Task-switching deficits and repetitive behaviour in genetic neurodevelopmental disorders: Data from children with Prader-Willi syndrome chromosome 15 q11-q13 deletion and boys with Fragile X syndrome

Prader-Willi syndrome (PWS) and Fragile X syndrome (FraX) are associated with distinctive cognitive and behavioural profiles. We examined whether repetitive behaviours in the two syndromes were associated with deficits in specific executive functions. PWS, FraX, and typically developing (TD) children were assessed for executive functioning using the Test of Everyday Attention for Children and an adapted Simon spatial interference task. Relative to the TD children, children with PWS and FraX showed greater costs of attention switching on the Simon task, but after controlling for intellectual ability, these switching deficits were only significant in the PWS group. Children with PWS and FraX also showed significantly increased preference for routine and differing profiles of other specific types of repetitive behaviours. A measure of switch cost from the Simon task was positively correlated to scores on preference for routine questionnaire items and was strongly associated with scores on other items relating to a preference for predictability. It is proposed that a deficit in attention switching is a component of the endophenotypes of both PWS and FraX and is associated with specific behaviours. This proposal is discussed in the context of neurocognitive pathways between genes and behaviour.

The relationship between the recognition of facial expressions and self-reported anger in people with intellectual disabilities

Background This study aims to examine the relationship between how individuals with intellectual disabilities report their own levels of anger, and the ability of those individuals to recognize emotions. It was hypothesized that increased expression of anger would be linked to lower ability to recognize facial emotional expressions and increased tendency to interpret facial expressions in a hostile or negative manner. It was also hypothesized increased levels of anger may lead to the altered perception of a particular emotion.

Method A cross-sectional survey design was used. Thirty participants completed a test of facial emotion recognition (FER), and a self-report anger inventory (Benson & Ivins 1992) as part of a structured interview.

Results Individuals with higher self-reported anger did not show significantly reduced performance in FER, or interpret facial expressions in a more hostile manner compared with individuals with less self-reported anger. However, they were less accurate in recognizing neutral facial emotions.

Conclusions It is tentatively suggested that individuals with high levels of anger may be likely to perceive emotional content in a neutral facial expression because of their high levels of emotional arousal.

The presence of a culturally similar or dissimilar social partner affects neural responses to emotional stimuli

Background: Emotional responding is sensitive to social context; however, little emphasis has been placed on the mechanisms by which social context effects changes in emotional responding.

Objective: We aimed to investigate the effects of social context on neural responses to emotional stimuli to inform on the mechanisms underpinning context-linked changes in emotional responding.

Design: We measured event-related potential (ERP) components known to index specific emotion processes and self-reports of explicit emotion regulation strategies and emotional arousal. Female Chinese university students observed positive, negative, and neutral photographs, whilst alone or accompanied by a culturally similar (Chinese) or dissimilar researcher (British).

Results: There was a reduction in the positive versus neutral differential N1 amplitude (indexing attentional capture by positive stimuli) in the dissimilar relative to alone context. In this context, there was also a corresponding increase in amplitude of a frontal late positive potential (LPP) component (indexing engagement of cognitive control resources). In the similar relative to alone context, these effects on differential N1 and frontal LPP amplitudes were less pronounced, but there was an additional decrease in the amplitude of a parietal LPP component (indexing motivational relevance) in response to positive stimuli. In response to negative stimuli, the differential N1 component was increased in the similar relative to dissimilar and alone (trend) context.

Conclusion: These data suggest that neural processes engaged in response to emotional stimuli are modulated by social context. Possible mechanisms for the social-context-linked changes in attentional capture by emotional stimuli include a context-directed modulation of the focus of attention, or an altered interpretation of the emotional stimuli based on additional information proportioned by the context.

Further refinement of the nature of the communication impairment in Cornelia de Lange syndrome

Purpose – Research into the communication skills of individuals with Cornelia de Lange syndrome (CdLS) is extremely limited. This paper aims to evaluate the nature of these skills and impairments in CdLS using a detailed informant assessment of pre-verbal communication skills.
Design/methodology/approach – The study used the Pre-verbal Communication Schedule to evaluate communication skills in individuals with CdLS (n ¼ 14), aged five to14 years. The group was compared with a contrast group of individuals with Cri du Chat syndrome (CdCS; n ¼ 14) who were matched for age and intellectual ability.
Findings – A significant difference was identified in understanding non-vocal communication (p , 0.005), with the CdLS group showing a greater deficit. These findings indicate the presence of a syndrome-specific deficit in understanding non-verbal communication in individuals with CdLS and suggest that there may be a dissociation between the processing of verbal and non-verbal communication.
Originality/value – The findings indicate that, in many ways, these two syndrome groups are not dissimilar in terms of their communication skills. However, individuals with CdLS show a syndrome-specific deficit in understanding non-vocal communication relative to the CdCS group.

Work-worlds colliding: Self-reflexivity, power and emotion in organizational ethnography

While organizational ethnographers have embraced the concept of self-reflexivity, problems remain. In this article we argue that the prevalent assumption that self-reflexivity is the sole responsibility of the individual researcher limits its scope for understanding organizations. To address this, we propose an innovative method of collective reflection that is inspired by ideas from cultural and feminist anthropology. The value of this method is illustrated through an analysis of two ethnographic case studies, involving a ‘pair interview’ method. This collective approach surfaced self-reflexive accounts, in which aspects of the research encounter that still tend to be downplayed within organizational ethnographies, including emotion, intersubjectivity and the operation of power dynamics, were allowed to emerge. The approach also facilitated a second contribution through the conceptualization of organizational ethnography as a unique endeavour that represents a collision between one ‘world of work’: the university, with a second: the researched organization. We find that this ‘collision’ exacerbates the emotionality of ethnographic research, highlighting the refusal of ‘researched’ organizations to be domesticated by the specific norms of academia. Our article concludes by drawing out implications for the practice of self-reflexivity within organizational ethnography.

The progenitor mass of the Type IIP supernova SN 2004et from late-time spectral modeling

SN 2004et is one of the nearest and best-observed Type IIP supernovae, with a progenitor detection as well as good photometric and spectroscopic observational coverage well into the nebular phase. Based on nucleosynthesis from stellar evolution/explosion models we apply spectral modeling to analyze its 140-700 day evolution from ultraviolet to mid-infrared. We find a M_ZAMS= 15 Msun progenitor star (with an oxygen mass of 0.8 Msun) to satisfactorily reproduce [O I] 6300, 6364 {\AA} and other emission lines of carbon, sodium, magnesium, and silicon, while 12 Msun and 19 Msun models under- and overproduce most of these lines, respectively. This result is in fair agreement with the mass derived from the progenitor detection, but in disagreement with hydrodynamical modeling of the early-time light curve. From modeling of the mid-infrared iron-group emission lines, we determine the density of the "Ni-bubble" to rho(t) = 7E-14*(t/100d)^-3 g cm^-3, corresponding to a filling factor of f = 0.15 in the metal core region (V = 1800 km/s). We also confirm that silicate dust, CO, and SiO emission are all present in the spectra.

Interferon regulatory factor (IRF) 3 is critical for the development of experimental autoimmune encephalomyelitis.

BACKGROUND: Experimental autoimmune encephalomyelitis (EAE) is an animal model of autoimmune inflammatory demyelination that is mediated by Th1 and Th17 cells. The transcription factor interferon regulatory factor 3 (IRF3) is activated by pathogen recognition receptors and induces interferon-beta production.

METHODS: To determine the role of IRF3 in autoimmune inflammation, we immunised wild-type (WT) and irf3-/- mice to induce EAE. Splenocytes from WT and irf3-/- mice were also activated in vitro in Th17-polarising conditions.

RESULTS: Clinical signs of disease were significantly lower in mice lacking IRF3, with reduced Th1 and Th17 cells in the central nervous system. Peripheral T-cell responses were also diminished, including impaired proliferation and Th17 development in irf3-/- mice. Myelin-reactive CD4+ cells lacking IRF3 completely failed to transfer EAE in Th17-polarised models as did WT cells transferred into irf3-/- recipients. Furthermore, IRF3 deficiency in non-CD4+ cells conferred impairment of Th17 development in antigen-activated cultures.

CONCLUSION: These data show that IRF3 plays a crucial role in development of Th17 responses and EAE and warrants investigation in human multiple sclerosis.