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
Characteristics
- 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
Causes
- 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
Testing
- 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%)
Prognosis
- 25% get progressive smaller pupil with parasympathetic reinnervation