An electron microscopic study of hypertrophic neuropathy of Dejerine and Sottas.

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Abstract
Electron microscopic Observations of the sural nerves from 3 cases of hypertrophic neuropathy are presented. The cases differ in that one is a young child severly affected from birth with no definite family history; the 2nd is a man of 61 years with no family history; and the 3rd is a truly familial type similar to that described by Dejerine and Suttas. In all three cases typical "onion-bulb" whorl were composed of overlapping Schwann or Remak cell processes arranged in concentric layers around a central axon. The layers were separated by endoneurial collagen and the central axon was usually surrounded by a layer of Schwann cytoplasm and often by a thin myelin sheath. Very few myelin sheaths of normal thickness were found. Evidence of active demyelina-tion and remyelination was present. In view of the fact that the same cellular picture is encountered in 3 divergent conditions, the changes of hypertrophic neuropathy should be regarded as a non-specific reaction of Schwann cells to several agencies and not as an isolated disease process.