Clinical neurology and neurosurgery · 2026
Surgical management of Rasmussen's encephalitis: A systematic review and meta-analysis of long-term seizure and functional outcomes.
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Alomari Omar, Matar Hasan, Bandak Natalie, Alrabadi Bassel, Halit Alioglu, Turan Süslü Hikmet
Аннотация
OBJECTIVE: Rasmussen's encephalitis (RE) is a rare, progressive inflammatory brain disease often leading to drug-resistant epilepsy and severe neurological decline. While surgery is the definitive treatment, evidence remains fragmented due to the rarity of the condition. We conducted a systematic review and meta-analysis to evaluate the efficacy of various surgical techniques and identify predictors of long-term seizure freedom.
METHODS: We performed a comprehensive search of PubMed, Scopus, Web of Science, and Cochrane Library in accordance with PRISMA guidelines. We included studies reporting surgical outcomes in patients with RE. Pooled proportions were calculated using random-effects meta-analysis, and meta-regression was employed to investigate predictors of seizure freedom, including age at surgery and surgical technique.
RESULTS: Thirty-one studies comprising 460 patients were included. The overall pooled rate of long-term seizure freedom (Engel Class I/ILAE Class 1) was 65.0% (95% CI: 53.0%-76.0%). Subgroup pooling demonstrated high rates of long-term seizure freedom following complete hemispheric disconnection procedures, with hemispherotomy achieving 75.0% (95% CI: 63.0%-85.0%) and functional hemispherectomy achieving 66.0% (95% CI: 50.0%-80.0%). However, meta-regression confirmed no statistically significant difference in efficacy between disconnective techniques (p = 0.2243). In contrast, focal or less-than-hemispheric resections demonstrated significantly inferior efficacy (35.0%, p = 0.0086). Meta-regression analysis indicated that earlier age at surgery was a robust predictor of superior long-term seizure outcomes (beta = -0.0383, p = 0.0165), explaining 27.98% of between-study heterogeneity. Shorter disease duration prior to surgery was likewise significantly associated with higher seizure freedom (beta = -0.0412, p = 0.0210). A modest trend toward improved outcomes was observed in recent publication eras. Baseline clinical severity (preoperative EPC, hemiparesis) and complication rates were not significant predictors. Postoperative gross motor function and language preservation were generally stable, ranging from 67.7% to 94.1% and 84-93.1%, respectively.
CONCLUSION: Complete hemispheric disconnection (whether via hemispherotomy or functional hemispherectomy) is highly effective for controlling seizures in RE, whereas focal resections provide inadequate control. Earlier surgical intervention and shorter disease duration significantly improve long-term seizure freedom. These findings support prompt referral for hemispheric disconnection in patients with medically refractory RE rather than delaying surgery with prolonged immunomodulatory trials.
METHODS: We performed a comprehensive search of PubMed, Scopus, Web of Science, and Cochrane Library in accordance with PRISMA guidelines. We included studies reporting surgical outcomes in patients with RE. Pooled proportions were calculated using random-effects meta-analysis, and meta-regression was employed to investigate predictors of seizure freedom, including age at surgery and surgical technique.
RESULTS: Thirty-one studies comprising 460 patients were included. The overall pooled rate of long-term seizure freedom (Engel Class I/ILAE Class 1) was 65.0% (95% CI: 53.0%-76.0%). Subgroup pooling demonstrated high rates of long-term seizure freedom following complete hemispheric disconnection procedures, with hemispherotomy achieving 75.0% (95% CI: 63.0%-85.0%) and functional hemispherectomy achieving 66.0% (95% CI: 50.0%-80.0%). However, meta-regression confirmed no statistically significant difference in efficacy between disconnective techniques (p = 0.2243). In contrast, focal or less-than-hemispheric resections demonstrated significantly inferior efficacy (35.0%, p = 0.0086). Meta-regression analysis indicated that earlier age at surgery was a robust predictor of superior long-term seizure outcomes (beta = -0.0383, p = 0.0165), explaining 27.98% of between-study heterogeneity. Shorter disease duration prior to surgery was likewise significantly associated with higher seizure freedom (beta = -0.0412, p = 0.0210). A modest trend toward improved outcomes was observed in recent publication eras. Baseline clinical severity (preoperative EPC, hemiparesis) and complication rates were not significant predictors. Postoperative gross motor function and language preservation were generally stable, ranging from 67.7% to 94.1% and 84-93.1%, respectively.
CONCLUSION: Complete hemispheric disconnection (whether via hemispherotomy or functional hemispherectomy) is highly effective for controlling seizures in RE, whereas focal resections provide inadequate control. Earlier surgical intervention and shorter disease duration significantly improve long-term seizure freedom. These findings support prompt referral for hemispheric disconnection in patients with medically refractory RE rather than delaying surgery with prolonged immunomodulatory trials.