Myasthenia Gravis
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  • ||||||||||  Retrospective data, Journal:  Weaning and extubation failure in myasthenic crisis: a multicenter analysis. (Pubmed Central) -  Jan 8, 2024   
    Older age, multiple comorbidities and development of cardiac and pulmonary complications are associated with a higher risk of WF and EF. Our data suggest that WF occurs less frequently under first-line plasma exchange/immunoadsorption compared with first-line use of IVIG.
  • ||||||||||  Journal:  Clinical and genetic characterisation of a large Indian congenital myasthenic syndrome cohort. (Pubmed Central) -  Jan 8, 2024   
    Myopathy and muscular dystrophy genes like GMPPB and DES presenting as gradually progressive limb girdle congenital myasthenic syndromes expand the phenotypic spectrum. The novel genes MACF1 and TEFM identified in this cohort add to the expanding list of genes with new mechanisms causing neuromuscular junction defects.
  • ||||||||||  Uplizna (inebilizumab-cdon) / Mitsubishi Tanabe, Amgen
    Enrollment closed, Trial completion date, Trial primary completion date:  MINT: Myasthenia Gravis Inebilizumab Trial (clinicaltrials.gov) -  Jan 8, 2024   
    P3,  N=238, Active, not recruiting, 
    Our data suggest that efgartigimod may be considered as a candidate drug for SPS and other autoantibody-mediated neurological disorders. Recruiting --> Active, not recruiting | Trial completion date: Mar 2029 --> Nov 2027 | Trial primary completion date: Oct 2024 --> May 2024
  • ||||||||||  Retrospective data, Review, Journal:  Effects of acupuncture treatment for myasthenia gravis: A systematic review and meta-analysis. (Pubmed Central) -  Jan 7, 2024   
    However, the quality of included studies was generally low and caution should be exercised when considering this treatment option. In the future, more rigorous study designs and high-quality RCTs are needed to verify the efficacy of acupuncture in the treatment of MG, because the results of high-quality RCTs are more reliable and accurate.
  • ||||||||||  Journal:  Hospitalizations and Mortality From Myasthenia Gravis. (Pubmed Central) -  Jan 6, 2024   
    While readmission and mortality rates have remained stable, the increasing hospitalization rates indicate that the raw numbers of readmissions-and deaths-are also increasing. Mortality rates are considerably higher in older patients hospitalized with MG.
  • ||||||||||  Journal, Real-world evidence, Real-world:  Potentially inappropriate drug use in myasthenia gravis: a real-world population-based cohort study in Italy. (Pubmed Central) -  Jan 5, 2024   
    Patients aged 75+ years, those with at least one specific complication or comorbidity, and those exposed to polytherapy were more likely to be treated with a potential contraindicated drug. Among incident users of pyridostigmine, more than 15% of patients have at least one of the contraindications considered, and among patients diagnosed with MG, in the first year of follow-up >60% of subjects were treated with potentially contraindicated drugs.
  • ||||||||||  DIAGNOSIS OF CARDIAC TAMPONADE PHYSIOLOGY UNABLE TO BE CHARACTERIZED ON CT IMAGING () -  Jan 4, 2024 - Abstract #SCCM2024SCCM_2168;    
    Bedside echo is a quick, non-invasive study that can reliably detect pericardial effusions and assess hemodynamic compromise in patients with suspected tamponade physiology. In cases where the amount of pericardial fluid is small, echo may be the only diagnostic tool that can accurately detect tamponade.
  • ||||||||||  Keytruda (pembrolizumab) / Merck (MSD)
    PEMBROLIZUMAB-INDUCED TRIPLE-M (MYOCARDITIS-MYOSITIS-MYASTHENIA GRAVIS) SYNDROME () -  Jan 4, 2024 - Abstract #SCCM2024SCCM_1683;    
    Given suspicion for acute coronary syndrome, he received aspirin and heparin infusion and admitted to the ICU...Heparin infusion was discontinued and 1g methylprednisolone and 10?mg/kg abatacept x 3 days were given for myocarditis, and immune globulin x 5 days for the concurrent MG...The exact pathogenesis of myocarditis and conduction abnormalities in this setting is poorly understood. Rapid diagnosis, close monitoring, multidisciplinary collaboration, and treatment with steroids is key to optimize patient outcomes.
  • ||||||||||  lorazepam / Generic mfg.
    ACUTE DESCENDING PARALYSIS: DECISIONS THAT COME BACK TO BITE YOU () -  Jan 4, 2024 - Abstract #SCCM2024SCCM_1347;    
    Initially given IV lorazepam, IV fluids, thiamine, and dextrose but remained combative...In addition to mechanical ventilation and airway protection, specific cobra neurotoxin anti-venom is required when dense paralysis and respiratory collapse has occurred. Poison Control centers in USA can assist in acquisition of monovalent anti-venoms from particularly Zoos or exotic animal facilities.
  • ||||||||||  THE ONLY WAY BACK DOWN IS BY GOING UP: AN ATYPICAL CASE OF ASCENDING PARALYSIS () -  Jan 4, 2024 - Abstract #SCCM2024SCCM_1237;    
    Acute myopathy can be life threatening due to the underlying etiology and/or respiratory failure if not recognized. Axonal GBS requiring intubation, while rare, is an example of a life-threatening myopathy carrying the potential for rehabilitation and recovery, further emphasizing the importance of a thorough workup and an expeditious diagnosis.
  • ||||||||||  Bavencio (avelumab) / EMD Serono
    A CASE OF AVELUMAB-ASSOCIATED MYASTHENIA GRAVIS, MYOSITIS, AND MULTIORGAN FAILURE () -  Jan 4, 2024 - Abstract #SCCM2024SCCM_1236;    
    Avelumab-associated myasthenia gravis, myositis, and multi-organ failure is a rare but life-threatening immune-mediated adverse event. Clinicians should suspect this diagnosis in patients presenting with dysphagia and bulbar symptoms, and initiate timely treatment to prevent fatal deterioration.
  • ||||||||||  dexamethasone / Generic mfg.
    ASYMPTOMATIC BRADYCARDIA INDUCED BY INTRAVENOUS IMMUNOGLOBULIN IN A PATIENT WITH MYASTHENIA GRAVIS () -  Jan 4, 2024 - Abstract #SCCM2024SCCM_928;    
    She was then started on dexamethasone and, one day later, IVIG...On literature review, there have been only two reported cases of IVIG induced bradycardia in patients without a cardiac history. This case report, along with the two previously indicated cases, represents a growing body of evidence that IVIG induced arrhythmias, particularly bradycardia, may be more common than previously thought in patients without a cardiac history.
  • ||||||||||  Journal:  Case Report: Unilateral Hemispheric Hemorrhage with Bilateral Ptosis and Upgaze Palsy. (Pubmed Central) -  Jan 3, 2024   
    https://clinicaltrials.gov/study/NCT03979521, Registered 7 June 2019 (retrospectively registered). Acute bilateral ptosis and upgaze palsy suggest the possibility of unilateral hemispheric hemorrhage, even though there is no direct involvement of the brainstem and its nuclei.
  • ||||||||||  Journal:  Safety update: statins and myasthenia gravis. (Pubmed Central) -  Jan 2, 2024   
    Acute bilateral ptosis and upgaze palsy suggest the possibility of unilateral hemispheric hemorrhage, even though there is no direct involvement of the brainstem and its nuclei. No abstract available
  • ||||||||||  Observational data, Retrospective data, Journal:  Prenatal diagnosis of isolated bilateral clubfoot: Is amniocentesis indicated? (Pubmed Central) -  Jan 2, 2024   
    Despite the low rate of pathogenic chromosomal abnormalities diagnosed prenatally after this ultrasound diagnosis, the risk of chromosomal aberration exceeds the risks of amniocentesis. These data may be helpful in prenatal counseling situations.
  • ||||||||||  Review, Journal, IO biomarker:  Guideline for the management of myasthenic syndromes. (Pubmed Central) -  Dec 28, 2023   
    Glucocorticosteroids and the classical immunosuppressants (primarily azathioprine) are the basic immunotherapeutics to treat mild/moderate to (highly) active generalized MG/young MG and ocular MG...In (highly) active generalized MG, complement inhibitors (currently eculizumab and ravulizumab) or neonatal Fc receptor modulators (currently efgartigimod) are recommended for AChR-Ab-positive status and rituximab for muscle-specific receptor tyrosine kinase (MuSK)-Ab-positive status...Specific aspects of ocular, juvenile, and congenital myasthenia are highlighted. The guideline will be further developed based on new study results for other immunomodulators and biomarkers that aid the accurate measurement of disease activity.
  • ||||||||||  Journal:  Correlation between Cardiovascular Autonomic and Pulmonary Ventilation Functions in Myasthenia Gravis Patients. (Pubmed Central) -  Dec 26, 2023   
    Our study demonstrated a significant relationship between a reduced pulmonary ventilation function and respiratory mechanics with cardiovascular autonomic parameters, including the E/I ratio, BRS, and HRV measures at rest, as shown in the MG group. Future studies should focus on the interplay between respiratory and autonomic function testing, as well as pulmonary rehabilitation, to mitigate cardiovascular risk in these patients.
  • ||||||||||  Kesimpta (ofatumumab subcutaneous) / Novartis, Genmab
    Journal:  Therapeutic effect of ofatumumab in patients with myasthenia gravis: immunoregulation of follicular T helper cells and T helper type 17 cells. (Pubmed Central) -  Dec 26, 2023   
    At weeks 0, 1, 2, and 4, an initial dose of 20 mg of ofatumumab was injected subcutaneously, with a 2-month follow-up after completing this first cycle...At 3 months, doses of prednisone were reduced by an average of 37%...The immunomodulatory effect of ofatumumab during the observation period may involve depletion of B cells, reduction of Tfh and Th17 cells frequencies, and reduced levels of IL-6, IL-21, and IL-17. The findings provide novel data for the potential application of ofatumumab in MG.
  • ||||||||||  hydroxychloroquine / Generic mfg., tacrolimus topical / Generic mfg.
    Journal:  A case report of dermatomyositis mimicking myasthenia gravis. (Pubmed Central) -  Dec 24, 2023   
    Further tumor screening should be performed for patients with dermatomyositis. Early detection and management of possible tumors are essential to the treatment of dermatomyositis linked to malignancies.
  • ||||||||||  Journal:  Special Issue: Immune-Mediated Neurological Disorders. (Pubmed Central) -  Dec 23, 2023   
    Early detection and management of possible tumors are essential to the treatment of dermatomyositis linked to malignancies. For a long time, the immune system has been considered responsible for only a minority of neurological conditions involving the central and peripheral nervous system (CNS, PNS), respectively, namely multiple sclerosis and myasthenia gravis (with myastheniform syndromes) [...].
  • ||||||||||  Review, Journal:  Refocusing generalized myasthenia gravis: Patient burden, disease profiles, and the role of evolving therapy. (Pubmed Central) -  Dec 20, 2023   
    gMG has a physical, mental, and social impact, resulting in a considerable burden of disease and substantially decreased QoL, despite standard treatments. The availability of novel, targeted treatments that influence key pathological mediators of gMG, together with new biomarkers, offers the potential to optimize patient management and ultimately enables a greater number of patients to achieve minimal manifestation status and a reduced burden of disease.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Holdings, Roche
    Journal:  Long-term efficacy and safety of low-dose rituximab in patients with refractory myasthenia gravis. (Pubmed Central) -  Dec 20, 2023   
    The availability of novel, targeted treatments that influence key pathological mediators of gMG, together with new biomarkers, offers the potential to optimize patient management and ultimately enables a greater number of patients to achieve minimal manifestation status and a reduced burden of disease. Long-term CD20+ B-cell-guided low-dose rituximab showed good efficacy and tolerance in patients with refractory myasthenia gravis.
  • ||||||||||  batoclimab (IMVT-1401) / Roivant
    Phase classification:  A Study of RVT-1401 in Myasthenia Gravis (MG) Patients (clinicaltrials.gov) -  Dec 20, 2023   
    P2,  N=17, Completed, 
    Long-term CD20+ B-cell-guided low-dose rituximab showed good efficacy and tolerance in patients with refractory myasthenia gravis. Phase classification: P2a --> P2
  • ||||||||||  Soliris (eculizumab) / AstraZeneca
    Journal, Checkpoint inhibition:  Eculizumab for myasthenic exacerbation during treatment with immune-checkpoint inhibitors. (Pubmed Central) -  Dec 18, 2023   
    Eculizumab potentially offers a mechanism-based treatment of MG in patients under anti-programmed cell death protein 1 (PD-1) agents, without interfering with their mechanism of action and avoiding their discontinuation. Larger case series deserve to be evaluated.
  • ||||||||||  Review:  EYE MYASTHENIA IN A YOUNG TOGOLESE: ABOUT A CASE AND LITERATURE REVIEW. (Pubmed Central) -  Dec 18, 2023   
    Finally, electromyography revealed a decrement greater than 10% to repetitive nerve stimulation. The interest of this issue lies in the rarity of this observation in Black Africa, where it is often confused with malaria.
  • ||||||||||  Review, Journal:  Identification and Management of Acute Neuromuscular Respiratory Failure in the ICU. (Pubmed Central) -  Dec 17, 2023   
    Prompt recognition and accurate diagnosis of new or worsening chronic neuromuscular disease have important clinical management and prognostic implications. In this article, we present an approach to the acute presentation of undifferentiated neuromuscular respiratory failure in the ICU and guidance for determination and respiratory management of the underlying disorder.