Adie syndrome

December 31, 2014

Synonyms

Overview

Adie syndrome 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 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. It is characterized by a tonically dilated pupil. Most cases of Adie Syndrome are not progressive, life threatening, or disabling.

Symptoms

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.

Causes

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. In these cases, it appears to follow an autosomal dominant pattern of inheritance.

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.

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.

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.