Dyke-Davidoff-Masson syndrome - A dainty spectrum with a diligent diagnosis

Dyke-Davidoff-Masson syndrome is a rare condition of unknown frequency resulting from brain injury, especially in early life, due to a multitude of causes. We present the case of a 2-year-old, developmentally normal female who presented with one episode of unprovoked generalized tonic-clonic seizures. This child had a history of complex febrile seizures at 1.5 years of age and was started on levetiracetam (20 mg/kg/day). During the current evaluation, a magnetic resonance imaging (MRI) brain showed left hemi cerebral atrophy. The literature review revealed that these findings are consistent with Dyke-Davidoff-Masson syndrome. The child was discharged with oral levetiracetam (30 mg/kg/day) and counseling from parents regarding the need for long-term treatment and follow-up. Due to its rarity, Dyke-Davidoff-Masson syndrome may easily be missed by the treating physician. Knowledge of its radio-imaging features enables timely and accurate diagnosis, allowing appropriate management.