Cases reported "Dementia"

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1/13. knowledge of the human body: a distinct semantic domain.

    BACKGROUND: patients with selective deficits in the naming and comprehension of animals, plants, and artifacts have been reported. These descriptions of specific semantic category deficits have contributed substantially to the understanding of the architecture of semantic representations. OBJECTIVE: This study sought to further understanding of the organization of the semantic system by demonstrating that another semantic category, knowledge of the human body, may be selectively preserved. methods: The performance of a patient with semantic dementia was compared with the performance of healthy controls on a variety of tasks assessing distinct types of body representations, including the body schema, body image, and body structural description. RESULTS: Despite substantial deficits on tasks involving language and knowledge of the world generally, the patient performed normally on all tests of body knowledge except body part naming; even in this naming task, however, her performance with body parts was significantly better than on artifacts. CONCLUSIONS: The demonstration that body knowledge may be preserved despite substantial semantic deficits involving other types of semantic information argues that body knowledge is a distinct and dissociable semantic category. These data are interpreted as support for a model of semantics that proposes that knowledge is distributed across different cortical regions reflecting the manner in which the information was acquired.
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2/13. Dissociating person-specific from general semantic knowledge: roles of the left and right temporal lobes.

    The cognitive architecture and neural underpinnings of different semantic domains remains highly controversial. We report two patients with focal temporal lobe atrophy who presented with contrasting and theoretically informative dissociations of person-specific versus general semantic knowledge. Subject J.P. showed severely impaired person-specific semantics, with relative preservation of knowledge about objects and animals, while subject M.A. exhibited the opposite pattern of performance (good knowledge of people in the context of impoverished general semantics). Voxel-based morphometric analysis of MR images in the two cases established predominantly right temporal atrophy associated with J.P.'s deficit for person knowledge and predominantly left temporal atrophy in M.A. who was impaired in general conceptual knowledge.
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3/13. Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy.

    We report five patients with a stereotyped clinical syndrome characterized by fluent dysphasia with severe anomia, reduced vocabulary and prominent impairment of single-word comprehension, progressing to a stage of virtually complete dissolution of the semantic components of language. A marked reduction in the ability to generate exemplars from restricted semantic categories (e.g. animals, vehicles, etc.) was a consistent and early feature. Tests of semantic memory demonstrated a radically impoverished knowledge about a range of living and man-made items. In contrast, phonology and grammar of spoken language were largely preserved, as was comprehension of complex syntactic commands. reading showed a pattern of surface dyslexia. Autobiographical and day-to-day (episodic) memory were relatively retained. Non-verbal memory, perceptual and visuospatial abilities were also strikingly preserved. In some cases, behavioural and personality changes may supervene; one patient developed features of the kluver-bucy syndrome. Radiological investigations have shown marked focal temporal atrophy in all five patients, and functional imaging by single positron emission tomography and positron emission tomography (one case) have implicated the dominant temporal lobe in all five. In the older literature, such cases would have been subsumed under the rubric of Pick's disease. Others have been included in series with progressive aphasia. We propose the term semantic dementia, first coined by Snowden et al. (1989), to designate this clinical syndrome.
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4/13. On the decline and etiology of high-incidence motor system disease in West Papua (southwest new guinea).

    The etiology of a high-incidence focus of amyotrophic lateral sclerosis and parkinsonism-dementia (ALS/P-D) in south West Papua (Irian Jaya, indonesia), first described in the 1960s and 1970s, has been attributed to mineral deficiencies, hyperparathyroidism, and metal neurotoxicity arising from reliance on drinking water obtained from springs and shallow wells. More recent visits (1987 and 1990) to the south West Papua focus of neurodegenerative disease cast doubt on this explanation by revealing changes in disease prevalence in communities with an unchanged water supply. These communities have experienced a dramatic decline in ALS and a reversal in the relative prevalence of ALS and parkinsonism. The extrapyramidal disorder can be distinguished from parkinson disease by pyramidal features (and dementia) reminiscent of guam P-D. Topical use of cycad seed (termed kurru) gametophyte to treat large skin lesions is advanced as a plausible but unproven etiologic factor. Medicinal use of untreated cycad seed (cycas sp.) has also been linked with ALS foci in japan (oral use) and guam (topical use), with the additional consumption on guam of food items prepared from cycas sp. seed or animals that consume cycad seed components.
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5/13. How Berlusconi keeps his face: a neuropsychological study in a case of semantic dementia.

    A patient (V.Z.) is described as being affected by progressive bilateral atrophy of the mesial temporal lobes resulting in semantic dementia. Vis-a-vis virtually nil recognition of even the most familiar faces (including those of her closest relatives) as well as of objects and animals, V.Z. could nevertheless consistently recognize and name the face of Silvio Berlusconi, the mass media tycoon and current Italian Prime Minister. The experimental investigation led to the conclusion that Mr Berlusconi's face was seen as an icon rather than as a face. This telling effect of Mr Berlusconi's pervasive propaganda constitutes an unprecedented case in the neuropsychological literature.
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6/13. Progressive language impairment without dementia: a case with isolated category specific semantic defect.

    A patient is described with a 5 year progressive defect of naming and auditory verbal comprehension, the pathological nature of which was presumably degenerative. The auditory comprehension defect unevenly affected different semantic categories, and was particularly severe for the names of animals, fruits and vegetables. The patients showed loss of the verbal knowledge of the physical attributes of the concepts corresponding to the words he was unable to understand, and sparing of the verbal knowledge of the functional attributes. His performance was defective also on the colour-figure and sound-picture matching test.
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7/13. Familial presenile dementia with CJD-like lesions: preliminary results.

    A case of an unknown type of familial presenile dementia is reported. The patient was a 56-year-old female, whose main clinical symptom was a gradually progressive dementia over 16 years. She had no myoclonus or periodic synchronous discharge in the EEG. The CT scans revealed marked cerebral atrophy with prominent atrophy of the cerebral white matter. One of her sisters is thought to suffer from the same disease, and is now in an apallic state. The patient was clinically diagnosed as having familial Alzheimer's disease. The neuropathological features consisted of severe cerebral cortical degeneration with conspicuous proliferation of gemistocytic astrocytes as well as severe cerebral white matter degeneration. These cerebral lesions are most similar to those of the panencephalopathic type of Creutzfeldt-Jakob's disease (CJD). However, our case differs from it in that the cerebellar degeneration was very mild. Transmission experiments of frozen tissue from the patient into animals are being tried. The neurochemical data suggested cholinergic deficits. As far as we know, such a case has never been reported in the literature.
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8/13. Thalamic dementia and motor neuron disease.

    A 46-year-old woman developed a progressive neurologic disorder over the course of 30 months which was characterized by profound dementia complicated by a motor neuron disorder that became evident 10 months prior to death. Postmortem examination of the nervous system disclosed extensive neuronal loss and gliosis of the thalamus, predominantly involving the dorsomedial nuclei, as well as severe degeneration of the corticospinal tracts, spinal anterior horns, and hypoglossal nuclei. The disease could not be transmitted to experimental animals by intracerebral inoculation of the patient's brain tissue. This case represents a unique dementing disorder, possibly familial, with associated motor neuron disease.
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9/13. An investigation of the effects of intracerebral injection in the marmoset of cytopathic cerebrospinal fluid from patients with schizophrenia or neurological disease.

    In experiments designed to investigate transmission, cerebrospinal fluid (CSF) from patients with schizophrenia and neurological disease (huntington's chorea and multiple sclerosis) which had been found to induce cytopathic effects in human embryonic fibroblast cell culture was injected intracerebrally into mice, hamsters and marmosets (small New World primates). No evidence was obtained of transmission to mice or hamsters. A total of 15 marmosets (callithrix jacchus) was injected intracerebrally with CSF [8 with samples from 4 patients with schizophrenia. 3 with samples from patients with neurological disease (2 with Huntington's chorea and 1 with multiple sclerosis) and 4 with samples from 3 patients without neurological or psychiatric disease] and was observed over a period of 2 1/2 years. analysis of variance on data obtained from behavioral observations averaged over 6-month periods revealed that animals injected with CSF from patients with schizophrenia and neurological disease became progressively more inactive when compared with animals injected with CSF from control patients. The change detected by behavioural observation was confirmed as a difference 2 and 2 1/2 years after injection by automated activity monitoring. There was an incidence of reproductive anomalies (including two occipital encephalocoeles) in the females in the experimental group, but the numbers are too small to draw firm conclusions from this observation. Many reported differences in biological samples from schizophrenic patients and normal controls have subsequently been found to be due to factors unrelated to the disease state. This may prove to be the case with the changes observed in this experiment. Nevertheless, the fact that marmosets injected with CSF from patients suffering from neuropsychiatric disease, including schizophrenia, subsequently differed in their behaviour from those injected with control CSF warrants further investigation.
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10/13. Alzheimer's disease and transmissible virus dementia (Creutzfeldt-Jakob disease).

    Ample justification exists on clinical, pathologic, and biologic grounds for considering a similar pathogenesis for AD and the spongiform virus encephalopathies. However, the crux of the comparison rests squarely on results of attempts to transmit AD to experimental animals, and these results have not as yet validated a common etiology. Investigations of the biologic similarities between AD and the spongiform virus encephalopathies proceed in several laboratories, and our own observation of inoculated animals will be continued in the hope that incubation periods for AD may be even longer than those of CJD.
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