Cortical myoclonus in Janz syndrome
Cortical myoclonus in Janz syndrome
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DOI：
10.1016/s1388-2457(01)00634-4
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发表时间：
2001-10-01
影响因子：
4.7
通讯作者：
Tassinari, CA
中科院分区：
文献类型：
--
作者：
Panzica, F;Rubboli, G;Tassinari, CA
目的：探讨青少年型肌阵挛性癫痫（JME）患者脑电图发作特征及脑电图峰与发作性肌阵挛性抽搐的关系。方法：6例典型的JME患者进入研究，并进行计算机测谎记录。在每个患者中，在重复的脑电图爆发中测量峰间尖峰间隔，并使用一个时间窗口（包括抽搐相关肌电电位偏离基线点前100毫秒和后100-200秒）对关键时期进行抽搐锁定回平均。结果：在所有病例中，肌阵挛性抽搐均与多刺波（PSW）复合物有关。在PSW复合体内重复尖峰的频率范围从16到27赫兹。抽搐锁定平均显示脑电瞬态为正阴性，最大振幅位于额叶导联，比肌阵挛性抽搐早10.25±0.96 ins。在两脑额叶脑电图导联上检测到的抽搐锁定阳性峰值之间存在9.50 +/- 1.73 in的延迟；在两个三角肌的肌阵挛痉挛发作之间观察到相当的时间滞后。结论：我们的数据表明，尽管有不同的致病底物和触发机制，但JME发作性肌阵挛痉挛的最终机制与进行性肌阵挛癫痫等更严重的病理条件下持续的皮质肌阵挛在很大程度上相似。(C) 2001爱思唯尔科学爱尔兰有限公司版权所有。
Objective: To evaluate the characteristics of EEG paroxysms and the relationship between EEG spikes and ictal myoclonic jerks in patients with juvenile myoclonic epilepsy (JME).Methods: Six patients with a typical form of JME entered the study and underwent computerized polygraphic recordings. In each patient, the inter-peak spike interval was measured on repeated EEG bursts, and jerk-locked back averaging was performed on ictal epochs using a time window including the 100 ms before and the 100-200 ins after the point at which the jerk-related EMG potential diverged from baseline.Results: In all cases, the myoclonic jerks were associated with polyspike waves (PSW) complexes. The frequency of repeated spikes within the PSW complex ranged from 16 to 27 Hz. Jerk-locked averaging revealed a positive-negative EEG transient with maximal amplitude on the frontal leads, which preceded the myoclonic jerk by 10.25 +/- 0.96 ins. A delay of 9.50 +/- 1.73 ins was measured between the jerk-locked positive peak detected on the frontal EEG leads of the two hemispheres; a comparable time lag was observed between the onset of myoclonic jerks in the two deltoid muscles.Conclusions: Our data suggest that the ultimate mechanism responsible for ictal myoclonic jerks in JME is largely similar to that sustaining cortical myoclonus in more severe pathological conditions such as progressive myoclonus epilepsies, despite the different pathogenic substrate and triggering mechanisms. (C) 2001 Elsevier Science Ireland Ltd. All rights reserved.