Case report: Behavioral variant FTD confounding a language variant FTD in a case of PSP-CBS.

Frontiers in dementia Pub Date : 2025-03-12 eCollection Date: 2025-01-01 DOI:10.3389/frdem.2025.1540519
Alexandra V Jürs, Elisabeth Kasper, Manuela Neumann, Jens Kurth, Bernd J Krause, Daniel Cantré, Johannes Prudlo
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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.

病例报告:一例PSP-CBS的行为变异型FTD合并语言变异型FTD。
额颞叶痴呆(FTD)有两种主要的临床亚型,它们可以相互转化:行为变体(bvFTD)和语言变体(原发性进行性失语症;PPA)。后者通常作为原发性进行性失语症(PPA)过渡到bvFTD;然而,相反的发展,即bvFTD之后出现“继发性进行性失语症”,却很少受到关注。这一组合尤其具有挑战性,因为额叶执行障碍综合征可将随后的进行性失语症混淆为冲动行为、缺乏抑制,而冷漠可导致非失语症交流障碍,包括语法不良、认知灵活性降低和错误监测不足。78岁患者,病程10 年,发病第3年确诊为皮质基底综合征(CBS)伴额部行为空间综合征(CBS- fbs),第4年确诊为CBS伴进行性非流畅性失语(CBS- pnfa)。严重眼麻痹是第7年将诊断改为进行性核上性麻痹伴CBS优势型(PSP-CBS)的原因。病理诊断为FTLD-tau,表现为PSP亚型。MRI显示不对称萎缩,尤其是左侧岛叶皮层和左侧额下回。2-[18F]FDG-PET显示左加重双额糖代谢低下。本病例报告强调了进行性神经退行性失语是如何在FTD中发生的,它不仅是一种主要的语言现象(在PPA的意义上),而且是一种次要现象(在原发性行为障碍和非失语交流障碍之后)。执行障碍综合症可以掩盖失语症。因此,将自发言语任务纳入标准的神经心理学语言测试,以及MRI和PET成像技术,可以帮助更好地识别这种继发性失语症,即使存在执行障碍综合征,从而拓宽我们对FTD自然史的理解。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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