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Previous Volume 339:702-703 September 3, 1998 Number 10 Next

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To the Editor: X-linked adrenoleukodystrophy is an inherited recessive disorder characterized by a defect in peroxisomal -oxidation of very-long-chain fatty acids (those with more than 22 carbon atoms) and secondary neuroinflammatory damage.^1,2 Even though the accumulation of very-long-chain fatty acids in plasma and tissues occurs early, the neurologic manifestations are not observed until the age of 4 to 8 years for childhood adrenoleukodystrophy or 20 to 30 years for adrenomyeloneuropathy. The neurologic damage in X-linked adrenoleukodystrophy may be mediated by the activation of astrocytes and the induction of proinflammatory cytokines. At present, dietary restrictions and therapy with Lorenzo's oil are . . . [Full Text of this Article]

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