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Training improves oxidative capacity, but not function, in spinal muscular atrophy type III
Author(s) -
Madsen Karen Lindhardt,
Hansen Regitze Sølling,
Preisler Nicolai,
Thøgersen Frank,
Berthelsen Martin Peter,
Vissing John
Publication year - 2015
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.24527
Subject(s) - spinal muscular atrophy , medicine , physical medicine and rehabilitation , atrophy , physical therapy , pathology , disease
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 (V O 2max ). V O 2max , 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 V O 2max (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 V O 2max 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. Muscle Nerve 52 : 240–244, 2015