Developmental prosopagnosia: a study of three patients

We studied perception in three patients with prosopagnosia of childhood onset. All had trouble with other 'within-category' judgments. All were deficient on face matching tests and severely impaired on tests of perception of the spatial relations of facial features and abstract designs, indicating a deficit in the encoding of coordinate relationships, similar to adult-onset prosopagnosia with lesions of the fusiform face area. Two had difficulty perceiving feature colour, which correlated with reduced luminance sensitivity. In contrast to adult-onset patients, saturation discrimination was spared in two and spatial resolution impaired in two. Curvature discrimination was relatively spared. Contrast sensitivity showed variable reductions at different spatial frequencies. We conclude that developmental prosopagnosia is similar to the adult-onset form in encoding deficits for the spatial arrangement of facial elements. Deficits in luminance perception and spatial resolution are more associated with defective encoding for basic object-level recognition, as shown on tests of object and spatial perception.     

Prosopagnosia: a defect in visual configural processing

A patient with severe, lasting prosopagnosia could not get an immediate overview of a face sufficiently specific for successful identification. He also failed completely in tasks of visual closure despite adequate performances on numerous other tests of visual perception and memory. We conclude that prosopagnosia represents a loss of visual "configural processing"--a learned skill enabling immediate identification of individual members of a class without conscious visuospatial analysis or remembering. Prosopagnosia and agnosic alexia represent two distinct defects of configural processing: Alexics cannot identify items with distinctive features that are themselves identifiable. Prosopagnosics cannot identify objects whose critical distinguishing features have no independent identities.     

Developmental prosopagnosia: a review

This article reviews the published literature on developmental prosopagnosia, a condition in which the ability to recognize other persons by facial information alone has never been acquired. Due to the very low incidence of this syndrome, case reports are sparse. We review the available data and suggest assessment strategies for patients suffering from developmental prosopagnosia. It is suggested that developmental prosopagnosia is not a unitary condition but rather consists of different subforms that can be dissociated on the grounds of functional impairments. On the basis of the available evidence, hypotheses about the aetiology of developmental prosopagnosia as well as about the selectivity of deficits related to face recognition are discussed.     

Perceptual functions in prosopagnosia

Some patients with prosopagnosia may have an apperceptive basis to their recognition defect. Perceptual abnormalities have been reported in single cases or small series, but the causal link of such deficits to prosopagnosia is unclear. Our goal was to identify candidate perceptual processes that might contribute to prosopagnosia, by subjecting several prosopagnosic patients to a battery of functions that may be necessary for accurate facial perception. We tested seven prosopagnosic patients. Three had unilateral right occipitotemporal lesions, two had bilateral posterior occipitotemporal lesions, and one had right anterior-to-occipital temporal damage along with a small left temporal lesion. These lesions all included the fusiform face area, in contrast to one patient with bilateral anterior temporal lesions. Most patients had impaired performance on face-matching tests and difficulty with subcategory judgments for non-face objects. The most consistent deficits in patients with lesions involving the fusiform face area were impaired perception of spatial relations in dot patterns and reduced contrast sensitivity in the 4 to 8 cycles deg(-1) range. Patients with bilateral lesions were impaired in saturation discrimination. Luminance discrimination was normal in all but two patients, and spatial resolution was uniformly spared. Curvature and line-orientation discrimination were impaired in only one patient, who also had the most difficulty with more basic-level object recognition. We conclude that deficits in luminance, spatial resolution, curvature, line orientation, and contrast at low spatial frequencies are unlikely to contribute to apperceptive prosopagnosia. More relevant may be contrast sensitivity at higher spatial frequencies and the analysis of object spatial structure. Deficits in these functions may impair perception of subtle variations in object shape, and may be one mechanism by which the recognition defect in prosopagnosia can extend to other classes of object subcategorization.     

Is the Right Anterior Temporal Variant of Prosopagnosia a Form of ‘Associative Prosopagnosia’ or a Form of ‘Multimodal Person Recognition Disorder’?

The construct of associative prosopagnosia is strongly debated for two main reasons. The first is that, according to some authors, even patients with putative forms of associative visual agnosia necessarily present perceptual defects, that are the cause of their recognition impairment. The second is that in patients with right anterior temporal lobe (ATL) lesions (and sparing of the occipital and fusiform face areas), who can present a defect of familiar people recognition, with normal results on tests of face perception, the disorder is often multimodal, affecting voices (and to a lesser extent names) in addition to faces. The present review was prompted by the claim, recently advanced by some authors, that face recognition disorders observed in patients with right ATL lesions should be considered as an associative or amnestic form of prosopagnosia, because in them both face perception and retrieval of personal semantic knowledge from name are spared. In order to check this claim, we surveyed all the cases of patients who satisfied the criteria of associative prosopagnosia reported in the literature, to see if their defect was circumscribed to the visual modality or also affected other channels of people recognition. The review showed that in most patients the study had been limited to the visual modality, but that, when the other modalities of people recognition had been taken into account, the defect was often multimodal, affecting voice (and to a lesser extent name) in addition to face.     

Prosopagnosia: A reclassification

We report a quantitative investigation of three patients who presented with a profound prosopagnosia. They were tested on a series of tests of face perception, face recognition, and paired associate learning using faces. A patient with marked perceptual difficulties but no difficulty whatever in recognizing faces was tested as a control. The control patient's performance was as bad or worse on the tests of perception of faces, and it is concluded that impaired performance on tests of face perception does not contribute to difficulties in recognizing familiar faces. The major difference between the three prosopagnosic patients was the evidence of covert recognition in two of these cases but not in the third. We use this evidence to argue that the perceptual/mnestic distinction is an inappropriate classification and would suggest instead that the two types may be described in terms either of a disconnection of the face recognition units or of damage to the units themselves.     

Impaired holistic and analytic face processing in congenital prosopagnosia: Evidence from the eye-contingent mask/window paradigm

There is abundant evidence that face recognition, in comparison to the recognition of other objects, is based on holistic processing rather than analytic processing. One line of research that provides evidence for this hypothesis is based on the study of people who experience pronounced difficulties in visually identifying conspecifics on the basis of their face. Earlier, we developed a behavioural paradigm to directly test analytic vs. holistic face processing. In comparison to a to be remembered reference face stimulus, one of two test stimuli was either presented in full view, with an eye-contingently moving window (only showing the fixated face feature, and therefore only affording analytic processing), or with an eye-contingently moving mask or scotoma (masking the fixated face feature, but still allowing holistic processing). In the present study we use this paradigm (that we used earlier in acquired prosopagnosia) to study face perception in congenital prosopagnosia (people having difficulties recognizing faces from birth on, without demonstrable brain damage). We observe both holistic and analytic face processing deficits in people with congenital prosopagnosia. Implications for a better understanding, both of congenital prosopagnosia and of normal face perception, are discussed.     

发展性面孔失认的认知机制及其神经基础

发展性面孔失认症是指个体在童年期就开始表现出来的一种终生性面孔识别缺陷,其不能归因于智力衰退、情感障碍、物体识别困难以及后天性脑损伤.发展性面孔失认症涉及的认知机制包括面孔特异性机制、构型加工障碍、面孔探测、面孔记忆和面孔身份识别.此外,该面孔失认症的神经网络由核心神经网络和延伸的神经网络组成,前者与面孔选择反应和记忆表征有关,后者主要负责面孔知识表征、面孔长时记忆和面孔工作记忆.今后的研究应集中在完善延伸的神经网络、拓展其认知网络;进一步明确面孔探测与发展性面孔失认之间的关系;考察发展性面孔失认症的基因基础、加强其发展性研究以及推动康复工作的展开.     

Preservation of mouth region processing in two cases of prosopagnosia

Although most adults are considered experts in face recognition, brain trauma can produce a selective loss in this ability, a condition referred to as prosopagnosia. This study examined the processing strategies of prosopagnosic patients LR and HH using the Face Dimensions Test. In this test, featural and configural information in the upper and lower halves of the face was parametrically varied and sensitivity to these changes measured. We found that relative to age‐matched control participants, LR and HH exhibited an impaired ability to discriminate differences in the eye region, but a preserved ability to detect featural and configural differences in the mouth region. This pattern of impairment and sparing was demonstrated in tests of direct perception and immediate memory. The obtained findings demonstrate that prosopagnosia does not necessarily cause a global impairment to face perception, but a selective impairment to the perception of information in the upper half of the face.     

Damasio's error – Prosopagnosia with intact within‐category object recognition

The sudden inability to recognize individual faces following brain damage was first reported in a scientific journal 150 years ago and termed ‘prosopagnosia’ 70 years ago. While the term originally identified a face‐selective neurological condition, it is now obscured by a sequence of imprecisions. First, prosopagnosia is routinely used to define symptoms of individual face recognition (IFR) difficulties in the context of visual object agnosia or other neurological conditions, or even in the normal population. Second, this over‐expansive definition has lent support to a long‐standing within‐category recognition account of prosopagnosia, that is, that the impairment of IFR reflects a general impairment in recognizing within‐category objects. However, stringent experimental studies of classical cases of prosopagnosia following brain damage show that their core impairment is not in recognizing physically similar exemplars within non‐face object categories. Instead, the impairment presents specifically for recognizing exemplars of the category of faces. Moreover, compared to typical observers, the impairment appears even more severe for recognizing individual faces against physically dissimilar than similar distractors. Here, I argue that we need to limit accordingly our definition of prosopagnosia to a clinical (i.e., neurological) condition in which there is no basic‐level object recognition impairment. Other criteria for prosopagnosia are proposed, with the hope that this conservative definition enables the study of human IFR processes in isolation, and supports progress in understanding the nature of these processes.     

What aspects of face processing are impaired in developmental prosopagnosia?

Developmental prosopagnosia (DP) is a severe impairment in identifying faces that is present from early in life and that occurs despite no apparent brain damage and intact visual and intellectual function. Here, we investigated what aspects of face processing are impaired/spared in developmental prosopagnosia by examining a relatively large group of individuals with DP (n = 8) using an extensive battery of well-established tasks. The tasks included measures of sensitivity to global motion and to global form, detection that a stimulus is a face, determination of its sex, holistic face processing, processing of face identity based on features, contour, and the spacing of features, and judgments of attractiveness. The DP cases showed normal sensitivity to global motion and global form and performed normally on our tests of face detection and holistic processing. On the other tasks, many DP cases were impaired but there was no systematic pattern. At least half showed deficits in processing of facial identity based on either the outer contour or spacing of the internal features, and/or on judgments of attractiveness. Three of the eight were impaired in processing facial identify based on the shape of internal features. The results show that DP is a heterogeneous condition and that impairment in recognizing faces cannot be predicted by poor performance on any one measure of face processing.     

Affective reactions to facial identity in a prosopagnosic patient

This study probes whether a prosopagnosic patient can make accurate explicit affective judgements towards faces. Patient MJH was shown photographs of faces of well-liked family members and public figures rated as “evil” by opinion polls. MJH was asked to rate each face on two 7-point scales (Likeability and Pleasantness). Since he is unable to explicitly recognise faces, his ratings were based on his evaluative reaction to the faces presented. In a second phase of the experiment, MJH was told the name of the faces previously presented and asked to rate them using the same scales. MJH's Likeability ratings during the picture-viewing phase of the experiment and the explicit phase were highly correlated. Based on these findings, we propose that thought consists of an explicit declarative and an implicit emotional aspect, which may become dissociated in prosopagnosia.     

Neural and genetic foundations of face recognition and prosopagnosia

Faces are of essential importance for human social life. They provide valuable information about the identity, expression, gaze, health, and age of a person. Recent face‐processing models assume highly interconnected neural structures between different temporal, occipital, and frontal brain areas with several feedback loops. A selective deficit in the visual learning and recognition of faces is known as prosopagnosia, which can be found both in acquired and congenital form. Recently, a hereditary sub‐type of congenital prosopagnosia with a very high prevalence rate of 2.5% has been identified. Recent research results show that hereditary prosopagnosia is a clearly circumscribed face‐processing deficit with a characteristic set of clinical symptoms. Comparing face processing of people of prosopagnosia with that of controls can help to develop a more conclusive and integrated model of face processing. Here, we provide a summary of the current state of face processing research. We also describe the different types of prosopagnosia and present the set of typical symptoms found in the hereditary type. Finally, we will discuss the implications for future face recognition research.     

Gaze behaviour in hereditary prosopagnosia

Prosopagnosia is the inability to recognize someone by the face alone in the absence of sensory or intellectual impairment. In contrast to the acquired form of prosopagnosia we studied the congenital form. Since we could recently show that this form is inherited as a simple monogenic trait we called it hereditary form. To determine whether not only face recognition and neuronal processing but also the perceptual acquisition of facial information is specific to prosopagnosia, we studied the gaze behaviour of four hereditary prosopagnosics in comparison to matched control subjects. This rarely studied form of prosopagnosia ensures that deficits are limited to face recognition. Whereas the control participants focused their gaze on the central facial features, the hereditary prosopagnosics showed a significantly different gaze behaviour. They had a more dispersed gaze and also fixated external facial features. Thus, the face recognition impairment of the hereditary prosopagnosics is reflected in their gaze behaviour.     

Visual agnosia and prosopagnosia in childhood: a prospective case study.

Selective impairments in visual processing are well documented in adults but rarely reported in children. The few childhood cases reported are mostly retrospective accounts with little attention paid to developmental, assessment or management issues. We report a prospective case study of a boy with prosopagnosia and visual processing deficits of presumed developmental origin. At the age of 4 years, AL presented with a range of cognitive and visual recognition deficits. Subsequent assessments revealed an evolving pattern in visual recognition and dissociations between developing skills. At the age of 7 AL has impairments in early perceptual analysis, visual organisation and in complex visual processing. Although he can identify facial features and match faces he is unable to recognise familiar faces. His reading and spelling are developing normally. The nature of his deficits and his progress are discussed within a cognitive neuropsychological framework.     

Covert face recognition in prosopagnosia: A review

Prosopagnosia, a spectacular face agnosia, is generally detected and discussed on the basis of explicit or overt performance of the subjects. However, recent observations suggest that an overt response is probably not sufficient to determine what is and what is not preserved in the cognitive processing of faces by prosopagnosia. Indeed, signs of covert knowledge have been evidenced in some, but not all, subjects, indicating that they are still able to use representations they cannot use overtly. Empirical data, showing that the nature of prosopagnosia is a more complex phenomenon than has been thought and that the processing models should be adjusted accordingly, are reviewed.     

Structure and function in acquired prosopagnosia: Lessons from a series of 10 patients with brain damage

Acquired prosopagnosia varies in both behavioural manifestations and the location and extent of underlying lesions. We studied 10 patients with adult‐onset lesions on a battery of face‐processing tests. Using signal detection methods, we found that discriminative power for the familiarity of famous faces was most reduced by bilateral occipitotemporal lesions that involved the fusiform gyri, and better preserved with unilateral right‐sided lesions. Tests of perception of facial structural configuration showed severe deficits with lesions that included the right fusiform gyrus, whether unilateral or bilateral. This deficit was most consistent for eye configuration, with some patients performing normally for mouth configuration. Patients with anterior temporal lesions had better configuration perception, though at least one patient showed a more subtle failure to integrate configural data from different facial regions. Facial imagery, an index of facial memories, was severely impaired by bilateral lesions that included the right anterior temporal lobe and marginally impaired by fusiform lesions alone; unilateral right fusiform lesions tended to spare imagery for facial features. These findings suggest that (1) prosopagnosia is more severe with bilateral than unilateral lesions, indicating a minor contribution of the left hemisphere to face recognition, (2) perception of facial configuration critically involves the right fusiform gyrus and (3) access to facial memories is most disrupted by bilateral lesions that also include the right anterior temporal lobe. This supports assertions that more apperceptive variants of prosopagnosia are linked to fusiform damage, whereas more associative variants are linked to anterior temporal damage. Next, we found that behavioural indices of covert recognition correlated with measures of overt familiarity, consistent with theories that covert behaviour emerges from the output of damaged neural networks, rather than alternative pathways. Finally, to probe the face specificity of the prosopagnosic defect, we tested recognition of fruits and vegetables: While face specificity was not found in most of our patients, the data of one patient suggested that this may be possible with more focal lesions of the right fusiform gyrus.     

Atypically heterogeneous vertical first fixations to faces in a case series of people with developmental prosopagnosia

ABSTRACT

When people recognize faces, they normally move their eyes so that their first fixation is in the optimal location for efficient perceptual processing. This location is found just below the centre-point between the eyes. This type of attentional bias could be partly innate, but also an inevitable developmental process that aids our ability to recognize faces. We investigated whether a group of people with developmental prosopagnosia would also demonstrate neurotypical first fixation locations when recognizing faces during an eye-tracking task. We found evidence that adults with prosopagnosia had atypically heterogeneous first fixations in comparison to controls. However, differences were limited to the vertical, but not horizontal, plane of the face. We interpret these findings by suggesting that subtle changes to face-based eye movement patterns in developmental prosopagnosia may underpin their face recognition impairments, and suggest future work is still needed to address this possibility.     

Face to face with an enactive approach: A sensorimotor account of face detection and recognition

The enactive approach to perception describes experience as a temporally extended activity of skillful engagement with the environment. This paper pursues this view and focuses on prosopagnosia both for the light that the theory can throw on the phenomenon, and for the critical light the phenomenon can throw on the theory. I argue that the enactive theory is insufficient to characterize the unique nature of experience specific to prosopagnosic subjects. There is a distinct difference in the overall process of detection (with respect to eye movement sequence) of familiar and unfamiliar faces in prosopagnosia; in contrast, normal subjects use the same scanning strategy when exploring both kinds of faces despite an obvious difference in qualitative character. In light of this limitation I outline a supplemental view basing sensorimotor contingencies upon the establishment and reaffirmation of regularities within the organism as it engages with the environment.