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建立对肌缩侧面硬化症的诊断和差异诊断标准

Edyta Dziadkowiak¹, Karol Marschollek¹, Anna Kwaśniak-Nowakowska²

¹Clinical Department of Neurology, University Centre of Neurology and Neurosurgery, Faculty of Medicine, Wroclaw Medical University, Borowska 213, 50-556 Wroclaw, Poland.

Journal of clinical medicine

|January 10, 2026

概括

运动神经元疾病 (MND) 是一组影响运动神经元的渐进性神经退行性疾病. 本综述侧重于MND的诊断标准和差异诊断,特别是肌缩性侧面硬化症 (ALS).

科学领域:

神经学神经学
神经科学是一个神经科学.
运动神经元疾病运动神经元疾病

背景情况:

运动神经元疾病 (MND) 包含影响上部和/或下部运动神经元的异质性疾病.
关键的亚型包括肌缩性侧面硬化 (ALS),初级侧面硬化 (PLS),渐进性肌肉缩 (PMA) 和渐进性气泡 (PBP).
主要运动神经病变涉及前角细胞或巨型金字塔贝茨细胞的退行性损失.

研究的目的:

审查MND的诊断标准.
概述了MND的差异诊断.
专注于在MND频谱内的肌缩性侧面硬化症 (ALS).

主要方法:

对MND的诊断标准的文献综述.
对运动神经病变的差异诊断方法的分析.
专注于对肌缩性侧面硬化症 (ALS) 诊断的审查.

主要成果:

MND分类包括典型的形式,如ALS,PLS,PMA和PBP.
非典型的MND变体包括臂/腿综合征,FOSMN,FEWDON-MND和长期/青少年ALS.
诊断标准和差异诊断对于准确的患者管理至关重要.

结论:

准确诊断MND亚型,特别是ALS,是非常重要的.
了解罕见变异有助于差异诊断.
这一审查为诊断和区分MND提供了一个框架.

关键词:

肌缩侧面硬化症 (ALS) 是一种疾病.非典型的MND-ALS变体运动神经元疾病 (MND)超氧化物去位酶1 (SOD1) 突变

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