无创机械通气在重症肌无力危象治疗中的疗效

目的 研究应用双水平气道正压 通气(BiPAP)治疗重症肌无力所致急性呼吸衰竭的治疗作用。方法 回顾性分析2011-2013年收治的重症肌无力合并急性呼吸衰竭患者而使用双水平气道正压通气(BiPAP)治疗23例,比较他们BiPAP 治疗前、治疗后2 h的动脉血气分析数据的变化,心率、呼吸频率及治疗2周后症状改善情况(QMG评分),并进行统计学分析。结果 23例患者中有18例无创机械通气治疗前患者心率(HR),呼吸频率(RR),血浆pH值,动脉血氧分压(PaO₂),动脉血二氧化碳分压(PaCO₂)分别为(108.7±17.6)次/min,(25.8±5.3)次/min,7.31±0.04,(58.7±13.3)mmHg,(53.8±7.8)mmHg; 治疗后2小时患者HR、RR、血浆pH值PaO₂、PaCO₂分别为(83.4±15.9)次/min,(21.1±4.9)次/min,7.36±0.04,(83.4±15.7)mmHg,(38.6±7.0)mmHg,较治疗前均明显改善。(P<0.05),治疗前患者QMG评分为17.94±2.07分,治疗2周后患者QMG评分为(10.06±2.71)分,也较治疗前有显著改善。(P<0.01)。结论 BiPAP治疗对重症肌无力所致急性呼吸衰竭的有重要治疗价值,能有效改善患者的呼吸功能。     

重症肌无力危象的机械通气治疗

目的　探讨机械通气治疗重症肌无力危象的指征、通气模式的选择及撤离机械通气的方法和指征。方法　分析 2 0例重症肌无力危象患者机械通气治疗前后血气指标 ,以及 1 2例患者机械通气初和撤离机械通气前床边呼吸功能检查。结果　 2 0例重症肌无力危象患者机械通气前后 3项血气指标有显著性差异 (P <0 0 5) ,其中 1 2例患者机械通气初和撤离机械通气前 2项呼吸功能检查有显著性差异 (P <0 0 5)。结论　呼吸衰竭是直接威胁重症肌无力危象患者生命的首要因素 ,及时气管切开行机械通气治疗 ,改善患者的通气功能是抢救成功的关键 ,积极控制肺部感染 ,消除诱因 ,使用大剂量糖皮质激素是患者早日撤离机械通气的保证。     

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。     

无创式机械通气与有创式机械通气治疗重症肌无力危象

目的比较无创性错落式正压通气(noninvasive bilevel positive ventilation,BiPAP)和有创式机械通气(invasive mechanical ventilation,IV)治疗重症肌无力危象(myasthenic crisis,MC)的临床疗效。方法收集2004-12—2005-12北京医院神经内科住院的40例MC患者临床资料,其中24例行BiPAP治疗,16例行气管插管IV治疗。于治疗前及治疗后1 h和24 h行动脉血气分析并记录心率、呼吸频率。结果使用BiPAP治疗者中18例(75%)避免了行气管插管。两组治疗1、24 h后血气分析、呼吸频率和心率均明显正常化(均P<0.05),两组间差异无统计学意义。IV治疗组呼吸机相关肺炎发生率、抗生素使用率较高,而BiPAP治疗组胃部不适发生率明显为高(P<0.05)。IV治疗组住院费用较BiPAP成功治疗组高(P<0.05)。结论(1)对MC患者可首先采用BiPAP治疗,大部分MC患者可避免气管插管;(2)于MC发生前24 h行BiPAP和IV治疗其疗效相同,BiPAP治疗组并发症较Ⅳ治疗组少。     

重症肌无力危象的临床护理

重症肌无力（MG）是神经肌肉部位传递障碍引起的一种自身免疫性疾病,病变主要累及神经肌肉接头后膜上乙酰胆碱受体,主要症状为部分或全身骨骼肌极易疲劳,通常在晨轻晚重,活动后加剧,经过休息和胆碱酯酶抑制剂治疗后有所恢复。肌无力危象是MG病人突然发生呼吸肌无力,致呼吸极度困难,难以吞咽、不能维持基本生活和生命体征的危急状态,其病死率为15．4％～50．0％,常见诱因为强烈的精神刺激、劳累、停药、感染等,若抢救不及时可加重病情,增加医疗费用,或因窒息及发生呼吸功能衰竭而死亡,     

重症肌无力危象的诱发因素、抢救及其护理

重症肌无力危象是神经内科的急重症,主要表现为急性呼吸功能衰竭,若抢救不及时,可因呼吸肌无力而窒息或呼吸衰竭死亡,其病死率可达15%～50%[1]。适时气管切开,恰当的呼吸机管理、呼吸道护理是提高抢救成功率,减少病死率的关键。本文对16例重症肌无力危象患者的临床资料     

大剂量丙球蛋白抢救重症肌无力危象的临床研究

目的:探讨大剂量丙球蛋白(Ig)在抢救重症肌无力危象的临床疗效。方法:根据Qsseman分型,Ⅱa型2例、Ⅱb型8例、Ⅲ型13例、Ⅳ型3例MG危象患者在综合抢救的同时联合应用大剂量Ig静脉注射为治疗组。Ⅱa型1例、Ⅱb型6例、Ⅲ型15例、Ⅳ型2例综合治疗患者为对照组。以临床绝对评分及临床相对评分作为治疗前、后疗效判定标准,于治疗前、后2周抽清晨空腹血查AChRAb,观察治疗前、后重症肌无力的免疫状态的变化。结果:治疗组治疗前、后AChRAb有明显差异(P<0.01)。对照组治疗前、后无明显差异。临床评分两组治疗前、后均有明显差异(P<0.05),但治疗组治疗前、后分值差明显大于对照组(P<0.01)。经治疗后两组危象持续时间及有效率有明显差异(P<0.01)。结论:大剂量Ig在抢救MG危象中疗效明显、安全、可靠,值得临床应用。     

重症肌无力危象多学科协作的华山经验

重症肌无力危象(MC)是重症肌无力(MG)患者出现呼吸衰竭、需要机械呼吸支持的最危急状态,具有较高的在院病死率和较长的ICU住院时间。在神经重症监护室内建立“危象单元”,由神经内科、重症医学科、感染科、输血科、心内科、护理部和康复医学科等多学科团队组成医疗综合体,从气道管理、快速疗法、重症肺炎的监测和预防、身心护理及综合康复等方面,为MC患者提供高效的个体化治疗。文中介绍复旦大学附属华山医院的单中心经验,以探讨MC患者管理的多学科诊疗协作模式。     

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例重症肌无力危象患者,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例重症肌无力危象病人均为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。诱发因素分别为: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。     

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.     

支气管肺泡灌洗治疗重症肌无力危象肺不张的临床研究

目的　探讨支气管肺泡灌洗对重症肌无力 ( MG)危象合并肺不张的治疗作用。方法　对 8例经常规吸痰及抗生素治疗无效的 MG危象合并肺不张患者 ,行支气管肺泡灌洗治疗 ,并根据患者的病情积极配合免疫治疗。结果　 8例患者中 ,2例由于吸入、6例由于痰液阻塞而致肺不张 ,经支气管肺泡灌洗治疗 1～ 3次均出现肺复张 ,8例患者均于治疗后 1周内脱离机械通气治疗。结论　支气管肺泡灌洗是治疗 MG危象合并肺不张的一种安全有效的方法 ,积极配合抗生素及免疫治疗可及早促进患者肺功能恢复 ,早日脱离机械通气治疗。     

A Patient with Coexisting Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome

Background

The coexistence of myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) is very rare and remains controversial.

Case Report

A 48-year-old woman initially presented with noticeable right ptosis and intermittent diplopia. She then developed fluctuating proximal limb weakness and difficulty in swallowing. The serum titer of anti-acetylcholine-receptor antibody was elevated and the edrophonium (Tensilon) test was positive. However, repetitive nerve stimulation revealed abnormalities typical of LEMS. The patient exhibited a good response to treatment with anticholinesterase inhibitors and steroids, and long-term evaluation disclosed that she presented with the clinical, electrophysiological, and immunological characteristics of both diseases.

Conclusions

The reported clinical and electrophysiological features suggest that this patient was a very rare case of combined MG and LEMS.     

癌性Lambert-Eaton肌无力综合征四例临床及电生理研究

目的研究癌性Lambert－Eaton肌无力综合征（LEMS）临床及电生理特征。方法回顾性地评价了4例病理证实的癌性LEMS（例1、2为小细胞性肺癌，例3为肾上腺癌，例4为乳腺癌）的临床和电生理资料。结果面神经、腋神经及尺神经重复电刺激示，诱发电位起始波幅减小（0．4～0．7mV）；5Hz以下低频刺激后波幅递减16％～54％（对照：小于15％），但在10Hz以上高频刺激后波幅递增120％～657％（对照：小于65％）；例2和例3肌电图和神经传导速度测定示，多发周围神经源性损害，以感觉神经纤维轴索损害为主。结论观察结果表明，部分癌性LEMS患者的神经肌肉接头和周围神经可同时受累。     

Efficacy and safety of IVIG in CIDP: Combined data of the PRIMA and PATH studies

Intravenous immunoglobulin (IVIG) is a potential therapy for chronic inflammatory demyelinating polyneuropathy (CIDP). To investigate the efficacy and safety of the IVIG IgPro10 (Privigen) for treatment of CIDP, results from Privigen Impact on Mobility and Autonomy (PRIMA), a prospective, open‐label, single‐arm study of IVIG in immunoglobulin (Ig)‐naïve or IVIG pre‐treated subjects (NCT01184846, n = 28) and Polyneuropathy And Treatment with Hizentra (PATH), a double‐blind, randomized study including an open‐label, single‐arm IVIG phase in IVIG pre‐treated subjects (NCT01545076, IVIG restabilization phase n = 207) were analyzed separately and together (n = 235). Efficacy assessments included change in adjusted inflammatory neuropathy cause and treatment (INCAT) score, grip strength and Medical Research Council (MRC) sum score. Adverse drug reactions (ADRs) and ADRs/infusion were recorded. Adjusted INCAT response rate was 60.7% in all PRIMA subjects at Week 25 (76.9% in IVIG pre‐treated subjects) and 72.9% in PATH. In the pooled cohort (n = 235), INCAT response rate was 71.5%; median time to INCAT improvement was 4.3 weeks. No clear demographic differences were noticed between early (responding before Week 7, n = 148) and late responders (n = 21). In the pooled cohort, median change from baseline to last observation was ?1.0 (interquartile range ?2.0; 0.0) point for INCAT score; +8.0 (0.0; 20.0) kPa for maximum grip strength; +3.0 (1.0; 7.0) points for MRC sum score . In the pooled cohort, 271 ADRs were reported in 105 subjects (44.7%), a rate of 0.144 ADRs per infusion. This analysis confirms the efficacy and safety of IgPro10, a recently FDA‐approved IVIG for CIDP, in a population of mainly pre‐treated subjects with CIDP [Correction added on 14 March 2019 after first online publication: the INCAT response rate has been corrected.].     

甲基强的松龙与硫唑嘌呤联合治疗对重症肌无力临床和免疫功能的影响

目的　通过检测重症肌无力 ( MG)患者乙酰胆碱受体 ( ACh R)特异性 IFN-γ、IL-1 0分泌细胞水平 ,观察大剂量甲基强的松龙 ( MP)与硫唑嘌呤 ( AZ)联合治疗对 MG患者 Th1及 Th2类细胞因子 ( CK)的影响。方法　用酶联免疫斑点 ( ELISPOT)技术检测 2 1例 MG患者免疫治疗前后外周血 ACh R特异性 IFN-γ、IL-1 0分泌细胞的数目。同时进行临床绝对评分。结果　 MP与 AZ联合治疗可使 MG患者外周血 ACh R特异性 IFN-γ分泌细胞的数目减少 ,对 IL-1 0分泌细胞影响不大。IFN-γ分泌细胞水平与临床绝对评分呈明显正相关 ( r=0 .5 1 2 ,P <0 .0 1 )。结论　 MP与 AZ联合治疗可抑制 MG患者 Th1类 CK的产生 ,恢复 Th1和 Th2类细胞因子之间的平衡 ,继而改善临床症状。 IFN-γ分泌细胞水平可作为反映肌无力严重程度的指标。     

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 profile and outcome of myasthenic crisis in a tertiary care hospital: A prospective study

Background:

The present understanding of the clinical course, complications, and outcome of myasthenic crisis (MC) is based chiefly on observational studies and retrospective case series.

Aim:

To study the baseline demographic and clinical variables, risk factors, complications, outcome, and mortality in patients of MC.

Materials and Methods:

All patients of myasthenia gravis (MG) who presented with myasthenic crisis between July 2009 and December 2010 were included.

Results:

Ten patients of MC were included in this study. The median age of the patients was 40.5 years (range 14-71 years). Seven were females and three were males. Nine had generalized MG and one patient had oculobulbar involvement only. Median duration of disease was 3 years (range 1 month to17 years). Two patients had thymoma. Two patients had history of thymectomy in the past. Infection was the most common triggering factor accounting for five cases (50%) followed by inadequate treatment/drug withdrawal in three (30%) and steroid initiation and hypokalemia in the remaining two patients (20%). Median duration of MC was 12 days (range 3-28 days). Mortality was in 3 out of 10 (30%) during MC. Management in the intensive care unit (ICU) and treatment with plasma exchange/intravenous immunoglobulins were associated with good outcome.

Conclusions:

Ventilator support and management in intensive care unit are the most important components in the management of MC. The high mortality rate seen in present study may be more reflective of the actual ground reality in resource constrained developing countries, however, larger prospective studies are needed to confirm these findings.