Psychological profile of children with Noonan syndrome

Abstract

A cohort of 48 children with Noonan syndrome, with a mean age of 9 years 10 months (SD 3y 7mo; 28 males, 20 females), was recruited from a national study. Children were assessed using the Wechsler Intelligence Scales and Test of Motor Impairment–Revised (TOMI-R). The Piers–Harris Self-evaluation Questionnaire was also completed in a subgroup of age-appropriate children. Children's parents and teachers completed the Rutter A and B scales to assess of the degree of behavioural and emotional problems respectively. Mean Full-scale IQ score was 84, and one-quarter of the participants had learning disabilities** US usage: mental retardation. . Verbal IQ tended to be slightly lower than Performance IQ. About half of the group showed evidence of mild to moderate impairment, confirming the impression of‘clumsiness/developmental coordination disorder’on the TOMI-R. Level of self-esteem, as determined by the Piers-Harris Questionnaire, was comparable to that of a standardized population. This research has identified some characteristic psychological features in Noonan syndrome. However, a specific behavioural phenotype could not be identified.