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We illustrate a case of post-traumatic recurrent transient prosopagnosia in a paediatric patient with a right posterior inferior temporal gyrus haemorrhage seen on imaging and interictal electroencephalogram abnormalities in the right posterior quadrant. Face recognition area mapping with magnetoencephalography (MEG) and functional MRI (fMRI) was performed to clarify the relationship between the lesion and his prosopagnosia, which showed activation of the right fusiform gyrus that colocalised with the lesion. Lesions adjacent to the right fusiform gyrus can result in seizures presenting as transient prosopagnosia. MEG and fMRI can help to attribute this unique semiology to the lesion., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Changes in health care and disease epidemiology have shifted the attention of neuropsychologists and cognitive neuroscientists from vascular lesions to degenerative diseases or other bilateral brain lesions. This displacement of attention from vascular patients to patients with degenerative brain diseases allowed the discovery of hitherto unexplored and unheralded aspects of the neural substrates of human cognition. Three aspects of research on the anterior parts of the temporal lobes (ATLs) are the focus of the present review. The first aspect is category-specific semantic disorders, including current accounts of categorical brain organization, the anatomical substrate of different categories (stressing the role of the ATLs with respect to the biological categories), and the "sources of knowledge" that contribute to construction of those categories. The second aspect is the role of the ATLs in conceptual knowledge, including the "hub-and-spokes" model of semantic representation and semantic control. The third aspect is the role of the right ATL in recognition of familiar people, including the distinction made between associative prosopagnosia and multimodal disorders of person recognition. Consistencies and inconsistencies of results obtained across these different domains are discussed, and the clinical implications of these findings are considered.

Background: Face recognition is a complex function sustained by a distributed large-scale neural network, with a core system involving the ventral occipitotemporal cortex, the inferior longitudinal fasciculus (ILF), and the splenial commissural fibers. This circuit seems to be bilaterally organized, but with a right hemispheric dominance. According to this anatomic functional model, prosopagnosia is usually, but not exclusively, generated by a damage of the right part of this brain network., Case Description: This report describes an original case of a multicentric diffuse low-grade glioma, with a right occipitotemporal tumor and a left anterior temporoinsular tumor. Awake surgery for the right occipitotemporal lesion, involving fusiform and inferior occipital gyri and ILF, was achieved in a first step without causing any neurologic deficit. A subsequent resection of the left anterior temporoinsular lesion, with removal of the anterior left ILF, was achieved 1 year later. Surprisingly, the patient experienced a strong and permanent prosopagnosia after this second surgery., Conclusions: The authors investigate the possible causes resulting in this prosopagnosia. Specifically, they suggest a decompensation within a reorganized neural network after the first operation, because of a disconnection syndrome induced by a bilateral surgical damage of the ventral occipitotemporal structural connectivity. These original data can be useful for neurosurgeons, especially when achieving resection for multicentric tumors involving both ventral streams, to inform patients before surgery about the possible risk of face recognition deficit, and to adapt the cognitive tasks intraoperatively during awake procedure., (Copyright © 2016 Elsevier Inc. All rights reserved.)

We report a patient with ventral simultanagnosia, prosopagnosia for "unfamiliar faces" (dorsal prosopagnosia), spatial agraphia, and constructional disorder, particularly on the left spatial side, due to a lesion in the right posterior superior and middle temporal gyri and angular gyrus. The patient showed impairment of fundamental visual and visuospatial recognition, such as in object size, configuration, and horizontal point location, which probably underlay the mechanism of simultanagnosia and prosopagnosia. This case also suggests that the coexistence of simultanagnosia and prosopagnosia results from a right hemispheric insult, and damage to the temporoparietal area interrupts the incorporation of spatial information into object recognition. This disconnection of information flow, together with impaired object recognition per se, may impair the parallel processing of multiple objects, leading to object-by-object or part-by-part recognition.

Although face detection likely played an essential adaptive role in our evolutionary past and in contemporary social interactions, there have been few rigorous studies investigating its neural correlates. MJH, a prosopagnosic with bilateral lesions to the ventral temporal-occipital cortices encompassing the posterior face areas (fusiform and occipital face areas), expresses no subjective difficulty in face detection, suggesting that these posterior face areas do not mediate face detection exclusively. Despite his normal contrast sensitivity and visual acuity in foveal vision, the present study nevertheless revealed significant face detection deficits in MJH. Compared with controls, MJH showed a lower tolerance to noise in the phase spectrum for faces (vs. cars), reflected in his higher detection threshold for faces. MJH's lesions in bilateral occipito-temporal cortices thus appear to have produced a deficit not only in face individuation, but also in face detection.

Face recognition plays a key role in initiation and coordination of social interactions. Face is a source of many valuable information about sex, age, health and emotional state of another person. Face perception is also considered as the best developed component of human visual perception. The term prosopagnosia means the inability to recognize faces previously known, while still remaining the ability to correctly identifying other objects from the surroundings. This dysfunction can affect face recognition of famous people, family members or even own face. Affected person correctly names the object (knows, that he deals with the "face"), properly assess the emotional expression of a second person, adequately identifies the sex and age of observed person, but cannot determine who the person is. The aim of this study is to present a case of a patient complaining about emotional dysfunctions and presence of prosopagnosia symptoms following neurosurgery for arteriovenous malformation in the right temporal lobe.

Whether a single perceptual process or separate and possibly independent processes support facial identity and expression recognition is unclear. We used a morphed-face discrimination test to examine sensitivity to facial expression and identity information in patients with occipital or temporal lobe damage, and structural and functional MRI to correlate behavioral deficits with damage to the core regions of the face-processing network. We found selective impairments of identity perception in two patients with right inferotemporal lesions and two prosopagnosic patients with damage limited to the anterior temporal lobes. Of these four patients one exhibited damage to the right fusiform and occipital face areas, while the remaining three showed sparing of these regions. Thus impaired identity perception can occur with damage not only to the fusiform and occipital face areas, but also to other medial occipitotemporal structures that likely form part of a face recognition network. Impaired expression perception was seen in the fifth patient with damage affecting the face-related portion of the posterior superior temporal sulcus. This subject also had difficulty in discriminating identity when irrelevant variations in expression needed to be discounted. These neuropsychological and neuroimaging data provide evidence to complement models which address the separation of expression and identity perception within the face-processing network., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Gaze-contingency is a method traditionally used to investigate the perceptual span in reading by selectively revealing/masking a portion of the visual field in real time. Introducing this approach in face perception research showed that the performance pattern of a brain-damaged patient with acquired prosopagnosia (PS) in a face matching task was reversed, as compared to normal observers: the patient showed almost no further decrease of performance when only one facial part (eye, mouth, nose, etc.) was available at a time (foveal window condition, forcing part-based analysis), but a very large impairment when the fixated part was selectively masked (mask condition, promoting holistic perception) (Van Belle, De Graef, Verfaillie, Busigny, & Rossion, 2010a; Van Belle, De Graef, Verfaillie, Rossion, & Lefèvre, 2010b). Here we tested the same manipulation in a recently reported case of pure prosopagnosia (GG) with unilateral right hemisphere damage (Busigny, Joubert, Felician, Ceccaldi, & Rossion, 2010). Contrary to normal observers, GG was also significantly more impaired with a mask than with a window, demonstrating impairment with holistic face perception. Together with our previous study, these observations support a generalized account of acquired prosopagnosia as a critical impairment of holistic (individual) face perception, implying that this function is a key element of normal human face recognition. Furthermore, the similar behavioral pattern of the two patients despite different lesion localizations supports a distributed network view of the neural face processing structures, suggesting that the key function of human face processing, namely holistic perception of individual faces, requires the activity of several brain areas of the right hemisphere and their mutual connectivity., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

The aim of this study consisted of using neuropsychological data obtained in two patients (VL and StG) showing a selective atrophy of the anterior parts of the right (VL) and left (StG) temporal lobes to check current cognitive models of familiar people identification. According to these models, information coming from modality-specific "face", "voice" and "name" recognition units converge into "Person Identity Nodes" (PINs) where familiarity feelings are generated and which provide a modality-free gateway to a unitary semantic system, where information about people is stored in an amodal format. Data obtained in patient VL (and to a lesser degree in StG) were at variance with this model because VL showed: (1) a very impaired familiarity for faces, contrasting with a spared familiarity for names, indicating that familiarity judgments are generated at the level of modality-specific recognition units and not at a supramodal PIN; (2) a prevalent impairment of person-specific information available from faces rather than from names also for people that (being recognized as familiar from both their face and their name) should be normally represented at the PINs level. This last finding is at variance with the hypothesis assuming that the PINs may provide a modality-free gateway to a unitary semantic system, where information about people is stored in an amodal format.

A 58-year-old man with metastatic brain tumor in his right occipito-temporal region was operated, using craniectomy. He had no neurological symptoms preoperatively. The tumor was 2.5 cm in diameter with minor perifocal edema. Two days after total removal of the tumor, typical prosopagnosia appeared, in which he could not recognize his wife's face as well as faces of medical stuff. He could see them as a whole, and described them undistinguishably from each other. He used voices, movements and clothing to recognize a familiar person. His recognition and semantic knowledge of people were found to be intact and he could recognize certain parts of the face (e.g. the nose or the mouth). He could clearly see other parts of the body, the environment and other objects, in color. The prosopagnostic condition lasted for a few weeks and slowly disappeared. Prosopagnosia caused by surgical procedure has been rarely reported. Although postoperative prosopagnosia is likely to be transient, it should be recognized as a complication in occipito-temporal cortex.

Acquired prosopagnosia is usually associated with bilateral or right-sided lesions of the occipital or temporal lobes. In rare cases of prosopagnosia after left-sided lesions in left-handed subjects, it is attributed to a reversed hemispheric specialization for face processing. This study examines the face-processing functions of a left-handed prosopagnosic patient with a left-sided lesion affecting the region of the occipital face area and possibly the fusiform face area, to contrast his deficits with those of prosopagnosic patients with right-hemispheric lesions. Similar to those patients, he has a moderately severe reduction in familiarity judgments, is impaired in processing face configuration, and shares with some of those patients a greater failure to process eye than mouth information, indicating an altered pattern of facial saliency. He has a mild reduction in the identification of exemplars of non-face objects. Unlike those patients, he has better residual familiarity on a two-alternative forced-choice task and can processing facial configuration if given more time, indicating a reduction in efficiency rather than a severe limitation. He has more difficulty accessing semantic-biographic information from names. He has trouble with facial feature imagery but not imagery for global face shape. Thus this subject's deficits represent a combination of impaired familiarity and configuration processing (normally right-sided functions in right-handed subjects), and impaired feature processing and access to semantic-biographic information (normally left-sided functions). His prosopagnosia likely reflects partially anomalous rather than reversed lateralization of hemispheric perceptual functions.

Two patients with large lesions of the medial temporal lobe were given four tests of semantic knowledge that could only have been acquired after the onset of their amnesia. In contrast to previous studies of postmorbid semantic learning, correct answers could be based on a simple, nonspecific sense of familiarity about single words, faces, or objects. According to recent computational models (for example, Norman and O'Reilly (2003) Psychol Rev 110:611-646), this characteristic should be optimal for detecting the kind of semantic learning that might be supported directly by the neocortex. Both patients exhibited some capacity for new learning, albeit at a level substantially below control performances. Notably, the correct answers appeared to reflect declarative memory. It was not the case that the correct answers simply popped out in some automatic way in the absence of any additional knowledge about the items. Rather, the few correct choices made by the patients tended to be accompanied by additional information about the chosen items, and the available knowledge appeared to be similar qualitatively to the kind of factual knowledge that healthy individuals gradually acquire over the years. The results are consistent with the idea that neocortical structures outside the medial temporal lobe are able to support some semantic learning, albeit to a very limited extent. Alternatively, the small amount of learning detected in the present study could depend on tissue within the posterior medial temporal lobe that remains intact in both patients., ((c) 2008 Wiley-Liss, Inc.)

A 67-year-old, right-handed woman became unable to recognize familiar faces following a period of nonconvulsive status epilepticus. Neuropsychological assessment revealed a relatively selective impairment of familiar face recognition in the absence of low-level visual deficits or widespread cognitive impairment. MRI scanning demonstrated an isolated lesion, probably a venous angioma, involving the left fusiform gyrus, mirror-symmetrical to the site typically linked to prosopagnosia. Potential explanations for the patient's prosopagnosia include seizure-related damage to a left fusiform region required for fully competent face recognition and damage to the contralateral fusiform gyrus via interhemispheric connections. Focal neuropsychological deficits in patients with refractory partial epilepsy who develop nonconvulsive status epilepticus may be underdiagnosed.

Introduction: The association of prosopagnosia and false recognition of faces is unusual and contributes to our understanding of the generation of facial familiarity., Method: A 67-year-old man with a left prefrontal traumatic lesion, developed a temporal variety of fronto-temporal dementia (semantic dementia) with amyotrophic lateral sclerosis. Cerebral imagery demonstrated a bilateral, temporal anterior atrophy predominating in the right hemisphere. The main cognitive signs consisted in severe difficulties to recognize faces of familiar people (prosopagnosia), associated with systematic false recognition of unfamiliar people., Result: Neuropsychological testing indicated that the prosopagnosia probably resulted from the association of an associative/mnemonic mechanism (inability to activate the Face Recognition Units (FRU) from the visual input) and a semantic mechanism (degradation of semantic/biographical information or deconnexion between FRU and this information). At the early stage of the disease, the patient could activate residual semantic information about individuals from their names, but after a 4-year course, he failed to do so. This worsening could be attributed to the extension of the degenerative lesions to the left temporal lobe. Familiar and unfamiliar faces triggered a marked feeling of knowing. False recognition concerned all the unfamiliar faces, and the patient claimed spontaneously that they corresponded to actors, but he could not provide any additional information about their specific identities. The coexistence of prosopagnosia and false recognition suggests the existence of different interconnected systems processing face recognition, one intended to identification of individuals, and the other producing the sense of familiarity. Dysfunctions at different stages of one or the other of these two processes could result in distortions in the feeling of knowing., Conclusion: From this case and others reported in literature, we propose to complete the classical model of face processing by adding a pathway linked to limbic system and frontal structures. This later pathway could normally emit signals for familiarity, essentially autonomic, in response to the familiar faces. These signals, primitively unconscious, secondly reach consciousness and are then integrated by a central supervisor system which evaluates and verifies identity-specific biographical information in order to make a decision about the sense of familiarity.

Background: Patients with prosopagnosia from occipitotemporal lesions have impaired perception of the configuration of facial features. This may be an example of impaired "within-object" spatial coding, which others propose to be distinct from "between-object" spatial coding., Objective: To determine whether the prosopagnosic deficit in perceiving spatial configuration was specific to within-face and not between-face spatial coding and whether the deficit was face-selective or extended to objects other than faces., Methods: Six prosopagnosic patients were tested using an oddity paradigm in which they detected which of three simultaneously seen stimuli was an altered target. In the "within-face" task, the target face had altered interocular distance or mouth position. In the "between-face" task, the target face was located farther away from the other two. In the "within-object" task, the stimulus was a two-dot pattern, and the target pattern had altered interdot distance., Results: Spatial judgments were impaired within faces for all six patients and within the two-dot pattern for five of six patients. However, all six had normal between-face spatial perception., Conclusions: Impaired perception of spatial relations in prosopagnosia is selective to the spatial structure within individual objects and spares the perception of spatial location of objects. It is not specific to faces. It reveals a process involved in analyzing object structure, consistent with the patients' deficits in recognizing facial identity, and illustrates a different type of "visuospatial" defect.

A 23-year-old right-handed woman developed isolated transient prosopagnosia following surgical resection of a right posterior temporal seizure focus. At 18 years of age she had developed secondarily generalized tonic-clonic seizures. Preoperative neuropsychological evaluation was normal, and neurological examination revealed only a left superior quadrant achromatopsia. MRI revealed a circumscribed lesion in the right inferolateral temporo-occipital junction. Following surgery she was agitated for 36 hours, and afterward, when her attention and orientation improved, she was unable to recognize familiar faces. She could, however, recognize familiar voices. Her prosopagnosia resolved over the next 6-7 days. This case demonstrates that isolated prosopagnosia can occur in patients with lesions restricted to the right inferior posterior temporal-anterior occipital region. The temporary nature of the prosopagnosia may result from postsurgical tissue injury, including focal cerebral edema, with compensation by ipsilateral or contralateral areas.

We report the case of a patient (C.O.) who showed a selective defect in the recognition of familiar people, with very mild disease progression during a period of 30 months resulting from focal atrophy of the right temporal lobe. On formal neuropsychological testing, C.O. obtained high scores on tests of general intelligence, episodic memory, language, executive functions, selective attention, visual recognition and visual-spatial abilities. On more specific tasks of familiar and unfamiliar face recognition, C.O. scored above the controls' means on perceptual tests, but obtained highly pathological results on amnesic-associative tests. His disorder of recognition of familiar people was not due to loss of person-specific information, since he obtained highly abnormal naming scores when presented with photographs of famous people but borderline or mildly abnormal scores in a tasks in which he had to name celebrities from verbal definitions. On the other hand, C.O.'s recognition disorder could not be considered to be a form of 'associative prosopagnosia' since a similar defect was observed when he was requested to access information about famous persons through their voice rather than their face. Two alternative interpretations are advanced to explain C.O.'s inability to access his relatively spared person-specific knowledge not only through the person's face but also through the person's voice. The first hypothesis is that, before accessing the person-specific information, unimodal recognition channels must converge into a multimodal, non-verbal person-recognition system and that the right anterior temporal cortices play a crucial role in this integrative activity. The second hypothesis is that the face-recognition units have privileged access to person-specific semantic knowledge and that other recognition subsystems require coactivation of the face-recognition units in order to access person-specific semantic information.

Right hemisphere dominance in face processing is well established and unilateral right inferior temporo-occipital damage can result in prosopagnosia. Here, we describe a 21-year-old right-handed woman with acute impairment in face recognition that selectively concerned unfamiliar faces, following a focal left lateral temporo-occipital venous infarct. She was severely impaired in discerning that unknown people seen in everyday life were unfamiliar, although she had no difficulty recognizing familiar people. Thus, she had no prosopagnosia, but abnormal 'hyperfamiliarity' for unknown faces. Her difficulty was not accompanied by delusions or deficits in discrimination, identification or memory for faces. Standard neuropsychological testing showed that her recognition of familiar faces was entirely normal. By contrast, her sense of personally knowing faces was severely impaired when unknown faces evoked weak signals of familiarity based on spurious cues, to the extent that she would misattribute fame to faces that were unknown but to which she had been incidentally exposed on a prior occasion. Priming experiments also revealed that, unlike normal subjects, she made familiarity judgements without accessing semantic identity representations. Moreover, in face recognition tests, she generally showed bias in that she relied more on right-hemisphere strategies to identify global traits and less on left-hemisphere processes compared with healthy subjects. This case provides novel evidence for a differential contribution of the two hemispheres to face recognition. Hyperfamiliarity for unknown faces might arise from an imbalance between reciprocal hemispheric functions in face recognition, with relative hypoactivation of left hemisphere processes but hyperactivation of right-hemisphere processes for retrieving stored associations about people, linking seen faces to representations of affective and personal relevance. Hence, abnormal bias in attributing some personal meaning to unknown faces could be evoked by spurious signals of familiarity based on irrelevant affective associations in the right hemisphere.

Brain imaging research has identified at least two regions in human extrastriate cortex responding selectively to faces. One of these is located in the mid-fusiform gyrus (FFA), the other in the inferior occipital gyrus (IOG). We studied activation of these areas using fMRI in three individuals with severely impaired face recognition (one pure developmental and two childhood prosopagnosics). None of the subjects showed the normal pattern of higher fMRI activity to faces than to objects in the FFA and IOG or elsewhere. Moreover, in two of the patients, faces and objects produced similar activations in the regions corresponding to where the FFA and IOG are found in normal subjects. Our study casts light on the important role of FFA and IOG in the network of areas involved in face recognition, and indicates limits of brain plasticity., (Copyright 2002 Wiley-Liss, Inc.)