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Clinical neurophysiology of neuromuscular junction disease
2019The neuromuscular junction (NMJ) is a cholinergic synapse where quantal release of acetylcholine (ACh) from motor nerve terminals generates a local endplate potential (EPP) on the muscle fiber. EPPs that reach threshold depolarize the entire muscle fiber and initiate the process of excitation-contraction coupling. Deficits of neuromuscular transmission
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Immune-Mediated Disease of the Neuromuscular Junction
2019The chapter is concerned with disorders that affect the neuromuscular junction (NMJ). Myasthenia gravis (MG) is a prototypic antibody-mediated disease affecting the neuromuscular junction. The chapter looks at the epidemiology of MG. It also considers clinical manifestations and presentations, classification and staging, and diagnosis. The chapter also
Robert P. Lisak, James Selwa
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2017 Electric Electronics, Computer Science, Biomedical Engineerings' Meeting (EBBT), 2017
In this study, the effect of spectrograms from neuromuscular junction and tendon records for normal, neurogenic and myopathic motor units being constructed via EMG Simulator v3.6 on the differential diagnosis were investigated. Multi-layer perceptron is chosen as classifier.
Artuğ, Tuğrul +3 more
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In this study, the effect of spectrograms from neuromuscular junction and tendon records for normal, neurogenic and myopathic motor units being constructed via EMG Simulator v3.6 on the differential diagnosis were investigated. Multi-layer perceptron is chosen as classifier.
Artuğ, Tuğrul +3 more
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Sleuthing molecular targets for neurological diseases at the neuromuscular junction
Nature Reviews Neuroscience, 2003The analysis of congenital myasthenic syndromes (CMSs) has disclosed a diverse array of molecular targets at the motor endplate and has delineated their contribution to synaptic function. Clinical, electrophysiological and morphological studies have paved the way for detecting CMS-related mutations in proteins such as choline acetyltransferase ...
Andrew G, Engel +2 more
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Acta Neuropathologica, 1983
The syndrome of arthrogryposis and palatoschisis (SAP), an inherited syndrome of muscular hypotonia in Charolais cattle, was used as an experimental model to study neuromuscular differentiation. The ultrastructural development of muscle, peripheral nerve, and neuromuscular junctions was studied to determine the sequence of events preceding hypotrophic ...
R G, Russell, F T, Oteruelo
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The syndrome of arthrogryposis and palatoschisis (SAP), an inherited syndrome of muscular hypotonia in Charolais cattle, was used as an experimental model to study neuromuscular differentiation. The ultrastructural development of muscle, peripheral nerve, and neuromuscular junctions was studied to determine the sequence of events preceding hypotrophic ...
R G, Russell, F T, Oteruelo
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Abnormal myomuscular junctions and AChE in a congenital neuromuscular disease
Muscle & Nerve, 1980AbstractWe studied a patient with a congenital neuromuscular disease clinically characterized by ophthalmoplegia, slight limb muscle weakness, and normal electromyography. In the muscle biopsy, there were frequent examples of interdigitation of areas of muscle and extracellular spaces giving a fragmented appearance to muscle fibers.
Bormioli S. P., Lucke S., Angelini C.
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Increased Probability of Release in Huntington's Disease Neuromuscular Junctions
The FASEB Journal, 2016Huntington's disease (HD) is a progressive and fatal degenerative disorder that results in debilitating cognitive and motor dysfunction. The majority of research related to HD has been focused on neurodegeneration in the central nervous system.
Ahmad Khedraki +3 more
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Diseases of Motor Neurons and Neuromuscular Junctions
2015Amyotrophic lateral sclerosis is the most common neurodegenerative disorder in younger to middle-aged adults. The progressive loss of upper (cortical) and lower (midbrain and spinal) motor neurons involved in voluntary movements causes a gradual paralysis that eventually affects all muscle groups, including those required for breathing, while ...
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Diseases of muscle and the neuromuscular junction
2018Orthopaedic disorders occur more frequently than neuromuscular diseases, but when a diagnosis cannot be reached following thorough orthopaedic evaluation, disorders of muscle and the neuromuscular junction should be considered. This chapter focuses on the most commonly encountered neuromuscular disorders with particular emphasis on those that can ...
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