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Myasthenia gravis

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https://www.readbyqxmd.com/read/28524043/gvhd-like-erythroderma-in-the-clinical-course-of-thymoma-associated-myasthenia-gravis
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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/28511871/effect-of-distigmine-on-the-contractile-response-of-guinea-pig-urinary-bladder-to-electrical-field-stimulation
#7
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
#8
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
#9
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
#10
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
#11
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/28495138/effect-of-ethnic-origin-and-gender-on-the-clinical-manifestations-of-myasthenia-gravis-among-the-jewish-population-in-israel
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/28495046/transient-neonatal-myasthenia-gravis-due-to-a-mother-with-ocular-onset-of-anti-muscle-specific-kinase-myasthenia-gravis
#15
Ju-Yeun Lee, Ju-Hong Min, Sueng-Han Han, Jinu Han
We describe a 27-year-old pregnant female with new onset of conjugate gaze deficit during the third trimester of pregnancy. Repetitive nerve stimulation tests, neostigmine tests, and acetylcholine receptor antibody assays were all negative. The patient delivered a normal healthy baby at a local clinic via cesarean section. The baby became hypotonic and had respiratory failure several minutes after birth. The result of acetylcholine receptor antibody was negative in the neonate. The neonate became healthy spontaneously and was extubated after 21 days of ventilation care...
April 4, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28494776/aggregated-n-of-1-trials-for-unlicensed-medicines-for-small-populations-an-assessment-of-a-trial-with-ephedrine-for-myasthenia-gravis
#16
Stephanie S Weinreich, Charlotte Vrinten, Marja R Kuijpers, Alexander F Lipka, Kirsten J M Schimmel, Erik W van Zwet, Christine Gispen-de Wied, Yechiel A Hekster, Jan J G M Verschuuren, Martina C Cornel
BACKGROUND: Inexpensive medicines with a long history of use may currently be prescribed off-label for rare indications. Reimbursement is at the discretion of health insurance companies, and may be unpredictable. The example addressed was ephedrine as add-on treatment for myasthenia gravis. Stakeholders from academia, a patient organization, the Dutch National Health Care Institute (NHCI) and Dutch Medicines Evaluation Board (MEB) advised on the trial design. The NHCI and MEB agreed to provide scientific advice on the suitability of the evidence generated by the trial, for regulatory decisions...
May 12, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28492464/validity-of-forced-eyelid-closure-test-a-novel-clinical-screening-test-for-ocular-myasthenia-gravis
#17
Supanut Apinyawasisuk, Xinkai Zhou, Jack J Tian, Giancarlo A Garcia, Rustum Karanjia, Alfredo A Sadun
BACKGROUND: Forced eyelid closure test (FECT) is a clinical screening test developed from the original Cogan lid twitch (CLT) sign to assist in the diagnosis of ocular myasthenia gravis (OMG), We evaluated the sensitivity and specificity of FECT compared with CLT and benchmarked to standard diagnostic tests. METHODS: This study was a retrospective chart review of 48 patients using electronic medical records of those that presented with ptosis and/or diplopia at Doheny Eye Institute, University of California, Los Angeles between February 2015 and April 2016...
May 10, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28490756/expressing-acetylcholine-receptors-after-innervation-suppresses-spontaneous-vesicle-release-and-causes-muscle-fatigue
#18
Meghan Mott, Victor M Luna, Jee-Young Park, Gerald B Downes, Kimberly Epley, Fumihito Ono
The formation and function of synapses are tightly orchestrated by the precise timing of expression of specific molecules during development. In this study, we determined how manipulating the timing of expression of postsynaptic acetylcholine receptors (AChRs) impacts presynaptic release by establishing a genetically engineered zebrafish line in which we can freely control the timing of AChR expression in an AChR-less fish background. With the delayed induction of AChR expression after an extensive period of AChR-less development, paralyzed fish displayed a remarkable level of recovery, exhibiting a robust escape response following developmental delay...
May 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28489755/kennedy-disease-with-difficulty-in-differential-diagnosis-a-case-report
#19
Yating Chen, Peng Luo, Zhongli Li, Hengping Hu, Duobin Wu, Tingting Xu, Xingzuo Wang, Haiting Xie
RATIONALE: Kennedy disease (KD) is also known as spinal bulbar muscular dystrophy. As KD has similar symptoms with most neuromuscular diseases, so it is difficult to make a rapid diagnosis clinically. PATIENT CONCERNS: We report a case of a 43-year-old male with progressive limb proximal weakness without family history. Physical examination showed gynecomastia, erectile dysfunction, bilateral tendon reflex and quadriceps weakness, and tongue muscle atrophy. DIAGNOSES: Laboratory examination found increased creatine kinase, impaired glucose tolerance, and abnormal lactic acid values...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28489146/acute-muscular-weakness-in-children
#20
Ricardo Pablo Javier Erazo Torricelli
Acute muscle weakness in children is a pediatric emergency. During the diagnostic approach, it is crucial to obtain a detailed case history, including: onset of weakness, history of associated febrile states, ingestion of toxic substances/toxins, immunizations, and family history. Neurological examination must be meticulous as well. In this review, we describe the most common diseases related to acute muscle weakness, grouped into the site of origin (from the upper motor neuron to the motor unit). Early detection of hyperCKemia may lead to a myositis diagnosis, and hypokalemia points to the diagnosis of periodic paralysis...
April 2017: Arquivos de Neuro-psiquiatria
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