Long‐term seizure outcome and mobility after surgical treatment for Rasmussen encephalitis in children: A single‐center experience

Abstract

ObjectiveRasmussen Encephalitis (RE) is a rare inflammatory neurodegenerative disease associated with refractory seizures, hemiparesis, and cognitive deterioration, due to lateralized cortical atrophy. Hemispheric surgery (hemispherotomy) is the mainstay of treatment, but its unavoidable motor deficits and lack of long‐term data regarding seizure outcomes can make patients and families apprehensive to undergo this procedure. The present study aimed at analyzing the results of surgical treatment for RE from a motor and epilepsy standpoint, and mitigate such concerns.MethodsClinical and operative data were retrospectively collected from medical records of pharmacoresistant patients treated with functional hemispherectomy at a tertiary reference center for epilepsy surgery, during a 24‐year period (1996–2020). Variables such as age of epilepsy onset, seizure semiology, seizure frequency, immunomodulatory therapy, age at surgery, duration of epilepsy, surgical procedures and complications, number of medications used preoperatively and postoperatively were described and statistically analyzed.ResultsForty‐three (43) patients were included in this study. Mean age of epilepsy onset was 6.14 years, the average interval between epilepsy onset and hemispherotomy was 2.21 years. and the mean age at surgery was 8.28 years. Thirty patients (69.7%) were Engel I at their last follow‐up, of whom 23 (56.4%) were Engel Ia, within a mean follow‐up of 11.3 years. Duration of epilepsy, seizure frequency, and age at surgery, among others, did not correlate with seizure outcome, except the use of immunotherapy which led to worse outcomes (p < .05). Also, after surgery, motor functionality was significantly recovered (i.e., most patients returned to their previous status) with time.SignificanceThis study tackled some issues regarding the surgical treatment of this disease, particularly showing that hemispherotomy is safe and leads to potentially recoverable disability of motor functions while providing high rates of effective and long‐lasting seizure control; therefore, early surgical indication should be warranted once medical refractoriness has been established.

Misdirection due to early magnetoencephalographic presentation and management in Rasmussen encephalitis: a case report

“…Reportedly, approximately 2.4 out of every 100,000 people in Germany suffer from this disease ( 1 ). These patients often have focal epilepsy, progressive hemiplegia, and atrophy of one hemisphere ( 2 , 3 ). Histopathological examination shows infiltration of T cells and microglia as prominent features.…”