Dataset Information

Electrical impedance myography in individuals with collagen 6 and laminin ?-2 congenital muscular dystrophy: a cross-sectional and 2-year analysis.

ABSTRACT: INTRODUCTION:Electrical impedance myography (EIM) is a noninvasive electrophysiological technique that characterizes muscle properties through bioimpedance. We compared EIM measurements to function, strength, and disease severity in a population with congenital muscular dystrophy (CMD). METHODS:Forty-one patients with CMD, either collagen 6 related disorders (COL6-RD; n?=?21) or laminin ?-2-related disorders (LAMA2-RD; n?=?20), and 21 healthy pediatric controls underwent 2 yearly EIM exams. In the CMD cohorts, EIM was compared with functional and strength measurements. RESULTS:Both CMD cohorts exhibited change over time and had correlation with disease severity. The 50-kHZ phase correlated well with function and strength in the COL6-RD cohort but not in the LAMA2-RD cohort. DISCUSSION:EIM is a potentially useful measure in clinical studies with CMD because of its sensitivity to change over a 1-year period and correlation with disease severity. For COL6-RD, there were also functional and strength correlations. Muscle Nerve 57: 54-60, 2018.

SUBMITTER: Nichols C

PROVIDER: S-EPMC6383203 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature

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Electrical impedance myography in individuals with collagen 6 and laminin α-2 congenital muscular dystrophy: a cross-sectional and 2-year analysis.

Nichols Carmel C Jain Minal S MS Meilleur Katherine G KG Wu Tianxia T Collins James J Waite Melissa R MR Dastgir Jahannaz J Salman Anam A Donkervoort Sandra S Duong Tina T Keller Katherine K Leach Meganne E ME Lott Donovan J DJ McGuire Michelle N MN Nelson Leslie L Rutkowski Anne A Vuillerot Carole C Bönnemann Carsten G CG Lehky Tanya J TJ

Muscle & nerve 20170425 1

<h4>Introduction</h4>Electrical impedance myography (EIM) is a noninvasive electrophysiological technique that characterizes muscle properties through bioimpedance. We compared EIM measurements to function, strength, and disease severity in a population with congenital muscular dystrophy (CMD).<h4>Methods</h4>Forty-one patients with CMD, either collagen 6 related disorders (COL6-RD; n = 21) or laminin α-2-related disorders (LAMA2-RD; n = 20), and 21 healthy pediatric controls underwent 2 yearly ...[more]

PMID: 28224647

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Project description:Loss of muscle mass and strength represents one of the most significant contributors to impaired function in older adults. Convenient and non-invasive biomarkers are needed that can readily identify and track age-related muscle change. Previous data has suggested electrical impedance myography (EIM) has the potential to serve in this capacity. In this study we investigated how changes in EIM compared with other standard measures of muscle structure and function in aged compared with young mice. A total of 19 male mice aged approximately 25 months and 19 male mice aged 3 months underwent surface multifrequency EIM of the right gastrocnemius muscle using standard methods. Fore and hind limb grip strength, sciatic compound muscle action potential amplitude, and in-situ force of the gastrocnemius were also measured; after sacrifice, gastrocnemius myofiber size was assessed using standard histology. Spearman correlation coefficients were calculated to investigate the association between EIM and muscle characteristics. EIM in aged animals demonstrated significantly lower 50 kHz impedance phase (p<0.001) and reactance (p<0.01) values as well as reduced multifrequency parameters. In contrast, absolute gastrocnemius muscle mass was no different between young and aged mice (p = 0.58) but was reduced in aged mice after normalization to body mass (p<0.001). Median myofiber size in the aged mice was not different from that of young mice (p = 0.72). Aged mice showed reduced muscle function on the basis of normalized fore limb (p<0.001) and normalized hind limb (p<0.001) grip strength, as well as normalized gastrocnemius twitch (p<0.001) and normalized maximal isometric force (p<0.001). Sciatic compound muscle action potential amplitude was reduced in aged mice (p<0.05). EIM parameters showed good correlation with reduced standard physiological and electrophysiological measures of muscle health. Our study suggests that EIM is sensitive to aged-related muscle change and may represent a convenient and valuable method of quantifying loss of muscle health.

Dataset Information

Electrical impedance myography in individuals with collagen 6 and laminin ?-2 congenital muscular dystrophy: a cross-sectional and 2-year analysis.

Publications

Electrical impedance myography in individuals with collagen 6 and laminin α-2 congenital muscular dystrophy: a cross-sectional and 2-year analysis.

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