Myasthenia Gravis
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  • ||||||||||  Soliris (eculizumab) / Alexion Pharma
    Journal:  Myasthenia gravis (Pubmed Central) -  Oct 11, 2019   
    This article reviews the basic and advanced treatment options of the different disease subtypes including plasma exchange and immunoglobulins for treatment in a myasthenic crisis. Recently, clinical approval of eculizumab, a complement inhibitor, enriched the pharmacological armamentarium for AChR antibody-positive MG patients not appropriately responding to immunosuppression alone.
  • ||||||||||  Journal:  Collagen XIII is required for neuromuscular synapse regeneration and functional recovery after peripheral nerve injury. (Pubmed Central) -  Oct 9, 2019   
    This study also describes a relevant contribution of the altered NMJ morphology and function to neuromuscular synapses, and PNS regeneration and functional recovery in collagen XIII-deficient mice after peripheral nerve injury. Correlating the animal model data on collagen XIII-associated CMS, it can be speculated that neuromuscular connections in CMS patients are not able to fully regenerate and restore normal functionality if exposed to peripheral nerve injury.
  • ||||||||||  Clinical, Journal:  Severe unintentional first trimester carbon monoxide poisoning: case report. (Pubmed Central) -  Oct 8, 2019   
    Fetal abnormalities may occur after CO poisoning in pregnancy and vary based on the gestational age of the fetus at the time of the exposure as well the chronicity of the exposure. Fetal survival after maternal CO exposure is possible even with significantly elevated maternal COHb concentrations, although teratogenic effects may occur depending on the timing of exposure.
  • ||||||||||  Clinical, Journal, PD(L)-1 Biomarker, IO Biomarker:  Programmed cell death ligand 1 expression is upregulated in the skeletal muscle of patients with myasthenia gravis. (Pubmed Central) -  Oct 8, 2019   
    Moreover, a unimodal relationship between PD-L1 mRNA levels and quantitative MG scores for disease severity was identified by a generalized additive model, indicating reciprocal effects. Together, these results suggest that MG symptoms might influence PD-L1 expression in muscle cells, and upregulated PD-L1 expression might inhibit autoimmune reactivity.
  • ||||||||||  Journal:  Circulating microRNA plasma profile in MuSK+ myasthenia gravis. (Pubmed Central) -  Oct 8, 2019   
    We found a distinct plasma profile of miR-210-3p and miR-324-3p that were significantly decreased in MuSK+ MG patients compared to healthy controls (4.1 ± 1.4 vs 5.1 ± 1.4, p = .006 and 4.7 ± 1.0 vs 5.4 ± 1.3, p = .02). These findings reveal a distinct plasma miRNA profile in MuSK+ MG.
  • ||||||||||  Soliris (eculizumab) / Alexion Pharma, Rituxan (rituximab) / Roche, Biogen
    Journal:  Therapies Directed Against B-Cells and Downstream Effectors in Generalized Autoimmune Myasthenia Gravis: Current Status. (Pubmed Central) -  Oct 8, 2019   
    The rarity of myasthenia gravis, heterogeneity in its clinical manifestations, and variability in immunosuppressive regimens are challenges to conducting successful trials. Nonetheless, these are promising times for myasthenia gravis, as renewed research efforts provide novel insights into its immunopathology, allowing for development of targeted therapies with increased efficacy and safety.
  • ||||||||||  Soliris (eculizumab) / Alexion Pharma
    Clinical, Journal:  Ravulizumab: First Global Approval. (Pubmed Central) -  Oct 8, 2019   
    Alexion is also evaluating the coadministration of subcutaneous ravulizumab with Halozyme's ENHANZE drug-delivery technology (rHuPH20), which may have the potential to further extend the dosing interval. This article summarizes the milestones in the development of ravulizumab leading to this first approval for PNH.
  • ||||||||||  fluoxetine / generics
    Review, Journal:  Congenital myasthenic syndromes. (Pubmed Central) -  Oct 8, 2019   
    CMSs are an increasingly recognised group of genetically transmitted defects, which usually respond favorably to drugs enhancing the neuromuscular transmission. CMSs need to be differentiated from neuromuscular disorders due to muscle or nerve dysfunction.
  • ||||||||||  Soliris (eculizumab) / Alexion Pharma
    Journal:  Eculizumab improves fatigue in refractory generalized myasthenia gravis. (Pubmed Central) -  Oct 8, 2019   
    P3
    Compared with placebo, eculizumab was associated with improvements in perceived fatigue that strongly correlated with improvements in MG-specific outcome measures. Trial ID Registration: NCT01997229, NCT02301624.
  • ||||||||||  Rituxan (rituximab) / Roche, Biogen, Cimzia (certolizumab pegol) / Astellas, Dermira, UCB
    Efficacy of Mycophenolate Mofetil in the Treatment of Rheumatoid Arthritis Associated Interstitial Lung Disease (Hall B5) -  Oct 7, 2019 - Abstract #ACRARHP2019ACR_3346;    
    About 55% of patients on combination MMF and RTX showed stability or improvement in FVC. Patients treated with MMF compared to combination MMF and RTX had similar FVC values at the end of the study period, however, the combination therapy had more hospital admissions and infections.
  • ||||||||||  Journal:  Myasthenia gravis. (Pubmed Central) -  Oct 2, 2019   
    These results suggested that the EAMG serum possessed several different pathogenic antibodies that might be sufficient to induce the EAMG phenotype. No abstract available
  • ||||||||||  tacrolimus / Generic Mfg., Rituxan (rituximab) / Roche, Biogen
    Clinical, Review, Journal:  Pediatric Ocular Myasthenia Gravis. (Pubmed Central) -  Sep 29, 2019   
    A recent open-label trial has provided evidence for the use of tacrolimus to improve symptoms of JMG including OMG that were refractory to prednisone...There are other treatments used in the pediatric population of MG, including intravenous immunoglobulin (IVIG), plasmapheresis, and rituximab, but currently there are no reports on use in OMG...In addition to this, thymectomy has been utilized in this population. In both of these instances, no pediatric randomized control trials have been performed to date.
  • ||||||||||  Journal:  Cannabinoid-induced increase of quantal size and enhanced neuromuscular transmission. (Pubmed Central) -  Sep 28, 2019   
    In a mouse model of myasthenia gravis the cannabinoid receptor agonist WIN 55,212 reversed fatiguing failure of neuromuscular transmission, suggesting future therapeutic potential. Our data suggest an endogenous pathway by which cannabinoids might help to regulate transmitter release at the neuromuscular junction.
  • ||||||||||  Journal:  Immunostick ELISA for rapid and easy diagnosis of myasthenia gravis. (Pubmed Central) -  Sep 28, 2019   
    Although this assay is mostly qualitative, compared to the results obtained from the gold-standard radioimmunoprecipitation assays, its specificity for anti-AChR antibodies was 99.1% (102/103 negative sera) and its sensitivity was 91.1% (72/79) or 97.5% (77/79) depending on counting the 5 ambiguous sera as negative or positive, respectively. Smaller scale experiments suggested that the method is efficient for anti-MuSK antibodies, and may also be used with whole blood instead of serum.
  • ||||||||||  Opdivo (nivolumab) / Ono Pharma, BMS, Yervoy (ipilimumab) / Ono Pharma, BMS
    Review, Journal, Checkpoint inhibition:  Proposed diagnostic and treatment paradigm for high-grade neurological complications of immune checkpoint inhibitors. (Pubmed Central) -  Sep 27, 2019   
    Our algorithm incorporates both peripheral nervous system (meningo-radiculitis, GBS, MG) and central nervous system presentations (myelitis, encephalopathy). It is anticipated that our algorithm will be useful both to oncologists and neurologists who are likely to encounter neurological irAEs more frequently in the future as immune checkpoint inhibitors become more widely used.
  • ||||||||||  TAKE MY BREATH AWAY: NEWLY DIAGNOSED MYASTHENIC IN CRISIS; A DIAGNOSTIC DILEMMA (Ernest N. Morial Convention Center - Exhibit Hall - Poster Area 4) -  Sep 25, 2019 - Abstract #CHEST2019CHEST_3555;    
    Usage of different forms of TCs was the cause of KS in a few cases. When respiratory failure is expected, bedside assessments can document weakness and indicate need for invasive ventilation prior to respiratory embarrassment.
  • ||||||||||  Tirosint (levothyroxine) / IBSA Institute Biochemical SA
    SEVERE HYPOTHYROIDISM LEADING TO RESPIRATORY FAILURE AND BULBAR WEAKNESS: A RARE CASE REPORT (Ernest N. Morial Convention Center - Exhibit Hall - Poster Area 5) -  Sep 25, 2019 - Abstract #CHEST2019CHEST_3421;    
    She was given 125 g of intravenous levothyroxine daily for 5 days which was then transitioned to oral 150 mg levothyroxine...To conclude, hypothyroidism should always be considered as a rare cause of respiratory failure with hypoventilation and bulbar weakness. Our case reinforces the importance of early recognition of this condition in order to prevent any patient mortality.
  • ||||||||||  methylprednisolone / generics
    A BROKEN HEART: CARDIAC ARREST AS THE INITIAL PRESENTATION OF MYASTHENIC CRISIS (Ernest N. Morial Convention Center - Exhibit Hall - Poster Area 5) -  Sep 25, 2019 - Abstract #CHEST2019CHEST_3311;    
    Treatment with plasmapheresis and methylprednisolone was initiated resulting in significant improvement of the patient's condition...Myasthenic crisis presenting as a cardiac arrest without other accompanying typical symptoms of MG is rare, and diagnostically challenging to providers. Clinicians should be aware of the possibility of cardiac manifestations in MG and be proactive in their approach.
  • ||||||||||  prednisone / generics
    IT'S ALL ABOUT PULMONARY FUNCTION TESTS (Ernest N. Morial Convention Center - Exhibit Hall - Poster Area 2) -  Sep 25, 2019 - Abstract #CHEST2019CHEST_2838;    
    He was started on pyridostigmine and prednisone with significant improvement in his symptoms and in hypercarbia.Myasthenia gravis results in T-cell mediated attack of the acetylcholine receptors and associated proteins at the neuromuscular junction...Even though the patient had obvious causes for hypercapnia like OSA and OHS, complete PFT's with inspiratory and expiratory pressures were done which lead to the diagnosis of MG. This case highlights the need to consider maximal inspiratory and expiratory pressures for diagnosis of neuromuscular disorders where the presenting complain is hypercarbic respiratory failure.
  • ||||||||||  Frumeron (indapamide) / Remedica
    A UNIQUE CASE OF THE SYNDROME OF INAPPROPRIATE ANTIDIURETIC HORMONE TREATED WITH NONINVASIVE VENTILATION (Ernest N. Morial Convention Center - Exhibit Hall - Poster Area 2) -  Sep 25, 2019 - Abstract #CHEST2019CHEST_2825;    
    During that admission, she was treated with fluid restriction and cessation of indapamide...This patient was discharged home on NIV, with no recurrent presentations of hyponatremia. While describing a unique etiology for SIADH, this case further depicts the sustained benefits of non-invasive ventilation from the acute to chronic setting.