Treatment of Sydenham's Chorea with Intravenous Immunoglobulin, Plasma Exchange, or Prednisone

Abstract

Sydenham's chorea has been established as a postinfectious autoimmune neuropsychiatric disorder. Corticosteroids have been used to treat patients with severe disease but are not always effective, and relapses are frequent after cessation. Eighteen subjects were entered into this randomized-entry controlled trial designed to determine if intravenous immunoglobulin or plasma exchange would be superior to prednisone in decreasing the severity of chorea. Mean chorea severity for the entire group was significantly lower at the 1-month follow-up evaluation (overall 48% improvement). Although the between-group differences were not statistically significant, clinical improvements appeared to be more rapid and robust in the intravenous immunoglobulin and plasma exchange groups than in the prednisone group (mean chorea severity scores decreased by 72% in the intravenous immunoglobulin group, 50% in the plasma exchange group, and 29% in the prednisone group). Larger studies are required to confirm these clinical observations and to determine if these treatments are cost-effective for this disorder.

The Immunobiology of Tourette's Disorder, Pediatric Autoimmune Neuropsychiatric Disorders Associated withStreptococcus, and Related Disorders: A Way Forward

“…Thus far, there has not been a randomized double-blind study of corticosteroids to treat PANDAS. However, Garvey et al (2005) reported the results of a randomized clinical trial for SC. In this study, clinical improvements appeared to be more rapid and robust in the IVIG and PE groups than in the prednisone group (mean chorea severity scores decreased by 72% in the intravenous immunoglobulin group, 50% in the PE group, and 29% in the prednisone group).…”

Antibody binding to neuronal surface in Sydenham chorea, but not in PANDAS or Tourette syndrome

“…Reports of the presence of serum autoantibodies and the therapeutic benefit of plasma exchange suggest that SC may be due to a pathogenic antibody response. [1][2][3][4] Additionally, an outbreak of streptococcal pharyngitis associated with tic disorders led to the hypothesis that some cases of Tourette syndrome (TS) and OCD are precipitated by streptococcus infections. This led to the term pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS).…”

Evidence-based guideline update: Plasmapheresis in neurologic disorders

“…In a randomized, controlled study, 18 children with Sydenham chorea received plasmapheresis, IVIg, or prednisone. 27 Investigators were not blinded as to which patients underwent plasmapheresis; therefore, this is a Class III study. The primary outcome measures were chorea severity as measured by a 6-item chorea severity scale and the ability to perform selected activities of daily living.…”