Department of Cognitive Science, Budapest University of Technology and Economics, Budapest, Hungary.
PLoS One. 2012;7(7):e40282. doi: 10.1371/journal.pone.0040282. Epub 2012 Jul 5.
Williams Syndrome is a genetically determined neurodevelopmental disorder characterized by an uneven cognitive profile and surprisingly large neurobehavioral differences among individuals. Previous studies have already shown different forms of memory deficiencies and learning difficulties in WS. Here we studied the capacity of WS subjects to improve their performance in a basic visual task. We employed a contour integration paradigm that addresses occipital visual function, and analyzed the initial (i.e. baseline) and after-learning performance of WS individuals. Instead of pooling the very inhomogeneous results of WS subjects together, we evaluated individual performance by expressing it in terms of the deviation from the average performance of the group of typically developing subjects of similar age. This approach helped us to reveal information about the possible origins of poor performance of WS subjects in contour integration. Although the majority of WS individuals showed both reduced baseline and reduced learning performance, individual analysis also revealed a dissociation between baseline and learning capacity in several WS subjects. In spite of impaired initial contour integration performance, some WS individuals presented learning capacity comparable to learning in the typically developing population, and vice versa, poor learning was also observed in subjects with high initial performance levels. These data indicate a dissociation between factors determining initial performance and perceptual learning.
威廉姆斯综合征是一种由基因决定的神经发育障碍,其特征是认知水平不均衡,个体之间的神经行为差异显著。先前的研究已经表明,WS 患者存在不同形式的记忆缺陷和学习困难。在这里,我们研究了 WS 患者在一项基本视觉任务中提高表现的能力。我们采用了一种轮廓整合范式来评估枕叶视觉功能,并分析了 WS 个体的初始(即基线)和学习后的表现。我们没有将 WS 患者非常不均匀的结果混为一谈,而是通过将其表现与具有相似年龄的正常发育个体的组平均表现的偏差来评估个体的表现。这种方法帮助我们揭示了有关 WS 患者在轮廓整合中表现不佳的可能起源的信息。尽管大多数 WS 患者的基线和学习表现都降低了,但个体分析也揭示了几个 WS 患者的基线和学习能力之间存在分离。尽管初始轮廓整合表现受损,但一些 WS 患者的学习能力与正常发育人群的学习能力相当,反之亦然,在初始表现水平较高的患者中也观察到了较差的学习能力。这些数据表明,决定初始表现和知觉学习的因素之间存在分离。