A case of acute functional hemispherotomy in a young woman with hemimegalencephaly and super-refractory status epilepticus

Abstract

Status epilepticus (SE) is a critical medical emergency that demands immediate and effective intervention. We report a unique case involving a 21-year-old woman with left hemimegalencephaly who was hospitalized for super-refractory status epilepticus (SRSE) that persisted for 8 weeks. Despite extensive treatment efforts including multiple anti-seizure medications, anesthetics, high-dose methylprednisolone, anakinra, magnesium infusion, and a ketogenic diet, her condition remained unchanged. Ultimately, a left functional hemispherotomy was performed, informed by MRI findings and EEG results showing predominantly left-sided epileptic activity. This decision was made collaboratively when all other therapeutic options had been exhausted. Postoperatively, the patient recovered with manageable neurological deficits and a satisfactory quality of life. To our knowledge, this is the first reported case of acute functional hemispherotomy in an adult with hemimegalencephaly and SRSE.