Sudden death is a not uncommon event in the evolution of patients suffering from myotonic dystrophy type 1 (DM1). Japanese researchers have analysed the clinical, genetic and pathological data from three of these patients who died prematurely:
- the three patients, one male and two females, were aged 18, 25 and 35 respectively;
- only the 25-year-old woman had a disturbed electrocardiogram prior to the sudden death;
- A post-mortem study of the heart revealed tissue abnormalities in only two patients;
- a complementary genetic study identified several sequence variations possibly linked to the risk of sudden death in genes other than DMPK1.
