Cases reported "Alzheimer Disease"

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1/90. A familial case of Alzheimer's disease without tau pathology may be linked with chromosome 3 markers.

    Alzheimer's disease is the most common form of dementia that occurs in later years. The diagnosis is confirmed by the pathological findings of betaA4-amyloid-containing neuritic plaques and neurofibrillary tangles, the former being present in sufficient quantity commensurate with age. Other forms of dementia are more difficult to diagnose clinically; their pathology is noted for the lack of plaques and tangles. A patient with a family history of dementia presented with the clinical signs of Alzheimer's disease which lasted for 13 years. At autopsy the brain tissue had betaA4-amyloid-containing neuritic plaques, but no neurofibrillary tangles (i.e., the tissue was negative for staining with the tau antibody). Genetic analysis of dna from family members revealed no linkage with chromosome 17 markers, indicating that this was not frontotemporal dementia. However, there was linkage with chromosome 3 markers. Thus, this form of Alzheimer's disease with a pathology of plaques only is linked with markers on chromosome 3.
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2/90. Cerebrovascular pathology in Alzheimer's disease and leukoaraiosis.

    A high percentage of patients with Alzheimer's disease (AD) show evidence of white matter degeneration known as leukoaraiosis (LA), which is due to chronic ischemia. We found that the periventricular veins tend to become occluded by multiple layers of collagen in the vessel walls in the elderly. This collagen deposition is particularly excessive in LA lesions. Therefore, it is present in the brains of many AD patients, along with other ischemia-causing cerebrovascular pathology. We found evidence that there is severe loss of oligodendrocytes in LA, due to extensive apoptosis. No evidence of inflammation was found in the LA lesions. In thick celloidin sections of AD brain, we have obtained detailed 3D views of small (early) deposits of amyloid (stained with beta-amyloid antibody) around capillaries (stained with collagen IV antibody).
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3/90. An autopsy case of Alzheimer's disease presenting with primary progressive aphasia: a clinicopathological and immunohistochemical study.

    This report describes an autopsied Alzheimer's disease (AD) patient with primary progressive aphasia (PPA) as an early symptom. The patient developed a progressive speech disturbance at the age of 70 years, and difficulty in comprehension became apparent 2 years later. magnetic resonance imaging scan disclosed asymmetrical brain atrophy, predominantly on the left temporal lobe. At the age of 74 years, the patient's dementia rapidly progressed with parkinsonism and he died after a disease duration of 6 years. At autopsy, the brain showed a marked temporo-frontal lobe atrophy, predominantly on the left side. There was severe neuronal loss with gliosis and tissue rarefaction in the atrophied cerebral cortex and amygdala. Many neurofibrillary tangles with neuropil threads were found in the cerebral cortex. Numerous amyloid deposits were distributed throughout the cerebral cortex, accompanied by amyloid angiopathies. This patient was clinically diagnosed with temporal lobe-dominant Pick's disease, although the possibility of corticobasal degeneration was made. The neuropathological diagnosis was AD with asymmetrical brain atrophy and widespread amyloid angiopathies.
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keywords = parkinsonism
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4/90. cholinesterase inhibitors. Comparing the options for mild-to-moderate dementia.

    cholinesterase inhibitors can be used as one element of a comprehensive approach to management of mild-to-moderate AD. Benefits include modest cognitive improvement, increased activation, and improved mood and behavior. patients with other disorders, such as Lewy-body dementia, may also improve. The most common side effects include GI disturbances, insomnia, dizziness, fatigue, and muscle cramps. Adverse effects can be significantly reduced by waiting 4 to 6 weeks before increasing doses. Insomnia may be alleviated by having the patient take the medication early in the day.
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5/90. 11: disorders of memory and intellect.

    The clinical approach to the patient with a suspected disorder of memory and intellect is to establish whether it is dementia, which parts of the brain are affected, what is the cause, what is the prognosis, and what can be done about it. The diagnosis of dementia usually requires the involvement of memory and at least one other cognitive system. delirium and depression are important differential diagnoses. patients with dementia should usually have some simple investigations after a careful history-taking and examination to identify "reversible" causes. The commonest cause of dementia is Alzheimer's disease, in which short-term memory disturbance is usually prominent. Other causes of dementia include cerebrovascular disease, Lewy-body disease and Pick's disease. There is now hope for patients with Alzheimer's disease (which can be treated with some success with cholinesterase inhibitors) and patients with vascular dementia, in whom aggressive control of causal risk factors may retard progression.
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6/90. Lewy body dementia: case report and discussion.

    BACKGROUND: Lewy body dementia is a common but frequently underdiagnosed cause of dementia often mistaken for the more familiar entity of alzheimer disease. Clinically the distinction is important, because it can have profound implications for management. methods: The medical literature was searched using the keywords "lewy bodies," "Lewy body dementia," "Alzheimer dementia," and "parkinsonian disorders." A case of Lewy body dementia is described. RESULTS: An elderly man had long-standing diagnoses of alzheimer disease and parkinson disease. After he was evaluated thoroughly, the diagnosis was revised to Lewy body dementia, leading to changes in treatment that were associated with dramatic improvement in the patient's mental status. Evidence from the literature suggests that Lewy body dementia can be diagnosed in primary care settings based on clinical criteria. The physician should be alert to this diagnosis, and special attention should be paid to dementia patients who exhibit parkinsonism, hallucinations, fluctuating cognition, or prominent visuosperceptual deficits. CONCLUSIONS: The diagnosis of Lewy body dementia has important implications. It is associated with a high incidence of neuroleptic sensitivity, necessitating great caution in the use of these common antipsychotic agents. Early studies indicate cholinesterase inhibitors can be beneficial for treating the hallucinations and behavior disturbances that afflict these patients and might also improve cognition.
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ranking = 64.679022451115
keywords = parkinsonism, body
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7/90. A novel tau mutation, S320F, causes a tauopathy with inclusions similar to those in Pick's disease.

    Mutations in the tau gene cause familial frontotemporal dementia and parkinsonism linked to chromosome 17. In this article, we describe a novel missense mutation, S320F, in the tau gene in a family with presenile dementia. To our knowledge, it is the first mutation to be described in exon 11 of tau. The proband died at age 53 years, after a disease duration of 15 years, and autopsy revealed a neuropathological picture similar to Pick's disease. Recombinant tau protein with the S320F mutation showed a greatly reduced ability to promote microtubule assembly.
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ranking = 54.679022451115
keywords = parkinsonism
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8/90. Congophilic angiopathy complicated by surgery and massive hemorrhage. A light and electron microscopic study.

    Three autopsied cases of congophilic angiopathy have been studied by means of polarized light microscopy and in 2 of these, biopsy material is available for electron microscopic study. All 3 patients had a surgical procedure and a subsequent massive hemorrhagic episode. Two of these patients had clinical evidence of dementing syndrome, for which shunts were performed. Ultrastructural studies have confirmed the amyloid nature of the congophilic material in the 2 biopsied cases. The deposition of amyloid in these cases is believed to be a primary event and is related to a generalized body disorder. The distinction of congophilic angiopathy from Alzheimer's disease is discussed.
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9/90. Mapping the C terminal epitope of the Alzheimer's disease specific antibody MN423.

    The mapping of monoclonal antibody epitopes is now predominantly carried out using molecular diversity techniques, phage display in particular. However, until very recently, phage display methods have been inappropriate for the analysis of epitopes that require a free carboxy terminus. Here we describe the use of two different techniques to analyze the known C terminal epitope specificity of MN423, a monoclonal antibody specifically staining truncated tau in Alzheimer's brain. Using a lambda phage based C-terminal random peptide library, and an intracellular expression library based on truncated tau, we show that this antibody has an absolute requirement for a glycine at position -3 with respect to the C terminus. Both methods give similar results, and identify other important residues in the binding site. However, affinity analysis of synthetic peptides revealed that the affinity of the antibody for identified tripeptides was far lower than the pentapeptide sequence in the native target, and that this in turn was considerably below the affinity for the native target itself. This suggests that molecular diversity methods may define minimum, but not necessarily complete epitopes. The methods described here have a general application to the analysis of antibody epitopes suspected to be found at the C terminus.
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10/90. Parkinson's disease mimicking senile dementia of the Alzheimer type: a clinicopathological study of four autopsy cases.

    This report concerns four Japanese autopsy cases of Parkinson's disease (PD) mimicking senile dementia of the Alzheimer type. Three patients with a clinical diagnosis of senile dementia of the Alzheimer type developed memory disturbance as the initial sign, and a patient with a clinical diagnosis of atypical senile dementia presented with hallucination and delusion as the initial sign. Dementia was evident in all four patients, and slight parkinsonism appeared in the middle to late stages of the disease in two patients. Macroscopical examination of the brain disclosed slight depigmentation of the substantia nigra and prominent depigmentation of the locus ceruleus in all four cases. Histological examination of the four patients showed neuronal loss with astrocytosis and the appearance of lewy bodies in the substantia nigra, locus ceruleus, and dorsal vagal nucleus. The nucleus basalis of Meynert was involved in three cases, in which this structure was examined. The total Lewy body scores of the four cases were 1 in three cases and 0 in the other, compatible with PD. Massive appearance of senile plaques, consistent with Braak stage C, was found in one case, and the slight appearance of senile plaques, consistent with Braak stage A, was evident in two cases. One case had no evidence of senile plaques. In all four cases, slight neurofibrillary changes were present in the limbic areas, compatible with Braak stages II to III. Based on these clinicopathological findings and a review of the literature, we concluded that PD simulating Alzheimer's disease without overt parkinsonism rarely exists. Furthermore, we postulate that the clinical features of PD are more widespread than previously believed.
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ranking = 110.35804490223
keywords = parkinsonism, body
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