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

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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

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Muscle Strength
Muscles
Isometric Contraction
Quadriceps Muscle
Energy Metabolism
Atrophy
Healthy Volunteers

Keywords

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

Cite this

Marra, Marco A. ; Heskamp, Linda ; Mul, Karlien ; Lassche, Saskia ; van Engelen, Baziel G.M. ; Heerschap, Arend ; Verdonschot, Nico. / Specific muscle strength is reduced in facioscapulohumeral dystrophy : An MRI based musculoskeletal analysis. In: Neuromuscular disorders. 2018 ; Vol. 28, No. 3. pp. 238-245.
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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.",
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Specific muscle strength is reduced in facioscapulohumeral dystrophy : An MRI based musculoskeletal analysis. / Marra, Marco A. (Corresponding Author); Heskamp, Linda; Mul, Karlien; Lassche, Saskia; van Engelen, Baziel G.M.; Heerschap, Arend; Verdonschot, Nico.

In: Neuromuscular disorders, Vol. 28, No. 3, 01.03.2018, p. 238-245.

Research output: Contribution to journalArticleAcademicpeer-review

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