Cognitive and behavioural characteristics of children with Smith-Magenis syndrome

The study aimed to identify behavioural and cognitive characteristics in 29 children with Smith-Magenis syndrome. Cognitive assessments were undertaken on the children, and detailed interviews assessing sleep patterns, maladaptive behaviours, self-injury, hyperactivity and autism were carried out with their parents and teachers.;The study identified high levels of sleep problems, aggression, self-injury, distractibility and autism in the sample, in comparison with rates reported for other groups of children with learning disabilities. These were associated with high levels of stress in the parents.;It is concluded that the combination of difficulties and abilities identified in the present sample of children with SMS is indicative of a behavioural phenotype for the syndrome, and that there is an urgent need for intervention studies on the challenging behaviours posed by this group of children.