Myasthenia Gravis
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  • ||||||||||  Fatiguing Saccades to Differentiate Ocular Motor Localization: A Pilot Study (Colorado Convention Center | Exhibit Hall B-E) -  Mar 8, 2024 - Abstract #AAN2024AAN_2773;    
    In contrast to what observed in normal subjects and central eye movement disorders, we hypothesize that in ocular myasthenia gravis (OMG), saccadic accuracy changes with fatigue from repetitive stimuli, increasing with repetition count. This would provide the basis for differentiating OMG from non-neuromuscular junction cases.
  • ||||||||||  Eye Movement Characteristics of Congenital Myasthenic Syndromes (Colorado Convention Center | Exhibit Hall B-E) -  Mar 8, 2024 - Abstract #AAN2024AAN_2772;    
    Despite reduced range of motion, saccades in MG have similar or increased peak velocities compared to normal individuals. Furthermore, horizontal saccades in MG tend to have late-course disconjugacy, in contrast to other motility disorders.
  • ||||||||||  Keytruda (pembrolizumab) / Merck (MSD), Avastin (bevacizumab) / Roche
    Successful Treatment of Immune Checkpoint Inhibitor Induced Optic Neuritis (Colorado Convention Center | Exhibit Hall B-E) -  Mar 8, 2024 - Abstract #AAN2024AAN_2607;    
    Our case study provides evidence for a tremendous recovery with PLEX for pembrolizumab-associated optic neuritis. Aggressive acute initial management of ICI-related complications should be considered, especially in cases of increased morbidity and mortality.
  • ||||||||||  Opdivo (nivolumab) / Ono Pharma, BMS
    Immune Checkpoint Inhibitor-associated Kelch-like Protein-11 IgG Rhombencephalitis (Colorado Convention Center | Exhibit Hall B-E) -  Mar 8, 2024 - Abstract #AAN2024AAN_2605;    
    Tumor staining revealed KLHL11 immunoreactivity, supporting an ICI-associated paraneoplastic syndrome. It is critical to recognize and report novel nirAEs, in an effort to initiate early treatment and prevent progressive neurological disability.
  • ||||||||||  mycophenolate mofetil / Generic mfg.
    Brain Lesions in a Myasthenia Gravis Patient on Monotherapy with Mycophenolate Mofetil (Colorado Convention Center | Exhibit Hall B-E) -  Mar 8, 2024 - Abstract #AAN2024AAN_2246;    
    Levetiracetam every twelve hours was started without further seizures...Patient subsequently underwent treatment with intravenous sulfamethoxazole and trimethoprim combination for six weeks...This is a rare case of neurotoxoplasmosis as a complication of monotherapy with mycophenolate mofetil and highlights the importance of having a high index of suspicion for opportunistic neuroinfectious processes. Especially in those patients with immunosuppressive therapy presenting with new neurological deficits or radiological findings.
  • ||||||||||  Vyvgart Hytrulo (efgartigimod alfa and hyaluronidase-qvfc) / argenx
    Clinical Experience with Subcutaneous Efgartigimod for Acetylcholine Receptor-positive Generalized Myasthenia Gravis (Colorado Convention Center | Exhibit Hall B-E) -  Mar 8, 2024 - Abstract #AAN2024AAN_2012;    
    The DOMYA clinical study aims to demonstrate ME&MGTM clinical performance, enabling its registration as a medical device in Europe and the USA. Subcutaneous efgartigimod maintained or improved the clinically meaningful changes in MG-ADL that had been achieved with IV efgartigimod in this cohort of AChR +ve gMG patients.
  • ||||||||||  Journal:  New Multitarget Molecules Derived from Caffeine as Potentiators of the Cholinergic System. (Pubmed Central) -  Mar 7, 2024   
    The nAChR agonist behavior of the compounds depends on their accessory group, whereas their ability to stabilize the receptor in a desensitized state depends on the interactions of the linker at the binding site. Our results show that the new compounds can inhibit AChE and activate nAChR with greater potency than caffeine and provide further information on the modulation mechanisms of pharmacological targets for the design of novel therapeutic interventions in cholinergic deficit.
  • ||||||||||  Journal:  The impact of myasthenia gravis severity on work and daily activities. (Pubmed Central) -  Mar 7, 2024   
    Patients with MG experience substantial work impairment particularly those with more severe symptoms, highlighting an important way in which patient quality of life is negatively affected. More effective treatment strategies would enable patients to lead more productive lives and could impact decisions relating to work and career.
  • ||||||||||  Journal:  How should newer therapeutic agents be incorporated into the treatment of patients with myasthenia gravis? (Pubmed Central) -  Mar 7, 2024   
    There are downsides, however, including treatment cost, unique side effect profiles, and intravenous and subcutaneous drug administration (though for some, this may be an advantage). As additional drugs enter the marketplace with unique mechanisms of action, routes of administration, and dosing schedules, the placement of the novel therapeutic agents in the gMG treatment algorithm will likely evolve.
  • ||||||||||  Journal:  The modified Lyon's position: an alternative approach to robotic thymectomy. (Pubmed Central) -  Mar 5, 2024   
    The lateral approach involves specific trocar placements and port arrangements to minimize conflicts between instruments. This report proposes an innovative approach to robotic thymectomy for patients diagnosed with thymoma or thymic hyperplasia associated with myasthenia gravis.
  • ||||||||||  Journal:  Epidemiology of myasthenia gravis in the United States. (Pubmed Central) -  Mar 4, 2024   
    Our updated US population-based estimates of MG epidemiology demonstrate an increase in the previously reported incidence and prevalence from over 20?years ago, in keeping with developments in westernized, industrialized countries. Notable findings of steadily increasing prevalence with age, driven by robust increases in elderly males, prompts questions for basic-translational research, therapeutics, and public health.
  • ||||||||||  Journal:  Role of recovery of acetylcholine release in compromised neuromuscular junction function. (Pubmed Central) -  Mar 4, 2024   
    Using recordings of endplate potentials, compound muscle action potential (CMAP) and force production in isolated skeletal muscles and living, anesthetized animals, we found that force and CMAP were markedly reduced by even very light activity performed up to 5 s prior to contraction showing that recovery of ACh release was insufficient to maintain synaptic transmission strength. Our results suggest that the timing of depletion and restoration of ACh release may impact clinical signs of weakness and fatigability in patients with impaired neuromuscular transmission and affect the sensitivity of electromyographic recordings in the clinic.
  • ||||||||||  Journal:  Congenital myasthenic syndrome: a tale of two siblings. (Pubmed Central) -  Mar 4, 2024   
    Traditional anticholinesterase inhibitors may not help in congenital myasthenic syndromes and in some variants may actually cause deterioration of symptoms. In this report, we describe a rare case of congenital myasthenic syndrome with heterozygous mutations in CHRNE gene (c.128A?>?T; heterozygous; exon 11) and COLQ gene (c.1201T?>?A; heterozygous; exon 16), which did not show improvement on neostigmine test but responded to treatment with oral salbutamol.
  • ||||||||||  Trial completion date, Trial primary completion date:  Acupuncture in Myasthenia Gravis (AcuMG) (clinicaltrials.gov) -  Mar 2, 2024   
    P=N/A,  N=20, Active, not recruiting, 
    No abstract available Trial completion date: Mar 2024 --> Jul 2024 | Trial primary completion date: Mar 2024 --> Jun 2024
  • ||||||||||  Journal:  Autoimmune thyroid disease and myasthenia gravis: a study bidirectional Mendelian randomization. (Pubmed Central) -  Mar 1, 2024   
    MG has a higher prevalence of TPOAb(OR: 1.84, 95% CI: 1.39 to 2.42, P =1.47e-5) positivity and may be linked to elevated TSH levels(Beta:0.08,95% CI:0.01 to 0.14,P =0.011), while there is no correlation between MG and FT4(Beta:-9.03e-3,95% CI:-0.07 to 0.05,P =0.796). AITD patients are more susceptible to developing MG, and MG patients also have a higher incidence of GD.
  • ||||||||||  Journal:  Home Noninvasive Ventilation in Myasthenia Gravis. (Pubmed Central) -  Feb 29, 2024   
    Taken together, our data highlight the essential role of GMPPB during development and differentiation, especially in myogenic and neuronal cell types. Home NIV was needed in a substantial percentage of medically stable subjects with MG, mainly in those with generalized type and with oropharyngeal and/or respiratory muscle involvement (MGFA grades IIB and IIIB).
  • ||||||||||  Journal:  Clinical features of double seronegative ocular myasthenia gravis. (Pubmed Central) -  Feb 28, 2024   
    About 30% of patients with DS OMG had no obvious NMJ disorder, and an oral pyridostigmine trial test was necessary to diagnose these patients. Although DS OMG is often considered as the mildest form of MG, its prognosis is not optimistic and it requires aggressive therapeutic intervention.
  • ||||||||||  Journal:  Mutations in PTPN11 could lead to a congenital myasthenic syndrome phenotype: a Noonan syndrome case series. (Pubmed Central) -  Feb 27, 2024   
    Although the link between PTPN11 gene and neuromuscular transmission is unconfirmed, an increasing number of patients with RASopathies are affected by muscle weakness and fatigability. Hence, NS or LPDR1 should be considered in children with suspected CMS but negative genetic workup for known CMS genes or additional symptoms indicative of NS, such as facial dysmorphism or intellectual disability.
  • ||||||||||  Journal:  Comparison of three methods for the detection of antibodies against muscle-specific kinase. (Pubmed Central) -  Feb 26, 2024   
    Advantages of non-radioactive methods for the detection of MuSK antibodies include reduced handling and disposal of hazardous materials, potential for automation and the reagents having a longer shelf-life, reducing costs associated with both workflow and lot validations. Thus, commercially available ELISA and transfected cell-based assays are viable alternatives to the traditional radioactive assay used for serologic determination of MuSK IgG.