Growth of Expressive Syntax in Children With Fragile X Syndrome

Purpose This research explored syntactic growth in children with fragile X syndrome (FXS) over a 5-year period, and variability in growth in relation to autism symptoms, nonverbal cognition, maternal responsivity, and gender. Method Language samples at 4 time points from 39 children with FXS, 31 boys and 8 girls, were analyzed using the Index of Productive Syntax (Scarborough, 1990) and mean length of utterance (Brown, 1973). The degree of autism symptoms was evaluated using the Childhood Autism Rating Scale (Schopler, Reichler, & Renner, 1988) at the first time point. Maternal responsivity estimates were averaged across time points. Results Children with FXS showed significant syntactic growth over time and a significant plateau (quadratic trend) in the later observations. Children who exhibited more autism symptoms at Time 1 had significantly lower syntactic abilities over time than children who exhibited fewer autism symptoms. Nonverbal cognition significantly predicted mean length of utterance scores but not Index of Productive Syntax scores. Maternal responsivity was not a significant predictor of syntactic outcomes. Girls with FXS generally demonstrated better expressive syntax than boys with FXS with notable individual differences. Conclusion Despite significant growth over time, expressive syntax is a vulnerable domain for children with FXS, especially for those with severe autism symptoms. Clinical implications arising from the current findings are discussed.

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

Journal of Speech Language and Hearing Research ◽

10.1044/1092-4388(2013/12-0341) ◽

2014 ◽

Vol 57 (1) ◽

pp. 212-226 ◽

Cited By ~ 32

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.

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Language Development From Early Childhood to Adolescence in Youths With Fragile X Syndrome

Journal of Speech Language and Hearing Research ◽

10.1044/2020_jslhr-20-00198 ◽

2020 ◽

Vol 63 (11) ◽

pp. 3727-3742

Author(s):

Nancy C. Brady ◽

Kandace Fleming ◽

Shelley L. Bredin-Oja ◽

Heather Fielding-Gebhardt ◽

Steven F. Warren

Keyword(s):

Early Childhood ◽

Language Development ◽

Fragile X Syndrome ◽

Standardized Tests ◽

Fragile X ◽

Receptive Vocabulary ◽

Language Growth ◽

Mean Length Of Utterance ◽

Language Sample ◽

Over Time

Purpose The aim of this study was to investigate language growth in individuals with fragile X syndrome (FXS) from early childhood to adolescence and the influence of maternal responsivity on language growth. Method We conducted a longitudinal analysis of language development in 55 youths (44 males, 11 females) with FXS. Data collection spanned the ages of 11–216 months. We measured expressive and receptive vocabulary with standardized tests. The number of different words and mean length of utterance were obtained from language sample analyses of mother–child interactions. We also measured maternal comments (responsivity indicator) produced during the language samples and child nonverbal IQ. Results Growth models indicated that rates of number of different words and receptive vocabulary were related to maternal commenting. Mean length of utterance did not change significantly over time. Expressive vocabulary measured with a standardized test grew, but the growth was not related to maternal commenting. Nonverbal IQ was related to all language outcomes at age of 10 years and to changes over time in vocabulary. Visual analysis indicated that the highest scores on standardized tests were produced by girls; however, measures derived from language sample analyses appeared similar for boys and girls. Language models for boys only were similar to the total sample models with lower scores at age of 10 years for some outcomes. Conclusion Results of persistent language impairments for most youths with FXS suggest the need for continued, focused interventions aimed at improved language productions in addition to a responsive environment. Supplemental Material https://doi.org/10.23641/asha.13022825

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Measuring Agency Change Across the Domain of Hypnosis

10.31234/osf.io/apceh ◽

2019 ◽

Author(s):

Vince Polito ◽

Amanda Barnier ◽

Erik Woody

Keyword(s):

Rating Scale ◽

Sense Of Agency ◽

Pass Rates ◽

Current Experiment ◽

Multidimensional Construct ◽

Time Points ◽

Hypnotic Induction ◽

Different Types ◽

Over Time ◽

Postexperimental Inquiry

Building on Hilgard’s (1965) classic work, the domain of hypnosis has been conceptualised by Barnier, Dienes, and Mitchell (2008) as comprising three levels: (1) classic hypnotic items, (2) responding between and within items, and (3) state and trait. The current experiment investigates sense of agency across each of these three levels. Forty-six high hypnotisable participants completed an ideomotor (arm levitation), a challenge (arm rigidity) and a cognitive (anosmia) item either following a hypnotic induction (hypnosis condition) or without a hypnotic induction (wake condition). In a postexperimental inquiry, participants rated their feelings of control at three time points for each item: during the suggestion, test and cancellation phases. They also completed the Sense of Agency Rating Scale (Polito, Barnier, & Woody, 2013) for each item. Pass rates, control ratings, and agency scores fluctuated across the different types of items and for the three phases of each item; also, control ratings and agency scores often differed across participants who passed versus failed each item. Interestingly, whereas a hypnotic induction influenced the likelihood of passing items, it had no direct effect on agentive experiences. These results suggest that altered sense of agency is not a unidimensional or static quality “switched on” by hypnotic induction, but a dynamic multidimensional construct that varies across items, over time and according to whether individuals pass or fail suggestions.

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Study of the Interaction between Executive Function and Adaptive Behavior at School in Girls with Fragile X Syndrome

Genes ◽

10.3390/genes12081108 ◽

2021 ◽

Vol 12 (8) ◽

pp. 1108

Author(s):

Lorena Joga-Elvira ◽

Jennifer Martinez-Olmo ◽

María-Luisa Joga ◽

Carlos Jacas ◽

Ana Roche-Martínez ◽

...

Keyword(s):

Executive Function ◽

Executive Functions ◽

Fragile X Syndrome ◽

Adaptive Behavior ◽

Neuropsychological Tests ◽

Rating Scale ◽

Fragile X ◽

School Setting ◽

Control Group ◽

The Relationship

The aim of this research is to analyze the relationship between executive functions and adaptive behavior in girls with Fragile X syndrome (FXS) in the school setting. This study is part of a larger investigation conducted at the Hospital Parc Tauli in Sabadell. The sample consists of a total of 40 girls (26 with FXS and 14 control) aged 7–16 years, who were administered different neuropsychological tests (WISC-V, NEPSY-II, WCST, TOL) and questionnaires answered by teachers (ABAS-II, BRIEF 2, ADHD Rating Scale). The results show that there is a greater interaction between some areas of executive function (cognitive flexibility, auditory attention, and visual abstraction capacity) and certain areas of adaptive behavior (conceptual, practical, social, and total domains) in the FXS group than in the control group. These results suggest that an alteration in the executive functions was affecting the daily functioning of the girls with FXS to a greater extent.

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The Emergence of Effortful Control in Young Boys With Fragile X Syndrome

American Journal on Intellectual and Developmental Disabilities ◽

10.1352/1944-7558-123.2.89 ◽

2018 ◽

Vol 123 (2) ◽

pp. 89-102 ◽

Cited By ~ 1

Author(s):

Marissa Robinson ◽

Jessica Klusek ◽

Michele D. Poe ◽

Deborah D. Hatton ◽

Jane E. Roberts

Keyword(s):

Fragile X Syndrome ◽

Effortful Control ◽

Fragile X ◽

Single Gene ◽

Social Emotional Development ◽

Social Emotional ◽

Autism Symptoms ◽

Emotional Outcomes ◽

Preschool Boys ◽

Molecular Measures

Abstract Effortful control, or the ability to suppress a dominant response to perform a subdominant response, is an early-emerging temperament trait that is linked with positive social-emotional development. Fragile X syndrome (FXS) is a single-gene disorder characterized by hallmark regulatory impairments, suggesting diminished effortful control. This study compared the development of effortful control in preschool boys with FXS (n = 97) and typical development (n = 32). Unlike their typical peers, the boys with FXS did not exhibit growth in effortful control over time, which could not be accounted for by adaptive impairments, FMR1 molecular measures, or autism symptoms. These results contribute to our understanding of the childhood phenotype of FXS that may be linked to the poor social-emotional outcomes seen in this group.

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Autism Spectrum Disorder in Children and Adolescents With Fragile X Syndrome: Within-Syndrome Differences and Age-Related Changes

American Journal on Intellectual and Developmental Disabilities ◽

10.1352/1944-7558-115.4.307 ◽

2010 ◽

Vol 115 (4) ◽

pp. 307-326 ◽

Cited By ~ 62

Author(s):

Andrea McDuffie ◽

Leonard Abbeduto ◽

Pamela Lewis ◽

Sara Kover ◽

Jee-Seon Kim ◽

...

Keyword(s):

Social Interaction ◽

Fragile X Syndrome ◽

Fragile X ◽

Repetitive Behaviors ◽

Effect Sizes ◽

Autism Symptoms ◽

Restricted Interests ◽

Age Related ◽

Reciprocal Social Interaction ◽

Age Related Changes

Abstract The Autism Diagnostic Interview-Revised (ADI-R) was used to examine diagnostic profiles and age-related changes in autism symptoms for a group of verbal children and adolescents who had fragile X syndrome, with and without autism. After controlling for nonverbal IQ, we found statistically significant between-group differences for lifetime and current autism symptoms for the Communication and Restricted Interests/Repetitive Behaviors domains, but not the Reciprocal Social Interaction domain. Effect sizes for differences in Reciprocal Social Interaction also were smaller than effect sizes for the other domains, with one exception. Overall, severity of autism symptoms improved with age for all participants, with the least improvement noted for Restricted Interests and Repetitive Behaviors. FMRP did not account for unique variance in autism symptoms over and above nonverbal IQ.

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

Journal of Speech Language and Hearing Research ◽

10.1044/2016_jslhr-l-15-0005 ◽

2016 ◽

Vol 59 (5) ◽

pp. 1087-1098 ◽

Cited By ~ 2

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.

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Daily Living Skills in Adolescent and Young Adult Males With Fragile X Syndrome

American Journal on Intellectual and Developmental Disabilities ◽

10.1352/1944-7558-127.1.64 ◽

2022 ◽

Vol 127 (1) ◽

pp. 64-83

Author(s):

Angela John Thurman ◽

Stephanie Summers Swinehart ◽

Jessica Klusek ◽

Jane E. Roberts ◽

Lauren Bullard ◽

...

Keyword(s):

Fragile X Syndrome ◽

Daily Living ◽

Fragile X ◽

Receptive Language ◽

Daily Living Skills ◽

Adolescent And Young Adult ◽

Adult Males ◽

Autism Symptomatology ◽

Living Skills ◽

Nonverbal Cognition

Abstract By adulthood, most males with fragile X syndrome (FXS) require support to navigate day-to-day settings. The present study cross-sectionally: (1) characterized the profile of daily living skills in males with FXS and (2) examined associated participant characteristics (i.e., fragile X mental retardation protein [FMRP] expression, nonverbal cognition, language, autism symptomatology, and anxiety symptomatology) using the Waisman-Activities of Daily Living questionnaire. Males with FXS (n = 57, ages 15–23 years) needed more help/support in the areas of domestic and community daily livings skills, than in the area of personal daily living skills. Significant associations were observed between reduced daily living skills and lower nonverbal cognition, receptive language, expressive language, and increased autism symptomatology. Receptive language emerged as the strongest unique predictor of daily living skill performance.

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Assessing the impact of conversational overlap in content on child language growth

Journal of Child Language ◽

10.1017/s0305000917000083 ◽

2017 ◽

Vol 45 (1) ◽

pp. 72-96 ◽

Cited By ~ 3

Author(s):

ELIZABETH S. CHE ◽

PATRICIA J. BROOKS ◽

MARIA F. ALARCON ◽

FRANCIS D. YANNACO ◽

SEAMUS DONNELLY

Keyword(s):

Language Development ◽

New England ◽

Child Language ◽

Language Outcomes ◽

Time Points ◽

Parents And Children ◽

Language Growth ◽

Mean Length Of Utterance ◽

The Impact ◽

Over Time

AbstractWhen engaged in conversation, both parents and children tend to re-use words that their partner has just said. This study explored whether proportions of maternal and/or child utterances that overlapped in content with what their partner had just said contributed to growth in mean length of utterance (MLU), developmental sentence score, and vocabulary diversity over time. We analyzed the New England longitudinal corpus from the CHILDES database, comprising transcripts of mother–child conversations at 14, 20, and 32 months, using the CHIP command to compute proportions of utterances with overlapping content. Rates of maternal overlap, but not child overlap, at earlier time-points predicted child language outcomes at later time-points, after controlling for earlier child MLU. We suggest that maternal overlap plays a formative role in child language development by providing content that is immediately relevant to what the child has in mind.

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