TY - JOUR
T1 - Endurance training improves fitness and strength in patients with Becker muscular dystrophy
AU - Sveen, Marie Louise
AU - Jeppesen, Tina D
AU - Hauerslev, Simon
AU - Køber, Lars
AU - Krag, Thomas O
AU - Vissing, John
N1 - Keywords: Activities of Daily Living; Adult; Bicycling; Biopsy; Creatine Kinase; Exercise Therapy; Humans; Lactic Acid; Male; Muscle Strength; Muscle, Skeletal; Muscular Dystrophy, Duchenne; Oxygen Consumption; Physical Endurance; Workload
PY - 2008
Y1 - 2008
N2 - 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 (VO(2max)) over 12 weeks, and six patients continued cycling for 1 year. VO(2max), 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 VO(2max) 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.
AB - 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 (VO(2max)) over 12 weeks, and six patients continued cycling for 1 year. VO(2max), 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 VO(2max) 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.
U2 - 10.1093/brain/awn189
DO - 10.1093/brain/awn189
M3 - Journal article
C2 - 18776212
SN - 0006-8950
VL - 131
SP - 2824
EP - 2831
JO - Brain
JF - Brain
IS - Pt 11
ER -