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.

Cognitive Functioning and Assessment in Adults with Cerebral Palsy: A Scoping Review.

In this scoping review, we summarize the current knowledge of cognitive functioning in adults with cerebral palsy (CP), and identify the neuropsychological tests typically used in this population. 39 studies from the period January 1990 - August 2023 were included in the review, and they differ widely in their aims and approach to studying cognition. Very few studies have cognitive assessment as their core aim and use a neuropsychological test battery. The included studies show great variability in reported intelligence and cognitive functioning in adults with CP, and cognitive deficits have been reported in all cognitive domains. Most of the studies suffer from methodological limitations, and there is ample room for improvement within the field. We conclude by suggesting a number of recommendations that may contribute to increasing our understanding of cognitive impairments in adults with CP.

Evidence for a Classical Dissociation between Face and Object Recognition in Developmental Prosopagnosia.

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.

Using representational similarity analysis to reveal category and process specificity in visual object recognition.

Cross-condition comparisons on neurodevelopmental conditions are central in neurodiversity research. In the realm of visual perception, the performance of participants with different category-specific disorders such as developmental prosopagnosia (problems with faces) and dyslexia (problems with words) have contributed to understanding of perceptual processes involved in word and face recognition. Alterations in face and word recognition are present in several neurodiverse populations, and improved knowledge about their relationship may increase our understanding of this variability of impairment. The present study investigates organizing principles of visual object processing and their implications for developmental disorders of recognition. Some accounts suggest that distinct mechanisms are responsible for recognizing objects of different categories, while others propose that categories share or even compete for cortical resources. We took an individual differences approach to estimate the relationship between abilities in recognition. Neurotypical participants (N = 97 after outlier exclusion) performed a match-to-sample task with faces, houses, and pseudowords. Either individual features or feature configurations were manipulated. To estimate the separability of visual recognition mechanisms, we used representational similarity analysis (RSA) where correlational matrices for accuracy were compared to predicted data patterns. Recognition abilities separated into face recognition on one hand and house/pseudoword recognition on the other, indicating that face recognition may rely on relatively selective mechanisms in neurotypicals. We also found evidence for a general visual object recognition mechanism, while some combinations of category (faces, houses, words) and processing type (featural, configural) likely rely on additional mechanisms. Developmental conditions may therefore reflect combinations of impaired and intact aspects of specific and general visual object recognition mechanisms, where featural and configural processes for one object category separate from the featural or configural processing of another. More generally, RSA is a promising approach for advancing understanding of neurodiversity, including shared aspects and distinctions between neurodevelopmental conditions of visual recognition.

Systematic evaluation of high-level visual deficits and lesions in posterior cerebral artery stroke.

Knowledge about the consequences of stroke on high-level vision comes primarily from single case studies of patients selected based on their behavioural profiles, typically patients with specific stroke syndromes like pure alexia or prosopagnosia. There are, however, no systematic, detailed, large-scale evaluations of the more typical clinical behavioural and lesion profiles of impairments in high-level vision after posterior cerebral artery stroke. We present behavioural and lesion data from the Back of the Brain project, to date the largest (N = 64) and most detailed examination of patients with cortical posterior cerebral artery strokes selected based on lesion location. The aim of the current study was to relate behavioural performance with faces, objects and written words to lesion data through two complementary analyses: (i) a multivariate multiple regression analysis to establish the relationships between lesion volume, lesion laterality and the presence of a bilateral lesion with performance and (ii) a voxel-based correlational methodology analysis to establish whether there are distinct or separate regions within the posterior cerebral artery territory that underpin the visual processing of words, faces and objects. Behaviourally, most patients showed more general deficits in high-level vision (n = 22) or no deficits at all (n = 21). Category-selective deficits were rare (n = 6) and were only found for words. Overall, total lesion volume was most strongly related to performance across all three domains. While behavioural impairments in all domains were observed following unilateral left and right as well as bilateral lesions, the regions most strongly related to performance mainly confirmed the pattern reported in more selective cases. For words, these included a left hemisphere cluster extending from the occipital pole along the fusiform and lingual gyri; for objects, bilateral clusters which overlapped with the word cluster in the left occipital lobe. Face performance mainly correlated with a right hemisphere cluster within the white matter, partly overlapping with the object cluster. While the findings provide partial support for the relative laterality of posterior brain regions supporting reading and face processing, the results also suggest that both hemispheres are involved in the visual processing of faces, words and objects.

The Danish Version of the 20-Item Prosopagnosia Index (PI20): Translation, Validation and a Link to Face Perception.

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.

Colour perception deficits after posterior stroke: Not so rare after all?

Cerebral achromatopsia is an acquired colour perception impairment caused by brain injury, and is generally considered to be rare. Both hemispheres are thought to contribute to colour perception, but most published cases have had bilateral or right hemisphere lesions. In contrast to congenital colour blindness that affects the discrimination between specific hues, cerebral achromatopsia is often described as affecting perception across all colours. Most studies of cerebral achromatopsia have been single cases or case series of patients with colour perception deficits. Here, we explore colour perception deficits in an unbiased sample of patients with stroke affecting the posterior cerebral artery (N = 63) from the Back of the Brain project. Patients were selected based on lesion location only, and not on the presence of a given symptom. All patients were tested with the Farnsworth D-15 Dichotomous Colour Blindness Test and performance compared to matched controls (N = 45) using single case statistics. In patients with abnormal performance, the patterns of colour difficulties were qualitatively analysed. 22% of the patients showed significant problems with colour discrimination (44% of patients with bilateral lesions, 28% with left hemisphere lesions and 5% with right hemisphere lesions). Lesion analyses identified two regions in ventral occipital temporal areas in the left hemisphere as particularly strongly related to impaired performance in colour perception, but also indicated that bilateral lesions are more strongly associated with impaired performance that unilateral lesions. While some patients only had mild deficits, colour perception impairments were in many cases severe. Many patients had selective deficits only affecting the perception of some hues. The results suggest that colour perception difficulties following PCA stroke are common, and that they vary in severity and expression. In addition, the results point towards bilateral processing of colour perception with a left hemispheric domination, contradicting previous reports.

The face inversion effect or the face upright effect?

The face inversion effect (FIE) refers to the observation that presenting stimuli upside-down impairs the processing of faces disproportionally more than other mono-oriented objects. This has been taken as evidence that processing of faces and objects differ qualitatively. However, nearly all FIE studies are based on comparing individuation of upright faces, which most people are rather good at, with individuation of objects most people are much less familiar with individuating (e.g., radios and airplanes). Consequently, the FIE may mainly reflect differences between categories in how they are processed prior to inversion, with within-category discrimination of upright faces being a much more familiar task than within-category discrimination among members belonging to other object classes. We tested this hypothesis by comparing inversion effects for faces and objects using object recognition tasks that do not require within-category discrimination (object decision and old/new recognition memory tasks). In all tasks (seven with objects and two with faces) we find credible inversion effects, but in no instance were these effects significantly larger for faces than for objects. This suggests that the FIE can be a product of familiarity with the type of identification process required in the upright conditions rather than some process that is selectively affected for faces when stimuli are inverted.

Lateralization of word and face processing in developmental dyslexia and developmental prosopagnosia.

In right-handed adults, face processing is lateralized to the right hemisphere and visual word processing to the left hemisphere. According to the many-to-many account (MTMA) of functional cerebral organization this lateralization pattern is partly dependent on the acquisition of literacy. Hence, the MTMA predicts that: (i) processing of both words and faces should show no or at least less lateralization in individuals with developmental dyslexia compared with controls, and (ii) lateralization in word processing should be normal in individuals with developmental prosopagnosia whereas lateralization in face processing should be absent. To test these hypotheses, 21 right-handed adults with developmental dyslexia and 21 right-handed adults with developmental prosopagnosia performed a divided visual field paradigm with delayed matching of faces, words and cars. Contrary to the predictions, we find that lateralization effects in face processing are within the normal range for both developmental dyslexics and prosopagnosics. Moreover, the group with developmental dyslexia showed right hemisphere lateralization for word processing. We argue that these findings are incompatible with the specific predictions of the MTMA.

Is It Just Face Blindness? Exploring Developmental Comorbidity in Individuals with Self-Reported Developmental Prosopagnosia.

Developmental prosopagnosia (DP)-or 'face blindness'-refers to life-long problems with facial recognition in the absence of brain injury. We know that neurodevelopmental disorders tend to co-occur, and this study aims to explore if individuals with self-reported DP also report indications of other neurodevelopmental disorders, deficits, or conditions (developmental comorbidity). In total, 115 individuals with self-reported DP participated in this online cross-sectional survey. Face recognition impairment was measured with a validated self-report instrument. Indications of difficulties with navigation, math, reading, or spelling were measured with a tailored questionnaire using items from published sources. Additional diagnoses were measured with direct questions. We also included open-ended questions about cognitive strengths and difficulties. Results: Overall, 57% reported at minimum one developmental comorbidity of interest, with most reflecting specific cognitive impairment (e.g., in memory or object recognition) rather than diagnostic categories (e.g., ADHD, dyslexia). Interestingly, many participants reported cognitive skills or strengths within the same domains that others reported impairment, indicating a diverse pattern of cognitive strengths and difficulties in this sample. The frequency and diversity of self-reported developmental comorbidity suggests that face recognition could be important to consider in future investigations of neurodevelopmental comorbidity patterns.

Contrasting domain-general and domain-specific accounts in cognitive neuropsychology: An outline of a new approach with developmental prosopagnosia as a case.

The backbone of cognitive neuropsychology is the observation of (double) dissociations in performance between patients, suggesting some degree of independence between cognitive processes (domain specificity). In comparison, observations of associations between disorders/deficits have been deemed less evidential in neuropsychological theorizing about cognitive architecture. The reason is that associations can reflect damage to independent cognitive processes that happen to be mediated by structures commonly affected by the same brain disorder rather than damage to a shared (domain-general) mechanism. Here we demonstrate that it is in principle possible to discriminate between these alternatives by means of a procedure involving large unbiased samples. We exemplify the procedure in the context of developmental prosopagnosia (DP), but the procedure is in principle applicable to all neuropsychological deficits/disorders. A simulation of the procedure on a dataset yields estimates of dissociations/associations that are well in line with existing DP-studies, and also suggests that seemingly selective disorders can reflect damage to both domain-general and domain-specific cognitive processes. However, the simulation also highlights some limitations that should be considered if the procedure is to be applied prospectively. The main advantage of the procedure is that allows for examination of both associations and dissociations in the same sample. Hence, it may help even the balance in the use of associations and dissociations as grounds for neuropsychological theorizing.

Whole-object effects in visual word processing: Parallels with and differences from face recognition.

Visual words and faces differ in their structural properties, but both are objects of high expertise. Holistic processing is said to characterize expert face recognition, but the extent to which whole-word processes contribute to word recognition is unclear, particularly as word recognition is thought to proceed by a component-based process. We review the evidence for experimental effects in word recognition that parallel those used to support holistic face processing, namely inversion effects, the part-whole task, and composite effects, as well as the status of whole-word processing in pure alexia and developmental dyslexia, contrasts between familiar and unfamiliar languages, and the differences between handwriting and typeset font. The observations support some parallels in whole-object influences between face and visual word recognition, but do not necessarily imply similar expert mechanisms. It remains to be determined whether and how the relative balance between part-based and whole-object processing differs for visual words and faces.

Reading and alexia.

Alexia refers to a reading disorder caused by some form of acquired brain pathology, most commonly a stroke or tumor, in a previously literate subject. In neuropsychology, a distinction is made between central alexia (commonly seen in aphasia) and peripheral alexia (a perceptual or attentional deficit). The prototypical peripheral alexia is alexia without agraphia (pure alexia), where patients can write but are impaired in reading words and letters. Pure alexia is associated with damage to the left ventral occipitotemporal cortex (vOT) or its connections. Hemianopic alexia is associated with less extensive occipital damage and is caused by a visual field defect, which creates problems reading longer words and passages of text. Reading impairment can also arise due to attentional deficits, most commonly following right hemisphere or bilateral lesions. Studying patients with alexia, along with functional imaging studies of normal readers, has improved our understanding of the neurobiological processes involved in reading. A key question is whether an area in the left ventral occipitotemporal cortex is specialized for or selectively involved in word processing, or whether reading relies on tuning of more general purpose perceptual areas. Reading deficits may also be observed in dementia and traumatic brain injury, but often with less consistent deficit patterns than in patients with focal lesions.

Category-selective deficits are the exception and not the rule: Evidence from a case-series of 64 patients with ventral occipito-temporal cortex damage.

The organisational principles of the visual ventral stream are still highly debated, particularly the relative association/dissociation between word and face recognition and the degree of lateralisation of the underlying processes. Reports of dissociations between word and face recognition stem from single case-studies of category selective impairments, and neuroimaging investigations of healthy participants. Despite the historical reliance on single case-studies, more recent group studies have highlighted a greater commonality between word and face recognition. Studying individual patients with rare selective deficits misses (a) important variability between patients, (b) systematic associations between task performance, and (c) patients with mild, severe and/or non-selective impairments; meaning that the full spectrum of deficits is unknown. The Back of the Brain project assessed the range and specificity of visual perceptual impairment in 64 patients with posterior cerebral artery stroke recruited based on lesion localization and not behavioural performance. Word, object, and face processing were measured with comparable tests across different levels of processing to investigate associations and dissociations across domains. We present two complementary analyses of the extensive behavioural battery: (1) a data-driven analysis of the whole patient group, and (2) a single-subject case-series analysis testing for deficits and dissociations in each individual patient. In both analyses, the general organisational principle was of associations between words, objects, and faces even following unilateral lesions. The majority of patients either showed deficits across all domains or in no domain, suggesting a spectrum of visuo-perceptual deficits post stroke. Dissociations were observed, but they were the exception and not the rule: Category-selective impairments were found in only a minority of patients, all of whom showed disproportionate deficits for words. Interestingly, such selective word impairments were found following both left and right hemisphere lesions. This large-scale investigation of posterior cerebral artery stroke patients highlights the bilateral representation of visual perceptual function.

Patterns of perceptual performance in developmental prosopagnosia: An in-depth case series.

Developmental prosopagnosia (DP) is a syndrome characterized by lifelong impairment in face recognition in the absence of brain damage. A key question regarding DP concerns which process(es) might be affected to selectively/disproportionally impair face recognition. We present evidence from a group of DPs, combining an overview of previous results with additional analyses important for understanding their pattern of preserved and impaired perceptual abilities. We argue that for most of these individuals, the common denominator is a deficit in (rapid) processing of global shape information. We conclude that the deficit in this group of DPs is not face-selective, but that it may appear so because faces are more visually similar-and recognized at a more fine-grained level-than objects. Indeed, when the demand on perceptual differentiation and visual similarity are held constant for faces and objects, we find no evidence for a disproportionate deficit for faces in this group of DPs.

Face recognition in developmental dyslexia: evidence for dissociation between faces and words.

Developmental dyslexia is primarily a reading disorder, but recent studies have indicated that face processing problems may also be present. Using a case-series approach, we tested face recognition and visual word recognition in 24 high school students diagnosed with developmental dyslexia. Contrary to previous findings, no face recognition problems were found on the group-level. Rather, a significant classical dissociation with impaired word reading and normal face recognition was demonstrated on a group-level and for six individuals with developmental dyslexia. However, four individuals with dyslexia did show face recognition problems. Thus, while problems in face recognition can be present in developmental dyslexia, the dissociation strongly suggests that face recognition can also be preserved. Combined with previously reported dissociations between face and word recognition in developmental prosopagnosia, this constitutes a double dissociation.