Frontotemporal dementia (FTD), a neurodegenerative disease broadly characterized by socioemotional impairments, includes three clinical subtypes: behavioral variant FTD (bvFTD), semantic variant primary progressive aphasia (svPPA) and non-fluent variant primary progressive aphasia (nfvPPA). Emerging evidence has shown emotional reactivity impairments in bvFTD and svPPA, whereas emotional reactivity in nfvPPA is far less studied. In 105 patients with FTD (49 bvFTD, 31 svPPA and 25 nfvPPA) and 27 healthy controls, we examined three aspects of emotional reactivity (physiology, facial behavior and subjective experience) in response to a sad film. In a subset of the sample, we also examined the neural correlates of diminished aspects of reactivity using voxel-based morphometry. Results indicated that all three subtypes of FTD showed diminished physiological responding in respiration rate and diastolic blood pressure; patients with bvFTD and svPPA also showed diminished subjective experience, and no subtypes showed diminished facial behavior. Moreover, there were differences among the clinical subtypes in brain regions where smaller volumes were associated with diminished sadness reactivity. These results show that emotion impairments extend to sadness reactivity in FTD and underscore the importance of considering different aspects of sadness reactivity in multiple clinical subtypes for characterizing emotional deficits and associated neurodegeneration in FTD.

中文翻译：

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额颞叶痴呆亚型的生理、行为和主观悲伤反应。


额颞叶痴呆（FTD）是一种以社会情绪障碍为主要特征的神经退行性疾病，包括三种临床亚型：行为变异型 FTD（bvFTD）、语义变异型原发性进行性失语症（svPPA）和非流利变异型原发性进行性失语症（nfvPPA）。新出现的证据表明 bvFTD 和 svPPA 中的情绪反应性受损，而 nfvPPA 中的情绪反应性研究却少得多。在 105 名 FTD 患者（49 名 bvFTD、31 svPPA 和 25 nfvPPA）和 27 名健康对照中，我们检查了对悲伤电影的情绪反应的三个方面（生理、面部行为和主观体验）。在样本的一个子集中，我们还使用基于体素的形态测量法检查了反应性减弱方面的神经相关性。结果表明，FTD 的所有三种亚型在呼吸频率和舒张压方面均表现出生理反应减弱； bvFTD 和 svPPA 患者也表现出主观体验减弱，并且没有亚型表现出面部行为减弱。此外，大脑区域的临床亚型之间存在差异，这些区域的体积较小与悲伤反应性减弱相关。这些结果表明，情绪障碍延伸到 FTD 中的悲伤反应性，并强调考虑多种临床亚型中悲伤反应性的不同方面对于表征 FTD 中情绪缺陷和相关神经退行性变的重要性。