Spotorno Nicola, McMillan Corey T, Irwin David J, Clark Robin, Lee Edward B, Trojanowski John Q, Weintraub Daniel, Grossman Murray
Penn Frontotemporal Degeneration Center, University of Pennsylvania Perelman School of MedicinePhiladelphia, PA, USA; Brain Plasticity and Neurodegeneration Group, German Center for Neurodegenerative Diseases (DZNE)Magdeburg, Germany.
Penn Frontotemporal Degeneration Center, University of Pennsylvania Perelman School of Medicine Philadelphia, PA, USA.
Front Hum Neurosci. 2017 Jan 11;10:693. doi: 10.3389/fnhum.2016.00693. eCollection 2016.
Lewy body disorders (LBD) are clinical syndromes characterized by pathological inclusions containing α-synuclein. Cognitive deficits are common or diagnostic in LBD, and may be associated with the presence of beta-amyloid (Aβ), which is a hallmark histopathologic abnormality characteristic of Alzheimer's disease (AD) that can also co-occur with LBD. In the present study we evaluated whether social decision-making difficulties in LBD are associated with Aβ burden. Decision-making abilities were measured with a simple, untimed, behavioral task previously validated in patients with behavioral variant frontotemporal dementia, and performance was related to gray matter atrophy on MRI. Aβ burden was assessed by examination of cerebrospinal fluid (CSF) level of Aβ and by autopsy confirmation in a subgroup of patients. The results revealed that LBD patients with evidence of Aβ have reduced social decision-making abilities compared to patients with no evidence of Aβ. The imaging analysis related greater decision-making difficulty in Aβ-positive patients in respect to Aβ-negative patients to gray matter atrophy in medial orbitofrontal. This region is a critical node of a decision-making network as well as a region previously associated with comorbid α-synuclein and Aβ in LBD. These preliminary findings suggest that cognitive difficulties in LBD extend to include deficits in social decision-making and that this may be related to the presence of Aβ.
路易体障碍（LBD）是一类临床综合征，其特征为含有α-突触核蛋白的病理性包涵体。认知缺陷在LBD中很常见或具有诊断意义，并且可能与β-淀粉样蛋白（Aβ）的存在有关，Aβ是阿尔茨海默病（AD）标志性的组织病理学异常特征，也可与LBD同时出现。在本研究中，我们评估了LBD患者的社会决策困难是否与Aβ负担相关。使用一项先前在行为变异型额颞叶痴呆患者中验证过的简单、无时间限制的行为任务来测量决策能力，并且将表现与MRI上的灰质萎缩相关联。通过检测脑脊液（CSF）中Aβ水平以及在一组患者中进行尸检确认来评估Aβ负担。结果显示，与无Aβ证据的患者相比，有Aβ证据的LBD患者的社会决策能力降低。成像分析表明，Aβ阳性患者相对于Aβ阴性患者决策困难更大，这与内侧眶额皮质的灰质萎缩有关。该区域是决策网络的关键节点，也是先前与LBD中共存的α-突触核蛋白和Aβ相关的区域。这些初步发现表明，LBD患者的认知困难扩展到包括社会决策缺陷，并且这可能与Aβ的存在有关。
