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Psychological profile of children with Noonan syndrome

D A Lee1, S Portnoy, P Hill

  • 1Sub Department of Clinical Health Psychology, University College London, 1-19 Philips House, Torrington Place, WC1E 6BT, London, UK. deborah.lee@ucl.ac.uk

Developmental Medicine and Child Neurology
|February 3, 2005
PubMed

Insights

Children with Noonan syndrome often experience learning disabilities and motor impairments. While self-esteem levels were comparable to peers, a distinct behavioral pattern was not identified in this study.

Area of Science:

  • Pediatric Psychology
  • Neurodevelopmental Disorders
  • Genetics

Background:

  • Noonan syndrome is a genetic disorder associated with various developmental challenges.
  • Previous research suggests potential cognitive and motor deficits in affected individuals.
  • Understanding the psychological profile is crucial for comprehensive care.

Purpose of the Study:

  • To investigate the cognitive, motor, and behavioral characteristics of children with Noonan syndrome.
  • To assess self-esteem and emotional/behavioral problems in this cohort.
  • To identify potential psychological phenotypes associated with Noonan syndrome.

Main Methods:

  • A cohort of 48 children with Noonan syndrome underwent standardized cognitive (Wechsler Intelligence Scales) and motor assessments (Test of Motor Impairment-Revised).
  • Self-esteem was evaluated using the Piers-Harris Self-evaluation Questionnaire.
  • Parent and teacher ratings on the Rutter A and B scales assessed behavioral and emotional issues.

Main Results:

  • The mean Full-scale IQ was 84, with 25% exhibiting learning disabilities; Verbal IQ was slightly lower than Performance IQ.
  • Approximately 50% demonstrated mild to moderate motor impairment, consistent with developmental coordination disorder.
  • Self-esteem levels were comparable to normative data, and no specific behavioral phenotype was identified.

Conclusions:

  • Children with Noonan syndrome exhibit characteristic psychological features, including cognitive and motor deficits.
  • While learning disabilities and motor impairments are common, self-esteem is generally within the normal range.
  • Further research is needed to fully elucidate the behavioral aspects of Noonan syndrome.

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