Laboratory of Experimental Psychology and Neuroscience (LPEN), Institute of Cognitive and Translational Neuroscience (INCYT), INECO Foundation, Favaloro University, Buenos Aires, Argentina; National Scientific and Technical Research Council (CONICET), Buenos Aires, Argentina.
Laboratory of Experimental Psychology and Neuroscience (LPEN), Institute of Cognitive and Translational Neuroscience (INCYT), INECO Foundation, Favaloro University, Buenos Aires, Argentina; National Scientific and Technical Research Council (CONICET), Buenos Aires, Argentina; Universidad Autónoma del Caribe, Barranquilla, Colombia; Center for Social and Cognitive Neuroscience (CSCN), School of Psychology, Universidad Adolfo Ibáñez, Santiago, Chile; Centre of Excellence in Cognition and its Disorders, Australian Research Council (ACR), Sydney, Australia.
Neurosci Biobehav Rev. 2019 May;100:263-284. doi: 10.1016/j.neubiorev.2019.02.020. Epub 2019 Mar 13.
Although primary progressive aphasia (PPA) is clinically typified by linguistic impairments, emerging evidence highlights the presence of early deficits in social cognition. This review systematically describes the latter patterns, specifying their relation to the characteristic linguistic dysfunctions and atrophy patterns of non-fluent, semantic, and logopenic variants of the disease (nfvPPA, svPPA, and lvPPA, respectively), relative to closely related dementia types. Whereas the evidence on lvPPA proves scant, studies on nfvPPA and svPPA patients show consistent deficits in emotion recognition, theory of mind, and empathy. Notably, these seem to be intertwined with language impairments in nfvPPA, but they prove primary and independent of language disturbances in svPPA. Also, only the profile of svPPA resembles that of behavioral variant frontotemporal dementia, probably reflecting the overlap of fronto-temporal disruptions in both conditions. In short, the neurocognitive relationship between linguistic and socio-cognitive deficits cannot be precisely predicated for PPA as a whole; instead, specific links must be acknowledged in each variant. These emergent patterns pave the way for fruitful dimensional research in the field.
尽管原发性进行性失语症 (PPA) 在临床上以语言障碍为特征，但新出现的证据强调了早期社会认知缺陷的存在。本综述系统地描述了这些模式，特别指出了它们与非流利型、语义型和词汇流畅性损害型 PPA 变异体 (nfvPPA、svPPA 和 lvPPA) 的特征性语言功能障碍和萎缩模式的关系，与密切相关的痴呆类型进行了对比。尽管关于 lvPPA 的证据很少，但对 nfvPPA 和 svPPA 患者的研究表明，他们在情绪识别、心理理论和同理心方面存在一致的缺陷。值得注意的是，这些似乎与 nfvPPA 中的语言障碍相互交织，但在 svPPA 中，它们是主要的，且独立于语言障碍。此外，只有 svPPA 的表现与行为变异型额颞叶痴呆相似，这可能反映了两种情况下额颞叶紊乱的重叠。简而言之，对于 PPA 整体而言，语言和社会认知缺陷之间的神经认知关系不能准确预测；相反，必须在每种变体中承认特定的联系。这些新出现的模式为该领域的富有成效的维度研究铺平了道路。
