Otiniano-Sifuentes Ricardo, Cuba Antezana Anali, De La Cruz Ramirez Walter F, Pacheco-Barrios Kevin, Segura Chavez Darwin A
Department of Neurovascular Diseases, Instituto Nacional de Ciencias Neurológicas, Lima, Peru.
Servicio de Neurología y Neuropsiquiatría, Departamento de Medicina, Hospital Nacional Cayetano Heredia, Lima, Peru.
Front Neurol. 2021 Jan 15;11:620483. doi: 10.3389/fneur.2020.620483. eCollection 2020.
Anti-LGI1 encephalitis is an autoimmune encephalitis with antibodies against leucine-rich glioma-inactivated 1 (LGI1), first described in 2010. It is a non-frequent and poorly understood entity that represents the second most frequent cause of autoimmune encephalitis. This entity is characterized by the presence of limbic encephalitis, hyponatremia, and faciobrachial dystonic seizures. Herein, we present the case of a male patient with an onset of epileptic seizures (generalized tonic-clonic seizure), and involuntary dystonic movements that affect the right side of his face and right upper limb associated with mental disorder, and affectation of higher functions. The electroencephalogram showed continuous generalized slowing of the background activity. The brain magnetic resonance imaging showed signal hyperintensity at the level of both mesial temporal lobes and hippocampi and in the head of the right caudate nucleus. Anti-thyroglobulin antibodies were positive, and he was initially diagnosed as Hashimoto's encephalopathy (HE). However, the response to corticosteroids was not completed as it is usually observed in HE. For that, antibodies for autoimmune encephalitis were tested, and the anti-LGI1 antibodies were positive in serum and cerebrospinal fluid. HE is an important differential diagnosis to consider. Furthermore, the presence of Anti-thyroglobulin antibodies should not be taken as the definitive diagnostic criteria, since these antibodies could be associated with other autoimmune encephalopathies, which include in addition to anti-LGI1, anti-NMDA and anti-Caspr2.
抗LGI1脑炎是一种自身免疫性脑炎，其体内存在针对富含亮氨酸的胶质瘤失活蛋白1（LGI1）的抗体，于2010年首次被描述。它是一种不常见且了解较少的疾病，是自身免疫性脑炎的第二大常见病因。该疾病的特征为边缘性脑炎、低钠血症和面臂肌张力障碍性癫痫发作。在此，我们报告一例男性患者，其首发症状为癫痫发作（全身强直阵挛性发作），伴有影响其右侧面部和右上肢体的非自主性肌张力障碍运动，同时伴有精神障碍及高级功能受累。脑电图显示背景活动持续广泛性减慢。脑磁共振成像显示双侧内侧颞叶、海马以及右侧尾状核头部信号增强。抗甲状腺球蛋白抗体呈阳性，该患者最初被诊断为桥本脑病（HE）。然而，其对皮质类固醇的反应并不像HE通常所见的那样完全。为此，对自身免疫性脑炎抗体进行了检测，血清和脑脊液中的抗LGI1抗体均呈阳性。HE是需要考虑的重要鉴别诊断。此外，抗甲状腺球蛋白抗体的存在不应被视为确诊标准，因为这些抗体可能与其他自身免疫性脑病相关，除抗LGI1外，还包括抗NMDA和抗Caspr2。
