Training improves oxidative capacity, but not function in Spinal Muscular Atrophy Type III

Introduction 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. Discussion Cycle exercise improves VO2max in SMA III without causing muscle damage, but it also induces significant fatigue. This warrants investigations into alternative training methods to improve exercise capacity in SMA III patients. This article is protected by copyright. All rights reserved.