II. Hypersociability in Williams Syndrome

Abstract

Studies of abnormal populations provide a rare opportunity for examining relationships between cognition, genotype and brain neurobiology, permitting comparisons across these different levels of analysis. In our studies, we investigate individuals with a rare, genetically based disorder called Williams syndrome (WMS) to draw links among these levels. A critical component of such a cross-domain undertaking is the clear delineation of the phenotype of the disorder in question. Of special interest in this paper is a relatively unexplored unusual social phenotype in WMS that includes an overfriendly and engaging personality. Four studies measuring distinct aspects of hypersocial behavior in WMS are presented, each probing specific aspects in WMS infants, toddlers, school age children, and adults. The abnormal profile of excessively social behavior represents an important component of the phenotype that may distinguish WMS from other developmental disorders. Furthermore, the studies show that the profile is observed across a wide range of ages, and emerges consistently across multiple experimental paradigms. These studies of hypersocial behavior in WMS promise to provide the groundwork for crossdisciplinary analyses of gene-brain-behavior relationships.

Heterogeneity of social approach behaviour in Williams syndrome: The role of response inhibition

“…In the current study, participants rated the positive stimuli similarly to participants with WS in the studies by Jones et al (2000) and Martens et al (2009). However the ratings for the negative stimuli were rated more favourably which indicates that participants in this study were more willing to approach the negative faces than the participants in studies by Jones et al (2000) and Martens et al (2009).…”

“…Hypersociability is frequently cited to be a defining feature of the social phenotype associated with WS (Järvinen-Pasley, Adolphs, Yam, Hill, Grichanik, Reilly & Bellugi, 2010) and has been described as a 'general presentation of extreme happiness' (Levine & Wharton, 2000; p.364); being 'unusually sociable, friendly and empathic' (Jones, Bellugi, Lai, Chiles, Reilly, Lincoln & Adolphs, 2000 p. 30), an excessive interest in others and a distinct lack of inhibition with regard to approaching others in social contexts (Bellugi, Järvinen-Pasley, Doyle Reilly, Reiss & Korenberg, 2007;Jones et al, 2000). Individuals with WS appear RUNNING HEAD: Social Approach in Williams syndrome 4 hypersociable from an early age (Doyle, Bellugi, Korenberg & Graham, 2004), demonstrating an eagerness to make eye contact with and to approach strangers (Mervis & Klein-Tasman, 2000).…”

Using developmental trajectories to examine verbal and visuospatial short-term memory development in children and adolescents with Williams and Down syndromes

“…Psychological and behavioural markers include mild-moderate intellectual disability (ID; Udwin, Yule, & Martin, 1987), a sociable disposition (e.g. Jones et al, 2000), heightened anxiety (Dykens, 2003) and hyper-sensitivity to sound (e.g. Gallo, Klein-Tasman, Gaffrey, & Curran, 2008).…”

Attachment disorders in family and social context

“…It is clear that RAD has been reported only in children with histories of either maltreatment or institutional rearing, but this may be because the pathogenic care criterion is required. Furthermore, the pathogenic care criterion is particularly important in distinguishing indiscriminate/disinhibited RAD from conditions such as Williams syndrome and fetal alcohol syndrome, both of which have been reported to be associated with indiscriminate social behavior (Jacobson & Jacobson, 2003;Jones et al, 2000).…”