I. The Neurocognitive Profile of Williams Syndrome: A Complex Pattern of Strengths and Weaknesses

Abstract

The rare, genetically based disorder, Williams syndrome (WMS), produces a constellation of distinctive cognitive, neuroanatomical, and electrophysiological features which we explore through the series of studies reported here. In this paper, we focus primarily on the cognitive characteristics of WMS and begin to forge links among these characteristics, the brain, and the genetic basis of the disorder. The distinctive cognitive profile of individuals with WMS includes relative strengths in language and facial processing and profound impairment in spatial cognition. The cognitive profile of abilities, including what is 'typical' for individuals with WMS is discussed, but we also highlight areas of variability across the group of individuals with WMS that we have studied. Although the overall cognitive abilities (IQs) of individuals with WMS are typically in the mild-to-moderate range of mental retardation, the peaks and valleys within different cognitive domains make this syndrome especially intriguing to study across levels. Understanding the brain basis (and ultimately the genetic basis) for higher cognitive functioning is the goal we have begun to undertake with this line of interdisciplinary research.

Profiles and Development of Adaptive Behavior in Adolescents and Adults with Williams Syndrome

“…In addition, individuals with WS have an unusual personality profile associated with high sociability, overfriendliness, empathy and excessive anxiety [7]- [10]. The syndrome results in mild to moderate intellectual or learning disability [11].…”

Do Faces Capture the Attention of Individuals with Williams Syndrome or Autism? Evidence from Tracking Eye Movements

“…Bellugi et al, 2000). Whilst individuals with autism are typically characterised by social withdrawal and a lack of desire to engage in social interactions (Frith, 1989;DSM IV, APA 1994), individuals with WS are associated with a drive towards social engagement, often labelled as 'hyper-sociability' or a 'pro-social compulsion' (Frigerio et al, 2006).…”

Cognitive architecture and descent with modification☆

“…Finally, although there appear to be rare cases of individuals with impaired language but intact cognition (van der Lely, Rosen, & McClelland, 1998) and cases of individuals with (nearly) normal language but significantly impaired cognition (Bellugi, Bihrle, Jernigan, Trauner, & Doherty, 1990;Bellugi, Lichtenberger, Jones, Lai, & St George, 2000;Johnson & Carey, 1998), across the population at large, language and cognition are correlated. Bright people tend to be articulate, less bright people less so (Colledge et al, 2002) and people with impairments in one typically have impairments in the other (Hill, 2001), empirical facts that are not directly anticipated by the standard take on modularity.…”