DYKE DAVIDOFF MASSON SYNDROME

Harshita Ganji*, Raghavendra Prasad and Prabhakar Kamarthy

ABSTRACT

Dyke-Davidoff- Masson syndrome (DDMS) is a rare congenital malformation that was first described by Dyke, Davidoff, and Masson in 1933. It is characterised by hemiparesis or hemiplegia, seizures, mental retardation, facial asymmetry. Radiological features of this syndrome includes hemiatrophy of cerebral hemisphere, ipsilateral compensatory hypertrophy of skull and sinuses. Aetiology of cerebral hemiatrophy can be divided into congenital or primary and acquired. Congenital causes can be idiopathic or intrauterine cerebral vascular injury. Acquired causes are birth trauma, perinatal intracranial haemorrhage, Rasmussen encephalitis, infection, Sturge Weber syndrome. DDMS, thus can be congenital or acquired and it is commonly due to vascular insult involving middle cerebral artery. Treatment of DDMS is symptomatic targeting convulsion, hemiparesis, learning difficulties, etc. Here we report a 29 year old male born via full-term uncomplicated normal vaginal delivery to a second degree consanguineously married parents presented to the emergency department with recurrent generalized tonic clonic seizures since 8 months of age.

Keywords: Dyke-Davidoff- Masson syndrome, cerebral hemiatrophy, Rasmussen encephalitis, Sturge Weber syndrome