Cases reported "Agnosia"

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1/20. Perceiving left and imagining right: dissociation in neglect.

    Signor Piazza, a patient with a left parieto-occipital haemorrhage and a right thalamic stroke, showed severe right personal neglect (e.g. touching own body parts) and right perceptual neglect in tasks with (e.g. cancelling tasks) or without (e.g. description of a complex picture) motor response. He had also right-sided neglect dyslexia (including single words), without language impairments. However, the patient also presented with a clear left-sided deficit in the representational domain (e.g. imagery tasks). Signor Piazza's pattern of performance suggests dissociation between imagery and perception within the neglect syndrome.
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2/20. Auditory agnosia restricted to environmental sounds following cortical deafness and generalized auditory agnosia.

    We encountered a case of auditory agnosia restricted to environmental sounds, which was associated with the development of bilateral subcortical lesions after suffering a bilateral putaminal hemorrhage. The patient had a history of a putaminal hemorrhage on her left side without any major disability. Three years later, she suffered a putaminal hemorrhage on the other side. The clinical picture started with cortical deafness, then changed to generalized auditory agnosia for verbal and environmental sounds, and finally developed into auditory agnosia confined to the perception of environmental sounds. Her errors in a test of sound recognition were discriminative rather than associative in nature. Neuro-radiological examinations revealed bilateral subcortical lesions involving the fibers from the medial geniculate body to the temporal lobes after bilateral putaminal hemorrhage. This case suggested that the subcortical lesion involving bilateral acoustic radiation could cause either cortical deafness, auditory agnosia of all sounds, or auditory agnosia restricted to environmental sounds.
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3/20. Disordered recognition of facial identity and emotions in three Asperger type autists.

    In this report we aim to explore severe deficits in facial affect recognition in three boys all of whom meet the criteria of Asperger's syndrome (AS), as well as overt prosopagnosia in one (B) and covert prosopagnosia in the remaining two (C and D). Subject B, with a familially-based talent of being highly gifted in physics and mathematics, showed no interest in people, a quasi complete lack of comprehension of emotions, and very poor emotional reactivity. The marked neuropsychological deficits were a moderate prosopagnosia and severely disordered recognition of facial emotions, gender and age. Expressive facial emotion, whole body psychomotor expression and speech prosody were quasi absent as well. In all three boys these facial processing deficits were more or less isolated, and general visuospatial functions, attention, formal language and scholastic performances were normal or even highly developed with the exception of deficient gestalt perception in B. We consider the deficient facial emotion perception as an important pathogenetic symptom for the autistic behaviour in the three boys. prosopagnosia, the absent facial and bodily expression, and speech prosody were important but varying co-morbid disorders. The total clinical picture of non-verbal disordered communication is a complex of predominantly bilateral and/or right hemisphere cortical deficits. Moreover, in B, insensitivity to pain, smells, noises and internal bodily feelings suggested a more general emotional anaesthesia and/or a deficient means of expression. It is possible that a limbic component might be involved, thus making affective appreciation also deficient.
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4/20. The role of size and binocular information in guiding reaching: insights from virtual reality and visual form agnosia III (of III).

    Reaching out to grasp an object requires information about the size of the object and the distance between the object and the body. We used a virtual reality system with a control population and a patient with visual form agnosia (DF) in order to explore the use of binocular information and size cues in prehension. The experiments consisted of a perceptual matching task in addition to a prehension task. In the prehension task, control participants modified their reach distance in response to step changes in vergence in the absence of any clear reference for relative disparity. Their reach distance was unaffected by equivalent step changes in size, even though they used this information to modify grasp and showed a size bias in a distance matching task. Notably, DF showed the same pattern of results as the controls but was far more sensitive to step changes in vergence. This finding complements previous research suggesting that DF relies predominantly on vergence information when gauging target distance. The results from the perceptual matching tasks confirmed previous findings suggesting that DF is unable to make use of size information for perceptual matching, including distance comparisons. These data are discussed with regard to the properties of the pathways subserving the two visual cortical processing streams.
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5/20. Neuropathology of auditory agnosia following bilateral temporal lobe lesions: a case study.

    Our patient was first diagnosed with auditory agnosia following his second cerebral vascular accident (CVA) in 1975 when he was 37 years old. Comprehensive follow-up examinations of auditory function were periodically conducted until his sudden death 15 years later. His brain was studied postmortem for neuropathology. Initial pure-tone audiometry revealed moderate sensorineural hearing loss in the right ear and mild sensorineural hearing loss in the left ear. However, repeated pure-tone audiometry revealed that thresholds became progressively poorer over time, bilaterally. Speech audiometry of both ears consistently revealed that the patient was unable to discriminate any monosyllabic words (i.e. speech intelligibility scores were 0%, bilaterally). In general, speech and hearing tests demonstrated that he could not comprehend spoken words, but could comprehend written commands and gestures. Postmortem neuropathological study of the left hemisphere revealed total defect and neuronal loss of the superior temporal gyrus, including Heschl's gyrus, and total gliosis of the medial geniculate body. In the right hemisphere, examination revealed subcortical necrosis, gliosis in the centre of the superior temporal gyrus and partial gliosis of the medial geniculate body. The pathological examination supports clinical results in which the patient's imperception of speech sounds, music and environmental sounds could be caused by progressive degeneration of bilateral medial geniculate bodies.
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6/20. Is autotopoagnosia real? EC says yes. A case study.

    We report a case of pure autotopagnosia (AT) following a left subcortical vascular accident. The absence of any language disorder, general mental deterioration or other cognitive impairments in this patient allowed an in-depth study of AT. Several tests of body representation and object and animal representation, as well as tests assessing semantic skills were administered to verify current interpretations of AT. Results showed a clear-cut dissociation between defective performances in body representation tests and normal performances on tests involving other kinds of stimuli.The patient's performances were particularly defective on tests relying on visuo-spatial body representation, but her semantic and linguistic knowledge seemed to be spared. This dissociation between different aspects of body representation supports Sirigu et al.'s hypothesis that multiple, partially independent systems are involved in body knowledge. In agreement with this hypothesis, in the present patient AT seems be due to a deficit in a system that processes the structural properties and relative position of single body parts.The present results, reporting the first observation of a subject not affected by any cognitive impairment other than AT, strongly support the existence of a system specifically devoted to body representation.
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7/20. Pure agnosia for mirror stimuli after right inferior parietal lesion.

    This study reports the experimental investigation of G.R., a patient suffering from a highly specific disorder in discriminating mirror stimuli following a right temporoparietal cerebrovascular accident. G.R. showed intact perceptual, attentional, mnestic, linguistic and executive abilities. Object recognition was accurate even under unusual viewing conditions. He was highly accurate in defining the canonical orientation of common objects and in discriminating misoriented objects among identical distracters. However, he was severely impaired in tasks requiring mirror-stimulus discrimination, a deficit that persisted even when the object's coordinates were systematically misaligned with respect to his body. The disorder was also dependent upon the frame of reference (allocentric versus egocentric) activated on the basis of task demands. These results demonstrate the existence of a highly specific disorder in discriminating mirror stimuli defined in object-based coordinates, suggesting a failure in processing the directionality of an object's intrinsic x-axis.
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8/20. Personal and extrapersonal space: a case of neglect dissociation.

    Dissociation between personal and extrapersonal neglect has rarely been observed in man. In this study we present a case of severe personal neglect in the absence of a deficit for extrapersonal space. An extensive neuropsychological assessment demonstrates the absence of cognitive impairments in visuo-spatial processing and confirms the selective presence of a severe representational deficit of the left side of the body.
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9/20. Dissociation between personal and extrapersonal neglect in a crossed aphasia study.

    Abstract Several reports document crossed aphasia following a right cerebral infarct. However, few of them provide a detailed investigation of associated neuropsychological disorders. A personal neglect disorder with no difficulty in orienting attention in the contralesional space has not been frequently reported independently of lesion side for the language deficit. In most cases, the deficit is described in the acute period. We report the case of a patient who showed severe crossed aphasia several months after cerebral damage. In addition to his language deficit, he suffered from finger agnosia, acalculia, and right/left confusion. Although he was able to orient attention in the contralesional space, he had a persistent personal neglect disorder with severe difficulty in attending to his own body. Results suggest that right hemisphere language dominance does not preclude ipsilateral specialisation for other functions. Furthermore, the dissociation between the two spatial functions provides further confirmation that they are subserved by two independent systems.
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10/20. Primary central nervous system angiitis, amyloid angiopathy, and Alzheimer's pathology presenting with Balint's syndrome.

    We report a patient who presented with Balint's syndrome as a manifestation of primary central nervous system angiitis. Clinical findings included simultanagnosia, optic ataxia, and optic apraxia. Pathologic evaluation demonstrated amyloid angiopathy and Alzheimer's plaques. The presence of primary central nervous system angiitis along with amyloid angiopathy and Alzheimer's plaques may not be coincidental. Angiitis may be a foreign body reaction to A4 amyloid deposition.
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