眼肌型重症肌无力治疗进展共39页

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《重症肌无力》PPT课件

病理机制研究
神经肌肉接头处的传导障碍
重症肌无力患者神经肌肉接头处的突触后膜结构异常，导致乙酰胆碱受体数量减少或功能异常，从而影响神经信号的传递。
免疫系统异常
免疫系统异常攻击突触后膜上的乙酰胆碱受体，导致神经肌肉接头处的传导障碍。
神经肌肉接头处的病理改变
重症肌无力患者神经肌肉接头处的超微结构发生改变，包括突触后膜的皱褶、增厚、细胞内肌醇磷脂的减少等。
《重症肌无力》ppt 课件
目录
• 重症肌无力概述 • 重症肌无力的病因与病理机制 • 重症肌无力的治疗与康复 • 重症肌无力的预防与日常护理 • 重症肌无力研究进展与未来展望
01
CATALOGUE
重症肌无力概述
定义与特点
定义
重症肌无力是一种由神经-肌肉接头处传递功能障碍所引起的自身免疫性疾病，临床主要表现为部分或全身骨骼肌无力和易疲劳，活动后症状加重，经休息后症状减轻。
合理休息
保证充足的睡眠时间，适当休息，避免长时间连续工作或学习。
适当锻炼
进行适当的体育锻炼，增强肌肉力量和耐力。
注意安全
在日常生活中注意安全，避免因肌肉无力而发生意外事故。
心理支持与健康生活方式
01
02
03
04
心理支持
对于患有重症肌无力的人群，应给予心理支持，帮助他们树
立信心，积极面对疾病。
特点
重症肌无力是一种慢性疾病，病程较长，病情容易反复发作，需要长期治疗和管理。
重症肌无力的症状与表现
症状
常见的症状包括眼睑下垂、复视、斜视、眼球运动障碍、面部肌肉无力、咀嚼吞咽困难、声音嘶哑、饮水呛咳、呼吸困难等。
表现
重症肌无力的症状表现多样，不同患者的症状可能有所不同，有些患者可能仅表现出轻微的症状，而有些患者可能出现严重的肌肉无力甚至呼吸衰竭。

重症肌无力课件PPT

康复训练
肌肉力量训练
针对患者具体情况制定训练计划，逐步加强肌肉力量。
日常生活能力训练
指导患者进行日常生活能力训练，提高生活质量。
语言康复训练
针对患者语言障碍进行康复训练，提高语言表达能力。
心理康复训练
关注患者心理健康，提供心理支持，帮助患者树立信心。
心理支持
建立信任关系
与患者建立良好的信任关系，倾听患者诉，
康复治疗
对患者进行康复训练和心理辅导，提高其生活质量和社会适应能力。
药物治疗
根据患者的具体情况，制定个性化的药物治疗方案，控制病情发展。
监测与评估
定期对患者的病情进行监测和评估，及时调整治疗方案，控制病情进展。
公共卫生视角下的重症肌无力
疾病负担
监测与报告
了解重症肌无力对公众健康造成的负担，制定相应的防控策略。
重症肌无力的治疗
药物治疗
01
02
03
胆碱酯酶抑制剂
如溴吡啶斯明，可抑制胆碱酯酶活性，缓解症状。
免疫抑制剂
如糖皮质激素、环磷酰胺等，用于控制疾病进展和减轻症状。
免疫调节剂
如免疫球蛋白、胸腺素等，用于调节免疫功能，控制疾病。
手术治疗
胸腺切除术
适用于伴有胸腺增生的患者，可减轻症状并降低复发率。
症状
常见症状包括眼睑下垂、视力模糊、复视、斜视等，严重时可能导致吞咽困难、呼吸困难等症状。
病因与病理机制
病因
重症肌无力的具体病因尚未完全明确，但多数研究认为与自身免疫、遗传、环境因素等有关。
病理机制
重症肌无力的病理机制主要是由于神经-肌肉接头处的突触后膜受损，导致乙酰胆碱受体数量减少或功能异常，从而影响神经信号的传递，引起肌肉无力。

眼肌型重症肌无力治疗进展(指南截取)

胸腺摘除手术治疗
• 确诊胸腺瘤患者应行胸腺摘除手术，而不考虑重症肌无力的严重程度，早期手术治疗可以降低肿瘤扩散的风险 • 不伴胸腺瘤MG患者，轻型者（Osserman 分型Ⅰ-Ⅱ）不能从手术中获益，而相对较重患者（Osserman分型Ⅱb-Ⅳ）特别是全身型合并乙酰胆碱受体抗体阳性的重症肌无力患者则可能在手术后临床症状得到改善或缓解
分型
MGFA分型 • 2000年美国重症肌无力协会（MGFA）提出新临床分型，比Osserrman分型更细致客观
分型
临床表现
I型
II型 IIa IIb III型 IIIa IIIb IV型 IVa IVb IV型
任何眼肌无力，可伴有眼闭合无力，其他肌群力正常
无论眼肌无力的程度，其他肌群轻度无力主要累及四肢或(和)躯干肌，可有同等程度以下的咽喉肌受累主要累及咽喉肌(和)呼吸肌，可有同等程度以下的四肢肌或（和）躯干肌受累无论眼肌无力的程度，其他肌群中等无力主要累及四肢肌和躯干肌，可有同等程度以下咽喉肌受累主要累及咽喉肌或（和）呼吸肌，可有同等程度以下的四肢肌或（和）躯干肌受累无论眼肌无力的程度，其他肌群重度无力主要累及四肢肌或（和）躯干肌，可有同等程度以下的咽喉肌受累主要累及咽喉肌或（和）呼吸肌，可有同等程度以下的四肢肌或（和）躯干肌受累气管插管伴或不伴机械通气（除外术后常规使用），无插管的鼻饲病例为IVb期。
眼肌型鉴别诊断
脑干病变
• 如滑车、外展和部分动眼神经麻痹、核间性眼肌麻痹、一个半综合征等 • 眼外肌麻痹，可伴有相应的中枢神经系统症状和体征 • 脑干诱发电位可有异常 • 头颅MRI检查有助于诊断
眼肌型鉴别诊断
Graves眼病
• 属于自身免疫性甲状腺病 • 限制性眼外肌无力不伴眼睑下垂，可有眼睑退缩症状 • 眼眶CT显示眼外肌肿胀，甲状腺功能亢进或减退

重症肌无力危象的治疗PPT课件

等）； ②停用诱发加重的药物（如洁霉素）； ③减少激素剂量。
3.γ球蛋白：蓉生静丙 0.2-0.4/Kg/d 静注，连用5天
4.血浆交换：每次2000ml，qod，3-4 次
5.换血疗法：放血300-400ml，输血 300-400ml，或换血浆疗法
通过以上5项措施，力争免作气管插管或气管切开
重症肌无力危象的治疗
定义
MG患者在患病过程中突然发生的严重的呼吸困难
分型
1.肌无力危象 2.胆碱能危象 3.反拗性危象
诱因
1.感染 2.月经 3.生产 4.劳累
5.药物 6.手术 7.外伤 8.精神刺激
治疗
1.吸氧、调节抗AchE的剂量和方法 2.去除诱因： ①尽内滴入 11.拔管后继续用激素，化疗或放
疗、手术。
• 6.气管插管、气管切开、使用人工
• 呼吸器
• 指征：
• ①严重的呼吸困难；
• ②肌注新斯的明无改善；
• ③血氧分析PO2<50mmHg，
•
PCO2>50mmHg，PH<7.25
• 7.暂停抗AchE剂3-4天
8.甲强龙1000mg，静注，qd×3 9.CTX 1000mg，静注，qw 10.用最敏感的抗生素迅速控制感

眼肌型重症肌无力临床特点及误诊分析

诊断吉兰一雷综合征要仔细了解患者发病过程，床巴临表现的多样性以复杂性，好全面体格检查、关影像检查做相和实验室检查，合临床特征，结病情呈进行性发展，侧症状双相对称，觉障碍的存在，神经受累表现，情逐步得到恢感脑病复，自主神经功能障碍存在等。腰穿脑脊液蛋白一细胞分有离有助于诊断，要反复查看，要早期做Ｆ波和多根神经且还检查，这样可以降低吉兰一巴雷综合征的误诊率。
眼肌型重症肌无力的常见症状是上睑下垂、肌麻痹和眼复视。这些症状可单独或同时出现。而这些症状亦是某些颅内神经病变、瘤和肌源性病变的共有表现。如果对眼肌肿
压史，药血压控制良好，糖尿病史。体检：身情况良服无全好。视力Ｒ０８Ｉ０６．，．。第一眼位：眼内斜约＋１。左眼左５，外转轻度受限，余各方位活动自如。眼眶Ｃ无异常，其Ｔ颅脑Ｃ腔隙性脑梗死。注射新斯的明后复视消失，断为重Ｔ：诊
某医院诊为“ 甲状腺功能低下 ” 口服甲状腺素片，状无改，症善。因左眼角膜畸形突出、痛，我院要求手术治疗。入疼来

重症肌无力诊疗现状进展荟萃(2024年)

一项来自印度的开放标签试验比较了IVIG（400mg/kg/天，共5天）和膜型PLEX（40mL/kg/隔日，共5个疗程）在MG危象（MC）中的疗效。该研究中17例患者共发生了44次MC，其中有15次使用了IVIG，有8次使用了PLEX，感染是导致MC的最常见原因（61.4%）。IVIG组患者的平均年龄高于PLEX（P=0.02），其他基线特征基本一致。结果显示，IVIG组所需机械通气时间较PLEX组更长（17.47±11.32天 vs 7.38±7.40天；P=0.018），但两组住院时间和达到恶化前状态的时间无显著差异。在不良反应方面，IVIG组出现过敏反应的概率为6.7%，出现肌酐升高的概率为13.3%，其中1次治疗时患者需停止治疗。在PLEX治疗过程中出现短暂的血压下降的概率为50%，但不需要停止治疗。该研究表明，与IVIG相比，膜型血浆置换可缩短MC的机械通气时间，在血浆置换过程中血压监测非常重要。
ME＆MG是一项在美国、加拿大开展的开放分散研究，其中纳入了199名全身型重症肌无力（gMG）患者，均使用ME&MG openTM数字工具完成每月的问卷调查。本次EAN大会上公布了基于该研究的电子患者报告结果（EPROS）和潜在影响因素描述的gMG给患者造成的影响。199名参与者中有65%为女性，平均年龄57±16岁。结果显示，在所有参与者中，PHQ-8（6.5±5.1）、ISI （9.0±6.0）、慢性疼痛（2.5±2.4）、MG-ADL（5.2±3.7）、MG QOL-15R（12.0±8.0）基线评分可表明gMG对患者生活有显著影响。当疾病控制不充分时，PHQ-8（9.8±4.0，P<0.05）、MGADL（10.6±3.2，P<0.0001）、MG-QOL-15R（21.0±6.0，P<0.01）评分显著高于对照组。与工作或失业患者相比，不能工作患者的PHQ-8（10.0±5.0，P<0.05）和MG-QOL-15R（20.0±5.7， P<0.001）评分显著增加。这与此前报道保持一致，未来将继续通过纵向分析确证ME&MG openTM数字工具收集EPROS的可靠性。

眼肌型重症肌无力研究进展及临床问题

临床类型，我国Ｏ在ＭＧ发病率较高。ＯＭＧ患者表现的眼外肌无力（如上睑下垂）以直观地显示可ＭＧ患者的表征特点，以及表现为晨轻暮重或活动后加重与休息后缓解的病态疲劳特征。与延髓肌
或呼吸肌受累的ＭＧ患者相比，ＯＭＧ的诊断更为
在重症肌无力（ＭＧ）者中眼肌型重症肌无患
力（ｃｌｒｍｙｓｈｎａｇａｉ，ＯＭＧ）最常见的ｏｕａａｔｅｉｒｖｓ是
２￣４岁，性占优势，二高峰为６～８００女第００岁，男
女发病几率均等。（）纯眼外肌受累多见：５０３单１２病例中Ｉ型为主占６．，Ｉ７１Ｉａ型占９３，Ｉｂ．１
高峰。这与国内报道接近，明我国以儿童期发病表
２ＯＭＧ的临床表现
ＯＭＧ患者发生频率最高的症状为上眼睑下垂和复视。很多患者会一直表现为上眼睑下垂和复视，度的上眼睑下垂往往会被忽视，一旦发轻而
女和老年男性的疾病 ” 临床证据可能来源于此。的
１～５，８９在全身型ＭＧ中８～９伴有眼５０肌受累。分析作者单位１８ —３０６０９７０ —２０１收治的１２５０例ＭＧ患者的资料，有如下特点：１性别具（）差异较小：１２在５０例患者中男：一１：．８与女１１，

陈氏经验方治疗眼肌型重症肌无力的临床观察

陈氏经验方治疗眼肌型重症肌无力的临床观察邹月兰;陈济东;金慧莉【摘要】目的：观察陈氏经验方对眼肌型重症肌无力（OMG）患者的疗效。

方法：收集2012年12月至2015年1月在上海市眼病防治中心诊断为OMG，且辨证为脾肾阴虚证的患者60例，随机数字表法分为治疗组和对照组，对照组采用常规西医治疗，在此基础上，治疗组采用口服陈氏经验方汤剂，疗程3个月。

对比两组临床疗效和治疗前后上睑肌力、上睑疲劳试验、眼球水平活动度。

结果：治疗组治疗总有效率为90．00％，对照组为63．33％，两组比较差异有统计学意义（P ＜0．05）；两组治疗后上睑肌力、上睑疲劳试验、眼球水平活动度均较治疗前有明显改善，且治疗组改善程度优于对照组，差异均有统计学意义（P＜0．05）。

结论：陈氏经验方对脾肾阴虚证OMG患者有效，能明显改善眼部症状，值得临床借鉴。

【期刊名称】《陕西中医》【年(卷),期】2016(037)004【总页数】2页(P461-462)【关键词】肌无力/中西医结合疗法;眼;补气剂/治疗应用;陈氏经验方【作者】邹月兰;陈济东;金慧莉【作者单位】上海市眼病防治中心上海200040;上海市眼病防治中心上海200040;上海市眼病防治中心上海200040【正文语种】中文【中图分类】R77.45主题词肌无力/中西医结合疗法眼补气剂/治疗应用@陈氏经验方重症肌无力(MG)是一种获得性自身免疫性疾病，主要由神经-肌肉接头间传递功能障碍造成。

其中I型眼肌型重症肌无力(OMG)是最常见的类型[1]。

药物治疗是OMG的主要治疗手段，常用的药物包括皮质类固醇激素、胆碱酯酶抑制剂和免疫抑制剂等，但副作用大。

目前多种中医药方注重整体论治，疗效肯定，深受病员欢迎。

陈氏经验方作为名老中医陈贯一摸索自创的治疗OMG中医药方，其疗效有待研究。

现收集上海市眼病防治中2012年12月至2015年1月OMG患者60例，观察其疗效，总结如下。

临床资料病例均来源于在上海市眼病防治中心就诊的OMG患者，经反复发生的肌无力、疲劳试验阳性、新斯的明试验阳性等确诊为OMG，辨证为脾肾阴虚证。

眼肌型重症肌无力的研究进展

合当归养血汤加减；脾肾气阴两虚型方用左归丸加减。刘小斌师从邓铁涛教授，主张以健脾益气为治疗大法，以补中益气汤加减为该病基本方［１。３．３专方治疗邓铁涛认为在抓住脾脏虚损的同时，应该注意
（ＯＭＧ），以后逐渐进展为全身型ＭＧ（ＧＭＧ），危重时可出现呼
吸肌麻痹、无力，发生肌无力危象而死亡［１］。目前，临床多采用改良的Ｏｓｓｅｒｍａｎ分型，将ＭＧ分为５型：其中Ｉ型只有眼部的
兼顾其他脏器，多元调治，研制出强肌健力饮ＬＩ服液及胶囊（黄芪、五爪龙、党参、白术、当归、升麻、柴胡、陈皮、甘草），方中重用黄芪６Ｏｇ～１２０ｇ［１。吴以岭从奇经理论研制出治疗本病的重肌灵散（由人参、鹿茸、黄芪、淫羊藿、巴戟天、白术、陈皮、茯苓等组成）在临床应用中取得了良好的疗效＿１。陈金亮等Ｅ ” 通过临床试验，认为参茸强力散（由鹿茸、人参、淫羊藿、马钱子、麻
眼肌型重症肌无力的研究进展
Ｂｕｌｉｎ—ＳｏｋｏｌｏｖａＥｋａｔｅｒｉｎａ，项宝玉
摘要：综述近十年眼肌型重症肌无力的中西医临床研究进展。该病的发病机制目前尚不完全清楚。治疗目标是缓解症状，预防复发，防止其转变为全身型。虽然治疗方法多样，但是药物的副作用很大，手术的方法也存在一些争议。中医虽无眼肌型重症肌无力病名，但根据其，床特点及中医理论知识，在对该病的病因病机及其治疗认识和临床实践等方面都取得了一定的进展。各种中医传统疗法对于本病的治疗积累了丰富的经验并取得了较好的疗效。关键词：重症肌无力；眼肌型；中西医；临床研究；研究进展中图分类号：Ｒ７４６Ｒ２５５文献标识码：Ａｄｏｉ：１０．３９６９／ｊ．ｉｓｓｒｕ１６７２ — １３４９．２０１４．０１．０５６文章编号：１６７２ —１３４９（２０１４）０１ —０１０６ — ０２

裴正学教授治疗眼肌型重症肌无力经验探析

裴正学教授治疗眼肌型重症肌无力经验探析高拴生;朱春晖;李炜;王彩琴;郑修丽;葛斌;陈光燕;王鑫;赵孝鹏【摘要】裴正学教授以“西医诊断,中医辨证,中药为主,西药为辅”十六字方针为原则,采用中医药辨证论治的方法,治疗眼肌型重症肌无力取得较好的疗效.在治疗过程中以“裴氏振痿汤”合“兰州方”为主分四型辨证论治,配合消风二号,加减化裁,目的在于扶正祛邪.该治疗方法对临床治疗重症肌无力等相关自身免疫性疾患有很好的启发.【期刊名称】《甘肃医药》【年(卷),期】2014(033)003【总页数】3页(P219-221)【关键词】眼肌型重症肌无力;中医药疗法;经验;裴正学【作者】高拴生;朱春晖;李炜;王彩琴;郑修丽;葛斌;陈光燕;王鑫;赵孝鹏【作者单位】730000 甘肃兰州,甘肃省人民医院药剂科;730000 甘肃兰州,甘肃省人民医院药剂科;730000 甘肃兰州,甘肃省人民医院药剂科;730000 甘肃兰州,甘肃省人民医院药剂科;730000 甘肃兰州,甘肃省人民医院药剂科;730000 甘肃兰州,甘肃省人民医院药剂科;730000 甘肃兰州,甘肃中医学院中西医结合系;730000 甘肃兰州,甘肃中医学院中西医结合系;730000 甘肃兰州,甘肃中医学院中西医结合系【正文语种】中文裴正学教授系我国著名中西医结合专家，主任医师，博士研究生导师，国家级高徒导师，从事临床医学教研五十余年，提出“西医诊断，中医辨证，中药为主，西药为辅”中西医结合的新思维指导临床。

在许多中西医都棘手的疑难杂症中，采取独特裴氏疗法均取得了明显疗效，现将裴正学教授治疗眼肌型重症肌无力症经验总结于下。

重症肌无力是一种累及神经肌肉接头突触后膜上乙酰胆碱受体的自身免疫性疾病。

目前神经肌肉病发病率较高且较难根治的疾病之一[1]。

眼肌型重症肌无力（ocularmyastheniagravls，oMG）是临床症状最轻，发病最早的重症肌力。

其症状仅表现眼外肌症状，而无其他肌群受累的临床表现和电生理所见。

重症肌无力眼肌型会加重吗,治疗方法

重症肌无力眼肌型会加重吗，治疗方法重症肌无力（Myasthenia gravis，MG）是一种罕见的神经肌肉疾病，该疾病主要表现为肌肉易疲劳、无力等症状，这种疾病不仅影响身体的日常活动，而且可能影响眼部健康。

在MG中，眼部症状是最明显、最普遍并且最可怕的。

本文将讨论重症肌无力眼肌型加重、治疗方法、注意事项等问题，以更好地了解MG病情和治疗方法。

一、重症肌无力眼肌型加重重症肌无力眼肌型（oculomotor型）属于MG的一种亚型，主要表现为双眼上睑下垂、眼球运动受限等眼部症状。

在MG患者中，眼肌症状通常是首先出现的。

重症肌无力眼肌型症状加重的原因很多，下面列举几条：1. 累积效应MG通常表现为症状短暂且间歇性的突发性发作，但是随着时间的推移，症状可能变得越来越频繁，也会变得越来越严重。

这可能是因为肌肉越来越疲劳，以至于已经无法再工作了，因此眼肌失调程度加重。

2. 触发因素MG的症状加重可能是由于机体内外的某些触发因素引起的，如生病、压力、药物、情绪等等。

3. 疾病的进展随着疾病的进展，MG可能会影响到身体的其他部位，例如喉咙、口腔、呼吸肌肉等等，进一步加重MG症状。

4. 治疗不当一些治疗方法可能会加重MG症状，特别是对某些药物过敏的患者。

二、治疗方法治疗重症肌无力眼肌型的方法主要包括药物治疗、手术治疗、康复训练等。

1. 药物治疗药物治疗是治疗重症肌无力眼肌型的主要方法。

以下是常用的药物：一生脉注射一生脉是一种抑制AChE的药物，能够提高ACh在神经末梢处的浓度，从而增强神经肌肉传递，提高骨骼肌收缩能力。

它是治疗MG的一线药物，同时也被证明对MG眼肌型治疗效果比较显著。

角膜上皮生长因子角膜上皮生长因子能够促进眼球表面上皮细胞的生长和恢复，促进角膜上皮再生。

这对于重症肌无力眼肌型患者来说非常重要，因为良好的眼睛表面充分藏有视力和眼部症状。

2. 手术治疗重症肌无力眼肌型的手术治疗主要有三种方式：眼外肌剥离术、眼外肌手术和同侧外眦内肌神经马尾草化。

眼肌型重症肌无力用药

重症肌无力重症肌无力见于任何年龄，约60%在30岁以前发病，女性多见。

发病者常伴有胸腺瘤。

颅神经支配的肌肉特别是眼外肌最易累及，常为早期或唯一症状；轻则眼球运动受累，多呈不对称性眼睑下垂，睁眼无力、斜视、复视、有时双眼睑下垂交替出现；重者双眼球固定不动。

症状通常晨轻晚重，亦可多变。

病程迁延，可自发减轻缓解。

感冒、情绪激动、过劳、月经来潮、使用麻醉、镇痛、镇静药物、分娩、手术等常使病情复发或加重。

诊断1.冰试验：将冰袋放置在两眼之间（placement of an ice pack across the eyes for 2-5mins）约2-5分钟，若是MG，睑裂可增大好转2.休息试验：需要病人闭眼2-5分钟，若是MG，睑裂可以好转改善3.睡眠试验：病人闭眼躺在安静无光线房间里30分钟，若是MG，睑裂可好转4.阿托品1mg,im20分钟后新斯的明0.02mg/kg（50kg=1mg）,im，再过20分钟观察，眼睑下垂明显改善，阳性5.血清ACHR自身抗体测定6.肌电图，特别是单纤维肌电图，需要做眼轮匝肌，口轮匝肌7.甲状腺功能全套你可以根据你那的条件来做，但诊断眼肌型MG，眼睑下垂的波动性症状最重要。

治疗：1.溴吡斯的明30-60mg tid是开始，如果证实有效且副作用最小可增加到90-120mg,每天每3-4小时应用。

并发症主要与毒蕈碱样作用，特别是腹部痉挛、恶心、呕吐、腹泻，该药对睑下垂有效，但对复视无效2.若单用溴吡斯的明无效，强的松（地塞米松0.75mg=强的松5mg=甲强龙4mg）用量为开始10-20mg/d，每3天增加5-10mg直到睑下垂和复视完全缓解或明显改善，这通常需要数周。

最大剂量为60-80mg/d。

症状缓解维持1个月后，开始以每2周减少5-10mg/d的速度缓慢减量，直到20mg/d，再减量的速度应更慢。

强的松最好是早八点一次服用。

治疗眼肌型MG的一个主要问题是激素治疗是否会阻止向全身型无力的进展。

眼肌型重症肌无力阳性严重吗,治疗方法

眼肌型重症肌无力阳性严重吗，治疗方法眼肌型重症肌无力（ocular myasthenia gravis，OMG）是一种罕见的自身免疫性疾病，主要表现为双眼下垂、模糊和重复闪烁，甚至可能导致视力丧失和生命危机。

本文将详细介绍OMG的治疗方法和注意事项。

一、治疗方法1. 激素治疗OMG的首选治疗方法是激素治疗，因为它可以通过抑制免疫系统来减少免疫系统攻击神经肌肉接头的影响。

通常，激素治疗每天均匀分几次使用口服泼尼松，起初剂量为每天1-1.5mg/kg。

治疗的目标是减轻症状并减少用药剂量。

对一些病情较轻或新诊断的OMG患者，1-2个月激素治疗后，症状可能会得到缓解甚至完全消失。

然而，激素治疗长期使用可以导致许多短期和长期副作用，比如水肿、高血压、糖尿病、骨质疏松等。

因此，医生需要跟进剂量，逐渐降低，直到最小有效剂量。

2. 免疫抑制剂免疫抑制剂用于OMG治疗，主要是通过抑制免疫系统中特定的细胞或信使来减轻免疫系统攻击。

最常用的药物是环磷酰胺和硫唑嘌呤。

免疫抑制剂的药效慢而持久，副作用也较多，需要密切观察。

3. 胆碱酯酶抑制剂胆碱酯酶抑制剂是OMG治疗的第二个选择。

这些药物通过抑制胆碱酯酶的分解，从而增加神经肌肉接头的乙酰胆碱浓度，提高神经肌肉接头的功能。

常用的药品有类胡萝卜素等。

然而，这些药物在短暂缓解症状的同时可能会导致一些不适感，如恶心、头晕和胃肠症状等。

4. 全身免疫生物治疗随着科技的进步，全身免疫生物治疗逐渐被视为治疗OMG的新方法。

这些治疗使用高科技药物，如靶向调控免疫系统的单克隆抗体和细胞因子。

临床试验显示，这些药物可以有效治疗OMG，但由于其高昂的费用和潜在的副作用，目前尚未被广泛采用。

二、注意事项1. 定期随访OMG是一种慢性疾病，需要长期维护治疗。

因此，OMG患者需要定期随访，以监测他们的症状和药物副作用，评估治疗方案的有效性，并及时纠正任何不适合的治疗方案。

2. 饮食调整OMG患者需要遵循健康的饮食习惯，以免影响药物的疗效。

2014 EFNS／ENS眼肌型重症肌无力治疗指南

EFNS/ENS GUIDELINES /CME ARTICLEEFNS/ENS Guidelines for the treatment of ocular myastheniaE.Kerty a,b *,A.Elsais a,b *,Z.Argov c ,A.Evoli d and N.E.Gilhus e,faDepartment of Neurology,Oslo University Hospital,Oslo;b Faculty of Medicine,University of Oslo,Oslo,Norway;c Department of Neu-rology,Hadassah-Hebrew University Medical Center,Jerusalem,Israel;d Institute of Neurology,Catholic University,Roma,Italy;eDepartment of Clinical Medicine,University of Bergen,Bergen;and f Department of Neurology,Haukeland University Hospital,Bergen,NorwayKeywords:myasthenia gravis,ocular myasthenia,treatmentReceived 21November 2013Accepted 17December 2013Background and purpose:The symptoms of acquired autoimmune ocular myasthe-nia are restricted to the extrinsic eye muscles,causing double vision and drooping eyelids.These guidelines are designed to provide advice about best clinical practice based on the current state of clinical and scientiﬁc knowledge and the consensus of an expert panel.Search strategy:Evidence for these guidelines was collected by searches in the MEDLINE and Cochrane databases.The task force working group reviewed evi-dence from original articles and systematic reviews.The evidence was classiﬁed (I,II,III,IV)and consensus recommendation graded (A,B or C)according to the EFNS guidance.Where there was a lack of evidence but clear consensus,good practice points are provided.Conclusions:The treatment of ocular myasthenia should initially be started with pyridostigmine (good practice point).If this is not successful in relieving symptoms,oral corticosteroids should be used on an alternate-day regimen (recommendation level C).If steroid treatment does not result in good control of the symptoms or if it is nec-essary to use high steroid doses,steroid-sparing treatment with azathioprine should be started (recommendation level C).If ocular myasthenia gravis is associated with thy-moma,thymectomy is indicated.Otherwise,the role of thymectomy in ocular myas-thenia is controversial.Steroids and thymectomy may modify the course of ocular myasthenia and prevent myasthenia gravis generalization (good practice point).ObjectivesMyasthenia gravis (MG)is an autoimmune disorder aﬀecting the postsynaptic neuromuscular junction membrane.Ocular manifestations at the onset of the disease are evident in a large majority of patients.Whilst there are accepted guidelines with regard to the treatment of generalized myasthenia gravis (GMG)[1],this is not the case for ocular myasthenia where the treatment strategy is controversial.Although there are already EFNS guidelines for treat-ment of autoimmune neuromuscular transmission dis-orders,they do not deal speciﬁcally with ocular myasthenia [1].The goal of therapy should be to minimize patients’symptoms and possibly prevent the generalization of the disease with minimal side eﬀects.BackgroundMyasthenia gravis (MG)is an acquired organospeciﬁc autoimmune disease.In most patients antibodies are directed against the nicotinic acetylcholine receptors (AChRs);in a few patients antibodies target posts-ynaptic AChR-associated proteins such as the muscle-speciﬁc tyrosine kinase (MuSK)and the low-density lipoprotein-related receptor 4(Lrp4).Despite the pro-gress in antibody detection,some patients still remain antibody negative (‘sero-negative’myasthenia).Almost all MG patients will have ocular manifesta-tions at some point during the course of their disease.Although ocular symptoms are often the ﬁrst to appear,most patients progress to GMG and onlyCorrespondence:E.Kerty,Department of Neurology,OsloUniversity Hospital,Rikshospitalet,Postbox 4950Nydalen,4Oslo,Norway (tel.:+4723071829;fax:+4723070490;e-mail:emilia.kerty@medisin.uio.no).*These two authors contributed equally to this paper.This is a Continuing Medical Education article,and can be found with corresponding questions on the Internet at /EFNS Continuing-Medical-Education-online.301.0.html.Certiﬁcates for cor-rectly answering the questions will be issued by the EFNS.©2014The Author(s)European Journal of Neurology ©2014EFNS687European Journal of Neurology 2014,21:687–693doi:10.1111/ene.1235915%continue to have isolated ocular complaints for the entire course of the disease[2].In the majority of patients,disease progression will take place within the ﬁrst year after onset and within2years in up to80% of cases[2–4].If patients have restricted ocular disease for2years without developing GMG,they are not likely to develop it later[5].When weakness is limited to the extrinsic ocular muscles and levator palpebrae superioris(LPS),the disease is called ocular myasthe-nia[6].All ages and both genders may be aﬀected. Ptosis may be unilateral or bilateral,and it is usu-ally asymmetric[7];the degree of LPS weakness var-ies from slight drop of one eyelid to nearly complete paralysis.Ophthalmoparesis is also variable and can mimic almost any pattern of ocular misalignment. Nearly all patients with diplopia have associated pto-sis[8].The pupil is not aﬀected.If ocular myasthenia is suspected on the basis of history and clinicalﬁnd-ings,the same diagnostic tests should be performed as for GMG in order to conﬁrm the diagnosis,i.e. response to the edrophonium test,the ice pack test or the rest test,antibody assays,and neurophysiolog-ical tests.40%–70%of ocular myasthenia patients have anti-AChR antibodies on the conventional assay [9,10];the use of a cell-based assay with clustered AChRs appears to signiﬁcantly increase the sensitiv-ity of anti-AChR antibody detection[11].On the other hand,high antibody levels and the presence of thymoma are uncommon in ocular myasthenia and when present are associated with an increased risk of secondary generalization[12].Only a few ocular myasthenia cases have antibodies to MuSK[13–15], whilst there are no data on the frequency of anti-Lrp4antibodies in this patient population[16–18]. Antibody detection conﬁrms the diagnosis of auto-immune myasthenia.In patients without detectable antibodies the suspicion of sero-negative MG must be conﬁrmed by the pharmacological and/or electro-myography test.Antibodies to voltage-gated potassium channel Kv1.4are relatively frequent in Japanese GMG patients and indicate a more severe disease.Such anti-bodies have recently been found in mild or predomi-nantly ocular myasthenia in a Caucasian cohort[19]. The diagnostic relevance of these antibodies is cur-rently unclear.Low-rate repetitive nerve stimulation shows low sensitivity(11%–39%)but high speciﬁcity(89%–98%)for the diagnosis of ocular myasthenia[20]. Examination of the orbicularis oculi,orbicularis oris or nasalis will increase the percentage of abnormalities identiﬁed,but these studies are not well tolerated. Single-ﬁbre electromyography,which is the most sensitive test for neuromuscular transmission,when performed in facial muscles has a high sensitivity for detecting MG in these subjects[21,22],false positives being described in ocular neuropathies and myopa-thies[23].The diagnosis of ocular myasthenia can be diﬃcult in patients with no detectable antibodies and with atypical signs(i.e.ﬂuctuations may be absent in long-standing disease).Thyroid dysfunction is a common comorbidity and endocrine orbitopathy can lead to diagnostic confusion,whilst treatment of thyroid dys-function may improve myasthenic weakness.Ocular myasthenia is usually regarded as a mild dis-ease,although it can signiﬁcantly impair patients’daily activities and can progress to GMG.Ptosis and diplopia are often disabling symptoms that require treatment.Evidence based clinical data derived from randomized,prospective,placebo-controlled clinical trials are lacking.Therapies are generally based on observational studies,case series,case reports and clinical experience.Search strategy and method for reaching consensusA literature search was performed using the reference databases MEDLINE and the Cochrane Library:the keywords used were myasthenia gravis and ocular myasthenia and these were combined with the terms treatment,medication,therapy,immunosuppression, clinical trial and thymectomy.Articles in English that contained data which could be rated according to the guidance statement for neurological management guidelines of EFNS were included[24].Scientiﬁc evi-dence for treatment was sorted into classes IÀIV and recommendations were rated at levels AÀC according to current EFNS guidance[24].When suﬃcient evi-dence for recommendations AÀC was not available, the task force oﬀered advice as‘good practice points’provided there was consensus amongst all members of the task force.Consensus was reached after several rounds of cir-culating drafts to the task force members. RecommendationsAcetylcholinesterase-inhibitor drugs Acetylcholinesterase(AChE)inhibitors improve neuro-muscular transmission by enhancing the availability of acetylcholine to the remaining AChRs.Pyridostigmine is the agent most widely used.It is usually started at 30mg three to four times per day and can be increased up to60mg four toﬁve times per day according to clinical eﬀect and the patient’s tolerance.The standard©2014The Author(s)European Journal of Neurology©2014EFNS688 E.Kerty et al.paediatric dose is7mg/kg/day[1].The maximum dose is generally determined by the occurrence of adverse eﬀects.Neostigmine is a short-acting AChE inhibitor that is usually started at15mg orally every4h or at0.5–2.5mg iv,im or sc every1–3h,not to exceed10mg/ day.The standard paediatric dose is2mg/kg/day, divided into doses administered every3–4h.The advantage of AChE inhibitors is that they are fast-acting,safe and free of long-term side eﬀects. Short-term side eﬀects are common and are caused by the increased concentration of acetylcholine at the nicotinic and muscarinic synapses.The most common muscarinic adverse eﬀects are increased secretions, abdominal cramps,diarrhoea,sweating,nausea and bradycardia;nicotinic eﬀects mostly consist of muscle fasciculations and cramps.AChE inhibitors have a better eﬀect on ptosis than on diplopia.Long-term follow-up suggests that most patients ultimately move to other treatment options [25,26].AChE inhibitors do not inﬂuence the develop-ment of generalization[12,27].There are no placebo-controlled randomized studies of AChE inhibitors.However,case series,case reports and daily clinical experience demonstrate an objective clinical eﬀect(Class IV evidence).The task force agreed that an AChE inhibitor drug should be the ﬁrst-line symptomatic treatment for ocular myasthenia (good practice point).Immune-directed treatmentCorticosteroidsCorticosteroids act on the immune system in diﬀerent ways, e.g.by modulating cytokine production[28], inducing T lymphocyte apoptosis[29]and repressing the transcription of inﬂammatory cytokines[30]. Based on several retrospective series,corticosteroids lead to signiﬁcant improvement in ocular myasthenia [12,31].The question of whether early treatment with steroids reduces the risk of generalization is contro-versial[32,33].Retrospective studies suggest a reduced rate of generalization in patients treated with corticosteroids for ocular MG[12,34,35].Some GMG patients have a temporary worsening of the disease if corticosteroids are started at high dose.Some patients may have unrecognized GMG,and therefore 10–20mg prednisolone or prednisone every other day as an initial dose is recommended,increasing by 5–10mg every5days until the symptoms improve signiﬁcantly.A lower peak dose than in GMG (50–60mg on alternate days)can often control the symptoms.After the symptoms have been resolvedÀusually after4–6weeks–a slow tapering oﬀof ste-roids can lead to a minimum eﬀective dose or to complete withdrawal[12,35].About one-third of the patients require long-term treatment[2,35–37]owing to disease relapses.Due to the potential long-term side eﬀects of corticosteroid therapy,some experts recommend its use for the treatment of ocular myas-thenia only with severe ocular disease[32].Side eﬀects from corticosteroids include obesity(including ‘moon face’),hypertension,diabetes,opportunistic infections,osteoporosis,glaucoma,cataracts, increased facial hair(women)and gastric ulcer.These side eﬀects are generally contingent on dose and treatment duration.A low carbohydrate,low sodium diet can prevent or minimize weight gain;vitamin D supplementation and bisphosphonates are useful to prevent osteoporosis.Observational studies have reported good eﬀects of oral corticosteroids in controlling ocular symptoms (Class III evidence),but the eﬃcacy has not been studied in double-blind,placebo-controlled trials.Six of the eight studies that examined the eﬀects of oral corticosteroids showed a beneﬁt in terms of reducing the risk of progression to GMG[2,12,27,38–42](Class III).AChE treatment alone usually will not solve the ocular problems and the task force agreed that oral prednisolone/prednisone should be the drug of choice when immunosuppressive treatment is necessary for ocular myasthenia(recommendation level C). AzathioprineAzathioprine is a purine analogue functioning as pur-ine antagonist and inhibits DNA synthesis and cell proliferation[43].Azathioprine is the most frequently used non-steroidal immunosuppressant for the treat-ment of MG.Treatment with azathioprine,as a ste-roid-sparing agent,should be started when steroids cannot be lowered to a safe level or at the same time as steroids are initiated in patients at risk for steroid adverse eﬀects(glucose intolerance,overweight,oste-oporosis).The onset of therapeutic response may be delayed for several months,and often occurs after3–10months of continuous therapy.The dosage is usu-ally 2.5–3mg/kg/day at the start of treatment and 1mg/kg/day as maintenance.Azathioprine-related side eﬀects(e.g.leukopenia,thrombocytopenia,nau-sea,vomiting,hepatotoxicity and risk of neoplasia) generally occur at higher doses[44].Blood counts and liver function analyses should be performed at baseline and rechecked weekly for4weeks and then every3months in theﬁrst year and every6months after that.About11%of the population are hetero-©2014The Author(s)European Journal of Neurology©2014EFNSEFNS/ENS Guidelines689zygous and0.3%homozygous for mutations of the thiopurine methyltransferase gene and have an increased risk of azathioprine-induced myelosuppres-sion.Two studies have examined the eﬀects of azathio-prine and both indicated a beneﬁcial eﬀect on the risk of progression from ocular to generalized MG[12,40] (Class III evidence).Azathioprine,usually adminis-tered concomitantly with prednisolone,signiﬁcantly reduced the development of GMG(12%compared with64%for patients without immunosuppressive treatment)[12](Class III evidence).A placebo-con-trolled randomized study showed that the combina-tion of prednisolone and azathioprine is more eﬀective than prednisolone alone in the treatment of GMG [45](Class I evidence).Testing for thiopurine methyl-transferase deﬁciency before start of treatment with azathioprine is recommended.The task force has agreed that when steroid treat-ment does not result in good control of the symptoms and/or if it is necessary to use high steroid doses,aza-thioprine should be started in order to reduce steroids to the lowest eﬀective dose(recommendation level C). Mycophenolate mofetilMycophenolate mofetil inhibits nucleic acid synthesis and the replication of B and T lymphocytes[46].A prospective observational study was performed on31 ocular myasthenia patients.Eighty-seven per cent of those who were on corticosteroids and then switched to mycophenolate mofetil remained as purely ocular MG over a mean period of4.2years.Four patients discontinued mycophenolate mofetil owing to side eﬀects and all four progressed to GMG[47].Symp-tom relapse,including ptosis and/or diplopia, occurred in23%and was treated with pyridostig-mine.Because these patients were initially treated with corticosteroids,it is diﬃcult to determine whether mycophenolate mofetil or prednisolone per se modiﬁed the natural history of disease progression. However,this study showed that mycophenolate mo-fetil can be used as a steroid-sparing agent for the long-term treatment of ocular myasthenia(Class IV evidence,good practice point).The average dose of mycophenolate mofetil was1g/day,which is lower than in generalized MG.Eﬀects are usually seen after a few weeks[48].Mycophenolate mofetil side eﬀects are usually mild and infections,myelosuppression and hepatotoxicity rarely occur.Blood count analyses should be performed at baseline and rechecked weekly for4weeks,then bimonthly for2months, then monthly for theﬁrst year and every6months after that.Cyclosporine A and tacrolimusCyclosporine A and tacrolimus are calcineurin inhibi-tors,causing blockade of the nuclear factor of acti-vated T cells[49].Cyclosporine has been used to treat ocular myasthenia in isolated cases,with a good clini-cal response(Class IV,good practice point).Potential side eﬀects,especially nephrotoxicity and hyperten-sion,are signiﬁcant.Tacrolimus as monotherapy was described in a ret-rospective study for treatment of ocular myasthenia with good eﬀect in four patients[50](Class IV evi-dence).A randomized,double-blind,placebo-con-trolled study that also included ocular myasthenia patients showed that tacrolimus had a moderate ste-roid-sparing eﬀect[51](Class III evidence,good prac-tice point).MethotrexateA small,single-blinded trial of methotrexate versus azathioprine as steroid-sparing agents in GMG showed that methotrexate has similar eﬃcacy and tol-erability to azathioprine and is an eﬀective steroid-sparing agent[52](Class III evidence,good practice point).Other immunosuppressive drugsOther immunosuppressant drugs, e.g.cyclophospha-mide,etanercept and rituximab,have not been reported systematically for the treatment of ocular myasthenia.Short-term treatmentsHigh doses of immunoglobulin and plasma exchange represent short-term treatments,are recommended in severe cases of GMG and are not indicated for patients with purely ocular symptoms(good practice point).ThymectomyDespite the absence of randomized studies,thymec-tomy is recommended for patients with non-thymo-matous GMG to increase the probability of remission or improvement[53].Postoperative improvement can take a long time,making it diﬃcult to distinguish the eﬀect of surgery from the immunosuppressive drug treatment.Thymectomy has been used to a limited extent and in highly selected populations with ocular myasthenia considered to be unresponsive to other therapies[20]. Remission rates were reported as6%–50% [2,12,54,55](Class III evidence).Some studies reported©2014The Author(s)European Journal of Neurology©2014EFNS690 E.Kerty et al.that thymectomized patients were less likely to pro-gress to GMG and more likely to undergo full remis-sion[56–58](Class III evidence).A retrospective review of110patients with ocular myasthenia who underwent extended trans-sternal thymectomy demon-strated that84.6%of patients experienced symptom-atic improvement after a median follow-up of 33.5months[59].The task force has agreed that thymectomy is not recommended for ocular myasthenia asﬁrst-line treat-ment but should be considered if drug treatment has failed(good practice point).Assistive devicesMechanical lid elevation for eyelid droop with eyelid crutches or tape is eﬀective and well tolerated for a short time[60](Class IV evidence).Prolonged use can result in exposure keratopathy.In the case ofﬁxed ptosis eyelid,lift surgery may be beneﬁcial[61](Class IV evidence).Prisms can be helpful for patients with stable ocular misalignment when the deviation is mild to moderate.Eye muscle surgery may be beneﬁcial in rare cases whenﬁxed strabismus persists for long term (good practice point).Occlusive devices and contact lenses will resolve diplopia through monocular vision but reduce the visualﬁeld[62](Class IV evidence). The possibility of assistive devices should be discussed with the patient as an alternative to pharmacological treatment,even for a short time. RecommendationThe treatment of ocular myasthenia should be started with pyridostigmine.If this is not successful in reliev-ing symptoms,oral corticosteroids should be used on an alternate-day regimen.If steroid treatment does not result in good symptom control and/or if it is nec-essary to use high steroid doses,steroid-sparing treat-ment with azathioprine should be started.In thymomatous MG,thymectomy is indicated.A few reports suggest that thymectomy can prevent MG generalization.Recommendation levels are generally C or good practice points.Well designed,randomized, placebo-controlled multicentre trials with a suﬃciently long follow-up period are needed to determine the eﬃ-cacy of diﬀerent immunosuppressive therapies in ocu-lar myasthenia.Future studies should measure the improvement of ocular symptoms,risk of progression to generalized disease,and side eﬀects.Disclosure of conﬂicts of interestThe authors declare noﬁnancial or other conﬂicts of interest.References1.Skeie 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眼肌型重症肌无力辅助诊断方法对比研究

眼肌型重症肌无力辅助诊断方法对比研究梁芙茹;张天佑;姚桂娟;贾艳红;李静;刘国荣【摘要】目的比较冰试验、新斯的明试验、单纤维肌电图(SFEMG)诊断眼肌型重症肌无力的敏感性和特异性,以期建立益于临床开展的诊断流程.方法共116例新发眼睑下垂和(或)复视患者,进行冰试验、新斯的明试验和SFEMG,经随访6个月观察病情变化和试验性治疗效果而最终明确诊断.结果剔除最终诊断不明、失访和随访期间进展为全身型重症肌无力的患者,最终纳入81例患者[包括眼肌型重症肌无力21例和其他疾病引起的眼睑下垂和(或)复视60例],冰试验诊断眼肌型重症肌无力的灵敏度为95.24％(20/21)、特异度为98.33％(59/60),新斯的明试验分别为90.48％(19/21)和85％(51/60),SFEMG分别为95.24％(20/21)和80％(48/60),3种诊断方法仅特异性差异有统计学意义(x2=5.232,P=0.022),且冰试验的特异度高于新斯的明试验(x2=5.707,P=0.017)和SFEMG(x 2=6.023,P=0.014).结论冰试验诊断眼肌型重症肌无力的敏感性和特异性均较高,结合新斯的明试验和SFEMG 对眼肌型重症肌无力的早期诊断具有重要临床意义.【期刊名称】《中国现代神经疾病杂志》【年(卷),期】2016(016)010【总页数】5页(P684-688)【关键词】重症肌无力;眼睑下垂;复视;冰试验(非MeSH词);新斯的明;肌电描记术【作者】梁芙茹;张天佑;姚桂娟;贾艳红;李静;刘国荣【作者单位】014040 内蒙古自治区包头市中心医院神经内科;014040 内蒙古自治区包头市中心医院神经内科;014040 内蒙古自治区包头市中心医院眼科;014040内蒙古自治区包头市中心医院神经内科;014040 内蒙古自治区包头市中心医院神经内科;014040 内蒙古自治区包头市中心医院神经内科【正文语种】中文重症肌无力（MG）是抗乙酰胆碱受体（AChR）抗体介导的、细胞免疫依赖性和补体参与的自身免疫性疾病。

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