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Neuromuscular Junction Disordersa 

Neuromuscular Junction Disordersa
Chapter:
Neuromuscular Junction Disordersa
Author(s):

Brent P. Goodman

DOI:
10.1093/med/9780190244927.003.0047
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date: 23 October 2019

The neuromuscular junction 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. Generation of muscle fiber contraction involves a coordinated series of steps that ultimately generates an action potential at the muscle endplate (also known as an end-plate potential). The end-late potential normally 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 neuromuscular junction reduce this safety factor, a change that results in fatigable weakness. Review of the events involved in the generation of a muscle fiber action potential at the level of the neuromuscular junctionfacilitates greater understanding of neuromuscular junction disorders, which include myasthenia gravis, Lambert-Eaton myasthenic syndrome, botulism, and congenital myasthenic syndromes (eg, primary AChR deficiency, plectin deficiency, and sodium channel defect).

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