title: [Social cognition disorders in Klinefelter syndrome: A specific phenotype? (KS)].
abstract: BACKGROUND
  The Klinefelter syndrome (KS) is a genetic condition characterized by an X supernumerary sex chromosome in males. The syndrome is frequently associated with cognitive impairment. Indeed, the different areas of the executive sphere can be affected such as inhibition, cognitive flexibility but also attentional and visual-spatial domain. Social cognition disorders, predominantly on emotional recognition processes, have also been documented. In addition, the syndrome may be associated with psychiatric symptoms.
  
  
  METHODS
  Our study aims to characterize of the various components of social cognition in the SK: facial emotional recognition, theory of mind and attributional style. For this two groups (SK group versus control group) of participants (n=16) matched for age and sociocultural level were recruited. Participants with intellectual disabilities, psychiatric or neurological disorders were excluded. Three social cognition tests were available: the TREF, the MASC, the AIHQ. Neurocognitive functions were assessed by the fNart, the subtest "logical memory" of the MEM-III, the subtests of the two VOSP battery, the d2, the TMT and the Stroop test.
  
  
  RESULTS
  The SK group had specific social cognition disorders in comparison to the control group. Two emotions in particular were less well recognized: fear and contempt. In addition, the SK group had significantly lower results in theory of mind. Regarding the hostile attribution bias, no significant difference was found. Finally, the results showed correlations between specific attentional disorders and facial emotional recognition.
  
  
  CONCLUSIONS
  Our study emphasizes social cognition disorders in SK. These disorders could be considered as a phenotypic trait in the syndrome. The interest of better characterizing the cognitive phenotype of genetic disorders that can affect the neurodevelopment is to offer specific cognitive remediation strategies.
authors.affiliation: GénoPsy, centre de dépistage et de prises en charge des troubles psychiatriques d'origine génétique, centre hospitalier Le-Vinatier, 95, boulevard Pinel, 69500 Bron, France; EDR-Psy, UMR 5929, CNRS, université Lyon 1, 67, boulevard Pinel, 69500 Bron, France.
authors.forename: M-N
authors.initials: MN
authors.lastname: Babinet
authors.affiliation: GénoPsy, centre de dépistage et de prises en charge des troubles psychiatriques d'origine génétique, centre hospitalier Le-Vinatier, 95, boulevard Pinel, 69500 Bron, France; EDR-Psy, UMR 5929, CNRS, université Lyon 1, 67, boulevard Pinel, 69500 Bron, France.
authors.forename: C
authors.initials: C
authors.lastname: Rigard
authors.affiliation: GénoPsy, centre de dépistage et de prises en charge des troubles psychiatriques d'origine génétique, centre hospitalier Le-Vinatier, 95, boulevard Pinel, 69500 Bron, France; EDR-Psy, UMR 5929, CNRS, université Lyon 1, 67, boulevard Pinel, 69500 Bron, France.
authors.forename: É
authors.initials: É
authors.lastname: Peyroux
authors.affiliation: GénoPsy, centre de dépistage et de prises en charge des troubles psychiatriques d'origine génétique, centre hospitalier Le-Vinatier, 95, boulevard Pinel, 69500 Bron, France; EDR-Psy, UMR 5929, CNRS, université Lyon 1, 67, boulevard Pinel, 69500 Bron, France; Service de médecine de la reproduction, laboratoire d'hormonologie-endocrinologie moléculaire et maladies rares, hôpital Femme-Mère-Enfant, hospices civils de Lyon, université Lyon 1, 3, quai des Célestins, 69002 Lyon, France.
authors.forename: A-R
authors.initials: AR
authors.lastname: Dragomir
authors.affiliation: Service de médecine de la reproduction, laboratoire d'hormonologie-endocrinologie moléculaire et maladies rares, hôpital Femme-Mère-Enfant, hospices civils de Lyon, université Lyon 1, 3, quai des Célestins, 69002 Lyon, France.
authors.forename: I
authors.initials: I
authors.lastname: Plotton
authors.affiliation: Service de médecine de la reproduction, laboratoire d'hormonologie-endocrinologie moléculaire et maladies rares, hôpital Femme-Mère-Enfant, hospices civils de Lyon, université Lyon 1, 3, quai des Célestins, 69002 Lyon, France.
authors.forename: H
authors.initials: H
authors.lastname: Lejeune
authors.affiliation: GénoPsy, centre de dépistage et de prises en charge des troubles psychiatriques d'origine génétique, centre hospitalier Le-Vinatier, 95, boulevard Pinel, 69500 Bron, France; EDR-Psy, UMR 5929, CNRS, université Lyon 1, 67, boulevard Pinel, 69500 Bron, France. Electronic address: caroline.demily@ch-levinatier.fr.
authors.forename: C
authors.initials: C
authors.lastname: Demily
mesh_terms: D000293:Adolescent; D000328:Adult; D003071:Cognition / Q000502:physiology*; D006801:Humans; D007713:Klinefelter Syndrome / Q000503:physiopathology / Q000523:psychology*; D008297:Male; D009483:Neuropsychological Tests; D010641:Phenotype; D012919:Social Behavior; D012938:Social Perception; D011795:Surveys and Questionnaires; D055815:Young Adult
keywords: Attributional style; Cognition sociale; Facial emotional recognition; Klinefelter syndrome; Neurocognition; Psychiatrie; Psychiatry; Reconnaissance des émotions faciales; Social cognition; Style attributionnel; Syndrome de Klinefelter; Theory of mind; Théorie de l’esprit; XYY