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自身免疫性腦炎患者臨床特征及診斷和治療的回顧性分析與研究

發(fā)布時(shí)間:2018-03-08 21:37

  本文選題:腦炎 切入點(diǎn):自身免疫疾病 出處:《浙江大學(xué)》2017年碩士論文 論文類型:學(xué)位論文


【摘要】:目的回顧性分析和研究自身免疫性腦炎患者的臨床特征,診斷和治療結(jié)果,并探討2016 Lancet Neurol發(fā)表的臨床診斷新標(biāo)準(zhǔn)在自身免疫性腦炎診斷和治療中的指導(dǎo)意義。方法收集2014年9月-2017年2月期間就診于浙江大學(xué)附屬邵逸夫醫(yī)院神經(jīng)內(nèi)科,確診或臨床診斷考慮自身免疫性腦炎患者的臨床資料。根據(jù)現(xiàn)行的診斷標(biāo)準(zhǔn)分為自身免疫性腦炎抗體陽(yáng)性和自身免疫性腦炎抗體陰性(含未檢測(cè)抗體)兩組,分析和描述兩組患者的臨床表現(xiàn)和實(shí)驗(yàn)室檢查(包括腦脊液(cerebrospinal fluid,CSF)、顱腦磁共振成像(magnetic resonance imaging,MRI)、腦電圖(electroencephalogram,EEG)、腫瘤篩查和共病檢查)特點(diǎn)。進(jìn)一步根據(jù)2016 Lancet Neurol發(fā)布的自身免疫性腦炎診斷新標(biāo)準(zhǔn),將20例患者進(jìn)行自身免疫性腦炎的診斷分級(jí)(確診,擬診或可能),討論分析各級(jí)自身免疫性腦炎的診斷要點(diǎn)。在上述基礎(chǔ)上,分析和評(píng)價(jià)20例患者的治療經(jīng)過(guò)和結(jié)果。使用改良RANKIN量表(modified Rankin Scale,mRS)評(píng)估患者治療前后的神經(jīng)評(píng)分,并統(tǒng)計(jì)分析治療前后mRS評(píng)分以及mRS評(píng)分改善與開始免疫治療的時(shí)間或患者年齡之間的相關(guān)性。結(jié)果首先,我們分析患者的臨床特點(diǎn),結(jié)果表明,20例考慮自身免疫性腦炎患者中,男性11例,女性9例,年齡為16-68歲,中位數(shù)為40歲,CSF自身免疫性腦炎相關(guān)抗體陽(yáng)性組患者10例(50%),包括抗N-甲基-D-天冬氨酸受體(N-methyl-D-aspartatereceptor,NMDAR)腦炎6例,抗富亮氨酸膠質(zhì)瘤失活蛋白(Leucine-rich glioma inactivated 1,LGI1)腦炎 1 例,抗γ-氨基丁酸 B 受體(γ-aminobutyric acid-B receptor,GABABR)腦炎 1 例,抗 amphiphysin 抗體腦炎2例。CSF抗體陰性和未檢測(cè)抗體組患者10例(50%)。20患者的臨床主要表現(xiàn)為亞急性的發(fā)病以及快速進(jìn)展的精神異常、癲癇發(fā)作、意識(shí)改變、運(yùn)動(dòng)障礙和記憶減退。其中,抗體陽(yáng)性組患者最常見的臨床表現(xiàn)為精神障礙,而抗體陰性組患最常見表現(xiàn)為癲癇。實(shí)驗(yàn)室檢查發(fā)現(xiàn),12例(60%)患者提示不同程度的CSF白細(xì)胞異常。16例(80%)患者頭顱影像學(xué)檢查表現(xiàn)腦部病變,11例(55%)患者表現(xiàn)為單側(cè)或雙側(cè)的邊緣系統(tǒng)受累,6例(30%)提示邊緣系統(tǒng)外病變。15例(75%)顯示EEG異常。5例(25%)患者合并腫瘤或腫瘤指標(biāo)升高。其次,我們進(jìn)一步根據(jù)2016臨床診斷新標(biāo)準(zhǔn),20例患者根據(jù)臨床神經(jīng)系統(tǒng)評(píng)估和常規(guī)實(shí)驗(yàn)室檢查(CSF,頭顱MRI和EEG)和合理的排除其他病因,可確診自身免疫性腦炎12例,擬診3例,可能5例。對(duì)于抗體陰性的患者,仍可臨床確診為自身免疫性腦炎。最后,我們表明20例自身免疫性腦炎患者中有19例患者進(jìn)行了免疫治療,包括激素、免疫球蛋白、血漿置換和環(huán)磷酰胺等,自身免疫性腦炎常見癥狀意識(shí)障礙、癲癇、神經(jīng)癥狀、運(yùn)動(dòng)和記憶障礙以及患者的實(shí)驗(yàn)室檢查明顯好轉(zhuǎn)。所有19例患者以及12例確診患者的免疫治療前后mRS評(píng)分比較有明顯差異(p0.01或p0.05);颊咧委熀髆RS評(píng)分改善與起病至開始免疫治療的時(shí)間(而非患者年齡)密切相關(guān)(r=-0.565,p0.01)。結(jié)論1.我們的數(shù)據(jù)表明,自身免疫性腦炎患者的主要臨床表現(xiàn)為亞急性起病的精神異常、癲癇發(fā)作、意識(shí)改變、運(yùn)動(dòng)障礙和記憶障礙。典型的影像學(xué)改變?yōu)閱蝹?cè)或雙側(cè)的邊緣系統(tǒng)受累。CSF中陽(yáng)性的自身免疫性腦炎抗體和細(xì)胞增多以及EEG異常也是自身免疫性腦炎的重要特征。2.2016臨床診斷新標(biāo)準(zhǔn)不完全依賴抗體檢測(cè),根據(jù)相對(duì)特異臨床表現(xiàn)以及實(shí)驗(yàn)室檢查,可實(shí)現(xiàn)自身免疫性腦炎的早期分級(jí)診斷和早期免疫治療。3.我院的臨床治療實(shí)踐表明,免疫治療是自身免疫性腦炎的主要治療方法。早期免疫治療是提高自身免疫性腦炎療效和改善預(yù)后的關(guān)鍵因素;谂R床診斷新標(biāo)準(zhǔn)的綜合診斷對(duì)實(shí)現(xiàn)自身免疫性腦炎的早期治療具有重要指導(dǎo)意義。
[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.

【學(xué)位授予單位】:浙江大學(xué)
【學(xué)位級(jí)別】:碩士
【學(xué)位授予年份】:2017
【分類號(hào)】:R742.9

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本文編號(hào):1585679


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