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

Johannes Rojahn, Lucy Barnard-Brak, David Richman, Wesley Dotson, Kristen Medeiros, Tianlan Wei, Layla Abby

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

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.

Original languageEnglish
Pages (from-to)505-515
Number of pages11
JournalJournal of Developmental and Physical Disabilities
Volume25
Issue number5
DOIs
StatePublished - Oct 2013

Keywords

  • Aggressive/destructive behavior
  • Cornelia de Lange Syndrome
  • Intellectual disabilities
  • Self-injurious behavior
  • Stereotyped behavior

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