triple-A syndrome


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triple-A syndrome

An autosomal recessive condition (OMIM:231550) characterised by glucocorticoid deficiency, achalasia of the gastric cardia, defective tear formation (alacrima) and often changes of autonomic dysfunction. Over time, many patients develop neurologic disease (e.g., polyneuropathy with sensory, motor and autonomic components; long-tract degeneration; parkinsonism; mild dementia).
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Triple-A syndrome, also known as achalasia-addisonianism-alacrimia syndrome or Allgrove syndrome, is a rare autosomal and life-threatening congenital progressive disorder.