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Muscle Nerve. 2015 Aug;52(2):240-4. doi: 10.1002/mus.24527. Epub 2015 May 23.

Training improves oxidative capacity, but not function, in spinal muscular atrophy type III.

Author information

1
Department of Neuromuscular Research Unit, Section 3342, Rigshospitalet and University of Copenhagen, Blegdamsvej 9, DK-2100 Copenhagen, Denmark.

Abstract

INTRODUCTION:

In this study we investigated the effect of 12 weeks of cycle ergometer training in patients with spinal muscular atrophy type III (SMA III), a hereditary motor neuron disease with progressive muscle weakness and atrophy.

METHODS:

Six SMA III patients and 9 healthy subjects completed a 12-week training program, performing 42 30-minute sessions exercising at 65-70% of maximal oxygen uptake (VO2max ). VO2max , muscle strength, functional tests, and self-reported activities of daily living were assessed before and after the training.

RESULTS:

Training induced a 27 ± 3% increase in VO2max (17 ± 2 to 21 ± 2 ml/kg/min, P < 0.001) in patients. However, fatigue was a major complaint and caused 1 patient to drop out, increased the need for sleep in 3 patients, and led to training modifications in 2 patients.

CONCLUSIONS:

Cycle exercise improves VO2max in SMA III without causing muscle damage, but it also induces significant fatigue. This warrants study into alternative training methods to improve exercise capacity in SMA III patients.

KEYWORDS:

SMA III; exercise; fatigue; motor neuron disease; neuromuscular disorders; spinal muscular atrophy type III; training

PMID:
25418505
DOI:
10.1002/mus.24527
[Indexed for MEDLINE]

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