Dyke Davidhoff Masson Syndrome Presenting in Adulthood: A Case Report

Abstract

Dyke Davidhoff Masson syndrome (DDMS) was first described by Dyke Davidhoff and Masson in 1933 in a series of 9 patients [1]. It’s a very rare condition presenting in childhood ranging from infancy to late adolescence [2]. It is characterised by cerebral hemiatrophy, unilateral ventriculomegaly, calvarial thickening, skull and facial asymmetry, sometimes even contralateral hemiplegia in childhood and seizures [2]. An adult presentation is seen in late adolescence or teen age. However in literature the latest presentation reported so far is at 19 years of age [3]. We report a case of Dyke Davidhoff Masson syndrome who presented first time at the age of 28 years which is probably the oldest presentation of this rare entity reported in the literature.