Imaging tau, amyloid, and neurodegeneration in PPA

摘要 原发性进行性失语是一种严重的神经退行性疾病 包括失语症的不断发展， 功能，至少在早期阶段。有多种亚型以及多种 潜在的病理学PPA及其亚型很难与其他亚型区分开来。 神经退行性疾病和彼此，特别是在其病程的早期。 目前几乎没有临床工具来帮助PPA的早期特异性诊断， 其子类型。 最近，我们在发展 新的成像技术，可能是非常有价值的早期特异性诊断， PPA潜在的特定病理学的分子基础。我们建议使用tau， 淀粉样蛋白成像示踪剂，试图区分这些潜在的分子 病理学，FDG-PET，功能连接MRI和形态结构 MRI测量和纵向监测神经变性的标志物， 语言网络和其他大脑区域。除了更准确的诊断， 这些措施可能对确定和监测下降很重要， 假定疗法的效果。 这项建议的总体目标是将这些方法从新的科学方法中转化出来， 将这些技术转化为临床上有用的工具， 提高诊断特异性和评估能力， PPA及其亚型。

Abstract Primary progressive aphasia (PPA) is a devastating neurodegenerative syndrome that involves relentless development of aphasia with relative sparing of other cognitive functions, at least early in its course. There are multiple subtypes as well as multiple underlying pathologies. PPA and its subtypes can be difficult to differentiate from other neurodegenerative disorders and from each other, particularly early in their course. There are currently few clinical tools to assist in the early specific diagnosis of PPA and its subtypes. We have recently made substantial preliminary progress toward the development of novel imaging techniques that could be extremely valuable in early specific diagnosis of the molecular basis of specific pathologies underlying PPA. We propose to use tau and amyloid imaging tracers to attempt to discriminate these underlying molecular pathologies, and FDG-PET, functional connectivity MRI, and morphometric structural MRI to measure and longitudinally monitor markers of neurodegeneration in the language network(s) and other brain regions. In addition to more accurate diagnosis, these measures will likely be important for prognostication and monitoring of decline and the effects of putative therapies. The overall goal of this proposal is to translate these methods from new scientific technologies into clinically useful tools that can be used by clinicians around the country and internationally to improve the diagnostic specificity and assessment capabilities for PPA and its subtypes.