Effectiveness of social management training on executive functions in males with Klinefelter syndrome (47, XXY).

Men with an extra X chromosome are at risk for social difficulties in which executive functions are known to play an important role. The aim of this study was to examine the potential efficacy of a novel neurocognitive-behavioral treatment program tailored to the specific vulnerabilities of Klinefelter syndrome (47, XXY). Social Management Training (SMT) aimed to increase the ability of individuals to regulate their thoughts, emotions and behaviors in ways that are socially adaptive. 16 Adolescents and men with Klinefelter Syndrome participated in SMT. This novel group treatment program consists of 10 sessions and includes psychoeducation, cognitive-behavioral skills training, home-assignments, and relaxation exercises. There were pre- and posttest cognitive assessments (five months apart) of executive functioning including sustained attention, inhibition, cognitive flexibility and working memory, as well as self-evaluation of executive functioning in daily life. Significant pre- to posttest improvements in inhibitory control (performance test) and metacognition skills (self-report) were found, with effects sizes of 1.3 and 0.5, respectively. No effects of intervention were found on sustained attention, cognitive flexibility and working memory. These findings suggest that SMT, with a key focus on executive dysfunction and tailored to the behavioral and cognitive profile of males with Klinefelter syndrome, may be a promising and potentially efficacious treatment approach for improving self-control and social adaptation, although larger and randomized controlled studies are warranted.

Social Management Training in Males With 47,XXY (Klinefelter Syndrome): A Pilot Study of a Neurocognitive-Behavioral Treatment Targeting Social, Emotional, and Behavioral Problems.

Klinefelter syndrome (47,XXY) is associated with problems in social interaction and behavioral adaptation. Sixteen adolescents and adult men with 47,XXY enrolled in a pilot-study evaluating the effectiveness of Social Management Training (SMT), a novel neurocognitive-behavioral treatment program targeted at improving social, emotional, and behavioral functioning. Participants reported improved emotional stability from pre- to post-test (5 months). Informants reported reductions in internalizing and externalizing symptoms, including improvement in self-regulation. Although informants did not report changes in autism-like symptoms, increased awareness of social challenges was found. SMT may improve emotional stability, self-regulation, and self-reflection in people males with Klinefelter syndrome. This potentially efficacious treatment approach may prove to be a promising psychosocial therapeutic intervention for this population.

Vulnerability for autism traits in boys and men with an extra X chromosome (47,XXY): the mediating role of cognitive flexibility.

The XXY chromosomal pattern (Klinefelter syndrome, KS) has been associated with specific effects on physical, neurobiological, endocrinological and psychological development. This study was focused on the described risk for autism in KS, and the cognitive mechanisms that mediate this risk. Our aim was to assess whether autistic features in KS result from impairments in executive functioning, more specifically difficulties in cognitive flexibility. In total, 71 boys and men with KS and 61 non-clinical controls participated in the study. Autistic features were assessed using the Autism-spectrum Quotient (AQ). Mental flexibility was measured using the Wisconsin Card Sorting Test (WCST). The level of autism traits was significantly increased in the KS group, the effect size for total AQ score was 1.6. The KS group also showed significantly more difficulties in cognitive flexibility, as indicated by and increased number of perseverative (but not non-perseverative) errors in the WCST. This effect was independent of intellectual functioning, age or testosterone supplements. Within the KS group, the number of perseverative errors was significantly (positively) correlated with total AQ score. Our findings suggest that KS can be associated with dysfunctions in mental flexibility, and that individuals with more mental flexibility problems also have more autism traits. This insight is relevant for diagnosis, prevention and treatment of severe problems in individuals with KS. Implications also extend beyond this specific syndrome. As executive dysfunctions in KS have also been linked to ADHD symptoms and thought disorder, this could be a shared mechanism contributing to overlap in symptoms and comorbidity between different psychiatric conditions.