血清10kD蛋白在重症肌无力病人中的特征

目的;探讨血清非免疫球蛋白成分10kD蛋白与重症肌无力的关系。方法(1)用SDS-PAGE分析健康人与MG患者的血清蛋白。(2)用抗体测血清中10kD蛋白水平。(3)观察10kD蛋白对培养的健康人及MG单核血细胞增殖的影响。结果(1)10kD与14kD扫描面积的比值存在明显差异,97％的健康人面积之比>1,而MG患者仅有30％。(2)10kD蛋白浓度在健康人及MG患者两组问不存在有意义的差别。(3)10kD蛋白100μg／ml可抑制健康人未激活的和PHA激活的单核血细胞增殖,但该蛋白和PHA对MG患者的细胞增殖无影响。结论(1)血清10kD与14kD蛋白扫描面积的比值可作为生化诊断MG的辅助参数。(2)10kD蛋白具有调节健康人单核血细胞增殖的生物活性。     

血清阴性重症肌无力研究进展

乙酰胆碱受体(acetylcholine receptor,AChR)抗体阴性的重症肌无力(myasthenia gravis,MG)称为血清阴性重症肌无力(SNMG)。SNMG在发病机制、临床表现和治疗上都与AChR抗体阳性MG不同。近年来研究者在SNMG患者血清中发现多种MG相关抗体,如肌肉特异性酪氨酸激酶(muscle-specific receptor tyro-sine kinase,MuSK)抗体、低亲和力AChR抗体、低密度脂蛋白受体相关蛋白4(low density lipoprotein receptor-related protein 4,LRP4)抗体等。本文就SNMG发病机制与临床表现的研究进展做一简要综述。     

抗体阴性重症肌无力患者血浆及其IgG和非IgG…

利用ＴＥ６７１细胞表达烟碱型乙酰胆碱受体的特性，探讨乙酸胆碱受体抗体阴性重症肌无力患者血浆和从中提取的ＩｇＧ和非－ＩｇＧ组分浆及其ＩｇＧ和非Ｉｇｇ组分对α－ＢｕＴｘ结合试验均无影响，而对ＡＣｈＲ功能有抑制作用。     

静脉大剂量免疫球蛋白治疗重症肌无力的进展

重症肌无力(MG)是较典型的神经系统自身免疫性疾病之一,其治疗方法很多,疗效各异.静脉大剂量免疫球蛋白(IVIg)治疗MG是新兴的方法之一,本文就其临床应用及机制作一简要综述.     

重症肌无力发病的非乙酰胆碱受体抗体机制

重症肌无力 (ＭＧ)是一种主要由乙酰胆碱受体 (ＡＣｈＲ)抗体介导的自身免疫性疾病。然而 ,约有 1 5 %的ＭＧ患者血清中未检测到ＡＣｈＲ抗体 ,这些患者被称为抗体阴性重症肌无力 (ｓｅｒｏｎｅｇａｔｉｖｅＭＧ ,ＳＮＭＧ) [1 ] 。由于ＳＮＭＧ患者的临床表现与抗体阳性的ＭＧ患者基本相似 ,对免疫抑制剂治疗亦敏感 ,而且给小鼠注射ＳＮＭＧ患者的血清或ＩｇＧ亦能复制出ＭＧ模型[2 ] ,因此 ,对ＭＧ发病机制而言 ,除ＡＣｈＲ抗体的介导外 ,可能还有其他的抗体或分子参与。近年来对ＭＧ发病的非ＡＣｈＲ抗体机制研究取得了一系列的进展 ,综…     

大剂量免疫球蛋白及肾上腺皮质激素治疗重症肌无力比较

目的为研究免疫球蛋白对重症肌无力（ＭＧ）的治疗效果,对６２例ＭＧ患者进行了免疫球蛋白及肾上腺皮质激素治疗的对比研究。方法３２例ＭＧ患者静脉注射大剂量免疫球蛋白（ＩＶＩｇ）,３０例ＭＧ患者应用了肾上腺皮质激素（ＡＣＳ）冲击治疗。临床绝对评分及相对评分作为治疗前后疗效判定标准。结果６２例ＭＧ患者治疗前后评分有明显差异（Ｐ＜０．０１）,ＡＣＳ治疗前后差值较ＩＶＩｇ明显加大（Ｐ＜０．０１）。结论ＩＶＩｇ治疗ＭＧ有效,ＡＣＳ治疗ＭＧ效果优于ＩＶＩｇ。ＩＶＩｇ可作为治疗ＭＧ的二线药物。     

血清阴性重症肌无力发病机制的研究进展

血清阴性重症肌无力是一种异质性疾病。大部分患者仍然是一种神经肌肉接头处自身免疫性疾病,但其发病机制不同于血清阳性重症肌无力。患者血清中的 ＩｇＧ和／或非 ＩｇＧ成分可能均参与了其致病过程,靶抗原也具有多样性。     

肌球蛋白抗体在重症肌无力病人血清中的表达及其在发病机？…

目的：探讨ｍｙｏｓｉｎ－Ａｂ在ＭＧ发病机制中所起的作用。方法：采用ＥＬＩＳＡ法检测重症肌无力（ＭＧ）组５４例,正常人组９２例及其他疾病对照组２８例血汪肿ｍｙｏｓｉｎ－Ａｂ的表达。结果：Ｍｙｏｓｉｎ－Ａｂ在血清中的正常值Ｐ／Ｎ（病人与正常人ＯＤ值之比）范围：０～２．９８。高于２．９３％则为阳性。Ｍｙｏｓｉｎ－Ａｂ阳性率与肌无力严重程度呈正相关。Ｍｙｏｓｉｎ－Ａｂ效价与ＡＣｈＲ－Ａｂ呈正相关。结论：Ｍ     

重症肌无力被动转移动物模型的研究

目的 :探讨血清阳性 (SPMG)和阴性重症肌无力 (SNMG)被动转移动物模型 (P EAMG)的异同。方法 :用ELISA法将重症肌无力 (MG)患者分为SNMG和SPMG两组 ,然后分别用两组患者血清制作P EAMG ,观察两组小鼠的临床表现、电生理及神经肌接头(NMJ)的改变。结果 :SPMG和SNMG组小鼠均表现出明显的肌无力症状 ,低频重复电刺激出现明显衰减反应 ,但SNMG组小鼠肌无力症状较SPMG组明显为轻 ,SPMG和SNMG组小鼠NMJ处棕黄色沉积物明显减少、变细短。结论 :SNMG和SPMG均是自身抗体介导的自身免疫性疾病 ,但两者不完全相同     

正常及重症肌无力肌肉蛋白成分的比较

目的：了解正常及重症肌无力（ＭＧ）肌肉蛋白成分是否存在差异。方法：用ＰＢＳ提取正常和ＭＧ肌肉的蛋白,以常量及超微量ＳＤＳ－ＰＡＧＥ分析蛋白成分;扫描计算有差异的谱带密度,并以双向电泳鉴定其组分。结果：正常肌肉有一相对分子质量约２５×１０３的蛋白明显高于ＭＧ肌肉,常量分析该谱带密度：正常肌肉为４．２０±２．３１,ＭＧ肌肉为１．４０±０．４７,差异十分显著（Ｐ＜０．０１）。微量分析该谱带密度：正常肌肉为４．６２±１．９４ＭＧ肌肉为１．６６±０．５６,差异非常显著（Ｐ＜０．００１）。双向电泳显示２５×１０３谱带至少有两个主要的蛋白成分组成,有差异的蛋白为一偏碱成分。结论：ＭＧ肌肉２５×１０３蛋白水平明显低于正常肌肉,提示该蛋白很可能与ＭＧ的发病或发展有密切的关系。     

肌球蛋白抗体在重症肌无力病人血清中的表达及其在发病机制中的作用

目的:探讨myosin-Ab在MG发病机制中所起的作用。方法:采用ELISA法检测重症肌无力(MG)组54例,正常人组92例及其他疾病对照组28例血清中myosin-Ab的表达。结果:Myosin-Ab在血清中的正常值P/N(病人与正常人OD值之比)范围:0~2.98。高于2.98则为阳性。Myosin-Ab阳性率与肌无力严重程度呈正相关。Myosin-Ab效价与AChR-Ab呈正相关。结论:Myosin-Ab虽然不是诱发MG的必要条件,但由于myosin与AChR存在抗原交叉性,myosin-Ab在MG的发病机制中可能起重要作用。     

Translation and validation of the 15‐item Myasthenia Gravis Quality of life scale in Dutch

Introduction: The 15‐item Myasthenia Gravis Quality of Life (MG‐QOL15) scale has been developed to assess the health‐related quality of life of patients with myasthenia gravis (MG). The aim of this study was to translate the original English version into Dutch and to test the test–retest reliability and construct validity. Methods: Fifty patients with MG were included. Test–retest reliability and internal consistency were assessed using the intraclass correlation coefficient (ICC) and the Cronbach α. Construct validity was assessed by testing 5 predefined hypotheses. Results: A good test–retest reliability was confirmed with an ICC of 0.866. The Cronbach α was 0.93. The predefined hypotheses were confirmed in 80% of cases, which points to good construct validity. Discussion: The Dutch MG‐QOL15 has good test–retest reliability and good construct validity. It can be used for research in a Dutch‐speaking population. It is also suitable for monitoring individual patients in clinical practice. Muscle Nerve 57 : 206–211, 2018     

AChRab阳性和阴性重症肌无力患者CD5+B细胞的研究

目的：研究ＣＤ５＋Ｂ细胞在重症肌无力（ＭＧ）发病中的作用。方法：采用免疫荧光双标记技术和流式细胞仪对３９例ＭＧ患者和１８例健康对照者周围血中ＣＤ５＋Ｂ细胞（ＣＤ５＋ＣＤ１９＋）的百分率进行测定,同时用ＥＬＩＳＡ间接法检测ＭＧ患者血清中ＡＣｈＲａｂ。结果：在ＭＧ患者周围血ＣＤ５＋Ｂ细胞百分率显著高于对照组,而且在血清ＡＣｈＲａｂ阳性和阴性ＭＧ中ＣＤ５＋Ｂ细胞均明显高于对照组;但在血清ＡＣｈＲａｂ阳性和阴性ＭＧ患者之间ＣＤ５＋Ｂ细胞百分率无显著性差异发现。结论：ＣＤ５＋Ｂ细胞与ＭＧ发病有关,但与ＡＣｈＲａｂ的产生之间无显著相关。     

History of allergic disorders in common neurologic diseases in Japanese patients

OBJECTIVE: To clarify the association between past and present history of allergic disorders and neurologic diseases. METHODS: The past and present history of common allergic disorders together with family history was prospectively studied in all out-patients at the Department of Neurology at Kyushu University Hospital from March 1998 to February 2000. RESULTS: Among 3113 out-patients, 2152 (69.1%) completed a questionnaire. Myelitis showed a statistically significant increase of concomitant atopic dermatitis (P=0.006) and concomitant and past atopic dermatitis (P=0.014), as compared with neurologically healthy controls. Moreover, patients with lower motoneuron disease (LMND) had a statistically significant increase of past and concomitant asthma (P=0.007). None of the other common neurologic diseases showed any increase of allergic disorders when compared with controls. CONCLUSIONS: The present study supports the significant association between allergic disorders and such spinal cord diseases as myelitis and LMND in Japanese patients.     

High-dose intravenous immunoglobulin for the treatment of MuSK antibody-positive seronegative myasthenia gravis

We treated two patients with anti-muscle specific tyrosine kinase (MuSK)-antibody positive seronegative myasthenia gravis (MG) with high-dose intravenous gammaglobulin (IVIg) and evaluated their clinical courses. Both patients were Japanese women, MuSK-positive seronegative MG, and were unresponsive to conventional treatments, including thymectomy, steroids, and tacrolimus. The patients required frequent hospitalization for plasmapheresis. In case 1, a 45-year-old woman, it was difficult to obtain blood access for plasmapheresis. High-dose IVIg, 400 mg/kg per day for 5 days, was administered in cases 1 and 2. In both cases, clinical improvement was observed 3 days after the start of IVIg therapy and lasted for 2 to 3 months. We propose that IVIg therapy is an effective treatment for MuSK-positive seronegative MG, when conventional treatments have failed.     

肿瘤坏死因子与重症肌无力病情的相关性研究

采用ＥＬＩＳＡ法检测了２０２例重症肌无力（ＭＧ）患者血清肿瘤坏死因子（ＴＮＦ）含量并与乙酰胆碱受体抗体（ＡＣｈＲａｂ）含量进行同步对比研究结果发现，ＴＮＦ和ＡＣｈＲａｂ均值都明显高于正常对照组（Ｐ＜０．０１），１０９例患者口服强的松治疗后，其均值都较治疗前显著降低（Ｐ＜０．０１）。据此认为，ＴＮＦ与ＭＧ发病有明显相关性，为ＭＧ的发病机理研究提供了实验室依据，并为观察ＭＧ病情轻重和评价免疫疗法治疗效果提供了实验室辅助指标。