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Abnormal cortical complexity and thickness profiles mapped in Williams syndrome
Paul M Thompson1, Agatha D Lee, Rebecca A Dutton
1Laboratory of Neuroimaging, Brain Mapping Division, Department of Neurology, University of California Los Angeles School of Medicine, Los Angeles, California 90095-1769, USA. thompson@loni.ucla.edu <thompson@loni.ucla.edu>
Summary
Researchers found a specific area of the right brain cortex shows abnormal thickening in Williams syndrome (WS), a genetic disorder. This contrasts with overall brain volume reduction, highlighting localized developmental differences in WS.
Area of Science:
- Neuroscience
- Genetics
- Developmental Biology
Background:
- Williams syndrome (WS) is a genetic disorder caused by a deletion on chromosome 7, often leading to developmental abnormalities.
- Previous research indicates pervasive gray and white matter deficits and reduced total cerebral volumes in individuals with WS.
Purpose of the Study:
- To identify and map localized failures in cortical maturation in Williams syndrome.
- To investigate alterations in cortical thickness and surface complexity in the brains of individuals with WS compared to controls.
Main Methods:
- Utilized magnetic resonance imaging (MRI) scans from 42 individuals with WS and 40 healthy controls.
- Generated detailed three-dimensional (3D) cortical thickness maps using a pattern-matching technique with sulcal landmarks.
- Computed fractal dimension (surface complexity) of cortical models for each hemisphere and lobe.
Main Results:
- Identified a delimited zone of right hemisphere perisylvian cortex with increased thickness (5-10%) in WS.
- Observed significantly increased surface complexity in both left and right hemispheres of individuals with WS.
- Found positive correlations between cortical thickness and complexity in controls, but this relationship was altered in WS.
Conclusions:
- Williams syndrome is characterized by a localized failure of cortical maturation, specifically an area of thickened cortex in the right hemisphere.
- Increased cortical complexity is a feature of WS brains, suggesting altered neurodevelopmental processes.
- These findings provide a detailed anatomical map of brain alterations in WS, warranting further functional and connectivity studies.