Hemispherectomy with corpus callosotomy in pediatric Lennox Gastaut Syndrome associated encephalomalacia cyst: The first case in Indonesia

Abstract

Lennox-Gastaut syndrome (LGS) is a form of severe epileptic encephalopathy in children. LGS with encephalomalacia cysts is rare in children. We report a six-year-old mentally retarded boy who was referred for an intractable seizure. Seizures were tonic, atonic, and dialeptic in frequency. EEG showed generalized SSW discharges of 1.5-2 Hz, polyspikes, and burst suppression typical for LGS. Head MRI showed an encephalomalacia cyst in the right subcortical temporoparietal lobes with hemiatrophy in the right cerebral hemisphere. He was already treated with three antiepileptic drugs, but the seizures persisted. The patient was then performed right hemispherectomy and corpus callosotomy. It resulted in a good response. A combination of hemispherectomy and corpus callosotomy could be promising in this form of epilepsy disease. Seizure reduction was achieved and showed cognitive improvement and hemiparesis.