Myotonic dystrophy is a relatively common type of muscular dystrophy, 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 death and causes of death in patients with the adult-onset type of myotonic dystrophy.
A register of myotonic dystrophy patients was set up in Southern Limburg (the Netherlands), using data longitudinally collected over a 47-year period (1950-97).
Survival for 180 patients (from the register) with adult-onset type myotonic dystrophy was established by the Kaplan-Meier method.
The median survival was 60 years for males and 59 years for females.
Survival of the patients 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 patients with adult-onset myotonic dystrophy is markedly reduced.
A weak positive correlation between the CTG repeat length and younger age at death was found in the 13 patients studied (r=0.50, P=0.08).
The cause of death could be determined in 70 of the 83 deceased patients. (...)
Mots-clés Pascal : Dystrophie myotonique, Age apparition, Mortalité, Survie, Long terme, Pays Bas, Europe, Pronostic, Etiologie, Epidémiologie, Adulte, Homme, Système nerveux pathologie, Neuromusculaire pathologie, Myotonie, Maladie héréditaire
Mots-clés Pascal anglais : Myotonic dystrophy, Age of onset, Mortality, Survival, Long term, Netherlands, Europe, Prognosis, Etiology, Epidemiology, Adult, Human, Nervous system diseases, Neuromuscular diseases, Myotonia, Genetic disease
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 98-0387986
Code Inist : 002B17H. Création : 25/01/1999. Dernière mise à jour : 12/08/2015.