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The Relationship between Expressive Language Sampling and Clinical Measures in Fragile X Syndrome and Typical Development
Open AccessArticle

Language across the Lifespan in Fragile X Syndrome: Characteristics and Considerations for Assessment

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Departments of Pediatrics and Communication Disorders, Rush University Medical Center, Chicago, IL 60612, USA
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Department of Pediatrics, Rush University Medical Center, Chicago, IL 60612, USA
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Department of Psychiatry, Rush University Medical Center, Chicago, IL 60612, USA
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Division of Developmental Pediatrics, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO 80045, USA
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Division of Child and Adolescent Psychiatry, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229, USA
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Departments of Pediatrics, Neurological Sciences, Biochemistry, Rush University Medical Center, Chicago, IL 60612, USA
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Author to whom correspondence should be addressed.
Brain Sci. 2020, 10(4), 212; https://doi.org/10.3390/brainsci10040212
Received: 3 March 2020 / Revised: 27 March 2020 / Accepted: 30 March 2020 / Published: 4 April 2020
(This article belongs to the Special Issue Update on the Treatment of Fragile X Syndrome)
While it is widely acknowledged that language development is delayed for the majority of individuals with fragile X syndrome (FXS), there has been limited research into how best to assess this area. This study aimed to deepen the understanding of standardized language assessment in FXS by addressing the three following objectives: (1) Examine the feasibility and validity of widely-used, standardized assessments in participants with FXS; (2) describe linguistic and cognitive profiles for a large sample of individuals with FXS; and (3) Compare results obtained from objective testing in clinic to those obtained using caregiver report. Results indicate that previous results indicating strong correlations between cognition and language results hold true across a wide range of ages as well as across multiple assessments, with an exception in very young children. Caregiver report tended to give lower estimates of language ability than what was found using an objectively administered assessment. Appropriate assessments remain difficult to find as a significant percentage of individuals scored at floor when scaled scores were calculated. Further, a sub-group of participants were coded for behavioral response to testing demands, the majority being able to complete a standardized assessment. These results speak to the need for assessments that provide a wider range of items so individuals can both achieve a valid score and demonstrate progress in their attainment of language skills. View Full-Text
Keywords: Fragile X syndrome; language assessment; intellectual disability Fragile X syndrome; language assessment; intellectual disability
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Hoffmann, A.; Wang, A.; Berger, N.; Cordeiro, L.; Shaffer, R.; Tartaglia, N.; Erickson, C.; Berry-Kravis, E. Language across the Lifespan in Fragile X Syndrome: Characteristics and Considerations for Assessment. Brain Sci. 2020, 10, 212.

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