Skip to main content
SpringerLink
Log in

Ultrastructure of the optic nerve in Schilder's disease, Devic's Disease and Disseminated Sclerosis

  • Original Investigations
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

Summary

The ultrastructure of the optic nerve is described and compared in:

a)Schilder's Disease,

b)Devic's Disease,

c)Disseminated Sclerosis.

  1. a)

    Schilder's Disease. Parts of the nerve were demyelinated, sometimes extensively so and from these regions the oligodendrocytes had disappeared and there was proliferation of astrocytes. Parallel bundles of fibres with a “railway line” formation, occurred in the cytoplasm of the astrocytes. Phagocytes infiltrated the damaged nerve bundles and the fibrous septa between them. Osmiophilic particles occurred in the astrocytes, capillary endothelial cells and in the phagocytes. The collagen fibres of some septa were widely separated presumably by fluid.

  2. b)

    Devic's Disease. This condition occurred in a patient with active pulmonary tuberculosis. The nerve was extensively demyelinated and showed absence of oligodendrocytes, proliferation of astrocytes and infiltration by macrophages. Some astrocytes possessed Rosenthal fibres. Intranuclear inclusions occurred in the astrocytes and electron dense cytoplasmic inclusions in the capillary endothelial cells and the macrophages.

  3. c)

    Disseminated Sclerosis. Parts of the nerve were partially and other parts completely demyelinated. Oligodendrocytes were absent from the completely demyelinated zones but were present in the partially demyelinated zones. In and around the demyelinated regions, there was proliferation of astrocytes and they frequently exhibited the “railway line” patterns in their cytoplasm. Phagocytes were frequent amongst the degenerating myelin and the proliferating astrocytes and also in the fibrous septa. Some macrophages presented intranuclear inclusions having a “corn on the cob” appearance.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Anderson, D. R.: Ultrastructure of meningeal sheaths: Normal human and monkey optic nerves. Arch. Ophthal.82, 659–674 (1969a)

    Google Scholar 

  • Anderson, D. R.: Ultrastructure of human and monkey lamina cribrosa and optic nerve head. Arch. Ophthal.82, 800–814 (1969b)

    Google Scholar 

  • Anderson, D. R.: Ultrastructure of the optic nerve head. Arch. Ophthal.83, 63–73 (1970)

    Google Scholar 

  • Anderson, D. R., Hoyt, W. F.: Ultrastructure of the intraorbital portion of human and monkey optic nerve. Arch. Ophthal.82, 506–530 (1969)

    Google Scholar 

  • Anderson, D. R., Hoyt, W. F., Hogan, M. J.: The fine structure of the astroglia in the human optic nerve and optic nerve head. Trans. Amer. ophthal. Soc.65, 275–305 (1967)

    Google Scholar 

  • Andrews, J. M.: The ultrastructural neuropathology of multiple sclerosis. Ucla forum in medical science 16, multiple sclerosis, immunology virology and ultrastructure. New York: Academic Press 1972

    Google Scholar 

  • Andrews, J. M., Andrews, R. L.: The significance of dense core particles in subacute demyelinating disease in an adult. Lab. Invest.28, 236–243 (1973)

    Google Scholar 

  • Blinzinger, K.: Bildung von markscheidenähnlichen Spiralstrukturen durch lamelläre Astrocytenausläufer innerhalb subpialer und perivasculäre Gliose des Säugetiergehirns. Acta neuropath. (Berl.)12, 98–102 (1969)

    Google Scholar 

  • Bouteille, M., Guazzi, G. C., Masselin, S., Houdart, R., Delarue, J.: Particules d'aspect viral observées au microscope électronique dans une biopsie cérébrale d'encephalite péri-axile diffuse de Schilder. Presse méd.74, 3253–3254 (1966)

    Google Scholar 

  • Bouteille, M., Guazzi, G. C., Martin, J. J., Masselin, S., Houdart, R., Delarue, J.: Un cas d'encéphalite péri-axile diffuse de Schilder: étude ultrastructurale. Ann. Anat. path.13, 43–54 (1968)

    Google Scholar 

  • Bouteille, M., Kalifat, S. R., Delarue, J.: Ultrastructural variations of nuclear bodies in human diseases. J. Ultrastruct. Res.19, 474–486 (1967)

    Google Scholar 

  • Cohen, A. I.: Ultrastructural aspects of the human optic nerve. Invest. Ophthal.6, 294–308 (1967)

    Google Scholar 

  • Davis, R. L.: Intranuclear structures in acute multiple sclerosis. J. Neuropath. exp. Neurol.32, 178 (1973)

    Google Scholar 

  • Devic, M. E.: Myélite aigue dorso-lombaire avec névrite optique. Congres Franc. med.1, 434 (1894)

    Google Scholar 

  • Field, E. J.: Role of viral infection and autoimmunity of aetiology and pathogenesis of multiple sclerosis. Lancet.1973I, 295–297

  • Field, E. J., Cowshall, S., Narang, H. K., Bell, T. M.: Virus in multiple sclerosis. Lancet1972II 280–281

  • Field, E. J., Raine, C. S.: Examination of multiple sclerosis biopsy specimen. In: Third European Regional Congress on Electron Microscopy, Vol. 2, p. 189. Prague: Publishing house of the Czechoslovak acad. Sci. 1964

    Google Scholar 

  • Fine, S., Yanoff, M.: Occular histology, pp. 234–247. New York-London: Harper, Row 1972

    Google Scholar 

  • Gessaga, E. C., Mair, W. G. P., Grant, D. N.: Ultrastructure of sacrococcygeal chordoma. Acta neuropath. (Berl.)25, 27–35 (1973)

    Google Scholar 

  • Gonatas, N. K., Martin, J., Evangelista, I.: The osmiophilic particles of astrocytes. Virus, lipid droplets or products of sectretion. J. Neuropath. exp. Neurol.26, 369–376 (1967)

    Google Scholar 

  • Herndon, R. M., Rubinstein, L. J., Freeman, J. M., Mathieson, G.: Light and electron microscopic observation on Rosenthal fibres in Alexander's disease and in multiple sclerosis. J. Neuropath. exp. Neurol.29, 524–551 (1970)

    Google Scholar 

  • Hogan, M. J., Alvarada, J. A., Weddel, J. E.: Histology of the human eye, pp. 523–606. Philadelphia-London: W. B. Saunders Comp. 1971

    Google Scholar 

  • Hogan, E. L., Joseph, K. C., Hurt, J. P., Krigman, M. R.: Schilder's diffuse sclerosis: a biochemical and ultrastructural study of myelinoclastic demyelination. Acta neuropath. (Berl.)20, 85–95 (1972)

    Google Scholar 

  • Hughes, R. A. C., Mair, W. G. P.: In preparation

  • Iwasaki, Y., Koprowski, H., Müller, D., Ter Meulen, V., Käckell, Y. M.: Morphogenesis and structure of a virus in cells cultured from brain tissue from two cases of multiple sclerosis. Lab. Invest.28, 494–500 (1973)

    Google Scholar 

  • Narang, H. K., Field, E. J.: Paramyxovirus like tubules in multiple sclerosis biopsy material. Acta neuropath. (Berl.)25, 281–290 (1973a)

    Google Scholar 

  • Narang, H. K., Field, E. J.: An electron microscopic study of multiple sclerosis biopsy material: some unusual inclusions. J. neurol. Sci.18, 287–300 (1973b)

    Google Scholar 

  • Nelson, E., Osterberg, K., Blaw, M., Story, J., Kozak, I.: Electron microscopic and histochemical studies in diffuse sclerosis (sudanophilic type). Neurology (Minneap.)12, 896–909 (1962)

    Google Scholar 

  • Popoff, N., Stewart, S.: The fine structure of nuclear inclusions in the brain of experimental golden hamsters. J. Ultrastruct. Res.23, 347–361 (1968)

    Google Scholar 

  • Prineas, J.: Paramyxovirus like particles associated with acute demyelination in chronic relapsing multiple sclerosis. Science178, 760–763 (1972)

    Google Scholar 

  • Raine, C. S., Field, E. J.: Nuclear structure in nerve cells in multiple sclerosis. Brain Res.10, 266–268 (1968)

    Google Scholar 

  • Rinne, U. K., Arstilla, A. U., Riekkinen, P. J.: Electron microscopic study on nuclear changes in multiple sclerosis brain biopsies. Acta neurol. scand.48, 529–537 (1972)

    Google Scholar 

  • Schilder, P.: Zur Kenntnis der sogenannten diffusen Sklerose. Über encephalitis peraxialis diffusa. Z. ges. Neurol. Psychiat.10, 1–60 (1912)

    Google Scholar 

  • Schlote, W.: „Fasern” und Spongioblasten im Zentralnervensystem. 11 Elektronenmikroskopische Untersuchungen. Bedeutung der Rosenthalschen „Fasern”. Beitr. path. Anat.133, 461–480 (1966)

    Google Scholar 

  • Stroud, R. S., Ceballos, R.: Neuromyelitis optica: a case report with reappraisal of its relation to multiple sclerosis. Ala. J. med. Sci.8, 288–296 (1971)

    Google Scholar 

  • Suzuki, K., Grover, W. D.: Ultrastructural and biochemical studies of Schilder's disease. I. Ultrastructure. J. Neuropath. exp. Neurol.29, 392–404 (1970)

    Google Scholar 

  • Ter Meulen, V., Koprowski, H., Iwasaki, Y., Käckell, Y. M., Müller, D.: Fusion of cultured multiple sclerosis brain cells with indicator cells: presence of nucleocapsids and virions and isolation of parainfluenza-type virus. Lancet1972II, 1–5

  • Wantanabe, I., Okazaki, H.: Virus-like structures in multiple sclerosis. Lancet1973II, 566–570

  • Yamamoto, T.: Electron microscopic observations of the human optic nerve. 1: the fine structure of the adjoining part of the eye ball folia. Ophthal. Jap.16, 161–167 (1965)

    Google Scholar 

  • Yamamoto, T.: Electron microscopic observation of human optic nerves. Jap. J. Ophthal.10, 40–53 (1966)

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. The Department of Neuropathology, Institute of Neurology, National Hospital, Queen Square, WC1N 3BG, London, England

    J. de Preux & W. G. P. Mair

Authors
  1. J. de Preux
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. W. G. P. Mair
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

de Preux, J., Mair, W.G.P. Ultrastructure of the optic nerve in Schilder's disease, Devic's Disease and Disseminated Sclerosis. Acta Neuropathol 30, 225–242 (1974). https://doi.org/10.1007/BF00688923

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00688923

Key words

Search

Navigation