scholarly journals The epidemiology of myasthenia gravis

2021 ◽  
Vol 14 (1) ◽  
pp. 7-16
Author(s):  
Ana-Maria Bubuioc ◽  
◽  
◽  
Aigerim Kudebayeva ◽  
Saule Turuspekova ◽  
...  
Keyword(s):  
Risk Factors ◽  
Autoimmune Disease ◽  
Myasthenia Gravis ◽  
Clinical Presentation ◽  
Epidemiological Data ◽  
Postsynaptic Membrane ◽  
Related Protein ◽  
The Past ◽  
Muscle Specific Kinase ◽  
Receptor Muscle

Neuromuscular junction (NMJ) disorders include several dysfunctions that ultimately lead to muscle weakness. Myasthenia gravis (MG) is the most prevalent NMJ disorder with a highly polymorphic clinical presentation and many different faces. Being an autoimmune disease, MG correlates with the presence of detectable antibodies directed against the acetylcholine receptor, muscle-specific kinase, lipoprotein-related protein 4, agrin, titin, and ryanodine in the postsynaptic membrane at the NMJ. MG has become a prototype serving to understand both autoimmunity and the function of the NMJ better. The aim of this review is to synthesize some of the epidemiological data available. Epidemiological data regarding MG are important for postulating hypotheses regarding its etiology and facilitating the description of MG subtypes. Thus, adequate documentation through broad databases is essential. The incidence and prevalence of MG reported around the globe have been rising steadily and consistently over the past decades. Ethnic aspects, gender-related differences, and environmental risk factors have been described, implying that these might contribute to a specific phenotype, further suggesting that MG may be considered an umbrella term that covers several clinical entities.

scholarly journals Recent advances in understanding and managing myasthenia gravis

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 1727 ◽  
Author(s):  
Allison Jordan ◽  
Miriam Freimer
Keyword(s):  
Myasthenia Gravis ◽  
Medical Management ◽  
Acetylcholine Receptors ◽  
Standard Of Care ◽  
Related Protein ◽  
The Past ◽  
Dramatic Decline ◽  
Trial Testing ◽  
Autoimmune Myasthenia ◽  
Muscle Specific Kinase

Autoimmune myasthenia gravis (MG) is a neuromuscular junction disorder marked clinically by fatigable muscle weakness and serologically by the presence of autoantibodies against acetylcholine receptors (AChRs), muscle-specific kinase (MuSK), or lipoprotein-related protein 4 (LPR4). Over the past few decades, the mortality of patients with MG has seen a dramatic decline secondary to evolving interventions in critical care and medical management. In the past 2 to 3 years, there have been several changes in standard of care for the treatment of MG. These changes include confirmation of the benefit of thymectomy versus medical management alone in AChR patients and a new US Food and Drug Administration-approved medication for refractory MG. There are also several exciting new prospective drugs in the pipeline, which are in different stages of clinical trial testing.

scholarly journals Plasma vitronectin is reduced in patients with myasthenia gravis: Diagnostic and pathophysiological potential

2019 ◽  
Vol 8 ◽  
pp. 184945441987591 ◽  
Author(s):  
Antonio Junior Lepedda ◽  
Giovanni Andrea Deiana ◽  
Omar Lobina ◽  
Gabriele Nieddu ◽  
Paola Baldinu ◽  
...  
Keyword(s):  
Immune Response ◽  
Extracellular Matrix ◽  
Myasthenia Gravis ◽  
Cell Attachment ◽  
Postsynaptic Membrane ◽  
Clinical Value ◽  
Disease Biomarkers ◽  
Skeletal Muscle Weakness ◽  
Plasma Fractions ◽  
Muscle Specific Kinase

Myasthenia gravis (MG) is an autoimmune disease leading to varying degrees of skeletal muscle weakness. It is caused by specific antibodies directed against definite components in the postsynaptic membrane at the neuromuscular junction (NMJ), such as the acetylcholine receptor (AChR) and the muscle-specific kinase (MUSK) receptor. In clinical practice, MG patients may be classified into three main subgroups based on the occurrence of serum autoantibodies directed against AChR or MUSK receptor or antibody-negative. As the MG subgroups differ in terms of clinical characteristics, disease pathogenesis, prognosis, and response to therapies, they could benefit from targeted treatment as well as the detection of other possible disease biomarkers. We performed proteomics on plasma fractions enriched in low-abundance proteins to identify potential biomarkers according to different autoimmune responses. By this approach, we evidenced a significant reduction of vitronectin in MG patients compared to healthy controls, irrespective of the autoantibodies NMJ target. The obtained results were validated by mono- and two-dimensional Western blotting analysis. Vitronectin is a multifunctional glycoprotein involved in the regulation of several pathophysiological processes, including complement-dependent immune response, coagulation, fibrinolysis, pericellular proteolysis, cell attachment, and spreading. The pathophysiological significance of the reduction of plasma vitronectin in MG patients has yet to be fully elucidated. It could be related either to a possible deposition of vitronectin at NMJ to counteract the complement-mediated muscle damage at this level or to a parallel variation of this glycoprotein in the muscle extracellular matrix with secondary induced alteration in clustering of AChRs at NMJ, as it occurs with variation in concentrations of agrin, another extracellular matrix component. The clinical value of measuring plasma vitronectin has yet to be defined. According to present findings, significantly lower plasma values of this glycoprotein might be indicative of an impaired complement-dependent immune response.

scholarly journals Functional monovalency amplifies the pathogenicity of anti-MuSK IgG4 in myasthenia gravis

2021 ◽  
Vol 118 (13) ◽  
pp. e2020635118
Author(s):  
Dana L. E. Vergoossen ◽  
Jaap J. Plomp ◽  
Christoph Gstöttner ◽  
Yvonne E. Fillié-Grijpma ◽  
Roy Augustinus ◽  
...  
Keyword(s):  
Stochastic Process ◽  
Autoimmune Disease ◽  
Myasthenia Gravis ◽  
Muscle Weakness ◽  
Binding Sites ◽  
Antiinflammatory Activity ◽  
Autoimmune Response ◽  
Cross Link ◽  
Muscle Specific Kinase ◽  
Opposing Effects

Human immunoglobulin (Ig) G4 usually displays antiinflammatory activity, and observations of IgG4 autoantibodies causing severe autoimmune disorders are therefore poorly understood. In blood, IgG4 naturally engages in a stochastic process termed “Fab-arm exchange” in which unrelated IgG4s exchange half-molecules continuously. The resulting IgG4 antibodies are composed of two different binding sites, thereby acquiring monovalent binding and inability to cross-link for each antigen recognized. Here, we demonstrate that this process amplifies autoantibody pathogenicity in a classic IgG4-mediated autoimmune disease: muscle-specific kinase (MuSK) myasthenia gravis. In mice, monovalent anti-MuSK IgG4s caused rapid and severe myasthenic muscle weakness, whereas the same antibodies in their parental bivalent form were less potent or did not induce a phenotype. Mechanistically this could be explained by opposing effects on MuSK signaling. Isotype switching to IgG4 in an autoimmune response thereby may be a critical step in the development of disease. Our study establishes functional monovalency as a pathogenic mechanism in IgG4-mediated autoimmune disease and potentially other disorders.

Muscle-Specific Kinase Autoimmune Myasthenia Gravis: Report of a Pediatric Case and Literature Review

2018 ◽  
Vol 50 (02) ◽  
pp. 116-121
Author(s):  
Hanene Benrhouma ◽  
Hedia Klaa ◽  
Rania Ben Aoun ◽  
Aida Rouissi ◽  
Melika Ben Ahmed ◽  
...  
Keyword(s):  
Literature Review ◽  
Tyrosine Kinase ◽  
Myasthenia Gravis ◽  
Clinical Presentation ◽  
Clinical Course ◽  
Rare Entity ◽  
Pediatric Case ◽  
Axial Muscles ◽  
Autoimmune Myasthenia ◽  
Muscle Specific Kinase

AbstractMyasthenia gravis (MG) with antibodies to the muscle-specific tyrosine kinase (MuSK-MG) receptor is a rare entity. It represents 5 to 8% of all MG patients. Few pediatric cases were reported. Clinical presentation is often atypical. It is characterized by predominant involvement of cranial, bulbar, and axial muscles and early respiratory crises. Myokymia and fasciculation are suggestive of MuSK-MG. The clinical course of patients with MuSK-MG is worse than other types of MG. Responses to standard therapies are variable. We report clinical, neurophysiological, serological, and outcome profile of a Tunisian child with MuSK-MG.

scholarly journals Can asthmatic subjects dive?

2016 ◽  
Vol 25 (140) ◽  
pp. 214-220 ◽  
Author(s):  
Yochai Adir ◽  
Alfred A. Bove
Keyword(s):  
Risk Factors ◽  
Decompression Sickness ◽  
Epidemiological Data ◽  
Common Disease ◽  
Pulmonary Barotrauma ◽  
The Past ◽  
Breathing Apparatus ◽  
Increased Risk ◽  
Recreational Diving ◽  
Theoretical Risk

Recreational diving with self-contained underwater breathing apparatus (scuba) has grown in popularity. Asthma is a common disease with a similar prevalence in divers as in the general population. Due to theoretical concern about an increased risk for pulmonary barotrauma and decompression sickness in asthmatic divers, in the past the approach to asthmatic diver candidates was very conservative, with scuba disallowed. However, experience in the field and data in the current literature do not support this dogmatic approach. In this review the theoretical risk factors of diving with asthma, the epidemiological data and the recommended approach to the asthmatic diver candidate will be described.

scholarly journals The role of gender in the prevalence of human leptospirosis in Albania

2018 ◽  
Vol 12 (03) ◽  
pp. 150-155 ◽  
Author(s):  
Edmond Puca ◽  
Pellumb Pipero ◽  
Arjan Harxhi ◽  
Erjona Abazaj ◽  
Arjet Gega ◽  
...  
Keyword(s):  
Risk Factors ◽  
Clinical Presentation ◽  
Age Groups ◽  
Clinical Manifestations ◽  
Epidemiological Data ◽  
Elisa Test ◽  
Human Leptospirosis ◽  
Males And Females ◽  
The Difference

Introduction: Leptospirosis is a zoonotic spirochetal disease with global importance, which continues to have a major impact on public health in developing countries. The prevalence of the disease is much higher in males. The objectives of this study were: to give some data and to share our experience with human leptospirosis in Albania; to describe the prevalence regarding to the role of gender in the prevalence of human leptospirosis; to make a gender specific analysis of the clinical manifestations in patients diagnosed and treated for leptospirosis in our service and to make a review of literature related to this hypothesis. Methodology: We reviewed the epidemiologic data, risk factors and differences in clinical presentation between males and females’ patients with leptospirosis. These data are analysed from hospitalized patients. Diagnosis of leptospirosis was established based on clinical presentation, epidemiological data and subsequently confirmed serologically by Anti-Leptospira IgM antibodies through ELISA test. Results: Between 2005-2016, 233 cases of confirmed leptospirosis were analysed. Males were 208 (89.27%) and 25 of patients (10.72%) were females in ratio 9:1 p < 0.001. The highest prevalence was observed in the 45-64 age groups. Overall mortality was found to be 8.58%, 19 were males and one female p < 0.001. Conclusions: There is a much higher prevalence of leptospirosis in middle aged men. Mortality rate seems to be similar in males and females. While the difference in prevalence may be related to exposure to risk factors, further investigation is necessary to study gender-based genetic and immunological predisposition.

scholarly journals Epidemiology of fall and its risk factors among elders in a rural area of Coimbatore, India

2017 ◽  
Vol 4 (10) ◽  
pp. 3864 ◽  
Author(s):  
Thomas V. Chacko ◽  
Prabha Thangaraj ◽  
Muhammad G. M.
Keyword(s):  
Risk Factors ◽  
Rural Area ◽  
Sampling Technique ◽  
Epidemiological Data ◽  
Cross Sectional Study ◽  
Community Based ◽  
Cross Sectional ◽  
The Past ◽  
Coimbatore District ◽  
History Of

Background: Global reports show that falls are the major cause of disability injuries among the elders. Developing countries lack the required epidemiological data on the burden of falls and factors that contribute to it.The objectives of the study were to find the burden of fall and its distribution in time, place and person among elders and to identify risk factors associated with fall among elders. Methods: A community–based cross-sectional study of 655 elders (≥ 60 years) was done in a rural area of Coimbatore District by two stage sampling technique. History of fall within the past 6 months was included in the study. Semi-structured pre-tested questionnaire was used to assess factors contributing to falls. The burden of falls was expressed as proportions and percentages. Logistic regression analysis was done to identify risk factors for fall. Results: 26% (95%CI: 22.59-29.32) elders experienced at least one fall. Of the total falls 50.2% occurred outside home and the remaining inside home. Most of the falls occurred during ambulation and at the morning hours (39.1%). Age ≥80 years (OR: 3.28, 95%CI: 1.28-5.98) and dizziness (OR: 3.27, 95%CI: 1.00-10.06) were only found to be significantly associated with fall on multivariate analysis. Conclusions: The occurrence of fall was found to be almost similar to that identified in western countries where fall preventive measures have been implemented. Similar efforts are yet to be initiated here and so this study provides the evidence for the need. Prospective studies through more frequent home visits are needed to confirm the enormity of the problem to suggest policy recommendation. 

scholarly journals Myasthenia Gravis and Associated Diseases

2018 ◽  
Vol 6 (3) ◽  
pp. 472-478 ◽  
Author(s):  
Nikolina Tanovska ◽  
Gabriela Novotni ◽  
Slobodanka Sazdova-Burneska ◽  
Igor Kuzmanovski ◽  
Bojan Boshkovski ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Myasthenia Gravis ◽  
Lupus Erythematosus ◽  
Neuromuscular Transmission ◽  
Respiratory Diseases ◽  
Postsynaptic Membrane ◽  
Initial Manifestation ◽  
Specific Antibodies ◽  
Associated Diseases ◽  
Common Basis

BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease caused by the action of specific antibodies to the postsynaptic membrane of the neuromuscular junction, leading to impaired neuromuscular transmission. Patients with MG have an increased incidence of other autoimmune diseases.AIM: to determine the presence of other associated diseases in patients with MG.METHOD: A group of 127 patients with MG followed in 10 years period, in which the presence of other associated diseases has been analysed.RESULTS: The sex ratio is in favour of the female sex, the average age of the initial manifestation of the disease is less than 50 years, 65.4% of the patients with MG have another disease. 15.0% patients have associated another autoimmune disease. Thyroid disease is the most common associated with MG, rarely rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and other autoimmune diseases. Other diseases include hypertension, heart disease, diabetes, respiratory diseases, dyslipidemia. 10.2% of the patients are diagnosed with extrathymic tumours of various origins.CONCLUSION: Associated diseases are common in patients with MG, drawing attention to the possible common basis for their coexistence, as well as their impact on the intensity and treatment of the disease.

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