Psychoeducational profile of the 22q11.2 microdeletion: A complex pattern

Objectives: To examine the psychoeducational profile associated with the ch romosome 22q11.2 microdeletion (DiGeorge/velocardiofacial syndrome). Study design: Thirty-three patients (aged 6 to 27 years) with a 22q11.2 mic rodeletion underwent psychoeducational testing as part of a comprehensive e valuation. Nonparametric statistics were used to compare verbal and perform ance Ia academic achievement scores, and receptive versus expressive langua ge scores. Post hoc comparisons were made of IQ subtest scores and of langu age versus verbal IQ. Results: Full-scale IQ ranged from the normal to the moderately retarded ra nge. Mean verbal IQ was significantly higher than mean performance Ie. In a similar manner, mean reading and spelling scores were superior to the mean mathematics score, although achievement scores typically were in the range of verbal IQ. In addition, many children showed clinically significant lan guage impairments, with mean language scores lower than mean verbal IQ. Conclusions: The IQ and academic profiles are reminiscent of a "nonverbal l earning disability," although achievement was not discrepant from IQ. The c oincidence of language impairment with a relative strength in reading belie s a unique neuropsychologic profile. Educational programming for these chil dren must address both verbal and nonverbal deficits.