11例自身免疫性脑炎临床分析与文献复习

发布时间：2018-05-24 07:18

本文选题：自身免疫性脑炎 + 抗NMDAR脑炎　；参考：《山东大学》2016年硕士论文

【摘要】：目的本文分析11例自身免疫性脑炎(Autoimmune encephalitis, AE)患者的一般信息、临床表现、实验室检验、影像学检查、脑电图(Electroencephalogram, EEG)结果、治疗方案以及疗效,并结合国内外相关文献,全面讨论各型AE的流行病学特点、发病机制、临床表现、辅助检查检验、诊断、治疗及预后,为临床提供参考。资料与方法本研究收集在2015年3月至2016年2月期间,于山东大学齐鲁医院神经内科住院治疗,最后诊断为AE的11例患者,收集患者的一般信息、临床表现、颅脑磁共振成像(Magnetic resonance Imaging, MRI)、EEG、脑脊液(Cerebrospinal fluid, CSF)及血清学检验、治疗经过和疗效。对收集的11例AE患者资料进行回顾性分析。根据所检测的抗体不同,将患者分成不同类型的AE,采用频数或百分数描述各组具体情况,分析各类型AE的特点。结果收集符合脑炎表现并于CSF中检测到神经细胞表面抗体(Neuronal surface antibodies, NSAbs)的11例患者,其中抗N-甲基-D-天冬氨酸受体(N-methyl-D-aspartate receptor, NMDAR)脑炎7例、抗富亮氨酸胶质瘤失活蛋白1 (Leucine-rich glioma inactivated 1, LGI1)脑炎3例、抗γ-氨基丁酸B受体(γ- aminobutyric acid-B receptor, GABABR)脑炎1例。其中男性患者7例,女性患者4例,年龄为19-71岁。患者的临床表现包括神经精神症状,癫痫,认知功能减退,烦躁或意识模糊。血清学检测显示6例患者血生化异常,其中4例存在低钠血症或低氯血症。6例患者肿瘤系列结果异常,提示可能存在潜在的肿瘤。CSF中主要是以小淋巴细胞为主的白细胞计数轻度升高,最高达120×106/L,蛋白含量正常或轻度升高,最高至0.79g/L。6例患者颅脑MRI异常,其中3例为缺血变性灶,1例为海马、颞叶异常信号,1例为额顶叶、枕叶异常信号,1例为脑动脉硬化,余5例患者结果示未见明显异常。11例患者在住院期间进行肿瘤筛查,1例患者发现患有子宫肌瘤,1例患者发现肺癌,但均未行病理学检查确诊。11例患者中有10例存在脑电图异常,仅1例正常,其中1例结果异常患者显示δ刷样慢活动。11例患者中均使用免疫抑制治疗,主要是糖皮质激素和静脉注射用免疫球蛋白(Intravenous immunoglobulin, IVIG)。大部分患者治疗有效,但出院时仍存在部分认知功能障碍。结论自身免疫性脑炎主要表现为神经精神症状,癫痫,认知功能减退,烦躁或意识模糊。辅助检查中应进行相关血液化验以排除其它疾病,并应进行肿瘤的筛查。CSF改变主要是白细胞计数轻度升高,蛋白含量正常或轻度升高。颅脑MRI显示正常或边缘性脑炎,EEG检查一般为异常,但缺乏特异性,然而此两项检查对其它疾病的排除非常重要。目前国际上尚无统一的AE诊断标准,临床上主要依据详细的病史和辅助检查进行诊断,特异性NSAbs检测阳性可明确诊断。AE的治疗主要是免疫抑制治疗,若患者伴发肿瘤,应对相关肿瘤进行处理。
[Abstract]:Objective to analyze the general information, clinical manifestation, laboratory examination, imaging examination, electroencephalogram (EEG) electroencephalograms (EEGG) results, therapeutic schemes and efficacy of 11 patients with autoimmune encephalitis (AEE). The epidemiological characteristics, pathogenesis, clinical manifestations, auxiliary examination, diagnosis, treatment and prognosis of various types of AE were discussed. Materials and methods from March 2015 to February 2016, 11 patients with AE were admitted to the Department of Neurology, Qilu Hospital, Shandong University, and their general information and clinical manifestations were collected. Magnetic resonance Imaging, Cerebrospinal fluidid, Cerebrospinal fluid (CSF) and serological examination, course of treatment and curative effect. The data of 11 AE patients were analyzed retrospectively. According to the different antibodies detected, the patients were divided into different types of AEs. The specific conditions of each group were described by frequency or percentage, and the characteristics of each type of AE were analyzed. Results 11 patients with neuronal surface antibodies, NSAbs) were detected in CSF. Among them, 7 were anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. There were 3 cases of Leucine-rich glioma inactivated 1 (LGI1) encephalitis and 1 case of 纬 -aminobutyric B receptor (GABA ABR) encephalitis. There were 7 males and 4 females aged 19-71 years. Clinical manifestations include neuropsychiatric symptoms, epilepsy, cognitive impairment, irritability or confusion. The results of serological examination showed that 6 patients had abnormal blood biochemistry, 4 of them had hyponatremia or hypochloremia, and 6 patients had abnormal tumor series. These results suggest that there may be a slight increase in the white blood cell count, mainly small lymphocytes, in the potential tumor. The highest level is 120 脳 10 6 / L, the protein content is normal or slightly elevated, and the maximum is abnormal MRI in the brain of 0.79g/L.6 patients. 3 cases were ischemic degeneration, 1 case was hippocampus, 1 case was frontal and parietal lobe, 1 case was cerebral arteriosclerosis. The results of the remaining 5 patients showed that there was no obvious abnormality. 11 patients underwent tumor screening during hospitalization. One patient found that 1 patient had uterine myoma and 1 patient had lung cancer. However, 10 out of 11 patients who were not diagnosed by pathological examination had abnormal electroencephalogram (EEG). One patient with abnormal results showed 未 brush-like slow activity. 11 patients were treated with immunosuppressive therapy, mainly glucocorticoid and intravenous immunoglobulin (IVIGG). Most of the patients were effective, but there were still some cognitive impairment at discharge. Conclusion the main manifestations of autoimmune encephalitis are neuropsychiatric symptoms, epilepsy, cognitive impairment, irritability or confusion. The related blood tests should be carried out in order to exclude other diseases, and the changes of tumor screening should be mainly caused by slight increase in white blood cell count, normal or slight increase in protein content. Craniocerebral MRI shows normal or marginal encephalitis usually abnormal, but it lacks specificity. However, these two examinations are very important for the exclusion of other diseases. At present, there is no unified diagnostic standard for AE in the world. Clinical diagnosis is mainly based on detailed medical history and auxiliary examination. Specific NSAbs positive detection can clearly diagnose the treatment of immunosuppressive therapy, if the patient is accompanied by tumor, The associated tumors should be treated.
【学位授予单位】：山东大学
【学位级别】：硕士
【学位授予年份】：2016
【分类号】：R742.9

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