Cases reported "Perceptual Disorders"

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1/16. Tactile morphagnosia secondary to spatial deficits.

    A 73-year old man showed visual and tactile agnosia following bilateral haemorrhagic stroke. Tactile agnosia was present in both hands, as shown by his impaired recognition of objects, geometrical shapes, letters and nonsense shapes. Basic somatosensory functions and the appreciation of substance qualities (hylognosis) were preserved. The patient's inability to identify the stimulus shape (morphagnosia) was associated with a striking impairment in detecting the orientation of a line or a rod in two- and three-dimensional space. This spatial deficit was thought to underlie morphagnosia, since in the tactile modality form recognition is built upon the integration of the successive changes of orientation in space made by the hand as it explores the stimulus. Indirect support for this hypothesis was provided by the location of the lesions, which could not account for the severe impairment of both hands. Only those located in the right hemisphere encroached upon the posterior parietal cortex, which is the region assumed to be specialised in shape recognition. The left hemisphere damage spared the corresponding area and could not, therefore, be held responsible for the right hand tactile agnosia. We submit that tactile agnosia can result from the disruption of two discrete mechanisms and has different features. It may arise from a parietal lesion damaging the high level processing of somatosensory information that culminates in the structured description of the object. In this case, tactile recognition is impaired in the hand contralateral to the side of the lesion. Alternatively, it may be caused by a profound derangement of spatial skills, particularly those involved in detecting the orientation in space of lines, segments and complex patterns. This deficit results in morphagnosia, which affects both hands to the same degree.
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2/16. 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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3/16. Naming of musical notes: a selective deficit in one musical clef.

    We investigated the ability to perform solfeggio, i.e. oral reading of musical notes in MP, a 65 year-old female professional musician, who, following a left temporoparietal ischemia, showed a complex pattern of amusia. The deficit on which we focused was her inability to read orally the bass (F) clef, often substituting it with the violin (G) clef. This problem could not be attributed to a lack of comprehension. The patient could in fact correctly perform on the piano the same sequences she erroneously read aloud; she was also able to correctly judge whether two strings, one in bass clef and the other in violin clef, represented the same sequence of notes. The problem seems to lie in the inability to retrieve note names keeping into account the clef-rule. It is hypothesized that, in the production of note names, this function requires the identification and application of syntactic-like information, in analogy with what is thought to happen in the retrieval of other words.
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4/16. Fast responses to neglected targets in visual search reflect pre-attentive processes: an exploration of response times in visual neglect.

    AE is a patient who suffered a right hemisphere stroke resulting in visual neglect symptoms. In the first experiment, AE neglected a single visual target that was present in half of the trials and appeared in variable and unpredictable positions on the computer screen. The contrast of the target to the screen's background was also varied. AE demonstrated severe neglect for left-sided targets, and yet his RTs to targets reported incorrectly as absent were faster than correct rejections and even right-sided hits. AEs fast "neglect" responses seem to indicate that the target was detected but that he remained unaware of its presence. Counter intuitively, his fast misses got faster as the discriminability of the target decreased. The possibility that fast responses to neglected targets reflected a guessing strategy, used proportionally to the degree of uncertainty of a target presence, was examined. AEs fast misses were indeed faster at lower level of contrast of the stimulus, but his error rate did not tend to approach the chance level as the guessing model would predict. In a second experiment, AE searched for the letter Z, present on half of the trials, among variable sets of distractor letters. In one condition the distractors were all O's and therefore differed from the target by an elementary feature. In the other condition, the distractors were various letters that differed from the target by combinations of features. The key finding was that fast responses to neglected targets occurred only in the simple feature search task and not in the complex features (conjunction) task. We interpret these findings as indicating that AEs pre-attentive processing can detect pop-out targets on the left-hand side, but that the attentional search is faulty and is aborted early. Hence, the patient's attentional system has an "early start" when "pop-out" forms are present, but can also fail to "grab" the detected target; consequently, by not attending to a stimulus, the patient remains unaware of its presence and will quickly respond "no" to present targets.
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5/16. Direction-specific motion blindness induced by focal stimulation of human extrastriate cortex.

    Motion blindness (MB) or akinetopsia is the selective disturbance of visual motion perception while other features of the visual scene such as colour and shape are normally perceived. Chronic and transient forms of MB are characterized by a global deficit of direction discrimination (pandirectional), which is generally assumed to result from damage to, or interference with, the motion complex MT /V5. However, the most characteristic feature of primate MT-neurons is not their motion specificity, but their preference for one direction of motion (direction specificity). Here, we report that focal electrical stimulation in the human posterior temporal lobe selectively impaired the perception of motion in one direction while the perception of motion in other directions was completely normal (unidirectional MB). In addition, the direction of MB was found to depend on the brain area stimulated. It is argued that direction specificity for visual motion is not only represented at the single neuron level, but also in much larger cortical units.
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6/16. Visuospatial hemi-inattention following cerebellar/brain stem bleeding.

    Neglect is a unilateral lack of responsiveness to stimuli caused by visuospatial hemi-inattention, a unilateral representation deficit and/or a unilateral hypokinesia. It results most frequently from right-hemisphere brain damage, particularly of the parietal lobe but also of the frontal cortex, the basal ganglia, the thalamus, and recently it has also been described after a cerebellar lesion. We report a patient with right-sided bleeding of the posterior inferior cerebellar artery, who developed a left-sided visual hemi-inattention. She had no visual field defects, yet she had problems detecting left-sided targets in visual extinction. Furthermore, she was impaired in detecting complex motion on the left side and targets in a fixation offset paradigm. Reactions to left-sided targets in covert shifts of attention were slowed in the invalid condition. Her text reading was impaired as she could not always find the initial word of the next line. However, she was aware of her deficit. Her visuoconstructive ability was normal and she gave no indication of tactile or acoustic extinction. As the cerebellar lesion was located in the right hemisphere and the inattention involved the left side of space, we suggest that the damage to the right brain stem led to a transient imbalance of the noradrenergic ascending activation system which may explain her hemi-inattention.
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7/16. Callosal neglect.

    BACKGROUND: According to the interhemispheric inhibition model of neglect, the uninjured hemisphere inhibits (via the corpus callosum) the injured hemisphere but the injured hemisphere can no longer inhibit the opposite hemisphere, which becomes hyperactive and produces an ipsilesional attentional bias. Alternatively, according to the compensation hypothesis, the uninjured hemisphere helps compensate for the damaged hemisphere, which is impaired in directing attention to contralateral stimuli. If the inhibition model of neglect is correct, callosal disconnection should reduce neglect. If the compensation model is correct, however, it may increase or induce neglect. PATIENT: A 32-year-old woman, at age 14 years, developed a right frontal astrocytoma and was treated with surgery and radiation but had a cardiopulmonary arrest secondary to aspiration. Subsequent imaging studies revealed damage to the frontal, parietal, and occipital regions of the right hemisphere and damage to the temporal region of the left hemisphere. After discharge, she was able to return to school and drive a car, without any evidence of neglect. About 10 years later, she developed complex partial and atonic seizures that were multifocal and medically intractable. She underwent a complete section of her corpus callosum at age 31 years. RESULTS: One year after the callosal section, she demonstrated (1) diminished spontaneous saccades to the left, hypometric leftward saccades, and left gaze impersistence; (2) left arm hemispatial limb akinesia; (3) unilateral spatial neglect; and (4) motor and cognitive impersistence. CONCLUSION: In patients with right hemisphere injury, callosal section may induce or enhance motor-intentional deficits and hemispatial neglect.
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8/16. The impact of right subcortical lesions on executive functions and spatio-cognitive abilities: a case study.

    Deep-seated cerebral lesions have been known to cause various cognitive impairments. In order to study the impact of these lesions on cognitive functioning, in particular on executive functions and spatial abilities, an investigation was carried out on a right-handed patient with lesions in the subcortical structures of the right hemisphere. Findings revealed a complex pattern of neuropsychological impairments that included a memory deficit, neglect and anosognosia. In addition, there was a severe executive function syndrome. Finally, observations show impairments in spatial abilities, usually attributed to right parietal damage. There was no obvious language deficit. The pattern of deficits was interpreted as a disconnection syndrome.
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9/16. Social processing deficits in agenesis of the corpus callosum: narratives from the Thematic Appreciation Test.

    Clinical observations suggest that individuals with agenesis of the corpus callosum (ACC) and normal IQ may have deficits in social intelligence. This study analyzed responses by normally intelligent individuals with ACC to pictures from the thematic apperception test. A rating system was developed to assess three elements of story-generation: story logic, social understanding, and common content. Six individuals with ACC (five complete and one partial; IQs > 85) were compared to eight controls matched for sex, age, and IQ. Based on independent rankings of story protocols by two raters, the five individuals with complete ACC were found to be significantly impaired on all three criteria. The one individual with partial ACC performed better than the majority of controls in all three domains. Results demonstrated that individuals with complete ACC are impaired in understanding socially complex scenes and generating appropriate narratives. Absence of the anterior corpus callosum appears to be important for this deficit.
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10/16. Grasping what is graspable: evidence from visual form agnosia.

    Patient DF has profound visual form agnosia. Despite this, she has no problem adjusting her finger-thumb grip aperture to the width of objects when reaching to grasp them. In a previous study, however, she was found to have great difficulty in scaling her grip aperture when attempting to grasp a transparent disc through two holes cut into it. This problem was attributed to a putative difference between the visual processing of size and distance in the brain, whereby DF retained the capacity for processing object size but not the separation between distinct elements such as holes. In the present study we have tested this idea more directly, and found no evidence to support such a distinction. Nonetheless, we replicated our earlier finding that DF is unable to produce normal prehension movements when attempting to grasp transparent stimuli by placing her digits into holes. We suggest that, whilst some simple objects offer themselves directly to the dorsal stream for grasping, an intact ventral stream is required to respond appropriately to more complex stimuli.
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