primary progressive aphasia (PPA, progressive non-fluent aphasia, PNFA)
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Etiology
- Alzheimer's disease (48%); (40%)[4]
- 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]
- anterior temporal lobe
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 (fluency preserved[5])
- preserved grammar & word comprehension
- semantic variant
- difficulty comprehending words & concepts
- impairment of object naming
- preserved fluency, repetition, & grammar
- agrammatic
- memory may be preserved over time (> 2.5 years)[7]
Diagnostic procedures
- swallowing evaluation
- cognitive testing
- most language-based, thus performance may be worse than functional impairment[5]
Radiology
- MRI of brain:
- dominant temporal lobe atrophy, parietal lobe atrophy[5]
Differential diagnosis
Management
- no good therapy
- speech therapy & language therapy[5]
- pharmaceuticals
- amantadine
- bromocriptine
- cholinesterase inhibitor may be useful, given high incidence of Alzheimer's disease
More general terms
References
- ↑ Tiffany Chow, MD, UCLA Brain Matters, 09/23/02 Director Rancho Los Amigos/USC Frontotemporal dementia Clinic & Research Program
- ↑ Mesulam M et al. Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Ann Neurol 2008 Jun; 63:709. PMID: https://pubmed.ncbi.nlm.nih.gov/18412267
- ↑ 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://pubmed.ncbi.nlm.nih.gov/21325651
- ↑ 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/
- ↑ 5.0 5.1 5.2 5.3 5.4 5.5 Medical Knowledge Self Assessment Program (MKSAP) 17, 18. American College of Physicians, Philadelphia 2015, 2018
Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025 - ↑ 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://pubmed.ncbi.nlm.nih.gov/25179257 Free PMC Article
- ↑ 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 - ↑ 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://pubmed.ncbi.nlm.nih.gov/33433867
- ↑ Tippett DC, Keser Z. Clinical and neuroimaging characteristics of primary progressive aphasia. Handb Clin Neurol. 2022;185:81-97. PMID: https://pubmed.ncbi.nlm.nih.gov/35078612