Abstract：
OBJECTIVE: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
摘要：
目的：拉斯穆森脑炎（RE）是一种罕见的与难治性癫痫发作相关的炎症性神经退行性疾病，偏瘫,和认知退化，由于皮质萎缩.半球手术（半球切开术）是治疗的主要手段，但其不可避免的运动障碍和缺乏有关癫痫发作结局的长期数据可能会使患者和家属担心接受该手术.本研究旨在从运动和癫痫的角度分析RE的手术治疗结果，减轻这种担忧。
方法：回顾性地收集了在三级参考中心癫痫手术接受功能性大脑半球切除术治疗的药物耐药患者的医疗记录，在24年期间（1996-2020年）。变量如癫痫发作的年龄，癫痫发作符号学，癫痫发作频率,免疫调节治疗,手术年龄，癫痫的持续时间，外科手术和并发症，对术前和术后使用的药物数量进行描述和统计分析.
结果：本研究纳入了43例患者。癫痫发作的平均年龄为6.14岁，癫痫发作与大脑半球切开术的平均间隔时间为2.21年.手术时的平均年龄为8.28岁.30例(69.7%)患者在最后一次随访时是EngelI，其中23人(56.4%)是恩格尔Ia，平均随访11.3年。癫痫的持续时间，癫痫发作频率,和手术年龄，其中,与癫痫发作结果无关，除了使用免疫疗法导致更差的结果（p<0.05）。此外，手术后,电机功能显著恢复(即，大多数患者恢复到以前的状态)。
结论：这项研究解决了一些关于这种疾病的手术治疗的问题，特别是显示半球切开术是安全的，并导致运动功能的潜在可恢复的残疾，同时提供有效和持久的癫痫发作控制的高比率;因此，一旦医学难治性确立,应保证早期手术指征.
方法：回顾性地收集了在三级参考中心癫痫手术接受功能性大脑半球切除术治疗的药物耐药患者的医疗记录，在24年期间（1996-2020年）。变量如癫痫发作的年龄，癫痫发作符号学，癫痫发作频率,免疫调节治疗,手术年龄，癫痫的持续时间，外科手术和并发症，对术前和术后使用的药物数量进行描述和统计分析.
结果：本研究纳入了43例患者。癫痫发作的平均年龄为6.14岁，癫痫发作与大脑半球切开术的平均间隔时间为2.21年.手术时的平均年龄为8.28岁.30例(69.7%)患者在最后一次随访时是EngelI，其中23人(56.4%)是恩格尔Ia，平均随访11.3年。癫痫的持续时间，癫痫发作频率,和手术年龄，其中,与癫痫发作结果无关，除了使用免疫疗法导致更差的结果（p<0.05）。此外，手术后,电机功能显著恢复(即，大多数患者恢复到以前的状态)。
结论：这项研究解决了一些关于这种疾病的手术治疗的问题，特别是显示半球切开术是安全的，并导致运动功能的潜在可恢复的残疾，同时提供有效和持久的癫痫发作控制的高比率;因此，一旦医学难治性确立,应保证早期手术指征.
