RARE CASE OF DYKE-DAVIDOFF MASSON SYNDROME

DYKE-DAVIDOFF MASSON SYNDROME is a rare clinical disorder of brain characterized by cerebral hemiatrophy, contralateral hemiparesis, seizures, facial asymmetry and mental retardation. It was initially described by CG DYKE, LM DAVIDOFF and C B MASSON in 1933. A 7 years old male child, born second to third degree consanguinous parents, was brought with complaints of multiple seizure episodes daily with weakness of left upper limb and lower limb for past 3 years. On examination child had microcephaly, facial asymmetry and mental retardation. MRI brain showed diffuse atrophy of right cerebral hemisphere with ipsilateral ventricular dilatation with occlusion of middle cerebral artery suggestive of DYKE DAVIDOFF MASSON SYNDROME. It is caused by an in utero or early childhood cerebral insult. Here we present a case of Dyke-Davidoff Masson Syndrome.