Dyke-Davidoff-Masson syndrome: A rare case of hemiatrophy with chronic seizure disorder

Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological disorder characterized by cerebral hemiatrophy, leading to seizures, hemiparesis, and cognitive deficits. We report the case of a 20-year-old female with a history of chronic seizure disorder and left-sided hemiparesis. The patient experienced her first seizure at 6 months of age, followed by recurrent generalized tonic seizures throughout childhood. Despite treatment, she continued to have mild seizure episodes (brief changes in consciousness or less noticeable symptoms) twice a month. Additionally, left-sided motor weakness had been present since infancy, significantly affecting her daily activities. Magnetic Resonance Imaging (MRI) revealed right-sided cerebral atrophy with extensive cystic encephalomalacia, ex vacuo dilatation of the right lateral ventricle, and gliosis in the right fronto-parietal and fronto-temporal regions. Compensatory bony changes, including asymmetrical thickening of the calvarium and enlargement of the right frontal sinus, were also noted. Wallerian degeneration on the right side was observed. These imaging findings, along with the patient's clinical presentation, were consistent with the diagnosis of Dyke-Davidoff-Masson Syndrome. Management was focused on controlling seizures with antiepileptic medications and improving motor function through physical therapy. This case underscores the importance of early recognition of DDMS in patients with early-onset seizures and hemiparesis. Early diagnosis and appropriate intervention are essential for optimizing patient outcomes and improving quality of life.