Adie Tonic Pupil

A benign condition caused by postganglionic parasympathetic denervation of the iris sphincter and ciliary muscle, probably from ciliary ganglion or short ciliary nerve damage (viral or inflammatory).

Typical patient: young woman aged 20 to 40, unilateral in 80 percent. Later bilateral involvement in about 4 percent per year.

Adie syndrome: tonic pupil with absent deep tendon reflexes (knees and ankles). Associated autonomic features: segmental hypohidrosis (Ross syndrome), orthostatic hypotension, chronic cough.

Other causes of a dilated poorly reactive pupil: third nerve palsy, pharmacological mydriasis, iris sphincter trauma, angle closure.

• A large pupil that reacts poorly to light, with a tonic (slow, sustained) constriction to near and slow redilation.
• Light near dissociation, with vermiform (worm like) movements of the iris border on slit lamp examination, due to segmental sphincter paralysis.
• Accommodation paresis: blurred near vision, photophobia, and difficulty shifting focus; loss of accommodation recovers with a tonic pattern.
• Over months to years, the large pupil becomes smaller (miotic) and the tonic accommodation recovers; the pupil may become the smaller of two.
• No ptosis, no ocular motility deficit.

• Slit lamp: sectoral iris sphincter palsy with vermiform movements; compare pupil size in light and dark and after a sustained near effort.
• Dilute pilocarpine test: 0.1 percent pilocarpine (or 0.125 percent) instilled in both eyes; the tonic pupil constricts (denervation hypersensitivity, constriction of 0.5 mm or more beyond the normal eye) while the normal pupil and a pharmacologically dilated or third nerve pupil do not. A full strength 1 percent pilocarpine test distinguishes pharmacological dilatation (no constriction) from tonic pupil (constricts).
• Check deep tendon reflexes, blood pressure lying and standing, sweat testing if suspicion of Ross syndrome.
• Exclude third nerve palsy (ptosis, motility), orbital or ciliary ganglion mass (MRI if atypical), syphilis serology (rapid plasma reagin, treponemal test) for Argyll Robertson type pupils, and herpes zoster history.

• Reassure; the condition is benign and not sight threatening, and imaging is not needed when features are typical.
• Near blur and accommodation: reading glasses with an add of +1.00 to +3.00 dioptres, sometimes in the affected eye only.
• Photophobia and glare: tinted or photochromic lenses; pilocarpine 0.0625 to 0.125 percent drops, 1 drop up to 3 or 4 times a day, to constrict the pupil and relieve accommodation spasm (use with caution, may cause brow ache and blur).
• Autonomic dysfunction (orthostatic hypotension in Ross or Adie syndrome): fluids, compression stockings, fludrocortisone 0.1 mg daily.
• Segmental hypohidrosis: avoid overheating; hyperhidrosis in compensatory regions may need topical aluminium chloride.
• Review annually; bilateral conversion is possible.