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症（LBD）， 包括路易体痴呆（DLB）或帕金森氏病痴呆（PDD）。患者 LBD患有认知能力下降，有时与阿尔茨海默病（AD）有关，并且运动和 帕金森病（PD）中观察到的行为变化。不幸的是，LBD的诊断可能很困难， 特别是在那些在运动或明显的行为之前存在认知障碍的DLB患者中， 变化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也很低。 有限公司 LBD生物标志物开发的障碍包括受试者人数少，缺乏系统的 患者特征描述，以及未能进行纵向尸检随访。AD和PD都有 受益于一些大型“财团”，这些财团通过利用 几个研究中心的小组，以联合收割机的努力与标准化的方法来研究， 疾病一个很好的例子是阿尔茨海默病中心（ADC）计划，由国家卫生部资助。 老龄化研究所，在美国各地的30多个研究中心已经同意了一个标准化的 AD患者的诊断和表征的方法。其他类似的计划包括 阿尔茨海默病合作研究（ADCS），阿尔茨海默病神经影像学倡议（ADNI）， 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）和帕金森进展标志物倡议（PPMI）。幸运的是，后两个PD程序 包括更系统的临床评估和收集生物液体和成像数据， PD中的认知最近，在PPMI项目中增加了病理学部分。无类似方案 存在DLB。 该提案的目的是建立一个DLB研究中心联盟， 入组受试者的数量、系统评估（与AD和PD项目兼容）、收集生物液体和 成像数据和最终的尸检DLB财团（DLBC）将为以下方面奠定必要的基础： 生物标志物开发，并具有创建可用于生物标志物开发的正在进行的受试者样本的次要益处。 额外的转化和治疗研究。我们汇集了九个中心， 路易体障碍，与路易体痴呆研究和一般社区有密切联系 参加DLBC。这组调查人员多年来一直在广泛合作 并将为这一急需的资源提供基础。

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.