NEUROPSYCHOLOGY OF HUNTINGTONS DISEASE

过去的努力集中在发展一批具有良好特征的 亨廷顿痴呆症患者的纵向研究 疾病(HD)。为此，80名HD患者和22名对照组 受试者已被招募。在接下来的五年里，患者样本 将增加早发和晚发患者的临床核心。 我们将跟踪150名患者和40名基因阴性对照的队列 我们的纵向核心研究首次表征了 先天性痴呆症的自然病史。这项研究是一项合作努力。 在这个项目之间。神经成像项目和临床核心。这个 这项纵向研究的认知数据将结合分析 以临床为索引的疾病严重程度年度评估 对同一患者进行每两年一次的测量(神经成像)。这个 将检查认知下降的模式和速度，以及相对的 纹状体和皮质萎缩的严重程度和比率的贡献 重复长度(核心)、发病年龄对下降率的差异 将会被确定。阿尔茨海默病自然病史的勾画 HD将有助于临床医生管理患者护理，并将 尤其与潜在治疗方法的未来临床试验有关 探员们。 自发性运动障碍在HD患者中很突出，但尚未进行检查 广泛地。损伤的严重程度似乎因患者和 不同的运动任务。自愿的运动能力将被温和地评估。 对中度患者使用运动障碍量表从 量化神经学检查，肢体失用电池，运动学习 任务，以及评估电机定时编程和排序的任务 行为。运动任务表现将与大脑皮层和 皮质下萎缩确定受损患者的解剖学相关性 HD的自愿行动，以及亨廷顿病的活动 日常生活能力量表评估自愿运动障碍对生活质量的影响 功能能力。这些研究将勾勒出以下方面 自主性运动能力在HD中最受影响并导致更好的 对成人自主运动的解剖学相关性的认识 将军。

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.