Back to Search
Start Over
Reduced specific force in patients with mild and severe facioscapulohumeral muscular dystrophy.
- Source :
-
Muscle & nerve [Muscle Nerve] 2021 Jan; Vol. 63 (1), pp. 60-67. Date of Electronic Publication: 2020 Oct 15. - Publication Year :
- 2021
-
Abstract
- Background: Specific force, that is the amount of force generated per unit of muscle tissue, is reduced in patients with facioscapulohumeral muscular dystrophy (FSHD). The causes of reduced specific force and its relation with FSHD disease severity are unknown.<br />Methods: Quantitative muscle magnetic resonance imaging (MRI), measurement of voluntary maximum force generation and quadriceps force-frequency relationship, and vastus lateralis muscle biopsies were performed in 12 genetically confirmed patients with FSHD and 12 controls.<br />Results: Specific force was reduced by ~33% in all FSHD patients independent of disease severity. Quadriceps force-frequency relationship shifted to the right in severe FSHD compared to controls. Fiber type distribution in vastus lateralis muscle biopsies did not differ between groups.<br />Conclusions: Reduced quadriceps specific force is present in all FSHD patients regardless of disease severity or fatty infiltration. Early myopathic changes, including fibrosis, and non-muscle factors, such as physical fatigue and musculoskeletal pain, may contribute to reduced specific force.<br /> (© 2020 The Authors. Muscle & Nerve published by Wiley Periodicals LLC.)
- Subjects :
- Adult
Female
Fibrosis complications
Fibrosis physiopathology
Humans
Magnetic Resonance Imaging methods
Male
Middle Aged
Muscle, Skeletal physiopathology
Muscular Dystrophy, Facioscapulohumeral complications
Musculoskeletal Pain complications
Musculoskeletal Pain physiopathology
Quadriceps Muscle physiopathology
Young Adult
Muscle, Skeletal pathology
Muscular Dystrophy, Facioscapulohumeral pathology
Muscular Dystrophy, Facioscapulohumeral physiopathology
Quadriceps Muscle pathology
Severity of Illness Index
Subjects
Details
- Language :
- English
- ISSN :
- 1097-4598
- Volume :
- 63
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Muscle & nerve
- Publication Type :
- Academic Journal
- Accession number :
- 32959362
- Full Text :
- https://doi.org/10.1002/mus.27074