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To characterize autism spectrum-related symptomatology in children with Williams syndrome (WS) with phrase speech or fluent language, the Autism Diagnostic Observation Schedule Module 2 or 3 was administered. The cutoff for autism spectrum was met by 35% (14/40) who completed Module 2 and 30% (18/60) who completed Module 3. Similarities and differences in socio-communicative strengths and weaknesses as a function of language ability were identified. Symptom severity was negatively associated with IQ for participants with phrase speech but not for those with fluent language. The findings suggest an elevated risk of ASD for individuals with WS relative to the general population and contribute to a more nuanced sense of the socio-communicative functioning of children with WS. [ABSTRACT FROM AUTHOR]

Gold-standard diagnostic assessments of autism spectrum disorder (ASD) symptomatology were conducted on 63 children (mean CA: 8.81 years) with 7q11.23 duplication syndrome, one of the copy number variants identified by Sanders et al. (Neuron 70:863-885, 2011a) as associated with ASD. ASD classification rate was 39.6% for the Autism Diagnostic Interview-Revised and 25.4% for the Autism Diagnostic Observation Schedule-2 (ADOS-2). Based on these assessments combined with clinical judgment, 19.0% of children were diagnosed with ASD. Reasons for these discrepancies are discussed, as are differences in rate of diagnosis as a function of sex, age, and ADOS-2 module administered and differences in intellectual and adaptive behavior abilities as a function of presence or absence of ASD diagnosis and ADOS-2 module administered. Implications are addressed. [ABSTRACT FROM AUTHOR]

The article discusses a study on the predictors of parenting stress in children with autism spectrum disorder (ASD). Cognitive and adaptive functioning, problem behaviors, and parenting stress were examined in children with ASD. Moreover, the findings suggest that cognitive ability was not significantly connected to parenting stress.

A variety of genetic disorders of known etiology present with behavioral profiles similar to that described in autism spectrum disorders (ASDs). Although some of these disorders are more likely to be associated with a comorbid ASD diagnosis, there exist cases in which there is a lack of empirical evidence to support a dual diagnosis. Two disorders, Williams syndrome (WS) and 15q13.3 deletion syndrome, have both been reported in the literature as examples of this phenotypic overlap. We present a case study of a young child with both WS and 15q13.3 deletion syndrome and significant ASD-related symptomatology. The results of a developmental evaluation, specifically the rationale for ruling out a comorbid ASD, are the focus of the present report. Implications for careful diagnostic consideration in cases of patients with known genetic conditions are also discussed. [ABSTRACT FROM AUTHOR]

Neurofibromatosis-1 (NF1) is one of the most common single-gene autosomal dominant disorders. In addition to significant medical complications, learning and attention problems are two of the most commonly reported difficulties in the NF1 population. Given that executive dysfunction often contributes to both learning and attention problems, the goal of the current study was to examine relations between executive and academic skills for adolescents with NF1 while considering the role of intellectual functioning. Participants were 26 adolescents with NF1 and their parents. Data from laboratory-based measures and parent ratings of executive skills in day-to-day life were integrated to elucidate the factors that contribute to academic success. Elevated rates of difficulties were observed on the laboratory-based and parent report measures of executive functioning. Findings indicate that learning difficulties are pervasive in the NF1 population, and that executive dysfunction relates to academic achievement even when intellectual functioning and age are taken into account. Interventions aimed at improving executive skills may therefore improve academic performance in children with NF1. [ABSTRACT FROM AUTHOR]

Williams syndrome (WS) is genetic neurodevelopmental disorder with a well-characterized cognitive and behavioral phenotype. Research has consistently demonstrated high rates of psychopathology in this population; however, little research has examined the use of empirically-supported psychosocial interventions in those with WS. The current case study reports on the use of Habit Reversal Therapy (HRT) to treat multiple body-focused repetitive behaviors in a child with WS. Although HRT is a well-established cognitive-behavioral intervention for body-focused repetitive behaviors, it has been infrequently used in populations with developmental disabilities. An etiologically-informed approach was used to adapt HRT to fit the known behavioral and cognitive phenotype of WS. Results suggest that HRT may be beneficial for this population. Modified treatment elements are described and future research areas highlighted. [ABSTRACT FROM AUTHOR]

The behavioral phenotype of Williams syndrome (WS) is characterized by difficulties with establishment and maintenance of friendships despite high levels of interest in social interaction. Here, parents and teachers rated 84 children with WS ages 4-16 years using two commonly-used measures assessing aspects of social functioning: the Social Skills Rating System and the Social Responsiveness Scale. Mean prosocial functioning fell in the low average to average range, whereas social reciprocity was perceived to be an area of significant difficulty for many children. Concordance between parent and teacher ratings was high. Patterns of social functioning are discussed. Findings highlight the importance of parsing the construct of social skills to gain a nuanced understanding of the social phenotype in WS. [ABSTRACT FROM AUTHOR]

The Child Behavior Checklist (CBCL/6-18) is a widely used parent-report measure of youth internalizing and externalizing problems. Accumulating evidence, however, suggests that the CBCL may not adequately assess clinical problems in diverse populations. We investigated the measurement equivalence (ME) of the CBCL in an African American adolescent sample ( N = 145). Confirmatory factor analysis indicated poor factor model fit; however, modification indices and an alternative two-factor model produced improved fit statistics. Internal consistency coefficients of CBCL scales ranged from α = .65 to .88 and were generally lower than those reported for the normative sample. The CBCL exhibited convergent validity with conceptually similar scales on the Parent Report Scale of the Behavior Assessment System for Children; however, the correlations were weaker than those reported for the normative sample. The CBCL Internalizing composite failed to predict clinician-rated Emotional problems, however, the Externalizing composite predicted clinician-rated behavior problems. In summary, the CBCL did not exhibit unequivocal evidence of ME, as it both possesses and lacks a number of statistical attributes that would demonstrate its equivalence with African Americans. Future empirical and theoretical work is discussed. [ABSTRACT FROM AUTHOR]

The ADOS characterizes socio-communicative deficits in autism spectrum disorders (ASD). In this study the effect of module choice on ADOS classification was examined. For 74 participants (52 autism, 22 PDD-NOS), Module 1 and Module 2 were administered in a single session. Fifty-one participants maintained ADOS classification, with 17 more impaired on M2 and 6 more impaired on M1. For 64 participants (25 autism, 39 PDD-NOS), Module 2 and Module 3 were administered. Thirty-nine participants maintained classification, with 24 more impaired on M3 and 1 more impaired on M2. As expected, more impairment was indicated when a module with more language and task demands was administered. Clinical judgment of the most appropriate module for administration was found to be important. [ABSTRACT FROM AUTHOR]

Group-matching designs are commonly used to identify the diagnosis-specific characteristics of children with developmental disabilities. In this paper, we address three issues central to the use of this design. The first concerns the α level to be used for considering groups to be matched on the control variable(s). The second involves the measurement characteristics of the control and target variables. We discuss the properties of standard scores, raw scores, and age equivalents and argue against the use of age equivalents. In addition, we consider the appropriateness of the commonly made prediction that groups that are matched for a control variable such as language ability or nonverbal reasoning ability but are not matched for chronological age should perform at equivalent levels on the target variable. Finally, we discuss issues related to the interpretation of significant between-group differences on the target variable, assuming groups are well-matched on the control variables, and describe the benefits of a method that focuses on characterizing a disorder on a case-by-case basis and then aggregating the cases, using the measures of sensitivity and specificity from signal detection theory. [ABSTRACT FROM AUTHOR]