Self-mutilative behavior as a feature of the de Lange syndrome

Cornelia De Lange, Cri Du Chat, and Rubinstein-Taybi Syndromes

“…Three separate studies have demonstrated the effect of environmental variables such as adult attention on the expression of self-injury and the effectiveness of behavioural treatment programmes in reducing the self-injury shown by three children with CdLS (Bay et al, 1993;Menolascino, McGee & Swanson, 1982;Singh & Pullman, 1979). The data from these three studies support the notion that self-injury in individuals with CdLS can be Detailed observations of individuals with CdLS have shown that those who self-injure may seek restraint and that distress is evident when physical restraints are removed (Dosseter et al, 2001;Shear, Nyhan, Kirman & Stern, 1971). Hyman et al (2002) found that 53% of individuals with CdLS showed at least one form of self-restraint.…”

“…Restraints and protective devices are also commonly required to manage self-injury in CdLS and the 38 to 40% prevalence rate for the use of restraints reported in the literature (Berney et al, 1999;Gualtieri, 1990) is three times that seen in general intellectual disability populations (Oliver et al, 1987). Individuals also express a preference for wearing restraints and a fear of being without restraints (Dossetor et al, 1991;Shear et al, 1971). Incorporating a measure of self-restraint, Hyman et al (2002) found that 53% of participants displayed at least one form of self-restraint with a significant association between self-injury and selfrestraint.…”

“…Incorporating a measure of self-restraint, Hyman et al (2002) found that 53% of participants displayed at least one form of self-restraint with a significant association between self-injury and selfrestraint. A number of authors have also suggested that self-injurious behaviour in CdLS has a "compulsive quality" (Bryson et al, 1971;Shear et al, 1971). Hyman et al (2002) demonstrated that compulsive behaviour was associated with self-injury and selfrestraint.…”

A Behavioral Phenotype in the de Lange Syndrome

“…Some of these self-stimulatory repetitive activities appear to be self-aversive. We have previously described self-mutilative behavior in patients with this syndrome (3,15). One of the two patients that died during the period covered by this study had developed an increasing pattern of self-mutilative behavior and died in an act that could be interpreted as suicide.…”

“…They reject the usual physical contacts children have with other people. Characteristically they have no speech (10,(14)(15)(16). They make low pitched, growling or animal-like sounds that are monotonous and without the remarkable variation in sound that characterizes speech or even the normal crying of infants.…”