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J Med Genet 34:453-458 doi:10.1136/jmg.34.6.453
  • Research Article

Intelligence and psychosocial adjustment in velocardiofacial syndrome: a study of 37 children and adolescents with VCFS.

  1. A Swillen,
  2. K Devriendt,
  3. E Legius,
  4. B Eyskens,
  5. M Dumoulin,
  6. M Gewillig,
  7. J P Fryns
  1. Centre for Human Genetics, University Hospital Gasthuisberg, Leuven, Belgium.

      Abstract

      We report data on a group of 37 VCFS patients with specific reference to their intelligence, behaviour, and social competence. Fifty five percent of the children had a borderline to normal IQ. Mental retardation (defined as IQ < 70 or > -2 SD below the mean) was found in 45%. In the majority, the mental retardation was mild (38%) and only two patients had moderate mental retardation. Severe mental retardation seems to be rare in VCFS. The present study shows also that the incidence of mental retardation is much higher in the familial than the de novo group. Intelligence is not correlated with the presence or absence of a heart defect. Significantly higher verbal IQs than performance IQs (probably related to deficits in visuospatial-perceptual functioning) were found. Problems in social-emotional functioning and attention were also found. Further longitudinal studies are necessary to provide an accurate prognosis and appropriate intervention for VCFS children.