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Am J Med Genet A. 2018 Nov;176(11):2292-2300. doi: 10.1002/ajmg.a.40480. Epub 2018 Oct 5.

The characteristics of temper outbursts in Prader-Willi syndrome.

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The Brain and Mind Centre, The University of Sydney, Camperdown, New South Wales, Australia.
Centre for Applied Psychology, School of Psychology, University of Birmingham, Birmingham, United Kingdom.
Institute for Mental Health, University of Birmingham, Birmingham, United Kingdom.


The purpose of this study was to develop a comprehensive understanding of temper outbursts in Prader-Willi syndrome (PWS). A survey was developed from interviews conducted with individuals with PWS and their caregivers. The survey was completed by 101 primary caregivers. The findings suggest that outburst frequency decreases with age while duration increases. Adolescents exhibited more severe behaviors than children or adults. No differences were found across gender or genetic subtype. Provocations fit into three themes: goal blockage, social injustice, and difficulty dealing with change. Distracting the person or giving them space to calm down were the only management strategies judged effective. Risperidone, sertraline, and fluoxetine were the most common medications prescribed for outbursts, though parents reported only minor effects.


Prader-Willi syndrome; aggression; outbursts; rages


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