Becker muscular dystrophy
Benign pseudohypertrophic muscular dystrophy; Becker's dystrophy
Becker muscular dystrophy is an inherited disorder that involves slowly worsening muscle weakness of the legs and pelvis.
Muscle weakness
Weakness is reduced strength in one or more muscles.
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.
Duchenne muscular dystrophy
Duchenne muscular dystrophy is an inherited disorder. It involves muscle weakness, which quickly gets worse.
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:
- Difficulty walking that gets worse over time; by age 25 to 30, the person is usually unable to walk
- Frequent falls
- Difficulty getting up from the floor and climbing stairs
- Difficulty with running, hopping, and jumping
- Loss of muscle mass
- Toe walking
- Muscle weakness in the arms, neck, and other areas is not as severe as in the lower body
Other symptoms may include:
- Breathing problems
- Cognitive problems (these do not get worse over time)
-
Fatigue
Fatigue
Fatigue is a feeling of weariness, tiredness, or lack of energy.
- Loss of balance and coordination
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:
-
Abnormally developed bones, leading to deformities of the chest and back (
scoliosis
)
Scoliosis
Scoliosis is an abnormal curving of the spine. Your spine is your backbone. It runs straight down your back. Everyone's spine naturally curves a b...
-
Abnormal heart muscle function (
cardiomyopathy
)
Cardiomyopathy
Cardiomyopathy is disease in which the heart muscle becomes weakened, stretched, or has another structural problem. It often occurs when the heart c...
-
Congestive heart failure
or irregular heartbeat (
arrhythmia
) - rare
Congestive heart failure
Heart failure is a condition in which the heart is no longer able to pump oxygen-rich blood to the rest of the body efficiently. This causes symptom...
Arrhythmia
An arrhythmia is a disorder of the heart rate (pulse) or heart rhythm. The heart can beat too fast (tachycardia), too slow (bradycardia), or irregul...
-
Muscle deformities, including
contractures
of heels and legs, abnormal fat and connective tissue in calf muscles
Contractures
A contracture develops when the normally stretchy (elastic) tissues are replaced by nonstretchy (inelastic) fiber-like tissue. This tissue makes it ...
- Muscle loss that begins in the legs and pelvis, then moves to the muscles of the shoulders, neck, arms, and respiratory system
Tests that may be done include:
-
CPK
blood test
CPK
Creatine phosphokinase (CPK) is an enzyme in the body. It is found mainly in the heart, brain, and skeletal muscle. This article discusses the test...
-
Electromyography (
EMG
) nerve testing
EMG
Electromyography (EMG) is a test that checks the health of the muscles and the nerves that control the muscles.
-
Muscle biopsy
or genetic blood test
Muscle biopsy
A muscle biopsy is the removal of a small piece of muscle tissue for examination.
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.
Stress
Stress is a feeling of emotional or physical tension. It can come from any event or thought that makes you feel frustrated, angry, or nervous. Stres...
Muscular dystrophy support group
The following organizations are good resources for information on muscular dystrophy:Muscular Dystrophy Association -- www. mda. orgNational Institut...
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:
- Heart-related problems such as cardiomyopathy
- Lung failure
-
Pneumonia
or other
respiratory
infections
Pneumonia
Pneumonia is a breathing (respiratory) condition in which there is an infection of the lung. This article covers community-acquired pneumonia (CAP). ...
Respiratory
The words "respiratory" and "respiration" refer to the lungs and breathing.
- Increasing and permanent disability that leads to decreased ability to care for self, decreased mobility
When to Contact a Medical Professional
Call your provider if:
- Symptoms of Becker muscular dystrophy appear
-
A person with Becker muscular dystrophy develops new symptoms (particularly
fever
with
cough
or
breathing difficulties
)
Fever
Fever is the temporary increase in the body's temperature in response to a disease or illness. A child has a fever when the temperature is at or abov...
Cough
Coughing is an important way to keep your throat and airways clear. But too much coughing may mean you have a disease or disorder. Some coughs are d...
Breathing difficulties
Breathing difficulty may involve:Difficult breathingUncomfortable breathingFeeling like you are not getting enough air
- You are planning to start a family and you or other family members have been diagnosed with Becker muscular dystrophy
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.
-
Superficial anterior muscles - illustration
Superficial muscles are close to the surface of the skin. Muscles which lie closer to bone or internal organs are called deep muscles.
Superficial anterior muscles
illustration
Review Date: 2/27/2016
Reviewed By: Amit M. Shelat, DO, FACP, Attending Neurologist and Assistant Professor of Clinical Neurology, SUNY Stony Brook, School of Medicine. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.