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["嗜银颗粒病的临床方面"]

[Clinical aspects of "argyrophilic grain disease"].

作者信息

Botez G, Schultz C, Ghebremedhin E, Bohl J, Braak E, Braak H

机构信息

J.-W.-Goethe-Universität, Frankfurt, Main.

出版信息

Nervenarzt. 2000 Jan;71(1):38-43. doi: 10.1007/s001150050005.

Abstract

Argyrophilic grain disease (AGD) is a frequently occurring degenerative illness of the aging human brain. It is accompanied by progressive pathological alterations of the cytsokeleton which are traceable to an abnormal phosphorylation of the microtubule associated tau protein. Histologically, it is possible with the help of suitable staining techniques to identify pathognomonic spindle-shaped cellular inclusions (argyrophilic grains). These cellular inclusions display a typical cortical as well as subcortical distribution pattern. The goal of the present study is the retrospective evaluation of the clinical findings from 53 individuals with neuropathologically demonstrable AGD-related changes of the brain. Nearly one-half of the cases (49%) was classifiable as demented in accordance with DSM IV-criteria. Moreover, the frequency of the dementia increased significantly in relation to the growing severity of the AGD-associated pathological cytoskeletal degeneration. These results confirm the assumption that AGD can cause degenerative changes ranging from cognitive impairment all the way to dementia. They also underscore the necessity of further prospective studies pertaining to the clinical aspects of this still enigmatic disease.

摘要

嗜银颗粒病(AGD)是一种在老年人脑中常见的退行性疾病。它伴随着细胞骨架的进行性病理改变,这可追溯到与微管相关的tau蛋白的异常磷酸化。在组织学上,借助合适的染色技术可以识别出具有诊断意义的纺锤形细胞内含物(嗜银颗粒)。这些细胞内含物呈现出典型的皮质及皮质下分布模式。本研究的目的是对53例经神经病理学证实有与AGD相关脑改变的个体的临床发现进行回顾性评估。根据《精神疾病诊断与统计手册》第四版标准,近一半的病例(49%)可归类为痴呆。此外,痴呆的发生率随着与AGD相关的病理性细胞骨架变性严重程度的增加而显著上升。这些结果证实了AGD可导致从认知障碍到痴呆等一系列退行性改变的假设。它们还强调了针对这种仍然神秘的疾病的临床方面进行进一步前瞻性研究的必要性。

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