结节性硬化症不同类型皮质结节的致痫性研究  

Epileptogenicity of different types of cortical tubers in patients with tuberous sclerosis complex

作　　者：王海祥[1] 张冰清[1] 贺晶[1] 丰倩[1] 柏建军[1] 周文静[1] Wang Haixiang;Zhang Bingqing;He Jing;Feng Qian;Bai Jianjun;Zhou Wenjing(Epilepsy Center,Tsinghua University Yuquan 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)癫痫中心,北京100040

出　　处：《中华实用儿科临床杂志》2023年第11期869-873,共5页Chinese Journal of Applied Clinical Pediatrics

基　　金：国家重点研发计划(2022YFC2503800)。

摘　　要：目的利用立体定向脑电图(SEEG)对结节性硬化症(TSC)患儿不同类型脑皮质结节进行记录及皮质电刺激,探讨其致痫性的不同。方法回顾性分析2016年11月至2022年9月在清华大学玉泉医院癫痫中心进行药物难治性癫痫术前评估的50例[男27例,女23例,年龄(5.5±3.4)岁]局灶性癫痫起始的TSC患儿的SEEG及皮质电刺激资料。根据头颅3.0T磁共振成像(3T-MRI)及CT结果,将患儿脑皮质结节进行分型,分析不同类型结节出现SEEG记录的电-临床发作、电发作及皮质电刺激诱发发作的情况,采用Fisher′s精确检验比较不同类型结节之间致痫结节率和电刺激发作阳性率的差异。结果50例SEEG局灶起始的TSC患儿共探查303个皮质结节。结节分为6型:Type A~Type E及类局灶性皮质发育不良(FCD-like)型,其中Type E为国际上首次提出。303个皮质结节中,7个出现电发作,57个出现电-临床发作,共有致痫结节64个(21.1%)。不同类型结节致痫结节率Type A~Type E分别为3.6%、1.4%、19.0%、77.8%、77.5%,FCD-like为90.0%。Fisher′s精确检验并经Bonferroni法校正两两比较(P<0.003为差异有统计学意义):Type A、Type B、Type C类型结节致痫结节率之间差异无统计学意义;Type A、Type B、Type C分别与Type D、Type E、FCD-like类型结节致痫结节率比较,差异均有统计学意义;Type D、Type E和FCD-like之间比较,差异均无统计学意义。36个(11.9%)皮质结节出现电刺激诱发发作,Type A~Type E类型结节电刺激发作阳性率分别为:0.7%、1.4%、4.8%、44.4%、45.0%,FCD-like型为70.0%。Type A、Type B分别与Type D、Type E、FCD-like比较,差异均有统计学意义;Type C与Type E、FCD-like型比较,差异有统计学意义;余两两比较差异均无统计学意义。结论本研究提出了新的TSC脑皮质结节分型,其中Type E型为国际上首次提出。SEEG记录证实不同类型皮质结节的致痫性存在较大差异,Type D、Type E及FCD-like型具有更高致�Objective To record stereoelectroencephalography(SEEG)data and to induce cortical electrical stimulation in children with tuberous sclerosis complex(TSC),thus exploring the epileptogenicity of different types of cortical tubers.Methods The SEEG recording and cortical electrical stimulation data of 50 children with TSC who underwent preoperative evaluation for drug-resistant epilepsy at Epilepsy Center,Tsinghua University Yuquan Hospital from November 2016 to September 2022 were retrospectively analyzed,involving 27 boys and 23 girls with the age of(5.5±3.4)years.According to the results of 3.0T magnetic resonance imaging(3T-MRI)and computed tomography(CT),cortical tubers were classified.The incidences of electroclinical seizures,electrical seizures and seizures induced by cortical electrical stimulation in different types of tubers recorded by SEEG were analyzed,and the differences in the proportion of the above seizures among different types of tubers were compared using the Fisher′s exact test.Results A total of 303 cortical tubers were explored using SEEG in 50 patients.The tubers were divided into 6 types,including Type A,B,C,D and E,and focal cortical dysplasia like(FCD-like)type,among which Type E was for the first time proposed in the world.Among these explored tubers,7 tubers had electrical seizures,and 57 tubers had electroclinical seizures.A total of 64 tubers(21.1%)were epileptogenic.The incidence of epileptogenic tubers in Type A-E and FCD-like type were 3.6%,1.4%,19.0%,77.8%,77.5%,and 90.0%,respectively.Fisher′s exact test and Bonferroni correction were performed for pairwise comparisons(P<0.003).There was no significant difference in the incidence of epileptogenic tubes among Type A,B and C.There was significant difference in the incidence of epileptogenic tubes between Type A-C with Type D,Type E and FCD-like type,respectively.There was no significant difference in the incidence of epileptogenic tubes between Type D,Type E and FCD-like like.Electrical stimulation-induced seizures occurred in 3

关 键 词：癫痫 结节性硬化症 立体定向脑电图 

分 类 号：R741.044[医药卫生—神经病学与精神病学] R725.9[医药卫生—临床医学]