Adolescents and young adults with 22qll deletion syndrome: psychopathology in an at-risk group

Abstract
Background: 22q11 deletion syndrome is associated with high rates of psychiatric morbidity, especially schizophrenia. If common neurodevelopmental trajectories characterise 22q11 deletion syndrome and idiopathic schizophrenia, then similar ‘premorbid’ features would be predicted.Aims: To define psychopathology in adolescents and young adults with 22q11 deletion syndrome.Method: Individuals with 22q11 deletion syndrome (n=25) and age- and IQ-matched comparison participants (n=25), along withtheir parents/carers, completed standardised semi-structured assessments.Results: Psychiatric disorders were more prevalent among those with the syndrome and included attention deficit, depression and anxiety; nearly half reported transient psychotic experiences. Inappropriate emotionality and disrupted social behaviour were also common. Poor functional adjustment was associated with schizotypal symptoms and was more marked in older individuals. Conclusions 22q11 deletion syndrome is associated with significant psychopathology prior to adulthood. Double the proportion expected to develop schizophrenia reported psychotic phenomena. These findings imply a continuum of developmental disruption in 22q11 deletion syndrome, associated with declining mental health in early adulthood.