Signs and Symptoms of Myopathy:
Although symptoms depend on the type of myopathy, some generalizations can be made. Skeletal muscle weakness is the hallmark of most myopathies, with some noticeable exceptions, such as myotonia and paramyotonia congenita. In these two inheritable muscular disorders, the muscles become enlarged, rather than weakened and atrophied, and do not relax after contracting.In most myopathies, weakness occurs primarily in the muscles of the shoulders, upper arms, thighs, and pelvis (proximal muscles). In some cases, the distal muscles of the hands and feet may be involved during the advanced stage of disease.
Other typical symptoms of muscle disease include the following:
- Aching.
- Cramping
- Pain
- Stiffness
- Tenderness\
- Tightnes
Initially, individuals may feel fatigued doing very light physical activity. Walking and climbing stairs may be difficult because of weakness in the pelvic and leg muscles that stabilize the trunk.
Patients often find it difficult to rise from a chair. As the myopathy progresses, there may be muscle wasting.
Treatment for Myopathy:
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.
Treatment for 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.
Treatment for Endocrine Myopathies:
Often, treating the underlying condition helps relieve muscle weakness and pain associated with endocrine myopathies.
Treatment for 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.
Treatment for 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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