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Moderate‐intensity aerobic exercise improves physical fitness in bethlem myopathy
Author(s) -
Vissing Christoffer Rasmus,
Hedermann Gitte,
Vissing John
Publication year - 2019
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.26498
Subject(s) - medicine , myopathy , physical therapy , creatine , exercise intolerance , weakness , aerobic exercise , creatine kinase , cardiology , muscle weakness , physical medicine and rehabilitation , surgery , heart failure
ABSTRACT 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 (VO 2peak ). Secondary outcomes were performances in functional tests. Results VO 2peak improved in the training group (16%, P = 0.017). Detraining led to regression of VO 2peak 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