| 电生理诊断先天性肌无力综合征2例报道 |
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| 引用本文： | 乔凯,朱雯华,林洁,卢家红,吕传真.电生理诊断先天性肌无力综合征2例报道[J].中国临床神经科学,2008,16(5):499-503. |
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| 作者姓名： | 乔凯 朱雯华 林洁 卢家红 吕传真 |
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| 作者单位： | 复旦大学附属华山医院神经科,200040 |
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| 摘 要： | 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出现干肢体的近端肌和远端肌，与主波的时间间隔为9．2～13．4ms；叠加的R—CMAP主要见于近端肌和面肌，与主波的潜伏期差为3．1～6．7ms。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。
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| 关 键 词： | 先天性肌无力综合征 重复复合肌肉动作电位 乙酰胆碱酯酶缺乏症 慢通道综合征 |
Electrophysiological Studies of Two Cases of Congenital Myasthenic Syndrome |
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| QIAO Kai,ZHU Wen-Hua,LIN Jie,LU Jia-Hong,LV Chuan-Zhen.Electrophysiological Studies of Two Cases of Congenital Myasthenic Syndrome[J].Chinese Journal of Clinical Neurosciences,2008,16(5):499-503. |
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| Authors: | QIAO Kai ZHU Wen-Hua LIN Jie LU Jia-Hong LV Chuan-Zhen |
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| Institution: | QIAO Kai ZHU Wen- Hua LIN Jie LU Jia-Hong LV Chuan-Zhen Department of Neurology,Huashan Hospital,Fudan University,Shanghai 200040,China |
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| Abstract: | Aim:To observe the electrophysiological characteristics of repetitive compound muscle action potential(R-CMAP)in congenital myasthenic syndrome patients and discuss the significance in diagnosis. Methods:Repetitive stimulations of low and high frequency were used in proximal,distal and facial muscles. Changes of R-CMAP as well as the relationship between the main wave and R-CMAP were measured.Results: In 2 patients of congenital myasthenic syndrome,two types of R-CMAP were observed:one was separated from an... |
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| Keywords: | congenital myasthenic syndrome repetitive compound muscle action potential acetylcholinesterase deficiency slow channel syndrome |
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