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

TY - JOUR

T1 - Training improves oxidative capacity, but not function, in spinal muscular atrophy type III

AU - Madsen, Karen Lindhardt

AU - Hansen, Regitze Sølling

AU - Preisler, Nicolai

AU - Thøgersen, Frank

AU - Berthelsen, Martin Peter

AU - Vissing, John

N1 - © 2014 Wiley Periodicals, Inc.

PY - 2015/8/1

Y1 - 2015/8/1

N2 - 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.

AB - 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.

KW - Adult

KW - Exercise

KW - Exercise Test

KW - Female

KW - Humans

KW - Male

KW - Middle Aged

KW - Muscle Fatigue

KW - Muscle Strength

KW - Oxygen Consumption

KW - Spinal Muscular Atrophies of Childhood

KW - Young Adult

U2 - 10.1002/mus.24527

DO - 10.1002/mus.24527

M3 - Journal article

C2 - 25418505

SN - 0148-639X

VL - 52

SP - 240

EP - 244

JO - Muscle & Nerve

JF - Muscle & Nerve

IS - 2

ER -