Asymmetry and heterogeneity of Alzheimer's and frontotemporal pathology in primary progressive aphasia
Open accessAbstract
Fifty-eight autopsies of patients with primary progressive aphasia are reported. Twenty-three of these were previously described (Mesulam et al., 2008) but had their neuropathological diagnoses updated to fit current criteria. Thirty-five of the cases are new. Their clinical classification was guided as closely as possible by the 2011 consensus guidelines (Gorno-Tempini et al., 2011). Tissue diagnoses included Alzheimer's disease in 45% and frontotemporal lobar degeneration (FTLD) in the others, with an approximately equal split between TAR DNA binding protein 43 proteinopathies and tauopathies. The most common and distinctive feature for all pathologies associated with primary progressive aphasia was the asymmetric prominence of atrophy, neuronal loss, and disease-specific proteinopathy in the language-dominant (mostly left) hemisphere. The Alzheimer's disease pathology in primary progressive aphasia displayed multiple atypical features. Males tended to predominate, the neurofibrillary pathology was more intense in the language-dominant hemisphere, the Braak pattern of hippocampo-entorhinal prominence was tilted in favour of the neocortex, and the APOE e4 allele was not a risk factor. Mean onset age was under 65 in the FTLD as well as Alzheimer's disease groups. The FTLD-TAR DNA binding protein 43 group had the youngest onset and fastest progression whereas the Alzheimer's disease and FTLD-tau groups did not differ from each other in either onset age or progression rate. Each cellular pathology type had a preferred but not invariant clinical presentation. The most common aphasic manifestation was of the logopenic type for Alzheimer pathology and of the agrammatic type for FTLD-tau. The progressive supranuclear palsy subtype of FTLD-tau consistently caused prominent speech abnormality together with agrammatism whereas FTLD-TAR DNA binding protein 43 of type C consistently led to semantic primary progressive aphasia. The presence of agrammatism made Alzheimer's disease pathology very unlikely whereas the presence of a logopenic aphasia or word comprehension impairment made FTLD-tau unlikely. The association of logopenic primary progressive aphasia with Alzheimer's disease pathology was much more modest than has been implied by results of in vivo amyloid imaging studies. Individual features of the aphasia, such as agrammatism and comprehension impairment, were as informative of underlying pathology as more laborious subtype diagnoses. At the single patient level, no clinical pattern was pathognomonic of a specific neuropathology type, highlighting the critical role of biomarkers for diagnosing the underlying disease. During clinical subtyping, some patients were unclassifiable by the 2011 guidelines whereas others simultaneously fit two subtypes. Revisions of criteria for logopenic primary progressive aphasia are proposed to address these challenges.
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In behavioural or dysexecutive variants, corticobasal syndrome, and semantic or non-fluent variants of primary progressive aphasia, positive Alzheimer's disease biomarkers are particularly ambiguous and can be considered either as Alzheimer's disease copathology or atypical forms of Alzheimer's disease.
“This biomarker positivity is particularly ambiguous in the case of behavioural or dysexecutive variants, corticobasal syndrome, and semantic or non-fluent variants of primary progressive aphasia, in which the presence of positive Alzheimer’s disease biomarkers can be considered either as Alzheimer’s disease copathology or atypical forms of Alzheimer’s disease94,96,98,99 (appendix p 2).”
From Clinical diagnosis of Alzheimer’s disease: Recommendations of the International Working Group (2021) · cites this paper as [99]
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Telerehabilitation of anomia in primary progressive aphasia
Meyer, A. M., Getz, H. R., Brennan, D. M., Hu, T. M., & Friedman, R. B. (2016) · Aphasiology · doi:10.1080/02687038.2015.1081142 · Cited by 68 · see citing works
“…Brambati, Ogar, Neuhaus, Miller, & Gorno-Tempini, 2009; Graham, 2014; Wilson et al., 2010). nfvPPA has been associated with tau-positive frontotemporal lobar degeneration in a majority of cases (FTLD-T; Hodges et al., 2004; Knibb, Xuereb, Patterson, & Hodges, 2006; Mesulam et al., 2008, 2014).”
BackgroundInfluentialBiomarkers for diseases with TDP-43 pathology
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Neuropsychological differentiation of progressive aphasic disorders
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Uniform data set language measures for bvFTD and PPA diagnosis and monitoring
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“Anotherchallengingsituationcan bethedisentanglementofPPAvariantswhichall includepredominantlanguageimpairmentbutare associatedtovariableneuropathologicallesions[32].”
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Altered functional connectivity of cortical networks in semantic variant primary progressive aphasia
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Frontotemporal dementia: does structural MRI-based clustering match clinical syndromes?
Singh-Reilly, N., Sintini, I., Ali, F., Duffy, J. R., Clark, H. M., Utianski, R. L., Meade, G., Machulda, M. M., Satoh, R., Lowe, V. J., et al. (2026) · Frontiers in Neuroscience · doi:10.3389/fnins.2026.1771092
- Authors
- Mesulam, M.-M., Weintraub, S., Rogalski, E. J., Wieneke, C., Geula, C., & Bigio, E. H.
- Affiliations
- Northwestern University (US)
- Corresponding author
- Mesulam, M.-Marsel
- Venue
- Brain
- Publisher
- Oxford University Press (OUP)
- Published
- 2014-02-25
- Volume
- 137
- Issue
- 4
- Pages
- 1176-1192
- ISSN
- 0006-8950 (ISSN-L)
- 1460-2156
- PMC
- PMC3959558
- OpenAlex
- W2150070900
- Cited by
- 334
- References
- 67
- Citations by year
- 2026: 12 · 2025: 21 · 2024: 16 · 2023: 17 · 2022: 34 · 2021: 40 · 2020: 37 · 2019: 33 · 2018: 36 · 2017: 23 · 2016: 30 · 2015: 22 · 2014: 13
- FWCI
- 22.40
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Brain (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institute on Aging (AG13854, P30AG013854)
- National Institute on Deafness and Other Communication Disorders (R01DC008552, DC008552)
- National Institute of Neurological Disorders and Stroke (R01NS075075)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- primary progressive aphasia, Alzheimer's disease, frontotemporal lobar degeneration, tauopathy, progressive supranuclear palsy, semantic dementia, logopenic aphasia, agrammatism, neurofibrillary pathology, APOE ε4, age of onset, disease progression
- MeSH terms
- Aged, Aged, 80 and over, Alzheimer Disease, Autopsy, Brain, Female, Humans, Male, Middle Aged, Aphasia, Primary Progressive, Frontotemporal Lobar Degeneration
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