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[肌萎缩侧索硬化症的电诊断:实际应用]

[Electrodiagnosis of ALS: its practical aspects].

作者信息

Sonoo Masahiro, Higashihara Mana, Hokkoku Keiichi

机构信息

Department of Neurology, Teikyo University School of Medicine.

出版信息

Rinsho Shinkeigaku. 2011 Nov;51(11):1111-3. doi: 10.5692/clinicalneurol.51.1111.

Abstract

Electrodiagnostic methods, especially needle EMG plays an important role for the ALS diagnosis. Existing diagnostic criteria such as revised El Escorial criteria (R-EEC) and Awaji algorithm have a drawback of low sensitivity. Our study revealed that the percentage of patients classified as confirmed ALS, i.e. clinically probable (laboratory supported) or higher, was 43% using the R-EEC and 37% using the Awaji algorithm. Needle EMG can strongly suggest ALS beyond these criteria. Fasciculation potentials (FPs) are sufficiently specific for ALS, and we have argued its diagnostic utitlity in ALS diagnosis. FPs are rare in other neurogenic diseases, such as cervical or lumbar spine disorders and spinal and bulbar muscular atrophy. Profuse FPs observed in both upper and lower limbs would strongly suggest ALS. EMG of the upper trapezius muscle is useful since it is easily relaxed, and the spontaneous activities in this muscle are sufficiently sensitive and specific for ALS. Inclusion body myositis (IBM) might be confused with ALS, but its differentiation is actually easy since a normal recruitment in a chronically weak muscle definitely indicates myopathy. Furthermore, EMG of the flexor digitorum profundus muscle in IBM patients would reveal typical myopathic, i.e. low-amplitude and thin, motor unit potentials.

摘要

电诊断方法,尤其是针电极肌电图在肌萎缩侧索硬化症(ALS)的诊断中起着重要作用。现有的诊断标准,如修订的埃尔埃斯科里亚尔标准(R-EEC)和阿波岐算法,存在灵敏度低的缺点。我们的研究表明,使用R-EEC将患者分类为确诊ALS,即临床很可能(实验室支持)或更高的比例为43%,使用阿波岐算法的比例为37%。针电极肌电图可以在这些标准之外强烈提示ALS。肌束震颤电位(FPs)对ALS具有足够的特异性,我们已经论证了其在ALS诊断中的诊断效用。FPs在其他神经源性疾病中很少见,如颈椎或腰椎疾病以及脊髓性和延髓性肌萎缩。在上下肢均观察到大量FPs强烈提示ALS。斜方肌上部的肌电图很有用,因为它容易放松,并且该肌肉中的自发电活动对ALS具有足够的敏感性和特异性。包涵体肌炎(IBM)可能与ALS混淆,但实际上很容易区分,因为慢性无力肌肉中的正常募集肯定表明是肌病。此外,IBM患者的指深屈肌肌电图将显示典型的肌病性,即低振幅和细的运动单位电位。

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