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Autoimmune myasthenia

Justyna Kubiszewska, Beata Szyluk, Piotr Szczudlik, Zbigniew Bartoszewicz, Małgorzata Dutkiewicz, Maksymilian Bielecki, Tomasz Bednarczuk, Anna Kostera-Pruszczyk
OBJECTIVES: Autoimmune thyroid diseases (ATDs) frequently accompany myasthenia gravis (MG) and may influence its course. We aimed to determine the association and impact of ATD with early- (<50 years), late-onset MG, or thymoma-MG. MATERIALS AND METHODS: Prevalence of ATD was measured in a cross-sectional study of 343 consecutive patients with MG (236 F, 107 M) aged 4-89 years; 83.8% were seropositive, in 2.9%, anti-MuSK antibodies were detected. Concentrations of antithyroid peroxidase antibodies, antithyroglobulin antibodies, antithyrotropin receptor antibodies, and TSH level were measured in all patients...
October 2016: Brain and Behavior
Yingguo Ren, Baochao Zhang, Dongpei Jia, Ke Hu
Objective To investigate the effects of tetramethylpyrazine (TMP) on experimental autoimmune myasthenia gravis (EAMG) in rats and explore the possible immune regulation mechanism. Methods Lewis rats were randomly divided into 4 groups: control group, EAMG group, TMP low-dose group (TMP-L, 10 mg/kg) and TMP high-dose group (TMP-H, 20 mg/kg). Except the control group, the other 3 groups were subjected to EAMG modeling. The body mass was determined and the symptoms of muscular weakness in rats were scored by Lennon EAMG criteria...
November 2016: Xi Bao Yu Fen Zi Mian Yi Xue za Zhi, Chinese Journal of Cellular and Molecular Immunology
Marion I Boldingh, Angelina H Maniaol, Cathrine Brunborg, Harald Weedon-Fekjær, Jan J G M Verschuuren, Chantal M E Tallaksen
OBJECTIVE: To study the risk of clinical onset of myasthenia gravis (MG) in pregnancy and during the first 6 months postpartum because an association between pregnancy or the postpartum period and the onset of autoimmune MG is widely assumed but not proven. METHODS: The design was a cross-sectional population-based cohort study of 2 MG cohorts (Norway and the Netherlands) with 1,038 healthy controls from Norway. Data were obtained on 246 women with MG (age at onset 15-45 years)...
October 21, 2016: Neurology
Paulo Victor Sgobbi de Souza, Gabriel Novaes de Rezende Batistella, Valéria Cavalcante Lino, Wladimir Bocca Vieira de Rezende Pinto, Marcelo Annes, Acary Souza Bulle Oliveira
Neuromuscular junction disorders represent a wide group of neurological diseases characterized by weakness, fatigability and variable degrees of appendicular, ocular and bulbar musculature involvement. Its main group of disorders includes autoimmune conditions, such as autoimmune acquired myasthenia gravis and Lambert-Eaton syndrome. However, an important group of diseases include congenital myasthenic syndromes with a genetic and sometimes hereditary basis that resemble and mimick many of the classic myasthenia neurological manifestations, but also have different presentations, which makes them a complex clinical, therapeutic and diagnostic challenge for most clinicians...
September 2016: Arquivos de Neuro-psiquiatria
Rui Fan, Ruirui Ji, Wenxin Zou, Guoliang Wang, Hu Wang, Daniel James Penney, Jin Jun Luo, Yuxin Fan
Andersen-Tawil syndrome (ATS) is an autosomal dominant, multisystem channelopathy characterized by periodic paralysis, ventricular arrhythmias and distinctive dysmorphic facial or skeletal features. The disorder displays marked intrafamilial variability and incomplete penetrance. Myasthenia gravis (MG) is an autoimmune disorder that demonstrates progressive fatigability, in which the nicotinic acetylcholine receptor (AChR) at neuromuscular junctions is the primary autoantigen. The present study reports a rare case of a 31-year-old woman with a history of morbid obesity and periodic weakness, who presented with hemodynamic instability, cardiogenic shock and facial anomalies...
October 2016: Experimental and Therapeutic Medicine
S Rutledge, O Kenny, S O'Riordan, C McGuigan, N Tubridy
Myasthenia Gravis (MG) is a disorder affecting components of the neuromuscular junction. Epidemiological studies show rising incidence and prevalence rates. The aim of this study was to determine the incidence and prevalence of MG in the Republic of Ireland. Data sources included patient lists from consultant neurologists and ophthalmologists, a neuroimmunology laboratory, general practitioners and the Myasthenia Gravis Association. A total of 1715 cases were identified, of which 706 definite, probable or possible autoimmune and congenital MG cases were included...
2016: Irish Medical Journal
Yi-Yun Weng, De-Hao Yang, Mei-Zi Qian, Mao-Mao Wei, Fang Yin, Jia Li, Xiang Li, Ying Chen, Zhang-Na Ding, Yi-Bo He, Xu Zhang
Serum albumin (S-Alb) is a widely used biomarker of nutritional status and disease severity in patients with autoimmune diseases. We investigated the correlation between S-Alb and the severity of myasthenia gravis (MG).A total number of 166 subjects were recruited in the study. Subjects were divided into 3 groups (T1 to T3) by S-Alb levels: T1: 21.1 to 38.4 g/L, T2: 38.5 to 41.5 g/L, T3: 41.6 to 48.9 g/L. Regression analysis was performed to determine the correlation of initial albumin concentrations and the severity of disease of MG...
September 2016: Medicine (Baltimore)
Jeffrey T Guptill, Vern C Juel, Janice M Massey, Amanda C Anderson, Manisha Chopra, John S Yi, Ehsanollah Esfandiari, Tim Buchanan, Bryan Smith, Paul Atherfold, Emma Jones, James F Howard
An integrated understanding of therapeutic plasma exchange (TPE) effects on immunoglobulins, autoantibodies, and natural or acquired (vaccine) protective antibodies in patients with autoimmune myasthenia gravis (MG) is lacking. Prior studies measured TPE effects in healthy volunteers or heterogeneous autoimmune disease populations. We prospectively profiled plasma IgA, IgM, IgG, IgG subclasses (IgG1-4), acetylcholine receptor autoantibodies (AChR+), and protective antibodies in patients with AChR + MG receiving TPE for an exacerbation...
August 11, 2016: Autoimmunity
T H Popperud, M I Boldingh, C Brunborg, K W Faiz, A T Heldal, A H Maniaol, K I Müller, M Rasmussen, K Øymar, E Kerty
BACKGROUND: The aim of this study was to assess the incidence rate and prevalence of autoimmune myasthenia gravis (MG) among children in Norway. METHODS: This retrospective population-based study was performed in Norway from January 2012 to December 2013. Cases of juvenile MG (JMG) with onset < 18 years were identified through searches in coding systems of electronic patient records at the 15 main hospitals in Norway from 1989 to 2013. In addition, the acetylcholine receptor antibody database at Haukeland University Hospital and the clinical nationwide MG database at Oslo University Hospital were searched for cases of JMG...
September 10, 2016: European Journal of Paediatric Neurology: EJPN
Mohammad Reza Zamani, Saeed Aslani, Arash Salmaninejad, Mohammad Reza Javan, Nima Rezaei
Programmed death 1 (PD-1) and its ligands, namely PD-L1 and PD-L2, are one of the key factors responsible for inhibitory T cell signaling, mediating the mechanisms of tolerance and providing immune homeostasis. Mounting evidence demonstrates that impaired PD-1:PD-L function plays an important role in a variety of autoimmune diseases such as Type 1 diabetes (T1D), encephalomyelitis, inflammatory bowel diseases (IBD), Rheumatoid Arthritis (RA), autoimmune hepatitis (AIH), Behcet's disease (BD), myasthenia gravis (MG), autoimmune uveitis (AU), Sjögren's syndrome (SjS), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), myocarditis, and ankylosing spondylitis (AS)...
September 15, 2016: Cellular Immunology
Alejandro M Gomez, Jo A A Stevens, Marina Mané-Damas, Peter Molenaar, Hans Duimel, Fons Verheyen, Judith Cossins, David Beeson, Marc H De Baets, Mario Losen, Pilar Martinez-Martinez
Myasthenia gravis (MG) is an autoimmune disease mediated by autoantibodies that target proteins at the neuromuscular junction, primarily the acetylcholine receptor (AChR) and the muscle-specific kinase. Because downstream of kinase 7 (Dok-7) is essential for the full activation of muscle-specific kinase and consequently for dense clustering of AChRs, we hypothesized that reduced levels of Dok-7 increase the susceptibility to passive transfer MG. To test this hypothesis, Dok-7 expression was reduced by transfecting shRNA-coding plasmids into the tibialis anterior muscle of adult rats by in vivo electroporation...
October 2016: American Journal of Pathology
M I Stefanou, L Komorowski, S Kade, A Bornemann, U Ziemann, M Synofzik
BACKGROUND: Myasthenia gravis is an autoimmune neuromuscular disorder, which has only rarely been reported to co-manifest with myositis. The diagnosis of concomitant myositis in patients with myasthenia gravis is clinically challenging, and requires targeted investigations for the differential diagnosis, including EMG, autoantibody assays, muscle biopsy and, importantly, imaging of the mediastinum for thymoma screening. CASE PRESENTATION: This report presents a case-vignette of a 72-year-old woman with progressive proximal muscle weakness and myalgias, diagnosed with thymoma-associated myasthenia and bioptically verified granulomatous myositis, with positive autoantibody status for ryanodine receptor and titin antibodies...
September 13, 2016: BMC Neurology
Vedat Uygun, Hayriye Daloğlu, Seda Irmak Öztürkmen, Levent Döşemeci, Gülsün Karasu, Volkan Hazar, Akif Yeşilipek
BACKGROUND: Myasthenia gravis (MG) is a neuromuscular disorder characterized by an autoimmune defect in the neuromuscular junction. In most patients, the autoimmune attack is mediated by antibodies against the acetylcholine receptor (AChR) on the postsynaptic membrane. Deficient immunoregulation, including regulatory T cells, is consistently observed. Extracorporeal photopheresis (ECP) leads to the induction of regulatory T cells that mediate immunologic tolerance in autoimmune diseases; however, the data regarding MG are very limited...
September 9, 2016: Transfusion
Qing-Fang Meng, Zheng Zhang, Yan-Jun Wang, Wei Chen, Fei-Fei Li, Long-Tao Yue, Chang-Jun Zhang, Heng Li, Min Zhang, Cong-Cong Wang, Peng Zhang, Hui Chen, Rui-Sheng Duan, Shan-Mei Sun, Yan-Bin Li
Astilbin, a major bioactive compound extracted from Rhizoma smilacis glabrae (RSG), has been reported to possess immunosuppressive properties. Our study first evaluated the effect of astilbin on experimental autoimmune myasthenia gravis (EAMG) in Lewis rats. The results showed that astilbin could attenuate the severity of EAMG by decreasing antigen-specific autoantibodies with up-regulation of regulatory T cells and down-regulation of Th17 cells. In addition to, astilbin also reduced the efficiency of the antigen presenting cells on which the expression of MHC class II decreased...
September 15, 2016: Journal of Neuroimmunology
Arnon Karni, Ali Asmail, Vivian E Drory, Hadar Kolb, Anat Kesler
The reasons for the abrogation of self-immunological tolerance in patients with myasthenia gravis (MG) may be different between those with concomitant thymic hyperplasia or thymoma, and those with no evidence of thymic involvement. We conducted a retrospective observational case series study to investigate the epidemiology as well as the clinical, serologic, and electromyographic (EMG) characteristics of individuals diagnosed as having MG. We found that the average age at MG onset of patients with either thymic hyperplasia or thymoma was much younger (by ~20years) than that of MG patients without thymic involvement...
September 15, 2016: Journal of Neuroimmunology
Yu Hong, Hai-Feng Li, Geir Olve Skeie, Fredrik Romi, Hong-Jun Hao, Xu Zhang, Xiang Gao, Jone Furlund Owe, Nils Erik Gilhus
Myasthenia gravis (MG) is an autoimmune disorder with heterogeneity. Antibodies against acetylcholine receptor (AChR), muscle-specific kinase (MuSK), titin and ryanodine receptor (RyR) were examined in 437 adult Chinese MG patients. The AChR, MuSK, titin and RyR antibodies were found in 82.2%, 2.3%, 28.4% and 23.8% of all patients. Autoantibody profiles vary among different MG subgroups. Thymoma MG patients had high frequencies of AChR (99.2%), titin (50.8%) and RyR antibodies (46.9%). The titin and RyR antibodies also showed high frequencies in late onset patients (54...
September 15, 2016: Journal of Neuroimmunology
Wei Chen, Qing-Fang Meng, Jun-Kang Sui, Yan-Jun Wang, Xiao-Li Li, Shen Liu, Heng Li, Cong-Cong Wang, Chun-Hong Li, Yan-Bin Li
Myasthenia gravis (MG) is an autoimmune neuromuscular disease characterized by fatigue and muscle weakness. Ginseng is used in the treatment of MG. Ginsenoside Rb1 (G-Rb1), the most abundant ginsenoside in ginseng root, has been proved to be immune regulatory in various diseases. In this study, we investigated the effects and mechanisms of G-Rb1 in treatment for MG in a rat model. Our data showed that G-Rb1 treatment markedly ameliorated the symptoms of experimental autoimmune myasthenia gravis (EAMG) rats, decreased the percentage of Th17 cells in mononuclear cells (MNCs), and increased the number of Treg and Th2 cells in MNCs...
September 2, 2016: International Immunopharmacology
Nicholas J Silvestri, Gil I Wolfe, David Lacomis, Mark B Bromberg
The Guillain-Barré syndrome (GBS) is one of the few neuropathies well known to the general public, in part because of its association with swine flu vaccinations in 1976. GBS has again reached the general public with its possible association with Zika virus. The virus, borne by infected Aedes aegypti mosquitos, is being linked to birth defects when pregnant women are bitten and infected. There are early reports also linking GBS to Zika infection, which could expose a wider range of infected people to the neuropathy...
September 2016: Journal of Clinical Neuromuscular Disease
Kristin L Jack, Wilma J Koopman, Denise Hulley, Michael W Nicolle
OBJECTIVES: Myasthenia gravis (MG) is an autoimmune disorder in which antibodies interfere with neuromuscular transmission. Azathioprine (AZA) is an immunosuppressant frequently used for treatment of various autoimmune conditions, including MG. The literature suggests that the rates of AZA-associated hepatotoxicity and myelosuppression in MG are highly variable. Published studies have not formally analyzed their pattern, severity, timing, and/or recovery. We assessed the prevalence, pattern and timing of AZA associated toxicity in a large group of MG patients...
September 2016: Journal of Clinical Neuromuscular Disease
Paola Ortiz-Salas, Alberto Velez-Van-Meerbeke, Camilo Alberto Galvis-Gomez, Jesús H Rodriguez Q
OBJECTIVES: To evaluate the efficacy and safety over a short time period of human intravenous immunoglobulin versus plasma exchange (PE) in the management of some autoimmune neurologic diseases. In addition, length of hospital stay and duration of ventilator support were compared. METHODS: Randomized controlled trials and analytical observational studies of more than 10 cases were reviewed. Cochrane Neuromuscular Disease Group trials, MEDLINE, EMBASE, HINARI, and Ovid, were searched as data source...
September 2016: Journal of Clinical Neuromuscular Disease
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