Abstract
ObjectiveTo understand whether the clinical phenotype of nonfluent/agrammatic primary progressive aphasia (nfvPPA) could present differences depending on the patient's native language.MethodsIn this cross-sectional study, we analyzed connected speech samples in monolingual English (nfvPPA-E) and Italian speakers (nfvPPA-I) who were diagnosed with nfvPPA and matched for age, sex, and Mini-Mental State Examination scores. Patients also received a comprehensive neuropsychological battery. All patients and 2 groups of age-matched healthy controls underwent an MRI scan with 3D T1-weighted sequences. Connected speech measures and the other cognitive features were compared between patient groups. MRI variables, in terms of gray matter volume, were compared between each patient group and the corresponding controls.ResultsCompared to nfvPPA-E, nfvPPA-I had fewer years of education and shorter reported disease duration. The 2 groups showed similar regional atrophy compatible with clinical diagnosis. Patients did not differ in nonlanguage domains, comprising executive scores. Connected speech sample analysis showed that nfvPPA-E had significantly more distortions than nfvPPA-I, while nfvPPA-I showed reduced scores in some measures of syntactic complexity. On language measures, Italian speakers performed more poorly on syntactic comprehension.ConclusionsnfvPPA-E showed greater motor speech impairment than nfvPPA-I despite higher level of education and comparable disease severity and atrophy changes. The data also suggest greater grammatical impairment in nfvPPA-I. This study illustrates the need to take into account the possible effect of the individual's spoken language on the phenotype and clinical presentation of primary progressive aphasia variants.
Original language | English |
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Pages (from-to) | e1062-e1072 |
Journal | Neurology |
Volume | 94 |
Issue number | 10 |
DOIs | |
State | Published - Mar 10 2020 |
Bibliographical note
Publisher Copyright:© American Academy of Neurology.
Funding
H.I. Hubbard, A. Moro, and G. Magnani report no disclosures relevant to the manuscript. S.F. Cappa is Section Editor of Cortex; has received compensation for consulting services and/or speaking activities from Biogen, Roche, Eli-Lilly, and Nutricia; and receives research support from the Italian Ministry of Health and Medical Research Council. B. Miller receives grants in support of the Memory and Aging Center from the NIH/NIA, the Quest Diagnostics Dementia Pathway Collaboration, Cornell University, and The Bluefield Project to Cure Frontotemporal Dementia; and serves as Medical Director for the John Douglas French Foundation, Scientific Director for the Tau Consortium, Director/Medical Advisory Board of the Larry L. Hillblom Foundation, and Past President of the International Society of Frontotemporal Dementia (ISFTD). M. Filippi is Editor-in-Chief of the Journal of Neurology; received compensation for consulting services and/or speaking activities from Biogen Idec, Merck-Serono, Novartis, and Teva Pharmaceutical Industries; and receives research support from Biogen Idec, Merck-Serono, Novartis, Teva Pharmaceutical Industries, Roche, Italian Ministry of Health, Fondazione Italiana Sclerosi Multipla, and ARiSLA (Fondazione Italiana di Ricerca per la SLA). M.L. Gorno-Tempini is funded by the NIH and the Charles Schwab foundation. Go to Neurology.org/N for full disclosures. E. Canu has received research support from the Italian Ministry of Health. F. Agosta is Section Editor of NeuroImage: Clinical;has received speaker honoraria from Biogen Idec, Novartis, and Philips; and receives or has received research support from the Italian Ministry of Health, AriSLA (Fondazione Italiana di Ricerca per la SLA), and the European Research Council. G. Battistella, E.G. Spinelli, J. DeLeon, A.E. Welch, M.L. Mandelli, This study has been supported by the Italian Ministry of Health (F.A., GR-2010-2303035; GR-2011-02351217) and by the NIH (M.L.G.-T., NINDS R01 NS050915; NIDCD K24 DC015544; NIA U01 AG052943) (B.M., NIA P50 AG023501; NIA P01 AG019724; Alzheimer’s Disease Center of California [03-75271 DHS/ADP/ARCC]), Larry L. Hillblom Foundation, John Douglas French Alzheimer’s Foundation, Koret Family Foundation, Consortium for Frontotemporal Dementia Research, and McBean Family Foundation.
Funders | Funder number |
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Alzheimer’s Disease Center of California | 03-75271 DHS/ADP/ARCC |
Charles Schwab foundation | |
Eli Lilly | |
International Society of Frontotemporal Dementia | |
Italian Ministry of Health | GR-2010-2303035, GR-2011-02351217 |
Italian Ministry of Health and Medical Research Council | |
John Douglas French Alzheimer’s Foundation | |
John Douglas French Alzheimer's Foundation | |
McBean Family Foundation | |
NIA/NIH | |
National Institutes of Health (NIH) | |
National Institute on Aging | P01AG019724 |
National Institute on Aging | |
National Institute on Deafness and Other Communication Disorders | K24 DC015544, P50 AG023501, NIA U01 AG052943 |
National Institute on Deafness and Other Communication Disorders | |
Institute of Neurological Disorders and Stroke National Advisory Neurological Disorders and Stroke Council | R01 NS050915 |
Institute of Neurological Disorders and Stroke National Advisory Neurological Disorders and Stroke Council | |
Larry L. Hillblom Foundation | |
Koret Foundation | |
Biogen IDEC | |
Cornell High Energy Synchrotron Source, Cornell University | |
Roche Italia | |
Rainwater Tau Consortium | |
H2020 European Research Council | |
Agenzia di Ricerca per la Sclerosi Laterale Amiotrofica |
ASJC Scopus subject areas
- Clinical Neurology