Adrenomyeloneuropathy as a cause of primary adrenal insufficiency and spastic paraparesis

Spurek, Monika; Taylor-Gjevre, Regina; Van Uum, Stan; Khandwala, Hasnain M.
October 2004
CMAJ: Canadian Medical Association Journal;10/26/2004, Vol. 171 Issue 9, p1073
Academic Journal
ADRENOMYELONEUROPATHY IS A VARIENT OF ADRENOLEUKODYSTROPHY, both of which are rare inherited disorders of peroxisomes 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. In this article, we describe an illustrative case of adreno-myeloneuropathy and discuss the clinical presentation, diagnosis and management of the 2 disorders.


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