Sydenham's chorea: not gone but perhaps forgotten

Crealey, Miranda; Allen, Nicholas M.; Webb, David; Bouldin, Anthony; Sweeney, Niamh; Peake, Deirdre; Tirupathi, Sandya; Butler, Karina; King, Mary D.

doi:10.1136/archdischild-2015-308693

Cited by 16 publications

(10 citation statements)

References 3 publications

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“…Approximately 30% of patients with either OCD or PANDAS present choreiform movements that are similar to those in Sydenham's chorea, which eventually might raise diagnostic issues (Table 1) [22]. Etiologically in both PANDAS and Sydenham's chorea present as a side effect of GAS infection, nevertheless, besides the presence of tics, which is common for both diseases, Sydenham's chorea appears with more severe obsessive-compulsive syndromes and definite chorea with the presence of hypotonia [93,94]. Moreover, Sydenham's chorea usually presents with complete remissions and a duration less than one year, whereas the PANDAS course is likely to be more chronic [95].…”

Section: Coexisting Neurological Impairmentsmentioning

confidence: 99%

Alterations in the Nervous System and Gut Microbiota after β-Hemolytic Streptococcus Group A Infection—Characteristics and Diagnostic Criteria of PANDAS Recognition

Baj

Sitarz

Forma

et al. 2020

IJMS

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The objective of this paper is to review and summarize conclusions from the available literature regarding Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS). The authors have independently reviewed articles from 1977 onwards, primarily focusing on the etiopathology, symptoms, differentiation between similar psychiatric conditions, immunological reactions, alterations in the nervous system and gut microbiota, genetics, and the available treatment for PANDAS. Recent research indicates that PANDAS patients show noticeable alterations within the structures of the central nervous system, including caudate, putamen, globus pallidus, and striatum, as well as bilateral and lentiform nuclei. Likewise, the presence of autoantibodies that interact with basal ganglia was observed in PANDAS patients. Several studies also suggest a relationship between the presence of obsessive-compulsive disorders like PANDAS and alterations to the gut microbiota. Further, genetic predispositions—including variations in the MBL gene and TNF-α—seem to be relevant regarding PANDAS syndrome. Even though the literature is still scarce, the authors have attempted to provide a thorough insight into the PANDAS syndrome, bearing in mind the diagnostic difficulties of this condition.

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Section: Coexisting Neurological Impairmentsmentioning

confidence: 99%

Alterations in the Nervous System and Gut Microbiota after β-Hemolytic Streptococcus Group A Infection—Characteristics and Diagnostic Criteria of PANDAS Recognition

Baj

Sitarz

Forma

et al. 2020

IJMS

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“…We identified 1479 patients with IPD (median [IQR] age at onset, 10 [8-13] years in 1354 patients; 985 of 1426 [69.1%] female and 441 of 1426 [30.9%] male) were identified from 307 articles…”

Section: Resultsmentioning

confidence: 99%

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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“…As there is no permanent structural disability, the natural course of the disease is that it continues with a waxing and waning quality for 2-6 months until it finally resolves (8,16,26) . Therefore, most of the time, mild Sydenham chorea may not necessitate symptomatic treatment, however, we treated all our patients like some physicians, even mild ones (15,27) . If the chorea is moderate to severe, and gait is affected, treatment may be more warranted.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of Patients with an Initial Diagnosis of Chorea: Sydenham Chorea and Differential Diagnoses

Uzunhan¹,

İrdem²

2020

BMJ

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Objective: Our aim is to evaluate patients being referred with an initial diagnosis of chorea according to their clinical, laboratory features and final diagnoses while emphasizing cardiological findings of patients with Sydenham chorea. Method: Children aged 4-18 years who were referred to Okmeydanı Research and Training Hospital Pediatric Neurology department with an initial diagnosis of acute, subacute chorea between January 2017-January 2020 were retrospectively included. Chronic chorea and diseases associated with chronic chorea were excluded from the study. Data concerning clinical, laboratory features, cardiological findings, etiologies, treatments, recurrence rates and follow-ups of patients were recorded. Descriptive statistical analysis were performed using SPSS 21.0. Results: Fifteen patients has been referred with the initial diagnosis of chorea. Mean age of the patients was 11.5±2.2 years. Ten (67%) patients were females, 5 (33%) patients were male. After admission, 8 (54%) patients were diagnosed with Sydenham chorea, and 2 (13%) patients with recurrent Sydenham chorea. During physical examination, 5 (33%) patients did not have chorea, and 3 cases had tic disorder. Out of 8 patients with new diagnosis of Sydenham chorea, 3 (37.5%) patients had subclinical carditis, and 5 (62.5%) patients clinical carditis. Chorea had been treated with one of haloperidol/biperiden, valproic acid and prednisolon options. The treatment of 6 patients attending regular follow-up visits was stopped 2-6 months later. Chorea of two patients recurred during our follow-up, and one of our newly diagnosed Sydenham chorea patients had been recognized as antiphospholipid antibody syndrome after recurrence. Conclusion: Sydenham chorea is the most common cause of acquired chorea in childhood. Most of the time it is self limiting. Differential diagnosis of chorea must be kept in mind especially when there is a recurrence.

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Sydenham's chorea: not gone but perhaps forgotten

Cited by 16 publications

References 3 publications

Alterations in the Nervous System and Gut Microbiota after β-Hemolytic Streptococcus Group A Infection—Characteristics and Diagnostic Criteria of PANDAS Recognition

Alterations in the Nervous System and Gut Microbiota after β-Hemolytic Streptococcus Group A Infection—Characteristics and Diagnostic Criteria of PANDAS Recognition

Treatments and Outcomes Among Patients with Sydenham Chorea

Evaluation of Patients with an Initial Diagnosis of Chorea: Sydenham Chorea and Differential Diagnoses

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