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Journal of Neurodevelopmental Disorders

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Open Access 01-12-2013 | Research

Social and emotional processing in Prader-Willi syndrome: genetic subtype differences

Authors: Alexandra P Key, Dorita Jones, Elisabeth M Dykens

Published in: Journal of Neurodevelopmental Disorders | Issue 1/2013

Abstract

Background

People with Prader-Willi syndrome (PWS) demonstrate social dysfunction and increased risk of autism spectrum disorder, especially those with the maternal uniparental disomy (mUPD) versus paternal deletion genetic subtype. This study compared the neural processing of social (faces) and nonsocial stimuli, varying in emotional valence, across genetic subtypes in 24 adolescents and adults with PWS.

Methods

Upright and inverted faces, and nonsocial objects with positive and negative emotional valence were presented to participants with PWS in an oddball paradigm with smiling faces serving as targets. Behavioral and event-related potential (ERP) data were recorded.

Results

There were no genetic subtype group differences in accuracy, and all participants performed above chance level. ERP responses revealed genetic subtype differences in face versus object processing. In those with deletions, the face-specific posterior N170 response varied in size for face stimuli versus inverted faces versus nonsocial objects. Persons with mUPD generated N170 of smaller amplitude and showed no stimulus differentiation. Brain responses to emotional content did not vary by subtype. All participants elicited larger posterior and anterior late positive potential responses to positive objects than to negative objects. Emotion-related differences in response to faces were limited to inverted faces only in the form of larger anterior late positive potential amplitudes to negative emotions over the right hemisphere. Detection of the target smiling faces was evident in the increased amplitude of the frontal and central P3 responses but only for inverted smiling faces.

Conclusion

Persons with the mUPD subtype of PWS may show atypical face versus object processes, yet both subtypes demonstrated potentially altered processing, attention to and/or recognition of faces and their expressions.

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Title: Social and emotional processing in Prader-Willi syndrome: genetic subtype differences
Authors: Alexandra P Key
Dorita Jones
Elisabeth M Dykens
Publication date: 01-12-2013
Publisher: BioMed Central
Published in: Journal of Neurodevelopmental Disorders / Issue 1/2013
Print ISSN: 1866-1947
Electronic ISSN: 1866-1955
DOI: https://doi.org/10.1186/1866-1955-5-7

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Social and emotional processing in Prader-Willi syndrome: genetic subtype differences

Abstract

Background

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Methods

Results

Conclusion

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