A Shrunken Hemisphere, A Life time of Seizures: Dyke-Davidoff- Masson Syndrome

Abstract

Dyke -Davidoff- Masson Syndrome (DDMS) is rare neurological disorder, first described in 1933, commonly affecting the children but is rarely reported in adults as well. It mostly presents with seizure, contralateral hemiparesis followed by mental retardation and facial asymmetry due to palsy of the facial nerve (CN VII). The classical radiological findings are cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. We here in report a case of 25 years old male who presented with repeated generalized tonic clonic seizures for ten years He was on regular anti convulsive therapy and failed to control seizure. General examination revealed no significant abnormality with delayed mile stones of development. Neurological examination revealed right sided spastic hemiparesis, brisk tendon reflexes and extensor planter on left side. Ultimately, he was diagnosed as DDM. This case report aims to draw the attention of health care professionals to keep DDM as a differential in a patient with drug resistant intractable epilepsy.