Editing Sydenham's chorea

{{For|the mysterious historical phenomenon also known as "St. Vitus' dance"|Dancing mania}}
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'''Sydenham's chorea''', also known as '''rheumatic chorea''', is a disorder characterized by [[Chorea|rapid, uncoordinated jerking movements]] primarily affecting the face, hands and feet.<ref>{{cite web |title=Sydenham Chorea Information Page {{!}} National Institute of Neurological Disorders and Stroke |url=https://www.ninds.nih.gov/Disorders/All-Disorders/Sydenham-Chorea-Information-Page |website=www.ninds.nih.gov |access-date=2 March 2021}}</ref> Sydenham's chorea is an [[autoimmune]] disease that results from childhood infection with Group A beta-[[haemolytic]] ''[[Streptococcus]]''.  It is reported to occur in 20–30% of people with acute [[rheumatic fever]] and is one of the major criteria for it, although it sometimes occurs in isolation. The disease occurs typically a few weeks, but up to 6 months, after the acute infection, which may have been a simple sore throat ([[pharyngitis]]).

Sydenham's chorea is more common in females than males, and most cases affect children between 5 and 15 years of age. Adult onset of Sydenham's chorea is comparatively rare, and the majority of the adult cases are recurrences following childhood Sydenham's chorea (although pregnancy<ref>{{cite journal |last1=Maia |first1=Débora P. |last2=Fonseca |first2=Patricia G. |last3=Camargos |first3=Sarah T. |last4=Pfannes |first4=Cláudia |last5=Cunningham |first5=Mauro C. |last6=Cardoso |first6=Francisco |title=Pregnancy in patients with Sydenham's Chorea |journal=Parkinsonism & Related Disorders |date=June 2012 |volume=18 |issue=5 |pages=458–461 |doi=10.1016/j.parkreldis.2011.12.013|pmid=22236583 }}</ref> and female hormone treatment<ref>{{cite journal |last1=Kyle |first1=Kevin |last2=Bordelon |first2=Yvette |last3=Venna |first3=Nagagopal |last4=Linnoila |first4=Jenny |title=Autoimmune and Paraneoplastic Chorea: A Review of the Literature |journal=Frontiers in Neurology |date=18 March 2022 |volume=13 |doi=10.3389/fneur.2022.829076|doi-access=free |pmid=35370928 |pmc=8972589 }}</ref> are also potential causes).

It is historically one of the conditions called ''[[St Vitus' dance (disambiguation)|St Vitus' dance]]''.<ref name=":0">{{Cite book |url=https://books.google.com/books?id=7sg5EAAAQBAJ&dq=%22rheumatic+chorea%22+eyes&pg=PA1735 |title=Mosby's Medical Dictionary |date=2021-07-23 |publisher=Elsevier Health Sciences |isbn=978-0-323-83162-8 |pages=1735 |language=en}}</ref>

==Signs and symptoms==
Sydenham's chorea is characterized by the abrupt onset (sometimes within a few hours) of [[neurological symptoms]], classically [[chorea]], which are non-rhythmic, writhing or explosive involuntary movements.  Usually all four limbs are affected, but there are cases reported where just one side of the body is affected (''hemichorea''). Typical chorea includes repeated wrist [[hyperextension]], grimacing, and lip pouting. The fingers can move as if playing the piano. There may be tongue [[fasciculations]] ("bag of worms") and ''motor impersistence'', for example, the "milkmaid sign" (grip strength fluctuates, as if [[hand milking]] a cow), or inability to sustain tongue protrusion (called [[jack-in-the-box]] tongue<ref>{{Cite book |last1=Verma |first1=Anoop |url=https://books.google.com/books?id=U3KSDwAAQBAJ&dq=Sydenham's+chorea+%22snake%22+tongue&pg=PA354 |title=IAP Textbook of Pediatric Neurology |last2=Kunju |first2=P. A. M. |date=2019-05-31 |publisher=Jaypee Brothers Medical Publishers |isbn=978-93-5270-979-3 |pages=354 |language=en}}</ref> or [[Snake#Locomotion|serpentine]] tongue,<ref>{{Cite book |last1=Talley |first1=Nicholas J. |url=https://books.google.com/books?id=j_r_DwAAQBAJ&dq=Sydenham's+chorea+eyes&pg=PA577 |title=Talley and O'Connor's Examination Medicine - epub: A Guide to Physician Training |last2=O’Connor |first2=Simon |date=2020-10-30 |publisher=Elsevier Health Sciences |isbn=978-0-7295-8875-1 |pages=577 |language=en}}</ref> as the tongue slides in and out of the mouth), or eye closure.

There is usually a loss of [[fine motor control]], which is particularly obvious in [[handwriting]] if the child is of school age.  Speech is often affected ([[dysarthria]]), as is walking; legs will suddenly give way or flick out to one side, giving an irregular [[gait]] and the appearance of skipping or dancing. Underlying the abnormal movements is often low tone ([[hypotonia]]) which may not become obvious until treatment is started to suppress the chorea.  

The severity of the condition can vary from just some instability on walking and difficulty with handwriting, to the extreme of being wholly unable to walk, talk, or eat (''chorea paralytica'').

Movements cease during sleep.

It is a neuropsychiatric disorder, so besides the motor problems there is classically emotional lability (mood swings, or inappropriate mood), but also tics, anxiety, attention deficit etc.  These can precede the motor symptoms.<ref>{{cite journal|last1=Oosterveer|first1=Daniëlla M.|last2=Overweg-Plandsoen|first2=Wilhelmina C.T.|last3=Roos|first3=Raymund A.C.|date=July 2010|title=Sydenham's Chorea: A Practical Overview of the Current Literature|url=https://www.pedneur.com/article/S0887-8994(10)00035-4/fulltext|journal=Pediatric Neurology|volume=43|issue=1|pages=1–6|doi=10.1016/j.pediatrneurol.2009.11.015|pmid=20682195|access-date=2 March 2021|url-access=subscription}}</ref>

Non-neurologic manifestations of acute rheumatic fever may be present, namely [[carditis]] (up to 70% of cases, often subclinical, so echocardiography required), [[arthritis]], [[erythema marginatum]], and subcutaneous [[Nodule (medicine)|nodules]].

[[File:Erythema marginatum.jpg|thumb|right|alt=Classic rash of rheumatic fever|Erythema marginatum]]

Differentiating these signs from other involuntary movements such as [[tics]] and [[stereotypies]] can be difficult, and since these things are not uncommon they can potentially co-exist.  Diagnosis is often delayed and attributed to another condition such as [[tic disorder]] or [[conversion disorder]].  The controversial [[PANDAS]] (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) hypothesis has overlapping clinical features, but Sydenham's chorea is one of the exclusion criteria.  PANDAS can present with chorea but more typically there are [[tics]] or [[stereotypies]] with a psychological component (e.g., [[Obsessive–compulsive disorder|OCD]]).<ref name= Wilbur2019>{{cite journal |vauthors=Wilbur C, Bitnun A, Kronenberg S, Laxer RM, Levy DM, Logan WJ, Shouldice M, Yeh EA |title=PANDAS/PANS in childhood: Controversies and evidence |journal=Paediatr Child Health |volume=24 |issue=2 |pages=85–91 |date=May 2019 |pmid=30996598 |pmc=6462125 |doi=10.1093/pch/pxy145}}</ref><ref name=Sigra2018>{{cite journal |vauthors=Sigra S, Hesselmark E, Bejerot S |title=Treatment of PANDAS and PANS: a systematic review |journal=Neurosci Biobehav Rev |volume=86 |issue= |pages=51–65 |date=March 2018 |pmid=29309797 |doi=10.1016/j.neubiorev.2018.01.001 |s2cid=40827012 |doi-access=free }}</ref><ref>{{cite journal |vauthors=Swedo SE, Leonard HL, Garvey M, etal |title=Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases |journal=The American Journal of Psychiatry |volume=155 |issue=2 |pages=264–71 |date=February 1998 |pmid=9464208 |doi= 10.1176/ajp.155.2.264 |s2cid=22081877 |url=http://ajp.psychiatryonline.org/cgi/pmidlookup?view=long&pmid=9464208|url-access=subscription }}</ref>

==Differential diagnosis==
Other disorders that may be accompanied by chorea include [[benign hereditary chorea]], [[bilateral striatal necrosis]], [[abetalipoproteinemia]], [[ataxia–telangiectasia]], [[biotin-thiamine-responsive basal ganglia disease (BTBGD)]], [[Fahr disease]], [[familial dyskinesia–facial myokymia]] (Bird–Raskind syndrome) due to an [[ADCY5]] gene mutation, [[glutaric aciduria]], [[Lesch–Nyhan syndrome]], [[mitochondrial disorders]], [[Huntington's disease]], [[Wilson disease]], [[hyperthyroidism]], [[lupus erythematosus]], pregnancy ([[chorea gravidarum]]), [[drug intoxication]] and side effects of certain anticonvulsants (e.g. [[phenytoin]]) or psychotropic agents. Although some of these can similarly present in an acute way, there will typically be other neurological signs (such as ataxia or cognitive impairment), or other disease manifestations, or positive family history, which will help distinguish between them.<ref name=Chun>{{cite journal |vauthors=Zomorrodi A, Wald ER | year = 2006 | title = Sydenham's Chorea in Western Pennsylvania | journal = Pediatrics | volume = 117 | issue = 4| pages = 675–679 | doi = 10.1542/peds.2005-1573 | pmid = 16533893 | s2cid = 32478765 }}</ref>

==Pathology==
One of the important manifestations of [[acute rheumatic fever]], Sydenham's chorea is believed to be caused by an [[autoimmune]] response following infection by [[group A β-hemolytic streptococci]].<ref name=Chan>{{cite journal |vauthors=Swedo SE, Leonard HL, Shapiro MB | year = 1993 | title = Sydenham's Chorea:Physical and Psychological Symptoms of St Vitus Dance | journal = Pediatrics | volume = 91 | issue = 4| pages = 706–713 | pmid = 8464654 }}</ref><ref name=":0" />

Two cross-reactive streptococcal [[antigens]] have been identified, the M protein and N-acetyl-beta-D-glucosamine, whereby infection leads to [[autoantibody|autoantibodies]] being produced against host tissues ([[molecular mimicry]]) causing a variety of streptococcal related diseases including Sydenham's chorea but also [[rheumatic heart disease]] and [[nephritic syndrome]].<ref>{{cite journal|last1=Cunningham|first1=Madeleine W.|date=2 August 2019|title=Molecular Mimicry, Autoimmunity, and Infection: The Cross-Reactive Antigens of Group A Streptococci and their Sequelae|journal=Microbiology Spectrum|volume=7|issue=4|doi=10.1128/microbiolspec.GPP3-0045-2018|pmid=31373269|pmc=6684244}}</ref> Autoantibodies against basal ganglia proteins have been found in Sydenham's chorea but these are non-specific.<ref>{{cite journal |vauthors=Dale RC, Merheb V, Pillai S, etal | year = 2012 | title = Antibodies to surface dopamine-2 receptor in autoimmune movement and psychiatric disorders | journal = Brain | volume = 135 | issue = 11| pages = 3453–3468 | doi=10.1093/brain/aws256 | pmid=23065479| doi-access = free }}</ref> Dopamine receptor autoantibodies have been reported to correlate with clinical symptoms.<ref>{{cite journal |vauthors=Ben-Pazi H, Stoner JA, Cunningham MW | year = 2013 | title = Dopamine receptor autoantibodies correlate with symptoms in Sydenham's chorea | journal = PLOS ONE | volume = 8 | issue = 9| pages = e73516 | doi=10.1371/journal.pone.0073516 | pmid=24073196| pmc = 3779221 | bibcode = 2013PLoSO...873516B | doi-access = free }}</ref> Whether these antibodies represent an [[epiphenomenon]] or are pathogenic, remains to be proven.<ref name=":0" />

==Epidemiology==

Sydenham's chorea is primarily seen in children.<ref name=":0" />

As with rheumatic fever, Sydenham's chorea is seen more often in less affluent communities, whether in the [[developing world]] or in [[Indigenous peoples|aboriginal communities]] in the [[global North]].  High rates of [[impetigo]] are a marker for widespread streptococcal transmission.

In the UK, there are approximately 20 cases per year, according to a BPSU surveillance study.<ref>{{cite journal |last1=Wooding |first1=Eva Louise |last2=Morton |first2=Michael John Stuart |last3=Lim |first3=Ming |last4=Mitrofan |first4=Oana |last5=Mushet |first5=Nadine |last6=Sie |first6=Adrian |last7=Knight |first7=Brodie |last8=Ford |first8=Tamsin |last9=Newlove-Delgado |first9=Tamsin |title=Childhood/adolescent Sydenham's chorea in the UK and Ireland: a BPSU/CAPSS surveillance study |journal=Archives of Disease in Childhood |date=September 2023 |volume=108 |issue=9 |pages=736–741 |doi=10.1136/archdischild-2023-325399|pmid=37225279 |pmc=10447407 }}</ref>

==Diagnosis==

Chorea is distinctive, if the health care provider is familiar with it.  The diagnosis is then made by the typical acute onset in the weeks following a sore throat or other minor infection, plus evidence of inflammation (raised [[C-reactive protein|CRP]] and/or [[Erythrocyte sedimentation rate|ESR]]) and evidence of recent streptococcal infection.

To confirm recent streptococcal infection:
* [[Throat culture]]
* [[Anti-DNase B|Anti-DNAse B]] titre (peaks at 8–12 weeks after infection)
* [[Anti-streptolysin O]] titre (peaks at 3–5 weeks)

None of these tests are 100% reliable, particularly when the infection was some months previously.

Further testing is directed more towards alternative diagnoses and other manifestations of rheumatic fever:
* [[Echocardiography]]
* [[Electroencephalography]] (EEG)
* [[Lumbar puncture]]
* [[Magnetic resonance imaging of the brain|Magnetic resonance imaging]] or [[Computed tomography scan|computed tomography (CT) scan]] of the brain (alterations in [[caudate nucleus]] and [[Putamen|putaminal]] enlargement have been described in some patients)<ref>{{Cite web | url=https://medlineplus.gov/ency/article/001358.htm | title=Sydenham chorea: MedlinePlus Medical Encyclopedia}}</ref><ref>{{cite journal |vauthors=Faustino PC, Terreri MT, Rocha AJ, etal | year = 2003 | title = Clinical, laboratory, psychiatric and magnetic resonance findings in patients with sydenham chorea | journal = Neuroradiology | volume = 45 | issue = 7| pages = 456–462 | doi=10.1007/s00234-003-0999-8| pmid = 12811441 | s2cid = 23605799 }}</ref>

==Management==
[[File:Penicillin core.svg|thumb|Penicillin]]
Management of Sydenham's chorea is based on the following principles:
* Eliminate the streptococcus – a course of penicillin is usually given at diagnosis to definitively clear any remaining streptococci (and avoid spread of a rheumatogenic clone), but there is no evidence that it makes any difference to the course of the illness. 
* Treat the movement disorder
* Immunosuppression
* Prevention of relapses and further cardiac damage
* Manage the disability - [[Occupational therapy]] and [[physiotherapy]] are useful for maintaining function and muscle tone.

===Treatment of chorea===
Treatment with [[sodium valproate]] is effective for controlling symptoms, but it does not speed up recovery. [[Haloperidol]] was used previously, but caused serious side effects e.g. [[tardive dyskinesia]]. Case reports exist to support [[carbamazepine]] and [[levetiracetam]]; other drugs tried include [[pimozide]], [[clonidine]], and [[phenobarbitone]].

===Immunosuppression===
Immunosuppression is used inconsistently in Sydenham's chorea.  The model of an autoimmune disorder would support its use.  One randomized controlled trial of steroids from Paz, Brazil in 2006 (22 cases) showed remission reduced to 54 days from 119 days.<ref>{{cite journal|last1=Paz|first1=José A.|last2=Silva|first2=Clovis A.A.|last3=Marques-Dias|first3=Maria J.|date=April 2006|title=Randomized Double-Blind Study With Prednisone in Sydenham's Chorea|journal=Pediatric Neurology|volume=34|issue=4|pages=264–269|doi=10.1016/j.pediatrneurol.2005.08.028|pmid=16638499}}</ref> Various other reports of use of oral or IV steroids from Israel, Italy and Brazil.<ref name="Acute and chronic corticosteroid tr">{{cite journal|last1=Fusco|first1=Carlo|last2=Ucchino|first2=Valentina|last3=Frattini|first3=Daniele|last4=Pisani|first4=Francesco|last5=Della Giustina|first5=Elvio|date=July 2012|title=Acute and chronic corticosteroid treatment of ten patients with paralytic form of Sydenham's chorea|journal=European Journal of Paediatric Neurology|volume=16|issue=4|pages=373–378|doi=10.1016/j.ejpn.2011.12.005|pmid=22197452}}</ref><ref>{{cite journal|last1=Fusco|first1=C.|last2=Spagnoli|first2=C.|date=March 2018|title=Corticosteroid treatment in Sydenham's chorea|journal=European Journal of Paediatric Neurology|volume=22|issue=2|pages=327–331|doi=10.1016/j.ejpn.2017.11.011|pmid=29287833}}</ref><ref>{{cite journal|last1=Teixeira Jr.|first1=Antônio L.|last2=Maia|first2=Débora P.|last3=Cardoso|first3=Francisco|date=August 2005|title=Treatment of acute Sydenham's chorea with methyl-prednisolone pulse-therapy|journal=Parkinsonism & Related Disorders|volume=11|issue=5|pages=327–330|doi=10.1016/j.parkreldis.2005.02.007|pmid=15878690}}</ref>  Immunoglobulin has been used in Holland and South Africa.<ref>{{cite journal|last1=Boersma|first1=Nienke Anne|last2=Schippers|first2=Herman|last3=Kuijpers|first3=Taco|last4=Heidema|first4=Jojanneke|date=27 January 2016|title=Successful treatment of Sydenham's chorea with intravenous immunoglobulin|journal=BMJ Case Reports|volume=2016|pages=bcr2015211673|doi=10.1136/bcr-2015-211673|pmid=26837939|pmc=4746543}}</ref><ref>{{cite journal|last1=Gregorowski|first1=Claire|last2=Lochner|first2=Christine|last3=Martin|first3=Lindi|last4=Simmons|first4=Candice|last5=Kidd|first5=Martin|last6=Walker|first6=Kathleen|last7=Wilmshurst|first7=Jo M.|last8=Seedat|first8=Soraya|date=1 February 2016|title=Neuropsychological manifestations in children with Sydenham's chorea after adjunct intravenous immunoglobulin and standard treatment|journal=Metabolic Brain Disease|volume=31|issue=1|pages=205–212|doi=10.1007/s11011-015-9681-1|pmid=25987537|s2cid=3234882}}</ref> Some improvement can be seen within a few days of IV steroids. In Italy, prednisolone reduced average duration of symptoms from 9 weeks to 4 weeks, and these were severe cases.<ref name="Acute and chronic corticosteroid tr"/> South African group found less neuropsychiatric complications at 6 months with IVIG treatment (IVIG preferred due to fear of TB reactivation).<ref>{{cite journal|last1=Dean|first1=Shannon L.|last2=Singer|first2=Harvey S.|date=1 June 2017|title=Treatment of Sydenham's Chorea: A Review of the Current Evidence|journal=Tremor and Other Hyperkinetic Movements|volume=7|pages=456|doi=10.7916/d8w95gj2|doi-broken-date=2024-11-01 |pmid=28589057|pmc=5459984}}</ref>

===Relapse prevention===
[[Antibiotic prophylaxis|Penicillin prophylaxis]] is essential to treat cardiac features of rheumatic fever, even if subclinical (American Heart Association guideline).<ref>{{cite journal|date=17 November 2020|title=Contemporary Diagnosis and Management of Rheumatic Heart Disease: Implications for Closing the Gap: A Scientific Statement From the American Heart Association|journal=Circulation|volume=142|issue=20|pages=e337–e357|doi=10.1161/CIR.0000000000000921|last1=Kumar|first1=Raman Krishna|last2=Antunes|first2=Manuel J.|last3=Beaton|first3=Andrea|last4=Mirabel|first4=Mariana|last5=Nkomo|first5=Vuyisile T.|last6=Okello|first6=Emmy|last7=Regmi|first7=Prakash Raj|last8=Reményi|first8=Boglarka|last9=Sliwa-Hähnle|first9=Karen|last10=Zühlke|first10=Liesl Joanna|last11=Sable|first11=Craig|author12=American Heart Association Council on Lifelong Congenital Heart Disease Heart Health in the Young; Council on Cardiovascular Stroke Nursing; Council on Clinical Cardiology|pmid=33073615|s2cid=224783747|doi-access=free}}</ref> If there are not features to warrant a diagnosis of rheumatic fever, it is arguable whether cardiac risk justifies prophylaxis or not; however, it is likely to reduce recurrence.

===Other treatments===
There are several historical case series reporting successful treatment of Sydenham's chorea by inducing fever.<ref>{{cite journal |last1=Sutton |first1=LP |last2=Dodge |first2=KG |title=The treatment of chorea by induced fever |journal=The Journal of Pediatrics |year=1933 |volume=3 |issue=6 |pages=813–26 |doi=10.1016/s0022-3476(33)80151-x}}</ref><ref>{{cite journal |last1=Sutton |first1=LP |last2=Dodge |first2=KG |title=Fever therapy in chorea and in rheumatic carditis with and without chorea |journal=The Journal of Laboratory and Clinical Medicine |year=1936 |volume=21 |issue=6 |pages=619–28}}</ref>

==Prognosis==
Symptoms usually get worse over the course of two weeks, then stabilize, and finally begin to improve.<ref name=":0" />  Motor problems, including chorea, settle within an average of 2–3 months.<ref name=":0" />

Recurrence is seen in 16–40% of cases. It is sometimes but not always associated with a rise in ASO titre or other evidence of new streptococcal infection.  Recurrence is more likely with poor compliance with penicillin prophylaxis.   It is more likely if there is failure to remit within 6 months of onset. Recurrence is associated in women with pregnancy (''chorea gravidorum'') and with female hormone treatment, although the onset of chorea can be delayed by months or more.  

Intramuscular penicillin given every 2–3 weeks is superior to a 4 weekly regime for preventing relapse, but a risk assessment may conclude that twice daily oral penicillin is sufficiently effective, less painful for the child, and less demanding on the family.

Higher recurrence rates are seen with the longest follow up – relapse can be seen 10 years or more after the initial episode, so might be underestimated by series with shorter follow up.

Recurrence is usually only chorea, even if the original case was associated with rheumatic fever. There are two total reports of heart disease worsening after recurrence of chorea. The Thailand study also had 2 cases where carditis, which had improved after initial diagnosis, came back again. Some suggest that recurrent chorea is a different disease altogether.<ref>{{cite journal |last1=Gurkas |first1=E |last2=Karalok |first2=ZS |last3=Taskin |first3=BD |last4=Aydogmus |first4=U |last5=Guven |first5=A |last6=Degerliyurt |first6=A |last7=Bektas |first7=O |last8=Yilmaz |first8=C |title=Predictors of recurrence in Sydenham's chorea: Clinical observation from a single center. |journal=Brain & Development |date=October 2016 |volume=38 |issue=9 |pages=827–34 |doi=10.1016/j.braindev.2016.04.010 |pmid=27209549|s2cid=2614986 }}</ref>

10% reported long-term tremor in one study (10 years follow up). Long term neuropsychiatric difficulties are increasingly recognized (49 studies so far, especially [[obsessive-compulsive disorder]] but also [[attention-deficit hyperactivity disorder]], [[affective disorders]], [[tic disorders]], executive function disturbances, psychotic features, and language impairment).<ref>{{cite journal |last1=Punukollu |first1=M |last2=Mushet |first2=N |last3=Linney |first3=M |last4=Hennessy |first4=C |last5=Morton |first5=M |title=Neuropsychiatric manifestations of Sydenham's chorea: a systematic review. |journal=Developmental Medicine and Child Neurology |date=January 2016 |volume=58 |issue=1 |pages=16–28 |doi=10.1111/dmcn.12786 |pmid=25926089|s2cid=31037155 |doi-access=free }}</ref>

Heart involvement improves in about a third of cases (whether silent or not).<ref>{{Cite journal |last1=Ekici |first1=Filiz |last2=Cetin |first2=Ibrahim Ilker |last3=Cevik |first3=Berna-Saylan |last4=Senkon |first4=Oben Gözde |last5=Alpan |first5=Nursel |last6=Değerliyurt |first6=Aydan |last7=Güven |first7=Alev |last8=Ateş |first8=Can |last9=Cakar |first9=Nilgün |date=March 2012 |title=What is the outcome of rheumatic carditis in children with Sydenham's chorea? |url=https://pubmed.ncbi.nlm.nih.gov/22734303 |journal=The Turkish Journal of Pediatrics |volume=54 |issue=2 |pages=159–167 |issn=0041-4301 |pmid=22734303}}</ref>

==History==
<!--<ref name=PBJ>{{cite journal |author1=D Martino |author2=A Tanner |author3=G Defazio |author4=A J Church |author5=K P Bhatia |author6=G Giovannoni |author7=R C Dale |title=Tracing Sydenham's chorea: historical documents from a British paediatric hospital |journal=Archives of Disease in Childhood |volume=90 |issue=5 |pages=507–511 |date=October 2004 |pmid=15851434|doi= 10.1136/adc.2004.057679|pmc=1720385}}</ref>-->

The incidence of acute rheumatic fever and rheumatic heart disease is not declining. Recent figures quote the incidence of Acute Rheumatic Fever as 0.6–0.7/1,000 population in the United States and Japan compared with 15–21/1,000 population in Asia and Africa.<ref name="ReferenceA"/> The prevalence of Acute Rheumatic Fever and Sydenham's Chorea has declined progressively in developed countries over the last decades.<ref>{{cite journal |vauthors=Nausieda PA, Grossman BJ, Koller WC, etal | year = 1980 | title = Sydenham's Chorea:An update | journal = Neurology | volume = 30 | issue = 3| pages = 331–334 | doi=10.1212/wnl.30.3.331| pmid = 7189038 | s2cid = 21035716 }}</ref><ref>{{cite journal |vauthors=Eshel E, Lahat E, Azizi E, etal | year = 1993 | title = Chorea as a manifestation of rheumatic fever-a 30-year survey | doi = 10.1007/bf01955239 | pmid = 8404967 | journal = European Journal of Pediatrics | volume = 152 | issue = 8| pages = 645–646 | s2cid = 29611352 }}</ref>

==Etymology==
It is named after British physician [[Thomas Sydenham]] (1624–1689).<ref name="ReferenceA">{{cite journal |vauthors=Walker K, Lawrenson J, Wilmshurst JM | year = 2006 | title = Sydenham's Chorea-clinical and therapeutic update 320 years down the line | journal = South African Medical Journal | volume = 96 | issue = 9| pages = 906–912 | pmid = 17077917 }}</ref><ref>{{cite web|url=http://www.whonamedit.com/synd.cfm/2226.html |title=Sydenham's chorea |publisher=Whonamedit |access-date=2011-09-16}}</ref> The alternative eponym, "Saint Vitus Dance", is in reference to [[Saint Vitus]], a Christian saint who was persecuted by [[Roman Empire|Roman]] emperors and died as a martyr in AD 303. Saint Vitus is considered to be the [[patron saint]] of dancers, with the eponym given as homage to the manic dancing that historically took place in front of his statue during the feast of Saint Vitus in Germanic and Latvian cultures.<ref>{{cite web|url=http://saints.sqpn.com/saint-vitus/ |title=St. Vitus Information Page - Star Quest Production Network |publisher=Saints.sqpn.com |date= 2009-01-07|access-date=2011-09-16}}</ref>

==References==
{{Reflist}}

== Further reading ==
* {{cite journal  |vauthors=Martino D, Tanner A, Defazio G, etal |title=Tracing Sydenham's chorea: historical documents from a British paediatric hospital |journal=Archives of Disease in Childhood |volume=90 |issue=5 |pages=507–11 |date=May 2005 |pmid=15851434 |pmc=1720385 |doi=10.1136/adc.2004.057679}}

== External links ==
* [http://www.sydenhamschorea.com Sydenhams Chorea Association] for patient & family support
* [https://www.movementdisorders.org/MDS/MDS-Rating-Scales/UFMG-Sydenhams-Chorea-Rating-Scale-USCRS.htm UFMG rating scale], research from Brazil's [[Universidade Federal de Minas Gerais]] (UFMG)

{{Medical resources
| DiseasesDB      = 29245
| ICD10           = {{ICD10|I|02||i|00}}
| ICD9            = {{ICD9|392}}
| ICDO            =
| OMIM            =
| MedlinePlus     = 001358
| eMedicineSubj   =
| eMedicineTopic  =
| MeshID          = D002819
| SNOMED CT       = 46826000
}}

{{Heart diseases}}
{{Gram-positive firmicutes diseases}}

{{DEFAULTSORT:Sydenham's Chorea}}
[[Category:Neurological disorders]]