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Genetic and Rare Diseases Information Center (GARD)

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Adie syndrome


Other Names for this Disease

  • Adie's Pupil
  • HAS
  • Holmes-Adie syndrome
  • Tonic, sluggishly reacting pupil and hypoactive or absent tendon reflexes
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Overview

What is Adie syndrome?

What are the symptoms of Adie syndrome?

What causes Adie syndrome?

What is Adie syndrome?

Adie syndrome is is a neurological disorder affecting the pupil of the eye and the autonomic nervous system.  It is characterized by one eye with a pupil that is larger than normal that constricts slowly in bright light (tonic pupil), along with the absence of deep tendon reflexes, usually in the Achilles tendon. Adie syndrome may be the result of a viral or bacterial infection that causes inflammation and damage to neurons in the ciliary ganglion, an area of the brain that controls eye movements, and the spinal ganglion, an area of the brain involved in the response of the autonomic nervous system.[1] Most cases of Adie Syndrome are not progressive, life threatening, or disabling.[1][2]  
Last updated: 5/3/2010

What are the symptoms of Adie syndrome?

Adie syndrome is characterized by one eye with a pupil that is larger than normal that constricts slowly in bright light (tonic pupil), along with the absence of deep tendon reflexes, usually in the Achilles tendon. It typically begins gradually in one eye, and often progresses to involve the other eye. At first, it may only cause the loss of deep tendon reflexes on one side of the body, but then progress to the other side. The eye and reflex symptoms may not appear at the same time.  People with Adie syndrome may also sweat excessively, sometimes only on one side of the body. The combination of these 3 symptoms – abnormal pupil size, loss of deep tendon reflexes, and excessive sweating – is usually called Ross’s syndrome, although some doctors will still diagnosis the condition as a variant of Adie syndrome. Some individuals will also have cardiovascular abnormalities. The symptoms of Adie syndrome can appear on their own, or in association with other diseases of the nervous system, such as Sjogren’s syndrome or migraine.[1] 
Last updated: 5/3/2010

What causes Adie syndrome?

Adie syndrome is thought to be the result of a viral or bacterial infection that causes inflammation and damage to neurons in the ciliary ganglion, an area of the brain that controls eye movements, and the spinal ganglion, an area of the brain involved in the response of the autonomic nervous system.  In rare cases, Adie syndrome may be inherited.[1] In these cases, it appears to follow an autosomal dominant pattern of inheritance.[3] 
  
Last updated: 5/3/2010

References
  1. NINDS Holmes-Adie syndrome Information Page. National Institute of Neurological Disorders and Stroke (NINDS). February 13, 2007; http://www.ninds.nih.gov/disorders/holmes_adie/holmes_adie.htm. Accessed 5/3/2010.
  2. Adie Syndrome. National Organization for Rare Disorders (NORD). 2007; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Adie%20Syndrome. Accessed 5/3/2010.
  3. Adie Pupil. Online Mendelian Inheritance in Man (OMIM). July 5, 2006; http://www.ncbi.nlm.nih.gov/omim/103100. Accessed 5/3/2010.


Other Names for this Disease
  • Adie's Pupil
  • HAS
  • Holmes-Adie syndrome
  • Tonic, sluggishly reacting pupil and hypoactive or absent tendon reflexes
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.