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.     

重症肌无力19例临床分析

目的方法对19例重症肌无力(MG)患者的临床特点和治疗效果进行回顾性分析、总结临床经验。结果男性患者8例,女性患者11例,男女比例1:1.375。发病年龄为2~77岁,平均年龄40.63岁。首发症状为眼睑下垂者10例,占52.6%;其中眼睑下垂合并锥体束征1例,合并甲状腺功能亢进者4例,合并右耳听力下降者1例。19例患者均在应用抗胆碱酶药物溴吡斯的明基础上加用激素或丙种球蛋白治疗,其中有效16例,无效3例,总有效率为84.2%。合并胸腺瘤者5例,均行胸腺切除,术后有效4例,无效1例,总有效率80%。结论 MG多青壮年发病,10%~15%合并胸腺瘤,临床表现复杂多样,首发症状以眼睑下垂最为多见,激素、丙种球蛋白及胸腺切除术均为治疗MG的有效手段。     

伴胸腺瘤重症肌无力的临床特点(附96例分析)

目的　研究伴胸腺瘤重症肌无力的临床特点。方法　对经手术病检证实的 96例MG伴胸腺瘤患者的临床资料进行回顾性分析 ,并采用 χ2 检验及t检验与 114 9例经影像学检查无胸腺瘤表现的MG患者进行比较。结果　胸腺瘤组男性发病者多 (男∶女 =1 82∶1) ,且多于 30岁后发病 (71 9% ) ,以肢体无力和延髓症状首发者多见 (4 3 8% ) ,按改良Os serman分型 ,以Ⅲ型和Ⅳ型为主 (4 5 8% ) ,危象发生率高 (38 5 % ) ,病死率高 (8 3 % )。结论　伴胸腺瘤的重症肌无力有其独特临床特点 ,充分认识这些特点将有利于指导对这类患者的临床诊断和治疗     

老年重症肌无力诊治分析

目的 探讨老年重症肌无力(MG)的临床特点.方法 回顾性分析2014-01—2020-01在郑州大学第二附属医院接受治疗的89例老年MG患者的临床资料.结果 老年MG患者男女比例为1:1.02.以OssermanⅠ型起病者44例,男24例(54.5％),女20例(45.5％);以OssermanⅡ型起病者43例,男18...     

老年期重症肌无力的临床特点

目的 研究老年期重症肌无力（ＭＧ）的临床特点，方法 将２０年间诊治的１９２４例ＭＧ，根据发病年龄分为小儿期ＭＧ（≤１４岁）成年期ＭＧ（１５～５９岁）及老年期ＭＧ（≥６０岁），采用Ｘ＾２检验比较３组的临床资料。结果 老年期ＭＧ在整个ＭＧ中构成比小（只占５．５％）男性发病者较多见（男：女＝１．４：１）以延髓症状首发者多见，合并胸腺者多而全并其他自体免疫病者少，以延髓症处发面误诊为脑血管者多见，病死率高     

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     

老年起病型重症肌无力患者的临床特点

目的探讨老年起病型重症肌无力(MG)的临床特点。方法回顾性分析332例起病年龄≥65岁的MG患者的临床资料。结果该组MG患者男女比例为1.3∶1。全身型MG (GMG)和眼肌型MG(OMG)的比例为1.9∶1,OMG在男性多于女性而GMG在女性多于男性(P=0.033)。以OMG起病和以GMG起病的比例为1.5∶1,起病症状以眼睑下垂最多见占38.6%;在以OMG起病的患者中,女性患者转化为GMG的比率要明显高于男性患者(P=0.035)。病程中位数为3.7年,平均定量MG评分为7分。新斯的明试验阳性率为96.7%。低频重复频率电刺激波幅递减的总体发生率为48.2%,其中面神经的阳性率最高。MG合并胸腺异常的发生率为36.3%,以合并胸腺瘤者最多;胸腺瘤手术后病理分型以AB型最多,女性患者AB型胸腺瘤所占的比率要明显高于男性(P=0.048)。MG合并甲状腺功能异常的发生率为16%,以合并甲状腺功能亢进者最多。结论老年起病型MG患者GMG多于OMG、以OMG起病的女性患者转化为GMG的比率高于男性患者,胸腺瘤是最常见的胸腺异常且最常见的胸腺瘤病理分型为AB型。充分认识其临床特点有利于更好的治疗该类患者。     

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。     

老年重症肌无力的临床特点(123例临床分析)

目的 研究老年重症肌无力(MG)的临床特点。方法 回顾性分析1990-01—2002-05北京医院123例老年MG住院患者的临床特点，并与507例青壮年MG患者进行对比。结果 老年MG患者占同期MG患者的18．14％，男性患者较多(占68．29％)，眼肌首发症状多见(占80．49％)，分型以I型和Ia型多见(占56．10％)，胸腺异常主要为胸腺瘤(占老年MG患者手术病理证实伴发胸腺异常的100％)，低频重复电刺激(RNS)阳性率为74.19％，老年MG危象发生率较低(为2．44％)。结论 老年MG患者具有男性、眼肌首发症状、I型与IA型多见，以及胸腺异常主要为胸腺瘤，而RNS阳性率与MG危象发生率低等临床特点。     

胸腺瘤切除术后重症肌无力患者11例的临床特点

在重症肌无力（MG）患者中常伴有胸腺的异常病变,尤其是胸腺瘤.95%以上的胸腺瘤是在发现MG后检查时被发现的,可能在出现MG临床相之前就已存在.胸腺瘤本身可无临床表现,但可合并多种免疫性疾病;也可因查体时偶然发现或由于增大出现呼吸困难症状就诊时发现.胸腺瘤发病时并不一定伴随有MG表现,可在胸腺手术后数月或数年才发生MG的临床相.胸腺手术可缓解MG的临床相,减少发生危象的几率.我们就我院发现的胸腺瘤术后发生的MG作一报道。     

Paraneoplastic myasthenia gravis: immunological and clinical aspects

Paraneoplastic myasthenia gravis (MG) is accompanied by a neoplasm, usually thymoma. In patients with thymoma and a specific genetic make‐up, the paraneoplastic immune response develops further in thymic remnant or peripheral lymphatic tissue. Paraneoplastic MG and late‐onset MG (age ≥ 50 years) share a similar immunological profile with high titin and ryanodine receptor (RyR) antibody prevalence. This profile is the most important predictor of clinical outcome in paraneoplastic MG. The presence of a thymoma per se does not cause more severe MG. MG severity is linked to the patient’s immunological profile. Paraneoplastic MG causes a distinctive non‐limb symptom profile at MG onset, characterized by bulbar, ocular, neck, and respiratory symptoms. When the diagnosis of paraneoplastic MG is established, the neoplasm should be removed surgically. Pre‐thymectomy plasmapheresis or iv‐IgG should be considered in these patients to minimize post‐thymectomy MG exacerbation risk. Paraneoplastic MG usually continues after thymectomy. The pharmacological treatment of paraneoplastic MG does not differ from non‐paraneoplastic MG, except for tacrolimus that should be considered in difficult cases. Tacrolimus is an immunosuppressant acting specifically in RyR antibody positive patients through enhancing RyR‐related sarcoplasmic calcium release that in theory might be blocked by RyR antibodies, causing symptomatic relief in paraneoplastic MG.     

重症肌无力患者血清连接素抗体与临床的关系

目的 探讨重症肌无力（MG）患者血清连接素抗体（Titin—Ab）水平与病情及胸腺异常的关系,以及其对诊断和预后判断的价值。方法 应用酶联免疫吸附法（ELISA）检测MG患者（MG组）52例、非MG（其他神经系统疾病）患者（NMG组）18例、健康对照者（NC组）50名血清中Titin—Ab水平,并对其中10例进行胸腺手术的MG患者随访。结果 MG组Titin—Ab阳性率为36．5％,NMG组和NC组均为阴性,MG组显著高于NC组和NMG组（均P〈0．01）;以MG伴胸腺瘤（MGT）组阳性率最高（83．3％）,MG伴胸腺萎缩（MGA）次之（37．5％）,胸腺正常的MG组为23．0％,MG伴胸腺增生组为阴性;Titin．Ab诊断MGT特异性高于胸腺CT（P〈0．05）。Titin—Ab阳性MG患者抗体水平与肌无力程度呈显著正相关（r=0．562,P〈0．01）;对10例不同胸腺病理类型的MG患者手术前后Titin—Ab测定显示差异具有显著性（P〈0．05）。结论 Titin—Ab阳性多见于MGT和MGA者,诊断MGT的特异性高于CT;Titin—Ab对MG的诊断、病情及预后判断均有帮助。     

Prevalence and clinical aspects of immigrants with myasthenia gravis in northern Europe

Introduction: Multiethnic studies can provide etiological clues toward the genetic and environmental influence of a disease. The aim of this study was to determine prevalence and clinical features of myasthenia gravis (MG) in immigrants compared with native patients in 2 population‐based cohorts. Methods: This cross‐sectional study included 843 MG patients (375 from Norway and 468 from the Netherlands). Ethnic background was defined by questionnaires. Results: Among the participating MG patients, 163 of 843 (19.3%) were first or second generation immigrants, mainly from Europe, Asia, and South America. No marked prevalence differences were found between immigrants and native ethnic groups. MG with muscle specific kinase antibodies and MG with thymoma were more frequent in Asian MG immigrants compared with other ethnic groups (8% vs. 0–4%; P < 0.001 and 21% vs. 6–10%; P < 0.001), respectively. Conclusions: Our findings indicate that Asian immigrant MG patients carry genetic factors or environmental/lifestyle factors which contribute to their specific phenotype, even after migration. Muscle Nerve 55 : 819–827, 2017     

Heart disease in myasthenia gravis

Patients with myasthenia gravis (MG) may develop heart disease. Our data on 108 MG patients were examined to assess the type and frequency of this. 17 of 108 patients (16%) showed signs of heart disease which could be regarded as MG-related. 11 of these, of whom 5 died suddenly, had clinical symptoms, mainly arrhythmias. Signs of heart disease were most frequent in thymoma patients (5 of 10), and all 3 microscopically examined hearts from these patients showed a focal myocarditis. 44 spinal muscular atrophy patients serving as controls showed a 16% frequency of signs of heart disease of unknown etiology. However, only 1 of 44 spinal muscular atrophy patients (2%) had clinical symptoms as compared to 11 of 108 MG patients (10%). Together with the characteristic focal nature of the myocarditis and the microscopic similarities between lesions of heart and skeletal muscle, this indicates that the heart disease is specifically related to MG.     

Myasthenia gravis in the elderly: a hospital based study

To evaluate clinical characteristics and outcome of myasthenia gravis (MG) in aged patients (>60yrs), we retrospectively reviewed a continuous series of 122 myasthenic patients observed from January 1968 through December 1994. Patients with congenital, neonatal, or penicillamine-induced myasthenia were excluded. Twenty-five subjects (20%) were >60yrs. The male/female ratio was 3:2; 20% of patients had an ocular form and 86% were seropositive. Mediastinum CT scan revealed thymic changes in 14%. During the first five years of disease, 60% of patients with ocular form progressed towards a generalized form and 15% had clinical relapses. At the time of their last visit, 40% of patients were asymptomatic and 60% had improved on medication. No patient died because of myasthenia-related causes. This study shows that MG in aged patients is characterized by prevalence in males, low frequency of ocular forms, low frequency of positive mediastinum CT which suggests low frequency of thymomas, high frequency of progression of ocular forms, and good response to corticosteroid therapy.     

Taste disorders in myasthenia gravis: a multicenter cooperative study

Background and purpose: The purpose of the present study was to investigate the prevalence and clinical characteristics of taste disorders in patients with myasthenia gravis (MG). Methods: We studied 371 Japanese patients with MG (127 men and 244 women; mean age, 56.6 ± 16.9 years) consecutively evaluated between May and September 2010 in six neurological centers comprising the East Japan MG Study Group. Ninety‐three patients (25%) had thymoma. We interviewed all patients to determine whether they had taste disorders during the clinical course of MG and then further evaluated the patients with MG, who reported having taste disorders, using a questionnaire. Results: Taste disorders were observed in 16 (4.3%) of the 371 patients with MG. We concluded that taste disorders in 2.4% of patients with MG excluding other factors were associated with MG itself. All patients had thymoma with seropositivity for anti‐acetylcholine receptor antibodies. Thymoma tended to be advanced, and four patients with Masaoka stage IVa required radiation therapy or chemotherapy. Five patients noticed taste disorders 2–3 months before the onset of MG. Sweet taste loss was more common than salty, bitter, and sour taste loss. Conclusions: This was the first systematic survey of taste disorders in patients with MG by a multicenter study. Taste disorders were more common in the present sample of patients with MG than in the general population.     

青少年起病的重症肌无力患者临床特点分析

目的分析青少年起病的重症肌无力(MG)患者的临床特点。方法回顾性分析2006-2013年作者医院神经内科住院的351例起病年龄≤18岁MG患者的临床资料,包括人口统计学(性别、年龄),临床特点(临床分型、起病症状、疾病转归、胸腺情况)和合并疾病(自身免疫性疾病)。结果青少年起病的MG患者发病年龄小于5岁者占45.9%,女性多见占53.6%。根据美国重症肌无力协会(myasthenia gravis foundation of America,MGFA)分型,Ⅰ型占80.3%,Ⅱa型占8.5%,Ⅱb型占9.1%,Ⅲa型占0.3%,Ⅲb型占1.4%,Ⅳb型占0.3%。由眼肌型MG(OMG)转化为全身型MG(GMG)患者39例(13.8%),主要诱发转化的因素为不规律用药或停药。诱发病情复发或加重的因素中以受凉多见,占63.6%。合并胸腺异常患者有60例,其中28例行胸腺切除术,术后病理分型:胸腺增生20例,胸腺瘤4例。合并有其他自身免疫性疾病共20例,其中甲状腺功能亢进占4.6%,甲状腺功能减低占0.6%,甲状腺囊肿占0.6%。共有6例患者发生过危象,其中5例发生肌无力危象者为由OMG转化为GMG患者。结论青少年MG患者有自己独特的临床特点:5岁之前发病更多见;诱发病情复发或加重的因素中以受凉多见;合并胸腺增生者多于合并胸腺瘤者;最常见的甲状腺功能异常为甲状腺功能亢进。     

Thymus imaging in myasthenia gravis: The relevance in clinical practice

Introduction: The ability to distinguish between normal thymus, thymic hyperplasia, and thymoma should aid clinical management and decision making in patients with myasthenia gravis (MG). We sought to determine the accuracy of routine imaging in predicting thymic pathology. Methods: We retrospectively analyzed records of patients with MG from the Oxford Myasthenia Centre registry who had undergone thymectomy. Each patient received 1 radiological diagnosis and 1 histological diagnosis. Results: We included 106 patients. Radiological and histological diagnoses agreed in 73 (68.9%) patients. Sensitivity and specificity, respectively, were calculated for each radiological diagnosis as follows: thymoma 90% and 95.5%, hyperplasia 17.6% and 98.6%, and normal 96.9% and 60.8%. Discussion: Routine chest computed tomography and MRI can effectively identify thymoma. However, they are not reliable tools to differentiate between thymic hyperplasia and normal thymus in patients with MG. Muscle Nerve 58 : 153–156, 2018     

胸腺切除术治疗重症肌无力的疗效及其影响因素分析

目的总结行胸腺切除术的重症肌无力(MG)患者的疗效,并探讨影响近远期疗效的相关因素。方法回顾性分析作者医院收治的103例行胸腺切除术的MG患者的临床资料,采用卡方检验及Logistic回归分析可能影响疗效的因素。MG患者的近期及远期疗效分别用Monden、美国重症肌无力联盟(MGFA)分型进行评估。结果 103例患者中完成随访94例(91.26%),随访时间3个月~8年。近期疗效总有效率为60.2%,远期疗效总有效率为73.4%。发病年龄、性别、Osserman分型与术后近远期疗效均无相关(P0.05);病程(OR=0.240、95%CI:0.081~0.712,P0.05)、手术方式(OR=0.289、95%CI:0.110~0.757,P0.05)与近期疗效密切相关,是否合并胸腺瘤与远期疗效密切相关(OR=0.073、95%CI:0.009~0.575,P0.05)。结论病程越短,手术近期疗效越好;行胸腔镜手术较开胸手术能明显提高患者近期预后;MG不合并胸腺瘤者行胸腺切除术较合并胸腺瘤的患者更能从手术中获益。