Smith Magensis Syndrome

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Taylor, L. and Oliver, C. (2008). The behavioural phenotype of Smith-Magenis syndrome: Evidence for a gene-environment interaction. Journal of Intellectual Disability Research, 52, 830-841. (DOI: 10.1111/j.1365-2788.2008.01066.x)

Smith-Magenis syndrome (SMS) is not only evident through physical characteristics such as, a prominent forehead, up-slanting eyes and short stature; but also by developmental delays and moderate to severe intellectual disabilities – one of which is the consistent demand for adult contact. When the need for social interaction is lacking, it generally results in problematic and disruptive attention seeking behaviours, which includes self-injury. This article intended to explore "the relationship between social interactions and self-injurious and aggressive/disruptive behaviours in Smith-Magenis syndrome by examining potential operant reinforcement of problem behaviours and thus a gene-environment interaction". The primary objective of this two-part study is to understand the duration of problem behaviours, whereas, the second objective is to focus on the association between problem behaviours and environmental events. Target Population/ Participant Number

The population for this study was comprised of 5 children - 2 females and 3 males. The inclusion criteria for this study were that participants have a clinical diagnosis of SMS; were between 3-18 years of age; and were attendees of a school within 150 miles from the location of the study. Candidates were selected through the UK Smith-Magentis Syndrome Foundation. Of the 5 participants, 4 attended specialized schools, and 1 attended a mainstream school; all students required one-to-one adult attention throughout the entire school day. Research Method

This mixed research employes study took place over a period of 2 days, between the hours of 9am and 3:15pm, while participants were observed in their typical day-to-day...
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