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Cornelia de Lange syndrome (CdLS) is a spectrum disorder due to variants in genes of the cohesin protein complex. The following abstracts are from the Cornelia de Lange Syndrome Scientific and Educational Symposium held virtually in October 2020. Aspects of behavior, including autistic features, impulsivity, adaptive skills, executive function, and anxiety are described. Applied behavioral analysis is a promising approach for autism, and an N-acetylcysteine trial is proposed. Children below 6 years with CdLS have an increased number of and further travel to medical providers, with insurance type comprising a significant barrier. Speech, language, and feeding abilities fall significantly below expectations for age in CdLS. Augmentative alternative communication can yield potential barriers as well as interesting benefits. Developmentally, studies in animal models further elucidate the mechanisms and roles of cohesin: link with mediator transcriptional complex; facilitation of enhancer-promoter communication; regulation of gene expression; allocation of cells to germ layers; and repair of spontaneous DNA damage in placental cells. Genome and RNA sequencing can help identify the molecular cause in the 20% of individuals with suspected CdLS and negative testing. The phenotypes in individuals with variants in the SMC1A gene are distinct, and that with intractable seizures has been further evaluated. AMA CME credits provided by GBMC, Baltimore, MD. All studies approved by an ethics committee.

The purpose of this study was to determine the effectiveness of a video-based instruction packet for teaching math-based vocational skills delivered through augmented reality (AR) to three adults with intellectual disabilities. The dependent variable was the percentage of steps performed correctly to solve each selected type of math problem. The independent variable was the video-based math intervention delivered via AR, which modeled the individual steps for solving three different multistep math problems: (1) adjusting a recipe to serve a different number of people, (2) calculating salary, and (3) calculating unit prices. Visual and statistical analyses demonstrated a functional relationship between the video-based math intervention and an increase in the percentage of steps completed correctly for each type of problem. All three participants showed significant gains immediately after receiving the intervention and maintained the learned skills following withdrawal of the intervention. Implications for practitioners and further research are discussed.

Middle school special education teachers often express concern about challenging student behavior. Class-Wide Function-Related Intervention Teams (CW-FIT), a behavior management program based on school-wide positive behavior support, have been effective in elementary general education classrooms. The present study, the first to apply it in a middle school special education setting, used an ABAC design to examine effects on student on-task behavior and teacher praise rates in a self-contained special education classroom for students with severe disabilities and their typically developing peer tutors. Results suggested that CW-FIT Tier 1 is associated with improvements in student on-task behavior and teacher praise rates, especially when peer tutors are included in the intervention and when the timer is silent. Both teachers and students reported the intervention to be socially valid. Study limitations and areas for future research are addressed.

This study investigated the effects of varying opportunities to respond when using a behavior management program with three elementary school students with developmental disabilities in a small group special education setting. These students exhibited both academic and behavioral difficulties. With the implementation of Class-Wide Function-Related Intervention Teams (CW-FIT) with low opportunities to respond (OTR), students increased their active engagement and correct responses, as well as decreased their disruptive behaviors. When CW-FIT with high OTR was implemented, students showed an even greater improvement than CW-FIT with low OTR. Higher rates of OTR resulted in higher levels of active engagement and correct responses and decreases in disruptive behavior for all three students. These results indicate that CW-FIT in combination with high OTR can help elementary school teachers manage students’ behavior and increase students’ correct responding when implemented in a small group special education setting.

Disruptive student behavior, a common concern for teachers, presents particular challenges for those who teach art. Class-Wide Function-Related Intervention Teams (CW-FIT) is a multitiered intervention for implementing effective classroom management strategies aligned with schoolwide positive behavior interventions and supports. CW-FIT has proven effective in general education classrooms, with its emphasis on social skills instruction, teacher praise, group contingency, and positive reinforcement. This first study of CW-FIT implementation in elementary art classrooms examined its effects on student on-task behavior. The researchers used a single-subject (AB) design in one classroom and reversal designs (ABAB) in two classrooms. Results indicated student on-task behavior significantly improved, and the teacher was able to implement CW-FIT with fidelity as well as increase her praise-to-reprimand ratios. Both teacher and students found the intervention to be socially valid. Study implications and limitations are discussed.

Students who experience social withdrawal and anxiety (internalizing difficulties) are often overlooked in schools due to the covert nature of their symptoms. The literature lacks empirically supported preventive approaches for kindergarten students who are socially withdrawn and behind their peers in developing social skills. Furthermore, research suggests that parents often are not adequately involved in interventions during this critical period of social development. In response to this need, a school-based intervention was implemented to increase the positive social interaction of three kindergarten students on the playground. Treatment consisted of (a) social skills training, (b) adult mediation, (c) self-evaluation and reinforcement, and (d) parent involvement through home notes. The effects of this intervention were assessed on the playground during recess using partial interval recording of target students’ positive interactions. During implementation of the intervention, all target students demonstrated improvements in social interaction. Future studies should investigate whether addressing the limitations of this study would yield stronger results with this underserved population.

Delivering individualized learning interventions to students with autism spectrum disorder (ASD) is daunting for education professionals already stretched to capacity meeting needs of all of their ...

Cornelia de Lange Syndrome (CdLS) is due to mutations in the genes for the structural and regulatory proteins that make up the cohesin complex, and is considered a cohesinopathy disorder or, more recently, a transcriptomopathy. New phenotypes have been recognized in this expanding field. There are multiple clinical issues facing individuals with all forms of CdLS, particularly in the neurodevelopmental system, but also gastrointestinal, cardiac, and musculoskeletal. Aspects of developmental and cell biology have found common endpoints in the biology of the cohesin complex, with improved understanding of the mechanisms, easier diagnostic tests, and the possibility of potential therapeutics, all major clinical implications for the individual with CdLS. The following abstracts are the presentations from the 7th Cornelia de Lange Syndrome Scientific and Educational Symposium, June 22–23, 2016, in Orlando, FL, in conjunction with the Cornelia de Lange Syndrome Foundation National Meeting. In addition to the scientific and clinical discussions, there were talks related to practical aspects of behavior including autism, transitions, communication, access to medical care, and databases. At the end of the symposium, a panel was held, which included several parents, affected individuals and genetic counselors, and discussed the greatest challenges in life and how this information can assist in guiding future research. The Research Committee of the CdLS Foundation organizes this meeting, reviews, and accepts abstracts, and subsequently disseminates the information to the families through members of the Clinical Advisory Board and publications. AMA CME credits were provided by Greater Baltimore Medical Center, Baltimore, MD.

The purpose of this study was to evaluate the effectiveness of teaching multi-step math skills to nine adults with disabilities in an 18–21 post-high school transition program using a video prompting intervention package. The dependent variable was the percentage of steps completed correctly. The independent variable was the video prompting intervention, which involved several multi-step math calculation skills: (a) calculating a tip (15%), (b) calculating item unit prices, and (c) adjusting a recipe for more or fewer people. Results indicated a functional relationship between the video prompting interventions and prompting package and the percentage of steps completed correctly. 8 out of the 9 adults showed significant gains immediately after receiving the video prompting intervention.

Challenging behavior in preschool is a serious concern for teachers. Positive behavior interventions and supports (PBIS) have been shown to be effective in reducing such behaviors. Class-Wide Function-Related Intervention Teams (CW-FIT) is a specific multi-tiered intervention for implementing effective classroom management strategies using PBIS practices. CW-FIT has been shown to be effective in elementary classrooms but has not yet been evaluated with younger age groups. CW-FIT Tier 1 is a group contingency utilizing social skills training, teacher praise, and positive reinforcement to improve student behavior. The present study examined the effects of CW-FIT Tier 1 implementation on student group on-task behavior and on teacher praise and reprimand rates in four preschool classrooms. A single-subject delayed multiple baseline design with embedded reversals was used to evaluate impact. Results indicated the intervention increased student group on-task behavior and teacher praise to reprimand ratios. Both teachers and children found CW-FIT Tier 1 to be socially valid. Limitations and implications of this study for researchers and practitioners are discussed.

Background Functional neuroimaging research in autism spectrum disorder has reported patterns of decreased long-range, within-network, and interhemispheric connectivity. Research has also reported increased corticostriatal connectivity and between-network connectivity for default and attentional networks. Past studies have excluded individuals with autism and low verbal and cognitive performance (LVCP), so connectivity in individuals more significantly affected with autism has not yet been studied. This represents a critical gap in our understanding of brain function across the autism spectrum. Methods Using behavioral support procedures adapted from Nordahl, et al. (J Neurodev Disord 8:20–20, 2016), we completed non-sedated structural and functional MRI scans of 56 children ages 7–17, including LVCP children (n = 17, mean IQ = 54), children with autism and higher performance (HVCP, n = 20, mean IQ = 106), and neurotypical children (NT, n = 19, mean IQ = 111). Preparation included detailed intake questionnaires, video modeling, behavioral and anxiety reduction techniques, active noise-canceling headphones, and in-scan presentation of the Inscapes movie paradigm from Vanderwal et al. (Neuroimage 122:222–32, 2015). A high temporal resolution multiband echoplanar fMRI protocol analyzed motion-free time series data, extracted from concatenated volumes to mitigate the influence of motion artifact. All participants had > 200 volumes of motion-free fMRI scanning. Analyses were corrected for multiple comparisons. Results LVCP showed decreased within-network connectivity in default, salience, auditory, and frontoparietal networks (LVCP

Cornelia de Lange Syndrome (CdLS) is the most common example of disorders of the cohesin complex, or cohesinopathies. There are a myriad of clinical issues facing individuals with CdLS, particularly in the neurodevelopmental system, which also have implications for the parents and caretakers, involved professionals, therapists, and schools. Basic research in developmental and cell biology on cohesin is showing significant progress, with improved understanding of the mechanisms and the possibility of potential therapeutics. The following abstracts are presentations from the 6th Cornelia de Lange Syndrome Scientific and Educational Symposium, which took place on June 25-26, 2014, in conjunction with the Cornelia de Lange Syndrome Foundation National Meeting in Costa Mesa, CA. The Research Committee of the CdLS Foundation organizes the meeting, reviews and accepts abstracts, and subsequently disseminates the information to the families through members of the Clinical Advisory Board. In addition to the scientific and clinical discussions, there were educationally focused talks related to practical aspects of behavior and development. AMA CME credits were provided by Greater Baltimore Medical Center, Baltimore, MD.

Repetitive self-injurious eye poking and stereotypic hand clapping were assessed in a child with autism and hearing impairment. A functional behavioral assessment conducted to identify the variables maintaining eye-poking behaviors indicated automatic reinforcement. Choice and environmental enrichment strategies were implemented to decrease the motivating operations associated with eye poking. Providing a choice of toys to hold in his hand led to the greatest reductions in both repetitive eye poking and hand clapping. Three follow-up observations were conducted 9 months after the intervention, which indicated that eye poking was eliminated and hand clapping was greatly reduced. This antecedent intervention was simple and effective, two hallmarks of sustainable behavior interventions.

Aggressive behaviors are common in individuals diagnosed with autism spectrum disorder (ASD) and may be phenotypic indicators of different subtypes within ASD. In current research literature for non-ASD samples, aggression has been linked to several brain structures associated with emotion and behavioral control. However, few if any studies exist investigating brain volume differences in individuals with ASD who have comorbid aggression as indicated by standardized diagnostic and behavioral measures.We examined neuroimaging data from individuals rigorously diagnosed with ASD versus typically developing (TD) controls. We began with data from brain volume regions of interest (ROI) taken from previous literature on aggression including the brainstem, amygdala, orbitofrontal cortex, anterior cingulate cortex, and dorsolateral prefrontal cortex. We defined aggression status using the Irritability subscale of the Aberrant Behavior Checklist and used lasso logistic regression to select among these predictor variables. Brainstem volume was the only variable shown to be a predictor of aggression status.We found that smaller brainstem volumes are associated with higher odds of being in the high aggression group.Understanding brain differences in individuals with ASD who engage in aggressive behavior from those with ASD who do not can inform treatment approaches. Future research should investigate brainstem structure and function in ASD to identify possible mechanisms related to arousal and aggression.

Classroom management in dual immersion classrooms includes unique challenges. The teacher must instruct and correct in the L2 language, in which students are beginning learners, and effective classroom management strategies appropriate to the L2 context. Class-Wide Function-Related Intervention Teams (CW-FIT) is a positive classroom management program that teaches social skills and uses group contingencies to improve behavior. The present study examined the ability of French immersion teachers to implement CW-FIT in the L2, including the effects of CW-FIT on teacher praise and reprimand rates and as well as on students’ classroom behavior. Social validity was also assessed. A single-subject multiple baseline design with embedded reversals was used to evaluate impact in second-, third-, and fourth-grade dual immersion classrooms. Results indicated that dual immersion teachers were able to implement CW-FIT in L2 with fidelity. The intervention significantly increased teacher praise and improved classroom on-task behavior. Changes in teacher reprimand rates were inconsistent. Students and teachers reported CW-FIT to be socially valid.

Parenting children with autism in countries with limited professional and financial resources can be overwhelming. Parent training led by non-governmental organizations may help alleviate some of these burdens. The present pilot study was conducted in the Republic of Macedonia, a country located in Southeastern Europe. The purpose of the study was to evaluate a parent training model for disseminating evidence-based practices through didactic and pyramidal training strategies. Results indicated that children improved on a number of different behaviors and results provide some evidence that parenting confidence and distress improved.

Noncompliance in three elementary age students with intellectual disabilities was assessed using functional behavioral assessments. Escape was identified as the primary function of the behavior in all three students, and access to tangible items was identified in one of the students as a secondary function. Teacher-monitoring and self-monitoring interventions were implemented within a multiple baseline design across three students to increase compliance. Results demonstrated that the intervention was effective and that all three students increased compliance during teacher-monitoring and self-monitoring conditions. These results are discussed in terms of recent research on function-based self-monitoring interventions.

This study evaluated the effects of an intervention for teaching phonological awareness skills to kindergarten-age children with intellectual and developmental disabilities. The intervention employed a combined multiple treatment and multiple baseline design, embedded in playtime and implemented under naturally occurring conditions. Six children in a special education kindergarten class were taught syllable segmentation, first sound identification, and phoneme segmenting. Results indicated that all children made gains on each skill. Results are discussed in light of current research on phonological awareness intervention for young children with intellectual and developmental disabilities.

Comprehensive evidence-based interventions are needed to help early childhood educators manage challenging student behaviors. One such intervention, class-wide function-related intervention teams (CW-FIT), is a multi-tiered behavioral intervention program based on positive behavior support principles, including four main elements: (a) teaching socially appropriate communication skills, (b) using differential reinforcement with an interdependent group contingency, (c) extinguishing or eliminating potential reinforcement for problem behavior, and (d) implementing individual interventions using self-management, help cards, and/or functional assessment. This study implemented CW-FIT with five kindergarten through second grade teachers. Results demonstrated efficacy of the program as early childhood teachers increased their praise rates and decreased their reprimand rates, and students responded with increased on-task behavior and fewer classroom disruptions. Study implications and limitations are addressed.

The differential effects of interventions aligned with indicated and non-in- dicated behavioral function were studied. Disruptive behavior of a fourth grade child at risk for emotional and behavioral disorders (EBD) was assessed through functional behavioral assessment (FBA) procedures. Following the FBA, three interventions were designed, only one based on an indicated be - havioral function. Each intervention was assessed within a multi-element de- sign. The intervention based on the student's identified behavioral function resulted in the greatest increase in on-task behaviors and the greatest reduc- tion in disruptions. These results are discussed in terms of the effectiveness of using functional behavioral assessment for designing interventions.

Writing is one of the primary skills that children learn in school. Interventions that address performance deficits and skill deficits have been shown to improve aspects of elementary school children's writing. This study demonstrates performance-based interventions (goal setting, feedback, and contingent reward) and a skill-based intervention (instruction) on the writing skills of a 10-year-old child. Results indicated that the performance intervention increased the number of correctly spelled words, and the combination of performance and instructional intervention increased the number of complete sentences.

Children with emotional and behavioral disorders (E/BD) struggle to achieve social and academic outcomes. Many studies have demonstrated self-management interventions to be effective at reducing problem behavior and increasing positive social and academic behaviors. Functional behavior assessment (FBA) information may be used in designing effective self-management interventions. The purpose of this study was to link self-management procedures to hypothesized behavior function in three children with E/BD. Results demonstrated that self-monitoring (SM) alone could be enhanced using information derived from FBA and that consequences delivered by teachers were less effective than a self-management treatment package.

Schools continue to strive for the use of evidenced-based interventions and policies to foster well-managed classrooms that promote improved student outcomes. The present study examined the effects of the Class-Wide Function-related Intervention Teams (CW-FIT), a group contingency intervention, on the on-task and disruptive behavior of elementary school students with or at risk for emotional behavior disorders (EBD). Seventeen elementary schools, 159 general education teachers, and 313 students participated in the randomized-control group design study. Fidelity of implementation was strong for intervention group teachers and was measured across groups and throughout baseline conditions. Results suggest that CW-FIT can be used to increase on-task behavior and reduce the disruptive behavior of students with or at risk for EBD. In addition, teachers in intervention classes increased praise and reduced reprimands to individual students and along with their students, reported high levels of consumer satisfaction.

The authors present an overview of how to implement the classwide function-based intervention team (CW-FIT) program. The CW-FIT program is a multilevel group contingency intervention that broadly addresses common functions of problem behavior. The CW-FIT is considered a classroom-level primary intervention because it is used with all students in a class. For students not responding to the primary-level intervention-group contingency, secondary-level or targeted interventions of self-management and help-card procedures are implemented. Students that do not respond to either primary or secondary intervention are candidates for a functional behavior assessment (tertiary level). The authors focus on describing the primary and secondary levels of CW-FIT. Results show that this intervention increases classwide time on on-task behavior during instruction and reduces disruptive behavior of students who are at risk for school failure.

The purpose of the study was to determine the efficacy of the Class-Wide Function-Related Intervention Teams (CW-FIT) program for improving students’ on-task behavior, and increasing teacher recognition of appropriate behavior. The intervention is a group contingency classroom management program consisting of teaching and reinforcing appropriate behaviors (i.e., getting the teacher’s attention, following directions, and ignoring inappropriate behaviors of peers). Seventeen elementary schools, the majority in urban and culturally diverse communities, participated in a randomized trial with 86 teachers (classrooms) assigned to CW-FIT, and 73 teachers (classrooms) assigned to the comparison group. Class-wide student on-task behavior improved over baseline levels in the intervention classes. Teachers were able to implement the intervention with high fidelity overall, as observed in adherence to 96% of the fidelity criteria on average. Teacher praise and attention to appropriate behaviors increased, and reprimands decreased. These effects were replicated in new classrooms each of the 4 years of the study, and for all years combined.

Writing is one of the primary skills that children learn in school. Interventions that address performance deficits and skill deficits have been shown to improve aspects of elementary school children's writing. This study demonstrates performance-based interventions (goal setting, feedback, and contingent reward) and a skill-based intervention (instruction) on the writing skills of a 10-year-old child. Results indicated that the performance intervention increased the number of correctly spelled words, and the combination of performance and instructional intervention increased the number of complete sentences.