scholarly journals Myasthenia Gravis Exacerbation and Myasthenic Crisis Associated With COVID19: Case Series and Literature Review.

2021 ◽  
Author(s):  
Cleonisio Leite Rodrigues ◽  
Hermany Capistrano Freitas ◽  
Paulo Reges Oliveira Lima ◽  
Pedro Helder de Oliveira Junior ◽  
José Marcelino Aragão Fernandes ◽  
...  
Keyword(s):  
Literature Review ◽  
Neuromuscular Junction ◽  
Hospital Mortality ◽  
Myasthenia Gravis ◽  
Viral Infections ◽  
Case Series ◽  
Previous Treatment ◽  
Autoimmune Disorder ◽  
Myasthenic Crisis

Abstract Myasthenia Gravis (MG) is an autoimmune disorder of the neuromuscular junction that can be exacerbated by many viral infections, including COVID19. Management of MG exacerbations is challenging in this scenario. We report 8 cases of MG exacerbation or myasthenic crisis associated with COVID19 and discuss prognosis and treatment based on a literature review. Most patients were female (7/8), with an average age of 47.1 years. Treatment was immunoglobulin (IVIG) in 3 patients, plasma exchange (PLEX) in 2 patients, and adjustment of baseline drugs in 3. In-hospital mortality was 25% and 37.5% in 2-month follow-up. This is the largest case series of MG exacerbation or myasthenic crisis due to COVID19 to this date. Mortality was considerably higher than in myasthenic crisis of other etiologies. Previous treatment for MG or acute exacerbation treatment did not seem to interfere with prognosis, although sample size was too small to draw definitive conclusions. Further studies are needed to understand the safety and effectiveness of interventions in this setting, particularly of PLEX, IVIG, rituximab and tocilizumab.

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.

scholarly journals Hybrid treatment of thoracoabdominal aortic aneurysm: Case series and review of the literature

2019 ◽  
Vol 76 (3) ◽  
pp. 331-340
Author(s):  
Petar Zlatanovic ◽  
Igor Koncar ◽  
Miroslav Markovic ◽  
Ranko Trailovic ◽  
Vladimir Cvetic ◽  
...  
Keyword(s):  
Overall Survival ◽  
Aortic Aneurysm ◽  
Literature Review ◽  
Hospital Mortality ◽  
Case Series ◽  
Thoracoabdominal Aortic Aneurysm ◽  
Graft Patency ◽  
Technical Failure ◽  
Hybrid Treatment

Background/Aim. Open thoracoabdominal aortic aneurysm (ThAAAs) repair is a challenging mission. Total endovascular approach is performed at selected institutions in developing countries, however these are not generalizable. Hybrid procedures offer an alternative approach. The aim of this study was to present our results of the ThAAA hybrid treatment and a literature review. Methods. This is a retrospective study of all patients that underwent the hybrid ThAAA repair at our institution between January 2011 and January 2018. Hybrid ThAAA repair was done as a twostaged procedure ? open visceral debranching followed by stent-graft placement (TEVAR). The following data from eligible studies were extracted and analyzed: first author, publication year, patient sample, 30-day/in-hospital mortality, permanent paraplegia rate, presence of endoleaks and graft patency after the follow-up period, overall survival and mean follow-up period. Results. Seven patients underwent the hybrid ThAAA repair at our institution. Neither intraoperative death nor technical failure due to TEVAR was observed. Mean follow-up rate after discharge was 51.71 months [95% confidence interval (CI): 14.67?88.74 months]. At the time of the follow-up, all bypasses were patent and no prosthesis migration was documented. After one year, a case of type Ib endoleak was identified and treated successfully. Twenty-five studies were eligible for the literature review. Primary technical success was 91.69% (95% CI: 85.34?97.24%). Mean percentage of permanent paraplegia was 5.27% (95% CI: 3.55?7.01%). Visceral graft patency during the mean follow-up of 27.54 months (95% CI: 17.41?37.66 months) was 94.5% (95% CI: 92.5?96.5%). Mean pooled percentage rate of overall endoleaks during the follow-up period was 16.72% (95% CI: 11.15?22.29%). Analysis revealed 15.32% (95% CI: 11.04?19.61%) of 30- day/in-hospital mortality and 65.98% (95% CI: 58.15? 73.81%) of overall survival after the follow-up period. Conclusion. Although thought as less invasive, the hybrid ThAAA repair is still associated with a considerable morbidity and mortality.

Predictive Score for In-Hospital Mortality of Myasthenic Crisis: A Retrospective Chinese Cohort Study

2019 ◽  
Vol 81 (5-6) ◽  
pp. 287-293
Author(s):  
Zhiguo Lv ◽  
Huahua Zhong ◽  
Xiao Huan ◽  
Jie Song ◽  
Chong Yan ◽  
...  
Keyword(s):  
Septic Shock ◽  
Cardiac Arrest ◽  
Hospital Mortality ◽  
Myasthenia Gravis ◽  
Scoring System ◽  
Laboratory Data ◽  
Case Series ◽  
Predictive Score ◽  
Hospital Death ◽  
Myasthenic Crisis

Introduction: Myasthenic crisis (MC) is a life-threatening condition usually occurred in patients with myasthenia gravis (MG). Objective: On the basis of a retrospective case series review, we try to develop a scoring system to evaluate the probability for in-hospital death in MC patients. Methods: We retrospectively reviewed 78 MC patients who were hospitalized from January 2014 to December 2018. Clinical and laboratory data including 17 variables were analyzed univariately. The main clinical outcome was defined as the in-hospital death. Then eligible variables were evaluated by a stepwise multivariate regression and a scoring system was then generated. Calibration and discrimination methods were used to evaluate and validate the model performance. Results: The overall in-hospital mortality was 11.5% (9/78) in the MC cohort. Five clinical variables including Myasthenia Gravis Foundation of America (MGFA) classification at onset, septic shock, thymoma classification, cardiac arrest, and the lowest serum albumin were found to be associated with in-hospital mortality. Further 3 variables entered the final regression, and internal validation showed that the area under the curve was 0.919 (95% CI 0.788–1.000). A scoring system with a full credit of 7 points was generated to predict the in-hospital mortality (MGFA at onset, 2 points; septic shock, 4 points; and cardiac arrest, 3 points). Conclusions: A 7-point scoring model was established on the basis of a retrospective review of MC patients to predict the in-hospital mortality.

scholarly journals Manajemen Perawatan Neurointensif pada Miastenia Gravis

2021 ◽  
Vol 1 (12) ◽  
pp. 1002-1010
Author(s):  
Dicki Apriansyah Haris Putra ◽  
Lale Sirin Rifdah S ◽  
Putu Mega Asri D ◽  
Muhammad Mahfuzzahroni
Keyword(s):  
Myasthenia Gravis ◽  
Surgical Therapy ◽  
Autoimmune Disorder ◽  
Myasthenic Crisis ◽  
Immunosuppressant Therapy ◽  
Ventilation Non Invasive ◽  
Short Term ◽  
Non Invasive ◽  
Non Invasive Ventilation ◽  
Motor End Plate

Myasthenia gravis (MG) is an autoimmune disorder that affects neuromuscular transmission, causing generalized or localized weakness characterized by fatigue. Myasthenia gravis is most commonly associated with antibodies to the acetylcholine receptor (AChR) on the motor end plate in the postsynaptic neuron. This article aims to determine the appropriate neurointensive management in patients with myasthenia gravis with complications of myasthenic crisis. The writing of this article includes various sources originating from scientific journals and government guidelines and related agencies. Source searches were carried out on online portals for journal publications such as MedScape, Google Scholar (scholar.google.com) and the National Center for Biotechnology Information (ncbi.nlm.nih.gov), with the keyword “Myasthenia Gravis”. The management of myasthenia gravis can be done in various ways, namely, mechanical intubation and ventilation, non-invasive ventilation, pridostigmine as an anticholinesterase inhibitor, immunosuppressant therapy, short term immunotherapy, intravenous immunoglobulin, and surgical therapy. In the treatment of myasthenia gravis, the main goal is to restore muscle condition, especially patient productivity where the management of myasthenia gravis consists of management of myasthenic crisis, cholinergic crisis, symptoms, immunosuppressant therapy, and thymectomy surgical therapy if a tumor is indicated.

Other Proven and Putative Autoimmune Disorders of the Peripheral Nervous System

2017 ◽  
Author(s):  
Doris G. Leung
Keyword(s):  
Neuromuscular Junction ◽  
Myasthenia Gravis ◽  
Nicotinic Acetylcholine Receptors ◽  
Acetylcholine Receptors ◽  
Autoimmune Disorder ◽  
Synaptic Proteins ◽  
Disease Treatment ◽  
Nicotinic Acetylcholine ◽  
System P ◽  
Presynaptic Calcium

Myasthenia gravis is in most cases an autoimmune disorder of the neuromuscular junction in which antibodies are directed at nicotinic acetylcholine receptors or other synaptic proteins, such as the MusK protein that is involved in the formation of the formation and maturation of the motor endplate. Less commonly, myasthenia gravis can result from antibodies directed to presynaptic calcium channels as a side effect of paraneoplastic antibodies (Lambert-Eaton syndrome) or from a developmental paucity of acetylcholine receptors in the neonatal form of the disease. Treatment is usually a combination of aceetylcoholinesterase inhibitors such as pyridostigmine to prolong the life of acetylcholine released at the neuromuscular junction and/or drugs such as corticosteroids aimed at reducing inflammation.

scholarly journals Long-term outcomes of acromegaly treated with fractionated stereotactic radiation: case series and literature review

2017 ◽  
Vol 4 (4) ◽  
pp. 255-262
Author(s):  
Ryan Rhome ◽  
Isabelle M Germano ◽  
Ren-Dih Sheu ◽  
Sheryl Green
Keyword(s):  
Systematic Review ◽  
Literature Review ◽  
Case Series ◽  
Tumor Control ◽  
Pituitary Neoplasms ◽  
Review Of The Literature ◽  
Biochemical Control ◽  
Low Incidence

Abstract Background Growth hormone (GH)-secreting pituitary adenomas represent an uncommon subset of pituitary neoplasms. Stereotactic radiosurgery (SRS) and fractionated stereotactic radiotherapy (FSRT) have been used as primary or adjuvant treatment. The purpose of this study is to report the long-term tumor control and toxicity from our institution and to perform a systematic literature review of acromegaly patients treated with FSRT. Methods We retrospectively reviewed all patients treated with FSRT (median dose 50.4 Gray [Gy], range 50.4–54 Gy) between 2005 and 2012 who had: 1) GH-secreting adenoma with persistently elevated insulin growth factor-1 (IGF-1) despite medical therapy and 2) clinical follow up >3 years after FSRT. Patients were treated with modern FSRT planning techniques. Biochemical control was defined as IGF-1 normalization. Systematic review of the literature was performed for FSRT in acromegaly. Results With a median follow-up of 80 months, radiographic control was achieved in all 11 patients and overall survival was 100%. Long-term biochemical control was achieved in 10 patients (90.9%) with either FSRT alone (36.4%) or FSRT with continued medical management (45.5%). No patient experienced new hypopituitarism, cranial nerve dysfunctions, or visual deficits. Our systematic review found published rates of biochemical control and hypopituitarism vary, with uniformly good radiographic control and low incidence of visual changes. Conclusions Adjuvant FSRT offered effective long-term biochemical control and radiographic control, and there was a lower rate of complications in this current series. Review of the literature shows variations in published rates of biochemical control after FSRT for acromegaly, but low incidence of serious toxicities.

Case Series Regarding Parastomal Variceal Bleeding: Presentation and Management

2018 ◽  
Vol 17 (5) ◽  
pp. 0-10
Author(s):  
John Romano ◽  
Charles V. Welden ◽  
Jordan Orr ◽  
Brendan McGuire ◽  
Mohamed Shoreibah
Keyword(s):  
Portal Hypertension ◽  
High Risk ◽  
Literature Review ◽  
Variceal Bleeding ◽  
Coil Embolization ◽  
Case Series ◽  
Treatment Modalities ◽  
Post Procedure ◽  
Lost To Follow Up

Parastomal variceal bleeding (PVB) is a serious complication occurring in up to 27% of patients with an ostomy and concurrent cirrhosis and portal hypertension. The management of PVB is difficult and there are no clear guidelines on this matter. TIPS, sclerotherapy, and /or coil embolization are all therapies that have been shown to successful manage PVB. We present a case series with five different patients who had a PVB at our institution. The aim of this case series is to report our experience on the management of this infrequently reported but serious condition. We also conducted a systemic literature review focusing on the treatment modalities of 163 patients with parastomal variceal bleeds. In our series, patient 1 had embolization and sclerotherapy without control of bleed and expired on the day of intervention due to hemorrhagic shock. Patient 2 had TIPS in conjunction with embolization and sclerotherapy and had no instance of rebleed 441 days after therapy. Patient 3 did not undergo any intervention due to high risk for morbidity and mortality, the bleed self-resolved and there was no further rebleed, this same patient died of sepsis 73 days later. Patient 4 had embolization and sclerotherapy and had no instance of rebleed 290 days after therapy. Patient 5 had TIPS procedure and was discharged five days post procedure without rebleed, patient has since been lost to follow-up.

scholarly journals Synovial fluid eosinophilia: a case series with a long follow-up and literature review

Rheumatology ◽  
2012 ◽  
Vol 52 (2) ◽  
pp. 346-351 ◽  
Author(s):  
C. Vazquez-Trinanes ◽  
B. Sopena ◽  
L. Gonzalez-Gonzalez ◽  
R. Diaz ◽  
A. Rivera ◽  
...  
Keyword(s):  
Synovial Fluid ◽  
Literature Review ◽  
Case Series

scholarly journals Optic Disc Neovascularization in Vogt-Koyanagi-Harada Disease- A Case Series and Literature Review

2021 ◽  
Author(s):  
Lagan Paul ◽  
Shalin Shah ◽  
Manisha Agarwal ◽  
Shalini Singh
Keyword(s):  
Optic Nerve ◽  
Literature Review ◽  
Case Series ◽  
Vascular Occlusion ◽  
Autoimmune Disorder ◽  
The Body ◽  
Low Grade ◽  
Retinal Vessels ◽  
History Of ◽  
Vkh Disease

Abstract BackgroundVogt–Koyanagi–Harada (VKH) disease is an autoimmune disorder which affects numerous organs of the body. We report two cases of Optic Nerve Head neovascularisation (NVD) in diagnosed cases of Vogt Koyanagi Harada(VKH) disease.FindingsCase 1: A 40 years female presented with acute loss of vision in both eyes for 10 days. She had multiple serous detachments in both eyes and diagnosed as acute VKH disease. After treatment, she was observed to have NVD later on OCT Angiography(OCTA). Case 2: A 52 years old man presented with history of sudden blackouts in front of left eye since 4 months. He was a case of chronic VKH and NVD was seen clinically and on OCTA. ConclusionThe exact etiopathogenesis of neovascularization in Harada’s disease is unclear. The low grade inflammations acts as a stimulus and can induce disc neovascularization even in the absence of vascular occlusion. ONH vessels appear to be more susceptible to developing NVD than retinal vessels in presence of chronic inflammation.

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