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肌萎缩侧索硬化症亚临床异常的运动单位数量指数

The Motor Unit Number Index of Subclinical Abnormality in Amyotrophic Lateral Sclerosis.

作者信息

Fukada Kei, Matsui Toyoko, Furuta Mitsuru, Hirozawa Daisuke, Matsui Misa, Kajiyama Yuta, Shimizu Mikito, Kinoshita Makoto, Mochizuki Hideki, Sawada Jin-Ichi, Hazama Takanori

机构信息

*Department of Neurology, Osaka General Medical Center, Osaka, Japan; †Osaka College of High Technology, Osaka, Japan; ‡Department of Neurology, Osaka University Graduate School of Medicine, Osaka, Japan; §Department of Neurology, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan; and ‖Department of Neurology, Toneyama National Hospital, Osaka, Japan.

出版信息

J Clin Neurophysiol. 2016 Dec;33(6):564-568. doi: 10.1097/WNP.0000000000000296.

Abstract

PURPOSE

Diagnosis of amyotrophic lateral sclerosis (ALS) at an early stage is challenging, thus making the enrollment of these patients in clinical trials infeasible. In this study, we investigated the potential usability of motor unit number index (MUNIX) to detect denervation of clinically intact muscles of ALS patients.

METHODS

Thirty-two first dorsal interosseous muscles of 26 ALS patients were evaluated with both MUNIX and needle electromyography.

RESULTS

The mean MUNIX value of first dorsal interosseous muscles was 131 in the control group, whereas it was 48, 34, 15, and 8 for Medical Research Council scales of 5, 4, 3, and 2, respectively, in the ALS patients. The optimal cutoff point gave a sensitivity of 0.89 and a specificity of 1.0. Among 9 intact first dorsal interosseous muscles of the ALS patients, 8 showed MUNIX values below the cutoff point, whereas only 2 first dorsal interosseous muscles showed denervation on needle electromyography.

CONCLUSIONS

MUNIX could serve as a sensitive technique to detect denervation of clinically intact muscles of ALS patients.

摘要

目的

肌萎缩侧索硬化症(ALS)的早期诊断具有挑战性,这使得这些患者难以纳入临床试验。在本研究中,我们调查了运动单位数量指数(MUNIX)检测ALS患者临床完好肌肉失神经支配的潜在实用性。

方法

对26例ALS患者的32块第一背侧骨间肌进行了MUNIX和针极肌电图评估。

结果

对照组第一背侧骨间肌的平均MUNIX值为131,而在ALS患者中,医学研究委员会肌力分级为5、4、3和2级时,相应的MUNIX值分别为48、34、15和8。最佳截断点的灵敏度为0.89,特异性为1.0。在ALS患者的9块临床完好的第一背侧骨间肌中,8块的MUNIX值低于截断点,而针极肌电图显示只有2块第一背侧骨间肌存在失神经支配。

结论

MUNIX可作为检测ALS患者临床完好肌肉失神经支配的一种敏感技术。

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