Lunven Marine, Hernandez Dominguez Karen, Youssov Katia, Hamet Bagnou Jennifer, Fliss Rafika, Vandendriessche Henri, Bapst Blanche, Morgado Graça, Remy Philippe, Schubert Robin, Reilmann Ralf, Busse Monica, Craufurd David, Massart Renaud, Rosser Anne, Bachoud-Lévi Anne-Catherine
Département d'Etudes Cognitives, École normale supérieure, PSL University, 75005 Paris, France.
University Paris Est Creteil, INSERM U955, Institut Mondor de Recherche Biomédicale, Equipe NeuroPsychologie Interventionnelle, F-94010 Creteil, France.
Brain Commun. 2023 Mar 6;5(2):fcad043. doi: 10.1093/braincomms/fcad043. eCollection 2023.
Cognitive deficits represent a hallmark of neurodegenerative diseases, but evaluating their progression is complex. Most current evaluations involve lengthy paper-and-pencil tasks which are subject to learning effects dependent on the mode of response (motor or verbal), the countries' language or the examiners. To address these limitations, we hypothesized that applying neuroscience principles may offer a fruitful alternative. We thus developed the SelfCog, a digitized battery that tests motor, executive, visuospatial, language and memory functions in 15 min. All cognitive functions are tested according to the same paradigm, and a randomization algorithm provides a new test at each assessment with a constant level of difficulty. Here, we assessed its validity, reliability and sensitivity to detect decline in early-stage Huntington's disease in a prospective and international multilingual study (France, the UK and Germany). Fifty-one out of 85 participants with Huntington's disease and 40 of 52 healthy controls included at baseline were followed up for 1 year. Assessments included a comprehensive clinical assessment battery including currently standard cognitive assessments alongside the SelfCog. We estimated associations between each of the clinical assessments and SelfCog using Spearman's correlation and proneness to retest effects and sensitivity to decline through linear mixed models. Longitudinal effect sizes were estimated for each cognitive score. Voxel-based morphometry and tract-based spatial statistics analyses were conducted to assess the consistency between performance on the SelfCog and MRI 3D-T1 and diffusion-weighted imaging in a subgroup that underwent MRI at baseline and after 12 months. The SelfCog detected the decline of patients with Huntington's disease in a 1-year follow-up period with satisfactory psychometric properties. Huntington's disease patients are correctly differentiated from controls. The SelfCog showed larger effect sizes than the classical cognitive assessments. Its scores were associated with grey and white matter damage at baseline and over 1 year. Given its good performance in longitudinal analyses of the Huntington's disease cohort, it should likely become a very useful tool for measuring cognition in Huntington's disease in the future. It highlights the value of moving the field along the neuroscience principles and eventually applying them to the evaluation of all neurodegenerative diseases.
认知缺陷是神经退行性疾病的一个标志，但评估其进展情况很复杂。目前大多数评估都涉及冗长的纸笔任务，这些任务会受到学习效应的影响，而学习效应取决于反应方式（运动或言语）、国家语言或考官。为了解决这些局限性，我们假设应用神经科学原理可能会提供一个富有成效的替代方案。因此，我们开发了SelfCog，这是一种数字化测试组合，可在15分钟内测试运动、执行、视觉空间、语言和记忆功能。所有认知功能均按照相同的范式进行测试，并且随机化算法会在每次评估时提供难度恒定的新测试。在此，我们在一项前瞻性的国际多语言研究（法国、英国和德国）中评估了它在检测早期亨廷顿舞蹈病衰退方面的有效性、可靠性和敏感性。85名亨廷顿舞蹈病患者中有51名以及基线时纳入的52名健康对照者中有40名接受了为期1年的随访。评估包括一套全面的临床评估组合，其中包括目前的标准认知评估以及SelfCog。我们使用斯皮尔曼相关性估计了各项临床评估与SelfCog之间的关联，以及通过线性混合模型对重测效应的倾向和对衰退的敏感性。估计了每个认知分数的纵向效应大小。对在基线和12个月后接受MRI检查的一个亚组进行了基于体素的形态测量和基于纤维束的空间统计分析，以评估SelfCog表现与MRI 3D-T1及扩散加权成像之间的一致性。SelfCog在1年的随访期内检测到了亨廷顿舞蹈病患者的衰退情况，其心理测量特性令人满意。亨廷顿舞蹈病患者与对照者能够被正确区分。SelfCog显示出比传统认知评估更大的效应大小。其分数与基线时以及1年期间的灰质和白质损伤相关。鉴于其在亨廷顿舞蹈病队列纵向分析中的良好表现，它未来很可能会成为测量亨廷顿舞蹈病认知的非常有用的工具。它突出了按照神经科学原理推动该领域发展并最终将其应用于所有神经退行性疾病评估的价值。
