Mild cognitive impairment in prediagnosed Huntington disease
Mild cognitive impairment in prediagnosed Huntington disease
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DOI：
10.1212/wnl.0b013e3181eccfa2
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发表时间：
2010-08-10
期刊：
影响因子：
9.9
通讯作者：
Harrington, D. L.
中科院分区：
文献类型：
--
作者：
Duff, K.;Paulsen, J.;Harrington, D. L.
背景：在亨廷顿病（HD）中，以及在诊断运动症状之前的时期（即，pre-HD）。然而，认知困难的严重程度，频率和特征尚未得到很好的描述。将类似的截止值应用于轻度认知障碍（MCI）研究中，目前的研究检查了轻微认知功能障碍（e.例如，在一个实施例中，方法：使用来自160名非基因扩展比较参与者的基线数据，建立情景记忆、处理速度、执行功能和视觉空间感知的认知测试的规范数据。在1.5个标准差低于对照组的平均值的截止分数，然后应用于575个基因扩展的前HD参与者的观察性研究，预测-HD，谁是分层的运动体征和遗传风险HD。结果：近40%的前HD个人符合MCI的标准，和个人更接近HD诊断有更高的MCI率。非遗忘型MCI较遗忘型MCI多见。单域MCI较多域MCI多见。在nonamnestic的单域亚型，在处理速度的损害是最频繁的。结论：与阿尔茨海默病文献一致，MCI作为前驱期是一个有效的概念，在HD前，近40%的个人显示这种程度的损害诊断前。未来的研究应该检查MCI作为HD前认知功能下降的标志物的效用。神经病学（R）2010; 75：500-507
Background: Cognitive decline has been reported in Huntington disease (HD), as well as in the period before diagnosis of motor symptoms (i.e., pre-HD). However, the severity, frequency, and characterization of cognitive difficulties have not been well-described. Applying similar cutoffs to those used in mild cognitive impairment (MCI) research, the current study examined the rates of subtle cognitive dysfunction (e. g., dysfunction that does not meet criteria for dementia) in pre-HD.Methods: Using baseline data from 160 non-gene-expanded comparison participants, normative data were established for cognitive tests of episodic memory, processing speed, executive functioning, and visuospatial perception. Cutoff scores at 1.5 standard deviations below the mean of the comparison group were then applied to 575 gene-expanded pre-HD participants from the observational study, PREDICT-HD, who were stratified by motor signs and genetic risk for HD.Results: Nearly 40% of pre-HD individuals met criteria for MCI, and individuals closer to HD diagnosis had higher rates of MCI. Nonamnestic MCI was more common than amnestic MCI. Single-domain MCI was more common than multiple-domain MCI. Within the nonamnestic single-domain subtype, impairments in processing speed were most frequent.Conclusions: Consistent with the Alzheimer disease literature, MCI as a prodromal period is a valid concept in pre-HD, with nearly 40% of individuals showing this level of impairment before diagnosis. Future studies should examine the utility of MCI as a marker of cognitive decline in pre-HD. Neurology (R) 2010; 75: 500-507