Differentiating between right-lateralised semantic dementia and behavioural-variant frontotemporal dementia: an examination of clinical characteristics and emotion processing

Differentiating between right-lateralised semantic dementia and behavioural-variant frontotemporal dementia: an examination of clinical characteristics and emotion processing
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DOI:
10.1136/jnnp-2014-309120
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发表时间:
2015-10-01
影响因子:
11
通讯作者:
Irish, Muireann
Irish, Muireann
中科院分区:
医学1区
文献类型:
--
作者:
Kamminga, Jody;Kumfor, Fiona;Irish, Muireann

文献摘要

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背景和目的右侧语义性痴呆(右SD)和行为变异性额颞叶痴呆(bvFTD)在临床上表现相似,尽管潜在的大脑变化模式不同。本研究旨在阐明右侧SD与bvFTD的临床和认知特征,强调情绪加工及其相关的神经相关。方法选取右侧SD患者12例,右侧ftd患者19例。记录临床特征。所有患者均接受标准化神经心理测试和面部情绪处理测试。他们的表现与20名年龄和教育程度相匹配的对照组进行了比较。使用基于全脑体素的形态学分析,灰质强度与情绪处理表现相关。结果与bvFTD患者相比,右侧SD患者表现出不成比例的语言功能障碍、面孔失认和强迫性人格/行为改变的增加。相比之下,与右侧SD患者相比,bvFTD患者表现出明显的注意力/工作记忆缺陷,冷漠增加和执行功能障碍更大。共情能力下降、去抑制和饮食改变在两种痴呆亚型中都很常见。两种FTD综合征都存在情绪处理缺陷,但与脑萎缩的不同模式有关。在右侧SD中,情绪加工功能障碍主要与右侧内侧和外侧颞完整性相关,而在bvFTD中,情绪加工功能障碍主要与左侧颞、下额叶、眶额叶和右侧额叶回完整性相关。结论:本研究显示右侧SD和bvFTD的面部情绪加工缺陷具有可比性,与他们相似的临床特征保持一致。这些缺陷可归因于每组患者神经基质的差异,即右侧SD的右侧偏侧区与bvFTD的左侧偏侧区为主。
Background and purpose Right-lateralised semantic dementia (right SD) and behavioural-variant frontotemporal dementia (bvFTD) appear clinically similar, despite different patterns of underlying brain changes. This study aimed to elucidate distinguishing clinical and cognitive features in right SD versus bvFTD, emphasising emotion processing and its associated neural correlates.Methods 12 patients with right SD and 19 patients with bvFTD were recruited. Clinical features were documented. All patients were assessed on standardised neuropsychological tests and a facial emotion processing battery. Performance was compared to 20 age-matched and education-matched controls. Grey matter intensity was related to emotion processing performance using whole-brain voxel-based morphometry analysis.Results Patients with right SD exhibited disproportionate language dysfunction, prosopagnosia and a suggestion of increased obsessive personality/behavioural changes versus patients with bvFTD. In contrast, patients with bvFTD demonstrated pronounced deficits in attention/working memory, increased apathy and greater executive dysfunction, compared to patients with right SD. Decreased empathy, disinhibition and diet changes were common to both dementia subtypes. Emotion processing deficits were present in both FTD syndromes but were associated with divergent patterns of brain atrophy. In right SD, emotion processing dysfunction was associated with predominantly right medial and lateral temporal integrity, compared to mainly left temporal, inferior frontal and orbitofrontal and right frontal gyrus integrity in bvFTD.Conclusions This study demonstrates comparable deficits in facial emotion processing in right SD and bvFTD, in keeping with their similar clinical profiles. These deficits are attributable to divergent neural substrates in each patient group, namely, right lateralised regions in right SD, versus predominantly left lateralised regions in bvFTD.