Identification of international classification of functioning,disability and health relevant categories to describe functioning and disability of patients with myasthenia gravis

Purpose.?To describe functioning and health of patients with myasthenia gravis (MG) and to identify which are the most common problems patients encounter, by using the international classification of functioning, disability and health (ICF).

Method.?Adult patients with MG were recruited at C. Besta Neurological Institute. The ICF checklist was administered in individual sessions. Categories were identified as relevant if they were reported as a problem by more than 30% of patients (within activities and participation, the threshold was counted on capacity qualifier).

Results.?One hundred two patients were enrolled (mean age 47.2; inpatients were 29.4%, females 68.6%) and 54 ICF categories were selected: 14 body functions categories (26% out of total selected categories), 2 body structures (4%), 22 activities and participation categories (41%) and 16 environmental factors (29%). Environmental factors were essentially reported as facilitators.

Conclusions.?Twelve ICF categories, not contained in ICF core-sets for neurological condition, related to mobility, household and labour activities were identified. The ICF categories identified in this study are an useful guideline for clinicians and researchers, for monitoring interventions and follow-up of clinical conditions on a broad set of functional areas, and for developing ICF-based assessment tools for patients with MG.     

Eculizumab for the treatment of myasthenia gravis

ABSTRACT

Introduction

Acetylcholine receptor antibody-positive generalized myasthenia gravis (gMG) is effectively treated with symptomatic and immunosuppressive drugs but a proportion of patients has a persistent disease and severe adverse events (AEs). The unmet medical needs are specific immunosuppression and AE lowering. Eculizumab blocks C5 protecting neuromuscular junction from the destructive autoantibody effects. Phase II (Study C08-001) and III (ECU-MG-301) studies, with the open-label extension (ECU-MG-302), demonstrated eculizumab efficacy and safety in refractory gMG patients.     

免疫调节疗法治疗重症肌无力临床观察

目的观察应用静脉注射用免疫球蛋白（IVIg）或血浆置换治疗全身型重症肌无力（MG）的临床疗效和安全性。方法对56例病情恶化需IVIg或血浆置换治疗的全身型重症肌无力患者分两组,27例应用IVIg治疗,29例应用血浆置换治疗。在接受治疗后14天、1个月、3个月分别进行疗效评估。结果两组患者治疗前后评分差异均有统计学意义,并且都能很好地耐受,在治疗初期两种方案疗效相差不大,有效率分别为77．8％（21／27）、75．9％（22／29）。好转后都可维持较长时间,但IVIg维持时间稍长于血浆置换。结论重症肌无力患者中IVIg的治疗效果可与血浆置换匹敌。IVIg在3个月后的评估中疗效优于血浆置换。但在病情恶化初期,患者可根据所在医院的医疗条件选择任一种方案。     

Laparoscopic assisted colectomy and myasthenia gravis

Summary

Myasthenia gravis is an auto-immune disorder posing particular challenges in the anaesthesia of patients requiring laparotomy. This report describes how a laparoscopic approach may overcome these difficulties.     

Long-term treatment of myasthenia gravis with immunoadsorption

Acute treatment of myasthenic crisis with immunoadsorption (IA) or plasma exchange is well established. The efficiency of chronic apheresis therapy in myasthenia gravis (MG), however, and its efficacy in reducing concomitant potentially harmful immunosuppressive therapy, is unknown. We treated 13 patients with therapy-resistant MG or severe steroid or azathioprine therapy-related side effects, or both, with long-term IA [median, 38 (range: 16-59) months]. IA was performed every second day until partial remission was achieved (modified Osserman score <2). Subsequently, oral immunosuppressive therapy was reduced and the frequency of IA adapted to the clinical symptoms. After initiation of IA the mean (SEM) Osserman score decreased from 3.23 +/- 0.12 to 1.23 +/- 0.08 within 1 month (P < 0.01). Mean azathioprine dose was reduced concomitantly from 89 +/- 9.4 mg/day to 56 +/- 11 mg/day (P < 0.05), and mean prednisolone dose from 41 +/- 7.6 mg/day to 22 +/- 8.5 mg/day (P < 0.05). After 36 months the number of IA-sessions/month had been reduced from 4.81 +/- 0.24 to 2.64 +/- 0.4 (P < 0.05), the mean azathioprine dose to 25 +/- 17 mg/day and the mean prednisolone dose to 9 +/- 3.6 mg/day. Six out of thirteen patients were weaned from IA after a median of 33 (range, 16-50) months and a decrease of the Osserman score to 0.33 +/- 0.33. In these patients MG remained stable during a follow-up period of 28 (range, 16-38) months. We conclude that long-term IA enables the reduction of oral immunosuppressants in patients with contraindications or resistance to steroid and azathioprine therapy. Furthermore, almost 50% of the patients can be weaned from IA with then substantial lower need of further immunosuppressive therapy.     

Case of the month: Unusual presentation of myasthenia gravis with acute respiratory failure in the emergency room

A 21 year old woman with no past medical history presented to the emergency room (ER) with signs and symptoms of sepsis and subsequently went into acute respiratory failure. She was found to have myasthenia gravis which was exacerbated by the infection. This report highlights the need to consider myasthenia gravis in the differential diagnosis of an otherwise unexplained respiratory failure in the critical care setting.     

胸腺瘤伴重症肌无力患者术后肌无力危象的临床分析

目的：探讨胸腺瘤伴重症肌无力（MG）患者行胸腺瘤切除术后,发生肌尢力危象的影响因素及治疗措施。方法：48例胸腺瘤伴MG患者行扩大胸腺切除术,术后是否发生肌无力危象分组。分析肌无力危象与性别、年龄、病史、Osserman分型、胸腺瘤病理类型、Masaoka病理分期及术前用药关系,建立人工气道与机械通气治疗肌无力危象疗效。结果：18例患者于术后（1．69±1．62）d发生肌无力危象,肌无力危象与年龄、性别、Osser—msn分型、病理类型、Masaoka分期及术前用药无关。9例气管切开、6例鼻腔气管插管、3例口腔气管插管．机械通气（12．0±13．88）d,肌无力危象组住院时间明显延长（34．61±23．90d vs 20．33±15．26d,P〈0．05）,10例给予大剂量激素冲击治疗,其中1例发生双侧股骨头坏死,无一例死亡。结论：胸腺瘤伴MG患者术后肌无力危象的发生与胸腺瘤关系不密切,迅速建立人工气道与合理的机械通气策略是救治肌无力危象的关键。     

胸腺瘤病理与重症肌无力关系的临床研究

目的进行胸腺瘤分类标准的临床研究,以探讨胸腺瘤最新 WHO病理分型与重症肌无力( MG)发生率、 Osserman分型及手术预后的关系.方法回顾分析 1974/2000 105例因胸腺瘤行胸腺切除的患者,分别应用胸腺瘤的传统病理分类法、 Levine Rosai分类法及最新 WHO分型标准对胸腺瘤分类,并在 MG发生率、 Osserman分型及手术预后等方面统计比较.结果 ( 1) A型+ AB型良性例数较多, B型恶性例数较多,体现出 A型及 AB型胸腺瘤良性的特点.( 2) B3型较 A型及 AB型易合并 MG(χ 2 = 3.294 8, P= 0.07), C型 13例均未合并 MG. B3型胸腺瘤合并 MG的手术危象发生率比 A型+ AB型、 B1+ B2型高,但统计学上无显著差异.( 3)手术危象与 Masaoka分期的良、恶性程度明显相关(χ 2= 4.218 8, P= 0.04) ,主要集中在 Osserman改良分型Ⅱ b型及Ⅲ型(χ 2= 13.099 4, P< 0.001).结论胸腺瘤最新 WHO病理分型对于区别良恶性肿瘤有指导意义;不同类型的胸腺瘤 MG易患性不同,并且结合 Osserman临床分型、 Masaoka病理分期对提示术后危象有一定的应用价值.     

The place of the ice pack test in the diagnosis of myasthenia gravis

In the evaluation of ptosis, the ice pack test is a simple, quick, bedside method to ascertain whether it is of myasthenic origin, which requires no specialised medication or equipment and is free of reported adverse effects. Pooling of published studies suggests very high sensitivity, specificity and likelihood ratios, but standardisation and operationalisation of the test are important. False-negative tests may occasionally occur.     

Maintenance plasma exchange treatment for muscle specific kinase antibody positive myasthenia gravis patients

Background:Anti‐muscle specific kinase antibody positive myasthenia gravis (MuSK MG) is often characterized by a relatively severe and progressive course, refractoriness to standard myasthenia gravis (MG) medications, and an increased risk of myasthenic crisis. We report here successful management of three MuSK MG patients using maintenance therapeutic plasma exchange (TPE) treatment for up to 4.5 years. Materials: The study was a 5‐year retrospective review of all MG patients treated with TPE between 2008 and 2013 at University of Michigan. Inclusion criteria of MuSK MG were positive for anti‐MuSK antibodies and a diagnosis of MuSK MG by staff neurologists. Patient data included age, gender, diagnostic testing results, medications, and the dates and response to TPE treatments. Results: A total of 153 MG patients underwent at least one course of TPE between 2008 and 2013. A total of 12 patients (7.8%) were positive for anti‐MuSK antibodies. Patients were predominantly female (83.3%) and a median age of onset was 46‐years old. Three MuSK MG patients were successfully managed with maintenance TPE. Conclusion: Maintenance TPE may be an effective option for MuSK MG patients. The key of successful maintenance treatment at our institution has been to tailor the TPE frequency for each individual, and to modify the treatment interval in conjunction with medical management. J. Clin. Apheresis 30:314–319, 2015. © 2014 Wiley Periodicals, Inc.     

重症肌无力重复神经刺激的研究

INTRODUCTIONRNSisthefirst－choiceelectrophysiologicalexaminationtodiagnosemyastheniagravis，inwhichdiagnosispositiverateoflow－frequentRNStoMGishighest．Nowmanylabsadoptlow－frequentRNASamplitudeofwaveattenuationvalueuniversalstandard：＞１０％or１５％．Busomeresearchesthoughtdifferentstimulationfrequencyanddifferenmuscleshaddifferentamplitudeofwaveattenuationvalue．MATERIALSANDMETHODSMaterialsMGgroup：２７caseswerediagnosedaccordingtothethirdedition－Neurology，including：ty…     

中西医结合救治10例重症肌无力危象分析

目的：分析中西医结合治疗重症肌无力危象的疗效。方法：１０例重症肌无力危象患者及时经鼻气管插管、呼吸机辅助正压通气，意识不清者，采用控制通气呼吸模式；意识清楚者，采用同步间隙指令呼吸（ＳＩＭＶ）加压力支持通气（ＰＳＶ）。并应用大剂量类固醇激素，对有激素禁忌证者静滴大剂量丙种球蛋白，对感染触发危象或危象后伴发感染者，积极选用抗生素，同时中药治以回阳救逆、扶正纳气、肃肺化痰治疗；同时进行了乙酰胆碱受体抗体、肺功能、食管运动功能等项检测。结果：抢救成功７例，死亡３例，危象病死率３０％。８例检测乙酰胆碱受体抗体平均为４．５２±１．６８，与同期无危象的重症肌无力组（１．１８±１．０２）比较有显著性差异（ｔ＝８．２，Ｐ＜０．０１）。肺功能检测：１０例患者均提示有中至重度肺容量受限。食管运动功能检测９例中８例异常。结论：及时建立呼吸通道给予呼吸机控制呼吸是保证抢救成功的首要步骤；充分关注和及时处置感染和心脏并发症；肌无力危象病死率的高低与危象救治是否及时、并发症的严重程度、呼吸机的掌握与合理使用、激素及其它免疫调节剂的应用以及综合措施的得力与否有关。合理应用中西医结合方法多途径救治重症肌无力危象效果较好     

胸腔镜胸腺扩大切除术治疗重症肌无力的临床疗效及影响因素研究

目的评估胸腔镜胸腺扩大切除术治疗重症肌无力(MG)的疗效,并分析影响手术疗效的因素。方法回顾性分析2011年1月-2016年5月该院收治的行胸腔镜胸腺扩大切除术的183例MG患者的临床资料,采用美国重症肌无力联盟(MGFA)标准评价手术疗效。采用单因素分析和Cox比例风险模型分析影响手术疗效的因素。结果 183例患者中,173例患者完成随访,失访10例,随访率94.5%。其中完全稳定缓解(CSR)率为66.5%、药物缓解(PR)率为7.5%、微小症状表现(MM)率为1.2%、改善(I)率为1.2%、无变化(U)率为11.6%、复发(E)率为4.6%、死亡(D)率为1.7%。进一步分析显示年龄(RR=1.53,P=0.031)、病理类型(RR=5.84,P=0.022)、MGFA分型(RR=3.72,P=0.028)是影响手术疗效的因素。结论胸腔镜扩大切除术治疗MG患者疗效显著,且年龄、病理类型及MGFA分型是影响手术疗效的主要因素。     

抗AMPAR脑炎合并重症肌无力1例并文献复习

本文描述了1例以认知障碍1周就诊的抗AMPAR脑炎伴重症肌无力患者并进行文献复习。值得临床医生思考的是,自身免疫性脑炎、重症肌无力伴胸腺瘤之间的发病机制是否具有关联性或相似性,目前还不清楚,通过本文论述希望能够提高临床医生对该病的重视及认识,以免造成漏诊。     

Prethymectomy plasmapheresis in myasthenia gravis

Plasma exchange before thymectomy may decrease the time on mechanical ventilation (MV) and shorten the stay in the intensive care unit (ICU) for patients with myasthenia gravis (MG). This study evaluated the effects of prethymectomy plasmapheresis. A total of 29 myasthenic patients, 18 women and 11 men aged 20-73 years, were treated with double filtration plasmapheresis (DFP) for two to five consecutive sessions over a period between 2 and 21 days (mean 8.1 days) before transsternal thymectomy. Acetylcholine receptor antibody (AchRAb) titers, vital capacity (VC), maximal inspiratory pressure (Pimax), and MG score were measured before and after the course of DFP. Three outcome measures including duration of postoperative hospital stay, duration of ICU stay, and duration of MV were analyzed for correlation with clinical variables. The duration of MV ranged from 6 to 93 h, with a median of 21 h. The median ICU stay was one day and the median postoperative hospital stay was 10 days. A higher removal rate of AchRAb was associated with a shorter duration of ICU and postoperative hospital stay (P = 0.001 and 0.019, respectively). Postoperative hospital stay was strongly correlated with post-DFP Pimax (P = 0.010), and marginally correlated with pre-DFP VC (P = 0.047) and to a lesser extent with pre-DFP Pimax (P = 0.063). Univariate analysis using the log rank test revealed that removal rate of AchRAb <30% (P = 0.043) and pre-DFP Pimax <-60 cmH2O (P = 0.024) were significantly associated with prolonged ICU stay. Risk factors for prolonged postoperative stay included post-DFP Pimax <-60 cmH2O (P = 0.017), pre-DFP Pimax <-60 cmH2O (P = 0.031), and post-DFP VC < 1.0 L (P = 0.046). Our results confirmed the efficacy and safety of DFP in prethymectomy preparation for myasthenic patients.     

重症肌无力病人胸腺手术围手术期的呼吸道管理

对40例重症肌无力病人切除胸腺组织或胸腺瘤治疗过程的围手术期呼吸道管理。术前进行必要的呼吸道准备，术中吸除气道分泌物，预防性应用抗生素。术后应动态观察血气指标，痰液性状及肌无力的各项指标，有效吸痰。使用呼吸机是肌无力危象和呼吸衰竭治疗的关键措施，须掌握时机，正确应用。     

血浆置换在重症肌无力患者实施胸腺切除术前的应用与护理

目的探讨血浆置换在重症肌无力患者实施胸腺切除术中的应用。方法回顾性分析22例重症肌无力患者实施胸腺切除术前应用血浆置换的病例,总结护理要点。结果全部患者经血浆置换治疗后,肌无力症状明显好转,相关指标达到手术所需指征。结论重症肌无力患者实施胸腺切除术前应用血浆置换可及时改善患者的临床症状,使手术治疗更为安全有效,血浆置换过程中护理措施得当,可保证血浆置换治疗顺利进行。     

Fremanezumab plus plasmapheresis in a patient with chronic migraine and myasthenia gravis: Case report of an effective treatment

A patient with chronic migraine and generalized myasthenia gravis was concurrently treated with fremanezumab and with therapeutic plasmapheresis (PEX). Fremanezumab was dosed right after a PEX session, or in the midpoint between sessions, and the efficacy of both treatments was maintained. This case broadens the drug's clinical applications and it helps in choosing the appropriate medical regimen in patients requiring both treatments.     

血浆交换丙种球蛋白甲基强的松龙治疗重症肌无力

目的探讨依次采用少量血浆交换、大剂量丙种球蛋白、大剂量甲基强的松龙治疗重症肌无力 (MG)Ⅱ b或Ⅲ型的临床疗效及安全性.方法对符合 Osserman 分型为Ⅱ b或Ⅲ型的 37例 MG患者,按住院先后顺序随机分成两组.治疗组 18例,依次采用少量血浆交换、大剂量丙种球蛋白、大剂量甲基强的松龙治疗.对照组 19例,采用传统的大剂量地塞米松治疗.观察两组近期的临床疗效及安全性.结果治疗组症状缓解快,激素治疗过程中发生呼吸肌瘫痪、使用呼吸机现象少,住院时间短,死亡率低 (均 p<0.05).结论依次采用少量血浆交换、大剂量丙种球蛋白、大剂量甲基强的松龙治疗Ⅱ b或Ⅲ型 MG效果好,安全性高,值得临床推广应用.     

儿童眼肌型重症肌无力胸腺MRI表现

目的:探讨儿童眼肌型重症肌无力(ocular-muscular type myasthenia gravis,o-MG)的胸腺变化特点。方法:对正在接受治疗的儿童o-MG患者60例(男32例,女28例,年龄3~14岁),进行胸腺MRI扫描,其中15例(男7例,女8例)行增强扫描,对照组为相同年龄段无重症肌无力?穴n-MG?雪30例(男13例,女17例),分析两组胸腺影像学变化。结果:①胸腺增大:o-MG组44例(73.33%),对照组0例。②胸腺脂肪变:o-MG组11例(18.33%),对照组4例(13.33%)。③胸腺占位性病灶:o-MG组1例(1.66%),对照组0例。两组对比具有明显差异。④o-MG组胸腺增大程度以及胸腺结节灶与临床症状的轻重无明显相关性(P>0.05)。结论:胸腺增大是儿童o-MG患者常见和主要的MRI征象;胸腺肿瘤与胸腺脂肪变罕见。MRI检查能显示胸腺变化的相关信息。