The prevalence and phenomenology of self-injurious and aggressive behaviour in genetic syndromes.
Self-injury and aggression risks are not equal across genetic syndromes—screen early and plan for tough automatic functions.
01Research in Context
What this study did
Leung et al. (2011) ran a large survey. They asked caregivers about self-injury and aggression in people with seven genetic syndromes. CdCS, CdLS, FXS, PWS, LS, SMS, and AS were included. The team counted how often each behavior showed up in each syndrome.
What they found
Some syndromes carry much higher risk than others. CdCS, CdLS, FXS, PWS, LS, and SMS all showed elevated self-injury. SMS and AS also showed elevated aggression. The numbers tell you where to focus your early-screening efforts.
How this fits with other research
Fovel et al. (1989) did an earlier survey inside one institution. They found self-injury plus self-restraint together. Leung et al. (2011) widened the lens to many syndromes and confirmed that self-injury is common, but they did not track self-restraint. The two studies line up: self-injury is frequent; self-restraint may ride along.
Pitchford et al. (2019) reviewed treatment studies from 2001-2016. They warn that automatically maintained self-injury is now more common and slightly harder to treat. Leung et al. (2011) give you the prevalence map that explains why: more cases arrive already at high severity, so plan for tougher functions.
Davies et al. (2014) caution that we still lack proof that challenging behaviors equal depression in ID. Leung et al. (2011) add detail: the behaviors vary by syndrome, so a single depression proxy is even less likely to fit all groups.
Why it matters
Use the syndrome list as a quick-risk screener. If a new client has CdLS or SMS, start assessment for self-injury on day one. Pair this with Pitchford et al. (2019): expect automatic function and prepare more powerful reinforcement. Do not assume depression as the cause; Davies et al. (2014) show the evidence is too thin. Instead, run a full FA and use the syndrome profile to set realistic severity benchmarks.
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02At a glance
03Original abstract
BACKGROUND: Self-injurious and aggressive behaviours are reported as components of some behavioural phenotypes but there are few studies comparing across syndrome groups. In this study we examined the prevalence of these behaviours and the associated person characteristics in seven genetic syndromes. METHODS: Questionnaire data on self-injury and aggression, mood, hyperactivity, autism spectrum disorder and repetitive behaviour were collected on Angelman (AS, n=104), Cornelia de Lange (CdLS, 101), Cri du Chat (CdCS, 58), Fragile X (FXS, 191), Lowe (LS, 56), Prader-Willi (PWS, 189) and Smith-Magenis (SMS, 42) syndromes. RESULTS: A significantly higher prevalence of self-injury was evident in CdCS, CdLS, FXS, PWS, LS and SMS. The prevalence of aggression was significantly heightened in AS and SMS. Self-injury was associated with repetitive and impulsive behaviour in CdLS, FXS, PWS and LS. Impulsivity and overactivity were significantly higher in those showing aggression across all syndrome groups. CONCLUSIONS: These data quantify the risk for self-injury and aggression in the syndromes studied with implications for early intervention. The associations between these behaviours and person characteristics both within and between syndromes warrant further research.
Journal of intellectual disability research : JIDR, 2011 · doi:10.1111/j.1365-2788.2010.01337.x