Psychological profile of children with Noonan syndrome

• Published in: Developmental medicine and child neurology 2005-01, Vol.47 (1), p.35-38
• Main Authors: Lee, DA, Portnoy, S, Hill, P, Gillberg, C, Patton, MA
• Format: Article
• Language: English
• Subjects: Adolescent; Child; Child Behavior Disorders; Child Behavior Disorders - diagnosis; Child Behavior Disorders - psychology; Child, Preschool; Cohort Studies; Communication Problems; Coordination; Diagnosis; Emotional Adjustment; Emotional Problems; England; Evidence; Family Characteristics; Female; Follow-Up Studies; Home Visits; Humans; Intelligence; Intelligence Quotient; Learning Disabilities; Learning Disorders; Learning Disorders - diagnosis; Learning Disorders - psychology; Male; Males; Neurologic Examination; Neurologic Examination - statistics & numerical data; Neuropsychological Tests; Neuropsychological Tests - statistics & numerical data; Noonan Syndrome; Noonan Syndrome - diagnosis; Noonan Syndrome - psychology; Original Articles; Parents; Personality Inventory; Personality Inventory - statistics & numerical data; Preschool; Psychiatry; Psychology; Psychometrics; Psychometrics - statistics & numerical data; Psychomotor Disorders; Psychomotor Disorders - diagnosis; Psychomotor Disorders - psychology; Psychomotor Performance; Psykiatri; Questionnaires; Reference Values; Self Concept; Self Evaluation (Individuals); Special Needs Students; Statistics & numerical data; Wechsler Scales
• ISSN: 0012-1622; 1469-8749
• DOI: 10.1017/S001216220500006X

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. 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.