Myasthenia Gravis
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  • ||||||||||  WS04: Treatable Myopathies (Gold Hall) -  Jul 17, 2022 - Abstract #ICNMD2022ICNMD_114;    
    Intensive Care Unit Weakness is a major problem for neurology residents and intensive care personnel. The electrodiagnostic and rehablitative features are both critical issues.
  • ||||||||||  TC07: Congenital Myasthenic Syndromes (Meeting Studio 206) -  Jul 17, 2022 - Abstract #ICNMD2022ICNMD_27;    
    The electrodiagnostic and rehablitative features are both critical issues. CMS are a field that is important on all the above-mentioned levels and bears many new and relevant aspects both on the basic research side as well as on the clinical side.
  • ||||||||||  TC03: Towards Targeted Treatments in Myasthenia Gravis: Update on Pathogenetic Mechanism and New Drugs (Meeting Studio 206) -  Jul 17, 2022 - Abstract #ICNMD2022ICNMD_1;    
    Finally, Immune Checkpoint Inhibitors (ICIs) are used widely in oncology and MG or MG/myositis overlap is an important complication of these treatments. The last talk will address immune checkpoints, the mechanism of action ICIs and the neuromuscular complications of ICI therapy with emphasis on the neuromuscular complications.
  • ||||||||||  A rare presentation of autoimmune thyroid diseases and myasthenia gravis: A clinical case report () -  Jul 16, 2022 - Abstract #ENDO2022ENDO_1079;    
    It has been a common practice to screen for associated autoimmune conditions in a patient with known autoimmune disease. However, a rare association exists between acute myasthenia gravis and Graves' disease and could pose a diagnostic challenge for a clinician as both share many overlapping identical clinical features.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    Review, Journal:  Rituximab in juvenile myasthenia gravis-an international cohort study and literature review. (Pubmed Central) -  Jul 15, 2022   
    We compared our cohort to previously reported JMG cases treated with rituximab and noted similar response rates but a slightly higher side-effect profile. Rituximab is a safe and effective treatment option in moderate to severe JMG and most patients have an improvement in MG symptoms post treatment.
  • ||||||||||  Review, Journal:  The relationship between myasthenia gravis and COVID-19: a systematic review. (Pubmed Central) -  Jul 15, 2022   
    COVID-19 infection can increase the risk of new-onset MG, myasthenic crisis, respiratory failure, and mortality rate due to cytokine storm in MG patients. The management of COVID-19 patients with MG is tailored to each person and based on national guidelines and local expert recommendations.
  • ||||||||||  Journal:  An update on the use of immunoglobulins as treatment for myasthenia gravis. (Pubmed Central) -  Jul 15, 2022   
    The alternative of SCIG is also effective and has advantages of infusion time flexibility, fewer side-effects, and patient independence. The safety and efficacy of both interventions, patient preferences and quality of life may direct therapeutic choices in the future.
  • ||||||||||  Journal:  Gut microbiota: a new insight into neurological diseases. (Pubmed Central) -  Jul 14, 2022   
    Such investigations may aid in the development of new microbiota-based strategies for diagnosis and treatment and improve the clinical management of neurological disorders. In this review, we describe the dysbiosis of gut microbiota and the corresponding mechanisms in common neurological diseases and discuss the potential roles that the intestinal microbiome may play in the diagnosis and treatment of neurological disorders.
  • ||||||||||  Journal:  Frequency of Neurological Diseases After COVID-19, Influenza A/B and Bacterial Pneumonia. (Pubmed Central) -  Jul 13, 2022   
    The risk of neurodegenerative and cerebrovascular, but not neuroimmune, disorders was increased among COVID-19 positive outpatients compared to COVID-negative outpatients. However, except for ischemic stroke, most neurological disorders were not more frequent after COVID-19 than after other respiratory infections.
  • ||||||||||  Journal:  Mutation Update: The Spectra of PLEC Sequence Variants and Related Plectinopathies. (Pubmed Central) -  Jul 12, 2022   
    This mutation update analyzes the clinical spectrum of PLEC in our cohort and in the literature and demonstrates the relationship between PLEC genotype and phenotypic manifestations. This study has integrated our seven novel PLEC variants and phenotypic findings with previously published data (totaling 116 variants) to provide the most complete overview of pathogenic PLEC variants and related disorders.
  • ||||||||||  Vyvgart (efgartigimod alfa-fcab) / argenx, Broteio
    Trial completion, Trial completion date, Trial primary completion date:  ADAPT: A Safety and Tolerability Study of ARGX-113 in Patients With Myasthenia Gravis Who Have Generalized Muscle Weakness. (clinicaltrials.gov) -  Jul 11, 2022   
    P3,  N=151, Completed, 
    This study has integrated our seven novel PLEC variants and phenotypic findings with previously published data (totaling 116 variants) to provide the most complete overview of pathogenic PLEC variants and related disorders. Active, not recruiting --> Completed | Trial completion date: Jun 2023 --> Jun 2022 | Trial primary completion date: Jun 2023 --> Jun 2022
  • ||||||||||  Journal:  Immunosuppression in Multiple Sclerosis and Other Neurologic Disorders. (Pubmed Central) -  Jul 10, 2022   
    This chapter focuses on the presently-available therapeutics and, importantly, the future directions of MS therapy based on preclinical studies and early clinical trials. Immunosuppression in other neurological disorders including neuromyelitis optica spectrum disorders, myasthenia gravis, and Guillain-Barré syndrome is also discussed.
  • ||||||||||  Review, Journal:  The glucocorticoid toxicity index: Measuring change in glucocorticoid toxicity over time. (Pubmed Central) -  Jul 9, 2022   
    The GTI has been validated in both real-world experiences and clinical trials, including a phase 3, label-enabling trial in ANCA associated vasculitis. This article reviews the history and rationale for the development of the GTI, describes key data from validation studies, considers the minimum clinically important difference, and provides instructions for use of the instrument.
  • ||||||||||  Journal:  Potpourri of retinopathies in rare eye disease - A case series. (Pubmed Central) -  Jul 7, 2022   
    A case of Senior Loken syndrome in pediatric age is described in this series with varied ophthalmic manifestations ranging from retinitis pigmentosa to orbital abscess. This series also enlightens features of Hallervorden Spatz syndrome presenting with bull's eye maculopathy and a case of spino-cerebellar ataxia type 7 presenting with pigmentary retinopathy.
  • ||||||||||  prednisone / Generic mfg., pyridostigmine Bromide / Generic mfg.
    Journal:  Acupuncture combined with western medication for ocular myasthenia gravis: a randomized controlled trial (Pubmed Central) -  Jul 7, 2022   
    Acupuncture combined with western medication can effectively improve ptosis, palpebra superior fatigability, eye movement disorder and neuromuscular junction dysfunction in patients with ocular myasthenia gravis, the therapeutic effect is superior to simple western medication. Its mechanism may be related to down-regulating serum levels of AChR-Ab, IFN-γ and IL-4 and promoting the recovery of orbicularis oculi function.
  • ||||||||||  Observational data, Journal:  Thymoma and myasthenia gravis - an observational study at a tertiary center. (Pubmed Central) -  Jul 6, 2022   
    This study helped to pinpoint important aspects concerning therapeutic orientation of MGAT patients, such as the clinical impact of thymectomy in the course of MGAT, the oncological prognostic value of surgical resection mar- gins, and the importance of preoperative intravenous immunoglobulin. Management of MGAT patients is only possible with a multidisciplinary approach.
  • ||||||||||  Journal:  Fragmented care and missed opportunities: the experiences of adults with myasthenia gravis in accessing and receiving allied health care in Australia. (Pubmed Central) -  Jul 6, 2022   
    Four themes were identified: (1) missed opportunities and unmet care needs were common, due to frequent patient-provider communication breakdowns and a lack of referral protocols, (2) personal factors - patient self-advocacy influenced their perceived need, with some lacking confidence to seek help, (3) perceived benefit and health provider capacities - most valued allied health despite differing perceptions of health professionals' attitudes, skills, and willingness to learn, and (4) a resultant fragmentation of care between services was universal. Findings highlighted a need for clear referral pathways, coordinated multidisciplinary care, improved access to community-based services and education for allied health professionals about MG.Implications for rehabilitationAdults with myasthenia gravis (MG) report a lack of referral pathways to allied health services, leading to unmet needs and fragmented care.Although there is a perceived benefit to allied health care, experiences are impacted by health provider capacity and attitudes.There needs to be an overall shift towards multidisciplinary care for people with MG, and the development of clear referral pathways.Specific education about MG should be available for allied health professionals to improve their knowledge and skills in treating this population.
  • ||||||||||  Review, Journal:  Paraneoplastic syndrome in neuroophthalmology. (Pubmed Central) -  Jul 3, 2022   
    The performance of paraneoplastic syndrome is diverse, the diagnosis is difficult, and the treatment should be considered systematically. Differential diagnosis, optimal evaluation and management of these manifestations is not only the key to treatment but also a challenge.
  • ||||||||||  Review, Journal:  Neuromuscular Active Zone Structure and Function in Healthy and Lambert-Eaton Myasthenic Syndrome States. (Pubmed Central) -  Jul 2, 2022   
    In LEMS, the reduced neurotransmission is traditionally thought to be caused by the antibody-mediated removal of presynaptic voltage-gated calcium channels. However, recent experimental data and AZ computer simulations have predicted that a disruption in the normally highly organized active zone structure, and perhaps autoantibodies to other presynaptic proteins, contribute significantly to pathological effects in the active zone and the characteristics of chemical transmitters.
  • ||||||||||  Review, Journal:  Neurological Manifestations of Myocarditis. (Pubmed Central) -  Jul 1, 2022   
    Multiple studies suggest the increasing risk of neurologic complications in patients with myocarditis. Neurologists should maintain a high suspicion of myocarditis in cases presenting with both cardiovascular and neurological dysfunction without a clear etiology.
  • ||||||||||  nipocalimab (M281) / J&J
    Enrollment open, Trial completion date, Trial initiation date:  A Study of Nipocalimab in Children Aged 2 to Less Than 18 Years With Generalized Myasthenia Gravis (clinicaltrials.gov) -  Jul 1, 2022   
    P2/3,  N=12, Recruiting, 
    Corticosteroids attenuated the link between optic neuritis and subsequent autoimmune diseases. Not yet recruiting --> Recruiting | Trial completion date: Mar 2025 --> Dec 2025 | Initiation date: Mar 2022 --> Jul 2022