The realization that autoantibodies can contribute to dysfunction of the brain has brought about a paradigm shift in neurological diseases over the past decade, offering up important novel diagnostic and therapeutic opportunities. Detection of specific autoantibodies to neuronal or glial targets has resulted in a better understanding of central nervous system autoimmunity and in the reclassification of some diseases previously thought to result from infectious, ‘idiopathic’ or psychogenic causes. The most prominent examples, such as aquaporin 4 autoantibodies in neuromyelitis optica or NMDAR autoantibodies in encephalitis, have stimulated an entire field of clinical and experimental studies on disease mechanisms and immunological abnormalities. Also, these findings inspired the search for additional autoantibodies, which has been very successful to date and has not yet reached its peak. This Review summarizes this rapid development at a point in time where preclinical studies have started delivering fundamental new data for mechanistic understanding, where new technologies are being introduced into this field, and — most importantly — where the first specifically tailored immunotherapeutic approaches are emerging.

在过去的十年里，人们认识到自身抗体会导致大脑功能障碍，这给神经系统疾病带来了范式转变，提供了重要的新型诊断和治疗机会。检测针对神经元或神经胶质靶点的特异性自身抗体可以更好地了解中枢神经系统自身免疫，并对一些以前认为是由传染性、“特发性”或心因性原因引起的疾病进行了重新分类。最突出的例子，例如视神经脊髓炎中的水通道蛋白 4 自身抗体或脑炎中的 NMDAR 自身抗体，刺激了整个疾病机制和免疫异常的临床和实验研究领域。此外，这些发现激发了对其他自身抗体的探索，迄今为止，这种探索非常成功，但尚未达到顶峰。这篇综述总结了这一快速发展，此时临床前研究已经开始为机制理解提供基本的新数据，新技术正在被引入该领域，最重要的是，第一个专门定制的免疫治疗方法正在出现。