The Japanese journal of neuropsychology

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ArticleTitle	Genetics and Social Cognition in Williams Syndrome and Fragile X Syndrome
Language	J
AuthorList	Masanori Nagamine¹⁾, Masaru Mimura²⁾, Allan L. Reiss³⁾, Fumiko Hoeft³⁾
Affiliation	¹⁾Department of Psychiatry, Japan Self Defense Force Fukuoka Hospital ²⁾Department of Neuropsychiatry, Showa University School of Medicine ³⁾Center for Interdisciplinary Brain Science Research (CIBSR), Stanford University School of Medicine
Publication	Japanese Journal of Neuropsychology: 26 (2), 104-117, 2010
Received
Accepted
Abstract	Among various approaches to investigate "social cognition", understanding the links between core clinical symptoms of developmental disorders and their neural bases could be particularly informative. In this review, we focus on two genetically confirmed developmental disorders, i.e., Williams syndrome and fragile X syndrome. Williams syndrome is a genetic disease, which presents itself with cardiovascular malformation and intellectual disability, and is characterized by atypical 'hypersociality'. On the other hand, fragile X syndrome, another genetic disorder, is known to present autism-like symptoms as well as intellectual disability. Within each condition, affected individuals share the same genetic risk factors for behavioral and cognitive dysfunction. Therefore, investigating individuals with these syndromes can reduce variance associated with etiologic heterogeneity and provides us with a unique opportunity to understand gene-brain-cognition relationships. In this article, we review Williams syndrome and fragile X syndrome, two disorders that present contrasting clinical features of 'sociability', and discuss the possible neural basis of social cognition.
Keywords	social cognition, brain imaging, developmental disorder, Williams syndrome, fragile X syndrome