Neurocognitive underpinnings among 47 XXY’s and the association with mental health problems

This study identified executive functions as predictors of emotion regulation abilities, specifically in relation to increased emotion expression. More difficulties in mental flexibility and attention regulation, as well as speeding responding were associated with more pronounced emotion expression, that is, emotional outbursts. Executive functions are essential in regulating and managing emotions, by allowing individuals to delay and reorient their behaviour under circumstances that trigger automatic emotional responses, and to override the action tendencies associated with the emotion, resulting in behaviours that are adaptive and socially acceptable. The present study suggests that impairments in executive functions that have been reported in earlier studies may limit the ability of 47,XXY’s to regulate their emotions and generate adaptive socioemotional behaviour. Although speculatively, based on the wide literature on the importance of the frontal lobes for self‐regulation, emotional reactivity, and executive control, one hypothesis may be that executive dysfunctions and related emotion regulation problems may be driven by abnormal structure and function of the frontal lobes in XXY, which is also supported by neuroimaging findings in XXY

This study suggests that emotion regulations problems in XXY may be associated with various mental health problems. An increased tendency for passive coping (e.g., isolating oneself from others, worrying about the past, taking refuge in fantasies) was associated with a wide range of symptoms of psychopathology, including symptoms of anxiety, depression, thought problems, and hostility. In addition, the strategy “avoiding” was specifically linked to depressive symptoms. These findings illustrate the importance of focusing on emotion regulation in improving daily life functioning and quality of life of adults with 47,XXY. The study has identified emotion regulation abilities as possibly relevant targets in treatment and intervention, with the potential to reduce the risk for more serious psychopathology and increase adaptive functioning within society, including functioning in academic and professional settings. Insight in EF as an underlying neurocognitive mechanism of emotion regulation may help us in providing more targeted treatment, focused on the mechanisms driving emotion regulation difficulties. Identifying the underlying mechanisms of mental health problems, and the interplay between such determinants may be a useful approach, as shown in other studies on XXY as well.

For clinicians, both emotion regulation and EF should be the focus of clinical assessment and treatment. Support should not be limited to training or intervention programs, but also include psychoeducation: Insight in own strengths and weaknesses may help to better understand, accept, and cope with challenges, and may contribute to making choices in daily life that better match the needs and limitations of the individual. To meet these aims, it is important to screen for executive dysfunction when 47,XXY’s present with emotional outbursts and other manifestations of loss of control over emotions. Our recommendations for clinical screening of problems in EF include: (a) The use of computerized neuropsychological testing rather than solely relying on questionnaires for EF,(b) assessment of specific/isolated executive functions to identify the type of EF deficit (if present), (c) relying on sensitive measures of EF to be able to also pick up on more subtle deficits, and not only the more severe cases, and (d) screening for EF as a part of a more comprehensive neuropsychological assessment.

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