Search

Children with Down syndrome (DS) have both strengths and difficulties in speech, language, and social communication. Mental state language—the ability to discuss others' perspectives such as their thoughts, feelings, and intentions—represents a foundational social communicative skill that is delayed in many children with DS, even into the school-age years. The purpose of this article is to review the evidence base on mental state language development in school-age children with DS, focusing in particular on assessment and intervention. We discuss assessment procedures that are both age appropriate and developmentally appropriate for this population. We also present preliminary data highlighting the role of caregivers in supporting mental state language development in school-age children with DS through shared storytelling. We propose that interventions aimed at supporting mental state language development in DS should include a focus on caregiver–child shared storybook reading, even in the school-age years. Therefore, we discuss key considerations for clinicians when teaching caregivers strategies for supporting mental state language and social communication in children with DS.

We examined autism spectrum disorder (ASD) risk in a large national sample of 203 individuals with Down syndrome, 6-25 years old, to determine the association of ASD risk with age, sex, IQ, adaptive behaviors, and maladaptive behaviors. We used a two-pronged approach by (1) considering ASD symptomatology continuously across the sample of individuals with DS and examining associations with each characteristic, and (2) dichotomizing our sample into high and low ASD risk groups and comparing groups on each characteristic. The pattern of results was largely similar across both types of analyses. ASD symptomatology/risk was negatively associated with IQ and adaptive behaviors and positively associated with certain types of maladaptive behaviors. Clinical implications for screening and therapeutic purposes are discussed.

Purpose This article (a) examined the cross-sectional trajectories of mental state language use in children with Down syndrome (DS) and (b) identified developmental factors associated with its use. Method Forty children with DS aged 6–11 years generated stories from a wordless picture book and completed an assessment battery of other linguistic, cognitive, and social–emotional skills. Their narratives were coded for mental state language density (the proportion of utterances containing mental state references) and diversity (the number of different mental state terms used). Results The emergence of mental state language use during narrative storytelling was observed across the sample; 0%–24% of children's utterances included references to mental states, and a variety of mental state terms were produced. Cross-sectional developmental trajectory analysis revealed that expressive vocabulary and morphosyntax were significantly related to increased mental state language density and diversity. Nonverbal emotion knowledge was significantly related to greater diversity of mental state terms used. Age and nonverbal cognition were not significant factors. Conclusions This first in-depth, within-syndrome characterization of mental state language use by school-age children with DS provides an important next step for understanding mental state and narrative development in this population. By identifying skills associated with the development of mental state language, this study provides an avenue for future longitudinal research to determine causal relationships, ultimately informing intervention efforts.

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.

Background and aimsLittle is known about how autism spectrum disorder (ASD) symptoms present in individuals with Down syndrome (DS). Some behaviors may be symptomatic of comorbid ASD or more broadly representative of the DS phenotype. A prior research study documented elevated ASD-like symptoms in adolescents and young adults with DS without comorbid ASD, using a common ASD risk screening tool—the Social Responsiveness Scale (SRS). The current study applied a similar approach to younger children with DS using the SRS-2. The primary aim was to document patterns of ASD-like symptoms in children with DS at low risk of comorbid ASD to distinguish the symptoms that may be present across DS in general.MethodsSRS-2 standard scores were analyzed in a sample of 40 children with DS, 6–11 years old, who were considered to be at low risk for ASD based on the Social Communication Questionnaire (SCQ) screener. Other developmental characteristics (i.e., age, nonverbal IQ, expressive language), social skills, and problem behaviors were also examined across the sample.ResultsSRS-2 scores were significantly elevated in this sample compared to the normative population sample. A pattern of ASD-like symptomatology was observed across SRS-2 subdomains. These findings were similar to the findings of the prior study. However, nuanced differences were observed across the two samples that may represent developmental differences across different ages in this population.ConclusionsReplicating and extending a prior study's findings, certain ASD-like behaviors may occur in individuals with DS who are at low risk for comorbid ASD. Implications: Understanding the pattern of ASD-like behaviors that occur in children with DS who are at low risk for comorbid ASD will help clinicians in screening and identification efforts. In particular, it will lead to better specification of the behaviors or symptoms that are not characteristic of the DS phenotype and thus are red flags for comorbid ASD in this population.

Background Despite 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. Aims The 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 Procedures Thirty-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 Results Examining 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 Implications The 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.

The cause of the high degree of variability in cognition and behavior among individuals with Down syndrome (DS) is unknown. We hypothesized that birth defects requiring surgery in the first years of life (congenital heart defects and gastrointestinal defects) might affect an individual's level of function. We used data from the first 234 individuals, age 6-25 years, enrolled in the Down Syndrome Cognition Project (DSCP) to test this hypothesis. Data were drawn from medical records, parent interviews, and a cognitive and behavior assessment battery. Results did not support our hypothesis. That is, we found no evidence that either birth defect was associated with poorer outcomes, adjusting for gender, race/ethnicity, and socioeconomic status. Implications for study design and measurement are discussed.

As individuals with Down syndrome (DS) are living longer, there is a growing need to support this population across the lifespan. In particular, the transition out of high school marks a critical time point that sets the course for the rest of adulthood, especially for the outcomes of community-based living, post-secondary education, employment, and community engagement. Little is known about these outcomes in adults with DS, who may experience unique post-school challenges and opportunities relative to those with other intellectual and developmental disabilities. In this paper, we review the state of the literature on post-high school transition outcomes in DS and infuse a phenotypic approach to highlight individual and environmental characteristics associated with these outcomes. We also emphasize current service delivery needs and gaps in the evidence base that must be addressed in order to best support this population during the critical post-high school transition period.

Interpreting emotional expressions appropriately poses a challenge for individuals with autism spectrum disorder (ASD). In particular, difficulties with emotional processing in ASD are more pronounced in contexts where emotional expressions are subtle, automatic, and reflexive—that is, implicit. In contrast, explicit emotional processing, which requires the cognitive evaluation of an emotional experience, appears to be relatively intact in individuals with ASD. In the present study, we examined the brain activation and functional connectivity differences underlying explicit and implicit emotional processing in age- and IQ-matched adults with (n = 17) and without (n = 15) ASD. Results indicated: (1) significantly reduced levels of brain activation in participants with ASD in medial prefrontal cortex (MPFC) and superior temporal gyrus (STG) during implicit emotion processing; (2) significantly weaker functional connectivity in the ASD group in connections of the MPFC with the amygdala, temporal lobe, parietal lobe, and fusiform gyrus; (3) No group difference in performance accuracy or reaction time; and (4) Significant positive relationship between empathizing ability and STG activity in ASD but not in typically developing participants. These findings suggest that the neural mechanisms underlying implicit, but not explicit, emotion processing may be altered at multiple levels in individuals with ASD. Autism Res 2015. © 2015 International Society for Autism Research, Wiley Periodicals, Inc.

The present study is an in-depth examination of receptive vocabulary in individuals with Down syndrome (DS) in comparison to control groups of individuals of similar nonverbal ability with typical development (TD) and non-specific etiology intellectual disability (ID). Verb knowledge was of particular interest, as it is known to be a predictor of later syntactic development. Fifty participants with DS, aged 10–21 years, 29 participants with ID, 10–21 years, and 29 participants with TD, 4–9 years, completed measures of receptive vocabulary (PPVT-4), nonverbal ability (Leiter-R), and phonological memory (Nonword Repetition subtest of the CTOPP). Groups were compared on percentage correct of noun, verb and attribute items on the PPVT-4. Results revealed that on verb items, the participants with ID performed significantly better than both participants with DS and TD, even when overall receptive vocabulary ability and phonological memory were held constant. Groups with DS and TD showed the same pattern of lexical knowledge, performing better on nouns than both verbs and attributes. In contrast, the group with ID performed similarly on nouns and verbs, but worse on attributes.

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.

The present study evaluated the ability of males with fragile X syndrome (FXS), nonsyndromic autism spectrum disorder (ASD), or typical development to learn new words by using as a cue to the intended referent an emotional reaction indicating a successful (excitement) or unsuccessful (disappointment) search for a novel object. Performance for all groups exceeded chance-levels in both search conditions. In the Successful Search condition, participants with nonsyndromic ASD performed similarly to participants with FXS after controlling for severity of ASD. In the Unsuccessful Search condition, participants with FXS performed significantly worse than participants with nonsyndromic ASD, after controlling for severity of ASD. Predictors of performance in both search conditions differed between the three groups. Theoretical and clinical implications are discussed.

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.

Emotion knowledge was examined in 19 youth with Down syndrome (DS) and compared to typically developing (TD) children of similar developmental levels. This project expanded upon prior research on emotion knowledge in DS by utilizing a measure that minimized the need for linguistic skills, presented emotion expressions dynamically, and included social context cues. In Study 1, participants with DS were as accurate as TD participants when judging emotions from static or dynamic expression stimuli and from facial or contextual cues. In Study 2, participants with DS and TD participants showed similar cross-sectional developmental trajectories of emotion knowledge across mental age. This project highlights the importance of measure selection when examining emotion knowledge in samples with intellectual and developmental disabilities.

Reading-related skills of youth with intellectual disability (ID) were compared with those of typically developing (TD) children of similar verbal ability level. The group with ID scored lower than the TD group on word recognition and phonological decoding, but similarly on orthographic processing and rapid automatized naming (RAN). Further, phonological decoding significantly mediated the relation between group membership and word recognition, whereas neither orthographic processing nor RAN did so. The group with ID also underperformed the TD group on phonological awareness and phonological memory, both of which significantly mediated the relation between group membership and phonological decoding. These data suggest that poor word recognition in youth with ID may be due largely to poor phonological decoding, which in turn may be due largely to poor phonological awareness and poor phonological memory. More focus on phonological skills in the classroom may help students with ID to develop better word recognition skills.

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.