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    Inclusion of the transcervical approach in video-assisted thoracoscopic extended thymectomy (VATET) for myasthenia gravis: a prospective trial. Shigemura N,Shiono H,Inoue M,Minami M,Ohta M,Okumura M,Matsuda H Surgical endoscopy BACKGROUND:Because evidence-based data regarding the quality of video-assisted thoracoscopic thymectomy for the treatment of myasthenia gravis are lacking, a prospective trial comparing three different operative approaches was conducted to evaluate their efficacy. METHODS:This prospective study enrolled 20 consecutive patients with nonthymomatous myasthenia gravis. A series of three approaches for bilateral video-assisted thoracoscopic extended thymectomy (VATET) using the anterior chest wall-lifting method (original), the original method with a flexed-neck position (modified), and the original method with a transcervical approach (final) were prospectively performed in each patient for quantitative and pathologic evaluation of the residual thymus after each approach. RESULTS:Complete VATET required 242 +/- 48 min, with the transcervical procedure requiring 23 +/- 12 min. After the modified method, the residual thymus in the cervical region was 1.5 cm in size and weighed 0.8 g (0.8% of the entire thymus), as compared with a size of 2.2 cm and a weight of 1.3 g (3.2%) after the original method. Each value is the result of comparison with the final method. Histopathologic studies showed residual tissue in the germinal center as well as Hassall's corpuscles in more than 70% of cases. CONCLUSION:The findings show that VATET without the transcervical approach could be an immunologically incomplete treatment for myasthenia gravis. Therefore, the transcervical approach should be included in VATET procedures to ensure radicality. 10.1007/s00464-005-0614-7
    Thoracoscopic thymectomy with the da Vinci robotic system for myasthenia gravis. Rückert Jens C,Ismail Mahmoud,Swierzy Marc,Sobel Holger,Rogalla Patrik,Meisel Andreas,Wernecke Klaus D,Rückert Ralph I,Müller Joachim M Annals of the New York Academy of Sciences Complete thymectomy (Thx) is a crucial part of treatment for myasthenia gravis (MG) and thymoma. The discussion about the necessity of radical, complete Thx and reduced invasiveness has led to no less than 14 different surgical approaches for Thx. The latest development is robotic-assisted surgery. Though its impact on minimally invasive surgery is not yet clear, it seems to be most promising for surgery in remote, narrow anatomical regions like the mediastinum. One hundred six consecutive robotic-assisted thymectomies (rThx) with the da Vinci robotic surgical system were performed between January 2003 and April 2007 in a prospective single-center study. Postoperative morbidity was recorded according to the Myasthenia Gravis Foundation of America (MGFA) classification. With zero mortality, the overall postoperative morbidity rate was 2%. The cumulative complete stable remission rate of MG was > 40% for all patients, and there was no statistical difference as compared to non-thymomatous MG patients. The cumulative rate of minimal manifestations (MM0-MM3) according to the MGFA classification showed a postoperative improvement in quality of life for most of the patients. The da Vinci robotic system allowed for technical refinements of the well-defined operation technique of thoracoscopic Thx (tThx). From the technical point of view, rThx has advantages for mediastinal dissection. rThx had a shorter learning curve. There might be better outcome results for rThx in MG patients, as compared with nonrobotic tThx. Therefore, rThx is a promising technique for minimally invasive Thx. 10.1196/annals.1405.013
    Extended transcervical thymectomy in the treatment of myasthenia gravis. Khicha Sanjay G,Kaiser Larry R,Shrager Joseph B Annals of the New York Academy of Sciences The ideal operative technique for thymectomy in myasthenia gravis remains controversial. Most surgeons perform thymectomy via median sternotomy, some supplementing this with an even more extensive mediastinal and cervical dissection designed to remove all areas of possible ectopic thymic tissue. We and others have advocated a transcervical approach that is less morbid and costly than sternotomy approaches. The transcervical approach allows a complete extracapsular thymic resection, but it does not address all areas of potential ectopic thymic tissue. We have published our experience with 151 extended transcervical thymectomies (TCT). At mean follow-up of 53 months (complete follow-up in 97%), Kaplan-Meier estimates of complete stable remission were 33% and 35% at 3 and 6 years. If one includes patients who became asymptomatic but remained on low dose, single-drug immunosuppression as complete remissions (CRs), then the CR rates were 43% and 45% at 3 and 6 years. Longer term (mean 83 months) follow-up of the earliest 84 patients in the series showed preserved CR rates. On multivariate analysis, only preoperative Osserman Class (group mean 2.3) was significantly associated with improved CR rate. These results were obtained with a major operative complication rate of 0.7% and minor complication rate of 6.6%, and nearly every operation was performed without the need for overnight hospital admission. We believe that these response rates following TCT are sufficiently similar to those following transsternal techniques of thymectomy to allow us to recommend this less morbid and less costly operation as an eminently reasonable choice in the surgical treatment of myasthenia gravis. 10.1196/annals.1405.006
    Video-assisted thoracic surgery thymectomy for nonthymomatous myasthenia gravis. Manlulu Anthony,Lee Tak Wai,Wan Innes,Law Chun Yat,Chang Carlin,Garzon Juan Carlos,Yim Anthony Chest STUDY OBJECTIVES:Minimal-access thymectomy has become increasingly popular as surgical treatment for patients with nonthymomatous myasthenia gravis (NTMG) because of its comparable efficacy, safety, and lesser degree of tissue trauma compared with conventional open surgery. We reviewed and analyzed our data on video-assisted thoracic surgery (VATS) thymectomy and present the clinical outcomes according to the Myasthenia Gravis Foundation of America classification. DESIGN:A retrospective review of VATS thymectomy for NTMG in a university hospital over a 12-year period. Data were collected from the medical records and supplemented with telephone surveys. The impact of surgery and other variables potentially affecting complete stable remission (CSR) were calculated using Kaplan-Meier survival curves; comparisons between survival curves was performed using the log-rank test. RESULTS:A total of 38 consecutive patients underwent VATS thymectomy for NTMG. Median postoperative stay was 3 days. Pathologic examination revealed thymic hyperplasia in 61.1% of cases, normal thymus in 22.2%, and thymic atrophy in 16.6%. There was no perioperative mortality; complications occurred in four patients. After a median follow-up of 69 months, 91.6% of patients experienced improvement, with crude CSR achieved in 22.2%. Kaplan-Meier survival curve demonstrated a 75% CSR rate at 10-year follow-up. On univariate analysis, only disease duration < or = 12 months (p = 0.03) was associated with a statistically significant improvement in CSR. CONCLUSIONS:VATS thymectomy for NTMG results in symptomatic improvement in the vast majority of patients, with a high rate of CSR. The procedure is associated with low morbidity and no perioperative mortality. Future studies on thymectomy for myasthenia gravis should be reported in a standardized manner to allow accurate comparisons between results in the absence of randomized prospective trials. 10.1378/chest.128.5.3454
    International consensus guidance for management of myasthenia gravis: Executive summary. Sanders Donald B,Wolfe Gil I,Benatar Michael,Evoli Amelia,Gilhus Nils E,Illa Isabel,Kuntz Nancy,Massey Janice M,Melms Arthur,Murai Hiroyuki,Nicolle Michael,Palace Jacqueline,Richman David P,Verschuuren Jan,Narayanaswami Pushpa Neurology OBJECTIVE:To develop formal consensus-based guidance for the management of myasthenia gravis (MG). METHODS:In October 2013, the Myasthenia Gravis Foundation of America appointed a Task Force to develop treatment guidance for MG, and a panel of 15 international experts was convened. The RAND/UCLA appropriateness methodology was used to develop consensus guidance statements. Definitions were developed for goals of treatment, minimal manifestations, remission, ocular MG, impending crisis, crisis, and refractory MG. An in-person panel meeting then determined 7 treatment topics to be addressed. Initial guidance statements were developed from literature summaries. Three rounds of anonymous e-mail votes were used to attain consensus on guidance statements modified on the basis of panel input. RESULTS:Guidance statements were developed for symptomatic and immunosuppressive treatments, IV immunoglobulin and plasma exchange, management of impending and manifest myasthenic crisis, thymectomy, juvenile MG, MG associated with antibodies to muscle-specific tyrosine kinase, and MG in pregnancy. CONCLUSION:This is an international formal consensus of MG experts intended to be a guide for clinicians caring for patients with MG worldwide. 10.1212/WNL.0000000000002790
    A propensity score analysis for comparison of T-3b and VATET in myasthenia gravis. Brenna Greta,Antozzi Carlo,Montomoli Cristina,Baggi Fulvio,Mantegazza Renato, Neurology OBJECTIVE:We performed propensity score (PS) models to compare the outcome of patients with myasthenia gravis (MG) submitted to 2 different surgical approaches: extended transsternal (T-3b) or thoracoscopic extended thymectomy (VATET). METHODS:Patients' clinical data were retrieved from the MG database of the C. Besta Neurologic Institute Foundation. In the PS analysis, a matching ratio of 1:1 of the main clinical variables was obtained for the 2 groups of patients and treatment effect was estimated by comparing their outcome. RESULTS:A total of 210 patients met the inclusion criteria, by having a complete set of clinical data, and were included in the PS model; a matched dataset of 122 participants (61 per group) showed an adequate balance of all the covariates. Our analysis demonstrated that 68.9% of patients who had thymectomy by the VATET technique reached the pharmacologic remission/remission status at 2 years from thymectomy compared to 34.4% of those operated on by the T-3b technique ( < 0.001), had a lower INCB-MG score ( < 0.001), and had less muscle fatigability ( = 0.004). Similar results were found considering only nonthymomatous patients with MG. Results were also confirmed by paired statistical tests. CONCLUSIONS:Our PS matching analysis showed that VATET is a reliable and effective surgical approach alternative to T-3b in patients with MG who are candidates for thymectomy. CLASSIFICATION OF EVIDENCE:This study provides Class IV evidence that for patients with MG, VATET is more effective than T-3b thymectomy. 10.1212/WNL.0000000000004082
    Ten-year results of thoracoscopic unilateral extended thymectomy performed in nonthymomatous myasthenia gravis. Tomulescu Victor,Sgarbura Olivia,Stanescu Codrut,Valciu Crisanda,Campeanu Ana,Herlea Vlad,Popescu Irinel Annals of surgery OBJECTIVE:The aim of this study was to analyze the 10-year results of thoracoscopic unilateral extended thymectomy (TUET) performed in nontumoral myasthenia gravis according to the Myasthenia Gravis Foundation of America recommendations. BACKGROUND DATA:Thoracoscopic unilateral extended thymectomy has the benefits of a minimally invasive approach. Previous data have shown promising midterm results but long-term results were lacking. METHODS:Two hundred forty patients with nontumoral myasthenia gravis who underwent surgery between 1999 and 2009 were eligible for the study. The mean follow-up was of 67 months (range: 12-125), 134 patients completed follow-up assessments more than 60 months after TUET. RESULTS:There were 39 males (16.3%) and 201 females (83.7%), with an age range from 8 to 60 years. The mean preoperative disease duration was 21.5 months. All patients underwent preoperative steroid therapy. Anticholinesterase drugs were required for 123 patients (51.3%), and immunosuppressive drugs were required for 87 (36.3%) patients. The pathologic findings were as follows: normal thymus in 13 patients (5.5%), involuted thymus in 65 patients (27%), and hyperplastic thymus in 162 patients (67.5%). The average weight of the thymus was 110 ± 45 g. Ectopic thymic tissue was found in 147 patients (61.3%). There was no mortality, and morbidity consisted of 12 patients (5%). Complete stable remission was achieved in 61% of the patients, and the cumulative probability of achieving complete stable remission was 0.88 at 10 years. CONCLUSIONS:With zero mortality, low morbidity, and comparable long-term results to open surgery, TUET can be regarded as the best treatment option for patients undergoing surgery for myasthenia gravis. 10.1097/SLA.0b013e31823686f6
    Intravenous immunoglobulin in autoimmune neuromuscular diseases. Dalakas Marinos C JAMA CONTEXT:Intravenous immunoglobulin (IVIG) enhances immune homeostasis by modulating expression and function of Fc receptors, interfering with activation of complement and production of cytokines, providing anti-idiotypic antibodies, and affecting the activation and effector functions of T and B cells. These mechanisms may explain the effectiveness of IVIG in autoimmune neuromuscular disorders. OBJECTIVE:To systematically review the current status of the treatment of autoimmune neuromuscular diseases with IVIG, with emphasis on controlled trials. DATA SOURCES:Peer-reviewed publications identified through MEDLINE (1966-2003), EMBASE (1974-2003), and references from bibliographies of pertinent articles. Each autoimmune neuromuscular disease term was searched in combination with the term intravenous immunoglobulin. STUDY SELECTION AND DATA EXTRACTION:Criteria for selection of studies included controlled study design, English language, and clinical pertinence. Data quality was based on venue of publication and relevance to clinical care. DATA SYNTHESIS:Outcomes of controlled trials indicate that IVIG at a total dose of 2 g/kg is effective as first-line therapy in Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy and as second-line therapy in stiff-person syndrome, dermatomyositis, myasthenia gravis, and Lambert-Eaton myasthenic syndrome. In other controlled studies, IVIG produced a modest, variable, and transient but not statistically significant benefit in patients with inclusion body myositis and paraproteinemic anti-myelin-associated glycoprotein antibody demyelinating polyneuropathy. Intravenous immunoglobulin is not effective in patients with multiple sclerosis who have established weakness or optic neuritis. In myasthenia gravis, it should be reserved for difficult cases or before thymectomy in lieu of plasma exchange. CONCLUSION:Intravenous immunoglobulin is effective in many autoimmune neurologic diseases, but its spectrum of efficacy, especially as first-line therapy, and the appropriate dose for long-term maintenance therapy are not fully established. Further controlled studies of IVIG, combined with a dose-finding effect, pharmacoeconomics, and quality-of-life assessments, are warranted to improve the evidence base for clinical practice. 10.1001/jama.291.19.2367
    Thymomas alter the T-cell subset composition in the blood: a potential mechanism for thymoma-associated autoimmune disease. Hoffacker V,Schultz A,Tiesinga J J,Gold R,Schalke B,Nix W,Kiefer R,Müller-Hermelink H K,Marx A Blood Thymomas are the only tumors that are proven to generate mature T cells from immature precursors. It is unknown, however, whether intratumorous thymopoiesis has an impact on the peripheral T-cell pool and might thus be related to the high frequency of thymoma-associated myasthenia gravis. This study shows, using fluorescence-activated cell sorting-based analyses and T-cell proliferation assays, that thymopoiesis and T-cell function in thymomas correspond with immunologic alterations in the blood. Specifically, the proportion of circulating CD45RA(+)CD8(+) T cells is significantly increased in patients with thymoma compared with normal controls, in accordance with intratumorous T-cell development that is abnormally skewed toward the CD8(+) phenotype. Moreover, it is primarily the proportion of circulating CD45RA(+)CD8(+) T cells that decreases after thymectomy. The results also demonstrate that T cells reactive toward recombinant autoantigens are distributed equally between thymomas and blood, whereas T-cell responses to foreign antigen (ie, tetanus toxoid) are seen only among circulating T cells and not among thymoma-derived T cells. These functional studies support the hypothesis that thymopoiesis occurring within thymomas alters the peripheral T-cell repertoire. Because many thymomas are enriched with autoantigen-specific T cells, a disturbance of circulating T-cell subset composition by export of intratumorous T cells may contribute to paraneoplastic autoimmune disease arising in patients with thymoma. (Blood. 2000;96:3872-3879)
    Thymus cells in myasthenia gravis selectively enhance production of anti-acetylcholine-receptor antibody by autologous blood lymphocytes. Newsom-Davis J,Willcox N,Calder L The New England journal of medicine We investigated the role of the thymus in 16 patients with myasthenia gravis without thymoma by studying the production of anti-acetylcholine-receptor antibody by thymic and blood lymphocytes cultured alone or together. In 19 responders (with the highest receptor-antibody titers in their plasma), cultured thymic cells spontaneously produced measurable receptor antibody. Receptor-antibody production by autologous blood lymphocytes was enhanced by the addition of responders' thymic cells, irradiated to abrogate antibody production and suppression (P less than 0.01). This enhancement was greater and more consistent than that by pokeweed mitogen; it depended on viable thymic cells, appeared to be selective for receptor antibody, and correlated with the ratio of thymic helper (OKT4-positive or OKT4+) to suppressor (OKT8+) T cells (P less than 0.01). These results suggest that myasthenic thymus contains cell-bound acetylcholine-receptor-like material or specific T cells (or both) that can aid receptor-antibody production. This may be relevant to the benefits of thymectomy in myasthenia and to the breakdown in self-tolerance in this and other autoimmune diseases. 10.1056/NEJM198111263052203
    Experimental autoimmune thymitis. An animal model of human myasthenia gravis. Goldstein G,Whittingham S Lancet (London, England) 10.1016/s0140-6736(66)92599-2
    Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity. Meriggioli Matthew N,Sanders Donald B The Lancet. Neurology Acquired myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction in which patients experience fluctuating skeletal muscle weakness that often affects selected muscle groups preferentially. The target of the autoimmune attack in most cases is the skeletal muscle acetylcholine receptor (AChR), but in others, non-AChR components of the neuromuscular junction, such as the muscle-specific receptor tyrosine kinase, are targeted. The pathophysiological result is muscle endplate dysfunction and consequent fatigable muscle weakness. Clinical presentations vary substantially, both for anti-AChR positive and negative MG, and accurate diagnosis and selection of effective treatment depends on recognition of less typical as well as classic disease phenotypes. Accumulating evidence suggests that clinical MG subgroups might respond differently to treatment. In this Review, we provide current information about the epidemiology, immunopathogenesis, clinical presentations, diagnosis, and treatment of MG, including emerging therapeutic strategies. 10.1016/S1474-4422(09)70063-8
    Neuromuscular disease: Benefit of thymectomy in myasthenia gravis. Fyfe Ian Nature reviews. Neurology 10.1038/nrneurol.2016.129
    Comparison of plasmapheresis and intravenous immunoglobulin as maintenance therapies for juvenile myasthenia gravis. Liew Wendy K M,Powell Christine A,Sloan Steven R,Shamberger Robert C,Weldon Christopher B,Darras Basil T,Kang Peter B JAMA neurology IMPORTANCE:Juvenile myasthenia gravis (MG) is a relatively rare autoimmune disorder. The comparative efficacy of plasmapheresis (PLEX) vs immunoglobulin as maintenance therapy is unclear for this childhood disease. OBJECTIVE:To determine whether PLEX or intravenous immunoglobulin (IVIG) therapy is more effective as maintenance therapy in this disease. DESIGN, SETTING, AND PARTICIPANTS:This retrospective analysis over a 33-year period involved 54 children and adolescents with juvenile MG at a specialized neuromuscular clinic and electromyography laboratory at a tertiary care academic pediatric hospital. INTERVENTIONS:Plasmapheresis and IVIG. MAIN OUTCOMES AND MEASURES:Response to treatment was measured by both improvement in objective physical examination findings and the patients’ reported improvement in symptoms and functional abilities. RESULTS:Subjective and objective outcomes correlated well. Both PLEX and IVIG had high response rates. Of the 27 patients with generalized juvenile MG receiving PLEX, IVIG, or both treatments, 7 of 7 patients treated with PLEX alone responded, 5 of 10 patients treated with IVIG alone responded, and 9 of 10 patients who received both responded. There was a significant difference in response rates between patients who received PLEX vs IVIG (P = .04). The youngest age at which PLEX was initiated via peripheral venous access was 9 years, while the youngest child who received IVIG was 9 months old. Thymectomy was performed in 17 children, of whom 11 experienced significant postoperative improvement. CONCLUSIONS AND RELEVANCE:This study provides class III evidence that PLEX and IVIG both have high response rates as maintenance therapies and are reasonable therapeutic options for juvenile MG. Plasmapheresis may have a more consistent response rate than IVIG in this setting. These findings will provide some guidance regarding the approach to therapy for juvenile MG, especially as the results differ somewhat from those of studies focusing on adult MG. 10.1001/jamaneurol.2014.17
    Is thymectomy necessary in nonmyasthenic patients with early thymoma? Tseng Yen-Chiang,Hsieh Chih-Cheng,Huang Hsin-Yi,Huang Chien-Sheng,Hsu Wen-Hu,Huang Biing-Shiun,Huang Min-Hsiung,Hsu Han-Shui Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer BACKGROUND:In thymoma patients without myasthenia gravis, it is debatable whether thymectomy should be performed in addition to thymomectomy, the procedure in which the thymoma alone is resected. In this study, we proposed to compare the surgical results in early-stage nonmyasthenic thymoma patients who underwent thymomectomy with and without extended thymectomy. METHODS:A total of 95 patients without clinical evidence of preoperative myasthenia gravis, who underwent surgery for early-stage thymoma (stages I and II), were selected for the study. Thymomectomy with extended thymectomy was performed through median sternotomy on 42 patients, whereas thymomectomy without thymectomy was carried out through video-assisted thoracoscopic surgery (VATS) or thoracotomy in 53 patients. Outcomes and surgical complications were compared between the two patient groups. RESULTS:The median duration of the follow-up was 57 months (6-121 months). Three patients, one in the thymomectomy group (1.9%) and two in the thymomectomy with thymectomy group (4.5%), developed tumor recurrences. Tumor recurrence rates between the two groups were not significantly different. During the follow-up period, we did not document the development of postoperative myasthenia gravis in any of the patients enrolled. Postoperative opioid use, the number of days of drainage, and hospitalization length were lower in patients undergoing thymomectomy through thoracotomy or VATS. CONCLUSIONS:In early-stage nonmyasthenic thymoma patients, thymomectomy without thymectomy through thoracotomy or VATS was associated with lower morbidity and shorter hospitalization, than thymomectomy with extended thymectomy. Postoperative myasthenia gravis did not develop in any of the patients enrolled in our study during the 57-month median follow-up period. Overall tumor recurrence rates were not significantly different between these two patient groups. On the basis of our results, we conclude that thymomectomy without thymectomy through thoracotomy or VATS is justified for early-stage nonmyasthenic thymoma patients, and longer follow-up is needed to investigate the necessity of thymectomy in this group. 10.1097/JTO.0b013e31828cb3c2
    Thymectomy in myasthenia gravis: when, why, and how? Berrih-Aknin Sonia,Le Panse Rozen The Lancet. Neurology 10.1016/S1474-4422(18)30467-8
    Myasthenia gravis - autoantibody characteristics and their implications for therapy. Gilhus Nils Erik,Skeie Geir Olve,Romi Fredrik,Lazaridis Konstantinos,Zisimopoulou Paraskevi,Tzartos Socrates Nature reviews. Neurology Myasthenia gravis (MG) is an autoimmune disorder caused by autoantibodies that target the neuromuscular junction, leading to muscle weakness and fatigability. Currently available treatments for the disease include symptomatic pharmacological treatment, immunomodulatory drugs, plasma exchange, thymectomy and supportive therapies. Different autoantibody patterns and clinical manifestations characterize different subgroups of the disease: early-onset MG, late-onset MG, thymoma MG, muscle-specific kinase MG, low-density lipoprotein receptor-related protein 4 MG, seronegative MG, and ocular MG. These subtypes differ in terms of clinical characteristics, disease pathogenesis, prognosis and response to therapies. Patients would, therefore, benefit from treatment that is tailored to their disease subgroup, as well as other possible disease biomarkers, such as antibodies against cytoplasmic muscle proteins. Here, we discuss the different MG subtypes, the sensitivity and specificity of the various antibodies involved in MG for distinguishing between these subtypes, and the value of antibody assays in guiding optimal therapy. An understanding of these elements should be useful in determining how to adapt existing therapies to the requirements of each patient. 10.1038/nrneurol.2016.44
    Myasthenia gravis: subgroup classification and therapeutic strategies. Gilhus Nils Erik,Verschuuren Jan J The Lancet. Neurology Myasthenia gravis is an autoimmune disease that is characterised by muscle weakness and fatigue, is B-cell mediated, and is associated with antibodies directed against the acetylcholine receptor, muscle-specific kinase (MUSK), lipoprotein-related protein 4 (LRP4), or agrin in the postsynaptic membrane at the neuromuscular junction. Patients with myasthenia gravis should be classified into subgroups to help with therapeutic decisions and prognosis. Subgroups based on serum antibodies and clinical features include early-onset, late-onset, thymoma, MUSK, LRP4, antibody-negative, and ocular forms of myasthenia gravis. Agrin-associated myasthenia gravis might emerge as a new entity. The prognosis is good with optimum symptomatic, immunosuppressive, and supportive treatment. Pyridostigmine is the preferred symptomatic treatment, and for patients who do not adequately respond to symptomatic therapy, corticosteroids, azathioprine, and thymectomy are first-line immunosuppressive treatments. Additional immunomodulatory drugs are emerging, but therapeutic decisions are hampered by the scarcity of controlled studies. Long-term drug treatment is essential for most patients and must be tailored to the particular form of myasthenia gravis. 10.1016/S1474-4422(15)00145-3
    RetroSternal--Looking Back at Thymectomy for Myasthenia Gravis. Ropper Allan H The New England journal of medicine 10.1056/NEJMe1607953
    Thymectomy for myasthenia gravis: video-assisted versus transsternal. Lo Chien-Ming,Lu Hung-I,Hsieh Ming-Jang,Lee Shao-Shuan,Chang Jen-Ping Journal of the Formosan Medical Association = Taiwan yi zhi BACKGROUND/PURPOSE:Thymectomy may benefit patients with generalized myasthenia gravis (MG) or myasthenia with thymomas. Currently, video-assisted (VA) thoracic surgery is popular and plays an important role in thymectomy. We compared the clinical outcomes of VA thymectomy with conventional transsternal (TS) approach to investigate the effectiveness of VA technology in the current era. METHODS:A retrospective review of our thymectomy results for patients with MG from 1998 to 2011 was conducted. A total of 83 consecutive patients were enrolled. According to the surgeons' and patients' preference, 39 patients received conventional TS thymectomy and 44 were operated on in a VA fashion. The results were categorized and analyzed according to the Myasthenia Gravis Foundation of America (MGFA) postintervention statuses with some modification. RESULTS:No obvious difference between conventional TS thymectomy and VA thymectomy was noted regarding the remission statuses and the clinical outcomes by the modified MGFA score. However, the patients who received VA thymectomy showed shorter intensive care unit (ICU) stays (4.2 ± 3.3 days vs. 2.3 ± 1.5 days, p = 0.001). CONCLUSION:Compared with the conventional TS thymectomy, the VA approach is similarly effective with shorter ICU stays. 10.1016/j.jfma.2014.05.010
    Comparative clinical outcomes after thymectomy for myasthenia gravis: Thoracoscopic versus trans-sternal approach. Siwachat Sophon,Tantraworasin Apichat,Lapisatepun Worakitti,Ruengorn Chidchanok,Taioli Emanuela,Saeteng Somcharoen Asian journal of surgery BACKGROUND:Thymectomy is an effective treatment option for long-term remission of myasthenia gravis. The superiority of the trans-sternal and thoracoscopic surgical approaches is still being debated. The aims of this study are to compare postoperative outcomes and neurologic outcomes between the two approaches and to identify prognostic factors for complete stable remission (CSR). METHODS:Myasthenia gravis patients who underwent thymectomy with trans-sternal or thoracoscopic approach in MahaRaj Nakorn Chiang Mai Hospital, Chiang Mai, Thailand between January1, 2006 and December 31, 2013 were retrospectively reviewed. The endpoints were postoperative outcomes and cumulative incidence function for CSR. The analysis was performed using multilevel model, Cox's proportional hazard model, and propensity score. RESULTS:Ninety-eight patients were enrolled in this study: 53 in the thoracoscopic group and 45 in the trans-sternal group. There were no significant differences between groups in composite postoperative complications, surgical time, ventilator support days, and length of intensive care unit stay. Intraoperative blood loss and length of hospital stay were significant less in the thoracoscopic group. The CSR and median time to remission were not significantly different between the two approaches. Prognostic factors for CSR were nonthymoma (hazard ratio: 3.5, 95% confidence interval: 1.01-12.22) and presence of pharmacological remission (hazard ratio: 24.3, 95% confidence interval: 3.27-180.41). CONCLUSION:Thoracoscopic thymectomy is safe and provides good neurologic outcomes in comparison to the trans-sternal approach. Two predictive factors should be considered for CSR. Further prospective studies with a larger sample size and longer follow-up period are warranted to confirm these results. 10.1016/j.asjsur.2016.09.006
    Predictive factors for myasthenic crisis after videoscopic thymectomy in patients with myasthenia gravis. Lee Hyung Seok,Lee Hye Sun,Lee Hyo Eun,Bae Mi Kyung,Chung Kyung Young,Shin Ha Young,Choi Young-Chul,Kim Seung Min Muscle & nerve INTRODUCTION:Predictive factors for myasthenic crisis after transsternal thymectomy have been reported, but little is known about myasthenic crisis after videoscopic thymectomy (MCAVT). METHODS:We investigated 146 myasthenia gravis patients who underwent videoscopic thymectomy. RESULTS:Patients with MCAVT had a lower forced vital capacity (FVC) (2.1 vs. 3.0 L, P < 0.001) than those without. Low-frequency repetitive nerve stimulation showed decremental responses of the orbicularis oculi (47.1% vs. 18.1%, P = 0.001) and nasalis muscles (54.1% vs. 21.4%, P < 0.001), which were more pronounced in patients with MCAVT than those without. According to multivariate analysis, FVC (OR 0.144, 95% confidence interval [CI], 0.044-0.479, P = 0.002) and decremental response of orbicularis oculi (odds ratio, 1.029; 95% CI, 1.001-1.058, P = 0.044) were independently associated with MCAVT. CONCLUSIONS:FVC and decremental response of orbicularis oculi were associated with MCAVT. 10.1002/mus.24531
    Intravenous immunoglobulin to prevent myasthenic crisis after thymectomy and other procedures can be omitted in patients with well-controlled myasthenia gravis. Gamez Josep,Salvadó María,Carmona Francesc,de Nadal Miriam,Romero Laura,Ruiz Daniel,Jáuregui Alberto,Martínez Olga,Pérez Javier,Suñé Pilar,Deu María Therapeutic advances in neurological disorders Background:Myasthenic crisis (MC) is a potentially life-threatening complication of myasthenia gravis. Its precipitating factors include surgical procedures, particularly thymectomy. The role of preoperative intravenous immunoglobulin (IVIg) in preventing MC in patients scheduled for thymectomy and other surgery with general anaesthesia is unknown. Our objective was to test the hypothesis that preoperative IVIg is effective in preventing myasthenic crisis in patients with myasthenia gravis scheduled for surgery under general anaesthesia, including thymectomy. Methods:A prospective, randomized, double-blind, single-centre study was conducted over a 4-year period. The treatment group received IVIg, 0.4 g/kg/day preoperatively for 5 consecutive days, and the placebo group received saline solution under the same conditions. The two groups were age-matched, with similar functional status, and Myasthenia Gravis Foundation of America class. All patients had well-controlled myasthenia gravis with minimal manifestations before surgery. The primary outcome measured was MC. Intubation times, time in the recovery room, number of postoperative complications, and days of hospitalization were the secondary outcomes measured. Results:A total of 47 patients were randomized, 25 to the IVIg group and 22 to placebo. There were 19 men and 28 women, with a mean age of 58.6 years, mean body mass index of 27.8 kg/m, and mean acetylcholine receptor antibodies of 12.9 nmol/l. The mean forced vital capacity was 84.4%. The mean quantitative myasthenia gravis sum score was 6.3. Ten patients (five in each arm) had a history of MC. Thymectomy was performed in 16 patients. Only one patient in the placebo group presented with MC requiring non-invasive ventilation (but no reintubation) for 6 days. Neither differences between groups in the univariate analysis nor risk factors for MC in the multivariate analysis were found. Conclusions:Preoperative IVIg to prevent MC does not appear to be justified in well-controlled myasthenia gravis patients. This study provides class I evidence that preparation with IVIg to prevent MC is not necessary in well-controlled myasthenia gravis patients scheduled for surgery with general anaesthesia. 10.1177/1756286419864497
    Thymectomy in myasthenia gravis: proposal for a predictive score of postoperative myasthenic crisis. Leuzzi Giovanni,Meacci Elisa,Cusumano Giacomo,Cesario Alfredo,Chiappetta Marco,Dall'armi Valentina,Evoli Amelia,Costa Roberta,Lococo Filippo,Primieri Paolo,Margaritora Stefano,Granone Pierluigi European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery OBJECTIVES:Thymectomy plays an important role in patients with myasthenia gravis (MG). This study aimed to explore predictors of postoperative myasthenic crisis (POMC) after thymectomy and to define a predictive score of respiratory failure. METHODS:The clinical data of 177 patients with MG undergoing thymectomy from January 1995 to December 2011 were retrospectively reviewed. The following factors were analysed in relation to the occurrence of myasthenic crisis: gender, age, body mass index (BMI), anti-acetylcholine receptor-antibody level, bulbar symptoms, comorbidities, duration of symptoms, Osserman-stage, Myasthenia Gravis Foundation of America (MGFA) stage, history of myasthenic crisis, use of immoglobulins or plasmapheresis, kind of therapy, spirometric and blood gas parameters, histology, kind of surgery, non-myasthenic complications and duration of intubation. RESULTS:Twenty-two patients experienced postoperative respiratory failure after thymectomy. Univariate analysis revealed a correlation with age >60 years (odds ratio (OR) = 1.79, 95% confidence interval (CI) = 1.04-6.78; P = 0.040); Osserman-stage (IIB- OR = 5.16, 95% CI = 1.10-24.18; P = 0.037, III-IV- OR = 8.75, 95% CI = 1.53-50.05; P = 0.015); bulbar symptoms (OR = 7.42, 95% CI = 1.67-32.84; P = 0.008); BMI >28 (OR = 3.99, 95% CI = 1.58-10.03; P = 0.003); preoperative plasmapheresis (OR = 2.97, 95% CI = 1.18-14.04; P = 0.021); duration of symptoms >2 years (OR = 4.00, 95% CI = 1.09-14.762; P = 0.036); extended surgery (OR = 2.52, 95% CI = 1.02-6.22; P = 0.045); lung (OR = 4.05, 95% CI = 1.44-11.42; P = 0.008), pericardial (OR = 3.78, 95% CI = 1.45-9.82; P = 0.006) or pleural resection (OR = 3.23, 95% CI = 1.30-8.03; P = 0.012); Vital Capacity % <80% (OR = 0.20, 95% CI = 0.05-0.82; P = 0.025) and PaCO2 >40 mmHg (OR = 3.76, 95% CI = 1.12-12.68; P = 0.032). Multivariate logistic regression analysis showed that Osserman-stage (IIB- OR = 5.69, 95% CI = 1.09-29.69; P = 0.039 (III-IV- OR = 11.33, 95% CI = 1.67-76.72; P = 0.013), BMI >28 (OR = 3.65, 95% CI = 1.10-12.15; P = 0.035), history of myasthenic crisis (OR = 24.10, 95% CI = 2.34-248.04; P = 0.007), duration of symptoms >2 years (OR = 5.94, 95% CI = 1.12-31.48; P = 0.036) and lung resection (OR = 8.48, 95% CI = 2.18-32.97; P = 0.002) independently predict POMC. Excluding history of preoperative myasthenic crisis (statistically associated with Osserman-stage), we built a scoring system according to the OR of Osserman-stage (I-IIA, IIB, III-IV), BMI (<28, ≥ 28), duration of symptoms (<1, 1-2, >2 years) and association with a pulmonary resection. This model helped in creating four classes with increasing risk of respiratory failure (Group I, 6%; Group II, 10%; Group III, 25%; Group IV, 50%). CONCLUSIONS:Our model facilitates the stratification of patient risk and prediction of the occurrence of POMC. Moreover, it could help to guide the anaesthesiologist's decision on the duration of intubation. Further studies based on larger series are needed to confirm these preliminary data. 10.1093/ejcts/ezt641
    Clinical Outcomes of Thymectomy in Myasthenia Gravis Patients with a History of Crisis. Liu Zhenguo,Lai Yingrong,Yao Shiyuan,Feng Huiyu,Zou Jianyong,Liu Weibin,Lei Yiyan,Zhu Hua,Cheng Chao World journal of surgery BACKGROUND:The use of thymectomy in myasthenia gravis (MG) patients with a history of myasthenic crisis (MC) has not been well established. Here, we determined the efficacy of thymectomy by assessing the long-term clinical outcomes and reviewed thymectomy reports on MC patients. METHODS:Subjects included 31 patients who suffered at least one crisis before surgery, with a cumulative total 73 episodes of MC in Southern China between May 2000 and December 2010. Long-term follow-up was performed and clinical outcomes were evaluated. We used complete stable remission (CSR), termed an asymptomatic status without medication for at least 12 months; general complete remission (GCR), termed an asymptomatic status with or without some form of therapy excluding cholinesterase inhibitors, to assess patient outcomes. RESULTS:All patients underwent thymectomy with an overall complication rate of 16.1 % and a perioperative mortality rate of 3.2 %. Long-term follow-up occurred between 12.6 and 177 months, at which point 18 (58.1 %) patients experienced improved status, including one patient who achieved CSR; 13 (41.9 %) patients achieved GCR; 6 (19.4 %) showed unchanged status and one worse (3.2 %) status. The remaining 6 patients died, with 3 due to MG-related causes. Using a multivariate Cox regression analysis of GCR by characteristics, patients with better response to medical treatments before thymectomy were positively associated with GCR rates (p = 0.028). CONCLUSIONS:Extended transsternal thymectomy is a feasible and effective therapy for MG patients with crisis history, especially for those patients who have shown positive signs of remission after exhausting conventional medical treatments. 10.1007/s00268-016-3599-6
    Thymectomy for myasthenia gravis in children: a comparison of open and thoracoscopic approaches. Goldstein Seth D,Culbertson Nicholas T,Garrett Deiadra,Salazar Jose H,Van Arendonk Kyle,McIltrot Kimberly,Felix Michelle,Abdullah Fizan,Crawford Thomas,Colombani Paul Journal of pediatric surgery PURPOSE:Thymectomy is an accepted component of treatment for myasthenia gravis (MG), but optimal timing and surgical approach have not been determined. Though small series have reported the feasibility of thoracoscopic resection, some studies have suggested that minimally invasive methods are suboptimal compared to open sternotomy owing to incomplete clearance of thymic tissue. Here we report the largest series of thymectomies for pediatric myasthenia gravis in the literature to date. METHODS:A retrospective review of patients undergoing thymectomy for MG between 1990 and 2013 in a tertiary referral hospital was performed. Twelve patients who underwent thoracoscopic thymectomy were compared to 16 patients who underwent open thymectomy via median sternotomy. Postoperative outcomes were determined by electronic chart review in consultation with the treating pediatric neurologist. Disease severities were graded according to a modified Myasthenia Gravis Foundation of America (MGFA) Quantitative MG (QMG) score. RESULTS:Overall, thoracoscopic resections tended to be performed on patients with earlier and less severe disease than open surgeries. Inpatient length of stay was significantly shorter after thoracoscopic surgery (mean 1.8 vs 8.0 days, p=0.045). The preoperative and postoperative MGFA QMG scores were equivalent between the two groups. Both groups experienced a decrease in disease severity (p<0.001) after median follow-up time of 23 months in the thoracoscopic group and 44 months in the open group. CONCLUSIONS:Minimally invasive thymectomy for MG in children has increased in popularity as surgeons and neurologists compare the risks and benefits of surgery against other therapies. This analysis suggests that thoracoscopic thymectomy is not inferior to median sternotomy in terms of disease control in this small series, and that the morbidity of the thoracoscopic approach appears sufficiently low to be considered for early stage disease. Low perioperative morbidity and shortened hospital course make thoracoscopic thymectomy an attractive option in centers with sufficient medical and surgical experience. 10.1016/j.jpedsurg.2014.10.005
    Outcomes of thoracoscopic thymectomy in patients with juvenile myasthenia gravis. Ashfaq Awais,Bernes Saunder M,Weidler Erica M,Notrica David M Journal of pediatric surgery INTRODUCTION:Myasthenia gravis (MG) is an autoimmune disorder of the postsynaptic neuromuscular junction resulting in fatigability of voluntary muscles. There has been increasing evidence supporting thymectomy for MG in adults, and evidence for the role of surgery in pediatric age groups is increasing. The purpose of this study is to describe the outcomes of our patients with juvenile MG undergoing thoracoscopic thymectomy. MATERIAL AND METHODS:All patients with juvenile MG who underwent thoracoscopic thymectomy at Phoenix Children's Hospital between 1999 and 2014 were included. Patients were diagnosed by their treating neurologist. An Osserman and Genkins criterion was used to classify the severity of the disease and DeFilippi classification was used to assess remission. RESULTS:Twelve patients underwent thoracoscopic thymectomy for juvenile MG during the time frame studied. Nine (75%) patients had an Osserman stage of IIB, with only two patients with ocular disease. There were no conversions to an open procedure. Seven (59%) patients had normal thymic histology, 4 (33%) had evidence of follicular hyperplasia and one (8%) had involutional changes. The median length of hospital stay was 2days (range 1-5days). There was no 30-day postoperative morbidity, reoperations or mortality. The median length of follow-up was 31months (range, 4-91months) and at the time of their last follow-up; all 12 (100%) patients had a DeFilippi Classification of 3 or better. CONCLUSION:Surgery for MG in children is indicated for antibody-receptor-positive patients with moderate to severe disease. Thoracoscopic thymectomy is a safe and acceptable treatment for juvenile MG with good disease control. The low morbidity and shorter hospital duration make it an excellent option for consideration. 10.1016/j.jpedsurg.2015.12.016
    Optimal timing of thymectomy in nonthymomatous myasthenia gravis patients in China: A prospective study. Pan Jinglin,Jiang Qilong,Liu Fengbin,Wen Yi Medicine INTRODUCTION:While thymectomy is a recommended therapy for patients with Myasthenia Gravis (MG), there is insufficient evidence of its benefits over other therapies in patients in China, specifically, or of the most optimal timing for the procedure. Thus, there remains a clinical need for the investigation of these questions. Therefore, it is important to compare the clinical efficacy of thymectomy plus oral prednisone, an immunosuppressant protocol with prednisone, or immunosuppressants alone. CONCLUSION:We propose here to prospectively assess 822 cases of MG and 1886 medical records from individuals hospitalized at the First Affiliated Hospital of Guangzhou University of Chinese Medicine and follow them for 3 years. Inclusion criteria will include the following: a Myasthenia Gravis Foundation of America (MGFA) clinical classification between I and IV while on optimal anticholinesterase therapy with or without oral prednisone or immunosuppressive therapy, an MG history of longer than 3 years, being 18 to 60 years of age, and positive testing for serum acetylcholine receptor antibodies (AchR-Ab). Both thymomatous-naïve and non-naïve participants will be included. The primary outcomes will be: mortality, frequency of myasthenic crises, MGFA classification, and changes to the required dose of prednisone and immunosuppressants. Based on these outcomes, we will evaluate the efficacy of thymectomy as well as oral drugs in managing patients with nonthymomatous MG. As of September 2017, this study has been approved by the ethics committee of the First Affiliated Hospital of Guangzhou University of Chinese Medicine and the Registration number is ChiCTR1800017564(Version1.0, September 8,2017). 10.1097/MD.0000000000012499
    Effect of thymectomy for thymic atrophy in myasthenia gravis: A retrospective study on 93 patients. Nakahara Keiichi,Nakane Shunya,Nakajima Makoto,Yamashita Satoshi,Mori Takeshi,Ando Yukio Journal of neuroimmunology To clarify the efficacy of thymectomy among myasthenia gravis (MG) patients with and without thymoma. We classified MG patients who underwent thymectomy into 3 groups, such as thymic atrophy group, thymic follicular hyperplasia (TFH) group and thymoma group. We compared the data of clinical features and postoperative prognosis at very short-term, short-term, and medium-term. The clinical course of MG patients with atrophic thymus after thymectomy was even better than those of TFH or thymoma, in this retrospective study. However, we found no significant differences in the comparison of mean dose of prednisolone between the 3 groups at each time point. 10.1016/j.jneuroim.2017.01.005
    Robotic thymectomy in patients with myasthenia gravis: neurological and surgical outcomes. Keijzers Marlies,de Baets Marc,Hochstenbag Monique,Abdul-Hamid Myrurgia,Zur Hausen Axel,van der Linden Marcel,Kuks Jan,Verschuuren Jan,Kessels Fons,Dingemans Anne-Marie C,Maessen Jos European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery OBJECTIVES:Thymectomy is frequently used in the treatment of myasthenia gravis (MG). But indication, timing or surgical approach remain controversial. This study reports our experiences with robotic thymectomy and surgical and neurological outcomes in a large cohort of patients with MG. METHODS:We retrospectively analysed the outcome of 125 patients with MG who underwent a robotic thymectomy using the da Vinci Surgical System (Intuitive Surgical, Inc., Sunnyvale, CA, USA) between 2004 and 2012. The Myasthenia Gravis Foundation of America (MGFA) Classification was used to determine preoperative and postintervention status. RESULTS:Ninety-five women and 30 men underwent a robotic thymectomy. One hundred patients had a neurological follow-up of more than 12 months. Preoperative most severe MGFA classification was Stage I in 11 patients (8.8%), Stage IIA in 18 patients (14.4%), Stage IIB in 18 patients (14.4%), Stage IIIA in 7 patients (5.6%), Stage IIIB in 29 patients (23.2%), Stage IVA in 10 patients (8.0%), Stage IVB in 29 patients (23.2%) and Stage V in 3 patients (2.4%). Median surgical procedure time was 123 min (range 45-353 min). There were no major perioperative complications or deaths. The median postoperative hospital stay was 3 days (range 2-24 days). Histological analysis showed thymic remnant tissue in 41 patients (32.8%), follicular hyperplasia in 52 patients (41.6%), thymoma in 31 patients (24%), lipoma in 1 patient (0.8%) and a cyst in 1 patient (0.8%). Patients with thymic remnant tissue were significantly more preoperative steroid users compared with the follicular hyperplasia group (P = 0.02). With a median follow-up of 33 months (range 12-104 months), 77% of the patients showed neurological improvement. Three-year probability remission rate [complete stable remission (CSR) and pharmacological remission] is 28.2%. Patients who were not treated with prednisolone preoperatively showed a significant higher CSR than patients who did take prednisolone (P = 0.014). No significant difference was observed regarding timing of surgery (P = 0.37). CONCLUSIONS:Robotic thymectomy in patients with MG is safe and feasible. A neurological benefit and decreased use of steroids can be obtained in the majority of patients. No significant difference in neurological outcome was observed as the result of timing of robot thymectomy after the onset of MG. 10.1093/ejcts/ezu352
    Predictive factors of myasthenic crisis after extended thymectomy for patients with myasthenia gravis. Ando Takeshi,Omasa Mitsugu,Kondo Takayuki,Yamada Tetsu,Sato Masaaki,Menju Toshi,Aoyama Akihiro,Sato Toshihiko,Chen Fengshi,Sonobe Makoto,Date Hiroshi European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery OBJECTIVES:Postoperative myasthenic crisis (POMC) is one of the serious complications after extended thymectomy for patients with myasthenia gravis (MG). This study aims to clarify the risk factors of POMC occurrence. METHODS:The clinical data of 55 MG patients (25 male, 30 female; median age, 51 years) who underwent extended thymectomy at Kyoto University from 2000 to 2013 were retrospectively reviewed. Surgical outcomes and pre- and perioperative predictive factors of POMC were analysed. RESULTS:The preoperative Myasthenia Gravis Foundation of America stage was I, II, III and IV in 24, 22, 8 and 1 patients, respectively. Ten patients (18.2%) developed POMC; 6 required prolonged intubation over 24 h and 4 required reventilatory support. All patients were weaned after 5.6 (2-26) days of ventilator support, and were discharged. Univariate analysis revealed a correlation with a high preoperative anti-acetylcholine receptor antibody titre (P = 0.009), history of myasthenic crisis (MC) (P = 0.0004) and unstable MG after preoperative medical therapy (P = 0.003). Multivariate logistic regression analysis showed history of MC (odds ratio, 11.84; 95% confidential interval, 1.05-372; P = 0.045) and unstable MG (odds ratio, 29.45; 95% confidential interval, 2.00-1063; P = 0.013) independently predicted POMC. The surgical response rate was not significantly different between the two groups (66.7% with POMC, 85.4% without POMC; P = 0.334). CONCLUSIONS:POMC occurred more frequently in unstable MG before surgery or in patients with a history of MC. Adequate preoperative medical therapy and perioperative care should be provided to these patients. 10.1093/ejcts/ezu530
    Value of Adjuvant Radiotherapy for Thymoma with Myasthenia Gravis after Extended Thymectomy. Lu Chang-Feng,Yu Lei,Jing Yun,Zhang Yun-Feng,Ke Ji Chinese medical journal Background:The co-existence of myasthenia gravis (MG) and thymoma makes the surgical treatment more complicated and adjuvant radiation more controversial. The aim of this study was to investigate adjuvant radiotherapy for thymoma with MG after extended thymectomy. Methods:A total of 181 patients with both MG and thymoma were recruited between 2003 and 2014 at Tongren Hospital, China. Among all the patients, 157 patients received radiation therapy after surgery (Group A); whereas the other 24 patients did not receive radiation therapy (Group B). According to the time that patients started mediastinal radiation therapy, we subdivided the 157 patients in Group A into subgroups (1-month subgroup, n = 98; 2-month subgroup, n = 7; and 3-month subgroup, n = 52). We then compared the effect of the mediastinal radiation therapy across these different groups using the survival rate, the rate of postoperative myasthenic crisis, and the complete stable remission (CSR) rate as the primary endpoints. Results:There was a significant difference in the occurrence of postoperative myasthenic crisis between 1-month subgroup and Group B (χ = 4.631, P = 0.031). The rates of reaching CSR were 32.6% in 1-month subgroup, 25% in 3-month subgroup, and 22.7% in Group B, respectively. The overall survival rates of 1-month subgroup, 3-month subgroup, and Group B were 88.8%, 83.3%, and 77.3%, respectively. Analysis on the Kaplan-Meier survival curves demonstrated that within 8 years after surgery, there was no significant difference in aspects of overall survival and disease-free survival between 1-month subgroup and Group B, and between 3-month subgroup and Group B; over 8 years after surgery, the disease-free survival rates in 1-month subgroup, 3-month subgroup and Group B were 79.4%, 70.6%, and 55.3%, respectively. Conclusions:Adjuvant radiation within 1 month after extended thymectomy may be helpful in controlling postoperative MG, such as decreasing the possibility of postoperative myasthenic crisis, and raising cumulative probabilities of reaching CSR. 10.4103/0366-6999.229894
    Preoperative Anxiety in Patients With Myasthenia Gravis and Risk for Myasthenic Crisis After Extended Transsternal Thymectomy: A CONSORT Study. Zou Jianyong,Su Chunhua,Lun Xueping,Liu Weibing,Yang Weiling,Zhong Beilong,Zhu Haoshuai,Lei Yiyan,Luo Honghe,Chen Zhenguang Medicine A thymectomy can ameliorate the symptoms of myasthenia gravis (MG) and prevent the progression of ocular MG (OMG) to generalized MG (GMG). However, postoperative myasthenic crisis (POMC) is a serious post-thymectomy complication. Preoperative anxiety (POA) is common but typically neglected in MG patients. The association of POA with POMC has not yet been examined.From June 2007 to December 2013, 541 cases of MG were admitted to the First Affiliated Hospital of Sun Yat-sen University (Guangzhou, China). All cases underwent extended transsternal thymectomy (ETT). The clinical and pathological characteristics of these patients, including POA and POMC, were analyzed.A total of 179 patients experienced POA and 67 patients experienced POMC. Patients with POA were more likely to have POMC, a thymoma, and an ectopic thymus. Univariate analysis showed that POMC correlated with POA, presence of an ectopic thymus, dose of pyridostigmine bromide (PYR), presence of a thymoma, MGFA stage, preoperative myasthenic crisis, and postoperative pneumonia. Multivariate logistic regression analysis showed that the independent risk factors for POMC were POA, preoperative myasthenic crisis, higher dose of PYR, and postoperative pneumonia.Our results suggest that clinicians should consider the risk factors for POMC-especially preoperative anxiety-before performing a thymectomy in patients with MG. 10.1097/MD.0000000000002828
    A comparison between IVIG and plasma exchange as preparations before thymectomy in myasthenia gravis patients. Alipour-Faz Athena,Shojaei Maziar,Peyvandi Hassan,Ramzi Davood,Oroei Mahbobeh,Ghadiri Fereshteh,Peyvandi Maryam Acta neurologica Belgica Myasthenia gravis (MG) is one of the curable neurologic disorders. Various pharmacological therapies are administered for these patients and a thymectomy plays an important role in the therapy of myasthenia gravis, which develops a permanent or relative remission. We investigated the efficacy of intravenous immunoglobulin (IVIG) and plasma exchange (PLEX) as a preparation before thymectomy in patients with MG. This randomized clinical trial was conducted on 24 patients with MG referred for thymectomy, which were randomized to two groups of IVIG and PLEX. The IVIG group received IVIG 1 g/kg/day for two consecutive days and the PLEX group underwent 1-L plasma exchange five times with 5 % albumin replacement fluid, every other day, 10-30 days before the procedure. The duration of hospitalization (day), length of intensive care unit (ICU) stay after surgery (day), length of intubation period (h), duration of surgery (h) and dose of steroid administered were compared between the two groups. Analysis was performed via SPSS version 20. In the PLEX group, post-operative outcomes (duration of hospitalization, ICU length of stay after surgery, intubation period and duration of surgery) were longer than those in the IVIG group. There was significant difference in intubation period (p value = 0.01) and duration of surgery (p value = 0.05) between the PLEX and IVIG groups. The administration of IVIG in comparison to PLEX can be more effective in the preparation before thymectomy in myasthenia gravis patients. 10.1007/s13760-016-0689-z
    Robotic thymectomy for myasthenia gravis with or without thymoma-surgical and neurological outcomes. Kumar Arvind,Goyal Vinay,Asaf Belal B,Trikha Anjan,Sood Jayashree,Vijay C L Neurology India Context (Background): We report our experience with robotic thymectomy in patients with myasthenia gravis (MG)and provide data on the surgical results and neurologic outcomes, as per the Myasthenia Gravis Foundation of America (MGFA) recommendations for MG clinical research standards. OBJECTIVE:The study aims at reporting the surgical and neurological outcomes of patients of Myasthenia gravis treated by robotic thymectomy. MATERIALS AND METHODS:Prospective data was collected from 71 patients with myasthenia gravis (in the age range 15-67 years) with or without thymoma, who had completed a minimum follow up of one year. All patients were treated with robotic radical thymectomy. The clinical classification, status of preoperative and postoperative therapy, evaluation of post-interventional clinical status, and descriptions of morbidity/mortality were done as per the MGFA recommendations. Univariate and multivariate analysis was done to assess the factors associated with achievement of complete stable remission(CSR). RESULTS:A total of 71 patients were included in this study. Twenty-one out of 71 patients (29.6%) with myasthenia gravis had thymoma. At the last follow up, 70 patients were alive. No evidence of tumour recurrence was found in patients with thymoma. The overall CSR rate was 38% with the median time to CSR of 17.5 months (range 11-48 months). The CSR rate for patient of MG with thymoma was 19 % (n=4/21). Factor found to be significantly predicting CSR were young age, lesser severity of MG and non-thymomatous histology. CONCLUSIONS:Robotic thymectomy is a technically feasible and safe operation with a low morbidity and short hospitalization. It is associated with good neurological long-term results in terms of both CSR and clinical improvement. 10.4103/0028-3886.198211
    Different neurologic outcomes of myasthenia gravis with thymic hyperplasia and thymoma after extended thymectomy: A single center experience. Zheng Yu,Cai Yi-Zhou,Shi Zi-Yan,Qiu Yan,Zhang Han-Lu,Wang Zi-Hao,Li Wen-Bin,Wang Yun Journal of the neurological sciences This study aimed to reveal the clinical course and outcomes of myasthenia gravis (MG) in patients with thymic lymphoid hyperplasia and thymoma undergoing extended thymectomy and to identify the clinical prognostic factors of remission for MG. In total, 73 patients undergoing extended thymectomy were divided into two groups: group A with lymphoid hyperplasia (n=39) and group B with thymoma (n=34). According to the MG Foundation of America (MGFA) post-intervention status, the primary endpoint was a composite measure defined as achievement of complete stable remission (CSR), pharmacologic remission (PR), minimal manifestations (MM) or improvement (IM). The secondary endpoint was CSR. The cumulative probabilities of reaching the primary endpoint were 71.8% in group A and 85.3% in group B (p=0.164), respectively. Using Kaplan-Meier survival analysis, the probability of reaching the primary endpoint in group B was remarkably greater than group A (p=0.036). Cox multivariate analysis indicated that pre-operative MGFA class I (HR: 3.019, 95% CI: 1.084-8.410) and MGFA II (2.665, 95% CI: 1.033-6.873) compared to MGFA III and presence of thymoma (HR: 2.229, 95% CI: 1.079-4.606), showed the most consistent association with remission of MG after thymectomy. Finally, thymic lymphoid hyperplasia and severe symptoms may negatively affect prognosis of MG following thymectomy. 10.1016/j.jns.2017.10.026
    A bilateral approach to extended thymectomy using the da Vinci Surgical System for patients with myasthenia gravis. Kawaguchi Koji,Fukui Takayuki,Nakamura Shota,Taniguchi Tetsuo,Yokoi Kohei Surgery today PURPOSE:We adopted a bilateral approach to complete robotic extended thymectomy with the excision of the pericardial fat tissue from both sides and analyzed the initial outcomes. METHODS:The patient cart was docked first from the left shoulder side. After dissection of the thymus and right pericardial fat tissue, the cart was temporarily rolled out, and the bed was rotated approximately 90° clockwise. The cart was then re-docked from the right-side shoulder, and extended thymectomy was performed via the left-side approach. The outcomes were compared with four cases of unilateral approach performed for mediastinal tumor in the same term. RESULTS:Four patients with myasthenia gravis (two of whom had stage I thymoma) underwent extended thymectomy by the bilateral approach. The mean operative time was 288 min, and the console time was 146 min in the right side and 67 min in the left side. The resected thymus and surrounding adipose tissue were almost symmetrical, in contrast with those obtained via the unilateral approach. No remarkable events were noted. CONCLUSION:Bilateral extended thymectomy for myasthenia gravis patients was safe and reasonable based on the initial outcomes. 10.1007/s00595-017-1567-x
    Eight-year follow-up of patients with myasthenia gravis after thymectomy. Yu S,Li F,Chen B,Lin J,Yang M,Fu X,Li J,Bu B Acta neurologica Scandinavica PURPOSE:To depict the long-term outcome of patients with myasthenia gravis after thymectomy in combination with immunotherapy, and the factors that may potentially affect the outcome. METHODS:The 306 patients with myasthenia gravis who underwent extended thymectomy from January 1984 to December 2011 at Tongji Hospital were retrospectively evaluated. RESULTS:The patients consisted of 174 cases with thymoma and 132 cases without thymoma. Pharmaceutical treatment was tailored for each case during follow-up. Nine patients with thymomatous myasthenia gravis died during the perioperative period, and 297 patients were followed for 8.6 years. By their latest visits, 241 patients (81.1%) gained satisfactory efficacy, 24 cases died (8.1%), and 32 cases (10.8%) remained unchanged or deteriorated. Favorable factors for satisfactory efficacy included the presence of ocular myasthenia gravis before operation, no presence of thymoma, and lack of concomitant diseases. It is interesting to mention that, patients with non-thymomatous myasthenia gravis obtained significantly higher rates of complete stable remission and clinical remission than the patients with thymomatous myasthenia gravis. CONCLUSIONS:Extended thymectomy combined with immunotherapy is a preferred treatment with a satisfactory long-term remission rate. Patients with non-thymomatous myasthenia gravis have a much more promising prognosis than the patients with thymomatous myasthenia gravis. However, appropriate caution must be taken to discontinue pharmaceutical therapy as relapse remains a major concern after a patient who has already undergone thymectomy becomes symptom-free. 10.1111/ane.12289
    Benefits of Comprehensive Rehabilitation Therapy in Thymectomy for Myasthenia Gravis: A Propensity Score Matching Analysis. Ambrogi Vincenzo,Mineo Tommaso Claudio American journal of physical medicine & rehabilitation OBJECTIVE:To demonstrate the effectiveness of a comprehensive program of rehabilitation therapy in patients undergoing thymectomy for myasthenia gravis (MG). DESIGN:From 2005 to 2010, 46 consecutive patients affected by MG underwent a rehabilitation program both before and after thymectomy. We matched each patient with a "control patient" who underwent thymectomy within the period 1999 to 2004 with no preoperative rehabilitation, who had the closest propensity score matching. RESULTS:All patients but 2 were able to complete the intended program. Eighteen patients (41%) experienced mild fatigue (>25 at MG quantitative score). Propensity score selected a group of 17 patients for the matching process. The group of patients who underwent the rehabilitation program showed significant preoperative improvement associated with a reduced operative risk, a decreased early postoperative morbidity, a lower rate of postoperative intensive care unit needed (12% vs 35%; P = 0.01) and a shorter hospital stay (3 vs 5 days; P = 0.04). After the expected perioperative decline, all major myasthenic outcomes demonstrated a significant faster recovery at 3 months. Complete stable remission did not reveal significant differences. CONCLUSIONS:Exercise is not necessarily a contraindication in MG, and rehabilitation can be safely performed before and after thymectomy, reducing operative risks and decreasing recovery time. TO CLAIM CME CREDITS:Complete the self-assessment activity and evaluation online at http://www.physiatry.org/JournalCMECME OBJECTIVES: Upon completion of this article, the reader should be able to do the following: (1) appreciate the benefits of physical therapy in individuals with myasthenia gravis; (2) describe the benefits of physical therapy on postoperative morbidity in myasthenia gravis patients who undergo thymectomy; and (3) incorporate appropriate rehabilitation into the treatment plan of patient with myasthenia gravis. LEVEL:AdvancedACCREDITATION: The Association of Academic Physiatrists is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.The Association of Academic Physiatrists designates this activity for a maximum of 1.5 AMA PRA Category 1 Credit(s)™. Physicians should only claim credit commensurate with the extent of their participation in the activity. 10.1097/PHM.0000000000000538
    The role of thymectomy in the treatment of juvenile myasthenia gravis: a systematic review. Madenci Arin L,Li George Z,Weil Brent R,Zurakowski David,Kang Peter B,Weldon Christopher B Pediatric surgery international BACKGROUND:The role of thymectomy in the treatment of juvenile myasthenia gravis (JMG) is poorly defined. The objective of this systematic review was to evaluate the effect of thymectomy on survival, disease severity, and peri-operative complications for patients with JMG. METHODS:A search of MEDLINE, EMBASE, and the Cochrane Library (1/1/2000-3/1/2016) identified all English language, human studies of thymectomy for JMG. The population was patients with JMG age ≤18 years who underwent thymectomy (comparator group was unexposed to thymectomy). Outcomes included survival, disease severity, and post-operative complications. Data extraction was performed by independent reviewers. RESULTS:Sixteen retrospective studies included 1131 participants with JMG and 488 (43%) underwent thymectomy. Post-operative improvement in JMG severity occurred for 77% (n = 376/488). Comparisons of thymectomy to non-operative management were mixed. Post-operative complications were poorly recorded. Power to compare surgical approaches was limited. Outcomes specific to antibodies, surgical pathology findings, severity of JMG, and timing of thymectomy were sparse. CONCLUSIONS:Existing data regarding thymectomy for JMG are limited and entirely retrospective. The majority of patients who underwent thymectomy had improvement in disease severity and post-operative complications were rare. Prospective, multicenter study of thymectomy for JMG is warranted. 10.1007/s00383-017-4086-3
    Risk factors of myasthenic crisis after thymectomy for thymoma patients with myasthenia gravis. Xue Liang,Wang Lin,Dong Jihong,Yuan Yunfeng,Fan Hong,Zhang Yi,Wang Qun,Ding Jianyong European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery OBJECTIVES:Total thymectomy should be performed on thymoma patients with myasthenia gravis. The aim of the present study was to investigate the risk factors of postoperative myasthenic crisis (POMC) occurrence in these patients. METHODS:The clinical records of 127 thymoma patients with myasthenia gravis (68 men, 59 women; median age, 50 years) who underwent total thymectomy at our institution from 2005 to 2014 were retrospectively reviewed. The following factors were analysed in relation to POMC: gender, age, duration of symptoms, bulbar symptoms, smoking history, history of myasthenic crisis, comorbidities, perioperative pyridostigmine and prednisolone therapy, spirometric and blood gas parameters, Osserman stage, operation approach, major complications, World Health Organization (WHO) histologic classification, Masaoka stage and use of immunoglobulins or plasmapheresis. RESULTS:Thirteen patients (10%) experienced POMC and required intubation. All patients were weaned after 2-28 days (median 9 days) and were discharged. Univariate analysis revealed a correlation between POMC and Osserman-stage IIA-IV [odds ratio (OR) = 4.928, 95% confidence interval (CI) = 1.286-18.882, P = 0.01], bulbar symptoms (OR = 3.828, 95% CI = 1.112-13.176, P = 0.04), and forced expiratory volume in one second <70% pred forced expiratory volume in one second (OR = 4.856, 95% CI = 1.380-17.081, P = 0.02). In addition, more frequent POMC occurred in WHO type B2-B3 than in type A-B1 thymomas (OR = 8.118, 95% CI = 1.020-64.590, P = 0.03). Multivariate logistic regression analysis showed that WHO histologic classification B2-B3 (OR = 10.041, 95% CI = 1.228-82.090, P = 0.03) and Osserman-stage IIA-IV (OR = 5.953, 95% CI = 1.506-23.538, P = 0.01) independently predicted POMC. CONCLUSIONS:Osserman stage (IIA-IV) and WHO type B2-B3 thymomas are independent predictors of POMC in thymoma patients with myasthenia gravis who have undergone total thymectomy. Thus, adequate perioperative care should be provided to these patients. 10.1093/ejcts/ezx163
    Criteria for Postoperative Mechanical Ventilation After Thymectomy in Patients With Myasthenia Gravis: A Retrospective Analysis. Chigurupati Keerthi,Gadhinglajkar Shrinivas,Sreedhar Rupa,Nair Muraleedharan,Unnikrishnan Madathipat,Pillai Manjusha Journal of cardiothoracic and vascular anesthesia OBJECTIVE:To determine the criteria for postoperative mechanical ventilation after thymectomy in patients with Myasthenia Gravis. DESIGN:Retrospective study. SETTING:Teritiary care centre. PARTICIPANTS:77 Myasthenia gravis patients operated for thymectomy were studied. INTERVENTIONS:After obtaining clearance from Institutional ethics committee, medical records of 77 patients with MG, who were operated for thymectomy between January 2005 and December 2015 were reviewed in a retrospective manner. Perioperative variables collected from the patient records were demographic data, duration of the disease, Osserman and Genkin classification, Anti-acetylcholine antibody (AChR) positivity, preoperative daily dose of drug, history of preoperative myasthenic crisis, preoperative vital capacity, technique of anesthesia, drugs used for anesthesia, perioperative complications, and duration of postoperative mechanical ventilation. The patients were divided into two groups, group I and group II consisting of those who required postoperative ventilation for < 300 minutes and > 300 minutes, respectively. The determinants of prolonged postoperative ventilation were studied. MEASUREMENTS AND MAIN RESULTS:The requirement of mechanical ventilation was higher in patients with higher Osserman's grade of myasthenia gravis. Duration of the disease had no effect on the duration of mechanical ventilation in myasthenic patients post thymectomy (p = 0.89). The patients with a preoperative history of myasthenic crisis had a requirement for prolonged mechanical ventilation (p=0.03). Patients with preoperative vital capacity < 2.9 litres and preoperative CT scan showing thymoma had a requirement for prolonged mechanical ventilation with p values < 0.001 and 0.035, respectively. Patients who showed positivity for anti-acetylcholine antibodies had a prolonged mechanical ventilation (p=0.026). Preoperative dose of pyridostigmine and the choice of continuation or discontinuation of antcholinesterases on the day of surgery had no influence on the duration of mechanical ventilation (p value of 0.19 and 0.36 respectively). Epidural analgesia intra and postoperatively significantly reduced the requirement of mechanical ventilation (p=0.006). CONCLUSION:The predictors of postoperative ventilation in myasthenic patients undergoing thymectomy as per our study are: 1. Grade of myasthenia; 2. History of preoperative myasthenic crisis; 3. Anti-acetylcholine antibodies positivity; 4. Presence of thymoma; and 5. a vital capacity < 2.9 litres. Use of thoracic epidural as a part of combined anesthetic technique helps to reduce the need of mechanical ventilation in these patients. 10.1053/j.jvca.2017.06.045
    Prognosis of thymectomy in myasthenia gravis patients with thymus hyperplasia. Yang Jing,Liu Chanchan,Li Tao,Li Chengyan The International journal of neuroscience 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. 10.1080/00207454.2016.1257993
    Thymectomy may not be associated with clinical improvement in MuSK myasthenia gravis. Clifford Katherine M,Hobson-Webb Lisa D,Benatar Michael,Burns Ted M,Barnett Carolina,Silvestri Nicholas J,Howard James F,Visser Amy,Crum Brian A,Nowak Richard,Beekman Rachel,Kumar Aditya,Ruzhansky Katherine,Chen I-Hweii Amy,Pulley Michael T,Laboy Shannon M,Fellman Melissa A,Howard Diantha B,Kolb Noah A,Greene Shane M,Pasnoor Mamatha,Dimachkie Mazen M,Barohn Richard J,Hehir Michael K Muscle & nerve INTRODUCTION:A randomized trial demonstrated benefit from thymectomy in nonthymomatous acetylcholine receptor (AChR)-antibody positive myasthenia gravis (MG). Uncontrolled observational and histologic studies suggest thymectomy may not be efficacious in anti-muscle-specific kinase (MuSK)-MG. METHODS:The therapeutic impact of thymectomy was evaluated from data collected for a multicenter, retrospective blinded review of rituximab in MuSK-MG. RESULTS:Baseline characteristics were similar between thymectomy (n = 26) and nonthymectomy (n = 29) groups, including treatment with rituximab (42% vs. 45%). At last visit, 35% of thymectomy subjects reached the primary endpoint, a Myasthenia Gravis Foundation of America (MGFA) post-intervention status (PIS) score of minimal manifestations (MM) or better, compared with 55% of controls (P = 0.17). After controlling for age at onset of MG, rituximab, prednisone, and intravenous immunoglobulin/plasma exchange treatment, thymectomy was not associated with greater likelihood of favorable clinical outcome (odds ratio = 0.43, 95% confidence interval 0.12-1.53, P = 0.19). DISCUSSION:Thymectomy was not associated with additional clinical improvement in this multicenter cohort of MuSK-MG patients. Muscle Nerve 59:404-410, 2019. 10.1002/mus.26404
    Thoracoscopic thymectomy for juvenile myasthenia gravis. Kim Aimee G,Upah Sydney A,Brandsema John F,Yum Sabrina W,Blinman Thane A Pediatric surgery international PURPOSE:A randomized controlled trial of thymectomy in myasthenia gravis demonstrated improved clinical outcomes in adults, but data surrounding juvenile cases, especially those treated with minimally invasive approaches, are limited. Here, we review our experience with thoracoscopic thymectomy for juvenile myasthenia gravis (JMG) in the largest cohort to date. METHODS:All cases of thymectomy for JMG in a single tertiary referral center between 2007 and 2018 were reviewed (N = 50). Patients underwent left thoracoscopic approach with extended dissection and without use of monopolar energy. Demographics, diagnostic criteria, and clinical classification, as well as surgical data were collected. Clinical status and medications were reviewed in follow-up. RESULTS:The mean age at surgery was 10.5 ± 0.8 years. Ocular disease and generalized disease each comprised half of the cohort. No patients suffered complications or increased risk of morbidity or mortality with thymectomy. At any interval of follow-up through 3.5 years, 49.8% of patients were improved compared to their pre-operative presentation, and there was a significant trend towards decreased steroid use. CONCLUSION:Thoracoscopic thymectomy is a safe treatment for juvenile myasthenia gravis in pediatric patients over a wide range of ages, body masses, and symptoms. Our experience adds evidence that pediatric patients likely benefit from thymectomy with improved clinical status and reduced medications. 10.1007/s00383-019-04441-0
    Thymectomy is a beneficial therapy for patients with non-thymomatous ocular myasthenia gravis: a systematic review and meta-analysis. Zhu Kai,Li Jiaoxing,Huang Xin,Xu Wei,Liu Weibin,Chen Jiaxin,Chen Pei,Feng Huiyu Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology Ocular myasthenia gravis, an autoimmune disease, is characterized by extraocular muscle weakness. Myasthenia gravis is closely associated with the functional status of the thymus gland. The efficacy of thymectomy for non-thymomatous ocular myasthenia gravis remains controversial. Here, we present the first systematic review and meta-analysis of studies assessing the outcome of thymectomy in patients with non-thymomatous ocular myasthenia gravis and found that the pooled rate of complete stable remission was 0.5074 with considerable heterogeneity. Furthermore, subgroup analysis showed that the efficacy of thymectomy differed according to geographical location. Furthermore, thymectomy outcomes are better in children than they are in adults. Thymectomy clearly represents an effective treatment for patients with non-thymomatous ocular myasthenia gravis. However, more multicenter, randomized, controlled clinical trials are now required to confirm these conclusions. 10.1007/s10072-017-3058-7
    Assessment of pre and post-thymectomy myasthenia gravis. Bokoliya Suresh C,Patil Shripad A Neurological research OBJECTIVE:To evaluate transcervical and transsternal thymectomy benefits in large myasthenia gravis (MG) cohort. METHOD:We retrospectively evaluated MG patients (n = 184) who had undergone thymectomy between 2004 and 2015 at National Institute of Mental Health and Neurosciences, Bangalore (India). Myasthenia gravis foundation of America guidelines were followed to assess clinical outcome. Anti-acetylcholine receptors (AChR) antibodies, repetitive nerve stimulation (RNS) and Neostigmine tests were performed at pre and post-thymectomy stage. RESULTS:Most of the patients were fell under MG grade IIA (82 of 184, 44.56%) and grade IIB (61 of 184, 33.15%). Thymoma and thymic hyperplasia was established in 64 (34.78%) and 89 (48.37%) patients respectively. Other thymic abnormalities such thymic atrophy, cysts and lipoma were established in 31 (16.85%) patients. MG patients were treated either with transcervical (n = 79) or (n = 105) transsternal thymectomy. At the pre-thymectomy stage, the majority of the patients were positive for anti-AChR antibodies (179 of 184, 97.28%), RNS (170 of 184, 92.4%), and Neostigmine (175 of 184, 95.11%). At the post-thymectomy stage, a significant reduction observed in anti-AChR antibodies positivity (p < 0.022) and RNS positivity (p < 0.015). Overall, benefits were observed in 61.41% (113 of 184) of patients. Clinical benefits (complete stable remission, pharmacological remission, minimal manifestation, and improvement) of transcervical and transsternal thymectomy observed in 69.62% (55 of 79) and 55.24% (58 of 105) of patients respectively. MG patients with thymoma showed the least improvement compared to thymic hyperplasia. DISCUSSION:Transcervical and transsternal thymectomy showed clinical benefits, however, there was no significant difference between them. 10.1080/01616412.2018.1557433
    Effect of thymectomy in elderly patients with non-thymomatous generalized myasthenia gravis. Kim Seung Woo,Choi Young-Chul,Kim Seung Min,Shim Hyo Sup,Shin Ha Young Journal of neurology Whether thymectomy is beneficial in elderly patients with myasthenia gravis (MG) is unclear. Thus, we assessed whether conducting thymectomy in MG patients aged ≥ 50 years is beneficial. This retrospective cohort study included patients with MG between 1990 and 2018. Thymectomy and control cohorts were selected from among the population of MG patients with an age at onset of ≥ 45 years and elevated concentrations of acetylcholine-receptor antibodies. Patients with evidence of thymic malignancy were excluded. Of these patients, those who underwent thymectomy at the age of ≥ 50 years were designated as the thymectomy group and those who received only medical treatment were designated as the medical treatment group. We compared the Myasthenia Gravis Foundation of America post-intervention status between the thymectomy and medical treatment groups. Landmark analysis was conducted with the landmark set at 24 months. A total of 34 and 105 patients were classified into the thymectomy and medical treatment groups, respectively. Before landmark analysis, the thymectomy group had a higher cumulative incidence of pharmacologic remission (p = 0.009) and complete stable remission (p = 0.022) than the medical treatment group. After landmark analysis, the thymectomy group had a 2.22-fold (95% confidence interval 1.01-4.80) increased chance of achieving pharmacologic remission compared to the medical treatment group after adjustment for age, sex, and disease severity. No significant difference was observed in the rate of relapse after pharmacological remission between the thymectomy (16.7%) and medical treatment groups (21.4%). In conclusion, thymectomy may have a beneficial effect in elderly patients with non-thymomatous generalized MG. 10.1007/s00415-019-09222-2
    Randomized Trial of Thymectomy in Myasthenia Gravis. Wolfe Gil I,Kaminski Henry J,Aban Inmaculada B,Minisman Greg,Kuo Hui-Chien,Marx Alexander,Ströbel Philipp,Mazia Claudio,Oger Joel,Cea J Gabriel,Heckmann Jeannine M,Evoli Amelia,Nix Wilfred,Ciafaloni Emma,Antonini Giovanni,Witoonpanich Rawiphan,King John O,Beydoun Said R,Chalk Colin H,Barboi Alexandru C,Amato Anthony A,Shaibani Aziz I,Katirji Bashar,Lecky Bryan R F,Buckley Camilla,Vincent Angela,Dias-Tosta Elza,Yoshikawa Hiroaki,Waddington-Cruz Márcia,Pulley Michael T,Rivner Michael H,Kostera-Pruszczyk Anna,Pascuzzi Robert M,Jackson Carlayne E,Garcia Ramos Guillermo S,Verschuuren Jan J G M,Massey Janice M,Kissel John T,Werneck Lineu C,Benatar Michael,Barohn Richard J,Tandan Rup,Mozaffar Tahseen,Conwit Robin,Odenkirchen Joanne,Sonett Joshua R,Jaretzki Alfred,Newsom-Davis John,Cutter Gary R, The New England journal of medicine BACKGROUND:Thymectomy has been a mainstay in the treatment of myasthenia gravis, but there is no conclusive evidence of its benefit. We conducted a multicenter, randomized trial comparing thymectomy plus prednisone with prednisone alone. METHODS:We compared extended transsternal thymectomy plus alternate-day prednisone with alternate-day prednisone alone. Patients 18 to 65 years of age who had generalized nonthymomatous myasthenia gravis with a disease duration of less than 5 years were included if they had Myasthenia Gravis Foundation of America clinical class II to IV disease (on a scale from I to V, with higher classes indicating more severe disease) and elevated circulating concentrations of acetylcholine-receptor antibody. The primary outcomes were the time-weighted average Quantitative Myasthenia Gravis score (on a scale from 0 to 39, with higher scores indicating more severe disease) over a 3-year period, as assessed by means of blinded rating, and the time-weighted average required dose of prednisone over a 3-year period. RESULTS:A total of 126 patients underwent randomization between 2006 and 2012 at 36 sites. Patients who underwent thymectomy had a lower time-weighted average Quantitative Myasthenia Gravis score over a 3-year period than those who received prednisone alone (6.15 vs. 8.99, P<0.001); patients in the thymectomy group also had a lower average requirement for alternate-day prednisone (44 mg vs. 60 mg, P<0.001). Fewer patients in the thymectomy group than in the prednisone-only group required immunosuppression with azathioprine (17% vs. 48%, P<0.001) or were hospitalized for exacerbations (9% vs. 37%, P<0.001). The number of patients with treatment-associated complications did not differ significantly between groups (P=0.73), but patients in the thymectomy group had fewer treatment-associated symptoms related to immunosuppressive medications (P<0.001) and lower distress levels related to symptoms (P=0.003). CONCLUSIONS:Thymectomy improved clinical outcomes over a 3-year period in patients with nonthymomatous myasthenia gravis. (Funded by the National Institute of Neurological Disorders and Stroke and others; MGTX ClinicalTrials.gov number, NCT00294658.). 10.1056/NEJMoa1602489