# Adie syndrome

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Neurological disorder

Medical condition

Adie's syndrome Other names Holmes–Adie syndrome, Adie's tonic pupil, Holmes–Adie pupil Bilateral mydriasis given the observational diagnosis Adie's pupils by an ophthalmologist Pronunciation /ˈeɪdi/ Specialty Ophthalmology

**Adie syndrome**, also known as **Holmes–Adie syndrome**, is a [neurological disorder](/source/Neurological_disorder) characterized by a [tonically](/source/Tonic_(physiology)) [dilated](/source/Mydriasis) pupil that reacts slowly to light but shows a more definite response to [accommodation](/source/Accommodation_reflex) (i.e., light-near dissociation).[1] It is frequently seen in females with absent [knee](/source/Patellar_reflex) or [ankle jerks](/source/Ankle_jerk_reflex) and impaired sweating.

The syndrome is caused by damage to the [postganglionic fibers](/source/Postganglionic_fiber) of the [parasympathetic innervation](/source/Parasympathetic_nervous_system) of the eye, usually by a [viral](/source/Virus) or [bacterial](/source/Bacterial) infection that causes [inflammation](/source/Inflammation), and affects the [pupil](/source/Pupil) of the eye and the [autonomic nervous system](/source/Autonomic_nervous_system).[1] It is named after the British neurologists [William John Adie](/source/William_John_Adie) and [Gordon Morgan Holmes](/source/Gordon_Morgan_Holmes), who independently described the same disease in 1931.[2]

## Signs and symptoms

Adie syndrome presents with three hallmark symptoms, namely at least one abnormally dilated pupil ([mydriasis](/source/Mydriasis)) which does not constrict in response to light, loss of deep tendon reflexes, and abnormalities of sweating.[1] Other signs may include [hyperopia](/source/Hyperopia) due to accommodative [paresis](/source/Paresis), [photophobia](/source/Photophobia) and difficulty reading.[3] Some individuals with Adie syndrome may also have [cardiovascular abnormalities](/source/Cardiovascular_abnormalities).[4]

## Pathophysiology

Pupillary symptoms of Holmes–Adie syndrome are thought to be the result of a viral or bacterial infection that causes inflammation and damage to neurons in the [ciliary ganglion](/source/Ciliary_ganglion), located in the posterior orbit, that provides [parasympathetic control](/source/Parasympathetic_nervous_system) of eye constriction. Additionally, patients with Holmes-Adie Syndrome can also experience problems with [autonomic control](/source/Autonomic_nervous_system) of the body. This second set of symptoms is caused by damage to the [dorsal root ganglia](/source/Dorsal_root_ganglia) of the [spinal cord](/source/Spinal_cord). Adie's pupil is supersensitive to [acetylcholine](/source/Acetylcholine) so a muscarinic agonist (e.g. [pilocarpine](/source/Pilocarpine)) whose dose would not be able to cause pupillary constriction in a normal patient, would cause it in a patient with Adie's Syndrome. The circuitry for the pupillary constriction does not descend below the upper midbrain, henceforth impaired pupillary constriction is extremely important to detect as it can be an early sign of brainstem herniation.[1]

## Diagnosis

Clinical exam may reveal sectoral paresis of the iris sphincter or vermiform iris movements. The tonic pupil may become smaller (miotic) over time which is referred to as "little old Adie's".[5] Testing with low dose (1/8%) [pilocarpine](/source/Pilocarpine) may constrict the tonic pupil due to [cholinergic](/source/Cholinergic) [denervation supersensitivity](/source/Denervation_supersensitivity).[1] A normal pupil will not constrict with the [dilute](/source/Concentration) dose of pilocarpine.[5] [CT scans](/source/CT_Scan) and [MRI](/source/MRI) scans may be useful in the diagnostic testing of [focal](/source/Focal_dystonia) hypoactive reflexes.[6]

## Treatment

The usual treatment of a standardised Adie syndrome is to prescribe reading glasses to correct for impairment of the eye(s).[1] [Pilocarpine](/source/Pilocarpine) drops may be administered as a treatment as well as a diagnostic measure.[1] [Thoracic sympathectomy](/source/Thoracic_sympathectomy) is the definitive treatment of [diaphoresis](/source/Diaphoresis), if the condition is not treatable by [drug therapy](/source/Drug_therapy).[1]

## Prognosis

Adie's syndrome is not life-threatening or disabling.[1] As such, there is no mortality rate relating to the condition; however, loss of deep tendon reflexes is permanent and may progress over time.[1]

## Epidemiology

It most commonly affects younger women (2.6:1 female preponderance) and is unilateral in 80% of cases.[5] Average age of onset is 32 years.[7]

## See also

- [Ciliary ganglion](/source/Ciliary_ganglion)

- [Ross' syndrome](/source/Ross'_syndrome)

## References

1. ^ [***a***](#cite_ref-ninds_1-0) [***b***](#cite_ref-ninds_1-1) [***c***](#cite_ref-ninds_1-2) [***d***](#cite_ref-ninds_1-3) [***e***](#cite_ref-ninds_1-4) [***f***](#cite_ref-ninds_1-5) [***g***](#cite_ref-ninds_1-6) [***h***](#cite_ref-ninds_1-7) [***i***](#cite_ref-ninds_1-8) [***j***](#cite_ref-ninds_1-9) ["Holmes-Adie syndrome Information Page"](https://web.archive.org/web/20071016162629/http://ninds.nih.gov/disorders/holmes_adie/holmes_adie.htm). [National Institute of Neurological Disorders and Stroke](/source/National_Institute_of_Neurological_Disorders_and_Stroke). Archived from [the original](http://www.ninds.nih.gov/disorders/holmes_adie/holmes_adie.htm) on 2007-10-16. Retrieved 2008-01-21.

1. **[^](#cite_ref-pmid24533698_2-0)** Siddiqui AA, Clarke JC, Grzybowski A (November 2014). ["William John Adie: the man behind the syndrome"](http://discovery.ucl.ac.uk/1470479/1/Siddiqui%20et%20al.%20William%20John%20Adie.pdf) (PDF). *Clinical & Experimental Ophthalmology*. **42** (8): 778–84. [doi](/source/Doi_(identifier)):[10.1111/ceo.12301](https://doi.org/10.1111%2Fceo.12301). [PMID](/source/PMID_(identifier)) [24533698](https://pubmed.ncbi.nlm.nih.gov/24533698).

1. **[^](#cite_ref-stedman_3-0)** [*Stedman's Medical Dictionary*](https://archive.org/details/stedmansmedicald00sted_3) (27th ed.). Lippincott Williams & Wilkins. 2000. [ISBN](/source/ISBN_(identifier)) [978-0-683-40007-6](https://en.wikipedia.org/wiki/Special:BookSources/978-0-683-40007-6).

1. **[^](#cite_ref-4)** ["Adie syndrome"](https://web.archive.org/web/20210319091549/https://rarediseases.info.nih.gov/diseases/5749/adie-syndrome). *Genetic and Rare Diseases Information Center (GARD) – an NCATS Program*. Archived from [the original](https://rarediseases.info.nih.gov/diseases/5749/adie-syndrome) on 2021-03-19. Retrieved 2018-04-17.

1. ^ [***a***](#cite_ref-fundneuro_5-0) [***b***](#cite_ref-fundneuro_5-1) [***c***](#cite_ref-fundneuro_5-2) Haines DE (2002). [*Fundamental Neuroscience, 2nd edition*](https://archive.org/details/fundamentalneuro0002unse). Churchill Livingstone. [ISBN](/source/ISBN_(identifier)) [978-0-443-06603-0](https://en.wikipedia.org/wiki/Special:BookSources/978-0-443-06603-0).

1. **[^](#cite_ref-wrong_6-0)** ["Diagnosis of Adie syndrome WrongDiagnosis.com"](http://www.wrongdiagnosis.com/a/adie_syndrome/diagnosis.htm). Retrieved 2008-01-21.

1. **[^](#cite_ref-y793_7-0)** Thompson, H S (1977). ["Adie's syndrome: some new observations"](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1311565). *Transactions of the American Ophthalmological Society*. **75**. American Ophthalmological Society: 587–626. [PMC](/source/PMC_(identifier)) [1311565](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1311565). [PMID](/source/PMID_(identifier)) [613531](https://pubmed.ncbi.nlm.nih.gov/613531).

## Further reading

- Estañol B, Callejas-Rojas RC, Cortés S, Martínez-Memije R, Infante-Vázquez O, Delgado-García G (2017). ["Asymptomatic Severe Vagal and Sympathetic Cardiac Denervation in Holmes-Adie's Syndrome"](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5385912). *Case Reports in Neurological Medicine*. **2017** 4919758. [doi](/source/Doi_(identifier)):[10.1155/2017/4919758](https://doi.org/10.1155%2F2017%2F4919758). [PMC](/source/PMC_(identifier)) [5385912](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5385912). [PMID](/source/PMID_(identifier)) [28428900](https://pubmed.ncbi.nlm.nih.gov/28428900).

## External links

Classification D ICD-10: H57.0 ICD-9-CM: 379.46 MeSH: D015845 DiseasesDB: 29742

v t e Diseases of the human eye Adnexa Eyelid Inflammation Stye Chalazion Blepharitis Meibomian gland dysfunction Entropion Ectropion Lagophthalmos Blepharochalasis Ptosis Blepharophimosis Xanthelasma Ankyloblepharon Eyelash Trichiasis Madarosis Distichiasis Trichomegaly Lacrimal apparatus Dacryoadenitis Epiphora Dacryocystitis Xerophthalmia Orbit Exophthalmos Enophthalmos Orbital cellulitis Orbital lymphoma Periorbital cellulitis Conjunctiva Chemosis Conjunctivitis allergic Pterygium Pseudopterygium Pinguecula Subconjunctival hemorrhage Globe Fibrous tunic Sclera Scleritis Episcleritis Cornea Keratitis herpetic acanthamoebic fungal Exposure Photokeratitis Corneal ulcer Thygeson's superficial punctate keratopathy Corneal dystrophy Fuchs' Meesmann Corneal ectasia Keratoconus Pellucid marginal degeneration Keratoglobus Terrien's marginal degeneration Post-LASIK ectasia Keratoconjunctivitis sicca Corneal opacity Corneal neovascularization Kayser–Fleischer ring Haab's striae Arcus senilis Band keratopathy Vascular tunic Iris Ciliary body Uveitis Intermediate uveitis Hyphema Rubeosis iridis Persistent pupillary membrane Iridodialysis Synechia Choroid Choroideremia Choroiditis Chorioretinitis Focal choroidal excavation Polypoidal choroidal vasculopathy Lens Cataract Congenital cataract Childhood cataract Aphakia Ectopia lentis Retina Retinitis Chorioretinitis Cytomegalovirus retinitis Retinal detachment Posterior vitreous detachment Retinoschisis Ocular ischemic syndrome / Central retinal vein occlusion Central retinal artery occlusion Branch retinal artery occlusion Retinopathy diabetic hypertensive Purtscher's of prematurity Bietti's crystalline dystrophy Coats' disease Sickle cell photic Macular degeneration Retinitis pigmentosa Retinal haemorrhage Central serous retinopathy Macular edema Epiretinal membrane (Macular pucker) Vitelliform macular dystrophy Leber's congenital amaurosis Birdshot chorioretinopathy Other Glaucoma / Ocular hypertension / Primary juvenile glaucoma Floater Leber's hereditary optic neuropathy Ocular hypotony Red eye Globe rupture Keratomycosis Phthisis bulbi Persistent fetal vasculature Persistent tunica vasculosa lentis Familial exudative vitreoretinopathy Vogt-Koyanagi-Harada disease Pathways Optic nerve Optic disc Optic neuritis optic papillitis Papilledema Foster Kennedy syndrome Optic atrophy Optic disc drusen Optic neuropathy Ischemic anterior (AION) posterior (PION) arteritic anterior (AAION or arteritic AION) non-arteritic anterior (NAION) Kjer's Leber's hereditary Toxic and nutritional Strabismus Extraocular muscles Binocular vision Accommodation Paralytic strabismus Ophthalmoparesis Chronic progressive external ophthalmoplegia Kearns–Sayre syndrome palsies Oculomotor (III) Fourth-nerve (IV) Sixth-nerve (VI) Other strabismus Esotropia / Exotropia Hypertropia Heterophoria Esophoria Exophoria Cyclotropia Brown's syndrome Duane syndrome Other binocular Conjugate gaze palsy Convergence insufficiency Internuclear ophthalmoplegia One and a half syndrome Refraction Refractive error Hyperopia Myopia Astigmatism Anisometropia / Aniseikonia Presbyopia Vision disorders Blindness Amblyopia Leber's congenital amaurosis Diplopia Scotoma Color blindness Achromatopsia Dichromacy Monochromacy Nyctalopia Oguchi disease Blindness / Vision loss / Visual impairment Anopsia Hemianopsia binasal bitemporal homonymous Quadrantanopia subjective Asthenopia Hemeralopia Photophobia Scintillating scotoma Pupil Anisocoria Argyll Robertson pupil Marcus Gunn pupil Adie syndrome Miosis Mydriasis Cycloplegia Parinaud's syndrome Other Nystagmus Childhood blindness Infections Trachoma Onchocerciasis

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Adapted from the Wikipedia article [Adie syndrome](https://en.wikipedia.org/wiki/Adie_syndrome) by Wikipedia contributors ([contributor history](https://en.wikipedia.org/wiki/Adie_syndrome?action=history)). Available under [Creative Commons Attribution-ShareAlike 4.0 International](https://creativecommons.org/licenses/by-sa/4.0/). Changes may have been made.