NEUROPSYCHOLOGY OF HUNTINGTONS DISEASE

亨廷顿病的神经心理学

• 批准号: 6243501
• 负责人: JASON BRANDT
• 金额: $ 19.25万
• 依托单位: JOHNS HOPKINS UNIVERSITY
• 依托单位国家: 美国
• 财政年份: 1997
• 资助国家: 美国
• 起止时间: 1997-01-01 至 1997-12-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6243501
• 关键词: Huntington's disease; abnormal involuntary movement; age difference; basal ganglia; cerebral cortex; cerebral degeneration; clinical research; cognition disorders; dementia; functional ability; human subject; longitudinal human study; magnetic resonance imaging; nervous system disorder diagnosis; neuroanatomy; neuropsychological tests; nucleic acid repetitive sequence; pathologic process; prognosis; psychomotor function

Past efforts have focused on developing a well-characterized cohort of patients for longitudinal studies of the dementia syndrome of Huntington's disease (HD). To that end, groups of 80 HD patients and 22 control subjects have been recruited. In the next five years, the patient sample will be augmented with patients of early and late onset age Clinical Core. We will follow that cohort of 150 patients and 40 gene-negative controls in our Longitudinal Core Study to characterize, for the first time, the natural history of dementia in HD. This study is a collaborative effort between this Project. Neuroimaging Project and the Clinical Core. The cognitive data from this longitudinal study will be analyzed in conjunction with the annual assessments of disease severity as indexed by clinical measures (neuroimaging) obtained biannually on the same patients. The pattern and rate of cognitive decline will be examined, and the relative contributions of severity and rate of striatal and cortical atrophy, CAG repeat length (CoreD), and onset age to differences in rates of decline will be determined. The delineation of the natural history of dementia in HD will be helpful to clinicians in managing patient care, and will be particularly relevant for future clinical trials of potential therapeutic agents. Voluntary motor impairment is prominent in HD, but has not been examined extensively. Severity of impairment appears to vary across patients and across motor tasks. Voluntary motor performance will be assessed in mildly to moderately affected patients using the motor impairment scale from the Quantitated Neurological Exam, a limb apraxia battery, motor learning tasks, and tasks assessing the timing programming and sequencing of motor actions. Motor task performance will be related to cortical and subcortical atrophy to determine the anatomical correlates of impaired voluntary movement in HD, and to the Huntington's Disease Activities of Daily Living Scale to assess the impact of voluntary motor impairment on functional abilities. These studies will delineate those aspects of voluntary motor performance most affected in HD and lead to a better understanding of the anatomical correlates of voluntary motor activity in general.

过去的努力集中在开发一个特征良好的队列 亨廷顿痴呆综合症纵向研究的患者 疾病（HD）。 为此，80例HD患者和22例对照组 受试者已被招募。 在接下来的五年中，患者样本 早期和晚期临床核心的患者将增强。 我们将遵循150例患者和40个基因阴性对照组的队列 我们的纵向核心研究，以首次表征 高清痴呆症的自然史。 这项研究是一项合作的努力 在这个项目之间。 神经影像学项目和临床核心。 这 这项纵向研究的认知数据将进行分析 随着疾病严重程度的年度评估，临床索引 同一患者一年一次获得的措施（神经影像学）。 这 认知能力下降的模式和速率将被检查，相对 严重程度和纹状体和皮质萎缩率的贡献，CAG 重复长度（振动）和开始年龄的下降率差异 将确定。 描述痴呆自然史的描述 高清将对临床医生在管理患者护理方面有帮助，并且将是 与潜在治疗的未来临床试验特别相关 代理商。 自愿运动障碍在HD中很明显，但尚未检查 广泛。 损害的严重程度在患者中似乎有所不同， 跨电机任务。 自愿性运动性能将在温和的情况下进行评估 使用运动障碍量表中适度影响的患者 定量的神经检查，肢体失调电池，运动学习 任务以及评估电动机的定时编程和测序的任务 动作。 运动任务性能将与皮质和 皮质下萎缩以确定受损的解剖学相关性 高清自愿运动，以及亨廷顿氏病活动 日常生活规模，以评估自愿运动障碍对 功能能力。 这些研究将描述 自愿性运动性能在高清方面受到影响最大，并导致更好 了解自愿运动活动的解剖学相关性 一般的。