Improving IQ measurement in intellectual disabilities using true deviation from population norms
Open accessAbstract
BACKGROUND: Intellectual disability (ID) is characterized by global cognitive deficits, yet the very IQ tests used to assess ID have limited range and precision in this population, especially for more impaired individuals. METHODS: We describe the development and validation of a method of raw z-score transformation (based on general population norms) that ameliorates floor effects and improves the precision of IQ measurement in ID using the Stanford Binet 5 (SB5) in fragile X syndrome (FXS; n = 106), the leading inherited cause of ID, and in individuals with idiopathic autism spectrum disorder (ASD; n = 205). We compared the distributional characteristics and Q-Q plots from the standardized scores with the deviation z-scores. Additionally, we examined the relationship between both scoring methods and multiple criterion measures. RESULTS: We found evidence that substantial and meaningful variation in cognitive ability on standardized IQ tests among individuals with ID is lost when converting raw scores to standardized scaled, index and IQ scores. Use of the deviation z- score method rectifies this problem, and accounts for significant additional variance in criterion validation measures, above and beyond the usual IQ scores. Additionally, individual and group-level cognitive strengths and weaknesses are recovered using deviation scores. CONCLUSION: Traditional methods for generating IQ scores in lower functioning individuals with ID are inaccurate and inadequate, leading to erroneously flat profiles. However assessment of cognitive abilities is substantially improved by measuring true deviation in performance from standardization sample norms. This work has important implications for standardized test development, clinical assessment, and research for which IQ is an important measure of interest in individuals with neurodevelopmental disorders and other forms of cognitive impairment.
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
A particular challenge for measuring change in intellectual disability is the potential for floor effects, which may lead to a mischaracterization of a potentially meaningful change over time in individuals with intellectual disability.
“However, a particular challenge for measuring change in ID is the potential for floor effects (Hessl et al., 2009; Sansone et al., 2014), which may lead to a mischaracterization of a potentially meaningful change over time in individuals with ID (Bishop et al., 2015; Thurm et al., 2020).”
From NIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome (2022) · cites this paper as Sansone et al., 2014
Re-scoring of the Wechsler Intelligence Scale for Children and the Stanford-Binet for populations with intellectual disability has been completed and yields more sensitive estimates of true ability below the traditional test floor.
“re-scoring of the Wechsler Intelligence Scale for Children (WISC)92 and Stanford–Binet10 for populations with intellectual disability has been completed, yielding much more sensitive estimates of true ability below the traditional test floor.”
From Drug development for neurodevelopmental disorders: lessons learned from fragile X syndrome (2018) · cites this paper as [10]
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Cites
R: A Language and Environment for Statistical Computing
R Core Team (2000) · doi:10.32614/r.manuals · Cited by 355,706 · see citing works
Diagnostic and Statistical Manual of Mental Disorders
American Psychiatric Association (2013) · doi:10.1176/appi.books.9780890425596 · Cited by 116,369 · see citing works
Diagnostic and Statistical Manual of Mental Disorders
Williams, J. B. W., & First, M. B. (2013) · Oxford University Press eBooks · doi:10.1093/acrefore/9780199975839.013.104 · Cited by 109,841 · see citing works
Lord, C., Rutter, M., & Le Couteur, A. (1994) · Journal of Autism and Developmental Disorders · doi:10.1007/bf02172145 · Cited by 9,065 · see citing works
(1998) · Cited by 4,172 · see citing works
Peabody Picture Vocabulary Test
Campbell, J. M., & Dommestrup, A. K. (2010) · The Corsini Encyclopedia of Psychology · doi:10.1002/9780470479216.corpsy0649 · Cited by 3,787 · see citing works
Mullen Scales of Early Learning
Bradley‐Johnson, S. (1997) · Psychology in the Schools · doi:10.1002/(sici)1520-6807(199710)34:4 · Cited by 2,329 · see citing works
Epidemiological Surveys of Autism and Other Pervasive Developmental Disorders: An Update
Fombonne, E. (2003) · Journal of Autism and Developmental Disorders · doi:10.1023/a:1025054610557 · Cited by 1,505 · see citing works
Abnormal dendritic spines in fragile X knockout mice: Maturation and pruning deficits
Comery, T. A., Harris, J. B., Willems, P. J., Oostra, B. A., Irwin, S. A., Weiler, I. J., & Greenough, W. T. (1997) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.94.10.5401 · Cited by 1,046 · see citing works
Devys, D., Lutz, Y., Rouyer, N., Bellocq, J., & Mandel, J. (1993) · Nature Genetics · doi:10.1038/ng0893-335 · Cited by 781 · see citing works
Peabody Picture Vocabulary Test–Third Edition
Dumont, R., & Willis, J. O. (2008) · Encyclopedia of Special Education · doi:10.1002/9780470373699.speced1554 · Cited by 711 · see citing works
Dendritic Spine Structural Anomalies in Fragile-X Mental Retardation Syndrome
Irwin, S. A. (2000) · Cerebral Cortex · doi:10.1093/cercor/10.10.1038 · Cited by 626 · see citing works
The fragile X prevalence paradox
Hagerman, P. J. (2008) · Journal of Medical Genetics · doi:10.1136/jmg.2008.059055 · Cited by 343 · see citing works
Incremental Validity of New Clinical Assessment Measures.
Haynes, S. N., & Lench, H. C. (2003) · Psychological Assessment · doi:10.1037/1040-3590.15.4.456 · Cited by 324 · see citing works
Mullen Scales of Early Learning
Hutchins, T. L., Vivanti, G., Mateljevic, N., Jou, R. J., Shic, F., Cornew, L., Roberts, T. P. L., Oakes, L. A., Gray, S. A. O., Ray-Subramanian, C. E., et al. (2012) · Springer eBooks · doi:10.1007/978-1-4419-1698-3_596 · Cited by 323 · see citing works
Sleep in Children With Autism Spectrum Disorder
Kotagal, S., & Broomall, E. M. (2012) · Pediatric Neurology · doi:10.1016/j.pediatrneurol.2012.05.007 · Cited by 271 · see citing works
Peabody Picture Vocabulary Test
Henninger, N. (2018) · Springer eBooks · doi:10.1007/978-3-319-56782-2_1581-2 · Cited by 269 · see citing works
Psychometrics, intelligence, and public perception
Carroll, J. B. (1997) · Intelligence · doi:10.1016/s0160-2896(97)90012-x · Cited by 208 · see citing works
Current status of intensive behavioral interventions for young children with autism and PDD-NOS
Matson, J. L., & Smith, K. R. M. (2007) · Research in Autism · doi:10.1016/j.rasd.2007.03.003 · Cited by 203 · see citing works
Hessl, D., Nguyen, D. V., Green, C., Chavez, A., Tassone, F., Hagerman, R. J., Senturk, D., Schneider, A., Lightbody, A., Reiss, A. L., et al. (2009) · Journal of Neurodevelopmental Disorders · doi:10.1007/s11689-008-9001-8 · Cited by 182 · see citing works
Self-citationLewis, P. F., Abbeduto, L., Murphy, M. M., Richmond, E. K., Giles, N. D., Bruno, L., & Schroeder, S. (2006) · Journal of Intellectual Disability Research · doi:10.1111/j.1365-2788.2006.00803.x · Cited by 167 · see citing works
Molecular Predictors of Cognitive Involvement in Female Carriers of Fragile X Syndrome
Taylor, A. K. (1994) · JAMA · doi:10.1001/jama.1994.03510310037035 · Cited by 150 · see citing works
McGrew, K. S., Flanagan, D. P., Keith, T. Z., & Vanderwood, M. L. (1997) · School Psychology Review · doi:10.1080/02796015.1997.12085858 · Cited by 147 · see citing works
Cognitive, behavior and intervention outcome in young children with autism
Ben Itzchak, E., Lahat, E., Burgin, R., & Zachor, A. D. (2008) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2007.08.003 · Cited by 142 · cited 1× · see citing works
“Previously, researchers have used tests of cognitive functioning to predict the severity of autism symptoms and as an outcome measure of early intervention programs for ASD (for examples see [14,15]).”
BackgroundAutism Diagnostic Observation Schedule
Kim, S. H., & Lord, C. (2018) · Springer eBooks · doi:10.1007/978-3-319-57111-9_1520 · Cited by 124 · see citing works
Profiling Fragile X Syndrome in males: Strengths and weaknesses in cognitive abilities
van der Molen, M. J. W., Huizinga, M., Huizenga, H. M., Ridderinkhof, K. R., van der Molen, M. W., Hamel, B. J. C., Curfs, L., & Ramakers, G. J. A. (2009) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2009.10.013 · Cited by 80 · see citing works
Coolican, J., Bryson, S. E., & Zwaigenbaum, L. (2007) · Journal of Autism and Developmental Disorders · doi:10.1007/s10803-007-0368-2 · Cited by 67 · see citing works
Couzens, D., Cuskelly, M., & Jobling, A. (2004) · International Journal of Disability Development and Education · doi:10.1080/1034912042000182193 · Cited by 32 · see citing works
The Distribution of Scaled Scores and Possible Floor Effects on the WISC‐III and WAIS‐III
Whitaker, S., & Wood, C. (2007) · Journal of Applied Research in Intellectual Disabilities · doi:10.1111/j.1468-3148.2007.00378.x · Cited by 27 · see citing works
Whitaker, S., & Gordon, S. (2012) · International Journal of Developmental Disabilities · doi:10.1179/2047387711y.0000000012 · Cited by 25 · see citing works
Does IQ decline with age in fragile‐X? A methodological critique
Hay, D. A. (1994) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320510412 · Cited by 23 · see citing works
A cross-sectional test of the similar-trajectory hypothesis among adults with mental retardation
Façon, B. (2006) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2006.10.003 · Cited by 23 · see citing works
Lobar holoprosencephaly in an infant born to a mother with classic phenylketonuria
Keller, K. M., McCune, H., Williams, C. A., & Muenke, M. (2000) · American Journal of Medical Genetics · doi:10.1002/1096-8628(20001113)95:2 · Cited by 12 · see citing works
Cognitive‐behavioral profiles of females with the fragile X mutation
Fisch, G. S. (2006) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.31113 · Cited by 9 · see citing works
Flagged first, then by citations
Research works printed but not linked: 1
- Sparrow S Balla D Cicchetti D Vineland Adaptive Behavior Scales-Interview Edition 1984 Circle Pines, MN: American Guidance Service
Cited by
Sorted by citations
Hagerman, R. J., Berry-Kravis, E., Hazlett, H. C., Bailey, D. B., Moine, H., Kooy, R. F., Tassone, F., Gantois, I., Sonenberg, N., Mandel, J. L., et al. (2017) · Nature Reviews Disease Primers · doi:10.1038/nrdp.2017.65 · Cited by 838 · see citing works
Self-citationThe molecular biology of FMRP: new insights into fragile X syndrome
Richter, J. D., & Zhao, X. (2021) · Nature Reviews Neuroscience · doi:10.1038/s41583-021-00432-0 · Cited by 390 · see citing works
Drug development for neurodevelopmental disorders: lessons learned from fragile X syndrome
Berry-Kravis, E. M., Lindemann, L., Jønch, A. E., Apostol, G., Bear, M. F., Carpenter, R. L., Crawley, J. N., Curie, A., Des Portes, V., Hossain, F., et al. (2018) · Nature Reviews Drug Discovery · doi:10.1038/nrd.2017.221 · Cited by 336 · see citing works
Self-citationGenetic Testing in Neurodevelopmental Disorders
Savatt, J. M., & Myers, S. M. (2021) · Frontiers in Pediatrics · doi:10.3389/fped.2021.526779 · Cited by 230 · see citing works
Ethridge, L. E., White, S. P., Mosconi, M. W., Wang, J., Pedapati, E. V., Erickson, C. A., Byerly, M., & Sweeney, J. A. (2017) · Molecular Autism · doi:10.1186/s13229-017-0140-1 · Cited by 164 · see citing works
Updated report on tools to measure outcomes of clinical trials in fragile X syndrome
Budimirovic, D. B., Berry‐Kravis, E., Erickson, C. A., Hall, S. S., Hessl, D. R., Reiss, A. L., King, M. K., Abbeduto, L., & Kaufmann, W. E. (2017) · Journal of Neurodevelopmental Disorders · doi:10.1186/s11689-017-9193-x · Cited by 157 · see citing works
Self-citationHessl, D., Sansone, S. M., Berry-Kravis, E., Riley, K., Widaman, K. F., Abbeduto, L., Schneider, A., Coleman, J., Oaklander, D., Rhodes, K. C., et al. (2016) · Journal of Neurodevelopmental Disorders · doi:10.1186/s11689-016-9167-4 · Cited by 126 · see citing works
Self-citationBerry-Kravis, E. M., Harnett, M. D., Reines, S. A., Reese, M. A., Ethridge, L. E., Outterson, A. H., Michalak, C., Furman, J., & Gurney, M. E. (2021) · Nature Medicine · doi:10.1038/s41591-021-01321-w · Cited by 131 · see citing works
Self-citationAuditory EEG Biomarkers in Fragile X Syndrome: Clinical Relevance
Ethridge, L. E., Stefano, L. A. D., Schmitt, L., Woodruff, N., Brown, K. L., Tran, M., Wang, J., Pedapati, E. V., Erickson, C. A., & Sweeney, J. A. (2019) · Frontiers in Integrative Neuroscience · doi:10.3389/fnint.2019.00060 · Cited by 114 · see citing works
Budimirovic, D. B., Schlageter, A. M., Filipovic-Sadic, S., Protic, D. D., Bram, E. E., Mahone, E. M., Nicholson, K., Culp, K., Javanmardi, K., Kemppainen, J., et al. (2020) · Brain Sciences · doi:10.3390/brainsci10100694 · Cited by 80 · see citing works
Self-citationFarmer, C., Golden, C., & Thurm, A. E. (2015) · Child Neuropsychology · doi:10.1080/09297049.2015.1020775 · Cited by 77 · see citing works
Validation of the NIH Toolbox Cognitive Battery in intellectual disability
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., et al. (2020) · Neurology · doi:10.1212/wnl.0000000000009131 · Cited by 73 · see citing works
Self-citationAssociation between IQ and FMR1 protein (FMRP) across the spectrum of CGG repeat expansions
Kim, K., Hessl, D. R., Randol, J. L., Espinal, G. M., Schneider, A., Protic, D. D., Aydin, E. Y., Hagerman, R. J., & Hagerman, P. J. (2019) · PLoS ONE · doi:10.1371/journal.pone.0226811 · Cited by 75 · see citing works
Self-citationCharacterising repetitive behaviours in young boys with fragile X syndrome
Oakes, A., Thurman, A. J., McDuffie, A. S., Bullard, L., Hagerman, R. J., & Abbeduto, L. (2015) · Journal of Intellectual Disability Research · doi:10.1111/jir.12234 · Cited by 71 · see citing works
Executive Function in Fragile X Syndrome: A Systematic Review
Schmitt, L., Shaffer, R. C., Hessl, D. R., & Erickson, C. A. (2019) · Brain Sciences · doi:10.3390/brainsci9010015 · Cited by 64 · see citing works
Self-citationAutism Spectrum Disorder and IQ – A Complex Interplay
Wolff, N., Stroth, S., Kamp‐Becker, I., Roepke, S., & Roessner, V. (2022) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2022.856084 · Cited by 64 · see citing works
Assessing general cognitive and adaptive abilities in adults with Down syndrome: a systematic review
Hamburg, S., Lowe, B., Startin, C. M., Padilla, C., Coppus, A. M. W., Silverman, W. P., Fortea, J., Zaman, S. H., Head, E., Handen, B. L., et al. (2019) · Journal of Neurodevelopmental Disorders · doi:10.1186/s11689-019-9279-8 · Cited by 58 · see citing works
Developmental Outcomes Among Young Children With Congenital Zika Syndrome in Brazil
Wheeler, A., Toth, D., Ridenour, T. A., Nóbrega, L., Firmino, R. C. B., da Silva, C. M., Carvalho, P., Marques, D., Okoniewski, K. C., Ventura, L. O., et al. (2020) · JAMA Network Open · doi:10.1001/jamanetworkopen.2020.4096 · Cited by 57 · see citing works
Pedapati, E. V., Schmitt, L., Ethridge, L. E., Miyakoshi, M., Sweeney, J. A., Liu, R., Smith, E. G., Shaffer, R. C., Dominick, K. C., Gilbert, D. L., et al. (2022) · Communications Biology · doi:10.1038/s42003-022-03395-9 · Cited by 54 · see citing works
Palmer, E. E., Howell, K., & Scheffer, I. E. (2021) · Neurotherapeutics · doi:10.1007/s13311-021-01133-3 · Cited by 53 · see citing works
Sex differences in resting EEG power in Fragile X Syndrome
Smith, E. G., Pedapati, E. V., Liu, R., Schmitt, L., Dominick, K. C., Shaffer, R. C., Sweeney, J. A., & Erickson, C. A. (2021) · Journal of Psychiatric Research · doi:10.1016/j.jpsychires.2021.03.057 · Cited by 52 · see citing works
Abbeduto, L., Berry‐Kravis, E., Sterling, A. M., Sherman, S. L., Edgin, J. O., McDuffie, A. S., Hoffmann, A., Hamilton, D., Nelson, M. N., Aschkenasy, J. R., et al. (2020) · Journal of Neurodevelopmental Disorders · doi:10.1186/s11689-020-09313-6 · Cited by 51 · see citing works
Self-citationRaspa, M., Bann, C., Gwaltney, A. Y., Benke, T. A., Fu, C., Glaze, D. G., Haas, R., Heydemann, P. T., Jones, M. D., Kaufmann, W. E., et al. (2020) · American Journal on Intellectual and Developmental Disabilities · doi:10.1352/1944-7558-125.6.493 · Cited by 44 · see citing works
Vacca, R. A., Augello, A., Gallo, L., Caggianese, G., Malizia, V., Grutta, S. L., Murero, M., Valenti, D., Tullo, A., Balech, B., et al. (2023) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2023.105156 · Cited by 44 · see citing works
Response to Placebo in Fragile X Syndrome Clinical Trials: An Initial Analysis
Luu, S., Province, H. S., Berry‐Kravis, E., Hagerman, R. J., Hessl, D. R., Vaidya, D. M., Lozano, R., Rosselot, H., Erickson, C. A., Kaufmann, W. E., et al. (2020) · Brain Sciences · doi:10.3390/brainsci10090629 · Cited by 40 · see citing works
Self-citationThe Down Syndrome Cognition Project, Channell, M. M., Mattie, L. J., Hamilton, D. R., Capone, G. T., Mahone, E. M., Sherman, S. L., Rosser, T. C., Reeves, R. H., & Kalb, L. G. (2021) · Journal of Neurodevelopmental Disorders · doi:10.1186/s11689-021-09365-2 · Cited by 41 · see citing works
Standardized Assessment Accommodations for Individuals with Intellectual Disability
Thompson, T., Coleman, J., Riley, K., Snider, L. A., Howard, L. J., Sansone, S. M., & Hessl, D. R. (2018) · Contemporary School Psychology · doi:10.1007/s40688-018-0171-4 · Cited by 37 · see citing works
Self-citationMeng, L. M., Kaufmann, W. E., Frye, R. E., Ong, K. S., Kaminski, J. W., Velinov, M. T., & Berry‐Kravis, E. (2021) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.62594 · Cited by 37 · see citing works
Self-citationCharacteristics Associated with Autism Spectrum Disorder Risk in Individuals with Down Syndrome
The Down Syndrome Cognition Project, Channell, M. M., Hahn, L. J., Rosser, T. C., Hamilton, D., Frank‐Crawford, M. A., Capone, G. T., & Sherman, S. L. (2019) · Journal of Autism and Developmental Disorders · doi:10.1007/s10803-019-04074-1 · Cited by 36 · see citing works
DiStefano, C., Sadhwani, A., & Wheeler, A. (2020) · American Journal on Intellectual and Developmental Disabilities · doi:10.1352/1944-7558-125.6.434 · Cited by 35 · see citing works
Clinical Development of Targeted Fragile X Syndrome Treatments: An Industry Perspective
Lee, A. W., Ventola, P. E., Budimirovic, D. B., Berry‐Kravis, E., & Visootsak, J. (2018) · Brain Sciences · doi:10.3390/brainsci8120214 · Cited by 33 · see citing works
Self-citationThurman, A. J., Edgin, J. O., Sherman, S. L., Sterling, A. M., McDuffie, A. S., Berry‐Kravis, E., Hamilton, D., & Abbeduto, L. (2021) · Journal of Neurodevelopmental Disorders · doi:10.1186/s11689-021-09361-6 · Cited by 33 · see citing works
Self-citationFarmer, C., Thurm, A. E., Troy, J. D., & Kaat, A. J. (2023) · Journal of Neurodevelopmental Disorders · doi:10.1186/s11689-022-09474-6 · Cited by 32 · see citing works
(2017) · The American Pharmacists Association eBooks · doi:10.21019/9781582122885.nationalclinicalguidelines · Cited by 32 · see citing works
Outcome Measures for Core Symptoms of Intellectual Disability: State of the Field
Thurm, A. E., Kelleher, B., & Wheeler, A. (2020) · American Journal on Intellectual and Developmental Disabilities · doi:10.1352/1944-7558-125.6.418 · Cited by 32 · see citing works
A neurophysiological model of speech production deficits in fragile X syndrome
Schmitt, L. M., Wang, J., Pedapati, E., Thurman, A., Abbeduto, L., Erickson, C., & Sweeney, J. (2019) · Brain Communications · doi:10.1093/braincomms/fcz042 · Cited by 28 · see citing works
Boggs, A. E., Schmitt, L., McLane, R. D., Adayev, T., LaFauci, G., Horn, P. S., Dominick, K. C., Groß, C., & Erickson, C. A. (2022) · Scientific Reports · doi:10.1038/s41598-022-09633-8 · Cited by 31 · see citing works
Shaffer, R. C., Schmitt, L., Thurman, A. J., Abbeduto, L., Hong, M. P., Pedapati, E. V., Dominick, K. C., Sweeney, J. A., & Erickson, C. A. (2020) · Brain Sciences · doi:10.3390/brainsci10020066 · Cited by 30 · see citing works
Quintin, E., Jo, B., Hall, S. S., Bruno, J. L., Chromik, L. C., Raman, M. M., Lightbody, A. A., Martin, A., & Reiss, A. L. (2015) · Development and Psychopathology · doi:10.1017/s0954579415001200 · Cited by 30 · see citing works
Comparison of Measures of Ability in Adolescents with Intellectual Disability
Mungkhetklang, C., Crewther, S. G., Bavin, E. L., Goharpey, N., & Parsons, C. (2016) · Frontiers in Psychology · doi:10.3389/fpsyg.2016.00683 · Cited by 28 · see citing works
A normative chart for cognitive development in a genetically selected population
Fiksinski, A., Bearden, C. E., Bassett, A. S., Kahn, R. S., Zinkstok, J., Hooper, S. R., Tempelaar, W. M., McDonald‐McGinn, D. M., Swillen, A., Emanuel, B. S., et al. (2021) · Neuropsychopharmacology · doi:10.1038/s41386-021-00988-6 · Cited by 27 · see citing works
Schworer, E., Hoffman, E., & Esbensen, A. (2021) · Brain Sciences · doi:10.3390/brainsci11070836 · Cited by 24 · see citing works
Development of Neural Response to Novel Sounds in Fragile X Syndrome: Potential Biomarkers
Ethridge, L. E., Thaliath, A. P., Kraff, J., Nijhawan, K., & Berry‐Kravis, E. (2020) · American Journal on Intellectual and Developmental Disabilities · doi:10.1352/1944-7558-125.6.449 · Cited by 24 · see citing works
Self-citationSymptoms of Autism Spectrum Disorder in Individuals with Down Syndrome
Nunnally, A. D., Nguyen, V. H., Anglo, C., Sterling, A. M., Edgin, J. O., Sherman, S. L., Berry‐Kravis, E., Soriano, L. D. H., Abbeduto, L., & Thurman, A. J. (2021) · Brain Sciences · doi:10.3390/brainsci11101278 · Cited by 24 · see citing works
Self-citationDelineating Repetitive Behavior Profiles across the Lifespan in Fragile X Syndrome
Reisinger, D. L., Shaffer, R. C., Tartaglia, N. R., Berry‐Kravis, E., & Erickson, C. A. (2020) · Brain Sciences · doi:10.3390/brainsci10040239 · Cited by 23 · see citing works
Self-citationRahmatullah, N., Schmitt, L., Stefano, L. A. D., Post, S., Robledo, J., Chaudhari, G., Pedapati, E. V., Erickson, C. A., Portera‐Cailliau, C., & Goel, A. (2023) · Journal of Neuroscience · doi:10.1523/jneurosci.0571-23.2023 · Cited by 22 · see citing works
Gaboxadol in Fragile X Syndrome: A 12-Week Randomized, Double-Blind, Parallel-Group, Phase 2a Study
Budimirovic, D. B., Dominick, K. C., Gabis, L. V., Adams, M., Adera, M., Huang, L., Ventola, P. E., Tartaglia, N. R., & Berry‐Kravis, E. (2021) · Frontiers in Pharmacology · doi:10.3389/fphar.2021.757825 · Cited by 21 · see citing works
Self-citationAltered frontal connectivity as a mechanism for executive function deficits in fragile X syndrome
Schmitt, L., Li, J., Liu, R., Horn, P. S., Sweeney, J. A., Erickson, C. A., & Pedapati, E. V. (2022) · Molecular Autism · doi:10.1186/s13229-022-00527-0 · Cited by 21 · see citing works
Cognitive profiles in individuals with Down syndrome
Pulina, F., Vianello, R., & Lanfranchi, S. (2019) · International review of research in developmental disabilities · doi:10.1016/bs.irrdd.2019.06.002 · Cited by 21 · see citing works
Norris, J. E., DeStefano, L. A., Schmitt, L., Pedapati, E. V., Erickson, C. A., Sweeney, J. A., & Ethridge, L. E. (2022) · ACS Chemical Neuroscience · doi:10.1021/acschemneuro.2c00404 · Cited by 19 · see citing works
Arpone, M., Baker, E. K., Bretherton, L., Bui, M., Li, X., Whitaker, S., Dissanayake, C., Cohen, J., Hickerton, C. L., Rogers, C., et al. (2018) · Scientific Reports · doi:10.1038/s41598-018-21990-x · Cited by 19 · see citing works
Berry‐Kravis, E. (2022) · Journal of Child Neurology · doi:10.1177/08830738221089740 · Cited by 18 · see citing works
Self-citationDakopolos, A., Condy, E. E., Smith, E. G., Harvey, D. J., Kaat, A. J., Coleman, J., Riley, K., Berry‐Kravis, E., & Hessl, D. R. (2024) · Journal of Neurodevelopmental Disorders · doi:10.1186/s11689-024-09542-z · Cited by 18 · see citing works
Self-citationSchworer, E. K., Ahmed, A. T., Hogenkamp, L., Moore, S., & Esbensen, A. J. (2022) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2022.104236 · Cited by 17 · see citing works
Shields, R. H., Kaat, A. J., Sansone, S., Michalak, C., Coleman, J., Thompson, T., McKenzie, F. J., Dakopolos, A., Riley, K., Berry‐Kravis, E., et al. (2022) · Neurology · doi:10.1212/wnl.0000000000201528 · Cited by 17 · see citing works
Self-citationSchworer, E. K., Soltani, A., Altaye, M., Fidler, D. J., & Esbensen, A. J. (2023) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2022.104416 · Cited by 17 · see citing works
Schmitt, L., Arzuaga, A. L., Dapore, A. E., Duncan, J., Patel, M., Larson, J. R., Erickson, C. A., Sweeney, J. A., & Ragozzino, M. E. (2023) · Frontiers in Behavioral Neuroscience · doi:10.3389/fnbeh.2022.1074682 · Cited by 18 · see citing works
Hoffmann, A., Wang, A. H., Berger, N. I., Cordeiro, L., Shaffer, R. C., Tartaglia, N. R., Erickson, C. A., & Berry‐Kravis, E. (2020) · Brain Sciences · doi:10.3390/brainsci10040212 · Cited by 17 · see citing works
Self-citationMccormack, L. A., Wylie, A. C., Moultrie, R., Furberg, R., Wheeler, A., Treiman, K., Bailey, D. B., & Raspa, M. (2019) · PLoS ONE · doi:10.1371/journal.pone.0223801 · Cited by 16 · see citing works
Soriano, L. D. H., Villarreal, J. C., Sterling, A. M., Edgin, J. O., Berry‐Kravis, E., Hamilton, D. R., Thurman, A. J., & Abbeduto, L. (2022) · Scientific Reports · doi:10.1038/s41598-022-24478-x · Cited by 15 · see citing works
Self-citationNear‐point Findings in Children with Autism Spectrum Disorder and in Typical Peers
Coulter, R. A., Bade, A., Jenewein, E. C., Tea, Y. C., & Mitchell, G. L. (2021) · Optometry and Vision Science · doi:10.1097/opx.0000000000001679 · Cited by 13 · see citing works
Schworer, E. K., Voth, K., Hoffman, E. K., & Esbensen, A. J. (2021) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2021.104147 · Cited by 13 · see citing works
Farmer, C., Kaat, A. J., Berry‐Kravis, E., & Thurm, A. E. (2022) · International review of research in developmental disabilities · doi:10.1016/bs.irrdd.2022.05.001 · Cited by 13 · see citing works
Self-citationStraub, D., Schmitt, L., Boggs, A. E., Horn, P. S., Dominick, K. C., Groß, C., & Erickson, C. A. (2023) · Scientific Reports · doi:10.1038/s41598-023-29786-4 · Cited by 13 · see citing works
A near normal distribution of IQ in Fragile X Syndrome
Schmitt, L., Nelson, M. N., Shaffer, R. C., & Erickson, C. A. (2024) · Scientific Reports · doi:10.1038/s41598-024-73626-y · Cited by 12 · see citing works
Cordeiro, L., Braden, M. L., Coan, E., Welnick, N., Tanda, T., & Tartaglia, N. R. (2020) · Brain Sciences · doi:10.3390/brainsci10040248 · Cited by 12 · see citing works
Cordeiro, L., Villagomez, A. N., Swain, D. M., Deklotz, S., & Tartaglia, N. R. (2020) · Brain Sciences · doi:10.3390/brainsci10060351 · Cited by 12 · see citing works
Decisional Capacity for Informed Consent in Males and Females with Fragile X Syndrome
Wheeler, A., Wylie, A. C., Raspa, M., Villagomez, A. N., Miller, K. M., Edwards, A., DeRamus, M., Appelbaum, P. S., & Bailey, D. B. (2019) · Journal of Autism and Developmental Disorders · doi:10.1007/s10803-019-03930-4 · Cited by 11 · see citing works
Soltani, A., Schworer, E., Amin, R., Hoffman, E. K., & Esbensen, A. (2023) · Journal of Developmental and Behavioral Pediatrics · doi:10.1097/dbp.0000000000001189 · Cited by 7 · see citing works
Psychometric evaluation of a working memory assessment measure in young children with Down syndrome
Pinks, M. E., Deusen, K. V., Prince, M. A., Esbensen, A. J., Thurman, A. J., Patel, L., Abbeduto, L., Walsh, M. M., Daunhauer, L. A., Feigles, R. T., et al. (2023) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2023.104564 · Cited by 10 · see citing works
Vocabulary comprehension in adults with fragile X syndrome (FXS)
Hoffmann, A., Krause, S. E., Wuu, J., Leurgans, S. L., Guter, S. J., Block, S. S., Salt, J., Cook, E. H., Maino, D. M., & Berry‐Kravis, E. (2019) · Journal of Neurodevelopmental Disorders · doi:10.1186/s11689-019-9285-x · Cited by 10 · see citing works
Self-citationExecutive functioning and verbal fluency performance in youth with Down syndrome
Soltani, A., Schworer, E. K., & Esbensen, A. J. (2022) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2022.104358 · Cited by 9 · see citing works
Startin, C. M., Hamburg, S., & Strydom, A. (2019) · Frontiers in Psychology · doi:10.3389/fpsyg.2018.02730 · Cited by 9 · see citing works
Prerequisite skills in cognitive testing: Innovations in theory and recommendations for practice
Kaat, A. J., Bishop, S. L., Condy, E. E., Sullivan, N. R., Soorya, L. V., & Thurm, A. E. (2021) · Cognitive Development · doi:10.1016/j.cogdev.2021.101038 · Cited by 9 · see citing works
Esbensen, A. J., Schworer, E. K., Fidler, D. J., & Thurman, A. J. (2022) · International review of research in developmental disabilities · doi:10.1016/bs.irrdd.2022.05.005 · Cited by 9 · see citing works
Profiles of Caregiver-Reported Executive Function in Children with Down Syndrome
Deusen, K. V., Prince, M. A., Esbensen, A. J., Edgin, J. O., Schworer, E. K., Thurman, A. J., Patel, L., Daunhauer, L. A., & Fidler, D. J. (2022) · Brain Sciences · doi:10.3390/brainsci12101333 · Cited by 9 · see citing works
Pedapati, E. V., Ethridge, L. E., Liu, Y., Liu, R., Sweeney, J. A., DeStefano, L. A., Miyakoshi, M., Razak, K. A., Schmitt, L., Moore, D. R., et al. (2025) · PLoS ONE · doi:10.1371/journal.pone.0306157 · Cited by 8 · see citing works
Abbeduto, L., Soriano, L. D. H., Berry‐Kravis, E., Sterling, A. M., Edgin, J. O., Abdelnur, N., Drayton, A., Hoffmann, A., Hamilton, D., Harvey, D. J., et al. (2023) · Scientific Reports · doi:10.1038/s41598-023-36087-3 · Cited by 8 · see citing works
Self-citationAbbeduto, L., Klusek, J., Taylor, J. L., Abdelnur, N., Sparapani, N., & Thurman, A. J. (2021) · Brain Sciences · doi:10.3390/brainsci11091179 · Cited by 8 · see citing works
Neuropsychological and ASD phenotypes in rare genetic syndromes: A critical review of the literature
Bush, L. C., & Scott, M. (2021) · The Clinical Neuropsychologist · doi:10.1080/13854046.2021.1980111 · Cited by 8 · see citing works
Schworer, E. K., Altaye, M., Fidler, D. J., Beebe, D. W., Wiley, S., Hoffman, E. K., & Esbensen, A. J. (2023) · International Journal of Environmental Research and Public Health · doi:10.3390/ijerph20065202 · Cited by 8 · see citing works
EEG Microstates as Markers for Cognitive Impairments in Fragile X Syndrome
Takarae, Y., Zanesco, A. P., Erickson, C. A., & Pedapati, E. V. (2023) · Brain Topography · doi:10.1007/s10548-023-01009-z · Cited by 8 · see citing works
Inclusion of Individuals With Neurodevelopmental Disorders in Norm-Referenced Language Assessments
Loveall, S. J., Channell, M. M., Mattie, L. J., & Barkhimer, A. E. (2022) · Frontiers in Psychology · doi:10.3389/fpsyg.2022.929433 · Cited by 7 · see citing works
Schworer, E. K., Belizaire, S., Hoffman, E. K., & Esbensen, A. J. (2021) · Brain Sciences · doi:10.3390/brainsci12010009 · Cited by 7 · see citing works
Soltani, A., Schworer, E. K., & Esbensen, A. J. (2022) · American Journal on Intellectual and Developmental Disabilities · doi:10.1352/1944-7558-128.1.82 · Cited by 7 · see citing works
Psychometric properties of inhibitory control measures among youth with Down syndrome
Soltani, A., Schworer, E. K., Altaye, M., Fidler, D. J., Beebe, D. W., Wiley, S., Hoffman, E. K., Voth, K., & Esbensen, A. J. (2023) · Journal of Intellectual Disability Research · doi:10.1111/jir.13043 · Cited by 7 · see citing works
Protic, D. D., Breeze, E., Mendoza‐Morales, G., Zafarullah, M., Abbeduto, L., Hagerman, R. J., Coffey, C. S., Cudkowicz, M., Durbin-Johnson, B. P., Ashwood, P., et al. (2024) · SAGE Open Medicine · doi:10.1177/20503121241282401 · Cited by 7 · see citing works
Self-citationHoffmann, A., Thurman, A. J., Sterling, A. M., Kover, S. T., Finestack, L. H., Berry‐Kravis, E., Edgin, J. O., Drayton, A., Fombonne, E. J., & Abbeduto, L. (2022) · Brain Sciences · doi:10.3390/brainsci12050575 · Cited by 7 · see citing works
Self-citationNIH Toolbox Cognition Battery Feasibility in Individuals With Williams Syndrome
Condy, E. E., Becker, L., Farmer, C., Kaat, A. J., Chlebowski, C., Kozel, B. A., & Thurm, A. (2022) · American Journal on Intellectual and Developmental Disabilities · doi:10.1352/1944-7558-127.6.473 · Cited by 6 · see citing works
Hyper-extralemniscal model of Fragile X syndrome
Miyakoshi, M., Kim, H., Stefano, L. A. D., Schmitt, L., Norris, J. E., Ethridge, L. E., Erickson, C. A., & Pedapati, E. V. (2025) · Cerebral Cortex · doi:10.1093/cercor/bhaf141 · Cited by 6 · see citing works
Shishido, Y., Mahone, E. M., & Jacobson, L. A. (2020) · Journal of the International Neuropsychological Society · doi:10.1017/s1355617720000636 · Cited by 6 · see citing works
Examining the validity of the use of ratio IQs in psychological assessments
Ostrolenk, A., & Courchesne, V. (2023) · Acta Psychologica · doi:10.1016/j.actpsy.2023.104054 · Cited by 5 · see citing works
Farmer, C., Thurm, A., Das, T., Bebin, E. M., Bernstein, J. A., Berry‐Kravis, E., Buxbaum, J. D., Eng, C., Frazier, T. W., Hardan, A. Y., et al. (2025) · American Journal on Intellectual and Developmental Disabilities · doi:10.1352/1944-7558-130.5.344 · Cited by 5 · see citing works
Self-citationA cross-comparison of cognitive ability across 8 genomic disorders
Mortillo, M., & Mullé, J. G. (2021) · Current Opinion in Genetics & Development · doi:10.1016/j.gde.2021.04.001 · Cited by 6 · see citing works
Associations Among Sex, Cognitive Ability, and Autism Symptoms in Individuals with Down Syndrome
Soriano, L. D. H., Sterling, A. M., Edgin, J. O., Hamilton, D. R., Berry‐Kravis, E., Nunnally, A. D., Thurman, A. J., & Abbeduto, L. (2022) · Journal of Autism and Developmental Disorders · doi:10.1007/s10803-022-05779-6 · Cited by 5 · see citing works
Self-citationElmaghraby, R., Liu, Y., Ramesh, P., Westerkamp, G. C., Nelson, M. A., Larsh, T. R., Smith, E. G., Gilbert, D. L., Wu, S. W., Erickson, C. A., et al. (2025) · Molecular Autism · doi:10.1186/s13229-025-00682-0 · Cited by 4 · see citing works
P < .05 is in the Eye of the Beholder: A Response to Beaujean and Farmer (2020)
Yi, Z., Schreiber, J. B., Paliliunas, D., Barron, B. F., & Dixon, M. R. (2021) · Journal of Behavioral Education · doi:10.1007/s10864-021-09435-4
Figueroa, M., & Darbra, S. (2024) · Journal of Applied Research in Intellectual Disabilities · doi:10.1111/jar.13330 · Cited by 4 · see citing works
Cognitive trajectories in rare neurogenetic diseases: minding the gaps and filling the potholes
Berry‐Kravis, E. (2016) · Developmental Medicine & Child Neurology · doi:10.1111/dmcn.13018 · Cited by 4 · see citing works
Self-citationSchworer, E. K., & Esbensen, A. J. (2023) · International Journal of Developmental Disabilities · doi:10.1080/20473869.2023.2230409 · Cited by 3 · see citing works
- Authors
- Sansone, S. M., Schneider, A., Bickel, E., Berry-Kravis, E., Prescott, C., & Hessl, D.
- Affiliations
- University of California Davis Medical Center (US)
- Rush University Medical Center (US)
- Corresponding author
- Hessl, David
- Venue
- Journal of Neurodevelopmental Disorders
- Published
- 2014-07-08
- Volume
- 6
- Issue
- 1
- Pages
- 16-16
- ISSN
- 1866-1947 (ISSN-L)
- 1866-1955
- PMC
- PMC4613563
- OpenAlex
- W2122684341
- Cited by
- 148
- References
- 34
- Citations by year
- 2026: 5 · 2025: 15 · 2024: 14 · 2023: 19 · 2022: 26 · 2021: 24 · 2020: 17 · 2019: 13 · 2018: 3 · 2017: 6 · 2016: 3 · 2015: 3
- FWCI
- 3.98
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- cc-by
- Locations
- Journal of Neurodevelopmental Disorders (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- eScholarship (California Digital Library) (submittedVersion, cc-by)
- Funders
- Autism Speaks
- National Fragile X Foundation
- University of California, Davis
- National Institute of Mental Health (U24MH081810)
- University of California Davis School of Medicine
- Topics
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Down syndrome and intellectual disability research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Social Sciences › Psychology › Experimental and Cognitive Psychology › Cognitive Abilities and Testing
- Keywords
- intellectual disability, IQ assessment, floor effect, fragile X syndrome, autism spectrum disorder, neurodevelopmental disorders
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
