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Becker Muscular Dystrophy

Disorder characterized by slowly progressing weakness in the muscles of the legs and pelvis.

PubMed Health Glossary
(Source: NIH - National Cancer Institute)

About Becker Muscular Dystrophy

Becker MD is less severe than but closely related to Duchenne MD. People with Becker MD have partial but insufficient function of the protein dystrophin. There is greater variability in the clinical course of Becker MD compared to Duchenne MD. The disorder usually appears around age 11 but may occur as late as age 25, and affected individuals generally live into middle age or later. The rate of progressive, symmetric (on both sides of the body) muscle atrophy and weakness varies greatly among affected individuals. Many individuals are able to walk until they are in their mid-thirties or later, while others are unable to walk past their teens. Some affected individuals never need to use a wheelchair. As in Duchenne MD, muscle weakness in Becker MD is typically noticed first in the upper arms and shoulders, upper legs, and pelvis.

Early symptoms of Becker MD include walking on one's toes, frequent falls, and difficulty rising from the floor. Calf muscles may appear large and healthy as deteriorating muscle fibers are replaced by fat, and muscle activity....Read more about Becker Muscular Dystrophy NIH - National Institute of Neurological Disorders and Stroke

What works? Research summarized

Evidence reviews

Calcium channel blocking drugs for Duchenne muscular dystrophy

Duchenne muscular dystrophy is a progressive wasting condition of muscles which starts in early childhood, leads to dependence on a wheelchair by the age of thirteen and respiratory failure by late teens. The condition is due to absence of dystrophin, a large muscle protein that has several functions within muscle cells. We know that calcium molecules build up in the muscle cells of people with Duchenne muscular dystrophy and this is associated with cell death. The rationale behind this review was to ascertain whether randomised controlled trials using drugs that block calcium entry into muscle would result in a reduction in progression of the condition. Although these trials were conducted over ten years ago a systematic review was not done at that time, and so a potential effect of calcium blocking drugs (antagonists) on the course of DMD may have been missed. If it were to exist, calcium antagonists might be an effective treatment in their own right or, more likely, could be used in combination with newer treatments such as corticosteroids or potential treatments such as gene related therapies.

Scoliosis surgery for people with Duchenne muscular dystrophy

Scoliosis, curvature of the spine, is common in people with Duchenne muscular dystrophy. It is usually progressive and surgery is often performed aiming to halt its progression, improve cosmetic appearance, facilitate care, preserve upper limb and respiratory function, and hopefully increase life expectancy. However, there were no randomized controlled clinical trials available to evaluate the effectiveness of scoliosis surgery. Randomized controlled clinical trials are needed in this group of patients to evaluate the benefits and risks of different surgical treatments. This is an updated review and an updated search was undertaken in which no new studies were found.

Glucocorticoid corticosteroid therapy in Duchenne muscular dystrophy (DMD) improves muscle strength and function for six months to two years.

Duchenne muscular dystrophy is an incurable disease of childhood. Muscle wasting and loss of walking lead to wheelchair dependence and eventually death. The precise way in which glucocorticoids increase strength is unknown. Randomised controlled trials have shown that glucocorticoid corticosteroids improved muscle strength and function for six months to two years. Short‐term side effects were significant but not severe and could be managed. Data from non‐randomized studies suggests functional benefit over a five year period in many treated patients, but the overall long‐term benefit remains unclear, and has to be weighed against the long‐term side effects of these drugs. Randomised controlled trials to clarify this uncertainty are desirable, but would require careful ethical consideration.

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Summaries for consumers

Calcium channel blocking drugs for Duchenne muscular dystrophy

Duchenne muscular dystrophy is a progressive wasting condition of muscles which starts in early childhood, leads to dependence on a wheelchair by the age of thirteen and respiratory failure by late teens. The condition is due to absence of dystrophin, a large muscle protein that has several functions within muscle cells. We know that calcium molecules build up in the muscle cells of people with Duchenne muscular dystrophy and this is associated with cell death. The rationale behind this review was to ascertain whether randomised controlled trials using drugs that block calcium entry into muscle would result in a reduction in progression of the condition. Although these trials were conducted over ten years ago a systematic review was not done at that time, and so a potential effect of calcium blocking drugs (antagonists) on the course of DMD may have been missed. If it were to exist, calcium antagonists might be an effective treatment in their own right or, more likely, could be used in combination with newer treatments such as corticosteroids or potential treatments such as gene related therapies.

Scoliosis surgery for people with Duchenne muscular dystrophy

Scoliosis, curvature of the spine, is common in people with Duchenne muscular dystrophy. It is usually progressive and surgery is often performed aiming to halt its progression, improve cosmetic appearance, facilitate care, preserve upper limb and respiratory function, and hopefully increase life expectancy. However, there were no randomized controlled clinical trials available to evaluate the effectiveness of scoliosis surgery. Randomized controlled clinical trials are needed in this group of patients to evaluate the benefits and risks of different surgical treatments. This is an updated review and an updated search was undertaken in which no new studies were found.

Glucocorticoid corticosteroid therapy in Duchenne muscular dystrophy (DMD) improves muscle strength and function for six months to two years.

Duchenne muscular dystrophy is an incurable disease of childhood. Muscle wasting and loss of walking lead to wheelchair dependence and eventually death. The precise way in which glucocorticoids increase strength is unknown. Randomised controlled trials have shown that glucocorticoid corticosteroids improved muscle strength and function for six months to two years. Short‐term side effects were significant but not severe and could be managed. Data from non‐randomized studies suggests functional benefit over a five year period in many treated patients, but the overall long‐term benefit remains unclear, and has to be weighed against the long‐term side effects of these drugs. Randomised controlled trials to clarify this uncertainty are desirable, but would require careful ethical consideration.

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Terms to know

Atrophy
Decrease in the size of a cell, tissue, organ, or multiple organs.
Duchenne Muscular Dystrophy
The main symptom of Duchenne muscular dystrophy is muscle weakness associated with muscle wasting. DMD is inherited. Females will typically be carriers for the disease while males will be affected.
Dystrophin
A protein that helps maintain the shape and structure of muscle fibers.
Genetic
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
Muscle Atrophy (Muscle Wasting)
A weakening, shrinking, and loss of muscle caused by disease or lack of use.
Muscles
Muscles function to produce force and motion. They are primarily responsible for maintaining and changing posture, locomotion, as well as movement of internal organs, such as the contraction of the heart and the movement of food through the digestive system.

More about Becker Muscular Dystrophy

Photo of a young adult

Also called: Becker MD, Becker's disease, Becker's muscular dystrophy, Benign Duchenne muscular dystrophy, BMD

Other terms to know: See all 6
Atrophy, Duchenne Muscular Dystrophy, Dystrophin

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