Surgical Treatment of Patients with Rasmussen Encephalitis

Abstract

Background: Rasmussen encephalitis (RE) is a rare progressive encephalitis that results in intractable seizures, cognitive decline and hemiparesis. Surgery seems to be the only effective way to control seizures in RE patients. Objective: To describe the clinical, electrophysiological, neuroradiological and histological findings of our patients with RE and to evaluate the outcome of their surgical treatment. Methods: A total of 20 patients were identified by the criteria of RE. Surgery was conducted in the left hemisphere in 6 patients and in the right hemisphere in 14. The surgical methods included anatomical hemispherectomy, functional hemispherectomy, hemispherotomy, lesion resection, multilobar resection, selective resection and bipolar electrocoagulation of functional cortexes. Results: The mean follow-up period was 5.45 years (range 3-8). After surgery, 16 patients (80%) were evaluated as being Engel class I. All of the patients had increases in cognitive abilities after surgery except 1 patient with bilateral RE. After surgery, most patients could walk independently, but the fine movement of the hands was lost. Postoperative hydrocephalus was observed in 1 patient after functional hemispherectomy, and there was no death in this series. Conclusion: Hemispherectomy and hemispherotomy were both confirmed as beneficial procedures in controlling seizures and improving quality of the life in RE cases.