We show that despite putative processing similarities at the cognitive level, binding in Williams syndrome and autism can be dissociated at the neurophysiological level by different abnormalities in underlying brain oscillatory activity.
WS was first reported in 1961 by Williams, who described children with unusual facial features in addition to growth retardation, supravalvular aortic stenosis (SVAS), and mild mental retardation.
The neuropsychological profile in WS is remarkable because of the magnitude of the disparity between cognitive strengths and weaknesses, a disparity that is thought to be rooted in the functional integrity of different neural networks.
This set of guidelines is designed to assist the pediatrician to care for children with Williams syndrome diagnosed by clinical features and with regional chromosomal microdeletion confirmed by fluorescence in situ hybridization.
In face processing, although both individuals with WS or autism succeed on judging direction of eye gaze, only WS subjects perform successfully on tasks involving inferring intention from eye gaze.
Autism and Williams syndrome are genetically based neurodevelopmental disorders that present strikingly different social phenotypes. Autism involves fundamental impairments in social reciprocity and communication, whereas people with Williams syndrome are highly sociable and engaging. This article reviews the behavioral and neuroimaging literature that has explored the neurocognitive mechanisms that underlie these contrasting social phenotypes, focusing on studies of face processing. The article concludes with a discussion of how the social phenotypes of both syndromes may be characterized by impaired connectivity between the amygdala and other critical regions in the 'social brain'.
Individuals with WS have a very endearing personality. They have a unique strength in their expressive language skills, and are extremely polite. They are typically unafraid of strangers and show a greater interest in contact with adults than with peers.
Most children with Williams Syndrome are able to master self help skills and often complete vocational or other educational programs. This allows them to be able to lead productive lives.
A genetic disorder in children with consistent findings of distinctive, characteristic facial features, a heart defect, elevated calcium levels associated with 'colic-like' symptoms as infants, and autistic-like behaviors.
In this chapter, we present first the unusual neuropsychological profile of WMS, a profile of peaks and valleys of abilities within and across domains of higher cognitive functioning. We then review the results of recent studies on the neuroanatomic basis of WMS and its neurophysiological characteristics, and the implications of this research program for an understanding of the neural systems that subserve language and cognitive functioning.