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adrenal leukodystrophy


Definition: sudanophilic leukodystrophy with bronzing of skin and adrenal atrophy. A metabolic disorder of young males, characterized by widespread myelin degeneration and associated adrenal insufficiency. The myelin degeneration is massive in various portions of the brain and sometimes the spinal cord, with the accumulation of degradation products of myelin in macrophages (sudanophilic demyelination); atrophy is present in the adrenal glands and testes, and markedly increased amounts of very long chain fatty acid are present in both the brain and the adrenal glands. Symptoms include bronzing of the skin, dysarthria, cortical blindness, bilateral hemiplegia, pseudobulbar paralysis, and progressive dementia. Probably sex-linked recessive inheritance.

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.