Abstract
BACKGROUND: The 22q11.2 deletion syndrome (22q11.2DS) represents a genetic condition at higher risk of transition to psychosis. Both self-esteem (SE), intended as self-evaluation based on cognitive and affective elements, and psychotic symptoms may be associated with patients' real-life functioning. We investigated whether these variables differently correlate with real-life functioning in 22q11.2DS. METHODS: We recruited 22 patients with 22q11.2DS (DEL, N = 22) and 10 with 22q11.2DS and psychosis (DEL-SCZ, N = 10); we administered the Positive And Negative Syndrome Scale (PANSS), the Specific Levels of Functioning scale (SLoF) and the Self Esteem Rating Scale (SERS). RESULTS: The DEL-SCZ and DEL groups did not significantly differ on the SERS (p = 0.228). The DEL group scored higher than DEL-SCZ on the SLoF-total (p = 0.006) and on the SLoF-social functioning (p = 0.031). PANSS-total negatively correlated with SLoF-total scores (ρ = -0.698; p < 0.001), with the SLoF-social functioning (ρ = -0.643; p < 0.001) and with SERS (ρ = -0.391; p = 0.036). SERS scores positively correlated with SLoF-total (ρ = 0.545; p = 0.003) but not with SLoF-social functioning. DISCUSSION AND CONCLUSIONS: DEL and DEL-SCZ display similar levels of SE suggesting that this psychological dimension is not associated with psychotic symptoms. Levels of SE and psychopathology differentially relate to real-life and social functioning in people with 22q11.2DS: Symptom severity is particularly associated with patients' social and interpersonal functioning. Psychological supportive interventions might be useful to improve real-life functioning in people with 22q11.2DS.