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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
Author(s) -
Nichols Carmel,
Jain Minal S.,
Meilleur Katherine G.,
Wu Tianxia,
Collins James,
Waite Melissa R.,
Dastgir Jahannaz,
Salman Anam,
Donkervoort Sandra,
Duong Tina,
Keller Katherine,
Leach Meganne E.,
Lott Donovan J.,
McGuire Michelle N.,
Nelson Leslie,
Rutkowski Anne,
Vuillerot Carole,
Bönnemann Carsten G.,
Lehky Tanya J.
Publication year - 2018
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.25629
Subject(s) - congenital muscular dystrophy , electrical impedance myography , medicine , cohort , muscular dystrophy , duchenne muscular dystrophy , collagen vi , physical therapy , physical medicine and rehabilitation , cardiology , extracellular matrix , biology , vasodilation , microbiology and biotechnology
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‐kH Z 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