Sydenham’s chorea: what is it?

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Sydenham’s chorea is a rare complication of acute rheumatic fever caused by strep throat. It causes sudden muscle movements, changes in mood and behavior, and affects language and cognition. Treatment includes antibiotics and anticonvulsants, and prevention involves early treatment of strep throat.

Sydenham’s chorea is a complication that can occur with strep throat if this disease develops into acute rheumatic fever (ARF). It is not a very common disease in most developed countries because there is access to diagnostic tools and antibiotics to treat strep in its early stages. Where these are not available or when the diagnosis of strep goes undiagnosed, approximately 20% of ARF cases may develop this complication of rheumatic fever. This disease is significant, causing sudden muscle movements that can be violent and dramatic changes in mood, cognition or behavior. There is a treatment and many people with this condition make a full recovery with the right medical care.

The presence of strep bacteria is thought to cause the body to produce an autoimmune response that begins to negatively affect some form of healthy function. Inflammation can occur in parts of the brain and other areas, and this is associated with a higher than normal production of certain antigens. While the total cause of Sydenham’s chorea is not fully understood, it is certain that the body’s response to strep germs tends to be clinically negative, which can lead to the symptoms associated with this condition.

The first symptoms of Sydenham’s chorea usually occur after the presence of some manifestations of acute rheumatic fever. These are fever, inflammation of the heart, joint swelling and pain, rash, and swelling or lumps that develop around some joints. Symptoms of ARF occur on average two months after the occurrence of the sore throat, but sometimes it can take much longer for these symptoms to develop.

When Sydenham’s chorea develops as a complication of ARF, there are many symptoms that can indicate this. As mentioned, sudden violent movements, often called choreic movements, can occur. Various areas of the body may find it difficult to move smoothly, and people may seem excessively clumsy or may have difficulty performing motor tasks such as writing. Sometimes grimaces or facial gestures appear and these may be more active when people are awake and excited.

Things like language can also be affected, and mood and cognition are definitely affected. People may have outbursts of emotion, be difficult to comfort, have trouble concentrating, be hyperactive, or exhibit frequent confusion. There may be some regression in maturity levels, most noted when this disease affects children and adolescents. Some people also experience psychotic episodes when they have Sydenham’s chorea.

There are two treatment approaches to manage these symptoms. The first is to clear the strep infection with antibiotics, although this may not cause chorea symptoms to disappear immediately. Especially to tame movement disorders, some anticonvulsants are often recommended, such as valproic acid (Depakote®). As the antibiotic fights off the strep and the body recovers, continued treatment is usually stopped.

While the most obvious signs of Sydenham’s chorea are usually cured within months, some people have the disease relapse a few years later, which isn’t common. Doctors support ARF prevention by paying attention to illnesses that can be strep. Strep requires early treatment to avoid progression to ARF.




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