| Definition
- One of nine types of muscular dystrophy,
a group of genetic, degenerative diseases
primarily affecting voluntary muscles.
Cause - Mutations in the
genes that produce emerin, lamin A or lamin
C, proteins in the membrane that surrounds
the nucleus of each muscle cell.
Onset - Usually by 10 years
of age.
Symptoms - Weakness and
wasting of shoulder, upper arm and calf muscles;
joint stiffening; fainting (because of cardiac
abnormalities).
Progression - Disease usually
progresses slowly. Cardiac complications are
common and sometimes require a pacemaker.
Inheritance -Can be X-linked
recessive, primarily affecting males, who
inherit the disease through their mothers.
Another type is autosomal dominant, meaning
it can be inherited through either parent;
an autosomal recessive type occurs when a
faulty gene is inherited from each parent.
 Facts About Rare Muscular Dystrophies
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