What is the treatment of Myotonia Congenita?

By  ,  Onlymyhealth editorial team
Oct 10, 2012

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Quick Bites

  • Myotonia congenita is an inherited neuromuscular disorder.
  • There are two forms - Becker-type and Thomsen’s disease.
  • Most people with myotonia congenita don’t require special treatments.
  • Stiff muscles usually resolve with exercise, or light movement.

Myotonia congenita is an inherited neuromuscular disorder. The characteristic of the disorder is the inability of muscles to quickly relax after a voluntary contraction.  The condition is present since early childhood, but symptoms can be mild.  Most children will be 2 or 3 years old when parents first notice their muscle stiffness, particularly in the legs, often provoked by sudden activity after rest.  The disease doesn’t cause muscle wasting; in fact, it may cause muscle enlargement.  Muscle strength is increased.


myotonia congenita


There are two forms of the disorder - Becker-type, which is the most common form; and Thomsen’s disease, which is a rare and milder form.  The disorder is cause by mutations in a gene responsible for shutting off electrical excitation in the muscles.



In the condition, the muscles are unable to quickly relax after contracting. The early symptoms of myotonia congenitamay are difficulty swallowing and gagging. Stiff movements that improve when they are repeated is another characteristic of the condition.Shortness of breath or tightening of the chest at the beginning of exercise is another sign.


Most people with myotonia congenita don’t require special treatments. Stiff muscles usually resolve with exercise, or light movement, especially after resting. For individuals whose symptoms are more limiting, doctors have had some success with medications such as quinine, or anticonvulsant drugs such as phenytoin. Physical therapy and other rehabilitative therapies are also sometimes used to improve muscle function.



(Image source:Gettyimages)

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  • Dr Mukesh D Jain16 Nov 2011

    People with mild myotonia can manage their disease without medication but in severe cases treatment is usually necessary. Drugs that have been used to treat myotonia include sodium channel blockers such as procainamide, phenytoin and mexiletine, tricyclic antidepressant drugs such as clomipramine or imipramine, benzodiazepines, calcium antagonists, taurine and prednisone. Ayurvedic investigatory drug Mamsagni Rasayana have shown lasting relief of abnormal delay in the relaxation of muscles.