NORD gratefully acknowledges Michael S. Okun, MD, Co-director of the University of Florida Movement Disorders Center; National Medical Director of the National Parkinson Foundation, for assistance in the preparation of this report.
Sydenham chorea is a rare neurological disorder characterized by rapid, involuntary, purposeless movements, especially of the face, feet and hands. Additional symptoms may include muscle weakness and emotional or behavioral problems. Sydenham chorea most often affects children and adolescents. Sydenham chorea usually develops following Streptococcal infection and may occur as an isolated finding or as a major complication of acute rheumatic fever. The movement disorder is considered an autoimmune disorder, meaning it occurs when the body's immune system (which normally responds to a foreign substance) mistakenly attacks healthy tissue.
The specific symptoms of Sydenham chorea may vary greatly from one person to another. Most cases follow a Streptococcal infection. Streptococcus is a group of bacteria that can cause several different infections including strep throat. Some individuals with Sydenham chorea exhibit symptoms of Streptococcal infection such as a sore throat (pharynitis) or fever. Symptoms of Sydenham chorea may appear anywhere from 1 to 6 months following Streptococcal infection.
The onset of the abnormal movements (chorea) that characterize Sydenham chorea may be sudden (acute) or gradual. Initially, children with Sydenham chorea may be mistakenly thought of as extremely fidgety, clumsy or uncoordinated and awkward. Affected children may have difficulty with handwriting or may appear to be “making faces”.
Chorea most often affects the face, arms, and hands. These rapid, random, uncontrolled, jerky movements may first be noticed during periods of stress, fatigue or excitement, all of which usually worsen the condition. The abnormal choreic movements may vary in severity. Sydenham chorea can be associated with mild symptoms such as poor coordination or it can severely disrupt daily life. Initially, chorea may only affect one side of the body or may be noticeably more severe on one side of the body (hemichorea). In severe cases, both sides of the body are noticeably affected. Choreic movements may gradually become more frequent and, in some cases, may eventually become almost continuous. In most cases, chorea usually disappears during sleep.
Chorea can potentially impede or interfere with voluntary movements, making it difficult for affected children to perform basic tasks or activities. Some children may have difficulty performing such tasks as getting dressed or feeding themselves, may frequently drop or spill objects, and may have a clumsy or uncoordinated manner of walking (abnormal gait). Some affected children may be unable to sustain a voluntary movement (motor impersistence) such as being unable to keep their tongue sticking out.
In addition to choreic movements, individuals with Sydenham chorea may develop muscle weakness, slurred speech (dysarthria), and diminished muscle tone (hypotonia). Less frequently, additional symptoms can be associated with Sydenham chorea including motor tics, abnormal reflexes, headaches and seizures. Muscle weakness associated with Sydenham chorea is usually mild or moderate. In some extremely rare cases (less than 2 percent), muscle weakness may be profound and affected children may become bedridden, a condition sometimes referred to as paralytic chorea.
Children with Sydenham chorea often display abnormal behavioral or emotional problems including obsessive-compulsive disorder, attention deficit and hyperactivity, cognitive issues, irritability, confusion, and excessive emotional reactions and frequent mood changes (emotional lability) such as uncontrollable crying or laughing at inappropriate times. Obsessive-compulsive disorder is a condition characterized by recurrent habitual obsessive or compulsive thoughts or actions. These obsessions and compulsions may become very distressing and time-consuming. In severe cases, they can significantly interfere with a person’s normal routine, occupational functioning, usual social activities or relationships with others.
Because Sydenham chorea sometimes occurs as a complication of rheumatic fever, some individuals will have additional symptoms commonly associated with rheumatic fever. Such symptoms include fever, arthritis, and inflammation of the heart (carditis), specifically the membrane lining the inside of the heart (endocarditis). In some cases, the valve (mitral valve) between the left upper and left lower chambers of the heart may be damaged.
Sydenham chorea usually runs a specific course (self-limited) and the symptoms usually resolve within three weeks to three months. However, symptoms may last longer in some cases or may come and go (wax and wane) for approximately a year. Occasionally, the symptoms of Sydenham chorea have recurred later during adult life, particularly in times of heightened stress or excitement.
Researchers believe that Sydenham chorea is an autoimmune disorder. Most cases of Sydenham chorea develop following a Streptococcal infection or rheumatic fever. An autoimmune disorder occurs when the body’s immune system mistakenly attacks healthy tissue. In Sydenham chorea, Streptococcal infection induces an immune system response. The body produces antibodies to combat the infection. For unknown reasons, these antibodies also target certain cells in the brain, specifically cells of the basal ganglia (the part of the brain that controls motor movements). Researchers believe this ultimately leads to the characteristic symptoms of Sydenham chorea.
The exact underlying mechanisms that cause Sydenham chorea are not fully understood. Researchers believe that antigens (substances that are capable of stimulating an immune system response) on Streptococcal cells are similar to antigens found on cells of the basal ganglia. When the immune system creates antibodies to combat the Streptococcal infection, the antibodies also mistakenly attack healthy, brain cells in genetically predisposed individuals. This process is referred to as a cross-reaction. More research is necessary to determine the exact cause of Sydenham chorea and the exact, underlying mechanisms by which symptoms develop.
According to most studies, Sydenham chorea affects women more often than men. Sydenham chorea usually develops in children between the ages of 5-15, usually following Streptococcal infection. One distinct finding of Sydenham chorea is that it is almost never seen in children below 5 years of age. Although Sydenham chorea usually affects children and adolescents, the disorder has been reported in individual more than 30 years of age. 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 60s and 70s, although there have been occasional outbreaks in the United States. Rheumatic fever is still common in developing countries. Sydenham chorea is the most common cause of acute chorea during childhood in the United States. Sydenham chorea remains a major public health problem in many developing countries.
A diagnosis of Sydenham chorea is made based upon identification of characteristic symptoms (e.g., choreic movements), a detailed patient history, and a thorough clinical evaluation. In some cases, certain imaging techniques such as magnetic resonance imaging (MRI) may aid in the diagnosis of a particular chorea, but most of the time in Sydenham chorea it will be normal. There is some interest in MR spectroscopy, but it remains investigational. Signs normally associated with rheumatic fever and Streptococcal infection may be useful in diagnosing Sydenham chorea. However, because the onset of Sydenham chorea usually occurs well after the infection, the characteristic signs of rheumatic fever or Streptococcal infection may no longer be present.
Individuals who are diagnosed with Sydenham chorea should receive an evaluation for inflammation of the heart (carditis), which is sometimes associated with rheumatic fever and Sydenham chorea.
There is no known cure for Sydenham chorea. Some mild cases may not require intervention because the disorder is self-limited and usually resolves on its own within three weeks or three months. In some cases, sedatives (e.g., benzodiazepine) have been used to help reduce the intensity of involuntary muscle movements. Additional medications may be used to control abnormal movements in affected individuals including anti-seizure drugs (anticonvulsants) such as valproic acid.
Dopamine-receptor blocking drugs may be used for individuals who do not respond to anticonvulsants or in individuals who develop more severe chorea syndromes. These drugs include haloperidol, fluphenazine and pimozide. Dopamine-receptor blocking drugs may be associated with certain side effects such as the development of tardive dyskinesia, a neurological movement disorder typically characterized by abnormal movements of the jaw, lips and tongue. Most practitioners now use milder dopamine blocking drugs such as risperdone or dopamine depleting agents such as tetrabenazine in an effort to avoid acute and chronic side effects. The rule is to not treat unless absolutely necessary as many cases will resolve spontaneously.
An important aspect in the treatment of Sydenham chorea is the preventive (prophylactic) use of penicillin or an equivalent drug that is active against Streptococcal infection. The use of penicillin can decrease the risk of bacterial infection of the heart (carditis). It is unproven whether prophylactic treatment of Streptococcal infection prevents recurrence of Sydenham chorea. The use of antibiotics is also controversial and more evidence base research is needed to determine its overall effectiveness in Sydenham chorea.
Additional therapies have been used to treat individuals with Sydenham chorea include corticosteroids, intravenous immunoglobulins, and plasma exchange. More research is necessary to determine the long-term safety and effectiveness of such therapies in treating individuals with Sydenham chorea.
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FROM THE INTERNET
Worldwide Education and Awareness for Movement Disorders (WE MOVE). Sydenham’s Chorea. December 7, 2008. Available at: http://www.wemove.org/syd/default.htm Accessed On: January 20, 2010.
National Institute of Neurological Disorders and Stoke. Sydenham Chorea Information Page. February 14, 2007. Available at: http://www.ninds.nih.gov/disorders/sydenham/sydenham.htm Accessed On: January 20, 2010.
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