Psychosocial Functioning and Social Cognitive Processing in Girls with Turner Syndrome

Hong, David S.; Dunkin, Bria; Reiss, Allan L.

doi:10.1097/dbp.0b013e3182255301

Cited by 63 publications

(62 citation statements)

References 45 publications

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“…This comprises a significant discrepancy between verbal and non-verbal IQ (t-test; p<.001) (Shaffer, 1962), as well as significant impairments on visuospatial tasks (Ross et al, 1997) and executive functions (Temple, Carney, & Mullarkey, 1996). Albeit not as often reported as PIQ findings, the presence of discrepancy on the VIQ in comparison to controls has also been previously reported (Hong et al, 2011; Loesch et al, 2005; Kirk et al, 2005). …”

Section: Discussionmentioning

confidence: 60%

“…This observation is particularly interesting when considering the view of TS having overlapping symptomatology with autism spectrum disorders (ASD) (Mazzola et al, 2006; Skuse, 2005). Indeed, while it is true some characteristic of social behavior seen here correspond to what is observed in ASD, namely autistic mannerisms as measured by the SRS, TS children differ on at least one important point from individuals with ASD in the sense that they are motivated to engage in social interactions, where as children diagnosed with ASD are not (Hong et al 2011). Plausibly, this drive to engage in social relations, coupled with their decreased ability to perceive, analyze, and communicate social information, contribute negatively to self-esteem of girls with TS (McCauley, Ross, Kushner, & Cutler, 1995), and to their perception of isolation (Amundson, Boman, Barrenäs, Bryman, & Landin-Wilhelmsen, 2010) and diminished social competency (McCauley et al, 2001) in adolescence and adulthood.…”

Section: Discussionmentioning

confidence: 69%

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Impact of cognitive profile on social functioning in prepubescent females with Turner syndrome

Lepage

Dunkin

Hong

et al. 2013

Child Neuropsychology

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Social deficits are prevalent in TS, however, the extent to which these difficulties are secondary to characteristic cognitive impairments is not well known. Here, we sought to establish the relative contribution of executive functions, visuospatial abilities and IQ to social difficulties in young girls with TS. Forty TS girls and 19 typically developing (TD) children were assessed with the Social Responsiveness Scale (SRS), the Motor-Free Visual Spatial test (MVPT-3), the Behavior Rating Inventory of Executive Function (BRIEF) and an IQ test. Hierarchical multiple regression analyses were conducted with the SRS subscales as outcome variables. In a first step, the cognitive factors were entered (verbal IQ, BRIEF global score, MVPT-3 and age), followed by the group variable in a second step. In comparison to TD, TS were significantly impaired on all main measures. All six regression models with the SRS subscales were significant and revealed that global executive functions explained the largest portion of the variance on all subscales and the total score. Even after controlling for cognitive elements, the group factor still explained a significant portion of the variance of the Social Cognition, Social Awareness, and Autistic Mannerisms subscales. In contrast, the group factor was not a significant predictor of Social Motivation and Social Communication scores. These results suggest that executive dysfunction play a role in social impairments encountered in TS, but also that some specific aspects of social behavior are altered beyond what can be attributed to cognitive difficulties in this population.

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Section: Discussionmentioning

confidence: 60%

Section: Discussionmentioning

confidence: 69%

Impact of cognitive profile on social functioning in prepubescent females with Turner syndrome

Lepage

Dunkin

Hong

et al. 2013

Child Neuropsychology

Self Cite

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“…Turner syndrome is associated with cognitive deficits, including problems with visuospatial perception and memory, emotionality, and attention (Skuse, 2005; Skuse et al , 2005). Girls with Turner syndrome also have reduced social competence and facial recognition (Hong et al , 2011; Lawrence et al , 2003), and may be at higher risk for autism (Burnett et al , 2010; Marco and Skuse, 2006). Using a tracking task that required increasing amounts of attention, Beaton and colleagues (Beaton et al , 2010) examined BOLD patterns in normal and Turner syndrome girls.…”

Section: Discussionmentioning

confidence: 99%

Mouse model systems to study sex chromosome genes and behavior: Relevance to humans

Cox

Bonthuis

Rissman

2014

Frontiers in Neuroendocrinology

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Sex chromosome genes directly influence sex differences in behavior. The discovery of the Sry gene on the Y chromosome (Gubbay et al., 1990; Koopman et al., 1990) substantiated the sex chromosome mechanistic link to sex differences. Moreover, the pronounced connection between X chromosome gene mutations and mental illness produces a strong sex bias in these diseases. Yet, the dominant explanation for sex differences continues to be the gonadal hormones. Here we review progress made on behavioral differences in mouse models that uncouple sex chromosome complement from gonadal sex. We conclude that many social and cognitive behaviors are modified by sex chromosome complement, and discuss the implications for human research. Future directions need to include identification of the genes involved and interactions with these genes and gonadal hormones.

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“…However, girls with TS differ in an important respect from patients with autism: they do desire social interaction (60). …”

Section: Adolescence 13–18 Yearsmentioning

confidence: 99%

Care of girls and women with Turner syndrome: beyond growth and hormones

Culen

Ertl

Schubert

et al. 2017

Endocrine Connections

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Turner syndrome (TS), although considered a rare disease, is the most common sex chromosome abnormality in women, with an incident of 1 in 2500 female births. TS is characterized by distinctive physical features such as short stature, ovarian dysgenesis, an increased risk for heart and renal defects as well as a specific cognitive and psychosocial phenotype. Given the complexity of the condition, patients face manifold difficulties which increase over the lifespan. Furthermore, failures during the transitional phase to adult care result in moderate health outcomes and decreased quality of life. Guidelines on the optimal screening procedures and medical treatment are easy to find. However, recommendations for the treatment of the incriminating psychosocial aspects in TS are scarce. In this work, we first reviewed the literature on the cognitive and psychosocial development of girls with TS compared with normal development, from disclosure to young adulthood, and then introduce a psychosocial approach to counseling and treating patients with TS, including recommendations for age-appropriate psychological diagnostics. With this work, we aim to facilitate the integration of emphasized psychosocial care in state-of-the-art treatment for girls and women with TS.

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Psychosocial Functioning and Social Cognitive Processing in Girls with Turner Syndrome

Cited by 63 publications

References 45 publications

Impact of cognitive profile on social functioning in prepubescent females with Turner syndrome

Impact of cognitive profile on social functioning in prepubescent females with Turner syndrome

Mouse model systems to study sex chromosome genes and behavior: Relevance to humans

Care of girls and women with Turner syndrome: beyond growth and hormones

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