Dyke-Davidoff-Masson Syndrome: A Rare Cause of Acquired Cerebral Hemiatrophy.

Abstract

Dyke–Davidoff–Masson syndrome is a rare disease of childhood which is clinically characterized by hemiparesis, refractory seizures, facial asymmetry, and mental retardation. The classical radiological findings are cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. Seizure refractory to medical management warrants surgical intervention with excellent outcome. Here, we are reporting two such cases who presented late and diagnosis was made on the basis of magnetic resonance imaging brain features. Both of our children responded to oral anticonvulsant and are on regular follow-up.