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Validation of the NIH Toolbox Cognitive Battery in intellectual disability
Open accessAbstract
OBJECTIVE: To advance the science of cognitive outcome measurement for individuals with intellectual disability (ID), we established administration guidelines and evaluated the psychometric properties of the NIH-Toolbox Cognitive Battery (NIHTB-CB) for use in clinical research. METHODS: We assessed feasibility, test-retest reliability, and convergent validity of the NIHTB-CB (measuring executive function, processing speed, memory, and language) by assessing 242 individuals with fragile X syndrome (FXS), Down syndrome (DS), and other ID, ages 6 through 25 years, with retesting completed after 1 month. To facilitate accessibility and measurement accuracy, we developed accommodations and standard assessment guidelines, documented in an e-manual. Finally, we assessed the sensitivity of the battery to expected syndrome-specific cognitive phenotypes. RESULTS: Above a mental age of 5.0 years, all tests had excellent feasibility. More varied feasibility across tests was seen between mental ages of 3 and 4 years. Reliability and convergent validity ranged from moderate to strong. Each test and the Crystallized and Fluid Composite scores correlated moderately to strongly with IQ, and the Crystallized Composite had modest correlations with adaptive behavior. The NIHTB-CB showed known-groups validity by detecting expected executive function deficits in FXS and a receptive language deficit in DS. CONCLUSION: The NIHTB-CB is a reliable and valid test battery for children and young adults with ID with a mental age of ≈5 years and above. Adaptations for very low-functioning or younger children with ID are needed for some subtests to expand the developmental range of the battery. Studies examining sensitivity to developmental and treatment changes are now warranted.
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The lowest possible age-corrected standard score on the NIHTB-CB is 54 because scores were winsorized in a 2018 Toolbox update.
“The lowest possible age-corrected standard score is 54 as they were winsorized (i.e., extreme values are limited through a transformation) in a Toolbox update in 2018, presumably because extreme scores have lower reliability.”
From NIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome (2022) · cites this paper as Shields et al., 2020
The Methods (Data analysis) state that the current NIHTB-CB winsorizes age-corrected standard scores at 54, and the Discussion calls 54 the lower limit. Our copy does not date that change to a 2018 Toolbox update. The suggested reliability rationale is framed as the citing authors’ presumption, not a finding attributed to this source. (LLM, conf=0.88)
Experimental — accuracy not yet measuredThe rates of successful administration for each NIHTB-CB task in Williams syndrome are comparable to the initial validation study of the Toolbox in typically developing children and to those previously reported in individuals with intellectual disability, though higher on the DCCS in the present study (90% vs. 64% in the previous study of individuals with ID).
“These rates of successful administrations for each task are comparable to the initial validation study of the Toolbox in typically developing children and to those previously reported in individuals with ID (Shields et al., 2020), though higher on the DCCS in the present study (90% vs. 64% in the previous study of individuals with ID).”
From NIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome (2022) · cites this paper as Shields et al., 2020
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Experimental — accuracy not yet measuredPrevious studies examining the NIHTB-CB in ID used mental age to determine which subtest version was administered.
“previous studies examining the NIHTB-CB in ID used mental age to determine which subtest version was administered (Hessl et al., 2016; Shields et al., 2020).”
From NIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome (2022) · cites this paper as Shields et al., 2020
The present study based the task version administered on chronological age, unlike other studies looking at the Toolbox in ID, which have used mental age as estimated through IQ testing.
“The present study based the task version administered on chronological age, unlike other studies looking at the Toolbox in ID, which have used mental age as estimated through IQ testing.”
From NIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome (2022) · cites this paper as Shields et al., 2020
A floor effect was noted for age-corrected standard scoring of the NIHTB-CB measures in intellectual disability, which was remedied through a rescoring procedure.
“A floor effect was noted for age-corrected standard scoring of the measures, which was remedied through a rescoring procedure.”
From NIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome (2022) · cites this paper as Shields et al., 2020
Previous studies using the NIHTB-CB in ID have used a z-score deviation scoring approach.
“Alternatively, previous studies using the NIHTB-CB in ID have used a z-score deviation scoring approach (Hessl et al., 2009; Shields et al., 2020).”
From NIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome (2022) · cites this paper as Shields et al., 2020
Efforts to validate the NIHTB-CB in individuals with intellectual disability indicate good feasibility in the moderate ID range and a mental age above 5 years and convergent validity comparable to the original NIHTB-CB norming study.
“Efforts to validate the measure in individuals with ID indicate good feasibility in the moderate ID range and a mental age above 5 years (Shields et al., 2020) and convergent validity comparable to the original NIHTB-CB norming study (Hessl et al., 2016).”
From NIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome (2022) · cites this paper as Shields et al., 2020
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Citations
Cites
Cognition assessment using the NIH Toolbox
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Self-citationOutcome Measures for Clinical Trials in Down Syndrome
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Improving IQ measurement in intellectual disabilities using true deviation from population norms
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“The battery includes 7 tests: Dimensional Change Card Sort (DCCS), Flanker Inhibitory Control and Attention (Flanker), List Sorting Working Memory (LS), Pattern Comparison Processing Speed (PC), Picture Sequence Memory (PSM), Picture Vocabulary (PVT), and Oral Reading Recognition (OR).(9,15) The NIHTB-CB provided expe…”
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Cited by
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Self-citationNIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome
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Self-citation
Metadata
- Authors
- Shields, R. H., Kaat, A. J., McKenzie, F. J., Drayton, A., Sansone, S. M., Coleman, J., Michalak, C., Riley, K., Berry-Kravis, E., Gershon, R. C., Widaman, K. F., & Hessl, D.
- Affiliations
- University of California, Riverside (US)
- From the University of California Davis Medical Center (R.H.S.); Human Development Graduate Group (R.H.S.), University of California Davis, Sacramento; Feinberg School of Medicine (A.J.K., R.C.G.), Northwestern University, Chicago, IL; MIND Institute and Department of Psychiatry and Behavioral Sciences (F.J.M., A.D., S.M.S., D.H.), University of California Davis Medical Center, Sacramento; Morgridge College of Education (J.C., K.R.), University of Denver, CO; Departments of Pediatrics (C.M., E.B.-K....
- Corresponding author
- Hessl, David
- ORCID
- Venue
- Neurology
- Published
- 2020-03-24
- Volume
- 94
- Issue
- 12
- Pages
- e1229-e1240
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- PMC
- PMC7274932
- OpenAlex
- W3007688159
- Cited by
- 73
- References
- 6
- Citations by year
- 2026: 12 · 2025: 7 · 2024: 13 · 2023: 15 · 2022: 12 · 2021: 11 · 2020: 3
- FWCI
- 7.69
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- cc-by-nc-nd
- Locations
- Neurology (publishedVersion, cc-by-nc-nd)
- PubMed Central (publishedVersion, cc-by-nc-nd)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- eScholarship (California Digital Library) (submittedVersion, cc-by-nc-nd)
- Funders
- Rush University
- Ultragenyx Pharmaceutical
- Zynerba Pharmaceuticals
- National Institutes of Health (UL1TR000002, 90DD0596, TR000002)
- Intellectual and Developmental Disabilities Research Center (U54-HD079125)
- National Institute of Child Health and Human Development (HD079125)
- National Center for Advancing Translational Sciences (#TR 000002)
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (U54HD079125, P50HD103526, R01HD076189)
- Topics
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Down syndrome and intellectual disability research
- Social Sciences › Psychology › Neuropsychology and Physiological Psychology › Developmental and Educational Neuropsychology
- Social Sciences › Psychology › Experimental and Cognitive Psychology › Cognitive Abilities and Testing
- Keywords
- intellectual disability, test-retest reliability, convergent validity, fragile X syndrome, Down syndrome, executive function, processing speed, memory, language, adaptive behavior, known-groups validity, assessment accommodations, children and young adults
- MeSH terms
- Adolescent, Adult, Child, Child, Preschool, Female, Humans, Male, Intellectual Disability, National Institutes of Health (U.S.), Neuropsychological Tests, Psychometrics, United States, Reproducibility of Results, Young Adult
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