儿童良性癫痫伴中央颞区棘波变异型的临床和脑电图特点研究分析

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

目的总结儿童良性癫痫伴中央颞区棘波(BECT)变异型的临床表现、脑电图特点和治疗。方法 1999年5月至2009年3月在北京大学第一医院儿科神经病房收治的BECT变异型患儿29例,其中男9例,女20例。对29例患儿临床资料进行分析,通过视频脑电图(VEEG)分析发作类型和脑电图(EEG)特点,18例同时监测双侧三角肌表面肌电图(EMG)。随访患儿对抗癫痫药物和皮质激素的治疗反应。结果睡眠期部分性发作起病年龄2～8岁,出现不典型症状距首次发作的病程为2～57个月(平均21个月),不典型症状包括手抖、持物落地27例,点头、站立不稳或跌倒4例,语言表达障碍13例,口咽部运动障碍11例,完全失语3例,认知功能下降18例。14例出现不典型症状期间睡眠期部分性发作加重。29例患儿EEG均显示清醒和睡眠期大量Rolandic区棘慢波发放,慢波睡眠期棘慢波指数为50%～85%。VEEG监测到癫痫性负性肌阵挛27例(通过直立伸臂试验证实),不典型失神11例,11例应用抗癫痫药物(丙戊酸单用或联合应用氯硝西泮、左乙拉西坦或拉莫三嗪)治疗有效,18例联合应用抗癫痫药物和皮质激素临床症状和EEG放电改善。结论 BECT变异型的临床特点包括病程中出现负性肌阵挛、不典型失神、口咽部失用和认知功能下降,同时伴EEG恶化。单用抗癫痫药物或联合应用皮质激素治疗有效。

Objective To study the electroclinical features of the variants of benign childhood epilepsy with central temporal spikes（BECT）.Methods Video electroencephalography（EEG）monitoring was performed in 29 BECT patients who had some atypical symptoms during the course of the disease,and some of them were examined simultaneously with electromyography（EMG）.The seizure semiology,electrophysiological features,responses to antiepileptic drugs and corticosteroids were analyzed.Results The onset age of partial seizures during sleep was from 2 to 8 years.The atypical symptoms following disease onset was from 2 to 57 months.The clinical presentations of the variants of BECT included hand trembling and objects dropping（n = 27）,head nodding and instability or dropping attack during standing（n = 4）,speech deterioration（n = 13）,oromotor deficits（n = 11）,and aphasia（n = 3）.Cognitive decline could be observed in 18 cases.The typical rolandic seizures were exaggerated in 14 patients during atypical symptoms occurrence.The EEG of all 29 patients showed abundance of spike and waves（SW）in rolandic areas during wake-up and sleep.The SW index was 50%～85% during slow sleep in all cases.Video EEG monitored epileptic negative myoclonus（ENM）in 27 patients confirmed by outstretched arms test,atypical absences with secondary bilateral synchronous 2～3 Hz spike-wave rhythms during ictal EEG in 11 patients.Valproic acid only or combined with clonazepam,levetiracetam or lamotrigine were effective in 11 patients.The clinical symptoms and EEG SW were improved in 18 patients by adding corticosteroid therapy.Conclusion The clinical presentations of the variants of BECT include ENM,atypical absences,oral pharynx apraxia and cognitive deterioration.The clinical presentations are associated with EEG deterioration.Antiepileptic drugs therapy or combined therapy with corticosteroids is effective in improving electroclinical manifestations of the variants of BECT.