Elsevier

NeuroImage

Volume 50, Issue 1, March 2010, Pages 340-346
NeuroImage

Abnormalities in neural processing of emotional stimuli in Williams syndrome vary according to social vs. non-social content

https://doi.org/10.1016/j.neuroimage.2009.11.069Get rights and content

Abstract

Williams syndrome (WS) is a rare genetic disorder caused by the deletion of ∼ 25 genes on chromosome 7q11.23 and is characterized by both hypersociability and increases in specific phobia and anticipatory anxiety regarding non-social entities or circumstances. Alterations in amygdala reactivity and prefrontal regulation consistent with the observed behavioral pattern of social versus non-social abnormalities have been previously demonstrated in individuals with WS (Meyer-Lindenberg et al., 2005). However, in that study, the social stimulus (faces) matching task was more difficult than the non-social scene (IAPS stimuli) matching task, making it impossible to disambiguate the relative contributions of task difficulty and stimulus type (social versus non-social). In the present study, we examined the performance of the same group of participants with WS and normal IQs during a more cognitively demanding task using the same scene stimuli as in the prior study. Confirming previous findings, the results indicated (a) a differential response of prefrontal regions as a function of task difficulty and (b) a persistently increased activation of the amygdala to non-social scenes by individuals with WS regardless of cognitive load. These data provide further evidence of disruption in amygdala-prefrontal circuitry in individuals with WS.

Section snippets

Participants

Demographics are summarized in Table 1. Thirteen normal-IQ individuals with WS and 13 general-population controls were matched for age, sex, and IQ. Three individuals in each group participated only in the matching experiment. All participants gave written informed consent and participated in the study according to the guidelines of the National Institute of Mental Health Institutional Review Board and were reimbursed for their time.

To rule out psychiatric disorders, healthy controls were

Behavior

Behavioral results are summarized in Table 1. Reflecting task difficulty, there were significantly fewer correct responses during the scene-labeling task than during scene-matching (NC: F(1,21) = 4.97, p < 0.04; WS: F(1,21) = 25.86, p < 0.001) and reaction times were significantly longer in the labeling experiment (NC: F(1,21) = 4.48, p < 0.05; WS: F(1,21) = 25.86, p < 0.001), reflecting increased cognitive demand. Neither accuracy (Label: F(1,18) = 2.07, p = 0.17; Match: F(1,24) = 2.27, p = 0.15) nor reaction time

Discussion

The purpose of the present experiment was to disambiguate between the effects of cognitive load and stimulus type on differential amygdala and prefrontal activation in individuals with WS. Together with our previous results (Meyer-Lindenberg et al., 2005), the present experiment establishes (a) differential activation in prefrontal regions as a consequence of task difficulty and (b) hyperreactivity of amygdala to non-social fearful scenes in individuals with WS regardless of cognitive load.

Acknowledgments

We thank Neha Dixit, Aaron-Bonner Jackson, Rosanna Olsen, John Holt, and Yunxia Tong for research assistance and Daniel Weinberger for helpful discussion of this manuscript. This work was supported by DHHS/NIH/NIMH/IRP and NINDS grant no NS35102 (C.B.M., principal investigator). K.E.M. is supported by the Multimodal Neuroimaging Training Program Fellowship: 1T90DA022761. A.R.H is supported by the National Institute of Mental Health: MH072837 and the National Alliance for Research on

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  • Cited by (0)

    1

    Current address: Department of Psychology and Center for the Neural Basis of Cognition, University of Pittsburgh, Pittsburgh, PA 15260, USA.

    2

    These authors contributed equally.

    3

    Current address: Central Institute of Mental Health, University of Heidelberg/Medical Faculty Mannheim, J5, 68159 Mannheim, Germany.

    4

    Current address: Departments of Psychology and Neuroscience, Institute for Genome Sciences and Policy, Duke University, Durham, NC 27708, USA.

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