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The interplay between facial age and facial identity is evident from several scenarios experienced in daily life, such as when recognising a face several decades after the last exposure. However, the link between age and identity processing, and how age perception abilities might diverge in individuals with different face processing abilities, has scarcely been considered. Furthermore, the approach used to test age perception ability may also influence outcome, but the effect of different paradigms on performance is not yet known. Across three studies, we compare super-recognisers (SRs), people with developmental prosopagnosia (DPs), and a group of neurotypical controls, on three age perception paradigms. There were no differences on the numeric age estimation task (i.e. providing precise age estimates for a series of faces; Study 1), and numeric age estimation task with added noise-distortion to stimuli (Study 2). However, SRs were more accurate when instructed to classify ambient faces as either over- or under- the age of 18 compared to both DPs and controls (Study 3). Thus, there may be nuanced differences in age processing which can be tapped into using separate paradigms; however, given that the difference is only with SRs it remains unclear to what extent these are linked to facial identity processing. [ABSTRACT FROM AUTHOR]

La prosopagnosie, décrite au XIXe siècle, a reçu son nom en 1947 de Joachim Bodamer (1910–1985), un neuropsychiatre allemand. Ce terme désigne l'incapacité à reconnaître un visage pourtant familier. Ce symptôme, rarement isolé, résulte habituellement de lésions cérébrales prédominant à la face inférieure de la jonction temporo-occipitale droite. Il existe des cas, plus fréquents, de prosopagnosie développementale. Le syndrome de Capgras (SC), décrit par Joseph Capgras (1873–1950), consiste en la conviction durable qu'un proche, qui est identifié, a été remplacé par un sosie. Le SC, considéré comme un trouble délirant de l'identification ou de la familiarité, a été décrit dans un contexte psychiatrique, au cours de psychoses chroniques, habituellement la schizophrénie. Il est aussi rencontré dans l'évolution de maladies neurodégénératives (maladie d'Alzheimer, démence à corps de Lewy, démence de la maladie de Parkinson). Des formes lésionnelles sont de connaissance plus récente. La prosopagnosie et le SC ont été parfois considérés comme résultant de lésions en miroir l'une de l'autre, n'impliquant qu'un facteur, l'un affectant un circuit ventral conscient de reconnaissance des visages (prosopagnosie), l'autre affectant un circuit dorsal inconscient impliqué dans les réactions émotionnelles et végétatives (SC). L'étude de formes lésionnelles en IRM par cartographie de réseaux lésionnels suggère un mécanisme plus complexe. Les lésions à l'origine de la prosopagnosie sont corrélées à une des régions majeures liées à la reconnaissance des visages, la Fusiform Face Area droite, et anticorrélées avec des régions frontales gauches possiblement impliquées dans le contrôle attentionnel. Dans les formes lésionnelles de SC, il existe une corrélation avec le cortex rétrosplénial gauche, en lien avec la familiarité, et des régions frontales droites, en lien avec l'évaluation des croyances, résultat en faveur de l'intervention de deux facteurs. Le débat sur les mécanismes n'est pas tranché, d'autant qu'il se cantonne aux formes lésionnelles, mais les études en cartographie de réseaux lésionnels marquent dans ce cadre un changement de paradigme, l'attention se portant des lésions (méthode clinico-anatomique) aux dysfonctions de circuits. First described in the 19th century in patients with brain lesions, prosopagnosia was named in 1947 by Joachim Bodamer (1910–1985), a German neuropsychiatrist. The term refers to the inability to recognise a familiar face, a rarely isolated symptom. Prosopagnosia is thought to result from brain lesions, predominantly at the ventral right temporo-occipital junction. There are more common cases of developmental prosopagnosia, where no lesion has been identified. Capgras syndrome (CS), described by Joseph Capgras (1873–1950), a psychiatrist, consists of the persistent belief that a close relative, identified, has been replaced by a look-alike. SC, considered a delusional disorder of identification or familiarity, has been described in chronic psychoses, mainly schizophrenia. It is also reported in the course of neurodegenerative diseases (mainly Alzheimer's disease, Lewy body dementia, Parkinson's dementia). Lesion-based forms have recently been described. Prosopagnosia and SC were initially thought to result from mirror-image lesions involving a single factor, one affecting an overt ventral circuit for face recognition (in the case of prosopagnosia), the other a covert dorsal circuit involved in emotional and vegetative responses (in the case of CS). Lesion network mapping has been used in both lesion-related conditions and suggests a more complex figure. Lesions causing prosopagnosia were correlated with one of the main regions associated with face recognition, the right fusiform face area, and anticorrelated with left frontal regions possibly involved in visual attentional control. In lesional forms of CS or other delusional misidentification syndromes, there was a correlation with left retrosplenial cortex, an area associated with familiarity processing, and a correlation with right frontal regions associated with belief evaluation. This finding supports the intervention of two factors in CS, one generating the impaired familiarity associated with perception and the other the genesis and persistence of a delusional idea. The debate on the mechanisms of CS is not settled, especially as it is limited to its lesional forms, but studies using lesion network mapping already mark a more general paradigm shift, with attention shifting from lesions (clinico-anatomical method) to circuit dysfunction. [ABSTRACT FROM AUTHOR]

The Diagnostic Statistical Manual of Mental Disorders (DSM-5) recommends diagnosing neurocognitive disorders (i.e., cognitive impairment) when a patient scores beyond – 1 SD below neurotypical norms on two tests. I review how this approach will fail due to cognitive tests' power limitations, validity issues, imperfect reliabilities, and biases, before summarizing their resulting negative consequences. As a proof of concept, I use developmental prosopagnosia, a condition characterized by difficulties recognizing faces, to show the DSM-5 only diagnoses 62–70% (n1 = 61, n2 = 165) versus 100% (n1 = 61) through symptoms alone. Pooling the DSM-5 missed cases confirmed the presence of group-level impairments on objective tests, which were further evidenced through meta-analyses, thus validating their highly atypical symptoms. These findings support a paradigm shift towards bespoke diagnostic approaches for distinct cognitive impairments, including a symptom-based method when validated effective. I reject dogmatic adherence to the DSM-5 approach to neurocognitive disorders, and underscore the importance of a data driven, transdiagnostic approach to understanding patients' subjective cognitive impairments. This will ultimately benefit patients, their families, clinicians, and scientific progress. [ABSTRACT FROM AUTHOR]

Abstract The interplay between facial age and facial identity is evident from several scenarios experienced in daily life, such as when recognising a face several decades after the last exposure. However, the link between age and identity processing, and how age perception abilities might diverge in individuals with different face processing abilities, has scarcely been considered. Furthermore, the approach used to test age perception ability may also influence outcome, but the effect of different paradigms on performance is not yet known. Across three studies, we compare super-recognisers (SRs), people with developmental prosopagnosia (DPs), and a group of neurotypical controls, on three age perception paradigms. There were no differences on the numeric age estimation task (i.e. providing precise age estimates for a series of faces; Study 1), and numeric age estimation task with added noise-distortion to stimuli (Study 2). However, SRs were more accurate when instructed to classify ambient faces as either over- or under- the age of 18 compared to both DPs and controls (Study 3). Thus, there may be nuanced differences in age processing which can be tapped into using separate paradigms; however, given that the difference is only with SRs it remains unclear to what extent these are linked to facial identity processing.

The present case study describes the patient N.G., who reported prosopagnosia along with difficulty in recognising herself in the mirror following a left‐sided temporo‐occipital hemispheric stroke. The neuropsychological and experimental investigation revealed only a mild form of apperceptive prosopagnosia, without visual agnosia, primarily caused by an impaired visual processing of face‐parts and body parts but not of full faces. Emotional expressions did not modulate her face processing. On the other hand, N.G. showed a marked impairment of visual self‐recognition, as assessed with visual matching‐to‐sample tasks, both at the level of body‐part and face‐part processing and at a full‐face level, featured by a deficit in the perceptual discrimination of her own face and body, as compared to the others' face and body. N.G.'s lesion mapping showed damage to the left inferior occipito‐temporal cortex, affecting the inferior occipital gyrus and compromising long‐range connections between the occipital/temporo‐occipital areas and the anterior fronto‐temporal areas. Overall, the present case report documents that visual processing of the person's own face may be selectively compromised by a left‐sided hemispheric lesion disconnecting extra‐striate body‐ and face‐selective visual areas to self‐representation regions. Moreover, others' (full) face processing may be preserved, as compared with the impaired ability to discriminate others' body and face parts. [ABSTRACT FROM AUTHOR]

Individuals with developmental prosopagnosia (DP) experience severe face memory deficits that are often accompanied by impairments in face perception. Images of human facial features are better discriminated between when they are presented in the locations on the visual field that they typically appear in while viewing human faces in daily life, than in locations which they do not typically appear (i.e., better performance for eyes in the upper visual field, and better performance for mouths in the lower visual field). These feature-to-location tuning effects (FLEs) can be explained by a retinotopically organised visual statistical learning mechanism. We had a large group of DP participants (N = 64), a control group (N = 74) and a group of individuals with a mild form of DP (N = 58) complete a single-feature discrimination task to determine whether face perception deficits in DP can be accounted for by an impairment in face feature-to-location tuning. The results showed that individuals with DP did not have significant FLEs, suggesting a marked impairment in the underlying visual statistical learning mechanism. In contrast, the mild DP group showed normal FLE effects which did not differ from the control group. Both DP groups had impaired single-feature processing (SFP) as compared to the control group. We also examined the effects of age on FLEs and SFP. [ABSTRACT FROM AUTHOR]

We assembled a test battery to investigate developmental prosopagnosia (DP), a neurodevelopmental syndrome resulting in severe face recognition difficulties. To screen for general cognitive deficits that could explain poor face test performance, participants completed a fluid reasoning task using abstract shapes. This initial screening showed that DPs (n = 21) were more accurate than neurotypical controls (n = 90) but significantly slower, suggesting speed-accuracy trade-off. To address this, we calculated the Balanced Integration Score and found no group differences, highlighting that DPs clearly adopted a different strategy from controls. DPs’ longer response times (RT) on face tasks vs controls are commonly interpreted as evidence of impairment and of lengthy, atypical featural face processing. Our data suggest this interpretation may be unreliable since clear RT differences were also observed in two non-face tasks where DPs showed no accuracy impairment. Instead, slower RTs appear to reflect a strategy shift in DPs. [ABSTRACT FROM AUTHOR]

The faces we see in daily life exist on a continuum of familiarity, ranging from personally familiar to famous to unfamiliar faces. Thus, when assessing face recognition abilities, adequate evaluation measures should be employed to discriminate between each of these processes and their relative impairments. We here developed the Italian Famous Face Test (IT-FFT), a novel assessment tool for famous face recognition in typical and clinical populations. Normative data on a large sample (N = 436) of Italian individuals were collected, assessing both familiarity (d′) and recognition accuracy. Furthermore, this study explored whether individuals possess insights into their overall face recognition skills by correlating the Prosopagnosia Index-20 (PI-20) with the IT-FFT; a negative correlation between these measures suggests that people have a moderate insight into their face recognition skills. Overall, our study provides the first online-based Italian test for famous faces (IT-FFT), a test that could be used alongside other standard tests of face recognition because it complements them by evaluating real-world face familiarity, providing a more comprehensive assessment of face recognition abilities. Testing different aspects of face recognition is crucial for understanding both typical and atypical face recognition. [ABSTRACT FROM AUTHOR]

In non-clinical populations, facial features (eyes, nose, mouth) may vary in their contribution to face identity perception. Changes to whole faces are easier to detect than changes to individual features, and eye changes are typically easier to detect than mouth changes, which in turn are easier to detect than nose changes. However, how this differs for people with face recognition difficulties (developmental prosopagnosia; DP) and for individuals with superior face recognition abilities (super-recognisers; SR) is not clear; although findings from previous studies have suggested differences, the nature of this difference is not understood. The aim of this study was to examine whether differences in the ability to detect feature changes in DPs and SRs were (a) quantitative, meaning that the pattern across feature changes remained the same but there was an overall upwards or downwards shift in performance, or (b) qualitative, meaning that the pattern across feature changes was different. Using a change detection task in which individual face features (eyes, nose, mouth) changed between sequentially presented faces, we found that while prosopagnosics showed a quantitative difference in performance with a downwards shift across all conditions, super-recognisers only showed qualitative differences: they were better able to detect when the face was the same and were marginally (but not non-significantly) worse at detecting when the eyes changed. Further, the only condition which distinguished between the three groups was the ability to identify when the same face was presented, with SRs being better than controls, and controls being better than DPs. Our findings suggest that, in feature-matching tasks, differences for DPs are due to them being overall worse at the task, while SRs use a qualitatively different strategy. [ABSTRACT FROM AUTHOR]

Face recognition is important for both visual and social cognition. While prosopagnosia or face blindness has been known for seven decades and face-specific neurons for half a century, the molecular genetic mechanism is not clear. Here we report results after 17 years of research with classic genetics and modern genomics. From a large family with 18 congenital prosopagnosia (CP) members with obvious difficulties in face recognition in daily life, we uncovered a fully cosegregating private mutation in the MCTP2 gene which encodes a calcium binding transmembrane protein expressed in the brain. After screening through cohorts of 6589, we found more CPs and their families, allowing detection of more CP associated mutations in MCTP2. Face recognition differences were detected between 14 carriers with the frameshift mutation S80fs in MCTP2 and 19 noncarrying volunteers. Six families including one with 10 members showed the S80fs-CP correlation. Functional magnetic resonance imaging found association of impaired recognition of individual faces by MCTP2 mutant CPs with reduced repetition suppression to repeated facial identities in the right fusiform face area. Our results have revealed genetic predisposition of MCTP2 mutations in CP, 76 years after the initial report of prosopagnosia and 47 years after the report of the first CP. This is the first time a gene required for a higher form of visual social cognition was found in humans. [ABSTRACT FROM AUTHOR]

Although it is generally assumed that face recognition relies on holistic processing, whether face recognition deficits observed in Developmental Prosopagnosics (DPs) can be explained by impaired holistic processing is currently under debate. The mixed findings from past studies could be the consequence of DP's heterogeneous deficit nature and the use of different measures of holistic processing—the inversion, part-whole, and composite tasks—which showed a poor association among each other. The present study aimed to gain further insight into the role of holistic processing in DPs. Groups of DPs and neurotypicals completed three tests measuring holistic face processing and non-face objects (i.e., Navon task). At a group level, DPs showed (1) diminished, but not absent, inversion and part-whole effects, (2) comparable magnitudes of the composite face effect and (3) global precedence effect in the Navon task. However, single-case analyses showed that these holistic processing deficits in DPs are heterogeneous. [ABSTRACT FROM AUTHOR]

Developmental prosopagnosia (DP) is characterised by deficits in face identification. However, there is debate about whether these deficits are primarily perceptual, and whether they extend to other face processing tasks (e.g., identifying emotion, age, and gender; detecting faces in scenes). In this study, 30 participants with DP and 75 controls completed a battery of eight tasks assessing four domains of face perception (identity; emotion; age and gender; face detection). The DP group performed worse than the control group on both identity perception tasks, and one task from each other domain. Both identity perception tests uniquely predicted DP/control group membership, and performance on two measures of face memory. These findings suggest that deficits in DP may arise from issues with face perception. Some non-identity tasks also predicted DP/control group membership and face memory, even when face identity perception was accounted for. Gender perception and speed of face detection consistently predicted unique variance in group membership and face memory; several other tasks were only associated with some measures of face recognition ability. These findings indicate that face perception deficits in DP may extend beyond identity perception. However, the associations between tasks may also reflect subtle aspects of task demands or stimuli. [ABSTRACT FROM AUTHOR]

The Cambridge Face Memory Test (CFMT) is one of the most important measures of individual differences in face recognition and for the diagnosis of prosopagnosia. Having two different CFMT versions using a different set of faces seems to improve the reliability of the evaluation. However, at the present time, there is only one Asian version of the test. In this study, we present the Cambridge Face Memory Test – Chinese Malaysian (CFMT-MY), a novel Asian CFMT using Chinese Malaysian faces. In Experiment 1, Chinese Malaysian participants (N = 134) completed two versions of the Asian CFMT and one object recognition test. The CFMT-MY showed a normal distribution, high internal reliability, high consistency and presented convergent and divergent validity. Additionally, in contrast to the original Asian CFMT, the CFMT-MY showed an increasing level of difficulties across stages. In Experiment 2, Caucasian participants (N = 135) completed the two versions of the Asian CFMT and the original Caucasian CFMT. Results showed that the CFMT-MY exhibited the other-race effect. Overall, the CFMT-MY seems to be suitable for the diagnosis of face recognition difficulties and could be used as a measure of face recognition ability by researchers who wish to examine face-related research questions such as individual differences or the other-race effect. [ABSTRACT FROM AUTHOR]

It is still a matter of debate whether developmental prosopagnosia is a disorder selective to faces or whether object recognition is also affected. In a previous study, based on a small sample of developmental prosopagnosics (DPs; N = 10), we found impairments in both domains although the difficulties were most pronounced for faces. Importantly, impairments with faces and objects were systematically related. We suggested that that the seemingly disproportional impairment for faces in DP was likely to reflect differences between stimulus categories in visual similarity. Here, we aimed to replicate these findings in a larger, independent sample of DPs (N = 21) using the same experimental paradigms. Contrary to our previous results, we found no disproportional effect of visual similarity on performance with faces or objects in the new DP group when compared to controls (N = 21). The new DP group performed within the control range, and significantly better than the old DP-group, on sensitive and demanding object recognition tasks, and we can demonstrate a classical dissociation between face and object recognition at the group level. These findings are perhaps the strongest evidence yet presented for a face-specific deficit in developmental prosopagnosia. [ABSTRACT FROM AUTHOR]

Existing evidence suggests that developmental prosopagnosia (DP) is a surprisingly prevalent condition, with some individuals describing lifelong difficulties with facial identity recognition. Together with case reports of multiple family members with the condition, this evidence suggests that DP is inherited in at least some instances. Here, we offer some novel case series that further support the heritability of the condition. First, we describe five adult siblings who presented to our lab with symptoms of DP. Second, for the first known time in the literature, we describe a pair of adult identical twins who contacted us in the belief that they both experience DP. The condition was confirmed in three of the five siblings (with minor symptoms observed in the remaining two) and in both twins. Supplementary assessments suggested that all individuals also experienced some degree of difficulty with facial identity perception, but that object recognition was preserved. These findings bolster the evidence supporting the heritability of DP and suggest that it can be a specific impairment in some cases. [ABSTRACT FROM AUTHOR]

Developmental prosopagnosia (DP) is a condition that indicates the inability to recognize individuals by their faces from birth, without any history of brain damage. The assessment of face recognition ability and diagnosis of DP involve the use of face tests such as the Cambridge Face Memory Test (CFMT) and the Cambridge Face Perception Test, along with self-reported measures like the 20-Item Prosopagnosia Index (PI20). Face recognition accuracy is affected by anxiety. However, previous studies on the relationship between face recognition ability and anxiety have not used the PI20 measure. This study aimed to investigate the relationship between self-reported measures of face recognition ability and anxiety tendencies among healthy young individuals for DP diagnosis and its implications. We used a face recognition test, involving the PI20, CFMT, Visual Perception Test for Agnosia–Famous Face Test (VPTA–FFT), and State-Trait Anxiety Inventory (STAI). We assessed the performance of 116 Japanese young adults (75 females, median age of 20.7 years, with a standard deviation of 1.2). Subsequently, we conducted a statistical analysis to examine the relationship between the outcomes of the face recognition tests and STAI scores using Pearson correlation analysis and single correlation coefficients. The results showed a positive correlation between state anxiety and PI20 (r = 0.308, p = 0.007), and a weak positive correlation was also observed between trait anxiety and PI20 (r = 0.268, p = 0.04). In contrast, there was no correlation between CFMT and VPTA–FFT with respect to STAI. The results of the hierarchical multiple regression analysis also suggested that the correlation between the performance on the PI20 (self-report) and objective measures of face recognition performance (the CFMT and the VPTA–FFT) are driven by differences in anxiety. This study is the first to explore the relationship between face recognition abilities and anxiety using the PI20 self-report measure. There are implications for future research on the diagnosis of DP and the relationship between anxiety and face recognition.

Individuals with developmental prosopagnosia (DP) experience severe face memory deficits that are often accompanied by impairments in face perception. Images of human facial features are better discriminated between when they are presented in the locations on the visual field that they typically appear in while viewing human faces in daily life, than in locations which they do not typically appear (i.e., better performance for eyes in the upper visual field, and better performance for mouths in the lower visual field). These feature-to-location tuning effects (FLEs) can be explained by a retinotopically organised visual statistical learning mechanism. We had a large group of DP participants (N = 64), a control group (N = 74) and a group of individuals with a mild form of DP (N = 58) complete a single-feature discrimination task to determine whether face perception deficits in DP can be accounted for by an impairment in face feature-to-location tuning. The results showed that individuals with DP did not have significant FLEs, suggesting a marked impairment in the underlying visual statistical learning mechanism. In contrast, the mild DP group showed normal FLE effects which did not differ from the control group. Both DP groups had impaired single-feature processing (SFP) as compared to the control group. We also examined the effects of age on FLEs and SFP.

Prosopagnosia, a neurological condition affecting perception and differentiation of faces, is categorized as either acquired or developmental (present since birth). Acquired cases of prosopagnosia are usually caused by right hemisphere or bilateral damage. We present a right-handed 17-year-old male with a history of focal epilepsy and a new diagnosis of prosopagnosia due to a perinatal stroke affecting the left lingual gyrus, a structure in close proximity to the fusiform face area. In addition to showing that early acquired cases of prosopagnosia may go unrecognized, this case shows that left hemisphere lesions may also affect facial recognition. It is important to screen for prosopagnosia via comprehensive neuropsychological evaluation in patients with lesions proximal to the fusiform face area.

Developmental prosopagnosia is characterized by severe, lifelong difficulties when recognizing facial identity. Unfortunately, the most common diagnostic assessment (Cambridge Face Memory Test) misses 50–65% of individuals who believe that they have this condition. This results in such excluded cases' absence from scientific knowledge, effect sizes of impairment potentially overestimated, treatment efficacy underrated, and may elicit in them a negative experience of research. To estimate their symptomology and group-level impairments in face processing, we recruited a large cohort who believes that they have prosopagnosia. Matching prior reports, 56% did not meet criteria on the Cambridge Face Memory Test. However, the severity of their prosopagnosia symptoms and holistic perception deficits were comparable to those who did meet criteria. Excluded cases also exhibited face perception and memory impairments that were roughly one standard deviation below neurotypical norms, indicating the presence of objective problems. As the prosopagnosia index correctly classified virtually every case, we propose it should be the primary method for providing a diagnosis, prior to subtype categorization. We present researchers with a plan on how they can analyze these excluded prosopagnosia cases in their future work without negatively impacting their traditional findings. We anticipate such inclusion will enhance scientific knowledge, more accurately estimate effect sizes of impairments and treatments, and identify commonalities and distinctions between these different forms of prosopagnosia. Owing to their atypicalities in visual perception, we recommend that the prosopagnosia index should be used to screen out potential prosopagnosia cases from broader vision research. [ABSTRACT FROM AUTHOR]

Faces play a crucial role in social interactions. Developmental prosopagnosia (DP) refers to the lifelong difficulty in recognizing faces despite the absence of obvious signs of brain lesions. In recent decades, the neural substrate of this condition has been extensively investigated. While early neuroimaging studies did not reveal significant functional and structural abnormalities in the brains of individuals with developmental prosopagnosia (DPs), recent evidence identifies abnormalities at multiple levels within DPs' face-processing networks. The current work aims to provide an overview of the convergent and contrasting findings by examining twenty-five years of neuroimaging literature on the anatomo-functional correlates of DP. We included 55 original papers, including 63 studies that compared the brain structure (MRI) and activity (fMRI, EEG, MEG) of healthy control participants and DPs. Despite variations in methods, procedures, outcomes, sample selection, and study design, this scoping review suggests that morphological, functional, and electrophysiological features characterize DPs' brains, primarily within the ventral visual stream. Particularly, the functional and anatomical connectivity between the Fusiform Face Area and the other face-sensitive regions seems strongly impaired. The cognitive and clinical implications as well as the limitations of these findings are discussed in light of the available knowledge and challenges in the context of DP. [ABSTRACT FROM AUTHOR]

La prosopagnosia es un tipo de agnosia visual caracterizada por la incapacidad para reconocer rostros. En estudios diagnósticos funcionales, el reconocimiento e interpretación de rostros se atribuye principal, pero no únicamente, a los giros fusiforme, lingual y parahipocampal del lóbulo temporal, irrigados por ramas de la arteria cerebral posterior. En este artículo se profundiza en las bases neuroanatómicas de este tipo de agnosia visual, así como en las etiologías más frecuentes, el abordaje clínico y los hallazgos imagenológicos. [ABSTRACT FROM AUTHOR]

BackgroundProsopagnosia is a rare neurological condition characterized by the impairment of face perception with preserved visual processing and cognitive functioning and is associated with injury to the fusiform gyrus and inferior longitudinal fasciculus (ILF). Reports of this clinical impairment following resection of right temporal lobe diffuse gliomas in the absence of contralateral injury are exceedingly scarce and not expected as a complication of surgery.ObservationsThe authors describe the case of a young female patient found to have an incidental diffuse glioma in the right inferior temporal lobe despite evidence of preoperative ILF disruption by the tumor. Following resection of the lesion, despite the preoperative disruption to the ILF by the tumor, the patient developed prosopagnosia. There was no evidence of contralateral, left-sided ILF injury.LessonsGiven the significant functional impairment associated with prosopagnosia, neurosurgeons should be aware of the exceedingly rare possibility of a visual-processing deficit following unilateral and, in this case, right-sided inferior temporal lobe glioma resections. More investigation is needed to determine whether preoperative testing can determine dominance of facial-processing networks for patients with lesions in the right inferior posterior temporooccipital lobe and whether intraoperative mapping could help prevent this complication.

In non-clinical populations, facial features (eyes, nose, mouth) may vary in their contribution to face identity perception. Changes to whole faces are easier to detect than changes to individual features, and eye changes are typically easier to detect than mouth changes, which in turn are easier to detect than nose changes. However, how this differs for people with face recognition difficulties (developmental prosopagnosia; DP) and for individuals with superior face recognition abilities (super-recognisers; SR) is not clear; although findings from previous studies have suggested differences, the nature of this difference is not understood. The aim of this study was to examine whether differences in the ability to detect feature changes in DPs and SRs were (a) quantitative, meaning that the pattern across feature changes remained the same but there was an overall upwards or downwards shift in performance, or (b) qualitative, meaning that the pattern across feature changes was different. Using a change detection task in which individual face features (eyes, nose, mouth) changed between sequentially presented faces, we found that while prosopagnosics showed a quantitative difference in performance with a downwards shift across all conditions, super-recognisers only showed qualitative differences: they were better able to detect when the face was the same and were marginally (but not non-significantly) worse at detecting when the eyes changed. Further, the only condition which distinguished between the three groups was the ability to identify when the same face was presented, with SRs being better than controls, and controls being better than DPs. Our findings suggest that, in feature-matching tasks, differences for DPs are due to them being overall worse at the task, while SRs use a qualitatively different strategy.

Aphantasia and prosopagnosia are both rare conditions with impairments in visual cognition. While prosopagnosia refers to a face recognition deficit, aphantasics exhibit a lack of mental imagery. Current object recognition theories propose an interplay of perception and mental representations, making an association between recognition performance and visual imagery plausible. While the literature assumes a link between aphantasia and prosopagnosia, other impairments in aphantasia have been shown to be rather global. Therefore, we assumed that aphantasics do not solely exhibit impairments in face recognition but rather in general visual recognition performance, probably moderated by stimulus complexity. To test this hypothesis, 65 aphantasics were compared to 55 controls in a face recognition task, the Cambridge Face Memory Test, and a corresponding object recognition task, the Cambridge Car Memory Test. In both tasks, aphantasics performed worse than controls, indicating mild recognition deficits without face-specificity. Additional correlations between imagery vividness and performance in both tasks were found, suggesting that visual imagery influences visual recognition not only in imagery extremes. Stimulus complexity produced the expected moderation effect but only for the whole imagery-spectrum and only with face stimuli. Overall, the results imply that aphantasia is linked to a general but mild deficit in visual recognition. [ABSTRACT FROM AUTHOR]

Children with prosopagnosia, also known as face blindness, struggle to recognize the faces of acquaintances, which can have a negative impact on their social interactions and overall functioning. This paper reviews existing research on interventions for children with prosopagnosia, including compensatory and remedial strategies, and provides a summary and comparison of their effectiveness. However, despite the availability of these interventions, their effectiveness remains limited and constrained by various factors. The lack of a widely accepted treatment for children with prosopagnosia emphasizes the need for further research to improve intervention strategies. Last, three future research directions were proposed to improve interventions for prosopagnosia, including ecological approaches, the social challenges faced by children, and new potential intervention methods. [ABSTRACT FROM AUTHOR]

A 42-year-old lady presented with acute aneurysmal subarachnoid haemorrhage and developed difficulty recognising faces (prosopagnosia), inability to process visual information in busy environments (simultagnosia) and difficulty to read (alexia). She was subsequently found to have superficial siderosis on MRI. [ABSTRACT FROM AUTHOR]

Developmental prosopagnosia is a relatively common visuo-cognitive condition, characterised by impaired facial identity recognition. Impairment severity appears to reside on a continuum, however, it is unknown whether instances of milder deficits reflect the successful use of spontaneous (typical) face recognition strategies, or the application of extraneous compensatory cues to recognition. Here, we explore this issue in two studies. First, 23 adults with developmental prosopagnosia were asked about their use of spontaneous versus compensatory face recognition techniques in everyday life, using a series of closed- and open-ended questions. Second, the same participants performed a computerised famous face recognition task where they were asked to provide reasons why they could make any successful identifications. Findings from both studies suggest that people with developmental prosopagnosia can successfully, and quite frequently, use compensatory strategies to recognition, and that these cues support the majority of instances of preserved familiar face recognition. In contrast, 16 of the 23 participants were able to spontaneously recognise familiar faces on at least some occasions, but there were vast individual differences in frequencies of success. These findings have important implications for our conceptualisation of the condition, as well as for diagnostic practice. [ABSTRACT FROM AUTHOR]

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Provoked overt recognition refers to the fact that patients with acquired prosopagnosia can sometimes recognize faces when presented in arrays of individuals from the same category (e.g., actors or politicians). We ask whether a prosopagnosic patient might experience recognition when presented with multiple different images of the same face simultaneously. Over two sessions, patient Herschel, a 66-year-old British man with acquired prosopagnosia, viewed face images individually or in arrays. On several occasions he failed to recognize single photos of an individual but successfully identified that person when the same photos were presented together. For example, Herschel failed to recognize any individual images of King Charles or Paul McCartney but recognised both in arrays of the same photos. Like reports based on category membership, overt recognition was transient and inconsistent. These findings are discussed in terms of models of covert recognition, alongside more recent research on within-person variability for face perception. [ABSTRACT FROM AUTHOR]

Objective: Although the prevalence of dementia is increasing globally, the data related to dementia patients living in North Cyprus is very limited. The aim of this study was to evaluate the demographic and neuropsychological characteristics of patients diagnosed with neurodegenerative dementia. Materials and Methods: The data of 105 patients with neurodegenerative dementia, who were followed up in the neurology departments of the Dr. Suat Günsel University of Kyrenia Hospital and Near East University Hospital between 2018 and 2021, were collected both retrospectively and prospectively. The patients underwent diagnostic procedures by neurologic examination, neuropsychologic evaluation based on the measures of attention and executive functions, memory, language, mood and visuospatial perception and neuroimaging. All the results were evaluated statistically according to the dementia subgroups. Results: Out of 105 patients, 58 were female and 47 were male. The mean age was 74.34±10.41. The most common dementia type was Alzheimer's dementia (AD) (77/105), followed by Lewy body dementia (LBD) (15/105) and frontotemporal dementia (FTD) (13/105). The initial median mini mental state examination (MMSE) score was 20. There was a positive correlation between years in higher education and lower MMSE scores. Out of the 105 patients, 81 spoke Turkish and 13 spoke English as a native language. Depression occurred more frequent in earlier stages and milder cases. The visuospatial functions were affected more in LBD and FTD patients when compared to AD patients. Prosopagnosia was significant for the differential diagnosis of Parkinson's dementia from other forms of dementia. Also, a decrease in categorical fluency was observed in patients with higher depression scores. Conclusion: These results are important as this is the first study determining the detailed dementia subgroups and neuropsychologic profiles of dementia patients living in North Cyprus. [ABSTRACT FROM AUTHOR]

Existing evidence suggests that developmental prosopagnosia (DP) is a surprisingly prevalent condition, with some individuals describing lifelong difficulties with facial identity recognition. Together with case reports of multiple family members with the condition, this evidence suggests that DP is inherited in at least some instances. Here, we offer some novel case series that further support the heritability of the condition. First, we describe five adult siblings who presented to our lab with symptoms of DP. Second, for the first known time in the literature, we describe a pair of adult identical twins who contacted us in the belief that they both experience DP. The condition was confirmed in three of the five siblings (with minor symptoms observed in the remaining two) and in both twins. Supplementary assessments suggested that all individuals also experienced some degree of difficulty with facial identity perception, but that object recognition was preserved. These findings bolster the evidence supporting the heritability of DP and suggest that it can be a specific impairment in some cases.

La demencia frontotemporal derecha es una variante anatómica de la demencia frontotemporal. Se asocia con síntomas cognitivos y comportamentales característicos, entre los que se destacan: agnosia topográfica, desorientación espacial, prosopagnosia, conductas obsesivas, agresividad, impulsividad, desinhibición y pérdida de la empatía. Se reportan dos casos, que desde el punto de vista clínico e imaginológico, resultan ilustrativos de la variante derecha en la demencia frontotemporal.

Subjects often look towards to previous location of a stimulus related to a task even when that stimulus is no longer visible. In this study we asked whether this effect would be preserved or reduced in subjects with developmental prosopagnosia. Participants learned faces presented in video-clips and then saw a brief montage of four faces, which was replaced by a screen with empty boxes, at which time they indicated whether the learned face had been present in the montage. Control subjects were more likely to look at the blank location where the learned face had appeared, on both hit and miss trials, though the effect was larger on hit trials. Prosopagnosic subjects showed a reduced effect, though still better on hit than on miss trials. We conclude that explicit accuracy and our implicit looking at nothing effect are parallel effects reflecting the strength of the neural activity underlying face recognition. [ABSTRACT FROM AUTHOR]

Understanding how the human brain recognizes faces is a primary scientific goal in cognitive neuroscience. Given the limitations of the monkey model of human face recognition, a key approach in this endeavor is the recording of electrophysiological activity with electrodes implanted inside the brain of human epileptic patients. However, this approach faces a number of challenges that must be overcome for meaningful scientific knowledge to emerge. Here we synthesize a 10 year research program combining the recording of intracerebral activity (StereoElectroEncephaloGraphy, SEEG) in the ventral occipito-temporal cortex (VOTC) of large samples of participants and fast periodic visual stimulation (FPVS), to objectively define, quantify, and characterize the neural basis of human face recognition. These large-scale studies reconcile the wide distribution of neural face recognition activity with its (right) hemispheric and regional specialization and extend face-selectivity to anterior regions of the VOTC, including the ventral anterior temporal lobe (VATL) typically affected by magnetic susceptibility artifacts in functional magnetic resonance imaging (fMRI). Clear spatial dissociations in category-selectivity between faces and other meaningful stimuli such as landmarks (houses, medial VOTC regions) or written words (left lateralized VOTC) are found, confirming and extending neuroimaging observations while supporting the validity of the clinical population tested to inform about normal brain function. The recognition of face identity – arguably the ultimate form of recognition for the human brain – beyond mere differences in physical features is essentially supported by selective populations of neurons in the right inferior occipital gyrus and the lateral portion of the middle and anterior fusiform gyrus. In addition, low-frequency and high-frequency broadband iEEG signals of face recognition appear to be largely concordant in the human association cortex. We conclude by outlining the challenges of this research program to understand the neural basis of human face recognition in the next 10 years. [ABSTRACT FROM AUTHOR]

Developmental prosopagnosia (DP) is a neurodevelopmental condition characterized by face recognition problems. Psychometrically sound self-report measures of face recognition problems are important tools in classification of DP. A widely used measure of such problems is the 20-item prosopagnosia index (PI20). Here, we present a Danish translation of the PI20 (PI20DK). We administered the PI20DK alongside three objective measures of face and object processing performance to 119 participants to validate the PI20DK. Further, we assess the underlying factor structure of the PI20DK. Finally, as the first study in the field, we investigate the association between self-reported face recognition ability and face perception performance. The project was preregistered prior to data collection. The results suggest excellent convergent validity, discriminant validity and internal consistency for the PI20DK. A confirmatory factor analysis, however, indicates a suboptimal fit of the PI20DK to a one factor solution. An investigation of the association between the PI20DK and face perception suggests that the poor fit may reflect that the PI20DK measures problems with face recognition in general and not specifically face memory problems. [ABSTRACT FROM AUTHOR]

Neurodevelopmental disorders are categorized and studied according to their manifestations as distinct syndromes. For instance, congenital prosopagnosia and dyslexia have largely non-overlapping research literatures and clinical pathways for diagnosis and intervention. On the other hand, the high incidence of neurodevelopmental comorbidities or co-existing extreme strengths and weaknesses suggest that transdiagnostic commonalities may be greater than currently appreciated. The core-periphery model holds that brain regions within the stable core perceptual and motor regions are more densely connected to one another compared to regions in the flexible periphery comprising multimodal association regions. This model provides a framework for the interpretation of neural data in normal development and clinical disorders. Considering network-level commonalities reported in studies of neurodevelopmental disorders, variability in multimodal association cortex connectivity may reflect a shared origin of seemingly distinct neurodevelopmental disorders. This framework helps to explain both comorbidities in neurodevelopmental disorders and profiles of strengths and weaknesses attributable to competitive processing between cognitive systems within an individual. [ABSTRACT FROM AUTHOR]