Benefit from alternate-day prednisone in myasthenia gravis.
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Biomedical subjects
Publications and source records attributed to W K Engel.
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The highly characteristic early and midstage histological lesions of Duchenne dystrophy were reproduced experimentally in the rat by the combination of a vascular abnormality, aortic ligation, which does not affect the structure of the intramuscular blood vessels, and the humoral vasoactive substance 5-hydroxytryptamine. Neither ligation nor injection of 5-hydroxytryptamine alone causes changes in the muscle fibers. This result establishes the possibility of a similar combined mechanism for a nonstructural ischemia pathogenesis in Duchenne dystrophy. The proposed pathogenesis is contrary to the generally held idea that the cause is an intrinsic abnormality of muscle fiber metabolism.
The histochemical reaction for phosphorylase is completely lost from anterior horn neurons rich in phosphorylase within 72 hours after proximal or distal axonal section. Using this new type of axonal reaction as a marking technique in the anterior horn of the seventh lumbar spinal cord segment of the cat, we demonstrated that (i) alpha motor neurons of slow twitch motor units, like those of fast twitch motor units, are rich in phosphorylase and poor in succinate dehydrogenase, and (ii) interneurons and Renshaw neurons are rich in succinate dehydrogenase and poor in phosphorylase. Gamma motor neurons, because of their small size, are considered to be rich in succinate dehydrogenase and poor in phosphorylase. Thus, anterior horn neurons capable of higher firing frequencies (Renshaw neurons, interneurons, and gamma motor neurons) are richer in mitochondrial oxidative enzyme activity as marked by succinate dehydrogenase. Those firing at lower frequencies (both types of alpha motor neurons) are richer in phosphorylase activity and glycogen content and, thus, apparently better equipped for anaerobic glycolysis.
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Two sibs with heredopathia atactica polyneuritiformis (Refsum's Disease) each had a relapse after ceasing to follow a special diet. On being placed on a very restricted low phytol, low phytanic acid diet both had marked subjective improvement, and marked objective improvement both in physical findings and in quantitative tests of strength, dexterity, and motor nerve conduction velocities, concomitant with a fall in serum phytanic acid levels. No relapse occurred in 22 months on this diet.
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