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@ARTICLE{Jrs:277796,
      author       = {Jürs, Alexandra V and Kasper, Elisabeth and Neumann,
                      Manuela and Kurth, Jens and Krause, Bernd J and Cantré,
                      Daniel and Prudlo, Johannes},
      title        = {{C}ase report: {B}ehavioral variant {FTD} confounding a
                      language variant {FTD} in a case of {PSP}-{CBS}.},
      journal      = {Frontiers in dementia},
      volume       = {4},
      issn         = {2813-3919},
      address      = {Lausanne, Switzerland},
      publisher    = {Frontiers Media S.A.},
      reportid     = {DZNE-2025-00474},
      pages        = {1540519},
      year         = {2025},
      abstract     = {Frontotemporal dementia (FTD) occurs in two main clinical
                      subtypes, which can transition into one another: the
                      behavioral variant (bvFTD) and the language variant (primary
                      progressive aphasia; PPA). It is common for the latter, as
                      primary progressive aphasia (PPA), to transition into bvFTD;
                      however, the opposite development, where bvFTD is followed
                      by 'secondary progressive aphasia,' has received little
                      attention. This constellation is particularly challenging to
                      recognize as frontal dysexecutive syndrome can confound
                      subsequent progressive aphasia as impulsive behavior, a lack
                      of inhibition, and apathy can lead to non-aphasic
                      communication disturbances, including impoverished syntax,
                      reduced cognitive flexibility, and insufficient error
                      monitoring. A 78-year-old patient, with a disease duration
                      of 10 years, was initially diagnosed in the 3rd year of the
                      disease with corticobasal syndrome (CBS) with frontal
                      behavioral-spatial syndrome (CBS-FBS) and subsequently with
                      CBS with progressive non-fluent aphasia (CBS-PNFA) in the
                      4th year. Severe ophthalmoplegia was the reason for changing
                      the diagnosis in the seventh year to progressive
                      supranuclear palsy with CBS predominance type (PSP-CBS). The
                      pathological diagnosis was FTLD-tau in the form of a PSP
                      subtype. The MRI showed asymmetric atrophy, particularly of
                      the left insular cortex and the left inferior frontal gyrus.
                      The 2-[18F]FDG-PET revealed left-accentuated bifrontal
                      glucose hypometabolism. This case report highlights how
                      progressive neurodegenerative aphasia can occur in FTD not
                      only as a primary language phenomenon (in the sense of PPA)
                      but also as a secondary phenomenon (following a primary
                      behavioral disorder with a non-aphasic communication
                      disorder). Dysexecutive syndrome can mask aphasia.
                      Therefore, incorporating spontaneous speech tasks into
                      standard neuropsychological language tests, in addition to
                      MRI and PET imaging techniques, could help better recognize
                      such secondary aphasias, even in the presence of
                      dysexecutive syndrome, and thus broaden our understanding of
                      the natural history of FTD.},
      keywords     = {behavioral variant (Other) / corticobasal syndrome (Other)
                      / frontotemporal dementia (Other) / natural history (Other)
                      / primary progressive aphasia (Other) / progressive
                      supranuclear palsy (Other)},
      cin          = {AG Teipel / AG Neumann},
      ddc          = {610},
      cid          = {I:(DE-2719)1510100 / I:(DE-2719)1210003},
      pnm          = {353 - Clinical and Health Care Research (POF4-353) / 352 -
                      Disease Mechanisms (POF4-352)},
      pid          = {G:(DE-HGF)POF4-353 / G:(DE-HGF)POF4-352},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:40144925},
      pmc          = {pmc:PMC11937099},
      doi          = {10.3389/frdem.2025.1540519},
      url          = {https://pub.dzne.de/record/277796},
}