Progressive prosopagnosia at a very early stage of frontotemporal lobar degeneration

Psychogeriatrics (Impact Factor: 0.99). 11/2007; 7(4):155 - 162. DOI: 10.1111/j.1479-8301.2007.00205.x


Background: The present paper describes a patient with a right temporal lobe variant (RTLV) of frontotemporal lobar degeneration (FTLD).Methods: The study was undertaken when the patient was completely independent in her environment and had not complained of any cognitive problems.Results: Under general neuropsychological assessment, the patient showed no notable deficit other than a difficulty in recognizing famous people by looking at photographs of their faces. Subsequent in-depth evaluation indicated prosopagnosia: the patient presented with an impaired ability to recognize the faces of famous people and family members, whereas her visuospatial abilities were intact. Because the patient was able to recognize familiar people by their voices, the impairment was not a general loss of knowledge about people, but an inability to access this knowledge from visual stimuli (i.e. via the visual modality). The patient also exhibited a ‘within-category’ learning deficit; however, her ability to learn from ‘across-category’ visual stimuli remained intact.Conclusions: Overall, the results of the present study support the proposed model of RTLV of FTLD, where the first sign would be the disruption of face recognition components, leading to a selective form of associative prosopagnosia. Further, the co-occurrence of face and ‘within-category’ object learning deficits favor an interpretation in which a more generalized deficit occurs ‘earlier’ in the sequence of events associated with the object recognition process.

    • "Some explicit attempts have recently been made to bring face recognition disorders of patients with right ATL atrophy back to the framework of prosopagnosia. Thus, Nakachi et al. (2007) and Williams et al. (2006) described patients affected by right anterior temporal atrophy who showed selective disorders in the recognition of familiar faces and considered them as cases of prosopagnosia. In particular, Williams et al. (2006) claimed that their patient BD had difficulty in the holistic treatment of faces, which is considered the basic face processing defect of patients with prosopagnosia. "
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    ABSTRACT: The aim of the present survey was to review clinical and experimental data concerning the visual (face), auditory (voice) and verbal (name) channels through which familiar people are recognized, by contrasting these data with assumptions made by modular cognitive models of familiar people recognition. Particular attention was paid to the fact that visual (face), auditory (voice) and verbal (name) recognition modalities have different hemispheric representations and that these asymmetries have important implications for cognitive models which have not considered hemispheric differences as an important variable in familiar people recognition. Several lines of research have, indeed, shown that familiar faces and voices are mainly underpinned by the right hemisphere, whereas names are mostly subsumed by the left hemisphere. Furthermore, anatomo-clinical data have shown that familiarity judgements are not generated at the level of the Person Identity Nodes (PINs), as suggested by influential cognitive models, but at the level of the modality-specific recognition units, with a right hemisphere dominance in the generation of face and voice familiarity feelings. Additionally, clinical and experimental data have shown that PINs should not be considered as a simple gateway to a unitary semantic system, which stores information about people in an abstract and amodal format, but as structures involved in the storage and retrieval of person-specific information, preferentially represented in a sensory-motor format in the right hemisphere and in a language-mediated format in the left hemisphere. Finally, clinical and experimental data have shown that before the level of the person identity nodes (PINs) a cross-communication exists between the perceptual channels concerning faces and voices, but not between the latter and personal names. These data show that person-specific representations are mainly based on perceptual (face and voice) information in the right hemisphere and on verbal information in the left hemisphere.
    No preview · Article · Sep 2015 · Neuropsychologia
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    • "Results obtained by analyzing the retrieval of person-specific semantic information from names are rather different, because this ability was severely impaired only in patient LP (De Renzi, 1986; De Renzi et al., 1991), who was affected by a left temporal lesion and showed severe semantic disorders, and mildly impaired in patient MT (Schweinberger et al., 1995). Retrieval of person-specific semantic information from names was, on the contrary, unimpaired in most right anterior temporal patients in whom it had been studied, namely in patients VA (Evans et al., 1995), FG (Joubert et al., 2003), BD (Williams et al., 2006), and MT (Nakachi and Muramatsu, 2007). These findings are consistent with results of a recent review (Gainotti, 2007a) of the patterns of famous people recognition in patients with right and left anterior temporal lesions. "
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    ABSTRACT: In the study of prosopagnosia, several issues (such as the specific or non-specific manifestations of prosopagnosia, the unitary or non-unitary nature of this syndrome and the mechanisms underlying face recognition disorders) are still controversial. Two main sources of variance partially accounting for these controversies could be the qualitative differences between the face recognition disorders observed in patients with prevalent lesions of the right or left hemisphere and in those with lesions encroaching upon the temporo-occipital (TO) or the (right) anterior temporal cortex. Results of our review seem to confirm these suggestions. Indeed, they show that (a) the most specific forms of prosopagnosia are due to lesions of a right posterior network including the occipital face area and the fusiform face area, whereas (b) the face identification defects observed in patients with left TO lesions seem due to a semantic defect impeding access to person-specific semantic information from the visual modality. Furthermore, face recognition defects resulting from right anterior temporal lesions can usually be considered as part of a multimodal people recognition disorder. The implications of our review are, therefore, the following: (1) to consider the components of visual agnosia often observed in prosopagnosic patients with bilateral TO lesions as part of a semantic defect, resulting from left-sided lesions (and not from prosopagnosia proper); (2) to systematically investigate voice recognition disorders in patients with right anterior temporal lesions to determine whether the face recognition defect should be considered a form of "associative prosopagnosia" or a form of the "multimodal people recognition disorder."
    Full-text · Article · Jun 2011 · Frontiers in Human Neuroscience
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    ABSTRACT: This thoroughly updated and extended edition covers the various cerebral visual disorders acquired after brain injury, as well as the rehabilitation techniques used to treat them. These are described within a brain plasticity framework, using data from single and group case studies along with follow up observation data. This original, tailor-made approach also includes the recording of eye movements for assessing scanning performance in scene perception and reading. The book gives a brief synopsis of the historical background on the subject, alongside an outline of intervention designs and methodological difficulties in the field, and goes on to discuss the mechanisms and processes that provide the foundations for recovery of function and successful adaptation in visually impaired patients. The author concludes by analyzing the importance of the procedures and outcomes of treatments to the reduction of patients' visual handicaps. The new edition also contains an appendix with recommendations on the case histories, diagnostics and treatments. It is ideal reading for students in clinical neuropsychology, as well as professionals in the fields of neurology, visual neuroscience and rehabilitation experts.
    No preview · Article · Jan 2010
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