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Autoimmune myasthenia

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https://www.readbyqxmd.com/read/29348988/anal-squamous-cell-carcinoma-in-a-patient-with-myasthenia-gravis-is-immunosuppression-the-main-underlying-etiology
#1
Muhammad Masab, Muhammad W Saif
Patients who are immunocompromised by diseases such as human immunodeficiency virus (HIV) infection are more prone to develop some malignancies such as Kaposi's sarcoma and central nervous system (CNS) lymphomas. Historically, anal squamous cell carcinoma (SCC) was also included on the list as an acquired immunodeficiency syndrome (AIDs)-defining cancer.  Similarly, compromised immune disorders including severe immunosuppression, haematologic malignancies, and solid organ transplantation have been identified as important risk factors for the development of anal SCC...
November 15, 2017: Curēus
https://www.readbyqxmd.com/read/29342325/the-neuromuscular-complications-of-immune-checkpoint-inhibitor-therapy
#2
REVIEW
Noah A Kolb, Christopher R Trevino, Waqar Waheed, Fatemeh Sobhani, Kara K Landry, Alissa A Thomas, Mike Hehir
Immune checkpoint inhibitor (ICPI) therapy unleashes the body's natural immune system to fight cancer. ICPIs improve overall cancer survival, however the unbridling of the immune system may induce a variety of immune related adverse events. Neuromuscular immune complications are rare but they can be severe. Myasthenia gravis and inflammatory neuropathy are the most common neuromuscular adverse events but a variety of others including inflammatory myopathy are reported. The pathophysiologic mechanism of these autoimmune disorders may differ from that of non-ICPI related immune diseases...
January 17, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29341125/gut-microbiota-and-probiotics-novel-immune-system-modulators-in-myasthenia-gravis
#3
REVIEW
Elena Rinaldi, Alessandra Consonni, Elena Guidesi, Marina Elli, Renato Mantegazza, Fulvio Baggi
Gut microorganisms (microbiota) live in symbiosis with the host and influence human nutrition, metabolism, physiology, and immune development and function. The microbiota prevents pathogen infection to the host, and in turn the host provides a niche for survival. The alteration of gut bacteria composition (dysbiosis) could contribute to the development of immune-mediated diseases by influencing the immune system activation and driving the pro- and anti-inflammatory responses in order to promote or counteract immune reactions...
January 17, 2018: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/29339325/induction-of-anti-agrin-antibodies-causes-myasthenia-gravis-in-mice
#4
Min Yan, Ziyang Liu, Erkang Fei, Wenbing Chen, Xinsheng Lai, Bin Luo, Peng Chen, Hongyang Jing, Jin-Xiu Pan, Michael H Rivner, Wen-Cheng Xiong, Lin Mei
Myasthenia gravis (MG) is an autoimmune disorder ofthe neuromuscular junction (NMJ). Most cases of MG are caused by autoantibodies against the acetylcholine receptor (AChR),muscle-specific kinase (MuSK) and low-density lipoprotein receptor-related protein 4 (LRP4). Recent studies have identified anti-agrin antibodies in MG patients lacking these three antibodies (i.e., triple negative MG). Agrin is a basal lamina protein that has two isoforms. Neural agrin (N-agrin) binds to LRP4 to activate MuSK to induce AChR clusters and is thus critical for NMJ formation...
January 12, 2018: Neuroscience
https://www.readbyqxmd.com/read/29337134/acetylcholine-receptor-antibody-titers-and-clinical-course-after-influenza-vaccination-in-patients-with-myasthenia-gravis-a-double-blind-randomized-controlled-trial-propatient-trial
#5
Björn Tackenberg, Maximilian Schneider, Franz Blaes, Christian Eienbröker, Carmen Schade-Brittinger, Anne Wellek, Marcus Deschauer, Markus Eickmann, Hans-Dieter Klenk, Hans-Helge Müller, Norbert Sommer
BACKGROUND: It is a continuous matter of discussion whether immune activation by vaccination in general and Influenza vaccination in particular increases the risk for clinical deterioration of autoimmune diseases. This prospective study investigated the serological and clinical course of autoimmune Myasthenia gravis (MG) after a seasonal influenza vaccination. METHODS: This randomized, placebo-controlled, double-blind study enrolled MG patients with antibodies against acetylcholine-receptors (AChR-ab)...
January 10, 2018: EBioMedicine
https://www.readbyqxmd.com/read/29334503/family-history-of-autoimmune-diseases-and-risk-of-gastric-cancer-a-national-cohort-study
#6
Jianguang Ji, Jan Sundquist, Kristina Sundquist
A personal history of autoimmune diseases is associated with an increased incidence of gastric cancer, but whether they share familial susceptibility is still unknown. The contribution of shared environmental or genetic factors toward the observed familial aggregation has not been determined. We used a few Swedish registers, including the Swedish Multigeneration Register and the Cancer Register, to examine the familial risk of gastric cancer among individuals with a family history of a set of autoimmune diseases...
January 12, 2018: European Journal of Cancer Prevention
https://www.readbyqxmd.com/read/29334041/autoantibodies-to-low-density-lipoprotein-receptor-related-protein-4-in-double-seronegative-myasthenia-gravis-a-systematic-review
#7
Stephen Bacchi, Philippe Kramer, Colin Chalk
BACKGROUND: Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction in which a clinical diagnosis may be confirmed with serological testing. The most common autoantibodies used to support a diagnosis of MG are anti-acetylcholine receptor antibodies and anti-muscle-specific tyrosine kinase antibodies. In cases in which both of these autoantibodies are negative (termed double-seronegative [dSNMG]), other autoantibodies such as low-density lipoprotein receptor-related protein 4 (LRP4) may be used to aid in diagnosis...
January 2018: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
https://www.readbyqxmd.com/read/29333908/false-positive-acetylcholine-receptor-antibodies-in-a-case-of-unilateral-chronic-progressive-external-ophthalmoplegia-case-report-and-review-of-literature
#8
Rehan Rajput, Amun Sachdev, Nizar Din, Erika Marie Damato, Aidan Murray
METHODS: We present a rare case with atypical presenting features of unilateral CPEO with a false positive Acetylcholine Receptor Antibody (AchRA) test resulting in diagnostic delay. We illustrate the unilateral nature of this case and demonstrate the caveats of performing myogenic ptosis correction in such patients. We also discuss the differential diagnosis of false positive AchRA, a test commonly performed in the investigation of ptosis. RESULTS: A 34-year old female presented with a more than 3-year history of slowly-progressive, unilateral, right-sided restriction in eye movements and ptosis...
January 15, 2018: Orbit
https://www.readbyqxmd.com/read/29331804/toll-like-receptor-9-antagonist-suppresses-humoral-immunity-in-experimental-autoimmune-myasthenia-gravis
#9
Peng Zhang, Chun-Lin Yang, Ru-Tao Liu, Heng Li, Min Zhang, Na Zhang, Long-Tao Yue, Cong-Cong Wang, Ying-Chun Dou, Rui-Sheng Duan
Recent studies have demonstrated the important role of toll-like receptor 9 (TLR9) signalling in autoimmune diseases, but its role in myasthenia gravis (MG) has not been fully established. We show herein that blocking TLR9 signalling via the suppressive oligodeoxynucleotide (ODN) H154 alleviated the symptoms of experimental autoimmune myasthenia gravis (EAMG). With the downregulation of dendritic cells (DCs), TLR9 interruption reduced follicular helper T cells (Tfh) and germinal centre (GC) B cells, leading to decreased antibody production...
January 11, 2018: Molecular Immunology
https://www.readbyqxmd.com/read/29322496/wt1-peptide-based-immunotherapy-for-advanced-thymic-epithelial-malignancies
#10
Yusuke Oji, Masayoshi Inoue, Yoshito Takeda, Naoki Hosen, Yasushi Shintani, Manabu Kawakami, Takuya Harada, Yui Murakami, Miki Iwai, Mari Fukuda, Sumiyuki Nishida, Jun Nakata, Yoshiki Nakae, Satoshi Takashima, Toshiaki Shirakata, Hiroko Nakajima, Kana Hasegawa, Hiroshi Kida, Takashi Kijima, Soyoko Morimoto, Fumihiro Fujiki, Akihiro Tsuboi, Eiichi Morii, Satoshi Morita, Junichi Sakamoto, Atsushi Kumanogoh, Yoshihiro Oka, Meinoshin Okumura, Haruo Sugiyama
Thymic epithelial tumors are rare malignancies, and no optimal therapeutic regimen has been defined for patients with advanced disease. Patients with advanced thymic epithelial tumors, which were resistant or intolerable to prior therapies, were eligible for this study. Patients received 9 mer-WT1-derived peptide emulsified with Montanide ISA51 adjuvant via intradermal administration once a week as a monotherapy. After the 3 month-protocol treatment, the treatment was continued mostly at intervals of 2 to 4 weeks until disease progression or intolerable adverse events occurred...
January 11, 2018: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/29321572/specific-inhibition-of-acetylcholinesterase-as-an-approach-to-decrease-muscarinic-side-effects-during-myasthenia-gravis-treatment
#11
Konstantin A Petrov, Alexandra D Kharlamova, Oksana A Lenina, Ayrat R Nurtdinov, Marina E Sitdykova, Victor I Ilyin, Irina V Zueva, Evgeny E Nikolsky
Non-selective inhibitors of cholinesterases (ChEs) are clinically used for treatment of myasthenia gravis (MG). While being generally safe, they cause numerous adverse effects including induction of hyperactivity of urinary bladder and intestines affecting quality of patients life. In this study we have compared two ChEs inhibitors, a newly synthesized compound C547 and clinically used pyridostigmine bromide, by their efficiency to reduce muscle weakness symptoms and ability to activate contractions of urinary bladder in a rat model of autoimmune MG...
January 10, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29309416/long-term-safety-of-rituximab-induced-peripheral-b-cell-depletion-in-autoimmune-neurological-diseases
#12
Anza B Memon, Adil Javed, Christina Caon, Shitiz Srivastawa, Fen Bao, Evanthia Bernitsas, Jessica Chorostecki, Alexandros Tselis, Navid Seraji-Bozorgzad, Omar Khan
BACKGROUND: B-cells play a pivotal role in several autoimmune diseases, including patients with immune-mediated neurological disorders (PIMND), such as neuromyelitis optica (NMO), multiple sclerosis (MS), and myasthenia gravis (MG). Targeting B-cells has been an effective approach in ameliorating both central and peripheral autoimmune diseases. However, there is a paucity of literature on the safety of continuous B-cell depletion over a long period of time. OBJECTIVE: The aim of this study was to examine the long-term safety, incidence of infections, and malignancies in subjects receiving continuous therapy with a B-cell depleting agent rituximab over at least 3 years or longer...
2018: PloS One
https://www.readbyqxmd.com/read/29305138/musk-myasthenia-gravis-and-pregnancy
#13
Ernestina Santos, Antonio Braga, Denis Gabriel, Sara Duarte, Ana Martins da Silva, Ilda Matos, Marta Freijo, Joao Martins, Fernando Silveira, Goreti Nadais, Filipa Sousa, Carla Fraga, Rosa Santos Silva, Carlos Lopes, Guilherme Gonçalves, Clara Pinto, Jorge Sousa Braga, Maria Isabel Leite
Muscle specific kinase (MuSK) myasthenia gravis (MG, MuSK-MG) is a rare subgroup of MG affecting mainly women during childbearing years. We investigated the influence of pregnancy in the course of MuSK-MG and pregnancy outcomes in females with MuSK-MG. A multicentre cohort of 17 women with MuSK-MG was studied retrospectively; 13 of them with ≥1 pregnancy. MuSK-MG onset age was 35,4 years; 23,0% had other autoimmune disorder; 46,2% were treatment refractory. Thirteen women experienced 27 pregnancies, either after MG onset (group I) (n = 4; maternal age at conception = 29...
November 28, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29246598/intravenous-immunoglobulin-as-monotherapy-for-myasthenia-gravis-during-pregnancy
#14
Josep Gamez, Maria Salvado, Manel Casellas, Susana Manrique, Felix Castillo
INTRODUCTION: Pregnant women with myasthenia gravis (MG) are at increased risk of complications and adverse outcomes, including the teratogenic effects of many drugs used to treat MG women of childbearing age. The effectiveness of intravenous immunoglobulins (IVIg) on other autoimmune mediated diseases has been extensively reported in recent years, although little is known about the role of IVIg in the treatment of MG during pregnancy. We designed this study to determine the effectiveness of IVIg as monotherapy during pregnancy for women with MG...
December 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29245252/graft-versus-host-disease-like-erythroderma-a-sign-of-recurrent-thymoma-a-case-report
#15
Xiujuan Gui, Xinhai Zhu, Liangjun Guo, Guoqiang Tan, Yan Liu, Yi Tan, Qiufang Chen, Yuwei Song, Shaoqiang Lin
RATIONALE: Thymomas are associated with numerous autoimmune disorders, such as myasthenia gravis (MG), pure red cell aplasia (PRCA), and systemic lupus erythematosus (SLE). However, graft-versus-host disease (GVHD)-like erythroderma is a relatively uncommon paraneoplastic disorder associated with thymomas and signifies a poor prognosis. PATIENT CONCERNS: A 35-year-old woman with medical history significant for stage IVa type AB thymoma presented with patchy erythema over face, trunk, and extremities that failed to respond to topical steroids...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29230265/-juvenile-myasthenia-gravis-in-sub-saharan-africa-a-case-study-of-two-consanguine-sisters-born-from-consanguinity-in-togo
#16
Nidain Maneh, Kossivi Apetse, Bénédicte Marèbe Diatewa, Sidik Abou-Bakr Domingo, Aidé Isabelle Agba, Koffi Didier Ayena, Koffi Agnon Balogou, Komi Patrice Balo
Myasthenia gravis is a rare acquired autoimmune pathology causing neuromuscular transmission impairment. Juvenile onset of myasthenia gravis is often characterized by ocular involvement. We report two cases of ocular juvenile myasthenia gravis (JMG) in two siblings. They were two young girls, XA and XB, aged 11 and 9 years, of Malian origin, residing in Togo, born from first-degree of consanguinity presenting to Ophthalmology due to progressive decrease in visual acuity. XA showed visual acuity 8/10 on both eyes while XB showed improvement in visual acuity from 3/10 to 7/10 using a pinhole occluder, suggesting ametropia...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/29224277/-clinicopathologic-analysis-of-micronodular-thymoma-with-lymphoid-stroma
#17
X Y Wang, M Xu, L G Shi, Y Z Ding, Q Cheng, Y W Zhao
Objective: To investigate the clinicopathologic features of micronodular thymoma with lymphoid stroma(MNT). Methods: Five cases of MNT diagnosed from January 2007 to December 2016 in Henan Provincial People's Hospital were collected.Hematoxylin-Eosin staining and immunohistochemistry were used to evaluate the histological and immunophenotypic characteristics in 5 MNT cases. Epstein-Barr virus (EBV) status was detected by in situ hybridization for EBV-encoded small RNA (EBER). Polymerase chain reaction was used to detect the rearrangement of immunoglobulin genes...
December 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29221139/epstein-barr-virus-in-tumor-infiltrating-b-cells-of-myasthenia-gravis-thymoma-an-innocent-bystander-or-an-autoimmunity-mediator
#18
Paola Cavalcante, Stefania Marcuzzo, Sara Franzi, Barbara Galbardi, Lorenzo Maggi, Teresio Motta, Raffaella Ghislandi, Antonella Buzzi, Luisella Spinelli, Lorenzo Novellino, Fulvio Baggi, Carlo Antozzi, Fabio Conforti, Tommaso Martino De Pas, Massimo Barberis, Pia Bernasconi, Renato Mantegazza
The thymus plays a key role in myasthenia gravis (MG), a B cell-mediated autoimmune disorder affecting neuromuscular junction. Most MG patients have thymic abnormalities, including hyperplasia and thymoma, a neoplasm of thymic epithelial cells. Epstein-Barr virus (EBV) is associated with autoimmune diseases and tumors. Recently, we showed EBV persistence and reactivation in hyperplastic MG thymuses, suggesting that EBV might contribute to intra-thymic B cell dysregulation in MG patients. Here, we investigated EBV involvement in thymoma-associated MG, by searching for EBV markers in MG (n=26) and non-MG (n=14) thymomas...
November 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/29211921/infections-and-the-relationship-to-treatment-in-neuromuscular-autoimmunity
#19
Devin E Prior, Emily Nurre, Stephanie L Roller, David Kline, Ramit Panara, Amro M Stino, John A Davis, Miriam L Freimer, W David Arnold
INTRODUCTION: This study aimed to identify infections in patients with myasthenia gravis, dermatomyositis and chronic inflammatory demyelinating polyradiculoneuropathy and investigate the relationship between infection and immunomodulation. METHODS: A retrospective chart review examined 631 patients with myasthenia gravis (n=358), chronic inflammatory demyelinating polyradiculoneuropathy (n=124), and dermatomyositis (n=149) patients over a 10 year time period. RESULTS: Infection rates were similar at approximately 19% in all three diseases...
December 6, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/29205338/s1p-receptor-antagonists-fingolimod-and-siponimod-do-not-improve-the-outcome-of-experimental-autoimmune-myasthenia-gravis-mice-after-disease-onset
#20
Andreas Pelz, Hanne Schaffert, Radharani Diallo, Falk Hiepe, Andreas Meisel, Siegfried Kohler
Myasthenia gravis (MG) is an autoimmune disease characterized by muscle weakness and fatigue in the presence of circulating antibodies against components of the neuromuscular junction. Most patients have a good prognosis, but some are refractory to standard-of-care immunosuppressive treatment and suffer from recurrent myasthenic crises. Functional sphingosine-1-phosphate (S1P) antagonists like fingolimod and siponimod (BAF312) are successfully used for the treatment of multiple sclerosis, and fingolimod was shown to prevent the development of myasthenic symptoms in experimental autoimmune myasthenia gravis (EAMG), the standard model of MG...
December 4, 2017: European Journal of Immunology
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