Clinical, imaging and pathological correlates of a hereditary deficit in verb and action processing
Clinical, imaging and pathological correlates of a hereditary deficit in verb and action processing
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DOI：
10.1093/brain/awh701
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发表时间：
2006-02-01
期刊：
影响因子：
14.5
通讯作者：
Hodges, JR
中科院分区：
文献类型：
--
作者：
Bak, TH;Yancopoulou, D;Hodges, JR
在许多神经系统疾病中观察到选择性动词和名词缺陷，它们的出现被解释为处理特定词类别的不同神经网络的证据。我们描述了第一个家族性动词处理选择性缺陷的案例。父亲（个人 I）和儿子（个人 II）出现了类似进行性核上性麻痹 (PSP) 的运动障碍，并与痴呆症相关。个体II的第二个孩子在连续检查中仍然没有症状。个体I和个体II中名词和动词加工之间的分离通过不同的方法得到证实，包括命名、理解、图片和单词关联的纵向评估以及词汇决策任务。尽管整体恶化，但在后续测试中差异仍然稳定。它与 FDG-PET 成像（个体 II）的左侧额叶代谢低下以及尸检检查的泛素阳性包涵体（个体 I）有关。选择性动词缺陷与家族运动障碍的关联提出了一个问题：相关的遗传因素是否可能影响动作及其以动作动词形式的抽象概念表征。通过证明病理学、遗传学、影像学和抽象认知障碍之间的联系，这项研究增进了我们对退行性脑疾病的理解，对神经科学和临床实践都有影响。
Selective verb and noun deficits have been observed in a number of neurological conditions and their occurrence has been interpreted as evidence for different neural networks underlying the processing of specific word categories. We describe the first case of a familial occurrence of a selective deficit of verb processing. Father (Individual I) and son (Individual II) developed a movement disorder resembling progressive supranuclear palsy (PSP) and associated with dementia. A second child of Individual II remained symptom-free on consecutive examinations. The dissociation between the processing of nouns and verbs in Individuals I and II was confirmed with different methods, including a longitudinal assessment of naming, comprehension, picture and word association, as well as a lexical decision task. The difference remained stable on follow-up testing despite overall deterioration. It was associated with left-sided frontal hypometabolism on FDG-PET imaging (Individual II) and with ubiquitin-positive inclusions on post-mortem examination (Individual I). The association of a selective verb deficit with a familial movement disorder raises the question whether related genetic factors might influence both movements and their abstract conceptual representations in the form of action verbs. By demonstrating a link between pathology, genetics, imaging and abstract cognitive impairments this study advances our understanding of degenerative brain disease with implications for both neuroscience and clinical practice.