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Sydenham's Chorea (Chorea Minor; Rheumatic Chorea; St. Vitus' Dance) A CNS disease, often of insidious onset but of finite duration, characterized by involuntary, purposeless, nonrepetitive movements and subsiding without neurologic residua. Etiology, Epidemiology, and Incidence Sydenham's chorea is generally regarded as an inflammatory complication of group A -hemolytic streptococcal infection, which causes rheumatic fever (see also Rheumatic Fever in Ch. 270). Chorea occurs in up to 10% of cases of rheumatic fever. The illness is probably immune-mediated: streptococcal antigens resemble neuronal tissue antigens, and cross-reactive antibodies bind to nerve tissue, which provokes an inflammatory cascade and tissue injury. The disease is more common in females than in males and in childhood. The incidence ratio is even more pronounced in adolescence, as affected populations are composed almost entirely of females. Chorea usually occurs (in temperate climates) in the summer and early fall, after the spring and early summer peak incidence of rheumatic fever. Symptoms and Signs After streptococcal infection, the interval before symptom onset (sometimes up to 12 mo) is longer than that of other manifestations of rheumatic fever, and chorea may begin when (or after) other clinical and laboratory features return to normal. It does not typically occur simultaneously with arthritis but occurs frequently with carditis. The patient develops rapid, purposeless, nonrepetitive, involuntary movements that disappear with sleep and may involve all except the ocular muscles. Voluntary movements are abrupt, with impaired coordination. Facial grimacing is common. In mild cases, the patient may appear clumsy and may have slight difficulties in dressing and feeding. The neurologic examination shows no defect in muscle strength or sensory perception except for an occasional pendulous knee jerk. The course is variable and difficult to delineate precisely because of its insidious onset and gradual cessation. A month or more may elapse before the movements become intense enough to make the patient or parents seek medical attention. The condition may end within another 3 mo but occasionally lasts 6 to 12 mo. Laboratory Findings and Diagnosis Aside from occasional lingering evidence of previous streptococcal infection, chorea has no characteristic laboratory features. The CSF is usually unremarkable, and the EEG shows no more than nonspecific dysrhythmias. The diagnosis frequently is entirely clinical. The irregular, involuntary movements of this disorder are pathognomonic. They resemble those of cerebral palsy but can be distinguished by a history of recent onset. Other conditions that must be differentiated are habit spasms, which are repetitive, and the movements of hyperkinetic children, which are purposeful. Huntington's chorea is usually associated with a family history and appears in adulthood. The Parkinson-like side effects of tranquilizers, given to control a hyperactive child, may confuse the diagnosis of chorea until the drugs are discontinued and the unaltered choreic movements can be noted. Chorea can also occur in SLE. Delayed-onset chorea is the only instance in which Jones criteria (see Ch. 270) do not have to be fulfilled to make the diagnosis of rheumatic fever. This is possible because chorea can occur many months after the streptococcal exposure, at a time when arthritis, carditis, and evidence of prior streptococcal infection are absent. Treatment In extreme cases, the patient may need vigorous sedation and protection to avoid self-injury from flailing arms or legs. No drug is consistently effective. When movements are severe, a benzodiazepine or an antipsychotic drug such as haloperidol or risperidone should be titrated to the lowest effective dose that moderates the movements. Dosing for benzodiazepines is as follows: diazepam 0.04 to 0.1 mg/kg/dose po q 6 h for children and 2 to 10 mg/dose po q 6 h for adults; lorazepam 0.05 mg/kg/dose po q 8 h for children and 1 to 2 mg po q 8 h for adults. Haloperidol may help with severe choreiform movements at a dosage of 0.01 to 0.03 mg/kg/day po given bid to tid but may cause tardive dyskinesia. Risperidone may have a lower risk of tardive dyskinesia, but use is approved by the FDA only for adults. Dosing is 0.5 mg bid, then titrated in 0.5-mg increments until control is achieved. If these fail, a corticosteroid may be given in the dosage described for rheumatic fever (see in Ch. 270). Although supportive studies are scarce, anecdotal reports as well as theory suggest that corticosteroids may help accelerate improvement. Chorea is best regarded and treated as a transitory event. Patients, parents, and others (eg, friends, nurses, teachers, classmates) should be reassured that the ailment will ultimately subside without residual damage and that the temporary impairment of motor functions will not affect intellectual capacity. Patients should miss school only if movements are uncontrollably severe and should return to school as soon as they can manage the necessary locomotion and residual dysfunction is minimal. Many of the so-called psychologic effects previously ascribed to chorea were due not to the disease itself, but to the associated scholastic deprivation and to the patients' anxiety and dismay at the bizarre movements and at the reactions they invoke in people who do not understand. .