title: The role of genes, intelligence, personality, and social engagement in cognitive performance in Klinefelter syndrome.
abstract: The determinants of cognitive deficits among individuals with Klinefelter syndrome (KS) are not well understood. This study was conducted to assess the impact of general intelligence, personality, and social engagement on cognitive performance among patients with KS and a group of controls matched for age and years of education.
  
  
  
  Sixty-nine patients with KS and 69 controls were assessed in terms of IQ, NEO personality inventory, the Autism Spectrum Quotient (AQ) scale, and measures of cognitive performance reflecting working memory and executive function.
  
  
  
  Patients with KS performed more poorly on memory and executive-function tasks. Patients with KS also exhibited greater neuroticism and less extraversion, openness, and conscientiousness than controls. Memory deficits among patients with KS were associated with lower intelligence, while diminished executive functioning was mediated by both lower intelligence and less social engagement.
  
  
  
  Our results suggest that among patients with KS, memory deficits are principally a function of lower general intelligence, while executive-function deficits are associated with both lower intelligence and poorer social skills. This suggests a potential influence of social engagement on executive cognitive functioning (and/or vice-versa) among individuals with KS, and perhaps those with other genetic disorders. Future longitudinal research would be important to further clarify this and other issues discussed in this research.
authors.affiliation: Department of Endocrinology and Internal Medicine (MEA) Aarhus University Hospital Aarhus Denmark; Department of Clinical Genetics Aarhus University Hospital Aarhus Denmark.
authors.forename: Anne
authors.identifier: 0000-0001-9178-4901
authors.initials: A
authors.lastname: Skakkebæk
authors.affiliation: Department of Psychology The George Washington University Washington DC USA.
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Moore
authors.affiliation: Department of Psychology and Behavioral Sciences Aarhus University Aarhus Denmark.
authors.forename: Anders Degn
authors.initials: AD
authors.lastname: Pedersen
authors.affiliation: Department of Clinical Genetics Aarhus University Hospita Aarhus Denmark; Department of Clinical Genetics Sygehus Lillebaelt Vejle Denmark.
authors.forename: Anders
authors.initials: A
authors.lastname: Bojesen
authors.affiliation: Department of Mental Health Odense University Clinic Odense Denmark.
authors.forename: Maria Krarup
authors.initials: MK
authors.lastname: Kristensen
authors.affiliation: Fertility Clinic Department of Gynecology and Obstetrics Odense University Hospital Odense Denmark.
authors.forename: Jens
authors.initials: J
authors.lastname: Fedder
authors.affiliation: Department of Endocrinology Aalborg University Hospital Aalborg Denmark.
authors.forename: Peter
authors.initials: P
authors.lastname: Laurberg
authors.affiliation: Department of Clinical Genetics Odense University Hospital Odense Denmark.
authors.forename: Jens Michael
authors.initials: JM
authors.lastname: Hertz
authors.affiliation: Department of Pediatrics Centre for Rare Diseases Aarhus University Hospital Aarhus Denmark.
authors.forename: John Rosendahl
authors.initials: JR
authors.lastname: Østergaard
authors.affiliation: Center for Semiotics Aarhus University Aarhus Denmark; Center of Functionally Integrative Neuroscience Aarhus University Hospital Aarhus Denmark.
authors.forename: Mikkel
authors.initials: M
authors.lastname: Wallentin
authors.affiliation: Department of Endocrinology and Internal Medicine (MEA) Aarhus University Hospital Aarhus Denmark; Department of Molecular Medicine Aarhus University Hospital Aarhus Denmark.
authors.forename: Claus Højbjerg
authors.initials: CH
authors.lastname: Gravholt
mesh_terms: D000293:Adolescent; D000328:Adult; D060825:Cognitive Dysfunction / Q000209:etiology / Q000235:genetics / Q000503:physiopathology*; D056344:Executive Function / Q000502:physiology*; D006801:Humans; D007360:Intelligence / Q000235:genetics / Q000502:physiology*; D007713:Klinefelter Syndrome / Q000150:complications / Q000235:genetics / Q000503:physiopathology*; D008297:Male; D008570:Memory, Short-Term / Q000502:physiology*; D008875:Middle Aged; D010551:Personality / Q000235:genetics / Q000502:physiology*; D066107:Social Skills; D055815:Young Adult
keywords: Klinefelter syndrome; cognitive performance; genetics; personality; social engagement