Achalasia-addisonism-alacrimia syndrome, also known as triple A syndrome, is characterized by the concomitant onset of Addison's disease, achalasia, and alacrimia.
The syndrome does not presume the joint occurrence of all three cardinal symptoms.In the absence of symptoms or varying neurological features and autonomic dysfunction it is a matter of the 'Double A (AA)' or 'Quaternary A (AAAA) syndrome'.
Other symptoms include adrenal insufficiency with isolated glucocorticoid deficiency, alacrimia, alachasia, autonomic dysfunction and neurodegeneration.
The onset of symptoms runs through all age groups, from infancy to adulthood.When the disease starts in early childhood, it is characterized by alacrimony and possibly achalasia.Adrenocortical insufficiency and achalasia are characteristic of childhood onset or adolescence.The disease only occurs in adulthood, the main symptoms being of neurological, vegetative and polyneuropathic nature.
Alacrimia is often the first clinical symptom, but it is only the achalasia of the cardia which leads to dysphagia that usually leads to the diagnosis.Adrenal insufficiency can cause seizures and hypoglycaemia.The neurological disorders vary widely and range from dyshidrosis to indigestion and sexual disorders to circulatory and urinary disorders.
Other symptoms seen in several patients include walking disability, sensory problems, amyotrophy or tongue palsy, soft palate insufficiency, oro-pharyngeal dysphagia, and orbicularis oris disorder.