Adie syndrome is a neurological disorder characterized by a tonically dilated pupil that reacts slowly to light but shows a more definite response to accommodation.
It is frequently seen in females with absent knee or ankle jerks and impaired sweating.
It most commonly affects younger women (2.6:1 female preponderance.
It is unilateral in 80% of cases.
Average age of onset is 32 years.
Adie pupil 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.
It is the damage to neurons in the ciliary ganglion, located in the posterior orbit, that provides parasympathetic control of eye constriction.
The inflammation affects the pupil of the eye and the autonomic nervous system.
The Adie syndrome has with three hallmark symptoms: at least one abnormally dilated pupil known as mydriasis, which does not constrict in response to light, the loss of deep tendon reflexes, and abnormalities of sweating.
Additionally, signs may include hyperopia due to accommodative paresis, photophobia and difficulty reading.
Adie syndrome patients may also have cardiovascular abnormalities.
Patients with the syndrome can also experience abnormal autonomic control of the body, caused by damage to the dorsal root ganglia of the spinal cord.
Adie’s pupil is supersensitive to acetylcholine.
Pupillary constriction can be early sign of the brainstem herniation.
Testing with low dose pilocarpine may constrict the tonic pupil due to cholinergic denervation supersensitivity.
Abnormal pupil will not constrict with the dilute dose of pilocarpine.
The usual treatment is reading glasses to correct for impairment of the eye.
Pilocarpine drops may be administered as a treatment as well as a diagnostic measure.
Thoracic sympathectomy is the definitive treatment of diaphoresis, if not treatable by drug therapy.
Loss of deep tendon reflexes is permanent and may progress over time.