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https://www.readbyqxmd.com/read/28635482/diagnosis-and-management-of-myasthenia-gravis
#1
Christopher Barber
Myasthenia gravis is a rare long-term neurological condition that is characterised by fluctuating skeletal muscle weakness and fatigue, as well as respiratory difficulties. It is both an acquired autoimmune disease and a chronic neuromuscular disorder. Because of its rarity, myasthenia gravis is relatively unknown and may be unfamiliar to many nurses. While there are various types of myasthenia, this article focuses on myasthenia gravis, exploring its symptoms, diagnosis and treatment, and examining the nurse's role in managing the condition...
June 21, 2017: Nursing Standard
https://www.readbyqxmd.com/read/28628938/outcome-after-robotic-assisted-thymectomy-in-children-and-adolescents-with-acetylcholine-receptor-antibody-positive-juvenile-myasthenia-gravis
#2
Adela Della Marina, Heike Kölbel, Maximilian Müllers, Olaf Kaiser, Mahmoud Ismail, Marc Swierzy, Jens-Carsten Rueckert, Ulrike Schara
No abstract text is available yet for this article.
June 19, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28627279/maternal-autoimmune-disorders-and-fetal-defects
#3
Anca Maria Panaitescu, Kypros Nicolaides
Maternal autoantibodies can cross the placenta and cause fetal damage. This article summarizes the development and management of fetal thyroid goiter in response to maternal Graves' disease and/or its treatment with antithyroid medication, fetal heart block due to maternal anti-Ro and anti-La antibodies, fetal athrogryposis multiplex congenita in association with maternal myasthenia gravis and fetal brain hemorrhage due to maternal autoimmune thrombocytopenia.
June 18, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28626780/igg-specific-cell-based-assay-detects-potentially-pathogenic-musk-abs-in-seronegative-mg
#4
Saif Huda, Patrick Waters, Mark Woodhall, Maria Isabel Leite, Leslie Jacobson, Anna De Rosa, Michelangelo Maestri, Roberta Ricciardi, Jeannine M Heckmann, Angelina Maniaol, Amelia Evoli, Judy Cossins, David Hilton-Jones, Angela Vincent
OBJECTIVE: To increase the detection of MuSK-Abs using a CBA and test their pathogenicity. METHODS: Sera from 69 MuSK-RIA-positive patients with myasthenia gravis (MG) (Definite MuSK-MG), 169 patients negative for MuSK-RIA and AChR-RIA (seronegative MG, SNMG), 35 healthy individuals (healthy controls, HCs), and 16 NMDA receptor-Ab-positive (NMDAR-Ab) disease controls were tested for binding to MuSK on a CBA using different secondary antibodies. RESULTS: Initially, in addition to 18% of SNMG sera, 11% of HC and 19% of NMDAR-Ab sera showed positive binding to MuSK-transfected cells; this low specificity was due to anti-IgG(H+L) detection of IgM bound nonspecifically to MuSK...
July 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28625092/factors-affecting-outcome-in-ocular-myasthenia-gravis
#5
Marco Mazzoli, Alessandra Ariatti, Franco Valzania, Shaniko Kaleci, Manuela Tondelli, Paolo F Nichelli, Giuliana Galassi
AIM OF THE STUDY: 50% to 60% of patients with ocular Myasthenia Gravis (OMG) progress to generalized disease (GMG) within 2 years.Aim of our study was to explore factors affecting prognosis of OMG and to test the predictive role of several independent clinical variables. MATERIALS AND METHODS: We reviewed a cohort of 168 Caucasian patients followed from September 2000 and January 2016. Several independent variables were considered as prognostic factors: gender, age of onset, results on electrophysiological tests, presence and level of antibodies against acetylcholine receptors (AChR-Abs), treatments, thymic abnormalities...
June 19, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/28623849/population-pharmacokinetics-pharmacodynamics-of-3-4-diaminopyridine-free-base-in-patients-with-lambert-eaton-myasthenia
#6
Nilay Thakkar, Jeffrey T Guptill, Kathy Aleš, David Jacobus, Laura Jacobus, Charles Peloquin, Michael Cohen-Wolkowiez, Daniel Gonzalez
Lambert-Eaton Myasthenia (LEM) is a rare autoimmune disorder associated with debilitating muscle weakness. There are limited treatment options and 3,4-diaminopyridine free base (3,4-DAP) is an investigational orphan drug used to treat LEM-related weakness. We performed a population PK/PD analysis using 3,4-DAP and metabolite concentrations collected from a phase 2 study in LEM patients. The Triple Timed Up & Go (3TUG) assessment, which measures lower extremity weakness, was the primary outcome measure. A total of 1270 PK samples (49 patients) and 1091 3TUG data points (32 randomized patients) were included in the PK/PD analysis...
June 17, 2017: CPT: Pharmacometrics & Systems Pharmacology
https://www.readbyqxmd.com/read/28623040/-neuro-ophthalmological-conditions-study-of-the-clinical-care-pathway
#7
I Layat, G Challe, P LeHoang, B Bodaghi, V Touitou
INTRODUCTION: Neuro-ophthalmologic conditions require specialized multidisciplinary management, both medical and surgical, for patients affected by visual loss due to nervous system disease. The primary goal of this study is to define the specificity of neuro-ophthalmology within the realm of visual health. The secondary goal is to review clinical care pathways by studying the organization of management, in terms of accessibility to care and personalization of the care pathway. PATIENTS AND METHODS: A field study was carried out from February to June 2015, within the ophthalmology service of the Pitié-Salpêtrière University Medical Center in Paris...
June 13, 2017: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/28620344/the-role-of-osteopontin-and-its-gene-on-glucocorticoid-response-in-myasthenia-gravis
#8
Yanchen Xie, Hai-Feng Li, Liang Sun, Linda L Kusner, Shuhui Wang, Yunxiao Meng, Xu Zhang, Yu Hong, Xiang Gao, Yao Li, Henry J Kaminski
Biomarkers that assess treatment response for patients with the autoimmune disorder, myasthenia gravis (MG), have not been evaluated to a significant extent. We hypothesized the pro-inflammatory cytokine, osteopontin (OPN), may be associated with variability of response to glucocorticoids (GCs) in patients with MG. A cohort of 250 MG patients treated with standardized protocol of GCs was recruited, and plasma OPN and polymorphisms of its gene, secreted phosphoprotein 1 (SPP1), were evaluated. Mean OPN levels were higher in patients compared to healthy controls...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28616293/video-assisted-thoracoscopic-completion-thymectomy-based-on-mediastinal-pleura-guidance
#9
Kai Guo, Liping Tong, Xiaofei Li, Xiaolong Yan
We describe a practical video-assisted thoracoscopic surgery (VATS) technique for the en bloc resection of the thymoma, thymus, and bilateral mediastinum lipid based on mediastinal pleura guidance. By taking advantage of single-lumen endotracheal tube (SLET) anesthesia and artificial pneumothorax, we acquired excellent exposure of the anterior mediastinum operative field. In addition, our limited data showed that this surgical approach might have a potential tendency on reducing the blood loss and the operative times...
May 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28608859/residua-of-thymus-in-the-mediastinum-clinical-aspects-cadaveric-study
#10
Ewa Mizia, Wiesława Klimek-Piotrowska, Jarosław Kużdżał, Tomasz Konopka, Monika Lis, Juliusz Pankowski, Artur Pasternak, Jarosław Zawiliński, Andrzej Wrona
The main goal of this study was to investigate possible residua of thymic tissue in 100 adult cadavers with no thoracic pathology known before, by dissection of standard locations of thymic tissue in perithyroid, periaortic, peritracheal and retrotracheal spaces, as well as areas located next to the course of phrenic, vagus and left recurrent laryngeal nerves. Thus obtained tissue samples were studied by two pathologists independently. The remnants of the thymic tissue were found in 61 out of 100 specimens studied...
2017: Folia Medica Cracoviensia
https://www.readbyqxmd.com/read/28608304/lambert-eaton-myasthenic-syndrome-lems-a-rare-autoimmune-presynaptic-disorder-often-associated-with-cancer
#11
REVIEW
Benedikt Schoser, Bruno Eymard, Joe Datt, Renato Mantegazza
Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune neuromuscular junction disorder that is related to the loss of functional P/Q-type voltage-gated calcium channels (VGCCs) on presynaptic nerve terminals. Up to 60% of cases occur as a paraneoplastic disorder (SCLC-LEMS), most commonly in association with small cell lung cancer. The remaining cases have an idiopathic non-tumor etiology but are associated with underlying autoimmune disease (NT-LEMS). Patients with LEMS invariably experience progressive proximal muscle weakness, often accompanied by general fatigue and autonomic symptoms...
June 12, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28604379/physiotherapy-immediately-after-thymectomy-in-patients-with-myasthenia-gravis-two-cases-and-review-of-the-literature
#12
Massimiliano Polastri, Franco Stella, Massimo Lambertini, Walter Trani, Annalisa Ghetti, Andrea Dell'Amore
AIM: Myasthenia gravis (MG) is a rare autoimmune disease characterized by activation of autoantibodies against acetylcholine receptors: patients with MG typically experience muscle weakness and fatigue. The aims of the present study were 1) to describe immediate postoperative physiotherapeutic interventions in two MG patients who underwent thymectomy, and 2) to discuss postoperative rehabilitative issues in MG patients. MATERIALS AND METHODS: This was a non-experimental study analyzing two subjects with MG who underwent thymectomy...
2017: Annali Italiani di Chirurgia
https://www.readbyqxmd.com/read/28601810/pattern-of-ocular-involvement-in-myasthenia-gravis-with-musk-antibodies
#13
Amelia Evoli, Paolo E Alboini, Raffaele Iorio, Valentina Damato, Emanuela Bartoccioni
BACKGROUND: Myasthenia gravis (MG) with antibodies to the muscle-specific kinase (MuSK) has a characteristic phenotype. Ocular manifestations have not been systematically evaluated. OBJECTIVE: To investigate the features of extrinsic ocular muscle involvement in patients with MuSK-MG. METHODS: We retrospectively evaluated the prevalence, time of onset, clinical pattern and outcome of ocular symptoms in 82 patients with a clinical follow-up ≥2 years...
June 10, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28599960/neuro-ophthalmological-conditions-study-of-the-clinical-care-pathway
#14
I Layat, G Challe, P LeHoang, B Bodaghi, V Touitou
INTRODUCTION: Neuro-ophthalmologic conditions require specialized multidisciplinary management, both medical and surgical, for patients affected by visual loss due to nervous system disease. The primary goal of this study is to define the specificity of neuro-ophthalmology within the realm of visual health. The secondary goal is to review clinical care pathways by studying the organization of management, in terms of accessibility to care and personalization of the care pathway. PATIENTS AND METHODS: A field study was carried out from February to June 2015, within the ophthalmology service of the Pitié-Salpêtrière University Medical Center in Paris...
June 6, 2017: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/28599652/regulatory-t-cells-in-multiple-sclerosis-and-myasthenia-gravis
#15
REVIEW
K M Danikowski, S Jayaraman, B S Prabhakar
Multiple sclerosis (MS) is a chronic debilitating disease of the central nervous system primarily mediated by T lymphocytes with specificity to neuronal antigens in genetically susceptible individuals. On the other hand, myasthenia gravis (MG) primarily involves destruction of the neuromuscular junction by antibodies specific to the acetylcholine receptor. Both autoimmune diseases are thought to result from loss of self-tolerance, which allows for the development and function of autoreactive lymphocytes. Although the mechanisms underlying compromised self-tolerance in these and other autoimmune diseases have not been fully elucidated, one possibility is numerical, functional, and/or migratory deficits in T regulatory cells (Tregs)...
June 9, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28599246/ablation-of-il-17-expression-moderates-experimental-autoimmune-myasthenia-gravis-disease-severity
#16
Gabriela Aguilo-Seara, Yanchen Xie, Jarrod Sheehan, Linda L Kusner, Henry J Kaminski
An array of cytokines influences the pathogenesis of early onset myasthenia gravis (MG) and its animal model, experimental autoimmune myasthenia gravis (EAMG). Patients with MG, in particular those with more severe weakness, have elevations of the pro-inflammatory cytokine IL-17 in the blood. We assessed the role of IL-17A in autoimmunity by inducing EAMG in mice with knockout of IL-17 and found a reduction of EAMG severity, but not a complete ablation of disease. The IL-17(ko) mice had no evidence of weakness, low levels of acetylcholine receptor antibodies, and retention of acetylcholine receptor at the neuromuscular junction...
June 6, 2017: Cytokine
https://www.readbyqxmd.com/read/28592450/a-propensity-score-analysis-for-comparison-of-t-3b-and-vatet-in-myasthenia-gravis
#17
Greta Brenna, Carlo Antozzi, Cristina Montomoli, Fulvio Baggi, Renato Mantegazza
OBJECTIVE: We performed propensity score (PS) models to compare the outcome of patients with myasthenia gravis (MG) submitted to 2 different surgical approaches: extended transsternal (T-3b) or thoracoscopic extended thymectomy (VATET). METHODS: Patients' clinical data were retrieved from the MG database of the C. Besta Neurologic Institute Foundation. In the PS analysis, a matching ratio of 1:1 of the main clinical variables was obtained for the 2 groups of patients and treatment effect was estimated by comparing their outcome...
June 7, 2017: Neurology
https://www.readbyqxmd.com/read/28592233/single-fiber-electromyography-in-the-orbicularis-oculi-muscle-in-patients-with-ocular-myasthenia-gravis-symptoms-does-abnormal-jitter-predict-response-to-treatment
#18
Goran Rakocevic, Mark Moster, Mary Kay Floeter
BACKGROUND: Seronegative ocular myasthenia gravis (OMG) is diagnosed by ocular symptoms with supporting SFEMG, typically of frontalis or extensor digitorum muscles. We aimed to determine the sensitivity and specificity of orbicularis oculi SFEMG to diagnose and exclude myasthenia gravis and predict response to therapy. METHODS: Orbicularis oculi SFEMG studies were conducted in 142 consecutive patients with symptoms and/or findings of OMG and negative AChR antibody during the period of 5 years...
June 7, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28591868/video-assisted-thoracoscopic-thymectomy-for-non-thymomatous-myasthenia-gravis-a-right-sided-or-left-sided-approach
#19
Hany Hasan Elsayed, Mahmoud Gamal, Saleh Raslan, Hossam Abdel Hamid
A best evidence topic in thoracic surgery was written according to a structured protocol. The question addressed was in [patients undergoing video-assisted thoracoscopic thymectomy for myasthenia gravis (MG)] is a [left-sided approach] superior to a [right-sided approach] in terms of [clinical outcome]? Two hundred and fifty-nine papers were found using the reported search. In looking at both procedures, we selected studies with a sizeable number of patients performing or studying both procedures and comparing their outcome...
June 7, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28590545/the-utility-of-a-single-simple-question-in-the-evaluation-of-patients-with-myasthenia-gravis
#20
Alon Abraham, Ari Breiner, Carolina Barnett, Hans D Katzberg, Vera Bril
INTRODUCTION: Assessing myasthenia gravis (MG) can be challenging, and multiple scales are available to evaluate disease severity. We evaluated the utility of a single simple question, namely "what percentage of normal do you feel regarding your MG, 0-100% normal", as part of the MG evaluation. METHODS: A retrospective chart review of patients attending the neuromuscular clinic from 01/2014 to 12/2015 was performed. Responses were correlated with symptoms and signs, QMGS (quantitative myasthenia gravis score), MGII (myasthenia gravis impairment index), and MG-QOL15 (myasthenia gravis quality of life)...
June 7, 2017: Muscle & Nerve
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