Lovastatin for X-Linked Adrenoleukodystrophy
1998
To the Editor: X-linked adrenoleukodystrophy is an inherited recessive disorder characterized by a defect in peroxisomal β-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 . . .
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