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Article: Adrenomyeloneuropathy as a cause of primary adrenal insufficiency and spastic paraparesis.

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

CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne

2004  Volume 171, Issue 9, Page(s) 1073–1077

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 ... ...

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.
MeSH term(s) Addison Disease/diagnosis ; Addison Disease/etiology ; Addison Disease/therapy ; Adrenoleukodystrophy/complications ; Adrenoleukodystrophy/diagnosis ; Adult ; Biopsy, Needle ; Combined Modality Therapy ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Male ; Paraparesis, Spastic/diagnosis ; Paraparesis, Spastic/etiology ; Paraparesis, Spastic/therapy ; Risk Assessment ; Severity of Illness Index ; Treatment Outcome
Language English
Publishing date 2004-10-26
Publishing country Canada
Document type Case Reports ; Journal Article ; Review
ZDB-ID 215506-0
ISSN 1488-2329 ; 0820-3946 ; 0008-4409
ISSN (online) 1488-2329
ISSN 0820-3946 ; 0008-4409
DOI 10.1503/cmaj.1032006
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