肌阵挛失神发作1例并文献复习

A sample of myoclonic absence and treatment effectiveness

目的：探讨肌阵挛失神发作的临床症状学、神经电生理学特点及治疗效果。方法：报告1例肌阵挛失神发作的临床表现、脑电图、肌电图特点及治疗效果，并结合文献进行回顾性分析。结果：肌阵挛失神发作临床表现为失神伴双侧节律性肌阵挛，常伴发肢体的强直，脑电图表现为双侧、广泛、节律性3Hz的棘慢复合波，肌电图则表现为与发作期放电频率一致的肌电暴发。此类患者对药物治疗反应较差，伴有强直发作的患者可行胼胝体切开术，该手术可有效减少强直发作导致的跌倒。结论：肌阵挛失神发作病程多样，大部分患者药物治疗反应差，伴有强直发作的患者可以考虑手术治疗。

Objective：To investigate the clinical and electrophysiological characteristics of myoclonic absence（MA） and treatment effectiveness. Methods： The characterastics of clinical manifestation, EEG, EMG and treatment effectiveness were analysed retrospectively. Results： MA is a specific type of seizures, and clinically characterized by the accompanion of diffuse rhythmical myoclonias, often associated with a progressive tonic contraction. The ietal EEG showed paroxysm of generalized spike and slow waves at 3 Hz, and in the polygraphic recording rhythmic 3 Hz myoclonus was observed with a strict correspondence between EEG spike and myoclonus. The response to drug treatment was generally weak,and the patiants with tonic seizures could prevent from falling down after corpus callosotomy. Conclusion： The prognosis of MA remains variable. From most of patients, seizures are resistant to drug treatment. Surgical treatment may be effective in patients with tonic seizures.