Face naming and recollection represent key memory deficits in developmental prosopagnosia.

Previous studies have found that face perception deficits do not fully account for the severity of face recognition deficits in developmental prosopagnosia (DP). Researchers have begun identifying deficient memory mechanisms such as impaired face recollection, but these findings require replication, and further characterization of additional memory deficits is necessary. Our goals were to replicate prior findings of face recollection impairment in DP and extend these findings to assess different types of face associative memory. We had 69 DPs and 99 controls perform a face perception battery as well as three face memory tasks: 1) Old/New task with confidence ratings to calculate recollection and familiarity using ROC analysis, 2) Face/Scene task to examine remember-know judgments and contextual memory for faces, and 3) Face-Name/Occupation task to assess the ability to learn semantic associations with faces. Compared to controls, DPs showed poorer recollection and familiarity across both Old/New and Face/Scene tasks as well as reduced scene accuracy for correct faces. Of these differences, only Old/New recollection remained significant after controlling for group differences in face perception abilities. In the Face-Name/Occupation task, after controlling for face perception, DPs showed poorer recall of names than controls but performed similarly in recalling occupations. Finally, we found that DPs with major, mild, and no face perception deficits showed consistent impairments in Old/New recollection and face-naming, and larger perceptual deficits were associated with larger memory deficits. Together, these results provide several mechanistic insights into the nature of memory deficits in DPs and have diagnostic and treatment implications.

Exploration of the Links Between Psychosocial Well-being and Face Recognition Skills in a French-Speaking Sample.

Face recognition abilities vary tremendously in the general population. People at the lower end of the spectrum, those with developmental prosopagnosia, report stress, anxiety or social interaction issues due to their poor face recognition abilities. It is thus important to develop adequate diagnostic tools convenient to use for clinicians and to examine relationships between face recognition skills and negative affects. In the present study, we provide a validated French translation of the 20-item prosopagnosia index (PI20), a self-report measure used to detect people with developmental facial identity recognition deficits (Shah et al., 2015; Tsantani et al., 2021). We also examined links between face recognition skills measured with the PI20 and a standard face recognition test (Cambridge face memory test-CFMT; Duchaine & Nakayama, 2006) and measures of social anxiety (social interaction anxiety scale, social phobia scale) and negative affects (state trait anxiety scale, Beck depression inventory). We did not find any significant correlation between the CFMT and measures of psychosocial well-being and only found a weak positive association between the PI20 and social interaction anxiety. Although this association is weak and warrants further research, raising awareness about developmental face recognition issues may help improve the well-being of people with facial identity recognition deficits and provide new investigation or intervention avenues for clinicians who treat patients with social interaction anxiety.

Visual self-face and self-body recognition in a left-brain-damaged prosopagnosic patient.

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.

Social Interaction Anxiety in Developmental Prosopagnosia: Prevalence, Severity, and Individual Differences.

OBJECTIVE: Although elevated social anxiety in developmental prosopagnosia (DP) has been reported in anecdotal and qualitative studies, the current study sought to better quantify the prevalence, severity, and moderators of social anxiety in a large DP sample. METHOD: A total of 88 DPs and 58 controls completed the validated Social Interaction Anxiety Scale and assessments of face recognition, autism traits, personality (Big Five Inventory), and coping strategies. RESULTS: DPs reported greater social anxiety symptoms (M = 30.25) than controls (M = 17.19), with 44% of DPs above a probable clinical cutoff compared with 14% of controls. Exploratory analyses revealed that DPs' social anxiety was more specific to items pertaining to mixing socially or interacting with unfamiliar people. Notably, several DPs reported minimal social anxiety, which was associated with being more extraverted and having fewer autism traits. A follow-up survey revealed that extraverted DPs more openly disclosed face recognition inabilities than introverted DPs, which may be a factor in their reduced social anxiety. CONCLUSION: These results better quantify the potential serious psychosocial consequences of DP and highlight the importance of individual differences as well as targeted intervention.

Prosopagnosia in the context of right handedness, left hemisphere perinatal stroke, epileptogenic cyst, and focal epilepsy: A pre-surgical case report.

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.

The impact of bilateral versus unilateral anterior temporal lobe damage on face recognition, person knowledge and semantic memory.

The functional importance of the anterior temporal lobes (ATLs) has come to prominence in two active, albeit unconnected literatures-(i) face recognition and (ii) semantic memory. To generate a unified account of the ATLs, we tested the predictions from each literature and examined the effects of bilateral versus unilateral ATL damage on face recognition, person knowledge, and semantic memory. Sixteen people with bilateral ATL atrophy from semantic dementia (SD), 17 people with unilateral ATL resection for temporal lobe epilepsy (TLE; left = 10, right = 7), and 14 controls completed tasks assessing perceptual face matching, person knowledge and general semantic memory. People with SD were impaired across all semantic tasks, including person knowledge. Despite commensurate total ATL damage, unilateral resection generated mild impairments, with minimal differences between left- and right-ATL resection. Face matching performance was largely preserved but slightly reduced in SD and right TLE. All groups displayed the familiarity effect in face matching; however, it was reduced in SD and right TLE and was aligned with the level of item-specific semantic knowledge in all participants. We propose a neurocognitive framework whereby the ATLs underpin a resilient bilateral representation system that supports semantic memory, person knowledge and face recognition.

Impaired Face Feature-to-Location Statistical Learning and Single-Feature Discrimination in Developmental Prosopagnosia.

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.

Humans' extreme face recognition abilities challenge the well-established familiarity effect.

Classification performance is better for learned than unlearned stimuli. This was also reported for faces, where identity matching of unfamiliar faces is worse than for familiar faces. This familiarity advantage led to the conclusion that variability across appearances of the same identity is partly idiosyncratic and cannot be generalized from familiar to unfamiliar identities. Recent advances in machine vision challenge this claim by showing that the performance for untrained (unfamiliar) identities reached the level of trained identities as the number of identities that the algorithm is trained with increases. We therefore asked whether humans who reportedly can identify a vast number of identities, such as super recognizers, may close the gap between familiar and unfamiliar face classification. Consistent with this prediction, super recognizers classified unfamiliar faces just as well as typical participants who are familiar with the same faces, on a task that generates a sizable familiarity effect in controls. Additionally, prosopagnosics' performance for familiar faces was as bad as that of typical participants who were unfamiliar with the same faces, indicating that they struggle to learn even identity-specific information. Overall, these findings demonstrate that by studying the extreme ends of a system's ability we can gain novel insights into its actual capabilities.

Autistic adults have insight into their relative face recognition ability.

The PI20 is a self-report questionnaire that assesses the presence of lifelong face recognition difficulties. The items on this scale ask respondents to assess their face recognition ability relative to the rest of the population, either explicitly or implicitly. Recent reports suggest that the PI20 scores of autistic participants exhibit little or no correlation with their performance on the Cambridge Face Memory Test-a key measure of face recognition ability. These reports are suggestive of a meta-cognitive deficit whereby autistic individuals are unable to infer whether their face recognition is impaired relative to the wider population. In the present study, however, we observed significant correlations between the PI20 scores of 77 autistic adults and their performance on two variants of the Cambridge Face Memory Test. These findings indicate that autistic individuals can infer whether their face recognition ability is impaired. Consistent with previous research, we observed a wide spread of face recognition abilities within our autistic sample. While some individuals approached ceiling levels of performance, others met the prevailing diagnostic criteria for developmental prosopagnosia. This variability showed little or no association with non-verbal intelligence, autism severity, or the presence of co-occurring alexithymia or ADHD.

EXPRESS: Prosopagnosia is highly comorbid in individuals with probable developmental coordination disorder (DCD).

Developmental co-ordination disorder (DCD) is characterised by difficulties in motor control and coordination from early childhood. While problems processing facial identity are often associated with neurodevelopmental conditions, such issues have never been directly tested in adults with DCD. We tested this possibility through a range of tasks, and assessed the prevalence of developmental prosopagnosia (i.e., lifelong difficulties with faces), in a group comprising individuals who self-reported a diagnosis of, or suspected that they had, DCD. Strikingly, we found 53% of this probable DCD group met recently recommended criteria for a diagnosis of prosopagnosia, with 22% acquiring a diagnosis using traditional cognitive task-based methods. Moreover, their problems with faces were apparent on both unfamiliar and familiar face memory tests, as well as on a facial perception task (i.e., could they tell faces apart). Positive correlations were found between self-report measures assessing movement and coordination problems, and objective difficulties on experimental face identity processing tasks, suggesting widespread neurocognitive disruption in DCD. Importantly, issues in identity processing in our probable DCD group remained even after excluding participants with comorbid conditions traditionally associated with difficulties in face recognition, i.e., autism and dyslexia. We recommend that any diagnostic test for DCD should include an assessment for prosopagnosia. Given the high prevalence of prosopagnosia in our probable DCD group, and the positive correlations between DCD and prosopagnosia symptoms, there may be a stronger link between movement and facial identity abilities than previously thought.

Improving the DSM-5 approach to cognitive impairment: Developmental prosopagnosia reveals the need for tailored diagnoses.

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.

The heterogeneity of holistic processing profiles in developmental prosopagnosia: holistic processing is impaired but not absent.

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.

Familiarity enhances functional connectivity between visual and nonvisual regions of the brain during natural viewing.

We explored the neural correlates of familiarity with people and places using a naturalistic viewing paradigm. Neural responses were measured using functional magnetic resonance imaging, while participants viewed a movie taken from Game of Thrones. We compared inter-subject correlations and functional connectivity in participants who were either familiar or unfamiliar with the TV series. Higher inter-subject correlations were found between familiar participants in regions, beyond the visual brain, that are typically associated with the processing of semantic, episodic, and affective information. However, familiarity also increased functional connectivity between face and scene regions in the visual brain and the nonvisual regions of the familiarity network. To determine whether these regions play an important role in face recognition, we measured responses in participants with developmental prosopagnosia (DP). Consistent with a deficit in face recognition, the effect of familiarity was significantly attenuated across the familiarity network in DP. The effect of familiarity on functional connectivity between face regions and the familiarity network was also attenuated in DP. These results show that the neural response to familiarity involves an extended network of brain regions and that functional connectivity between visual and nonvisual regions of the brain plays an important role in the recognition of people and places during natural viewing.

Face Feature Change Detection Ability in Developmental Prosopagnosia and Super-Recognisers.

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.

Isolated prosopagnosia caused by damage to the right inferior longitudinal fasciculus: a case report.

Prosopagnosia is a cognitive disorder in which facial recognition is severely impaired despite normal vision and intelligence. Prosopagnosia was first reported in the 1800s, but its cause remains unclear. Although other neurological symptoms are often present, some patients have pure prosopagnosia. The bilateral occipital lobes are believed to be associated with symptoms. Recent brain imaging techniques have identified the right fusiform gyrus (rFG), located at the junction of the right occipital temporal lobe, as the affected region. In this report, we present a case of associative prosopagnosia with no concomitant symptoms in a 76-year-old man. Brain magnetic resonance imaging detected a subcortical hemorrhage in the right temporal lobe. Using tractography based on diffusion tensor imaging, we visualized atrophy of the right inferior longitudinal fasciculus (ILF). This is the first time tractography has been used to show a clear association between associative prosopagnosia and ILF damage projecting from the rFG.

Perceptual heterogeneity in developmental prosopagnosia is continuous, not categorical.

Developmental prosopagnosia (DP) is associated with considerable perceptual heterogeneity, though the nature of this heterogeneity and whether there are discrete subgroups versus continuous deficits remains unclear. Bennetts et al. (2022) recently found that holistic versus featural processing deficits distinguished discrete DP subgroups, but their sample was relatively small (N = 37), and subgroups were defined using a single task. To characterize perceptual heterogeneity in DPs more comprehensively, we administered a broad face perception battery to a large sample of 109 DPs and 134 controls, including validated measures of face matching (Cambridge Face Perception Test - CFPT, Computerized Benton Facial Recognition Test, Same/Different Face Matching Task), holistic processing (Part-Whole Task), and feature processing (Georges Task and Part-Whole part trials). When examining face matching measures, DPs exhibited a similar distribution of performance as controls, though shifted towards impairment by an average of 1.4 SD. We next applied Bennetts (2022) hierarchical clustering approach and k-means clustering to the CFPT upright, inverted, and inversion index measures, similarly finding one group of DPs with poorer inverted face performance and another with a decreased face inversion effect (holistic processing). However, these subgroup differences failed to generalize to other measures of feature and holistic processing beyond the CFPT. We finally ran hierarchical and k-means cluster analyses on our larger battery of face matching, feature, and holistic processing measures. Results clearly showed subgroups with generally better versus worse performance across all measures, with the distinction between groups being somewhat arbitrary. Together, these findings support a continuous account of DP perceptual heterogeneity, with performance differing primarily across all aspects of face perception.

Neural computations in prosopagnosia.

We report an investigation of the neural processes involved in the processing of faces and objects of brain-lesioned patient PS, a well-documented case of pure acquired prosopagnosia. We gathered a substantial dataset of high-density electrophysiological recordings from both PS and neurotypicals. Using representational similarity analysis, we produced time-resolved brain representations in a format that facilitates direct comparisons across time points, different individuals, and computational models. To understand how the lesions in PS's ventral stream affect the temporal evolution of her brain representations, we computed the temporal generalization of her brain representations. We uncovered that PS's early brain representations exhibit an unusual similarity to later representations, implying an excessive generalization of early visual patterns. To reveal the underlying computational deficits, we correlated PS' brain representations with those of deep neural networks (DNN). We found that the computations underlying PS' brain activity bore a closer resemblance to early layers of a visual DNN than those of controls. However, the brain representations in neurotypicals became more akin to those of the later layers of the model compared to PS. We confirmed PS's deficits in high-level brain representations by demonstrating that her brain representations exhibited less similarity with those of a DNN of semantics.

Disentangling developmental prosopagnosia: A scoping review of terms, tools and topics.

The goal of this preregistered scoping review is to create an overview of the research on developmental prosopagnosia (DP). Through analysis of all empirical studies of DP in adults, we investigate 1) how DP is conceptualized and defined, 2) how individuals are classified with DP and 3) which aspects of DP are investigated in the literature. We reviewed 224 peer-reviewed studies of DP. Our analysis of the literature reveals that while DP is predominantly defined as a lifelong face recognition impairment in the absence of acquired brain injury and intellectual/cognitive problems, there is far from consensus on the specifics of the definition with some studies emphasizing e.g., deficits in face perception, discrimination and/or matching as core characteristics of DP. These differences in DP definitions is further reflected in the vast heterogeneity in classification procedures. Only about half of the included studies explicitly state how they classify individuals with DP, and these studies adopt 40 different assessment tools. The two most frequently studied aspects of DP are the role of holistic processing and the specificity of face processing, and alongside a substantial body of neuroimaging studies of DP, this paints a picture of a research field whose scientific interests and aims are rooted in cognitive neuropsychology and neuroscience. We argue that these roots - alongside the heterogeneity in DP definition and classification - may have limited the scope and interest of DP research unnecessarily, and we point to new avenues of research for the field.

Prosopagnosia Due to Metastatic Brain Tumor: A Case-Based Review.

Prosopagnosia, also referred to as "face blindness," is a type of visual agnosia characterized by a decreased capacity to recognize familiar faces with a preserved ability to identify individuals based on non-facial visual traits or voice. Prosopagnosia can be categorized as developmental (DP) or acquired (AP) owing to a variety of underlying conditions, including trauma, neurodegenerative diseases, stroke, neuroinfections, and, less frequently, malignancies. Facial recognition is a complex process in which different neuronal networks are involved. The infrequent but notable higher visual-processing abnormalities can be caused by lesions of the inferior longitudinal fasciculus (ILF) in the non-dominant temporal lobe. We report a rare case of AP in a 69-year-old patient who is right-hand dominant with rectal carcinoma cerebral metastases. The patient complained of dizziness, vertigo, falls, and trouble recognizing her family members' faces. The CT scan of the head with contrast revealed two metastatic brain lesions with vasogenic edema, as one of them was in the right cerebellar hemisphere, causing dislocation and compression of the ILF. Corticosteroids and osmotherapy were utilized as a conservative treatment approach, which resulted in the prosopagnosia being completely withdrawn. In conclusion, patients with primary brain tumors or metastatic disease rarely present with an isolated cognitive deficit such as prosopagnosia. Based on the anatomical features and the personalized approach, a conservative or surgical approach may be useful to improve higher cortical functioning.

Human genetics of face recognition: discovery of MCTP2 mutations in humans with face blindness (congenital prosopagnosia).

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.