Temper outbursts in Lowe syndrome:  Characteristics, sequence, environmental context and comparison to Prader–Willi syndrome

Helen Cressey; Chris  Oliver; Hayley Crawford; Jane Waite

doi:10.1111/jar.12613

Temper outbursts in Lowe syndrome: Characteristics, sequence, environmental context and comparison to Prader–Willi syndrome

Helen Cressey, Chris Oliver, Hayley Crawford, Jane Waite^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

Abstract

Background: There is limited research into the nature and aetiology of temper outbursts in people with intellectual disabilities. In this study, we describe 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 goaldirected 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.

Original language	English
Pages (from-to)	1216-1227
Number of pages	12
Journal	Journal of Applied Research in Intellectual Disabilities
Volume	32
Issue number	5
Early online date	29 May 2019
DOIs	https://doi.org/10.1111/jar.12613
Publication status	Published - Sept 2019

Bibliographical note

© 2019 The Authors. Journal of Applied Research in Intellectual Disabilities Published by John Wiley & Sons Ltd

This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

Funding: Lowe Syndrome Trust.

Keywords

behavioural phenotypes
challenging behaviours
intellectual disabilities
Lowe syndrome
temper outbursts

Access to Document

10.1111/jar.12613

Temper outbursts in Lowe syndrome - Waite
© 2019 The Authors. Journal of Applied Research in Intellectual Disabilities Published by John Wiley & Sons Ltd This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Final published version, 682 KBLicence: CC BY 3.0

Cite this

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title = "Temper outbursts in Lowe syndrome: Characteristics, sequence, environmental context and comparison to Prader–Willi syndrome",

abstract = "Background: There is limited research into the nature and aetiology of temper outbursts in people with intellectual disabilities. In this study, we describe 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 goaldirected 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.",

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N1 - © 2019 The Authors. Journal of Applied Research in Intellectual Disabilities Published by John Wiley & Sons Ltd This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. Funding: Lowe Syndrome Trust.

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N2 - Background: There is limited research into the nature and aetiology of temper outbursts in people with intellectual disabilities. In this study, we describe 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 goaldirected 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.

AB - Background: There is limited research into the nature and aetiology of temper outbursts in people with intellectual disabilities. In this study, we describe 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 goaldirected 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.

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