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Updated: Aug 14, 2026

Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies
14:10

Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies

Published on: January 31, 2013

The heart in Becker muscular dystrophy, facioscapulohumeral dystrophy, and Bethlem myopathy

M de Visser1, W G de Voogt, G V la Rivière

  • 1Department of Neurology, University of Amsterdam, The Netherlands.

Muscle & Nerve
|May 1, 1992
PubMed

Insights

Becker muscular dystrophy (BMD) can cause heart issues, with 45% of patients showing EKG changes. Facioscapulohumeral (FSH) dystrophy and Bethlem myopathy showed minimal cardiac involvement in this study.

Area of Science:

  • Cardiology
  • Neurology
  • Genetics

Background:

  • Familial cardiomyopathies are a concern in muscular dystrophies.
  • Becker muscular dystrophy (BMD), facioscapulohumeral (FSH) dystrophy, and Bethlem myopathy are inherited neuromuscular disorders.
  • Cardiac involvement can significantly impact prognosis in muscular dystrophies.

Purpose of the Study:

  • To assess cardiac involvement in patients with familial Becker muscular dystrophy (BMD), facioscapulohumeral (FSH) dystrophy, and Bethlem myopathy.
  • To investigate correlations between myocardial involvement and clinical parameters in BMD.
  • To characterize the cardiac phenotype in these muscular dystrophy subtypes.

Main Methods:

  • Physical examination, chest X-ray, electrocardiography (EKG), and echocardiography were performed on all 91 patients.
  • Continuous EKG Holter monitoring was conducted for patients with FSH dystrophy.
  • Data analysis focused on identifying cardiac abnormalities and their associations.

Main Results:

  • Thirteen out of 33 (45%) patients with BMD exhibited EKG changes similar to those in Duchenne muscular dystrophy.
  • Cardiac involvement in BMD patients was not strongly correlated with wheelchair dependency.
  • No significant cardiac changes were detected in the 31 patients with FSH dystrophy; one patient with Bethlem myopathy showed hypertrophic cardiomyopathy.

Conclusions:

  • Becker muscular dystrophy (BMD) frequently presents with cardiac abnormalities, including EKG changes, irrespective of disease severity.
  • Facioscapulohumeral (FSH) dystrophy appears to have a low risk of cardiac involvement.
  • Bethlem myopathy shows a low incidence of cardiomyopathy, with only one case identified.

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