What is it?

Rheumatic chorea or Sydenham chorea (SC) is a major clinical sign of acute rheumatic fever (ARF) and is the most common form of acquired chorea in childhood. It is a disorder in which the child starts manifesting with involuntary brief, random, and irregular movements of the limbs and face. Other symptoms may include emotional lability, and hypotonia. Chorea is commonly present in rheumatic fever especially in developing countries. It is more common to see in children from ages 5 to 13 years, and in females rather than males. There appears to be a familial predisposition for chorea. The onset of chorea usually occurs one to eight months after the beginning of the infection with Strep group A.Symptoms improves gradually, typically lasting 3-4 months. Most patients experience full recovery.

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Signs & symptoms

Chorea characterized by rapid, irregular, and aimless involuntary movements of the arms and legs, trunk, and facial muscles. If no other symptom of rhematic fever is present, mild cases of SC may be wrongly diagnosed as behavioral or emotional disorders, Tourette syndrome, or clumsiness. Chorea usually aggravates over hours to days and symptoms typically improve with sleep. The chorea usually is generalized but may be more prominent on one side. There are also psychiatric symptoms for rhematic chorea such as irritability, frequent mood changes, inappropriate behavior, obsessive-compulsive behavior, and inability to concentrate. These symptoms typically occur during the first two months of rheumatic fever.

Diagnosis

In order to diagnose SC, an acute rheumatic fever (ARF) diagnosis must be done. Testing for group A streptococcal (GAS) can be done by throat culture, but it will typically be negative by the time the chorea will appear. In addition to the throat culture, blood tests for both streptococcal antibodies antistreptolysin O (ASO) and antideoxyribonuclease (anti-DNAse) B should be obtained to assess for preceding streptococcal infection. Either or both antibodies may remain elevated for months after a GAS infection. IF SC is suspicioned, a cardiac evaluation should be made. Also, the doctor can check for inflammatory markers in the blood, such as CRP and ESR. In rare cases, where the diagnose isn't known neuroimaging and CSF analysis can be performed.

Treatment

Patients diagnosed with SC receive antibiotics treatment, penicillin G , to eradicate GAS carriage and also as the first dose of secondary prophylaxis. Treatment of chorea can be treated whie dopamine (D2) receptor blocker like haloperidol anti-epileptic drugs or immune modulation drugs such as corticosteroids.

☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.

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