Myasthenia Gravis
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  • ||||||||||  Bavencio (avelumab) / EMD Serono, Pfizer
    Journal:  Myasthenia gravis induced by avelumab. (Pubmed Central) -  May 23, 2020   
    We conclude that MG is a rare, but potentially life-threatening immune-related adverse event of avelumab therapy. This case provides support to the hypothesis that PD-1/PD-L1 signaling may have a protective role in MG.
  • ||||||||||  Journal:  Lambert-Eaton Myasthenic Syndrome Plus. (Pubmed Central) -  May 23, 2020   
    We present a patient with MLOS who tested negative for all 5 (AChR, MuSK, VGCC, Agrin, and LRP-4) serologic markers. No abstract available
  • ||||||||||  Rituxan (rituximab) / Roche, Biogen, Zenyaku Kogyo
    [VIRTUAL] SERONEGATIVE MYASTHENIA GRAVIS ASSOCIATED WITH WALDENSTROM'S MACROGLOBULINEMIA () -  May 16, 2020 - Abstract #EHA2020EHA_2313;    
    Rituximab was held to prevent an IgM flare...He received spot doses of prednisone but he was eventually started on pyridostigmine 60 mg po three times per day with improvement in his symptoms, functionality, and quality of life...Conclusion We confirm a rare association between Waldenstrom's macroglobulinemia and seronegative myasthenia gravis. Clinicians need to maintain a high degree of clinical suspicion of MG in a patient with WM in order to initate treatment promptly to improve quality of life.
  • ||||||||||  Soliris (eculizumab) / Alexion Pharma, Ultomiris IV (ravulizumab IV) / Alexion Pharma
    Formation of multivalent complexes in the presence of more than one conventional antibody to complement factor C5 () -  May 14, 2020 - Abstract #BSH2020BSH_273;    
    This highlights the importance of rigorous functional characterization of such complexes and incorporation of monitoring and mitigation processes to assess and prevent their potential formation in patients. Other methods of avoiding multivalent complexes would include switching among antibodies recognizing and binding the same epitope, such as switching from eculizumab to ravulizumab, or switching to a monovalent C5 antagonist which is unable to promote the formation of multivalent immune complexes.
  • ||||||||||  Journal, Checkpoint inhibition:  Cardiovascular Toxicities Associated with Immune Checkpoint Inhibitors. (Pubmed Central) -  May 13, 2020   
    In addition, pericardial disease and vasculitis, including temporal arteritis, have been recently described as immune-related toxicities from ICI. This narrative review describes the epidemiology, diagnosis, pathophysiology, and treatment of cardiovascular toxicities of ICI therapy, highlighting recent developments in the field in the past year.
  • ||||||||||  fasudil / Generic mfg., NT-1654 / Neurotune
    Journal:  Modulation of Agrin and RhoA Pathways Ameliorates Movement Defects and Synapse Morphology in MYO9A-Depleted Zebrafish. (Pubmed Central) -  May 13, 2020   
    In addition, treatment of zebrafish with fasudil, a ROCK inhibitor, also provided improvements to the morphology of NMJs in early development, as well as rescuing movement defects, but not to the same extent as NT1654 and not at later time points. Therefore, this study highlights a role for MYO9A at the NMJ, the first unconventional myosin motor protein associated with a neuromuscular disease, and provides a potential mechanism of action of MYO9A-pathophysiology.
  • ||||||||||  Retrospective data, Review, Journal:  Acupuncture for ocular myasthenia gravis: A protocol for systematic review and meta-analysis. (Pubmed Central) -  May 13, 2020   
    Therefore, this study highlights a role for MYO9A at the NMJ, the first unconventional myosin motor protein associated with a neuromuscular disease, and provides a potential mechanism of action of MYO9A-pathophysiology. This protocol will present the evidence of whether acupuncture is an effective and safe intervention for ocular myasthenia gravis.
  • ||||||||||  Clinical, Journal:  Under-diagnosis of neuromuscular diseases in patients of 80 years and older (Pubmed Central) -  May 9, 2020   
    Over the past 25 years, several population-based studies have been conducted in the Osona area (Barcelona), which, for the first time, has led to describing the high global incidences in the elderly, especially those over 80 years-old. The results suggest the possibility of underdiagnosis of these neuromuscular diseases in the elderly, a fact that could be especially relevant in the case of MG and GBS, since they are 2potentially reversible entities with high mortality in the event of underdiagnosis and absence of treatment.
  • ||||||||||  Keytruda (pembrolizumab) / Merck (MSD)
    Journal, Checkpoint inhibition:  Rechallenge of immune checkpoint inhibitor after pembrolizumab-induced myasthenia gravis. (Pubmed Central) -  May 9, 2020   
    The results suggest the possibility of underdiagnosis of these neuromuscular diseases in the elderly, a fact that could be especially relevant in the case of MG and GBS, since they are 2potentially reversible entities with high mortality in the event of underdiagnosis and absence of treatment. No abstract available
  • ||||||||||  Actemra IV (tocilizumab) / Roche, JW Pharma, Opdivo (nivolumab) / Ono Pharma, BMS
    Journal, Adverse events, Checkpoint inhibition:  Myositis and neuromuscular side-effects induced by immune checkpoint inhibitors. (Pubmed Central) -  May 7, 2020   
    These side-effects can be life threatening and may result in permanent sequelae. Occurrence of these side-effects must be taken into consideration for patient information, especially when considering adjuvant immunotherapy with anti-programmed cell-death protein 1 (PD-1) antibodies and monitoring, which should include regular surveillance of creatine kinase.
  • ||||||||||  Review, Journal:  S1P/S1P Receptor Signaling in Neuromuscolar Disorders. (Pubmed Central) -  May 6, 2020   
    The signaling transduced by S1P binding appears to be much more complex than previously thought, with important implications for clinical applications and for personalized medicine. In particular, the understanding of S1P/S1P receptor signaling functions in specific compartmentalized locations of the cell is worthy of being better investigated, because in various circumstances it might be crucial for the development or/and the progression of neuromuscular diseases, such as Charcot-Marie-Tooth disease, myasthenia gravis, and Duchenne muscular dystrophy.
  • ||||||||||  Impact of 1992 FDA Stereoisomer Statement on Global Drug Trends () -  May 6, 2020 - Abstract #AMCP2020AMCP_353;    
    In particular, the understanding of S1P/S1P receptor signaling functions in specific compartmentalized locations of the cell is worthy of being better investigated, because in various circumstances it might be crucial for the development or/and the progression of neuromuscular diseases, such as Charcot-Marie-Tooth disease, myasthenia gravis, and Duchenne muscular dystrophy. Pending Sponsorship: None
  • ||||||||||  Review, Journal:  Multiple MuSK signaling pathways and the aging neuromuscular junction. (Pubmed Central) -  May 1, 2020   
    However, elucidating the function of this receptor in this range of settings is challenging since MuSK participates in at least three signaling pathways: as a tyrosine kinase-dependent receptor for agrin-LRP4 and Wnts; and, as a kinase-independent BMP co-receptor. Here we focus on NMJ stability during aging and discuss open questions regarding the molecular mechanisms that govern active maintenance of the NMJ, with emphasis on MuSK and the potential role of its multiple signaling contexts.