Sydenham's chorea

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An infectious disease of the central nervous system, appearing after a streptococcal infection, with subsequent rheumatic fever, characterised by involuntary purposeless contractions of the muscles of the trunk and extremities.

Description

An infectious disease of the central nervous system commonly occurring between 5 and 15 years of age, but it may also occur later in life or be associated with pregnancy (chorea gravidarum). Affects females about twice as often as males, appearing after a streptococcal infection, with subsequent rheumatic fever. It may cause valvular disturbances. It is characterised by involuntary purposeless contractions of the muscles of the trunk and extremities, initially in one limb; face movements that simulate smirking expressions, anxiety; impairment of memory and sometimes of speech. May be associated with emotional instability and inappropriate behaviour. Movements cease during sleep. Recovery takes place in 2 to 6 months. Besides in rheumatic fever, it has also been reported in association with hyperthyroidism, systemic lupus erythematosus and some other common erythemas.

The term Chorea Saint Viti was originally used for dancing mania, a form of hysteria common in Europe in the 15th and 16th century. The dancing mania became known as chorea magna, and Sydenham’s disease as chorea minor – Sydenham’s chorea. This mania had greatly declined in Sydenham’s time, and it is unlikely that he himself observed the phenomenon. Paracelsus (Theophrastus Philippus Aureolus Bombastus von Hohenheim, 1493-1541) probably did, and termed it chorea naturalis.

First described by the German physician Gregor Horst (Horstius), 1578-1636, in 1625.

" This is a kind of convulsion, which attacks boys and girls from the tenth year to the time of puberty. It first shows itself by limping or unsteadiness in one of the legs, which the patient drags. The hand cannot be steady for a moment. It passes from one position to another by a convulsive movement, however, much the patient may strive to the contrary."
Thomas Sydenham

"Before he can raise a cup to his lips, he makes as many gesticulations as a mountebank; since he does not move in a straight line, but has his hand drawn aside by spasms, until by some good fortune he brings it at last to his mouth. He then gulps it off at once, so suddenly and so greedily as if he were trying to amuse the lookers-on."
Thomas Sydenham

Chorea major, or chronic degenerative chorea, has been entered as Huntington's chorea, under George Huntington, American physician, 1850-1916.

Bibliography

  • T. Sydenham:
    Schedula monitoria de novae febris ingressu. Londini, Kettilby, 1686, pp. 25-28.

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