Background
Williams syndrome (WS) has provided researchers with an exciting opportunity to understand
the complex interplay among genes, neurobiological and cognitive functions. However,
despite a well-characterized cognitive and behavioral phenotype, little attention
has been paid to the marked deficits in social and behavioral inhibition. Here we
explore the neural systems that mediate response inhibition in WS.
Methods
We used functional MRI (fMRI) to obtain blood oxygenation level dependence (BOLD)
signal maps during the performance of a Go/NoGo response inhibition task from 11 clinically
and genetically diagnosed WS patients and 11 age- and gender-matched typically developing
(TD) control subjects. Correlations between behavioral, neuropsychological measures,
and BOLD signal were also conducted.
Results
Although TD control subjects showed significantly faster response times, no group
differences in behavioral accuracy were observed. Compared with control subjects,
WS participants demonstrated significantly reduced activity in the striatum, dorsolateral
prefrontal, and dorsal anterior cingulate cortices. These findings support the hypothesis
that persons with WS fail to activate critical cortical and subcortical structures
involved in behavioral inhibition.
Conclusions
Our results provide important evidence for reduced engagement of the frontostriatal
circuits in WS and provide putative biological markers for the deficits in response
inhibition and the unusual social phenotype.
Key Words
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Article info
Publication history
Published online: September 25, 2006
Accepted:
May 23,
2006
Received in revised form:
May 9,
2006
Received:
March 15,
2006
Identification
Copyright
© 2007 Society of Biological Psychiatry. Published by Elsevier Inc. All rights reserved.
