Adie’s Syndrome

Adie’s Syndrome
SYMPTOMS Photophobia, Blurry vision (especially at near), Headaches The symptoms should improve or resolve in the course of a few months
SIGNS Typically unilateral, especially at onset
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.
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
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)
Pupil involvement with progressive loss of deep tendon reflexes is called Adie’s syndrome.
Ross syndrome is Adie’s pupil + loss of deep tendon reflexes + segmental anhidrosis.
WORK-UP Pupils | EOMs | Full eye exam with dilation | Ptosis testing | Cranial nerve testing | Pharmacologic testing
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.
TREATMENT May need to give a temporary add or bifocal for blur at near until symptoms resolve
Usually a benign condition so no further treatment needed but if suspecting a systemic etiology, refer to PCP for additional lab testing
FOLLOW-UP Monitor in 1 month to re-evaluate pupils and follow-up on symptoms
ADDITIONAL LAB | TESTS 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)
ETIOLOGY Caused by damage to the parasympathetic innervation to the ciliary ganglion along the postganglionic pathway.
Most common etiology is idiopathic but can also be caused by various infections, inflammatory disorders, diabetes, giant cell arteritis, trauma, and surgery
DIFFERENTIAL DX Third nerve palsy with pupil involvement, Pharmacologic block, Horner’s syndrome, Argyll Robertson pupil, Physiologic anisocoria
NOTES Females are much more likely to be affected by Adie’s syndrome (about 2.6:1) than males
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)
Chronic coughing can be associated with Adie’s syndrome
The most common reflex affected by Adie’s syndrome is the Achilles tendon reflex
Ross syndrome involves abnormalities of sweating as well
If a child presents with Adie’s pupil, Riley-Day syndrome must be ruled out