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Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

A 56-year-old, right-handed man with no known past medical history presented with sudden onset of inability to recognize familiar individuals in person, including his wife and his mother. He also couldn't recognize himself in the mirror. There was no weakness, numbness, visual disturbances, or speech difficulty. Face recognition test, using Warrington Recognition Memory Test (1), showed the presence of complete prosopagnosia. The rest of the neurological and cranial nerves examinations were normal. Magnetic resonance imaging (MRI) of the brain showed restricted diffusion at the right temporal and occipital lobes (the fusiform gyrus) [Figure 1]. Magnetic resonance angiogram (MRA) of the brain was unremarkable. The 24-hours Holter monitoring showed paroxysmal atrial fibrillation. The transthoracic echocardiogram and carotid doppler ultrasound scan were normal. He was then treated with rivaroxaban 20mg daily for secondary stroke prevention in non-valvular atrial fibrillation. Face recognition skill training was started in the ward, which includes compensatory strategies to achieve person recognition by circumventing the face processing impairment, and remediation to enhance mnemonic function for face recognition. His prosopagnosia resolved completely after one week. Prosopagnosia, also known as face blindness, is an impairment in recognizing faces. The core defects are the loss of familiarity with previously known faces and the inability to recognize new faces. Patients with prosopagnosia may present with poor recognition of familiar individuals in person or in the photograph, confusion with plotlines in movies or plays with numerous characters, and difficulty distinguishing individuals wearing a uniform or similar clothing. Stroke is the most common cause of acquired prosopagnosia (2). Other less common aetiologies include traumatic brain injury, carbon monoxide poisoning, temporal lobectomy, and encephalitis. Literature has shown that areas involved in acquired prosopagnosia are the right fusiform gyrus or anterior temporal cortex, or both (3). The fusiform gyrus is part of the lateral temporal lobe and occipital lobe in 'Brodmann area 37' (4). The fusiform gyrus is considered a key structure for functionally specialized computations of high-level vision such as face perception, object recognition, and reading. Individuals with fusiform lesions are more likely to have apperceptive prosopagnosia, while those with anterior temporal lesions have an amnestic variant (5). In summary, prosopagnosia can be the sole presentation for the right fusiform gyrus stroke. It is important to recognize prosopagnosia for early stroke diagnosis and avoid misdiagnosing it as a psychiatric or ocular disorder. Keywords: prosopagnosia, fusiform gyrus, stroke.

Prosopamnesia is a face-selective memory disorder in which face learning is impaired, while face-perception disorder (prosopagnosia) and memory disorders for stimuli other than faces are not present. To date, only two cases of prosopamnesia have been reported in adults - one congenital and one secondary to brain damage. This article reports a case of a 68-year-old woman complaining difficulties recognizing persons she had got to know recently. Neuropsychological examination revealed face-specific anterograde amnesia in the absence of prosopagnosia and other memory impairments. Brain MRI did not present any focal abnormality; PET-scan revealed hypoactivation mostly in the frontotemporal area bilaterally. This patient represents the first case of late-onset primary prosopamnesia.

Parosmia, defined as the distorted perception of an odor stimulus, has been reported to be associated with head trauma, upper respiratory tract infections, sinonasal diseases, and toxin/drug consumption. To date, little is known about parosmia in right-lateralized semantic variant primary progressive aphasia. A 60-year-old right-handed man presented with a 2-year history of parosmia and prosopagnosia. Brain magnetic resonance imaging demonstrated severe atrophy of the right anterior and mesial temporal lobe, particularly in the fusiform cortex and the regions known as the primary olfactory cortex. 18F-fluorodeoxyglucose position emission tomography showed asymmetric hypometabolism of the bilateral temporal lobes (right > left). We clinically diagnosed him with right-lateralized semantic variant primary progressive aphasia. As the right hemisphere is known to be more involved in the processing of pleasant odors than the left hemisphere, we speculate that the unique manifestation of parosmia observed in this patient might be associated with the lateralization of the olfactory system., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Proposapnosia is a type of visual agnosia characterized by the inability to recognize people's faces. There are basically two variants, apperceptive and associative. The "Tortoni effect" is a phenomenon described by Bekinschtein et al a few years ago in waiters from Buenos Aires, who used this tool to remember the orders of each member of a table. We present a case of prosopagnosia associated with bilateral temporo-occipital injury secondary to head trauma, initially manifested by the lack of face recognition with the use of an associative strategy similar to that described in the "Tortoni effect" as compensation, in a 62-year-old female who suffered a severe head injury. A few months after this event, the patient had difficulty in recognizing familiar people, a fact evidenced by her relatives when at a restaurant table, they changed their seats, remained silent momentarily, and right after the patient kept naming them by their previous location. The magnetic resonance imaging of the brain revealed blunt sequelae lesions in the bilateral temporo-occipital region. Acquired prosopagnosia due to focal lesions in the temporo-occipital region, generally bilateral and right, and less frequently left, is a rare condition. The strategy used in the "Tortoni effect" was one of the initial manifestations of the condition in our patient. Carrying out an ecological neuropsychological test that considers this strategy could be useful in the screening and early detection of this entity.

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.

Despite its basic and translational importance, the neural circuitry supporting the perception of emotional faces remains incompletely understood. Functional imaging studies and chronic lesion studies indicate distinct roles of the amygdala and insula in recognition of fear and disgust in facial expressions, whereas intracranial encephalography studies, which are not encumbered by variations in human anatomy, indicate a somewhat different role of these structures. In this article, we leveraged lesion-mapping techniques in individuals with acute right hemisphere stroke to investigate lesions associated with impaired recognition of prototypic emotional faces before significant neural reorganization can occur during recovery from stroke. Right hemisphere stroke patients were significantly less accurate than controls on a test of emotional facial recognition for both positive and negative emotions. Patients with right amygdala or anterior insula lesions had significantly lower scores than other right hemisphere stroke patients on recognition of angry and happy faces. Lesion volume within several regions, including the right amygdala and anterior insula, each independently contributed to the error rate in recognition of individual emotions. Results provide additional support for a necessary role of the right amygdala and anterior insula within a network of regions underlying recognition of facial expressions, particularly those that have biological importance or motivational relevance and have implications for clinical practice., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

The present study examined the effects of genetic subtype on social memory in children (7-16 years) with Prader-Willi syndrome (PWS). Visual event-related potentials (ERPs) during a passive viewing task were used to compare incidental memory traces for repeated vs single presentations of previously unfamiliar social (faces) and nonsocial (houses) images in 15 children with the deletion subtype and 13 children with maternal uniparental disomy (mUPD). While all participants perceived faces as different from houses (N170 responses), repeated faces elicited more positive ERP amplitudes ('old/new' effect, 250-500ms) only in children with the deletion subtype. Conversely, the mUPD group demonstrated reduced amplitudes suggestive of habituation to the repeated faces. ERP responses to repeated vs single house images did not differ in either group. The results suggest that faces hold different motivational value for individuals with the deletion vs mUPD subtype of PWS and could contribute to the explanation of subtype differences in the psychiatric symptoms, including autism symptomatology., (© The Author (2017). Published by Oxford University Press.)

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. Here, we studied BN, a 9 y.o. boy who has normal basic-level vision, abnormal (limited) oculomotor pursuit and saccades, and shows developmental object and contour integration deficits but with no indication of prosopagnosia. Although we cannot infer causation from the data presented here, we suggest that normal pursuit and saccades could be critical for the development of contour integration and object perception. While faces and perhaps reading, when fixated upon, take up a small portion of central visual field and require only small eye movements to be properly processed, common objects typically prevail in mid-peripheral visual field and rely on longer-distance voluntary eye movements as saccades to be brought to fixation. While retinal information feeds into early visual cortex in an eccentricity orderly manner, we hypothesize that propagation of non-foveal information to mid and high-order visual cortex critically relies on circuitry involving eye movements. Limited or atypical eye movements, as in the case of BN, may hinder normal information flow to mid-eccentricity biased high-order visual cortex, adversely affecting its development and consequently inducing visual perceptual deficits predominantly for categories associated with these regions., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

While cerebral amyloid angiopathy is a common cause of lobar hemorrhage, rarely it may be associated with an inflammatory response, thought to be incited by amyloid deposits. We report a 73-year-old woman with an extensive cancer history who presented with tumor-like lesions and symptoms of homonymous hemianopia and prosopagnosia. Found to have cerebral amyloid angiopathy-related inflammation proven by brain biopsy, she was treated successfully with immunosuppression.

Despite many studies of acquired prosopagnosia, there have been only a few attempts at its rehabilitation, all in single cases, with a variety of mnemonic or perceptual approaches, and of variable efficacy. In a cohort with acquired prosopagnosia, we evaluated a perceptual learning program that incorporated variations in view and expression, which was aimed at training perceptual stages of face processing with an emphasis on ecological validity. Ten patients undertook an 11-week face training program and an 11-week control task. Training required shape discrimination between morphed facial images, whose similarity was manipulated by a staircase procedure to keep training near a perceptual threshold. Training progressed from blocks of neutral faces in frontal view through increasing variations in view and expression. Whereas the control task did not change perception, training improved perceptual sensitivity for the trained faces and generalized to new untrained expressions and views of those faces. There was also a significant transfer to new faces. Benefits were maintained over a 3-month period. Training efficacy was greater for those with more perceptual deficits at baseline. We conclude that perceptual learning can lead to persistent improvements in face discrimination in acquired prosopagnosia. This reflects both acquisition of new skills that can be applied to new faces as well as a degree of overlearning of the stimulus set at the level of 3-D expression-invariant representations.

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.)

Whipple disease is a rare, chronic multisystem infectious disease. The central nervous system (CNS) is secondarily involved in 43% of patients; 5% of patients have isolated or primary CNS involvement. The most frequent CNS symptoms are cognitive changes. Prosopagnosia is an inability to recognize familiar faces, in a person who does not have vision impairments or cognitive alterations. This relatively rare condition is usually related to vascular, traumatic, degenerative, or infectious lesions. We report a 54-year-old woman who presented subacutely with fever, headache, and seizures that led to a diagnosis of infectious meningoencephalitis. She improved temporarily on broad-spectrum antibiotics, but then developed a chronically evolving cognitive impairment with associative prosopagnosia as the major complaint. She had a history of sporadic abdominal pain and mild sacroiliac arthralgia. After a negative duodenal biopsy, we confirmed primary CNS Whipple disease by polymerase chain reaction and brain biopsy. We treated the patient with ceftriaxone for 15 days and then co-trimoxazole for 2 years. At 8-year follow-up, she had no further impairments, but continuing prosopagnosia. To our knowledge, this is the first description of isolated prosopagnosia in a patient with primary CNS Whipple disease. Because CNS Whipple disease can lead to serious, irreversible lesions if not promptly treated, clinicians must suspect the diagnosis, treat with long-term antibiotics, and follow patients carefully to prevent recurrence.

Developmental prosopagnosia (DP) is defined by severe face recognition difficulties due to the failure to develop the visual mechanisms for processing faces. The two-process theory of face recognition (Morton & Johnson, 1991) implies that DP could result from a failure of an innate face detection system; this failure could prevent an individual from then tuning higher-level processes for face recognition (Johnson, 2005). Work with adults indicates that some individuals with DP have normal face detection whereas others are impaired. However, face detection has not been addressed in children with DP, even though their results may be especially informative because they have had less opportunity to develop strategies that could mask detection deficits. We tested the face detection abilities of seven children with DP. Four were impaired at face detection to some degree (i.e. abnormally slow, or failed to find faces) while the remaining three children had normal face detection. Hence, the cases with impaired detection are consistent with the two-process account suggesting that DP could result from a failure of face detection. However, the cases with normal detection implicate a higher-level origin. The dissociation between normal face detection and impaired identity perception also indicates that these abilities depend on different neurocognitive processes., (© 2015 John Wiley & Sons Ltd.)

Background: Deficits in facial emotion recognition (FER) have been shown to substantially impair several aspects in everyday life of affected individuals (e.g. social functioning). Presently, we aim at assessing differences in emotion recognition performance in three patient groups suffering from mild forms of cognitive impairment compared to healthy controls., Methods: Performance on a concise emotion recognition test battery (VERT-K) of 68 patients with subjective cognitive decline (SCD), 44 non-amnestic (non-aMCI), and 25 amnestic patients (aMCI) with mild cognitive impairment (MCI) was compared with an age-equivalent sample of 138 healthy controls all of which were recruited within the framework of the Vienna Conversion to Dementia Study. Additionally, patients and controls underwent individual assessment using a comprehensive neuropsychological test battery examining attention, executive functioning, language, and memory (NTBV), the Beck Depression Inventory (BDI), and a measure of premorbid IQ (WST)., Results: Type of diagnosis showed a significant effect on emotion recognition performance, indicating progressively deteriorating results as severity of diagnosis increased. Between-groups effect sizes were substantial, showing non-trivial effects in all comparisons (Cohen's ds from -0.30 to -0.83) except for SCD versus controls. Moreover, emotion recognition performance was higher in women and positively associated with premorbid IQ., Conclusions: Our findings indicate substantial effects of progressive neurological damage on emotion recognition in patients. Importantly, emotion recognition deficits were observable in non-amnestic patients as well, thus conceivably suggesting associations between decreased recognition performance and global cognitive decline. Premorbid IQ appears to act as protective factor yielding lesser deficits in patients showing higher IQs.

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.

Using a gaze-contingent morphing approach, we asked human observers to choose one of two faces that best matched the identity of a target face: one face corresponded to the reference face's fixated part only (e.g., one eye), the other corresponded to the unfixated area of the reference face. The face corresponding to the fixated part was selected significantly more frequently in the inverted than in the upright orientation. This observation provides evidence that face inversion reduces an observer's perceptual field of view, even when both upright and inverted faces are displayed at full view and there is no performance difference between these conditions. It rules out an account of the drop of performance for inverted faces--one of the most robust effects in experimental psychology--in terms of a mere difference in local processing efficiency. A brain-damaged patient with pure prosopagnosia, viewing only upright faces, systematically selected the face corresponding to the fixated part, as if her perceptual field was reduced relative to normal observers. Altogether, these observations indicate that the absence of visual knowledge reduces the perceptual field of view, supporting an indirect view of visual perception., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Presented case report illustrates symptoms of prosopometamorphopsia (PM) and prosopagnosia, observed in the early days after the onset of a hemorrhagic stroke resulting from a complication of endovascular treatment of intracranial aneurysms and the use of anticoagulation therapy. PM is a visual disorder in which faces are perceived as distorted. The female patient described in the present study reported that faces she looked at seemed younger or older than in reality or as if they were dirty, swollen, or with a grimace. She also experienced symptoms of prosopagnosia, which is difficulty of recognizing familiar faces of people (e.g., of her husband and daughter). In the interview 6 months after the first examination, the patient reported spontaneous withdrawal of the visual disturbances.

Emotional processing deficits have recently been identified in individuals with traumatic brain injury (TBI), specifically in the domain of facial affect recognition. However, the neural networks underlying these impairments have yet to be identified. In the current study, 42 individuals with moderate to severe TBI and 23 healthy controls performed a task of facial affect recognition (Facial Emotion Identification Test (FEIT)) in order to assess their ability to identify and discriminate six emotions: happiness, sadness, anger, surprise, shame, and fear. These individuals also underwent structural neuroimaging including diffusion tensor imaging to examine white matter (WM) integrity. Correlational analyses were performed to determine where in the brain WM damage was associated with performance on the facial affect recognition task. Reduced performance on the FEIT was associated with reduced WM integrity (fractional anisotropy, mean diffusivity, axial diffusivity, and radial diffusivity) in the inferior longitudinal fasciculus and inferior-fronto-occipital fasciculus in individuals with TBI. Poor performance on the task was additionally associated with reduced gray matter (GM) volume in lingual gyrus and parahippocampal gyrus. The results implicate a pattern of WM and GM damage in TBI that may play a role in emotional processing impairments.

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.

Considerable research has supported the view that faces and words are subserved by independent neural mechanisms located in the ventral visual cortex in opposite hemispheres. On this view, right hemisphere ventral lesions that impair face recognition (prosopagnosia) should leave word recognition unaffected, and left hemisphere ventral lesions that impair word recognition (pure alexia) should leave face recognition unaffected. The current study shows that neither of these predictions was upheld. A series of experiments characterizing speed and accuracy of word and face recognition were conducted in 7 patients (4 pure alexic, 3 prosopagnosic) and matched controls. Prosopagnosic patients revealed mild but reliable word recognition deficits, and pure alexic patients demonstrated mild but reliable face recognition deficits. The apparent comingling of face and word mechanisms is unexpected from a domain-specific perspective, but follows naturally as a consequence of an interactive, learning-based account in which neural processes for both faces and words are the result of an optimization procedure embodying specific computational principles and constraints.

Purpose: To determine how visual field loss as assessed by microperimetry is correlated with deficits in face recognition., Methods: Twelve patients (age range, 26-70 years) with impaired visual sensitivity in the central visual field caused by a variety of pathologies and 12 normally sighted controls (control subject [CS] group; age range, 20-68 years) performed a face recognition task for blurred and unblurred faces. For patients, we assessed central visual field loss using microperimetry, fixation stability, Pelli-Robson contrast sensitivity, and letter acuity., Results: Patients were divided into two groups by microperimetry: a low vision (LV) group (n = 8) had impaired sensitivity at the anatomical fovea and/or poor fixation stability, whereas a low vision that excluded the fovea (LV:F) group (n = 4) was characterized by at least some residual foveal sensitivity but insensitivity in other retinal regions. The LV group performed worse than the other groups at all blur levels, whereas the performance of the LV:F group was not credibly different from that of the CS group. The performance of the CS and LV:F groups deteriorated as blur increased, whereas the LV group showed consistently poor performance regardless of blur. Visual acuity and fixation stability were correlated with face recognition performance., Conclusions: Persons diagnosed as having disease affecting the central visual field can recognize faces as well as persons with no visual disease provided that they have residual sensitivity in the anatomical fovea and show stable fixation patterns. Performance in this task is limited by the upper resolution of nonfoveal vision or image blur, whichever is worse.

Primary Objective: The occurrence of facial affect recognition difficulties in a sample of people accessing traumatic brain injury (TBI) rehabilitation services was examined. It was hypothesized that between 13-39% of participants would demonstrate clinically significant impairment in facial affect recognition., Research Design: Cross-sectional sample of eligible participants who were current clients of eight brain injury services were invited to participate., Methods and Procedures: Forty-five participants with mild-to-severe TBI were assessed for facial affect recognition difficulties using the facial affect discrimination, naming, selection and matching sub-tests of the Florida Affect Battery., Main Outcomes and Results: Fifty-one per cent of participants had at least moderate difficulties with facial affect recognition., Conclusions: The current sample was not a random selection from the population of people with TBI, so the results do not establish a formal estimate of prevalence. Nonetheless, the data indicate that when assessing typical clients with severe brain injuries presenting to neurorehabilitation services, there is likely to be a high frequency of occurrence of facial affect recognition difficulties. Rehabilitation outcomes may be improved by screening for and treating facial affect recognition difficulties following TBI. Further examination and development of treatment options is warranted.

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.

The ability to recognize and identify people and determine how they may be feeling from looking at their faces is an important skill that people normally achieve effortlessly in infancy. Effective face recognition skills remain essential for social competence throughout the life course. A major cause of impairment in face processing, conventionally known as prosopagnosia, is stroke. In this article, the potentials for acquired prosopagnosia after stroke are examined. The incidence of prosopagnosia after stroke is difficult to establish, but in one clinical sample about half of those who survived a right hemisphere stroke had prosopagnosia. The recently published National Clinical Guideline for Stroke 2012 omits reference to assessment for prosopagnosia, which suggests that the personal distress and negative impact on social life that can accompany prosopagnosia is not fully appreciated or at least not considered a priority after stroke. The few published cases where there has been a focused attempt to provide rehabilitation for chronic prosopagnosia suggest that lesions in face-processing areas are resistant to treatment but that some recovery can accompany extended practice. It is concluded that where there is evidence of prosopagnosia following stroke, treatment should be offered, although rehabilitation may be better focused on supporting and extending existing compensatory strategies, such as the use of voice, body shape, and gait to assist in person recognition and, as an important consequence, social functioning.

Neurologic disorders can produce "secondary" mania, and clinicians must distinguish secondary mania from bipolar disorders (BD). Patients with new and late onset mania require an evaluation that includes a thorough history, a neurologic examination, neuroimaging, and other selected tests. Neurologic causes of mania include strokes in the right basotemporal or inferofrontal region, strokes or tumors in the perihypothalamic region, Huntington's disease and other movement disorders, multiple sclerosis and other white matter diseases, head trauma, infections such as neurosyphilis and Creutzfeldt-Jakob disease, and frontotemporal lobar degeneration. The term Frontotemporal Lobar Degeneration (FTLD) is suggested for neurodegenerative diseases characterized by focal degeneration such as Primer Progressive Aphasia (PPA), Frontal Lobe Dementia, PPA- Amyotrophic Lateral Sclerosis (ALS), and Corticobasal Degeneration. In this article, we report a frontotemporal dementia (FTD) case that referred with manic symptoms. The female patient was 46 years old, married, graduated from primary school, and had been admitted with complaints of hyperactivity, excessive talking, and decreased sleep for one week. She presented first with complaints that began three years ago that included the inability to remember names, recognize faces, use household appliances, and follow rules. She had also been repeating the same words and behaviors. Prosopagnosia, aphasia, and a positive family history of ALS were discussed with related index in our case.

Background: Prosopagnosia, the selective inability to recognize known faces, has been described in Alzheimer's disease and fronto-temporal dementia but is not expected to occur in Parkinson's disease (PD)., Methods and Results: We report three PD patients who developed recurrent, paroxysmal and short-lasting episodes of prosopagnosia, before progressing to PD dementia (PDD). Hallucinations and other higher-order visual deficits - such as optic ataxia and micro/macropsia - were also seen., Conclusion: Progressive signs of temporal and parietal dysfunction have been suggested to herald dementia in PD. The observation of prosopagnosia and other higher-order visuoperceptive defects in the transition to dementia, reinforce the importance of posterior-cortical deficit in PD., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Many community-residing patients with Alzheimer's disease (AD) have way-finding problems, particularly at twilight or on rainy days. In an attempt to understand the mechanism, we prepared pictures of street scenes, including 8 personally familiar and 8 unfamiliar, divided into Low Spatial Frequency (LSF) and Low Luminance (LL) conditions to simulate foggy or rainy days and nighttime. Each picture was presented from the most difficult (level 10) to the easiest (level 1). The participants, including 20 very mild AD patients and 20 normal controls (NC) with equal basic visual acuity, were asked to judge whether a picture was familiar or not and to describe how they came to that conclusion. The accuracy of familiar scene recognition was measured by the number of pictures successfully recognized and the ability thereof by the level needed. Compared with NC, AD patients showed poorer accuracy (2.7 ± 0.2 versus 3.6 ± 0.1, mean ± SEM, p = 0.003 under LSF; 2.8 ± 0.2 versus 3.8 ± 0.1, p = 0.001 under LL) and poorer ability (2.2 ± 0.4 versus 4.3 ± 0.4 p = 0.000 under LSF; 2.9 ± 0.3 versus 5.2 ± 0.5, p = 0.000 under LL) for both conditions. The AD patients used a global element to help judge when personally familiar scenes were displayed, which was the method NC usually adopted when presented with novel scenes. In summary, this study demonstrated poorer recognition ability in very mild AD patients when personally familiar street scenes were displayed, and the underlying mechanisms may include impaired visual search performance and efficiency. The deficits also reflect their difficulty in real life situations when their familiar environments become blurred or dark.

Developmental prosopagnosia (DP) is defined by severe face recognition problems resulting from a failure to develop the necessary visual mechanisms for processing faces. While there is a growing literature on DP in adults, little has been done to study this disorder in children. The profound impact of abnormal face perception on social functioning and the general lack of awareness of childhood DP can result in severe social and psychological consequences for children. This review discusses possible aetiologies of DP and summarizes the few cases of childhood DP that have been reported. It also outlines key objectives for the growth of this emerging research area and special considerations for studying DP in children. With clear goals and concerted efforts, the study of DP in childhood will be an exciting avenue for enhancing our understanding of normal and abnormal face perception for all age groups.

Conspicuous deficits in face recognition characterize prosopagnosia. Information on whether agnosic deficits may extend to non-facial body parts is lacking. Here we report the neuropsychological description of FM, a patient affected by a complete deficit in face recognition in the presence of mild clinical signs of visual object agnosia. His deficit involves both overt and covert recognition of faces (i.e. recognition of familiar faces, but also categorization of faces for gender or age) as well as the visual mental imagery of faces. By means of a series of matching-to-sample tasks we investigated: (i) a possible association between prosopagnosia and disorders in visual body perception; (ii) the effect of the emotional content of stimuli on the visual discrimination of faces, bodies and objects; (iii) the existence of a dissociation between identity recognition and the emotional discrimination of faces and bodies. Our results document, for the first time, the co-occurrence of body agnosia, i.e. the visual inability to discriminate body forms and body actions, and prosopagnosia. Moreover, the results show better performance in the discrimination of emotional face and body expressions with respect to body identity and neutral actions. Since FM's lesions involve bilateral fusiform areas, it is unlikely that the amygdala-temporal projections explain the relative sparing of emotion discrimination performance. Indeed, the emotional content of the stimuli did not improve the discrimination of their identity. The results hint at the existence of two segregated brain networks involved in identity and emotional discrimination that are at least partially shared by face and body processing., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

We present a new case of acquired prosopagnosia resulting from extensive lesions predominantly in the right occipitotemporal cortex. Functional brain imaging revealed atypical activation of all core face areas in the right hemisphere, with reduced signal difference between faces and objects compared to controls. In contrast, Herschel's lateral occipital complex showed normal activation to objects. Behaviourally, Herschel is severely impaired with the recognition of familiar faces, discrimination between unfamiliar identities, and the perception of facial expression and gender. Notably, his visual recognition deficits are largely restricted to faces, suggesting that the damaged mechanisms are face-specific. He showed normal recognition memory for a wide variety of object classes in several paradigms, normal ability to discriminate between highly similar items within a novel object category, and intact ability to name basic objects (except four-legged animals). Furthermore, Herschel displayed a normal face composite effect and typical global advantage and global interference effects in the Navon task, suggesting spared integration of both face and nonface information. Nevertheless, he failed visual closure tests requiring recognition of basic objects from degraded images. This abnormality in basic object recognition is at odds with his spared within-class recognition and presents a challenge to hierarchical models of object perception.

We examined the ability of people 70 to 90 years old to apply global, configural, and featural face-processing strategies. In addition we investigated age-related changes in the ability to categorize faces at basic, subordinate, and individual levels. Using the N170 potential as index of early face processing and the P300 component as index of categorical decision making and effort, we found significant age-related perceptual changes which slowed and somewhat impaired face processing. Specifically, older participants had problems integrating face features into global structures, demonstrating enhanced dependence on distal global information. They did not apply configural computations by default while processing faces which suggests that, unless identification is required, they process faces only at a basic level. These perceptual changes could be the cause for slower and less accurate subordinate categorization, particularly when it is based on details. At the neural levels face processing was not right-lateralized, reflecting excessive involvement of the left hemisphere in perception leading to a more general reduction of interhemispheric asymmetry. In addition we found excessive but nonselective activation of frontal regions adding support to the view that executive control and particularly inhibition of irrelevant input are reduced in the elderly., (Copyright © 2012 Elsevier Inc. All rights reserved.)

A 48-year-old woman presented with a 2-week history of headache. The headache was so severe in the standing position that she could hardly stand up. The results of general and neurological examination were unremarkable. MRI studies of the brain showed diffuse pachymeningeal gadolinium enhancement on the T(1) weighted images. The cerebrospinal fluid (CSF) opening pressure on lumbar puncture was 70 mm H(2)O. MR myelography and RI-cisternography disclosed leak of cerebrospinal fluid at the lumbar level. Then, we diagnosed her as headache associated with liquorrhea. Two weeks later, she noticed an inability to recognize familiar faces, including her own face in the mirror. The Cambridge Face Memory Test (CFMT) proved prospagnosia. Brain MRI revealed multiple subdural hematomas below the bilateral fusiform gyrus. SPECT demonstrated diffuse hypoperfusion in the brain including bilateral fusiform gyrus. Months later, she showed gradual improvement of prospagnosia. Follow-up brain MRI revealed disappearance of both subdural hematomas and diffuse pachymeningeal gadolinium enhancement. SPECT demonstrated marked improvement of cerebral blood flow in the whole brain including the right temporal-occipital lesion. This is the first report of acquired prospagnosia during the course of liquorrhea causing subdural hematomas. Subdural hematoma below a right fusiform gyrus may cause reversible prospagnosia.

Recently we found that adult children whose mothers had had a right-arm preference for holding infants have a reduced left bias for recognising faces, suggesting that they are less well right-hemisphere lateralised for perceiving faces. One possible explanation of this finding is that early visual exposure to faces is suboptimal for right-held infants. To test this idea, we asked mothers to pick up a doll with an inbuilt camera in its face and to start bottle-feeding it. The results showed that less was visible of the face of mothers who held the doll on their right arm in comparison to those who held the doll on their left arm: From the right arm, the mother's left half of the face was less visible when the mothers were looking up and their right half of the face was less visible when they were looking at the doll. These results suggest that right-held infants receive suboptimal information from faces. Because early face exposure is important for face-processing development, the suboptimal face exposure probably experienced by right-held infants may have consequences for their ability to recognise faces and facial emotion later in life.

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.)

People with 22q11.2 deletion syndrome (22q11DS) have deficits in face emotion recognition. However, it is not known whether this is a deficit specific to faces, or represents maladaptive information processing strategies to complex stimuli in general. This study examined the specificity of face emotion processing deficits in 22q11DS by exploring recognition accuracy and visual scanpath performance to a Faces task compared to a Weather Scene task. Seventeen adolescents with 22q11DS (11=females, age=17.4) and 18 healthy controls (11=females, age=17.7) participated in the study. People with 22q11DS displayed an overall impoverished scanning strategy to face and weather stimuli alike, resulting in poorer accuracy across all stimuli for the 22q11DS participants compared to controls. While the control subjects altered their information processing in response to faces, a similar change was not present in the 22q11DS group indicating different visual scanpath strategies to identify category within each of the tasks, of which faces appear to represent a particularly difficult subcategory. To conclude, while this study indicates that people with 22q11DS have a general visual processing deficit, the lack of strategic change between tasks suggest that the 22q11DS group did not adapt to the change in stimuli content as well as the controls, indicative of cognitive inflexibility rather than a face specific deficit., (Copyright © 2011 Elsevier Ltd. All rights reserved.)