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Behavior Problems in Individuals with Cornelia de Lange Syndrome: Population-Specific Validation of the Behavior Problem Inventory-01

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Abstract

The purpose of this study was to validate the Behavior Problem Inventory-01 (BPI-01; Rojahn J Autism Dev Disord 31:577–588, 2001) in a population of 179 individuals with Cornelia de Lange Syndrome (CdLS) and to extend the literature on the behavioral phenotype of individuals with CdLS. We also set out to determine the relationship between the frequency of self-injurious behavior (SIB), stereotyped behavior, and aggressive/destructive behavior topographies with the level of intellectual disability (ID). The BPI-01 among the individuals with CdLS showed a sufficient factor structure and internal consistency of each of the three assessed construct (SIB, stereotyped behavior, and aggressive/destructive behaviors). Similar to the findings in other populations with intellectual disabilities, those with severe/profound intellectual impairment exhibited significantly more frequent SIB and stereotypic behavior than those with moderate or mild intellectual impairment or average intellectual abilities while no statistically a differences were noted for aggressive behavior across levels of intellectual impairment. There is a need for uniformity of assessment tools across different study to improve our ability to compare results across different prevalence studies.

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Notes

  1. Each of the three subscales contains an open item so that raters can add a clinically relevant topography for a particular individual that was not represented in the BPI-01. Buy that count the BPI-01 consists of 51 items.

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Acknowledgements

We thank the Cornelia de Lange Syndrome Foundation USA for their assistance with participant recruitment and the caregivers that provided data for the study.

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Authors and Affiliations

  1. George Mason University, Fairfax, USA

    Johannes Rojahn & Kristen Medeiros

  2. Texas Tech University, Lubbock, USA

    Lucy Barnard-Brak, David Richman, Wesley Dotson, Tianlan Wei & Layla Abby

  3. Department of Psychology, George Mason University, 10340 Democracy Lane, Suite 202, Fairfax, VA, 22030, USA

    Johannes Rojahn

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  1. Johannes Rojahn
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  2. Lucy Barnard-Brak
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  7. Layla Abby
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Correspondence to Johannes Rojahn.

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Rojahn, J., Barnard-Brak, L., Richman, D. et al. Behavior Problems in Individuals with Cornelia de Lange Syndrome: Population-Specific Validation of the Behavior Problem Inventory-01. J Dev Phys Disabil 25, 505–515 (2013). https://doi.org/10.1007/s10882-012-9329-6

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