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

Abstract

BackgroundResearch 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.MethodsData 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.ResultsRates 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.ConclusionsBehavioural 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.

A clinical update on tuberous sclerosis complex‐associated neuropsychiatric disorders (TAND)

“…Aggressive outbursts and temper tantrums in children and adolescents with TSC have been reported to be much more stable across ability levels (de Vries, Hunt, & Bolton, 2007), although aggressive outbursts also seems to reduce into adulthood. Aggression in adults with TSC who have ID is reported at lower rates than children and adolescents without ID and mixed ID/no ID samples (de Vries, Hunt, & Bolton, 2007; Eden, de Vries, Moss, Richards, & Oliver, 2014). This suggests that childhood might be a high-risk time for aggressive behaviour for all children with TSC, but, like self-injury, this may ameliorate with age.…”

“…Evidence suggests that in children and adolescents, overactivity and impulsivity correlates strongly with the presence or absence of ID (de Vries, Hunt, & Bolton, 2007), and there is some indication that overactivity may decrease with age (see Table 2). More nuanced studies of overactivity and impulsivity are warranted to identify priorities for intervention for these problematic behaviours in TSC, particularly given robust associations between impulsivity and self-injury and aggression in TSC (Eden et al, 2014; Wilde et al, 2017; Wilde et al, 2018), suggesting that impulsivity may be a risk marker for these adverse behavioural outcomes.…”

Tuberous Sclerosis Associated Neuropsychiatric Disorders (TAND) and the TAND Checklist

“…High frequency behavioral presentations in TSC include anxiety, depressed mood, aggressive behaviors, temper tantrums, attention-related behaviors (such as difficulty concentrating, hyperactivity, impulsivity), social, and communication-related behaviors (such as speech and language delays, poor eye contact, difficulties in relationships with peers, repetitive behaviors), self-injurious behaviors, and eating or sleep difficulties. 11,20–22 …”

Persistence of self‐injury, aggression and property destruction in children and adults with tuberous sclerosis complex

“…Eden et al . () found rates of self‐injury of 27% in a study of children and adolescents with TSC (with and without ID). Using the same measures with an adult sample (all of whom had ID), rates of self‐injury were 31% (Wilde et al .…”

“…Two recent studies have examined whether these potential risk markers were associated with self‐injury and other challenging behaviours in children/adolescents and adults with TSC (Eden et al . ; Wilde et al . ).…”