The atrophy pattern in Alzheimer-related PPA is more widespread than that of the frontotemporal lobar degeneration associated variants

Objective: The three recognized variants of primary progressive aphasia (PPA) are associated with different loci of degeneration—left posterior perisylvian in logopenic variant (lvPPA), left frontal operculum in non-fluent variant (nfvPPA), and left rostroventral-temporal in semantic variant (svPPA)...

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Main Authors: Daniel Preiß, Ornella V. Billette, Anja Schneider, Nicola Spotorno, Peter J. Nestor
Format: Article
Language:English
Published: Elsevier 2019-01-01
Series:NeuroImage: Clinical
Online Access:http://www.sciencedirect.com/science/article/pii/S2213158219303444
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author Daniel Preiß
Ornella V. Billette
Anja Schneider
Nicola Spotorno
Peter J. Nestor
author_facet Daniel Preiß
Ornella V. Billette
Anja Schneider
Nicola Spotorno
Peter J. Nestor
author_sort Daniel Preiß
collection DOAJ
description Objective: The three recognized variants of primary progressive aphasia (PPA) are associated with different loci of degeneration—left posterior perisylvian in logopenic variant (lvPPA), left frontal operculum in non-fluent variant (nfvPPA), and left rostroventral-temporal in semantic variant (svPPA). Meanwhile, it has become apparent that patients with lvPPA, in which Alzheimer pathology is the norm, frequently have more extensive language deficits—namely semantic and grammatical problems—than is captured in the strict diagnostic recommendations for this variant. We hypothesized that this may be because the degeneration in AD-related PPA typically extends beyond the left posterior perisylvian region. Methods: Magnetic resonance images from 25 PPA patients (9AD-related PPA, 10 svPPA, 6 nfvPPA) and a healthy control cohort were used to calculate cortical thickness in three regions of interest (ROIs). The three ROIs being the left-hemispheric loci of maximal reported degeneration for each of the three variants of PPA. Results: Consistent with past studies, the most severe cortical thinning was in the posterior perisylvian ROI in AD-related PPA; the ventral temporal ROI in svPPA; and the frontal opercular ROI in nfvPPA. Significant cortical thinning in AD-related PPA, however, was evident in all three ROIs. In contrast, thinning in svPPA and nfvPPA was largely restricted to their known peak loci of degeneration. Conclusions: Although cortical degeneration in AD-related PPA is maximal in the left posterior perisylvian region, it extends more diffusely throughout the left hemisphere language network offering a plausible explanation for why the linguistic profile of lvPPA so often includes additional semantic and grammatic deficits. Keywords: PPA, Alzheimer pathology, Atrophy, AD-related PPA
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spelling doaj.art-3335a010eb054f329db2c7b0bd7ead802022-12-21T18:39:03ZengElsevierNeuroImage: Clinical2213-15822019-01-0124The atrophy pattern in Alzheimer-related PPA is more widespread than that of the frontotemporal lobar degeneration associated variantsDaniel Preiß0Ornella V. Billette1Anja Schneider2Nicola Spotorno3Peter J. Nestor4German Center for Neurodegenerative Diseases (DZNE), Ulm, GermanyGerman Center for Neurodegenerative Diseases (DZNE), Magdeburg, GermanyGerman Center for Neurodegenerative Diseases (DZNE), Bonn, GermanyPenn Frontotemporal Degeneration Center, University of Pennsylvania, Philadelphia, USAQueensland Brain Institute, University of Queensland, Brisbane, Australia; Mater Hospital, South Brisbane, Australia; Corresponding author at: Queensland Brain Institute, Building 79, University of Queensland, St Lucia, QLD 4072, Australia.Objective: The three recognized variants of primary progressive aphasia (PPA) are associated with different loci of degeneration—left posterior perisylvian in logopenic variant (lvPPA), left frontal operculum in non-fluent variant (nfvPPA), and left rostroventral-temporal in semantic variant (svPPA). Meanwhile, it has become apparent that patients with lvPPA, in which Alzheimer pathology is the norm, frequently have more extensive language deficits—namely semantic and grammatical problems—than is captured in the strict diagnostic recommendations for this variant. We hypothesized that this may be because the degeneration in AD-related PPA typically extends beyond the left posterior perisylvian region. Methods: Magnetic resonance images from 25 PPA patients (9AD-related PPA, 10 svPPA, 6 nfvPPA) and a healthy control cohort were used to calculate cortical thickness in three regions of interest (ROIs). The three ROIs being the left-hemispheric loci of maximal reported degeneration for each of the three variants of PPA. Results: Consistent with past studies, the most severe cortical thinning was in the posterior perisylvian ROI in AD-related PPA; the ventral temporal ROI in svPPA; and the frontal opercular ROI in nfvPPA. Significant cortical thinning in AD-related PPA, however, was evident in all three ROIs. In contrast, thinning in svPPA and nfvPPA was largely restricted to their known peak loci of degeneration. Conclusions: Although cortical degeneration in AD-related PPA is maximal in the left posterior perisylvian region, it extends more diffusely throughout the left hemisphere language network offering a plausible explanation for why the linguistic profile of lvPPA so often includes additional semantic and grammatic deficits. Keywords: PPA, Alzheimer pathology, Atrophy, AD-related PPAhttp://www.sciencedirect.com/science/article/pii/S2213158219303444
spellingShingle Daniel Preiß
Ornella V. Billette
Anja Schneider
Nicola Spotorno
Peter J. Nestor
The atrophy pattern in Alzheimer-related PPA is more widespread than that of the frontotemporal lobar degeneration associated variants
NeuroImage: Clinical
title The atrophy pattern in Alzheimer-related PPA is more widespread than that of the frontotemporal lobar degeneration associated variants
title_full The atrophy pattern in Alzheimer-related PPA is more widespread than that of the frontotemporal lobar degeneration associated variants
title_fullStr The atrophy pattern in Alzheimer-related PPA is more widespread than that of the frontotemporal lobar degeneration associated variants
title_full_unstemmed The atrophy pattern in Alzheimer-related PPA is more widespread than that of the frontotemporal lobar degeneration associated variants
title_short The atrophy pattern in Alzheimer-related PPA is more widespread than that of the frontotemporal lobar degeneration associated variants
title_sort atrophy pattern in alzheimer related ppa is more widespread than that of the frontotemporal lobar degeneration associated variants
url http://www.sciencedirect.com/science/article/pii/S2213158219303444
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