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 The Metabolic Stability of the Nicotinic Acetylcholine Receptor at the Neuromuscular Junction

Cells ◽  
2021 ◽  
Vol 10 (2) ◽  
pp. 358
Author(s):  
Isabel Martinez-Pena y Valenzuela ◽  
Mohammed Akaaboune
Keyword(s):  
Neuromuscular Junction ◽  
Nicotinic Acetylcholine Receptors ◽  
Acetylcholine Receptors ◽  
Turnover Rate ◽  
State Of The Art ◽  
Postsynaptic Membrane ◽  
Metabolic Stability ◽  
Synaptic Activity ◽  
Nicotinic Acetylcholine

The clustering and maintenance of nicotinic acetylcholine receptors (AChRs) at high density in the postsynaptic membrane is a hallmark of the mammalian neuromuscular junction (NMJ). The regulation of receptor density/turnover rate at synapses is one of the main thrusts of neurobiology because it plays an important role in synaptic development and synaptic plasticity. The state-of-the-art imaging revealed that AChRs are highly dynamic despite the overall structural stability of the NMJ over the lifetime of the animal. This review highlights the work on the metabolic stability of AChRs at developing and mature NMJs and discusses the role of synaptic activity and the regulatory signaling pathways involved in the dynamics of AChRs.

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 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]

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