An event related functional magnetic resonance imaging (fMRI) study of cognitive deficits in Huntington's disease

亨廷顿氏病（HD）是一种进行性疾病，其临床特征在于不自主的急动、自主运动受损、认知-注意力恶化和精神症状的发作。这是进行性和选择性脑退化的结果。在过去的十年中，我们的研究单位在描述HD的认知缺陷方面取得了重大进展;然而，我们仍然不太了解这些缺陷背后的大脑部分。该项目的目的是使用事件相关的功能性磁共振成像（fMRI），通过使用各种认知行为措施来关联大脑中的结构和功能变化。通过这种方式，我们可以阐明哪些大脑区域负责各种认知功能。此外，我们打算确定不同的大脑区域如何随着时间的推移而减少功能，并确定疾病进展速度，发病年龄和某些遗传特征之间是否存在关系。从患者管理的角度来看，当新的药物治疗最终能够预防疾病的发展或减缓疾病的进一步发展时，了解大脑的功能以及疾病如何随着时间的推移而发展将是至关重要的。

Huntington's disease (HD) is a progressive disorder clinically characterized by the onset of involuntary jerky movements, impaired voluntary movements, cognitive-attentional deterioration, and psychiatric symptoms. It is a consequence of progressive and selective brain degeneration. Over the last decade our research unit has made significant progress in describing the cognitive deficits in HD; however, we still don't know much about the parts of the brain that underlie these deficits. The aim of this project is to use event related functional magnetic resonance imaging (fMRI) to correlate structural and functional changes in the brain via the use of various cognitive behavioural measures. In this way we can elucidate what brain areas are responsible for various cognitive functions. In addition, we intend to ascertain how different brain areas decrement in function over time and to determine whether there is a relationship between rate of disease progression, age at onset and certain genetic characteristics. Understanding how the brain functions and how the disease progresses over time will be essential, from the point of view of patient management, when new drug treatments eventually permit prevention of the development, or slows further progress, of the disorder.