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January 26, 2009

Effect of aerobic training in patients with spinal and bulbar muscular atrophy (Kennedy disease)

January 27, 2009 issue
72 (4) 317-323

Abstract

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.

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Information & Authors

Information

Published In

Neurology®
Volume 72Number 4January 27, 2009
Pages: 317-323
PubMed: 19171827

Publication History

Published online: January 26, 2009
Published in issue: January 27, 2009

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Authors

Affiliations & Disclosures

N. Preisler, MD
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.
G. Andersen, BSc
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.
F. Thøgersen, BSc
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.
C. Crone, MD, PhD
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.
T. D. Jeppesen, MD
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.
F. Wibrand, PhD
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.
J. Vissing, MD, PhD
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.

Notes

Address correspondence and reprint requests to Dr. Nicolai Preisler, Neuromuscular Research Unit 3342, University of Copenhagen, Rigshospitalet, Blegdamsvej 9, DK-2100 Copenhagen, Denmark [email protected]

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  8. The French national protocol for Kennedy’s disease (SBMA): consensus diagnostic and management recommendations, Orphanet Journal of Rare Diseases, 15, 1, (2020).https://doi.org/10.1186/s13023-020-01366-z
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