抗体阴性自身免疫性脑炎的研究进展

The research progress of autoantibody-negative autoimmune encephalitis

随着自身免疫性脑炎的研究领域迅速扩大,抗体阴性自身免疫性脑炎(AbNAE)已成为主要亚型。但疾病本身的高度异质性和抗体的缺乏给诊断带来了挑战,对该亚型的认识仍不足,具体表现在误诊的增加和诊断标准的不恰当应用。该文结合现有研究对其诊断、临床特征、治疗、预后及随访等方面进行综述。目前,AbNAE患者的临床表现及辅助检查结果与抗体阳性自身免疫性脑炎患者相比尚未发现特征性不同。少部分研究显示癫痫和局灶性神经功能缺损、脑脊液特异性寡克隆区带在AbNAE患者中较少出现,该结论未来仍需进一步证实。理论上,AbNAE患者可能包括尚未识别的新型抗体、细胞免疫介导和低滴度抗体的患者,治疗反应也可能是异质性的。但整体来看,AbNAE患者同抗体阳性自身免疫性脑炎患者一样,均可从免疫治疗中获益。未来应继续研究潜在致病机制,重点寻找新的特异性生物标志物,以便进一步对患者进行亚分类,并制订针对性的治疗方案。

As the spectrum of autoimmune encephalitis continues to grow,autoantibody-negative autoimmune encephalitis(AbNAE)has emerged as a significant subtype.However,due to its inherent heterogeneity and the absence of auto-antibodies,diagnosing this subtype poses a challenge.Furthermore,there is still a lack of understanding regarding AbNAE,as evidenced by an increase in misdiagnosis and inappropriate application of diagnostic criteria.This article aims to enhance clinicians'comprehension of patients with autoantibody-negative autoimmune encephalitis by reviewing existing studies on its diagnosis,clinical characteristics,treatment,prognosis and follow-up.No characteristic difference has been observed in the clinical presentation and ancillary findings between AbNAE and autoantibody-positive autoimmune encephalitis patients.However,some studies have indicated that epilepsy,focal neurological deficits,and cerebrospinal fluid-specific oligoclonal bands are less common in the AbNAE patients.Further research is necessary to validate these findings.The AbNAE patients may potentially include individuals with unrecognized novel antibodies,cellular immune-mediated responses and low-titer antibodies.Therefore,the response to treatment may be heterogeneous.Overall,the AbNAE patients may derive similar benefits from immunotherapy as individuals who test positive for autoantibodies.In the future,it is important to continue research on the underlying pathogenic mechanisms,with a particular emphasis on identifying new specific biomarkers,which will allow for further classification of patients and the development of targeted therapeutic regimens.