A description of adaptive and maladaptive behaviour in children and adolescents with Cri-du-chat syndrome
adolescent; development; Rehabilitation; epidemiology; Neurology; child; Education & Educational Research; prevalence; Genetics & Heredity; individuals; Neurosciences &; Psychiatry; adults; behaviour; Cri-du-chat syndrome; behavioral problems; Cri-du-chat; trajectory; characteristics; maladaptive behaviors
Background Psychological tests can be useful to record adaptive and maladaptive behaviours of children with intellectual disability. The objective of this study was to describe the adaptive and maladaptive behaviour of children and adolescents with Cri-du-chat syndrome. Methods The sample consisted of 10 children and adolescents with Cri-du-chat syndrome (mean chronological age = 11.3 years, mean mental age = 18 months). The developmental quotient was calculated through the Psychoeducational Profile - Revised. An observational protocol was used to record adaptive and maladaptive behaviours. Results The number of maladaptive behaviours observed was different among participants. However, all of them had high rates of adaptive behaviours, such as rule-following. Conclusions These results, though preliminary, justify that we continue to think about the need for psychoeducational interventions aimed at stimulating the repertoire of adaptive behaviours, in people with Cri-du-chat syndrome.
Teixeira Mctv; Emerich D R; Orsati F T; Rimerio R C; Gatto K R; Chappaz I O; Kim C A
Journal of Intellectual Disability Research
2011
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1365-2788.2010.01377.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2010.01377.x</a>
Self-injurious behaviour in Cornelia de Lange syndrome: 2. association with environmental events
intervention; symptoms; children; Rehabilitation; Neurology; Education & Educational Research; prevalence; Genetics & Heredity; behavioural phenotype; brachmann-delange syndrome; Cornelia de Lange syndrome; descriptive analysis; functional-analysis; individuals; Neurosciences &; phenomenology; phenotype; prader-willi syndrome; Psychiatry; self-injurious behaviour; social reinforcement; behavioral problems; De Lange syndrome; psychological intervention; environmental conditions; demand denial no contact; environment; self-injury
Background 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. Methods We 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. Results 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. Conclusions 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.
Sloneem J; Arron K; Hall S; Oliver C
Journal of Intellectual Disability Research
2009
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1365-2788.2009.01183.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2009.01183.x</a>
The Behavior Problems Inventory-Short Form for individuals with intellectual disabilities: Part II: reliability and validity
children; reliability; Rehabilitation; validity; Neurology; Education & Educational Research; Genetics & Heredity; Neurosciences &; Psychiatry; autism spectrum disorders; developmental-disabilities; checklist; disabilities; validation; Behavior Problems Inventory; diagnostic-assessment; factor structure; fit indexes; handicapped dash; intellectual; profound mental-retardation; rating form; severely; behavioral problems; unspecified ID; tool development; scale development
Background The Behavior Problems Inventory-01 (BPI-01) is an informant-based behaviour rating instrument for intellectual disabilities (ID) with 49 items and three sub-scales: Self-injurious Behavior, Stereotyped Behavior and Aggressive/Destructive Behavior. The Behavior Problems Inventory-Short Form (BPI-S) is a BPI-01 spin-off with 30 items. Methods The psychometric properties of these two versions of the scale were computed using aggregated archival data from nine different sites in the USA, Wales, England, the Netherlands and Romania with a total of 1122 cases with a BPI-01 total score >0. Results The internal consistency of the BPI-01 and the BPI-S ranged from fair to excellent with the BPI-01 showing slightly stronger reliability. Construct validity (confirmatory and discriminant) was computed by comparing BPI sub-scale scores with the scores of four other behaviour rating scales (the Aberrant Behavior Checklist, the Diagnostic Assessment for the Severely Handicapped-II, the Nisonger Child Behavior Rating Form and the Inventory for Client and Agency Planning). Strong evidence for confirmatory and discriminant validity was found for both the BPI-01 and the BPI-S. Confirmatory fit indices for the BPI and the BPI-S were comparable and suggesting that the factor structures fit the data well. Conclusion In summary, both BPI versions were found to be equally sound psychometrically and can be endorsed for future use. However, independent future studies are needed to replicate the psychometrics of the BPI-S with new data.
Rojahn J; Rowe E W; Sharber A C; Hastings R; Matson J L; Didden R; Kroes D B H; Dumont E L M
Journal of Intellectual Disability Research
2012
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1365-2788.2011.01506.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2011.01506.x</a>
Self-injurious behaviour in Cornelia de Lange syndrome: 1. Prevalence and phenomenology
Rehabilitation; Neurology; Education & Educational Research; Genetics & Heredity; behavioural phenotype; brachmann-delange syndrome; Cornelia de Lange syndrome; individuals; Neurosciences &; phenotype; Psychiatry; self-injurious behaviour; mental-retardation; mutilative behavior; compulsive behaviour; intellectual disabilities; maladaptive behavior; people; prader-willi-syndrome; rating-scale; repetitive behaviour; stereotyped behaviour; behavioral problems; De Lange syndrome; trajectory; characteristics; hyperactivity; stereotyped disorders; compulsive behaviors
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.
Oliver C; Sloneem J; Hall S; Arron K
Journal of Intellectual Disability Research
2009
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1365-2788.2009.01179.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2009.01179.x</a>
Sleep problems and daytime problem behaviours in children with intellectual disability
children; Rehabilitation; adolescents; Neurology; disorders; Education & Educational Research; Genetics & Heredity; Neurosciences &; Psychiatry; handicapped-children; disturbance; challenging behavior; adults; community-based sample; daytime problem behaviour; difficulties; severe learning-disabilities; sleep problem; behavior; breathing difficulties; sleep disturbance; urinary incontinence; Rett syndrome; tuberous sclerosis; trajectory; characteristics; intellectual disability
BACKGROUND: Sleep problems are common among children with intellectual disability (ID). METHOD: The present study assessed the prevalence of severe sleep problems in a sample of children (n=286) with mild to profound ID who lived at home with their parents(s) in the Netherlands. It also explored relationships between severe sleep problems, and family and child variables. Demographic information, data on children's sleep behaviours and parent variables were collected using questionnaires. RESULTS: Severe settling problems, night waking and early waking were present in 4.2%, 10.8% and 4.2% of cases, respectively; 16.1% of children had at least one type of sleep problem. Children with a severe sleep problem had more severe levels of ID, used medication more often, had a greater frequency of epilepsy, were younger, had a greater frequency of cerebral palsy, and showed more daytime drowsiness and daytime napping than children without a severe sleep problem. Furthermore, children with a severe sleep problem showed more severe levels of daytime problem behaviours; for example, aggression, non-compliance and hyperactivity. CONCLUSION: The results of the present study are discussed with regard to the assessment and treatment of sleep problems in children with ID.
Didden R; Korzilius H; van Aperlo B; van Overloop C; de Vries M
Journal of Intellectual Disability Research
2002
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1046/j.1365-2788.2002.00404.x" target="_blank" rel="noreferrer noopener">10.1046/j.1365-2788.2002.00404.x</a>
The behavioural phenotype of Cornelia de Lange Syndrome: a study of 56 individuals
children; Syndrome; Rehabilitation; communication; Neurology; Education & Educational Research; autism; Genetics & Heredity; brachmann-delange syndrome; Neurosciences &; Psychiatry; intellectual disability; mental-retardation; intelligence; behavioural phenotypes; cognitive level; Cornelia de Lange; language-skills; nipbl; normal; self-injury; self-mutilative behavior; behavioral problems; De Lange syndrome; trajectory; characteristics; CdLS; hyperactivity; attention disorder; anxiety; compulsive disorders; self-injurious behavior; autistic-like features
Background Few studies have investigated functional and behavioural variables of Cornelia de Lange Syndrome (CdLS) in a large sample of individuals. The aim of this study is to provide greater insight into the clinical, behavioural and cognitive characteristics that are associated with CdLS. Methods In total, 56 individuals with CdLS participated in the study. During hospitalization, their mothers received a number of questionnaires to complete. The behavioural phenotype was investigated using the following scales: Developmental Behaviour Scale Primary Carer Version; Autism Behaviour Checklist; Childhood Autism Rating Scale. Results Our participants demonstrated some behavioural characteristics that are frequently associated with CdLS (hyperactivity, attention disorder, anxiety, compulsive disorders, self-injurious behaviour and autistic-like features). Our findings demonstrate the variability of behavioural characteristics in CdLS in addition to highlighting the contribution of some variables to both the CdLS behavioural profile and the developmental trajectory of the behavioural pattern. Conclusions The behavioural characteristics identified in our sample were correlated with some clinical and functional aspects (chronological age, cognitive level and clinical phenotype). The variability of the behavioural profile in CdLS reflected the wide variability in cognitive and adaptive functioning across individuals and led us to conclude that there may be multiple behavioural phenotypes associated with the syndrome. Further comparative studies between CdLS and individuals with intellectual disability or other genetic syndromes may help to provide further understanding of the behavioural phenotype of CdLS.
Basile E; Villa L; Selicorni A; Molteni M
Journal of Intellectual Disability Research
2007
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1365-2788.2007.00977.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2007.00977.x</a>