Watanabe Hirohisa, Shima Sayuri, Mizutani Yasuaki, Ueda Akihiro, Ito Mizuki
Department of Neurology, Fujita Health University, School of Medicine, Toyoake, Japan.
Department of Neurology, Fujita Health University Okazaki Medical Center, Okazaki, Japan.
J Mov Disord. 2023 Jan;16(1):13-21. doi: 10.14802/jmd.22082. Epub 2022 Dec 20.
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.
本综述总结了在理解多系统萎缩（MSA）的病理生理学和早期临床症状方面取得的进展，以及该疾病诊断方法和疾病修饰疗法的进展。2022年，运动障碍协会提出了新的诊断标准，以开发疾病修饰疗法并推动MSA的临床试验，自2008年提出第二次共识以来一直如此。关于发病机制，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积累了前沿研究成果。在神经影像学方面，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能力。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、针对5-羟色胺的疗法以及间充质干细胞疗法，这些疗法被认为会影响该疾病的多个方面，包括神经炎症。在过去几年中，MSA发病机制的阐明取得了加速进展，早期MSA检测诊断技术的发展有望推动针对这种最难治疗的神经退行性疾病之一的疾病修饰疗法的开发。
