Département d'Etudes Cognitives, Ecole Normale Supérieure, PSL University, Paris, France; Université Paris Est, Faculté de Médecine, Créteil, France; Inserm U955, Equipe E01 Neuropsychologie Interventionnelle, Créteil, France; AP-HP, Centre de référence Maladie de Huntington, Service de Neurologie, Hôpital Henri Mondor-Albert Chenevier, Créteil, France; NeurATRIS, Creteil, France.
Département d'Etudes Cognitives, Ecole Normale Supérieure, PSL University, Paris, France; Université Paris Est, Faculté de Médecine, Créteil, France; Inserm U955, Equipe E01 Neuropsychologie Interventionnelle, Créteil, France; AP-HP, Centre de référence Maladie de Huntington, Service de Neurologie, Hôpital Henri Mondor-Albert Chenevier, Créteil, France; NeurATRIS, Creteil, France.
Neuroimage Clin. 2021;32:102865. doi: 10.1016/j.nicl.2021.102865. Epub 2021 Oct 27.
Time processing over intervals of hundreds of milliseconds to minutes, also known as interval timing, is associated with the striatum. Huntington's disease patients (HD) with striatal degeneration have impaired interval timing, but the extent and specificity of these deficits remain unclear. Are they specific to the temporal domain, or do they extend to the spatial domain too? Do they extend to both the perception and production of interval timing? Do they appear before motor symptoms in Huntington's disease (Pre-HD)? We addressed these issues by assessing both temporal abilities (in the seconds range) and spatial abilities (in the cm range) in 20 Pre-HD, 25 HD patients, and 25 healthy Controls, in discrimination, bisection and production paradigms. In addition, all participants completed a questionnaire assessing temporal and spatial disorientation in daily life, and the gene carriers (i.e., HD and Pre-HD participants) underwent structural brain MRI. Overall, HD patients were more impaired in the temporal than in the spatial domain in the behavioral tasks, and expressed a greater disorientation in the temporal domain in the daily life questionnaire. In contrast, Pre-HD participants showed no sign of a specific temporal deficit. Furthermore, MRI analyses indicated that performances in the temporal discrimination task were associated with a larger striatal grey matter volume in the striatum in gene carriers. Altogether, behavioral, brain imaging and questionnaire data support the hypothesis that the striatum is a specific component of interval timing processes. Evaluations of temporal disorientation and interval timing processing could be used as clinical tools for HD patients.
时间在数百毫秒到数分钟之间的处理，也称为间隔计时，与纹状体有关。纹状体退化的亨廷顿病（HD）患者的间隔计时能力受损，但这些缺陷的程度和特异性仍不清楚。它们是特定于时间域的，还是也延伸到空间域？它们是否延伸到间隔计时的感知和产生？它们是否出现在亨廷顿病（Pre-HD）的运动症状之前？我们通过评估 20 名 Pre-HD、25 名 HD 患者和 25 名健康对照者在区分、二分法和产生范式中的时间能力（秒范围内）和空间能力（cm 范围内），来解决这些问题。此外，所有参与者都完成了一份评估日常生活中时间和空间定向障碍的问卷，而基因携带者（即 HD 和 Pre-HD 参与者）则接受了结构脑 MRI 检查。总体而言，HD 患者在行为任务中在时间域的表现比在空间域更差，并且在日常生活问卷中表现出更大的时间定向障碍。相比之下，Pre-HD 参与者没有表现出特定的时间缺陷迹象。此外，MRI 分析表明，在时间辨别任务中的表现与基因携带者纹状体的纹状体灰质体积较大有关。总之，行为、脑成像和问卷数据支持纹状体是间隔计时过程的特定组成部分的假设。对时间定向障碍和间隔计时处理的评估可以作为 HD 患者的临床工具。
