Abstract
The pathogenesis of Sydenham's (rheumatic) chorea is not well understood. Laboratory tests are not helpful in establishing the diagnosis. The chorea can be successfully treated with haloperidol, an agent known to interfere with the binding of dopamine to its receptors. This suggests that dopamine, and its urine catabolite homovanylic acid (HVA), might be increased in Sydenham's chorea. To test this hypothesis the urines of three patients with the clinical diagnosis of Sydenham's chorea were analyzed for HVA and Vanyllylmandel acid (VMA) during the acute phase of the disease. Urine HVA was 28.07 ± 6.1 mcg per mg. creatinine (m ± SEM). Seven age matched control children had urine HVA levels of 8.8 ± 0.7 mcg per mg creatinine (m ± SEM) (p 0.1). The ratio HVA:VMA was 3.7 ± 0.7 (m ± SEM) in the 3 patients, with chorea and 1.4 ± 0.2 in the control group (p< 0.005). In conclusion, urine HVA is increased in patients with Sydenham's chorea, suggesting increased dopamine, the metabolic precursor of HVA, in the disease. The determination of HVA and HVA:VMA ratio may be helpful in establishing this diagnosis.
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