Sydenham’s Chorea (Rheumatic Chorea)

Sydenham’s chorea (Rheumatic chorea)

Sydenham’s chorea is one of the major manifestations of rheumatic fever. It is a quasi purposive, non repetitive involuntary movement. Sydenham’s chorea is often associated with hypotonia and emotional instability. Rheumatic chorea is characterised by various signs like pronator sign, milking sign of the hand and jack in the box sign of the tongue. Sydenham’s chorea is a late manifestation of rheumatic fever in that it can occur months after a group A beta-hemolytic streptococcal infection, when other manifestations of acute phase like arthritis have subsided.

When the history is atypical or when only hemichorea is present investigations like a magnetic resonance imaging of the brain are needed to exclude other neuropathologies [1]. Immune mediated injury of the basal ganglia is thought to be the mechanism of Sydenham’s chorea. This is supported by the finding of antibodies against the neurons of the caudate nucleus. This results in imbalance between dopaminergic and cholinergic systems which may cause the involuntary choreiform movements. This explanation can account for the beneficial effects of certain drugs used in the treatment of chorea. Sodium valproate, carbamazepine and haloperidol have been used in those with disturbing chorea [2].

References

1. Daniëlla M Oosterveer, Wilhelmina C T Overweg-Plandsoen, Raymund A C Roos. Sydenham’s chorea: a practical overview of the current literature. Pediatr Neurol. 2010 Jul;43(1):1-6.
2. Gordon N. Sydenham’s chorea, and its complications affecting the nervous system. Brain Dev. 2009;31:11-4.