Research Review: Williams syndrome: a critical review of the cognitive, behavioral, and neuroanatomical phenotype.

@article{Martens2008ResearchRW,
  title={Research Review: Williams syndrome: a critical review of the cognitive, behavioral, and neuroanatomical phenotype.},
  author={Marilee Martens and Sarah J Wilson and David C. Reutens},
  journal={Journal of child psychology and psychiatry, and allied disciplines},
  year={2008},
  volume={49 6},
  pages={
          576-608
        }
}
This review critically examines the research findings which characterize the cognitive, behavioral, and neuroanatomical features of Williams syndrome (WS). This article analyzes 178 published studies in the WS literature covering the following areas: 1) General intelligence, 2) Language skills, 3) Visuospatial and face processing skills, 4) Behavior patterns and hypersociability, 5) Musical abilities, and 6) Brain structure and function. We identify methodological issues relating to small… Expand
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386 Citations
Highly Influential Citations
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106
Methods Citations
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Results Citations
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386 Citations

Citation Type

Citation Type

Williams syndrome and its cognitive profile: the importance of eye movements.
People with Williams syndrome (WS), a rare neurodevelopmental disorder that is caused by a deletion on the long arm of chromosome 7, often show an uneven cognitive profile with participants… Expand
Williams syndrome and its cognitive profile: the importance of eye movements
TLDR
Although more research on eye movements in WS is required, the importance of eye movements for cognitive development suggests a possible intervention pathway to improve cognitive abilities in this population. Expand
Williams syndrome-specific neuroanatomical profile and its associations with behavioral features
TLDR
The results imply that the unique behaviors in WS were mediated through the constellation of abnormalities in cortical-subcortical circuitry consistent in child WS and adult WS. Expand
Chapter 5 - Williams Syndrome
Publisher Summary This chapter explores the current state of knowledge regarding the cognitive, social, and behavioral profiles that are associated with Williams syndrome (WS) and research based… Expand
Social Brain Development in Williams Syndrome: The Current Status and Directions for Future Research
TLDR
This article discusses how distinctive social behaviors in WS may be associated with alterations or delays in the cortical representation of faces, connectivity within the ventral stream, structure and function of the amygdala and how long- and short-range connections develop within the brain. Expand
Cognitive abilities in Williams syndrome.
TLDR
Cognitive heterogeneity was found on the Woodcock Johnson III Tests of Cognitive Abilities, Australian Adaptation (WJ-III COG), with cognitive abilities ranging from profoundly impaired to superior and individualized profiles of strength and weakness varying considerably. Expand
Variability and standardized test profiles in typically developing children and children with Williams Syndrome.
TLDR
There was significantly less variability in younger WS participants for performance scores on Pattern Construction compared to the TD group, and methodological issues and the importance of taking CA into account in analyses will be discussed. Expand
Williams syndrome: on the role of intellectual abilities in anxiety
TLDR
Using ordinal logistic regression analyses, it is shown that decreasing intelligence quotient (IQ) and age are associated with elevated anxiety and low IQ was associated with higher risk of having an anxiety diagnosis. Expand
Relationship Between Brain Abnormalities and Cognitive Profile in Williams Syndrome
Previous studies have shown inconsistent results when reporting brain abnormalities in Williams syndrome (WS). This makes an interpretation of clinical and behavioural data uncertain in terms of… Expand
William’s Syndrome: Brain-Behavior Relationships and Implications for School Psychologist
William’s Syndrome (WS) is a rare neurodevelopmental disorder caused by a genetic abnormality, affecting about 1 in 10,000 people worldwide. While there are some behavioral similarities between WS… Expand
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References

SHOWING 1-10 OF 242 REFERENCES
Neuropsychological, neurological, and neuroanatomical profile of Williams syndrome.
TLDR
A dissociation between language and cognitive functions in Williams syndrome adolescents, in contrast to age- and IQ-matched Down syndrome adolescents is reported, indicating an unusual fractionation of higher cortical functioning, with marked cognitive deficits, but selective sparing of syntax. Expand
Analysis of cerebral shape in Williams syndrome.
TLDR
Subjects with WMS have significantly different cerebral shape from normal controls, perhaps due to decreased parieto-occipital lobe volumes relative to frontal regions and similar observation in the corpus callosum may be associated with a decreased size of the splenium in WMS. Expand
I. The Neurocognitive Profile of Williams Syndrome: A Complex Pattern of Strengths and Weaknesses
The rare, genetically based disorder, Williams syndrome (WMS), produces a constellation of distinctive cognitive, neuroanatomical, and electrophysiological features which we explore through the… Expand
Williams Syndrome: Neuropsychological Findings and Implications for Practice
Williams syndrome (WS) is a genetic disorder resulting from a deletion on Chromosome 7. Individuals with WS exhibit distinct physical features such as facial abnormalities, hypercalcemia, and… Expand
Hypothesis for development of a behavioral phenotype in Williams syndrome.
TLDR
Theoretical constructs from developmental behavioral genetics, attachment theory, operant conditioning, neuropsychology, and psychosocial theory considered interactively offer explanations for the characteristic behavioral phenotype of 6 factors of Williams syndrome. Expand
Language, communication, and neural systems in Williams syndrome
Purpose Williams syndrome, a rare, genetically based disorder, results in a distinctive facies, a specific heart defect, mental retardation, but remarkably spared language. We discuss results of a… Expand
Williams syndrome: an exploration of neurocognitive and genetic features
Abstract We report here on significant attempts to forge links between neurodevelopmental disorders, development of specific neuropsychological abilities, and the functional establishment of patterns… Expand
An Experiment of Nature: Brain Anatomy Parallels Cognition and Behavior in Williams Syndrome
TLDR
Findings point to specific neuroanatomical correlates for the unique topography of cognitive and behavioral features associated with this disorder. Expand
Cognitive, adaptive, and behavioral characteristics of Williams syndrome.
TLDR
A variety of cognitive, adaptive, and behavioral patterns have been shown to be possible in Williams syndrome; therefore, a single predictable cognitive or behavioral phenotype cannot be assumed. Expand
Characterizing the Musical Phenotype in Individuals With Williams Syndrome
TLDR
Compared to all other groups including the controls, the WS individuals displayed greater emotional responses to music, manifested interest in music at an earlier age, and spent more hours per week listening to music. Expand
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