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ocular myasthenia gravis

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https://www.readbyqxmd.com/read/28120340/anti-lrp4-autoantibodies-in-chinese-patients-with-myasthenia-gravis
#1
Yuan Li, Yifan Zhang, Gang Cai, Dian He, Qingqing Dai, Zhu Xu, Lan Chu
: Instruction: We assessed antibodies against low-density lipoprotein receptor-related protein 4 (LRP4-Ab) in a Chinese population with myasthenia gravis (MG). METHODS: Serum samples from 116 patients and 80 controls were collected. Acetylcholine receptor antibodies(AChR-Ab) and muscle-specific receptor tyrosine kinase antibodies(MuSK-Ab) were tested using an enzyme-linked immune absorption assay, and LRP4-Ab was identified using a cell-based assay. MG patients with neither AChR-Ab nor MuSK-Ab were defined as double-seronegative MG (dSN-MG)...
January 24, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28119492/correlation-of-hla-d-q-and-tnf-%C3%AE-gene-polymorphisms-with-ocular-myasthenia-gravis-combined-with-thyroid-associated-ophthalmopathy
#2
Hong-Wei Yang, Ying-Xue Wang, Jie Bao, Shu-Hui Wang, Ping Lei, Zhao-Lin Sun
The study aims to explore the correlation of HLA-DQ and TNF-α gene polymorphisms with ocular myasthenia gravis (OMG) combined with thyroid associated ophthalmopathy (TAO). From March 2009 to March 2015, 56 OMG patients complicated with TAO (OMG + TAO group), 134 patients diagnosed with OMG only (OMG group) and 236 healthy individuals (control group) were enrolled in this study. Polymerase chain reaction-sequence specific primer (PCR-SSP) was used for HLA-DQ genotyping and polymerase chain reaction-restriction fragment length polymorphism (PCR-RELP) for TNF-α genotyping...
January 24, 2017: Bioscience Reports
https://www.readbyqxmd.com/read/28079665/anesthetic-management-of-a-patient-with-antimuscle-specific-kinase-antibody-positive-myasthenia-gravis-undergoing-an-open-cholecystectomy-a-case-report
#3
Masahiko Akatsu, Yukihiro Ikegami, Choichiro Tase, Koichi Nishikawa
Myasthenia gravis (MG) is an autoimmune disease characterized by the production of antibodies against the acetylcholine receptor, muscle-specific kinase (MuSK), or other proteins at the neuromuscular junction. MG with antibodies against MuSK (MuSK-MG) has been described recently. Here, we report the first case of anesthetic management of a patient with MuSK-MG undergoing an open cholecystectomy. In our case, propofol and remifentanil-based anesthesia were used for successful management without using muscle relaxants...
January 11, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28061919/myasthenia-gravis-and-its-aeromedical-implications
#4
Tania Jagathesan, Michael D O'Brien
BACKGROUND: Myasthenia gravis is an autoimmune condition where antibodies form against the acetylcholine receptors at the neuromuscular junction, eventually causing damage to the motor end plate. The clinical features include muscle fatigability as well as ocular, bulbar, and limb weakness, which can have implications on the role of a pilot or air traffic controller. This retrospective study reviewed the United Kingdom Civil Aviation Authority (UK CAA) experience of myasthenia gravis...
January 1, 2017: Aerospace Medicine and Human Performance
https://www.readbyqxmd.com/read/28029691/electrophysiological-testing-is-correlated-with-myasthenia-gravis-severity
#5
Alon Abraham, Ari Breiner, Carolina Barnett, Hans D Katzberg, Leif E Lovblom, Mylan Ngo, Vera Bril
INTRODUCTION: Electrophysiological studies play an important role in the diagnosis of myasthenia gravis (MG). OBJECTIVES: To explore the correlation of jitter and decrement with various clinical symptoms and signs and disease severity. METHODS: We performed a retrospective chart review of 75 MG patients who attended the neuromuscular clinic from April 2013 to May 2014. We compared clinical characteristics between patients with high jitter (>100 µs) and decrement (>10%), and patients with lower values to explore the correlations and optimal thresholds of jitter and decrement for different clinical features...
December 28, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27886889/update-on-ocular-myasthenia-gravis
#6
REVIEW
Stacy V Smith, Andrew G Lee
Ocular myasthenia gravis (OMG) is a localized form of myasthenia gravis in which autoantibodies directed against acetylcholine receptors block or destroy these receptors at the postsynaptic neuromuscular junction. The hallmark of OMG is a history of painless weakness or fatigability of the extraocular muscles and ptosis with normal pupillary function and visual acuity. Clinical, laboratory, electrophysiologic, and pharmacologic tests are available for diagnosis. Treatment can begin with symptom management; there is no cure...
February 2017: Neurologic Clinics
https://www.readbyqxmd.com/read/27855632/response-to-treatment-of-myasthenia-gravis-according-to-clinical-subtype
#7
MULTICENTER STUDY
Tetsuya Akaishi, Yasushi Suzuki, Tomihiro Imai, Emiko Tsuda, Naoya Minami, Yuriko Nagane, Akiyuki Uzawa, Naoki Kawaguchi, Masayuki Masuda, Shingo Konno, Hidekazu Suzuki, Hiroyuki Murai, Masashi Aoki, Kimiaki Utsugisawa
BACKGROUND: We have previously reported using two-step cluster analysis to classify myasthenia gravis (MG) patients into the following five subtypes: ocular MG; thymoma-associated MG; MG with thymic hyperplasia; anti-acetylcholine receptor antibody (AChR-Ab)-negative MG; and AChR-Ab-positive MG without thymic abnormalities. The objectives of the present study were to examine the reproducibility of this five-subtype classification using a new data set of MG patients and to identify additional characteristics of these subtypes, particularly in regard to response to treatment...
November 17, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27854225/myasthenia-gravis-unusual-presentations-and-diagnostic-pitfalls
#8
Carmelo Rodolico, Daniela Parisi, Simona Portaro, Fiammetta Biasini, Stefano Sinicropi, Annamaria Ciranni, Antonio Toscano, Sonia Messina, Olimpia Musumeci, Giuseppe Vita, Paolo Girlanda
BACKGROUND: Myasthenia gravis (MG) is an autoimmune disorder presenting with fluctuating, fatigable muscle weakness. Initial symptoms classically involve ocular and proximal limb muscles. Rarely, MG may onset with unusual features, so it can be misdiagnosed with other neuromuscular diseases. OBJECTIVE: To describe unusual and atypical presentations of MG in a large cohort of patients, considering and discussing diagnostic difficulties and pitfalls. METHODS: We report on 21 out of 508 MG patients, coming to our department in the last 27 years and presenting with atypical or unusual features...
August 30, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27853084/double-seronegative-myasthenia-gravis-with-anti-lrp4-antibodies-presenting-with-dropped-head-and-acute-respiratory-insufficiency
#9
Goichi Beck, Taiki Yabumoto, Kousuke Baba, Tsutomu Sasaki, Osamu Higuchi, Hidenori Matsuo, Hideki Mochizuki
We herein report the case of a 72-year-old man demonstrating myasthenia gravis (MG) with a dropped head and acute respiratory insufficiency. There was no ocular, bulbar, or limb involvement. The patient was seronegative for anti-acetylcholine receptor (AChR) antibodies and anti-muscle-specific tyrosine kinase (MuSK) antibodies. Subsequent tests showed seropositivity for anti-low-density lipoprotein receptor-related protein 4 (LRP4) antibodies. The addition of steroid pulse therapy resulted in a full remission of his respiratory symptoms...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27802446/human-leucocyte-antigen-b50-is-associated-with-conversion-to-generalized-myasthenia-gravis-in-patients-with-pure-ocular-onset
#10
Bedile Irem Tiftikcioglu, Irem Fatma Uludag, Yasar Zorlu, İbrahim Pirim, Ufuk Sener, Figen Tokucoglu, Meltem Korucuk
OBJECTIVES: The aim of this study was to investigate the associations between major histocompatibility complex (MHC) class I and II alleles and disease characteristics in Turkish patients with myasthenia gravis (MG). SUBJECTS AND METHODS: The MHC class I and II alleles of 108 unrelated MG patients were genotyped. The human leucocyte antigen (HLA) distribution of all MG patients and subgroups of MG patients (grouped according to disease characteristics) was compared to that of 250 healthy controls...
2017: Medical Principles and Practice: International Journal of the Kuwait University, Health Science Centre
https://www.readbyqxmd.com/read/27781146/prevalence-and-impact-of-autoimmune-thyroid-disease-on-myasthenia-gravis-course
#11
Justyna Kubiszewska, Beata Szyluk, Piotr Szczudlik, Zbigniew Bartoszewicz, Małgorzata Dutkiewicz, Maksymilian Bielecki, Tomasz Bednarczuk, Anna Kostera-Pruszczyk
OBJECTIVES: Autoimmune thyroid diseases (ATDs) frequently accompany myasthenia gravis (MG) and may influence its course. We aimed to determine the association and impact of ATD with early- (<50 years), late-onset MG, or thymoma-MG. MATERIALS AND METHODS: Prevalence of ATD was measured in a cross-sectional study of 343 consecutive patients with MG (236 F, 107 M) aged 4-89 years; 83.8% were seropositive, in 2.9%, anti-MuSK antibodies were detected. Concentrations of antithyroid peroxidase antibodies, antithyroglobulin antibodies, antithyrotropin receptor antibodies, and TSH level were measured in all patients...
October 2016: Brain and Behavior
https://www.readbyqxmd.com/read/27776019/two-cases-of-clinical-myasthenia-gravis-associated-with-pembrolizumab-use-in-responding-melanoma-patients
#12
Bella H V Nguyen, James Kuo, Anadian Budiman, Hayden Christie, Sayed Ali
Immune checkpoint inhibitors have changed the landscape of the treatment of multiple solid malignancies, and have been used increasingly in the recent years. Although usually well tolerated, given the relative inexperience of using immune checkpoint inhibitors, we are still learning of new side effects from the treatment. We report on two cases of ocular myasthenia gravis that occurred after treatment with pembrolizumab, an antiprogrammed-death (anti-PD1) monoclonal antibody for advanced melanoma in responding patients...
October 21, 2016: Melanoma Research
https://www.readbyqxmd.com/read/27749461/diagnostic-accuracy-of-single-fiber-electromyography-for-myasthenia-gravis-in-patients-followed-longitudinally
#13
John A Morren, Kerry H Levin, Robert W Shields
INTRODUCTION: The literature lacks data on accuracy of single fiber electromyography (SFEMG) for myasthenia gravis (MG) patients followed longitudinally. METHODS: We included patients with a clinical suspicion of MG who received SFEMG and follow-up at our institution between 2003 and 2013. Data collected included demographics, symptom details, clinical deficits, other diagnostic testing results, MG medication regimen, duration on treatment, response to therapy, and ultimate diagnosis after follow-up...
October 2016: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/27729761/ocular-myasthenia-gravis-in-monozygotic-twins-with-mirror-image-myopic-anisometropia
#14
Seung Ah Chung, Seran Jang
No abstract text is available yet for this article.
October 2016: Korean Journal of Ophthalmology: KJO
https://www.readbyqxmd.com/read/27706425/clinical-and-genetic-basis-of-congenital-myasthenic-syndromes
#15
Paulo Victor Sgobbi de Souza, Gabriel Novaes de Rezende Batistella, Valéria Cavalcante Lino, Wladimir Bocca Vieira de Rezende Pinto, Marcelo Annes, Acary Souza Bulle Oliveira
Neuromuscular junction disorders represent a wide group of neurological diseases characterized by weakness, fatigability and variable degrees of appendicular, ocular and bulbar musculature involvement. Its main group of disorders includes autoimmune conditions, such as autoimmune acquired myasthenia gravis and Lambert-Eaton syndrome. However, an important group of diseases include congenital myasthenic syndromes with a genetic and sometimes hereditary basis that resemble and mimick many of the classic myasthenia neurological manifestations, but also have different presentations, which makes them a complex clinical, therapeutic and diagnostic challenge for most clinicians...
September 2016: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/27690672/myasthenia-gravis-recent-advances-in-immunopathology-and-therapy
#16
John-Ih Lee, Sebastian Jander
Myasthenia gravis is the most frequent acquired disorder of neuromuscular transmission. In the majority of cases, pathogenic antibodies against components of the postsynaptic muscle endplate membrane can be detected. In recent years there have been significant advances in the pathophysiological understanding and therapy of the disease. Areas covered: PubMed searches were conducted for the term 'myasthenia gravis' cross-referenced with the terms 'immunology', 'subgroups', 'antibody', 'ocular', 'thymoma', 'treatment' and 'thymectomy'...
March 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/27677768/cd19-tim-1-b-cells-are-decreased-and-negatively-correlated-with-disease-severity-in-myasthenia-gravis-patients
#17
Yong Zhang, Xiuying Zhang, Yan Xia, Xiao Jia, Hao Li, Yanyan Zhang, Zhen Shao, Ning Xin, Mingfeng Guo, Jing Chen, ShuangShuang Zheng, YuZhong Wang, Linlin Fu, Chenghua Xiao, Deqin Geng, Yonghai Liu, Guiyun Cui, Ruiguo Dong, Xiaoyu Huang, Tingyan Yu
T cell immunoglobulin mucin domain-1(Tim-1) was recently identified to be critical and essential for optimal regulatory B cells function in maintaining immune tolerance. We aimed to measure the expression levels of Tim-1 on B cells from patients with Myasthenia Gravis (MG) and to investigate whether the expression of Tim-1 is associated with pathogenesis of MG. A total of 34 patients with MG (18 generalized MG (GMG) and 16 ocular MG (OMG) and 24 healthy donors were recruited in this study. The quantitative myasthenia gravis score (QMGS) was used to evaluate the clinical severity...
September 28, 2016: Immunologic Research
https://www.readbyqxmd.com/read/27648598/diagnostic-accuracy-of-single-fiber-emg-for-myasthenia-gravis-in-patients-followed-longitudinally
#18
John A Morren, Kerry H Levin, Robert W Shields
INTRODUCTION: The literature lacks data on accuracy of single fiber electromyography (SFEMG) for myasthenia gravis (MG) patients followed longitudinally. METHODS: We included patients with a clinical suspicion of MG who received SFEMG and follow-up at our institution between 2003-2013. Data collected included demographics, symptom details, clinical deficits, other diagnostic testing results, MG medication regimen, duration on treatment, response to therapy and ultimate diagnosis after follow-up...
March 31, 2016: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/27609276/thymus-involvement-in-myasthenia-gravis-epidemiological-and-clinical-impacts-of-different-self-tolerance-breakdown-mechanisms
#19
Arnon Karni, Ali Asmail, Vivian E Drory, Hadar Kolb, Anat Kesler
The reasons for the abrogation of self-immunological tolerance in patients with myasthenia gravis (MG) may be different between those with concomitant thymic hyperplasia or thymoma, and those with no evidence of thymic involvement. We conducted a retrospective observational case series study to investigate the epidemiology as well as the clinical, serologic, and electromyographic (EMG) characteristics of individuals diagnosed as having MG. We found that the average age at MG onset of patients with either thymic hyperplasia or thymoma was much younger (by ~20years) than that of MG patients without thymic involvement...
September 15, 2016: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/27581849/juvenile-myasthenia-gravis-in-korea-subgroup-analysis-according-to-sex-and-onset-age
#20
Ha Neul Lee, Hoon-Chul Kang, Joon Soo Lee, Heung Dong Kim, Ha Young Shin, Seung Min Kim, Il Nam Sunwoo, Young-Mock Lee
Juvenile myasthenia gravis presents before 18 years of age with different characteristics according to racial background and pubertal development. The authors aimed to determine the clinical characteristics of children and adolescents of Korean ethnicity with myasthenia gravis, and evaluate the presentation and clinical outcomes according to the sex and onset age of the patients. The authors recruited 88 Korean juvenile myasthenia gravis patients between September 2005 and August 2015. Worse clinical severity from presentation, more aggressive treatment strategies, and worse final treatment outcomes were noted in girls with postpubertal onset than in the other patients...
August 31, 2016: Journal of Child Neurology
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