scholarly journals Minimal manifestation status and prednisone withdrawal in the MGTX trial

Neurology ◽  
2020 ◽  
Vol 95 (6) ◽  
pp. e755-e766
Ikjae Lee ◽  
Hui-Chien Kuo ◽  
Inmaculada B. Aban ◽  
Gary R. Cutter ◽  
Tarrant McPherson ◽  

ObjectiveTo examine whether sustained minimal manifestation status (MMS) with complete withdrawal of prednisone is better achieved in thymectomized patients with myasthenia gravis (MG).MethodsThis study is a post hoc analysis of data from a randomized trial of thymectomy in MG (Thymectomy Trial in Non-Thymomatous Myasthenia Gravis Patients Receiving Prednisone Therapy [MGTX]). MGTX was a multicenter, randomized, rater-blinded 3-year trial that was followed by a voluntary 2-year extension for patients with acetylcholine receptor (AChR) antibody–positive MG without thymoma. Patients were randomized 1:1 to thymectomy plus prednisone vs prednisone alone. Participants were age 18–65 years at enrollment with disease duration less than 5 years. All patients received oral prednisone titrated up to 100 mg on alternate days until they achieved MMS, which prompted a standardized prednisone taper as long as MMS was maintained. The achievement rate of sustained MMS (no symptoms of MG for 6 months) with complete withdrawal of prednisone was compared between the thymectomy plus prednisone and prednisone alone groups.ResultsPatients with MG in the thymectomy plus prednisone group achieved sustained MMS with complete withdrawal of prednisone more frequently (64% vs 38%) and quickly compared to the prednisone alone group (median time 30 months vs no median time achieved, p < 0.001) over the 5-year study period. Prednisone-associated adverse symptoms were more frequent in the prednisone alone group and distress level increased with higher doses of prednisone.ConclusionsThymectomy benefits patients with MG by increasing the likelihood of achieving sustained MMS with complete withdrawal of identifierNCT00294658.Classification of evidenceThis study provides Class II evidence that for patients with generalized MG with AChR antibody, those receiving thymectomy plus prednisone are more likely to attain sustained MMS and complete prednisone withdrawal than those on prednisone alone.

2007 ◽  
Vol 13 (3) ◽  
pp. 332-335 ◽  
M. Nakamura ◽  
I. Nakashima ◽  
S. Sato ◽  
I. Miyazawa ◽  
K. Fujihara ◽  

Of 23 neuromyelitis optica (NMO) cases, we found two cases with oligoclonal IgG bands (OBs). Both patients were positive for NMO-IgG. Their common features were long disease duration and co-existing autoimmune diseases (myasthenia gravis and sicca syndrome). Although OBs are mostly negative in NMO, which distinguishes it from multiple sclerosis (MS), they can be positive by long-standing autoimmunity, which may not be directly related to NMO. Multiple Sclerosis 2007; 13: 332-335.

2021 ◽  
Vol 13 ◽  
pp. 117957352110160
Thomas Mathew ◽  
Kurian Thomas ◽  
Saji K John ◽  
Shruthi Venkatesh ◽  
Raghunandan Nadig ◽  

Background: Rituximab is reserved for treating refractory myasthenia gravis (MG) patients. Here we report our experience with rituximab in AChR antibody positive generalized MG (gMG) and impending myasthenic crisis (IMC). Methods: This retrospective, observational study, conducted at a tertiary care, neuroimmunology clinic, analyzed the data of patients with AChR antibody positive gMG, treated with rituximab between 1st January 2016 and 30th October 2018. Results: Eleven patients with AChR antibody positive gMG received rituximab. Mean age of the cohort was 50.54 ± 18.71 years with 9 males. Seven out of 11 patients received rituximab in the early stage (<2 years from onset) and had good response to treatment. Four of the 5 patients with IMC improved with rituximab alone. In the 10 patients who regularly followed up, there was a significant difference between the QMG scores at baseline and at 1, 2, 6, 12, and 18 months ( P < .0001). Conclusion: Rituximab appears to be a potentially effective early treatment option for AChR antibody positive generalized MG and impending myasthenic crisis.

Diabetes ◽  
2021 ◽  
Vol 70 (Supplement 1) ◽  
pp. 651-P

PLoS ONE ◽  
2014 ◽  
Vol 9 (9) ◽  
pp. e106757 ◽  
Tetsuya Akaishi ◽  
Takuhiro Yamaguchi ◽  
Yasushi Suzuki ◽  
Yuriko Nagane ◽  
Shigeaki Suzuki ◽  

2012 ◽  
Vol 25 (4) ◽  
pp. 397-405 ◽  
Kazumasa Miyake ◽  
Masafumi Kusunoki ◽  
Nobue Ueki ◽  
Akiyoshi Yamada ◽  
Hiroyuki Nagoya ◽  

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012050
Shahar Shelly ◽  
John R Mills ◽  
Divyansu Dubey ◽  
Andrew McKeon ◽  
Anastasia Zekeridou ◽  

Objective:To critically assess the clinical utility of striational antibodies (StrAbs) within paraneoplastic and myasthenia gravis serological evaluations.Methods:All Mayo Clinic patients tested for StrAbs from January 1st 2012-December 31st 2018 utilizing Mayo’s Unified Data Platform (UDP) were reviewed for neurological diagnosis and cancer.Results:38,502 unique paraneoplastic and 1,899 MG patients were tested. In paraneoplastic evaluations, the StrAbs positivity rate was higher in cancer vs without cancer (5% [321/6775] vs 4% [1154/31727]; p<0.0001; OR 1.35; CI=1.19-1.53) but ROC analysis indicated no diagnostic accuracy in cancer (AUC=0.505). No neurological phenotype was significantly associated with StrAbs in the paraneoplastic group. Positivity was more common in all MG cancers compared to paraneoplastic cancers (p<0.0001). In MG evaluations, the StrAbs positivity rate was higher in those with cancer vs without (46% [217/474] vs 26% [372/1425]; p<0.0001; OR 2.39, CI 1.9-2.96) with ROC analysis indicating poor diagnostic accuracy for thymic cancer (AUC 0.634, recommended cutoff=1:60, sensitivity=56%, specificity=71%), with worse accuracy for extrathymic cancers (AUC 0.543). In paraneoplastic or MG evaluations, the value of antibody positivity did not improve cancer predictions. Paraneoplastic evaluated patients were more likely with positive StrAbs to obtain computed tomography (CT) (p=0.0001) with 3% (12/468) cancer found.Conclusion:Despite a statistically significant association with cancer, an expansive review of performance in clinical service demonstrates that StrAbs are neither specific nor sensitive in predicting malignancy or neurological phenotypes. CT imaging is over utilized with positive StrAbs results. Removal of StrAbs from paraneoplastic or MG evaluations will improve the diagnostic characteristics of the current MG test.Classification of Evidence:This study provides Class II evidence that the presence of StrAbs do not accurately identify patients with malignancy or neurological phenotypes.

2010 ◽  
Vol 22 (9) ◽  
pp. 133
N. Hatzirodos ◽  
H. F. Irving-Rodgers ◽  
R. J. Rodgers

Small antral follicles <5 mm in bovine ovaries undergo one of two fates: further growth and selection to become the dominant follicle for ovulation, or atresia. Atresia can occur before, during or after selection. As follicle grow past >5 mm there is upregulation in expression of focimatrix genes and later upregulation of the LH receptor and steroidogenic enzymes, especially aromatase, in the granulosa cells. For follicles at sizes >5 mm entering atresia the granulosa cells are the first in the follicle to die. Thus expression of genes in granulosa cells is critical to the fate of the follicle. To examine granulosa cells of small follicles we collected bovine ovaries and dissected follicles, removed part of the follicle wall for subsequent classification of health or atresia, and harvested the remaining granulosa cells for RNA isolation. Follicles examined included small follicles (<5 mm), both healthy (n = 10) and atretic (n =5), and healthy large follicles (>10 mm, n = 4). RNA was hybridized to Affymetrix GeneChip Bovine Genome Arrays and the results were analysed using Partek Genomics Suite software. The number of genes which were 2 fold differentially regulated between large and small follicles by Benjamini Hochberg post hoc test (False Discovery Rate, P < 0.05) was 2408 and between healthy and atretic small follicles was 4931. The coefficient of variation (CV; SD/mean × 100) for the expression level of each gene for each group was calculated. A gene frequency distribution indicated greater heterogeneity in expression levels in small follicles in comparison to large follicles. Furthermore, the greatest variability in genes in small follicles includes those that are either up or down regulated due to atresia or growth. We therefore conclude that variability in small follicles is a consequence of alternative fates that small follicle can undergo.

C.W. Olanow ◽  
A.D. Roses ◽  
J.W. Fay

SUMMARY:Five myasthenia gravis patients with post-thymectomy residual ocular signs were treated with plasmapheresis. Despite a significant reduction in AChR antibody titer, there was no clinical improvement. Subsequently, there was a dramatic response to prednisone. The AChR antibody titer did not correlate with the clinical state of the individual patient. It is suggested that plasmapheresis may operate by removing a thymic factor and that prednisone acts by a different mechanism.

Neurology ◽  
2020 ◽  
pp. 10.1212/WNL.0000000000011207
Renato Mantegazza ◽  
Gil I. Wolfe ◽  
Srikanth Muppidi ◽  
Heinz Wiendl ◽  
Kenji P. Fujita ◽  

ObjectiveTo evaluate whether eculizumab helps patients with anti-acetylcholine receptor-positive (AChR+) refractory generalized myasthenia gravis (gMG) to achieve the Myasthenia Gravis Foundation of America (MGFA) post-intervention status of minimal manifestations (MM), we assessed patients' status throughout REGAIN and its open-label extension.MethodsPatients who completed the REGAIN randomized controlled trial and continued into the open-label extension were included in this tertiary endpoint analysis. Patients were assessed for the MGFA post-intervention status of improved, unchanged, worse, MM and pharmacologic remission at defined time points during REGAIN and through week 130 of the open-label study.ResultsA total of 117 patients completed REGAIN and continued into the open-label study (eculizumab/eculizumab: 56; placebo/eculizumab: 61). At week 26 of REGAIN, more eculizumab-treated patients than placebo-treated patients achieved a status of improved (60.7% vs 41.7%) or MM (25.0% vs 13.3%; common OR: 2.3; 95% CI: 1.1–4.5). After 130 weeks of eculizumab treatment, 87.1% of patients achieved improved status and 57.1% of patients achieved MM status. The safety profile of eculizumab was consistent with its known profile and no new safety signals were detected.ConclusionsEculizumab led to rapid and sustained achievement of MM in patients with AChR+ refractory gMG. These findings support the use of eculizumab in this previously difficult-to-treat patient population.Classification of evidenceThis study provides Class II evidence that, after 26 weeks of eculizumab treatment, 25.0% of adults with AChR+ refractory gMG achieved MM, compared with 13.3% who received placebo.Trial registrationREGAIN, NCT01997229; REGAIN open-label extension, NCT02301624 (

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