Purpose: This study was undertaken with a view to establishing the occurren
ce of neuropsychiatric disorders in the 22q11 deletion syndrome. Methods: T
hirty-two children and young adults with genetically confirmed 22q11 deleti
on were given comprehensive neuropsychiatric assessments. Results: Altogeth
er, 56% had a neuropsychiatric disorder. Only 6% were of normal IQ and free
of psychiatric disorder. Attention-deficit/hyperactivity disorder was diag
nosed in 44% and 31% had an autism spectrum problem. In 16% criteria for bo
th these diagnoses were met, fifty-three percent had mental retardation, of
ten with a test-profile suggesting a nonverbal learning disorder. Conclusio
n: The findings imply that a majority of children and adolescents with 22q1
1 deletion syndrome are in need of neuropsychiatric assessment and interven
tion.