scholarly journals Insights into the Classification of Myasthenia Gravis

PLoS ONE ◽  
2014 ◽  
Vol 9 (9) ◽  
pp. e106757 ◽  
Author(s):  
Tetsuya Akaishi ◽  
Takuhiro Yamaguchi ◽  
Yasushi Suzuki ◽  
Yuriko Nagane ◽  
Shigeaki Suzuki ◽  
...  
Keyword(s):  
Myasthenia Gravis

Clinical Utility of Striational Antibodies in Paraneoplastic and Myasthenia Gravis Paraneoplastic Panels

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012050
Author(s):  
Shahar Shelly ◽  
John R Mills ◽  
Divyansu Dubey ◽  
Andrew McKeon ◽  
Anastasia Zekeridou ◽  
...  
Keyword(s):  
Diagnostic Accuracy ◽  
Myasthenia Gravis ◽  
Clinical Utility ◽  
Roc Analysis ◽  
Thymic Cancer ◽  
Diagnostic Characteristics ◽  
Data Platform ◽  
Neurological Phenotype ◽  
Antibody Positivity

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.

scholarly journals Post-intervention status in patients with refractory myasthenia gravis treated with eculizumab during REGAIN and its open-label extension

Neurology ◽  
2020 ◽  
pp. 10.1212/WNL.0000000000011207
Author(s):  
Renato Mantegazza ◽  
Gil I. Wolfe ◽  
Srikanth Muppidi ◽  
Heinz Wiendl ◽  
Kenji P. Fujita ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Controlled Trial ◽  
Open Label ◽  
Post Intervention ◽  
Open Label Study ◽  
Label Study ◽  
Link Type ◽  
Open Label Extension ◽  
Anti Acetylcholine

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 (ClinicalTrials.gov).

Diagnostic and clinical classification of autoimmune myasthenia gravis

2014 ◽  
Vol 48-49 ◽  
pp. 143-148 ◽  
Author(s):  
Sonia Berrih-Aknin ◽  
Mélinée Frenkian-Cuvelier ◽  
Bruno Eymard
Keyword(s):  
Myasthenia Gravis ◽  
Clinical Classification ◽  
Autoimmune Myasthenia

A propensity score analysis for comparison of T-3b and VATET in myasthenia gravis

Neurology ◽  
2017 ◽  
Vol 89 (2) ◽  
pp. 189-195 ◽  
Author(s):  
Greta Brenna ◽  
Carlo Antozzi ◽  
Cristina Montomoli ◽  
Fulvio Baggi ◽  
Renato Mantegazza ◽  
...  
Keyword(s):  
Propensity Score ◽  
Myasthenia Gravis ◽  
Clinical Data ◽  
Propensity Score Analysis ◽  
Statistical Tests ◽  
Surgical Approaches ◽  
Extended Thymectomy ◽  
Score Analysis ◽  
Complete Set

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 (p < 0.001), had a lower INCB-MG score (p < 0.001), and had less muscle fatigability (p = 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.

scholarly journals Specific features of anesthesia in patients with myasthenia gravis

2016 ◽  
Vol 69 (9-10) ◽  
pp. 305-311
Author(s):  
Ivana Spasojevic ◽  
Danica Hajdukovic ◽  
Milena Komarcevic ◽  
Stanislava Petrovic ◽  
Jelena Jovanovic ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Conservative Treatment ◽  
Myasthenia Gravis ◽  
Nicotinic Acetylcholine Receptors ◽  
Acetylcholine Receptors ◽  
Surgical Interventions ◽  
Different Types ◽  
Increased Sensitivity ◽  
Tensilon Test

Introduction. Myasthenia gravis is an autoimmune disease caused by antibodies leading to the destruction of nicotinic acetylcholine receptors on the neuromuscular junction. It is characterized by muscle weakness that gets aggravated with physical activity and improves at rest. Myasthenia Gravis Foundation of America made the clinical classification of Myasthenia gravis which is still in use today. ?Tensilon test? is still the gold standard for the diagnosis of Myasthenia gravis. In addition to this test repeated muscular stimulation can be used as well as the analysis of specific autoantibodies. Treatment of Myasthenia Gravis. In conservative treatment of Mysthenia gravis anticholinesterases, immunosuppressants and plasmapheresis can be used. If conservative treatment does not lead to the desired remission, surgical treatment is indicated. The most accepted indication for thymectomy is the presence of thymoma with generalized form of Myasthenia gravis in adults. How to Distinguish Myasthenic From Cholinergic Crisis. The following is important to make a difference between these two crises: knowledge of the events that preceded the crisis, the size of pupils as well as the presence of muscarinic signs and tensilon test. Specific Features of Anesthesia in Patients with Myasthenia Gravis. Mechanism of the disease development is the reason for the increased sensitivity or resistance of these patients to certain types of drugs used in anesthesia. Protocol of Perioperative Anesthesia in Patients with Myasthenia Gravis. Based on 35 years of experience in the surgical treatment of patients with Myasthenia gravis anesthesiologists at the Department of Thoracic Surgery, Institute for Pulmonary Diseases of Vojvodina, made the protocol of anesthesia and perioperative treatment for these patients. Conclusion. Anesthesiologists may have to deal with a patient with myasthenia gravis in different types of surgical interventions. The protocol for anesthesia and perioperative management of these patients herewith presented may greatly help them in their clinical practice.

Serum metabolomics for the diagnosis and classification of myasthenia gravis

Metabolomics ◽  
2011 ◽  
Vol 8 (4) ◽  
pp. 704-713 ◽  
Author(s):  
Yonghai Lu ◽  
Chunmei Wang ◽  
Zhixi Chen ◽  
Hui Zhao ◽  
Jinyan Chen ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Diagnosis And Classification ◽  
Serum Metabolomics

scholarly journals Classification of Myasthenia Gravis Based on Autoantibody Status

2007 ◽  
Vol 64 (8) ◽  
pp. 1121 ◽  
Author(s):  
Shigeaki Suzuki ◽  
Kimiaki Utsugisawa ◽  
Yuriko Nagane ◽  
Takashi Satoh ◽  
Yasuo Terayama ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Autoantibody Status

scholarly journals Rehabilitation of the patients with myasthenia gravis as an Integral part of the patient’s Treatment Algorithm in the postoperative period

2021 ◽  
Vol 64 (3) ◽  
pp. 155-159
Author(s):  
Olena L. Tovazhnyanska ◽  
Olena M. Klimova ◽  
Hanna P. Samoilova ◽  
Dmytro V. Minukhin ◽  
Kateryna V. Ponomarova ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Treatment Algorithm ◽  
International Classification Of Diseases ◽  
Medical Sciences ◽  
Urgent Surgery ◽  
Classification Of Diseases ◽  
Icd 10 ◽  
Class Iib ◽  
Severity Of The Disease

Aim: The purpose of this article is to determine the role of rehabilitation in the structure of the treatment algorithm for patients with myasthenia gravis. materials and methods: All patients admitted to the SI «Zaycev V.T. Institute of General and Urgent surgery of National Academy of Medical Sciences of Ukraine», Kharkiv, Ukraine for surgical treatment for thymoma or carcinoma of the thymus gland. 102 people aged 18 to 69 with myasthenia have been comprehensively surveyed and their data have been analyzed and studied. Diagnosis of myasthenia was established according to the tenth edition of the International Classification of Diseases ICD-10 (WHO, 1992). Results: The severity of the patients’ condition corresponded to grade IIIA in 5 patients (16.7%), IIIB grade – 14 patients (46.7%), IVA grade – 7 patients (23.3%), IVB grade – in 4 patients (13.3%). According to the clinical classification of MGFA, the severity of the condition in most patients in this group corresponded to Class IIB (32.4%) and IIIA class (35.2%). Conclusions: The results of our study suggest that clinical and anamnestic features of myasthenia in absence of structural thymus disorders are debut before the age of 40 years, predominant affection of skeletal muscles, mild course (severity of the disease corresponds to 12.7 ± 1.76 points on the QMGS scale) and characterized by a debut after 40 years regardless of sex, manifestation of generalized muscle weakness and severe course (in 68.4% of cases severity of the disease was 31.68 ± 3.76 points on the QMGS scale).

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