Myasthenia Gravis
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  • ||||||||||  Soliris (eculizumab) / Alexion Pharma
    Clinical, Review, Journal:  Myasthenia gravis: Historical achievements and the "golden age" of clinical trials. (Pubmed Central) -  Sep 16, 2020   
    Some of the therapies (eg, thymectomy, corticosteroids, plasma exchange, and intravenous immunoglobulin [IVIg]) have survived the test of time. Others (eg, eculizumab and neonatal Fc receptor inhibitor) are novel and hold promise.
  • ||||||||||  Uplizna (inebilizumab-cdon) / Mitsubishi Tanabe, Amgen
    Enrollment open:  MINT: Myasthenia Gravis Inebilizumab Trial (clinicaltrials.gov) -  Sep 16, 2020   
    P3,  N=252, Recruiting, 
    Others (eg, eculizumab and neonatal Fc receptor inhibitor) are novel and hold promise. Not yet recruiting --> Recruiting
  • ||||||||||  Clinical, Review, Journal:  Pathomechanisms and Clinical Implications of Myasthenic Syndromes Exacerbated and Induced by Medical Treatments. (Pubmed Central) -  Sep 16, 2020   
    From a clinical perspective, it is of utmost importance for the treating physicians to be aware of such adverse treatment effects and their consequences. In this article, we aim to summarize existing evidence regarding the key molecular and immunological mechanisms as well as the clinical implications of medication-aggravated and medication-induced myasthenic syndromes.
  • ||||||||||  Review, Journal:  Gender issues of antibody-mediated diseases in neurology: (NMOSD/autoimmune encephalitis/MG). (Pubmed Central) -  Sep 16, 2020   
    Herein, we present the characteristics of NMOSD, AE, MG and LEMS in general, and review published data regarding the influence of the different diseases on fertility, pregnancy, puerperium, treatment strategy during pregnancy and post-partum period, and menopause but also male factors. We summarise key elements that should be borne in mind when confronted with such cases.
  • ||||||||||  Clinical, Journal:  Associations of pemphigus or pemphigoid with autoimmune disorders in US adult inpatients. (Pubmed Central) -  Sep 13, 2020   
    Trial completion date: Apr 2022 --> May 2021 | Trial primary completion date: Apr 2022 --> May 2021 Inpatients with pemphigus or pemphigoid had increased odds of multiple cutaneous, extracutaneous, and systemic autoimmune disorders, which were associated with a considerable cost burden.
  • ||||||||||  Review, Journal:  Dropped Head Syndrome: A Systematic Review. (Pubmed Central) -  Sep 11, 2020   
    A trial of medical management and/or immunomodulators is warranted. Failing nonoperative management, surgery is predictive of a positive outcome.
  • ||||||||||  Review, Journal, Adverse events, Checkpoint inhibition:  Neurologic Immune-Related Adverse Events Associated with Immune Checkpoint Inhibition. (Pubmed Central) -  Sep 10, 2020   
    The outcomes are generally favorable; however, rarely severe events can lead to significant morbidity and even mortality. Identifying and treating the range of neurologic adverse events that may potentially arise with ICIs is very important as the oncologic indications for their use continues to expand.
  • ||||||||||  pyridostigmine Bromide / Generic mfg.
    Clinical, Journal:  Epidemiology of myasthenia gravis in the province of Ourense (Galicia, Spain). (Pubmed Central) -  Sep 10, 2020   
    Identifying and treating the range of neurologic adverse events that may potentially arise with ICIs is very important as the oncologic indications for their use continues to expand. The prevalence of MG in our health district is one of the highest published figures, and the disease is highly prevalent in the older population.
  • ||||||||||  Journal:  Slow-channel myasthenia due to novel mutation in M2 domain of AChR delta subunit. (Pubmed Central) -  Sep 8, 2020   
    Both δL273F and εL269F impair channel gating by disrupting hydrophobic interactions with neighboring α-subunits. Differences in the extent of impairment of channel gating in δ and ε mutant receptors suggest unequal contributions of ε/α and δ/α subunit pairs to gating efficiency.
  • ||||||||||  Clinical, Clinical data, Journal:  Clinical outcome measures following plasma exchange for MG exacerbation. (Pubmed Central) -  Sep 8, 2020   
    Changes from baseline to 2 weeks post-TPE were: MG-ADL median -5.0, P < 0.0033, MG-QoL15 median -13.0, P < 0.001, MG-MMT median -10.0, P < 0.0001, and MG-Composite median -10.0, P < 0.005. TPE produced a rapid, clinically significant change in all instruments, indicating these outcome measures are robust endpoints for clinical trials of rapidly efficacious MG therapies.
  • ||||||||||  prednisone / Generic mfg.
    Journal:  Late-onset myasthenia gravis successfully treated with local resection of cervical ectopic thymoma. (Pubmed Central) -  Sep 5, 2020   
    After the operation, she got minimal manifestations and no relapse of thymoma. Although international and Japanese guidelines recommend extended thymectomy for myasthenia gravis with thymoma, isolated local resection of ectopic thymoma may be enough for controlling myasthenia gravis especially in elderly patients.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    Clinical, Journal:  Antigen specific B cells in myasthenia gravis patients. (Pubmed Central) -  Sep 5, 2020   
    Accumulating studies show rituximab therapy is more effective in MuSK-MG patients than in AChR-MG patients. Advances in molecular biology may lead to greater understanding of pathogenic B cells in MG patients and thus potentially lead to the development of novel therapies for MG.
  • ||||||||||  Biomarker, Journal:  An update on thymectomy in myasthenia gravis. (Pubmed Central) -  Sep 2, 2020   
    Minimally invasive techniques have many advantages over open approaches, provided they are as extensive as trans-sternal thymectomy. The investigation of thymectomy-related biomarkers will contribute to enhance the knowledge of its impact on the specific immune response.
  • ||||||||||  Journal:  Antibody-induced crosslinking and cholesterol-sensitive, anomalous diffusion of nicotinic acetylcholine receptors. (Pubmed Central) -  Aug 29, 2020   
    Cholesterol depletion increases the size of the clustering phenomenon; cholesterol enrichment has the opposite effect. The disclosed high proportion of monoclonal antibody-crosslinked immobile receptors, together with their anomalous, cholesterol-sensitive diffusion and clustering, provides new insights into the antibody-enhanced antigenic modulation that leads to physiopathological internalization and degradation of receptors in myasthenia.
  • ||||||||||  Journal:  Lowering the cut-off value for increment increases the sensitivity for the diagnosis of Lambert-Eaton myasthenic syndrome. (Pubmed Central) -  Aug 29, 2020   
    The disclosed high proportion of monoclonal antibody-crosslinked immobile receptors, together with their anomalous, cholesterol-sensitive diffusion and clustering, provides new insights into the antibody-enhanced antigenic modulation that leads to physiopathological internalization and degradation of receptors in myasthenia. Lowering the cut-off value for abnormal increment to 60% greatly increases sensitivity to diagnose LEMS without an overt loss in specificity.
  • ||||||||||  prednisone / Generic mfg., pyridostigmine Bromide / Generic mfg.
    Clinical, Journal:  Efficacy of thymectomy in patients with non-thymoma myasthenia gravis (Pubmed Central) -  Aug 29, 2020   
    After 12-month follow-up, the dosage of prednisone and pyridostigmine in the operation group was significantly lower than that in the control group (5(0,10)mg/d vs 7.5(5,10)mg/d and 30(0,105)mg/d vs 90(15,180)mg/d; P=0.038, 0.032). In patients with mild to moderate non-thymoma generalized MG, thymectomy does not achieve faster remission, but it does reduce the long-term dosage of prednisone and bromopyrazine.
  • ||||||||||  Review, Journal:  Myasthenia Gravis and Physical Exercise: A Novel Paradigm. (Pubmed Central) -  Aug 28, 2020   
    This knowledge is important in order to establish collective as well as personalized guidelines on physical exercise for MG patients. This review discusses the present knowledge on physical exercise in MG.
  • ||||||||||  Soliris (eculizumab) / Alexion Pharma
    Clinical, PK/PD data, Review, Journal:  Pharmacology, Pharmacokinetics and Pharmacodynamics of Eculizumab, and Possibilities for an Individualized Approach to Eculizumab. (Pubmed Central) -  Aug 27, 2020   
    This review provides insight into the pharmacokinetic and pharmacodynamic properties of eculizumab, both for reported on-label (paroxysmal nocturnal hemoglobinuria, atypical hemolytic uremic syndrome, generalized myasthenia gravis) and off-label (hematopoietic stem cell transplantation-associated thrombotic microangiopathy) indications. Furthermore, we discuss the potential of therapeutic drug monitoring to individualize treatment and reduce costs.
  • ||||||||||  Biomarker, Review, Journal, IO Biomarker:  Paraneoplastic and Therapy-Related Immune Complications in Thymic Malignancies. (Pubmed Central) -  Aug 27, 2020   
    In advanced, refractory cases of TETs (both thymoma and thymic carcinoma), additional therapeutic approaches are needed. Immune checkpoint inhibitors have revolutionized the treatment of several malignancies and hold promise in the treatment of TETs; however, the risks for immune-related adverse events (especially for inducing PNDs as well as in the setting of pre-existing PNDs) underscore the need to optimize patient selection and improve clinical management before there can be widespread acceptance of checkpoint inhibitor therapy in patients with TETs.