Antonio L Teixeira, Debora P Maia, Maria Carmo Pereira Nunes, Gabriel A de Erausquin, Cristian P Zeni, Aline S Miranda
{"title":"Revisiting an immunoneuropsychiatry classic: Sydenham's chorea.","authors":"Antonio L Teixeira, Debora P Maia, Maria Carmo Pereira Nunes, Gabriel A de Erausquin, Cristian P Zeni, Aline S Miranda","doi":"10.1080/1744666X.2026.2660763","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Sydenham chorea (SC) is the major neurologic manifestation of rheumatic fever. It is defined by characteristic involuntary movements and frequently associated with neuropsychiatric symptoms that may develop weeks or months following a Group A beta hemolytic streptococcal (GAS) infection.</p><p><strong>Areas covered: </strong>The authors searched PubMed database for recent original studies and systematic reviews on SC pathogenesis, clinical spectrum and therapeutic approaches.</p><p><strong>Expert opinion: </strong>Compelling evidence supports that autoantibodies targeting neuronal antigens underly the pathogenesis of SC. In the basal ganglia, autoantibodies react, among other epitopes, with dopamine type 2 (D2) receptors. The resulting dopaminergic signaling dysfunction leads to SC-related motor and neuropsychiatric symptoms. A key gap in the antibody-mediated hypothesis of SC lays on the limited understanding on how peripherally generated autoantibodies reach the basal ganglia. D2 antagonists and valproic acid are the main symptomatic therapeutic strategies for SC. The use of corticosteroids can accelerate the resolution of motor symptoms, and must be considered in severe cases, including <i>chorea paralytica</i>. Plasmapheresis and immunoglobulin have been considered investigational for SC.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":" ","pages":"1-11"},"PeriodicalIF":3.7000,"publicationDate":"2026-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Expert Review of Clinical Immunology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1080/1744666X.2026.2660763","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"IMMUNOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Introduction: Sydenham chorea (SC) is the major neurologic manifestation of rheumatic fever. It is defined by characteristic involuntary movements and frequently associated with neuropsychiatric symptoms that may develop weeks or months following a Group A beta hemolytic streptococcal (GAS) infection.
Areas covered: The authors searched PubMed database for recent original studies and systematic reviews on SC pathogenesis, clinical spectrum and therapeutic approaches.
Expert opinion: Compelling evidence supports that autoantibodies targeting neuronal antigens underly the pathogenesis of SC. In the basal ganglia, autoantibodies react, among other epitopes, with dopamine type 2 (D2) receptors. The resulting dopaminergic signaling dysfunction leads to SC-related motor and neuropsychiatric symptoms. A key gap in the antibody-mediated hypothesis of SC lays on the limited understanding on how peripherally generated autoantibodies reach the basal ganglia. D2 antagonists and valproic acid are the main symptomatic therapeutic strategies for SC. The use of corticosteroids can accelerate the resolution of motor symptoms, and must be considered in severe cases, including chorea paralytica. Plasmapheresis and immunoglobulin have been considered investigational for SC.
期刊介绍:
Expert Review of Clinical Immunology (ISSN 1744-666X) provides expert analysis and commentary regarding the performance of new therapeutic and diagnostic modalities in clinical immunology. Members of the International Editorial Advisory Panel of Expert Review of Clinical Immunology are the forefront of their area of expertise. This panel works with our dedicated editorial team to identify the most important and topical review themes and the corresponding expert(s) most appropriate to provide commentary and analysis. All articles are subject to rigorous peer-review, and the finished reviews provide an essential contribution to decision-making in clinical immunology.
Articles focus on the following key areas:
• Therapeutic overviews of specific immunologic disorders highlighting optimal therapy and prospects for new medicines
• Performance and benefits of newly approved therapeutic agents
• New diagnostic approaches
• Screening and patient stratification
• Pharmacoeconomic studies
• New therapeutic indications for existing therapies
• Adverse effects, occurrence and reduction
• Prospects for medicines in late-stage trials approaching regulatory approval
• Novel treatment strategies
• Epidemiological studies
• Commentary and comparison of treatment guidelines
Topics include infection and immunity, inflammation, host defense mechanisms, congenital and acquired immunodeficiencies, anaphylaxis and allergy, systemic immune diseases, organ-specific inflammatory diseases, transplantation immunology, endocrinology and diabetes, cancer immunology, neuroimmunology and hematological diseases.