首页|改良大脑半球离断术治疗儿童Sturge-Weber综合征并发癫痫的疗效分析

改良大脑半球离断术治疗儿童Sturge-Weber综合征并发癫痫的疗效分析

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目的 探讨改良大脑半球离断术治疗儿童Sturge-Weber综合征(SWS)并发癫痫的手术疗效.方法 回顾性分析2009年1月至2020年12月首都医科大学三博脑科医院神经外科采用改良大脑半球离断术治疗的38例SWS并发癫痫患儿的临床资料.术后2年通过门诊或网络平台随访并分析患儿的手术疗效,包括癫痫预后及认知功能的改善情况.癫痫预后采用国际抗癫痫联盟(ILAE)癫痫预后标准,认知功能评估采用Kelly神经学评分.同时按手术年龄≤1岁、>1~3岁、>3~6岁、>6岁分层比较患儿手术前、后的认知功能评分.结果 38例患儿均顺利行改良大脑半球离断术.术后无一例患儿死亡或出现神经功能障碍.术后2年随访时,38例患儿中33例ILAE1级,2例3级,1例4级,2例5级.认知功能方面,38例患儿术后2年的认知功能评分优于术前[分别为(1.32±0.96)分、(1.79±1.09)分],差异具有统计学意义(配对t=3.17,P=0.003).38例患儿中,16例术后认知功能较术前改善,19例无变化,3例恶化.手术年龄≤1岁和>1~3岁的患儿术后认知功能评分[(0.90±0.57)分和(1.20±1.01)分]均优于术前[(1.80±1.14)分和(1.73±1.10)分],差异均具有统计学意义(均P<0.05),而手术年龄>3~6岁和>6岁患儿的术前与术后2年认知功能评分的差异均无统计学意义(均P>0.05).结论 改良大脑半球离断术可安全、有效地控制SWS并发药物难治性癫痫的发作,并可在一定程度上改善患儿的认知功能.
Analysis of the efficacy of modified hemispherotomy in the treatment of children with Sturge-Weber syndrome complicated by epilepsy
Objective To explore the surgical efficacy of modified hemispherotomy in the treatment of children with Sturge-Weber syndrome(SWS)complicated by epilepsy.Methods A retrospective analysis was conducted on the clinical data of 38 children with SWS complicated by epilepsy who were treated with modified hemispherotomy at the Department of Neurosurgery,Sanbo Brain Hospital,Capital Medical University from January 2009 to December 2020.The children's surgical efficacy,including seizure outcome and improvement in cognitive function,was followed up through outpatient clinics or online platforms 2 years after surgery and analyzed.Epileptic seizure outcome was assessed based on the International League Against Epilepsy(ILAE)criteria,and cognitive function was assessed using the Kelly score.At the same time,the cognitive function scores of children before and after surgery were stratified according to the age of surgery 1 year old,>1 to 3 years old,>3 to 6 years old,and>6 years old.Results A total of 38 children underwent modified hemispherotomy.There were no instances of mortality or the development of neurological deficits following surgery.At the two-year follow-up,33 of the 38 children exhibited ILAE grade 1,2 exhibited grade 3,1 exhibited grade 4,and 2 exhibited grade 5.With regard to cognitive function,the children's cognitive function scores were superior to their preoperative scores at the two-year follow-up,with a statistically significant difference observed between the two groups(1.32±0.96 vs.1.79±1.09).The paired t-test yielded a statistically significant result(t=3.17,P=0.003).Of the 38 children,16 exhibited improved cognitive function postoperatively compared with their preoperative scores,19 demonstrated no change,and 3 exhibited a decline.Postoperative cognitive function scores(0.90±0.57)and(1.20±1.01)were superior to preoperative scores(1.80±1.14)and(1.73±1.10)in children aged≤1 year and>1-3 years following surgery.The observed differences were statistically significant(all P<0.05),whereas the differences between the preoperative and 2-year postoperative cognitive function scores in children aged>3-6 and>6 years were not statistically significant(all P>0.05).Conclusion Modified hemispherotomy can safely and effectively control seizures in medically refractory epilepsy complicated by SWS and can improve cognitive function to some extent in children.

Sturge-Weber syndromeDrug resistant epilepsyTreatment outcomeModi-fied hemispherotomy

彭鑫涛、丁浩然、孙卫进、王雄飞、周健、王梦阳、王静、李天富、栾国明、关宇光

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首都医科大学三博脑科医院神经外科,北京 100093

首都医科大学三博脑科医院神经内科,北京 100093

Sturge-Weber综合征 耐药性癫痫 治疗结果 改良大脑半球离断术

国家重点研发计划

2021YFC2401203

2024

中华神经外科杂志
中华医学会

中华神经外科杂志

CSTPCD北大核心
影响因子:1.107
ISSN:1001-2346
年,卷(期):2024.40(9)