Myasthenia Gravis
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  • ||||||||||  Review, Journal:  Class IX Myosins: Motorized RhoGAP Signaling Molecules. (Pubmed Central) -  Aug 13, 2020   
    These cellular functions affect embryonic development, adult organ homeostasis and immune responses. Human diseases associated with mutations in the two class IX myosins, Myo9a and Myo9b, have been identified, including hydrocephalus and congenital myasthenic syndrome in connection with Myo9a and autoimmune diseases in connection with Myo9b.
  • ||||||||||  tofacitinib / Generic mfg.
    Enrollment open:  Safety And Efficacy Of Tofacitinib In Patients With Refractory Myasthenia Gravis: A Pilot Study (clinicaltrials.gov) -  Aug 12, 2020   
    P1,  N=5, Recruiting, 
    Human diseases associated with mutations in the two class IX myosins, Myo9a and Myo9b, have been identified, including hydrocephalus and congenital myasthenic syndrome in connection with Myo9a and autoimmune diseases in connection with Myo9b. Not yet recruiting --> Recruiting
  • ||||||||||  tropicamide / Generic mfg.
    Journal:  Exacerbation of ocular myasthenia gravis (Pubmed Central) -  Aug 11, 2020   
    This case report describes an exacerbation of ocular myasthenia gravis after intravitreal injection using local anesthesia. A possible association with local therapeutic agents is critically discussed but cannot be excluded.
  • ||||||||||  Clinical, Journal:  Case report: cardiac herniation following robotic-assisted thymectomy. (Pubmed Central) -  Aug 11, 2020   
    This potentially fatal complication, although rare, should always be considered whenever there is hemodynamic instability entry or resection of the pericardium during surgery. We now routinely sew in a pericardial patch using our robotic surgical system for any defect over 3 cm × 3 cm that extends from the mid- to inferior portions of the heart.
  • ||||||||||  Trial completion date, Trial primary completion date:  3,4-Diaminopyridine Use in Lambert-Eaton Myasthenic Syndrome(LEMS) and Congenital Myasthenic Syndromes (CMS) (clinicaltrials.gov) -  Aug 11, 2020   
    P=N/A,  N=22, Active, not recruiting, 
    Taken together, the distinct pattern of disease association and the unique amino acid sequence of the third hypervariable region of the HLA-DRB1 provide some hints on how HLA-DRB1*16:02 is involved in the pathogenesis of autoimmune diseases. Trial completion date: Jun 2020 --> Jun 2021 | Trial primary completion date: Jun 2020 --> Jun 2021
  • ||||||||||  Review, Journal, Checkpoint inhibition:  Immune Checkpoint Inhibitor-Induced Myasthenia Gravis. (Pubmed Central) -  Aug 9, 2020   
    Treatment plans should be individualized on the basis of the extent of organ involvement and clinical severity. Additional therapeutic studies on irMG in the future are required to minimize irAE-related mortality and increase the safety of patients with cancer in the ICI era.
  • ||||||||||  Journal:  New Insights into the Exploitation of Vitis vinifera L. cv. Aglianico Leaf Extracts for Nutraceutical Purposes. (Pubmed Central) -  Aug 8, 2020   
    Regarding enzymatic inhibitory activity, ASE 50 (IC = 107.16 ± 8.12 μg/mL) and SOX (IC = 171.34 ± 12.12 μg/mL) extracts exhibited the highest AChE and BChE inhibitory activity, respectively, while UAE (IC = 293.2 ± 25.6 μg/mL, followed by SOX (IC = 302.5 ± 38.3 μg/mL) showed the highest tyrosinase inhibition value. Our results demonstrated for the first time that Aglianico leaves are important sources of phenols that could be used to prevent oxidative stress and be potentially helpful in diseases treatable with tyrosinase and cholinesterase inhibitors, like myasthenia gravis or Alzheimer's.
  • ||||||||||  Clinical, Journal:  Takotsubo Cardiomyopathy associated with myasthenic crisis: An Illustrative Case. (Pubmed Central) -  Aug 8, 2020   
    Our results demonstrated for the first time that Aglianico leaves are important sources of phenols that could be used to prevent oxidative stress and be potentially helpful in diseases treatable with tyrosinase and cholinesterase inhibitors, like myasthenia gravis or Alzheimer's. No abstract available
  • ||||||||||  batoclimab (IMVT-1401) / Roivant
    Enrollment open:  A Study to Evaluate the Efficacy, Safety and PD and PK of HBM9161 in MG Patients (clinicaltrials.gov) -  Aug 7, 2020   
    P2,  N=30, Recruiting, 
    Transcervical thymectomy, which provides overlapping myasthenia gravis remission rates vs. more invasive approaches, is equally safe and far less costly than sternotomy and video/robotic approaches. Not yet recruiting --> Recruiting
  • ||||||||||  Journal:  Diagnoses of pathological states based on acetylcholinesterase and butyrylcholinesterase. (Pubmed Central) -  Aug 6, 2020   
    But it appears that changes in these enzymes take place in other processes including oxidative stress, inflammation, some types of cancer and genetically conditioned diseases. In this review, the cholinesterases are introduced, mechanism of inhibitors action is explained and relations between the cholinesterases and pathologies are explained.
  • ||||||||||  Journal:  Congenital myasthenic syndrome caused by a frameshift insertion mutation in GFPT1. (Pubmed Central) -  Aug 6, 2020   
    Ultrastructural evaluation of the muscle biopsy from one of the patients showed extremely attenuated postsynaptic folds at neuromuscular junctions and extensive autophagic vacuolar pathology. These results expand on the spectrum of known loss-of-function GFPT1 mutations in CMS12 and in one family demonstrate a novel mode of inheritance due to UPD.
  • ||||||||||  Biomarker, Journal:  Causes and Consequences of miR-150-5p Dysregulation in Myasthenia Gravis. (Pubmed Central) -  Aug 5, 2020   
    We also showed that antimiR-150 caused increased cellular death of CD4 and CD8 T cells, along with the overexpression of pro-apoptotic genes targeted by miR-150 suggesting that miR-150 controlled the survival of these cells. Altogether, these results showed that miR-150 could play a role in MG both at the thymic level and in periphery by modulating the expression of target genes and peripheral cell survival.
  • ||||||||||  Review, Journal:  Identifying the culprits in neurological autoimmune diseases. (Pubmed Central) -  Aug 4, 2020   
    The identification of a specific culprit in NADs is challenging since a myriad of triggering factors interplay with each other to cause an autoimmune response. Among the factors that have been associated with NADs are genetic susceptibility, epigenetic mechanisms, and environmental factors such as infection, microbiota, vitamins, etc. This review focuses on the most studied culprits as well as the mechanisms used by these to trigger NADs.
  • ||||||||||  [VIRTUAL] Preferential X chromosome inactivation in women with myasthenia gravis (Interactive e-Poster Area) -  Aug 4, 2020 - Abstract #ESHG2020ESHG_1162;    
    Grant references JSPS, MHLW, AMED, and NCNP. In this study, we observed preferential XCI in MG female patients, this could suggest a potential role of this mechanism in the disease etiology, and in the increased prevalence of autoimmune disorders in females.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    Clinical, Review, Journal:  A Practical Approach to Managing Patients With Myasthenia Gravis-Opinions and a Review of the Literature. (Pubmed Central) -  Aug 1, 2020   
    Rituximab has a role in refractory MG, while plasmapheresis and immunoglobulin therapy are commonly prescribed to treat MG crisis and in some cases of refractory MG...MG patients may also develop dysfunctional breathing and the necessary respiratory physiotherapy techniques need to be implemented to alleviate the patient's symptoms of dyspnoea. In this review, we discuss various facets of myasthenia management in adults with ocular and generalized disease, including some practical approaches and our personal opinions based on our experience.
  • ||||||||||  Clinical, Journal:  Myasthenia Gravis Patient and Physician Opinions About Immunosuppressant Reduction. (Pubmed Central) -  Jul 30, 2020   
    In this review, we discuss various facets of myasthenia management in adults with ocular and generalized disease, including some practical approaches and our personal opinions based on our experience. Patients and physicians favor considering IS dose reduction but are also concerned about potential negative sequelae.
  • ||||||||||  Journal, Adverse events, Checkpoint inhibition:  Neurologic toxicity associated with immune checkpoint inhibitors: a pharmacovigilance study. (Pubmed Central) -  Jul 29, 2020   
    These observations provide pragmatic risk stratification for tumour vigilance in patients with thymomatous MG. ICIs produce a spectrum of distinct classes of neurologic AEs that can cause significant morbidity and mortality and tend to occur early and with class-specific associations.
  • ||||||||||  Clinical, Journal:  Tumor location may affect the clinicopathological features and prognosis of thymomas. (Pubmed Central) -  Jul 29, 2020   
    The clinicopathological features of thymomas on MG, Masaoka-Koga staging, disease progression, and recurrence were different between locations of superior and inferior mediastinum locations. Thymomas in the superior mediastinum tended to be associated with worse survival and increased recurrence.
  • ||||||||||  Clinical, Journal:  Autoimmune conditions and primary central nervous system lymphoma risk among older adults. (Pubmed Central) -  Jul 29, 2020   
    We utilized data from a large, nationally-representative cohort of older adults in the United States and found that PCNSL is significantly associated with systemic lupus erythematosus, polyarteritis nodusa, autoimmune hepatitis, myasthenia gravis and uveitis. Immunosuppressive drugs given to treat these conditions may increase PCNSL risk, but these associations cannot explain the observed temporal increase in PCNSL rates, given the low prevalence of these conditions.
  • ||||||||||  tacrolimus / Generic mfg.
    Review, Journal:  Novel Treatments in Myasthenia Gravis. (Pubmed Central) -  Jul 28, 2020   
    These agents include terminal complement C5 inhibitors, Fc receptor inhibitors, B cell depleting agents (anti CD 19 and 20 and B cell activating factor [BAFF)]inhibitors), proteosome inhibitors, T cells and cytokine based therapies (chimeric antigen receptor T [CART-T] cell therapy), autologous stem cell transplantation, and subcutaneous immunoglobulin (SCIG). Most of these new agents have advantages over conventional immunosuppressive treatment (IST) for MG therapy in terms of faster onset of action, favourable side effect profile and the potential for a sustained and long-term remission.
  • ||||||||||  Actemra IV (tocilizumab) / Roche, JW Pharma
    Clinical, Journal:  COVID-19 in Patients with Myasthenia Gravis. (Pubmed Central) -  Jul 24, 2020   
    Our findings suggest that the clinical course and outcomes in patients with MG and COVID-19 are highly variable. Further large studies are needed to define best practices and determinants of outcomes in this unique population.
  • ||||||||||  Clinical, Journal:  Clinical and therapeutic features of myasthenia gravis in adults based on age at onset. (Pubmed Central) -  Jul 22, 2020   
    Patients with MG are primarily ≥65 years of age with anti-AChR antibodies and no thymoma. Although patients with very-late-onset MG may present life-threatening events at onset, they achieve a good outcome with fewer immunosuppressants when diagnosed and treated properly.
  • ||||||||||  Clinical, Journal:  Congenital myasthenic syndrome: Ten years clinical experience from a quaternary care south-Indian hospital. (Pubmed Central) -  Jul 21, 2020   
    Our study and review of literature imply that CMS should be suspected in cases of seronegative myasthenia gravis cases if the onset is at less than 20 years and strongly so if the onset is within the first two years of life. In addition, a positive family history, delayed motor milestones, and a poor response to immune-modulators should be actively sought for as indicators of CMS.
  • ||||||||||  Opdivo (nivolumab) / Ono Pharma, BMS
    Journal:  Myasthenia Gravis and Myositis(PD-1 Myopathy) (Pubmed Central) -  Jul 21, 2020   
    Immunotherapy with corticosteroids was generally effective for muscle weakness with prompt normalization of serum CK levels. Myasthenia gravis and myositis induced by PD-1 inhibitors may have the common clinical features and pathological mechanisms.
  • ||||||||||  Keytruda (pembrolizumab) / Merck (MSD)
    Biomarker, Clinical, Journal, Adverse events, PD(L)-1 Biomarker, IO Biomarker:  Fatal adverse events in two thymoma patients treated with anti-PD-1 immune check point inhibitor and literature review. (Pubmed Central) -  Jul 16, 2020   
    Since myasthenia gravis symptoms interfere with therapy, patients with GS and their physicians should carefully consider the antibacterial treatment options against disseminated NTM. In thymomas, the administration of PD1 inhibitors seems to be associated with a high percentage of severe immune related adverse events, thus requiring special caution on the usage of these agents in thymomas.