重症肌无力的纵隔影像:胸片、CT、MR和手术所见的对照

重症肌无力(MG)患者作胸腺切除后,有时可使症状改善和缓解,因此MG患者胸腺的常规放射学检查很重要。本文探讨了16例MG患者胸腺切除术前一个月内胸片,CT和核磁共振(MR)纵隔影像与切除胸腺组织学对照,并认为在摄取胸片后,需要进一步地胸腺影像时CT仍是一种选择的方法。     

胸腺瘤手术后早期新发重症肌无力的误诊分析

目的探讨胸腺瘤切除术后早期新发重症肌无力(myasthenia gravis,MG)的误诊原因及防治措施。方法对2013-01—2018-12河南中医药大学第一附属医院心胸外科行胸腺瘤切除术,且术后早期出现新发的4例MG患者的临床资料进行回顾性分析。结果 4例患者术前均不存在肌无力症状,行胸部影像学检查时发现胸腺瘤,胸腺瘤切除术后早期均出现不典型肌无力症状,但未能及时明确诊断。随着肌无力症状快速进展,出现四肢无力、严重呼吸困难等MG症状,行新斯的明试验阳性,明确诊断,积极治疗,4例病情缓解均顺利出院。结论胸腺瘤切除术后早期新发MG的初起表现缺乏特异性,易与一些术后常见并发症相混淆,延误诊断及治疗。对于术前单纯胸腺瘤患者,仍需考虑到术后早期合并新发MG的可能。     

重症肌无力169例临床分析

目的研究重症肌无力(MG)临床特点、误诊原因与MG危象临床表现。方法分析本院收治的169例MG患者的临床资料。结果本文患者均行新斯的明试验,阳性率100%。85例行重复神经电刺激(RNS)检查,64例低频波幅递减,8例同时高频波幅递减。157例行胸腺影像学检查,发现胸腺异常80例。发生肌无力危象31例次。121例行甲状腺功能(FT3、FT4、TSH)检测,发现甲状腺功能异常17例。结论MG临床表现多样,时有误诊,易于合并胸腺瘤或胸腺增生及甲状腺功能异常。肌无力危象发生率高。     

电压门控钾离子通道复合物抗体相关临床综合征合并伴胸腺瘤的重症肌无力患者2例及文献复习

目的探讨电压门控钾离子通道复合物(VGKCc)抗体相关临床综合征合并伴胸腺瘤的重症肌无力(MG)患者的临床特点及转归。方法回顾分析山东大学齐鲁医院(青岛)分别于2020年9月和12月收治的2例确诊为VGKCc抗体相关临床综合征合并伴胸腺瘤的MG患者, 总结其临床及辅助检查、随访预后等资料, 并结合相关文献进行复习总结。结果例1为64岁女性, 临床表现为认知障碍、精神异常和癫痫样发作, 血清富亮氨酸胶质瘤失活蛋白1(LGI1)抗体阳性, 明确诊断为抗LGI1脑炎, 既往存在球部起病的MG, 胸部CT提示胸腺瘤, 入院后给予免疫治疗后症状改善。例2为67岁男性, MG诊断明确, 后期出现认知功能下降、肌颤搐、自主神经症状, 神经电生理可见F波后放电及肌颤搐电位, 血清接触蛋白相关蛋白2抗体阳性, 明确诊断为莫旺综合征合并伴胸腺瘤的MG, 入院后给予免疫治疗及胸腺瘤切除等治疗, 症状改善。结论 VGKCc抗体相关临床综合征合并胸腺瘤的MG患者同时存在两类疾病各自的临床特点, 同时又有交叉。免疫治疗及针对胸腺瘤的治疗通常能取得较好的疗效。     

重症肌无力伴多发性肌炎(附7例报告)

重症肌无力 ( Myasthenia gravis,MG)与多发性肌炎( Polymyositis PM)并存 ,国内外罕见报道。在我们 2 30 0例MG患者中 ,作者发现有 7例与 PM并存 ,现报告如下。1　临床资料两病并存的 7例患者中 ,男 1例 ,女 6例 ,年龄 2 6～ 57岁 ,均有四肢无力、持物和行走困难 ,其中颈项肌无力 2例 ,眼睑下垂和复视 2例 ,构音障碍和吞咽困难 2例 ,肌无力危象 1例 ,肌肉疼痛和压痛 3例。上述肌无力症状都有疲劳后加重的现象。7例患者开始新斯的明试验均为 ( )。肌电图检查均符合肌原性疾病 ,肌电图表现开始有渐减波现象 ,以后部分病人有肌炎改变。纵…     

重症肌无力患者的护理体会

重症肌无力（MG）是一种累及神经肌肉接头突触后膜上乙酰胆碱受体（AchR）的自身免疫性疾病.临床症状：主要表现为部分或全身骨骼肌无力,易疲劳,活动后症状加重,经休息和胆碱酯酶抑制剂（ChEI）治疗后症状减轻[1].发病率为8～20/10万,患病率为50/10万,我国南方发病率较高.其中MG患者中65%～80%有胸腺增生,10%～20%伴发胸腺瘤.重症肌无力患者的治疗：包括药物治疗、放射治疗和手术治疗.其中药物治疗,口服肾上腺皮质激素和胆碱酯酶抑制剂是现今国际公认有效的常规保守疗法.手术治疗,胸腺切除加前纵隔脂肪清扫术是目前认为治疗MG最根本的方法.     

中国北方地区老年重症肌无力患者的临床特点

目的 总结中国北方地区60岁以后发病的老年重症肌无力(MG)患者的临床特点.方法 回顾性总结47例老年MG患者的性别构成、首发症状、病情严重程度、胸腺改变、伴发疾病、治疗情况、发生危象比例等,并与中青年MG患者进行比较.结果 老年MG患者占所有住院MG患者的23.04%,男女比例为0.96∶1.00;老年MG患者以上睑下垂为主要首发症状,病情最重时约68.09%的患者进展为全身型MG,这一比例低于中青年组(P<0.05);该组老年MG患者合并胸腺瘤的比例为22.5%,低于中青年组(P<0.05); 59.57%的患者合并糖尿病、原发性高血压、冠心病等内科疾病,高于中青年组(P<0.05);除应用溴吡斯的明治疗以外,72.34%的患者应用了免疫抑制剂治疗,仅19.15%的患者应用激素治疗,应用激素治疗的比例低于中青年组(P<0.05);12.76%的老年MG患者出现肌无力危象,与中青年组比较差异无统计学意义.结论 老年MG患者并不少见,男女比例相近,以上睑下垂为最常见首发症状,病情最重时大多数患者会发展为全身型MG,故仍需要给予免疫调节治疗;老年MG患者合并胸腺瘤比例低于中青年患者,但合并其他内科疾病比例高于中青年患者,其治疗以免疫抑制剂为主,而激素应用和胸腺切除术受到限制.     

重症肌无力伴Caspr2抗体相关莫旺综合征的临床特点(附1例报告)

目的 探讨抗乙酰胆碱受体(AChR)抗体及抗接触蛋白相关蛋白-2(Caspr2)抗体重叠综合征的发病机制、临床表现、诊断及治疗方法。方法 回顾性分析1例抗AChR抗体阳性的重症肌无力(MG),同时伴抗Caspr2抗体阳性的莫旺综合征患者的临床表现及治疗过程。结果 患者有胸腺瘤及手术切除病史,抗AChR抗体及Caspr2抗体双阳性,表现除眼睑下垂、复视,吞咽困难、肢体无力及肌无力危象等MG症状,还表现有自主神经症状、肌肉颤搐及疼痛、严重睡眠障碍和波动性生动视幻觉等莫旺综合征的典型临床表现,予丙种球蛋白、糖皮质激素等免疫治疗后症状好转,继续泼尼松、硫唑嘌呤续贯治疗,随访2年未再复发。结论 MG的抗AChR抗体及莫旺综合征的抗Caspr2抗体均于胸腺瘤密切相关,提示可能存在胸腺瘤相关抗体疾病谱。其发病机制尚待进一步研究,临床表现复杂,长程免疫治疗有助于控制病情、减少复发。     

胸腺与重症肌无力发病机制的研究进展

重症肌无力(MG)是一种主要累及神经-肌肉接头突触后膜乙酰胆碱受体的获得性自身免疫性疾病。临床上主要表现为部分或全身骨骼肌无力和极易疲劳,活动后加重,经休息和胆碱酯酶抑制剂治疗后症状减轻。大多数MG患者可见胸腺病理改变。研究表明,胸腺病毒感染及慢性炎症刺激、异常免疫激活、胸腺中枢耐受性破坏及免疫调节失衡参与了MG的发生和发展过程。     

老年重症肌无力住院患者48例临床特点分析

目的分析老年重症肌无力(MG)住院患者的有关临床特点。方法回顾性分析近年来在我院住院诊治的48例老年MG患者的临床资料。结果老年MG患者占总住院MG病例数(175例)的比例为27.4%。男性多于女性,男∶女=2.7∶1。老年MG多在60~69岁之间发病,在其常见首发症状中,眼部症状最多,为34例(70.8%)、球麻痹其次,为8例(16.7%)、肢体肌无力5例(10.4%)、肌萎缩1例(2.1%)。老年MG患者中,全身型比例(70.8%)显著高于眼肌型(29.2%),较少合并自身免疫性疾病和发生危象,常合并胸腺病变(17例)及其他内外科疾病(36例),其中15例为胸腺瘤。伴胸腺病变的老年MG患者出现首发症状的年龄与无胸腺病变的老年MG患者比较差异无统计学意义;而伴胸腺病变的老年MG患者需住院治疗时的年龄显著小于无胸腺病变的老年MG患者,差异有统计学意义(P0.05)。结论老年MG具有其自身特点,男性多见,早期表现多为眼肌无力,全身型比例高于眼肌型,胸腺病变主要为胸腺瘤,危象发生率较低等临床特点。     

A study of cyclic nucleotide acids in the serum in patients with myasthenia gravis]

The levels of cAMP and cGMP in serum of 100 patients with myasthenia gravis have been measured by 125I-RIA. The results showed that cAMP and cGMP were higher in the patients than in the controls significantly (P < 0.001). In 33 cases of these patients after using anticholinesterase drugs and prednisone, the levels of cAMP and cGMP reduced significantly. According to our study, we presumed that there would be cyclic nucleotide acid metabolic disturbances in patients with MG and cAMP might play a regulatory role in morbidity of MG.     

Lifetime course of myasthenia gravis

Between 1940 and 2000 a total of 1976 patients with myasthenia gravis (MG) were studied. Diagnosis was made by improvement in weakness after anticholinesterase medication. The historical developments in diagnosis and treatment of MG are reviewed. We analyzed the clinical course of MG as influenced by age, gender, thymectomy, thymomectomy, and the presence of antibodies to acetylcholine receptors (AChR). The clinical course of MG was significantly influenced by age and gender, and these need special attention in managing patients. The most severe level of weakness and high mortality occurred during the first 1 to 2 years of the disease, after which many patients experienced improvement. For treating MG patients the usefulness of thymectomy remains to be proven, and novel drugs need to be developed to increase the number as well as normal functioning of the AChRs and other components of the neuromuscular system.     

Dysphonia as a primary manifestation in myasthenia gravis (MG): a retrospective review of 7 cases among 1520 MG patients

Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission, usually recognized with ocular complaints or generalized muscle weakness. However, among the 1520 MG cases that had been diagnosed and treated in our hospital in the last 15 years (1990–2005), we have identified 7 MG patients whose initial and prominent complaint was dysphonia and all had been misdiagnosed elsewhere. The diagnoses were confirmed with fibrolaryngoscope and voice analysis employed before and after a positive neostigmine (anticholinesterase) test. Electromyography with repetitive stimulations, single-fiber electromyography, and laboratory and radiographic evaluations were also conducted for diagnosis. A surprisingly low seropositivity rate of anti-acetylcholine-receptor antibodies (1/7) and anti-MuSK (Muscle Specific Kinase) antibodies (0/6) were found in these dysphonia MG patients. A cholinesterase inhibitor (ChEI) and immunosuppressive therapy were applied for treatment. Extended thymectomy was applied to MG patients with thymus hyperplasia or thymic tumor. Significant improvement was found in all 7 cases after these treatments. We have developed a sere of diagnostic protocol for this rare type of laryngeal MG, and discussed the clinical implication of our data. In summary, dysphonia or laryngeal disorder can be the only prominent manifestation of MG in rare cases, which should be taken into consideration during the diagnosis to patients with exclusive laryngeal complaints.     

肋间神经重复电刺激在激素冲击治疗重症肌无力中的应用

目的探讨肋间神经重复电刺激(IRNS)和膈神经重复电刺激(PRNS)对激素冲击治疗时重症肌无力(MG)患者呼吸受累的预测价值.方法治疗开始前3天内检测36例MG患者PRNS和IRNS,同时观察用力肺活量(FVC)、MG临床评分、治疗中临床呼吸症状变化.结果大剂量激素治疗后2～13天14例(40%)患者出现呼吸功能受累或原有呼吸困难加重,呼吸功能恶化患者与未恶化患者相比,上述参数及MG临床类型均有明显差异.Lo-gistic回归分析显示3Hz及5Hz的IRNS双侧波幅衰减均值超过30%时比不超过时发生呼吸困难或原有呼吸困难加重的相对危险度均为19.523.结论治疗中呼吸功能受累与上述指标及MG临床分型均有关系,IRNS可以预测是否发生呼吸功能恶化.     

Long-term spinal reflex studies in awake behaving mice

The increasing availability of genetic variants of mice has facilitated studies of the roles of specific molecules in specific behaviors. The contributions of such studies could be strengthened and extended by correlation with detailed information on the patterns of motor commands throughout the course of specific behaviors in freely moving animals. Previously reported methodologies for long-term recording of electromyographic activity (EMG) in mice using implanted electrodes were designed for intermittent, but not continuous operation. This report describes the fabrication, implantation, and utilization of fine wire electrodes for continuous long-term recordings of spontaneous and nerve-evoked EMG in mice. Six mice were implanted with a tibial nerve cuff electrode and EMG electrodes in soleus and gastrocnemius muscles. Wires exited through a skin button and traveled through an armored cable to an electrical commutator. In mice implanted for 59-144 days, ongoing EMG was monitored continuously (i.e., 24 h/day, 7 days/week) by computer for 18-92 days (total intermittent recording for 25-130 days). When the ongoing EMG criteria were met, the computer applied the nerve stimulus, recorded the evoked EMG response, and determined the size of the M-response (MR) and the H-reflex (HR). It continually adjusted stimulation intensity to maintain a stable MR size. Stable recordings of ongoing EMG, MR, and HR were obtained typically 3 weeks after implantation. This study demonstrates the feasibility of long-term continuous EMG recordings in mice for addressing a variety of neurophysiological and behavioral issues.     

脑损伤后发作性自主神经功能障碍的临床特点

目的探讨脑损伤后发作性自主神经功能障碍的临床特点。方法回顾性分析22例脑损伤后发作性自主神经功能障碍患者的临床资料。结果22例患者均处于植物状态,其原发损伤为重型颅脑外伤14例,脑或小脑出血血肿清除术后2例,海洛因中毒性脑病1例,重度一氧化碳中毒2例,电击伤心肺复苏术后1例,冠状动脉造影支架植入术心脏骤停复苏后1例,麻醉意外致心脏骤停心肺复苏后1例。全组患者均出现发作性躁动、发热、多汗、呼吸急促、心动过速、血压升高、肌张力障碍、抽搐等症状中的多项表现。发作时脑电图未见癫疒间放电;听觉诱发电位或体感诱发电位显示各波潜伏期延长、波幅下降甚至未引出。神经影像学检查提示大脑皮质、皮质下、脑干等部位有不同程度的损害。治疗上应用多巴胺受体激动剂或阻滞剂、苯二氮艹卓类及肌松剂等药物对症处理;22例中10例在起病后1~13个月脱离植物状态。结论脑损伤后发作性自主神经功能障碍临床主要表现为发作性自主神经功能紊乱伴肌张力障碍,重症者多处于植物状态;药物治疗仅限于对症处理。     

Cyclosporine in the treatment of myasthenia gravis

Cyclosporine A (CsA) treatment was evaluated in 52 patients with severe generalized myasthenia gravis (MG) whose illness was not controlled by anticholinesterase drugs, thymectomy, corticosteroids, and azathioprine. The efficacy of CsA treatment was expressed by mean disability score quotient (MDSQ), which was obtained by comparing mean disability score (MDS) at the beginning of the treatment with the MDS at the end of the follow-up period. For the entire group of patients MDSQ was 53.3%, indicating moderate improvement. Analyzing individual cases, eight patients (15%) did not improve, 17 (33%) showed moderate improvement, 20 (38%) showed remarkable improvement, and seven patients (14%) achieved complete remission. The most common side effects were rise of serum creatinine (seven), hypertension (two), gingival hyperplasia (two), hypertrichosis (six), myalgia (10), and 'flu-like' symptoms (10 patients). The results of this study suggest that CsA is efficacious and safe treatment in severe and resistant forms of MG.     

Lambert-Eaton肌无力综合征45例临床及电生理回顾性分析

目的 通过对我院Lambert-Eaton肌无力综合征(Lambert-Eaton myasthenia syndrome,LEMS)患者的回顾性分析,分析此病的临床表现和伴发的神经电生理异常.方法 总结1993-2008年我院诊断的45例LEMS患者的一般情况、神经系统临床表现和体征、伴发的内科和全身疾病的情况.所有患者均进行神经电生理检测,包括神经传导速度(NCV)和重复神经电刺激(RNS).部分患者行针极肌电图和皮肤交感反射(SSR)检查.结果 (1)患者出现神经系统症状的平均年龄为(51.2±6.8)岁.最常见临床表现为双下肢无力(35例),其后依次为双上肢无力(10例)、构音障碍(3例)和颈肌无力(2例).神经系统体征中最常见为双下肢或上肢轻度力弱(40例),双下肢腱反射或跟反射减低或消失(38例)以及口干或便秘等自主神经症状(30例).(2)神经电生理检查:所有患者尺神经高频刺激递增达156% ～636%,其中29例同时出现低频递减.所有患者行NCV检查,感觉神经传导速度(SCV)异常或SCV合并运动神经传导速度异常者19例(42%).30例患者行针极肌电图检查,有异常发现者20例.25例行SSR的患者中发现13例异常.结论 LEMS最常见的临床症状为双下肢无力,其次为自主神经症状.患者除了有重复神经电刺激的异常,还伴有周围神经和肌肉的电生理异常以及自主神经系统的异常,提示临床表现可能混杂有神经或肌肉病变的原因.     

非胸腺瘤性眼外肌麻痹起病重症肌无力胸腺切除时机探讨

目的比较非胸腺瘤性眼外肌麻痹起病重症肌无力(ocular onset myasthenia gravis,OMG-O)患者于不同病程行胸腺切除后病情缓解与向全身型重症肌无力(GMG)转化的差异,以期对手术时机进行探讨。方法采用双向队列研究方法,对作者医院2005年1月至2017年9月因药物治疗效果不佳而行胸腺切除的86例OMG-O患者,按起病至手术时间间隔分为早手术组(≤1年)、晚手术组(>1年),比较两组患者不同随访时间理想状态(干预后状态达到轻微异常及更好)率、GMG转化率的差异。结果 (1)与早手术组比较,晚手术组少年MG(<18岁)比例较高,起病至手术时间间隔、起病至使用激素时间间隔、激素用药时间较长(均P <0.05)。(2)与晚手术组比较,早手术组术后第3年理想状态率高(P=0.029),而第2、4~8年理想状态率有升高趋势,但差异无统计学意义(P>0.05)。(3)9例术后发生GMG转化,起病至发生GMG转化时间中位数17.8个月,其中6例(66.7%)在3年内发生GMG转化。早手术组与晚手术组间GMG转化率比较,差异无统计学意义(12.5%比8.7%;χ~2=0.049,P=0.825)。结论对于药物治疗无效或不耐受的OMG-O患者,早期行胸腺切除手术疗效较好;但即使手术,仍有可能发生GMG转化,约1/2患者于起病后1.5年内发生,约2/3于起病后3年内发生。     

Experimental myasthenia gravis induced in mice by passive transfer of human myasthenic immunoglobulin : Evidence for an ameliorating effect by alpha-fetoprotein

The induction of experimental autoimmune myasthenia gravis (EAMG) was studied by the passive transfer of gamma-globulin from myasthenia gravis (MG) patients to C57BL/6 mice. Muscular weakness and electromyographic decrements (EMG) could be consistently induced in all mice injected with gamma-globulin from certain selected MG patients. There was, however, no correlation between the antiacetylcholine receptor antibody titre in the donor gamma-globulin and the ability to induce EAMG. The possible beneficial effects of immunoregulatory alpha-fetoprotein (AFP) treatment were investigated employing the passive EAMG model. Mice were protected against the onset of severe symptoms provided the AFP was administered before and after passive transfer. The exaggerated fatigue characteristics associated with murine EAMG as detected by EMG could be alleviated by AFP treatment. These findings raise the possibility that AFP may be of some therapeutic value in the control of MG.