Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution?

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

Publication:
Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution?

dc.contributor.author	Sánchez Ruiz de Gordoa, Javier
dc.contributor.author	Zelaya Huerta, María Victoria
dc.contributor.author	Tellechea-Aramburo, Paula
dc.contributor.author	Acha Santamaría, Blanca
dc.contributor.author	Roldán, Miren
dc.contributor.author	López Molina, Carlos
dc.contributor.author	Coca, Valle
dc.contributor.author	Galbete Jiménez, Arkaitz
dc.contributor.author	Mendióroz Iriarte, Maite
dc.contributor.author	Erro Aguirre, María Elena
dc.contributor.department	Estadística, Informática y Matemáticas	es_ES
dc.contributor.department	Estatistika, Informatika eta Matematika	eu
dc.date.accessioned	2022-07-20T11:03:05Z
dc.date.available	2022-07-20T11:03:05Z
dc.date.issued	2022
dc.date.updated	2022-07-18T06:11:59Z
dc.description.abstract	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.	en
dc.format.extent	10 p.
dc.format.mimetype	application/pdf	en
dc.identifier.citation	Sánchez-Ruiz de Gordoa, J.; Zelaya, V.; Tellechea-Aramburo, P.; Acha-Santamaría, B.; Roldán, M.; López-Molina, C.; Coca, V.; Galbete-Jiménez, A.; Mendioroz, M.; Erro, M. E.. (2022). Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution?. Frontiers in Neurology. 13.	en
dc.identifier.doi	10.3389/fneur.2022.827338
dc.identifier.issn	1664-2295
dc.identifier.uri	https://academica-e.unavarra.es/handle/2454/43365
dc.language.iso	eng	en
dc.publisher	Frontiers Media	en
dc.relation.ispartof	Frontiers in Neurology, 2022, 13	en
dc.relation.publisherversion	https://doi.org/10.3389/fneur.2022.827338
dc.rights	https://creativecommons.org/licenses/by/4.0/
dc.rights.accessRights	Acceso abierto / Sarbide irekia	es
dc.rights.accessRights	info:eu-repo/semantics/openAccess	en
dc.subject	Clinical-pathological correlation	en
dc.subject	Phenotypes	en
dc.subject	Progressive supranuclear palsy (PSP)	en
dc.subject	PSP-MDS criteria	en
dc.subject	Tau protein load	en
dc.subject	Tauopathies	en
dc.title	Is the phenotype designation by PSP-MDS criteria stable throughout the disease course and consistent with tau distribution?	en
dc.type	info:eu-repo/semantics/article
dc.type.version	Versión publicada / Argitaratu den bertsioa	es
dc.type.version	info:eu-repo/semantics/publishedVersion	en
dspace.entity.type	Publication
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