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Sydenham chorea

Sydenham chorea is a movement disorder that occurs after infection with certain bacteria called group A streptococcus.

Causes

Sydenham chorea is caused by an infection with bacteria called group A streptococcus. This is the bacteria that causes rheumatic fever (RF) and strep throat. Group A streptococcus bacteria can react with a part of the brain called basal ganglia to cause this disorder. The basal ganglia are a set of structures deep in the brain. They help control movement, posture, and speech.

Sydenham chorea is a major sign of acute RF. The person may currently or recently have had the disease. Sydenham chorea may be the only sign of RF in some people.

Sydenham chorea occurs most often in girls before puberty, but may be seen in boys.

Symptoms

Sydenham chorea mainly involves jerky, uncontrollable and purposeless movements of the hands, arms, shoulder, face, legs, and trunk. These movements look like twitches, and disappear during sleep. Other symptoms may include:

  • Changes in handwriting
  • Loss of fine motor control, especially of the fingers and hands
  • Loss of emotional control, with bouts of inappropriate crying or laughing

Symptoms of RF may be present. These may include high fever, heart problem, joint pain or swelling, skin lumps or skin rashes, and nosebleeds.

Exams and Tests

The health care provider will perform a physical exam. Detailed questions will be asked about the symptoms.

If a streptococcus infection is suspected, tests will be done to confirm the infection. These include:

  • Throat swab
  • Anti-DNAse B blood test
  • Antistreptolysin O (ASO) blood test

Further testing may include:

  • Blood tests such as ESR, CBC
  • MRI or CT scan of the brain

Treatment

Antibiotics are used to kill the streptococcus bacteria. The provider may also prescribe antibiotics to prevent future RF infections. This is called preventive antibiotics, or antibiotic prophylaxis.

Severe movement or emotional symptoms may need to be treated with medicines.

Outlook (Prognosis)

Sydenham chorea usually clears up in a few months. In rare cases, an unusual form of Sydenham chorea may begin later in life.

Possible Complications

No complications are expected.

When to Contact a Medical Professional

Call your provider if your child develops uncontrollable or jerky movements, especially if the child has recently had a sore throat.

Prevention

Pay careful attention to children's complaints of sore throats and get early treatment to prevent acute RF. If there is a strong family history of RF, be especially watchful, because your children may be more likely to develop this infection.

Alternative Names

St. Vitus dance; Chorea minor; Rheumatic chorea; Rheumatic fever - Sydenham chorea; Strep throat - Sydenham chorea; Streptococcal - Sydenham chorea; Streptococcus - Sydenham chorea

References

Jankovic J. Parkinson disease and other movement disorders. In: Daroff RB, Jankovic J, Maziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 96.

Lang AE. Other movement disorders. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 410.

Shulman ST, Bisno AL. Nonsuppurative poststreptococcal sequelae. In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases, Updated Edition. 8th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 200.

Review Date 5/30/2016

Updated by: Amit M. Shelat, DO, FACP, Attending Neurologist and Assistant Professor of Clinical Neurology, SUNY Stony Brook, School of Medicine, Stony Brook, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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