Adie tonic pupil

Damage to the postganglionic parasympathetic neurons in the ciliary ganglion or short ciliary nerves
Characterized by denervation supersensitivity of the iris sphincter muscle

Anisocoria worse in bright light (poor iris sphincter constriction)
Most often affects young to middle-aged women (average onset 32 years, prevalence estimated at 0.02%)
Unilateral in 80%
Light-near dissociation
- dysinnervation of the iris sphincter parasympathetic innervation is present but recovery of the accommodative iris sphincter input occurs
- slow tonic constriction to near fixation (light/near dissociation) and slow redilation
Segmental iris sphincter paresis produces vermiform movements

Idiopathic
Infectious
- HSV, VZV, Syphilis
Autoimmune/Inflammatory
- Lupus
- Sjögren syndrome
- Guillian-Barré syndrome
- Miller-Fisher syndrome
Autonomic neuropathy
- Acute Pandysautonomia
- Shy-Drager syndrome
- Ross syndrome ( tonic pupil + hyporeflexia +segmental anhidrosis)
Metabolic/Toxic
- Diabetes
- Chronic alcoholism
- toxic neuropathies
  - botulism
Paraneoplastic
- Small-cell lung carcinoma (ANNA-1/anti-Hu)
- Hodgkin lymphoma
- testicular seminoma
Ocular trauma
Hereditary
- Spinocerebellar ataxia
- hereditary neuropathy

Dilute pilocarpine test
- Pilocarpine 0.0625%-0.125% causes constriction of a tonic pupil with no effect on a normal pupil
- One study of 0.0625% pilocarpine showed 100% sensitivity and 82.8% specificity.
- Because this response is due to supersensitvity, it may not be present within 2 months of onset
- False positive results in pre ganglionic CN III lesions.
Dilute Pilocarpine 1% in solution with sterile saline
- 1 part pilocarpine 1% and 9 parts saline gives 0.1%)