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ocular myasthenia gravis

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
Seung Ah Chung, Seran Jang
No abstract text is available yet for this article.
October 2016: Korean Journal of Ophthalmology: KJO
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
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
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
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
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
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
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
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
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
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
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
Katarina Ivana Tudor, Damir Petravić, Anđela Jukić, Zlatko Juratovac
OBJECTIVE: To present a patient with a sudden onset ocular tilt reaction (OTR) and review recent knowledge and evolving insights of the underlying pathophysiological mechanisms of skew deviation and OTR. METHODS: A middle-aged hypertensive man who had previously suffered stroke with good recovery presented with sudden-onset double vision, slurred speech, ataxia, and a head tilt. Romberg test was positive. The patient denied having disturbances of visual acuity, eye pain, or recent trauma...
July 29, 2016: Seminars in Ophthalmology
Elena Cortés-Vicente, Eduard Gallardo, María Ángeles Martínez, Jordi Díaz-Manera, Luis Querol, Ricard Rojas-García, Isabel Illa
IMPORTANCE: Double-seronegative myasthenia gravis (dSNMG) includes patients with myasthenia gravis (MG) without detectable antibodies to the nicotinic acetylcholine receptor (AChR) or to muscle-specific tyrosine kinase (MuSK). The lack of a biomarker hinders the diagnosis and clinical management in these patients. Cortactin, a protein acting downstream from agrin/low-density lipoprotein receptor-related protein 4 (LRP4)/MuSK, has been described as an antigen in dSNMG. OBJECTIVE: To describe the frequency and clinical features of patients with dSNMG who have cortactin antibodies...
September 1, 2016: JAMA Neurology
Donald B Sanders, Gil I Wolfe, Michael Benatar, Amelia Evoli, Nils E Gilhus, Isabel Illa, Nancy Kuntz, Janice M Massey, Arthur Melms, Hiroyuki Murai, Michael Nicolle, Jacqueline Palace, David P Richman, Jan Verschuuren, Pushpa Narayanaswami
OBJECTIVE: To develop formal consensus-based guidance for the management of myasthenia gravis (MG). METHODS: In October 2013, the Myasthenia Gravis Foundation of America appointed a Task Force to develop treatment guidance for MG, and a panel of 15 international experts was convened. The RAND/UCLA appropriateness methodology was used to develop consensus guidance statements. Definitions were developed for goals of treatment, minimal manifestations, remission, ocular MG, impending crisis, crisis, and refractory MG...
July 26, 2016: Neurology
Sławomir Kroczka, Katarzyna Stasiak, Marek Kaciński
BACKGROUND: Myasthenia gravis (MG) is an autoimmunologic disorder. It is characterized by various clinical symptoms and their dependency upon the exertion and the rest as well. MATERIAL AND METHODS: Between 2002-20014 in the Neurophysiology Laboratory at the Chair of Pediatric and Adolescent Neurology, Jagiellonian University in Krakow, the electrophysiological repetitive nerve stimulation study were performed in 44 children. The clinical picture and positive electrophysiological test were the ground to diagnose MG in 19 of them (12 girls and 7 boys)...
2016: Przegla̧d Lekarski
Flavia C N Machado, João A Kouyoumdjian, Paulo E Marchiori
OBJECTIVES: To estimate jitter parameters in the orbicularis oculi muscle using concentric needle electrode (CNE) in patients with myasthenia gravis (MG) and determine its diagnostic accuracy for jitter analysis (CNEMG-jitter). METHODS: CNEMG-jitter was performed in 20 healthy subjects and 33 MG patients using the voluntary contraction technique. Receiver operating characteristic (ROC) curves were constructed to determine cutoff points with the best sensitivity/specificity combination for jitter analysis...
June 27, 2016: Muscle & Nerve
Darlene Deters, Stephanie L Fowler, Raymundo Orozco, Patrick R Smith, Shelby Spurlock, Darlene Blackmon, Samantha Thomas
Myasthenia gravis is a chronic neuromuscular disorder that causes skeletal muscle weakness. Typically, myasthenia gravis affects the ocular, bulbar, neck, proximal limbs, and respiratory muscles. Although the presentation is typically observed with complaints of vision and bulbar symptoms such as diplopia, dystonia, and dysphagia, this article presents a case study of an elderly man with a history of increasing upper extremity weakness with complaints of worsening hand dexterity and intermittent episodes of expressive aphasia...
July 2016: Dimensions of Critical Care Nursing: DCCN
Su Jung Baik, Tae Hun Kim, Hye In Kim, Jeong Yeon Rhie
Myasthenia gravis is occasionally associated with thymoma that needs surgical resection and may progress to severe respiratory failure. We experienced a rare case of myasthenia crisis during antiviral therapy for chronic hepatitis C, in whom mediastinal thymoma was discovered and successfully managed with surgical thymectomy and meticulous medical care.A 47-year-old-male patient complained of sudden diplopia 1 week after stopping 11-week administration of pegylated-interferon and ribavirin for chronic hepatitis C...
May 2016: Medicine (Baltimore)
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