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

Carmel Nichols; Minal S. Jain; Katherine G. Meilleur; Tianxia Wu; James Collins; Melissa R. Waite; Jahannaz Dastgir; Anam Salman; Sandra Donkervoort; Tina Duong; Katherine Keller; Meganne E. Leach; Donovan J. Lott; Michelle N. McGuire; Leslie Nelson; Anne Rutkowski; Carole Vuillerot; Carsten G. Bönnemann; Tanya J. Lehky

doi:10.1002/mus.25629

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

Carmel Nichols, Minal S. Jain, Katherine G. Meilleur, Tianxia Wu, James Collins, Melissa R. Waite, Jahannaz Dastgir, Anam Salman, Sandra Donkervoort, Tina Duong, Katherine Keller, Meganne E. Leach, Donovan J. Lott, Michelle N. McGuire, Leslie Nelson, Anne Rutkowski, Carole Vuillerot, Carsten G. Bönnemann, Tanya J. Lehky

Physical Therapy

Research output: Contribution to journal › Article › peer-review

10 Scopus citations

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 α-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.

Original language	English (US)
Pages (from-to)	54-60
Number of pages	7
Journal	Muscle and Nerve
Volume	57
Issue number	1
DOIs	https://doi.org/10.1002/mus.25629
State	Published - Jan 2018

Keywords

COL6 related dystrophies
congenital muscular dystrophy
electrical impedance myography
LAMA2 related dystrophies
neuromuscular disorders

ASJC Scopus subject areas

Physiology
Clinical Neurology
Cellular and Molecular Neuroscience
Physiology (medical)

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Nichols, C., Jain, M. S., Meilleur, K. G., Wu, T., Collins, J., Waite, M. R., Dastgir, J., Salman, A., Donkervoort, S., Duong, T., Keller, K., Leach, M. E., Lott, D. J., McGuire, M. N., Nelson, L., Rutkowski, A., Vuillerot, C., Bönnemann, C. G., & Lehky, T. J. (2018). Electrical impedance myography in individuals with collagen 6 and laminin α-2 congenital muscular dystrophy: a cross-sectional and 2-year analysis. Muscle and Nerve, 57(1), 54-60. https://doi.org/10.1002/mus.25629

Nichols, C, Jain, MS, Meilleur, KG, Wu, T, Collins, J, Waite, MR, Dastgir, J, Salman, A, Donkervoort, S, Duong, T, Keller, K, Leach, ME, Lott, DJ, McGuire, MN, Nelson, L, Rutkowski, A, Vuillerot, C, Bönnemann, CG & Lehky, TJ 2018, 'Electrical impedance myography in individuals with collagen 6 and laminin α-2 congenital muscular dystrophy: a cross-sectional and 2-year analysis', Muscle and Nerve, vol. 57, no. 1, pp. 54-60. https://doi.org/10.1002/mus.25629

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title = "Electrical impedance myography in individuals with collagen 6 and laminin α-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 α-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.",

keywords = "COL6 related dystrophies, congenital muscular dystrophy, electrical impedance myography, LAMA2 related dystrophies, neuromuscular disorders",

author = "Carmel Nichols and Jain, {Minal S.} and Meilleur, {Katherine G.} and Tianxia Wu and James Collins and Waite, {Melissa R.} and Jahannaz Dastgir and Anam Salman and Sandra Donkervoort and Tina Duong and Katherine Keller and Leach, {Meganne E.} and Lott, {Donovan J.} and McGuire, {Michelle N.} and Leslie Nelson and Anne Rutkowski and Carole Vuillerot and B{\"o}nnemann, {Carsten G.} and Lehky, {Tanya J.}",

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doi = "10.1002/mus.25629",

language = "English (US)",

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AU - Nichols, Carmel

AU - Jain, Minal S.

AU - Meilleur, Katherine G.

AU - Wu, Tianxia

AU - Collins, James

AU - Waite, Melissa R.

AU - Dastgir, Jahannaz

AU - Salman, Anam

AU - Donkervoort, Sandra

AU - Duong, Tina

AU - Keller, Katherine

AU - Leach, Meganne E.

AU - Lott, Donovan J.

AU - McGuire, Michelle N.

AU - Nelson, Leslie

AU - Rutkowski, Anne

AU - Vuillerot, Carole

AU - Bönnemann, Carsten G.

AU - Lehky, Tanya J.

PY - 2018/1

Y1 - 2018/1

N2 - 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 α-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.

AB - 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 α-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.

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KW - LAMA2 related dystrophies

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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

Abstract

Keywords

ASJC Scopus subject areas

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