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Found Missing
In Short
Function Junction
Plugged In
Filling in the Blanks
Food Pharmacy
Fall 1999
Battery not included
--Michael H. Brooke., M.D.
Any disturbance in the biochemical pathways that support ATP levels in the muscle inevitably results in exercise intolerance. 

One common symptom is muscle fatigue, a sense that the muscle will no longer perform in a normal fashion. This is true fatigue and not simply a feeling of tiredness or weariness.

It may be difficult for the patient to describe the fatigue in terms that the physician can understand because it has nothing to do with the sensations experienced by a healthy person after strenuous exercise. It has an unpleasant quality, and patients describe it in terms of a barrier through which they cannot break. Other symptoms include muscle pain and sometimes muscle cramps.

The normal fatigue of strenuous exercise is painless. Muscle pain following strenuous exercise, for instance the next day, is almost universal in the untrained individual, but pain during exercise is more the hallmark of disturbed muscle function.

Normally functioning muscle appears to have a series of safety mechanisms that prevent humans from exercising it to the point of destruction. In the metabolic muscle diseases, maintenance of ATP levels is impaired, and the protective mechanism that functions in the normal person is absent.

If exercise is forced in a patient with a metabolic myopathy, muscle pain develops, and a muscle contracture subsequently may be found, in which state the muscle is hard, swollen, and tender. This reflects actual destruction of the muscle. Fatigue, muscle pain, contracture, and myoglobinuria are the increasingly severe effects of the biochemical defect. 

It should be noted that a tissue in which ATP levels are depleted is probably dead tissue. Even in the metabolic myopathies, the muscle seldom reaches this critical state. What does happen, however, is that most of the support pathways are overworked with the production of unwelcome by-products which are probably responsible for the symptoms. 

Dr. Brooke is Director of the Muscle Disease Research Center at the University of Alberta in Edmonton.
--Reprinted with permission from http://plaza.v-wave.com/mbrooke/ublish/page12.html
Related links:
Matter over Mind: The Realities of a Common Muscle Disease
CPT deficiency article by Georgirene Vladutiu, Ph.D.

Running on Empty 
December 1999 article from Quest on CPT II deficiency and other metabolic myopathies.

Metabolic myopathies
Scroll through summaries of clinical findings in 11 different disorders, including CPT deficiency. 

To read Dr. Brooke's comments about CPT deficiency, visit In the long run.
 
To read more about the effects of exercise in metabolic disorders, visit When exercise cramps your style and Quick on the trigger.

  

A common complaint is that the patient feels that activity is limited because he or she just "runs out of gas." This is an apt analogy that draws attention to the fact that skeletal muscle is a sophisticated "machine" which is powered by a remarkable "engine" that is able to convert a variety of fuels to energy. 

      --Ronald Haller, M.D.
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