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ocular myasthenia gravis

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https://www.readbyqxmd.com/read/28738395/outcomes-of-preoperative-and-postoperative-corticosteroid-therapies-in-myasthenia-gravis
#1
Yun Jing, Xinxin Liu, Lei Yu, Ran Li
AIMS: To compare the long-term outcomes of non-thymomatous myasthenia gravis (MG) patients receiving pre-thymectomy (Pre-CS) or post-thymectomy corticosteroid (Post-CS) therapy. METHODS: In a retrospective cohort study, 41 patients with MG were treated with Pre-CS therapy, and 110 were treated with Post-CS therapy. RESULTS: In the MG cohorts, 9 of 40 patients (22.5%) in the Pre-CS group vs. 28 of 105 patients (26.7%) in the Post-CS group achieved a complete remission (CR) at 1 year, 29...
July 24, 2017: European Neurology
https://www.readbyqxmd.com/read/28731133/irrelevance-of-anti%C3%A2-nachr-cytoplasmic-loop-antibody-in-the-degree-of-myasthenia-gravis
#2
Ye Lin, Chen Song, Jiang Xu, Yongxiang Yang, Hang Qin, Cong Zhao, Jiaji Lin, Rui Liu, Zhuyi Li
Anti‑nicotinic acetylcholine receptor (nAChR) antibody in myasthenia gravis (MG) usually refers to that against the extracellular domain (ECD) of nAChR. However, growing evidence has indicated that there also exists the nAChR antibody against the nAChR cytoplasmic loop (CL) in patients with MG. Some studies have demonstrated that the anti‑CL antibody may play a protective role in animal models of experimental autoimmune MG. However, to date, limited or no information is available as to whether anti‑CL antibody plays a beneficial role in patients with MG...
July 19, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28707128/thymectomy-is-a-beneficial-therapy-for-patients-with-non-thymomatous-ocular-myasthenia-gravis-a-systematic-review-and-meta-analysis
#3
Kai Zhu, Jiaoxing Li, Xin Huang, Wei Xu, Weibin Liu, Jiaxin Chen, Pei Chen, Huiyu Feng
Ocular myasthenia gravis, an autoimmune disease, is characterized by extraocular muscle weakness. Myasthenia gravis is closely associated with the functional status of the thymus gland. The efficacy of thymectomy for non-thymomatous ocular myasthenia gravis remains controversial. Here, we present the first systematic review and meta-analysis of studies assessing the outcome of thymectomy in patients with non-thymomatous ocular myasthenia gravis and found that the pooled rate of complete stable remission was 0...
July 13, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28694075/double-seropositive-myasthenia-gravis-with-acetylcholine-receptor-and-low-density-lipoprotein-receptor-related-protein-4-antibodies-associated-with-invasive-thymoma
#4
Hidehiro Ishikawa, Akira Taniguchi, Yuichiro Ii, Osamu Higuchi, Hidenori Matsuo, Shunya Nakane, Masaru Asahi, Atsushi Niwa, Hidekazu Tomimoto
We describe two cases of myasthenia gravis (MG) with double seropositivity for acetylcholine receptor (AChR) and low-density lipoprotein receptor-related protein 4 (LRP4) antibodies (AChR/LRP4-MG) with invasive thymoma. Both cases showed myasthenic weakness, which was restricted to the ocular muscles for >5 months from onset, and then unprovoked severe clinical deterioration supervened with predominant bulbar symptoms. The patients responded adequately to therapeutic intervention. Serum AChR antibody levels at post-intervention were markedly decreased, whereas LRP4 antibodies were almost unchanged in case 1 and slightly decreased in case 2...
June 3, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28666511/unilateral-external-ophthalmoplegia-arare-presentation-of-myasthenia-gravis
#5
Muhammad Saim Khan, Asad Habib, Imran Basit
Myasthenia gravis (MG) is a disease of autoimmunity with variable and diverse clinical presentations. The target tissue is neuromuscular junction of skeletal muscles, where efficient nerve impulse transmission is hampered leading to less effective muscle contraction. Patients of MG usually present with bilateral ptosis, diplopia and fatigability, which may or may not coexist with generalised weakness, dysphagia and dysarthria. A46-year male presented with unilateral ptosis and diplopia. Except for unilateral moderate ptosis and restriction of extraocular movements, ocular and systemic examination was normal...
November 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28662779/ocular-vestibular-evoked-myogenic-potential-in-patients-with-myasthenia-gravis-a-prospective-clinical-study
#6
Mohammad El-Sayed Mojahed, Elsaeid Mohamed Thabet, Mohamed Galal El-Khateeb, Ashraf Elsayed Morgan
OBJECTIVE: Myasthenia gravis (MG) is an archetypic disorder of neuromuscular junctions (NMJs) and autoantibody-mediated disease causing fatigable weakness of skeletal muscles with an ocular onset in up to 85%. The aim of this study was to detect extra ocular muscles (EOMs) abnormalities in MG patients using ocular vestibular evoked myogenic potential (oVEMP) n10 response. METHODS: The oVEMP was performed on 40 myasthenia gravis patients that were divided into three groups: newly diagnosed (10 patients), uncontrolled on treatment (15 patients) and controlled on treatment (15 patients) groups in addition to a control group of 10 subjects...
June 26, 2017: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/28650411/recording-fewer-than-20-potential-pairs-with-sfemg-may-suffice-for-the-diagnosis-of-myasthenia-gravis
#7
Alon Abraham, Ari Breiner, Carolina Barnett, Hans D Katzberg, Vera Bril
PURPOSE: Our aim in the current study was to determine the minimum number of SFEMG potential pairs required to confirm neuromuscular junction impairment and relate this number to disease severity. METHODS: Ninety-four patients with myasthenia gravis (MG) attending the neuromuscular clinic from February 2013 to November 2015 were included. The SFEMG sensitivity was determined for each number of recorded pairs up to 20. In addition, we compared clinical and electrophysiologic characteristics between patients with abnormality within the first 3, 4 to 7, and ≥8 recorded pairs to determine whether this number is associated with disease severity...
June 20, 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28641305/incidence-of-autoimmune-myasthenia-gravis-in-a-health-maintenance-organization-in-buenos-aires-argentina
#8
Mariela Bettini, Marcelo Chaves, Edgardo Cristiano, Vanina Pagotto, Lucia Perez, Diego Giunta, Marcelo Rugiero
BACKGROUND: Different epidemiological studies, especially in Europe, have estimated the incidence density of myasthenia gravis (MG) to range between 1.7 and 21.3/1,000,000/person-year; however, data from regions such as Latin America are scarce. This study is aimed at estimating the incidence and prevalence of acquired MG in Buenos Aires, Argentina. METHODS: The study population comprised of affiliates of the Italian Hospital Medical Care Program, a prepaid health maintenance organization located in Buenos Aires...
June 23, 2017: Neuroepidemiology
https://www.readbyqxmd.com/read/28625092/factors-affecting-outcome-in-ocular-myasthenia-gravis
#9
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/28601810/pattern-of-ocular-involvement-in-myasthenia-gravis-with-musk-antibodies
#10
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/28592233/single-fiber-electromyography-in-the-orbicularis-oculi-muscle-in-patients-with-ocular-myasthenia-gravis-symptoms-does-abnormal-jitter-predict-response-to-treatment
#11
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/28590545/the-utility-of-a-single-simple-question-in-the-evaluation-of-patients-with-myasthenia-gravis
#12
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
https://www.readbyqxmd.com/read/28588549/amyotrophic-lateral-sclerosis-and-myasthenia-gravis-overlap-syndrome-a-review-of-two-cases-and-the-associated-literature
#13
Hongfei Tai, Liying Cui, Yuzhou Guan, Mingsheng Liu, Xiaoguang Li, Yan Huang, Jing Yuan, Dongchao Shen, Dawei Li, Feifei Zhai
OBJECTIVE: To describe the characteristics of patients with amyotrophic lateral sclerosis (ALS) and myasthenia gravis (MG) overlap syndrome and explore the relationship between the two diseases. METHODS: We conducted a search of medical records at Peking Union Medical University Hospital from 1983 to 2015 for coexistence of ALS and MG and searched the PubMed database for all literature describing ALS and MG overlap syndrome published through December 2016. We analyzed the clinical and neurophysiological characteristics of patients by groups according to strict diagnostic criteria...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28562609/diagnostic-value-of-repeated-ice-tests-in-the-evaluation-of-ptosis-in-myasthenia-gravis
#14
Jun Young Park, Hee Kyung Yang, Jeong-Min Hwang
Twenty-six patients with ptosis related to Myasthenia gravis (MG) and 38 controls with ptosis other than MG were included. All patients were tested with the ice test 2 times on separate days in the afternoon. The margin reflex distance (MRD) was measured before and immediately after 2-minute application of ice on the eyelids. The ice test was judged positive if there was an improvement of at least 2.0 mm of MRD after the ice test. Among the patients with negative test results, 'equivocal' was defined by improvement of MRD from at least 1...
2017: PloS One
https://www.readbyqxmd.com/read/28562525/ophthalmoplegia-associated-with-lung-adenocarcinoma-in-a-patient-with-the-lambert-eaton-myasthenic-syndrome-a-case-report
#15
Yufeng Tang, Ke Wang, Zhonglun Chen, Muke Zhou, Jingfeng Duan, Tao Liu, Dong Zhou
RATIONALE: The Lambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular disease; its unique symptoms of LEMS include dry mouth with a metallic taste, constipation, and erectile dysfunction. As it is quite rare, isolated ocular muscle impairment associated with LEMS east to ignore. PATIENT CONCERNS: A 65-year-old man presented with alternating ptosis and diplopia. Isolated ocular muscle impairment had lasted for 6 years, and the patient was initially diagnosed with ocular myasthenia gravis (MG)...
June 2017: Medicine (Baltimore)
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...
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
#17
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/28495046/transient-neonatal-myasthenia-gravis-due-to-a-mother-with-ocular-onset-of-anti-muscle-specific-kinase-myasthenia-gravis
#18
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...
July 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28492464/validity-of-forced-eyelid-closure-test-a-novel-clinical-screening-test-for-ocular-myasthenia-gravis
#19
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/28458152/increased-expression-of-p2x7-receptor-in-peripheral-blood-mononuclear-cells-correlates-with-clinical-severity-and-serum-levels-of-th17-related-cytokines-in-patients-with-myasthenia-gravis
#20
Yong Zhang, Yanyan Zhang, Hao Li, Xiao Jia, Xiuying Zhang, Yan Xia, YuZhong Wang, Linlin Fu, Chenghua Xiao, Deqin Geng
OBJECTIVES: P2X7R is a well-documented activator of innate and adaptive immune responses. We aimed to measure the expression levels of P2X7R in peripheral blood mononuclear cells (PBMCs) from patients with myasthenia gravis (MG) and to investigate whether the expression of P2X7R is associated with pathogenesis of MG. PATIENTS AND METHODS: A total of 32 patients with MG (12 generalized MG (GMG) and 20 Ocular MG (OMG) and 22 healthy donors were recruited in this study...
June 2017: Clinical Neurology and Neurosurgery
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