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在临床环境中预测迟发性非典型性溶血尿毒综合征(LATE-NC)的新标准:可能/疑似迟发性非典型性溶血尿毒综合征和晚期非典型性溶血尿毒综合征。

New criteria to predict LATE-NC in the clinical setting: Probable/Possible LATE and LANS.

作者信息

Nelson Peter T

机构信息

Sanders-Brown Center on Aging, Division of Neuropathology, Department of Pathology, University of Kentucky, Lexington, KY 40536, United States.

出版信息

J Neuropathol Exp Neurol. 2025 Jan 1;84(1):2-7. doi: 10.1093/jnen/nlae113.

Abstract

This review discusses terminology recently proposed for the classification of dementia and, more specifically, nosology related to aging-associated TDP-43 pathology: limbic-predominant age-related TDP-43 encephalopathy (LATE), and limbic-predominant amnestic neurodegenerative syndrome (LANS). While the "gold standard" for these clinical conditions is still LATE neuropathologic changes (LATE-NC), clinical criteria and biomarkers are evolving. The newly proposed clinical rubrics are discussed with emphasis on the need for terminology that acknowledges the distinctions between clinical syndrome-, molecular biomarker-, and pathologically defined disease concepts. As further progress is made on research into the specific biomarker-based detection and prediction of TDP-43 proteinopathy in the clinical setting, the definitions of "Probable" and "Possible" LATE are likely to become more useful clinically. For people interested in the pathological diagnoses or basic research related to LATE-NC, the relevant terminology remains unchanged by the newly proposed clinical criteria.

摘要

本综述讨论了近期提出的痴呆分类术语,更具体地说,是与衰老相关的TDP-43病理学相关的疾病分类:边缘叶为主的年龄相关性TDP-43脑病(LATE)和边缘叶为主的遗忘性神经退行性综合征(LANS)。虽然这些临床病症的“金标准”仍然是LATE神经病理学改变(LATE-NC),但临床标准和生物标志物正在不断发展。本文讨论了新提出的临床分类,重点强调了术语的必要性,该术语应承认临床综合征、分子生物标志物和病理定义的疾病概念之间的区别。随着在临床环境中基于特定生物标志物的TDP-43蛋白病检测和预测研究取得进一步进展,“可能的”和“疑似的”LATE定义在临床上可能会变得更有用。对于对LATE-NC相关病理诊断或基础研究感兴趣的人来说,新提出的临床标准并未改变相关术语。

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