{"orpha":95432,"name":"Primary progressive aphasia","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["Mesulam syndrome","PPA"],"definition":"Primary progressive aphasia (PPA) is a neurodegenerative disorder, characterized by a primary dissolution of language, with relative sparing of other mental faculties for at least the first 2 years of illness. PPA is recognized as the language variant in the frontotemporal dementia (FTD) spectrum of disorders. PPA can be classified into 3 subtypes based on specific speech and language features: semantic dementia (SD), progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (lv-PPA).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-95432/","orphanet_url":"https://www.orpha.net/en/disease/detail/95432","names":{"fr":{"name":"Aphasie primaire progressive","synonyms":["PPA","Syndrome de Mesulam"],"definition":"L'aphasie primaire progressive (PPA) est un trouble neurodégénératif caractérisé par une dissolution primaire du langage, avec une préservation relative des autres facultés mentales sur au moins 2 ans après le début de la maladie. La PPA est reconnue comme une variante de langage du spectre de la démence fronto-temporale (DFT). Elle peut être classifiée en 3 sous-types sur la base du discours spécifique et des caractéristiques du langage : la démence sémantique (APP sémantique), l'aphasie primaire progressive non fluente (APP non fluente) et l'aphasie primaire progressive logopénique (APP logopénique)."},"de":{"name":"Aphasie, primäre progressive","synonyms":["Mesulam-Syndrom","PPA"]},"es":{"name":"Afasia progresiva primaria","synonyms":["APP","Síndrome de Mesulam"],"definition":"La afasia progresiva primaria (APP) es un trastorno neurodegenerativo caracterizado por un deterioro primario del lenguaje, con relativa preservación de otras facultades mentales durante al menos los primeros 2 años de la enfermedad. La APP se reconoce como la variante del lenguaje en el espectro de trastornos de la demencia frontotemporal (DFT). La APP se puede clasificar en 3 subtipos o variantes según las características específicas del habla y el lenguaje: demencia semántica (APP-vs), afasia progresiva no fluente (APP-vnf) y afasia progresiva logopénica (APP-vl)."},"it":{"name":"Afasia primitiva progressiva","synonyms":["PPA","Sindrome di Mesulam"]},"nl":{"name":"Primaire progressieve afasie","synonyms":["PPA","Syndroom van Mesulam"],"definition":"'Primaire progressieve afasie (PPA) is een neurodegeneratieve aandoening die wordt gekarakteriseerd door vooral stoornis van de taal, terwijl andere mentale vermogens relatief gespaard blijven, op zijn minst gedurende de eerste 2 jaren van de ziekte. PPA wordt erkend als de taalvariant van het fronotemporale dementie (FTD; zie deze term) spectrum van aandoeningen. Op basis van specifieke kenmerken van de spraak en de taal kan PPA ingedeeld worden in 3 subtypes: semantische dementie (SD), progressieve niet-vloeiende afasie (PNFA) en logopenische progressieve afasie (Iv-PPA) (zie deze termen).'"},"pt":{"name":"Afasia primária progressiva"},"pl":{"name":"Pierwotna postępująca afazja","synonyms":["PPA","Zespół Mesulama"]},"cs":{"name":"Primární progresivní afázie","synonyms":["PPA","Mesulamův syndrom"]},"uk":{"name":"Первинна прогресуюча афазія","synonyms":["Синдром Мезулама","PPA"],"definition":"Primary progressive aphasia (PPA) is a neurodegenerative disorder, characterized by a primary dissolution of language, with relative sparing of other mental faculties for at least the first 2 years of illness. PPA is recognized as the language variant in the frontotemporal dementia (FTD; see this term) spectrum of disorders. PPA can be classified into 3 subtypes based on specific speech and language features: semantic dementia (SD), progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (lv-PPA) (see these terms)."},"zh":{"name":"原发性进行性失语","synonyms":["Mesulam综合征"],"definition":"原发性进行性失语症（PPA）是一种神经退行性疾病，其特征是原发性语言丧失，在发病的至少前2年内，其他精神功能受累相对较少。PPA被认为是额颞叶痴呆（FTD；见该词条）谱系障碍中的语言变体。根据特定的语言特征，PPA可分为3个亚型：语义性痴呆（SD）、进行性非流利性失语（PNFA）和Logopenic型进行性失语（lv-PPA）（见这些词条）。"},"tr":{"name":"Primer ilerleyici afazi","synonyms":["PPA","Mesulam sendromu"]}},"xrefs":[{"source":"ICD-11","code":"6D83","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1693157226"},{"source":"MONDO","code":"0019806","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D018888","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0282513","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"8541","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10081268","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":98534,"name":"Neurodegenerative disease with dementia","classifications":[181]}],"children":[{"orpha":250831,"name":"Logopenic progressive aphasia","type":"Disease"},{"orpha":100070,"name":"Progressive non-fluent aphasia","type":"Disease"},{"orpha":100069,"name":"Semantic dementia","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":7.0,"area":"Worldwide","status":"Not yet validated","source":"24108322[PMID]"}],"phenotypes":null}