[Steinert's myotonic dystrophy. Attempts at therapy]

Myotonic dystrophy or Steinert's disease is an autosomal dominant hereditary disease affecting the entire system. Apart from the myotonic phenomenon it involves muscular atrophy, endocrine disorders, baldness, cardiac arrhythmias, hyperglycaemia, cataracts. Over the years a number of drugs have been tried with results on the myotonia that are still debated. They include quinine, corticosteroids, L-DOPA, potassium-binding resins, procainamide, phenytoin, diphenylhydantoin, N-propylajmaline, dantrolene sodium, carbamazepine, imipramine, baclofen, mexiletine. It is emphasised that only the identification of the biochemical defect involved in the disease will permit any efficient treatment of its symptoms or causes.