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Becker Muscular Dystrophy

 

 

Becker Muscular Dystrophy (BMD) is an X-linked recessive disease very similar, although milder, to Duchenne Muscular Dystrophy.  It is a slow progressive disease that predominantly affects males.  A diagnosis is generally made in the early teens although symptoms may be present as early as in childhood.  Muscle cramps are an early symptom of BMD.  Affected individuals may have had difficulty in learning how to walk as a toddler.  Later symptoms include difficulty in walking, running, climbing stairs and lifting heavy objects.  Some muscles, especially calf muscles, tend to enlarge as the disease progresses.  Other muscles including thigh, upper arm and shoulder muscles may waste away.  Patients may lose the ability to walk later in life as the disease progresses.

 

The cause of BMD lies in the production of a protein called dystrophin.  A mutation on the gene for dystrophin causes an abnormally smaller dystrophin to be produced.  Although the mechanism is unknown, this abnormal dystrophin causes muscle fibers to gradually break down causing muscle weakness.

 

There is no cure for BMD.  Regular exercise and physical therapy are generally prescribed to patients to combat the weakening of the muscles. Strenuous physical activity and competition is discouraged for patients with BMD for they are more vulnerable to injury.  The use of a wheelchair may be necessary later in life.  A “night splint,” can be used to apply a slight stretch on the calf muscles to prevent cramps while sleeping.  Mental capacity is rarely affected.

 

Genetic counseling is strongly recommended for families with a history of all types of muscular dystrophy.  In families with Becker Muscular Dystrophy, a new test called Preimplantation Genetic Diagnosis (PGD) maybe beneficial.  PGD tests for genetic abnormalities and is able to determine the gender of an embryo prior to implantation.  Through this technique, the chances of having an affected can be significantly reduced.  A thorough genetic evaluation can determine whether PGD will be beneficial.  PGD can currently test for various genetic disorders including Duchenne Muscular Dystrophy and will hopefully able to test for many more hereditary diseases in the future.

 

 

Click here for more information on PGD

 

Click here to learn about other diseases

 

 

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Last modified: 06/09/04