重症肌无力危象患者气管插管或气管切开的管理

目的对重症肌无力发生危象患者行气管插管或气管切开管理的总结。方法 28例重症肌无力危象患者,其中26例采用经前胸第二肋间横断胸骨小切口行胸腺切除加前纵隔脂肪清扫术术后发生危象,2例因感冒诱发危象。对危象患者积极进行气管插管或气管切开,加强呼吸道管理。结果 25例患者安全渡过危象期,自动出院2例,死亡1例。结论规范合理的呼吸道管理可保证MG患者安全渡过危象期,提高治愈效果,降低病死率。     

重症肌无力危象患者的肠内营养支持

目的探讨肠内营养支持对重症肌无力(MG)危象的营养状况和疾病发展的影响。方法回顾分析本院2004-10～2010-10收治的16例MG危象患者的肠内营养治疗经过,观察营养治疗前后ALB、PA、Hb、TSF及气管插管时间、危象持续时间、病死率。结果气管插管时间最短3d,最长52d,平均(17±10)d;危象持续时间最短2d,最长50d,平均(15±8)d。ALB、PA较治疗后明显提高,差异有统计学意义(P<0.05),Hb、TSF无明显变化,差异无统计学意义(P>0.05),病死率12.5%。结论肠内营养支持可改善MG危象病人的营养状况、有效缩短气管插管的时间和危象持续时间,降低病死率。     

27例重症肌无力危象抢救分析

目的复习重症肌无力（MG）危象的抢救体会及经验。方法对近5年抢救27例MG危象的临床特点、抢救措施进行总结分析。结果平均发病年龄约41岁，感染为常见诱因，气管切开，呼吸机辅助呼吸，静脉注射大剂量免疫球蛋白（IVIg）及肾上腺皮质激素（ACS）冲击治疗，预防感染为主等抢救治疗方法，无1例死亡。结论及时插管至气管切开。应用呼吸机，预防感染，肾上腺皮质激素冲击治疗，静脉注射大剂量免疫球蛋白（IVIg）等综合抢救措施，明显缩短了病程，显著降低了死亡率。     

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。     

重症肌无力18例死因分析

文献报告重症肌无力(简称MG)病死率为5.7％,肌无力危象病死率为15.4～50％。本院1968年3月～1991年5月住院MG患者80例、死亡18例,病死率为22.5％,今就18例的死亡原因予以分析探讨。     

隔日顿服强的松治疗重症肌无力疗效及安全性观察

目的 探讨隔日顿服强的松治疗重症肌无力（MG）患者的临床疗效及安全性。方法 对116例MG患者的临床及副作用表现进行跟踪观察。结果 初诊MG患者隔日顿服渐增法，显效率91．4％；MG危象患者，危象控制后强的松渐减法，均取得成功。未发现肌无力加重现象，副作用发生率为16．4％。结论 强的松可作为治疗MG的第一线药物，采用隔日顿服法临床疗效明显，避免初期肌无力加重现象，减少副作用发生。     

延髓型重症肌无力166例的临床特点

目的 研究延髓型重症肌无力（MG）的临床特点。方法 回顾性分析1983年5月至2005年10月间我院诊治的166例延髓型MG患者的临床资料，并与本院MG资料库中其他类型的NG患者2722例进行比较。结果 延髓型MG在本组患者中的发生率为5．7％（166／2888）。女性多于男性（男：女=1：1．35），发病年龄高峰为20～40岁。危象发生率高（44／166，26．5％），18例（10．8％）延髓型MG患者半年内发生危象，总病死率达6．0％（10／166）。肺部感染发生率高（30／166，18．1％）。早期误诊率高达19．9％（33／166）。治疗相对困难，激素“中剂量冲击、小剂量维持”疗法和中剂量环磷酰胺疗法短期疗效较好，胸腺切除术远期缓解达80．0％（20／25）。结论 本组结果显示，延髓型MG具有不同于其他类型MG的临床特点，充分认识其临床特点有助于降低误诊率，提高诊断水平和治疗效果。     

神经外科术后气管切开术的并发症与手术时机的选择

目的 探讨神经外科术后气管切开术并发症发生率及气管切开时机的选择。方法 回顾性分析2012年8月至2013年8月收治的266例神经外科术后建立人工气道的临床资料。结果 266例中,单纯气管插管209例（18例出院随访数据缺失）,其中气管插管时间<7 d 148例,7~14 d 24例,>14 d 19例;行气管切开术57例[3例出院随访数据缺失,余54例中,早期气管切开术（气管插管时间≤7 d）38例,晚期气管切开术（气管插管时间>7 d）16例]。单纯气管插管病人肺部感染发生率及院内病死率均明显低于气管切开术病人（P<0.05）,入住ICU时间较气管切开术病人明显缩短（P<0.05）。住院期间,早期与晚期气管切开术病人肺部感染发生率、机械通气时间、入住ICU时间、GCS评分均无统计学差异（P>0.05）;出院后,早期与晚期气管切开术病人严重出血、皮下气肿、气胸、肺部感染、气管狭窄等发生率以及病死率均无统计学差异（P>0.05）。结论 神经外科术后病人是否需要早期气管切开术或晚期气管切开术,需要综合考虑病人情况及利弊得失,做出对病人最有利的决策。     

27例重症肌无力危象抢救分析

目的复习重症肌无力(MG)危象的抢救体会及经验。方法对近5年抢救27例MG危象的临床特点、抢救措施进行总结分析。结果平均发病年龄约41岁,感染为常见诱因,气管切开,呼吸机辅助呼吸,静脉注射大剂量免疫球蛋白(IVIg)及肾上腺皮质激素(ACS)冲击治疗,预防感染为主等抢救治疗方法,无1例死亡。结论及时插管至气管切开,应用呼吸机,预防感染,肾上腺皮质激素冲击治疗,静脉注射大剂量免疫球蛋白(IVIg)等综合抢救措施,明显缩短了病程,显著降低了死亡率。     

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。     

Myasthenic crisis

Myasthenic crisis is a serious occurrence, affecting up to 27% of patients with myasthenia gravis. In the approach to the patient with myasthenic crisis, (1) the diagnosis of myasthenia gravis is confirmed; (2) respiratory failure is evaluated and treated in the intensive care unit, while potential precipitating factors are identified and managed; (3) immunomodulatory treatment is initiated; and (4) complications are avoided or managed promptly. The mortality rate should be 5% or less, with the elderly being most vulnerable.     

The management and outcome of patients with myasthenia gravis treated acutely in a neurological intensive care unit

The management and clinical course of patients with myasthenia gravis admitted to a neurological intensive therapy unit (ITU) over a 66 month period were reviewed. Twenty-seven patients were admitted in myasthenic crisis, eight of whom had multiple admissions. One patient had a cholinergic crisis and a further patient an acute myocardial infarction. A specific aetiological factor precipitating myasthenic crisis was identified in 19 instances: infection (8), reduction in medication (5), menstruation (4), and steroid administration (2). Thirteen patients with crisis had had a previous thymectomy, six with thymoma. Twenty-three out of 35 (66%) patients admitted in crisis required intubation; nine subsequently needed a tracheostomy. Twenty-nine patients received plasma exchange and seven intravenous immunoglobulin. Four patients in myasthenic crisis died in ITU [adult respiratory distress syndrome (1), disseminated intravascular coagulation and cytomegalovirus (CMV) pneumonitis (1), cardiac failure (1) and multiple organ failure (1)]. Appropriate management of myasthenia gravis requires the easy availability of specialised neuro-intensive care facilities.     

Myasthenia gravis: management of myasthenic crisis and perioperative care

Myasthenic crisis may be defined as respiratory failure or delayed postoperative extubation for more than 24 hours resulting from myasthenic weakness. Myasthenic crisis results from weakness of upper airway muscles leading to obstruction and aspiration, weakness of respiratory muscles leading to reduced tidal volumes, or from weakness of both muscle groups. About one-fifth of patients with myasthenia gravis experience crisis, usually within the first year of illness. Over the last four decades, prognosis from myasthenic crisis has dramatically improved from a mortality rate of 75% to the current rate of less than 5%. Common precipitating factors for myasthenic crisis include respiratory infections, aspiration, sepsis, surgical procedures, rapid tapering of immune modulation, beginning treatment with corticosteroids, exposure to drugs that may increase myasthenic weakness, and pregnancy. Myasthenic crisis should not be fatal, as long as patients receive timely respiratory support and appropriate immunotherapy to reduce myasthenic weakness of the upper airway and respiratory muscles. Myasthenic patients with oropharyngeal or respiratory muscle weakness should receive preoperative plasma exchange or intravenous immunoglobulin therapy to a minimal level of weakness to prevent postoperative complications.     

Predictors of outcome of myasthenic crisis

There is paucity of study on predictors of myasthenic crisis (MC), prolonged ventilation and their outcome, a reason why this study was undertaken. Sixty-four patients with myasthenia gravis (MG) were included whose median age was 45 (6–84) years. Their clinical treatment, presence of thymoma, anti-acetylcholine receptor antibody (AchRAb), thymectomy, comorbidities, offending drugs and occurrence of MC were noted. Patients needing prolonged ventilation (>15 days) were noted. Hospital mortality, MG quality of life (QOL) at discharge and thereafter annual hospital visit, admission, expenditure and work day loss were enquired. Fourteen (21.9 %) patients had MC within 1–120 (median 8.5) months of disease onset within a median follow-up of 48 (3–264) months. The precipitating factors were infection in six, surgery in five, tapering of drugs in two and reaction to iodinated contrast in one patient. Male gender, bulbar weakness, AchRAb, thymoma, surgery and comorbid illnesses were related to MC. Eight of them (57.1 %) needed prolonged ventilation. Half the patients with MC had recurrent crisis (2–4 attacks). Death was not related to MC although MC patients had worse QOL, higher annual treatment expenditure with frequent hospital visit and hospitalization. In conclusion, association of comorbid illness with MC and prolonged ventilation highlights the need of close follow-up and appropriate management.     

Ventilatory failure in myasthenia gravis.

This retrospective study over the decade 1969-1978 examines the precipitating factors and outcome in thirty-one patients with myasthenia gravis who developed ventilatory failure. An unusual example of chronic alveolar hypoventilation is discussed in detail. The most favourable outcome occurred in younger patients with a hyperplastic thymus, in contrast to a poorer outlook for older patients with an atrophic gland. Eleven patients died during the period of follow up: three deaths were unrelated to myasthenia but the remaining eight were attributed directly or indirectly to it. The mortality of 36% represents a marked improvement on a 70% mortality in a similar group of patients, reported from this hospital for the years 1960-1968.     

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.     

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.     

The Role of Non-invasive Ventilation and Factors Predicting Extubation Outcome in Myasthenic Crisis

Introduction  Myasthenic crisis is a great threat to patients with myasthenia gravis. Usage of non-invasive ventilation (NIV) to prevent intubation and timing of extubating of patients in myasthenic crisis are important issues though not well documented. Methods  To explore the factors predicting NIV success and extubation outcome in myasthenic crisis, we reviewed the records of 41 episodes of myasthenia crisis. Results  NIV was applied to 14 episodes of myasthenic crisis and eight (57.1%) of them were successfully prevented from intubation. An Acute Physiology and Chronic Health Evaluation (APACHE) II score of <6 and a serum bicarbonate level of <30 mmol/l were independent predictors of NIV success. For patients undergoing invasive mechanical ventilation, extubation failure was observed in 13 (39.4%) of 33 episodes, and the most common cause was sputum impaction due to a poor cough strength (61.5%). A maximal expiratory pressure (Pemax) of ≥40 cmH₂O was a good predictor of extubation success. Extubation failure led to poorer outcomes. Conclusions  NIV may be applied to those patients with a low APACHE II score and a lesser degree of metabolic compensation for respiratory acidosis. For patients undergoing invasive mechanical ventilation, extubation failure is associated with significant in-hospital morbidity in myasthenic crisis. Adequate levels of Pemax and cough strength correlate significantly with extubation success.     

Noninvasive ventilation in myasthenic crisis

BACKGROUND: Myasthenic crisis (MC) is often associated with prolonged intubation and with respiratory complications. OBJECTIVES: To assess predictors of ventilation duration and to compare the effectiveness of endotracheal intubation and mechanical ventilation (ET-MV) with bilevel positive airway pressure (BiPAP) noninvasive ventilation in MC. DESIGN: Retrospective cohort study. SETTING: Academic research. Patients We reviewed consecutive episodes of MC treated at the Mayo Clinic, Rochester, Minnesota. MAIN OUTCOME MEASURES: Collected information included patients' demographic data, immunotherapy, medical complications, mechanical ventilation duration, and hospital lengths of stay, as well as baseline and preventilation measurements of forced vital capacity, maximal inspiratory and expiratory pressures, and arterial blood gases. RESULTS: We identified 60 episodes of MC in 52 patients. BiPAP was the initial method of ventilatory support in 24 episodes and ET-MV was performed in 36 episodes. There were no differences in patient demographics or in baseline respiratory variables and arterial gases between the groups of episodes initially treated using BiPAP vs ET-MV. In 14 episodes treated using BiPAP, intubation was avoided. The mean duration of BiPAP in these patients was 4.3 days. The only predictor of BiPAP failure (ie, requirement for intubation) was a Pco(2) level exceeding 45 mm Hg on BiPAP initiation (P= .04). The mean ventilation duration was 10.4 days. Longer ventilation duration was associated with intubation (P= .02), atelectasis (P< .005), and lower maximal expiratory pressure on arrival (P= .02). The intensive care unit and hospital lengths of stay statistically significantly increased with ventilation duration (P< .001 for both). The only variable associated with decreased ventilation duration was initial BiPAP treatment (P< .007). CONCLUSIONS: BiPAP is effective for the treatment of acute respiratory failure in patients with myasthenia gravis. A BiPAP trial before the development of hypercapnia can prevent intubation and prolonged ventilation, reducing pulmonary complications and lengths of intensive care unit and hospital stay.     

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.