Narrative language competence in children and adolescents with Down syndrome

This study was designed to examine the narrative language abilities of children and adolescents with Down syndrome in comparison to same-age peers with fragile X syndrome and younger typically developing children matched by nonverbal cognitive ability levels. Participants produced narrative retells from a wordless picture book. Narratives were analyzed at the macrostructural (i.e., their internal episodic structure) and the microstructural (i.e., rate of use of specific word categories) levels. Mean length of utterance, a microstructural metric of syntactic complexity, was used as a control variable. Participants with Down syndrome produced fewer episodic elements in their narratives (i.e., their narratives were less fully realized) than the typically developing participants, although mean length of utterance differences accounted for the macrostructural differences between participant groups. At the microstructural level, participants with Down syndrome displayed a lower rate of verb use than the groups with fragile X syndrome and typical development, even after accounting for mean length of utterance. These findings reflect both similarities and differences between individuals with Down syndrome or fragile X syndrome and contribute to our understanding of the language phenotype of Down syndrome. Implications for interventions to promote language development and academic achievement are discussed

Inclusion of Individuals With Neurodevelopmental Disorders in Norm-Referenced Language Assessments

Standardized, norm-referenced language assessment tools are used for a variety of purposes, including in education, clinical practice, and research. Unfortunately, normreferenced language assessment tools can demonstrate floor effects (i.e., a large percentage of individuals scoring at or near the lowest limit of the assessment tool) when used with some groups with neurodevelopmental disorders (NDDs), such as individuals with intellectual disability and neurogenetic syndromes. Without variability at the lower end of these assessment tools, professionals cannot accurately measure language strengths and difficulties within or across individuals. This lack of variability may be tied to poor representation of individuals with NDDs in normative samples. Therefore, the purpose of this study was to identify and examine common standardized, norm-referenced language assessment tools to report the representation of individuals with NDDs in normative samples and the range of standard/index scores provided. A systematic search identified 57 assessment tools that met inclusion criteria. Coding of the assessment manuals identified that most assessment tools included a “disability” or “exceptionality” group in their normative sample. However, the total number of individuals in these groups and the number of individuals with specific NDDs was small. Further, the characteristics of these groups (e.g., demographic information; disability type) were often poorly defined. The floor standard/index scores of most assessment tools were in the 40s or 50s. Only four assessment tools provided a standard score lower than 40. Findings of this study can assist clinicians, educators, and researchers in their selections of norm-referenced assessment tools when working with individuals with NDDs

Matching Variables for Research Involving Youth with Down Syndrome: Leiter-R versus PPVT-4

Much of what is known about the cognitive profile of Down syndrome (DS) is based on using either receptive vocabulary (e.g., PPTV-4) or nonverbal ability (e.g., Leiter-R) as a baseline to represent cognitive developmental level. In the present study, we examined the relation between these two measures in youth with DS, with non-DS intellectual disability (ID) and with typical development (TD). We also examined the degree to which these two measures produce similar results when used as a group matching variable. In a cross-sectional developmental trajectory analysis, we found that the relation between PPVT-4 and Leiter-R was largely similar across groups. However, when contrasting PPVT-4 and Leiter-R as alternate matching variables, the pattern of results was not always the same. When matched on Leiter-R or PPVT-4, the group with DS performed below that of the groups with ID and TD on receptive grammar and below the group with TD on category learning. When matched on the PPVT-4, the group with ID performed below that of the group with TD on receptive grammar and category learning, but these differences between the groups with ID and TD were not found when matched on the Leiter-R. The results of the study suggest that the PPVT-4 and Leiter-R are interchangeable at least for some outcome measures for comparing youth with DS and TD, but they may produce different results when comparing youth with ID and TD

Patterns of autism spectrum symptomatology in individuals with Down syndrome without comorbid autism spectrum disorder

Background Prevalence estimates of autism spectrum disorder (ASD) in Down syndrome (DS) are highly varied. This variation is partly due to the difficulty of screening for and diagnosing comorbid ASD in individuals with a syndrome that carries its own set of social communicative and behavioral difficulties that are not well documented. The aim of this study was to identify the typical range of social communicative impairments observed in children, adolescents, and young adults with DS who do not have comorbid ASD. Methods We examined patterns of scores from the five subscales of the Social Responsiveness Scale (SRS) in 46 individuals with DS (ages 10–21 years) without comorbid ASD relative to the published normative sample. We also explored the correlations between SRS symptomatology and age, nonverbal cognition, and receptive language. Results SRS scores were elevated (i.e., more ASD symptoms endorsed), with mean scores falling into the clinically significant range. Analysis by subscale revealed a specific pattern, with Autistic Mannerisms and Social Cognition scores significantly more elevated than Social Communication scores, which were significantly more elevated than Social Awareness and Social Motivation scores. Correlations between SRS scores and the other measures varied by subscale. Conclusions General elevated ASD symptomatology on the SRS indicates the need for developing population-based norms specific to DS. The pattern of scores across subscales should inform clinicians of the typical range of behaviors observed in DS so that individuals with atypical patterns of behavior can be more easily identified and considered for a full ASD evaluation

Using the Social Skills Improvement System (SSiS) Rating Scales to assess social skills in youth with Down syndrome

Introduction and MethodsThis study provides preliminary data on the Social Skills Improvement System (SSiS) Rating Scales Parent Form to measure social skills in a sample of 124 children and adolescents with Down syndrome (DS) ages 6–17 years.ResultsOverall, participants demonstrated relatively mild symptoms, with the sample’s average standard score falling within 1 standard deviation from the mean of the normative sample for the social skills (M = 92, SD = 15) and problem behaviors (M = 104, SD = 12) domains (normative sample M = 100, SD = 15 for both domains). However, a wide range of scores was observed across the sample for the composite and subscale scores. Differential patterns were also observed by subscale. For some subscales (i.e., Cooperation, Assertion, Responsibility, Engagement, Externalizing, Hyperactivity/Inattention, and Autism Spectrum), a disproportionate number of participants scored in the below average (i.e., lower levels of social skills) or above average (i.e., more symptomatic in problem behaviors or autism spectrum) range relative to the normative sample; for other subscales (i.e., Communication, Empathy, Self-Control, Bullying, and Internalizing), participants’ score distribution aligned more closely to that of the normative sample. SSiS composite scores correlated in the expected directions with standardized measures of autism characteristics, executive function, and expressive language.DiscussionThis study provides some of the first evidence validating the use of the SSiS in youth with DS, filling a gap in standardized measures of social functioning in this population

Inferential language use by youth with Down syndrome during narration

We examined inferential language use by youth with Down syndrome (DS) in the context of narrative storytelling relative to younger typically developing (TD) children and same-aged peers with fragile X syndrome (FXS) matched on nonverbal cognitive ability level. Participants' narratives were coded for the use of different types of inferential language. Participants with DS used proportionately less inferential language overall relative to their counterparts with TD or FXS, although mean length of utterance accounted for group differences observed for the DS-TD group comparison only. Patterns of inferential language use varied across inferential subtypes and across participant groups, with mean length of utterance playing a significant role in group differences. These findings suggest potential syndrome specificity to the DS phenotype regarding impairments in inferential language use that can be partially explained by level of expressive syntactic ability and should be considered in future research. Clinical interventions within the DS population, therefore, should target to some extent the use of inferential language and complex sentence structure

Verb production by individuals with Down syndrome during narration

BackgroundDespite research identifying verb knowledge as a strong predictor of later syntactic skills in typical development, virtually no research has examined verb development in Down syndrome.AimsThe purpose of this study was to examine verb production (density, diversity, and type-token ratios) by individuals with Down syndrome in the context of story generation relative to two comparison groups - nonverbal cognitive ability level matches with typical development and chronological age matches with mixed-etiology intellectual disability.Methods and proceduresThirty-five participants with Down syndrome (11-21 years), 27 participants with intellectual disability (13-20 years), and 29 participants with typical development (4-6 years) completed a narrative story generation task. Transcripts were coded and analyzed for verb production.Outcomes and resultsExamining overall verb production, participants with Down syndrome produced narratives with less verb density than participants with typical development and had smaller verb type-token ratios than participants with intellectual disability. Upon examining lexical verb production, participants with Down syndrome produced narratives with less lexical verb density than participants with typical development.Conclusions and implicationsThe results indicate that individuals with Down syndrome have a developmentally appropriate diversity of verbs in their lexicon but are not using verbs as frequently as comparison groups

Patterns of change in nonverbal cognition in adolescents with Down syndrome.

This study was designed to examine longitudinal change in nonverbal cognitive abilities across adolescence for 20 males with Down syndrome (DS). We used hierarchical linear modeling to examine the rate of change in performance on the subtests of the Leiter-R Brief IQ across four annual time points and to determine the relation between maternal IQ and level and rate of change in performance. Results indicated no significant change in IQ (standard scores) with age in the sample, suggesting IQ stability during adolescence for individuals with DS, although several participants performed at floor level on the standard scores for the Leiter-R, limiting interpretation. Growth scores, however, provide a metric of absolute ability level, allow for the examination of change in Leiter-R performance in all participants, and minimize floor effects. Results from the analysis of growth scores indicated significant gain in absolute nonverbal cognitive ability levels (growth score values) over time for the adolescents with DS, although the growth varied by subdomain. Maternal IQ did not explain variability in cognitive performance or change in that performance over time in our sample of adolescents with DS