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.
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.
J Am Acad Child Adolesc Psychiatry. 2009 Nov;48(11):1060-1068. doi: 10.1097/CHI.0b013e3181b76683.
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.
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.
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.
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.
心脏面部综合征（VCFS）与认知缺陷以及精神分裂症和其他神经精神障碍的高发病率有关。我们报告了来自以色列和西欧的两个大型 VCFS 个体队列的数据，以在大样本中描述从儿童期到成年期的神经精神表型。
年龄在 5 至 54 岁之间的 VCFS 个体（n=172）接受了精神病障碍的结构化临床访谈以及适合年龄的韦氏智力测验版本。
精神障碍的频率很高，两个样本之间非常相似。精神疾病和抑郁症在儿童期不常见，但在成年期发病率增加（抑郁障碍：年轻成年人[18-24 岁]中为 40.7%；精神障碍：成年[年龄>24 岁]中为 32.1%）。认知评分与 VCFS 患者的年龄呈反比，包括无精神病的患者。具体而言，言语智商（VIQ）评分与年龄呈负相关，且患有精神病的 VCFS 患者的 VIQ 评分明显低于非精神病性 VCFS 患者。
VCFS 个体的神经精神缺陷似乎遵循一种发展模式。VIQ 评分与年龄和情绪障碍的发生率呈负相关，且心境和精神病障碍在青年期急剧增加。此处呈现的数据支持在 VCFS 中青少年和成年期期间仔细监测精神症状。需要前瞻性纵向研究来检查与 VCFS 相关的与年龄相关的认知变化的性质及其与精神发病率的关联。
