儿童和青少年癫相关局灶性皮质发育不良22例临床分析  

Clinical analysis on 22 patients with childhood and juvenile epilepsy related focal cortical dysplasia

作　　者：刘旸[1] 邓馨[1] 刘凤君[1] 陈静[1] 郭小明[1] 王利霞[1] 吴逊[1] 王薇薇[1] LIU Yang;DENG Xin;LIU Feng-jun;CHEN Jing;GUO Xiao-ming;WANG Li-xia;WU Xun;WANG Wei-wei(Department of Neurology, Peking University First Hospital, Beijing 100034, China)

机构地区：[1]北京大学第一医院神经内科,100034

出　　处：《中国现代神经疾病杂志》2018年第6期412-417,共6页Chinese Journal of Contemporary Neurology and Neurosurgery

摘　　要：研究背景局灶性皮质发育不良是一组包括皮质分层异常、细胞结构异常和细微白质异常的疾病,是难治性癫的主要病因。本研究总结儿童和青少年局灶性皮质发育不良的临床表现、脑电图和头部MRI特点,以期提高诊断与治疗水平。方法回顾分析22例儿童和青少年局灶性皮质发育不良患者的临床表现、脑电图和头部MRI特点。结果 22例患者中13例(59.09%)单纯表现为局灶性发作,6例(27.27%)单纯表现为全面性发作,3例(13.64%)表现为局灶性发作继发全面性发作;发作频率较频繁,6例(27.27%)每日均有发作,13例(59.09%)≥1次/月,3例(13.64%)<1次/月;21例(95.45%)脑电图监测到异常慢波和样放电。12例MRI显示局灶性皮质发育不良位于额叶,头皮脑电图显示7例(7/12)局灶性额区异常慢波和样放电,2例(2/12)广泛性慢波和棘慢复合波,2例(2/12)颞区尖波,1例(1/12)中央中线少量尖波;单纯局灶性发作7例(7/12),全面性发作4例(4/12),局灶性发作继发全面性发作1例(1/12)。6例MRI显示局灶性皮质发育不良位于顶叶,头皮脑电图显示3例(3/6)局灶性顶区异常慢波和样放电,2例(2/6)颞枕区样放电,1例(1/6)无异常;单纯局灶性发作5例(5/6),局灶性发作继发全面性发作1例(1/6)。2例MRI显示局灶性皮质发育不良位于颞叶,头皮脑电图显示1例(1/2)局灶性颞区异常慢波和样放电,1例(1/2)额区样放电;均为全面性发作。2例MRI显示局灶性皮质发育不良位于岛叶,头皮脑电图显示1例(1/2)双侧颞区样放电,1例(1/2)全导联尖波和尖慢复合波;单纯局灶性发作1例(1/2),局灶性发作继发全面性发作1例(1/2)。结论局灶性皮质发育不良患者通常于学龄前期和学龄期发病,主要呈现局灶性或全面性发作,亦可见其他发作类型,发作频率较频繁;头部MRI是诊断局灶性皮质发育不良的重要方法;治疗方法主要是抗癫药物,发作控制欠佳的患者考虑癫外科手术Background Focal cortical dysplasia （FCD） is a group of diseases including abnormal cortical layer, abnormal cell structure and abnormal white matter. It is one of the main causes of intractable epilepsy. In this paper, the clinical manifestations, EEG and MRI characteristics of FCD in children and adolescents were summarized in order to improve the diagnosis and treatment level of clinicians. Methods The clinical symptoms, EEG and MRI manifestations of 22 cases were retrospectively analyzed. Results Among 22 cases, 13 cases （59.09%） only showed focal seizures, 6 cases （27.27%） only showed generalized seizures, and 3 cases （13.64%） showed focal to generalized seizures. The seizure frequency of 22 patients was namely： 6 cases （27.27%） had seizures daily, 13 cases （59.09%） were more than one time/month, and 3 cases （13.64%） were less than one time/month. EEG in 21 cases （95.45%） showed abnormal slow waves and epileptiform discharges. MRI of 12 cases showed FCD in frontal lobe, the scalp EEG of whom showed abnormal slow waves and epileptiform discharges in focal frontal region in 7 cases （7/12）, extensive slow waves and spike and slow wave complex in 2 cases （2/12）, temporal sharp waves in 2 cases （2/12）, and a small amount of sharp waves at midline in one case （1/12）. Simple focal seizures occurred in 7 cases （7/12）, generalized seizures occurred in 4 cases （4/12）, and focal to generalized seizures occurred in one case （1/12）. MRI of 6 cases showed FCD in parietal lobe, the scalp EEG of whom showed abnormal slow waves and epileptiform discharges in focal parietal region in 3 cases （3/ 6）, epileptiform discharges in temporo-occipftal region in 2 cases （2/6）, and no abnormality in one case （1/ 6）. Simple focal seizures occurred in 5 cases （5/6）, and focal to generalized seizures occurred in one case （1/6）. MRI of 2 cases showed FCD in temporal lobe, the scalp EEG of whom showed abnormal slow waves and epileptiform discharges in focal te

关 键 词：癫[疒间] 皮质发育畸形 儿童 青少年 磁共振成像 脑电描记术 

分 类 号：R742.1[医药卫生—神经病学与精神病学]