Myasthenia Gravis
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  • ||||||||||  Concomitant Chorea, Head Tremor and Cerebellar Degeneration; a Rare Combination in a Case of CRMP-5 Encephalitis () -  Jan 21, 2020 - Abstract #AAN2020AAN_2364;    
    Although chorea is commonly seen with CRMP-5 encephalitis, our patient was unique in the sense that she also had cerebellar degeneration, head tremor, neuropathy, and possibly neuromuscular junction dysfunction, manifesting as diplopia, proximal muscle weakness and positive VGCC antibodies. Thus, multiple neurological manifestations in an elderly patient should warrant further workup for paraneoplastic etiologies to ensure early diagnosis and management with immunosuppressive therapies.
  • ||||||||||  Comparison of Different Criteria for Refractory Myasthenia Gravis () -  Jan 21, 2020 - Abstract #AAN2020AAN_2283;    
    The more conservative criteria resulted in less than 10% of refractory patients in this cohort. The fact that patients classified as refractory with more lenient criteria had less disease severity and fatigue, and better QoL than conservative criteria, suggests that lenient definitions may unnecessarily subject patients to aggressive therapies.
  • ||||||||||  A Simple Question to Self-Report Myasthenia Gravis Severity Can Predict Patient Acceptable States () -  Jan 21, 2020 - Abstract #AAN2020AAN_2281;    
    These results preliminary support IVIG use for ocular symptoms in myasthenia gravis resistant to moderate dose prednisone, however prospective trials are needed. A cut-point of 70% in the SQ can be used to predict acceptable states (PASS) in patients with MG, whereby scores lower than 70% predict unsatisfactory MG status.
  • ||||||||||  Long-term risk of generalization in patients with pure ocular myasthenia gravis () -  Jan 21, 2020 - Abstract #AAN2020AAN_2280;    
    In a preliminary analysis, only older age at onset was associated with generalization; other factors did not appear to influence spread of disease. Results of the propensity score models assessing the effect of prednisone will be presented.
  • ||||||||||  mycophenolate mofetil / Generic mfg.
    Familial MuSK Myasthenia Gravis () -  Jan 21, 2020 - Abstract #AAN2020AAN_2279;    
    Cellcept 1000 mg BID was added and prednisone 60 daily continued with stable improvement... We describe the first report of familial MuSK+ (AChR-) myasthenia gravis in 2 siblings.
  • ||||||||||  Effect of Ramadan fasting on Myasthenia Gravis () -  Jan 21, 2020 - Abstract #AAN2020AAN_2278;    
    A stable outcome was seen with ocular MG, lower pyridostigmine dose and frequency before Ramadan, less number of immunosuppressants and who had grade (IIb) severity or less before fasting. Those who worsened in Ramadan returned to pre-Ramadan status during the follow up period.
  • ||||||||||  Myasthenia Gravis, Myocarditis, and Pericarditis in a Patient with Thymoma () -  Jan 21, 2020 - Abstract #AAN2020AAN_2277;    
    Our patient was treated with immunosuppressives at presentation and hence likely tested negative. Constrictive pericarditis with invasion of the thymoma into the pericardium and pericarditis from post-radiation therapy of thymoma has been described.
  • ||||||||||  Respiratory Assessments in the Neuromuscular Clinic in Patients with Myasthenia Gravis () -  Jan 21, 2020 - Abstract #AAN2020AAN_2275;    
    Outpatients with GMG have a low burden of respiratory complaints, however, nearly 1 in 5 patients have excessive daytime sleepiness and one third of patients have abnormal vital capacity. No additional benefit to testing FVC in the supine position was shown in this study.
  • ||||||||||  Copper levels in maintenance plasmapheresis () -  Jan 21, 2020 - Abstract #AAN2020AAN_1917;    
    Copper levels decrease during periods of maintenance plasmapheresis with recovery on cessation of plasmapheresis sessions. Physicians may consider obtaining baseline copper levels prior to initiation of plasmapheresis and monitoring of levels during maintenance plasmapheresis.
  • ||||||||||  A Rare Case Of Dysferlinopathy With Recurrent Rhabdomyolysis () -  Jan 21, 2020 - Abstract #AAN2020AAN_1809;    
    This case represents an atypical manifestation of dysferlinopathy with severe recurrent rhabdomyolysis and in one instance leading on to respiratory failure. This case also highlights the importance of early muscle biopsy and genetic studies to assist with diagnosis.
  • ||||||||||  Triple M: A case of Nivolumab-induced myasthenic crisis, myocarditis and myositis () -  Jan 21, 2020 - Abstract #AAN2020AAN_1779;    
    This case demonstrates the rare triple M triad of myasthenia crisis, myositis, and myocarditis in a single patient treated with Nivolumab. As more patients with cancer are treated with immune modulating therapies neurologists must be aware of these potential adverse events, which can be life-threatening.
  • ||||||||||  Development of a Patient-Centered Tool for the Assessment of Muscle Cramps () -  Jan 21, 2020 - Abstract #AAN2020AAN_763;    
    Patients to date show high levels of pain and stiffness and show a good range of responses across the various domains in our novel, patient-reported cramp scale. The final results of the validation of the score are expected at time of presentation.
  • ||||||||||  Grave’s Disease Mimicking a Structural Myelopathy () -  Jan 21, 2020 - Abstract #AAN2020AAN_732;    
    Thyroid disorders, in particular Grave’s disease, should be considered in patients presenting with myelopathic signs and symptoms even if findings are asymmetric and mimic a structural lesion. Neurologic symptoms improve with treatment of the underlying thyroid disease.
  • ||||||||||  Analysis of the Duke Myasthenia Gravis (MG) Clinic Registry: Demographic and Clinical Characteristics () -  Jan 21, 2020 - Abstract #AAN2020AAN_558;    
    This initial analysis demonstrates increasing onset age over 3 decades for both men and women, the variable diagnoses initially given to explain patients’ symptoms, and decreasing time to specialty clinic referral. The database is currently undergoing data verification to permit further analyses including frequency of ocular MG and generalization, response to treatments, and factors predicting outcome.
  • ||||||||||  Clinical, Retrospective data, Journal:  Clinical differences of early and late-onset myasthenia gravis in 985 patients. (Pubmed Central) -  Jan 17, 2020   
    Rarer still is involvement of the extraocular muscles in childhood thyroid disease. These results have potential significance for the recognition of clinical features and the determination of management strategies in EOMG and LOMG.
  • ||||||||||  Review, Journal, Checkpoint inhibition, IO Biomarker:  Neurologic Autoimmunity in the Era of Checkpoint Inhibitor Cancer Immunotherapy. (Pubmed Central) -  Jan 15, 2020   
    Importantly, these disorders generally respond favorably to early-initiated immunotherapy and cancer treatment. Small cell lung cancer and thymoma are commonly associated with neurologic autoimmunity, but in the context of checkpoint inhibitor therapy, other malignancy associations are increasingly recognized.
  • ||||||||||  Review, Journal, IO Biomarker:  Trouble at the junction: when myopathy and myasthenia overlap. (Pubmed Central) -  Jan 15, 2020   
    The identification of a defect of neuromuscular transmission in an inherited myopathy may assist in establishing a molecular diagnosis and in selecting patients who would benefit from pharmacological correction of this defect. Acquired cases meanwhile stem from the co-occurrence of myasthenia gravis or Lambert-Eaton myasthenic syndrome with an immune-mediated myopathy, which may be due to paraneoplastic disorders or exposure to immune checkpoint inhibitors.
  • ||||||||||  Journal:  Factors Associated with Acute Exacerbations of Myasthenia Gravis. (Pubmed Central) -  Jan 15, 2020   
    Patients prescribed beta-blockers were associated with more exacerbations (P<0.01). Patients prescribed medications that may worsen MG had a higher exacerbation frequency shortly after administration Infections and cautioned medications are frequently factors in acute MG exacerbations needing urgent medical attention and warrant caution.
  • ||||||||||  Rituxan (rituximab) / Roche, Biogen
    Clinical, Journal:  Rituximab Desensitization in Two Patients with MuSK Myasthenia Gravis. (Pubmed Central) -  Jan 15, 2020   
    Patients prescribed medications that may worsen MG had a higher exacerbation frequency shortly after administration Infections and cautioned medications are frequently factors in acute MG exacerbations needing urgent medical attention and warrant caution. No abstract available