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https://www.readbyqxmd.com/read/28538257/what-s-in-the-literature
#1
Mark Bromberg, Nicholas J Silvestri, David Lacomis
In this edition, we provide a detailed summary of an informative book, "GBS100: Celebrating a Century of Progress in Guillain-Barré Syndrome" developed by the Peripheral Nerve Society to honor the centenary of the original paper on Guillain-Barré Syndrome. We also review various studies in myasthenia gravis including: management with rituximab; the efficacy of early fast-acting treatment with corticosteroids; and various dosing strategies for tacrolimus. Finally, we review new studies including: the potential pathogenesis, risk factors, and functional decline of patients with inclusion body myositis; MxA immunoreactivity in dermatomyositis; diagnostic approaches for evaluating patients with myalgia, fatigue, and exercise intolerance; MRI patterns in genetic muscle disease; and MRI as an outcome measure in facioscapulohumeral muscular dystrophy...
June 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28538252/concurrent-paraspinous-myopathy-and-myasthenia-gravis
#2
Alissa E Romano, Zaid Al-Qudah, Henry J Kaminski, Bashar Katirji, Karim Salame
Paraspinous myopathy is a rare neuromuscular disorder characterized by selective involvement of the cervical, thoracic, or lumbar muscles. Leading clinical features include a bent spine or dropped head (antecollis). In myasthenia gravis (MG), patients may have camptocormia secondary to neuromuscular junction dysfunction of the paraspinal muscles, and this condition usually responds to acetylcholinesterase inhibitors or immunosuppressive treatments. However, concomitant MG and paraspinous myopathy with histologic and electrophysiologic evidence of myopathic changes of the paraspinal muscles has only been reported twice in the literature...
June 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28535636/-characteristics-of-distribution-and-quantity-of-dendritic-cells-subtypes-in-hyperplastic-thymus-in-patients-with-myasthenia-gravis
#3
H Huang, P Chen, C Y Ou, L Qiu, Z Z Lin, Z D Huang, W B Liu
Objective: To investigate the characteristics of the distribution and amount of different subtypes of dendritic cell (DC) in myasthenia gravis(MG). Methods: We collected the thymic specimens from 32 patients who received thymoctomy because of cardiac surgery from January 2016 to December 2016 and selected 14 of them as normal control. Meantime, 61 MG patients who combined with thymic hyperplasia and received extended thymectomy were collected and selected 8 of them as experiment group.Immunohistochemical methods were used to label the two subtype DCs: plasmacytoid dendritic cell (pDC) and classical/conventional dendritic cell (cDC), to observe the distribution of these two DC subtypes in thymus and also quantify them in different thymus structures by image analysis software...
May 23, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28533621/correlations-between-slow-rate-repetitive-nerve-stimulation-and-characteristics-associated-with-amyotrophic-lateral-sclerosis-in-chinese-patients
#4
Yan Wang, Zheman Xiao, Hong Chu, Jingjing Liang, Xu Wu, Hongjuan Dong, Yang Yan, Zuneng Lu
[Purpose] To clarify the features associated with decrements in compound muscle action potentials (CMAP) during slow-rate repetitive nerve stimulation (RNS) of muscles involved in amyotrophic lateral sclerosis (ALS) in mainland China. [Subjects and Methods] A retrospective study of decremental responses to slow-rate RNS was performed to compare patients with ALS to those with myasthenia gravis (MG). [Results] A significant decrement (>5%) was observed in at least one muscle in 54% of ALS patients. The trapezius muscle was the most commonly affected (67%)...
April 2017: Journal of Physical Therapy Science
https://www.readbyqxmd.com/read/28524043/gvhd-like-erythroderma-in-the-clinical-course-of-thymoma-associated-myasthenia-gravis
#5
Hisashi Nomura, Shigeaki Suzuki, Fumiyo Yasuda-Sekiguchi, Masayuki Amagai, Satoshi Yamada, Jin Nakahara, Norihiro Suzuki, Mitsutomo Kohno, Takeru Funakoshi
No abstract text is available yet for this article.
May 19, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28523463/immature-exosomes-derived-from-microrna-146a-overexpressing-dendritic-cells-act-as-antigen-specific-therapy-for-myasthenia-gravis
#6
Weifan Yin, Song Ouyang, Zhaohui Luo, Qiuming Zeng, Bo Hu, Liqun Xu, Yuan Li, Bo Xiao, Huan Yang
Myasthenia gravis (MG) is a neurological autoimmune disease characterized by fluctuating weakness of certain voluntary muscles. Current treatments for MG are largely directed at suppressing the whole immune system by using immunosuppressants or glucocorticoids and often cause several side effects. The ideal therapeutic methods for MG should suppress aberrant immunoactivation specifically, while retaining normal function of the immune system. In this study, we first produced exosomes from microRNA-146a overexpressing dendritic cells (DCs)...
May 18, 2017: Inflammation
https://www.readbyqxmd.com/read/28516744/repetitive-nerve-stimulation-in-musk-antibody-positive-myasthenia-gravis
#7
Seung Woo Kim, Mun Kyung Sunwoo, Seung Min Kim, Ha Young Shin, Il Nam Sunwoo
BACKGROUND AND PURPOSE: Responses to repetitive nerve stimulation (RNS) in patients with muscle-specific tyrosine kinase (MuSK) antibody (Ab)-positive myasthenia gravis (MG) vary depending on the muscles tested. We analyzed the RNS responses of limb and facial muscles in MuSK-Ab-positive and acetylcholine receptor (AChR)-Ab-negative MG (MuSK MG) and MuSK-Ab-negative and AChR-Ab-negative [double-seronegative (DSN)] MG patients. METHODS: We retrospectively compared RNS responses between 45 MuSK MG and 29 DSN MG...
May 15, 2017: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/28516329/screening-for-lipoprotein-receptor-related-protein-4-agrin-and-titin-antibodies-and-exploring-the-autoimmune-spectrum-in-myasthenia-gravis
#8
Isabell Cordts, Nicolas Bodart, Kathi Hartmann, Katerina Karagiorgou, John S Tzartos, Lin Mei, Jens Reimann, Philip Van Damme, Michael H Rivner, Alain Vigneron, Joachim Weis, Jörg B Schulz, Socrates J Tzartos, Kristl G Claeys
In autoimmune myasthenia gravis (MG), the identification of antibodies and characterization of serological subgroups is of great importance for diagnosis and management of the disease. Our aims were to study the frequency of antibodies against lipoprotein-related protein 4 (LRP4), agrin, and titin using the most recent techniques, and to characterize corresponding clinical features and autoimmune diseases (AID) in 100 MG-patients. The antibody frequencies in the 55 AChR-antibody positive patients were 7% LRP4, 5% agrin, 53% titin, and in the 45 AChR-antibody negative patients 2% MuSK, 2% LRP4, 2% agrin, and 27% titin...
May 17, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28514336/-the-quality-of-life-in-patients-with-a-different-types-of-myasthenia-gravis
#9
Yu N Bykov, V I Okladnikov, A I Smolin
AIM: Researching quality of life in patients with myasthenia gravis at different clinical variants of disease. MATERIAL AND METHODS: We studied 103 patients with myasthenia gravis. A battery of tests and scales for assessment of neurological deficit and quality of life was used. RESULTS AND CONCLUSION: Two types of disease have been identified in myasthenia gravis: favorable and unfavorable. The minimal duration of disease before thymectomy is the main determinant of the best outcome...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28514294/tnfaip3-gene-rs7749323-polymorphism-is-associated-with-late-onset-myasthenia-gravis
#10
Hong-Wei Yang, Yanchen Xie, Yuan Zhao, Liang Sun, Xiaoquan Zhu, Shuhui Wang, Yong-Qiang Zhang, Ping Lei, Yunxiao Meng
In this study, we intended to genotype 2 single nucleotide polymorphisms (SNPs) of tumor necrosis factor α-induced protein 3 (TNFAIP3) genes and explore an association of TNFAIP3 genetic polymorphism with the patients of myasthenia gravis (MG) at clinical level. In brief, 215 of adult MG patients were divided into subgroups according to their clinical features, age of onset, thymic pathology, and autoantibodies. Two hundred thirty-five of healthy controls were also divided into subgroups with gender- and age-matched...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28512504/a-case-report-of-drug-induced-myopathy-involving-extraocular-muscles-after-combination-therapy-with-tremelimumab-and-durvalumab-for-non-small-cell-lung-cancer
#11
William Carrera, Brandon J Baartman, Gregory Kosmorsky
Recently developed anti-tumour therapies targeting immune checkpoints include tremelimumab and durvalumab. These agents have incompletely characterised side effect profiles. The authors report a 68-year-old man treated for non-small cell lung cancer (NSCLC) with a combination of tremelimumab and durvalumab. After treatment he developed diplopia, ptosis, fatigue, weakness, and an inflammatory myopathy affecting the extraocular muscles requiring hospitalisation. Electromyography (EMG) testing and muscle biopsy suggested inflammatory myopathy without sign of myasthenia...
June 2017: Neuro-ophthalmology
https://www.readbyqxmd.com/read/28511871/effect-of-distigmine-on-the-contractile-response-of-guinea-pig-urinary-bladder-to-electrical-field-stimulation
#12
Keisuke Obara, Yurina Kobayashi, Daisuke Chino, Yoshio Tanaka
Distigmine bromide (distigmine) is a reversible carbamate group cholinesterase (ChE) inhibitor. Although mainly used clinically for the treatment of myasthenia gravis, distigmine is also indicated for detrusor underactivity in Japan. According to the pharmacological classification of distigmine, its therapeutic effect against detrusor underactivity appears to be produced by enhanced urinary bladder smooth muscle (UBSM) contractility due to an increased concentration of acetylcholine between parasympathetic nerve endings and UBSM cells...
May 13, 2017: European Journal of Pharmacology
https://www.readbyqxmd.com/read/28509648/video-assisted-extirpation-of-cranial-mediastinal-masses-in-dogs-18-cases-2009-2014
#13
Melissa A MacIver, J Brad Case, Eric L Monnet, Geraldine B Hunt, Philipp D Mayhew, Michelle L Oblak, Jeffrey J Runge, Ameet Singh, Daniel D Smeak, Michele A Steffey, Sarah E Boston
OBJECTIVE To characterize clinical findings, surgical procedures, complications, and outcomes in dogs undergoing extirpation of masses from the cranial mediastinum via video-assisted thoracic surgery (VATS) and establish preliminary guidelines for case selection when considering VATS for thymectomy in dogs. DESIGN Retrospective case series. ANIMALS 18 client-owned dogs that underwent extirpation of a cranial mediastinal mass by means of VATS at 5 academic referral hospitals from 2009 through 2014. PROCEDURES Medical records were reviewed and data extracted regarding signalment, clinical signs, physical examination findings, diagnostic imaging results, surgical approach and duration, cytologic and histologic examination results, complications, outcome, and cause of death, when applicable...
June 1, 2017: Journal of the American Veterinary Medical Association
https://www.readbyqxmd.com/read/28508416/frame-shift-variant-in-the-chrne-gene-in-a-juvenile-dog-with-suspected-myasthenia-gravis-like-disease
#14
Vanessa Herder, Malgorzata Ciurkiewicz, Wolfgang Baumgärtner, Vidhya Jagannathan, Tosso Leeb
No abstract text is available yet for this article.
May 16, 2017: Animal Genetics
https://www.readbyqxmd.com/read/28506261/a-possible-role-of-low-regulatory-t-cells-in-anti-acetylcholine-receptor-antibody-positive-myasthenia-gravis-after-bone-marrow-transplantation
#15
Masahiko Fukatsu, Takenobu Murakami, Hiroshi Ohkawara, Shunichi Saito, Kazuhiko Ikeda, Suguru Kadowaki, Itaru Sasaki, Mari Segawa, Tomoko Soeda, Akihiko Hoshi, Hiroshi Takahashi, Akiko Shichishima-Nakamura, Kazuei Ogawa, Yoshihiro Sugiura, Hitoshi Ohto, Yasuchika Takeishi, Takayuki Ikezoe, Yoshikazu Ugawa
BACKGROUND: Chronic graft-versus-host disease (GVHD) appears several months following allogenic hematopoietic stem cell transplantation (HSCT) and is clinically analogous to autoimmune disorder. Polymyositis is a common neuromuscular disorder in chronic GVHD, but myasthenia gravis (MG) is extremely rare. Hence, its pathophysiology and treatment have not been elucidated. CASE PRESENTATION: A 63-year-old man with a history of chronic GVHD presented with ptosis, dropped head, and dyspnea on exertion, which had worsened over the previous several months...
May 15, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28505981/severe-exacerbation-of-myasthenia-gravis-associated-with-checkpoint-inhibitor-immunotherapy
#16
Dana S Cooper, Matthew N Meriggioli, Philip D Bonomi, Rabia Malik
Monoclonal antibodies that target either PD-1 or PD-L1 have recently been approved for treatment of advanced non-small cell lung cancer. These antibodies are immune checkpoint inhibitors which have been shown to exacerbate Myasthenia Gravis (MG) and other autoimmune diseases. While effective in preventing tumor cells from evading immune attack, immune checkpoint inhibitors such as nivolumab, an antibody directed against the programmed cell death protein-1 (PD-1) receptor located on T-cells, may also cause immune dysregulation and could cause or potentiate pre-existing autoimmune conditions...
May 6, 2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/28495245/massive-parallel-sequencing-identifies-rapsn-and-pdha1-mutations-causing-fetal-akinesia-deformation-sequence
#17
Lore Winters, Evelien Van Hoof, Luc De Catte, Kris Van Den Bogaert, Thomy de Ravel, Liesbeth De Waele, Anniek Corveleyn, Jeroen Breckpot
INTRODUCTION: Fetal akinesia deformation sequence (FADS) or arthrogryposis multiplex congenita (AMC) is characterized by clinical ambiguity and genetic heterogeneity, hampering genetic diagnosis via traditional sequencing methods. Next generation sequencing (NGS) of all known disease-causing genes offers an elegant solution to identify the genetic etiology of AMC/FADS in a diagnostic setting. METHODS: An in-house developed disease-associated gene panel was conducted in two unrelated fetuses with FADS...
April 26, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28495138/effect-of-ethnic-origin-and-gender-on-the-clinical-manifestations-of-myasthenia-gravis-among-the-jewish-population-in-israel
#18
Ali Asmail, Anat Kesler, Vivian E Drory, Hadar Kolb, Arnon Karni
Reports on patients with myasthenia gravis (MG) of different ethnic origins demonstrated differences in weakness distribution and serological results. We studied MG characteristics in a cohort of Ashkenazi (ASH) and non-Ashkenazi (NASH) Jewish origin according to their ethnic origins and gender. The frequency of age of MG onset was distributed in a bi-modal fashion in the female patients and increased gradually over time, with a peak around 70years of age in the male patients. Ocular MG was more frequent in males and ASH patients...
June 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28495049/selective-or-predominant-triceps-muscle-weakness-in-african-american-patients-with-myasthenia-gravis
#19
Alon Abraham, Charles D Kassardjian, Hans D Katzberg, Vera Bril, Ari Breiner
Myasthenia gravis (MG) can lead to weakness in different patterns of muscle groups. Limb muscle weakness is most typically seen in a limb girdle pattern, although variants exist. In the current study, we aimed to describe a unique MG phenotype consisting of selective or predominant triceps muscle weakness. We performed a retrospective review of MG patients who developed focal or predominant triceps muscle weakness between 2006 and 2016. The clinical, electrophysiological and serological characteristics of these patients were examined...
April 21, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28495048/hla-and-age-of-onset-in-myasthenia-gravis
#20
Ernestina Santos, Andreia Bettencourt, Ana Martins da Silva, Daniela Boleixa, Dina Lopes, Sandra Brás, Paulo Pinho E Costa, Carlos Lopes, Guilherme Gonçalves, Maria Isabel Leite, Berta Martins da Silva
The aetiology of MG is unknown, but both genetic and environmental factors are important. Over the years association of MG with Human Leucocyte Antigens (HLA) has been described in different populations. We investigated a possible association between HLA-DRB1 alleles and age of onset in MG. One hundred and fourteen MG patients (82 females) and 282 control individuals (CP) were studied. Patients were classified according to the age of onset (early-onset <50, n = 74 and late-onset ≥ 50, n = 20)...
April 5, 2017: Neuromuscular Disorders: NMD
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