Oxidative stress and dystrophy Facioscapulohumeral: Effects of vitamin C, vitamin E, zinc gluconate and selenomethionine supplementation

Abstract

Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant disease characterized by progressive weakness and atrophy of specific skeletal muscles. Despite major progress in the understanding of the genetic basis of FSHD, the exact mechanisms that lead to FSHD defects are not completely understood and no curative treatment is available. However, there is growing evidence that oxidative stress may contribute to FSHD pathology. We recently reported that reduced physical performance in patients with FSHD is associated with important redox unbalance and oxidative stress in blood. Hence, we hypothesized that insufficient intake of antioxidant vitamins and minerals may reduce the body capacity to regulate free radical insults, leading to a condition known as oxidative stress that could affect muscle function performance in patients with FSHD. We thus conducted a pilot randomized double-blind placebo-controlled study to test whether oral administration of vitamins and minerals could improve the physical performance of patients with FSHD. The results of this randomized double-blind placebo-controlled trial show that supplementation with vitamin C, vitamin E (as alpha tocopherol), zinc gluconate and selenomethionine in patients with FSHD significantly improves the maximal voluntary contraction and endurance of both quadriceps by enhancing the antioxidants defences and reducing oxidative stress.