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Frontostriatal Dysfunction During Response Inhibition in Williams Syndrome

Mobbs, Dean and Eckert, Mark A. and Mills, Debra and Korenberg, Julie and Bellugi, Ursula and Galaburda, Albert M. and Reiss, Allan L. (2007) Frontostriatal Dysfunction During Response Inhibition in Williams Syndrome. Biological Psychiatry, 62 (3). pp. 256-261. ISSN 0006-3223. doi:10.1016/j.biopsych.2006.05.041.

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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

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Mobbs, Dean0000-0003-1175-3772
Additional Information:© 2007 Society of Biological Psychiatry. Received 15 March 2006, Revised 9 May 2006, Accepted 23 May 2006, Available online 25 September 2006. The authors thank Asya Karchemskiy, J. Eric Schmitt, Vinod Menon, Adam Tenforde, and Katie McKenzie for their help with data acquisition and analysis. This study was supported by the National Institute of Health (Grant Nos. MH01142, MH50047, HD31715, HD33113, and HD40761 to ALR).
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Subject Keywords:fMRI; Go/NoGo; prefrontal cortex; response inhibition; striatum; Williams syndrome
Issue or Number:3
Record Number:CaltechAUTHORS:20180305-071218227
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Official Citation:Dean Mobbs, Mark A. Eckert, Debra Mills, Julie Korenberg, Ursula Bellugi, Albert M. Galaburda, Allan L. Reiss, Frontostriatal Dysfunction During Response Inhibition in Williams Syndrome, Biological Psychiatry, Volume 62, Issue 3, 2007, Pages 256-261, ISSN 0006-3223, (
Usage Policy:No commercial reproduction, distribution, display or performance rights in this work are provided.
ID Code:85082
Deposited On:05 Mar 2018 23:48
Last Modified:15 Nov 2021 20:25

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