PT  - JOURNAL ARTICLE
AU  - Xu, Xiaolu
AU  - Lu, Qiang
AU  - Huang, Yan
AU  - Fan, Siyuan
AU  - Zhou, Lixin
AU  - Yuan, Jing
AU  - Yang, Xunzhe
AU  - Ren, Haitao
AU  - Sun, Dawei
AU  - Dai, Yi
AU  - Zhu, Huadong
AU  - Jiang, Yinan
AU  - Zhu, Yicheng
AU  - Peng, Bin
AU  - Cui, Liying
AU  - Guan, Hongzhi
TI  - Anti-NMDAR encephalitis
AID  - 10.1212/NXI.0000000000000633
DP  - 2020 Jan 01
TA  - Neurology - Neuroimmunology Neuroinflammation
PG  - e633
VI  - 7
IP  - 1
4099  - http://nn.neurology.org/content/7/1/e633.short
4100  - http://nn.neurology.org/content/7/1/e633.full
SO  - Neurol Neuroimmunol Neuroinflamm2020 Jan 01; 7
AB  - Objective To describe the detailed clinical characteristics, immunotherapy, and long-term outcomes of patients with anti-NMDA receptor (NMDAR) encephalitis in China.Methods A 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.Results The 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).Conclusions Patients 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.AE=autoimmune encephalitis; AQP4=aquaporin-4; AZA=azathioprine; CTX=cyclophosphamide; HSV=herpes simplex virus; ICU=intensive care unit; IQR=interquartile range; IVIG=IV immunoglobulin; MMF=mycophenolate mofetil; MOG=myelin oligodendrocyte glycoprotein; mRS=modified Rankin Scale; MTX=methotrexate; NMDAR=NMDA receptor; PE=plasmapheresis; PUMCH=Peking Union Medical College Hospital; RTX=rituximab