A first positive assessment of the Japanese myotonic dystrophies registry

Steinert’s disease or myotonic dystrophy type 1 (DM1) is, in Japan as elsewhere, one of the most common neuromuscular diseases, especially in the adult population. The census of patients is carried out in Japan through the national neuromuscular registry called Remudy:

  • the sub-registry devoted to myotonic dystrophies (DM) created complies with international standards as laid down by the Treat-NMD alliance and the ENMC,
  • it includes 976 DM patients (in March 2020), only one of whom has the DM2 variant (myotonic dystrophy type 2),
  • the congenital forms seem to be particularly prevalent, which represent 11% of all the cases of DM1 identified,
  • the registry is useful for normalizing care standards and studying the natural history of the disease.


Characteristics of myotonic dystrophy patients in the national registry of Japan. Sugimoto M, Kuru S, Takada H, et al. J Neurol Sci. 2022 Jan 15;432:120080