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Autoimmune myasthenia

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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/28599246/ablation-of-il-17-expression-moderates-experimental-autoimmune-myasthenia-gravis-disease-severity
#12
Gabriela Aguilo-Seara, Yanchen Xie, Jarrod Sheehan, Linda L Kusner, Henry J Kaminski
An array of cytokines influences the pathogenesis of early onset myasthenia gravis (MG) and its animal model, experimental autoimmune myasthenia gravis (EAMG). Patients with MG, in particular those with more severe weakness, have elevations of the pro-inflammatory cytokine IL-17 in the blood. We assessed the role of IL-17A in autoimmunity by inducing EAMG in mice with knockout of IL-17 and found a reduction of EAMG severity, but not a complete ablation of disease. The IL-17(ko) mice had no evidence of weakness, low levels of acetylcholine receptor antibodies, and retention of acetylcholine receptor at the neuromuscular junction...
August 2017: Cytokine
https://www.readbyqxmd.com/read/28548588/injection-of-inactive-bordetella-pertussis-and-complete-freund-s-adjuvant-with-torpedo-californica-achr-increases-the-occurrence-of-experimental-autoimmune-myasthenia-gravis-in-c57bl-6-mice
#13
Takahiro Maruta, Minako Oshima, Dennis R Mosier, M Zouhair Atassi
An animal model of myasthenia gravis (MG), termed experimental autoimmune MG (EAMG), is an important tool for investigations of disease mechanisms and/or methods of treatment for this disease. EAMG can be induced in C57BL/6 (B6, H-2(b)) mice by 2-3 times injections at 4 weeks intervals with Torpedo californica (t) acetylcholine receptor (AChR) in complete Freund's adjuvant (CFA). However, the protocol especially with a two-injection schedule occasionally produces a poor incidence of EAMG. We have investigated the efficacy of the additional adjuvant, inactive organisms of Bordetella pertussis (iBP), on the induction with a two-injection schedule...
May 26, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28523463/immature-exosomes-derived-from-microrna-146a-overexpressing-dendritic-cells-act-as-antigen-specific-therapy-for-myasthenia-gravis
#14
Weifan Yin, Song Ouyang, Zhaohui Luo, Qiuming Zeng, Bo Hu, Liqun Xu, Yuan Li, Bo Xiao, Huan Yang
Myasthenia gravis (MG) is a neurological autoimmune disease characterized by fluctuating weakness of certain voluntary muscles. Current treatments for MG are largely directed at suppressing the whole immune system by using immunosuppressants or glucocorticoids and often cause several side effects. The ideal therapeutic methods for MG should suppress aberrant immunoactivation specifically, while retaining normal function of the immune system. In this study, we first produced exosomes from microRNA-146a overexpressing dendritic cells (DCs)...
August 2017: Inflammation
https://www.readbyqxmd.com/read/28516329/screening-for-lipoprotein-receptor-related-protein-4-agrin-and-titin-antibodies-and-exploring-the-autoimmune-spectrum-in-myasthenia-gravis
#15
Isabell Cordts, Nicolas Bodart, Kathi Hartmann, Katerina Karagiorgou, John S Tzartos, Lin Mei, Jens Reimann, Philip Van Damme, Michael H Rivner, Alain Vigneron, Joachim Weis, Jörg B Schulz, Socrates J Tzartos, Kristl G Claeys
In autoimmune myasthenia gravis (MG), the identification of antibodies and characterization of serological subgroups is of great importance for diagnosis and management of the disease. Our aims were to study the frequency of antibodies against lipoprotein-related protein 4 (LRP4), agrin, and titin using the most recent techniques, and to characterize corresponding clinical features and autoimmune diseases (AID) in 100 MG-patients. The antibody frequencies in the 55 AChR-antibody positive patients were 7% LRP4, 5% agrin, 53% titin, and in the 45 AChR-antibody negative patients 2% MuSK, 2% LRP4, 2% agrin, and 27% titin...
June 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28506261/a-possible-role-of-low-regulatory-t-cells-in-anti-acetylcholine-receptor-antibody-positive-myasthenia-gravis-after-bone-marrow-transplantation
#16
Masahiko Fukatsu, Takenobu Murakami, Hiroshi Ohkawara, Shunichi Saito, Kazuhiko Ikeda, Suguru Kadowaki, Itaru Sasaki, Mari Segawa, Tomoko Soeda, Akihiko Hoshi, Hiroshi Takahashi, Akiko Shichishima-Nakamura, Kazuei Ogawa, Yoshihiro Sugiura, Hitoshi Ohto, Yasuchika Takeishi, Takayuki Ikezoe, Yoshikazu Ugawa
BACKGROUND: Chronic graft-versus-host disease (GVHD) appears several months following allogenic hematopoietic stem cell transplantation (HSCT) and is clinically analogous to autoimmune disorder. Polymyositis is a common neuromuscular disorder in chronic GVHD, but myasthenia gravis (MG) is extremely rare. Hence, its pathophysiology and treatment have not been elucidated. CASE PRESENTATION: A 63-year-old man with a history of chronic GVHD presented with ptosis, dropped head, and dyspnea on exertion, which had worsened over the previous several months...
May 15, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28505981/severe-exacerbation-of-myasthenia-gravis-associated-with-checkpoint-inhibitor-immunotherapy
#17
Dana S Cooper, Matthew N Meriggioli, Philip D Bonomi, Rabia Malik
Monoclonal antibodies that target either PD-1 or PD-L1 have recently been approved for treatment of advanced non-small cell lung cancer. These antibodies are immune checkpoint inhibitors which have been shown to exacerbate Myasthenia Gravis (MG) and other autoimmune diseases. While effective in preventing tumor cells from evading immune attack, immune checkpoint inhibitors such as nivolumab, an antibody directed against the programmed cell death protein-1 (PD-1) receptor located on T-cells, may also cause immune dysregulation and could cause or potentiate pre-existing autoimmune conditions...
2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/28495138/effect-of-ethnic-origin-and-gender-on-the-clinical-manifestations-of-myasthenia-gravis-among-the-jewish-population-in-israel
#18
Ali Asmail, Anat Kesler, Vivian E Drory, Hadar Kolb, Arnon Karni
Reports on patients with myasthenia gravis (MG) of different ethnic origins demonstrated differences in weakness distribution and serological results. We studied MG characteristics in a cohort of Ashkenazi (ASH) and non-Ashkenazi (NASH) Jewish origin according to their ethnic origins and gender. The frequency of age of MG onset was distributed in a bi-modal fashion in the female patients and increased gradually over time, with a peak around 70years of age in the male patients. Ocular MG was more frequent in males and ASH patients...
June 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28470860/multiple-antibody-detection-in-seronegative-myasthenia-gravis-patients
#19
Y Hong, P Zisimopoulou, N Trakas, K Karagiorgou, C Stergiou, G O Skeie, H-J Hao, X Gao, J F Owe, X Zhang, Y-X Yue, F Romi, Q Wang, H-F Li, N E Gilhus, S J Tzartos
BACKGROUND AND PURPOSE: Myasthenia gravis (MG) is an autoimmune disease caused by antibody mediated impairment in the neuromuscular junction. Seronegative MG (SNMG) without antibodies against acetylcholine receptor (AChR) and muscle-specific kinase (MuSK) by routine assays accounts for about 20% of all MG patients. METHODS: Plasma from 81 Chinese MG patients previously found to be seronegative was tested by routine assays for AChR and MuSK antibodies. These samples were screened by (i) a novel, highly sensitive radioimmunoassay for AChR antibodies; (ii) cell-based assays for clustered AChR, MuSK and lipoprotein receptor-related protein 4 (LRP4) antibodies; (iii) a radioimmunoassay for titin antibodies...
June 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28463217/-neuropsychological-performance-in-patients-with-myasthenia-gravis
#20
María Bárbara Eizaguirre, Florencia Aguirre, Cecilia Yastremiz, Sandra Vanotti, Andrés Villa
Myasthenia gravis is a chronic autoimmune disease that affects the neuromuscular transmission. Controversial findings had been reported about cognitive impairment in this disease. The aim of this study was to investigate the cognitive pattern of patients with myasthenia gravis. There were enrolled 24 patients with myasthenia gravis, anti-acetylcholine receptor antibodies (ACRA) positive, and 24 healthy controls. PATIENTS: age 43.9 ± 14.8, years of education 10.9 ± 3.3. CONTROLS: age 44...
2017: Medicina
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