儿童Sturge-Weber综合征继发癫痫的临床脑电图特征及手术治疗  

The Clinical manifestations,electroencephalogram features and surgical treatment and of epilepsy secondary to Sturge-Weber syndrome in children

作　　者：于国静 季涛云[1,2] 车圆圆 王文[1] 刘通 王宏杰 王若凡[1,2] 王爽[1,2] 刘庆祝[1] 蔡立新[1] 刘晓燕[1,2] YU Guojing;JITaoyun;CHE Yuanyuan;WANG Wen;LIU Tong;WANG Hongjie;WANG Ruofan;WANG Shuang;LIU Qingzhu;CAI Lixin;LIU Xiaoyan(Department of Pediatrics,Peking University First Hospital,Beijing 100034,China;Pediatric Epilepsy Center,Peking University First Hospital,Beijing 100034,China)

机构地区：[1]北京大学第一医院儿童癫痫中心,北京100034 [2]北京大学第一医院儿科,北京100034

出　　处：《癫痫杂志》2022年第1期41-47,共7页Journal of Epilepsy

基　　金：北京大学第一医院交叉临床研究专项课题(2017CR23)。

摘　　要：目的总结经手术治疗儿童Sturge-Weber综合征(Sturge-Weber syndrome,SWS)继发癫痫的癫痫发作特点、脑电图特征、手术方式及术后疗效,提高对此类疾病的认识。方法回顾性分析我院儿童癫痫中心自2015年5月—2020年5月经手术治疗7例SWS的患儿资料。收集7例患儿人口学特征、癫痫发作形式、脑电图、头颅影像学、手术方式及预后等。结果共收集患儿7例,其中男1例、女6例。癫痫起病年龄2月龄~1岁6月龄,平均(6.43±5.26)月龄。7例患儿均以局灶性发作起病,2例病程中出现癫痫持续状态(1例为持续性部分性癫痫),1例有痉挛发作,1例表现为丛集性发作的特点。发作间期脑电图特点:(1)随着病程延长,脑电图逐渐加重,表现为患侧慢波逐渐增多(7/7),波幅逐渐变低(7/7),生理波消失(4/7);(2)发作间期脑电图常表现为无癫痫样放电/偶见或少量癫痫样放电。4例行半球离断术,1例颞顶枕离断术,2例行病灶切除术。术后随访时间6个月~5年,平均随访时间(79.29±24.50)个月。6例术后定期随访均EngelⅠa,1例术后3个月EngelⅡ级,术后6个月及1年EngelⅢ级。结论SWS继发癫痫发作形式多样,脑电图具有特征性改变。若为药物难治性癫痫,通过详尽的术前评估,采取合理手术方式,可取的较好的治疗效果。Objective To investigate the clinicalmanifestations,electroencephalogram(EEG)characteristics,surgical treatment and prognosis of epilepsy secondary to Sturge-Weber syndrome(SWS)in children.Methods The data of 7 children with epilepsysecondaryto Sturge-Weber syndrome who were treated by surgery from May 2015 to May2020 in our Children’s Epilepsy Center were retrospectively reviewed.Their demographic characteristics,seizure forms,results of EEG and cranial imaging investigations,surgical methods,postoperative pathological reports and prognosis during follow-upwere summarized and analyzed.The prognosis were evaluated by Engel classificationat the last time point during follow-up.Results Totally 7 pediatric patients were enrolled,including 1 male(16/25,64.0%)and 6 females.All the 7 cases presented with focal seizures at the onset among whom 2 cases developed status epilepticus during the course of the disease(epilepsiapartialiscontinuain 1 case),one case had epileptic spasmsand 1 case was characterized by cluster seizures.The interictal EEG manifestations of the cases gradually deteriorated as the course of the disease prolonged,including the slow wave on the affected side gradually increases(7/7),the amplitude gradually decreases(7/7),and the physiological wave disappears(4/7).Besides,no epileptiform discharges/incidental or a few epileptiform dischargeswere found in their interictal EEGs.Four cases underwent hemispherotomy,1 case underwent temporo-parieto-occipital disconnection and 2 cases underwent lesion resection.The cases were followed up for 6 months to 5 years,and the average follow-up duration was 79.29 months.Six cases were rated as EngelⅠa during the regularfollow-up.Only 1 case was rated as EngelⅡand EngelⅢat 3 month and 1 year after the operation.Conclusion Children with epilepsy secondary to SWS usually present with focal seizures and have diverse seizure forms.The EEG show characteristic changes.For the caseswith drug refractory epilepsy,detailed preoperative evaluation and reasonable surgical

关 键 词：STURGE-WEBER综合征 药物难治性癫痫 脑电图 外科治疗 

分 类 号：R725.9[医药卫生—儿科] R726.5[医药卫生—临床医学]