Abstract
More than half the individuals with Prader-Willi syndrome (PWS) have infantile hypotonia, obesity, hypogonandism, mental retardation, and an interstitial deletion of the proximal long arm of chromosome 15. Because many such individuals die in adulthood as a result of complications of morbid obesity, weight management is an essential aspect of treatment for this syndrome. The present article reports a case study of a 13-year-old boy with Prader-Willi syndrome who required a low-calorie diet because of his obesity and its severe complications (heart failure, diabetes, Pickwickian syndrome, and hypertension). In addition, the boy had many behavior problems, including both food-related behavior (food stealing or stealing money for food) and other maladaptive behavior (including running away from home, lying, insisting on routines, and temper tantrums). Because of his behavior problems, medical treatments prescribed by pediatricians had not been effective for keeping his weight down. Therefore, we devised an operant conditioning program in order to motivate him to participate in the weight-control program. He then lost a significant amount of weight (16 kg), and the complications of his obesity and maladaptive behavior were alleviated.
