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myasthenia gravis recognition

André P C Matta, Ana C Andorinho F Ferreira, Arielle Kirmse, Anna Carolina Damm, João Gabriel D I B Farinhas, Mariane D Barbosa, Mayara C M Teles, Camila Fiorelli, Rossano Fiorelli, Osvaldo J M Nascimento, Marco Orsini
Myasthenia gravis (MG) is a relatively uncommon disorder with an annual incidence of approximately 7 to 9 new cases per million. The prevalence is about 70 to 165 per million. The prevalence of the disease has been increasing over the past five decades. This is thought to be due to better recognition of the condition, aging of the population, and the longer life span of affected patients. MG causes weakness, predominantly in bulbar, facial, and extra-ocular muscles, often fluctuating over minutes to weeks, in the absence of wasting, sensory loss, or reflex changes...
November 2, 2016: Neurology International
Katherine L March, Michael J Samarin, Amik Sodhi, Ryan E Owens
Purpose Pembrolizumab, a monoclonal antibody which inhibits the programmed cell death 1 receptor, has been shown to efficaciously enhance pre-existing immune responses to malignancies. However, safety concerns must also be considered as pembrolizumab use has been associated with several life-threatening immune-related adverse events (irAEs). We report a fatal case of pembrolizumab-induced myasthenia gravis in a patient with no prior myasthenia gravis history. Case report A 63-year-old male presented with right eyelid drooping, puffiness, blurred vision, and shortness of breath two weeks after an initial infusion of pembrolizumab...
January 1, 2017: Journal of Oncology Pharmacy Practice
Crystal R Sundburg, Janelle M Belanger, Danika L Bannasch, Thomas R Famula, Anita M Oberbauer
BACKGROUND: Gonadectomy is one of the most common procedures performed on dogs in the United States. Neutering has been shown to reduce the risk for some diseases although recent reports suggest increased prevalence for structural disorders and some neoplasias. The relation between neuter status and autoimmune diseases has not been explored. This study evaluated the prevalence and risk of atopic dermatitis (ATOP), autoimmune hemolytic anemia (AIHA), canine myasthenia gravis (CMG), colitis (COL), hypoadrenocorticism (ADD), hypothyroidism (HYPO), immune-mediated polyarthritis (IMPA), immune-mediated thrombocytopenia (ITP), inflammatory bowel disease (IBD), lupus erythematosus (LUP), and pemphigus complex (PEMC), for intact females, intact males, neutered females, and neutered males...
December 8, 2016: BMC Veterinary Research
Michael W Nicolle
PURPOSE OF REVIEW: This article discusses the pathogenesis, diagnosis, and management of autoimmune myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS). RECENT FINDINGS: Recognition of new antigenic targets and improved diagnostic methods promise to improve the diagnosis of MG, although the clinical phenotypes associated with newer antibodies have not yet been defined. Future therapies might specifically target the aberrant immune response. The apparent increase in the prevalence of MG is not fully explained...
December 2016: Continuum: Lifelong Learning in Neurology
Alexis A Lizarraga, Karlo J Lizarraga, Michael Benatar
After prompt diagnosis, severe myasthenia gravis and Guillain-Barré syndrome (GBS) usually require management in the intensive care unit. In the myasthenic patient, recognition of precipitating factors is paramount, and frequent monitoring of bulbar, upper airway, and/or respiratory muscle strength is needed to identify impending myasthenic crisis. Noninvasive ventilation can be attempted prior to intubation and mechanical ventilation in the setting of respiratory failure. Cholinesterase inhibitors should be discontinued, but resumed prior to extubation, and steroid dosage could be increased once the airway is secured...
December 2016: Seminars in Neurology
Daniel B Drachman
The basic abnormality in myasthenia gravis (MG) is a reduction in acetylcholine receptors (AChRs) at neuromuscular junctions due to the effects of autoantibodies that are directed against the AChRs in most patients, or against neighboring proteins involved in the clustering of AChRs (MuSK, LRP-4, or agrin). Clinically, MG is characterized by muscle weakness and fatigue, often in typical patterns. The diagnosis may be missed early, and depends on the recognition of clinical manifestations, the measurement of autoantibodies, and/or electrophysiological features...
October 2016: Seminars in Neurology
Francesc Miralles
Myasthenia gravis (MG) is an autoimmune postsynaptic disorder of neuromuscular transmission caused, in most patients, by antibodies against postsynaptic acetylcholine receptors. Lambert-Eaton myasthenic syndrome (LEMS) is a presynaptic autoimmune disease in which there is a reduction in Ca(2+) entry with each impulse due to the action of antibodies against Ca(2+) channels. These diseases have a distinct pattern of response to low-frequency repetitive nerve stimulation which allows its recognition in a particular subject...
November 2016: Medical & Biological Engineering & Computing
Bo Hu, Katja Simon-Keller, Stefan Küffer, Philipp Ströbel, Thomas Braun, Alexander Marx, Stefan Porubsky
Myasthenia gravis (MG) is caused by autoantibodies against the neuromuscular junction of striated muscle. Most MG patients have autoreactive T- and B-cells directed to the acetylcholine receptor (AChR). To achieve immunologic tolerance, developing thymocytes are normally eliminated after recognition of self-antigen-derived peptides. Presentation of muscle-specific antigens is likely achieved through two pathways: on medullary thymic epithelial cells and on medullary dendritic cells cross-presenting peptides derived from a unique population of thymic myoid cells (TMC)...
March 2016: Experimental Neurology
Danilo R Bernardo, Nizar Chahin
OBJECTIVE: To show that immunotherapy with medications such mycophenolate mofetil (MMF) can cause serious complications in patients with neuromuscular disorders. METHODS: Two patients with neuromuscular disorders on immunotherapy with long-term MMF who developed toxoplasmic encephalitis (TE) were included in this case series. RESULTS: One patient with myasthenia gravis and one patient with inflammatory myopathy on immunotherapy with long-term MMF developed severe TE...
February 2015: Neurology® Neuroimmunology & Neuroinflammation
Yurika Watanabe, Shigeaki Suzuki, Hiroaki Nishimura, Ken-ya Murata, Takashi Kurashige, Masamichi Ikawa, Masaru Asahi, Hirofumi Konishi, Satsuki Mitsuma, Satoshi Kawabata, Norihiro Suzuki, Ichizo Nishino
Statins have a variety of myotoxic effects and can trigger the development of inflammatory myopathies or myasthenia gravis (MG) mediated by immunomodulatory properties. Autoantibodies to 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) have been identified in patients with statin-associated myopathy. The purpose of the present study is to develop an enzyme-linked immunosorbent assay (ELISA) of anti-HMGCR antibodies and to elucidate the clinical significance of anti-HMGCR antibodies in Japanese patients with inflammatory myopathies or MG...
January 2015: Medicine (Baltimore)
Jacqueline Kerr, Isabella Quinti, Martha Eibl, Helen Chapel, Peter J Späth, W A Carrock Sewell, Abdulgabar Salama, Ivo N van Schaik, Taco W Kuijpers, Hans-Hartmut Peter
The consumption of immunoglobulins (Ig) is increasing due to better recognition of antibody deficiencies, an aging population, and new indications. This review aims to examine the various dosing regimens and research developments in the established and in some of the relevant off-label indications in Europe. The background to the current regulatory settings in Europe is provided as a backdrop for the latest developments in primary and secondary immunodeficiencies and in immunomodulatory indications. In these heterogeneous areas, clinical trials encompassing different routes of administration, varying intervals, and infusion rates are paving the way toward more individualized therapy regimens...
2014: Frontiers in Immunology
James Huang, Usman Ahmad, Alberto Antonicelli, Ann Christine Catlin, Wentao Fang, Daniel Gomez, Patrick Loehrer, Marco Lucchi, Edith Marom, Andrew Nicholson, Enrico Ruffini, William Travis, Paul Van Schil, Heather Wakelee, Xiaopan Yao, Frank Detterbeck
BACKGROUND: Our knowledge of thymic malignancies has largely been derived from small, single-institution series. Recognition of the need for broad collaboration led to the creation of the International Thymic Malignancy Interest Group (ITMIG) and the development of a large, centralized database to advance knowledge of these rare tumors. METHODS: A multidisciplinary Database Committee was convened to define a common set of data elements a priori. Retrospective data were solicited from ITMIG members and collated using standardized fields...
October 2014: Journal of Thoracic Oncology
Amelia Evoli, Eric Lancaster
Thymic malignancy is often associated with paraneoplastic neurological diseases (PNDs) and recognition of these disorders is important for physicians who treat these patients. The most common thymoma-associated PNDs are myasthenia gravis (MG), acquired neuromyotonia (Isaacs' syndrome), encephalitis, Morvan's syndrome, and myositis. Diagnosis of these disorders is complex but often aided by testing for specific autoantibodies, including those to the acetylcholine receptor for MG and to contactin-associated protein-like 2, protein of the voltage-gated potassium channel complex, in patients with acquired neuromyotonia, Morvan's syndrome, or encephalitis...
September 2014: Journal of Thoracic Oncology
Maryann C Deak, Douglas B Kirsch
Sleep-related breathing disorder or sleep-disordered breathing (SDB) encompasses central sleep apnea (CSA), obstructive sleep apnea (OSA), and sleep-related hypoventilation or hypoxemic syndromes. SDB is common in neurologic conditions that affect the central and/or peripheral nervous systems. Patients with neurologic conditions are at risk for SDB due to a combination of factors such as muscular weakness, damage to areas of the brain that control respiration, use of sedating medications, and weight gain from limited physical activity...
September 2014: Clinics in Chest Medicine
Zsuzsanna Pal, László Tóthfalusi, Zsolt Lörincz, Bence György, Maria Judit Molnar, Andras Falus, Edit I Buzás
Increasing number of evidences support the role of glycosylation in the evolution of autoimmunity. We examined carbohydrate-reactive natural autoantibodies systematically for the first time in patients with autoimmune myasthenia gravis. Antibodies reactive to glycosaminoglycans were measured with CovaLink ELISA in the sera of 59 myasthenia patients as well as in 54 healthy controls. We used the GlycoChip carbohydrate array to characterize individual carbohydrate recognition patterns. Chondroitin-sulphate C and anti-α-mannose-specific IgG levels were significantly elevated in myasthenia patients...
November 15, 2014: Journal of Neuroimmunology
Anand Iyer, Liene Elsone, Richard Appleton, Anu Jacob
Neuromyelitis optica (NMO) is an immune-mediated neurological disorder characterised by recurrent episodes of optic neuritis and longitudinally extensive transverse myelitis. A serum biomarker, aquaporin-4 IgG, the autoantibody against aquaporin-4 water channel, has been specifically associated with NMO and has assisted early recognition and prediction of relapses. Less commonly, a monophasic course, associated with antibodies to myelin oligodendrocyte glycoprotein has been reported. Specific diagnostic criteria have been defined; however, some cases that do not fulfil these criteria (but are nevertheless associated with aquaporin-4 IgG) are classified as NMO spectrum disorder and follow the same relapsing course...
May 2014: Autoimmunity
Bing Liao, Sheetal Shroff, Carlos Kamiya-Matsuoka, Sudhakar Tummala
BACKGROUND: Ipilimumab is a novel FDA-approved recombinant human monoclonal antibody that blocks cytotoxic T-lymphocyte antigen-4 and has been used to treat patients with metastatic melanoma. Immune-related neurological adverse effects include inflammatory myopathy, aseptic meningitis, posterior reversible encephalopathy syndrome, Guillain-Barré syndrome, myasthenia gravis-type syndrome, sensorimotor neuropathy, and inflammatory enteric neuropathy. To date, there is no report for ipilimumab-induced chronic inflammatory demyelinating polyneuropathy (CIDP), transverse myelitis (TM), or concurrent myositis and myasthenia gravis-type syndrome...
April 2014: Neuro-oncology
Stuart W Morell, Vu B Trinh, Eswari Gudipati, Alexander Friend, Nelson A Page, Mark A Agius, David P Richman, Robert H Fairclough
To develop antigen-specific immunotherapies for autoimmune diseases, knowledge of the molecular structure of targeted immunological hotspots will guide the production of reagents to inhibit and halt production of antigen specific attack agents. To this end we have identified three noncontiguous segments of the Torpedo nicotinic acetylcholine receptor (AChR) α-subunit that contribute to the conformationally sensitive immunological hotspot on the AChR termed the main immunogenic region (MIR): α(1-12), α(65-79), and α(110-115)...
March 2014: Molecular Immunology
Julie A Koch, Marlee R Steele, Logan M Koch
Myasthenia gravis (MG) is a chronic neuromuscular disorder characterized by fluctuating skeletal muscle weakness and fatigue. This rare autoimmune condition can present a diagnostic challenge for the advanced practice nurse (APN). Because of its low incidence in clinical practice and frequently unrecognized symptoms, a delay of 1 to 2 years prior to diagnosis is not uncommon. Recognition of the clinical features is a key component of prompt diagnosis. To avoid delay in treatment, APNs should be aware of variations in MG symptoms and the tools used to confirm a suspected diagnosis...
December 2013: Journal of Gerontological Nursing
John H Pula, Angela M Kao, Jorge C Kattah
PURPOSE OF REVIEW: Systemic medications may cause side-effects manifesting primarily as neuro-ophthalmologic problems. It is paramount for the physician to be updated on both well recognized and novel associations between drugs and their potential adverse reactions. RECENT FINDINGS: There is a growing list of medications that can cause pupil dilation, pupil constriction, dyschromatopsia, worsening of ocular myasthenia gravis, posterior reversible leukoencephalopathy syndrome, pseudotumor cerebri, disturbances in eye movements, accommodation problems, or optic neuropathy...
November 2013: Current Opinion in Ophthalmology
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