title: Altered microstructure within social-cognitive brain networks during childhood in Williams syndrome.
abstract: Williams syndrome (WS) is a neurodevelopmental condition caused by a hemizygous deletion of ∼26-28 genes on chromosome 7q11.23. WS is associated with a distinctive pattern of social cognition. Accordingly, neuroimaging studies show that WS is associated with structural alterations of key brain regions involved in social cognition during adulthood. However, very little is currently known regarding the neuroanatomical structure of social cognitive brain networks during childhood in WS. This study used diffusion tensor imaging to investigate the structural integrity of a specific set of white matter pathways (inferior fronto-occipital fasciculus [IFOF] and uncinate fasciculus [UF]) and associated brain regions [fusiform gyrus (FG), amygdala, hippocampus, medial orbitofrontal gyrus (MOG)] known to be involved in social cognition in children with WS and a typically developing (TD) control group. Children with WS exhibited higher fractional anisotropy (FA) and axial diffusivity values and lower radial diffusivity and apparent diffusion coefficient (ADC) values within the IFOF and UF, higher FA values within the FG, amygdala, and hippocampus and lower ADC values within the FG and MOG compared to controls. These findings provide evidence that the WS genetic deletion affects the development of key white matter pathways and brain regions important for social cognition.
authors.affiliation: Department of Psychology, University of Georgia, Center for Interdisciplinary Brain Sciences Research (CIBSR), Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, Palo Alto, CA, USA.
authors.forename: Brian W
authors.initials: BW
authors.lastname: Haas
authors.affiliation: Center for Interdisciplinary Brain Sciences Research (CIBSR), Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, Palo Alto, CA, USA.
authors.forename: Naama
authors.initials: N
authors.lastname: Barnea-Goraly
authors.affiliation: Center for Interdisciplinary Brain Sciences Research (CIBSR), Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, Palo Alto, CA, USA.
authors.forename: Kristen E
authors.initials: KE
authors.lastname: Sheau
authors.affiliation: Center for Interdisciplinary Brain Sciences Research (CIBSR), Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, Palo Alto, CA, USA.
authors.forename: Bun
authors.initials: B
authors.lastname: Yamagata
authors.affiliation: Department of Psychology, University of California, Los Angeles, Los Angeles, CA 90095, USA.
authors.forename: Shruti
authors.initials: S
authors.lastname: Ullas
authors.affiliation: Center for Interdisciplinary Brain Sciences Research (CIBSR), Department of Radiology, and Department of Pediatrics, Stanford University School of Medicine, 401 Quarry Rd. Palo Alto, CA 94305-5795, USA.
authors.forename: Allan L
authors.initials: AL
authors.lastname: Reiss
mesh_terms: D000293:Adolescent; D001921:Brain / Q000473:pathology*; D002648:Child; D003072:Cognition Disorders / Q000473:pathology; D056324:Diffusion Tensor Imaging; D004644:Emotions; D005260:Female; D006801:Humans; D008297:Male; D009415:Nerve Net / Q000473:pathology*; D012919:Social Behavior; D066127:White Matter / Q000473:pathology*; D018980:Williams Syndrome / Q000473:pathology*
keywords: DTI; Williams syndrome; genetics; social cognition