scholarly journals Concurrent Associations between Expressive Language Ability and Independence in Adolescents and Adults with Fragile X Syndrome

2021 ◽  
Vol 11 (9) ◽  
pp. 1179
Author(s):  
Leonard Abbeduto ◽  
Jessica Klusek ◽  
Julie Lounds Taylor ◽  
Nadia Abdelnur ◽  
Nicole Sparapani ◽  
...  
Keyword(s):  
Fragile X Syndrome ◽  
Peer Relations ◽  
Fragile X ◽  
Language Skills ◽  
Expressive Language ◽  
Adult Life ◽  
Expressive Vocabulary ◽  
Partial Correlations ◽  
Adolescents And Adults ◽  
Independent Functioning

Background. Few individuals with fragile X syndrome (FXS) successfully meet adult normative expectations in education, employment, peer relations, and habitation, although there is within-syndrome variability in this regard. The primary goal of this study was to determine whether expressive language skills contribute to the capacity for independent functioning in adulthood even after controlling for nonverbal cognitive ability. Methods. Participants were 18- to 23-year-olds with FXS. Expressive language was assessed using the psychometrically validated Expressive Language Sampling (ELS) conversation and narration procedures. The language produced was transcribed and analyzed to yield measures of expressive vocabulary, syntax, and intelligibility. Parents concurrently completed questionnaires on the independent functioning of the participants with FXS. Results. All three ELS measures were significantly corelated with multiple measures of independence. The magnitudes of the correlations were reduced when nonverbal IQ was controlled through partial correlation. Nonetheless, many of the partial correlations were medium to large and several were statistically significant. Conclusions. Expressive language skills appear to contribute uniquely to the capacity for independence, although longitudinal data are needed to evaluate the possibility of a bidirectional relationship between these domains. Thus, language intervention may be a prerequisite for preparing youth with FXS for an independent adult life.

scholarly journals Joint Engagement and Early Language in Young Children With Fragile X Syndrome

2016 ◽  
Vol 59 (5) ◽  
pp. 1087-1098 ◽  
Author(s):  
Laura J. Hahn ◽  
Nancy C. Brady ◽  
Kandace K. Fleming ◽  
Steven F. Warren
Keyword(s):  
Young Children ◽  
Fragile X Syndrome ◽  
Rating Scale ◽  
Fragile X ◽  
Language Skills ◽  
Expressive Language ◽  
Autism Spectrum ◽  
Language Abilities ◽  
Joint Engagement ◽  
Autism Symptomatology

PurposeIn this study, we examine joint engagement (JE) in young children with fragile X syndrome (FXS) and its relationship to language abilities and autism spectrum disorder symptomatology at 24 to 36 months (toddler period) and 59 to 68 months (child period).MethodParticipants were 28 children with FXS (24 boys, four girls) and their mothers. Videotaped home observations were conducted during the toddler period and coded for JE. Language abilities were measured at both ages from a developmental assessment, a functional measure, and from a language sample. The Childhood Autism Rating Scale (Schopler, Reichler, & Renner, 1988) was completed at both ages.ResultsChildren with FXS spent more time in supported JE than in coordinated JE. Using a weighted JE variable, we found that children with FXS who had higher weighted JE scores also had more advanced expressive language skills at both the toddler and child periods. Weighted JE was negatively related to autism symptomatology in the toddler period.ConclusionThis study provides evidence that children with FXS who use more JE also have more advanced expressive language skills in early development. Therefore, existing early interventions that target JE behaviors may be effective for promoting language, social communication, and social interaction in this population.

Expressive Language During Conversational Speech in Boys With Fragile X Syndrome

2007 ◽  
Vol 112 (1) ◽  
pp. 1 ◽  
Author(s):  
Joanne E. Roberts ◽  
Elizabeth A. Hennon ◽  
Johanna R. Price ◽  
Elizabeth Dear ◽  
Kathleen Anderson ◽  
...  
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Expressive Language ◽  
Conversational Speech

scholarly journals Vocabulary comprehension in adults with fragile X syndrome (FXS)

2019 ◽  
Vol 11 (1) ◽  
Author(s):  
Anne Hoffmann ◽  
Sue Ellen Krause ◽  
Joanne Wuu ◽  
Sue Leurgans ◽  
Stephen J. Guter ◽  
...  
Keyword(s):  
Fragile X Syndrome ◽  
Cognitive Skills ◽  
Fragile X ◽  
Language Skills ◽  
Receptive Vocabulary ◽  
Fourth Edition ◽  
Mental Age ◽  
Autism Symptomatology ◽  
Global Language ◽  
Verbal Cognition

Abstract Background Receptive and expressive vocabulary in adult and adolescent males with fragile X syndrome (FXS) have been shown as significantly lower than their chronological age; however, receptive vocabulary has been considered a strength relative to mental age. This has not been formally examined, however, and data are needed to compare receptive vocabulary with other language skills and with mental age in individuals with FXS. This is especially important as vocabulary measures are sometimes used as a proxy to estimate language ability. Methods This preliminary study examined receptive vocabulary, global language, and cognitive skills in 42 adults (33 males and 9 females) with FXS as a portion of the baseline evaluation prior to randomization in a clinical trial of ampakine CX516. The battery of standardized tests addressed receptive vocabulary with the Peabody Picture Vocabulary Test, Third Edition (PPVT-III), receptive and expressive language (termed henceforth as global language) via the Preschool Language Scale, Fourth Edition or the Clinical Evaluation of Language Fundamentals, Third Edition, and non-verbal cognition via the Stanford-Binet Intelligence Scales, Fourth Edition (SB-IV). Results Results showed (1) significantly higher receptive vocabulary than global language, (2) significantly better receptive vocabulary than non-verbal cognition, (3) equivalent non-verbal cognition and global language, and (4) severity of autism symptomatology was not correlated to receptive vocabulary or global language once non-verbal cognition was removed as factor. The scores from the PPVT-III did not represent the global language skills in our sample of adults with FXS. Conclusions Findings from this investigation strongly suggest that the PPVT-III should not be used as a screening tool for language levels or cognitive function in clinical studies since the scores from the PPVT-III were not representative of global language or non-verbal cognitive skills in adults with intellectual disabilities. This finding is critical in order to understand how to evaluate, as well as to treat, language in individuals with FXS. Development of efficient and appropriate tools to measure language, cognition, and behavior in individuals with FXS is essential.

scholarly journals Delineating Repetitive Behavior Profiles across the Lifespan in Fragile X Syndrome

2020 ◽  
Vol 10 (4) ◽  
pp. 239
Author(s):  
Debra L. Reisinger ◽  
Rebecca C. Shaffer ◽  
Nicole Tartaglia ◽  
Elizabeth Berry-Kravis ◽  
Craig A. Erickson
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Repetitive Behavior ◽  
Autism Spectrum ◽  
Repetitive Behaviors ◽  
Motor Behaviors ◽  
Sensory Motor ◽  
Adolescents And Adults ◽  
Males And Females ◽  
The Impact

Restricted repetitive behaviors (RRBs) are a core area of impairment in autism spectrum disorder (ASD), but also affect several other neurodevelopmental disorders including fragile X syndrome (FXS). Current literature has begun to describe the RRB profile in FXS up through adolescence; however, little is known about the subtypes of RRBs in adolescents and adults. Further, literature on the RRB profile of females with FXS is limited. The present study examines the RRB profile across subtypes and specific items in both males and females with FXS while assessing for differences based on age, ASD diagnosis and the impact of IQ. Participants included 154 individuals with FXS (ages 2 to 50 years old). Results revealed a peak in RRB severity in FXS between 7–12 years for the majority of RRB subscales with the exception of Sensory-Motor behaviors peaking between 2 and 12 years before declining. Distinct RRB profiles in males and females with FXS emerged in addition to significant overlap among the item and subscale levels of RRBs across gender. Further, an added diagnosis of ASD significantly increased rates of RRBs across all subscale levels, but not necessarily across all items. Lastly, IQ did not solely account for the presence of RRBs in FXS, with Sensory-Motor behaviors being driven by comorbid ASD in males with FXS, and Restricted Interest behaviors being driven by comorbid ASD regardless of gender. These findings build on the current understanding of RRBs in FXS based on gender and comorbid ASD and lay important groundwork for the development of targeted behavioral and pharmacological treatments.

scholarly journals Exploring the Adult Life of Men and Women With Fragile X Syndrome: Results From a National Survey

2011 ◽  
Vol 116 (1) ◽  
pp. 16-35 ◽  
Author(s):  
Sigan L Hartley ◽  
Marsha Mailick Seltzer ◽  
Melissa Raspa ◽  
Murrey Olmstead ◽  
Ellen Bishop ◽  
...  
Keyword(s):  
Mental Health ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
Adult Life ◽  
Autism Spectrum ◽  
Health Conditions ◽  
Functional Skills ◽  
Employment Level ◽  
Men And Women ◽  
Mental Health Conditions

Abstract Using data from a national family survey, the authors describe the adult lives (i.e., residence, employment, level of assistance needed with everyday life, friendships, and leisure activities) of 328 adults with the full mutation of the FMR1 gene and identify characteristics related to independence in these domains. Level of functional skills was the strongest predictor of independence in adult life for men, whereas ability to interact appropriately was the strongest predictor for women. Co-occurring mental health conditions influenced independence in adult life for men and women, in particular, autism spectrum disorders for men and affect problems for women. Services for adults with fragile X syndrome should not only target functional skills but interpersonal skills and co-occurring mental health conditions.

scholarly journals Effect of Sustained Maternal Responsivity on Later Vocabulary Development in Children With Fragile X Syndrome

2014 ◽  
Vol 57 (1) ◽  
pp. 212-226 ◽  
Author(s):  
Nancy Brady ◽  
Steven F. Warren ◽  
Kandace Fleming ◽  
Juliana Keller ◽  
Audra Sterling
Keyword(s):  
Fragile X Syndrome ◽  
Middle Childhood ◽  
Fragile X ◽  
Vocabulary Development ◽  
Receptive Vocabulary ◽  
Significant Variable ◽  
Expressive Vocabulary ◽  
Autism Symptoms ◽  
Maternal Responsivity ◽  
Time Period

Purpose This research explored whether sustained maternal responsivity (a parent–child interaction style characterized by warmth, nurturance, and stability as well as specific behaviors, such as contingent positive responses to child initiations) was a significant variable predicting vocabulary development of children with fragile X syndrome through age 9 years. Method Fifty-five mother–child dyads were followed longitudinally when children were between 2 and 10 years of age. Measures of maternal responsivity and child vocabulary were obtained at regular intervals starting at age 2.9 years. Sustained responsivity was indicated by the average responsivity measured over Observations 2–5. Responsivity at the 1st time period, autism symptoms, and cognitive development were used as control variables. Results After controlling for development and autism symptoms, the authors found significant effects for sustained responsivity on receptive vocabulary, expressive vocabulary, and the rate of different words children produced through age 9. Conclusions Maternal responsivity, which is typically a variable of interest during early childhood, continues to be a significant variable, predicting vocabulary development through the middle childhood period. Thus, responsivity is a potential target for language interventions through this age period.

scholarly journals A Pilot Quantitative Evaluation of Early Life Language Development in Fragile X Syndrome

2019 ◽  
Vol 9 (2) ◽  
pp. 27 ◽  
Author(s):  
Debra Reisinger ◽  
Rebecca Shaffer ◽  
Ernest Pedapati ◽  
Kelli Dominick ◽  
Craig Erickson
Keyword(s):  
Language Development ◽  
Language Learning ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
Language Skills ◽  
Infants And Toddlers ◽  
Early Language ◽  
Early Language Development ◽  
Automated Vocal Analysis ◽  
Analysis System

Language delay and communication deficits are a core characteristic of the fragile X syndrome (FXS) phenotype. To date, the literature examining early language development in FXS is limited potentially due to barriers in language assessment in very young children. The present study is one of the first to examine early language development through vocal production and the language learning environment in infants and toddlers with FXS utilizing an automated vocal analysis system. Child vocalizations, conversational turns, and adult word counts in the home environment were collected and analyzed in a group of nine infants and toddlers with FXS and compared to a typically developing (TD) normative sample. Results suggest infants and toddlers with FXS are exhibiting deficits in their early language skills when compared to their chronological expectations. Despite this, when accounting for overall developmental level, their early language skills appear to be on track. Additionally, FXS caregivers utilize less vocalizations around infants and toddlers with FXS; however, additional research is needed to understand the true gap between FXS caregivers and TD caregivers. These findings provide preliminary information about the early language learning environment and support for the feasibility of utilizing an automated vocal analysis system within the FXS population that could ease data collection and further our understanding of the emergence of language development.

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