Department of Neuropsychiatry, Huntington Center South, kbo-Isar-Amper-Klinikum, Taufkirchen (Vils), Germany.
Department of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic.
Arch Clin Neuropsychol. 2021 Jan 15;36(1):74-86. doi: 10.1093/arclin/acaa038.
Cognitive decline is a key characteristic of Huntington's disease (HD). This study aimed to investigate the diagnostic accuracy of a cognitive battery with six tests used by most HD research centers to assess cognitive impairment in HD.
In total, 106 HD patients in different disease stages with more (HD-CD, N = 30) and less cognitive impairments (HD-NC, N = 70) and 100 healthy controls (NC) were matched by age, sex, and education and were examined using a standardized protocol including cognitive, motor, and functional assessments.
One-way between-groups analysis of variance showed that controls performed significantly better than HD patients and that HD-NC significantly outperformed HD-CD patients in all cognitive tests (NC > HD-NC > HD-CD), with all Games-Howell post-hoc tests p < .001. Analyses using area under the receiver-operating characteristic curve (AUC) disclosed the diagnostic accuracy of all tests included in the battery to discriminate between NC and HD patients with AUC ranging from 0.809 to 0.862 (all p < .001) and between HD-CD and HD-NC patients with AUC ranging from 0.833 to 0.899 (all p < .001). In both analysis, Stroop Color Naming Test showed the highest discriminative potential. Additional analyses showed that cognitive deficits in all domains progressed with disease duration. Moreover, cognitive performance correlated with the severity of motor and functional impairment (all p < .001) and with the Disease Burden Score regardless of disease duration and age.
Our results indicate that the cognitive battery is a suitable tool for assessing cognitive impairment in HD.
认知能力下降是亨廷顿病（HD）的一个关键特征。本研究旨在调查由大多数 HD 研究中心用于评估 HD 认知障碍的六项测试组成的认知测试组合的诊断准确性。
共有 106 名处于不同疾病阶段的 HD 患者（HD-CD，N=30 名）和认知障碍较少的患者（HD-NC，N=70 名）以及 100 名健康对照者（NC），他们按年龄、性别和教育程度相匹配，并使用包括认知、运动和功能评估的标准化方案进行检查。
单向组间方差分析显示，对照组的表现明显优于 HD 患者，HD-NC 在所有认知测试中的表现均明显优于 HD-CD 患者（NC>HD-NC>HD-CD），所有 Games-Howell 事后检验 p<0.001。使用接受者操作特征曲线（AUC）下面积的分析显示，电池中包含的所有测试区分 NC 和 HD 患者的诊断准确性，AUC 范围从 0.809 到 0.862（均 p<0.001），区分 HD-CD 和 HD-NC 患者的 AUC 范围从 0.833 到 0.899（均 p<0.001）。在这两种分析中，Stroop 颜色命名测试显示出最高的区分潜力。额外的分析表明，所有领域的认知缺陷都随着疾病持续时间的延长而进展。此外，认知表现与运动和功能障碍的严重程度相关（均 p<0.001），并且与疾病负担评分相关，无论疾病持续时间和年龄如何。
我们的结果表明，认知测试组合是评估 HD 认知障碍的合适工具。
