Psychiatric disorders and intellectual functioning throughout development in velocardiofacial (22q11.2 deletion) syndrome.
OBJECTIVE
Velocardiofacial syndrome (VCFS) is associated with cognitive deficits and high rates of schizophrenia and other neuropsychiatric disorders. We report the data from two large cohorts of individuals with VCFS from Israel and Western Europe to characterize the neuropsychiatric phenotype from childhood to adulthood in a large sample.


METHODS
Individuals with VCFS (n = 172) aged 5 to 54 years were evaluated with structured clinical interviews for psychiatric disorders and age-appropriate versions of the Wechsler intelligence tests.


RESULTS
The frequency of psychiatric disorders was high and remarkably similar between samples. Psychotic disorders and depression were uncommon during childhood but increased in rates during adulthood (depressive disorders: 40.7% in young adults [aged 18-24 years]; psychotic disorders: 32.1% in adults [age >24 years]). Cognitive scores were inversely associated with age in subjects with VCFS, including patients without psychosis. Specifically, Verbal IQ (VIQ) scores negatively correlated with age, and the subjects with VCFS and psychotic disorders had significantly lower VIQ scores than nonpsychotic VCFS subjects.


CONCLUSIONS
Neuropsychiatric deficits in individuals with VCFS seem to follow a developmental pattern. The VIQ scores are negatively associated with age and rates of mood, and psychotic disorders increase dramatically during young adulthood. The data presented here support careful monitoring of psychiatric symptoms during adolescence and young adulthood in VCFS. Prospective longitudinal studies are needed to examine the nature of age-related cognitive changes and their association with psychiatric morbidity in VCFS.
Drs. Green, Gothelf, Frisch, Kotler, and Weizman are with the Sackler Faculty of Medicine, Tel Aviv University; Drs. Green and Kotler are with the Nes-Ziyyona-Beer Yaakov Mental Health Center; Dr. Gothelf is with the Behavioral Neurogenetics Center, Schneider Children's Medical Center affiliated with Sackler Faculty of Medicine, Tel Aviv University; Drs. Glaser, Debbane, and Eliez are with the University of Geneva School of Medicine; Drs. Frisch and Weizman are with the Felsenstein Medical Research Center; Dr. Weizman is with the Geha Mental Health Center.
Tamar
T
Green
Drs. Green, Gothelf, Frisch, Kotler, and Weizman are with the Sackler Faculty of Medicine, Tel Aviv University; Drs. Green and Kotler are with the Nes-Ziyyona-Beer Yaakov Mental Health Center; Dr. Gothelf is with the Behavioral Neurogenetics Center, Schneider Children's Medical Center affiliated with Sackler Faculty of Medicine, Tel Aviv University; Drs. Glaser, Debbane, and Eliez are with the University of Geneva School of Medicine; Drs. Frisch and Weizman are with the Felsenstein Medical Research Center; Dr. Weizman is with the Geha Mental Health Center. Electronic address: gothelf@post.tau.ac.il.
Doron
D
Gothelf
Drs. Green, Gothelf, Frisch, Kotler, and Weizman are with the Sackler Faculty of Medicine, Tel Aviv University; Drs. Green and Kotler are with the Nes-Ziyyona-Beer Yaakov Mental Health Center; Dr. Gothelf is with the Behavioral Neurogenetics Center, Schneider Children's Medical Center affiliated with Sackler Faculty of Medicine, Tel Aviv University; Drs. Glaser, Debbane, and Eliez are with the University of Geneva School of Medicine; Drs. Frisch and Weizman are with the Felsenstein Medical Research Center; Dr. Weizman is with the Geha Mental Health Center.
Bronwyn
B
Glaser
Drs. Green, Gothelf, Frisch, Kotler, and Weizman are with the Sackler Faculty of Medicine, Tel Aviv University; Drs. Green and Kotler are with the Nes-Ziyyona-Beer Yaakov Mental Health Center; Dr. Gothelf is with the Behavioral Neurogenetics Center, Schneider Children's Medical Center affiliated with Sackler Faculty of Medicine, Tel Aviv University; Drs. Glaser, Debbane, and Eliez are with the University of Geneva School of Medicine; Drs. Frisch and Weizman are with the Felsenstein Medical Research Center; Dr. Weizman is with the Geha Mental Health Center.
Martin
M
Debbane
Drs. Green, Gothelf, Frisch, Kotler, and Weizman are with the Sackler Faculty of Medicine, Tel Aviv University; Drs. Green and Kotler are with the Nes-Ziyyona-Beer Yaakov Mental Health Center; Dr. Gothelf is with the Behavioral Neurogenetics Center, Schneider Children's Medical Center affiliated with Sackler Faculty of Medicine, Tel Aviv University; Drs. Glaser, Debbane, and Eliez are with the University of Geneva School of Medicine; Drs. Frisch and Weizman are with the Felsenstein Medical Research Center; Dr. Weizman is with the Geha Mental Health Center.
Amos
A
Frisch
Drs. Green, Gothelf, Frisch, Kotler, and Weizman are with the Sackler Faculty of Medicine, Tel Aviv University; Drs. Green and Kotler are with the Nes-Ziyyona-Beer Yaakov Mental Health Center; Dr. Gothelf is with the Behavioral Neurogenetics Center, Schneider Children's Medical Center affiliated with Sackler Faculty of Medicine, Tel Aviv University; Drs. Glaser, Debbane, and Eliez are with the University of Geneva School of Medicine; Drs. Frisch and Weizman are with the Felsenstein Medical Research Center; Dr. Weizman is with the Geha Mental Health Center.
Moshe
M
Kotler
Drs. Green, Gothelf, Frisch, Kotler, and Weizman are with the Sackler Faculty of Medicine, Tel Aviv University; Drs. Green and Kotler are with the Nes-Ziyyona-Beer Yaakov Mental Health Center; Dr. Gothelf is with the Behavioral Neurogenetics Center, Schneider Children's Medical Center affiliated with Sackler Faculty of Medicine, Tel Aviv University; Drs. Glaser, Debbane, and Eliez are with the University of Geneva School of Medicine; Drs. Frisch and Weizman are with the Felsenstein Medical Research Center; Dr. Weizman is with the Geha Mental Health Center.
Abraham
A
Weizman
Drs. Green, Gothelf, Frisch, Kotler, and Weizman are with the Sackler Faculty of Medicine, Tel Aviv University; Drs. Green and Kotler are with the Nes-Ziyyona-Beer Yaakov Mental Health Center; Dr. Gothelf is with the Behavioral Neurogenetics Center, Schneider Children's Medical Center affiliated with Sackler Faculty of Medicine, Tel Aviv University; Drs. Glaser, Debbane, and Eliez are with the University of Geneva School of Medicine; Drs. Frisch and Weizman are with the Felsenstein Medical Research Center; Dr. Weizman is with the Geha Mental Health Center.
Stephan
S
Eliez
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D003431:Cross-Cultural Comparison; D004062:DiGeorge Syndrome / Q000175:diagnosis* / Q000235:genetics* / Q000523:psychology; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis* / Q000235:genetics* / Q000523:psychology; D007557:Israel; D007805:Language Development Disorders / Q000175:diagnosis / Q000235:genetics / Q000523:psychology; D008297:Male; D001523:Mental Disorders / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D008875:Middle Aged; D010641:Phenotype; D011594:Psychometrics; D011618:Psychotic Disorders / Q000175:diagnosis* / Q000235:genetics* / Q000523:psychology; D012559:Schizophrenia / Q000175:diagnosis* / Q000235:genetics*; D012565:Schizophrenic Psychology; D013557:Switzerland; D014888:Wechsler Scales / Q000706:statistics & numerical data; D055815:Young Adult