Adie syndrome

Adie syndrome, sometimes known as Holmes-Adie's syndrome or Adie's Tonic Pupil, is a neurological disorder which affects the pupil of the eye and the autonomic nervous system. 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 characterized by a tonically dilated pupil.

Signs and Symptoms
Adie syndrome presents with three hallmark symptoms, namely abnormal pupil size, loss of deep tendon reflexes and diaphoresis. Other signs may include hyperopia due to accommodative paresis, photophobia and difficulty reading.

Diagnosis
Clinical exam may reveal sectoral paresis of the iris sphincter and/or vermiform iris movements. The tonic pupil may become smaller (miotic) over time which is referred to as "little old Adie's". Testing with low dose (1-8%) pilocarpine may constrict the tonic pupil due to cholinergic denervation supersensitivity. A normal pupil will not constrict with the dilute dose of pilocarpine. CT scans and MRI scans may be useful in the diagnostic testing of focal hypoactive reflexes.

Treatment
The usual treatment of a standardised Adie syndrome is to prescribe reading glasses to correct for impairment of the eye(s). Pilocarpine drops may be administered as a treatment as well as a diagnostic measure, and should be applied three times daily. Thoracic sympathectomy is the definitive treatment of diaphoresis, if the condition is not treatable by drug therapy.

Prognosis
Adie's syndrome is not life threatening or disabling. As such, there is no mortality rate relating to the condition, however loss of deep tendon reflexes is permanent and may progress over time.

Epidemiology
It most commonly affects younger women and is unilateral in 80% of cases.