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From the Neuromuscular Research Unit, Department of Neurology and Copenhagen Muscle Research Center (N.P., G.A., F.T., T.D.J., J.V.), Department of Clinical Neurophysiology (C.C.), and Department of Clinical Genetics (F.W.), University of Copenhagen, Rigshospitalet, Denmark.
Address correspondence and reprint requests to Dr. Nicolai Preisler, Neuromuscular Research Unit 3342, University of Copenhagen, Rigshospitalet, Blegdamsvej 9, DK-2100 Copenhagen, Denmark npreisler{at}hotmail.com
Objective: We examined the effect of aerobic exercise in patients with spinal and bulbar muscular atrophy (SBMA). SBMA is caused by a defect androgen receptor. This defect causes motor neuron death, but considering the important function of androgens in muscle, it is possible that muscle damage in SBMA also occurs independently of motor neuron damage.
Methods: Eight patients with SBMA engaged in regular cycling exercise for 12 weeks. Maximum oxygen uptake (Vo2max), maximal work capacity (Wmax), muscle morphology, citrate synthase (CS) activity, body composition, EMG, static strength measurements, lung function, plasma proteins, and hormones were evaluated before and after training. Evaluation of improvements in activities of daily living (ADL) was conducted after training.
Results: Wmax increased by 18%, and CS activity increased by 35%. There was no significant change in Vo2max or any of the other variables examined before and after training, and the patients with SBMA did not feel improvements in ADL.
Conclusions: Frequent, moderate-intensity aerobic conditioning is of little beneficial effect in patients with spinal and bulbar muscular atrophy (SBMA). High levels of plasma creatine kinase and muscle regeneration indicate a primary myopathic affection, which, in parallel with the motor neuron deficiency, may attenuate the response to exercise training in patients with SBMA.
Abbreviations: ADL = activities of daily living; AR = androgen receptor; ASI = androgen sensitivity index; BMI = body mass index; CK = creatine kinase; CS = citrate synthase; FSH = follicle-stimulating hormone; LH = luteinizing hormone; MU = motor unit; SBMA = spinal and bulbar muscular atrophy; Vo2max = maximum oxygen uptake; Wmax = maximal work capacity.
Supplemental data at www.neurology.org
Supported by grants from the Sara and Ludvig Elsass Foundation, The Danish Medical Research Council, the NOVO Nordic Foundation, and the Copenhagen Hospital Community Foundation.
Disclosure: The authors report no disclosures.
Received June 16, 2008. Accepted in final form October 15, 2008.
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