{"orpha":624166,"name":"Non-specific autoimmune supratentorial encephalitis with characteristic antibodies","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Non-specific supratentorial AE with characteristic antibodies"],"definition":null,"note":null,"url":"https://raremap.alftech.space/d/ORPHA-624166/","orphanet_url":"https://www.orpha.net/en/disease/detail/624166","names":{"fr":{"name":"Encéphalite auto-immune supratentoriale non-spécifique avec anticorps caractéristiques","synonyms":["EA supratentoriale non-spécifique avec anticorps caractéristiques"]},"de":{"name":"Unspezifische autoimmune supratentorielle Enzephalitis mit charakteristischen Antikörpern","synonyms":["Unspezifische supratentorielle AE mit charakteristischen Antikörpern"]},"es":{"name":"Encefalitis autoinmune supratentorial inespecífica con anticuerpos característicos","synonyms":["EA supratentorial inespecífica con anticuerpos característicos"]},"it":{"name":"Encefalite autoimmune sopratentoriale aspecifica con anticorpi caratteristici","synonyms":["EA sopratentoriale aspecifica con anticorpi caratteristici"]},"nl":{"name":"Aspecifieke auto-immune supratentoriële encefalitis met typische antilichamen","synonyms":["Aspecifieke supratentoriële AE met typische antilichamen"]},"pt":{"name":"Encefalite supratentorial autoimune não-específica com anticorpos característicos","synonyms":["AE supratentorial não-específica com anticorpos característicos"]},"pl":{"name":"Niespecyficzne autoimmunologiczne nadnamiotowe zapalenie mózgu z charakterystycznymi przeciwciałami","synonyms":["Non-specific supratentorial AE with characteristic antibodies"]},"cs":{"name":"Nespecifická autoimunitní supratentoriální encefalitida s charakteristickými protilátkami","synonyms":["Non-specific supratentorial AE with characteristic antibodies"]},"uk":{"name":"Неспецифічний аутоімунний супратенторіальний енцефаліт з характерними антитілами","synonyms":["Неспецифічний супратенторіальний АЕ з характерними антитілами"]}},"xrefs":[{"source":"ICD-10","code":"G04.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0850102","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680408","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":622014,"name":"Autoimmune encephalitis","classifications":[181]},{"orpha":166484,"name":"Inflammatory and autoimmune disease with epilepsy","classifications":[181]},{"orpha":36388,"name":"Paraneoplastic neurologic syndrome","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2025-08-19","source":"35150165[PMID]_31009048[PMID]_32196746[PMID]","terms":[{"hpo":"HP:6001059","label":"Anti-DREBRIN antibody positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:6000877","label":"Anti-dachshund family transcription factor 1 antibody positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:6000878","label":"Anti-potassium channel tetramerization domain containing 16 antibody positivity","frequency":"Frequent (79-30%)","criteria":null}]}}