Social Function and Autism Spectrum Disorder in Children and Adults with Neurofibromatosis Type 1: a Systematic Review and Meta-Analysis

In light of the proliferation of recent research into social function in neurofibromatosis type 1 (NF1), a systematic review and meta-analysis is required to synthesise data and place findings within the context of a theoretical framework. This paper reviews findings from research into social function and autism spectrum disorder (ASD) in children and adults with NF1 and integrates these findings with the Socio-Cognitive Integration Abilities Model (SOCIAL). It also critically appraises links between social outcomes, internal and external factors moderating social functioning, cognitive domains implicated in social functioning, and underlying neural pathology in NF1. A systematic literature search conducted in MedLine (Ovid), PsycINFO (Ovid), Embase (Ovid), and PubMed electronic databases yielded 35 papers that met inclusion criteria for the systematic review. Out of these papers, 22 papers provided sufficient data for meta-analysis. Findings from this review and meta-analysis provide evidence that children and adults with NF1 exhibit significantly higher prevalence and severity of social dysfunction and ASD symptomatology. To date, very few studies have examined social cognition in NF1 but results indicate the presence of both perceptual and higher-level impairments in this population. The results of this review also provide support for age, gender, and comorbid ADHD as moderating factors for social outcomes in NF1. Suggestions for future research are offered to further our understanding of the social phenotype in NF1 and to facilitate the development of targeted interventions.     

A “spoon full of sugar” helps the medicine go down: How a participant friendly version of a psychophysics task significantly improves task engagement,performance and data quality in a typical adult sample

Few would argue that the unique insights brought by studying the typical and atypical development of psychological processes are essential to building a comprehensive understanding of the brain. Often, however, the associated challenges of working with non-standard adult populations results in the more complex psychophysical paradigms being rejected as too complex. Recently we created a child- (and clinical group) friendly implementation of one such technique – the reverse-correlation Bubbles approach – and noted an associated performance boost in adult participants. Here, we compare the administration of three different versions of this participant-friendly task in the same adult participants to empirically confirm that introducing elements in the experiment with the sole purpose of improving the participant experience, not only boosts the participant’s engagement and motivation for the task but results in a significantly improved objective task performance and stronger statistical results.     

Rapid communication: Sex differences in artificial grammar learning: evidence for different strategies in men and women

In this article we report an experiment that investigated differences in classification performance of women and men in artificial grammar learning (AGL). Women's and men's responses correspond to a large extent. Consistent differences, however, were found in a variable that codes overlaps between training and test items in terms of string fragments. The results are best explained by the assumption that women and men apply different cognitive strategies at test.     

Strategic use of familiarity in display-based problem solving

The authors report 4 experiments that investigated the role of recognition memory and plausibility in a display-based problem-solving task (computer menu search). It was found that both the familiarity of options and their plausibility played a role in determining choices when the correct options could not be recollected. The use of familiarity was adaptive: Participants relied less on familiarity when it was a less valid guide to correct choices. The implications of these findings for theories of problem solving and learning are discussed.     

The influence of depth segmentation on colour constancy

In real scenes, surfaces in different depth planes often differ in the luminance and chromatic content of their illumination. Scene segmentation is therefore an important issue when considering the compensation of illumination changes in our visual perception (lightness and colour constancy). Chromatic adaptation is an important sensory component of colour constancy and has been shown to be linked to the two-dimensional spatial structure of a scene (Werner, 2003 Vision Research 43 1611 - 1623). Here, the question is posed whether this cooperation also extends to the organisation of a scene in depth. The influence of depth on colour constancy was tested by introducing stereo disparity, whereby the test patch and background were perceived in either the same or one of five different depth planes (1.9-57 min of arc). There were no additional cues to depth such as shadows or specular highlights. For consistent illumination changes, colour constancy was reduced when the test patch and background were separated in depth, indicating a reduction of contextual influences. An interaction was found between the influences of stereo depth and spatial frequency on colour constancy. In the case of an inconsistent illumination change, colour constancy was reduced if the test patch and background were in the same depth plane (2-D condition), but not if they were separated in depth (3-D condition). Furthermore, colour constancy was slightly better in the 3-D inconsistent condition than in the 2-D inconsistent condition. It is concluded that depth segmentation supports colour constancy in scenes with inconsistent illumination changes. Processes of depth segmentation are implemented at an early sensory stage of colour constancy, and they define visual regions within which the effects of illuminant changes are discounted for separately. The results support recent models that posit such implementation of scene segmentation in colour constancy.     

Semantic ambiguity and the failure of inhibition hypothesis as an explanation for reading errors in deep dyslexia

We report evidence for dissociation between explicit and implicit access to word representations in a deep dyslexic patient (JO). JO read aloud a series of ambiguous (e.g., bank) and unambiguous (e.g., food) words and performed a lexical decision task using these same items. When required to explicitly access the items (i.e., naming), JO showed relative impairment for ambiguous compared with unambiguous words. In contrast, the same stimuli produced an advantage for ambiguous words in lexical decision. The results are discussed within a framework of deep dyslexia that considers errors in production to arise through a failure to inhibit spuriously activated candidate representations.     

Dissociating the effects of attention and contingency awareness on evaluative conditioning effects in the visual paradigm

Two experiments are described that investigate the effects of attention in moderating evaluative conditioning (EC) effects in a picture-picture paradigm in which previously discovered experimental artifacts (e.g., Field & Davey, 1999 ) were overcome by counterbalancing conditioned stimuli (CSs) and unconditioned stimuli (USs) across participants. Conditioned responses for individuals who had attention enhanced were compared against a control group and groups for whom attention was impeded using a distracter task. In a second experiment the effects of attention were dissociated from those of contingency awareness by using backward-masked US presentations. The results of these experiments indicate that although associative EC effects may not be disrupted by a lack of contingency awareness, attention is an important factor in establishing conditioning. These results shed some light onto the possible boundary conditions that could explain past inconsistencies in obtaining EC effects in the visual paradigm.     

Deep dyslexia and semantic errors: a test of the failure of inhibition hypothesis using a semantic blocking paradigm

Deep dyslexia is an acquired reading disorder that involves the production of semantic errors and the inability to read aloud nonwords successfully. Several explanations for this reading impairment posit multiple loci of damage to account for the various error types produced in deep dyslexia. In contrast, the failure of inhibition hypothesis suggests that damage in the phonological output lexicon alone can explain these errors. Specifically, this hypothesis proposes normal processing via orthographic and phonological reading routes, as well as an intact semantic system. However, slowed or reduced inhibitory connections result in the failure to suppress spuriously activated neighbours in the phonological output lexicon, where neighbourhood can be defined in terms of phonology, orthography, or semantics. Given a failure to inhibit semantically related candidates, semantic reading errors occur. Important to the test of this hypothesis is that it evolves several predictions that are contrary to performance observed in the normal population. In particular, semantic errors are predicted to be greater in conditions where words are blocked according to semantic category than in random presentations. In addition, a semantic interference effect is expected. The results of semantic blocking were consistent with these predictions and lend support to the failure of inhibition hypothesis.     

Speculation about behavior, brain damage, and self-organization: the other way to herd a cat

This article contrasts aphasic patients' performance of word naming and lexical decision with that of intact college-aged readers. We discuss this contrast within a framework of self-organization; word recognition by aphasic patients is destabilized relative to intact performance. Less stable performance shows itself as an increase in the dispersion of patients' response times compared to college students'. Dispersion is also more pronounced for low-frequency words than for high frequency words. We speculate, that increased dispersion originates in a reduction of constraints that support naming and lexical decision performances. A sufficient reduction of constraints yields qualitative changes in performance such as the production of semantic errors in deep dyslexia. These hypotheses are offered as alternatives to postulating distinct modules.