Cognitive profiles associated with the fra(X) syndrome in males and females

Am J Med Genet. 1991 Mar 15;38(4):542-7. doi: 10.1002/ajmg.1320380409.


Twenty-three fragile X [fra(X)] males (mean IQ 50, mean age 10 years) and 11 fra(X) females (mean IQ 84, mean age 11.3 years) were administered the Stanford-Binet Intelligence Scale, 4th ed. (S-B). The cognitive profiles generated from the S-B indicated a pattern of subtest strengths and weaknesses that was similar for both groups. Consistent weaknesses in both groups were found in quantitative skills and short-term memory recall for visually presented, abstract stimuli, whereas a consistent strength was observed for short-term memory recall of visually presented, meaningful stimuli. Strengths in verbal labeling and comprehension and deficits in spatial visualization and visual-motor coordination were identified by the S-B for the male group but not for the female group. Fragility in the female group was negatively correlated with the S-B score for the short-term memory area and the combined S-B scores for the verbal reasoning and quantitative reasoning areas. The specific, S-B-identified cognitive strengths and weaknesses associated with the fra(X) condition are potentially useful indicators for investigation of specific cognitive process deficits associated with the fra(X) condition and the design of appropriate educational interventions for fra(X) children.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Cognition*
  • Female
  • Fragile X Syndrome / genetics*
  • Humans
  • Male
  • Memory
  • Spatial Behavior
  • Stanford-Binet Test
  • Verbal Behavior
  • Visual Perception