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重症肌无力的临床电生理学

Clinical electrophysiology in myasthenia gravis.

作者信息

Stalberg E

出版信息

J Neurol Neurosurg Psychiatry. 1980 Jul;43(7):622-33. doi: 10.1136/jnnp.43.7.622.

Abstract

Effective diagnostic methods are of great importance in order to recognise myasthenic patients among those with muscle fatigability. Intracellular recordings are useful for research work within the field and for detailed description of the motor end-plate's physiology in the individual case. The method is not used for the routine diagnosis of myasthenia gravis. The decrement of the electrical muscle response with nerve stimulation is the most commonly used method. The diagnostic yield is higher in proximal muscles, in warmed muscles, after exercise, and after ischaemia. A significant number of patients may be undiagnosed with this technique. The mechanical response with nerve stimulation shows the same type of decrement but also an abnormal response to long stimulation. The diagnostic value of this is under dispute. Single fibre ENG needs more patient cooperation than do these tests. The diagnostic yield is significantly higher. Some patients considered to have myasthenia gravis do not show any abnormalities with this technique, particularly those with the pure ocular form. Conventional EMG is not useful for the diagnosis of myasthenia, but may be indicated in these patients when concurrent nerve or muscle disease is in question. Tests for eye movement fatique have not proved useful. Stapedius reflex fatigability is demonstrated in about the same proportion of patients as have positive SFEMG findings. The technique is not uncomfortable for the patient and requires minimal cooperation. The general usefulness must be assessed by further routine use. Even with the advent of immunological tests, neurophysiological investigations are indispensable in helping establish the diagnosis of myasthenia gravis. Discrepancies between the results comparing electrophysiological and immunological tests may indicate that myasthenia gravis is a heterogenous entity within which subgroups may be identified.

摘要

为了在有肌肉疲劳症状的患者中识别出重症肌无力患者,有效的诊断方法至关重要。细胞内记录对于该领域的研究工作以及个别病例中运动终板生理学的详细描述很有用。该方法不用于重症肌无力的常规诊断。神经刺激时肌肉电反应的递减是最常用的方法。在近端肌肉、温热的肌肉、运动后以及缺血后,诊断阳性率更高。相当一部分患者可能无法通过这种技术被诊断出来。神经刺激时的机械反应显示出相同类型的递减,但对长时间刺激也有异常反应。其诊断价值存在争议。单纤维肌电图比这些测试需要患者更多的配合。诊断阳性率显著更高。一些被认为患有重症肌无力的患者用这种技术并未显示出任何异常,尤其是那些纯眼肌型患者。传统肌电图对重症肌无力的诊断无用,但当同时存在神经或肌肉疾病问题时,可能对这些患者有诊断意义。眼球运动疲劳测试尚未证明有用。镫骨肌反射疲劳在大约相同比例的患者中得到证实,与单纤维肌电图阳性结果的患者比例相同。该技术对患者来说并不难受,且所需配合极少。其总体实用性必须通过进一步的常规应用来评估。即使有了免疫学检测,神经生理学检查在帮助确诊重症肌无力方面仍然不可或缺。电生理测试和免疫学测试结果之间的差异可能表明重症肌无力是一个异质性实体,其中可能存在亚组。

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