Clinical dimensions along the non-fluent variant primary progressive aphasia spectrum

Apraxia of speech; Corticobasal degeneration; Primary progressive aphasia

Detalles Bibliográficos
Autores: Illán-Gala, Ignacio, Lorca-Puls, Diego L., Tee, Boon Lead, Ezzes, Zoe, de Leon, Jessica, Miller, Zachary A, Gómez-Andrés, David
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2023
País:España
Institución:Departament de Salut de la Generalitat de Catalunya (DS)
Repositorio:Scientia. Dipòsit d'Informació Digital del Departament de Salut
OAI Identifier:oai:scientiasalut.gencat.cat:11351/11313
Acceso en línea:https://hdl.handle.net/11351/11313
Access Level:acceso abierto
Palabra clave:Afàsia
Trastorns de l'articulació
Trastorns de la parla
DISEASES::Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Dementia::Aphasia, Primary Progressive
DISEASES::Nervous System Diseases::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech Disorders::Articulation Disorders::Dysarthria
DISEASES::Nervous System Diseases::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech Disorders
ENFERMEDADES::enfermedades del sistema nervioso::enfermedades del sistema nervioso central::enfermedades cerebrales::demencia::afasia primaria progresiva
ENFERMEDADES::enfermedades del sistema nervioso::manifestaciones neurológicas::manifestaciones neuroconductuales::trastornos de la comunicación::trastornos del lenguaje::trastornos del habla::trastornos de la articulación del habla::disartria
ENFERMEDADES::enfermedades del sistema nervioso::manifestaciones neurológicas::manifestaciones neuroconductuales::trastornos de la comunicación::trastornos del lenguaje::trastornos del habla
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network_name_str España
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dc.title.none.fl_str_mv Clinical dimensions along the non-fluent variant primary progressive aphasia spectrum
title Clinical dimensions along the non-fluent variant primary progressive aphasia spectrum
spellingShingle Clinical dimensions along the non-fluent variant primary progressive aphasia spectrum
Illán-Gala, Ignacio
Afàsia
Trastorns de l'articulació
Trastorns de la parla
DISEASES::Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Dementia::Aphasia, Primary Progressive
DISEASES::Nervous System Diseases::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech Disorders::Articulation Disorders::Dysarthria
DISEASES::Nervous System Diseases::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech Disorders
ENFERMEDADES::enfermedades del sistema nervioso::enfermedades del sistema nervioso central::enfermedades cerebrales::demencia::afasia primaria progresiva
ENFERMEDADES::enfermedades del sistema nervioso::manifestaciones neurológicas::manifestaciones neuroconductuales::trastornos de la comunicación::trastornos del lenguaje::trastornos del habla::trastornos de la articulación del habla::disartria
ENFERMEDADES::enfermedades del sistema nervioso::manifestaciones neurológicas::manifestaciones neuroconductuales::trastornos de la comunicación::trastornos del lenguaje::trastornos del habla
title_short Clinical dimensions along the non-fluent variant primary progressive aphasia spectrum
title_full Clinical dimensions along the non-fluent variant primary progressive aphasia spectrum
title_fullStr Clinical dimensions along the non-fluent variant primary progressive aphasia spectrum
title_full_unstemmed Clinical dimensions along the non-fluent variant primary progressive aphasia spectrum
title_sort Clinical dimensions along the non-fluent variant primary progressive aphasia spectrum
dc.creator.none.fl_str_mv Illán-Gala, Ignacio
Lorca-Puls, Diego L.
Tee, Boon Lead
Ezzes, Zoe
de Leon, Jessica
Miller, Zachary A
Gómez-Andrés, David
author Illán-Gala, Ignacio
author_facet Illán-Gala, Ignacio
Lorca-Puls, Diego L.
Tee, Boon Lead
Ezzes, Zoe
de Leon, Jessica
Miller, Zachary A
Gómez-Andrés, David
author_role author
author2 Lorca-Puls, Diego L.
Tee, Boon Lead
Ezzes, Zoe
de Leon, Jessica
Miller, Zachary A
Gómez-Andrés, David
author2_role author
author
author
author
author
author
dc.contributor.none.fl_str_mv Institut Català de la Salut
[Illán-Gala I] Sant Pau Memory Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain. Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain. Global Brain Health Institute, University of California, San Francisco, CA, USA. [Lorca-Puls DL] Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA, USA. Sección de Neurología, Departamento de Especialidades, Facultad de Medicina, Universidad de Concepción, Concepción, Chile. [Tee BL, Ezzes Z, de Leon J, Miller ZA] Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA, USA. [Gómez-Andrés D] Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Vall d’Hebron Hospital Universitari, Barcelona, Spain
Vall d'Hebron Barcelona Hospital Campus
dc.subject.none.fl_str_mv Afàsia
Trastorns de l'articulació
Trastorns de la parla
DISEASES::Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Dementia::Aphasia, Primary Progressive
DISEASES::Nervous System Diseases::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech Disorders::Articulation Disorders::Dysarthria
DISEASES::Nervous System Diseases::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech Disorders
ENFERMEDADES::enfermedades del sistema nervioso::enfermedades del sistema nervioso central::enfermedades cerebrales::demencia::afasia primaria progresiva
ENFERMEDADES::enfermedades del sistema nervioso::manifestaciones neurológicas::manifestaciones neuroconductuales::trastornos de la comunicación::trastornos del lenguaje::trastornos del habla::trastornos de la articulación del habla::disartria
ENFERMEDADES::enfermedades del sistema nervioso::manifestaciones neurológicas::manifestaciones neuroconductuales::trastornos de la comunicación::trastornos del lenguaje::trastornos del habla
topic Afàsia
Trastorns de l'articulació
Trastorns de la parla
DISEASES::Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Dementia::Aphasia, Primary Progressive
DISEASES::Nervous System Diseases::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech Disorders::Articulation Disorders::Dysarthria
DISEASES::Nervous System Diseases::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech Disorders
ENFERMEDADES::enfermedades del sistema nervioso::enfermedades del sistema nervioso central::enfermedades cerebrales::demencia::afasia primaria progresiva
ENFERMEDADES::enfermedades del sistema nervioso::manifestaciones neurológicas::manifestaciones neuroconductuales::trastornos de la comunicación::trastornos del lenguaje::trastornos del habla::trastornos de la articulación del habla::disartria
ENFERMEDADES::enfermedades del sistema nervioso::manifestaciones neurológicas::manifestaciones neuroconductuales::trastornos de la comunicación::trastornos del lenguaje::trastornos del habla
description Apraxia of speech; Corticobasal degeneration; Primary progressive aphasia
publishDate 2023
dc.date.none.fl_str_mv 2023
2024
2024
2024
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://hdl.handle.net/11351/11313
url https://hdl.handle.net/11351/11313
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Brain;147(4)
https://doi.org/10.1093/brain/awad396
dc.rights.none.fl_str_mv Attribution-NonCommercial 4.0 International
http://creativecommons.org/licenses/by-nc/4.0/
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Attribution-NonCommercial 4.0 International
http://creativecommons.org/licenses/by-nc/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Oxford University Press
publisher.none.fl_str_mv Oxford University Press
dc.source.none.fl_str_mv Scientia
reponame:Scientia. Dipòsit d'Informació Digital del Departament de Salut
instname:Departament de Salut de la Generalitat de Catalunya (DS)
instname_str Departament de Salut de la Generalitat de Catalunya (DS)
reponame_str Scientia. Dipòsit d'Informació Digital del Departament de Salut
collection Scientia. Dipòsit d'Informació Digital del Departament de Salut
repository.name.fl_str_mv
repository.mail.fl_str_mv
_version_ 1869414868707180544
spelling Clinical dimensions along the non-fluent variant primary progressive aphasia spectrumIllán-Gala, IgnacioLorca-Puls, Diego L.Tee, Boon LeadEzzes, Zoede Leon, JessicaMiller, Zachary AGómez-Andrés, DavidAfàsiaTrastorns de l'articulacióTrastorns de la parlaDISEASES::Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Dementia::Aphasia, Primary ProgressiveDISEASES::Nervous System Diseases::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech Disorders::Articulation Disorders::DysarthriaDISEASES::Nervous System Diseases::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech DisordersENFERMEDADES::enfermedades del sistema nervioso::enfermedades del sistema nervioso central::enfermedades cerebrales::demencia::afasia primaria progresivaENFERMEDADES::enfermedades del sistema nervioso::manifestaciones neurológicas::manifestaciones neuroconductuales::trastornos de la comunicación::trastornos del lenguaje::trastornos del habla::trastornos de la articulación del habla::disartriaENFERMEDADES::enfermedades del sistema nervioso::manifestaciones neurológicas::manifestaciones neuroconductuales::trastornos de la comunicación::trastornos del lenguaje::trastornos del hablaApraxia of speech; Corticobasal degeneration; Primary progressive aphasiaApraxia del habla; Degeneración corticobasal; Afasia primaria progresivaApràxia de la parla; Degeneració corticobasal; Afàsia primària progressivaIt is debated whether primary progressive apraxia of speech (PPAOS) and progressive agrammatic aphasia (PAA) belong to the same clinical spectrum, traditionally termed non-fluent/agrammatic variant primary progressive aphasia (nfvPPA), or exist as two completely distinct syndromic entities with specific pathologic/prognostic correlates. We analysed speech, language and disease severity features in a comprehensive cohort of patients with progressive motor speech impairment and/or agrammatism to ascertain evidence of naturally occurring, clinically meaningful non-overlapping syndromic entities (e.g. PPAOS and PAA) in our data. We also assessed if data-driven latent clinical dimensions with aetiologic/prognostic value could be identified. We included 98 participants, 43 of whom had an autopsy-confirmed neuropathological diagnosis. Speech pathologists assessed motor speech features indicative of dysarthria and apraxia of speech (AOS). Quantitative expressive/receptive agrammatism measures were obtained and compared with healthy controls. Baseline and longitudinal disease severity was evaluated using the Clinical Dementia Rating Sum of Boxes (CDR-SB). We investigated the data’s clustering tendency and cluster stability to form robust symptom clusters and employed principal component analysis to extract data-driven latent clinical dimensions (LCD). The longitudinal CDR-SB change was estimated using linear mixed-effects models. Of the participants included in this study, 93 conformed to previously reported clinical profiles (75 with AOS and agrammatism, 12 PPAOS and six PAA). The remaining five participants were characterized by non-fluent speech, executive dysfunction and dysarthria without apraxia of speech or frank agrammatism. No baseline clinical features differentiated between frontotemporal lobar degeneration neuropathological subgroups. The Hopkins statistic demonstrated a low cluster tendency in the entire sample (0.45 with values near 0.5 indicating random data). Cluster stability analyses showed that only two robust subgroups (differing in agrammatism, executive dysfunction and overall disease severity) could be identified. Three data-driven components accounted for 71% of the variance [(i) severity-agrammatism; (ii) prominent AOS; and (iii) prominent dysarthria]. None of these data-driven LCDs allowed an accurate prediction of neuropathology. The severity-agrammatism component was an independent predictor of a faster CDR-SB increase in all the participants. Higher dysarthria severity, reduced words per minute and expressive and receptive agrammatism severity at baseline independently predicted accelerated disease progression. Our findings indicate that PPAOS and PAA, rather than exist as completely distinct syndromic entities, constitute a clinical continuum. In our cohort, splitting the nfvPPA spectrum into separate clinical phenotypes did not improve clinical-pathological correlations, stressing the need for new biological markers and consensus regarding updated terminology and clinical classification.I.I.-G. is a senior Atlantic Fellow for Equity in Brain Health at the Global Brain Health Institute (GBHI), and receives funding from the GBHI, the Alzheimer’s Association, and the Alzheimer Society (GBHI ALZ UK-21-720973 and AACSF-21-850193). I.I.-G. was also supported by the Juan Rodés Contract (JR20/0018) and PI21/00791 from Instituto de Salud Carlos III. D.L.L-P. is supported with funding from the Chilean National Agency for Research and Development (ANID SUBVENCIÓN A LA INSTALACIÓN EN LA ACADEMIA 85220006). D.L.L-P. was also supported by a postdoctoral fellowship from the Chilean National Agency for Research and Development (ANID BECAS-CHILE 74200073). This work was supported by the National Institutes of Health (L.T.G., NIA K24 AG053435; S.S., K08AG052648; M.L.H., NIDCD R01DC016291; A.L.B., NS092089, R01AG038791, U19AG063911; H.J.R., AG045333, AG056749, AG032306, AG045390; B.L.M., NIA P50 AG023501, NIA P01 AG019724; M.L.G-T., NINDS R01 NS050915, NIDCD K24 DC015544, NIA U01 AG052943). The UCSF Neurodegenerative Disease Brain Bank receives funding support from NIH grants P30 AG062422, P01 AG019724, U01 AG057195, and U19 AG063911, as well as the Rainwater Charitable Foundation and the Bluefield Project to Cure FTD.Oxford University PressInstitut Català de la Salut[Illán-Gala I] Sant Pau Memory Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain. Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain. Global Brain Health Institute, University of California, San Francisco, CA, USA. [Lorca-Puls DL] Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA, USA. Sección de Neurología, Departamento de Especialidades, Facultad de Medicina, Universidad de Concepción, Concepción, Chile. [Tee BL, Ezzes Z, de Leon J, Miller ZA] Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California, San Francisco, CA, USA. [Gómez-Andrés D] Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Vall d’Hebron Hospital Universitari, Barcelona, SpainVall d'Hebron Barcelona Hospital Campus2024202420232024info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://hdl.handle.net/11351/11313Scientiareponame:Scientia. Dipòsit d'Informació Digital del Departament de Salutinstname:Departament de Salut de la Generalitat de Catalunya (DS)InglésBrain;147(4)https://doi.org/10.1093/brain/awad396Attribution-NonCommercial 4.0 Internationalhttp://creativecommons.org/licenses/by-nc/4.0/info:eu-repo/semantics/openAccessoai:scientiasalut.gencat.cat:11351/113132026-06-12T09:38:37Z
score 15.811543

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