| Adie syndrome | |
|---|---|
| Classification and external resources | |
| ICD-9 | 379.46 |
| DiseasesDB | 29742 |
| MeSH | D015845 |
Adie syndrome, sometimes known as Holmes-Adie's syndrome or Adie's Tonic Pupil, is a neurological disorder characterized by a tonically dilated pupil.[1] It is named after the British neurologist William John Adie. It is caused by damage to the postganglionic fibers of the parasympathetic innervation of the eye, usually by a viral or bacterial infection which causes inflammation, and affects the pupil of the eye and the autonomic nervous system. [1]
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Adie syndrome presents with three hallmark symptoms, namely at least one abnormally dilated pupil (mydriasis) which does not constrict in response to light, loss of deep tendon reflexes and diaphoresis (excessive sweating).[1] Other signs may include hyperopia due to accommodative paresis, photophobia and difficulty reading.[2]
Holmes-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.[1]
Clinical exam may reveal sectoral paresis of the iris sphincter or vermiform iris movements. The tonic pupil may become smaller (miotic) over time which is referred to as "little old Adie's".[3] Testing with low dose (1/8%) pilocarpine may constrict the tonic pupil due to cholinergic denervation supersensitivity.[1] A normal pupil will not constrict with the dilute dose of pilocarpine.[3] CT scans and MRI scans may be useful in the diagnostic testing of focal hypoactive reflexes.[4]
The usual treatment of a standardised Adie syndrome is to prescribe reading glasses to correct for impairment of the eye(s).[1] Pilocarpine drops may be administered as a treatment as well as a diagnostic measure.[1] Thoracic sympathectomy is the definitive treatment of diaphoresis, if the condition is not treatable by drug therapy.[1]
Adie's syndrome is not life threatening or disabling.[1] As such, there is no mortality rate relating to the condition; however, loss of deep tendon reflexes is permanent and may progress over time.[1]
It most commonly affects younger women (2.6:1 female preponderance) and is unilateral in 80% of cases.[3] Average age of onset is 32 years.