Progressive Aphasia Cognition Anatomy and Progression

进行性失语症认知解剖和进展

• 批准号: 6922040
• 负责人: MARIA LUISA GORNO TEMPINI
• 金额: $ 28.54万
• 依托单位: UNIVERSITY OF CALIFORNIA, SAN FRANCISCO
• 依托单位国家: 美国
• 财政年份: 2004
• 资助国家: 美国
• 起止时间: 2004-07-15 至 2008-02-29
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6922040
• 关键词: aphasia; apraxias; brain morphology; clinical research; cognition disorders; human subject; longitudinal human study; magnetic resonance imaging; morphometry; neural degeneration; patient oriented research; prefrontal lobe /cortex; semantics; syntax; temporal lobe /cortex

DESCRIPTION (provided by applicant): Speech and language impairments can often be the first manifestation of neurodegenerative diseases such as frontotemporal lobar degeneration, corticobasal degeneration and Alzheimer's disease. When the deficits remain limited to language symptoms for at least two years, the term primary progressive aphasia applies. Characterization of the linguistic, cognitive and anatomical features of patients in this first stage of the diseases could provide critical information for early diagnosis of the underlying etiology of the symptoms. However, speech and language functions are rarely assessed in the setting of dementia clinics. Moreover, data from large-scale, longitudinal clinical and neuroimaging studies of primary progressive aphasia have not been available to help guide clinicians. In this study, we propose to perform a detailed, five-year longitudinal investigation of the linguistic and anatomical features of over 200 patients with primary progressive aphasia. This large cohort will be possible because the UCSF Memory and Aging Center has an internationally renowned program of research in atypical and early-onset dementias. General neuropsychological, neurological, functional and most neuroimaging data will also be collected as part of other ongoing projects at the Memory and Aging Center. We will combine modem cognitive and neuroimaging analysis techniques to isolate the subcomponents of the speech and language system that are affected in progressive aphasia and their corresponding sites of anatomical damage. Our preliminary data from 40 patients already show that specific speech and language deficits correspond to remarkably focal patterns of brain atrophy. We will also follow the progression of the disease for five years, and apply statistical models to investigate how changes in language and anatomical features correlate with each other, with changes in functional impairment and with etiological diagnosis at the end of the study period. Evidence gathered from this research will increase our knowledge about the neural basis of speech and language functions and provide crucial data for the diagnosis of neurodegenerative diseases in their early stages, when treatment can be most effective.

描述（由申请人提供）：言语和语言障碍通常是神经退行性疾病（如额颞叶变性、皮质基底节变性和阿尔茨海默病）的最初表现。当缺陷仍然局限于语言症状至少两年，术语原发性进行性失语症适用。在疾病的第一阶段，对患者的语言、认知和解剖特征进行表征，可以为早期诊断症状的潜在病因提供关键信息。然而，言语和语言功能很少在痴呆症诊所进行评估。此外，原发性进行性失语症的大规模、纵向临床和神经影像学研究的数据还不能帮助指导临床医生。在这项研究中，我们建议进行一项详细的，五年的纵向调查的语言和解剖特征的200多例原发性进行性失语症。这个大的队列将是可能的，因为UCSF记忆和衰老中心有一个国际知名的非典型和早发性痴呆研究计划。一般神经心理学，神经学，功能和大多数神经影像学数据也将作为记忆和衰老中心其他正在进行的项目的一部分收集。我们将结合联合收割机现代认知和神经影像分析技术，分离出在进行性失语症中受影响的言语和语言系统的子成分及其相应的解剖损伤部位。我们从40名患者的初步数据已经表明，特定的语言和语言缺陷对应于显着的局灶性脑萎缩模式。我们还将跟踪疾病的进展五年，并应用统计模型来研究语言和解剖特征的变化如何相互关联，功能障碍的变化以及研究期结束时的病因诊断。从这项研究中收集的证据将增加我们对语音和语言功能的神经基础的了解，并为早期神经退行性疾病的诊断提供关键数据，此时治疗可能最有效。