Processing Deficits Research Articles

Impaired speech input and output processing abilities in children with cleft palate speech disorder.

Cleft lip and palate is one of the most common oral and maxillofacial deformities associated with a variety of functional disorders. Cleft palate speech disorder (CPSD) occurs the most frequently and manifests a series of characteristic speech features, which are called cleft speech characteristics. Some scholars believe that children with CPSD and poor speech outcomes may also have weaknesses in speech input processing ability, but evidence is still lacking so far. (1) To explore whether children with CPSD and speech output disorders also have defects in speech input processing abilities; (2) to explore the correlation between speech input and output processing abilities. Children in the experimental group were enrolled from Beijing Stomatological Hospital, Capital Medical University, and healthy volunteers were recruited as controls. Then three tasks containing real and pseudo words were performed sequentially. Reaction time, accuracy and other indicators in three tasks were collected and then analysed. The indicators in the experimental group were significantly lower than those in the control group. There was a strong correlation between speech input and output processing tasks. The performance of both groups when processing pseudo words in the three tasks was worse than that when dealing with real words. Compared with normal controls, children with CPSD have deficits in both speech input and output processing, and there is a strong correlation between speech input and output speech processing abilities. In addition, the pseudo words task was more challenging than the real word task for both groups. What is already known on the subject Children with cleft lip and palate often have speech sound disorders known as cleft palate speech disorder (CPSD). CPSD is characterised by consonant errors called cleft speech characteristics, which can persist even after surgery. Some studies suggest that poor speech outcomes in children with CPSD may be associated with deficits in processing speech input. However, this has not been validated in mainland China. What this paper adds to existing knowledge The results of our study indicate that children with CPSD exhibit poorer performance in three tasks assessing speech input and output abilities compared to healthy controls, suggesting their deficits in both speech input and output processing. Furthermore, a significant correlation was observed between speech input and output processing abilities. Additionally, both groups demonstrated greater difficulty in processing pseudo words compared to real words, as evidenced by their worse performance in dealing with pseudo words. What are the potential or actual clinical implications of this work? The pseudo word tasks designed and implemented in our study can be employed in future research and assessment of speech input and output abilities in Chinese Mandarin children with CPSD. Additionally, our findings revealed the significance of considering both speech output processing abilities and potential existence of speech input processing ability for speech and language therapists when evaluating and developing treatment options for children with CPSD as these abilities are also important for the development of literacy development.

The effect of social anxiety on threat acquisition and extinction: a systematic review and meta-analysis.

Although exposure-based therapy has been found to be effective at alleviating symptoms of social anxiety disorder, it often does not lead to full remission, and relapse after treatment is common. Exposure therapy is based on theoretical principles of extinction of conditioned fear responses. However, there are inconsistencies in findings across experiments that have investigated the effect of social anxiety on threat conditioning and extinction processes. This systematic review and meta-analysis aimed to examine whether elevated levels of social anxiety are associated with abnormalities in threat conditioning and extinction processes. A second aim was to examine the sensitivity of various study designs and characteristics to detect social anxiety-related differences in threat conditioning and extinction. A systematic search was conducted, which identified twenty-three experiments for inclusion in the review. The findings did not demonstrate compelling evidence that high levels of social anxiety are associated with atypical threat conditioning or extinction. Further, when systematically examining the data, there was no convincing support that the use of a particular psychophysiological measure, subjective rating, or experimental parameter yields more consistent associations between social anxiety and conditioning processes during threat acquisition or extinction. Meta-analyses demonstrated that during threat extinction, the use of anxiety ratings as a dependent variable, socially relevant unconditioned stimuli, and a higher reinforcement schedule produced more detectable effects of social anxiety on compromised extinction processes compared to any other dependent variable (subjective or physiological) or experimental parameter. Overall, the results of this study suggest that social anxiety is not reliably related to deficits in conditioning and extinction processes in the context of laboratory-based Pavlovian conditioning paradigms.

Sex differences during development in cortical temporal processing and event related potentials in wild-type and fragile X syndrome model mice

BackgroundAutism spectrum disorder (ASD) is currently diagnosed in approximately 1 in 44 children in the United States, based on a wide array of symptoms, including sensory dysfunction and abnormal language development. Boys are diagnosed ~ 3.8 times more frequently than girls. Auditory temporal processing is crucial for speech recognition and language development. Abnormal development of temporal processing may account for ASD language impairments. Sex differences in the development of temporal processing may underlie the differences in language outcomes in male and female children with ASD. To understand mechanisms of potential sex differences in temporal processing requires a preclinical model. However, there are no studies that have addressed sex differences in temporal processing across development in any animal model of ASD.MethodsTo fill this major gap, we compared the development of auditory temporal processing in male and female wildtype (WT) and Fmr1 knock-out (KO) mice, a model of Fragile X Syndrome (FXS), a leading genetic cause of ASD-associated behaviors. Using epidural screw electrodes, we recorded auditory event related potentials (ERP) and auditory temporal processing with a gap-in-noise auditory steady state response (ASSR) paradigm at young (postnatal (p)21 and p30) and adult (p60) ages from both auditory and frontal cortices of awake, freely moving mice.ResultsThe results show that ERP amplitudes were enhanced in both sexes of Fmr1 KO mice across development compared to WT counterparts, with greater enhancement in adult female than adult male KO mice. Gap-ASSR deficits were seen in the frontal, but not auditory, cortex in early development (p21) in female KO mice. Unlike male KO mice, female KO mice show WT-like temporal processing at p30. There were no temporal processing deficits in the adult mice of both sexes.ConclusionsThese results show a sex difference in the developmental trajectories of temporal processing and hypersensitive responses in Fmr1 KO mice. Male KO mice show slower maturation of temporal processing than females. Female KO mice show stronger hypersensitive responses than males later in development. The differences in maturation rates of temporal processing and hypersensitive responses during various critical periods of development may lead to sex differences in language function, arousal and anxiety in FXS.

Early childhood trauma exposure and neurocognitive and emotional processes: Associations in young children in a partial hospital program.

Early childhood trauma has been linked to neurocognitive and emotional processing deficits in older children, yet much less is known about these associations in young children. Early childhood is an important developmental period in which to examine relations between trauma and executive functioning/emotion reactivity, given that these capacities are rapidly developing and are potential transdiagnostic factors implicated in the development of psychopathology. This cross-sectional study examined associations between cumulative trauma, interpersonal trauma, and components of executive functioning, episodic memory, and emotion reactivity, conceptualized using the RDoC framework and assessed with observational and performance-based measures, in a sample of 90 children (ages 4-7) admitted to a partial hospital program. Children who had experienced two or more categories of trauma had lower scores in episodic memory, global cognition, and inhibitory control as measured in a relational (but not computerized) task, when compared to children with less or no trauma. Interpersonal trauma was similarly associated with global cognition and relational inhibitory control. Family contextual factors did not moderate associations. Findings support examining inhibitory control in both relationally significant and decontextualized paradigms in early childhood, and underscore the importance of investigating multiple neurocognitive and emotional processes simultaneously to identify potential targets for early intervention.

The role of emotional factors in face processing abilities in autism spectrum conditions

Facial emotion recognition is considered atypical in individuals with autism spectrum conditions (ASC), but emotion recognition abilities vary widely in autistic people, and there are inconsistent findings on the causes of these differences. Research indicates alexithymia may result in facial emotion recognition differences in ASC. Alternatively, mood disorders have been linked to atypical facial emotional expression recognition abilities in neurotypical adults. Investigating both the effects of alexithymia and mood disorders (depression and anxiety) is necessary to establish which of these factors may cause atypical facial emotion recognition in ASC. This study aimed to examine whether alexithymia or mood disorder symptomology is a predictor of atypical facial emotion recognition in individuals with ASC. Ninety-eight non-autistic adults and 80 autistic adults were recruited. Participants completed an online facial processing task to examine emotion and identity recognition abilities, the AQ-28, the TAS-20, and the HADS to measure autism severity, alexithymia symptoms, and depression and anxiety symptoms. Regression-based analyses found that autistic traits and autistic group membership did not predict facial emotion processing abilities after accounting for demographic variables, alexithymia and mood disorders: however, neither alexithymia nor mood disorder symptoms predicted variance in face processing abilities either. Our results concur with previous meta-analyses of facial emotion processing in autism spectrum disorder which report that studies do not always report deficits in face processing in autism: our findings are also not supportive of the model that argues that alexithymia explains facial emotion processing difficulties in autism.

Alexithymia in primary ciliary dyskinesia.

Recent evidence suggests that alexithymic deficits in emotional processing may also affect physical health, and alexithymia may also be associated with organic disorders. The emotional well-being of patients with primary ciliary dyskinesia (PCD) is often negatively affected by uncertainty about the prognosis, lack of ongoing medical care, and lack of symptom control. This study aims to evaluate the frequency of alexithymia and its possible impact on the management of childhood PCD. Subjects were recruited from patients with PCD and healthy volunteers aged 8-18 years. The questionnaire included sociodemographic characteristics and self-report scales. Data were compared between patient and control groups. In the >14 years of age group, the total Toronto Alexithymia Scale (TAS-20) score was significantly higher in the patients (56.60 ± 13.01) compared to the control group (46.47 ± 7.50) (p = .007). There were 6 (30) patients with a TAS-20 score ≥61. There was a significant correlation between TAS-20 and Pediatric Quality of Life (PedsQL) score (child), but no correlation between TAS-20 and Kovacs Children Depression Inventory (CDI) and PedsQL score (parents). The TAS-20 score was significantly higher in patients with bronchiectasis (p = .035), nasal polyps (p = .045), and siblings with PCD (p = .001). Furthermore, the TAS-20 score had a significant negative correlation with pulmonary function tests. Although this study is based on limited data from a single center and cannot be generalized to all PCD patient communities, our results show that PCD patients are more likely to have alexithymia compared to healthy controls and highlight the need to evaluate for alexithymia in patients with PCD. It is important for pediatric pulmonologists to have a thorough knowledge of the alexithymic features associated with PCD and to refer patients to pediatric psychiatry when necessary, especially in patients who are noncompliant with treatment protocols.

Lower resting state functional connectivity partially mediates adverse effects of prenatal alcohol exposure on arithmetic performance in children.

Fetal alcohol spectrum disorders (FASD) include a range of neurocognitive and behavioral impairments resulting from prenatal alcohol exposure (PAE). Among the PAE-related cognitive deficits, number processing is particularly affected. This study examines alterations in number processing networks and whether changes in functional connectivity mediate the adverse effects of PAE on arithmetic performance. Magnetic resonance imaging (MRI) was acquired in 57 children (mean (SD) age = 11.3 (+0.9) yr), 38 with FASD (19 fetal alcohol syndrome (FAS) or partial FAS (PFAS), 19 heavily exposed (HE)) and 19 controls. Whole-brain correlation analyses were performed from five seeds located in regions involved in number processing. Children with FAS/PFAS showed dose-dependent reductions in resting state functional connectivity between the seed in the right (R) posterior superior parietal lobule and a cluster in the left (L) inferior frontal gyrus, and between a seed in the R horizontal intraparietal sulcus and clusters in the R precentral gyrus and L cerebellar lobule VI. HE children showed lower resting state functional connectivity in a subset of these regions. Lower functional connectivity in the two fronto-parietal connections partially mediated the adverse effects of PAE on arithmetic performance. This study demonstrates PAE-related functional connectivity impairments in functional networks involved in number processing. The weaker connectivity between the R posterior superior parietal lobule and the L inferior frontal gyrus suggests that impaired verbal processing and visuospatial working memory may play a role in number processing deficits, while weaker connectivity between the R intraparietal sulcus and the R precentral gyrus points to poorer finger-based numerical representation, which has been linked to arithmetic computational skills.

Experimental evidence for a robust, transdiagnostic marker in functional disorders: Erroneous sensorimotor processing in functional dizziness and functional movement disorder

ObjectiveRecent neuroscientific models suggest that functional bodily symptoms can be attributed to perceptual dysregulation in the central nervous system. Evidence for this hypothesis comes from patients with functional dizziness, who exhibit marked sensorimotor processing deficits during eye-head movement planning and execution. Similar findings in eye-head movement planning in patients with irritable bowel syndrome confirmed that these sensorimotor processing deficits represent a shared, transdiagnostic mechanism. We now examine whether erroneous sensorimotor processing is also at play in functional movement disorder. MethodsWe measured head movements of 10 patients with functional movement disorder (F44.4, ICD-10), 10 patients with functional dizziness (F45.8, ICD-10), and (respectively) 10 healthy controls during an eye-head experiment, where participants performed large gaze shifts under normal, increased, and again normal head moment of inertia. Head oscillations at the end of the gaze shift served as a well-established marker for sensorimotor processing problems. We calculated Bayesian statistics for comparison. ResultsPatients with functional movement disorder (Bayes Factor (BF)10 = 5.36, BFincl = 11.16; substantial to strong evidence) as well as patients with functional dizziness (BF10 = 2.27, BFincl = 3.56; anecdotal to substantial evidence) showed increased head oscillations compared to healthy controls, indicating marked deficits in planning and executing movement. ConclusionWe replicate earlier experimental findings on erroneous sensorimotor processing in patients with functional dizziness, and show that patients with functional movement disorder show a similar impairment of sensorimotor processing during large gaze shifts. This provides an objectively measurable, transdiagnostic marker for functional disorders, highlighting important implications for diagnosis, treatment, and de-stigmatization.

How Hospitals Differentiate Health Information Technology Portfolios for Clinical Care Efficiency: Insights from the HITECH Act

Our research reveals the significant impact of evolving policy uncertainty on hospital strategies to mitigate cost-based performance deficits in clinical care processes through differentiation in search for health information technology (HIT). Key for hospital administrators and managers, our findings reveal the benefit of leveraging external benchmarks for performance feedback, enabling strategic, innovative approaches to HIT investments tailored to enhance clinical care efficiency and cost-effectiveness. Moreover, our findings have important implications for healthcare policymakers, highlighting the necessity to consider the varied responses of hospitals to policy fluctuations. Recognizing that hospitals proactively adapt their HIT portfolios in anticipation of new laws, and that these adaptations are most diverse under moderate policy uncertainty, is crucial. This nuanced understanding can guide policymakers in fostering an environment that encourages both the exploration of novel technologies and the widespread adoption of effective solutions across the healthcare spectrum. By bridging the gap between policy development and hospital administration, our work offers a road map for aligning strategic technological differentiation with policy objectives, ultimately enhancing healthcare delivery and outcomes.

The relationship between numerical magnitude processing and math anxiety, and their joint effect on adult math performance, varied by indicators of numerical tasks.

According to the hypothesis of Maloney et al. (Cognition 114(2):293-297, 2010. https://doi.org/10.1016/j.cognition.2009.09.013), math anxiety is related to deficits in numerical magnitude processing, which in turn compromises the development of advanced math skills. Because previous studies on this topic are contradictory, which may be due to methodological differences in the measurement of numerical magnitude processing, we tested Maloney et al.'s hypothesis using different tasks and their indicators: numerical magnitude processing (symbolic and non-symbolic comparison tasks: accuracy, reaction time, numerical ratio, distance and size effects, and Weber fraction; number line estimation task: estimation error), math anxiety (combined scores of learning, testing, math problem solving, and general math anxiety), and math performance. The results of our study conducted on 119 young adults mostly support the hypothesis proposed by Maloney et al. that deficiency in symbolic magnitude processing is related to math anxiety, but the relationship between non-symbolic processes and math anxiety was opposite to the assumptions. Moreover, the results indicate that estimation processes (but not comparison processes) and math anxiety are related to math performance in adults. Finally, high math anxiety moderated the relationship between reaction time in the symbolic comparison task, reaction time in the non-symbolic comparison task, numerical ratio effect in the symbolic comparison task, and math performance. Because the results of the joint effect of numerical magnitude processing and math anxiety on math performance were inconsistent, this part of the hypothesis is called into question.

Topography and ensemble activity in auditory cortex of a mouse model of Fragile-X-Syndrome

Autism spectrum disorder (ASD) is often associated with social communication impairments and specific sound processing deficits, for example problems in following speech in noisy environments. To investigate underlying neuronal processing defects located in the auditory neocortex (AC), we performed two-photon Ca2+imaging inFMR1(Fragile X Messenger Ribonucleoprotein1) knockout (KO) mice, a model for Fragile-X-Syndrome (FXS), the most common cause of hereditary ASD in humans. For primary AC (A1) and the anterior auditory field (AAF), topographic frequency representation was less ordered compared to control animals. We additionally analyzed ensemble AC activity in response to various sounds and found subfield-specific differences. In A1, ensemble correlations were lower in general, while in secondary AC (A2), correlations were higher in response to complex sounds, yet not to pure tones (PT). Furthermore, sound specificity of ensemble activity was decreased in AAF. Repeating these experiments one week later revealed no major differences regarding representational drift. Nevertheless, we found subfield- and genotype-specific changes in ensemble correlation values between the two times points, hinting at alterations in network stability inFMR1KO mice. These detailed insights into AC networks activity and topography inFMR1KO mice add to the understanding of auditory processing defects in FXS.Significance statementCommunicative challenges often observed in people with autism spectrum disorder might be due to defects in cortical brain circuits responsible for sound analysis. To investigate these in detail, we used a mouse model of Fragile-X-Syndrome, which often is associated with autism spectrum disorder in humans. We found several alterations compared to control animals, including a less well-ordered topography of frequency analysis in auditory cortex. Furthermore, neuronal population activity patterns in response to various sounds were altered. This was also highly dependent on whether pure tones or complex sounds were presented. These data help to understand the causes of sound processing defects in Fragile-X-Syndrome.

Development and validation of a nonverbal consensus-based semantic memory paradigm in patients with epilepsy.

Brain areas implicated in semantic memory can be damaged in patients with epilepsy (PWE). However, it is challenging to delineate semantic processing deficits from acoustic, linguistic, and other verbal aspects in current neuropsychological assessments. We developed a new Visual-based Semantic Association Task (ViSAT) to evaluate nonverbal semantic processing in PWE. The ViSAT was adapted from similar predecessors (Pyramids & Palm Trees test, PPT; Camels & Cactus Test, CCT) comprised of 100 unique trials using real-life color pictures that avoid demographic, cultural, and other potential confounds. We obtained performance data from 23 PWE participants and 24 control participants (Control), along with crowdsourced normative data from 54 Amazon Mechanical Turk (Mturk) workers. ViSAT reached a consensus >90% in 91.3% of trials compared to 83.6% in PPT and 82.9% in CCT. A deep learning model demonstrated that visual features of the stimulus images (color, shape; i.e., non-semantic) did not influence top answer choices (p = 0.577). The PWE group had lower accuracy than the Control group (p = 0.019). PWE had longer response times than the Control group in general and this was augmented for the semantic processing (trial answer) stage (both p < 0.001). This study demonstrated performance impairments in PWE that may reflect dysfunction of nonverbal semantic memory circuits, such as seizure onset zones overlapping with key semantic regions (e.g., anterior temporal lobe). The ViSAT paradigm avoids confounds, is repeatable/longitudinal, captures behavioral data, and is open-source, thus we propose it as a strong alternative for clinical and research assessment of nonverbal semantic memory.

Early cognitive predictors of spelling and reading in German-speaking children

Theoretical backgroundWhile reading and spelling skills often are interconnected in models of literacy development, recent research suggests that the two skills can dissociate and that reading and spelling are associated with at least partly different cognitive predictors. However, previous research on dissociations between reading and spelling skills focused on children who have already mastered the first phases of literacy development. These findings suggest that dissociations are due to distinct deficits in orthographic processing (i.e., unprecise orthographic representations vs. inefficient serial processing). It is therefore unclear whether dissociations already become apparent during the initial stages, or rather emerge later in development. This study aims to enhance the understanding of the predictors of early spelling and reading skills, investigating potential variations, by considering various cognitive factors beyond well-established ones.MethodsData were collected at two time points: cognitive predictors and early reading and spelling skills were assessed at the end of kindergarten (T1) before formal literacy instruction started, and reading and spelling skills were again assessed in Grade 1 (T2). The data analysis included 353 first-grade participants. Linear regression analyses assessed predictive patterns, while logistic regression analyses explained children's likelihood of belonging to different proficiency groups (at-risk or typical skills).ResultsResults revealed phonological processing, letter knowledge, and intelligence, as significant predictors for Spelling in grade 1 (T2), even after adding the autoregressor (Spelling in kindergarten at T1) and the respective other literacy skill (Reading T2). For Reading in grade 1 (T2), phonological processing, and rapid automatized naming (RAN) surfaced as significant predictors after adding the autoregressor (Reading T1). However, only RAN surfaced as a significant predictor for Reading T2 after adding the respective other literacy skill (Spelling T2). In line with these findings, logistic regression analyses revealed that phonological processing predicted group allocation for Spelling T2 and RAN predicted group allocation for Reading T2.ConclusionsOverall, the study underscores the importance of phonological processing and letter knowledge as early predictors of spelling and reading skills in Grade 1. Moreover, intelligence is identified as a predictor for early spelling, while rapid automatized naming (RAN) emerges as a predictor for early reading.

Diminished differentiation of rewards in individuals at clinical high-risk for psychosis.

Reward processing is impaired in people with schizophrenia, which may begin in the clinical high-risk (CHR) for psychosis period. The Monetary Incentive Delay (MID) task has been important in understanding the neural correlates of reward processing deficits in various psychiatric disorders. Previous research has found that CHR individuals have an imprecise mental representation of rewards, which leads to a diminished differentiation between rewards, though this has not been observed behaviorally. A total of 19 CHR individuals and 20 controls were given a novel variant of the MID task, designed to examine how modulating reward context may impact responses to reward cues, a process often referred to as "adaptive coding." Both groups appeared to update their behavior in response to the rewards available in this adaptive task. However, when compared to controls who showed a more graded decrease in response time to increasing reward contexts, CHR individuals appeared to have a sharp decrease in response time in the low reward context that is nearly stable across higher reward contexts. This is largely driven by the exponential component of the response time distribution, which is often interpreted to be more cognitively or effortfully influenced. Response times are related to negative symptoms, but not positive symptoms, disorganized symptoms, or estimated intelligence. Although an adaptive coding effect was not observed, these results provide novel insight into the reward processing mechanisms and volitional processes in the CHR population, as this was the first study to observe the diminished differentiation of rewards behaviorally.

Temporal processing abilities in normal hearing individuals with tinnitus: a systematic review

PurposeTinnitus, characterized by the perception of auditory phantoms, is prevalent worldwide and can lead to a range of hearing-related issues. Understanding its influence on temporal processing helps to delineate the auditory manifestations of tinnitus. This systematic review aimed to identify the patterns of temporal processing difficulties in individuals with tinnitus and normal hearing abilities. Furthermore, this review evaluates the potential of specific measurement techniques as tools for diagnosing temporal processing deficits in tinnitus.MethodsA comprehensive search was conducted in multiple international databases, followed by rigorous screening of the titles, abstracts, and full-length content. The inclusion and exclusion criteria were formulated using the Population, intervention, compression, Outcome, and Study design (PICOS) format, and the study bias was determined. After excluding irrelevant articles, nine studies were selected for the analysis.ResultsOver 50% of the selected studies demonstrated a significant impact on temporal processing in individuals with tinnitus, especially with gap detection test (GDT) and gaps in noise (GIN) tests, indicating of the deficits in peripheral temporal process in tinnitus individuals with normal hearing. However, the other central auditory tests showed no major changes.ConclusionsThe findings from this review underscore the importance of understanding temporal processing impairments in tinnitus and hold promise for enhancing the diagnostic accuracy and treatment outcomes, ultimately improving the lives of those affected by tinnitus. This review highlights the potential of the GDT and GIN tests as sensitive tools for assessing temporal processing deficits in the peripheral auditory system, which in turn can manifest as central changes in temporal processing.Systematic review registrationhttp://www.crd.york.ac.uk/PROSPERO/display_record.asp?ID=CRD42021287194, Prospero [CRD42021287194].

Pupillary responses as a biomarker of cognitive effort and the impact of task difficulty on reward processing in schizophrenia

Negative symptoms of schizophrenia robustly predict functional outcomes but remain relatively resistant to available treatments. Better measures of negative symptoms, especially motivational deficits, are needed to better understand these symptoms and improve treatment development. Recent research shows promise in linking behavioral effort tasks to motivational negative symptoms, reward processing deficits, and defeatist attitudes, but few studies account for individual or group (patient v. control) differences in cognitive ability to perform the tasks. Individuals with poorer abilities might be less motivated to perform tasks because they find them more difficult to perform. This study used a personalized digit span task to control task difficulty while measuring task effort via pupillary responses (greater dilation indicates greater cognitive effort) at varying monetary rewards ($1 & $2). Participants with schizophrenia (N = 34) and healthy controls (N = 41) performed a digit span task with personalized max span lengths and easy (max− 2 digits) and overload (max+ 2 digits) conditions. Consistent with many studies, pupillary responses (cognitive effort) increased with greater difficulty until exceeding capacity. A similar pattern of reward responsivity was seen in both groups, such that greater reward increased dilation (effort) comparably for both groups when difficulty was within capacity. Neither patients nor controls exerted increased effort for greater reward when difficulty exceeded capacity. In patients, positive relationships were found between pupil dilation and defeatist performance beliefs if task difficulty was within capacity; a relationship that reversed if the task was too difficult. The findings demonstrate the importance of accounting for cognitive capacity and task difficulty when evaluating motivation and reward sensitivity and illustrate the utility of pupillary responses as an objective measure of effort in schizophrenia.

Neurocognitive and psycho-emotional profile of children with disabilities

Objective Neurocognitive deficits in attention, short-term memory, and sequential information processing are present in children with a variety of disabilities, whereas language and visuospatial abilities vary. Method We compared the performance of 59 children (mean age, 15 years) with learning disabilities (n = 18), Down syndrome (n = 21), and intellectual disabilities (n = 20). A series of neuropsychological tests were used to evaluate the neurocognitive processes of memory, attention, visuospatial perception, and executive function. To better understand what emotions they experience, we assessed emotions like anxiety, depression, and positive and negative mood. Results The performance of children with Down syndrome was statistically significantly different from that of other groups, indicating lower performance (p = 0.001). In comparison to other groups, children with Down syndrome performed significantly worse across all cognitive domains. Additionally, there were no statistically significant differences between groups and low emotional functioning scores across the board for all children. People with DS frequently have distinctive neurocognitive and neurobehavioral profiles that appear during particular developmental phases and have many distinct strengths and weaknesses that should be respected as they mature over the course of their lives. The current findings have substantial consequences for interventions that are focused on achieving the best results.

A hierarchy of visual processing deficits in body dysmorphic disorder: a conceptual review and empirical investigation

ABSTRACT Objective Abnormal visual processing has been proposed as a mechanism underlying excessive focus on minor appearance flaws in body dysmorphic disorder (BDD). Existing BDD research has not differentiated the various stages of face processing (featural, first-order configural, holistic and second-order configural) that are required for higher-order processes such as emotion recognition. This study investigated a hierarchical visual processing model to examine the nature of abnormalities in face processing in BDD. Method Thirty BDD participants and 27 healthy controls completed the Navon task, a featural and configural face processing task and a facial emotion labelling task. Results BDD participants performed similarly to controls when processing global and local non-face stimuli on the Navon task, when detecting subtle changes in the features and spacing of a target face, and when labelling emotional faces. However, BDD participants displayed poorer performance when viewing inverted faces, indicating difficulties in configural processing. Conclusions The findings only partially support prior work. However, synthesis of results with previous findings indicates that heterogenous task methodologies may contribute to inconsistent findings. Recommendations are provided regarding the task parameters that appear most sensitive to abnormalities in BDD.

Emotion recognition in amyotrophic lateral sclerosis in a dynamic environment

ObjectiveThe aim of our study was to measure the ability of ALS patients to process dynamic facial expressions as compared to a control group of healthy subjects and to correlate this ability in ALS patients with neuropsychological, clinical and neurological measures of the disease. MethodsSixty-three ALS patients and 47 healthy controls were recruited. All the ALS patients also underwent i) the Geneva Emotion Recognition Test (GERT) in which ten actors express 14 types of dynamic emotions in brief video clips with audio, ii) the Edimburgh Cognitive and Behavioral ALS Screen (ECAS) test; iii) the ALS Functional Rating Scale Revised (ALSFRS-R) and iv) the Medical Research Council (MRC) for the evaluation of muscle strength. All the healthy subjects enrolled in the study underwent the GERT. ResultsThe recognition of irritation and pleasure was significantly different between ALS patients and the control group. The amusement, despair, irritation, joy, sadness and surprise had been falsely recognized differently between the two groups. Specific ALS cognitive impairment was associated with bulbar-onset phenotype (OR = 14,3889; 95%CI = 3,96-52,16). No association was observed between false emotion recognition and cognitive impairment (F(1,60)=,56,971, p=,45,333). The number of categorical errors was significantly higher in the ALS patients than in the control group (27,66 ± 7,28 vs 17,72 ± 5,29; t = 8723; p = 0.001). ConclusionsALS patients show deficits in the dynamic processing of a wide range of emotions. These deficits are not necessarily associated with a decline in higher cognitive functions: this could therefore lead to an underestimation of the phenomenon.

Female-specific dysfunction of sensory neocortical circuits in a mouse model of autism mediated by mGluR5 and estrogen receptor α

Little is known of the brain mechanisms that mediate sex-specific autism symptoms. Here, we demonstrate that deletion of the autism spectrum disorder (ASD)-risk gene, Pten, in neocortical pyramidal neurons (NSEPten knockout [KO]) results in robust cortical circuit hyperexcitability selectively in female mice observed as prolonged spontaneous persistent activity states. Circuit hyperexcitability in females is mediated by metabotropic glutamate receptor 5 (mGluR5) and estrogen receptor α (ERα) signaling to mitogen-activated protein kinases (Erk1/2) and de novo protein synthesis. Pten KO layer 5 neurons have a female-specific increase in mGluR5 and mGluR5-dependent protein synthesis. Furthermore, mGluR5-ERα complexes are generally elevated in female cortices, and genetic reduction of ERα rescues enhanced circuit excitability, protein synthesis, and neuron size selectively in NSEPten KO females. Female NSEPten KO mice display deficits in sensory processing and social behaviors as well as mGluR5-dependent seizures. These results reveal mechanisms by which sex and a high-confidence ASD-risk gene interact to affect brain function and behavior.