Pearls & Oy-sters: Acute Obstructive Hydrocephalus Co-Occurrence With Vigabatrin-Associated MRI Changes in an Infant With LIS1-Related Classical Lissencephaly.

We present a case of a 4-month-old infant with classical type I lissencephaly treated with vigabatrin for hypsarrhythmia. The patient subsequently developed hypotonia and respiratory failure. Imaging revealed vigabatrin-associated brain abnormalities on MRI (VABAM), with acute obstructive hydrocephalus requiring emergent extraventricular drainage. VABAM involve a predictable pattern of restricted diffusion, are generally clinically silent, and are reversible on cessation of the medication. This case presents a rare, life-threatening complication in association with VABAM. In this report, we review the pathophysiology of this phenomenon and the potential influence of lissencephaly.