Dyke-Davidoff-Masson syndrome: Adult female patient with refractory epilepsy and global cognitive decline

Abstract

The Dyke-Davidoff-Masson syndrome is characterized by the presence of cerebral hemiatrophy, craneal vault thickening, epileptic seizures, hemiparesis, and cognitive impairment. It is typically diagnosed in childhood and requires specific diagnostic tools for accurate assessment. This report describes the case of a 19-year-old woman who presented with epileptic seizures and regression in neurodevelopment. She was admitted to the emergency department due to high ictal frequency. During her hospitalization, imaging and electroencephalographic findings were consistent with Dyke-Davidoff-Masson syndrome. Additionally, neuropsychological tests revealed global cognitive impairment. After ten days of hospitalization and five days without epileptic seizures, the patient was discharged. Dyke-Davidoff-Masson syndrome is a rare and often unrecognized condition with high morbidity. Clinicians has the responsibility to identify the key characteristics of the syndrome and perform an adequate differential diagnosis.