Erazo Torricelli Ricardo
Hospital de Niños Dr. Luis Calvo Mackenna, Clínica Alemana de Santiago, Universidad del Desarrollo, Santiago, Chile. E-mail:
Medicina (B Aires). 2019;79 Suppl 3:54-59.
Autoimmune encephalitis (AE) is defined as neurological syndromes of subacute installation of compromise of consciousness, alteration of working memory and psychiatric disorders associated with abnormal movements and epileptic seizures and that are produced by the action of anti-neuronal antibodies. They bind to neurotransmitter receptors or membrane proteins. Antibody to NMDAR is the origin of the majority of cases of AD in children and young adults, followed by anti-LGI1 antibody for presentation in adults. The AE has increased in the last decade, with a large number of new agents described that produce mostly neurological syndromes that involve the central nervous system, with predominance of psychiatric signaling, except in children and the predominant abnormal movements, epileptic seizures and compromise of conscience. They are frequently associated with tumors in adults but in children this association is more infrecuent. All AEs respond to immunomodulatory therapy although in different measures depending on the type of antibody involved. In general, the evolution to improvement is slow and can be completed in months or even in one year or more. In this review, the main EA clinical pictures related to specific antibodies are highlighted, also mentioning recently discovered immunophenotypes.
自身免疫性脑炎（AE）被定义为因抗神经元抗体作用而导致的亚急性起病的神经综合征，表现为意识障碍、工作记忆改变、伴有异常运动和癫痫发作的精神障碍。这些抗体与神经递质受体或膜蛋白结合。NMDAR抗体是儿童和青年成人中大多数AE病例的病因，其次是抗LGI1抗体，多见于成人。在过去十年中，AE的发病率有所上升，出现了大量新的致病因素，这些因素大多导致涉及中枢神经系统的神经综合征，以精神症状为主，但儿童中以异常运动、癫痫发作和意识障碍为主。AE在成人中常与肿瘤相关，但在儿童中这种关联较少见。所有AE对免疫调节治疗均有反应，不过根据所涉及抗体的类型，反应程度不同。一般来说，病情改善的进展缓慢，可能需要数月甚至一年或更长时间才能完成。在本综述中，重点介绍了与特定抗体相关的主要AE临床症状，同时也提及了最近发现的免疫表型。
