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Symptomatic choreic syndromes 

Symptomatic choreic syndromes

Ivan Donaldson

, C. David Marsden

, Susanne A. Schneider

, and Kailash P. Bhatia

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date: 03 July 2022

There is no precise definition of Sydenham’s chorea. The disorder as described by Sydenham consisted of chorea in late childhood, adolescence, or early adult life. As it is now known that rheumatic chorea was the commonest variety in this age group it is assumed that this was the disorder he described. Chorea at this age, as in the adult, can have a variety of causes including a number of systemic disorders. Some authors have used the term ‘Sydenham’s chorea’ to cover all such cases (Colony and Malamud 1956, Paradise 1960) while others have been more restrictive. This problem of definition is increased by the fact that in many cases of so-called pure chorea there may be no other clinical evidence of rheumatic fever and laboratory tests may not suggest recent streptococcal infection. Follow-up of these cases, however, has shown that approximately a quarter develop rheumatic valvular disease (Aron et al. 1965 ). It is thus apparent that many such cases are post-streptococcal although corroborative evidence for this may be lacking during the choreic stage. Most, in particular historical, reported that series of patients with Sydenham’s chorea contain a number of cases in which a streptococcal aetiology is assumed.

In accordance with the current trend, the use of the term ‘Sydenham’s chorea’ in this book is restricted to only those cases that follow a streptococcal infection and Sydenham’s chorea is the prototype of chorea resulting from immune mechnisms. In clinical practice this can be verified by finding other clinical manifestations of rheumatic fever or evidence of recent streptococcal infection, as in the revised Jones criteria (Special Writing Group of the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease 1992). The Jones criteria were first established in 1944 and they have been modifi ed four times since then. The most recent modification was in 1992. These criteria outline major and minor manifestations of the disease.

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