Toward a deeper characterization of the social phenotype of Williams syndrome: The association between personality and social drive

Abstract

Previous research has robustly established a Williams syndrome (WS) specific personality profile, predominantly characterized a gregarious, people-oriented, and tense predisposition. Extending this work, the aims of the current, cross-sectional study were two-fold: (1) to elucidate the stability of personality characteristics in individuals with WS and typically developing (TD) comparisons across development, and (2) to explore the personality attributes that may be related to the respective profiles of social functioning characterizing the two groups, which is currently poorly understood. The sample comprised of participants with WS and TD matched on chronological age. The test battery included the Multidimensional Personality Questionnaire (MPQ) and the Salk Institute Sociability Questionnaire (SISQ), an index of real-life social behavior. The main results showed that compared to the TD individuals, the WS group were consistently rated higher in Social Closeness, and this trait remained stable across development. Interpersonal behaviors were best predicted by Social Closeness in WS and by Social Potency in TD. Regression analysis highlighted that while a central motive underlying the increased drive toward social interaction in individuals with WS pertains to a desire to form affectionate relationships, TD individuals by contrast are motivated by a desire to exert social influence over others (leadership, social-dominance) and Well-Being (positive emotional disposition). In conclusion, these findings provide novel insight into social motivational factors underpinning the WS social behavior in real life, and contribute toward a deeper characterization of the WS affiliative drive. We suggest potential areas for behavioral intervention targeting improved social adjustment in individuals with WS.

Relations between social-perceptual ability in multi- and unisensory contexts, autonomic reactivity, and social functioning in individuals with Williams syndrome

“…This profile suggesting more pronounced impairments in social-cognitive over pro-social/motivational functions appears stable across development in WS (cf. Klein-Tasman et al, 2011; Riby et al, 2014; Ng, Järvinen, & Bellugi, 2014). …”

Structural integrity of the limbic–prefrontal connection: Neuropathological correlates of anxiety in Williams syndrome

“…Finally, measures of sociability (i.e., approach strangers/familiars, social emotionality) were unrelated to either the white matter structural characteristics of the UF or anxiety ratings. Altogether, the unique association between microstructural features of the UF and anxiety in the WS group underscores their anxiety profile (Ng et al, 2014a, 2014b). …”

“…The social phenotype associated with WS is characterized by a gregarious personality, exaggerated empathetic gestures, and robust drive to interact with others (Järvinen et al, 2013). Despite their motivation to develop close relationships with others (Ng et al, 2014b), individuals with WS tend to demonstrate greater anxiety relative to peers with mental retardation, mixed etiology, as well as typical development (TD)(Dykens, 2003; Ng et al, 2014a). Importantly, behavioral and neuroimaging research with individuals with WS seemingly indicate that their anxiety is largely nonsocial in nature (Meyer-Lindenberg et al, 2005; Ng et al, 2014a; Rodgers et al, 2012).…”

A Diagnosis to Consider in an Adult Patient with Facial Features and Intellectual Disability: Williams Syndrome

“…The symptoms in adulthood are not as suggestive as they are during childhood. In adulthood, patients usually present with mild intellectual disability and psychiatric disorders, including depression and anxiety disorder 7). The patients usually have subtle findings, but their unique behavioral patterns may be suggestive of Williams syndrome.…”

“…Moderate cognitive impairment and unique hypersocial behavioral features, such as diminished fear to approach strangers, elevated emphatic concern, reduced social fear, an increased attraction towards faces and eyes in social interactions, and a greater interest in contact with adults rather than younger peers are characteristic neurobehavioral features of the syndrome 6). Generalized anxiety disorder or specific phobias may also coexist with the above behavioral characteristics 7). Williams syndrome is usually diagnosed during infancy or childhood.…”