Sensory Processing and Maladaptive Behavior: Profiles Within the Down Syndrome Phenotype.

Aim: Sensory processing impairments are well characterized in children with neurodevelopmental disorders, particularly autism, and have been associated with maladaptive behaviors. However, little is known regarding sensory processing difficulties within Down syndrome, or how these difficulties may influence maladaptive behavior. This study aims to characterize sensory processing difficulties within the Down syndrome phenotype and determine the influence of processing difficulties on maladaptive behavior. Methods: To explore this issue, we administered the Short Sensory Profile and the Developmental Behavior Checklist to parents or primary caregivers of young children with DS (N = 49; M nonverbal mental age (NVMA) = 30.92 months (SD = 12.30); M chronological age (CA) = 67.04 (SD = 25.13). Results: Results indicated that Low Energy/Weak, Under-responsive/Seeks Sensation, and Auditory Filtering were the areas of greatest sensory regulation difficulty, and that Self-Absorbed behavior and Disruptive/Antisocial behavior were elevated areas of maladaptive behavior. Multivariate regression analyses indicated that Under-responsive/Seeks Sensation was the only sensory regulation domain significantly associated with Self-Absorbed and Disruptive/Antisocial behavior. Conclusion: Findings indicate a consistent pattern of sensory processing impairments and associations with maladaptive behavior in children with DS. Implications for interventions are discussed.

Use of a teacher nomination strategy to screen for autism spectrum disorders in general education classrooms: a pilot study.

Given a rising prevalence of autism spectrum disorders (ASD), this project aimed to develop and pilot test various teacher nomination strategies to identify children at risk for ASD in a timely, reliable, cost-effective manner. Sixty participating elementary school teachers evaluated 1323 children in total. Each teacher nominated students who most fit a description of ASD-associated characteristics, and completed the Autism Syndrome Screening Questionnaire (ASSQ) on every child in the classroom. The proportion of overall agreement between teacher nomination and ASSQ was 93-95%, depending upon the nomination parameters. Nomination required 15 min per class versus 3.5-5.5 h per class for the ASSQ. These results support the need for further study of teacher nomination strategies to identify children at risk for ASD.

Rapid facial reactions to emotional facial expressions in typically developing children and children with autism spectrum disorder.

Typical adults mimic facial expressions within 1000 ms, but adults with autism spectrum disorder (ASD) do not. These rapid facial reactions (RFRs) are associated with the development of social-emotional abilities. Such interpersonal matching may be caused by motor mirroring or emotional responses. Using facial electromyography (EMG), this study evaluated mechanisms underlying RFRs during childhood and examined possible impairment in children with ASD. Experiment 1 found RFRs to happy and angry faces (not fear faces) in 15 typically developing children from 7 to 12 years of age. RFRs of fear (not anger) in response to angry faces indicated an emotional mechanism. In 11 children (8-13 years of age) with ASD, Experiment 2 found undifferentiated RFRs to fear expressions and no consistent RFRs to happy or angry faces. However, as children with ASD aged, matching RFRs to happy faces increased significantly, suggesting the development of processes underlying matching RFRs during this period in ASD.

Use of the ADOS and ADI-R in children with psychosis: importance of clinical judgment.

The Autism Diagnostic Observation Schedule (ADOS) and the Autism Diagnostic Interview - Revised (ADI-R) are considered to be the 'gold standard' in diagnostic evaluations for autism. Developed as research tools and now gaining wide clinical use, the ADOS/ADI-R assessment package has been demonstrated to differentiate children with autism from those with other developmental disabilities; however, little work concerning the reliability and validity of the tools in children with a known history of psychosis has been undertaken. We report on the administration of the ADOS, ADI-R and clinical judgment in three cases of Childhood-Onset Schizophrenia. All 3 children met both ADOS and ADI-R criteria for an autism spectrum diagnosis, even though none of them received a clinical diagnosis of autism from either a research child psychiatrist or an experienced clinically trained, research psychologist with expertise in autism. Issues concerning overlap of symptom presentation and implications for research and clinical use of these assessment tools are discussed.

Executive function in preschoolers with autism: evidence consistent with a secondary deficit.

Recent research on executive function (EF) deficits in autism has led investigators to conclude that EF deficits are secondary to the disorder. The current study has two major goals: (1) Examine whether specific EF deficits are present in the youngest autism group to date (mean=2.9 years), and (2) examine whether such deficits are secondary to autism, or act as an early non-specific cognitive risk factor for autism by comparing EF abilities of this autism group to a CA-matched typically developing group. Results from Experiment 1 suggest no specific EF deficits in autism relative to MA-matched controls, while results from Experiment 2 are consistent with the hypothesis that EF deficits may emerge as a secondary deficit in autism. Alternative hypotheses are also considered.

Brief report: perception of body posture--what individuals with autism spectrum disorder might be missing.

Autism has been associated with atypical face and configural processing, as indicated by the lack of a face inversion effect (better recognition of upright than inverted faces). We investigated whether such atypical processing was restricted to the face or extended to social information found in body postures. An inversion paradigm compared recognition of upright and inverted faces, body postures, and houses. Typical adults demonstrated inversion effects for both faces and body postures, but adults with autism demonstrated only a face inversion effect. Adults with autism may not have a configural processing deficit per se, but instead may have strategies for recognizing faces not used for body postures. Results have implications for therapies employing training in imitation and body posture perception.

Emotional responsivity in young children with Williams syndrome.

The hypothesis that young children with Williams syndrome show higher rates of emotional responsivity relative to other children with developmental disabilities was explored. Performance of 23 young children with Williams syndrome and 30 MA-matched children with developmental disabilities of nonspecific etiologies was compared on an adaptation of Repacholi and Gopnik's (1997) "Yummy-Yucky" task. Results show that children with Williams syndrome were more likely to mimic and/or imitate facial affect and vocalizations than children in the mixed comparison group. Yet, this increased emotional responsivity did not substantially improve decision-making based on the affective display; children with Williams syndrome were more likely to attempt to convince the experimenter that the disliked food was likable. Implications of a social profile that includes enhanced emotional responsivity paired with impaired perspective taking are discussed.

Using Carey Temperament Scales to assess behavioral style in children with autism spectrum disorders.

Many researchers have suggested that temperament information could be useful for understanding the behavioral variability within the autism spectrum. The purpose of this brief report is to examine temperament profiles of 110 children with ASD (ages 3-8 years, 61 with Autistic Disorder, 42 with PDD-NOS; and 7 with Asperger Disorder) via a commonly used parent report measure of child temperament. Internal consistency of temperament dimensions, test-retest reliability, descriptions of means and standard deviations are examined, relative to previously published norms. Internal consistency of the dimensions and test-retest reliability were comparable to published norms; however, children with autism were rated as presenting with more extreme scores than typically-developing children on several dimensions. Limitations and implications for future work are discussed.

A two-year follow-up on risk status identified by the checklist for autism in toddlers.

We examined the characteristics of children at 4 to 5 years of age who were correctly and incorrectly classified as "at risk" for an autism spectrum diagnosis using the Checklist for Autism in Toddlers (CHAT) at age 2 to 3 years. Information is provided on the stability of risk/disorder status over a 2-year period of early development. Participants were 19 children with autism and 11 children with other developmental disabilities who had all been administered the CHAT between 2 and 3 years of age (Time 1) and received diagnostic and developmental re-evaluations between 4 and 6 years of age (Time 2). The risk status of children was discussed based on the original CHAT authors' criteria for risk of autism and the Denver modification for risk. High levels of stability in risk/diagnostic status from Time 1 assessments to Time 2 assessments were noted. Specifically, the original CHAT criteria for medium to high risk of autism applied at Time 1 predicted Time 2 diagnostic classification for 83% of the sample, and the Denver modification of the CHAT risk criteria predicted Time 2 diagnostic classification for 93% of the sample. Implications of the findings are discussed as they relate to early screening and identification of autism spectrum disorders.

Telehealth delivery of cognitive-behavioral intervention to youth with autism spectrum disorder and anxiety: A pilot study.

Youth with autism spectrum disorders frequently experience significant symptoms of anxiety. Empirically supported psychosocial interventions exist, yet access is limited, especially for families in rural areas. Telehealth (i.e. videoconferencing) has potential to reduce barriers to access to care; however, little is known about the feasibility or efficacy of directly intervening with youth with autism spectrum disorders through this modality. This study details the pilot testing of a telehealth version of an empirically supported intervention targeting anxiety in youth with autism spectrum disorders. The primary focus of this study is on feasibility, with evaluation of outcomes as a starting point for future randomized trials. In all, 33 families of youth with autism spectrum disorders and significant anxiety symptoms participated in this study (Telehealth Facing Your Fears (FYF) Intervention: n = 17; Wait-list control: n = 16). Youth of all functioning levels were included. Acceptability was strong; however, the usability of the technology was problematic for some families and impeded some sessions significantly. Fidelity of the telehealth version to the critical elements of the original, in vivo version was excellent. More work is needed to improve delivery of exposure practices and parent coaching. Preliminary efficacy analyses are promising, with improvements observed in youth anxiety over time (relative to a comparison group waiting for live intervention) and parent sense of competence (within group). Clearly, stronger designs are necessary to evaluate efficacy sufficiently; however, this study does provide support for further investigation of clinic-to-home videoconferencing as a direct intervention tool for youth with autism spectrum disorders and their parents.

Nonverbal requesting and problem-solving by toddlers with down syndrome.

The association between nonverbal requesting (as measured by the Early Social Communication Scales) and problem-solving skills (as measured by an object retrieval task) was examined in 16 toddlers who had Down syndrome, 18 toddlers with developmental disabilities of mixed etiologies, and 19 typically developing infants and toddlers. Toddlers with Down syndrome showed fewer instrumental requests than did those in the typically developing group, but equal numbers of social routine requests. Toddlers with Down syndrome also showed poorer problem-solving strategies and received more help than children in both comparison groups on the object-retrieval task. Results showed a significant association between instrumental requests and problem-solving in the Down syndrome group. Implications for strengthening problem-solving skills in Down syndrome are discussed.

Praxis skills in young children with Down syndrome, other developmental disabilities, and typically developing children.

This study explored whether young children with Down syndrome show praxis deficits that impact activities of daily living, and whether these deficits are specific to Down syndrome. We compared the performance of young children with Down syndrome, a mental age-matched group of children with developmental disabilities of mixed or unknown etiologies, and a group of typically developing infants and toddlers on praxis tasks and overall adaptive behavior (Vineland Adaptive Behavior Scales). Children with Down syndrome showed poorer overall motor functioning than the developmental disabilities comparison group as measured by the Vineland Adaptive Behavior Scales, F(2, 47) = 5.24, p < .01 (using one-way analysis of variance [ANOVA]). A one-way multivariate analysis of variance also showed that children with Down syndrome performed significantly worse overall than the developmental disabilities comparison group on a battery of praxis tasks, F(7, 18) = 2.95, p < .05, and a series of object retrieval tasks, F(7, 18) = 2.95, p < .05, suggesting a deficit in praxis that is specific to Down syndrome. Children with Down syndrome elicited significantly more help than both comparison groups during object retrieval trials, F(2, 48) = 4.94, p < .01 (using one-way ANOVA). When chronological age was partialled out, a strong relationship was observed between praxis and adaptive functioning in Down syndrome, r(8) = .69, p < .05. These findings suggest that young children with Down syndrome may need targeted interventions that focus on both praxis skills and motivational orientation.

Examining the Relationship Between Parental Anxiety and Treatment Response in Children and Adolescents with Autism Spectrum Disorder and Anxiety.

In response to the high co-occurrence of anxiety symptoms in youth with autism spectrum disorder (ASD), several interventions have been developed for this population. In spite of promising findings, some youth with ASD respond only minimally to such interventions. To understand potential factors that may impact treatment response, the current study explores the role of parental anxiety in youth treatment outcome. Thirty-one youth with ASD, ages 7-18, and their parents participated in the study. Parents completed the State/Trait Anxiety Inventory pre- and post-treatment. Contrary to previous research, there was no correlation between parental anxiety and youth anxiety at baseline or post-treatment. However, parental trait anxiety significantly decreased from pre- to post-treatment for parents of treatment responders. The findings are consistent with previous research and suggest a youth-to-parent influence.

Improving transportability of a cognitive-behavioral treatment intervention for anxiety in youth with autism spectrum disorders: results from a US-Canada collaboration.

Anxiety disorders frequently co-occur in youth with autism spectrum disorders. In addition to developing efficacious treatments for anxiety in children with autism spectrum disorders, it is important to examine the transportability of these treatments to real-world settings. Study aims were to (a) train clinicians to deliver Facing Your Fears: Group Therapy for Managing Anxiety in Children with High-Functioning Autism Spectrum Disorders to fidelity and (b) examine feasibility of the program for novel settings. A secondary aim was to examine preliminary youth treatment outcome. Results indicated that clinicians obtained excellent fidelity following a workshop and ongoing consultation. Acceptability ratings indicated that Facing Your Fears Therapy was viewed favorably, and critiques were incorporated into program revisions. Meaningful reductions in anxiety were reported posttreatment for 53% of children. Results support the initial effectiveness and transportability of Facing Your Fears Therapy in new clinical settings.

Linguistic and cognitive functioning and autism symptoms in young children with fragile X syndrome.

Linguistic and cognitive profiles were examined in 18 children with autism and 18 children with fragile X syndrome (mean ages = 34 months). State-of-the-art diagnostic procedures for autism symptom identification were administered. Eight children with fragile X met criteria for autism. Comparison of linguistic and cognitive profiles (autism, fragile X without autism, fragile X with autism) revealed that children with fragile X (with autism) were more impaired in nonverbal cognition and expressive language. Receptive language was a relative strength for children with fragile X (without autism). There were no differences in receptive language in children with autism, regardless of fragile X status. Low receptive language may be a marker for autism symptoms in young children with fragile X.

Adaptive behavior and problem behavior in young children with Williams syndrome.

The present study compares the adaptive behavior profile of 18 young children with Williams syndrome (WS) and a developmentally matched group of 19 children with developmental disabilities and examines the relationship between adaptive behavior and problem behaviors in WS. Parents completed the Vineland Adaptive Behavioral Scales-Interview edition and the Developmental Behavior Checklist-Primary Caregiver version (WS only). Children with WS had higher adaptive communication scores than children with other developmental disabilities. Children with WS demonstrated relative strengths in adaptive communication and socialization, coupled with relative weaknesses in daily living. Adaptive communication and socialization were negatively associated with problem behaviors in social relating in WS.

Complex Psychiatric Comorbidity of Treatment-Seeking Youth With Autism Spectrum Disorder and Anxiety Symptoms.

Anxiety disorders and other co-occurring psychiatric disorders significantly impact adaptive functioning for many children with autism spectrum disorder (ASD). This descriptive study examines the complexity of psychiatric comorbidity in treatment-seeking youth with ASD and anxiety symptoms. Forty-two parents of 8- to 14-year-old children with ASD and anxiety symptoms completed a structured psychiatric interview (K-SADS) and provided information about the child's past and current psychological functioning as part of a screening process to enter an anxiety intervention program. Overall, comorbidity was very complex, with children obtaining an average of 4 psychiatric diagnoses (including anxiety disorders) on a structured clinical interview (range = 0-9). Onset and course differed by psychiatric disorder. Complexity of comorbidity did not differ significantly by age, sex, or autism severity. Despite clinical significance of the symptoms reported, few children were currently (or ever) engaged in mental health treatment or group psychosocial intervention. Although the specificity of the current sample limits the generalizability of these results, findings suggest that treatment-seeking children with ASD and anxiety often present with additional psychiatric symptoms, which supports a transdiagnostic approach to research and intervention in this area. Accurate assessment of comorbidity may provide valuable information for families and clinicians regarding individualized treatment approaches.

Early intersubjective skills and the understanding of intentionality in young children with Down syndrome.

This study examined the relationship between early intersubjective skills (joint attention and affect sharing) and the development of the understanding of intentionality in 16 young children with Down syndrome (DS) and 16 developmentally matched children with other developmental disabilities (DD). The study of intentionality focuses on how children come to understand the goal-directed actions of others and is an important precursor to the development of more complex social cognitive skills, such as theory of mind. Joint attention and affect sharing were examined using the Early Social Communication Scales (Mundy, Sigman, & Kasari, 1990; Seibert, Hogan, & Mundy, 1982). Meltzoff's (1995) behavioral reenactment paradigm was used to examine the understanding of intentionality. For children with DS, higher rates of affect sharing were associated with poorer intention reading abilities. This pattern was not observed in children with other DD. These results suggest that the intersubjective strengths associated with DS may not support the development of intentionality-interpretation skills. Future research is needed to explore if children with DS have the joint attention behaviors needed to be intentional.

Brief Report: impaired Flexible Item Selection Task (FIST) in school-age children with autism spectrum disorders.

Cognitive flexibility has been measured with inductive reasoning or explicit rule tasks in individuals with autism spectrum disorders (ASD). The Flexible Item Selection Task (FIST) differs from previous cognitive flexibility tasks in ASD research by giving children an abstract, ambiguous rule to switch. The ASD group (N = 22; Mean age = 8.28 years, SD = 1.52) achieved a lower shift percentage than the typically developing verbal mental-age control group (N = 22; Mean age = 6.26 years, SD = 0.82). There was a significant positive correlation between verbal mental age and shift percentage for children with ASD. Group differences on the FIST converge and extend prior evidence documenting an impaired ability to adapt rapidly to changes in task demands for individuals with ASD.

Diagnosing Autism in Individuals with Known Genetic Syndromes: Clinical Considerations and Implications for Intervention.

Assessing symptoms of autism in persons with known genetic syndromes associated with intellectual and/or developmental disability is a complex clinical endeavor. We suggest that a developmental approach to evaluation is essential to reliably teasing apart global impairments from autism-specific symptomology. In this chapter, we discuss our assumptions about autism spectrum disorders, the process of conducting a family-focused, comprehensive evaluation with behaviorally complex children and some implications for intervention in persons with co-occurring autism and known genetic syndromes.