Dyke-Davidoff-Masson Syndrome: A Case of Unilateral Cerebral Atrophy and Seizure Disorder

Dyke-Davidoff-Masson syndrome (DDMS) is an infrequently occurring neurological entity characterized by cerebral hemiatrophy and a collection of cognitive, motor and seizure-related symptoms. We describe the case of an 18-year-old male with a long-standing history of generalized tonic-clonic seizures following a significant fall at the age of 4. His antenatal and perinatal history was insignificant, with appropriately achieved developmental milestones. Despite limited academic attainment, he was oriented and fully independent in daily functions. The Magnetic Resonance Imaging (MRI) of the brain revealed marked left cerebral hemiatrophy with prominent parieto-occipital and temporal involvement, dilation of the occipital horn of the lateral ventricle, and T2/FLAIR hyperintense signals that are consistent with chronic gliosis. Laboratory investigations and electroencephalography (EEG) were unremarkable. Clinical correlation with radiological evidence supported the diagnosis of DDMS. This report contributes to the growing understanding of DDMS as a varied condition with presentations that may diverge from classical descriptions. Recognizing atypical radiological findings and correlating them with patient history is essential for making a correct diagnosis and leading a long-term prognosis.