Read by QxMD icon Read

Autoimmune myasthenia

S Martínez Torre, I Gómez Molinero, R Martínez Girón
Myasthenia gravis is one of the most common disorders that affect neuromuscular transmission. It is currently one of the most understood and characterised autoimmune disorders Its typical symptoms are fluctuating weakness and fatigue that affects a combination of ocular muscles, bulbar functions, as well as limb and respiratory muscles, which are due to an immune attack against the postsynaptic membrane of the neuromuscular junction. The diagnosis of myasthenia gravis is based on clinical and serological test...
March 16, 2018: Semergen
Kee Hong Park, Patrick Waters, Mark Woodhall, Bethan Lang, Thomas Smith, Jung-Joon Sung, Kwang-Kuk Kim, Young-Min Lim, Jee-Eun Kim, Byung-Jo Kim, Jin-Sung Park, Jeong-Geon Lim, Dae-Seong Kim, Ohyun Kwon, Eun Hee Sohn, Jong Seok Bae, Byung-Nam Yoon, Nam-Hee Kim, Suk-Won Ahn, Jeeyoung Oh, Hyung Jun Park, Kyong Jin Shin, Yoon-Ho Hong
Acquired myasthenia gravis (MG) is a prototype autoimmune disease of the neuromuscular junction, caused in most patients by autoantibodies to the muscle nicotinic acetylcholine receptor (AChR). There seem to be ethnic and regional differences in the frequency and clinical features of MG seronegative for the AChR antibody. This study aimed to describe the autoantibody profiles and clinical features of Korean patients with generalized MG seronegative for the AChR antibody. A total of 62 patients with a high index of clinical suspicion of seronegative generalized MG were identified from 18 centers, and we examined their sera for antibodies to clustered AChR, muscle-specific tyrosine kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (LRP4) by cell-based assays (CBA) and to MuSK by radioimmunoprecipitation assay (RIPA)...
2018: PloS One
Inga Koneczny
IgG4 autoimmune diseases are characterized by the presence of antigen-specific autoantibodies of the IgG4 subclass and contain well-characterized diseases such as muscle-specific kinase myasthenia gravis, pemphigus, and thrombotic thrombocytopenic purpura. In recent years, several new diseases were identified, and by now 14 antigens targeted by IgG4 autoantibodies have been described. The IgG4 subclass is considered immunologically inert and functionally monovalent due to structural differences compared to other IgG subclasses...
2018: Frontiers in Immunology
Enrique M San Norberto, Irene García-Saiz, Diana Gutiérrez, Liliana Domingos, Carlos Vaquero
The non-VKA oral anticoagulant rivaroxaban is indicated in prevention and treatment of venous thromboembolism. A 60-year-old male patient complained of bilateral ptosis after administration of rivaroxaban for deep vein thrombosis. Myasthenia gravis was confirmed by positive serum antiacetylcholine receptor antibody test. No mediastinal thymoma was found. The ocular myasthenia reversed after discontinuing rivaroxaban treatment. Nevertheless, ptosis recurred and chronic oral pyridostigmine bromide treatment was necessary...
February 15, 2018: Annals of Vascular Surgery
Milan Radovich, Curtis R Pickering, Ina Felau, Gavin Ha, Hailei Zhang, Heejoon Jo, Katherine A Hoadley, Pavana Anur, Jiexin Zhang, Mike McLellan, Reanne Bowlby, Thomas Matthew, Ludmila Danilova, Apurva M Hegde, Jaegil Kim, Mark D M Leiserson, Geetika Sethi, Charles Lu, Michael Ryan, Xiaoping Su, Andrew D Cherniack, Gordon Robertson, Rehan Akbani, Paul Spellman, John N Weinstein, D Neil Hayes, Ben Raphael, Tara Lichtenberg, Kristen Leraas, Jean Claude Zenklusen, Junya Fujimoto, Cristovam Scapulatempo-Neto, Andre L Moreira, David Hwang, James Huang, Mirella Marino, Robert Korst, Giuseppe Giaccone, Yesim Gokmen-Polar, Sunil Badve, Arun Rajan, Philipp Ströbel, Nicolas Girard, Ming S Tsao, Alexander Marx, Anne S Tsao, Patrick J Loehrer
Thymic epithelial tumors (TETs) are one of the rarest adult malignancies. Among TETs, thymoma is the most predominant, characterized by a unique association with autoimmune diseases, followed by thymic carcinoma, which is less common but more clinically aggressive. Using multi-platform omics analyses on 117 TETs, we define four subtypes of these tumors defined by genomic hallmarks and an association with survival and World Health Organization histological subtype. We further demonstrate a marked prevalence of a thymoma-specific mutated oncogene, GTF2I, and explore its biological effects on multi-platform analysis...
February 12, 2018: Cancer Cell
Ayumi Mitsune, Satoru Yanagisawa, Tatsuro Fukuhara, Eisaku Miyauchi, Mami Morita, Manabu Ono, Yutaka Tojo, Masakazu Ichinose
Nivolumab is a newly introduced promising therapy for treating lung cancer that restores the anti-tumor immunity by disrupting programmed cell death (PD)-1-mediated immuno-suppressive signaling. Although "new-onset" autoimmune diseases are well-known immune-related adverse events (irAEs), whether or not nivolumab exacerbates "pre-existing" autoimmune disease remains unclear. We herein report a patient with "pre-existing" myasthenia gravis (MG) in whom nivolumab was administered that flared up after the treatment with nivolumab...
February 9, 2018: Internal Medicine
Jianwen Wang, Yatao Xiao, Kejing Zhang, Benyan Luo, Chengyong Shen
The neuromuscular junction (NMJ) is a peripheral synapse between motor neurons and skeletal muscle fibers that controls muscle contraction. The NMJ is the target of various disorders including myasthenia gravis (MG), an autoimmune disease in which auto-antibodies (auto-Abs) attack the synapse, and thus cause muscle weakness in patients. There are multiple auto-Abs in the MG patient sera, but not all the Abs are proven to be pathogenic, which increases the difficulties in clinical diagnoses and treatments. To establish the causative roles of auto-Abs in MG pathogenesis, the experimental autoimmune MG (EAMG) induced by the active immunization of auto-antigens (auto-Ags) or the passive transfer of auto-Abs is required...
February 5, 2018: Neuroscience
Mina Hosseini, Behrouz Robat-Jazi, Vahid Shaygannejad, Shahriar Naffisi, Omid Mirmossayeb, Abbas Rezaei, Marjan Mansourian, Nafiseh Esmaeil
OBJECTIVE: Myasthenia gravis (MG) is an autoimmune disease mediated by autoantibodies against the neuromuscular junction. The thymus has an important role in the pathogenesis of MG because most patients have thymic pathology, and thymectomy (TE) can reduce the severity of the disease. METHODS: In this study, the frequency of Th17 and Tc17 cells was studied in 12 MG patients (pre-TE and 6 months post-TE) and in 12 healthy controls (HC). RESULTS: The frequency of Tc17 cells in the pre-TE patients was significantly higher than in the HC (p < 0...
February 8, 2018: Neuroimmunomodulation
Jose Adolfo Villegas, Jérôme Van Wassenhove, Rozen Le Panse, Sonia Berrih-Aknin, Nadine Dragin
A chronic autoimmune disease, myasthenia gravis (MG) is characterized in 85% of patients by antibodies directed against the acetylcholine receptor (AChR) located at the neuromuscular junction. The functional and effective balance between regulatory T cells (Treg cells) and effector T cells (Teff cells) is lost in the hyperplastic thymus of MG patients with antibodies specific for the AChR (AChR+ MG patients). The objective of this review is to describe how Treg cells and inflammatory T cells participate in this imbalance and contribute to induce a chronic inflammatory state in the MG thymus...
February 2018: Annals of the New York Academy of Sciences
Nayara Felicidade Tomaz Braz, Natalia Pessoa Rocha, Érica Leandro Marciano Vieira, Izabela Guimarães Barbosa, Rodrigo Santiago Gomez, Adriana Maria Kakehasi, Antonio Lucio Teixeira
Myasthenia gravis (MG) is a neuromuscular autoimmune disease characterized by skeletal muscle weakness which can impact motor function and, furthermore, produce negative impact on the health-related quality of life (HRQOL). OBJECTIVE: To evaluate the predictors for HRQOL in patients with MG. METHODS: Eighty patients were evaluated with the MG Foundation of America classification and the MG Composite scale. HRQOL was estimated by the MGQOL15, while anxious and depressive symptoms were evaluated with the Hospital Anxiety and Depression Scale (HAD)...
January 22, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Sa-Yoon Kang, Ji-Hoon Kang, Jay Chol Choi, Sook Keun Song, Jung-Hwan Oh
Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disease. Vitamin D has important roles both in the autoimmune response and in skeletal muscles. We investigated the levels of 1,25-dihydroxy vitamin D [1,25(OH)2D] and 25-hydroxy vitamin D [25(OH)D] in patients with MG and healthy subjects. MG patients were classified by disease stage, age of onset and treatment status whether or not to taking immunosuppressive agents. MG patients had lower plasma 25(OH)D levels (mean, 18.8 ± 8.4 ng/mL) than healthy controls (26...
January 26, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Jyh Yung Hor, Thien Thien Lim, Mei Chih Cheng, Yuen Kang Chia, Chee Keong Wong, Su Min Lim, Chun Fai Cheah, Kenny Tan, P E Samuel Easaw, M Isabel Leite
Thymoma is associated with a wide spectrum of autoimmune paraneoplastic syndromes, though it is uncommon for multiple paraneoplastic syndromes to be present in a single individual. We report a rare case of an elderly gentleman who was found to have thymoma-associated myasthenia gravis and LGI1-encephalitis with myokymia, who presented with nephrotic syndrome (minimal change glomerulopathy) after thymectomy. The latter two paraneoplastic syndromes had manifested when prednisolone was tapered down to low dose...
January 30, 2018: Journal of Neuroimmunology
Manon Lee, David Beeson, Jacqueline Palace
To date, more than 25 genes have been implicated in the etiology of the congenital myasthenic syndromes (CMS), and an ever-growing phenotypic landscape is now encountered in the CMS clinic. Unlike the autoimmune form of myasthenia, there is no role for immunomodulatory agents in the treatment of CMS. The present-day drug repertoire comprises acetylcholinesterase inhibitors (mainly pyridostigmine), 3,4-diaminopyridine (3,4-DAP), ephedrine, salbutamol/albuterol, open-channel blockers (fluoxetine, quinidine), or a combination of these...
January 2018: Annals of the New York Academy of Sciences
Min Yan, Guang-Lin Xing, Wen-Cheng Xiong, Lin Mei
Myasthenia gravis (MG) is a common disorder that affects the neuromuscular junction. It is caused by antibodies against acetylcholine receptor and muscle-specific tyrosine kinase; however, some MG patients do not have antibodies against either of the proteins. Recent studies have revealed antibodies against agrin and its receptor LRP4-both critical for neuromuscular junction formation and maintenance-in MG patients from various populations. Results from experimental autoimmune MG animal models indicate that anti-LRP4 antibodies are causal to MG...
January 28, 2018: Annals of the New York Academy of Sciences
Jie Luo, Jon Lindstrom
Experimental autoimmune myasthenia gravis (EAMG) and myasthenia gravis (MG) are caused by autoantibodies to the extracellular domain of muscle nicotinic acetylcholine receptors (AChRs). Autoantibodies to the cytoplasmic domain of AChRs do not cause EAMG because they cannot bind AChRs in vivo. The ideal MG therapy would quickly and permanently suppress only the pathological autoimmune response to AChRs. We have developed a specific immunosuppressive therapy for EAMG that involves immunizing rats with bacterially expressed cytoplasmic domains of human muscle AChRs...
January 29, 2018: Annals of the New York Academy of Sciences
Muriel Sudres, Julien Verdier, Frédérique Truffault, Rozen Le Panse, Sonia Berrih-Aknin
Autoimmune diseases (AIDs) are chronic disorders characterized by inflammatory reactions against self-antigens that can be either systemic or organ specific. AIDs can differ in their epidemiologic features and clinical presentations, yet all share a remarkable complexity. AIDs result from an interplay of genetic and epigenetic factors with environmental components that are associated with imbalances in the immune system. Many of the pathogenic mechanisms of AIDs are also implicated in myasthenia gravis (MG), an AID in which inflammation of the thymus leads to a neuromuscular disorder...
January 28, 2018: Annals of the New York Academy of Sciences
Maartje G Huijbers, Jaap J Plomp, Silvère M van der Maarel, Jan J Verschuuren
Immunoglobulin 4 (IgG4) is one of four human IgG subclasses and has several unique functional characteristics. It exhibits low affinity for complement and for most Fc receptors. It furthermore has generally high affinity for its antigen, with binding occurring in a monovalent fashion, as IgG4 can exchange Fab-arms with other IgG4 molecules. Because of these characteristics, IgG4 is believed to block its targets and prevent inflammation, which, depending on the setting, can have a protective or pathogenic effect...
January 28, 2018: Annals of the New York Academy of Sciences
Yuichi Muraki, Shugo Mizuno, Kaname Nakatani, Hiroki Wakabayashi, Eiji Ishikawa, Toshimitsu Araki, Akira Taniguchi, Shuji Isaji, Masahiro Okuda
A total of 25 patients with autoimmune diseases receiving tacrolimus were screened using a peripheral blood cluster of differentiation 4+ adenosine triphosphate (ATP) activity assay (IMK assay) between October 2013 and July 2014. The autoimmune diseases of patients were as follows: Rheumatoid arthritis (n=15), lupus nephritis (n=6), ulcerative colitis (n=2) and myasthenia gravis (n=2). Patients were divided into two groups based on CYP3A5 genotype [expression of *1 allele: Expressor (EX; n=6) and non-expressor (NEX; n=19)]...
January 2018: Experimental and Therapeutic Medicine
Nils Erik Gilhus, Fredrik Romi, Yu Hong, Geir Olve Skeie
BACKGROUND AND PURPOSE: Myasthenia gravis (MG) is an autoimmune disease with muscular weakness as the only symptom, and often with immunosuppressive treatment. All these aspects could have relevance for the risk of infections as well as their prophylactic and curative treatment. METHODS: This is a review article, where Web of Science has been searched for relevant key words and key word combinations. Full papers were selected first by title and then by abstract...
January 25, 2018: Journal of Neurology
Mariapaola Marino, Emanuela Bartoccioni, Paolo Emilio Alboini, Amelia Evoli
In recent years, rituximab (RTX), a monoclonal antibody that binds the B lymphocyte membrane protein CD20, has been increasingly used for the treatment of autoimmune diseases, with the rationale of destroying pathogenic B lymphocytes and decreasing autoantibody formation. Surprisingly, RTX has also proven effective in predominantly T cell-mediated diseases, raising the question whether additional mechanisms may play roles in determining the therapeutic response. Here, we review the current literature on the effects of RTX in autoimmune diseases, with special emphasis on myasthenia gravis (MG)...
January 25, 2018: Annals of the New York Academy of Sciences
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"