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Primary progressive aphasia

Disease 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).

ORPHA:95432

Classification level: Group of disorders
  • Synonym(s):
    • Mesulam syndrome
    • PPA
  • Prevalence: 1-9 / 100 000
  • Inheritance: Multigenic/multifactorial or Not applicable 
  • Age of onset: Adult
  • ICD-10: G31.0
  • OMIM: -
  • UMLS: C0282513
  • MeSH: D018888
  • GARD: 8541
  • MedDRA: -
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