Research
Print page Print page
Switch language
The Capital Region of Denmark - a part of Copenhagen University Hospital
Published

Moderate-intensity aerobic exercise improves physical fitness in bethlem myopathy

Research output: Contribution to journalJournal articleResearchpeer-review

DOI

  1. Hydroxylated Long-Chain Acylcarnitines are Biomarkers of Mitochondrial Myopathy

    Research output: Contribution to journalJournal articleResearchpeer-review

  2. MSTO1 mutations cause mtDNA depletion, manifesting as muscular dystrophy with cerebellar involvement

    Research output: Contribution to journalJournal articleResearchpeer-review

  3. Growth and differentiation factor 15 as a biomarker for mitochondrial myopathy

    Research output: Contribution to journalJournal articleResearchpeer-review

  4. Fat oxidation is impaired during exercise in lipin-1 deficiency

    Research output: Contribution to journalJournal articleResearchpeer-review

View graph of relations

INTRODUCTION: Bethlem myopathy is caused by dysfunctional collagen VI assembly, leading to varying degrees of hyperlaxity, contractures and muscle weakness. Previous studies demonstrate that cardiovascular training is safe and beneficial in patients with myopathies. However, exercise exacerbates the dystrophic phenotype in collagen VI-knockout mice.

METHODS: Six men with Bethlem myopathy were included (4 training; 2 controls). After training, 2 patients detrained. Patients performed 10 weeks of home-based, moderate-intensity exercise monitored by a pulse-watch. The primary outcome was change in peak oxygen uptake (VO2peak ). Secondary outcomes were performances in functional tests.

RESULTS: VO2peak improved in the training group (16%, P = 0.017). Detraining led to regression of VO2peak toward baseline values (-8%; P = 0.03). No change was seen in the control group (-7%; P = 0.47). Performance in functional tests did not change significantly. Creatine kinase values were stable during the study.

CONCLUSIONS: Moderate-intensity exercise seems to safely improve oxidative function in patients with Bethlem myopathy. Muscle Nerve 60: 183-188, 2019.

Original languageEnglish
JournalMuscle & Nerve
Volume60
Issue number2
Pages (from-to)183-188
Number of pages6
ISSN0148-639X
DOIs
Publication statusPublished - Aug 2019

ID: 58148971