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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]
Depiction of smooth muscle contraction. Muscle contraction is the activation of tension-generating sites within muscle cells. [1] [2] In physiology, muscle contraction does not necessarily mean muscle shortening because muscle tension can be produced without changes in muscle length, such as when holding something heavy in the same position. [1]
The sliding filament theory explains the mechanism of muscle contraction based on muscle proteins that slide past each other to generate movement. [1] According to the sliding filament theory, the myosin ( thick filaments ) of muscle fibers slide past the actin ( thin filaments ) during muscle contraction, while the two groups of filaments ...
Myotonia is a symptom of a small handful of certain neuromuscular disorders characterized by delayed relaxation (prolonged contraction) of the skeletal muscles after voluntary contraction or electrical stimulation, and the muscle shows an abnormal EMG. [1] [2]
Diagram of skeletal muscle fiber structure. Skeletal muscle cells are the individual contractile cells within a muscle and are more usually known as muscle fibers because of their longer threadlike appearance. [10] Broadly there are two types of muscle fiber performing in muscle contraction, either as slow twitch or fast twitch .
To elicit muscle contraction, the muscle is stimulated by a series of electrical pulses delivered by an electrode to stimulate either the motor nerve or the muscle tissue itself. Simultaneously, a computer-controlled servo motor in the testing apparatus oscillates the muscle while measuring the force generated by the stimulated muscle.
Sliding filament model of muscle contraction. Cardiac sarcomere structure featuring myosin. Myosin II (also known as conventional myosin) is the myosin type responsible for producing muscle contraction in muscle cells in most animal cell types. It is also found in non-muscle cells in contractile bundles called stress fibers. [18]
Like skeletal muscle contractions, Calcium (Ca 2+) ions are required for polarization and depolarization through a voltage-gated calcium channel. The rapid influx of calcium into the cell signals for the cells to contract. When the calcium intake travels through an entire muscle, it will trigger a united muscular contraction.