Case Report: Management of Hemiconvulsion Hemiplegic Epilepsy Syndrome Utilizing Ketamine and Ketogenic Diet

Hemiconvulsion-Hemiplegia-Epilepsy (HHE) syndrome is a rare but serious cause of super-refractory status epilepticus (SRSE) in young children. It is characterized by prolonged febrile hemiconvulsions, acute unilateral hemispheric injury, and later development of epilepsy. Because of its rarity and heterogeneous presentation, standardized management guidelines are lacking. We report a 17-month-old girl with HHE syndrome in whom seizure control was achieved using a multimodal treatment strategy that included high-dose ketamine and initiation of a ketogenic diet.