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Ocular myasthenia

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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
#1
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
https://www.readbyqxmd.com/read/28505981/severe-exacerbation-of-myasthenia-gravis-associated-with-checkpoint-inhibitor-immunotherapy
#2
Dana S Cooper, Matthew N Meriggioli, Philip D Bonomi, Rabia Malik
Monoclonal antibodies that target either PD-1 or PD-L1 have recently been approved for treatment of advanced non-small cell lung cancer. These antibodies are immune checkpoint inhibitors which have been shown to exacerbate Myasthenia Gravis (MG) and other autoimmune diseases. While effective in preventing tumor cells from evading immune attack, immune checkpoint inhibitors such as nivolumab, an antibody directed against the programmed cell death protein-1 (PD-1) receptor located on T-cells, may also cause immune dysregulation and could cause or potentiate pre-existing autoimmune conditions...
May 6, 2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/28495138/effect-of-ethnic-origin-and-gender-on-the-clinical-manifestations-of-myasthenia-gravis-among-the-jewish-population-in-israel
#3
Ali Asmail, Anat Kesler, Vivian E Drory, Hadar Kolb, Arnon Karni
Reports on patients with myasthenia gravis (MG) of different ethnic origins demonstrated differences in weakness distribution and serological results. We studied MG characteristics in a cohort of Ashkenazi (ASH) and non-Ashkenazi (NASH) Jewish origin according to their ethnic origins and gender. The frequency of age of MG onset was distributed in a bi-modal fashion in the female patients and increased gradually over time, with a peak around 70years of age in the male patients. Ocular MG was more frequent in males and ASH patients...
June 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28495046/transient-neonatal-myasthenia-gravis-due-to-a-mother-with-ocular-onset-of-anti-muscle-specific-kinase-myasthenia-gravis
#4
Ju-Yeun Lee, Ju-Hong Min, Sueng-Han Han, Jinu Han
We describe a 27-year-old pregnant female with new onset of conjugate gaze deficit during the third trimester of pregnancy. Repetitive nerve stimulation tests, neostigmine tests, and acetylcholine receptor antibody assays were all negative. The patient delivered a normal healthy baby at a local clinic via cesarean section. The baby became hypotonic and had respiratory failure several minutes after birth. The result of acetylcholine receptor antibody was negative in the neonate. The neonate became healthy spontaneously and was extubated after 21 days of ventilation care...
April 4, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28492464/validity-of-forced-eyelid-closure-test-a-novel-clinical-screening-test-for-ocular-myasthenia-gravis
#5
Supanut Apinyawasisuk, Xinkai Zhou, Jack J Tian, Giancarlo A Garcia, Rustum Karanjia, Alfredo A Sadun
BACKGROUND: Forced eyelid closure test (FECT) is a clinical screening test developed from the original Cogan lid twitch (CLT) sign to assist in the diagnosis of ocular myasthenia gravis (OMG), We evaluated the sensitivity and specificity of FECT compared with CLT and benchmarked to standard diagnostic tests. METHODS: This study was a retrospective chart review of 48 patients using electronic medical records of those that presented with ptosis and/or diplopia at Doheny Eye Institute, University of California, Los Angeles between February 2015 and April 2016...
May 10, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28458152/increased-expression-of-p2x7-receptor-in-peripheral-blood-mononuclear-cells-correlates-with-clinical-severity-and-serum-levels-of-th17-related-cytokines-in-patients-with-myasthenia-gravis
#6
Yong Zhang, Yanyan Zhang, Hao Li, Xiao Jia, Xiuying Zhang, Yan Xia, YuZhong Wang, Linlin Fu, Chenghua Xiao, Deqin Geng
OBJECTIVES: P2X7R is a well-documented activator of innate and adaptive immune responses. We aimed to measure the expression levels of P2X7R in peripheral blood mononuclear cells (PBMCs) from patients with myasthenia gravis (MG) and to investigate whether the expression of P2X7R is associated with pathogenesis of MG. PATIENTS AND METHODS: A total of 32 patients with MG (12 generalized MG (GMG) and 20 Ocular MG (OMG) and 22 healthy donors were recruited in this study...
June 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28457757/juvenile-myasthenia-gravis-in-norway-clinical-characteristics-treatment-and-long-term-outcome-in-a-nationwide-population-based-cohort
#7
T H Popperud, M I Boldingh, M Rasmussen, E Kerty
BACKGROUND: This study aimed to characterize juvenile myasthenia gravis in a national population-based cohort in Norway, and to evaluate long-term outcome and potential differences correlated with prepubertal versus postpubertal disease onset. PATIENTS AND METHODS: Patients with onset of myasthenia gravis aged ≤18 years were identified through multiple strategies. Retrospective clinical data were collected by means of medical charts. All patients had an updated clinical examination...
April 20, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28457040/tolosa-hunt-syndrome-and-ocular-myasthenia-a-rare-coexistence-or-real-association
#8
Jaydeep Majumdar, Sarmishtha Mukhopadhyay, Abhijeet Sharan, Sarbani Sengupta, Bhaskar Ghosh
Tolosa- Hunt syndrome is a rare steroid responsive disorder caused by granulation tissue involving the cavernous sinus or superior orbital fissure presenting as painful ophthalmoplegia and facial pain. In this report, we describe coexistence of Tolosa-Hunt syndrome with ocular myasthenia which may point towards an autoimmune etiological basis behind the cavernous sinus granulation tissue formation and also offered therapeutic challenge to ameliorate the symptoms.
February 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28431604/a-reappraisal-of-diagnostic-tests-for-myasthenia-gravis-in-a-large-asian-cohort
#9
Yew Long Lo, Raymond P Najjar, Kelvin Y Teo, Sharon L Tow, Jing Liang Loo, Dan Milea
BACKGROUND: Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disease characterized by weakness of bodily skeletal muscles. Office-based diagnostic tests such as repetitive nerve stimulation (RNS), single fiber electromyography (SFEMG), and the ice test, are used to refine the differential clinical diagnosis of this disease. Evaluating the clinical sensitivity and specificity of these tests, however, may be confounded by lack of a gold standard, non-blinding, incorporation bias, use of non-representative populations and retrospective data...
May 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28429750/correlations-of-tnf-%C3%AE-gene-promoter-polymorphisms-with-the-risk-of-thymoma-associated-myasthenia-gravis-in-a-northern-chinese-han-population
#10
H-W Yang, P Lei, Y-C Xie, Z-L Han, D Li, S-H Wang, Z-L Sun
This study was performed with the aim to investigate the correlations of tumor necrosis factor-alpha (TNF-α) gene promoter polymorphisms with the risk of thymoma-associated myasthenia gravis (T-MG) in a northern Chinese Han population. Between June 2005 and June 2015, 305 MG patients (150 males and 155 females, MG group) and 293 healthy volunteers (negative control (NC) group) were enrolled in this study. Among the MG patients, there were 121 patients with thymoma-associated MG (T-MG group) and 184 without T-MG (NT-MG group)...
April 21, 2017: Cancer Gene Therapy
https://www.readbyqxmd.com/read/28420327/clinical-predictors-for-the-prognosis-of-myasthenia-gravis
#11
Lili Wang, Yun Zhang, Maolin He
BACKGROUND: Clinical predictors for myasthenia gravis relapse and ocular myasthenia gravis secondary generalization during the first two years after disease onset remain incompletely identified. This study attempts to investigate the clinical predictors for the prognosis of Myasthenia Gravis. METHODS: Eighty three patients with myasthenia gravis were concluded in this study. Baseline characteristics were analyzed as predictors. RESULTS: Relapse of myasthenia gravis developed in 26 patients (34%)...
April 19, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28364296/juvenile-onset-myasthenia-gravis-autoantibody-status-clinical-characteristics-and-genetic-polymorphisms
#12
Yu Hong, Geir Olve Skeie, Paraskevi Zisimopoulou, Katerina Karagiorgou, Socrates J Tzartos, Xiang Gao, Yao-Xian Yue, Fredrik Romi, Xu Zhang, Hai-Feng Li, Nils Erik Gilhus
Myasthenia gravis (MG) is an autoimmune disorder mediated by antibodies against proteins at the neuromuscular junction. Juvenile-onset MG (JMG) has been reported to have special characteristics. It is still unclear whether there are any pathogenic and genetic differences between juvenile and adult MG. In this study, we evaluated the clinical characteristics, autoantibody status (antibodies against AChR, MuSK, LRP4, titin and RyR) and genetic susceptibility (CHRNA1, CTLA4 and AIRE) in 114 Chinese JMG patients, and compared with 207 young adult MG patients (onset age 18-40 years)...
May 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28350570/low-density-lipoprotein-receptor-related-protein-4-positive-myasthenia-gravis-in-a-double-seronegative-electromyography-negative-patient
#13
Joshua M Kruger, Dimitrios Karussis, Paraskevi Zisimopoulou, Panayiota Petrou
We describe a patient with ocular myasthenia gravis, where single-fiber electromyography and testing for acetylcholine receptor and muscle-specific kinase antibodies were negative. However, antibodies to low-density lipoprotein receptor-related protein 4 (LRP4) were positive, and this prompted appropriate management. We recommend that testing for LRP4 antibodies be considered when the clinical suspicion for myasthenia gravis is high despite negative conventional diagnostic tests.
March 27, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28338183/psychogenic-convergence-spasm-mimicking-ocular-myasthenia
#14
C Scoppetta, G Di Gennaro
A 14-year-old girl presented with a two-years history of fluctuating convergent strabismus, diplopia, and reading difficulty. She has been previously diagnosed by experienced neurologists as having ocular myasthenia and she had been treated for two years with anticholinesterase inhibitors and immunomodulatory drugs. After a thorough medical interview and neurological examination, a diagnosis of psychogenic convergence spasm was made. The patient was then reassured and the symptoms immediately disappeared. She also had psychotherapy and maintained a condition of sustained freedom from symptoms...
March 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28318833/bibliometric-analysis-of-the-scientific-production-as-regards-statin-use-for-ophthalmological-symptoms-of-myasthenia-gravis
#15
S M Salado-Font, F López-Muñoz, F J Povedano-Montero, F Labella Quesada
INTRODUCTION: The first symptoms of myasthenia gravis (MG) usually involve weakness of the ocular muscles, making it relevant that ophthalmologists have updated information on studies as regards its relationship with the consumption of drugs, such as statins. MATERIALS AND METHODS: A bibliometric analysis was performed using the Scopus database and by a search strategy in the selection of documents containing descriptors related to statins in the «Title» («TI») field and the descriptors «ophthalm *', «myast *', «visual *' in other fields of the document (period 1986-2015)...
March 16, 2017: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/28293536/early-onset-bilateral-juvenile-myasthenia-gravis-masquerading-as-simple-congenital-ptosis
#16
Md Shahid Alam, Pratheeba Devi Nivean
Myasthenia gravis is an autoimmune disorder affecting the neuromuscular junction. Ocular myasthenia gravis presents as ptosis with extraocular motility restriction and is prone to be misdiagnosed as third nerve palsy or congenital or aponeurotic ptosis. Juvenile ocular myasthenia gravis in very young children is difficult to diagnose and can be easily labeled as a case of congenital ptosis, the more so when the condition is bilateral. We present a case of a two-year-old child who presented with bilateral ptosis and was diagnosed as a case of simple congenital ptosis elsewhere with the advice to undergo tarsofrontalis sling surgery...
2017: GMS Ophthalmology Cases
https://www.readbyqxmd.com/read/28284335/oxidative-modifications-of-blood-serum-proteins-in-myasthenia-gravis
#17
Monika Adamczyk-Sowa, Edyta Bieszczad-Bedrejczuk, Sabina Galiniak, Izabela Rozmiłowska, Damian Czyżewski, Grzegorz Bartosz, Izabela Sadowska-Bartosz
Myasthenia gravis (MG) is an autoimmune disease caused by production of antibodies against acetylcholine receptors of the neuromuscular junction (Ab). The aim of this study was to ascertain if oxidative stress accompanies MG by estimation of the several independent parameters of oxidative damage, mainly the levels of oxidative modifications of blood serum proteins. The group studied consisted of 50 MG patients (28 females and 22 males), 24 with ocular MG (OMG) and 26 with generalized MG (GMG), of mean age of 66...
April 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28273704/-clinical-and-genetic-characteristics-of-children-with-leigh-syndrome
#18
F Fang, Y Shen, D M Shen, Z M Liu, C H Ding, W C Zhang, S Z Sun, J L Lyu, T L Han, X H Wang, W H Zhang, X Y Yang, J W Li, H S Wu
Objective: To investigate the clinically and genetic characteristics of children with Leigh syndrome. Method: Patients with clinically diagnosed Leigh syndrome(LS)in the department of Neurology, Beijing Children's Hospital from January 2013 to February 2016 underwent the mitochondrial DNA (mtDNA) and nuclear DNA (nDNA) detecting with next generation sequencing (NGS) technology. The clinical data of gene confirmed cases were retrospectively collected and analyzed. The differences in the onset age, clinical manifestations, lactic acid level and MRI results between the mtDNA variation and nDNA variation were compared and analyzed...
March 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28268468/detection-of-myasthenia-gravis-using-electrooculography-signals
#19
T Liang, M I Boulos, B J Murray, S Krishnan, H Katzberg, K Umapathy
Myasthenia gravis (MG) is an autoimmune neuromuscular disorder resulting from skeletal muscle weakness and fatigue. An early common symptom is fatigable weakness of the extrinsic ocular muscles; if symptoms remain confined to the ocular muscles after a few years, this is classified as ocular myasthenia gravis (OMG). Diagnosis of MG when there are mild, isolated ocular symptoms can be difficult, and currently available diagnostic techniques are insensitive, non-specific or technically cumbersome. In addition, there are no accurate biomarkers to follow severity of ocular dysfunction in MG over time...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28255284/clinical-evaluation-of-blepharoptosis-distinguishing-age-related-ptosis-from-masquerade-conditions
#20
REVIEW
Michelle W Latting, Alison B Huggins, Douglas P Marx, Joseph N Giacometti
Aponeurotic ptosis accounts for the majority of acquired ptosis encountered in clinical practice. Other types of ptosis include traumatic, mechanical, neurogenic, and myogenic. In addition to true ptosis, some patients present with pseudoptosis caused by globe dystopia, globe asymmetry, ocular misalignment, or retraction of the contralateral lid. It is particularly important for the clinician to rule out neurologic causes of ptosis such as dysfunction of the third cranial nerve, Horner's syndrome, and myasthenia gravis, as these conditions can be associated with significant systemic morbidity and mortality...
February 2017: Seminars in Plastic Surgery
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