Myasthenia Gravis
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  • ||||||||||  Clinical, Journal:  Targeted Treatments for Inherited Neuromuscular Diseases of Childhood. (Pubmed Central) -  Jul 7, 2021   
    This review highlights these advances and emerging targeted therapies for Duchenne muscular dystrophy and centronuclear myopathy, while also covering enzyme replacement therapy and small molecule-based targeted therapies for conditions such as Pompe's disease and congenital myasthenic syndromes. With these and other newer techniques for targeted correction of genetic defects, such as CRISPR/Cas9, there is now hope that treatments for many more genetic diseases of the nervous system will follow in the near future.
  • ||||||||||  methylprednisolone sodium succinate / Generic mfg.
    Clinical, Journal, IO biomarker:  Clinical Profile and Treatment Response in Patients with CASPR2 Antibody-Associated Neurological Disease. (Pubmed Central) -  Jul 6, 2021   
    Patients were treated with intravenous methylprednisolone and plasma exchange during the acute symptomatic phase followed by pulsed intravenous methyl prednisolone to maintain remission...CASPR2 antibody-associated disease has favorable response to immunotherapy with complete improvement and good outcome. Underlying malignancy may be a marker for poor prognosis.
  • ||||||||||  dexamethasone / Generic mfg.
    Journal:  Biomodulatory Treatment Regimen, MEPED, Rescues Relapsed and Refractory Classic Hodgkin's Disease. (Pubmed Central) -  Jul 6, 2021   
    MEPED is well tolerated with low toxicity and highly efficacious in relapsed/refractory cHL, including severely comorbid patients. Due to its immunomodulatory components, MEPED might also have a synergistic potential when combined with ICPi but requires further evaluation within a clinical trial.
  • ||||||||||  Trial completion date, Trial primary completion date:  3,4-Diaminopyridine Use in Lambert-Eaton Myasthenic Syndrome(LEMS) and Congenital Myasthenic Syndromes (CMS) (clinicaltrials.gov) -  Jul 6, 2021   
    P=N/A,  N=22, Active, not recruiting, 
    Due to its immunomodulatory components, MEPED might also have a synergistic potential when combined with ICPi but requires further evaluation within a clinical trial. Trial completion date: Jun 2021 --> Dec 2021 | Trial primary completion date: Jun 2021 --> Dec 2021
  • ||||||||||  [VIRTUAL] Porocarcinoma: a case series of 41 patients diagnosed over a 12-year period () -  Jul 5, 2021 - Abstract #BAD2021BAD_220;    
    Locoregional metastasis occurred in 12%, with no distant organ involvement seen. There is some evidence that MMS is the best option to reduce local recurrence rates, and although our data support this, three times fewer patients were managed with MMS than with other surgery.
  • ||||||||||  Journal:  Effects of acute neurologic disease on the heart. (Pubmed Central) -  Jul 2, 2021   
    Although typically reversible, these cardiac complications are important to recognize as they are associated with increased morbidity and mortality. In this chapter, we discuss the suspected pathophysiology, clinical presentation, and management of the cardiac dysfunction that occur as a consequence of different types of acute neurologic illness.
  • ||||||||||  azathioprine / Generic mfg.
    Review, Journal:  History of Myasthenia Gravis Revisited. (Pubmed Central) -  Jul 1, 2021   
    Treatment modalities which completely changed the prognosis of MG, including positive pressure mechanic ventilation and corticosteroids as well as plasma exchange/IVIg and azathioprine, were put to use...Valuable guidelines were published. In the last part of the review, the experience in MG of Istanbul University, a pioneer in Turkey, is related.
  • ||||||||||  Journal:  Neuromuscular disorders in pregnancy. (Pubmed Central) -  Jul 1, 2021   
    Many commonly used therapies have fetal animal but no proven human toxicity concerns, complicating treatment and risk decisions. Weaning off effective therapeutic agents or preemptive aggressive treatment or surgery prior to planned pregnancy is an option in some conditions.
  • ||||||||||  Clinical, Observational data, Journal:  Diagnostic yields and clinical features of ocular myasthenia gravis. (Pubmed Central) -  Jul 1, 2021   
    Unequivocal response to pyridostigmine was observed in 31 (86.11%) patients. For seropositive cases, AchR Ab titer was significantly higher in the group with 2 clinical symptoms than that in the 1 clinical symptom (P = .011).This study presents the usefulness and diagnostic validity of antimyasthenic treatment for OMG, especially seronegative OMG, with detailed symptom analysis.
  • ||||||||||  Clinical, Journal:  Digestive involvement in a patient with Lambert-Eaton syndrome. (Pubmed Central) -  Jun 30, 2021   
    Neuromuscular junction disorders are not characterized by gastrointestinal involvement, as in our case. They have previously sporadically been related to intestinal motility dysfunction.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    Clinical, Journal:  Efficacy and safety of Rituximab in myasthenia gravis: A French multicentre real-life study. (Pubmed Central) -  Jun 30, 2021   
    Our study demonstrates the efficacy and safety of rituximab in MG patients. Additional studies are still necessary to set the actual place of rituximab in pharmacopeia of MG treatment and to establish precise recommendations for infusion protocol.
  • ||||||||||  Retrospective data, Review, Journal:  Effects of thymectomy on late-onset non-thymomatous myasthenia gravis: systematic review and meta-analysis. (Pubmed Central) -  Jun 30, 2021   
    Thymectomy in late-onset NTMG also yielded no benefit to CSR or PR compared with conservative treatments. In late-onset NTMG patients, thymectomy should therefore be performed with caution, and the appropriate cutoff between early- and late-onset MG should be further explored in order to tailor and execute the proper therapeutic strategies.
  • ||||||||||  Review, Journal:  Fc-Receptor Targeted Therapies for the Treatment of Myasthenia gravis. (Pubmed Central) -  Jun 30, 2021   
    Furthermore, we evaluate prospects of therapies targeting classical Fc receptors that have shown promising therapeutic efficacy in other antibody-mediated conditions. Increased availability of Fc- and Fc receptor-targeting biologics might foster the development of personalized immunotherapies with the potential to induce sustained disease remission in patients with MG.
  • ||||||||||  ketamine / Generic mfg.
    Clinical, Review, Journal:  Neurocritical Care of the Pregnant Patient. (Pubmed Central) -  Jun 29, 2021   
    Early studies on hypothermia, ketamine, and immunomodulating therapies in pregnancy are promising...When difficult decisions regarding preterm delivery arise, recent outcome studies can help inform discussion. Lastly, when the feared complication of maternal death by neurologic criteria occurs, fetal somatic support may help to save at least one life.
  • ||||||||||  Uplizna (inebilizumab) / Horizon Therapeutics
    Review, Journal:  Inebilizumab: First Approval. (Pubmed Central) -  Jun 26, 2021   
    The drug is also undergoing clinical evaluation for kidney transplant desensitization, myasthenia gravis, and IgG4-related disease. This article summarizes the milestones in the development of inebilizumab leading to this first approval for the treatment of AQP4-IgG seropositive NMOSD.
  • ||||||||||  Clinical, Journal:  Epidemiology of Myasthenia Gravis in Sweden 2006-2016. (Pubmed Central) -  Jun 26, 2021   
    This article summarizes the milestones in the development of inebilizumab leading to this first approval for the treatment of AQP4-IgG seropositive NMOSD. The incidence and prevalence of Myasthenia Gravis have increased over time in Sweden, and the rates are high in comparison with other countries.
  • ||||||||||  Clinical, Journal:  Myasthenia gravis posterior to thymectomy: Case report. (Pubmed Central) -  Jun 26, 2021   
    The late onset of MG and other autoimmune disorders should be considered as possible complications of the surgical treatment of thymomas. Therefore, after the resection of a thymoma, follow-up care with post-operative MG consideration is necessary.
  • ||||||||||  methylprednisolone sodium succinate / Generic mfg.
    Journal, Checkpoint inhibition:  Immune Checkpoint Inhibitor-Mediated Myasthenia Gravis. (Pubmed Central) -  Jun 24, 2021   
    He was immediately commenced on 1 mg/kg of intravenous methylprednisolone and pyridostigmine 60 mg 3 times a day...WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Immune-mediated myasthenia gravis is an important toxicity of ICIs. Early recognition and treatment of this presentation may reduce the significant morbidity and mortality associated with it.
  • ||||||||||  Review, Journal:  Classification of myasthenia gravis and congenital myasthenic syndromes in dogs and cats. (Pubmed Central) -  Jun 24, 2021   
    Congenital myasthenic syndromes are classified according to the affected NMJ component, the mechanism of the defect of neuromuscular transmission, the affected protein, and ultimately the mutated gene responsible. In proposing this categorization of MG and CMSs, we hope to aid recognition of the disease groups for both conditions, as well as guide treatment, refine prognosis, and provide a framework for additional studies of these conditions.
  • ||||||||||  Journal:  Imbalance of Th17 and Tregs in thymoma may be a pathological mechanism of myasthenia gravis. (Pubmed Central) -  Jun 23, 2021   
    Overall, Th17 cells and related transcription factors and cytokines in thymoma with MG patients were higher than those in thymoma patients, whereas, Tregs showed the opposite results, the mechanism may be that thymoma can secrete IL6 and IL21. These findings indicated that imbalances in Th17/Tregs may account for the pathogeny of thymoma with MG.
  • ||||||||||  Clinical, Journal:  Acute Neuromuscular Disorders in the Pediatric Intensive Care Unit. (Pubmed Central) -  Jun 22, 2021   
    Although acute and acute-on-chronic neuromuscular disorders are encountered relatively rarely in the PICU, familiarity with the presenting features of these disorders is important in facilitating timely diagnosis. This, in turn, enables the institution of effective management strategies, thereby avoiding complications associated with diagnostic delays.