Myasthenia Gravis
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  • ||||||||||  Review, Journal:  Application Prospect of Artificial Intelligence in Rehabilitation and Management of Myasthenia Gravis. (Pubmed Central) -  May 27, 2021   
    Therefore, this paper reviewed the current status of MG rehabilitation research and the difficulties faced by stable MG patients in self-management. It is suggested that we should take advantage of artificial intelligence (AI) and leverage it to develop the data-driven decision support platforms for MG management which can be used for adverse event monitoring, disease education, chronic management, and a wide variety of data collection and analysis.
  • ||||||||||  Journal:  "Afferent and Efferent Neuro-Ophthalmic Complications of Coronavirus Disease 19". (Pubmed Central) -  May 27, 2021   
    Proposed mechanisms of neurologic disease include immunologic upregulation, vasodilation and vascular permeability, endothelial dysfunction, coagulopathy, and direct viral neurotropism. When patients present to medical centers with new onset neuro-ophthalmic conditions during the pandemic, COVID-19 infection should be kept on the differential..
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    Retrospective data, Journal:  Low-dose rituximab treatment for new-onset generalized myasthenia gravis. (Pubmed Central) -  May 26, 2021   
    Clinical improvement was observed in parallel with the maintenance of low-dose corticosteroids or the complete discontinuation of corticosteroids. Long-term depletion of B cells with low-dose rituximab treatment has shown favorable efficacy and tolerance in reducing disease activity for AChR+ generalized MG.
  • ||||||||||  Journal:  Therapeutic Apheresis in Neurological Disorders (Pubmed Central) -  May 26, 2021   
    Recently, the incidence and prevalence of autoimmune encephalitis have been increasingly reported in comparison to infective encephalitis, and these often respond to therapeutic apheresis. Therapeutic apheresis for immune-mediated neurological disorders is described in this section.
  • ||||||||||  Journal:  Subxiphoid approach for robotic single-site-assisted thymectomy. (Pubmed Central) -  May 25, 2021   
    This study demonstrated that robotic single-site-assisted thymectomy via the subxiphoid approach is a safe and technically feasible procedure. Although this initial series comprised relatively simple cases, more advanced and complex procedures can soon be performed with the advent of single-port robotic platform.
  • ||||||||||  Review, Journal:  Thymus and autoimmunity. (Pubmed Central) -  May 25, 2021   
    In polygenic autoimmune diseases, subtle thymic dysfunctions may compound genetic, hormonal and environmental cues. Here, we cover (a) tolerance-inducing cell types, whether thymic epithelial or tuft cells, or dendritic, B- or thymic myoid cells; (b) tolerance-inducing mechanisms and their failure in relation to thymic anatomic compartments, and with special emphasis on human monogenic and polygenic autoimmune diseases and the related thymic pathologies, if known; (c) polymorphisms and mutations of tolerance-related genes with an impact on positive selection (e.g. the gene encoding the thymoproteasome-specific subunit, PSMB11), promiscuous gene expression (e.g. AIRE, PRKDC, FEZF2, CHD4), Treg development (e.g. SATB1, FOXP3), T-cell migration (e.g. TAGAP) and egress from the thymus (e.g. MTS1, CORO1A); (d) myasthenia gravis as the prototypic outcome of an inflamed or disordered neoplastic 'sick thymus'.
  • ||||||||||  Review, Journal, Adverse events, Checkpoint inhibition:  Neurologic Adverse Events of Immune Checkpoint Inhibitors: A Systematic Review. (Pubmed Central) -  May 22, 2021   
    These results demonstrate that rituximab is not fully effective at eliminating autoantibody-producing B cells and provide a mechanistic understanding of post-rituximab relapse in MuSK MG. Considering the increasing use of ICI therapy in the forthcoming future, this information can be valuable in assisting neurologists and oncologists in early n-irAEs diagnosis and treatment.
  • ||||||||||  Journal:  Mechanism of the Long-lasting Potentiating Effect of Distigmine on Urinary Bladder Motility (Pubmed Central) -  May 21, 2021   
    We also discuss the long-lasting potentiating effect of distigmine on urinary bladder motility and the mechanism of these effects using guinea pig urinary bladder smooth muscle in vivo and in vitro. In addition, we present our investigations on the long-lasting mechanism of distigmine using recombinant human acetylcholinesterase.
  • ||||||||||  Rystiggo (rozanolixizumab-noli) / UCB
    Trial completion date, Trial primary completion date:  A Study to Test Efficacy and Safety of Rozanolixizumab in Adult Patients With Generalized Myasthenia Gravis (clinicaltrials.gov) -  May 20, 2021   
    P3,  N=240, Recruiting, 
    In addition, we present our investigations on the long-lasting mechanism of distigmine using recombinant human acetylcholinesterase. Trial completion date: Jul 2021 --> Dec 2021 | Trial primary completion date: May 2021 --> Oct 2021
  • ||||||||||  Journal:  Impact of Coronavirus Disease 2019 in a French Cohort of Myasthenia Gravis. (Pubmed Central) -  May 20, 2021   
    However, the risk of severe COVID-19 is elevated in patients with high MGFA classes [odds ratio: 102.6 (4.4; 2,371.9)]. These results are important for establishing evidence-based guidelines for the management of MG patients during the COVID-19 pandemic.
  • ||||||||||  [VIRTUAL] Impact of age-associated decreases in thymic B cell expression of Aire and self-antigen genes on T cell tolerance () -  May 19, 2021 - Abstract #IMMUNOLOGY2021IMMUNOLOGY_1924;    
    We also used MHCII tetramers and ELISpot to detect T cells recognizing a model TRA expressed in thymic B cells, Titin (Ttn), an auto-antigen involved in late-onset myasthenia gravis, in the thymus and secondary lymphoid organs of young and aged mice. Together, our preliminary results suggest that the reduced capacity of aged thymic B cells to express Aire also impairs their ability to properly tolerize developing T cells in the thymus.
  • ||||||||||  Preclinical, Journal:  Introducing a mammalian nerve-muscle preparation ideal for physiology and microscopy, the transverse auricular muscle in the ear of the mouse. (Pubmed Central) -  May 15, 2021   
    These new observations strongly encourage further in-depth studies of ear-muscle architecture, in the many available mouse-models of various devastating human muscle-diseases. Finally, we demonstrate that the delicate and defined physical characteristics of this 'new' mammalian muscle are ideal for ultrastructural study, and thereby facilitate the imaging of synaptic vesicle membrane recycling in mammalian neuromuscular junctions, a topic that is critical to myasthenia gravis and related diseases, but which has, until now, completely eluded electron microscopic analysis.
  • ||||||||||  Soliris (eculizumab) / Alexion Pharma
    Clinical, Review, Journal:  Maintenance immunosuppression in myasthenia gravis, an update. (Pubmed Central) -  May 15, 2021   
    The practical aspects of long-term immunosuppressive therapy in MG are critically reviewed in this article. Application of one or more of these specific therapies is guided based on known efficacy, adverse effect profile, particular disease subtype and severity, and patient co-morbidities.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    Retrospective data, Journal:  Differential response to rituximab in anti-AChR and anti-MuSK positive myasthenia gravis patients: a single-center retrospective study. (Pubmed Central) -  May 15, 2021   
    Rituximab treatment response is observed in both AChR+ and MuSK+ patients supporting the role of B cell depletion in the management of MG. While there was no significant difference between these groups in terms of clinical improvement, symptom-free state, and prednisone burden, MuSK+ MG patients may experience greater benefits, including earlier time to remission, fewer exacerbations and hospitalizations post-treatment.
  • ||||||||||  azathioprine / Generic mfg.
    Journal, Adverse events:  Myasthenia gravis and azathioprine treatment: Adverse events related to thiopurine S-methyl-transferase (TPMT) polymorphisms. (Pubmed Central) -  May 15, 2021   
    Although TPMT genotype has been associated with AZA-related adverse events, since no statistically significant difference among wild-type and other TPMT genotypes for minor adverse events, our study supports the view that TPMT genotype alone is not enough to adequately personalise the AZA therapy in MG patients. In conclusion, these results were important to characterise the prevalence of TPMT gene variants in MG patients treated with AZA and correlate the adverse events of this therapy in a real-world outpatient clinic from Southern Brazil.
  • ||||||||||  Review, Journal:  ANTIBODIES AND RECEPTORS: from neuromuscular junction to central nervous system. (Pubmed Central) -  May 15, 2021   
    They form the first of a spectrum of diseases in which serum autoantibodies bind to extracellular domains of neuronal proteins throughout the nervous system and lead to constellations of clinical features including paralysis, sensory disturbance and pain, memory loss, seizures, psychiatric disturbance and movement disorders. This review will briefly summarize the ways in which this field has developed, since the 1970s when considerable contributions were made in Ricardo Miledi's laboratory at UCL.