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Ocular myasthenia

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https://www.readbyqxmd.com/read/27886889/update-on-ocular-myasthenia-gravis
#1
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
#2
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
#3
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
#4
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
#5
Bedile Irem Tiftikcioglu, Irem Fatma Uludag, Yasar Zorlu, İbrahim Pirim, Ufuk Sener, Figen Tokucoglu, Meltem Korucuk
No abstract text is available yet for this article.
October 31, 2016: 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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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"...
October 3, 2016: 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
#12
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
#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 2003-2013. 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
#14
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
#15
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
https://www.readbyqxmd.com/read/27569990/repetitive-facial-nerve-stimulation-in-myasthenia-gravis-1min-after-muscle-activation-is-inferior-to-testing-a-second-muscle-at-rest
#16
Alon Abraham, Majed Alabdali, Abdulla Alsulaiman, Ari Breiner, Carolina Barnett, Hans D Katzberg, Vera Bril
OBJECTIVES: To explore the increased diagnostic yield of repetitive nerve stimulation (RNS) following activation for myasthenia gravis (MG) diagnosis, and compare with testing an additional muscle at rest only. METHODS: We performed a retrospective chart review of patients diagnosed with MG attending the neuromuscular clinic from 2013 to 2015 and extracted data on electrophysiological studies, including frequency of decrement of 10% or above, with facial RNS at rest, and following activation...
October 2016: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/27564076/extensive-postradiation-ocular-and-diffuse-cranial-neuromyotonia-mimicking-myasthenia-gravis
#17
Todd A Hardy, Andrew W Lee, Con Yiannikas, Celia S Chen, Stephen W Reddel
BACKGROUND: Ocular neuromyotonia is a rare, but well-recognized, complication of cranial irradiation. CASE REPORT: Using figures and videos, we report a 52-year-old man with extensive ocular, brainstem, and lower cranial nerve neuromyotonia postradiation therapy for a fourth ventricle glioma who, in the context of an apparently positive edrophonium test, was initially misdiagnosed with myasthenia gravis. CONCLUSIONS: This is the first case of postirradiation neuromyotonia to be reported with such extensive cranial nerve and brainstem involvement...
September 2016: Neurologist
https://www.readbyqxmd.com/read/27543617/augmentation-of-circulating-follicular-helper-t-cells-and-their-impact-on-autoreactive-b-cells-in-myasthenia-gravis
#18
Cun-Jin Zhang, Ye Gong, Wenli Zhu, Yuan Qi, Chun-Sheng Yang, Ying Fu, Guoqiang Chang, Yujing Li, Samuel Shi, Kristofer Wood, Shafeeq Ladha, Fu-Dong Shi, Qiang Liu, Yaping Yan
Myasthenia gravis (MG) is a chronic humoral immunity-mediated autoimmune disorder of the neuromuscular junction characterized by muscle weakness. Follicular helper T (Tfh) cells may be the key Th cell subset that promotes MG development, as their major function is helping B cell activation and Ab production. Aberrance of thymus-derived Tfh cells might be implicated in autoimmune diseases including MG; just how circulating Tfh cells, especially those from patients with a normal thymus, contribute to MG pathogenesis remains to be uncovered...
October 1, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/27529328/diagnosis-of-tensilon-negative-ocular-myasthenia-gravis-by-daily-selfie
#19
Elan L Guterman, James V Botelho, Jonathan C Horton
The initial symptoms of myasthenia gravis are usually ptosis and diplopia. The diagnosis is often confirmed by testing for anti-acetylcholine receptor antibodies or by observing the effects of intravenous edrophonium (Tensilon) injection. However, these standard tests may be negative in patients with isolated ocular findings. We present the case of an 83-year-old woman with negative serologic and Tensilon testing. She was asked to photograph herself daily. The resulting sequence of daily selfies captured striking fluctuations in her ocular alignment and ptosis...
September 2016: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/27511866/new-strategy-for-improving-the-diagnostic-sensitivity-of-repetitive-nerve-stimulation-in-myasthenia-gravis
#20
Hanna Bou Ali, Emmanuelle Salort-Campana, Aude Marie Grapperon, Julien Gallard, Jerome Franques, Amandine Sevy, Emilien Delmont, Annie Verschueren, Jean Pouget, Shahram Attarian
INTRODUCTION: The diagnostic sensitivity of repetitive nerve stimulation (RNS) in patients with myasthenia gravis (MG) varies as a function of the number of muscles or the choice of muscles studied. METHODS: By exploring 12 muscles bilaterally, we evaluated the global sensitivity of RNS at rest, the sensitivity in different clinical forms, and the sensitivity of different combinations of muscles studied. RESULTS: The global sensitivity of RNS was 82%, and specificity was 100%...
August 11, 2016: Muscle & Nerve
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