title: Safety and efficacy of a 6-month home-based exercise program in patients with facioscapulohumeral muscular dystrophy: A randomized controlled trial.
abstract: BACKGROUND
  Previous randomized controlled trials investigating exercise training programs in facioscapulohumeral muscular dystrophy (FSHD) patients are scarce and of short duration only. This study assessed the safety and efficacy of a 6-month home-based exercise training program on fitness, muscle, and motor function in FSHD patients.
  
  
  METHODS
  Sixteen FSHD patients were randomly assigned to training (TG) and control (CG) groups (both n = 8) in a home-based exercise intervention. Training consisted of cycling 3 times weekly for 35 minutes (combination of strength, high-intensity interval, and low-intensity aerobic) at home for 24 weeks. Patients in CG also performed an identical training program (CTG) after 24 weeks. The primary outcome was change in peak oxygen uptake (VO2 peak) measured every 6 weeks. The principal secondary outcomes were maximal quadriceps strength (MVC) and local quadriceps endurance every 12 weeks. Other outcome measures included maximal aerobic power (MAP) and experienced fatigue every 6 weeks, 6-minute walking distance every 12 weeks, and muscle characteristics from vastus lateralis biopsies taken pre- and postintervention.
  
  
  RESULTS
  The compliance rate was 91% in TG. Significant improvements with training were observed in the VO2 peak (+19%, P = 0.002) and MAP by week 6 and further to week 24. Muscle endurance, MVC, and 6-minute walking distance increased and experienced fatigue decreased. Muscle fiber cross-sectional area and citrate synthase activity increased by 34% (P = 0.008) and 46% (P = 0.003), respectively. Dystrophic pathophysiologic patterns were not exacerbated. Similar improvements were experienced by TG and CTG.
  
  
  CONCLUSIONS
  A combined strength and interval cycling exercise-training program compatible with patients' daily professional and social activities leads to significant functional benefits without compromising muscle tissue.
authors: Laboratoire Interuniversitaire de Biologie de la Motricité, UJM-Saint-Etienne, Université de Lyon, Saint-Etienne, France Unité de Myologie, Centre Hospitalier, Universitaire de Saint-Etienne, Saint-Etienne, France Division of Sport Sciences, School of Health and Medical Sciences, Orebro University, Orebro, Sweden Human Performance Laboratory, Faculty of Kinesiology, University of Calgary, Calgary, Alberta, Canada U1042, INSERM, Grenoble, France Laboratoire HP2, Grenoble Alpes University, Grenoble, France Centre Référent Maladies Neuromusculaires Rares Rhône-Alpes, Saint-Etienne, France.
authors: Landry-Cyrille
authors: LC
authors: Bankolé
authors: Guillaume Y
authors: GY
authors: Millet
authors: John
authors: J
authors: Temesi
authors: Damien
authors: D
authors: Bachasson
authors: Marion
authors: M
authors: Ravelojaona
authors: Bernard
authors: B
authors: Wuyam
authors: Samuel
authors: S
authors: Verges
authors: Elodie
authors: E
authors: Ponsot
authors: Jean-Christophe
authors: JC
authors: Antoine
authors: Fawzi
authors: F
authors: Kadi
authors: Léonard
authors: L
authors: Féasson
mesh_terms: D000328:Adult; D001706:Biopsy; D003402:Creatine Kinase / Q000097:blood; D005080:Exercise Test; D005081:Exercise Therapy; D005221:Fatigue / Q000503:physiopathology / Q000517:prevention & control; D005260:Female; D006801:Humans; D008297:Male; D053580:Muscle Strength / Q000502:physiology; D018482:Muscle, Skeletal / Q000473:pathology; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000503:physiopathology / Q000628:therapy*; D010101:Oxygen Consumption / Q000502:physiology; D010807:Physical Endurance / Q000502:physiology; D011788:Quality of Life
chemical_list: D003402:Creatine Kinase