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Temper outbursts in Lowe syndrome: Characteristics, sequence, environmental context and comparison to Prader–Willi syndrome
- Authors:
- CRESSEY Helen, et al
- Journal article citation:
- Journal of Applied Research in Intellectual Disabilities, 32(5), 2019, pp.1216-1227.
- Publisher:
- Wiley
Background: There is limited research into the nature and aetiology of temper outbursts in people with intellectual disabilities. This study describes the phenomenology and environmental context of temper outbursts in Lowe syndrome, a rare genetic syndrome in which outbursts are purportedly frequent. Method: A temper outburst interview (TOI) was conducted with caregivers of seventeen individuals with Lowe syndrome to generate an account of the behavioural sequence, common antecedents and consequences of temper outbursts, and to enable comparisons with similar work on Prader–Willi syndrome. Results: Outbursts in Lowe syndrome were frequently triggered by thwarted goal‐directed behaviour and were associated with high levels of physical aggression and property destruction. Conclusions: Form and sequence of outbursts showed similarities to Prader–Willi syndrome and to behaviours reported in literature on typically developing children. The results highlight the importance of considering shared aetiology as well as syndrome‐specific pathways in the development of outbursts. (Edited publisher abstract)
Maternal parenting stress in families with a child with Angelman syndrome or Prader-Willi syndrome
- Authors:
- WULFFAERT Josette, SCHOLTE Evert M., BERCKELAER-ONNES Ina A.
- Journal article citation:
- Journal of Intellectual and Developmental Disability, 35(3), September 2010, pp.165-174.
- Publisher:
- Taylor and Francis
To assess maternal parenting stress mothers of 24 children with Angelman syndrome (AS) and 23 children with Prader-Willi syndrome (PWS) completed the Nijmegen Parenting Stress Index-Short (NPSI-S), the Dutch version of the Developmental Behaviour Checklist-Primary Carer, and the Vineland Screener 0-12 years assessment instruments. Children were aged between 2 and 12 years and were living at home. Maternal parenting stress was coded as high in 58% of mothers with a child with AS and 26% of mothers with a child with PWS. For both syndromes no relationship existed with the child’s gender, age or behaviour problems. In PWS there was no effect on level of functioning. Overall, more mothers with a child with AS perceived high parenting stress. When children showed low levels of behavioural problems this difference was contained. However, when children exhibited severe behavioural problems, parenting stress was the same for both syndromes. The authors conclude that the findings suggest that professional support is needed for families with a child with AS because stress levels are high in a large proportion of mothers, which can have a negative influence on parenting behaviour. In PWS the need for support is more prominent when the child exhibits substantial behavioural problems.