Abstract
IntroductionThe 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.MethodsSixty‐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.ResultsPatients 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.ConclusionOur 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.
Highlights
The determinants of cognitive deficits among individuals with Klinefelter syndrome (KS) are not well understood
In addition to providing valuable information about the interaction between KS and social experience, KS-related deficits can inform a number of fundamental questions about cognitive performance: First, is cognitive performance a reflection of intelligence? Mean scores for groups of research patients with KS lie below normative averages on tests of general intelligence (Fales et al, 2003; van Rijn, Swaab, Aleman, & Kahn, 2008), and lower intelligence has been associated with poorer working memory and executive function in many studies in the general population (Ackerman, Beier, & Boyle, 2005; Ardila, Pineda, & Rosselli, 2000; Fales et al, 2003; Polderman et al, 2009; Salthouse & Pink, 2008), these findings are not universal (Friedman et al, 2006)
What role does personality play in cognitive performance? KS is associated with higher levels of neuroticism and lower levels of extraversion, conscientiousness, and openness to experience (Skakkebaek et al, 2013), and these personality characteristics are often correlated with measures of cognitive performance
Summary
Klinefelter syndrome (KS) is the most common sex chromosome disorder, present in 150 of every 100,000 men (Bojesen, Juul, & Gravholt, 2003; Nielsen & Wohlert, 1990). While many studies have examined important bivariate relationships between these factors and KS status, research has yet to examine the simultaneous effects of genetic, personality, and social variables among individuals with and without KS, or how these factors combine to determine differences in cognitive functioning. To address these issues, the current research investigated the impact of intelligence, personality traits, and social engagement on cognitive performance in a sample of patients with KS and in a group of controls matched for age and years of education
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