Bellamkonda Nikhil, Phillips H Westley, Chen Jia-Shu, Tucker Alexander M, Maniquis Cassia, Mathern Gary W, Fallah Aria
Departments of1Neurosurgery.
4Department of Neuroscience, Brown University, Providence, Rhode Island; and.
J Neurosurg Pediatr. 2020 Jul 17;26(4):389-397. doi: 10.3171/2020.4.PEDS2098. Print 2020 Oct 1.
Rasmussen encephalitis (RE) is a rare inflammatory neurological disorder typically involving one hemisphere and resulting in drug-resistant epilepsy and progressive neurological decline. Here, the authors present seizure outcomes in children who underwent epilepsy surgery for RE at a single institution.
The records of consecutive patients who had undergone epilepsy surgery for RE at the UCLA Mattel Children's Hospital between 1982 and 2018 were retrospectively reviewed. Basic demographic information, seizure history, procedural notes, and postoperative seizure and functional outcome data were analyzed.
The cohort included 44 patients, 41 of whom had sufficient data for analysis. Seizure freedom was achieved in 68%, 48%, and 22% of the patients at 1, 5, and 10 years, respectively. The median time to the first seizure for those who experienced seizure recurrence after surgery was 39 weeks (IQR 11-355 weeks). Anatomical hemispherectomy, as compared to functional hemispherectomy, was independently associated with a longer time to postoperative seizure recurrence (HR 0.078, p = 0.03). There was no statistically significant difference in postoperative seizure recurrence between patients with complete hemispherectomy and those who had less-than-hemispheric surgery. Following surgery, 68% of the patients could ambulate and 84% could speak regardless of operative intervention.
A large proportion of RE patients will have seizure relapse after surgery, though patients with anatomical hemispherectomies may have a longer time to postoperative seizure recurrence. Overall, the long-term data in this study suggest that hemispheric surgery can be seen as palliative treatment for seizures rather than a cure for RE.
拉斯穆森脑炎（RE）是一种罕见的炎症性神经系统疾病，通常累及一个半球，导致药物难治性癫痫和进行性神经功能衰退。在此，作者介绍了在单一机构接受RE癫痫手术的儿童的癫痫发作结果。
回顾性分析了1982年至2018年期间在加州大学洛杉矶分校马特儿童医院接受RE癫痫手术的连续患者的记录。分析了基本人口统计学信息、癫痫发作史、手术记录以及术后癫痫发作和功能结果数据。
该队列包括44例患者，其中41例有足够的数据进行分析。分别有68%、48%和22%的患者在1年、5年和10年时实现了无癫痫发作。术后癫痫复发患者首次癫痫发作的中位时间为39周（四分位间距11 - 355周）。与功能性半球切除术相比，解剖性半球切除术与术后癫痫复发时间较长独立相关（风险比0.078，p = 0.03）。全半球切除术患者与半球以下手术患者术后癫痫复发无统计学显著差异。手术后，无论手术干预如何，68%的患者能够行走，84%的患者能够说话。
很大一部分RE患者术后会癫痫复发，尽管接受解剖性半球切除术的患者术后癫痫复发时间可能较长。总体而言，本研究的长期数据表明，半球手术可视为癫痫的姑息治疗而非RE的治愈方法。
