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 nâ=â8) in a home-based exercise intervention. Training consisted of cycling 3 times weekly for 35âminutes (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%, Pâ=â0.002) 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% (Pâ=â0.008) and 46% (Pâ=â0.003), 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.
Subject(s)
Exercise Therapy , Muscular Dystrophy, Facioscapulohumeral/therapy , Adult , Biopsy , Creatine Kinase/blood , Exercise Test , Fatigue/physiopathology , Fatigue/prevention & control , Female , Humans , Male , Muscle Strength/physiology , Muscle, Skeletal/pathology , Muscular Dystrophy, Facioscapulohumeral/physiopathology , Oxygen Consumption/physiology , Physical Endurance/physiology , Quality of LifeABSTRACT
AIMS: Muscle satellite cells (SCs) are responsible for the regenerative events following muscle fibre injury. This study aimed to improve our understanding of SC behaviour in two models of muscle disorder with different pathological mechanisms and onset of disease. METHODS AND RESULTS: Pax7(+) SC content was assessed in types I and II fibres of patients with Duchenne muscular dystrophy (DMD; n = 9; age 13 ± 2 years), polymyositis/dermatomyositis (PM/DM; n = 9; age 52 ± 12 years) and in controls (n = 5; age 26 ± 5 years). Pax7(+) SCs number in type I and II fibres was higher (P < 0.05) in DMD and in PM/DM compared to controls. Type I fibres were associated with a higher number of Pax7(+) SCs compared to type II fibres only in DMD; Pax7(+) SCs number in type I fibres was about threefold higher in DMD compared to PM/DM (P < 0.05). In DMD, Pax7(+) SC content in small regenerating fibres (0.09 ± 0.09 SCs/fibre) was similar to that in fibres from healthy skeletal muscle. The proportion of activated SCs (Ki-67(+) SCs) was fivefold lower in DMD (0.4 ± 0.4%) compared to PM/DM (2.8 ± 2%). Pax7(+) cells located outside the basal lamina were observed in DMD muscles only. CONCLUSION: The capacity to generate new SCs is increased even in severely impaired muscles and a fibre type-specific enhancement of SC occurs in type I muscle fibres in DMD.
