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NEUROLOGY 1980;30:331
© 1980 American Academy of Neurology

Sydenham chorea

An update

Paul A. Nausieda, M. D., Burton J. Grossman, M. D., William C. Koller, M. D., Ph.D., William J. Weiner, M. D. and Harold L. Klawans, M. D.

Department of Neurological Sciences (Drs. Nausieda, Koller, Weiner, and Klawans), Rush-Presbyterian-St. Luke's Medical Center, and the Department of Pediatrics (Dr. Grossman), La Rabida Children's Hospital and Research Center, University of Chicago, Chicago, IL.

To document possible changing characteristics of Sydenham chorea, we reviewed records of 240 patients with this diagnosis who were seen between 1951 and 1976. A dramatic progressive decline in the number of cases was observed. The syndrome occurred mainly in childhood. Female predominance was apparent only after age 10. There was a high familial incidence for both chorea and rheumatic fever. Most patients had generalized chorea, and fewer than 20% had hemichorea. Dysarthria, probably of extrapyramidal origin, was frequent but neurologic abnormalities other than diffuse encephalopathy were rare. One-third of the patients had coexisting heart disease. Repeat attacks of Sydenham chorea occurred, but the recurrence rate was much less than noted in previous studies.

Address correspondence and reprint requests to Dr. Koller, 1725 West Harrison Street, Chicago, IL 60612.

Accepted for publication August 10, 1979

This paper was presented at the thirty-first annual meeting of the American Academy of Neurology, Chicago, April 1979.

This work was supported in part by grants from the United Parkinson Foundation and the Boothroyd Foundation, Chicago, Illinois.




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