title: Impact of cognitive profile on social functioning in prepubescent females with Turner syndrome.
abstract: Social deficits are prevalent in Turner syndrome (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 participants 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 dysfunctions 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.
authors.affiliation: Center for Interdisciplinary Brain Sciences Research, Stanford University School of Medicine, Stanford, CA 94305, USA.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Lepage
authors.forename: Bria
authors.initials: B
authors.lastname: Dunkin
authors.forename: David S
authors.initials: DS
authors.lastname: Hong
authors.forename: Allan L
authors.initials: AL
authors.lastname: Reiss
mesh_terms: D002648:Child; D003072:Cognition Disorders / Q000209:etiology / Q000503:physiopathology*; D056344:Executive Function / Q000502:physiology; D005260:Female; D006801:Humans; D007360:Intelligence / Q000502:physiology; D009483:Neuropsychological Tests; D011569:Psychiatric Status Rating Scales; D011597:Psychomotor Performance / Q000502:physiology; D012919:Social Behavior; D014424:Turner Syndrome / Q000150:complications / Q000503:physiopathology*