Steinert’s disease (or DM1 for myotonic dystrophy type 1) is one of the most common neuromuscular diseases in the country, mainly in the adult population. It is even more widespread in the Lake Saguenay region of Quebec. Its very varied clinical manifestations testify to a multisystemic involvement: muscular and cardiac involvement, cataract, various endocrine disturbances…
In an article published in June 2019, a Franco-Quebec consortium, headed by researchers from the Paris Institute of Myology, presents an overview of the DM-Scope registry. Supported by the AFM-Telethon, it is intended to promote clinical research and epidemiology in myotonic dystrophies. It contains the demographic, clinical and biological data of nearly 3,000 people, most of whom suffer from DM1 (2,828) and, more rarely, DM2 (142). About 10% of the data concern the paediatric population.
This is the largest DM1 registry in the world. The entire country is well covered by the registry, including overseas regions, thanks to close cooperation with 55 neuromuscular reference or competence centres. Since its creation, DM-Scope has initiated ten ancillary clinical studies.
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