2018 Volume 35 Issue 4 Pages 561-566
Since 1999, the Japanese Muscular Dystrophy Research Group has been developing and maintaining a database regarding the clinical conditions of inpatients with muscular dystrophy and related disorders in 26 institutions belonging to the National Hospital Organization and National Center of Neurology and Psychiatry, which are surveyed each year on October 1. In this study, we analyzed the records of patients with myotonic dystrophy (DM). The total number of DM inpatients, 331 in 1999, showed a gradual increase to about 370 in 2005 and has since remained at about that level. The majority of inpatients were aged 40∼50 years and those 60 years and older have gradually increased. The rate of respirator dependence, 19.9% in 1999, has also gradually increased and has been greater than 50% since 2009. On the other hand, the rate of oral nutritional supply, 86.1% in 1999, has gradually decreased and became less than 50% in 2013. There were 602 cases of death reported in the database from 2000 to 2013, with a respiratory–related cause in approximately 30–50% and a heart–related cause noted in approximately 20–30%. Those two causes accounted for 50–80% of total death cases. The mean age at death has shown a gradual increasing tendency from 57.6 years old in 2000 to 59.6 years in 2013. Our findings indicate that DM inpatients being treated at institutions specialized in muscular dystrophy in Japan have aged over time and their general condition is serious. We should consider the measures for it.
