title: Neural systems for social cognition in Klinefelter syndrome (47,XXY): evidence from fMRI.
abstract: Klinefelter syndrome (KS) is a chromosomal condition (47, XXY) that may help us to unravel gene-brain behavior pathways to psychopathology. The phenotype includes social cognitive impairments and increased risk for autism traits. We used functional MRI to study neural mechanisms underlying social information processing. Eighteen nonclinical controls and thirteen men with XXY were scanned during judgments of faces with regard to trustworthiness and age. While judging faces as untrustworthy in comparison to trustworthy, men with XXY displayed less activation than controls in (i) the amygdala, which plays a key role in screening information for socio-emotional significance, (ii) the insula, which plays a role in subjective emotional experience, as well as (iii) the fusiform gyrus and (iv) the superior temporal sulcus, which are both involved in the perceptual processing of faces and which were also less involved during age judgments in men with XXY. This is the first study showing that KS can be associated with reduced involvement of the neural network subserving social cognition. Studying KS may increase our understanding of the genetic and hormonal basis of neural dysfunctions contributing to abnormalities in social cognition and behavior, which are considered core abnormalities in psychiatric disorders such as autism and schizophrenia.
authors: Leiden University, Faculty of Social Sciences, Clinical Child and Adolescent Studies, Wassenaarseweg 52, 2333 AK, Leiden, The Netherlands. srijn@fsw.leidenuniv.nl
authors: Sophie
authors: S
authors: van Rijn
authors: Hanna
authors: H
authors: Swaab
authors: Daan
authors: D
authors: Baas
authors: Edward
authors: E
authors: de Haan
authors: René S
authors: RS
authors: Kahn
authors: André
authors: A
authors: Aleman
mesh_terms: D000328:Adult; D001288:Attention / Q000502:physiology; D001921:Brain / Q000098:blood supply* / Q000473:pathology; D001931:Brain Mapping; D003072:Cognition Disorders / Q000209:etiology* / Q000473:pathology*; D005145:Face; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007600:Judgment / Q000502:physiology; D007713:Klinefelter Syndrome / Q000150:complications* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D010100:Oxygen / Q000097:blood; D010775:Photic Stimulation; D012720:Severity of Illness Index; D012919:Social Behavior; D055815:Young Adult
chemical_list: D010100:Oxygen