Abstracts

Rationale: To describe the clinical, electrophysiologic, neuroradiologic, and histologic findings of patients with Rasmussen encephalitis (RE) and to evaluate the outcome of their surgical treatment.Methods: This report was based on a consecutive series of 34 patients affected by RE studied and operated on at the Department of Functional Neurosurgery, Sanbo Brain Hospital from April 2004 to December 2010. The age at surgery was from 2.8 years to 17 years. 34 RE patients were confirmed by presurgical evaluation including semiology, magnetic resonance imaging (MRI), interictal/ictal scalp video-electroencephalography (VEEG), intracranial recording and biopsy. Presurgical evaluation modalities and surgical treatment were analyzed retrospectively.Results: 19 functional hemispherectomy (FH), 9 anatomical hemispherectomy (AH), 6 hemispherotomy procedures were undertaken. According to Engel s criteria, 28 patients (83%) achieved an Engel Class I status, and 3 patient (9%) had an Engel Class II outcome, 2 patients (6%) had a significant decrease in seizure frequency (Engel Class III) and 1 patients (3%) had no change in seizure frequency (Engel Class IV). 1 patient showed contralateral seizure after AH and be diagnosed as bilateral RE. All of the patients excepting one bilateral RE had increases in cognitive abilities, behavior, and quality of life after the surgery. After the surgery, most of the patients can walk independently, but the fine movement of the hands were lost. The main early complications were fever (35%) after hemispheric surgeries. Postoperative hydrocephalus was observed one and a half years after AH in one patient and no death in of this series.Conclusions: Hemispherectomy and hemispherotomy were confirmed as both beneficial procedures in controlling seizures and improving quality of the life in cases with RE.