Behavioural phenotype of Cornelia de Lange syndrome: case-control study

Title

Behavioural phenotype of Cornelia de Lange syndrome: case-control study

Creator

Oliver C; Arron K; Sloneem J; Hall S

Publisher

The British Journal of Psychiatry

Date

2008

Subject

Male; Case-Control Studies; Young Adult; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Female; Child Preschool; Phenotype; Autistic Disorder/psychology; Communication Disorders/psychology; Compulsive Behavior/psychology; De Lange Syndrome/psychology; behavioral problems; De Lange syndrome; trajectory; characteristics; global behavior disorder

Description

BACKGROUND: Cornelia de Lange syndrome is associated with abnormalities on chromosomes 5, 10 and X. AIMS: To delineate the behavioural phenotype of Cornelia de Lange syndrome with specific reference to autistic-spectrum disorder. METHOD: A total of 54 individuals with Cornelia de Lange syndrome (mean age 13.88 years; s.d.=8.58) and 46 comparable individuals with intellectual disability (mean age 13.74 years; s.d.=7.99) were assessed on measures of autistic-spectrum disorder, and adaptive, compulsive and disordered behaviour. RESULTS: There was no difference between the groups in global behaviour disorder. Severe autism was significantly more prevalent in the syndrome group (32.1%) than the comparison group (7.1%). In addition, the syndrome group also evidenced significantly higher levels of compulsive behaviour. CONCLUSIONS: These data suggest that autistic-spectrum disorder is part of the behavioural phenotype of Cornelia de Lange syndrome and that compulsive behaviours are evident. Future research should investigate this behavioural phenotype using contemporary diagnostic algorithms for autism with detailed examination of the phenomenology of compulsive behaviours.

Rights

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).

Citation

Oliver C; Arron K; Sloneem J; Hall S, “Behavioural phenotype of Cornelia de Lange syndrome: case-control study,” Pediatric Palliative Care Library, accessed October 22, 2021, https://pedpalascnetlibrary.omeka.net/items/show/16775.

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