Shyness discriminates between children with 22q11.2 deletion syndrome and Williams syndrome and predicts emergence of psychosis in 22q11.2 deletion syndrome.

[22q11.2 deletion syndrome]

22 q 11 .2 deletion syndrome (22 q 11 .2 DS ) is a common neurogenetic syndrome associated with high rates of psychosis . The aims of the present study were to identify the unique temperament traits that characterize children with 22 q 11 .2 DS compared to children with Williams syndrome (WS ) and typically developing (TD ) controls , and to examine temperamental predictors of the emergence of psychosis in 22 q 11 .2 DS .T he temperament of 55 children with 22 q 11 .2 DS , 36 with WS , and 280 TD children was assessed using the Emotionality , Activity , Sociability (EAS ) Temperament Survey , Parental Ratings . The presence of a psychotic disorder was evaluated in 49 children and adolescents with 22 q 11 .2 DS at baseline and again 5 .43 ± 2 .23 years after baseline temperament assessment .Children with 22 q 11 .2 DS scored higher on Shyness compared to WS and TD controls . Children with 22 q 11 .2 DS and WS scored higher on Emotionality and lower on Activity compared to TD controls . Shyness was more severe in older compared to younger children with 22 q 11 .2 DS . Baseline Shyness scores significantly predicted the later emergence of a psychotic disorder at follow-up , in children with 22 q 11 .2 DS .O ur results suggest that shyness is an early marker associated with the later emergence of psychosis in 22 q 11 .2 DS .