BEHAVIOR IN MUCOPOLYSACCHARIDE DISORDERS

This paper reports a study of the nature and prevalence of behaviour p roblems in 258 children with mucopolysaccharide disorders. Questionnai re data obtained through the post was supplemented by home visits to 4 2 families in the sample and by regular discussions with families at m eetings of the Society for Mucopolysaccharide Diseases. High rates of behaviour problems were found, particularly in children with Sanfilipp o's and Hunter's disease aged 5 to 9 years. These included destructive ness, restlessness, and aggressiveness. Sleep problems were common acr oss subtypes with an overall prevalence of 66%. Parents reported that they received little or no support in the management of these difficul t behaviours. It is concluded that behaviour problems are a primary fe ature of the mucopolysaccharide disorders and place a major strain on families. Services to help families cope with these problems are urgen tly needed.