Sydenham's chorea

For the mysterious historical phenomenon also known as "St. Vitus' dance", see Dancing mania.

Other names

Sydenham chorea (SC), Rheumatic encephalitis, Chorea minor, St Vitus' dance.

Pathophysiology

Sydenham's chorea is a neurological disorder of childhood resulting from infection via Group A beta-hemolytic streptococcus (GABHS), the bacterium that causes rheumatic fever. 

Clinical features

SC is characterized by rapid, irregular, and aimless involuntary movements of the arms and legs, trunk, and facial muscles. 

Sex distribution

It affects girls more often than boys and typically occurs between 5 and 15 years of age.

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Signs and symptoms

Some children will have a sore throat several weeks before the symptoms begin, but the disorder can also strike up to 6 months after the fever or infection has cleared.  Symptoms can appear gradually or all at once, and also may include uncoordinated movements, muscular weakness, stumbling and falling, slurred speech, difficulty concentrating and writing, and emotional instability.  The symptoms of SC can vary from a halting gait and slight grimacing to involuntary movements that are frequent and severe enough to be incapacitating.  The random, writhing movements of chorea are caused by an auto-immune reaction to the bacterium that interferes with the normal function of a part of the brain (the basal ganglia) that controls motor movements. 

Distribution

Due to better sanitary conditions and the use of antibiotics to treat streptococcal infections, rheumatic fever, and consequently SC, are rare in North America and Europe.  The disease can still be found in developing nations.

Treatment

There is no specific treatment for SC.  For people with the mildest form, bed rest during the period of active movements is sufficient.  When the severity of movements interferes with rest, sedative drugs, such as barbiturates or benzodiazepines, may be needed.  Antiepileptic medications, such as valproic acid, are often prescribed.  Doctors also recommend that children who have had SC take penicillin over the course of the next 10 years to prevent additional manifestations of rheumatic fever.

Prognosis

Most children recover completely from SC, although a small number will continue to have disabling, persistent chorea despite treatment.   The duration of symptoms varies, generally from 3 to 6 weeks, but some children will have symptoms for several months.  Cardiac complications may occur in a small minority of children, usually in the form of endocarditis.

In a third of the children with the disease, SC will recur, typically 1 ½ to 2 ½ years after the initial attack.  Researchers have noted an association between recurrent SC and the later development of the abrupt onset forms of obsessive-compulsive disorder, attention deficit/hyperactivity disorder, tic disorders, and autism, which they call PANDAS, for Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus infection.

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Classification	ICD-10: I02 ICD-9-CM: 392 MeSH: D002819 DiseasesDB: 29245 SNOMED CT: 46826000
External resources	MedlinePlus: 001358

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External links

• Comprehensive information from the National Institute of health.
  

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