发作性睡病猝倒发作视频-脑电图-肌电图监测与分析

Ambulatory video-EEG-EMG monitoring and analysis during cataplexy in narcolepsy

目的通过对猝倒过程进行动态视频-脑电图-肌电图监测,全面解析猝倒发作动态演变的临床过程和脑电图特征。方法共6例伴典型猝倒发作的1型发作性睡病患者(其中2例为猝倒持续状态),行夜间多导睡眠图监测和日间多次睡眠潜伏期试验;通过情感刺激诱发猝倒发作,动态视频-脑电图-肌电图监测猝倒过程,分析特征性脑电图改变。结果共记录到6例患者的14个全面性猝倒发作事件。根据猝倒过程中临床表现和肌电图特征将猝倒发作分为4期,即诱发期、对抗期、无张力期和恢复期,其中对抗期初期脑电图可见阵发性高同步化θ节律,可能是猝倒发作的特征性脑电图改变。结论全面性猝倒发作分为4期,代表复杂而动态的临床和神经电生理学改变过程。阵发性高同步化θ节律可能是猝倒发作的重要机制。

Objective To comprehensively analyze the clinical and electroneurophysiological characteristics during the process of cataplexy by dynamic video？EEG？EMG monitoring. Methods Six narcolepsy type 1 patients with typical cataplexy were enrolled and 2 of them were diagnosed as status cataplecticus. All patients underwent polysomnography （PSG） and daytime Multiple Sleep Latency Test （MSLT） to clarify the diagnosis. Cataplexy was triggered by emotional stimulus and recorded under dynamic video-EEG-EMG monitoring. EEG characteristics during cataplexy were further compared and analyzed. Objective To comprehensively analyze the clinical and electroneurophysiological characteristics during the process of cataplexy by dynamic video-EEG-EMG monitoring. Methods Six narcolepsy type 1 patients with typical cataplexy were enrolled and 2 of them were diagnosed as status cataplecticus. All patients underwent polysomnography （PSG） and daytime Multiple Sleep Latency Test （MSLT） to clarify the diagnosis. Cataplexy was triggered by emotional stimulus and recorded under dynamic video-EEG-EMG monitoring. EEG characteristics during cataplexy were further compared and analyzed. Results Fourteen cataplectic attacks in 6 patients were recorded. According to the clinical and video- EMG characteristics, cataplectic attack was divided into 4 stages, including triggering phase （CA1）, resisting phase （CA2）, atonic phase （CA3） and recovering phase （CA4）. EEG frequency and amplitude varied from one stage to another and hypersynchronous paroxysmal theta （HSPT） was observed in early resisting phase （CA2）, which was supposed to be a distinctive EEG characteristic during the onset of cataplexy. Conclusions Generalized cataplectic ttack contain 4 stages, which indicate a complicated and dynamic process in clinical and electroneurophysiology. Moreover, it＇s highly possible that HSPT during resisting phase （CA2） is critical in the mechanism of cataplexy.