[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.

[Congenital amusia and its effects on non-musical skills]. / Amusia congénita y sus efectos en habilidades no musicales.

INTRODUCTION: Congenital amusia is a specific condition in which the individual is unable to recognise tonal variations in a piece of musical. This cannot be explained by a previous brain injury, hearing loss, cognitive deficit, socio-affective disorder or lack of environmental stimulation. The current estimated prevalence is 1.5% of the world population, with a significant genetic component among those who suffer from it. It has been claimed that certain cognitive abilities in the emotional, spatial and language fields may be affected in people with amusia. AIM: To review the literature describing the effects on non-musical skills that may coexist in individuals with congenital amusia. DEVELOPMENT: Several neuroimaging studies have observed morphological and functional changes in the temporal lobe, as well as in the white matter connections between the superior temporal gyrus and the inferior frontal gyrus. From these affected regions, there may be a deficit in cognitive skills related to adjacent areas. CONCLUSIONS: Congenital amusia has been associated with poor performance in different non-musical cognitive skills, such as visuospatial processing, language processing, reading difficulties, face recognition and emotional aspects.

TITLE: Amusia congénita y sus efectos en habilidades no musicales.Introducción. La amusia congénita es una condición específica en la que el individuo afectado es incapaz de reconocer variaciones tonales en las piezas musicales. Esto no puede explicarse por una lesión encefálica previa, una pérdida auditiva, un déficit cognitivo, un trastorno socioafectivo o una falta de estimulación ambiental. Actualmente se estima una prevalencia del 1,5% de la población mundial, con un importante componente genético entre los afectados. Se ha descrito que en las personas con amusia puede haber afectación de ciertas habilidades cognitivas en el campo emocional, espacial y del lenguaje. Objetivo. Revisar la bibliografía donde se describen los efectos en las habilidades no musicales que pueden coexistir en individuos con amusia congénita. Desarrollo. Varios estudios de neuroimagen han permitido observar cambios morfológicos y funcionales en el lóbulo temporal, así como en las conexiones de la sustancia blanca entre el giro temporal superior y el giro frontal inferior. Partiendo de estas regiones afectadas, podría existir un déficit en habilidades cognitivas relacionadas con áreas adyacentes. Conclusiones. La amusia congénita se ha relacionado con un pobre desempeño en diferentes habilidades cognitivas no musicales, como el procesamiento visuoespacial, el procesamiento del lenguaje, alteraciones de la lectura, el reconocimiento de rostros y aspectos emocionales.

Anxiety disorders in adolescence are associated with impaired facial expression recognition to negative valence.

OBJECTIVE: The aim of the present study was to test the ability of adolescents with a current anxiety diagnosis to recognize facial affective expressions, compared to those without an anxiety disorder. METHODS: Forty cases and 27 controls were selected from a larger cross sectional community sample of adolescents, aged from 10 to 17 years old. Adolescent's facial recognition of six human emotions (sadness, anger, disgust, happy, surprise and fear) and neutral faces was assessed through a facial labeling test using Ekman's Pictures of Facial Affect (POFA). RESULTS: Adolescents with anxiety disorders had a higher mean number of errors in angry faces as compared to controls: 3.1 (SD=1.13) vs. 2.5 (SD=2.5), OR=1.72 (CI95% 1.02 to 2.89; p=0.040). However, they named neutral faces more accurately than adolescents without anxiety diagnosis: 15% of cases vs. 37.1% of controls presented at least one error in neutral faces, OR=3.46 (CI95% 1.02 to 11.7; p=0.047). No differences were found considering other human emotions or on the distribution of errors in each emotional face between the groups. CONCLUSION: Our findings support an anxiety-mediated influence on the recognition of facial expressions in adolescence. These difficulty in recognizing angry faces and more accuracy in naming neutral faces may lead to misinterpretation of social clues and can explain some aspects of the impairment in social interactions in adolescents with anxiety disorders.

Covert face recognition without the fusiform-temporal pathways.

Patients with prosopagnosia are unable to recognize faces consciously, but when tested indirectly they can reveal residual identification abilities. The neural circuitry underlying this covert recognition is still unknown. One candidate for this function is the partial survival of a pathway linking the fusiform face area (FFA) and anterior-inferior temporal (AIT) cortex, which has been shown to be essential for conscious face identification. Here we performed functional magnetic, and diffusion tensor imaging in FE, a patient with severe prosopagnosia, with the goal of identifying the neural substrates of his robust covert face recognition. FE presented massive bilateral lesions in the fusiform gyri that eliminated both FFAs, and also disrupted the fibers within the inferior longitudinal fasciculi that link the visual areas with the AITs and medial temporal lobes. Therefore participation of the fusiform-temporal pathway in his covert recognition was precluded. However, face-selective activations were found bilaterally in his occipital gyri and in his extended face system (posterior cingulate and orbitofrontal areas), the latter with larger responses for previously-known faces than for faces of strangers. In the right hemisphere, these surviving face selective-areas were connected via a partially persevered inferior fronto-occipital fasciculus. This suggests an alternative occipito-frontal pathway, absent from current models of face processing, that could explain the patient's covert recognition while also playing a role in unconscious processing during normal cognition.

Prosopagnosia congênita: relato de caso / Congenital prosopagnosia: a case report

Prosopagnosia is a visual agnosia characterized by an inability to recognize previously known human faces and to learn new faces. The aim of this study was to present a forty-six year-old woman with congenital prosopagnosia, and to discuss the neural bases of perception and recognition of faces. The patients had a lifetime impairment in recognizing faces of family members, close friends, and even her own face in photos. She also had impairment in recognizing animals such as discriminating between cats and dogs. The patients basic visual skills showed impairment in identifying and recognizing the animal form perception on the coding subtest of the WAIS-R, recognizing overlapping pictures (Luria), and in identifying silhouettes depicting animals and objects (VOSP). Unconventional tests using pictures evidenced impairment in her capacity to identify famous faces, facial emotions and animals. Her face perception abilities were preserved, but recognition could not take place. Therefore, it appears that the agnosia in this case best fits the group of categories termed associative.

Prosopagnosia é uma agnosia visual caracterizada por uma incapacidade de reconhecer faces humanas vistas anteriormente e aprender outras. O objetivo é apresentar uma mulher de 46 anos com prosopagnosia congênita e discutir as bases neurais da percepção e do reconhecimento de faces. Ela nos procurou referindo apresentar desde a infância problemas no reconhecimento de faces de membros da família, amigos próximos e mesmo para sua própria imagem numa fotografia. Também diz apresentar prejuízo no reconhecimento de animais, como discriminar cães de gatos. Apresentou dificuldades em identificar e reconhecer animais desenhados; reconhecer figuras sobrepostas (Luria), incorrendo em paragnosias visuais e identificar silhuetas de animais (VOSP). Em testes não convencionais, usando figuras, evidenciou diminuição da capacidade em identificar faces famosas, expressões faciais e animais, mas não em estimar o sexo e a idade das pessoas. Concluindo, suas habilidades perceptuais para face estão preservadas, mas há um déficit de reconhecimento. Tudo indica que sua agnosia pertence ao grupo das associativas.

Migraine aura and related phenomena: beyond scotomata and scintillations.

Migraine affects the cortical physiology and may induce dysfunction both ictally and interictally. Although visual symptoms predominate during aura, other contiguous cortical areas related to less impressive symptoms are also impaired in migraine. Answers from 72.2% migraine with aura and 48.6% of migraine without aura patients on human faces and objects recognition, colour perception, proper names recalling and memory in general showed dysfunctions suggestive of prosopagnosia, dyschromatopsia, ideational apraxia, alien hand syndrome, proper name anomia or aphasia, varying in duration and severity. Symptoms frequently occurred in a successively building-up pattern fitting with the geographical distribution of the various cortical functions. When specifically inquired, migraineurs reveal less evident symptoms that are not usually considered during routine examination. Spreading depression most likely underlies the aura symptoms progression. Interictal involvement indicates that MWA and MWoA are not completely silent outside attacks, and that both subforms of migraine may share common mechanisms.

Síndrome de Capgras en enfermedad de Alzheimer: presentación de 2 casos / Capgras syndrome in Alzheimer's disease: two cases

Se presentan dos casos de síndrome de Capgras (SC) en mujeres con enfermedad de Alzheimer. Ambas tenían la convicción delirante de que su esposo no era tal sino que un impostor; la primera en forma consistente y duradera, la segunda en forma fluctuante. La revisión de la literatura permite comparar el SC con la paramnesia reduplicativa y el “jamais vu” y contrastarlo con la prosopagnosia. La fisiopatología del SC dependería de que al percibir un rostro se procesan en forma paralela a) los detalles visuales que permiten reconocerlo como un rostro característico (capacidad que se pierde en la prosopagnosia), y b) la respuesta emocional que se pierde en el SC. La pérdida de familiaridad de una persona conocida induce la sensación de que se trata de un impostor. Los estudios de imágenes funcionales permiten suponer que en el procesamiento de los rostros y voces familiares participa en forma destacada la parte posterior del girus cingulado.

Two cases Capgras syndrome (CS) are presented in women with Alzheimer’s disease. Both patients had the conviction that their husbands had been supplanted by an impostor; in the first one consistently and permanently, and the second one in a fluctuating form. Revision of the literature allows to compare CS with reduplicative paramnesia and “jamais vu”; and contrast these with prosopagnosia. The physiopathology of CS can be explained by assuming that during face recognition two processes are participating in parallel: a) the recognition of visual details, that allow identification of a face (which is lost in prosopagnosia), and b) the emotional response, which is lost in CS. The loss of familiarity of a known person induces the sensation of an impostor. Functional imaging studies suggest that the posterior cingulate gyrus has a predominant role in the processing of familiar faces and voices.

Covert matching of unfamiliar faces in a case of prosopagnosia: an ERP study.

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

[Procesing of faces: neurological bases, disorders and evaluation]. / Procesamiento de caras: bases neurológicas, trastornos y evaluación.

INTRODUCTION AND DEVELOPMENT: The processing of faces and recognition of familiar faces involves activity in many regions of the brain: 1. The visual association cortex in the act of constructing the perceived object; 2. Hippocampus and frontotemporal regions in the process of comparing what has been perceived with remembered images to activate sentiments of familiarity; 3. The temporoparietal regions in the semantic memory related to people, and 4. The left hemisphere in the activation of linguistic structures which codify information for access to the name. Lesions in any of these structures may give rise to alterations in the different steps of the cerebral processing of faces, amongst which prosopagnosia and prosopamnesia stand out. CONCLUSION: The psychophysiological and functional neuroimaging studies will permit, in the near future, evaluation and trial of cognitive models and advances in the understanding of cerebral processing of faces, their evaluation and disorders.

Prosopagnosia após ferimento por arma de fogo: relato de caso / Prosopagnosia after gubshot head injury: case report

Relata-se o caso de uma jovem, vítima de estupro, seguido de ferimento na regão occipital do crânio por arma de fogo. Após procedimento de limpeza cirúrgica e recuperação clínica pós-operatória, constatou-se que a paciente estava com quadro de prosopagnosia, com dificuldade no reconhecimento até mesmo de seus familiares, chegando a fazê-lo baseado em outros comemorativos como a voz e a forma de se vestir. A literatura foi revista, ressaltando-se alguns aspectos principais do quadro prosopagnósico.