Myasthenia Gravis
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  • ||||||||||  Soliris (eculizumab) / Alexion Pharma
    [VIRTUAL] Neuromuscular Clinician Preferences and Experience in the Management of Myasthenic Crisis () -  Mar 18, 2021 - Abstract #AAN2021AAN_1932;    
    This study provides Class IV evidence that channel blocker therapy in SCCMS patients improves symptoms. Our survey indicates that there is considerable variability in the acute and ongoing treatment of myasthenic crisis, and escalated therapy continues into the post-discharge recovery period.
  • ||||||||||  [VIRTUAL] Structured Review of Opportunistic Infections in Neuromuscular Disease () -  Mar 18, 2021 - Abstract #AAN2021AAN_1927;    
    Infections are most commonly reported in myositis, which may reflect a tendency in this disease or reporting bias in the literature. The relative lack of reported infections in inflammatory neuropathies suggests opportunistic infections in inflammatory neuropathies may be underreported.
  • ||||||||||  Actemra IV (tocilizumab) / Roche, JW Pharma
    [VIRTUAL] Characteristics and Outcomes of Myasthenia Gravis Patients with COVID-19 – A Case Series () -  Mar 18, 2021 - Abstract #AAN2021AAN_1912;    
    The management of immunosuppression in this patient population is challenging, but a benefit of immunosuppression is suggested. Further larger scale investigations to delineate specific guidelines and establish factors that affect clinical outcomes in this specific patient population is warranted.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    [VIRTUAL] Rituximab in refractory myasthenia gravis: experience in a single healthcare center in Mexico. () -  Mar 18, 2021 - Abstract #AAN2021AAN_1898;    
    This real-world study demonstrated improvement in outcome measures and decreased concomitant drug requirement within 12 months of eculizumab initiation in patients with treatment-refractory AChR+ gMG. Rituximab appears safe and effective for the treatment of refractory MG with positive AchR antibodies.
  • ||||||||||  [VIRTUAL] Immune-mediated Rippling Muscles with and without Myasthenia Gravis () -  Mar 18, 2021 - Abstract #AAN2021AAN_1897;    
    The caveolin-3 staining in hRMD is homogeneously and much more markedly decreased, with dystrophic changes reported on muscle biopsies. These analogous findings suggest that iRMD and hRMD may share common pathophysiological characteristics.
  • ||||||||||  [VIRTUAL] An unusual case of MuSK- Congenital Myasthenic Syndrome () -  Mar 18, 2021 - Abstract #AAN2021AAN_1896;    
    This unique patient with MuSK-CMS and a compound heterozygote mutation showed clinical features similar to autoimmune MuSK-Myasthenia, with a favorable response to anticholinesterase agents. It is imperative to create awareness of the variable spectrum of MuSK-CMS to ensure proper identification and treatment, and prevent unnecessary interventions.
  • ||||||||||  Actemra IV (tocilizumab) / Roche, JW Pharma
    [VIRTUAL] Case Series of Myasthenia Gravis patients with COVID-19 infection () -  Mar 18, 2021 - Abstract #AAN2021AAN_1255;    
    The prognosis of patients with MG and COVID-19 infection was favorable based on the results of these patients. The baseline immuno-suppressive therapy was not associated with worse outcomes and hence maintenance doses should be continued.
  • ||||||||||  [VIRTUAL] The Autoimmune Encephalitis: Historical Overview () -  Mar 18, 2021 - Abstract #AAN2021AAN_1185;    
    The baseline immuno-suppressive therapy was not associated with worse outcomes and hence maintenance doses should be continued. The 20th century discoveries in pathophysiology behind autoimmune encephalitis resulted in the evolution of emerging entity within the field of neurology characterized by a growing number of antibodies discovered in last two decades.
  • ||||||||||  [VIRTUAL] Brachial Plexopathy: A Rare Association With A Wasp Sting () -  Mar 18, 2021 - Abstract #AAN2021AAN_960;    
    Further research is needed to determine what factors influence an individual’s risk of neurological autoimmune disease complication while receiving immune checkpoint inhibitor therapy. This case highlights the rare association of brachial plexopathy with wasp sting.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    [VIRTUAL] A Case of Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM syndrome) in a Patient with Myasthenia Gravis () -  Mar 18, 2021 - Abstract #AAN2021AAN_311;    
    Further studies in larger cohorts are needed to determine the exact incidence of multilocus variation across different populations with implications for disease management. This case describes a patient with MG presenting clinically with PERM, found to have positive anti-GAD antibodies and symptom resolution with rituximab. For prompt diagnosis and treatment of this life-threatening disorder, it is important recognize PERM in patients with existing autoimmune disorders.
  • ||||||||||  Review, Journal:  The role of innate immunity in myasthenia gravis. (Pubmed Central) -  Mar 17, 2021   
    Futher, we also highlight the role of innate immune cells in the pathogenic response. Finally, we provide some future perspectives in developing new therapeutic approaches particularly targeting the innate immunity for MG.
  • ||||||||||  Biomarker, Journal:  Surgical effect and prognostic factors of myasthenia gravis with thymomas. (Pubmed Central) -  Mar 16, 2021   
    What this study adds Our study had a relatively large sample size of MG patients with thymomas only. We emphasize the analysis of the postoperative effect of MG and overall survival for these patients, which is a complement to previous studies.
  • ||||||||||  Biomarker, Clinical, Journal, Next-generation sequencing:  Targeted Next-Generation Sequencing in a Large Cohort of Genetically Undiagnosed Patients with Neuromuscular Disorders in Spain. (Pubmed Central) -  Mar 16, 2021   
    In combination with patients' clinical and myopathological data, the custom gene panel designed in our lab proved to be a powerful tool to diagnose patients with myopathies, muscular dystrophies and congenital myasthenic syndromes. Targeted NGS approaches enable a rapid and cost-effective analysis of NMD- related genes, offering reliable results in a short time and relegating invasive techniques to a second tier.
  • ||||||||||  Journal:  Identifying jitter outliers in single fiber electromyography: comparison of four methods. (Pubmed Central) -  Mar 16, 2021   
    Simple estimation of outlier limits by adding two SDs to the mean of the data, leads to unacceptable deviations from the true cut-off values. Moreover, in a clinical scenario in which the final electrodiagnosis depends only on the number of outliers, it is meaningful to accept a tolerance zone of about 2 μs, which is the approximate variation range among the different methods.
  • ||||||||||  Clinical, Journal:  Autoantibodies in Japanese patients with ocular myasthenia gravis. (Pubmed Central) -  Mar 16, 2021   
    Abs against fetal-specific AChR, MuSK and LRP4 are found in some patients with OMG. Further studies attempting to predict conversion from ocular symptoms to generalized MG may benefit from the measurement of these antibodies.
  • ||||||||||  Journal:  The Duke Myasthenia Gravis Clinic Registry: I. Description and Demographics. (Pubmed Central) -  Mar 16, 2021   
    These observations demonstrate the value of collecting comprehensive clinical information and comparing historic and contemporary populations. Other potential uses of Registry data include comparison of outcome measures in different disease subgroups and the response to specific treatments.
  • ||||||||||  prednisolone / Generic mfg., azathioprine / Generic mfg.
    Clinical, Journal:  Impact of COVID-19 pandemic and lockdown in a cohort of myasthenia gravis patients in India. (Pubmed Central) -  Mar 16, 2021   
    Other potential uses of Registry data include comparison of outcome measures in different disease subgroups and the response to specific treatments. COVID-19 and lockdown were associated with anxiety, depression, poor MGQOL and sleep especially in severe MG patients.
  • ||||||||||  efgartigimod IV (ARGX-113 IV) / argenx, Broteio
    Review, Journal, IO biomarker:  The importance of FcRn in neuro-immunotherapies: From IgG catabolism, FCGRT gene polymorphisms, IVIg dosing and efficiency to specific FcRn inhibitors. (Pubmed Central) -  Mar 16, 2021   
    Whether FCGRT polymorphisms affect the efficacy of other therapeutic monoclonal antibodies by influencing their distribution clearance and pharmacokinetics, explaining their variable effectiveness, is also addressed. Finally, the very promising effect of monoclonal antibodies that inhibit FcRn, such as efgartigimod, rozanolixizumab and nipocalimab, in treating antibody-mediated neurological diseases is discussed along with their efficacy in the IgG4 subclass of pathogenic antibodies and their role in the blood-brain barrier endothelium, that abundantly expresses FcRn.
  • ||||||||||  [VIRTUAL] The Misdiagnosis of Chronic Obstruction Pulmonary Disease, An Observational Study () -  Mar 14, 2021 - Abstract #ATS2021ATS_3878;    
    It appears that the diagnosis of COPD is carried by patients who do not have any diagnostic evidence of the disease resulting in overuse of bronchodilator therapy and several missed diagnoses. Creating an educational intervention at the MHC may be necessary to reduce misdiagnosis of COPD and overuse of bronchodilators.
  • ||||||||||  hydrocortisone / Generic mfg., propranolol / Generic mfg.
    [VIRTUAL] Thymolipoma Found During Thyroid Storm () -  Mar 14, 2021 - Abstract #ATS2021ATS_3754;    
    Thymolipoma has been said to be associated with certain autoimmune diseases including Myasthenia Gravis, hypogammaglobulinemia, and red cell aplasia. We report the first case of thymolipoma in a patient presenting with thyroid storm.