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HPI
23 mo o/w healthy male
Vomiting x1 day, dec PO, nml UOP
Shaking, clinging to Mom x2 days
Tremors with movements started two days ago
while camping with family
No abnormal movements when he is still
Frustrated when trying to move
Refuses to walk, even shaky when crawling
History contd
PMH: term, weighed 7 lbs, 9 oz. Delivery at U of U without
complication. No major or chronic illnesses. No surgeries.
Exam
T 36.5, HR 118, BP 165/46, RR 26, SpO2 96% RA.
Weight 55%ile, Length 75%ile, OFC 90%ile
HEENT: WNL
NECK: unsteadiness of head when not supported, no LAD
CV: WNL
RESP: WNL
ABD: WNL
SKIN: WNL
Neuro Exam
MS: fussy but consolable, tired but appropriate
CN: EOMI. PERRL. Visual fields seem full to confrontation. Face
symmetric. No nystagmus. Tongue midline.
Gait: Ataxic, wide based gait. Cannot remain upright without support.
https://www.youtube.com/watch?v=Dox3_ox8C2U
Heme
Cerebellar stroke
Intracranial hemorrhage
Onc
Brain tumor
Neuroblastoma
ID
Meningitis/encephalitis
Cerebellar abscess
Tick paralysis
Other
Trauma
Drugs of abuse/EtOH toxicity
Lead toxicity
Conversion
Hereditary ataxia
Ataxia-telangiectasia
Friedreich ataxia
Multiple Sclerosis
Work-up
Labs from ED:
CBC: wbc 5.7 (21N, 63L), hgb 14.4, hct 42.5, plt 433
CMP: WNL
VRP neg
CRP <0.5, ESR 5
Urine tox: +benzos (given versed in ED for CT)
Serum tox: neg
CT brain: normal
CT angio: normal
MRI brain: normal
Infection
Cerebellar abscess often from contiguous spread
Brainstem encephalitis CN abnormalities, AMS, seizure
ADEM
Labyrinthitis
Hearing loss, vomiting, vertigo
Can be difficult to distinguish from acute cerebellar ataxia in a toddler
Toxic exposure
Responsible for up to 30% of acute ataxia in kids
Generally with AMS as well
Migraine syndromes
Generally episodic, but can be difficult to dx at first presentation
Trauma
Hospital course
Working diagnosis of acute cerebellar ataxia
Continued admission for poor PO, PT/OT
HD 2, developed urinary retention that resolved by HD 3-4,
unclear etiology, behavioral?
Autoimmune
50% of kids with OMA have neuroblastoma
2% of kids with neuroblastoma develop OMA
Prognosis
Guarded motor symptoms improve or resolve in 60% of cases with initial
treatment, but can relapse
6080% of patients have some residual behavioral abnormalities or
psychomotor slowing
Our patient
IVIG x3 days, ACTH QOD, weekly rituximab, monthly IVIG followed by
dexamethasone
Repeat imaging has been negative for neuroblastoma
Significant regression after diagnosis, regaining milestones, but about 1 year
behind, slight dysarthria, myoclonus when falling asleep/waking up,
significant behavioral outbursts when he doesnt get his way
References
UpToDate. Approach to the child with acute
ataxia.