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Myotonia

In medical terms, Myotonia is a neuromuscular condition. It is characterized by delayed relaxation of muscles after voluntary muscle contraction. It can affect any muscle type, the skeletal muscles, cardiac muscles (obvious) and smooth muscles (not obvious). The condition is usually inherited.


Myotonia is a characteristic feature in Myotonic Dystrophy, Myotonia Congenital and Paramyotonia Congenital. Myotonia is only a part in myotonic dystrophy. In Myotonia congenital and paramyotonia, it is the only main symptom. Also, in people who have a group of neurological disorders called the channelopathies, Myotonia is a symptom.


What happens in Myotonia?

Extra effort is required to relax the muscle soon after contraction. When the same action is repeated immediately, the condition improves as the muscles have warmed up.

Releasing the grip on objects or difficulty rising from a seated position is common with people with Myotonia. Immediately after rising from a seated position the individual may walk with a stiff, awkward gait. The posture improves after taking few steps forward. Another incidence is the time lag in opening eyes after initial tight eyelid closure.


What causes Myotonia?

Myotonia is caused by mutations in the chloride sodium or potassium channels that regulate the muscle membrane. Specific triggers that have been recognized so far include stress and exposure to cold.


What is the treatment for Myotonia?

If the condition is mild, exercise would suffice. If the condition is severe, treatment is necessary. Neurologist or medical geneticist with specific knowledge of the condition treats Myotonia. After a physical examination and a detailed family history, doctors prescribe medicines. Sodium channel blockers such as procainamide, phenytoin and mexiletine, tricyclic antidepressant drugs such as clomipramine or imipramine, benzodiazepines, calcium antagonists, taurine and prednisone are prescribed for the treatment. Physical therapy and other rehabilitative measures are recommended to help muscle function.


What is the prognosis for Myotonia?

Recognized as a chronic disorder, prescription medications when combined with physical therapy and other rehabilitative measures help alleviate symptoms later in life.


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Collection of Pages - Last revised Date: December 9, 2019