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A Wobbly Child

5/8/2015 Morning Report


Laura Williams, MD MPH, PGY3

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.

Development: WNL per parents


Meds: occasional OTC meds for seasonal allergies, none
recently; NKDA; Imms UTD

Fam Hx: Mom with PFO, had stroke during previous


pregnancy 2/2 PFO. MGM with thrombosis while on OCP.
Mom and MGM with multiple miscarriages.

Soc Hx: Lives in Utah with parents, older brother. No


tobacco exposure. No daycare. No pets. Recent camping.

Exam
T 36.5, HR 118, BP 165/46, RR 26, SpO2 96% RA.
Weight 55%ile, Length 75%ile, OFC 90%ile

GEN: fussy, sitting in Moms lap, consolable, but not


cooperative with exam

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.

Motor: muscle tone and bulk nml. Normal strength demonstrated


when resisting exam.

Sensation: intact to light touch throughout.


Reflexes: 1+ and symmetric in upper and lower extremities. Toes
down-going. No clonus.

Coordination: Notably abnormal with truncal titubation (tremor) and


appendicular ataxia. Dyssynergy on reaching for objects.

Gait: Ataxic, wide based gait. Cannot remain upright without support.
https://www.youtube.com/watch?v=Dox3_ox8C2U

DDx of acute ataxia


Neuro
Acute cerebellar ataxia
BPPV
Acute labyrinthitis
Basilar migraine
Seizure
Guillan Barre
Acute demyelinating encephalomyelitis
Anticonvulsant toxicity

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

DDx of chronic ataxia


Congenital anomaly of posterior fossa
Dandy-Walker
Chiari
Encephalocoele
Cerebellar aplasia/dysplasia/hypoplasia

Hereditary ataxia
Ataxia-telangiectasia
Friedreich ataxia

Degenerative CNS disease


Spinocerebellar ataxia
Niemann-Pick disease

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

Approach to acute ataxia


Acute ataxia generally due to acute infection,
post-infectious inflammatory process, toxin,
tumor, or trauma
80% of pts eval for ataxia and subsequently
admitted were d/cd home with dx of acute
cerebellar ataxia, toxic ingestion, or Guillan-Barre

Approach to acute ataxia


Life-threatening
Tumor
4560% of childhood brain tumors arise from brainstem or
posterior fossa

Slowly progressive ataxia, sxs of increased ICP


Intra-cranial hemorrhage trauma v. vascular malformation
Stroke
Sickle cell
Hypercoagulable state (as in SLE)

Infection
Cerebellar abscess often from contiguous spread
Brainstem encephalitis CN abnormalities, AMS, seizure
ADEM

Approach to acute ataxia


Acute cerebellar ataxia
Most common; accounts for 3050% of childhood ataxia; ages 25 yo
Frequently post-infectious
Varicella involved in over 25% of cases
Can also occur after vaccines

Symptoms other than ataxia can include nystagmus (50%), slurred


speech, vomiting, headache
Do NOT have fever, meningismus, seizure
Onset within hours to 1-2 days

Work-up: tox screen


Brain imaging only if asymmetric or focal exam, AMS, atypical disease
course, s/s of increased ICP, potential trauma

Prognosis: usually resolves without sequelae in 2-3 weeks


If sxs not improving in several weeks, should consider alternate diagnosis

Approach to acute ataxia


Common conditions
Guillan-Barre
Predominantly affects motor nerves, but up to 15% may develop
sensory ataxia

Miller-Fisher variant triad of ataxia, areflexia, ophthalmoplegia

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?

HD 5-6 urinary retention improved, considering transfer to


inpatient rehab

HD 6, opsoclonus noted on exam


https://www.youtube.com/watch?v=UCiAz8YA0iY

Opsoclonus Myoclonus Ataxia

Opsoclonus Myoclonus Ataxia


Rare: 0.18 cases/million ppl/year in UK
Generally affects girls 1.52 yo

Autoimmune
50% of kids with OMA have neuroblastoma
2% of kids with neuroblastoma develop OMA

Clinical diagnosis; no paraneoplastic biomarkers


Must evaluate for neuroblastoma
Chest, abdomen, pelvic MRI
Urine VMA/HVA
MIBG scan if MRI is unrevealing

Opsoclonus Myoclonus Ataxia


Treatment
Removal of neuroblastoma doesnt improve symptoms
No controlled studies
Immunologic treatment
Steroids, ACTH, plasma exchange, IVIG, immuran, cyclophosphamide, cellcept

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.

UpToDate. Acute cerebellar ataxia in children.


UpToDate. Opsoclonus myoclonus ataxia.