title: Training improves oxidative capacity, but not function, in spinal muscular atrophy type III.
abstract: BACKGROUND
  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.
authors.affiliation: Department of Neuromuscular Research Unit, Section 3342, Rigshospitalet and University of Copenhagen, Blegdamsvej 9, DK-2100 Copenhagen, Denmark.
authors.forename: Karen Lindhardt
authors.initials: KL
authors.lastname: Madsen
authors.affiliation: Department of Neuromuscular Research Unit, Section 3342, Rigshospitalet and University of Copenhagen, Blegdamsvej 9, DK-2100 Copenhagen, Denmark.
authors.forename: Regitze Sølling
authors.initials: RS
authors.lastname: Hansen
authors.affiliation: Department of Neuromuscular Research Unit, Section 3342, Rigshospitalet and University of Copenhagen, Blegdamsvej 9, DK-2100 Copenhagen, Denmark.
authors.forename: Nicolai
authors.initials: N
authors.lastname: Preisler
authors.affiliation: Department of Neuromuscular Research Unit, Section 3342, Rigshospitalet and University of Copenhagen, Blegdamsvej 9, DK-2100 Copenhagen, Denmark.
authors.forename: Frank
authors.initials: F
authors.lastname: Thøgersen
authors.affiliation: Department of Neuromuscular Research Unit, Section 3342, Rigshospitalet and University of Copenhagen, Blegdamsvej 9, DK-2100 Copenhagen, Denmark.
authors.forename: Martin Peter
authors.initials: MP
authors.lastname: Berthelsen
authors.affiliation: Department of Neuromuscular Research Unit, Section 3342, Rigshospitalet and University of Copenhagen, Blegdamsvej 9, DK-2100 Copenhagen, Denmark.
authors.forename: John
authors.initials: J
authors.lastname: Vissing
mesh_terms: D000328:Adult; D015444:Exercise / Q000502:physiology*; D005080:Exercise Test / Q000379:methods*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018763:Muscle Fatigue / Q000502:physiology; D053580:Muscle Strength / Q000502:physiology*; D010101:Oxygen Consumption / Q000502:physiology*; D014897:Spinal Muscular Atrophies of Childhood / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy*; D055815:Young Adult
keywords: SMA III; exercise; fatigue; motor neuron disease; neuromuscular disorders; spinal muscular atrophy type III; training