Suzuki Shigeaki, Seki Morinobu, Suzuki Norihiro
Department of Neurology, Keio University School of Medicine.
Nihon Rinsho Meneki Gakkai Kaishi. 2013;36(2):86-94. doi: 10.2177/jsci.36.86.
Patients with limbic encephalitis usually present with rapidly progressive short-term memory deficits, psychiatric symptoms, and seizures. The recent concept of limbic encephalitis has been expanded. Especially, various types of autoimmune limbic encephalitis are associated with autoantibodies of intracellular or cell membrane antigens. Sine autoimmune limbic encephalitis is also associated with some types of tumors, it has also an aspect of paraneoplastic syndrome. Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a new category of treatment-responsive limbic encephalitis associated with anti-NMDAR antibodies, which is the most frequent autoantibody to cell membrane antigen. The autoantibodies are detected in the CSF and serum of young women with ovarian teratoma, who typically develop schizophrenia-like psychiatric symptoms. There is a highly characteristics syndrome evolving in 5 stages; the prodromal, psychotic, unresponsive, hyperkinetic, and gradual recovery phases. The hyperkinetic phase is the most prolonged and crucial. This disorder is usually severe and can be fatal, but it is potentially reversible. Although the pathogenesis remains unclear, this disorder is considered to be the autoantibody-mediated encephalitis. This review focuses in the recent concept of limbic encephalitis and clinical characteristics of anti-NMDA receptor encephalitis.
边缘性脑炎患者通常表现为快速进展的短期记忆缺陷、精神症状和癫痫发作。边缘性脑炎的最新概念已经得到扩展。特别是，各种类型的自身免疫性边缘性脑炎与细胞内或细胞膜抗原的自身抗体有关。由于自身免疫性边缘性脑炎也与某些类型的肿瘤有关，它也具有副肿瘤综合征的一面。抗N-甲基-D-天冬氨酸（NMDA）受体脑炎是一种新的与抗NMDAR抗体相关的对治疗有反应的边缘性脑炎，它是最常见的针对细胞膜抗原的自身抗体。在患有卵巢畸胎瘤的年轻女性的脑脊液和血清中检测到自身抗体，她们通常会出现类似精神分裂症的精神症状。有一个高度特征性的综合征按5个阶段发展；前驱期、精神病期、无反应期、运动增多期和逐渐恢复期。运动增多期是最长且最关键的。这种疾病通常很严重，可能致命，但有潜在的可逆性。虽然发病机制尚不清楚，但这种疾病被认为是自身抗体介导的脑炎。这篇综述重点介绍边缘性脑炎的最新概念和抗NMDA受体脑炎的临床特征。
