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X-linked adrenoleukodystrophyAn X-linked recessive peroxisomal disease due to a defective gene on chromosome Xq28, which is characterised by impaired degradation of saturated very-long-chain fatty acids (VLCFAs) that accumulate with the cholesterol ester and gangliosides in the CNS, adrenal glands, plasma cells, and leukocytes, causing progressive multifocal demyelination.
Variable; often first seen in childhood with major neurologic deterioration and death in a few years.
Modifications in lipid intake may normalise VLCFA levels, the long-term effects of which are unknown; bone marrow transplantation.
It had been reported that a concoction of oleic and erucic acids–glycerol trioleate and trierucic acids (popularly, “Lorenzo’s Oil”) was of use in treating adrenoleukodystrophy or adrenomyeloneuropathy.