AAA Syndrome in Progress

AAA Syndrome in Progress

A rare autosomal recessive condition of early onset characterised by alacrima, achalasia, autonomic disturbance and ACTH insensitivity, which usually presents in childhood with dysphagia or severe (occasionally fatal) episodes of hypoglycaemia or hypotension due to adrenocortical insufficiency, which itself may be delayed until adulthood. Adults with AAA syndrome, also known as Allgrove’s syndrome, may present with progressive neurologic disease including hyperreflexia, muscle wasting, dysarthria, ataxia, optic atrophy and intellectual impairment.