Dyke-Davidoff-Masson Syndrome Presenting as Status Epilepticus: A Case Report

Dyke-Davidoff-Masson Syndrome (DDMS) is an uncommon neurological condition resulting from damage to the developing cerebral hemisphere during the prenatal period or early childhood, leading to impaired neuronal development and hemispheric volume loss. Patients commonly manifest with recurrent seizures, unilateral motor deficits, facial asymmetry, and varying degrees of cognitive impairment. Neuroimaging typically demonstrates unilateral cerebral hemiatrophy accompanied by compensatory osseous changes of the skull. Although DDMS is a recognised cause of chronic epilepsy, its presentation as status epilepticus is unusual. The authors report a case of a 17-year-old female who presented with status epilepticus and was subsequently diagnosed with DDMS based on characteristic magnetic resonance imaging findings showing ipsilateral cerebral atrophy, mild ipsilateral falcine shift and asymmetric thickening of calvarium. The diagnosis was supported by clinical history, neurological examination, and radiological features. Management involved acute seizure control followed by long-term antiepileptic therapy and rehabilitative support. The present case highlights the importance of considering DDMS in the differential diagnosis of status epilepticus, particularly in patients with long-standing unilateral neurological deficits.