|Classification and external resources|
Adie’s Tonic Pupil (or Holmes-Adie Syndrome) is a neurological disorder that causes one or both pupils to be abnormally dilated (mydriasis) with delayed constriction in response to exposure to light. Patients commonly present with areflexia of the knee and/or ankles in addition to tonic pupil.
Pupil constriction and dilation is controlled by both the parasympathetic and sympathetic components of the autonomic nervous system. In the case of tonic pupil this is due to a disorder of the parasympathetic nervous system. Specifically, tonic pupil is caused by loss of postganglionic parasympathetic innervation to the iris sphincter and ciliary muscle. Causes of this denervation include viral infection, trauma, vasospasm due to migraine, ocular surgery, tumors, and possibly chronic cough, among other potential causes.
Adie’s Tonic Pupil is a benign idiopathic syndrome. However, mydriasis can also be caused by a more general neuropathy or pharmacologic blockade.
Common clinical findings associated with Adie’s syndrome beyond mydriasis include hypersensitivity to muscarinic receptor agonists (e.g. pilocarpine), reduced tendon reflexes, and occasionally hypohidrosis (this is a variant called Ross syndrome). Any history or findings indicating a more general neuropathy, such as history of Diabetic Autonomic Neuropathy, could suggest an etiology of the mydriasis other than Adie’s Tonic Pupil. One suggested method for distinguishing between these two possible causes is examination for anisocoria and sector palsy. Anisocoria >1mm and presence of sector palsy on slit-lamp examination indicate Adie’s Tonic Pupil versus general neuropathy causation of the mydriasis with 90% specificity. However, there still may be an underlying neuropathy unrelated to the causation of the mydriasis in this case.
Rule-out of pharmacologic blockade may be performed through a detailed medication history.
Historically, Adie’s Tonic Pupil has been considered a benign, idiopathic syndrome that does not necessitate ocular imaging or further evaluation. However, recent evidence suggests that ongoing infectious, inflammatory, malignant, or autoimmune pathology are potential causes. While these infectious or malignant pathologies have been discovered concurrent with Adie’s Tonic Pupil, it is unknown if they are causal, and comorbidity is very rare.
Pilocarpine drops can be applied multiple times per day in order to constrict the pupil. Prescription reading glasses may be necessary to correct impaired vision.
- American Academy of Ophthalmology. Neuro-ophthalmology/Orbit: Adie pupil Practicing Ophthalmologists Learning System, 2017 - 2019 San Francisco: American Academy of Ophthalmology, 2017.
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- Kawasaki A. Physiology, assessment, and disorders of the pupil (1999)
- Ibrahm M., et al. A case of Horner Syndrome with intermittent mydriasis in a patient with hypoplasia of the internal carotid artery. Am J Neuroradiol (2006) 6, 1318-1320
- Bremner F. and Smith SE. Bilateral tonic pupils: Holmes–Adie syndrome or generalised neuropathy? Br J Ophthalmol (2007) 12, 1620-1623
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- National Institute of Neurological Disorders and Stroke, NINDS Holmes-Adie Syndrome Information Page, www.ninds.gov/disorders/holmes_adie (2011)