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Neuro-psiquiatriA
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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)
Apoio:
Editors-in-Chief Luís dos Ramos Machado (São Paulo, Brazil)
Ayrton Roberto Massaro (São Paulo, Brazil)
Hélio Afonso Ghizoni Teive (Curitiba, Brazil) Founding Editor (in memoriam)
Paulo Caramelli (Belo Horizonte, Brazil) Oswaldo Lange
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)
Ana Carolina Coan (Campinas, Brazil) Marcondes Cavalcante França Jr. (Campinas, Brazil)
Alexandra Pruffer Queiroz Campos Araújo (Rio de Janeiro, Brazil) Wilson Marques Jr. (Ribeirão Preto, Brazil)
Juliana Gurgel-Giannetti (Belo Horizonte, Brazil)
Neurogenetics
Clinical Neurophysiology
Iscia Teresinha Lopes Cendes (Campinas, Brazil)
Carlos Otto Heise (São Paulo, Brazil)
Sarah Teixeira Camargos (Belo Horizonte, Brazil)
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
Sonia Maria Dozzi Brucki (São Paulo, Brazil) Maria Fernanda Mendes (São Paulo, Brazil)
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)
Neuropathology
General Neurology
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)
Fernando Kok (São Paulo, Brazil) Oscar Gershanik (Buenos Aires, Argentina)
Francisco Cardoso (Belo Horizonte, Brazil) Osvaldo José Moreira do Nascimento (Rio de Janeiro, Brazil)
Giancarlo Comi (Milan, Italy) Osvaldo Massaiti Takayanagui (Ribeirão Preto, Brazil)
Gilmar Fernandes do Prado (São Paulo, Brazil) Pedro Chaná-Cuevas (Santiago, Chile)
Henrique Ballalai Ferraz (São Paulo, Brazil) Raimundo Pereira da Silva Neto (Teresina, Brazil)
Hugh J. Willison (Glasgow, United Kingdom) Regina Maria Papais-Alvarenga (Rio de Janeiro, Brazil)
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
de Propriedade Industrial (97414) and 1º Ofício de Títulos e Documentos de São Paulo, Brasil. Its owner and publisher is the Academia
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the Ministério da Fazenda (CNPJ 43.185.230/0001-85) and in the Secretaria de Finanças do Município de São Paulo (CCM 9.129.811-3).
It is the Official Journal of the Academia Brasileira de Neurologia / (Brazilian Academy of Neurology) since 1970. It is published monthly
since 2012, with absolute regularity over these 70 years since its foundation in 1943.
The announcer is entirely responsible for the advertisements included in this issue. The authors are fully responsible for the opinions and
concepts expressed in the manuscripts published in this issue, which do not necessarily reflect the opinion of the editors.
DIRETORIA EXECUTIVA ABN
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
ÍNDICE DE AUTORES
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
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
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
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
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
Ataxias
14
XXX Congresso Brasileiro de Neurologia
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
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
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
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
Cefaleia
19
XXX Congresso Brasileiro de Neurologia
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
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).
21
XXX Congresso Brasileiro de Neurologia
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
22
XXX Congresso Brasileiro de Neurologia
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
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.
24
XXX Congresso Brasileiro de Neurologia
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
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.
26
XXX Congresso Brasileiro de Neurologia
Doença Cerebrovascular
Doença Cerebrovascular
27
XXX Congresso Brasileiro de Neurologia
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
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.
29
XXX Congresso Brasileiro de Neurologia
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
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
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
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.
33
XXX Congresso Brasileiro de Neurologia
Doença Cerebrovascular
34
XXX Congresso Brasileiro de Neurologia
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
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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Doença Cerebrovascular
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XXX Congresso Brasileiro de Neurologia
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.
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XXX Congresso Brasileiro de Neurologia
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
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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
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XXX Congresso Brasileiro de Neurologia
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.
43
XXX Congresso Brasileiro de Neurologia
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
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
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.
Doença Cerebrovascular
46
XXX Congresso Brasileiro de Neurologia
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
48
XXX Congresso Brasileiro de Neurologia
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
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
Doenças Neuromusculares
51
XXX Congresso Brasileiro de Neurologia
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
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
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
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
56
XXX Congresso Brasileiro de Neurologia
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
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.
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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.
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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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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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XXX Congresso Brasileiro de Neurologia
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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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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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.
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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.
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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
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
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
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
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
Epilepsia
77
XXX Congresso Brasileiro de Neurologia
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
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
80
XXX Congresso Brasileiro de Neurologia
Miscelânea
81
XXX Congresso Brasileiro de Neurologia
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.
Miscelânea
Miscelânea
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XXX Congresso Brasileiro de Neurologia
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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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.
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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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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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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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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.
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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.
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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.
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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.
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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.
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XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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.
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XXX Congresso Brasileiro de Neurologia
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
Neurofisiologia Clínica
106
XXX Congresso Brasileiro de Neurologia
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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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
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.
Neurogenética
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XXX Congresso Brasileiro de Neurologia
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.
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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.
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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.
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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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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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Neurogenética
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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.
Neurogenética
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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.
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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
Neurogenética
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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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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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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
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
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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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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.
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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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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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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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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
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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
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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.
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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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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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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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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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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.
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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.
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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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XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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
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
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XXX Congresso Brasileiro de Neurologia
178
XXX Congresso Brasileiro de Neurologia
Neurologia Infantil
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XXX Congresso Brasileiro de Neurologia
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.
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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
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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
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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.
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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.
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XXX Congresso Brasileiro de Neurologia
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
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XXX Congresso Brasileiro de Neurologia
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
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190
XXX Congresso Brasileiro de Neurologia
Neuropatias Periféricas
191
XXX Congresso Brasileiro de Neurologia
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.
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XXX Congresso Brasileiro de Neurologia
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>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.
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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.
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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.
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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
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
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-
Transtornos do Movimento
NMDAr encephalitis.
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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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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
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
Transtornos do Movimento
are important, in order to avoid the automatic diagnosis
Transtornos do Movimento
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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XXX Congresso Brasileiro de Neurologia
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.
Transtornos do Movimento
Transtornos do Movimento
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XXX Congresso Brasileiro de Neurologia
Transtornos do Movimento
208
XXX Congresso Brasileiro de Neurologia
Transtornos do Movimento
209
XXX Congresso Brasileiro de Neurologia
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
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
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XXX Congresso Brasileiro de Neurologia
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
Ataxias
214
XXX Congresso Brasileiro de Neurologia
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
215
XXX Congresso Brasileiro de Neurologia
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
216
XXX Congresso Brasileiro de Neurologia
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
217
XXX Congresso Brasileiro de Neurologia
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
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
“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
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
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.
Doença Cerebrovascular
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XXX Congresso Brasileiro de Neurologia
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.
Doença Cerebrovascular
223
XXX Congresso Brasileiro de Neurologia
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.
Doença Cerebrovascular
224
XXX Congresso Brasileiro de Neurologia
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.
Doença Cerebrovascular
225
XXX Congresso Brasileiro de Neurologia
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.
Doença Cerebrovascular
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XXX Congresso Brasileiro de Neurologia
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.
Doença Cerebrovascular
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XXX Congresso Brasileiro de Neurologia
Doença Cerebrovascular
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XXX Congresso Brasileiro de Neurologia
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.
Doença Cerebrovascular
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XXX Congresso Brasileiro de Neurologia
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
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XXX Congresso Brasileiro de Neurologia
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
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XXX Congresso Brasileiro de Neurologia
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
Doença Cerebrovascular
233
XXX Congresso Brasileiro de Neurologia
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.
Doença Cerebrovascular
234
XXX Congresso Brasileiro de Neurologia
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.
Doença Cerebrovascular
235
XXX Congresso Brasileiro de Neurologia
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
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
Doença Cerebrovascular
237
XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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
Doença Cerebrovascular
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XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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.
Doença Cerebrovascular
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XXX Congresso Brasileiro de Neurologia
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
243
XXX Congresso Brasileiro de Neurologia
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.
244
XXX Congresso Brasileiro de Neurologia
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.
245
XXX Congresso Brasileiro de Neurologia
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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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.
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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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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.
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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.
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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.
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XXX Congresso Brasileiro de Neurologia
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
Doenças Neuromusculares
Doenças Neuromusculares
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XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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XXX Congresso Brasileiro de Neurologia
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
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XXX Congresso Brasileiro de Neurologia
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.
Doenças Neuromusculares
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XXX Congresso Brasileiro de Neurologia
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
Dor
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XXX Congresso Brasileiro de Neurologia
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.
Educação Médica
263
XXX Congresso Brasileiro de Neurologia
Educação Médica
264
XXX Congresso Brasileiro de Neurologia
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.
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XXX Congresso Brasileiro de Neurologia
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.
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XXX Congresso Brasileiro de Neurologia
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
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XXX Congresso Brasileiro de Neurologia
Epilepsia
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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.
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XXX Congresso Brasileiro de Neurologia
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
Epilepsia
Epilepsia
271
XXX Congresso Brasileiro de Neurologia
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
Epilepsia Epilepsia
272
XXX Congresso Brasileiro de Neurologia
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
273
XXX Congresso Brasileiro de Neurologia
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.
274
XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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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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.
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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.
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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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XXX Congresso Brasileiro de Neurologia
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.
Neuroepidemiologia
Neuroepidemiologia Neuroepidemiologia
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XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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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Neurofisiologia Clínica
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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.
Neurogenética
Neurogenética
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Neurogenética
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Neurogenética Neurogenética
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XXX Congresso Brasileiro de Neurologia
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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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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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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XXX Congresso Brasileiro de Neurologia
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.
Neuroimunologia
300
XXX Congresso Brasileiro de Neurologia
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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301
XXX Congresso Brasileiro de Neurologia
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.
Neuroimunologia
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XXX Congresso Brasileiro de Neurologia
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.
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XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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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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
Neuroinfecção
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XXX Congresso Brasileiro de Neurologia
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XXX Congresso Brasileiro de Neurologia
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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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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.
Neuroinfecção Neuroinfecção
Neuroinfecção
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XXX Congresso Brasileiro de Neurologia
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.
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XXX Congresso Brasileiro de Neurologia
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
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XXX Congresso Brasileiro de Neurologia
Neuroinfecção
Neuroinfecção
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XXX Congresso Brasileiro de Neurologia
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.
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XXX Congresso Brasileiro de Neurologia
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
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.
320
XXX Congresso Brasileiro de Neurologia
321
XXX Congresso Brasileiro de Neurologia
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.
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XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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.
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XXX Congresso Brasileiro de Neurologia
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XXX Congresso Brasileiro de Neurologia
327
XXX Congresso Brasileiro de Neurologia
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.
328
XXX Congresso Brasileiro de Neurologia
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.
329
XXX Congresso Brasileiro de Neurologia
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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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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XXX Congresso Brasileiro de Neurologia
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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Neurologia Infantil
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XXX Congresso Brasileiro de Neurologia
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
Neurologia Infantil
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Neurologia Infantil
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XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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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XXX Congresso Brasileiro de Neurologia
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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Neurologia Intervencionista
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XXX Congresso Brasileiro de Neurologia
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
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
343
XXX Congresso Brasileiro de Neurologia
Neuropatias Periféricas
344
XXX Congresso Brasileiro de Neurologia
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
345
XXX Congresso Brasileiro de Neurologia
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.
346
XXX Congresso Brasileiro de Neurologia
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.
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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.
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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.
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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.
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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
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XXX Congresso Brasileiro de Neurologia
Neurorreabilitação
355
XXX Congresso Brasileiro de Neurologia
Neurossonologia
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XXX Congresso Brasileiro de Neurologia
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.
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XXX Congresso Brasileiro de Neurologia
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
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XXX Congresso Brasileiro de Neurologia
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
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XXX Congresso Brasileiro de Neurologia
Sono
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XXX Congresso Brasileiro de Neurologia
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.
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361
XXX Congresso Brasileiro de Neurologia
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
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
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
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
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
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.
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367
XXX Congresso Brasileiro de Neurologia
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.
Transtornos do Movimento
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368
XXX Congresso Brasileiro de Neurologia
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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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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XXX Congresso Brasileiro de Neurologia
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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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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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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Traumatismo cranioencefálico
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ÍNDICE DE AUTORES
Índice de Autores – XXX Congresso Brasileiro de Neurologia
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
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
AGOURAKIS, Demetrios Chiuratto TL 1104937, TL 1104938 ALMEIDA, Igor Melo de TL 1104862, TL 1104940
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, 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
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
ANTUNES, Luiza Silva TL 1106347 ARAÚJO, Pedro Lucas Grangeiro Batista Sá TL 1105435
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
ARALDI, Bianca Barbosa TL 1105104, TL 1105369, TL 1105371 ARNAUD, Frederico Carlos Sousa TL 1105704
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, 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
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, Marina Nolasco Manhaes Gomes TL 1105401 BENESI, Ricardo Canuto TL 1106306
380
Índice de Autores – XXX Congresso Brasileiro de Neurologia
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
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
385
Índice de Autores – XXX Congresso Brasileiro de Neurologia
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
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
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
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
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, 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
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, 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, Hélio Rodrigues TL 1105076 GRIZA, Rafaela Luiza Altheia TL 1105477, TL 1105751, TL 1105302, TL 1104977
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
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
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
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
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
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
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, 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
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 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, 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, 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, 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, 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, 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
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
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
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
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 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
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
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, 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, 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
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
ZAIDAN, Paloma Peter Travassos TL 1105613 ZETOLA, Viviane de Hiroki Flumignan TL 1105192, TL 1106318
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
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