CMAJ • October 26, 2004; 171 (9). doi:10.1503/cmaj.1032006.
© 2004 Canadian Medical Association or its licensors
All editorial matter in CMAJ represents the opinions of the authors and not necessarily those of the Canadian Medical Association.
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Review
Synthèse

Adrenomyeloneuropathy as a cause of primary adrenal insufficiency and spastic paraparesis

Monika Spurek, Regina Taylor-Gjevre, Stan Van Uum and Hasnain M. Khandwala

All authors are with the Department of Medicine, University of Saskatchewan, Saskatoon, Sask.

Abstract

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 adrenomyeloneuropathy and discuss the clinical presentation, diagnosis and management of the 2 disorders.




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