Department of Geriatrics, The First Affiliated Hospital of Nanjing Medical University, Nanjing Medical University, Nanjing, China.
Front Immunol. 2024 Oct 3;15:1444288. doi: 10.3389/fimmu.2024.1444288. eCollection 2024.
Autoimmune encephalitis (AE) comprises a group of inflammatory brain disorders mediated by autoimmune responses. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis, and anti-γ-aminobutyric acid-B receptor (GABABR) encephalitis are the most prevalent forms, characterized by the presence of antibodies against neuronal cell-surface antigens. Efgartigimod, an antagonist of the neonatal Fc receptor, has proven efficacy in myasthenia gravis treatment. This clinical case report describes the clinical progression and functional outcomes of AE in three patients who received efgartigimod treatment.
Case 1 was a 60-year-old man exhibiting memory impairment and psychiatric disturbances over 11 days. Case 2 was a 38-year-old man with a 1-month history of rapid cognitive decline and seizures. Case 3 was a 68-year-old woman with mental behavioral changes and seizures for 4 months. Anti-GABABR, anti-LGI1, and anti-NMDAR antibodies were confirmed in the respective patients' cerebrospinal fluid or serum. All three patients experienced marked and swift symptomatic relief after four cycles of efgartigimod treatment, with no complication.
Current first-line and second-line treatments for AE have limitations, and efgartigimod has demonstrated potential in the rapid and efficacious treatment of AE, emerging as a promising option for the management of this disease.
自身免疫性脑炎（AE）是一组由自身免疫反应介导的炎症性脑疾病。抗 N-甲基-D-天冬氨酸受体（NMDAR）脑炎、抗亮氨酸丰富胶质瘤失活 1（LGI1）脑炎和抗 γ-氨基丁酸 B 受体（GABABR）脑炎是最常见的形式，其特征是存在针对神经元细胞表面抗原的抗体。Efgartigimod 是一种新生 Fc 受体拮抗剂，已被证明在重症肌无力治疗中有效。本临床病例报告描述了三名接受 Efgartigimod 治疗的 AE 患者的临床进展和功能结局。
病例 1 是一名 60 岁男性，表现为记忆力减退和精神障碍超过 11 天。病例 2 是一名 38 岁男性，有 1 个月的认知功能迅速下降和癫痫发作史。病例 3 是一名 68 岁女性，有 4 个月的精神行为改变和癫痫发作史。在各自患者的脑脊液或血清中均证实存在抗 GABABR、抗 LGI1 和抗 NMDAR 抗体。所有三名患者在接受 4 个周期的 Efgartigimod 治疗后均迅速显著缓解症状，且无并发症。
目前 AE 的一线和二线治疗方法存在局限性，Efgartigimod 在 AE 的快速和有效治疗中显示出潜力，成为治疗这种疾病的有前途的选择。
