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

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

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").

Inclusion Criteria:

• FSHD patients will be recruited on the basis of:

  • The number of repeat units (4 to 9)
  • FSHD patients with a positive family history for FSHD
  • Not confined to a wheelchair
  • No smokers
  • No associated co-morbidity (cardiac or pulmonary disease, diabetes etc.)
  • No Medications or nutritional supplementation (vitamins and/or antioxidants) at the time of the study
  • No HIV positive

Exclusion Criteria:

• No consent form