Prader-willi Syndrome

Response: PWS
By: Alley Dezenhouse, BA ABS

Prader-willi syndrome (PWS) is often associated with certain characteristic cognitive profiles including a preference for sameness, consistency and routine (Woodcock, Oliver, Humpreys, 2011); perhaps this is motivated by inadequate adaptive behaviours. PWS is the result of insufficient chromosomal information (Woodcock et al., 2012); individuals with PWS often present with abnormal facial features, and a physical small appearance. Individuals with PWS typically demonstrate a mild cognitive delay, and a strength based treatment approach is recommended to manage quality of life (Woodcock et al., 2012). Individuals are impacted by their environment; according to Woodcock et al, (2012) frequent changes in routine and/or discrepancies between expectation and fulfilment result in increased behavioural issues like temper tantrums, repetitive behaviour and stereotypy  (Woodcock et al., 2012). A difficulty in the ability to shift attention, similar to what is often associated with ADHD, is often the cause of inflexibility and concerns pertaining to routine.

Whittington and Holland (2011) sought to determine what social delays exist in individuals with PWS; the Vineland Adaptive Scale of Behaviour can be used to highlight specific peer and socially based deficits (Whittington & vHolland, 2011). Understanding which social impairments impact individuals with PWS, will allow practitioners to produce individualized programs that make meaningful and socially significant change.

References

Whittington, J. J., &  Holland, T. T. (2011). Recognition of emotion in facial expression by

people with Prader-Willi syndrome. Journal of Intellectual Disability Research, pp.  75-84. doi:10.1111/j.1365-2788.2010.01348.x

Woodcock, K. A., Oliver, C. C., & Humphreys, G. W. (2011). The relationship between specific

cognitive impairment and behaviour in Prader-Willi syndrome. Journal of Intellectual Disability Research, pp. 152-171. doi:10.1111/j.1365-2788.2010.01368.x