Specific muscle strength is reduced in facioscapulohumeral dystrophy: An MRI based musculoskeletal analysis

Marco A. Marra* (Corresponding Author), Linda Heskamp, Karlien Mul, Saskia Lassche, Baziel G.M. van Engelen, Arend Heerschap, Nico Verdonschot

*Corresponding author for this work

    Research output: Contribution to journalArticleAcademicpeer-review

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    Abstract

    The aim was to test whether strength per unit of muscle area (specific muscle strength) is affected in facioscapulohumeral dystrophy (FSHD) patients, as compared to healthy controls. Ten patients and ten healthy volunteers underwent an MRI examination and maximum voluntary isometric contraction measurements (MVICs) of the quadriceps muscles. Contractile muscle volume, as obtained from the MR images, was combined with the MVICs to calculate the physiological cross-sectional area (PCSA) and muscle strength using a musculoskeletal model. Subsequently, specific strength was calculated for each subject as muscle strength divided by total PCSA. FSHD patients had a reduced quadriceps muscle strength (median(1st quartile-3rd quartile): 2011 (905.4-2775) N vs. 5510 (4727-8321) N, p <0.001) and total PCSA (83.6 (62.3-124.8) cm2 vs. 140.1(97.1-189.9) cm2, p = 0.015) compared to healthy controls. Furthermore, the specific strength of the quadriceps was significantly lower in patients compared to healthy controls (20.9 (14.7-24.0) N/cm2 vs. 41.9 (38.3-49.0) N/cm2, p <0.001). Thus, even when correcting for atrophy and fatty infiltration, patients with FSHD generated less force per unit area of residual muscle tissue than healthy controls. Possible explanations include impaired force propagation due to fatty infiltration, reduced intrinsic force-generating capacity of the muscle fibers, or mitochondrial abnormalities leading to impaired energy metabolism.

    Original languageEnglish
    Pages (from-to)238-245
    Number of pages8
    JournalNeuromuscular disorders
    Volume28
    Issue number3
    DOIs
    Publication statusPublished - 1 Mar 2018

    Keywords

    • UT-Hybrid-D
    • Intrinsic weakness
    • Musculoskeletal modeling
    • Quantitative MRI
    • Specific strength
    • Facioscapulohumeral muscular dystrophy

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