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DYKE-DAVIDOFF-MASSON SYNDROME (DDMS): A RARE PREVENTABLE CAUSE OF REFRACTORY EPILEPSY

PRADOSH KUMAR SARANGI, PRAGNYAMAYA DEBADATTA MANGARAJ, JAYASHREE MOHANTY, SASMITA PARIDA, BASANTA MANJARI SWAIN.

Abstract
Dyke-Davidoff-Masson syndrome (DDMS), also called as cerebral hemiatrophy is a rare disease which clinically presents with contralateral spastic hemiplegia or hemiparesis, seizures, facial asymmetry, and mental retardation. The classical radiological findings include cerebral hemiatro-phy, calvarial thickening and hyperpneumatization of the frontal sinuses. This disease is a rare entity, and it classically presents in childhood. Adult presentation of DDMS is unusual and has been rarely reported in the medical literature. This case illustrates typical computed tomographic features of DDMS in a 11-year- old boy who presented with recurrent generalized seizures since childhood, left hemiparesis with mental retardation.

Key words: Dyke-Davidoff-Masson syndrome, cerebral hemiatrophy, Sturge-Weber syndrome, epilepsy, Rasmussen encephalitis


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