Autonomic Dysfunction in the Adrenal Insufficiency/Alacrima/Achalasia (Triple A) Syndrome (Is it Quaternary A Syndrome?)

Abstract

Allgrove or Triple A Syndrome (AS) is an autosomal recessive condition associated with adrenal insufficiency due to ACTH resistance, alacrima, and achalasia. Other features of AS include autonomic and/or peripheral neuropathy, hyperkeratosis and delayed wound healing, mental retardation and dementia, and rarely, short stature. To increase awareness about the frequent autonomic nervous system involvement we want to report a child with 3A syndrome who later developed distal polyneuropathy in lower extremities.