The prevalence and phenomenology of self-injurious and aggressive behaviour in genetic syndromes.
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.
Arron
K
K
School of Psychology, University of Birmingham, Edgbaston, Birmingham, UK.
Oliver
C
C
Moss
J
J
Berg
K
K
Burbidge
C
C
D000015:Abnormalities, Multiple / Q000453:epidemiology / Q000235:genetics / Q000523:psychology; D000293:Adolescent; D000328:Adult; D000374:Aggression; D017204:Angelman Syndrome / Q000453:epidemiology / Q000523:psychology; D001526:Behavioral Symptoms / Q000453:epidemiology* / Q000235:genetics / Q000523:psychology; D016022:Case-Control Studies; D002648:Child; D002659:Child Development Disorders, Pervasive / Q000453:epidemiology* / Q000523:psychology; D002675:Child, Preschool; D025063:Chromosome Disorders / Q000453:epidemiology / Q000523:psychology; D015897:Comorbidity; D003410:Cri-du-Chat Syndrome / Q000453:epidemiology / Q000523:psychology; D003635:De Lange Syndrome / Q000453:epidemiology / Q000523:psychology; D005260:Female; D005600:Fragile X Syndrome / Q000453:epidemiology / Q000523:psychology; D030342:Genetic Diseases, Inborn / Q000453:epidemiology* / Q000523:psychology; D006801:Humans; D008607:Intellectual Disability / Q000523:psychology; D008297:Male; D008875:Middle Aged; D009800:Oculocerebrorenal Syndrome / Q000453:epidemiology / Q000523:psychology; D011218:Prader-Willi Syndrome / Q000453:epidemiology / Q000523:psychology; D015995:Prevalence; D016728:Self-Injurious Behavior / Q000453:epidemiology* / Q000523:psychology; D058496:Smith-Magenis Syndrome / Q000453:epidemiology / Q000523:psychology; D013577:Syndrome; D055815:Young Adult