Infectious central nervous system disease which is one of the manifestations of rheumatic disease appearing 4 to 8 weeks after infection by a  beta-hemolytic group A streptococcus. This is probably a post-infectious autoimmune phenomenon by molecular mimesis between the pathogen and the host, and with a genetic predisposition. Rare disease in the developed countries but still frequent in the poor countries (+ migrant populations) where it especially affects girls aged around 8 to 10 years. Average duration 3 months. Risk of recurrence by about 20%.

Signs:

-        fever,

-         sometimes arthralgia, cardiac symptoms

-        classical form: emotional lability, sudden and quick movements exaggerated by emotions, especially at the root of the limbs and neck, important dysarthria

-        rough form: discrete and slow movements, clumsiness, motor instability

-        flaccid hemichorea (1/3 of cases): if involuntary movements and muscle hypotonia are confined to one side

-        electric chorea, Bergeron chorea or Dubini syndrome, manifested by the appearance of myoclonus

Treatment: symptomatic; haloperidol, sometimes corticosteroids for the severe forms.

Anesthetic implications:

echocardiography

References : 

-        Ghram N, Allani C, Oudali B, Fitouri Z, Ben Becher S.
Chorée de Syndeham chez l’enfant.
Arch Pédiatr 1999 ; 6 : 1048-52.

-        Brousse V, Bahi-Buisson N, Lucet V, Deloche A, Abadie V.
Chorée aiguë post-streptococcique : une étrange réaction après chirurgie d’une insuffisance mitrale rhumatismale .
Arch Pédiatr 209 ; 16 : 1124-8.

Updated: June 2016