Adult myotonic muscular dystrophy (Steinert's disease) is the most common inherited
neuromuscular disorder. Cardiac rhythm disturbances occur frequently in this disease state
and may be responsible for up to one-third of deaths. In this study, we intend to evaluate
the utility of non-invasive electrocardiographic screening methods and history in predicting
serious arrhythmic events.
The long term objectives of this population study is a more defined natural history, optimal
diagnostic testing methodology, and methods of therapy for arrhythmias in individuals with
myotonic muscular dystrophy. The goal is a more adequate definition of appropriate diagnosis
and therapy for arrhythmias in order to decrease the likelihood of cardiac morbidity and
mortality in this disorder.
The specific aims of the study involve an initial survey of individuals with myotonic
muscular dystrophy detailing multiple factors. Non-invasive electrocardiographic testing
will be done. Using this initial data and subsequent follow-up data collected yearly the
cohort of patients will be followed as to arrhythmia development over a minimum of five
years and likely longer with a long-term registry and evaluation of National Death Records
and Ancestry.com. This project is unique in that it characterizes a non-neurologic
abnormality associated with a neuromuscular disease, myotonic muscular dystrophy.
1. Age 18 and over
2. Willing to sign informed consent
3. Have a previous diagnosis of myotonic muscular dystrophy
1. Under age 18.
2. Unwilling to sign consent.
3. Unwilling to commit to long-term follow-up.