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目的:观察先天性肌无力综合征(CMS)患者重复复合肌肉动作电位(R-CMAP)的特征,讨论其诊断意义。方法:采用低频和高频重复电刺激方法,记录肢体近端肌、远端肌和面肌的R-CMAP,观察其改变特点,并测量重复放电与主波的关系。结果:2例CMS中有特征性的R-CMAP以独立于主波和叠加于主波两种形式存在。独立的R-CMAP出现于肢体的近端肌和远端肌,与主波的时间间隔为9.2～13.4 ms;叠加的R-CMAP主要见于近端肌和面肌,与主波的潜伏期差为3.1～6.7 ms。两种R-CMAP在低频刺激时波幅均有明显的衰减。结论:R-CMAP是诊断CMS中胆碱酯酶缺乏症和慢通道综合征的重要依据。
Objective: To observe the characteristics of repeated compound muscle action potential (R-CMAP) in patients with congenital myasthenic syndrome (CMS) and to discuss its diagnostic significance. Methods: R-CMAP was recorded in the proximal, distal and facial muscles of the limbs by using low-frequency and high-frequency repetitive electrical stimulation. The changes of R-CMAP were observed and the relationship between repetitive discharge and the main wave was measured. Results: Two R-CMAPs characteristic of CMS existed in two forms independent of the main wave and superposed on the main wave. Independent R-CMAP appeared in the proximal and distal limb muscles, with the main wave of the time interval of 9.2 ~ 13.4 ms; superimposed R-CMAP mainly seen in the proximal muscles and facial muscles, and the main wave latency was 3.1 ~ 6.7 ms. Both R-CMAP significantly attenuated amplitude at low frequency stimulation. Conclusion: R-CMAP is an important basis for diagnosis of cholinesterase deficiency and chronic channel syndrome in CMS.