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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.