Uniform data set language measures for bvFTD and PPA diagnosis and monitoring
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Introduction: The Frontotemporal Lobar Degeneration Module (FTLD‐MOD) includes a neuropsychological battery designed to assess the clinical features of FTLD, although much is unknown about its utility. We investigated FTLD‐MOD and Uniform Data Set 3.0 (UDS) language tests for differential diagnosis and disease monitoring. Methods: Linear regressions compared baseline performances in 1655 National Alzheimer's Coordinating Center participants (behavioral variant frontotemporal dementia (bvFTD, n = 612), semantic variant primary progressive aphasia (svPPA, n = 185), non‐fluent/agrammatic variant PPA (nfvPPA, n = 168), logopenic variant PPA (lvPPA, n = 109), and controls (n = 581)). Sample sizes to detect treatment effects were estimated using longitudinal data. Results: Among PPAs, the FTLD‐MOD language tasks and UDS Multilingual Naming Test accurately discriminated svPPA. Number Span Forward best discriminated lvPPA; Phonemic:Semantic Fluency ratio was excellent for nfvPPA classification. UDS fluency and naming measures required the smallest sample size to detect meaningful change. Discussion: The FTLD‐MOD and UDS differentiated among PPA subtypes. UDS 3.0 measures performed best for longitudinal monitoring.
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Irregular word reading, word-picture matching, and semantic association are more impaired in people with semantic variant primary progressive aphasia than in those with non-fluent primary progressive aphasia.
“Irregular word reading, word-picture matching, and semantic association are more impaired in people with semantic variant primary progressive aphasia than in those with non-fluent primary progressive aphasia.43”
From Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations (2022) · cites this paper as [43]
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“Staffaroni et al. (2021) found that naming and word comprehension in the behavioral variant ( N ¼ 612) were worse than in healthy controls ( N ¼ 581).”
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“…dysfunction in FTD is often used to differentiate subtypes but is typically assessed using clinician-administered assessments of language abilities (15, 23), with some recent reports using natural language processing to detect changes to linguistic and acoustic patterns in speech in FTD subtypes…”
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“Picture stimuli were black-and-white line drawings developed on the basis of prior studies including sets of standardized pictures [51, 66, 67].”
MethodDecision making in frontotemporal dementia: neuropsychological and neuroimaging assessments
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“Finally, the Uniformed Data Set-Frontotemporal Lobar degeneration (UDS-FTLD) battery represents an informative clinical tool that discriminates between bvFTD and PPA variants (svPPA, nfvPPA, logopenic PPA; Staffaroni et al., 2021).”
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“Staffaroni et al. (2021) found that naming and word-to-picture matching were worse in semantic dementia ( N ¼ 185) than in bvFTD ( N ¼ 612), and performance in both patient groups was worse than in healthy controls ( N ¼ 581).”
BackgroundResultEnhanced Imaging and Language Assessments for Primary Progressive Aphasia
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“In a study of 6,155 NACC participants, which included 185 participants with svPPA, 168 with nfaPPA, and 109 with lvPPA, Staffaroni et al.12 found that sentence repetition was the best subtest of the FTLD-MOD for discriminating lvPPA from other variants.”
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“5 Regarding the neuropsychological signature, early lvPPA-AD is characterized by a decline in language fluency, 36,43 which is one of the neuropsychological complaints of patients with mTBI as well.”
BackgroundČihák, M., Horáková, H., Vyhnálek, M., Veverová, K., Matušková, V., Laczó, J., Hort, J., & Nikolai, T. (2024) · Archives of Clinical Neuropsychology · doi:10.1093/arclin/acae028 · Cited by 2 · see citing works
Auditory verb generation performance patterns dissociate variants of primary progressive aphasia.Preprint
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Self-citationClinical dimensions along the progressive nonfluent variant primary progressive aphasia spectrumPreprint
Illán‐Gala, I., Lorca‐Puls, D. L., Ezzes, Z., Deleon, J., Miller, Z., Rubio‐Guerra, S., Santos‐Santos, M., Gómez‐Andrés, D., Grinberg, L. T., Spina, S., et al. (2023) · medRxiv · doi:10.1101/2023.04.18.23288702 · Cited by 1 · see citing works
“Such stratification may reduce the heterogeneity of participants in future trials and reduce sample size requirements.(53)”
BackgroundSelf-citationLanguage Impairment in the Genetic Forms of Behavioural Variant Frontotemporal DementiaPreprint
Samra, K., MacDougall, A. M., Bouzigues, A., Bocchetta, M., Cash, D. M., Greaves, C. V., Convery, R. S., Swieten, J. C. V., Jiskoot, L. C., Moreno, F., et al. (2022) · Research Square · doi:10.21203/rs.3.rs-1416934/v1
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“These syndromes include semantic variant primary progressive aphasia (svPPA) by definition, as well as Alzheimer ’ s dementia, and traumatic brain injury (Gorno-Tempini et al., 2011; McGeown et al., 2009; McWilliams & Schmitter-Edgecombe, 2008; Staffaroni et al., 2021).”
Self-citationLanguage in behavioral variant frontotemporal dementia
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Multiple types of dyslexia in primary progressive aphasia
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Savage, S. A., Aung, O., Foxe, D., & Piguet, O. (2026) · Journal of Neurology · doi:10.1007/s00415-026-14064-w
- Authors
- Staffaroni, A. M., Weintraub, S., Rascovsky, K., Rankin, K. P., Taylor, J., Fields, J. A., Casaletto, K. B., Hillis, A. E., Lukic, S., Gorno‐Tempini, M. L., Heuer, H., Teylan, M. A., Kukull, W. A., Miller, B. L., Boeve, B. F., Rosen, H. J., Boxer, A. L., & Kramer, J. H.
- Affiliations
- University of California, San Francisco (US)
- Mesulam Center for Cognitive Neurology and Alzheimer's Disease and Department of Psychiatry and Behavioral Sciences Feinberg School of Medicine Department of Neurology Northwestern University Chicago Illinois USA
- University of Pennsylvania (US)
- Mayo Clinic (US)
- Johns Hopkins University (US)
- University of Washington (US)
- Corresponding author
- Staffaroni, Adam M.
- ORCID
- Venue
- Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring
- Publisher
- Wiley
- Published
- 2021-01
- Volume
- 13
- Issue
- 1
- Pages
- e12148-e12148
- ISSN
- 2352-8729 (ISSN-L)
- PMC
- PMC7896637
- OpenAlex
- W3131330772
- Cited by
- 33
- References
- 51
- Citations by year
- 2026: 3 · 2025: 3 · 2024: 5 · 2023: 9 · 2022: 9 · 2021: 4
- FWCI
- 4.84
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- Alzheimer s & Dementia Diagnosis Assessment & Disease Monitoring (publishedVersion, cc-by-nc-nd)
- PubMed Central (publishedVersion, cc-by-nc-nd)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion, cc-by-nc-nd)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- eScholarship (California Digital Library) (submittedVersion)
- Funders
- Larry L. Hillblom Foundation (2018‐A‐0‐FEL, 2017‐A‐004‐FEL)
- National Science Foundation (P30 AG019610, P30 AG013846, P30 AG062428‐01, P50 AG008702, P50 AG025688, P50 AG047266, P30 AG010133, P50 AG005146, P30 AG062421‐01, P30 AG062422‐01, P50 AG005138, P30 AG008051, P30 AG013854, P30 AG008017, P30 AG010161, P50 AG047366, P30 AG010129, P50 AG016573, P30 AG062429‐01, P50 AG023501, P30 AG035982, P30 AG028383, P30 AG053760, P30 AG010124, P50 AG005133, P50 AG005142, P30 AG012300, P30 AG049638, P50 AG005136, P30 AG062715‐01, P50 AG005681, P50 AG047270)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Social Sciences › Psychology › Clinical Psychology › Historical and Modern Theater Studies
- Life Sciences › Neuroscience › Cognitive Neuroscience › Neurobiology of Language and Bilingualism
- Keywords
- frontotemporal lobar degeneration, behavioral variant frontotemporal dementia, primary progressive aphasia, semantic variant PPA, logopenic variant PPA, language assessment, differential diagnosis, disease surveillance, sample size determination, naming
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