Dyke-Davidoff-Masson Syndrome Presenting With Recurrent Seizures and Left-Sided Motor Weakness: A Case Report.

This study aimed to highlight that, regardless of a condition's rarity, every patient seeks timely recognition and effective management, underscoring clinicians' responsibility to remain vigilant and informed to provide optimal care. Dyke-Davidoff-Masson syndrome, first described in 1933, represents a classic radiological and clinical manifestation of this condition. This syndrome is characterized by unilateral cerebral atrophy accompanied by compensatory skull thickening and hyperpneumatization of the paranasal sinuses. The underlying cerebral insult may occur during fetal development or early childhood, and based on the timing of injury, the condition can be categorized as congenital or acquired. Various etiologies have been implicated, including vascular insults, trauma, infections, neoplasms, and idiopathic causes. Neuroimaging plays a crucial role not only in establishing the diagnosis but also in providing insight into the timing and extent of the cerebral injury. We report a case of a 15-year-old male with a history of recurrent focal seizures since the third day of life, developmental delay, and left-sided motor weakness. He had been on long-term antiepileptic therapy and presented with a breakthrough seizure following brief non-adherence to medication. Radiological evaluation revealed classical features of right cerebral hemiatrophy, along with other findings confirming the diagnosis of Dyke-Davidoff-Masson syndrome. This case highlights the classical clinical presentation and imaging findings of a rare syndrome and emphasizes the importance of early recognition, strict adherence to antiepileptic drugs, caregiver counseling, and supportive rehabilitation to improve long-term functional outcomes in affected individuals.