重症肌无力的治疗现状

Myasthenia gravis is a most common neurological disease with autoimmune disorder. Most of the research on myasthenia gravis has focused on treatment . we will assess the effect of the treatments of myasthenia gravis, which including corticosteroids, immunosuppressive , IV immunoglobulin , plasmapheresis and thymectomy. The treatment for patients with myasthenia gravis usually depends on age, clinical characteristics and thymoma or nonthymoma-related MG and so on.     

女性重症肌无力的临床特点

目的：对收治的118例女性重症肌无力病人的临床特点进行研究。方法：对118例胸腺切除术后的女性病人进行0．5－30年观察随访。结果：女性重症肌无力发病率高，重症肌无类型广泛，胸腺增生发生率高，而胸腺瘤发生率偏低，但恶性胸腺瘤发生率高。少数病人合并甲状腺功能亢进，围手术期死亡率较低，预后较好。结论：女性重症肌无力外科治疗效果较好。     

重症肌无力患者护理

重症肌无力是一种横纹肌神经肌肉接头点处传导障碍的自身免疫性疾病,以肌肉易疲劳为特点,常累及眼外肌、咀嚼肌、吞咽肌和呼吸肌。密切观察病情及加强护理是保证治疗成功的关键,我科2005年2月至2009年2月共收治重症肌无力患者7例,取得了满意效果,现将护理总结如下。     

青霉胺引起重症肌无力

患者女,52岁。因“双睑下垂,吞咽困难,呼吸困难16d”来诊,16d前因“类风湿性关节炎”症状加剧,当地医院予服青霉胺片治疗(剂量、时间不详),数天后出现双睑     

重症肌无力的护理体会

目的探讨重症肌无力护理要点,重症肌无力的预后,提高患者的生活质量。方法对23例重症肌无力患者的临床资料进行回顾性分析、总结与归纳。结果 23例患者均病情平稳出院。结论科学合理的护理措施,不但有助于其主动参与积极配合治疗,还能促进其心理康复,对提高患者生活质量有重要意义,特别是对肌无力危象患者的转归和预后有着重要的意义。     

重症肌无力的护理体会

目的通过对重症肌无力及其危象的观察,总结护理经验,协助医生采集临床资料。达到更好的治愈患者的目的。方法对本院的25例重症肌无力和5例重症肌无力危象患者的临床资料进行回顾分析。结果本组25例重症肌无力患者均好转出院。5例肌无力危象患者1例死亡。1例无效,余痊愈。结论科学合理的护理措施对重症肌无力患者的预后及疾病的转归有着积极的重要意义。     

重症肌无力的护理体会

总结对重症肌无力患者的护理体会,强调周到、有计划的护理对推动患者积极配合治疗及提高患者生活质量有重要意义。     

重症肌无力的诊治要点

<正>重症肌无力是主要累及神经肌肉接头处突触后膜上烟碱样乙酰胆碱受体,主要由乙酰胆碱受体抗体介导、细胞免疫依赖性、补体参与的获得性自身免疫性疾病。重症肌无力是当今抗原、抗体最为明确,免疫学发展机制相对最为清楚的自身免疫性疾病之一,这有可能成为攻克自身免疫性疾病的突破口。重症肌无力已经成为近年神经病学和免疫学的重点研究课题之一。因为重症肌无力的诊断是以临床为基     

重症肌无力的治疗进展

重症肌无力(MG)是随意肌的突触后膜乙酰胆碱受体(AchR)的自身免疫性疾病。即由于血中抗AchR抗体对受体的占领、封闭使它不能与乙酰胆碱(Ach)有效地结合;抗体与受体的复合物在补体C3的参与下可溶解受体;杀伤T细胞及淋巴因子并破坏受体;辅助T细胞可促进B细胞合成抗AchR抗体,这种抗体的大部分在胸腺内合成,T淋巴细胞在胸腺内致敏,可引起MG的自身免疫反应的始动抗原(即肌样细胞)也存在于胸腺。现今治疗方式主要是针对抗体、淋巴细胞和胸腺组织的免疫干预治疗以及对症治疗。     

纳武利尤单抗注射液致重症肌无力

1例53岁女性患者于肝内胆管癌术后20个月给予纳武利尤单抗注射液140 mg静脉滴注、1次/d,第1、20天。第2次应用该药后第3天,患者出现双下肢乏力;第14天乏力加重,出现视物模糊、上眼睑下垂、胸闷气促伴心悸;第24天出现胡言乱语,发展为呼之不应。给予气管插管机械通气及对症支持治疗后患者神志恢复。诊断为药源性重症肌无力危象,合并心力衰竭、呼吸衰竭。给予激素、人免疫球蛋白治疗19 d后,患者眼睑下垂、心悸症状明显好转,但呼吸衰竭无明显好转,无法脱机自主呼吸。     

Anesthetic implications of myasthenia gravis

Myasthenia gravis is a disease of great significance to the anesthesiologist, because it affects the neuromuscular junction. Many patients with this condition are treated by surgical thymectomy, using techniques developed by Mount Sinai physicians, including Dr. Paul Kirschner, Dr. Alan E. Kark, and the late Dr. Angelos E. Papatestas. The authors review the anesthetic considerations in the management of patients with myasthenia gravis who are undergoing thymectomy and other surgical procedures.     

Treatment strategies for myasthenia gravis

Advances in the treatment of myasthenia gravis (MG) have reduced mortality rates due to the disease and improved patients' quality of life. Nowadays, attending neurologists can choose among different treatment strategies for MG patients. An exhaustive revision of published data on the efficacy of the different therapeutic options for MG indicates that there are insufficient evidence-based results. However, recommendations based on expert opinion can be provided. Thymectomy is indicated in all patients with a thymoma or for generalized acetylcholine receptor-seropositive patients aged 18 – 55 years. Steroids are the most widely used immunosuppressive drug for MG. They are recommended as the first-line drug in all patients with generalized MG without response to thymectomy, or in those patients who do not fulfill criteria for the surgery. The selection of second-line drugs may vary between protocols. We recommend to start with azathioprine if insufficient remission is achieved with steroids, followed by ciclosporin, mycophenolate and others. We use rituximab or cyclophosphamide only in severely drug-resistant patients. Finally, we recommend intravenous immunoglobulins or plasma exchange in MG crisis, or for unstable patients before thymectomy or in clinical exacerbations.     

AChR-specific immunosuppressive therapy of myasthenia gravis

Myasthenia gravis (MG) is an organ-specific autoimmune disease characterized by muscle fatigability. In most cases, it is mediated by autoantibodies targeting muscle nicotinic acetylcholine receptors (AChRs) at the neuromuscular junction. Experimental autoimmune myasthenia gravis (EAMG) is an animal model for MG, which is usually induced by immunization with AChR purified from fish electric organ. Pathological autoantibodies to AChRs are directed at the extracellular surface, especially the main immunogenic region (MIR). Current treatments for MG can help many but not all patients. Antigen-specific immunosuppressive therapy for MG that specifically suppresses the autoimmune response without affecting the entire immune system and avoids side effects of general immunosuppression is currently unavailable. Early attempts at antigen-specific immunosuppression for EAMG using AChR extracellular domain sequences that form epitopes for pathological autoantibodies risked provoking autoimmunity rather than suppressing it. We discovered a novel approach to specific immunosuppression of EAMG with a therapeutic vaccine consisting of bacterially-expressed human AChR cytoplasmic domains, which has the potential to specifically suppress MG without danger of causing exacerbation. This approach prevents development of chronic EAMG when initiated immediately after the acute phase of EAMG, and rapidly reverses established chronic EAMG when started during the chronic phase of EAMG. Successfully treated rats exhibited long-term resistance to re-induction of EAMG. In this review we also discuss the current understanding of the mechanisms by which the therapy works. Vaccination with AChR cytoplasmic domains in adjuvant is promising as a safe, antigen-specific, potent, effective, rapidly acting, and long lasting approach to therapy of MG.