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Ency. home > Disease > B > Becker's muscular dystrophy

Becker's muscular dystrophy   

Overview | Symptoms | Treatment | Prevention

Alternative names:

Benign pseudohypertrophic muscular dystrophy

Definition:

Becker's muscular dystrophy is an inherited disorder characterized by slowly progressive muscle weakness of the legs and pelvis.

Causes and Risks

Becker's muscular dystrophy is very similar to Duchenne's muscular dystrophy, except that it progresses at a much slower rate.

The disorder is inherited with an X-linked recessive inheritance pattern -- the gene is located on the X chromosome. Men will develop symptoms if the female transmits the gene for the disorder. Women may be carriers of the gene.

There is progressive muscle weakness of the legs and pelvis which is associated with a loss of muscle mass (wasting). Muscle weakness also occurs in the arms, neck, and other areas, but not as severely as in the lower half of the body.

Calf muscles initially enlarge (an attempt by the body to compensate for loss of muscle strength), but the enlarged muscle tissue is eventually replaced by fat and connective tissue (pseudohypertrophy).

Muscle contractures occur in the legs and heels, causing inability to use the muscles because of shortening of muscle fibers and fibrosis of connective tissue. Bones develop abnormally, causing skeletal deformities of the chest and other areas.

Cardiomyopathy does not occur as commonly as with Duchennes cases. Cognitive problems may accompany the disorder, but it is not inevitable and does not worsen as the disorder progresses.

Becker's muscular dystrophy occurs in approximately 3 to 6 out of 100,000 male births. Symptoms usually appear in men at about age 12, but may be later. The average age of becoming unable to walk is 25-30. Women rarely develop symptoms.

Because this is an inherited disorder, risks include having a family history of Becker's muscular dystrophy.

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