Résumé :
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Electron microscopy of a nerve biopsy in a case of Refsum's disease confirmed Cammermeyer's light-microscopic description of a hypertrophic neuropathy with onion-bulb-like whorls. In this disease, as in Déjerine-Sottas disease, the Schwann cells played the fondamental role in the composition of the whorls. On the basis of this morphological pattern, Déjerine-Sottas disease and Refsum's disease appear to be closely related, even if the onionbulb formation is not in the strictest sense a specific lesion. The ultrastructural studies showed that myelin and axon degradation were minor and thought secondary to the Schwann cell changes. Also demonstrated in the Schwann cell cytoplasm were frequent, non-specific lipoid deposits and unusual, presumably intramitochondrial, crystalline-like inclusions.
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