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.

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.

Face recognition performance with a bioptic telescopic device

To determine to what extent performance in face recognition can be improved using a bioptic device. 15 patients with central visual loss were recruited for the study. Their age ranged from 16 to 75 years. Distance visual acuity ranged between 6/24 to 6/120. perceived disability in face recognition was assessed by a four-item questionnaire. These tasks were repeated with the patients using a bioptic telescope. visual acuity improved in all patients with the use of the bioptic telescope. Mean visual acuity improved from 6/36 to 6/12. Face recognition performance improved in 80% of patients [median gain = 33%].A bioptic low vision device can offer a significant improvement in performance for face recognition and may be useful in reducing the handicap associated with this disability

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.

Temporal Variant of Frontotemporal Dementia: A Case of Semantic Dementia

Semantic dementia is a rare, distinct form of frontotemporal lobar degeneration, characterized by a deficit in semantic memory with relative preservation of attention and executive functions. We report a case of semantic dementia that pre-sented with a 3-year history of progressive word-finding difficulty and prosopagnosia. Brain MRI showed prominent atrophic changes in the left temporal region and neuropsychological tests demonstrated semantic memory deficits.

Neuropsychological and Neuroimaging Findings of Semantic Dementia

BACKGROUND: Semantic dementia (SD) is a temporal variant of frontotemporal lobar degeneration (FTLD), which is characterized by naming difficulty, decreased comprehension of words, prosopagnosia and object visual agnosia. We report clinical features, neuropsychological and neuroimaging findings of 4 patients with SD. METHODS: Of 55 patients diagnosed as having FTLD between Jan 1995 and May 2001 at Samsung Medical Center, four patients fulfilled the diagnostic criteria of SD proposed by consensus on FTLD diagnostic criteria. We investigated their clinical features such as presenting symptoms and abnormal behaviors, neuropsychological and neuroimaging findings. Neuropsychological tests included the Seoul Neuropsychological Screening Battery, the Korean-version of Western Aphasia Battery and Hanja reading and writing. All patients underwent brain MRI and FDG-PET. RESULTS: All of the patients showed naming difficulty as a presenting symptom. Language assessments showed severe naming and compre-hension difficulties with preserved fluency and repetition, which were compatible with transcortical sensory aphasia. Whereas Hangul reading aloud and writing were intact, three patients were impaired at Hanja reading and writing. Other neuropsychological tests were remarkable for prosopagnosia, object visual agnosia and memory loss. Brain MRI showed asymmetric temporal atrophies, mainly left antero-inferior temporal cortices. FDG-PET also showed hypome-tabolism in bilateral anterior temporal lobes, more severe on the left. CONCLUSIONS: Our SD patients had characteristic neuropsychological and neuroimaging findings, which can be differentiated from other neurodegenerative diseases. We also found Hanja alexia and agraphia in SD patients, which has not been reported yet.

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.

A Case of Alzheimer's Disease Manifesting as Posterior Cortical Atrophy

A subgroup of patients with progressive degenerative dementia that begins with higher visual dysfunction has been referred to as posterior cortical atrophy (PCA). A 55-year-old woman presented with progressive visual disturbance for 4 years, which was followed by geographical disorientation, impairment of writing and calculation, and memory distur-bance. Neuropsychological deficits were characterized by Balint syndrome, visuospatial dysfunction, prosopagnosia, Gerstmann syndrome and apraxia. Brain MRI showed mild diffuse atrophy. F18-FDG-PET disclosed bilateral occipi-totemporoparietal hypometabolism, more pronounced on the right. Biopsy from right temporal lobe revealed neu-ropathological findings consistent with Alzheimer's disease.

A Case of Alzheimer's Disease Manifesting as Posterior Cortical Atrophy

A subgroup of patients with progressive degenerative dementia that begins with higher visual dysfunction has been referred to as posterior cortical atrophy (PCA). A 55-year-old woman presented with progressive visual disturbance for 4 years, which was followed by geographical disorientation, impairment of writing and calculation, and memory distur-bance. Neuropsychological deficits were characterized by Balint syndrome, visuospatial dysfunction, prosopagnosia, Gerstmann syndrome and apraxia. Brain MRI showed mild diffuse atrophy. F18-FDG-PET disclosed bilateral occipi-totemporoparietal hypometabolism, more pronounced on the right. Biopsy from right temporal lobe revealed neu-ropathological findings consistent with Alzheimer's disease.