A solution to limitations of cognitive testing in children with intellectual disabilities: The case of fragile X syndrome

Medical Investigation of Neurodevelopmental Disorders (M.I.N.D.), Institute, University of California-Davis Medical Center, 2825 50th Street, Sacramento, CA 95817, USA.
Journal of Neurodevelopmental Disorders (Impact Factor: 3.27). 03/2009; 1(1):33-45. DOI: 10.1007/s11689-008-9001-8
Source: PubMed


Intelligence testing in children with intellectual disabilities (ID) has significant limitations. The normative samples of widely used intelligence tests, such as the Wechsler Intelligence Scales, rarely include an adequate number of subjects with ID needed to provide sensitive measurement in the very low ability range, and they are highly subject to floor effects. The IQ measurement problems in these children prevent characterization of strengths and weaknesses, poorer estimates of cognitive abilities in research applications, and in clinical settings, limited utility for assessment, prognosis estimation, and planning intervention. Here, we examined the sensitivity of the Wechsler Intelligence Scale for Children (WISC-III) in a large sample of children with fragile X syndrome (FXS), the most common cause of inherited ID. The WISC-III was administered to 217 children with FXS (age 6-17 years, 83 girls and 134 boys). Using raw norms data obtained with permission from the Psychological Corporation, we calculated normalized scores representing each participant's actual deviation from the standardization sample using a z-score transformation. To validate this approach, we compared correlations between the new normalized scores versus the usual standard scores with a measure of adaptive behavior (Vineland Adaptive Behavior Scales) and with a genetic measure specific to FXS (FMR1 protein or FMRP). The distribution of WISC-III standard scores showed significant skewing with floor effects in a high proportion of participants, especially males (64.9%-94.0% across subtests). With the z-score normalization, the flooring problems were eliminated and scores were normally distributed. Furthermore, we found correlations between cognitive performance and adaptive behavior, and between cognition and FMRP that were very much improved when using these normalized scores in contrast to the usual standardized scores. The results of this study show that meaningful variation in intellectual ability in children with FXS, and probably other populations of children with neurodevelopmental disorders, is obscured by the usual translation of raw scores into standardized scores. A method of raw score transformation may improve the characterization of cognitive functioning in ID populations, especially for research applications.

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    • "For each index, we calculated normalized scores (Z scores) based on procedures described by Hessl et al. (2009). We obtained raw score means and standard deviations from the WISC-III normative sample to calculate Z scores for each datum of each participant. "
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    ABSTRACT: Few studies have investigated developmental strengths and weaknesses within the cognitive profile of children and adolescents with fragile X syndrome (FXS), a single-gene cause of inherited intellectual impairment. With a prospective longitudinal design and using normalized raw scores ( Z scores) to circumvent floor effects, we measured cognitive functioning of 184 children and adolescents with FXS (ages 6 to 16) using the Wechsler Scale of Intelligence for Children on one to three occasions for each participant. Participants with FXS received lower raw scores relative to the Wechsler Scale of Intelligence for Children normative sample across the developmental period. Verbal comprehension, perceptual organization, and processing speed Z scores were marked by a widening gap from the normative sample, while freedom from distractibility Z scores showed a narrowing gap. Key findings include a relative strength for verbal skills in comparison with visuospatial–constructive skills arising in adolescence and a discrepancy between working memory (weakness) and processing speed (strength) in childhood that diminishes in adolescence. Results suggest that the cognitive profile associated with FXS develops dynamically from childhood to adolescence. Findings are discussed within the context of aberrant brain morphology in childhood and maturation in adolescence. We argue that assessing disorder-specific cognitive developmental profiles will benefit future disorder-specific treatment research.
    No preview · Article · Dec 2015 · Development and Psychopathology
    • "Alternatives to the RIQ are available. For example, Fragile X researchers have accounted for floor effects in some common intelligence tests by developing alternative deviation scoring systems (Hessl et al., 2009; Sansone et al., 2014), though this approach has not yet been applied to preschool testing. In the context of research studies, a predicted score might be used (Whitaker & Gordon, 2012). "
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    ABSTRACT: Estimates of intelligence in young children with neurodevelopmental disorders are critical for making diagnoses, in characterizing symptoms of disorders, and in predicting future outcomes. The limitations of standardized testing for children with developmental delay or cognitive impairment are well known: Tests do not exist that provide developmentally appropriate material along with norms that extend to the lower reaches of ability. Two commonly used and interchanged instruments are the Mullen Scales of Early Learning (MSEL), a test of developmental level, and the Differential Ability Scales, second edition (DAS-II), a more traditional cognitive test. We evaluated the correspondence of contemporaneous MSEL and the DAS-II scores in a mixed sample of children aged 2-10 years with autism spectrum disorder (ASD), non-ASD developmental delays, and typically developing children across the full spectrum of cognitive ability. Consistent with published data on the original DAS and the MSEL, scores on the DAS-II and MSEL were highly correlated. However, curve estimation revealed large mean differences that varied as a function of the child's cognitive ability level. We conclude that interchanging MSEL and DAS-II scores without regard to the discrepancy in scores may produce misleading results in both cross-sectional and longitudinal studies of children with and without ASD, and, thus, this practice should be implemented with caution.
    No preview · Article · Apr 2015 · Child Neuropsychology
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    • "All children but one were in the lower end of the standard scores, indicating a floor effect. This effect is well-known in research with intellectually disabled persons (e.g., Carlier & Ayoun, 2007; Carlier & Roubertoux, 2014; Hessl et al., 2009). It arises with most intelligence tests (Reynolds & Kamphaus, 2003), which therefore typically fail to detect valid differences in the lower ranges. "
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    ABSTRACT: People spontaneously use faces to make inferences about other's personality traits or abilities, which generally lead to invalid conclusions. Here, we show first evidence that perceived variations in the facial appearance of 20 children with trisomy 21 (t21) influence how they are perceived in terms of intelligence (or intellectual disability), the more “trisomic” faces being rated as less intelligent (or more intellectually disabled). Despite high degrees of inter-rater agreement (80 raters), these inferences were unrelated to individuals’ actual test scores which were also unrelated to perceived facial appearance. All these findings indicate that social inferences about intelligence based on facial appearance are unreliable even in groups characterized by a genetic disorder such as t21.
    Full-text · Article · Dec 2014 · Research in Developmental Disabilities
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