Sydenham Chorea

Excerpt

Sydenham chorea, or St. Vitus dance, is a major manifestation of rheumatic fever in up to 40% of patients with rheumatic fever. It is uncommon in the United States but occurs much more frequently in developing countries. This neuropsychiatric disorder is thought to be mediated by an autoimmune process resulting from antigenic mimicry between central nervous system basal ganglia cells and group A β-hemolytic Streptococci antigens. Anti-basal ganglia antibodies that result from the antigenic mimicry attack portions of the brain, especially the dorsal and ventral striatum, and are responsible for the pathological findings and symptoms.

Sydenham chorea is much less common today than in the past, but when it does occur, the symptoms are less severe, and the relapses are less frequent. This may be partially due to aggressive treatment of group A streptococcal infections, improved general hygiene practices, and a reduction in the strain of group A Streptococcus that causes the antigenic mimicry that triggers the disease. The Jones Criteria proposed by TD Jones for the diagnosis of rheumatic fever included Sydenham chorea as a major criterion when diagnosing rheumatic fever. A very high proportion of rheumatic fever patients who develop Sydenham chorea suffer from carditis and develop valvular heart disease.