Anti-NMDAR encephalitis: A single-center, longitudinal study in China

Anti-NMDAR encephalitis: A single-center, longitudinal study in China
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DOI:
10.1212/nxi.0000000000000633
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发表时间:
2020-01-01
影响因子:
8.8
通讯作者:
Guan, Hongzhi
Guan, Hongzhi
中科院分区:
医学1区
文献类型:
--
作者:
Xu, Xiaolu;Lu, Qiang;Guan, Hongzhi

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目的描述我国抗NMDAR脑炎患者的临床特点、免疫治疗及远期疗效。2011年至2017年,对符合诊断标准的患者进行了入选和随访。前瞻性收集患者的临床特征、治疗方法和远期疗效。对影响远期预后的因素进行分析。结果共纳入220例患者。最常见的临床表现为精神病(82.7%)和癫痫(80.9%)。在这些患者中,19.5%的人有潜在的肿瘤;其中卵巢畸胎瘤占女性肿瘤的100%,只有一名男性有肺癌。大多数患者(99.5%)接受一线治疗(糖皮质激素、静脉注射免疫球蛋白、单独或联合血浆置换),只有7.3%接受二线免疫治疗(利妥昔单抗、环磷酰胺单独或联合治疗)。53.2%的患者接受了长期免疫治疗(霉酚酸酯或硫唑嘌呤1年)。在前12个月中,207名患者(94.1%)病情好转,5名患者(2.3%)死亡,38名患者(17.3%)复发。在12个月的随访中,92.7%的患者有良好的临床结果(改良的Rankin量表评分
ObjectiveTo describe the detailed clinical characteristics, immunotherapy, and long-term outcomes of patients with anti-NMDA receptor (NMDAR) encephalitis in China.MethodsA single-center, prospective study. Patients who met the diagnostic criteria were enrolled from 2011 to 2017 and followed up. The clinical features, treatment, and long-term outcomes were collected prospectively. Factors affecting the long-term prognosis were analyzed.ResultsThe study included 220 patients. The most common clinical presentations were psychosis (82.7%) and seizures (80.9%). Of the patients, 19.5% had an underlying neoplasm; of which ovarian teratoma was 100% of tumors in females and only one male had lung cancer. Most patients (99.5%) received first-line therapy (glucocorticoids, IV immunoglobulin, or plasmapheresis alone or combined), and only 7.3% received second-line immunotherapy (rituximab, cyclophosphamide alone, or combined). Long-term immunotherapy (mycophenolate mofetil or azathioprine >1 year) was administered to 53.2% of patients. During the first 12 months, 207 (94.1%) patients experienced improvement, and 5 (2.3%) died, whereas 38 (17.3%) experienced relapses. At 12-month follow-up, 92.7% had favorable clinical outcomes (modified Rankin Scale score