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SYMPTOMS
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Photophobia, Blurry vision (especially at near), Headaches The symptoms should improve or resolve in the course of a few months
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SIGNS
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Typically unilateral, especially at onset
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Acute signs: Dilated pupil, Anisocoria greater in light than dark, Initial loss of the direct light response and near response with a quick (few weeks) reinnervation of the near response more than the light response. This creates a light-near dissociation.
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Slow sustained constriction with near response, Segmental contractions of the iris sphincter which gives the pupil vermiform-like movement when it constricts, Slow pupil re-dilation
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As the Adie’s pupil becomes chronic, the affected dilated pupil will be more miotic and could even become smaller than the unaffected eye (reverse anisocoria)
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Pupil involvement with progressive loss of deep tendon reflexes is called Adie’s syndrome.
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Ross syndrome is Adie’s pupil + loss of deep tendon reflexes + segmental anhidrosis.
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WORK-UP
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Pupils | EOMs | Full eye exam with dilation | Ptosis testing | Cranial nerve testing | Pharmacologic testing
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Pilocarpine 0.125% drop: The affected pupil will constrict but the normal pupil will not. Can often see a reverse anisocoria if the affected pupil is still larger than the normal pupil. Need to wait for 30-60 minutes to see results. Occurs due to cholinergic denervation supersensitivity.
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TREATMENT
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May need to give a temporary add or bifocal for blur at near until symptoms resolve
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Usually a benign condition so no further treatment needed but if suspecting a systemic etiology, refer to PCP for additional lab testing
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FOLLOW-UP
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Monitor in 1 month to re-evaluate pupils and follow-up on symptoms
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ADDITIONAL LAB | TESTS
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Ordering additional lab tests is based on suspicion of an underlying systemic etiology: HbA1c | Fasting blood sugar | VDRL/RPR | FTA-ABS/MHA-TA/T | Pallidum-particle agglutination | ESR (especially in older patients) | C-reactive protein (especially in older patients)
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ETIOLOGY
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Caused by damage to the parasympathetic innervation to the ciliary ganglion along the postganglionic pathway.
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Most common etiology is idiopathic but can also be caused by various infections, inflammatory disorders, diabetes, giant cell arteritis, trauma, and surgery
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DIFFERENTIAL DX
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Third nerve palsy with pupil involvement, Pharmacologic block, Horner’s syndrome, Argyll Robertson pupil, Physiologic anisocoria
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NOTES
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Females are much more likely to be affected by Adie’s syndrome (about 2.6:1) than males
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About 20% of Adie’s pupils are bilateral upon onset (If unilateral upon onset, there is a rate of 4% bilateral involvement with each successive year)
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Chronic coughing can be associated with Adie’s syndrome
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The most common reflex affected by Adie’s syndrome is the Achilles tendon reflex
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Ross syndrome involves abnormalities of sweating as well
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If a child presents with Adie’s pupil, Riley-Day syndrome must be ruled out
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