抗体阴性的自身免疫性脑炎的研究进展

Research Progress of Autoantibody-Negative Autoimmune Encephalitis

摘要: 随着自身免疫性脑炎(AE)相关抗体不断发现和诊断的完善下，AE发病率和患病率呈现逐年提高趋势，抗体阴性的自身免疫性脑炎(AbNAE)发病率和患病率也出现逐年提高的趋势。但在缺乏抗体这一重要诊断依据的情况下，如何早期诊断AbNAE，早期进行免疫治疗，改善AbNAE患者预后是现在亟待解决的问题。本文通过结合现有研究，从AbNAE的诊断、临床表现、辅助检查和治疗方案等方面综述。由于缺少相关抗体，AbNAE的诊断主要依赖于临床表现和辅助检查，MRI和动脉自选标记等技术对AbNAE有着重要诊断价值。AbNAE的治疗方案与抗体阳性AE一致，但AbNAE的预后较抗体阳性AE差。如何进一步完善诊断标准、发现特异性抗体、提高辅助检查手段、降低AbNAE误诊率和改善AbNAE患者预后是今后相关研究的主要方向。

Abstract: With the continuous discovery and improvement of autoimmune encephalitis (AE) related antibodies, the incidence and prevalence of AE have shown an increasing trend year by year, and the incidence and prevalence of antibody-negative autoimmune encephalitis (AbNAE) have also shown an increasing trend year by year. However, in the absence of antibodies, an important diagnostic basis, how to diagnose AbNAE early, conduct early immunotherapy, and improve the prognosis of AbNAE patients is now an urgent problem to be solved. This article reviews the diagnosis, clinical manifestations, auxiliary examinations and treatment plans of AbNAE by combining existing research. Due to the lack of relevant antibodies, the diagnosis of AbNAE mainly depends on clinical manifestations and auxiliary examinations. MRI and arterial self-selected markers have important diagnostic value for AbNAE. The treatment plan of AbNAE is consistent with that of antibody-positive AE, but the prognosis of AbNAE is worse than that of antibody-positive AE. How to further improve the diagnostic criteria, discover specific antibodies, improve auxiliary examination methods, reduce the misdiagnosis rate of AbNAE and improve the prognosis of AbNAE patients is the main direction of future related research.