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Emotion Potentiated Startle in Fragile X Syndrome

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Abstract

Social avoidance and anxiety are prevalent in fragile X syndrome (FXS) and are potentially mediated by the amygdala, a brain region critical for social behavior. Unfortunately, functional brain resonance imaging investigation of the amygdala in FXS is limited by the difficulties experienced by intellectually impaired and anxious participants. We investigated the relationship between social avoidance and emotion-potentiated startle, a probe of amygdala activation, in children and adolescents with FXS, developmental disability without FXS (DD), and typical development. Individuals with FXS or DD demonstrated significantly reduced potentiation to fearful faces than a typically developing control group (p < .05). However, among individuals with FXS, social avoidance correlated positively with fearful-face potentiation (p < .05). This suggests that general intellectual disability blunts amygdalar response, but differential amygdala responsiveness to social stimuli contributes to phenotypic variability among individuals with FXS.

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Acknowledgments

The authors thank Lori Abucayan, Dr. Aaron Campbell, Susan Harris, Maisha Ollison, Dr. Andrea Schneider and Dr. Jennifer Yuhas-Duffy for their contribution to this research. Additionally, thanks to Dr. David Amaral for his suggestion to examine fear potentiated startle as a probe of amygdala involvement in the fragile X syndrome phenotype. We are especially thankful to the participants and their families for their contribution to the understanding of FXS. This work was supported by National Institute of Mental Health Grant MH7754. A version of this paper was presented at the 8th Annual International Meeting for Autism Research in 2009 and at the 12th Annual International Fragile X Conference in 2010.

Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Graduate Program in Neuroscience, Department of Neurobiology and Behavior, Stony Brook University, Stony Brook, NY, 11794-5230, USA

    Elizabeth C. Ballinger

  2. Department of Pediatrics, University of Colorado School of Medicine, 13123 East 16th Ave., B140, Aurora, CO, 80045, USA

    Lisa Cordeiro

  3. Empirical Education, 425 Sherman Avenue, Suite 210, Palo Alto, CA, 94306-1850, USA

    Alyssa D. Chavez

  4. Department of Pediatrics, MIND Institute, University of California at Davis Medical Center, 2825 50th Street, Sacramento, CA, 95817, USA

    Randi J. Hagerman

  5. Department of Psychiatry and Behavioral Sciences, MIND Institute, University of California at Davis Medical Center, 2825 50th Street, Sacramento, CA, 95817, USA

    David Hessl

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Correspondence to David Hessl.

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Ballinger, E.C., Cordeiro, L., Chavez, A.D. et al. Emotion Potentiated Startle in Fragile X Syndrome. J Autism Dev Disord 44, 2536–2546 (2014). https://doi.org/10.1007/s10803-014-2125-7

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