Williams syndrome: a genetic deletion disorder presenting clues to the biology of sociability and clinical challenges of hypersociability

Williams syndrome is a neurodevelopmental disorder that results from the deletion of approximately 25-30 genes spanning about 1.5 megabases in the q11.23 region of chromosome 7. Patients with this syndrome present with a combination of a distinctive elfin-like facial appearance; growth retardation; mild mental retardation; an inconsistent cognitive profile that includes visuospatial impairments with good facial discrimination and relatively preserved expressive language skills; and cardiovascular abnormalities. In addition, a striking behavioral feature of the syndrome is the high sociability and empathy that these patients show for others. The study of patients with "partial" deletions of the chromosome band 7q11.23, mutated genes in this region and knockout mice with deletions of specific genes in the homologous G1-G2 region of mouse chromosome 5 are clarifying some genotype/phenotype relationships. Furthermore, genes located in this region that are prominently expressed have been implicated in brain development and function. The neuropsychological profile of patients with Williams syndrome is heterogeneous, highlights important dissociations between cognitive functions and suggests that the behavioral dimensions of sociability, empathy, engageability, and talkativeness may be independent of, or not easily explained by, the cognitive deficits. Williams syndrome has enormous heuristic value because its pathological feature of heightened "sociability" can be a "deficit" symptom of major complex neuropsychiatric disorders, such as schizophrenia and autism. Data consistent with a core inability of patients with Williams syndrome to inhibit social approach suggest that this disorder may afford an opportunity to study the biological basis of the "drive" toward socialization. From a research perspective, the syndrome lends itself to neurobiological studies of sociability as a dimension that varies independently of cognition (or at least many separable cognitive processes). Importantly, from a clinical perspective, the syndrome challenges us to administer strategic psychosocial interventions that take advantage of the opportunities that "pathological" sociability provide, while avoiding its threats. An illustrative example of an effective strategically planned psychosocial intervention for a patient with Williams syndrome is briefly presented.     

Agent-based modeling: a new approach for theory building in social psychology.

Most social and psychological phenomena occur not as the result of isolated decisions by individuals but rather as the result of repeated interactions between multiple individuals over time. Yet the theory-building and modeling techniques most commonly used in social psychology are less than ideal for understanding such dynamic and interactive processes. This article describes an alternative approach to theory building, agent-based modeling (ABM), which involves simulation of large numbers of autonomous agents that interact with each other and with a simulated environment and the observation of emergent patterns from their interactions. The authors believe that the ABM approach is better able than prevailing approaches in the field, variable-based modeling (VBM) techniques such as causal modeling, to capture types of complex, dynamic, interactive processes so important in the social world. The article elaborates several important contrasts between ABM and VBM and offers specific recommendations for learning more and applying the ABM approach.     

I feel our pain: Antecedents and consequences of emotional self-stereotyping

According to Intergroup Emotions Theory people categorized as group members experience the emotions of their ingroup as a consequence of that membership. Four experiments showed that participants converged toward what they believed to be their specific ingroup’s distinct emotional experience when reporting emotions as group members, but not when reporting emotions as individuals. Such self-stereotyping of ingroup emotions occurred for an experimentally fabricated ingroup as well as a range of naturally occurring groups. Demonstrating the roots of this process in categorization, self-stereotyping was increased when motivations to affiliate were amplified and was moderated by ingroup identification. The adoption of ingroup emotions changed participants’ cognitive processing in a predictable way, demonstrating that emotional self-stereotyping involved the experience rather than merely the expression of group-based emotions. Self-stereotyping of ingroup emotions is thus one mechanism by which group-based emotions are shared and can be changed.     

Subtle activation of a social categorization triggers group-level emotions

Previous research [Smith, E. R., Seger, C. R., & Mackie, D. M. (2007). Can emotions be truly group-level? Evidence regarding four conceptual criteria. Journal of Personality and Social Psychology, 93, 431-446] has demonstrated that when people are explicitly asked about the emotions they experience as members of a particular group, their reported emotions converge toward a profile typical for that group. Two studies demonstrate that the same type of convergence occurs when a group identity is made situationally salient through priming, without an explicit request to report group-level emotions. People who identify more strongly with the group converge more, and show more similarity between their group-primed emotions and explicitly reported group-level emotions. This research confirms that activating a social identity produces convergence for emotions as well as for attitudes and behaviors. It also suggests that some previous emotion research may have tapped group rather than individual-level emotions, potentially requiring some reconceptualization.     

How do we locate ourselves on a map: a method for analyzing self-location processes

The activity of self-location can be described as the making of a connection between the spatial relationships perceived from the subject's position (egocentric frame of reference) and all the external spatial relationships which contain this position (geocentric frame of reference: a map for example). We propose a method for analyzing the cognitive processes involved in a self-location task where the subjects had to match 2 spatial frames of reference. The processing methods observed by measuring various parameters used to evaluate the spatial relationships showed that the subjects (adults) could use a wide range of spatial tools - which were often equivalent and thus redundant - to locate themselves on a map, and that they drew upon this spatial repertory according to their requirements.     

Learning of gastrointestinal satiety signals

Rats were trained to ingest corn oil emulsion. Such ingestion was accompanied by intragastric injection either of dilute amino acid (e.g., 4.3 mM L-tryptophan) in one group or saline in a second. No significant difference between the two groups was observed at the end of training. However sudden omission of the amino acid led to a large increase in intake, whereas sudden introduction of the amino acid produced no significant change. It seems that signals for the amount of nutrient ingested can be arbitrarily associated with different nutrients and that the value of such satiety signals is not innately fixed in the amount of feeding inhibition exerted.