French Reference Center on Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, Neuro-oncology, F-69677 Bron, France.
Infectious Diseases Department, French Institute for Public Health Surveillance, F-94410 Saint Maurice, France.
J Infect. 2014 May;68(5):419-25. doi: 10.1016/j.jinf.2013.12.001. Epub 2013 Dec 17.
For 60% of acute febrile encephalitis cases, the cause is unknown. Autoantibodies directed against different synaptic proteins or receptors in patients with autoimmune encephalitis have recently been described and could indicate a differential diagnosis of infectious encephalitis.
The aim of this study was to retrospectively investigate the presence of autoantibodies directed against synaptic proteins or receptors in patients with acute febrile encephalitis. Samples were collected in France in 2007 during a national prospective study.
A total of 253 patients with acute febrile encephalitis were enrolled in 2007. Clinical data were collected with a standardized questionnaire. When possible, cerebrospinal fluid CSF was collected and stored at -80 °C. A total of 108 CSF samples were available for retrospective autoantibody screening. Among the 108 patients, infectious etiology had been detected in 38 cases (35%); of these 38 patients, 29 (27%) had viral encephalitis, and 9 (8%) had bacterial encephalitis. No specific diagnosis was indicated for the other 70 patients (65%). Autoantibodies were detected using a cell-based assay in which HEK293 cells were transfected with plasmids coding for different synaptic proteins or receptors.
Two patients had anti-NMDA receptor antibodies (NMDAR-Abs), and all patients were negative for anti-Lgi1, CASPR2, GABABR, AMPAR, and mGluR5 antibodies. The two patients with NMDAR-Abs presented neurological and psychiatric symptoms typical of NMDAR-Abs encephalitis.
Autoimmune etiology seems to be rare (less than 2%) in patients with acute febrile encephalitis. However, patients should be systematically screened for the presence of NMDAR-Abs, particularly patients presenting with psychiatric symptoms.
对于 60%的急性热性脑炎病例，病因未知。最近描述了自身免疫性脑炎患者针对不同突触蛋白或受体的自身抗体，这可能提示感染性脑炎的鉴别诊断。
本研究旨在回顾性调查急性热性脑炎患者中是否存在针对突触蛋白或受体的自身抗体。样本于 2007 年在法国采集，来自一项全国性前瞻性研究。
2007 年共纳入 253 例急性热性脑炎患者。通过标准化问卷收集临床数据。如有可能，采集脑脊液（CSF）并储存在-80°C。共对 108 例 CSF 样本进行了回顾性自身抗体筛查。在 108 例患者中，38 例（35%）检测到感染性病因；其中 29 例（27%）为病毒性脑炎，9 例（8%）为细菌性脑炎。其余 70 例患者（65%）无明确诊断。使用基于细胞的测定法检测自身抗体，该方法将编码不同突触蛋白或受体的质粒转染到 HEK293 细胞中。
两名患者存在抗 N-甲基-D-天冬氨酸受体抗体（NMDAR-Abs），所有患者均为抗 Lgi1、CASPR2、GABABR、AMPAR 和 mGluR5 抗体阴性。两名患有 NMDAR-Abs 的患者出现了与 NMDAR-Abs 脑炎一致的神经和精神症状。
自身免疫性病因在急性热性脑炎患者中似乎很少见（<2%）。然而，患者应系统筛查 NMDAR-Abs 的存在，尤其是有精神症状的患者。
