重症肌无力患者胸腺手术后血清AchRab和CAEab的变化

目的：研究重症肌无力（ＭＧ）患者手术后血清乙酰胆碱受体抗体（ＡｃｈＲａｂ）与抗骨骼肌柠檬酸提取物抗体（ＣＡＥａｂ）的变化。方法：用固相酶联免疫吸附法测定血清ＡｃｈＲａｂ和ＣＡＥａｂ。结果：ＭＧ患者血清ＡｃｈＲａｂ和ＣＡＥａｂ的水平明显高于对照组（Ｐ＜０．０１）。ＡｃｈＲａｂ水平在手术后１个月开始下降（Ｐ＜０．０５）,在手术后１年下降更明显（Ｐ＜０．０１）;ＣＡＥａｂ水平在手术后１个月和６个月下降不明显（Ｐ＞０．０５）,在手术后１年下降有显著性差异（Ｐ＜０．０５）。手术后１年患者血清中ＡｃｈＲａｂ和ＣＡＥａｂ水平仍高于对照组（Ｐ＜０．０１）。结论：手术后血清ＡｃｈＲａｂ和ＣＡＥａｂ的检测有助于了解患者的病情。     

重症肌无力患者胸腺手术后血清AchRab和CAEab的变化

目的：研究重症肌无力（MG）患者手术后血清乙酰胆碱受体抗体（AchRab）与抗骨骼肌柠檬酸提取物抗体（CAEab）的变化。方法：用固相酶联免疫吸附法测定血清AchRab和CAEab。结果：MG患者血清AchRab和CAEab的水平明显高于对照组（P＜0．01）。AchRab水平在手术后1个月开始下降（P＜0．05）,在手术后1年下降更明显（P＜0．01）;CAEab水平在手术后1个月和6个月下降不明显（P＞0．05）,在手术后1年下降有显著性差异（P＜0．05）。手术后1年患者血清中AchRab和CAEab水平仍高于对照组（P＜0．01）。结论：手术后血清AchRab和CAEab的检测有助于了解患者的病情。     

胸腺切除对重症肌无力病人AChRAb,PsmRAb水平的影响

目的:观察胸腺切除对重症肌无力的影响。方法:采用ELISA法,对比了手术前后病人周围血中乙酰胆碱受体抗体及突触前膜受体抗体水平的变化。结果:伴发胸腺瘤的病者,乙酰胆碱受体抗体及突触前膜受体抗体水平高于伴发胸腺增生的病者;手术切除增生的胸腺可引起乙酰胆碱受体抗体水平的下降,而手术切除胸腺瘤可引起突触前膜受体抗体水平的增加。结论:术后周围血中抗PsmRAb水平增加,机制有待研究。     

重症肌无力和胸腺的关系(综述)

该文概述了重症肌无力(MG)发病和胸腺的关系,以及和胸腺相关的MG治疗进展。提示对于疗效不理想的免疫性疾病MG,将有望在针对胸腺的免疫治疗方面取得突破性进展。     

血清乙酰胆碱受体抗体、突触前膜抗体和胸腺瘤相关抗体与重症肌无力发病关系的研究

本研究用ＥＬＩＳＡ法同步、动态地检测了２８５例ＭＧ患者血清乙酰胆碱受体抗体（ＡｃｈＲａｂ）、突触前膜抗体（ＰｓＭａｂ）和胸腺瘤相关抗体（ＣＡＥａｂ）的变化。实验结果表明，ＭＧ患者体内增高的ＡｃｈＲａｂ是ＭＧ发病的主要体液因素。部分血清ＡｃｈＲａｂ阴性ＭＧ患者（１７．５％）的发病，可能与血清ＰｓＭａｂ异常有关。另一部分血清ＡｃｈＲａｂ和ＰｓＭａｂ均异常的ＭＧ患者（１２．６％），则可能同时存在骨骼肌神经肌接头突触前和突触后的损害。血清ＣＡＥａｂ测定在辅助诊断胸腺瘤方面具有高度的灵敏度和特异性，可作为临床早期诊断胸腺瘤的过筛检查。     

血清CAE-Ab、nAchR-Ab检测对伴发胸腺瘤的重症肌无力患者早期诊断意义

目的探讨柠檬酸提取物抗体(CAE-Ab)及烟碱型乙酰胆碱受体抗体(nAchR-Ab)测定对临床早期诊断伴发胸腺瘤的重症肌无力(MG)患者的价值,与改良Osserman分型及病理分型的关系.方法采用酶联免疫吸附法检测20例伴发胸腺瘤的重症肌无力患者的血清CAE-Ab、nAchR-Ab.结果 CAE-Ab阳性率75%,nAchR-Ab阳性率60%,大多在Ⅱa型与Ⅱb型患者.上皮细胞为主型4例中Ⅱb型3例、CAE-Ab均阳性,术后发生肌无力危象2例,1例死亡.结论 CAE-Ab及nAchR-Ab检测可作为临床诊断 MG患者伴发胸腺瘤的筛选检查;术后病理分型可指导治疗和判断预后.     

重症肌无力患者血清Ryanodine受体抗体与临床的相关性研究

目的 探讨血清Ryanodine受体抗体(RyR-Ab)与重症肌无力(MG)的关系.方法 应用ELISA和Western blot法分别检测81例MG患者、45例非MG神经系统其他疾病(NMG)患者和50例健康对照者(HC)血清中RyR-Ab水平.结果 ELISA法显示:MG组RyR-Ab阳性率为23.4%.明显高于HC组和NMG组(均为阴性),差异有统计学意义(P<0.05);MG伴胸腺瘤(MGT)组阳性率最高(77.2%),MG伴胸腺萎缩(MGA)者次之(14.2%),MG伴胸腺增生(MGH)者为6.6%;而且RyR-Ab水平与肌无力的严重程度成正相关(r=0.547.P<0.05);RyR-Ab诊断MGT的敏感性(77.2%)低于胸腺CT(88.2%),但特异性(91%)高于后者(72.2%),差异有统计学意义(P<0.05).Westemblot法显示:MGT组RyR-Ab阳性条带检出率最高(81.8%),明显高于非胸腺瘤MG(NTMG)组(4.5%)和NMG组(0),差异有统计学意义(P<0.05).结论 RyR-Ab阳性多见于MGT,检测RyR-Ab可以弥补影像学的不足,有助于诊断MGT和评估病情.     

重症肌无力免疫病原学研究Ⅰ重症肌无力患者血清免疫学研究

本文用ＥＬＩＳＡ法同步、动态地观测了２８５例ＭＧ患者血清ＡｃｈＲａｂ、ＰｓＭａｂ和ＣＡＥａｂ的变化。实验结果表明ＭＧ患者体内增高的ＡｃｈＲａｂ是ＭＧ发病的主要体液因素。部分血清ＡｃｈＲａｂ阴性ＭＧ患者（１７．５％）的发病可能与血清ＰｓＭａｂ异常有关。另一部分血清ＡｃｈＲａｂ和ＰｓＭａｂ均异常的ＭＧ患者（１２．６％）则可能同时存在骨骼肌神经肌肉接头突触前和突触后的损害。血清ＣＡＥａｂ测定在辅助诊断胸腺瘤方面具有高度的灵敏度和特异性，可作为临床早期诊断胸腺瘤的过筛检查。     

重症肌无力自身抗体的特异性和序列分析

通过对2株乙酰胆碱受体（AChR）自身抗体分子结构以及与致病性关系的分析，探讨了重症肌无力（MG）的发病机理。这2株抗体均为抗AChR上主要免疫原区（MIR）抗体，二者重链核苷酸同源性为99.7％，轻链同源性只有64.8％，说明重链在与AChRMIR结合上起重要作用。这2株抗体均未能在健康大鼠体内诱导出MG。通过与致病性AChR抗体可变区基因序列比较发现，这2株抗体的分子结构尤其是互补决定区（CDR）的氨基酸组成与致病性AChR抗体不同，表明抗AChRMIR抗体并不都具有致病性。     

雷诺丁受体抗体阳性的重症肌无力临床特点

雷诺丁受体（ryanodine receptor, RyR）是存在于内质网/肌质网中的一种重要钙离子通道,在骨骼肌兴奋收缩偶联机制中起重要作用。RyR抗体阳性的重症肌无力（myasthenia gravis, MG）患者常合并胸腺瘤,对常规治疗不敏感,会导致延误临床早期识别及治疗。血清RyR抗体水平与患者临床症状的严重程度显著相关。该文就4例RyR抗体阳性MG患者的临床特点及治疗过程进行讨论并文献复习,旨在提高对RyR抗体阳性MG的认识及诊疗水平。     

Effects of thymectomy on late-onset myasthenia gravis without thymoma

OBJECTIVES: This study aims to investigate whether thymectomy is beneficial for late-onset (>50 years) myasthenia gravis patients with no thymoma, particularly for those with mild generalized weakness. PATIENTS AND METHODS: A total of 34 patients were included in the study. The clinical course and long-term outcomes over 2 years were reviewed in 20 patients who underwent thymectomy and in 14 without thymectomy. RESULTS: Of the 34 patients, 20 (59%) underwent thymectomy. Thymectomized patients had more severe disability at entry than non-thymectomized patients, but outcome measures did not significantly differ between the two patient groups. Moreover, subgroup analyses including 22 patients with mild generalized weakness at entry showed that the thymectomized group (n=10) showed a greater percentage of clinical remission (no symptoms; 50% versus 17%; p=0.11) and a lower frequency of the presence of generalized symptoms (30% versus 75%; p<0.05) than the non-thymectomized group (n=12) at the end of follow-up (means 9.6 years after onset). CONCLUSIONS: Thymectomy is a potentially effective treatment for late onset, non-thymomatous patients with mild generalized myasthenia gravis.     

Myasthenia gravis,thymectomy, and antiacetylcholine receptor antibody

Summary The antiacetylcholine receptor antibody was titered in the serum of 63 patients with myasthenia gravis (MG) and 20 control healthy subjects. The titer was significantly high in 92% of MG patients in contrast with none of the controls and no correlation was found with the thymus pathology and the severity of the disease. The titer decreased after thymectomy almost steadily with the improvement of the myasthenic signs. The role of the antibody in the pathogenesis of the disease is discussed.

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Zusammenfassung Die Antikörper gegen Acetylcholin-Rezeptoren wurden quantitativ im Serum von 63 Patienten mit Myasthenia Gravis und von 20 gesunden Kontrollpersonen bestimmt. Der Titer war bei 92% der Myasthenie Gravis-Patienten signifikant erhöht, jedoch bei keinem der Kontrollfälle. Es wurde keine Korrelation zwischen dem Titer einerseits und den pathologischanatomischen Besonderheiten beziehungsweise dem klinischen Schweregrad der Erkrankung andererseits festgestellt. Der Titer nahm nach Thymektomie ziemlich geradlinig ab, parallel zur klinischen Besserung der Myasthenie. Es wird die Rolle der Antikörper bei der Pathogenese der Erkrankung diskutiert.

     

Myasthenia gravis appearing after thymectomy

We aimed to evaluate the clinical characteristics of patients with postoperative myasthenia gravis (MG). We retrospectively studied the data of 174 thymoma patients treated between 1990 and 2008 in Xiangya Hospital. Six of 125 patients without preoperative MG (4.8%) developed postoperative MG. The anti-acetylcholine-receptor binding antibody (ARAb) titers were elevated preoperatively in 22 of the 125 patients (17.6%) who did not have preoperative MG (range, 0.5-67.6 nmol/L). Four of six patients with postoperative MG had positive ARAb levels preoperatively. Serum titers were exacerbated in all six patients at the onset of postoperative MG. Postoperative MG was responsive to anti-cholinesterase compounds and/or steroids. We concluded that a thymectomy did not prevent postoperative MG. Exacerbated ARAb levels after thymectomy suggested an extrathymic production of ARAb. We suggest that a rise in the ARAb titer might be a risk indictor for post-thymectomy MG.     

重症肌无力患者血清IgG-乙酰胆硷受体抗体亚型研究

目的 探讨重症肌无力（ＭＧ）患者血清ＩｇＧ－乙酰胆硷受体抗体（ＡＣｈＲＡｂ）亚型的分布规律及其临床意义。方法 采用ＡＢＣ－ＥＬＩＳＡ法检测４３例ＭＧ组和２５例临床对照组、２０例正常对照组血清中ＩｇＧ－ＡＣｈＲＡｂ亚型ＩｇＧ１－４。结果 ＭＧ组与两对照组相比ＩｇＧ１和ＩｇＧ４亚型抗体无显著差别,ＩｇＧ２亚型抗体显著升高（Ｐ〈０．０５）,ＩｇＧ３亚型抗体显著降低（Ｐ〈０．０５）;ＭＧ组各临床类型间各亚型抗体无显著差别。结论 ＩｇＧ－ＡＣｈＲＡｂ亚型以ＩｇＧ２活性为主,但未显示与ＭＧ临床类型有关。     

重症肌无力患者血清白细胞介素—6水平测定

目的探讨重症肌无力(MG)与白细胞介素-6(IL-6)的关系.方法采用双抗体夹心ELISA法对30例MG患者用糖皮质激素(GC)治疗前、治疗2个月后和22例正常对照血清IL-6、乙酰胆碱受体抗体(AchRab)水平进行检测.结果MG患者组血清IL-6水平显著高于对照组(P＜0.01),MG患者组血清IL-6水平在用GC治疗2个月后显著降低(P＜0.01),其血清IL-6与血清AchRab水平呈正相关(r=0.693,P＜0.01).结论IL-6与MG发病密切相关,IL-6参加了MG的免疫病理过程;检测血清IL-6水平对MG临床有重要价值;GC可抑制IL-6合成及AchRab产生.     

胸腺切除术对重症肌无力患者电生理学指标和临床评分的影响

目的评价胸腺切除术对重症肌无力(MG)的近期疗效。方法对15例MG在胸腺切除术前(平均9天)及术后(平均11天)依次进行了临床绝对评分、右侧腋神经低频重复电刺激(RNS)和右侧伸指总肌单纤维肌电图(SFEMG)检查。结果手术后SFEMG和RNS异常率分别为100%和73%,手术后平均颤抖(jitter)值、异常电位对比例、阻滞电位对比例和临床绝对评分都较手术前有显著的降低;病理为胸腺瘤5例,手术前后平均jitter值、阻滞电位对比例和临床绝对评分均无显著差异,胸腺增生9例,手术后阻滞电位对比例和临床绝对评分较术前显著减低。结论MG患者胸腺切除术后在临床表现和电生理学指标上都有一定程度的改善,但此时大多数患者仍有临床症状和电生理学指标的异常,需要进一步的免疫调节治疗;MG并发胸腺瘤者可能手术后近期疗效差,并发胸腺增生者可能疗效相对较好。     

Acetylcholine-receptor-like protein from human thymoma associated with myasthenia gravis

Summary Cobrotoxin-binding protein was isolated by affinity chromatography from human thymoma which had been surgically removed from patients with myasthenia gravis. The protein was composed of polypeptides with a molecular mass of 40, 51, 65, and 74 kilodaltons as determined by polyacrylamide gel electrophoresis in the presence of sodium dodecyl-sulphate. Isoelectric focusing of the protein gave pI values of 5.2–5.6 and 11. This is the first report of the isolation of the protein from human thymoma. These findings suggest that the cobrotoxin-binding protein from human thymoma patients with myasthenia gravis has subunits similar to those of fish electric organs or mammalian muscles.     

Thymectomy in the treatment of myasthenia gravis: report of 247 patients

Summary We made a retrospective assessment of the long-term outcome in 247 consecutive patients with myasthenia gravis (MG) who underwent thymectomy in the period January 1971–December 1985. In 84 cases a thymoma was found at surgery, while 163 patients had a non-neoplastic thymus. The duration of symptoms before surgery, the age at onset of the disease and the presence of germinal centres in the thymus did not appear to influence the prognosis. Patients with a non-neoplastic thymus showed a better response to thymectomy. Thymoma was associated with more severe disease and with a higher mortality; moreover, more thymoma patients required corticosteroid treatment in order to achieve good therapeutic results. In our opinion, thymectomy is indicated in the treatment of generalized MG, while ocular myasthenia seems not to be improved by the removal of the thymus.     

重症肌无力行胸腺切除术后合并视神经脊髓炎1例

重症肌无力（myasthenia gravis,MG）和视神经脊髓炎（neuromyelitis optica,NMO）均为较少见的神经系统自身免疫性疾病,且两病极少在同一个体先后出现。首都医科大学宣武医院神经内科收治1例重症肌无力患者行胸腺切除术后4个月合并视神经脊髓炎患者,现报道如下。     

伴胸腺瘤重症肌无力的临床特点(附96例分析)

目的　研究伴胸腺瘤重症肌无力的临床特点。方法　对经手术病检证实的 96例MG伴胸腺瘤患者的临床资料进行回顾性分析 ,并采用 χ2 检验及t检验与 114 9例经影像学检查无胸腺瘤表现的MG患者进行比较。结果　胸腺瘤组男性发病者多 (男∶女 =1 82∶1) ,且多于 30岁后发病 (71 9% ) ,以肢体无力和延髓症状首发者多见 (4 3 8% ) ,按改良Os serman分型 ,以Ⅲ型和Ⅳ型为主 (4 5 8% ) ,危象发生率高 (38 5 % ) ,病死率高 (8 3 % )。结论　伴胸腺瘤的重症肌无力有其独特临床特点 ,充分认识这些特点将有利于指导对这类患者的临床诊断和治疗