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

Etiology: 1) Alzheimer's disease (48%); (40%) [4] 2) frontotemporal lobar degeneration (52%); (60%) [4]* * ref [5] describes primary progressive aphasia as a language variant of frontotemporal dementia Pathology: - each variant of primary progressive aphasia is associated with a different anatomical site of maximal neuropathology - agrammatic variant: inferior frontal gyrus (Broca's area) - logopenic variant: temporoparietal junction (Wernicke's area) - semantic variant: - anterior temporal lobe - role in object naming & word comprehension - left anterior temporal lobe is specialized for word comprehension (recognition) - right anterior temporal lobe may serve a similar function for objects & faces [4] Genetics: 40% associated with apoE4 allele Clinical manifestations: 1) inability to construct sentences 2) effortful, halting speech consisting largely of single words 3) inconsistent correct pronunciation of words 4) less likely to manifest neuropsychiatic symptoms than frontotemporal dementia or semantic dementia 5) dysphagia in later phases 6) understanding of words & general concepts intact 7) most commonly starts before age 65 years [4] 8) 3 variants a) agrammatic - construction of grammatically incorrect sentences - loss of fluency - preserved word comprehension b) logopenic - impairment of word finding - poor language repetition - fluctuating fluency - preserved grammar & word comprehension c) semantic variant - difficulty comprehending words & concepts - impairment of object naming - preserved fluency, repetition, & grammar 9) memory may be preserved over time (> 2.5 years) [7] Special laboratory: - swallowing evaluation - cognitive testing - most language-based, thus performance may be worse than functional impairment [5] Radiology: - MRI of brain: left temporal atrophy [5] Differential diagnosis: 1) Alzheimer's disease (AD) 2) frontotemporal dementia (FTD) 3) stroke Management: 1) no good therapy 2) speech therapy & language therapy [5] 2) pharmaceuticals a) amantadine b) bromocriptine c) cholinesterase inhibitor may be useful, given high incidence of Alzheimer's disease

General

aphasia

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: 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: 21325651
  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. 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: 25179257 Free PMC Article
  7. 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: 33433867