面肩肱型肌营养不良症。一项定量肌电图研究。

Department of Neurology, University of Pretoria, South Africa.

Electromyogr Clin Neurophysiol. 2007 May-Jun;47(3):175-82.

BACKGROUND

Quantitative electromyography (EMG) using different needle techniques has not been performed or reported on a relatively large group of patients with facioscapulohumeral muscular dystrophy (FSHD).

PURPOSE

To establish statistically: (1) correlations between clinical features of patients (age, disease duration and degree of weakness) and quantitative needle EMG/SFEMG,; (2) correlations between different EMG parameters in the patient group, and (3) quantitative EMG differences comparing patients with a healthy control group.

METHODS

Nerve conduction studies, and needle EMG (motorunit analysis, MacroEMG, SFEMG) were performed on Mm. triceps brachii and Mm. tibialis anterior according to standard techniques on 20 patients with FSHD.

RESULTS

Nerve conduction studies were normal. In Mm. triceps brachii and, to a lesser extent, Mm. tibialis anterior motorunit analysis and MacroEMG showed myopathic changes, that correlated with patient clinical parameters. In Mm. triceps brachii (but not in Mm. tibialis anterior) EMG results were statistically different in patients compared to control group data. The most sensitive indicators of a myopathy were MUP duration (motorunit analysis) and MUP area (MacroEMG). In the Mm. triceps brachii SFEMG revealed correlations between worsening pooled MCD data and patient clinical parameters. Pooled MCD results did not correlate with other MUP parameters. SFEMG showed abnormal jitter only in 2 patients with the longest disease duration.

CONCLUSION

Quantitative EMG results are compatible with a mild, slowly progressive myopathy. The most sensitive indicators of early muscle disease were MUP duration (motorunit analysis) and MUP area (MacroEMG) that would not be detected on "routine" EMG SFEMG showed subtle, progressive worsening of neuromuscular junction physiology. However, quantitative EMG and SFEMG showed that muscle fiber degeneration and loss followed a course independent of muscle fiber regeneration and reinnervation.

背景

尚未对相对大量的面肩肱型肌营养不良症（FSHD）患者群体进行或报道使用不同针电极技术的定量肌电图（EMG）研究。

目的

进行统计学分析以确定：（1）患者的临床特征（年龄、病程和肌无力程度）与定量针电极EMG/SFEMG之间的相关性；（2）患者组中不同EMG参数之间的相关性；（3）患者与健康对照组之间的定量EMG差异。

方法

按照标准技术，对20例FSHD患者的肱三头肌和胫前肌进行神经传导研究及针电极EMG（运动单位分析、巨肌电图、单纤维肌电图）检查。

结果

神经传导研究结果正常。在肱三头肌以及程度较轻的胫前肌中，运动单位分析和巨肌电图显示出肌病性改变，且与患者临床参数相关。在肱三头肌（而非胫前肌）中，患者的EMG结果与对照组数据相比存在统计学差异。肌病最敏感的指标是运动单位电位（MUP）时限（运动单位分析）和MUP面积（巨肌电图）。在肱三头肌中，单纤维肌电图显示综合MCD数据恶化与患者临床参数之间存在相关性。综合MCD结果与其他MUP参数无关。单纤维肌电图仅在病程最长的2例患者中显示出异常颤抖。