Social functioning in Smith Magenis syndrome
Funded by Cerebra from 2009 – 2011
Smith Magenis syndrome (SMS) is a rare genetic disorder that affects about 1 in 25,000 individuals. The syndrome is associated with a behavioural phenotype that includes a range of challenging behaviours, including self injury, aggression, impulsivity, attention seeking and sleep problems. Social motivation is a factor that has been linked to a number of the difficulties reported in the syndrome. For example, challenging behaviour has been found to increase in conditions of low social attention and a particularly strong preference for certain people has been reported.
My research aims to investigate social motivation in SMS by examining various aspects of social behaviour and potentially related factors such as impulsivity and challenging behaviour. These issues might provide information to improve interventions to reduce difficult behaviour and increase our understanding of the behavioural phenotype; providing greater information to new parents, professionals and support groups.
In my research, social behaviour of children with SMS is contrasted to that of a matched sample of children with Down syndrome (DS), in both observational and experimental studies. I also examine some of the other difficult behaviours seen such as impulsivity, sleep disturbance and self injury/aggression using questionnaire methods, cognitive assessments and behavioral observations. Children over 2 years and under 16 years old with diagnoses of SMS or DS were approached to take part in this study.
Progress to date
The study is now nearing completion. Recruitment was completed in mid 2010 and now over 40 families from across the UK have been visited to collect data. The results of these visits are now being analysed and written up for academic papers and feedback reports for caregivers. Early results from the study have been fed back at the SMS Foundation conference 2011 and at a number of national and international conferences.
Tel: 0121 414 6245