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Autoimmune myasthenia

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https://www.readbyqxmd.com/read/29773681/decreased-expression-of-circulating-aire-and-increased-tfh-tfr-cells-in-myasthenia-gravis-patients
#1
Sijia Zhao, Jiaqi Ding, Shengyuan Wang, Chuan Li, Peng Guo, Min Zhang, Zhuyi Li
Myasthenia gravis (MG) is a rare prototypical autoimmune disorder caused by antibodies (Ab) against postsynaptic membrane proteins. Most reports have investigated the role of Autoimmune regulator gene (Aire) in thymic tissue in machianism of MG initiation. So far, the expression of Aire in human peripheral blood cells(we call it circulating Aire expression in the following passage) has not been reported. Herein, we explore the expression of Aire in peripharal blood, circulating T follicular helper (cTfh) and T follicular regulatory (cTfr) cells in MG patients...
May 17, 2018: Bioscience Reports
https://www.readbyqxmd.com/read/29765992/serological-immunoglobulin-free-light-chain-profile-in-myasthenia-gravis-patients
#2
Umberto Basile, Mariapaola Marino, Cecilia Napodano, Krizia Pocino, Paolo Emilio Alboini, Francesca Gulli, Amelia Evoli, Carlo Provenzano, Emanuela Bartoccioni
Background: Serological levels of free immunoglobulin light chains (FLCs), produced in excess of heavy chains during synthesis of immunoglobulins by plasma cells, can be considered a direct marker of B cell activity in different systemic inflammatory-autoimmune conditions and may represent a useful predictor of rituximab (RTX) therapeutic efficacy, as reported for rheumatoid arthritis and systemic lupus erythematosus. Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction with antibodies (abs) targeting the acetylcholine receptor (AChR) or the muscle-specific tyrosine kinase (MuSK), inducing muscle weakness and excessive fatigability...
2018: Journal of Immunology Research
https://www.readbyqxmd.com/read/29761126/seronegative-antibody-mediated-neurology-after-immune-checkpoint-inhibitors
#3
Robert Wilson, David A Menassa, Alexander J Davies, Sophia Michael, Joanna Hester, Wilhelm Kuker, Graham P Collins, Judith Cossins, David Beeson, Neil Steven, Paul Maddison, Simon Rinaldi, Saiju Jacob, Sarosh R Irani
Checkpoint inhibitor medications have revolutionized oncology practice, but frequently induce immune-related adverse events. During autoimmune neurology practice over 20 months, we prospectively identified four patients with likely antibody-mediated neurological diseases after checkpoint inhibitors: longitudinally extensive transverse myelitis, Guillain-Barré syndrome, and myasthenia gravis. All patients shared three characteristics: symptoms commenced 4 weeks after drug administration, responses to conventional immunotherapies were excellent, and autoantibodies traditionally associated with their syndrome were absent...
May 2018: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29759142/heterogeneity-of-auto-antibodies-against-nachr-in-myasthenic-serum-and-their-pathogenic-roles-in-experimental-autoimmune-myasthenia-gravis
#4
Ryuichi Nakamura, Tomohiro Makino, Takeshi Hanada, Maki Terakawa, Kazuhiro Nagahira, Jyoji Yamate, Hirokazu Shiraishi, Masakatsu Motomura
Many myasthenia gravis (MG) patients have auto-antibodies against the nicotinic acetylcholine receptor (nAChR), and monoclonal antibodies against the main immunogenic region (MIR) of nAChR can induce experimental autoimmune MG (EAMG). We investigated whether Fab fragment of MIR antibody (Fab35) could block the pathogenicity of polyclonal antibodies. Fab35 partially inhibited nAChR downmodulation, blocked EAMG serum-induced binding of polyclonal antibodies and complement deposition in vitro. Moreover, Fab35 did not ameliorate the EAMG serum-induced EAMG phenotype in rats...
July 15, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29746944/increased-risk-of-autoimmune-diseases-in-dengue-patients-a-population-based-cohort-study
#5
Hao-Ming Li, Ying-Kai Huang, Yuan-Chih Su, Chia-Hung Kao
OBJECTIVE: To investigate the risk of autoimmune diseases in dengue patients. METHODS: We conducted a population-based cohort study by the Taiwan National Health Insurance Research Database, including a total of 12506 newly diagnosed dengue patients and 112554 control subjects between 2000 and 2010, matched by gender, age, income, urbanization, and comorbidities. Both cohorts were followed for a 3-year period to examine the incidence of autoimmune diseases. A Cox proportional hazards regression analysis was applied to calculate the risk of autoimmune diseases between both groups...
May 7, 2018: Journal of Infection
https://www.readbyqxmd.com/read/29742813/mutated-cancer-autoantigen-implicated-cause-of-paraneoplastic-myasthenia-gravis
#6
Anastasia Zekeridou, Guy E Griesmann, Vanda A Lennon
INTRODUCTION: Anti-tumor immune responses are postulated to initiate paraneoplastic neurological disorders when proteins normally restricted to neural cells are expressed as oncoproteins. Mutated oncopeptides could bypass self-tolerant T-cells to activate cytotoxic effector T-lymphocytes and requisite helper T-lymphocytes to stimulate autoantibody production by B-lymphocytes. METHODS: We investigated muscle-type nicotinic acetylcholine receptor (AChR) antigen expression at transcriptional and protein levels in a small-cell lung cancer line (SCLC) established from a patient with AChR-IgG-positive myasthenia gravis...
May 9, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29730659/myxedema-coma-due-to-hashimoto-thyroiditis-a-rare-but-real-presentation-of-failure-to-thrive-in-infancy
#7
Ryan A Heksch, Rohan K Henry
BACKGROUND: Hashimoto thyroiditis (HT) is uncommon in infancy, and myxedema coma (MC) is even less common. While prior reports have documented these entities separately, to our knowledge, MC in combination with HT has not been reported before in this age group. METHODS/RESULTS: A 10-month-old female presented with ptosis, lethargy, dysphagia, and failure to thrive (FTT). She developed hypotension, bradycardia, hypothermia, and apnea requiring intubation. Initial thyroid-stimulating hormone was 422 μIU/mL, and free thyroxine was < 0...
May 4, 2018: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29720797/the-spectrum-of-autonomic-dysfunction-in-myasthenic-crisis
#8
Rohit Ninan Benjamin, Sanjith Aaron, Ajith Sivadasan, Suresh Devasahayam, Amalan Sebastin, Mathew Alexander
Background: Autoimmune autonomic dysfunction is described in Myasthenia Gravis. In myasthenic crisis, the spectrum of autonomic dysfunction is hitherto uncharacterized. Objective: The objective of this study is to describe the spectrum of autonomic dysfunction in myasthenic crises using the composite autonomic symptom scale 31 (COMPASS 31) autonomic symptom questionnaire and power spectral analysis of heart rate variability (HRV), which is a simple way of estimating general autonomic dysfunction...
January 2018: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/29718870/ctla-4-methylation-regulates-the-pathogenesis-of-myasthenia-gravis-and-the-expression-of-related-cytokines
#9
Ti-Kun Fang, Cheng-Jun Yan, Juan Du
BACKGROUND: Myasthenia gravis (MG) is a progressive autoimmune disease that occurs as a result of the failure of neuromuscular transmission and is characterized by muscle weakness. There has been evidence on the correlations between the genetic predisposition of cytotoxic T lymphocyte and the antigen-4 (CTLA-4) and MG. Thus, the present study was conducted to study is designed to examine the effects of CTLA-4 methylation on the pathogenesis of MG and the expressions of related cytokines...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29699793/serum-interleukin-15-levels-in-patients-with-seropositive-myasthenia-gravis-do-not-correlate-with-disease-severity
#10
Ewa Sobieszczuk, Justyna Kubiszewska, Marta Lipowska, Piotr Szczudlik, Beata Szyluk, Małgorzata Dutkiewicz, Anna Kostera-Pruszczyk
AIM: To assess interleukin 15 (IL-15) serum levels in patients with seropositive myasthenia gravis (MG); searching for potential relationship between IL-15 levels and clinical features such as gender, age at onset, clinical presentation or treatment received. BACKGROUND: IL-15 plays pivotal role in T-cell dependent autoimmunity. Increased IL-15 serum levels have been reported in several autoimmune diseases including MG patients from Japan. PATIENTS AND METHODS: Sera of 42 seropositive MG patients (66...
January 6, 2018: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/29689030/checkpoint-inhibitor-immune-therapy-systemic-indications-and-ophthalmic-side-effects
#11
Lauren A Dalvin, Carol L Shields, Marlana Orloff, Takami Sato, Jerry A Shields
PURPOSE: To review immune checkpoint inhibitor indications and ophthalmic side effects. METHODS: A literature review was performed using a PubMed search for publications between 1990 and 2017. RESULTS: Immune checkpoint inhibitors are designed to treat system malignancies by targeting one of three ligands, leading to T-cell activation for attack against malignant cells. These ligands (and targeted drug) include cytotoxic T-lymphocyte antigen-4 (CTLA-4, ipilimumab), programmed death protein 1 (PD-1, pembrolizumab, nivolumab), and programmed death ligand-1 (PD-L1, atezolizumab, avelumab, durvalumab)...
April 23, 2018: Retina
https://www.readbyqxmd.com/read/29683988/case-report-ocular-myasthenia-gravis-associated-with-in-vitro-fertilization-procedures
#12
Yung Ju Yoo, Sang Beom Han, Hee Kyung Yang, Jeong-Min Hwang
SIGNIFICANCE: Ocular myasthenia gravis is a localized form of myasthenia gravis, which is a postsynaptic disorder of the neuromuscular junction that causes fluctuating weakness of extraocular muscles resulting from autoimmune mechanisms. In women with myasthenia, changes in sex hormone levels and administration of corticosteroids can trigger or worsen symptoms of myasthenia gravis. PURPOSE: To describe a case of seronegative ocular myasthenia gravis whose first symptom appeared a day after in vitro fertilization procedure...
April 20, 2018: Optometry and Vision Science: Official Publication of the American Academy of Optometry
https://www.readbyqxmd.com/read/29668540/aplastic-anemia-as-an-immune-mediated-complication-of-thymoma-a-case-report
#13
Ersin Toret, Bengü Demirag, Sultan A Köker, Onur Doyurgan, Malik Ergin, Sinan Genc, Tuba H Karapinar, Yilmaz Ay, Yeşim Oymak, Canan Vergin
Thymomas are the most common masses located in the anterior mediastinum, and they are often associated with autoimmune disorders including myasthenia gravis, polymyositis, and aplastic anemia (AA). Autoreactive T-cell clones generated by the thymoma may lead to autoimmune disorders. We report the case of a 14-year-old boy who was examined for AA, and the underlying cause was determined to be an immune-mediated complication of thymoma. He had no matched sibling donors. He underwent thymectomy, and 3 months later he was treated with immunosuppressive therapy (IST), consisting of antithymocyte globulin and cyclosporine A...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29666602/myasthenia-gravis-induced-by-ipilimumab-in-a-patient-with-metastatic-melanoma
#14
Vera Montes, Sandra Sousa, Fernando Pita, Rui Guerreiro, Cátia Carmona
In daily clinical practice, there is a growing number of patients receiving new biological agents used in the treatment of malignancies. Ipilimumab is a fully humanized monoclonal antibody approved for patients with melanoma. It acts as an immune checkpoint inhibitor, binding and blocking cytotoxic T-lymphocyte antigen-4 in order to increase the antitumor immune response. There are several reports of autoimmune responses after its use. A 74-year-old man developed a mild rash and pruritus a few hours after the second infusion of ipilimumab and 24 h after the third dose of ipilimumab, he presented with shortness of breath, proximal limb muscle weakness, and diplopia...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29664459/multiple-sclerosis-and-myasthenia-gravis-following-severe-weight-loss
#15
Mohammed H Alanazy, Majed A Alomar, Bandar N Aljafen, Taim A Muayqil
Weight loss has substantial health benefits, but it is not risk-free. Various neurological disorders have been reported following bariatric surgery-induced weight loss. Here, we report 3 patients who developed multiple sclerosis (MS), one of whom also developed myasthenia gravis (MG), shortly after significant weight loss. Two patients lost weight by following a diet plan and one underwent bariatric surgery. There may be an association between significant weight loss and the development of an autoimmune neurological disorder such as MS or MG; a high index of suspicion is required...
April 2018: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
https://www.readbyqxmd.com/read/29661539/mir-548k-regulates-cxcl13-expression-in-myasthenia-gravis-patients-with-thymic-hyperplasia-and-in-jurkat-cells
#16
Jinpin Li, Di Qiu, Zezhi Chen, Weiwei Du, Jingli Liu, Xuean Mo
Myasthenia gravis (MG) is a B cell-mediated and T cell-dependent autoimmune disease. Thymic hyperplasia has great significance for MG pathogenesis and treatment. MicroRNAs (miRNAs) are a newly recognized type of gene expression regulatory factor that regulate gene expression at the post-transcriptional level. Additionally, miRNAs are involved in immune regulation of the thymus and the occurrence and development of autoimmune diseases. In this study, we found 33 miRNAs that were significantly dysregulated in thymic tissues from MG patients with thymus hyperplasia (MGH) compared with thymic tissues from normal controls using a miRNA microarray chip...
April 4, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29655674/analysis-of-microrna-expression-in-the-thymus-of-myasthenia-gravis-patients-opens-new-research-avenues
#17
REVIEW
Mélanie A Cron, Solène Maillard, Fabien Delisle, Nolwenn Samson, Frédérique Truffault, Maria Foti, Elie Fadel, Julien Guihaire, Sonia Berrih-Aknin, Rozen Le Panse
In early-onset Myasthenia Gravis (MG) with anti-acetylcholine receptor antibodies, thymic abnormalities associated with ectopic germinal centers are frequent. miRNAs by acting as post-transcriptional regulators are involved in autoimmunity. To investigate the implication of miRNAs in thymic changes associated with early-onset MG, we performed a miRnome study and data were analyzed with different approaches. miRNAs of interest were further investigated by RT-PCR and transfection experiments for functional tests...
June 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29655450/nature-and-action-of-antibodies-in-myasthenia-gravis
#18
REVIEW
Robert L Ruff, Robert P Lisak
This article discusses antibodies associated with immune-mediated myasthenia gravis and the pathologic action of these antibodies at the neuromuscular junctions of skeletal muscle. To explain how these antibodies act, we consider the physiology of neuromuscular transmission with emphasis on 4 features: the structure of the neuromuscular junction; the roles of postsynaptic acetylcholine receptors and voltage-gated Na+ channels and in converting the chemical signal from the nerve terminal into a propagated action potential on the muscle fiber that triggers muscle contraction; the safety factor for neuromuscular transmission; and how the safety factor is reduced in different forms of autoimmune myasthenia gravis...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655448/generalized-myasthenia-gravis-classification-clinical-presentation-natural-history-and-epidemiology
#19
REVIEW
Michael K Hehir, Nicholas J Silvestri
Myasthenia gravis (MG) is a rare disease, but the most common disorder of the neuromuscular junction. It is the prototypic autoimmune disease most commonly caused by antibodies to the acetylcholine receptor (AChR) leading to characteristic fatigable weakness of the ocular, bulbar, respiratory, axial, and limb muscles. The majority of patients with MG first present with ocular symptoms. Most patients with MG will experience at least 1 exacerbation of symptoms throughout the course of their illness. This article will cover the epidemiology, clinical presentation, classification, and natural history of MG...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29652962/-myasthenia-gravis-and-its-association-with-lymphoproliferative-disorders-a-case-series
#20
Gabriel Cea, Andrés Gallardo V, María Elena Cabrera C
Myasthenia gravis (MG) is a rare autoimmune disease of the neuromuscular junction. It is characterized by variable weakness and excessive fatigability of skeletal muscles. In the last few years, numerous reports have been published showing the association between autoimmune diseases, such as systemic erythematous lupus or rheumatoid arthritis, with lymphoid neoplasias. The association between MG and lymphoid neoplasia seems to be less frequent. To analyze this association we reviewed the MG patients in the Department of Neurology, Hospital Salvador of Santiago, Chile...
December 2017: Revista Médica de Chile
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