Becker's Muscular Dystrophy

Definition

Definition of Becker's Muscular Dystrophy
Becker muscular dystrophy (also known as Benign pseudohypertrophic muscular dystrophy) is an X-linked recessive inherited disorder characterized by slowly progressive muscle weakness of the legs and pelvis.

It is a type of dystrophinopathy, which includes a spectrum of muscle diseases in which there is insufficient dystrophin produced in the muscle cells, resulting in instability in the structure of muscle cell membrane. This is caused by mutations in the dystrophin gene, which encodes the protein dystrophin. Becker muscular dystrophy is related to Duchenne muscular dystrophy in that both result from a mutation in the dystrophin gene, but in Duchenne muscular dystrophy no functional dystrophin is produced making DMD much more severe than BMD.

Symptoms

Symptoms of Becker's Muscular Dystrophy

1. Females rarely develop symptoms. Males will develop symptoms if they inherit the defective gene. Symptoms usually appear in boys at about age 12, but may begin later.
2. Muscle weakness of the lower body, including the legs and pelvis area, slowly gets worse, causing:

• Difficulty walking that gets worse over time; by age 25-30 the person is usually unable to walk.
• Frequent falls
• Difficulty with running, hopping, and jumping
• Loss of muscle mass

Causes

Causes of Becker's Muscular Dystrophy

1. Becker muscular dystrophy is very similar to Duchenne muscular dystrophy, except that it gets worse at a much slower rate.
2. The disorder is passed down through families (inherited). Having a family history of the condition raises your risk.
3. Becker muscular dystrophy occurs in approximately 3 - 6 out of every 100,000 males. It is less common than Duchenne muscular dystrophy.

Diagnosis

Diagnosis of Becker's Muscular Dystrophy
The health care provider will do a nervous system (neurological) and muscle examination. A careful medical history is also important, because the pattern of symptom development resembles that of Duchenne's muscular dystrophy. However, Becker muscular dystrophy gets worse much more slowly.

Treatment

Treatment of Becker's Muscular Dystrophy
There is no known cure for Becker muscular dystrophy. The goal of treatment is to control symptoms to maximize the person's quality of life. Some doctors prescribe steroids to help keep a patient walking for as long as possible.

Prognosis

Prognosis of Becker's Muscular Dystrophy
Becker muscular dystrophy leads to slowly worsening disability, although the degree of disability varies. Some men may need a wheelchair, while others may only need to use walking aids such as canes or braces.

Prevention

Prevention of Becker's Muscular Dystrophy
Genetic counseling may be advised if there is a family history of Becker muscular dystrophy.