Reversible basal ganglia signal changes associated with vigabatrin treatment in infants with epilepsy

Abstract

We describe four children with an apparently unique form of easily treatable early onset epilepsy. All had an unremarkable family history, pregnancy and delivery. One child presented with neonatal seizures. None of them had neonatal jaundice. They presented with developmental impairment, cortical visual agnosia or squint, and clusters of epileptic spasms [3] beginning in infancy. Electroencephalogram showed features of modified (atypical) hypsarrhythmia. Neuroimaging in infancy showed unilateral or bilateral signal changes in the globus pallidus. All infants were treated with high dose vigabatrin (VGB). The seizure disorder responded well to treatment and VGB was discontinued. Repeat magnetic resonance imaging brain scan showed complete resolution of the signal changes in the globus pallidi. All remained seizure free but continued to show severe intellectual disability. We suggest that VGB treatment was associated with "reversible" signal changes in the globus pallidi in these children.

Keywords

Vigabatrin - epilepsy - basal ganglia - MRI