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      Progressive associative phonagnosia: A neuropsychological analysis

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          Abstract

          There are few detailed studies of impaired voice recognition, or phonagnosia. Here we describe two patients with progressive phonagnosia in the context of frontotemporal lobar degeneration. Patient QR presented with behavioural decline and increasing difficulty recognising familiar voices, while patient KL presented with progressive prosopagnosia. In a series of neuropsychological experiments we assessed the ability of QR and KL to recognise and judge the familiarity of voices, faces and proper names, to recognise vocal emotions, to perceive and discriminate voices, and to recognise environmental sounds and musical instruments. The patients were assessed in relation to a group of healthy age-matched control subjects. QR exhibited severe impairments of voice identification and familiarity judgments with relatively preserved recognition of difficulty-matched faces and environmental sounds; recognition of musical instruments was impaired, though better than recognition of voices. In contrast, patient KL exhibited severe impairments of both voice and face recognition, with relatively preserved recognition of musical instruments and environmental sounds. Both patients demonstrated preserved ability to analyse perceptual properties of voices and to recognise vocal emotions. The voice processing deficit in both patients could be characterised as associative phonagnosia: in the case of QR, this was relatively selective for voices, while in the case of KL, there was evidence for a multimodal impairment of person knowledge. The findings have implications for current cognitive models of voice recognition.

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          Most cited references65

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          Thinking the voice: neural correlates of voice perception.

          The human voice is the carrier of speech, but also an "auditory face" that conveys important affective and identity information. Little is known about the neural bases of our abilities to perceive such paralinguistic information in voice. Results from recent neuroimaging studies suggest that the different types of vocal information could be processed in partially dissociated functional pathways, and support a neurocognitive model of voice perception largely similar to that proposed for face perception.
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            Understanding face recognition with an interactive activation model.

            In this paper we describe how the microstructure of the Bruce & Young (1986) functional model of face recognition may be explored and extended using an interactive activation implementation. A simulation of the recognition of familiarity of individuals is developed which accounts for a range of published findings on the effects of semantic priming, repetition priming and distinctiveness. Finally, we offer some speculative predictions made by the model, and point to an empirical programme of research which it suggests.
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              The clinical profile of right temporal lobe atrophy.

              Frontotemporal lobar degeneration is currently associated with three syndromic variants. Disorders of speech and language figure prominently in two of the three variants, and are associated with left-sided frontotemporal atrophy. The detailed characterization of these syndromes contrasts with the relative paucity of information relating to frontotemporal lobar degeneration primarily affecting the right cerebral hemisphere. The objective of this study was to identify the clinical profile associated with asymmetrical, predominantly right-sided, temporal lobe atrophy. Twenty patients with predominant right temporal lobe atrophy were identified on the basis of blinded visual assessment of the MRI scans. The severity of right temporal lobe atrophy was quantified using volumetric analysis of the whole temporal lobes, the amygdala and the hippocampus. Profiles of cognitive function, behavioural and personality changes were obtained on each patient. The pattern of atrophy and the clinical features were compared with those observed in a group of patients with semantic dementia and predominant left-sided temporal lobe atrophy. The mean right temporal lobe volume in the right temporal lobe atrophy group was reduced by 37%, with the mean left temporal lobe volume reduced by 19%. There was marked atrophy of the right hippocampus and right amygdala, with mean volumes reduced by 41 and 51%, respectively (left hippocampus and amygdala volumes were reduced by 18 and 33%, respectively). The most prominent cognitive deficits were impairment of episodic memory and getting lost. Prosopagnosia was a symptom in right temporal lobe atrophy patients. These patients also exhibited a variety of behavioural symptoms including social disinhibition, depression and aggressive behaviour. Nearly all behavioural disorders were more prevalent in the right temporal lobe atrophy patient group than the semantic dementia group. Symptoms particular to the right temporal lobe atrophy patient group included hyper-religiosity, visual hallucinations and cross-modal sensory experiences. The combination of clinical features associated with predominant right temporal lobe atrophy differs significantly from those associated with the other syndromes associated with focal degeneration of the frontal and temporal lobes and it is, therefore, proposed that this right temporal variant should be considered a separate syndromic variant of frontotemporal lobar degeneration.
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                Author and article information

                Journal
                Neuropsychologia
                Neuropsychologia
                Neuropsychologia
                Pergamon Press
                0028-3932
                1873-3514
                March 2010
                March 2010
                : 48
                : 4
                : 1104-1114
                Affiliations
                [a ]Dementia Research Centre, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, United Kingdom
                [b ]Centre for the Neural Basis of Hearing, Physiology Department, University of Cambridge, Cambridge, United Kingdom
                Author notes
                [* ]Corresponding author. Tel.: +44 0207 829 8773; fax: +44 0207 676 2066. jwarren@ 123456drc.ion.ucl.ac.uk
                Article
                NSY3511
                10.1016/j.neuropsychologia.2009.12.011
                2833414
                20006628
                50281915-2c8e-4e4b-89a4-8300d2b23dca
                © 2010 Elsevier Ltd.

                This document may be redistributed and reused, subject to certain conditions.

                History
                : 21 September 2009
                : 23 November 2009
                : 7 December 2009
                Categories
                Article

                Neurology
                person knowledge,prosopagnosia,face,dementia,voice,frontotemporal lobar degeneration
                Neurology
                person knowledge, prosopagnosia, face, dementia, voice, frontotemporal lobar degeneration

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