Sturge-Weber综合征继发癫痫的临床特点及预后分析  

Clinical features and surgical prognosis of epilepsy caused by Sturge-Weber syndrome

作　　者：史洁[1] 王海祥[1] 柏建军[1] 林久銮[1] 张冰清[1] 刘一鸥[1] 周文静[1] Shi Jie;Wang Haixiang;Bai Jianjun;Lin Jiuluan;Zhang Bingqing;Liu Yiou;Zhou Wenjing(Department of Neurosurgery,Tsinghua University Yuquan Hospital(Tsinghua University Integrated Traditional Chinese and Western Medicine Hospital),Beijing 100040,China)

机构地区：[1]清华大学玉泉医院(清华大学中西医结合医院)神经外科,北京100040

出　　处：《中华神经外科杂志》2024年第12期1205-1211,共7页Chinese Journal of Neurosurgery

基　　金：国家重点研发计划(2022YFC2503800)。

摘　　要：目的分析总结Sturge-Weber综合征(SWS)继发癫痫的临床特点及预后。方法回顾性分析2013年1月至2024年3月于清华大学玉泉医院(清华大学中西医结合医院)神经外科进行癫痫术前评估的SWS患者,共9例。术前评估后6例行手术治疗,其中1例行大脑半球切除术,1例行大脑半球离断术;4例行小于半球切除术,其中单侧颞顶枕叶切除术1例,单侧颞顶枕叶离断术2例,局部剪裁式切除术1例。3例行药物治疗。观察患者的临床特点及疗效。结果9例患者中,SWSⅠ型5例,Ⅲ型4例。癫痫起病中位年龄为8个月(1个月至7.5岁);9例均为局灶性发作,其中2例为局灶性癫痫性痉挛发作。行头颅MRI T1增强扫描的8例患者均可见局部软脑膜强化,其中累及单侧半球4例、单侧局部3例、双侧半球1例;9例患者头颅MRI均显示灰白质交界不清等局灶性皮质发育不良(FCD)征象。6例手术患者的中位随访时间为2.9(0.6~10.5)年,其中Engel分级Ⅰ级4例,Engel分级Ⅱ级2例(分别于术后10个月及5.0年复发,发作频率均较术前减少90%以上)。术前2例视频脑电图显示异常范围大于手术切除范围的患者术后均复发;而2例影像学异常范围大于手术切除范围者中,术后1例复发。3例药物治疗者随访期均无复发。结论SWS继发癫痫起病较早,癫痫发作分型多为局灶性发作。除软脑膜血管畸形外,MRI影像常见FCD征象。切除性手术对癫痫发作的控制良好。术前VEEG异常范围大于手术切除范围可能与术后癫痫复发有关。ObjectiveTo analyze and summarize the clinical characteristics and prognosis of epilepsy secondary to Sturge-Weber syndrome(SWS).MethodsA retrospective analysis was conducted on 9 SWS patients who underwent preoperative evaluation for epilepsy in the Department of Neurosurgery at Tsinghua University Yuquan Hospital(Tsinghua University Integrated Traditional Chinese and Western Medicine Hospital)from January 2013 to March 2024.Six patients underwent surgical treatment after preoperative evaluation,which included 1 case of right hemispherectomy,1 case of right hemispherotomy,and 4 cases of resections of less than a hemisphere(1 case of unilateral temporo-parieto-occipital resection,2 cases of unilateral temporo-parieto-occipital disconnections,and 1 case of focal tailored resection).Three patients received medical treatment.The clinical characteristics and outcomes were documented.ResultsThis group had 5 SWSⅠand 4 SWSⅢcases.The median age of seizure onset was 8 months(range:1 month to 7.5 years).All 9 patients had focal seizures,including 2 with focal epileptic spasms.MRI T1-enhanced scans of 8 patients showed localized leptomeningeal enhancement(one hemisphere involved in 4,part of one hemisphere involved in 3,and both hemispheres involved in 1).All patients showed signs of focal cortical dysplasia(FCD)on MRI,such as unclear gray-white matter junctions.The median follow-up time for the 6 surgical patients was 2.9 years(range:0.6-10.5 years),with 4 patients achieving Engel classⅠand 2 patients achieving Engel classⅡ(relapsed at 10 months and 5.0 years postoperatively,with>90%reduction in seizure frequency).Two patients with preoperative video EEG abnormalities exceeding the surgical resection area relapsed after surgery,while among the two patients with imaging abnormalities exceeding the resection area,only one relapsed.None of the three patients receiving medical treatment had recurrence during follow-up.ConclusionsEpilepsy secondary to SWS has an early onset,with focal seizures being the most common type

关 键 词：STURGE-WEBER综合征 癫痫 疾病特征 神经外科手术 预后 

分 类 号：R742.1[医药卫生—神经病学与精神病学]