Multiple System Atrophy: Advances in Diagnosis and Therapy.
Multiple System Atrophy: Advances in Diagnosis and Therapy.
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DOI：
10.14802/jmd.22082
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发表时间：
2023-01
影响因子：
3.9
通讯作者：
中科院分区：
文献类型：
--
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本文综述了多系统萎缩(MSA)的病理生理学和早期临床症状的研究进展，以及对MSA的诊断方法和疾病调整治疗的进展。自2008年提出第二项共识以来，运动障碍学会于2022年提出了新的诊断标准，以开发疾病修改疗法并促进MSA的临床试验。在发病机制方面，在α-突触核蛋白、神经炎症和少突胶质细胞与神经元的相互作用方面已经积累了尖端的发现。在神经成像方面，引入人工智能、机器学习和深度学习显著提高了诊断准确性和个体分析。治疗方面也取得了进展，包括针对α-突触核蛋白的免疫疗法以及5-羟色胺靶向和间充质干细胞疗法，这些疗法被认为影响疾病的几个方面，包括神经炎症。过去几年在阐明MSA发病机制方面的加速进展以及检测早期MSA的诊断技术的发展，有望促进针对这一最难治的神经退行性疾病之一的疾病修改疗法的发展。
This review summarizes improvements in understanding the pathophysiology and early clinical symptoms of multiple system atrophy (MSA) and advancements in diagnostic methods and disease-modifying therapies for the condition. In 2022, the Movement Disorder Society proposed new diagnostic criteria to develop disease-modifying therapies and promote clinical trials of MSA since the second consensus was proposed in 2008. Regarding pathogenesis, cutting-edge findings have accumulated on the interactions of α-synuclein, neuroinflammation, and oligodendroglia with neurons. In neuroimaging, introducing artificial intelligence, machine learning, and deep learning has notably improved diagnostic accuracy and individual analyses. Advancements in treatment have also been achieved, including immunotherapy therapy against α-synuclein and serotonin-targeted and mesenchymal stem cell therapies, which are thought to affect several aspects of the disease, including neuroinflammation. The accelerated progress in clarifying the pathogenesis of MSA over the past few years and the development of diagnostic techniques for detecting early-stage MSA are expected to facilitate the development of disease-modifying therapies for one of the most intractable neurodegenerative diseases.