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首页> 外文期刊>Folia neuropathologica >On the 'classification' of neurodegenerative disorders: Discrete entities, overlap or continuum?
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On the 'classification' of neurodegenerative disorders: Discrete entities, overlap or continuum?

机译：关于神经退行性疾病的“分类”：离散实体，重叠或连续体？

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摘要

The traditional method of classifying neurodegenerative diseases is based on the original clinico-pathological concept supported by 'consensus' criteria and data from molecular pathological studies. This review discusses first, current problems in classification resulting from the coexistence of different classificatory schemes, the presence of disease heterogeneity and multiple pathologies, the use of 'signature' brain lesions in diagnosis, and the existence of pathological processes common to different diseases. Second, three models of neurodegenerative disease are proposed: (1) that distinct diseases exist ('discrete' model), (2) that relatively distinct diseases exist but exhibit overlapping features ('overlap' model), and (3) that distinct diseases do not exist and neurodegenerative disease is a 'continuum' in which there is continuous variation in clinical/pathological features from one case to another ('continuum' model). Third, to distinguish between models, the distribution of the most important molecular 'signature' lesions across the different diseases is reviewed. Such lesions often have poor 'fidelity', i.e., they are not unique to individual disorders but are distributed across many diseases consistent with the overlap or continuum models. Fourth, the question of whether the current classificatory system should be rejected is considered and three alternatives are proposed, viz., objective classification, classification for convenience (a 'dissection'), or analysis as a continuum.
机译：对神经退行性疾病进行分类的传统方法是基于原始的临床病理学概念，并得到“共识”标准和分子病理学研究数据的支持。这篇综述首先讨论了由于不同分类方案的共存，疾病异质性和多种病理的存在，诊断中使用“特征性”脑病变以及不同疾病共有的病理过程而导致的当前分类问题。其次，提出了三种神经退行性疾病模型：（1）存在不同的疾病（“离散”模型）;（2）存在相对不同的疾病但呈现出重叠特征（“ overlap”模型）;（3）神经退行性疾病不存在，神经退行性疾病是一种“连续体”，其中临床/病理特征从一种情况到另一种情况都存在连续变化（“连续体”模型）。第三，为了区分模型，回顾了不同疾病中最重要的分子“特征”病变的分布。这样的病变通常具有较差的“保真度”，即它们不是个别疾病所独有的，而是分布在许多与重叠或连续模型一致的疾病中。第四，考虑是否应拒绝当前分类系统的问题，并提出了三种选择，即目标分类，方便分类（“剖析”）或作为连续体进行分析。

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