Myasthenia Gravis
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  • ||||||||||  Enrollment closed, Enrollment change, Trial completion date:  Influence of Minimally Invasive Thymectomy on the Subsequent Clinical Course of Myasthenia Gravis (clinicaltrials.gov) -  Sep 23, 2021   
    P=N/A,  N=248, Active, not recruiting, 
    Coadministration of therapies for underlying malignancy and LEMS improve the functional disability. Recruiting --> Active, not recruiting | N=400 --> 248 | Trial completion date: Jan 2022 --> Jun 2022
  • ||||||||||  dalfampridine / Generic mfg.
    Journal:  Aminopiridines in the treatment of multiple sclerosis and other neurological disorders. (Pubmed Central) -  Sep 22, 2021   
    This review describes the use of APs for the symptomatic treatment of neurological conditions. We will describe trials leading to the approval of the extended-release 4-aminopyridine for MS and evidence in support of the use in other neurological diseases.
  • ||||||||||  Remicade (infliximab) / Merck (MSD), Mitsubishi Tanabe, J&J
    Clinical, Journal, Checkpoint inhibition:  Intensified immunosuppressive therapy in patients with immune checkpoint inhibitor-induced myocarditis. (Pubmed Central) -  Sep 22, 2021   
    The need for IIST was more common in patients who developed myocarditis very early after the start of ICI therapy, as well as when hemodynamic/electrical instability or neuromuscular adverse events occurred. Treatment with infliximab might be associated with an increased risk of cardiovascular death.
  • ||||||||||  Journal:  Bioengineered optogenetic model of human neuromuscular junction. (Pubmed Central) -  Sep 22, 2021   
    By combining tissue engineering, optogenetics, microfabrication, optoelectronics and video processing, we created a novel platform for the precise investigation of the development and degeneration of human NMJ. We demonstrate the utility of this platform for the detection and diagnosis of myasthenia gravis, an antibody-mediated autoimmune disease that disrupts the NMJ function.
  • ||||||||||  Spectrum of myositis as a rare side effect under checkpoint inhibitor therapy (Stream 4) -  Sep 20, 2021 - Abstract #ADO2021ADO_200;    
    In addition to the asymptomatic course and progressive increase in CK (patient 1), the involvement of the bulbar muscles, a myasthenic symptom complex and myocarditis are suggestive clinical findings (patient 2), which suggest a checkpoint inhibitor-induced course. Depending on the severity of the symptoms and other accompanying immunotherapy-associated side effects, the intensity of therapy with glucocorticoids should be adjusted.
  • ||||||||||  Interdisciplinary management of immune-mediated CNS side effects (Stream 4) -  Sep 20, 2021 - Abstract #ADO2021ADO_193;    
    In addition to a description of the symptoms, a clear recommendation is made on the necessary diagnostic measures and therapy depending on the severity of the side effects. With increasing use of the ICI, this interdisciplinary SOP supports the various specialist disciplines at the UKD in the challenging management of immune-mediated neurological NW in everyday clinical practice.
  • ||||||||||  amphotericin b liposome / Generic mfg., azathioprine / Generic mfg.
    Journal:  Visceral leishmaniasis masquerading as drug-induced pancytopenia in myasthenia gravis. (Pubmed Central) -  Sep 19, 2021   
    While working up to evaluate pancytopenia, bone marrow examination revealed presence of Donovan bodies and the patient showed good response to liposomal amphotericin-B. In retrospect, a case of myasthenia gravis, who presented with pancytopenia presumably drug-induced, was found to have visceral leishmaniasis.
  • ||||||||||  Journal:  Acetylcholinesterase inhibitor responsive myasthenia in a Filipino male with X-linked recessive spinal and bulbar muscular atrophy. (Pubmed Central) -  Sep 19, 2021   
    Although the anti-acetylcholine receptor antibody (anti-AChR Ab) was negative, the myasthenic state was confirmed by decremental response in repetitive nerve stimulation and increased jitter frequency and blocking in single fiber-electromyography. While myasthenia gravis and SBMA may co-exist independently in an individual having the signs and symptoms of both conditions, the absence of anti-AChR Ab may imply that myasthenia can be an exaggerated activity-induced fatigue or weakness from the latter.
  • ||||||||||  Journal:  Examination on the necessity of pericardial fat tissue resection in extended thymectomy for myasthenia gravis. (Pubmed Central) -  Sep 18, 2021   
    It is necessary to resect the pericardial fat tissue in patients whose preoperative images show the presence of pericardial fat tissue hyperplasia or ectopic thymoma. However, extensive pericardial fat tissue resection might not be necessary for all MG patients who undergo extended thymectomy with opening of the bilateral pleural by video-assisted or robot-assisted thoracoscopic surgery (RATS) via a subxiphoid approach.
  • ||||||||||  Journal, Video:  A novel method of subxiphoid video-assisted thoracic surgery for thymectomy. (Pubmed Central) -  Sep 18, 2021   
    Compared with the conventional method of S-VATS, the "four-zone one-way" method significantly decreased OT and estimated blood loss. These results demonstrated the feasibility and safety of the "four-zone one-way" method of S-VATS.
  • ||||||||||  zilucoplan (RA101495) / UCB
    Journal:  Chemical synthesis and characterisation of the complement C5 inhibitory peptide zilucoplan. (Pubmed Central) -  Sep 15, 2021   
    Despite being at an advanced stage of clinical development, there have been no published reports in the literature detailing its chemical synthesis. In this work, we describe an approach for the chemical synthesis of zilucoplan and validate that the synthesised compound blocks LPS-induced C5a production from human blood.
  • ||||||||||  Review, Journal:  The Neuromuscular Junction: Roles in Aging and Neuromuscular Disease. (Pubmed Central) -  Sep 15, 2021   
    In this short review, we provide an overview of the pathologic etiology, symptoms, and therapeutic strategies related to the NMJ. In particular, we examine the role of the NMJ as a disease modifier and a potential therapeutic target in neuromuscular injury and disease.
  • ||||||||||  Journal:  Timing and localization of myasthenia gravis-related gene expression. (Pubmed Central) -  Sep 12, 2021   
    For each of these genes several (new) tissues, brain areas and cortical cell types with (relatively) high expression were identified suggesting a potential role for these genes outside skeletal muscle. The possible presence of MG-related antigens outside skeletal muscle suggests that autoimmune MG, congenital MG or treatments targeting the same proteins may affect MG-related protein function in other organs.
  • ||||||||||  Clinical, Retrospective data, Journal:  Rethymectomy in patients with myasthenia gravis and recurrent thymoma (Pubmed Central) -  Sep 12, 2021   
    Rethymectomy is a safe and effective treatment option for patients with refractory myasthenia gravis (especially in case of detected residual thymic tissue) or recurrent thymoma. Radical surgery for recurrent thymoma ensures favorable survival.
  • ||||||||||  Benlysta (belimumab) / GSK
    Clinical, Review, Journal:  Systematic review of safety and efficacy of belimumab in treating immune-mediated disorders. (Pubmed Central) -  Sep 11, 2021   
    Results were further promising for the use of belimumab in patients with rheumatoid arthritis and Sjögren's syndrome. Conversely, results using belimumab in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and myasthenia gravis were rather disappointing.
  • ||||||||||  Vimpat (lacosamide) / UCB, Daiichi Sankyo
    Thymoma Associated with Autoimmune Encephalitis and Subsequent Myasthenia Gravis. () -  Sep 10, 2021 - Abstract #NCS2021NCS_286;    
    Conclusions Thymoma associated paraneoplastic syndromes of multifocal cortical encephalitis and subsequent myasthenia gravis may present in one person. Thymectomy and aggressive immunotherapy are required to achieve clinical improvement.
  • ||||||||||  Soliris (eculizumab) / AstraZeneca
    Autoimmune Encephalitis in a 31-year-old Male with Myasthenia Gravis and COVID-19 () -  Sep 10, 2021 - Abstract #NCS2021NCS_228;    
    Results A 31-year-old male with newly diagnosed acetylcholine receptor positive MG and thymoma requiring prednisone, plasma exchange (PLEX), and eculizumab infusion for outpatient management presented with two weeks of progressive disorientation and psychosis...COVID-19 PCR returned positive, warranting treatment with remdesivir and dexamethasone...Given persistently poor neurological function, he underwent resection of his thymoma followed by treatment with rituximab...We theorize that our patient's autoantibodies and autoimmune encephalitis were triggered by COVID-19 infection. In patients with MG and thymoma who develop new psychosis, autoimmune/paraneoplastic encephalitides should be considered on the differential.
  • ||||||||||  Clinical, Journal:  Mortality rates and causes of death in Swedish Myasthenia Gravis patients. (Pubmed Central) -  Sep 10, 2021   
    When MG was stated as the ultimate cause of death, there was a strikingly higher likelihood of having influenza/pneumonia as a contributing cause of death (OR 2.5, p<0.0001). In conclusion, although we could not confirm a higher mortality rate in Swedish MG patients compared to the general population, and despite modern advancements in treatment, we observed that MG itself was stated as the third most common ultimate cause of death in Swedish MG patients.