Tonic (Adie) Pupil

    • Affected pupil constricts poorly to light and slowly to a near target (“tonic light-near dissociation”)
    • Affected pupil dilates slowly as the patient switches fixation from a near target to a distant target (“tonic redilatation”)
    • Attributed to denervation of the iris by a lesion of the ciliary ganglion or ciliary nerves
    • Common causes: presumed para-infectious dysautonomia
    • Uncommon causes: retinal buckle surgery, retinal photocoagulation, cyclocryotherapy, orbital tumor/surgery/ trauma, Guillain-Barré syndrome, hereditary neuropathies, amyloidosis, pandysautonomia
    • Core clinical features
      • Patient reports abnormal light sensitivity, blurred vision, difficulty focusing, or that the abnormal pupil was detected only when looking in the mirror or incidentally on a medical examination
      • One pupil or both pupils may be affected
      • In dim light, the affected pupil may be larger or smaller than the unaffected pupil
      • Affected pupil constricts minimally to a direct light and slowly to a near target (“tonic light-near dissociation”)
      • Affected pupil dilates slowly as fixation is directed from a reading target to a distant target (“tonic redilatation”)
      • Segmental iris sphincter palsy is visible as an irregular pupil shape and uneven constriction of the iris when the patient views a near target (“stromal streaming”)
    • Possible accompanying clinical features
      • Subtle iris transillumination defects (iris atrophy) visible on slit-lamp examination
      • Reduced accommodation in younger patients, often mild and quickly resolving
      • Reduced corneal sensation in the affected eye
      • Reduced or absent deep tendon reflexes (“Holmes–Adie syndrome”)
      • Peripheral neuropathy, dysautonomia
      • History of retinal buckle/photocoagulation, cyclocryotherapy, orbital tumor/trauma/surgery
    • Pharmacologic mydriasis
    • Third nerve palsy, but other features of third nerve palsy must be present
    • Traumatic, inflammatory, dysplastic iridoplegia
    • Botulism, but...
    • Tip: other features will be present in botulism, including reduced accommodation, diplopia, or epiphora
    • Be sure that there are no ocular ductional deficits, misalignment, or ptosis, which would suggest third nerve palsy
    • Be sure there are no features to suggest a diagnosis of peripheral neuropathy or systemic dysautonomia
    • Look for these pupillary abnormalities (aided by slit lamp examination)
      • Light-near dissociation
      • Slow (tonic) pupil constriction and redilatation
      • Segmental palsy with stromal streaming
    • If clinical features are not diagnostic, instill dilute (0.1%) pilocarpine into both eyes, wait 30 minutes, and look for miosis in the affected eye (“cholinergic denervation supersensitivity”)
    • If no denervation supersensitivity is present, instill 0.5% pilocarpine in the affected eye, wait 30 minutes, and look for lack of constriction of the affected pupil, which would suggest a diagnosis of pharmacologic mydriasis or iridoplegia
    • Prescribe bifocal glasses if the patient has lingering deficient accommodation
    • Caregivers often miss this diagnosis, incorrectly assuming that it reflects third nerve palsy or incipient brain herniation, which elicits unnecessary brain imaging
    • Patients should be reassured and advised of the following prognosis
      • Accommodation will recover fully within weeks
      • The affected pupil will become smaller within months but never regain normal constriction to light
      • If only one eye is affected at presentation, the other eye may become affected within months to years
      • There will be no lingering ophthalmic symptoms or future neurologic or systemic consequences if not already present

    Pupil Disorders

    Anisocoria Overview Physiologic Anisocoria Horner Syndrome Tonic (Adie) Pupil Argyll Robertson Pupil Pharmacologic Mydriasis Episodic Mydriasis Iridoplegic Mydriasis Mydriasis of Third Nerve Palsy Tectal Pupils