title: Shyness discriminates between children with 22q11.2 deletion syndrome and Williams syndrome and predicts emergence of psychosis in 22q11.2 deletion syndrome.
abstract: BACKGROUND
  22q11.2 deletion syndrome (22q11.2DS) 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 22q11.2DS compared to children with Williams syndrome (WS) and typically developing (TD) controls, and to examine temperamental predictors of the emergence of psychosis in 22q11.2DS.
  
  
  METHODS
  The temperament of 55 children with 22q11.2DS, 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 22q11.2DS at baseline and again 5.43 ± 2.23 years after baseline temperament assessment.
  
  
  RESULTS
  Children with 22q11.2DS scored higher on Shyness compared to WS and TD controls. Children with 22q11.2DS 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 22q11.2DS. Baseline Shyness scores significantly predicted the later emergence of a psychotic disorder at follow-up, in children with 22q11.2DS.
  
  
  CONCLUSIONS
  Our results suggest that shyness is an early marker associated with the later emergence of psychosis in 22q11.2DS.
authors: Yael
authors: Y
authors: Schonherz
authors: Maayan
authors: M
authors: Davidov
authors: Ariel
authors: A
authors: Knafo
authors: Hadas
authors: H
authors: Zilkha
authors: Gal
authors: G
authors: Shoval
authors: Gil
authors: G
authors: Zalsman
authors: Amos
authors: A
authors: Frisch
authors: Abraham
authors: A
authors: Weizman
authors: The Behavioral Neurogenetics Center, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel. gothelf@post.tau.ac.il.
authors: Doron
authors: D
authors: Gothelf