自身免疫性脑炎患者临床特征及诊断和治疗的回顾性分析与研究

发布时间：2018-03-08 21:37

本文选题：脑炎　切入点：自身免疫疾病　出处：《浙江大学》2017年硕士论文　论文类型：学位论文

【摘要】：目的回顾性分析和研究自身免疫性脑炎患者的临床特征,诊断和治疗结果,并探讨2016 Lancet Neurol发表的临床诊断新标准在自身免疫性脑炎诊断和治疗中的指导意义。方法收集2014年9月-2017年2月期间就诊于浙江大学附属邵逸夫医院神经内科,确诊或临床诊断考虑自身免疫性脑炎患者的临床资料。根据现行的诊断标准分为自身免疫性脑炎抗体阳性和自身免疫性脑炎抗体阴性(含未检测抗体)两组,分析和描述两组患者的临床表现和实验室检查(包括脑脊液(cerebrospinal fluid,CSF)、颅脑磁共振成像(magnetic resonance imaging,MRI)、脑电图(electroencephalogram,EEG)、肿瘤筛查和共病检查)特点。进一步根据2016 Lancet Neurol发布的自身免疫性脑炎诊断新标准,将20例患者进行自身免疫性脑炎的诊断分级(确诊,拟诊或可能),讨论分析各级自身免疫性脑炎的诊断要点。在上述基础上,分析和评价20例患者的治疗经过和结果。使用改良RANKIN量表(modified Rankin Scale,mRS)评估患者治疗前后的神经评分,并统计分析治疗前后mRS评分以及mRS评分改善与开始免疫治疗的时间或患者年龄之间的相关性。结果首先,我们分析患者的临床特点,结果表明,20例考虑自身免疫性脑炎患者中,男性11例,女性9例,年龄为16-68岁,中位数为40岁,CSF自身免疫性脑炎相关抗体阳性组患者10例(50%),包括抗N-甲基-D-天冬氨酸受体(N-methyl-D-aspartatereceptor,NMDAR)脑炎6例,抗富亮氨酸胶质瘤失活蛋白(Leucine-rich glioma inactivated 1,LGI1)脑炎 1 例,抗γ-氨基丁酸 B 受体(γ-aminobutyric acid-B receptor,GABABR)脑炎 1 例,抗 amphiphysin 抗体脑炎2例。CSF抗体阴性和未检测抗体组患者10例(50%)。20患者的临床主要表现为亚急性的发病以及快速进展的精神异常、癫痫发作、意识改变、运动障碍和记忆减退。其中,抗体阳性组患者最常见的临床表现为精神障碍,而抗体阴性组患最常见表现为癫痫。实验室检查发现,12例(60%)患者提示不同程度的CSF白细胞异常。16例(80%)患者头颅影像学检查表现脑部病变,11例(55%)患者表现为单侧或双侧的边缘系统受累,6例(30%)提示边缘系统外病变。15例(75%)显示EEG异常。5例(25%)患者合并肿瘤或肿瘤指标升高。其次,我们进一步根据2016临床诊断新标准,20例患者根据临床神经系统评估和常规实验室检查(CSF,头颅MRI和EEG)和合理的排除其他病因,可确诊自身免疫性脑炎12例,拟诊3例,可能5例。对于抗体阴性的患者,仍可临床确诊为自身免疫性脑炎。最后,我们表明20例自身免疫性脑炎患者中有19例患者进行了免疫治疗,包括激素、免疫球蛋白、血浆置换和环磷酰胺等,自身免疫性脑炎常见症状意识障碍、癫痫、神经症状、运动和记忆障碍以及患者的实验室检查明显好转。所有19例患者以及12例确诊患者的免疫治疗前后mRS评分比较有明显差异(p0.01或p0.05)。患者治疗后mRS评分改善与起病至开始免疫治疗的时间(而非患者年龄)密切相关(r=-0.565,p0.01)。结论1.我们的数据表明,自身免疫性脑炎患者的主要临床表现为亚急性起病的精神异常、癫痫发作、意识改变、运动障碍和记忆障碍。典型的影像学改变为单侧或双侧的边缘系统受累。CSF中阳性的自身免疫性脑炎抗体和细胞增多以及EEG异常也是自身免疫性脑炎的重要特征。2.2016临床诊断新标准不完全依赖抗体检测,根据相对特异临床表现以及实验室检查,可实现自身免疫性脑炎的早期分级诊断和早期免疫治疗。3.我院的临床治疗实践表明,免疫治疗是自身免疫性脑炎的主要治疗方法。早期免疫治疗是提高自身免疫性脑炎疗效和改善预后的关键因素。基于临床诊断新标准的综合诊断对实现自身免疫性脑炎的早期治疗具有重要指导意义。
[Abstract]:Objective To retrospectively analyze and study the clinical characteristics of autoimmune encephalitis patients, the results of diagnosis and treatment, and to explore the significance of the new standard of clinical diagnosis of 2016 Lancet Neurol published in autoimmune encephalitis diagnosis and treatment. Methods from September 2014 -2017 year in February in Zhejiang University Hospital Affiliated Sir Run Run Shaw Hospital neurology, diagnosis or clinical diagnosis consideration the clinical data of autoimmune encephalitis patients. According to the diagnostic criteria of the current divided into autoimmune encephalitis antibodies and autoimmune encephalitis antibody negative (without antibody) two groups, analyze and describe the clinical manifestations and laboratory examinations of the two groups of patients (including cerebrospinal fluid (cerebrospinal fluid, CSF), brain magnetic resonance imaging (magnetic resonance imaging, MRI) (electroencephalogram, EEG), EEG, cancer screening and comorbidity). According to the 20 check The new standard for the diagnosis of autoimmune encephalitis 16 Lancet Neurol released, 20 patients were diagnosed classification of autoimmune encephalitis (confirmed, suspected or possible), diagnosis of various autoimmune encephalitis are discussed. On the basis of the above analysis, treatments and results of analysis and evaluation of 20 patients improved. The RANKIN scale (modified Rankin Scale, mRS) before and after treatment in patients with neurological evaluation score, the results before and after treatment mRS score and mRS score improvement and start correlation between immune treatment time or the age of the patients and statistical results. First, we analyze the clinical characteristics of patients, the results showed that 20 cases were considered autoimmune encephalitis patients in 11 cases of male and 9 females, the median age was 16-68 years, 40 years, CSF related autoimmune encephalitis antibody positive patients 10 cases (50%), including anti N- methyl -D- aspartate receptor (N-methy L-D-aspartatereceptor, NMDAR) 6 cases of encephalitis, anti leucine rich glioma inactivated protein (Leucine-rich glioma inactivated 1, LGI1) in 1 cases of encephalitis, anti GABA B receptor (gamma -aminobutyric acid-B receptor, GABABR) in 1 cases of encephalitis, encephalitis and 2 cases of anti amphiphysin antibody.CSF antibody negative and not detected in 10 cases of patients with antibody (50%).20 patients mainly manifested as acute onset and rapid progression of psychosis, seizures, altered consciousness, movement disorders and memory loss. Among them, the most common clinical antibody positive patients is a mental disorder, and negative group suffering from the most common manifestations of epilepsy. Laboratory examination found. 12 cases (60%) patients with abnormal.16 CSF white blood cells of patients with different degrees (80%) showed brain lesions in patients with brain imaging, 11 cases (55%) involved in the limbic system patients showed unilateral or bilateral, 6 cases (30 That limbic system lesions%).15 cases (75%) showed abnormal EEG.5 cases (25%) patients with elevated tumor or tumor index. Secondly, we further according to the new standard of 2016 clinical diagnosis, 20 patients according to clinical neurological assessment and routine laboratory examinations (CSF, head MRI and EEG) and reasonable to exclude other etiology, diagnosis of autoimmune encephalitis in 12 cases, 3 cases of suspected cases, May 5. For the antibody negative patients, can be diagnosed as autoimmune encephalitis. Finally, we show that 20 cases of autoimmune encephalitis in 19 patients who underwent immunotherapy, including hormones, immune globulin. Plasmapheresis and cyclophosphamide, autoimmune encephalitis, common symptoms of consciousness disorders, epilepsy, neurological symptoms, motor and memory disorders and patients with laboratory examination significantly improved. All 19 cases of patients before and after treatment of 12 cases of patients diagnosed by mRS There was significant difference between the scores (P0.01 or P0.05). After the treatment of patients with mRS score and improve the onset to treatment time to immune (rather than age) are closely related (r=-0.565, P0.01) 1.. Conclusion our data suggest that the main clinical autoimmune encephalitis patients were subacute onset of psychosis, epilepsy seizures, altered consciousness, movement disorders and memory disorders. Typical imaging changes increased positive for unilateral or bilateral limbic involvement in.CSF autoimmune encephalitis antibody and cell EEG and abnormal autoimmune inflammation of the brain is an important characteristic of.2.2016 clinical diagnosis standard does not depend entirely on the new antibody detection, according to the clinical manifestations of specific and the laboratory examination, clinical practice shows that autoimmune encephalitis can achieve early diagnosis and early treatment of.3. immune classification in our hospital, immunotherapy of autoimmune Early treatment is the key factor to improve the curative effect and prognosis of autoimmune encephalitis. The comprehensive diagnosis based on the new standard of clinical diagnosis is of great guiding significance for the early treatment of autoimmune encephalitis.

【学位授予单位】：浙江大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R742.9

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