The Use of Psychotropic Medications in Persons with Prader-Willi Syndrome

Abstract

Prader-Willi Syndrome (PWS) is an unusual and multifacted birth defect characterized by severe hypotonia, hypogenitalism, delayed development, and mental retardation (Prader et al, 1956; Zellweger and Schneider, 1968; Zellweger 1988). Hyperphagia becomes evident early in childhood and is accompanied by predisposition to increasingly unstable emotions and behavior Problems that intensify over time (Sulzbacher, 1988). If the insatiable appetite is left unmanaged, affected individuals may become morbidly obese and die of complications of obesity. A no less important component of the Syndrome is the evolution over time of inappropriate and often bizarre behaviors that escalate in frequency and intensity with age, necessitating ongoing management and Intervention (Greenswag, 1987). Anecdotal reports of chemical management of these behaviors, accompanied by conflicting opinions regarding their appropriateness and effectiveness, are emerging. No data exists to guide clinicians’ choice of medication, dosage, Potential side effects, and time frame for administration. Moreover, information comparing the effectiveness of psychotropic medications with non-drug behavioral Intervention is not available.