Experimental functional analysis of aggression in children with Angelman syndrome

The behavioral phenotype of the Angelman syndrome

“…However, by age 3-5 years they are The study of the behavioral phenotype in Angelman syndrome has been helpful in understanding and treating problematic behaviors. For example, the strong drive for pro-social activity means that educational strategies and behavioral modification therapies should incorporate the effects of social reinforcers or extinguishers [Strachan et al, 2009]. Also, knowledge that attraction to/fascination with water is a strong behavioral interest can be used to fashion both recreational and behavioral modification activities [Didden et al, 2008].…”

“…Even without the ability for verbal communication, individuals with Angelman syndrome interact with children and adults by pointing out interesting events or taking (sometimes grabbing) other's arms and leading them toward their favorite interests and activities. At times, pinching, grabbing, biting, slapping, and hitting can represent behaviors aimed at maintaining or reestablishing social interaction [Strachan et al, 2009]. The need for social interaction is thus an important behavioral reinforcer and modifier for children and adults with the syndrome.…”

From Causes of Aggression to Interventions

“…A number of genetic syndromes causing intellectual disabilities are associated with high levels of aggression, involving different psychological mechanisms: for example, aggression in Smith-Magenis syndrome is likely to be maintained by attention from carers (Taylor & Oliver, 2008), but aggression in Angelman syndrome may not be (Strachan et al, 2009). In the Fragile X syndrome, one of the most common forms of inherited cognitive impairment, abnormalities of face processing have been reported, associated with abnormal patterns of activation in the amygdala and anterior cingulate cortex (Schneider, Hagerman, & Hessl, 2009).…”

Functional analysis and treatment of problem behavior exhibited by children with fragile X syndrome

“…Commonly identified consequences that reinforce and maintain problem behavior in persons with IDD include provision of adult attention, access to tangible items, and escape from non-preferred tasks (Iwata et al, 1994a,b); however, a number of other functions for problem behavior have been identified (Lancioni, Singh, O'Reilly, Sigafoos, & Didden, 2012;Schlichenmeyer, Roscoe, Rooker, Wheeler, & Dube, 2013). Studies reporting on the functional analysis of problem behavior have included subpopulations of IDD such as individuals with ASD (Love, Carr, & LeBlanc, 2009;O'Reilly et al, 2010); prenatal drug exposure (Kurtz, Chin, Rush, & Dixon, 2008); and genetic disorders such as Angelman (Strachan et al, 2009), Cornelia de Lange (Bay, Mauk, Radcliffe, & Kaplan, 1993), Prader-Willi (Hall, Hustyi, Chui, & Hammond, 2014), Rett (Roane, Piazza, Sgro, Volkert, & Anderson, 2001), Soto (Harding et al, 2001), and Williams syndromes (O'Reilly, Lacey, & Lancioni, 2000).…”