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

Introduction and Methods: This 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. Results: Overall, 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. Discussion: This 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.

Perspectives on adaptive functioning and intellectual functioning measures for intellectual disabilities behavioral research.

Introduction: Intellectual disability (ID) is a significant limitation in both intellectual ability and adaptive functioning, but many studies of participants with ID only include a measure of overall intellectual functioning when describing their samples. The purpose of this perspective article was to provide a starting point for future research regarding the utility of including measures of both intellectual and adaptive functioning in research focused on ID. In this article, we discuss the differences and similarities between the constructs of intellectual and adaptive functioning, how they are measured, and the benefits of using both measures to describe participant abilities. Data are presented to demonstrate that intellectual and adaptive functioning measures capture separate but related skills in a sample of individuals with ID (i.e., children with Down syndrome [DS]; the leading genetic cause of ID). Methods: Thirty children with DS (7-31 months) were administered the Mullen Scales of Early Learning and their mothers were interviewed using the Vineland Adaptive Behavior Scales. Results: At the group level, Vineland and Mullen composite scores were relatively normally distributed and positively correlated. At the individual level, a concordance correlation coefficient indicated moderate agreement between Vineland and Mullen composite scores. Discussion: Although many children showed consistency between measures, others did not. Our discussion and findings, though preliminary, highlight that intellectual and adaptive functioning are separate but related skills and that there are benefits to including both measures when describing samples with ID. We discuss considerations for including adaptive functioning measures to enhance future research on individuals with ID.

Exploring Caregiver Perceptions of Post-High School Employment Experiences Among Young Adults With Down Syndrome.

This qualitative study explored employment experiences and perceived satisfaction of young adults with Down syndrome (DS) who recently exited high school, as reported by their caregivers (n = 101). We analyzed caregivers' open-ended responses about their young adults' type of employment (n = 52 were employed) and identified themes associated with reported satisfaction (for both employed and unemployed). Natural supports were key to caregiver satisfaction; few opportunities for paid, community-based employment and long waiting lists for formal services were related to caregiver dissatisfaction. Job fit (e.g., hours, responsibilities, location), socialization opportunities, and independence were related to caregiver and perceived young adult (dis)satisfaction. These findings highlight unmet service needs, including assistance with finding a job that is the right fit for the individual with DS.

Mental State Language Development in Children With Down Syndrome Versus Typical Development.

This study compared mental state language (talk about emotions, thoughts, intentions, etc.) used by 6- to 11-year-old children with Down syndrome (DS) to a younger typically developing (TD) comparison group matched by nonverbal cognition. We aimed to determine (1) whether mental state language use is delayed in DS relative to developmental expectations, and (2) if there are differences between groups in the association between mental state language and developmental factors (emotion knowledge, expressive language). Rate of mental state language use was significantly lower in the group with DS, but the number of different mental state terms was not significantly different. Nuanced patterns of similarity and difference emerged between groups regarding the association between mental state language and other developmental factors.

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, norm-referenced 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.

Post-High School Transition Outcomes for Young Adults With Down Syndrome.

There is limited available research on the post-high school outcomes of young adults with Down syndrome (DS). The purpose of this study, therefore, was to characterize employment, community-based living, and community engagement outcomes and their correlates among young adults with DS who recently transitioned out of high school. Caregivers (n = 100) of young adults with DS who exited high school within the past 5 years completed an online survey. Approximately half of the individuals with DS were working in some capacity; almost all were living with caregivers. Individuals with DS were engaging in a variety of community activities each week. Adaptive functioning was related to both employment and community engagement. Parent involvement in transition planning was also related to community engagement.

Mental State Language Use in Children with Down Syndrome and the Role of Caregivers.

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.

Capturing cognitive and behavioral variability among individuals with Down syndrome: a latent profile analysis.

BACKGROUND: There is a high degree of inter- and intra-individual variability observed within the phenotype of Down syndrome. The Down Syndrome Cognition Project was formed to capture this variability by developing a large nationwide database of cognitive, behavioral, health, and genetic information on individuals with Down syndrome, ages 6-25 years. The current study used the Down Syndrome Cognition Project database to characterize cognitive and behavioral variability among individuals with Down syndrome. METHODS: Latent profile analysis was used to identify classes across a sample of 314 participants based on their cognition (IQ and executive functioning), adaptive and maladaptive behavior, and autism spectrum disorder symptomatology. A multivariate multinomial regression model simultaneously examined demographic correlates of class. RESULTS: Results supported a 3-class model. Each class demonstrated a unique profile across the subdomains of cognition and behavior. The "normative" class was the largest (n = 153, 48%) and displayed a relatively consistent profile of cognition and adaptive behavior, with low rates of maladaptive behavior and autism symptomatology. The "cognitive" class (n = 109, 35%) displayed low cognitive scores and adaptive behavior and more autism symptomatology, but with low rates of maladaptive behavior. The "behavioral" class, the smallest group (n = 52, 17%), demonstrated higher rates of maladaptive behavior and autism symptomatology, but with cognition levels similar to the "normative" class; their adaptive behavior scores fell in between the other two classes. Household income and sex were the only demographic variables to differ among classes. CONCLUSIONS: These findings highlight the importance of subtyping the cognitive and behavioral phenotype among individuals with Down syndrome to identify more homogeneous classes for future intervention and etiologic studies. Results also demonstrate the feasibility of using latent profile analysis to distinguish subtypes in this population. Limitations and future directions are discussed.

Cross-Sectional Trajectories of Mental State Language Development in Children With Down Syndrome.

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.

The Social Responsiveness Scale (SRS-2) in school-age children with Down syndrome at low risk for autism spectrum disorder.

BACKGROUND AND AIMS: Little 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. METHODS: SRS-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. RESULTS: SRS-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. CONCLUSIONS: Replicating 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.

Characteristics Associated with Autism Spectrum Disorder Risk in Individuals with Down Syndrome.

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.

Verb production by individuals with Down syndrome during narration.

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.

Associations Between Medical History, Cognition, and Behavior in Youth With Down Syndrome: A Report From the Down Syndrome Cognition Project.

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.

Narrative Language Sampling in Typical Development: Implications for Clinical Trials.

Purpose: This study examined cross-sectional age-related trajectories of expressive language variables (syntactic complexity, lexical diversity, unintelligibility, dysfluency, and talkativeness) derived from a narrative language sampling procedure. Method: Narrative samples were analyzed from 103 typically developing individuals, ages 4-21 years. Results: Results showed that this procedure was effective for the entire age range, with participants producing an utterance on virtually every page of the wordless picture books used to prompt the narrative. Importantly, the cross-sectional trajectories for syntactic complexity and lexical diversity showed age-related increases through the age of 18 years, although measures of other dimensions of language showed different relationships with age. Conclusions: These data inform developmental work and document the extent to which the narrative procedure can be used to characterize expressive language over a wide age range. This procedure has been proposed as an outcome measure for clinical trials and interventions involving individuals with intellectual and developmental disabilities. The present data document the developmental levels for which the procedure and metrics derived are appropriate.

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.

Receptive vocabulary analysis in Down syndrome.

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.

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 (DS) in comparison to same-age peers with fragile X syndrome (FXS) and younger typically developing (TD) 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 (MLU), a microstructural metric of syntactic complexity, was used as a control variable. Participants with DS produced fewer episodic elements in their narratives (i.e., their narratives were less fully realized) than the TD participants, although MLU differences accounted for the macrostructural differences between participant groups. At the microstructural level, participants with DS displayed a lower rate of verb use than the groups with FXS and typical development, even after accounting for MLU. These findings reflect both similarities and differences between individuals with DS or FXS and contribute to our understanding of the language phenotype of DS. Implications for interventions to promote language development and academic achievement are discussed.

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.

Use of emotional cues for lexical learning: a comparison of autism spectrum disorder and fragile X syndrome.

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.

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.