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The characteristics of temper outbursts in Prader-Willi syndrome

NOV 2018 – In this study, Drs. Rice, Einfeld and Woodcock aimed to develop a comprehensive understanding of temper outbursts in 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 behaviours 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 daily medications prescribed for outbursts, though parents rated them only somewhat effective and reported only minor effects. A small number of individuals had been prescribed medication to use during a tantrum with varying effectiveness (Valium rated as not effective while Lorazepam & Quetiapine rated as effective).

More detailed information can be found at the FPWR website. Read More >