Cited by 316Genetics and Neurodevelopmental DisordersAutism Spectrum Disorder ResearchDown syndrome and intellectual disability research

Clinical assessment of DSM-IV anxiety disorders in fragile X syndrome: prevalence and characterization

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Cordeiro, Lisa, Ballinger, Elizabeth, Hagerman, Randi, Hessl, David·2011·Journal of Neurodevelopmental Disorders

Abstract

Fragile X syndrome (FXS) is the most common form of inherited intellectual disability (ID). Anxiety and social withdrawal are considered core features of the FXS phenotype, yet there is limited diagnostic evidence of the prevalence of formal anxiety disorders in FXS. This study assessed the prevalence of anxiety disorders in a sample of 58 males and 39 females with FXS (ages 5.0-33.3 years). Participants' parents completed the Anxiety Disorders Interview Schedule (ADIS-IV), a clinical interview based on DSM-IV criteria, and the Anxiety Depression and Mood Scale (ADAMS), a psychiatric disorders screening instrument normed in ID. We conducted cognitive (IQ) and autism (AUT) assessments and surveyed medication use. Despite a high rate of psychopharmacological treatment, 86.2% of males and 76.9% of females met criteria for an anxiety disorder, with social phobia and specific phobia the most commonly diagnosed. Proband status, gender, and IQ were not significantly related to any anxiety disorders, however significantly higher rates of a few anxiety disorders were found in older age and AUT groups. Significant correlations between ADIS diagnoses and ADAMS scores provided cross-validation of instruments, indicating that the ADIS is suitable for use in FXS. A greater percentage of our sample met criteria for most anxiety disorders than has been reported in other ID groups or the general population. The rate of anxiety compared to general ID suggests that the FMR1 full mutation confers an especially high risk for these disorders, regardless of factors commonly associated with FXS clinical involvement. A thorough clinical assessment and treatment of anxiety should be included in the FXS standard of care.

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  • Boys with fragile X syndrome typically show ADHD with impulsivity and anxiety, repetitive language, hand biting, hand stereotypies, rocking, and sometimes head banging.

    “The behavior of boys with FXS typically includes attention deficit hyperactivity disorder (ADHD), with significant impulsivity and anxiety, as well as behaviors that include repetitive language, hand biting, hand stereotypies, rocking, and sometimes head banging (24,25).”

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  1. Miller LJ McIntosh DN McGrath J Shyu V Lampe M Taylor AK Electrodermal responses to sensory stimuli in individuals with fragile X syndrome: a preliminary report Am J Med Genet 1999 83 4 268 79 10.1002/(SICI)1096-8628(19990402)83:4<268::AID-AJMG7>3.0.CO;2-K 10208160 · DOI
  2. Newcombe RG Two-sided confidence intervals for the single proportion: comparison of seven methods Stat Med 1998 17 8 857 72 10.1002/(SICI)1097-0258(19980430)17:8<857::AID-SIM777>3.0.CO;2-E 9595616 · DOI · Paper page
  3. Silverman WK Albano AM Anxiety Disorders Interview Schedule (ADIS-IV) child/parent clinician manual 2004 New York Oxford University Press

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Authors
Cordeiro, L., Ballinger, E., Hagerman, R., & Hessl, D.
Affiliations
University of California Davis Medical Center (US)
Corresponding author
Hessl, David
Venue
Journal of Neurodevelopmental Disorders
Published
2010-12-03
Volume
3
Issue
1
Pages
57-67
ISSN
  • 1866-1947 (ISSN-L)
  • 1866-1955
OpenAlex
W2060911896
Cited by
316
References
75
Citations by year
2026: 8 · 2025: 9 · 2024: 14 · 2023: 16 · 2022: 30 · 2021: 29 · 2020: 21 · 2019: 32 · 2018: 18 · 2017: 30 · 2016: 19 · 2015: 14 · 2014: 31 · 2013: 17 · 2012: 15
FWCI
8.10
Type
journal article
Language
en
Open access
Licence
cc-by
Locations
  • Journal of Neurodevelopmental Disorders (publishedVersion, cc-by)
  • Europe PMC (PubMed Central) (publishedVersion, cc-by-nc)
  • PubMed Central (publishedVersion, cc-by-nc)
  • PubMed (publishedVersion)
  • ProQuest Demo Repository (submittedVersion)
Funders
  • National Institutes of Health (HD02274)
  • National Institute of Mental Health (R21MH080025, K23MH077554)
  • Eunice Kennedy Shriver National Institute of Child Health and Human Development (P30HD002274)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
  • Life Sciences › Neuroscience › Cognitive Neuroscience › Autism Spectrum Disorder Research
  • Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Down syndrome and intellectual disability research
Keywords
fragile X syndrome, anxiety disorders, prevalence, social anxiety disorder, specific phobia, intellectual disability, FMR1 full mutation, autism spectrum disorder, psychopharmacological treatment, gender differences, age differences

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