{"orpha":3451,"name":"West syndrome","type":"Clinical syndrome","group":"Disorder","status":"inactive","flags":["Deprecated entity"],"synonyms":["Intellectual disability-hysarrhytmia syndrome"],"definition":null,"note":"This entity has been excluded from the Orphanet nomenclature of rare diseases and moved to Infantile epileptic spasms syndrome","url":"https://raremap.alftech.space/d/ORPHA-3451/","orphanet_url":"https://www.orpha.net/en/disease/detail/3451","names":{"fr":{"name":"Syndrome de West","synonyms":["Syndrome de déficience intellectuelle-hysarrhythmie"]},"de":{"name":"West-Syndrom","synonyms":["Intelligenzminderung-Hypsarrhythmie-Syndrom"]},"es":{"name":"Síndrome de West","synonyms":["Síndrome de discapacidad intelectual-hipsarritmia"]},"it":{"name":"Sindrome di West","synonyms":["Sindrome disabilità intellettiva-disaritmia","Disabilità intellettiva-isarritmia","IESS"]},"nl":{"name":"Syndroom van West","synonyms":["Intellectuele achterstand - hypsaritmie-syndroom"]},"pt":{"name":"Síndrome West"},"pl":{"name":"Zespół Westa","synonyms":["Drgawki dziecięce","Niepełnosprawność intelektualna - hipsarytmia"]},"cs":{"name":"Westův syndrom","synonyms":["Infantilní spasmy","Syndrom zahrnující mentální retardaci a hypsarytmii na EEG","Bleskové křeče"]},"uk":{"name":"Синдром інфантильних спазмів","synonyms":["Синдром Веста"],"definition":"A rare epilepsy syndrome characterized by onset of epileptic spasms in infants between 2 and 12 months of age, and rarely up to 24 months. Infants may have no antecedent history, or a history reflecting the underlying cause. The classical triad of epileptic spasms, hypsarrhythmia and developmental stagnation or regression is historically referred to as West syndrome."},"zh":{"name":"West综合征","synonyms":["智力障碍-高度节律失调综合征"]},"tr":{"name":"West sendromu","synonyms":["İnfantil spazmlar","Zihinsel yetersizlik-hipsaritmi sendromu"]}},"xrefs":[{"source":"ICD-11","code":"8A62.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1023597213"},{"source":"MONDO","code":"0018097","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D013036","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0037769","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7887","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10021750","qualifier":"E","icd_relation":null,"validated":true}],"associations":[{"type":"Moved to","orpha":697160,"name":"Infantile epileptic spasms syndrome"}],"incoming_associations":[],"preferential_parent":null,"parents":[],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}