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It allows the motor neuron to transmit a signal to the muscle fiber, causing muscle contraction. [2] Muscles require innervation to function—and even just to maintain muscle tone, avoiding atrophy. In the neuromuscular system, nerves from the central nervous system and the peripheral nervous system are linked and work together with muscles. [3]
Neuromuscular junction diseases are a result of a malfunction in one or more steps of the above pathway. As a result, normal functioning can be completely or partially inhibited, with the symptoms largely presenting themselves as problems in mobility and muscle contraction as expected from disorders in motor end plates.
The electrical impulse travels along the pre-synaptic neurone axon to synapse with the muscle at the neuromuscular junction (NMJ) to cause muscle contraction. [17] When the action potential reaches the axon terminal, it triggers the opening of the calcium ion gated channels, which causes the influx of Ca 2+.
This causes prolonged stimulation and desensitization of neuroreceptors, causing skeletal muscle relaxation effects such as paralysis. [1] Depolarizing neuromuscular blockers, notably succinylcholine, tend to be preferred over non-depolarizing neuromuscular blockers due to their long-acting and rapid-onset properties. [1]
This magnified image of type 2 muscle fibers shows denervation atrophy occurring at the white spaces at the top left and bottom center of the image. The white space represents a disruption of the nerve fibers, resulting in a loss of nerve supply to the muscle fibers. Denervation is any loss of nerve supply regardless of
Myotonia congenita is a congenital neuromuscular channelopathy that affects skeletal muscles (muscles used for movement). It is a genetic disorder.The hallmark of the disease is the failure of initiated contraction to terminate, often referred to as delayed relaxation of the muscles and rigidity. [1]
In myasthenia gravis, the receptor at the neuromuscular junction is targeted by antibodies, leading to muscle weakness. Muscarinic acetylcholine receptors can be blocked by the drugs atropine and scopolamine. Congenital myasthenic syndrome (CMS) is an inherited neuromuscular disorder caused by defects of several types at the neuromuscular ...
Myoclonic dystonia or Myoclonus dystonia syndrome is a rare movement disorder that induces spontaneous muscle contraction causing abnormal posture. The prevalence of myoclonus dystonia has not been reported, however, this disorder falls under the umbrella of movement disorders which affect thousands worldwide. [1]