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Unusual causes of adrenal insufficiency

Lynnette K Nieman, MD
Section Editor
André Lacroix, MD
Deputy Editor
Kathryn A Martin, MD


The major causes of primary adrenal insufficiency (Addison's disease) are autoimmune diseases and, to a lesser degree, infections, metastatic disease, and drugs. (See "Causes of primary adrenal insufficiency (Addison's disease)".)

A number of other less common disorders can also cause adrenal insufficiency; these conditions will be reviewed here (table 1).


The two phenotypes of this X-linked recessive disorder, which affects 1 in 20,000 males, are characterized by progressive neurologic dysfunction and primary adrenal insufficiency [1]. This disorder may account for as many as 10 percent of all cases of adrenal insufficiency, but can be distinguished from autoimmune adrenalitis by the lack of circulating adrenal autoantibodies [2]. The diagnosis is confirmed by measurement of very long chain fatty acids (VLCFAs), which accumulate in all tissues.

Adrenoleukodystrophy begins in infancy or childhood with weakness and spasticity and progresses rapidly to dementia, blindness, and quadriparesis [1,3,4]. Adrenal insufficiency may be the only sign of ALD [5].

Adrenomyeloneuropathy begins in adolescence or early adulthood with weakness, spasticity, and distal polyneuropathy, but is milder and progresses more slowly [6].


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Literature review current through: Sep 2016. | This topic last updated: Sep 5, 2014.
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