自身免疫性脑炎的临床特点与预后分析

Clinical characteristics and outcome of patients with autoimmune encephalitis

目的:分析自身免疫性脑炎(AE)的临床特点及影响预后的危险因素。方法:收集40例AE患者临床资料,分析该病的发病特点,并采用Logistic回归分析影响预后的危险因素。结果:本研究纳入5类AE患者,共40例(男19,女21),年龄2~68岁,高峰期16~30岁,中位确诊时间达24. 5 d,合并肿瘤者5例(12. 5%),主要临床症状包括精神行为异常(55%)、痫性发作(65%)、头痛(35%)、认知功能下降(32. 5%)、意识模糊(27. 5%)等。治疗方面,14例仅使用小剂量激素,10例采用单纯激素冲击,16例采用激素冲击+丙种球蛋白/免疫抑制剂的联合方案。血清和脑脊液致病抗体滴度均与初始病情严重程度有关(P=0. 014、0. 043)。出院时,26例(65%)患者短期预后良好。随访结束时,30例(75%)患者长期预后良好。脑脊液异常、进ICU、脑脊液抗体滴度升高是短期预后不佳的独立危险因素(P=0. 024、0. 037、0. 047),而年龄> 60岁、认知功能下降、气管插管是影响长期预后的独立危险因素(P=0. 027、0. 026、0. 017)。结论:AE以青少年为主,临床表现多样,致病抗体滴度与初始病情严重程度有关。脑脊液抗体滴度是反映短期预后的重要因素,与长期预后无关。

Objective: To analyze the clinical characteristics and related prognostic factors in children and adults with autoimmune encephalitis( AE). Methods: The clinical data and disease characteristics of 40 patients diagnosed as having AE upon admission and during follow-up were reported,and prognostic factors were investigated by logistic regression analysis. Results: Forty patients( 19 men and 21 women) with AE were collected with age ranging from 2 to 68 years old. Age of peak onset was between 16 and 30 years old. Average duration till the diagnosis was 24 days. Five cases( 12. 5%) were also found to have cancer. The main symptoms were mental and behavioral abnormalities( 55%),seizure attack( 65%),headache( 35%),cognitive decline( 32. 5%),and confusion( 27. 5%). Ten cases received high-dose glucocorticoid therapy and 16 cases were treated with corticosteroid therapy combined with intravenous immunoglobulin or immunosuppressive drugs. The antibody titer in the serum and cerebrospinal fluid( CSF) was associated with severity of the disease. Twenty-six patients( 65%) recovered well upon discharge,and 30( 75%) had good outcome at the end of follow-up. CSF abnormalities,intensive care unit admission and antibody titer in CSF were the independent risk factors for worse short-term outcome.At the same time,Age of more than 60 years old,cognitive decline and trachea intubation were associated with worse longterm prognosis. Conclusion: AE of various types presents with complex clinical manifestations. The antibody titer in the serum and CSF is associated with severity of the disease at onset. The antibody titer in CSF is an important factor for short-term other than long-term prognosis.