不典型儿童良性部分性癫痫患儿癫痫性负性肌阵挛的临床和神经电生理研究

Clinical and electrophysiologic studies on epileptic negative myoclonus in atypical benign partial epilepsy of childhood

目的探讨不典型儿童良性部分性癫痫（ABPE）患儿癫痫性负性肌阵挛（ENM）的临床和神经电生理特点及对治疗的反应。方法对1998年1月至2007年9月在北京大学第一医院儿科就诊的17例伴ENM的ABPE患儿在视频脑电图（VEEG）监测下行直立伸臂试验，9例同时监测双侧三角肌表面肌电图（EMG）。对ENM的临床、神经电生理特征及对抗癫痫药物（AED）的治疗反应进行分析。结果本组起病早期均符合儿童良性癫痫伴中央颞区棘波（BECT）的诊断标准，并已接受AED治疗。病程中出现手抖、掉物、点头、站立不稳等症状提示可能存在ENM。所有患儿均在VEEG监测时行直立伸臂试验并观察到ENM发作，与EEG对侧Rolandic区高波幅棘慢波发放同步出现。9例同时进行EMG记录，证实EEG的棘波与EMG的短暂肌电静息具有锁时关系。在出现ENM期间，原有的部分运动性发作频率及发作间期EEG放电明显增多，6例亦有不典型失神发作。部分患儿ENM的出现可能与加用卡马西平、奥卡西平、苯巴比妥等药物或突然减停丙戊酸有关。丙戊酸、苯二氮革类药物及肾上腺皮质激素以不同方式联合治疗可消除多数患儿的ENM发作。结论ABPE病程中可出现ENM。VEEG监测时行直立伸臂试验及同步EMG记录有助于确诊ENM。ENM的出现常伴有原有惊厥发作频率的增多及发作问期EEG的恶化。卡马西平等AED可能诱发ENM。丙戊酸、苯二氮革类药物及肾上腺皮质激素对消除ENM发作有效。

Objective To investigate the clinical, neurophysiologic characteristics and therapeutic considerations of epileptic negative myoclonus （ENM） in atypical benign partial epilepsy of childhood （ABPE）. Methods Video-EEG monitoring with outstretched arm tests were carried out in 17 patients, and 9 of them were examined with simultaneous electromyography （EMG）. The ENM manifestations, electrophysiologic features and responses to antiepileptic drugs （AED） were analyzed. Results Seventeen patients were diagnosed as having benign childhood epilepsy with centrotemporal spikes （BECT） during the early course of the disease and were treated with AED. During the course of the disease, hand trembling, objects dropping, head nodding and instability during standing might be clues for ENM occurrence. ENM had been confirmed in our patients by outstretched arm tests during video-EEG recording. The ictal EEG showed that high-amplitude spikes followed by a slow wave over the contralateral motor areas. This was further confirmed by time-locked silent EMG in 9 patients. During ENM occurrence or recurrence, the habitual seizures and interictal discharges were exaggerated. Atypical absence seizures also occurred in 6 patients. The alteration of therapeutic options of AED relating to ENM appearance in some patients included the add-on therapy with carbamazepine （ CBZ）, oxcarbazepine, phenobarbital, or withdrawal of valproate （VPA）. ENM was controlled in most cases by using VPA, clonazepam （CZP） and corticosteroid with different combination. Conclusion ENM could occur during the course of ABPE. Outstretching arm tests during video-EEG monitoring in combination with EMG was essential to confirm ENM. The ENM occurrence was always associated with the frequency increasing of habitual seizures and the aggravation of interictal discharges. Some AED such as CBZ might induce ENM. VPA, benzodiazepines and corticosteroid with different combination were relatively effective in treatment of ENM.