Cases reported "Prosopagnosia"

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1/8. Severe developmental prosopagnosia in a child with superior intellect.

    Developmental prosopagnosia, a lifelong inability to learn and recognize familiar faces, has rarely been reported, and there are even fewer cases that have been studied during childhood. Of the cases studied during childhood, significant "apperceptive" features to the face recognition defect have been noted. We had an opportunity to conduct extensive standard and experimental neuropsychological, psychophysiological, and neuroanatomical studies in a five-year-old child with severe developmental prosopagnosia. The subject was intellectually gifted (FSIQ = 130), but had a marked discrepancy between verbal and nonverbal abilities (VIQ = 140, PIQ = 110). Although some visual perceptual weaknesses were apparent, the subject's face recognition defect was found to cnform most closely to the "associative" type, and he did not have visual recognition deficits for any categories of nonunique entities. A novel finding was that the child's covert recognition of familiar faces based on an autonomic index was normal, suggesting that as in some adult-onset cases, the brain is capable of acquiring some information about familiar faces, even without conscious recognition. The child also had normal judgments of facial emotional expressions. Our report extends the understanding of the neuropsychological features of developmental prosopagnosia, and may help narrow the search for neuroanatomical correlates of this condition, which have yet to be identified.
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2/8. Less impairment in face imagery than face perception in early prosopagnosia.

    There have been a number of reports of preserved face imagery in prosopagnosia. We put this issue to experimental test by comparing the performance of MJH, a 34-year-old prosopagnosic since the age of 5, to controls on tasks where the participants had to judge faces of current celebrities, either in terms of overall similarity (Of Bette Midler, Hillary Clinton, and Diane Sawyer, whose face looks least like the other two?) or on individual features (Is Ronald Reagan's nose pointy?). For each task, a performance measure reflecting the degree of agreement of each participant with the average of the others (not including MJH) was calculated. On the imagery versions of these tasks, MJH was within the lower range of the controls for the agreement measure (though significantly below the mean of the controls). When the same tasks were performed from pictures, agreement among the controls markedly increased whereas MJH's performance was virtually unaffected, placing him well below the range of the controls. This pattern was also apparent with a test of facial features of emotion (Are the eyes wrinkled when someone is surprised?). On three non-face imagery tasks assessing color (What color is a football?), relative lengths of animal's tails (Is a bear's tail long in proportion to its body?), and mental size comparisons (What is bigger, a camel or a zebra?), MJH was within or close to the lower end of the normal range. As most of the celebrities became famous after the onset of MJH's prosopagnosia, our confirmation of the reports of less impaired face imagery in some prosopagnosics cannot be attributed to pre-lesion storage. We speculate that face recognition, in contrast to object recognition, relies more heavily on a representation that describes the initial spatial filter values so the metrics of the facial surface can be specified. If prosopagnosia is regarded as a form of simultanagnosia in which some of these filter values cannot be registered on any one encounter with a face, then multiple opportunities for repeated storage may partially compensate for the degraded representation on that single encounter. Imagery may allow access to this more complete representation.
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3/8. Covert matching of unfamiliar faces in a case of prosopagnosia: an ERP study.

    In addition to their deficit in overt face recognition, patients with prosopagnosia also have difficulties in matching sequentially presented unfamiliar faces. Here we assessed the possibility that covert matching of faces was present in a case with prosopagnosia using event-related potentials (ERPs). The participants (patient FE and normal controls) were challenged with a face-identity matching task, in which they decided whether two sequentially presented photographs of unfamiliar faces represented the same person. Only internal face features were used and the two faces in a pair differed in emotional expression. FE failed to overtly match these stimuli. In contrast, the ERPs revealed evidence of covert matching. If the two faces within a pair of stimuli depicted different posers, then the response to the second face contained an enhanced N300 compared to the situation where the identity of the faces was the same. The latency of the N300 was the same as a similar component found in controls. These results suggest that some cases with prosopagnosia have a covert ability to match unfamiliar faces, with similar temporal dynamics as controls, which in contrast with the idea that a generalized slowing of face processing occurs in all cases of prosopagnosia.
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4/8. Normal recognition of emotion in a prosopagnosic.

    In the leading model of face perception, facial identity and facial expressions of emotion are recognized by separate mechanisms. In this report, we provide evidence supporting the independence of these processes by documenting an individual with severely impaired recognition of facial identity yet normal recognition of facial expressions of emotion. NM, a 40-year-old prosopagnosic, showed severely impaired performance on five of six tests of facial identity recognition. In contrast, she performed in the normal range on four different tests of emotion recognition. Because the tests of identity recognition and emotion recognition assessed her abilities in a variety of ways, these results provide solid support for models in which identity recognition and emotion recognition are performed by separate processes.
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5/8. Exploring the role of motion in prosopagnosia: recognizing, learning and matching faces.

    HJA has been completely unable to recognize faces since suffering a stroke some 22 years ago. Previous research has shown that he is poor at judging expressions from static photographs of faces, but performs relatively normally at these judgements when presented with moving point-light patterns (Humphreys et al., 1993). Recent research with non-prosopagnosic participants has suggested a beneficial role for facial motion when recognizing familiar faces and learning new faces. Three experiments are reported that investigate the role of face motion for HJA when recognizing (Experiment 1), learning (Experiment 2) and matching faces (Experiment 3). The results indicate that HJA is unable to use face motion to explicitly recognize faces and is no better at learning names for moving faces than static ones. However, HJA is significantly better at matching moving faces for identity, an opposite pattern to that found with age-matched and undergraduate control participants. We suggest that HJA is not impaired at processing motion information but remains unable to use motion as a cue to identity.
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6/8. The fusiform face area is not sufficient for face recognition: evidence from a patient with dense prosopagnosia and no occipital face area.

    We tested functional activation for faces in patient D.F., who following acquired brain damage has a profound deficit in object recognition based on form (visual form agnosia) and also prosopagnosia that is undocumented to date. Functional imaging demonstrated that like our control observers, D.F. shows significantly more activation when passively viewing face compared to scene images in an area that is consistent with the fusiform face area (FFA) (p < 0.01). Control observers also show occipital face area (OFA) activation; however, whereas D.F.'s lesions appear to overlap the OFA bilaterally. We asked, given that D.F. shows FFA activation for faces, to what extent is she able to recognize faces? D.F. demonstrated a severe impairment in higher level face processing--she could not recognize face identity, gender or emotional expression. In contrast, she performed relatively normally on many face categorization tasks. D.F. can differentiate faces from non-faces given sufficient texture information and processing time, and she can do this is independent of color and illumination information. D.F. can use configural information for categorizing faces when they are presented in an upright but not a sideways orientation and given that she also cannot discriminate half-faces she may rely on a spatially symmetric feature arrangement. Faces appear to be a unique category, which she can classify even when she has no advance knowledge that she will be shown face images. Together, these imaging and behavioral data support the importance of the integrity of a complex network of regions for face identification, including more than just the FFA--in particular the OFA, a region believed to be associated with low-level processing.
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7/8. 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.
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8/8. Compensatory strategies in processing facial emotions: evidence from prosopagnosia.

    We report data on the processing of facial emotion in a prosopagnosic patient (H.J.A.). H.J.A. was relatively accurate at discriminating happy from angry upright faces, but he performed at chance when the faces were inverted. Furthermore, with upright faces there was no configural interference effect on emotion judgements, when face parts expressing different emotions were aligned to express a new emergent emotion. We propose that H.J.A.'s emotion judgements relied on local rather than on configural information, and this local information was disrupted by inversion. A compensatory strategy, based on processing local face parts, can be sufficient to process at least some facial emotions.
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