BEHAVIORAL AND EMOTIONAL DISTURBANCE IN FRAGILE-X-SYNDROME

A range of behaviours has been postulated as specific manifestations o f the Fragile X (fra(X)) syndrome. This study examines a broad range o f behaviours in subjects with fra(X) syndrome and in control individua ls. It was designed to take account of methodological factors which ma y account for differing findings in previous studies. Subjects were 48 children, adolescents, and young adults with fra(X) syndrome. They we re compared with a large community epidemiological sample (N = 454) of persons with mental retardation (M.R.), matched for age, sex, and IQ. Behavioural problems were studied using the Developmental Behaviour C hecklist, an instrument of established reliability and validity in the evaluation of behavioural and emotional disturbance in children and a dolescents with mental retardation. Fragile X subjects had more shynes s and avoidance of eye contact and less antisocial behaviour. Further methodological issues pertaining to studies of behaviour phenotype in general are discussed. (C) 1994 Wiley-Liss, Inc.