Acta Neuropathologica

, Volume 42, Issue 2, pp 81–86 | Cite as

Ultrastructure of the Bunina bodies in anterior horn cells of amyotrophic lateral sclerosis

  • M. Tomonaga
  • M. Saito
  • M. Yoshimura
  • H. Shimada
  • H. Tohgi
Original Investigations

Summary

Light and electron microscopic studies were made on the anterior horn cells in a case of amyotrophic lateral sclerosis. Eosinophilic inclusions of Bunina type were observed almost selectively in the motor neurons of spinal cord, as well as of brain stem, at the light microscopic level. Fine structural study revealed the presence of two types of cytoplasmic inclusions. The first, mainly corresponding to the light microscopic inclusions, were homogeneous, electron-dense, round- or oval-shaped bodies with vesicular or tubular rims and ribosome particles, about 2–5 μ in diameter, which contained filaments or other cytoplasmic components in the clear areas within them. The second were lamellar structures (laminated cytoplasmic bodies, Morales) which appeared to be originating from endoplasmic reticulum. There was no distinct transition in these two types of inclusions and the relationship to each other is not clear. The significance of Bunina body is unknown, but some manifestation of a primary disorder, e.g., protein metabolism, rather than a secondary degenerative change in the motor neurons in amyotorophic lateral sclerosis.

Key words

Bunina bodies Anterior horn cells Amyotrophic lateral sclerosis Cytoplasmic laminar bodies Electron microscopy 

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References

  1. Bunina, T. L.: On intracellular inclusions in familial amyotrophic lateral sclerosis. Korsakow. J. Neuropath. Psychiat.62, 1293–1299 (1962)Google Scholar
  2. Doolin, P. F., Barron, K. D., Kwak, S.: Ultrastructural and histochemical analysis of cytoplasmic laminar bodies in lateral geniculate neurons of adult cat. Am. J. Anat.121, 601–622 (1967)Google Scholar
  3. Field, E. J., Narang, H. K.: An electron-microscopic study of scrapie in the rat: Further observations on “inclusion bodies” and virus-like particles. J. neurol. Sci.17, 347–364 (1972)Google Scholar
  4. Gross, B. G.: Annulate lamellae in the axillary apocrine glands of adult man. J. Ultrastr. Res.14, 64–73 (1966)Google Scholar
  5. Harrison, G. A.: Some observations on the presence of annulate lamellae in alligator and sea gull adrenal cortical cells. J. Ultrastr. Res.14, 158–166 (1966)Google Scholar
  6. Hart, M. N., Cancilla, P. A., Frommes, S. P.: Ultrastructure of “Bunina bodies”. 52nd Annual Meeting of American Neuropathologists, 1976Google Scholar
  7. Hart, M. N., Cancilla, P. A., Frommes, S. and Hirano, A.: Anterior horn cell degeneration and Bunina-type inclusions associated with dementia. Acta neuropath.38, 225–228 (1977)Google Scholar
  8. Herman, M. M., Ralston, H. J.: Laminated cytoplasmic bodies and annulate lamellae in the cat ventrobasal and posterior thalamus. Anat. Rec.167, 183–196 (1970)Google Scholar
  9. Hirano, A.: Pathology of amyotrophic lateral sclerosis. In: Gadjusek, D. C., Gibbs, C. J. and Alpers, M. (Eds.). Slow, latent, and temperate virus infections. NINDB Monograph No. 2, p. 23 Washington: National Institute of Health, 1965Google Scholar
  10. Hirano, A., Llena, J. F., Streifler, M., Cohn, D. F.: Anterior horn cell changes in a case of neurolathyrism. Acta neuropath.35, 277–283 (1976)Google Scholar
  11. Hirano, A., Kurland, L. T., Sayre, G. P.: Familial amyotrophic lateral sclerosis. Arch. Neurol.16, 232–243 (1967)Google Scholar
  12. Hirano, A.: Changes of the neuronal endoplasmic reticulum in the peripheral nervous system in mutant hamsters with hind leg paralysis and normal controls. J. Neuropath. exp. Neurol.37, 75–84 (1978)Google Scholar
  13. Kepes, J. J., Chou, S. M., Price, L. W.: Progressive multifocal leucoencephalopathy with 10-year survival in a patient with nontropic sprue. Report of a case with unusual light and electron microscopic features. Neurology25, 1006–1012 (1975)Google Scholar
  14. Kessel, R. G.: Intranuclear and cytoplasmic annulate lamellae in tunicate oocytes. J. Cell Biol.24, 471–487 (1965)Google Scholar
  15. Kruger, L., Maxwell, D. S.: Cytoplasmic laminar bodies in the striate cortex. J. Ultrastr. Res.26, 387–390 (1969)Google Scholar
  16. Martinez, A. J., Ohya, T., Jabbour, J. T., Duenas, D.: Subacute sclerosing panencephalitis (SSPE). Reappraisal of nuclear, cytoplasmic and axonal inclusions. Ultrastructural study of eight cases. Acta neuropath.28, 1–13 (1974)Google Scholar
  17. Mendell, J. R., Markesbery, W. R.: Neuronal intracytoplasmic hyalin inclusions. J. Neuropath. exp. Neurol.30, 233–239 (1971)Google Scholar
  18. Merkow, L., Leighton, J.: Increase numbers of annulate lamellae in myocardium of chick embryo incubated at abnormal temperatures. J. Cell Biol.28, 127–137 (1966)Google Scholar
  19. Morales, R. D., Duncan, D., Rehmet, R.: A distinctive laminated cytoplasmic body in the lateral geniculate body neurons of the cat. J. Ultrastr. Res.10, 116–123 (1964)Google Scholar
  20. Morales, R., Duncan, D.: Multilaminated bodies and other unusual configurations of endoplasmic reticulum in the cerebellum of the cat. An electron microscopic study. J. Ultrastr. Res.15, 480–489 (1966)Google Scholar
  21. Morecki, R., Zimmerman, H. M.: Human rabies encephalitis. Fine structure study of cytoplasmic inclusions. Arch. Neurol.20, 599–604 (1969)Google Scholar
  22. Nakamura, H., Takahashi, K., Nakashima, R., Kamoto, T.: Demyelinating encephalitis associated with progressive systemic scleroderma. Clin. Neurol. (Tokyo)14, 672–677 (1974)Google Scholar
  23. Norman, M. G.: Hyalin (“colloid”) cytoplasmic inclusions in motoneurons in association with familial microencephaly, retardation and seizures. J. neurol. Sci.23, 63–70 (1974)Google Scholar
  24. Novikoff, A. B.: Lysosomes in nerve cells. In: Hydén, H. (Ed.): The cell, p. 319. Amsterdam: Elsevier 1967Google Scholar
  25. Peters, A., Palay, S. L.: The morphology of laminae A and Al of the dorsal nucleus of the lateral geniculate body of the cat. J. Anat.100, 451–486 (1966)Google Scholar
  26. Schochet, S. S., Hardman, J. M., Ladeweg, P. P., Earle, K. M.: Intraneuronal conglomerates in sporadic motor neuron disease: A light and electron microscopic study. Arch. Neurol.20, 548–553 (1969)Google Scholar
  27. Sung, J. H., Hayano, M., Mastri, A. R., Okagaki, T.: A case of human rabies and ultrastructure of the Negri body. J. Neuropath. exp. Neurol.35, 541–559 (1976)Google Scholar
  28. Szlachta, H. L., Habel, R. E.: Inclusions resembling Negri bodies in the brains of nonrabid cats. Cornell Veterinarian,43, 207–212 (1953), cited by Doolin et al. (1962)Google Scholar
  29. Takahashi, K., Nakamura, H., Okada, E.: Hereditary amyotrophic lateral sclerosis. Histochemical and electron microscopic study of hyalin inclusions in motor neurons. Arch. Neurol.27, 292–299 (1972)Google Scholar
  30. Takahashi, K., Nakamura, H., Agari, M., Yamasaki, I.: Demyelinating myelitis with painful tonic seizures. Report of an autopsy case. Clin. Neurol. (Tokyo)16, 117–123 (1976)Google Scholar
  31. Takei, Y., Mirra, S. S.: Intracytoplasmic hyalin inclusion bodies in the nerve cells of the hypoglossal nucleus in human autopsy material. Acta neuropath.17, 14–23 (1971)Google Scholar
  32. Wisotzkey, H. M., Mossy, J.: Hyalin (“colloid”) cytoplasmic inclusions in neurons of human hypoglossal nuclei. Arch. Path. (Chic.)93, 61–70 (1972)Google Scholar

Copyright information

© Springer-Verlag 1978

Authors and Affiliations

  • M. Tomonaga
    • 1
    • 2
  • M. Saito
    • 1
    • 2
  • M. Yoshimura
    • 1
    • 2
  • H. Shimada
    • 1
    • 2
  • H. Tohgi
    • 1
    • 2
  1. 1.Department of Clinical Pathology (Neuropathology)Tokyo Metropolitan Institute of GerontologyTokyoJapan
  2. 2.Departments of Pathology and NeurologyTokyo Metropolitan Geriatric HospitalTokyoJapan

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