Myasthenia Gravis
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  • ||||||||||  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.
  • ||||||||||  Clinical, Journal:  Glycopyrrolate and the Management of "Death Rattle" in Patients with Myasthenia Gravis. (Pubmed Central) -  Jun 22, 2021   
    Agents that antagonize acetylcholine activity (e.g., anticholinergic agents, such as glycopyrrolate) can, therefore, exacerbate myasthenia gravis. We discuss the case of a patient dying with myasthenia gravis that developed problematic "death rattle," and the successful use of glycopyrrolate in treating this symptom.
  • ||||||||||  Journal:  Unravelling the role of Receptor for advanced glycation end products (RAGE) and its ligands in Myasthenia Gravis. (Pubmed Central) -  Jun 22, 2021   
    Several studies have shown that the presence of thymic abnormalities, the onset age of MG and the duration of the disease may affect the levels of these proteins in MG patients. Herein, we discuss the emerging role of RAGE and its ligands in MG immunopathogenesis, their clinical significance as promising biomarkers, as well as the potential therapeutic implications of targeting RAGE signaling in MG treatment.
  • ||||||||||  Journal:  Gene expression profiling of orbital muscles in treatment-resistant ophthalmoplegic myasthenia gravis. (Pubmed Central) -  Jun 22, 2021   
    The gene expression in orbital muscles derived from OP-MG individuals compared with normal controls, support the pathogenic hypothesis previously generated from whole genome sequence analyses. Repression of gene transcripts in OP-MG orbital muscle implicate tissue-specific regulatory mechanisms, which may inform future biomarker discovery approaches.
  • ||||||||||  Journal:  Specific Induction of Double Negative B Cells During Protective and Pathogenic Immune Responses. (Pubmed Central) -  Jun 22, 2021   
    Immunoglobulin VH transcriptome sequencing and analysis of recombinant antibodies revealed clonal expansion of DN B cells that were targeted against the vaccine antigen. Our study suggests that DN B cells are expanded in multiple inflammatory neurologic diseases and represent an inducible B cell population that responds to antigenic stimulation, possibly through an extra-follicular maturation pathway.
  • ||||||||||  Journal:  Subclinical myasthenia gravis in thymomas. (Pubmed Central) -  Jun 22, 2021   
    One in four patients who experienced no neurological symptoms before thymectomy, appeared to have anti-AChR-antibodies and 91 % of these patients developed clinical MG within six years after the thymectomy. Analyzing anti-AChR-antibodies in the serum is recommended in all suspected thymomas before a thymectomy is performed.
  • ||||||||||  Clinical, Journal:  COLQ-Related Congenital Myasthenic Syndrome in a Child from Western India. (Pubmed Central) -  Jun 22, 2021   
    The aim of this review is to provide an update on findings arguing for a common pathogenic mechanism in IgG4-AID in general and to provide hypotheses about the role of distinct HLA haplotypes, T-cells and cytokines in IgG4-AID. No abstract available
  • ||||||||||  Journal:  MiR-522-3p inhibits proliferation and activation by regulating the expression of SLC31A1 in T cells. (Pubmed Central) -  Jun 22, 2021   
    Overexpressed SLC31A1 reversed the inhibitory effects of miR-522-3p mimic on cell viability, EdU positive cell, cycle progression, and the levels of IL-2 and IL-10 in transfected Jurkat cells. MiR-522-3p expression was down-regulated in TAMG, and miR-522-3p inhibited proliferation and activation by regulating SLC31A1 expression in T cells.
  • ||||||||||  Clinical, Journal:  A Case Report of Subclinical Myasthenia Gravis Associated with Castleman's Disease. (Pubmed Central) -  Jun 19, 2021   
    CONCLUSIONS The aim of this case report is to add more to the literature by reporting a rare case of an asymptomatic subclinical Myasthenia Gravis associated with Castleman's disease. It highlights the importance of considering a Castleman's Disease in an asymptomatic case who presented with a mediastinal mass and in order to avoid an unusual intraoperative finding such as massive bleeding by performing a biopsy and an angiography preoperatively.
  • ||||||||||  Review, Journal:  Noninfectious neurologic complications of hematopoietic cell transplantation: A systematic review. (Pubmed Central) -  Jun 16, 2021   
    These findings demonstrate that the recipients of HCT had a significantly higher risk of neurological complications and that their early recognition can enhance the monitoring of HCT survivors for the early developmental signs of neurological toxicity. This would facilitate timely interventions, thus ensuring a better quality of life.
  • ||||||||||  Biomarker, Journal:  Peripheral blood hsa-circRNA5333-4: A novel biomarker for myasthenia gravis. (Pubmed Central) -  Jun 12, 2021   
    In the third cohort, hsa-circRNA5333-4 had a larger AUC: 0.864 (95% confidence interval [CI] = 0.801-0.928, P < 0.001), a stronger correlation with the Quantitative Myasthenia Gravis Score (qMG): r = 0.505 (P < 0.001) and was correlated with gender and acetylcholine receptor antibody levels (P < 0.05). So hsa-circRNA5333-4 represents a novel biomarker for the diagnosis and monitoring of MG.
  • ||||||||||  Clinical, Journal:  Developing outcome measures of disease activity in Pediatric Myasthenia. (Pubmed Central) -  Jun 11, 2021   
    Our study suggests that MGC and PM-QOL15 are important disease outcome measures in pediatric myasthenia that are easy to administer and provide reliable assessment of disease activity in the clinic setting. Further studies are needed to validate their use for pediatric clinical research trials.
  • ||||||||||  Clinical, Review, Journal:  Ectopic thymoma in the middle mediastinum: A case report and literature review. (Pubmed Central) -  Jun 10, 2021   
    Most of them were located in the upper right mediastinum, adjacent to the right trachea, and in the retro innominate vein area. Thymoma should be considered a differential diagnosis when a mass is observed in the middle mediastinum.
  • ||||||||||  Clinical, Journal:  Sleep disorders in patients with myastenia gravis (Pubmed Central) -  Jun 10, 2021   
    Thus, given the inconclusive evidence and limited literature, further study of sleep disorders in patients with MG is needed. The topic is relevant and requires further development.
  • ||||||||||  Journal:  Paraneoplastic neurological syndromes (Pubmed Central) -  Jun 10, 2021   
    A prompt diagnosis of neurological paraneoplastic syndrome is of major importance as it often reveals the underlying tumour. The treatment relies on both the elimination of the neoplasia and the control of the immune response.
  • ||||||||||  Clinical, Journal:  Risk factors associated with myasthenia gravis in thymoma patients: The potential role of thymic germinal centers. (Pubmed Central) -  Jun 9, 2021   
    We also clearly associated MG symptoms with higher IFN-γ, IL-1β and sCD40L serum levels, specifically in MGT patients compared to TOMA patients. Altogether, these analyses allowed the clear identification of histological, in particular the presence of GCs, and biological parameters that would facilitate the evaluation of the probability of the MG outcome postoperatively in thymoma patients.
  • ||||||||||  Review, Journal:  Myasthenia gravis at the crossroad of COVID-19: focus on immunological and respiratory interplay. (Pubmed Central) -  Jun 8, 2021   
    Infections are recognized trigger of exacerbations and crisis in MG and patients with MG probably exhibit a mortality higher than the general population during this COVID-19 pandemic. We review the current state of knowledge on MG during the COVID-19 pandemic to focus the immunological and respiratory interplay between these two conditions.
  • ||||||||||  Blincyto (blinatumomab) / Astellas, Amgen, Yervoy (ipilimumab) / Ono Pharma, BMS
    Review, Journal:  Neurological complications of cancer immunotherapy. (Pubmed Central) -  Jun 8, 2021   
    The approval of ipilimumab, a monoclonal antibody targeting the immune cell receptor CTLA-4, has marked the beginning of the era of immune checkpoint inhibitors...Close clinical monitoring for neurological symptoms is key for early recognition of immunotherapy-related side effects. Comprehensive diagnostic work-up and adequate therapeutic measures are essential to avoid further clinical deterioration and residual neurological deficits.