Definition


Description

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 spherical-shaped 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:


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.

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