伴中央颞区棘波的儿童良性癫痫变异型的电-临床研究

Electroclinical features of the atypical of benign childhood epilepsy with central temporal spikes

目的 探讨儿童及青少年伴中央颞区棘波良性癫痫(Benign epilepsy with centrotemporal spikes,BECTs)变异型的电-临床特征及意义.方法 收集2008年7月-2015年12月在儿童医疗中心神经科住院的51例BECTs变异型患儿资料,整理并分析临床发作类型、电生理特征及对抗癫痫药物治疗的反应.结果 患儿年龄2.5~11岁,平均7.03岁.病程4d^6年,平均2.36年.其中19例患儿脑电图(EEG)放电指数约达90%,26例放电指数约达75%,6例放电指数约达52%;所有患儿平均放电指数为82.44%.不典型症状手抖、掉物等47例,占92.1%;口、咽部自动症8例,占15.6%;语言障碍7例,占13.7%;清醒期出现失神样发作或跌倒11例,占21.5%;认知功能下降17例,占33.3%.12例患儿单药丙戊酸钠(VPA)效果好,33例患儿需VPA及卡马西平(CBZ)联合用药.6例难以控制发作,加用甲强龙激素治疗后症状改善.结论 BECTs变异型伴有明显的EEG恶化,不规范用药可造成BECTs患儿病情的加重,应注意EEG的监测随访.抗癫痫药物治疗的目的不仅在于控制发作,同时应有效抑制EEG的异常放电.

Objective To identify clinical and electroencephalographic features of the variants of benign childhood epilespy with centrotemporal spikes(BECTs).Methods A total of 51 children with BECTs were restrospectively reviewed from July 2008 to December 2015 in the study,ineluding the clinical data,electrophysiologic characteristics and efects of antiepileptic drugs.Results Age of the patients ranged from2.5 lo 11 years old,which were averged 7.03 years old.The duration of disease varied from 4 days to6 years,and 2.36 years in average.Nearly continuous electrie discharge were detected in slow sleeping,during which,the busting index was 90%in 19 patients',78%in 26,52%in 6.the average busting index was 82.44%.47 patients(92.1%)had synptom of hand shaking;8 patients(15.6%)had oropharyngeal automatism;7 patients(13.7%)had language barrier;sample absence seizures or lumble occurred in 11 patients(21.5%);cognitive declined in 17 patients(33.3%).VPA monotherapy had good effeet on 12 patients,33 patients need combination of VPA and CBZ.However,there's still 6 patients need adrenocortical hormone to control seizures.Conclusion The variants of BECT are companied with obvious deterioration of EEG.Lack of standard AED therapy may cause aggravations,so we need to monitor EEG closely.We use AED not merely in orde to control seizure but also inhibit abnormal EEG discharge.