Brief Report: A Longitudinal Study of Excessive Smiling and Laughing in Children with Angelman Syndrome.

Elevated laughing and smiling is a key characteristic of the Angelman syndrome behavioral phenotype, with cross-sectional studies reporting changes with environment and age. This study compares levels of laughing and smiling in 12 participants across three experimental conditions [full social interaction (with eye contact), social interaction with no eye contact, proximity only] at two data points. No differences were noted in frequency of laughing and smiling over time in any condition.

Age related change in social behavior in children with Angelman syndrome.

We investigated the relationship between age and laughing and smiling in children with Angelman syndrome. Twenty-four children with Angelman syndrome were exposed to three experimentally manipulated conditions: proximity only, restricted social interaction, and social interaction. Children smiled the most in the social interaction condition and the least in the proximity only condition confirming the effect of social interaction on these behaviors.

Effects of adult familiarity on social behaviours in Angelman syndrome.

Background: Individuals with Angelman syndrome appear strongly motivated by social contact, but there have been few studies that have examined the relationship between sociability and familiarity. In this study we compared social behaviour in Angelman syndrome when in contact with mothers and strangers.

Methods: We systematically manipulated adult familiarity, eye contact and speech to examine the effect on social approach behaviours in children with Angelman syndrome.

Experimental functional analysis of aggression in children with Angelman syndrome.

Background: Kinship theory suggests that genomic imprinting could account for phenotypic behaviors that increase (in the case of Angelman syndrome) or decrease (for Prader-Willi syndrome) the drive to access social resources (adult contact) depending on the imprinting parent-of-origin. Difficult to manage behaviors, such as aggression that is common in Angelman syndrome, could serve the function of increasing social interaction. We hypothesise that the commonly reported aggressive behavior in children with Angelman syndrome will be attention maintained.

Genomic imprinting and the expression of affect in Angelman syndrome: what's in the smile?

Background: Kinship theory (or the genomic conflict hypothesis) proposes that the phenotypic effects of genomic imprinting arise from conflict between paternally and maternally inherited alleles. A prediction arising for social behaviour from this theory is that imbalance in this conflict resulting from a deletion of a maternally imprinted gene, as in Angelman syndrome (AS), will result in a behavioural phenotype that should evidence behaviours that increase access to maternally provided social resources (adult contact).

Method: Observation of the social behaviour of children with AS (n = 13), caused by a deletion at 15q11-q13, and a matched comparison group (n = 10) was undertaken for four hours in a socially competitive setting and the effect of adult attention on child behaviours and the effect of child smiling on adult behaviours evaluated using group comparisons and observational lag sequential analyses.