Adrenomyeloneuropathy

A progressive degenerative disorder of the myelin sheathing of the spinal cord.

a varient of adrenoleulkodystrophy.

disorder can cause adrenal insufficiency and hypogonadism in addition to neurological symptoms

Biochemistry:

disorder of the peroxisomes, (intracellular organelles responsible for a variety of biochemical reactions) the most important being the oxidation of VLCFAs, biosynthesis of bile acids and glycoxylate detoxification.

People with adrenoleukodystrophy and adrenomyeloneuropathy do not produce fatty acylcoenzyme A (acyl-CoA) synthetase, which breaks down VLCFAs in the first step in their oxidation.

VLCFAs accumulate and form cytoplasmic inclusions, which leads to progressive dysfunction in the nervous system, adrenal glands and testes, all of which are sites of VLCFA metabolism.

Etiology:

characterized by the accumulation of very-long-chain fatty acids in plasma, the central and peripheral nervous systems, adrenal glands and testes, which leads to dysfunction of these organs and systems.

X-linked genetic disorder,  the gene is located on the long arm of the X chromosome (Xq28)

Management:

 limited and includes hormonal replacement therapy for concomitant adrenal insufficiency or hypogonadism, or both.

dietary goals:

to decrease the exogenous sources of VLCFA by instituting a low-fat diet; however, dietary modifications have not been consistently shown to prevent or slow the course of the disease.

Administration of monounsaturated fatty acids leads to competitive inhibition between saturated and unsaturated fatty acid precursors in the microsomal elongation pathway, thereby leading to a reduction in endogenous VLCFA synthesis.

Khandwala, Hasnain M, Spurek, Monika,Taylor-Gjevre,  Regina,  and Van Uum, Stan. 2004. Adrenomyeloneuropathy as a cause of primary adrenal insufficiency and spastic paraparesis. CMAJ. 171:9. Retrieved February 1, 2010 from http://www.cmaj.ca/cgi/content/full/171/9/1073

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