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.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Schwartz DP,Dastgir J,Salman A,Lear B,Bönnemann CG,Lehky TJdoi
10.1002/mus.24770subject
Has Abstractpub_date
2016-03-01 00:00:00pages
402-6issue
3eissn
0148-639Xissn
1097-4598journal_volume
53pub_type
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