Summary
The changes in a sural nerve biopsy of a patient with porphyric neuropathy were studied by light and electron microscopy. Linear arrays of myelin ovoids constituted the most common abnormality in whole mounts of teased-fiber preparations. Round or irregular formations of variable osmiophilia were the most frequent finding in thick-section preparations examined by phase contrast microscopy. Lamellar whorls represented the most prevalent lesion in thinsections studied under the electron microscope. In addition, along the teased fibers, segmental demyelination was definitely detected, although rarely; in thick sections, the true extent of the nerve fiber loss was fully appreciated; in thin sections, a variety of axon and myelin changes of a distinct character were discovered. The studies demonstrate that in peripheral nerves of porphyric neuropathy, axonand myelin changes generally run together and proceed pari passu in the same segment of nerve fiber. Furthermore, among the pathogenetic mechanisms invoked to account for the neuropathic changes none are favored to the exclusion of others by these studies. Therefore, a primary axonaland myelinic disorder on the basis of a deranged porphyrin metabolism is as good a possibility as any hitherto advanced explanation of the pathogenesis of the neuropathic changes. The secondary lesions of Wallerian degeneration and segmental demylination may simple be grafted upon the primary lesions evoked by the metabolic abnormality.
Similar content being viewed by others
References
Blümeke, S., Niedorf, H. R.: Electron microscope studies of Schwann cells during the Wallerian degeneration with special reference to the cytoplasmic filaments. Acta neuropath. (Berl.)6, 46–60 (1966)
Blümcke, S., Niedorf, H. R., Rode, J.: Axoplasmic alterations in the proximal and distal stumps of transsected nerves. Acta neuropath. (Berl.)7, 44–61 (1966)
Cavanagh, J. B., Mellick, R. S.: On the nature of the peripheral nerve lesions associated with acute intermittent porphyria. J. Neurol. Neurosurg. Psychiat.28, 320–327 (1965)
Denny-Brown, D., Sciarra, D.: Changes in the nervous system in acute porphyria. Brain68, 1–16 (1945)
Do ié, S., Gospavié, J., Anzil, A. P., Krstić, S., Cvelković, D.: Some light and electron microscopic findings in porphyric neuropathy. In: International Congress Series No. 427 (eds. W. A. den Hartog Jager, G. W. Bruyn and A. P. J. Heijstee), pp. 216–217. Amsterdam: Excerpta Medica 1977
Dyck, P. J., Johnson, W. J., Lambert, E. H., O'Brien, P. C.: Segmental demyelination secondary to axonal degeneration in uremic neuropathy. Mayo Clin. Proc.46, 400–431 (1971)
Fardeau, M., Engel, W. K.: Ultrastructural study of a peripheral nerve biopsy in Refsun's disease. J. Neuropath. exp. Neurol.28, 278–294 (1969)
Fessel, W. J.: Fat disorders and peripheral neuropathy. Brain94, 531–540 (1971)
Gibson, J. B., Goldberg, A.: The neuropathology of acute porphyria. J. Path. Bact.71, 495–509 (1956)
Hager, H.: Allgemeine morphologische Pathologie des Nervengewebes. In: Handb. d. allg. Pathol., Bd. III/3, pp. 149–230. Berlin-Heidelberg-New York: Springer 1968
Jędrzejowska, H., Rafalowska, J., Fidziańska, A., Sawicka, E.: Morphological changes in the nervous system and muscles in acute intermittent porphyria. Neuropatol. Pol.12, 35–50 (1974)
Lapresle, J.: Neuropathologie de la porphyrie aiguë intermittente. Bull. Soc. Méd. Hop. Paris115, 1125–1134 (1964)
Ochoa, J.: Microscopic anatomy of unmyelinated nerve fibers. In: Peripheral Neuropathy (eds. P. J. Dyck, P. K. Thomas and E. H. Lambert), pp. 131–150. Philadelphia: W. B. Saunders 1975
Sandbank, U., Bechar, M., Bornstein, B.: Hyperlipemic neuropathy. Case Report: histological and electron-microscopical study. Acta neuropath. (Berl.)19, 290–300 (1971)
Schlaepfer, W. W.: Calcium-induced degeneration of axoplasm in isolated segments of rat peripheral nerve. Brain Res.69, 203–215 (1974)
Schoental, R., Cavanagh, J. B.: Mechanisms involved in the “dyingback” process—an hypothesis implicating coenzymes. Neuropathol. appl. Neurobiol.3, 145–157 (1977)
Sweeney, V. P., Pathak, M. A., Asbury, A. K.: Acute intermittent porphyria. Increased ALA-synthetase activity during an acute attack. Brain93, 369–380 (1970)
Thomas, P. K.: The morphological basis for alterations in nerve conduction in peripheral neuropathy. Proc. R. Soc. Med.64, 295–298 (1971)
Thomas, P. K., Hollinrake, K., Lascelles, R. G., O'Sullivan, D. J., Baillod, R. A., Moorhead, J. F., Mackenzie, J. C.: The polyneuropathy of chronic renal failure. Brain94, 761–780 (1971)
Thomas, P. K., Sheldon, H.: Tubular arrays derived from myelin breakdown during Wallerian degeneration of peripheral nerve. J. Cell Biol.22, 715–718 (1964)
Tschudy, D. P., Valsamis, M., Magnussen, C. R.: Acute intermittent porphyria: clinical selected research aspects. Ann. Intern. Med.83, 851–864 (1975)
Wakayama, Y., Muroga, T., Hibino, R., Matsui, T., Utsumi, M.: On the study of sural nerve biopsy in a case of acute intermittent porphyria. Clin. Neurol. (Tokyo)15, 333–339 (1975)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Anzil, A.P., Dožić, S. Peripheral nerve changes in porphyric neuropathy: Findings in a sural nerve biopsy. Acta Neuropathol 42, 121–126 (1978). https://doi.org/10.1007/BF00690977
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00690977