TY - JOUR
T1 - A nationwide study on Sydenham's chorea
T2 - Clinical features, treatment and prognostic factors: A multicenter cohort study on Sydenham's chorea
AU - Orsini, Alessandro
AU - Foiadelli, Thomas
AU - Magistrali, Mariasole
AU - Carli, Niccolò
AU - Bagnasco, Irene
AU - Dassi, Patrizia
AU - Verrotti, Alberto
AU - Marcotulli, Daniele
AU - Canavese, Carlotta
AU - Nicita, Francesco
AU - Capuano, Alessandro
AU - Marra, Chiara
AU - Fetta, Anna
AU - Nosadini, Margherita
AU - Sartori, Stefano
AU - Papa, Amanda
AU - Viri, Maurizio
AU - Greco, Filippo
AU - Pavone, Piero
AU - Simonini, Gabriele
AU - Matricardi, Sara
AU - Siquilini, Sabrina
AU - Marchese, Francesca
AU - De Grandis, Elisa
AU - Brunenghi, Bernadette Marrè
AU - Malattia, Clara
AU - Bassanese, Francesco
AU - Bergonzini, Patrizia
AU - Bonuccelli, Alice
AU - Consolini, Rita
AU - Marseglia, Gian Luigi
AU - Peroni, Diego
AU - Striano, Pasquale
AU - Cordelli, Duccio
AU - Savasta, Salvatore
N1 - Publisher Copyright:
© 2021
PY - 2022/1
Y1 - 2022/1
N2 - 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.
AB - 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.
KW - Immunotherapy
KW - Intravenous immunoglobulins
KW - Italy
KW - Neuropsychiatric symptoms
KW - Streptococcus
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U2 - 10.1016/j.ejpn.2021.11.002
DO - 10.1016/j.ejpn.2021.11.002
M3 - Article
AN - SCOPUS:85118895479
VL - 36
SP - 1
EP - 6
JO - European Journal of Paediatric Neurology
JF - European Journal of Paediatric Neurology
SN - 1090-3798
ER -