@entity3918 is a relatively common @entity3986 , associated with a variety of systemic complications. Long term follow-up is difficult because of the slow progression. The objective of this study was to determine survival, age at @entity204 and causes of @entity204 in @entity1 with the adult-onset type of @entity3918 . A register of @entity3918 @entity1 was set up in Southern Limburg (the Netherlands), using data longitudinally collected over a 47-year period (1950-97). Survival for 180 @entity1 (from the register) with adult-onset @entity3918 was established by the Kaplan-Meier method. The median survival was 60 years for males and 59 years for females. Survival of the @entity1 was also estimated from the age of 15 years to the ages of 25, 45 and 65 years and compared with the expected survival of age- and sex-matched birth cohorts from the normal Dutch population. The observed survival to the ages of 25, 45 and 65 years was 99%, 88% and 18% compared with an expected survival of 99%, 95% and 78%, respectively. Thus, survival to the age of 65 in @entity1 with adult-onset @entity3918 is markedly reduced. A weak positive correlation between the CTG repeat length and younger age at @entity204 was found in the 13 @entity1 studied (r = 0.50, P = 0.08). The cause of @entity204 could be determined in 70 of the 83 deceased @entity1 . @entity55 and @entity943 were the most frequent primary causes of @entity204 , each occurring in approximately 30%, which was far more than expected for the general Dutch population. In addition, we assessed mobility in the years before @entity204 in a subgroup of 18 @entity1 , as a reflection of the long-term physical handicap in @entity3918 @entity1 . Half of the @entity1 studied were either partially or totally wheelchair-bound shortly before their @entity204 .

Age and causes of XXXX in adult-onset @entity3918 .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity943 :: ('MESH:D001145', 'Disease') :: ['cardiac arrhythmias'] @entity55 :: ('MESH:D011014', 'Disease') :: ['Pneumonia'] @entity3986 :: ('MESH:D009136', 'Disease') :: ['type of muscular dystrophy'] @entity3918 :: ('MESH:D009223', 'Disease') :: ['type myotonic dystrophy', 'Myotonic dystrophy', 'myotonic dystrophy'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death']
@entity204 :: (MESH:D003643,Disease) :: ['death']
