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01.12.2014 | Research | Ausgabe 1/2014 Open Access

Journal of Neurodevelopmental Disorders 1/2014

Self-injury and aggression in tuberous sclerosis complex: cross syndrome comparison and associated risk markers

Zeitschrift:
Journal of Neurodevelopmental Disorders > Ausgabe 1/2014
Autoren:
Kate E Eden, Petrus J de Vries, Jo Moss, Caroline Richards, Chris Oliver
Wichtige Hinweise

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

KEE contributed to the design of the study, collected and analyzed the data and drafted the manuscript. CO, PJdV and JM contributed to the design of the study and were involved in revising the manuscript. JM and CR were involved in the data collection for the comparison groups. All authors read and approved the final version of the manuscript.

Abstract

Background

Research reporting prevalence rates of self-injurious and aggressive behaviour in people with tuberous sclerosis complex (TSC) is limited. No studies have compared rates of these behaviours in TSC with those in other syndrome groups matched for degree of disability or investigated risk markers for these behaviours in TSC.

Methods

Data from the Challenging Behaviour Questionnaire were collected for 37 children, aged 4 to 15 years, with TSC. Odds ratios were used to compare rates of self-injury and aggression in children with TSC with children with idiopathic autism spectrum disorder (ASD), fragile X, Cornelia de Lange and Down syndromes. Characteristics were measured using the Mood Interest and Pleasure Questionnaire, the Activity Questionnaire, the Social Communication Questionnaire, the Repetitive Behaviour Questionnaire, the Wessex Behaviour Schedule and the revised Non-communicating Children Pain Checklist. Mann-Whitney U analyses were used to compare characteristics between individuals with self-injury and aggression and those not showing these behaviours.

Results

Rates of self-injury and aggression in TSC were 27% and 50%, respectively. These are high but not significantly different from rates in children with Down syndrome or other syndrome groups. Both self-injury and aggression were associated with stereotyped and pain-related behaviours, low mood, hyperactivity, impulsivity and repetitive use of language. Children who engaged in self-injury also had lower levels of interest and pleasure and showed a greater degree of ‘insistence on sameness’ than children who did not self-injure. Aggression was associated with repetitive behaviour. The majority of these associations remained significant when the association with level of adaptive functioning was controlled for.

Conclusions

Behavioural profiles can be used to identify those most at risk of developing self-injury and aggression. Further research is warranted to understand the influence of such internal factors as mood, ASD symptomatology and pain on challenging behaviour in people with intellectual disability.
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