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Becker Muscular Dystrophy (BMD)

Becker Muscular Dystrophy (BMD)

What is Becker muscular dystrophy?

Becker muscular dystrophy affects the muscles of the hips, pelvic area, thighs and shoulders, as well as the heart.Becker muscular dystrophy (BMD) is one of nine types of muscular dystrophy, a group of genetic, degenerative diseases primarily affecting voluntary muscles. It is named after German doctor Peter Emil Becker, who first described this variant of Duchenne muscular dystrophy (DMD) in the 1950s. BMD is similar to DMD but allows the voluntary muscles to function better than they do in DMD. The heart muscle, however, can be affected similarly to the way it is in DMD.

What are the symptoms of BMD?

BMD's onset is usually in late childhood or adolescence, and the course is slower and less predictable than that of DMD. Generalized weakness first affects muscles of the hips, pelvic area, thighs and shoulders. Calves are often enlarged. There can be significant heart involvement. For more, see Signs and Symptoms.

What causes BMD?

Until the 1980s, little was known about the cause of any kind of muscular dystrophy. In 1986, MDA-supported researchers identified the gene that, when flawed — a problem known as a mutation — causes DMD and BMD. In 1987, the protein associated with this gene was identified and named dystrophin.

Genes contain codes, or recipes, for proteins, which are important biological components in all forms of life. BMD occurs when the dystrophin protein that's made from a particular gene on the X chromosome is only partially functional.

While DMD mutations cause virtually no functional dystrophin to be made, people with BMD make dystrophin that is partially functional. They make a shortened form of the protein, which protects the muscles of those with Becker from degenerating as completely or as quickly as those of people with DMD.

BMD primarily affects boys and men, who inherit the disease through their mothers. Women can be carriers but usually exhibit no symptoms. For more about the way gene mutations cause Becker dystrophy, see Causes/Inheritance.

What is life expectancy in BMD?

Most people with BMD survive well into mid- to late adulthood. For more about living with BMD, see Medical Management. If the cardiac aspects of the disease are minimal, or if they are adequately controlled through medical intervention, a normal or nearly normal life span can be expected.

What is the status of BMD research?

Researchers are actively pursuing several strategies in BMD. Among the major strategies are gene replacement; gene modification; stem cells; inhibiting a protein called myostatin; expanding the distribution and increasing the level of a protein called utrophin; and increasing blood flow to muscles.

MDA is supporting research in several of these areas. Human trials are under way for some of these strategies. For details, see Research.

How can I learn more about BMD?

Each of the 14 expert presentations made at the 2012 Becker Muscular Dystrophy Conference is available for viewing online. See BMD Conference Videos Cover Health Care, Research and Daily Living to learn more.

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