伴负性肌阵挛发作的儿童良性癫痫伴中央颞区棘波7例临床分析  

Clinical analysis of 7 cases of BECT in children with negative myoclonus

作　　者：李玉勤[1] 王新华[2] 邱鹏玲[2] 周水珍[2] 王艺[2] 周渊峰[2] 

机构地区：[1]江苏大学附属医院儿科,江苏镇江212001 [2]复旦大学儿科医院神经内科,上海200032

出　　处：《中国实用儿科杂志》2015年第4期287-291,共5页Chinese Journal of Practical Pediatrics

摘　　要：目的探讨儿童良性癫痫伴中央颞区棘波(BECT)患儿伴癫痫性负性肌阵挛(ENM)发作的临床表现、电生理特点以及诊治分析。方法对2010年1月至2014年7月复旦大学儿科医院神经科住院的7例伴ENM的BECT患儿临床表现特点(发病年龄、发作表现、电生理特点、智力受损情况)、抗癫痫药物及肾上腺皮质激素疗效进行回顾性分析。结果 7例发病前智力发育均正常,无癫痫家族史,头颅磁共振未见器质性病变。其中3例首发伴睡眠中癫痫性电持续状态(ESES),1例伴不典型失神发作。发病后2年内(平均数17.7个月)出现ENM发作形式,临床表现为站立不稳、持物掉落、手抖、跌倒等。视频脑电图监测时行直立伸臂试验阳性,脑电图和同步肌电图证实为ENM发作。出现ENM表现后,原癫痫发作频率多增加,伴智力受损,对抗癫痫药物出现难治现象,发作间期脑电图恶化。6例(85.7%)患儿存在ESES,其中1例(14.3%)存在语言障碍、5例(71.4%)存在疑似智力低下。6例进行肾上腺皮质激素治疗,甲基泼尼松龙冲击治疗后3周、3个月及6个月随访脑电图棘慢波放电指数明显减少或恢复正常,但3例(50.0%)激素减量后出现临床发作和脑电反复。结论伴ENM的不典型BECT患儿常有ESES现象及智力低下,仅用抗癫痫药物治疗多难以控制,联合激素冲击治疗对大部分患儿可控制或减少发作,但有反复现象。伴ENM的不典型BECT患儿的智力受损长期预后仍需随访。Objective To investigate the clinical manifestations, electrophysiological characteristics and diagnosis of benign epilepsy of childhood with centrotemporal spikes （BECT） accompanied by epileptic negative myoclonus （ENM）. Methods The electroclinical features （age of onset, seizure types, electrophysiological characteristics and the intelli- gence impairment）, effect of antiepileptic drugs and adrenal cortical hormone of 7 BECT patients with ENM accepted to Department of Neurology in Children＇ s Hospital of Fudan University from January 2010 to July 2014 were retrospective- ly analyzed. Results The mental development of the 7 patients was normal before the onset; there was no family history of epilepsy ; brain magnetic resonance imaging showed no organic disease. There were 7 patients with BECT conformed to the diagnostic criteria, 3 cases of first attack with electrical status epilepticus during sleep （ESES）, and 1 case of atypi- cal absence seizures. Two years after the onset （average 17.7 months）, ENM attack appeared;the clinical manifestations included standing instability, holding objects falling, tremor and falling down. Video EEG monitoring showed erect arm test was positive, and EEG and synchronization EMG confirmed the attack of ENM. After the attack of ENM, there was in- crease in seizure frequency, complicated with cognitive impairment ; antiepileptic drug had refractory phenomenon and in- terictal EEG deterioration appeared. Among the 7 patients, 6 cases were with ESES, 1 case with language barrier, 5 cases with mental retardation. Among the 7 patients, 6 cases accepted adrenal cortical hormone treatment by methylpredniso-lone. After the treatment with adrenal cortical hormone for 3 weeks, 3 months and 6 months follow-up showed electroencephalogram spike wave discharge index de- creased or returned to normal, but seizures recurred and EEG deteriorated in 3 cases after hormone decreased. Conclusion The atypical BECT in children with ENM is always associated with ESES phenomenon and

关 键 词：儿童良性癫痫伴中央颞区棘波 负性肌阵挛 抗癫痫药物治疗 预后 

分 类 号：R72[医药卫生—儿科]