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Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100016608","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,4,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>It 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.<\/jats:p>\n                  <jats:p>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\u2019s 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.<\/jats:p>\n                  <jats:p>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.<\/jats:p>","DOI":"10.1093\/brain\/awad396","type":"journal-article","created":{"date-parts":[[2023,11,20]],"date-time":"2023-11-20T17:48:39Z","timestamp":1700502519000},"page":"1511-1525","source":"Crossref","is-referenced-by-count":17,"title":["Clinical dimensions along the non-fluent variant primary progressive aphasia spectrum"],"prefix":"10.1093","volume":"147","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5418-2052","authenticated-orcid":false,"given":"Ignacio","family":"Ill\u00e1n-Gala","sequence":"first","affiliation":[{"name":"Sant Pau Memory Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Aut\u00f2noma de Barcelona , 08025, Barcelona , Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Neurodegenerativas (CIBERNED) , Madrid, 28029 , Spain"},{"name":"Global Brain Health Institute, University of California , San Francisco, CA 94143 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diego L","family":"Lorca-Puls","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"},{"name":"Secci\u00f3n de Neurolog\u00eda, Departamento de Especialidades, Facultad de Medicina, Universidad de Concepci\u00f3n , Concepci\u00f3n, 4070001 , Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2217-3466","authenticated-orcid":false,"given":"Boon Lead","family":"Tee","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zoe","family":"Ezzes","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"de Leon","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zachary A","family":"Miller","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Rubio-Guerra","sequence":"additional","affiliation":[{"name":"Sant Pau Memory Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Aut\u00f2noma de Barcelona , 08025, Barcelona , Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel","family":"Santos-Santos","sequence":"additional","affiliation":[{"name":"Sant Pau Memory Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Aut\u00f2noma de Barcelona , 08025, Barcelona , Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"G\u00f3mez-Andr\u00e9s","sequence":"additional","affiliation":[{"name":"Vall d'Hebron Institut de Recerca (VHIR), Hospital Universitari Vall d'Hebron , 08035, Barcelona , Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6809-0618","authenticated-orcid":false,"given":"Lea T","family":"Grinberg","sequence":"additional","affiliation":[{"name":"Global Brain Health Institute, University of California , San Francisco, CA 94143 , USA"},{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"},{"name":"Department of Pathology, University of California San Francisco , San Francisco, CA 94143 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3570-9143","authenticated-orcid":false,"given":"Salvatore","family":"Spina","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joel H","family":"Kramer","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa D","family":"Wauters","sequence":"additional","affiliation":[{"name":"Department of Communication Sciences and Disorders, University of Texas , Austin, TX 78712-0114 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maya L","family":"Henry","sequence":"additional","affiliation":[{"name":"Department of Communication Sciences and Disorders, University of Texas , Austin, TX 78712-0114 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam L","family":"Boxer","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Howard J","family":"Rosen","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5991-3053","authenticated-orcid":false,"given":"Bruce L","family":"Miller","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William W","family":"Seeley","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Luisa","family":"Mandelli","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Luisa","family":"Gorno-Tempini","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,11,21]]},"reference":[{"key":"2024040418375085900_awad396-B1","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1097\/WCO.0000000000000673","article-title":"Primary progressive aphasia: A model for neurodegenerative 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