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myasthenia gravis recognition

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https://www.readbyqxmd.com/read/29114250/a-sensitive-method-for-detecting-peptide-specific-cd4-t-cell-responses-in-peripheral-blood-from-patients-with-myasthenia-gravis
#1
Sapna Sharma, Clas Malmeström, Christopher Lindberg, Sarah Meisel, Karin Schön, Martina Verolin, Nils Yngve Lycke
Myasthenia gravis (MG) is an autoimmune neurological disorder typified by skeletal muscle fatigue and most often production of autoantibodies against the nicotinic acetylcholine receptor (AChR). The present study was undertaken to assess the extent of AChR-peptide recognition in MG patients using co-culturing (DC:TC) of autologous monocyte-derived dendritic cells (moDCs) and highly enriched CD4(+) T cells from the blood as compared to the traditional whole peripheral blood mononuclear cell (PBMC) cultures. We found that the DC:TC cultures were highly superior to the PBMC cultures for detection of reactivity toward HLA-DQ/DR-restricted AChR-peptides...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28938341/neurological-toxicities-associated-with-immune-checkpoint-inhibitors
#2
Mehdi Touat, Daniel Talmasov, Damien Ricard, Dimitri Psimaras
PURPOSE OF REVIEW: Immune-checkpoint inhibitors (ICIs) constitute a novel class of agents recently approved to treat a number of human malignancies. Due to their immunomodulatory mechanism of action, ICIs can generate a wide range of immune-related adverse events (irAEs) of which neurological toxicities are of special interest because of their potential severity. The objective of this review is to examine the recent literature describing neurological irAEs and discuss their optimal management...
December 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28821685/nivolumab-related-myasthenia-gravis-with-myositis-and-myocarditis-in-japan
#3
Shigeaki Suzuki, Nobuhisa Ishikawa, Fumie Konoeda, Nobuhiko Seki, Satoshi Fukushima, Kikuko Takahashi, Hisashi Uhara, Yoshikazu Hasegawa, Shinichiro Inomata, Yasushi Otani, Kenji Yokota, Takashi Hirose, Ryo Tanaka, Norihiro Suzuki, Makoto Matsui
OBJECTIVE: To report the clinical features of myasthenia gravis (MG) induced by treatment with immune checkpoint inhibitors using 2-year safety databases based on postmarketing surveys in Japan. METHODS: We studied 10,277 patients with cancer who had received monotherapy with either nivolumab or ipilimumab between September 2014 and August 2016. As the control group, 105 patients with idiopathic MG were used. RESULTS: There were 12 MG cases (0...
September 12, 2017: Neurology
https://www.readbyqxmd.com/read/28820310/orbital-mantle-cell-lymphoma-presenting-as-myasthenia-gravis
#4
Justin Karlin, Travis Peck, Karyn Prenshaw, Craig A Portell, Maria Kirzhner
A 69-year-old man, previously treated with pyridostigmine for myasthenia gravis (manifesting as ptosis and diplopia) was evaluated for several concomitant bilateral anterior orbital masses. Imaging revealed 3 discrete, solid masses within and around the orbits. An incisional biopsy demonstrated atypical lymphocytes positive for CD20 and Cyclin-D1, consistent with mantle cell lymphoma. The patient received induction chemotherapy with a rituximab-based regimen. He experienced resolution of his diplopia and ptosis after one cycle of chemotherapy and achieved complete remission of the orbital masses and myasthenia symptoms after 6 cycles...
August 18, 2017: Orbit
https://www.readbyqxmd.com/read/28666240/myasthenia-gravis-an-emerging-toxicity-of-immune-checkpoint-inhibitors
#5
REVIEW
D Makarious, K Horwood, J I G Coward
The advent of immunotherapy has heralded a number of significant advances in the treatment of particular malignancies associated with poor prognosis (melanoma, non-small-cell lung, renal and head/neck cancers). The success witnessed with therapeutic agents targeting cytotoxic T-lymphocyte-associated protein 4, programmed cell death protein 1 and programmed cell death ligand 1 immune checkpoints has inevitably led to an explosion in their clinical application and the subsequent recognition of specific toxicity profiles distinct from those long recognised with chemotherapy...
September 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28446137/progressive-cutaneous-cryptococcosis-complicated-with-meningitis-in-a-myasthenia-gravis-patient-on-long-term-immunosuppressive-therapy-a-case-report
#6
Nguyen Thi Cam Huong, Ahmed M A Altibi, Nguyen My Hoa, Le Anh Tuan, Samar Salman, Sara Morsy, Nguyen Thi Bich Lien, Nguyen Thanh Truong, Nguyen Thi Hoang Mai, Pham Thi Le Hoa, Nguyen Ba Thang, Van The Trung
BACKGROUND: Cryptococcosis is an opportunistic infection caused by the encapsulated yeast Cryptococcus neoformans and most remarkably manifests in HIV-infected individuals, especially in the settings of very low CD4 count. Development of cryptococcosis in HIV-uninfected individuals is exceedingly rare and usually signifies a marked immunodeficiency. Cryptococcosis in association with myasthenia gravis or thymoma has been previously documented in only very few cases in the literature. CASE PRESENTATION: We reported a complicated case of severe cutaneous cryptococcosis in a 39-year-old Vietnamese male patient with myasthenia gravis on long-term immunosuppressive therapy...
April 26, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28414153/inflammatory-myopathy-associated-with-myasthenia-gravis-with-and-without-thymic-pathology-report-of-four-cases-and-literature-review
#7
Ernestina Santos, Ester Coutinho, Ana Martins da Silva, António Marinho, Carlos Vasconcelos, Ricardo Taipa, Manuel Melo Pires, Guilherme Gonçalves, Carlos Lopes, Maria Isabel Leite
INTRODUCTION: The association of myasthenia gravis (MG) and inflammatory myopathy is rare and often only one of the diseases is diagnosed. Thymus pathology may be in the origin of such disease association. METHODS: We described four patients with both MG and inflammatory myopathy. RESULTS: These cases correspond to 2.3% of our MG cohort. Case 1: MG, polymyositis and thymolipoma; case 2: MG and necrotizing myopathy without thymic pathology on a background of scleroderma, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia (CREST); case 3: MG and dermatomyositis without thymic pathology; case 4: MG and dermatomyositis with type C thymoma...
June 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28217268/anti-musk-positive-myasthenia-gravis-and-three-semiological-cardinal-signs
#8
André P C Matta, Ana C Andorinho F Ferreira, Arielle Kirmse, Anna Carolina Damm, João Gabriel D I B Farinhas, Mariane D Barbosa, Mayara C M Teles, Camila Fiorelli, Rossano Fiorelli, Osvaldo J M Nascimento, Marco Orsini
Myasthenia gravis (MG) is a relatively uncommon disorder with an annual incidence of approximately 7 to 9 new cases per million. The prevalence is about 70 to 165 per million. The prevalence of the disease has been increasing over the past five decades. This is thought to be due to better recognition of the condition, aging of the population, and the longer life span of affected patients. MG causes weakness, predominantly in bulbar, facial, and extra-ocular muscles, often fluctuating over minutes to weeks, in the absence of wasting, sensory loss, or reflex changes...
November 2, 2016: Neurology International
https://www.readbyqxmd.com/read/28147928/pembrolizumab-induced-myasthenia-gravis-a-fatal-case-report
#9
Katherine L March, Michael J Samarin, Amik Sodhi, Ryan E Owens
Purpose Pembrolizumab, a monoclonal antibody which inhibits the programmed cell death 1 receptor, has been shown to efficaciously enhance pre-existing immune responses to malignancies. However, safety concerns must also be considered as pembrolizumab use has been associated with several life-threatening immune-related adverse events (irAEs). We report a fatal case of pembrolizumab-induced myasthenia gravis in a patient with no prior myasthenia gravis history. Case report A 63-year-old male presented with right eyelid drooping, puffiness, blurred vision, and shortness of breath two weeks after an initial infusion of pembrolizumab...
January 1, 2017: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/27931211/gonadectomy-effects-on-the-risk-of-immune-disorders-in-the-dog-a-retrospective-study
#10
Crystal R Sundburg, Janelle M Belanger, Danika L Bannasch, Thomas R Famula, Anita M Oberbauer
BACKGROUND: Gonadectomy is one of the most common procedures performed on dogs in the United States. Neutering has been shown to reduce the risk for some diseases although recent reports suggest increased prevalence for structural disorders and some neoplasias. The relation between neuter status and autoimmune diseases has not been explored. This study evaluated the prevalence and risk of atopic dermatitis (ATOP), autoimmune hemolytic anemia (AIHA), canine myasthenia gravis (CMG), colitis (COL), hypoadrenocorticism (ADD), hypothyroidism (HYPO), immune-mediated polyarthritis (IMPA), immune-mediated thrombocytopenia (ITP), inflammatory bowel disease (IBD), lupus erythematosus (LUP), and pemphigus complex (PEMC), for intact females, intact males, neutered females, and neutered males...
December 8, 2016: BMC Veterinary Research
https://www.readbyqxmd.com/read/27922503/myasthenia-gravis-and-lambert-eaton-myasthenic-syndrome
#11
Michael W Nicolle
PURPOSE OF REVIEW: This article discusses the pathogenesis, diagnosis, and management of autoimmune myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS). RECENT FINDINGS: Recognition of new antigenic targets and improved diagnostic methods promise to improve the diagnosis of MG, although the clinical phenotypes associated with newer antibodies have not yet been defined. Future therapies might specifically target the aberrant immune response. The apparent increase in the prevalence of MG is not fully explained...
December 2016: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/27907966/getting-rid-of-weakness-in-the-icu-an-updated-approach-to-the-acute-management-of-myasthenia-gravis-and-guillain-barr%C3%A3-syndrome
#12
Alexis A Lizarraga, Karlo J Lizarraga, Michael Benatar
After prompt diagnosis, severe myasthenia gravis and Guillain-Barré syndrome (GBS) usually require management in the intensive care unit. In the myasthenic patient, recognition of precipitating factors is paramount, and frequent monitoring of bulbar, upper airway, and/or respiratory muscle strength is needed to identify impending myasthenic crisis. Noninvasive ventilation can be attempted prior to intubation and mechanical ventilation in the setting of respiratory failure. Cholinesterase inhibitors should be discontinued, but resumed prior to extubation, and steroid dosage could be increased once the airway is secured...
December 2016: Seminars in Neurology
https://www.readbyqxmd.com/read/27704496/myasthenia-gravis
#13
Daniel B Drachman
The basic abnormality in myasthenia gravis (MG) is a reduction in acetylcholine receptors (AChRs) at neuromuscular junctions due to the effects of autoantibodies that are directed against the AChRs in most patients, or against neighboring proteins involved in the clustering of AChRs (MuSK, LRP-4, or agrin). Clinically, MG is characterized by muscle weakness and fatigue, often in typical patterns. The diagnosis may be missed early, and depends on the recognition of clinical manifestations, the measurement of autoantibodies, and/or electrophysiological features...
October 2016: Seminars in Neurology
https://www.readbyqxmd.com/read/27016366/modelling-the-response-to-low-frequency-repetitive-nerve-stimulation-of-myasthenia-gravis-and-lambert-eaton-myasthenic-syndrome
#14
Francesc Miralles
Myasthenia gravis (MG) is an autoimmune postsynaptic disorder of neuromuscular transmission caused, in most patients, by antibodies against postsynaptic acetylcholine receptors. Lambert-Eaton myasthenic syndrome (LEMS) is a presynaptic autoimmune disease in which there is a reduction in Ca(2+) entry with each impulse due to the action of antibodies against Ca(2+) channels. These diseases have a distinct pattern of response to low-frequency repetitive nerve stimulation which allows its recognition in a particular subject...
November 2016: Medical & Biological Engineering & Computing
https://www.readbyqxmd.com/read/26708556/myf5-and-myogenin-in-the-development-of-thymic-myoid-cells-implications-for-a-murine-in-vivo-model-of-myasthenia-gravis
#15
Bo Hu, Katja Simon-Keller, Stefan Küffer, Philipp Ströbel, Thomas Braun, Alexander Marx, Stefan Porubsky
Myasthenia gravis (MG) is caused by autoantibodies against the neuromuscular junction of striated muscle. Most MG patients have autoreactive T- and B-cells directed to the acetylcholine receptor (AChR). To achieve immunologic tolerance, developing thymocytes are normally eliminated after recognition of self-antigen-derived peptides. Presentation of muscle-specific antigens is likely achieved through two pathways: on medullary thymic epithelial cells and on medullary dendritic cells cross-presenting peptides derived from a unique population of thymic myoid cells (TMC)...
March 2016: Experimental Neurology
https://www.readbyqxmd.com/read/25635260/toxoplasmic-encephalitis-during-mycophenolate-mofetil-immunotherapy-of-neuromuscular-disease
#16
Danilo R Bernardo, Nizar Chahin
OBJECTIVE: To show that immunotherapy with medications such mycophenolate mofetil (MMF) can cause serious complications in patients with neuromuscular disorders. METHODS: Two patients with neuromuscular disorders on immunotherapy with long-term MMF who developed toxoplasmic encephalitis (TE) were included in this case series. RESULTS: One patient with myasthenia gravis and one patient with inflammatory myopathy on immunotherapy with long-term MMF developed severe TE...
February 2015: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/25634171/statins-and-myotoxic-effects-associated-with-anti-3-hydroxy-3-methylglutaryl-coenzyme-a-reductase-autoantibodies-an-observational-study-in-japan
#17
Yurika Watanabe, Shigeaki Suzuki, Hiroaki Nishimura, Ken-ya Murata, Takashi Kurashige, Masamichi Ikawa, Masaru Asahi, Hirofumi Konishi, Satsuki Mitsuma, Satoshi Kawabata, Norihiro Suzuki, Ichizo Nishino
Statins have a variety of myotoxic effects and can trigger the development of inflammatory myopathies or myasthenia gravis (MG) mediated by immunomodulatory properties. Autoantibodies to 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) have been identified in patients with statin-associated myopathy. The purpose of the present study is to develop an enzyme-linked immunosorbent assay (ELISA) of anti-HMGCR antibodies and to elucidate the clinical significance of anti-HMGCR antibodies in Japanese patients with inflammatory myopathies or MG...
January 2015: Medicine (Baltimore)
https://www.readbyqxmd.com/read/25566244/is-dosing-of-therapeutic-immunoglobulins-optimal-a-review-of-a-three-decade-long-debate-in-europe
#18
REVIEW
Jacqueline Kerr, Isabella Quinti, Martha Eibl, Helen Chapel, Peter J Späth, W A Carrock Sewell, Abdulgabar Salama, Ivo N van Schaik, Taco W Kuijpers, Hans-Hartmut Peter
The consumption of immunoglobulins (Ig) is increasing due to better recognition of antibody deficiencies, an aging population, and new indications. This review aims to examine the various dosing regimens and research developments in the established and in some of the relevant off-label indications in Europe. The background to the current regulatory settings in Europe is provided as a backdrop for the latest developments in primary and secondary immunodeficiencies and in immunomodulatory indications. In these heterogeneous areas, clinical trials encompassing different routes of administration, varying intervals, and infusion rates are paving the way toward more individualized therapy regimens...
2014: Frontiers in Immunology
https://www.readbyqxmd.com/read/25521402/development-of-the-international-thymic-malignancy-interest-group-international-database-an-unprecedented-resource-for-the-study-of-a-rare-group-of-tumors
#19
James Huang, Usman Ahmad, Alberto Antonicelli, Ann Christine Catlin, Wentao Fang, Daniel Gomez, Patrick Loehrer, Marco Lucchi, Edith Marom, Andrew Nicholson, Enrico Ruffini, William Travis, Paul Van Schil, Heather Wakelee, Xiaopan Yao, Frank Detterbeck
BACKGROUND: Our knowledge of thymic malignancies has largely been derived from small, single-institution series. Recognition of the need for broad collaboration led to the creation of the International Thymic Malignancy Interest Group (ITMIG) and the development of a large, centralized database to advance knowledge of these rare tumors. METHODS: A multidisciplinary Database Committee was convened to define a common set of data elements a priori. Retrospective data were solicited from ITMIG members and collated using standardized fields...
October 2014: Journal of Thoracic Oncology
https://www.readbyqxmd.com/read/25396312/paraneoplastic-disorders-in-thymoma-patients
#20
REVIEW
Amelia Evoli, Eric Lancaster
Thymic malignancy is often associated with paraneoplastic neurological diseases (PNDs) and recognition of these disorders is important for physicians who treat these patients. The most common thymoma-associated PNDs are myasthenia gravis (MG), acquired neuromyotonia (Isaacs' syndrome), encephalitis, Morvan's syndrome, and myositis. Diagnosis of these disorders is complex but often aided by testing for specific autoantibodies, including those to the acetylcholine receptor for MG and to contactin-associated protein-like 2, protein of the voltage-gated potassium channel complex, in patients with acquired neuromyotonia, Morvan's syndrome, or encephalitis...
September 2014: Journal of Thoracic Oncology
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