Myotonic Dystrophy Steinert disease








There is no treatment for this disorder although a number of preventive measures can contribute to reduce the risks and help people who have Steinert Disease.

Difficulty with walking

Difficulty with walking can be helped significantly by wearing high and rigid shoes or ankle braces in order to avoid sprains. If this measure is insufficient, it is suggested to wear tibia protectors.

Cardiac monitoring

Cardiac monitoring is essential throughout the progress of the disease. It is designed to detect cardiac anomalies and monitor their evolution in order to prevent heart attacks with the help of a pacemaker. An ECG at least once a year is necessary.

Respiratory failure

Respiratory failure is responsible for symptoms of fatigue. Monitoring of respiratory functions is necessary and can lead to nightly oxygen therapy which in many cases reduces fatigue. Respiratory and pulmonary infections must be avoided and anti flu vaccination is highly recommended.

Loss of dexterity

Loss of dexterity (hands and fingers) is mainly due to myotonia, i.e. the lack of muscular release after contraction. There are a certain number of medications that bring relief to this problem. Although the efficacy of the different medications is relative, improvement can be seen with Dilantin, quinine sulphates or Neurontin. Please discuss this aspect with your healthcare provider. Another significant problem associated with the disease is drowsiness, which disrupts daily activities. A certain number of medications such as Ritalin and modafinil (Alertec) have been shown to be effective although results are quite variable from one individual to another. Please discuss this with your healthcare provider.

Difficulty swallowing

Many patients complain about difficulty swallowing as well as episodes of choking. It must be understood that if you cough when consuming liquids or food this means that part of the liquid or food will pass into the bronchial tubes, which might become a contributing factor to reduced or compromised pulmonary function in the long run. If this happens it is important to contact a dietician in order to obtain advice on what type of food and liquids to avoid.

Non effective medications

Medications such as CoQ, DHEA, creatine or vitamins have shown some efficacy in the treatment of the disease. There is no medication or supplement to date that has shown significant efficacy.

Sports and Myotonic Dystrophy

It should be known that not all muscles are affected in the same way by this disease, so exercise is very good to maintain muscle function over time. Violent efforts are not recommended since they can worsen the weakened state of the muscles. If exercise brings on fatigue or leg cramps it means that the efforts are too strenuous. The intensity of the effort must thus be reduced.