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 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.

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.

[Aphasia, prosopagnosia and mania: a case diagnosed with right temporal variant semantic dementia]. / Afazi, Prosopagnozi ve Mani: Semantik Demans Sag Temporal Varyant Tanili Bir Olgu.

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.

"Seeing but not identifying": pure alexia coincident with prosopagnosia in occipital arteriovenous malformation.

BACKGROUND: Pure alexia and prosopagnosia are two separate and uncommon disorders of visual recognition in neuro-ophthalmology. We report an extremely rare case of pure alexia coincident with prosopagnosia secondary to occipital arteriovenous malformation. The manifestations of these two visual recognition disorders are also described. METHODS: A 35-year-old, left-handed women had suffered from severe blurred vision when recognizing her family's faces and was unable to read or associate separate parts of a word into a whole word. Her visual field revealed slight right homonymous hemianopia. Computed tomography scans and magnetic resonance images were arranged and vertebral angiography confirmed the diagnosis of left occipital arteriovenous malformation. RESULTS: Gamma-knife stereoscopic radiotherapy was performed. Two months after the treatment, the ability to recognize faces and read improved and the visual field recovered. CONCLUSIONS: Ophthalmologists should keep in mind that usual complaints of "blurred vision" might correlate with unusual visual recognition disorders. Pure alexia and prosopagnosia have not been reported to occur together and the left-handed- dominance in our case leads to this scarce concurrence.

[Memory improvement in patients with temporal lobe epilepsy at one-year postoperative]. / Amélioration mnésique postopératoire un an après traitement chirurgical de l'épilepsie du lobe temporal.

INTRODUCTION: Several studies have assessed the impact of surgery on both anterograde and remote memory in patients with temporal lobe epilepsy (TLE). The majority of results have shown an extensive memory deficit in patients after temporal resection (TL). However, few protocols have used a prospective longitudinal design. Moreover, the postoperative delays were variable from one study to the next, instead of regular monitoring to identify the potential effect of time elapsed after surgery on memory performance. In addition, some studies have not used strict inclusion criteria to establish homogeneous patient groups. Finally, the impact of surgery on memory has been often assessed by comparing memory skills between epileptic patients and healthy controls. Our aim was to examine the impact of TL on memory in patients with TLE, recruited according to clear-cut clinical criteria. Moreover, we focused on memory performance progression per se in epileptic patients pre- and postoperatively, rather than on memory performance analysis expressed as "deficient" or "normal". METHODS: We assessed 30 patients with unilateral TLE (17 right TLE and 13 left TLE) on four anterograde memory tests and six remote memory tasks. Patients completed all tests preoperatively, and 5 and 12 months after TL. RESULTS: Five months after surgery, performance was equivalent to the preoperative scores for both groups in anterograde memory tasks and remote memory tests. One year after TL, patients with right TLE showed enhanced performance in the verbal anterograde memory tests and in retrieving recent autobiographical memories. Results for left TL showed improved scores only in a recognition memory test of faces. CONCLUSIONS: In the present study, surgical patients were "double winners" gaining seizure freedom and potential of memory stability or recovery. The gain was observed only 12 months after surgery and following temporal resection lateralisation. Our data showed postoperative memory improvement in patients with temporal lobe epilepsy presenting with specific clinical characteristics.

[Transient prosopagnosia after removal of a tumor in the right occipito-temporal cortex: a case report].

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

Prosopagnosia following nonconvulsive status epilepticus associated with a left fusiform gyrus malformation.

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

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.

Normal pressure hydrocephalus manifesting as transient prosopagnosia, topographical disorientation, and visual objective agnosia.

Usually, dementia, gait disturbance and urinary incontinence are an integral part of the clinical presentation of normal pressure hydrocephalus (NPH). However, NPH with transient visual cognitive disorders has not been reported previously. We herein describe an extremely rare case of NPH that presented with transient visual cognitive disorders and long lasting visual memory disturbances that subsided after CSF shunting. A 38-years-old man developed transient prosopagnosia, topographical disorientation, color vision disturbance, and visual objective agnosia that progressed over 5 years. Magnetic resonance images showed ventriculomegaly with ischemic lesions in the paraventricular deep white matter. ECD-SPECT showed a reduction of the cerebral blood flow (CBF) in the periventricular deep white matter. Three months after CSF shunting, the radiological findings normalized with dramatic improvement of the extremely rare symptoms.

Transient postoperative prosopagnosia.

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

Prosopagnosia in a preschool child with Asperger syndrome.

We investigated a male, aged 4 years 11 months, who fulfilled the criteria of Asperger syndrome). In addition to the typical pattern of autistic symptoms, psychological testing revealed prosopagnosia in tasks for face recognition and matching. Prosopagnosia was also present when he tried to identify the faces of his parents and himself in photographs whenever these were presented with photographs of other persons. Although impairment in reciprocal social interaction in individuals with Asperger syndrome is closely correlated to their impaired perceptional abilities in non-verbal communication, especially facial expression, overt prosopagnosia seems to be a rare neuropsychological symptom in persons with autistic disorders.

Ictal prosopagnosia and epileptogenic damage of the dominant hemisphere. A case history.

This case report concerns a patient whose episodes of prosopagnosia appeared to be epileptic manifestations arising from the left occipital lobe. The underlying pathology, as revealed by radiologic and surgical findings, was an atrophic-hypoplastic lesion of the left hemisphere, more prominent in the posterior sections, likely dating from early life. Removal of an arachnoidal cyst of the occipital pole and excision of the damaged gray matter were followed by the disappearance of the prosopagnosic seizures for the two years between the date of the surgical intervention and the publication of this article.