**8. Hypotesis and intervention**

It is possible to improve the life quality of Duchenne patients by biological active substances (antioxidants), and psychotherapeutic intervention. Therapy of DMD has been an elusive goal. Studies with isolated myocytes have shown that lipid peroxidation with an enhanced free radical production can be activated by increasing Ca concentration. No wonder that several kinds of antioxidants have been proposed as a treatment since increased levels of thiobarbituric acid (TBA) reactive material has been found in the muscles and blood of patients with DMD.

Vitamin E has been observed to decrease the amount of TBA reactive material in dystrophic muscle. Previously we have reported that the biological half-life of selenium -75 (75Se) in DMD patients is significantly shorter than in healthy controls (Westermarck et al 1982). On the basis of these facts we started to treat some DMD patients with selenium and other antioxidants. Selenium is activating a well-known antioxidative enzyme glutathione peroxidase (GSH-Px, iodothyronine desiodinase, selenoprotein P, thioredoxin reductase and the selenoprotein W, that all contain selenocystein. Beside iodothyronine desiodinase that facilitates the activation of thyroid hormones; all other selenoproteins have a role in the metabolisation and detoxifi‐ cation of reactive oxygen species and in the maintenance of the antioxidant defense of cells.

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For example, in 1981 the antioxidant treatment of two siblings with DMD, aged six and ten years, was started. At that time the older brother was wheel-chair bound and was not able to walk since eight years of age, the younger one was still able to walk almost normally. The condition of the older brother, who practically had not got carnitine or coenzyme Q10 supple‐ mentation, was gradually deteriorated, and he deceased at 17 years of age. However, at the age of 15 years the younger brother was still able to walk without any assistance. Six months later he became wheel-chair bound. The younger brother was one of the most talented graduated students in his high-school. The mean IQ of DMD patients is without treatment 15-20 points lower than that of normal populations. Anyway for some years he was able to study at the University of Joensuu. Now he is 31 years old and still able to swim some meters, and to produce computer art. Actually he had his first art-show in 2003 in Helsinki. Moreover, only during the nights it is necessary to give him non-invasive breathing assistance.

The present daily megadoses of nutrients supplements (vitamin E 1200mg; sodium selenite 8mg; riboflavin 3mg; pyridoxine 75mg; carnitine 600mg; and coenzyme Q10 180mg) have been well tolerated and no side effects have observed in our open controlled trial (Westermarck et al 1997). The long-term pilot study speaks in favor for a larger study. Dr. Anders Erikson from Sweden (personal communication) has made the same observations with two Swedish siblings suffering from DMD, as we have also made with two 13- and 14-year old cousins 2with DMD.
