Allgrove syndrome (AAA syndrome, triple A syndrome) is a rare disorder initially described in a consanguineous family of French Canadian and Indian heritage. Cases have now been identified in many countries from around the world.

Chromosome: 12q13.13

Gene: AAAS (aladin WD repeat nucleoporin, ALADIN)


Inheritance: autosomal recessive


Key features (AAA):

(1) adrenal insufficiency unresponsive to ACTH (Addison's disease)

(2) achalasia (failure of lower esophageal sphincter to relax)

(3) alacrima (reduced or absent tear production; abnormal Schirmer's test)


Variable findings:

(1) optic atrophy

(2) neurologic abnormalities including autonomic dysfunction and motor weakness



(1) adrenal failure during stress

(2) hypoglycemic seizures

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