Assessment and Treatment of Self-Injurious Behavior Associated with Donnai-Barrow Syndrome.

Donnai and Barrow (American Journal of Medical Genetics, 68, 441-444, 1993) reported multiple participants who shared a variety of specific physical and neurological anomalies. Relatively few cases have been reported since then and few of those have progressed to an age such that the developmental progression of the disorder can be ascertained. We describe one participant with Donnai-Barrow syndrome who engaged in repetitive self-injurious behavior (SIB), which heretofore has not be described in this population. Direct observations of the participant's SIB were conducted across a range of contexts, and the behavior was successfully decreased using a combination of procedures. This case is discussed within the context of the behavioral phenotype of Donnai-Barrow syndrome.