Neuromuscular Junction Disorders

2021 ◽  
pp. 772-778
Author(s):  
Brent P. Goodman
Keyword(s):  
Neuromuscular Junction ◽  
Action Potential ◽  
Motor Unit ◽  
Safety Factor ◽  
Muscle Fiber ◽  
Nerve Terminal ◽  
Neuromuscular Transmission ◽  
Critical Component ◽  
End Plate ◽  
Unmyelinated Nerve

The neuromuscular junction (NMJ) is a critical component of the motor unit that is made up of the distal, unmyelinated nerve terminal, synaptic space, and end-plate region of the muscle fiber. Contraction of muscle fiber involves a coordinated series of steps that ultimately generates an action potential at the muscle end plate (also known as an end-plate potential). Normally the end-plate potential substantially exceeds the threshold necessary to trigger an action potential in the muscle fiber, and this difference is termed the safety factor of neuromuscular transmission. Disorders that affect the NMJ reduce this safety factor, a change that results in fatigable weakness.

scholarly journals The Neuromuscular Junction in Health and Disease: Molecular Mechanisms Governing Synaptic Formation and Homeostasis

2020 ◽  
Vol 13 ◽  
Author(s):  
Pedro M. Rodríguez Cruz ◽  
Judith Cossins ◽  
David Beeson ◽  
Angela Vincent
Keyword(s):  
Neuromuscular Junction ◽  
Action Potential ◽  
Motor Neuron ◽  
Muscle Fiber ◽  
Neuromuscular Transmission ◽  
Molecular Mechanisms ◽  
Ex Vivo ◽  
Motor Nerve ◽  
And Function

The neuromuscular junction (NMJ) is a highly specialized synapse between a motor neuron nerve terminal and its muscle fiber that are responsible for converting electrical impulses generated by the motor neuron into electrical activity in the muscle fibers. On arrival of the motor nerve action potential, calcium enters the presynaptic terminal, which leads to the release of the neurotransmitter acetylcholine (ACh). ACh crosses the synaptic gap and binds to ACh receptors (AChRs) tightly clustered on the surface of the muscle fiber; this leads to the endplate potential which initiates the muscle action potential that results in muscle contraction. This is a simplified version of the events in neuromuscular transmission that take place within milliseconds, and are dependent on a tiny but highly structured NMJ. Much of this review is devoted to describing in more detail the development, maturation, maintenance and regeneration of the NMJ, but first we describe briefly the most important molecules involved and the conditions that affect their numbers and function. Most important clinically worldwide, are myasthenia gravis (MG), the Lambert-Eaton myasthenic syndrome (LEMS) and congenital myasthenic syndromes (CMS), each of which causes specific molecular defects. In addition, we mention the neurotoxins from bacteria, snakes and many other species that interfere with neuromuscular transmission and cause potentially fatal diseases, but have also provided useful probes for investigating neuromuscular transmission. There are also changes in NMJ structure and function in motor neuron disease, spinal muscle atrophy and sarcopenia that are likely to be secondary but might provide treatment targets. The NMJ is one of the best studied and most disease-prone synapses in the nervous system and it is amenable to in vivo and ex vivo investigation and to systemic therapies that can help restore normal function.

scholarly journals Near-Fibre Electromyography

2020 ◽  
Author(s):  
Mathew Piasecki ◽  
Oscar Garnés C. Estruch ◽  
Daniel W Stashuk
Keyword(s):  
Neuromuscular Junction ◽  
Motor Unit ◽  
Neuromuscular Transmission ◽  
Neuromuscular Disorders ◽  
Fibre Diameter ◽  
Time Variability ◽  
Motor Unit Potential ◽  
Muscle Recruitment ◽  
End Plate ◽  
High Pass

AbstractNear fibre electromyography (NFEMG) is the use of specifically high-pass filtered motor unit potential (MUPs) (i.e. near fibre MUPs (NFMs)) extracted from needle-detected EMG signals for the examination of changes in motor unit (MU) morphology and electrophysiology caused by neuromuscular disorders or ageing. The concepts of NFEMG, the parameters used, including NFM duration and dispersion, which relates to fibre diameter variability and/or endplate scatter, and a new measure of neuromuscular junction transmission (NMJ) instability, NFM segment jitter, and the methods for obtaining their values are explained. Evaluations using simulated needle-detected EMG data and exemplary human data are presented, described and discussed. The data presented demonstrate the ability of using NFEMG parameters to detect changes in MU fibre diameter variability, end plate scatter, and neuromuscular transmission time variability. These changes can be detected prior to alterations of MU size, numbers or muscle recruitment patterns.

Fractionation of the motor unit: response to tetanus

1961 ◽  
Vol 200 (4) ◽  
pp. 689-693
Author(s):  
Simeon Locke
Keyword(s):  
Action Potential ◽  
Motor Unit ◽  
Nerve Terminal ◽  
Differential Effect ◽  
Blocking Agent ◽  
Single Motor Unit ◽  
Single Motor ◽  
Blocking Agents ◽  
Before And After ◽  
Post Tetanic Potentiation

The effect of a tetanus on the motor unit of the gastrocnemius of the rat has been studied before and after administration of blocking agents. Post-tetanic potentiation of action potential of the single motor unit occurs following depression of response by curare or decamethonium. Increased amplitude of unit potential results from partial resynchronization of subunit potential contributions which had been desynchronized by the differential effect of the blocking agent on subunit latency. Decline of unit potential subsequent to post-tetanic potentiation results from desynchronization of component contributions as had been observed with initial administration of blocking agent. The occurrence of these events in a single motor unit indicates that they take place at the nerve terminal or subterminal portion of the unit.

Spike-triggered average torque and muscle fiber conduction velocity of low-threshold motor units following submaximal endurance contractions

2005 ◽  
Vol 98 (4) ◽  
pp. 1495-1502 ◽  
Author(s):  
Dario Farina ◽  
Lars Arendt-Nielsen ◽  
Thomas Graven-Nielsen
Keyword(s):  
Action Potential ◽  
Motor Unit ◽  
Conduction Velocity ◽  
Surface Potential ◽  
Muscle Fiber ◽  
Discharge Rate ◽  
Motor Units ◽  
Muscle Fiber Conduction Velocity ◽  
Sustained Contraction ◽  
Low Threshold

The motor unit twitch torque is modified by sustained contraction, but the association to changes in muscle fiber electrophysiological properties is not fully known. Thus twitch torque, muscle fiber conduction velocity, and action potential properties of single motor units were assessed in 11 subjects following an isometric submaximal contraction of the tibialis anterior muscle until endurance. The volunteers activated a target motor unit at the minimum discharge rate in eight 3-min-long contractions, three before and five after an isometric contraction at 40% of the maximal torque, sustained until endurance. Multichannel surface electromyogram signals and joint torque were averaged with the target motor unit potential as trigger. Discharge rate (mean ± SE, 6.6 ± 0.2 pulses/s) and interpulse interval variability (33.3 ± 7.0%) were not different in the eight contractions. Peak twitch torque and recruitment threshold increased significantly (93 ± 29 and 12 ± 5%, P < 0.05) in the contraction immediately after the endurance task with respect to the preendurance values (0.94 ± 0.26 mN·m and 3.7 ± 0.5% of the maximal torque), whereas time to peak of the twitch torque did not change (74.4 ± 10.1 ms). Muscle fiber conduction velocity decreased and action potential duration increased in the contraction after the endurance (6.3 ± 1.8 and 9.8 ± 1.8%, respectively, P < 0.05; preendurance values, 3.9 ± 0.2 m/s and 11.1 ± 0.8 ms), whereas the surface potential peak-to-peak amplitude did not change (27.1 ± 3.1 μV). There was no significant correlation between the relative changes in muscle fiber conduction velocity or surface potential duration and in peak twitch torque ( R2 = 0.04 and 0.10, respectively). In conclusion, modifications in peak twitch torque of low-threshold motor units with sustained contraction are mainly determined by mechanisms not related to changes in action potential shape and in its propagation velocity.

Acetaldehyde affects mammalian neuromuscular transmission without observable postsynaptic effects

1978 ◽  
Vol 56 (6) ◽  
pp. 1063-1066 ◽  
Author(s):  
Peter L. Carlen ◽  
Allan L. Staiman ◽  
William A. Corrigall
Keyword(s):  
Action Potential ◽  
Phrenic Nerve ◽  
Neuromuscular Transmission ◽  
Compound Action Potential ◽  
Base Line ◽  
Muscle Action ◽  
End Plate ◽  
Muscle Action Potential ◽  
Higher Doses ◽  
Compound Action

Acetaldehyde, the first metabolite of ethanol, caused a reversible block of the end-plate potential (EPP) in the rat and mouse phrenic nerve – hemidiaphragm preparation. Decrease and block of the EPP occurred over a bath concentration range from 3 to 25 mM. The phrenic nerve compound action potential was blocked along with the EPP, and this block was not reversed by high bath Ca2+ concentration. The muscle action potential was unaffected even at concentrations up to 50 mM. Over the same concentration range (3–25 mM), miniature end-plate potential (MEPP) frequency sometimes decreased a few minutes after application, and over the ensuing 10–20 min would steadily increase to as much as 11 times the base-line frequency, particularly with higher doses. However, the shape of averaged MEPPs remained unchanged after acetaldehyde application, suggesting that this aldehyde does not have postsynaptic effects.

scholarly journals CHANGES IN THE FINE STRUCTURE OF THE NEUROMUSCULAR JUNCTION OF THE FROG CAUSED BY BLACK WIDOW SPIDER VENOM

1972 ◽  
Vol 52 (1) ◽  
pp. 1-14 ◽  
Author(s):  
Allen W. Clark ◽  
William P. Hurlbut ◽  
Alexander Mauro
Keyword(s):  
Neuromuscular Junction ◽  
Nerve Terminal ◽  
Synaptic Vesicles ◽  
Spider Venom ◽  
Presynaptic Membrane ◽  
Black Widow ◽  
Black Widow Spider ◽  
End Plate ◽  
Black Widow Spider Venom ◽  
Widow Spider

Application of black widow spider venom to the neuromuscular junction of the frog causes an increase in the frequency of miniature end-plate potentials (min.e.p.p.) and a reduction in the number of synaptic vesicles in the nerve terminal. Shortly after the increase in min.e.p.p. frequency, the presynaptic membrane of the nerve terminal has either infolded or "lifted." Examination of these infoldings or lifts reveals synaptic vesicles in various stages of fusion with the presynaptic membrane. After the supply of synaptic vesicles has been exhausted, the presynaptic membrane returns to its original position directly opposite the end-plate membrane. The terminal contains all of its usual components with the exception of the synaptic vesicles. The only other alteration of the structures making up the neuromuscular junction occurs in the axon leading to the terminal. Instead of completely filling out its Schwann sheath, the axon has pulled away and its axoplasm appears to be denser than the control. The relation of these events to the vesicle hypothesis is discussed.

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