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000277796 1001_ $$aJürs, Alexandra V$$b0
000277796 245__ $$aCase report: Behavioral variant FTD confounding a language variant FTD in a case of PSP-CBS.
000277796 260__ $$aLausanne, Switzerland$$bFrontiers Media S.A.$$c2025
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000277796 520__ $$aFrontotemporal 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.
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000277796 650_7 $$2Other$$abehavioral variant
000277796 650_7 $$2Other$$acorticobasal syndrome
000277796 650_7 $$2Other$$afrontotemporal dementia
000277796 650_7 $$2Other$$anatural history
000277796 650_7 $$2Other$$aprimary progressive aphasia
000277796 650_7 $$2Other$$aprogressive supranuclear palsy
000277796 7001_ $$0P:(DE-2719)9000419$$aKasper, Elisabeth$$b1$$udzne
000277796 7001_ $$0P:(DE-2719)2810592$$aNeumann, Manuela$$b2$$udzne
000277796 7001_ $$aKurth, Jens$$b3
000277796 7001_ $$aKrause, Bernd J$$b4
000277796 7001_ $$aCantré, Daniel$$b5
000277796 7001_ $$0P:(DE-2719)2380559$$aPrudlo, Johannes$$b6$$eLast author$$udzne
000277796 773__ $$0PERI:(DE-600)3172541-7$$a10.3389/frdem.2025.1540519$$gVol. 4, p. 1540519$$p1540519$$tFrontiers in dementia$$v4$$x2813-3919$$y2025
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