Dyke-Davidoff-Masson Syndrome

Dyke-Davidoff-Masson Syndrome (DDMS) is rarely seen and a clinical entity with features of drug-resistant epileptic seizures, cerebral hemiatrophy, contralateral hemiparesia, facial asymmetry, mental retardation or learning disability. A 27 year-old-male patient presented with complaint of seizure. His first attack was occurred when he was three-days-old. His seizures were complex partial seizures. He has used varios of antiepileptics but seizures could not be controlled since he was three-years-old. Neurological examination revealed mild hemiparesis, hyperactive deep tendon reflexes and Babinsky’s sign positivity at the right side. Magnetic resonance imaging (MRI) of the brain revealed left cerebral cortical hemiatrophy. The patient was diagnosed with DDMS considering that learning difficulties in additon to the above clinical features. He has used 1000 mg/day carbamezepine and 200 mg/day topiramate for 10 months and he is seizure free now. Absence, complex partial or secondary generalized seizures may seen in patients with DDMS. Surgical procedures like hemispherectomy may be performed in patients who have frequent and drug-resistant seizures. In our patient, seizures were controlled with oral antiepileptic drugs without any need of surgery. This case is presented because of the rarity of the syndrome and demonstratively of the brain MRI.