Dementia with Lewy Bodies Consortium

项目摘要 据估计，美国有超过140万人患有路易体 痴呆（LBD），包括路易体痴呆（DLB）或帕金森病伴痴呆 （项目设计司）。LBD患者患有认知能力下降，有时与阿尔茨海默病（AD）有关， 以及帕金森病（PD）中的运动和行为变化。不幸的是， LBD的治疗可能是困难的，特别是在那些DLB患者中， 运动或明显的行为变化。虽然LBD的诊断生物标志物有限，但最近 发展表明，确定路易体病理学（LBP）和AD病理学变化 可能是生物流体生物标志物。 直到最近，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，未能进行纵向尸检随访。 AD和PD都受益于一些大型“财团”，这些财团通过以下方式推进了研究： 利用几组研究中心的优势，将联合收割机的工作与标准化的 研究疾病的方法。一个很好的例子是阿尔茨海默病中心（ADC） 该计划由美国国家老龄问题研究所资助，美国有30多个研究中心 各国已同意采用标准化方法来诊断和定性患有 AD.其他类似的项目包括阿尔茨海默病合作研究（ADCS），阿尔茨海默病 疾病神经影像学倡议（ADNI），国家神经疾病和中风研究所 （NINDS）帕金森病生物标志物计划（PDBP）和帕金森病进展标志物 倡议。幸运的是，后两个PD项目已经包括了更系统的临床 评估和收集与PD认知相关的生物流体和成像数据。直到资助 路易体痴呆症联盟（DLBC），没有类似的美国计划存在。 本DLBC更新提案的目的是利用和扩大DLBC队列， 入组受试者，继续纵向系统评估，收集生物液体和成像 数据，最终尸检。作为一个更假设驱动的更新建议，DLBC将利用 DLBC参与者的纵向数据、成像和生物流体收集，以专注于诊断， 进展和临床变异性。两名新的联合首席研究员和多名新的联合首席研究员 研究人员和合作者将加入DLBC，以加强对新的 假设驱动的目标。

Project Summary It has been estimated that over 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. While diagnostic biomarkers for LBD have been limited, recent developments have suggested that determining Lewy body pathology (LBP) and AD pathology change may be possible with biofluid biomarkers. Until recently, 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. Until funding of the Dementia with Lewy Body Consortium (DLBC), no similar US-based program had existed. The objective of this DLBC renewal proposal is to utilize and expand the DLBC cohort with additional subjects enrolled, continued longitudinal systematic assessments, collection of biofluids and imaging data, and ultimately autopsy. As a more hypothesis-driven renewal proposal, the DLBC will utilize the longitudinal data, imaging and biofluid collection from DLBC participants to focus on diagnosis, progression and clinical variability. Two new Co-Principal Investigators and a number of new co- investigators and collaborators will join the DLBC to enhance the additional focus on the new hypothesis-driven aims.