{"orpha":282,"name":"Frontotemporal dementia","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["FTD"],"definition":"Frontotemporal dementia (FTD) comprises a group of neurodegenerative disorders, characterized by progressive changes in behavior, executive dysfunction and language impairment, as a result of degeneration of the medial prefrontal and frontoinsular cortices. Four clinical subtypes have been identified: semantic dementia, progressive non-fluent aphasia, behavioral variant FTD and right temporal lobar atrophy.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-282/","orphanet_url":"https://www.orpha.net/en/disease/detail/282","names":{"fr":{"name":"Démence fronto-temporale","synonyms":["DFT","FTD"],"definition":"La démence fronto-temporale (DFT) comprend un groupe de troubles neurodégénératifs caractérisés par des changements progressifs du comportement, une dysfonction exécutive et un trouble du langage, résultant de la dégénérescence du cortex médian préfrontal et fronto-insulaire. Quatre sous-types cliniques ont été identifiés : la démence sémantique, l'aphasie primaire progressive non fluente, la DFT variante comportementale et l'atrophie du lobe temporal droit."},"de":{"name":"Frontotemporale Demenz","synonyms":["FTD"],"definition":"Die Frontotemporale Demenz (FTD) umfasst eine Gruppe neurodegenerativer Erkrankungen, die durch fortschreitende Verhaltensänderungen, exekutive Dysfunktion und Sprachstörungen infolge einer Degeneration der medialen präfrontalen und frontoinsulären Kortex gekennzeichnet sind. Es wurden vier klinische Subtypen identifiziert: Semantische Demenz, progrediente nicht-flüssige Aphasie, Verhaltensvariante der FTD und Atrophie des rechten Temporallappens (siehe dort)."},"es":{"name":"Demencia frontotemporal","synonyms":["DFT"],"definition":"La demencia frontotemporal (DFT) comprende un grupo de trastornos neurodegenerativos, caracterizados por cambios progresivos de la conducta, disfunción ejecutiva y deterioro del lenguaje, como resultado de la degeneración de las cortezas prefrontal medial y frontoinsular. Se han identificado cuatro subtipos clínicos: demencia semántica, afasia progresiva no fluente, DFT variante conductual y atrofia del lóbulo temporal derecho (consulte estos términos)."},"it":{"name":"Demenza frontotemporale","synonyms":["FTD"]},"nl":{"name":"Frontotemporale dementie","synonyms":["FTD"],"definition":"Frontotemporale dementie (FTD) omvat een groep neurodegeneratieve aandoeningen die worden gekarakteriseerd door progressieve gedragsveranderingen, executieve disfunctie en spraakstoornis ten gevolge van degeneratie van de mediale prefrontale cortex en de insula. Er werden vier klinische subtypes geïdentificeerd: semantische dementie, progressieve niet-vloeiende afasie, gedragsvariant van FTD en rechter temporale atrofie variant (zie deze termen)."},"pt":{"name":"Demência fronto-temporal"},"pl":{"name":"Demencja czołowo-skroniowa","synonyms":["FTD"]},"cs":{"name":"Frontotemporální demence","synonyms":["FTD"]},"uk":{"name":"Лобно-скронева деменція","synonyms":["FTD"],"definition":"Frontotemporal dementia (FTD) comprises a group of neurodegenerative disorders, characterized by progressive changes in behavior, executive dysfunction and language impairment, as a result of degeneration of the medial prefrontal and frontoinsular cortices. Four clinical subtypes have been identified: semantic dementia, progressive non-fluent aphasia, behavioral variant FTD and right temporal lobar atrophy (see these terms)."},"zh":{"name":"额颞叶痴呆","synonyms":["FTD"]},"tr":{"name":"Frontotemporal demans","synonyms":["FTD"]}},"xrefs":[{"source":"OMIM","code":"172700","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"OMIM","code":"600274","qualifier":"E","icd_relation":null,"validated":true},{"source":"OMIM","code":"600795","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"OMIM","code":"607485","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-11","code":"6D83","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"831337417"},{"source":"MONDO","code":"0017276","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"17276","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D057180","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0338451","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"8436","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10068968","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Moved to","orpha":293848,"name":"Frontotemporal dementia, right temporal atrophy variant","status":"inactive"}],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":98535,"name":"Frontotemporal degeneration with dementia","classifications":[181]},{"orpha":276061,"name":"Genetic frontotemporal degeneration with dementia","classifications":[156]}],"children":[{"orpha":275864,"name":"Behavioral variant of frontotemporal dementia","type":"Disease"},{"orpha":100070,"name":"Progressive non-fluent aphasia","type":"Disease"},{"orpha":100069,"name":"Semantic dementia","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal dominant"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.0,"area":"Europe","status":"Not yet validated","source":"20971753[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":3.05,"area":"Italy","status":"Validated","source":"30979859[PMID]"}],"phenotypes":null}