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Visual object agnosia, prosopagnosia, achromatopsia, loss of visual imagery, and autobiographical amnesia following recovery from cortical blindness: case M.H.

A number of higher visual deficits accompanied by severe retrograde autobiographical memory loss following bilateral medial occipital infarctions are described in case M.H. Assessment of M.H.'s visual object agnosia and prospagnosia suggested that he was unable to integrate the elements of a percept to form a meaningful whole. This deficit may occur at the level the percept is encoded into the visual buffer and inspected. M.H. also describes a loss of visual memories, and it is hypothesized that this may similarly be a result of an inability to integrate the elements of the visual representation (e.g. of an object or face) following its generation from long-term visual memory store into the visual buffer. M.H.'s retrograde autobiographical memory loss is postulated to be a consequence of the severe impoverishment of episodic memories that must occur when events originally stored multimodally, must be recalled without any visual component.

Adult↗

The nature of tactile agnosia: a case study.

A chronic tactile agnosic with a small, MRI-documented left inferior parietal infarction underwent detailed somesthetic testing to assess (1) the acquisition of sensory data, (2) the manipulation of somatosensory percept and its association with previous knowledge, and (3) recognition occurring at a deeper taxonomic level. Results suggest that tactile agnosia can arise from faulty high-level perceptual processes, but that the ability to associate tactually defined objects and object parts with episodic memory can be preserved. Consistent with anatomic and physiologic studies in nonhuman primates, inferior parietal cortex (including Brodmann area 40, possibly area 39) appears to serve as a high-level somatosensory region.

Agnosia↗

Preserved visual imagery in visual form agnosia.

We investigated the ability of a patient (D.F.) with profound visual form agnosia to perform a variety of tasks requiring visual imagery. Despite her inability to discriminate between objects and patterns of different shapes, sizes, and orientations, D.F. showed quite normal visual imagery involving these same 'visual' properties when the images were drawn from long-term memory. Thus, she was able both to scan mental images in search of particular features and to form new images by combining several known images. While there is growing evidence that perception and imagery share common neural substrates, the fact that D.F. shows intact visual imagery in the face of a massive perceptual deficit in form vision challenges recent suggestions that these two psychological processes share common input pathways in early vision. It is suggested that regions in the occipitotemporal pathway may be important for the generation of visual images while regions in the posterior parietal system might be involved in the manipulation of these images.

Adult↗

Visuomotor sensitivity for shape and orientation in a patient with visual form agnosia.

We have previously demonstrated that a patient with visual form agnosia (DF), who is unable to report the orientation or size of visual targets, can nevertheless use these same visual attributes to control motor acts. In the first of three new experiments, we found that DF is able to grasp everyday tools and utensils proficiently (i.e. with a well-formed hand posture) but has difficulty in visually selecting the correct part of the object to grasp (e.g. the handle) for subsequent use of that object. A second experiment revealed that DF's visuomotor system is able to adjust concurrently to variations in both the size and orientation of target objects; when these visual attributes were both varied, she adjusted both her grip aperture and the orientation of her hand well in advance of target contact. These spared visuomotor abilities do not seem to extend to shape processing per se, however. In the final experiment we found that DF was insensitive to changes in the orientation of a cross-shaped object, where no single principal axis could be extracted to control orientation of the grasp. These observations extend our knowledge of DF's residual visuomotor abilities, and suggest limitations on the visual processing capacities of the human dorsal stream.

Adult↗

Correlation between EEG and auditory perceptual measures in auditory agnosia.

We describe a child who acquired a gradual sustained speech deficit for which no specific etiology was found, and who had an associated epileptogenic abnormality on EEG. Assessment of auditory perceptual skills and receptive language confirmed that gradual parallel improvement occurred with the EEG after therapeutic anticonvulsant blood levels were obtained. Prospective trials of anticonvulsant drugs in conjunction with serial measures of central auditory abilities are necessary to establish their value in the management of the linguistic deficit in children with auditory agnosia.

Agnosia↗

Self-monitoring behavior in a case of severe auditory agnosia with aphasia.

This case report describes an unusual combination of speech and language deficits secondary to bilateral infarctions in a 62-year-old woman. The patient was administered an extensive series of speech, language, and audiologic tests and was found to exhibit a fluent aphasia in which reading and writing were extremely well preserved in comparison to auditory comprehension and oral expression, and a severe auditory agnosia. In spite of her auditory processing deficits, the patient exhibited unexpected self-monitoring ability and the capacity to form acoustic images on visual tasks. The manner in which she corrected and attempted to correct her phonemic errors, while ignoring semantic errors, suggests that different mechanisms may underlie the monitoring of these errors.

Agnosia↗

Auditory agnosia: apperceptive or associative disorder?

Neuropsychological testing of a patient with auditory agnosia showed that certain difficulties in the initial analysis of sounds may be the cause of his inability to understand spoken words and other sounds. Abnormalities included a slow reaction time to brief auditory stimuli (but not to equally brief visual stimuli or to longer auditory stimuli) and the need for approximately 1/4 sec of silence between two tones before the patient was able to hear them as separate. He could identify words and word associations if he was able to view the object whose name or word associate he was hearing. The findings imply that this patient's deficit in comprehending speech was probably apperceptive rather than associative in origin.

Agnosia↗

Subcortical auditory agnosia.

A case of generalized auditory agnosia without aphasia secondary to cardiogenic cerebral embolism is reported. The infarcts in this patient were localized within the bitemporal subcortices as confirmed by computerized axial tomography and magnetic resonance imaging. The findings suggested that interruption of both auditory radiations by bilateral subcortical lesions may play an important role in the occurrence of "cerebral auditory disorders."

Aged↗

Non-conscious face recognition in patients with face agnosia.

Four patients with face agnosia, a condition in which there is a failure to recognize previously known faces or faces that ought to have been learned without difficulty, were studied with an experimental paradigm in which both overt (verbal self-report) and covert (electrodermal skin conductance response) probes of recognition were used. The results showed a striking dissociation between the two indices: (1) all 4 patients generated more frequent and significantly larger-amplitude skin conductance responses to familiar faces, as compared to unfamiliar ones. This outcome contrasts with the finding that (2) none of the patients was able to give discriminatory verbal ratings of familiar versus unfamiliar faces. The positive electrodermal discrimination is interpreted as an index of the rich co-activation of records that is produced when familiar facial stimuli successfully trigger the reassembly of previously acquired representations of the physical structure of faces. The results of this co-activation, however, are unavailable to consciousness. This is seen as the consequence of lesion-related disruption of linkages between face records, on the one hand, and non-face records that contain information uniquely and unequivocally apposite to the possessor of a particular face.

Adult↗

Progressive visual agnosia with posterior cortical atrophy.

A patient of posterior cortical atrophy characterized by early signs of progressive visual agnosia documented by repeated neuropsychological tests, is reported. SPECT and MRI findings showed left unilateral parieto-occipital involvement in the earlier stage. A PET study executed eight months later showed bilateral parieto-occipital hypometabolism, but predominantly in the left hemisphere. This suggests that the degeneration may have developed asymmetrically, progressing from left unilateral to bilateral.

Agnosia↗

Left-right spatial agnosia and other mental defects that characterize clinical researchers.

There are mathematical rules for determining the logical significance of data, just as there are such rules for determining the statistical significance of data. Logical significance reflects the question a study is asking, whereas statistical significance reflects how well it has been answered. Clinical researchers routinely make an incorrect determination as to their study's logical significance because of a spatial (left-right) agnosia. More diffuse cognitive dysfunctions are encouraged by journals that expect clinicians to report on a study as a prerequisite for communicating their pre-existing opinions. The unwillingness or inability of the clinical community to acknowledge this problem reflects a related anosagnosia.

Agnosia↗

Agnosia.

Object recognition can break down in a variety of ways after brain damage. The resulting different forms of agnosia provide us with useful constraints on theories of normal object recognition. Recent studies suggest a division of labor for the recognition of different types of stimuli (common objects, words, faces, direction of eye gaze, spatial relations among parts of the human body), a high degree of interactivity in the processes underlying object recognition, and the possibility that recognition and awareness of recognition may be neurally distinct.

Agnosia↗

The role of sensory-motor information in object recognition: evidence from category-specific visual agnosia.

The role of sensory-motor representations in object recognition was investigated in experiments involving AD, a patient with mild visual agnosia who was impaired in the recognition of visually presented living as compared to non-living entities. AD named visually presented items for which sensory-motor information was available significantly more reliably than items for which such information was not available; this was true when all items were non-living. Naming of objects from their associated sound was normal. These data suggest that both information about object form computed in the ventral visual system as well as sensory-motor information specifying the manner of manipulation contribute to object recognition.

Acoustic Stimulation↗

Visuomotor performance in a patient with visual agnosia due to an early lesion.

We tested a patient with visual agnosia who had suffered severe bilateral brain damage early in life, on a series of visuomotor tasks. The broad pattern of results confirms that S.B., like the extensively tested patient D.F., shows an impressive array of preserved skills, despite his severe perceptual problems. Also like D.F., S.B. shows certain subtle visuomotor difficulties that can be related to the idea that his partially intact occipito-parietal areas are unable to benefit from interactions with the apparently severely damaged occipito-temporal regions. Unlike D.F., however, he is able to make accurate discriminations of simple visual features, such as object width and orientation, albeit with very slow response times. We hypothesize that several factors such as the early onset of S.B.'s lesion and the long period since his brain lesion have allowed his brain to compensate to a degree what has been impossible in D.F., whose brain damage occurred in adulthood. This may include an element of 'rewiring' and self-monitoring of visuomotor processes that allow S.B. to achieve perceptual access to visual information processed in the dorsal stream: information that is normally only available for on-line visuomotor control.

Adult↗

Normal pressure hydrocephalus manifesting as transient prosopagnosia, topographical disorientation, and visual objective agnosia.

Usually, dementia, gait disturbance and urinary incontinence are an integral part of the clinical presentation of normal pressure hydrocephalus (NPH). However, NPH with transient visual cognitive disorders has not been reported previously. We herein describe an extremely rare case of NPH that presented with transient visual cognitive disorders and long lasting visual memory disturbances that subsided after CSF shunting. A 38-years-old man developed transient prosopagnosia, topographical disorientation, color vision disturbance, and visual objective agnosia that progressed over 5 years. Magnetic resonance images showed ventriculomegaly with ischemic lesions in the paraventricular deep white matter. ECD-SPECT showed a reduction of the cerebral blood flow (CBF) in the periventricular deep white matter. Three months after CSF shunting, the radiological findings normalized with dramatic improvement of the extremely rare symptoms.

Adult↗

Preserved implicit form perception and orientation adaptation in visual form agnosia.

Visual form agnosia is mainly characterized by profound deficits in visual form and shape discrimination. Previous studies have shown that patients retain the capacity for coordinated motor behaviors, color naming and implicit letter perception. However, it is unknown to what extent other visual functions, such as implicit form and orientation perception, are preserved. To address these questions, we investigated a single visual form agnosic patient, X.F., in two distinct experiments. X.F.'s visual lesions were mainly localized in the bilateral occipitotemporal cortex, with the dorsal visual stream and early visual cortex largely spared. In Experiment 1, X.F. named the color of different forms across 12 blocks of trials. After the first six blocks, the combinations of a form with its color were changed and the new combination was presented for the remaining six blocks. X.F.'s reaction time increased during the switch block and was significantly greater than the overall RT changes between adjacent, non-switch blocks. This indicates that X.F. retained the ability to perceive changes in form despite her inability to discriminate the forms. In Experiment 2, X.F. showed selective orientation adaptation effects to different spatial frequencies; that is, her contrast threshold was significantly higher when the adapting and test orientations were the same than when they were orthogonal, although her orientation discrimination performance was severely impaired. These data provide evidence of a functional dissociation between explicit and implicit visual abilities, and suggest that the residual early visual cortex mediates form and orientation processing in the absence of awareness.

Adaptation, Physiological↗

No double-dissociation between optic ataxia and visual agnosia: multiple sub-streams for multiple visuo-manual integrations.

The current dominant view of the visual system is marked by the functional and anatomical dissociation between a ventral stream specialised for perception and a dorsal stream specialised for action. The "double-dissociation" between visual agnosia (VA), a deficit of visual recognition, and optic ataxia (OA), a deficit of visuo-manual guidance, considered as consecutive to ventral and dorsal damage, respectively, has provided the main argument for this dichotomic view. In the first part of this paper, we show that the currently available empirical data do not suffice to support a double-dissociation between OA and VA. In the second part, we review evidence coming from human neuropsychology and monkey data, which cast further doubts on the validity of a simple double-dissociation between perception and action because they argue for a far more complex organisation with multiple parallel visual-to-motor connections: 1. A dorso-dorsal pathway (involving the most dorsal part of the parietal and pre-motor cortices): for immediate visuo-motor control--with OA as typical disturbance. The latest research about OA is reviewed, showing how these patients exhibit deficits restricted to the most direct and fast visuo-motor transformations. We also propose that mild mirror ataxia, consisting of misreaching errors when the controlesional hand is guided to a visual goal though a mirror, could correspond to OA with an isolated "hand effect". 2. A ventral stream-prefrontal pathway (connections from the ventral visual stream to pre-frontal areas, by-passing the parietal areas): for "mediate" control (involving spatial or temporal transpositions [Rossetti, Y., & Pisella, L. (2003). Mediate responses as direct evidence for intention: Neuropsychology of Not to-, Not now- and Not there-tasks. In S. Johnson (Ed.), Cognitive Neuroscience perspectives on the problem of intentional action (pp. 67-105). MIT Press.])--with VA as typical disturbance. Preserved visuo-manual guidance in patients with VA is restricted to immediate goal-directed guidance, they exhibit deficits for delayed or pantomimed actions. 3. A ventro-dorsal pathway (involving the more ventral part of the parietal lobe and the pre-motor and pre-frontal areas): for complex planning and programming relying on high representational levels with a more bilateral organisation or an hemispheric lateralisation--with mirror apraxia, limb apraxia and spatial neglect as representatives. Mirror apraxia is a deficit that affects both hands after unilateral inferior parietal lesion with the patients reaching systematically and repeatedly toward the virtual image in the mirror. Limb apraxia is localized on a more advanced conceptual level of object-related actions and results from deficient integrative, computational and "working memory" capacities of the left inferior parietal lobule. A component of spatial working memory has recently been revealed also in spatial neglect consecutive to lesion involving the network of the right inferior parietal lobule and the right frontal areas. We conclude by pointing to the differential temporal constraints and integrative capabilities of these parallel visuo-motor pathways as keys to interpret the neuropsychological deficits.

Agnosia↗

Visual agnosia and Klüver-Bucy syndrome in marmosets (Callithrix jacchus) following ablation of inferotemporal cortex, with additional mnemonic effects of immunotoxic lesions of cholinergic projections to medial temporal areas.

Inferotemporal ablations in the New World monkey, the common marmoset (Callithrix jacchus), produced a persistent impairment on visual discrimination learning and a florid, but transient, Klüver-Bucy syndrome. Monkeys with these ablations were impaired on acquisition of object discriminations to a high criterion and on concurrent discrimination learning, to a single high criterion across all trials. Neither the control monkeys nor the monkeys with inferotemporal ablations found acquisition more difficult when the component discriminations of a set were presented concurrently compared to consecutively, although the monkeys with inferotemporal ablations found acquisition under both these conditions somewhat more difficult than did control monkeys. This suggests that the severe impairment caused by inferotemporal ablations on concurrent learning measured across all trials is due to the need for sustained performance across a concurrent set rather than to the extra mnemonic demands of concurrent presentation. When immunotoxic lesions of the cholinergic projection to the hippocampal formation were added to the inferotemporal ablations, a further impairment on retention, and a differential impairment on concurrent, compared to consecutive, learning was observed. Previous studies have shown that lesions of the cholinergic projection to the hippocampus alone, or excitotoxic hippocampal lesions, do not affect simple visual discrimination learning. It is suggested that large inferotemporal ablations in monkeys produce a visual agnosia which causes severe 'psychic blindness' in the first instance, and a persistent impairment on visual discrimination learning. The hippocampus makes a contribution, which may be mnemonic, to discrimination performance after inferotemporal ablations.

Acetylcholinesterase↗