scholarly journals The Epidemiologic, Clinical and Laboratory Findings of Patients with Myasthenia Gravis in Southern Iran

2012 ◽  
Vol 1 (1) ◽  
pp. 20-23
Author(s):  
Abbas Rahimi Jaberi ◽  
Ali Reza Manafi ◽  
Hossein Movahhedan ◽  
Marzieh Zeighami ◽  
Sara Honarparvaran
Keyword(s):  
Myasthenia Gravis ◽  
Nicotinic Acetylcholine Receptors ◽  
Acetylcholine Receptors ◽  
Care Center ◽  
Age At Onset ◽  
Autoimmune Disorder ◽  
Iranian Population ◽  
Laboratory Findings ◽  
Health Care Center ◽  
Geographical Regions

Introduction: Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular junction associated with presence of antibodies against nicotinic acetylcholine receptors (nAChRs). The pattern of the MG varies in different ethnical and geographical regions. Data regarding the pattern of the disease in Iran is scarce. Thus we performed this study in order to describe the epidemiologic, clinical and laboratory characteristics of MG in Iranian population. Method and Material: This was a retrospective study being performed in Nemazee Hospital, a tertiary health care center affiliated with Shiraz University of Medical Sciences, from 2001 to 2010. The medical records were reviewed and the data were entered into a computer database. Data are presented as mean ± SD and proportions as appropriate. Result: Overall we included 208 patients with MG among whom there were 62 (29.8%) men and 146 (70.2%) women. The mean age of the patients was found to be 33.19 ± 15.75 (range 1-85) years. The median age at onset was 28±2.3 years. Eight (3.9%) patients had family history of MG, 1.9% and 2.4% of patients had a background of Diabetes Mellitus and Rheumatoid disease in family, respectively. Thymoma was observed in 9.1% of patients. The presenting symptom was found to be ocular ones detected in 67 (34.9%) patients out of which 59 (30.7%) had bulbar and 12 (6.3%) had appendicular involvement. Edrophonium test was done for 28 patients out of whom 78.6% tested positive. The most common histopathology finding was thymic hyperplasia and the second most common was thymoma. Conclusion: This is the first study describing MG in an Iranian population. The pattern of disease was found to be much more similar to North America and Europe. MG was found to be more common in females consistent with western studies. [GMJ. 2012;1(1):20 -23]

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 Thymic B lymphocyte clones from patients with myasthenia gravis secrete monoclonal striational autoantibodies reacting with myosin, alpha actinin, or actin.

1986 ◽  
Vol 164 (4) ◽  
pp. 1043-1059 ◽  
Author(s):  
C L Williams ◽  
V A Lennon
Keyword(s):  
Skeletal Muscle ◽  
Epithelial Cells ◽  
Myasthenia Gravis ◽  
Nicotinic Acetylcholine Receptors ◽  
Acetylcholine Receptors ◽  
B Lymphocyte ◽  
High Yield ◽  
Thymic Epithelial Cells ◽  
Anchoring Proteins ◽  
Mucosal Cells

Striational autoantibodies (StrAb), which react with elements of skeletal muscle cross-striations, occur frequently in patients with thymoma associated with myasthenia gravis (MG). Dissociated thymic lymphocytes from 22 of 72 MG patients secreted StrAb when cultured with PWM. A high yield of EBV-transformed B cell lines was established from thymus, thymoma, and peripheral blood of seven patients with MG, but clones secreting StrAb arose only from the three patients who had StrAb in their sera. The monoclonal StrAb bound to A bands or I bands in skeletal muscle of human, rat, and frog. One bound to mitochondria in addition to myofibrillar I bands. None bound to nuclei, smooth muscle, or gastric mucosal cells. In immunoblot analyses and ELISAs the monoclonal StrAb bound to muscle and nonmuscle isotypes of myosin, alpha actinin, and/or actin. All bound to contractile proteins common to thymus and muscle, and one selectively immunostained epithelial cells of the thymic medulla. From these antigenic specificities we suggest that StrAb might arise as an immune response directed against the cytoskeletal anchoring proteins associated with nicotinic acetylcholine receptors in thymic epithelial cells undergoing neoplastic transformation to thymoma.

scholarly journals Specific features of anesthesia in patients with myasthenia gravis

2016 ◽  
Vol 69 (9-10) ◽  
pp. 305-311
Author(s):  
Ivana Spasojevic ◽  
Danica Hajdukovic ◽  
Milena Komarcevic ◽  
Stanislava Petrovic ◽  
Jelena Jovanovic ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Conservative Treatment ◽  
Myasthenia Gravis ◽  
Nicotinic Acetylcholine Receptors ◽  
Acetylcholine Receptors ◽  
Surgical Interventions ◽  
Different Types ◽  
Increased Sensitivity ◽  
Tensilon Test

Introduction. Myasthenia gravis is an autoimmune disease caused by antibodies leading to the destruction of nicotinic acetylcholine receptors on the neuromuscular junction. It is characterized by muscle weakness that gets aggravated with physical activity and improves at rest. Myasthenia Gravis Foundation of America made the clinical classification of Myasthenia gravis which is still in use today. ?Tensilon test? is still the gold standard for the diagnosis of Myasthenia gravis. In addition to this test repeated muscular stimulation can be used as well as the analysis of specific autoantibodies. Treatment of Myasthenia Gravis. In conservative treatment of Mysthenia gravis anticholinesterases, immunosuppressants and plasmapheresis can be used. If conservative treatment does not lead to the desired remission, surgical treatment is indicated. The most accepted indication for thymectomy is the presence of thymoma with generalized form of Myasthenia gravis in adults. How to Distinguish Myasthenic From Cholinergic Crisis. The following is important to make a difference between these two crises: knowledge of the events that preceded the crisis, the size of pupils as well as the presence of muscarinic signs and tensilon test. Specific Features of Anesthesia in Patients with Myasthenia Gravis. Mechanism of the disease development is the reason for the increased sensitivity or resistance of these patients to certain types of drugs used in anesthesia. Protocol of Perioperative Anesthesia in Patients with Myasthenia Gravis. Based on 35 years of experience in the surgical treatment of patients with Myasthenia gravis anesthesiologists at the Department of Thoracic Surgery, Institute for Pulmonary Diseases of Vojvodina, made the protocol of anesthesia and perioperative treatment for these patients. Conclusion. Anesthesiologists may have to deal with a patient with myasthenia gravis in different types of surgical interventions. The protocol for anesthesia and perioperative management of these patients herewith presented may greatly help them in their clinical practice.

scholarly journals Analysis of Social Determinants and the Utilization of Tele-Urgent Pediatrics Care During a Pandemic (Preprint)

2020 ◽  
Author(s):  
Saif Khairat ◽  
Phillip McDaniel ◽  
Matthew Jensen ◽  
Tia Francis ◽  
Barbara Edson ◽  
...  
Keyword(s):  
Vulnerable Populations ◽  
Care Center ◽  
Urgent Care ◽  
Pediatric Care ◽  
High Concentration ◽  
Cross Sectional ◽  
Low Concentration ◽  
Health Care Center ◽  
Geographical Regions ◽  
Before And After

BACKGROUND Telehealth is increasingly used to provide specialty consultations to infants and children receiving care. However, there is uncertainty if the Covid-19 pandemic has influenced the use of telehealth among vulnerable populations. OBJECTIVE The objective of this research was to compare the overall use of tele-urgent care visits for pediatric before and after the pandemic especially among vulnerable populations. METHODS We conducted a cross-sectional analysis of Pediatric tele-urgent care visits at a Virtual Care Center at a Southeastern Health Care Center. The main outcome of this study was the utilization of Pediatrics tele-urgent visits across geographical regions with different levels of social disparities and between 2019 and 2020. RESULTS Of 584 tele-urgent care visits, 388 (66.4%) visits occurred in 2020 during the pandemic compared to 196 (33.6%) visits in 2019. Among 808 NC zip codes, 181 (22%) consisted of a high concentration of vulnerable populations, where 17.7% of the tele-urgent care visits originated from. The majority (67.8%) of tele-urgent care visits originated from zip codes with a low concentration of vulnerable populations. There was a significant association between the rate of Covid19 cases and the concentration level of social factors in a given ZCTA area. CONCLUSIONS The use of tele-urgent visits for pediatric care doubled during the Covid19 pandemic. The majority of the tele-urgent care visits after Covid19 originated from regions where there is a low presence of vulnerable populations. In addition, our geospatial analysis found that geographic regions with a high concentration of vulnerable populations had a significantly higher rate of Covid19 confirmed cases and deaths compared to regions with a low concentration of vulnerable populations.

Pediatric Miller Fisher Syndrome and Ocular Myasthenia Gravis (A Reminder of Clinical Mimicry): A Case Report

2021 ◽  
Author(s):  
Zakaria Barsoum
Keyword(s):  
Myasthenia Gravis ◽  
Acetylcholine Receptors ◽  
Correct Diagnosis ◽  
Clinical Signs ◽  
Autoimmune Disorder ◽  
Miller Fisher Syndrome ◽  
Neurological Deficits ◽  
Fisher Syndrome ◽  
Ocular Myasthenia ◽  
Ocular Myasthenia Gravis

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.

scholarly journals Clinical Characteristic Myasthenia Gravis among Indonesians

2020 ◽  
Vol 2 (2) ◽  
pp. 257-263
Author(s):  
Syahrul Syahrul ◽  
Endang Mutiawati ◽  
Nur Astini ◽  
Nurul Fajri ◽  
Suherman Suherman
Keyword(s):  
Myasthenia Gravis ◽  
Epidemiological Data ◽  
Autoimmune Disorder ◽  
Myasthenic Crisis ◽  
Thyroid Disorder ◽  
Design Data ◽  
Laboratory Findings ◽  
Cross Sectional ◽  
Ocular Myasthenia ◽  
Wide Range

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.

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.

scholarly journals Role of complement in the pathogenesis of experimental autoimmune myasthenia gravis.

1978 ◽  
Vol 147 (4) ◽  
pp. 973-983 ◽  
Author(s):  
V A Lennon ◽  
M E Seybold ◽  
J M Lindstrom ◽  
C Cochrane ◽  
R Ulevitch
Keyword(s):  
Myasthenia Gravis ◽  
Nicotinic Acetylcholine Receptors ◽  
Acetylcholine Receptors ◽  
Neuromuscular Transmission ◽  
Passive Transfer ◽  
Experimental Autoimmune Myasthenia Gravis ◽  
Autoimmune Myasthenia ◽  
Experimental Autoimmune

An acute phase of experimental autoimmune myasthenia gravis (EAMG) occurs transiently early in the immune response of Lewis rats to nicotinic acetylcholine receptors (AChR) when Bordetella pertussis is used as adjuvant. It is characterized by a destructive cellular attack directed at the postsynaptic membranes of muscle. Acute EAMG can be passively transferred to normal rats by IgG from serum of rats with chronic EAMG. In the present study, acute EAMG, induced either by passive transfer of syngeneic antibodies or by active immmunization, was inhibited in rats depleted of complement by treatment with cobra venom factor (CoF). Furthermore, passive transfer of antibodies in excess of the muscle's content of AChR was without any measurable effect in rats treated with CoF. Although 60% of the muscle's AChR was complexed with antibody, there was no reduction in the muscle's content of AChR, and neuromuscular transmission was not compromised as judged electromyographically by curare sensitivity. These data imply that redistribution, accelerated degradation, and impairment of the ionophore function of AChR, effects of antibodies described in vitro on extrajunctional AChR, do not play a significant role in vivo in impairing neuromuscular transmission in an intact neuromuscular junction. Complement appears to be a critical mediator of anti-AChR antibodies' pathogenicity in vivo.

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