Myasthenia Gravis
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  • ||||||||||  Clinical, Journal:  Seronegative myasthenic crisis: a multicenter analysis. (Pubmed Central) -  Jun 25, 2022   
    We found no differences in duration of mechanical ventilation (16.2 ± 15.8 vs 16.5 ± 15.9 days; p = 0.94) and length of stay at intensive care unit (17.6 ± 15.2 vs 17.8 ± 15.4 days; p = 0.96), or in-hospital mortality (11.8% vs. 10.1%; p = 0.69). We conclude that MC in seronegative MG affects younger patients after a longer period of disease, but that crisis treatment efficacy and outcome do not differ compared to AChR-MCs.
  • ||||||||||  Review, Journal:  Physiological Roles of the Autoantibodies to the 78-Kilodalton Glucose-Regulated Protein (GRP78) in Cancer and Autoimmune Diseases. (Pubmed Central) -  Jun 25, 2022   
    Although csGRP78 is common in both cancer and autoimmune diseases, there are major differences in the specificity of its autoantibodies. Here, we discuss how ER mechanisms modulate csGRP78 antigenicity and the production of autoantibodies, permitting this chaperone to function as a dual compartmentalized receptor with independent signaling pathways that promote either pro-proliferative or apoptotic signaling, depending on whether the autoantibodies bind csGRP78 N- or C-terminal regions.
  • ||||||||||  Journal:  Complement Activation Profile in Myasthenia Gravis Patients: Perspectives for Tailoring Anti-Complement Therapy. (Pubmed Central) -  Jun 25, 2022   
    We obtained sensitivity and specificity performance results suggesting plasma C2, C3, C3b, and C5 as biomarkers for AChR-MG. Our findings reveal a plasma complement "C2, C3, C5, C3b, and C5a" profile associated with AChR-MG to be further investigated as a biomarker of complement activation status in AChR-MG patients, opening new perspectives for tailoring of anti-complement therapies to improve the disease treatment.
  • ||||||||||  Journal:  Mitochondrial bioenergetics in ocular fibroblasts of two myasthenia gravis cases. (Pubmed Central) -  Jun 25, 2022   
    Taken together we showed similar basal and metabolic adaptive responses after exposure to mitochondrial inhibitors in ocular fibroblasts derived from OP-MG cases and controls, although the OP-MG cells showed greater activation in response to MG conditions. These pilot results in orbital-derived tissues provide support for myasthenic-induced changes in cellular metabolism and evidence that orbital fibroblasts may be useful for dynamic bioenergetic assessments.
  • ||||||||||  Journal:  Electrodiagnostic Characteristics Suggestive of Muscle-Specific Kinase Myasthenia Gravis. (Pubmed Central) -  Jun 25, 2022   
    Myopathic appearing MUPs were found in milder cases of MuSK + MG (MGFA class I-IIB) compared with AChR + MG (MGFA Class IIB-V). Patients with MuSK + MG may have a recognizable EDX profile from AchR + MG that includes (1) myotonic discharges, (2) greater occurrence of myopathic appearing MUPs in clinically mild disease, and (3) symptoms leading to earlier testing.
  • ||||||||||  Journal:  Development and validation of the Myasthenia Gravis TeleScore (MGTS). (Pubmed Central) -  Jun 24, 2022   
    Patients with MuSK + MG may have a recognizable EDX profile from AchR + MG that includes (1) myotonic discharges, (2) greater occurrence of myopathic appearing MUPs in clinically mild disease, and (3) symptoms leading to earlier testing. The MGTS demonstrated a good correlation with INCB-MG, reliability and construct validity.
  • ||||||||||  Activated Pathways of Myastenia Gravis in Thymic Epithelial (TETs) (Hall C8) -  Jun 24, 2022 - Abstract #IASLCWCLC2022IASLC_WCLC_862;    
    TET with MG have suppressed invasion and metastatic pathways, but no AIDs specific activation pathways were found. The analysis shows new acknowledge of MG activation pathways in TETs.
  • ||||||||||  Journal:  Bilateral Vocal Cord Palsy as the Only Symptom of Thymoma Associated-Myasthenia Gravis. (Pubmed Central) -  Jun 24, 2022   
    In our case, a 58-year-old woman presented in the emergency room with solely dyspnea, caused by bilateral cord palsy, and that appeared to be the only symptom of thymoma associated-myasthenia gravis. Another interesting fact about this case is the quick recovery and no need for tracheostomy and intubation in the first hours of her admission to hospital.
  • ||||||||||  Clinical, Review, Journal:  Pharmacotherapy of Generalized Myasthenia Gravis with Special Emphasis on Newer Biologicals. (Pubmed Central) -  Jun 23, 2022   
    Among novel targets, interleukin 6 (IL6) inhibitors such as satralizumab are promising and currently undergoing evaluation...While MG treatment is moving beyond conventional agents towards target-specific biologicals, lack of knowledge as to the initiation, maintenance, switching, tapering and long-term safety profile necessitates further research. These concerns and the high financial burden of novel agents may hamper widespread clinical use in the near future.
  • ||||||||||  prednisone / Generic mfg.
    Retrospective data, Journal:  Key Factors Associated With Administration of Ventilator Support After Thymoma Resection. (Pubmed Central) -  Jun 22, 2022   
    Our data suggest that median sternotomy, MG grade three status, and preoperative use of anti-MG drugs are associated with greater incidence of severe respiratory failure requiring respiratory support after thymoma surgery. Therefore, patients with these risk factors should be closely monitored to reduce the incidence of severe postoperative respiratory failure.
  • ||||||||||  Review, Journal:  Acute neuromuscular syndromes with respiratory failure during COVID-19 pandemic: Where we stand and challenges ahead. (Pubmed Central) -  Jun 22, 2022   
    Some patients with COVID-19 may present with concurrent acute neuromuscular syndromes such as myasthenic crisis (MC), Guillain-Barré syndrome (GBS) and idiopathic inflammatory myopathies (IIM); these conditions coupled with respiratory failure could trigger a life-threatening condition. Here, we review the current state of knowledge on acute neuromuscular syndromes with respiratory failure related to COVID-19 infection in an attempt to clarify and to manage the muscle dysfunction overlapping SARS-COV-2 infection.
  • ||||||||||  Biomarker, Journal:  Machine Learning Analysis Reveals Biomarkers for the Detection of Neurological Diseases. (Pubmed Central) -  Jun 22, 2022   
    This indicates overlapping genetic characterizations among NLDs which challenges current clinical definitions of the neurological disorders. Taken together, this work demonstrates the value of data-driven approaches to identify novel biomarkers in the absence of any known or promising biomarkers.
  • ||||||||||  TOL 2 / Toleranzia
    Journal:  A Recombinant Acetylcholine Receptor α1 Subunit Extracellular Domain Is a Promising New Drug Candidate for Treatment Of Myasthenia Gravis. (Pubmed Central) -  Jun 22, 2022   
    We hypothesized that an efficacious drug candidate for antigen-specific therapy in MG should comprise a broad range of these auto-epitopes and be administered in a noninflammatory and tolerogenic context...Further, we did not observe any signs of toxicity or intolerability in animals treated with α1-ECD We conclude that intravenous treatment with α1-ECD is safe and effective in suppressing EAMG. α1-ECD is in preclinical development as a promising new drug candidate for MG.
  • ||||||||||  Feasibility of Peak Inspiratory Flow measurement in neuromuscular patients (TP-3 in thematic poster area) -  Jun 22, 2022 - Abstract #ERS2022ERS_912;    
    With this method PIF can be measured reproducibly and reliably in neuromuscular patients. This quantitative assessment of PIF could help to screen for inspiratory muscle weakness, obviating the need for additional MIP measurement.
  • ||||||||||  Home noninvasive ventilation in Myasthenia Gravis: features and predictors. (TP-6 in thematic poster area) -  Jun 22, 2022 - Abstract #ERS2022ERS_253;    
    FVC is the only parameter that could predict the need of NIV. Previous to NIV initiation After NIV initiation p pH 7,06+/-0,98 7,42+/-0,02 0,311 PaO2(mmHg) 79,33+/-21,94 85,66+/-8,41 0,352 PaCO2(mmHg) 47,33+/-7,24 39,00+/-3,50 0,029 HCO3(mmol/L) 28,12+/-1,96 24,62+/-2,13 0,008
  • ||||||||||  Enrollment open:  Acupuncture in Myasthenia Gravis (AcuMG) (clinicaltrials.gov) -  Jun 21, 2022   
    P=N/A,  N=20, Recruiting, 
    Furthermore, she was seen regularly in recall visits every 3 months. Not yet recruiting --> Recruiting
  • ||||||||||  Retrospective data, Journal:  Orthostatic myoclonus - A retrospective study of Asian patients. (Pubmed Central) -  Jun 19, 2022   
    It is associated with central and peripheral nerve system disorders, systemic diseases, and medication use. We hypothesize that OM is a pathological disintegration of motor circuit.