Summary
Distinctive cytoplasmic alterations of Schwann cells were observed by electron microscopy in rats and mice with peripheral neuropathy induced by chronic exposure to 2,5-hexanedione. Pronounced enlargement of Schwann cells was due to accumulation of 100 Å cytoplasmic filaments and endoplasmic reticulum and was most often observed after 12–15 weeks exposure to 2,5-hexanedione. Examination of teased nerve fibres revealed segmental demyelination and remyelination involving axons of normal diameter as well as giant axons. The filament disorder induced by 2,5-hexanedione administration is not limited solely to axoplasmic contents. Possible mechanisms of demyelination are discussed and the changes are compared to those observed in human neuropathy for which 2,5-hexanedione appears to be the closest experimental model.
Similar content being viewed by others
References
Asbury, A. K., Gale, M. K., Cox, S. C., Baringer, J. R., andBerg, B. O. (1972) Giant axonal neuropathy — a unique case with segmentai neurofilamentous masses.Acts Neuro- pathologica (Berlin)20, 237–47.
Berg, B. O., Rosenberg, S., andAsbury, A. A. (1972) Giant axonal neuropathy.Pediatrics 49, 894–9.
Boltshauser, E., Bischoff, A. andIsler, W. (1977) Giant axonal neuropathy. Report of a case with normal hair.Journal of Neurological Science 13, 269–78.
Duckett, S., Williams, N., andFrancis, S. (1974) Peripheral neuropathy associated with the inhalation of methyln-butyl ketone.Experientia 30, 1283–4.
Dyck, P. J., Johnson, W. J., Lambert, E. H., andO'brien, P. C. (1971) Segmental demyelination secondary to axonal degeneration in uremie neuropathy.Mayo Clinic Proceedings 46, 400–31.
Friede, R. L. andMartinez, A. J. (1970a) Analysis of the process of sheath expansion in swollen nerve fibers.Brain Research 19, 165–82.
Friede, R. L. andMartinez, A. J. (1970b) Analysis of axon-sheath relations during early Wallerian degeneration.Brain Research 19, 199–212.
Friede, R. L. andSamorajski, T. (1967) Relation between the number of myelin lamellae and axon circumference in fibers of vagus and sciatic nerves of mice.Journal of Comparative Neurology 30; 223–31.
Gandolfi, A. J., Nakaue, H. S. andBuhler, D. R. (1974) Effect of hexachlorophene on hepatic drug-metabolizing enzymes in the rat.Biochemical Pharmacology 23, 1997–2003.
Hirano, A. (1977) Fine structural changes in the mutant hamster with hind leg paralysis.Acta Neuropathologica (Berlin)39, 225–30.
Jedrzejowska, H. andDrac, H. (1977) Infantile chronic peripheral neuropathy with giant axons.Acta Neuropath ologica (Berlin)37, 213–7.
Koch, T., Schultz, P., Williams, R. andLampert, P. (1977) Giant axonal neuropathy: a childhood disorder of microfilaments.Annals of Neurology 1, 438–51.
Mendell, J. R., Saida, K., Ganansia, M. F., Jackson, D. B., Weiss, H. S., Gardier, R., Christman, C., Allen, N., Couri, D., O'Neill, J., Marks, B. andHetland, L. (1974) Toxic polyneuropathy produced by methyl n-butyl ketone.Science 195, 787–9.
Powell, H. C. andLampert P. W. (1975) Effects of hexachlorophene on the central and peripheral nervous systems.Proceedings of the VII International Congress on Neuropathy pp. 377–382, (Budapest, Hungary 1974). Amsterdam: Excerpta Medica.
Prineas, J. W., Ouvrier, R. A., Wright, R. G., Walsh, J. C. andMcLeod, J. G. (1976) Giant axonal neuropathy—a generalized disorder of cytoplasmic microfilament formation.Journal of Neuropathology and Experimental Neurology 34, 458–70.
Reich, F. (1903) Über eine neue Granulation in den Nervenzellen.Archiv für Physiologie (Leipzig)27, 208–14.
Saida, K., Mendell, J. R. andWeiss, H. S. (1976) Peripheral nerve changes induced by methyl n-butyl ketone and potentiation by methyl ethyl ketone.Journal of Neuropathology and Experimental Neurology 35, 207–25.
Schaumburg, H. H. andSpencer, P. S. (1976) Degeneration in central and peripheral nervous systems produced by pure n-Hexane: an experimental study.Brain 99, 183–92.
Schröder, J. M. (1970) Zur pathogenese der Isoniazid-Neuropathie I. Eine feinstrukturelle Differenzierung gegen über der Wallerschen Degeneration.Acta Neuropathologica (Berlin)16, 301–23.
Spencer, P. S. andSchaumburg, H. H. (1975) Nervous system dying back produced by 2,5 hexanedione.Transactions of American Neurological Association 100, 148–51.
Spencer, P. S. andSchaumburg, H. H. (1976) Central-peripheral distal axonopathy—the pathology of the dying back polyneuropathies. InProgress in Neuropathology Vol. III (edited byZimmerman, H. M.), pp. 253–95. New York: Grune and Stratton.
Spencer, P. S. andSchaumburg, H. H. (1977) Ultrastructural studies of the dying-back process. II. The evolution of experimental peripheral giant axonal disease.Journal of Neuropathology and Experimental Neurology 36, 276–99.
Spencer, P. S., Schaumburg, H. H., Raleigh, R. L. andTerhaar, C. J. (1975) Nervous system degeneration produced by the industrial solvent methyl n-butyl ketone.Archives of Neurology 32, 219–22.
Spencer, S. P., andThomas, P. K. (1974) Ultrastructural studies of the dying-back process. II. The sequestration and removal by Schwann cells and oligodendrocytes of organelles from normal and diseased axons.Journal of Neurocytology 3, 763–83.
Thomas, P. K. andEliasson, S. G. (1975) Diabetic neuropathy. InPeripheral Neuropathy (edited byDyck, P. J., Thomas, P. D. andLambert, E. H.), pp. 956–81. Philadelphia: Saunders.
Wit, J. andGenderen H. (1962) Some aspects of the fate of hexachlorophene in rabbits, rats and dairy cattle.Acta Physiologica Pharmacologica Neerlandia 11, 123–32.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Powell, H.C., Koch, T., Garrett, R. et al. Schwanm cell abnormalities in 2, 5-hexanedione neuropathy. J Neurocytol 7, 517–528 (1978). https://doi.org/10.1007/BF01173995
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01173995