Search results for ‘Subject term:"prader-willi syndrome"’ Sort:
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The relationship between components of the behavioural phenotype in Prader-Willi syndrome
- Authors:
- OLIVER C., WOODCOCK K.A., HUMPHREYS G.W.
- Journal article citation:
- Journal of Applied Research in Intellectual Disabilities, 22(4), July 2009, pp.403-407.
- Publisher:
- Wiley
Repetitive questions and temper outbursts form part of the behavioural phenotype of Prader-Willi syndrome (PWS). This study investigated the phenomenology of temper outbursts in PWS and their relationship with other PWS behavioural characteristics. Four individuals with PWS were observed (5–10 h), during a number of experimental and natural environment challenges, some of which were expected to trigger temper outbursts. Individual behaviours including crying, ignoring, arguing, questioning, stereotypy, frowning and posture changes were recorded and subjected to lag sequential analysis. All participants were significantly more likely to show repetitive questioning before more challenging behaviours such as crying, arguing or ignoring requests. Precursor behaviours such as frowning and stereotypical behaviour were identified in three participants. The authors conclude that temper outbursts in PWS may be associated with other PWS behavioural phenotypic characteristics such as repetitive questions and 'stubbornness'. A progression of behaviours may lead up to the most challenging temper outburst behaviours. This may have important implications for effective coping strategies.
Assessment of behavioral and psychiatric problems in people with Prader–Willi syndrome: a review of the literature
- Author:
- SPENDELOW Jason S.
- Journal article citation:
- Journal of Policy and Practice in Intellectual Disabilities, 8(2), June 2011, pp.104-112.
- Publisher:
- Wiley
Prader–Willi syndrome (PSW) is a rare genetic disorder that has a prevalence of 1 in 50,000 to 70,000 people and equal gender distribution. The clinical presentation of people with PWS involves a wide range of behavioural, psychiatric, neurocognitive, and medical problems. The aim of this review is to help clinicians make a comprehensive assessment of mental health problems with this population. People with PWS are particularly susceptible to the development of problems in the areas of hyperphagia, compulsions and repetitive behaviour, self-injury (especially skin-picking), and aggressive behaviours. The most common psychiatric features in people with PWS are psychosis, affective disorders, and obsessive–compulsive disorders. Assessment should involve consideration of how behavioural and psychiatric problems can be influenced by neurocognitive functioning and medical issues. The heterogeneous and progressive nature of many behavioural and psychiatric problems in this population presents significant challenges to psychological assessment and management. Comprehensive assessment requires an understanding of the phenotype of Prader–Willi syndrome, and associated issues that can impact upon clinical presentation.
Skin-Picking in individuals with Prader-Willi syndrome: prevalence, functional assessment, and its comorbidity with compulsive and self-injurious behaviours
- Authors:
- DIDDEN Robert, KORZILIUS Hubert, CURFS Leopold M G
- Journal article citation:
- Journal of Applied Research in Intellectual Disabilities, 20(5), September 2007, pp.409-419.
- Publisher:
- Wiley
Individuals with Prader-Willi syndrome (PWS) are at increased risk for mental health and behaviour problems, such as skin-picking and compulsive behaviours. Prevalence and functional assessment of skin-picking, and its association with compulsive behaviour and self-injury, were investigated in a large group of individuals with PWS (n = 119) from the Netherlands. Data on demographic characteristics, skin-picking and compulsive and self-injurious behaviours were collected by questionnaires. Behavioural function of skin-picking was assessed by administering the Questions About Behavioral Function scale. Skin-picking was found in 86% of the sample, and correlated positively with compulsive behaviours. No associations were found between skin-picking and other variables. Functional assessment suggest that in most cases (i.e. 70%) skin-picking primarily had non-social functions. Skin-picking and compulsive behaviours are common in PWS. In most cases, skin-picking may be maintained by contingent arousal reduction. Controlled studies on behavioural treatment are lacking. Implications for treatment selection are discussed.
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.
Enabling carers to access specialist training in breakaway techniques: a case study
- Authors:
- GREEN T., WRAY J.
- Journal article citation:
- Journal of Learning Disabilities for Nursing Health and Social Care, 3(1), March 1999, pp.34-38.
This article explores the experience of one family, whose child had Prader-Willi Syndrome and severe behavioural difficulties, and the process undertaken to access specialist training in 'Breakaway' training. Prader-Willi syndrome has been associated with a range of behavioural difficulties including hyperphagia and food preoccupation, temper tantrums, stubbornness, lability, impulsivity, argumentativeness, depression, anxiety and repetitive behaviours. To ensure safety of both the parents and the child, access to training in 'Breakaway techniques' for the carers was procured, at the carers request. This paper concentrates upon the difficulties encountered during this process, both for the family and the professionals involved.