Williams Janet K, Kim Ji-In, Downing Nancy, Farias Sarah, Harrington Deborah L, Long Jeffrey D, Mills James A, Paulsen Jane S
College of Nursing, University of Iowa.
Department of Psychiatry, Carver College of Medicine, University of Iowa.
Neuropsychology. 2015 Mar;29(2):255-67. doi: 10.1037/neu0000102. Epub 2014 Jul 7.
Assessment of daily functions affected by cognitive loss in prodromal Huntington's disease (HD) is necessary in practice and clinical trials. We evaluated baseline and longitudinal sensitivity of the Everyday Cognition (ECog) scales in prodromal HD and compared self- and companion-ratings.
Everyday cognition was self-assessed by 850 participants with prodromal HD and 768 companions. We examined internal structure using confirmatory factor analysis (CFA) on baseline data. For longitudinal analysis, we stratified participants into Low, Medium, and High disease progression groups. We examined ECog scores for group differences and participant-and-companion differences using linear mixed effects regression (LMER). Comparison with the Total Functional Capacity (TFC) scale was made.
CFA revealed good fit of a 5-factor model having a global factor (total score), and subfactors (subscales) of memory, language, visuospatial perception, and executive function. At study entry, participants and companions in the Medium and High groups reported significantly worsened everyday cognition as well as significant functional decline over time. Losses became more pronounced and participant and companion ratings diverged as individuals progressed. TFC showed significant functional loss over time in the High group but not in the Medium group.
Disease progression is associated with reduced self- and companion-reported everyday cognition in prodromal HD participants who are less than 13 years to estimated motor onset. Our findings suggest companion ratings are more sensitive than participants' for detecting longitudinal change in daily cognitive function. ECog appears more sensitive to specific functional changes in the prodrome of HD than the TFC.
在临床实践和临床试验中，评估前驱期亨廷顿舞蹈症（HD）中受认知功能减退影响的日常功能很有必要。我们评估了前驱期HD患者日常认知（ECog）量表的基线敏感性和纵向敏感性，并比较了自评和他人评分数值。
850名前驱期HD患者及其768名同伴进行了日常认知自评。我们使用验证性因子分析（CFA）对基线数据进行内部结构分析。在纵向分析中，我们将参与者分为疾病进展低、中、高三个组。我们使用线性混合效应回归（LMER）研究ECog分数的组间差异以及参与者与同伴之间的差异。并与总功能能力（TFC）量表进行比较。
CFA显示，一个包含全局因子（总分）以及记忆、语言、视觉空间感知和执行功能等子因子（子量表）的五因子模型具有良好拟合度。在研究开始时，中、高组的参与者和同伴报告日常认知显著恶化，且随着时间推移功能显著下降。随着个体病情进展，功能丧失变得更加明显，参与者和同伴的评分出现分歧。TFC显示，高疾病进展组随时间推移功能显著丧失，而中疾病进展组则不然。
在预计运动症状出现前13年以内的前驱期HD患者中，疾病进展与自评和同伴报告的日常认知功能减退相关。我们的研究结果表明，在检测日常认知功能的纵向变化方面，同伴评分比参与者评分更敏感。与TFC相比，ECog似乎对HD前驱期的特定功能变化更敏感。
