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.
Key-words:
Calvarium thickening - cerebral hemiatrophy - Dyke–Davidoff–Masson syndrome - intellectual
disability - seizures
