Sydenham's chorea

"Saint Vitus' dance" redirects here. For other uses, see Saint Vitus' dance (disambiguation).

Sydenham's chorea
Classification and external resources
ICD-10	I02
ICD-9	392
DiseasesDB	29245
MeSH	D002819

Sydenham's chorea or chorea minor (historically referred to as Saint Vitus Dance)^[1] is a disease characterized by rapid, uncoordinated jerking movements affecting primarily the face, feet and hands. Sydenham's chorea (SC) results from childhood infection with Group A beta-hemolytic Streptococci^[2] and is reported to occur in 20-30% of patients with acute rheumatic fever (ARF). The disease is usually latent, occurring up to 6 months after the acute infection, but may occasionally be the presenting symptom of rheumatic fever. Sydenham's Chorea is more common in females than males and most patients are children, below 18 years of age. Adult onset of Sydenham's Chorea is comparatively rare and most of the adult cases are associated with exacerbation of chorea following childhood Sydenham's Chorea.

It is named after British physician Thomas Sydenham (1624–1689).^[3][4] The alternate eponym, "Saint Vitus Dance", is in reference to Saint Vitus, a Christian saint who was persecuted by 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.^[5]

Characteristics

Sydenham's chorea is characterised by the acute onset (sometimes a few hours) of neurologic symptoms, classically chorea, usually affecting all limbs. Other neurologic symptoms include behavior change, dysarthria, gait disturbance, loss of fine and gross motor control with resultant deterioration of handwriting, headache, slowed cognition, facial grimacing, fidgetiness and hypotonia.^[6][7] Also, there may be tongue fasciculations ("bag of worms"), and a "milk sign", which is a relapsing grip demonstrated by alternate increases and decreases in tension, as if hand milking.^[8]

Non-neurologic manifestations of acute rheumatic fever are carditis, arthritis, erythema marginatum, and subcutaneous nodules.^[6]

Fifty percent of patients with acute Sydenham's Chorea spontaneously recover after 2 to 6 months whilst mild or moderate chorea or other motor symptoms can persist for up to and over 2 years in some cases (for example a patient in the UK who has suffered the illness since 1999)^{[citation needed]}. Sydenham's is also associated with psychiatric symptoms with obsessive compulsive disorder being the most frequent manifestation.

The PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections) syndrome is similar, but is not characterized by Sydenham's motor dysfunction, but presenting with tics and/or with psychological components (OCD) and much sooner, days to week after GABHS infection rather than 6–9 months.^[9] It may be confused with other conditions such as lupus and Tourette syndrome.

Movements cease during sleep, and the disease usually resolves after several months. It is associated with post-streptococcal rheumatic fever, pregnancy, hyperthyroidism, and systemic lupus erythematosus.

Causes

A major manifestation of acute rheumatic fever, Sydenham's chorea is a result of an autoimmune response that occurs following infection by group A β-hemolytic streptococci^[10] that destroys cells in the corpus striatum of the basal ganglia.^[7][10][11] The incidence of Acute Rheumatic Fever and rheumatic heart disease (RHD) is not declining. Recent figures quote the incidence of Acute Rheumatic Fever as 0.6 - 0.7/1 000 population in the USA and Japan compared with 15 - 21/1 000 population in Asia and Africa.^[3] The prevalence of Acute Rheumatic Fever and Sydenham's Chorea has declined progressively in developed countries over the last decades.^[12][13] There are many causes of childhood chorea, including cerebrovascular accidents, collagen vascular diseases, drug intoxication, hyperthyroidism, Wilson's disease, Huntington's disease, and infectious agents.^[6]

Treatment and management

Treatment of Sydenham's Chorea is based on the following three principles:

1. The first tenet of treatment is to eliminate the streptococcus at a primary, secondary and tertiary level. Strategies involve the adequate treatment of throat and skin infections, with a 10-day course of penicillin when Sydenham's Chorea is newly diagnosed, followed by long-term penicillin prophylaxis. Behavioural and emotional changes may precede the movement disorders in a previously well child.
2. Treatment of movement disorders. Therapeutic efforts are limited to palliation of the movement disorders. Haloperidol is frequently used because of its anti-dopaminergic effect.It has serious potential side-effects, e.g. tardive dyskinesia. In a study conducted at the RFC, 25 out of 39 patients on haloperidol reported side-effects severe enough to cause the physician or parent to discontinue treatment or reduce the dose. Other medications which have been used to control the movements include pimozide, clonidine, valproic acid, carbamazepine and phenobarbitone.
3. Immunomodulatory interventions include steroids, intravenous immunoglobulins, and plasma exchange. Patients may benefit from treatment with steroids; controlled clinical trials are indicated to explore this further.

Society and culture

St. Vitus' dance is noted as the malady suffered by the elderly physician from whom Dr. Watson purchased a medical practice in "The Stock-Broker's Clerk" by Arthur Conan Doyle, included in a number of Holmes' stories published collectively in The Memoirs of Sherlock Holmes.

American artist Andy Warhol was afflicted with rheumatic fever as a young child, and developed the symptoms of St. Vitus' Dance.

Michael Caine suffered from St. Vitus' Dance when he was a child, as written in his autobiography What's it All About.

References

1. ^ NINDS Sydenham Chorea Information Page Saint Vitus Dance, Rheumatic Encephalitis from the National Institute of Neurological Disorders and Stroke. Accessed April 26, 2008
2. ^ Sydenham's chorea: Symptoms/Findings from WeMOVE.Org Accessed April 26, 2008
3. ^ ^{a b} Walker K, Lawrenson J, Wilmshurst JM. Sydenham's Chorea-clinical and therapeutic update 320 years down the line. SAMJ. 2006; 96(9):906-912
4. ^ "Sydenham's chorea". Whonamedit. http://www.whonamedit.com/synd.cfm/2226.html. Retrieved 2011-09-16. 
5. ^ "St. Vitus Information Page - Star Quest Production Network". Saints.sqpn.com. http://saints.sqpn.com/saint-vitus/. Retrieved 2011-09-16. 
6. ^ ^{a b c} Zomorrodi A, Wald ER. Sydenham's Chorea in Western Pennsylvania . Pediatriatrics. 2006; 117(4):675-679
7. ^ ^{a b} Swedo SE,Leonard HL, Shapiro MB. Sydenham's Chorea:Physical and Psychological Symptoms of St Vitus Dance. Pediatrics.1993; 91(4): 706–713.
8. ^ Medscape > Pediatric Rheumatic Heart Disease Clinical Presentation > Noncardiac manifestations. Author: Thomas K Chin, MD; Chief Editor: Stuart Berger, MD. Updated: Aug 4, 2010
9. ^ Swedo SE, Leonard HL, Garvey M, et al (February 1998). "Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases". Am J Psychiatry 155 (2): 264–71. PMID 9464208. http://ajp.psychiatryonline.org/cgi/pmidlookup?view=long&pmid=9464208. 
10. ^ ^{a b} Sydenham's Chorea Symptoms.Accessed September 24, 2009.
11. ^ Faustino PC, Terreri MT, da Rocha AJ et al. Clinical, laboratory, psychiatric and magnetic resonance findings in patients with sydenham chorea.Neuroradiology. 2003; 45(7): 456-462
12. ^ Nausieda PA, Grossman BJ, Koller WC et al. Sydenham's Chorea:An update.Neurology. 1980;30(3):331-334
13. ^ Eshel E, Lahat E, Azizi E et al. Chorea as a manifestation of rheumatic fever-a 30-year survey. Eur J Pediatr. 1993; 158(8):645-646

External links

• synd/2226 at Who Named It?
• -596967410 at GPnotebook
• WE MOVE - Sydenham's Chorea - Symptoms/Findings
• Sydenham's chorea: Not gone and not forgotten* - Elsevier
• On defining Sydenham's chorea: where do we draw the line? - Elsevier