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Adie (Tonic) Pupil

ROUTINE — Signs

Symptoms

  • Difference in pupil size (anisocoria) noticed by patient or others.
  • Blurred near vision due to accommodative paresis.
  • Photophobia.
  • Often asymptomatic.

Clinical Findings

Demographics & presentation

  • Classic patient: Young, healthy adult women (ages 20–40).
  • Presentation: Acute or subacute unilateral mydriasis; up to 20% become bilateral over time.

Pupil exam

  • Large (mydriatic) and irregular pupil with minimal to no direct light reflex.
  • Vermiform (worm-like) movements: Pathognomonic sectoral palsy of the iris sphincter visible at the slit lamp.

Near response & hallmark

  • Light-near dissociation: Poor response to light but present (though slow) response to near.
  • Tonic behavior: Slow, sustained constriction with accommodation and very slow redilation when returning to distance viewing.

Pharmacologic testing

  • Denervation supersensitivity: Instill dilute pilocarpine (0.125% or 0.1%) in both eyes.
  • Adie pupil constricts briskly within 10–15 minutes; the normal pupil does not react.

Natural history

  • "Little old Adie's": Over years, the pupil progressively becomes smaller (miotic) due to reinnervation and supersensitivity.
  • Accommodative paresis: Common at onset; requires assessment for a temporary reading add.

Systemic associations

  • Holmes-Adie syndrome: Tonic pupil + diminished or absent deep-tendon reflexes (usually lower extremity).
  • Ross syndrome: Tonic pupil + absent reflexes + segmental anhidrosis (patchy loss of sweating).

Etiology

Mechanism

  • Postganglionic parasympathetic denervation caused by damage to the ciliary ganglion or short ciliary nerves.

Causes

  • Idiopathic (most common): Presumed post-viral inflammation.
  • Secondary: Orbital trauma, orbital surgery, or varicella zoster virus (VZV).
  • Rare: Early syphilis, paraneoplastic syndromes, giant cell arteritis (GCA), sarcoidosis, Sjögren syndrome, or neurologic Lyme disease.

Red Flags

  • Sudden unilateral mydriasis without ptosis or diplopia suggests a benign Adie pupil.
  • Presence of ptosis or extraocular muscle weakness suggests a CN III palsy (requires urgent imaging for aneurysm).
  • Bilateral simultaneous tonic pupils in a young patient warrant an autonomic neuropathy or paraneoplastic workup.
  • Tonic pupil in an older adult or with constitutional symptoms requires investigation for GCA or malignancy.

Investigations

Clinical assessment

  • Slit-lamp exam to identify vermiform sectoral iris palsy.
  • Assessment of tonic near response and light-near dissociation.
  • Systemic check: Test deep-tendon reflexes.

Pharmacologic confirmation

  • Dilute pilocarpine test (0.125%): Constriction of the affected pupil confirms the diagnosis.

Systemic workup (if atypical)

  • Unilateral Adie in a young patient: No further testing; reassurance.
  • Bilateral or atypical cases: Consider testing for syphilis (RPR/FTA-ABS), sarcoidosis (ACE), Sjögren syndrome, or paraneoplastic markers.

Treatment

General management

  • Reassurance: Most cases are benign and require no intervention.
  • Near-vision blur: Prescribe a reading add if accommodative paresis is symptomatic.

Symptomatic relief

  • Photophobia: Dilute pilocarpine 0.125% BID can be used to constrict the pupil.
  • Tinted lenses: May help with glare.

Referrals

  • Neurology referral: Indicated for bilateral cases, autonomic symptoms (Ross syndrome), or atypical neurologic findings.

Clinical Pearl

A large tonic pupil with light-near dissociation in a young woman is almost always Adie's. Confirm with dilute (0.125%) pilocarpine and check deep-tendon reflexes for Holmes-Adie syndrome.

Differential Diagnosis

  • CN III palsy (presents with ptosis and motility deficits)
  • Pharmacologic mydriasis (does not react to 1% pilocarpine)
  • Argyll Robertson pupils (typically bilateral, miotic, and associated with neurosyphilis)
  • Dorsal midbrain (Parinaud) syndrome (associated with upgaze palsy and convergence-retraction nystagmus)

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