• Disease Overview
  • Synonyms
  • Signs & Symptoms
  • Causes
  • Affected Populations
  • Disorders with Similar Symptoms
  • Diagnosis
  • Standard Therapies
  • Clinical Trials and Studies
  • References
  • Programs & Resources
  • Complete Report

Sydenham Chorea


Last updated: September 01, 2020
Years published: 1989, 1997, 1998, 2007, 2009, 2010, 2017, 2020


NORD gratefully acknowledges Donald L. Gilbert, MD, MS, Co-director, Cincinnati Children’s Hospital Medical Center Movement Disorders Clinic, for assistance in preparation of this report.

Disease Overview


Sydenham chorea is a rare neurological disorder characterized by sudden onset chorea, usually in childhood. Chorea is defined as random-appearing, continuous (while awake), involuntary movements which can affect the entire body. This often includes the face and tongue. Symptoms in arms and legs are often worse on one side of the body. Additional symptoms of Sydenham chorea may include slurring of speech and difficulty maintaining steady hand grip. Anxiety, sadness, inattention, and obsessive compulsive thoughts and behaviors may also occur. Sydenham chorea most often affects children over the age of 5 years and adolescents. Sydenham chorea usually develops within weeks to months following group A beta-hemolytic streptococcal infection and may occur as an isolated finding or as a major complication of acute rheumatic fever. It is considered an autoimmune disorder, meaning it occurs when the body’s immune system (which normally responds to foreign substances) mistakenly targets part of the body, disrupting normal function.

  • Next section >
  • < Previous section
  • Next section >


  • rheumatic chorea
  • St. Vitus dance
  • < Previous section
  • Next section >
  • < Previous section
  • Next section >

Signs & Symptoms

The severity of chorea and the presence of non-chorea symptoms of Sydenham chorea may vary greatly from one person to another. Most cases follow an identifiable streptococcal infection. Streptococcus is a group of bacteria that can cause several different infections, most commonly “strep throat” – often presenting with a sore throat (pharyngitis) or fever. Symptoms of Sydenham chorea may appear anywhere from 1 week to 6 months following streptococcal infection.

The abnormal movements (chorea) that characterize Sydenham chorea usually emerge over hours, peaking within a few hours or days. Pediatricians and emergency physicians seldom see chorea and may not recognize it. Initially, doctors may misattribute the restless movements and involuntary facial expressions of Sydenham chorea to a child being extremely fidgety, hyperactive, clumsy and/or purposely uncooperative. Parents (and children) generally recognize however that these movements, even in mild cases, are a clear change from the child’s usual status.
The abnormal movements in Sydenham chorea range from subtle symptoms, affecting coordination and tasks such as writing, to severe symptoms, disrupting walking, talking, and performing basic tasks such as dressing, eating, or simply holding objects. Choreic movements may fluctuate through the day. In most cases, chorea disappears during sleep.

In addition to choreic movements, individuals with Sydenham chorea may develop muscle weakness, slurred speech (dysarthria), diminished muscle tone (hypotonia), tics, obsessions, compulsions, inattention, anxiety, labile mood, and decreased verbal output. In some extremely rare cases (less than 2 percent), severe muscle weakness, irritability, or confusion may be profound and affected children may become bedridden, a condition sometimes referred to as paralytic chorea.

Because Sydenham chorea is a complication of rheumatic fever, some individuals will have additional symptoms of joint arthritis or arthralgia, inflammation of the heart valves causing permanent damage to the valves, and ongoing fever.

Sydenham chorea symptoms usually resolve within three weeks to six months. However, symptoms may last longer than one year. Occasionally, the symptoms of Sydenham chorea have recurred later during adult life, particularly in young women during the first trimester of pregnancy (so-called chorea gravidarum, which may represent a recurrence of Sydenham chorea in some cases).

  • < Previous section
  • Next section >
  • < Previous section
  • Next section >


Sydenham chorea is believed to be an autoimmune disorder. Most cases develop following a streptococcal infection or more severe rheumatic fever. An autoimmune disorder occurs when the body’s immune system mistakenly reacts against healthy tissue. In Sydenham chorea, streptococcal infection induces the body’s immune system to produce antibodies to combat the infection. For unknown reasons, these antibodies persist and subsequently target certain cells in the joints, kidneys, heart, and, in the brain, specifically cells of the basal ganglia (a key part of the brain for controlling motor movements). Researchers believe this ultimately leads to the characteristic symptoms of Sydenham chorea.

The exact underlying mechanisms that cause Sydenham chorea are poorly understood. Researchers believe that antigens (substances that are capable of stimulating an immune system response) on streptococcal bacterial cells are similar to antigens found on cells on the human body. When the immune system creates antibodies to combat the streptococcal infection, the antibodies also, in genetically predisposed individuals, mistakenly bind to healthy cells. When they bind to brain cells, they cause disruption in their “signaling” – their ability to control movement.

  • < Previous section
  • Next section >
  • < Previous section
  • Next section >

Affected populations

According to most studies, Sydenham chorea affects girls more often than boys. It usually develops in children between the ages of 5-15. Rarely, the disorder has been reported in children under age 5 years or in adults. Sydenham chorea affects individuals of all races and ethnicities.

Sydenham chorea may occur as a complication of rheumatic fever. Approximately, 25 percent of individuals with rheumatic fever develop Sydenham chorea. The incidence of rheumatic fever in North America declined steadily in the past 50 years, although there have been occasional outbreaks. Sydenham chorea is the most common cause of acute chorea during childhood in the United States. In areas of the world with less access to medical care and antibiotics, rheumatic fever remains a major public health problem due primarily to cases where there is damage to heart valves.

  • < Previous section
  • Next section >
  • < Previous section
  • Next section >


A diagnosis of Sydenham chorea is made based upon identification of new onset choreic movements, a detailed patient history, and a thorough clinical evaluation. In the presence of new onset chorea, which is uncommon in childhood, the documentation of a prior streptococcal infection through throat swabs and/or the current presence of high blood titers of streptococcal antibodies (ASO, anti DNAseB) is useful, as are identification of co-occurring arthritis or cardiac valve abnormalities. In some cases, certain imaging techniques such as magnetic resonance imaging (MRI) may be recommended to exclude other causes. Usually, brain imaging is normal in Sydenham chorea. Of note, because the onset of Sydenham chorea usually occurs weeks after the infection, the characteristic signs of rheumatic fever or streptococcal infection are usually no longer present.

Individuals who are diagnosed with Sydenham chorea should receive an evaluation for inflammation of the heart (carditis).

  • < Previous section
  • Next section >
  • < Previous section
  • Next section >

Standard Therapies

Secondary Prevention
A confirmed diagnosis of Sydenham chorea is nearly always an indication for long-term antibiotic treatment, until adulthood. The purpose of this treatment is to prevent permanent heart valve damage which could result if the child experiences recurrent streptococcal infections. Most often penicillin is used. Physicians should consult current rheumatic fever guidelines.

Chorea-suppressing Treatment
Chorea suppressing medications should be considered. Some mild cases may not cause much impairment for the child. These may resolve within weeks. When chorea symptoms are disabling, low doses of potent dopamine receptor blocking agents such as haloperidol, dopamine depleting agents such as tetrabenazine, anti-seizure medications such as valproic acid, or benzodiazepines may help. Because in most cases the treatment will only be needed for weeks to months and at low doses, long term neurological side effects such as tardive dyskinesia are extremely unlikely. Short term side effects such as weight gain may occur. As is the case for any neurological medications, however, a careful discussion of potential benefits as well as risks is advised.

Immune system treatment
Additional short-term immune therapies have been used to treat individuals with impairing Sydenham chorea during the first weeks of symptoms, based on the idea that ongoing acute inflammation is contributing to symptoms. There is some scientific support for using oral steroids and intravenous immunoglobulins from small but rigorous clinical trials.

  • < Previous section
  • Next section >
  • < Previous section
  • Next section >

Clinical Trials and Studies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]

Some current clinical trials also are posted on the following page on the NORD website:

For information about clinical trials sponsored by private sources, contact:

For information about clinical trials conducted in Europe, contact:

  • < Previous section
  • Next section >
  • < Previous section
  • Next section >


Baker CJ, American Academy of Pediatrics. Red book atlas of pediatric infectious diseases, 3rd edition. ed. Elk Grove Village, IL: American Academy of Pediatrics, 2017.

Singer HS, Mink JW, Gilbert DL, Jankovic J. Movement Disorders in Childhood, 2nd ed. Philadelphia, PA: Elsevier, Inc., 2016.1.

Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation 2015;131:1806-1818.

Ekici F, Cetin, II, Cevik BS, et al. What is the outcome of rheumatic carditis in children with Sydenham’s chorea? Turk J Pediatr 2012;54:159-167.

Walker K, Brink A, Lawrenson J, Mathiassen W, Wilmshurst JM. Treatment of sydenham chorea with intravenous immunoglobulin. Journal of Child Neurology 2012;27:147-155.

Ridel KR, Lipps TD, Gilbert DL. The prevalence of neuropsychiatric disorders in Sydenham’s chorea. Pediatr Neurol 2010;42:243-248.

Sanger TD, Chen D, Fehlings DL, et al. Definition and classification of hyperkinetic movements in childhood. Mov Disord 2010; Aug 15; 25(11):1538–1549.

Gilbert DL. Acute and chronic chorea in childhood. Semin Pediatr Neurol 2009;16:71-76.

Paz JA, Silva CA, Marques-Dias MJ. Randomized double-blind study with prednisone in Sydenham’s chorea. Pediatr Neurol 2006;34:264-269.

Zomorrodi A, Wald ER. Sydenham’s chorea in western Pennsylvania. Pediatrics 2006;117:e675-679.
Risavi BL, Iszkula E, Yost B. Sydenham’s Chorea. J Emerg Med 2019;56:e119-e121.

National Institute of Neurological Disorders and Stroke. Sydenham Chorea Information Page. Available at:https://www.ninds.nih.gov/Disorders/All-Disorders/Sydenham-Chorea-Information-Page Accessed August 27, 2020.

  • < Previous section
  • Next section >

Programs & Resources

RareCare® Assistance Programs

NORD strives to open new assistance programs as funding allows. If we don’t have a program for you now, please continue to check back with us.

Additional Assistance Programs

MedicAlert Assistance Program

NORD and MedicAlert Foundation have teamed up on a new program to provide protection to rare disease patients in emergency situations.

Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/

Rare Disease Educational Support Program

Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.

Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/

Rare Caregiver Respite Program

This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.

Learn more https://rarediseases.org/patient-assistance-programs/caregiver-respite/

Patient Organizations

NORD Breakthrough Summit | Rare Disease Conference