Bhagavati Satyakam
Department of Neurology, Downstate Medical Center, State University of New York College of Medicine, New York, NY, United States.
Front Neurol. 2021 Apr 14;12:664664. doi: 10.3389/fneur.2021.664664. eCollection 2021.
Remarkable discoveries over the last two decades have elucidated the autoimmune basis of several, previously poorly understood, neurological disorders. Autoimmune disorders of the nervous system may affect any part of the nervous system, including the brain and spinal cord (central nervous system, CNS) and also the peripheral nerves, neuromuscular junction and skeletal muscle (peripheral nervous system, PNS). This comprehensive overview of this rapidly evolving field presents the factors which may trigger breakdown of self-tolerance and development of autoimmune disease in some individuals. Then the pathophysiological basis and clinical features of autoimmune diseases of the nervous system are outlined, with an emphasis on the features which are important to recognize for accurate clinical diagnosis. Finally the latest therapies for autoimmune CNS and PNS disorders and their mechanisms of action and the most promising research avenues for targeted immunotherapy are discussed.
过去二十年间的重大发现阐明了几种先前了解甚少的神经系统疾病的自身免疫基础。神经系统自身免疫性疾病可能会影响神经系统的任何部分,包括脑和脊髓(中枢神经系统,CNS)以及周围神经、神经肌肉接头和骨骼肌(周围神经系统,PNS)。本文对这一快速发展的领域进行了全面概述,介绍了可能引发某些个体自身耐受性破坏和自身免疫性疾病发生的因素。接着概述了神经系统自身免疫性疾病的病理生理基础和临床特征,重点强调了对于准确临床诊断至关重要的特征。最后讨论了自身免疫性中枢神经系统和周围神经系统疾病的最新治疗方法及其作用机制,以及靶向免疫治疗最有前景的研究途径。