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症的儿童和成人以及正常发育的儿童的基本视觉能力。威廉姆斯综合症是一种罕见的遗传性疾病，它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。这一特征非常有趣，因为它表明基因缺陷可能针对特定的认知系统，在这种情况下，是空间认知系统。迄今为止，大多数关于WS的研究都使用复杂的视觉运动任务，如绘画或积木构建来评估他们的视觉空间能力。一些人认为，这些缺陷与“低水平”视觉功能（如立体视觉和灵敏度）无关。然而，我们对WS在基本视觉任务中的能力知之甚少。我计划评估WS的空间认知缺陷是否与重要的视觉功能有关，这些视觉功能允许在空间上整合特征。使用心理物理任务，我计划看看WS和正常控制如何检测单个对象（例如检测各种空间频率的光栅）以及它们如何集成多个对象（例如检测由许多光栅组成的轮廓）。这些研究将使我们更好地了解简单的视觉功能如何导致WS患者复杂的空间认知缺陷，并可能为改善WS缺陷的可能治疗方法提供见解。此外，他们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.