Our experience of the spatial world is a unitary one; we perceive objects and layouts, we remember them and act on them, and we can even talk about them with ease. Despite this impression of seamlessness, spatial representations in human adults appear to be specialized in domain-dependent manner, engaging different properties and computational mechanisms for different functions. In this book, the authors present evidence that this domain-specific specialization in cognitive function emerges early in development and is reflected in patterns of breakdown that occur under genetic defect. The authors focus on spatial representation in children and adults with Williams syndrome, a relatively rare genetic syndrome that gives rise to an unusual profile of severely impaired spatial representation together with spared language. Results from a variety of spatial domains -- including object representation, motion perception, action, navigation, and spatial language -- appear to display a strikingly uneven profile of sparing and deficit within spatial representations, consistent with the idea that specialization of function drives development and breakdown. These findings raise a crucial question: Can specific genes target specific aspects of cognitive structure? Looking deeper into the patterns of performance across spatial domains, the book explores the notion that understanding patterns of normal development across domains is crucial to understanding unusual development. Using insights from normal development, the authors propose a speculative hypothesis that explains the emergence of the William syndrome profile, and how complex cognitive outcomes can arise from the deletion of a small set of genes.

Table of Contents:
Chapter 1. The Puzzle of Williams Syndrome ; 1.1 Hallmarks of the WS cognitive profile ; 1.2. Three principles for solving the puzzle ; 1.2.1 How can genes cause cognitive deficits? Complexity of the chain, and the importance of cognition ; 1.2.2 The cognitive architecture of space: The importance of specialization of function ; 1.2.3 Timing matters: The importance of normal development ; 1.3 Summary ; Chapter 2. Background on the problem: Genes, Brains, and the Hallmark Spatial Profile ; 2.1 Genes and the WS profile ; 2.1.1 LIMK1 and the spatial deficit ; 2.2 Brain structure and function ; 2.2.1 Brain structure ; 2.2.2 Brain function ; 2.3 Understanding the block construction task and why it might be so difficult ; 2.3.1 The cognitive requirements of the block construction task ; 2.3.2 Components of the block task ; 2.3.3 Summary of the cognitive components of the block construction task and reflections on possible brain correlates ; 2.4 Towards an hypothesis: Weakness in the dorsal stream/parietal lobe functions, strength in ventral stream functions? ; Chapter 3. Objects ; 3.1 Object recognition and levels of processing in the visual system ; 3.1.1 Levels of visual analysis for objects ; 3.2 Object Recognition in People with Williams Syndrome ; 3.2.1 Early Vision ; 3.2.1.1 A comment about orientation sensitivity ; 3.2.1.2 Summary of evidence on early visual processing ; 3.2.2 Middle Level ; 3.2.3 Vision ; 3.2.3.1 Visual Grouping ; 3.2.3.2 Grouping from motion ; 3.2.3.3 Summary of evidence on grouping ; 3.2.4 High level vision: Object recognition ; 3.2.4.1 Recognizing familiar objects ; 3.2.4.2 A special problem: Handedness, or left-right reflections ; 3.2.4.3 Summary of evidence on object recognition ; 3.3 Face recognition ; 3.3.1 Summary of evidence on face recognition ; 3.4 Summary ; Chapter 4. Objects in Places ; 4.1 Review of the components of the block construction task and their relationship to parietal functions ; 4.2 Marking objects: More than one at a time, but only up to 2 ; 4.3 Locating objects: Constructing and using reference frames ; 4.3.1 Matching locations: Are object locations defined in terms of a reference system? ; 4.3.2 Copying locations: Axes, directions, and spatial precision in location representations ; 4.3.2.1 Copying: Task 1 ; 4.3.2.2 Copying: Task 2 ; 4.3.3 Summary of locating objects: Matching, copying ; 4.4 Acting on objects ; 4.5 Summary of marking, locating, and acting on objects ; Chapter 5. Finding our Way ; 5.1 The components of navigation: Division of labor ; 5.1.1 Egocentric and allocentric reference systems ; 5.1.2 The importance of geometry for allocentric representations ; 5.1.3 The special importance of geometric layouts for re-establishing one's orientation after becoming disoriented ; 5.1.4 Summary of behavioral and neural findings ; 5.2 Navigation in people with WS ; 5.2.1 Oriented navigation: Egocentric and allocentric reference systems ; 5.2.2 Reorientation and the geometric representation of layouts ; 5.3 Summary ; Chapter 6. Space and Language ; 6.1 Structure in Spatial language: Places and Paths ; 6.1.1 Geometric meanings of prepositions and reference object construals ; 6.1.2 A basic fact: The small lexicon of place and path terms results in coarse coding of space by language ; 6.1.3 Spatial language and Williams syndrome ; 6.2. Paths ; 6.2.1 The language of motion events: Figure, reference object, motion, path ; 6.2.2 Overall Results ; 6.2.3 Path expression ; 6.2.4 A follow up study: Bias to express TO paths, bias to omit FROM paths ; 6.2.5 Summary: The language of motion events ; 6.3 Places ; 6.3.1 Production task ; 6.3.1.1 Do axial terms engage axes? ; 6.3.1.2 Do axial terms engage direction within axes? ; 6.3.1.3 What else were they saying? ; 6.3.2 Comprehension task ; 6.3.2.1 Do axial terms engage axes? ; 6.3.2.2 Do axial terms engage direction within axes? ; 6.3.3 Summary: Studies of axial term Production and Comprehension ; 6.4 Beyond concrete physical spatial relationships ; 6.4.1 Using "geometric imagination:>" Matching spatial prepositions and reference objects ; 6.4.2 The study ; 6.4.2.1 Stimuli and procedure ; 6.4.2.2 Results ; 6.4.3 Summary of abstract uses of spatial prepositions ; 6.5 How do findings on spatial language fit with other aspects of language in people with WS ; 6.5.1 Vocabulary onset and growth ; 6.5.2 Spatial vocabulary: findings from other labs ; 6.5.3 Morphosyntax ; 6.5.4 Syntax ; 6.5.4.1 Subject and object-relative clauses ; 6.5.4.2 Hierarchical structure, c-command, and the interaction of <"not>" and <"or ; 6.5.4.3 Binding, Raising, and Passives ; 6.5.5 Summary of pattern for broad aspects of language ; Chapter 7. Conclusions: Revisiting the Puzzle of Williams Syndrome ; 7.1 Specialization of function ; 7.1.1 Specialization of function by domain ; 7.1.1.1 Spatial representation in people with WS embodies rich and highly differentiated structure, as in normal development ; 7.1.1.2 Face perception vs. reorientation ; 7.1.2 Specialization of function by stream of visual processing: Dorsal vs. ventral streams ; 7.2 Developmental timing ; 7.2.1 Two examples ; 7.2.2 Extension to other spatial functions ; 7.2.3 Extension to language ; 7.3 A developmental mechanism: Slow development, arrest at an early functional level ; 7.4 Is that all there is? What slow development and early arrest cannot account for ; 7.4.1 Spatial systems that flourish, spatial systems that fail ; 7.4.2 Cognitive systems that continue to grow ; 7.4.2.1 Vocabulary growth ; 7.4.2.2 Reading and mathematical skill ; 7.4.2.3 Copying ; 7.5 Remaining puzzles ; Appendix A ; Appendix B ; References ; Author Index ; Subject Index