Dyke-Davidoff-Masson syndrome

Dyke-Davidoff-Masson syndrome (DDMS) refers to atrophy or hypoplasia of one cerebral hemisphere, due to an insult to the developing brain in fetal or early childhood period. Age of presentation depends on the time of neurologic insult, and characteristic changes may be seen only in adolescence. Male gender and left hemisphere are more frequently involved. A 17-year-old female adolescent with a history of recurrent refractory seizures, hemiplegia and mental retardation reported to Department of Radiology for computed tomography (CT) assessment of brain. On examination, she had facial asymmetry, delayed milestones, and spastic hemiplegia. The CT brain showed right cortical atrophy with ventricular dilatation, prominent sulci, and shifting of falx to the right side. Bone window image showed asymmetry in skull vault thickness, the width of diploic space, the size of paranasal air sinuses and inclination of the petrous ridge between the affected and normal sides. As the above case deviates from the usual presentation of male left sided DDMS, hence the report.