Gole Shrey, Anand Amritpal
Northwestern Medicine McHenry Hospital
Autoimmune encephalitis (AIE) is an immune-mediated condition that induces brain inflammation and is one of the most common causes of non-infectious encephalitis. In the past decade, AIE has become an emerging addition to the differential diagnosis when a classical infection cannot explain focal neurological symptoms. While the exact mechanism of AIE is unknown, current literature suggests that autoimmune antibodies target synaptic proteins, leading to widespread inflammation. AIE commonly presents as a new onset of memory loss, psychosis, altered mental status, or seizures, with the presentation taking place over a few weeks to three months. AIE involves several parts of the nervous system, including the limbic system, the spinal cord, and/or the entire neuraxis. AIE can be confirmed by multiple modalities, including laboratory testing (antibody detection), neuroimaging, and electrophysiological studies (electroencephalogram). Although classical paraneoplastic encephalitis is commonly associated with cancer, AIE may or may not be related. Therefore, once the diagnosis is established, patients should undergo cancer screening due to a high degree of association with underlying malignancy. Although these tests can be time-consuming, clinicians should consider initiating treatment early in the course if their clinical suspicion is high, as they are often treatment-responsive and have significantly improved outcomes. The most commonly studied subtype of AIE is anti-N-methyl-D-aspartate receptor encephalitis, so the management approach is primarily based on that. This review describes the current state of knowledge on various types of autoimmune encephalitis and educates readers with a concise, up-to-date summary.
自身免疫性脑炎(AIE)是一种免疫介导的疾病,可引发脑部炎症,是非感染性脑炎最常见的病因之一。在过去十年中,当经典感染无法解释局灶性神经症状时,AIE已成为鉴别诊断中新增的一种疾病。虽然AIE的确切机制尚不清楚,但目前的文献表明,自身免疫抗体靶向突触蛋白,导致广泛炎症。AIE通常表现为新发的记忆丧失、精神病、精神状态改变或癫痫发作,症状在数周至三个月内出现。AIE累及神经系统的多个部位,包括边缘系统、脊髓和/或整个神经轴。AIE可通过多种方式确诊,包括实验室检测(抗体检测)、神经影像学和电生理研究(脑电图)。虽然经典的副肿瘤性脑炎通常与癌症相关,但AIE可能与之有关,也可能无关。因此,一旦确诊,由于与潜在恶性肿瘤高度相关,患者应接受癌症筛查。尽管这些检查可能耗时,但如果临床怀疑度高,临床医生应考虑在病程早期开始治疗,因为患者通常对治疗有反应,且预后有显著改善。研究最多的AIE亚型是抗N-甲基-D-天冬氨酸受体脑炎,因此管理方法主要基于此。本综述描述了各种类型自身免疫性脑炎的当前知识状态,并以简洁、最新的总结对读者进行教育。