A Case Report on Rasmussen Encephalopathy (RE)

Abstract

Summary: Rasmussen's encephalitis (RE) is a rare and severe chronic inflammatory brain disease resulting in drug-resistant epilepsy and progressive hemispheric destruction with neurological deficit. ER is associated with deterioration of background EEG activity, progressive atrophy of the affected hemisphere on MRI and extensive PET hypometabolism over the affected hemisphere. We report the case of a 6-year-old girl who presented with pharmacoresistant epilepsy. Observation: A 6-year-old child with no particular medical history presented 6 months prior to admission with epilepsy resistant to all therapeutic classes (antiepileptic drugs). Clinical examination revealed left hemiparesis with facial involvement and constant left hemi-myoclonus. An EEG showed a pattern compatible with a continuous epileptic encephalopathy that could correlate with Rasmussen syndrome. Cerebral MRI Revealed: right cerebral hemiatrophy with secondary homolateral hippocampal sclero-atrophy, compatible with Rasmussen encephalitis. The patient received immunoglobulins and a corticosteroid bolus, but without improvement; she was therefore a candidate for surgical treatment. Rasmussen's encephalopathy is a rare and serious disease that can be responsible for paresis and cognitive decline. Its pathophysiology is still poorly defined, and it mainly affects children.