Triple A syndrome
- Achalasia Addisonianism Alacrimia syndrome
- AAA syndrome
- Alacrima-achalasia-adrenal insufficiency neurologic disorder
- Addisonian achalasia syndrome
Your QuestionMy son is 5 years old and has triple A syndrome. He has Addison's and alacrima, and we want to know if it is likely he will develop achalasia. What are the neurological effects of triple A syndrome and when could they affect him?
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Many of the neurological features of triple A syndrome are caused by dysfunction of the autonomic nervous system. This part of the nervous system controls involuntary body processes such as digestion, blood pressure, and body temperature. People with triple A syndrome often experience abnormal sweating, difficulty regulating blood pressure, unequal pupil size (anisocoria), and other signs and symptoms of autonomic nervous system dysfunction (dysautonomia).
People with this condition may have other neurological abnormalities, such as developmental delay, intellectual disability, speech problems (dysarthria), and a small head size (microcephaly). In addition, affected individuals commonly experience muscle weakness, movement problems, and nerve abnormalities in their extremities (peripheral neuropathy). Some develop optic atrophy, which is the degeneration (atrophy) of the nerves that carry information from the eyes to the brain.
Most of the neurologic features of triple A syndrome begin during adulthood, and they often worsen over time.
- Achalasia. International Foundation for Functional Gastrointestinal Disorders. April 28, 2015; http://www.aboutgimotility.org/site/about-gi-motility/disorders-of-the-esophagus/achalasia.
- Stuart J Sechler. Clinical Manifestations and diagnosis of achalasia. UpToDate. February 24, 2016; http://www.uptodate.com/contents/achalasia-beyond-the-basics#H1.
- John E. Pandolfino, Andrew J. Gawron. Achalasia A Systematic Review. JAMA. May 12, 2015; 313(18):1841-1852. http://www.ncbi.nlm.nih.gov/pubmed/25965233.
- Triple A syndrome. Genetics Home Reference. February 2010; http://ghr.nlm.nih.gov/condition/triple-a-syndrome. Accessed 9/24/2015.