{{short description|Neurological disorder}} {{Infobox medical condition (new) | name = Adie's syndrome | synonyms = Holmes–Adie syndrome, Adie's tonic pupil, Holmes–Adie pupil | pronounce = {{IPAc-en|ˈ|eɪ|d|i}} | field = | image = Adies.png | caption = Bilateral mydriasis given the observational diagnosis Adie's pupils by an ophthalmologist | symptoms = | complications = | #nset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}
'''Adie syndrome''', also known as '''Holmes–Adie syndrome''', is a [[neurological disorder]] characterized by a [[Tonic (physiology)|tonic]]ally [[Mydriasis|dilated]] pupil that reacts slowly to light but shows a more definite response to [[Accommodation reflex| accommodation]] (i.e., light-near dissociation).<ref name=ninds>{{cite web |url=http://www.ninds.nih.gov/disorders/holmes_adie/holmes_adie.htm |title=Holmes-Adie syndrome Information Page |publisher=[[National Institute of Neurological Disorders and Stroke]] |access-date=2008-01-21 |archive-url=https://web.archive.org/web/20071016162629/http://ninds.nih.gov/disorders/holmes_adie/holmes_adie.htm |archive-date=2007-10-16 }}</ref> It is frequently seen in females with absent [[Patellar reflex|knee]] or [[ankle jerk reflex|ankle jerks]] and impaired sweating.
The syndrome is caused by damage to the [[postganglionic fiber]]s of the [[parasympathetic nervous system|parasympathetic innervation]] of the eye, usually by a [[virus|viral]] or [[bacterial]] infection that causes [[inflammation]], and affects the [[pupil]] of the eye and the [[autonomic nervous system]].<ref name="ninds" /> It is named after the British neurologists [[William John Adie]] and [[Gordon Morgan Holmes]], who independently described the same disease in 1931.<ref name="pmid24533698">{{cite journal|vauthors=Siddiqui AA, Clarke JC, Grzybowski A|date=November 2014|title=William John Adie: the man behind the syndrome|url=http://discovery.ucl.ac.uk/1470479/1/Siddiqui%20et%20al.%20William%20John%20Adie.pdf|journal=Clinical & Experimental Ophthalmology|volume=42|issue=8|pages=778–84|doi=10.1111/ceo.12301|pmid=24533698|doi-access=free}}</ref>
==Signs and symptoms== Adie syndrome presents with three hallmark symptoms, namely at least one abnormally dilated pupil ([[mydriasis]]) which does not constrict in response to light, loss of deep tendon reflexes, and abnormalities of sweating.<ref name=ninds/> Other signs may include [[hyperopia]] due to accommodative [[paresis]], [[photophobia]] and difficulty reading.<ref name=stedman>{{cite book | title = Stedman's Medical Dictionary | edition = 27th | year = 2000 | publisher = Lippincott Williams & Wilkins | url = https://archive.org/details/stedmansmedicald00sted_3 | isbn = 978-0-683-40007-6 }}</ref> Some individuals with Adie syndrome may also have [[cardiovascular abnormalities]].<ref>{{cite web|url=https://rarediseases.info.nih.gov/diseases/5749/adie-syndrome|title=Adie syndrome|work=Genetic and Rare Diseases Information Center (GARD) – an NCATS Program|access-date=2018-04-17|archive-date=2021-03-19|archive-url=https://web.archive.org/web/20210319091549/https://rarediseases.info.nih.gov/diseases/5749/adie-syndrome}}</ref>
==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]], located in the posterior orbit, that provides [[parasympathetic nervous system|parasympathetic control]] of eye constriction. Additionally, patients with Holmes-Adie Syndrome can also experience problems with [[autonomic nervous system|autonomic control]] of the body. This second set of symptoms is caused by damage to the [[dorsal root ganglia]] of the [[spinal cord]]. Adie's pupil is supersensitive to [[acetylcholine]] so a muscarinic agonist (e.g. [[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.<ref name=ninds/>
==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".<ref name=fundneuro>{{cite book | last = Haines | first = Duane E. | name-list-style = vanc | title = Fundamental Neuroscience, 2nd edition | year = 2002 | publisher = Churchill Livingstone | url = https://archive.org/details/fundamentalneuro0002unse | isbn = 978-0-443-06603-0 }}</ref> Testing with low dose (1/8%) [[pilocarpine]] may constrict the tonic pupil due to [[cholinergic]] [[denervation supersensitivity]].<ref name=ninds/> A normal pupil will not constrict with the [[Concentration|dilute]] dose of pilocarpine.<ref name=fundneuro/> [[CT Scan|CT scans]] and [[MRI]] scans may be useful in the diagnostic testing of [[Focal dystonia|focal]] hypoactive reflexes.<ref name=wrong>{{cite web |url= http://www.wrongdiagnosis.com/a/adie_syndrome/diagnosis.htm |title= Diagnosis of Adie syndrome WrongDiagnosis.com |access-date=2008-01-21 }}</ref>
==Treatment== The usual treatment of a standardised Adie syndrome is to prescribe reading glasses to correct for impairment of the eye(s).<ref name=ninds/> [[Pilocarpine]] drops may be administered as a treatment as well as a diagnostic measure.<ref name=ninds/> [[Thoracic sympathectomy]] is the definitive treatment of [[diaphoresis]], if the condition is not treatable by [[drug therapy]].<ref name=ninds/>
==Prognosis== Adie's syndrome is not life-threatening or disabling.<ref name=ninds/> As such, there is no mortality rate relating to the condition; however, loss of deep tendon reflexes is permanent and may progress over time.<ref name=ninds/>
== Epidemiology == It most commonly affects younger women (2.6:1 female preponderance) and is unilateral in 80% of cases.<ref name=fundneuro/> Average age of onset is 32 years.<ref name="y793">{{cite journal | last=Thompson | first=H S | title=Adie's syndrome: some new observations. | journal=Transactions of the American Ophthalmological Society | publisher=American Ophthalmological Society | volume=75 | date=1977 | pages=587–626 | pmid=613531 | pmc=1311565 }}</ref>
== See also == * [[Ciliary ganglion]] * [[Ross' syndrome]]
== References == {{reflist}}
== Further reading == {{refbegin}} * {{cite journal | vauthors = Estañol B, Callejas-Rojas RC, Cortés S, Martínez-Memije R, Infante-Vázquez O, Delgado-García G | title = Asymptomatic Severe Vagal and Sympathetic Cardiac Denervation in Holmes-Adie's Syndrome | journal = Case Reports in Neurological Medicine | volume = 2017 | article-number = 4919758 | date = 2017 | pmid = 28428900 | pmc = 5385912 | doi = 10.1155/2017/4919758 | doi-access = free }} {{refend}}
== External links == {{Medical resources | DiseasesDB = 29742 | ICD10 = {{ICD10|H|57|0}} | ICD9 = {{ICD9|379.46}} | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = D015845 }}
{{Eye pathology}}
[[Category:Neurological disorders]] [[Category:Syndromes affecting the eye]] [[Category:Syndromes affecting the nervous system]] [[Category:Human pupil]]