Kalman Bernadette
*University of Pecs, Pecs †Markusovszky University Teaching Hospital, Center for Research and Education, Szombathely, Hungary.
Neurologist. 2017 Jan;22(1):1-13. doi: 10.1097/NRL.0000000000000087.
Neurology has been continuously transforming by the refinement of molecular diagnostics and the development of disease-modifying treatments. The discovery of new antibody markers has elucidated the pathogenesis, provided the means of diagnostics, and offered cure or treatment for several immune-mediated neurological and neuropsychiatric disorders. The identification of pathogenic and marker autoantibodies has also facilitated defining the associated phenotypic spectra and the overlap among the phenotypes linked to individual immune markers.
This survey presents the list of currently known autoimmune encephalitis entities along with the associated marker autoantibodies, highlights the phenotypic and immune pathogenic relationships, calls attention to the recently described rare syndromes, discusses the biological significance of the autoantibodies and targeted molecules, points out the potential postinfectious origin of immune pathogenesis in several of the disorders, and directs the readers to the latest diagnostic guidelines as well as to the generally used treatment approaches.
Owing to the successful and usually combined use of various methods to detect serum and cerebrospinal fluid autoantibodies on rodent brain sections, in primary neuronal cell culture, in immune precipitation, and cell-based assays, or in other antigen-specific immune assays (Western blot, enzyme-linked immunosorbent assay, and radioimmune assay), the subgroup of antibody marker-negative autoimmune encephalopathy syndromes is contracting, whereas the numbers of entities within the overall group are expanding. Recognition of the correct diagnosis is becoming increasingly rewarding not only for neurologists, but also for pediatric neurologists and psychiatrists.
随着分子诊断技术的完善和疾病修饰治疗方法的发展，神经病学一直在不断变革。新抗体标志物的发现阐明了发病机制，提供了诊断手段，并为多种免疫介导的神经和神经精神疾病提供了治愈或治疗方法。致病性和标志物自身抗体的鉴定也有助于确定相关的表型谱以及与个体免疫标志物相关的表型之间的重叠。
本综述列出了目前已知的自身免疫性脑炎实体及其相关的标志物自身抗体，强调了表型与免疫发病机制之间的关系，提请注意最近描述的罕见综合征，讨论了自身抗体和靶向分子的生物学意义，指出了几种疾病中免疫发病机制潜在的感染后起源，并引导读者查阅最新的诊断指南以及常用的治疗方法。
由于成功且通常是联合使用多种方法来检测血清和脑脊液中的自身抗体，这些方法包括在啮齿动物脑切片上、原代神经元细胞培养中、免疫沉淀和基于细胞的检测中，或在其他抗原特异性免疫检测（蛋白质印迹法、酶联免疫吸附测定和放射免疫测定）中进行检测，抗体标志物阴性的自身免疫性脑病综合征亚组正在缩小，而整个组内的实体数量正在增加。正确诊断不仅对神经科医生，而且对儿科神经科医生和精神科医生来说都越来越有价值。
