Rationale: Rasmussen Encephalitis (RE) is an inflammatory unihemispheric disease with focal seizures and progressive neurological decline. Hemispherotomy (HE) is considered the most effective seizure treatment. The expected postsurgical deficits (hemiparesis, hemianopia and - in case of an affected dominant hemisphere – aphasia) often make a decision in favor of surgery difficult.
Information about long-term seizure outcome, neurological performance and quality of life (QoL) might help weighing the benefits of surgical versus conservative treatment. The aim of this study is to compare long-term outcomes of RE patients after HE compared to those without this treatment.
Methods: We retrospectively identified the patients with RE and a disease duration of ≥ 2 years from our hospital information system and invited them to a personal study visit. We assessed seizure outcome, gross- and fine-motor function (GMFCS level, grip strength, finger tapping, pegboard), QoL (Quolie31) and burden of disease (FaBel questionnaire). Patients who were unable or unwilling to come in person were asked for a telephone interview (seizure outcome).
Results: We identified 89 patients. Forty-one patients could not be assessed (18 from foreign countries, 15 without valid contact information, and eight refusals). Three non-operated patients had died, all from epilepsy-related causes. Forty-five patients were included: 17 of them underwent HE (38%) with a mean follow up after surgery of 9.0 years (2.0-25.2). Disease duration of the non-HE patients was 15.1 years (3.9-62.4). One RE patient never experienced seizures.
While all patients with epilepsy and without HE still had seizures, 13/17 (77%) of the HE group had an Engel 1A outcome, and 3/4 non-seizure free patients had only single focal non-aware seizures (Engel IIB). Antiseizure medication was completely withdrawn in 9/13 (69%) of the seizure-free patients after surgery, while non-operated patients had a high drugload ( > 2 ASM: 13/19%).
A group of 30 patients completed the whole study protocol including functional testing, QoL- and burden of disease assessment. Hemiparesis was associated with loss of finger grip and finger extension, but all operated patients were able to walk without assistance (GMFCS level 2).
While overall QoL was not different between the two groups, health state was perceived as better in the HE group (median HE group 80 vs. 65 non-HE, U-Test p< .05). The burden of disease (FaBel) was higher in the non-operated group (mean HE group 0.23 vs. non-HE 0.41, T-test p< .05).