Autistic behavior in young boys with fragile X syndrome

Bailey, Donald B.; Mesibov, Gary B.; Hatton, Deborah D.; Clark, Renee D.; Roberts, Jane E.; Mayhew, Lisa

doi:10.1023/a:1026048027397

Cited by 260Genetics and Neurodevelopmental DisordersAutism Spectrum Disorder ResearchWilliams Syndrome Research

Autistic behavior in young boys with fragile X syndrome

Bailey, Donald B., Mesibov, Gary B., Hatton, Deborah D., Clark, Renee D., Roberts, Jane E., Mayhew, Lisa·1998·Journal of Autism and Developmental Disorders

DOI 10.1023/a:1026048027397

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Abstract

A sample of 57 boys with fragile X syndrome (fraX) between the ages of 24 and 133 months was rated using the Childhood Autism Rating Scale (CARS) to assess the extent to which autism and autistic features were evident in a young population. Fourteen subjects (approximately 25% of the sample) scored above the cutoff for autism, suggesting a relatively high incidence of autistic behavior. All but 2 of these 14 were in the mildly or moderately autistic range, however, and only a few items received severe ratings, suggesting that severe autism is relatively rare in fraX, at least during the early years. The CARS resulted in a continuum of autistic ratings in the fraX population, but no particular items on the CARS contributed disproportionately to autism ratings. A visual comparison of ratings on an autistic, non-fraX sample revealed similar profiles of ratings, suggesting that differentiating fraX and autism on the basis of CARS ratings is not likely. Within the fraX group, chronological age and socioeconomic status did not correlate with CARS ratings, but severity of delay was strongly related, such that more severely delayed children scored higher (more autistic) on the CARS.

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Fragile X Syndrome results from expansion and hyper-methylation of CGG trinucleotide repeats in the FMR1 gene, leading to reduced FMRP.
“FXS results from expansion and hyper-methylation of CGG trinucleotide repeats in the un-translated 5’ region of the Fragile X Mental Retardation (FMR1) gene, leading to reduced FMR1 protein (FMRP) (Bailey et al., 1998; O'Donnell and Warren, 2002).”
From Matrix metalloproteinase-9 deletion rescues auditory evoked potential habituation deficit in a mouse model of Fragile X Syndrome (2016) · cites this paper as Bailey et al., 1998

Toward objective classification of childhood autism: Childhood Autism Rating Scale (CARS)
Schopler, E., Reichler, R. J., DeVellis, R. F., & Daly, K. (1980) · Journal of Autism and Developmental Disorders · doi:10.1007/bf02408436 · Cited by 2,432 · cited 1× · see citing works
“A number of research studies have demonstrated the reliability and usefulness of the CARS in identifying children with autism (Eaves & Milner, 1993; Garfin, McCallon, & Cox, 1988; Kurita, Miyake, & Katsuno, 1989; Mesibov, Schopler, Schaffer, & Michal, 1989; Schopler, Reichler, DeVellis, & Daly, 1980; Sevin, Matson, Co…”
Specification of the Neurobehavioral Phenotype in Males with Fragile X Syndrome
Baumgardner, T. L., Reiss, A. L., Freund, L. S., & Abrams, M. T. (1995) · PEDIATRICS · doi:10.1542/peds.95.5.744 · Cited by 245 · see citing works
An analysis of autism in fifty males with the fragile X syndrome
Hagerman, R. J., Jackson, A. W., Levitas, A., Rimland, B., Braden, M., Opitz, J. M., & Reynolds, J. F. (1986) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320230128 · Cited by 238 · see citing works
Autism is associated with the fragile-X syndrome
Brown, W. T., Jenkins, E. C., Friedman, E., Brooks, J., Wisniewski, K. E., Raguthu, S., & French, J. H. (1982) · Journal of Autism and Developmental Disorders · doi:10.1007/bf01531375 · Cited by 224 · see citing works
Fragile X and autism: A multicenter survey
Brown, W. T., Jenkins, E. C., Cohen, I. L., Fisch, G. S., Wolf‐Schein, E. G., Gross, A. C., Waterhouse, L. H., Fein, D., MASON-BROTHERS, A., RITVO, E. R., et al. (1986) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320230126 · Cited by 180 · see citing works
Use of the Childhood Autism Rating Scale with Autistic Adolescents and Adults
Mesibov, G. B., Schopler, E., Schaffer, B., & MICHAL, N. (1989) · Journal of the American Academy of Child & Adolescent Psychiatry · doi:10.1097/00004583-198907000-00012 · Cited by 164 · see citing works
Self-citation
Conversational analyses of males with fragile X, Down syndrome, and autism: comparison of the emergence of deviant language.
Sudhalter, V., Cohen, I. L., Silverman, W. P., & Wolf‐Schein, E. G. (1990) · PubMed · Cited by 158 · see citing works
Behavioral phenotype of fragile X syndrome: DSM‐III‐R autistic behavior in male children
Reiss, A. L., & Freund, L. S. (1992) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320430106 · Cited by 138 · see citing works
A comparison and evaluation of three commonly used autism scales
Sevin, J. A., Matson, J. L., Coe, D. A., Fee, V. E., & Sevin, B. M. (1991) · Journal of Autism and Developmental Disorders · doi:10.1007/bf02206868 · Cited by 123 · see citing works
Why are autism and the fragile-X syndrome associated? Conceptual and methodological issues.
Cohen, I. L., Sudhalter, V., Pfadt, A., et al. (1991) · PubMed · Cited by 120 · see citing works
The criterion-related validity of the Childhood Autism Rating Scale and the Autism Behavior checklist
Eaves, R. C., & Milner, B. (1993) · Research on Child and Adolescent Psychopathology · doi:10.1007/bf00916315 · Cited by 119 · see citing works
Validity and reliability of the Childhood Autism Rating Scale with autistic adolescents
Garfin, D. G., & McCallon, D. (1988) · Journal of Autism and Developmental Disorders · doi:10.1007/bf02212193 · Cited by 112 · see citing works
Autism and the Fragile X Syndrome
Levitas, A. S., Hagerman, R. J., Braden, M. L., Rimland, B., McBogg, P., & Matus, I. (1983) · Journal of Developmental & Behavioral Pediatrics · doi:10.1097/00004703-198309000-00002 · Cited by 101 · see citing works
A validity analysis of selected instruments used to assess autism
Teal, M. B., & Wiebe, M. J. (1986) · Journal of Autism and Developmental Disorders · doi:10.1007/bf01531713 · Cited by 97 · see citing works
Reliability and validity of the Childhood Autism Rating Scale-Tokyo version (CARS-TV)
Kurita, H., Miyake, Y., & Katsuno, K. (1989) · Journal of Autism and Developmental Disorders · doi:10.1007/bf02212937 · Cited by 96 · see citing works
Fragile (X) syndrome: a study of the psychological profile in 23 prepubertal patients
Borghgraef, M., Fryns, J. P., Dlelkens, A., Pyck, K., & BERGHE, H. V. D. (1987) · Clinical Genetics · doi:10.1111/j.1399-0004.1987.tb03351.x · Cited by 93 · see citing works
CONVERSATIONAL SKILLS OF INDIVIDUALS WITH FRAGILE‐X SYNDROME: A COMPARISON WITH AUTISM AND DOWN SYNDROME
Ferrier, L. J., Bashir, A. S., Meryash, D. L., Johnston, J., & Wolff, P. (1991) · Developmental Medicine & Child Neurology · doi:10.1111/j.1469-8749.1991.tb14961.x · Cited by 91 · see citing works
Fragile X Syndrome, DSM-HI-R, and Autism
Reiss, A. L., & Freund, L. S. (1990) · Journal of the American Academy of Child & Adolescent Psychiatry · doi:10.1097/00004583-199011000-00007 · Cited by 89 · see citing works
A theoretical analysis of the role of hyperarousal in the learning and behavior of fragile X males
Cohen, I. L. (1995) · Mental Retardation and Developmental Disabilities Research Reviews · doi:10.1002/mrdd.1410010410 · Cited by 88 · see citing works
A comparison of language characteristics of mentally retarded adults with fragile X syndrome and those with nonspecific mental retardation and autism
Paul, R., Dykens, E., Leckman, J. F., Watson, M. S., Breg, W. R., & Cohen, D. J. (1987) · Journal of Autism and Developmental Disorders · doi:10.1007/bf01486963 · Cited by 79 · see citing works
Is autism associated with the fragile X syndrome?
Fisch, G. S. (1992) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320430107 · Cited by 78 · see citing works
Fragile-X Syndrome: Variability of Phenotypic Expression
Bregman, J. D., Dykens, E. M., Watson, M. S., Ort, S. I., & Leckman, J. F. (1987) · Journal of the American Academy of Child & Adolescent Psychiatry · doi:10.1097/00004583-198707000-00001 · Cited by 69 · see citing works
Brief report: Analysis of the internal consistency of three autism scales
Sturmey, P., Matson, J. L., & Sevin, J. A. (1992) · Journal of Autism and Developmental Disorders · doi:10.1007/bf01058159 · Cited by 57 · see citing works
Autistic features, personality, and adaptive behavior in males with the fragile X syndrome and no autism.
Kerby, D., & Dawson, B. (1994) · PubMed · Cited by 52 · see citing works
What is associated with the fragile X syndrome?
Fisch, G. S. (1993) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320480210 · Cited by 50 · see citing works
Biomedical advances in developmental psychology: The case of fragile X syndrome.
Hagerman, R. J. (1996) · Developmental Psychology · doi:10.1037/0012-1649.32.3.416 · Cited by 24 · see citing works

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Authors: Bailey, D. B., Mesibov, G. B., Hatton, D. D., Clark, R. D., Roberts, J. E., & Mayhew, L.
Affiliations: University of North Carolina at Chapel Hill (US)
ORCID: Bailey, Donald B.
Roberts, Jane E.
Mayhew, Lisa
Venue: Journal of Autism and Developmental Disorders
Publisher: Springer Science and Business Media LLC
Published: 1998-12
Volume: 28
Issue: 6
Pages: 499-508
ISSN: 0162-3257 (ISSN-L)
1573-3432
DOI: 10.1023/a:1026048027397
OpenAlex: W1552013470
Cited by: 260
References: 26
Citations by year: 2026: 2 · 2025: 1 · 2024: 5 · 2023: 4 · 2022: 8 · 2021: 4 · 2020: 7 · 2019: 8 · 2018: 7 · 2017: 7 · 2016: 16 · 2015: 11 · 2014: 15 · 2013: 9 · 2012: 18
FWCI: 4.15
Type: journal article
Language: en
Open access: closed
Licence: www.springernature.com/gp/researchers/text-and-data-mining (tdm)
www.springernature.com/gp/researchers/text-and-data-mining (vor)
Locations: Journal of Autism and Developmental Disorders (publishedVersion)
PubMed (publishedVersion)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
Life Sciences › Neuroscience › Cognitive Neuroscience › Autism Spectrum Disorder Research
Life Sciences › Neuroscience › Developmental Neuroscience › Williams Syndrome Research
Keywords: fragile X syndrome, autism spectrum disorder, autistic behavior, Childhood Autism Rating Scale, autism prevalence, young boys, socioeconomic status
MeSH terms: Autistic Disorder, Child, Child, Preschool, Fragile X Syndrome, Humans, Infant, Male, Neuropsychological Tests, Psychometrics, Reproducibility of Results

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Autistic behavior in young boys with fragile X syndrome

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