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Title: [Adrenoleukodystrophy (Schilder-Addison's disease): a probable case in an adult (author's transl)]. Author: Duprey J, Lubetzki J, Lemaignen H. Journal: Ann Endocrinol (Paris); 1979; 40(1):77-8. PubMed ID: 443745. Abstract: Adrenoleukodystrophy (Schilder-Addison's disease) is a sex-linked recessive syndrome usually observed in children, associating neurological disorders and Addison's disease. Its outcome is death after a few years. The authors report the case of a man in whom a spastic paraparesis and a mental deterioration appeared at the age of 30 years. A biological adrenal deficiency was found: plasma cortisol and urinary 17-hydroxy-steroids and 17-ketosteroids failed to increase after synacthen stimulation; plasma ACTH level was above normal. Since two nephews of the patient suffered from adrenoleukodystrophy, the same disease is likely in the reported patient.[Abstract] [Full Text] [Related] [New Search]