Department of Neurology, Duke University School of Medicine, Durham, NC, 27710, USA.
Brain Injury Translational Research Laboratories, Bryan Research Building, 227F, 311 Research Drive, Durham, NC, 27710, USA.
Neurotherapeutics. 2020 Apr;17(2):404-413. doi: 10.1007/s13311-020-00861-2.
Autoimmune encephalitis (AE) is a group of disorders causing synaptic receptor dysfunction with a broad range of neurological symptoms that has been historically difficult to differentiate clinically. Today, AE represents an excellent example of the rapid determination of the cause of a disease and the ability to identify potential treatments using relatively simple basic science techniques of investigation. Of the number of autoimmune encephalitides identified thus far, one of the best examples of the impact of basic science studies on disease management is NMDA receptor mediated autoimmune encephalitis (NMDAr-AE). In this review, we will provide an overview of the epidemiology of NMDAr-AE, clinical features and treatments, and the basic science tools and techniques that were used to identify the cause, correlate symptoms to underlying pathophysiology, and to understand the mechanism of disease pathology.
自身免疫性脑炎（AE）是一组导致突触受体功能障碍的疾病，具有广泛的神经症状，在临床上一直难以区分。如今，AE 是一个很好的例子，说明了使用相对简单的基础科学调查技术，快速确定疾病原因并确定潜在治疗方法的能力。在迄今为止确定的自身免疫性脑炎中，NMDA 受体介导的自身免疫性脑炎（NMDAr-AE）是基础科学研究对疾病管理产生影响的最佳范例之一。在这篇综述中，我们将概述 NMDAr-AE 的流行病学、临床特征和治疗方法，以及用于确定病因、将症状与潜在病理生理学相关联以及了解疾病发病机制的基础科学工具和技术。
