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Myasthenia Gravis

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Rituximab in Late-Onset Myasthenia Gravis is Safe and Effective.

Supreet K Sahai, A Hadi Maghzi, Richard A Lewis,

INTRODUCTION:Late-onset myasthenia gravis (LOMG; onset >50 years) has different therapeutic decision-making challenges than MG in younger patients. METHODS:This is a retrospective series of seven patients with acetylcholine receptor antibody-positive MG, treated with rituximab. RESULTS:The mean age of onset was 66 years. Three patients were non-responders to previous therapy and six had developed ... Read more >>

Muscle Nerve (Muscle & nerve)
[2020, :]

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LncRNA XLOC_003810 promotes T-cell activation and inhibits PD-1/PD-L1 expression in patients with myasthenia gravis related thymoma.

Bo Hu, Li Niu, Zheng Jiang, Shenglin Xu, Yong Hu, Kun Cao,

This study aimed to investigate the effect of long non-coding RNA XLOC_003810 on the activation of CD4+ T cells and expression of PD-1/PD-L1 in patients with myasthenia gravis related thymoma (MG-T). Thymus specimens and thymic mononuclear cells were obtained from MG and MG-T patients or cardiac surgery patients undergoing thoracotomy ... Read more >>

Scand. J. Immunol. (Scandinavian journal of immunology)
[2020, :]

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Fingolimod (FTY720) improves postoperative cognitive dysfunction in mice subjected to D-galactose-induced aging.

Jie Zhang, Bin Xiao, Chen-Xu Li, Yi Wang,

Neurocognitive dysfunction is a common postoperative complication, especially in older adult patients. Fingolimod (FTY720) is a sphingosine-1-phosphate receptor modulator that has been found to be neuroprotective in several animal models of central nervous system disease. However, few reports have examined whether FTY720 could mitigate postoperative cognitive dysfunction. In this study, ... Read more >>

Neural Regen Res (Neural regeneration research)
[2020, 15(7):1308-1315]

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Case report: cardiac herniation following robotic-assisted thymectomy.

John Espey, Stephen Acosta, Lavinia Kolarczyk, Jason Long,

BACKGROUND:The first reported case of cardiac herniation was in 1948 and occurred following pericardiectomy during a lung cancer resection. Although rare, this potentially fatal surgical complication may occur following any operation in which a pericardial incision or resection is performed. The majority of literature on cardiac herniation involves case reports ... Read more >>

J Cardiothorac Surg (Journal of cardiothoracic surgery)
[2020, 15(1):54]

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Reply to Finsterer.

Feng Li, Andreas Meisel, Jens-C Rueckert,

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2020, 57(4):814-815]

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Case report: cardiac herniation following robotic-assisted thymectomy.

John Espey, Stephen Acosta, Lavinia Kolarczyk, Jason Long,

BackgroundThe first reported case of cardiac herniation was in 1948 and occurred following pericardiectomy during a lung cancer resection. Although rare, this potentially fatal surgical complication may occur following any operation in which a pericardial incision or resection is performed. The majority of literature on cardiac herniation involves case reports ... Read more >>

J Cardiothorac Surg (Journal of cardiothoracic surgery)
[2020, 15:]

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Repetitive ocular vestibular evoked myogenic potentials in myasthenia gravis.

Robert H P de Meel, Kevin R Keene, Magdalena A Wirth, Konrad P Weber, Umesh A Badrising, Jan J Verschuuren, Martijn R Tannemaat,

OBJECTIVE:To validate the repetitive ocular vestibular evoked myogenic potentials (RoVEMP) test for diagnostic use in myasthenia gravis (MG) and to investigate its value in diagnostically challenging subgroups. METHODS:The RoVEMP test was performed in 92 patients with MG, 22 healthy controls, 33 patients with a neuromuscular disease other than MG (neuromuscular ... Read more >>

Neurology (Neurology)
[2020, :]

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Teaching Video NeuroImages: Useful bedside testing for myasthenia gravis: The phenomenon of enhanced ptosis.

Euripedes Gomes de Carvalho Neto, Matheus Ferreira Gomes, Iuri Pereira Dos Santos, Mateus Damiani Monteiro, Ana Maria Hoppe, Francisco Tellechea Rotta,

Neurology (Neurology)
[2020, :]

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Practice advisory: Thymectomy for myasthenia gravis (practice parameter update): Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology.

Gary S Gronseth, Richard Barohn, Pushpa Narayanaswami,

OBJECTIVE:To review updated evidence regarding the effectiveness of thymectomy for treating patients with myasthenia gravis (MG). METHODS:The practice advisory panel performed a systematic review and developed practice recommendations using methods developed by the American Academy of Neurology. RESULTS:One Class I study of patients younger than 65 years with nonthymomatous acetylcholine ... Read more >>

Neurology (Neurology)
[2020, :]

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Suitable indications of eculizumab for patients with refractory generalized myasthenia gravis.

Munenori Oyama, Kensuke Okada, Masayuki Masuda, Yuko Shimizu, Kazumasa Yokoyama, Akiyuki Uzawa, Naoki Kawaguchi, Ryotaro Ikeguchi, Yasunobu Hoshino, Taku Hatano, Yukiko Ozawa, Jin Nakahara, Hitoshi Aizawa, Kazuo Kitagawa, Nobutaka Hattori, Satoshi Kuwabara, Hiroyuki Murai, Shigeaki Suzuki,

Background:Eculizumab is a humanized monoclonal antibody that targets complement protein C5 and inhibits terminal complement-mediated damage at the neuromuscular junction. Recently, the REGAIN study showed that eculizumab was effective and well tolerated in patients with anti-acetylcholine receptor antibody-positive refractory generalized myasthenia gravis (gMG). However, there is no consensus regarding which ... Read more >>

Ther Adv Neurol Disord (Therapeutic advances in neurological disorders)
[2020, 13:1756286420904207]

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Guidance for the management of myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) during the COVID-19 pandemic.

S. Jacob, S. Muppidi, A. Guidon, J. Guptill, M. Hehir, J.F. Howard, I. Illa, R. Mantegazza, H. Murai, K. Utsugisawa, J. Vissing, H. Wiendl, R.J. Nowak,

J. Neurol. Sci. (Journal of the neurological sciences)
[0, :]

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MuSK EAMG: Immunological Characterization and Suppression by Induction of Oral Tolerance.

Debby Reuveni, Revital Aricha, Miriam C. Souroujon, Sara Fuchs,

Myasthenia gravis (MG) with antibodies to the muscle-specific receptor tyrosine kinase (MuSK) is a distinct sub-group of MG, affecting 5–8% of all MG patients. MuSK, a receptor tyrosine kinase, is expressed at the neuromuscular junctions (NMJs) from the earliest stages of synaptogenesis and plays a crucial role in the development ... Read more >>

Front Immunol (Frontiers in immunology)
[2020, 11:]

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Surgical effect and prognostic factors of myasthenia gravis with thymomas.

Wenxin Tian, Xiao Li, Hongfeng Tong, Wenhan Weng, Fan Yang, Guanchao Jiang, Jun Wang,

BACKGROUND:To evaluate the surgical effect and prognostic factors of extended thymectomy for myasthenia gravis (MG) patients with thymomas. METHODS:Patients with MG with thymomas who underwent extended thymectomy at Peking University People's Hospital and Beijing Hospital between January 2010 and December 2018 were retrospectively enrolled. Patients were followed up by telephone ... Read more >>

Thorac Cancer (Thoracic cancer)
[2020, :]

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AGRN Gene Mutation Leads to Congenital Myasthenia Syndromes: A Pediatric Case Report and Literature Review.

Siyi Gan, Haiyan Yang, Ting Xiao, Zou Pan, Liwen Wu,

The congenital myasthenia syndromes (CMS) are a group of autosomal recessive or autosomal dominant diseases that affect neuromuscular junctions. CMS caused by AGRN mutations is very uncommon typically characterized by ptosis, mild weakness, and proximal limb weakness. We report the case of an 8-year-old female who exhibited the onset of ... Read more >>

Neuropediatrics (Neuropediatrics)
[2020, :]

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Measuring parent proxy-reported quality of life of 11 rare diseases in children in Zhejiang, China

Qisheng Gao, Shanshan Wang, Jianping Ren, Xin Wen,

<title>Abstract</title> <p> Background It is increasingly important to measure the health-related quality of life (HRQoL) of rare disease in children and adolescents in recent decades. Much attention has been paid to investigate the HROoL of one specific rare disease by self-report in previous studies. This study aimed to ... Read more >>

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Pembrolizumab treatment of metastatic urothelial cancer without exacerbating myasthenia gravis.

Akiko Ishii, Minato Yokoyama, Hiroshi Tsuji, Yasuhisa Fujii, Akira Tamaoka,

Pembrolizumab is an immune checkpoint inhibitor (ICI) that targets the programmed cell death (PD)-1 receptor. It significantly increases the overall survival in patients with locally advanced or metastatic urothelial cancer. However, its administration may induce serious immune-related adverse effects, such as myocarditis and myasthenia gravis (MG). Therefore, ICI treatment may ... Read more >>

eNeurologicalSci (eNeurologicalSci)
[2020, 19:100236]

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Celebrating the 70 years of pyridostigmine on therapy of Myasthenia Gravis: historical aspects of the preliminary trials.

Paulo José Lorenzoni, Cláudia Suemi Kamoi Kay, Renata Dal-Prá Ducci, Otto Jesus Hernandez Fustes, Lineu Cesar Werneck, Rosana Herminia Scola,

Currently, pyridostigmine bromide is an indispensable anticholinesterase agent used worldwide to treat patients with Myasthenia Gravis (MG). However, pyridostigmine bromide was unsuccessful in its "pioneering trials" to treat a series of MG patients. There are important historical landmarks before pyridostigmine bromide becomes useful, safe and indispensable for MG therapy. After ... Read more >>

Arq Neuropsiquiatr (Arquivos de neuro-psiquiatria)
[2020, :]

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Comprehensive meta-analysis reveals an association of the HLA-DRB1*1602 allele with autoimmune diseases mediated predominantly by autoantibodies.

Yan Chen, Shasha Li, Renliang Huang, Zhongjian Zhang, Frank Petersen, Junfeng Zheng, Xinhua Yu,

The human leukocytes antigen (HLA)-DRB1*16:02 allele has been suggested to be associated with many autoimmune diseases. However, a validation of the results of the different studies by a comprehensive analysis of the corresponding meta data is lacking. In this study, we performed a meta-analysis of the association between HLA-DRB1*16:02 allele ... Read more >>

Autoimmun Rev (Autoimmunity reviews)
[2020, :102532]

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Myasthenia gravis AChR antibodies inhibit function of rapsyn-clustered AChRs.

Hakan Cetin, Richard Webster, Wei Wei Liu, Akiko Nagaishi, Inga Koneczny, Fritz Zimprich, Susan Maxwell, Judith Cossins, David Beeson, Angela Vincent,

OBJECTIVE:Direct inhibition of acetylcholine receptor (AChR) function by autoantibodies (Abs) is considered a rare pathogenic mechanism in myasthenia gravis (MG), but is usually studied on AChRs expressed in cell lines, rather than tightly clustered by the intracellular scaffolding protein, rapsyn, as at the intact neuromuscular junction. We hypothesised that clustered ... Read more >>

J. Neurol. Neurosurg. Psychiatry (Journal of neurology, neurosurgery, and psychiatry)
[2020, :]

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Population Pharmacokinetic Analysis of Tacrolimus in Adult Chinese Patients with Myasthenia Gravis: A Prospective Study.

Jue Liu, Yun-Peng Guo, Zheng Jiao, Chong-Bo Zhao, Hui Wu, Zi-Ran Li, Xiao-Jun Cai, Jian-Ying Xi,

BACKGROUND AND OBJECTIVES:Tacrolimus is a widely used immunosuppressive agent with narrow therapeutic window. Nowadays, tacrolimus has gained acceptance as a therapeutic option in myasthenia gravis (MG) treament, however, little is known about its pharmacokinetic characteristics in MG population. In this study, we aimed to investigate the population pharmacokinetic (PopPK) of tacrolimus in patients with MG and to develop model-informed dosing regimens. METHODS:Trough concentrations ... Read more >>

Eur J Drug Metab Pharmacokinet (European journal of drug metabolism and pharmacokinetics)
[2020, :]

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New insights into very-late-onset myasthenia gravis.

Carolina Barnett, Vera Bril,

Nat Rev Neurol (Nature reviews. Neurology)
[2020, :]

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Neurologic complications of immune checkpoint inhibitors.

Alexandra M Haugh, John C Probasco, Douglas B Johnson,

Introduction: Immune checkpoint inhibitors (ICI) are associated with a wide spectrum of neurologic immune-related adverse events (irAEs) including meningo-encephalitis, myasthenia gravis and various neuropathies. Although relatively rare, they often present significant diagnostic complexity and may be under-recognized. Permanent neurologic deficits and/or fatality have been described but improvement is noted in ... Read more >>

Expert Opin Drug Saf (Expert opinion on drug safety)
[2020, :1-10]

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Self-antigen driven affinity maturation is required for pathogenic monovalent IgG4 autoantibody development

Miriam Fichtner, Casey Vieni, Rachel Redler, Ljuvica Kolich, Ruoyi Jiang, Kazushiro Takata, Panos Stathopoulos, Pablo Suarez, Richard Nowak, Steven Burden, Damian Ekiert, Kevin O’Connor,

Abstract Pathogenic IgG4 autoantibodies in autoimmune myasthenia gravis (MG) are functionally monovalent as a result of Fab-arm exchange. The origin and development of these unique autoantibodies are not well understood. We examined MG patient-derived monoclonal autoantibodies (mAbs), their corresponding germline-encoded unmutated common ancestors (UCA) and monovalent antigen-binding fragments (Fabs) to ... Read more >>

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Autophagy-deficiency in bone marrow mononuclear cells from patients with myasthenia gravis: a possible mechanism of pathogenesis.

Jingqun Tang, Ziming Ye, Yi Liu, Mengxiao Zhou, Liqiang Huang, Qin Mo, Xiaotao Su, Chao Qin,

Myasthenia gravis (MG) is a chronic autoimmune disorder resulting from autoantibodies against neuromuscular junction components. Research shows that this disease might be a primary bone marrow (BM) stem cell disorder. Autophagy protects the dynamics and homeostasis of the host cells by removing damaged mitochondria, protein aggregates and other intercellular materials. ... Read more >>

Int. J. Neurosci. (The International journal of neuroscience)
[2020, :1-15]

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Neutralizing antibodies against endogenous interferon in myasthenia gravis patients.

Francesca Bagnato, Alessandro Clemenzi, Carolina Scagnolari, Luigi Strizzi, Antonella Di Pasquale, Francesca Bellomi, Paola Di Marco, Guido Antonelli, Giovanni Antonini,

Anti-cytokine antibodies (Abs) play an important role in the regulation of the immune response, both under normal conditions and in several autoimmune and neoplastic disorders. In the present study, we have investigated the occurrence and the clinical significance of natural neutralizing Abs (NAbs) against interferons (IFNs) alpha, beta, and gamma, ... Read more >>

Eur. Cytokine Netw. (European cytokine network)
[2004, 15(1):24-29]

Cited: 8 times

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