Journey from Cognition to Brain to Gene

Perspectives from Williams Syndrome

A blueprint for the investigation of neurodevelopmental disorders, this book presents the work of a team of scientists using a multidisciplinary, integrated approach to link genes with human behavior. Using Williams syndrome as a model, leading researchers in neuroanatomy, neurocognition, neurophysiology, and molecular genetics have built bridges between disciplines to link higher cognitive functions, their underlying neurobiological bases, and their molecular genetic underpinnings. One of the book's many strengths is that the scientists from each discipline studied the same individuals with Williams syndrome. As the book shows, Williams syndrome is a fascinating disorder because of the "peaks and valleys" among cognitive domains: severe intellectual deficits but remarkably spared and effusive language; specific impairment in spatial construction but great strength in face processing and sociability. By capitalizing on these dissociations in higher cognitive functioning, the book provides a model for the study of brain-behavior relationships as well as for the mapping of brain and behavior phenotypes to the genome and beyond.

Contributors: Ralph Adolphs, Twyla Alvarez, Lawrence Appelbaum, Ursula Bellugi, Dennis Burian, Xiao-Ning Chen, Michael Chiles, Stephan Eliez, Albert Galaburda, Hanao Hirota, Wendy Jones, Julie Korenberg, Zona Lai, Liz Lichtenberger, Alan Lincoln, Rumiko Matsuoka, Debra Mills, Helen Neville, Judy Reilly, Allan Reiss, Bruce Roe, Marie St. George, J. Eric Schmidtt, Erica Straus.

Table of Contents

  1. Contributors
  2. Preface
  3. 1. The Neurocognitive Profile of Williams Syndrome: A Complex Pattern of Strengths and Weaknesses

    Ursula Bellugi, Liz Lichtenberger, Wendy Jones, Zona Lai, and Marie St. George

  4. 2. Hypersociability: The Social and Affective Phenotype of Williams Syndrome

    Wendy Jones, Ursula Bellugi, Zona Lai, Michael Chiles, Judy Reilly, Alan Lincoln, and Ralph Adolphs

  5. 3. Neurophysiological Markers of face Processing in Williams Syndrome

    Debra L. Mills, Twyla D. Alvarez, Marie St. George, Lawrence G. Appelbaum, Ursula Bdellugi, and Helen Neville

  6. 4. Neuroanatomy of Williams Syndrome: A High-Resolution MRI Study

    Allan L. Reiss, Stephan Eliez, J. Eric Schmitt, Erica Straus, Zona Lai, Wendy Jones, and Ursula Bellugi

  7. 5. Cellular and Molecular Cortical Neuroanatomy i Williams Syndrome

    Albert M. Galaburda and Ursula Bellugi

  8. 6. Genome Structure and Cognitive Map of Williams Syndrome

    Julie R. Korenberg, Xiao-Ning Chen, Hamao Hirota, Zona Lai, Ursula Bellugi, Dennis Burian, Bruce Roe, and Rumiko Matsuoka

  9. Epilogue
  10. Editors and Authors
  11. Index
  12. Epilogue
  13. Editors and Authors
  14. Index