Becker Muscular Dystrophy

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Becker Muscular Dystrophy
Body Parts: Whole Body
Medical Subjects: Genetic disease Children's Health
Overview

What Is Becker Muscular Dystrophy?

Becker muscular dystrophy is a rare degenerative disease of the muscle, which mainly manifests as slow, gradually aggravating and symmetrical muscle weakness and atrophy, leading to patients' difficulty in walking and frequent falls.

Becker muscular dystrophy is an X-linked recessive genetic disease that only occurs in boys. Girls will not get sick but will carry disease-causing genes, and their male offspring may get sick.

Currently, Becker muscular dystrophy cannot be cured. Patients onset at the age of 11 on average and die at the age of 42 on average. The main causes of death are respiratory muscle involvement and respiratory insufficiency.

How many people suffer from Becker muscular dystrophy?

Very rare, the incidence is about 3/100,000 male infants.

What is Duchenne muscular dystrophy?

Duchenne muscular dystrophy is a disease somewhat similar to Becker muscular dystrophy, but it is slightly more common, more severe, and older than Becker muscular dystrophy.

Cause

What Is The Cause Of Becker Muscular Dystrophy?

Becker type muscular dystrophy is an inherited disorder associated with mutations in the dystrophin gene at locus p21.2 on chromosome X.

The gene mutation causes abnormal production or insufficient production of "dystrophin" on the muscle cell membrane, leading to muscle weakness and muscle atrophy.

How is Becker muscular dystrophy inherited?

The genetic pattern of Becker muscular dystrophy is X-linked recessive inheritance. Namely:

  • None of the children born to the sick male will get sick, but if they are daughters, they will carry the disease-causing genes.
  • Sons born to women with disease-causing genes have a 50% chance of getting sick.

Is Becker muscular dystrophy contagious?

No.

Symptom

What Are The Symptoms Of Becker Muscular Dystrophy

Becker muscular dystrophy usually begins to show symptoms in childhood.

  • Muscle weakness: The child will first experience weakness in the pelvic muscles and thigh muscles, and then slowly spread to the shoulder muscles and limbs. It is manifested as slow walking, easy wrestling, difficulty in going upstairs and squatting, and obvious bulging of the shoulder blades when raising the arms.
  • Muscle atrophy: the child will also have muscle pseudohypertrophy (that is, muscle fiber atrophy, fat and connective tissue replace the atrophic muscle, the appearance looks like the muscle is large, and it is also very tough to the touch, but in fact, the muscle strength is weakened, the patient will feel unable to use it). The most obvious part of pseudohypertrophy is the gastrocnemius, which is the muscle on the back of the calf, and muscles in other parts may also occur.
Detect

How Is Becker Muscular Dystrophy Diagnosed?

Doctors will make comprehensive judgments based on the patients' clinical manifestations, genetic mode, age of onset, and familial onset, in combination with electromyography, muscle pathological examination, serum examination, genetic analysis, and others.

What tests are needed for Becker muscular dystrophy?

  • Serum enzyme test: It is a blood test that will check the content of muscle-related enzymes in the blood, such as creatine kinase, lactate dehydrogenase, and creatine kinase isoenzymes. Serum enzymes in Becker muscular dystrophy will be abnormally significantly increased (12-100 times the normal value).
  • Electromyography: Electrodes are applied to the muscles or Acupuncture electrode may be used to check the muscle response. Acupuncture electrode will cause sharp pain, just like inserting a needle.
  • Muscle biopsy: A small portion of muscle tissue is removed by simple surgery or invasive manipulation and taken to the laboratory for examination. Observe for necrosis and regeneration of muscle tissue and detect specific proteins in muscle cells.
  • Gene test: it directly tests the patient's body for any related gene mutation, it takes a long time.
  • Other examinations: X-ray, ECG, echocardiography and other examinations of the patient's heart involvement, CT or MRI can be found to the extent and degree of muscle damage.

What diseases are Becker muscular dystrophy easily confused with? 

Becker muscular dystrophy is easy to be confused with Duchenne muscular dystrophy, but Becker muscular dystrophy has a late onset age, and children can still walk after 12 years old, and their intelligence is normal, and the disease rarely affects the heart. Gene diagnosis can clearly distinguish these two diseases.

Prevention

Can Becker Muscular Dystrophy Be Prevented?

The best prevention method is genetic counseling and prenatal genetic diagnosis. If a sick fetus is detected, the pregnancy will be terminated to prevent the child from being born.

Treatment

How Is Becker Muscular Dystrophy Treated?

At present, there is no special treatment, and only corresponding treatment can be performed for clinical symptoms.

Moderate physical activity and passive exercise can delay disuse atrophy of muscles and avoid various complications caused by long-term bed rest.

Orthopedic treatment. The purpose is to maintain limb function and prevent contractures. Including: wearing ankle and foot orthoses during sleep to prevent flexion contractures, leg braces to help walking and standing, and orthopedic surgery to correct scoliosis.

Respiratory insufficiency occurs and respiratory support therapy is required, which helps to prolong life. Include non-invasive ventilation, tracheotomy.

Must Becker muscular dystrophy go to hospital?

Yes, it is necessary to confirm the diagnosis and evaluate the condition.

Can patients with Becker muscular dystrophy be cured after treatment?

Can't. Patients often die from complications caused by respiratory failure or prolonged bed rest.

Life

What Should Patients With Becker Muscular Dystrophy Pay Attention To In Life?

Muscles will degenerate if there is no exercise, so you should exercise properly, do not stay in bed for a long time, and engage in daily activities as much as possible to delay muscle atrophy.

Diet

There are no special taboos for foods. Pay attention to increase the protein food in the diet, such as eggs, meat, fish, etc. At the same time, due to reduced physical activity, we should pay attention to control food intake, prevent obesity.

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