Ⅱ型重症肌无力危象的诱因及其多因素分析

目的 探讨Ⅱ型重症肌无力危象的诱因。方法 回顾性分析2014年1月-2018年1月本院诊治的48例Ⅱ型重症肌无力患者的临床病例资料,选择性别、发病年龄、病程、合并感染、激素应用、免疫抑制剂应用、不规则用药史、胸腺切除术史等因素进行多因素回归分析其对此型重症无力危象发生的影响。结果 共21例发生重症肌无力危象,重症肌无力危象的发生与合并感染、不规则用药及激素的使用有关。结论 合并感染、不规则用药及激素的使用是此型重症肌无力危象发生的危险因素,认识这些危险因素对于预防此型重症肌无力危象的发生,改善预后提供了依据。     

研究重症肌无力危象发生的危险因素及其防治措施

目的研究重症肌无力(MG)危象发生的危险因素及防治措施。方法对我院2010-03—2015-10收治的100例重症肌无力患者临床资料进行整理、归纳分析,包括患者年龄、激素治疗、胸腺切除与否等,行单因素及多因素Logistic回归分析,总结重症肌无力危象危险因素并提出相应防治措施。结果 MG危象组平均年龄(33.4±10.5)岁,显著低于MG非危象组的(38.8±11.0)岁,差异有统计学意义(P0.05)。MG危象组激素治疗、合并感染、胸腺切除史比例分别为85.7%、57.1%、54.8%,显著高于MG非危象组的56.9%、12.1%、27.6%,差异有统计学意义(P0.05)。多因素Logistic回归分析年龄、激素治疗、合并感染及胸腺切除史是重症肌无力危象发生的独立危险因素。结论激素治疗、合并感染、胸腺切除术史是重症肌无力危象发生危险因素,需规范激素治疗,积极预防感染及严格筛选胸腺切除手术指征。     

重症肌无力80例临床分析

目的 探讨重症肌无力患者的临床特点、发病情况及治疗效果.方法 回顾性分析了80例重症肌无力患者的发病年龄、发病诱因、临床表现、合并症及治疗效果.结果 重症肌无力患者发病年龄平均(32.5±10.8)岁,男女之比为1.1:1.发病的诱因以感染为首位,80例病人均采用药物治疗,总有效率达97.5%.有35 %的病人合并有胸腺瘤、胸腺增生,行胸腺切除术.结论 重症肌无力临床表现复杂多样,药物及胸腺切除治疗均有效.预防及控制感染是防止重症肌无力反复发作,减少重症肌无力危象的有效方法.     

胸腺瘤切除术后发生重症肌无力的治疗方法和高危因素分析

目的探讨胸腺瘤切除术后发生重症肌无力的治疗方法和高危因素。方法选取我院行胸腺瘤切除术治疗的胸腺瘤患者379例,术后发生重症肌无力35例,未发生重症肌无力344例。术后发生重症肌无力患者采用大剂量激素冲击疗法。观察重症肌无力的治疗效果,分析术后发生重症肌无力的高危因素。结果重症肌无力组治疗总有效率为85.71%;2组手术方式、不完整切除、术后肺部感染比较差异有统计学意义(P0.05)。多因素分析显示,不完整切除及术后肺部感染为发生重症肌动的独立危险因素。结论大剂量激素冲击治疗胸腺瘤切除术后重症肌无力患者的临床疗效满意,不完整切除及术后肺部感染为胸腺瘤切除术后发生重症肌无力的独立危险因素。     

重症肌无力患者胸腺切除术后危象发生的多因素分析

目的:探讨重症肌无力(MG)患者胸腺切除术后危象发生的影响因素。方法:回顾性分析2002年7月至2005年12月确诊为MG患者进行胸腺切除术101例患者的资料。对性别、年龄、术前危象史、Ossermen分型、激素使用时间和剂量、非口服止痛药、术前心电图、血红蛋白、白蛋白、肌力、伴随免疫疾病;手术及麻醉时间、切口方式、切除范围、胸腺瘤、病理类型、输血和病程;术后最高体温、肺部感染、术后第2天白细胞数、中性粒细胞百分比、乙酰胆碱受体抗体(AChRab)、突触前膜受体抗体(PrsmRab)、连接素抗体(Titinab)进行多因素Logistic回归分析。结果:术后出现MG危象29(28.7%)例。危象的发生与伴随免疫疾病、Ossermen分型、术后肺部感染、术前危象史和年龄因素有关。结论:伴随免疫疾病、OssermenⅡb型及以上、术后肺部感染、术前有危象史和大龄患者均易发生术后危象。     

重症肌无力患者胸腺切除术围手术期重症肌无力危象的危险因素分析

目的探讨重症肌无力(MG)患者胸腺切除术围手术期并发肌无力危象的危险因素。方法收集63例MG患者的临床资料,按照围手术期是否发生肌无力危象,分为发生危象组和未发生危象组,分析肌无力危象的危险因素。结果本组发生肌无力危象12例(19.0%)(发生危象组),未发生肌无力危象51例(81.0%)(未发生危象组)。与发生危象组比较,未发生危象组Osserman分型、术式、手术时间、术中出血量及病理类型差异有统计学意义(P0.05~0.01)。多变量Logistic回归分析显示,Osserman分型(Ⅱb+Ⅲ型)、手术时间(3 h)、术中出血量(100 ml)以及病理类型(侵袭性胸腺瘤)是肌无力危象的独立危险因素(OR=55.257,95%CI:7.953~383.909,P=0.000;OR=105.243,95%CI:8.570~1292.357,P=0.000;OR=14.008,95%CI:2.597~75.559,P=0.002;OR=18.552,95%CI:4.368~78.789,P=0.000)。结论 Osserman分型Ⅱb+Ⅲ型、手术时间3 h、术中出血量100 ml以及侵袭性胸腺瘤是胸腺切除术围手术期并发肌无力危象的独立危险因素。充分的术前评估准备、减少术中出血及缩短手术时间有助减少肌无力危象发生。     

74例重症肌无力危象的临床分析

目的 探讨重症肌无力危象患者的临床特点和预后。方法 回顾74 例第一次发生肌无 力危象的重症肌无力患者的一般资料，分析Osserman 分型、危象发生时间、危象前特点、危象持续时间， 评价重症肌无力危象治疗效果及预后。结果 伴胸腺瘤的重症肌无力危象患者45 例，占60.81%，非胸 腺瘤患者29 例，占39.19%。60.81%（45/74）的肌无力危象发生在重症肌无力起病的1 年之内，胸腺切除 术后6 个月内发生危象的比例为76.00%（38/50）。呼吸费力和吞咽费力（24/74）是出现危象前最显著的特 征，其次为胸腺手术后（11/74）、感染（9/74）、激素相关（7/74）。所有危象患者中Osserman 分型ⅡB 型所占 比例最高，为45.95%（34/74）。重症肌无力危象患者的插管时间为15（7，30）d，44.60%（33/74）的危象患者 需要丙种免疫球蛋白和（或）血浆置换联合甲泼尼龙冲击治疗。肌无力危象最常见的并发症为肺部感染 （32/74，43.24%），反复气管插管最严重的并发症为支气管扩张伴出血、气胸。随访2～10 年，17 例患者 出现再次或多次危象，死亡率为9.46%（7/74）。结论 伴胸腺瘤的重症肌无力患者较不伴胸腺瘤患者出 现肌无力危象的比例更高。重症肌无力危象治疗困难，需要多种免疫抑制剂联合治疗。胸腺切除后的 半年内，仍然是肌无力危象发生的高峰。肌无力危象并发症、反复危象、胸腺瘤转移是患者死亡的主要 原因。     

25例重症肌无力的临床分析

对２５例重症肌无力进行回顾性分析。根据首发症状、病程，采用Ｏｓｅｒｍａｎ分型并结合影像学检查及手术胸腺的病理改变，将合并胸腺增生或胸腺瘤的重症肌无力病人的临床特点进行了比较。不论临床分型如何，都可能存在胸腺的异常（４７．６％）。伴胸腺瘤者发病年龄较大，男性居多，病程较短，危象发生率高。胸腺瘤术后仍发生危象有其免疫学基础。此时免疫抑制剂及抗胆碱酯酶的调整及合理应用仍然是重要的     

22例重症肌无力合并胸腺瘤的手术治疗体会

目的 探讨重症肌无力(myasthenia gravis,MG)合并胸腺瘤患者的围手术期处理方法及手术治疗效果.方法 对本院2006-03～2008-03接受手术治疗的22例重症肌无力合并胸腺瘤患者的临床资料进行回顾性分析.采用改良Osserman标准分为:Ⅰ型9例,Ⅱa型5例,Ⅱb型7例,Ⅲ型1例,手术切口采用胸骨正中切口.手术均行胸腺瘤、胸腺脂肪组织切除及纵隔脂肪组织清扫.结果 22例无手术死亡,3例术后早期发生MG危象,经气管切开、辅助呼吸等抢救治疗治愈.结论 完善围手术期处理措施,减少MG危象的发生,手术治疗重症肌无力合并胸腺瘤可获得良好的疗效.     

11例重症肌无力危象患者的护理体会

我科自2007年以来成功救治了11例发生肌无力危象的重症肌无力患者,现将护理体会报告如下。1资料与方法1.1一般资料本组11例重症肌无力危象均为2007-01—2012-09在我科住院的患者,男5例,女6例;年龄21～60岁,平均40岁;病程6个月～10a;诱发因素:呼吸道感染5例,劳累过度3例,应用激素2例,胸腺瘤术后1例。1.2抢救方法肌无力危象发生时采取的急救措施主要包括:(1)及时吸痰,保障气道通畅,持续吸氧,密切配合医生行     

Double filtration plasmapheresis in the treatment of myasthenic crisis--analysis of prognostic factors and efficacy

OBJECTIVES: To examine the prognostic factors and outcome of myasthenia gravis (MG) patients in crisis with double filtration plasmapheresis (DFP) treatment. MATERIAL AND METHODS: A total of 15 patients experienced 20 episodes of crisis during the study period. Plasmapheresis was carried out using a double filtration METHOD: Demographic information, clinical features of crisis, and associated complications were analyzed. RESULTS: The median duration of crisis was 9 days. Chest infection was the most common precipitant of crisis. Twelve out of the 20 episodes (60%) responded well to DFP and mechanical ventilation was discontinued after the third session of DFP in 8 of them. Three significant predictors for prolonged crisis were shorter intervals between the onset of MG and the first crisis (P=0.04), higher serum bicarbonate levels at baseline (P=0.03) and the thymic pathology of thymoma (P=0.03). CONCLUSION: DFP can ameliorate the profound weakness in crisis and seems to be a rational therapy for patients with myasthenic crisis.     

Myasthenia gravis in children: analysis of 18 patients

Myasthenia gravis (MG) in childhood is rare comprising 10 to 20 % of all myasthenic patients. We studied 18 patients with MG whose first symptoms started from 1 to 12 years of age, followed at the Department of Neurology of the UNIFESP-EPM, from January 1983 to August 1997. There were 10 girls and 8 boys (1.2:1). Eleven patients (61%) presented moderate or severe generalized disease and 4 (22%) had at least one myasthenic crisis. EMG with supramaximal repetitive nerve stimulation was diagnostic in 8 (47%) out of 17 patients, and chest CT was normal in 14 patients. Seropositivity to acetylcholine receptor antibodies was found in 81.6% (9 out of 11 tested) and the levels had no relation to clinical severity. Nine out of 16 patients (56%) worsened with pyridostigmine alone and were treated with prednisone. Four out of those nine continued worsening despite steroids and were subjected to thymectomy (all showed thymic lymphoid follicular hyperplasia). Three patients (75%) improved markedly after thymectomy and one (25%) worsened, eventually getting better with intravenous immunoglobulin and oral azathioprine. MG treatment, using all resources available, has to be individualized for each child.     

抗体阴性重症肌无力的短程血浆置换疗法初探

血浆置换(plasma exchange,PE)在重症肌无力(MG)上的疗效已有报道,但缺乏明确一致的置换方案。我们对12例MG患者进行了37人次的短程PE,各例均在10d内行3～4次置换术,术前和术后第7天按Mantegazza肌力评分表进行临床疗效评定,结果除1例外,均获得肯定疗效,仅1人次发生较严重的副反应。4例抗体阴性病人置换后均获显著疗效,提示抗体阴性MG血浆中存在某些未知的致病因子。短程PE可供选择作为MG危象的治疗方案。     

Myasthenic crisis: clinical features, complications and mortality

BACKGROUND AND OBJECTIVE: Myasthenic crisis is a life-threatening complication of myasthenia gravis (MG) and when treated aggressively is associated with good outcome. MATERIALS AND METHODS: Retrospective study of case records of patients with episodes of myasthenic crisis. RESULTS: Twenty-one (22%) of the 95 patients with MG (9 with thymoma), experienced 23 episodes of myasthenic crisis, 3 (33%) in patients with thymoma. The crisis episodes occurred within 2 years of disease onset in 11 (52%) patients. Infection was the most common primary precipitant of the crisis occurring in 65%. The median duration of the crisis episode was 11 days (7-39 days), and the median neurological intensive care unit stay was 15 days (range 9-47 days). Fifteen (65%) episodes were treated with small volume plasma exchange (PE) and 8 (35%) episodes received intravenous immunoglobulin (IVIg). The time taken for disease stabilization, the median number of days for extubation, was 8 days (range 7-12) in the PE group and 10 days (range 7-39) in the IVIg group. Disease stabilization could not be achieved in one patient in the IVIg group. Ventilator-associated pneumonia (VAP) was the commonest complication, seen in 30%. Two (8%) of the 23 episodes of crisis were fatal, one resulting from VAP and septicemia, and the other due to crisis itself. All the 19 patients who survived to discharge had complete resolution of admission symptoms. CONCLUSIONS: In patients with myasthenic crisis, both therapeutic options, PE and IVIg, are equally effective in disease stabilization. To achieve good outcomes all efforts should be directed at decreasing the duration of intubation and also aggressively treating the associated medical complications.     

重症肌无力病人危象82例临床分析

目的 探讨重症肌无力（MG）危象的诱发因素、延长气管插管的因素以及并发症、病死率。方法 回顾性分析了82例MG病人（87次危象）的年龄、性别、发病年龄、诱发因素、气管插管并发症及影响插管时间的因素。结果 75％病人存在诱发危象的因素，其中感染为常见因素，其次外伤、劳累、月经及分娩，医源性因素占24％。有9个因素延长气管插管。12例死亡，病死率为14％。结论 积极治疗及预防肌无力危象病人气管切开后并发症，可有效缩短气管插管的时间，降低病死率。     

Safety of influenza vaccination in patients with myasthenia gravis: A population‐based study

Influenza vaccination has been associated with adverse events including Guillain–Barré syndrome. Because the safety of influenza vaccination in patients with myasthenia gravis (MG) has not been established, some clinicians discourage vaccination for these patients. We explored whether the administration of influenza vaccine to patients with MG might increase the risk of myasthenic crisis. Using population‐based healthcare data from Ontario, Canada, from 1992 to 2007, we utilized the self‐matched, case‐series method of detecting adverse events following vaccination. We studied patients with established myasthenia who were hospitalized for MG within 42 weeks of influenza vaccination. We defined the primary risk interval as the 6 weeks following vaccination. Between January 1, 1992 and March 31, 2006, we identified 3667 hospital admissions for MG. No seasonal trend in MG admissions was evident. In 513 instances, hospitalization occurred within 42 weeks following vaccination in patients previously diagnosed with MG. Among these patients, 266 (52%) were men, the median age was 74 years, and 86 (17%) had previously undergone thymectomy. The estimated relative incidence of admission for MG in the primary risk interval compared with the control interval was 0.84 (95% confidence interval 0.65–1.09). We found similar results in stratified analyses according to gender, age, and thymectomy status. Vaccination of patients with MG against influenza was not found to be associated with exacerbations of the disease. Our findings do not support the practice of withholding influenza vaccination in patients with MG. Muscle Nerve, 2009     

Mycophenolate mofetil – as an adjunctive immunosuppressive therapy in refractory myasthenia gravis: The Singapore experience

We report our experience, using mycophenolate mofetil (MyM) as an adjunctive immunosuppressive therapy in patients with severe, refractory and high dose steroid-dependant myasthenia gravis (MG). Five patients were commenced on MyM in addition to other immunosuppressive therapies. All had significant clinical improvement and no subsequent myasthenic crisis requiring intensive care unit admission. MyM was well tolerated and no serious adverse effects were observed. MyM is an effective adjunctive therapy for the treatment of severe, refractory and steroid-dependant MG in our experience.     

Burden of illness in patients with treatment refractory myasthenia gravis

Introduction: This study assessed the clinical burden of refractory myasthenia gravis (MG), relative to nonrefractory MG. Methods: Rates of myasthenic crises, exacerbations, inpatient hospitalizations, and emergency room (ER) visits over a 1‐year period were measured for 403 refractory, 3,811 nonrefractory, and 403 non‐MG control patients from two administrative health plan databases. Results: Compared with nonrefractory patients, a significantly greater percentage of refractory patients had at least one myasthenic crisis (21.3% vs. 6.1%; P < 0.001) and at least one exacerbation (71.2% vs. 32.4%; P < 0.001) over a 1‐year period. Refractory patients were also significantly more likely to be hospitalized and/or have an ER visit than nonrefractory patients and non‐MG controls (P < 0.001 for all). Discussion: Refractory MG patients have significantly greater clinical burden and are more likely to utilize intensive healthcare resources than nonrefractory patients. Furthermore, refractory patients may be at greater risk of crises throughout the disease course than previous studies have suggested. Muscle Nerve 58 : 99–105, 2018     

Clinical characteristics and predictive factors of myasthenic crisis after thymectomy

The aims of this study were to assess the clinical characteristics of myasthenic crisis after thymectomy (MCAT) and to identify predictors affecting the occurrence of MCAT. Of 66 patients with myasthenia gravis (MG), MCAT occurred in 20 patients (30.3%). The median time interval from thymectomy to MCAT was 3.4 months. MCAT occurred in 65.0% of patients within the first 6 months of a thymectomy, and 35.0% after 6 months. A second MCAT occurred in nine (45.0%) patients who survived the first MCAT, and in seven (50.0%) of 14 patients with a history of a preoperative myasthenic crisis before thymectomy (MCBT). A history of MCBT, and clinical factors reflecting perioperative clinical severity at thymectomy, including preoperative Osserman’s grade, bulbar symptoms, use of immunosuppressants, pulmonary function, and postoperative delayed ventilator weaning, were significantly correlated with the occurrence of MCAT on univariate analysis. However, a history of MCBT was the only independent factor affecting the occurrence of MCAT on multivariate logistic regression analysis (odds ratio, 17.9; 95% confidence interval, 4.019-79.873; p < 0.001). Thus, the occurrence of MCAT may be correlated only with a history of MCBT rather than with factors reflecting perioperative clinical severity. MG patients with a history of MCBT are more susceptible to MCAT, particularly within the first 6 months of thymectomy.     

Myasthenia gravis in Hong Kong Chinese: 2. Paediatric disease

In a study covering 85% of the population of Hong Kong, 39% of all myasthenia gravis (MG) patients, i.e. 103 individuals (54 girls and 49 boys) were found to have had MG with onset before puberty. Two patients had transient neonatal MG, 20 had early onset juvenile MG and 81 had late onset juvenile MG. Restricted ocular MG occurred in 71% of patients and the remainder had generalised MG. The median age at onset was 4 years. Complete remission occurred in 34 patients (34%), a good response in 14 (14%), and fair response in 32 (32%). The clinical course remained static in 16 patients (16%) and 3 patients deteriorated. Two patients died, 1 with myasthenic crisis and the other with cholinergic crisis. All patients, except 2 with neonatal MG, were initially treated with anticholinesterase, but 24% also required steroid therapy. Thymectomy was performed for 12 patients, of whom 5 (42%) showed marked improvement. Thymic histology was normal in 3, showed hyperplasia in 6, non-invasive thymoma in 1 and involution in 2. The most commonly associated disease was Graves' disease which occurred in 7 patients (7%).