期刊
MUSCLE & NERVE
卷 61, 期 5, 页码 644-649出版社
WILEY
DOI: 10.1002/mus.26792
关键词
facioscapulohumeral muscular dystrophy; genetics; muscle disease; natural history studies; neuromuscular disease; outcome measures
资金
- FSH Society
- Heartland Institute for Clinical and Translational Research [UL1TR000001]
- National Institutes of Health [KL2TR000119, S10 RR29577]
- Paul D. Wellstone Muscular Dystrophy Cooperative Research Centers [U54 AR065139]
Introduction Electrical impedance myography (EIM) has been proposed as a noninvasive biomarker of muscle composition in facioscapulohumeral muscular dystrophy (FSHD). Here we determine the associations of EIM variables with muscle structure measured by MRI. Methods We evaluated 20 patients with FSHD at two centers, comparing EIM measurements (resistance, reactance, and phase at 50, 100, and 211 kHZ) recorded from bilateral vastus lateralis, tibialis anterior, and medial gastrocnemius muscles to MRI skin and subcutaneous fat thickness, MRI T1-based muscle severity score (T1 muscle score), and MRI quantitative intramuscular Dixon fat fraction (FF). Results While reactance and phase both correlated with FF and T1 muscle score, 50 kHz reactance was most sensitive to muscle structure alterations measured by both T1 score (rho = -0.71, P < .001) and FF (rho = -0.74, P < .001). Discussion This study establishes the correlation of EIM with structural MRI features in FSHD and supports further evaluation of EIM as a potential biomarker in FSHD clinical trials.
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