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Congenital Visual Agnosia and Prosopagnosia in a Child: A Case Report
Abstract
We present an eight years old child, L.G. with congenital agnosia and prosopagnosia. This is a special case of perceptual deficits in a child which are discrete and exist in the context of a very high verbal intelligence. L.G. was administered an extensive battery of tests of cognitive functioning. He has intact basic visual skills, although his visual analysis is sometimes slow. L.G. can read, write and do math at age level or above. Four normal eight years old boys were used as controls on a selection of the perceptual tests, administered to L.G., which did not have normative data. L.G.'s object recognition skills bear the hallmarks of adult apperceptive agnosia. His visual memory and imagery are normal. Tests of face processing skills reveal, unlike adult prosopagnosics, severe deficits in addition to the familiar face recognition problem. L.G.'s agnosia and prosopagnosia are compared to the relevant literature.
... Unlike the prosopagnosia cases reported before [2,3], McConachie's patient showed no signs of brain damage that could explain the condition. It took 20 years until a second case of DP was reported [4], and hence the condition was considered to be very rare. However, with increased awareness of prosopagnosia in public, more people reporting symptoms of "face blindness" have come forward [5]. ...
... Object recognition and ventral stream functions DP is a condition of varying specificity and particularly the early cases were impaired in more general object recognition as well [1,4]. Later, very "pure" cases, restricted to deficits in recognizing only the identity of faces were reported [23,26,27]. ...
... While remembering faces seen only on one occasion is a difficult task per se, people with DP are also unable to recognize individuals they have known for years [4,23,24,40]. Similarly, J reported several occasions on which she passed her husband on the street without recognizing him. ...
Background
Not being able to recognize a person’s face is a highly debilitating condition from which people with developmental prosopagnosia (DP) suffer their entire life. Here we describe the case of J, a 30 year old woman who reports being unable to recognize her parents, her husband, or herself in the mirror.
Case presentation
We set out to assess the severity of J’s prosopagnosia using tests with unfamiliar as well as familiar faces and investigated whether impaired configural processing explains her deficit. To assess the specificity of the impairment, we tested J’s performance when evaluating emotions, intentions, and the attractiveness and likability of faces. Detailed testing revealed typical brain activity patterns for faces and normal object recognition skills, and no evidence of any brain injury. However, compared to a group of matched controls, J showed severe deficits in learning new faces, and in recognizing familiar faces when only inner features were available. Her recognition of uncropped faces with blurred features was within the normal range, indicating preserved configural processing when peripheral features are available. J was also unimpaired when evaluating intentions and emotions in faces. In line with healthy controls, J rated more average faces as more attractive. However, she was the only one to rate them as less likable, indicating a preference for more distinctive and easier to recognize faces.
Conclusions
Taken together, the results illustrate both the severity and the specificity of DP in a single case. While DP is a heterogeneous disorder, an inability to integrate the inner features of the face into a whole might be the best explanation for the difficulties many individuals with prosopagnosia experience.
... While these predictions are straightforward for healthy participants, in Experiment 2 we aimed to examine the dynamic gain effect in LG, an individual with a rare form of developmental visual agnosia. LG is prosopagnosic, he has deficient facial expression recognition (Ariel and Sadeh, 1996;Gilaie-Dotan, 2016a), and he presents a specific impairment with visual integration (Aviezer et al., 2012a). On the one hand, ...
... After demonstrating the importance of temporal information for subtle, non-prototypical emotion recognition in healthy individuals, we turned to investigate the dynamic gain effect in LG, an individual with developmental visual agnosia and prosopagnosia (Ariel and Sadeh, 1996). Developmental visual agnosia is a rare syndrome, consisting of unique symptoms of profound visual deficits, which typically include form agnosia, deficient perceptual integration skills and impaired generalized processing of faces, including gender, emotion, and identity information (Ariel and Sadeh, 1996;Duchaine et al., 2003). ...
... After demonstrating the importance of temporal information for subtle, non-prototypical emotion recognition in healthy individuals, we turned to investigate the dynamic gain effect in LG, an individual with developmental visual agnosia and prosopagnosia (Ariel and Sadeh, 1996). Developmental visual agnosia is a rare syndrome, consisting of unique symptoms of profound visual deficits, which typically include form agnosia, deficient perceptual integration skills and impaired generalized processing of faces, including gender, emotion, and identity information (Ariel and Sadeh, 1996;Duchaine et al., 2003). Prosopagnosia is a condition of face processing impairments that is defined by difficulties in recognizing facial identity. ...
Facial expressions are inherently dynamic cues that develop and change over time, unfolding their affective signal. Although facial dynamics are assumed important for emotion recognition, testing often involves intense and stereotypical expressions and little is known about the role of temporal information in the recognition of subtle, non-stereotypical expressions. In Experiment 1 we demonstrate that facial dynamics are critical for recognizing subtle and non-stereotypical facial expressions, but not for recognizing intense and stereotypical displays of emotion. In Experiment 2 we further examined whether the facilitative effect of motion can lead to improved emotion recognition in LG, an individual with developmental visual agnosia and prosopagnosia, who has poor emotion recognition when tested with static facial expressions. LG's emotion recognition improved when subtle, non-stereotypical faces were dynamic rather than static. However, compared to controls, his relative gain from temporal information was diminished. Furthermore, LG's eye-tracking data demonstrated atypical visual scanning of the dynamic faces, consisting of longer fixations and lower fixation rates for the dynamic-subtle facial expressions, comparing to the dynamic-intense facial expressions. We suggest that deciphering subtle dynamic expressions strongly relies on integrating broad facial regions across time, rather than focusing on local emotional cues, skills which are impaired in developmental visual agnosia.
... absence of brain damage and in the presence of preserved sensory and intellectual functions (Ariel & Sadeh, 1996;McConachie, 1976;Schmalzl, Palermo, Green, Brunsdon, & Coltheart, 2008). In accordance with previous evidence suggesting a genetic contribution to the impairment (Grüter, Grüter, & Carbon, 2008;Kennerknecht et al., 2006), recent preliminary findings indicate that CP could be associated with the DNA polymorphism of the receptor gene of oxytocin (a hormone that regulates basic social and reproductive behaviors; Cattaneo et al., 2016). ...
... The IE is the difference between the total score of the upright and inverted faces (i.e., the "cost" for recognizing inverted faces) and was included as a further criterion of face recognition impairment because it represents a qualitative index of face processing, which is often not present or inverted in participants with prosopagnosia (e.g., Behrmann & Avidan, 2005). The BFRT (Benton, 1994;Benton & Van Allen, 1968) was included as part of the neuropsychological battery in order to investigate the perceptual aspects of face recognition in our participants, as some studies have suggested that some, but not all, individuals with CP can experience face discrimination difficulties in addition to face memory impairment (Ariel & Sadeh, 1996;de Gelder & Rouw, 2000). The FFRT was administered in order to assess participants' ability to identify famous people from their faces (see Malaspina et al., 2017 for more details). ...
... Furthermore, all congenital prosopagnosics showed a smaller IE and, particularly, four of them had an IE score two standard deviations lower than controls. In the BFRT, only two of seven congenital prosopagnosics performed pathologically, consistently with other studies proving that some individuals with CP can experience difficulty with face discrimination in addition to face memory (Ariel & Sadeh, 1996;de Gelder & Rouw, 2000). Moreover, previous studies have also highlighted that individuals with prosopagnosia may achieve normal score on the BFRT, thanks to the availability of external cues in this test (Duchaine & Nakayama, 2004). ...
Objective:
Recent evidence showed that individuals with congenital face processing impairment (congenital prosopagnosia [CP]) are highly accurate when they have to recognize their own face (self-face advantage) in an implicit matching task, with a preference for the right-half of the self-face (right perceptual bias). Yet the perceptual strategies underlying this advantage are unclear. Here, we aimed to verify whether both the self-face advantage and the right perceptual bias emerge in an explicit task, and whether those effects are linked to a different scanning strategy between the self-face and unfamiliar faces.
Method:
Eye movements were recorded from 7 CPs and 13 controls, during a self/other discrimination task of stimuli depicting the self-face and another unfamiliar face, presented upright and inverted.
Results:
Individuals with CP and controls differed significantly in how they explored faces. In particular, compared with controls, CPs used a distinct eye movement sampling strategy for processing inverted faces, by deploying significantly more fixations toward the nose and mouth areas, which resulted in more efficient recognition. Moreover, the results confirmed the presence of a self-face advantage in both groups, but the eye movement analyses failed to reveal any differences in the exploration of the self-face compared with the unfamiliar face. Finally, no bias toward the right-half of the self-face was found.
Conclusions:
Our data suggest that the self-face advantage emerges both in implicit and explicit recognition tasks in CPs as much as in good recognizers, and it is not linked to any specific visual exploration strategies. (PsycINFO Database Record
... Perhaps unsurprisingly, more general tests of face perception, such as the Cambridge Face Perception Test (Avidan et al., 2011;Chatterjee & Nakayama, 2012;Dingle, Duchaine, & Nakayama, 2005;Duchaine, Germine, & Nakayama, 2007), face matching paradigms (Ariel & Sadeh, 1996;Behrmann et al., 2005;Humphreys, Avidan, & Behrmann, 2007;Lee et al., 2009;Nunn et al., 2001) and matching across viewpoint (Behrmann et al., 2005;Duchaine, 2000;Lee et al., 2009;Schmalzl et al., 2008), that are not designed to isolate a particular type of processing (such as the recovery of first-or secondorder relations), also unveil impairment. These perceptual deficits are sometimes accompanied by problems apprehending emotional expression (Ariel & Sadeh, 1996;De Haan & Campbell, 1991;Garrido et al., 2009;Kracke, 1994;Minnebusch, Suchan, Ramon, & Daum, 2007), age (Ariel & Sadeh, 1996;De Haan & Campbell, 1991;Kracke, 1994), gender (Ariel & Sadeh, 1996, De Haan & Campbell, 1991, and attractiveness Le Grand et al., 2006), although it is unclear whether these latter problems are purely perceptual in nature. ...
... Perhaps unsurprisingly, more general tests of face perception, such as the Cambridge Face Perception Test (Avidan et al., 2011;Chatterjee & Nakayama, 2012;Dingle, Duchaine, & Nakayama, 2005;Duchaine, Germine, & Nakayama, 2007), face matching paradigms (Ariel & Sadeh, 1996;Behrmann et al., 2005;Humphreys, Avidan, & Behrmann, 2007;Lee et al., 2009;Nunn et al., 2001) and matching across viewpoint (Behrmann et al., 2005;Duchaine, 2000;Lee et al., 2009;Schmalzl et al., 2008), that are not designed to isolate a particular type of processing (such as the recovery of first-or secondorder relations), also unveil impairment. These perceptual deficits are sometimes accompanied by problems apprehending emotional expression (Ariel & Sadeh, 1996;De Haan & Campbell, 1991;Garrido et al., 2009;Kracke, 1994;Minnebusch, Suchan, Ramon, & Daum, 2007), age (Ariel & Sadeh, 1996;De Haan & Campbell, 1991;Kracke, 1994), gender (Ariel & Sadeh, 1996, De Haan & Campbell, 1991, and attractiveness Le Grand et al., 2006), although it is unclear whether these latter problems are purely perceptual in nature. ...
... Perhaps unsurprisingly, more general tests of face perception, such as the Cambridge Face Perception Test (Avidan et al., 2011;Chatterjee & Nakayama, 2012;Dingle, Duchaine, & Nakayama, 2005;Duchaine, Germine, & Nakayama, 2007), face matching paradigms (Ariel & Sadeh, 1996;Behrmann et al., 2005;Humphreys, Avidan, & Behrmann, 2007;Lee et al., 2009;Nunn et al., 2001) and matching across viewpoint (Behrmann et al., 2005;Duchaine, 2000;Lee et al., 2009;Schmalzl et al., 2008), that are not designed to isolate a particular type of processing (such as the recovery of first-or secondorder relations), also unveil impairment. These perceptual deficits are sometimes accompanied by problems apprehending emotional expression (Ariel & Sadeh, 1996;De Haan & Campbell, 1991;Garrido et al., 2009;Kracke, 1994;Minnebusch, Suchan, Ramon, & Daum, 2007), age (Ariel & Sadeh, 1996;De Haan & Campbell, 1991;Kracke, 1994), gender (Ariel & Sadeh, 1996, De Haan & Campbell, 1991, and attractiveness Le Grand et al., 2006), although it is unclear whether these latter problems are purely perceptual in nature. ...
Developmental prosopagnosia (DP) is commonly associated with the failure to properly perceive individuating facial properties, notably those conveying configural or holistic content. While this may indicate that the primary impairment is perceptual, it is conceivable that some cases of DP are instead caused by a memory impairment, with any perceptual complaint merely allied rather than causal. To investigate this possibility, we administered a battery of face perception tasks to 11 individuals who reported that their face recognition difficulties disrupt daily activity and who also performed poorly on two formal tests of face recognition. Group statistics identified, relative to age- and gender-matched controls, difficulties in apprehending global-local relations and the holistic properties of faces, and in matching across viewpoints, but these were mild in nature and were not consistently evident at the level of individual participants. Six of the 11 individuals failed to show any evidence of perceptual impairment. In the remaining five individuals, no single perceptual deficit, or combination of deficits, was necessary or sufficient for poor recognition performance. These data suggest that some cases of DP are better explained by a memorial rather than perceptual deficit, and highlight the relevance of the apperceptive/associative distinction more commonly applied to the allied syndrome of acquired prosopagnosia.
... showing no structural lesion (though see Garrido et al., 2009 for evidence of subtle changes in grey matter volume) and seemingly evident from the early years of life (e.g., Temple, 1992; Kracke, 1994; Ariel & Sadeh, 1996; Bentin, Deouell, & Soroker, 1999; Grueter et al., 2007; Avidan, Tanzer, & Behrmann, 2011; Rivolta, Palermo, Schmalzl, & Coltheart, 2012). Both acquired and developmental prosopagnosia (DP) show a relatively high co-occurrence with difficulties in object identification (Behrmann, Avidan, Marotta, & Kimchi, 2005; Gauthier, Behrmann, & Tarr, 1999) and poor navigational skill (De Haan & Campbell, 1991; Duchaine, Parker, & Nakayama, 2003; Jones & Tranel, 2001). ...
... Lower-level deficits associated with the recovery of first-order relations, such as those capturing the familiar configuration of the eyes, nose and mouth (Garrido, Duchaine, & Nakayama, 2008), and the apprehension of global-local hierarchical levels (as tested with Navon compound letters; Navon, 1977) (Avidan et al., 2011; Behrmann et al., 2005: Schmalzl et al., 2008) have also been reported. Perhaps unsurprisingly, more general tests of face perception, such as the Cambridge Face Perception Test (Avidan et al., 2011; Chatterjee & Nakayama, 2012; Dingle, Duchaine, & Nakayama, 2005; Duchaine, Germine, & Nakayama, 2007), face matching paradigms (Ariel & Sadeh, 1996; Behrmann et al., 2005; Humphreys, Avidan, & Behrmann, 2007; Lee et al., 2009; Nunn et al., 2001) and matching across viewpoint (Behrmann et al., 2005; Duchaine, 2000; Lee et al., 2009; Schmalzl et al., 2008), that are not designed to isolate a particular type of processing (such as the recovery of first-or secondorder relations), also unveil impairment. These perceptual deficits are sometimes accompanied by problems apprehending emotional expression (Ariel & Sadeh, 1996; De Minnebusch, Suchan, Ramon, & Daum, 2007), age (Ariel & Sadeh, 1996; De Haan & Campbell, 1991; Kracke, 1994), gender (Ariel & Sadeh, 1996, De Haan & Campbell, 1991), and attractiveness ( Le Grand et al., 2006), although it is unclear whether these latter problems are purely perceptual in nature. ...
... Perhaps unsurprisingly, more general tests of face perception, such as the Cambridge Face Perception Test (Avidan et al., 2011; Chatterjee & Nakayama, 2012; Dingle, Duchaine, & Nakayama, 2005; Duchaine, Germine, & Nakayama, 2007), face matching paradigms (Ariel & Sadeh, 1996; Behrmann et al., 2005; Humphreys, Avidan, & Behrmann, 2007; Lee et al., 2009; Nunn et al., 2001) and matching across viewpoint (Behrmann et al., 2005; Duchaine, 2000; Lee et al., 2009; Schmalzl et al., 2008), that are not designed to isolate a particular type of processing (such as the recovery of first-or secondorder relations), also unveil impairment. These perceptual deficits are sometimes accompanied by problems apprehending emotional expression (Ariel & Sadeh, 1996; De Minnebusch, Suchan, Ramon, & Daum, 2007), age (Ariel & Sadeh, 1996; De Haan & Campbell, 1991; Kracke, 1994), gender (Ariel & Sadeh, 1996, De Haan & Campbell, 1991), and attractiveness ( Le Grand et al., 2006), although it is unclear whether these latter problems are purely perceptual in nature. At first glance, these findings might be taken as evidence that DP is perceptual in origin, with impaired recall arising because faces are not adequately encoded at the level of the structural description. ...
... However, for each ability that is sometimes impaired, some developmental prosopagnosics have been shown to perform normally. Some show deficits with other types of face processing such as emotion recognition (Ariel & Sadeh, 1996;de Haan & Campbell, 1991;Duchaine, 2000;Kracke, 1994) and gender discrimination (Ariel & Sadeh, 1996;de Haan & Campbell, 1991;Jones & Tranel, 2001). Many, though not all (Bentin, Deouell, & Soroker, 1999;Nunn et al., 2001) developmental prosopagnosics have trouble with nonface object recognition (Ariel & Sadeh, 1996;Laeng & Caviness, 2001;McConachie, 1976), but usually this affects only exemplar recognition (a particular car, a particular horse), not basic-level recognition (cars or horses, in general). ...
... However, for each ability that is sometimes impaired, some developmental prosopagnosics have been shown to perform normally. Some show deficits with other types of face processing such as emotion recognition (Ariel & Sadeh, 1996;de Haan & Campbell, 1991;Duchaine, 2000;Kracke, 1994) and gender discrimination (Ariel & Sadeh, 1996;de Haan & Campbell, 1991;Jones & Tranel, 2001). Many, though not all (Bentin, Deouell, & Soroker, 1999;Nunn et al., 2001) developmental prosopagnosics have trouble with nonface object recognition (Ariel & Sadeh, 1996;Laeng & Caviness, 2001;McConachie, 1976), but usually this affects only exemplar recognition (a particular car, a particular horse), not basic-level recognition (cars or horses, in general). ...
... Some show deficits with other types of face processing such as emotion recognition (Ariel & Sadeh, 1996;de Haan & Campbell, 1991;Duchaine, 2000;Kracke, 1994) and gender discrimination (Ariel & Sadeh, 1996;de Haan & Campbell, 1991;Jones & Tranel, 2001). Many, though not all (Bentin, Deouell, & Soroker, 1999;Nunn et al., 2001) developmental prosopagnosics have trouble with nonface object recognition (Ariel & Sadeh, 1996;Laeng & Caviness, 2001;McConachie, 1976), but usually this affects only exemplar recognition (a particular car, a particular horse), not basic-level recognition (cars or horses, in general). About one third of those who have contacted us have difficulties with everyday large-scale navigation (Duchaine, Parker, & Nakayama, 2003b), and approximately one fifth report that they have trouble understanding speech in noisy settings. ...
... Other deficits of face processing in developmental prosopagnosia are also subject of debate. For example, some studies found impaired gender recognition in congenital prosopagnosics (Ariel & Sadeh, 1996;Duchaine & Nakayama, 2006a), while others reported gender recognition to be normal . Also, some, but not all prosopagnosic participants show impairments in object recognition (Kress & Daum, 2003;Le Grand et al., 2006). ...
... Most prosopagnosics self-report normal recognition of the gender of faces (Gru¨ter, Gru¨ter, & Carbon, 2008) which is also reflected by the results of behavioral studies DeGutis, Chatterjee, Mercado, & Nakayama, 2012;Le Grand et al., 2006). Nevertheless, there are some single-case studies which report prosopagnosics' gender recognition to be impaired (Ariel & Sadeh, 1996;De Haan & Campbell, 1991;Duchaine, Yovel, Butterworth, & Nakayama, 2006;Jones & Tranel, 2001). In view of those conflicting reports, we aimed at clarifying this issue. ...
... This differs from the self-reports of prosopagnosics (Gru¨ter et al., 2008) and also from behavioral tests in several studies DeGutis et al., 2012;Le Grand et al., 2006). However, there are some single case studies of prosopagnosics which report impairments of gender recognition (Ariel & Sadeh, 1996;De Haan & Campbell, 1991;Duchaine et al., 2006;Jones & Tranel, 2001). But to the best of our knowledge, our study is the first to report an impairment in gender recognition on a groupwise level for prosopagnosics. ...
Congenital prosopagnosia, the innate impairment in recognizing faces, is a very heterogeneous disorder with different phenotypical manifestations. To investigate the nature of prosopagnosia in more detail, we tested 16 prosopagnosics and 21 controls with an extended test battery addressing various aspects of face recognition. Our results show that prosopagnosics exhibited significant impairments in several face recognition tasks: impaired holistic processing (they were tested amongst others with the Cambridge Face Memory Test (CFMT)) as well as reduced processing of configural information of faces. This test battery also revealed some new findings. While controls recognized moving faces better than static faces, prosopagnosics did not exhibit this effect. Furthermore, prosopagnosics had significantly impaired gender recognition—which is shown on a groupwise level for the first time in our study. There was no difference between groups in the automatic extraction of face identity information or in object recognition as tested with the Cambridge Car Memory Test. In addition, a methodological analysis of the tests revealed reduced reliability for holistic face processing tests in prosopagnosics. To our knowledge, this is the first study to show that prosopagnosics showed a significantly reduced reliability coefficient (Cronbach’s alpha) in the CFMT compared to the controls. We suggest that compensatory strategies employed by the prosopagnosics might be the cause for the vast variety of response patterns revealed by the reduced test reliability. This finding raises the question whether classical face tests measure the same perceptual processes in controls and prosopagnosics.
... Other deficits of face processing in developmental prosopagnosia are also subject of debate. For example, some studies found impaired gender recognition in congenital prosopagnosics (Ariel & Sadeh, 1996;Duchaine & Nakayama, 2006a), while others reported gender recognition to be normal . Also, some, but not all prosopagnosic participants show impairments in object recognition (Kress & Daum, 2003;Le Grand et al., 2006). ...
... Most prosopagnosics self-report normal recognition of the gender of faces (Gru¨ter, Gru¨ter, & Carbon, 2008) which is also reflected by the results of behavioral studies DeGutis, Chatterjee, Mercado, & Nakayama, 2012;Le Grand et al., 2006). Nevertheless, there are some single-case studies which report prosopagnosics' gender recognition to be impaired (Ariel & Sadeh, 1996;De Haan & Campbell, 1991;Duchaine, Yovel, Butterworth, & Nakayama, 2006;Jones & Tranel, 2001). In view of those conflicting reports, we aimed at clarifying this issue. ...
... This differs from the self-reports of prosopagnosics (Gru¨ter et al., 2008) and also from behavioral tests in several studies DeGutis et al., 2012;Le Grand et al., 2006). However, there are some single case studies of prosopagnosics which report impairments of gender recognition (Ariel & Sadeh, 1996;De Haan & Campbell, 1991;Duchaine et al., 2006;Jones & Tranel, 2001). But to the best of our knowledge, our study is the first to report an impairment in gender recognition on a groupwise level for prosopagnosics. ...
The fact that people are better at recognizing faces of their own race than others is called the other-race-effect (ORE). Most studies use only a single test to map and determine the characteristics of the ORE, however. Here, we investigated how two groups of fifteen age-matched Korean and German participants recognize Asian and Caucasian faces with three experiments as part of testing a new battery for characterizing face-processing performance. Participants first underwent the standard Cambridge face memory test in which they had to learn Caucasian target faces at varying noise levels which then were to be recognized in a forced-choice task. In this task, German participants performed significantly better than Koreans (83% versus 72%). The second experiment used a standard old-new recognition task with 20 Caucasian and 20 Asian faces (courtesy of the tarrlab@CMU). Here, Koreans were better with Asian faces (d'-difference = 1.23) whereas Germans only showed a tendency towards an ORE (d'-difference = 0.44). In the third experiment, participants had to rate the similarity of Caucasian face pairs which varied parametrically along featural and configural dimensions using the morphable faces from the MPI face-database. Here, we found that Korean participants were significantly less sensitive to featural changes than German participants. In conclusion, we were able to demonstrate an ORE for most of our experimental conditions. Interestingly, data from the third experiment suggest that the ORE may be due more to lessened sensitivity to featural than to configural processing for other-race faces. Future studies will extend this new test battery to prosopagnosics.
... Consistent with this view, many DPs exhibit difficulties distinguishing unfamiliar faces presented simultaneously (Avidan, Tanzer, & Behrmann, 2011;Biotti, Gray, & Cook, 2017;Duchaine et al., 2007;Shah, Gaule, Gaigg, Bird, & Cook, 2015;White, Rivolta, Burton, Al-Janabi, & Palermo, 2017) or sequentially, either side of sub-second interval (Duchaine, Yovel, Butterworth, & Nakayama, 2006;Le Grand et al., 2006;. In addition to problems matching or recognising facial identities, many DPs appear to have problems recognising facial emotion Burns, Martin, Chan, & Xu, 2017;Duchaine et al., 2006), facial age (Ariel & Sadeh, 1996), and facial gender (Ariel & Sadeh, 1996;Esins, Schultz, Stemper, Kennerknecht, & Bulthoff, 2016). Moreover, electrophysiological markers thought to index early face encoding (e.g. the N170 ERP component) are often atypical in cases of DP (Fisher, Towler, & Eimer, 2016;Towler, Gosling, Duchaine, & Eimer, 2012;Towler, Parketny, & Eimer, 2016). ...
... Consistent with this view, many DPs exhibit difficulties distinguishing unfamiliar faces presented simultaneously (Avidan, Tanzer, & Behrmann, 2011;Biotti, Gray, & Cook, 2017;Duchaine et al., 2007;Shah, Gaule, Gaigg, Bird, & Cook, 2015;White, Rivolta, Burton, Al-Janabi, & Palermo, 2017) or sequentially, either side of sub-second interval (Duchaine, Yovel, Butterworth, & Nakayama, 2006;Le Grand et al., 2006;. In addition to problems matching or recognising facial identities, many DPs appear to have problems recognising facial emotion Burns, Martin, Chan, & Xu, 2017;Duchaine et al., 2006), facial age (Ariel & Sadeh, 1996), and facial gender (Ariel & Sadeh, 1996;Esins, Schultz, Stemper, Kennerknecht, & Bulthoff, 2016). Moreover, electrophysiological markers thought to index early face encoding (e.g. the N170 ERP component) are often atypical in cases of DP (Fisher, Towler, & Eimer, 2016;Towler, Gosling, Duchaine, & Eimer, 2012;Towler, Parketny, & Eimer, 2016). ...
Traditionally, developmental prosopagnosia (DP) has been thought of as an apperceptive
condition that hinders individuals’ ability to encode face structure. However, several authors
have recently raised the possibility that many DPs may be able to form accurate percepts,
but be unable to maintain those percepts over time. The present study sought to distinguish
these possibilities. In our first experiment 16 DPs and 22 typical controls completed a
delayed match-to-sample task with face and car stimuli, with a retention interval of 1-second
(low demand) or 6-seconds (high demand). As expected, the participants with DP were
worse than the controls at face matching, and were disproportionately impaired at matching
faces relative to cars. However, the relative degree of impairment seen in the DPs did not
interact with retention interval; they exhibited similar levels of impairment when matching
faces with 1- and 6-second delays. Next, we compared the performance of 72 DPs and 54
typical controls on the Cambridge Face Perception Test (CFPT), a task that measures face
perception ability in a way that minimises the memory demands. As expected, we found
that the DPs were impaired at the group level. This difference was not attributable to a few
individuals with an apperceptive profile; rather we found evidence that the distribution of
CFPT scores seen in the DP sample was shifted relative to that of typical controls. Some
heterogeneity is likely in any neurodevelopmental population, and DP is no different.
Generally, however, these findings suggest that selective STFM impairment may be
relatively uncommon in this population. Instead, deficits of perceptual encoding may play
a larger role in DP than currently acknowledged.
... Another study (Wilson, Palermo, Schmalzl, & Brock, 2010) reported normal object processing in an 8-year-old with DP, but only one object task was used, and the child scored in the normal range for the matched face task, suggesting that these tasks might have been too easy. This latter study and three others have reported face-and object-processing impairments in children with DP (a 5-year-old, 7-year-old, two 8year-olds, and a 12-year-old; Ariel & Sadeh, 1996;Brunsdon, Coltheart, Nickels, & Joy, 2006;McConachie, 1976), though one of the 8-year-olds was later found to have deficits in early visual cortex (Gilaie-Dotan, Perry, Bonneh, Malach, & Bentin, 2009). ...
... Our findings from children with DP are consistent with other studies that have demonstrated facespecific deficits in some children (Jones & Tranel, 2001;Wilson et al., 2010) and more general visual deficits in others (Ariel & Sadeh, 1996;Brunsdon et al., 2006;McConachie, 1976;Wilson et al., 2010). In adults, findings across studies show a similar distribution of face-specific versus general visual deficits. ...
Evidence suggests that face and object recognition depend on distinct neural circuitry within the visual system. Work with adults with developmental prosopagnosia (DP) demonstrates that some individuals have preserved object recognition despite severe face recognition deficits. This face selectivity in adults with DP indicates that face- and object-processing systems can develop independently, but it is unclear at what point in development these mechanisms are separable. Determining when individuals with DP first show dissociations between faces and objects is one means to address this question. In the current study, we investigated face and object processing in six children with DP (5-12-years-old). Each child was assessed with one face perception test, two different face memory tests, and two object memory tests that were matched to the face memory tests in format and difficulty. Scores from the DP children on the matched face and object tasks were compared to within-subject data from age-matched controls. Four of the six DP children, including the 5-year-old, showed evidence of face-specific deficits, while one child appeared to have more general visual-processing deficits. The remaining child had inconsistent results. The presence of face-specific deficits in children with DP suggests that face and object perception depend on dissociable processes in childhood.
... (Compare with localization of lesions in cases of acquired prosopagnosia; Barton et al. 2002;Bouvier and Engel 2006.) Unfortunately, the few reported cases of developmental object agnosia (case AB;McConachie 1976;De Haan and Campbell 1991), case AL (Joy and Brunsdon 2002), case NM (Duchaine el al. 2003), case TA (Jones and Tranel 2001), as well as the previous report of case LG (Ariel and Sadeh 1996), who is at the focus of the present study, did not include descriptive neuroimaging correlates. To this end, we report here a detailed functional neuroimaging and event-related potentials (ERP) study of LG, a person with developmental (perhaps congenital) rather than acquired object agnosia. ...
... LG is a 21 years old male (he was 19 years old at the time of testing) who was first diagnosed with object recognition problems in early childhood (Ariel and Sadeh 1996). He does not suffer from any neurological disease. ...
... year-old male, currently a graduate student, who was diagnosed with developmental object agnosia at an early age after exhibiting symptoms of impaired object and face recognition at the age of 2 years (Ariel & Sadeh, 1996). For example, at that age he was not able to recognize his grandparents visually or to assemble simple puzzles under visual guidance. ...
... For example, at that age he was not able to recognize his grandparents visually or to assemble simple puzzles under visual guidance. Full neuropsychological assessment at the age of 8 revealed normal intellectual and social skills, alongside profound object agnosia and prosopagnosia (Ariel & Sadeh, 1996). LG's visual agnosia was further investigated during adulthood in a series of studies These results may point to a potential developmental dissociation between LG's dorsal-related and ventral-related functions. ...
According to the two visual systems model, the cortical visual system is segregated into a ventral pathway mediating object recognition, and a dorsal pathway mediating visuomotor control. In the present study we examined whether the visual control of action could develop normally even when visual perceptual abilities are compromised from early childhood onward. Using his fingers, LG, an individual with a rare developmental visual object agnosia, manually estimated (perceptual condition) the width of blocks that varied in width and length (but not in overall size), or simply picked them up across their width (grasping condition). LG's perceptual sensitivity to target width was profoundly impaired in the manual estimation task compared to matched controls. In contrast, the sensitivity to object shape during grasping, as measured by maximum grip aperture (MGA), the time to reach the MGA, the reaction time and the total movement time were all normal in LG. Further analysis, however, revealed that LG’s sensitivity to object shape during grasping emerged at a later time stage during the movement than in the controls. Taken together, these results demonstrate a dissociation between action and perception of object shape, and also point to a distinction between different stages of the grasping movement, namely planning versus online control. Moreover, the present study implies that visuomotor abilities can develop normally even when perceptual abilities developed in a profoundly impaired fashion.
... This dichotomy attributes the former condition to a deficit in deriving a sufficiently intact perception, whereas in the latter type, the root of the impairment is at the level of recognition or assignment of meaning. 3,4 CP contrasts with the more general term "developmental prosopagnosia", which includes not only individuals with CP but also those who have sustained brain damage either before birth or in early childhood. 2 Notably, in recent years a growing number of CP cases have been reported, but whether this is due to increasing prevalence or increased recognition of the disorder, is not known. ...
... 15 Cognitive models of normal visual information processing are increasingly used as frameworks for explaining neuropsychological impairment. 4,16 Within the framework of the Bruce and Young (1986) model of face recognition, the locus of impairment in "apperceptive" prosopagnosia is at a relatively early stage of face processing where abstract structural descriptions of the encountered face stimuli are generated by the visual system. By contrast, in "associative" prosopagnosia, face perception abilities are preserved, but recognition cannot take place because face recognition units (FRU) are destroyed, or cannot be accessed. ...
Prosopagnosia is a visual agnosia characterized by an inability to recognize previously known human faces and to learn new faces. The aim of this study was to present a forty-six year-old woman with congenital prosopagnosia, and to discuss the neural bases of perception and recognition of faces. The patients had a lifetime impairment in recognizing faces of family members, close friends, and even her own face in photos. She also had impairment in recognizing animals such as discriminating between cats and dogs. The patient's basic visual skills showed impairment in identifying and recognizing the animal form perception on the coding subtest of the WAIS-R, recognizing overlapping pictures (Luria), and in identifying silhouettes depicting animals and objects (VOSP). Unconventional tests using pictures evidenced impairment in her capacity to identify famous faces, facial emotions and animals. Her face perception abilities were preserved, but recognition could not take place. Therefore, it appears that the agnosia in this case best fits the group of categories termed "associative".
... Furthermore, unlike controls, LG's facial expression recognition was not affected by the presence of a conflicting bodily expression. (Ariel & Sadeh, 1996;Aviezer, Hassin, & Bentin, 2012). Overall, there seems to be evidence that DPs are more likely to score worse on tasks of facial emotion recognition than controls. ...
... In our sample of DPs however, the results seem in line with the suggestion of Calder and Young (2005), indicating that the mechanisms for both abilities overlap to some extent, as we find a less than normal emotion recognition ability even though their scores aren't so far behind controls as to warrant impairments in daily life (Ariel & Sadeh, 1996;Aviezer et al., 2012;de Haan & Campbell, 1991;Duchaine et al., 2006;McConachie & Helen, 1976). However, this goes against the model proposed by Bruce & Young (1986) and Haxby et al. (2000) and also conflicts with those reports that find unimpaired face recognition ability in DP (Dinkelacker et al., 2011;Duchaine et al., 2003;Humphreys et al., 2007;Lee et al., 2010;Nunn et al., 2001;Palermo et al., 2011). ...
Chapter 1: Introduction.
Part 1: Perception of identity and emotion.
Chapter 2: The Facial Expression Action Stimulus Test. A test battery for the assessment of face memory, face and object perception, configuration processing and facial expression recognition.
Chapter 3: Configuration perception, face memory and face context effects in developmental prosopagnosia.
Chapter 4: Facial identity and emotional expression recognition in developmental prosopagnosia.
Chapter 5: Recognition and integration of facial and bodily expressions in acquired prosopagnosia.
Part 2: Emotional social interactions between two or more people
Chapter 6: The Body Action Coding System I. Muscle activations during the perception and expression of emotion.
Chapter 7: The Body Action Coding System II. Muscle activations during the perception and expression of emotion.
Chapter 8: From personal fear to mass panic: The neurological basis of crowd perception.
Chapter 9: Discussion
... Here we study the case of a young patient who suffered from a severe developmental impairment affecting the perception of visual objects while sparing motion perception and other dorsal stream functions. As compared to the few similar published cases (Ariel and Sadeh, 1996;Joy and Brunsdon, 2002;Eriksson et al., 2003;Gilaie-Dotan et al., 2009, 2011, anatomical and functional imaging allows us to relate this impairment to a probable lack of functional differentiation of the ventral visual stream, which resulted from abnormal electrical activity and cortical development in occipital and temporal visual regions. ...
... Finally, patient LG received the most extensive scrutiny. When first studied at the age of 8 (Ariel and Sadeh, 1996), LG showed an object recognition impairment which, like in patient MJ, depended heavily on the quality of stimuli, ranging from black and white line drawings to photographs and real objects (10%, 57% and 78% correct, respectively). He was also severely prosopagnosic. ...
We report the case of a 14-year-old girl suffering from severe developmental visual impairment along with delayed language and cognitive development, and featuring a clear-cut dissociation between spared dorsal and impaired ventral visual pathways. Visual recognition of objects, including faces and printed words, was affected. In contrast, movement perception and visually guided motor control were preserved. Structural MRI was normal on inspection, but Voxel Based Morphometry (VBM) revealed reduced grey matter density in the mesial occipital and ventral occipito-temporal cortex. Functional MRI during the perception of line drawings uncovered impaired differentiation which is normally observed at even younger ages: no local category preferences could be identified within the occipito-temporal cortex for faces, houses, words or tools. In contrast, movement-related activations appeared to be normal. Finally, those abnormalities evolved on the background of chronic bilateral occipital epileptic activity, including continuous spike-wave discharges during sleep, which may be considered as the primary cause of non-specific intellectual disability and visual impairment.
Copyright © 2015. Published by Elsevier Ltd.
... An early survey of the literature found ten subjects with impairments of both perception and imagery, as inferred from their drawings and verbal responses to questions, and argued for degradation of longterm visual memories (Farah, 1984). This was followed by case reports of intact imagery on questionnaires in agnosia (Bartolomeo et al., 1998;Behrmann et al., 1992Behrmann et al., , 1994, including the subtypes of form agnosia (Servos & Goodale, 1995), apperceptive agnosia (Aglioti et al., 1999;Grossman et al., 1997;Shelton et al., 1994), integrative agnosia (Ariel & Sadeh, 1996), and associative agnosia (Jankowiak et al., 1992;Trojano & Grossi, 1992). On the other hand, various imagery impairments have been described in other agnosic patients, sometimes based on drawings and descriptions (Ogden, 1993;Wilson & Davidoff, 1993), sometimes on questionnaires (Farah et al., 1988a;Mehta et al., 1992). ...
Visual imagery has a close overlapping relationship with visual perception. Posterior cortical atrophy (PCA) is a neurodegenerative syndrome marked by early impairments in visuospatial processing and visual object recognition. We asked whether PCA would therefore also be marked by deficits in visual imagery, tested using objective forced-choice questionnaires, and whether imagery deficits would be selective for certain properties. We recruited four patients with PCA and a patient with integrative visual agnosia due to bilateral occipitotemporal strokes for comparison. We administered a test battery probing imagery for object shape, size, colour lightness, hue, upper-case letters, lower-case letters, word shape, letter construction, and faces. All subjects showed significant impairments in visual imagery, with imagery for lower-case letters most likely to be spared. We conclude that PCA subjects can show severe deficits in visual imagery. Further work is needed to establish how frequently this occurs and how early it can be found.
... These criticisms apply less to a study of developmental prosopagnosia (Duchaine, Parker & Nakayama 2003). Although, some studies in developmental prosopagnosics do report impaired facial expression recognition (De Haan & Campbell 1991;Ariel & Sadeh 1996;Duchaine et al. 2006), a review of studies show that certain cases of developmental prosopagnosia clearly have unimpaired face expression recognition, even across multiple tests for face expression recognition (Duchaine, Parker & Nakayama 2003). ...
The primary models of understanding human face recognition aim to understand not only facial identity information processing but also non-identity facial information processing. The present review discusses the major theoretical approaches in this regard and critically evaluates the existing empirical evidence in conjunction with the said models.
... So far in the literature, this syndrome has primarily been studied in adults. However, a few cases of this syndrome have also been reported in children, and even the adult studies suggest an early onset of these problems (Ariel and Sadeh, 1996;Grueter et al., 2007). ...
Faces are central to human communication and social functioning, conveying a wealth of information, from identity to gender to emotional states. In this chapter, an overview of the research on the development of face processing, from infancy to adulthood, is presented. For each stage of development, findings from both behavioral and neurophysiological studies are reviewed. A summary of impairments in face development that emerge as a result of brain injury and neurological syndromes is also provided.
... A very small number of 2017 papers are also included as we had access to them prior to July 2016-at that time their definitive publication date was unknown. We identified the candidate papers by (a) searching PubMed with the term "prosopagnosia" and/or (2) using the forward search function in Web of Science, taking as the seed four relatively early but key papers in the CP literature reporting behavioural findings: Behrmann et al. (2005); Ariel and Sadeh (1996);Duchaine (2000); and Galaburda and Duchaine (2003). We assumed that at least one of these papers would be cited by any subsequent article. ...
... Leading models of face processing propose that a common structural description, formed early in the face processing stream, informs subsequent processing of identity, expression, age, and sex, mediated by largely independent, divergent pathways 25,26 . Subtle deficits of facial sex, and facial emotion perception 33 , together with problems sorting simultaneously presented faces by similarity 24 , suggest that this early structural description may be impoverished in DP. ...
Developmental prosopagnosia (DP) is a neurodevelopmental condition characterised by difficulties recognising and discriminating faces. It is currently unclear whether the perceptual impairments seen in DP are restricted to identity information, or also affect the perception of other facial characteristics. To address this question, we compared the performance of 17 DPs and matched controls on two sensitive sex categorisation tasks. First, in a morph categorisation task, participants made binary decisions about faces drawn from a morph continuum that blended incrementally an average male face and an average female face. We found that judgement precision was significantly lower in the DPs than in the typical controls. Second, we used a sex discrimination task, where female or male facial identities were blended with an androgynous average face. We manipulated the relative weighting of each facial identity and the androgynous average to create four levels of signal strength. We found that DPs were significantly less sensitive than controls at each level of difficulty. Together, these results suggest that the visual processing difficulties in DP extend beyond the extraction of facial identity and affects the extraction of other facial characteristics. Deficits of facial sex categorisation accord with an apperceptive characterisation of DP.
... Leading models of face processing propose that a common structural description, formed early in the face processing stream, informs subsequent processing of identity, expression, age, and sex, mediated by largely independent, divergent pathways 25,26 . Subtle deficits of facial sex, and facial emotion perception 33 , together with problems sorting simultaneously presented faces by similarity 24 , suggest that this early structural description may be impoverished in DP. ...
... The 8-year-old boy LG was presented by Ariel and Sadeh [32]. He had a high IQ and intact basic perceptual skills. ...
In this chapter, I will present an overview of early case descriptions of specific isolated cognitive deficits in children for which no clear brain impairment could be demonstrated and which were therefore considered to be congenital or developmental in nature. Three kinds of syndromes will be discussed. First, more general deficits like the attention and hyperactivity disorder and congenital aphasia will be presented. The second category relates to the more specific cognitive deficits, like developmental prosopagnosia, that have been reported, especially from the early 1980s onwards. In particular, early reports of specific congenital deficits in the areas of attention, language, perception, and memory will be presented. And finally, I will briefly discuss early case descriptions of individuals with autism and savant syndrome. Instead of suffering from a specific cognitive deficit, the latter show a special talent.
... Así, por ejemplo, numerosos pacientes comienzan a desarrollar este déficit a partir de una determinada lesión que afecta a alguna zona específica del cerebro, lo que podemos identificar como 'prosopagnosia adquirida'; es decir, en estos pacientes podríamos establecer una relación directa de causa y efecto entre la lesión cerebral y su déficit en la percepción, el reconocimiento y la identificación de rostros. En cambio, en la literatura científica encontramos otros casos clínicos de pacientes cuya dificultad para percibir, reconocer o identificar rostros conocidos se observa a lo largo de toda su vida, y en muchos casos en ausencia de una lesión neurológica conocida [3][4][5], en lo que podríamos identificar como 'prosopagnosia evolutiva o desarrollada'. En este sentido, algunos casos de prosopagnosia evolutiva leve podrían identificarse erróneamente en personas jóvenes como un problema de tipo atencional. ...
... Selective impairments in visual object processing occur in children with acquired (O'Hare, Dutton, Green, & Coull, 1998;Temple, 1997;Young & Ellis, 1989) and congenital brain damage (Ariel & Sadeh, 1996;Joy & Brunsdon, 2002). Research strongly supports the use of cognitive neuropsychological models in assessment and treatment of visual processing disorders in childhood (Brunsdon, Nickels, Coltheart, & Joy, 2007;Joy & Brunsdon, 2002;Schmalzl, Palermo, Green, Brunsdon, & Coltheart, 2008;Temple, 1997). ...
The Birmingham Object Recognition Battery (BORB) is a theoretically based test battery that is used in adult cognitive neuropsychology in research and for clinical assessment. It allows a detailed analysis of underlying impairments in individuals with brain injury who have visual object recognition difficulties. The BORB’s usefulness in pediatrics is supported by numerous research studies. However, there is no published normative data for children, making clinical use of the test difficult. The aim of this brief report is to publish some preliminary normative data in 70 children aged between 3 and 8 years to assist both researchers and clinicians with interpretation of test scores. Results indicate that children’s performance on individual BORB subtests varies according to task demands and age. For some subtests there is improvement in performance with increasing age. However, very young children (age 3-4 years) perform at adult levels on some subtests, or alternatively on other subtests they perform at the level of chance. The current paper supports the need for pediatric data for the BORB due to large normal individual variation in performance and varying age-related performance on individual BORB subtests.
... A very small number of 2017 papers are also included as we had access to them prior to July 2016-at that time their definitive publication date was unknown. We identified the candidate papers by (a) searching PubMed with the term "prosopagnosia" and/or (2) using the forward search function in Web of Science, taking as the seed four relatively early but key papers in the CP literature reporting behavioural findings: Behrmann et al. (2005); Ariel and Sadeh (1996); Duchaine (2000); and Galaburda and Duchaine (2003). We assumed that at least one of these papers would be cited by any subsequent article. ...
A longstanding controversy concerns the functional organization of high-level vision, and the extent to which the recognition of different classes of visual stimuli engages a single system or multiple independent systems. We examine this in the context of congenital prosopagnosia (CP), a neurodevelopmental disorder in which individuals, without a history of brain damage, are impaired at face recognition. This paper reviews all CP cases from 1976 to 2016, and explores the evidence for the association or dissociation of face and object recognition. Of the 238 CP cases with data permitting a satisfactory evaluation, 80.3% evinced an association between impaired face and object recognition whereas 19.7% evinced a dissociation. We evaluate the strength of the evidence and correlate the face and object recognition behaviour. We consider the implications for theories of functional organization of the visual system, and offer suggestions for further adjudication of the relationship between face and object recognition.
... We have found that BN was impaired in contour integration (performed at an age level of 3-4 years) and in figure-ground separation (in some of the tests). These deficits are similar to those of LG, a young adult with developmental visual object agnosia (Ariel and Sadeh 1996, Gilaie-Dotan et al. 2009, Gilaie-Dotan 2016. Like BN, LG's contour integration was impaired when tested with the paradigm used here , Gilaie-Dotan et al. 2009, Lev et al. 2015. ...
Visual categories are associated with eccentricity biases in high-order visual cortex: Faces and reading with foveally-biased regions, while common objects and space with mid- and peripherally-biased regions. As face perception and reading are among the most challenging human visual skills, and are often regarded as the peak achievements of a distributed neural network supporting common objects perception, it is unclear why objects, which also rely on foveal vision to be processed, are associated with mid-peripheral rather than with a foveal bias.
... The BFRT was included to investigate the perceptual aspects of face recognition without involvement of the mnemonic component because recent studies have suggested that some, but not all, individuals with CP can experience face discrimination difficulties in addition to face memory impairment (Ariel & Sadeh, 1996;de Gelder & Rouw, 2000). ...
Objective:
The lack of inversion effect for face recognition in congenital prosopagnosia (CP) is consistent with the hypothesis of a failure in holistic processing. However, although CPs' abnormal gaze behavior for upright faces has already been demonstrated, neither their scanning strategy for inverted faces, nor the possibility that their abnormal gaze behavior with upright faces is because of reasons other than the holistic deficit have been investigated yet.
Method:
We recorded the eye movements of a congenital prosopagnosic and a control group during the encoding of unknown faces, objects, and flowers. Two types of stimuli (faces and objects) were presented upright and inverted.
Results:
CPs explored upright and inverted faces in the same way (i.e., similar number of fixations of the same duration and similarly distributed), whereas controls increased the number of fixations and their duration during the presentation of inverted faces. By contrast, the 2 groups showed a similar inversion effect during the encoding of objects. Finally, CPs showed anomalous exploration of within-class objects (i.e., flowers) and impairment in subordinate-level object discrimination.
Conclusions:
Our results demonstrate that: (a) CPs use the same part-based strategy in encoding both upright and inverted faces, suggesting a possible interpretation of the lack of inversion effect in this population; (b) CPs' lack of inversion effect is face-specific and does not affect objects; (c) however, CPs' deficit seems not to be limited to faces, and to extend to individual-item recognition within a class. (PsycINFO Database Record
... Nevertheless, many DPs report problems recognising facial expressions in their daily lives (e.g. Lee et al., 2010), and case studies have described individuals with DP, who do exhibit deficits of expression recognition (Ariel & Sadeh, 1996;De Haan & Campbell, 1991;Duchaine, Murray, Turner, White, & Garrido, 2009;Duchaine, Yovel, Butterworth, & Nakayama, 2006;Minnebusch, Suchan, Ramon, & Daum, 2007;Schmalzl et al., 2008). For example, Duchaine et al. (2006) described a 53-year-old male DP, Edward, who exhibited clear expression recognition impairments on The Reading the Mind in the Eyes Test and on a 3-AFC match-to-sample task. ...
Developmental prosopagnosia is a neurodevelopmental condition characterised by difficulties recognising faces. Despite severe difficulties recognising facial identity, expression recognition is typically thought to be intact in developmental prosopagnosia; case studies have described individuals who are able to correctly label photographic displays of facial emotion, and no group differences have been reported. This pattern of deficits suggests a locus of impairment relatively late in the face processing stream, after the divergence of expression and identity analysis pathways. To date, however, there has been little attempt to investigate emotion recognition systematically in a large sample of developmental prosopagnosics using sensitive tests. In the present study, we describe three complementary experiments that examine emotion recognition in a sample of 17 developmental prosopagnosics. In Experiment 1, we investigated observers’ ability to make binary classifications of whole-face expression stimuli drawn from morph continua. In Experiment 2, observers judged facial emotion using only the eye-region (the rest of the face was occluded). Analyses of both experiments revealed diminished ability to classify facial expressions in our sample of developmental prosopagnosics, relative to typical observers. Imprecise expression categorisation was particularly evident in those individuals exhibiting apperceptive profiles, associated with problems encoding facial shape accurately. Having split the sample of prosopagnosics into apperceptive and non-apperceptive subgroups, only the apperceptive prosopagnosics were impaired relative to typical observers. In our third experiment, we examined the ability of observers’ to classify the emotion present within segments of vocal affect. Despite difficulties judging facial emotion, the prosopagnosics exhibited excellent recognition of vocal affect. Contrary to the prevailing view, our results suggest that many prosopagnosics do experience difficulties classifying expressions, particularly those with apperceptive profiles. These individuals may have difficulties forming view-invariant structural descriptions at an early stage in the face processing stream, before identity and expression pathways diverge.
... Bodamer's theoretical, developmental perspective on the particular nature of visual face recognition is also supported by reports of single cases with developmental, congenital prosopagnosia. The condition is characterized by a child's inability to learn to recognize faces, while recognition performance for other visual categories is less affected, although not normal (e.g., Ariel & Sadeh, 1996;Barton, Cherkasova, Press, Intriligator, & O'Connor, 2003). Identification of facial expressions is more often preserved in developmental compared with acquired prosopagnosia (K. ...
Visual neuroscience is concerned with the neurobiological foundations of visual perception, that is, the morphological, physiological, and functional organization of the visual brain and its co-operative partners. One important approach for understanding the functional organization of the visual brain is the study of visual perception from the pathological perspective. The study of patients with focal injury to the visual brain allows conclusions about the representation of visual perceptual functions in the framework of association and dissociation of functions. Selective disorders have been reported for more "elementary" visual capabilities, for example, color and movement vision, but also for visuo-cognitive capacities, such as visual agnosia or the visual field of attention. Because these visual disorders occur rather seldom as selective and specific dysfunctions, single cases have always played, and still play, a significant role in gaining insights into the functional organization of the visual brain. © 2016 The Institute of Psychology, Chinese Academy of Sciences and John Wiley & Sons Australia, Ltd.
... Contrary, the development of face-selective functional organization in the FFA does not become adult-like until early adolescence (Aylward et al., 2005;Gathers, Bhatt, Corbly, Farley, & Joseph, 2004;Passarotti et al., 2003;Scherf et al., 2007). Extremely rare appearance of prosopagnosia in children, combined with its more difficult separation from visual agnosia (Ariel & Sadeh, 1996) further support the hypothesis that child face vision stronger relies on the object recognition system than adult face vision. The performance asymmetries found for external and internal features with children may therefore reflect differently developed states of maturation of object related and face related cortical visual areas. ...
... So far in the literature, this syndrome has primarily been studied in adults. However, a few cases of this syndrome have also been reported in children, and even the adult studies suggest an early onset of these problems (Ariel and Sadeh, 1996;Grueter et al., 2007). ...
Faces are very important for humans. They convey a wealth of information, from identity to gender to emotional states. In this chapter, an overview of the research on the development of face processing, from infancy to adulthood, is presented. For each stage of development, findings from both behavioral and neurophysiological studies are reviewed. A summary of impairments in face development that emerge as a result of brain injury and neurological syndromes is also provided.
... Three of the participants with CP scored at least 1.98 SD below the mean of the control group on the BFRT, suggesting impairment in face discrimination. These results are consistent with recent work suggesting that some, but not all, individuals with CP experience difficulty with face discrimination in addition to face memory (Ariel & Sadeh, 1996;de Gelder & Rouw, 2000). ...
The existence of a drift to base judgments more on the right half-part of facial stimuli, which falls in the observer's left visual field (left perceptual bias (LPB)), in normal individuals has been demonstrated. However, less is known about the existence of this phenomenon in people affected by face impairment from birth, namely congenital prosopagnosics. In the current study, we aimed to investigate the presence of the LPB under face impairment conditions using chimeric stimuli and the most familiar face of all: the self-face. For this purpose we tested 10 participants with congenital prosopagnosia and 21 healthy controls with a face matching task using facial stimuli, involving a spatial manipulation of the left and the right hemi-faces of self-photos and photos of others. Even though congenital prosopagnosics performance was significantly lower than that of controls, both groups showed a consistent self-face advantage. Moreover, congenital prosopagnosics showed optimal performance when the right side of their face was presented, that is, right perceptual bias, suggesting a differential strategy for self-recognition in those subjects. A possible explanation for this result is discussed.
... Moreover, a study of four DPs indicated that they made typical judgements of facial trustworthiness (Todorov & Duchaine, 2008), an inference thought to be mediated by subtle emotion cues. Nevertheless, many DPs report problems recognising facial expressions in their daily lives (e.g. Lee et al., 2010), and case studies have described individuals with DP, who do exhibit deficits of expression recognition (Ariel & Sadeh, 1996; De Haan & Campbell, 1991; Duchaine, Murray, Turner, White, & Garrido, 2009; Duchaine, Yovel, Butterworth, & Nakayama, 2006; Minnebusch, Suchan, Ramon, & Daum, 2007; Schmalzl et al., 2008(1991) tested AB, the original case of DP first described by McConachie (1976), and found that as an adult she exhibited problems labelling prototypical basic emotions. Importantly, however, these reports are relatively infrequent (regarded as 'the exception' rather than 'the norm'), and no systematic investigation has found evidence for a group difference. ...
Leading models of face perception posit that, after initial structural encoding, separate streams are responsible for the analysis of identity and expression. Interestingly, many individuals with developmental prosopagnosia (DP), a condition characterized by difficulties recognizing faces, are apparently unimpaired on expression recognition tasks. This pattern of performance is suggestive of a specific deficit in the identity processing stream, rather than a deficit of structural encoding. However, some authors have suggested that this apparent dissociation may reflect the use of expression recognition tasks that lack the necessary sensitivity to detect subtle impairments. The present study sought to investigate emotion recognition in DP using a task that systematically varies judgment difficulty. Sixteen adults with DP and 16 typically developing (TD) controls completed a computer-based emotion recognition task. Stimuli consisted of cropped eye-regions taken from happy, fearful, disgusted, angry, surprised and sad faces. Each source face was morphed with an image of the same actor exhibiting no emotion to obtain three sets of stimuli with varying levels of emotion intensity (100%, 66% and 33%). Results showed that DP and TD observers did not differ in emotion recognition ability at the 100% intensity level. However, as the emotion signal became weaker (in the 66% and 33% conditions), members of the DP group were increasingly likely to exhibit evidence of impairment, relative to members of the control group. Residual perceptual sensitivity, augmented by compensatory strategies, may be sufficient to judge unambiguous expressions to a broadly typical level of performance. However, as tasks become increasingly difficult, perceptual deficits may often be revealed. Where observed, co-occurring deficits of identity and expression recognition are consistent with impaired structural encoding of faces.
Meeting abstract presented at VSS 2015
... LG is a young intelligent and completely independently-functioning adult (now 28 year old), who suffers from developmental visual agnosia and prosopagnosia. He was diagnosed with visual form agnosia as a young boy before the age of 8 (Ariel and Sadeh, 1996), following an extensive neuropsychological examination. That study found that LG had significant impairments in object and face perception, as well as a difficulty in overcoming occlusion (as when text or objects are partially covered), in contrast to his adequate social skills and normal or above normal performance on tests assessing intelligence (within the superior range in the verbal skills), language (reading, writing, verbal), basic and spatial vision, visual imagery, and short-term visual memory. ...
... Conversely, lesions in the monkey V4 [50] and IT [51] areas impaired recognition of objects only after they had been globally transformed by changing size, contrast, or degree of occlusion, while non-transformed objects were normally detected. Likewise, studies in humans with face or object agnosia [52][53][54][55][56] have consistently shown that low-level vision is not impaired; subjects were able to compare objects, read, and perceive all details comprising the objects. In other words, damage to areas downstream from V1 severely impairs transformed holistic perception and recognition but not perception of non-transformed objects or of spatial details and topography. ...
The current view posits that objects, despite changes in appearance, are uniquely encoded by 'expert' cells. This view is untenable. First, even if cell ensemble responses are invariant and unique, we are consciously aware of all of the objects' details. Second, in addition to detail preservation, data show that the current hypothesis fails to account for uniqueness and invariance. I present an alternative view whereby objects' representation and recognition are based on parallel representation of space by primary visual cortex (V1) responses. Information necessary for invariance and other attributes is handled in series by other cortical areas through integration, interpolation, and hierarchical convergence. The parallel and serial mechanisms combine to enable our flexible space perception. Only in this alternative view is conscious perception consistent with the underlying architecture.
Copyright © 2015 Elsevier Ltd. All rights reserved.
... Very little work has been done to characterize DP in children (Dalrymple et al., 2012). The largest sample size of child DPs to-date is three (Wilson et al., 2010), and the remaining studies each report only a single case (Ariel and Sadeh, 1996;Brunsdon et al., 2006;de Haan and Campbell, 1991;Jones and Tranel, 2001;Joy and Brunsdon, 2002;McConachie, 1976;Schmalzl et al., 2008). Yet testing face memory and face perception in children with DP is particularly important: children may be less adept than adults at using compensatory strategies for recognizing faces because they have had less time to develop such strategies in daily life, and they may be less likely to devise alternative strategies that are effective in laboratory tests. ...
Cognitive models propose that face recognition is accomplished through a series of discrete stages, including perceptual representation of facial structure, and encoding and retrieval of facial information. This implies that impaired face recognition can result from failures of face perception, face memory, or both. Studies of acquired prosopagnosia, autism spectrum disorders, and the development of normal face recognition support the idea that face perception and face memory are distinct processes, yet this distinction has received little attention in developmental prosopagnosia (DP). To address this issue, we tested the face perception and face memory of children and adults with DP. By definition, face memory is impaired in DP, so memory deficits were present in all participants. However, we found that all children, but only half of the adults had impaired face perception. Thus, results from adults indicate that face perception and face memory are dissociable, while the results from children provide no evidence for this division. Importantly, our findings raise the possibility that DP is qualitatively different in childhood versus adulthood. We discuss theoretical explanations for this developmental pattern and conclude that longitudinal studies are necessary to better understand the developmental trajectory of face perception and face memory deficits in DP.
... Prosopagnosia can be a transient manifestation of migraine (Martins and Cunha e Sa, 1999). There is increasing interest in a developmental form of prosopagnosia also (McConachie, 1976;Young and Ellis, 1989;de Haan and Campbell, 1991;Kracke, 1994;Ariel and Sadeh, 1996;Barton et al., 2003a;Duchaine and Nakayama, 2006), which may be associated with social developmental disorders such as Asperger syndrome (Kracke, 1994). ...
Purpose of review:
This article reviews the various types of visual dysfunction that can result from lesions of the cerebral regions beyond the striate cortex.
Recent findings:
Patients with dyschromatopsia can exhibit problems with color constancy. The apperceptive form of prosopagnosia is associated with damage to posterior occipital and fusiform gyri, and an associative/amnestic form is linked to damage to more anterior temporal regions. Pure alexia can be accompanied by a surface dysgraphia. New word-length effect criteria distinguish pure alexia from hemianopic dyslexia. Subtler problems with perception of numbers and faces can be seen in patients with pure alexia as well. Also, a developmental form of topographic disorientation, which is due to problems with forming cognitive maps of the environment, has been discovered. In Balint syndrome, added features of decreased flexibility of attention in simultanagnosia include local and global capture. Balint syndrome can affect not just localization in space, but also in time, as manifest in sequence agnosia.
Summary:
Lesions at intermediate levels of a processing hierarchy can cause difficulty with color perception or motion perception. At a higher level, ventral lesions of the occipitotemporal lobes can lead to a variety of problems with object recognition. Dorsal lesions of the occipitoparietal lobes can cause difficulty with spatial localization and guidance.
Examination tools such as functional magnetic resonance imaging (fMRI) and magnetoencephalography (MEG) have enabled extensive research in neuroanatomy in normal subjects and in individuals with congenital or acquired prosopagnosia. Greater awareness is necessary for early diagnosis and treatment.
Three different aspects of prosopagnosia are covered below.
In part one, neuroanatomical findings responsible for face recognition and identification in healthy subjects are described, highlighting the core and extended neural network of the extrastriate visual cortex. The three main areas in the core system are: the fusiform face area (FFA), the occipital face area (OFA), and posterior superior temporal sulcus (pSTS).
In part two, the essential clinical and diagnostic features of developmental (congenital) prosopagnosia (DP) are described. DP remains life-long during life. This prosopagnosia is often hereditary. Its prevalence is 2.5% among Caucasians.
Of interest to ophthalmologists are functional changes in individuals with DP. Subjects with DP exhibit normal visual acuity and visual fields.
Part three covers acquired prosopagnosia (AP), a defect in face recognition following focal brain damage, caused by various brain diseases: it is usually due to bilateral or unilateral occipitotemporal lesions or anterior temporal damage. A better functioning right hemisphere in patients with unilateral brain damage as the cause of AP should be considered. Neuro-ophthalmological deficits have often been diagnosed, i. e., visual field defects in 82%, color vision disturbances (achromatopsia or dyschromatopsia) in 23.3%. Of special interest to ophthalmologists are the findings in subjects’ gaze behavior. There is evidence that averted-gaze and mutual gaze-activated areas differ from those involved in face processing.
This thesis explored the influence of X-linked genes on the development of face- processing abilities. It assessed face-processing abilities in women with Turner syndrome (TS) who have just one, instead of two, X-chromosomes. Study One assessed the nature and severity of face processing deficits by applying a diverse battery of neuropsychological tests to 45,Xm and control females. Women with TS performed at below average levels in terms of face and emotion recognition (particularly fearful faces) despite processing faces in a typical configural manner. Study Two found equivalent deficits in 45,Xp women. Using Voxel Based Morphometry, Study Three found evidence for increased volume of the amygdalae and orbito-frental cortices in women with TS. Because males, like 45,X females, have a single X-chromosome, Study Four sought to identify whether there was any sexual dimorphism in face processing abilities - there was not. However, differences were found between normal males and females in terms of correlations between face and emotion recognition task performance. These differences were similar to those seen in 45,Xm compared with 45,Xp females and are consistent with the hypothesis that imprinted X-linked genes influence functional mechanisms that are relevant to social cognition. Together, the results of these studies suggest a role for X-linked genes in the typical development of face processing abilities. This role might involve the development of structures involved in social and emotional processing, including the amygdala and orbito-frontal cortices. It is suggested that affective responses to faces may have an important role in our subsequent memory for them. Ways in which issues raised by these studies could be explored further are discussed.
Alexithymia is a disease affecting emotional awareness. Those suffering from it are not capable of identifying the emotions they experience and thus cannot describe them: in emotionally salient situations, they are aware of internal bodily changes, but do not feel genuine emotions. In this article, we discuss the hypothesis that alexithymia is a specific form of emotional agnosia. We show that there are significant similarities between alexithymia and agnosia and argue that, on the basis of these similarities, we can draw several conclusions on the nature of emotions and on how they are processed by the cognitive system. In particular, we highlight how our analysis is relevant for the classical contraposition between cognitive and perceptual theories of emotions. In fact, the similarities between alexithymia and agnosia provide good arguments to substantiate a particular kind of perceptual theory of emotions: the identification and the recognition of emotions relies on similar mechanisms for processing proprioceptive and interceptive information as those used to process the exteroceptive information that allows us to identify and recognize objects in the external world.
Disorders of brain function can be distinguished in relation to the time of occurrence of the pathological event in brain development, prenatal (before birth), perinatal (around birth) and postnatal (after birth). Such disorders may manifest as missing, incomplete or delayed development of the function(s) affected. Practically, all functional disorders that arise during the pre-, peri- and postnatal periods, irrespective of the underlying aetiology, can be subsumed under the term ‘early developmental disorder’. For labelling of the pathological event, the global term ‘brain injury’ is used to subsume the wide range of conditions including cerebrovascular, hypoxic and traumatic events that lead to disorder and/or dysfunction of cortical and subcortical structures of the visual system, including the white matter that contains the inter- and intracortical fibre connections, being affected.
Direct link to the Dove Press article can be found here: https://www.dovepress.com/articles.php?article_id=29143
Prosopagnosia is a selective visual agnosia characterized by the inability to recognize the identity of faces. There are both acquired forms secondary to brain damage and developmental forms without obvious structural lesions. In this review, we first discuss the diagnosis of acquired and developmental prosopagnosia, and the challenges present in the latter case. Second, we discuss the evidence regarding the selectivity of the prosopagnosic defect, particularly in relation to the recognition of other objects, written words (another visual object category requiring high expertise), and voices. Third, we summarize recent findings about the structural and functional basis of prosopagnosia from studies using magnetic resonance imaging, functional magnetic resonance imaging, and event-related potentials. Finally, we discuss recent attempts at rehabilitation of face recognition in prosopagnosia.
Brain developmental disorders and those occurring in early childhood give rise to a variety of functional impairments. This does not however necessarily mean that these impairments, and their consequences for everyday life activities, are immutable, that the developmental processes and functional capacities of the (very) young brain are persistently affected or that the capacity of adaptation to these functional consequences in terms of learning will be irreversibly reduced or lost. Thus, specific and systematic intervention measures are needed as early as possible after brain injury, or the onset of brain dysfunction.
Up to now we have learned about cognitive science and its methods, about the cognitive and neural features of typical face processing, about congenital prosopagnosia and the mechanisms behind covert face recognition
Dieses Buch beschäftigt sich mit der Entwicklung und den Störungen der visuellen Wahrnehmung bei Kindern. Die einzelnen Kapitel befassen sich mit der Organisation und Funktionsweise des Zentralnervensystems, insbesondere des visuellen Systems und der Okulomotorik, der Entwicklung der verschiedenen Teilleistungen, ihren Störungen, Aspekten der Sehbehinderung sowie der Diagnostik und Dokumentation von Funktionsstörungen. Im lezten Kapitel werden die diagnostischen und therapeutischen Vorgehensweisen beispielhaft an vier Einzelfällen dargestellt. Ein umfangreiches Literaturverzeichnis sowie zahlreiche Abbildungen und Tabellen vervollständigen das Buch. Die Besonderheiten dieses Buches liegen in der empirisch fundierten Darstellung der visuellen Teilleistungen und ihrer Störungen auf dem Hintergrund der zentralnervösen Organisation von Kognition, Erleben und Handeln sowie in der Anwendung neuer wissenschaftlicher Erkenntnisse in Diagnostik und Behandlung bzw. Frühförderung von Kindern mit Sehstörungen.
Visual imagery is the creation of mental representations that share many features with veridical visual percepts. Studies of normal and brain-damaged people reinforce the view that visual imagery and visual perception are mediated by a common neural substrate and activate the same representations. Thus, brain-damaged patients with intact vision who have an impairment in perception should have impaired visual imagery. Here we present evidence to the contrary from a patient with severely impaired object recognition (visual object agnosia) but with normal mental imagery. He draws objects in considerable detail from memory and uses information derived from mental images in a variety of tasks. In contrast, he cannot identify visually presented objects, even those he has drawn himself. He has normal visual acuity and intact perception of equally complex material in other domains. We conclude that rich internal representations can be activated to support visual imagery even when they cannot support visually mediated perception of objects.
Three prosopagnosic patients were given four face tests, two perceptual (an unknown face identification test and an age estimation test) and two also implying memory (a familiarity check test and a famous face recognition test). The patients' performance was assessed with reference to the score distribution of the normal population. A patient was found to fail both perceptual and mnestic tests, without any noticeable difference between them. Also the second patient had poor scores on both kinds of tests, but his impairment was significantly greater on the perceptual ones. The third patient, on the contrary, showed no perceptual deficit and only failed the mnestic tests. His inability to recognize the individuality of an item among members of the same category was strictly confined to faces and never present for other classes of stimuli (cars, coins, personal belongings). This finding is supportive of the thesis that in a few patients the deficit underlying prosopagnosia is face specific.
Projected slides of 112 coherent or jumbled real-world scenes for single-object detection by 36 introductory psychology students. The speed at which objects were detected was reduced when the scene was jumbled. Jumbling was most disruptive when the target object was not in the scene but had a high probability of occurring in that kind of scene. Results are discussed in terms of the possible role played by schemas in the processing of information from real-world scenes. (PsycINFO Database Record (c) 2012 APA, all rights reserved)
Are faces recognized using more holistic representations than other types of stimuli? Taking holistic representation to mean representation without an internal part structure, we interpret the available evidence on this issue and then design new empirical tests. Based on previous research, we reasoned that if a portion of an object corresponds to an explicitly represented part in a hierarchical visual representation, then when that portion is presented in isolation it will be identified relatively more easily than if it did not have the status of an explicitly represented part. The hypothesis that face recognition is holistic therefore predicts that a part of a face will be disproportionately more easily recognized in the whole face than as an isolated part, relative to recognition of the parts and wholes of other kinds of stimuli. This prediction was borne out in three experiments: subjects were more accurate at identifying the parts of faces, presented in the whole object, than they were at identifying the same part presented in isolation, even though both parts and wholes were tested in a forced-choice format and the whole faces differed only by one part. In contrast, three other types of stimuli--scrambled faces, inverted faces, and houses--did not show this advantage for part identification in whole object recognition.
A left-handed woman developed visual object agnosia, prosopagnosia, and visual disorientation after resection of the right occipital lobe. Color agnosia and alexia were absent. When asked to identify objects presented visually, the patient's errors represented visually related objects (underspecifications) or perseverations. Identification was facilitated when she observed the object being used in a natural way. Identification was impaired by surrounding the object with unrelated objects, decreasing the background illumination, decreasing the duration of exposure of the object to the patient, and probably also by decreasing the visual angle subtended by the object. In addition, there were disturbances of visualization (i.e., imaging in the absence of a visual stimulus) that paralleled the perceptual difficulties. We conclude that: (1) A deficit in visual perception, characterized by insufficient feature analysis of visual stimuli, was the basis of the visual agnosia in this case. (2) The visual agnosia could not be explained by (a) a vision-language disconnection syndrome, (b) decay of visual memory traces, or (c) deficiencies in the visual fields (pathologic Funktionswandel). (3) The ability to visualize (visual imagery) probably utilizes some of the same neural pathways used in perception. (4) The results in this case probably can be generalized to some but not all cases of visual agnosia; in particular, the deficit in most previously reported patients with prosopagnosia is similar to that of our case. However, agnosic alexia and color agnosia usually have a different neuropsychological basis.
Visual object recognition was investigated in a group of eighty-one patients with right- or left-hemisphere lesions. Two tasks were used, one maximizing perceptual categorization by physical identity, the other maximizing semantic categorization by functional identity. The right-hemisphere group showed impairment on the perceptual categorization task and the left-hemisphere group were impaired on the semantic categorization task. The findings are discussed in terms of categorical stages of object recognition. A tentative model of their cerebral organization is suggested.
A patient with prosopagnosia is described. It is suggested that the deficit in this case is primarily apperceptive, and possible associated symptoms were investigated. The fact that the patient did not present initially as brain damaged is discussed.
Prosopagnosia is a neurologically based deficit characterized by the inability to recognize faces of known individuals in the absence of severe intellectual, perceptual, and memory impairments. The nature of the underlying disturbance was investigated in three patients in an attempt to identify the structural and functional levels at which the processing of faces breaks down, the relation between prosopagnosia and associated deficits, and the specificity of the prosopagnosic disturbance. The breakdown of face processing resulted from unilateral damage in different cerebral structures of the right hemisphere in the three patients, and it involved different functional levels of face processing, but all three patients displayed perceptual impairments of unequal severity. In one patient (R.M.), the deficit encompassed all perceptual operations on faces, including matching identical views of the same faces, but it did not extend to all categories of objects characterized by a close similarity among their instances; the second patient (P.M.) exhibited a less severe perceptual impairment but was unable to derive the configurational properties from a facial representation and to extract its physiognomic invariants; the third patient (P.C.) had not lost the capacity to differentiate faces on the basis of their configurations but could not associate a facial representation with its pertinent memories. Associated deficits were present in each patient but differed depending on the anatomofunctional locus of the breakdown, although all patients were impaired at recognizing noncanonical views of objects that they readily recognized when shown from a conventional viewpoint. However, performance dissociation within patients and double dissociation between patients suggest that these associated deficits are not necessary concomitants of prosopagnosia.
Studies of brain-damaged patients have revealed the existence of a selective impairment of face processing, prosopagnosia, resulting from lesions at different loci in the occipital and temporal lobes. The results of such studies have led to the identification of several cortical areas underlying the processing of faces, but it remains unclear what functional aspects of face processing are served by these areas and whether they are uniquely devoted to the processing of faces. The present study addresses these questions in a positron emission tomography (PET) study of regional cerebral blood flow in normal adults, using the 15 oxygen water bolus technique. The subjects participated in six tasks (with gratings, faces and objects), and the resulting level of cerebral activation was mapped on images of the subjects' cerebral structures obtained through magnetic resonance and was compared between tasks using the subtraction method. Compared with a fixation condition, regional cerebral blood flow (rCBF) changes were found in the striate and extrastriate cortex when subjects had to decide on the orientation of sine-wave gratings. A face-gender categorization resulted in activation changes in the right extrastriate cortex, and a face-identity condition produced additional activation of the fusiform gyrus and anterior temporal cortex of both hemispheres, and of the right parahippocampal gyrus and adjacent areas. Cerebral activation during an object-recognition task occurred essentially in the left occipito-temporal cortex and did not involve the right hemisphere regions specifically activated during the face-identity task. The results provide the first empirical evidence from normal subjects regarding the crucial role of the ventro-medial region of the right hemisphere in face recognition, and they offer new information about the dissociation between face and object processing.
The term developmental prosopagnosia refers to an impairment in the recognition of familiar faces which has been present from birth in the absence of neurological disease or birth complications. The first reported study was by McConachie (1976, Cortex, 12: 76-82) and we report here a fifteen year follow-up on this case (AB). Recently developed theoretical models postulating separate processes involved in face perception and recognition were used to guide the exploration of her functional deficit. Our investigations with AB showed that basic visuo-sensory functions (acuity, contrast sensitivity, colour, etc.) were largely intact. General face perception (e.g. distinguishing between a face and a "nonface") was relatively well preserved. Recognition of familiar faces was severely impaired and she also showed problems with other face processing tasks (e.g. analysis of facial expression) and in object recognition. In object recognition she made errors based on visual similarity, and she had problems identifying exemplars from categories with many visually similar items. In addition, she was very poor at identifying objects or silhouettes from an unusual viewpoint. We conclude that AB has always been poor at constructing an effective internal representation sufficient to permit recognition of items which are visually difficult to discriminate. Therefore, she may not have been able to acquire useable stored representations either, because this deficit has been present since birth. This functional account was supported by subsequent studies which demonstrated a complete absence of covert face recognition.
The first reported case of prosopagnosia in a right hemispherectomized woman, B.M., whose intellectual and cognitive functions were otherwise normal or only slightly impaired, is presented. She was totally unable to identify, and to experience a sense of familiarity with, faces of persons she knew, but she could evoke semantic information about them and retrieve their names from visual contextual cues. She was unaware that she was lacking face-recognition skills and that faces alone could be used to access the identity of individuals. The functional nature of her deficit was investigated through sensory, perceptual, memory, and learning tasks to determine the level at which her prosopagnosic disturbance occurred. She was defective at resolving low spatial-frequency information, but this was insufficient to explain the selectivity of her impairment. She was able to carry out cognitive operations specific to faces as long as facial identity did not have to be ascertained, and she performed as well as control subjects at deriving information about the gender, age, and emotion of faces. She was impaired at matching different views of the same faces, and multidimensional scaling analysis of dissimilarity judgements between faces indicated an inability to combine the component features into a configurational facial representation that would uniquely define each face. In contrast to recently reported cases of prosopagnosia, B.M. showed no sign of covert recognition of known faces in a learning task, and there was no indication that she could, even for a few seconds, store a faithful facial representation. The occurrence of prosopagnosia in this hemispherectomized patient confirms that this deficit can emerge without damage to the left hemisphere, and her unawareness of her deficit, which had remained unnoticed for several years, raises the possibility that other hemispherectomized patients may be prosopagnosic. The pattern of cognitive impairments displayed by B.M. indicates a perceptual basis to her disturbance and is discussed in relation to other cases of prosopagnosia.
K.D. has been unable to recognize people's faces since sustaining cerebral injury in infancy. Investigation of this disorder carried out when K.D. was aged 8 to 11 years showed that although her basic visual abilities were impaired, they were no poorer than those of other children who recognized faces without difficulty. K.D. had learned to read, but had not regained ability to recognize people's faces; instead she relied primarily on voices as a cue to person recognition. There was no evidence of any degree of overt or covert recognition of familiar faces, and K.D. also experienced problems in visual object recognition. She could, however, classify a visual input as a face, was able to perceive and imitate facial expressions, and was able to perform face matching tasks to an extent limited by her use of a feature by feature matching strategy. It is suggested that K.D.'s impairment affected higher order perceptual abilities, and is in a number of respects comparable to the impairments found in adult prosopagnosic patients.
It has been postulated that for prosopagnosia to occur, bilateral lesions of the central visual system are usually necessary. All but 1 of the 10 previously documented cases that came to autopsy showed this pattern. However, the long survival period after the onset of prosopagnosia in most of these patients limits the value of the autopsy findings for clinicopathological correlation. A patient is presented who died 10 days after she had developed prosopagnosia, topographagnosia and an agnosia for real objects seen from noncanonical views. These clinical symptoms corresponded directly to the autopsy finding of a recent large occipitotemporal ischaemic infarct in the territory of the right posterior cerebral artery. An additional right frontal infarct and a cortical microinfarct in a deep left lateral parieto-occipital sulcus were both old lesions and had passed unnoticed clinically. This first report of a direct clinicopathological correlation between a fresh right posterior lesion and prosopagnosia demonstrates that bilateral involvement of the visual system is not a prerequisite for prosopagnosia.
We conducted a series of experiments to assess the ability to recognize the meaning of facial expressions, gender, and age in four patients with severe impairments of the recognition of facial identity. In three patients the recognition of face identity could be dissociated from that of facial expression, age, and gender. In one, all forms of face recognition were impaired. Thus, a given lesion may preclude one type of recognition but not another. We conclude that (1) the cognitive demands posed by different forms of recognition are met at different processing levels, and (2) different levels depend on different neural substrates.
The visuo-perceptual abilities of three cases with unilateral lesions of the right hemisphere selected on the basis of the co-occurrence of impaired performance on a test of visual object perception and normal performance on a test of shape discrimination are reported. Their performance was also impaired on other tests of visual object recognition in which the perceptual difficulty of the task was manipulated by obscuring the salient features of the representation. At the same time it was found that on a variety of other tests of visual-sensory processing their performance was entirely normal, as was their performance on tests of visual semantic knowledge, tests that were failed by a patient with a visual associative agnosia. It is argued that this syndrome has all the hallmarks of an apperceptive agnosia, a failure of perceptual categorisation in which the physical identity of the object is specified. The two discontinuities between visual-sensory processing, perceptual categorisation and visual-semantic processing are discussed in terms of a 2 categorical stage model of object recognition.
A single case study of a patient with visual agnosia is presented. The patient had a marked impairment in visual object recognition along with good tactile object identification and a preserved ability to copy. Detailed investigations demonstrated impaired perceptual processes, with the patient's identification strongly affected by duration of stimulus exposure and by using overlapping figures. However, his stored knowledge of objects was shown to be intact. The results demonstrate that agnosia may be determined by a specific deficit in integrating form information; and that the input description for visual object recognition, disrupted in this patient, is functionally separate from stored object descriptions, which are intact. The implications of the results for understanding visual agnosia and for theories of normal visual object recognition are discussed.
We investigated the neuropsychological correlates of left posterior cerebral artery (LPCA) infarcts with a quantitative systematic approach and found a pattern of impairment extending well beyond the classical syndrome of alexia without agraphia. Sixteen consecutive patients with CT scan evidence of an infarct confined to the territory of LPCA were given a battery of tests assessing the following abilities. (1) Reading and writing; (2) naming and pointing to colours; (3) naming the same 30 objects on visual (objects and coloured photographs), tactile and verbal presentation; and (4) verbal memory. These tests were administered to large control samples and the performance of LPCA patients was considered pathological if it fell below the score of the last or second to last control patient.
Seventy five per cent of PCA patients had alexia without agraphia. Although a lesion of the CT scal slice where the pineal is represented appeared to be crucially associated with alexia, the severity of the disorder increased when contiguous upper or lower slices were also involved. Not only colour anomia, but also object and especially photograph anomia could almost always be shown in alexies and were highly correlated with the degree of the reading impairment. The naming deficit was also present when items were presented in the tactile and verbal modality, in spite of the integrity of the oral language areas. Every right-handed patient, alexic as well as nonalexic, was impaired on at least two of the three verbal memory tests and most on all of them. The findings are discussed in terms of the anatomofunctional mechanisms subserving verbal memory and the transmission of visual information to the speech areas.
The aim of this paper is to develop a theoretical model and a set of terms for understanding and discussing how we recognize familiar faces, and the relationship between recognition and other aspects of face processing. It is suggested that there are seven distinct types of information that we derive from seen faces; these are labelled pictorial, structural, visually derived semantic, identity‐specific semantic, name, expression and facial speech codes. A functional model is proposed in which structural encoding processes provide descriptions suitable for the analysis of facial speech, for analysis of expression and for face recognition units. Recognition of familiar faces involves a match between the products of structural encoding and previously stored structural codes describing the appearance of familiar faces, held in face recognition units. Identity‐specific semantic codes are then accessed from person identity nodes, and subsequently name codes are retrieved. It is also proposed that the cognitive system plays an active role in deciding whether or not the initial match is sufficiently close to indicate true recognition or merely a ‘resemblance’; several factors are seen as influencing such decisions.
This functional model is used to draw together data from diverse sources including laboratory experiments, studies of everyday errors, and studies of patients with different types of cerebral injury. It is also used to clarify similarities and differences between processes responsible for object, word and face recognition.
A case (J.R.) of associative visual agnosia (loss of meaning for visually presented objects without any serious sensory disorder) is reported. When asked to match a given name to sample the patient made both visual and semantic errors. Whereas semantic errors were reduced by priming, visual errors were intractable. It is argued that there is a disorder at the stage of pre-semantic abstraction, similar to that postulated elsewhere for some cases of deep dyslexia, contributing to the agnosia.
Visual recognition of pictorial material was investigated in a group of 74 patients with localised cerebral lesions. Four tasks of visual perception, figure/ ground, fragmented drawings, enlarged drawings, and photographs of objects from an unconventional view, were administered. An unimpaired performance of the right posterior group on the figure/ground task contrasted with a marked deficit on the unconventional view objects task. It was demonstrated that there is a favoured view for efficiency of object recognition. The findings provide evidence that gestalt formation is intact whereas perceptual classification is impaired in patients with right posterior lesions. The implications of this interpretation of the data for theories of object recognition are discussed.
Knowledge of the neuropsychological mechanisms underlying facial recognition has come from both experimental study of normal Ss and clinical investigation of patients who show defects in facial perception or memory. A basic difference exists between the identification of the faces of familiar persons and the discrimination of unfamiliar faces. Defects in these 2 forms of facial recognition have different anatomical correlates, and a patient with brain disease may show one type of impairment and not the other. The right hemisphere appears to play a primary role in mediating both forms of facial recognition. However, there is evidence to indicate that left-hemisphere mechanisms are also involved in facial perception and memory and that the relative contribution of each hemisphere to the process may vary among individuals. (50 ref) (PsycINFO Database Record (c) 2012 APA, all rights reserved)
A brain-damaged patient with prosopagnosia and a group of age- and education-matched control subjects evaluated curved and straight versions of different sorts of stimuli in different tasks. The patient consistently required more time to encode curved than straight stimuli, relative to the control subjects. Specifically, he had a deficit when he compared curved lines that were simultaneously visible, when he compared curved lines with those previously seen, when he examined a curved shape to determine whether an X was on or off the shape, and when he read curved script. He also made more errors when he named pictures of curved objects. Implications of these findings for some types of clinical disorders and for the role of "end-stopped" cells in visual cortex are discussed.
A man with an infarction of his inferior temporal and occipital association cortex bilaterally, which spared primary visual cortex, had impaired visual recognition of objects, faces, colors, words, and gestures. Analysis of visual function indicated that the recognition failures resulted from an agnosia, rather than elemental visual impairment. Whereas his impairment of gesture recognition appeared to be related to an associative agnosia, his inability to recognize objects was related to an apperceptive agnosia. There may be four subtypes of apperceptive agnosia: one where the internal object representations or structural descriptions are impaired, another where an adequate percept cannot be derived, a third where the internal referent and percept are dissociated, and a fourth where both levels are impaired. Our patient demonstrated a failure to relate individual elements to the whole, a failure to integrate multiple elements, and a reliance on global perception. He had normal object imagery. These results suggest that, whereas internal representations were intact, he was unable to form adequate perceptual representations.
We describe a young woman, J.R., who sustained a very severe head injury in 1981 at the age of 17 years. She was assessed in 1982 and found to have visual agnosia. Since then J.R. has been assessed on several occasions over a period of ten years. Her agnosia for real objects has resolved and she has improved on the identification of other classes of stimuli. However, she still has some problems with the identification of line drawings, photographs and model animals. Her drawing from memory remains particularly poor and she has difficulty with visual imagery. We consider her residual deficits in the light of Farah's (1990) theoretical framework; this proposes that associative agnosia could be due to a disconnection syndrome, a loss of stored visual representations or to the loss of knowledge of how to perceive objects. J.R.'s residual impairments appear to be mainly due to a loss of access to visual representations in the absence of visual input.
We report data contrasting the processing of facial identity from static photographs, and facial expression from static and moving images, in two patients with face processing impairments. One patient is markedly impaired at perceiving facial identity and he is poor at discriminating facial expression and gender from static photographs of faces. In contrast, he performs normally when required to make judgements of facial expression and gender to faces depicted by sets of moving light dots. The second patient is relatively good at perceiving facial identity, but poor at judging emotional expression from both static and moving faces. The data are consistent with the existence of separate processes for encoding face identity and facial expression, and, furthermore, indicate the separate encoding of expression from moving and static images.