Myasthenia Gravis
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  • ||||||||||  C185 - Spanish: Neuroimmunology (Both in-person and online) -  Jan 7, 2023 - Abstract #AAN2023AAN_670;    
    Participants should become familiar with the current therapeutic challenges in antibody-associated neurologic disorders and with the diverse ongoing clinical trials that will change the landscape of therapeutic options. In particular, participants should be able to recognize new therapeutic targets in autoimmune encephalitis and myasthenia gravis, and develop strategies to manage immune-related adverse events of immune checkpoint inhibitors in oncologic patients.
  • ||||||||||  N6 - Neuroscience in the Clinic: Myasthenia Gravis: From Pathogenesis to Targeted Therapies (Both in-person and online) -  Jan 7, 2023 - Abstract #AAN2023AAN_653;    
    In particular, participants should be able to recognize new therapeutic targets in autoimmune encephalitis and myasthenia gravis, and develop strategies to manage immune-related adverse events of immune checkpoint inhibitors in oncologic patients. Participants should gain an understanding of
  • ||||||||||  Journal, Stroma:  GTF2I mutation in micronodular thymoma with lymphoid stroma. (Pubmed Central) -  Jan 6, 2023   
    A CBA for detecting LRP4-Ab associated with MG has been developed, and was used to find LRP4-Ab in the sera of patients with MG. GTF2I mutation status and presence of spindle cell morphology may indicate that type A and AB thymoma, and micronodular thymoma represent a group biologically distinct from type B thymomas, which generally lack this mutation.
  • ||||||||||  Journal:  Stings on wings: Proteotranscriptomic and biochemical profiling of the lesser banded hornet (Vespa affinis) venom. (Pubmed Central) -  Jan 6, 2023   
    Their ability to exert potent effects on diverse molecular targets makes them a treasure trove for discovering life-saving therapeutics. Fascinatingly, wasp venoms, being evolutionarily ancient, exhibit a greater degree of compositional and sequence conservation across very distant populations/species, which contrasts with the patterns of venom evolution observed in evolutionarily younger lineages, such as advanced snakes and cone snails.
  • ||||||||||  Journal:  ASO Visual Abstract: Human Parvovirus B19 May Be a Risk Factor in Myasthenia Gravis with Thymoma. (Pubmed Central) -  Jan 5, 2023   
    Fascinatingly, wasp venoms, being evolutionarily ancient, exhibit a greater degree of compositional and sequence conservation across very distant populations/species, which contrasts with the patterns of venom evolution observed in evolutionarily younger lineages, such as advanced snakes and cone snails. No abstract available
  • ||||||||||  Keytruda (pembrolizumab) / Merck (MSD)
    Journal, Adverse events, Checkpoint inhibition:  Myasthenia gravis, myositis and myocarditis: a fatal triad of immune-related adverse effect of immune checkpoint inhibitor treatment. (Pubmed Central) -  Jan 4, 2023   
    Pembrolizumab, a humanised monoclonal antibody and immune checkpoint inhibitor (ICI) that blocks programmed death receptor 1 and its ligands, is an effective immunotherapy for malignancies such as melanoma, lung, head and neck, cancers, and Hodgkin's lymphoma...She was diagnosed with myasthenia gravis, myocarditis and myositis as ICI-induced immune-related adverse effects resistant to medical intervention. We wish to raise awareness of the triad of life-threatening complication of ICI therapy that accounts for 30%-50% of fatal complications.
  • ||||||||||  GRAVE CONSEQUENCES OF IMMUNE CHECKPOINT INHIBITOR TOXICITY BEYOND MYOCARDITIS (Poster Hall_Hall F) -  Jan 4, 2023 - Abstract #ACCWCC2023ACC_WCC_1690;    
    Patients with ICI toxicity manifesting as myocarditis should be evaluated for additional irAEs that may contribute to cardiopulmonary symptoms. It is important to highlight this rare complication as MG is not managed with immunosuppressive agents traditionally used in management of the majority of irAEs.
  • ||||||||||  Opdivo (nivolumab) / Ono Pharma, BMS
    IMMUNE CHECKPOINT INHIBITOR-ASSOCIATED FULMINANT MYOCARDITIS (Poster Hall_Hall F) -  Jan 4, 2023 - Abstract #ACCWCC2023ACC_WCC_1677;    
    Discontinuation of the drug and treatment with high-dose steroids is the standard of care. For refractory cases, adding another immunosuppressive agent or plasmapheresis can be considered.
  • ||||||||||  MYASTHENIC CRISIS-ASSOCIATED TAKOTSUBO CARDIOMYOPATHY: AN UNFAVORABLE COMBINATION (Poster Hall_Hall F) -  Jan 4, 2023 - Abstract #ACCWCC2023ACC_WCC_1552;    
    For refractory cases, adding another immunosuppressive agent or plasmapheresis can be considered. MC-associated TTC results in an unfortunate synergistic effect that significantly increases mortality compared to MC or TTC alone.
  • ||||||||||  Baizean (tislelizumab) / BeiGene, Novartis, Sulanda (surufatinib) / Hutchmed
    ACUTE IMMUNE MYOCARDITIS AS AN IMMUNE RELATED ADVERSE EVENT CAUSED BY SURUFATINIB PLUS TISLELIZUMAB (Poster Hall_Hall F) -  Jan 4, 2023 - Abstract #ACCWCC2023ACC_WCC_987;    
    There may be an association of increased risk with other autoimmune conditions, though this remains an area of study. MRI can be a useful diagnostic tool, with early discontinuation of ICI, initiation of corticosteroids, and heart failure guideline-directed medical therapy as the preferred treatment.
  • ||||||||||  DIAGNOSIS AND MANAGEMENT OF MULTIPLE DIRECT ARTERIAL PLACED PERMANENT PACEMAKER LEADS: USE OF MULTI-MODALITY IMAGING (Poster Hall_Hall F) -  Jan 4, 2023 - Abstract #ACCWCC2023ACC_WCC_415;    
    MRI can be a useful diagnostic tool, with early discontinuation of ICI, initiation of corticosteroids, and heart failure guideline-directed medical therapy as the preferred treatment. Direct arterial CIED multi-lead placement is a rare complication necessitating individualized management and can be avoided by establishing peri-operative protocols consisting of multiple redundant verifications.
  • ||||||||||  pyridostigmine Bromide / Generic mfg.
    Journal:  Analysis of influencing factors of postoperative myasthenic crisis in 564 patients with myasthenia gravis in a single center. (Pubmed Central) -  Jan 4, 2023   
    Direct arterial CIED multi-lead placement is a rare complication necessitating individualized management and can be avoided by establishing peri-operative protocols consisting of multiple redundant verifications. Patients with poor Osserman stages, higher preoperative dosage of pyridostigmine bromide, higher ASA score, poor pulmonary function (low DLCO%), and more intraoperative bleeding should be highly vigilant for the occurrence of postoperative myasthenic crisis.
  • ||||||||||  prednisolone / Generic mfg.
    Journal, IO biomarker:  Minimal symptom expression achievement over time in generalized myasthenia gravis. (Pubmed Central) -  Jan 3, 2023   
    This paper outlines the eye signs in MG, including practical tips on the examination technique relevant to MG, and pitfalls to avoid. From the early phases of immunotherapy, MSE is a good marker of therapeutic goal in patients with generalized MG.
  • ||||||||||  Review, Journal:  Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome: New Developments in Diagnosis and Treatment. (Pubmed Central) -  Dec 30, 2022   
    While LEMS is comparatively rare, the unique clinical presentation, the association with cancer, and evolving treatment strategies require the neurologist to be familiar with its presentation, diagnosis, and management. In this paper we provide a summary of the meaningful recent clinical developments in the diagnosis and treatment of both MG and LEMS.
  • ||||||||||  Journal:  Thymic carcinoma with myasthenia gravis:A case report. (Pubmed Central) -  Dec 30, 2022   
    A possible hypothesis is that the rarity of the disease decreased the awareness regarding the primary prevention or even a diagnosis by an untrained physician. No abstract available
  • ||||||||||  Journal, IO biomarker:  Pneumonia in myasthenia gravis: Microbial etiology and clinical management. (Pubmed Central) -  Dec 27, 2022   
    Moreover, peripheral lymphocyte percentage [odds ratio (OR) 0.88, 95% CI 0.75-0.96, p = 0.02] and serum globulin (OR 1.16, 95% CI 1.02-1.35, p = 0.03) were significantly associated with the risk of MV demand. Our identification of the microbial etiology of pneumonia in MG patients may provide future perspectives on accurate antibiotic options and enable early interventions when risk factors are present.
  • ||||||||||  Review, Journal:  Cholesterol Management in Neurology: Time for Revised Strategies? (Pubmed Central) -  Dec 24, 2022   
    Neurologists find it sometimes difficult to apply these guidelines, having to confront situations such as (1) ischemic strokes, mainly cardioembolic ones, in patients with already low LDL-cholesterol levels; (2) myasthenic patients, whose lifespan has been extended by available treatment, and whose age and cholesterol levels put them at risk for ischemic stroke; (3) patients with myotonic dystrophy, whose disease often associates diabetes mellitus and heart conduction defects, and in whom blood cholesterol management is also not settled. As such, further trials are needed to address these issues.
  • ||||||||||  Retrospective data, Journal:  Rapid Targeted Sequencing Using Dried Blood Spot Samples for Patients With Suspected Actionable Genetic Diseases. (Pubmed Central) -  Dec 23, 2022   
    This pilot study evaluated the possibility of rapid and timely diagnosis of treatable rare genetic diseases using a panel designed by a multidisciplinary team. The automated analytic pipeline maximized the clinical utility of rapid targeted sequencing for medically actionable genes, providing a strategy for appropriate and timely treatment of rare genetic diseases.
  • ||||||||||  azathioprine / Generic mfg.
    Journal:  Severe azathioprine-induced liver injury 22 months after initiation of treatment. (Pubmed Central) -  Dec 22, 2022   
    After discontinuation of azathioprine, biochemical parameters progressively normalised and remarkable clinical improvement was achieved. With this report, we suggest that azathioprine should be considered among the causes of liver injury, despite long treatment duration.
  • ||||||||||  Journal, HEOR:  Impact of Thymectomy on Crisis Incidence and Quality of Life amongst Generalised Myasthenia Gravis Patients. (Pubmed Central) -  Dec 21, 2022   
    No statistically significant differences were observed in quality of life scores between thymectomy and nonthymectomy groups, either before (P = 0.86) or after surgery (P = 0.939). The odds of myasthenia crisis was lesser in people, who underwent thymectomy even after controlling for MGFA grade and other potential confounders but no significant differences in quality of life were found with thymectomy.
  • ||||||||||  Firdapse (amifampridine) / BioMarin, Catalyst Pharma
    Journal:  Lambert-Eaton Myasthenic Syndrome and Botulism. (Pubmed Central) -  Dec 21, 2022   
    A targeted clinical history and a thorough neurologic examination with support from serologic and electrodiagnostic studies are key to early diagnosis of LEMS and botulism. Early diagnosis of both conditions creates opportunities for therapy and improves outcomes.
  • ||||||||||  Journal:  Diagnosis and Management of Myasthenia Gravis. (Pubmed Central) -  Dec 21, 2022   
    Carefully selected treatment improves outcomes in MG. Additional treatment options for MG will likely be available in the near future.
  • ||||||||||  Journal:  Incidence and causes of overdiagnosis of myasthenia gravis. (Pubmed Central) -  Dec 20, 2022   
    Overdiagnosis of MG is not uncommon, and occurs more frequently in seronegative patients. To make an accurate diagnosis of MG, there is a need to recognize atypical presentations, and avoid overreliance on minor or non-specific serological and electrodiagnostic findings.
  • ||||||||||  Journal:  Proteolytic ectodomain shedding of muscle-specific tyrosine kinase in myasthenia gravis. (Pubmed Central) -  Dec 18, 2022   
    The results may explain why MuSK-MG is refractory to treatments and causes rapid muscle atrophy in some patients due to the denervation associated with Ab-induced disruption of neuromuscular transmission at the NMJ. Such discoveries pave the way for new MG treatments, and MuSK may be used as a biomarker for other neuromuscular diseases in preclinical studies, clinical diagnostics, therapeutics, and drug discovery.
  • ||||||||||  Journal:  Anxiety and depression in thymoma patients in China before surgery. (Pubmed Central) -  Dec 18, 2022   
    Such discoveries pave the way for new MG treatments, and MuSK may be used as a biomarker for other neuromuscular diseases in preclinical studies, clinical diagnostics, therapeutics, and drug discovery. Patients with myasthenia gravis and resigned coping style were found to have higher anxiety and depression before surgery for Chinese thymoma patients.
  • ||||||||||  Review, Journal:  Incidence of Facial Nerve Palsy in Pregnancy. (Pubmed Central) -  Dec 17, 2022   
    Women are also susceptible to paralysis after childbirth. Most of the women who develop Bell's palsy after pregnancy start showing signs and symptoms seven to 10 days after the date of delivery.