scholarly journals Anaesthetic Management of a Patient with Myasthenia Gravis Posted for Tonsillectomy- A Case Report

2021 ◽  
Author(s):  
Dinesh K Chauhan ◽  
Vibhakar Vasudeva ◽  
Pooja Shah ◽  
Priya Kishnani
Keyword(s):  
Neuromuscular Junction ◽  
Myasthenia Gravis ◽  
Autoimmune Disorder ◽  
Anaesthetic Management ◽  
Mortality And Morbidity ◽  
Risk Of Mortality ◽  
Increased Risk ◽  
Skeletal Muscle Weakness ◽  
Congenital Myasthenia ◽  
Chronic Autoimmune Disease

Myasthenia gravis is a chronic autoimmune disease of neuromuscular junction which causes skeletal muscle weakness and fatigability, characterised by decrement in postsynaptic acetylcholine receptor at neuromuscular junction caused by auto-antibodies destruction. There are three types of myasthenia gravis in children- transient neonatal, congenital and juvenile. Juvenile Myasthenia Gravis (JMG) is an autoimmune disorder that can affect the all skeletal muscles including extraocular muscles, which may lead to fatigability and generalised weakness. This report is about a case of seven-year-old patient weighing 25 kg presented with throat pain since one and half month. Patient was a known case of congenital myasthenia gravis and diagnosis was confirmed by Edrophonium test. He underwent tonsillectomy and was successfully managed under general anaesthesia with no muscle relaxant technique. Tonsillectomy in paediatric patients is not an uncommon surgical procedure. In spite of being a common surgery, it is still challenging to the surgeon as well as the anaesthesiologist as there is a shared airway between the two. Also, in the postoperative period there are chances of post-tonsillectomy bleeding causing airway obstruction, if not diagnosed and treated early could risk the life of the patient. Therefore, this surgery poses an increased risk of mortality and morbidity.

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 Muscle-Specific Tyrosine Kinase-Associated Myasthenia Gravis: A Neuromuscular Surprise

2021 ◽  
Vol 2021 ◽  
pp. 1-3
Author(s):  
Hassam Ali ◽  
Rahul Pamarthy ◽  
Nayab Ahsan ◽  
WashmaAwan ◽  
Shiza Sarfraz
Keyword(s):  
Neuromuscular Junction ◽  
Tyrosine Kinase ◽  
Myasthenia Gravis ◽  
Muscle Weakness ◽  
Acetylcholine Receptors ◽  
Muscular Contraction ◽  
Skeletal Muscle Weakness ◽  
Hypoxic Respiratory Failure ◽  
Receptor Antibodies

Myasthenia gravis is a neuromuscular autoimmune disease that results in skeletal muscle weakness that worsens after periods of activity and improves after rest. Myasthenia gravis means “grave (serious), muscle weakness.” Although not completely curable, it can be managed well with a relatively high quality of life and expectancy. In myasthenia gravis, antibodies against the acetylcholine receptors at the neuromuscular junction interfere with regular muscular contraction. Although most commonly caused by antibodies to the acetylcholine receptor, antibodies against MuSK (muscle-specific kinase) protein can also weaken transmission at the neuromuscular junction. Muscle-specific tyrosine kinase myasthenia gravis (MuSK-Ab MG) is a rare subtype of myasthenia gravis with distinct pathogenesis and unique clinical features. Diagnosis can be challenging due to its atypical presentation as compared to seropositive myasthenia gravis. It responds inconsistently to steroids, but plasma exchange and immunosuppressive therapies have shown promising results. We report a case of a 49-year-old female who presented with acute hypoxic respiratory failure. Our patient experienced progressive, undiagnosed MuSK-Ab MG for years without a diagnosis.

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 Particulate Matter as a Driven Factor Covid19 Transmission at Outdoor: Review

2020 ◽  
Vol 12 (3) ◽  
pp. 225
Author(s):  
Hanien Firmansyah ◽  
Azmi Nur Fadlillah ◽  
Aditya Sukma Pawitra
Keyword(s):  
Particulate Matter ◽  
Physiological Condition ◽  
The Body ◽  
Mortality And Morbidity ◽  
Virus Rna ◽  
Risk Of Mortality ◽  
Increased Risk ◽  
Mucous Membranes ◽  
The Face ◽  
Droplet Transmission

Introduction: Coronavirus Disease 2019 (Covid19) is an infectious disease caused by SARS-CoV-2 (Severe Acute Respiratory Syndrome-related Coronavirus) which attacks the respiratory tract, with mild to severe symptoms. The virus can infect the body through mucous membranes on the face with droplet transmission. Air pollution is thought to contribute to Covid19 events which can worsen the situation of people with Covid19. The aims of this literature review is to analyze Particulate Matter (PM) as environmental factors that contributes Covid19, so it is expected to be a study in terms of prevention and prevention in the field of environmental health. Discussion: PM is thought to have contributed to an increased risk of mortality and morbidity in Covid19 events. PM which has toxic properties can enter the lungs and affect the physiological condition of the lung organs. The findings regarding the presence of SARS-CoV-2 virus RNA strengthen the suspicion that PM plays a role in Covid19 transmission. Several studies have found that there is a relationship between PM and Covid19. PM2.5 which is smaller than PM10 has a higher ability to be a risk factor for ballast in Covid19. Conclusion: The results of this synthesis state that PM can be one of the driving factors of Covid19 transmission in air.

Rituximab in AChR subtype of myasthenia gravis: systematic review

2020 ◽  
Vol 91 (4) ◽  
pp. 392-395 ◽  
Author(s):  
Vincenzo Di Stefano ◽  
Antonino Lupica ◽  
Marianna Gabriella Rispoli ◽  
Antonio Di Muzio ◽  
Filippo Brighina ◽  
...  
Keyword(s):  
Systematic Review ◽  
Neuromuscular Junction ◽  
Myasthenia Gravis ◽  
Acetylcholine Receptor ◽  
Autoimmune Disorder ◽  
Postsynaptic Membrane ◽  
Receptor Subtype ◽  
Registration Number ◽  
Randomised Controlled ◽  
Time To Relapse

Myasthenia gravis (MG) is a chronic autoimmune disorder of the neuromuscular junction characterised by an autoantibody against acetylcholine receptor (AChR-Ab), autoantibody against muscle-specific kinase (MuSK-Ab), lipoprotein-related protein 4 or agrin in the postsynaptic membrane at the neuromuscular junction. Many patients are resistant to conventional treatment and effective therapies are needed. Rituximab (RTX) is a monoclonal antibody directed against CD20 antigen on B cells which has been successfully employed in anti-MuSK-Ab+MG, but the efficacy in anti-AChR-Ab+MG is still debated. The purpose of this systematic review was to describe the best evidence for RTX in the acetylcholine receptor subtype. The authors undertook a literature search during the period of 1999–2019 according to the Preferred Reporting Items for Systematic Reviews and Meta-Analys methodology, employing (myasthenia)+(gravis)+(RTX) as search terms. The analysis was confined to studies that include at least five patients with confirmed anti-AChR-Ab+MG. Thirteen studies have been selected, showing a good safety. The data obtained were heterogeneous in terms of posology, administration scheme and patients’ evaluation, ranging from a minimum of two to a maximum of three cycles. RTX led to a sustained clinical improvement with prolonged time to relapse, in parallel to a reduction or discontinuation of other immunosuppressive therapies. Treatment with RTX appears to work in some but not all patients with anti-AChR-Ab+MG, but randomised controlled trials are needed. Future studies should take into account the subtype of MG and employ reliable measures of outcome and severity focusing on how to identify patients who may benefit from the treatment. Trial registration number: NCT02110706.

scholarly journals Anaesthetic Considerations in Paediatric Myasthenia Gravis

2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
Oliver William Masters ◽  
Oliver N. Bagshaw
Keyword(s):  
Mechanical Ventilation ◽  
Neuromuscular Junction ◽  
Myasthenia Gravis ◽  
Treatment Options ◽  
Anaesthetic Management ◽  
Management Options ◽  
The Face ◽  
Muscle Groups ◽  
The Relationship

Myasthenia gravis is of particular interest to anaesthetists because of the muscle groups affected, the pharmacology of the neuromuscular junction, and interaction of both the disease and treatment with many anaesthetic drugs. Anaesthetists may encounter children with myasthenia either to facilitate treatment options or to institute mechanical ventilation in the face of a crisis. This paper reviews the literature pertaining to the pathophysiology and applied pharmacology of the disease and explores the relationship between these and the anaesthetic management. In addition to illustrating the tried-and-tested techniques, some newer management options are explored.

scholarly journals Simultaneous Presence of Acetylcholine Receptor and Muscle Specific Tyrosine Kinase Antibodies in Myasthenia Gravis

2019 ◽  
Vol 4 (4) ◽  
pp. 128-130
Author(s):  
Maryam Poursadeghfard ◽  
Sara Azhdari
Keyword(s):  
Neuromuscular Junction ◽  
Tyrosine Kinase ◽  
Myasthenia Gravis ◽  
Acetylcholine Receptor ◽  
Receptor Tyrosine Kinase ◽  
Good Condition ◽  
Autoimmune Disorder ◽  
Simultaneous Presence ◽  
Specific Receptor ◽  
Progressive Course

Myasthenia gravis (MG) is known as an autoimmune disorder which affects transmission in neuromuscular junction. The serologic tests used for diagnosis include acetylcholine receptor and muscle specific receptor tyrosine kinase antibodies. Studies often have reported that patients with formal antibody are negative for the latter one. However, very limited studies have reported positive anti-muscle specific receptor tyrosine kinase antibody in a small percentage of patients with acetylcholine receptor antibody. Here, we reported a young woman who was diagnosed with MG and had a rapid and progressive course of the disease. She was seropositive for both acetylcholine receptor and muscle-specific receptor tyrosine kinase antibodies simultaneously. However, she discharged from the hospital with good condition after treatment.

scholarly journals Myasthenia Gravis – A Review of Current Therapeutic Options

2018 ◽  
Vol 13 (2) ◽  
pp. 86
Author(s):  
Saiju Jacob ◽  
Keyword(s):  
Neuromuscular Junction ◽  
Myasthenia Gravis ◽  
Treatment Options ◽  
Standard Of Care ◽  
Autoimmune Disorder ◽  
Therapeutic Options ◽  
Current Standard ◽  
Autoimmune Attack ◽  
Key Factor ◽  
Randomised Controlled

Myasthenia gravis (MG) is an autoimmune disorder that leads to skeletal muscle weakness and fatigue. The autoimmune attack is caused by autoantibodies against the acetylcholine postsynaptic receptors at the neuromuscular junction of skeletal muscles. However, other antigenic targets that are components of the neuromuscular junction have also been implicated in the pathogenesis of MG. The current standard of care is immunosuppressive therapy; however, many existing therapeutic options have not been validated for use in MG in large randomised controlled trials. Furthermore, around 10% of patients with generalised MG are refractory to treatment. The complement system is involved in numerous inflammatory, neurodegenerative and autoimmune diseases, and is a key factor in the pathogenesis of acetylcholine receptor antibody-related MG. Targeting complement and other components involved in the underlying pathogenesis of the disease may provide useful treatment options, particularly for refractory patients.

Myasthenia Gravis

2013 ◽  
Author(s):  
Aaron E. Miller ◽  
Teresa M. DeAngelis
Keyword(s):  
Surgical Treatment ◽  
Neuromuscular Junction ◽  
Myasthenia Gravis ◽  
Acetylcholine Receptors ◽  
Diagnostic Testing ◽  
Autoimmune Disorder ◽  
Postsynaptic Membrane ◽  
Treatment Recommendations ◽  
Clinical Findings

Myasthenia gravis (MG) is an autoimmune disorder that results in loss of functional acetylcholine receptors (AChR) on the postsynaptic membrane of the neuromuscular junction caused by the presence of antibodies to the AChR. In this chapter, we review the cardinal clinical findings of MG, the standard diagnostic testing including electrophysiological features, and the medical and surgical treatment recommendations.

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