Becker muscular dystrophy


Definition

Becker muscular dystrophy is an inherited disorder that involves slowly worsening muscle weakness of the legs and pelvis.

Alternative Names

Benign pseudohypertrophic muscular dystrophy; Becker's dystrophy

Causes

This EM Should be displayed at the top of the article section "Causes"

Becker muscular dystrophy is very similar to Duchenne muscular dystrophy. The main difference is that it gets worse at a much slower rate and it is less common.

The disorder is passed down through families (inherited). Having a family history of the condition raises your risk.

Becker muscular dystrophy occurs in about 3 to 6 out of every 100,000 births. The disease is found mostly in boys.

Symptoms

Females rarely develop symptoms. Males will develop symptoms if they inherit the defective gene. Symptoms most often appear in boys between ages 5 and 15, but may begin later.

Muscle weakness of the lower body, including the legs and pelvis area, slowly gets worse, causing:

Other symptoms may include:

Exams and Tests

The health care provider will do a nervous system (neurological) and muscle exam. A careful medical history is also important, because symptoms are similar to those of Duchenne muscular dystrophy. However, Becker muscular dystrophy gets worse much more slowly.

An exam may find:

Tests that may be done include:

Treatment

There is no known cure for Becker muscular dystrophy. However there are many new drugs currently undergoing clinical testing that show significant promise in treating the disease.The current goal of treatment is to control symptoms to maximize the person's quality of life. Some providers prescribe steroids to help keep a patient walking for as long as possible.

Activity is encouraged. Inactivity (such as bed rest) can make the muscle disease worse. Physical therapy may be helpful to maintain muscle strength. Orthopedic appliances such as braces and wheelchairs may improve movement and self-care.

Genetic counseling may be recommended. Daughters of a man with Becker muscular dystrophy may carry the defective gene and could pass it on to their sons.

Support Groups

You can ease the stress of the illness by joining a muscular dystrophy support group where members share common experiences and problems.

Outlook (Prognosis)

Becker muscular dystrophy leads to slowly worsening disability. However, the amount of disability varies. Some people may need a wheelchair. Others may only need to use walking aids such as canes or braces.

Lifespan is most often shortened if there are heart and breathing problems.

Possible Complications

Complications may include:

When to Contact a Medical Professional

Call your provider if:

Prevention

Genetic counseling may be advised if there is a family history of Becker muscular dystrophy.

References

Amato AA. Disorders of the skeletal muscle. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 110.

Sarnat HB. Muscular dystrophies. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 609.

Selcen D. Muscle diseases. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 421.

A.D.A.M. content is best viewed in IE9 or above, Firefox and Google Chrome browser.