Myasthenia Gravis
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  • ||||||||||  Journal:  A Rare Case of Metaplastic Thymoma Presenting With Myasthenia Gravis. (Pubmed Central) -  Jan 23, 2024   
    A YAP1::MAML2 gene fusion, with an in-frame fusion between genes YAP1 Exon5 (NM_001130145) and MAML2 Exon2 (NM_032427) was found, supporting further the diagnosis of metaplastic thymoma (Anchored multiplex RNA sequencing [Archer Dx, Boulder, CO] assay). The patient's gender and relatively young age, the presence of an autoimmune condition, and the lack of lymphocytic infiltrate all contribute unusual features to this case and suggest avenues for further exploration.
  • ||||||||||  Actemra IV (tocilizumab) / Roche, JW Pharma
    Enrollment status, Phase classification:  tMG-E: Open-Label Extension of Tocilizumab in Subjects With Generalized Myasthenia Gravis (clinicaltrials.gov) -  Jan 22, 2024   
    P2/3,  N=64, Recruiting, 
    The patient's gender and relatively young age, the presence of an autoimmune condition, and the lack of lymphocytic infiltrate all contribute unusual features to this case and suggest avenues for further exploration. Enrolling by invitation --> Recruiting | Phase classification: P3 --> P2/3
  • ||||||||||  Botox (onabotulinumtoxin A) / GSK, AbbVie, Healis Therap
    Case Report of Myasthenia Gravis in a Patient With Muscular Dystonia () -  Jan 22, 2024 - Abstract #TOXINS2024TOXINS_299;    
    At the time of the last injection on 12/01/2022, 100 units of onabotulinumtoxinA were administered...The patient was diagnosed with generalized myasthenia gravis and muscular myoclonus-dystonia. Conclusions 1.Always remember the possibility of the onset of a neuromuscular disorder in people receiving BoNT-A, including those with muscular dystonia.2.Dysphagia, semi-ptosis, weakness in the neck muscles, and general weakness may not always be a manifestation of side effects of botulinum neurotoxin therapy.3.MG is a contraindication to BoNT therapy, but with proper treatment of concomitant MG, BoNT can be successfully used.
  • ||||||||||  Journal:  Association between myasthenia gravis and Alzheimer's disease. (Pubmed Central) -  Jan 19, 2024   
    Conclusions 1.Always remember the possibility of the onset of a neuromuscular disorder in people receiving BoNT-A, including those with muscular dystonia.2.Dysphagia, semi-ptosis, weakness in the neck muscles, and general weakness may not always be a manifestation of side effects of botulinum neurotoxin therapy.3.MG is a contraindication to BoNT therapy, but with proper treatment of concomitant MG, BoNT can be successfully used. Our results suggest that there might be an increase in the prevalence of AD in patients previously diagnosed with MG.
  • ||||||||||  Journal:  The Burden Patients with (Pubmed Central) -  Jan 19, 2024   
    A considerable burden was observed in this comparison of breathing, sleep, fatigue, mental health, and usual activities between patients with MG and the general population, using data from two international studies and published population norms. Even mildly affected patients had significantly worse outcomes than the general population.
  • ||||||||||  Journal:  Evaluation of Medication Exposure on Exacerbation of Disease in Patients With Myasthenia Gravis. (Pubmed Central) -  Jan 18, 2024   
    In 5/7 events, at least 1 other risk factor associated with a myasthenic exacerbation was present. Of the medications reported to potentially worsen MG, intravenous labetalol and intravenous magnesium were the 2 agents associated with myasthenic exacerbations with a higher incidence in patients harboring additional risk factors.
  • ||||||||||  Journal:  Diaphragmatic ultrasonography as a predictor of respiratory muscle fatigue in myasthenia gravis. (Pubmed Central) -  Jan 18, 2024   
    Of the medications reported to potentially worsen MG, intravenous labetalol and intravenous magnesium were the 2 agents associated with myasthenic exacerbations with a higher incidence in patients harboring additional risk factors. DUS could identify diaphragm fatiguability in GMG patients, which may be more reliable and sensitive in assessment of diaphragm fatigue than conventional methods.
  • ||||||||||  Observational data, Journal:  Burden of illness and costs in patients with myasthenia gravis currently receiving treatment in the United States. (Pubmed Central) -  Jan 18, 2024   
    The updated prevalence and incidence of MG in the United States in 2021 are higher than previous reports from the 1980s and early 2000s, following a global trend of increased prevalence and incidence for this disorder in the last few decades. The burden of crises/exacerbations was substantially higher and healthcare costs were considerably greater in patients with MG treated with chronic IVIg or multiple NSISTs than in patients not receiving these treatments.
  • ||||||||||  Journal:  Sound-based cough peak flow estimation in patients with neuromuscular disorders. (Pubmed Central) -  Jan 18, 2024   
    Our findings suggest that sounds measured with a smartphone app provide a reliable estimate of CPF in patients with neuromuscular disorders, even in the presence of with orofacial weakness. This may be a convenient way to monitor respiratory involvement in patients with neuromuscular disorders, but larger studies of more diverse patient cohorts are needed.
  • ||||||||||  Zilbrysq (zilucoplan) / UCB
    Review, Journal:  Zilucoplan: First Approval. (Pubmed Central) -  Jan 17, 2024   
    Zilucoplan is also currently under regulatory review in Australia and Canada for use in the treatment of gMG. This article summarises the milestones in the development of zilucoplan leading to this first approval for gMG.
  • ||||||||||  batoclimab (IMVT-1401) / Roivant
    Clinical, P2a data, Journal:  Subcutaneous batoclimab in generalized myasthenia gravis: Results from a Phase 2a trial with an open-label extension. (Pubmed Central) -  Jan 17, 2024   
    This article summarises the milestones in the development of zilucoplan leading to this first approval for gMG. The safety profile, pharmacodynamics, and preliminary clinical benefits observed in this study support further investigation of subcutaneous batoclimab injections as a potential patient-administered therapy for seropositive generalized myasthenia gravis.
  • ||||||||||  dexamethasone / Generic mfg.
    Journal:  Dexamethasone improves thymoma-associated myasthenia gravis via the AKT-mTOR pathway. (Pubmed Central) -  Jan 17, 2024   
    This study demonstrates that dexamethasone can promote apoptosis through the AKT-mTOR pathway for the treatment of thymoma-associated MG, as validated by network pharmacology predictions and clinical specimen experiments, and can be verified by large-scale clinical trials in the future. This study also provides theoretical support and new research perspectives for this disease.
  • ||||||||||  Review, Journal:  Complement and MHC patterns can provide the diagnostic framework for inflammatory neuromuscular diseases. (Pubmed Central) -  Jan 16, 2024   
    Our approach acknowledges the current limitations in the field of inflammatory neuromuscular diseases, particularly the scarcity of large-scale, prospective studies that validate the diagnostic potential of these markers. Further efforts are needed to establish a consensus on the diagnostic protocol to effectively distinguish these diseases.
  • ||||||||||  Review, Journal:  SARS-CoV-2 Vaccination and Neuroimmunological Disease: A Review. (Pubmed Central) -  Jan 16, 2024   
    The associated risk between SARS-CoV-2 vaccination and GBS, and possibly Bell palsy, is slight, and this should not change the recommendation for individuals to be vaccinated. The same advice should be given to those with preexisting neurological autoimmune disease.
  • ||||||||||  Biomarker, Journal:  Antibody Response to HERV-K and HERV-W Envelope Epitopes in Patients with Myasthenia Gravis. (Pubmed Central) -  Jan 15, 2024   
    These findings lay the foundation for future investigations aimed at elucidating the molecular mechanisms driving this immune response. The detection of these autoantibodies suggests the potential for novel biomarkers, especially within the MG-DSN patient subgroup, addressing the need for new biomarkers in this population.
  • ||||||||||  Observational data, Journal:  Correlation of fatigue on walking ability in myasthenia gravis patients: a cross-sectional study. (Pubmed Central) -  Jan 15, 2024   
    No significant correlation of fatigue on walking ability including FSS vs. CWS (r=-0.141; P=0.520), FSS vs MWS (r=-0.169; P=0.442), and FSS vs. natural cadence (r=-0.050; P=0.822). There was no significant correlation between fatigue and walking ability in MG patients who had MGFA 1, MGFA 2A, and MGFA 2B.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Holdings, Roche
    Journal:  Mapping current trends and hotspots in myasthenia gravis from 2003 to 2022: a bibliometric analysis. (Pubmed Central) -  Jan 12, 2024   
    The keyword with the highest strength is "neuromuscular transmission," followed by "safety" and "rituximab." Co-citation analysis includes 103 publications cited at least 65 times, categorized into four clusters...This bibliometric analysis shows the framework of research over the past 20?years by mapping the scholarly contributions of various countries or regions, institutions, journals, and authors in MG. The analysis also explores future trends and prospective directions, emphasizing individualized treatment based on subtypes, novel immunotherapeutic approaches, and thymectomy.
  • ||||||||||  Biomarker, Journal:  Study of serum exosome miRNA as a biomarker for early onset adult ouclar myasthenia gravis. (Pubmed Central) -  Jan 12, 2024   
    The analysis also explores future trends and prospective directions, emphasizing individualized treatment based on subtypes, novel immunotherapeutic approaches, and thymectomy. The present study identified three novel miRNAs as candidate biomarkers for early-onset OMG patients and it was expected to provide a possibility and a new orientation for serum exosomal miRNAs as OMG diagnostic biomarkers.
  • ||||||||||  Journal:  Exploring Factors That Prolong the Diagnosis of Myasthenia Gravis. (Pubmed Central) -  Jan 11, 2024   
    Patient and clinician education about MG and outpatient diagnostic efficiency needs emphasis. Further studies are also needed to explore the delayed presentation time of women and nonworking patients in MG.
  • ||||||||||  Review, Journal, PD(L)-1 Biomarker, IO biomarker:  Molecular and Functional Key Features and Oncogenic Drivers in Thymic Carcinomas. (Pubmed Central) -  Jan 11, 2024   
    Anti-angiogenic drugs and immune checkpoint inhibitors targeting the PD-1/PD-L1 axis have shown promising results for the treatment of unresectable tumors. Since thymic carcinomas are frankly aggressive tumors, this report presents insights into their oncogenic drivers, categorized under the established hallmarks of cancer.
  • ||||||||||  Review, Journal:  Dysregulated Long Non-coding RNAs in Myasthenia Gravis- A Mini-Review. (Pubmed Central) -  Jan 9, 2024   
    Moreover, with the development of lncRNA expression regulation technology, it is possible to regulate the differentiation of immune cells and influence the immune response by regulating the expression of lncRNAs, which will provide a potential therapeutic option for MG. Here, we review the research progress on the role of lncRNAs in different pathophysiological events contributing to MG, focusing on specific lncRNAs that may largely contribute to the pathophysiology of MG, which could be used as potential diagnostic biomarkers and therapeutic targets.
  • ||||||||||  Journal:  Acetylcholine Receptor Antibody Neutralization and Selective B-Cell Suppression Using Fc Fusion Protein (Pubmed Central) -  Jan 9, 2024   
    Based on the concept of "selective removal of pathogenic antibodies and cells without suppression of normal immunity," we are developing a fusion protein referred to as AChR-Fc (composed of the AChR alpha subunit and Fc region of human immunoglobulin G1), which shows the following mechanisms of action: selective neutralization of AChR antibodies and cytotoxic activity against AChR antibody-producing pathogenic B cells. Treatment with AChR-Fc is a novel therapeutic approach that may be useful in the management of MG.
  • ||||||||||  Journal:  Pathogenesis of MuSK Antibody-positive Myasthenia Gravis (Pubmed Central) -  Jan 9, 2024   
    Recent studies that have investigated the pathogenesis of MuSK antibody-positive MG have reported contributors to treatment refractoriness in cases of MG. In this article, we review the most recent findings.
  • ||||||||||  Journal:  Congenital Myasthenic Syndromes (Pubmed Central) -  Jan 9, 2024   
    Tubular aggregates are observed in muscle biopsy in four forms of CMS, and serum CK levels are elevated in some forms of CMS. As rational therapies are available for most forms of CMS, identification of causative gene variants by genetic analysis is required.
  • ||||||||||  Journal:  Diagnosis and Treatment of Lambert-Eaton Myasthenic Syndrome (Pubmed Central) -  Jan 9, 2024   
    The first Japanese guideline for LEMS was developed in May 2022 as MG/LEMS Practice Guideline 2022. This article describes the epidemiology, symptoms, diagnosis, examination, treatment, and prognosis of this condition, based on the LEMS guidelines.
  • ||||||||||  Journal, Adverse events, IO biomarker:  Clinical Features of Myasthenia Gravis as an Immune-related Adverse Event (Pubmed Central) -  Jan 9, 2024   
    Immunotherapy with corticosteroids is effective for management of irAE-MG and should be initiated promptly. Collaboration between consulting neurologists is necessary for safe management of cancer immunotherapy.
  • ||||||||||  Journal:  Treatment of Juvenile Myasthenia Gravis (Pubmed Central) -  Jan 9, 2024   
    Steroid use in children in particular requires caution due to the risk of growth retardation, and while lower doses are desirable, the efficacy of lower doses has not been as fully investigated as in adults. Conversely, experience has shown that the use of adequate doses of steroids results in high remission rates, and there is currently no rationale for recommending lower doses.
  • ||||||||||  Journal:  Treatment and Management of Myasthenia Gravis in Adults (Pubmed Central) -  Jan 9, 2024   
    Following the availability of a variety of treatments and the increasing incidence of MG in the aging population, the scenario of MG treatments has changed significantly. In this study, we review the various treatment strategies and management of MG in adults.
  • ||||||||||  Review, Journal:  Myasthenia Gravis: A Systematic Review. (Pubmed Central) -  Jan 8, 2024   
    Improved knowledge of this relatively uncommon but curable condition is required among primary carers. The objective of this review is to provide information about MG and to help people recognize its symptoms and start treatment without panic so that the progression of this disease can be stopped and complications can be avoided.