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

Study Overview
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Brief Summary
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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.

Detailed Description
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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).

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

Conditions
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Facioscapulohumeral Muscular Dystrophy

Intervention / Treatment
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* Procedure: Taking of blood
* Dietary Supplement: needle biopsy of the vastus lateralis muscle
* Dietary Supplement: Vit C Vit E Zn Se
* Dietary Supplement: Placebo Vit E Placebo Vit C Zn Se


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 60 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Supportive Care
Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: Double


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Active Comparator: vitamins minerals<br>VitE 400/d, vitC 500mg/d, Se 200µg/d (selenomethionine), Zn 25 mg/d gluconate Venous blood samples( analysis oxidative stress inflammatory markers) Needle biopsy of the vastus lateralis muscle (analysis oxidative stress inflammatory markers) | Procedure: Taking of blood<br>* Taking venous blood samples to analyse oxidant stress<br>Dietary Supplement: needle biopsy of the vastus lateralis muscle<br>* 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<br>Dietary Supplement: Vit C Vit E Zn Se<br>* 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<br>* Other names: Antioxidant;|
| Placebo Comparator: Placebo<br>Supplementation 17 weeks placebo venous blood samples (analysis of oxidative stress inflammatory markers) needle biopsy of the vastus lateralis muscle | Procedure: Taking of blood<br>* Taking venous blood samples to analyse oxidant stress<br>Dietary Supplement: needle biopsy of the vastus lateralis muscle<br>* 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<br>Dietary Supplement: Placebo Vit E Placebo Vit C Zn Se<br>* venous blood samples and needle biopsy<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 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). | duration study 3 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 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. | 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. | duration study 3 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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FSHD, Antioxidants, Oxidative stress, Muscle function, Quality of life, Activity, (FSHD;4q35)