A syndrome with characteristic facial and skeletal features resulting from a specific deletion of material on the short arm of chromosome 17 was first described by Smith et al in 1982.1 The common findings are brachycephaly, hypoplasia of the middle face, and short broad hands associated with mental retardation. More than 50 cases have been reported, and about one third have congenital heart disease. We report here a patient with atrial and ventricular septal defects together with pulmonary valvar stenosis.

A behavioural approach was utilized in the assessment and treatment of severe self-injurious behaviour (SIB) in a woman with Smith-Magenis Syndrome (SMS). The results showed a marked reduction in the frequency of SIB following the introduction of functional assessment driven behavioural interventions (differential reinforcement of other behaviour, self-monitoring and social reinforcement, and extinction). There were also considerable reduc-tions in the frequencies of aggressive behaviour towards others, the use of rapid tranquilization and the use of control and restraint. The results of this study suggest environmental factors may play a significant role in maintaining some self-injurious behaviour shown by people with Smith Magenis Syndrome. The results are discussed in relation to emerging models of SIB in SMS.