Self-injurious behaviour in Cornelia de Lange syndrome: 1. Prevalence and phenomenology

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Self-injurious behaviour in Cornelia de Lange syndrome: 1. Prevalence and phenomenology. / Oliver, Christopher; Sloneem, Jennifer; Hall, S; Arron, Kate.

In: Journal of Intellectual Disability Research, Vol. 53, No. 7, 01.07.2009, p. 575-89.

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@article{d057048fb0004cdfa1c1c1990bd6416f,
title = "Self-injurious behaviour in Cornelia de Lange syndrome: 1. Prevalence and phenomenology",
abstract = "BACKGROUND: Self-injurious behaviour is frequently identified as part of the behavioural phenotype of Cornelia de Lange syndrome (CdLS). We conducted a case-control study of the prevalence and phenomenology of self-injurious behaviour (SIB) in CdLS. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.",
keywords = "behavioural phenotype, Cornelia de Lange syndrome, stereotyped behaviour, self-injurious behaviour, repetitive behaviour, compulsive behaviour",
author = "Christopher Oliver and Jennifer Sloneem and S Hall and Kate Arron",
year = "2009",
month = "7",
day = "1",
doi = "10.1111/j.1365-2788.2009.01179.x",
language = "English",
volume = "53",
pages = "575--89",
journal = "Journal of Intellectual Disability Research",
issn = "0964-2633",
publisher = "Wiley",
number = "7",

}

RIS

TY - JOUR

T1 - Self-injurious behaviour in Cornelia de Lange syndrome: 1. Prevalence and phenomenology

AU - Oliver, Christopher

AU - Sloneem, Jennifer

AU - Hall, S

AU - Arron, Kate

PY - 2009/7/1

Y1 - 2009/7/1

N2 - BACKGROUND: Self-injurious behaviour is frequently identified as part of the behavioural phenotype of Cornelia de Lange syndrome (CdLS). We conducted a case-control study of the prevalence and phenomenology of self-injurious behaviour (SIB) in CdLS. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

AB - BACKGROUND: Self-injurious behaviour is frequently identified as part of the behavioural phenotype of Cornelia de Lange syndrome (CdLS). We conducted a case-control study of the prevalence and phenomenology of self-injurious behaviour (SIB) in CdLS. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

KW - behavioural phenotype

KW - Cornelia de Lange syndrome

KW - stereotyped behaviour

KW - self-injurious behaviour

KW - repetitive behaviour

KW - compulsive behaviour

U2 - 10.1111/j.1365-2788.2009.01179.x

DO - 10.1111/j.1365-2788.2009.01179.x

M3 - Article

VL - 53

SP - 575

EP - 589

JO - Journal of Intellectual Disability Research

JF - Journal of Intellectual Disability Research

SN - 0964-2633

IS - 7

ER -