Background: Although traditionally conceptualized as a language disorder, semantic variant primary progressive aphasia (svPPA) is often accompanied by significant behavioral and affective symptoms which considerably increase disease morbidity. Specifically, these neuropsychiatric symptoms are characterized by breaches in normative socioaffective function, for example, an inability to read social cues, excessive trusting of others, and decreased empathy. Our prior neuroimaging work identified 3 corticolimbic networks anchored in the amygdala, temporal pole, and frontoinsular cortex: an affiliation network, theorized to mediate social approach behavior; an aversion network, theorized to subserve the appraisal of social threat; and a perception network, theorized to mediate the detection of social cues. We hy­pothesized that degeneration of these networks could provide neuroanatomical substrates for socioaffective deficits in svPPA. Methods: We examined hypothesized relationships between subscores on the Social Impairment Rating Scale (SIRS) and atrophy in each of these 3 networks in a group of 16 svPPA patients (using matched cognitively normal controls as a reference). Results: Consistent with our predictions, the magnitude of atrophy in the affiliation network in svPPA patients correlated with the SIRS subscore of socioemotional detachment, while the magnitude of atrophy in the aversion network in svPPA patients correlated with the SIRS subscore of inappropriate trusting. We did not find the predicted association between perception network atrophy and the SIRS subscore of lack of attention to social cues. Conclusion: These findings highlight specific socioaffective deficits in svPPA and provide a neuroanatomical basis for these impairments by linking them to networks commonly targeted in this disorder.
Dement Geriatr Cogn Disord

中文翻译：

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不同皮质边缘网络的萎缩有助于语义变异原发性进行性失语症的社会情感行为

背景：虽然传统上被概念化为一种语言障碍，但语义变异原发性进行性失语症 (svPPA) 通常伴随着显着增加疾病发病率的显着行为和情感症状。具体来说，这些神经精神症状的特征是规范社会情感功能的破坏，例如，无法阅读社会线索、过度信任他人和同理心下降。我们之前的神经影像学工作确定了 3 个锚定在杏仁核、颞极和额岛皮质的皮质边缘网络：一个从属网络，理论上可以调节社交接近行为；厌恶网络，理论上有助于评估社会威胁；和一个感知网络，理论上可以调解社会线索的检测。方法：我们在一组 16 名 svPPA 患者（使用匹配的认知正常对照作为参考）中检查了社交障碍评定量表 (SIRS) 子评分与这 3 个网络中每一个网络中的萎缩之间的假设关系。结果：与我们的预测一致，svPPA 患者的附属网络萎缩程度与社会情感分离的 SIRS 子评分相关，而 svPPA 患者的厌恶网络萎缩程度与不适当信任的 SIRS 子评分相关。我们没有发现感知网络萎缩与缺乏对社会线索关注的 SIRS 子评分之间的预测关联。结论：这些发现突出了 svPPA 中特定的社会情感缺陷，并通过将它们与通常针对这种疾病的网络联系起来，为这些损伤提供了神经解剖学基础。
痴呆老年认知障碍