31/57. Cortical Lewy body dementia: clinical features and classification.Seven patients, aged 65-72 years, are described with dementia and cortical lewy bodies. In one patient a Parkinsonian syndrome was followed by dementia and motor neuron disease. In the remaining six patients dementia was accompanied by dysphasia, dyspraxia and agnosia. One developed a Parkinsonian syndrome before the dementia, in three cases a Parkinsonian syndrome occurred later, and in two cases not at all. All patients showed lewy bodies and cell loss in the substantia nigra, locus coeruleus and dorsal vagal nucleus, as in Parkinson's disease. The severity of cell loss in the nucleus basalis varied from mild to severe. lewy bodies were also present in the parahippocampus and cerebral cortex, but Alzheimer-type pathology was mild or absent, and insufficient for a diagnosis of Alzheimer's disease. patients with moderate or severe dementia, some with temporal or parietal features, may have cortical lewy body disease, Alzheimer's disease, or a combination of the two. Cortical lewy body disease may be associated with dementia in Parkinson's disease more often than realised, but is not necessarily associated with extensive Alzheimer pathology.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
32/57. dementia in Parkinson's disease: the problem of clinicopathological correlation.Four cases of Parkinson's disease with advanced dementia are described. Postmortem examination revealed cell loss in the substantia nigra, with lewy bodies present, and loss of cells in the basal nucleus of meynert. A few tangles were observed in the hippocampus, but no senile plaques or neurofibrillary tangles were found in the neocortex. The authors note that a dramatic dementia syndrome may occur with Parkinson's disease alone, without the associated cytoskeletal markers of Alzheimer's disease. Cases were characterized by disorientation, episodic confusion and hallucinations persisting off medication, disturbed behavior, and the absence of aphasia.- - - - - - - - - - ranking = 0.5keywords = nucleus (Clic here for more details about this article) |
33/57. ataxia, chorea, seizures, and dementia. Pathologic features of a newly defined familial disorder.Five generations of a family exhibit a unique autosomal dominant neurologic disorder characterized by the development (usually between 15 and 30 years of age) of ataxia, seizures, choreiform movements, progressive dementia, and death after 15 to 25 years of illness. Neuropathologic findings in two deceased family members revealed remarkably similar findings, including marked neuronal loss of the dentate nucleus, microcalcification of the globus pallidus, neuroaxonal dystrophy of the nucleus gracilis, and demyelination of the centrum semiovale. The clinical and pathologic findings are closely correlated. ataxia and chorea are related to severe neuronal loss in the dentate nucleus with calcification in the globus pallidus. dementia occurs with progressive demyelination of the centrum semiovale, and loss of posterior column function occurs with neuroaxonal dystrophy of the nucleus gracilis and nucleus cuneatus.- - - - - - - - - - ranking = 2.5keywords = nucleus (Clic here for more details about this article) |
34/57. dementia without Alzheimer pathology.We studied five demented patients who, on neuropathologic examination, had cell loss and lewy bodies in substantia nigra and locus ceruleus and few Alzheimer-type changes. The nucleus basalis had minimal cell loss in three patients and was not available in two. The lesions in the substantia nigra and locus ceruleus were unlikely to account for the dementia, and other structural or biochemical derangements, probably cortical but possibly subcortical, must also have been present but not visible at the light microscopic level.- - - - - - - - - - ranking = 0.5keywords = nucleus (Clic here for more details about this article) |
35/57. Lewy body dementia without Alzheimer changes.Three patients with clinical Alzheimer's disease were found at postmortem examination to have Lewy-bodies and Lewy-like bodies in the cerebral cortex and the pigmented brainstem nuclei. Neuritic plaques were found in neocortical areas but no neurofibrillary tangles. The distribution of cortical neuronal inclusions correlated with the proposed projection of dopamine terminals. Neuronal cell loss was marked in the ventral tegmental area (paranigral nucleus) and the basal nucleus of meynert, suggesting a defect in dopaminergic and cholinergic innervation of the cerebral cortex. Immunohistochemical investigations revealed positive staining of cortical Lewy- and Lewy-like bodies for monoclonal antibodies to phosphorylated neurofilaments (03-44, 06-17, 04-7). Also cerebral neurons containing no inclusions showed positivity, suggesting an early neurofilament abnormality, preceding the formation of Lewy-type inclusions.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
36/57. High-resolution nuclear magnetic resonance imaging and single photon emission computerized tomography--cerebral blood flow in a case of pure sensory stroke and mild dementia owing to subcortical arteriosclerotic encephalopathy (Binswanger's disease).Pure sensory stroke (PSS) is typically caused by a lacunar infarct located in the ventral-posterior (VP) thalamic nucleus contralateral to the paresthetic symptoms. The lesion is usually so small that it cannot be seen on computerized tomography (CT), as illustrated by our case. In our moderately hypertensive, 72-year-old patient with PSS, CT scanning and conventional nuclear magnetic resonance imaging (NMRI) scanning using a 7-mm-thick slice on a 1.5 Tesla instrument all failed to visualize the thalamic infarct. Using the high-resolution mode with 2-mm slice thickness it was, however, clearly seen. In addition, NMRI unexpectedly showed diffuse periventricular demyelinization as well as three other lacunar infarcts, i.e., findings characteristic of subcortical arteriosclerotic encephalopathy (SAE). This prompted psychometric testing, which revealed signs of mild (subclinical) dementia, in particular involving visiospatial apraxia; this pointed to decreased function of the right parietal cortex, which was structurally intact on CT and NMRI. Single photon emission computerized tomography by xenon-133 injection and by hexamethyl-propyleneamine-oxim labeled with technetium-99m showed asymmetric distribution of cerebral blood flow (CBF), with an 18% lower value in the right parietal cortex compared to the left side; this indicated asymmetric disconnection of the cortex by the SAE. Thus, the tomograms of the functional parameter, CBF, correlated better with the deficits revealed by neuropsychological testing than by CT or NMRI.- - - - - - - - - - ranking = 0.5keywords = nucleus (Clic here for more details about this article) |
37/57. Pathologic correlates of dementia in Parkinson's disease.Histopathologic studies of the cerebral cortex, hippocampus, and three subcortical nuclei were performed in four patients with Parkinson's disease whose mental status had been evaluated by neuropsychologic testing. Clinicopathologic correlations suggest that dementia with marked visuospatial disturbance as well as severe aphasia may be associated with severe neuronal loss in subcortical nuclei, without significant numbers of plaques or tangles in the hippocampus and cerebral cortex. Furthermore, memory loss may not be consistently related to neuronal loss in the nucleus basalis of Meynert, particularly in non-Lewy body parkinsonism.- - - - - - - - - - ranking = 0.5keywords = nucleus (Clic here for more details about this article) |
38/57. Unusual cases of presenile dementia with Fahr's syndrome.Three patients with an unusual type of presenile dementia were studied. Atypical clinical pictures emerged from an evaluation of these cases. Their symptoms and signs were thought to be neither those of Alzheimer's disease nor those of Pick's disease but a partial mixture of those of both diseases. The neuropathological changes were characteristic and the common findings were as as follows: the absence of senile plaques, the widespread presence of numerous neurofibrillary tangles, a calcareous deposition of Fahr's type, a circumscribed cerebral atrophy in the temporal or/and frontal lobes, a moderate or severe demyelination and fibrous gliosis in the white matter of the atrophied areas, and a slight or moderate neuronal loss in the nucleus basalis of Meynert. Similar cases reported previously were reviewed.- - - - - - - - - - ranking = 0.5keywords = nucleus (Clic here for more details about this article) |
39/57. Cortical degeneration with swollen chromatolytic neurons: its relationship to Pick's disease.We report two cases of dementia in which cortical degeneration with widespread swollen chromatolytic neurons (SCN) was the dominant pathologic feature. Each patient had received the diagnosis of Alzheimer's disease on the basis of clinical findings. There was no deficit of cortical choline acetyltransferase activity, assayed in one case, or lesions of the nucleus basalis of Meynert. The brains had moderate to marked frontal atrophy. Comparison of SCN with several other cerebral degenerative disorders indicates a similarity with certain features of the transmissible spongiform encephalopathies and with corticodentatonigral degeneration. The pathologic features of our cases are those of a number of other cases reported as "Pick's disease," and may represent an earlier stage in the pathogenetic process than the severe, sharply circumscribed atrophy with "nonspecific" cell loss and gliosis as the only microscopic residuals. Our findings re-emphasize the need to search for pathogenetically distinct subgroups which have been wholly or partially subsumed into the concept of Pick's disease.- - - - - - - - - - ranking = 0.5keywords = nucleus (Clic here for more details about this article) |
40/57. dementia with cerebral lewy bodies. A mesocortical dopaminergic defect?We describe a patient with dementia who at postmortem examination was found to have Lewylike bodies throughout the cerebral cortex associated with typical Lewy bodies in the pigmented brainstem nuclei. Moderate numbers of senile plaques but no neurofibrillary tangles were found in the neopallium or in the limbic areas. A positive correlation was found between the distribution of the cortical Lewylike bodies and the proposed distribution of dopamine terminals in the cerebral cortex. Cell loss in the ventral tegmental area and the basal nucleus of meynert suggest abnormalities in the dopaminergic and cholinergic innervation of the cerebral cortex. The relative lack of senile changes of Alzheimer type make these two proposed abnormalities a more plausible explanation for the dementia in this patient.- - - - - - - - - - ranking = 0.5keywords = nucleus (Clic here for more details about this article) |
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