Beckers Muscular Dystrophy is a form of inherited disorder characterized by progressive muscle weakness in the pelvis and legs.
Defining Becker's Muscular Dystrophy
A form of muscular dystrophy that sets in in adolescence or adulthood and progresses slowly but will affect all voluntary muscles; characterized by generalized weakness and muscle wasting that affects limb and trunk muscles first; similar to Duchenne's muscular dystrophy but less severe; inheritance is X-linked recessive (carried by females but affecting only males). Females rarely develop symptoms. Males will develop symptoms if they inherit the defective gene. Symptoms usually appear in boys between ages 5 and 15, but may begin later.
Becker's Muscular Dystrophy is a form of inherited disorder that is characterized by slow, progressive muscle weakness which a person experiences in both their pelvis and their legs. Becker's Muscular Dystrophy is also known as, 'Benign Pseudohypertrophic Muscular Dystrophy.' The disorder is similar to Duchenne's Muscular Dystrophy with the difference being that it tends to progress more slowly.
Becker's Muscular Dystrophy is inherited with an x-linked, recessive inheritance pattern; the gene is located on the x chromosome. Men have both and x and y chromosome, without another x chromosome to compensate for a potentially defective gene. They will develop symptoms should they inherit a defective gene. Women have two x chromosomes. If of these x chromosomes has a defective gene present, the second x chromosome will have a functional, 'copy,' of the gene in order to compensate; in these cases women may present milder symptoms due to the ability to compensate.
Symptoms of Becker's Muscular Dystrophy
Persons with Becker's Muscular Dystrophy experience muscular weakness in both their pelvis and their legs that is progressive and is associated with loss of muscle mass referred to as, 'wasting.' Less severe muscle weakness is also experienced in the person's neck, arms and additional areas of the body, although not to as great an extent as in their lower body. Their calf muscles first enlarge through the body's attempts to compensate for their loss of muscle strength. Following this enlargement of the person's calf muscles, the tissue becomes replaced by connective tissues and fat; a process referred to as, 'Pseudohypertrophy.'
Additional muscle effects experienced by persons with Becker's Muscular Dystrophy include muscle contractures in their heels and legs, which cause an inability to use these muscles due to a shortening of muscle fibers and fibrosis of connective tissues. The person's bones develop in an abnormal manner, causing deformities in their skeletal structure in the chest and additional areas of the body. The heart muscle is not as commonly affected as with Duchenne's Muscular Dystrophy. Cardiopathy is not as much of an issue, although it is still something that persons with this disorder experience. Cognitive problems may be experienced by persons with Becker's Muscular Dystrophy - they are not an inevitability. Cognitive problems experienced by persons with this disorder do not worsen as the disorder progresses.
Rate of Occurrence
About three-to-six out of every 100,000 males born are affected by Becker's Muscular Dystrophy. The symptoms of the disorder commonly begin to appear in males around the age of twelve years, although they may begin at a later age. The inability to walk may begin around the ages of twenty-five to thirty; the ability to walk may continue up to around age forty. Women are rarely affected by Becker's Muscular Dystrophy.
Treatment of Becker's Muscular Dystrophy
Medical Science has found no cure for Becker's Muscular Dystrophy at this time. Treatment for the disorder is focused on controlling the symptoms the person experiences, as well as maximizing their quality of life. Activity on the part of the person is something that is encouraged because inactivity such as bed rest can actually worsen the person's muscle strength. Physical therapies may help to improve the person's muscle strength. Use of orthopedic appliances including braces or wheelchairs can help to improve the person's mobility and independence, as well as their ability to care for themselves.
Becker's Muscular Dystrophy is an inherited disorder. Because of this, genetic counseling is something that may be considered by those affected who are considering having children. A man with this disorder who has a son will find that their son will not develop the disorder. If the man has a daughter, their daughter might be a carrier of Becker's Muscular Dystrophy, and the daughter may one day have a son that potentially develops this disorder.
There are support groups available for persons with Becker's Muscular Dystrophy and family members and friends of those affected. The disorder can present a variety of stresses, and these groups can help. Persons with Becker's Muscular Dystrophy often experience a number of different complications such as deformities, decreased mobility, a decreased ability to care for themselves, respiratory infections or failure, and cardiomyopathy. The ability to share with others is important.