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Update in the Management of Myasthenia Gravis
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Update in the Management of Myasthenia Gravis

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Clinical Neurology".

Deadline for manuscript submissions: closed (15 September 2022) | Viewed by 13370

Special Issue Editor

Prof. Dr. Carlos Casasnovas

E-Mail Website
Guest Editor
1. Neuromuscular unit, Department of Neurology, Hospital Universitari de Bellvitge, Barcelona, Spain
2. Neurometabolic Diseases Group, Bellvitge Biomedical Research Institute (IDIBELL) and CIBERER, Barcelona, Spain
Interests: neuropathy; clinical neurology; polyneuropathies; Guillain–Barre syndrome; myasthenia gravis; peripheral neuropathy

Special Issue Information

Dear Colleagues,

Myasthenia gravis is the most frequent neuromuscular junction disorder. There has been progress in the understanding of the pathophysiology, diagnosis, and treatment of this condition. The ongoing progress of treatments for myasthenia gravis has improved the prognosis and quality of life of patients. However, despite these new treatments, there are still between 10 and 15% of patients who are refractory to current therapies, which forces us to continue to improve the pharmacological and non-pharmacological management of the disease with an approach that includes all the professionals involved.

The Journal of Clinical Medicine is planning a Special Issue titled “Advances in the Management of Myasthenia Gravis”. It is our great privilege to invite you to contribute to this Special Issue. We are interested in clinical and epidemiological studies and systematic reviews with a focus on the management of myasthenia gravis.

Prof. Dr. Carlos Casasnovas
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • Myasthenia gravis
  • Immunosuppressant
  • IVIg
  • Plasmapheresis
  • Thymectomy
  • Corticosteroids
  • Eculizumab

Published Papers (5 papers)

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Research

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10 pages, 572 KiB  
Article
Myasthenia Gravis Induced by Immune Checkpoint Inhibitors: An Emerging Neurotoxicity in Neuro-Oncology Practice: Case Series
by Carla Marco, Marta Simó, Montse Alemany, Carlos Casasnovas, Raúl Domínguez, Noelia Vilariño, Mariona Calvo, Juan Martín-Liberal, Jesús Brenes, Joan Sabater-Riera, Jordi Bruna and Roser Velasco
J. Clin. Med. 2023, 12(1), 130; https://doi.org/10.3390/jcm12010130 - 24 Dec 2022
Cited by 6 | Viewed by 2570
Abstract
Immunotherapy with immune checkpoint inhibitors (ICIs) have been reported to induce de novo or exacerbate pre-existing Myasthenia Gravis (MG). We present a single center case series of patients who developed an immune-related myasthenia gravis (irMG) related with ICIs. We performed a retrospective chart [...] Read more.
Immunotherapy with immune checkpoint inhibitors (ICIs) have been reported to induce de novo or exacerbate pre-existing Myasthenia Gravis (MG). We present a single center case series of patients who developed an immune-related myasthenia gravis (irMG) related with ICIs. We performed a retrospective chart review of the electronic medical records between 1 September 2017 and 2022. We report the clinical features, presentation forms, diagnostic workflows, general management and outcomes of six patients who received ICIs for different solid organ malignancies and developed an irMG frequently overlapping with immune-related myocarditis and/or myositis. The aim of the article is to describe the clinical features, treatment and outcomes of this challenging and potentially life-threating syndrome, comparing our data with those described in the literature. Differences between irMG and classic MG are highlighted. Full article
(This article belongs to the Special Issue Update in the Management of Myasthenia Gravis)
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10 pages, 1457 KiB  
Article
Myocardial Injury in Hospitalized Patients with Myasthenia Gravis
by Hongxi Chen, Lingyao Kong, Ying Zhang, Xue Lin, Ziyan Shi, Qin Du, Xiaofei Wang, Yanlin Lang, Linjun Cai, Zichao Mou, Wenqin Luo, Shuangjie Li and Hongyu Zhou
J. Clin. Med. 2022, 11(23), 7106; https://doi.org/10.3390/jcm11237106 - 30 Nov 2022
Viewed by 1401
Abstract
Objective: To investigate the clinical characteristics and outcome of myocardial injury in patients with myasthenia gravis (MG). Methods: We retrospectively searched medical records to screen hospitalized patients with MG at our hospital. The troponin T (TnT) levels were deemed necessary to be performed [...] Read more.
Objective: To investigate the clinical characteristics and outcome of myocardial injury in patients with myasthenia gravis (MG). Methods: We retrospectively searched medical records to screen hospitalized patients with MG at our hospital. The troponin T (TnT) levels were deemed necessary to be performed based on the patient’s clinical symptoms and were used as biomarkers of myocardial injury. The patients’ demographic and clinical information were collected. Death was the primary outcome. Results: A total of 336 patients with MG measured TnT levels and were included in the final analysis. The male MG patients with elevated TnT levels had a higher prevalence of infection (56.8% vs. 30.0%, p = 0.001) and myasthenic crisis (37.5% vs. 13.3%, p = 0.001) than those with normal TnT levels. Meanwhile, the female MG patients with elevated TnT levels were older (56.0 (16.6) vs. 49.2 (17.2)) years old, p = 0.007] and had a higher prevalence of infection (65.4% vs. 32.1%, p < 0.001), myasthenic crisis (33.6% vs. 17.9%, p = 0.015), and thymoma (38.5% vs. 16.7%, p = 0.001) than those with normal TnT levels. Older age (coef. = 0.004; p = 0.034), infection (coef. = 0.240; p = 0.001), myasthenic crisis (coef. = 0.312; p < 0.001), thymoma (coef. = 0.228; p = 0.001), and ICI therapy (coef. = 1.220; p < 0.001) were independent risk predictors for increasing log TnT levels. Thirty-seven patients died during hospitalization. High log TnT levels (OR = 8.818; p < 0.001), female sex (OR = 0.346; p = 0.023), thymoma (OR = 5.092; p = 0.002), and infection (OR = 14.597; p < 0.001) were independent risk predictors of death. Conclusions: Our study revealed that the surveillance of myocardial injury biomarkers in MG patients might be beneficial. Full article
(This article belongs to the Special Issue Update in the Management of Myasthenia Gravis)
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10 pages, 536 KiB  
Article
Our Clinical Experience in the Treatment of Myasthenia Gravis Acute Exacerbations with a Novel Nanomembrane-Based Therapeutic Plasma Exchange Technology
by Dimitar Tonev, Radostina Georgieva and Evgeniy Vavrek
J. Clin. Med. 2022, 11(14), 4021; https://doi.org/10.3390/jcm11144021 - 12 Jul 2022
Cited by 5 | Viewed by 1563
Abstract
According to the American Academy of Neurology 2011 guidelines, there is insufficient evidence to support or refute the use of therapeutic plasma exchange (TPE) for myasthenia gravis (MG). The goal of this study was to determine whether a novel nanomembrane-based TPE could be [...] Read more.
According to the American Academy of Neurology 2011 guidelines, there is insufficient evidence to support or refute the use of therapeutic plasma exchange (TPE) for myasthenia gravis (MG). The goal of this study was to determine whether a novel nanomembrane-based TPE could be useful in the treatment of MG. Thirty-six adult patients, MGFA 4/4B and 5, with acute MG episodes were enrolled into a single-center retrospective before-and-after study to compare a conventional treatment group (n = 24) with a nanomembrane-based TPE group (n = 12). TPE or intravenous immunoglobulins (IVIG) infusions were used in impending/manifested myasthenic crises, especially in patients at high-risk for prolonged invasive ventilation (IMV) and in those tolerating non-invasive ventilation (NIV). The clinical improvement was assessed using the Myasthenia Muscle Score (0–100), with ≥20 increase for responders. The primary outcome measures included the rates of implemented TPE, IVIG, and corticosteroids immunotherapies, NIV/IMV, early tracheotomy, MMS scores, extubation time, neuro-ICU/hospital LOS, complications, and mortality rates. The univariate analysis found that IMV was lower in the nanomembrane-based group (42%) compared to the conventional treatment group (83%) (p = 0.02). The multivariate analysis using binary logistic regression revealed TPE and NIV as independent predictors for short-term (≤7 days) respiratory support (p = 0.014 for TPE; p = 0.002 for NIV). The novel TPE technology moved our clinical practice towards proactive rather than protective treatment in reducing prolonged IMV during MG acute exacerbations. Full article
(This article belongs to the Special Issue Update in the Management of Myasthenia Gravis)
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15 pages, 1948 KiB  
Article
Association of Cardiac Autonomic Responses with Clinical Outcomes of Myasthenia Gravis: Short-Term Analysis of the Heart-Rate and Blood Pressure Variability
by Monika Zawadka-Kunikowska, Łukasz Rzepiński, Małgorzata Tafil-Klawe, Jacek J. Klawe, Paweł Zalewski and Joanna Słomko
J. Clin. Med. 2022, 11(13), 3697; https://doi.org/10.3390/jcm11133697 - 27 Jun 2022
Cited by 5 | Viewed by 1747
Abstract
Introduction: The aim of the study was to assess cardiac and autonomic function in patients with myasthenia gravis (MG) and to explore its relationship with disease outcomes. Methods: Thirty-eight patients with an MG were enrolled (median age 40.5 years; median disease duration 5.5 [...] Read more.
Introduction: The aim of the study was to assess cardiac and autonomic function in patients with myasthenia gravis (MG) and to explore its relationship with disease outcomes. Methods: Thirty-eight patients with an MG were enrolled (median age 40.5 years; median disease duration 5.5 years). Cardiovascular parameters, baroreflex sensitivity (BRS), spectral indices of short-term heart rate (HRV), and systolic blood pressure variability (SBPV) were compared with age- and gender-matched controls (n = 30). Cardiac autonomic function was assessed during the response to standing (tilt) and deep breathing tests (expiration/inspiration ratio-E/I). Results: HR and BP responses to the tilt test were similar in both groups. MG patients, as compared to controls, were characterized by altered SBPV at rest, significantly reduced HR response to the deep breathing test (p < 0.001), increased sympathovagal balance after tilt (delta LF/HF-RRI, p = 0.037), and lower values of BRS (p = 0.007) and hemodynamic parameters, i.e., cardiac index, index contractility, left ventricular work index, at rest and during tilt. There was no association between disease duration and autonomic parameters. Disease severity, as determined by MGFA (Myasthenia Gravis Foundation of America) corrected for age and sex, was an independent predictor of diminished vagal tone (E/I ratio) and increased sympathetic response to tilt (delta LF/HF-RRI) as measured with HRV. Lower BRS was associated with greater disease severity and older age. Hemodynamic parameters were predominantly predicted by age and sex. Conclusion: Our results confirm cardiac autonomic dysfunction among MG patients with predominant parasympathetic impairment. Clinicians should consider evaluation of autonomic balance in MG patients with, or at risk for, cardiovascular disease. Full article
(This article belongs to the Special Issue Update in the Management of Myasthenia Gravis)
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Review

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20 pages, 1887 KiB  
Review
New Targeted Agents in Myasthenia Gravis and Future Therapeutic Strategies
by Daniel Sánchez-Tejerina, Javier Sotoca, Arnau Llaurado, Veronica López-Diego, Raul Juntas-Morales and Maria Salvado
J. Clin. Med. 2022, 11(21), 6394; https://doi.org/10.3390/jcm11216394 - 28 Oct 2022
Cited by 8 | Viewed by 5294
Abstract
Myasthenia gravis (MG) is a chronic autoimmune disease for which multiple immunomodulatory therapies are available. Nevertheless, MG has a significant impact on patient quality of life. In recent years, experts’ main efforts have focused on optimizing treatment strategies, since disease burden is considerably [...] Read more.
Myasthenia gravis (MG) is a chronic autoimmune disease for which multiple immunomodulatory therapies are available. Nevertheless, MG has a significant impact on patient quality of life. In recent years, experts’ main efforts have focused on optimizing treatment strategies, since disease burden is considerably affected by their safety and tolerability profiles, especially in patients with refractory phenotypes. This article aims to offer neurologists caring for MG patients an overview of the most innovative targeted drugs specifically designed for this disease and summarizes the recent literature and more recent evidence on agents targeting B cells and plasmablasts, complement inhibitors, and neonatal fragment crystallizable receptor (FcRn) antagonists. Positive clinical trial results have been reported, and other studies are ongoing. Finally, we briefly discuss how the introduction of these novel targeted immunological therapies in a changing management paradigm would affect not only clinical outcomes, disease burden, safety, and tolerability, but also health spending in a condition that is increasingly managed based on a patient-centred model. Full article
(This article belongs to the Special Issue Update in the Management of Myasthenia Gravis)
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