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Self-injurious behaviour in Cornelia de Lange syndrome: 1. Prevalence and phenomenology
- Authors:
- OLIVER C., et al
- Journal article citation:
- Journal of Intellectual Disability Research, 53(7), July 2009, pp.575-589.
- Publisher:
- Wiley
Self-injurious behaviour is frequently identified as part of the behavioural phenotype of Cornelia de Lange syndrome (CdLS). The authors conducted a case-control study of the prevalence and phenomenology of self-injurious behaviour (SIB) in CdLS. A total of 54 participants with CdLS were compared with 46 individuals who were comparable on key variables including age, degree of intellectual disability and wheelchair use, using questionnaire and observational measures. Clinically significant self-injury was not more prevalent in the CdLS group (55.6%), nor was it different in presentation from that seen in the comparison group. Hyperactivity, stereotyped and compulsive behaviours predicted clinically significant self-injury in all participants. Hand directed, mild self-injury was more prevalent in CdLS. The results show that clinically significant self-injury may not be part of the behavioural phenotype of CdLS but a specific body target for proto-SIB is more common.
The behavioural phenotype of Smith–Magenis syndrome: evidence for a gene–environment interaction
- Authors:
- TAYLOR L., OLIVER C.
- Journal article citation:
- Journal of Intellectual Disability Research, 52(10), October 2008, pp.830-841.
- Publisher:
- Wiley
Behaviour problems and a preference for adult contact are reported to be prominent in the phenotype of Smith–Magenis syndrome. This UK study examined the relationship between social interactions and self-injurious and aggressive/disruptive behaviour in Smith–Magenis syndrome to explore potential operant reinforcement of problem behaviours and thus a gene–environment interaction. Observational data were collected on five children with Smith–Magenis syndrome (age range 3 to 13 years) . The associations between purported phenotypic behaviours and two environmental events (adult attention and demands) were examined using descriptive analysis. All participants engaged in self-injurious behaviour and aggressive/disruptive outbursts. Sequential analyses of aggressive/disruptive outbursts and self-injury revealed that these behaviours were evoked by low levels of adult attention and led to increased levels of attention following the behaviours in three and two participants respectively out of the four for whom this analysis was possible. Problem behaviour in Smith–Magenis syndrome was evoked by decreased social contact in three out of four children. These data, considered alongside the preference for adult contact and the significantly increased prevalence of these behaviours in Smith–Magenis syndrome, illustrate a potential gene–environment interaction for problem behaviour in this syndrome.
The relationship between components of the behavioural phenotype in Prader-Willi syndrome
- Authors:
- OLIVER C., WOODCOCK K.A., HUMPHREYS G.W.
- Journal article citation:
- Journal of Applied Research in Intellectual Disabilities, 22(4), July 2009, pp.403-407.
- Publisher:
- Wiley
Repetitive questions and temper outbursts form part of the behavioural phenotype of Prader-Willi syndrome (PWS). This study investigated the phenomenology of temper outbursts in PWS and their relationship with other PWS behavioural characteristics. Four individuals with PWS were observed (5–10 h), during a number of experimental and natural environment challenges, some of which were expected to trigger temper outbursts. Individual behaviours including crying, ignoring, arguing, questioning, stereotypy, frowning and posture changes were recorded and subjected to lag sequential analysis. All participants were significantly more likely to show repetitive questioning before more challenging behaviours such as crying, arguing or ignoring requests. Precursor behaviours such as frowning and stereotypical behaviour were identified in three participants. The authors conclude that temper outbursts in PWS may be associated with other PWS behavioural phenotypic characteristics such as repetitive questions and 'stubbornness'. A progression of behaviours may lead up to the most challenging temper outburst behaviours. This may have important implications for effective coping strategies.