Skinner Martie, Hooper Stephen, Hatton Deborah D, Roberts Jane, Mirrett Penny, Schaaf Jennifer, Sullivan Kelly, Wheeler Anne, Bailey Donald B
Social Development Research Group, University of Washington, Seattle, Washington 98115, USA.
Am J Med Genet A. 2005 Jan 1;132A(1):25-32. doi: 10.1002/ajmg.a.30353.
This study examines the developmental changes in nonverbal intellectual functioning evident in males with fragile X syndrome (FXS) measured by the Leiter International Performance Scales-Revised (Leiter-R). The Leiter-R provides both IQ scores and associated growth scores which permit the examination of both age-based IQ scores and overall intellectual growth. Participants were 45 males with full mutation FXS and ranged in age from 4.0 to 13.8 years. Each child was assessed annually using the Leiter-R as part of a larger longitudinal battery for an average of 3.5 assessments per child and a range of 2-6 assessments, representing a total of 156 assessment occasions. Longitudinal analyzes of Leiter scores consisted primarily of hierarchical linear modeling, with the impact of chronological age, maternal education, fragile X mental retardation 1 protein (FMRP), autistic behaviors also being assessed. Findings revealed a significant linear decline in nonverbal IQ scores, with no effects of maternal education, autistic behaviors, or FMRP on mean level or rate of change in IQ scores over time. The decline slowed significantly around 8 years of age, but scores continued to decline into the 12th year of age. In contrast, a significant linear increase was observed in Leiter-R growth scores, which was negatively influenced by autistic behaviors. The rate of increase did not change over time, and neither mean level nor rate of increase was influenced by maternal education or FMRP levels. These findings suggest that declines in IQ are the result of steady, but suboptimal intellectual growth, rather than a true deterioration in overall intellectual functioning.
本研究通过修订版莱特尔国际操作量表(Leiter-R),考察了脆性X综合征(FXS)男性患者非言语智力功能的发育变化。Leiter-R提供智商分数和相关的增长分数,这使得我们能够同时考察基于年龄的智商分数和整体智力增长情况。研究参与者为45名患有完全突变型FXS的男性,年龄在4.0至13.8岁之间。每个孩子每年都使用Leiter-R进行评估,这是一个更大规模纵向测评组合的一部分,每个孩子平均接受3.5次评估,评估次数在2至6次之间,总共进行了156次评估。对Leiter分数的纵向分析主要包括分层线性建模,同时还评估了实足年龄、母亲教育程度、脆性X智力低下1蛋白(FMRP)、自闭症行为的影响。研究结果显示,非言语智商分数呈显著线性下降,母亲教育程度、自闭症行为或FMRP对智商分数随时间的平均水平或变化率均无影响。下降趋势在8岁左右显著放缓,但分数在12岁时仍持续下降。相比之下,Leiter-R增长分数呈显著线性上升,且受到自闭症行为的负面影响。增长速度并未随时间变化,平均水平和增长速度均不受母亲教育程度或FMRP水平的影响。这些发现表明,智商下降是稳定但未达最优的智力增长的结果,而非整体智力功能的真正衰退。