Reyes-Sosa Luis Carlos, León-Castillo Daniela Alexia, Jiménez-Islas Juan Carlos, Aguilar-Vázquez Crhistian Alejandro
Instituto Mexicano del Seguro Social, Centro Médico Nacional Siglo XXI, Hospital de Especialidades "Dr. Bernardo Sepúlveda Gutiérrez", Servicio de Neurología. Ciudad de México, México.
Rev Med Inst Mex Seguro Soc. 2023 Nov 6;61(6):868-874. doi: 10.5281/zenodo.10064492.
Background: Anti-LGI1 encephalitis is characterized by a pattern of inflammation that predominantly affects the limbic system It is part of the autoimmune encephalitis that attack neuronal surface antigens. It is characterized by the triad of subacute dementia, faciobrachial dystonic crises, and hyponatremia, presenting an excellent response to immunotherapy. The aim of this article is to describe the clinical evolution and functional outcome at 6 months of two patients with anti-LGI1 encephalitis using clinical cases.
Case 1: 62-year-old man with 8-week symptoms manifested by changes in mood, disorientation, and focal motor seizures. Case 2 A 72-year-old woman with a 5-month evolution of rapidly progressive dementia, hyponatremia and bitemporal hyperintensities on MRI. In both, due to clinical suspicion, acute dual immunotherapy with steroid and immunoglobulin was given with substantial improvement. Subsequently, the existence of anti-LGI1 antibodies in cerebrospinal fluid was confirmed. Although both patients received a dose of rituximab during their hospitalization, only the patient in the first case continued biannual doses of rituximab. The second patient was not initially considered to continue long-term immunomodulatory treatment and experienced a relapse.
These clinical vignettes present the reader with the classic characteristics of this disease. This can facilitate its recognition and timely initiation of treatment, improving the functional prognosis of patients.
背景：抗LGI1脑炎的特征是炎症模式主要影响边缘系统。它是攻击神经元表面抗原的自身免疫性脑炎的一部分。其特征为亚急性痴呆、面臂肌张力障碍性危机和低钠血症三联征，对免疫治疗反应良好。本文旨在通过临床病例描述两名抗LGI1脑炎患者6个月时的临床演变和功能转归。
病例1：一名62岁男性，有8周症状，表现为情绪改变、定向障碍和局灶性运动性癫痫发作。病例2：一名72岁女性，病程5个月，表现为快速进展性痴呆、低钠血症，MRI显示双侧颞叶高信号。两人均因临床怀疑，给予类固醇和免疫球蛋白急性双重免疫治疗，病情有显著改善。随后，脑脊液中抗LGI1抗体的存在得到证实。虽然两名患者在住院期间均接受了一剂利妥昔单抗，但只有第一例患者继续每半年注射一剂利妥昔单抗。第二例患者最初未被考虑继续长期免疫调节治疗，出现了复发。
这些临床病例向读者展示了这种疾病的典型特征。这有助于其识别和及时开始治疗，改善患者的功能预后。
