Adie's Tonic Pupil
|Adie's Tonic Pupil|
|Classification and external resources|
- 1 Disease Entity
- 2 Diagnosis
- 3 Management
- 4 Additional Resources
- 5 References
Tonic Pupil ICD-10: H57.051 (right), H57.052 (left), H57.053 (bilateral)
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. The entity is named after William John Adie, an Australian neurologist who extensively described the features. When paired with segmental anhidrosis it is termed Ross’ Syndrome. Ross syndrome is a triad of progressive segmental impairment of sweating, absent muscle stretch reflexes and tonic pupil.
At this time, the underlying etiology of this disease is unknown.
Adie’s tonic pupil occurs in a 3:1 female to male ratio with an average age of onset of 32 years.
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). 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.
Histologic examination of patients with Adie’s Syndrome has shown a reduction of ciliary ganglion cells. 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. 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.
The diagnosis of Adie’s tonic pupil is clinical, based on history and physical exam.
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.
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.
Patients will demonstrate anisocoria greater in light than dark with segmental iris palsies. They may also have areflexia, especially of the Achilles' tendon.
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. After 30-60 minutes the affected pupil will constrict more than the normal pupil.
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.
The differential diagnosis for Adie’s tonic pupil includes:
- Giant cell arteritis
- Tertiary syphilis
- Sjӧgren syndrome
- Diabetes mellitus
- Chronic alcoholism
- Cancer-associated dysautonomia
- Miller-Fisher syndrome
- Charcot-Marie-Tooth disease.
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.
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.
- American Academy of Ophthalmology. Neuro-ophthalmology/Orbit: Adie pupil Practicing Ophthalmologists Learning System, 2017 - 2019 San Francisco: American Academy of Ophthalmology, 2017.
- 2014-2015 Basic and Clinical Science Course, Section 05: Neuro-Ophthalmology. American Academy of Ophthalmology, 2014.
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- Jivraj I, Johnson M. A rare presentation of neurosyphilis mimicking a unilateral Adie’s tonic pupil. Seminars in Ophthalmology. July 2014. 29:189-191.
- 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.