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Clinical characteristics of myasthenia gravis (MG) patients developing other autoimmune diseases after thymectomy from one single center cohort - PubMed

We observed a higher incidence rate of autoimmune diseases, especially rheumatoid arthritis, in MG patients after thymectomy. The most common types of ADs after thymectomy were different from those before ..... see more

Source : https://pubmed.ncbi.nlm.nih.gov/36419347/


Conclusions: We observed a higher incidence rate of autoimmune diseases, especially rheumatoid arthritis, in MG patients after thymectomy. The most common types of ADs after thymectomy were different from those before thymectomy. New onset ADs tended to occur in female and young nonthymoma MG patients. The postoperative effect of MG was not...

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Test-Retest Reliability of Repetitive Ocular Vestibular Evoked Myogenic Potentials in Myasthenia Gravis Patients and Healthy Control Subjects - PubMed

This study shows that in our hands, the test-retest reliability of the ROVEMP is not optimal. Measurements with higher reference amplitude had a better quality, higher reproducibility, and increased diagnostic ..... see more

Source : https://pubmed.ncbi.nlm.nih.gov/36413652/


Conclusions: This study shows that in our hands, the test–retest reliability of the ROVEMP is not optimal. Measurements with higher reference amplitude had a better quality, higher reproducibility, and increased diagnostic yield. We caution against the use of ROVEMP measurements of lower amplitude in clinical practice. In addition, given the...

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Therapeutic plasma exchange for myasthenia gravis, Guillain-Barre syndrome and other immune-mediated neurological diseases, over a 40-year experience - PubMed

doi: 10.1080/14737175.2022.2147827. Online ahead of print. 1 Neuroimmunology and Multiple Sclerosis Unit, Department of Neurology, Hospital Universitario La Paz & Institute for Health Research-IdiPAZ (La Paz University Hospital-Universidad Autónoma de ..... see more

Source : https://pubmed.ncbi.nlm.nih.gov/36408604/


Conclusions: TPE is well-tolerated with low complication rate (<4% procedures), mainly hypotensive/vasovagal reactions. Patients with GBS seem more prone to them than MG patients. Acquaintance with this technique seems necessary."

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Motor end-plate analysis to diagnose immune-mediated myasthenia gravis in seronegative patients - PubMed

This study aimed to evaluate the diagnostic usefulness of motor end-plate (MEP) analysis along with clustered acetylcholine receptor (AChR) antibody (Ab) assays in patients with myasthenia-like symptoms but negative routine ..... see more

Source : https://pubmed.ncbi.nlm.nih.gov/36403297/


Conclusions: These results suggest the usefulness of MEP analysis of muscle biopsies in diagnosing immune-mediated MG in seronegative patients with myasthenia-like symptoms but, due to the invasiveness of the muscle biopsy procedure, clustered AChR Abs should, if possible, be tested first.

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argenx

Sponsored

VYVGART® (efgartigimod alfa-fcab) offers a unique approach in the treatment of adults with anti-AChR antibody positive generalized myasthenia gravis (gMG).1,2 Learn more at VYVGARTHCP.com.
 

MOAvidDesktopMOAvidMobile

AChR=acetylcholine receptor.
References: 1. VYVGART. Prescribing information. argenx US Inc; 2022. 2. Wolfe GI et al. J Neurol Sci. 2021;430:118074. doi:10.1016/j.jns.2021.118074

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IMPORTANT SAFETY INFORMATION AND INDICATION—WARNINGS AND PRECAUTIONS 

INDICATION
VYVGART® (efgartigimod alfa-fcab) is indicated for the treatment of generalized myasthenia gravis in adult patients
who are anti-acetylcholine receptor (AChR) antibody positive.

Infection
VYVGART may increase the risk of infection. The most common infections observed in Study 1 were urinary tract
infection (10% for VYVGART vs 5% for placebo) and respiratory tract infection (33% for VYVGART vs 29% for
placebo). Patients on VYVGART vs placebo had below normal levels for white blood cell counts (12% vs 5%,
respectively), lymphocyte counts (28% vs 19%, respectively), and neutrophil counts (13% vs 6%, respectively). The
majority of infections and hematologic abnormalities were mild to moderate in severity. Delay VYVGART
administration in patients with an active infection until the infection is resolved; monitor for clinical signs and
symptoms of infections. If serious infection occurs, administer appropriate treatment and consider withholding
VYVGART until the infection has resolved.

Immunization
Immunization with vaccines during VYVGART treatment has not been studied; the safety with live or live-attenuated
vaccines and the response to immunization with any vaccine are unknown. Because VYVGART causes a
reduction in immunoglobulin G (IgG) levels, vaccination with live-attenuated or live vaccines is not recommended
during VYVGART treatment. Evaluate the need to administer age-appropriate vaccines according to immunization
guidelines before initiation of a new treatment cycle with VYVGART.

Hypersensitivity Reactions
Hypersensitivity reactions, including rash, angioedema, and dyspnea, were observed with VYVGART. In clinical
trials, hypersensitivity reactions were mild or moderate, occurred within 1 hour to 3 weeks of administration, and
did not lead to treatment discontinuation. Monitor patients during administration and for 1 hour thereafter for
clinical signs and symptoms of hypersensitivity reactions. If a hypersensitivity reaction occurs during
administration, discontinue VYVGART infusion and institute appropriate supportive measures if needed.

ADVERSE REACTIONS
The most common (≥10%) adverse reactions with VYVGART were respiratory tract infection, headache, and urinary
tract infection.

USE IN SPECIFIC POPULATIONS
Pregnancy
As VYVGART is expected to reduce maternal IgG antibody levels, reduction in passive protection to the newborn is
anticipated. Risks and benefits should be considered prior to administering live or live-attenuated vaccines to
infants exposed to VYVGART in utero.

Lactation
There is no information regarding the presence of VYVGART in human milk, the effects on the breastfed infant, or
the effects on milk production. The developmental and health benefits of breastfeeding should be considered
along with the mother’s clinical need for VYVGART and any potential adverse effects on the breastfed infant from
VYVGART or from the underlying maternal condition.

Please see the full Prescribing Information.

You may report side effects to the US Food and Drug Administration by visiting http://www.fda.gov/medwatch or
calling 1-800-FDA-1088. You may also report side effects to argenx US, Inc, at 1-833-argx411 (1-833-274-9411).

Argenx Logo

VYVGART is a registered trademark of argenx. © 2022 argenx US-VYV-22-00242v1 10/2022. All Rights Reserved. For US audiences only.

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VYVGART® (efgartigimod alfa-fcab): a unique approach in the treatment of adults with anti-AChR antibody positive generalized myasthenia gravis (gMG).1,2

VYVGART is the Fc portion of an IgG antibody,* engineered for affinity to FcRn.1-3

AChR autoantibodies exert a direct pathogenic effect and disrupt neurotransmission in gMG in 3 ways: binding to AChR and creating a functional blockade; cross-linking of AChR, leading to internalization and degradation; and activating the complement system.4-8

FcRn binds IgG antibodies, preventing them from being destroyed in the lysosome. In doing so, FcRn helps maintain high levels of circulating IgG antibodies, including AChR autoantibodies.4,5,8,9

VYVGART competes with IgG antibodies, including AChR autoantibodies, in binding to and blocking FcRn.1,2 Unbound IgG antibodies and AChR autoantibodies are then destroyed in the lysosome, which results in their clearance.1,4,5,9

The most common adverse reactions observed in the clinical trial for VYVGART (n=84) vs placebo (n=83) were respiratory tract infection (33% vs 29%), headache (32% vs 29%), urinary tract infection (10% vs 5%), paraesthesia (7% vs 5%), and myalgia (6% vs 1%), respectively.1

See the full safety profile

By binding to and blocking FcRn, VYVGART helps clear circulating IgG antibodies, including AChR autoantibodies, that cause NMJ damage and dysfunction.1,10,11

Explore the mechanism of action for VYVGART

See clinical trial data for VYVGART

*Human IgG-derived.
AChR=acetylcholine receptor; Fc=fragment, crystallized; FcRn=neonatal Fc receptor; IgG=immunoglobulin G;
NMJ=neuromuscular junction.

 

Expand +

INDICATION AND IMPORTANT SAFETY INFORMATION
INDICATION
VYVGART® (efgartigimod alfa-fcab) is indicated for the treatment of generalized myasthenia gravis in adult patients
who are anti-acetylcholine receptor (AChR) antibody positive.
IMPORTANT SAFETY INFORMATION
WARNINGS AND PRECAUTIONS
Infection
VYVGART may increase the risk of infection. The most common infections observed in Study 1 were urinary tract infection (10% for VYVGART vs 5% for placebo) and respiratory tract infection (33% for VYVGART vs 29% for placebo). Patients on VYVGART vs placebo had below normal levels for white blood cell counts (12% vs 5%, respectively), lymphocyte counts (28% vs 19%, respectively), and neutrophil counts (13% vs 6%, respectively). The majority of infections and hematologic abnormalities were mild to moderate in severity. Delay VYVGART administration in patients with an active infection until the infection is resolved; monitor for clinical signs and symptoms of infections. If serious infection occurs, administer appropriate treatment and consider withholding VYVGART until the infection has resolved.

Immunization
Immunization with vaccines during VYVGART treatment has not been studied; the safety with live or live-attenuated vaccines and the response to immunization with any vaccine are unknown. Because VYVGART causes a reduction in immunoglobulin G (IgG) levels, vaccination with live-attenuated or live vaccines is not recommended during VYVGART treatment. Evaluate the need to administer age-appropriate vaccines according to immunization guidelines before initiation of a new treatment cycle with VYVGART.

Hypersensitivity Reactions
Hypersensitivity reactions, including rash, angioedema, and dyspnea, were observed with VYVGART. In clinical trials, hypersensitivity reactions were mild or moderate, occurred within 1 hour to 3 weeks of administration, and did not lead to treatment discontinuation. Monitor patients during administration and for 1 hour thereafter for clinical signs and symptoms of hypersensitivity reactions. If a hypersensitivity reaction occurs during administration, discontinue VYVGART infusion and institute appropriate supportive measures if needed.

ADVERSE REACTIONS
The most common (≥10%) adverse reactions with VYVGART were respiratory tract infection, headache, and urinary tract infection.

USE IN SPECIFIC POPULATIONS
Pregnancy
As VYVGART is expected to reduce maternal IgG antibody levels, reduction in passive protection to the newborn is anticipated. Risks and benefits should be considered prior to administering live or live-attenuated vaccines to infants exposed to VYVGART in utero.

Lactation
There is no information regarding the presence of VYVGART in human milk, the effects on the breastfed infant, or the effects on milk production. The developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for VYVGART and any potential adverse effects on the breastfed infant from VYVGART or from the underlying maternal condition.

Please see the full Prescribing Information.

You may report side effects to the US Food and Drug Administration by visiting http://www.fda.gov/medwatch or
calling 1-800-FDA-1088. You may also report side effects to argenx US, Inc, at 1-833-argx411 (1-833-274-9411).

Argenx Logo

VYVGART is a registered trademark of argenx.
© 2022 argenx US-VYV-22-00240v1 10/2022.
All Rights Reserved. For US audiences only.

References: 1. VYVGART. Prescribing information. argenx US Inc; 2022. 2. Wolfe GI et al. J Neurol Sci. 2021;430:118074.
doi:10.1016/j.jns.2021.118074 3. Howard JF et al. Lancet Neurol. 2021;20(7):526-536. doi:10.1016/S1474-4422(21)00159-9 4.
Roopenian DC, Akilesh S. Nat Rev Immunol. 2007;7(9):715-725. doi:10.1038/nri2155 5. Ward ES, Ober RJ. Trends Pharmacol Sci.
2018;39(10):892-904. doi:10.1016/j.tips.2018.07.007 6. Gilhus NE et al. Nat Rev Neurol. 2016;12(5):259-268.
doi:10.1038/nrneurol.2016.44 7. Huijbers MG et al. J Intern Med. 2014;275(1):12-26. doi:10.1111/joim.12163 8. Mantegazza R
et al. Neuropsychiatr Dis Treat. 2011;7:151-160. doi:10.2147/NDT.S8915 9. Ulrichts P et al. J Clin Invest. 2018;128(10):4372-
4386. doi:10.1172/JCI97911 10. Koneczny I, Herbst R. Cells. 2019;8(7):671. doi:10.3390/cells8070671 11. Howard JF Jr et al.
Neurology. 2019;92(23):e2661-e2673. doi:10.1212/WNL.0000000000007600

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Clinical pitfalls and serological diagnostics of MuSK myasthenia gravis - Journal of Neurology

Background We aimed to evaluate the diagnostic accuracy of enzyme-linked immunosorbent assay (ELISA) for anti-muscle specific tyrosine kinase (MuSK) antibody (Ab) in a large cohort of anti-acetylcholine receptor (AChR) Ab-negative ..... see more

Source : https://link.springer.com/article/10.1007/s00415-022-11458-4

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miRNAs as the important regulators of myasthenia gravis: involvement of major cytokines and immune cells - Immunologic Research

Myasthenia gravis (MG) is a type of muscle paralysis created by immune responses against acetylcholine receptor proteins in neuromuscular synapses. This disease is characterized by muscle weakness, especially ocular weakness ..... see more

Source : https://link.springer.com/article/10.1007/s12026-022-09342-4


Relevance: This review was aimed at providing an overview of the critical regulatory roles of various miRNAs in the pathogenesis of this autoimmune disease focusing on human MG studies and the interaction between different miRNAs with important cytokines and immune cells during the development of this autoimmune disease.


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CD59 Expression in Skeletal Muscles and Its Role in Myasthenia Gravis

Background and Objectives Complement regulatory proteins at the neuromuscular junction (NMJ) could offer protection against complement-mediated damage in myasthenia gravis (MG). However, there is limited information on their expression at ..... see more

Source : https://nn.neurology.org/content/10/1/e200057


Discussion: CD59, which acts as a complement regulator, may protect the NMJ from complement attack. Our findings could provide a basis for further research that investigates the underlying pathogenesis in MG and the immunomodulating interactions of the muscle cells.

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Developing and validating a nomogram to predict myasthenia gravis exacerbation in patients with postoperative thymoma recurrence

Thymoma is a rare and potentially malignant tumor, and complete surgical resection is currently recognized globally as the first choice of treatment ( 1). Nevertheless, 10-30% of patients may have ..... see more

Source : https://gs.amegroups.com/article/view/103515/html


Conclusions: We identified the risk factors for MG exacerbation in patients with postoperative recurrence of thymoma and drew a nomogram, which can be used to calculate the probability of MG exacerbation and guide clinicians to choose post-operative treatment.


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Diplopia: characteristics and etiologic distribution in a referral-based university hospital - Journal of Neurology

Background and objectives The etiologic distribution and clinical features of diplopia may differ according to the specialties involved in the management. This study aimed to establish the clinical features and ..... see more

Source : https://link.springer.com/article/10.1007/s00415-022-11471-7



Conclusions: "Conclusions: Given the differences in the etiologic distribution of diplopia according to the patients’ age and the specialties involved in the management, the results of previous reports on the characteristics and etiology of diplopia, primarily performed in a single specialty department, should be interpreted with a possible...

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Granulomatous myopathy: Sarcoidosis and beyond - PubMed

Non-necrotizing granulomatous inflammation is a rare but easily recognized histopathological finding in skeletal muscle biopsy. A limited number of diseases are known to be associated with non-necrotizing granulomatous myopathy. Once ..... see more

Source : https://pubmed.ncbi.nlm.nih.gov/36352751/


Conclusions: In the appropriate clinical context, exposure to immune checkpoint inhibitors and chronic graft-vs-host disease can be causes of granulomatous myopathy. In cases of unexplained granulomatous myopathy, natural killer/T-cell lymphoma should be considered and careful histopathological examination for atypical cells and appropriate...

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Metalloproteinases and Tissue Inhibitors in Generalized Myasthenia Gravis. A Preliminary Study - PubMed

1 Department of Biomedicine, Neuroscience and Advanced Diagnostic (BIND), University of Palermo, 90127 Palermo, Italy. 2 Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University ..... see more

Source : https://pubmed.ncbi.nlm.nih.gov/36358365/


Discussion: Our preliminary findings suggest that MMPs and TIMPs could play a role in the pathogenesis of MG and might be associated with the risk of clinical deterioration.

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Recap of the 2022 American Academy of Neurology Fall Conference

The American Academy of Neurology (AAN) 2022 Fall Conference featured the latest advances in diagnosing and treating dementia, epilepsy, movement disorders, myasthenia gravis, and other brain disorders. The three-day hybrid AAN Fall Conference took place at Caesars Palace in Las Vegas from Oct. 28 to 30, 2022. The digital on-demand content will be available in the Online Learning Center from Nov. 14, 2022, until Aug. 1, 2023.

Click above for a wrap-up of all the conference highlights.

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Impact of the COVID-19 pandemic on Patients with Myasthenia Gravis: A survey of the Myasthenia Gravis Foundation of America MG Patient Registry - PubMed

COVID-19 affected MG patients increasingly through the early pandemic. While face-to-face contact with a COVID-19 infected individual was an obvious risk factor, MG patients who had more people in the ..... see more

Source : https://pubmed.ncbi.nlm.nih.gov/36324261/


Discussion: COVID-19 affected MG patients increasingly through the early pandemic. While face-to-face contact with a COVID-19 infected individual was an obvious risk factor, MG patients who had more people in the household and unstable disease were at elevated risk for COVID-19 infection.


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Conclusions: The developed models succeeded to predict the proposed drugs in their tertiary combinations and spiked human plasma with satisfied precision, accuracy, and good greenness behavior.

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The best and worst of times in therapy development for myasthenia gravis - PubMed

Within the last 5 years the U.S. Federal Drug Administration (FDA) has approved complement and neonatal Fc receptor (FcRN)-inhibitors for treatment of generalized myasthenia gravis; and several other therapies are ..... see more

Source : https://pubmed.ncbi.nlm.nih.gov/36321730/


Relevance: The authors provide a summary of these discussions, but not a consensus opinion, and offer a series of recommendations to guide focused research in the most critical areas. We welcome ongoing discussion through comments on this publication.


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A Heterozygous Mutation in MFF Associated with a Mild Mitochondrial Phenotype

You are viewing a javascript disabled version of the site. Please enable Javascript for this site to function properly. Go to header Go to navigation Go to search Go to ..... see more

Source : https://content.iospress.com/articles/journal-of-neuromuscular-diseases/jnd221532


Background:The number of mutations in nuclear encoded genes causing mitochondrial disease is ever increasing. Identification of these mutations is particularly important in the diagnosis of neuromuscular disorders as their presentation may mimic other acquired disorders.


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Day 2 & 3 Highlights from the 2022 American Academy of Neurology Fall Conference

The American Academy of Neurology (AAN) 2022 Fall Conference featured the latest on diagnosing and treating dementia, epilepsy, movement disorders, headache, and other brain disorders, with content developed by neurologists for neurologists. The three-day hybrid AAN Fall Conference included in-person-only preconference programming for advanced practice providers plus a sports concussion workshop for general neurologists and subspecialists as well as anyone who treats sports injuries – specifically concussions – in their practice.

Click above for a summary of conference highlights.

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Conclusions: This study provides both a mechanistic understanding of MuSK MG relapse and a valuable candidate biomarker for relapse prediction.