Amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis.
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肌萎缩性侧硬化症。
DOI：
10.1016/s0140-6736(22)01272-7
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发表时间：
2022-10-15
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肌萎缩侧索硬化症（amyotrophiclateralsclerosis，ALS）是一种致死性中枢神经系统退行性疾病。尽管进行了大量的研究，但目前的ALS管理从诊断到预后仍然不理想。认识到ALS表型异质性，全球中枢神经系统功能障碍，遗传结构和新的诊断标准的发展，澄清了临床表现的频谱，并促进诊断。对ALS病理生理学的深入了解，疾病生物标志物和可改变风险的识别，沿着新的预测模型，量表和评分系统，以及基于机制的治疗的临床试验管道正在改变预后前景。虽然最新的进展尚未转化为患者的利益，但ALS作为一种复杂综合征的观点已经在临床上产生了切实的影响。本文将概述这些最新的见解，并讨论一般神经科医生的ALS管理的状态，沿着未来的前景，这可能会改善护理和ALS患者的结果。
Amyotrophic lateral sclerosis (ALS) is a fatal central nervous system neurodegenerative disease. Despite intense research, current ALS management remains suboptimal, from diagnosis to prognosis. Recognition of ALS phenotypic heterogeneity, global central nervous system dysfunction, genetic architecture, and development of novel diagnostic criteria are clarifying the spectrum of clinical presentation and facilitating diagnosis. Insights into ALS pathophysiology, identification of disease biomarkers and modifiable risks, along with new predictive models, scales, and scoring systems, and a clinical trial pipeline of mechanism-based therapies are changing the prognostic landscape. Although most recent advances have yet to translate to patient benefit, the view of ALS as a complex syndrome is already having tangible effects in the clinic. This review will outline these recent insights and discuss the status of ALS management for the general neurologist, along with future prospects, which may improve care and outcomes for ALS patients.