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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.