Departments of Psychiatry, Neurology, Neurosciences, and Psychology, The University of Iowa Roy J. and Lucille A. Carver College of Medicine, Research, Iowa City, IA, 52242-1000, USA.
Curr Neurol Neurosci Rep. 2011 Oct;11(5):474-83. doi: 10.1007/s11910-011-0215-x.
Cognition has been well characterized in the various stages of Huntington disease (HD) as well as in the prodrome before the motor diagnosis is given. Although the clinical diagnosis of HD relies on the manifestation of motor abnormalities, the associated impairments have been growing in prominence for several reasons. First, research to understand the most debilitating aspects of HD has suggested that cognitive and behavioral changes place the greatest burden on families, are most highly associated with functional decline, and can be predictive of institutionalization. Second, cognitive impairments are evident at least 15 years prior to the time at which motor diagnosis is given. Finally, cognitive decline is associated with biological markers such as brain atrophy, circulating levels of brain-derived neurotrophic factors, and insulin-like growth factor 1. Efforts are now underway to develop valid and reliable measures of cognition in the prodrome as well as in all stages of HD so that clinical trials can be conducted using cognitive outcomes.
认知在亨廷顿病（HD）的各个阶段以及在运动诊断之前的前驱期都有很好的特征。尽管 HD 的临床诊断依赖于运动异常的表现，但由于以下几个原因，相关的障碍越来越受到关注。首先，研究表明，认知和行为的变化对家庭造成最大的负担，与功能下降的关联度最高，并且可以预测住院治疗。其次，认知障碍至少在运动诊断前 15 年就已经出现。最后，认知衰退与脑萎缩等生物标志物有关，循环中的脑源性神经营养因子和胰岛素样生长因子 1 的水平也与认知衰退有关。目前正在努力开发前驱期和 HD 所有阶段的认知有效且可靠的测量方法，以便使用认知结果进行临床试验。
