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

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) V...

Full description

Saved in:
Bibliographic Details
Published in:Journal of child psychology and psychiatry 2008-06, Vol.49 (6), p.576-608
Main Authors: Martens, Marilee A., Wilson, Sarah J., Reutens, David C.
Format: Article
Language:English
Subjects:
Adult and adolescent clinical studies
Behavior
Behavior Patterns
Biological and medical sciences
Brain - pathology
Child
Child Behavior Disorders - diagnosis
Child Behavior Disorders - genetics
Child Behavior Disorders - pathology
Child clinical studies
Child psychology
cognition
Cognition & reasoning
Cognition Disorders - diagnosis
Cognition Disorders - genetics
Cognition Disorders - pathology
Control Groups
Developmental disabilities
Developmental disorders
Emotions
Facial Expression
Genetic disorders
Humans
Intellectual deficiency
Intelligence
language
Language Development Disorders - diagnosis
Language Development Disorders - genetics
Language Development Disorders - pathology
Language Skills
Medical sciences
music
Neuropsychological Tests
Neurosciences
Pattern Recognition, Visual
Personality Assessment
Phenotype
Psychology. Psychoanalysis. Psychiatry
Psychopathology. Psychiatry
review
Social Behavior
Williams syndrome
Williams Syndrome - diagnosis
Williams Syndrome - genetics
Williams Syndrome - pathology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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 sample size, use and type of control groups, and multiple measures of task performance. Previously described ‘peaks’ within the cognitive profile are closely examined to assess their veracity. This review highlights the need for methodologically sound studies that utilize multiple comparison groups, developmental trajectories, and longitudinal analyses to examine the WS phenotype, as well as those that link brain structure and function to the cognitive and behavioral phenotype of WS individuals.
ISSN:0021-9630
1469-7610
DOI:10.1111/j.1469-7610.2008.01887.x