Characteristics and Prognosis of Autoimmune Encephalitis in the East of China: A Multi-Center Study.
Characteristics and Prognosis of Autoimmune Encephalitis in the East of China: A Multi-Center Study.
复制标题
中国东部地区自身免疫性脑炎的特点和预后：一项多中心研究。
DOI：
10.3389/fneur.2021.642078
复制
发表时间：
2021
影响因子：
3.4
通讯作者：
Liu XW
中科院分区：
文献类型：
--
作者：
Qiao S;Wu HK;Liu LL;Zhang RR;Wang ML;Han T;Zhang SC;Liu XW
目的：了解中国东部地区自身免疫性脑炎(AE)的流行病学特征、临床表现及远期转归。方法：从2015年1月至2019年12月，从5个临床中心招募226例潜在的脑脊髓炎患者，符合诊断标准的患者共185例。我们回顾了AE的临床特征、辅助检查、治疗细节和结果，并确定了预后不良的危险因素。神经功能评分采用改良Rankin评分，评分3~6分为预后不良。结果：入选的AE患者主要有5种亚型：抗NMDAR 79例，抗LGI1 55例，抗CASPR2 30例，抗GABR 16例，抗AMPAR 5例。185例中男性占58.38%(108/185)，女性占41.62%(77/185)。发病时的中位年龄为41岁(四分位数范围为17-62岁)。最常见的临床表现为癫痫(146例，78.92%)和记忆障碍(123例，66.49%)。共有95例(51.35%)患者的脑部磁共振成像结果异常。脑电异常131例(70.81%)，其中168例(90.81%)接受一线免疫治疗，26例(14.05%)接受二线免疫治疗。所有存活患者均获得至少1年(12~36个月)的随访。预后良好者117例(63.24%)，预后不良者68例(36.76%)。此外，33例(17.84%)患者在出院后1年内复发，10例(5.41%)死亡。年龄较大的患者预后较差，心理行为障碍、运动障碍、意识障碍、中枢性低通气量和肿瘤的发生在预后不良组中比例过高。结论：AE是一种可治疗的疾病，多数患者预后良好。不同亚型的AE患者临床表现有一定差异。部分AE患者会复发，长期随访对进一步研究具有重要意义。
Objective: This study aimed to investigate epidemiological characteristics, clinical manifestations, and long-term outcomes of patients with autoimmune encephalitis (AE) in the east of China. Methods: From January 2015 to December 2019, 226 potential AE patients were recruited from five clinical centers, and a total of 185 patients who met the diagnostic criteria were included in the study. We retrospectively reviewed clinical features, auxiliary examinations, details of treatments, and outcomes of AE, and identified risk factors of poor prognosis. Modified Rankin Scale scores were used to evaluate neurological function, and scores of 3–6 indicated a poor-prognosis. Results: Patients with five main subtypes of AE were enrolled in the study, as follows: anti-NMDAR (79), anti-LGI1 (55), anti-CASPR2 (30), anti-GABABR (16), and anti-AMPAR (5). Among 185 patients, 58.38% (108/185) were male and 41.62% (77/185) were female. The median age at disease onset was 41 years (interquartile range, 17–62). The most common clinical manifestations of AE were seizures (146, 78.92%) and memory deficit (123, 66.49%). A total of 95 (51.35%) patients had abnormal brain magnetic resonance imaging results. Electroencephalographic findings were abnormal in 131 (70.81%) patients, and 168 (90.81%) and 26 (14.05%) patients were treated with first- and second-line immunotherapies, respectively. All surviving patients were followed-up for at least 1 year (range 12–36 months). Good clinical outcomes were achieved in 117 (63.24%), while 68 (36.76%) patients had a poor prognosis. Further, 33 (17.84%) patients relapsed and 10 (5.41%) died within 1 year post-discharge. Older patients tended to have a poorer prognosis, and the occurrence of mental behavioral disorders, movement disorders, disturbance of consciousness, central hypoventilation, and tumors were overrepresented in the poor-prognosis group. Conclusions: AE is a treatable disease, and most patients have a good prognosis. There are differences in the clinical manifestations of patients with different AE subtypes. Some with AE will relapse, and long-term follow-up is of great significance for further research.
登录
查看更多内容
影响因子：
6
作者：
Giordano, Antonino;Fazio, Raffaella;Martinelli, Vittorio
通讯作者：
Martinelli, Vittorio
影响因子：
9.9
作者：
Martinez-Hernandez, Eugenia;Guasp, Mar;Dalmau, Josep
通讯作者：
Dalmau, Josep
影响因子：
3.4
作者：
Zhao, Jing;Wang, Cancan;Guan, Hongzhi
通讯作者：
Guan, Hongzhi
影响因子：
7.3
作者：
Holz, Kristian;Prinz, Marco;Hoelscher, Christoph
通讯作者：
Hoelscher, Christoph
DOI：
10.1016/s1474-4422(15)00401-9
发表时间：
2016-04
期刊：
The Lancet. Neurology
影响因子：
--
作者：
Graus F;Titulaer MJ;Balu R;Benseler S;Bien CG;Cellucci T;Cortese I;Dale RC;Gelfand JM;Geschwind M;Glaser CA;Honnorat J;Höftberger R;Iizuka T;Irani SR;Lancaster E;Leypoldt F;Prüss H;Rae-Grant A;Reindl M;Rosenfeld MR;Rostásy K;Saiz A;Venkatesan A;Vincent A;Wandinger KP;Waters P;Dalmau J
通讯作者：
Dalmau J