CASE REPORT


A 40-year-old healthy man presented with a 4-month history of photophobia, blurred vision, and a right dilated pupil. Examination revealed a right pupil that was not reactive to light but constricted strongly to a near target and slowly redilated when he looked back in the distance.


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Adie tonic pupil, Adie’s Syndrome, Holmes-Adie Syndrome

Pharmacological testing with dilute pilocarpine 0.1% resulted in constriction of the right pupil but no change in the left pupil. This also resulted in the resolution of his photophobia and blurry vision.

Neurological examination was otherwise normal, and a diagnosis of Adie’s tonic pupil was made.

DISEASE


Adie tonic pupil, known as Adie’s Syndrome or Holmes-Adie Syndrome, is a disorder in which there is parasympathetic denervation of the afflicted pupil resulting in poor light but better and tonic near constriction.

The affected pupil, either unilateral or bilateral typically initially appears abnormally dilated at rest and has a poor or sluggish pupillary constriction in bright light.

Constriction is typically more notable with the near reaction and typically remains tonically constricted with slow re-dilation with segmental paralysis of the iris sphincter. Patients may also present with decreased deep tendon reflexes in the full Holmes-Adie Syndrome.

Adie tonic pupil, Adie’s Syndrome, Holmes-Adie Syndrome

MANAGEMENT


Adie tonic pupil is a benign condition and generally patients only require reassurance. However, patients may experience photophobia and blurry vision. Accommodative paresis may resolve with time, ranging from months to years.

However, patients may experience increased light-near dissociation over time since the pupil’s reaction to light does not typically recover.

While in general treatment is not required for Adie tonic pupil, dilute topical pilocarpine or physostigmine can be used for symptomatic relief in cases with severe photophobia.

It must be noted that these medications can precipitate ciliary spasms, and brow aches, worsen anisocoria, or induce nearsightedness. In patients with persistent accommodative paresis, frosted bifocal segments may be used to correct vision.

If an underlying systemic cause for the tonic pupil is suspected, patients should have treatment directed toward their systemic neuropathies.

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REFERENCES


  1.  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. American Journal of Ophthalmology. March 2002. 133:333-336.
  2.  Adie WJ. Pseudo-Argyll Robertson Pupils With Absent Tendon Reflexes: A Benign Disorder Simulating Tabes Dorsalis. Br Med J 1931; 1:928.
  3.  Morgan OG, Symonds CP. Internal Ophthalmoplegia with Absent Tendon-jerks. Proc R Soc Med 1931; 24:867.
  4.  Holmes G. Partial iridoplegia with symptoms of other diseases of the nervous system. Trans Ophthalmol Soc UK 1931; 51:209.
  5.  Thompson HS. Adie’s syndrome: some new observations. Trans Am Ophthalmol Soc 1977; 75:587.

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