First Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
J Diabetes Investig. 2021 Feb;12(2):290-292. doi: 10.1111/jdi.13341. Epub 2020 Aug 11.
Anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis is an autoimmune disorder in which autoantibodies in the limbic system bind to GluN1 subunits of NMDA-Rs in the brain. We report a rare case of autoimmune polyendocrine syndrome type 3 complicated by anti-NMDA-R encephalitis. After hospitalization for type 1 diabetes, the 39-year-old patient developed various schizophreniform symptoms and seizures after cold-like symptoms. These findings are consistent with the diagnosis of anti-NMDA-R encephalitis. Immune-related encephalitis was suspected at the early phase of the disease, and cerebrospinal fluid was positive for anti-NMDA-R antibody. Early steroid pulse therapy was initiated during the disease course. The condition improved gradually to full recovery. Early detection and treatment of anti-NMDA-R encephalitis should enhance a positive outcome, considering that besides thyroid diseases and type 1 diabetes, various autoimmune diseases are associated with autoimmune polyendocrine syndrome type 3.
抗 N-甲基-D-天冬氨酸受体(NMDA-R)脑炎是一种自身免疫性疾病,其中边缘系统的自身抗体与大脑中 NMDA-R 的 GluN1 亚基结合。我们报告了一例罕见的自身免疫性多内分泌腺综合征 3 型合并抗 NMDA-R 脑炎。1 型糖尿病住院后,该 39 岁患者出现感冒样症状后出现各种类精神分裂样症状和癫痫发作。这些发现与抗 NMDA-R 脑炎的诊断一致。疾病早期怀疑为免疫相关脑炎,脑脊液抗 NMDA-R 抗体阳性。在病程中早期开始类固醇脉冲治疗。病情逐渐好转,完全恢复。考虑到除甲状腺疾病和 1 型糖尿病外,各种自身免疫性疾病与自身免疫性多内分泌腺综合征 3 型相关,因此早期发现和治疗抗 NMDA-R 脑炎应可获得良好的结果。