Myopathies

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Myopathy Treatment

Treatment for myopathies depends on the cause. The goals of myopathy treatment are to slow progression of the disease and relieve symptoms. Treatments range from drug therapy for muscular dystrophies and inflammatory myopathies to avoiding situations that work the muscles too hard for metabolic myopathies. Some physicians recommend that patients with myopathy keep their weight down (a lighter body demands less work from the muscles) and avoid overexerting the muscles.

When breathing problems develop, an incentive spirometer can help improve breathing function in some patients. Unfortunately, there is no way to strengthen the breathing muscles.

Muscular Dystrophies
The goals of MD treatment are to slow progression of disease and relieve symptoms. Duchenne MD and Becker MD are the subjects of current medical research and clinical trials may be available for patients with either disease.

Corticosteroids (e.g., deflazacort, prednisone) seem to be the most effective medications. Both improve strength and walking ability for about 6 months in boys with Duchenne dystrophy. Following initial improvement, further progression of the disease may be delayed for 3 to 5 years in some cases.

Prolonged use of corticosteroids can cause severe side effects including the following:

• Bone loss (osteoporosis)
• Depression
• High blood pressure (hypertension)
• Thinning of the skin
• Weight gain

Calcium supplements and antidepressants may be prescribed to counteract these side effects.

Preventive treatment for permanent contraction of a muscle (contractures) includes physical therapy and bracing. There are currently no drugs available to prevent or treat contractures.

Heel cord surgery (also called tendon release) and spine-straightening surgery (i.e., rod insertion) may be necessary in cases of severe contractures. Heel cord surgery is performed when the patient is still able to walk. Braces are usually required following surgery.

Endocrine Myopathies
Often, treating the underlying condition helps relieve muscle weakness and pain associated with endocrine myopathies.

Inflammatory Myopathies
Inflammatory myopathies, such as polymyositis and dermatomyositis, are usually treated with drugs that suppress the action of the immune system. Prednisone is most commonly used to treat inflammatory myopathies. It is used initially in high doses (up to 100mg/day) and then slowly tapered to the lowest possible dose that relieves symptoms. Long-term use of prednisone can cause severe side effects, including bone loss, depression, and high blood pressure.

Metabolic Myopathies
The primary goal in treating metabolic myopathies is to avoid situations that tax the muscles and promote muscle pain and weakness, like strenuous exercise.

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