重症肌无力患者血清titin抗体

目的 探讨血清抗titin抗体对重症肌无力合并胸腺瘤诊断的临床意义。方法 用ELISA法分别测定正常对照、重症肌无力、重症肌无力合并胸腺瘤患者及非重症肌无力胸腺瘤患者血清titin抗体及乙酰胆碱受体抗体,并比较其临床意义。结果 与重症肌无力患者相比,重症肌无力合并胸腺瘤患者的血清titin抗体阳性率明显增高,分别为3％和94％,且titin抗体阳性的重症肌无力病情较重。非重症肌无力胸腺瘤患者血清中亦可检出titin抗体。结论 titin抗体检查对于重症肌无力合并胸腺瘤的诊断及判定预后具有重要临床意义。     

肌肉特异性激酶抗体阳性重症肌无力的研究进展

<正>重症肌无力(MG)是由自身抗体介导的,主要导致神经肌肉接头处(NMJ)信号传导障碍的自身免疫性疾病.80%⁹0%的MG是由乙酰胆碱受体抗体(AchRAb)介导的,在MG患者血清中能检测到AchRAb的称为血清阳性重症肌无力(SPMG),而10%90%的MG是由乙酰胆碱受体抗体(AchRAb)介导的,在MG患者血清中能检测到AchRAb的称为血清阳性重症肌无力(SPMG),而10%²0%的MG患者血清中未能检测到AchRAb,被称为血清阴性重症肌无力(SNMG).在SNMG中,有一部分患者血清中可检测到肌肉特异性激酶抗体     

重症肌无力患者肌肉特异性酪氨酸激酶抗体的研究进展

重症肌无力（myasthenia gravis,MG）是乙酰胆碱受体抗体介导的,细胞免疫依赖及补体参与的神经．肌肉接头处传递障碍的自身免疫性疾病。在重症肌无力患者中仅有约80％血清乙酰胆碱受体抗体（AchR．Ab）阳性,10％-20％血清中无AchR—Ab的患者被称为“血清反应阴性MG”（seronegative MG,SNMG）。     

肌肉与神经

腕管综合征中的皮肤静息期,糖尿病周围神经病变患者催汗与伤害感受器轴突反射的差异损伤,MuSK抗体阳性重症肌无力患者面肌的重复神经刺激,线粒体肌病患者的有氧训练与肌肉新陈代谢,肌肉特异性酪氨酸激酶抗体与乙酰胆碱受体抗体阳性重症肌无力患者四肢与面肌的单纤维肌电图,兄弟二人IIb型糖原贮积症的良性病程：自然还是营养的结果？利妥昔单抗成功治疗MuSK抗体阳性重症肌无力患者.[编者按]     

电视胸腔镜胸腺扩大切除治疗重症肌无力的体会(附14例报告)

重症肌无力（myasthemia gravis，MG）是一种神经肌肉接头处兴奋传递障碍性疾病，其特点为骨髂肌无力和疲劳。重症肌无力是一种累及神经肌肉接头处突触后膜上乙酰胆碱受体，主要以乙酰胆碱受体抗体（AchRAb）介导，细胞免疫依赖并有补体参与的自身免疫性疾病。胸腺功能异常是引起重症肌无力上述症状的重要原因。     

血浆置换术抢救重症肌无力危象7例临床观察

重症肌无力危象（myasthenic crisis,MC）是指重症肌无力（myasthenic gravis,MG）患者血清中抗乙酰胆碱受体抗体（AchRAb）明显升高,封闭或破坏神经肌肉接头突触后膜上的乙酰胆碱受体,影响信息传递从而导致肌无力。     

重症肌无力的研究进展

重症肌无力是由乙酰胆碱受体抗体介导的，针对神经肌肉接头处突触后膜上乙酰胆碱受体的自身免疫性疾病。本文主要对其发病机制中的抗体(前膜抗体、抗肌联蛋白抗体、RyR抗体)、细胞因子、淋巴细胞亚群的研究进展及神经电生理改变和治疗中的问题作一综述。     

重症肌无力患者血清兰尼定碱受体抗体的测定及临床意义

目的探讨血清兰尼定碱受体抗体测定对于重症肌无力诊断的临床意义。方法采用梯度离心法纯化兔骨骼肌肌浆网作为兰尼定碱受体抗体的抗原,ELISA法测定74例重症肌无力患者（其中21例合并胸腺瘤）血清中兰尼定碱受体抗体的水平,并比较其临床意义。结果Western blot验证梯度离心纯化的肌浆网中存在兰尼定碱受体,血清受体抗体测定在重症肌无力合并胸腺瘤患者中的阳性率较高,与重症肌无力病情严重程度密切相关。结论血清兰尼定碱受体抗体水平检测对于重症肌无力合并胸腺瘤诊断及判定预后具有重要临床意义。     

重症肌无力乙酰胆碱受体抗体的研究

重症肌无力 (ｍｙｓｔｈｅｎｉａｇｒａｖｉｓ)是累及神经肌肉接头处乙酰胆碱受体抗体 (ａｃａｔｙｌｃｈｏｌｉｎｅｒｅｃｅｐｔｏｒａｎｔｉｂｏｄｙ ,ＡｃｈＲａｂ)的自身免疫性疾病[1] 。近年来发现一系列的非ＡｃｈＲａｂ骨骼肌抗体[2 ] ,Ｋｙａｎｏｄｉｎｅ受体抗体及补体Ｃ9等均与重症肌无力的发病有关[3] 。其发病与患者体内异常增高的ＡｃｈＲａｂ造成骨骼肌终板上ＡｃｈＲａｂ数目减少及功能障碍有关。目前常用检测方法主要有放射免疫法 (ｒａｄｉｏｉｍｍｕｎｉｚａｔｉｏｎａｓｓａｙ ,ＲＩＡ )和酶联免疫吸附法 (ｅｎｚｙｍｅ…     

乙酰胆碱受体单克隆抗体对大鼠的致病性

[ 目的 ]筛选能引起重症肌无力的抗乙酰胆碱受体自身抗体 ,为探讨抗体的分子结构与致病性的关系准备材料 .[方法 ]将一株抗乙酰胆碱受体上主要免疫区的单克隆抗体A7被动地转移至健康大鼠 ,根据动物发病后的临床体征、体重丧失率和全身肌肉乙酰胆碱受体损失率判定致病性 .[结果 ]大鼠在接受A7注射后 48h即已出现严重的实验性自身免疫性重症肌无力 ,体重丧失率和全身肌肉乙酰胆碱受体损失率分别为 (6 .8± 1.7) %和 (38.4±7.2 ) % .[结论 ]A7是一株致病性很强的抗乙酰胆碱受体自身抗体     

重症肌无力患者血清中多种抗体的检测及意义

Background Antibodies against acetylcholine receptor, acetylcholinesterase, ryanodine receptor and titin have been found in patients with myasthenia gravis. However, the relations between these antibodies and character of myasthenia gravis are unknown. This study aimed to detect multiple antibodies in myasthenia gravis and to investigate its clinical significance. Methods These antibodies were detected by enzyme-linked immunoabsorbent assay in 89 cases of myasthenia gravis, 66 cases of other neurological diseases and 66 healthy controls. The incidences of antibodies were compared using the chi-square test. Results Acetylcholine receptor, acetylcholinesterase, titin and ryanodine receptor antibodies were detected in 53.9%, 20.2%, 64.0% and 55.0% of myasthenia gravis patients respectively, higher than in patients of other neurological diseases and controls groups. The combination of the four antibodies assays provided 94.4% sensitivity and 84.0% specificity for the diagnosis of myasthenia gravis. Acetylcholinesterase antibody occurred more frequently in acetylcholine receptor antibody negative patients with adverse reactions to neostigmine test. Titin antibody provided 82.1% sensitivity and 52.5% specificity for myasthenia gravis with thymoma. Incidences of titin and of ryanodine receptor antibody were higher in late onset myasthenia gravis than in early onset myasthenia gravis. The proportion of titin antibody positive patients increased with the severity of myasthenia gravis as graded by a modified Osserman scale. Conclusions Testing for acetylcholine receptor, acetylcholinesterase, titin and ryanodine receptor antibodies can offer a better diagnostic method for myasthenia gravis than each antibody test alone. Titin antibody combined with computed tomography was better for the diagnosis of thymoma. Titin antibody occurred most frequently in severe myasthenia gravis.     

重症肌无力合并胸腺瘤的CT及血清相关抗体

目的探讨胸腺瘤相关抗体与CT检查对重症肌无力合并胸腺瘤诊断的临床意义。方法对32例胸腺CT检查异常并行手术治疗的重症肌无力患者的血清titin和兰尼定碱受体(RyR)抗体进行检测,并与术后病理诊断进行比较。结果32例患者经术后病理检查,21例为胸腺瘤、11例为胸腺增生;CT检查敏感性为90·5%;CT结合胸腺瘤相关抗体检查对胸腺瘤诊断的特异性为100%。结论血清胸腺瘤相关抗体检查有助于胸腺瘤及胸腺增生的鉴别诊断,如与CT相结合,能提高其诊断胸腺瘤敏感性。     

Enzyme-linked Immunosorbent Assay for Detection of Anti-idiotype Antibodies to Antibodies to Ligand of Nicotinic Acetylcholine Receptor in Sera of Patients with Myasthenia Gravis

Anti-bungarotoxin anti-serum,which has the internal image of nicotinicacetylcholine receptor,was used as a tool to measure anti-idiotypic antibodies toantibodies to Iigand of nicotinic acctylcholine receptor in scra from 81 patients withmyasthenia gravis.Enzyme-linked immunosorbcnt assay was adopted.Thc positive ratewas 46.9%(38/81).The specific cross inhibitory test with nicotinic acetylcholinereceptor was positive.Anti-idiotype antibodies to antibodies to ligand of nicotinicacetylcholine receptor in sera of different types of myasthenia gravis patients classified ac-cording to modified Osserman's standard and myasthenia gravis patients with or withoutthymoma were comparcd in this study and the role of anti-idiotype antibodies toantibodies to Iigand of nicotinic acctylcholinc receptor in the immunity of myasthcniagravis and the possibility of thcrapeutic use of anti-idiotype antibodies arc discussed.     

胸腺瘤切除术后的重症肌无力发生情况及影响因素

目的 分析胸腺瘤切除术后的重症肌无力发生情况及影响因素,旨在为临床预防术后重症肌无力提供参考依据。 方法 选取金华市中心医院2019年3月—2020年3月收治的92例胸腺瘤切除术患者,观察并记录术后重症肌无力的发生情况,统计发生率,并根据术后是否发生重症肌无力将患者分为重症肌无力组(A组,11例)与未发生重症肌无力者(B组,81例),收集2组患者的一般资料,整理临床资料,对比2组一般资料与临床资料的差异性,应用多因素logistic回归分析法分析术后发生重症肌无力的危险因素。 结果 92例患者术后发生重症肌无力11例,发生率为12.0%;2组患者的术前病程、合并免疫疾病、手术路径、合并前纵隔脂肪清扫术、术后肺部感染、术后放化疗情况对比,差异有统计学意义(均P<0.05);多因素logistic回归分析结果显示,术前病程(>12个月)、合并免疫疾病、手术路径(开胸)、术后肺部感染是术后发生重症肌无力的危险因素,合并前纵隔脂肪清扫术、术后放化疗是其保护因素。 结论 胸腺瘤切除术后的重症肌无力发生率较高,影响因素也较多,常见如术前病程长、合并免疫疾病、开胸手术、术后肺部感染等,因此行胸腺瘤切除术时应针对患者的危险因素采取对症处理措施,以降低重症肌无力发生率,提高手术治疗效果。      

Update on myasthenia gravis

Myasthenia gravis is an autoimmune disorder caused by autoantibodies against the nicotinic acetylcholine receptor on the postsynaptic membrane at the neuromuscular junction and characterised by weakness and fatigability of the voluntary muscles. It has a bimodal peak of incidence with first peak in the third decade and the second peak in the sixth decade. It is probably underdiagnosed in the very old population. Our understanding of the pathogenesis, immunology, and molecular biology of myasthenia gravis has greatly improved in last three decades. It is almost always possible to establish the diagnosis of myasthenia gravis with the current tests. The modern treatment is highly successful and the mortality of treated myasthenia gravis is practically zero. However, there are still important gaps in our knowledge of the origin of myasthenia gravis, the factors that contribute to chronic disease, and the way to cure the disease. In this article the current knowledge of the various aspects of myasthenia gravis are outlined.     

试论重症肌无力亦属经筋病范畴

中医文献无重症肌无力的记载 ,根据其主要症状多归属于痿证范畴。笔者认为亦属于经筋病范畴 ,且属于经筋病之筋纵型。     

汤一新运用嘉州滋脾疗法治疗重症肌无力

概括汤一新老师对古人及现代医家对重症肌无力病机的认识及辨证论治,讲述汤老师自己对重症肌无力病机分析及辨证论治,并例举医案两例。     

小儿重症肌无力的发病特点(附350例分析)

350例小儿重症肌无力中,男171例,女179例,男女之比为1:1.05;发病高峰为2～4岁,占42.9%;10岁前发病者居多数,占85.4%;首发症状以眼部症状最多,占97.4%,其中尤以脸下垂最常见,占90.6%;小儿患者并非罕见,占同期诊治的重症肌无力患者总数的44.9%.     

乙酰胆碱受体单克隆抗体A49的序列分析

[ 目的 ]探讨重症肌无力自身抗体分子结构及其在发病机理中的作用 .[方法 ]利用聚合酶链反应从分泌乙酰胆碱受体单克隆抗体A49的杂交瘤细胞株扩增抗体的可变区基因 ,经转化受体菌大肠杆菌DH 5α克隆后 ,进行核苷酸序列分析 .[结果 ]A49的重链可变区基因由小鼠VHNP族基因编码 ,轻链可变区基因由小鼠Vk2组基因编码 ,与致病性乙酰胆碱受体抗体可变区核苷酸序列比较 ,其同源性均在 70 %以下 .[结论 ]A49分子结构与致病性乙酰胆碱受体抗体不同 ,A49诱导大鼠实验性自身免疫性重症肌无力的测定将有助于揭示抗体分子结构与致病性的关系     

Acetylcholine receptor antibody in the diagnosis of myasthenia gravis

The acetylcholine receptor (AchR) antibody assay has a key role in the diagnosis of myasthenia gravis. In this article, the role of AchR antibody assay in the diagnosis of ocular and generalized myasthenia gravis is reviewed, and compared to standard means of diagnosing the disease by clinical and electrophysiological methods.