Adie's Tonic Pupil

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Adie's Tonic Pupil
Classification and external resources
ICD-10 H57.0


Disease Entity

Tonic Pupil ICD-10: H57.051 (right), H57.052 (left), H57.053 (bilateral)

Disease

Adie’s Tonic Pupil, also known as Adie’s Syndrome or Holmes-Adie Syndrome, is a disorder of the autonomic nervous system characterized by mydriasis with poor or sluggish pupillary constriction in bright light with slow redilation and decreased deep tendon reflexes.[1] The entity is named after William John Adie, an Australian neurologist who extensively described the features.[2] When paired with segmental anhidrosis it is termed Ross’ Syndrome.[3] Ross syndrome is a triad of progressive segmental impairment of sweating, absent muscle stretch reflexes and tonic pupil.[4]

Etiology

At this time, the underlying etiology of this disease is unknown.

Epidemiology

Adie’s tonic pupil occurs in a 3:1 female to male ratio with an average age of onset of 32 years.[1]

General Pathology

It is thought that either a bacterial or viral infection causes inflammation which damages neurons in the ciliary ganglion of the orbit as well as the dorsal root ganglion in the spinal cord. This damage leads to sectoral denervation of the iris sphincter muscle and poor or sluggish constriction of the pupil in bright light but with normal near response (Light-Near dissociation) and slow redilation. Roughly 80% of cases are unilateral, but can become bilateral (4% chance per year).[5] Damage to the dorsal root ganglion leads to areflexia. These symptoms do not always occur simultaneously, and the onset of areflexia often occurs after the onset of the tonic pupil.

Pathophysiology

Histologic examination of patients with Adie’s Syndrome has shown a reduction of ciliary ganglion cells.[1] The affected short ciliary neurons are postganglionic parasympathetic neurons which synapse within the ciliary ganglion and innervate the iris sphincter muscles. This denervation leads to sectoral palsy of the iris sphincter muscle, stromal thinning, and occasionally iris atrophy.[1] Over time the neurons regenerate allowing for recovery of the near response. A similar process is felt to occur in the dorsal root ganglion of the spinal cord where pre- and post-ganglionic parasympathetic fibers synapse. These fibers provide parasympathetic innervation to various muscles depending on the reflex involved.


Diagnosis

The diagnosis of Adie’s tonic pupil is clinical, based on history and physical exam.

History

The classic history for Adie’s tonic pupil will be a patient, most commonly a woman in her third or fourth decade of life, with symptoms of blurred vision, photophobia, and anisocoria with difficulty reading.

Physical examination

Examination reveals anisocoria greater in light than dark indicating dysfunction of the parasympathetically innervated iris sphincter muscle in the affected eye. The affected pupil will also slowly constrict and preferentially to a near stimulus than to light. Pupil motility can be further tested by having a patient focus on a near target and one should see normal or near normal pupillary constriction. When then asked to focus on a distant target the affected pupil will slowly dilate when transitioning from near to distance vision when compared to the normal eye. On slit lamp exam, sectoral iris paralysis is appreciated as segmental contracture of the iris sphincter seen as vermiform movements of the pupillary margin. Areflexia is often elicited, especially of the Achilles' tendon. Areflexia often does not develop at the same time as the tonic pupil and may not be elicited on first presentation.

Signs

Patients will demonstrate anisocoria greater in light than dark with segmental iris palsies. They may also have areflexia, especially of the Achilles' tendon.

Symptoms

Patients often present with blurry vision often noted especially when transitioning from near to distance vision. Patients also may present with photophobia.[6][7]

Diagnostic procedures

The denervated sphincter muscles can show increased sensitivity to dilute pilocarpine (0.125%) due to up regulation of receptors in about 80% of cases. Dilute pilocarpine can be made by diluting readily available commercial 1% solution with sterile saline. There are studies which show that even normal pupils can react to dilute pilocarpine (0.125%) and that a more dilute solution of 0.0625% or even 0.03125% is more specific for identifying tonic pupils.[8] After 30-60 minutes the affected pupil will constrict more than the normal pupil.

Laboratory test

While the diagnosis of Adie's tonic pupil is clinical, other causes of tonic pupil must be ruled out. Syphilis serologies should be checked, and a thorough history should be obtained for diabetes, trauma, or other neurological diseases such as Charcot-Marie-Tooth disease or Miller-Fisher syndrome.[9][10]

Differential diagnosis

The differential diagnosis for Adie’s tonic pupil includes:

  • Trauma
  • Varicella-zoster
  • Giant cell arteritis
  • Tertiary syphilis
  • Sjӧgren syndrome
  • Diabetes mellitus
  • Chronic alcoholism
  • Amyloidosis
  • Cancer-associated dysautonomia
  • Miller-Fisher syndrome
  • Charcot-Marie-Tooth disease.

Management

General treatment

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

Prognosis

Adie's tonic pupil is not a life threatening disease, but can cause photophobia and blurry vision due to poor iris function. With time, ranging from months to years, the affected pupil often constricts with resolution of most symptoms.[1]

Additional Resources

References

  1. 1.0 1.1 1.2 1.3 1.4 2014-2015 Basic and Clinical Science Course, Section 05: Neuro-Ophthalmology. American Academy of Ophthalmology, 2014.
  2. Siddiqui AA, Clarke JC, Gryzbowski A. William John Adie: the man behind the syndrome. Clinical and Experimental Ophthalmology. March 2014. 42:778-784.
  3. Shin RK, Galetta SL, Ting TY, Armstron K, Bird SJ. Ross syndrome plus: beyond horner, Holmes-Adie, and harlequin. Neurology. December 200. 55:1841-1846.
  4. Tripathy K, Sharma YR, Chawla R, Basu K, Vohra R, Venkatesh P. Triads in Ophthalmology: A Comprehensive Review. Semin Ophthalmol. July 2017;32:237–50.
  5. Kardon RH , CorbettJJ, ThompsonHS. Segmental denervation and reinnervation of the iris sphincter as shown by infrared videographic transillumination.Ophthalmology.1998;105(2):313–321. Thompson HS . Segmental palsy of the iris sphincter in Adie’s syndrome.Arch Ophthalmol.1978;96(9):1615–1620.
  6. Ford PA, Barnes PJ, Usmani OS. Chronic cough and Holmes-Adie syndrome. The Lancet. January 2007 369:342.
  7. Kimber J. Mitchell D, Mathias CJ. Chronic cough in the Holmes-Adie syndrome: association in five cases with autonomic dysfunction. Journal of Neurology, Neurosurgery, and Psychiatry. March 1998. 65:583-586.
  8. Leavitt JA, WaymanLL, 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.
  9. Jivraj I, Johnson M. A rare presentation of neurosyphilis mimicking a unilateral Adie’s tonic pupil. Seminars in Ophthalmology. July 2014. 29:189-191.
  10. Camoriano GD, Kassab J, Suchak A, Gimbel HV. Neurosyphilis Masquerading as an Acute Adie’s Tonic Pupil: Report of a Case. Case Reports in Ophthalmology. May 2011. 2:205-210.