儿童眼睑肌阵挛持续状态的临床和脑电图特点

The clinical features and Video-EEG of Eyelid myoclonia-nonconvulsive status epilepticus in children

目的探讨儿童眼睑肌阵挛非惊厥性癫痫持续状态(Eyelid myoclonia-nonconvulsive status epilepticus,EM-NCSE)的临床和脑电图特点、治疗以及预后。方法收集2015年1月-2016年8月在山东大学齐鲁儿童医院神经科诊治的3例EM-NCSE患儿的临床和视频脑电图(VEEG)资料,以及对抗癫痫药物(AEDs)治疗反应进行观察分析。结果 3例患儿中女2例,男1例。发病年龄6~10岁,平均8.67岁。发作时临床表现为精神错乱、烦躁不安、挤眉弄眼、眼球频繁向上滚动等,VEEG示广泛性3~6 Hz棘慢综合波或多棘慢综合波,以前额、额、前颞为著,闪光刺激后可见患儿眼睑节律性眨动。结论 EM-NCSE由于其发作时症状复杂多样不易发现,常导致漏诊、误诊,详细询问病史并及时进行VEEG检查以及进行诊断性用药对确诊具有极其重要价值;地西泮能够有效改善发作时临床表现和VEEG特征,AEDs疗效较好。

Objective To study the clinical and EEG features,therapeutic response and prognosis of eyelid myoclonia-nonconvulsive status epilepticus( EM-NCSE) in children. Methods Collected the clinical and EEG data of 3 children with EM-NCSE that were diagnosed in department of neurology in Qilu Children Hospital of Shandong university during the January in 2015 to August in 2016. Analysed the therapeutic response to antiepletic drugs( AEDs). Results Among the three children,there were 2 girls and1 boy. The age at the onset of the disease was from 6 to 10 years old. The average age of them is 8. 67 years old. The clinical manifestations include mental confusion,dysphoria,winking and scrolling up the eyes. The typical vedio electroencephalography( VEEG) in the patients showed 3 ~ 6 Hz generalized spike and waves and polyspikes burst,especially in the frontal and the anterior temporal region. In addition,the eye closure and intermittent photic stimulation helped to induce discharges and clinical events as eyelid myoclonia( EM). Conclusions EM-NCSE is one of the idiopathic and generalized epileptic disease and characterized by EM. Video EEG monitoring plays an important role in the diagnosis of this disease. The drugs of choice for treatment was diazepam. When the event was controlled,AEDs were effective for the following therapy.