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… 
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423 Citations
Highly Influential Citations
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Background Citations
145
Methods Citations
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Results Citations
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423 Citations

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…

Williams syndrome-specific neuroanatomical profile and its associations with behavioral features

Chapter 5 - Williams Syndrome

Cognitive abilities in Williams syndrome.

Variability and standardized test profiles in typically developing children and children with Williams Syndrome.

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.

NeuroImage: Clinical Williams syndrome-speci fi c neuroanatomical pro fi le and its associations with behavioral features

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.

Williams syndrome: on the role of intellectual abilities in anxiety

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.

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…

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…

Neuropsychological Genotype-Phenotype in Patients with Williams Syndrome with Atypical Deletions: A Systematic Review.

A systematic review of case series reports and clinical case studies in Williams syndrome revealed four neuropsychological phenotypes, depending on the genes involved, and established the need for more comprehensive study of the neuropsychology profile of these patients.
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References

SHOWING 1-10 OF 241 REFERENCES

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…

Analysis of cerebral shape in Williams syndrome.

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.

Hypothesis for development of a behavioral phenotype in Williams syndrome.

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.

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…

Language, communication, and neural systems in Williams syndrome

The special characteristics of Williams syndrome provide an opportunity to challenge and develop theories of cognition and forge links between specific neuropsychological, neuroanatomical, and genetic underpinnings relevant to developmental cognitive neuroscience.

Williams syndrome: an exploration of neurocognitive and genetic features

An Experiment of Nature: Brain Anatomy Parallels Cognition and Behavior in Williams Syndrome

Findings point to specific neuroanatomical correlates for the unique topography of cognitive and behavioral features associated with this disorder.

Cognitive, adaptive, and behavioral characteristics of Williams syndrome.

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.

Characterizing the Musical Phenotype in Individuals With Williams Syndrome

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.

The Williams Syndrome Cognitive Profile

A Williams Syndrome Cognitive Profile (WSCP) is outlined that operationalizes the cognitive characteristics of the syndrome using measures of absolute and relative performance on subtests of the Differential Abilities Scales (Elliot, 1990a).
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