Center for Pediatric Epilepsy Surgery (CIREP), Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
Department of Pediatrics, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
Epileptic Disord. 2023 Oct;25(5):749-757. doi: 10.1002/epd2.20147. Epub 2023 Aug 17.
Rasmussen Encephalitis (RE) is a rare inflammatory neurodegenerative disease associated with refractory seizures, hemiparesis, and cognitive deterioration, due to lateralized cortical atrophy. Hemispheric surgery (hemispherotomy) is the mainstay of treatment, but its unavoidable motor deficits and lack of long-term data regarding seizure outcomes can make patients and families apprehensive to undergo this procedure. The present study aimed at analyzing the results of surgical treatment for RE from a motor and epilepsy standpoint, and mitigate such concerns.
Clinical and operative data were retrospectively collected from medical records of pharmacoresistant patients treated with functional hemispherectomy at a tertiary reference center for epilepsy surgery, during a 24-year period (1996-2020). Variables such as age of epilepsy onset, seizure semiology, seizure frequency, immunomodulatory therapy, age at surgery, duration of epilepsy, surgical procedures and complications, number of medications used preoperatively and postoperatively were described and statistically analyzed.
Forty-three (43) patients were included in this study. Mean age of epilepsy onset was 6.14 years, the average interval between epilepsy onset and hemispherotomy was 2.21 years. and the mean age at surgery was 8.28 years. Thirty patients (69.7%) were Engel I at their last follow-up, of whom 23 (56.4%) were Engel Ia, within a mean follow-up of 11.3 years. Duration of epilepsy, seizure frequency, and age at surgery, among others, did not correlate with seizure outcome, except the use of immunotherapy which led to worse outcomes (p < .05). Also, after surgery, motor functionality was significantly recovered (i.e., most patients returned to their previous status) with time.
This study tackled some issues regarding the surgical treatment of this disease, particularly showing that hemispherotomy is safe and leads to potentially recoverable disability of motor functions while providing high rates of effective and long-lasting seizure control; therefore, early surgical indication should be warranted once medical refractoriness has been established.
拉森姆脑炎（RE）是一种罕见的炎症性神经退行性疾病，由于侧化皮质萎缩，与难治性癫痫发作、偏瘫和认知恶化有关。半球切除术（半球切开术）是主要的治疗方法，但它不可避免的运动缺陷和缺乏长期的关于癫痫发作结果的数据，会使患者和家属对接受这种手术感到担忧。本研究旨在从运动和癫痫发作的角度分析 RE 的手术治疗结果，并减轻这种担忧。
回顾性收集了 24 年间（1996-2020 年）在一家癫痫手术三级参考中心接受功能性半球切除术治疗的耐药性癫痫患者的病历中的临床和手术数据。描述和统计分析了发病年龄、癫痫发作半侧化、癫痫发作频率、免疫调节治疗、手术年龄、癫痫持续时间、手术过程和并发症、术前和术后使用的药物数量等变量。
本研究共纳入 43 例患者。癫痫发作的平均年龄为 6.14 岁，癫痫发作和半球切开术之间的平均间隔为 2.21 年，手术平均年龄为 8.28 岁。30 例（69.7%）患者在最后一次随访时为 Engel I 级，其中 23 例（56.4%）为 Engel Ia 级，平均随访时间为 11.3 年。除免疫治疗导致较差的结果（p<0.05）外，癫痫持续时间、癫痫发作频率和手术年龄等因素与癫痫发作结果无相关性。此外，手术后，随着时间的推移，运动功能显著恢复（即大多数患者恢复到以前的状态）。
本研究探讨了这种疾病的手术治疗的一些问题，特别是表明半球切开术是安全的，并导致潜在可恢复的运动功能障碍，同时提供高比例的有效和持久的癫痫控制；因此，一旦确定了药物耐药性，就应该尽早进行手术治疗。
