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.
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.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 9 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 17 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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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
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Coreia de Sydenham
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
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Outros ensaios clínicos
4 ensaios clínicos encontrados, 1 ativos.
Publicações mais relevantes
Secondary tics: Etiology, presentation, and management.
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.
An Internal Focus of Attention Benefits Motor Function in Children with Sydenham Chorea Syndrome: A Case Study.
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.
Sydenham Chorea With Elevated Interleukin-12 Levels Responsive to Plasmapheresis and Immunotherapy.
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.
Evaluation, Diagnosis, and Treatment of Sydenham Chorea: Consensus Guidelines.
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.
A Refractory Case of Sydenham Chorea Managed With Intravenous Pulse-Dose Methylprednisolone.
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
Revisiting an immunoneuropsychiatry classic: Sydenham's chorea.
Secondary tics: Etiology, presentation, and management.
Beyond the dance: A muted narrative of Sydenham chorea.
Sydenham Chorea With Elevated Interleukin-12 Levels Responsive to Plasmapheresis and Immunotherapy.
Evaluation, Diagnosis, and Treatment of Sydenham Chorea: Consensus Guidelines.
📚 EuropePMC84 artigos no totalmostrando 72
Secondary tics: Etiology, presentation, and management.
Handbook of clinical neurologyBeyond the dance: A muted narrative of Sydenham chorea.
Indian journal of psychiatrySydenham Chorea With Elevated Interleukin-12 Levels Responsive to Plasmapheresis and Immunotherapy.
CureusEvaluation, Diagnosis, and Treatment of Sydenham Chorea: Consensus Guidelines.
PediatricsAn Internal Focus of Attention Benefits Motor Function in Children with Sydenham Chorea Syndrome: A Case Study.
Physical & occupational therapy in pediatricsA Neurological Curtain Unmasking Rheumatic Carditis in Early Adolescents: Two Illustrative Cases From a Tertiary Care Center in Maharashtra, India.
CureusA Refractory Case of Sydenham Chorea Managed With Intravenous Pulse-Dose Methylprednisolone.
Case reports in pediatricsSingle-Cell RNA Seq in Sydenham Chorea Shows B Cell HLA-DR/DQ Upregulation and Plasma Cell Proteasomal Activation.
Annals of clinical and translational neurologyDisruption of the blood-brain barrier contributes to neurobehavioral changes observed in rheumatic heart disease.
Animal models and experimental medicineProgressive Choreiform Movements in a Child: Early Recognition and Management of Sydenham Chorea.
Tremor and other hyperkinetic movements (New York, N.Y.)Children With Sydenham Chorea and Psychiatric Disorders Had Variable Long-Term Outcomes and Required Multidisciplinary Management.
Acta paediatrica (Oslo, Norway : 1992)Ultrarare Cause of Childhood Chorea: Celiac Disease.
Journal of child neurologyOpen Heart Dual Valve Surgery Without Blood Transfusion: A Case Report.
CureusAn 8-year single-centre experience of patients with subclinical rheumatic carditis.
Cardiology in the youngDopamine receptor autoantibody signaling in infectious sequelae differentiates movement versus neuropsychiatric disorders.
JCI insightTreatments and Outcomes Among Patients with Sydenham Chorea: A Meta-Analysis.
JAMA network openNarcolepsy type 1 and Sydenham chorea - Report of 3 cases and review of the literature.
Sleep medicineGroup A Streptococcus Vaccine Targeting the Erythrogenic Toxins SpeA and SpeB Is Safe and Immunogenic in Rabbits and Does Not Induce Antibodies Associated with Autoimmunity.
VaccinesPostinfectious Inflammation, Autoimmunity, and Obsessive-Compulsive Disorder: Sydenham Chorea, Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infection, and Pediatric Acute-Onset Neuropsychiatric Disorder.
Developmental neuroscienceSydenham chorea in the top end of Australia's Northern Territory: A 20-year retrospective case series.
Journal of paediatrics and child healthTreatable Sydenham Chorea in a 76-Year-Old Woman.
NeurologySydenham Chorea in Sudan; Presentation Panorama.
Neuropsychiatric disease and treatmentChorea 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 DiseasesArthritis in Children with Psychiatric Deteriorations: A Case Series.
Developmental neuroscienceRare Recurrence of Sydenham Chorea in an Adult: A Case Report.
International medical case reports journalManaging and treating Sydenham chorea: A systematic review.
Brain and behaviorCorticosteroid Treatment in Sydenham Chorea: A 27-Year Tertiary Referral Center Experience.
Children (Basel, Switzerland)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 medicineNeuroinflammation 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 AmericaChildhood Neurologic Conditions: Movement Disorders.
FP essentialsCharacterization of an experimental model to determine streptococcal M protein-induced autoimmune cardiac and neurobehavioral abnormalities.
Immunology and cell biologyOxcarbazepine May Be Useful in Sydenham Chorea.
Turkish archives of pediatricsAcute Movement Disorders in Childhood: A Cohort Study and Review of the Literature.
Pediatric emergency careAn unexpected cause of chorea in an adolescent girl: systemic lupus erythematosus.
Zeitschrift fur RheumatologieGroup A streptococcal antigen exposed rat model to investigate neurobehavioral and cardiac complications associated with post-streptococcal autoimmune sequelae.
Animal models and experimental medicineSydenham Chorea Managed With Immunoglobulin in Acute Rheumatic Fever.
CureusRequirements for a Robust Animal Model to Investigate the Disease Mechanism of Autoimmune Complications Associated With ARF/RHD.
Frontiers in cardiovascular medicineChorea as the presenting feature of acute rheumatic fever in childhood; case reports from a low-prevalence European setting.
BMC infectious diseasesAre Antibody Panels Under-Utilized in Movement Disorders Diagnosis? No.
Movement disorders clinical practiceAcute chorea: case series from the emergency room of a Brazilian tertiary-level center.
Arquivos de neuro-psiquiatriaNeurologic complications of rheumatic fever.
Handbook of clinical neurologyA sydenham chorea attack associated with COVID-19 infection.
Brain, behavior, & immunity - healthTreatment of Disorders of Tone and Other Considerations in Pediatric Movement Disorders.
Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeuticsSydenham Chorea: Putaminal Enlargement.
Journal of child neurologyChorea in children: etiology, diagnostic approach and management.
Journal of neural transmission (Vienna, Austria : 1996)Autoantibody Biomarkers for Basal Ganglia Encephalitis in Sydenham Chorea and Pediatric Autoimmune Neuropsychiatric Disorder Associated With Streptococcal Infections.
Frontiers in psychiatrySydenham chorea in a12- year old Saudi girl.
eNeurologicalSciEfficacy of levetiracetam in the treatment of Sydenham chorea.
Pediatrics international : official journal of the Japan Pediatric SocietyLevodopa-Responsive Chorea: A Review.
Annals of Indian Academy of NeurologySimultaneous Occurrence of Sydenham Chorea with Erythema Marginatum: A Case of Rheumatic Fever.
Pediatric health, medicine and therapeuticsPreliminary data on prednisone effectiveness in children with Sydenham chorea.
European journal of pediatricsChorea gravidarum: Case report and review of the literature.
Revista colombiana de obstetricia y ginecologiaSerum periostin levels in acute rheumatic fever: is it useful as a new biomarker?
Paediatrics and international child healthRheumatic Chorea as the First Presenting Sign in a 13-year-old Female Child.
CureusFocus on Cardiologic Findings in 30 Children With PANS/PANDAS: An Italian Single-Center Observational Study.
Frontiers in pediatricsMolecular Mimicry, Autoimmunity, and Infection: The Cross-Reactive Antigens of Group A Streptococci and their Sequelae.
Microbiology spectrumRare case of chorea-hyperglycaemia-basal ganglia (C-H-BG) syndrome.
BMJ case reports[A boy with involuntary movements].
Nederlands tijdschrift voor geneeskundePrinciples and approaches to the treatment of immune-mediated movement disorders.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology SocietyLongitudinal outcomes of children with pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS).
European child & adolescent psychiatryA retrospective study: Acute rheumatic fever and post-streptococcal reactive arthritis in Japan.
Allergology international : official journal of the Japanese Society of AllergologyClinical Value of Perfusion Abnormalities of Brain on Technetium-99m HMPAO Single-Photon Emission Computed Tomography in Children With Sydenham Chorea.
Journal of child neurologySystematic review of immunoglobulin use in paediatric neurological and neurodevelopmental disorders.
Developmental medicine and child neurologyAcute rheumatic fever outbreak in southern central European country.
European journal of pediatricsLanguage Impairment in Adolescents with Sydenham Chorea.
Pediatric neurology briefsAutoimmunity against dopamine receptors in neuropsychiatric and movement disorders: a review of Sydenham chorea and beyond.
Acta physiologica (Oxford, England)The usefulness of immunotherapy in pediatric neurodegenerative disorders: A systematic review of literature data.
Human vaccines & immunotherapeuticsA New Alternative Drug With Fewer Adverse Effects in the Treatment of Sydenham Chorea: Levetiracetam Efficacy in a Child.
Clinical neuropharmacologyAutoantibodies in movement and psychiatric disorders: updated concepts in detection methods, pathogenicity, and CNS entry.
Annals of the New York Academy of SciencesAntineuronal antibodies in a heterogeneous group of youth and young adults with tics and obsessive-compulsive disorder.
Journal of child and adolescent psychopharmacologyTherapeutic 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 psychopharmacologySydenham chorea in a 5-year-old Saudi patient.
Neurosciences (Riyadh, Saudi Arabia)Associações
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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.
- Secondary tics: Etiology, presentation, and management.
- An Internal Focus of Attention Benefits Motor Function in Children with Sydenham Chorea Syndrome: A Case Study.
- Sydenham Chorea With Elevated Interleukin-12 Levels Responsive to Plasmapheresis and Immunotherapy.
- Evaluation, Diagnosis, and Treatment of Sydenham Chorea: Consensus Guidelines.
- A Refractory Case of Sydenham Chorea Managed With Intravenous Pulse-Dose Methylprednisolone.
- Revisiting an immunoneuropsychiatry classic: Sydenham's chorea.
- Beyond the dance: A muted narrative of Sydenham chorea.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:306731(Orphanet)
- MONDO:0017648(MONDO)
- GARD:7716(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- 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
