Why reinvent the wheel? A behaviour analyst's reflections on pedagogy for inclusion for students with intellectual and developmental disability

The number of children identified as having intellectual or developmental disability is rising worldwide and their education has been found wanting. It has been said that “they simply need better teaching.” At the same time, there is an increasing evidence base that pedagogy that is based on the discipline of behaviour analysis offers the best prospect for individuals diagnosed with autism spectrum disorders. On the basis of this evidence, it is proposed that behaviour analysis should be applied more broadly to improve teaching for all children with intellectual or developmental disability.

Fetal brain function in response to maternal alcohol consumption: Early evidence of damage

Background: Studies of the adverse neurobehavioral effects of maternal alcohol consumption on the fetus have been largely confined to the postnatal period, after exposure to alcohol has finished. This study explored the brain function of the fetus, at the time of exposure to alcohol, to examine its effect on information processing and stability of performance. Methods: Five groups of fetuses, defined by maternal alcohol consumption patterns, were examined: control (no alcohol); moderate (5 to 10 units/wk either drunk evenly across the week or as a binge, in 2 to 3 days); heavy (20+ units/wk drunk evenly or as a binge). Fetal habituation performance was examined on 3 occasions, separated by 7 days, beginning at 35 weeks of gestation. The number of trials required to habituate on each test session and the difference in performance across test sessions were recorded. Results: Fetuses exposed to heavy binge drinking required significantly more trials to habituate and exhibited a greater variability in performance across all test sessions than the other groups. Maternal drinking, either heavily but evenly or moderately as a binge, resulted in poorer habituation, and moderate binge drinking resulted in greater variability compared with no, or even, drinking. Conclusions: Decreased information processing, reflected by poorer habituation, and increased variability in performance may reflect the initial manifestations of structural damage caused by alcohol to the brain. These results will lead to a greater understanding of the effects of alcohol on the fetus's brain, enable the antenatal identification of fetal alcohol spectrum disorders, and lead to the early implementation of better management strategies. © 2012 by the Research Society on Alcoholism.


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Spontaneous Attention to Faces in Asperger Sndrome using Ecologically Valid Static Stimuli

Previous eye tracking research on the allocation of attention to social information by individuals with autism spectrum disorders is equivocal and may be in part a consequence of variation in stimuli used between studies. The current study explored attention allocation to faces, and within faces,
by individuals with Asperger syndrome using a range of static stimuli where faces were either viewed in isolation or viewed in the context of a social scene. Results showed that faces were viewed typically by the individuals with Asperger syndrome when presented in isolation, but attention to the eyes was significantly diminished in comparison to age and IQ-matched typical viewers when faces were viewed as part of social scenes. We show that when using static stimuli, there is evidence of atypicality for individuals with Asperger syndrome depending on the extent of social context. Our findings shed light on the previous explanations of gaze behaviour that have emphasised the role of movement in atypicalities of social attention in autism spectrum disorders and highlight the importance of consideration of the realistic portrayal of social information for future studies.

Sensory-motor problems in Autism

Despite being largely characterised as a social and cognitive disorder, strong evidence indicates the presence of significant sensory-motor problems in Autism Spectrum Disorder (ASD). This paper outlines our progression from initial, broad assessment using the Movement Assessment Battery for Children (M-ABC) to subsequent targeted kinematic assessment. In particular, pronounced ASD impairment seen in the broad categories of manual dexterity and ball skills was found to be routed in specific difficulties on isolated tasks, which were translated into focused experimental assessment. Kinematic results from both subsequent studies highlight impaired use of perception-action coupling to guide, adapt and tailor movement to task demands, resulting in inflexible and rigid motor profiles. In particular difficulties with the use of temporal adaption are shown, with "hyperdexterity" witnessed in ballistic movement profiles, often at the cost of spatial accuracy and task performance. By linearly progressing from the use of a standardised assessment tool to targeted kinematic assessment, clear and defined links are drawn between measureable difficulties and underlying sensory-motor assessment. Results are specifically viewed in-light of perception-action coupling and its role in early infant development suggesting that rather than being 'secondary' level impairment, sensory-motor problems may be fundamental in the progression of ASD. This logical and systematic process thus allows a further understanding into the potential route of observable motor problems in ASD; a vital step if underlying motor problems are to be considered a fundamental aspect of autism and allow a route of non-invasive preliminary diagnosis.

Awareness and knowledge of autism and autism interventions: A general population survey.

Recent figures show that Autism Spectrum Disorder (ASD) affects at least 1 in 88 of the population, yet for years, international public awareness of ASD was limited. Over the past 5-10 years intense efforts have been made to raise autism awareness in the general population in countries such as UK and US. In this paper we report data from a large-scale general population survey (n=1204) in which we assessed autism awareness, knowledge about autism, and perceptions about autism interventions in Northern Ireland. We found high levels of autism awareness, in fact over 80% of the sample were aware of ASD and over 60% of these respondents knew someone with ASD in their own family, circle of friends or work colleagues. Generally, knowledge of strengths and challenges faced by individuals with ASD was relatively accurate. However, perceptions of interventions and service provider responsibilities were vague and uncertain. Results show that local and international autism awareness campaigns have largely been successful and that the focus should shift towards disseminating accurate information regarding intervention and service provider responsibilities.

Causal Models of Clinically Significant Behaviors in Angelman, Cornelia de Lange, Prader-Willi and Smith-Magenis Syndromes

The operant learning theory account of behaviors of clinical significance in people with intellectual disability (ID) has dominated the field for nearly 50 years. However, in the last two decades, there has been a substantial increase in published research that describes the behavioral phenotypes of genetic disorders and shows that behaviors such as self-injury and aggression are more common in some syndromes than might be expected given group characteristics. These cross-syndrome differences in prevalence warrant explanation, not least because this observation challenges an exclusively operant learning theory account. To explore this possible conflict between theoretical account and empirical observation, we describe the genetic cause and physical, social, cognitive and behavioral phenotypes of four disorders associated with ID (Angleman, Cornelia de Lange, Prader-Willi and Smith-Magenis syndromes) and focus on the behaviors of clinical significance in each syndrome. For each syndrome we then describe a model of the interactions between physical characteristics, cognitive and motivational endophenotypes and environmental factors (including operant reinforcement) to account for the resultant behavioral phenotype. In each syndrome it is possible to identify pathways from gene to physical phenotype to cognitive or motivational endophenotype to behavior to environment and back to behavior. We identify the implications of these models for responsive and early intervention and the challenges for research in this area. We identify a pressing need for meaningful dialog between different disciplines to construct better informed models that can incorporate all relevant and robust empirical evidence.

The Evidence Debate for Behavioural Interventions for Autism

For more than fifty years evidence has been accrued regarding the efficacy of applied behaviour analysis-based interventions for individuals with autism spectrum disorders. Despite this history of empirical evidence, some researchers and ASD experts still are reluctant to accept behavioral interventions as best practice for ASD. In this paper, we consider both random control trials and single subject experimental designs as forms of evidenced-based practice (EBP). Specific application of these methods to ASD research is considered. In an effort to provide scientifically based evidence for interventions for ASD, EBP standards have been debated without a consensus being achieved. Service users of ASD interventions need access to sound empirical evidence to choose appropriate programmes for those they care for with ASD rather than putting their hopes in therapies backed by pseudoscience and celebrity endorsements.

Attention during social interaction in children with autism: Comparison to specific language impairment, typical development, and links to cognition

Eye-tracking studies have shown how people with autism spend significantly less time looking at socially relevant information on-screen compared to those developing typically. This has been suggested to impact on the development of socio-cognitive skills in autism. We present novel evidence of how attention atypicalities in children with autism extend to real-life interaction, in comparison to typically developing (TD) children and children with specific language impairment (SLI). We explored the allocation of attention during social interaction with an interlocutor, and how aspects of attention (awareness checking) related to traditional measures of social cognition (false belief attribution). We found divergent attention allocation patterns across the groups in relation to social cognition ability. Even though children with autism and SLI performed similarly on the socio- cognitive tasks, there were syndrome-specific atypicalities of their attention patterns. Children with SLI were most similar to TD children in terms of prioritising attention to socially pertinent information (eyes, face, awareness checking). Children with autism showed reduced attention to the eyes and face, and slower awareness checking. This study provides unique and timely insight into real-world social gaze (a)typicality in autism, SLI and typical development, its relationship to socio-cognitive ability, and raises important issues for intervention.

Multidisciplinary Teamwork in Autism: Can One Size Fit All?

Multidisciplinary practice has become an accepted approach in many education and social and health care fields. In fact, the right to a multidisciplinary assessment is enshrined in the United Nations Convention of the Rights for Persons with Disabilities (United Nations, 2007). In order to avert a 'one size fits all' response to particularly heterogeneous diagnoses, such as autism spectrum disorders (ASD), the National Institute for Clinical Excellence (NICE) recommends multidisciplinary input. Yet, multidisciplinarity lacks empirical evidence of effectiveness, is fraught with conceptual difficulties and methodological incompatibilities, and therefore there is a danger of resorting to an ill-defined eclectic 'hodgepodge' of interventions. Virtually all evidence-based interventions in autism and intellectual disabilities are behaviourally based. Not surprisingly, therefore, professionals trained in behaviour analysis to international standards are increasingly becoming key personnel in multidisciplinary teams. In fact, professionals from a range of disciplines seek training in behaviour analysis. In this article we brought together a multidisciplinary group of professionals from education, health, and social care, most of whom have a dual qualification in an allied health, social care, or educational profession, as well as in behaviour anlaysis. Together we look at the initial training in these professions and explore how behaviour analysis can offer a common and coherent conceptual framework for true multidisciplinarity, based on sound scientific knowledge about behaviour, without resort to reifying theories. We illustrate how this unifying approach can enhance evidence-based multidisciplinary practice so that 'one size' will fit all. Copyright © Australian Psychological Society Ltd 2014.

Fast response to human voices in autism

Individuals with autism spectrum disorders (ASD) are reported to allocate less spontaneous attention to voices. Here, we investigated how vocal sounds are processed in ASD adults, when those sounds are attended. Participants were asked to react as fast as possible to target stimuli (either voices or strings) while ignoring distracting stimuli. Response times (RTs) were measured. Results showed that, similar to neurotypical (NT) adults, ASD adults were faster to recognize voices compared to strings. Surprisingly, ASD adults had even shorter RTs for voices than the NT adults, suggesting a faster voice recognition process. To investigate the acoustic underpinnings of this effect, we created auditory chimeras that retained only the temporal or the spectral features of voices. For the NT group, no RT advantage was found for the chimeras compared to strings: both sets of features had to be present to observe an RT advantage. However, for the ASD group, shorter RTs were observed for both chimeras. These observations indicate that the previously observed attentional deficit to voices in ASD individuals could be due to a failure to combine acoustic features, even though such features may be well represented at a sensory level.

Systematic Review of the Measurement Properties of Tools Used to Measure Behaviour Problems in Young Children with Autism

Background
Behaviour problems are common in young children with autism spectrum disorder (ASD). There are many different tools used to measure behavior problems but little is known about their validity for the population.

Objectives
To evaluate the measurement properties of behaviour problems tools used in evaluation of intervention or observational research studies with children with ASD up to the age of six years.

Methods
Behaviour measurement tools were identified as part of a larger, two stage, systematic review. First, sixteen major electronic databases, as well as grey literature and research registers were searched, and tools used listed and categorized. Second, using methodological filters, we searched for articles examining the measurement properties of the tools in use with young children with ASD in ERIC, MEDLINE, EMBASE, CINAHL, and PsycINFO. The quality of these papers was then evaluated using the COSMIN checklist.

Results
We identified twelve tools which had been used to measure behaviour problems in young children with ASD, and fifteen studies which investigated the measurement properties of six of these tools. There was no evidence available for the remaining six tools. Two questionnaires were found to be the most robust in their measurement properties, the Child Behavior Checklist and the Home Situations Questionnaire—Pervasive Developmental Disorders version.

Conclusions
We found patchy evidence on reliability and validity, for only a few of the tools used to measure behaviour problems in young children with ASD. More systematic research is required on measurement properties of tools for use in this population, in particular to establish responsiveness to change which is essential in measurement of outcomes of intervention.

PROSPERO Registration Number

CRD42012002223

Factors influencing success of clinical genome sequencing across a broad spectrum of disorders

To assess factors influencing the success of whole-genome sequencing for mainstream clinical diagnosis, we sequenced 217 individuals from 156 independent cases or families across a broad spectrum of disorders in whom previous screening had identified no pathogenic variants. We quantified the number of candidate variants identified using different strategies for variant calling, filtering, annotation and prioritization. We found that jointly calling variants across samples, filtering against both local and external databases, deploying multiple annotation tools and using familial transmission above biological plausibility contributed to accuracy. Overall, we identified disease-causing variants in 21% of cases, with the proportion increasing to 34% (23/68) for mendelian disorders and 57% (8/14) in family trios. We also discovered 32 potentially clinically actionable variants in 18 genes unrelated to the referral disorder, although only 4 were ultimately considered reportable. Our results demonstrate the value of genome sequencing for routine clinical diagnosis but also highlight many outstanding challenges.

Motor Skills in Children Aged 7–10 Years, Diagnosed with Autism Spectrum Disorder

This study used the Movement Assessment Battery for Children (M-ABC2) to assess the level of motor skill in children aged 7-10 years with autism (n=18) and compared their performance to two groups of age-matched typically developing children; a receptive vocabulary matched group (n=19) and a nonverbal IQ matched group (n=22). Although the results support previous work, as indicated by a significant general motor impairment in the group with autism, a sub-analysis of the M-ABC2 revealed that there were only 2 out of 8 subcomponent skills which showed universally significant specific deficits for the autism group; namely catching a ball and static balance. These results suggest that motor skill deficits associated with autism may not be pervasive but more apparent in activities demanding complex, interceptive actions or core balance ability.

Postural Control Deficits in Autism Spectrum Disorder: The Role of Sensory Integration

We investigated the nature of sensory integration deficits in postural control of young adults with ASD. Postural control was assessed in a fixed environment, and in three environments in which sensory information about body sway from visual, proprioceptive or both channels was inaccurate. Furthermore, two levels of inaccurate information were used within each channel (gain 1 and 1.6). ASD participants showed greater postural sway when information from proprioceptive and both channels were inaccurate. In addition, control participants' ellipse area at gain 1.6 was identical to ASD participants' at gain 1, reflecting hyper-reactivity in ASD. Our results provide evidence for hyper-reactivity in posture-related sensory information, which reflects a general, rather than channel-specific sensory integration impairment in ASD.

The spectrum of endogenous human chromogranin A-derived peptides identified using a modified proteomic strategy.

The hypothesis that chromogranin A (CgA), a protein of neuroendocrine cell secretory granules, may be a precursor of biologically active peptides, rests on observed activities of peptide fragments largely produced by exogenous protease digestion of the bovine protein. Here we have adopted a modified proteomic strategy to isolate and characterise human CgA-derived peptides produced by endogenous prohormone convertases. Initial focus was on an insulinoma as previous studies have shown that CgA is rapidly processed in pancreatic beta cells and that tumours arising from these express appropriate prohormone convertases. Eleven novel peptides were identified arising from processing at both monobasic and dibasic sites and processing was most evident in the C-terminal domain of the protein. Some of these peptides were identified in endocrine tumours, such as mid-gut carcinoid and phaeochromocytoma, which arise from endocrine cells of different phenotype and in different anatomical sites. Two of the most interesting peptides, GR-44 and ER-37, representing the C-terminal region of CgA, were found to be amidated. These data would imply that the intact protein is C-terminally amidated and that these peptides are probably biologically active. The spectrum of novel CgA-derived peptides, described in the present study, should provide a basis for biological evaluation of authentic entities.