High-dose intravenous immunoglobulin in myasthenia gravis

The effects of high-dose intravenous immunoglobulin (i.v.Ig) in 12 MG patients were studied. All patients had severe symptoms. In two cases anti-acetylcholine receptor antibodies (anti-AChR abs) were not detectable. I.V.Ig was administered to 9 patients already on long-term immunosuppressive therapy and to 3 patients at the beginning of azathioprine treatment. 10 patients (83%) improved; the duration of improvement was longer in immunosuppressed patients. Anti-AChR abs generally decreased after infusion but we did not find a constant correlation between reduction in ab titers and clinical improvement. Side effects included one case of severe hemolysis. In our experience i.v.Ig therapy is effective in MG. The chief indication for its use appears to be the treatment of deteriotation of the disease in patients already on immunosuppressive therapy. Paper presented at the National Congress at Sorrento in 1991 and selected by the Editorial Board of the Journal     

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.     

大剂量丙种球蛋白、甲基强的松龙治疗重症肌无力

目的探讨依次采用大剂量丙种球蛋白静滴、大剂量的甲基强的松龙治疗重症肌无力的临床疗效。方法时符合Osserman分型的61例患者,随机分为两组：治疗组（A组）31例,依次采用大剂量丙种球蛋白厦大剂量的甲基强的松龙治疗;对照组（B组）30例,采用传统大剂量地塞米松静滴治疗;观察两组的临床疗效。结果治疗组症状缓解快,激素治疗过程中发生呼吸肌瘫痪、使用呼吸机现象少,住院时间短,疗效好（均P〈0．05）。结论依次采用大剂量丙种球蛋白、大剂量的甲基强的松龙冲击治疗重症肌无力的临床疗效好,值得临床推广。     

High-dose intravenous immunoglobulin therapy in juvenile myasthenia gravis

Autoimmune neurologic disease management has been significantly modified by the use of high-dose intravenous immunoglobulin (HDIVIG) during the past 15 years. Venous access, readily available IgG (until recently), and the relative lack of serious identifiable complications have prompted its use in myasthenia gravis. In adults, its effectiveness has been inconsistent, with variable acetylcholine receptor (AChR) antibody responses. Ten children were evaluated for clinical responses to, and complications of, HDIVIG. Weekly anti-AChR antibody titers in three patients were obtained. The HDIVIG dosage was 2 gm/kg body weight, infused at variable rates of 2 gm/kg for 1 day, 0.66 gm/kg daily for 3 days, and 0.5 g/kg daily for 4 days; in one patient the total dose was 0.8 gm/kg to correct to the ideal body weight. All children but one tolerated HDIVIG without complications. Eight patients exhibited definite improvement in functional strength after HDIVIG, but a decreasing response to HDIVIG was evident after multiple monthly treatments, warranting the additional use of corticosteroids in two patients. A decrease in anti-AChR antibody levels was observed in the three patients tested, but this decrease was constant in one patient. No correlation was observed between clinical response and antibody titers. HDIVIG is safe and effective in most patients for short-term management of juvenile myasthenia gravis, in myasthenic crises, and in preparing patients for surgery but appears to be of limited long-term benefit.     

218例重症肌无力危象的临床分析

目的总结重症肌无力危象的临床特点及急救和预防经验.方法回顾性分析1956～2004年诊治的218例患者369例次重症肌无力危象的病例资料.结果死亡51例,病死率23.39%;近十年(1994～2004年)死亡4人,病死率4.2%.结论综合利用气管切开正压辅助呼吸、激素冲击治疗、丙种球蛋白静滴及血浆交换可改善重症肌无力危象的预后,且明显降低病死率.     

静脉注射人免疫球蛋白治疗全身型重症肌无力的短期疗效观察

目的观察静脉注射人免疫球蛋白(intravenous immunoglobulin, IVIg)治疗全身型重症肌无力(myasthenia gravis, MG)的短期临床疗效、安全性。方法纳入2016年1月至2019年9月期间北京医院就诊的58例Osserman分型Ⅱb及以上的全身型MG患者。收集患者一般信息、入院诊断、治疗用药情况,采用许氏绝对与相对评分法(the absolute and relative score of MG,ARS-MG)评估患者IVIg治疗前及治疗后7、14 d临床症状的改善情况,并比较不同年龄(<60岁和≥60岁)、病程(≤1年和>1年)以及有无构音障碍、咀嚼障碍、呼吸困难者间IVIg疗效的差异。结果 58例MG患者IVIg治疗后第7天许氏评分较治疗前降低[(17.21±10.52)分比(23.22±10.45)分,P<0.01]。IVIg治疗7 d时其总有效率为48.28%(n=58),治疗后14 d其总有效率为59.46%(n=37)。不同年龄、病程以及有无构音障碍、咀嚼障碍、呼吸困难者间IVIg疗效比较无统计学差异(均P>0...     

眼肌型重症肌无力进展为全身型重症肌无力的临床相关因素分析

目的 :探讨眼肌型重症肌无力进展为全身型重症肌无力的临床相关预测因素。方法 :33例初诊为眼肌型重症肌无力的患者经过3年随访,根据疾病进展结局分为眼肌型重症肌无力组(13例)和进展为全身型重症肌无力组(20例)。对与疾病进展可能相关的临床因素进行分析。结果 :进展为全身型重症肌无力组患者初诊时的定量重症肌无力评分、乙酰胆碱受体抗体阳性率、抗核抗体阳性率、合并胸腺瘤的比例以及合并糖尿病的比例均高于眼肌型重症肌无力组(P值均0.05)。结论 :定量重症肌无力评分高、乙酰胆碱受体抗体阳性、抗核抗体阳性以及合并胸腺瘤和糖尿病可能是眼肌型重症肌无力进展为全身型重症肌无力的预测指标。     

非手术免疫抑制疗法治疗伴胸腺瘤的重症肌无力患者的远期疗效观察

目的评价非手术的免疫抑制疗法对伴有胸腺瘤的重症肌无力（MG）的远期疗效。方法采用类固醇、化学疗法、放射疗法治疗84例MG伴胸腺瘤患者,作回顾性研究,经诊断治疗后1－21年（平均4．8年）长期随访,评价其远期有效率、存活率和存活质量。结果远期有效率为86．9％（73／84）,即31例（36．9％）获完全缓解,37例（44．0％）获药物缓解,5例（6．0％）获明显改善。5年存活率76．5％,10年存活率54．5％。完全缓解期0．5－20．0年（平均4．6年）。总病死率为13．1％（11／84）。结论非手术免疫抑制疗法治疗MG伴胸腺瘤患者远期疗效良好,从远期存活率和存活质量来看并不亚于手术疗法。死因大多仍为危象,不是瘤转移。     

成人重症肌无力与胸腺病变

本文研究分析了７７０例成人重症肌无力患者中有关胸腺的检测结果。１５６例共同接受血清柠檬酸提取物抗体（ＣＡＥ—Ａｂ）检测和纵隔ＣＴ检查，结果显示两者的相符率为８７．８％（１３７／１５６）；６２例术前ＣＴ和术后病理诊断的相符率为８５．８％（５３／６２）；８２例血清ＣＡＥ—Ａｂ与胸腺病理的相符率为８７．８％（７２／８２；７７例胸腺病理与ＭＧ临床Ｏｓｓｅｒｍａｎ分型研究说明ＭＧ伴发胸腺瘤患者中以ⅡＢ、Ⅲ、Ⅳ型为多，病情相对较重。文章对各种胸腺检查方法的优缺点进行了分析比较，强调综合考虑对胸腺摘除术前、术后准确评价胸腺改变的重要性。     

High-dose intravenous immunoglobulin G treatment of myasthenia gravis

Neurological Sciences - IVIg is a safe and effective adjunctive treatment for myasthenia gravis, but there are no well established guidelines for the use of IVIg in this disease, lacking controlled...     

High-dose intravenous immunoglobulin G treatment of myasthenia gravis

IVIg is a safe and effective adjunctive treatment for myasthenia gravis, but there are no well established guidelines for the use of IVIg in this disease, lacking controlled randomized trials to assess its efficacy in homogeneous group of patients. The main advantages of IVIg are the rapid onset of the effect, the lack of long-term toxicity, and the possibility to reduce the required doses of immunosuppressive drugs. IVIg appears to have a role as an acute treatment in rapidly progressive myasthenia gravis weakness, particularly in situations when therapeutic apheresis is not feasible. In addition, IVIg is safer than plasma exchange (PE) in patients with hypotension or autonomic instability, in children, in patients of older age (>65 years), and in those suffering from sepsis. For these reasons, at present, IVIg are recommended during crises of myasthenia gravis in older patients when PE is contraindicated or not feasible IVIg can be also used as a chronic maintenance therapy when other immunosuppressive treatments have failed or cannot be used. Periodic administration of IVIg on a bimonthly or monthly basis may be able to stabilize chronic, nonresponding patients.     

Treatment of myasthenia gravis with high-dose intravenous immunoglobulin

We treated 37 patients affected by autoimmune generalized myasthenia gravis (MG) with high-dose intravenous gammaglobulin (HDIVIg), 400 mg/kg per day on 5 consecutive days. A one-degree improvement of Oosterhuis global clinical classification of myasthenic severity (OGCCMS), the disappearance of bulbar involvement or both were recorded 12 days after the beginning of the treatment in 70.3% of the patients and persisted up to 60 days in 58.7%. A two-degree improvement of OGCCMS was recorded in 54.1% of the patients and it was maintained up to 60 days in 37.8%. The percentage of improvement did not significantly differ between patients entering the treatment in a long-standing, drug-refractory stationary phase of the illness (n = 26) and patients who received HDIVIg in an acute phase of MG (n = 11). None of the patients experienced side effects. Our data indicates that HDIVIg is an interesting, virtually riskless therapeutic choice for MG patients, and allows the planning of a controlled trial versus plasma-exchange.     

眼肌型重症肌无力的临床治疗

目的 比较激素和溴吡斯的明在眼肌型重症肌无力(MG)患者中的不同疗效,寻找最佳治疗方法.方法 对2001年12月至2009年1月就诊的初次发病的43例眼肌型MG患者资料进行回顾性研究,根据治疗方式分为溴吡斯的明治疗(A)组6例,溴吡斯的明无效后改用泼尼松治疗(B)组16例,泼尼松治疗(C)组21例,分别选择MG临床绝对评分法的眼肌功能评分和相对评分法对每种治疗方法的疗效进行量化比较.结果 泼尼松治疗期(B+C)前后绝对评分差为7.86±4.28,溴吡斯的明治疗期(A+B)为2.64±2.52(t=-5.2,P<0.01);两者的相对评分(分别为0.76±0.32和0.31±0.30,Z=-4.72,P<0.01)及复发病例数(分别为12例和13例,χ~2=4.02,P=0.045)差异有统计学意义.B组和C组治疗前后疗效无明显差异.结论 激素治疗眼肌型MG的效果优于溴吡斯的明,半年内激素治疗的早晚对治疗效果可能无明显影响.     

老年重症肌无力患者免疫抑制疗法的远期疗效分析

目的评价免疫抑制治疗老年重症肌无力(myastheniagravis,MG)患者的远期疗效。方法回顾性评价随访时间≥12个月的99例老年MG患者(发病年龄≥60岁)的临床疗效,其中完全缓解、药物缓解和显著改善均属于远期疗效良好。结果免疫抑制治疗对老年MG的总有效率为79·8%(79/99)。糖皮质激素(glucocorticoids,GC)对老年MG的总有效率为72·9%(35/48),其中完全缓解者8例,缓解期18~67个月,平均(54·8±19·8)个月;药物缓解者16例,缓解期7~37个月,平均(15·7±8·0)个月。GC对男性MG患者的有效率为83·3%(25/30),对女性MG患者的有效率仅为55·6%(10/18),两者相比,差异具有统计学意义(P<0·05)。GC联合环磷酰胺(CTX)治疗对老年MG的总有效率达86·3%(44/51),其中完全缓解者11例,缓解期17~90个月,平均(50·3±25·4)个月;药物缓解者19例,缓解期7~56个月,平均(25·5±15·9)个月。联合治疗对老年男性MG患者有效率为88·5%(23/26),对女性MG患者的有效率为84·0%(21/25),两者相比差异无统计学意义。然而,联合治疗与激素治疗相比,不仅能够显著延长老年MG患者药物缓解期的持续时间(P<0·05),而且能够明显提高老年女性MG的远期有效率(P<0·05)。结论免疫抑制剂的应用是治疗老年MG的重要措施。GC对老年MG的治疗效果存在性别差异,对男性的效果优于女性。GC联合CTX治疗能够延长老年MG患者的药物缓解时间,能够显著提高老年女性MG患者的远期有效率。     

胸腺放射治疗重症肌无力的疗效观察

目的 观察胸腺放射治疗重症肌无力（MG）的疗效。方法 回顾性分析我院自1957年到1999年42年间应用胸腺放射治疗的68例MG患者（深部X线放疗22例，^60Co治疗46例）的临床资料，并对他们作了长期随访。结果 放射治疗后总缓解率达90%，临床疗效显著，明显改善了患者生活质量，副反应以皮肤损害（10例）和消化道症状（9例）多见。结论 对于药物反应差，手术时扫现胸腺瘤对周围组织有明显浸润或术后症状不稳定MG患者加用胸腺放射治疗，可明显提高疗效。     

葡萄球菌蛋白A免疫吸附治疗全身型重症肌无力的临床研究

目的研究葡萄球菌蛋白A免疫吸附治疗全身型重症肌无力(myasthenia gravis,MG)的疗效和安全性。方法采用蛋白A免疫吸附治疗19例成人全身型MG并进行临床疗效和安全性的判定。结果所有入选病人均完成2次免疫吸附治疗;第2次治疗后的IgG、IgA、IgM浓度明显下降(P<0.0001);Osserman分级、改良美国MG基金会(MGFA)评分、MG日常生活量表(ADL)和徒手肌力量表(MMT)均明显改善(P<0.0001)。MG特异性乙酰胆碱受体(AChR)抗体阳性的患者2次治疗后抗体滴度明显下降;治疗后外周血调节性T细胞百分比明显上升。部分病人有轻度不良反应,但总体耐受性良好,各项安全性临床评定指标在治疗前后的变化均无统计学意义。结论葡萄球菌蛋白A免疫吸附可以迅速改善全身型MG病人的病情。     

Determination of erythrocyte-bound acetylcholinesterase activity for monitoring pyridostigmine therapy in myasthenia gravis

Summary Monitoring of pyridostigmine therapy in patients with myasthenia gravis is not routinely performed, since the daily pyridostigmine doses are adjusted to the patients's actual clinical status rather than to pyridostigmine plasma concentrations (PPC). Moreover, PPC determination is time-consuming and needs much technical equipment. Since pyridostigmine reversible blocks acetylcholinesterase (AChE) at the neuromuscular junction, we studied the correlation between the enzyme's blood activity (erythrocyte-bound AChE) and PPC, on the one hand, and between blood AChE activity and the clinical status of the individual patient, on the other. In five previously untreated patients with myasthenia gravis blood AChE activity decreased in accordance with the actual PPC after a single oral dose of 60 mg pyridostigmine (group A). Amelioration of the clinical status corresponded to the decrease of AChE activity in the same way. In another five patients, who were on stable pyridostigmine medication for at least 1 week, AChE activity and PPC were constant during the day (group B). Since it is easier to perform than PPC, our results suggest that the determination of AChE activity may be superior to measuring PPC for monitoring cholinesterase inhibitor therapy in selected cases.     

Efficacy and tolerability of subcutaneously administered immunoglobulin in myasthenia gravis: A systematic review

Subcutaneous immunoglobulin (SCIg) is an emerging therapeutic alternative in the management of myasthenia gravis (MG) due to its potential efficacy, safety, cost effectiveness and ease of administration. At present, there are no systematic reviews that summarized the effects of SCIg in patients with MG. The objective of this study is to determine the efficacy and safety of SCIg in the treatment of adult patients with myasthenia gravis. Relevant records were identified from August 2018 to January 2019 systematic search. Five relevant articles with a total of 34 patients with MG were included in this review. Data on functional disability score and adverse events were obtained. Based on the included uncontrolled studies, the functional disability scores of adult MG patients after SCIg administration showed consistent improvement. Headache and local site injection reactions were the most common adverse events reported. The evidence from limited uncontrolled studies gathered in this review showed that SCIg may improve functional disability in patients with MG. Local and mild adverse events were reported with its administration, but no systemic and serious adverse events were noted.