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The Gower Sign, Landouzy-Dejerine Disease, Steinert Disease
Fundraising telethons and vague images of disabled children loom in most parents' minds when they hear the words "muscular dystrophy." The truth is that rapid advances in molecular genetic engineering hold promise for children with muscular dystrophy. Support of these efforts makes good sense.
The muscular dystrophies are a group of illnesses that share several things in common. They are all, first and foremost, muscle diseases as opposed to brain or nerve diseases. They all involve the death of individual muscle fibers. They are all hereditary conditions. And they all progress over time (as opposed to cerebral palsy, which doesn't get worse or better).
Duchenne muscular dystrophy is the most common of the muscular dystrophies. It is an X-linked recessive condition, so it occurs in boys whose mothers are carriers. It appears around the world in all ethnic groups. Becker muscular dystrophy is a milder form of the same condition.
Emery-Dreifuss muscular dystrophy is a very rare form of muscular dystrophy that is also an X-linked recessive condition, with affected boys born to mothers who are carriers.
Myotonic muscular dystrophy (Steinert disease) is the second most common type. It is found most often in North America, Europe, and Australia. It is an autosomal dominant condition, which means that when one of the parents has the condition, about half of the children will (both boys and girls). There are no carriers without the illness.
Facioscapulohumeral muscular dystrophy (Landouzy-Dejerine disease) is a very rare form of muscular dystrophy that is also an autosomal dominant condition. When a parent has the condition, about half of the children will. There are no carriers without the illness.
Congenital muscular dystrophy and limb-girdle muscular dystrophy are autosomal recessive conditions. If both of the parents are carriers, about half of the children will be carriers, and about one-fourth of the children will have the disease. If only one parent is a carrier, the disease is not passed on, but about one-fourth of the children will be carriers.
The Fukuyama type of congenital muscular dystrophy is especially common in Japan, but is also found in Europe and the Middle East.