Department of Neurology, GuangDong 999 Brain Hospital, Guangzhou, China.
Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, TX, United States.
Front Immunol. 2023 Mar 9;14:1120894. doi: 10.3389/fimmu.2023.1120894. eCollection 2023.
Encephalitis has been recognized in patients with autoimmunity related to the 65-kDa isoform of glutamic acid decarboxylase (GAD65) antibodies; however, patients with meningoencephalitis associated with those antibodies have been rarely identified in the medical literature. We aimed to define the frequency, clinical features, response to therapy, and functional outcomes of patients with meningoencephalitis associated with GAD antibodies.
We retrospectively studied consecutive patients attending a tertiary care center for evaluation of an autoimmune neurological disorder from January 2018 to June 2022. The modified Rankin Scale (mRS) was used to assess the functional outcome at the last follow-up.
We evaluated 482 patients with confirmed autoimmune encephalitis during the study period. Four among the 25 patients with encephalitis related to GAD65 antibodies were identified. One patient was excluded owing to the coexistence of NMDAR antibodies. Three male patients aged 36, 24, and 16 years had an acute ( = 1) or subacute ( = 2) onset of confusion, psychosis, cognitive symptoms, seizures, or tremor. No patient had fever or clinical signs of meningeal irritation. Mild pleocytosis (<100 leukocytes/106) was identified in two patients, whereas one patient had normal CSF. Following immunotherapy with corticosteroids ( = 3) or intravenous immunoglobulin ( = 1), significant improvement was observed in all three cases, achieving a good outcome (mRS 1) in all cases.
Meningoencephalitis is an uncommon presentation of GAD65 autoimmunity. Patients present with signs of encephalitis but with meningeal enhancement and have good outcomes.
谷氨酸脱羧酶 65 千道尔顿同工型（GAD65）抗体相关自身免疫患者中已认识到脑炎的存在；然而，相关抗体引起脑膜脑炎的患者在医学文献中很少被发现。我们旨在明确与 GAD 抗体相关脑膜脑炎患者的频率、临床特征、治疗反应和功能结局。
我们回顾性研究了 2018 年 1 月至 2022 年 6 月期间在三级医疗中心就诊评估自身免疫性神经疾病的连续患者。采用改良 Rankin 量表（mRS）评估末次随访时的功能结局。
在研究期间，我们评估了 482 例确诊自身免疫性脑炎患者。在 25 例与 GAD65 抗体相关的脑炎患者中，有 4 例符合条件。由于存在 NMDAR 抗体，1 例患者被排除在外。3 名男性患者年龄分别为 36 岁、24 岁和 16 岁，急性（=1）或亚急性（=2）起病时出现意识混乱、精神病、认知症状、癫痫发作或震颤。无患者有发热或脑膜刺激征。2 例患者有轻度白细胞增多（白细胞/106＜100），1 例患者 CSF 正常。3 例患者接受皮质类固醇（=3）或静脉免疫球蛋白（=1）免疫治疗后，所有 3 例均有显著改善，所有患者均获得良好结局（mRS 1）。
脑膜脑炎是 GAD65 自身免疫的一种不常见表现。患者表现为脑炎的体征，但脑膜强化，结局良好。
