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 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.

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 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.

scholarly journals Antibodies to striated muscle and acetylcholine receptors in evaluation of surgical treatment outcomes in patients with autoimmune myasthenia gravis

2017 ◽  
Vol 176 (3) ◽  
pp. 21-27
Author(s):  
M. G. Tovbina ◽  
V. G. Pishchik ◽  
S. V. Lapin ◽  
S. M. Nuraliev
Keyword(s):  
Surgical Treatment ◽  
Myasthenia Gravis ◽  
Treatment Outcomes ◽  
Antibody Titer ◽  
Acetylcholine Receptors ◽  
Striated Muscle ◽  
Striated Muscles ◽  
Course Material ◽  
Autoimmune Myasthenia

OBJECTIVE. The authors evaluated the role of antibodies to striated muscle and acetylcholine receptors in diagnostics of myasthenia gravis and thymoma, as well as outcomes of thymectomy and prognosis of myasthenia course. MATERIAL AND METHODS. The study investigated correlations of antibody content to striated muscles and acetylcholine receptors from the presence and size of thymoma, myasthenia in 157 patients with various pathologies of the thymus. The dynamics of antibody concentrations was followed up after thymectomy. RESULTS. Antibody titer to striated muscle depended on the presence and size of thymoma, severity of myasthenia and changed after thymectomy. Concentration of antibodies was associated with the presence of thymoma and it didn’t change after surgical treatment. Thymoma wasn’t revealed in patients who were seronegative to both antibodies. CONCLUSIONS. Seropositivity according to one of antibody could indicate the presence of thymoma, but its absence to both antibodies allowed doctors to eliminate this diagnosis. Antibodies to acetylcholine receptors are important markers of myasthenia. Monitoring of antibody titer dynamics to striated muscles after thymectomy could be useful for assessment of response to surgical treatment and prognosis of course of myasthenia.

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