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Autoimmune myasthenia

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https://www.readbyqxmd.com/read/28801669/balance-between-estrogens-and-proinflammatory-cytokines-regulates-chemokine-production-involved-in-thymic-germinal-center-formation
#1
Nadine Dragin, Patrice Nancy, José Villegas, Régine Roussin, Rozen Le Panse, Sonia Berrih-Aknin
The early-onset form of Myasthenia Gravis (MG) is prevalent in women and associates with ectopic germinal centers (GCs) development and inflammation in the thymus. we aimed to investigate the contribution of estrogens in the molecular processes involved in thymic GCs formation. We examined expression of genes involved in anti-acetylcholine receptor (AChR) response in MG, MHC class II and α-AChR subunit as well as chemokines involved in GC development (CXCL13, CCL21and CXCL12). In resting conditions, estrogens have strong regulatory effects on thymic epithelial cells (TECs), inducing a decreased protein expression of the above molecules...
August 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28796013/electroconvulsive-therapy-for-depression-comorbid-with-myasthenia-gravis-a-case-report-and-review-of-the-literature
#2
Mark B Warren, Scott Elder, Norman P Litchfield
OBJECTIVES: Myasthenia gravis (MG) is a rare but well-described autoimmune disease, which is sometimes comorbid with psychiatric illness. There have been several case reports describing the use of electroconvulsive therapy (ECT) for the treatment of core psychopathology in the context of MG. We sought to review the available published data on ECT in MG and add another case example to the literature. METHODS: We performed a PubMed search for relevant articles or case reports in English describing ECT in MG and summarized findings...
August 9, 2017: Journal of ECT
https://www.readbyqxmd.com/read/28782655/on-the-role-of-igg4-in-inflammatory-conditions-lessons-for-igg4-related-disease
#3
REVIEW
David C Trampert, Lowiek M Hubers, Stan F J van de Graaf, Ulrich Beuers
The pathophysiology of immunoglobulin G4-related disease (IgG4-RD) and its most common manifestations, IgG4-associated (sclerosing) cholangitis and autoimmune pancreatitis, remains largely unknown, but IgG4 is presumably involved. IgG4 is a promiscuous antibody, which could be directly pathogenic, fulfill a protective role, or could just be a fortuitous marker of an aberrant inflammatory response. IgG4 antibodies possess exclusive structural and functional characteristics suggesting anti-inflammatory and tolerance-inducing effects...
August 3, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28780294/pathophysiology-and-immunological-profile-of-myasthenia-gravis-and-its-subgroups
#4
REVIEW
Fredrik Romi, Yu Hong, Nils Erik Gilhus
Myasthenia gravis (MG) is an autoimmune antibody-mediated disease characterized by muscle weakness and fatigability. It is believed that the initial steps triggering humoral immunity in MG take place inside thymic tissue and thymoma. The immune response against one or several epitopes expressed on thymic tissue cells spills over to neuromuscular junction components sharing the same epitope causing humoral autoimmunity and antibody production. The main cause of MG is acetylcholine receptor antibodies. However, many other neuromuscular junction membrane protein targets, intracellular and extracellular proteins are suggested to participate in MG pathophysiology...
August 3, 2017: Current Opinion in Immunology
https://www.readbyqxmd.com/read/28756524/muscle-satellite-cells-are-functionally-impaired-in-myasthenia-gravis-consequences-on-muscle-regeneration
#5
Mohamed Attia, Marie Maurer, Marieke Robinet, Fabien Le Grand, Elie Fadel, Rozen Le Panse, Gillian Butler-Browne, Sonia Berrih-Aknin
Myasthenia gravis (MG) is a neuromuscular disease caused in most cases by anti-acetyl-choline receptor (AChR) autoantibodies that impair neuromuscular signal transmission and affect skeletal muscle homeostasis. Myogenesis is carried out by muscle stem cells called satellite cells (SCs). However, myogenesis in MG had never been explored. The aim of this study was to characterise the functional properties of myasthenic SCs as well as their abilities in muscle regeneration. SCs were isolated from muscle biopsies of MG patients and age-matched controls...
July 29, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28751878/autoimmune-thyroiditis-and-myasthenia-gravis
#6
REVIEW
Angela Lopomo, Sonia Berrih-Aknin
Autoimmune diseases (AIDs) are the result of specific immune responses directed against structures of the self. In normal conditions, the molecules recognized as "self" are tolerated by immune system, but when the self-tolerance is lost, the immune system could react against molecules from the body, causing the loss of self-tolerance, and subsequently the onset of AID that differs for organ target and etiology. Autoimmune thyroid disease (ATD) is caused by the development of autoimmunity against thyroid antigens and comprises Hashimoto's thyroiditis and Graves disease...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28743581/caspase-1-inhibitor-regulates-humoral-responses-in-experimental-autoimmune-myasthenia-gravis-via-il-6-dependent-inhibiton-of-stat3
#7
Cong-Cong Wang, Min Zhang, Heng Li, Xiao-Li Li, Long-Tao Yue, Peng Zhang, Ru-Tao Liu, Hui Chen, Yan-Bin Li, Rui-Sheng Duan
We have previously demonstrated that Cysteinyl aspartate-specific proteinase-1 (caspase-1) inhibitor ameliorates experimental autoimmune myasthenia gravis (EAMG) by inhibited cellular immune response, via suppressing DC IL-1 β, CD4(+) T and γdT cells IL-17 pathways. In this study, we investigated the effect of caspase-1 inhibitor on humoral immune response of EAMG and further explore the underlying mechanisms. An animal model of MG was induced by region 97-116 of the rat AChR α subunit (R97-116 peptide) in Lewis rats...
July 22, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28731133/irrelevance-of-anti%C3%A2-nachr-cytoplasmic-loop-antibody-in-the-degree-of-myasthenia-gravis
#8
Ye Lin, Chen Song, Jiang Xu, Yongxiang Yang, Hang Qin, Cong Zhao, Jiaji Lin, Rui Liu, Zhuyi Li
Anti‑nicotinic acetylcholine receptor (nAChR) antibody in myasthenia gravis (MG) usually refers to that against the extracellular domain (ECD) of nAChR. However, growing evidence has indicated that there also exists the nAChR antibody against the nAChR cytoplasmic loop (CL) in patients with MG. Some studies have demonstrated that the anti‑CL antibody may play a protective role in animal models of experimental autoimmune MG. However, to date, limited or no information is available as to whether anti‑CL antibody plays a beneficial role in patients with MG...
July 19, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28729915/the-concomitant-association-of-thyroid-disorders-and-myasthenia-gravis
#9
Yu-Pei Lin, Usman Iqbal, Phung-Anh Nguyen, Md Mohaimenul Islam, Suleman Atique, Wen-Shan Jian, Yu-Chuan Jack Li, Chen-Ling Huang, Chung-Huei Hsu
BACKGROUND: Some of the thyroid disorders (TD) and Myasthenia gravis (MG) are autoimmune related disease. The purpose of the study to evaluate the relationship of MG with all morphological and functional thyroid disorders. METHODS: We constructed a population-based cohort study during the period from January 2000-December 2002 by using reimbursement data from the Bureau National Health Insurance (NHI) system in Taiwan. Patients with TD and MG were identified by referring to the ICD-9-CM codes...
2017: Translational Neuroscience
https://www.readbyqxmd.com/read/28707128/thymectomy-is-a-beneficial-therapy-for-patients-with-non-thymomatous-ocular-myasthenia-gravis-a-systematic-review-and-meta-analysis
#10
Kai Zhu, Jiaoxing Li, Xin Huang, Wei Xu, Weibin Liu, Jiaxin Chen, Pei Chen, Huiyu Feng
Ocular myasthenia gravis, an autoimmune disease, is characterized by extraocular muscle weakness. Myasthenia gravis is closely associated with the functional status of the thymus gland. The efficacy of thymectomy for non-thymomatous ocular myasthenia gravis remains controversial. Here, we present the first systematic review and meta-analysis of studies assessing the outcome of thymectomy in patients with non-thymomatous ocular myasthenia gravis and found that the pooled rate of complete stable remission was 0...
July 13, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28666511/unilateral-external-ophthalmoplegia-arare-presentation-of-myasthenia-gravis
#11
Muhammad Saim Khan, Asad Habib, Imran Basit
Myasthenia gravis (MG) is a disease of autoimmunity with variable and diverse clinical presentations. The target tissue is neuromuscular junction of skeletal muscles, where efficient nerve impulse transmission is hampered leading to less effective muscle contraction. Patients of MG usually present with bilateral ptosis, diplopia and fatigability, which may or may not coexist with generalised weakness, dysphagia and dysarthria. A46-year male presented with unilateral ptosis and diplopia. Except for unilateral moderate ptosis and restriction of extraocular movements, ocular and systemic examination was normal...
November 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28655853/rapamycin-alleviates-inflammation-and-muscle-weakness-while-altering-the-treg-th17-balance-in-a-rat-model-of-myasthenia-gravis
#12
Feng Jing, Fei Yang, Fang Cui, Zhao Hui Chen, Li X Ling, Xu S Huang
Myasthenia gravis (MG) is an autoimmune disease commonly treated with immunosuppressants. We evaluated the novel immunosuppressant, rapamycin (RAPA), in a rat model of experimental autoimmune MG (EAMG). Mortality rates in the RAPA (12%) was significantly down compared to the EAMG (88%) or CTX (68%) intervention groups. Muscular weakness decreased after both RAPA and cyclophosphamide (CTX) treatment; however, Lennon scores were lower (1.74 ± 0.49, 3.39 ± 0.21, and 3.81 ± 0.22 in RAPA, CTX, and EAMG groups, respectively), and body weights (203...
June 27, 2017: Bioscience Reports
https://www.readbyqxmd.com/read/28641305/incidence-of-autoimmune-myasthenia-gravis-in-a-health-maintenance-organization-in-buenos-aires-argentina
#13
Mariela Bettini, Marcelo Chaves, Edgardo Cristiano, Vanina Pagotto, Lucia Perez, Diego Giunta, Marcelo Rugiero
BACKGROUND: Different epidemiological studies, especially in Europe, have estimated the incidence density of myasthenia gravis (MG) to range between 1.7 and 21.3/1,000,000/person-year; however, data from regions such as Latin America are scarce. This study is aimed at estimating the incidence and prevalence of acquired MG in Buenos Aires, Argentina. METHODS: The study population comprised of affiliates of the Italian Hospital Medical Care Program, a prepaid health maintenance organization located in Buenos Aires...
June 23, 2017: Neuroepidemiology
https://www.readbyqxmd.com/read/28635482/diagnosis-and-management-of-myasthenia-gravis
#14
Christopher Barber
Myasthenia gravis is a rare long-term neurological condition that is characterised by fluctuating skeletal muscle weakness and fatigue, as well as respiratory difficulties. It is both an acquired autoimmune disease and a chronic neuromuscular disorder. Because of its rarity, myasthenia gravis is relatively unknown and may be unfamiliar to many nurses. While there are various types of myasthenia, this article focuses on myasthenia gravis, exploring its symptoms, diagnosis and treatment, and examining the nurse's role in managing the condition...
June 21, 2017: Nursing Standard
https://www.readbyqxmd.com/read/28627279/maternal-autoimmune-disorders-and-fetal-defects
#15
Anca Maria Panaitescu, Kypros Nicolaides
Maternal autoantibodies can cross the placenta and cause fetal damage. This article summarizes the development and management of fetal thyroid goiter in response to maternal Graves' disease and/or its treatment with antithyroid medication, fetal heart block due to maternal anti-Ro and anti-La antibodies, fetal athrogryposis multiplex congenita in association with maternal myasthenia gravis and fetal brain hemorrhage due to maternal autoimmune thrombocytopenia.
June 18, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28623849/population-pharmacokinetics-pharmacodynamics-of-3-4-diaminopyridine-free-base-in-patients-with-lambert-eaton-myasthenia
#16
Nilay Thakkar, Jeffrey T Guptill, Kathy Aleš, David Jacobus, Laura Jacobus, Charles Peloquin, Michael Cohen-Wolkowiez, Daniel Gonzalez
Lambert-Eaton Myasthenia (LEM) is a rare autoimmune disorder associated with debilitating muscle weakness. There are limited treatment options and 3,4-diaminopyridine free base (3,4-DAP) is an investigational orphan drug used to treat LEM-related weakness. We performed a population PK/PD analysis using 3,4-DAP and metabolite concentrations collected from a phase 2 study in LEM patients. The Triple Timed Up & Go (3TUG) assessment, which measures lower extremity weakness, was the primary outcome measure. A total of 1270 PK samples (49 patients) and 1091 3TUG data points (32 randomized patients) were included in the PK/PD analysis...
June 17, 2017: CPT: Pharmacometrics & Systems Pharmacology
https://www.readbyqxmd.com/read/28620344/the-role-of-osteopontin-and-its-gene-on-glucocorticoid-response-in-myasthenia-gravis
#17
Yanchen Xie, Hai-Feng Li, Liang Sun, Linda L Kusner, Shuhui Wang, Yunxiao Meng, Xu Zhang, Yu Hong, Xiang Gao, Yao Li, Henry J Kaminski
Biomarkers that assess treatment response for patients with the autoimmune disorder, myasthenia gravis (MG), have not been evaluated to a significant extent. We hypothesized the pro-inflammatory cytokine, osteopontin (OPN), may be associated with variability of response to glucocorticoids (GCs) in patients with MG. A cohort of 250 MG patients treated with standardized protocol of GCs was recruited, and plasma OPN and polymorphisms of its gene, secreted phosphoprotein 1 (SPP1), were evaluated. Mean OPN levels were higher in patients compared to healthy controls...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28608304/lambert-eaton-myasthenic-syndrome-lems-a-rare-autoimmune-presynaptic-disorder-often-associated-with-cancer
#18
REVIEW
Benedikt Schoser, Bruno Eymard, Joe Datt, Renato Mantegazza
Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune neuromuscular junction disorder that is related to the loss of functional P/Q-type voltage-gated calcium channels (VGCCs) on presynaptic nerve terminals. Up to 60% of cases occur as a paraneoplastic disorder (SCLC-LEMS), most commonly in association with small cell lung cancer. The remaining cases have an idiopathic non-tumor etiology but are associated with underlying autoimmune disease (NT-LEMS). Patients with LEMS invariably experience progressive proximal muscle weakness, often accompanied by general fatigue and autonomic symptoms...
June 12, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28604379/physiotherapy-immediately-after-thymectomy-in-patients-with-myasthenia-gravis-two-cases-and-review-of-the-literature
#19
Massimiliano Polastri, Franco Stella, Massimo Lambertini, Walter Trani, Annalisa Ghetti, Andrea Dell'Amore
AIM: Myasthenia gravis (MG) is a rare autoimmune disease characterized by activation of autoantibodies against acetylcholine receptors: patients with MG typically experience muscle weakness and fatigue. The aims of the present study were 1) to describe immediate postoperative physiotherapeutic interventions in two MG patients who underwent thymectomy, and 2) to discuss postoperative rehabilitative issues in MG patients. MATERIALS AND METHODS: This was a non-experimental study analyzing two subjects with MG who underwent thymectomy...
2017: Annali Italiani di Chirurgia
https://www.readbyqxmd.com/read/28599652/regulatory-t-cells-in-multiple-sclerosis-and-myasthenia-gravis
#20
REVIEW
K M Danikowski, S Jayaraman, B S Prabhakar
Multiple sclerosis (MS) is a chronic debilitating disease of the central nervous system primarily mediated by T lymphocytes with specificity to neuronal antigens in genetically susceptible individuals. On the other hand, myasthenia gravis (MG) primarily involves destruction of the neuromuscular junction by antibodies specific to the acetylcholine receptor. Both autoimmune diseases are thought to result from loss of self-tolerance, which allows for the development and function of autoreactive lymphocytes. Although the mechanisms underlying compromised self-tolerance in these and other autoimmune diseases have not been fully elucidated, one possibility is numerical, functional, and/or migratory deficits in T regulatory cells (Tregs)...
June 9, 2017: Journal of Neuroinflammation
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