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Superficial anterior muscles
Superficial anterior muscles
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Limb-girdle muscular dystrophies

Definition:
Limb-girdle muscular dystrophies include at least 10 different inherited disorders that initially affect the muscles around the shoulder girdle and the hips. These diseases are progressive and may involve other muscles over a period of time.

Alternative Names:
Muscular dystrophy - limb-girdle type

Causes, incidence, and risk factors:

This is a large group of genetic diseases featuring muscle weakness and wasting (muscular dystrophy). Most are inherited in an autosomal recessive manner (both parents must have the defective gene for a child to inherit it), but some are autosomal dominant (only one parent needs to carry the bad gene to affect the child). For some of these conditions, the defective gene has been discovered, but for others, the gene is not yet known.

Typically, onset of pelvic muscle weakness (difficulty standing from a sitting position without using arms, difficulty climbing stairs) starts in childhood to young adulthood. Later there is the onset of shoulder weakness with progression to significant loss of mobility or wheelchair dependence over the next 20-30 years.

An important risk factor is having a family member with muscular dystrophy.



Review Date: 11/12/2002
Reviewed By: David G. Brooks, M.D., Ph.D., Division of Medical Genetics, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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