Language in Primary Progressive Aphasia

项目描述（由申请人提供）：该项目的目的是研究原发性进行性失语症（PPA）的语言障碍及其治疗方式。该方法是多学科的，将包括神经病学、言语病理学、神经心理学、电生理学和成像方面的专家。这些方法将包括计时神经语言学研究、事件相关电位、基于体素的形态测量和扩散张量成像。有几个理由可以证明一个专注于PPA的研究项目是合理的。首先，PPA是唯一一种选择性攻击大脑语言网络的成人发病疾病。其次，PPA患者患有失语障碍，值得更充分地描述，特别是因为该疾病的早期阶段与有效的治疗干预相适应。第三，PPA中语言能力的缓慢消退为研究语言的神经认知组织提供了一个独特的环境，这是任何其他失语障碍所无法复制的。第四，PPA患者的相对稀缺，疾病的时间进展，以及需要招募足够数量的患者的广泛集水区，创造了一种情况，即多学科团队的综合工作是开展该综合征研究计划的最有效方式。该提案来自一个研究PPA多年并进行了广泛合作的研究小组。这些患者将从世界上最容易被认可的PPA转诊中心之一的诊所招募。这项工作将利用西北阿尔茨海默病中心的资源，以一种提高工作经济性的方式。

DESCRIPTION (provided by applicant): The goal of the project is to investigate the language disorder and treatment modalities in primary progressive aphasia (PPA). The approach is multidiscipiinary and will include experts in neurology, speech pathology, neuropsychology, electrophysiology and imaging. The methods will include chronometric neurolinguistic investigations, event-related potentials, voxel based morphometry and diffusion tensor imaging. Several reasons justify a research program focusing on PPA. First, PPA constitutes the only adult-onset disease that selectively attacks the language network in the brain. Second, PPA patients suffer from aphasic disturbances that deserve to be characterized more fully, especially since the early stages of the disease are compatible with effective therapeutic interventions. Third, the slow dissolution of language abilities in PPA offers a unique setting, not duplicated by any other aphasic disturbance, for investigating the neurocognitive organization of language. Fourth, the relative scarcity of PPA patients, the temporal progression of the disease, and the wide catchment area needed to recruit a sufficient number of patients create a situation where the integrated work of a multidiscipiinary team is the most efficient way to conduct a research program focusing on this syndrome. The proposal comes from a team of investigators who have worked on PPA for many years and have collaborated extensively. The patients will be recruited from a clinic that is one of the world's most readily recognized referral centers for PPA. The work will use the resources of the Northwestern Alzheimer's Disease Center in a way that will enhance economy of effort.