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Ocular myasthenia

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https://www.readbyqxmd.com/read/29858757/antibody-profile-may-predict-outcome-in-ocular-myasthenia-gravis
#1
Giuliana Galassi, Marco Mazzoli, Alessandra Ariatti, Shaniko Kaleci, Franco Valzania, Paolo F Nichelli
An unsolved issue remains whether there are clinical and immunological features to predict in a single patient the risk of conversion from ocular Myasthenia Gravis (OMG) to generalized disease (GMG) as 50-60% of patients may progress within 1-2 years since onset. Anti-acetylcholine receptor antibodies (AChR Abs) are found in up to 50% of OMG patients; muscle-specific tyrosine kinase antibodies (MuSK-Abs) are present in about 70% of the whole seronegative (SN), who usually develop a severe disease with bulbar involvement...
June 1, 2018: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/29845611/clinical-and-epidemiological-features-of-myasthenia-gravis-in-chilean-population
#2
G Cea, D Martinez, R Salinas, C Vidal, L Hoffmeister, A Stuardo
OBJECTIVE: To provide an estimated prevalence and describe the clinical features of myasthenia gravis (MG) in Chile. METHOD: We carried out (i) a prevalence study of MG using the capture-recapture method and the hospital register of pyridostigmine prescription in South-East Santiago (ii) a nationwide survey of MG patients. RESULTS: Prevalence in adults in South-East Santiago was estimated to be 8.36/100 000 inhabitants (CI: 95%, 7.98-8.80)...
May 29, 2018: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29804819/circulating-microrna-mir-21-5p-mir-150-5p-and-mir-30e-5p-correlate-with-clinical-status-in-late-onset-myasthenia-gravis
#3
Liis Sabre, Paul Maddison, Girija Sadalage, Philip Alexander Ambrose, Anna Rostedt Punga
There are no biomarkers for late onset myasthenia gravis (LOMG; onset >50 years). We evaluated circulating microRNA in a discovery cohort of 4 LOMG patients and 4 healthy controls and in a prospective diagnostic validation cohort of 73 LOMG patients (48 male) with longitudinal follow-up samples. In immunosuppression naïve patients, levels of miRNAs miR-150-5p, miR-21-5p and miR-30e-5p decreased in parallel with clinical improvement after initiation of immunosuppression and their levels positively correlated with the clinical MG composite score...
May 8, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29780960/waldenstrom-macroglobulinemia-involving-the-superior-rectus-muscle
#4
J B Hellman, G J Harocopos, L K Lin
Purpose: We present the first reported case of Waldenstrom macroglobulinemia in the right superior rectus causing diplopia. Observations: A 72-year-old man with a 6-month history of untreated asymptomatic Waldenstrom macroglobulinemia presented with 2 years of diagonal binocular diplopia that was previously thought to be due to ocular myasthenia gravis. Examination showed mild right proptosis and right hypotropia, and MRI revealed a focal lesion of the right superior rectus muscle...
June 2018: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29773681/decreased-expression-of-circulating-aire-and-increased-tfh-tfr-cells-in-myasthenia-gravis-patients
#5
Sijia Zhao, Jiaqi Ding, Shengyuan Wang, Chuan Li, Peng Guo, Min Zhang, Zhuyi Li
Myasthenia gravis (MG) is a rare prototypical autoimmune disorder caused by antibodies (Ab) against postsynaptic membrane proteins. Most reports have investigated the role of Autoimmune regulator gene (Aire) in thymic tissue in machianism of MG initiation. So far, the expression of Aire in human peripheral blood cells(we call it circulating Aire expression in the following passage) has not been reported. Herein, we explore the expression of Aire in peripharal blood, circulating T follicular helper (cTfh) and T follicular regulatory (cTfr) cells in MG patients...
May 17, 2018: Bioscience Reports
https://www.readbyqxmd.com/read/29730659/myxedema-coma-due-to-hashimoto-thyroiditis-a-rare-but-real-presentation-of-failure-to-thrive-in-infancy
#6
Ryan A Heksch, Rohan K Henry
BACKGROUND: Hashimoto thyroiditis (HT) is uncommon in infancy, and myxedema coma (MC) is even less common. While prior reports have documented these entities separately, to our knowledge, MC in combination with HT has not been reported before in this age group. METHODS/RESULTS: A 10-month-old female presented with ptosis, lethargy, dysphagia, and failure to thrive (FTT). She developed hypotension, bradycardia, hypothermia, and apnea requiring intubation. Initial thyroid-stimulating hormone was 422 μIU/mL, and free thyroxine was < 0...
May 4, 2018: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29689030/checkpoint-inhibitor-immune-therapy-systemic-indications-and-ophthalmic-side-effects
#7
Lauren A Dalvin, Carol L Shields, Marlana Orloff, Takami Sato, Jerry A Shields
PURPOSE: To review immune checkpoint inhibitor indications and ophthalmic side effects. METHODS: A literature review was performed using a PubMed search for publications between 1990 and 2017. RESULTS: Immune checkpoint inhibitors are designed to treat system malignancies by targeting one of three ligands, leading to T-cell activation for attack against malignant cells. These ligands (and targeted drug) include cytotoxic T-lymphocyte antigen-4 (CTLA-4, ipilimumab), programmed death protein 1 (PD-1, pembrolizumab, nivolumab), and programmed death ligand-1 (PD-L1, atezolizumab, avelumab, durvalumab)...
June 2018: Retina
https://www.readbyqxmd.com/read/29683988/case-report-ocular-myasthenia-gravis-associated-with-in-vitro-fertilization-procedures
#8
Yung Ju Yoo, Sang Beom Han, Hee Kyung Yang, Jeong-Min Hwang
SIGNIFICANCE: Ocular myasthenia gravis is a localized form of myasthenia gravis, which is a postsynaptic disorder of the neuromuscular junction that causes fluctuating weakness of extraocular muscles resulting from autoimmune mechanisms. In women with myasthenia, changes in sex hormone levels and administration of corticosteroids can trigger or worsen symptoms of myasthenia gravis. PURPOSE: To describe a case of seronegative ocular myasthenia gravis whose first symptom appeared a day after in vitro fertilization procedure...
May 2018: Optometry and Vision Science: Official Publication of the American Academy of Optometry
https://www.readbyqxmd.com/read/29655451/muscle-specific-tyrosine-kinase-and-myasthenia-gravis-owing-to-other-antibodies
#9
REVIEW
Michael H Rivner, Mamatha Pasnoor, Mazen M Dimachkie, Richard J Barohn, Lin Mei
Around 20% of patients with myasthenia gravis are acetylcholine receptor antibody negative; muscle-specific tyrosine kinase antibodies (MuSK) were identified as the cause of myasthenia gravis in 30% to 40% of these cases. Anti MuSK myasthenia gravis is associated with specific clinical phenotypes. One is a bulbar form with fewer ocular symptoms. Others show an isolated head drop or symptoms indistinguishable from acetylcholine receptor-positive myasthenia gravis. These patients usually respond well to immunosuppressive therapy, but not as well to cholinesterase inhibitors...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655449/diagnosis-of-myasthenia-gravis
#10
REVIEW
Mamatha Pasnoor, Mazen M Dimachkie, Constantine Farmakidis, Richard J Barohn
Myasthenia gravis (MG) diagnosis is primarily clinically based. By the end of the clinical evaluation, clinicians have a sense as to whether presenting symptoms and elicited signs are weakly or strongly supportive of MG. Diagnostic tests can reaffirm the clinicians' impression. Edrophonium testing is rarely used but helpful in cases of measurable ptosis. Decremental response on slow-frequency repetitive nerve stimulation has a modest diagnostic yield in ocular MG but is helpful in generalized MG cases. The most sensitive test is single-fiber electromyography...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655448/generalized-myasthenia-gravis-classification-clinical-presentation-natural-history-and-epidemiology
#11
REVIEW
Michael K Hehir, Nicholas J Silvestri
Myasthenia gravis (MG) is a rare disease, but the most common disorder of the neuromuscular junction. It is the prototypic autoimmune disease most commonly caused by antibodies to the acetylcholine receptor (AChR) leading to characteristic fatigable weakness of the ocular, bulbar, respiratory, axial, and limb muscles. The majority of patients with MG first present with ocular symptoms. Most patients with MG will experience at least 1 exacerbation of symptoms throughout the course of their illness. This article will cover the epidemiology, clinical presentation, classification, and natural history of MG...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655447/ocular-myasthenia
#12
REVIEW
Mohammed Al-Haidar, Michael Benatar, Henry J Kaminski
Ocular myasthenia is a form of myasthenia gravis in which weakness is restricted to the ocular muscles and may produce significant visual disability. Patients present with fluctuating ptosis, diplopia, or a combination of both. Examination may show any type of ocular motility deficit ranging from isolated muscle palsy to complete ophthalmoplegia. Cogan lid twitch, enhanced ptosis, peek sign, and saccadic fatigue are specific examination findings that support the clinical diagnosis of myasthenia gravis. Confirmation of the diagnosis is challenging with autoantibody serology, and repetitive nerve stimulation studies are often negative...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29569949/altered-expression-of-transcription-factors-irf4-and-irf8-in-peripheral-blood-b-cells-is-associated-with-clinical-severity-and-circulating-plasma-cells-frequency-in-patients-with-myasthenia-gravis
#13
Yong Zhang, Xiao Jia, Yan Xia, Hao Li, Fei Chen, Jie Zhu, Xiuying Zhang, Yanyan Zhang, YuZhong Wang, Yanan Xu, Meng Pan, Xiaoyu Huang, Tingyan Yu, Linlin Fu, Chenghua Xiao, Deqin Geng
Previous studies have shown that interferon regulatory factor-4 (IRF4) and IRF8 play critical but distinct roles in the differentiation of B cells into plasma cells (PCs). In the present study, we aimed to measure the expression levels of IRF4 and IRF8 in B cells from patients with myasthenia gravis (MG) and to investigate whether the expression of IRF4 and IRF8 associates with pathogenesis of MG. A total of 35 anti-acetylcholine receptor (AChR) antibody (Ab)-positive patients with MG [20 generalized MG (GMG) and 15 ocular MG (OMG) and 25 healthy donors were recruited in this study...
May 2018: Autoimmunity
https://www.readbyqxmd.com/read/29555378/-an-update-on-myasthenia-gravis
#14
S Martínez Torre, I Gómez Molinero, R Martínez Girón
Myasthenia gravis is one of the most common disorders that affect neuromuscular transmission. It is currently one of the most understood and characterised autoimmune disorders Its typical symptoms are fluctuating weakness and fatigue that affects a combination of ocular muscles, bulbar functions, as well as limb and respiratory muscles, which are due to an immune attack against the postsynaptic membrane of the neuromuscular junction. The diagnosis of myasthenia gravis is based on clinical and serological test...
March 16, 2018: Semergen
https://www.readbyqxmd.com/read/29554816/ocular-surface-alterations-and-in-vivo-confocal-microscopic-characteristics-of-corneas-in-patients-with-myasthenia-gravis
#15
Kadriye Erkan Turan, Sibel Kocabeyoglu, Can Ebru Bekircan-Kurt, Figen Bezci, Sevim Erdem-Ozdamar, Murat Irkec
PURPOSE: To evaluate ocular surface alterations and characteristics of corneal basal epithelium and subbasal nerves in patients with myasthenia gravis. MATERIALS AND METHODS: Myasthenia gravis patients (n = 21) and healthy controls (n = 20) were enrolled. All participants underwent ocular surface testing in the following order: tear break-up time, lissamine green staining, Schirmer I test with anesthesia, and Ocular Surface Disease Index questionnaire. The Cochet-Bonnet esthesiometer was used to measure corneal sensitivity...
March 1, 2018: European Journal of Ophthalmology
https://www.readbyqxmd.com/read/29535672/clinical-characteristics-of-juvenile-myasthenia-gravis-in-southern-china
#16
Xin Huang, Yingkai Li, Huiyu Feng, Pei Chen, Weibin Liu
Objectives: To describe the clinical profile, clinical outcomes and factors that may affect the outcome of juvenile myasthenia gravis (JMG) patients in southern China. Methods: We reviewed information relating to JMG patients treated and evaluated at the First Affiliated Hospital, Sun Yat-sen University, between 1998 and 2015. The study involved 327 JMG patients who had been followed up for ≥1 year. Results: Overall, 77.4% patients showed initial symptoms in the prepubertal period (<12 years)...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29518096/myasthenia-gravis-seronegative-for-acetylcholine-receptor-antibodies-in-south-korea-autoantibody-profiles-and-clinical-features
#17
Kee Hong Park, Patrick Waters, Mark Woodhall, Bethan Lang, Thomas Smith, Jung-Joon Sung, Kwang-Kuk Kim, Young-Min Lim, Jee-Eun Kim, Byung-Jo Kim, Jin-Sung Park, Jeong-Geon Lim, Dae-Seong Kim, Ohyun Kwon, Eun Hee Sohn, Jong Seok Bae, Byung-Nam Yoon, Nam-Hee Kim, Suk-Won Ahn, Jeeyoung Oh, Hyung Jun Park, Kyong Jin Shin, Yoon-Ho Hong
Acquired myasthenia gravis (MG) is a prototype autoimmune disease of the neuromuscular junction, caused in most patients by autoantibodies to the muscle nicotinic acetylcholine receptor (AChR). There seem to be ethnic and regional differences in the frequency and clinical features of MG seronegative for the AChR antibody. This study aimed to describe the autoantibody profiles and clinical features of Korean patients with generalized MG seronegative for the AChR antibody. A total of 62 patients with a high index of clinical suspicion of seronegative generalized MG were identified from 18 centers, and we examined their sera for antibodies to clustered AChR, muscle-specific tyrosine kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (LRP4) by cell-based assays (CBA) and to MuSK by radioimmunoprecipitation assay (RIPA)...
2018: PloS One
https://www.readbyqxmd.com/read/29507855/pure-ocular-weakness-as-the-initial-manifestation-of-lambert-eaton-myasthenic-syndrome
#18
Nakul Katyal, Raghav Govindarajan
Pure ocular presentation of Lambert-Eaton syndrome is not a common phenomenon. Such presentation poses significant diagnostic challenges and requires conscientious evaluation. In this review, we have described a case of a patient with pure ocular weakness, initially diagnosed as seronegative ocular myasthenia which on further evaluation was found to have ocular Lambert-Eaton myasthenic syndrome (LEMS).
December 31, 2017: Curēus
https://www.readbyqxmd.com/read/29490884/repetitive-nerve-stimulation-and-jitter-measurement-with-disposable-concentric-needle-electrode-in-newly-diagnosed-myasthenia-gravis-patients
#19
Nermin Gorkem Sirin, Elif Kocasoy Orhan, Hacer Durmus, Feza Deymeer, Mehmet Baris Baslo
INTRODUCTION: The aim of this study was to define the diagnostic accuracy of concentric needle (CN)-jitter in newly diagnosed myasthenia gravis (MG) patients and to compare CN-jitter with repetitive nerve stimulation. METHODS: In 30 MG patients, repetitive nerve stimulation in 4 muscles (orbicularis oculi, nasalis, trapezius and abductor digiti minimi) and CN-jitter of extensor digitorum (ED) and frontalis muscles were evaluated. RESULTS: Twenty-eight of 30 patients (93%) had high jitter in at least one muscle...
February 25, 2018: Neurophysiologie Clinique, Clinical Neurophysiology
https://www.readbyqxmd.com/read/29455019/ocular-myasthenia-induced-by-rivaroxaban-in-patient-with-deep-vein-thrombosis
#20
Enrique M San Norberto, Irene García-Saiz, Diana Gutiérrez, Liliana Domingos, Carlos Vaquero
The non-vitamin K antagonist oral anticoagulant rivaroxaban is indicated in prevention and treatment of venous thromboembolism (VTE). A 60-year-old male patient complained of bilateral ptosis after administration of rivaroxaban for deep vein thrombosis (DVT). Myasthenia gravis (MG) was confirmed by positive serum antiacetylcholine receptor antibody test. No mediastinal thymoma was found. The ocular myasthenia reversed after discontinuing rivaroxaban treatment. Nevertheless, ptosis recurred and chronic oral pyridostigmine bromide treatment was necessary...
May 2018: Annals of Vascular Surgery
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