| Adie's syndrome | |
|---|---|
| Other names | Holmes–Adie syndrome, Adie's tonic pupil, Holmes–Adie pupil |
 | |
| Bilateral mydriasis given the observational diagnosis Adie's pupils by an ophthalmologist | |
| Pronunciation | |
| Specialty | Ophthalmology  |
Adie syndrome, also known asHolmes–Adie syndrome, is aneurological disorder characterized by atonicallydilated pupil that reacts slowly to light but shows a more definite response to accommodation (i.e., light-near dissociation).[1] It is frequently seen in females with absentknee orankle jerks and impaired sweating.
The syndrome is caused by damage to thepostganglionic fibers of theparasympathetic innervation of the eye, usually by aviral orbacterial infection that causesinflammation, and affects thepupil of the eye and theautonomic nervous system.[1] It is named after the British neurologistsWilliam John Adie andGordon Morgan Holmes, who independently described the same disease in 1931.[2]
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 abnormalities of sweating.[1] Other signs may includehyperopia due to accommodativeparesis,photophobia and difficulty reading.[3] Some individuals with Adie syndrome may also havecardiovascular abnormalities.[4]
Pupillary symptoms of Holmes–Adie syndrome are thought to be the result of a viral or bacterial infection that causes inflammation and damage to neurons in theciliary ganglion, located in the posterior orbit, that providesparasympathetic control of eye constriction. Additionally, patients with Holmes-Adie Syndrome can also experience problems withautonomic control of the body. This second set of symptoms is caused by damage to thedorsal root ganglia of thespinal cord. Adie's pupil is supersensitive toacetylcholine so a muscarinic agonist (e.g.pilocarpine) whose dose would not be able to cause pupillary constriction in a normal patient, would cause it in a patient with Adie's Syndrome. The circuitry for the pupillary constriction does not descend below the upper midbrain, henceforth impaired pupillary constriction is extremely important to detect as it can be an early sign of brainstem herniation.[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".[5] Testing with low dose (1/8%)pilocarpine may constrict the tonic pupil due tocholinergicdenervation supersensitivity.[1] A normal pupil will not constrict with thedilute dose of pilocarpine.[5]CT scans andMRI scans may be useful in the diagnostic testing offocal hypoactive reflexes.[6]
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 ofdiaphoresis, if the condition is not treatable bydrug 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.[5] Average age of onset is 32 years.[7]