Self-injurious behavior and Prader-Willi syndrome: Behavioral forms and body locations

With few exceptions (e.g.,Lesch-Nyhan syndrome), the specific nature of sel f-injury in relation to identified genetic syndromes associated with mental retardation is poorly understood. In the present study we surveyed the fam ilies of 62 persons with Prader-Willi syndrome to determine the prevalence, topographies, and specific body locations of self-injurious behavior. Self -injury was reported for 81% of the participants. Skin-picking was the most prevalent form, with the front of the legs and head being disproportionate ly targeted as preferred self-injury body sites. Individuals with the 15q11 -q13 deletion injured significantly more body sites than did individuals wi th maternal disomy 15. Results are discussed in relation to previous self-i njury body site findings and implications for the relevance of syndrome-spe cific behavioral phenotypes.