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In patients with nonthymomatous myasthenia gravis, thymectomy plus prednisone was associated with better clinical outcomes than prednisone alone. Patients treated with thymectomy had fewer hospitalizations for exacerbations and required lower prednisone doses. The first reported use of thymectomy in patients with nonthymomatous myasthenia gravis was 75 years ago. 1 Of six patients who underwent surgery, three had a favorable response. Subsequent retrospective studies have shown benefits of thymectomy in patients with nonthymomatous myasthenia gravis but with widely varying rates of clinical improvement or remission. A compilation of retrospective studies comparing surgery with medical management did not show a difference in remission rates. 2 Two studies that showed clinical improvements after thymectomy indicated that benefit occurred in the first few years after the procedure, but after 5 years, rates of clinical improvement were similar among . . .

The Thymectomy Trial in Non-Thymomatous Myasthenia Gravis Patients Receiving Prednisone (MGTX) showed that thymectomy combined with prednisone was superior to prednisone alone in improving clinical status as measured by the Quantitative Myasthenia Gravis (QMG) score in patients with generalised non-thymomatous myasthenia gravis at 3 years. We investigated the long-term effects of thymectomy up to 5 years on clinical status, medication requirements, and adverse events. We did a rater-blinded 2-year extension study at 36 centres in 15 countries for all patients who completed the randomised controlled MGTX and were willing to participate. MGTX patients were aged 18 to 65 years at enrolment, had generalised non-thymomatous myasthenia gravis of less than 5 years' duration, had acetylcholine receptor antibody titres of 1·00 nmol/L or higher (or concentrations of 0·50–0·99 nmol/L if diagnosis was confirmed by positive edrophonium or abnormal repetitive nerve stimulation, or abnormal single fibre electromyography), had Myasthenia Gravis Foundation of America Clinical Classification Class II–IV disease, and were on optimal anticholinesterase therapy with or without oral corticosteroids. In MGTX, patients were randomly assigned (1:1) to either thymectomy plus prednisone or prednisone alone. All patients in both groups received oral prednisone at doses titrated up to 100 mg on alternate days until they achieved minimal manifestation status. The primary endpoints of the extension phase were the time-weighted means of the QMG score and alternate-day prednisone dose from month 0 to month 60. Analyses were by intention to treat. The trial is registered with ClinicalTrials.gov, number NCT00294658. It is closed to new participants, with follow-up completed. Of the 111 patients who completed the 3-year MGTX, 68 (61%) entered the extension study between Sept 1, 2009, and Aug 26, 2015 (33 in the prednisone alone group and 35 in the prednisone plus thymectomy group). 50 (74%) patients completed the 60-month assessment, 24 in the prednisone alone group and 26 in the prednisone plus thymectomy group. At 5 years, patients in the thymectomy plus prednisone group had significantly lower time-weighted mean QMG scores (5·47 [SD 3·87] vs 9·34 [5·08]; p=0·0007) and mean alternate-day prednisone doses (24 mg [SD 21] vs 48 mg [29]; p=0·0002) than did those in the prednisone alone group. 14 (42%) of 33 patients in the prednisone group, and 12 (34%) of 35 in the thymectomy plus prednisone group, had at least one adverse event by month 60. No treatment-related deaths were reported during the extension phase. At 5 years, thymectomy plus prednisone continues to confer benefits in patients with generalised non-thymomatous myasthenia gravis compared with prednisone alone. Although caution is appropriate when generalising our findings because of the small sample size of our study, they nevertheless provide further support for the benefits of thymectomy in patients with generalised non-thymomatous myasthenia gravis. National Institutes of Health, National Institute of Neurological Disorders and Stroke.

Background The safety and feasibility of robotic‐assisted (RATS) thymectomy for myasthenia gravis (MG) with onset age ≥ 50 years remain unverified, particularly in very late‐onset MG (V‐LOMG). Methods Patients were classified into late‐onset MG (LOMG, 50–64) and very late‐onset MG (V‐LOMG ≥ 65) based on age of onset. Composite neurological remission (CNR) included complete stable remission (CSR), pharmacologic remission (PR), and minimal manifestations‐0 (MM‐0), while favorable outcomes comprised CNR and MM1‐3. Results Among 1041 patients, 172 with MG onset at ≥ 50 years who underwent RATS extended thymectomy were included in the final analysis. The LOMG group comprised 104 patients (45.2% male), while the V‐LOMG group included 68 patients (60.3% male). V‐LOMG patients had more preoperative MG crises, shorter onset‐to‐thymectomy intervals, heavier thymic specimens, and less hyperplasia. In ocular‐onset MG, generalization was more frequent in LOMG than in V‐LOMG. No significant differences were found in other baseline characteristics, perioperative parameters, postoperative complications, and adverse composite outcomes. At a 5.1‐year mean follow‐up, the V‐LOMG group had slightly higher CSR (7.4% vs. 6.7%), CNR (16.2% vs. 11.5%), and favorable outcome rates (52.9% vs. 45.2%) than the LOMG group, with no statistical significance. Both groups, especially V‐LOMG (16.0 mg vs. 2.1 mg, p < 0.001), showed a significant corticosteroid dose reduction at the last follow‐up, confirming the steroid‐sparing effect of thymectomy. Conclusions RATS extended thymectomy appears to be a safe and feasible treatment for patients with MG of onset at age ≥ 50 years, including those with V‐LOMG, demonstrating a significant steroid‐sparing effect while maintaining favorable neurological outcomes. This 21‐year retrospective cohort study evaluated the safety and long‐term outcomes of robotic extended thymectomy in 172 patients with late‐onset myasthenia gravis (onset age ≥ 50 years). Both late‐ and very late‐onset subgroups showed comparable perioperative outcomes and achieved favorable neurological remission. A significant corticosteroid‐sparing effect was observed at long‐term follow‐up, particularly in the very late‐onset group.

Introduction There are only a few studies exploring post-thymectomy outcome in patients with acetylcholine receptor antibody (AChR-Ab)-positive generalised myasthenia gravis (MG). Objective To assess the predictors of outcome in patients with AChR-Ab-positive generalised MG who underwent thymectomy. Methods A retrospective study of 53 patients from a single neuroscience centre in the UK. Results The mean disease duration from diagnosis was 6.2 ± 4.3 years. Pre-thymectomy, 37 patients had mild weakness affecting muscles other than ocular muscles, 11 patients had moderate weakness and 5 patients had severe weakness. 27/53 patients had thymoma. Post-thymectomy (mean duration of 5.7 ± 4.2 years), 34 patients (64%) had a good outcome characterised by Myasthenia Gravis Foundation of America Post-Intervention Status of complete stable remission (no symptoms or signs of MG for at least 1 year without any therapy) or pharmacological remission (no symptoms or signs of MG with some form of therapy) or minimal manifestations (no symptoms of functional limitations from MG but weakness on examination of some muscles with or without some form of therapy) on last follow-up visit. Having thymomatous or non-thymomatous MG did not predict the outcome. The only variable that did predict outcome was pre-thymectomy disease severity; patients with mild weakness before thymectomy had a favourable outcome. We found an accuracy of 83% predicting outcome (95% confidence interval (CI) 60%, 100%) with a sensitivity of 84% (95% CI 68%, 94%) and specificity of 81% (95% CI 54%, 96%). Conclusion Disease severity before thymectomy predicts outcome in patients with AChR-Ab-positive generalised MG.

The ability of thymic histopathology to predict the long-term impact of thymectomy in non-thymomatous myasthenia gravis (NTMG) is mainly uncharted. We applied digital pathology to quantitatively characterize differences of thymic histology between early-onset (EOMG) and late-onset MG (LOMG) and to investigate the role of thymic changes for thymectomy outcomes in MG. We analyzed 83 thymic H&E slides from thymectomized NTMG patients, of which 69 had EOMG and 14 LOMG, using digital pathology open-access software QuPath. We compared the results to the retrospectively assessed clinical outcome at two years after thymectomy and at the last follow-up visit where complete stable remission and minimal use of medication were primary outcomes. The automated annotation pipeline was an effective and reliable way to analyze thymic H&E samples compared to manual annotation with mean intraclass correlation of 0.80. The ratio of thymic tissue to stroma and fat was increased in EOMG compared to LOMG (p = 8.7e-07), whereas no difference was observed in the ratio of medulla to cortex between these subtypes. AChRAb seropositivity correlated with the number of ectopic germinal centers (eGC; p = 0.00067) but not with other histological areas. Patients with an increased number of eGCs had better post-thymectomy outcomes at two years after thymectomy (p = 0.0035) and at the last follow-up (p = 0.0267). ROC analysis showed that eGC area predicts thymectomy outcome in EOMG with an AUC of 0.79. Digital pathology can thus help in providing a predictive tool to the clinician, the eGC number, to guide the post-thymectomy treatment decisions in EOMG patients.

Background To identify the associations between thymic pathology and the prognosis of myasthenia gravis (MG) patients. Methods In this multicenter retrospective study, 1,254 myasthenia gravis (MG) patients who underwent thymectomy across four clinical centers were included. Participants were categorized by thymic pathology into thymomatous and non-thymomatous groups. Primary outcome was postoperative deterioration. Secondary outcomes comprised the proportion of patients achieving minimal manifestation status (MMS) within the first year after surgery and conversion from ocular MG (OMG) to generalized MG (GMG) within 2 years of symptom onset. Subgroup analyses assessed associations between world health organization (WHO) pathological type (both groups) or Masaoka stage (thymoma patients) and prognosis. Results Thymomas were associated with an increased risk of deterioration in both Cox regression (adjusted HR = 1.40 [1.18, 1.66], p  < 0.001) and logistic regression analyses (1-year deterioration: adjusted OR = 1.59 [1.15, 2.20], p  = 0.005; 3-year deterioration: adjusted OR = 1.40 [1.01, 1.94], p  = 0.047). Additionally, thymomas were linked to a higher conversion rate (adjusted OR = 2.37 [1.15, 4.86], p  = 0.019). However, thymoma showed no significant association with MMS (adjusted p  = 0.682). In the thymoma subgroup, neither pathological type nor Masaoka stage was significantly associated with deterioration (pathological type: 1-year p  = 0.069, 3-year p  = 0.220; Masaoka stage: 1-year p  = 0.944, 3-year p  = 0.909), first-year MMS attainment (pathological type: p  = 0.067; Masaoka stage: p  = 0.579), or conversion rate (pathological type: p  = 0.606; Masaoka stage: p  = 0.163). Similarly, in the nonthymomatous group, WHO pathological type was not significantly correlated with deterioration (1-year p  = 0.806, 3-year p  = 0.654), MMS achieved ( p  = 0.940), or conversion ( p  = 0.755). Conclusions This study demonstrated an association between thymoma and higher risks of clinical deterioration, which was independent of WHO pathological type or Masaoka stage.

This study aims to investigate the association between thymectomy and the risk of generalization in patients with ocular myasthenia gravis (MG). Data on patients with ocular MG from seven neurological centers in China were retrospectively reviewed. Ocular MG naïve to immunotherapy was categorized according to whether thymectomy was performed (thymectomized group vs. nonsurgical group). Patients in the thymectomized group all underwent surgery within 2 years since ocular symptom onset. The main outcome measure was the generalization. The follow-up period was defined from the date of ocular symptom onset to the date of generalization confirmation, immunotherapy initiation, or last follow-up (defined as 60 months). Of 519 eligible patients (mean [SD] age, 48.7 [15.2] years, 46.6% women), 31 (23.7%) of 131 generalized in the thymectomized group and 122 (31.4%) of 388 did in the nonsurgical group during a median follow-up of 19 months (IQR 8.0–50.0). Thymectomy was independently associated with reduced generalization risk (adjusted HR 0.41, 95% CI 0.25–0.66, P < 0.001). Multivariable stratified analysis also verified this association across the subgroups. Kaplan–Meier curves showed that the 5-year cumulative rate was significantly lower in the thymectomized group than in the nonsurgical group. To conclude, thymectomy may be considered effective in modifying the progression from ocular to generalized MG irrespective of thymoma.

ObjectiveThymectomy has been utilized as a treatment for Myasthenia Gravis (MG) for many decades, with both open and minimally invasive surgical (MIS) techniques currently used. Although MIS has shown improved short-term results, long-term effects remain uncertain. This systematic review and meta-analysis aim to compare the post-operative and long-term outcomes of MIS versus open thymectomy in MG patients.MethodsMEDLINE, EMBASE and CENTRAL databases were searched from inception till January 2022 for keywords related to MG and open or MIS thymectomy. Primary outcome was complete stable remission (CSR), and secondary outcomes were clinical improvement, complications, length of stay, operation time, and blood loss. Grading of recommendations, assessment, development, and evaluation was used to assess the certainty of evidence.Results26 studies with 3588 patients were included in the analysis. At 1, 3 and 5 years, there was no statistical difference noted in CSR between open versus MIS thymectomy. However, CSR was improved at 1 year for MIS thymectomy in non-thymomatous MG (P = 0.03). There was no significant difference in rates of partial clinical improvement between techniques at 1-year. Although analyses on length of hospital stay and blood loss showed improvement following MIS thymectomy, operative time was shorter for open thymectomy.ConclusionThis is the first systematic review and meta-analysis assessing long-term effects of MIS versus open thymectomy in MG patients. Given the lack of significant differences noted, either MIS or open thymectomy can be performed, based on surgeon preference. Further high-level, long-term research should be conducted to determine the benefit of each technique.

Objective This case demonstrates the efficacy and safety of efgartigimod in managing an acute exacerbation of myasthenia gravis (MG) with comorbid Castleman disease, particularly during the perioperative care of retroperitoneal mass resection. Methods A 23-year-old female was admitted with the discovery of a retroperitoneal Mass, confirmed via abdominal enhanced CT and MRI. The patient had generalized MG since 2017, with controlled but fluctuating symptoms. Results Preoperative assessment showed albumin levels were slightly reduced and respiratory function demonstrated severe restrictive ventilation impairment. There was acute exacerbation of muscle weakness (MG Foundation of America (MGFA) class IVb, MG-activities of daily living (ADL) score 8, and quantitative MG (QMG) score 20) with respiratory and medullary symptoms. Treatment with efgartigimod (10 mg/kg/week), prednisone 15 mg/day and pyridostigmine 270 mg/day was initiated. The symptoms improved substantially after four infusions (MG-ADL score 0, QMG score 6), and the lung function recovered. The patient continued efgartigimod treatment and underwent retroperitoneal mass resection. The mass showed massive lymph node hyperplasia (Castleman’s disease, mixed type). Postoperatively, the patient experienced no infection, MG exacerbation, or other complications. Discussion Efgartigimod had a rapid onset of action, swiftly improved symptoms during acute MG exacerbation, and maintained symptom stability during the perioperative care.

Introduction Myasthenia gravis (MG) is an autoimmune disease presenting typically at an earlier age in females compared to males. However, whether sex affects the diagnostic delay and clinical course of MG has not been extensively explored in systematic cohorts. Methods We conducted a retrospective single‐center cohort study on 251 thymectomized MG patients, including 124 males and 127 females. Mean follow‐up was 10.7 (±9.1) years post‐thymectomy. We analyzed factors associated with longer diagnostic delay and the type of first‐onset symptoms (ocular vs. generalized). For 195 patients with nonthymomatous generalized MG (gMG) pre‐thymectomy, we estimated the effect of diagnostic delay, symptoms at onset, and sex on reaching complete stable remission (CSR) or minimal need for medication (MNM) during postoperative follow‐up. We also assessed their effect on the overall need for in‐hospital treatments, immunosuppressant use, and pyridostigmine dose at the last follow‐up visit. Results Generalized symptoms at onset were more frequent for females than for males (n = 90, 70.9% vs. n = 65, 52.4%, respectively; p < 0.001). Diagnostic delay was significantly longer in females (6.0 months [0–117.5] vs. 3.2 months in males [0.1–84.0]; p = 0.012). First‐onset symptoms and diagnostic delay did not affect the post‐thymectomy prognosis of gMG. However, females achieved CSR (17.1% vs. 4.5%; p = 0.006) and MNM (30.8% vs. 16.9%; p = 0.029) more frequently and required fewer in‐hospital treatments (40.6% vs. 55.2%; p = 0.010) and immunosuppressants (29.2% vs. 61.7%; p < 0.001) than males. Conclusions We report sex‐related differences in symptoms at MG onset, length of diagnostic delay, and prognosis, the origins of which should be further studied.