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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

Classification level: Disorder
  • 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
  • ICD-11: 6D83
  • OMIM: 172700  600274  607485
  • UMLS: C0751706
  • MeSH: D057178
  • GARD: 10793
  • MedDRA: 10029542

  A summary on this disease is available in Español (2014) Français (2014) Nederlands (2014)

Detailed information

General public

Guidelines

Disease review articles

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

Newborn screening

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.