Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution?
Introduction: the MDS-PSP criteria have shown high sensitivity for the PSP diagnosis, but do not discriminate the phenotype diversity. Our purpose was to search for anatomopathological differences among PSP phenotypes resulting from the application of the MDS-PSP criteria comparing with the previous...
| Autores: | , , , , , , , , , |
|---|---|
| Tipo de recurso: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2022 |
| País: | España |
| Institución: | Universidad Pública de Navarra |
| Repositorio: | Academica-e. Repositorio Institucional de la Universidad Pública de Navarra |
| OAI Identifier: | oai:academica-e.unavarra.es:2454/43365 |
| Acceso en línea: | https://hdl.handle.net/2454/43365 |
| Access Level: | acceso abierto |
| Palabra clave: | Clinical-pathological correlation Phenotypes Progressive supranuclear palsy (PSP) PSP-MDS criteria Tau protein load Tauopathies |
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Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution?Sánchez Ruiz de Gordoa, JavierZelaya Huerta, María VictoriaTellechea-Aramburo, PaulaAcha Santamaría, BlancaRoldán, MirenLópez Molina, CarlosCoca, ValleGalbete Jiménez, ArkaitzMendióroz Iriarte, MaiteErro Aguirre, María ElenaClinical-pathological correlationPhenotypesProgressive supranuclear palsy (PSP)PSP-MDS criteriaTau protein loadTauopathiesIntroduction: the MDS-PSP criteria have shown high sensitivity for the PSP diagnosis, but do not discriminate the phenotype diversity. Our purpose was to search for anatomopathological differences among PSP phenotypes resulting from the application of the MDS-PSP criteria comparing with the previous ones. Methods: thirty-four PSP cases from a single brain bank were retrospectively classified according to the criteria used by Respondek et al. in 2014 and the PSP-MDS criteria at 3 years (MDS-3y), 6 years (MDS-6y) and at the last clinical evaluation before death (MDS-last). Semiquantitative measurement of total, cortical and subcortical tau load was compared. For comparative analysis, PSP-Richardson syndrome and PSP postural instability were grouped (PSP-RS/PI) as well as the PSP atypical cortical phenotypes (PSP-Cx). Results: applying the Respondek's criteria, PSP phenotypes were distributed as follow: 55.9% PSP-RS/PI, 26.5% PSP-Cx, 11.8% PSP-Parkinsonism (PSP-P), and 5.9% PSP-Cerebellum. PSP-RS/PI and PSP-Cx had a higher total tau load than PSP-P; PSP-Cx showed a higher cortical tau load than PSP-RS/PI and PSP-P; and PSP-RS/PI had a higher subcortical tau load than PSP-P. Applying the MDS-3y, MDS-6y and MDS-last criteria; the PSP-RS/PI group increased (67.6, 70.6 and 70.6% respectively) whereas the PSP-Cx group decreased (8.8, and 8.8 and 11.8%). Then, only differences in total and subcortical tau burden between PSP-RS/PI and PSP-P were observed. Interpretation: after the retrospective application of the new MDS-PSP criteria, total and subcortical tau load is higher in PSP-RS/PI than in PSP-P whereas no other differences in tau load between phenotypes were found, as a consequence of the loss of phenotypic diversity.Frontiers MediaEstadística, Informática y MatemáticasEstatistika, Informatika eta Matematika2022info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://hdl.handle.net/2454/43365reponame:Academica-e. Repositorio Institucional de la Universidad Pública de Navarrainstname:Universidad Pública de NavarraIngléshttps://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:academica-e.unavarra.es:2454/433652026-06-17T12:41:47Z |
| dc.title.none.fl_str_mv |
Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution? |
| title |
Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution? |
| spellingShingle |
Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution? Sánchez Ruiz de Gordoa, Javier Clinical-pathological correlation Phenotypes Progressive supranuclear palsy (PSP) PSP-MDS criteria Tau protein load Tauopathies |
| title_short |
Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution? |
| title_full |
Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution? |
| title_fullStr |
Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution? |
| title_full_unstemmed |
Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution? |
| title_sort |
Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution? |
| dc.creator.none.fl_str_mv |
Sánchez Ruiz de Gordoa, Javier Zelaya Huerta, María Victoria Tellechea-Aramburo, Paula Acha Santamaría, Blanca Roldán, Miren López Molina, Carlos Coca, Valle Galbete Jiménez, Arkaitz Mendióroz Iriarte, Maite Erro Aguirre, María Elena |
| author |
Sánchez Ruiz de Gordoa, Javier |
| author_facet |
Sánchez Ruiz de Gordoa, Javier Zelaya Huerta, María Victoria Tellechea-Aramburo, Paula Acha Santamaría, Blanca Roldán, Miren López Molina, Carlos Coca, Valle Galbete Jiménez, Arkaitz Mendióroz Iriarte, Maite Erro Aguirre, María Elena |
| author_role |
author |
| author2 |
Zelaya Huerta, María Victoria Tellechea-Aramburo, Paula Acha Santamaría, Blanca Roldán, Miren López Molina, Carlos Coca, Valle Galbete Jiménez, Arkaitz Mendióroz Iriarte, Maite Erro Aguirre, María Elena |
| author2_role |
author author author author author author author author author |
| dc.contributor.none.fl_str_mv |
Estadística, Informática y Matemáticas Estatistika, Informatika eta Matematika |
| dc.subject.none.fl_str_mv |
Clinical-pathological correlation Phenotypes Progressive supranuclear palsy (PSP) PSP-MDS criteria Tau protein load Tauopathies |
| topic |
Clinical-pathological correlation Phenotypes Progressive supranuclear palsy (PSP) PSP-MDS criteria Tau protein load Tauopathies |
| description |
Introduction: the MDS-PSP criteria have shown high sensitivity for the PSP diagnosis, but do not discriminate the phenotype diversity. Our purpose was to search for anatomopathological differences among PSP phenotypes resulting from the application of the MDS-PSP criteria comparing with the previous ones. Methods: thirty-four PSP cases from a single brain bank were retrospectively classified according to the criteria used by Respondek et al. in 2014 and the PSP-MDS criteria at 3 years (MDS-3y), 6 years (MDS-6y) and at the last clinical evaluation before death (MDS-last). Semiquantitative measurement of total, cortical and subcortical tau load was compared. For comparative analysis, PSP-Richardson syndrome and PSP postural instability were grouped (PSP-RS/PI) as well as the PSP atypical cortical phenotypes (PSP-Cx). Results: applying the Respondek's criteria, PSP phenotypes were distributed as follow: 55.9% PSP-RS/PI, 26.5% PSP-Cx, 11.8% PSP-Parkinsonism (PSP-P), and 5.9% PSP-Cerebellum. PSP-RS/PI and PSP-Cx had a higher total tau load than PSP-P; PSP-Cx showed a higher cortical tau load than PSP-RS/PI and PSP-P; and PSP-RS/PI had a higher subcortical tau load than PSP-P. Applying the MDS-3y, MDS-6y and MDS-last criteria; the PSP-RS/PI group increased (67.6, 70.6 and 70.6% respectively) whereas the PSP-Cx group decreased (8.8, and 8.8 and 11.8%). Then, only differences in total and subcortical tau burden between PSP-RS/PI and PSP-P were observed. Interpretation: after the retrospective application of the new MDS-PSP criteria, total and subcortical tau load is higher in PSP-RS/PI than in PSP-P whereas no other differences in tau load between phenotypes were found, as a consequence of the loss of phenotypic diversity. |
| publishDate |
2022 |
| dc.date.none.fl_str_mv |
2022 |
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info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
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article |
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publishedVersion |
| dc.identifier.none.fl_str_mv |
https://hdl.handle.net/2454/43365 |
| url |
https://hdl.handle.net/2454/43365 |
| dc.language.none.fl_str_mv |
Inglés |
| language_invalid_str_mv |
Inglés |
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https://creativecommons.org/licenses/by/4.0/ info:eu-repo/semantics/openAccess |
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https://creativecommons.org/licenses/by/4.0/ |
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openAccess |
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application/pdf |
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Frontiers Media |
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Frontiers Media |
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reponame:Academica-e. Repositorio Institucional de la Universidad Pública de Navarra instname:Universidad Pública de Navarra |
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Universidad Pública de Navarra |
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Academica-e. Repositorio Institucional de la Universidad Pública de Navarra |
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Academica-e. Repositorio Institucional de la Universidad Pública de Navarra |
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