电阻抗肌电图可将先天性肌营养不良与对照组区分开来。

Electrical impedance myography discriminates congenital muscular dystrophy from controls.

作者信息

Schwartz Daniel P, Dastgir Jahannaz, Salman Anam, Lear Barbara, Bönnemann Carsten G, Lehky Tanya J

机构信息

Electromyography Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10, CRC Room 7SW-5680, 10 Center Drive MSC 1404, Bethesda, Maryland, 20892, USA.

Neuromuscular and Neurogenetic Disorders of Childhood Section, NINDS, Bethesda, MD.

出版信息

Muscle Nerve. 2016 Mar;53(3):402-6. doi: 10.1002/mus.24770. Epub 2015 Aug 13.

DOI:10.1002/mus.24770

PMID:26179210

Abstract

INTRODUCTION

Electrical impedance myography (EIM) is an emerging non-invasive, highly reproducible electrophysiological technique that objectively characterizes muscle structure and composition by measuring bioimpedance. We assessed the ability of EIM ability to discriminate 2 forms of congenital muscular dystrophy (CMD), laminin α2 (LAMA2)-deficient CMD and collagen VI-deficient (COL6) CMD, from a group of healthy children. We also investigated correlations between subcutaneous fat thickness and EIM parameters.

METHODS

Twenty-eight children with LAMA2 CMD (n = 12) or COL6 (n = 16) CMD and 18 normal children underwent EIM testing.

RESULTS

The EIM 50-kHz phase was decreased in LAMA2 and COL6 CMD when compared with controls (P < 0.001). Reactance, however, was decreased in COL6 but not LAMA2 CMD compared with controls (P < 0.001).

CONCLUSIONS

Our findings suggest that EIM may be useful in discriminating CMD from controls and may serve as a useful biomarker to follow disease progression in clinical trials.

摘要

引言

电阻抗肌描记术（EIM）是一种新兴的非侵入性、高度可重复的电生理技术，通过测量生物阻抗来客观地表征肌肉结构和组成。我们评估了EIM区分2种先天性肌营养不良（CMD），即层粘连蛋白α2（LAMA2）缺陷型CMD和胶原蛋白VI缺陷（COL6）型CMD与一组健康儿童的能力。我们还研究了皮下脂肪厚度与EIM参数之间的相关性。