Visual detection and integration in Williams Syndrome

描述(由申请人提供)：我建议描述患有威廉姆斯综合征(WS)的儿童和成人以及发育正常的儿童的基本视觉能力。威廉姆斯综合征是一种罕见的遗传性疾病，具有独特的认知特征：语言能力相对较强，但视觉空间认知能力严重不足。这个轮廓非常有趣，因为它表明遗传缺陷可能针对特定的认知系统，在这种情况下，空间认知系统。到目前为止，大多数关于WS的研究都使用了复杂的视觉运动任务，如绘画或积木结构来评估他们的视觉空间能力。一些人认为，这些缺陷与立体视觉和敏锐度等“低水平”视觉功能无关。然而，人们对WS在基本视觉任务中的能力知之甚少。我计划评估WS的空间认知缺陷与重要的视觉功能有关的可能性，这些功能允许在空间上整合特征。利用心理物理任务，我计划观察WS和正常对照如何检测单个对象(例如，检测不同空间频率的栅格)以及它们如何整合多个对象(例如，检测由多个栅格组成的轮廓)。这些研究将使我们更好地理解简单的视觉功能如何导致WS的复杂空间认知缺陷，并可能为改善WS缺陷的可能疗法提供洞察力。此外，他们还将揭示正常儿童特征检测和特征整合的发展轨迹。

DESCRIPTION (provided by applicant): I propose to characterize the basic visual abilities of children and adults with Williams Syndrome (WS), and of normally developing children. Williams Syndrome is a rare genetic disorder that has a distinct cognitive profile: a relative strength in language, but a profound weakness in visual-spatial cognition. This profile is of great interest because it suggests the possibility that genetic deficit might target specific cognitive systems, in this case, the system of spatial cognition. To date, most studies with WS have used complex visuo-motor tasks such as drawing or block construction to evaluate their visual-spatial abilities. Some have suggested that these deficits are not linked to "low-lever' visual functions such as stereopsis and acuity. However, very little is known about the abilities of WS in basic visual tasks. I plan to evaluate the possibility that the spatial cognitive deficits of WS are linked to important visual functions that allow integration of features over space. Using psychophysical tasks, I plan to look at how WS and normal controls detect a single object (e.g. detecting a grating at various spatial frequencies) as well as how they integrate multiple objects (e.g. detecting a contour made up of many gratings). These studies will give us a better understanding of how simple visual functions may contribute to complex spatial cognition deficits in WS, and perhaps provide insight into possible therapies that could ameliorate WS deficits. Furthermore, they will uncover the developmental trajectory of feature detection and feature integration in normal children.