Fu Z Y, Ren H T, Xue L P, Tian Y N, Fan S Y, Zhao Y H, Lin N, Yin H X, Jiang N, Peng B, Cui L Y, Guan H Z
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
Department of Neurology, Shanxi Bethune Hospital, Taiyuan 030032, China.
Zhonghua Yi Xue Za Zhi. 2020 Jul 7;100(25):1933-1936. doi: 10.3760/cma.j.cn112137-20200115-00095.
To explore the clinical features of post-viral-encephalitis autoimmune encephalitis (PVEAE). Ten cases of PVEAE, who were hospitalized in the Neurology Department of Peking Union Medical College Hospital (PUMCH) between November 2014 and October 2019, were retrospectively reviewed. Clinical manifestation, immunology, neuroradiology, treatment and outcomes were analyzed. There were 5 males and 5 females, with a median age of 44 (18, 66) years. Of 9 cases, the median interval between the two onsets of encephalitis was 37 (24, 60) days, and there was no obvious interval in case 7. In viral encephalitis phase, the peak modified Rankin scale (mRS) was 4.5 (4.0, 5.0) and the remission mRS was 2.0 (1.0, 3.0). In autoimmune encephalitis (AE) phase, the peak mRS was 4.0 (3.0, 5.0). Symptoms of AE included mental and behavioral abnormalities (10/10), amnesia (10/10), motor disorders (5/10), autonomic dysfunction (5/10), speech disorders (4/10), seizures (2/10) and consciousness disturbance (2/10). On average, each case presented with 4 (2, 6) symptoms. In AE phase, the positive rate of anti-N-methyl-D-aspartate (anti-NMDA) receptor antibody in cerebrospinal fluid (CSF) was 80% (8/10), while in serum it was only 20% (2/10). Neuroimaging showed that in AE phase, the lesions expanded in 8 cases, remained unchanged in 1 case and shrank in 1 case. In AE phase, 10 cases received first line treatments, and 2 cases accepted long-course immunotherapy. After treatment, symptoms of 9 cases were obviously relieved. The mRS for short-term and long-term outcomes was 2.0 (1.0, 4.0) and 1.0 (0, 2.0), respectively. PVEAE might present with either typical biphasic course or monophasic/pseudo-monophasic course. In AE phase, anti-NMDA receptor antibody turned positive in most cases. Much importance should be attached to the recognition and diagnosis of PVEAE and treat it actively thereafter.
探讨病毒性脑炎后自身免疫性脑炎（PVEAE）的临床特征。回顾性分析2014年11月至2019年10月在北京协和医院神经内科住院的10例PVEAE患者的临床表现、免疫学、神经影像学、治疗及预后情况。患者共10例，男女各5例，中位年龄44（18，66）岁。9例患者两次脑炎发作的中位间隔时间为37（24，60）天，7号病例无明显间隔。在病毒性脑炎阶段，改良Rankin量表（mRS）峰值为4.5（4.0，5.0），缓解时mRS为2.0（1.0，3.0）。在自身免疫性脑炎（AE）阶段，mRS峰值为4.0（3.0，5.0）。AE症状包括精神和行为异常（10/10）、失忆（10/10）、运动障碍（5/10）、自主神经功能障碍（5/10）、言语障碍（4/10）、癫痫发作（2/10）和意识障碍（2/10）。平均每例出现4（2，6）种症状。在AE阶段，脑脊液（CSF）中抗N-甲基-D-天冬氨酸（anti-NMDA）受体抗体阳性率为80%（8/10），而血清中仅为20%（2/10）。神经影像学显示，在AE阶段，8例病变扩大，1例不变，1例缩小。在AE阶段，10例接受一线治疗，2例接受长疗程免疫治疗。治疗后，9例症状明显缓解。短期和长期预后的mRS分别为2.0（1.0，4.0）和1.0（0，2.0）。PVEAE可能表现为典型的双相病程或单相/假单相病程。在AE阶段，多数病例抗NMDA受体抗体呈阳性。应重视PVEAE的识别与诊断，并积极进行治疗。
