Purpose: Patients with one of the 22q11.2 deletion syndromes provide a uniq
ue opportunity to research the interface between genetics and brain-behavio
r relationships. This study investigates the neuropsychological characteris
tics and behavioral phenotype of children with this deletion syndrome. Meth
ods: We report updated findings from descriptive and nonparametric analyses
of neuropsychological data from 80 children with the 22q11.2 deletion. Res
ults: The subjects showed higher verbal than nonverbal IQ scores, assets in
verbal memory, and deficits in the areas of attention, story memory, visuo
spatial memory, arithmetic performance relative to other areas of achieveme
nt, and psychosocial functioning. Conclusion: Children with 22q11.2 deletio
n syndromes exhibit a behavioral phenotype reflective of nonverbal learning
disabilities, concomitant language deficits, and social-emotional concerns
.