Cases reported "Agnosia"

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11/117. 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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ranking = 1
keywords = perception
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12/117. Neural fate of seen and unseen faces in visuospatial neglect: a combined event-related functional MRI and event-related potential study.

    To compare neural activity produced by visual events that escape or reach conscious awareness, we used event-related MRI and evoked potentials in a patient who had neglect and extinction after focal right parietal damage, but intact visual fields. This neurological disorder entails a loss of awareness for stimuli in the field contralateral to a brain lesion when stimuli are simultaneously presented on the ipsilateral side, even though early visual areas may be intact, and single contralateral stimuli may still be perceived. Functional MRI and event-related potential study were performed during a task where faces or shapes appeared in the right, left, or both fields. Unilateral stimuli produced normal responses in V1 and extrastriate areas. In bilateral events, left faces that were not perceived still activated right V1 and inferior temporal cortex and evoked nonsignificantly reduced N1 potentials, with preserved face-specific negative potentials at 170 ms. When left faces were perceived, the same stimuli produced greater activity in a distributed network of areas including right V1 and cuneus, bilateral fusiform gyri, and left parietal cortex. Also, effective connectivity between visual, parietal, and frontal areas increased during perception of faces. These results suggest that activity can occur in V1 and ventral temporal cortex without awareness, whereas coupling with dorsal parietal and frontal areas may be critical for such activity to afford conscious perception.
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ranking = 2
keywords = perception
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13/117. Generalized auditory agnosia with spared music recognition in a left-hander. Analysis of a case with a right temporal stroke.

    After a right temporoparietal stroke, a left-handed man lost the ability to understand speech and environmental sounds but developed greater appreciation for music. The patient had preserved reading and writing but poor verbal comprehension. Slower speech, single syllable words, and minimal written cues greatly facilitated his verbal comprehension. On identifying environmental sounds, he made predominant acoustic errors. Although he failed to name melodies, he could match, describe, and sing them. The patient had normal hearing except for presbyacusis, right-ear dominance for phonemes, and normal discrimination of basic psychoacoustic features and rhythm. Further testing disclosed difficulty distinguishing tone sequences and discriminating two clicks and short-versus-long tones, particularly in the left ear. Together, these findings suggest impairment in a direct route for temporal analysis and auditory word forms in his right hemisphere to Wernicke's area in his left hemisphere. The findings further suggest a separate and possibly rhythm-based mechanism for music recognition.
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ranking = 1.3496682638411
keywords = discrimination, speech
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14/117. 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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ranking = 2.0539465756088
keywords = perception, speech
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15/117. Visual agnosia after treatment of a posterior fossa ependymoma in a 16-month-old girl.

    We present the clinical observation of a 16-month-old girl treated for a posterior fossa ependymoma who experienced severe and delayed visual dysfunction. She was initially treated by surgery and conventional chemotherapy. When she relapsed at age 3 years, the salvage treatment combined high-dose chemotherapy, second surgery, and local irradiation. At age 4 years, disturbed gait and dysarthric speech appeared rapidly, and she became unable to recognize objects and people. Computed tomography revealed bilateral calcifications in the cerebellum and temporal and occipital lobes but no relapse. The neuropsychologic evaluations revealed signs of visual agnosia and marked intellectual impairment. The role of the different treatment modalities in the pathogenesis of this unusual syndrome is discussed.
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ranking = 0.02697328780439
keywords = speech
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16/117. 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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ranking = 1.0809198634132
keywords = perception, speech
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17/117. Pure word deafness in two patients with subcortical lesions.

    We report two patients with pure word deafness (PWD) with tumour in the III ventricle region with obstructive hydrocephalus. A diagnosis of PWD was made in these two patients in view of impaired verbal comprehension in the presence of adequate hearing, intact acoustic stapedius reflex and well preserved environmental sound perception. Return of verbal comprehension following the radiation therapy observed is probably due to the reduction of the tumour mass and the release of thalamocortical auditory pathways from its compressive effect. Our findings support the hypothesis of the presence of discrete auditory pathways for mediation of verbal and non-verbal stimuli independently.
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ranking = 1
keywords = perception
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18/117. Seeing, since childhood, without ventral stream: a behavioural study.

    We report the case of a 30-year-old man (S.B.) who developed visual agnosia following a meningoencephalitis at the age of 3 years. MRI disclosed extensive bilateral lesions of the occipital temporal visual pathway (ventral stream) and lesions in the right dorsal pathway, sparing primary visual cortices. S.B. showed a severe visual recognition deficit (texture, colour, objects, faces and words), although movement and space perception were largely preserved. His remaining visual capacities illustrate the competence of an isolated dorsal system which essentially functions on the sole basis of magnocellular afferents (low spatial resolution, high sensitivity to low contrast and moving stimuli). Patient S.B. also shows remarkable visuomotor competences, despite his perceptual limitations. It is suggested that his perceptual capacities correspond to the visual processing limitations of the dorsal visual stream, which in this patient have become accessible to perceptual awareness.
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ranking = 1
keywords = perception
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19/117. auditory perception in auditory neuropathy: clinical similarity with auditory verbal agnosia.

    The precise features of auditory perception in patients with auditory neuropathy have not been well described. In the present study, we examined auditory perception in a patient with auditory neuropathy. The patient was a right-handed 7-year-old boy. His chief complaint was delayed speech and suspected of verbal learning disability. He could talk, read and repeat rather fluently but could not understand fully what was asked. V-IQ, P-IQ and F-IQ of Wechsler Scale for Children III-R were 53, 118 and 81, respectively. Pure tone audiogram was completely normal. His speech discrimination ability was very poor. He could identify environmental sounds with visual matching. He could differentiate intensity difference but not time difference. This phenomenon was reported in patients with hemispheric symptoms. These clinical features are very similar to verbal auditory agnosia. ABR showed no response at 90dBnHL alternating clicks and tone bursts. Click evoked and distortion product otoacoustic emissions (OAE) were normal. Electrocochleogram was also normal. Motor and sensory nerve conduction velocity was completely normal. Pa of MLR and N1 of SVR were present. His diagnosis should be "pure type" of auditory neuropathy or auditory nerve disease. Importance of both ABR and OAE examination should be widely announced and auditory neuropathy must be campaigned stressed to be clinical entity among personnel who take care of children with speech delay.
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ranking = 7.3766415516455
keywords = discrimination, perception, speech
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20/117. visual perception without awareness in a patient with posterior cortical atrophy: impaired explicit but not implicit processing of global information.

    A patient with progressive posterior cortical atrophy (PCA) was examined on several tests of visual cognition. The patient displayed multiple visual cognitive deficits, which included problems identifying degraded stimuli, attending to two or more stimuli simultaneously, recognizing faces, tracing simple visual stimuli, matching simple shapes, and copying objects. The patient was also impaired in identifying visual targets contained at the global level within global-local stimuli (i.e., smaller letters that compose a larger letter). Although the patient denied any conscious awareness of the global form, he nevertheless displayed a normal pattern of global interference when asked to identify local level targets. Thus, the patient processed the global information despite not being consciously aware of such information. These results suggest that global-local processing can take place in the absence of awareness. Possible neurocognitive mechanisms explaining this dissociation are discussed.
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ranking = 4
keywords = perception
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