Myasthenia Gravis
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  • ||||||||||  Journal, Checkpoint inhibition:  Consider Myocarditis When Patients Treated with Immune Checkpoint Inhibitors Present with Ocular Symptoms. (Pubmed Central) -  May 12, 2022   
    We report a case series of 3 patients treated with ICIs presenting with diplopia or ptosis, found to have concurrent myocarditis in addition to immune-related myopathy (irMyopathy) or myasthenia gravis (irMG). None of the patients described cardiac symptoms, underscoring the importance of screening for myocarditis in patients presenting with diplopia and/or other neuromuscular symptoms which may suggest either irMyopathy or irMG.
  • ||||||||||  Journal:  Thymic Lesions in Myasthenia Gravis: A Clinicopathological Study from India. (Pubmed Central) -  May 12, 2022   
    AChR antibody positivity rate was more frequent in thymomas. This study indicates that clinico-radiological evaluation adequately supported by serology and histopathology can effectively recognize the type of thymic pathology that can guide these patients' treatment planning, management, prognosis and follow-up.
  • ||||||||||  Enrollment change, Trial withdrawal, Adherence:  Medication Adherence and Non-adherence in Adults With Rare Disease (clinicaltrials.gov) -  May 12, 2022   
    P=N/A,  N=0, Withdrawn, 
    This study indicates that clinico-radiological evaluation adequately supported by serology and histopathology can effectively recognize the type of thymic pathology that can guide these patients' treatment planning, management, prognosis and follow-up. N=1000 --> 0 | Not yet recruiting --> Withdrawn
  • ||||||||||  Retrospective data, Journal:  A retrospective longitudinal cohort study of the clinical burden in myasthenia gravis. (Pubmed Central) -  May 12, 2022   
    These results show that conventional treatments for MG are not adequately managing patients' symptoms and that patients with refractory MG are more likely to experience certain comorbidities than those with non-refractory MG or matched controls without MG. Future research should focus on the impact of newer targeted therapies on long-term clinical outcomes and comorbid conditions.
  • ||||||||||  Darzalex (daratumumab) / J&J
    Journal:  Daratumumab for treatment-refractory antibody-mediated diseases in neurology. (Pubmed Central) -  May 10, 2022   
    Larger prospective studies are required to provide further evidence. Our findings suggest that daratumumab provided a clinically relevant depletion of autoreactive long-lived plasma cells, identifying plasma cell-targeted therapies as promising escalation therapy for highly active, otherwise treatment-refractory autoantibody-mediated neurological diseases.
  • ||||||||||  Review, Journal:  Long Non-Coding RNAs in the Cell Fate Determination of Neoplastic Thymic Epithelial Cells. (Pubmed Central) -  May 10, 2022   
    Starting from our recently published studies, this review focuses on the involvement of lncRNAs in the acquisition of malignant traits by neoplastic thymic epithelial cells, and describes the possible use of these molecules as targets for the design of novel therapeutic approaches specific for TET. Furthermore, the involvement of lncRNAs in myasthenia gravis (MG)-related thymoma, which is still under investigation, is discussed.
  • ||||||||||  Journal:  Oculopharyngeal muscular dystrophy coexisting with myasthenia gravis. (Pubmed Central) -  May 10, 2022   
    We report a patient with atypical OPMD who also had acetylcholine receptor antibody-mediated myasthenia gravis. This report raises awareness of the diagnostic and treatment implications of the association of myasthenia gravis with genetic myopathies.
  • ||||||||||  Review, Journal:  Autoimmune Neurogenic Dysphagia. (Pubmed Central) -  May 10, 2022   
    Less common, but often overlooked, is dysphagia in stiff-person syndrome especially in conjunction with cerebellar ataxia and high anti-GAD autoantibodies, and in gastrointestinal dysmotility syndromes associated with autoantibodies against the ganglionic acetyl-choline receptor. In the setting of many neurological autoimmunities, acute-onset or progressive dysphagia is a potentially treatable condition, requiring increased awareness for prompt diagnosis and early immunotherapy initiation.
  • ||||||||||  Journal:  Engineering and Characterization of an Optogenetic Model of the Human Neuromuscular Junction. (Pubmed Central) -  May 9, 2022   
    Stimulation of optogenetic motoneurons by light to induce skeletal muscle contractions recapitulates human NMJ physiology and allows for repeated functional measurements of NMJ over time and in response to various inputs. We demonstrate this platform's ability to show functional improvements in neuromuscular connectivity over time and characterize the damaging effects of patient MG antibodies or neurotoxins on NMJ function.
  • ||||||||||  riluzole / Generic mfg.
    Clinical, Journal:  ALS patients with concurrent neuroinflammatory disorders; a nationwide clinical records study. (Pubmed Central) -  May 6, 2022   
    Compared to patients with only ALS, the concurrent patients were significantly older at symptoms onset, had higher prevalence of bulbar onset, but used Riluzole and noninvasive ventilation less frequently...A minority of patients had a true concurrence, where MS, MG, IP, and DMPM preceded the ALS diagnosis, which might be due to chance alone. Four patients were diagnosed with MG shortly after onset of ALS, suggesting that neurodegeneration might trigger autoimmunity.
  • ||||||||||  Inaugural dropped head syndrome and camptocormia in inflammatory myopathies (Poster View 5) -  May 6, 2022 - Abstract #EULAR2022EULAR_2256;    
    Initial neuroimaging combined with laboratory testing is useful and provides rational tools for proper diagnosis. While IM diagnosis is challenging in the presence of inaugural axial involvement, these results highlight the subset of IM to be considered according to the age at first symptoms and the type of axial involvement (e.g., DHS and/or CC).
  • ||||||||||  Review, Journal:  Role of the CXCL13/CXCR5 Axis in Autoimmune Diseases. (Pubmed Central) -  May 6, 2022   
    In this review, we discuss the biological features of CXCL13 and CXCR5 and the recent findings on the pathogenic roles of the CXCL13/CXCR5 axis in autoimmune diseases. Furthermore, we discuss the potential role of CXCL13 as a disease biomarker and therapeutic target in autoimmune diseases.
  • ||||||||||  Clinical, Journal:  Effectiveness of modified Buzhong Yiqi decoction in treating myasthenia gravis: study protocol for a series of N-of-1 trials. (Pubmed Central) -  May 5, 2022   
    Some confounding variables such as TCM syndrome type and potential carryover effect of TCM will be introduced into the hierarchical Bayesian statistical method to improve the sensitivity and applicability of the trials, and the use of prior available information within the analysis may improve the sensitivity of the results of a series of N-of-1 trials, from both the individual and population level to study the efficacy of TCM syndrome differentiation. We assumed that this study would reveal that MBYD is effective for MG and provide robust evidence of the efficacy of TCM to treat MG.
  • ||||||||||  Review, Journal:  CD40-CD40L in Neurological Disease. (Pubmed Central) -  Apr 30, 2022   
    We review the role of CD40-CD40L in traumatic brain injury, Alzheimer's Disease, Parkinson's Disease, stroke, epilepsy, nerve injury, multiple sclerosis, ALS, myasthenia gravis and brain tumors. We also highlight therapeutic advancements targeting the CD40 system to either attenuate the neuroinflammatory response or leverage the downstream effects of CD40 signaling for direct tumor cell lysis.
  • ||||||||||  Biomarker, Review, Journal:  Exploring the Gut Microbiome in Myasthenia Gravis. (Pubmed Central) -  Apr 30, 2022   
    Probiotics may influence MG by restoring the gut microbiome imbalance, aiding the prevention of MG, and lowering the risk of gut inflammation by normalizing serum biomarkers. Hence, this review will discuss how alterations of gut microbiome composition and function relate to MG and the benefits of gut modulation.
  • ||||||||||  Journal:  Minimally invasive combined approach robotic thymectomy and transcervical total thyroidectomy. (Pubmed Central) -  Apr 30, 2022   
    The treatment of thyroid and thymus pathologies varies drastically from medical observation to surgery. This combined approach surgery clearly benefits the patient by offering similar operative time, fewer operative and postoperative recovery experiences, decreased anaesthesia risks associated with MG patients through fewer intubations, and a faster return to baseline function.
  • ||||||||||  Review, Journal, Checkpoint inhibition:  Immune checkpoint inhibitor-induced myocarditis, myositis, myasthenia gravis and transaminitis: a case series and review. (Pubmed Central) -  Apr 29, 2022   
    Herein the authors review the literature and describe the current diagnostic and management approach for this overlapping syndrome. The authors' series highlights the importance of a high index of clinical suspicion, prompt comprehensive investigations, early multidisciplinary team involvement and initiation of immunosuppressive therapy when immune-related adverse events are suspected.
  • ||||||||||  Journal:  Intravenous immunoglobulin-induced eczematous eruption in autoimmune neuromuscular diseases (Pubmed Central) -  Apr 29, 2022   
    MADSAM or MMN patients had more frequently IVIg-induced eczematous eruption than other autoimmune neuromuscular diseases. Pathophysiology of MADAM and MMN is considered to be cell-mediated immunity against the peripheral nerve and the accumulation of IgG in both epidermis and dermis of the hand after IVIg may induce the infiltration of inflammatory cells around the vessels in the skin, causing eczematous eruption in MADSAM and MMN.
  • ||||||||||  Journal:  Analysis of clinical features of myasthenia gravis complicated with hyperthyroidism. (Pubmed Central) -  Apr 29, 2022   
    Patients with MG complicated with hyperthyroidism are mainly female, with a lower age of onset, a lower level of acetylcholine receptor antibody, a lower incidence of thymoma, and a higher incidence of thymic hyperplasia. The clinical features of such patients are remarkably different from those of MG without thyroid disease.
  • ||||||||||  Retrospective data, Review, Journal:  Spontaneous remission in juvenile myasthenia gravis: A cohort of 13 cases and review of the literature. (Pubmed Central) -  Apr 28, 2022   
    Strikingly, none of the 36 patients with thyroid antibodies had spontaneous remission compared with 13/58 (22.4%) of those without thyroid antibodies (95% CI: 7.3-21.8; p < 0.001). Ten percent of patients with juvenile myasthenia gravis achieved spontaneous remission, mainly in those with Class II-III disease and no associated thyroid antibodies.
  • ||||||||||  mezagitamab (TAK-079) / Takeda
    Enrollment closed:  A Study of TAK-079 in People With Generalized Myasthenia Gravis (clinicaltrials.gov) -  Apr 28, 2022   
    P2,  N=36, Active, not recruiting, 
    The assay may provide a better understanding of the heterogeneous autoantibody molecular pathology and identify patients expected to benefit from complement inhibitor therapy. Recruiting --> Active, not recruiting
  • ||||||||||  Thymoma and pure red cell aplasia: A single institution experience. () -  Apr 28, 2022 - Abstract #ASCO2022ASCO_5784;    
    Most patients will recover with immunosuppressive therapy, but approximately 30.0% patients remained transfusion dependent. Our experience does not support thymectomy alone as primary treatment of PRCA.