Hiperacusia: Sindrome de Williams

Using novel control groups to dissect the amygdala's role in Williams syndrome

Tricia A. Thornton-Wells^a, Suzanne N. Avery^b and Jennifer Urbano Blackford^{b, , , ,}

^a Center for Human Genetics Research, Vanderbilt University Medical Center, 519 Light Hall, Nashville, TN 37232, United States
^b Department of Psychiatry, Vanderbilt University Medical Center, 1601 23rd Avenue South, Suite 3057 J, Nashville, TN 37212, United States

Received 20 October 2010; 

revised 7 March 2011; 

accepted 19 March 2011. 

Available online 29 March 2011.

Abstract

Williams syndrome is a neurodevelopmental disorder with an intriguing behavioral phenotype—hypersociability combined with significant non-social fears.
Previous studies have demonstrated abnormalities in amygdala function in individuals with Williams syndrome compared to typically developing controls.
However, it remains unclear whether the findings are related to the atypical neurodevelopment of Williams syndrome, or are also associated with behavioral traits at the extreme end of a normal continuum.

We used functional magnetic resonance imaging (fMRI) to compare amygdala blood-oxygenation-level-dependent (BOLD) responses to non-social and social images in individuals with Williams syndrome compared to either individuals with inhibited temperament (high non-social fear) or individuals with uninhibited temperament (high sociability).

Individuals with Williams syndrome had larger amygdala BOLD responses when viewing the non-social fear images than the inhibited temperament control group.

In contrast, when viewing both fear and neutral social images, individuals with Williams syndrome did not show smaller amygdala BOLD responses relative to the uninhibited temperament control group, but instead had amygdala responses proportionate to their sociability.

These results suggest heightened amygdala response to non-social fear images is characteristic of WS, whereas, variability in amygdala response to social fear images is proportionate to, and might be explained by, levels of trait sociability.

Highlights

► Williams syndrome (WS) is characterized by hypersociability contrasted with non-social fears. ► Amygdala response to non-social fear images is increased in WS versus inhibited temperament. ► Amygdala response to social fear images is not decreased in WS versus uninhibited temperament. ► Comparison groups with similar behavioral traits can elucidate neural bases of disorders.

Keywords: Williams syndrome; Inhibited temperament; Amygdala; Sociability; Anxiety; fMRI

Developmental Cognitive Neuroscience

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doi:10.1016/j.dcn.2011.03.003 |