Preface v

Contributors ix


1 The Genetics and Genomics of Neurobehavioral Disorders:

Historical Introduction and Overview

Gene S. Fisch 3

2 Neuroanatomical Considerations

Specific to the Study of Neurogenetics

Albert M. Galaburda and J. Eric Schmitt 21

3 Modeling Cognitive Disorders: From Genes to Therapies

Rui M. Costa, Ype Elgersma, and Alcino J. Silva 39

4 What Can the Study of Behavioral Phenotypes Teach Us

About the Pathway from Genes to Behavior?

Jonathan Flint 69


5 The Central Nervous System in Neurofibromatosis Type 1

Nancy Ratner and Kathryn North 97

6 Prader-Willi and Angelman Syndromes:

Cognitive and Behavioral Phenotypes

Elisabeth M. Dykens and Suzanne B. Cassidy 133

7 Tuberous Sclerosis

Julian R. Sampson and Julia C. Lewis 159

8 Behavioral Phenotype in Velo-Cardio-Facial Syndrome

Kieran C. Murphy 195

9 Williams-Beuren Syndrome

Monica Bayés and Luis A. Pérez Jurado 209

10 Behavioral Phenotype in Myotonic Dystrophy (Steinert's Disease)

Jean Steyaert 237

Part III. X-Linked Nonsyndromal Disorders and Neurobehavioral Dysfunction

11 Genetics of X-Linked Mental Retardation

Jamel Chelly and Ben C. J. Hamel 263

12 Nonsyndromal Mental Retardation Associated with the FRAXE Fragile Site and the FMR2 Gene

Jozef Gecz and Gene S. Fisch 289

Part IV. X-Linked Syndromal Disorders and Neurobehavioral Dysfunction

13 ATR-X Syndrome

Takahito Wada and Richard J. Gibbons 309

14 The Fragile X Syndrome and the Fragile X Mutation

Mark C. Hirst and Gene S. Fisch 335

15 Rett Syndrome: Clinical-Molecular Correlates

Alan K. Percy, Joanna Dragich, and N. Carolyn Schanen 391

Index 419

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