scholarly journals Development of an Expressive Language Sampling Procedure in Fragile X Syndrome

2013 ◽  
Vol 34 (4) ◽  
pp. 245-251 ◽  
Author(s):  
Elizabeth Berry-Kravis ◽  
Emily Doll ◽  
Audra Sterling ◽  
Sara T. Kover ◽  
Susen M. Schroeder ◽  
...  
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Expressive Language ◽  
Sampling Procedure ◽  
Language Sampling

scholarly journals Correction to: Expressive language sampling as a source of outcome measures for treatment studies in fragile X syndrome: feasibility, practice effects, test-retest reliability, and construct validity

2020 ◽  
Vol 12 (1) ◽  
Author(s):  
Leonard Abbeduto ◽  
Elizabeth Berry-Kravis ◽  
Audra Sterling ◽  
Stephanie Sherman ◽  
Jamie O. Edgin ◽  
...  
Keyword(s):  
Construct Validity ◽  
Fragile X Syndrome ◽  
Outcome Measures ◽  
Fragile X ◽  
Expressive Language ◽  
Practice Effects ◽  
Original Publication ◽  
Retest Reliability ◽  
Language Sampling ◽  
Test Retest Reliability

In the original publication of this article [1], the author name Leonard Abbeduto was misspelled as Leonardkk Abbeduto. The original article has been corrected.

scholarly journals The Relationship between Expressive Language Sampling and Clinical Measures in Fragile X Syndrome and Typical Development

2020 ◽  
Vol 10 (2) ◽  
pp. 66 ◽  
Author(s):  
Rebecca C. Shaffer ◽  
Lauren Schmitt ◽  
Angela John Thurman ◽  
Leonard Abbeduto ◽  
Michael Hong ◽  
...  
Keyword(s):  
Fragile X Syndrome ◽  
Clinical Symptoms ◽  
Fragile X ◽  
Expressive Language ◽  
Autism Spectrum ◽  
Syntactic Complexity ◽  
Typical Development ◽  
Language Sampling ◽  
Wide Range ◽  
Clinical Measures

Language impairment is a core difficulty in fragile X syndrome (FXS), and yet standardized measures lack the sensitivity to assess developmental changes in the nature of these impairments. Expressive Language Sampling Narrative (ELS-N) has emerged as a promising new measure with research demonstrating its usefulness in a wide range of ages in developmental disabilities and typical development. We examined ELS-N results in FXS and age-matched typically developing (TD) controls along with cognitive, adaptive, and clinical measures. We found the groups differed significantly on all ELS-N variables. Cognitive abilities were related to lexical diversity, syntactic complexity, and unintelligibility for the FXS group, but only verbal abilities were related to syntactic complexity in TD. Autism spectrum disorder (ASD) symptomatology was related to less intelligibility in speech. Measures of hyperactivity were related to increased talkativeness and unintelligibility. In addition, FXS males in comparison to FXS females were more impaired in cognitive ability, ASD symptoms, hyperactivity, and anxiety. This study extends the previous ELS research, supporting its use in FXS research as a measure to characterize language abilities. It also demonstrates the relationships between ELS-N variables and measures of cognitive, adaptive, ASD symptoms, and clinical symptoms.

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 Expressive language sampling as a source of outcome measures for treatment studies in fragile X syndrome: feasibility, practice effects, test-retest reliability, and construct validity

2020 ◽  
Vol 12 (1) ◽  
Author(s):  
Leonard Abbeduto ◽  
Elizabeth Berry-Kravis ◽  
Audra Sterling ◽  
Stephanie Sherman ◽  
Jamie O. Edgin ◽  
...  
Keyword(s):  
Construct Validity ◽  
Outcome Measures ◽  
Symptom Severity ◽  
Fragile X ◽  
Expressive Language ◽  
Practice Effects ◽  
Full Sample ◽  
Retest Reliability ◽  
Language Sampling ◽  
Test Retest Reliability

Abstract Background The evaluation of treatment efficacy for individuals with fragile X syndrome (FXS) or intellectual disability (ID) more generally has been hampered by the lack of adequate outcome measures. We evaluated expressive language sampling (ELS) as a procedure for generating outcome measures for treatment research in FXS. We addressed: (a) feasibility, (b) practice effects over two administrations, (c) test-retest reliability over the repeated administrations, and (d) construct validity. We addressed these issues for the full sample as well as for subgroups defined by age, IQ, and ASD status. Methods Participants were 106 individuals with FXS between ages 6 and 23 years who had IQs within the range of intellectual disability (IQ < 70). ELS procedures for collecting samples in conversation and narration were followed and analyzed separately. Five measures were derived from transcripts segmented into C-units (i.e., an independent clause and its modifiers): number of C-units per minute (talkativeness), number of different word roots (vocabulary), C-unit length in morphemes (syntax), percentage of C-units containing dysfluency (utterance planning), and percentage of C-units that were fully or partly unintelligible (articulatory quality). ELS procedures were administered twice at 4-week intervals for each participant. Standardized tests and informant reports were administered and provided measures for evaluating construct validity of ELS measures. Results We found low rates of noncompliance, suggesting the task can be completed meaningfully by most individuals with FXS, although noncompliance was higher for younger, lower IQ, and more autistic participants. Minimal practice effects and strong test-retest reliability over the 4-week interval were observed for the full sample and across the range of ages, IQs, and autism symptom severity. Evidence of convergent construct validity was observed for the measures of vocabulary, syntax, and unintelligibility for the full sample and across the range of IQ and autism symptom severity, but not for participants under age 12. Conversation and narration yielded largely similar results in all analyses. Conclusions The findings suggest that the ELS procedures are feasible and yield measures with adequate psychometric properties for a majority of 6 to 23 years with FXS who have ID. The procedures work equally well regardless of level of ID or degree of ASD severity. The procedures, however, are more challenging and have somewhat less adequate psychometric properties for individuals with FXS under age 12.

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.

scholarly journals Perseveration in the Connected Speech of Boys with Fragile X Syndrome with and Without Autism Spectrum Disorder

2012 ◽  
Vol 117 (5) ◽  
pp. 384-399 ◽  
Author(s):  
Gary E. Martin ◽  
Joanne E. Roberts ◽  
Nancy Helm-Estabrooks ◽  
John Sideris ◽  
Jacqueline Vanderbilt ◽  
...  
Keyword(s):  
Autism Spectrum Disorder ◽  
Social Interaction ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
Autism Spectrum ◽  
Spectrum Disorder ◽  
Typically Developing ◽  
Connected Speech ◽  
Language Sampling ◽  
Language Characteristic

Abstract Verbal perseveration is a frequently reported language characteristic of males with Fragile X syndrome and may be a defining feature or hallmark of the syndrome. We compared the verbal perseveration of boys with Fragile X syndrome with (n  =  29) and without (n  =  30) autism spectrum disorder, boys with Down syndrome (n  =  27), and typically developing boys (n  =  25) at similar nonverbal mental ages. During a social interaction, boys with both Fragile X syndrome and autism spectrum disorder produced significantly more topic perseveration than all other groups. In social interaction as compared to narration, boys with Fragile X syndrome (regardless of autism status) produced significantly more topic perseveration. These findings suggest that autism status, as well as language sampling context, affect perseveration in boys with Fragile X syndrome.

scholarly journals A neurophysiological model of speech production deficits in fragile X syndrome

2019 ◽  
Vol 2 (1) ◽  
Author(s):  
Lauren M Schmitt ◽  
Jun Wang ◽  
Ernest V Pedapati ◽  
Angela John Thurman ◽  
Leonard Abbeduto ◽  
...  
Keyword(s):  
Fragile X Syndrome ◽  
Speech Production ◽  
Fragile X ◽  
Expressive Language ◽  
Temporal Cortex ◽  
Autism Spectrum ◽  
Event Related Potential ◽  
Speech Sounds ◽  
Speech Activity ◽  
Gamma Power

Abstract Fragile X syndrome is the most common inherited intellectual disability and monogenic cause of autism spectrum disorder. Expressive language deficits, especially in speech production, are nearly ubiquitous among individuals with fragile X, but understanding of the neurological bases for these deficits remains limited. Speech production depends on feedforward control and the synchronization of neural oscillations between speech-related areas of frontal cortex and auditory areas of temporal cortex. Interaction in this circuitry allows the corollary discharge of intended speech generated from an efference copy of speech commands to be compared against actual speech sounds, which is critical for making adaptive adjustments to optimize future speech. We aimed to determine whether alterations in coherence between frontal and temporal cortices prior to speech production are present in individuals with fragile X and whether they relate to expressive language dysfunction. Twenty-one participants with full-mutation fragile X syndrome (aged 7–55 years, eight females) and 20 healthy controls (matched on age and sex) completed a talk/listen paradigm during high-density EEG recordings. During the talk task, participants repeated pronounced short vocalizations of ‘Ah’ every 1–2 s for a total of 180 s. During the listen task, participants passively listened to their recordings from the talk task. We compared pre-speech event-related potential activity, N1 suppression to speech sounds, single trial gamma power and fronto-temporal coherence between groups during these tasks and examined their relation to performance during a naturalistic language task. Prior to speech production, fragile X participants showed reduced pre-speech negativity, reduced fronto-temporal connectivity and greater frontal gamma power compared to controls. N1 suppression during self-generated speech did not differ between groups. Reduced pre-speech activity and increased frontal gamma power prior to speech production were related to less intelligible speech as well as broader social communication deficits in fragile X syndrome. Our findings indicate that coordinated pre-speech activity between frontal and temporal cortices is disrupted in individuals with fragile X in a clinically relevant way and represents a mechanism contributing to prominent speech production problems in the disorder.

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.

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