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Understanding the multidimensional cognitive deficits of logopenic variant primary progressive aphasia.

Accepted version
Peer-reviewed

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Article

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Authors

Ramanan, Siddharth 
Irish, Muireann 
Rowe, James B 
Gorno-Tempini, Maria Luisa 

Abstract

The logopenic variant of primary progressive aphasia is characterized by early deficits in language production and phonological short-term memory, attributed to left-lateralized temporoparietal, inferior parietal and posterior temporal neurodegeneration. Despite patients primarily complaining of language difficulties, emerging evidence points to performance deficits in non-linguistic domains. Temporoparietal cortex, and functional brain networks anchored to this region, are implicated as putative neural substrates of non-linguistic cognitive deficits in logopenic variant primary progressive aphasia, suggesting that degeneration of a shared set of brain regions may result in co-occurring linguistic and non-linguistic dysfunction early in the disease course. Here, we provide a Review aimed at broadening the understanding of logopenic variant primary progressive aphasia beyond the lens of an exclusive language disorder. By considering behavioural and neuroimaging research on non-linguistic dysfunction in logopenic variant primary progressive aphasia, we propose that a significant portion of multidimensional cognitive features can be explained by degeneration of temporal/inferior parietal cortices and connected regions. Drawing on insights from normative cognitive neuroscience, we propose that these regions underpin a combination of domain-general and domain-selective cognitive processes, whose disruption results in multifaceted cognitive deficits including aphasia. This account explains the common emergence of linguistic and non-linguistic cognitive difficulties in logopenic variant primary progressive aphasia, and predicts phenotypic diversification associated with progression of pathology in posterior neocortex.

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Keywords

Alzheimer’s disease, inferior parietal lobe, language, non-linguistic functions, primary progressive aphasia, temporoparietal junction

Journal Title

Brain

Conference Name

Journal ISSN

0006-8950
1460-2156

Volume Title

Publisher

Oxford University Press (OUP)
Sponsorship
National Institute for Health and Care Research (IS-BRC-1215-20014)
Medical Research Council (MC_UU_00005/12)
Cambridge University Hospitals NHS Foundation Trust (CUH) (146281)
MRC (MC_UU_00030/9)
Medical Research Council (MR/R023883/1)