Self-injurious behavior

Abstract

Self-injurious behavior (SIB) is a relatively common behavior in individuals with intellectual disabilities (ID). Severe SIB can be devastating and potentially life-threatening. There is increasing attention for somatic substrates of behavior in genetic syndromes, and growing evidence of an association between pain and discomfort with SIB in people with ID and genetic syndromes. In this review on SIB phenomenology in people with ID in general and in twelve genetic syndromes, we summarize different SIB characteristics across these etiologically distinct entities and identify influencing factors. We demonstrate that the prevalence of SIB in several well-known genetic intellectual disability syndromes is noticeably higher than in individuals with ID in general, and that characteristics such as age of onset and topographies differ widely across syndromes. Each syndrome is caused by a mutation in a different gene, and this allows detection of several pathways that lead to SIB. Studying these with the behavioral consequences as specific aim will be an important step toward targeted early interventions and prevention.

Keywords: Angelman syndrome; Cornelia de Lange Syndrome; Cri du Chat Syndrome; Down syndrome; Fragile X syndrome; Genes; Genetic syndromes; Intellectual disability; Interdisciplinary study; Lesch-Nyhan syndrome; Lowe syndrome; Pain; Prader-Willi syndrome; Rett syndrome; Self-injurious behavior; Smith-Magenis syndrome; Somatic factors; Tuberous Sclerosis Syndrome; Williams-Beuren syndrome.