Abstracts

Holoprosencephaly is usually associated with a diffuse disruption of neuronal proliferation, migration and cortical organization. Epilepsy surgery is rarely performed. This report evaluates the outcome of hemispherectomy in an infant with holoprosencephaly microsigns.
A 15-months old girl presented with global development delay and seizures which started almost immediately after birth. Seizures were described as short cessation of activity followed by blank staring spells, followed by multifocal jerking of arms, legs and body. She was treated unsuccessfully with phenobarbital, gabapentin, levetiracetam and lorazepam. Her exam revealed plagiocephaly, right hemiatrophy and right hemiparesis. Teething and development were delayed. Neuroimaging studies revealed a left hemiphere hypoplasia associated with enlargement of the left ventricle, left periventricular heterotopia and abnormal gyral patterns on the left. Prolonged video-EEG study supported that all epileptic events were emanating from the left hemisphere.
Left hemispherectomy resulted in a complete seizure control. Her first tooth, a single central incisor, appeared at 20 months.
This observation illustrates the need to consider epilepsy surgery in patients with refractory partial seizures unresponsive to medical intervention even when holoprosencephaly microsigns are present.