Skip to main content
Log in

Ultrastructural study of a muscle biopsy from a patient with subacute myelo-optic neuropathy

  • Original Investigations
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Summary

A man, aged 46, who had been taking Clioquinol in high doses for a long period, developed a characteristic neurological syndrome of subacute myelo-optic neuropathy rather abruptly. Electron microscopical examination of the muscle biopsy, obtained five months after the onset of the disease, revealed severe degenerative changes of the presynaptic nerve endings and some unique paracrystalline inclusions in the sole plate region.

The latter may represent the morphological expression of the toxic agent which is held responsible for the subacute myelo-optic neuropathy.

Zusammenfassung

Ein 46jähriger Mann, welcher über lange Zeit hohe Dosen von Clioquinol einnahm, entwickelte recht plötzlich charakteristische neurologische Ausfälle, entsprechend einer subakuten myelooptischen Neuropathie (SMON). Bei der elektronenoptischen Untersuchung einer Muskelbiopsie, die 5 Monate nach Beginn der Erkrankung entnommen worden war, zeigten sich ausgeprägte degenerative Veränderungen der präsynaptischen Nervenendigungen und vereinzelte parakristalline Einschlüsse in der Endplattenregion, die einmalig erscheinen. Letzteres könnte das morphologische Substrat der toxischen Substanz darstellen, die für den SMON verantwortlich gemacht wird.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Benke, B., Szendröi, M.: Myopathy with mitochondrial inclusions. Virchows Arch. Abt. B (Zellpathol.) 9, 145–152 (1971)

    Google Scholar 

  2. Bergman, R. A., Jones, R. J.: Ultrastructural alterations in muscle from patients with myasthenia gravis and Eaton-Lambert syndrome. Ann. N.Y. Acad. Sci. 183, 88–122 (1971)

    Google Scholar 

  3. Dalton, A. J., Haguenau, F. (Eds.): Ultrastructure of animal virusses and bacteriophages—An Atlas. New York: Acad. Press 1973

    Google Scholar 

  4. De Crousaz, G., Voumard, Ch., De Werra, P.: A nephropathy in the genesis of S.M.O.N. Lancet 1973 I, 378–379

    Google Scholar 

  5. Engel, A. G.: Evolution and content of vacuoles in primary hypokaliemic periodic paralysis. Proc. Staff. Meet. Mayo Clin. 45, 774–814 (1970)

    Google Scholar 

  6. Gonzenbach, H. R., Waser, P. G.: Electron microscopic studies of degeneration and regeneration of rat neuromuscular junctions. Brain Res. 63, 167–174 (1973)

    Google Scholar 

  7. Hudgson, P., Bradley, W. G., Jenkison, M.: Familial mitochondrial myopathy. Part I. Clinical, electrophysiological and pathological findings. J. neurol. Sci. 16, 343–370 (1972)

    Google Scholar 

  8. Igata, A.: Halogenated oxyquinoleine derivates and neurological syndromes. Lancet 1971 II, 42–43

    Google Scholar 

  9. Inoue, Y. K.: The etiology of S.M.O.N. Lancet 1971 II, 869–870

    Google Scholar 

  10. Inoue, Y. K.: Avian infectious laryngotracheitis and S.M.O.N. in Japan. Lancet 1973 I, 1009

    Google Scholar 

  11. Inoue, Y. K.: An avian related new Herpes virus infection in man—Subacute myelo-opticoneuropathy (S.M.O.N.). Progr. med. Virol. 21, 35–42 (1975)

    Google Scholar 

  12. Inoue, Y. K., Nishibe, Y.: Serological relationship between S.M.O.N. and avian infectious laryngotracheitis virus. Lancet 1973 I, 776–777

    Google Scholar 

  13. Inoue, Y. K., Nishibe, Y., Kimura, T., Shimada, Y., Yamada, G.: Antibody response in cases of S.M.O.N. against the associated virus. Lancet 1972 II, 705–706

    Google Scholar 

  14. Inoue, Y. K., Nishibe, Y., Nakamura, Y.: Virus associated with S.M.O.N. in Japan. Lancet 1971 I, 853–854

    Google Scholar 

  15. Jones, E. L., Searle, C. E., Thomas Smith, W.: Peripheral neuropathy in ageing rats fed Clioquinol and a maize diet. Acta neuropath. (Berl.) 24, 256–262 (1973)

    Google Scholar 

  16. Kimura, S., Yoshida, N.: Inoue virus and neuropathy. Lancet 1972 II, 233

    Google Scholar 

  17. Lapresle, J., Fardeau, M., Godet-Guillain, J.: Myopathie distale et congenitale avec hypertrophie des mollets. J. neurol. Sci. 17, 87–102 (1972)

    Google Scholar 

  18. Mair, W. G. P., Tomé, F. M. S.: Atlas of the ultrastructure of diseased human muscle. Edinburgh & London: Churchill Livingstone 1972

    Google Scholar 

  19. Mamoli, B., Thaler, A., Heilig, P., Siakos, G.: Subacute myelo-optico-Neuropathie nach Clioquinolmedication. J. Neurol. 209, 139–147 (1975)

    Google Scholar 

  20. Mashiko, N.: Crystalline structures in mitochondria of human dystrofic muscle. In: Basic Research in Myology, 512–516. Proc. 2nd. Int. Congr. Muscle Diseases, Perth, Australia 1971, Part I. Amsterdam: Exc. Med. 1973

    Google Scholar 

  21. Mastaglia, F. L.: Pathological changes in skeletal muscle in acromegaly. Acta neuropath. (Berl.) 24, 273–286 (1973)

    Google Scholar 

  22. McDonald, R. D., Rewcastle, H. B., Humphry, J. G.: The myopathy of hyperkaliemic periodic paralysis. An electron microscopic study. Arch. Neurol. (Chic.) 19, 274–283 (1968)

    Google Scholar 

  23. Miledi, E., Slater, G. R.: On the degeneration of rat neuromuscular junctions after nerve section. J. Physiol. (Lond.) 207, 507–528 (1970)

    Google Scholar 

  24. Miyakawa, T., Sumiyoshi, S., Murayama, E., Deshimaru, M., Ishikawa, H., Miyakawa, K., Tatetsu, S.: Ultrastructural study of a nerve and muscle biopsy from a case of “subacute myelo-optico-neuropathy”. Acta neuropath. (Berl.) 16, 17–24 (1970)

    Google Scholar 

  25. Miyakawa, T., Sumiyoshi, S., Murayama, E., Deshimaru, M., Goto, K., Tatetsu, S.: Electron microscopical study of a nerve biopsy from a case in the chronic state of “Subacute Myelo-Optico Neuropathy”. Acta neuropath. (Berl.) 19, 323–330 (1971)

    Google Scholar 

  26. Nakae, K., Yamamoto, S., Igata, A.: Subacute myelo-optic neuropathy in Japan. Lancet 1971 II, 510–512

    Google Scholar 

  27. Nakae, K., Yamamotoi, S., Shigematsu, I., Kono, K. R.: Relation between subacute myelo-optic neuropathy (S.M.O.N.) and Clioquinol: nation wide survey. Lancet 1973 I, 171–173

    Google Scholar 

  28. Nakamura, Y., Inoue, Y. K.: Pathogenicity of virus associated with subacute myelo-optico-neuropathy. Lancet 1972 I, 223–226

    Google Scholar 

  29. Nickel, E., Waser, P. G.: Electronenmikroskopische Untersuchungen am Diaphragma der Maus nach einseitiger Phrenicotomie. I. Die degenerierende motorische Endplatte. Z. Zellforsch. 88, 278–296 (1968)

    Google Scholar 

  30. Ogata, M., Watanabe, S., Tateishi, J., Kuroda, S., Otsuki, S.: Distribution of radioactive clioquinol in animals. Lancet (7814), 1973 I, 1248

    Google Scholar 

  31. Ogata, M., Watanabe, S., Tateishi, J., Kuroda, S., Otsuki, S.: Accumulation of Clioquinol in mice. Lancet 1973 I, 1325

    Google Scholar 

  32. Pachter, B. R., Davidowitz, J., Breinin, G. M.: Muscle fibers and motor end-plate involvement in the extraocular muscles of the myotonic mouse. Invest. Ophtal. 14, 418–427 (1975)

    Google Scholar 

  33. Pannekoek, J. H.: Neurotoxische verschijnselen na Clioquinol (Enterovioform). Ned. T. Geneesk. 32, 1611–1615 (1972)

    Google Scholar 

  34. Pittman, F. E., Westphal, M.: SMON and inflammation of the colon. Lancet 1973 II, 566–567

    Google Scholar 

  35. Schröder, J. M., Becker, P. E.: Anomalien des T-systems und des sarkoplasmatischen Reticulums bei der Myotonie, Paramyotonie und Adynamie. Virchows Arch. Abt. A. Path. Anat. 357, 319–344 (1972)

    Google Scholar 

  36. Schrodt, G. R., Walker, S. M.: Ultrastructure of membranes in denervation Atrophy. Am. J. Path. 49, 33–51 (1966)

    Google Scholar 

  37. Sobue, I., Ando, K., Jida, M., Takayanagi, Y., Yamamura, Y., Matsuoka, Y.: Myeloneuropathy with abdominal disorders in Japan. A clinical study of 725 cases. Neurol. (Minneap.) 21, 168–173 (1971)

    Google Scholar 

  38. Sobue, I., Mukoyama, M., Takayanagi, T., Nishigaki, S., Matsuoka, Y., Ando, K.: Myeloneuropathy with abdominal disorders in Japan. Neuropathological findings in seven autopsied cases. Neurol. (Minneap.) 22, 1034–1039 (1972)

    Google Scholar 

  39. Tateishi, J., Kuroda, S., Saito, A., Otsuki, S.: Myelo-optico neuropathy induced by Clioquinol in animals. Lancet 1971 I, 1263–1264

    Google Scholar 

  40. Tateishi, J., Kuroda, S., Saito, A., Otsuki, S.: Experimental myelo-optic neuropathy induced by Clioquinol. Acta neuropath. (Berl.) 24, 304–320 (1973)

    Google Scholar 

  41. Tsujihata, M., Engel, A. G., Lambert, E. H.: Motor end-plate fine structure in acrylamide dying-back neuropathy: A sequential morphometric study. Neurol. (Minneap.) 24, 849–856 (1974)

    Google Scholar 

  42. Tsubaki, T., Honma, Y., Hoshi, M.: Neurological syndrome associated with Clioquinol. Lancet 1971 I, 896–897

    Google Scholar 

  43. Watanabe, S., Tateishi, J., Kuroda, S., Otsuki, S., Ogata, M.: Distribution of Clioquinol in rats with hepatic dysfunction. Lancet 1973 II, 681–682

    Google Scholar 

  44. Yarom, R., Chaco, J., Steibuegel, D.: Ultrastructure of muscle in Stiff-man syndrome. Virchows Arch. Abt. A. Path. and Histol. 362, 207–221 (1974)

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Otte, G., de Coster, W., Thiery, E. et al. Ultrastructural study of a muscle biopsy from a patient with subacute myelo-optic neuropathy. J. Neurol. 215, 91–102 (1977). https://doi.org/10.1007/BF00313056

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00313056

Key words

Navigation