scholarly journals Pure Ocular Myasthenia gravis

2018 ◽  
Vol 02 (01) ◽  
pp. E51-E55
Author(s):  
Lukas Kirzinger ◽  
Sophie Schötz ◽  
Berthold Schalke
Keyword(s):  
Neuromuscular Junction ◽  
Myasthenia Gravis ◽  
Diagnostic Procedure ◽  
Cholinesterase Inhibitors ◽  
Separate Entity ◽  
Ocular Myasthenia ◽  
Randomized Controlled ◽  
Ocular Symptoms ◽  
Randomized Controlled Studies ◽  
Ocular Myasthenia Gravis

AbstractPure ocular myasthenia was always described as a separate entity in historical publications. More than 50% of patients develop generalized symptoms in the course of the disease. Therefore pure ocular symptoms can be the beginning of generalized myasthenia, nevertheless there is a small group of patients who suffer lifelong purely ocular symptoms. The basis, therefore, seems to be special immunological, biochemical and pathophysiological mechanisms leading to specific local changes of the neuromuscular junction. The diagnostic procedure is hampered as antibodies against acethylcholine receptors and MuSK can be demonstrated in less than 50% of these patients. Therapy of pure ocular myasthenia does not differ fundamentally from other subgroups of myasthenia gravis and is implemented with cholinesterase inhibitors, steroids and common immunosuppressants. Thymomectomy is not recommended in general, but randomized controlled studies are lacking.

scholarly journals Do early prednisolone and other immunosuppressant therapies prevent generalization in ocular myasthenia gravis in Western populations: a systematic review and meta-analysis

2019 ◽  
Vol 12 ◽  
pp. 175628641987652
Author(s):  
Mingxia Li ◽  
Fangfang Ge ◽  
Rongjing Guo ◽  
Zhe Ruan ◽  
Yanwu Gao ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Controlled Trial ◽  
Meta Analysis ◽  
Disease Onset ◽  
Mild Disease ◽  
Ocular Myasthenia ◽  
Randomized Controlled ◽  
Asian Populations ◽  
Prospective Randomized Controlled Trial ◽  
Ocular Myasthenia Gravis

Background: The majority of ocular myasthenia gravis (OMG) patients will progress to generalized myasthenia gravis (GMG), usually within 2 years of disease onset. The aim of this meta-analysis was to evaluate the effect of early prednisolone and other immunosuppressants therapy on the generalization rate in OMG patients. Methods: We searched the CENTRAL, EMBASE, and MEDLINE databases via the Ovid SP database for all relevant publications on 16 July 2018. Results: Eight studies comprising a total of 547 participants were included in our meta-analysis. Compared with pyridostigmine treatment, prednisolone and other immunosuppressants therapy produced an odds ratio (OR) for the development of GMG of 0.19 [95% confidence interval (CI), 0.11–0.30; I2 = 37%], indicating that early prednisolone and other immunosuppressants therapy reduced the generalization rate in OMG by 81%. Conclusions: Early prednisolone and other immunosuppressants therapy can significantly reduce the risk of generalization in OMG patients, and should be considered in newly diagnosed OMG patients. Due to the inclusion of retrospective studies, this noted effect might have been related to corticosteroids, especially when immunosuppressants used at low dosages and in mild disease. Additionally, the data derived from Western populations, thus a prospective randomized controlled trial (RCT) is warranted to confirm this effect of early prednisolone and other immunosuppressants therapy on OMG generalization both in Western and Asian populations.

scholarly journals Seeking spark- ocular myasthenia gravis in a juvenile – an Indian case report

2020 ◽  
Vol 5 (7) ◽  
pp. 190-193
Author(s):  
Dr. Usha BR. ◽  
◽  
Dr. Nandhini K ◽  
Dr. Chaitra MC ◽  
◽  
...  
Keyword(s):  
Case Report ◽  
Neuromuscular Junction ◽  
Myasthenia Gravis ◽  
Autoimmune Disorder ◽  
Bilateral Ptosis ◽  
Ocular Myasthenia ◽  
Ocular Myasthenia Gravis ◽  
History Of ◽  
High Index Of Suspicion

Myasthenia gravis (MG) is a rare autoimmune disorder affecting neuromuscular junction by muscleweakness. Myasthenia gravis can be generalized or localized as ocular myasthenia gravis. Casepresentation: We report an 8-year-old boy who presented with 10 days history of drooping of botheyelids and 8 days history of diplopia. Examination revealed bilateral ptosis. A diagnosis of JuvenileOcular Myasthenia gravis was made when symptoms improved with intramuscular Edrophoniumadministration. He was commenced on oral Neostigmine at a dose of 2mg/Kg/ day,4 hourly individed doses and is on regular follow up and had a good response. Conclusion: Ocular Myastheniagravis (OMG) is a rare disease in itself. A high index of suspicion is required in a juvenile as it iseven rarer.

Prognosis of Ocular Myasthenia Gravis in an Argentinian Population

2018 ◽  
Vol 79 (3-4) ◽  
pp. 113-117 ◽  
Author(s):  
Florencia Aguirre ◽  
Andres M. Villa
Keyword(s):  
Myasthenia Gravis ◽  
Retrospective Analysis ◽  
Remission Rate ◽  
Acetylcholinesterase Inhibitors ◽  
Ocular Involvement ◽  
Achr Antibody ◽  
Ocular Myasthenia ◽  
Ocular Symptoms ◽  
Increased Risk ◽  
Ocular Myasthenia Gravis

Background: We investigated the prognosis of patients with myasthenia gravis (MG) initially presenting with only ocular symptoms in an Argentinian population. Summary: We performed a retrospective analysis of 61 patients with MG with pure ocular involvement at onset. Generalized MG (gMG) developed in 73.7% of patients, while the rest only exhibited ocular symptoms throughout the course of the disease (ocular MG [oMG]). The AChR antibody (AChR-ab) was found in 81.4% of the gMG and 50% of the oMG cases. AChR-ab titers were also significantly higher in the gMG than in the oMG group. Patients with oMG more frequently required treatment only with acetylcholinesterase inhibitors and showed more remissions. Key Messages: Our study showed high generalization in patients with MG debuting with ocular symptoms. Patients with AChR seropositivity and higher AChR-ab titers had an increased risk for gMG, while patients with oMG showed lower corticosteroid requirements and a higher remission rate.

THUR 231 Developing a new rating scale for ocular myasthenia gravis

2018 ◽  
Vol 89 (10) ◽  
pp. A33.3-A33
Author(s):  
Sui H Wong ◽  
Eduardo Miranda ◽  
Helena Lee ◽  
Eric Eggenberger ◽  
Wayne Cornblath ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Rating Scales ◽  
Rating Scale ◽  
Observational Cohort Study ◽  
Ocular Myasthenia ◽  
Ocular Symptoms ◽  
Composite Scale ◽  
Ocular Myasthenia Gravis ◽  
Observational Cohort ◽  
Future Work

Ocular Myasthenia Gravis (OMG) causes ptosis and diplopia, which can be disabling. For this study, OMG is defined as MG patients who have ocular symptoms only and no generalised involvement. A robust way of assessing the severity of OMG symptoms is important for research, to assess treatment and outcome. The rating scales recommended for MG research have a predominant focus on generalised disease, and are insufficiently sensitive for OMG due to the limited number of ocular questions. This study aims to create a new rating scale for OMG that is sensitive, reliable and clinically usable. We present our proposal of such a rating scale, which incorporates physician- and patient-rated components. We report the preliminary results of this pilot observational cohort study, in 60 patients with OMG. We compare the results of this with the MG composite scale.Future work is planned to validate this rating scale and to develop this alongside the MG Impairment Index (MGII). For future phases of this study we plan to assess the usability of this rating scale by Neurologists without specialist Neuro-ophthalmology.We invite feedback from Neurologists at the ABN.

scholarly journals Thymectomy in ocular myasthenia gravis before generalization results in a higher remission rate

2019 ◽  
Author(s):  
Feng Li ◽  
Zhongmin Li ◽  
Yanli Chen ◽  
Gero Bauer ◽  
Deniz Uluk ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Remission Rate ◽  
Post Intervention ◽  
Data Set ◽  
Ocular Myasthenia ◽  
Ocular Symptoms ◽  
Ocular Myasthenia Gravis ◽  
Before And After ◽  
Robotic Thymectomy ◽  
Lost To Follow Up

Abstract OBJECTIVES This study aimed to compare the outcomes of patients with ocular myasthenia gravis (OMG) who underwent thymectomy before generalization with the outcomes of those who underwent thymectomy after generalization. METHODS We retrospectively reviewed patients who underwent robotic thymectomy for myasthenia gravis between January 2003 and February 2018. Patients who presented with purely ocular symptoms at myasthenia gravis onset were eligible for inclusion. Exclusion criteria were patients who were lost to follow-up and patients who underwent re-thymectomy. Patients with OMG who developed generalization before thymectomy were categorized into gOMG group and those who did not were categorized into OMG group. The primary outcome was complete stable remission according to the Myasthenia Gravis Foundation of America Post-Intervention Status (MGFA-PIS). RESULTS One hundred and sixty-five (66 males and 99 females) out of 596 patients with myasthenia gravis were eligible for inclusion. Of these, there were 73 and 92 patients undergoing thymectomy before and after the generalization of OMG, respectively. After propensity score matching, a data set of 130 patients (65 per group) was formed and evaluating results showed no statistical differences between the 2 groups. The estimated cumulative probabilities of complete stable remission at 5 years were 49.5% [95% confidence interval (CI) 0.345–0.611] in the OMG group and 33.4% (95% CI 0.176–0.462) in the gOMG group (P = 0.0053). Similar results were also found in patients with non-thymomatous subgroup [55 patients per group, OMG vs gOMG, 53.5% (95% CI 0.370–0.656) vs 28.9% (95% CI 0.131–0.419), P = 0.0041]. CONCLUSIONS Thymectomy in OMG before generalization might result in a higher rate of complete stable remission than thymectomy after generalization.

Update on Ocular Myasthenia Gravis

2019 ◽  
Vol 39 (06) ◽  
pp. 749-760 ◽  
Author(s):  
Meabh O'Hare ◽  
Christopher Doughty
Keyword(s):  
Neuromuscular Junction ◽  
Myasthenia Gravis ◽  
Clinical Features ◽  
Clinical Examination ◽  
Autoimmune Disorder ◽  
Therapeutic Options ◽  
Diagnostic Approach ◽  
Ocular Myasthenia ◽  
Ocular Myasthenia Gravis ◽  
Recent Developments

AbstractMyasthenia gravis is an antibody-mediated autoimmune disorder of the post-synaptic neuromuscular junction resulting in fluctuating, fatigable weakness. Most patients first present with extraocular symptoms (diplopia and/or ptosis), and in 15% of cases symptoms will remain restricted to only the extraocular muscles (ocular myasthenia gravis [OMG]). The history and clinical examination are of the utmost importance in correctly identifying OMG patients, as supportive serologic or electrodiagnostic studies are frequently nondiagnostic. In this review, we outline a diagnostic approach to OMG (focusing on key clinical features), discuss therapeutic options, and highlight recent developments in the understanding of OMG.

scholarly journals GRAVIS MYASTHENIA – ACUTE ERYTHROBLASTIC LEUKEMIA CORRELATION

2017 ◽  
Vol 64 (2) ◽  
pp. 147-150
Author(s):  
Angela Mirela Soare ◽  
◽  
Cezar Mihai Popescu ◽  
Keyword(s):  
Rheumatoid Arthritis ◽  
Myasthenia Gravis ◽  
Thyroid Dysfunction ◽  
Cholinesterase Inhibitors ◽  
The United States ◽  
Neuromuscular Disorder ◽  
Ocular Myasthenia ◽  
Ocular Myasthenia Gravis ◽  
Autoimmune Condition ◽  
Muscle Groups

Myasthenia Gravis is a chronic autoimmune neuromuscular disorder that is characterized by fluctuating weakness of the voluntary muscle groups. It occurs in all races, at any age (especially under 40 or over 60), both genders, with a prevalence of 20/100,000 population in the United States. About 15 percent of patients with myasthenia gravis have thymomas, much rarely another solid tumor, thyroid dysfunction and more often another autoimmune condition, such as rheumatoid arthritis, lupus, Biermer anaemia. We present a 72 year-old male patient with ocular myasthenia gravis treated with cholinesterase inhibitors, that, after many laboratory exams, was diagnosed with acute erythroblastic leukemia, a very rare clinical association.

scholarly journals Mitral Valve Replacement with Thymectomy in a Patient with Ocular Myasthenia Gravis: Case Report

2019 ◽  
Vol 22 (5) ◽  
pp. E340-E342
Author(s):  
Ovidiu Stiru ◽  
Mihai Stefan ◽  
Roxana Carmen Geana ◽  
Diana Sorostinean ◽  
Răzvan Radu ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Myasthenia Gravis ◽  
Valve Replacement ◽  
Mitral Valve Replacement ◽  
Mitral Valve Stenosis ◽  
Management Problem ◽  
Ocular Myasthenia ◽  
Anaesthetic Agents ◽  
Ocular Symptoms ◽  
Ocular Myasthenia Gravis

Patients diagnosed with ocular myasthenia gravis (MG) and mitral valve disease represent a significant perioperative management problem, especially for the anaesthesiologist, due to complex inter-actions between the disease, drugs to treat the disease, and anaesthetic agents, such as neuromuscu-lar blocking agents (NMBAs). This paper describes the successful management of a 31-year-old female with mitral valve stenosis and ocular MG who was diagnosed with MG 4 years prior to the indication for cardiac surgery. Preoperatively, the patient was under treatment with Pyridostigmine and Prednisone. Mitral valve replacement and full thymectomy were performed, under general anaesthesia, using Fentanyl, Sevoflurane and low doses of non-depolarising NMBAs. The postoperative course was uneventful, the patient was extubated at 6 hours postoperatively, in-tensive care unit stay was 48 hours, and the patient was discharged after 6 days without any compli-cations. After 3 months, at the follow-up examination, the patient’s ocular symptoms (eyelid ptosis) disappeared.

Abstract #1154: Ocular Myasthenia Gravis in the Setting of Graves’ Disease

2016 ◽  
Vol 22 ◽  
pp. 277-278
Author(s):  
Lima Lawrence ◽  
Aleida Rodriguez ◽  
Tahira Yasmeen ◽  
Erin Drever
Keyword(s):  
Myasthenia Gravis ◽  
Graves Disease ◽  
Ocular Myasthenia ◽  
Ocular Myasthenia Gravis
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