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Neuroanatomical and cellular degeneration associated with a social disorder characterized by new ritualistic belief systems in a TDP-C patient vs. a Pick patient
Frontotemporal dementia (FTD) is a spectrum of clinically and pathologically heterogenous neurodegenerative dementias. Clinical and anatomical variants of FTD have been described and associated with underlying frontotemporal lobar degeneration (FTLD) pathology, including tauopathies (FTLD-tau) or TD...
Autores principales: | , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10600461/ https://www.ncbi.nlm.nih.gov/pubmed/37900607 http://dx.doi.org/10.3389/fneur.2023.1245886 |
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author | Ohm, Daniel T. Rhodes, Emma Bahena, Alejandra Capp, Noah Lowe, MaKayla Sabatini, Philip Trotman, Winifred Olm, Christopher A. Phillips, Jeffrey Prabhakaran, Karthik Rascovsky, Katya Massimo, Lauren McMillan, Corey Gee, James Tisdall, M. Dylan Yushkevich, Paul A. Lee, Edward B. Grossman, Murray Irwin, David J. |
author_facet | Ohm, Daniel T. Rhodes, Emma Bahena, Alejandra Capp, Noah Lowe, MaKayla Sabatini, Philip Trotman, Winifred Olm, Christopher A. Phillips, Jeffrey Prabhakaran, Karthik Rascovsky, Katya Massimo, Lauren McMillan, Corey Gee, James Tisdall, M. Dylan Yushkevich, Paul A. Lee, Edward B. Grossman, Murray Irwin, David J. |
author_sort | Ohm, Daniel T. |
collection | PubMed |
description | Frontotemporal dementia (FTD) is a spectrum of clinically and pathologically heterogenous neurodegenerative dementias. Clinical and anatomical variants of FTD have been described and associated with underlying frontotemporal lobar degeneration (FTLD) pathology, including tauopathies (FTLD-tau) or TDP-43 proteinopathies (FTLD-TDP). FTD patients with predominant degeneration of anterior temporal cortices often develop a language disorder of semantic knowledge loss and/or a social disorder often characterized by compulsive rituals and belief systems corresponding to predominant left or right hemisphere involvement, respectively. The neural substrates of these complex social disorders remain unclear. Here, we present a comparative imaging and postmortem study of two patients, one with FTLD-TDP (subtype C) and one with FTLD-tau (subtype Pick disease), who both developed new rigid belief systems. The FTLD-TDP patient developed a complex set of values centered on positivity and associated with specific physical and behavioral features of pigs, while the FTLD-tau patient developed compulsive, goal-directed behaviors related to general themes of positivity and spirituality. Neuroimaging showed left-predominant temporal atrophy in the FTLD-TDP patient and right-predominant frontotemporal atrophy in the FTLD-tau patient. Consistent with antemortem cortical atrophy, histopathologic examinations revealed severe loss of neurons and myelin predominantly in the anterior temporal lobes of both patients, but the FTLD-tau patient showed more bilateral, dorsolateral involvement featuring greater pathology and loss of projection neurons and deep white matter. These findings highlight that the regions within and connected to anterior temporal lobes may have differential vulnerability to distinct FTLD proteinopathies and serve important roles in human belief systems. |
format | Online Article Text |
id | pubmed-10600461 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-106004612023-10-27 Neuroanatomical and cellular degeneration associated with a social disorder characterized by new ritualistic belief systems in a TDP-C patient vs. a Pick patient Ohm, Daniel T. Rhodes, Emma Bahena, Alejandra Capp, Noah Lowe, MaKayla Sabatini, Philip Trotman, Winifred Olm, Christopher A. Phillips, Jeffrey Prabhakaran, Karthik Rascovsky, Katya Massimo, Lauren McMillan, Corey Gee, James Tisdall, M. Dylan Yushkevich, Paul A. Lee, Edward B. Grossman, Murray Irwin, David J. Front Neurol Neurology Frontotemporal dementia (FTD) is a spectrum of clinically and pathologically heterogenous neurodegenerative dementias. Clinical and anatomical variants of FTD have been described and associated with underlying frontotemporal lobar degeneration (FTLD) pathology, including tauopathies (FTLD-tau) or TDP-43 proteinopathies (FTLD-TDP). FTD patients with predominant degeneration of anterior temporal cortices often develop a language disorder of semantic knowledge loss and/or a social disorder often characterized by compulsive rituals and belief systems corresponding to predominant left or right hemisphere involvement, respectively. The neural substrates of these complex social disorders remain unclear. Here, we present a comparative imaging and postmortem study of two patients, one with FTLD-TDP (subtype C) and one with FTLD-tau (subtype Pick disease), who both developed new rigid belief systems. The FTLD-TDP patient developed a complex set of values centered on positivity and associated with specific physical and behavioral features of pigs, while the FTLD-tau patient developed compulsive, goal-directed behaviors related to general themes of positivity and spirituality. Neuroimaging showed left-predominant temporal atrophy in the FTLD-TDP patient and right-predominant frontotemporal atrophy in the FTLD-tau patient. Consistent with antemortem cortical atrophy, histopathologic examinations revealed severe loss of neurons and myelin predominantly in the anterior temporal lobes of both patients, but the FTLD-tau patient showed more bilateral, dorsolateral involvement featuring greater pathology and loss of projection neurons and deep white matter. These findings highlight that the regions within and connected to anterior temporal lobes may have differential vulnerability to distinct FTLD proteinopathies and serve important roles in human belief systems. Frontiers Media S.A. 2023-10-11 /pmc/articles/PMC10600461/ /pubmed/37900607 http://dx.doi.org/10.3389/fneur.2023.1245886 Text en Copyright © 2023 Ohm, Rhodes, Bahena, Capp, Lowe, Sabatini, Trotman, Olm, Phillips, Prabhakaran, Rascovsky, Massimo, McMillan, Gee, Tisdall, Yushkevich, Lee, Grossman and Irwin. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Neurology Ohm, Daniel T. Rhodes, Emma Bahena, Alejandra Capp, Noah Lowe, MaKayla Sabatini, Philip Trotman, Winifred Olm, Christopher A. Phillips, Jeffrey Prabhakaran, Karthik Rascovsky, Katya Massimo, Lauren McMillan, Corey Gee, James Tisdall, M. Dylan Yushkevich, Paul A. Lee, Edward B. Grossman, Murray Irwin, David J. Neuroanatomical and cellular degeneration associated with a social disorder characterized by new ritualistic belief systems in a TDP-C patient vs. a Pick patient |
title | Neuroanatomical and cellular degeneration associated with a social disorder characterized by new ritualistic belief systems in a TDP-C patient vs. a Pick patient |
title_full | Neuroanatomical and cellular degeneration associated with a social disorder characterized by new ritualistic belief systems in a TDP-C patient vs. a Pick patient |
title_fullStr | Neuroanatomical and cellular degeneration associated with a social disorder characterized by new ritualistic belief systems in a TDP-C patient vs. a Pick patient |
title_full_unstemmed | Neuroanatomical and cellular degeneration associated with a social disorder characterized by new ritualistic belief systems in a TDP-C patient vs. a Pick patient |
title_short | Neuroanatomical and cellular degeneration associated with a social disorder characterized by new ritualistic belief systems in a TDP-C patient vs. a Pick patient |
title_sort | neuroanatomical and cellular degeneration associated with a social disorder characterized by new ritualistic belief systems in a tdp-c patient vs. a pick patient |
topic | Neurology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10600461/ https://www.ncbi.nlm.nih.gov/pubmed/37900607 http://dx.doi.org/10.3389/fneur.2023.1245886 |
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