scholarly journals Clinical Characteristic Myasthenia Gravis among Indonesians

Author(s):  
Syahrul Syahrul ◽  
Endang Mutiawati ◽  
Nur Astini ◽  
Nurul Fajri ◽  
Suherman Suherman

Myasthenia gravis (MG) is a relatively rare autoimmune disorder on peripheral nerves where an antibody of nicotinic acetylcholine postsynaptic receptor is formed on neuromuscular junction (NMJ). MG is clinically characterized with a fluctuating muscle weakness. The incidence rate is 0.25 to 2.0 per 1,000,000 among population. 10% of the population is children and adolescents.  Over the past forty years, a mortality rate has improved in the myasthenic crisis from 75% to less than 5%. Moreover, in Indonesia, this is still considered a rare disease. This study applied a retrospective cross-sectional study design. Data were gathered from patients diagnosed and treated for myasthenia gravis admitted in the neurological emergencies in the hospital during January to November 2019. Afterwards, the follow-up clinical information was also being analyzed. 12 cases of myasthenia gravis were collected with females predominated (75%), and one male (25%) with the average age of 30-40 years. The majority (75%) had the onset of < 35 years. The patients were from various districts, with 75% referral from the district and 58% housewives. Clinically, 8% patients had the ocular myasthenia and 92% was the generalized one. The ocular myasthenia had a relatively earlier onset. As MG symptomatology bears a wide range of variability and severity, it was graded based on Osserman’s and Myasthenia Gravis Foundation of America’s (MGFA) classification. Out of all patients, 13.7% belonged to Osserman’s class 1-3 (33%) and class 4-5 (67%). Myasthenia Gravis Foundation of America (MGFA) grading calibrated the severity of each crisis. The findings were 17% crisis of milder degree and 83% of moderate to severe nature. The thyroid disorder was evident in one of the patients (8.3%). Laboratory findings were 75% patients with the increase in leukocyte count. The treatment was only 25% anticholinesterase, 50% anticholinesterase and steroid, in addition to 25% anticholinesterase and therapeutic plasma exchange (TPE). The hospital stay was (the average in days) 83% with >7 days. The myasthenia gravis reported was 12 cases. Patients’ outcome depended on the clinical condition when they first arrived in the hospital. These findings will be useful for the resource allocation and planning in health services. Many regions worldwide have few or no epidemiological data on the myasthenia gravis, and more studies are required to yield more estimates that are accurate.

2021 ◽  
Vol 1 (12) ◽  
pp. 1002-1010
Author(s):  
Dicki Apriansyah Haris Putra ◽  
Lale Sirin Rifdah S ◽  
Putu Mega Asri D ◽  
Muhammad Mahfuzzahroni

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.


Author(s):  
Tiziana Ciarambino ◽  
filomena pietrantonio ◽  
Sara Rotunno ◽  
Alessandra Fiorentini ◽  
Rosalba Cipriani ◽  
...  

Background: from December 2019 and the spreading of syndemics, a lot of medical centers reg-istered data about their patients. In Italy, the most relevant quantity of patients was hospitalized in internal medicine wards. Methods: In this observational, retrospective cross-sectional study, all data of the COVID-19 patients, admitted Latio hospitals, from March 01 to December 31, 2020, were collected and their Epidemiological data, demographics, signs and symptoms on admission, comorbidities, laboratory findings, chest radiography and CT findings, treatment received and mortality rate were analyzed by gender to find any differences of gravity of disease. Clinician details were registered on database (one for every hospital). Cost analysis was performed by length of stay and antiviral drugs use, using point of view of Italian Healthcare System. Results: 2256 patients with mean age of 71.01 ± 28.02 years were included. For men, frequency of hyper-tension, COPD, use of oxygen therapy, Tocilizumab were significantly higher and epidemiolog-ical link was related to rehabilitation ward and community. The gender difference about hospi-talization was one day more for man. No strong significant difference by gender in the death rate was observed. Considering antiviral drugs and hospitalization, a man costs €1000 more than woman. Conclusions: In male patients, hypertension and COPD were observed more frequently and the epidemiological link was related to rehabilitation ward and community. In female sub-jects, the epidemiological link was related to Hospital and we observed significantly higher atypical chest-X ray. Tocilizumab, oxygen therapy and antiviral drugs were prescribed more in male subjects. No differences by gender we report in other treatments and outcomes. Future studies should be analyzed to get a more comprehensive understanding of COVID-19 by gender.


2021 ◽  
Author(s):  
Cleonisio Leite Rodrigues ◽  
Hermany Capistrano Freitas ◽  
Paulo Reges Oliveira Lima ◽  
Pedro Helder de Oliveira Junior ◽  
José Marcelino Aragão Fernandes ◽  
...  

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.


2020 ◽  
Vol 5 (7) ◽  
pp. 190-193
Author(s):  
Dr. Usha BR. ◽  
◽  
Dr. Nandhini K ◽  
Dr. Chaitra MC ◽  
◽  
...  

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.


Author(s):  
Zakaria Barsoum

AbstractMiller Fisher syndrome (MFS) is a rare immune-mediated neuropathy that often presents with diplopia and bilateral external ophthalmoplegia. Other neurological deficits may occur such as ataxia and areflexia but not in all cases. Although MFS is a clinical diagnosis, serological confirmation is possible by identifying the anti-GQ1b antibody found in the majority of patients. Myasthenia gravis is an autoimmune disorder of the availability of acetylcholine receptors in the neuromuscular junction. Ocular myasthenia gravis is a disease subtype characterized by variable patterns of weakness of extraocular muscles, eyelid elevator, and orbicular muscle in which the initial sign in most adults and children is ptosis. We report a child with MFS who presented with clinical signs suggestive of ocular myasthenia gravis, but in whom the correct diagnosis was made on the basis of serological testing for the anti-GQ1b antibody. We aim to highlight the similarity between the two rare conditions and address the importance of early liaison with neurologists and ophthalmologists in reaching to the proper diagnosis.


2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Stacey Ho ◽  
Antonio Liu

We report on a co-occurrence case of ocular myasthenia gravis with exacerbation leading to myasthenic crisis in addition to pharyngeal-cervical-brachial variant of Guillain–Barré syndrome in a patient with severe oropharyngeal dysphagia and acute respiratory failure.


2002 ◽  
Vol 47 (6) ◽  
pp. 132-135 ◽  
Author(s):  
M. E. Farrugia

Myasthenia gravis is an acquired disorder of the neuromuscular junction characterised by fatiguable weakness of the limbs, bulbar and facial muscles and may be complicated by respiratory muscle weakness and failure. One often confirms the diagnosis by a simple serological test looking for the presence of the nicotinic acetylcholine receptor antibody. However, seronegative myasthenia constitutes about 20% of cases and in the case of ocular myasthenia, only 50% will have the antibody. Therefore, the diagnosis can be less than straightforward especially if the patient presents with vague symptoms such as fatigue or presents to specialities other than neurology or ophthalmology. The fact that the diagnosis may prove to be challenging, compounded by the fact that the condition is relatively rare and that the antibody to the acetylcholine receptor is not always present, epidemiological data is often less than precise and indeed difficult to acquire. We felt it was necessary to try to establish the epidemiological data on seropositive myasthenia gravis in Tayside, (this has never been carried out) bearing in mind the above pitfalls, and see how the incidence compares with similar and previous studies.


2021 ◽  
Vol 55 (1) ◽  
pp. 62-69
Author(s):  
Ewa Sobieszczuk ◽  
Łukasz Napiórkowski ◽  
Piotr Szczudlik ◽  
Anna Kostera-Pruszczyk

Introduction: Myasthenia gravis (MG) is a rare autoimmune disorder of the neuromuscular junction. MG epidemiology has not been studied in Poland in a nationwide study before. Methods: Our epidemiological data were drawn from the National Health Fund (Narodowy Fundusz Zdrowia, NFZ) database; an MG patient was defined as a person who received at least once medical service coded in ICD-10 as MG (G70) and at least 2 reimbursed prescriptions for pyridostigmine bromide (Mestinon®) or ambenonium chloride (Mytelase®) in 2 consecutive years. Results: On 1st of January 2019, 8,702 patients with MG were receiving symptomatic treatment (female:male ratio: 1.65:1). MG incidence was 2.36/100,000. The mean age of incident cases in 2018 was 61.37 years, 59.17 years for women and 64.12 years for men. Incidence of early-onset MG (<50 years) was 0.80/100,000 and 4.98/100,000 for late-onset MG (LOMG), with male predominance in LOMG. Prevalence was 22.65/100,000. In women, there was a constant increase in prevalence of symptomatic MG from the first decade of life up to 80–89 years. In men, an increase in prevalence appeared in the 6th decade. The highest prevalence was observed in the age group of 80–89 years: 59.65/100,000 in women and 96.25/100,000 in men. Conclusions: Our findings provide information on epidemiology of MG in Poland and can serve as a tool to evaluate healthcare resources needed for MG patients.


Pulse ◽  
1970 ◽  
Vol 3 (1) ◽  
pp. 25-26
Author(s):  
MI Hussain ◽  
L Aziz

Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction. The disease is characterized by exacerbation and remission of the symptoms from time to time. Myasthenic crisis is a serious complication of MG and is defined as weakness from acquired MG that is severe enough to require intubations [1]. Myasthenic crisis may complicate in 15-20% of patient with MG [2], [3]. Most patients require ventilation for a brief period, usually less than two weeks [4]. Repeated intubations may cause tracheal stenosis. The potential risk factors for post-intubation subglottic stenosis include, the underlying disease requiring endotracheal intubations(EI), the age and body weight, the duration and number of EIs, absence of sedation and the occurance of infections, hypotensive or hypoxic events during the period of EI and traumatic EI [5], [6].Key words: Tracheal stenosis, Repeated intubations, Myasthenia gravis.DOI: 10.3329/pulse.v3i1.6550Pulse Vol.3(1) July 2009 p25-26


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