Prosopamnesia: a case report of amnesia for faces.

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.

Right Fusiform Gyrus Infarct with Acute Prosopagnosia.

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.

[The "Tortoni effect" in prosopagnosia]. / El "efecto Tortoni" en la prosopagnosia.

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.

La prosopagnosia es un tipo de agnosia visual caracterizada por la incapacidad de reconocer los rostros de las personas. Existen básicamente dos variantes, aperceptivas y asociativas. El "efecto Tortoni" es un fenómeno descripto por Bekinschtein y col. hace unos años en mozos de café en Buenos Aires, quienes utilizaban esta herramienta para recordar los pedidos de cada integrante de una mesa. Presentamos un caso de prosopagnosia asociada a lesión temporo-occipital bilateral secundaria a traumatismo encefalocraneano, manifestada en forma inicial por la falta de reconocimiento de rostros, con la utilización de una estrategia asociativa similar a la descripta en el efecto "Tortoni" como compensación. Mujer de 62 años que sufrió un traumatismo encefalocraneano grave. Pocos meses después del evento, presentó dificultad para reconocer personas conocidas, hecho evidenciado por sus allegados cuando en una mesa los integrantes cambiaron su asiento, permanecieron callados por unos instantes, y posteriormente la paciente continuó nombrándolos por su ubicación previa. En la resonancia magnética de cerebro se objetivaron lesiones contusas de aspecto secuelar en región temporo-occipital bilateral. La prosopagnosia adquirida secundaria a lesiones focales en la región temporo-occipital generalmente bilateral, derecha, y raramente izquierda, es un cuadro poco frecuente. La estrategia utilizada en el "efecto Tortoni" fue en nuestra paciente una de las manifestaciones iniciales del cuadro. La realización de un test neuropsicológico ecológico que considere esta estrategia podría ser de utilidad en el rastreo y detección precoz de esta entidad.

El "efecto Tortoni" en la prosopagnosia / The "Tortoni effect" in prosopagnosia

Resumen La prosopagnosia es un tipo de agnosia visual caracterizada por la incapacidad de reconocer los rostros de las personas. Existen básicamente dos variantes, aperceptivas y asociativas. El "efecto Tortoni" es un fenómeno descripto por Bekinschtein y col. hace unos años en mozos de café en Buenos Aires, quienes utilizaban esta herramienta para recordar los pedidos de cada integrante de una mesa. Presentamos un caso de prosopagnosia asociada a lesión temporo-occipital bilateral secundaria a traumatismo encefalocra neano, manifestada en forma inicial por la falta de reconocimiento de rostros, con la utilización de una estra tegia asociativa similar a la descripta en el efecto "Tortoni" como compensación. Mujer de 62 años que sufrió un traumatismo encefalocraneano grave. Pocos meses después del evento, presentó dificultad para reconocer personas conocidas, hecho evidenciado por sus allegados cuando en una mesa los integrantes cambiaron su asiento, permanecieron callados por unos instantes, y posteriormente la paciente continuó nombrándolos por su ubicación previa. En la resonancia magnética de cerebro se objetivaron lesiones contusas de aspecto secuelar en región temporo-occipital bilateral. La prosopagnosia adquirida secundaria a lesiones focales en la región temporo-occipital generalmente bilateral, derecha, y raramente izquierda, es un cuadro poco frecuente. La es trategia utilizada en el "efecto Tortoni" fue en nuestra paciente una de las manifestaciones iniciales del cuadro. La realización de un test neuropsicológico ecológico que considere esta estrategia podría ser de utilidad en el rastreo y detección precoz de esta entidad.

Abstract 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.

Putative Alcohol-Related Dementia as an Early Manifestation of Right Temporal Variant of Frontotemporal Dementia.

Diagnosis of frontotemporal dementia is challenging in the early stages. Various psychiatric and neurological diseases are misdiagnosed as frontotemporal dementia and vice versa. Here we present a case with right temporal variant of frontotemporal dementia who presented with alcohol dependency and remarkable behavioral symptoms and was first misdiagnosed as having alcohol-related dementia. He then revealed symptoms related to right temporal variant of frontotemporal dementia, such as prosopagnosia, difficulty recognizing his housemates, loss of empathy, ritualistic behaviors, and difficulty finding and comprehending words. Retrospectively, his alcohol dependency itself was considered an early manifestation of right temporal variant of frontotemporal dementia.

Parosmia in Right-lateralized Semantic Variant Primary Progressive Aphasia: A Case Report.

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.

Prosopagnosia seizure semiology in a 10-year-old boy: a functional neuroimaging study.

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.

Impaired Recognition of Emotional Faces after Stroke Involving Right Amygdala or Insula.

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.

The Anterior Temporal Lobes: New Frontiers Opened to Neuropsychological Research by Changes in Health Care and Disease Epidemiology.

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.

Developmental visual perception deficits with no indications of prosopagnosia in a child with abnormal eye movements.

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.

Incidental memory for faces in children with different genetic subtypes of Prader-Willi syndrome.

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.

Perceptual Learning of Faces: A Rehabilitative Study of Acquired Prosopagnosia.

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.

Tumoral Presentation of Homonymous Hemianopia and Prosopagnosia in Cerebral Amyloid Angiopathy-Related Inflammation.

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.

Prosopagnosia Induced by a Left Anterior Temporal lobectomy Following a Right Temporo-occipital Resection in a Multicentric Diffuse Low-Grade Glioma.

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.

Prosopagnosia as the Presenting Symptom of Whipple Disease.

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.