On the basis of published data and the investigators' results indicating that oxidative stress may contribute to the peripheral skeletal muscle dysfunction in patients with FSHD, the investigators propose a study to test whether or not an antioxidant supplementation has a therapeutic interest for patients with FSHD. Their results have important implications for the successful implementation of rational antioxidant therapy in FSHD in which cell loss could be linked to oxidative stress.
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").
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
SUPPORTIVE_CARE
Masking
DOUBLE
Enrollment
54
Taking venous blood samples to analyse oxidant stress
T0 needle biopsy of the vastus lateralis muscle (analysis of oxidative stress and inflammatory markers)During 17 weeks supplementation by Vit E, C , Zn Se After 17 weeks: veinous blood samples and needle biopsy
T0 venous blood samples and needle biopsy of the vastus lateralis muscle During 17 weeks vit E 400mg/d, Se 200µg/d, Vit C 500mg/day, Zn 25 mg/d After the supplementation of 17 weeks:venous blood samples and needle biopsy of the vastus lateralis muscle
Montpellier University Hospital- Saint Eloi Hospital
Montpellier, Languedoc-Roussillon, France
Improvement of muscle effort tolerance after antioxidant supplementation
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).
Time frame: duration study 3 years
Changes in inflammatory and oxidative stress parameters after antioxidant supplementation
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.
Time frame: duration study 3 years
Changes in muscular function after antioxidant supplementation
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.
Time frame: duration study 3 years
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.
venous blood samples and needle biopsy