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

Authors: Rojahn, Johannes1; Barnard-Brak, Lucy2; Richman, David2; Dotson, Wesley2; Medeiros, Kristen3; Wei, Tianlan2; Abby, Layla2

Source: Journal of Developmental and Physical Disabilities, Volume 25, Number 5, October 2013 , pp. 505-515(11)

Publisher: Springer

Buy & download fulltext article:


Price: $47.00 plus tax (Refund Policy)


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.

Keywords: Aggressive/destructive behavior; Cornelia de Lange Syndrome; Intellectual disabilities; Self-injurious behavior; Stereotyped behavior

Document Type: Research Article


Affiliations: 1: George Mason University, Fairfax, USA, Email: 2: Texas Tech University, Lubbock, USA 3: George Mason University, Fairfax, USA

Publication date: October 1, 2013

Related content


Free Content
Free content
New Content
New content
Open Access Content
Open access content
Subscribed Content
Subscribed content
Free Trial Content
Free trial content

Text size:

A | A | A | A
Share this item with others: These icons link to social bookmarking sites where readers can share and discover new web pages. print icon Print this page