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.

Anti–tumor necrosis factor alpha therapy (adalimumab) in Rasmussen's encephalitis: An open pilot study

“…Current treatments of RE aim at reducing seizure frequency/severity and avoiding the progressive cognitive and/or motor decline usually observed in long‐term outcomes. Currently, hemispherotomy is the best option to cure seizures, with a seizure‐free rate of ~90%, but this treatment is associated with inevitable functional consequences . Therefore, hemispherotomy is mainly indicated in patients with the classical form of RE who have intractable epilepsy and a severe deficit.…”

“…Currently, hemispherotomy is the best option to cure seizures, with a seizure-free rate of~90%, but this treatment is associated with inevitable functional consequences. 30,31 Therefore, hemispherotomy is mainly indicated in patients with the classical form of RE who have intractable epilepsy and a severe deficit. Moreover, in cases of dominant hemi- Adalimumab effect in patients with or without tendency to seizure frequency fluctuation.…”

Endoscopy-Assisted Interhemispheric Transcallosal Hemispherotomy

“…Hemispherotomy can achieve excellent outcomes when performed in optimally indicated patients. 3,[6][7][8]10,14,16,18,20,28,35,[43][44][45][46][47][48][49][50][51][52][53] Since the introduction of functional hemispherectomy by Rasmussen, morbidity and mortality have steadily decreased. 8,10,11,13,14,28,[43][44][45][46][47]54 However, this procedure still cannot be considered as a trivial surgery because most of the surgeries are performed in children who cannot tolerate blood loss and are prone to other perioperative morbidities such as hypothermia, electrolyte disturbances, and other problems associated with pediatric patients.…”

Rasmussen’s encephalitis: mechanisms update and potential therapy target

“…Surgical treatment is a more satisfactory therapeutic strategy for RE. 26,27 There are three main types of surgical operation: anatomical hemispherectomy, functional hemispherectomy, and disconnection hemisphere. [28][29][30] Moreover, anatomical hemispherectomy surgery is more effective in controlling epileptic seizures within acceptable complications compared with hemisphere disconnection surgery and functional hemispherectomy surgery, as shown by a series of 45 cases at our epilepsy center.…”