NEUROIMAGING IN HUNTINGTONS DISEASE

拟议的神经成像项目的目的是使用定量的 通过MRI测量来绘制萎缩的纵向过程， 亨廷顿病患者的大脑结构。 结合 通过项目（神经心理学）和核心（临床），我们还将确定 这些神经解剖学变化与纵向 神经心理功能和临床病理学的变化。 我们 将对150名亨廷顿病患者和150名 个体匹配的对照组。 患者将每两周接受一次扫描 使用相同的标准化MRI方案。 MRI测量将 包括基底神经节（尾状核、壳核、苍白球）、脑 脑干、小脑和比较区域，以及总灰色、白色和 脑脊液容量和特定皮质区域的容量。 MRI测量将 在同一次访视中获得相关和神经心理学测量。 我们假设特定脑区的MRI测量结果与 具有特定的神经心理学功能、临床变量和长度 HD患者的三核苷酸重复序列。 MRI纵向变化 还将分析措施。 我们假设，在不同的萎缩， 大脑的各个区域会以不同的速度发展 疾病 我们还假设，特定大脑的萎缩率 区域将与具体的下降率相关 神经心理功能 该项目将提供第一个 系统地观察了脑内几个特定脑区的萎缩， 相同的HD患者。 通过比较具体的 神经心理功能和临床症状与萎缩的具体 可能以不同速度和在不同点恶化的区域 在患病期间，我们将能够提供更多 HD中大脑与行为关系的完整图片。

The purpose of the proposed Neuroimaging Project is to use quantitative measures from MRI to chart the longitudinal course of atrophy in specific brain structures in patients with Huntington's Disease. In conjunction with Project (Neuropsychology) and Core (Clinical), we will also determine the association between these neuroanatomical changes and longitudinal changes in neuropsychological functioning and clinical symptomatology. We will obtain MRI scans on 150 patients with Huntington's Disease, and on 150 individually matched control subjects. Patients will be scanned every two years using the same standardized MRI protocol. MRI measurements will include volumes of basal ganglia (caudate, putamen, globus pallidus), brain stem, cerebellum, and comparison regions, as well as total gray, white, and CSF volumes, and volumes of specific cortical regions. MRI measures will be correlated and neuropsychological measures obtained at the same visit. We hypothesize that MRI measures of specific brain regions will correlate with specific neuropsychological functions, clinical variables, and length of trinucleotide repeat for the HD patients. Longitudinal changes in MRI measures will also be analyzed. We hypothesize that atrophy in various regions of the brain will progress at different rates during the course of the illness. We also hypothesize that rate of atrophy in specific brain regions will be correlated with rate of decline in specific neuropsychological functions. This project will provide the first systematic view of atrophy in several specific brain regions within the same HD patients over time. By comparing decline in specific neuropsychological functions and clinical symptoms with atrophy in specific regions that may deteriorate at different rates and at different points during the course of the illness, we will be able to provide a more complete picture of brain-behavior relationships in HD.