Language in Primary Progressive Aphasia

原发性进行性失语症（PPA）是一种成人发病的疾病，选择性地攻击左半球语言网络。尽管在这一领域取得了相当大的进展，但PPA患者及其家属仍然得不到充分的服务。在专业中心之外，准确的医学诊断可能具有挑战性，相关的教育材料稀缺，社区中很少有言语和语言病理学家有能力提供知情的干预措施，许多患者往往被排除在临床试验之外，因为与潜在神经病理学的复杂关系没有得到充分认识。这项建议来自一个主要关注PPA的研究小组。该公司寻求开展一项研究项目，该项目目前已进入第10个年头，招募了近150名患者进行纵向调查。通过这项研究，我们希望加深对患者所经历的词汇语义和语法障碍的理解，获得对左半球语言网络组织的新见解，阐明由阿尔茨海默病与额颞叶变性引起的PPA的诊断指纹，并提高我们追踪疾病时间轨迹的准确性。这项工作还将继续产生附带效益，为患者和家庭提供新的教育材料，提高对该综合征的认识，可能使PPA患者更容易获得相关临床试验，以及培训学生和研究员，这些学生和研究员将发展成为服务PPA患者需求的下一代临床医生和研究人员。

Primary progressive aphasia (PPA) is an adult onset disease that selectively attacks the left hemisphere language network. Despite considerable progress in this field, patients with PPA and their families remain underserved. Accurate medical diagnosis can be challenging outside of specialized centers, relevant educational materials are scarce, few speech and language pathologists in the community are equipped to provide informed interventions, and many patients tend to be excluded from clinical trials because the complex relationship to underlying neuropathology is not fully appreciated. This proposal comes from a team of investigators with a primary focus on PPA. It seeks to pursue a research program that is now in its 10th year and that has recruited nearly 150 patients for longitudinal investigation. As a consequence of the proposed work we expect to sharpen our understanding of lexicosemantic and grammatical impairments experienced by the patient, obtain new insights into the organization of the left-hemisphere language network, clarify the diagnostic fingerprints of PPA caused by Alzheimer’s disease versus frontotemporal degenerations, and increase the precision with which we can trace the temporal trajectory of the disease. The work will also continue to generate collateral benefits in the form of new educational materials for patients and families, improved recognition of the syndrome, potentially greater access of PPA patients to relevant clinical trials, and the training of students and fellows who will develop into the next generation of clinicians and investigators serving the needs of patients with PPA.