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Sydenham's chorea is an acute but self-limited movement disorder that occurs most commonly in children between the ages of 5 and 15, and occasionally in pregnant women. It is closely associated with rheumatic fever following a throat infection. The disorder is named for Thomas Sydenham (1624–1689), an English doctor who first described it in 1686. Other names for Sydenham's chorea include simple chorea, chorea minor, acute chorea, rheumatic chorea, juvenile chorea, and St. Vitus' dance. The English word "chorea" itself comes from the Greek word choreia, which means "dance." The disorder takes its name from the rapid involuntary jerking or twitching movements of the patient's face, limbs, and upper body.
Sydenham's chorea is a disorder that occurs in children and is associated with rheumatic fever. Rheumatic fever is an acute infectious disease caused by certain types of streptococci bacteria. It usually starts with strep throat or tonsillitis. These types of streptococci are able to cause disease throughout the body. The most serious damage caused by rheumatic fever is to the valves in the heart. At one time, rheumatic fever was the most common cause of damaged heart valves, and it still is in most developing countries around the world. Rheumatic fever and rheumatic heart disease are still present in the industrialized countries, but the incidence has dropped substantially.
Both acute rheumatic fever and Sydenham's chorea are relatively uncommon disorders in the United States as of 2004. According to the Centers for Disease Control and Prevention (CDC), only 1%-3% of people with streptococcal throat infections develop acute rheumatic fever (ARF); thus the incidence of ARF in the United States is thought to be about 0.5 per 100,000 patients between 5 and 17 years of age.
With regard to age, the incidence of Sydenham's chorea is higher in childhood and adolescence than in adult life. It occurs more frequently in females than in males; the gender ratio is thought to be about 2 F: 1 M. Since the peak incidence of rheumatic fever in North America occurs in late winter and spring, Sydenham's chorea is more likely to occur in the summer and early fall. There is no evidence as of 2004 that the disorder selectively affects specific racial or ethnic groups.
Rheumatic fever may appear in several different forms. Sydenham's chorea is one of five major criteria for the diagnosis of rheumatic fever. There are also four minor criteria and two types of laboratory tests associated with the disease. The "Jones criteria" define the diagnosis. They require laboratory evidence of a streptococcal infection plus two or more of the criteria. The laboratory evidence may be identification of streptococci from a sore throat or antibodies to streptococcus in the blood. The most common criteria are arthritis and heart disease, occurring in half to three-quarters of the patients. Sydenham's chorea, characteristic nodules under the skin, and a specific type of skin rash occur only 10% of the time.
About 20% of patients diagnosed with Sydenham's chorea experience a recurrence of the disorder, usually within two years of the first episode. Most women who develop Sydenham's during pregnancy have a history of acute rheumatic fever in childhood or of using birth control pills containing estrogen.
Causes and symptoms
Sydenham's is caused by certain types of streptococci called Group A beta-hemolytic streptococci or GAS bacteria. In general, streptococci are sphericalshaped anaerobic bacteria that occur in pairs or chains. GAS bacteria belong to a subcategory known as pyogenic streptococci, which means that the infections they cause produce pus. These particular germs seem to be able to create an immune response that attacks the body's own tissues along with the germs. Those tissues are joints, heart valves, skin, and brain.
The initial throat infection that leads to Sydenham's chorea is typically followed by a symptom-free period of 1-5 weeks. The patient then develops an acute case of rheumatic fever (ARF), an inflammatory disease that affects multiple organ systems and tissues of the body. In most patients, ARF is characterized by fever, arthritis in one or more joints, and carditis, or inflammation of the heart. In about 20% of patients, however, Sydenham's chorea is the only indication of ARF. Sydenham's is considered a delayed complication of rheumatic fever; it may begin as late as 12 months after the initial sore throat, and it may start only after the patient's temperature and other physical signs have returned to normal. The average time interval between the pharyngitis and the first symptoms of Sydenham's, however, is eight or nine weeks.
It is difficult to describe a "typical" case of Sydenham's chorea because the symptoms vary in speed of onset as well as severity. Most patients have an acute onset of the disorder, but in others, the onset is insidious, which means that the symptoms develop slowly and gradually. In some cases, the child's physical symptoms are present for four to five weeks before they become severe enough for the parents to consult a doctor. In other cases, emotional or psychiatric symptoms precede the clumsiness and involuntary muscular movements that characterize the disorder. The psychiatric symptoms that may develop in patients with Sydenham's chorea are one reason why it is sometimes categorized as a PANDAS disorder. PANDAS stands for Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections.
Behavioral or emotional disturbances that have been observed with Sydenham's include:
- frequent mood changes
- episodes of uncontrollable crying
- behavioral regression; that is, acting like much younger children
- mental confusion
- general irritability
- difficulty concentrating
- impulsive behavior
Some researchers think that children who have had Sydenham's are at increased risk of developing obsessive-compulsive disorder (OCD). OCD is characterized by obsessions, which are unwanted recurrent thoughts, images, or impulses, and by compulsions, which are repetitive rituals, mental acts, or behaviors. Obsessions in children often take the form of fears of intruders or harm coming to a family member. Compulsions may include such acts as counting silently, washing the hands over and over, insisting on keeping items in a specific order, checking repeatedly to make sure a door is locked, and similar behaviors.
Because rheumatic fever is such a damaging disease, a complete evaluation should be done whenever it is suspected. This includes cultures for streptococci, blood tests, and usually an electrocardiogram (heartbeat mapping to detect abnormalities).
The diagnosis of Sydenham's is also based on the doctor's observation of the patient's involuntary movements. Unlike tics, the movements associated with chorea are not repetitive; and unlike the behavior of hyperactive children, the movements are not intentional. The recent onset of the movements rules out a diagnosis of cerebral palsy. If the doctor suspects Sydenham's, he or she may ask the patient to stick out the tongue and keep it in that position, or to squeeze the doctor's hand. Many patients with Sydenham's cannot hold their mouth open and keep the tongue out for more than a second or two. Another characteristic of Sydenham's is an inability to grip with a steady pressure; when the patient squeezes the doctor's hand, the strength of the grip will increase and decrease in an erratic fashion. This characteristic is sometimes called the "milking sign."
Suspected streptococcal infections must be treated. All the other manifestations of rheumatic fever, including Sydenham's chorea and excluding heart valve damage, remit with the acute disease and do not require treatment. Sydenham's chorea generally lasts for several months.
Most patients with Sydenham's chorea recover after a period of bed rest and temporary limitation of normal activities. In most cases the symptoms disappear gradually rather than stopping abruptly.
Most doctors recommend ongoing treatment with penicillin to prevent a recurrence of rheumatic fever or Sydenham's chorea, although there is some disagreement as to whether this treatment should continue for 5 years after an acute attack or for the rest of the patient's life. The penicillin may be given orally or by injection. Patients who cannot take penicillin may be given erythromycin or sulfadiazine.
Syndenham's chorea usually clears up without complications when the rheumatic fever is treated. The heart valve damage associated with rheumatic fever may lead to heart trouble and require a surgical valve repair or replacement.
In most cases of Sydenham's, the patient recovers completely, although a recurrence is possible. In a very few cases—about 1.5% of patients diagnosed with Sydenham's—there may be increasing muscle stiffness and loss of muscle tone resulting in disability. This condition is occasionally referred to as paralytic chorea
All cases of strep throat in children should be treated with a full 10 days of antibiotics (penicillin or erythromycin). Treatment may best be delayed a day or two to allow the body to build up its own antibodies. In addition, for those who have had an episode of rheumatic fever or have damaged heart valves from any other cause, prophylactic antibiotics should be continued to prevent recurrence.
Arthralgia — Joint pain.
Chorea — A term that is used to refer to rapid, jerky, involuntary movements of the limbs or face that characterize several different disorders of the nervous system, including chorea of pregnancy and Huntington's chorea as well as Sydenham's chorea.
Electrocardiogram — Mapping the electrical activity of the heart.
Insidious — Developing in a stealthy or gradual manner. Sydenham's chorea may have an insidious onset.
PANDAS disorders — A group of childhood disorders associated with such streptococcal infections as scarlet fever and "strep throat." The acronym stands for Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections. Sydenham's chorea is considered a PANDAS disorder.
Pharyngitis — Inflammation of the throat, accompanied by dryness and pain. Pharyngitis caused by a streptococcal infection is the usual trigger of Sydenham's chorea.
Rheumatic fever — Chiefly childhood disease marked by fever, inflammation, joint pain, and Syndenham's chorea. It is often recurrent and can lead to heart valve damage.
St. Vitus' dance — Another name for Sydenham's chorea. St. Vitus was a fourth-century martyr who became the patron saint of dancers and actors during the Middle Ages. He was also invoked for protection against nervous disorders, epilepsy, and the disease that bears his name.
Streptococcus (plural, streptococci) — A genus of spherical-shaped anaerobic bacteria occurring in pairs or chains. Sydenham's chorea is considered a complication of a streptococcal throat infection.
Tonsillitis — Inflammation of the tonsils, which are in the back of the throat.
It is possible to eradicate dangerous GAS bacteria from a community by culturing everyone's throat and treating everyone who tests positive. This is worth doing wherever a case of rheumatic fever appears, but it is expensive and requires many resources.
Beers, Mark H., MD, and Robert Berkow, MD., editors. "Sydenham's Chorea (Chorea Minor; Rheumatic Fever; St. Vitus' Dance)." In The Merck Manual of Diagnosis and Therapy. Whitehouse Station, NJ: Merck Research Laboratories, 2004.
Bhidayasiri, R., and D. D. Truong. "Chorea and Related Disorders." Postgraduate Medical Journal 80 (September 2004): 527-534.
Bonthius, D. J., and B. Karacay. "Sydenham's Chorea: Not Gone and Not Forgotten." Seminars in Pediatric Neurology 10 (March 2003): 11-19.
Dale, R. C., I. Heyman, R. A. Surtees, et al. "Dyskinesias and Associated Psychiatric Disorders following Streptococcal Infections." Archives of Disease in Childhood 89 (July 2004): 604-610.
Herrera, Maria Alejandra, MD, and Nestor GalvezJiminez, MD. "Chorea in Adults." eMedicine February 1, 2002. http://www.emedicine.com/neuro/topic62.htm.
Kim, S. W., J. E. Grant, S. I. Kim, et al. "A Possible Association of Recurrent Streptococcal Infections and Acute Onset of Obsessive-Compulsive Disorder." Journal of Neuropsychiatry and Clinical Neurosciences 16 (Summer 2004): 252-260.
Korn-Lubetzki, I., A. Brand, and I. Steiner. "Recurrence of Sydenham Chorea: Implications for Pathogenesis." Archives of Neurology 61 (August 2004): 1261-1264.
Snider, L. A., and S. E. Swedo. "Post-Streptococcal Autoimmune Disorders of the Central Nervous System." Current Opinion in Neurology 16 (June 2003): 359-365.
American Academy of Child and Adolescent Psychiatry (AACAP). 3615 Wisconsin Avenue, NW, Washington, DC 20016-3007. (202) 966-7300. Fax: (202) 966-2891. http://www.aacap.org.
American Academy of Family Physicians (AAFP). 11400 Tomahawk Creek Parkway, Leawood, KS 66211-2672. (800) 274-2237 or (913) 906-6000. ∗lt;http://www.aafp.org.
National Institute of Neurological Disorders and Stroke (NINDS). NIH Neurological Institute, P. O. Box 5801, Bethesda, MD 20824. (800) 352-9424 or (301) 496-5751. http://www.ninds.nih.gov.
American Academy of Child and Adolescent Psychiatry (AACAP). AACAP Facts for Families, No. 60. Obsessive-Compulsive Disorder in Children and Adolescents. http://www.aacap.org/publications/factsfam/ocd.htm.
National Institute of Neurological Disorders and Stroke (NINDS). NINDS Sydenham Chorea Information Page. http://www.ninds.nih.gov/health_and_medical/disorders/sydenham.htm.
a disorder of the central nervous system that is a manifestation of rheumatic fever; it is usually self-limited and occurs between the ages of 5 and 15 or during pregnancy. It is characterized by involuntary irregular jerky spasmodic movements of the voluntary muscles, ranging from mild to severe; these often begin as awkwardness and uncontrollable facial grimaces that can cause the child considerable embarrassment. Emotional instability and extreme nervousness usually accompany the physical symptoms. Treatment and care are based on relief of symptoms. Complete mental and physical rest are prescribed and a mild sedative or tranquilizer may be given to promote relaxation. The prognosis is good and complete recovery is the rule, although the symptoms and signs can last up to three months. Called also Saint Vitus's dance.
A nervous disorder occurring chiefly in childhood or during pregnancy, closely associated with rheumatic fever, and characterized by rapid, jerky, involuntary movements of the body. Also called Saint Vitus' dance.
Sydenham's choreaSt. Vitus dance Neurology Rapid, involuntary movement of face–grins, grimaces, contortions, tics; extremities–erratic flailing,
if unilateral, hemichorea; hands–repeated partial fisting of the hands or a 'milkmaid's grip'; tongue–muscular fasciculation is likened to a 'bag of worms'; SC is most common in children and adolescents, and a major criterion for diagnosing rheumatic fever–status/post untreated streptococal infection by the Jones' criteria; SC develops after a latency of wks to months, ± accompanied by emotional lability and rheumatic heart disease. See Rheumatic fever.