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

被引：140

作者：

Xu, Xiaolu ^{[1
]}

Lu, Qiang ^{[1
]}

Huang, Yan ^{[1
]}

Fan, Siyuan ^{[1
]}

Zhou, Lixin ^{[1
]}

Yuan, Jing ^{[1
]}

Yang, Xunzhe ^{[1
]}

Ren, Haitao ^{[1
]}

Sun, Dawei ^{[2
]}

Dai, Yi ^{[2
]}

Zhu, Huadong ^{[3
]}

Jiang, Yinan ^{[4
]}

Zhu, Yicheng ^{[1
]}

Peng, Bin ^{[1
]}

Cui, Liying ^{[1
,5
]}

Guan, Hongzhi ^{[1
]}

机构：

[1] Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Neurol, Beijing, Peoples R China

[2] Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Gynecol & Obstet, Beijing, Peoples R China

[3] Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Emergency, Beijing, Peoples R China

[4] Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Psychol, Beijing, Peoples R China

[5] Chinese Acad Med Sci, Ctr Neurosci, Beijing, Peoples R China

来源：

NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION | 2020年 / 7卷 / 01期

关键词：

ASPARTATE RECEPTOR ENCEPHALITIS; MELANOCYTIC NEVI; DIAGNOSIS; RESECTION; DISORDER;

D O I：

10.1212/NXI.0000000000000633

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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 <= 2).ConclusionsPatients in China present with psychosis and seizure frequently but have a low percentage of underlying neoplasms. Re-enforced first-line immunotherapy is effective in managing anti-NMDAR encephalitis in the acute phase. Although relapse is relatively common, with combined first-line and long-term immunotherapy, most patients reached favorable outcomes.

引用

页数：9