A nationwide study on Sydenham's chorea: Clinical features, treatment and prognostic factors

Orsini, Alessandro; Foiadelli, Thomas; Magistrali, Mariasole; Carli, Niccolò; Bagnasco, Irene; Dassi, Patrizia; Verrotti, Alberto; Marcotulli, Daniele; Canavese, C; Nicita, Francesco; Capuano, Alessandro; Marra, Chiara; Fetta, Anna; Nosadini, Margherita; Sartori, Stefano; Papa, Amanda; Viri, Maurizio; Greco, Filippo; Pavone, Piero; Simonini, Gabriele; Matricardi, Sara; Siquilini, Sabrina; Marchese, Francesca; Grandis, Elisa De; Brunenghi, Bernadette Marrè; Malattia, Clara; Bassanese, Francesco; Bergonzini, Patrizia; Bonuccelli, Alice; Consolini, Rita; Marseglia, Gian Luigi; Peroni, Diego; Striano, Pasquale; Cordelli, Duccio Maria; Savasta, Salvatore

doi:10.1016/j.ejpn.2021.11.002

Cited by 13 publications

(36 citation statements)

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“…The predominance of female gender observed in our population was in accordance with the literature [ 32 ], while the mean age at the time of SC presentation (10.36 ± 2.47 years) was slightly higher than the expected, since the peak incidence is generally considered 8 to 9 years [ 32 ] and a previous study on a large Italian cohort observed a mean age of 9.35 years [ 6 ]. Regarding occupational status and level of education, most of the patients in our overall population were still studying (68.1%) or working (27.7%), and only 2 patients (4.3%) were unemployed, with no significant differences between SC and nSC groups.…”

Section: Discussionsupporting

confidence: 91%

“…The literature reports neurological manifestations in approximately two-thirds of patients with Sydenham’s chorea, the most common ones being dysgraphia and dysarthria [ 6 ]. Our study confirms this finding, since in the SC group neurologic symptoms were observed in 61.9% of patients.…”

Section: Discussionmentioning

confidence: 99%

“…In addition to them, SC also features several other neurologic symptoms, as hypotonia, migraine, dysarthria, dysgraphia, dysphagia, and lingual dystonia. Despite mainly being known for its neurological manifestations, SC is also characterized by many neuropsychiatric symptoms (as also happens for many other SNC pathologies [ 4 , 5 ]), the most common ones being emotional lability, anxiety, attention deficit hyperactivity disorder, mood disorders, and obsessive-compulsive symptoms [ 6 , 7 , 8 ].…”

Section: Introductionmentioning

confidence: 99%

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Psychopathological Impact in Patients with History of Rheumatic Fever with or without Sydenham’s Chorea: A Multicenter Prospective Study

Orsini

Foiadelli

Sica

et al. 2022

IJERPH

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Sydenham’s chorea (SC) is a post-streptococcal autoimmune disorder of the central nervous system, and it is a major criterium for the diagnosis of acute rheumatic fever (ARF). SC typically improves in 12–15 weeks, but patients can be affected for years by persistence and recurrencies of both neurological and neuropsychiatric symptoms. We enrolled 48 patients with a previous diagnosis of ARF, with or without SC, in a national multicenter prospective study, to evaluate the presence of neuropsychiatric symptoms several years after SC’s onset. Our population was divided in a SC group (n = 21), consisting of patients who had SC, and a nSC group (n = 27), consisting of patients who had ARF without SC. Both groups were evaluated by the administration of 8 different neuropsychiatric tests. The Work and Social Adjustment Scale (WSAS) showed significantly (p = 0.021) higher alterations in the SC group than in the nSC group. Furthermore, 60.4% (n = 29) of the overall population experienced neuropsychiatric symptoms other than choreic movements at diagnosis and this finding was significantly more common (p = 0.00) in SC patients (95.2%) than in nSC patients (33.3%). The other neuropsychiatric tests also produced significant results, indicating that SC can exert a strong psychopathological impact on patients even years after its onset.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Psychopathological Impact in Patients with History of Rheumatic Fever with or without Sydenham’s Chorea: A Multicenter Prospective Study

Orsini

Foiadelli

Sica

et al. 2022

IJERPH

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“…6 Also, the patients with SC frequently have clinical or subclinical carditis. Orsini et al reported carditis in 81% (subclinical in 65%), 9 and Gürses et al in 93% of of children with SC. 6 In our case had active carditis, SC and migratory polyarthritis.…”

Section: Discussionmentioning

confidence: 97%

“…They declared 66% had generalized chorea, 34% had hemichorea, and 51% had neuropsychiatric findings. 9 In the differential diagnosis of SC included that drug intoxication, tic disorder, choreoathetoid cerebral palsy, encephalitis, intracranial tumor, hyperthyroidism, antiphospholipid antibody syndrome, autoimmune (systemic lupus erythematosus-SLE, systemic vasculitis) and metabolic diseases (e.g. Huntington disease, Wilson's disease).…”

Section: Discussionmentioning

confidence: 99%

Resistant Chorea Successfully Treated with Intravenous Immunoglobulin: A Case Report

et al. 2021

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Sydenham’s chorea (SC) is common cause of acquired chorea in childhood. SC occurs mainly in children with untreated streptococcal infections. An effective list of therapeutic options has been used to treat this disorder: antiepileptic drugs (valproic acid, carbamazepine etc.), haloperidol, chlorpromazine, amphetamines, steroids, plasma exchange and intravenous immunoglobulins (IVIG). We report a 12-year-old girl with carditis and severely generalized chorea and successfully treated with IVIG. This case report shows that IVIG is an effective treatment for the chorea cases resistant to anticonvulsants, dopamine antagonists and steroids, although larger studies are needed to confirm this conclusion.

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Treatments and Outcomes Among Patients with Sydenham Chorea

Eyre,

Thomas,

Ferrarin

et al. 2024

JAMA Netw Open

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ImportanceSydenham chorea is the most common acquired chorea of childhood worldwide; however, treatment is limited by a lack of high-quality evidence.ObjectivesTo evaluate historical changes in the clinical characteristics of Sydenham chorea and identify clinical and treatment factors at disease onset associated with chorea duration, relapsing disease course, and functional outcome.Data SourcesThe systematic search for this meta-analysis was conducted in PubMed, Embase, CINAHL, Cochrane Library, and LILACS databases and registers of clinical trials from inception to November 1, 2022 (search terms: [Sydenham OR Sydenham’s OR rheumatic OR minor] AND chorea).Study SelectionPublished articles that included patients with a final diagnosis of Sydenham chorea (in selected languages).Data Extraction and SynthesisThis study followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline. Individual patient data on clinical characteristics, treatments, chorea duration, relapse, and final outcome were extracted. Data from patients in the modern era (1945 through 2022) were entered into multivariable models and stratified by corticosteroid duration for survival analysis of chorea duration.Main Outcomes and MeasuresThe planned study outcomes were chorea duration at onset, monophasic course (absence of relapse after ≥24 months), and functional outcome (poor: modified Rankin Scale score 2-6 or persisting chorea, psychiatric, or behavioral symptoms at final follow-up after ≥6 months; good: modified Rankin Scale score 0-1 and no chorea, psychiatric, or behavioral symptoms at final follow-up).ResultsIn total, 1479 patients were included (from 307 articles), 1325 since 1945 (median [IQR] age at onset, 10 [8-13] years; 875 of 1272 female [68.8%]). Immunotherapy was associated with shorter chorea duration (hazard ratio for chorea resolution, 1.51 [95% CI, 1.05-2.19]; P = .03). The median chorea duration in patients receiving 1 or more months of corticosteroids was 1.2 months (95% CI, 1.2-2.0) vs 2.8 months (95% CI, 2.0-3.0) for patients receiving none (P = .004). Treatment factors associated with monophasic disease course were antibiotics (odds ratio [OR] for relapse, 0.28 [95% CI, 0.09-0.85]; P = .02), corticosteroids (OR, 0.32 [95% CI, 0.15-0.67]; P = .003), and sodium valproate (OR, 0.33 [95% CI, 0.15-0.71]; P = .004). Patients receiving at least 1 month of corticosteroids had significantly lower odds of relapsing course (OR, 0.10 [95% CI, 0.04-0.25]; P &lt; .001). No treatment factor was associated with good functional outcome.Conclusions and RelevanceIn this meta-analysis of treatments and outcomes in patients with Sydenham chorea, immunotherapy, in particular corticosteroid treatment, was associated with faster resolution of chorea. Antibiotics, corticosteroids and sodium valproate were associated with a monophasic disease course. This synthesis of retrospective data should support the development of evidence-based treatment guidelines for patients with Sydenham chorea.

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A nationwide study on Sydenham's chorea: Clinical features, treatment and prognostic factors

Cited by 13 publications

References 35 publications

Psychopathological Impact in Patients with History of Rheumatic Fever with or without Sydenham’s Chorea: A Multicenter Prospective Study

Psychopathological Impact in Patients with History of Rheumatic Fever with or without Sydenham’s Chorea: A Multicenter Prospective Study

Resistant Chorea Successfully Treated with Intravenous Immunoglobulin: A Case Report

Treatments and Outcomes Among Patients with Sydenham Chorea

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