Abstract
Language impairments, hallmarks of speech/language variant progressive supranuclear palsy, also occur in Richardson’s syndrome (PSP-RS). Impaired communication may interfere with daily activities. Therefore, assessment of language functions is crucial. It is uncertain whether the Aachen Aphasia Test (AAT) is practicable in PSP-RS, behavioral variant frontotemporal dementia (bvFTD) and Alzheimer’s dementia (AD) and language deficits differ in these disorders. 28 PSP-RS, 24 AD, and 24 bvFTD patients were investigated using the AAT and the CERAD-Plus battery. 16–25% of all patients failed in AAT subtests for various reasons. The AAT syndrome algorithm diagnosed amnestic aphasia in 5 (23%) PSP-RS, 7 (36%) bvFTD and 6 (30%) AD patients, Broca aphasia in 1 PSP-RS and 1 bvFTD patient, Wernicke aphasia in 1 bvFTD and 3 (15%) AD patients. However, aphasic symptoms resembled non-fluent primary progressive aphasia in 14 PSP-RS patients. In up to 46% of PSP-RS patients, 61% of bvFTD and 64% of AD patients significant impairments were found in the AAT subtests spontaneous speech, written language, naming, language repetition, language comprehension and the Token subtest. The CERAD-Plus subtest semantic fluency revealed significant impairment in 81% of PSP-RS, 61% of bvFTD, 44% of AD patients, the phonemic fluency subtest in 31, 40 and 31%, respectively. In contrast to bvFTD and AD, severity of language impairment did not correlate with cognitive decline in PSP-RS. In summary, the patterns of aphasia differ between the diagnoses. Local frontal language networks might be impaired in PSP-RS, whereas in AD and bvFTD, more widespread neuropathology might underly language impairment.
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The datasets generated and/or analyzed during the current study are available from the first and corresponding author on reasonable request.
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The study was supported by the Jubilee Funds of the Austrian National Bank, Project number 13240.
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Conceptualization: LR, GR; methodology: LR, GR; formal analysis and investigation: LR, AF, SR-T, RK, MK, PS, FF, MG, CE; writing—original draft preparation: LR; writing—review and editing: MK, PS, FF, MG, CE, RD, SM, GR; funding acquisition: GR; resources: GR; supervision: RD, GR.
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Lucia Ransmayr, Alexandra Fuchs, Sibylle Ransmayr-Tepser, Romana Kommenda, Franz Fellner, Christian Eggers, Robert Darkow and Stephanie Mangesius have nothing to declare. Mariella W. Kögl received honoraria as a speaker and consulting honoraria from AbbVie GmbH. Petra Schwingenschuh received consulting honoraria from AbbVie GmbH and Bial. Michael Guger received support and honoraria for research, consultation, lectures and education from Almirall, Bayer, Biogen, Celgene, Genzyme, MedDay, Merck, Novartis, Octapharma, Roche, Sanofi Aventis, Shire and TEVA ratiopharm. Gerhard Ransmayr received research support from the Jubilee Funds of the Austrian National Bank and the Austrian Research Promotion Funds, honoraria as speaker and for consultations from AbbVie GmbH, Alpine Market Research, Biogen, Grünenthal, MedAhead, Novartis Pharma GmbH, Ratiopharm, Roche Austria GmbH, Sanofi Aventis GmbH, Stada Arzneimittel-Gesellschaft, UCB Pharma GmbH. There are no competing financial or non-financial interests of the authors.
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The study was conducted according to the Declaration of Helsinki and approved by the local ethics review board (Ethikkommission des Landes Oberösterreich; FTLA Study, Protocol Number 254).
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The study was approved by the Ethikkommission des Landes Oberösterreich (application number 254) and conducted according to the 1975 Helsinki declaration. Participants gave their informed written consent.
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Ransmayr, L., Fuchs, A., Ransmayr-Tepser, S. et al. Differences in aphasia syndromes between progressive supranuclear palsy–Richardson’s syndrome, behavioral variant frontotemporal dementia and Alzheimer’s dementia. J Neural Transm 129, 1039–1048 (2022). https://doi.org/10.1007/s00702-022-02524-2
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DOI: https://doi.org/10.1007/s00702-022-02524-2