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

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

Rojahn J; Barnard-Brak L; Richman D; Dotson W; Medeiros K; Wei T L; Abby L

Journal of Developmental and Physical Disabilities

2013

reliability; Rehabilitation; Intellectual disabilities; behavior; validity; prevalence; phenomenology; delange-syndrome; intellectual disabilities; Aggressive/destructive behavior; behaviors; Cornelia de Lange Syndrome; repetitive; scale; Self-injurious; self-injurious-behavior; short form; Stereotyped behavior; behavioral problems; De Lange syndrome; trajectory; characteristics

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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