57例局灶性皮质发育不良所致癫痫患儿的临床分析  

Clinical analysis of 57 children with epilepsy caused by focal cortical dysplasia

作　　者：董燕[1] 姚荷 王新军 李梦春 杨继学 单峤 贾天明[1] 李东明 吴功澳 王海艳[1] 张珂[1] Dong Yan;Yao He;Wang Xinjun;Li Mengchun;Yang Jixue;Shan Qiao;Jia Tianming;Li Dongming;Wu Gong'ao;Wang Haiyan;Zhang Ke(Department of Pediatrics,Third Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China;Department of Neurosurgery,Third Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第三附属医院儿科,郑州450052 [2]郑州大学第三附属医院神经外科,郑州450052

出　　处：《中华神经医学杂志》2024年第3期233-239,共7页Chinese Journal of Neuromedicine

基　　金：河南省医学科技攻关计划项目(LHGJ20220526);河南省小儿脑损伤重点实验室暨河南省儿科疾病临床医学研究中心联合开放课题(KFKT2021003)。

摘　　要：目的总结局灶性皮质发育不良(FCD)所致癫痫患儿的临床特征,并初步筛选影响外科手术后患儿癫痫发作控制效果的相关因素。方法选择郑州大学第三附属医院神经外科自2019年7月至2023年11月手术治疗的57例FCD所致癫痫患儿为研究对象。所有患儿均进行规范的癫痫术前评估、外科手术、术后管理及随访。回顾性总结患儿的一般资料、影像学资料、视频脑电图资料、手术方式、病理资料及随访资料等,并初步分析外科手术后患儿癫痫发作控制效果的影响因素。结果57例患儿中男29例(50.88%),女28例(49.12%);首次癫痫发作年龄为30.00(8.00,74.50)个月,外科手术年龄为95.00(50.00,138.50)个月;42例(73.68%)的癫痫发作类型为局灶性泛化至全面性强直-阵挛发作,13例(22.81%)为癫痫性痉挛发作。34例(59.65%)患儿头颅MRI阳性,常见表现为脑沟/脑回形态异常(17/57,29.82%)。接受PET-CT检查的43例患儿中40例(93.02%)检测出低代谢部位,40例(93.02%)的PET/MRI融合显像上病灶范围与实际手术范围一致。FCD病理分型Ⅰ型16例(28.07%)、Ⅱ型39例(68.42%)、Ⅲ型2例(3.51%)。截至2023年12月,44例(77.19%)患儿的预后为EngelⅠ级、4例(7.02%)为Ⅱ级、4例(7.02%)为Ⅲ级、5例(8.77%)为Ⅳ级。预后良好(EngelⅠ+Ⅱ级)与预后不良(EngelⅢ+Ⅳ级)患儿在首次癫痫发作至外科手术时间、MRI阳性或阴性、术后是否规律服用抗癫痫发作药物等方面的差异均有统计学意义(P<0.05)。结论局灶性泛化至全面性强直-阵挛发作是FCD所致癫痫患儿最常见的癫痫发作类型,脑沟/脑回形态异常是其最常见的MRI阳性表现,PET/MRI融合显像在致痫灶确定方面具有较大优势。首次癫痫发作至外科手术时间长、头颅MRI阴性及术后不规律服用抗癫痫药物患儿术后癫痫发作控制效果较差。Objective To summarize the clinical characteristics of patients with epilepsy caused by focal cortical dysplasia(FCD),and identify the influencing factors for postoperative seizure controls.Methods Fifty-seven patients with epilepsy caused by FCD admitted to Department of Neurosurgery,Third Affiliated Hospital of Zhengzhou University from July 2019 to November 2023 were chosen;standard preoperative evaluation,surgery,postoperative management and follow-up were performed.A retrospective study of clinical data,imaging and video electroencephalogram(VEEG)data,surgical approaches,pathological findings,and follow-up data was performed;influencing factors for postoperative seizure controls were analyzed.Results In these 57 patients with epilepsy caused by FCD,29 were males(50.88%)and 28 were females(49.12%).Onset age was 30.00(8.00,74.50)months,and surgery age was 95.00(50.00,138.50)months.Focal to bilateral tonic-clonic seizures(42/57;73.68%)and epileptic spasms(13/57;22.81%)were common seizure types.Cranial MRI was positive in 34 patients(59.65%),mainly manifested as abnormal cortical gyri/sulci morphology(17/57;29.82%).In 43 patients accepted PET-CT,hypometabolic sites were detected in 40(93.02%),and complete agreement between PET/MRI fusion results and actual lesion sites was noted in 40(93.02%).FCD type I was noted in 16 patients(28.07%),type II in 39(68.42%),and type III in 2(3.51%).By December 2023,44(77.19%)had Engel grading I,4(7.02%)had grading II,4(7.02%)had grading III,and 5(8.77%)had grading IV.Children with good prognosis(Engel grading I+II)and those with poor prognosis(Engel grading III+IV)showed significant differences in terms of time from first seizure to surgery,positive/negative MRI,and regularity of postoperative ASMs(P<0.05).Conclusions Focal to bilateral tonic-clonic seizure is the most common seizure type in patients with epilepsy caused by FCD,and abnormal cortical gyri/sulci morphology is the most common MRI manifestation;PET/MRI fusion imaging is superior to PET-CT or MRI in identifying epile

关 键 词：局灶性皮质发育不良 癫痫 临床特征 癫痫发作控制 

分 类 号：R742.1[医药卫生—神经病学与精神病学]