Adie syndrome: Difference between revisions

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==Overview==
==Overview==
[[Adie syndrome]] is characterized by abnormal pupillary function and loss of deep tendon reflexes. Abnormal pupillary functioning in [[adie syndrome]] consists of poor pupillary response to light but a better [[near response]] ([[light near dissociation]]). Damage to [[ciliary ganglion]] is responsible for pupillary abnormalities while the loss of [[deep tendon reflexes]] is caused by damage to [[dorsal root ganglion]] of [[spinal cord]]. Most of the times the cause of [[Adie syndrome]] is unknown, however, multiple pathologies can cause [[adie syndrome]]. It is a benign condition and is mostly diagnosed clinically. Management is mostly symptom directed and depends upon the underlying etiology


==Historical Perspective==
==Historical Perspective==
*[[Adie] syndrome]] was first discovered by [[William John Adie]], a [[British]] [[neurologist]], and [[Sir Gordon Morgan Holmes]], an [[Irish]] [[neurologist]] in 1931. This [[syndrome]] was named after these 2 [[neurologists]]. <ref name="pmid613531">{{cite journal |vauthors=Thompson HS |title=Adie's syndrome: some new observations |journal=Transactions of the American Ophthalmological Society |volume=75 |issue= |pages=587–626 |date=1977 |pmid=613531 |pmc=1311565 |doi= |url= |issn=}}</ref>
*[[Adie] syndrome]] was first discovered by [[William John Adie]], a [[British]] [[neurologist]], and [[Sir Gordon Morgan Holmes]], an [[Irish]] [[neurologist]] in 1931. This [[syndrome]] was named after these 2 [[neurologists]]. <ref name="pmid613531">{{cite journal |vauthors=Thompson HS |title=Adie's syndrome: some new observations |journal=Transactions of the American Ophthalmological Society |volume=75 |issue= |pages=587–626 |date=1977 |pmid=613531 |pmc=1311565 |doi= |url= |issn=}}</ref>
*In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].


==Classification==
==Classification==
*[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
*[[Adie syndrome]] has a variant known as [[Ross syndrome]], characterized by a triad of abnormal [[pupillary function]], loss of [[deep tendon reflexes]] and [[sweating abnormalities]]. <ref name="urlAdie Syndrome - NORD (National Organization for Rare Disorders)">{{cite web |url=https://rarediseases.org/rare-diseases/adie-syndrome/#:~:text=Adie%20syndrome%2C%20or%20Holmes%2DAdie,also%20associated%20with%20this%20disorder. |title=Adie Syndrome - NORD (National Organization for Rare Disorders) |author= |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>
:*[group1]
 
:*[group2]
:*[group3]
*Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
==Pathophysiology==
==Pathophysiology==
*The pathogenesis of [[adie syndrome]] is characterized by damage to [[ciliary ganglion]] and [[dorsal root ganglion]] of [[spinal cord]]. [[Ciliary ganglion]] provides innervation to [[iris]] and [[ciliary muscle]], thus controlling [[pupillary function]] and [[accommodation]]. [[Ciliary ganglion]] [[neurons]] responsible for [[accommodation]] are in a much greater number than the [[neurons]] controlling [[pupillary function]]. In [[adie syndrome]], [[neurons]]/[[nerve fibers]] controlling both [[accommodation]] and [[pupillary function]] are damaged. But because of the large no. of [[neruons]], [[accommodation]] is unaffected or minimally affected in [[adie syndrome]]. Some of the damaged [[nerve fibers]] start to [[regenerate]]. As nerve fibers innervating [[pupillary sphincter muscle]] are less in no. so very few of these fibers [[regenerate]] and are insufficient to restore the normal [[pupillary function]]. On the other hand, [[nerve fibers]] to the [[ciliary muscle]] also [[regenerate]] but may do so in an inappropriate fashion, providing [[innervation]] to [[pupillary sphincter muscle]] along with [[ciliary muscle]]. As the [[pupillary sphincter muscle]] is now innervated by fibers originally responsible for [[accommodation]](which is a slower process than [[pupillary light reaction]]) so [[pupillary light response]] is impaired. <ref name="urlAdie Syndrome (Tonic Pupil Syndrome) Article - StatPearls">{{cite web |url=https://www.statpearls.com/kb/viewarticle/17207#ref_21610838 |title=Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls |author= Sarao MS, Elnahry AG, Sharma S |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>  
*The pathogenesis of [[adie syndrome]] is characterized by damage to [[ciliary ganglion]] and [[dorsal root ganglion]] of [[spinal cord]]. [[Ciliary ganglion]] provides innervation to [[iris]] and [[ciliary muscle]], thus controlling [[pupillary function]] and [[accommodation]]. [[Ciliary ganglion]] [[neurons]] responsible for [[accommodation]] are in a much greater number than the [[neurons]] controlling [[pupillary function]]. In [[adie syndrome]], [[neurons]]/[[nerve fibers]] controlling both [[accommodation]] and [[pupillary function]] are damaged. But because of the large no. of [[neruons]], [[accommodation]] is unaffected or minimally affected in [[adie syndrome]]. Some of the damaged [[nerve fibers]] start to [[regenerate]]. As nerve fibers innervating [[pupillary sphincter muscle]] are less in no. so very few of these fibers [[regenerate]] and are insufficient to restore the normal [[pupillary function]]. On the other hand, [[nerve fibers]] to the [[ciliary muscle]] also [[regenerate]] but may do so in an inappropriate fashion, providing [[innervation]] to [[pupillary sphincter muscle]] along with [[ciliary muscle]]. As the [[pupillary sphincter muscle]] is now innervated by fibers originally responsible for [[accommodation]](which is a slower process than [[pupillary light reaction]]) so [[pupillary light response]] is impaired. <ref name="urlAdie Syndrome (Tonic Pupil Syndrome) Article - StatPearls">{{cite web |url=https://www.statpearls.com/kb/viewarticle/17207#ref_21610838 |title=Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls |author= Sarao MS, Elnahry AG, Sharma S |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>  
*Loss of [[deep tendon reflexes]] in [[adie syndrome]] is caused by damage to [[dorsal root ganglio]] of [[spinal cord]]. <ref name="urlAdie Syndrome (Tonic Pupil Syndrome) Article - StatPearls">{{cite web |url=https://www.statpearls.com/kb/viewarticle/17207#ref_21610838 |title=Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls |author= Sarao MS, Elnahry AG, Sharma S |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>   
*Loss of [[deep tendon reflexes]] in [[adie syndrome]] is caused by damage to [[dorsal root ganglion]] of [[spinal cord]]. <ref name="urlAdie Syndrome (Tonic Pupil Syndrome) Article - StatPearls">{{cite web |url=https://www.statpearls.com/kb/viewarticle/17207#ref_21610838 |title=Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls |author= Sarao MS, Elnahry AG, Sharma S |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>   
*On microscopic histopathological analysis, [[degeneration]] of [[ciliary ganglion]] and [[atrophy]] of [[sphincter pupillae muscle]], are characteristic findings of [[adie syndrome]]. Other findings associated with [[adie syndrome]] include degenration of [[sacral dorsal root ganglion]], [[superior cervical ganglion]] and [[sciatic nerve]]. <ref name="pmid3668764">{{cite journal |vauthors=Agbeja AM, Dutton GN |title=Adie's syndrome as a cause of amblyopia |journal=Journal of Pediatric Ophthalmology and Strabismus |volume=24 |issue=4 |pages=176–7 |date=1987 |pmid=3668764 |doi= |url= |issn=}}</ref>
*On microscopic histopathological analysis, [[degeneration]] of [[ciliary ganglion]] and [[atrophy]] of [[sphincter pupillae muscle]], are characteristic findings of [[adie syndrome]]. Other findings associated with [[adie syndrome]] include degenration of [[sacral dorsal root ganglion]], [[superior cervical ganglion]] and [[sciatic nerve]]. <ref name="pmid3668764">{{cite journal |vauthors=Agbeja AM, Dutton GN |title=Adie's syndrome as a cause of amblyopia |journal=Journal of Pediatric Ophthalmology and Strabismus |volume=24 |issue=4 |pages=176–7 |date=1987 |pmid=3668764 |doi= |url= |issn=}}</ref>


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* The annual incidence of [[adie syndrome]] is estimated to be [4.7] cases per 100,000 individuals. <ref name="urlAdie Syndrome (Tonic Pupil Syndrome) Article - StatPearls">{{cite web |url=https://www.statpearls.com/kb/viewarticle/17207#ref_21610838 |title=Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls |author= Sarao MS, Elnahry AG, Sharma S |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>
* The annual incidence of [[adie syndrome]] is estimated to be [4.7] cases per 100,000 individuals. <ref name="urlAdie Syndrome (Tonic Pupil Syndrome) Article - StatPearls">{{cite web |url=https://www.statpearls.com/kb/viewarticle/17207#ref_21610838 |title=Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls |author= Sarao MS, Elnahry AG, Sharma S |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>
   
   
===Age===
===Age===  
*[[Adie syndrome]] is more commonly observed among patients aged [[25 to 45]] years old. <ref name="urlAdie Syndrome (Tonic Pupil Syndrome) Article - StatPearls">{{cite web |url=https://www.statpearls.com/kb/viewarticle/17207#ref_21610838 |title=Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls |author= Sarao MS, Elnahry AG, Sharma S |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>
*[[Adie syndrome]] is more commonly observed among patients aged [[25 to 45]] years old. <ref name="urlAdie Syndrome (Tonic Pupil Syndrome) Article - StatPearls">{{cite web |url=https://www.statpearls.com/kb/viewarticle/17207#ref_21610838 |title=Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls |author= Sarao MS, Elnahry AG, Sharma S |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>


===Gender===  
===Gender===
*[[Females]] are more commonly affected with [[Adie pupil]] than [[males]]. <ref name="urlAdie Syndrome (Tonic Pupil Syndrome) Article - StatPearls">{{cite web |url=https://www.statpearls.com/kb/viewarticle/17207#ref_21610838 |title=Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls |author= Sarao MS, Elnahry AG, Sharma S |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>
*[[Females]] are more commonly affected with [[Adie pupil]] than [[males]]. <ref name="urlAdie Syndrome (Tonic Pupil Syndrome) Article - StatPearls">{{cite web |url=https://www.statpearls.com/kb/viewarticle/17207#ref_21610838 |title=Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls |author= Sarao MS, Elnahry AG, Sharma S |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>


===Race===
===Race===
*There is no racial predilection for [disease name].
*There is no racial predilection for [[adie syndrome]].


==Risk Factors==
==Risk Factors==
*Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
*Common risk factors in the development of [[adie syndrome]] are [[infections]], [[trauma]], [[tumors]], [[ischemia]] and [[autoimmune disorders]].


== Natural History, Complications and Prognosis==  
== Natural History, Complications and Prognosis==  
*Early clinical features include [[accommodative paresis]], [[impaired light reflex]] and [[loss of deep tendone reflexes]]. [[Accomodative paresis]] tends to improve overtime, however, impairment of [[pupillary light reaction]] and [[deep tendon reflexes]]is persistent and in some cases may worsen overtime.   
*Early clinical features include [[accommodative paresis]], [[impaired light reflex]] and [[loss of deep tendon reflexes]]. [[Accomodative paresis]] tends to improve overtime, however, impairment of [[pupillary light reaction]] and [[deep tendon reflexes]] is persistent and in some cases may worsen overtime.  <ref name="urlAdie Syndrome (Tonic Pupil Syndrome) Article - StatPearls">{{cite web |url=https://www.statpearls.com/kb/viewarticle/17207#ref_21610838 |title=Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls |author= Sarao MS, Elnahry AG, Sharma S |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>
*Complications of [[adie syndrome]] include [[angle closure glaucoma]] and [[amblyopia]]. However, they occur very rarely.
*Complications of [[adie syndrome]] include [[angle closure glaucoma]] and [[amblyopia]]. However, they occur very rarely.<ref name="pmid11901294">{{cite journal |vauthors=Leibovitch I, Kurtz S, Almog Y |title=Adie's tonic pupil-induced angle-closure glaucoma |journal=Ophthalmologica. Journal International D'ophtalmologie. International Journal of Ophthalmology. Zeitschrift Fur Augenheilkunde |volume=216 |issue=1 |pages=71–2 |date=2002 |pmid=11901294 |doi=10.1159/000048302 |url= |issn=}}</ref><ref name="pmid3668764">{{cite journal |vauthors=Agbeja AM, Dutton GN |title=Adie's syndrome as a cause of amblyopia |journal=Journal of Pediatric Ophthalmology and Strabismus |volume=24 |issue=4 |pages=176–7 |date=1987 |pmid=3668764 |doi= |url= |issn=}}</ref>
*Prognosis is generally good. [[Adie syndrome]] has no associated mortality rate as it is a nonlife-threatening condition.
*Prognosis is generally good. [[Adie syndrome]] has no associated mortality rate as it is a nonlife-threatening condition. <ref name="urlAdie Syndrome (Tonic Pupil Syndrome) Article - StatPearls">{{cite web |url=https://www.statpearls.com/kb/viewarticle/17207#ref_21610838 |title=Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls |author= Sarao MS, Elnahry AG, Sharma S |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>


== Diagnosis ==
== Diagnosis ==
===Diagnostic Criteria===
===Diagnostic Criteria===
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
*The diagnosis of [[adie syndrome] is made when both pupillary abnormalities and absence or impairment of [[deep tendon reflexes]] are present. Although, both of these features may not coexist. If only pupillary abnormalities are present then the condition is referred to as [[adie pupil]]. <ref name="urlAdie Syndrome - NORD (National Organization for Rare Disorders)">{{cite web |url=https://rarediseases.org/rare-diseases/adie-syndrome/#:~:text=Adie%20syndrome%2C%20or%20Holmes%2DAdie,also%20associated%20with%20this%20disorder. |title=Adie Syndrome - NORD (National Organization for Rare Disorders) |author= |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>
:*[criterion 1]
 
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]
=== History and Symptoms ===
=== History and Symptoms ===
*Symptoms of [[adie syndrome]] may include the following:
*Symptoms of [[adie syndrome]] may include the following:<ref name="pmid613531">{{cite journal |vauthors=Thompson HS |title=Adie's syndrome: some new observations |journal=Transactions of the American Ophthalmological Society |volume=75 |issue= |pages=587–626 |date=1977 |pmid=613531 |pmc=1311565 |doi= |url= |issn=}}</ref> <ref name="pmid9758294">{{cite journal |vauthors=Wilhelm H |title=Neuro-ophthalmology of pupillary function--practical guidelines |journal=Journal of Neurology |volume=245 |issue=9 |pages=573–83 |date=September 1998 |pmid=9758294 |doi=10.1007/s004150050248 |url= |issn=}}</ref>
:*[[larger pupil size of one eye than the other eye]]
:*[[larger pupil size of one eye than the other eye]]
:*[[sweating abnormalities]]
:*[[sweating abnormalities]]
:*[[reading difficulty]]
:*[[reading difficulty]]
:*[[Photophobia]]
:*[[Photophobia]]
:*[symptom 5]
:*[symptom 6]


=== Physical Examination ===
=== Physical Examination ===
*Physical examination may be remarkable for:
*Physical examination may be remarkable for:<ref name="pmid613531">{{cite journal |vauthors=Thompson HS |title=Adie's syndrome: some new observations |journal=Transactions of the American Ophthalmological Society |volume=75 |issue= |pages=587–626 |date=1977 |pmid=613531 |pmc=1311565 |doi= |url= |issn=}}</ref><ref name="pmid12214264">{{cite journal |vauthors=Emond D, Lebel M |title=Orthostatic hypotension and Holmes-Adie syndrome. Usefulness of the Valsalva ratio in the evaluation of baroreceptor dysfunction |journal=Journal of Human Hypertension |volume=16 |issue=9 |pages=661–2 |date=September 2002 |pmid=12214264 |doi=10.1038/sj.jhh.1001455 |url= |issn=}}</ref><ref name="pmid9758294">{{cite journal |vauthors=Wilhelm H |title=Neuro-ophthalmology of pupillary function--practical guidelines |journal=Journal of Neurology |volume=245 |issue=9 |pages=573–83 |date=September 1998 |pmid=9758294 |doi=10.1007/s004150050248 |url= |issn=}}</ref>
:*absent or impaired [[pupillary light reflex]]
:*absent or impaired [[pupillary light reflex]]
:*absent [[deep tendon reflexes]] (most commonly [[achilles tendon]] is involved).
:*absent [[deep tendon reflexes]] (most commonly [[achilles tendon]] is involved).
Line 88: Line 75:
:*[[orthostatic hypotension]]
:*[[orthostatic hypotension]]
:*[[hypersensitivity to cholinergic agonists]]
:*[[hypersensitivity to cholinergic agonists]]
*[[Ross syndrome]] is a variant of adie syndrome characterized by triad of [[tonic pupil]], [[absent deep tendon reflexes]], [[abnormal sweating]].


=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].
*There are no specific laboratory findings associated with [[adie syndrome]].


*A  [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
===Electrocardiogram===
===Electrocardiogram===
There are no ECG findings associated with [disease name].
There are no ECG findings associated with [[adie syndrome]].
 
OR
 
An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].


===X-ray===
===X-ray===
There are no x-ray findings associated with [disease name].
There are no x-ray findings associated with [[adie syndrome]].
 
OR
 
An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===Echocardiography or Ultrasound===
===Echocardiography or Ultrasound===
There are no echocardiography/ultrasound findings associated with [disease name].
There are no echocardiography/ultrasound findings associated with [[adie syndrome]].
 
OR
 
Echocardiography/ultrasound  may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no echocardiography/ultrasound  findings associated with [disease name]. However, an echocardiography/ultrasound  may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===CT scan===
===CT scan===
There are no CT scan findings associated with [disease name].
There are no CT scan findings associated with [[adie syndrome]].
 
OR
 
[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===MRI===
===MRI===
There are no MRI findings associated with [disease name].
There are no MRI findings associated with [[adie syndrome]].
 
OR
 
[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===Other Imaging Findings===
===Other Imaging Findings===
There are no other imaging findings associated with [disease name].
There are no other imaging findings associated with [[adie syndrome]].
 
OR
 
[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].


===Other Diagnostic Studies===
===Other Diagnostic Studies===
[[Low dose pilocarpine test]] may be helpful in the diagnosis [[adie syndrome]]. Finding suggestive of [[adie pupil]] includes an exaggerated miotic reaction of the affected [[pupil]] as compared to the normal [[pupil]]. This exaggerated response to low dose [[pilocarpine]] occurs as a consequence of [[cholinergic denervation hypersensitivity]] of the affected [[pupil]]. Normal [[pupils]] do not respond to such low doses of [[pilocarpine]]. To rule out Ross syndrome, sweating abnormalities can be checked by using [[starch iodine test]] and [[spoon test]].
[[Low dose pilocarpine test]] may be helpful in the diagnosis of [[adie syndrome]]. Finding suggestive of [[adie pupil]] includes an exaggerated miotic reaction of the affected [[pupil]] as compared to the normal [[pupil]]. This exaggerated response to low dose [[pilocarpine]] occurs as a consequence of [[cholinergic denervation hypersensitivity]] of the affected [[pupil]]. Normal [[pupils]] do not respond to such low doses of [[pilocarpine]].<ref name="pmid11860969">{{cite journal |vauthors=Leavitt JA, Wayman LL, Hodge DO, Brubaker RF |title=Pupillary response to four concentrations of pilocarpine in normal subjects: application to testing for Adie tonic pupil |journal=American Journal of Ophthalmology |volume=133 |issue=3 |pages=333–6 |date=March 2002 |pmid=11860969 |doi=10.1016/s0002-9394(01)01420-9 |url= |issn=}}</ref> To rule out Ross syndrome, sweating abnormalities can be checked by using [[starch iodine test]] and [[spoon test]]. <ref name="pmid16268880">{{cite journal |vauthors=Ballestero-Díez M, García-Río I, Daudén E, Corrales-Arroyo M, García-Díez A |title=Ross syndrome, an entity included within the spectrum of partial disautonomic syndromes |journal=Journal of the European Academy of Dermatology and Venereology : JEADV |volume=19 |issue=6 |pages=729–31 |date=November 2005 |pmid=16268880 |doi=10.1111/j.1468-3083.2005.01254.x |url= |issn=}}</ref>


== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*There is no treatment required for [[idiopathic adie syndrome]]. If any etiology is found, treatment should be directed against that etiology. Topical low dose [[pilocarpine]] or [[physostigmine]] can be used in symptomatic patients. For patients with [[accommodative paresis]] [[reading glasses]] should be prescribed.
*There is no treatment required for [[idiopathic adie syndrome]]. If any etiology is found, treatment should be directed against that etiology. Topical low dose [[pilocarpine]] or [[physostigmine]] can be used in symptomatic patients. For patients with [[accommodative paresis]] [[reading glasses]] should be prescribed.<ref name="urlHolmes-Adie Syndrome Information Page | National Institute of Neurological Disorders and Stroke">{{cite web |url=https://www.ninds.nih.gov/Disorders/All-Disorders/Holmes-Adie-Syndrome-Information-Page |title=Holmes-Adie Syndrome Information Page &#124; National Institute of Neurological Disorders and Stroke |author= |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref>
 
=== Surgery ===
=== Surgery ===
*[[Thoracic sympathectomy]] can be performed for patients with excessive [[sweating]].
*[[Thoracic sympathectomy]] can be performed for patients with excessive [[sweating]].<ref name="pmid14746734">{{cite journal |vauthors=Serra Mitjans M, Callejas Pérez MA, Valls Solé J, Grimalt Santacana R, Rubio Garay M, Iglesias Sentís M |title=[Surgical treatment for compensatory hyperhidrosis in Adie syndrome] |language=Spanish; Castilian |journal=Archivos De Bronconeumologia |volume=40 |issue=2 |pages=97–9 |date=February 2004 |pmid=14746734 |doi= |url= |issn=}}</ref>
 
=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for [disease name].
*There are no primary preventive measures available for [[adie syndrome]].
*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
 
*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].


==References==
==References==

Latest revision as of 04:03, 20 November 2020


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Muneeb, MBBS[2] Synonyms and keywords: Holmes-Adie Syndrome; Syndrome, Holmes-Adie; Syndrome, Adie's; Syndrome, Adie; Poorly Reacting Pupil; Holmes Adie Syndrome; Pupil, Poorly Reacting; Adie's Syndrome; Poorly Reacting Pupils; Pupils, Poorly Reacting

Overview

Adie syndrome is characterized by abnormal pupillary function and loss of deep tendon reflexes. Abnormal pupillary functioning in adie syndrome consists of poor pupillary response to light but a better near response (light near dissociation). Damage to ciliary ganglion is responsible for pupillary abnormalities while the loss of deep tendon reflexes is caused by damage to dorsal root ganglion of spinal cord. Most of the times the cause of Adie syndrome is unknown, however, multiple pathologies can cause adie syndrome. It is a benign condition and is mostly diagnosed clinically. Management is mostly symptom directed and depends upon the underlying etiology

Historical Perspective

Classification

Pathophysiology

Causes

Most commonly the cause of Adie syndrome is unknown(idiopathic). Less common causes of adie syndrome include infections like HIV[5], syphilis[6], varicella, lyme's disease[7], Human parvovirus-B19, autoimmune diseases like amyloidosis, sarcoidosis, guillain-barre syndrome, sjogren syndrome, polyarterities nodosa[8], vogt-koyanagi-haraga disease[9], ischemia caused by giant cell arteritis[10], migraine[11], lymphatoid granulomatosis, neuromuscular diseases like Lambert eaten syndrome[12], tumors affecting the orbit or choroid[13], orbital surgery[14], cardiovascular diseases[15], general anesthesia[16]. ,anti-hu antibody[17].

Differentiating adie syndrome from other Diseases

Epidemiology and Demographics

  • The prevalence of adie syndrome is approximately 2 per 1000 individuals. [3]
  • The annual incidence of adie syndrome is estimated to be [4.7] cases per 100,000 individuals. [3]

Age

Gender

Race

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

  • The diagnosis of [[adie syndrome] is made when both pupillary abnormalities and absence or impairment of deep tendon reflexes are present. Although, both of these features may not coexist. If only pupillary abnormalities are present then the condition is referred to as adie pupil. [2]

History and Symptoms

Physical Examination

Laboratory Findings

  • There are no specific laboratory findings associated with adie syndrome.

Electrocardiogram

There are no ECG findings associated with adie syndrome.

X-ray

There are no x-ray findings associated with adie syndrome.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with adie syndrome.

CT scan

There are no CT scan findings associated with adie syndrome.

MRI

There are no MRI findings associated with adie syndrome.

Other Imaging Findings

There are no other imaging findings associated with adie syndrome.

Other Diagnostic Studies

Low dose pilocarpine test may be helpful in the diagnosis of adie syndrome. Finding suggestive of adie pupil includes an exaggerated miotic reaction of the affected pupil as compared to the normal pupil. This exaggerated response to low dose pilocarpine occurs as a consequence of cholinergic denervation hypersensitivity of the affected pupil. Normal pupils do not respond to such low doses of pilocarpine.[24] To rule out Ross syndrome, sweating abnormalities can be checked by using starch iodine test and spoon test. [25]

Treatment

Medical Therapy

Surgery

Prevention

  • There are no primary preventive measures available for adie syndrome.

References

  1. ↑ 1.0 1.1 1.2 Thompson HS (1977). "Adie's syndrome: some new observations". Transactions of the American Ophthalmological Society. 75: 587–626. PMC 1311565. PMID 613531.
  2. ↑ 2.0 2.1 "Adie Syndrome - NORD (National Organization for Rare Disorders)".
  3. ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Sarao MS, Elnahry AG, Sharma S. "Adie Syndrome (Tonic Pupil Syndrome) Article - StatPearls".
  4. ↑ 4.0 4.1 Agbeja AM, Dutton GN (1987). "Adie's syndrome as a cause of amblyopia". Journal of Pediatric Ophthalmology and Strabismus. 24 (4): 176–7. PMID 3668764.
  5. ↑ Cerny R, Rozsypal H, Kozner P, Machala L (October 2010). "Bilateral Holmes-Adie syndrome as an early manifestation of the HIV neuropathy". Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology. 31 (5): 661–3. doi:10.1007/s10072-010-0355-9. PMID 20567990.
  6. ↑ Sakai T, Shikishima K, Mizobuchi T, Yoshida M, Kitahara K (2003). "Bilateral tonic pupils associated with neurosyphilis". Japanese Journal of Ophthalmology. 47 (4): 368–71. doi:10.1016/s0021-5155(03)00058-3. PMID 12842205.
  7. ↑ Stricker RB, Winger EE (March 2001). "Holmes-Adie syndrome and Lyme disease". Lancet (London, England). 357 (9258): 805. doi:10.1016/S0140-6736(05)71234-4. PMID 11254002.
  8. ↑ Bennett JL, Pelak VA, Mourelatos Z, Bird S, Galetta SL (1999). "Acute sensorimotor polyneuropathy with tonic pupils and an abduction deficit: an unusual presentation of polyarteritis nodosa". Survey of Ophthalmology. 43 (4): 341–4. doi:10.1016/s0039-6257(98)00047-2. PMID 10025516.
  9. ↑ Garza Leon M, Herrera-Jimenez IP, González-Madrigal PM (August 2014). "Complete Vogt-Koyanagi-Harada disease and Holmes-Adie syndrome: case report". Ocular Immunology and Inflammation. 22 (4): 336–40. doi:10.3109/09273948.2013.848906. PMID 24215593.
  10. ↑ Foroozan R, Buono LM, Savino PJ, Sergott RC (April 2003). "Tonic pupils from giant cell arteritis". The British Journal of Ophthalmology. 87 (4): 510–2. doi:10.1136/bjo.87.4.510. PMC 1771609. PMID 12642330.
  11. ↑ Purvin VA (March 1995). "Adie's tonic pupil secondary to migraine". Journal of Neuro-ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society. 15 (1): 43–4. PMID 7780572.
  12. ↑ Wirtz PW, de Keizer RJ, de Visser M, Wintzen AR, Verschuuren JJ (March 2001). "Tonic pupils in Lambert-Eaton myasthenic syndrome". Muscle & Nerve. 24 (3): 444–5. doi:10.1002/1097-4598(200103)24:3<444::aid-mus1021>3.0.co;2-w. PMID 11353435.
  13. ↑ Goldstein SM, Liu GT, Edmond JC, Katowitz JA, Rorke LB (February 2002). "Orbital neural-glial hamartoma associated with a congenital tonic pupil". Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus. 6 (1): 54–5. doi:10.1067/mpa.2002.120171. PMID 11907481.
  14. ↑ Stromberg BV, Knibbe M (November 1988). "Anisocoria following reduction of bilateral orbital floor fractures". Annals of Plastic Surgery. 21 (5): 486–8. doi:10.1097/00000637-198811000-00016. PMID 3232939.
  15. ↑ Guaraldi P, Mathias CJ (September 2011). "Progression of cardiovascular autonomic dysfunction in Holmes-Adie syndrome". Journal of Neurology, Neurosurgery, and Psychiatry. 82 (9): 1046–9. doi:10.1136/jnnp.2009.195917. PMID 20562402.
  16. ↑ Kobayashi M, Takenami T, Kimotsuki H, Mukuno K, Hoka S (February 2008). "Adie syndrome associated with general anesthesia". Canadian Journal of Anaesthesia = Journal Canadien D'anesthesie. 55 (2): 130–1. doi:10.1007/BF03016329. PMID 18245077.
  17. ↑ Zhang L, Luo S, Jin H, Lv X, Chen J (2019). "Anti-Hu Antibody-Associated Adie's Pupil and Paraneoplastic Sensorimotor Polyneuropathy Caused by Primary Mediastinal Small Cell Carcinoma". Frontiers in Neurology. 10: 1236. doi:10.3389/fneur.2019.01236. PMC 6901962 Check |pmc= value (help). PMID 31849812.
  18. ↑ Thompson HS, Kardon RH (June 2006). "The Argyll Robertson pupil". Journal of Neuro-ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society. 26 (2): 134–8. doi:10.1097/01.wno.0000222971.09745.91. PMID 16845316.
  19. ↑ Shin RK, Galetta SL, Ting TY, Armstrong K, Bird SJ (December 2000). "Ross syndrome plus: beyond horner, Holmes-Adie, and harlequin". Neurology. 55 (12): 1841–6. doi:10.1212/wnl.55.12.1841. PMID 11134383.
  20. ↑ Martin TJ (February 2018). "Horner Syndrome: A Clinical Review". ACS Chemical Neuroscience. 9 (2): 177–186. doi:10.1021/acschemneuro.7b00405. PMID 29260849.
  21. ↑ Leibovitch I, Kurtz S, Almog Y (2002). "Adie's tonic pupil-induced angle-closure glaucoma". Ophthalmologica. Journal International D'ophtalmologie. International Journal of Ophthalmology. Zeitschrift Fur Augenheilkunde. 216 (1): 71–2. doi:10.1159/000048302. PMID 11901294.
  22. ↑ 22.0 22.1 Wilhelm H (September 1998). "Neuro-ophthalmology of pupillary function--practical guidelines". Journal of Neurology. 245 (9): 573–83. doi:10.1007/s004150050248. PMID 9758294.
  23. ↑ Emond D, Lebel M (September 2002). "Orthostatic hypotension and Holmes-Adie syndrome. Usefulness of the Valsalva ratio in the evaluation of baroreceptor dysfunction". Journal of Human Hypertension. 16 (9): 661–2. doi:10.1038/sj.jhh.1001455. PMID 12214264.
  24. ↑ Leavitt JA, Wayman LL, Hodge DO, Brubaker RF (March 2002). "Pupillary response to four concentrations of pilocarpine in normal subjects: application to testing for Adie tonic pupil". American Journal of Ophthalmology. 133 (3): 333–6. doi:10.1016/s0002-9394(01)01420-9. PMID 11860969.
  25. ↑ Ballestero-Díez M, García-Río I, Daudén E, Corrales-Arroyo M, García-Díez A (November 2005). "Ross syndrome, an entity included within the spectrum of partial disautonomic syndromes". Journal of the European Academy of Dermatology and Venereology : JEADV. 19 (6): 729–31. doi:10.1111/j.1468-3083.2005.01254.x. PMID 16268880.
  26. ↑ "Holmes-Adie Syndrome Information Page | National Institute of Neurological Disorders and Stroke".
  27. ↑ Serra Mitjans M, Callejas Pérez MA, Valls Solé J, Grimalt Santacana R, Rubio Garay M, Iglesias Sentís M (February 2004). "[Surgical treatment for compensatory hyperhidrosis in Adie syndrome]". Archivos De Bronconeumologia (in Spanish; Castilian). 40 (2): 97–9. PMID 14746734.

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