Revisiting an immunoneuropsychiatry classic: Sydenham’s chorea

Compelling evidence supports that autoantibodies targeting neuronal antigens underly the pathogenesis of SC. In the basal ganglia, autoantibodies react, among other epitopes, with dopamine type 2 (D2) receptors. The resulting dopaminergic signaling dysfunction leads to SC-related motor and neuropsychiatric symptoms. A key gap in the antibody-mediated hypothesis of SC lays on the limited understanding on how peripherally generated autoantibodies reach the basal ganglia. D2 antagonists and valproic acid are the main symptomatic therapeutic strategies for SC. The use of corticosteroids can accelerate the resolution of motor symptoms, and must be considered in severe cases, including chorea paralytica. Plasmapheresis and immunoglobulin have been considered investigational for SC.