Joubert Sven, Felician Olivier, Barbeau Emmanuel, Ranjeva Jean-Philippe, Christophe Marion, Didic Mira, Poncet Michel, Ceccaldi Mathieu
Centre de Recherche de l'Institut, Universitaire de Gériatrie, 4565, Chemin Queen Mary, Montreal, Quebec, Canada.
J Neurol. 2006 Nov;253(11):1447-58. doi: 10.1007/s00415-006-0232-x. Epub 2006 Jun 13.
The right temporal variant of frontotemporal lobar degeneration (Rtv-FTLD) is a focal degenerative condition affecting predominantly the right temporal lobe. The aim of this study was to further characterize the profile of cognitive impairment and the neuroanatomical basis of Rtv-FTLD patients without behavioural disturbances. A group of three patients with this syndrome had a detailed neuropsychological assessment, along with Voxel-Based Morphometry (VBM) of their brain to determine location of cortical atrophy. VBM analyses showed a pattern of atrophy that was predominant in the right hemisphere and concerned primarily the right anterior temporal lobe region. Patients carried out a test of famous people in which their ability to recognize, name and provide semantic information about famous persons from their faces, their voices and their names was investigated. They all showed a severe defect in recognizing, naming and identifying famous people irrespective of modality. Therefore, their inability to recognize famous people resulted from a multimodal defect (semantic). These results highlight the semantic nature of the defect, and suggest that the anterior right temporal lobe may have a prominent role in processing person-based semantic knowledge. This study helps in further understanding the neuropsychological profile of patients with Rtv-FTLD.
额颞叶变性的右侧颞叶变异型（Rtv-FTLD）是一种主要影响右侧颞叶的局灶性退行性疾病。本研究的目的是进一步描述无行为障碍的Rtv-FTLD患者的认知障碍特征及神经解剖学基础。一组三名患有该综合征的患者接受了详细的神经心理学评估，并对其大脑进行基于体素的形态学测量（VBM）以确定皮质萎缩的位置。VBM分析显示萎缩模式以右侧半球为主，主要涉及右侧前颞叶区域。患者进行了一项名人测试，调查他们从名人的面部、声音和名字识别、说出并提供有关名人语义信息的能力。他们在识别、说出和辨认名人方面均表现出严重缺陷，且与方式无关。因此，他们无法识别名人是由多模态缺陷（语义）导致的。这些结果突出了缺陷的语义本质，并表明右侧前颞叶可能在处理基于人物的语义知识方面发挥重要作用。本研究有助于进一步了解Rtv-FTLD患者的神经心理学特征。
