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Abstract

Studies in a dystrophinopathy model (the mdx mouse) suggest that exercise training may be deleterious for muscle integrity, but exercise has never been studied in detail in humans with defects of dystrophin. We studied the effect of endurance training on conditioning in patients with the dystrophinopathy, Becker muscular dystrophy (BMD). Eleven patients with BMD and seven matched, healthy subjects cycled 50, 30 min sessions at 65% of their maximal oxygen uptake (VO2max) over 12 weeks, and six patients continued cycling for 1 year. VO2max, muscle biopsies, echocardiography, plasma creatine kinase (CK), lower extremity muscle strength and self-reported questionnaires were evaluated before, after 12 weeks and 1 year of training. Endurance training for 12 weeks, improved VO2max by 47 ± 11% and maximal workload by 80 ± 19% in patients (P < 0.005). This was significantly higher than in healthy subjects (16 ± 2% and 17 ± 2%). CK levels did not increase with training, and number of central nuclei, necrotic fibres and fibres expressing neonatal myosin heavy chain did not change in muscle biopsies. Strength in muscles involved in cycle exercise (knee extension, and dorsi- and plantar-flexion) increased significantly by 13–40%. Cardiac pump function, measured by echocardiography, did not change with training. All improvements and safety markers were maintained after 1 year of training. Endurance training is a safe method to increase exercise performance and daily function in patients with BMD. The findings support an active approach to rehabilitation of patients with BMD.

muscular dystrophy, Becker muscular dystrophy, aerobic exercise
© The Author (2008). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: [email protected]
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