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Ocular myasthenia

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https://www.readbyqxmd.com/read/28981152/low-conversion-rate-of-ocular-to-generalized-myasthenia-gravis-in-singapore
#1
Kelvin Y Teo, Sharon L Tow, Benjamin Haaland, Tushar D Gosavi, Jing-Liang Loo, Yew Long Lo, Dan Milea
INTRODUCTION: Ocular myasthenia gravis (OMG) is a common condition of the neuro-muscular junction, which may convert to generalized myasthenia gravis (GMG). Our aim was to assess the conversion rate and predictive factors for generalization in OMG, in an Asian population. METHODS: Retrospective study of OMG patients with a minimum 2 years follow-up. RESULTS: Among 191 patients with OMG, 155 patients had a minimum 2years follow-up. The conversion rate at the median follow-up (40...
October 5, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28882932/hickam-s-dictum-myasthenia-gravis-presenting-concurrently-with-graves-disease
#2
Shekhar Sehgal, Roshan Rebello, Louise Wolmarans, Marianne Elston
We present two patients with Graves' disease and concurrent myasthenia gravis. The impact of the dual diagnosis on the clinical course and the potential for a delayed diagnosis of myasthenia gravis is discussed. Patient 1, a 28-year-old man was diagnosed with Graves' disease following his second respiratory arrest. His history was strongly suggestive of a second pathology. Patient 2, a 66-year-old Cantonese woman with established Graves' disease presented with thionamide-related neutropaenia. Examination revealed bilateral ptosis and right lateral rectus palsy...
September 7, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28881457/ocular-presentation-of-myasthenia-gravis-a-natural-history-cohort
#3
Sivesh K Kamarajah, Girija Sadalage, Jonathan Palmer, Helena Carley, Paul Maddison, Arulmaran Sivaguru
INTRODUCTION: There is limited data on the natural history of untreated myasthenia gravis (MG) with ocular presentation. METHODS: We analysed 93 patients from symptom onset presenting to Birmingham Midlands Eye Centre (BMEC) from January 2004 to July 2015. We used multiple stepwise logistic regression to identify predictive factors of generalisation and Kaplan-Meier analysis on time to generalisation. RESULTS: 46% of patients developed generalised symptoms during the study period...
September 7, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28872522/diagnostic-utility-of-repetitive-nerve-stimulation-in-a-large-cohort-of-patients-with-myasthenia-gravis
#4
Åsa Amandusson, Kristin Elf, Margareta E Grindlund, Anna R Punga
PURPOSE: Optimizing the diagnostic utility of repetitive nerve stimulation in myasthenia gravis (MG) may include tailoring the examination to clinical phenotype. Therefore, we analyzed all available repetitive nerve stimulation parameters in a large cohort of patients with confirmed MG diagnosis. METHODS: All repetitive nerve stimulation examinations at the Uppsala University Hospital rendering an MG diagnosis during 1996 to 2014 were analyzed. The deltoid, trapezius, anconeus, nasalis, abductor digiti quinti, and frontalis muscles were examined...
September 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28738395/outcomes-of-preoperative-and-postoperative-corticosteroid-therapies-in-myasthenia-gravis
#5
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...
2017: European Neurology
https://www.readbyqxmd.com/read/28731133/irrelevance-of-anti%C3%A2-nachr-cytoplasmic-loop-antibody-in-the-degree-of-myasthenia-gravis
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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...
September 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
#15
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
#16
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
#17
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
#18
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
#19
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/28512504/a-case-report-of-drug-induced-myopathy-involving-extraocular-muscles-after-combination-therapy-with-tremelimumab-and-durvalumab-for-non-small-cell-lung-cancer
#20
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
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