A broad natural history study in myotonic dystrophies

The American Myotonic Dystrophy Registry is, after the French DM-scope database, one of the most important registries for Steinert disease (DM1) and myotonic dystrophy type 2 (DM2).

An analysis of the data recorded during the first 17 years of operation of this register has been published. It includes 929 people with DM1, excluding infantile and paediatric forms, and 222 with DM2, followed for an average of 7 years.

  • In DM1, even after excluding infantile and paediatric forms, the disease starts earlier. The use of technical aids and assisted ventilation occurs earlier than in DM2.
  • In DM2, the diagnosis is made later than in DM1 and the risk of developing diabetes is higher.

 

Milestones of progression in myotonic dystrophy type 1 and type 2. Johanna I Hamel, Michael P McDermott, James E Hilbert et al. Muscle Nerve . 2022 Jul