Adie tonic pupil -CEW

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====== 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 

====References====
  - [[https://pmc.ncbi.nlm.nih.gov/articles/PMC8917782/|Adie's Pupil: A diagnostic challenge of the Physician. Sui-yi Xu et al. Med Sci Monit. 2022. 28:e934657-1-e93457-7]]
  - [[https://pubmed.ncbi.nlm.nih.gov/15582062/|The eye in neurological disease. Miller, Neil R and Newman Nancy J. Lancet. 2004. 364(9450):2045-54]]
  - [[https://pmc.ncbi.nlm.nih.gov/articles/PMC8115311/|Dilute pilocarpine test for diagnosis of Adie's tonic pupil. Yung-Ju Yoo et al.  Sci Rep. 2021. 11L:10089]]
  - [[https://eyewiki.org/Adie_Pupil|Eyewiki: Adie Pupil]]
  - [[https://pubmed.ncbi.nlm.nih.gov/11860969/|Leavitt JA, Wayman LL, Hodge DO, Brubaker RF. Pupillary response to four concentrations of pilocarpine in normal subjects: application to testing for Adie tonic pupil. Am J Ophthalmol. 2002;133:333-336]]