Summary
In this case of Creutzfeldt-Jakob disease cortical biopsy demonstrated a large number of papova virus-like particles in the axons, dendrites, astrocytic processes and blood-vessel walls, while necropsy findings disclosed a striking status spongiosus and neuronal degeneration in the substantia nigra.
The patient also suffered from a testicular feminization syndrome and was treated with immunosuppressive drugs for pemphigus vulgaris.
The ready demonstration of viral particles, the rapid course of the disease and the unusual necropsy findings are discussed and related to the pre-existing diseases of the patient.
Zusammenfassung
Es wird ein Fall von Creutzfeldt-Jakobscher Erkrankung beschrieben, bei welchem eine Biopsie der Großhirnrinde eine große Zahl von Papovavirus-artigen Partikeln in den Axonen, Dendriten, Astrocytenfortsätzen und Blutgefäßwänden ergab. Bei der Autopsie fanden sich ein Status spongiosus und Nervenzelldegenerationen in der Substantia nigra. Beim Patienten lag auch ein Syndrom der testikulären Feminisierung vor, und er wurde wegen eines Pemphigus vulgaris mit Immunosuppressiva behandelt. In der Diskussion werden die nachgewiesenen Viruspartikeln, der rasche Verlauf der Erkrankung und die ungewöhnlichen Autopsiebefunde im Zusammenhang mit der Grundkrankheit diskutiert.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Adams, R. D., Sidman, R. L.: Introduction to neuropathology, pp. 456–467. New York: McGraw-Hill 1968
Alter, M.: Creutzfeldt-Jakob disease: hypothesis for high incidence in Libyan Jews in Israël. Science 186, 848 (1974)
Alter, M., Frank, Y., Doyne, H., Webster, D. P.: Creutzfeldt-Jakob disease after eating ovine brain. New Engl. J. Med. 292, 927 (1975)
Bignami, A., Forno, L. S.: Status spongiosus in Jakob-Creutzfeldt disease. Brain 93, 89–94 (1970)
Bobowick, A. R., Brody, J. A., Matthews, M. R., Roos, R., Gajdusek, D. C.: Creutzfeldt-Jakob disease: a case control study. Amer. J. Epidem. 98, 381–394 (1973)
Bots, G., De Man, J. C., Verjaal, A.: Virus-like particles in Creutzfeldt-Jakob disease. Acta neuropath. (Berl.) 18, 267–270 (1971)
De Reuck, J., De Coster, W., vander Eecken, H., Daneels, P.: Creutzfeldt-Jakob disease: a comparative light-microscopic, histological and electron-microscopic study. Europ. Neurol. 13, 154–166 (1975)
Duffy, P., Wolf, J., Collins, G., De Voe, A., Streeten, B., Cowen, D.: Possible person-to-person transmission of Creutzfeldt-Jakob disease. New Engl. J. Med. 290, 692 (1974)
Dumas, J. L. R.: Enroulements de fragments membranaires dans un cas de maladie de Creutzfeldt-Jakob. Acta neuropath. (Berl.) 19, 343–346 (1971)
Fisher, C. M.: The clinical picture in Creutzfeldt-Jakob disease. Trans. Amer. neurol. Ass. 147–150 (1960)
Gardner, G. D., Field, A. M., Coleman, D. V., Hulme, B.: New human papova virus (BK) isolated from urine after renal transplantation. Lancet 1971 I, 1253–1257
Gibbs, C. J., Jr., Gajdusek, D. C.: Infection as the etiology of spongiform encephalopathy (Creutzfeldt-Jakob disease). Science 165, 1023–1025 (1969)
Gibbs, C. J., Jr., Gajdusek, D. C.: Biology of Kuru and Creutzfeldt-Jakob disease. In: slow virus disease (eds. Zeman and Lennette), pp. 39–48. American Association of Pathologists and Bacteriologists. Baltimore: Williams and Wilkins 1974
Gonatas, N. K., Terry, R. D., Weis, M.: Electron microscopic study in two cases of Jakob-Creutzfeldt disease. J. Neuropath. exp. Neurol. 24, 575–598 (1965)
Hager, H.: Electron microscopy of nervous tissue. In: Pathology of the nervous system (ed. J. Minckler), pp. 149–167. New York: McGraw-Hill 1968
Herzberg, L., Herzberg, B. N., Gibbs, C. J., Jr., Sullivan, W., Amyx, H., Gajdusek, D. C.: Creutzfeldt-Jakob disease: Hypothesis for high incidence in Libyan jews in Israël. Science 186, 848 (1974)
Kahana, E., Alter, M., Braham, J., Sofer, D.: Creutzfeldt-Jakob disease: Focus among Libyan jews in Israël. Science 183, 90–91 (1974)
Kidd, M.: Some electron microscopical observations on status spongiosus. Acta neuropath. (Berl.), Suppl. 3, 137–144 (1967)
Kirschbaum, W. R.: Jakob-Creutzfeldt disease. New York: American Elsevier Publishing Company 1968
Lampert, P. W., Gajdusek, D. C., Gibbs, C. J., Jr.: Subacute spongiform virus encephalopathies. Amer. J. Path. 68, 626–652 (1972)
Marsh, R. F., Hanson, R. P.: Transmissible mink encephalopathy, infectivity of corneal epithelium. Science 187, 656 (1975)
Narang, H. K.: Virus-like particles in Creutzfeldt-Jakob. Biopsy material. Acta neuropath. (Berl.) 32, 163–168 (1975)
Peters, A., Palay, S. L., De F. Webster, H.: The fine structure of the nervous system. The cells and their processes, p. 108. New York: Hoeber 1970
Sack, G. H., Narayan, O., Danna, K. J., Weiner, L. P., Nathans, D.: The nucleid acid of a SV 40-like virus isolated from a patient with progressive multifocal leukoencephalopathy. Virology 51, 345–350 (1973)
Shah, K. V., Ozer, H. L., Pond, H. S., Palma, L. D., Murphy, G. P.: Appearance of SV 40 neutralizing antibodies in sera of U.S. residents without history of polio immunization. Nature (Lond.) 231, 448–449 (1971)
Siedler, H., Malamud, N.: Creutzfeldt-Jakob disease. Clinico-pathologic report of 15 cases and review of the literature. J. Neuropath. exp. Neurol. 22, 381–402 (1963)
Van Rossum, A.: Spastic pseudosclerosis (Creutzfeldt-Jakob disease). In: Handbook of clinical neurology, Vol. 6: Diseases of the basal ganglia (eds. P. J. Vinken, W. De Bruyn), pp. 726–760. Amsterdam: North Holland Publishing Company 1968
Vernon, M. L., Horta-Barbarosa, L., Fuccillo, D. A., Sever, J. L., Baringer, J. R., Birnbaum, G.: Virus-like particles and nucleoprotein type filaments in brain tissue from two patients with Creutzfeldt-Jakob disease. Lancet 1970 I, 964–966
Weiner, L. P., Herndon, R. M., Narayan, O., Johnson, R. T., Shah, K., Rubenstein, L. P., Preziosi, T. J., Conley, F. K.: Isolation of virus related SV40 from patients with progressive multifocal leukoencephalopathy. New Engl. J. Med. 286, 385–390 (1972)
Weiner, L. P., Narayan, O.: Progressive multifocal leukoencephalopathy. In: Infections diseases of the central nervous system (eds. R. A. Thompson, J. R. Green). Advances in neurology, Vol. 6, pp. 87–92. New York: Raven Press 1974
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
de Reuck, J., de Coster, W., Otte, G. et al. Papova virus-like particles in a nigral type of creutzfeldt-jakob disease. J. Neurol. 213, 179–188 (1976). https://doi.org/10.1007/BF00312868
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00312868