic. One case disclosed acute tremor
after striatal ischemia, and in the other patients, dystonia
was the prevalent movement disorder. Conclusion: To
Transtornos do Movimento sum up, this large cohort confirms that PSMD in acute
stroke is rare, with an established prevalence of 0.5% for
all stroke events. PSMD was associated with striatal and
thalamic ischemic stroke, and all cases presented acute
hyperkinetic movement disorder.

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XXX Congresso Brasileiro de Neurologia

TL 1106331 TL 1106333 TL 1106417

INTERLEAVED STIMULATION FOR CLINICAL ASPECTS OF SPINOCEREBELLAR ASSOCIATION BETWEEN BALANCE


REFRACTORY TREMOR IN PD: A SERIES ATAXIA IN THE STATE OF AMAZONAS PERFORMANCE AND COGNITIVE
CASE IMPAIRMENT IN PEOPLE WITH PARKINSON’S
Marcus Vinicius Della Coletta, Diana V. Brito, Giselle
B. M. Ferreira DISEASE
Maria Sheila Guimarães Rocha, Iara Amorim Torres,
Julian Leticia Freitas, Luiza Corazza, Percilia Gabriel Venas Santos, Matheus Silva d’Alencar,
Universidade do Estado do Amazonas. Manaus AM,
Cardoso, Jamana Barbosa, João Felipe Tasch Maria Elisa Pimentel Piemonte
Brazil
Hospital Santa Marcelina. São Paulo SP, Brazil Universidade Federal do Amazonas. Manaus AM, Universidade de São Paulo. São Paulo SP, Brazil
Brazil
iara.torres@hotmail.com gabriel.venas@usp.br
mcolletta@uea.edu.br
Background: Interleaved stimulation (IS) in DBS provides Background: People with Parkinson’s Disease (PD) often
individualized energy for two contacts alternately. Most Background: Spinocerebellar ataxias (SCAs) are a group use cognitive strategies to maintain balance and postural
documented indication concerns the management for of neurodegenerative hereditary diseases, with an autoso- stability due to their deficits in terms of automaticity,
levodopa induced dyskinesias. On the other hand, it is mal dominant pattern. The prevalence of SCAs subtypes among the motor symptoms presented in the disease,
well acknowledged that refractory PD tremor responds is variable among geographic regions and ethnicities. In postural instability is one of the most disabling and seems
better to high frequency DBS, although with adverse events Brazil, the occurrence of the main subtypes was stud- to be related to cognitive deficits. Aim: In this study, we
on balance and speach. Animal studies demonstrate that ied mainly in the Southern and Southeastern regions. determined the cognitive factors that may be related to
coordinated reset stimulation of the subthalamic nucleus However, considering the ethnic diversity among brazil- balance deficits in individuals with PD. Methods: A sam-
presents short and long-term improvement in the parkin- ian regions, those findings cannot be generalized. Thus, ple of 74 participants, recruited in a Parkinson’s associ-
sonian motor condition, with lower energy expenditure this study aimed to investigate and clinically characterize ation in São Paulo, being 22 healthy elderly (CG) and 52
and lower incidence of complications. We hypothesized cases of SCAs in the state of Amazonas. Method: Research people with Parkinson’s, subdivided into early (IPG) and
if IS, as a quasi-coordinated reset mode, would control participants were recruited at the reference center for moderate (MPG) Parkinson’s was evaluated based on vari-
tremor and avoid complications. Objective: To test IS movement disorders in Manaus-AM. Genetic tests for ables clinical and social, among the variables evaluated,
would improve tremor without causing adverse effects. SCAs 2, 3, 6, and 7 were performed. Results: Of 21 par- the groups were homogeneous in terms of age (p=0.14),
Methods: We evaluated 5 tremor-dominant Parkinson’s ticipants, nine were genetically confirmed: two families education (p=0.21). The groups were different in terms
disease patients, on medication and previously stimulated (N=5) were diagnosed with SCA 3; one family (N=3), with of motor presentation measured from the MDS-Unified
in a basic pattern, all at high voltage and frequency. All SCA 2; and one patient with SCA 7. In one of the SCA 3 Parkinson’s Disease Rating Scale Part II and III. For bal-
had DBS complication due to high total energy delivered, families, symptoms were predominantly cerebellar: gait ance evaluation, the Balance Evaluation Systems Test
like balance and dysarthrophonia, and pain. We modified ataxia, dysarthria, hypotonia, and nystagmus. Pyramidal (BESTest) was used in its complete format, evaluating
the stimulation to the interleaved pattern, using the con- signs (hyperreflexia) and peripheral neuropathy were also domains such as Biomechanics, Stability Limits, Postural
tacts that the patients already had before. Data analyzed observed. In the other SCA 3 family, cerebellar signs of Responses, Anticipatory Postural Adjustments, Sensory
included Unified PD Rating Scale III and tremor score. gait ataxia and dysarthria were associated with peripheral Orientation and Dynamic Balance during gait and cogni-
Results: There were 4 male and 1 female, with mean age neuropathy, cognitive dysfunctions (memory loss), and tive effects and for the cognitive impairment, the Montreal
of 59. Mean baseline UPDRS was 60,6 and mean tremor psychiatric disorders (irritability and depression). The Cognitive Assessment (MoCA), a brief screening instru-
score was 3,5. All 5 patients showed satisfactory improve- SCA 2 family was clinically characterized by ataxia (more ment for mild cognitive impairment. It accesses different
ment in parkinsonian symptoms and relief from stimu- severe gait, but also appendicular and axial), movement cognitive domains: attention and concentration, execu-
lation-induced side effects after interleaved stimulation, decomposition, and dysarthria in association with slow tive functions, memory, language, visual-constructive
established by a large effect size (Cohen d) in UPDRS III saccades and mild bradykinesia. Lastly, the patient diag- skills, conceptualization, calculation and orientation. This
(d=3,71285) and tremor score (d=1,543766). All patients nosed with SCA 7 showed a condition of mild cerebellar study was approved by a Local Ethics Committee (CAAE
were free of complications after changing the stimulation signs (gait ataxia, hypermetria, and dysarthria) and pre- 67388816.2.0000.0065). Results: MoCA data, especially
mode to IS. Discussion: The use of the interleaved mode dominant symptoms of loss of visual acuity, ophthalmo- when comparing the MPG group with the others, were
is known to improve dyskinesias, through stimulating pal- paresis, bilateral ptosis, and facial hypomimia. Pyramidal different due to the presence of some cognitive deficit
lid-fugal fibers in the Zona Incerta, and sensory-motor signs (hyperreflexia), distal atrophy, and tongue fascic- with the progression of the disease (p=0.031). A positive
fibers in the laterodorsal zone of the subthalamic nucleus. ulations were also observed. Conclusion: The research correlation was demonstrated between the total values
Requires frequency reduction, so it would be a bad alter- demonstrated the presence of families with SCA2, SCA3 of MoCA and the Best TEST (p<0.009; r=.3047), as in spe-
native for the tremor-dominant patient, as they tend and SCA7 in Amazonas. New data will be added with test- cific domains related to visuospatial memory and visuo-
respond to high frequencies. On the other hand, the high ing for SCA10. So far, we have been able to detect a low spatial orientation (p<0.001; r=.3786), evaluated respec-
frequency leads to adverse effects such as hypophonia, incidence of families with SCAs in the state of Amazonas, tively. Conclusion: Visuospatial memory is important in
dysarthrophonia and worsening of balance. Conclusion: compared to other brazilian studies. everyday activities that require location of the individual
With this study, we demonstrated a substantial improve- in space, differentiation of shape and spatial location
ment in the motor condition of these patients, in addition of objects. Previous studies indicate that visuospatial
to an improvement in side effects. Transtornos do Movimento
memory is related to gait rhythm and postural control in
healthy elderly people and those with PD. Thus, deficits
in visuospatial memory are related to a higher risk of falls
Transtornos do Movimento in this group of people.

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XXX Congresso Brasileiro de Neurologia

TL 1106574 TL 1106626 TL 1104816

PREVALENCE OF PSYCHOSIS IN PARKINSONISM FOLLOWING COVID-19 IN PRELIMINAR STUDY FOR NEUROVISUAL


PARKINSON’S DISEASE – PRELIMINARY A LONG-COVID COHORT – CASE SERIES ASSESMENT MODEL USING EYE TRACKING
RESULTS OF A CROSS-SECTIONAL STUDY REPORT (NAMET) RELATED TO COGNITIVE
FUNCTIONS FOR TBI PATIENTS
Henrique Nascimento Dourado, Gustavo Cavalcante Danilo Nunes Oliveira, Jose Wagner Leonel Tavares-
Procópio, Victoria Faustino Silva Reis, Lara Cordeiro Júnior, Safira de Brito Gaspar, Álissa Elen Formiga Daniela Yoshida
Magalhães, Fernanda Souza Gracílio Silva, Ana Moura, Manoel Alves Neto Sobreira, Pedro Braga-
Rafaela Soares Vale, Gabriel Vianna Pereira Aragão, Neto FMUSP
Hugo Oliveira Polito Barreto, Isabelle Jacqueline
Universidade Federal do Ceará. Fortaleza CE, Brazil daniyosh@gmail.com
Weber Oliveira, Guilherme Teixeira Valença

Escola Bahiana de Medicina e Saúde Pública. danilonunesoliveira@gmail.com Neuro Visual Assessment Model for visual and neuro
Salvador BA, Brazil cognitive rehabilitation following TBI can include Visual
Hospital Geral Roberto Santos HGRS Background: Even though SARS-CoV-2’s role in causing Acuity (VA), Visual Field, eye movements (Eye Tracking),
Parkinson’s disease (PD) has not yet been confirmed, Accommodation and Convergence. The main objective of
henriquendourado@gmail.com recent studies describe a possible association between the study is to present a standardized NAMET in patients
COVID-19 and movement disorders. SARS-CoV-2 inva- who suffered traumatic brain injury (TBI) and are in the
Background: Parkinson’s disease (PD) is the second most sion of vasculature in the brain may cause multiple out- cognitive rehabilitation process. The main symptoms
common neurodegenerative disease. Its prevalence is comes through inflammatory responses that could lead to found were: Binocular Visual Deficit (80%) Photophobia
close to 1% in the population over 60y and its incidence protein misfolding, a characteristic of neurodegenerative (75%) Accommodation and Convergence Problems (70%)
ranges from 8-18: 100,000 person-years. Its etiology is mul- disorders. Objective: This study describes three patients Diplopia (50%) Reading Deficit (50%) Oculomotor Deficits
tifactorial, with the main risk factor being aging. Although who started parkinsonian symptoms after confirmed diag- (80%) Visual Field (20%) NAMET detects alterations in
known for its motor symptoms, it encompasses a variety nosis of COVID-19 infection. Methods: We evaluated a saccadic eye movements, persuits and fixation not visi-
of non-motor symptoms such as psychosis, whose patho- prospective cohort study with patients followed in a post- ble in subjective tests without the use of the Eye Tracking
physiology involves a combination of endogenous (dis- COVID neurological outpatient clinic, recruited based on Device. Some of the visual dysfunctions found could be
ease/patient) and exogenous (treatment) factors. There is positive COVID-19 PCR and post-COVID neurological carried out with the help of prisms, lenses and filters,
still no direct association between dopaminergic treatment symptoms. The total sample consisted of 221 patients improving the patients’ time reading, oculomotricity,
and psychosis, but it is believed that it may be triggered or recruited for the study. Three had a diagnosis compatible balance and posture performance. This improvement in
“accelerated” by it. Its management involves the gradual with Parkinson’s disease. Results: The first patient, a 44 the visual performance of post-TBI patients helps in the
withdrawal of contributing drugs and the background of years-old-female developed mild flu-like symptoms last- cognitive rehabilitation process with other professionals
antipsychotics if necessary. Estimates of the prevalence of ing 6 days. One month later, she describes an asymmetric in a transdisciplinary team.
PDP vary widely in the literature. Objective: To describe rest tremor. Neurological exam shows facial hypomimia
the prevalence of Parkinson’s disease Psychosis (PDP) and cogwheel stiffness with bradykinesia in the left upper
and investigate patients’ sociodemographic and clinical limb. The second patient, a 57 years-old-man, reported Traumatismo cranioencefálico
profile. Methods: Ongoing descriptive, observational, mild flu-like symptoms with anosmia lasting 7 days. The
cross-sectional study conducted in a Movement Disorders symptoms progressive after 10 days with difficult to count
Outpatient Clinic in Salvador, Bahia. We included patients money at his work and difficult walking. On examination
over 18y diagnosed with PD by the UK Brain Bank Criteria he had mild hyposmia and mild bradykinesia with stiffness
and excluded patients with psychiatric comorbidities. in the left upper limb. The third patient, a 56 years-old-
The study was approved by the local Ethics Committee. female had a history of antiphospholipid syndrome. She
Data gathering involved questionnaires for clinical and reported flu-like symptoms lasting 4 days. After 2 months
sociodemographic evaluation. The NINDS/NIHM crite- started a rest tremor. Neurological exam disclosed mild
ria, validated by the MDS, was used to diagnose PDP. The cogwhell stiffness with bradykinesia. All the three patients
modified Hoehn & Yahr Scale (mH&Y) was used to stage were responsive to levodopa / benzeraside. Discussion:
the disease’s progression and the MMSE for cognitive Since the beginning of the pandemic, several neurologi-
assessment. Results: Until now, the sample size has 23 cal manifestations have been described associated with
patients. The median age was 63y (IIQ 52-72), with 65.2% COVID-19. Parkinsonism associated with viral infections
being male, 39,1% self-reported as mixed-race and 52,2% has been described with other viruses. Historically, several
were married. Regarding education, 43.5% had completed cases of post-encephalitic parkinsonism were described
high school, and the median MMSE was 28 (IIQ 21-29). after the Spanish flu in 1918. The cause of parkinsonism
High Blood Pressure was the most common comorbidity in the present cases has some hypotheses, among which
(43.5%). The mean time of disease was 9.3±4.5 years and direct viral infection with neurodegeneration, inflamma-
34.8% had mH&Y of 2, while only 4.3% had mH&Y of 5. All tory mechanism, or multiple hits. Conclusion: Available
patients were on levodopa therapy, 34.8% on dopamine data does not confirm a clear association between the
agonist, 26.1% on amantadine, 21.7% on COMTi and 8.7% Covid-19 infection and PD. Parkinsonian symptoms
on MAO-Bi. The prevalence of psychosis in the sample after COVID-19 infection seems to be a rare condition.
studied is 8.7% (n=2). Conclusion: It is concluded that the We propose a possible association between Covid-19 and
prevalence of PDP in the studied sample so far is low, but the development of PD.
corroborates with the current literature. We emphasize
the importance of investigating non-motor symptoms in
PD, often neglected, as evidenced by the low number of Transtornos do Movimento
studies on PDP, especially in Brazil.

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XXX Congresso Brasileiro de Neurologia

TL 1105834 TL 1105857 TL 1106282

QUANTITATIVE ANALYSIS OF CONSERVATIVE DECOMPRESSIVE CRANIECTOMY DIGITAL BIOMARKER-BASED


TREATMENT FOR TRAUMATIC BRAIN INJURY TECHNIQUES AND THEIR OUTCOMES IN INDIVIDUALIZED ASSESSMENT IN
IN BRAZIL REDUCING INTRACRANIAL HYPERTENSION PROFESSIONAL BOXING AND MMA
ATHLETES: A DIGITAL NEURO SIGNATURE IN
Alberto Henrique Torres Trindade da Silva, Déborah Alberto Henrique Torres Trindade da Silva, Déborah
Emmily de Carvalho, Henry Martins Soares Fortes, Emmily de Carvalho, Henry Martins Soares Fortes, ASSESSING SPORTS-RELATED TRAUMATIC
Mariana Gonçalves Maciel Pinheiro, Ana Cristina Leonardo Afonso Lorenzoni, Luiz Severo Bem Junior BRAIN INJURY
Veiga Silva, Marcelo Moraes Valença
UPE. Recife PE, Brazil Matheus Gonçalves Maia, Renato Anghinah, Diogo
Universidade de Pernambuco. Recife PE, Brazil Universidade Federal de Pernambuco. Recife PE, Haddad Santos, Yngrid Dieguez Ferreira, Luís
Universidade Federal de Pernambuco. Recife PE, Brazil Eduardo d’Almeida Manfrinati
Brazil Hospital da Restauração. Recife PE, Brazil
Santa Casa de Misericórdia de São Paulo
Hospital da Restauração. Recife PE, Brazil
leonardo_afl@outlook.com Hospital das Clínicas da Faculdade de Medicina da
gmpmariana@gmail.com Universidade de São Paulo
Background: Decompressive craniectomy is a procedure matheusgmaia1@gmail.com
Background: Traumatic brain injury (TBI) is a struc- that removes part of the skull and the underlying dura to
tural injury of the skull, brain or associated structures. reduce brain swelling-related intracranial pressure (ICP).
Background: Current computerized neuropsychological
Conservative treatment involves the use of drugs and a The present study compares four DC techniques and their
assessment (NPA) batteries for athletes at risk of sports
non-surgical approach. The present study compares the outcomes in ICP. Methods: We searched by PUBMED in
concussion (SC) are inadequate proxies for traditional
number of Hospitalizations, the total cost and the average November 2021, using the Mesh Terms “ Decompressive
neuropsychological tests and have limited clinical utility in
length of stay between conservative treatments for mild, Craniectomy AND Intracranial Pressure ”. Exclusion crite-
postconcussion assessments. Therefore, it becomes neces-
moderate and severe TBI in 10 years in Brazil through ria were studies that did not mention positive and nega-
sary research for new and supplementary methods of NPA
DATASUS. Methods: It is a cross-sectional epidemio- tive points of the surgery. Novel studies in English about
on SC. An active digital biomarker-based tool is the Altoida
logical study with values ??obtained from the DATASUS decompressive craniectomy techniques published in the
Digital Neuro Signature (ADNS), an augmented-reality
database from December 2011 to November 2021. Data last ten years were inclusion criteria. Results: Classic DC
based test. A previous trial demonstrated 94% prognostic
of conservative treatment of mild, moderate and severe is the most commonly performed operation for patients
accuracy to measure dementia progression on patients
traumatic brain injury were selected and grouped into that require cerebral decompression after suffering a trau-
with Mild Cognitive Disorder (MCI). Objective: Describe
Excel for the number of Hospitalizations, the average matic brain injury (TBI) or stroke. Window DC proved to
a case series of 15 professional fighters who underwent
length of stay and total value. Results: There were 427,916 be an effective alternative to classic DC in the process of
NPA by the ADNS and correlate worse performance with
admissions for mild conservative treatment, 290,130 for high ICP after severe TBI, showing results in reducing ICP
higher burden of subconcussive traumatic events. Study
moderate TBI and 112,141 for severe. As for the Hospital and improving ECG scores. In addition, it offers a better
design: Case series; Level of evidence, 4. Methods: The
cost, conservative treatment for mild TBI had a total aesthetic result and prevents the skin flap from sinking
study participants were 15 professional fighters who
value of $35,7340.07, moderate $61,552,281, and severe into the incision cavity and a second bone replacement
underwent NPA by ADNS. The mean age was 26.7 years.
$64,395,813. The mean stay in the health care institu- surgery. Bifrontal DC allow better visualization and explo-
The higher number of head blows were estimated based
tion was 2.8 days for mild TBI, seven days for moderate ration of the brain, a technique used in patients with a very
on the longer exposure to sparring sessions and conven-
TBI, and 11.5 days for severe TBI. Conclusion: Although high risk of mortality due to cerebral oedema secondary
tional training. The sparring hours practiced, measured in
conservative treatment for mild TBI was more frequent to trauma. Modified decompressive hemicraniectomy
hours based on the last month, varied from 1 to 16 hours.
(51.5%), severe TBI had a higher Hospital cost (39.8%), access the middle cerebral artery territory and, in gen-
The conventional training, measured in hours based on
justified by the higher Hospital stay and greater demand eral, the described technique allows lower postoperative
the last week, varied from 5 to 30 hours. Performance
for supplies to maintain the clinical status. The mean risk. Conclusion: All of the techniques presented in this
measures were computed using the assessment system
length of Hospital stay and total expenses were increas- article reduce the ICP, but they have different esthetic and
software (ALTOIDA INC). All significant values reported
ing concerning the severity of the trauma. postoperative outcomes. The choice of the best procedure
for correlation between a higher burden of subconcus-
will depend on what causes intracranial hypertension and
sive events and the ADNS performance were obtained
the patient condition.
Traumatismo cranioencefálico by the Spearman rank, which estimated the coefficient
rho (?) of the respective analyzed measures. Results:
Traumatismo cranioencefálico Subjects performed a series of tests, determined by the
ADNS. 8 athletes (53.3%) performed worse on tests that
assessed planning skills and cognitive processing speed,
despite not reporting complaints in the initial interview.
Statistical analysis showed correlation between higher
number of subconcussive events and poor performance
on ADNS, mainly on tests that assessed cognitive pro-
cessing speed (? = -.689, p < 0,01), and planning skills (?
= -.644, p < 0,01). We also observed that 9 athletes (60%)
met diagnostic criteria for diseases associated with sports
practice. Conclusion: These results show linear correla-
tion between higher exposure to subconcussive events
and worse performance on ADNS. In summary, this study
adds to the literature describing a new neurocognitive
assessment method called Altoida DNS, on assessing
cognitive decline at professional fighters.

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375
ÍNDICE DE AUTORES
Índice de Autores – XXX Congresso Brasileiro de Neurologia

ABATTI, Marcus Vinícius  TL 1105880 ALEXANDRIA, Auzuir Ripardo  TL 1106273


ABBADE, Pedro Fernandes  TL 1105655, TL 1105672, TL 1106272 ALGEMIRO, Juliana Bruneli Secchin  TL 1105314
ABICALAF, Ricardo Souza  TL 1105615 ALGEMIRO, Waldemar Carlos Barros  TL 1105314
ABRANCHES, Gabriela de Paula  TL 1105716, TL 1105799 ALMEIDA, Alexia Carneiro de  TL 1105353, TL 1105647, TL 1105315, TL 1105168,
ABREU, Fernanda Ferreira de  TL 1105412, TL 1105651 TL 1105738

ABREU, Gabriel Queiroz de  TL 1105441 ALMEIDA, Ana Elizabeth Cunha Guimarães de  TL 1105601

ABREU, Thiago Martins de  TL 1105877 ALMEIDA, Ana Raquel Lorena Lindquist  TL 1106490, TL 1106503

ABREU, Vicente Paulo Afonso  TL 1105414 ALMEIDA, An Raquel Lorena Lindquist  TL 1106440

ABREU, Vinícius Pereira de  TL 1105877 ALMEIDA, Brenda Santana  TL 1105519, TL 1105413

ABUCHAHIN, Nabil  TL 1105393 ALMEIDA, Bruna Gonçalves Dantas de  TL 1105961, TL 1105753

ABUD, Daniel Giansante  TL 1106487, TL 1105875 ALMEIDA, Carlos Mauricio Oliveira de  TL 1104707, TL 1105948

ACCIOLY, Amanda Cacaes Modesto  TL 1106059 ALMEIDA, Carlos Mauricio Oliveira  TL 1105487

ADAMO, Chadi Emil  TL 1106258, TL 1106114 ALMEIDA, Carlos Maurício de  TL 1104824, TL 1106647

ADOLPHSSON, Lorena Izabel Esteves  TL 1106380 ALMEIDA, Cynthia von Paumgartten Ribeiro  TL 1106410

ADONI, Tarso  TL 1105703, TL 1105740 ALMEIDA, Daniel Oliveira  TL 1105414

AGOURAKIS, Demetrios Chiuratto Agourakis  TL 1104938 ALMEIDA, Filipe  TL 1105036

AGOURAKIS, Demetrios Chiuratto  TL 1104937, TL 1104938 ALMEIDA, Igor Melo de  TL 1104862, TL 1104940

AGUIAR, Antonio Handerson Lima  TL 1105587 ALMEIDA, Inara Taís de  TL 1105006

AGUIAR, Caroline Nogueira  TL 1106124 ALMEIDA, Isabel Junqueira de  TL 1104692

AGUIAR, Gabrielle Batista de  TL 1105240, TL 1105242 ALMEIDA, Isabella Cristina Guimarães de  TL 1105601

AGUIAR, Gilnard Caminha de Menezes  TL 1106526 ALMEIDA, Jose Artur Costa D’ TL 1106501

AGUIAR, Leandro Santos Franco de  TL 1105759 ALMEIDA, José Artur Costa D’ TL 1106158

AGUIAR, Leandro  TL 1106580 ALMEIDA, Juliana Oliveira de Almeida  TL 1104978

AGUIAR, Nalcer Eduardo Canedo de  TL 1105843 ALMEIDA, Juliana Oliveira  TL 1104833, TL 1105908, TL 1104832

AGUIAR, Rafaela Feitosa  TL 1106431 ALMEIDA, Kelson James  TL 1106621, TL 1106631, TL 1106598, TL 1105909,
TL 1105548, TL 1105653,
AGUIAR, Renato Santana de  TL 1105782 TL 1106575, TL 1105814,
AGUIAR, Tiago Silva  TL 1106311 TL 1105826, TL 1105033,
AGUILAR, Ana C. R.  TL 1106328 TL 1105452, TL 1105731

AGUILAR, Ana Carolina Rodrigues  TL 1106309 ALMEIDA, Laryssa Garcia de  TL 1106127

AGUZZOLI, Arthur  TL 1106049 ALMEIDA, Marcela Patricia de  TL 1106656

AGUZZOLI, Cristiano Schaffer TL 1105542, TL 1105026, TL 1105295, ALMEIDA, Marcele Schettini de  TL 1105905, TL 1105336
TL 1105621 ALMEIDA, Marcele  TL 1105187
AIELLO, Bárbara Pina  TL 1106506 ALMEIDA, Maria Teresa Mamere de  TL 1105599
ALAMY, Alexandre Hofke  TL 1105442 ALMEIDA, Miguel Vieira de  TL 1106251, TL 1106403, TL 1105082, TL 1106567,
ALBUQUERQUE, Ana Letícia Amorim de  TL 1106144 TL 1106192

ALBUQUERQUE, Carolina Rouanet Calvacante de  TL 1105101 ALMEIDA, Nathalia Alves Araújo de  TL 1106168

ALBUQUERQUE, Deborah Inayara Mendes Tenorio de TL 1105315, ALMEIDA, Pedro Ignacio Ferraz Montenegro de  TL 1105495, TL 1105443,
TL 1105804, TL 1105299, TL 1105468, TL 1105473,
TL 1105353, TL 1105822, TL 1105745, TL 1105636
TL 1105647, TL 1105754 ALMEIDA, Pedro Ignacio Ferraz Montenegro de  TL 1105495, TL 1105443,
ALBUQUERQUE, Déborah Inayara Mendes Tenório  TL 1106349 TL 1105468, TL 1105473,
TL 1105745, TL 1105636
ALBUQUERQUE, Flávia Timbó  TL 1106104
ALMEIDA, Rafael de  TL 1105666, TL 1105686, TL 1105699, TL 1105691,
ALBUQUERQUE, Lucas Alverne Freitas de  TL 1106526 TL 1105641
ALBUQUERQUE, Marco Antonio Veloso  TL 1106469 ALMEIDA, Rafael  TL 1105264, TL 1105390
ALBUQUERQUE, Marco Antônio Veloso  TL 1105049 ALMEIDA, Renata Parente de  TL 1106057
ALBUQUERQUE, Maria de Fatima Pessoa Militao de  TL 1106320 ALMEIDA, Renata Santos  TL 1106559
ALBUQUERQUE, Rejane Karla Santana  TL 1105119 ALMEIDA, Samuel Brito TL 1106057, TL 1106217, TL 1106081
ALBURQUERQUE, Dara Lucas  TL 1104891 ALMEIDA, Sephora Sabrina Candido de  TL 1105299, TL 1105274
ALCARAZ, Helena França  TL 1105530 ALMEIDA, Sephora Sabrina Candido  TL 1105738, TL 1106349
ALCÂNTARA, Cássia de  TL 1106132, TL 1106204 ALMEIDA, Sephora Sabrina Cândido de  TL 1105804, TL 1105822
ALENCAR, Emilly Vieira Zuza  TL 1106555 ALMEIDA, Vivian Pinto de  TL 1105438, TL 1105431, TL 1105468, TL 1105473,
ALENCAR, Francisco José  TL 1105861 TL 1105485
ALENCAR, Jéssica Monique Dias  TL 1105006 ALMEIDAL, Igor Melo de  TL 1104862
ALENCAR, Mariana Asmar  TL 1106132, TL 1106204 ALTAÍDE, Rodrigo Alves  TL 1106617
D’ALENCAR, Matheus Silva TL 1106417 ALVARENGA, Igor Camilo Eisemberg  TL 1105092
ALENCAR, Sarah Diogenes TL 1106616, TL 1106461, ALVARENGA, Lara Cristina Rocha  TL 1106161
TL 1106090, TL 1106159, ALVARENGA, Regina Maria Papais  TL 1105921
TL 1106450, TL 1106632,
TL 1106537, TL 1106473, ALVARENGA, Tarcísio Nunes  TL 1105157, TL 1106407, TL 1105144, TL 1105488
TL 1106499 ALVARENGA, Tiago Camilo Eisemberg  TL 1105818, TL 1105092
ALENCAR, Sarah Diógenes TL 1106105, TL 1106162, ÁLVARES, Mônica Maria Costa do Rêgo  TL 1106245, TL 1106173
TL 1106229, TL 1106233, ALVAREZ, Nathalia Raquel Cristaldo  TL 1106329, TL 1106206
TL 1106570, TL 1106620,
TL 1106218, TL 1106280, ALVERNE, Francisco Jose Arruda Mont’ TL 1106434
TL 1106107, TL 1106308, ALVERNE, Francisco José Arruda Mont’ TL 1106461
TL 1106226

377
Índice de Autores – XXX Congresso Brasileiro de Neurologia

ALVES, Amabile Rodrigues  TL 1106198 AMORIM, Iara  TL 1106247, TL 1106129, TL 1106248, TL 1106331
ALVES, Amanda Freitas  TL 1106349, TL 1105754 AMORIM, Lucas Frederico Silveira de  TL 1105645
ALVES, Anna Leticia de Moraes  TL 1104970, TL 1105808, TL 1105836, AMORIM, Lucas Frederico Silveira  TL 1105658
TL 1105387, TL 1105798, AMORIM, Maria Clara Carvalho Silva de  TL 1106447
TL 1105399, TL 1106599
AMORIM, Mary Anne Pasta de  TL 1105527, TL 1105533
ALVES, Antônio Rodrigo Lima  TL 1104889
AMORIN, Maria Clara Carvalho Silva de  TL 1106507
ALVES, Arthur Antônio Freire  TL 1106059
ANDRADA, Anne Elizabeth Ferraz de  TL 1105773
ALVES, Calila Oliveira  TL 1105519, TL 1105563, TL 1105413
ANDRADE, Alisson L  TL 1104775
ALVES, Emily Tainara Miranda  TL 1105448
ANDRADE, Ana Beatriz Oliveira  TL 1106134
ALVES, Fernanda Mara  TL 1105403
ANDRADE, Antonio Carlos Dias  TL 1106428
ALVES, Fernando Henrique Magri  TL 1105133, TL 1105198,
TL 1105083, TL 1105338, ANDRADE, Bianca Mara  TL 1105588
TL 1105366, TL 1105292 ANDRADE, Bruno Montagna  TL 1105194
ALVES, Frederico F A  TL 1105065 ANDRADE, Camile Feijó de  TL 1105085, TL 1105145
ALVES, Frederico Fernandes Alessio  TL 1105338, TL 1105450, TL 1106528 ANDRADE, Daniele Santos  TL 1105469, TL 1105361, TL 1105697, TL 1105823,
ALVES, Frederico Fernandes Aléssio  TL 1105461 TL 1105746
ALVES, Gabriela Tomás  TL 1105586, TL 1105850 ANDRADE, Felipe Cesar Gomes  TL 1105440
ALVES, Gerson Pereira  TL 1105784 ANDRADE, Fernanda Gonçalves de  TL 1105394, TL 1105381
ALVES, Gustavo Maximiano  TL 1105065, TL 1105083, TL 1105338, TL 1105387, ANDRADE, Helen Maia Tavares  TL 1105569, TL 1106271
TL 1106566, TL 1106515 ANDRADE, Isadora Constantini Soares  TL 1105371
ALVES, Jamille Soares Moreira  TL 1106652 ANDRADE, Isadora Constantini Sorares  TL 1105382
ALVES, João Marcus de Lima Brito  TL 1105277, TL 1105717, TL 1105355 ANDRADE, Isadora Constatini Soares  TL 1105104, TL 1105369
ALVES, Karen Fernanda  TL 1106363 ANDRADE, João Brainer Clares  TL 1104942, TL 1105271
ALVES, Leticia Silva  TL 1105881, TL 1105891 ANDRADE, Laise Cristina de Oliveira Rego  TL 1105440
ALVES, Letícia da Silva  TL 1105581 ANDRADE, Lara de Menezes  TL 1105230, TL 1105774, TL 1106559
ALVES, Lorenzo Fernades  TL 1105902 ANDRADE, Ligia Rocha  TL 1105578, TL 1105130, TL 1105255, TL 1105287,
ALVES, Lucas Abner  TL 1105469 TL 1105278
ALVES, Luiziane Maria da Silva  TL 1105597, TL 1105626, TL 1105609 ANDRADE, Marcello Holanda de  TL 1106631, TL 1106379
ALVES, Luiziane Maria Silva  TL 1105728, TL 1105535, TL 1105553, TL 1105749 ANDRADE, Maria Denise Fernandes Carvalho de  TL 1105225
ALVES, Maria Laizy Ribeiro  TL 1105391 ANDRADE, Maria Luiza de Sousa  TL 1105732, TL 1105639, TL 1105061,
TL 1105744
ALVES, Maria Laura de Almeida  TL 1105413
ANDRADE, Mariana Soares Barros de  TL 1106238
ALVES, Mariana Saraiva Bezerra  TL 1106090
ANDRADE, Márcio Azevedo de  TL 1106072
ALVES, Paula Camila  TL 1104731, TL 1106343, TL 1106247, TL 1106408,
TL 1106248 ANDRADE, Nathalie Souza de  TL 1105745, TL 1105636
ALVES, Paula C  TL 1106129 ANDRADE, Nayara Costa  TL 1105769
ALVES, Paula Queiroz  TL 1105324 ANDRADE, Nayara da Costa  TL 1105359, TL 1105839, TL 1106326, TL 1106500,
TL 1105920
ALVES, Pedro Henrique Sirotheau Corrêa  TL 1105307
ANDRADE, Patricia Pereira de  TL 1106473, TL 1106234
ALVES, Pedro Vinicius Brito  TL 1105421, TL 1105378, TL 1105078, TL 1105859,
TL 1105740, TL 1105422 ANDRADE, Pedro Henrique Sousa de  TL 1106023
ALVES, Priscila Natiele Mauricio  TL 1106262, TL 1105318 ANDRADE, Pedro Mafra de  TL 1106181
ALVES-LEON, Soniza Vieira  TL 1105570, TL 1105668, TL 1105757, TL 1105500, ANDRADE, Philip George Glass  TL 1105842, TL 1106437, TL 1105871,
TL 1105269, TL 1105494, TL 1105583
TL 1105607, TL 1105530, ANDRADE, Rafael de Souza  TL 1105693, TL 1105810, TL 1106439,
TL 1105782, TL 1105665 TL 1105748, TL 1106294,
ALVES-LEON, Soniza V  TL 1106168 TL 1105453, TL 1105778
ALVIM, Marina K M  TL 1105215 ANDRADE, Rodrigo Aragão  TL 1105876
ALVIM, Marina Koutsdontis Machado  TL 1106212, TL 1105610, TL 1106353 ANDRADE, Suellen Marinho  TL 1106538
ALVIM, Marina Machado  TL 1106594 ANDRADE, Thays Neri  TL 1105503, TL 1105628, TL 1106357
ALVIN, Marina  TL 1105276 ANDRADE, Tiago Gomes de  TL 1106578, TL 1106638
AMADO-LEON, Luciane Almeida  TL 1105668, TL 1105530 ANDRADE, Ítalo Barros  TL 1105752
AMARAL, Carla Marineli Saraiva do  TL 1106057 ANDRADE-VALENÇA, Luciana Patrizia Alves de  TL 1106208
AMARAL, Gabriel Rivadavia de Farias  TL 1105066, TL 1105114 ANDRAUS, Maria Emilia Cosenza  TL 1106311
AMARAL, Paulo Hermes Lima  TL 1105597, TL 1105626, TL 1105609, ANDRE, Amanda Leticia  TL 1105505, TL 1105606, TL 1105618,
TL 1105728, TL 1105553, TL 1106283, TL 1105171,
TL 1105749, TL 1105535 TL 1105541, TL 1105844,
TL 1105538, TL 1105771
AMARAL, Vanise Campos Gomes  TL 1105445, TL 1105638, TL 1105656,
TL 1105742, TL 1105005, ANDRE, Amanda Letícia  TL 1105831
TL 1105522, TL 1105506 ANDRÉ, Amanda Leticia  TL 1105795, TL 1105367, TL 1106123
AMARAL-CARVALHO, Viviane  TL 1106259 ANGELA, Rita Cassia Oliveira  TL 1105380
AMARANTE, Daniel Fortes  TL 1105617 ANGELIM, Abraao Iuri Medeiros  TL 1104995
AMBRÓSIO, Natália Virgínia de Oliveira  TL 1106554, TL 1106596, TL 1106613, ANGELIM, Abraão Iuri Medeiros  TL 1105169
TL 1106573 ANGELO, Rita Cassia Oliveira  TL 1105252, TL 1105388
AMBRÓSIO, Natália Virgínia Oliveira  TL 1106585 ANGHINAH, Renato  TL 1106282
AMORIM, Ana Karla Jansen de  TL 1106515 ANJOS, Alexandre Gonzaga dos  TL 1105413
AMORIM, Barbara J  TL 1105284 ANJOS, Caroline Souza dos  TL 1105384
AMORIM, Bárbara Juarez  TL 1105724 ANJOS, Stefâni Soares dos  TL 1106392

378
Índice de Autores – XXX Congresso Brasileiro de Neurologia

ANNES, Marcelo  TL 1104860, TL 1105285 ARAÚJO, Luziany Carvalho  TL 1106465


ANTONELI, Fernando  TL 1106328 ARAÚJO, Maria Isabella Farias de  TL 1105753
ANTONIOL, Tiago  TL 1106222 ARAÚJO, Maria Luísa Granja de  TL 1105148
ANTUNES, Douglas E  TL 1104755 ARAÚJO, Marx Lima de Barros  TL 1106065
ANTUNES, Douglas Eulálio  TL 1105307, TL 1105873 ARAÚJO, Natália Rebeca Alves de  TL 1105732, TL 1105061, TL 1105744,
ANTUNES, Gustavo Leite  TL 1104889 TL 1105861, TL 1105870

ANTUNES, Lorena Martins  TL 1104889 ARAÚJO, Nicolas Rodrigues  TL 1105070

ANTUNES, Luiza Silva  TL 1106347 ARAÚJO, Pedro Lucas Grangeiro Batista Sá  TL 1105435

ANZOLIN, Eduardo  TL 1106054 ARAÚJO, Raquel Vassao  TL 1106389

APPIO, Pedro Roa  TL 1106537 ARAÚJO, Rodrigo André de Souza  TL 1106317, TL 1106612, TL 1106398,
TL 1106119
AQUINO, Eduardo R. Pessoa de  TL 1106559
ARAÚJO, Sheila Espírito Santo  TL 1105494
AQUINO, Emanuelle Roberta da Silva  TL 1105198, TL 1105292, TL 1104957
ARAÚJO, Thiago Medeiros Palmeira de  TL 1105693, TL 1105810
AQUINO, Kairo Igor Freitas de  TL 1105639
ARAÚJO, Victor Arthur Soares Costa  TL 1105263, TL 1105858, TL 1105772
ARAGÃO, Afonso  TL 1106296
ARAÚJO, Victor Oliveira  TL 1105848, TL 1106222
ARAGÃO, Gabriel Vianna Pereira  TL 1106572, TL 1106135, TL 1106134,
TL 1106605, TL 1106118, ARAÚJO, Victor  TL 1105847, TL 1105865
TL 1106524, TL 1106556, ARCA, Vitor Maia  TL 1106188, TL 1105734, TL 1105777, TL 1106423,
TL 1106574 TL 1105773, TL 1106397, TL 1106427, TL 1106444, TL 1106465,
ARAGÃO, Marcelo de Melo  TL 1106602 TL 1105237, TL 1106548, TL 1106496, TL 1105125, TL 1105512

ARAKAKI, Rodrigo Oliveira  TL 1105326, TL 1105475 ARNAUD, Davi  TL 1105704

ARALDI, Bianca Barbosa  TL 1105104, TL 1105369, TL 1105371 ARNAUD, Frederico Carlos Sousa  TL 1105704

ARAMBULA, Omar Gurrola  TL 1106329, TL 1106206 ARNAUD, Frederico Carlos  TL 1104844

ARANHA, Mateus Rozalem  TL 1104692 ARRAIS, Joel Perdiz  TL 1106263

ARANTES, Halisson Flamini  TL 1105335 ARROYO, Tatiane Lopes Alves de Jesus  TL 1105505, TL 1105606, TL 1105618,
TL 1106283
ARAS, Roque  TL 1104775, TL 1105672
ARRUDA, Ana Karolina Santana  TL 1105235
ARAUJO, Augusto Righetti Vieira Ferreira de  TL 1105479
ARRUDA, Arnaldo Ribeiro de  TL 1105435
ARAUJO, Davi Farias de  TL 1106414
ARRUDA, Emanuelly Nogueira Gomes de  TL 1105620
ARAUJO, Geovanna Maia da Nóbrega  TL 1106555
ARRUDA, José Arnaldo Mota  TL 1105593
ARAUJO, Illymack Canedo Ferreira de  TL 1105325
ARRUDA, José Arnaldo Motta de  TL 1105435
ARAUJO, Janaina Moraes de  TL 1105521
ARRUDA, Rodrigo Medeiros Barbosa  TL 1106545
ARAUJO, Jorge Ernesto Miyazaki  TL 1105295
ARRUDA, Walter Oleschko  TL 1105790
ARAUJO, Juliana Sousa Soares de  TL 1106538
ARTHUZO, Lorrane Campidelli  TL 1105552, TL 1105293, TL 1105340,
ARAUJO, Lorena Dias  TL 1105390 TL 1106459
ARAUJO, Rafael Santiago  TL 1105320 ARTILHEIRO, Mariana Cunha TL 1105048, TL 1106411, TL 1106416, TL 1106445,
ARAUJO, Raquel Vassão  TL 1106659 TL 1106400
ARAUJO, Roger Santana  TL 1105950 ASPAHAN, Maira Cardoso  TL 1105346, TL 1106364
ARAUJO, Thiago Medeiros Palmeira de  TL 1105748 ASSIS, Natália Diane Ricarte Lins Freitas  TL 1105557
ARAUJO, Álvaro Luiz dos Santos  TL 1105752 ASSIS, Shalom Pôrto de Oliveira  TL 1106238
ARAÚJO, Abelardo de Queiroz  TL 1105568 ASSONE, Tatiane  TL 1106451
ARAÚJO, André Camacho Oliveira  TL 1105511, TL 1106141 ASSUNÇÃO, Darah Fontes da Silva  TL 1106516
ARAÚJO, Caroline Martins de  TL 1106132, TL 1106204 ASSUNÇÃO, Marcos Paulo do Carmo  TL 1105877
ARAÚJO, Cristiano Miranda  TL 1106564, TL 1106590 ATAÍDE, Milene Teixeira  TL 1105570, TL 1105269, TL 1105607
ARAÚJO, Davi Augusto Batista Sá  TL 1106414 ATAÍDE, Rodrigo Alves  TL 1106583
ARAÚJO, David Augusto Batista Sá  TL 1105435, TL 1105316, TL 1106196, ATHAYDE, Natalia Merten  TL 1106271, TL 1106066, TL 1106122
TL 1105949, TL 1106207 atrophy of the hypothenar eminence
ARAÚJO, Davi Farias de  TL 1106581 AUGSTEN, Ilana Werneck  TL 1106281, TL 1106127
ARAÚJO, Denise Soares  TL 1105692 AUGUSTO, Geovana Veronezi  TL 1106249
ARAÚJO, Elidianne Layanne Medeiros de  TL 1106538 AVANSINI, Simoni Helena  TL 1104690
ARAÚJO, Fabíola Mara de Castro  TL 1106379 AVELAR, Wagner Mauad  TL 1105630
ARAÚJO, Francisco  TL 1106252 AVENTURATO, Italo Karmann  TL 1106074
ARAÚJO, Janaina Moraes de  TL 1105197, TL 1105154, TL 1105190, TL 1105232, AVENTURATO, Ítalo Karmann TL 1106312, TL 1106345, TL 1106353, TL 1106607
TL 1105151
AVEZUM, Luisa Pacheco  TL 1105552, TL 1105565, TL 1105843
ARAÚJO, John Fontenele  TL 1105587
AVOLLIO, Isabella  TL 1105056
ARAÚJO, Jorge Ernesto Miyazaki  TL 1105542
AZEVEDO, Helen Maria de  TL 1106380
ARAÚJO, Juliana Lago de  TL 1106442, TL 1106481, TL 1105259, TL 1105812,
TL 1105916, TL 1106078 AZEVEDO, Maria Paula Carvalho  TL 1105863, TL 1105166

ARAÚJO, Juliana Sousa Soares de  TL 1105853 AZEVEDO, Sofia  TL 1105407

ARAÚJO, Lorena Dias  TL 1105666, TL 1105686, TL 1105699, TL 1105264, AZEVÊDO, Tharcilia  TL 1105627
TL 1105691, TL 1105641 BABANI, Hitesh  TL 1105783, TL 1105384, TL 1105890
ARAÚJO, Louisy Carvalho  TL 1106597, TL 1106458 BACELLAR, Aroldo Luiz da Silva  TL 1105767, TL 1106370
ARAÚJO, Louisy Carvalho  TL 1106597, TL 1106458 BADALOTTI, Eduarda Basso  TL 1105477, TL 1105751, TL 1105302, TL 1104977,
ARAÚJO, Luana Larisse de Sousa  TL 1105732, TL 1105061, TL 1105744, TL 1105776
TL 1105861, TL 1105870 BADIA, Bruno de Mattos Lombardi  TL 1104860, TL 1104862, TL 1105226,
ARAÚJO, Lucas Samuel da Silva  TL 1105348, TL 1105423 TL 1105283, TL 1105559
BADIA, Bruno Mattos Lombardi  TL 1105516, TL 1105560

379
Índice de Autores – XXX Congresso Brasileiro de Neurologia

BADIA, Bruno  TL 1105212, TL 1105221 BARRETO, Paula Caracas  TL 1106308


BAESSO, Isadora Bitencourt  TL 1105309 BARRETO, Pedro Olavo Montenegro Stolzmann  TL 1105925
BAETA, Alex Machado  TL 1105615, TL 1105306, TL 1105146, TL 1105793 BARRETO, Rodrigo Lopes  TL 1105692
BAHIA, Jane Lacerda  TL 1105294, TL 1105807, TL 1105216, TL 1105759 BARROS, Alexandre Drayton Maia  TL 1106085
BAHIA, Valéria Santoro  TL 1106259 BARROS, Arthur Prando de  TL 1105990
BAIMA, Camila Bonfanti  TL 1104943 BARROS, Arthur Prando  TL 1105705
BAIMA, José Pedro Soares  TL 1105213, TL 1105358, TL 1106084, TL 1105178, BARROS, Daniele Montenegro da Silva  TL 1106263, TL 1106426
TL 1106082, TL 1106231, BARROS, Daniele Montenegro da Silva  TL 1106263, TL 1106426
TL 1105335, TL 1105222
BARROS, Daniele Montenegro da Silva  TL 1106263, TL 1106426
BAIOCCHI, Otavio Cesar Carvalho Guimarães  TL 1105006
BARROS, Glenda Barbosa  TL 1105283, TL 1105559, TL 1105633, TL 1105516
BALASSIANO, Salim Lazaro  TL 1105130, TL 1105255, TL 1105278, TL 1105578,
TL 1105287 BARROS, Glenda  TL 1105212, TL 1105221
BALDASSO, Guilherme Vassoler  TL 1105765 BARROS, João Gabriel Lucena de  TL 1106239
BALDINO, Vanessa Ferreira  TL 1105235 BARROS, Livio Leite  TL 1106125, TL 1106581, TL 1106431
BALDIVIA, Guilherme Cristianini  TL 1105629 BARROS, Pedro Henrique Ximenes Ramalho  TL 1106598
BALLESTERO, Matheus Fernando Manzolli  TL 1106209, TL 1106214 BARROSO, Cecilia Tizatto  TL 1105656
BALTAZAR, Gabriel Ferri  TL 1106212, TL 1106594 BARROSO, Cecília Tizatto  TL 1105445, TL 1105005
BALTHAZAR, Marcio Luiz Figueredo  TL 1105118, TL 1106237 BARROSO, Luana Lima  TL 1106544
BANDEIRA, Geovanni Guimarães  TL 1105435, TL 1105405, TL 1106335, BARROSO, Luan Monte  TL 1106522, TL 1106484, TL 1106595, TL 1106511
TL 1105949 BARROSO, Pedro Lucas de Souza  TL 1106622
BANDINI, Márcia  TL 1106074 BARSOTTINI, Orlando G.P.  TL 1104731, TL 1105284, TL 1105785, TL 1105545
BANDO, Mauricio Ossamu  TL 1104937, TL 1104938 BARSOTTINI, Orlando Graziani P.  TL 1104979, TL 1105150,
BAPTISTA, João Pedro Ribeiro  TL 1106063 TL 1106635, TL 1104711,
TL 1105166, TL 1106656
BAPTISTA, João Pedro Ribeiro  TL 1106063
BARSOTTINI, Orlando  TL 1106408
BARAUNA, Valerio Garrone  TL 1106531
BASEIO, Maria Clara  TL 1106487, TL 1105867, TL 1106337, TL 1105805,
BARBALHO, Ingridy Marina Pierre  TL 1106384 TL 1105837
BARBALHO, Sâmela Laura da Silva  TL 1106538 BASLER, Ana Karoline  TL 1105299, TL 1105274
BARBOSA, Alana Strucker  TL 1105472, TL 1105131, TL 1105694 BASSO, Josué  TL 1104957
BARBOSA, Ana Carolina Santiago Dias  TL 1105216 BASSO, Luciano Silveira  TL 1106054
BARBOSA, Ana Letícia Batista Leal  TL 1105548, TL 1105653 BASTIANELLO, João Eduardo  TL 1105398, TL 1105408
BARBOSA, Anderson Matheus de Lima  TL 1105363, TL 1105534, TL 1105373 BASTON, Ana Beatriz Marangoni  TL 1105157, TL 1106407, TL 1105144,
BARBOSA, Breno José Alencar Pires  TL 1105138, TL 1105056 TL 1105488
BARBOSA, Caline Almeida  TL 1106492 BASTOS, Deryc Cleyner Piones  TL 1106651, TL 1106578, TL 1106638
BARBOSA, Cinthia Grisolia  TL 1105092 BASTOS, Leticia Alves  TL 1105441
BARBOSA, eduardo  TL 1105130, TL 1105255, TL 1105278 BASTOS, Lêda Maria Veloso  TL 1106222
BARBOSA, eduardo  TL 1105130, TL 1105255, TL 1105278 BASTOS, Mariana Cota  TL 1104832
BARBOSA, Egberto Reis  TL 1104692, TL 1105036 BASTOS, Ricardo Funes  TL 1105154, TL 1105232, TL 1105151
BARBOSA, Egberto R  TL 1105648 BATISTA, Alex de Novais  TL 1106317, TL 1106119
BARBOSA, Fernanda Figueiredo  TL 1105695 BATISTA, Aléx de Novaes  TL 1106398
BARBOSA, Jamana  TL 1106331 BATISTA, Carlos Eduardo  TL 1104957
BARBOSA, Júlia Neves  TL 1105181 BATISTA, Emily de Carvalho  TL 1106583
BARBOSA, K R Nobrega  TL 1106440 BATISTA, Filipe Prohaska  TL 1105631
BARBOSA, Kátia Richelma Nóbrega  TL 1106490, TL 1106503 BATISTA, Rafael João  TL 1106312
BARBOSA, Liandra Rayanne de Sousa  TL 1106431 BATISTA, Rogério da Silva  TL 1106379, TL 1106367, TL 1106369, TL 1106517,
BARBOSA, Liandra Rayanne Sousa  TL 1106125 TL 1106495

BARBOSA, Maira Tonidandel  TL 1105562, TL 1106259 BATISTELLA, Gabriel Novaes de Rezende  TL 1105503, TL 1105628, TL 1106357,
TL 1105483
BARBOSA, Marco Antonio Martins  TL 1105605, TL 1104844
BATISTELLA, Gabriel Novaes Rezende  TL 1105429
BARBOSA, Marcos Castello  TL 1106252, TL 1105206
BAUDLER, Monika  TL 1105660
BARBOSA, Thaís  TL 1106267, TL 1106619, TL 1106591
BAZAN, Rodrigo TL 1105144, TL 1105365, TL 1106348, TL 1105033, TL 1106023,
BARBOZA, Bruno Ricardo Leite  TL 1106577 TL 1106435
BARBOZA, Gisele Regina  TL 1104939 BAZAN, Silméia Garcia Zanati  TL 1106435
BARBOZA, Ingrid Caroline Freitas  TL 1105429 BECCHELLI, José Flávio  TL 1106607
BARBOZA, Vinícius de Noronha  TL 1105155, TL 1105223, TL 1105224 BECHARA, Edson Junior Gonçalves  TL 1106637, TL 1106610
BARILE, João Paulo  TL 1106019, TL 1105274 BECKER, Jefferson  TL 1105398, TL 1105820, TL 1105925, TL 1105479
BARREIRA, Amilton Antunes  TL 1104755 BEKMAN, Evguenia  TL 1106511
BARREIRA, Raphael Palomo  TL 1106157, TL 1106228, TL 1106395, TL 1106167 BELLEZE, Pedro Lucas Kuibida  TL 1105420
BARREIRA, Raphael Palomo  TL 1106157, TL 1106228, TL 1106395, TL 1106167 BELMONTE, Lucas Arone  TL 1105569
BARREIRO, Maria Ercilia Lima  TL 1106004 BELO, Mateus  TL 1106252
BARRETO, Esther Grangeiro  TL 1106229, TL 1106570 BELTRAME, Lucas Bastos  TL 1105988
BARRETO, Hugo Oliveira Polito  TL 1106135, TL 1106134, TL 1106118, BELÉM, Abel Dantas de Figueiredo  TL 1106391
TL 1106524, TL 1106556,
TL 1106574 BENASSI, Anderson Machado  TL 1105422

BARRETO, Ingredy Piton  TL 1106563 BENATI, João Pedro  TL 1105847, TL 1105865

BARRETO, Marina Nolasco Manhaes Gomes  TL 1105401 BENESI, Ricardo Canuto  TL 1106306

380
Índice de Autores – XXX Congresso Brasileiro de Neurologia

BENEVIDES, Maria Luiza  TL 1106257 BEZERRA, Marcillio Holanda  TL 1105119


BENNETT, Jeffrey L  TL 1105040 BEZERRA, Marcio Luiz Escorcio  TL 1105516, TL 1105560
BENÍCIO, Maria Eduarda Teixeira  TL 1105937 BEZERRA, Marcos Eugenio Ramalho  TL 1105734
BERARDI, Gel Roberto Marmitt  TL 1106172 BEZERRA, Marcos Eugênio Ramalho  TL 1106188, TL 1105777, TL 1106423
BERETTA, Luiza de Lima  TL 1105581 BEZERRA, Mylena Gaudencio  TL 1105748, TL 1106633, TL 1105453
BERETTA, Luiza Lima  TL 1105881 BEZERRA, Mylena Gaudêncio  TL 1105810, TL 1106439, TL 1106294,
BERGAMASCHI, Eduardo de Novaes Costa  TL 1105395, TL 1105062 TL 1106603

BERGAMINI, Mariana Mendes  TL 1105843 BEZERRA, Márcio Luiz Escórcio  TL 1105283

BERNADON, Marcia  TL 1105751 BEZERRA, Rodrigo de Paiva  TL 1105556, TL 1105876, TL 1105901

BERNARDES, Anderlanny Moura  TL 1105851, TL 1105856, TL 1105860, BIANCHI, Dante  TL 1104814


TL 1105862 BIANCHI, Washington  TL 1104814
BERNARDES, Andersanny Moura  TL 1105851, TL 1105856, TL 1105860, BICALHO, Albert Louis Rocha  TL 1106126
TL 1105862 BICCA, Yan de Assunção  TL 1106049
BERNARDES, Anderson Moura  TL 1106414, TL 1105490, TL 1105499, BICHARA, Erick Broder  TL 1105263, TL 1105858, TL 1105772
TL 1105501, TL 1105851,
TL 1105856, TL 1105860, BICHUETTI, Denis Bernardi  TL 1105006
TL 1105497, TL 1105862 BIFF, Vinicius  TL 1106156
BERNARDES, Anderssany Moura  TL 1105490, TL 1105499, TL 1105501, BIONDI, Luis Gustavo  TL 1106522
TL 1105497 BITENCOURT, Rafael Mariano  TL 1106293
BERNARDES, Leonardo de Sousa  TL 1105429, TL 1105628, TL 1106357 BITTAR, Roseli Saraiva Moreira  TL 1105198
BERNARDES, Marina Musse  TL 1105542, TL 1105621, TL 1105195 BLAKE, Julia C  TL 1105714
BERNARDES, Wendell  TL 1105824 BO, Carolina Rodrigues Dal  TL 1105097
BERNARDI, Priscila  TL 1105194, TL 1105642 BOBST, Pedro Gadbem  TL 1106126
BERNARDINO, Angela  TL 1106193 BOGEL, Gustav  TL 1106228
BERNARDINO, Marco Andre de Moraes  TL 1105524, TL 1105557 BOLDRINI, Vinicius de Oliveira  TL 1105724
BERNARDINO, Marco Andre de Moraes  TL 1105524, TL 1105557 BOLLI, Fernando von Bock  TL 1105047, TL 1105087, TL 1105088, TL 1105090
BERNARDINO, Marco Andre Moraes  TL 1105102, TL 1106210, BOLZAN, Gabriela  TL 1104685
TL 1105809, TL 1106418,
TL 1105589, TL 1105613 BONADIA, Luciana Cardoso  TL 1104731
BERNARDINO, Marco André de Moraes  TL 1106467, TL 1105743 BONATO, Giuseppe Dick  TL 1105183
BERNARDINO, Marco André de Moraes  TL 1106467, TL 1105743 BONFANDINI, Janine de Carvalho  TL 1106217
BERNARDINO, Marco André Moraes  TL 1105789, TL 1106187, BONFANTE, Sabrina  TL 1106295, TL 1106297
TL 1106365, TL 1105764, BONFIM, Dara Maria de Sá  TL 1106544
TL 1105815, TL 1106184 BONILHA, Patricia Aurea Andreucci Martins  TL 1105507, TL 1106199,
BERNARDINO, Silvya Nery  TL 1105189, TL 1105432 TL 1106279, TL 1106205
BERNARDON, Marcia  TL 1105477, TL 1105787, TL 1105776 BONILHA, Patrícia Aurea Andreucci Martins  TL 1105185, TL 1106278
BERNARDON, Márcia  TL 1105302, TL 1105305, TL 1104977 BONILHA, Patrícia Áurea Andreucci Martins  TL 1105790, TL 1106277,
BERRETA, Luiza Lima  TL 1105891 TL 1106475, TL 1106527
BERTHOLDI, Lucas Oliveira Pinto  TL 1105472, TL 1105264, TL 1105390 BORBA, Antonio Levi Farias  TL 1105732, TL 1105870
BERTO, Beatriz Schmid Dal  TL 1106478 BORBA, Antônio Levi Farias  TL 1105744
BERTO, Beatriz Schmidt Dal  TL 1105196 BORBA, Fabrício Castro de  TL 1106363
BERTOLDO, Hélvia  TL 1105212, TL 1105221, TL 1105283, TL 1105633 BORELL, Wyllians José Vendramini  TL 1105295, TL 1105621
BERTOLUCCI, Paulo Henrique Ferreira  TL 1105726, TL 1106318 BORELLI, Wyllians José Vendramini  TL 1105295
BERTON, Giovanni Gosch  TL 1106315 BORELLI, Wyllians Vendramini  TL 1106298, TL 1105542, TL 1106144
BERTRAND, Júlia Leite Xavier  TL 1106442, TL 1105753, TL 1106078 BORGES, Beatriz Santana  TL 1105689, TL 1105877, TL 1105889, TL 1105902
BESERRA, Susyane Ribeiro  TL 1104966 BORGES, Bruno Rezende Trindade  TL 1105754
BESSA, Marco Antonio do Socorro Marques Ribeiro  TL 1105192 BORGES, Danielle Patrícia  TL 1105109, TL 1106449
BESSA, Matheus Costa  TL 1106234, TL 1106244, TL 1106553, TL 1106218, BORGES, Dimitria Fortes de Oliveira  TL 1105294, TL 1105807, TL 1105216,
TL 1106453, TL 1106159, TL 1105759
TL 1106429, TL 1106501, BORGES, Fernanda Lago de Araújo  TL 1105916
TL 1106502, TL 1106537, BORGES, Fernando Elias  TL 1106179, TL 1106161, TL 1106142
TL 1106236, TL 1106473,
TL 1106434, TL 1106499, BORGES, Grasiely Faccin  TL 1105519, TL 1105563, TL 1105413
TL 1106226, TL 1106452 BORGES, Heloise Helena Silveira  TL 1105103
BETINI, Beatriz Gioppo  TL 1105756, TL 1105366, TL 1105604, TL 1105372, BORGES, Isabella Sabião  TL 1105850, TL 1105873
TL 1106599, TL 1106337 BORGES, Marco Aurelio Fraga  TL 1104805
BETTING, Luiz Eduardo  TL 1106348 BORGES, Otávio  TL 1106023
BEVILAQUA-GROSSI, Deborah  TL 1106654 BORGES, Sirlene Mendes  TL 1106563
BEVILAQUA-GROSSI, Debora  TL 1106655 BORGES, Vanderci  TL 1106100, TL 1105410
BEZERRA, Allef Roberto Gomes  TL 1104833, TL 1105908, TL 1104832, BORGES, Yuri Ferreira Felloni  TL 1105195
TL 1104978
BORGES, Érico Induzzi  TL 1105353
BEZERRA, Augusto Carvalho  TL 1106155
BORTOLUZ, Ricardo Lorenzato  TL 1105571, TL 1106315
BEZERRA, Daniel C  TL 1106319, TL 1106139, TL 1106307, TL 1106360
BOSAIPO, Nayanne B  TL 1105948
BEZERRA, Emilly Holanda  TL 1105391
BOSSONI, Alexandre  TL 1105218
BEZERRA, Gabriel Marinheiro Santos  TL 1105900
BOTELHO, João Bosco Lopes  TL 1104824
BEZERRA, Henrique Alves  TL 1106248
BOTELHO, Victor Luis Peixoto Pereira  TL 1105655, TL 1106272
BEZERRA, Lorena Praia de Souza  TL 1105626

381
Índice de Autores – XXX Congresso Brasileiro de Neurologia

BOTTARO, Karen Isabelle Pontes Duran  TL 1106329, TL 1106206 BRUCKI, Sonia Dozzi  TL 1104692
BOTTARO, Karen Isabelle Pontes Duran  TL 1106329, TL 1106206 BRUCKI, Sonia Maria Dozzi  TL 1106343, TL 1105739, TL 1105056
BRAGA, Carolina  TL 1105897, TL 1106300 BRUCKI, Sônia Maria Dozzi  TL 1105340, TL 1106459, TL 1106241
BRAGA, Gabriel Pereira  TL 1106329, TL 1106206, TL 1105365 BRUM, Igor Vilela  TL 1106246, TL 1105377, TL 1105076
BRAGA, Julia Cordeiro  TL 1106547 BRUNALE, Fernando  TL 1106295, TL 1106297
BRAGA, Lúcia Libanez Bessa Campelo  TL 1105780 BRUNO, Carlos Eduardo Malavasi  TL 1104633
BRAGA, Maria Cynthia  TL 1106320 BRUNO, Danielle  TL 1105276
BRAGA, Vinicius Lopes  TL 1104874 BRUNO, Luísa Bedê  TL 1105107, TL 1105236, TL 1105244
BRAGA, Vinícius Lopes  TL 1104864, TL 1104860, TL 1104862, TL 1105121, BRUSTOLIN, Giovanna Testa  TL 1105617
TL 1104940, TL 1105006, BUARQUE, Lucas Marenga Arruda  TL 1105158, TL 1106274
TL 1105560, TL 1104934,
TL 1104981 BUARQUE, Lucas Marenga de Arruda  TL 1105650
BRAGA-NETO, Pedro  TL 1105934, TL 1105951, TL 1106656, TL 1106604, BUARQUE, Lucas Marenga  TL 1105713
TL 1105933, TL 1106057, BUCHPIGUEL, Carlos Alberto  TL 1104692
TL 1106217, TL 1106626 BUENO, Manoella Guerra Albuquerque  TL 1105314
BRAINER, João  TL 1106287, TL 1104942, TL 1105271 BUENO, Manoella Guerra de Albuquerque TL 1104731, TL 1106408, TL 1105355
BRANCO, André Barbosa Castelo  TL 1105672 BUENO, Manoella Guerra de Albuquerque TL 1104731, TL 1106408, TL 1105355
BRANCO, Bruno Mendes de Carvalho Castelo  TL 1106369 BUENO, Mateus Barros  TL 1105586, TL 1105601, TL 1105510, TL 1105599
BRANCO, Eduardo Silveira Marques  TL 1105472, TL 1105131, TL 1105694 BUFFARA, Leonardo Trindade  TL 1105123, TL 1105134
BRANCO, Lucas de Melo Teixeira  TL 1106378, TL 1105724, BUHLER, Vinicius Slongo  TL 1105751, TL 1105302
TL 1105863, TL 1105296,
TL 1105630, TL 1105564 BURIGO, bruna  TL 1106145, TL 1106167
BRANCO, Lucas de Melo Teixera  TL 1105166 BURIGO, bruna  TL 1106145, TL 1106167
BRANCO, Lucas Melo Teixeira  TL 1105750, TL 1105785 BUROCK, Helena  TL 1104936
BRANCO, Lucas M T  TL 1105284, TL 1105545 BUSNELLO, Sabrina  TL 1106053, TL 1106054
BRANCO, Lucas MT  TL 1105288 BUSTAMANTE, Ana Paula de Goes Louly  TL 1105839
BRANCO, Renan Carvalho Castello  TL 1106447, TL 1106547 BUSTAMANTE, Ana Paula de Góes Louly  TL 1105359, TL 1106326, TL 1106500,
TL 1105920
BRANDAO, Carlos Otavio  TL 1106306
BUSTAMANTE, Ana Paula Góes Louly  TL 1105769
BRANDI, Ana Laura  TL 1106110
BÜHLER, Vinicius Slongo  TL 1105787, TL 1105305, TL 1104977, TL 1105776
BRANDÃO, André Iglesias  TL 1105253
CABEÇA, Hideraldo Luis Souza  TL 1105112
BRANDÃO, Carlos Otavio  TL 1104814
CABOCLO, Luis Otavio  TL 1106106
BRANDÃO, Tatiana  TL 1106139
CABRAL, Felipe Teijeiro  TL 1106610, TL 1106406
BRASIL, Maria das Graças Nunes  TL 1105689
CABRAL, Filipe Laurindo  TL 1105349, TL 1105116
BRASIL, Pedrina Célia  TL 1105692
CABRAL, Pedro Thiago de Cristo Rojas  TL 1105597, TL 1105005
BRAUN, Débora Mendes  TL 1106239
CACHO, Enio Walker Azevedo  TL 1105245, TL 1105692
BRAVIM, Vinicius Teixeira  TL 1105563
CACHO, Roberta de Oliveira  TL 1105692
BRAYNER, Juliane Lima Fonseca  TL 1106493
CACHO, Roberta Oliveira  TL 1105033, TL 1105245, TL 1105391
BREINIS, Paulo  TL 1106122
CAETANO, Andre Santos  TL 1104937, TL 1104938
BRESSANE, Helena Moretti  TL 1106382
CAFÉ, Ana Flávia  TL 1106185
BRINGEL, Sara Arcanjo  TL 1106218
CAGY, Marcelo  TL 1104995, TL 1104996, TL 1105169
BRIONES, Marcelo R. S.  TL 1106328
CALDERARO, Marcelo  TL 1106139
BRIOSCHI, Ricardo  TL 1106212, TL 1106594
CALEFFI, Adilson Luciano  TL 1106363
BRISSON, Rodrigo Tavares  TL 1105921
CALICH, Ana Luisa  TL 1105358
BRITO, Diana V.  TL 1105885, TL 1105471, TL 1105487, TL 1106333
CALIL, Samia Rogatis  TL 1105283, TL 1105559, TL 1105633
BRITO, Lara Albuquerque de  TL 1106359
CALIL, Samia  TL 1105212, TL 1105221
BRITO, Leonard Rocha Fonceca de  TL 1105347
CALIXTO, Renê Ripardo  TL 1106273
BRITO, Leonard Rocha Fonseca de  TL 1105349, TL 1105116
CALLAND, Alessandra  TL 1106586
BRITO, Luiza Barbosa  TL 1105270
CALLEGARI, Fernanda Garcia  TL 1105765
BRITO, Manuelina M.C.  TL 1105948
CAMAPUM, Alessandra Monteiro  TL 1106481, TL 1105916
BRITO, Marcelo Houat de  TL 1106252
CAMARA-SETE, Alexandra Rubim  TL 1106075
BRITO, Maria Lucia  TL 1106320, TL 1105230, TL 1106432
CAMARGO, Carlos Henrique Ferreira  TL 1106475, TL 1106527, TL 1105471
BRITO, Mariana Rabelo de  TL 1106352, TL 1106353, TL 1106607
CAMARGO, Gabriela de Moura  TL 1106203
BRITO, Mariana R de  TL 1105288
CAMARGOS, Sarah Teixeira  TL 1105779, TL 1105852, TL 1106132, TL 1106061,
BRITO, Matheus Oliveira de  TL 1106575 TL 1106190
BRITO, Paulo Santiago de Morais  TL 1105320 CAMELO, Clara Gontiijo  TL 1105158
BRITO, Paulo Santiago Morais  TL 1106557 CAMELO, Clara Gontijo  TL 1105213, TL 1106274, TL 1105768, TL 1105048,
BRITO, Paulo Santiago  TL 1106474 TL 1105049, TL 1105650,
BRITO, Pedro Nascimento Araujo  TL 1106538 TL 1105713, TL 1106175,
TL 1106189, TL 1106270,
BRITO, Pedro Nascimento Araújo  TL 1105853 TL 1106411, TL 1106416,
BRITO, Pedro Vinícius Alves  TL 1105703 TL 1106424, TL 1106436,
BRITO, Raquel Taumaturgo Dias  TL 1105682 TL 1106445, TL 1106047,
TL 1106400, TL 1106255
BRITO-MARQUES, Paulo roberto  TL 1105789
CAMILO, Millene Rodrigues  TL 1105065, TL 1105338, TL 1105450, TL 1105461,
BROCCHI, Beatriz Servilha  TL 1105181 TL 1105574, TL 1106528,
BROCH, Fernanda Nicoli  TL 1105864, TL 1105617 TL 1105717, TL 1105875

382
Índice de Autores – XXX Congresso Brasileiro de Neurologia

CAMINHA, Rafaela Trindade do O  TL 1106555 CARELLO-COLLAR, Giovanna  TL 1106144


CAMOZZATO, Analuiza  TL 1106110 CARLETTI, Carla de Oliveira  TL 1106646
CAMPOREZE, Bruno  TL 1106296, TL 1105646, TL 1105624, CARLOS, Carolina Sales  TL 1106124
TL 1105625, TL 1105619, CARLOS, Karla  TL 1106472, TL 1106316, TL 1106489, TL 1106646, TL 1106657
TL 1105464, TL 1105466
CARMO, Nathalia Borges  TL 1105081, TL 1105568
CAMPOS, Adria Melissa Silva  TL 1105393, TL 1105384
CARMO, Nathália Borges  TL 1105241
CAMPOS, Adriana Rolim  TL 1105164
CARMO, Robercon Alves  TL 1105702, TL 1105580, TL 1105585, TL 1105590
CAMPOS, Ana Luiza Figueiredo  TL 1105370, TL 1105346
CARMO, Tamires Ferreira do  TL 1106245
CAMPOS, Ana Luiza Jácome Franca  TL 1105628
CARNEIRO, Camila Coelho  TL 1106489
CAMPOS, ana paula almeida  TL 1106167
CARNEIRO, Camila de Godoi  TL 1104692
CAMPOS, Anne Larissa Silva  TL 1105384, TL 1105890
CARNEIRO, José Luiz  TL 1106360
CAMPOS, Brunno Machado de  TL 1106312, TL 1106345
CARNEIRO, Maria Luiza Miranda  TL 1104998, TL 1105107, TL 1105145
CAMPOS, Chistiane Monteiro de Siqueira  TL 1105793
CARNEIRO, Regina Coeli Carvalho Porto  TL 1106589, TL 1105900
CAMPOS, Christiane Monteiro de Siqueira  TL 1105615
CARNEIRO, Renan  TL 1105165
CAMPOS, Christiane Monteiro Siqueira  TL 1105657
CARPANEDA, Jessica de Medeiros  TL 1106142
CAMPOS, Eliene Dutra  TL 1106175, TL 1106189
CARPILOVISKY, Paula Köhler  TL 1105090
CAMPOS, Enzo Lustosa  TL 1106214
CARPILOVSKY, Paula Köhler  TL 1105088
CAMPOS, João H. C.  TL 1106328
CARREIRO, Matheus  TL 1105916
CAMPOS, Laura Altomare Fonseca  TL 1106596, TL 1106613, TL 1106573
CARRIJO, Carolina Garcia nunez  TL 1106460
CAMPOS, Maria Clara Paulino  TL 1105656
CARRIJO, Carolina Garcia Nuñez  TL 1105431, TL 1105495,
CAMPOS, Raphaelly Ribeiro  TL 1106188, TL 1105734, TL 1105777, TL 1106423, TL 1105443, TL 1105473,
TL 1105773, TL 1106397, TL 1105485, TL 1105736,
TL 1106444, TL 1106465, TL 1105623, TL 1105636
TL 1105237, TL 1106548
CARRILHO, Paulo Eduardo Mestrinelli  TL 1105531
CAMPOS, Renata Diefenthaeler  TL 1105195
CARVALGO, Larissa Bittencourt  TL 1105130
CAMPOS, Renata Diefenthaler  TL 1105026
CARVALHO, Adriano Araújo  TL 1105245
CAMPOS, Victor Sousa  TL 1105842, TL 1105871, TL 1105583
CARVALHO, Alzira Alves de Siqueira  TL 1106066, TL 1106122, TL 1105031,
CAMPOS, Ádria Melissa Silva  TL 1105890 TL 1105935
CANAL, Grazieli  TL 1105314 CARVALHO, Alzira Alves de Siqueira  TL 1106066, TL 1106122, TL 1105031,
CANDIDO, Vitor Hugo Ferreira  TL 1105402 TL 1105935
CANEDO, Nalcer Eduardo  TL 1105115, TL 1105843 CARVALHO, Alzira Alves Siqueira  TL 1106271
CANELHAS, Stephanie de Freitas  TL 1105443, TL 1105468, TL 1105473, CARVALHO, Ana Beatriz Cavalcante  TL 1105391
TL 1105485 CARVALHO, Ana Raquel Batista de  TL 1105870
CANELHAS, Stephanie de Freitas  TL 1105443, TL 1105468, TL 1105473, CARVALHO, Antonella Brum de  TL 1105026
TL 1105485
CARVALHO, Antonella Brun de  TL 1105820, TL 1105542
CANELLO, Andréia  TL 1104891
CARVALHO, Barbara Maini de  TL 1105615, TL 1105146
CANETE, Luis Alcides Quevedo  TL 1106540
CARVALHO, Barbara Maini  TL 1105306
CANOVA, Alice Berto  TL 1106512
CARVALHO, Benilton  TL 1105276
CANTALICE, Télio Diego  TL 1106118
CARVALHO, Bárbara Maini de  TL 1105793
CANTERI, Andre Luiz  TL 1106480
CARVALHO, Camila Gonçalves Monteiro  TL 1106076
CANTO, Amanda Morato do  TL 1105215, TL 1105276
CARVALHO, Cristiano Sobral de  TL 1106467
CANZIAN, Kássia Braga  TL 1105754, TL 1105629
CARVALHO, Diego Monteiro de  TL 1104824
CANÊDO, Vítor Silveira Reis  TL 1105155, TL 1105223, TL 1105224
CARVALHO, Déborah Emmily de  TL 1105834, TL 1105857
CAPATO, Tamine  TL 1105648
CARVALHO, Eduardo Vital de  TL 1106093, TL 1106377, TL 1105312
CAPELLI, Amanda Henz  TL 1104685
CARVALHO, Ellaine Doris Fernandes  TL 1105225
CAPOBIANCO, João Gabriel Pacetti  TL 1106382, TL 1105761
CARVALHO, Ellen Silva de  TL 1105821, TL 1106487, TL 1105461,
CAPRARA, Ana Letícia Fornari  TL 1105047 TL 1106404, TL 1104970,
CARAMALHO, Monica Ferreira  TL 1106540 TL 1105604, TL 1105805
CARAMELLI, Paulo  TL 1105562, TL 1106259, TL 1106061, TL 1106190 CARVALHO, Ellen Silva  TL 1105867, TL 1106337, TL 1105837
CARDOSO, Alexandra Seide  TL 1105555 CARVALHO, Elmano Henrique Torres de  TL 1105706
CARDOSO, Beatriz Oliveira  TL 1105393 CARVALHO, Fabíola Aureliano  TL 1105763
CARDOSO, Carolina Rocha da Silveira Brügger  TL 1105525 CARVALHO, Fernanda Dalgé de  TL 1105097
CARDOSO, Fernanda  TL 1104633 CARVALHO, Fernanda Martins Maia de  TL 1106616, TL 1106501, TL 1106493,
CARDOSO, Francisco Costa  TL 1106061, TL 1106190 TL 1106632

CARDOSO, Gabriel Souza Dias  TL 1105889 CARVALHO, Fernanda Martins Maia TL 1106105, TL 1106162,
TL 1106234, TL 1106244, TL 1106218, TL 1106453,
CARDOSO, Guilherme Rocha  TL 1105842, TL 1105871, TL 1105583 TL 1106107, TL 1106090, TL 1106159, TL 1106173,
CARDOSO, Luan Felipe de Souza  TL 1104824 TL 1106359, TL 1106429, TL 1106170, TL 1106450,
CARDOSO, Lucas Eduardo Lucena  TL 1106185, TL 1105780 TL 1106502, TL 1106308, TL 1106537, TL 1106236,
TL 1106499, TL 1106226, TL 1106336, TL 1106386,
CARDOSO, Mariana Driemeier  TL 1105430, TL 1105297 TL 1105763, TL 1106105, TL 1106162, TL 1106234,
CARDOSO, Marina Driemeier  TL 1105001, TL 1105792, TL 1105806 TL 1106244, TL 1106218, TL 1106453, TL 1106107,
CARDOSO, Matheus de Melo Aziz  TL 1106317 TL 1106090, TL 1106159, TL 1106173, TL 1106359,
TL 1106429, TL 1106170, TL 1106450, TL 1106502,
CARDOSO, Percilia  TL 1106249, TL 1106331 TL 1106308, TL 1106537, TL 1106236, TL 1106499,
CARDOSO, Tania Aparecida Marchiori de Oliveira  TL 1105555 TL 1106226, TL 1106336, TL 1106386, TL 1105763
CARDOSO, Tânia Aparecida Marchiori de Oliveira  TL 1105863 CARVALHO, Francisco Anderson de Sa  TL 1106633

383
Índice de Autores – XXX Congresso Brasileiro de Neurologia

CARVALHO, Francisco Anderson de Sá  TL 1105810, TL 1106439, TL 1105748, CASTRO, Kaline dos Santos Kishishita  TL 1105812
TL 1106603 CASTRO, Luiz Henrique Martins  TL 1105377, TL 1105950, TL 1106140,
CARVALHO, Francisco Matheus Oliveira de  TL 1105405, TL 1106335, TL 1105076
TL 1106010, TL 1105949 CASTRO, Mariana Jacob de  TL 1104975, TL 1105323
CARVALHO, Gabriela  TL 1106654, TL 1106655 CASTRO, Matheus Nunes Ferreirinha Leite de  TL 1105869, TL 1105710
CARVALHO, Gabriela Torres Alves de  TL 1106262, TL 1105318 CASTRO, Matheus Nunes Ferreirinha Leite  TL 1105462, TL 1105897,
CARVALHO, Gustavo Balthazar da Silveira  TL 1105379 TL 1105741, TL 1106300
CARVALHO, Hyasmin Maria Oliveira  TL 1105381 CASTRO, Milena Fernandes  TL 1105556
CARVALHO, Iago Resende  TL 1105510, TL 1105307, TL 1105599 CASTRO, Renato Ortolani Marcondes de  TL 1105551
CARVALHO, João Jose Freitas de  TL 1106319, TL 1106307 CASTRO, Vinicius Furtado da Silva  TL 1106133
CARVALHO, João José de Freitas  TL 1106360 CASTRO-AFONSO, Luiz Henrique  TL 1105875
CARVALHO, João José Freitas de  TL 1106287, TL 1106139, TL 1106652, CATITO, Murillo Silva  TL 1105503, TL 1105628, TL 1106357
TL 1106215, TL 1106520 CATTO, Marília B  TL 1104775
CARVALHO, Julia Monteiro Barros Pereira  TL 1106605, TL 1106630 CAVAGNOLLO, Marcia Tatsch  TL 1105395
CARVALHO, Larissa Bittencourt de  TL 1105578, TL 1105287 CAVALCANTE, Ana Karolinne Cruz  TL 1105384, TL 1105890
CARVALHO, Larissa Bittencourt  TL 1105255, TL 1105278 CAVALCANTE, Andrea Caroline  TL 1105119
CARVALHO, Larissa Clementino Leite de Sa  TL 1105524, TL 1105557 CAVALCANTE, Caio Oliveira  TL 1105159, TL 1105162
CARVALHO, Larissa Clementino Leite de Sá  TL 1105743 CAVALCANTE, Carlos Eduardo Cordeiro  TL 1106621, TL 1105909,
CARVALHO, Larissa Clementino Leite Sa  TL 1105102, TL 1106418, TL 1105589, TL 1105548, TL 1105653,
TL 1105613 TL 1105452, TL 1105731
CARVALHO, Larissa Clementino Leite Sá  TL 1105789, TL 1106187, CAVALCANTE, Gabriel Montenegro Ribeiro da Silva  TL 1105877, TL 1105889,
TL 1106365, TL 1105764, TL 1105902
TL 1105815, TL 1106184, CAVALCANTE, Isadora de Oliveira  TL 1106375, TL 1106392
TL 1106210, TL 1105809
CAVALCANTE, Klaus Manoel Melo  TL 1105481
CARVALHO, Lays Saraiva Rodrigues  TL 1105511, TL 1105855, TL 1106141,
TL 1106130, TL 1105801, CAVALCANTE, Labibe Manoela Melo  TL 1105481
TL 1106143, TL 1106446, CAVALCANTE, Neyde Alegre de Souza  TL 1105656, TL 1105742, TL 1105522,
TL 1106624 TL 1105816
CARVALHO, Lilian Kriger Ramos de  TL 1105780 CAVALCANTE, Neyde Alegre  TL 1105892
CARVALHO, Luis Felipe Ferreira  TL 1106545 CAVALCANTE, Nícolas Ruan dos Santos  TL 1105335, TL 1105222
CARVALHO, Marcela Botelho de  TL 1105097 CAVALCANTE, Yasmin Silveira  TL 1106587, TL 1105136
CARVALHO, Marcelo Simplicio  TL 1105505, TL 1105606, TL 1106283, CAVALCANTE, Ângelo Antônio Lins  TL 1105826
TL 1105171, TL 1105541, CAVALCANTI, Bruna Melo Holanda  TL 1105198
TL 1105844, TL 1105538,
TL 1105795, TL 1105367, CAVALCANTI, Eduardo Boiteux Uchôa  TL 1105239
TL 1105771, TL 1106123, CAVALCANTI, Hellysson Phyllipe Firmino  TL 1105809, TL 1106418
TL 1105831 CAVALCANTI, Maria Alice Mendonça Gomes  TL 1105589
CARVALHO, Marcelo Simplico  TL 1105618 CAVALCANTI, Maria Alice Mendonça Gomes  TL 1105589
CARVALHO, Maria P A  TL 1105284 CAVALCANTI, Maria Eduarda Almeida  TL 1105326
CARVALHO, Marta Rodrigues de  TL 1105359, TL 1105839, CAVALCANTI, Matheus de Lima  TL 1105882
TL 1106326, TL 1106478,
TL 1106320, TL 1106075 CAVASANA, Gabriel Flamarin  TL 1105472, TL 1105131, TL 1105694
CARVALHO, Marta Rodrigues  TL 1105196 CELESTINO, Isadora Costa  TL 1105850
CARVALHO, Matheus dos Santos do Nascimento  TL 1105148 CENDES, Fernando  TL 1104731, TL 1105555, TL 1106312, TL 1106352,
TL 1105215, TL 1105276,
CARVALHO, Pedro Rubens Araujo  TL 1106229, TL 1106159 TL 1105610, TL 1106212,
CARVALHO, Pedro Rubens Araújo  TL 1106570 TL 1106345, TL 1106594,
CARVALHÊDO, Guilherme Belo  TL 1106078 TL 1105288, TL 1106074,
TL 1106353, TL 1106607
CASANOVA, Cristiane  TL 1106306
CENTENARO, Lígia Aline  TL 1105240, TL 1105242
CASAROTTI, Beatriz  TL 1106279, TL 1106278
CERQUEIRA-SILVA, Thiago  TL 1104775
CASELLA, Marcela Santarelli  TL 1105016, TL 1106602, TL 1106635
CETLIN, Roberto Satler  TL 1105821, TL 1106487, TL 1105836, TL 1105604,
CASIMIRO, Luciana  TL 1105056 TL 1106566, TL 1105399
CASSAROTTI, Beatriz  TL 1105507, TL 1105790, TL 1106199, TL 1106205, CHAGAS, Marcos Nisihara  TL 1106647
TL 1105472, TL 1105185,
TL 1106475, TL 1106527 CHAGAS, Tatiane  TL 1106111
CASSEB, Raphael Fernandes  TL 1106345 CHAGAS, Victor de Moraes  TL 1106423, TL 1106465, TL 1105237
CASTELLO, Rafael Paterno  TL 1106406 CHAGAS, Víctor de Moraes  TL 1105734
CASTELLÓN, Iris Montaño  TL 1105447 CHALEGRE, Paulo Eduardo Sales  TL 1105882
CASTILHOS, Raphael Machado de  TL 1106144 CHAPARIM, Guilherme Robles  TL 1105372
CASTILHOS, Raphael Machado  TL 1106298, TL 1105988, TL 1106068, CHATKIN, Gustavo  TL 1105195
TL 1106110 CHAVES, Bruno Paulo Teles  TL 1105440
CASTRILLO, Bruno Batitucci  TL 1105705, TL 1106455, TL 1105990, TL 1106531 CHAVES, Cleuber Esteves  TL 1105817
CASTRO, Ana Flávia Barbosa de  TL 1106040 CHAVES, Daniel Ribeiro  TL 1105414
CASTRO, André Teixeira Souza  TL 1105702, TL 1105580, TL 1105585, CHAVES, Fabíola Gondim Medeiros  TL 1105414
TL 1105590, TL 1105595 CHAVES, Gabriel Silva  TL 1105557
CASTRO, Gisele Franco  TL 1105414 CHAVES, Márcia Lorena Fagundes  TL 1105660, TL 1106068, TL 1106110,
CASTRO, Jose Daniel Vieira  TL 1106431 TL 1106144
CASTRO, José Daniel  TL 1106539 CHIEIA, Marco Antônio Troccoli  TL 1104862
CASTRO, José Daniel Vieira de  TL 1106103 CHINELATO, Sarah de Souza  TL 1105131

384
Índice de Autores – XXX Congresso Brasileiro de Neurologia

CHIOVATTO, Ellen Yukie Fukuda  TL 1105031 CORRÊA, Beatriz Medeiros  TL 1106395


CHIRANO, Camille Albuquerque Rodrigues  TL 1105597, CORRÊA, Matheus Rodrigues TL 1105061, TL 1106575, TL 1105452, TL 1105731
TL 1105626, TL 1105609, CORRÊA, Vítor Guimarães  TL 1106363, TL 1106378, TL 1106312, TL 1106353
TL 1105728, TL 1105535,
TL 1105553, TL 1105749 CORTEZ, Arthur Fernandes  TL 1105442
CHRISTO, Paulo Pereira  TL 1106659, TL 1106364 COSTA, Adriélle da  TL 1105123
CIMBORA, Daniel  TL 1105040 COSTA, Alberto Luiz Cunha  TL 1104999
COELHO, Arthur da Veiga Kalil  TL 1105804, TL 1105905, TL 1105822 COSTA, Assiria Leite de Azevedo  TL 1106598, TL 1106575
COELHO, Daniel Boari  TL 1106440 COSTA, Assíria Leite de Azevedo  TL 1106621, TL 1105909
COELHO, Daniel B  TL 1106490, TL 1106503 COSTA, Beatriz Amorim da  TL 1106312, TL 1106353
COELHO, Erica Nogueira  TL 1105190 COSTA, Bernardo Gratival Gouvea  TL 1105469
COELHO, Fernando Morgadinho Santos  TL 1104934, TL 1106601, TL 1106618 COSTA, Breno Henrique Gomes da  TL 1105882, TL 1105899
COELHO, Fernando Morgadinho  TL 1106305, TL 1106186, TL 1106100, COSTA, Bruna Klein da  TL 1105904, TL 1106432
TL 1106309, TL 1106313 COSTA, Bruna Klein  TL 1106080
COELHO, Fernando M S  TL 1106328, TL 1106330, TL 1106541 COSTA, Carlos Daniel Miranda  TL 1105861
COELHO, Iara de Sousa  TL 1105062 COSTA, Dayana Dourado de Oliveira  TL 1105259, TL 1106078
COELHO, Murilo Lopes  TL 1106572, TL 1106605, TL 1106630 COSTA, Dennise Lanna Barbosa  TL 1105498, TL 1105508
COELHO-FILHO, Otávio R  TL 1105288 COSTA, Erideise Gurgel da  TL 1106131
COLAÇO, Carolina Ferreira  TL 1106441 COSTA, Felipe Oliveira  TL 1106020, TL 1106563, TL 1106485
COLETTA, Marcus Vinicius Della  TL 1106155, TL 1105506, COSTA, Flavia  TL 1105728, TL 1105535, TL 1105553, TL 1105749, TL 1105824
TL 1105885, TL 1105471, COSTA, Francisco Bruno Santana da  TL 1106223
TL 1105487, TL 1106333
COSTA, George Vasconcelos Calheiros de Oliveira  TL 1105047, TL 1105087,
COLUSSI, Mateus Andres  TL 1106156 TL 1105088, TL 1105090
COMERLATTI, Luiz Roberto  TL 1106246, TL 1106252 COSTA, Greice de Lemos Cardoso  TL 1105054
CONFORTO, Adriana Bastos  TL 1105218, TL 1105336, TL 1105033, TL 1105177, COSTA, Igor Castelo Branco Fontenele  TL 1106339
TL 1105279
COSTA, Italo Darlan Soares  TL 1105391
CONFORTO, Adriana B  TL 1105187
COSTA, Jaderson Costa da  TL 1105195, TL 1105407
CONSTANTE, Eduardo Claus  TL 1104860, TL 1104862, TL 1104874, TL 1105006,
TL 1104934, TL 1104981 COSTA, Larissa Pereira  TL 1106538
CONTE, Gabriella  TL 1106352 COSTA, Laysa Moreira Campos  TL 1105877
COPPIETERS, Lays Katharina Assis  TL 1105658, TL 1105678 COSTA, Leandro Araújo  TL 1105575, TL 1105054, TL 1104800, TL 1106515
CORADINE, Tacio Luis Cavalcante  TL 1105718 COSTA, Leonardo Mesquita  TL 1106359
CORADINE, Tácio Luis Cavalcante  TL 1105121 COSTA, Letícia Silva  TL 1105391
CORADINE, Tácio Luís Cavalcante  TL 1105070 COSTA, Lia Camurça  TL 1106117, TL 1106339
CORAZZA, Luiza  TL 1106247, TL 1106129, TL 1106248, TL 1106331 COSTA, Louise Ferreira Nascimento Pestana da  TL 1105696
CORAZZA, Luíza Alves  TL 1106343 COSTA, Lucio Hakkinen dos Santos Rosa  TL 1105902
CORAZZINI, Roseli  TL 1106066, TL 1106122, TL 1105031, TL 1106271, COSTA, Marcela Camara Machado  TL 1106572, TL 1106388, TL 1106263,
TL 1105935 TL 1106477
CORDEIRO, Anna Beatriz Perdigão  TL 1105428, TL 1105726 COSTA, Maria Paula batalha  TL 1104824
CORDEIRO, Iago Alvino  TL 1105684, TL 1105725 COSTA, Mateus Machado  TL 1106273
CORDEIRO, Isabela Cristina Soares  TL 1105818, TL 1105092 COSTA, Melissa dos Santos  TL 1105812
CORDEIRO, Isabella Rodrigues  TL 1106538 COSTA, Rafael Gomes  TL 1106140
CORDEIRO, Luisa Rodrigues  TL 1106040 COSTA, Rafaella Iughetti da  TL 1106222
CORDELLINI, Marcela Ferreira  TL 1105619 COSTA, Raquel Quimas Molina da  TL 1105066
CORIA, Vivian Romanholi  TL 1105600 COSTA, Raquel Quimas Molina  TL 1104814
CORONATTO, Ligia Henriques  TL 1105483 COSTA, Rebeca Teixeira  TL 1105365
CORONETTI, Fernando  TL 1106407, TL 1105109, TL 1106448, TL 1106449 COSTA, Valéria Oliveira  TL 1106369
CORREA, Beatriz Medeiros  TL 1106145, TL 1106157, TL 1106228, TL 1106167, COSTA, Vanessa Maia  TL 1104805
TL 1106058 COSTA, Victoria Maria Fontenele  TL 1106583
CORREA, Beatriz Medeiros  TL 1106145, TL 1106157, TL 1106228, TL 1106167, COSTA, Victoria Maria  TL 1106617
TL 1106058 COSTA, Vinicius Gabriel Coutinho  TL 1105500, TL 1105269, TL 1105494,
CORREA, Clynton L  TL 1106440, TL 1106490, TL 1106503 TL 1105607
CORREA, Léo Freitas  TL 1106306 COSTA, Émerson  TL 1106204
CORREIA, Alice Rodrigues Pimentel  TL 1106239 COUGO-PINTO, Pedro Telles  TL 1105875
CORREIA, Carolina Cunha  TL 1105102, TL 1105789, TL 1105743, TL 1106271, COUTINHO, Ana Beatriz Luz Barradas  TL 1105548, TL 1105653
TL 1106289 COUTINHO, Artur Martins Novaes  TL 1105950, TL 1106241
CORREIA, Carolina de Cunha  TL 1106419 COUTINHO, Artur Martins  TL 1104692
CORREIA, Debora Leticia  TL 1106042 COUTINHO, Beatriz Beatriz Vieira  TL 1105865
CORREIA, Fabiana Carla dos Santos  TL 1105253, TL 1105309 COUTINHO, Beatriz  TL 1105847
CORREIA, Gabriela Soares  TL 1105761 COUTINHO, Bruno  TL 1105879, TL 1106300
CORREIA, Iverson Silva  TL 1106512 COUTINHO, Leo  TL 1106279, TL 1105185, TL 1106278, TL 1106475
CORREIA, Matheus Maia Gonçalves Bringel  TL 1106117, TL 1106339 COUTINHO, Léo  TL 1105507, TL 1105790, TL 1106199, TL 1106205, TL 1106527
CORREIA, Sofia Gomes  TL 1105107, TL 1105236 COUTINHO, Maria Vitória Menezes  TL 1105326
CORREIA, Thaissa Gianolla Arnaut  TL 1106122 COUTO, Anna Beatriz Feitosa  TL 1105744
CORREIA, Thamyris Vilar  TL 1106563 COUTO, Anna Bheatriz Feitosa  TL 1105732

385
Índice de Autores – XXX Congresso Brasileiro de Neurologia

COUTO, Christian Marques  TL 1104797 DALBEM, Juliane Salter  TL 1105103


COUTO, Karla Oliveira  TL 1106447, TL 1106507 DALLA, Maria Fiorot  TL 1104996
COUTO, Luciana Siuves  TL 1104949 DAMASCENO, Alfredo  TL 1105635, TL 1106378
COUTO, Naiade Andrade  TL 1105854 DAMASCENO, Jéssica Cristina Silveira  TL 1106596, TL 1106613, TL 1106573
COUTO, Pedro Miguel Mendonça  TL 1105881, TL 1105581, TL 1105891 DAMASCENO, Patricia Gomes  TL 1106048
COVALESKI, Anna Paula Paranhos Miranda  TL 1106263, TL 1106120 DAMASCENO, Patricia  TL 1106586
COÊLHO, Hitalo Roberto Araújo  TL 1106598 DAMASCENO, Patrícia Gomes  TL 1106649
COÊLHO, Hitalo Roberto de Araújo  TL 1106575 Daniel Lacerda da Costa
CRAVO, Amanda de Araújo  TL 1105481 Daniel Lacerda da Costa
CREE, Bruce AC  TL 1105040 DANIELSKI, Lucineia Gainski  TL 1106293
CRELIER, Viviane Tavares Carvalho  TL 1105479 DANIELSKI, Lucinéia Gainski  TL 1106491
CREMASCHI, Renata Maria de Carvalho  TL 1106186 DANTAS, Eder Leandro da Silva  TL 1105362, TL 1106404, TL 1105717
CREMONESE, Gabriela da Silva  TL 1106198, TL 1106257, TL 1105208, DANTAS, Gabriel Nunes  TL 1105653, TL 1105814, TL 1105826
TL 1105209 DANTAS, IONY SANTANA Santos  TL 1106170
CRHISTO, Paulo Pereira  TL 1106389 DANTAS, Iury Hélder Santos  TL 1106170, TL 1106291, TL 1106158
CRIPPA, Jose A.S  TL 1105948 DANTAS, Iury Hélder Santos  TL 1106170, TL 1106291, TL 1106158
CRISTINO-FILHO, Gerardo  TL 1105907 DAVICO, Clarissa de Araujo  TL 1105431, TL 1105495, TL 1105443,
CRUZ, Cristiani Rocha Lima  TL 1105076 TL 1105468, TL 1105485,
CRUZ, Gabriel Carvalho Oliveira  TL 1106557 TL 1105719, TL 1105736,
TL 1105623, TL 1105636
CRUZ, João Vitor Nunes Sobreira  TL 1104833, TL 1105908, TL 1104832,
TL 1104978 DAVICO, Clarissa de Araujo  TL 1105431, TL 1105495, TL 1105443,
TL 1105468, TL 1105485,
CRUZ, Juliana Aparecida Braga  TL 1105818 TL 1105719, TL 1105736,
CRUZ, Leonardo Cardoso  TL 1105562 TL 1105623, TL 1105636
CRUZ, Miguel Meira e  TL 1105627 DAVICO, Clarissa de Araújo  TL 1105438
CRUZ, Márcia Waddington  TL 1105269 DAVIDOVICH, Eduardo Rodrigues  TL 1105578
CRUZ, Renato Ramon da  TL 1105461 DAVIDOVICH, Eduardo  TL 1105130, TL 1105255, TL 1105879, TL 1105278
CRUZ, Vinicius Leal Borges da  TL 1106619, TL 1105651, DAVOGLIO, Larissa Maria  TL 1106512
TL 1105819, TL 1106421, DE, Lucas Assis Santos  TL 1106227, TL 1105076
TL 1106428, TL 1106579,
TL 1106591, TL 1106611 DEBASTIANI, Mariana Severo  TL 1105864

CRUZ, Vinicius Leal Borges da  TL 1106619, TL 1105651, DELGADO, Larissa Peres  TL 1105836, TL 1105756, TL 1106404, TL 1104970
TL 1105819, TL 1106421, DELMAR, Paul  TL 1105660
TL 1106428, TL 1106579, DENICOL, Taís Luise  TL 1106491
TL 1106591, TL 1106611
DERING, Lorena Maria  TL 1105466
CRUZ, Vinícius Leal Borges da  TL 1106543
DEULEFEU, Flávio Clemente  TL 1106652
CRUZEIRO, Marcelo Maroco  TL 1105309, TL 1106132
DEUS, Júlia Dantas de  TL 1105449
CUNHA, Amanda Alencar Cavalcanti Carneiro  TL 1106433
DEVENS, Leticia Bassani  TL 1105183
CUNHA, Francisco Marcos Bezerra da  TL 1105046, TL 1106185, TL 1106263
DIAS, Alice Estevo  TL 1104939, TL 1104936, TL 1104937, TL 1104938
CUNHA, Francisco Marcos Bezerra da  TL 1105046, TL 1106185, TL 1106263
DIAS, Aline de Fátima  TL 1105555, TL 1105610, TL 1106212
CUNHA, Giuliano  TL 1105880
DIAS, Aline de Fátima  TL 1105555, TL 1105610, TL 1106212
CUNHA, João Luiz Silva Botelho Albuquerque  TL 1105892
DIAS, Amanda Selvátici dos Santos  TL 1105154, TL 1105151, TL 1105521
CUNHA, Laís Felix Teieira da  TL 1104814
DIAS, Bruna de Freitas  TL 1105430, TL 1105792
CUNHA, Rafael Gonçalves Duarte  TL 1105693, TL 1105748, TL 1105778
DIAS, Bruna Freitas  TL 1105001, TL 1105297, TL 1105806
CUNHA, Vívian Garcia da  TL 1105575
DIAS, Bárbara Sampaio de Souza  TL 1105066
CURY, Denise Maria Menezes  TL 1106484
DIAS, Bárbara Sampaio Souza  TL 1104814
CURY, Italo Felipe  TL 1105490, TL 1105499, TL 1105501, TL 1105851,
TL 1105856, TL 1105860, DIAS, Caio Rodrigues Gomes  TL 1105902
TL 1105497, TL 1105862 DIAS, Camila Moreira Carvalho  TL 1105394, TL 1105270
CURY, Lucas Vergara  TL 1105114 DIAS, Francisco Antunes  TL 1105450, TL 1105574, TL 1105875
CURY, Rubens G  TL 1105648 DIAS, Giovana da Rocha Leal  TL 1105814
CWAJGENBERG, Ilana  TL 1105878 DIAS, Kélia Rejane Santiago  TL 1105216
CYRINO, Lorena Raulik  TL 1106472 DIAS, Lucas Silva  TL 1105666, TL 1105686, TL 1105699,
CÂMARA, Márcio Alex Reis  TL 1105054, TL 1104800 TL 1105264, TL 1105390,
TL 1105691, TL 1105641
CÂMARA, Nair Assunta Antônia Corso  TL 1106261, TL 1106558
DIAS, Paula Zago Melo  TL 1105566, TL 1106531
CÂMARA, Samir  TL 1106091, TL 1106196, TL 1106207, TL 1106273, TL 1106586
DIAS, Ronaldo Maciel  TL 1105702, TL 1105580, TL 1105585,
CÂNCIO, João Felipe Pereira  TL 1106134 TL 1105590, TL 1105839,
CÂNCIO, João Felipe Pereira  TL 1106134 TL 1106500, TL 1106478
CÔRREA, Matheus Rodrigues  TL 1105861 DIAS, Samuel Filippe Motta Martins  TL 1105294
DACACHE, Thais  TL 1104807 DIAS, Sandra Lucia  TL 1105759
DACH, Fabiola  TL 1105821, TL 1104970, TL 1105604 DIBLASI, Ana Luiza Lavrado  TL 1106306
DACH, Fabíola  TL 1106487, TL 1105836, TL 1105387, TL 1106566, TL 1105399, DIEGOLI, Henrique  TL 1105096
TL 1106654, TL 1106655 DINIZ, Deborath Lúcia de Oliveira  TL 1106261, TL 1106558
DAGOSTINI, Carolina Matté  TL 1106053, TL 1106054, TL 1106049 DINIZ, Denise Sisterolli  TL 1105689
DAHER, Maria Beatriz Santos Elias  TL 1105031 DINIZ, Déborath Lúcia Oliveira  TL 1106245
DAHY, Flavia Esper  TL 1106451 DINIZ, Joanisson Gomes  TL 1106474

386
Índice de Autores – XXX Congresso Brasileiro de Neurologia

DINIZ, Joanisson Rubens Gomes  TL 1105320, TL 1105256, TL 1106557 EIRAS, Renata  TL 1105879
DINIZ, Maria Ondina Machado  TL 1106179 EISELE, Roberto Rocha Eisele Rocha  TL 1105848
DIONIZIO, Jeanina Cabral  TL 1105693, TL 1105810, TL 1106439, ELIAS, Victor Vitalino  TL 1106280, TL 1106359, TL 1106450, TL 1106656
TL 1106633, TL 1106294, ENEAS, Caroline Santos  TL 1105359, TL 1105769, TL 1105839, TL 1106326,
TL 1106603, TL 1105778 TL 1106500, TL 1105920
DIPIERE, Natalia  TL 1105587 ERNESTO, Ianka Cristina  TL 1105779, TL 1105852
DIRCKSEN, Nathalye Fernanda Pedrosa  TL 1105367 ESCARSO, Silvia Helena Andrião  TL 1105706
DIRCKSEN, Nathalye Fernanda Pedroso  TL 1105505, TL 1105606, TL 1105618, ESCORCIO-BEZERRA, Marcio Luiz  TL 1104711
TL 1106283, TL 1105171,
TL 1105541, TL 1105844, ESPÍNDOLA, Gisele  TL 1105194, TL 1105409, TL 1105642, TL 1105395
TL 1105538, TL 1105795, ESTANISLAU, Rogério Vale  TL 1106126
TL 1105771, TL 1106123, ESTEPHAN, Eduardo de Paula  TL 1105293, TL 1105565, TL 1105190,
TL 1105831 TL 1106550, TL 1105176,
DISSEROL, Caio César Diniz  TL 1106441 TL 1105739, TL 1106231,
DIÓGENES, Ana Vitória Gabriel  TL 1106461, TL 1106226 TL 1106270, TL 1106436
DIÓGENES, Ana Vitória Gabriel  TL 1106461, TL 1106226 ESTEPHAN, Eduardo P  TL 1104679
DIÓGENES, Armando Diógenes  TL 1106474 ESTEPHAN, Eduardo  TL 1106175, TL 1106189
DIÓGENES, Levi Lucena  TL 1106617 ESTEVES, Laura Helena Poggianella  TL 1106121
DOMINGOS, Francine de Paula Roberto  TL 1105666, TL 1105686, TL 1105699, EVANGELISTA, Júlia Rabêlo  TL 1105548
TL 1105691, TL 1105641 EVANGELISTA, Victor  TL 1104807
DOMINGOS, Francine Paula Roberto  TL 1105264, TL 1105390 Fabricio Silva Pessoa
DOMINGOS, João Pedro  TL 1105537 FAGUNDES, Thales Pardini  TL 1105821, TL 1106404, TL 1106599
DOMINGUES, Pedro de Souza  TL 1105115, TL 1105552, TL 1105565, FAIÇAL, Adriana Virgínia Barros  TL 1106572
TL 1105843 FALAVIGNA, Asdrubal  TL 1106049
DOMINGUES, Pedro Souza  TL 1106459 FALCÃO, Ana Carolina Araújo  TL 1105704
DOMINGUES, Renan Barros  TL 1106295 FALCÃO, Ana Carolina Piauilino Santos  TL 1106237
DOMINGUES, Renan  TL 1106287, TL 1106319, TL 1106139, TL 1106307, FALCÃO, Bruna Tavares  TL 1106631, TL 1105534, TL 1105653, TL 1105826
TL 1106360, TL 1106297
FALCÃO, Fernando Cavalcanti de Sá e Benevides  TL 1106246, TL 1105817
DOMIT, Pietro  TL 1104891
FALCÃO, Gustav Barbosa  TL 1105705, TL 1106455, TL 1105716, TL 1105566,
DONADEL, Camila Derminio  TL 1105277, TL 1105355 TL 1105799
DOODY, Rachelle S  TL 1105660 FALCÃO, Maria Alice Pimentel  TL 1105817
DOSUALDO, Caique Alberto  TL 1105154, TL 1105151 FALCÃO, Renata Maia de Medeiros  TL 1106555
DOURADO, Anédia  TL 1106479 FALSARELLA, Mateus Lopes de Lima  TL 1105029
DOURADO, Henrique Nascimento  TL 1106388, TL 1106572, TL 1106135, FANTIN, Cleiton  TL 1105471
TL 1106134, TL 1106630,
TL 1106118, TL 1106524, FARIA, Bárbara Caroline Dias  TL 1106061
TL 1106556, TL 1106574 FARIA, Christina Danielli Coelho de Morais  TL 1105508, TL 1105594
DOURADO, Mário Emílio  TL 1106263 FARIA, Graziella Aguiar Santos  TL 1106550
DOURADO, Rodrigo  TL 1106580 FARIA, Rodolfo Souza de  TL 1105474, TL 1105029
DOUSSEAU, Gabriella Corrêa TL 1105293, TL 1105340, TL 1106459, TL 1105344 FARIA, Rodolfo Souza  TL 1104831
DUARTE, Ana Carla Campelo  TL 1104824 FARIA, Victor Cardoso de  TL 1106635
DUARTE, Angelo Chelotti  TL 1106637 FARIA, Vinicius Gabriel Coutinho  TL 1105570
DUARTE, Carolina Medina  TL 1105440 FARIAS, Brenno  TL 1106293
DUARTE, Edson Pillotto  TL 1105062 FARIAS, Daiane Pereira de  TL 1105693, TL 1105453, TL 1105778
DUARTE, Joel Fernando Sanabria  TL 1105466 FARIAS, Daniel Santana  TL 1106547
DUARTE, Larissa Araujo  TL 1105570, TL 1105668, TL 1105757, TL 1105269, FARIAS, Igor Braga  TL 1104864, TL 1104860, TL 1104862, TL 1105226,
TL 1105530, TL 1105665 TL 1105283, TL 1105285,
DUARTE, Larissa Araújo  TL 1105607 TL 1105559, TL 1105633,
TL 1104874, TL 1105428,
DUARTE, Larisse Araújo  TL 1105494 TL 1105516, TL 1105560,
DUARTE, Murilo Reverendo  TL 1105379 TL 1106426, TL 1105536
DUCCI, Renata Dal-Prá  TL 1106172 FARIAS, Igor Braga  TL 1104864, TL 1104860, TL 1104862, TL 1105226,
DUPONT, Erick  TL 1105738, TL 1105804, TL 1106349, TL 1105822, TL 1105647 TL 1105283, TL 1105285,
TL 1105559, TL 1105633,
DUTRA, Ana Marina  TL 1106066, TL 1106122, TL 1106271 TL 1104874, TL 1105428,
DUTRA, Bruda Guimarães  TL 1105638 TL 1105516, TL 1105560,
DUTRA, Bruna Guimarães  TL 1105656, TL 1105742, TL 1105005, TL 1105522, TL 1106426, TL 1105536
TL 1105816 FARIAS, Igor  TL 1105212, TL 1105221
DUTRA, Livia Almeida  TL 1105297, TL 1105792 FARIAS, Jonathas Silva  TL 1106437
DUTRA, Lívia Almeida  TL 1105430, TL 1105001, TL 1105806 FARIAS, Julia Barreto de  TL 1106272
EBERHARDT, Camila Moraes  TL 1105197 FARIAS, Júlia Barreto de  TL 1105672
ECCO, Gabriela  TL 1104685 FARIAS, Júlia Barreto  TL 1105655
ECHER, Priscila Altoé  TL 1105575 FARIAS, Lucas Cruz  TL 1105104, TL 1105371
ECKELI, Alan Luiz  TL 1105948 FARIAS, Pollyana Marcela Silva de  TL 1104814, TL 1105770
ECKELI, Alan  TL 1106586, TL 1106647 FARIASL, Igor Braga  TL 1104862
EDMAR, Zanoteli  TL 1104679 FARINHAS, João Gabriel Dib  TL 1105570, TL 1105757, TL 1105269, TL 1105607,
EIRAS, Renata Cavalcanti  TL 1105878 TL 1106168

EIRAS, Renata Cavalvanti  TL 1105869 FAVARETTO, Laís Elizabete  TL 1105617

387
Índice de Autores – XXX Congresso Brasileiro de Neurologia

FAVERO, Gabriella Maria Martins  TL 1106191, TL 1106480 FERREIRA, Camila Carneiro  TL 1106296, TL 1105646, TL 1105624, TL 1105625,
FAÇANHA, João Pedro Barros  TL 1105780 TL 1105619, TL 1105464,
TL 1105466
FEDER, David  TL 1105031, TL 1106271
FERREIRA, Fabiana Veloso  TL 1106596, TL 1106613, TL 1106573
FEDERISI, Alexandre  TL 1106512
FERREIRA, Felipe Rodrigues Marques  TL 1105379, TL 1105324
FEIJO, Carolina Queiroz  TL 1105373
FERREIRA, Francisco Ramon Canale  TL 1105438
FEIJO, Tiago Pinho  TL 1106453, TL 1106493
FERREIRA, Giovanna Mattos  TL 1105336
FEIJÓ, Carolina Queiroz  TL 1105363
FERREIRA, Giselle B. M.  TL 1106333
FEITOSA, Camila Ximenes  TL 1105236, TL 1105244
FERREIRA, Giselle Benevides Monteiro  TL 1105626
FEITOSA, Esther Alencar Araripe Falcao  TL 1106386
FERREIRA, Giselle Benevides  TL 1105471, TL 1105487
FEITOSA, Esther Alencar Araripe Falcão  TL 1106336
FERREIRA, Glauber de Menezes  TL 1106616, TL 1106173, TL 1106466,
FEITOSA, Jhoney Francieis  TL 1104697 TL 1106493, TL 1106502
FEITOSA, Júlia Albuquerque  TL 1106410 FERREIRA, Glauber de Menezes  TL 1106616, TL 1106173, TL 1106466,
FEITOSA, Myllan Eiroa  TL 1105062 TL 1106493, TL 1106502
FEITOSA, Raul Raposo Pereira  TL 1106336 FERREIRA, Glauber de Meneze  TL 1106429
FEITOSA, Raul Raposo  TL 1106386 FERREIRA, Henrique Amancio  TL 1105779, TL 1105758, TL 1105730,
FEITOZA, Pablo Vinicius Silveira  TL 1105728, TL 1105553 TL 1105852, TL 1105849

FELICE, Thais Fresneda  TL 1105805 FERREIRA, Isadora Munik Oliveira  TL 1106203

FELIX, Victor Rodrigo  TL 1104707 FERREIRA, Isadora Santos  TL 1106066

FELLINI, Beatriz Maria Andreoli  TL 1104889 FERREIRA, João Henrique Fregadolli  TL 1105430, TL 1105001, TL 1105297,
TL 1105792, TL 1105806
FERMON, Kellen Paiva  TL 1106245
FERREIRA, Jônatas Wesley  TL 1106617
FERNADEZ, Andre Lopez  TL 1105738
FERREIRA, Kamila Santos  TL 1105875
FERNANDES, André Felipe Oliveira  TL 1106589, TL 1105142
FERREIRA, Lisiane Seguti  TL 1106375, TL 1106390, TL 1106392
FERNANDES, Ariane Lima  TL 1105723
FERREIRA, Lucas Pereira  TL 1106523
FERNANDES, Breno Franco Silveira  TL 1105779, TL 1105758, TL 1105730,
TL 1105852, TL 1105849 FERREIRA, Marcos Ravi Cerqueira  TL 1105770

FERNANDES, Felipe Ricardo dos Santos  TL 1106384 FERREIRA, Maria Fernanda Silva  TL 1105369

FERNANDES, Fábia Lais Cotrim  TL 1106561, TL 1106615 FERREIRA, Maria Lucia Brito  TL 1106320, TL 1105230

FERNANDES, Gabriel Haiek  TL 1106390 FERREIRA, Maria Lúcia Brito  TL 1105774, TL 1106559

FERNANDES, GLORIA JEANE de OLIVEIRA  TL 1105620 FERREIRA, Nadjeda Madjessy Morais Leite  TL 1105532

FERNANDES, Isabela Badan  TL 1105472, TL 1105131, TL 1105694 FERREIRA, Nathalia Barros  TL 1105456

FERNANDES, Isabela Danziato  TL 1105633 FERREIRA, Pedro Thiago Simoes  TL 1105908, TL 1104978

FERNANDES, Isabella Araujo Mota  TL 1106419, TL 1105009, TL 1105600, FERREIRA, Pedro Thiago Simões  TL 1104832
TL 1105010 FERREIRA, Pedro Vitor  TL 1105593, TL 1106010
FERNANDES, José Marcelino Aragão  TL 1106185 FERREIRA, Renata C.  TL 1106328
FERNANDES, João Paulo Moreira  TL 1105586, TL 1105510, TL 1105599 FERREIRA, Robson de Sales  TL 1106105, TL 1106234, TL 1106553, TL 1106493,
FERNANDES, MARIA EDUARDA de OLIVEIRA  TL 1105620 TL 1106632, TL 1106502,
TL 1106499, TL 1106452
FERNANDES, Milena  TL 1105556, TL 1106267, TL 1106619, TL 1105819,
TL 1106428, TL 1106611 FERREIRA, Robson de Sales  TL 1106105, TL 1106234, TL 1106553, TL 1106493,
TL 1106632, TL 1106502,
FERNANDES, Natália Silva  TL 1105483 TL 1106499, TL 1106452
FERNANDES, Regina  TL 1106647 FERREIRA, Wardislau  TL 1105150, TL 1104874, TL 1105006, TL 1104934,
FERNANDES, Renan Amphilophilo  TL 1105530 TL 1106305, TL 1104981
FERNANDES, Renan Amphilophio  TL 1105570, TL 1105668, TL 1105757, FERREIRA, Yngrid Dieguez  TL 1106533, TL 1106471, TL 1106268, TL 1106282
TL 1105500, TL 1105269, FERRO, Bianca de Melo  TL 1105812
TL 1105607, TL 1105782
FIDELIX, Emanuela Coriolano TL 1106412, TL 1106062, TL 1106426, TL 1106133
FERNANDES, Rita de Cássia Leite  TL 1105081, TL 1105921
FIDELIX, Emanuela Coriolano TL 1106412, TL 1106062, TL 1106426, TL 1106133
FERNANDES, Silvio Marcelo Silva  TL 1104889
FIGUEIRA, Lívia Carvalho  TL 1106236
FERNANDES, Tatiana Ribeiro  TL 1105048, TL 1106416, TL 1106400
FIGUEIRAS, Paulo Roberto  TL 1106531
FERNANDES, Tiago  TL 1106511
FIGUEIREDO, Alex Cerqueira Silveira  TL 1106421
FERNANDES, Tiótrefis Gomes  TL 1105519
FIGUEIREDO, Alex de Cerqueira Silveira  TL 1105819, TL 1106543, TL 1106579,
FERNANDEZ, Andre Lopes  TL 1105754 TL 1106591
FERNANDEZ, Andre Lopez  TL 1106019 FIGUEIREDO, Alex de Cerqueira Silveira  TL 1105819, TL 1106543, TL 1106579,
FERRACIOLLI, Sueli Fazio  TL 1105048 TL 1106591
FERRAZ, Henrique Ballalai  TL 1104981, TL 1106100, TL 1105410 FIGUEIREDO, Fernanda Barbosa  TL 1106408
FERRAZ, Laura  TL 1106068 FIGUEIREDO, Fernanda B  TL 1105837
FERRAZ, Maria Aparecida  TL 1105235 FIGUEIREDO, Gabriel Sá  TL 1105142
FERREIRA, Ana Carolina Andorinho de Freitas  TL 1104995, TL 1104996, FIGUEIREDO, Ronaldo Duarte  TL 1105092
TL 1105169 FIGUEIREDO, Savio Daniel Freire de Albuquerque  TL 1106562
FERREIRA, Andre Gustavo Fonseca  TL 1105580 FIGUEIREDO, Thainá Gonçalves Tolentino de  TL 1105767, TL 1106370,
FERREIRA, André Gustavo Fonseca  TL 1105702, TL 1105769, TL 1105417
TL 1105585, TL 1105590, FIGUEIRÊDO, Gabriella Bezerra de  TL 1106244
TL 1105839, TL 1106326,
TL 1106500, TL 1105920 FIGUEREDO, Thaina Gonçalves Tolentino de  TL 1106085

FERREIRA, Bianca  TL 1104955 FILGUEIRA, José Samuel Pereira  TL 1105318

388
Índice de Autores – XXX Congresso Brasileiro de Neurologia

FILGUEIRAS, Raquel Mattos  TL 1105666, TL 1105686, TL 1105699, FIN, Manuella Giusti  TL 1105864
TL 1105264, TL 1105390, FIQUENE, Juliana Cardoso  TL 1105448
TL 1105691, TL 1105641
FIRMINO, Júlia Silva Pinheiro  TL 1106117, TL 1106339, TL 1106544
FILH, Antônio Souza Andrade  TL 1106479
FLORES, José Augusto Rodrigues  TL 1105481
FILHO, Adriano Marcelo Silva  TL 1105569
FONSECA, Adna Karolinne  TL 1105391
FILHO, Alberto Pereira Firmino  TL 1105256
FONSECA, Alulin Tacio Quadro Santos Monteiro  TL 1106270
FILHO, Alexandre Saboia Augusto Borges  TL 1106117, TL 1106339
FONSECA, Alulin Tacio Quadros de Monteiro  TL 1106602
FILHO, Andre Bertola Vanzan  TL 1105712
FONSECA, Alulin Tacio Quadros Santos Monteiro  TL 1106411, TL 1106255
FILHO, André Bertola Vanzan TL 1105438, TL 1105431, TL 1105495, TL 1105442,
TL 1105443, TL 1105485 FONSECA, Alulin Tacio Quadros Santos  TL 1106424
FILHO, Angelo Marcelo Wosniacki  TL 1105751, TL 1105302, TL 1104977 FONSECA, Alulin Tácio Quadros Monteiro  TL 1105768, TL 1105049, TL 1106047
FILHO, Antonio Edvan Camelo  TL 1105335 FONSECA, Alulin Tácio Quadros Santos Monteiro  TL 1105158,
TL 1105602, TL 1105650,
FILHO, Antonio Valterno Nogueira Pinheiro  TL 1106117 TL 1105713, TL 1106436
FILHO, Antônio Valterno Nogueira Pinheiro  TL 1106339 FONSECA, Ana Monize Ribeiro  TL 1106209
FILHO, Augusto Celso Scarparo Amato  TL 1105635 FONSECA, Barbara da Silva  TL 1105336
FILHO, Bruno Henrique Carneiro Costa  TL 1106423, TL 1105237, TL 1106548 FONSECA, Bruno Henrique de Souza  TL 1106008, TL 1106023, TL 1106435
FILHO, Carlos Gonzaga Melo  TL 1105259 FONSECA, Bárbara Silva da  TL 1105187
FILHO, Carlos Henrique Ferreira Ramos  TL 1105668, TL 1105530 FONSECA, Camilla Souza  TL 1105324
FILHO, Cicero Gilmário Alves Pereira de Lima  TL 1105107 FONSECA, Clarice Neuenschwander Lins de Morais  TL 1106320, TL 1106559
FILHO, Cícero Gilmário Alves Pereira de Lima  TL 1105236, TL 1105244 FONSECA, Elvis Oliveira  TL 1105898
FILHO, Flavio Moura Rezende  TL 1105726 FONSECA, Elvis Oliveira  TL 1105898
FILHO, Flavio Vieira Marques  TL 1105115, TL 1105565 FONSECA, Igor Oliveira  TL 1105157, TL 1106407, TL 1105144, TL 1105488
FILHO, Flávio Teles de Farias  TL 1106578, TL 1106638 FONSECA, Luisa Almeida  TL 1105235
FILHO, Flávio Vieira Marques  TL 1105552, TL 1105843, TL 1105739 FONSECA, Thaís Laura Almada da  TL 1105420
FILHO, George Gláucio Carneiro Leão de Guimarães  TL 1106583 FONSÊCA, Aleika Lwiza Alves  TL 1106384
FILHO, Gerardo Cristino  TL 1105272, TL 1105419 FONTANA, Pedro Nogueira  TL 1105102, TL 1105789, TL 1105743, TL 1106271,
FILHO, Hildo Rocha Cirne de Azevedo  TL 1105895, TL 1105182 TL 1106289
FILHO, Jamary Oliveira  TL 1105651, TL 1105747 FONTANINI, Carlos Eduardo Massote  TL 1105450
FILHO, Jarbas Sá Roriz  TL 1106081 FONTANINI, Carlos Eduardo Massoti  TL 1105461
FILHO, Jose Marcos Vieira Albuquerque  TL 1106635 FONTENELE, Victoria  TL 1105865
FILHO, Jose Marcos Vieira de Albuquerque  TL 1106602 FONTES, Juliana de Alencar  TL 1105412, TL 1105651, TL 1106591, TL 1106611
FILHO, Jose Rosemberg Lima  TL 1105717 FONTES, Juliana de Alencar  TL 1105412, TL 1105651, TL 1106591, TL 1106611
FILHO, José Anchieta Rodrigues  TL 1106589 FONTES-DANTAS, Fabrícia Lima  TL 1105782
FILHO, José Joaquim Laurindo  TL 1106603 FONTOURA, Paulo  TL 1105660
FILHO, José Marcos Vieira Albuquerque  TL 1105602 FORMIGA, Alissa Elen Moura  TL 1106048
FILHO, José Marcos Vieira de Albuquerque  TL 1104981 FORMOSO, Carolina Rodrigues  TL 1105988
FILHO, José Rosemberg Costa Lima  TL 1105574, TL 1105836, TL 1106528, FORTES, Henry Martins Soares  TL 1105834, TL 1105857
TL 1105277, TL 1105355 FORTINI, Ida  TL 1105377, TL 1105950, TL 1106140, TL 1105076, TL 1105218
FILHO, Luciano Barroso de Albuquerque  TL 1106152 FORZA, Valeska Julio  TL 1105314
FILHO, Lucio Huebra Pimentel  TL 1106309, TL 1106330 FOSS, Norma T  TL 1104755
FILHO, Luiz Alves Ferreira  TL 1105689 FRADE, Marco Andrey  TL 1104755
FILHO, Lázaro Freire Silva  TL 1105658, TL 1105678 FRAGA, Luiza Gonçalves  TL 1106296, TL 1105646, TL 1105624, TL 1105625,
FILHO, Marcos Baruch Portela  TL 1105469, TL 1105361, TL 1105412, TL 1105619, TL 1105464,
TL 1105651, TL 1105697, TL 1105459, TL 1105466
TL 1105823, TL 1105746 FRAGOMENI, Manuela de Oliveira  TL 1106432
FILHO, Marcílio Ferreira de Paiva  TL 1105913, TL 1105874 FRAGOSO, Vanessa  TL 1106432
FILHO, Marcílio José de Oliveira  TL 1106317, TL 1106398, TL 1106119 FRAIMAN, Bruno  TL 1105408
FILHO, Marcílio José Oliveira  TL 1106612, TL 1106289 FRAIMAN, Pedro Henrique Almeida  TL 1105148
FILHO, Max Luiz Mendes Ramires  TL 1106181, TL 1106651, TL 1106578, FRANCISCO, San  TL 1105026, TL 1105621
TL 1106638
FRANCO, André Silva  TL 1105222
FILHO, Pedro Augusto Sampaio Rocha  TL 1105777, TL 1106400
FRANCO, Breno  TL 1106580, TL 1105779, TL 1105758, TL 1105730, TL 1105852,
FILHO, Raimundo Nonato Marques  TL 1105348, TL 1105423, TL 1105653 TL 1105849
FILHO, Renato Evando Moreira  TL 1106185 FRANCO, Clelia Maria Ribeiro  TL 1105613
FILHO, Rui Kleber do Vale Martins  TL 1105798, TL 1106599 FRANCO, Clélia Maria Ribeiro  TL 1106397
FILHO, Rui Kleber Martins  TL 1105875 FRANCO, Isadora Loiola  TL 1104831
FILHO, Rui Kleber Vale Martins  TL 1105450, TL 1106528 FRANGE, Cristina  TL 1106305, TL 1106330, TL 1106541
FILHO, Rui Kléber do Vale Martins  TL 1105574 FRANKLIN, Gustavo Leite  TL 1106475
FILHO, Rui Kléber Vale Martins  TL 1105461 FRANÇA, Adriele Ribeiro  TL 1106572
FILHO, Sileno Fortes  TL 1105890 FRANÇA, Daise Larissa Ribeiro  TL 1106572
FILHO, Stelio da Conceição Araújo  TL 1106526 FRANÇA, Gustavo Sales  TL 1105684, TL 1105725
FILHO, Valmir Vicente  TL 1106318 FRANÇA, Gêneton Romualdo de  TL 1106294
FILHO, Velko Veras Pereira de Matos  TL 1106339 FRANÇA, Helena Ferreira  TL 1105668
FILHO, Victor de Menezes Carvalho  TL 1106559 FRANÇA, Laíse Carolina  TL 1105782
FILPO, Alessandra  TL 1106172 FRANÇA, Marcondes C  TL 1105785, TL 1105288, TL 1105545

389
Índice de Autores – XXX Congresso Brasileiro de Neurologia

FRANÇA, Monalisa Silva  TL 1105391 TL 1105914, TL 1106197,


FRANÇA, Ítalo  TL 1105784 TL 1106216, TL 1106219

FRATINI, Paula  TL 1106271 FROTA, Norberto  TL 1106606

FRAZAO, Frederico Del Duca  TL 1104814 FRÖHLICH, Alan Christimann  TL 1105571, TL 1106315

FRAZÃO, Frederico Del Duca  TL 1105066, TL 1105114 FUKASE, Carine Luri  TL 1106186

FREGONEZI, Guilherme Augusto de Freitas  TL 1106062 FUKUDA, Bruno Barco  TL 1106209

FREGONEZI, Raísa Laisner  TL 1106193 FUKUDA, Thiago Gonçalves  TL 1106507, TL 1105412, TL 1105651,
TL 1105819, TL 1106421,
FREIRE, Bruna Franchito  TL 1105569 TL 1106579, TL 1106611
FREIRE, Diana Pessoa Silva  TL 1105320, TL 1105256 FULADOR, Beatriz  TL 1105382, TL 1105104, TL 1105369, TL 1105371
FREIRE, Lukas Santos  TL 1105842, TL 1105871, TL 1105583 FUSSIGER, Helena Helena  TL 1106256
FREIRE, Magda de Melo  TL 1106120 FUSSIGER, Helena Tairine  TL 1106080
FREITAS, Ana Carolina Andorinho de  TL 1104995, TL 1104996, TL 1105169, FUSSIGER, Helena  TL 1105880, TL 1105904, TL 1106256
TL 1105770
FUSTES, Otto Jesus Hernandez  TL 1106172
FREITAS, Arthur Batista de  TL 1105046, TL 1104966
FÉLIX, Iuri F  TL 1104775
FREITAS, Aston Alves de  TL 1105791
FÉLIX, Rafaela Silva  TL 1106220, TL 1106488
FREITAS, Débora Viana  TL 1105698, TL 1105719, TL 1105636
FÉRRER, João Victor Cabral Correia  TL 1105121
FREITAS, Fernando Cini  TL 1105409
GABAY, Daniel Moreira  TL 1105121
FREITAS, Franklin Tertulino  TL 1105207
GADENZ, Sabrina  TL 1104957
FREITAS, Hermany Capistrano  TL 1106280, TL 1106537, TL 1106452
GAGLIARDI, Rubens José  TL 1106610
FREITAS, Ivan Guerra  TL 1106586
GALDINO, Pedro Arnaud  TL 1106553, TL 1106608, TL 1106623
FREITAS, Julian Leticia  TL 1106331
GALEGO, Andressa Regina de Mello  TL 1105197, TL 1105154, TL 1105232,
FREITAS, Julian Letícia de  TL 1106343 TL 1105151
FREITAS, Julian Letícia  TL 1105344 GALEGO, Andressa Regina  TL 1105521
FREITAS, Júlian Letícia de  TL 1105552, TL 1105843 GALHARDO, Helena Machado  TL 1105394
FREITAS, Júlian Letícia  TL 1106459, TL 1106247, TL 1106249, TL 1106129, GALHARDONI, Ricardo  TL 1106367, TL 1106517
TL 1106248
GALVÃO, Gustavo Fontoura  TL 1105500
FREITAS, Livia  TL 1106606
GALVÃO, Heloisy Maria Nunes  TL 1106545
FREITAS, Luiza Ramos de  TL 1105483
GALVÃO, Maria Lourdes Souza  TL 1105535, TL 1105749
FREITAS, Mariana Carmo  TL 1105645, TL 1105658, TL 1105678, TL 1105449
GALVÃO, Wagner Rodrigues  TL 1105498, TL 1105508, TL 1105594, TL 1105465
FREITAS, Ricardo Góes  TL 1105395
GAMA, Maria Thereza Drumond  TL 1106656
FREITAS, Tereza Brenda Clementino de  TL 1105155, TL 1105223, TL 1105224
GAMA, Mylana Dandara Pereira  TL 1106184
FREITAS, Thiago Holanda  TL 1106081, TL 1106217
GAMA, Natália Araújo Sundfeld da  TL 1106132
FREUA, Fernando  TL 1106082
GAMA, Rayanderson Nunes da  TL 1106447, TL 1106507
FREUA, Fernando  TL 1106082
GAMA, Vitor Carneiro de Vasconcelos  TL 1104780
FREZATTI, Rodrigo Siqueira Soares  TL 1105342, TL 1106512, TL 1105247,
TL 1105540, TL 1105547, GAMBIRASIO, Bruna Gutierres  TL 1105150, TL 1106357
TL 1105781, TL 1105592, GAMBIRÁSIO, Bruna Gutierres  TL 1105428
TL 1105277, TL 1105167, GAMELEIRA, Fernando Tenório  TL 1104832, TL 1104978
TL 1105355, TL 1105509
GARCIA, Gabriel Palomares  TL 1106375
FREZATTI, Rodrigo Siqueira  TL 1105695
GARCIA, Isadora Martins  TL 1105394
FREZATTI, Rodrigo Soares  TL 1105588
GARCIA, Jose Carlos Teixeira  TL 1106287
FREZATTI, Rodrigo S  TL 1105867, TL 1105805, TL 1105714, TL 1105837
GARCIA, Leonardo Peixoto  TL 1105586, TL 1105850, TL 1105599, TL 1105873
FREZATTI, Rodrigo  TL 1105808
GARCIA, Maria Teresa Fernandes Castilho  TL 1106042
FREZATTI, Tomásia Oliveira de Holanda Monteiro  TL 1106512
GARDONA, Rodrigo Galvão Bueno  TL 1105285, TL 1105536
FRIGIERI, Gustavo Henrique  TL 1106316, TL 1106489
GARIBE, Raíssa  TL 1106580
FRIGO, Yasmim Nadime José  TL 1106413, TL 1106448
GASPAR, Renata Coelho Chaves  TL 1105528, TL 1105576
FRIZZO, Luana  TL 1105684, TL 1105725
GASPAR, Safira de Brito  TL 1106604, TL 1106626
FROEHNER, Giullia Victória  TL 1105122, TL 1105124
GAVA, Fernanda Frederico  TL 1106491
FROTA, Aline Xavier  TL 1106460
GAVA, Fernanda  TL 1106293
FROTA, João Gabriel Dias Brasiliense  TL 1106553, TL 1106616, TL 1106218,
TL 1106461, TL 1106173, GAVA, Thiago Voigt  TL 1105177
TL 1106501, TL 1106493, GAVA, Tiago Voigt  TL 1105279
TL 1106226 GEISLER, Aline  TL 1105448
FROTA, Noberto Anizio Ferreira  TL 1106234, TL 1106236 GENEROSO, Jaqueline Silva  TL 1106293
FROTA, Noberto Anízio Ferreira  TL 1106466 GERALDIS, Jaqueline Cruz  TL 1105215
FROTA, Norberto Anizio Ferreira  TL 1105455, TL 1106244, GERALDIS, Jaqueline  TL 1105276
TL 1106616, TL 1106453,
TL 1106159, TL 1106450, GERMINIANI, Francisco Manoel Branco  TL 1105507, TL 1106279, TL 1106278,
TL 1106493, TL 1106502, TL 1106564, TL 1106590
TL 1106537, TL 1106473, GERMINIANI, Francisco Manoel Branco  TL 1105507, TL 1106279, TL 1106278,
TL 1106656, TL 1106434, TL 1106564, TL 1106590
TL 1106499, TL 1106336, GERVÁSIO, Daniel Monte de Andrade  TL 1106251, TL 1106403
TL 1106386, TL 1106452
GESU, Amanda Di  TL 1105474
FROTA, Norberto Anízio Ferreira  TL 1106105, TL 1106218, TL 1106090,
TL 1106173, TL 1106429, GHANI, Luana Miler  TL 1106463
TL 1106291, TL 1104780, GHIZONI, Enrico  TL 1105215
GIAFFERI, Carlos  TL 1106295, TL 1106297

390
Índice de Autores – XXX Congresso Brasileiro de Neurologia

GIFFONI, Silvyo  TL 1104772 GONÇALVES, Daniel Buzaglo  TL 1105005


GIFONI, Angela Rodrigues  TL 1106064, TL 1106091 GONÇALVES, Isabelle Carvalho  TL 1105780
GIOIA, Carolina Gabriela Divino Soares  TL 1105902 GONÇALVES, Marcelo Rodrigues  TL 1106068
GIONEDIS, Murillo Cesar  TL 1106049 GONÇALVES, Marcia Rubia Rodrigues  TL 1105950, TL 1105076
GIORDANI, Manuella Edler Zandoná  TL 1106144 GONÇALVES, Marcus Vinicius Magno  TL 1106156
GISOLDI, Emerson  TL 1106533, TL 1106471, TL 1106406 GONÇALVES, Maria de Jesus  TL 1106062
GITAÍ, Livia leite Goes  TL 1105908 GONÇALVES, Márcia Rubia Rodrigues  TL 1106140
GITAÍ, Lívia Leite Goes  TL 1106578, TL 1106638 GONÇALVES, Márcia Rúbia Rodrigues  TL 1105377
GITAÍ, Lívia Leite Góes  TL 1105070 GONÇALVES, Raíssa Amorim  TL 1105051
GIUBERTI, Beatriz Astun  TL 1106657 GONÇALVES, Trajano Aguiar Pires  TL 1105263, TL 1106528, TL 1106404,
GLEIZER, Rene de Araujo  TL 1105097 TL 1106599, TL 1105277,
TL 1105858, TL 1105355
GODINHO, Caio Fagundes  TL 1105270
GONÇALVES, Trajano  TL 1105717
GOEBEL, Gabriella Cruz  TL 1104697
GONÇALVES, Victoria Veiga Ribeiro  TL 1105421, TL 1105703, TL 1105378,
GOES, Cristiana Pessoa de Queiroz Faria  TL 1105101 TL 1105078, TL 1105859,
GOES, Idna Lara Sena de  TL 1105791 TL 1105740, TL 1105422
GOES, Julieta Sobreira  TL 1106111, TL 1105823 GOULART, Isabela M. B.  TL 1104755
GOIS, Weslley Medeiros  TL 1106523 GOULART, Isabela Maria Bernardes  TL 1105850, TL 1105510, TL 1105307,
GOLDSCHMIDT, Leila Delorme  TL 1104996 TL 1105873

GOMES, Ana Carolina Dias  TL 1106309 GOULART, Thiago Oscar  TL 1105450, TL 1105461, TL 1105574, TL 1106528

GOMES, Andryel de Morais  TL 1105853 GOUVEA, Elisa Gutman  TL 1105431, TL 1105495, TL 1105668,
TL 1105443, TL 1105473,
GOMES, André Borges Ferreira  TL 1106245, TL 1106173 TL 1105698, TL 1105736,
GOMES, Bruno Teixeira  TL 1104999 TL 1105745, TL 1105623
GOMES, Claudia do Nascimento  TL 1106563 GOUVEA, Silmara  TL 1105695
GOMES, Emanuel Assis Bertolino Martins  TL 1105316 GOUVÊA, Sabrina Vechini  TL 1105765, TL 1106237
GOMES, Emanuel de Assis Bertulino Martins  TL 1105405, TL 1105593, GRACIANO, Ana Carolina  TL 1105347
TL 1106335, TL 1105949 GRADVOHL, Letícia Brazil  TL 1104998, TL 1105085
GOMES, Fernando Cotrim  TL 1105216, TL 1105138 GRAMASCO, Hendrick Henrique Fernandes  TL 1106413, TL 1106448
GOMES, Flávia Carvalho Alcantara  TL 1105494 GRAMASCO, Hendrick  TL 1106449
GOMES, Gustavo Rodrigues Ferreira  TL 1106391, TL 1106526, TL 1106376, GRANJA, Valeria Nogueira Tobias  TL 1106445
TL 1106381, TL 1106396,
TL 1106525, TL 1106641 GRAUPNER, Roberta Sena  TL 1105005

GOMES, Gustavo Rodrigues  TL 1106539 GRAUPNER, Roberta Sena  TL 1105005

GOMES, Helio Aquaroni Farao  TL 1105157 GRAÇA, Felipe Franco da  TL 1106302, TL 1105863

GOMES, Helio Aquaroni Farão  TL 1106407 GRENFELL, Mariana Lacerda Reis  TL 1105716, TL 1105799

GOMES, Helio Rodrigues  TL 1105688 GREVET, Laura Tietzmann  TL 1105925

GOMES, Hélio Rodrigues  TL 1105076 GRIZA, Rafaela Luiza Altheia  TL 1105477, TL 1105751, TL 1105302, TL 1104977

GOMES, Irenio  TL 1105089, TL 1105095 GROSSKLAUSS, Luis Fernando  TL 1106551

GOMES, Jose Carlos Teixeira  TL 1106307 GUARDA, Suzete Nascimento Farias da  TL 1105033

GOMES, José Carlos Teixeira  TL 1106319 GUARDA, Suzete Nascimento Farias da  TL 1105033

GOMES, José Ítalo da Silva Damasceno Gomes  TL 1105107 GUAZZELLI, Sofia Brunchport  TL 1105562

GOMES, José Ítalo da Silva Damasceno  TL 1105107, TL 1105244 GUBEISSI, Mariana Campos  TL 1106472

GOMES, João Pedro Izidoro  TL 1105197, TL 1105154, TL 1105190, TL 1105232, GUEDES, Bruno Fukelmann  TL 1105076
TL 1105151, TL 1105521 GUEDES, Matheus da Costa  TL 1106234, TL 1106616, TL 1106461,
GOMES, Marcos Vinícius Bertoldo  TL 1105861 TL 1106453, TL 1106107,
TL 1106159, TL 1106501,
GOMES, Marcus Vinícius  TL 1105837 TL 1106493, TL 1106632,
GOMES, Paulyane Thalita Miranda  TL 1106389, TL 1106659 TL 1106502, TL 1106434
GOMES, Rafael Henrique Neves  TL 1105253 GUEDES, Matheus da Costa  TL 1106234, TL 1106616, TL 1106461,
GOMES, Samila Marissa Pinheiro  TL 1105155, TL 1105223, TL 1105224 TL 1106453, TL 1106107,
TL 1106159, TL 1106501,
GOMES, Victor Hugo Souza Silva  TL 1105382, TL 1105104, TL 1105369, TL 1106493, TL 1106632,
TL 1105371 TL 1106502, TL 1106434
GOMES, Vitor Picanço Lima  TL 1105571, TL 1106315 GUERRA, Davi Veloso  TL 1106597, TL 1105778, TL 1106458, TL 1106588
GOMES, Vlademir Carneiro  TL 1104998, TL 1105085, TL 1105145, TL 1105236 GUERRA, Davi Veloso  TL 1106597, TL 1105778, TL 1106458, TL 1106588
GOMEZ, Rodrigo Santiago  TL 1105370, TL 1105779, TL 1105758, TL 1105730, GUERRA, Felipe Borelli Del  TL 1105769, TL 1105336
TL 1105852, TL 1105849
GUERRA, João Victor Lage  TL 1105779, TL 1105758, TL 1105730, TL 1105852,
GONDIM, Francisco de Assis Aquino  TL 1106185, TL 1105780 TL 1105849
GONDIM, ISABELA LUZINI  TL 1105084 GUERRA, Pedro Lucas Negromonte  TL 1106427, TL 1106496, TL 1105125,
GONDIM, PEDRO ANGELO LUZINI  TL 1105084 TL 1105512
GONZALEZ, Giuliana Maria Morais  TL 1106492 GUERRA, Ruth Maria Ribeiro  TL 1105528, TL 1105576
GONZÁLES-SALAZAR, Carelis  TL 1105166 GUERREIRO, Carlos Montovani  TL 1106212
GONZÁLEZ-SALAZAR, Carelis  TL 1104979 GUIDA, Fernanda Ribeiro  TL 1104831
GONÇALO, Ana Clara Mota  TL 1105753 GUIDA, Lucas de Oliveira Cantaruti  TL 1105121
GONÇALVES, Beatriz Caldas  TL 1105889 GUIDA, Lucas de Oliveira Cantarutti  TL 1105718
GONÇALVES, Beatriz MM  TL 1104775 GUILHERME, João Pedro Moreira  TL 1105445, TL 1105638, TL 1105656,
GONÇALVES, Bruno Silva  TL 1106100 TL 1105742, TL 1105005,

391
Índice de Autores – XXX Congresso Brasileiro de Neurologia

TL 1105522, TL 1105816, HORTA, Mauro Souza  TL 1105018


TL 1105506, TL 1105885, HORTA, Wagner Gonçalves  TL 1105018
TL 1105892
HOULDEN, Henry  TL 1105342, TL 1105695, TL 1105714
GUILHOTO, Laura Maria de Figueiredo Ferreira Guilhoto  TL 1106009
HUANG, Zhiyue  TL 1105660
GUIMARAES, Julia Loripe  TL 1106106
HUSSEIN, Mohamed Ali  TL 1106296
GUIMARAES, Willian Medina  TL 1106042
IACHINSKI, Renato Endler  TL 1105477, TL 1105751, TL 1105787,
GUIMARÃES, Domingos Sávio Amorim de Souza Dias  TL 1105882 TL 1105302, TL 1105305,
GUIMARÃES, Henrique Cerqueira  TL 1106259 TL 1104977, TL 1105776
GUIMARÃES, Ingred Pimentel TL 1106391, TL 1106526, TL 1106177, TL 1106567, IBSEN, Christian Ricardo  TL 1105822
TL 1106192, TL 1106385, IEPSEN, Bruno Diógenes  TL 1106197, TL 1106216, TL 1106219
TL 1106286, TL 1106402,
TL 1106641 IESBIK, Ivana da Rosa  TL 1105165
GUIMARÃES, Jane Mary de Medeiros  TL 1105413 IESKIK, Ivana da Rosa  TL 1105134
GUIMARÃES, Maria de Fátima de Menezes  TL 1105082 IKEDA, Raissa Kaori  TL 1105441, TL 1105271
GUIMARÃES, Renata  TL 1105880 IKEDA, Rebeca Aiko  TL 1105441
GUIMARÄES, Pedro Passos  TL 1105556 INOCENTE, Orizon Hilario  TL 1105656
GULHOTE, Daniela Alves  TL 1106209 INOJOSA, Jose Luiz de Mirando Coelho  TL 1106548
GUSMÃO, Breno Moreno  TL 1105146 INOJOSA, José Luiz de Miranda Coelho  TL 1106120
GUSTAVO HENRIQUE FRIGIERI1, TL 1106316 INOJOSA, José Luiz Miranda de  TL 1106465
GUTMAN, Elisa Gouvea  TL 1105530 INÁCIO, Maria Cecília  TL 1105586, TL 1105850, TL 1105599
GUZMAN, Danielle  TL 1105568 IRIKURA, Rodrigo Batista  TL 1105131
GUZZO, Thiago Parreira  TL 1106067, TL 1106148 ISRAEL, Adriana Vera Guimarães  TL 1106440, TL 1106490, TL 1106503
GÓES, Cristina Pessoa de Queiroz Faria  TL 1105696 ITINOSE, Anelisa Anelisa  TL 1104707
GÓIS, Lourdes Maria Dantas de  TL 1106523 IUGHETTI, Rafaela  TL 1105865
HAAG, Aline  TL 1105116, TL 1105134, TL 1105165 IUGUETTI, Rafaella  TL 1105847
HAAG, Karine  TL 1105134 IWABE, Cristina  TL 1106352
HAAS, Leandro José  TL 1105537 JAEGER, Antônio  TL 1106132, TL 1106190
HAES, Tissiana Marques de  TL 1105362, TL 1105836, TL 1105387 JAMA-ANTÓNIO, Job Monteiro Chilembo  TL 1105532
HAES, Tissiana Marques  TL 1106337 JANUZZI, Mayara Apolinario  TL 1106448
HALLAK, Jaime E.C  TL 1105948 JANUZZI, Mayara Apolinário  TL 1106413, TL 1106449, TL 1106348
HAMMERLE, Mariana Beiral  TL 1105698, TL 1105719, TL 1105736, TL 1105745, JAQUES, Cristina Saade  TL 1104711
TL 1105623, TL 1105636 JARDIM, Laura Bannach  TL 1104685
HAMMERSCHIMIDT, Rafaela  TL 1105116 JARDIM, Marcia rodrigues  TL 1105578, TL 1105130, TL 1105255, TL 1105278
HARGER, Rodrigo  TL 1106063 JARDIM, Marcia rodrigues  TL 1105578, TL 1105130, TL 1105255, TL 1105278
HECK, Nathalia Luiza Mattes  TL 1106053 JAURIS, Paulo Gilberto Medeiros  TL 1105047, TL 1105087, TL 1105088
HECKERT, Rômulo  TL 1105578 JEIRESSATI, Lia  TL 1105865
HEISE, Carlos Otto  TL 1104679, TL 1105176, TL 1105335, TL 1105222 JEREISSATI, Lia  TL 1105847
HELITO, Paulo V. P.  TL 1104679 JEROSCH-HEROLD, Michael  TL 1105288
HEMERLY, Matheus Compart  TL 1105821, TL 1106487, TL 1105756, TL 1104970 JESUS, Argel Gava de  TL 1105551
HENNING, Pamela Regina  TL 1105232 JESUS, Dkaion Vilela de  TL 1106179, TL 1106229, TL 1106570, TL 1106161,
HENRIQUES-SOUZA, Adélia Maria Miranda  TL 1106418 TL 1106142
HERBSTER, Elizeu Alves  TL 1105791 JESUS, Maria Clara Ferreira  TL 1105056
HERCULANO, Fernanda Groppo Nogueira  TL 1106613 JESUS, Nelson Felipe Venas de  TL 1105898
HERGOZ, Ana Carolina Frinhane  TL 1105495 JESUS, Nelson Felipe Venas de  TL 1105898
HERLINGER, Alice Laschuk  TL 1105782 JESUS, Paulo Atila Castro Carvalho de  TL 1106428, TL 1106611
HERWIG, Gabriela Klein  TL 1105864 JESUS, Pedro Antonio Pereira de  TL 1106447, TL 1105379, TL 1105324,
TL 1106267, TL 1105819,
HERZOG, Ana Carolina Frinhane  TL 1105468, TL 1105473, TL 1105698, TL 1106421, TL 1106428,
TL 1105719, TL 1105745, TL 1106543, TL 1106579,
TL 1105623 TL 1106591, TL 1106611
HIGUCHI, Eunice  TL 1106576 JESUS, Pedro Antonio Pereira de  TL 1106447, TL 1105379, TL 1105324,
HIRAYAMA, Sayuri Aparecida  TL 1105666, TL 1105686, TL 1105699, TL 1106267, TL 1105819,
TL 1105264, TL 1105390, TL 1106421, TL 1106428,
TL 1105691, TL 1105641 TL 1106543, TL 1106579,
HOEFEL, Alice Maria Luderitz  TL 1106372 TL 1106591, TL 1106611

HOLANDA, Arthur Cesário de  TL 1106188, TL 1105734, TL 1105777, JESUS, Pedro Antônio Pereira de  TL 1105655, TL 1106619
TL 1106423, TL 1105773, JESUS, Pedro Antônio Pereira  TL 1106118
TL 1106397, TL 1106444, JESUS, Pedro Ap  TL 1104775
TL 1106465, TL 1105237,
TL 1106548, TL 1106208 JESUS, Tatiane Arroyo Lopes Alves de  TL 1105171, TL 1105541, TL 1105844,
TL 1105538, TL 1105795,
HOLANDA, Maurus Marques de Almeida  TL 1106059, TL 1105913, TL 1106562, TL 1105771, TL 1106123
TL 1105874, TL 1106571
JESUS, Tatiane Arroyo Lopes Alves  TL 1105367
HOLZ, Maila Rossato  TL 1106144
JESUS, Tatiane Arroyo Lopes Aves de  TL 1105831
HONORATO, Marcos Manoel  TL 1106186
JIMENEZ, Barbara  TL 1105448
HONORIO, Fernanda Paiva Pereira  TL 1106064
JOAQUIM, LARISSA SILVA  TL 1106293
HONORIO, Gustavo L de Freitas  TL 1104809
JOAQUIM, Larissa  TL 1106491
HORODESKI, Jaqueline Sueli  TL 1105134, TL 1105165, TL 1105329
JOMAA, Danny  TL 1104679

392
Índice de Autores – XXX Congresso Brasileiro de Neurologia

JORDAO, Daisi Carolina Fritz  TL 1106228 JUNIOR, Silvio Henrique dos Reis  TL 1105054
JORDAO, daisi fritz  TL 1106157 JUNIOR, Tales de Oliveira  TL 1105742
JORDÃO, Daisi Carolina Fritz  TL 1106395 JUNIOR, Tales Oliveira  TL 1105522, TL 1105506
JORGE, Ana Carolina dos Santos  TL 1104862, TL 1105559, TL 1105516, JUNIOR, Valdecir Boeno Spenazato  TL 1106214
TL 1105560 JUNIOR, Wilson Marques  TL 1105756, TL 1105247, TL 1105781, TL 1105867,
JOSÉ, Maria Renata  TL 1106564, TL 1106590 TL 1105592, TL 1105277,
JOÃO, Rafael Baptista  TL 1106607 TL 1105717, TL 1105239,
TL 1106408, TL 1105167,
JOÃO, Rafael Batista  TL 1105610, TL 1106212, TL 1106353 TL 1105588, TL 1105837,
JOÃO, Rafael  TL 1106312, TL 1106594 TL 1105355
João Pedro Gammardella Rizzi JUNIOR, Wilson Merques  TL 1105805
João Pedro Gammardella Rizzi JUNQUEIRA, Iori Rodrigues  TL 1105688
JR, Ciro Matsui  TL 1106542, TL 1106551 JUNQUEIRA, Pedro Durante  TL 1106078
JR, Luiz Dalfior  TL 1106249 JURNO, Mauro Eduardo  TL 1106097
JR, Marcodes C França  TL 1105284 JUSTINO, Maria Eduarda Costa Brandão  TL 1105125
JR, Marcondes Cavalcante França  , TL 1104731, TL 1104778, JUSTINO, Maria Eduarda da Costa Brandão  TL 1106427, TL 1106496,
TL 1106302, TL 1105296, TL 1105512
TL 1106132, TL 1106352, JÚNIOR, Adaucto Wanderley da Nóbrega  TL 1105062
TL 1104979, TL 1105797
JÚNIOR, Aluísio Cláudio Mentor Neves de Couto Melo  TL 1105833
JR, Marcondes Cavalcante França  TL 1104731, TL 1104778, TL 1106302,
TL 1105296, TL 1106132, JÚNIOR, Amilton Komnitski  TL 1105833
TL 1106352, TL 1104979, JÚNIOR, Antônio Lazaro Santos Soares  TL 1106543, TL 1106591
TL 1105797 JÚNIOR, Antônio Lázaro Santos Soares  TL 1106421
JR, Marcondes C França  TL 1105785 JÚNIOR, Armando Carlos Diógenes  TL 1106557
JR, Marcos Rosa  TL 1105705 JÚNIOR, Avelino Missialdes Dutra  TL 1106223
JR, Mario Wolak  TL 1104886 JÚNIOR, Carlos Mauricio Jaborandy de Mattos Dourado  TL 1105498
JR, Pedro Heder Oliveira  TL 1106431 JÚNIOR, Daniel Vicente de Siqueira Lima  TL 1105693, TL 1105748
JR, Valério Carvalho  TL 1106360 JÚNIOR, Euldes Mendes  TL 1105791, TL 1105872
JR, Wilson Marques  TL 1104731, TL 1105070, TL 1105785, TL 1104979, JÚNIOR, Francisco Sales da Silva  TL 1105061
TL 1105695, TL 1105714
JÚNIOR, Fábio Antonio Serra de Lima  TL 1105872
JR, Wilson Marques  TL 1104731, TL 1105070, TL 1105785, TL 1104979,
TL 1105695, TL 1105714 JÚNIOR, Fábio Lima  TL 1104712
JUCÁ, Mikaio de Sousa  TL 1105848, TL 1105872 JÚNIOR, Fábio Pereira da Silva  TL 1105511, TL 1105855, TL 1106141,
TL 1106130, TL 1105801,
JUCÁ, Renata Viana Brígido de Moura  TL 1105498, TL 1105508, TL 1105594, TL 1106143, TL 1106446,
TL 1105465 TL 1106624
JUNGER, Arthur Costa  TL 1105511, TL 1105855, TL 1106141, TL 1106130, JÚNIOR, Jefferson Heber Marques Fontes TL 1105361, TL 1105697, TL 1105823,
TL 1105801, TL 1106143, TL 1105746
TL 1106446, TL 1106624
JÚNIOR, Jefferson Heber Marques Fontes TL 1105361, TL 1105697, TL 1105823,
JUNIOR, Albano Mayer  TL 1105787, TL 1105305 TL 1105746
JUNIOR, Antonio Lazaro Santos Soares  TL 1106579 JÚNIOR, José Lopes de Vasconcelos  TL 1105565
JUNIOR, Antonio Lázaro Santos Soares  TL 1105819 JÚNIOR, José Lopes Vasconcelos  TL 1105340
JUNIOR, Artemio Moreira Peixoto  TL 1106126 JÚNIOR, Luiz José Malta Gaia Ferreira  TL 1105536
JUNIOR, Daniel Vicente de Siqueira Lima  TL 1106439, TL 1106633, TL 1106294, JÚNIOR, Luiz Severo Bem  TL 1105895, TL 1105182
TL 1105453, TL 1106603,
TL 1105778, TL 1106549 JÚNIOR, Marcondes Cavalcante França  TL 1105564
JUNIOR, Euldes Mendes  TL 1104712 JÚNIOR, Miguel Cândido da Costa  TL 1105902
JUNIOR, Fabio Antonio Serra de Lima  TL 1105848 JÚNIOR, Mário Emílio Teixeira Dourado  TL 1106412, TL 1106062, TL 1106384,
TL 1106426, TL 1106133
JUNIOR, Hermes Castro Araujo  TL 1105657
JÚNIOR, Mário Emílio Teixeira Dourado  TL 1106412, TL 1106062, TL 1106384,
JUNIOR, Ivo Knut Anders  TL 1105347 TL 1106426, TL 1106133
JUNIOR, Jorge Alberto Martins Pentiado  TL 1105133, TL 1105083, TL 1105366, JÚNIOR, Mário Luciano Mélo Silva  TL 1106523
TL 1105372
JÚNIOR, Pedro Helder de Oliveira  TL 1106581
JUNIOR, Jose Wagner Leonel Tavares  TL 1106604
JÚNIOR, Pedro José da Silva  TL 1106507
JUNIOR, José Wagner Leonel Tavares  TL 1105945
JÚNIOR, Renato Campos Freire  TL 1105519
JUNIOR, Laerte Honorato Borges  TL 1105601
JÚNIOR, Renato C F  TL 1106440, TL 1106490, TL 1106503
JUNIOR, Luiz Dalfior  TL 1106411, TL 1106424
JÚNIOR, Renato Pereira da Costa  TL 1106126
JUNIOR, Luiz Severo Bem  TL 1105857
JÚNIOR, Roberto Santos de Oliveira  TL 1106040
JUNIOR, Marcondes Cavalcante França  TL 1106363, TL 1105166, TL 1106408
JÚNIOR, Sergio Roberto Pereira da Silva  TL 1105309
JUNIOR, Mario Emilio Teixeira Dourado  TL 1105320
JÚNIOR, Sérgio Roberto Pereira da Silva  TL 1105253
JUNIOR, Mario Luciano Melo Silva  TL 1106492
JÚNIOR, Tales de Oliveira  TL 1105445, TL 1105638, TL 1105656, TL 1105005
JUNIOR, Oscar Nelson Reimann  TL 1105123, TL 1105165, TL 1105329
JÚNIOR, Tales de Oliveira  TL 1105445, TL 1105638, TL 1105656, TL 1105005
JUNIOR, Oscar Nelson Reimman  TL 1105116, TL 1105134
JÚNIOR, Valdecir Boeno Spenazato  TL 1106203, TL 1106209
JUNIOR, Pedro Helder de Oliveira  TL 1106177, TL 1105405, TL 1106335,
TL 1106010, TL 1105949 JÚNIOR, Wilson Alves de Oliveira  TL 1106208
JUNIOR, Pedro Helder Oliveira  TL 1106125 JÚNIOR, Wilson Marques  TL 1104755, TL 1105342, TL 1106404, TL 1105540,
TL 1105547, TL 1105314,
JUNIOR, Roberto Carlos Sousa Alves  TL 1106492 TL 1105307, TL 1105509
JUNIOR, Sandro Adriano Souza Lima  TL 1105885, TL 1105471, TL 1105487 JÚNIOR, Álvaro Alves de Sá  TL 1106214
JUNIOR, Silas Fernandes Avelar  TL 1106155

393
Índice de Autores – XXX Congresso Brasileiro de Neurologia

KAIMEN-MACIEL, Damacio Ramon  TL 1105844, TL 1105795, TL 1105771, LAZERA, Vitória de Mendes Sá  TL 1106442
TL 1105831 LEAL, André Giacomelli  TL 1105459, TL 1105466
KAMADA, Daniela Midori  TL 1105235 LEAL, Leonardo Barbosa  TL 1106531
KANAWA, Gleica Maria de Macena  TL 1105565 LEAL, Lucas Luan Gonçalves Barros  TL 1105363, TL 1105373
KANEMATSU, Maiara  TL 1105031 LEAL, Patricia Provenzano  TL 1106306
KAUARK, Roberta Borges Gomes  TL 1105447, TL 1105665 LEAL, Patrick Ramon dos Santos  TL 1106545
KAY, Cláudia Suemi Kamoi  TL 1106172 LEAL, Paulo Roberto Lacerda  TL 1106587, TL 1106589, TL 1105142,
KERCHNER, Geoffrey A  TL 1105660 TL 1105272, TL 1105907,
KERSTING, Nathalia  TL 1104685 TL 1105900, TL 1105136,
TL 1105419, TL 1106264
KIM, Ho Jin  TL 1105040
LEANDRO, Cícera Simplicio Machado  TL 1105066, TL 1105114
KIM, Kevin  TL 1104957
LEDA, Jóshua Matheus de Sousa  TL 1105639
KIM, Lenise Jihe  TL 1106100
LEDERMAN, Alex  TL 1106516
KINJO, Juliana Yumi  TL 1105031
LEE, Annelise Akemi Higa  TL 1106516, TL 1106610, TL 1106576, TL 1106406,
KLEIN, Gregory  TL 1105660 TL 1106637
KLOEPPEL, Manuela Aparecida  TL 1105062 LEE, Stefanie Hitomi de Nishi  TL 1105031
KNOCHENHAUER, André Enoch  TL 1105122, TL 1105124 LEITE, Amanda Nassur Corrêa  TL 1105347
KNORST, Gabriel Rocha Santos  TL 1105689 LEITE, Bruno de Magalhães Barbosa  TL 1106596, TL 1106613, TL 1106573
KOFF, Marco Antonio Eduardo  TL 1105820 LEITE, Camila Ferreira  TL 1106273, TL 1106586, TL 1106653
KOFF, Marco Antonio  TL 1105183, TL 1105407 LEITE, Christian Max Uchôa  TL 1104780, TL 1105914, TL 1106197, TL 1106216,
KOGA, César Minoru Toita  TL 1106480 TL 1106219
KOSMINSKY, Ellen  TL 1106239 LEITE, Elder Machado  TL 1105882, TL 1105899, TL 1106549
KOUYOUMDJIAN, João Aris  TL 1105190, TL 1105176 LEITE, Filipe Augusto Silva  TL 1106479, TL 1106556
KOUYOUMDJIAN, João A  TL 1104679 LEITE, Gerlânia de Oliveira  TL 1105164
KOWACS, Fernando  TL 1105617 LEITE, Gleif Farias  TL 1105361
KRONBAUER, Lorena Fernandes  TL 1105505, TL 1105606, LEITE, Heloisa Ferraz Troijo  TL 1106329
TL 1105618, TL 1106283, LEITE, João Marcelo Borba  TL 1106329, TL 1106206
TL 1105171, TL 1105541,
TL 1105844, TL 1105538, LEITE, Juliana Magalhães  TL 1105693, TL 1106294, TL 1106603, TL 1105778
TL 1105795, TL 1105367, LEITE, Luis Felipe Magalhães Serne  TL 1106382
TL 1105771, TL 1105831 LEITE, Marco Antonio Araujo  TL 1105878
KRONBAUER, Lorena Ferndandes  TL 1106123 LEITE, Marjorie Bindá  TL 1105393
KRUEGER, Mariana Braatz  TL 1106091, TL 1106649 LEITE, Nícolas Fonteles  TL 1106273
KRUG, Bárbara  TL 1106110 LEITE, Patrícia Lúcia Silva Sampaio  TL 1105070
KULIKOWSKI, Leslie Domenici  TL 1106175, TL 1106189 LEITÃO, Antônio Miguel Furtado  TL 1105780
KUS, William Pegoraro  TL 1106522 LEITÃO, Déborah Araújo  TL 1105937
KUSTER, Gustavo  TL 1106287, TL 1106319, TL 1106139, TL 1106307, LEMOS, Ana Flavia Andrade  TL 1105157
TL 1106360
LEMOS, Ana Flávia Andrade  TL 1106413, TL 1106448, TL 1105111
LACERDA, Danielle  TL 1106580
LEMOS, Andreza Salvio  TL 1105570, TL 1105668, TL 1105757, TL 1105269,
LACERDA, Eliza Maria da Costa Brito  TL 1105812 TL 1105607, TL 1105530,
LACERDA, Rebeca Montenegro de  TL 1105148 TL 1105782, TL 1105665
LACERDA, Rebeca Montenegro  TL 1105326, TL 1105475 LEMOS, Isadora Versiane  TL 1105897
LACERDA, Roberta Ismael  TL 1104860, TL 1105285, TL 1104874, TL 1105428 LEMOS, Isadora Versiani de  TL 1105869, TL 1105710
LACERDA, Saulo Pimenta  TL 1106085 LEMOS, Isadora Versiani  TL 1105462
LAIS, Lucia Leite  TL 1106062 LEMOS, Nilson Batista  TL 1105895, TL 1105182
LAMAS, Gustavo Manginelli  TL 1105750, TL 1105635, TL 1105863, TL 1105296, LEOCÁDIO, Jéssica de Cássia Marques  TL 1106124
TL 1105166, TL 1105630 LEON, Luciane Almeida Amado  TL 1106168
LANCELLOTTI, Carmen Lucia Penteado  TL 1105793 LEON, Soniza Vieira Alves  TL 1106311
LANDIM, João Igor Dantas  TL 1106105, TL 1106234, TL 1106244, TL 1106616, LEONI, Tauana Bernardes  TL 1104778
TL 1106453, TL 1106107,
TL 1106173, TL 1106632, LEOTTI, Vanessa Bielefeldt  TL 1104685
TL 1106537, TL 1106236 LEPRE, Vanessa Bernardo Nunes  TL 1106329
LANDIM, João Igor  TL 1106429, TL 1106466 LESTINGI, Silvia Cordova  TL 1106100
LANG, Michael Ricardo  TL 1105347, TL 1105349, TL 1105116, TL 1105123, LEÃO, Bianca Lopes Cavalcante  TL 1106564, TL 1106590
TL 1105134, TL 1105165, LEÃO, Bruna Acioly  TL 1104833, TL 1105908, TL 1104978
TL 1105329
LEÃO, Maria Eduarda de Souza Arêa  TL 1106631, TL 1105373, TL 1105653,
LANGE, Marcos Christiano  TL 1106191, TL 1106480, TL 1105365, TL 1105684, TL 1105814, TL 1105826
TL 1105725, TL 1105096
LIEM, Assuncion Martinez  TL 1105570, TL 1105757, TL 1105269, TL 1105607
LARA, Carolina Haveroth  TL 1106156
LIEM, Asunción Martinez  TL 1105494
LARCIPRETTI, Anna Laura Lima  TL 1105216, TL 1105138
LIMA, Aline Vieira Scarlatelli  TL 1104955
LAURA, Matilde  TL 1105714
LIMA, Ana Rosa Melo Correa  TL 1105102, TL 1106239, TL 1106365,
LAURINI, Lia Leal  TL 1105732, TL 1105639, TL 1105061, TL 1105744, TL 1105764, TL 1105815,
TL 1105870 TL 1106184, TL 1105631
LAVOR, Vytor Alves de  TL 1105225 LIMA, Ana Rosa Melo Correia  TL 1106467
LAZEIRA, Victória de Menezes Sá  TL 1105259 LIMA, Arieny Karen Santos  TL 1105363, TL 1105548
LAZERA, Victória de Menezes Sá  TL 1105812, TL 1105916, TL 1106078 LIMA, Arissa Crispim Maximo  TL 1106555
LAZERA, Vitoria de Menezes Sá  TL 1105753 LIMA, Brenda Matheus Nogueira de  TL 1104998, TL 1105085

394
Índice de Autores – XXX Congresso Brasileiro de Neurologia

LIMA, Caio Vinicius Barroso de  TL 1106104, TL 1105726 LINHARES, Kennedy Soares  TL 1105565, TL 1106343, TL 1105843
LIMA, Camilla Silva Dantas Mendes  TL 1105658 LINO, Angelina Maria Martins  TL 1105178, TL 1105222
LIMA, Carlos Frederico Leite Souza  TL 1105815 LINS, Alice Cavalcante Almeida  TL 1104833, TL 1105908, TL 1104978
LIMA, Clarice  TL 1105414 LINS, Cícero José Pacheco  TL 1105481
LIMA, Danielle Pessoa  TL 1104998, TL 1105085, TL 1105107, TL 1105145, LINS, Jose Jefferson Silva Cavalcanti  TL 1105388
TL 1105236, TL 1105244, LINS, Jose Jefferson Silva Cavancanti  TL 1105380
TL 1106217
LINS, José Jefferson Silva Cavalcanti  TL 1105252
LIMA, Daniel Meohas de Souza  TL 1105696
LINS, João Pedro Sá  TL 1105784
LIMA, Eduarda Rabêlo  TL 1105164
LINS, Lucas Vergne Cheade  TL 1106547
LIMA, Emmanuel Avelino de  TL 1105445, TL 1105638
LINS, Otávio Gomes  TL 1106467
LIMA, Ester Costa  TL 1105697
LINZMEIER, Pietra Cani  TL 1105537
LIMA, Fabricio D de  TL 1105545
LIRA, Arthur Oliveira  TL 1105895, TL 1105182
LIMA, Fabricio Oliveira  TL 1106229, TL 1106553, TL 1106570, TL 1106520
LIRA-BATISTA, MARTA MARIA da SILVA  TL 1106379, TL 1106367, TL 1106369,
LIMA, Fabricío Oliveira  TL 1106244 TL 1106517, TL 1106495
LIMA, Fabrícia Fontes  TL 1105500 LIRA-BATISTA, MARTA MARIA da SILVA  TL 1106379, TL 1106367, TL 1106369,
LIMA, Fabrício D. de  TL 1104731 TL 1106517, TL 1106495
LIMA, Fabrício Diniz de  TL 1104979 LIRA-BATISTA, MARTA MARIA da SILVA  TL 1106379, TL 1106367, TL 1106369,
LIMA, Fabrício Oliveira  TL 1106245, TL 1106620 TL 1106517, TL 1106495

LIMA, Fernanda Sousa  TL 1105702, TL 1105580, TL 1105585, TL 1105590 LISBOA, João Vitor Mortari  TL 1105157, TL 1106407, TL 1104943, TL 1105109,
TL 1105110, TL 1105111,
LIMA, Gabrielle Benevides  TL 1105455, TL 1106501, TL 1106632 TL 1105144, TL 1105488
LIMA, Gleydyson Wesley Freire  TL 1106367, TL 1106495 LISBOA, João Vitor M  TL 1106279, TL 1106278
LIMA, Isabel Maria Oliveira Macedo  TL 1106621, TL 1106631, TL 1106598, LISE, Emerson Oliveira  TL 1104889, TL 1104824
TL 1106575
LIU, Jonathan Wei Ting Wen  TL 1105123
LIMA, Izadora Vieira Bolzani Lopes  TL 1106237
LOBATO, Enedina Maria Monteiro  TL 1106104
LIMA, Jessica Layanne de Sousa  TL 1105164
LOBATO, Lara Willers  TL 1105864
LIMA, Joana Clara Oliveira Macedo  TL 1106598, TL 1105909
LOBO, Camila Caroso  TL 1105863, TL 1105166, TL 1105785
LIMA, José Guilherme Martins  TL 1105705, TL 1105716, TL 1105566,
TL 1105990 LOBO, Camila C  TL 1105284, TL 1105288

LIMA, José Rosemberg Costa  TL 1105450, TL 1105461, TL 1105574, LOBO, Izabella Rocha  TL 1106209
TL 1105836, TL 1106528, LOBO, Luiz Felipe Nogueira de Figueiredo  TL 1105853
TL 1105277, TL 1105355 LOCHMÜLLER, Hanns  TL 1104679
LIMA, Karla Vanessa Morais  TL 1105812 LOIOLA, Rodrigo Bindá de Magalhães  TL 1104824
LIMA, Karlla Danielle Ferreia  TL 1106469 LONDERO, Renata  TL 1106298, TL 1106301
LIMA, Karlla Danielle Ferreira  TL 1105306, TL 1105049, TL 1106270 LONGO, Alexandre Luiz  TL 1106063, TL 1105096
LIMA, Lidiane Andrea Oliveira  TL 1105498, TL 1105508 LONGO, José Eduardo Trentim  TL 1105157
LIMA, Lidiane Andréa Oliveira  TL 1105594, TL 1105465 LONGO, Maria Francisca Moro  TL 1104891
LIMA, Ludmila Machado  TL 1106296, TL 1105646, TL 1105624, TL 1105625, LOPES, Alessandro Augusto Viana Oliveira  TL 1105630
TL 1105619, TL 1105464
LOPES, Alexandre Bussinger  TL 1106104
LIMA, Luiza Aguiar  TL 1105869, TL 1105897
LOPES, Daniel Thomas Pereira  TL 1105293, TL 1105739
LIMA, Luiz Fernando Melo  TL 1105227
LOPES, Edson Gabriel de Lima  TL 1105148
LIMA, Lázaro de  TL 1105531
LOPES, Fernanda Cristina Rueda  TL 1105782, TL 1106540
LIMA, Manuela Cavalcante Coling  TL 1106608, TL 1106623
LOPES, Janine Lemos Mel Lobo Jofili  TL 1105861, TL 1105870
LIMA, Marcelo Ataíde de  TL 1106559
LOPES, Laura Cardia Gomes  TL 1105157, TL 1104943, TL 1105109, TL 1105110,
LIMA, Marco Antonio Dantas Salles  TL 1104809 TL 1105111
LIMA, Marco Antonio Sales Dantas de  TL 1106506 LOPES, Maycon Melo  TL 1106203, TL 1106281, TL 1105630
LIMA, Maria Luíza Marinho de Sá de Paula  TL 1106390 LOPES, Pedro Luiz  TL 1106223
LIMA, Micael Porto Portela  TL 1105848, TL 1105872, TL 1106222 LOPES, Rafaella Albuquerque  TL 1105092
LIMA, Pedro Lucas Grangeiro de Sá Barreto  TL 1105316, TL 1105405, LOPES, Renan Barros  TL 1105836, TL 1106337, TL 1105837
TL 1106581, TL 1105949
LOPES, Sarah Cerillo  TL 1105321
LIMA, Pedro Lucas Grangeiro Sá Barreto  TL 1106196, TL 1106207
LOPES-CENDES, Iscia  TL 1105215, TL 1105276
LIMA, Raynrich Kevin Assis  TL 1106452
LORENA, Ivan Augusto  TL 1105588
LIMA, Rian Vilar  TL 1106262, TL 1105301, TL 1105318, TL 1106117,
TL 1106339, TL 1106544, LORENZONI, Leonardo Afonso  TL 1105857
TL 1106608, TL 1106623 LORENZONI, Paulo José  TL 1106172
LIMA, Sylvio Ricard Gonçalves de Souza  TL 1106162, TL 1106620, TL 1106280, LOURENÇO, Elora Sampaio  TL 1105619
TL 1106090, TL 1106359, LOURENÇO, Galileu Chagas  TL 1106121
TL 1106429, TL 1106466,
TL 1106308 LOZ, Sabrina Hafemann  TL 1106063
LIMA, Sylvio Ricard Gonçalves Souza  TL 1106450 LOZ, Sabrina Hafemann  TL 1106063
LIMA, Sylvio Ricardo Gonçalves de Souza  TL 1106105 LUCAS, Ana Beatriz da Costa  TL 1105066, TL 1105114
LIMA-SILVA, Thais Bento  TL 1106259 LUCAS, Elizabeth Lemos Silveira  TL 1106551
LIMONTE, Fábio Henrique  TL 1104728 LUCATO, Leandro Tavares  TL 1106252, TL 1106140, TL 1105048
LIN, Katia  TL 1105409, TL 1105103, TL 1105122, TL 1105124, TL 1105062 LUCENA, Adson Freitas de  TL 1106162
LIN, Kátia  TL 1105395 LUCENA, Paulo Antonio Farias de  TL 1105748, TL 1105453
LINDNER, Stephanie  TL 1105123, TL 1105329 LUCENA, Paulo Antonio Farias  TL 1106603
LINHARES, Ana Paula Costa  TL 1105812 LUCENA, Paulo Antônio Farias de  TL 1105810, TL 1106439

395
Índice de Autores – XXX Congresso Brasileiro de Neurologia

LUCENA, Paulo Antônio Farias  TL 1106294 MAGALHÃES, João Eudes  TL 1106317, TL 1106612, TL 1105524, TL 1106398,
LUEMBA, Martinho  TL 1105532 TL 1106187, TL 1106239,
TL 1106365, TL 1105764,
LUNA, Débora Nalígia Moraes  TL 1105672 TL 1105815, TL 1106184,
LUNARDI, Mariana dos Santos  TL 1105103 TL 1106467, TL 1106210,
LUPPI, Andrea de Martino  TL 1105850, TL 1105307 TL 1105557, TL 1105809

LUSTOSA, Júlio Augusto  TL 1105895, TL 1105182 MAGALHÃES, Juliana Silva Almeida  TL 1106572, TL 1106605, TL 1106630

LUVIZUTTO, Gustavo José  TL 1106008, TL 1106023, TL 1106435 MAGALHÃES, Jurandy Júnior Ferraz de  TL 1106320, TL 1106559

LUVIZUTTO, Gustavo  TL 1106348 MAGALHÃES, Lara Cordeiro  TL 1106572, TL 1106135, TL 1106134, TL 1106605,
TL 1106118, TL 1106524,
LÉGORA, Davi Bravo Huguinim  TL 1106533, TL 1106471, TL 1106268 TL 1106556, TL 1106574
LÉGORA, Sophia Bravo Huguinim  TL 1106533, TL 1106471 MAGALHÃES, Luciana Zelante Ambiel  TL 1105353
LÓCIO, Aline Moreira  TL 1105847 MAGALHÃES, Marilza Campos de  TL 1105712
LÓCIO, Aline  TL 1105865 MAGALHÃES, Paolla Giovanna Rossito de  TL 1105346
MACDONALD, Matthew L  TL 1105215 MAGALHÃES, Paolla Giovanna Rossito  TL 1105370
MACEDO, Aline Barbosa  TL 1105240, TL 1105242 MAGALHÃES, Pedro Silva Correa  TL 1105096
MACEDO, Amanda Souza Chaves  TL 1105414 MAGALHÃES, Samir Camara  TL 1106649
MACEDO, Laiane de Alencar  TL 1106410 MAGALHÃES, Samir Câmara  TL 1106091, TL 1106196, TL 1106207, TL 1106273
MACEDO, Lidiane Alves  TL 1105230 MAGESTE, Priscila dos Santos  TL 1105462, TL 1105710, TL 1105479
MACEDO, Victor Felippe Bispo  TL 1106418 MAGESTE, Priscila Santos  TL 1105869, TL 1105897, TL 1105741, TL 1106300
MACEDO, Victor Fellipe Bispo TL 1105102, TL 1105789, TL 1106187, TL 1105764, MAIA, Fernanda Martins  TL 1106105, TL 1106162, TL 1106233, TL 1106234,
TL 1105815, TL 1106184, TL 1106244, TL 1106616, TL 1106218, TL 1106461,
TL 1105589, TL 1105613 TL 1106453, TL 1106107, TL 1106090, TL 1106159,
MACEDO, Victor Fellipe Bispo TL 1105102, TL 1105789, TL 1106187, TL 1105764, TL 1106173, TL 1106359, TL 1106429, TL 1106501,
TL 1105815, TL 1106184, TL 1106170, TL 1106450, TL 1106466, TL 1106493,
TL 1105589, TL 1105613 TL 1106632, TL 1106502, TL 1106308, TL 1106537,
MACEDO, Yuri Silva  TL 1105770 TL 1106236, TL 1106473, TL 1106434, TL 1106499,
TL 1105019, TL 1106226, TL 1106336, TL 1106386,
MACHADO, Amanda Batista  TL 1105624 TL 1106452, TL 1105763
MACHADO, Antônio Fernando  TL 1105177 MAIA, Fernanda  TL 1106606
MACHADO, Carolina Carmona Pinheiro  TL 1106214 MAIA, Isaac Holanda Mendes  TL 1106263
MACHADO, Denise  TL 1105407 MAIA, Larissa Almeida  TL 1105465
MACHADO, Luis Felipe Dantas da Nóbrega  TL 1106588 MAIA, Matheus Gonçalves  TL 1106533, TL 1106471, TL 1106433, TL 1106268,
MACHADO, Marcela Alcântara  TL 1106189 TL 1106282
MACHADO, Marcelo Teixeira  TL 1105833 MAIA, Raphael de Paula Doyle  TL 1105716
MACHADO, Maria Iris de Moraes  TL 1105230 MAIA, Renata M  TL 1104775
MACHADO, Maria Iris Moraes  TL 1105774 MAIA, Tonyato Fernandes Ribeiro  TL 1105872
MACHADO, Michel Ferreira  TL 1105115, TL 1105552, TL 1106249 MAIA, Víctor Queirós Calheiros Campelo  TL 1106608, TL 1106623
MACHADO, Richard Simon  TL 1106293, TL 1106491 MAITO, Vitor Teixeira  TL 1105402
MACHADO, Richard Simon  TL 1106293, TL 1106491 MAKAREM, Louise  TL 1105247
MACHADO, Roberta Ismael Lacerda  TL 1104860, TL 1105285, TL 1105428 MALHEIROS, Júlia Evaristo Figueiredo  TL 1105441
MACHADO, Stefano  TL 1105881, TL 1105581, TL 1105891 MALISKI, Jessica  TL 1106564
MACHADO, Vitor Oliveira  TL 1106179, TL 1106142 MALTA, Crissia Pitanga  TL 1105696
MACIEL, Damacio Ramon Kaimen  TL 1105505, TL 1105606, TL 1105618, MALTA, Maisa Vieira da Silva  TL 1105428
TL 1106283, TL 1105171, MANFRINATI, Luís Eduardo d’Almeida  TL 1106282
TL 1105541, TL 1105538,
TL 1105367, TL 1106123 MANGANELLI, Luciane Aparecida Gonçalves  TL 1105519, TL 1105413

MACIEL, Ernane Pires  TL 1105702, TL 1105580, TL 1105585, TL 1105590 MANGAS, Gabriel de Moraes  TL 1106203, TL 1106214

MACIEL, Guilherme Pablo de Santana  TL 1106384 MANGOLIN, Alison  TL 1105808, TL 1105836, TL 1105604, TL 1105798,
TL 1105399, TL 1105592
MACIEL, Nicoly  TL 1106654, TL 1106655
MANGOLIN, Alisson  TL 1105821
MACIEL, Thiago de Oliveira  TL 1106061
MANHAGUANHA, Gustavo  TL 1106156
MACIEL, Victor Augusto Zanesi  TL 1105808, TL 1105574, TL 1105836,
TL 1106528, TL 1105387, MANIVA, Samia Jardelle Costa de Freitas  TL 1106261, TL 1106558
TL 1105604, TL 1105798, MANSUR, Kalil Ibrahim  TL 1105189, TL 1105432
TL 1105781, TL 1105399, MANSUR, Leticia Fêzer  TL 1105066, TL 1105114
TL 1105592
MANTESE, Carlos Eduardo  TL 1104957
MACÊDO, Luísa Couceiro de Albuquerque  TL 1105777, TL 1106397
MANTOVANI, Charles Maroly Lessa  TL 1106404
MACÊDO, Stella Maria  TL 1105318
MANTOVANI, Gabriel Paulo  TL 1106298
MACÊDO, Victor Fellipe Bispo  TL 1105524, TL 1106365,
TL 1105743, TL 1106210, MANZOLI, Isabela Reis  TL 1104697
TL 1105557, TL 1105809 MANZOLINI, Matheus Fernando  TL 1106595
MADRUGA, Kauan Alves Sousa  TL 1105227 MARAFON, Hellen Camila  TL 1105403
MAGALHAES, Daiane  TL 1106362 MARANGA, Carina  TL 1106511
MAGALHAES, Felipe Barbosa  TL 1106600 MARANHÃO, Eduardo  TL 1106188
MAGALHAES, Francisco Neuton de Oliveira  TL 1106555 MARANHÃO, Vicente Albuquerque  TL 1105397, TL 1105402, TL 1105403
MAGALHAES, Samir Camara  TL 1106064 MARAZZI, Thire Baggio Machado  TL 1105450, TL 1105574, TL 1106528
MAGALHAES, Sarah de Sousa  TL 1106280 MARCELINO, Paulo Filho Soares  TL 1105738, TL 1105299, TL 1105274,
MAGALHÃES, Daiane  TL 1105346 TL 1105791, TL 1105848,

396
Índice de Autores – XXX Congresso Brasileiro de Neurologia

TL 1105348, TL 1105423, MARTINEZ, Alberto RM  TL 1105288


TL 1106379, TL 1106065 MARTINEZ, Alberto Rolim Muro  TL 1105296, TL 1106408
MARCELINO, Paulo  TL 1104712 MARTINS, Ana Carolina  TL 1104685
MARCHI, Bruno de  TL 1105988 MARTINS, Beatriz Souza  TL 1105752
MARCHI, Luan  TL 1106252 MARTINS, Camila Dias  TL 1105092
MARCIÃO, Adriano Robert  TL 1105506 MARTINS, Daniel Isoni  TL 1106126, TL 1106121, TL 1106124
MARCO, Maísa de  TL 1106110 MARTINS, Danyela  TL 1106391, TL 1106526, TL 1106622, TL 1106076,
MARCOLINO, Luiza Carolina Moreira  TL 1105576 TL 1106385, TL 1106286,
MARCOLLA, Isabela Maria Garcia  TL 1106205 TL 1106402, TL 1106641

MARCUSSO, Rosa Maria Nascimento  TL 1106451 MARTINS, Emerson Fachin  TL 1105465

MARGATO, Danielle Patricia Borges  TL 1106407, TL 1106448 MARTINS, Fabio Pacheco  TL 1105571, TL 1106315

MARGATO, Danielle Patrícia Borges  TL 1105109 MARTINS, Fabio Pacheco  TL 1105571, TL 1106315

MARGUTI, Ivo  TL 1105040 MARTINS, Gabriela Caetano Lopes  TL 1105365

MARIA, Victória  TL 1105847 MARTINS, Gabriela Joca  TL 1106461, TL 1106173, TL 1106501, TL 1106223,
TL 1106158
MARIANO, Albina Karolyne Diniz  TL 1105855, TL 1106130
MARTINS, Henrique Resende  TL 1105465
MARIANO, Luciano Inácio  TL 1106259, TL 1106061, TL 1106190
MARTINS, MARIA do CARMO de CARVALHO E  TL 1106367, TL 1106517,
MARIANO, Tamiris Carneiro  TL 1106376, TL 1106381, TL 1106396 TL 1106495
MARINHEIRO, Maria Eduarda Pasculli  TL 1105181 MARTINS, MARIA do CARMO de CARVALHO E  TL 1106367, TL 1106517,
MARINHEIRO, Maria Eduarda Pinto  TL 1104998, TL 1105085, TL 1105145 TL 1106495
MARINHO, Antonia Rosivalda Teixeira  TL 1106236, TL 1106499 MARTINS, Marina Isoni  TL 1106190
MARINHO, Arthur Coelho Moura  TL 1105505, TL 1105606, TL 1105618, MARTINS, Rebecca Ranzani  TL 1105688
TL 1106283, TL 1105171, MARTINS, Roger  TL 1106606
TL 1105541, TL 1105844,
TL 1105538, TL 1105795, MARTINS, Sônia Renata Pinheiro  TL 1106138
TL 1105367, TL 1105771, MARTINS, Sônia Renata Pinheiro  TL 1106138
TL 1106123, TL 1105831 MARTINS, Tayane Moura  TL 1106577
MARINHO, Rodrigo Leite  TL 1105705, TL 1105566, TL 1105990, TL 1105799 MARUSSI, Victor Hugo Rocha  TL 1105657, TL 1105146
MARINHO, Samuel Cavalcante  TL 1105082, TL 1106622, TL 1106192 MASCARENHAS, Alice Santos  TL 1105697
MARINOWICK, Daniel  TL 1105407 MASRUHA, Marcelo Rodrigues  TL 1106091, TL 1106649
MAROLI, Keila  TL 1105397 MASSARO, Carina da Silveira  TL 1104874, TL 1105006, TL 1104934
MARQUARDT, Matheus  TL 1105194, TL 1105409, TL 1105642, TL 1105395 MASSARO, Carina Silveira  TL 1104981
MARQUES, Alexandre Coelho TL 1105430, TL 1105001, TL 1105297, TL 1105792, MASSUYAMA, Breno Kazuo  TL 1105166
TL 1105806
MATA, Ignacio F  TL 1105410
MARQUES, Carlindo Malheiros Castro  TL 1106615
MATAS, Sandro Luis de Andrade  TL 1105718
MARQUES, Ingrid Pereira  TL 1105101, TL 1105081
Matheus Mistieri Simabukuro
MARQUES, Jessica Daniele  TL 1105750, TL 1105863
Matheus Mistieri Simabukuro
MARQUES, Juliene de Oliveira  TL 1105227
MATHIAS, Khiany  TL 1106293
MARQUES, Jéssica Daniele  TL 1105555, TL 1105635, TL 1106212
MATIAS, Rafael Braganca Rodrigues  TL 1105306
MARQUES, Luís Felipe Ferreira  TL 1105895, TL 1105182
MATIAS, Rafael Bragança Rodrigues  TL 1105615, TL 1105146, TL 1105793
MARQUES, Marcos Vinícius Oliveira  TL 1105768, TL 1106270, TL 1105186
MATOS, Aline Moura Brazil  TL 1106451
MARQUES, Maria Eduarda Lisbôa  TL 1106272
MATOS, Isabella Nunes  TL 1106234
MARQUES, Maria El  TL 1104775
MATOS, Isabella  TL 1106586
MARQUES, Mayara Silva  TL 1104942, TL 1105271, TL 1105267
MATOS, Janaina Alves Leite  TL 1105420
MARQUES, Miriam Lindsay Silva  TL 1104998, TL 1105085
MATOS, Klayne Cunha  TL 1105763
MARQUES, Patricia Approbato  TL 1105133
MATOS, Luis Eduardo Oliveira  TL 1106589
MARQUES, Patrícia Approbato  TL 1105366, TL 1105372
MATOS, Luiz Filipe Alves  TL 1105324
MARQUES, Pedro Henrique Araújo  TL 1106339
MATOS, Luís Eduardo Oliveira TL 1106587, TL 1105142, TL 1105272, TL 1105907,
MARQUES, Thiago da Cruz  TL 1106145, TL 1106157, TL 1106228, TL 1106395, TL 1105900, TL 1105136,
TL 1106167, TL 1106058 TL 1105419, TL 1106264
MARQUES, Thiago da Cruz  TL 1106145, TL 1106157, TL 1106228, TL 1106395, MATOS, Paula C A A P  TL 1105284
TL 1106167, TL 1106058
MATOS, Paula Camila A.A.P.  TL 1104979
MARQUES, Vanessa Daccach  TL 1104755, TL 1106599
MATOS, Paula Camila A A P  TL 1105785, TL 1105545
MARQUES, Victoria Rosas  TL 1105506, TL 1105885, TL 1105892
MATOS, Paula Camila Alves de A P  TL 1104731
MARQUES, Victória Rosas  TL 1105445, TL 1105638, TL 1105522
MATOS, Paula Camila Alves Pereira de Assis  TL 1106343
MARQUES, Wilson  TL 1104731, TL 1104755,
TL 1105808, TL 1105342, TL 1105756, MATOS, Ursula Medeiros Araujo de  TL 1105479
TL 1106404, TL 1105247, TL 1105540, MAURICIO, Rebeca Bessa  TL 1106391
TL 1105547, TL 1105781, TL 1105867, MAURÍCIO, Rebeca Bessa  TL 1105082, TL 1106177, TL 1106525,
TL 1105592, TL 1105277, TL 1105314, TL 1106539, TL 1106567,
TL 1105717, TL 1105070, TL 1105785, TL 1106622, TL 1106286
TL 1105239, TL 1106408, TL 1104979,
TL 1105167, TL 1105545, TL 1105588, MAYER, Bibiana  TL 1106258, TL 1106114
TL 1105695, TL 1105714, TL 1105837, MAYER, Mariana Matos  TL 1106198
TL 1105307, TL 1105355, TL 1105509 MAZZA, Giordanno Santana  TL 1105567
MARTIN, Daniel Lordelo San  TL 1106447 MAZZALI, Marilda  TL 1104999
MARTINEZ, Alberto R.M.  TL 1104731, TL 1104979 MAZZONI, Bianca Gomes  TL 1105779, TL 1105758, TL 1105730, TL 1105852,
MARTINEZ, Alberto R M  TL 1105284, TL 1105545 TL 1105849

397
Índice de Autores – XXX Congresso Brasileiro de Neurologia

MECE, Alexandre Motta  TL 1106302 MENDES, Lucas Emmanuel Freitas  TL 1105853


MECÊ, Alexandre Motta  TL 1105564 MENDES, Lucas Silvestre  TL 1106359, TL 1106501, TL 1106158
MEDEIROS, Artur Pereira de França  TL 1105882, TL 1105899 MENDES, Maria Fernanda  TL 1106637
MEDEIROS, Caroline Lourenço de  TL 1105540, TL 1105547, TL 1105509 MENDES, Maria Fernandes  TL 1106406
MEDEIROS, Fabiola Lys de  TL 1105524, TL 1105557 MENDES, Maria Julia  TL 1106312
MEDEIROS, Fabiola Lys de  TL 1105524, TL 1105557 MENDES, Patrícia Marques  TL 1106281, TL 1106127
MEDEIROS, Fabiola Lys  TL 1105102, TL 1106187, TL 1106365, TL 1106418, MENDES, Renan Furtado de Almeida  TL 1106571
TL 1105613 MENDES, Ricardo Rogério  TL 1105456
MEDEIROS, Fabíola Lys de  TL 1106317, TL 1106398, TL 1106467 MENDONCA, Rodrigo Holanda  TL 1106542
MEDEIROS, Fabíola Lys  TL 1105789, TL 1105764, TL 1105815, MENDONÇA, Marcelo Sobrinho  TL 1106554, TL 1106585, TL 1106596
TL 1106184, TL 1105743,
TL 1106210, TL 1105809 MENDONÇA, Mariana Macêdo Militão  TL 1106262, TL 1105301
MEDEIROS, Marina Scop  TL 1105542, TL 1105026, TL 1105295, TL 1105621, MENDONÇA, Rodrigo de Holanda  TL 1106092, TL 1106093,
TL 1105195 TL 1106084, TL 1105178,
TL 1106082, TL 1106094
MEDEIROS, Moisés de Oliveira  TL 1104824
MENDONÇA, Rodrigo de Holanda  TL 1106092, TL 1106093,
MEDEIROS, Paula Fiuza Rodrigues de  TL 1105421, TL 1105859 TL 1106084, TL 1105178,
MEDEIROS, Paula Fiuza Rodrigues  TL 1105703, TL 1105378, TL 1105078, TL 1106082, TL 1106094
TL 1105740 MENDONÇA, Rodrigo Holanda TL 1106377, TL 1105049, TL 1105206,
MEDEIROS, Paulo Ricardo Andrade de  TL 1105189, TL 1105432 TL 1105312, TL 1105650, TL 1106175, TL 1106189,
MEDEIROS, Rafaela Volpini  TL 1106651 TL 1106551, TL 1106600, TL 1106255

MEDEIROS, Sara Diógenes Peixoto de  TL 1106152 MENDONÇA, Romero Antonio Ramos de  TL 1106369

MEDICINA, Escola Paulista de  TL 1104862 MENESES, JÚLIA RACHEL FERREIRA  TL 1106379, TL 1106495

MEDIEROS, Fabiola Lys  TL 1105589 MENESES, JÚLIA RACHEL FERREIRA  TL 1106379, TL 1106495

MEDIEROS, Fabiola Lys  TL 1105589 MENESES, Murilo Sousa  TL 1105459, TL 1105466

MEDINA, Luiza Helena Chuque  TL 1106610, TL 1106406 MENEZES, Catarina Ester Gomes  TL 1105469, TL 1105361, TL 1105898,
TL 1105412, TL 1105697,
MEDRADO, Ana Carolina Godoi  TL 1105696 TL 1105823, TL 1105746
MEDRONHO, Roberto de Andrade  TL 1105757 MENEZES, Felipe Toscano Lins de  TL 1105006
MEES, Wallace  TL 1105537 MENEZES, Gisele Tezolin  TL 1104889
MEIRA, Alex Tiburtino  TL 1106439 MENEZES, Karoline Ferreira Mororo  TL 1106616, TL 1106501, TL 1106493,
MEIRA, Caroline da Silva  TL 1105563 TL 1106632, TL 1106473,
MEIRA, Rodrigo Defensor  TL 1106561, TL 1106615 TL 1106434, TL 1106452

MELGES, Luiz Domingos Mendes  TL 1105131, TL 1105694 MENEZES, Karoline Ferreira Mororó  TL 1106218, TL 1106359, TL 1106429,
TL 1106450, TL 1106466,
MELGES, Natalia Samadello  TL 1105694 TL 1106502, TL 1106308,
MELLO, Alberto Andrade de  TL 1105335 TL 1106537, TL 1106226
MELLO, Ana Lucia Carvalho  TL 1106307 MENEZES, Leonardo Rodrigues Leopoldo de  TL 1105597, TL 1105626,
MELLO, Ana Lucia de Carvalho  TL 1106287, TL 1106319 TL 1105609

MELO, Ailton  TL 1105053, TL 1105627, TL 1105089, TL 1105095 MENEZES, Leonardo Rodrigues Leopoldo TL 1105728, TL 1105535, TL 1105553,
TL 1105749
MELO, Ana Clara Tavares de  TL 1105889
MENEZES, Neila Clediane Sousa  TL 1106492
MELO, Deise Andrade  TL 1105885, TL 1105471, TL 1105487
MENEZES, Raquel Barbosa de  TL 1105853
MELO, Eduardo Sousa de Melo  TL 1106548
MENEZES, Renata Oliveira Almeida  TL 1105009, TL 1105010
MELO, Eduardo Sousa de TL 1106188, TL 1106423, TL 1105773, TL 1106397,
TL 1106444, TL 1106465, TL 1105237, TL 1106548, MENEZES, Renata Oliveira  TL 1105600
TL 1106208, TL 1105631 MENEZES, Éric Moreira  TL 1105853
MELO, Eduardo Souza de  TL 1106120 MERIDA, Kristel Beck  TL 1104942
MELO, Isabelle Diniz  TL 1105937, TL 1105159, TL 1105162 MERIDA, Kristel Larisa Back  TL 1105271, TL 1105267
MELO, João Victor Oliveira de  TL 1105638, TL 1105656, TL 1105742, MESCOLOTTE, Guilherme Menezes  TL 1105630
TL 1105005, TL 1105522 MESCOLOTTI, Guilherme Menezes  TL 1106042
MELO, Juliana Carneiro  TL 1105301, TL 1105318 MESQUITA, Danielle  TL 1106048, TL 1106649
MELO, Juliana  TL 1105053, TL 1105627, TL 1105089, TL 1105095 MESQUITA, Maria Renata Matos de  TL 1105763
MELO, Julia Renata Pinto Correia  TL 1105589 MESQUITA, Maria Thereza Leitão  TL 1106262, TL 1105301
MELO, Julia Renata Pinto Correia  TL 1105589 MESSIAS, Katharina Vieira  TL 1106599
MELO, Kirsten Araujo  TL 1104832 MEURER, Gregorio das Neves  TL 1104955
MELO, Kirsten Araújo  TL 1104833, TL 1105908, TL 1104978 MICHELON, Guilherme  TL 1105571, TL 1106315
MELO, Laiza Paula Candido  TL 1105252, TL 1105380, TL 1105388 MIELKE, João Felipe da Silva  TL 1105877
MELO, Leonardo Guimarães  TL 1106421 MILAGRES, antonio carlos de padua  TL 1106157, TL 1106167
MELO, Mikaely Lima  TL 1106273 MILAGRES, Antonio Carlos  TL 1106228
MELO, Renata Pinheiro Martins de  TL 1106138 MILAN, Rebeca Tamara  TL 1106191
MELO, Átila de Oliveira  TL 1105877 MILANI, Cristiano  TL 1105065
MENDES, Débora Ferreira  TL 1105657 MILHOME, Caroline Barra Souza Santana  TL 1106142
MENDES, Gabrielle Brito Bezerra  TL 1106107, TL 1105164, TL 1105085, MINELLI, Cesar  TL 1105033
TL 1105107, TL 1105145, TL 1105236, TL 1105244
MINIELLO, Lucas Felipe  TL 1105097
MENDES, George Nilton Nunes  TL 1106162, TL 1106245,
TL 1106553, TL 1106570, MINKOVICIUS, Débora Santos  TL 1105177
TL 1106620, TL 1106159 MINÁ, Larissa Brenda Gonaçalves  TL 1106450
MENDES, Lorena Alves  TL 1106479

398
Índice de Autores – XXX Congresso Brasileiro de Neurologia

MINÁ, Larissa Brenda Gonçalves  TL 1106105, TL 1106162, TL 1106620, MORAIS, Gabriel Maciel  TL 1105155, TL 1105223, TL 1105224
TL 1106280, TL 1106090, MORAIS, João Lucas A  TL 1104780
TL 1106429, TL 1106466
MORAIS, Lorenzo Marinho  TL 1106525, TL 1106539, TL 1106192
MIOSSI, João Pedro Moraes  TL 1105705, TL 1105566, TL 1105990, TL 1105799
MORAIS, Lécio Aragão Souza TL 1105469, TL 1105361, TL 1105412, TL 1105697,
MIOTTO, Eliane Correa  TL 1106241 TL 1105823, TL 1105746
MIRANDA, Carolina F. V.  TL 1106319 MORAIS, Norma Martins de Menezes  TL 1106064
MIRANDA, Carolina Franciely Vitor  TL 1106287 MORAIS, Thiago Assis Borges  TL 1106631, TL 1105373, TL 1105061
MIRANDA, Carolina F V  TL 1106307 MORALES, Rogerio Rizo  TL 1105581
MIRANDA, Erica Otoni Pereira  TL 1105842, TL 1105871, TL 1105583 MOREIRA, Adriana Boschi  TL 1105122, TL 1105124
MIRANDA, Jessica Mariana de Aquino  TL 1106023, TL 1106435 MOREIRA, Aline Cortes da Silva  TL 1106563
MIRANDA, José Lucivan  TL 1105937 MOREIRA, Alvaro Jose Porto  TL 1106548, TL 1105230
MIRANDA, João Pedro Rosal  TL 1105534, TL 1105548, TL 1105826 MOREIRA, Amanda Souza  TL 1106215
MIRANDA, Lelis do Vale  TL 1105474 MOREIRA, Ana Lucila  TL 1105213, TL 1105206, TL 1106195, TL 1106400
MIRANDA, Natalia Figueiredo TL 1106145, TL 1106157, TL 1106228, TL 1106167, MOREIRA, Anna Klara Bremer  TL 1105645, TL 1105658
TL 1106058
MOREIRA, Antonio Marcelo  TL 1106588
MIRANDA, Natalia Figueiredo TL 1106145, TL 1106157, TL 1106228, TL 1106167,
TL 1106058 MOREIRA, Arthur Holanda  TL 1106632, TL 1106197, TL 1106216, TL 1106219,
TL 1104998, TL 1105145
MIRANDA, Natália Figueiredo  TL 1106395
MOREIRA, Carolina Lavigne  TL 1104755, TL 1105805, TL 1105695, TL 1105509
MIRANDA, Rita de Cássia  TL 1106442, TL 1105916
MOREIRA, Caroline Lavgne  TL 1105277
MIRÔ, Hugo Salomão Grangeiro Furtado  TL 1105739
MOREIRA, Caroline Lavigne  TL 1105717, TL 1105355
MIYAHIRA, Clara Kimie  TL 1106145, TL 1106157, TL 1106228, TL 1106395,
TL 1106167, TL 1106058 MOREIRA, Heloisa Lopes Cohim  TL 1104864, TL 1105016
MIYAHIRA, Clara Kimie  TL 1106145, TL 1106157, TL 1106228, TL 1106395, MOREIRA, Humberto Lucca Andrade  TL 1105225
TL 1106167, TL 1106058 MOREIRA, Júlio Cesar  TL 1106348
MOHR, Henrique  TL 1105880 MOREIRA, Nicolas Andrade  TL 1105704
MONTAÑO, Iris  TL 1105447, TL 1105747 MOREIRA, Stefan  TL 1105116, TL 1105134, TL 1105329
MONTE, Paula Carolina Brabo  TL 1105575 MOREIRA, Vanessa de Freitas  TL 1105299, TL 1105274,
MONTEIRO, Ana Karine da Costa  TL 1106065 TL 1105353, TL 1105647,
TL 1105315, TL 1105629
MONTEIRO, ANA KAROLINE da COSTA  TL 1106064, TL 1106065
MOREIRA, Vanessa Freitas  TL 1106349
MONTEIRO, ANA KAROLINE da COSTA  TL 1106064, TL 1106065
MOREIRA, Wirna Queiroz Belém  TL 1106117, TL 1106544
MONTEIRO, Carlos Mello  TL 1104937, TL 1104938
MOREIRA, Álvaro José Porto  TL 1106120
MONTEIRO, Enzo Barnabé  TL 1105483
MOREIRA, Ávaro Jose Porto  TL 1105774
MONTEIRO, Kathleen Torres Tenorio  TL 1105066, TL 1105114
MOREIRA-NETO, Acácio  TL 1105036
MONTEIRO, Kathleen Torres Tenório  TL 1105770
MORENO, Beatriz do Nascimento Garcia  TL 1106040
MONTEIRO, Larissa  TL 1105053
MORENO, Cristiane A. M.  TL 1104679
MONTEIRO, Lucas Nascimento  TL 1106181, TL 1106651, TL 1106578,
TL 1106638 MORENO, Cristiane Araujo Martins  TL 1105158, TL 1106274, TL 1105650,
TL 1106270, TL 1106411,
MONTEIRO, Marcus Vinícius Rocha  TL 1106506 TL 1106424, TL 1106436,
MONTEIRO, Mateus Brasil Câmara  TL 1105907 TL 1106445
MONTEIRO, Matheus Brazil Câmara  TL 1106589, TL 1105272, TL 1105900, MORENO, Cristiane Araújo Martins  TL 1105344, TL 1106084,
TL 1105419 TL 1105048, TL 1105049,
MONTEIRO, Pauliana Alencar  TL 1105244 TL 1105176, TL 1106175,
TL 1106189, TL 1106047
MONTEIRO, Waldélia Maria Santos  TL 1106558
MORENO, Cristiane de Araujo Martins  TL 1106550, TL 1105178,
MONTENEGRO, Ana Cristina  TL 1106131 TL 1106416, TL 1105186,
MONTENEGRO, Clarissa Rocha  TL 1106220, TL 1106488 TL 1106400, TL 1106255
MONTENEGRO, Demetrius  TL 1105631 MORENO, Cristiane de Araújo Martins  TL 1105565, TL 1105768, TL 1105713,
MONTOURO, Laura Alonso Matheus  TL 1105197, TL 1105154, TL 1105190, TL 1105739
TL 1105232, TL 1105521 MORENO, Mariana Santaliz de Godoy  TL 1106545
MONTOYA, Marco Tulio Medina  TL 1105118, TL 1106237 MORESCO, Rafaela Maria  TL 1105240, TL 1105242
MORAES, Débora Beserra Vilar  TL 1105070 MORGANTETTI, Giuliano Ferreira  TL 1105301, TL 1105318
MORAES, Geridice Lorna Andrade  TL 1106410 MORITA, Maria da Penha Ananias  TL 1105190, TL 1105176
MORAES, Kalec Thiago Simonek de  TL 1106506 MORITA, Maria da Penha  TL 1104679
MORAES, Marianna Pinheiro Moraes de  TL 1105718 MORO, Carla Heloisa Cabral  TL 1105096, TL 1105033
MORAES, Rafael Ragazzi de  TL 1105148 MORO, Giuliana  TL 1106156
MORAES, Samia Talise El Horr de  TL 1106191, TL 1106480 MORO, Kelmer Mozer  TL 1105314
MORAIS, Alessandra Braga Cruz Guedes de  TL 1106162, TL 1106179, MORO, Maria Ludmila Setti Aguiar  TL 1106329
TL 1106229, TL 1106553, MORORÓ, Karoline Ferreira Menezes  TL 1106233, TL 1106107
TL 1106570, TL 1106218,
TL 1106161, TL 1106142, MORROW, Jasper  TL 1105714
TL 1106159, TL 1106236, MOSINI, Amanda Cristina  TL 1106009
TL 1106226 MOTA, José Ítalo Soares  TL 1106226
MORAIS, Alessssandra Braga Cruz Guedes de  TL 1106308 MOTA, João Gabriel Teixeira  TL 1106447, TL 1106507
MORAIS, Antonio Higor Freire de  TL 1106263 MOTA, Kelly Christina da  TL 1105029
MORAIS, Fabiano Moulin de  TL 1105016 MOTA, Luciano de Albuquerque  TL 1105435, TL 1105159, TL 1105162,
MORAIS, Gabriela Lopes de  TL 1105362, TL 1105342, TL 1106404, TL 1105798, TL 1105405, TL 1105593,
TL 1106566, TL 1105781 TL 1106335, TL 1106010

399
Índice de Autores – XXX Congresso Brasileiro de Neurologia

MOTA, Simone da  TL 1105988 NEIVA, Marcia Silva Santos  TL 1106320


MOTTA, MAriana  TL 1105880 NEPOMUCENO, Camila Rodrigues  TL 1105340, TL 1106459
MOURA, Ademir Aragão  TL 1105348, TL 1105423 NERES, Agnes Laura Silva  TL 1105510, TL 1105307, TL 1105599
MOURA, Alissa Elen Formiga TL 1106090, TL 1106502, TL 1106414, TL 1106473, NERI, Beatriz Rodrigues  TL 1105107, TL 1105236
TL 1106434, TL 1106452, NERI, Lidianne Ramos  TL 1105655
TL 1105945, TL 1106649
NERO, Antonio de cesare Del  TL 1106074
MOURA, Amanda Gonçalves  TL 1104780
NERTO, Oscar Loiola Alencar  TL 1106586
MOURA, Carolina Braga  TL 1105897
NERY, Lia Belchior  TL 1106586
MOURA, Caroline Farias Barreto de  TL 1106410
NESELLO, Alberto Chitolina  TL 1106054
MOURA, Dafne Dalledone  TL 1105410
NETA, Pedro Braga  TL 1106622
MOURA, Edmilson Leal Bastos  TL 1105196
NETO, Adalberto Studart  TL 1106246
MOURA, Fernanda Maria Gonçalves de Sousa  TL 1106019, TL 1105754
NETO, Adelmar Marinho  TL 1106251, TL 1106403
MOURA, Victor Elmo Gomes Santos de  TL 1106262
NETO, Agabio Diógenes Pessoa  TL 1106133
MOURA, Álissa Elen Formiga  TL 1106107, TL 1106429, TL 1106466, TL 1106626
NETO, Agábio Diógenes Pessoa  TL 1105256
MOURA, Álissa Ellen Formiga  TL 1106233, TL 1106604
NETO, Alberto Martins Pina Rodrigues  TL 1104809, TL 1104807, TL 1106506
MOURA, Érica Dapont  TL 1104844
NETO, Antonio Pereira Gomes  TL 1106389, TL 1106659
MOURÃO, Flavia Costa  TL 1105728, TL 1105535, TL 1105553, TL 1105749
NETO, Antonio Serpa Amaral  TL 1105194, TL 1105642
MOURÃO, Flavia da Costa  TL 1105597, TL 1105626, TL 1105609
NETO, Antônio Alves Sobreira  TL 1105082, TL 1106567
MUIÑOS, Pedro Jr  TL 1104775
NETO, Antônio Alves  TL 1106192
MUNHOZ, Pamella Christine de Sousa  TL 1106502
NETO, Antônio de Matos Lima  TL 1104975, TL 1105323
MUNIZ, Davi Dantas  TL 1106316, TL 1106489
NETO, Eduardo Bernardo Chaves  TL 1105359, TL 1105769,
MUNIZ, Débora de Moura  TL 1106239 TL 1105839, TL 1106326,
MUNIZ, Gabriela Oliveira  TL 1105301 TL 1106500, TL 1105920
MUNIZ, Maria Carolina Rocha  TL 1106262, TL 1105301, TL 1105318 NETO, Eduardo de Moura  TL 1106008
MURRAY, Brian James  TL 1106541 NETO, Eliseu Paglioli  TL 1105407
MUSA, Lucas  TL 1104936 NETO, Elizeu Pereira dos Santos  TL 1105534
MUTARELLI, Eduardo Genaro  TL 1105421, TL 1105703, TL 1105740 NETO, Feres Eduardo Aparecido Chaddad  TL 1105615
NADIME, Yasmin Jose  TL 1106449 NETO, Fernando de Paiva Melo  TL 1106597, TL 1106555, TL 1106458,
NAGEL, Vivian  TL 1105096 TL 1106588

NAGEM, Danilo Alves Pinto  TL 1106384, TL 1106426 NETO, Fernando de Paiva Melo  TL 1106597, TL 1106555, TL 1106458,
TL 1106588
NAGEM, Danilo Alves Pinto  TL 1106384, TL 1106426
NETO, Francisco Mauricio Maia  TL 1105414
NAKAMURA, Eduardo Yukio  TL 1105133, TL 1105366, TL 1105372
NETO, Francisco Moura  TL 1104886
NAKAMURA, Marcelo Tohoru  TL 1105081
NETO, Franklin de Castro Alves  TL 1106391, TL 1106526, TL 1106622,
NAKANO, Bruno Eiji  TL 1105472, TL 1105131, TL 1105694 TL 1106385, TL 1106641
NAKAO, Natalia de Castro Fim  TL 1106413 NETO, Franklin de Castro Alves  TL 1106391, TL 1106526, TL 1106622,
NAKAZATO, Paula Carolina Grande  TL 1105472, TL 1105131, TL 1105694 TL 1106385, TL 1106641
NARIMATSU, Keila  TL 1106516, TL 1106610, TL 1106576 NETO, Franklin de Castro Aves  TL 1106402
NASCIMENTO, Ana Dolores Firmino Santos do  TL 1106427, TL 1106496, NETO, Franklin de Castro  TL 1106286
TL 1105125, TL 1105512 NETO, Gilberto José de Melo  TL 1105534, TL 1105814
NASCIMENTO, Arthur Costa  TL 1105881, TL 1105581, TL 1105891 NETO, Haldson Cesar Barbosa  TL 1105189, TL 1105432
NASCIMENTO, Bruna Essado  TL 1105528 NETO, Heitor Nunes de Oliveira Sento-Sé  TL 1105552, TL 1105293
NASCIMENTO, Denner Matheus Ribeiro  TL 1105783 NETO, Heitor Nunes Oliveira Sento-Sé  TL 1106459
NASCIMENTO, Fabricio Porto do  TL 1105560 NETO, Herval Ribeiro Soares TL 1105738, TL 1105299, TL 1105274, TL 1105353,
NASCIMENTO, Filipe Barroso  TL 1105892 TL 1105822
NASCIMENTO, Isaíra A P S  TL 1106440, TL 1106490, TL 1106503 NETO, Ismar Andrade da Silveira  TL 1105294
NASCIMENTO, Jeddson Rêgo  TL 1106418 NETO, Jasson José Moscon  TL 1105168
NASCIMENTO, Marcia Helena Cassago  TL 1106531 NETO, Joaquim Fechine de Alencar  TL 1105895, TL 1105182
NASCIMENTO, Monique Evelyn Mendonça do  TL 1106427, NETO, Jose Hortencio dos Santos  TL 1106453
TL 1106496, TL 1105125, NETO, José Dias de Assis  TL 1105799
TL 1105512, TL 1106569
NETO, José Elias Makaron  TL 1106329, TL 1106206
NASCIMENTO, Osvaldo J. M.  TL 1104755
NETO, José Lopes Tabatinga  TL 1106185
NASCIMENTO, Osvaldo J M  TL 1106097
NETO, José Luis Lima  TL 1106305
NASCIMENTO, Osvaldo José Moreira do  TL 1105869, TL 1105479, TL 1105782
NETO, José Ribamar Pereira  TL 1106375, TL 1106390, TL 1106392
NASCIMENTO, Osvaldo José Moreira  TL 1106095, TL 1105239, TL 1105406
NETO, João Batista Guerra Barretto  TL 1106131
NASCIMENTO, Osvaldo  TL 1105824, TL 1105879, TL 1106300
NETO, João Henrique Orsi  TL 1105270
NASCIMENTO, Oswaldo  TL 1106380
NETO, Luis Bandeira Alves  TL 1105895, TL 1105182
NASCIMENTO, Priscila Alves  TL 1105031
NETO, Manoel Alves Sobreira TL 1106125, TL 1106177, TL 1106525, TL 1106567,
NASCIMENTO, Rairis Barbosa  TL 1106631, TL 1105348, TL 1105423 TL 1106622, TL 1106414,
NASCIMENTO, Simony Lira do  TL 1106057, TL 1106217 TL 1106091, TL 1106581,
NASCIMENTO, Suzete Farias da Guarda  TL 1105177 TL 1106103, TL 1105945,
TL 1106048, TL 1106586
NAUFEL, Maria Fernanda Soares  TL 1106618
NETO, Manoel Moreira  TL 1105320
NAUFEL, Maria Fernanda  TL 1106313
NETO, Moacir Pereira Leite  TL 1105046, TL 1104966
NEDER, Luciano  TL 1104755
NETO, Nivaldo Alves Calado  TL 1106588
NEIDORF, Leonardo  TL 1105165

400
Índice de Autores – XXX Congresso Brasileiro de Neurologia

NETO, Octavio Marques Pontes  TL 1105338, TL 1105875 NUNES, Diego Eduardo  TL 1105414
NETO, Octávio Marques Pontes  TL 1105450, TL 1105461, TL 1106528, NUNES, Glaucus Fernando Vieira  TL 1105759
TL 1105798 NUNES, Helio Rubens de Carvalho  TL 1106435
NETO, Olavo Santa Cruz Valadares  TL 1105557 NUNES, João Victor da Costa  TL 1105445, TL 1105892
NETO, Otilio Paulo da Silva  TL 1106369 NUNES, Julia de Melo  TL 1106419, TL 1106538
NETO, Otávio da Cunha Ferreira  TL 1105895, TL 1105182 NUNES, Juliana de Oliveira  TL 1105752
NETO, Paulo Barbosa Leite  TL 1105102, TL 1105789, TL 1105524, TL 1106187, NUNES, Júlia de Melo  TL 1105853
TL 1106365, TL 1105764,
TL 1105815, TL 1106184, NUNES, Maria Cristina de Alencar  TL 1105267
TL 1106467, TL 1105743, NUNES, Patricia Pereira  TL 1104833, TL 1104832
TL 1106210, TL 1105557, NUNES, Patrícia Pereira  TL 1105908, TL 1104978, TL 1106578
TL 1105809, TL 1106418,
TL 1105589, TL 1105613 NUNES, Rebeca Holanda  TL 1106215
NETO, Paulo Roberto Matos  TL 1106589, TL 1105272, TL 1105419, TL 1106264 NUNES, Thabata Emanuelle Martins  TL 1106279, TL 1106278, TL 1106475
NETO, Pedro Braga  TL 1106125, TL 1106177, TL 1106525, TL 1106539, NUNES, Thábata Emanuelle Martins  TL 1105507, TL 1105790,
TL 1106567, TL 1106414, TL 1106199, TL 1106205,
TL 1106076, TL 1106192, TL 1105185, TL 1106527
TL 1106385, TL 1106286, NUNES, Zuriel Rodrigues Seixas  TL 1105005
TL 1106402, TL 1106641, NÓBREGA, Ivna Lacerda Pereira  TL 1106158
TL 1106581, TL 1106103,
TL 1106081, TL 1105763, NÓBREGA, Katia Cirilo Costa  TL 1105682
TL 1105945, TL 1106048, NÓBREGA, Kátia Cirilo Costa  TL 1105051
TL 1104998, TL 1105085, NÓBREGA, Paulo Ribeiro  TL 1106125, TL 1105082, TL 1106177,
TL 1105107, TL 1105145, TL 1106525, TL 1106539,
TL 1105236, TL 1105244 TL 1106567, TL 1106622,
NETO, Raimundo Marcial Brito  TL 1105462, TL 1105897, TL 1105741, TL 1106431, TL 1106414,
TL 1106300 TL 1106223, TL 1105316,
NETO, Raimundo Marcial de Brito  TL 1105869, TL 1105854, TL 1105878, TL 1106196, TL 1106581,
TL 1105710 TL 1106103, TL 1106207
NETO, Túlio Maranhão  TL 1106337, TL 1105277, TL 1105717 ODA, Elder Yanaze  TL 1105420
NEVE, Anuja  TL 1105660 OGUSUKU, Caroline Suemi  TL 1105115, TL 1105340, TL 1106343
NEVES, Carolina Daher de Alencar  TL 1105889 OKADA, Mariana  TL 1106287, TL 1106319, TL 1106307
NEVES, Larissa Lorrhan Santos  TL 1106380 OKAMOTO, Erika  TL 1105036
NEVES, Luciana Oliveira  TL 1106220, TL 1106488 OLIVAL, Guilherme Sciascia  TL 1104936
NEVES, Pedro Rodrigues  TL 1106298, TL 1106301, TL 1105820 OLIVEIRA, Acary Souza Bulle  TL 1104864, TL 1104860, TL 1104862,
TL 1105212, TL 1105221,
NEVES, Rafaela de Souza Dias  TL 1105575 TL 1105226, TL 1105283,
NICARETTA, Denise Hack  TL 1105921 TL 1105285, TL 1105559,
NICOLI, Fernanda Michely  TL 1105833 TL 1105602, TL 1105633,
TL 1104874, TL 1104940,
NIEDERAUER, Andressa Gomes  TL 1104970, TL 1106463 TL 1105428, TL 1105516,
NIMER, Gabriela Flor  TL 1105462 TL 1105560, TL 1106189
NISHIYAMA, Alzira Nobuko  TL 1105369 OLIVEIRA, Acay Souza Bulle  TL 1105536
NITRINI, Ricardo  TL 1104692, TL 1106140, TL 1106259 OLIVEIRA, Afonso Henrique Costa de  TL 1106544
NOBREGA, Ana Caline  TL 1105089, TL 1105095 OLIVEIRA, Americo Danuzio Pereira  TL 1105252, TL 1105380, TL 1105388
NOBREGA, Ivna Lacerda Pereira  TL 1106501 OLIVEIRA, Ana Beatriz Garcez de  TL 1105696
NOBREGA, Paulo Ribeiro  TL 1106076, TL 1106656, TL 1105945 OLIVEIRA, Ana Carolina Silveira  TL 1105892
NOGUEIRA, Bruno Miceli Gonzalez  TL 1106311 OLIVEIRA, Ana Gabriella Camelo  TL 1106523
NOGUEIRA, Carlos Bruno  TL 1105406 OLIVEIRA, Ana Helena Barbalho Bezerra de  TL 1104805
NOGUEIRA, Clebeson Azevêdo  TL 1105245, TL 1105391 OLIVEIRA, Ana Maria dos Santos  TL 1105684, TL 1105725
NOGUEIRA, Guilherme Nobre  TL 1106185, TL 1105780 OLIVEIRA, Ana Paula Pessoa de  TL 1105563
NOGUEIRA, Gustavo Carvalho  TL 1106617 OLIVEIRA, Ana Vitória Costa de  TL 1106544
NOGUEIRA, Luciana Tolstenko  TL 1105870 OLIVEIRA, Andrea Lira  TL 1104707
NOGUEIRA, Mateus Henrique  TL 1106353, TL 1106607 OLIVEIRA, Andre Dias  TL 1105194
NOGUEIRA, Matheus Henrique  TL 1106312 OLIVEIRA, André Dias de  TL 1105409
NOGUEIRA, Patrícia T. B.  TL 1104755 OLIVEIRA, Anna Melissa Noronha  TL 1105593
NOLETO, Gustavo Sousa  TL 1105732, TL 1105744, TL 1105861, TL 1105870 OLIVEIRA, Arlindo Américo de TL 1105347, TL 1105349, TL 1105123, TL 1105329
NOLETO, Gustavo Sousa  TL 1105732, TL 1105744, TL 1105861, TL 1105870 OLIVEIRA, Augusto César Penalva de  TL 1106451
NONES, Diogo Pasquali  TL 1105116, TL 1105123, TL 1105134, TL 1105165, OLIVEIRA, Beatriz Barrozo Gonzalez de  TL 1105961
TL 1105329 OLIVEIRA, Beatriz Barrozo Gonzalez  TL 1106442, TL 1106481, TL 1105753
NOUGUEIRA, Mateus Henrique  TL 1106074 OLIVEIRA, Bianca Etelvina dos Santos de  TL 1106588
NOVAES, Bernardo Caetano  TL 1105684, TL 1105725 OLIVEIRA, Bruna Andrade de  TL 1105525
NOVAES, Maria Rita Carvalho Garbi  TL 1106075 OLIVEIRA, Camila Maria  TL 1104685
NOVAIS, Aurea Maria Lago  TL 1106134 OLIVEIRA, Camilla Ribeiro de  TL 1105528, TL 1105576
NOVAIS, Rodrigo Nobre de  TL 1106120 OLIVEIRA, Carlos Mauricio  TL 1104707, TL 1105892, TL 1105948, TL 1105487
NOVAK, Edison Matos  TL 1106191 OLIVEIRA, Celina de  TL 1106306
NOVAK, Felipe Trevisan Matos  TL 1106441 OLIVEIRA, Clara Maria Limaverde  TL 1105763
NOVIS, Luiz Eduardo  TL 1105507, TL 1105790, TL 1106279, TL 1106278 OLIVEIRA, Clariana Nascimento de  TL 1105447, TL 1105747
NUCCI, Anamarli TL 1106302, TL 1106363, TL 1105564, TL 1106352, TL 1104979 OLIVEIRA, Clarice Pereira Sales  TL 1105586, TL 1105599

401
Índice de Autores – XXX Congresso Brasileiro de Neurologia

OLIVEIRA, Daniel Alves de  TL 1106188, TL 1105734, TL 1105777, TL 1106423, OLIVEIRA, Luis Henrique Sunderhus de  TL 1105716, TL 1105566, TL 1105990,
TL 1105773, TL 1106397, TL 1105799
TL 1106444, TL 1106465, OLIVEIRA, Luiza Barbosa de  TL 1105528, TL 1105576
TL 1105237, TL 1106548
OLIVEIRA, Luiz Fernando Rodrigues de  TL 1105115
OLIVEIRA, Daniel Sabino de  TL 1105253
OLIVEIRA, Luiz Fernando  TL 1106249
OLIVEIRA, Danilo Nunes  TL 1106391, TL 1106262, TL 1106604, TL 1105933,
TL 1105945, TL 1106626 OLIVEIRA, Maira Okada de  TL 1105056
OLIVEIRA, Dayana Dourado de  TL 1106481, TL 1105259, TL 1106078 OLIVEIRA, Marcella Cabral de  TL 1105692
OLIVEIRA, Denise Nunes  TL 1105301, TL 1105318 OLIVEIRA, Marcos Castello Barbosa  TL 1105206
OLIVEIRA, Diego Silveira  TL 1106555 OLIVEIRA, Maria Clara Barbosa de  TL 1105155, TL 1105223, TL 1105224
OLIVEIRA, Déborah Castro Ferreira de  TL 1105359, TL 1105839, TL 1105920 OLIVEIRA, Maria de  TL 1106606
OLIVEIRA, Déborah Castro Ferreira  TL 1105769, TL 1106500 OLIVEIRA, Mariana Pacheco de  TL 1106491
OLIVEIRA, Déborah de Castro Ferreira de  TL 1106326 OLIVEIRA, Marina Rossi  TL 1105235
OLIVEIRA, Elienay Cassio  TL 1105730 OLIVEIRA, Matheus Franco Andrade  TL 1106523
OLIVEIRA, Enedina Maria Lobato  TL 1105006 OLIVEIRA, Milena Fernandes de  TL 1106619, TL 1105819, TL 1106428,
TL 1106611
OLIVEIRA, Enrico Pinheiro de  TL 1106526
OLIVEIRA, Murilo A  TL 1104775
OLIVEIRA, Enzo Derick Guterres  TL 1106442, TL 1105259
OLIVEIRA, Pablo Nascimento  TL 1105115, TL 1105552, TL 1106249
OLIVEIRA, Ericles Willy Machado de  TL 1105474
OLIVEIRA, Paula Luanna Carvalho de  TL 1105763
OLIVEIRA, Fabio de Nazaré  TL 1105521
OLIVEIRA, Paulo Henrique Martinelli  TL 1105666, TL 1105686, TL 1105699,
OLIVEIRA, Fabio Pascotto de  TL 1105088 TL 1105264, TL 1105390,
OLIVEIRA, Fernanda Aquino de  TL 1105381 TL 1105691, TL 1105641
OLIVEIRA, Francisca Adna Almeida de  TL 1105301 OLIVEIRA, Pedro Antonio Medeiros de  TL 1106549
OLIVEIRA, Francisco Tomaz Meneses de  TL 1106516, TL 1106610 OLIVEIRA, Pedro Barbosa  TL 1105150, TL 1105428, TL 1105726
OLIVEIRA, Fábio de Nazaré  TL 1105151 OLIVEIRA, Pedro Dantas  TL 1106437
OLIVEIRA, Gabriela Sanford Guimarães Caminha de  TL 1106226 OLIVEIRA, Pietra Baggio Peixoto de  TL 1105787, TL 1105302, TL 1104977
OLIVEIRA, Gabriel Carvalho  TL 1106474, TL 1106557 OLIVEIRA, Pietra Baggio Peixoto  TL 1105305
OLIVEIRA, Gabriel Cipriano Feitosa  TL 1106621 OLIVEIRA, Ricardo Santos de  TL 1105133
OLIVEIRA, Gabriel Salim Saud de  TL 1106506 OLIVEIRA, Roberto Teodoro Gurgel de  TL 1106062, TL 1106133
OLIVEIRA, Giovana Andrade de  TL 1105842, TL 1105871, TL 1105583 OLIVEIRA, Rodrigo Almeida Magalhães  TL 1106428
OLIVEIRA, Giuliano da Paz  TL 1105567 OLIVEIRA, Samantha Lopes  TL 1106605, TL 1106630
OLIVEIRA, Guiomar Nascimento de  TL 1105034 OLIVEIRA, Sarah Almeida Sales de  TL 1106544
OLIVEIRA, Gustavo Mesquita de  TL 1106090 OLIVEIRA, Sebastiao Carlos de Sousa  TL 1106042
OLIVEIRA, Helvia Bertoldo de  TL 1105559 OLIVEIRA, Silmara Ferreira de  TL 1105732, TL 1105744
OLIVEIRA, Herisson Rodrigues de  TL 1106059, TL 1106419, TL 1105913, OLIVEIRA, Steffany Protásio Moura de  TL 1105783
TL 1106562, TL 1105874, OLIVEIRA, SUZANA NOVAIS TEIXEIRA  TL 1105084
TL 1106571
OLIVEIRA, Thais Barbosa de  TL 1105819, TL 1106428, TL 1106611
OLIVEIRA, Hyago Casimiro Mendes de  TL 1105168
OLIVEIRA, Thales Junqueira  TL 1105510
OLIVEIRA, Hélvia Bertoldo de  TL 1105283, TL 1105633
OLIVEIRA, Thalis Silva de  TL 1106298, TL 1106301
OLIVEIRA, Igor de  TL 1106548
OLIVEIRA, Thaís Barbosa de  TL 1106619, TL 1106591
OLIVEIRA, Isabelle Jacqueline Weber  TL 1106574
OLIVEIRA, Vanessa Fernandes de  TL 1105763
OLIVEIRA, Isabelle Jacqueline Weber  TL 1106574
OLIVEIRA, Vinicius Pafume de  TL 1105601
OLIVEIRA, Isabel Lopes de  TL 1106343
OLIVEIRA, Viviane de Sousa  TL 1106410
OLIVEIRA, Isadora Castro Ferreira  TL 1105769, TL 1106500
OLIVEIRA, Yuri Almeida  TL 1106492
OLIVEIRA, Isadora de Castro Ferreira de  TL 1105359, TL 1105839, TL 1106326,
TL 1105920, TL 1105909 OLIVEIRA-FILHO, Jamary  TL 1104775, TL 1105655, TL 1105672, TL 1106272,
TL 1105665
OLIVEIRA, Isadora Mônica Ponte de  TL 1106222
OLIVÉ, Montse  TL 1106175
OLIVEIRA, Jessica Blanc Leite  TL 1106302
ONO, Carla Rachel  TL 1104692
OLIVEIRA, João Gabriel Mansano de  TL 1105377
ORGE, Carolina  TL 1105053, TL 1105627
OLIVEIRA, João Gustavo dos Anjos Morais  TL 1106477
ORIKASA, Guilherme Seiti  TL 1105556
OLIVEIRA, Juliana Marilia Pereira de  TL 1105551
ORRICO, Camila  TL 1105447, TL 1105053, TL 1105747
OLIVEIRA, Juliana Martini  TL 1106135
ORSOLIN, Priscila Capelari  TL 1105595
OLIVEIRA, Juliana Martini  TL 1106135
ORTEGA, Adriana Banzzatto  TL 1106551
OLIVEIRA, Lais Machado de  TL 1105861
ORTH, Luiza  TL 1105477, TL 1105302, TL 1104977, TL 1105776
OLIVEIRA, Leandro Freitas  TL 1105872, TL 1106152
OSBORN, Ellen  TL 1105181
OLIVEIRA, Leandro  TL 1104712
OTTONI, Letícia Pires  TL 1105770
OLIVEIRA, Leo Araújo  TL 1104844
PACE, Filipe Tupinambá Di  TL 1105377, TL 1105076
OLIVEIRA, Letícia Bezerra de  TL 1105704
PACHECO, Ana Beatriz Vieira  TL 1106067, TL 1106148
OLIVEIRA, Letícia Farias  TL 1105639
PACHECO, Evelyn TL 1106287, TL 1106319, TL 1106139, TL 1106307, TL 1106360
OLIVEIRA, Lilian Vieira de Sousa  TL 1105528
PACHECO, Glauber Mota  TL 1105601, TL 1105510
OLIVEIRA, Liêver Moura de  TL 1105164
PACHITO, Daniela  TL 1104957
OLIVEIRA, Lucas Casimiro de  TL 1105420
PACIFICO, Fernando Augusto  TL 1106617
OLIVEIRA, Lucas de Souza  TL 1105325
PACÍFICO, Fernando Augusto  TL 1106583
OLIVEIRA, Luciana Mattos Barros  TL 1105665
PADILHA, Wilton Wilney Nascimento  TL 1105853
PAES, Maria Alice da Silva  TL 1105921

402
Índice de Autores – XXX Congresso Brasileiro de Neurologia

PAIVA, Ana Letícia Lacerda  TL 1106583 TL 1106385, TL 1106402,


PAIVA, Anderson Rodrigues Brandão de  TL 1106085, TL 1106370 TL 1105236, TL 1105244

PAIVA, Eduardo dos Santos  TL 1105784 PELLEGRIN, Eduarda de  TL 1105138

PAIVA, Gabriel Pina  TL 1105197, TL 1105154, TL 1105232, TL 1105151, PELLINI, Rodrigo Teixeira  TL 1106329
TL 1105521 PENA-PEREIRA, Marcio  TL 1106647
PAIVA, Isadora  TL 1105847 PENICHE, Paula da Cruz  TL 1105508
PAIVA, Kettleyn Alves  TL 1106273 PEREIRA, Arthur Braga  TL 1105370, TL 1105346
PAIVA, Lara  TL 1105847, TL 1105865 PEREIRA, Ayllan Pabllo Viana Nunes  TL 1105639
PAIVA, Michelle Abdo  TL 1105158, TL 1105213, TL 1105358, TL 1105178, PEREIRA, Camila B  TL 1104775
TL 1106082, TL 1106416 PEREIRA, Cristiana Borges  TL 1105198, TL 1105292
PAIXÃO, Barbara Oliveira  TL 1105370 PEREIRA, Deny Glauber  TL 1105765
PAIXÃO, Bárbara Oliveira  TL 1105346 PEREIRA, Felipe Salvagni  TL 1106522
PALHARES, Rafael Andrade  TL 1105657 PEREIRA, Flávio Faria Costa  TL 1105196
PALMINI, Andre  TL 1105407 PEREIRA, Fábio Araujo  TL 1105472
PAOLILO, Renata Barbosa  TL 1106432 PEREIRA, Fábio de Araújo  TL 1105694
PAPAIZ, Fabiano  TL 1106263 PEREIRA, Ivanio Alves  TL 1106198, TL 1106257, TL 1105208, TL 1105209
PARANHOS, Daniel Rocha  TL 1106135, TL 1106134, TL 1106118 PEREIRA, Jorge Fernando de Miranda  TL 1105378
PARANHOS, Daniel Rocha  TL 1106135, TL 1106134, TL 1106118 PEREIRA, Jéssica Gonçalves  TL 1106168
PARENTE, Ana Raquel Jucá  TL 1105455 PEREIRA, Katriane Endiel  TL 1105240, TL 1105242
PARENTE, Enzo Lima Alcântara  TL 1105791 PEREIRA, Késia Sindy Alves Ferreira  TL 1106391, TL 1106526, TL 1106177,
PARMERA, JAcy Bezerra  TL 1104692 TL 1106567, TL 1106385,
PASETO, Ryann Pancieri  TL 1106455 TL 1106402, TL 1106641

PASQUALLI, Aline Lourdes  TL 1105347 PEREIRA, Késia Sindy  TL 1106192, TL 1106286

PASSOS, Giordani dos  TL 1105398 PEREIRA, Laura Gomes  TL 1105026, TL 1105295

PASSOS, Giordani Rodrigues dos  TL 1105925 PEREIRA, Mariana Ribeiro  TL 1105431, TL 1105495, TL 1105442, TL 1105443,
TL 1105473, TL 1105485
PASSOS, Luiz Carlos  TL 1106563
PEREIRA, Marina Mayara  TL 1106522, TL 1106484, TL 1106595, TL 1106511
PASTORIO, Indianara Keila  TL 1105264, TL 1105390, TL 1105641
PEREIRA, Paula Vieira  TL 1105672
PASTÓRIO, Indianara Keila  TL 1105666, TL 1105686, TL 1105699, TL 1105691
PEREIRA, Rodrigo Moreira  TL 1105066, TL 1105114
PATATT, Julia  TL 1105295
PEREIRA, Rouse Barbosa  TL 1105406
PATATT, Júlia  TL 1105026
PEREIRA, Valeria Coelho Santa Rita  TL 1105570, TL 1105757, TL 1105269
PAUL, Friedemann  TL 1105040
PEREIRA, Valéria Coelho Santa Rita  TL 1106311, TL 1105607
PAULA, Luiza Carvalho de  TL 1106239
PERERIRA, Mariana Ribeiro  TL 1105438
PAULA, Telio Diego Cantalice de  TL 1105379
PERES, Daniele  TL 1105123
PAULA, Thomas Vieira de  TL 1106378, TL 1105724, TL 1105863, TL 1105296
PERES, Gabriel Hoher  TL 1105349, TL 1105116
PAULA, Thomas Viera de  TL 1105635
PERES, Mario  TL 1105218
PAULA, Vanessa Salete de  TL 1106168
PEREZ, Jairo Aragão  TL 1106588
PAULO, Dalla Cristiane Sampaio de Sousa  TL 1106369
PERIN, Daiana  TL 1106068
PAULO, Larissa Emile  TL 1106206
PERINI, Laís Bissoli  TL 1105409, TL 1105395
PAZ, Gabriela  TL 1105988
PERINO, Lais Bissoli  TL 1105194, TL 1105642
PAZ, José Albino da  TL 1106432
PERISSINOTO, Jacy  TL 1105181
PAZINI, Andreia Martini  TL 1105429, TL 1106357
PERISSINOTTI, Iago Navas  TL 1105336
PAZINI, Andréia Martini  TL 1105503
PERUFFO, Daniel Wallbach  TL 1105271
PB, Bianca Etelvina Santos de Oliveira. João Pessoa  TL 1106597, TL 1106458
PESSOA, Alberlucio Esquirio  TL 1105807
PE, Wilson Alves de Oliveira Júnior. Recife  TL 1106208
PESSOA, Alberlúcio Esquirio  TL 1105294
PECORARI, Alana Gomes  TL 1106318
PESSOA, André Luiz Santos  TL 1106376, TL 1106381, TL 1106396, TL 1106091
PEDRO, Matheus Kahakura Franco  TL 1105646, TL 1105464, TL 1105459,
TL 1105466 PESSOA, André  TL 1106649

PEDROSO, Jose L  TL 1105284 PESSOA, Fabricio Silva  TL 1105812

PEDROSO, Jose Luiz  TL 1106635, TL 1106656 PESSOA, Paola Maria Barros Diógenes  TL 1106410

PEDROSO, José Luiz  TL 1104731, TL 1104791, TL 1105150, TL 1104711, PESSOA, Sheila Ferreira  TL 1106085, TL 1105767, TL 1106370, TL 1105417
TL 1105785, TL 1106408, PETREÇA, Daniel Rogério  TL 1105329
TL 1104979, TL 1105545 PETRONILHO, Fabricia  TL 1106293, TL 1106491
PEDROZO, José Luiz  TL 1105166 PETRONILHO, Fabricia  TL 1106293, TL 1106491
PEDROZO, Leidys Marina  TL 1105398, TL 1105408 PETRONILHO, Gabriel Bagarolo  TL 1106209, TL 1106214
PEDROZO, Leidys Marina  TL 1105398, TL 1105408 PICANÇO, Miguel Rossi  TL 1106360
PEIXOTO, Camila Orrico  TL 1105447, TL 1105747 PICCOLO, Ana Claudia  TL 1105321, TL 1105235
PEIXOTO, Daniel Escobar Bueno  TL 1106455 PICON, Paulo Dorneles  TL 1106110
PEIXOTO, Juliana de Castro Naves  TL 1105689 PIEMONTE, Maria Elisa Pimentel  TL 1105051, TL 1105682, TL 1106417
PEIXOTO, Kaliny Oliveira  TL 1105320, TL 1106474, TL 1105256, TL 1106557 PIEMONTE, Maria Elisa P  TL 1106440, TL 1106490, TL 1106503
PEIXOTO, Maria Clara Borges Girão  TL 1106222 PIMENTA, Matheus Resende Costa  TL 1105216
PEIXOTO, Satiko Andrezza Takano  TL 1106241 PIMENTEL, Ana Luiza Utrine  TL 1105897
PEIXOTO, Vitória Maria Torres TL 1105225, TL 1106177, TL 1106376, TL 1106381, PIMENTEL, Angela V  TL 1105948
TL 1106396, TL 1106525,
PIMENTEL, Leonardo Halley Carvalho  TL 1105363, TL 1105373

403
Índice de Autores – XXX Congresso Brasileiro de Neurologia

PIMENTEL, Maria Lucia V  TL 1104814 PITOMBEIRA, Milena Sales  TL 1106461, TL 1106173, TL 1106158
PIMENTEL, Maria Lúcia Vellutini  TL 1105066, TL 1105114 PITTA, Isadora Baldissara da Rocha  TL 1105696
PIMENTEL, Vinicio Manella  TL 1105505 PITTA, Izabela Jardim Rodrigues  TL 1105578
PIMENTEL, Vitoria  TL 1105820 PITTA, Izabela Rodrigues  TL 1105130, TL 1105255, TL 1105278
PINHEIRO, Ana Letícia Vieira de Oliveira  TL 1106308 PITTOCK, Sean J  TL 1105040
PINHEIRO, Carina  TL 1106654, TL 1106655 PITZ, Ana Flávia Machado  TL 1106316, TL 1106489
PINHEIRO, Claudio Joaquim Borba  TL 1105054, TL 1104800 PIVA, Mariana Floriano Luiza TL 1105552, TL 1105565, TL 1105843, TL 1105739
PINHEIRO, Kezia de Souza  TL 1105716 PIVA, Mariana Floriano Luíza  TL 1106343
PINHEIRO, Kézia de Souza  TL 1106455, TL 1105566, TL 1105799 PIZZATTO, Thamilee  TL 1104995, TL 1105169
PINHEIRO, Kézia Souza  TL 1105705 PLAGGERT, Paulo Scatulin Gerritsen  TL 1105441
PINHEIRO, Leon  TL 1104886 POLAVARAPU, Kiran  TL 1104679
PINHEIRO, Marcelo de Almeida  TL 1106376, TL 1106381, TL 1106396 POLIDO, Graziela Jorge  TL 1106092, TL 1106093, TL 1106377, TL 1105206,
PINHEIRO, Maria Clara Apolônio  TL 1106544 TL 1105312, TL 1106542,
TL 1106094
PINHEIRO, Mariana Gonçalves Maciel  TL 1105834
PONTE, Airton Ferreira  TL 1106587, TL 1105142
PINHEIRO, Mariana Soares  TL 1106447, TL 1106388, TL 1106507, TL 1106547
PONTE, Isadora  TL 1105865
PINHEIRO, Maria Suelly Nogueira  TL 1105405, TL 1105593, TL 1106196,
TL 1106010 PONTE, Keven Ferreira  TL 1105142, TL 1105136, TL 1106264

PINHEIRO, Natália da Costa Chiote  TL 1105525 PONTE, Sarah Gurgel  TL 1104844

PINHEIRO, Patricia Gomes  TL 1105438, TL 1105431, TL 1105495, PONTELLO, Maria Clara  TL 1104949
TL 1105443, TL 1105468, PONTES, Letícia Pinheiro  TL 1106608, TL 1106623
TL 1105485, TL 1105736, PONTES, Luana Jessica da Silva  TL 1106563
TL 1105623, TL 1105636
PONTES, Maciel Eduardo de  TL 1106478
PINHEIRO, Patrícia Gomes  TL 1105698, TL 1105719, TL 1105745
PONTES, Patryck Andrew Ribeiro de Melo TL 1106224, TL 1106592, TL 1106238,
PINHEIRO, Samuel Luca Rocha  TL 1105752 TL 1106131
PINHEIRO, Thiago Duque  TL 1105879 PONTES, Pedro Alberto Diogenes Saldanha  TL 1105906
PINHEIRO, Warlisson Fonseca  TL 1105555 PONTES-NETO, Octavio Marques  TL 1105362
PINHO, Patricia  TL 1105089 PONTES-NETO, Octávio Marques  TL 1105065, TL 1105574
PINHO, Ricardo Silva  TL 1106472 PORTELA, Denise Maria Meneses Cury  TL 1105732, TL 1105061, TL 1105744,
PINHO, Ricardo S  TL 1106602 TL 1105861, TL 1105870
PINTO, Elen Beatriz  TL 1105033 PORTELA, Eduardo Jardel  TL 1106124
PINTO, Elen Beatriz  TL 1105033 PORTILHO, Sthéfani Spricigo  TL 1105103
PINTO, Gabrielle Miranda Magalhães  TL 1105937 PORTO, Filipe Brito  TL 1106459
PINTO, Giulia Maria Ximenes Verdi  TL 1105277 PORTO, Louise M  TL 1104775
PINTO, Gustavo Neves  TL 1105085, TL 1105145 PORTO, Nara Fontenele Dias  TL 1104998, TL 1105145
PINTO, Isabela Pires Porto Santarém  TL 1106067, TL 1106148 PORTUGAL, Chiara Gübel  TL 1105082, TL 1106567, TL 1106385, TL 1106286,
PINTO, Lecio Figueira  TL 1106009 TL 1106402

PINTO, Lécio Figueira  TL 1106453 PORTUGAL, Miguel Vieira de Almeida Chiara Gübel  TL 1105082

PINTO, Milton Cabral de Melo  TL 1106588 POTENCIANO, Thales Fernandes  TL 1105849

PINTO, Nayara Queiroz Cardoso  TL 1106226 POTON, André  TL 1104996

PINTO, Paula Oliveira  TL 1106080, TL 1106463 POUZA, Ana Flávia Pincerno  TL 1105804

PINTO, Roberta Arb Saba Rodrigues  TL 1106349, TL 1105647, TL 1105315 POUZA, Ana Flávia Pincerzo  TL 1106231

PINTO, Roberta Caramico  TL 1106122 POZZOBON, Pedro Machry  TL 1105157, TL 1106407, TL 1104943, TL 1105109,
TL 1105110, TL 1105111,
PINTO, Stella Duarte  TL 1105925 TL 1105144, TL 1105488
PINTO, Wladimir Bocca Vieira de Rezende TL 1104864, TL 1104860, PRADO, Catarina Sodre de Castro  TL 1105854, TL 1105710
TL 1104862, TL 1105226,
TL 1105283, TL 1105285, PRADO, Catarina Sodré Castro  TL 1105741
TL 1105633, TL 1104874, PRADO, Catarina Sodré de Castro  TL 1105462
TL 1104940, TL 1105428, PRADO, Gilmar Fernandes do  TL 1106646
TL 1105516, TL 1105536
PRADO, Gilmar Fernandes  TL 1106472, TL 1106316, TL 1106489, TL 1106657
PINTO, Wladimir Bocca V R  TL 1105212
PRADO, Marcelle Sanjuan Ganem  TL 1106516, TL 1106576
PINTO, Wladimir B V R  TL 1105221
PRAXEDES, Gabriel  TL 1106267
PIONES, Deryc Cleyner Bastos  TL 1106181
PRAÇA, Henrique Oliveri Leite  TL 1106121
PIOVESAN, Elcio Juliato  TL 1106205
PRESOTO, Augusto Arrebola  TL 1106172
PIRES, Ane Caroline Chales de Carvalho  TL 1105500
PROCÓPIO, Gustavo Cavalcante  TL 1106574
PIRES, Henrique Guimaraes  TL 1106126
PROENÇA, Bruna Moreira de Souza  TL 1105650
PIRES, Karina Lebeis  TL 1105438, TL 1105431, TL 1105495, TL 1106460,
TL 1105442, TL 1105443, PROENÇA, Bruna Moreira Souza  TL 1105158, TL 1106274
TL 1105468, TL 1105473, PROENÇA, Bruna Moreira  TL 1105713
TL 1105485, TL 1105698, PRUDENTE, Lorena Ohrana Braz  TL 1106478, TL 1105196
TL 1105719, TL 1105736,
TL 1105745, TL 1105623, PRYSTHON, Amanda Gadelha  TL 1106020
TL 1105636 PRZYSIEZNY, Bernardo  TL 1105537
PIROLA, Renann Nunes  TL 1106270 PUGLIANE, Karen  TL 1105587
PIROLA, Renann  TL 1105187 PUPE, Camila Branco  TL 1106380
PITA, Rodrigo Torres Sena  TL 1105702, TL 1105580, TL 1105585, TL 1105590 PUPE, Camila Castelo Branco  TL 1106095, TL 1105869, TL 1105770,
PITARO, Lívia Maria Ribeiro  TL 1104728 TL 1105406, TL 1106097

404
Índice de Autores – XXX Congresso Brasileiro de Neurologia

PUPE, Camila  TL 1105741, TL 1105824, TL 1105879, TL 1106300 RECH, Bruna Constantino  TL 1105571, TL 1106315
PURIFICAÇÃO, Isaac Rêgo  TL 1106485, TL 1106524, TL 1106556 RECH, Matheus Machado  TL 1106049
QEUIROZ, Maria Yvone Carlos Formiga de  TL 1105693 REED, Umbertina Conti  TL 1105048, TL 1105049, TL 1105176, TL 1106411,
QUADROS, Fernanda Silveira de  TL 1106432 TL 1106416, TL 1106424,
TL 1106436, TL 1106445,
QUADROS, Fernanda Silveira  TL 1106080 TL 1106469, TL 1106542,
QUEIROGA, Morgana Feitosa de  TL 1106229, TL 1106570, TL 1106159, TL 1106047, TL 1106400
TL 1106291, TL 1106236 REED, Umbertina C  TL 1104679
QUEIROZ, Andressa Gabriella Duarte de  TL 1105874 REGADAS, Carolina Murad  TL 1106215
QUEIROZ, André Luiz Guimarães  TL 1105657 REGADAS, Marina Murad  TL 1106215
QUEIROZ, Barbara Matos Almeida  TL 1106553, TL 1106616, TL 1106493, REGIS, Railson Cipriano  TL 1105877
TL 1106537, TL 1106473,
TL 1106434, TL 1106499, REGO, Guilhermina  TL 1105009, TL 1105600, TL 1105010
TL 1106452 REILLY, Mary M  TL 1105342, TL 1105247, TL 1105167, TL 1105695, TL 1105714,
QUEIROZ, Barbara Matos de Almeida  TL 1106502 TL 1105837

QUEIROZ, Breno Gabriel Rodrigues  TL 1105881, TL 1105581, TL 1105891 REILLY, Mary  TL 1105808, TL 1105781

QUEIROZ, Camila Crispim  TL 1106555 REIS, Beatriz Castro  TL 1105285, TL 1105536

QUEIROZ, Davi Teixeira Urezêdo  TL 1105758, TL 1105730 REIS, Fabiano  TL 1104731, TL 1105785

QUEIROZ, Davi Teixeira Urzêdo  TL 1105779, TL 1105852, TL 1105849 REIS, Felipe Ibiapina  TL 1105096

QUEIROZ, Dánton Campos de  TL 1106539 REIS, Franklin  TL 1105393, TL 1105783

QUEIROZ, Luciana  TL 1106580 REIS, Lorena Silva dos  TL 1106040

QUEIROZ, Luisa Diogenes  TL 1106222 REIS, Maria Julia Figueiró  TL 1106313

QUEIROZ, Luísa Diógenes  TL 1104712 REIS, Maria Júlia Figueiró  TL 1106601, TL 1106618

QUEIROZ, Maria Eduarda Bezerra Sales  TL 1106538 REIS, Savio Batista dos  TL 1106059

QUEIROZ, Mariana Costa Nascimento  TL 1106479 REIS, Suzana Bleckmann  TL 1105177

QUEIROZ, Maria Yvone Carlos Formiga de  TL 1105810, TL 1105748, TL 1105453 REIS, Victoria Faustino Silva  TL 1106572, TL 1106135, TL 1106605, TL 1106630,
TL 1106524, TL 1106574
QUEIROZ, Rayana Tavares de  TL 1106597, TL 1106458
REIS, Victoria  TL 1106118
QUEIROZ, Rayana Tavares de  TL 1106597, TL 1106458
REIS, Victória Faustino  TL 1106556
QUITERIO, Alex Bertolazzo  TL 1105197, TL 1105154, TL 1105190, TL 1105232,
TL 1105521 REIS, Virgínia Angelica Silveira  TL 1106652

RABELLO, Francisco de Assis Pinto Cabral Júnior  TL 1106095, TL 1106097 REMONTTI, Daniela  TL 1105142, TL 1105272

RABELLO, Nicole Jansen  TL 1105240, TL 1105242 REQUIÃO, Letícia Escorse  TL 1106028, TL 1106040

RABELO, Samia Thabida de Oliveira  TL 1106499 RESENDE, Caroline Meneses TL 1106181, TL 1106651, TL 1106578, TL 1106638

RABELO, Samuel Ramos  TL 1105791, TL 1105848 RESENDE, Elisa de Paula França  TL 1105852, TL 1105562, TL 1106061

RABELO, Samuel  TL 1104712 RESENDE, Elisa de Paula França  TL 1105852, TL 1105562, TL 1106061

RADKTE, Lucas Soares  TL 1105435 RESENDE, Giovana Carvalho de  TL 1105889

RADTKE, Lucas Soares  TL 1105316, TL 1105405, TL 1105593, TL 1106335, RESENDE, Heitor Cherulli  TL 1104728
TL 1105949, TL 1106207 RESENDE, Renata Cristina  TL 1106124, TL 1105849
RAGNINI, Daniela  TL 1105571, TL 1106315 REZENDE, Andre Luiz  TL 1106019
RAMINA, Kristofer  TL 1106296 REZENDE, André Luiz de  TL 1105905, TL 1105168
RAMINA, Ricardo  TL 1105459 REZENDE, Lucas Bruno  TL 1106121, TL 1105366, TL 1105372
RAMOS, Carlos Henrique Ferreira  TL 1105668, TL 1105668, TL 1105530, REZENDE, Thiago J. R.  TL 1104731
TL 1105530 REZENDE, Thiago JR  TL 1105288
RAMOS, Cristiane Cagnoni  TL 1104633 REZENDE, Thiago J R  TL 1105785, TL 1105545
RAMOS, Diogo Lima de Souza  TL 1106145 REZENDE, Thiago Junqueira R.  TL 1106352
RAMOS, Larissa Russo  TL 1104975 REZENDE, Thiago Junqueira Ribeiro de  TL 1105166
RAMOS, Mariana Michiles Santos  TL 1106125, TL 1106431 REZENDE, Thiago Junqueira Ribeiro  TL 1105797
RAMOS, Murilo Sousa  TL 1105519, TL 1105413 REZENDE, Wladimir Bocca Vieira de  TL 1104864, TL 1104860,
RAMOS, Vera Lúcia Nogueira  TL 1106261, TL 1106558 TL 1104862, TL 1105226,
RAMOS, Vera Lúcia Nogueira  TL 1106261, TL 1106558 TL 1105283, TL 1105285,
TL 1105559, TL 1105633,
RAMPELOTI, Breno  TL 1106156 TL 1104874, TL 1104940,
RANGEL, Deborah Moreira  TL 1106450 TL 1105428, TL 1105516,
RANGEL, Déborah Moreira  TL 1106234, TL 1106076 TL 1105560, TL 1105536

RANGEL, Júlio Farias  TL 1106251, TL 1106403 REZIN, Gislaine Tezza  TL 1106491

RANZOLIN, Aline  TL 1105524 REZK, Eduardo Abrão Spinola  TL 1104943, TL 1105109, TL 1105110,
TL 1105111
RAPOSO, Camila Cristina Bastos Silva  TL 1105119
RIBAS, Guilherme Carvalhal  TL 1106106
RAPOSO, Jéssica Vasques  TL 1105570, TL 1105757, TL 1105500, TL 1105607,
TL 1105782, TL 1106168 RIBAS, Michelle Zonkowski  TL 1106191, TL 1106480

RAPOSO, Yan da Silva  TL 1106126, TL 1106121, TL 1106124, TL 1106281, RIBAS, Márcia Cristina Antunes  TL 1105462
TL 1106127 RIBEIRO, André Luiz Pinto Fabricio  TL 1105913
RAPOSO-VEDOVI, Jessica Vasques  TL 1105668 RIBEIRO, Erlane Marques  TL 1106376, TL 1106381, TL 1106396
RAPOSO-VEDOVI, Jessica Vasquez  TL 1105530 RIBEIRO, Espartaco Moraes Lima  TL 1106589
RAPOSO-VEDOVI, Jéssica Vasques  TL 1105570, TL 1105757, TL 1105607, RIBEIRO, Espártaco Moraes Lima  TL 1106587, TL 1105142,
TL 1105782, TL 1106168 TL 1105272, TL 1105907,
RASKIN, Salmo  TL 1105790 TL 1105900, TL 1105136,
TL 1105419, TL 1106264
REAL, Danilo Martins  TL 1105198

405
Índice de Autores – XXX Congresso Brasileiro de Neurologia

RIBEIRO, Giovana Barros E Silva  TL 1105555, TL 1105118, ROCHA, Ismael Lima  TL 1105594
TL 1105635, TL 1105765, ROCHA, Jessica Santos de Souza  TL 1105456
TL 1106237, TL 1106378
ROCHA, Julia Maria Rodruigues da  TL 1104942
RIBEIRO, Giovana Barros E Silva  TL 1105555, TL 1105118,
TL 1105635, TL 1105765, ROCHA, Jéssica Santos Souza  TL 1105112
TL 1106237, TL 1106378 ROCHA, Luciano Chaves  TL 1105456, TL 1105112
RIBEIRO, Helder de Lima  TL 1105756 ROCHA, Margleice Marinho Vieira  TL 1105706
RIBEIRO, Ian da Silva  TL 1106308 ROCHA, Maria Luísa  TL 1105895, TL 1105182
RIBEIRO, Ian Silva  TL 1106105, TL 1106233, TL 1106234, ROCHA, Maria Sheila Guimaraes  TL 1105115
TL 1106244, TL 1106553, ROCHA, Maria Sheila Guimarães  TL 1105293, TL 1105340, TL 1106459,
TL 1106620, TL 1106280, TL 1105344, TL 1106550,
TL 1106453, TL 1106107, TL 1106343, TL 1105843,
TL 1106291, TL 1106236 TL 1106331
RIBEIRO, Laisse Leite  TL 1106121 ROCHA, Maria Sheila  TL 1106247, TL 1106249, TL 1106129, TL 1106248
RIBEIRO, Lara Samanta Barbosa  TL 1105548 ROCHA, Murilo Tomaz  TL 1105155, TL 1105223, TL 1105224
RIBEIRO, Leticia Franceschet  TL 1105610 ROCHA, Natalia Pessoa  TL 1106190
RIBEIRO, Luis Felippe Barros Silva Pereira  TL 1105019 ROCHA, Rafael Cavalcanti Fernandes  TL 1105597, TL 1105609
RIBEIRO, Marcel Leal  TL 1106547 ROCHA, Ray Almeida da Silva  TL 1106404
RIBEIRO, Marlise Castro  TL 1105864 ROCHA, Ray Almeida Silva  TL 1106337
RIBEIRO, Marlise de Castro  TL 1106432 ROCHA, Rubson Soares  TL 1106347, TL 1105379, TL 1106134, TL 1106547
RIBEIRO, Natana Rangel da Silva  TL 1105864 ROCHA, Rubson Soares  TL 1106347, TL 1105379, TL 1106134, TL 1106547
RIBEIRO, Nathália Helena Vieira  TL 1105279 ROCHA, Simão Teixeira  TL 1106511
RIBEIRO, Patrícia Nunes  TL 1106638 ROCHA, Yan Pereira  TL 1106239
RIBEIRO, Paulo de Lima Serrano Roberta Correa  TL 1105633 ROCHA-FILHO, Pedro Augusto Sampaio  TL 1105734
RIBEIRO, Reja Syanne de Aguiar  TL 1105575 RODRIGO, Patrícia  TL 1106175
RIBEIRO, Roberta Corrêa  TL 1105536 RODRIGUES, Ada Virginia Alves  TL 1105742
RIBEIRO, Roberta Lessa  TL 1106170, TL 1106291 RODRIGUES, Alberto Martins Pina  TL 1104809, TL 1105081, TL 1104807,
RIBEIRO, Roberta Lessa  TL 1106170, TL 1106291 TL 1106506
RIBEIRO, Rodrigo Bitencourt  TL 1105270 RODRIGUES, Aline Campos Fontenele  TL 1106376, TL 1106381, TL 1106396
RIBEIRO, Rodrigo Mariano  TL 1105435, TL 1105593, TL 1106335, TL 1106010 RODRIGUES, Aline Rabelo  TL 1105684, TL 1105725, TL 1106214
RIBEIRO, Saulo Ramos  TL 1106287, TL 1106319, TL 1106139, TL 1106307, RODRIGUES, Amanda Tomaz  TL 1105704
TL 1106360 RODRIGUES, Amelia Maia  TL 1105196
RIBEIRO, Saulo Ramos  TL 1106287, TL 1106319, TL 1106139, TL 1106307, RODRIGUES, Ana Clara Guilherme  TL 1106085, TL 1105767, TL 1106370,
TL 1106360 TL 1105417
RIBEIRO, Tatiana Souza  TL 1105033 RODRIGUES, Andre Felipe Lucchi  TL 1105758
RIBEIRO, Tatiana Souza  TL 1105033 RODRIGUES, Andre Filipe Lucchi  TL 1105730
RIBEIRO, Vanessa Asfura Pinto  TL 1106418 RODRIGUES, Andre Filipe Luchi  TL 1105779
RIBEIRO, Vitoria Flexa  TL 1106434 RODRIGUES, André Filipe Lucchi  TL 1105852, TL 1105849
RICARTE, Marilia Brito  TL 1106125 RODRIGUES, Bernardo  TL 1105053, TL 1105627, TL 1105089, TL 1105095
RICCHEZZA, Lucas Leroux de  TL 1105568 RODRIGUES, Bruno Cassis Antunes  TL 1106406
RICCHEZZA, Lucas Leroux  TL 1105081 RODRIGUES, Bruno Castelo Branco  TL 1105101, TL 1106506
RICHEZZA, Lucas Leroux de  TL 1105241 RODRIGUES, Cleonisio Leite  TL 1106537, TL 1106452
RIECK, Mariana  TL 1104685 RODRIGUES, Cleonísio Leite  TL 1106280
RIEDER, Carlos Roberto de Mello  TL 1105617 RODRIGUES, Daniela Laranja  TL 1104957
RIGOTI, Leticia Yabushita  TL 1105397 RODRIGUES, Gabriel Martins  TL 1105062
RIGUEIRAL, Júlia Rodrigues Picazo  TL 1105889 RODRIGUES, João Pedro Cotrim  TL 1106561, TL 1106615
RIOS, Beatriz Pereira  TL 1105379, TL 1105324 RODRIGUES, Julio Cesar  TL 1106157, TL 1106228
RISO, Ivy Liger  TL 1105739 RODRIGUES, Julio Cesar  TL 1106157, TL 1106228
RISSARDO, Jamir Pitton  TL 1105047 RODRIGUES, Júlia Chartouni  TL 1106241
RISSO, Isabela Fonseca  TL 1105421, TL 1105378 RODRIGUES, Klesia Adaynny  TL 1105382, TL 1105104, TL 1105369, TL 1105371
RITA, Valéria Coelho Santa  TL 1106311, TL 1105494, TL 1105607 RODRIGUES, Lucas Bentes  TL 1105638
RIVERA, Beatriz de Moraes  TL 1104807 RODRIGUES, Lívia Carla de Melo  TL 1106531
RIZELIO, Vanessa  TL 1105646, TL 1104942, TL 1105271, TL 1105464 RODRIGUES, Maria Clara Alvarenga  TL 1106142
RIZON, Erica Garbin  TL 1105183 RODRIGUES, Márcia Maria Jardim  TL 1105287
RIZON, Erica  TL 1105820 RODRIGUES, Nathália Pinheiro  TL 1106262, TL 1105318, TL 1106608,
ROBSON, Vital Teixeira  TL 1105578 TL 1106623
ROCHA, Anastacia Guimarães  TL 1104685 RODRIGUES, Paula Raquel do Vale Pascoal  TL 1106172
ROCHA, Antônio José  TL 1106637, TL 1105048 RODRIGUES, Paulo Henrique Rodrigues e  TL 1105898
ROCHA, Danielly Maximino da  TL 1106209 RODRIGUES, Rafael Felipe Silva  TL 1105562
ROCHA, Emanuelle Bianchi da Silva  TL 1105197, TL 1105154, RODRIGUES, Renata Cotrim  TL 1106561, TL 1106615
TL 1105190, TL 1105232, RODRIGUES, Rodrigo Fellipe  TL 1105197, TL 1105190, TL 1105232, TL 1105521
TL 1105151, TL 1105521
RODRIGUES, Rubia  TL 1105950, TL 1106140, TL 1105076, TL 1105648
ROCHA, Felipe Araújo  TL 1106162
RODRIGUES-FILHO, José Anchieta  TL 1105907
ROCHA, Fernando Coronetti Gomes da  TL 1106448
RODRIGUES-RIBEIRO, Lucy  TL 1105672, TL 1106272, TL 1105665
ROCHA, Fernando Coronetti Gomes  TL 1106407, TL 1105109
ROGATTO, Fernanda Botta Tarallo  TL 1105036

406
Índice de Autores – XXX Congresso Brasileiro de Neurologia

ROGERIO, Fabio  TL 1105276 SALGADO, João Lucas Gomes  TL 1104886


ROGÉRIO, Fabio  TL 1105215 SALIS, Guilherme Vedovato Vilela de  TL 1105151
ROGÉRIO, Ricardo Mendes  TL 1105112 SALLES, Cristina  TL 1105053, TL 1105627
ROJAS, Pedro Thiago de Cristo  TL 1105597, TL 1105005, TL 1105892 SALLUM, Fabiana Jallad  TL 1105031
ROJO, Joana Luiza  TL 1105807, TL 1105759 SALMON, Thierry  TL 1104633
ROLIM, Flavia de Paiva Santos  TL 1106632, TL 1106236 SALOMÃO, Daiane  TL 1106295, TL 1106297
ROLIM, Flavia Paiva  TL 1106473 SALVADOR, Gabriel Monteiro  TL 1106074
ROLIM, Flávia de Paiva Santos  TL 1104780, TL 1105914, TL 1106197, SALVADOR, Nayra Roberta Sales  TL 1104833, TL 1105908, TL 1104978
TL 1106216, TL 1106219 SALVI, Cristiane Campello Bresani  TL 1106320, TL 1106559
ROLIM, Karla Maria Carneiro  TL 1106220 SALZEDAS, Hennan Teixeira  TL 1105793
ROMANCINI, Fabiana  TL 1104891 SAMPAIO, Marina Vasconcelos  TL 1106185
ROMANO, Camila Malta  TL 1106451 SAMPAIO, Pedro Henrique Marte Arruda  TL 1105293
ROMANO, Thais Malta  TL 1106451 SAMPAIO, Pedro Henrique Marte de Arruda  TL 1105602, TL 1106550,
ROMUALDO, Gabriela  TL 1106580 TL 1105768, TL 1106084,
ROMUALDO, Isabel Monique Leite  TL 1105046, TL 1104966 TL 1105739, TL 1106270,
TL 1106436, TL 1106255
ROMÃO, Wanderson  TL 1106531
SAMPER, Isabela Corrêa  TL 1105586, TL 1105307
RONCHI, Nathalia Rossoni  TL 1106140
SAMUELI, Pedro Cougo  TL 1105625
RONI, Gabriel Marim  TL 1105705, TL 1105716, TL 1105566, TL 1105990
SANCHES, Jacqueline Dulce Yumi  TL 1105537
ROQUE, Alberto Jorge Castelo Branco  TL 1106245
SANCHES, Ícaro Tavares  TL 1104697
ROSA, Bruna Faria  TL 1106372
SANCHEZ, Hugo  TL 1106440, TL 1106490, TL 1106503
ROSA, Eduardo Antonio  TL 1105192
SANTANA, Alfredo Nicodemos da Cruz  TL 1106075
ROSA, Maria Fernanda Prado  TL 1105601
SANTANA, Ana Rosa  TL 1106188, TL 1105734, TL 1105777, TL 1106423,
ROSA, Thiago de Souza  TL 1106363, TL 1106378, TL 1105863 TL 1105773, TL 1106397,
ROSALEM, Rafael Augusto  TL 1106246 TL 1106444, TL 1106465,
ROSAURO, Jucimara  TL 1105165 TL 1105237, TL 1106548

ROSENSTEIN, Renan Fabri  TL 1106252 SANTANA, Carita Victora Carvalho de  TL 1105655

ROSETTE, Vanessa Moraes  TL 1106058 SANTANA, Carita Victoria Carvalho de  TL 1106272, TL 1105665

ROSO, Raisa do Val  TL 1105555 SANTANA, Carita Victoria Carvalho de  TL 1106272, TL 1105665

ROSSATO, Artur Vestena  TL 1105864 SANTANA, Glauciane Costa  TL 1106062

ROSSETE, vanessa moraes  TL 1106157 SANTANA, Joanna Sousa Fonsênca  TL 1106135

ROSSETTE, vanessa moraes  TL 1106145, TL 1106228, TL 1106395, TL 1106167 SANTANA, João Pedro Matos de  TL 1105777, TL 1106397

ROSSETTE, vanessa moraes  TL 1106145, TL 1106228, TL 1106395, TL 1106167 SANTANA, Laura Helena Esteves Poggianella  TL 1105294, TL 1105807,
TL 1105759
ROSSI, Matheus Andreghetti  TL 1106632
SANTANA, Marcela Araújo de Oliveira  TL 1105850
ROSSI, Matheus Andrighetti  TL 1106359
SANTIAGO, Adriano José Xavier  TL 1106273
ROSSO, Ana Lucia de Zuma  TL 1105921
SANTIAGO, Ana Luisa Carvalho  TL 1105807, TL 1105216, TL 1105759
ROSSO, Laura Motter  TL 1106298, TL 1106301
SANTIAGO, Igor Bessa  TL 1106220, TL 1106488, TL 1106223, TL 1106158
ROSSOR, Alexander M  TL 1105714
SANTIAGO, Nadja Layane Gomes  TL 1105447
ROSÁRIO, Mateus Santana do  TL 1105697, TL 1105746
SANTIAGO, Nadja  TL 1105053, TL 1105747
ROSÁRIO, Matheus Santana do  TL 1105469, TL 1105361
SANTIAGO, Raí dos Santos  TL 1106531
ROUANET, Carolina  TL 1105101, TL 1104809
SANTILLAN, Thiago Ivan Vilchez  TL 1106019
ROVANI, Samira Soligo  TL 1105402
SANTILLÁN, Ivan Vilchez  TL 1105905
ROVANI, Samyra Soligo  TL 1105397, TL 1105403
SANTOS, Adrialdo Jose  TL 1105016
RUEDA, Fernanda  TL 1105479
SANTOS, Adrialdo José  TL 1105121, TL 1105429, TL 1105503, TL 1105628,
RUFINO, Erlan Pércio Lopes  TL 1106583 TL 1106357, TL 1105483
RUFINO, Erlan Pércio  TL 1106617 SANTOS, Alair Augusto Sarmet  TL 1106540
RÊGO, Cláudia Cecília da Silva  TL 1106311 SANTOS, Alan Ferreira dos  TL 1106607
SABA, Roberta Arb  TL 1106349, TL 1105647, TL 1105754, TL 1105315, SANTOS, Amanda Michelucci dos  TL 1105863
TL 1105629
SANTOS, Andre Cleriston Jose dos  TL 1105756, TL 1105805
SABINO, João Vitor  TL 1105635
SANTOS, André Cleriston Jose dos  TL 1105592, TL 1105695
SAFANELLI, Juliana  TL 1105096
SANTOS, André Cleriston José dos  TL 1104755
SAIDELES, Rafael  TL 1106080, TL 1106463
SANTOS, André Cleriston José  TL 1105355
SAITO, Augusto Obuti  TL 1106433
SANTOS, Anselmo de Jesus  TL 1106085, TL 1105767, TL 1106370, TL 1105417
SAKAJIRI, Raissa Kitaguchi  TL 1105097
SANTOS, Antônio Carlos dos  TL 1105362, TL 1106654, TL 1106655
SALAMARGO, Mariana Oliveira  TL 1105842, TL 1106437, TL 1105871,
TL 1105583 SANTOS, Beatriz Marques dos  TL 1105905, TL 1105353, TL 1105647,
TL 1105315, TL 1105168
SALES, Alana Bacelar Limeira  TL 1106191
SANTOS, Beatriz  TL 1105031, TL 1105648
SALES, Amanda Sabino Pinho  TL 1105440
SANTOS, Benevides José Silva  TL 1106220, TL 1106488
SALES, Deborah Santos  TL 1105438, TL 1105431, TL 1106460, TL 1105468,
TL 1105473, TL 1105485, SANTOS, Breno William Correa dos  TL 1105505, TL 1106283
TL 1105698, TL 1105719, SANTOS, Bárbara Loiola  TL 1105338
TL 1105736, TL 1105745, SANTOS, Camilo Vieira  TL 1106605, TL 1106630
TL 1105623, TL 1105636
SANTOS, Carolina Barcha  TL 1106595
SALES, Hebert Falcão  TL 1106384
SANTOS, Carolina de Figueiredo  TL 1105082, TL 1105316
SALES, Milena Pitombeira  TL 1106244
SANTOS, Carolina Figueiredo  TL 1106125, TL 1106196, TL 1106207

407
Índice de Autores – XXX Congresso Brasileiro de Neurologia

SANTOS, Caroline Correa  TL 1105324 SANTOS, Luiz Gustavo Brenneisen  TL 1105421, TL 1105703, TL 1105378,
SANTOS, Caroline Corrêa  TL 1106347 TL 1105078, TL 1105740

SANTOS, Clauham Williams Soares dos  TL 1106398 SANTOS, Luís Daniel Nóbrega  TL 1106238

SANTOS, Clauhan Willams Soares dos  TL 1106317 SANTOS, Maria Eduarda Alencar  TL 1105885, TL 1105471, TL 1105487

SANTOS, Clauhan Williams Soares dos  TL 1106612, TL 1106239, TL 1106119 SANTOS, Mariusi Glasenapp dos  TL 1105087

SANTOS, Daniel Abreu  TL 1105263, TL 1105858 SANTOS, Matheus Corrêa  TL 1106347

SANTOS, Daniel Lucas de Lima Silva  TL 1105438, TL 1105495, SANTOS, Nadson Bruno Serra  TL 1105765, TL 1105630
TL 1105468, TL 1105473, SANTOS, Nínivi Daniely Farias  TL 1105511, TL 1105855, TL 1106141,
TL 1105698, TL 1105719, TL 1106130, TL 1105801,
TL 1105736, TL 1105623 TL 1106143, TL 1106446,
SANTOS, Daniel Lucas de Lima Silva  TL 1105438, TL 1105495, TL 1106624
TL 1105468, TL 1105473, SANTOS, Patrick Emanuell Mesquita Sousa  TL 1105157, TL 1106407,
TL 1105698, TL 1105719, TL 1104943, TL 1105109,
TL 1105736, TL 1105623 TL 1105110, TL 1105111,
SANTOS, Daniel Teixeira dos  TL 1106298, TL 1106301 TL 1105144, TL 1105488

SANTOS, Davi Lopes  TL 1106391, TL 1106526, TL 1105082, TL 1106177, SANTOS, Paula Adriele  TL 1106205
TL 1106622, TL 1106192, SANTOS, Priscila Silva  TL 1104939
TL 1106385, TL 1106286, SANTOS, Raderi Luiz Cardoso dos  TL 1105666, TL 1105686, TL 1105699,
TL 1106402, TL 1106641 TL 1105691, TL 1105641
SANTOS, Diogo F dos  TL 1105714 SANTOS, Raderi Luiz Cardoso  TL 1105264, TL 1105390
SANTOS, Diogo Fernandes dos  TL 1104755, TL 1105586, TL 1105581, SANTOS, Rafael Zieglitz  TL 1106563
TL 1105850, TL 1105601,
TL 1105510, TL 1105307, SANTOS, Rasec Kayan Oliveira  TL 1106492
TL 1105599, TL 1105873 SANTOS, Rebeca Lima de Almeida  TL 1105842, TL 1106437, TL 1105871,
SANTOS, Diogo Fernandes  TL 1105891 TL 1105583
SANTOS, Diogo Haddad  TL 1106533, TL 1106471, TL 1106268, TL 1106282 SANTOS, Renata Alicya Alves dos  TL 1106651
SANTOS, Débora Lopes dos  TL 1105412 SANTOS, Ricardo Felipe dos  TL 1105872
SANTOS, Emanuel Cassou Santos  TL 1106199 SANTOS, Roberto Pereira  TL 1105081, TL 1106506
SANTOS, Felipe Edson Couto dos  TL 1105746 SANTOS, Roberto Queiroz dos  TL 1105169
SANTOS, Fernanda Thaysa Avelino dos  TL 1105148 SANTOS, Rômulo Cerqueira Heckert dos  TL 1105287
SANTOS, Fernanda Thaysa Avelino  TL 1105326, TL 1105475 SANTOS, Rômulo Martins Ferreira  TL 1106492
SANTOS, Gabriele Reis dos  TL 1105599 SANTOS, Sacha Fuchs dos  TL 1105329
SANTOS, Gabriel Magalhães  TL 1105159, TL 1105162 SANTOS, Savana Camilla Lima  TL 1105239
SANTOS, Gabriel Venas  TL 1106417 SANTOS, Sthefany Lorrany Nepomuceno  TL 1106220, TL 1106488
SANTOS, Gecila Aragão  TL 1104939 SANTOS, Tayla Samanta Silva dos  TL 1106267, TL 1106619, TL 1105412,
TL 1105651, TL 1105819,
SANTOS, Geslaine Janaina B.  TL 1106564, TL 1106590 TL 1106421, TL 1106428,
SANTOS, Giovanna Maria Feitoza Barbosa dos  TL 1106549 TL 1106543, TL 1106579,
SANTOS, Gustavo Camargos de Toledo  TL 1106124 TL 1106591, TL 1106611

SANTOS, Gutemberg Augusto Cruz dos  TL 1105479 SANTOS, Tayla Samanta Silva dos  TL 1106267, TL 1106619, TL 1105412,
TL 1105651, TL 1105819,
SANTOS, Gutemberg Augusto Cruz  TL 1105897 TL 1106421, TL 1106428,
SANTOS, Gérsika Bitencourt  TL 1105034 TL 1106543, TL 1106579,
SANTOS, Heitor Caetano dos  TL 1105320, TL 1105256 TL 1106591, TL 1106611

SANTOS, Heitor Caetano  TL 1106474, TL 1106557 SANTOS, Tibério Silva Borges dos  TL 1106379, TL 1105061

SANTOS, Isabel de Oliveira  TL 1105293 SANTOS, Vanessa Gil Humberto dos  TL 1105114

SANTOS, Isabel Oliveira  TL 1106459 SANTOS, Vaneza Mirele Gomes dos  TL 1105692

SANTOS, Isadora Oliveira  TL 1105177 SANTOS, Vito Thayson Damasceno dos  TL 1106616

SANTOS, Israela Souza Brito  TL 1105655, TL 1106272, TL 1105665 SANTOS, Vitória Gabrielle Castilho dos  TL 1105902

SANTOS, Itamar Meireles Andrade  TL 1104943, TL 1105109, TL 1105110, SANTOS, Weybkenedy José Oliveira  TL 1105391
TL 1105111 SANTOS, Yves Glauber Silva  TL 1105797
SANTOS, João Nicoli Ferreira dos  TL 1105540, TL 1105547, TL 1105509 SANTOS-BEZERRA, Gabriel Marinheiro  TL 1106587, TL 1105907
SANTOS, João Vitor Monteiro  TL 1105639 SANTOS-LOBATO, Bruno Lopes  TL 1105410
SANTOS, João Vitor Ribeiro dos  TL 1104995, TL 1105169 SAPORITO, Marcela Tiezzi  TL 1105441
SANTOS, Juliana Costa dos  TL 1104824 SARAIVA, Matheus Gurgel  TL 1105693, TL 1105810, TL 1106439,
SANTOS, Julio Cesar Claudino dos  TL 1105848 TL 1105748, TL 1106633,
TL 1106294, TL 1105453,
SANTOS, Julio  TL 1105847 TL 1106603, TL 1105778
SANTOS, Júlio Cesar dos  TL 1104712 SARAIVA-PEREIRA, Maria Luiza  TL 1104685
SANTOS, Júlio César Claudino dos  TL 1105791, TL 1105872, TL 1106222, SARMENTO, Analuiza Silva Tenório Luna  TL 1106181, TL 1106651
TL 1106152
SARMENTO, Filipe Pereira  TL 1105718, TL 1104981
SANTOS, Júlio  TL 1105865
SARTORI, Cesar Renato  TL 1104831
SANTOS, Karollyne de Castro  TL 1105746
SARTORI, Cesar  TL 1105029
SANTOS, Laura Goedel  TL 1104955
SARTORI, César Renato  TL 1105474
SANTOS, Leila SB  TL 1104775
SARUBI, Giovana de Oliveira  TL 1105783, TL 1105384, TL 1105890
SANTOS, Leila Souza Brito  TL 1105655
SARUWATARI, Thamilyn Yoshizaki  TL 1106042
SANTOS, Leonardo Matos  TL 1105227
SASSE, Thiago Vieira  TL 1105528, TL 1105576
SANTOS, Lucas Silva  TL 1105842, TL 1105871, TL 1105583
SATO, Douglas Kazutoshi  TL 1105183, TL 1106432
SANTOS, Luiz Gustavo Breneissen  TL 1105859

408
Índice de Autores – XXX Congresso Brasileiro de Neurologia

SATO, Henry Koiti  TL 1105625 SEVERO, Rafaela Sousa  TL 1104975, TL 1105323


SAUTE, Jonas Alex Morales  TL 1106372 SEVERO, Reed André Siqueira  TL 1106244
SCARDUA, Lucas Silva  TL 1105630 SHE, Dewei  TL 1105040
SCARPEL, Renata  TL 1105089 SILAGI, Marcela Lima  TL 1106367, TL 1106369, TL 1106517
SCATOLA, Guilherme Egidio Rocha  TL 1106480 SILVA, Ailton de Souza  TL 1106559
SCAVASINE, Valéria Cristina  TL 1106191, TL 1106480 SILVA, Alberto Henrique Torres Trindade da  TL 1105834, TL 1105857
SCHAFASCHECK, Gustavo Silva  TL 1105869, TL 1105710 SILVA, Alex Eduardo da  TL 1105528, TL 1105576
SCHEIBE, Alexandre Henrique  TL 1106480 SILVA, Amanda Karla Alves Gomes  TL 1105252, TL 1105380, TL 1105388
SCHEIDT, Gabriela  TL 1105537 SILVA, Ana Carolina Felipe  TL 1105466
SCHELP, Andreas Batista  TL 1105804, TL 1105905, TL 1106019, SILVA, Ana Cristina Veiga  TL 1105834
TL 1105299, TL 1106349, SILVA, Ana Flávia Souza Freire da Silva  TL 1106388
TL 1105274, TL 1105353,
TL 1105822, TL 1105647, SILVA, Ana Flávia Souza Freire da  TL 1106447, TL 1106388, TL 1106507,
TL 1105754, TL 1105315 TL 1106547
SCHERER, Rodrigo Twardowski  TL 1105026, TL 1105295, TL 1105621, SILVA, Andre Macedo Serafim  TL 1105178, TL 1105206, TL 1106411,
TL 1105195 TL 1106436
SCHETTINI, Daniel Teixeira Martins  TL 1106596, TL 1106613, TL 1106573 SILVA, Andressa Gabrielle da  TL 1105371
SCHILLING, Lucas Porcello  TL 1105542, TL 1105026, TL 1105295, TL 1105621, SILVA, André M. S.  TL 1104679
TL 1105195 SILVA, André Macedo Serafim da  TL 1105768, TL 1105713, TL 1105186
SCHILLING, Lucas Porcello  TL 1105542, TL 1105026, TL 1105295, TL 1105621, SILVA, André Macedo Serafim  TL 1105158, TL 1105213, TL 1105358,
TL 1105195 TL 1106274, TL 1105048,
SCHIMITT, Gabriel S  TL 1105284, TL 1105545 TL 1105049, TL 1105176,
TL 1105650, TL 1106082,
SCHIMITTI, Gabriel S  TL 1105284 TL 1106175, TL 1106189,
SCHINZARI, Priscila Souza  TL 1105235 TL 1106195, TL 1106270,
SCHLINDWEIN-ZANINI, Rachel  TL 1105122, TL 1105124 TL 1106416, TL 1106424,
TL 1106400, TL 1105222,
SCHLOIKA, Larissa Lucas  TL 1105702, TL 1105580, TL 1105585, TL 1105590 TL 1106255
SCHMIDT, Felipe  TL 1105479 SILVA, André Macedo  TL 1106047
SCHMITT, Gabirel  TL 1106408 SILVA, Ariel Lucas Medeiros  TL 1105252, TL 1105380, TL 1105388
SCHMITT, Gabriel S.  TL 1104731 SILVA, Arthur Santos da  TL 1106209
SCHMITT, Gabriel  TL 1104979 SILVA, Beatriz Carneiro Gondim  TL 1105158, TL 1106274, TL 1105950,
SCHNEIDER, Fernando Tonon  TL 1105295, TL 1105621, TL 1105195 TL 1105178, TL 1105650,
SCHUSTER, Matheus Padão  TL 1105542, TL 1105295 TL 1105713

SCHWARZBOLD, Marcelo Liborio  TL 1105122, TL 1105124 SILVA, Bruna Santos  TL 1105131

SCIARINNI, Bruna Bruna  TL 1104937 SILVA, Bruno Eron Almeida da  TL 1105051

SCIARINNI, Bruna Helena  TL 1104938 SILVA, Cacia Caroline Carvalho  TL 1105789

SCOLA, Rosana Hermínia  TL 1106172 SILVA, Caio César Molina  TL 1105263

SCOLARI, Leticia Pereira  TL 1105369 SILVA, Caio Ferreira de Lima  TL 1105882

SCOTT, Stephanie Suzanne de Oliveira  TL 1106656 SILVA, Carla Beatriz Jales da  TL 1104975, TL 1105323

SCOTT, Stheffane Suzane Oliveira  TL 1106076 SILVA, Carlos Antônio de Arroxelas  TL 1105227

SCÁRDUA-SILVA, Lucas  TL 1106345, TL 1106353 SILVA, Caroline Figueiredo da  TL 1106063

SECUNDINO, Catarina  TL 1106267 SILVA, Caíque Martins Mortati  TL 1105595

SENA, Antonio Andrei da Silva  TL 1106525, TL 1106385 SILVA, Daniel Melchiades da  TL 1105090

SENA, Antonio Andrei da Silva  TL 1106525, TL 1106385 SILVA, Danilo Jorge da  TL 1105309

SENA, Antônio Andrei da Silva  TL 1106402 SILVA, David Elison Lima  TL 1106587, TL 1106589, TL 1105907, TL 1105419,
TL 1106264
SENA, Antônio Andrei  TL 1106539, TL 1106192, TL 1106286
SILVA, Debora Rayssa Siqueira  TL 1105252, TL 1105380, TL 1105388
SENA, Daniel Cavalcanti  TL 1106224, TL 1106592, TL 1106238, TL 1106131
SILVA, Deiziani Cristina Valadares  TL 1105818, TL 1105092
SENA, Idna Lara Goes de  TL 1105848, TL 1105872
SILVA, Delson Jose da  TL 1105689
SENA, Lucas Schenatto  TL 1104685
SILVA, Djanino Fernades  TL 1106187
SENEOR, Daniel Delgado  TL 1105285, TL 1105536
SILVA, Djanino Fernandes da  TL 1105524, TL 1106467, TL 1105557
SENNE, Carlos  TL 1106295, TL 1106297
SILVA, Djanino Fernandes da  TL 1105524, TL 1106467, TL 1105557
SERAFIM, Joab Lins  TL 1105252, TL 1105380, TL 1105388
SILVA, Djanino Fernandes  TL 1105102, TL 1105789, TL 1106365, TL 1105764,
SERAFINI, Marina Cunha  TL 1105562 TL 1105815, TL 1106184,
SERAIDARIAN, Marina Buldrini Filogonio  TL 1105370, TL 1105346 TL 1105743, TL 1106210,
SERANO, Paulo de Lima  TL 1105536 TL 1105809, TL 1106418,
TL 1105589, TL 1105613
SERPA, Evelin Duarte  TL 1105412
SILVA, Eduardo Antônio Roquim e  TL 1106121, TL 1106124
SERPA, Marcela de Moares  TL 1105241
SILVA, Eduardo Mariano Carvalho  TL 1105511, TL 1105855, TL 1106141,
SERPA, Marcela de Moraes  TL 1105401, TL 1105568 TL 1106130, TL 1105801,
SERRANO, Paulo de Lima  TL 1105212, TL 1105283, TL 1105285, TL 1105559, TL 1106143, TL 1106446,
TL 1105633, TL 1105428, TL 1106624
TL 1105516, TL 1105560 SILVA, Ellen Dayanne Barros  TL 1106181, TL 1106651, TL 1106578
SERRANO, Paulo Lima  TL 1105221 SILVA, Ellen Dayanne  TL 1106638
SEVERIANO, Maria Izabel Rodrigues  TL 1106564, TL 1106590 SILVA, Elton Spirty Rodrigues  TL 1105743
SEVERINO, Fernanda Gadelha  TL 1106652 SILVA, Erika Christina  TL 1106155
SEVERINO, Isadora Garcia Carneiro Kriunas  TL 1106179, TL 1106161 SILVA, Everton Vieira Lopes  TL 1105070
SEVERINO, Wesley  TL 1105537

409
Índice de Autores – XXX Congresso Brasileiro de Neurologia

SILVA, Ewerton Emmanuel Soares  TL 1106119 SILVA, Luís Eduardo de Medeiros e  TL 1105159, TL 1105162
SILVA, Felipe von Glehn da  TL 1106320 SILVA, Lígia Rufino  TL 1106289
SILVA, Felipe William Dias  TL 1104891 SILVA, Marcos Manoel Sousa  TL 1105465
SILVA, Fernanda Souza Gracílio  TL 1106135, TL 1106630, TL 1106485, SILVA, Marcos Martins da  TL 1105101
TL 1106118, TL 1106524, SILVA, Marcos Martins  TL 1105081
TL 1106556, TL 1106574
SILVA, Marcus Tulius Teixeira da  TL 1106540
SILVA, Filipe Nolasco de Souza e  TL 1106447, TL 1106507, TL 1106547
SILVA, Marcus Tulius Teixeira  TL 1106306
SILVA, Francisca Soraya Lima  TL 1106653
SILVA, Maria Carolina Cardoso da  TL 1106315
SILVA, Francisco Hanlly da  TL 1105594
SILVA, Maria Júnia Lira e  TL 1106317, TL 1106398, TL 1106119, TL 1105631
SILVA, Frank Gregory Cavalcante  TL 1105235
SILVA, Maria Júnia Lira  TL 1106612
SILVA, Gabriella Braga da Cunha  TL 1105370, TL 1105346
SILVA, Mariana Cardoso Costa da  TL 1106515
SILVA, Gabriel Montenegro Ribeiro da  TL 1105689, TL 1105877, TL 1105889,
TL 1105902 SILVA, Maria Rita Carvalho  TL 1106367, TL 1106517, TL 1106495
SILVA, Geovane Gomes  TL 1106188, TL 1105734, TL 1105777, SILVA, Maria Souza  TL 1106474
TL 1106423, TL 1105773, SILVA, Maria Weryca de Souza Belo  TL 1105320, TL 1105256
TL 1106397, TL 1106444, SILVA, Maria Weryca Souza Belo  TL 1106557
TL 1106465, TL 1105237
SILVA, Mariella Reinol da  TL 1106491
SILVA, Gisele Sampaio  TL 1105177
SILVA, Marilia Fontenelle e  TL 1105807
SILVA, Guilherme Diogo  TL 1106246, TL 1105950, TL 1106140, TL 1105817
SILVA, Matheus Alves da  TL 1105615, TL 1105306, TL 1105146, TL 1105793
SILVA, Guilherme Diogo  TL 1106246, TL 1105950, TL 1106140, TL 1105817
SILVA, Miclecio Luiz da  TL 1106181
SILVA, Gustavo Figueiredo da  TL 1106063
SILVA, Milena Pereira Ribeiro da  TL 1105696
SILVA, Gustavo Figueiredo da  TL 1106063
SILVA, Natalia de Oliveira  TL 1105821, TL 1105836, TL 1104970
SILVA, Hiago Antunis  TL 1106281, TL 1106127
SILVA, Natalia Oliveira da  TL 1105133, TL 1106566
SILVA, Igor Barbosa Ferreira da  TL 1106359
SILVA, Nathalia Tomazoni  TL 1106257, TL 1105208
SILVA, Igor Fortunato da  TL 1105394, TL 1105551, TL 1105381, TL 1105270,
TL 1105034 SILVA, Natália de Oliveira  TL 1106487, TL 1105387, TL 1105604, TL 1105399,
TL 1106654, TL 1106655
SILVA, Isabela Louise Caldeira  TL 1105689
SILVA, Natália Oliveira  TL 1105362
SILVA, Iva Taiuan Fialho  TL 1106267
SILVA, Nickolas Souza  TL 1105136
SILVA, Ivã Taiuan Fialho  TL 1106619, TL 1105412, TL 1105651
SILVA, Ornella Aquino da  TL 1105783
SILVA, Izadora Celant Miranda da  TL 1105624
SILVA, Pablo Henrique Costa  TL 1105769
SILVA, Jackson Daniel Sousa  TL 1105639
SILVA, Pablo Henrique da Costa  TL 1105359, TL 1105839,
SILVA, Jasiel da  TL 1105267 TL 1106326, TL 1106500,
SILVA, Jaynara Keylla Moreira da  TL 1106367, TL 1106517, TL 1106495 TL 1105920, TL 1106478
SILVA, Jaynara Keylla Moreira da  TL 1106367, TL 1106517, TL 1106495 SILVA, Paola Marques da  TL 1106249
SILVA, Jhonata Gabriel Moura  TL 1105511, TL 1105855, TL 1106141, SILVA, Paula Baleeiro Rodrigues  TL 1105421, TL 1105378, TL 1105429,
TL 1106130, TL 1105801, TL 1105078, TL 1105859,
TL 1106143, TL 1106446, TL 1105740, TL 1105422
TL 1106624 SILVA, Paula Baleeiro  TL 1105703
SILVA, Joyce Maria Leite e  TL 1105046, TL 1104966 SILVA, Pedro Lorenzo Neves da  TL 1105864
SILVA, João Paste  TL 1105819, TL 1106421, TL 1106579 SILVA, Pedro Victor Castro  TL 1105806
SILVA, João Paulo Yoshio da  TL 1106252 SILVA, Pedro Victor de Castro  TL 1105430, TL 1105001, TL 1105792
SILVA, João Valdêncio  TL 1105272, TL 1105136, TL 1106264 SILVA, Pedro Vitor de Castro  TL 1105297
SILVA, João Victor Cunha  TL 1106143 SILVA, Raisa Cristina Teodoro e  TL 1105807
SILVA, João Victor da Cunha  TL 1105511, TL 1106141, TL 1105801, TL 1106446, SILVA, Rayane Leite da  TL 1106578, TL 1106638
TL 1106624
SILVA, Regina Taís da  TL 1105320, TL 1105256
SILVA, João Vitor Gomes da  TL 1105889
SILVA, Ricardo Augusto Machado  TL 1105440
SILVA, Juan Felipe Galvão da  TL 1106214
SILVA, Ricardo Eberhart Ribeiro da  TL 1105420
SILVA, Kennedy Soares Linhares  TL 1105565, TL 1105843
SILVA, Roberta Bessa Veloso  TL 1105270
SILVA, Lara Guimarães Queiroz  TL 1105450, TL 1105461, TL 1105574,
TL 1106528 SILVA, Rodrigo Alencar e  TL 1106557
SILVA, Laura de Lourdes Cardoso e  TL 1105586, TL 1105510, TL 1105307 SILVA, Simone Cássia Silveira  TL 1104833
SILVA, Leila Camila Santos  TL 1106607 SILVA, Simone de Cássia Silveira  TL 1104832
SILVA, Leila Gabriele Nunes  TL 1105155, TL 1105223, TL 1105224 SILVA, Sonia Maria Cesar de Azevedo  TL 1105738
SILVA, Leonardo Galdino da  TL 1106298, TL 1106301 SILVA, Sônia Maria Azevedo  TL 1106100
SILVA, Leticia Souza Borges da  TL 1105235 SILVA, Sônia Maria Cesar de Azevedo  TL 1105629
SILVA, Ligia Rufino  TL 1105102 SILVA, Sônia Maria César de Azevedo  TL 1105168
SILVA, Luana Karoline Castro TL 1105498, TL 1105508, TL 1105594, TL 1105465 SILVA, Talita Dias  TL 1104937, TL 1104938
SILVA, Lucas Brandao Holanda da  TL 1106549 SILVA, Tatiane Morgana da  TL 1105542, TL 1105621
SILVA, Lucas Ravagnani da  TL 1105133, TL 1105366, TL 1105372 SILVA, Thiago Yoshinaga Tonholo  TL 1105150
SILVA, Lucas Scardua  TL 1106074, TL 1106607 SILVA, Tiago César Costa da  TL 1105913, TL 1106562, TL 1106571
SILVA, Lucas Scárdua  TL 1105724, TL 1106312, TL 1105610, TL 1106212, SILVA, Tulio Francisco de Vasconcelos  TL 1105256
TL 1106594 SILVA, Vanessa Lauanna Lima  TL 1105808, TL 1105342, TL 1105387,
SILVA, Luis M  TL 1105288 TL 1105781, TL 1105399
SILVA, Luiza Ferreira Gomes da  TL 1106156 SILVA, Vinicius Gomes  TL 1106271
SILVA, Luma Rodrigues da  TL 1106598 SILVA, Vitória Pimentel da  TL 1105925
SILVA, Luísa Van der Linden Ferreira da  TL 1106188 SILVA, Viviane Alexandre da  TL 1105854, TL 1105878

410
Índice de Autores – XXX Congresso Brasileiro de Neurologia

SILVA, Viviane Alexandre  TL 1105462, TL 1105710 SOBREIRA-NETO, Manoel Alves  TL 1105951, TL 1105933, TL 1106273,
SILVA, Walisson Grangeiro Bringel  TL 1106245, TL 1106581 TL 1106647, TL 1106649,
TL 1106653, TL 1106217
SILVA, Wallison Granjeiro Bringel  TL 1106125
SOBREIRA-NETO, Manoel  TL 1106606
SILVA, Wellisson Rodrigues  TL 1106651
SOBRINHA, Raquel Minervino de Carvalho  TL 1106597, TL 1106458
SILVA, Wesley Lopes da  TL 1105597, TL 1105626, TL 1105609
SOBRINHA, Raquel Minervino de Carvalho  TL 1106597, TL 1106458
SILVA, Wesley Lopes  TL 1105728, TL 1105535, TL 1105553, TL 1105749
SOLIZ, Yago Eduardo Frezza  TL 1105784
SILVA, William Nicoleti Turazza da  TL 1105601
SOLLA, Davi Fontoura  TL 1104692
SILVA-BATISTA, Carla  TL 1105036, TL 1106440, TL 1106490, TL 1106503
SOLLA, Davi Jorge Fontoura  TL 1106040
SILVA-NUNES, Mônica da  TL 1105227
SOMMER, Rafael Canani  TL 1106432
SILVEIRA, Jacqueline Gomes da  TL 1106126, TL 1105294
SOUSA, Ana Carolina Nascimento de  TL 1105855, TL 1106130, TL 1105801,
SILVEIRA, Jaqueline Gomes da  TL 1105807, TL 1105759 TL 1106143, TL 1106446
SILVEIRA, Juliana Oliveira Freitas  TL 1105047, TL 1105087, TL 1105088, SOUSA, Ana Carolina Nascimento  TL 1106624
TL 1105090
SOUSA, Bruna Cavalcante de  TL 1105696
SILVEIRA, Luana Ribeiro  TL 1105442
SOUSA, Cristian Douglas Dantas de  TL 1105508
SILVEIRA, Rafael Pallos da  TL 1105294, TL 1105807, TL 1105216, TL 1105759
SOUSA, Daniela Cabral  TL 1106081
SILVEIRA, Raissa Domingues de Simoni  TL 1105092
SOUSA, Fernando Lopes de  TL 1105528
SILVEIRA, Rodrigo Queiroz  TL 1105854, TL 1105479
SOUSA, Gabriel Pereira  TL 1106481
SILVEIRA-LUCAS, Elizabeth  TL 1106445
SOUSA, Jonata Ribeiro de  TL 1106317, TL 1106612, TL 1105524,
SILVESTRE, Zenilda Gueiros  TL 1105882, TL 1105899, TL 1106549 TL 1106398, TL 1106119,
SIMABUKURO, Mateus Mistieri  TL 1106246, TL 1106252, TL 1105950, TL 1106467, TL 1106186
TL 1106140 SOUSA, Jorge Luiz Brito de  TL 1106391
SIMÃO, Maria Lucia Habib  TL 1105133 SOUSA, Luiz Felipe Bezerra de  TL 1105511, TL 1105855, TL 1106141,
SIMÕES, Marcus Vinicius  TL 1105509 TL 1106130, TL 1105801,
SIMÕES, Pedro Thiago  TL 1104833, TL 1104832 TL 1106143, TL 1106446,
TL 1106624
SIQUARA, Ana  TL 1105130, TL 1105255, TL 1105278
SOUSA, Nise Alessandra Carvalho  TL 1105535, TL 1105553, TL 1105749
SIQUEIRA, Carla Lenita Coelho  TL 1106375, TL 1106390, TL 1106392
SOUSA, Nise Alessandra de Carvalho  TL 1105597, TL 1105626, TL 1105609
SIQUEIRA, Elisa Tavares Diogo de  TL 1105107, TL 1105244
SOUSA, Paulo RS P de  TL 1104775
SIQUEIRA, Filipe Caetano  TL 1105937
SOUSA, Vanessa Maria de  TL 1105441
SIQUEIRA, Heloise Helena  TL 1105382, TL 1105104, TL 1105369, TL 1105371
SOUSA, Victória Pagani Samora  TL 1105566, TL 1105990
SLAVIERO, Wesley  TL 1105988
SOUSA, Yan Gondim de  TL 1106251, TL 1106403
SMID, Jerusa  TL 1106451
SOUZA, Abner Carnizello  TL 1105876
SOARES, Ana Lara Vieira  TL 1106339
SOUZA, Alex Soares Souza  TL 1105207
SOARES, Beatriz Carvalho  TL 1105696
SOUZA, Alisson Dias Azevedo  TL 1105198
SOARES, Camilla Vanessa Araujo  TL 1106419, TL 1105853
SOUZA, Ana Beatriz Matos de  TL 1105753
SOARES, Camilla Vanessa Araújo  TL 1106562, TL 1105874, TL 1106571,
TL 1106538 SOUZA, Bruno Rafael Antunes de  TL 1105051

SOARES, Cristiano Ribeiro  TL 1106522, TL 1106484, TL 1106595, TL 1106511 SOUZA, Bruno Rodrigues de  TL 1106449

SOARES, Daniella Rocha  TL 1105684, TL 1105725 SOUZA, Clarice Cristina Cunha de  TL 1105498, TL 1105594

SOARES, Danyela Martins Bezerra  TL 1106391, TL 1106526, TL 1106622, SOUZA, Danielle Carolay Guzman  TL 1105401
TL 1106076, TL 1106385, SOUZA, Daniel Sampaio e  TL 1105445, TL 1105638, TL 1105742
TL 1106402, TL 1106641 SOUZA, Daniel Sampaio  TL 1105522
SOARES, Diego Bezerra  TL 1104697 SOUZA, Diego de Oliveira  TL 1106563
SOARES, Douglas Sousa  TL 1106472, TL 1106316, TL 1106489 SOUZA, Eduardo Mesquita de  TL 1105146, TL 1105793
SOARES, Elidivane Martins de Freitas  TL 1106410 SOUZA, Eduardo Mesquita  TL 1105615, TL 1105306
SOARES, Gihana Larissa  TL 1105741 SOUZA, Fernando Henrique Morais de  TL 1105189, TL 1105432
SOARES, Guilherme Silva  TL 1105859 SOUZA, Gabriel Pinheiro Martins de Almeida e  TL 1105226
SOARES, Hiram Fernandes  TL 1105359, TL 1105769, TL 1106326, TL 1105920 SOUZA, Gloria Maria Schitini de  TL 1105101, TL 1106506
SOARES, Izadora Fonseca Zaiden  TL 1105935 SOUZA, Gloria Maria Shtini  TL 1105081
SOARES, Juliana da Silva  TL 1105441 SOUZA, Haniel Bispo de  TL 1105988
SOARES, Liliane Rodrigues  TL 1105469, TL 1105361 SOUZA, Iana Maciel silva  TL 1106605, TL 1106630
SOARES, Lilliane Rodrigues  TL 1105697, TL 1105823, TL 1105746 SOUZA, Iana Maciel silva  TL 1106605, TL 1106630
SOARES, Maria Rita Sousa  TL 1106544 SOUZA, Ian Felipe Barbosa  TL 1106507, TL 1106072
SOARES, Raissa Barreto Vieira  TL 1106142 SOUZA, Isaac Pantaleão  TL 1104943, TL 1105109, TL 1105110, TL 1105111
SOARES, Raíssa Barreto Vieira  TL 1106179, TL 1106161 SOUZA, Isabela Silva  TL 1105472, TL 1105540, TL 1105547, TL 1105509
SOARES, Tito Bastos Siqueira  TL 1106525 SOUZA, Isabel Nery Bernardino de  TL 1105189, TL 1105432
SOARES, Tito Bastos  TL 1106539, TL 1106192 SOUZA, Jamison Menezes de  TL 1104995
SOARES, Vinicius Pinheiro  TL 1105241, TL 1105568 SOUZA, Jano Alves de  TL 1105710
SOARES, YGOR ROBERTO RAMOS GONÇALVES  TL 1105620 SOUZA, Jorge Luiz de Brito de  TL 1105082, TL 1106177, TL 1106525,
SOBREIRA, Claudia Ferreira da Rosa  TL 1105756 TL 1106539, TL 1106567,
SOBREIRA, Cláudia Ferreira da Rosa  TL 1105706 TL 1106622, TL 1106385,
TL 1106402, TL 1106641
SOBREIRA, Emmanuelle Silva Tavares  TL 1106656, TL 1106217
SOUZA, Jorge Luiz de Brito de  TL 1105082, TL 1106177, TL 1106525,
SOBREIRA, Emmanuelle  TL 1106647 TL 1106539, TL 1106567,
SOBREIRA, Manoel Alves Neto  TL 1106604, TL 1106626 TL 1106622, TL 1106385,
TL 1106402, TL 1106641

411
Índice de Autores – XXX Congresso Brasileiro de Neurologia

SOUZA, Jorge Luiz  TL 1106286 SPERA, Raphael Ribeiro  TL 1106241


SOUZA, Jorge Paes Barreto Marcondes de  TL 1106311, TL 1105500 SPERLING, Stephan  TL 1104957
SOUZA, João Renato Figueiredo  TL 1106220, TL 1106488 SPITZ, Clarissa neves  TL 1105578, TL 1105130, TL 1105255, TL 1105287,
SOUZA, Jully Gabriela Silva  TL 1105474 TL 1105278

SOUZA, Larissa Baccoli de  TL 1105299, TL 1105315, TL 1105629, TL 1105168 SPITZ, Clarissa neves  TL 1105578, TL 1105130, TL 1105255, TL 1105287,
TL 1105278
SOUZA, Leonardo Cruz de  TL 1106132, TL 1106204, TL 1105562,
TL 1106259, TL 1106364, SPITZ, Mariana  TL 1104995, TL 1104996, TL 1105169
TL 1106061, TL 1106190 STAVALE, João Norberto  TL 1105503
SOUZA, Leonardo Cruz de  TL 1106132, TL 1106204, TL 1105562, STECLAN, Chelin Auswaldt  TL 1105347, TL 1105116, TL 1105123, TL 1105134,
TL 1106259, TL 1106364, TL 1105165, TL 1105329
TL 1106061, TL 1106190 STECLAN, Chelin  TL 1105349
SOUZA, Lidiane Alves de Macedo  TL 1105774 STEFANI, Rodrigo Rosa de  TL 1105026
SOUZA, Lohana Guimarães  TL 1105519, TL 1105563, TL 1105413 STEFANO, Luiz Henrique Soares Santos  TL 1105694
SOUZA, Lucas Assis Santos de  TL 1105076 STEGLICH, Matheus Souza  TL 1105062
SOUZA, Lucas Assis Santos  TL 1105688 STEINER, Beatriz  TL 1106293
SOUZA, Lílian Karine Machado  TL 1105252, TL 1105380, TL 1105388 STELLA, Isabela de Almeida  TL 1105274, TL 1105754, TL 1105629
SOUZA, Marcellus Henrique Loiola Ponte de  TL 1105780 STEPHAN, Eduardo P  TL 1105602
SOUZA, Marcelo Alves de  TL 1105240, TL 1105242 STOLIAR, Gabriel Abrahão  TL 1106191
SOUZA, Marcio Nattan Portes  TL 1106139 STORTE, Guilherme Rocha  TL 1106395
SOUZA, Marcus Vinicius de  TL 1105253 STRAUCH, João Gabriel  TL 1105531
SOUZA, Maria Eduarda Iglesias Berardo de  TL 1106592, TL 1106238, STRELOW, Matheus  TL 1106144
TL 1106131
STUDART-NETO, Adalberto  TL 1104692
SOUZA, Maria Eduarda Iglesias Berardo Pessoa de  TL 1106224
STÁBILLE, Isadora Lyrio  TL 1105528, TL 1105576
SOUZA, Maria Valeska Berardo Pessoa de  TL 1106224, TL 1106592,
TL 1106238, TL 1106131 SUSEMIHL, Maria Alice Araujo de Moraes Mello  TL 1106009
SOUZA, Moysés Loiola Ponte de  TL 1106103 SUWA, Samuel Masao  TL 1105988
SOUZA, Murilo Santos de  TL 1106085, TL 1105417, TL 1106028 SUZART, Gabriel Souza  TL 1106267, TL 1106619, TL 1105651, TL 1106421,
TL 1106428, TL 1106579,
SOUZA, Paulo Victor Machado  TL 1105252 TL 1106591
SOUZA, Paulo Victor Sggobi de  TL 1105516, TL 1105560 SÁ, Fabiane Elpídio de  TL 1105937
SOUZA, Paulo Victor Sgobbi de  TL 1104864, TL 1104860, TL 1104862, SÁ, Renata Silva  TL 1105589
TL 1105226, TL 1105285,
TL 1104874, TL 1104940, SÁ, Renata Silva  TL 1105589
TL 1105428, TL 1105491, SÜFFERT, Soraya Camargo Ito  TL 1104957
TL 1105504, TL 1105536 TACLA, Rafaella do Rosario  TL 1105296
SOUZA, Paulo Victor Sgobbi  TL 1105212, TL 1105221, TL 1105283, TL 1105559, TACLA, Rafaella do Rosário  TL 1106302
TL 1105633
TAKAMURA, Thais  TL 1105624
SOUZA, Raissa Gontijo Cabral de  TL 1105706
TAKAMURA, Thaís  TL 1105646, TL 1105625, TL 1105619, TL 1105464,
SOUZA, Raphaell  TL 1105571 TL 1105459, TL 1105466
SOUZA, Rayanne da Silva  TL 1105438, TL 1105431, TL 1105443, TL 1105468, TALITA, Conte A  TL 1106277
TL 1105485, TL 1105698,
TL 1105719, TL 1105736, TAMANINI, João Vitor Gerdulli  TL 1105118, TL 1106237
TL 1105745 TAMY, Mariana Soares de Freitas  TL 1105462, TL 1105869, TL 1105710
SOUZA, Rayanne da Silva  TL 1105438, TL 1105431, TL 1105443, TL 1105468, TAMY, Mariana Soares Freitas  TL 1105897, TL 1105741, TL 1106300
TL 1105485, TL 1105698, TANAKA, Kátia  TL 1105036
TL 1105719, TL 1105736,
TL 1105745 TANAMATI, Karen Yumie  TL 1104860
SOUZA, Regina Nery Bernardino de  TL 1105189, TL 1105432 TARDELLI, Erica  TL 1105036
SOUZA, Roberta Monteiro de  TL 1105241, TL 1105401, TL 1105568 TARDIM, Caio Faria  TL 1106506
SOUZA, Rodrigo Becco de  TL 1105159, TL 1105162, TL 1106581 TARDIN, Caio Faria  TL 1104809, TL 1105081, TL 1104807
SOUZA, Silas Paulo Lima de  TL 1106267, TL 1105651, TL 1105819, TL 1106421, TASCH, João Felipe  TL 1106331
TL 1106579, TL 1106591, TASCH, João F  TL 1106247, TL 1106129, TL 1106248
TL 1106611 TATSCH, João Fellipe Santos  TL 1106343
SOUZA, Silas Paulo Lima de  TL 1106267, TL 1105651, TL 1105819, TL 1106421, TATSCH, Nayara Karina Ferreira Pereira  TL 1105245
TL 1106579, TL 1106591,
TL 1106611 TAUFNER, André Fonseca  TL 1106214
SOUZA, Silas Paulo Lima de  TL 1106267, TL 1105651, TL 1105819, TL 1106421, TAVARES, Beatriz Rihs Matos  TL 1105563, TL 1105413
TL 1106579, TL 1106591, TAVARES, Ciro Vespasiano Coutinho  TL 1105799
TL 1106611 TAVARES, Gabriela Arruda Navarro  TL 1105704
SOUZA, Victor Mascarenhas de Andrade  TL 1105767 TAVARES, Maria Luísa Marcão  TL 1105270
SOUZA, Victória Tizeli  TL 1105988 TAVARES, Rafael Mattos  TL 1106121, TL 1106124
SOUZA, Victória Tizelli  TL 1106144 TAVARES-JÚNIOR, Jose Wagner Leonel  TL 1106626
SOUZA, Vitor Santos de  TL 1105562 TAVARES-JÚNIOR, José Wagner Leonel  TL 1105934, TL 1105951, TL 1105933
SOUZA, Welliton  TL 1105276 TEDESCHI, Helder  TL 1105215
SOUZA, Wendell Silveira de  TL 1105445, TL 1105742, TL 1105522, TL 1105816 TEDESCO, Walton Luiz Del  TL 1106283
SOUZA-LIMA, Carlos Frederico Leite  TL 1105922 TEIVE, Helio Afonso Ghizoni  TL 1105507, TL 1106279, TL 1105185, TL 1106277,
SOUZA-TALARICO, Juliana Nery  TL 1105056 TL 1106278, TL 1106475,
SOÁRES, André César  TL 1106617 TL 1105471
SPECIALI, José Geraldo  TL 1104949 TEIVE, Hélio Afonso G.  TL 1106590

412
Índice de Autores – XXX Congresso Brasileiro de Neurologia

TEIVE, Hélio Afonso Ghizoni  TL 1105790, TL 1106199, TL 1106205, TL 1106527 TOSO, Fabio Fieni 
TEIVE, Hélio Afonso G  TL 1106564 TL 1105001, TL 1105297,
TL 1105792, TL 1105806,
TEIXEIRA, Antônio Lúcio  TL 1106061, TL 1106190 TL 1105430
TEIXEIRA, Bernardo Corrêa de Almeida  TL 1106296 TOTO, Lelia Lage  TL 1104772
TEIXEIRA, Caroliny Trevisan  TL 1104942, TL 1105271 TRAVASSOS, Fernando Tenório  TL 1105019, TL 1106545
TEIXEIRA, Davi Vargas Freitas TL 1105804, TL 1105299, TL 1106349, TREGNAGO, Aline Caldart  TL 1106053
TL 1105274, TL 1105353, TL 1105822,
TL 1105647, TL 1105315 TREHOFF, Igor Vieira  TL 1105115

TEIXEIRA, Elaine Calumby  TL 1105293, TL 1105340 TRELHA, Rejane Sambrana  TL 1105575

TEIXEIRA, Hennan Salzedas  TL 1105306, TL 1105146 TRENCH, Janayna  TL 1105089, TL 1105095

TEIXEIRA, Igor Lima  TL 1105144, TL 1105488 TRENTIN, Sheila  TL 1105408

TEIXEIRA, Mateus Alves  TL 1105763 TRINDADE, Andresa Aline Silva  TL 1104831

TEIXEIRA, Matheus Acioly Muniz  TL 1104707 TRIVELLATO, Stella de Angelis  TL 1104886

TEIXEIRA, Mauricio Silva  TL 1105378, TL 1105078, TL 1105859 TROIAN, Amanda Loureiro de Toledo  TL 1105299, TL 1105274, TL 1105353,
TL 1105420, TL 1105647
TEIXEIRA, Milene Ataíde  TL 1105757
TROIAN, Amanda Loureiro Toledo  TL 1105738
TEIXEIRA, Thais Bonfim  TL 1105689
TROIJO, Heloisa Ferraz  TL 1106329, TL 1106206
TEIXEIRA, Victoria Coelho  TL 1105177
TROMBETTA, Tainã Paulo Zanata  TL 1106316, TL 1106489
TEIXEIRA, Vitor Gil Gonçalves  TL 1105848
TRUZZI, Giselle de Martin  TL 1106309, TL 1106618
TEIXEIRA, Wagner Afonso  TL 1105702, TL 1105580, TL 1105585, TL 1105590,
TL 1106500, TL 1105920 TRUZZI, Giselle  TL 1106328, TL 1106313

TEIXEIRA, Wanderson Rebello  TL 1106360 TRZESNIAK, Clarissa Ferreira  TL 1104831

TELLES, Juliana Rhein  TL 1104937, TL 1104938 TRZESNIAK, Clarissa Maria Ferreira  TL 1105474, TL 1105029

TEMPONE, Tiago Abreu  TL 1106039, TL 1106025, TL 1106213, TL 1106176, TRÉS, Amandha Espavier  TL 1106587, TL 1105900, TL 1105136
TL 1106193 TUBERO, Luciana de Azevedo  TL 1105197
TENFEN, Leonardo  TL 1106491 TUFIK, Sergio  TL 1106309, TL 1106313
TEODORO, Wender Rodrigues  TL 1105881, TL 1105581, TL 1105891 TUMA, Raphael de Luca e  TL 1106241
TERRA, Dayane Menezes  TL 1105270 TUMAS, Vitor  TL 1105070, TL 1105723, TL 1105948, TL 1106647, TL 1105410
TERRIM, Sara  TL 1105950, TL 1105817 TUMAS, Vítor  TL 1106190
TERTULINO, Franklin Freitas  TL 1105906 TURÍBIO, Pedro Igor Figueiredo  TL 1105294
TERUYA, Alexandre  TL 1106576 TWARDOWSCHY, Carlos Alexandre  TL 1105119
TESSARO, Camila Lorenzini  TL 1106441 TÁVORA, Daniel Gurgel Fernandes  TL 1106233, TL 1106173
TEXEIRA, Letícia Fontenele  TL 1105704 TÉLIZ, Marco Andrée Morel  TL 1106121
TEÓFILO, Melina Nunes  TL 1106262 TÖPF, Ana  TL 1104679
THOMPSON, Rachel  TL 1104679 UCHIDA, Paula Tomiko  TL 1105864
THOMÉ, Bibiana  TL 1105398, TL 1105408 AGUZZOLI, Cristiano Schaffer  TL 1105295
THÉ, George André Pereira  TL 1105594, TL 1105465 URBANO, João Cláudio da Costa  TL 1105377
TIETBÖHL, Lucas Michelon  TL 1104955 VALE, Ana Rafaela Soares  TL 1106479, TL 1106135, TL 1106134, TL 1106118,
TINONE, Gisela  TL 1105336 TL 1106524, TL 1106556, TL 1106574

TINÓS, Maria Fernanda Grael  TL 1106209 VALE, Sancha Helena de Lima  TL 1106062

TOLEDO, Amanda Loureiro de TL 1105299, TL 1105274, VALE, Thiago Cardoso  TL 1105253, TL 1106554, TL 1106585, TL 1106596,
TL 1105353, TL 1105420, TL 1106613, TL 1106573
TL 1105647, TL 1105168 VALENTE, Andre Lopes  TL 1105016, TL 1105718, TL 1106635
TOLENTINO, Mateus Pradebon TL 1105552, TL 1105565, VALENTE, André Lopes  TL 1105121
TL 1105843, TL 1105739 VALENTE, Kette Dualibi Ramos  TL 1105122, TL 1105124
TOLOI, Marcella Canato TL 1105804, TL 1105822, VALENTE, Maria Clara Oliveira  TL 1105658
TL 1105754, TL 1105629
VALENTIM, Ricardo Alexsandro de Medeiros  TL 1106263, TL 1106384,
TOMASELLI, Pedro Jose TL 1104755, TL 1105592, TL 1105805, TL 1105588, TL 1106426
TL 1104731, TL 1105808, TL 1105342, TL 1106404,
TL 1105247, TL 1105540, TL 1105781, TL 1105867,
TL 1105277, TL 1105314, TL 1105717, TL 1105239, VALENÇA, Guilherme Teixeira  TL 1106574
TL 1106408, TL 1105167, TL 1105695, TL 1105714, VALENÇA, Marcelo Moraes  TL 1105834
TL 1105837, TL 1105355, TL 1105509, TL 1104731,
TL 1105808, TL 1105342, TL 1106404, TL 1105247, VALENÇA, Marcelo Morais  TL 1105019
TL 1105540, TL 1105781, TL 1105867, TL 1105277, VALERIANO, Katrine Freitas  TL 1106472
TL 1105314, TL 1105717, TL 1105239, TL 1106408, Valeska Julio Forza
TL 1105167, TL 1105695, TL 1105714, TL 1105837,
TL 1105355, TL 1105509, TL 1105547 VALLER, Lenise  TL 1105630
TONHON, Maria Laura Caetano  TL 1105073, TL 1105072 VALLINOTO, Izaura Maria Vieira Cayres  TL 1105054, TL 1104800
TONIOLO, Juliana Naback  TL 1106487, TL 1105362, TL 1105387, TL 1106599 VARELA, Daniel Lima  TL 1105571, TL 1106315
TORQUATO, Mariana da Cruz  TL 1105097 VARELLA, Fabio Jean  TL 1105407
TORRES, Danielle. Mesquita  TL 1105945, TL 1106048 VARGAS, Maria Eduarda Mercado  TL 1105087
TORRES, Gabriel Saboia de Araújo  TL 1105377, TL 1105076 VASCONCELLOS, Laís Fortes Hidd  TL 1105548, TL 1105653
TORRES, Iara Amorim  TL 1106331 VASCONCELLOS, Luiz Felipe Rocha  TL 1105241, TL 1105401, TL 1105568
TORRES, Paula Cely da Silva  TL 1105469, TL 1105361, TL 1105697, TL 1105823, VASCONCELOS, Ana Claudia Bellini  TL 1104831
TL 1105746 VASCONCELOS, Arthur Felipe Barbosa  TL 1105810, TL 1106439, TL 1106633,
TORREÃO, Jorge Andion  TL 1105655 TL 1106294, TL 1105256,
TL 1105453, TL 1106603
TOSCANO, Camila de Araújo  TL 1106597, TL 1106458
VASCONCELOS, Artur Felipe Barbosa  TL 1105778

413
Índice de Autores – XXX Congresso Brasileiro de Neurologia

VASCONCELOS, Claudia Cristina Ferreira  TL 1105442, TL 1105712, TL 1105698, VIEIRA, Ana Júlia Trierweiler  TL 1106156
TL 1105736 VIEIRA, Bianca Maranhão  TL 1105259
VASCONCELOS, Cláudia Cristina Ferreira  TL 1105719, TL 1105745, TL 1105623, VIEIRA, Bruna Queiróz  TL 1106554, TL 1106585, TL 1106596, TL 1106613,
TL 1105636 TL 1106573
VASCONCELOS, Elzeleide Souza de  TL 1106119 VIEIRA, Bruno Ludivig  TL 1105104
VASCONCELOS, Elzeleide Souza  TL 1106187, TL 1105764, TL 1105815, VIEIRA, Bruno Ludvig  TL 1105369
TL 1106184
VIEIRA, Caroline Darold  TL 1106487, TL 1106337
VASCONCELOS, Gessica Almeida  TL 1105469
VIEIRA, Gabriel de Deus  TL 1105395
VASCONCELOS, Igor Andrade  TL 1105505
VIEIRA, Guilherme Machado  TL 1105716, TL 1105566, TL 1105990, TL 1105799
VASCONCELOS, Karla Rafaele Silva  TL 1106105, TL 1106162,
TL 1106233, TL 1106620, VIEIRA, Isabella Reis  TL 1105672, TL 1106272
TL 1106218, TL 1106280, VIEIRA, Lucas Goldim Briand  TL 1105592
TL 1106107, TL 1106090, VIEIRA, Lucas Gondim Briand  TL 1105808, TL 1106233, TL 1105342,
TL 1106359, TL 1106429, TL 1105798, TL 1106599
TL 1106450, TL 1106466
VIEIRA, Lucas Luiz  TL 1106320
VASCONCELOS, Maria Alice Silva  TL 1105732, TL 1105061, TL 1105744
VIEIRA, Manoel  TL 1105784
VASCONCELOS, Mariana Carvalho  TL 1106064
VIEIRA, Maria Eduarda de Sousa  TL 1105639
VASCONCELOS, Paulo Prado  TL 1105270
VIEIRA, Pedro Robson Sousa  TL 1105780
VASCONCELOS, Rodrigo Mesquita de  TL 1105498
VIEIRA, Vera Lúcia Ferreira  TL 1105705, TL 1105990
VASCONCELOS, Sophia Costa  TL 1105316, TL 1105593, TL 1106196,
TL 1106207 VILANOVA, Marcos Vinícius de Souza  TL 1106492, TL 1106523
VASCONCELOS, Sâmara Pinto  TL 1106605, TL 1106630 VILAR, Debora Beserra  TL 1105723
VASCONCELOS, Tamires Cristina Martins de  TL 1105447 VILELA, Gabriel Rosa  TL 1105065, TL 1105338
VASCONCELOS, Tamires  TL 1105053, TL 1105747 VILLANOVA, Bianca  TL 1105240, TL 1105242
VASCONCELOS, Tatheane Couto de  TL 1105693, TL 1106064, TL 1105453, VILLAR, Jordana  TL 1104891
TL 1106065 VILLARIM, Luiza Alves Monteiro Torreao  TL 1106633
VASCONCELOS, Thaís de Maria Frota  TL 1106103 VILLARIM, Luiza Alves Monteiro Torreão  TL 1106439, TL 1105748, TL 1106294,
VASSOLER, Maria Eduarda Messias  TL 1106619, TL 1106428, TL 1106579, TL 1105453, TL 1105778
TL 1106591, TL 1106611 VILLARIM, Luíza Alves Monteiro Torreão  TL 1105810, TL 1106603
VASSOLER, Maria Eduarda  TL 1106267 VILLELA, Cesar  TL 1106139
VAZQUEZ, Fernando Degani  TL 1105405, TL 1105593, TL 1106196, TL 1106010 VINCENZI, Otávio Costa  TL 1105574
VEGA, Márcio  TL 1106295, TL 1106297 VITAL, Robson Teixeira  TL 1105287
VEIGA, Marielza Fernandez  TL 1106111 VITAL, Robson  TL 1105130, TL 1105255, TL 1105278
VELAZQUEZ, Milagros Leopoldina Clavijo  TL 1105227 VITORINO, João Augusto Nunes  TL 1105586, TL 1105850, TL 1105510
VELEZ, Yasmine Portela Velez Portela  TL 1105301 VITORINO, Luciano Magalhaes  TL 1105474
VELOSO, Renan Camarço do Lago  TL 1105639 VIVAS, Thiago Barbosa  TL 1105645, TL 1105658, TL 1105678, TL 1105449
VENTURA, Cledson Gonçalves  TL 1105647 WALUSINSKI, Olivier  TL 1106475, TL 1106527
VENTURA, Laís Maria Gomes de Brito  TL 1105767, TL 1105417 WANABE, Nathalia  TL 1105378
VENTURI, Alexandre  TL 1106471 WANDALL, Guilherme  TL 1105349, TL 1105537
VERAS, Danilo Issa Mitozo  TL 1105445, TL 1105742, TL 1105522, TL 1105892 WANDERLEY, Bianca Gomes  TL 1105635
VERAS, Samuel Ranieri Oliveira  TL 1106105, TL 1106620, TL 1106431 WANZILEU, Ingrid Demosthenes  TL 1105626, TL 1105609,
VERAS, Samuel Ranieri  TL 1106244, TL 1106245, TL 1106553 TL 1105728, TL 1105535,
TL 1105553, TL 1105749
VERDE, Ana Silvia Sobreira Lima TL 1106162, TL 1106229,
TL 1106570, TL 1106620, WASEM, Matheus Pedro  TL 1105624, TL 1105625
TL 1106218, TL 1106107, WATANABE, Nathalia  TL 1105421, TL 1105703, TL 1105078, TL 1105859,
TL 1106090, TL 1106159, TL 1105740
TL 1106450, TL 1106466, WAYSZCEYK, Sheila  TL 1105349, TL 1105537, TL 1105527, TL 1105533
TL 1106308
WEBER, Eduarda Kotlinsky  TL 1105542, TL 1105026, TL 1105621
VERDE, Ana Sílvia Sobreira Lima  TL 1106280
WEINSHENKER, Brian G  TL 1105040
VERDE, Lucas Antonissen Lima  TL 1105419, TL 1106264
WELKER, Carolina Flores  TL 1105702, TL 1105580, TL 1105585, TL 1105590
VERDI, Giulia Maria Ximenes  TL 1106487, TL 1105362, TL 1105277, TL 1105717
WERLE, Taís Michele  TL 1105820, TL 1105925
VERSIANI, Isadora  TL 1105462, TL 1105869, TL 1105741, TL 1105710,
TL 1105879, TL 1106300 WERNECK, Lineu César  TL 1106172
VIANA, Eloisa Alves  TL 1105155, TL 1105223, TL 1105224 WERTHEIMER, Guilherme Soares Oliveira  TL 1105785
VIANA, Leda Maria Cavalcante  TL 1106222 WESCHENFELDER, Cesar Augusto  TL 1106298, TL 1106301, TL 1106372
VIANA, Lorena da Silva  TL 1105511, TL 1105855, TL 1106141, TL 1106130, WESTIN, Larissa Kozow  TL 1105406
TL 1105801, TL 1106446 WHITAKER, Maria Eduarda Dall’Oglio  TL 1105751
VIANA, Lorena Silva  TL 1106143, TL 1106624 WIECKIEWICZ, Mieszko  TL 1105627
VIANA, Natália Araújo Nascimento  TL 1106631 WILLRICH, Louise Bruns  TL 1105684, TL 1105725
VIANA, Patrick Silva  TL 1105818 WINGERCHUK, Dean M  TL 1105040
VIANA, Ramon Távora  TL 1105498, TL 1105508, TL 1105594, TL 1105465 WITTLICH, Eric Aguiar  TL 1105672, TL 1106272, TL 1105665
VIANEZ, Talisia Nascimento  TL 1105816 WOLLMANN, Giulia Murillo  TL 1106063, TL 1106156
VIANEZ, Talísia Nascimento  TL 1105597, TL 1105609, TL 1105749 WOLMER, Paulo Schneider  TL 1106363
VICTOR HUGO CASTRO SÁ2, TL 1105294 XAVIER, Ana Claudia Galvão  TL 1106369
VIDAL, Mariana Alemeida  TL 1105630 XAVIER, Arthur  TL 1105824
VIDIGAL, Daniela Name Chaul  TL 1105601 XAVIER, Fernando  TL 1105407
VIDIGAL, Giovanna Paula  TL 1104937, TL 1104938 XAVIER, Gabriel Meira  TL 1105842, TL 1105871, TL 1105583

414
Índice de Autores – XXX Congresso Brasileiro de Neurologia

XAVIER, Iara Proença  TL 1105551 TL 1105768, TL 1106084, TL 1105048, TL 1105049,


XAVIER, Luciana Ferreira  TL 1106185 TL 1105176, TL 1105178, TL 1105206, TL 1105312,
TL 1105650, TL 1105713, TL 1106082, TL 1106175,
XIMENES, Abraão Wagner Pessoa  TL 1106583 TL 1106189, TL 1106195, TL 1106231, TL 1106270,
XIMENES, Luciana Barberino Rocha  TL 1106547 TL 1106411, TL 1106416, TL 1106424, TL 1106436,
XIMENES, Marcelo Tognato  TL 1106406 TL 1106445, TL 1106469, TL 1106542, TL 1106551,
TL 1106600, TL 1105186, TL 1106047, TL 1106400,
YAMAGUCHI, Laís Ciribelli  TL 1106596, TL 1106613, TL 1106573 TL 1105222, TL 1106255, TL 1106094
YANKELEVICH, Daniel Natalio Gauss  TL 1105121, TL 1105718 ZANOVELLO, Ana Beatriz Gonçalves  TL 1105756, TL 1106599
YASSUDA, Clarissa  TL 1105276 ZARATINI, Douglas Marques  TL 1105207, TL 1105906
YASSUDA, Mônica Sanches  TL 1106259 ZEIGELBOIM, Bianca Simone  TL 1106564, TL 1106590
YASUDA, Clarissa Lin  TL 1105555, TL 1105724, TL 1106312, TL 1105610, ZENATTI, Gabriel Angelo Garute  TL 1105477, TL 1105751, TL 1105787,
TL 1106212, TL 1106345, TL 1106594, TL 1106074, TL 1105302, TL 1105305,
TL 1106353, TL 1106607 TL 1104977, TL 1105776
YASUDA, Clarissa S  TL 1105215 ZENGO, Lucas Victoy Guimarães  TL 1105477, TL 1105751, TL 1105787,
YOSHIDA, Daniela  TL 1104816 TL 1105302, TL 1105305,
ZABETIAN, Cyrus P  TL 1105410 TL 1104977, TL 1105776

ZAIDAN, Paloma Peter Travassos  TL 1105613 ZETOLA, Viviane de Hiroki Flumignan  TL 1105192, TL 1106318

ZAIDEN, Júlia Suzana  TL 1105177 ZIMMER, Eduardo  TL 1106144

ZAMBON, Amélia Limongi  TL 1105321 ZIRPOLI, Bianca Berardo Pessoa  TL 1106224, TL 1106592, TL 1106238,
TL 1106131
ZAMBON, Antonio Alberto  TL 1105176, TL 1105178
ZOTIN, Maria Clara Zanon  TL 1105065, TL 1105362
ZAMBON, Antônio Alberto  TL 1106231
ZUARDI, Antônio W  TL 1105948
ZAMBON, Atonio Alberto  TL 1104679
ZUCHNER, Stephan  TL 1105837
ZAMORA, Paulo Henrique Fabiano  TL 1104955
ZUCHNER, Stephen  TL 1105695
ZAMPOLLI, Tainá Montini  TL 1105189, TL 1105432
ZUMBANO, Giulia Matiusso  TL 1105441
ZANCANARO, Rodrigo Bianchi  TL 1105531
ZUNIGA, Rubén David dos Reis  TL 1105935
ZANETTE, Fernando Netto  TL 1105194, TL 1105642
ZUZA, Stelly Vieira  TL 1106555
ZANETTE, Fernando  TL 1105409, TL 1105395
ZWANG, Temistocles Carvalho  TL 1106369
ZANETTI, Luisa  TL 1104891
ZÉTOLA, Viviane de HIROKI FLUMIGNAN  TL 1106191, TL 1106480, TL 1105365
ZANINI, Marco Antonio  TL 1104886
ZÉTOLA, Viviane Flumignan  TL 1105267
ZANIRATI, Gabriele  TL 1105407
ZÉTOLA, Viviane Hiroki Flumignan  TL 1105096
ZANOTELI, Edmar TL 1105158, TL 1105213, TL 1105306, TL 1105358,
TL 1106092, TL 1106093, TL 1106274, TL 1106377,

415
Arquivos de Volume

Neuro-psiqu

Demências no Brasil: aumento da carga no pe


Estimativas do Estudo Carga Global de

A depressão enquanto um dos principais d


anormalidades do sono em pacientes

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BRASILEIRA Material destinado exclusivamente à
DE NEUROLOGIA

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