Language in Primary Progressive Aphasia

原发性进行性失语(PPA)是一种成人发病的疾病，选择性地攻击左半球的语言网络。尽管在这一领域取得了相当大的进展，但PPA患者及其家人仍然得不到足够的服务。在专业中心之外，准确的医疗诊断可能是具有挑战性的，相关的教育材料稀缺，社区中很少有言语和语言病理学家具备提供知情干预的能力，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的调查团队。它寻求进行一项研究计划，该计划目前已进入第10个年头，已招募了近150名患者进行纵向调查。作为拟议工作的结果，我们希望提高我们对患者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，澄清阿尔茨海默病引起的PPA与额颞部退行性疾病的诊断指纹，并提高我们追踪疾病时间轨迹的精确度。这项工作还将继续产生附带好处，包括为患者和家属编写新的教育材料，提高对综合征的认识，可能使PPA患者更多地参加相关临床试验，以及培训学生和研究员，他们将发展成为服务于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.