Reduced specific force in patients with mild and severe facioscapulohumeral muscular dystrophy.

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.

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.

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 muscl

SUBMITTER: Lassche S 

PROVIDER: S-EPMC7821115 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature