Abstract Details

Title A rare pediatric case of hemichorea-hemiballismus secondary to vein of Galen thrombosis

Topic Child Neurology and Developmental Neurology

Presentation(s) P7 - Poster Session 7 (8:00 AM-9:00 AM)

Poster/Presentation
Number 6-001

Objective To report a case of a 19 month old girl with vein of Galen thrombosis and bilateral thalamic hemorrhages who developed subacute hemichorea–hemiballismus.

Background Chorea is a hyperkinetic movement disorder characterized by irregular and random movements with flowing quality. Chorea generally does not have good localizing or lateralizing value except for hemichorea. Hemichorea is usually indicative of a structural pathology in the contralateral hemisphere.

Design/Methods Case Report

Results 19 month old girl with unrepaired vein of Galen malformation diagnosed on prenatal ultrasound presented with signs of increased intracranial pressure (ICP), including encephalopathy, hypertension, bradycardia and irregular breathing. CT head showed bilateral thalamic hemorrhages with intraventricular hemorrhage and obstructive hydrocephalus. MR venogram revealed thrombosis of vein of Galen. External ventriculostomy catheter was placed and she remained intubated and sedated in the ICU for 2 weeks. After extubation, she was noted to have left hemibody choreiform and ballistic movements. She was treated with scheduled lorazepam with mild improvement.

Conclusions Acute/subacute chorea is most likely related to acquired causes, although genetic choreas can have a subacute course. In children, the most common causes of genetic and acquired chorea are benign hereditary chorea and Sydenham chorea respectively. Benign hereditary chorea is typically characterized by a more chronic course and generalized choreiform movements. Sydenham chorea, on the other hand, often presents as hemichorea. Infectious or autoimmune encephalitides and non-ketotic hyperglycemia are well known causes of pediatric hemichorea. Strokes, being uncommon in pediatric population, are less likely to be the cause of hemichorea in children. Our case presents a rare instance of a child with bilateral thalamic hemorrhages and increased ICP due to vein of Galen malformation and resultant thrombosis, who developed hemichorea-hemiballismus within 2 weeks of the initial presentation. This case highlights the importance of head imaging in cases of pediatric hemichorea to rule out acute vascular causes.

Authors/Disclosures
Gaurav Chenji, MD PRESENTER	Dr. Chenji has nothing to disclose.
Hemani Ticku, MD	Dr. Ticku has nothing to disclose.
Ankita Prasad, DO (University hospitals)	Dr. Prasad has nothing to disclose.
Neel Fotedar, MD (University Hospitals Cleveland Medical Center)	Dr. Fotedar has received research support from NINDS.

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