A review of behavioral treatments used for Lesch-Nyhan syndrome

Lesch-Nyhan syndrome is a genetic disorder resulting in hyperuricemia chore oathetosis, mental retardation, and self-mutilation. The most salient featu re of this disorder is the self-injurious behavior (SIB). Although the util ity of behavioral interventions with SIE has been well documented, behavior al interventions with Lesch-Nyhan syndrome have been limited in number and long-term success. This article reviews the behavioral treatments that have been used in treating individuals with Lesch-Nyhan syndrome and discusses the strengths and weaknesses of these methods. Suggestions for future direc tions in the use of behavioral interventions for controlling SIE in Lesch-N yhan syndrome are provided.