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

Lookup NU author(s): Dr Linda Heskamp

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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) cm2vs. 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/cm2vs. 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.


Publication metadata

Author(s): Marra MA, Heskamp L, Mul K, Lassche S, van Engelen BGM, Heerschap A, Verdonschot N

Publication type: Article

Publication status: Published

Journal: Neuromuscular Disorders

Year: 2018

Volume: 28

Issue: 3

Pages: 238-245

Print publication date: 01/03/2018

Online publication date: 12/12/2017

Acceptance date: 26/11/2017

ISSN (print): 0960-8966

ISSN (electronic): 1873-2364

Publisher: Elsevier

URL: https://doi.org/10.1016/j.nmd.2017.11.017

DOI: 10.1016/j.nmd.2017.11.017


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