Beckers muscular dystrophy

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Slide 1 :

Becker's muscular dystrophy

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

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Symptoms *Cognitive problems *Fatigue & Frequent falls *Loss of balance and coordination *Muscle weakness in the arms, neck, and other areas (not as severe as in the lower body) *Muscle weakness of the legs and pelvis that slowly worsens, causing *Difficulty walking that worsens over time *The average age of becoming unable to walk is age 25 – 30 *Difficulty with muscle skills (running, hopping, jumping) *Loss of muscle mass (wasting)*Problems breathing

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Treatment There is no known cure for Becker's muscular dystrophy. Treatment tries to control symptoms to maximize quality of life. Some doctors prescribe steroids to help keep a patient walking for as long as possible. Activity is encouraged. Inactivity (such as bed rest) can worsen the muscle disease. Physical therapy may be helpful to maintain muscle strength. Orthopedic appliances such as braces and wheelchairs may improve mobility and self-care. Genetic counseling may be advisable. Sons of a man with Becker's muscular dystrophy do not develop the disorder, but daughters may be carriers. The daughters' sons may develop the disorder.

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Causes Becker's muscular dystrophy is very similar to Duchenne's muscular dystrophy, except that it gets worse at a much slower rate. The disorder is inherited. Women rarely develop symptoms. Men will develop symptoms if they inherit the defective gene. Becker's muscular dystrophy occurs in approximately 3 - 6 out of every 100,000 males. Because this is an inherited disorder, risks include having a family history of Becker's muscular dystrophy.

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Tests & diagnosis *Abnormally developed bones *cardiomyopathy *irregular heartbeat (arrhythmias) *Muscle deformities *Contractures of heels and legs *Fat and connective tissue (pseudohypertrophy) in calf muscles *Muscle wasting that begins in the legs and pelvis. *CPK & Electromyography (EMG) *Muscle biopsy or genetic test (blood test) 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's muscular dystrophy gets worse much more slowly. An exam may find:

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Prognosis Becker's muscular dystrophy leads to slowly worsening disability. Death may occur in the 50s or 60s, but patients can live longer than that.

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

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Complications *Cardiomyopathy *Deformities *Permanent, worsening disability *Decreased ability to care for self *Decreased mobility *Mental impairment (varies) -- see mental retardation *Pneumonia or other respiratory infections *Respiratory failure

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When to contact a doctor Call your health care provider if:

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Beckers muscular dystrophy
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