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Progressive multifocal leukoencephalopathy with paramyxovirus-like structures, Hirano bodies and neurofibrillary tangles

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Summary

We have identified three structures that have not previously been reported in electron microscopic examinations of brain from patients with PML: 1. coiled nuclear bodies that may possibly represent tubular paramyxovirus nucleocapsids, 2. Hirano bodies and 3. Neurofibrillary tangles.

The paramyxovirus-like material is interesting in view of reports of a transition between nuclear bodies and tubular paramyxovirus nucleocapsids and because of a possible interaction between syncytiogenic paramyxoviruses and Papova viruses in the pathogenesis of SSPE and PML. A study of the Hirano bodies in this case, taken in conjunction with the findings of others leads us to believe that these structures are formed from an overgrowth of neurofilaments. The presence of tangled masses of neurofilaments in the processes of neurons also seen in the present case is consistent with this hypothesis.

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References

  • Åström, K. E., Mancal, E. L., Richardson, E. P., Jr.: Progressive multifocal leukoencephalopathy. Brain81, 93–111 (1958)

    Google Scholar 

  • Gambetti, P., Nellman, W. J., Gonatas, N. K.: Familial spongy-degeneration of the central nervous system (Van Bogaert-Bertrand disease). Acta neuropath. (Berl.)12, 103–116 (1969)

    Google Scholar 

  • Gibbs, C. J., Jr., Gadjusek, D. C., Asher, D. M., Alpers, M. P., Beck, E., Daniel, P. M., Matthews, W. B.: Creutzfeldt-Jakob disease (spongioform encephalopathy): Transmission to the chimpanzee. Science161, 388–389 (1968)

    Google Scholar 

  • Györkey, F., Sinkovics, J. G., Min, K. W., Györkey, P.: A morphologic study on the occurrence and distribution of structures resembling viral nucleocapsids in collagen disease. Amer. J. Med.53, 148–158 (1972)

    Google Scholar 

  • Hadfield, M. G., David, R. B., Rosenblum, W. I.: Coiled nucleocapsid configuration in subacute sclerosing panencephalitis (SSPE). Acta neuropath. (Berl.)21, 263–271 (1972)

    Google Scholar 

  • Herndon, R. M., Rubinstein, L. J.: Light and electron microscopy observations on the development of viral particles in the inclusions of Dawson's encephalitis (subacute sclerosing panencephalitis). Neurology (Minneap.)18, Part 2 8–20 (1968)

    Google Scholar 

  • Hirano, A., Dembitzer, H. M., Kurland, L. T., Zimmerman, H. M.: The fine structure of some intraganglionic alterations. J. Neuropath. exp. Neurol.27, 167–182 (1968)

    Google Scholar 

  • Hirano, A.: Amyotrophic lateral sclerosis and Parkinsonism-dementia complex on Guam. Arch. Neurol. (Chic.)15, 35 (1966)

    Google Scholar 

  • Koprowski, H., Barbanti-Brodano, G., Katz, M.: Interaction between Papova-like virus and paramyxovirus in human brain cells: a hypothesis. Nature (Lond.)225, 1045–1047 (1970)

    Google Scholar 

  • Levy, N. L.: Synergism between polyoma and inactivated sendai virus in human astrocytes, fibroblasts and Hela cells. J. Neuropath. exp. Neurol.31, 639–644 (1972)

    Google Scholar 

  • Luft, R., Ikkos, D., Palmieri, G., Ernster, L., Afzelius, B.: A case of severe hypermet of non-thyroid origin with a defect in the maintenance of mitochondrial respiration control; a correlated clinical biochemical and morphological study. J. clin. Invest.41, 1776–1804 (1962)

    Google Scholar 

  • Müller, D., ter Meulen, V., Katz, M., Koprowski, H.: Cytochemical evidence for the presence of two viral agents in subacute sclerosing panencephalitis. Lab. Invest.25, 337–342 (1971)

    Google Scholar 

  • Ogata, J., Budzilovich, G. N., Cravioto, H.: A study of rod-like structures (Hirano bodies) in 240 normal and pathological brains. Acta neuropath. (Berl.)21, 61–67 (1972)

    Google Scholar 

  • Oyanagi, S., Müller, D., ter Meulen, V., Katz, M., Koprowski, H.: Electron microscopic observations in subacute sclerosing panencephalitis brain cell cultures: Their correlation with cytochemical and immunocytological findings. J. Virol.6, 370–379 (1970)

    Google Scholar 

  • Oyanagi, S., Rorke, L. B., Katz, M., Koprowski, H.: Histopathology and electron microscopy of three cases of subacute sclerosing panencephalitis (SSPE). Acta neuropath. (Berl.)18, 58–73 (1971)

    Google Scholar 

  • Ramsey, H. J.: Altered synaptic terminals in cortex near tumor. Amer. J. Path.51, 1093 (1967)

    Google Scholar 

  • Richardson, E. P., Jr.: Progressive multifocal leukoencephalopathy. In: Contemporary neurology symposia, I: The remote effects of cancer on the nervous system.6, 16 (1965)

  • Schochet, S. S., Jr., McCormick, W. F.: Ultrastructure of Hirano bodies. Acta neuropath. (Berl.)21, 50–60 (1972)

    Google Scholar 

  • Shafiq, S. A., Dubowitz, V., Peterson, Hart de C., Milhorrat, A. T.: Nemaline myopathy report of a fatal case with histochemical and electron microscopic studies. Brain90, 817–828 (1967)

    Google Scholar 

  • Shutta, H. S., Armitage, J. L.: Thyrotoxic hypokalemic periodic paralysis. J. Neuropath. exp. Neurol.28, 321–336 (1969)

    Google Scholar 

  • Shutta, H. S., Kelly, A. M., Zacks, S. I.: Necrosis and regeneration of muscle in paroxysmal idiopathic myoglobinuria. Electron Microscopic observation. Brain92, 191–202 (1969)

    Google Scholar 

  • Toga, M., Bérard-Badier, M., Gambarelli, D., Pinsard, N., Hassoun, J.: Un cas de dystrophie neuroaxonale infantile ou maladie de Seitelberger. III Etude ultrastructurale du muscle strié. Acta neuropath. (Berl.)18, 367–341 (1971)

    Google Scholar 

  • Weiss, P. A., Mayr, R.: Organelles in neuroplasmic (“axonal”) flow: neurofilaments. Proc. nat. Acad. Sci. (Wash.)68, 846 (1971)

    Google Scholar 

  • Zu Rhein, G. M., Chou, S. M.: Subacute sclerosing panencephalitis. Ultrastructural study of a brain biopsy. Neurology (Minneap.)18, 146–160 (1968a)

    Google Scholar 

  • Zu Rhein, g. M., Chou, S. M., Papovavirus in progressive multifocal leukoencephalopathy. Res. Publ. Ass. nerv. ment. Dis.44, 307–362 (1968b) (Proceedings of the 1964 conference on infections of the nervous system.)

    Google Scholar 

  • Zu Rhein, G. M.: Association of Papova-virions with a human demyelinating disease (Progressive multifocal leukoencephalopathy). Progr. med. Virol.11, 185–247 (1969)

    Google Scholar 

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Hadfield, M.G., Martinez, A.J. & Gilmartin, R.C. Progressive multifocal leukoencephalopathy with paramyxovirus-like structures, Hirano bodies and neurofibrillary tangles. Acta Neuropathol 27, 277–288 (1974). https://doi.org/10.1007/BF00690693

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