title: Resistance strength training exercise in children with spinal muscular atrophy.
abstract: BACKGROUND
  Preliminary evidence in adults with spinal muscular atrophy (SMA) and in SMA animal models suggests exercise has potential benefits in improving or stabilizing muscle strength and motor function.
  
  
  METHODS
  We evaluated feasibility, safety, and effects on strength and motor function of a home-based, supervised progressive resistance strength training exercise program in children with SMA types II and III. Up to 14 bilateral proximal muscles were exercised 3 times weekly for 12 weeks.
  
  
  RESULTS
  Nine children with SMA, aged 10.4 ± 3.8 years, completed the resistance training exercise program. Ninety percent of visits occurred per protocol. Training sessions were pain-free (99.8%), and no study-related adverse events occurred. Trends in improved strength and motor function were observed.
  
  
  CONCLUSIONS
  A 12-week supervised, home-based, 3-day/week progressive resistance training exercise program is feasible, safe, and well tolerated in children with SMA. These findings can inform future studies of exercise in SMA.
authors.affiliation: Division of Physical Medicine and Rehabilitation, Pediatric Motor Disorders Research Program, University of Utah School of Medicine, 30 North 1900 East, Salt Lake City, Utah, 84132, USA.
authors.forename: Aga
authors.initials: A
authors.lastname: Lewelt
authors.affiliation: Department of Physical Therapy and Human Movement Sciences, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
authors.forename: Kristin J
authors.initials: KJ
authors.lastname: Krosschell
authors.affiliation: Study Design and Biostatistics Center, University of Utah, Salt Lake City, Utah, USA.
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Stoddard
authors.affiliation: Study Design and Biostatistics Center, University of Utah, Salt Lake City, Utah, USA.
authors.forename: Cindy
authors.initials: C
authors.lastname: Weng
authors.affiliation: Department of Biomedical Informatics, University of Utah, Salt Lake City, Utah, USA.
authors.forename: Mei
authors.initials: M
authors.lastname: Xue
authors.affiliation: Department of Physical Therapy, University of Utah, Salt Lake City, Utah, USA.
authors.forename: Robin L
authors.initials: RL
authors.lastname: Marcus
authors.affiliation: Department of Physical Therapy, University of Utah, Salt Lake City, Utah, USA.
authors.forename: Eduard
authors.initials: E
authors.lastname: Gappmaier
authors.affiliation: Department of Neurology, Pediatric Motor Disorders Research Program, University of Utah School of Medicine, Salt Lake City, Utah, USA.
authors.forename: Louis
authors.initials: L
authors.lastname: Viollet
authors.affiliation: Department of Neurology, Pediatric Motor Disorders Research Program, University of Utah School of Medicine, Salt Lake City, Utah, USA.
authors.forename: Barbara A
authors.initials: BA
authors.lastname: Johnson
authors.affiliation: Department of Exercise and Sport Science, University of Utah, College of Health, Salt Lake City, Utah, USA.
authors.forename: Andrea T
authors.initials: AT
authors.lastname: White
authors.affiliation: Department of Neurology, Pediatric Motor Disorders Research Program, University of Utah School of Medicine, Salt Lake City, Utah, USA.
authors.forename: Donata
authors.initials: D
authors.lastname: Viazzo-Trussell
authors.affiliation: Neuromuscular Degeneration and Plasticity Laboratory, Institut National de la Santé et de la Recherche Médicale UMR-S 1124, University Paris Descartes, Paris, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Lopes
authors.affiliation: Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
authors.forename: Robert H
authors.initials: RH
authors.lastname: Lane
authors.affiliation: Division of Pediatric Genetics, University of Utah School of Medicine, Salt Lake City, Utah, USA.
authors.forename: John C
authors.initials: JC
authors.lastname: Carey
authors.affiliation: Department of Neurology, Pediatric Motor Disorders Research Program, University of Utah School of Medicine, Salt Lake City, Utah, USA.
authors.forename: Kathryn J
authors.initials: KJ
authors.lastname: Swoboda
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D009043:Motor Activity / Q000502:physiology; D053580:Muscle Strength; D018482:Muscle, Skeletal / Q000503:physiopathology; D009134:Muscular Atrophy, Spinal / Q000503:physiopathology / Q000534:rehabilitation*; D012018:Reflex / Q000502:physiology; D015203:Reproducibility of Results; D055070:Resistance Training / Q000379:methods*; D016896:Treatment Outcome
keywords: home-based exercise program; neuromuscular disorder; progressive resistance training exercise; spinal muscular atrophy; strength training exercise