Published 2022
| Version v1
Publication
A nationwide study on Sydenham's chorea: Clinical features, treatment and prognostic factors: A multicenter cohort study on Sydenham's chorea
Creators
- Orsini A.
- Foiadelli T.
- Magistrali M.
- Carli N.
- Bagnasco I.
- Dassi P.
- Verrotti A.
- Marcotulli D.
- Canavese C.
- Nicita F.
- Capuano A.
- Marra C.
- Fetta A.
- Nosadini M.
- Sartori S.
- Papa A.
- Viri M.
- Greco F.
- Pavone P.
- Simonini G.
- Matricardi S.
- Siquilini S.
- Marchese F.
- De Grandis E.
- Brunenghi B. M.
- Malattia C.
- Bassanese F.
- Bergonzini P.
- Bonuccelli A.
- Consolini R.
- Marseglia G. L.
- Peroni D.
- Striano P.
- Cordelli D.
- Savasta S.
Contributors
Others:
- Orsini, A.
- Foiadelli, T.
- Magistrali, M.
- Carli, N.
- Bagnasco, I.
- Dassi, P.
- Verrotti, A.
- Marcotulli, D.
- Canavese, C.
- Nicita, F.
- Capuano, A.
- Marra, C.
- Fetta, A.
- Nosadini, M.
- Sartori, S.
- Papa, A.
- Viri, M.
- Greco, F.
- Pavone, P.
- Simonini, G.
- Matricardi, S.
- Siquilini, S.
- Marchese, F.
- De Grandis, E.
- Brunenghi, B. M.
- Malattia, C.
- Bassanese, F.
- Bergonzini, P.
- Bonuccelli, A.
- Consolini, R.
- Marseglia, G. L.
- Peroni, D.
- Striano, P.
- Cordelli, D.
- Savasta, S.
Description
Objectives: Sydenham's Chorea (SC) is a neuropsychiatric disorder and a major manifestation of acute rheumatic fever. The erroneous assumption that SC is a benign and self-limiting disease, has led to a lack of high-quality scientific evidence of the therapeutical and prognostic features of SC. Study design: We retrospectively analyzed the medical records of patients <18-years old with SC in 17 Italian pediatric centers. Recorded data included clinical, instrumental and laboratory parameters. Prognostic risk factors including treatment regimens were assessed with univariate and multivariate sub-analysis. Results: We included 171 patients with SC. 66% had generalized chorea, and 34% hemichorea. 81% had carditis (subclinical in 65%). Additional neurological symptoms were reported in 60% of the patients, mainly dysarthria and dysgraphia. 51% had neuropsychiatric symptoms at onset, which persisted after 12 months in 10%. Among psychiatric manifestations, the most common was anxiety disorder/depression (77%). Neurological remission was reached by 93% of the patients at 6 months; 9% relapsed. Patients were treated as follows: 11% penicillin alone, 37% immunomodulatory therapy, 16% symptomatic drugs (i.e. anti-seizure medication, dopamine antagonists) and 37% both symptomatic and immunomodulatory treatment. Neurological outcome did not differ between groups. Patients receiving symptomatic drugs had a higher risk of relapse on multivariate analysis (p = 0.045). Conclusions: Treatment of SC was largely heterogeneous. Based on our results, immunomodulatory therapy did not show higher efficacy at medium term, although it was associated to a slightly lower risk of relapse compared to symptomatic therapy. Longitudinal studies are needed to assess specific risk factors and best treatment options.
Additional details
Identifiers
- URL
- https://hdl.handle.net/11567/1078892
- URN
- urn:oai:iris.unige.it:11567/1078892
Origin repository
- Origin repository
- UNIGE