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Muscular dystrophy (MD) is a chronic, progressive disease of the skeletal muscles. An inherited disorder, MD involves muscle wastage and associated nervous system defects.
The most common type of muscular dystrophy begins in early childhood and is first noticed by clumsiness in walking and a tendency to fall. In addition to changes in the leg muscles, there may be involvement of the pelvic and shoulder muscles, and in some cases, the heart muscle. This muscular wasting appears to involve all muscles in a random fashion. Paradoxically, the wasted muscle may enlarge due to connective tissue and fat deposits, giving a false appearance of muscle strength (pseudohypertrophy).
The best-known form, Duchenne's disease (pseudohypertropohic MD), is present in half of all cases, mostly affecting boys between three and five. Scoliosis is a common complication that can prove fatal, owing to cardio-respiratory difficulties caused by the distorted rib cage.
1. Jackson MJ, et al: Vitamin E and muscle diseases, Journal of Inherit Metab Dis, 8 suppl 1, 1985, p 84-87.