The are a group of
Genetic and
Hereditary Muscle diseases; characterized by progressive
Skeletal Muscle weakness, defects in muscle
Protein s, and the death of muscle
Cells and
Tissue . In some forms of muscular dystrophy,
Cardiac and
Smooth Muscle s are affected. The muscular dystrophies are the most-known
Hereditary Disease s.
The most common form is
Duchenne Muscular Dystrophy . This form is caused by
Mutation s of the gene for the
Dystrophin Protein . The dystrophin gene is the second largest gene in mammals.
The dystrophin gene is located on the
X Chromosome , thus making it a '
Sex-linked ' disorder. Accordingly, muscular dystrophies are much more common in males, as females have two copies of the X chromosome while males have only one.
There is no known cure for muscular dystrophy. Inactivity (such as bed-rest and even sitting for long periods) can worsen the disease. Physical therapy and orthopedic instruments (''e.g.,''
Wheelchair s,
Standing Frames ) may be helpful.
There is no specific treatment for any of the forms of muscular dystrophy. Physical therapy to prevent
Contractures (a condition in which shortened muscles around joints cause abnormal and sometimes painful positioning of the joints),
Orthoses (orthopedic appliances used for support) and corrective
Orthopedic Surgery may be needed to improve the quality of life in some cases. The cardiac problems that occur with Emery-Dreifuss muscular dystrophy and myotonic muscular dystrophy may require a
Pacemaker . The
Myotonia (delayed relaxation of a muscle after a strong contraction) occurring in myotonic muscular dystrophy may be treated with medications such as
Quinine ,
Phenytoin or
Mexiletine .
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