Maladaptive behaviors and risk factors among the genetic subtypes of Prader-Willi syndrome

Abstract

Maladaptive behaviors among 65 people with Prader-Willi syndrome were assessed using the Reiss Screen for maladaptive behaviors. Young adults in their twenties were more likely to display aggressive behavior than adolescents and older adults. Differences in maladaptive behaviors between the typical deletion and uniparental disomy (UPD) subtypes were evaluated. The typical deletion subtype had higher self-injury and stealing scores than the UPD subtype. Subject characteristics were differentially related to maladaptive behavior among the typical deletion and UPD subtypes. Differences in maladaptive behavior were also examined between the typical deletion type I and type II subtypes. The type I deletion subtype had greater physical depression scores than the type II deletion subtype. The Reiss Screen cut-off scores were used to determine whether differences occurred between the subtypes at a clinically significant level. These findings offer insight into the health care needs of people with PWS.

Fig. 1

Human chromosome 15q11-q13 region. Deletion type I spans from breakpoint 1 (BP1) to breakpoint 3 (BP3) whereas deletion type II spans from breakpoint 2 (BP2) to breakpoint 3. IC represents the location of the imprinting center. ^*Ordered according to the UCSC Genome Browser, May 2004 Assembly http://genome.ucsc.edu/.

Fig. 2

Mean Reiss scale scores and 26-item total score for deletion type I and deletion type II, and median Reiss maladaptive behavior items for deletion type I and deletion type II. Note: Means are used for the eight scale scores and 26-item total score and medians are used for the six maladaptive behavior items.