重症肌无力患者胸腺切除术后合并视神经脊髓炎1例（附文献复习）

目的：分析重症肌无力（MG）患者胸腺切除后合并视神经脊髓炎的临床特点,探讨两者合并发生的机制。方法：结合文献对1例MG胸腺切除后合并视神经脊髓炎患者的临床特点和血清学改变进行分析。结果：现有的MG合并视神经脊髓炎的文献报道中,绝大多数（13/15例）在胸腺切除后发生。胸腺切除后抑制T细胞产生减少,B细胞过度增殖,自身免疫抗体增加,可能与视神经脊髓炎的发病有关。此外,HLAB8、DR2和DR3也可能与MG合并视神经脊髓炎的发生相关。结论：MG患者胸腺切除后引起的免疫系统改变（如血清中抑制T细胞数的改变和自身抗体的出现）,患者的HLA抗原类型（如HLA-B8、DR2和DR3）可能与合并视神经脊髓炎的发生相关。     

视神经脊髓炎谱系疾病合并重症肌无力的临床特点及相关机制

目的探讨视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder,NMOSD)与重症肌无力(myasthenia gravis,MG)共病的临床特点及可能机制。方法对北京协和医院炎性脱髓鞘疾病临床研究队列中的NMOSD与MG共病患者2例进行报道,并进行文献复习,总结NMOSD合并MG患者的临床特点。结果自2011年1月至2019年4月,本队列共登记NMOSD患者654例,其中2例(3.06‰)合并MG;结合既往文献报道,本文共纳入79例NMOSD与MG共病患者。NMOSD与MG共病多见于青中年,女性居多(64例,85.3%);MG多先于NMOSD发生,两病出现的平均间隔时间为(12.05±5.04)年;MG类型多为全身型(55例,69.6%),共48例(60.8%)接受了胸腺切除术;NMOSD与MG共病者可合并其他免疫疾病及存在其他免疫相关抗体阳性。结论NMOSD及MG的共病不仅仅是巧合,其共病存在一定特点,值得临床注意。     

重症肌无力合并胸腺结核1例报道

<正>1病例报告患者,女,58岁。因"双眼睑下垂伴复视20d"于2014—02入院。患者于本次入院前20d,无明显诱因出现双眼睑下垂,以左侧为重,晨轻暮重,伴复视。入院后检查:T 36.5℃,P 76次/min,R 19次/min,Bp 138/95mmHg,神志清,精神差,言语清晰,双眼睑下垂,双眼球活动受限,四肢肌力Ⅳ级。既往史:"高血压"病史7a余,口服酒石酸美托洛尔6.25     

重症肌无力胸腺切除术后远期生存多因素分析

目的　探讨重症肌无力患者胸腺切除术后影响远期生存的因素。方法　采用胸腺切除术治疗重症肌无力170例,对其中124例进行平均39.8个月的长期随访,运用统计软件对年龄、性别、病程、临床分型、病理类型等因素进行COX回归模型多因素分析。结果　影响远期生存的因素是胸腺的不同病理类型(胸腺增生、胸腺萎缩、良性胸腺瘤、恶性胸腺瘤)(P<0.05),临床分型与病理类型具有相关性(P<0.01)。结论　重症肌无力胸腺切除术后远期生存率依胸腺增生、良性胸腺瘤、胸腺萎缩、恶性胸腺瘤等不同病理类型依次下降,临床分型在一定程度上可反映预后,强调早期手术治疗和术后巩固治疗是提高远期生存率的关键。     

胸腺切除术在重症肌无力治疗中的地位

目的 评估胸腺切除术治疗重症肌无力(MG)的疗效.探讨个体化中西医治疗方案.方法 21例患者均在手术前后口服强的松、溴化吡啶斯的明,中医中药控制无力症状,均行扩大胸腺切除术,术后继续行中西医的内科治疗,1年以后评定疗效.结果 总有效率83%,无效17%,术后早期发生肌无力危象3例,无手术死亡者.结论 胸腺切除术结合中西医内科治疗重症肌无力疗效满意.     

行胸腺切除重症肌无力患者临床分析

目的探讨重症肌无力患者的临床特点、诊断方法和治疗效果。方法收集本院2003-09—2010-09收治的122例重症肌无力行胸腺切除患者的临床资料,术后随访2a观察并发症及病死率。结果最常见的临床表现为眼睑下垂(77.0%)和上肢无力(70.5%),CT扫描显示51.6%患者胸腺增生,11.5%患者存在胸腺瘤。术后并发症发生率为13.1%,病死率6.6%。结论行胸腺切除术后重症肌无力患者有较高的病死率,应进行更谨慎的术前评估和治疗。     

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。     

胸腺瘤术后重症肌无力六例报告

33 %～ 75 %胸腺瘤患者伴发重症肌无力 (ＭＧ) ,大多数先有ＭＧ症状而后发现胸腺瘤 ,约 3 %胸腺瘤患者手术后出现ＭＧ症状。我们报道 6例胸腺瘤患者手术后出现ＭＧ。例 1　 男性 ,3 2岁 ,体检时发现纵隔占位性病变 ,1977年9月行肿瘤大部分切除 ,病理报告为淋巴细胞型胸腺瘤。 11月出现视物双影 ,双眼睑下垂 ,1978年 1月症状加重 ,出现四肢无力 ,不能穿衣等。新斯的明试验阳性 ,服用吡啶斯的明治疗 ,2个月后症状逐渐好转 ,基本恢复正常。例 2 　 女性 ,4 0岁 ,1979年 6月发现左侧颈旁淋巴结肿大 ,胸片左心隔心包囊肿 ,手术切除 ,病理诊断淋巴…     

类固醇与胸腺放疗联合疗法治疗22例胸腺切除后无效的重症肌无力

本文报告用类固醇和胸腺放射联合疗法治疗２２例胸腺切除后无效的重症肌无力患者的疗效。２个月内的近期有效率高达１００％。经１～１２年的长期随访，达完全缓解和显著改善者１９例，占８６．４％。停用一切药物长达２～１０年而无任何症状者７例，占３１．８％。     

胸腺切除治疗重症肌无力围术期处理及疗效评价

目的对行胸腺切除术治疗的重症肌无力病人按围术期处理，进行疗效评估。方法应用胸腺切除术治疗的６４例重症肌无力病人，手术前后口服皮质类固醇、胆碱酯酶抑制剂，静脉滴注丙种球蛋白，并行血浆置换等以缓解肌无力症状。测定外周血乙酰胆碱受体抗体（ＡＣｈＲａｂ）和胸腺瘤相关抗体（ＣＡＥａｂ）的滴度。结果病人组术后ＡＣｈＲａｂ滴度明显下降，而ＣＡＥａｂ则无明显变化。术后随访１～１０年，总缓解率为８１．３％。结论所有重症肌无力病人，只要手术前后应用皮质类固醇调整免疫功能以及胆碱酯酶抑制剂、大剂量丙种球蛋白和血浆置换疗法等缓解症状，均可接受胸腺手术治疗，不受年龄、性别和病程的限制。     

Neuromyelitis optica associated with myasthenia gravis: characteristic phenotype in Japanese population

We report two female patients with neuromyelitis optica (NMO, Devic's syndrome) following or coincidental with myasthenia gravis (MG). Their illnesses were characterized by subacute myelitis with optic neuritis, high serum levels of muscle acetylcholine receptor antibody, and autoimmune thyroid disease. Both patients fulfilled the clinical criteria of NMO, however, NMO-IgG, autoantibody against aquaporin-4 water channel, was absent from their sera. Both NMO and MG are relatively rare diseases. The considerable coincidence of these two disorders suggests that there is a subgroup of patients with NMO having a common immunological pathogenesis with MG.     

Sleep apnoea in well-controlled myasthenia gravis and the effect of thymectomy

We conducted overnight polysomnographic sleep studies of 16 patients (5 men and 11 women) with clinically well-controlled myasthenia gravis (MG). The subtypes of MG were IIA (3 patients), IIB (11 patients), IV (1 patient) and V (1 patient). Twelve patients were found on polysomnography to have obstructive and/or central types of the sleep apnoeas (SA). Their mean age was 42.4, SD 16.4 years, and the mean duration of MG was 7.4, SD 6.96 years. SA was not detected in 4 patients whose mean age was 30.8, SD 10.71 years and who had manifested MG for a mean duration of only 0.9, SD 0.65 years. Thus, patients with a longer duration of MG tended to have more SA. In 9 of the 12 SA patients, polysomnographic studies were repeated following thymectomy. SA had resolved in 6 patients, but persisted in 3. These findings suggest that SA is a possible clinical manifestiation of MG and that nocturnal dysfunction of both peripheral and central colinergic systems may be involved. Received: 10 April 1996 Received in revised form: 12 September 1997 Accpeted: 1 October 1997     

Evaluation of results of thymectomy in myasthenia gravis

Summary The results of thymectomy carried out in 150 cases of myasthenia gravis are discussed. In a group of 123 cases followed for 1 to 5 years after the operation, full remission was observed in 24.4% of cases, significant improvement in 36.6%, slight improvement in 24.4% and no improvement in 8.1%, while deterioration occurred in 1.6% of patients. No correlation was found between the result of the operation and the age and sex of patients, but better results were achieved in those treated surgically rather soon after the onset of symptoms. This correlation was particularly evident in the group with full remissions. The results obtained in the cases without thymic tumors were better than in the cases with tumors. No correlation was noted between the results of the operation and the histological characteristics of the thymus in the group with thymic hyperplasia and in the group with thymic atrophy. The surgically treated group (150 cases), compared with the conservatively treated group (75 cases), showed the superiority of the surgical method (lower rate of death and deterioration, higher rate of improvement and remission).In discussing the indications for surgical treatment the authors emphasize that advances in anaesthesiology in recent years have reduced the risk of operation. It is suggested that the indications for surgical treatment should be expanded and operations should be performed as early as possible after the onset of clinical manifestations without regard to the age and sex of the patient. Operation should not be considered in cases belonging to group 1, 2a (sometimes 2b) only, with duration of the disease over 8–10 years and with little or no progression of the process, if the presence of a thymic tumor has been excluded.This work was supported by NIH., N.I.N.D.S.—Bethesda (agreement 05-002-1).     

Effects of thymectomy on late-onset myasthenia gravis without thymoma

OBJECTIVES: This study aims to investigate whether thymectomy is beneficial for late-onset (>50 years) myasthenia gravis patients with no thymoma, particularly for those with mild generalized weakness. PATIENTS AND METHODS: A total of 34 patients were included in the study. The clinical course and long-term outcomes over 2 years were reviewed in 20 patients who underwent thymectomy and in 14 without thymectomy. RESULTS: Of the 34 patients, 20 (59%) underwent thymectomy. Thymectomized patients had more severe disability at entry than non-thymectomized patients, but outcome measures did not significantly differ between the two patient groups. Moreover, subgroup analyses including 22 patients with mild generalized weakness at entry showed that the thymectomized group (n=10) showed a greater percentage of clinical remission (no symptoms; 50% versus 17%; p=0.11) and a lower frequency of the presence of generalized symptoms (30% versus 75%; p<0.05) than the non-thymectomized group (n=12) at the end of follow-up (means 9.6 years after onset). CONCLUSIONS: Thymectomy is a potentially effective treatment for late onset, non-thymomatous patients with mild generalized myasthenia gravis.     

Anti-acetylcholine receptor antibodies in myasthenia gravis. Part 3. The effect of thymectomy

The clinical condition and the serum levels of antibodies to acetylcholine receptor protein were followed in 30 patients with myasthenia gravis before and in a period varying from 2 to 4 (mean 3) yr after thymectomy. Twenty-five patients improved in the 2 yr following thymectomy. A highly significant correlation was found between the change in clinical condition and the change in antibody levels. Only 3 patients improved without a fall of antibody level. Prethymectomy antibody levels were positively correlated with the severity of the clinical condition and with the degree of thymus hypertrophy.     

胸腺切除术对重症肌无力患者电生理学指标和临床评分的影响

目的评价胸腺切除术对重症肌无力(MG)的近期疗效。方法对15例MG在胸腺切除术前(平均9天)及术后(平均11天)依次进行了临床绝对评分、右侧腋神经低频重复电刺激(RNS)和右侧伸指总肌单纤维肌电图(SFEMG)检查。结果手术后SFEMG和RNS异常率分别为100%和73%,手术后平均颤抖(jitter)值、异常电位对比例、阻滞电位对比例和临床绝对评分都较手术前有显著的降低;病理为胸腺瘤5例,手术前后平均jitter值、阻滞电位对比例和临床绝对评分均无显著差异,胸腺增生9例,手术后阻滞电位对比例和临床绝对评分较术前显著减低。结论MG患者胸腺切除术后在临床表现和电生理学指标上都有一定程度的改善,但此时大多数患者仍有临床症状和电生理学指标的异常,需要进一步的免疫调节治疗;MG并发胸腺瘤者可能手术后近期疗效差,并发胸腺增生者可能疗效相对较好。     

重症肌无力病人胸腺切除术后血清自身抗体水平变化和临床转归分析

６９例重症肌无力（ＭＧ）病人行胸腺切除术，术前血清乙酰胆碱受体抗体水平均高于正常，术后半年抗体水平明显下降，胸腺增生组（２０例）尤为显著．胸腺瘤组（４９例）术前胸腺瘤相关抗体水平增高，但术后半年内无明显变化．６９例ＭＧ患者胸腺切除术后４年，症状总缓解率为７６．８１％，其中胸腺增生组９５％，胸腺瘤组６９．３９％。作者认为，对于伴有胸腺增生或胸腺瘤的ＭＧ患者，胸腺切除术是有效的，应该列为治疗的第一选择。     

Neuromyelitis optica in patients with myasthenia gravis who underwent thymectomy

BACKGROUND: Myasthenia gravis (MG) and neuromyelitis optica (NMO, also known as Devic disease) are rare autoimmune disorders, with upper-limit prevalence estimates in the general population of 15 per 100,000 and 5 per 100,000, respectively. To our knowledge, an association between these diseases has not been previously reported. OBJECTIVES: To describe 4 patients with MG who developed NMO after thymectomy and to analyze possible causes of apparent increased prevalence of NMO among patients with MG. DESIGN: Case series. PATIENTS: Four patients with MG who underwent thymectomy. INTERVENTIONS: None. RESULTS: The prevalence of MG within the published cohort of patients with NMO is more than 150 times higher than that in the general population. CONCLUSION: Dysregulation of B-cell autoimmunity in myasthenia, possibly exacerbated by loss of control over autoreactive cells as a result of thymectomy, may predispose patients to the development of NMO.     

Myasthenia gravis appearing after thymectomy

We aimed to evaluate the clinical characteristics of patients with postoperative myasthenia gravis (MG). We retrospectively studied the data of 174 thymoma patients treated between 1990 and 2008 in Xiangya Hospital. Six of 125 patients without preoperative MG (4.8%) developed postoperative MG. The anti-acetylcholine-receptor binding antibody (ARAb) titers were elevated preoperatively in 22 of the 125 patients (17.6%) who did not have preoperative MG (range, 0.5-67.6 nmol/L). Four of six patients with postoperative MG had positive ARAb levels preoperatively. Serum titers were exacerbated in all six patients at the onset of postoperative MG. Postoperative MG was responsive to anti-cholinesterase compounds and/or steroids. We concluded that a thymectomy did not prevent postoperative MG. Exacerbated ARAb levels after thymectomy suggested an extrathymic production of ARAb. We suggest that a rise in the ARAb titer might be a risk indictor for post-thymectomy MG.