Summary
Biopsy study by ultrastructural methods of sural nerve from two patients with chronic hypertrophic neuropathy showed giant vacuolation of endoneurial fibroblasts. These fibroblast changes have been described previously only in two sisters with hereditary sensory neuropathy (Schoeneet al.). The pathological background in which giant vacuolation of fibroblasts occurs is in chronic neuropathies with severe nerve fiber loss and prominent looselyarranged interstitial spaces containing amorphous substance of moderate electron density. The histochemical staining reactions of the amorphous interstitial substance are variable and of uncertain usefulness.
It is tentatively concluded that giant vacuolation of endoneurial fibroblasts is not a disease-specific reaction, but accompanies those chronic, scarring disease processes of nerve that result in extreme nerve fiber loss and an enlarged loose-textured myxoid interstitium.
Similar content being viewed by others
References
Ballin, R. H., Thomas, P. K.: Hypertrophic changes in diabetic neuropathy. Acta neuropath. (Berl.)11, 93 (1968).
Boveri, P.: De la névrite hypertrophique familiale. Sem. méd. (Paris)30, 145–150 (1920).
Cammermeyer, J.: Neuropathological changes in hereditary neuropathies: Manifestations of the syndrome heredopathia atactica polyneuritiformis in the presence of interstitial hypertrophic polyneuropathy. J. Neuropath. exp. Neurol.15, 340–361 (1956).
Dyck, P. J., Gomez, M. R.: Segmental demyelinization in Dejerine-Sottas disease: light, phase, and electron microscopic studies. Proc. Mayo Clin.43, 280–296 (1968).
—, Lambert, E. H.: Lower motor and primary sensory neuron diseases with peroneal muscular atrophy. I. Neurologic, genetic, and electrophysiologic findings in hereditary polyneuropathies. Arch. Neurol. (Chic.)18, 603–618 (1968).
Green, L. N., Herzog, I., Aberfeld, D.: A case of hypertrophic interstitial neuritis coexisting with dementia and cerebellar degeneration. J. Neuropath. exp. Neurol.24, 682–689 (1965).
Greenfield, J. G.: In: Neuropathology, Chapt. 12, p. 608. London: Edw. Arold Ltd. 1958.
Krücke, W.: Die mucoide Degeneration der peripheren Nerven. Virchows Arch. path. Anat.304, 442–463 (1939).
Schoene, W. C., Asbury, A. K., Åstrøm, K. E., Masters, R.: Hereditary sensory neuropathy. A clinical and ultrastructural study. J. neurol. Sci.11, 463–487 (1970).
Thomas, P. K., Lascelles, R. G.: Hypertrophic neuropathy. Quart. J. Med.36, 223–238 (1967)
Webster, H. de F., Schröder, J. M., Asbury, A. K., Adams, R. D.: The role of Schwann cells in the formation of “onion bulbs” found in chronic neuropathies. J. Neuropath. exp. Neurol.26, 276–299 (1967).
Weller, R. L.: An electron microscopic study of hypertrophic neuropathy of Dejerine and Sottas. J. Neurol. Neurosurg. Psychiat.30, 111–125 (1967).
Zacks, S. I., Lipshutz, H., Elliott, F.: Histochemical and electron microscopic observations on “Onion Bulb” formations in a case of hypertrophic neuritis of 25 years duration with onset in childhood. Acta neuropath. (Berl.)11, 157–173 (1968).
Author information
Authors and Affiliations
Additional information
Supported in part by USPHS research grant NS-09359.
Rights and permissions
About this article
Cite this article
Asbury, A.K., Cox, S.C. & Baringer, J.R. The significance of giant vacuolation of endoneurial fibroblasts. Acta Neuropathol 18, 123–131 (1971). https://doi.org/10.1007/BF00687600
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00687600