Effects Antioxidants Supplementation on Muscular Function Patients Facioscapulohumeral Dystrophy (FSHD)

• Improvement of muscle effort tolerance after antioxidant supplementation [ Time Frame: duration study 3 years ] [ Designated as safety issue: No ]
  17-weeks evaluation of an antioxidant supplementation in order to modulate or delay oxidative insult that could be useful to maintain FSHD muscle function. At T0, each patient will perform functionnal evaluation (Exercise Tolerance (2 minutes walking test), and/or Maximal Volontary Contraction and/or Endurance of quadriceps muscles).
  
  

• Changes in inflammatory and oxidative stress parameters after antioxidant supplementation [ Time Frame: duration study 3 years ] [ Designated as safety issue: No ]
  T0 evaluations spirometry, electrocardiogram, holter,Magnetic Resonance imaging of the both thighs,estimation muscle oxygen consumption during acute exercise . T7 venous blood samples (oxidative stress inflammatory markers)needle biopsy of the vastus lateralis muscle (oxidative stress inflammatory markers). analysis oxidative stress on muscle biochemical analyses.T7 patient receives placebo or antioxidant supplementation corresponding randomly-assigned trial. After 17 weeks supplementation the subjects will obtain venous blood samples and needle biopsy.
  
  
• Changes in muscular function after antioxidant supplementation [ Time Frame: duration study 3 years ] [ Designated as safety issue: No ]
  T0 evaluations spirometry, electrocardiogram, holter Magnetic Resonance imaging of the both thighs,estimation muscle oxygen consumption . T7 venous blood samples (oxidative stress inflammatory markers)needle biopsy of the vastus lateralis muscle (oxidative stress inflammatory markers). This one-week lap's time is needed to avoid potential confounding effects of exercise-induced oxidative stress on muscle biochemical analyses.T7 patient receives placebo or antioxidant supplementation corresponding randomly-assigned trial. After 17 weeks supplementation venous blood samples and needle biopsy.
  
  

This study will compare the effect of the therapeutic interest of an antioxidant supplementation on the functional deficits and the molecular muscle abnormalities into two groups of patients affected by FSHD, one treated with the antioxidant supplementation during 17 weeks. The antioxidant by capsule consisted of:Vitamin E (400 mg /day), Selenium (200 µg/day in the form of selenomethionine), Vitamin C (500 mg/day), Zinc (25 mg/day in the form of gluconate). The second one treated with a placebo during 17 weeks. Patients will be assigned to intervention groups by chance, and neither physician, nor patient, will know which product is administrated (study in "double blind").