Capone George T, Grados Marco A, Kaufmann Walter E, Bernad-Ripoll Susana, Jewell Amy
Department of Neurology and Developmental Medicine, Kennedy Krieger Institute, Baltimore, Maryland 21205, USA.
Am J Med Genet A. 2005 May 1;134(4):373-80. doi: 10.1002/ajmg.a.30622.
To report on the cognitive and behavioral attributes of 61 children with Down syndrome (DS) and autistic-spectrum disorder (ASD) according to DSM-IV criteria; to determine the utility of the aberrant behavior checklist (ABC) to characterize these subjects for research purposes; and to test the hypothesis that subjects with DS + ASD could be distinguished from their typical DS peers using the ABC. Cross-sectional design. Cases with DS + ASD (N = 61), comparison group of DS + stereotypy movement disorder (SMD) (N = 26) and typical DS controls without behavior problems (N = 44) were ascertained and enrolled sequentially upon presentation to a DS clinic at an academic medical center over a 10-year period from 1991 to 2001. All subjects underwent neurodevelopmental and medical evaluation, and standardized cognitive testing. The parents provided responses to standardized behavioral questionnaires. Cognitive function (IQ) differed markedly across the three groups. The Lethary and Stereotypy subscales of the ABC were highly significant (P < 0.001) in distinguishing the three groups from one another. Within the ASD group differences were apparent by DSM-IV type on the Lethargy subscale, which reached significance, ANOVA (F = 0.002) and t-test (Autism > PDD, P = 0.005; PDD < CDD, P = 0.002). Using a multivariate regression model, the ABC scales alone explained 62% of variance of ASD outcome; addition of demographic variables explained up to 68% of the variance. There is good correlation between DSM-IV criteria for autism and subscales scores on the ABC in subjects with DS. This study demonstrates the feasibility of using the ABC to characterize the neurobehavioral phenotype of a cohort of children with trisomy 21 and ASD for ongoing research purposes.
根据《精神疾病诊断与统计手册第四版》(DSM-IV)标准,报告61例患有唐氏综合征(DS)和自闭症谱系障碍(ASD)儿童的认知和行为特征;确定异常行为检查表(ABC)用于对这些受试者进行研究特征描述的效用;并检验使用ABC可将患有DS+ASD的受试者与其典型的DS同龄人区分开来的假设。横断面设计。确定并连续纳入了患有DS+ASD的病例(N=61)、DS+刻板运动障碍(SMD)的对照组(N=26)以及无行为问题的典型DS对照组(N=44),这些病例是在1991年至2001年的10年期间,在一家学术医疗中心的DS诊所就诊时确定的。所有受试者均接受了神经发育和医学评估以及标准化认知测试。家长对标准化行为问卷做出了回应。三组之间的认知功能(智商)差异显著。ABC的 lethary和刻板性子量表在区分这三组方面具有高度显著性(P<0.001)。在ASD组中,根据DSM-IV类型,在嗜睡子量表上存在明显差异,差异具有显著性,方差分析(F=0.002)和t检验(自闭症>PDD,P=0.005;PDD<CDD,P=0.002)。使用多元回归模型,仅ABC量表就解释了ASD结果变异的62%;加入人口统计学变量后,解释的变异高达68%。DS受试者中,自闭症的DSM-IV标准与ABC子量表得分之间存在良好的相关性。本研究证明了使用ABC对一组患有21三体综合征和ASD的儿童进行神经行为表型特征描述以用于正在进行的研究目的的可行性。