重症肌无力患者血清补体成分的测定

应用免疫扩散法测定55例重症肌无力（MG）患者和41例健康对照血清中补体（C1q、C3、C4、C9、C1－INH因子、B因子）的浓度。结果发现，MG患者G1－INH因子浓度明显高于健康对照组（P＜0．001）。而C1q和C9明显低于对照组（P＜0．001）；并且，随着受螺肌群的增加，C1q和C9有降低的趋势，而C1－INH因子有升高趋势；其它补体成分的浓度与对照组相比无显著性差异（P＞0．05）。16例MG初诊患者用药后，C9浓度显著升高（P＜0．001），C1q增高趋势有C1－INH因子下降趋势均明显，但三者均未恢复至正常水平（P＜0．01）。提示对MG患者测定血清补体，有助于进行动态观察，并可作为判断病情严重程度（活动性）的客观指标。     

青霉胺致肾病综合征重症肌无力二例并文献复习

目的提高临床医生对青霉胺(D-PA)致肾病综合征和重症肌无力的认识。方法报道2例患者经D-PA治疗后出现肾病综合征、重症肌无力,并复习近年国内外文献,对有关此二种不良反应的发生机制、病理、治疗及鉴别诊断进行总结。结果例1为系统性硬化症,服用D-PA后出现肾病综合征、双侧眼睑下垂症状,考虑为D-PA不良反应．停药观察,随访至今好转。例2为类风湿关节炎患者,经用D-PA后出现吞咽困难、饮水呛咳．经反复就诊疑为D-PA不良反应,停药后好转。结论D-PA是目前临床上治疗自身免疫性疾病的常用药物,但应对此罕见的副作用加以警惕。     

Sarcoidosis in a patient with myasthenia gravis. Case report and review of the literature

Summary The case of a 38-year-old white female, with a long history of myasthenia gravis, who developed sarcoidosis is presented. The diagnosis of myasthenia gravis had been established on the basis of typical clinical symptoms with severe myastenic crises necessitating respiratory support, characteristic electromyographic findings, positive anti-acetylcholine receptor antibodies and response to appropriate medications. After 9 years of disease and while in apparent remission, she presented with ankle arthritis and bilateral hilar adenopathy. A transbronchial lung biopsy showed noncaseating granuloma, typical of sarcoidosis. To our knowledge, the combination of myasthenia gravis and sarcoidosis is extremely rare and emphasizes the intriguing tendency of some immunologic disorders to appear together in certain individuals.     

D-penicillamine induced myasthenia gravis in rheumatoid arthritis: An unpredictable common occurrence?

Summary Five patients out of 71 with rheumatoid arthritis (RA), who received D-penicillamine, developed myasthenia gravis (MG) within a two-year period. They all responded promptly to discontinuation of the drug and pyridostigmine administration. None of the patients had anti-Ro(SSA) antibodies or features of Sjögren's syndrome, whereas three of the five had the HLA-DR₁ phenotype. The relatively high frequency of MG observed in our population, along with its unpredictability and potentially serious sequelae, necessitates its inclusion in the list of side effects of D-penicillamine routinely discussed with the patient, prior to initiation of the treatment. Full alertness of both the patient and the physician to even minor initial myasthenic symptoms, that dictate immediate discontinuation of the drug, is of obvious importance.     

232例老年人重症肌无力的临床分析

目的:分析老年人重症肌无力(MG)的有关临床特点。方法:回顾性分析26年间诊治的232例老年MG患者的临床资料。结果:老年人MG占总MG病例数(3010例)的比例为7．7％。男性多于女性，男：女=1．3：1。老年人MG多在60～70岁间发病，在其常见首发症状中，眼症状184例(79．3％)、咽喉肌无力30例(12．9％)、肢体肌肉无力17例(7．3％)。老年人MG中，全身型的构成比多于眼型(62．9％对37．1％)，较少并存自身免疫性疾病和发生危象，常合并胸腺瘤(33例)及内外科其他疾病(87例)。结论:老年人MG具有独特的临床特点，了解这些特点将有利于指导临床诊断和治疗。     

甲亢合并重症肌无力患者的临诊应对

本文报道1例甲亢多年后合并重症肌无力的29岁男性患者,经新斯的明试验确诊并治疗后,甲亢与重症肌无力均得到有效控制.随访2年余未再复发.     

重症肌无力伴骨骼肌以外受损的临床特征分析

目的 对伴心脏、肝脏损害,瞳孔不等,听力障碍,手足血管舒缩功能及皮肤营养障碍和阳痿等少见症状的重症肌无力(MG)患者的特点进行分析,提出诊断和鉴别诊断的方法。方法 (1)给予MG患者胆碱酯酶抑制剂(ChEI)及免疫治疗,再复查病情和随访;(2)伴瞳孔不等和听力障碍患者肌注新斯的明1mg,30～120min后观察症状变化,后者同时检查脑干听觉诱发电位2次。结果 55例MG患者临床症状随典型的骨骼肌病态疲劳的症状改善而好转,一般不需要特殊治疗。结论 ChEI治疗MG患者合并上述少见症状均有效,是与其他疾病鉴别的重要依据,也证实了MG是一种广泛的自身免疫性疾病。     

Severe asthma associated with myasthenia gravis

Severe asthma constitutes a subgroup of approximately 10% of all asthma cases. Approximately one-half of these individuals have a refractory form of the disease in which atopy and T-helper cell 2-skewed immunological response may not be as closely linked to the disease as in other phenotypes of asthma. This suggests that not all asthma is explained by a T-helper cell 2-skewed immunological response, and that other immunological mechanisms may be important in this category of nonatopic asthma. The authors present a case involving a 55-year-old Caucasian man with nonatopic, adult-onset asthma, nonsteroidal anti-inflammatory drug sensitivity and idiopathic urticaria. This individual presented two years following his initial asthma diagnosis with diplopia and mild ptosis, and was subsequently diagnosed with seropositive myasthenia gravis.     

Robotic thymectomy for myasthenia gravis surgical techniques and outcomes

Myasthenia gravis (MG) is an autoimmune disorder in which antibodies are produced against post-synaptic acetylcholine receptors, thereby causing impairment of neuromuscular transmission. Diagnosis of MG is confirmed with the AChR antibody test and via an Electromyography. Although medical treatment with acetylcholinesterase inhibitors remains the main treatment of MG, in recent years thymectomy has become an integral part of the treatment algorithm. Numerous factors such as the Patient’s age, presence of AChR antibodies, or MuSK antibody, the severity of disease affect the decision of preforming the thymectomy. Historically thymectomy was preformed via sternotomy associated with significant morbidity. Advancement in the minimally invasive approaches to thymic resection has led to more acceptance of thymectomy in the management of MG. Among these approaches, robotic thymectomy is gaining popularity across the globe due to the unique advantages of the robotic platform like 3D visibility, enhanced dexterity, and wrist like articulating movements of instruments. This has led to less post-operative pain and morbidity; faster recovery and shorter hospital stay. Successful treatment of MG requires a multi-modality approach, which has led to the formation of MG teams in most academic centers, comprising of a specialist neurologist, intensivist, and thoracic surgeon. In this article, we describe the techniques and outcomes of the robotic thymectomy for MG.     

免疫治疗对重症肌无力病人淋巴细胞亚型的影响

目的 研究重症肌无力（ＭＧ）患者细胞免疫的异常改变和糖皮质激素及胸腺切除对各淋巴细胞亚群的影响。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淋巴细胞表型分析,并对１５例ＭＧ患者经患者经糖皮质激素治疗前后和７例ＭＧ患者在胸腺切除前后淋巴细胞亚群的百分率的变化进行观察。结果（１）ＭＧ患者总Ｔ细胞,总Ｂ细胞（ＣＤ１９＾＋）,传统Ｂ细胞（ＣＤ５＾－ＣＤ１９＾＋）,ＣＤ     

早发型与晚发型重症肌无力患者的临床特点分析

目的 分析早发型与晚发型重症肌无力(MG)的临床特点.方法 回顾分析同期住院的819例MG患者资料,比较早发型及晚发型MG组患者的构成情况、首发症状、临床分型、新斯的明试验及重复电刺激阳性率、合并甲状腺功能异常及胸腺病理类型等临床特点.结果 MG发病仍以早发型人群为主,占77.66%,两组男女比例分别为1∶1.08及1∶0.81(P＞0.05),皆以ⅡB型为主(41.51%比51.37%,P＜0.05),MG患者新斯的明试验和重复电刺激阳性率差异无统计学意义.甲状腺功能异常在早发型MG组较晚发型MG组多见(P＜0.05),早发型MG组患者胸腺异常以增生为多见(达67.57%,P＜0.01),而晚发型MG组患者胸腺异常则胸腺瘤和胸腺增生比例相当(分别为48.68%和47.37%).结论 早发型与晚发型MG患者在临床分型、合并甲状腺功能异常及胸腺病理方面具有不同的特点,值得注意.

Abstract：

objective To compare the clinical characteristics of early-onset and late-onset myasthenia gravis(MG).Methods A total of 819 MG patients admitted in our department during the sanle period were reviewed retrospectively.The patients with MG were divided into two groups by the age of onset, which were the early-onset MG(＜49 years)and late-onset MG(≥50 years).Several clinical features were compared in the two groups including the percentage, initial symptoms, MG types,the positive rates of neostigmine test and repetitive nerve stimulation, thyroid function and thymie abnormalities. Results The more common onset age of MG was 0-49 years(early-onset).In both groups the male and female ratio was presented as the most common type in both groups(41.51%and 51.37% respectively in early-onset and late-onset groups).Type Ⅰ was more common in early-onset group while typeⅡB Was more common in lateonset group(P＜0.05).There Was no significant difference in the positive rates of neostigmine test and repetitive nerve stimulation between the two groups.The thyroid function alnormality ratio was higher in early-onset group. Hyperplastic thymus was common in early-onset group(67.57%)while both hyperplastic thymus and thymoma were common in late-onset group(48.68% and 47.37%).Conclusions The clinical characteristics of early-onset and late-onset myasthenia gravis(MG)Was different in MG type, the positive rate of thyroid function abnormalities and thymic pathologic type.     

D-Penicillamine induced toxicity in rheumatoid arthritis: the role of sulphoxidation status and HLA-DR3

Sulphoxidation of carbocysteine, a drug structurally similar to D-penicillamine, displays a skewed distribution within a population. In 66 patients with rheumatoid arthritis (RA) a significant association between impaired sulphoxidation and toxicity (p less than 0.001) was found; HLA-DR3, although associated with toxicity (p less than 0.05), appeared to be an independent risk factor of most importance in the group with extensive sulphoxidation. The relative risk of toxicity in a patient possessing either DR3 or impaired sulphoxidation was 25. The prevalence of poor sulphoxidizers within this group of RA patients was increased compared to that in a previous population study and requires further investigation. Our findings explain a number of the toxic phenomena associated with D-penicillamine administration in RA.     

大鼠重症肌无力中枢受损时白细胞介素-1的变化

目的 探讨重症肌无力（ＭＧ）患者中枢神经系统损害与白细胞介素（ＩＬ）－１之间的关系。方法 将ＭＧ患者中提取的ＩｇＧ注入大鼠脑室系统,建立大鼠中枢神经系统受损模型,然后观察模型大鼠脑、胸腺,血清ＩＬ－１水平的变化。结果 脑室内注入ＩｇＧ后第１周起,脑,胸腺及血清中ＩＬ－１水平升高,其中脑组织上升最为明显,第２周未达高峰,而在胸腺及血中上升则相对缓慢,第３周未时仍在缓慢上升。结论 大鼠中枢神经系统损受     

重症肌无力伴发甲状腺异常的临床分析

目的 分析重症肌无力(MG)合并甲状腺异常患者的临床特点.方法 回顾性分析2008年7月-2009年9月同期住院的300例MG患者临床资料,比较260例甲状腺正常及40例伴甲状腺异常的MG患者临床特点,血清甲状腺抗体与乙酰胆碱受体抗体水平的相关性及外周血T淋巴细胞亚群百分比情况.结果 (1)合并甲状腺异常的MG占13.3%,MG伴甲状腺异常以出现甲状腺抗体阳性为多见(30例,占10.0%);(2)甲状腺正常与异常的MG患者在发病性别构成、年龄分布、病程、临床分型及胸腺病理上差异均无统计学意义;(3)伴有甲状腺抗体阳性MG患者血突触后膜乙酰胆碱受体水平(1.15±0.11)显著高于甲状腺抗体阴性者(1.01±0.11);(4)甲状腺异常组CD8+T淋巴细胞百分比(21.63±5.17)%明显低于甲状腺正常组(24.28±5.79)%,P＜0.05,CD4+/CD8+比值甲状腺异常组明显高于正常组(2.10±0.67比1.81±0.61,P＜0.05).结论 MG合并甲状腺异常以甲状腺抗体阳性多见,伴有甲状腺抗体阳性MG患者血突触后膜乙酰胆碱受体水平显著升高;MG合并甲状腺异常者其外周血T淋巴细胞亚群紊乱更为明显.     

他克莫司治疗重症肌无力疗效及安全性评价

目的 评价他克莫司治疗全身型重症肌无力(MG)的疗效及不良反应.方法 回顾性分析69例全身型MG患者接受他克莫司(2～6 mg/d)治疗前及治疗后1、3、6及12个月时MG严重程度评分及不良反应,并监测服药1个月后他克莫司血药浓度(FK506),分析其与临床疗效的相关性.结果 他克莫司治疗1、3、6及12个月的总有效率分别为81.2％、87.6％、92.2％及93.8％.治疗1个月后,临床显效及好转组FK506[(7.1±3.9) μg/L,(6.3±3.8) μg/L]明显高于无效组[(3.4±1.3) μg/L] (P ＜0.01,P＜0.05).主要的不良反应:血糖升高5例,出现白细胞减少及头晕、耳鸣各3例.结论 他克莫司治疗MG,起效快,临床疗效确切.在治疗剂量范围内其副作用小,主要为血糖升高及骨髓抑制.     

重症肌无力患者妊娠期临床特点及预后分析

目的:探讨重症肌无力（MG）患者妊娠前后肌无力症状的变化及MG对妊娠预后的影响。方法:回顾性研究2013年1月至2018年10月28例就诊于解放军总医院第八医学中心MG患者38例次妊娠分娩的临床资料。比较妊娠前与妊娠不同时期MG严重程度评分、血乙酰胆碱受体（AChR）抗体变化及药物的使用情况,并分析妊娠后MG不同转归（...     

Efficacy of tacrolimus in treatment of polymyositis associated with myasthenia gravis

We report a patient with polymyositis (PM) associated with myasthenia gravis (MG). Both disorders had been controlled for around 15 years by oral prednisolone and a cholinesterase inhibitor following surgical removal of invasive thymoma and radiotherapy, but muscular weakness due to myalgia and an increase in serum levels of myogenic enzymes, mainly ascribable to the recurrence of PM, reappeared immediately after cessation of these drugs, which was done because the patient had multiple bone fractures and severe osteoporosis due to the long-term corticosteroid therapy. Oral tacrolimus was therefore tried, and produced an improvement in muscular symptoms in association with normalization of myogenic enzymes. PM associated with MG as in this patient might be the best indication for tacrolimus, considering its efficacy in MG, but this drug should also be actively considered as a therapeutic option in refractory cases of PM alone, particularly when either corticosteroids or other immunosuppressive agents are not usable.Abbreviations MG Myasthenia gravis - PM Polymyositis     

重症肌无力的膈神经和肋间神经重复电刺激

目的　探讨重症肌无力 (MG)患者膈神经重复电刺激 (PRNS)和肋间神经重复电刺激(IRNS)的特点和临床应用。方法　检测 113例MG患者PRNS、IRNS、肢体及颅神经重复电刺激 (RNS)和临床症状评分 ,并同时检测用力肺活量 (FVC)。结果　FVC取决于PRNS、IRNS及面神经RNS ;I型和Ⅱ型患者存在临床下呼吸功能障碍 ,Ⅱb型与Ⅲ型和Ⅳ型相比 ,虽PRNS波幅递减程度不同 ,但阳性率差异无显著性 ;PRNS和IRNS阳性率高于FVC和呼吸困难症状阳性率 ;PRNS敏感性高于IRNS。结论PRNS和IRNS是直接反映MG患者呼吸功能受累的神经电生理指标 ,有助于准确评估病情及进行临床分类 ;建议对Ⅱ型患者常规行PRNS和IRNS检查。     

The co-existence of myasthenia gravis in patients with myositis: A case series

ObjectiveMyositis and myasthenia gravis (MG) are both autoimmune disorders presenting with muscle weakness. Rarely, they occur simultaneously in the same patient. Since the management of myasthenia gravis differs from that of myositis, it is important to recognize when patients have both diseases. We reviewed the cases of 6 patients with both myositis and MG to identify clinical features that suggest the possibility of co-existing MG in myositis patients.MethodsWe identified 6 patients with dermatomyositis or polymyositis and MG. We reviewed their medical records to assess their clinical presentations, laboratory findings, and electrophysiological features.ResultsAll 6 patients had definite dermatomyositis or polymyositis by the criteria of Bohan and Peter as well as electrophysiologic and/or serologic confirmation of MG. Among overlap patients, 5/6 (83%) had bulbar weakness, 2/6 (33%) had ptosis, and 1/6 (17%) had diplopia. Fatigable weakness was noted by 5/6 (83%) patients. Treatment with pyridostigmine improved symptoms in 5/6 (83%) patients. High-dose steroids were associated with worsening weakness in 2/6 (33%) patients.ConclusionsProminent bulbar symptoms, ptosis, diplopia, and fatigable weakness should suggest the possibility of MG in patients with myositis. A suspicion of MG may be confirmed through appropriate electrophysiologic and laboratory testing. In those with myositis–MG overlap, high-dose steroids may exacerbate symptoms and pyridostigmine may play an important therapeutic role.