Dementia with Lewy Bodies Consortium

项目摘要 据估计，美国有140万人患有路易体痴呆(LBD)， 包括路易体痴呆(DLB)或帕金森氏病痴呆(PDD)。患有疾病的患者 LBD患有认知能力下降，有时与阿尔茨海默病(AD)有关，而运动和 帕金森氏病(PD)患者的行为改变。不幸的是，LBD的诊断可能很困难， 尤其是那些在运动或明显行为障碍之前出现认知障碍的DLB患者 改变。LBD的生物标志物很少，它们在诊断、预后和治疗反应方面的价值是 有限的。 阻碍LBD生物标记物开发的因素包括受试者数量较少，缺乏系统性 患者特征，以及未能通过尸检进行纵向随访。AD和PD都有 受益于一些大型“财团”，这些“财团”通过利用 几个研究中心小组将努力与标准化方法相结合，以研究 疾病。一个很好的例子是阿尔茨海默氏症中心(ADC)计划，该计划由国家 老龄化研究所，全美30多个研究中心已经同意一项标准化的 阿尔茨海默病的诊断和特征探讨。其他类似的计划包括 阿尔茨海默病合作研究(ADCS)、阿尔茨海默病神经成像倡议(ADNI)、 美国国家神经疾病和中风研究所(NINDS)帕金森病生物标志物项目 (PDBP)和帕金森进展标记物倡议(PPMI)。幸运的是，后两个PD程序 包括更系统的临床评估和收集与以下相关的生物液和成像数据 帕金森病患者的认知功能。最近，PPMI项目增加了一个病理学部分。没有类似的计划 存在于DLB。 这项建议的目标是建立一个由多个研究中心组成的联合体， 注册的科目数量、系统评估(与AD和PD计划兼容)、收集 生物体液和成像数据，以及最终的尸检。DLB财团(DLBC)将创建必要的 为生物标记物开发奠定基础，并从创建正在进行的主题中获得次要好处 样本可用于其他翻译和治疗研究。我们已经汇集了九个中心 拥有路易体障碍的专业知识，并与路易体痴呆症研究有很强的联系 和普通社区一起参与DLBC。这组调查人员广泛合作 并将为这一亟需的资源提供基础。

Project Summary It has been estimated that 1.4 million people in the United States suffer from Lewy body dementia (LBD), including both dementia with Lewy bodies (DLB) or Parkinson's disease with dementia (PDD). Patients with LBD suffer from cognitive decline, sometimes linked to Alzheimer's disease (AD), and the motor and behavioral changes seen in Parkinson's disease (PD). Unfortunately, the diagnosis of LBD can be difficult, particularly in those DLB patients that present with cognitive impairment prior to motor or marked behavioral changes. Biomarkers for LBD are few and their value in diagnosis, prognosis, and for treatment response is limited. Impediments to biomarker development in LBD have included small subject numbers, a lack of systematic patient characterization, and a failure to perform longitudinal follow up with autopsy. Both AD and PD have benefited from a number of large “consortiums” that have advanced research by leveraging the strengths of several groups of research centers to combine efforts with standardized approaches to the study of the disease. One good example is the Alzheimer's Disease Centers (ADC) program, funded by the National Institute on Aging, where over 30 research centers across the United States have agreed to a standardized approach to the diagnosis and characterization of patients with AD. Other similar programs include the Alzheimer's Disease Cooperative Study (ADCS), Alzheimer's Disease Neuroimaging Initiative (ADNI), the National Institute of Neurological Disorders and Stroke (NINDS) Parkinson's Disease Biomarker Program (PDBP), and the Parkinson's Progression Marker Initiative (PPMI). Fortunately, the latter two PD programs have included more systematic clinical assessments and collection of biofluids and imaging data relevant to cognition in PD. Recently, a pathology component has been added to the PPMI project. No similar program exists for DLB. The objective of this proposal is to establish a consortium of centers for the study of DLB with a large number of subject enrolled, systematic assessments (compatible with AD and PD programs), collection of biofluids and imaging data, and ultimately autopsy. The DLB consortium (DLBC) would create the necessary foundation for biomarker development and have the secondary benefit of creating an ongoing subject sample available for additional translational and therapeutic studies. We have brought together nine centers with expertise in the Lewy body disorders and with strong connections to the Lewy body dementia research and general community to participate in the DLBC. This group of investigators has collaborated extensively together for many years and will provide the foundation for this much needed resource.