CLINICAL CRITERIA

该项目的目标是完善额颞叶变性(FTLD)的生前诊断，并 通过将其与阿尔茨海默病(AD)区分开来，通过改进早期诊断和通过 区分泛素包涵体和tau包涵体的患者。到第十年年底 PPG这个项目将在107年AD，131 FTD，67 SD，56 PNFA，43 CBD，30获得新的评估 PSP，59 ALS，对照。神经病理学将在37个AD完成，FTD，25个SD，23个PNFA，23个 CBD 23例，PSP 23例，ALS 27例。通过探索这些人群中的临床病理相关性， 项目将实现以下目标：1.完善FTD、PNFA、SD、CBD和 PSP，以改善这些患者群体在生活中与AD患者的分离。这一目标将是 通过使用匹兹堡化合物B(PIB)的淀粉样蛋白成像促进。此外，基因组学和 蛋白质组学将作为鉴别诊断FTD、PNFA、SD、CBD和PSP的工具进行初步探索 彼此之间，以及从公元后。2.探讨临床痴呆评分FTD患者的最早变化 CDR评分为0.5分，ALS患者(许多人会有早期FTD)。这一目标将通过使用诺基亚进行测试 通过临床和管理核心捕获社交、情感和成像领域的范例 以及在项目2、3和4中。此外，基因组学和蛋白质组学将被探索为早期的潜在标记 FTD或FTD-ALS。3.确定最能预测FTLD-T与病理学定义的FTLD-U的症状。这个 AIM将通过分析通过临床和管理核心和项目2收集的数据来执行 此外，还将探索这些种群的基因组和蛋白质组图谱。

The project's goal is to refine antemortem diagnosis of frontotemporallobar degeneration (FTLD) and related disorders by distinguishing them from Alzheimer's disease (AD), by improving early diagnosis and by differentiating patients with ubiquitin inclusions from those with tau inclusions. By the end of year 10 of the PPG this project will have obtained novel assessment on 107 AD, 131 FTD, 67 SD, 56 PNFA, 43 CBD, 30 PSP, 59 ALS, and 89 controls. Neuropathology will be completed in 37 AD, 64 FTD, 25 SD, 23 PNFA, 23 CBD, 23 PSP and 27 ALS patients. By exploring clinical pathological correlates in these populations the project will accomplish the following aims: 1. Refine diagnostic approaches to FTD, PNFA, SD, CBD, and PSP in order to improve separation of these patient groups during life from patients with AD. This aim will be facilitated by the use of amyloid imaging with the Pittsburgh-compound-B (PIB). Also, genomics and proteomics will be preliminarily explored as tools for differential diagnosis of FTD, PNFA, SD, CBD and PSP from each other, and from AD. 2. Explore the earliest changes in FTD patients with a clinical dementia rating (CDR) score of 0.5, and ALS patients (many will have early FTD). This aim will be tested using novel paradigms that capture social, emotional and imaging domains through the Clinical and Administrative Core and in projects 2, 3 and 4. Also, genomics and proteomics will be explored as potential markers of early FTD or FTD-ALS. 3. Identify syndromes that best predict FTLD-T vs. FTLD-U as defined at pathology. The aim will be performed by analyzing data collected through the Clinical and Administrative Core and projects 2 and 3. Also, genomic and proteomic profiles will be explored in these populations.