Atypical Face Research Articles

Primary Histiocytic Sarcoma of Bone with Disseminated Involvement - Initial Presentation Mimicking Multiple Myeloma: The Atypical Face of a Rare Neoplasm

Primary Histiocytic Sarcoma of Bone with Disseminated Involvement - Initial Presentation Mimicking Multiple Myeloma: The Atypical Face of a Rare Neoplasm

Eye-gaze processing in the broader bipolar phenotype revealed by electrical neuroimaging

Previous studies have documented atypical brain responses to faces in individuals with bipolar disorder (BD) and in their relatives. In view of previous findings of atypical face processing in youths at risk for BD, the aim of this study was to examine whether BD patients and offspring would show differential activation in networks of the social brain when processing eye-gaze.Data from 18 euthymic BD patients and 18 offspring, as well as 36 age-matched healthy controls, were collected using a delayed face-matching paradigm, event related potentials and electrical neuroimaging methods.The P200 component, which is implicated in facial cues decoding, differentiated the BD groups from their age-matched controls. P200 source reconstruction indicates impairments conveyed by eye-contact in a network involved in experiencing others’ social intentions in BD patients (supplementary motor cortex, precentral gyrus, inferior parietal lobe), and the engagement of compensatory prefrontal mechanisms for modulating these functions in BD offspring. When viewing faces that had an averted gaze, BD patients and offspring showed a hypo-activation, compared to controls, particularly in regions involved in experiencing others’ feelings (post-central gyrus in BD patients / ventral premotor cortex in offspring). Therefore, the neural mechanism for decoding shifts in eye-gaze may be a familial characteristic of BD.

Face perception in autism spectrum disorder: Modulation of holistic processing by facial emotion

Individuals with Autism Spectrum Disorder (ASD; autistic individuals) may exhibit atypical face perception because they fail to process faces holistically. In the context of this hypothesis, it is critical to determine whether autistic individuals exhibit diminished susceptibility to the composite face illusion, widely regarded as a key marker of holistic face processing. To date, however, previous studies have yielded inconsistent findings. In light of recent evidence suggesting that facial emotion cues increase the strength of the composite face illusion in typical individuals, the present study sought to determine whether the presence of facial emotion also modulates the strength of the composite face illusion in autistic individuals, many of whom experience difficulties recognizing facial expressions. We therefore measured composite face effects in a sample of autistic individuals (N = 20) and matched typical controls (N = 29) using an incidental emotion procedure in which distractor regions varied systematically in their emotion strength. As expected, the presence of facial emotion in the distractor regions of composite face arrangements increased the strength of the illusory distortion induced. The extent of the modulation by facial emotion was similar in the two groups. The composite effects seen in the ASD group were qualitatively and quantitatively similar to those seen in the typical group, suggestive of intact holistic processing in this population.

Reduced distractor interference in neurotypical adults with high expression of autistic traits irrespective of stimulus type.

Attention atypicality is evident in autism spectrum disorder (ASD) and its broader phenotype with previous studies suggesting that in some cases participants can be more efficient at ignoring distracting irrelevant information. However, it is not clear to what extent this improved filtering capacity is driven by perceptual atypicality, such as local bias or atypical face processing, which is also sometimes reported in these populations. For instance, better ability to ignore the global aspect of a display could stem from a local perceptual bias rather than from improved distractor inhibition. To test whether distractor suppression per se, is associated with high expression of autistic traits, in the present study a large cohort of neurotypical participants (n = 218), in whom expression of autistic traits was assessed, performed two nonspatial attention selection tasks with different categories of stimuli (global/local and face/scene). Importantly, both tasks involved a conflict with one aspect of the stimuli designated as the target and the other designated as the distractor. Across the two experiments adults with high autistic traits were overall, better able to ignore distractors than adults with low autistic traits, irrespective of the type of perceptual processing involved. These results support the notion that autistic tendencies are associated with increased attention filtering (at least when target and distractor remain constant) which is not dependent on perceptual biases. Thus, future work in the broader autism phenotype should explicitly consider the effect played by attention mechanisms in this population. Autism Res 2018, 11: 1345-1355. © 2018 International Society for Autism Research, Wiley Periodicals, Inc. LAY SUMMARY: In the present study, we show that neurotypical adults with high autistic traits are better able to avoid distraction from conspicuous (but completely irrelevant) distractors when told in advance to do so. This ability is not affected by the type of visual input (for instance, whether the distractor is a face or whether small rather than large letters should be reported). This finding could be important in better understanding the way attention is utilized in Autism.

Face processing in 22q11.2 deletion syndrome: atypical development and visual scanning alterations

BackgroundPrevious research links social difficulties to atypical face exploration in 22q11.2 deletion syndrome (22q11.2DS). Two types of face processing are distinguished: configural (CFP) and featural (FFP). CFP develops later in life and plays an important role in face and emotion recognition abilities. Recent studies reported atypical development of CFP in several neurodevelopmental disorders. Taking previous reports of atypical face exploration one step further, our study aims at characterizing face processing in children and adolescents with 22q11.2DS. First, we sought to identify biases in the first two fixation positions on faces and to detect differences between CFP and FFP in 22q11.2DS using eye-tracking technology. Second, we investigated the developmental trajectories of CFP and FFP using accuracy data from follow-up evaluation.MethodsSeventy-five individuals with 22q11.2DS and 84 typically developed (TD) individuals (aged 6–21 years) completed a discrimination task (“Jane task”) inducing CFP and FFP in an eye-tracking setting. Thirty-six individuals with 22q11DS and 30 TD from our sample completed a longitudinal follow-up evaluation.ResultsFindings revealed that individuals with 22q11.2DS demonstrate an early bias toward the mouth region during the initial fixations on the faces and reduced flexibility exploration of the faces, with a reduced number of transitions between faces and longer fixations compared to the TD group. Further, scanpaths did not differ between CFP and FFP in the 22q11.2DS group. Longitudinal analysis of accuracy data provided evidence for atypical development of CFP in 22q11.2DS.ConclusionsThe current study brings new evidence of altered face exploration in 22q11.2DS and identifies developmental mechanisms that may contribute to difficulties impacting social interactions in the syndrome.

Social cognition in autism: Face tuning

Faces convey valuable information for social cognition, effective interpersonal interaction, and non-verbal communication. Face perception is believed to be atypical in autism, but the origin of this deficit is controversial. Dominant featural face encoding is suggested to be responsible for face tuning scarcity. Here we used a recently developed Face-n-Food paradigm for studying face tuning in individuals with autistic spectrum disorders (ASD). The key benefit of these images is that single components do not explicitly trigger face processing. In a spontaneous recognition task, adolescents with autism and typically developing matched controls were presented with a set of Face-n-Food images in different degree resembling a face (slightly bordering on the Giuseppe Arcimboldo style). The set of images was shown in a predetermined order from the least to most resembling a face. Thresholds for recognition of the Face-n-Food images as a face in ASD individuals were substantially higher than in typically developing controls: they did not report seeing a face on the images, which controls easily recognized as a face, and gave overall fewer face responses. This outcome not only lends support to atypical face tuning, but provides novel insights into the origin of face encoding deficits in autism.

Facing a Disruptive Face: Embodiment in the Everyday Experiences of \u201cDisfigured\u201d Individuals

In recent years, facial difference is increasingly on the public and academic agenda. This is evidenced by the growing public presence of individuals with an atypical face, and the simultaneous emergence of research investigating the issues associated with facial variance. The scholarship on facial difference approaches this topic either through a medical and rehabilitation perspective, or a psycho-social one. However, having a different face also encompasses an embodied dimension. In this paper, we explore this embodied dimension by interpreting the stories of individuals with facial limb absence against the background of phenomenological theories of the body, illness and disability. Our findings suggest that the atypical face disrupts these individuals’ engagement with everyday projects when it gives rise to disruptive perceptions, sensations, and observations. The face then ceases to be the absent background to perception, and becomes foregrounded in awareness. The disruptions evoked by facial difference call for adjustments: as they come to terms with their altered face, the participants in our study gradually develop various new bodily habits that re-establish their face’s absence, or relate to its disruptive presence. It is through these emergent habits that facial difference comes to be embodied. By analyzing the everyday experiences of individuals with facial limb absence, this article provides a much-needed exploration of the embodied aspects of facial difference. It also exemplifies how a phenomenological account of illness and disability can do justice both to the impairments and appearance issues associated with atypical embodiment.

Atypical Processing of Gaze Cues and Faces Explains Comorbidity between Autism Spectrum Disorder (ASD) and Attention Deficit/Hyperactivity Disorder (ADHD)

This study investigated the neurobiological basis of comorbidity between autism spectrum disorder (ASD) and attention deficit/hyperactivity disorder (ADHD). We compared children with ASD, ADHD or ADHD+ASD and typically developing controls (CTRL) on behavioural and electrophysiological correlates of gaze cue and face processing. We measured effects of ASD, ADHD and their interaction on the EDAN, an ERP marker of orienting visual attention towards a spatially cued location and the N170, a right-hemisphere lateralised ERP linked to face processing. We identified atypical gaze cue and face processing in children with ASD and ADHD+ASD compared with the ADHD and CTRL groups. The findings indicate a neurobiological basis for the presence of comorbid ASD symptoms in ADHD. Further research using larger samples is needed.

A Case of Jugular Paraganglioma Presented as Temporomandibular Joint Disorder

Background:Jugular paraganlioma is a slow-growing tumor originating from the paraganglion cells. The diagnosis of a jugular paraganglioma may be delayed due to its atypical symptoms. We report a case of jugular paraganglioma presented as temporomandibular joint (TMJ) disorder. Clinical presentation: 29-years old woman with preliminary diagnosis probable atypical face pain. She experienced her pain for the first time 2 months prior to reference. Pain intensity was seven to eight out of ten points on Visual Analogue Scale, simple and combined analgetics and NSAIDs were ineffective. Pain was dull, aching, localized in the right temporal area with irradiation to the ipsilateral maxillar and periauricular areas. Pain was more intensive in the nighttime. Temporomandibular joint movement (maximum unassisted and assisted opening, right to left movements) also increased pain. Significant dilatation of right foramen jugularis with its right side destruction was detected by computed tomography with contrast. The radiological findings were consistent with those of a jugular paraganglioma. Conclusion:This case confirms that in refractory headache and TMJ pain other reasons should be considered with rigorous investigation including neuroimaging

Face perception develops similarly across viewpoint in children and adolescents with and without autism spectrum disorder.

Atypical face perception has been associated with the socio-communicative difficulties that characterize autism spectrum disorder (ASD). Growing evidence, however, suggests that a widespread impairment in face perception is not as common as once thought. One important issue arising with the interpretation of this literature is the relationship between face processing and a more general perceptual tendency to focus on local rather than global information. Previous work has demonstrated that when discriminating faces presented from the same view, older adolescents and adults with ASD perform similarly to typically developing individuals. When faces are presented from different views, however, they perform more poorly-specifically, when access to local cues is minimized. In this study, we assessed the cross-sectional development of face identity discrimination across viewpoint using same- and different-view conditions in children and adolescents with and without ASD. Contrary to the findings in adults, our results revealed that all participants experienced greater difficulty identifying faces from different views than from same views, and demonstrated similar age-expected improvements in performance across tasks. These results suggest that differences in face discrimination across views may only emerge beyond the age of 15 years in ASD.

An Event-Related Potential Study on the Perception and the Recognition of Face, Facial Features, and Objects in Children With Autism Spectrum Disorders.

The study investigated whether children with autism spectrum disorders (ASD) showed atypical patterns of brain specialization for face processing, whether the response to familiar and unfamiliar faces, facial features, and objects were different from typically developing children. Event-related potentials were recorded in 5- to 8-year-old children (12 children with ASD, 12 typically developing children) using passive viewing paradigm. The fastest P1 latencies to faces and the largest P1 amplitudes to objects were observed in both participant groups. Both groups exhibited larger N170 response to faces and eyes, F(3, 66) = 46.94, p < .0001). However, earlier P1 and N170 latencies were found on left hemisphere in children with ASD, respectively, F(1, 83) = 4.32, p = .04; F(1, 83) = 6.73, p = .01, indicating an atypical face processing pattern. All children showed a significant effect of familiarity for objects and mouths, F(1, 71) = 33.97, p < .0001; F(1, 71 = 15.94, p = .0002. Children with ASD revealed smaller negative central to faces relative to typically developing children. Face processing abnormalities revealed in children with ASD very likely exist.

Social Cognition in Williams Syndrome: Face Tuning.

Many neurological, neurodevelopmental, neuropsychiatric, and psychosomatic disorders are characterized by impairments in visual social cognition, body language reading, and facial assessment of a social counterpart. Yet a wealth of research indicates that individuals with Williams syndrome exhibit remarkable concern for social stimuli and face fascination. Here individuals with Williams syndrome were presented with a set of Face-n-Food images composed of food ingredients and in different degree resembling a face (slightly bordering on the Giuseppe Arcimboldo style). The primary advantage of these images is that single components do not explicitly trigger face-specific processing, whereas in face images commonly used for investigating face perception (such as photographs or depictions), the mere occurrence of typical cues already implicates face presence. In a spontaneous recognition task, participants were shown a set of images in a predetermined order from the least to most resembling a face. Strikingly, individuals with Williams syndrome exhibited profound deficits in recognition of the Face-n-Food images as a face: they did not report seeing a face on the images, which typically developing controls effortlessly recognized as a face, and gave overall fewer face responses. This suggests atypical face tuning in Williams syndrome. The outcome is discussed in the light of a general pattern of social cognition in Williams syndrome and brain mechanisms underpinning face processing.

Identifying children with autism spectrum disorder based on their face processing abnormality: A machine learning framework.

The atypical face scanning patterns in individuals with Autism Spectrum Disorder (ASD) has been repeatedly discovered by previous research. The present study examined whether their face scanning patterns could be potentially useful to identify children with ASD by adopting the machine learning algorithm for the classification purpose. Particularly, we applied the machine learning method to analyze an eye movement dataset from a face recognition task [Yi et al., 2016], to classify children with and without ASD. We evaluated the performance of our model in terms of its accuracy, sensitivity, and specificity of classifying ASD. Results indicated promising evidence for applying the machine learning algorithm based on the face scanning patterns to identify children with ASD, with a maximum classification accuracy of 88.51%. Nevertheless, our study is still preliminary with some constraints that may apply in the clinical practice. Future research should shed light on further valuation of our method and contribute to the development of a multitask and multimodel approach to aid the process of early detection and diagnosis of ASD. Autism Res 2016, 9: 888-898. © 2016 International Society for Autism Research, Wiley Periodicals, Inc.

Adults with autism spectrum disorder show evidence of figural aftereffects with male and female faces

The norm-based coding model of face perception posits that face perception involves an implicit comparison of observed faces to a representation of an average face (prototype) that is shaped by experience. Using some methods, observers with autism spectrum disorder (ASD) have shown atypical face perception, but other methods suggest preserved face perception. Here, we used a figural aftereffects paradigm to test whether adults with ASD showed evidence of norm-based coding of faces, and whether they encode separate prototypes for male and female faces, as typical observers do. Following prolonged exposure to distorted faces that differ from their stored prototype, neurotypical adults show aftereffects: their prototype shifts in the direction of the adapting face. We measured aftereffects following adaptation to one distorted gender. There were no significant group differences in the size or direction of the aftereffects; both groups showed sex-selective aftereffects after adapting to expanded female faces but showed aftereffects for both sexes after adapting to contracted face of either sex, demonstrating that adults with and without ASD show evidence of partially dissociable male and female face prototypes. This is the first study to examine sex-selective prototypes using figural aftereffects in adults with ASD and replicates the findings of previous studies examining aftereffects in adults with ASD. The results contrast with studies reporting diminished adaptation in children with ASD.

Causation model of autism: Audiovisual brain specialization in infancy competes with social brain networks

Earliest identifiable findings in autism indicate that the autistic brain develops differently from the typical brain in the first year of life, after a period of typical development. Twin studies suggest that autism has an environmental component contributing to causation. Increased availability of audiovisual (AV) materials and viewing practices of infants parallel the time frame of the rise in prevalence of autism spectrum disorder (ASD). Studies have shown an association between ASD and increased TV/cable screen exposure in infancy, suggesting AV exposure in infancy as a possible contributing cause of ASD.Infants are attracted to the saliency of AV materials, yet do not have the experience to recognize these stimuli as socially relevant. The authors present a developmental model of autism in which exposure to screen-based AV input in genetically susceptible infants stimulates specialization of non-social sensory processing in the brain. Through a process of neuroplasticity, the autistic infant develops the skills that are driven by the AV viewing. The AV developed neuronal pathways compete with preference for social processing, negatively affecting development of social brain pathways and causing global developmental delay. This model explains atypical face and speech processing, as well as preference for AV synchrony over biological motion in ASD. Neural hyper-connectivity, enlarged brain size and special abilities in visual, auditory and motion processing in ASD are also explained by the model. Positive effects of early intervention are predicted by the model. Researchers studying causation of autism have largely overlooked AV exposure in infancy as a potential contributing factor. The authors call for increased public awareness of the association between early screen viewing and ASD, and a concerted research effort to determine the extent of causal relationship.

Atypical Face Perception in Autism: A Point of View?

Face perception is the most commonly used visual metric of social perception in autism. However, when found to be atypical, the origin of face perception differences in autism is contentious. One hypothesis proposes that a locally oriented visual analysis, characteristic of individuals with autism, ultimately affects performance on face tasks where a global analysis is optimal. The objective of this study was to evaluate this hypothesis by assessing face identity discrimination with synthetic faces presented with and without changes in viewpoint, with the former condition minimizing access to local face attributes used for identity discrimination. Twenty-eight individuals with autism and 30 neurotypical participants performed a face identity discrimination task. Stimuli were synthetic faces extracted from traditional face photographs in both front and 20° side viewpoints, digitized from 37 points to provide a continuous measure of facial geometry. Face identity discrimination thresholds were obtained using a two-alternative, temporal forced choice match-to-sample paradigm. Analyses revealed an interaction between group and condition, with group differences found only for the viewpoint change condition, where performance in the autism group was decreased compared to that of neurotypical participants. The selective decrease in performance for the viewpoint change condition suggests that face identity discrimination in autism is more difficult when access to local cues is minimized, and/or when dependence on integrative analysis is increased. These results lend support to a perceptual contribution of atypical face perception in autism.

Neural and behavioural responses to face-likeness of objects in adolescents with autism spectrum disorder

Numerous studies have revealed atypical face processing in autism spectrum disorders (ASD) characterized by social interaction and communication difficulties. This study investigated sensitivity to face-likeness in ASD. In Experiment 1, we found a strong positive correlation between the face-likeness ratings of non-face objects in the ASD (11–19 years old) and the typically developing (TD) group (9–21 years old). In Experiment 2 (the scalp-recorded event-related potential experiment), the participants of both groups (ASD, 12–19 years old; TD, 12–18 years old) exhibited an enhanced face-sensitive N170 amplitude to a face-like object. Whereas the TD adolescents showed an enhanced N170 during the face-likeness judgements, adolescents with ASD did not. Thus, both individuals with ASD and TD individuals have a perceptual and neural sensitivity to face-like features in objects. When required to process face-like features, a face-related brain system reacts more strongly in TD individuals but not in individuals with ASD.

“Please Draw Me a Face…” Atypical Face Mental Concept in Autism

Autism is a severe neurodevelopmental disorder characterized by early manifestations of social difficulties and atypical sensory-based behaviors. As faces are essential for social interaction, they have been widely investigated in autism, revealing disruptions in face processing. Cognitive theories argue that people with autism process the world differently, showing a processing bias for local information. However, the literature currently neglects the mental representation of faces, or face concept, in people with ASD. The current study sought to establish how young children with ASD “see” a human face. A key question is whether this face concept is a function of cognitive style and/or sensory impairment. By comparing simple face drawings in young children with ASD, with those of deaf children and controls, we highlight an atypical face concept in ASD that does not show, however, global processing deficits: face drawing presents a preserved overall configuration. The atypical face drawings in children with ASD showed similarities with those of deaf children: eyes were not an essential feature, whereas a marked interest for ears and non-facial external features (accessories, body parts) was shown. These findings suggest that the face mental concept in ASD may be impacted by sensory processing deficits.

Face-sensitive brain responses measured from a four-year-old child with a custom-sized child MEG system

BackgroundPrevious magnetoencephalography (MEG) studies have failed to find a facesensitive, brain response-M170 in children. If this is the case, this suggests that the developmental trajectory of the M170 is different from that of its electrical equivalent, the N170. We investigated the alternative possibility that the child M170 may not be detectable in conventional adult-sized MEG systems. New methodBrain responses to pictures of faces and well controlled stimuli were measured from the same four-year-old child with a custom child MEG system and an adult-sized MEG system. ResultsThe goodness of fit of the child's head was about the same over the occipital head surface in both systems, but was much worse over all other parts of the head surface in the adult MEG system compared to the child MEG system. The face-sensitive M170 was measured from the child in both MEG systems, but was larger in amplitude, clearer in morphology, and had a more accurate source localization when measured in the child MEG system. Comparison with existing methodThe custom-sized child MEG system is superior for measuring the face-sensitive M170 brain response in children than the conventional adult MEG system. ConclusionsThe present results show that the face-sensitive M170 brain response can be elicited in a four-year-old child. This provides new evidence for early maturation of face processing brain mechanisms in humans, and offers new opportunities for the study of neurodevelopmental disorders that show atypical face processing capabilities, such as autism spectrum disorder.

Sex Differences in Social Perception in Children with ASD

Autism spectrum disorder (ASD) is more common in males than females. An underrepresentation of females in the ASD literature has led to limited knowledge of differences in social function across the sexes. Investigations of face perception represent a promising target for understanding variability in social functioning between males and females. The current study analyzed electrophysiological brain recordings during face perception to investigate sex differences in the neural correlates of face perception and their relationship to social function. Event related potentials (ERP) were recorded from children with ASD while viewing faces, inverted faces, and houses. Relative to males, females showed attenuated response at an ERP marker of face perception, the N170. Among females, but not males, atypical face response was associated with symptom severity. Observed sex differences reflect influential differences in social information processing, and impairment in these features correlates with deficits in social information processing in females, but not males, with ASD. These findings hold significance for future treatment protocols, which should account for differences in males and females with ASD in clinical presentation and neural phenotypes.