Described as the “most variable of all diseases found in medicine,” myotonic dystrophy (DM) is a multi-systemic genetic disorder that is the most common form of muscular dystrophy. There are different types of DM (DM1, DM2 and congenital DM), and some cause more serious problems than others.
There is currently no cure for myotonic dystrophy and for the most part DM is not well understood by the general medical community, requiring that people living with myotonic dystrophy and their caregivers be advocates who help their clinicians understand the disease and best practices with regard to DM care. Myotonic dystrophy is a very complicated condition. The symptoms and disease progression can vary widely, and effects can be quite different even among members of the same family.
The most common effects of DM are muscle problems, including muscle weakness (myopathy), trouble relaxing a muscle (myotonia), and muscle wasting that gets worse over time (atrophy). DM also affects many other body functions, including the heart, lungs, and gastrointestinal (GI) system. The disorder can also cause problems with cognitive function, personality, behaviour and vision.
DM is also characterised by anticipation, a phenomenon in which the DM gene tends to increase with each affected generation. As a result, the symptoms of DM1 appear earlier in life and are more severe in each successive generation. Anticipation seems to be less severe in DM2.
Regardless of the form of DM or the severity of symptoms experienced by a patient, individuals with myotonic dystrophy can have severe and life-threatening reactions to anesthesia and should be monitored carefully whenever anesthesia is administered. For more comprehensive information on myotonic dystrophy, visit the following websites: