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What is the prognosis for individuals that have the congenital form of myotonic dystrophy?
GaleEncyofNeuroDis
The congenital form is the most severe, although patients live, on average, until they are 45 years old.
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What are the symptoms of congenital myotonic dystrophy?
Symptoms in the congenital form of myotonic dystrophy are evident at birth. Affected infants show muscle weakness, respiratory defects, and eventually, mental retardation
What is the prognosis for individuals that have the classic form of myotonic dystrophy?
Affected individuals that have the classic form have a more severe prognosis. They have more clinical manifestations and lifespan usually ranges 48-55 years.
What does diagnosis for myotonic dystrophy entail?
Myotonic dystrophy is diagnosed clinically in individuals that have a specific type of muscle weakness. This is confirmed with molecular genetics testing, where the DMPK is analyzed
How may myotonic dystrophy be said to be a rare disorder?
Myotonic dystrophy is relatively rare, occurring approximately once in 8,000 people. There is also a more rare, severe congenital form that occurs with an incidence of about 1 in 100,000.
Does Myotonic dystrophy have attributed behavior problems?
Myotonic dystrophy does not have attributed behavior problems per se. However, Childhood and adolescent forms of the disease can exhibit cognitive difficulties which can present in a number of different ways such as attention deficit type disorders which could be mislabeled as behavior problems. Your best source of information on Myotonic Dystrophy on the web is the Myotonic Dystrophy Foundation.
What is the treatment for myotonic dystrophy?
Myotonic dystrophy cannot be cured, and no treatment can delay its progression. As of the early 2000s there is no standardized treatment for these disorders because the precise reasons for muscle weakness are not yet fully understood.
What is the symptoms of myotonic dystrophy?
People who have myotonic dystrophy have progressive muscle wasting and weakness beginning in their 20's or 30's. The muscle wasting and weakness develop in their lower legs, hands, neck and face.
What are the effects of myotonic dystrophy?
limitations resulting from myotonic dystrophy can be significant, involving muscle weakness and difficulty lifting items and performing certain routine daily tasks. There are many cases in which affected persons experience mental delays
Can you be a carrier of myotonic dystrophy if you test negative by EMG?
Yes, myotonic dystrophy affects people in multiple ways and can often affect other systems of the body before the skeletal muscles therefore an EMG is no longer a reliable diagnostic tool.
What systems are affected with Myotonic dystrophy?
affects heart muscle, causing arrhythmias and heart block, and the muscles of the digestive system, leading to motility disorders and constipation. Other body systems are affected as well: Myotonic dystrophy may cause cataracts
What special concerns are associated with myotonic dystrophy?
Premutation carriers, or individuals that have repeats that do not usually cause disease but are likely to expand in their offspring, should be identified (if possible) in cases where there is a family history of the disorder
What is the frequency of myotonic dystrophy in your population?
stated to be 1 in 8000 in the USA, a few places have a higher incidence rate
