{"orpha":1183,"name":"Opsoclonus-myoclonus syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Ataxo-opso-myoclonus syndrome","Dancing eye syndrome","Dancing eye-dancing feet syndrome","Kinsbourne syndrome","OMA syndrome","OMS","Opsoclonus-myoclonus-ataxia syndrome","POMA syndrome","Paraneoplastic opsoclonus-myoclonus","Paraneoplastic opsoclonus-myoclonus-ataxia syndrome"],"definition":"Opsoclonus myoclonus syndrome (OMS) is a rare neuroinflammatory disease of paraneoplastic, parainfectious or idiopathic origin, characterized by opsoclonus, myoclonus, ataxia, and behavioral and sleep disorders.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-1183/","orphanet_url":"https://www.orpha.net/en/disease/detail/1183","names":{"fr":{"name":"Syndrome d'opsoclonie-myoclonie","synonyms":["OMP","OMS","Opsoclonus-myoclonus paranéoplasique","Syndrome OMA","Syndrome ataxo-opso-myoclonus","Syndrome d'opsoclonie-myoclonie-ataxie","Syndrome de Kinsbourne","Syndrome des yeux dansants","Syndrome néoplasique d'opsoclonie-myoclonie-ataxie","Syndrome opsoclonus-myoclonus","syndrome des yeux et des jambes dansantes"],"definition":"Maladie neuroinflammatoire rare d'origine paranéoplasique, parainfectieuse ou idiopathique, caractérisée par une opsoclonie, une myoclonie, une ataxie et des troubles du comportement et du sommeil."},"de":{"name":"Opsoklonus-Myoklonus-Syndrom","synonyms":["Kinsbourne-Syndrom","OMA-Syndrom","OMS","Opsoklonie - Myoklonie - Ataxie","Opsomyoklonus-Ataxie-Syndrom","POMA-Syndrom","Paraneoplastische Opsoklonus-Myoklonus-Ataxie","Paraneoplastischer Opsomyoklonus"],"definition":"Das Opsoklonus-Myoklonus-Syndrom (OMS) ist eine seltene neuroinflammatorische Krankheit des Kindesalters. Es ist gekennzeichnet durch Opsoklonus, Myoklonus, Ataxie, Verhaltens- und Schlafstörungen und tritt entweder im Gefolge eines Neuroblastoms als paraneoplastisches Syndrom oder nach einer viralen Infektion auf."},"es":{"name":"Síndrome opsoclono-mioclono","synonyms":["OMS","Opsoclono-mioclono paraneoplásico","Síndrome OMA","Síndrome POMA","Síndrome ataxo-opso-mioclono","Síndrome de Kinsbourne","Síndrome de los ojos bailarines","Síndrome de ojos bailarines-pies bailarines","Síndrome de opsoclono-mioclono-ataxia","Síndrome de opsoclono-mioclono-ataxia paraneoplásico"],"definition":"El síndrome de opsoclono-mioclono (OMS) es una enfermedad neuroinflamatoria rara de origen paraneoplásico, parainfeccioso o idiopático, caracterizada por opsoclono, mioclono, ataxia, y trastornos del comportamiento y del sueño."},"it":{"name":"Sindrome opsoclono-mioclono","synonyms":["OMS","Opsoclono-mioclono, paraneoplastico","Sindrome OMA","Sindrome POMA","Sindrome da atasso-opso-mioclono","Sindrome degli ''occhi ballerini''","Sindrome degli occhi e dei piedi ballerini","Sindrome di Kinsbourne","Sindrome opsoclono-mioclono-atassia","Sindrome opsoclono-mioclono-atassia, paraneoplastico"],"definition":"La sindrome opsoclonia-mioclonia (OMS) è una malattia neuroinfiammatoria rara di origine paraneoplastica, parainfettiva o idiopatica, caratterizzata da opsoclonia, mioclonia, atassia e disturbi comportamentali e del sonno."},"nl":{"name":"Opsoclonus - myoclonus-syndroom","synonyms":["Ataxo-opso-myoclonussyndroom","Dancing eye - dancing feet-syndroom","Dancing eye-syndroom","OMA-syndroom","OMS","Opsoclonus - myoclonus - ataxie-syndroom","POMA-syndroom","Paraneoplastisch opsoclonus - myoclonus - ataxie-syndroom","Paraneoplastische opsoclonus-myoclonus","Syndroom van Kinsbourne"],"definition":"Opsoclonus-myoclonussyndroom (OMS) is een zeldzame neuro-inflammatoire ziekte met een paraneoplastische, para-infectieuze of idiopathische oorsprong die gekarakteriseerd wordt door opsoclonus, myoclonus, ataxie, en gedrags- en slaapstoornissen."},"pt":{"name":"Síndrome de opsoclonia-mioclonia","synonyms":["Síndrome Kinsbourne"]},"pl":{"name":"Zespół mioklonii i opsoklonii","synonyms":["OMS","Paraneoplastyczne opsoklonie i mioklonie","Paraneoplastyczny zespół ataksji, opsoklonii i mioklonii","Zespół ataksji, opsoklonii i mioklonii","Zespół Kinsbourne'a","Zespół OMA","Zespół POMA","Zespół tańczącego oka","Zespół tańczącego oka i tańczących stóp"]},"cs":{"name":"Syndrom zahrnující opsoklonus a myoklonus","synonyms":["Syndrom ataxoopsomyoklonus","OMA syndrom","OMS","Syndrom zahrnující opsoklonus, myoklonus a ataxii","POMA syndrom","Paraneoplastický opsoklonus a myoklonus","Paraneoplastický syndrom zahrnující opsoklonus, myoklonus a ataxii","Syndrom tančících očí","Syndrom tančících očí a nohou","Kinsbournův syndrom"]},"uk":{"name":"Синдром опсоклонус-міоклонус","synonyms":["Атаксо-опсо-міоклонусний синдром","Синдром танцюючих очей","Синдром танцюючих очей-танцюючих ніг","Синдром Кінсборна","Синдром ОМА","OMS","Синдром опсоклонус-міоклонус-атаксія","Паранеопластичний опсоклонус-міоклонус","Паранеопластичний синдром опсоклонус-міоклонус-атаксія","Синдром POMA"],"definition":"Opsoclonus myoclonus syndrome (OMS) is a rare neuroinflammatory disease of paraneoplastic, parainfectious or idiopathic origin, characterized by opsoclonus, myoclonus, ataxia, and behavioral and sleep disorders."},"zh":{"name":"眼球阵挛-肌阵挛综合征","synonyms":["副肿瘤性斜视性眼肌阵挛-肌阵挛-共济失调综合征"]},"tr":{"name":"Opsoklonus-miyoklonus sendromu","synonyms":["OMS","OMA sendromu","POMA sendromu","Kinsbourne sendromu","Dans eden göz sendromu","Atakso-opso-miyoklonus sendromu","Dans eden göz dansı ayak sendromu","Paraneoplastik opsoklonus-miyoklonus","Opsoklonus-miyoklonus-ataksi sendromu","Paraneoplastik opsoklonus-miyoklonus-ataksi sendromu"]}},"xrefs":[{"source":"ICD-10","code":"G25.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9C85.02","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"936930510"},{"source":"MONDO","code":"0015247","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D053578","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0393626","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10009","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10053854","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":102005,"name":"Brain inflammatory disease","classifications":[181]},{"orpha":36388,"name":"Paraneoplastic neurologic syndrome","classifications":[181]},{"orpha":519341,"name":"Rare brainstem or cerebellar disorder with ophthalmic involvement as a major feature","classifications":[189]},{"orpha":306753,"name":"Rare disease with myoclonus as a major feature","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood","Infancy"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.02,"area":"Europe","status":"Validated","source":"[EXPERT]_19423368[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.018,"area":"United Kingdom","status":"Validated","source":"19423368[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-01-05","source":"30232989[PMID]_30483882[PMID]_23622330[PMID]","terms":[{"hpo":"HP:0000570","label":"Abnormal saccadic eye movements","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001251","label":"Ataxia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001336","label":"Myoclonus","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010543","label":"Opsoclonus","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002360","label":"Sleep abnormality","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:5000002","label":"Anti-Amphiphysin antibody","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000708","label":"Atypical behavior","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100543","label":"Cognitive impairment","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000737","label":"Irritability","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0045084","label":"Limb myoclonus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002664","label":"Neoplasm","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003006","label":"Neuroblastoma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002321","label":"Vertigo","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030057","label":"Autoimmune antibody positivity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003002","label":"Breast carcinoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031035","label":"Chronic infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001298","label":"Encephalopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002861","label":"Melanoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100526","label":"Neoplasm of the lung","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012226","label":"Ovarian teratoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002063","label":"Rigidity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030357","label":"Small cell lung carcinoma","frequency":"Occasional (29-5%)","criteria":null}]}}