伴中央颞区棘波小儿良性癫痫变异型临床分析  

Clinical analysis of the variants of benign childhood epilepsy with centro-temporal spikes

作　　者：施晓容[1,2] 包新华[1] 刘晓燕[1] 侯绍荣[1] 秦炯[1] 吴希如[1] 

机构地区：[1]北京大学第一医院儿科,北京100034 [2]福建医科大学附属第一医院儿科,福建福州350005

出　　处：《中国实用儿科杂志》2014年第7期528-533,共6页Chinese Journal of Practical Pediatrics

摘　　要：目的探讨伴中央颞区棘波小儿良性癫痫（BECT）变异型临床表现、神经电生理特点、治疗及预后。方法对2001年10月至2012年12月北京大学第一医院儿科神经科门诊或住院的67例BECT变异型儿童临床资料、视频脑电图（VEEG）特点、治疗反应及预后进行2～10年的随访及总结。结果患儿首次出现癫痫发作的年龄为1岁6个月至10岁,出现BECT变异型临床表现的年龄为2岁6个月至10岁6个月。所有患儿脑电图（EEG）均表现为Rolandic区放电,其中25例（37.3%）为非快速眼动睡眠（NREM）期放电指数≥85%,42例（62.7%）NREM期放电指数为50%～〈85%。51例（76.1%）监测到负性肌阵挛发作。应用口服抗癫痫药物治疗10例发作控制,5例发作减少〈50%,52例无效。单独应用口服抗癫痫药物无效患儿中50例联合应用皮质激素治疗,2～10年随访,其中21例（42.0%）发作控制,25例（50.0%）发作减少〈50%,4例发作未控制。14例（28.0%）于甲基泼尼松龙治疗后1个月至1年2个月复发。激素治疗后6个月评估言语智商（VIQ）及操作智商（PIQ）均有提高（P〈0.05）。长期随访的学龄期患儿60例中30例（50.0%）学习成绩差,对其中25例进行神经心理评估,20例（80.0%）有不同程度认知功能受损。结论 BECT变异型除发作频繁及出现新的发作形式外,常伴认知受损、EEG恶化。多数患儿口服抗癫痫药物难以控制发作,激素治疗对大部分患儿有效,6个月治疗使临床症状、认知和EEG显著改善,但复发率较高。部分BECT变异型远期预后不理想,遗留认知功能下降及行为异常。Objective To study the electro-clinical features and prognosis of the variants of benign childhood epilepsy with centro-temporal spikes（BECT）.Methods Sixty-seven patients with BECT variants were diagnosed in the hospital.They have been followed up for 2 to 10 years.The clinical information, including clinical manifestation,video-EEG,the response to the medical treatment,and the prognosis of the patients, was studied. Results The onset of the seizures in these patients was from 1 year and 6 months to 10 years,and the median age was 4 years and 5 months. The symptoms of BECT variants presented from 2 years and 6 months to 10 years and 6 months,the median age was 6 years. The EEGs of all patients showed abundance of spike and waves in Rolandic areas during wake-up and sleep.In 25 cases（37.3%）,the index of discharge in non-rapid eye movements（NREM）was more than 85%.In 42 cases（62.7%）,the index was between 50% and 85%.Epileptic negative myoclonus（ENM）was detected in 51 cases（76.1%）by the outstretched arm tests during the EEG recording.All the patients were treated with antiepileptic drugs（AED）,only 10 cases were seizure free,and the seizure frequency decreased more than 50% in 5 cases.There were no responses in the other 52 cases.Then the corticosteroid was administered to 50 cases who were resistant to AED. Among them,21 cases were seizure free; in 25 cases,the seizure frequency decreased more than 50%,and only 4 cases had no response to this treatment. Fourteen cases（28%）relapsed 1 month to 1 year and 2 months after corticosteroid therapy.Six months after the corticosteroid therapy,verbal intelligence quotient（VIQ）and performance intelligence quotient（PIQ）increased（P0.05）.A long time follow-up of 60 school-aged children showed that 30 cases（50%）had poor school performance.Neuropsychological assessment was performed on 25 cases,and the cognitive decline was observed in 20 cases（80%）.Conclusion BECT variants should be considered in patients with BECT when the seizure

关 键 词：伴中央颞区棘波小儿良性癫痫变异型 脑电-临床特点 治疗 

分 类 号：R72[医药卫生—儿科]