重症肌无力行胸腺切除术后合并视神经脊髓炎1例

重症肌无力（myasthenia gravis,MG）和视神经脊髓炎（neuromyelitis optica,NMO）均为较少见的神经系统自身免疫性疾病,且两病极少在同一个体先后出现。首都医科大学宣武医院神经内科收治1例重症肌无力患者行胸腺切除术后4个月合并视神经脊髓炎患者,现报道如下。     

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.     

NMO-IgG positive neuromyelitis optica in a patient with myasthenia gravis but no thymectomy

Here we report on a 44-year old woman presenting with both myasthenia gravis (MG) and neuromyelitis optica (NMO). MRI showed transverse myelitis extending from C2 to T4, multifocal demyelinating lesions in the supratentorial white matter, and left optic neuritis. Serological analysis demonstrated antibodies to acetylcholine receptors as well as NMO-IgG. To our knowledge, this is the first case of NMO-IgG positive NMO in a patient with MG but no history of thymectomy or immunosuppression.     

胸腺切除对重症肌无力患者T淋巴细胞亚型的影响

目的：观察胸腺切除(Tx)术对重症肌无力(MG)病人的临床疗效及对T淋巴细胞亚型的影响。方法：用许氏评分法观察30例伴胸腺增生或胸腺瘤的MG患者的病情严重程度及Tx术后2个月的临床疗效;采用直接免疫荧光染色和流式细胞仪技术测定60名志愿健康者和30例伴胸腺增生或胸腺瘤的MG患者Tx术前及术后2个月T淋巴细胞亚型的变化。结果：伴胸腺增生或胸腺瘤的MG病人Tx术前外周血中CD4＋T淋巴细胞的百分率较正常人显著增多(P＜0．01),CD8＋T淋巴细胞的百分率较正常人显著减少(P＜0．01),CD4＋/CD8＋T细胞的比例明显增高(P＜0．01)。伴胸腺增生MG病人Tx术后随着临床症状的改善,CD8＋T淋巴细胞的百分率较术前显著升高(P＜0．05),CD4＋/CD8＋T细胞的比例较术前显著下降(P＜0．05)。伴胸腺瘤MG病人Tx术后随着临床症状的改善,CD4+T淋巴细胞的百分率较术前显著下降(P＜0．05),CD4＋/CD8＋T细胞的比例较术前显著下降(P＜0．05)。结论：重症肌无力患者T淋巴细胞亚型的测定既可以为MG免疫病理学的发病机理的研究提供实验依据,也能为Tx治疗MG提供一个客观的实验室指标,为判断疾病的转归提供实验依据。     

Response to thymectomy in South Indian patients with myasthenia gravis

Aims – The rate of remission among patients with myasthenia gravis (MG) following thymectomy and the predictors of the outcome have revealed vast variation in studies from different geographic regions raising suspicion about the influence of ethnic factors. Material & methods – We retrospectively evaluated the outcome of 71 South Indian MG patients who were thymectomized between 1987 through 1993 and analyzed the relationship between clinical and histopathological features and postthymectomy outcome. Results – The clinical severity of the disease did not differ between the 29 patients with and 42 patients without a thymoma. Seventynine percent of our patients responded favourably to thymectomy; without additional immunosupression therapy, 52% achieved a near-complete remission. An younger age and milder disease correlated with a good outcome. Patients with thymoma responded as favourably as those without a thymoma. Conclusions – The postthymectomy response of South Indian MG patients in general did not differ from that of Western and Oriental patients.     

Prognosis of thymectomy in myasthenia gravis patients with thymus hyperplasia

Purpose: To compare the post-thymectomy prognosis in different conditions of myasthenia gravis (MG) patients with thymus hyperplasia. Materials and methods: Collecting medical record and carrying out the follow-up study of 123 myasthenia gravis patients with thymus hyperplasia who have underwent thymectomy during the period between 2003 and 2013. Dividing into different groups based on gender, age of onset, duration of disease and Myasthenia Gravis Association of America (MGFA) clinical classification to analyze different prognosis in different groups. Results: Complete stable remission (CSR) was achieved in 71 of 123 patients (59.5%). There is no gender-related difference in achieving CSR. Patients with early onset of MG (≤40 years old) or disease duration less than 12 months had significantly higher CSR rates than those with late onset of MG (>40 years old) or disease duration more than 12 months respectively, while no difference was found in remission rate between MGFA clinical classification I and MGFA II. Conclusion: Myasthenia gravis patients with thymus hyperplasia who had thymectomy are proved to possess greater chance of achieving CSR. The onset age of disease and duration are the prognostic factors.     

Indications for thymectomy in myasthenia gravis

Fifty-six board-certified neurologists with interest and expertise in myasthenia completed a survey of indications for thymectomy in myasthenia gravis. Thymectomy was advocated for virtually all patients with thymoma, for a variable subset of patients with generalized myasthenia without thymoma, and occasionally for selected patients with disabling ocular myasthenia.     

198例胸腺切除的重症肌无力临床分析

目的探讨重症肌无力患者胸腺的病理改变与临床的关系。方法回顾分析1990年1月至2005年1月哈尔滨医科大学附属第一及第二医院收治的行胸腺切除术的重症肌无力198例,研究胸腺的病理改变与重症肌无力临床特点的关系及影响重症肌无力手术治疗效果的因素。结果198例重症肌无力患者伴胸腺增生158例,胸腺瘤34例。前者Osserman分型以Ⅰ、Ⅱ型为主;后者Osserman分型以Ⅱa、Ⅱb为主,病理分型以上皮细胞型和混合细胞型为主,Masaoka分期以Ⅰ、Ⅱ期为主。伴胸腺增生与胸腺瘤的重症肌无力患者临床特点不同。年龄、病程、胸腺病理改变及临床分型是影响手术疗效的因素。结论重症肌无力的临床特点与胸腺的病理改变有一定的相关性,胸腺切除是治疗重症肌无力一种较为有效的方法,术后疗效受多种因素影响。     

重症肌无力患者围手术期呼吸肌训练的效果

目的 观察围手术期呼吸肌训练在重症肌无力术后恢复中的效果.方法 选取2016-01—2019-12在郑州大学第二附属医院胸外科行胸腔镜胸腺扩大切除治疗的30例重症肌无力患者为研究对象,2016-01—2017-12手术患者为对照组,术前仅常规术前准备;2018-01—2019-12手术患者为实验组,常规术前准备加围手术...     

Repeat thymectomy in chronic refractory myasthenia gravis

Encouraged by recent results with "extended" thymectomy in the treatment of myasthenia gravis, we carried out repeat thymectomy in six patients with chronic, refractory disease who did not initially have extended thymectomy. All were completely disabled with longstanding myasthenia. Initial thymectomy (four transsternal, one transcervical, and one substernal) was carried out at a mean of 8.9 years previously (range, 5 to 18). There was no residual thymus observed with CT, but at repeat thymectomy, residual thymic tissue was present in five of six patients. Five patients significantly improved and four returned to full-time work. Mean prednisone dose declined from 51 mg to 18 mg/d, and mean pyridostigmine dose fell from 1,290 mg to 415 mg/d. No patient to date has had a complete remission. These results suggest that repeat thymectomy may benefit some patients with chronic disabling myasthenia gravis, especially when it is uncertain from a review of the operative report whether all thymic tissue was removed at the initial thymectomy.     

Video-assisted thoracoscopic thymectomy in juvenile myasthenia gravis

As video-assisted thoracoscopic surgery for thymectomy has been reported to be as effective as traditional open surgical approaches in predominantly adult patients with myasthenia gravis, we applied this procedure to juvenile patients with this condition. Six patients underwent total thymectomy using the video-assisted technique (1997-98). Six patients in whom a median transsternal approach was used (1989-95) formed the comparison group. The two patient groups were similar in terms of age at thymectomy and preoperative clinical severity. There were no serious perioperative complications in either group. Minimum post-thymectomy duration of follow-up in the video-assisted thoracoscopic surgery patients was 2.3 years (mean 2.7 years), with all patients clinically improved over their baseline status. Preliminary results suggest that video-assisted thymectomies are comparably effective to transsternal procedures in treating generalized juvenile myasthenia gravis and can be safely performed in children as young as 20 months of age. In addition, video-assisted surgeries are less invasive than transsternal approaches, significantly shorten the postoperative hospital stay, and have superior cosmetic results.     

Outcomes after robotic thymectomy in nonthymomatous versus thymomatous patients with acetylcholine-receptor-antibody-associated myasthenia gravis

The aim of this study was to investigate the surgical and long-term neurological outcomes of patients with acetylcholine-receptor-antibody-associated myasthenia gravis (AChR-MG) who underwent robotic thymectomy (RATS). We retrospectively analyzed the clinical-pathological data of all patients with AChR-MG who underwent RATS using the DaVinci® Robotic System at the MUMC+ between April 2004 and December 2018. Follow-up data were collected from 60 referring Dutch hospitals. In total, 230 myasthenic patients including 76 patients with a thymoma (33.0%) were enrolled in this study. Mean follow-up time, procedure time and hospitalization were, respectively 65.7 ± 43.1 months, 111±52.5 min and 3.3 ± 2.2 days. Thymomatous patients had significantly more frequently and more severe complications than nonthymomatous patients (18.4% vs. 3.9%, p<0.001). Follow up data was available in 71.7% of the included patients. The Myasthenia Gravis Foundation of America postintervention score showed any kind of improvement of MG-symptoms after RATS in 82.4% of the patients. Complete stable remission (CSR) or pharmacological remission (PR) of MG was observed in 8.4% and 39.4% of the patients, respectively. Mean time till CSR/PR remission after thymectomy was 26.2 ± 29.2 months. No statistical difference was found in remission or improvement in MGFA scale between thymomatous and nonthymomatous patients. RATS is safe and feasible in patients with MG. The majority of the patients (82.4%) improved after thymectomy. CSR and PR were observed in 8.4% and 39.4% of the patients, respectively, with a mean of 26.2 months after thymectomy. Thymomatous patients had more frequently and more severe complications compared to nonthymomatous patients.     

Juvenile seropositive myasthenia gravis with anti-MuSK antibody after thymectomy

We report the case of a 17.5-year old girl with generalized myasthenia gravis (MG). When she was 13, she started to complain of episodic diplopia, ptosis and mild fatigability of upper and lower extremity muscles. She was diagnosed with MG 3 years later, after exacerbation of her limb muscle weakness. Acetylcholine receptor antibody was positive (2.56 nmol/l, n < 0.4 nmol/l), anti-MuSK antibodies were not tested at that time. She was treated with a thymectomy. After the operation a gradual worsening of her MG was seen; she reported pirydostygmine intolerance. On retesting, acetylcholine receptor antibodies were negative and she was positive for anti-MuSK antibodies. She received immunosuppressive treatment with prednisone and azathioprine with clinical improvement. She is the third reported patient diagnosed with acetylcholine receptor antibody-positive MG, who became MuSK-positive after thymectomy. Recognition of such clinical patterns may be important for decision making in myasthenia gravis patients.