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Progressive non-fluent aphasia

Disease definition

Progressive non-fluent aphasia (PNFA) is a form of frontotemporal dementia (FTD; see this term), characterized by agrammatism, laborious speech, alexia, and agraphia, frequently accompanied by apraxia of speech (AOS). Language comprehension is relatively preserved.

ORPHA:100070

  • Synonym(s):
    • Agramatic variant of PPA
    • Agramatic variant of primary progressive aphasia
    • Non-fluent variant PPA
  • Prevalence: 1-9 / 100 000
  • Inheritance: Multigenic/multifactorial or Not applicable 
  • Age of onset: Adult
  • ICD-10: G31.0
  • OMIM: 600274  607485
  • UMLS: C0751706
  • MeSH: D057178
  • GARD: 10793
  • MedDRA: 10029542

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