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Intellectual disabilities: genetics, behaviour and inclusion
- Author:
- RONDAL J. A.
- Publisher:
- Whurr
- Publication year:
- 2004
- Pagination:
- 203p.
- Place of publication:
- London
Advances in biobehavioural sciences are bringing important changes in the field of intellectual disabilities. Of particular interest is the description of particular behavioural phenotypes related to but distinct from genotypes, and the extent to which they are specific. In this text, more than 20 particular genetic syndromes with marked levels of intellectual disability are identified and described from a variety of points of view, including cognitive, language, behavioural, adaptive and social and community inclusion aspects.
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.
Self-injurious behaviour in Cornelia de Lange syndrome: 2. association with environmental events
- Authors:
- SLONEEM J., et al
- Journal article citation:
- Journal of Intellectual Disability Research, 53(7), July 2009, pp.590-603.
- Publisher:
- Wiley
Self-injurious behaviour is commonly seen in Cornelia de Lange syndrome (CdLS). However, there has been limited research into the aetiology of self-injury in CdLS and whether environmental factors influence the behaviour. The authors observed the self-injury of 27 individuals with CdLS and 17 participants who did not have CdLS matched for age, gender, level of intellectual disability and mobility. Descriptive analyses were used to determine the extent to which environmental events were associated with self-injury. Lag sequential analysis of the association between self-injurious behaviour and environmental events revealed no differences between the two groups in terms of either the number or degree of environmental associations. The results suggest that the associations between the environment and self-injury in CdLS do not differ from those seen in the broader population of people with intellectual disability. By implication the social reinforcement hypothesis is equally applicable to both groups.
Specific disorders and your child's behaviour: a parent's guide to behavioural phenotypes
- Authors:
- BARNETT Diane, TURK Jeremy
- Publisher:
- Contact a Family
- Publication year:
- 2003
- Pagination:
- 8p.
- Place of publication:
- London
'Behavioural phenotype' is a medical term which describes behaviour associated with specific disorders, particularly those which lead to learning disabilities. These disorders may result from a genetic or other biological cause e.g. an infection early in life. These behaviours may include: common challenging behaviours: emotional and temperamental tendencies; learning disability; and increased likelihood of psychiatric and psychological difficulties. Knowing the cause of the child's condition and the reason for his or her behaviour can be helpful. Sometimes, a pattern of behaviour may be suggestive of the presence of an underlying genetic cause. Not all behavioural difficulties originate in a genetic disorder. They may have other causes such as family stresses and relationships, conduct disorders and oppositional defiant disorder.
Self-injurious behaviour as part of genetic syndromes
- Author:
- DEB Shoumitro
- Journal article citation:
- British Journal of Psychiatry, 172, May 1998, pp.385-388.
- Publisher:
- Cambridge University Press
Reviews the association between genetic syndromes and self-injurious behaviour. Collates and presents the information available on the subject of self-injurious behaviours and genetic syndromes with a critical appraisal. Finds that self-injurious behaviours are associated with some genetic syndromes. However, the causal relationship between the genetic syndromes and the self-injurious behaviour remains far from clear.
Treating neurodevelopmental disabilities: clinical research and practice
- Author:
- FARMER Janet
- Publisher:
- Guilford Press
- Publication year:
- 2006
- Pagination:
- 336p.
- Place of publication:
- London
This volume describes how to intervene effectively to promote the optimal health and functioning of children with neurodevelopmental disabilities. Leading scientist-practitioners discuss the psychosocial aspects of a range of acquired and congenital disorders and how they can be targeted in assessment and treatment. Presented are innovative approaches to managing cognitive, emotional, and behavioural challenges; enhancing participation in social activities and school; and facilitating family problem solving. Particular attention is given to ways that psychologists, health professionals, and educators can collaborate to meet children's changing rehabilitation needs over the course of development.