41 - Muscular dystrophy and other myopathies

Summary

The Rehabilitation needs of patients with skeletal muscle disease demand a rehabilitation treatment plan that is thoughtful, creative and unique to the needs of the individual patient.

Definition of myopathy and dystrophy

Although often used interchangeably, it is important to differentiate “myopathy” from “dystrophy”. Myopathy refers to any disorder (acquired or congenital/inherited) that can be attributed to pathological, biochemical or electrical changes occurring in muscle fibers or in the interstitial tissue of voluntary musculature, and in which there is no evidence that such changes are due to nervous system dysfunction (Walton and Gardner-Medwin, 1974). The term myopathy (other than the rapidly progressive dystrophies or polymyositis) usually implies a slowly progressive or non-progressive disorder of muscle function. Dystrophy, on the other hand refers to a congenital or inherited disorder of muscle characterized by progressive degeneration of skeletal muscle fibers with weakness and atrophy resulting from a rate of degeneration which outpaces regeneration. The dystrophic process eventually leads to connective tissue replacement of muscle fibers (Harper, 2002). Many of the dystrophies have associated features that are not a result of the muscle weakness. An example of this is Myotonic Dystrophy, which is a systemic disorder affecting multiple organ systems. Dystrophies are genetically transmitted and to date have no known cure. However, research developments continue and rehabilitation intervention resources are vast.

Classifications of muscle diseases

Various classification systems have been used to categorize the large number of known muscle diseases. Examples of classification systems are by age of onset, inheritance pattern, physiological process, severity, and rate of progression.