{"orpha":306731,"name":"Sydenham chorea","type":"Particular clinical situation in a disease or syndrome","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare acquired autoimmune movement disorder characterized by chorea, hypotonia, and emotional lability. Additional clinical features may include motor impersistence and various neuropsychiatric symptoms. As a major clinical manifestation of acute rheumatic fever (ARF), the disorder typically emerges as a post-infectious complication of a group A streptococcal infection and frequently presents alongside cardiac involvement, such as clinical or subclinical carditis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-306731/","orphanet_url":"https://www.orpha.net/en/disease/detail/306731","names":{"fr":{"name":"Chorée de Sydenham"},"de":{"name":"Sydenham-Chorea"},"es":{"name":"Corea de Sydenham"},"it":{"name":"Corea di Sydenham"},"nl":{"name":"Sydenhamchorea"},"pt":{"name":"Coreia de Sydenham"},"pl":{"name":"pląsawica Sydenhama"},"cs":{"name":"Sydenhamova chorea"},"uk":{"name":"Хорея Сиденхема"},"zh":{"name":"Sydenham舞蹈病"},"tr":{"name":"Sydenham koresi"}},"xrefs":[{"source":"ICD-10","code":"I02.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"I02.9","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"1B42","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1295812812"},{"source":"MONDO","code":"0017648","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0152113","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7716","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10042732","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":306727,"name":"Postinfectious autoimmune disease with chorea","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Adult","Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2018-04-18","source":"21386976[PMID]_16533893[PMID]","terms":[{"hpo":"HP:0000708","label":"Atypical behavior","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002072","label":"Chorea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000722","label":"Compulsive behaviors","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001260","label":"Dysarthria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000712","label":"Emotional lability","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000273","label":"Facial grimacing","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001290","label":"Generalized hypotonia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100248","label":"Hemiballismus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000719","label":"Inappropriate behavior","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000737","label":"Irritability","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000182","label":"Movement abnormality of the tongue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002317","label":"Unsteady gait","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100584","label":"Endocarditis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005366","label":"Recurrent streptococcus pneumoniae infections","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003095","label":"Septic arthritis","frequency":"Occasional (29-5%)","criteria":null}]}}