Raras
Buscar doenças, sintomas, genes...
Coreia de Sydenham
ORPHA:306731CID-10 · I02.0CID-11 · 1B42DOENÇA RARA

Uma doença neurológica caracterizada por movimentos rápidos, bruscos, irregulares e involuntários, um quadro conhecido como coreia, especialmente no rosto e nos membros (braços e pernas). Outros sintomas podem incluir fraqueza nos músculos, fala arrastada, dores de cabeça e convulsões.

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Introdução

O que você precisa saber de cara

📋

Uma doença neurológica caracterizada por movimentos rápidos, bruscos, irregulares e involuntários, um quadro conhecido como coreia, especialmente no rosto e nos membros (braços e pernas). Outros sintomas podem incluir fraqueza nos músculos, fala arrastada, dores de cabeça e convulsões.

Pesquisas ativas
1 ensaio
4 total registrados no ClinicalTrials.gov
Publicações científicas
169 artigos
Último publicado: 2026 Apr 16

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adolescent
+ adult, childhood
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: I02.0
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧠
Neurológico
5 sintomas
🫁
Pulmão
1 sintomas
🫘
Rins
1 sintomas
❤️
Coração
1 sintomas

+ 9 sintomas em outras categorias

Características mais comuns

55%prev.
Anormalidade de movimento da língua
Frequente (79-30%)
55%prev.
Disartria
Frequente (79-30%)
55%prev.
Cefaleia
Frequente (79-30%)
55%prev.
Labilidade emocional
Frequente (79-30%)
55%prev.
Marcha instável
Frequente (79-30%)
55%prev.
Irritabilidade
Frequente (79-30%)
17sintomas
Frequente (13)
Ocasional (4)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 17 características clínicas mais associadas, ordenadas por frequência.

Anormalidade de movimento da línguaMovement abnormality of the tongue
Frequente (79-30%)55%
DisartriaDysarthria
Frequente (79-30%)55%
CefaleiaHeadache
Frequente (79-30%)55%
Labilidade emocionalEmotional lability
Frequente (79-30%)55%
Marcha instávelUnsteady gait
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico169PubMed
Últimos 10 anos75publicações
Pico202312 papers
Linha do tempo
2026Hoje · 2026🧪 1990Primeiro ensaio clínico📈 2023Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 31
·Pré-clínico2
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 3 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Coreia de Sydenham

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

4 ensaios clínicos encontrados, 1 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
82 papers (10 anos)
#1

Secondary tics: Etiology, presentation, and management.

Handbook of clinical neurology2026

Tics are involuntary or semivoluntary, abrupt, brief, nonrhythmic, recurrent movements or sounds. Tourette syndrome (TS) is the most common cause of tics; however, several other disorders have been associated with tics and tic-like movements. Etiologies of secondary tic syndromes and disorders (STS) include hereditary, drug-induced (including tardive), toxins, traumatic, cerebrovascular, infectious, parainfectious, autoimmune, functional disorders, and others. Age at onset after 18 years, lack of comorbid attention-deficit/hyperactivity disorder, obsessive-compulsive disorder, and no family history of tics are considered typical features of STS. Atypical neurologic manifestations such as additional movement disorders, seizures, decreased level of consciousness, and neurologic deficits, temporally related to some triggering event, such as brain trauma, stroke, or drug exposure, should raise suspicion of STS. These patients usually show a more restricted body distribution, less severe and less complex tics compared with patients with TS. Some tics, typically observed in TS, such as eye-blinking, facial grimacing, and complex motor and phonic tics that are preceded by a premonitory urge may not be present in STS. However, there is a substantial overlap of features between patients with TS and STS. Pharmacologic treatment of STS is similar to TS. Additionally, these patients may require specific treatment, such as anticonvulsants, antibiotics, immunotherapy, or drug-discontinuation. Therefore, prompt recognition and early treatment intervention of STS is imperative for a favorable outcome.

#2

An Internal Focus of Attention Benefits Motor Function in Children with Sydenham Chorea Syndrome: A Case Study.

Physical &amp; occupational therapy in pediatrics2026

The aim of the present case series was to describe an instructional (attention) intervention for improving motor function in two children rehabilitating from Sydenam's chorea syndrome. Evidence suggests that paying attention to the body can be advantageous for afferent proprioceptive processing during motor skills wherein this information is relevant for successful task execution. This unique approach may also hold true for individuals with movement disorders, such as Sydenham chorea syndrome: a rare neurological condition that results in uncoordinated movements. The present study provides a case study of two children unable to walk independently due to low postural stability. Both children completed "10-Meter Walk" and "Timed Up and Go" tests as a baseline, before undergoing three sessions per week of an intervention, for three months. Both children practiced walking and dynamic balance, with one child instructed to adopt an internal (bodily) focus and the other an external focus. A post-test experimental phase was then conducted to measure learning. Results revealed superior motor function when adopting a focus on body motion. When movement disorders create ambiguity in motor efference, an internal focus on task-relevant proprioceptive information may facilitate more appropriate movement organization. Sydenham chorea syndrome is a rare neurological movement disorder, resulting in involuntary and uncoordinated movements. The present case study found that children with this condition have improved walking performance when able to attend to their body movements (i.e. an internal focus of attention). This has implications for future clinical practice.

#3

Sydenham Chorea With Elevated Interleukin-12 Levels Responsive to Plasmapheresis and Immunotherapy.

Cureus2026 Jan

Sydenham chorea (SC) presents with random abnormal involuntary movements that occur after an autoimmune reaction to a prior group A beta-hemolytic streptococcal infection. While most cases resolve spontaneously, some cases have a prolonged duration of symptoms and recurrences. We discuss a 22-year-old woman who presented with a two-month history of involuntary, brief, random, and irregular movements of the limbs. She had a history of multiple streptococcal throat infections. At age two, she had scarlet fever. After ruling out other causes of chorea, she was diagnosed with Sydenham chorea. She was treated with intravenous immunoglobulin, oral prednisone, and amantadine, resulting in full symptom resolution. Interleukin-12 (IL-12) was elevated approximately seven months after hospital discharge. Chronic elevation of IL-12 differs from previously published findings, which describe elevation only during the acute phase of the disease. Intensive immunosuppressive treatment during the acute phase, along with adherence to antibiotic therapy, may have contributed to the full resolution of her chorea.

#4

Evaluation, Diagnosis, and Treatment of Sydenham Chorea: Consensus Guidelines.

Pediatrics2025 Dec 01

An international panel of 27 experts (pediatric and movement disorder neurologists, psychiatrists, and parent representatives) from all continents participated in a Delphi process to establish international consensus guidelines for the evaluation, diagnosis, and management of children with Sydenham chorea (SC) based on best evidence and expert opinion. In total, 88 recommendations reached consensus. Practitioners should identify key signs of SC (chorea and hypotonia), screen for behavioral, mobility, swallowing, speech, and cognitive impairments, and acute rheumatic fever (ARF) features including carditis. Etiological evaluation will differ according to population ARF risk. At all times, patients, families, and educators should receive support, information, and guidance to minimize the impact of SC on academic and social functioning. Antibiotic treatment is recommended at first presentation. Long-term secondary antibiotic prophylaxis should follow international or local guidelines, and measures to reduce pain and distress associated with intramuscular antibiotics will aid in adherence. Immunotherapy (corticosteroids) is recommended in moderate to severe SC. In those with inadequate recovery, intravenous immunoglobulin or plasma exchange should be given. In SC relapse, repeat clinical assessments, etiological investigation, and antibiotics plus corticosteroid therapy should be considered. This consensus guideline will standardize the evaluation and management of patients with SC and direct future research to improve the lived experience and outcomes of patients and families.

#5

A Refractory Case of Sydenham Chorea Managed With Intravenous Pulse-Dose Methylprednisolone.

Case reports in pediatrics2025

We describe the case of an eight-year-old female presenting with abrupt-onset involuntary movements, emotional lability, and gait disturbances, consistent with Sydenham's chorea (SC). Her condition deteriorated despite initial antibiotic treatment and symptomatic management. Notable findings included elevated antistreptolysin O titers and antideoxyribonuclease B antibodies, suggestive of recent Group A Streptococcus infection. Brain magnetic resonance imaging revealed punctate gliosis in the supratentorial white matter, and extensive workup excluded alternative diagnoses. Despite escalating therapy with valproic acid, clonidine, and haloperidol, the patient exhibited persistent choreiform movements and emotional dysregulation. High-dose corticosteroids (methylprednisolone) were initiated, resulting in significant symptomatic improvement and restoration of ambulatory function. Long-term prophylaxis with benzathine penicillin G was implemented to prevent recurrence. SC remains an under-researched complication of acute rheumatic fever, with treatment often extrapolated from limited case reports and expert consensus. This case underscores the potential role of corticosteroids in refractory SC. This case also highlights the complexity of managing prolonged SC and the importance of individualized, multifaceted treatment strategies.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC84 artigos no totalmostrando 72

2026

Secondary tics: Etiology, presentation, and management.

Handbook of clinical neurology
2025

Beyond the dance: A muted narrative of Sydenham chorea.

Indian journal of psychiatry
2026

Sydenham Chorea With Elevated Interleukin-12 Levels Responsive to Plasmapheresis and Immunotherapy.

Cureus
2025

Evaluation, Diagnosis, and Treatment of Sydenham Chorea: Consensus Guidelines.

Pediatrics
2026

An Internal Focus of Attention Benefits Motor Function in Children with Sydenham Chorea Syndrome: A Case Study.

Physical &amp; occupational therapy in pediatrics
2025

A Neurological Curtain Unmasking Rheumatic Carditis in Early Adolescents: Two Illustrative Cases From a Tertiary Care Center in Maharashtra, India.

Cureus
2025

A Refractory Case of Sydenham Chorea Managed With Intravenous Pulse-Dose Methylprednisolone.

Case reports in pediatrics
2025

Single-Cell RNA Seq in Sydenham Chorea Shows B Cell HLA-DR/DQ Upregulation and Plasma Cell Proteasomal Activation.

Annals of clinical and translational neurology
2025

Disruption of the blood-brain barrier contributes to neurobehavioral changes observed in rheumatic heart disease.

Animal models and experimental medicine
2025

Progressive Choreiform Movements in a Child: Early Recognition and Management of Sydenham Chorea.

Tremor and other hyperkinetic movements (New York, N.Y.)
2025

Children With Sydenham Chorea and Psychiatric Disorders Had Variable Long-Term Outcomes and Required Multidisciplinary Management.

Acta paediatrica (Oslo, Norway : 1992)
2025

Ultrarare Cause of Childhood Chorea: Celiac Disease.

Journal of child neurology
2024

Open Heart Dual Valve Surgery Without Blood Transfusion: A Case Report.

Cureus
2024

An 8-year single-centre experience of patients with subclinical rheumatic carditis.

Cardiology in the young
2024

Dopamine receptor autoantibody signaling in infectious sequelae differentiates movement versus neuropsychiatric disorders.

JCI insight
2024

Treatments and Outcomes Among Patients with Sydenham Chorea: A Meta-Analysis.

JAMA network open
2023

Narcolepsy type 1 and Sydenham chorea - Report of 3 cases and review of the literature.

Sleep medicine
2023

Group A Streptococcus Vaccine Targeting the Erythrogenic Toxins SpeA and SpeB Is Safe and Immunogenic in Rabbits and Does Not Induce Antibodies Associated with Autoimmunity.

Vaccines
2023

Postinfectious Inflammation, Autoimmunity, and Obsessive-Compulsive Disorder: Sydenham Chorea, Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infection, and Pediatric Acute-Onset Neuropsychiatric Disorder.

Developmental neuroscience
2023

Sydenham chorea in the top end of Australia's Northern Territory: A 20-year retrospective case series.

Journal of paediatrics and child health
2023

Treatable Sydenham Chorea in a 76-Year-Old Woman.

Neurology
2023

Sydenham Chorea in Sudan; Presentation Panorama.

Neuropsychiatric disease and treatment
2023

Chorea following SARS-CoV-2 infection and vaccination: a systematic review of reported cases.

International journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases
2023

Arthritis in Children with Psychiatric Deteriorations: A Case Series.

Developmental neuroscience
2023

Rare Recurrence of Sydenham Chorea in an Adult: A Case Report.

International medical case reports journal
2023

Managing and treating Sydenham chorea: A systematic review.

Brain and behavior
2023

Corticosteroid Treatment in Sydenham Chorea: A 27-Year Tertiary Referral Center Experience.

Children (Basel, Switzerland)
2022

IgG2 rules: N-acetyl-β-D-glucosamine-specific IgG2 and Th17/Th1 cooperation may promote the pathogenesis of acute rheumatic heart disease and be a biomarker of the autoimmune sequelae of Streptococcus pyogenes.

Frontiers in cardiovascular medicine
2023

Neuroinflammation in Obsessive-Compulsive Disorder: Sydenham Chorea, Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections, and Pediatric Acute Onset Neuropsychiatric Syndrome.

The Psychiatric clinics of North America
2022

Childhood Neurologic Conditions: Movement Disorders.

FP essentials
2022

Characterization of an experimental model to determine streptococcal M protein-induced autoimmune cardiac and neurobehavioral abnormalities.

Immunology and cell biology
2021

Oxcarbazepine May Be Useful in Sydenham Chorea.

Turkish archives of pediatrics
2021

Acute Movement Disorders in Childhood: A Cohort Study and Review of the Literature.

Pediatric emergency care
2022

An unexpected cause of chorea in an adolescent girl: systemic lupus erythematosus.

Zeitschrift fur Rheumatologie
2021

Group A streptococcal antigen exposed rat model to investigate neurobehavioral and cardiac complications associated with post-streptococcal autoimmune sequelae.

Animal models and experimental medicine
2021

Sydenham Chorea Managed With Immunoglobulin in Acute Rheumatic Fever.

Cureus
2021

Requirements for a Robust Animal Model to Investigate the Disease Mechanism of Autoimmune Complications Associated With ARF/RHD.

Frontiers in cardiovascular medicine
2021

Chorea as the presenting feature of acute rheumatic fever in childhood; case reports from a low-prevalence European setting.

BMC infectious diseases
2021

Are Antibody Panels Under-Utilized in Movement Disorders Diagnosis? No.

Movement disorders clinical practice
2021

Acute chorea: case series from the emergency room of a Brazilian tertiary-level center.

Arquivos de neuro-psiquiatria
2021

Neurologic complications of rheumatic fever.

Handbook of clinical neurology
2021

A sydenham chorea attack associated with COVID-19 infection.

Brain, behavior, &amp; immunity - health
2020

Treatment of Disorders of Tone and Other Considerations in Pediatric Movement Disorders.

Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics
2021

Sydenham Chorea: Putaminal Enlargement.

Journal of child neurology
2020

Chorea in children: etiology, diagnostic approach and management.

Journal of neural transmission (Vienna, Austria : 1996)
2020

Autoantibody Biomarkers for Basal Ganglia Encephalitis in Sydenham Chorea and Pediatric Autoimmune Neuropsychiatric Disorder Associated With Streptococcal Infections.

Frontiers in psychiatry
2020

Sydenham chorea in a12- year old Saudi girl.

eNeurologicalSci
2020

Efficacy of levetiracetam in the treatment of Sydenham chorea.

Pediatrics international : official journal of the Japan Pediatric Society
2020

Levodopa-Responsive Chorea: A Review.

Annals of Indian Academy of Neurology
2020

Simultaneous Occurrence of Sydenham Chorea with Erythema Marginatum: A Case of Rheumatic Fever.

Pediatric health, medicine and therapeutics
2020

Preliminary data on prednisone effectiveness in children with Sydenham chorea.

European journal of pediatrics
2019

Chorea gravidarum: Case report and review of the literature.

Revista colombiana de obstetricia y ginecologia
2020

Serum periostin levels in acute rheumatic fever: is it useful as a new biomarker?

Paediatrics and international child health
2019

Rheumatic Chorea as the First Presenting Sign in a 13-year-old Female Child.

Cureus
2019

Focus on Cardiologic Findings in 30 Children With PANS/PANDAS: An Italian Single-Center Observational Study.

Frontiers in pediatrics
2019

Molecular Mimicry, Autoimmunity, and Infection: The Cross-Reactive Antigens of Group A Streptococci and their Sequelae.

Microbiology spectrum
2018

Rare case of chorea-hyperglycaemia-basal ganglia (C-H-BG) syndrome.

BMJ case reports
2018

[A boy with involuntary movements].

Nederlands tijdschrift voor geneeskunde
2018

Principles and approaches to the treatment of immune-mediated movement disorders.

European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
2018

Longitudinal outcomes of children with pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS).

European child &amp; adolescent psychiatry
2017

A retrospective study: Acute rheumatic fever and post-streptococcal reactive arthritis in Japan.

Allergology international : official journal of the Japanese Society of Allergology
2017

Clinical Value of Perfusion Abnormalities of Brain on Technetium-99m HMPAO Single-Photon Emission Computed Tomography in Children With Sydenham Chorea.

Journal of child neurology
2017

Systematic review of immunoglobulin use in paediatric neurological and neurodevelopmental disorders.

Developmental medicine and child neurology
2017

Acute rheumatic fever outbreak in southern central European country.

European journal of pediatrics
2015

Language Impairment in Adolescents with Sydenham Chorea.

Pediatric neurology briefs
2016

Autoimmunity against dopamine receptors in neuropsychiatric and movement disorders: a review of Sydenham chorea and beyond.

Acta physiologica (Oxford, England)
2015

The usefulness of immunotherapy in pediatric neurodegenerative disorders: A systematic review of literature data.

Human vaccines &amp; immunotherapeutics
2015

A New Alternative Drug With Fewer Adverse Effects in the Treatment of Sydenham Chorea: Levetiracetam Efficacy in a Child.

Clinical neuropharmacology
2015

Autoantibodies in movement and psychiatric disorders: updated concepts in detection methods, pathogenicity, and CNS entry.

Annals of the New York Academy of Sciences
2015

Antineuronal antibodies in a heterogeneous group of youth and young adults with tics and obsessive-compulsive disorder.

Journal of child and adolescent psychopharmacology
2015

Therapeutic plasma apheresis as a treatment for 35 severely ill children and adolescents with pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections.

Journal of child and adolescent psychopharmacology
2015

Sydenham chorea in a 5-year-old Saudi patient.

Neurosciences (Riyadh, Saudi Arabia)
Ver todos os 84 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Coreia de Sydenham

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Secondary tics: Etiology, presentation, and management.
    Handbook of clinical neurology· 2026· PMID 41633741mais citado
  2. An Internal Focus of Attention Benefits Motor Function in Children with Sydenham Chorea Syndrome: A Case Study.
    Physical &amp; occupational therapy in pediatrics· 2026· PMID 41185949mais citado
  3. Sydenham Chorea With Elevated Interleukin-12 Levels Responsive to Plasmapheresis and Immunotherapy.
    Cureus· 2026· PMID 41497421mais citado
  4. Evaluation, Diagnosis, and Treatment of Sydenham Chorea: Consensus Guidelines.
    Pediatrics· 2025· PMID 41265690mais citado
  5. A Refractory Case of Sydenham Chorea Managed With Intravenous Pulse-Dose Methylprednisolone.
    Case reports in pediatrics· 2025· PMID 41178977mais citado
  6. Revisiting an immunoneuropsychiatry classic: Sydenham's chorea.
    Expert Rev Clin Immunol· 2026· PMID 41989060recente
  7. Beyond the dance: A muted narrative of Sydenham chorea.
    Indian J Psychiatry· 2025· PMID 41503084recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:306731(Orphanet)
  2. MONDO:0017648(MONDO)
  3. GARD:7716(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. Q522163(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Coreia de Sydenham
Compêndio · Raras BR

Coreia de Sydenham

ORPHA:306731 · MONDO:0017648
Prevalência
Unknown
CID-10
I02.0 · Coréia reumática com comprometimento cardíaco
CID-11
Ensaios
1 ativos
Início
Adolescent, Adult, Childhood
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0152113
EuropePMC
Wikidata
Wikipedia
Papers 10a
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