自身免疫性脑炎临床特点及治疗分析

Clinical characteristics and treatment analysis of autoimmune encephalitis

目的总结自身免疫性脑炎的临床特点及治疗转归。方法收集2017-10—2022-03在聊城市人民医院住院治疗的18例自身免疫性脑炎患者的临床资料,回顾性分析其临床表现、实验室检查、影像学检查、脑电图和治疗转归。结果18例患者中男12例,女6例,平均发病年龄41.8岁(15~68岁)。临床表现:16例患者有癫痫发作史,13例患者认知功能下降,15例患者精神行为异常。脑脊液检查:3例患者脑脊液压力超过300 mmH_(2)O,8例患者白细胞计数升高,均低于100×10^(6)个/L,以淋巴细胞和单核细胞为主,部分可见激活单核细胞和转化淋巴细胞,8例脑脊液蛋白轻度升高,脑脊液相关抗体检测均为阳性。影像学检查:8例抗NMDAR脑炎患者发现异常,1例抗GABABR抗体相关脑炎及2例抗LGI1抗体相关脑炎患者可见颞叶及海马异常信号。脑电图检查:10例患者存在异常,重度异常者2例,中度异常者2例。治疗转归:所有患者均予以糖皮质激素联合免疫球蛋白的一线治疗,12例患者出院时改良Rankin评分0~2分。结论自身免疫性脑炎特征性临床表现较少,仍以癫痫发作、认知障碍、精神异常等表现为主,对于临床上有此表现的患者,应注意完善脑脊液、自身免疫性脑炎特异性抗体、头颅MRI、脑电图等相关检查,以期尽早明确诊断,启动免疫治疗。

Objective To summarize the clinical characteristics and treatment outcomes of autoimmune en⁃cephalitis.Methods A total of 18 patients with autoimmune encephalitis diagnosed in the Liaocheng People’s Hos⁃pital from October 2017 to March 2022 were collected,and their clinical manifestations,laboratory examinations,im⁃aging examinations,electroencephalography(EEG)and treatment outcomes were analyzed retrospectively.Results Among the 18 patients,12 were males and 6 were females,aged 15 to 68 years,with an average age of 41.8 years.The clinical manifestations were epileptic seizures in 16 cases,cognitive impairment in 13 cases,mental and behavioral disorders in 15 cases.CSF examination showed that there were 3 cases with the cerebrospinal fluid pressure exceeds 300 mmH_(2)O,8 cases with increased leukocyte number,and all lower than 100×10^(6)/L,mainly were lymphocytes and monocytes,partially visible activated monocytes and transformed lymphocytes,8 cases with mild increased protein,CSF-related antibody tests were all positive.Eight cases with anti-NMDAR encephalitis found abnormal in MRI of the brain.One case with anti-GABAB receptor encephalitis and 2 cases with anti-LGI1 antibody-related encephalitis showed temporal lobe and hippocampal abnormal signal in MRI of the brain.There were 10 cases with abnormal EEG,2 cases with severe abnormality and 2 cases with moderate abnormality.All patients were given first-line treat⁃ment of glucocorticoid combined with immunoglobulin.The modified Rankin Scale(mRS)score of 12 patients was 0-2 points on discharge.Conclusions The autoimmune encephalitis had few characteristic clinical manifestations.Epileptic seizures,cognitive impairment and mental disorders were still the main clinical manifestations.For patients with such clinical manifestations,it is necessary to improve the examination of cerebrospinal fluid,autoimmune en⁃cephalitis specific antibodies,brain MRI,and EEG,in order to clear diagnosis as early as possible and start immuno⁃therapy.