primary progressive aphasia (PPA, progressive non-fluent aphasia, PNFA)

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Etiology

* ref[5] describes primary progressive aphasia as a language variant of frontotemporal dementia

Pathology

Genetics

40% associated with apoE4 allele

Clinical manifestations

  • inability to construct sentences
  • effortful, halting speech consisting largely of single words
  • inconsistent correct pronunciation of words
  • less likely to manifest neuropsychiatic symptoms than frontotemporal dementia or semantic dementia
  • dysphagia in later phases
  • understanding of words & general concepts intact
  • most commonly starts before age 65 years[4]
  • 3 variants
    • agrammatic
      • construction of grammatically incorrect sentences
      • loss of fluency
      • preserved word comprehension
    • logopenic
      • impairment of word finding
      • poor language repetition
      • fluctuating fluency
      • preserved grammar & word comprehension
    • semantic variant
      • difficulty comprehending words & concepts
      • impairment of object naming
      • preserved fluency, repetition, & grammar
  • memory may be preserved over time (> 2.5 years)[7]

Diagnostic procedures

Radiology

Differential diagnosis

Management

More general terms

References

  1. Tiffany Chow, MD, UCLA Brain Matters, 09/23/02 Director Rancho Los Amigos/USC Frontotemporal dementia Clinic & Research Program
  2. Mesulam M et al. Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Ann Neurol 2008 Jun; 63:709. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18412267
  3. Gorno-Tempini ML, Hillis AE, Weintraub S et al Classification of primary progressive aphasia and its variants. Neurology. 2011 Mar 15;76(11):1006-14 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21325651
  4. 4.0 4.1 4.2 4.3 4.4 Rothaus C A Woman with Progressive Loss of Language. NEJM Resident 360 clinical pearls. Jan 11, 2017 https://resident360.nejm.org/content_items/2170/
  5. 5.0 5.1 5.2 5.3 5.4 Medical Knowledge Self Assessment Program (MKSAP) 17, 18. American College of Physicians, Philadelphia 2015, 2018
  6. Mesulam MM, Rogalski EJ, Wieneke C et al Primary progressive aphasia and the evolving neurology of the language network. Nat Rev Neurol. 2014 Oct;10(10):554-69. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/25179257 Free PMC Article
  7. 7.0 7.1 George J Primary Progressive Aphasia in Alzheimer's Doesn't Foretell Memory Loss. No memory decline over more than 2 years in small study, though speech deficits grew. MedPage Today January 13, 2021 https://www.medpagetoday.com/neurology/alzheimersdisease/90683
    Mesulam, Coventry C, Kuang A et al Memory resilience in Alzheimer's disease with primary progressive aphasia. Neurology, 2021. Jan 13. https://n.neurology.org/content/early/2021/01/11/WNL.0000000000011397
  8. Mesulam M-, Coventry C, Bigio EH et al Nosology of Primary Progressive Aphasia and the Neuropathology of Language. Adv Exp Med Biol. 2021;1281:33-49 PMID: https://www.ncbi.nlm.nih.gov/pubmed/33433867