SYDENHAM'S CHOREA: A NEW APPROACH TO AN OLD DISEASE

Objectives: Medical treatment of Sydenham's chorea (SC) remains symptomatic. Intravenous immunoglobulins (IVIG) may have a role. We compared the outcome of patients receiving standard protocol to patients receiving IVIG in addition.

Methods: Between 2002 and 2005 patients presenting with moderate to severe SC were randomised to either IVIG protocol or standard protocol (penicillin and low dose haloperidol). Outcome measures of clinical state and features of SC were taken by the investigator and a blinded observer at onset, one, three and six months. Spectroscopy studies were performed at onset and one month. Scans were reviewed with intervention group blinded. Specific features of basal ganglia hyper- or hypoperfusion were recorded along with other areas of the cortex.

Results: Ten children presented (6 M: 4 F) (median age122 mths); IVIG protocol (n=5) (3M:2F) and standard protocol (n=5)(3M:2F). There was no statistical difference between the two group's initial clinical scores. Patients who received IVIG had a statistically improved clinical score compared to the standard protocol group at 3 months. The spectroscopy studies (n=9) identified changes in the basal ganglia in all: hyperperfusion n=2, asymmetry n=2, hypoperfusion n=5. Eight patients had additional areas of altered perfusion. At the one month follow-up, one patient in the IVIG group had definite improvement in the BG and all had clear improvement in the rest of the cortex. Of the standard group studied (n=3) there was progression in abnormal perfusion in all patients.

Conclusion: The indications for IVIG in a resource poor country are debatable, but in our small cohort improved clinical outcome within three months was demonstrated.