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[三种肌强直形式的肌电图特征]

[Electromyographic features of 3 forms of myotonia].

作者信息

Luk'ianov M V, Chuchin M Iu

出版信息

Zh Nevropatol Psikhiatr Im S S Korsakova. 1982;82(11):37-44.

PMID:7180301
Abstract

On the basis of a great body of examination data the electromyographic peculiarities of three forms of myotonia are analyzed. As a result, the authors come to a conclusion that in the third form of myotonia the function of muscular fibres is grossly impaired, this impairment following the pattern of their being put out of activity. This failure appears invariably during the muscle activity and leads, in the end to the clinically observed transient, and in more advanced cases, moderately permanent weakness of the muscles and their hypotrophy. In addition, data on synaptic apparatus involvement were also obtained. The time course of the development of those disorders, as regards the neuromuscular conduction and the distal parts of the muscular fibres is followed. In atrophic myotonia, certain peculiarities of the electrophysiological characteristics of the neuromuscular periphery were noted. In particular, a marked phase of secondary refractoriness was revealed. Thomsen's myotonia was found to differ substantially in the electrophysiological characteristics from the above two forms of the myotonia. Although in a number of cases it is difficult to class a particular case with one or another form of myotonia (this can be explained by an exceedingly great diversity of the biochemical peculiarities inherited by each individual) the differentiation of the above three forms of myotonia is justified from the viewpoint of both exploring the pathogenesis of the disease forms, and choosing the treatment method the most effective in this particular case. Of importance in solving these problems may be diversified electrophysiological examinations of the motor apparatus, and comparisons of their results with those of clinical examinations.

摘要

基于大量的检查数据,对三种肌强直形式的肌电图特征进行了分析。结果,作者得出结论,在第三种肌强直形式中,肌纤维功能严重受损,这种损伤遵循其失去活性的模式。这种功能障碍在肌肉活动期间总是出现,最终导致临床上观察到的短暂性,在更严重的情况下,肌肉出现中度永久性无力及其萎缩。此外,还获得了有关突触装置受累的数据。跟踪了这些疾病在神经肌肉传导和肌纤维远端部分发展的时间进程。在萎缩性肌强直中,注意到神经肌肉外周电生理特征的某些特点。特别是,发现了明显的继发性不应期阶段。发现托姆森肌强直在电生理特征上与上述两种肌强直形式有很大不同。尽管在许多情况下,很难将特定病例归类为某一种或另一种肌强直形式(这可以用每个个体遗传的生化特性极其多样来解释),但从探索疾病形式的发病机制以及选择在该特定病例中最有效的治疗方法的角度来看,区分上述三种肌强直形式是合理的。在解决这些问题时,运动装置的多样化电生理检查以及将其结果与临床检查结果进行比较可能很重要。

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