Catalog

Autonomic neuropathy due to Allgrove syndrome

No MMS code

Link to WHO Definition

Autonomic neuropathy due to Allgrove syndrome – http://id.who.int/icd/entity/482253216

This is an autosomal recessive disorder secondary to a mutation in the AAAS ( achalasia, addisonianism, alacrima syndrome) gene coding for a protein called ALADIN ( alacrima, achalasia, adrenal insufficiency neurologic disorder). Autonomic dysfunction is associated with achalasia, adrenal insufficiency and alacrima, pyramidal syndrome, peripheral motor neuropathy, bulbar dysfunction.[1] Vallet AE. et al. Neurological features in adult triple-A (Allgrove) syndrome. J Neurol (2012) 259:39-46