MALADAPTIVE BEHAVIOR IN CHILDREN WITH PRADER-WILLI-SYNDROME, DOWN-SYNDROME, AND NONSPECIFIC MENTAL-RETARDATION

Although some genetic, mental retardation syndromes have well-describe d behavioral features, comparative studies have not yet assessed the r elative uniqueness of these so-called phenotypes. Maladaptive behavior of 43 children with Prader-Willi syndrome was compared to age-and gen der-matched children with Down syndrome and with nonspecific mental re tardation. The Prader-Willi group showed more frequent and severe inte rnalizing, externalizing, and total problem behaviors on the Child Beh avior Checklist. Some problems were elevated in all groups, and 12 beh aviors were significantly elevated in Prader-Willi subjects relative t o both comparison groups. Seven behaviors predicted membership into th e Prader-Willi group with 91% accuracy. Implications were discussed fo r research on behavioral phenotypes in general and for dual diagnosis in particular.