Temper outbursts in Lowe syndrome: Characteristics, sequence, environmental context and comparison to Prader–Willi syndrome
Helen Cressey
The Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Birmingham, UK
Search for more papers by this authorChris Oliver
The Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Birmingham, UK
Search for more papers by this authorHayley Crawford
The Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Birmingham, UK
Faculty of Health and Life Sciences, Coventry University, Coventry, UK
Search for more papers by this authorCorresponding Author
Jane Waite
School of Life and Health Sciences, Aston University, Birmingham, UK
Correspondence
Jane Waite, School of Life and Health Sciences, Aston University, Aston Triangle, Birmingham, B4 7ET, UK.
Email: [email protected]
Search for more papers by this authorHelen Cressey
The Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Birmingham, UK
Search for more papers by this authorChris Oliver
The Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Birmingham, UK
Search for more papers by this authorHayley Crawford
The Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Birmingham, UK
Faculty of Health and Life Sciences, Coventry University, Coventry, UK
Search for more papers by this authorCorresponding Author
Jane Waite
School of Life and Health Sciences, Aston University, Birmingham, UK
Correspondence
Jane Waite, School of Life and Health Sciences, Aston University, Aston Triangle, Birmingham, B4 7ET, UK.
Email: [email protected]
Search for more papers by this authorFunding information
This research formed part of a larger study on the behavioural phenotype of Lowe syndrome, funded by the Lowe Syndrome Trust, UK. Thanks are also due to the Lowe Syndrome Association, USA, for assistance with participant recruitment.
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 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.
REFERENCES
- Allmendinger, A. M., Desai, N. S., Burke, A. T., Viswanadhan, N., & Prabhu, S. (2014). Neuroimaging and renal ultrasound manifestations of oculocerebrorenal syndrome of Lowe. Journal of Radiology Case Reports, 8(10), 1–7. https://doi.org/10.3941/jrcr.v8i10.1740
- Arron, K., Oliver, C., Moss, J., Berg, K., & Burbidge, C. (2011). The prevalence and phenomenology of self-injurious and aggressive behaviour in genetic syndromes. Journal of Intellectual Disability Research, 55(2), 109–120. https://doi.org/10.1111/j.1365-2788.2010.01337.x
- Beavers, G. A., Iwata, B. A., & Lerman, D. C. (2013). Thirty years of research on the functional analysis of problem behavior. Journal of Applied Behavior Analysis, 46(1), 1–21. https://doi.org/10.1002/jaba.30
- Beresford-Webb, J., Manning, K., Aman, L., & Ring, H. (2018). Vagus nerve stimulation for the treatment of problem behaviour in people with Prader-Willi syndrome [Abstract]. Journal of Applied Research in Intellectual Disabilities, 31, 547.
- Bhatia, M. S., Dhar, N. K., Singhal, P. K., Nigam, V. R., Malik, S. C., & Mullick, D. N. (1990). Temper tantrums. Prevalence and etiology in a non-referral outpatient setting. Clinical Pediatrics, 29(6), 311–315.
- Bull, L. E., Oliver, C., & Woodcock, K. A. (2017). Signalling changes to individuals who show resistance to change can reduce challenging behaviour. Journal of Behavior Therapy and Experimental Psychiatry, 54, 58–70. https://doi.org/10.1016/j.jbtep.2016.06.006
- Carlson, G. A., Potegal, M., Margulies, D., Gutkovich, Z., & Basile, J. (2009). Rages–what are they and who has them? Journal of Child and Adolescent Psychopharmacology, 19(3), 281–288.
- Carr, E. G., & Durand, V. M. (1985). Reducing behavior problems through functional communication training. Journal of Applied Behavior Analysis, 18(2), 111–126.
- Cornish, K. M., & Pigram, J. (1996). Developmental and behavioural characteristics of Cri du chat syndrome. Archives of Disease in Childhood, 75(5), 448–450.
- Dolinsky, Z., Jacobs, D., & Knight, C. (2008). Report of the Lowe Syndrome Association comprehensive survey. The Lowe Syndrome Association, USA. Emerson.
- Douglas, H. (2007). Containment and reciprocity: Integrating psychoanalytic theory and child development research for work with children. Hove, UK: Routledge.
10.4324/9780203961285 Google Scholar
- Dykens, E. M., Hodapp, R. M., & Finucane, B. M. (2000). Behaviour and development in genetic mental retardation syndromes. Baltimore, Md.; London: Paul H Brookes.
- Emerson, E. (1993). Challenging behaviours and severe learning disabilities: Recent developments in behavioural analysis and intervention. Behavioural and Cognitive Psychotherapy, 21(3), 171–198.
10.1017/S1352465800010468 Google Scholar
- Grapel, J. N., Cicchetti, D. V., & Volkmar, F. R. (2015). Sensory features as diagnostic criteria for autism: Sensory features in autism. The Yale Journal of Biology and Medicine, 88(1), 69–71.
- Hunter, S. J., & Sparrow, E. P. (2012). Executive function and dysfunction: Identification, assessment and treatment. Cambridge, UK: Cambridge University Press.
10.1017/CBO9780511977954 Google Scholar
- Iwata, B. A., Pace, G. M., Dorsey, M. F., Zarcone, J. R., Vollmer, T. R., Smith, R. G., & Willis, K. D. (1994). The functions of self-injurious behavior: An experimental- epidemiological analysis. Journal of Applied Behavior Analysis, 27(2), 215–240. https://doi.org/10.1901/jaba.1994.27-215
- John, A. E., & Mervis, C. B. (2010). Sensory modulation impairments in children with Williams syndrome. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 154C(2), 266–276. https://doi.org/10.1002/ajmg.c.30260
- Kenworthy, L., & Charnas, L. (1995). Evidence for a discrete behavioral phenotype in the oculocerebrorenal syndrome of Lowe. American Journal of Medical Genetics, 59(3), 283–290.
- Kenworthy, L., Park, T., & Charnas, L. R. (1993). Cognitive and behavioral profile of the oculocerebrorenal syndrome of Lowe. American Journal of Medical Genetics, 46(3), 297–303.
- Lewis, R. A., Nussbaum, R. L., & Brewer, E. D. (2012). Lowe Syndrome. R. A. In Pagon, M. P. Adam, H. H. Ardinger, S. E. Wallace, A. Amemiya, L. J. Bean, & K. Stephens (Eds.), GeneReviews(®). Seattle (WA): University of Washington, Seattle. Retrieved from http://www.ncbi.nlm.nih.gov/books/NBK1480/
- Loi, M. (2006). Lowe syndrome. Orphanet Journal of Rare Diseases, 1, 16. https://doi.org/10.1186/1750-1172-1-16
- Lowe, K., Allen, D., Jones, E., Brophy, S., Moore, K., & James, W. (2007). Challenging behaviours: Prevalence and topographies. Journal of Intellectual Disability Research, 15(8), 625–636.
- Manning, K. E., McAllister, C. J., Ring, H. A., Finer, N., Kelly, C. L., Sylvester, K. P., … Holland, A. J. (2016). Novel insights into maladaptive behaviours in Prader-Willi syndrome: Serendipitous findings from an open trial of vagus nerve stimulation. Journal of Intellectual Disability Research, 60(2), 149–155. https://doi.org/10.1111/jir.12203
- McGill, P. (1999). Establishing operations: Implications for the assessment, treatment, and prevention of problem behavior. Journal of Applied Behavior Analysis, 32(3), 393–418. https://doi.org/10.1901/jaba.1999.32-393
- Moss, J., Oliver, C., Arron, K., Burbidge, C., & Berg, K. (2009). The prevalence and phenomenology of repetitive behavior in genetic syndromes. Journal of Autism and Developmental Disorders, 39(4), 572–588. https://doi.org/10.1007/s10803-008-0655-6
- Oliver, C., McClintock, K., Hall, S., Smith, M., Dagnan, D., & Stenfert-Kroese, B. (2003). Assessing the severity of challenging behaviour: Psychometric properties of the challenging behaviour interview. Journal of Applied Research in Intellectual Disabilities, 16(1), 53–61. https://doi.org/10.1046/j.1468-3148.2003.00145.x
- Österman, K., & Björkqvist, K. (2010). A cross-sectional study of onset, cessation, frequency, and duration of children’s temper tantrums in a non-clinical sample. Psychological Reports, 106(2), 448–454.
- Potegal, M., Carlson, G., Margulies, D., Gutkovitch, Z., & Wall, M. (2009). Rages or temper tantrums? The behavioral organization, temporal characteristics, and clinical significance of angry-agitated outbursts in child psychiatry inpatients. Child Psychiatry and Human Development, 40(4), 621–636. https://doi.org/10.1007/s10578-009-0148-7
- Potegal, M., & Davidson, R. J. (2003). Temper tantrums in young children: 1. Behavioral composition. Journal of Developmental and Behavioral Pediatrics: JDBP, 24(3), 140–147.
- Potegal, M., Kosorok, M. R., & Davidson, R. J. (1996). The time course of angry behavior in the temper tantrums of young children. Annals of the New York Academy of Sciences, 794(1), 31–45.
- Rice, L. J., Woodcock, K., & Einfeld, S. L. (2018). The characteristics of temper outbursts in Prader-Willi syndrome. American Journal of Medical Genetics Part A., 176A, 2292–2300. https://doi.org/10.1002/ajmg.a.40480
- Smith, S., Branford, D., Collacott, R. A., Cooper, S.-A., & McGrother, C. (1996). Prevalence and cluster typology of maladaptive behaviours in a geographically defined population of adults with learning disabilities. The British Journal of Psychiatry, 169(2), 219–227.
- Sparrow, S. S., Cicchetti, D. V., Balla, D. A., & Doll, E. A. (2005). Vineland-II: Vineland Adaptive Behavior Scales. Minneapolis, MN: Pearson Assessments.
- Tunnicliffe, P. L. (2012). An investigation into temper outburst behaviour in people with Prader-Willi syndrome. Unpublished doctoral thesis., University of Birmingham. Doctorate in Clinical Psychology.
- Tunnicliffe, P., & Oliver, C. (2011). Phenotype–environment interactions in genetic syndromes associated with severe or profound intellectual disability. Research in Developmental Disabilities, 32(2), 404–418. https://doi.org/10.1016/j.ridd.2010.12.008
- Tunnicliffe, P., Woodcock, K., Bull, L., Oliver, C., & Penhallow, J. (2014). Temper outbursts in Prader-Willi syndrome: Causes, behavioural and emotional sequence and responses by carers. Journal of Intellectual Disability Research, 58(2), 134–150. https://doi.org/10.1111/jir.12010
- Waite, J., Heald, M., Wilde, L., Woodcock, K., Welham, A., Adams, D., & Oliver, C. (2014). The importance of understanding the behavioural phenotypes of genetic syndromes associated with intellectual disability. Paediatrics and Child Health, 24(10), 468–472. https://doi.org/10.1016/j.paed.2014.05.002
10.1016/j.paed.2014.05.002 Google Scholar
- Wakschlag, L. S., Briggs-Gowan, M. J., Carter, A. S., Hill, C., Danis, B., Keenan, K., … Leventhal, B. L. (2007). A developmental framework for distinguishing disruptive behavior from normative misbehavior in preschool children. Journal of Child Psychology and Psychiatry, 48(10), 976–987.
- Warren, S. A., & Mondy, L. W. (1971). To what behaviors do attending adults respond? American Journal of Mental Deficiency, 75(4), 449–455.
- Woodcock, K. A., Humphreys, G. W., Oliver, C., & Hansen, P. C. (2010). Neural correlates of task switching in paternal 15q11–q13 deletion Prader-Willi syndrome. Brain Research, 1363, 128–142. https://doi.org/10.1016/j.brainres.2010.09.093
- Yuksel, A., Karaca, E., & Albayram, M. S. (2009). Magnetic resonance imaging, magnetic resonance spectroscopy, and facial dysmorphism in a case of Lowe syndrome with novel OCRL1 gene mutation. Journal of Child Neurology, 24(1), 93–96. https://doi.org/10.1177/0883073808321047
