Skip to main content

Chronic Neurological Diseases

Subacute Sclerosing Panencephalitis, Progressive Multifocal Leukoencephalopathy, Kuru, Creutzfeldt-Jakob Disease

  • Chapter
Book cover Viral Infections of Humans

Abstract

Most subacute and chronic progressive degenerative diseases of the central nervous system of man have been classified as disorders of unknown etiology. Few if any are curable, and although some are genetically determined, most are sporadic in occurrence, and there appears not to be a history of the disease in close relatives. That any one or more of these chronic idiopathic disorders might have infection as their etiology was not recognized until the subacute progressive degenerative heredofamilial disease kuru was transmitted to chimpanzees and was subsequently shown to be serially transmissible in experimental animals inoculated with bacteria- free filtrates of brain tissues from animals dying with the disease. Kuru thus became the first subacute fatal central nervous system disease of man to have a virus-induced “slow infection” established as its etiology.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Alema, G., Transmissible and genetic late dementiae, in: Proceedings of the Tenth International Congress of Neurology, Abstract No. 29 (A. Subirana and J. M. Burrows, eds.), p. 13, Barcelona, September 9–14, 1973, Int. Congr. Ser., No. 319, Excerpta Medica, Amsterdam, 1973.

    Google Scholar 

  2. Alpers, M., Epidemiological changes in kuru, 1957 to 1963, in: Slow, Latent and Tempurate Virus Infections, NINDB Monogr. No. 2 (D. C. Gajdusek, C. J. Gibbs, Jr., and M. Alpers, eds.), pp. 65–82, National Institutes of Health, PNS Publ. No. 1378, Department of Health, Education, and Welfare, Government Printing Office, Washington, D.C., 1965.

    Google Scholar 

  3. Alpers, M. P., Kuru: Implications of its transmissi- bility for the interpretation of its changing epidemiologic pattern, in: The Central Nervous System Some Experimental Models of Neurological Diseases, (O. T. Bailey and D. E. Smith, eds.), pp. 234–251, Proceedings of the Fifty-sixth Annual Meeting of the International Academy of Pathology, Washington, D. C., March 12–15, 1967, International Academy of Pathology Monograph No. 9, Williams and Wilkins, Baltimore, 1968.

    Google Scholar 

  4. Alpers, M. P., Epidemiology and ecology of kuru, in: Slow Transmissible Diseases of the Nervous System, Vol. I, Clinical, Epidemiological, Genetic, and Pathological Aspects of the Spongiform Encephalopathies (S. B. Prusiner and W. J. Hadlow, eds.), pp. 67–90, Academic Press, New York, 1979.

    Google Scholar 

  5. Baguley, D. M., and Glasgow, G. L., Subacute sclerosing panencephalitis and Salk vaccine, Lancet 2:763–765 (1973).

    PubMed  CAS  Google Scholar 

  6. Bernoulli, C., Siegfried, J., Baumgartner, C., Regli, F., Rabinowicz, T., Gajdusek, D. C., and Gibbs, C. J., Jr., Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery, Lancet 1:478–479 (1979).

    Google Scholar 

  7. Bobowick, A. R., Brody, J. A., Matthews, M. R., Roos, R., and Gajdusek, D. C, Creutzfeldt-Jakob disease: A case control study, Am. J. Epidemiol. 98:381–394 (1973).

    PubMed  CAS  Google Scholar 

  8. Bolton, C. F., and Rozdilsky, B., Primary progressive multifocal leukoencephalopathy, Neurology 21:72–77 (1971).

    PubMed  CAS  Google Scholar 

  9. Brody, J. A., and Detels, R., Subacute sclerosing panencephalitis: A zoonosis following aberrant measles: Hypothesis, Lancet 2:500–501 (1970).

    PubMed  CAS  Google Scholar 

  10. Brody, J. A., Detels, R., and Sever, J. L., Measles-antibody titers in sibships of patients with subacute sclerosing panencephalitis and controls, Lancet 1:177–178 (1972).

    PubMed  CAS  Google Scholar 

  11. Brown, F., and Cathala, F., Creutzfeldt-Jakob disease in France, in: Slow Transmissible Diseases of the Nervous System, Vol. I, Clinical, Epidemiological, Genetic, and Pathological Aspects of the Spongiform Encephalopathies (S. B. Prusiner and W. J. Hadlow, eds.), pp. 213–227, Academic Press, New York, 1979.

    Google Scholar 

  12. Brown, P., Cathala, F., and Gajdusek, D. C, Cruetzfeldt-Jakob disease in France. III. Epidemiological study of 170 patients dying during the decade 1968–1977, Ann. Neurol. 6:438–446 (1979).

    PubMed  CAS  Google Scholar 

  13. Brown, P., Hooks, J., Roos, R., Gajdusek, D. C, and Gibbs, C. J. Jr., Attempt to identify the agent of Creutzfeldt-Jakob disease by antibody relationship to known viruses, Nature (London) New Biol. 235:149–152 (1972).

    CAS  Google Scholar 

  14. Brown, P., Tsai, T., and Gajdusek, C., Seroepide- miology of human papovaviruses, Am. J. Epidemiol. 102:331–340 (1975).

    PubMed  CAS  Google Scholar 

  15. Brun, A., Nordenfeldt, E., and Kjellen, L., Aspects on the variability of progressive multifocal leukoencephalopathy, Acta Neuropathol. 24:232–243 (1973).

    PubMed  CAS  Google Scholar 

  16. Byington, D. P., and Burnstein, T., Measles encephalitis produced in suckling rats, Exp. Mol. Pathol. 19:36–43 (1973).

    PubMed  CAS  Google Scholar 

  17. Canal, N., and Torck, P., An epidemiological study of subacute sclerosing leucoencephalitis in Belgium, J. Neurol. Sci. 1:380–389 (1964).

    Google Scholar 

  18. Candeias, J. A. N., Baruzzi, R. G., Pripes, S., and Iunes, M., Prevalence of antibodies to the BK and JC papovaviruses in isolated populations, Rev. Saude Publica 11:510–514 (1977).

    Google Scholar 

  19. Canelas, H. M., Juliao, O. F., Lefevre, A. B., Lamartine de Assis, Tognola, W. A., de Jorge, F. B., Fonseca, L. C., and Xavier-Lima, A., Subacute sclerosing panencephalitis: epidemiological, clinical and biochemical study of 31 cases, Arch. Neuro-Psychiatr. (Säo Paulo) 25:255–268 (1967).

    Google Scholar 

  20. CASTAIGNE, P., RONDOT, P., ESCOURELLE, R., RIDA-DEAU, D., LUMAS, J. L., CATHALA, F., and HAUW, J. J., Leucoencephalopathe multifocale progressive et “gliomes” multiples, Rev. Neurol. 130:379–393 (1974).

    PubMed  CAS  Google Scholar 

  21. Connolly, J. H., Haire, M., and Hadden, D. S. M., Measles immunoglobulins in subacute sclerosing panencephalitis, Br. Med. J. 1:23–25 (1971).

    PubMed  CAS  Google Scholar 

  22. Dayan, A. D., and Stokes, M. I., Immune complexes and visceral deposits of measles antigens in subacute sclerosing panencephalitis, Br. Med. J. 2:374–376 (1972).

    PubMed  CAS  Google Scholar 

  23. Detels, R., Brody, J. A., McNew, J., and Edgar, A. H., Further epidemiologic studies of subacute sclerosing panecephalitis, Lancet 2:11–14 (1973).

    PubMed  CAS  Google Scholar 

  24. Donner, M., Halonen, H., and Haltia, M., Subakuutti sklerosoiva panenkefaliitti, Duodecim 85:541–553 (1969).

    PubMed  CAS  Google Scholar 

  25. Duffy, P., Wolf, J., Collins, G., Devoe, A. G., Streeten, B., and Cowen, D., Person-to-person transmission of Creutzfeldt-Jakob disease, N. Engl. J. Med. 299:692–693 (1974).

    Google Scholar 

  26. Ferber, R. A., Wiesenfeld, S. L., Roos, R. P., Bobowick, A. R., Gibbs, C. J., Jr., and Gajdusek, D. C., Familial Creutzfeldt-Jakob disease: Transmission of the familial disease to primates, in: Neurology (A. Subriana and J. E. Burrows, eds.), pp. 358–380 Proceedings of the Tenth International Congress of Neurology, Barcelona, September 8–15, 1973, Int. Congr. Ser., No. 319, Excerpta Medica, Amsterdam, 1973.

    Google Scholar 

  27. Fermaglich, J., Hardman, J. M., and Earle, K. M., Spontaneous progressive multifocal leukoencephalopathy, Neurology 20:479–484 (1970).

    PubMed  CAS  Google Scholar 

  28. Gajdusek, D. C., Kuru in the New Guinea highlands, in: Tropical Neurology, Vol. 29 (J. D. Spillane, ed.), pp. 376–383, Oxford University Press, London, 1973.

    Google Scholar 

  29. Gajdusek, D. C., Slow infections with unconventional viruses, Harvey Led. 72:283–353 (1978).

    CAS  Google Scholar 

  30. Gajdusek, D. C., and Gibbs, C. J., Jr., Kuru and the virus dementias, in: Conference on Biohazards in Cancer Research, Pacific Grove, California, January 22–24, 1973, Biohazards in Biological Research (A. Hell-men, M. N. Oxman, and R. Pollack, eds.), pp. 288–299, Cold Spring Harbor Press, Cold Spring Harbor, New York, 1973.

    Google Scholar 

  31. Gajdusek, D. C., and Gibbs, C. J., Jr., Subacute and chronic diseases caused by atypical infections with unconventional viruses in aberrant hosts, in: Persistent Virus Infections, Vol. VIII, Perspectives in Virology M. Pollard, ed.), pp. 279–311, Academic Press, New York, 1973.

    Google Scholar 

  32. Gajdusek, D. C., and Gibbs, C. J., Jr., Familial and sporadic chronic neurologic degenerative disorders transmitted from man to primates, in: Advances in Neurology, Vol. 10, Primate Models of Neurological Disorders (B. S. MeldrumandC. D. Marsden, eds.), pp. 291–375, Raven Press, New York, 1975.

    Google Scholar 

  33. Gajdusek, D. C., Gibbs, C. J., Jr., and Alpers, M., Experimental transmission of a kuru-like syndrome to chimpanzees, Nature (London) 209:794–796 (1966).

    PubMed  CAS  Google Scholar 

  34. Gajdusek, D. C., Gibbs, C. J., Jr., Earle, K., Dammin, G. J., Schoene, W. C., and Tyler, H. R., Transmission of subacute spongiform encephalopathy to the chimpanzee and squirrel monkey from a patient with papulosis atrophicans maligna of Kohlmeier-Degos, in: Proceedings of the Tenth Internationational Congress on Neurology (A. Subirana and J. Burrows, eds.), pp. 390–392, Int. Cong. Ser., No. 319, Excerpta Medica, Amsterdam, 1973.

    Google Scholar 

  35. Gajdusek, D. C., Gibbs, C. J., Jr., and Alpers, M. (eds.), Slow, Latent and Temperate Virus Infections, NINDB Monogr. No. 2, National Institutes of Health, PNS Publ. No. 1378, Department of Health, Education, and Welfare, Government Printing Office, Washington, D.C., 1965.

    Google Scholar 

  36. Gajdusek, D. C., Gibbs, C. J., Jr., Asher, D. M., Brown, P., Duvan, A., Hoffman, P., Nemo, G., Rohwer, R., and White, L., Precautions in medical care of, and in handling materials from, patients with transmitted virus dementia (Creutzfeldt-Jakob disease), N. Engl. J. Med. 297:1253–1258 (1977).

    PubMed  CAS  Google Scholar 

  37. Gajdusek, D. C., Gibbs, C. J., Jr., and Asher, D. M., Letter to the editor, N. Engl. J. Med. 298:976 (1978).

    Google Scholar 

  38. Gardner, S. D., Prevalence in England of antibody to human polyomavirus (B.K.), Br. Med. J. 1:77–78 (1973).

    PubMed  CAS  Google Scholar 

  39. GARDNER, S. D., FIELD, A., COLEMAN, D., AND HULME, B., New Human Papovavirus (B.K.) Isolated From Urine After Renal Transplantation, Lancet 1:1253–1257 (1971).

    PubMed  CAS  Google Scholar 

  40. Gerber, M. A., Shah, K. V., Thung, S. N., and Zu Rhein, G. M., Immunohistochemical demonstration of common antigen of polyomaviruses in routine histologic tissue sections of animals and man, Amer. J. Clin. Pathol. 73:794–797 (1980).

    Google Scholar 

  41. Gerson, K. L., and Haslam, R. H. A., Subtle immunologic abnormalities in SSPE, N. Engl. J. Med. 285:78–82 (1971).

    PubMed  CAS  Google Scholar 

  42. GIBBS, C. J., JR., AMYX, H. L., BACOTE, A., MASTERS, C. L., AND GAJDUSEK, D. C., Oral Transmission Of Kuru, Creutzfeldt-Jakob Disease, And Scrapie To Non- Human Primates J. Infect. Dis. 142:205–208 (1980).

    PubMed  Google Scholar 

  43. Gibbs, C. J., Jr., and Gajdusek, D. C., Transmission and characterization of the agents of spongiform virus encephalopathies, kuru, Creutzfeldt-Jakob disease, scrapie and mink encephalopathy, in: Immunological Disorders of the Nervous System, Vol. XLIX (L. P. Rowland, ed.), Res. Publ. A.R.N.M.D., pp. 383–410, Williams and Wilkins, Baltimore, 1971.

    Google Scholar 

  44. Gibbs, C. J., Jr., and Gajdusek, D. C., Experimental subacute spongiform virus encephalopathies in primates and other laboratory animals, Science 182:67–68 (1973).

    PubMed  Google Scholar 

  45. Gibbs, C. J., Jr., and Gajdusek, D. C, Biology of kuru and Creutzfeldt-Jakob disease, in: Slow Virus Diseases (W. Zeman and E. H. Lennette, eds.), pp. 39–48, Williams and Wilkins, Baltimore, 1973.

    Google Scholar 

  46. Gibbs, C. J., Jr., Gajdusek, D. C, Asher, D. M., Alpers, M. P., Beck, E., Daniel, P. M., and Matthews, W. B., Creutzfeldt-Jakob disease (subacute spongiform encephalopathy): Transmission to the chimpanzee, Science 161:388–389 (1968).

    PubMed  Google Scholar 

  47. Goldberg, H., Alter, M., and Kahana, E., The Lybian Jewish focus of Creutzfeldt-Jakob disease: A search for the mode of natural transmission, in: Slow Transmissible Diseases of the Nervous System, Vol. I, Clinical, Epidemiological, Genetic, and Pathological Aspects of the Spongiform Encephalopathies (S. B. Prusiner and W. J. Hadlow, eds.), pp. 195–211, Academic Press, New York, 1979.

    Google Scholar 

  48. Hadlow, W. J., Scrapie and kuru, Lancet 2:289–290 (1959).

    Google Scholar 

  49. Hadlow, W. J., Prusiner, S. B., Kennedy, R. C, and Race, R. E., Brain tissue from persons with Creutzfeldt-Jakob disease causes scrapie-like encephalopathy in goats, Ann. Neurol. 8:628–631 (1980).

    PubMed  CAS  Google Scholar 

  50. Hall, W. W., Lamb, R. A., and Choppin, P. W., Measles and subacute sclerosing panencephalitis virus proteins: Lack of antibodies to the M protein in patients with subacute sclerosing panencephalitis, Proc. Natl. Acad. Sci. U.S.A. 76:2047–2051 (1979).

    PubMed  CAS  Google Scholar 

  51. Halsey, N. A., Modlin, J. F., Jabbour, J. T., Dubey, L., Eddens, D. L., and Ludwig, D. D., Risk factors in SSPE: A case-control study, Am. J. Epidemiol. 111:415–431 (1980).

    PubMed  CAS  Google Scholar 

  52. Hedley-Whyte, E. T., Smith, B. P., Tyler, H. R., and Peterson, W. P., Multifocal leukoncephalopathy with remission and five year survival, J. Neuropathol. Exp. Neurol 25:107–116 (1966).

    Google Scholar 

  53. Herzberg, L., Herzberg, B. W., Gibbs, C. J., Jr., Sullivan, W., Amyx, H., and Gajdusek, D. C, Creutzfeldt-Jakob disease: Hypothesis for high incidence in Libyan Jews in Israel, Science 186:848 (1974).

    PubMed  CAS  Google Scholar 

  54. Hirano, A., Personal communication, 1974.

    Google Scholar 

  55. Ho, K., Garancis, J. C., Paegle, R. D., Gerber, M. A., and Borkowski, W. J., Progressive multifocal leukoencephalopathy and malignant lymphoma of the brain in a patient with immunosuppressive therapy, Acta Neuropathol. (Berlin) 52:81–83 (1980).

    PubMed  CAS  Google Scholar 

  56. Hooks, J. J., Gibbs, C. J., Jr., Chopra, H., Lewis, M., and Gajdusek, D. C, Spontaneous transformation of human brain cells grown in vitro and characterization of associated virus particles, Science 176:1420–1422 (1972).

    PubMed  CAS  Google Scholar 

  57. Hornabrook, R. W., Kuru—A subacute cerebellar degeneration: The natural history and clinical features, Brain 91:53–74 (1968).

    PubMed  CAS  Google Scholar 

  58. Horta-Barbosa, L., Subacute sclerosing panencephalitis: Isolation of suppressed measles virus from lymph node biopsies, Science 173:840–841 (1971).

    PubMed  CAS  Google Scholar 

  59. Horta-Barbosa, L., Fuccillo, D. A., London, W. T., Jabbour, J. T., Zeman, W., and Sever, J. L., Isolation of measles virus from brain cell cultures of two patients with subacute sclerosing panencephalitis, Proc. Soc. Exp. Biol Med. 132:272–277 (1969).

    PubMed  CAS  Google Scholar 

  60. Jabbour, J. T., Duenas, D. A., Sever, J. L., Krebs, H. M., and Horta-Barbosa, L., Epidemiology of subacute sclerosing panencephalitis (SSPE), J. Am. Med. Assoc. 220:959–962 (1972).

    CAS  Google Scholar 

  61. Jabbour, J. T., and Sever, J. L., Serum measles antibody titers in patients with subacute sclerosing panencephalitis, compared with parents and siblings, J. Pediatr. 73:905–907 (1968).

    PubMed  CAS  Google Scholar 

  62. Jellinger, V. K., Seitelberger, F., Heiss, W. D., and Holczabek, W., Konjugale Form der subakutem spongiosen Enzephalopathie (Jakob-Creutzfeldt-Er-krankung), Wien. Klin. Wochenschr. 84:245–249 (1972).

    PubMed  CAS  Google Scholar 

  63. Joasoo, A., and Wolfenden, W. H., Subacute spongiform encephalopathy, Med. J. Aust. 1:354–356 (1968).

    PubMed  CAS  Google Scholar 

  64. Kahana, E., Alter, M., Braham, J., and Sofer, D., Creutzfeldt-Jakob disease: A focus among Libyan Jews in Israel, Science 183:90–91 (1974).

    PubMed  CAS  Google Scholar 

  65. Katz, M., and Koprowski, H., The significance of failure to isolate infectious viruses in cases of subacute sclerosing panencephalitis, Arch. Gesamte Virusforsch. 41:390–393 (1973).

    PubMed  CAS  Google Scholar 

  66. Katz, M., Rorke, L. B., Masland, W. S., Brodano, G. B., and Koprowski, H., Subacute sclerosing panencephalitis: Isolation of a virus encephalitogenic for ferrets, J. Infect. Dis. 121:188–195 (1970).

    PubMed  CAS  Google Scholar 

  67. Kirschbaum, W. R., Jakob-Creutzfeldt Disease, American Elsevier, New York, 1968, 251 pp.

    Google Scholar 

  68. Koprowski, H., Interaction between papova-like virus and paramyxovirus in human brain cells: A hypothesis, Nature (London) 225:1045–1047 (1970).

    PubMed  CAS  Google Scholar 

  69. Kuroda, Y., Gibbs, C. J., Jr., Amyx, H. L., and Gajdusek, D. C., The pathogenesis of Creutzfeldt-Jakob disease in the mouse, Infect. Immun, (in press) (1981).

    Google Scholar 

  70. Legrain, M., et al, Leuco-encepholie multifocal progressive après transplantation renale, J. Neurol Sci. 23:49–62 (1974).

    PubMed  CAS  Google Scholar 

  71. London, W. T., Houff, S. A., Madden, D. L., Fucillo, D. A., Gravell, M., Wallen., W. C., Palmer., A. E., Sever, J. L., Padgett, B. L., Walker, D. L., Zu Rhein, G. M., and Ohashi, T., Brain tumors in owl monkeys inoculated with a human Polyomavirus (JC virus), Science 201:1246–1249 (1978).

    PubMed  CAS  Google Scholar 

  72. Machamer, C. E., Hayes, E. C., Gollobin, S. D., Westfall, L. K., and Zweerink, H. J., Antibodies against the measles matrix polypeptide after clinical infection and vaccination, Infect. Immun. 27:817–825 (1980).

    PubMed  CAS  Google Scholar 

  73. Malmgren, R., Kurland, L., Mokri, B., andKurtzke, J., The epidemiology of Creutzfeldt-Jakob disease, in: Slow Transmissible Diseases of the Nervous System, Vol. I, Clinical, Epidemiological, Genetic, and Pathological Aspects of the Spongiform Encephalopathies, (S. B. Prusiner and W. J. Hadlow, eds.), pp. 93–112, Academic Press, New York, 1979.

    Google Scholar 

  74. Manuelidis, E. E., Angelo, J. N., Gorgacz, E. J., Kim, J. H., and Manuelidis, L., Experimental Creutzfeldt-Jakob disease transmitted via the eye with infected cornea, N. Engl J. Med. 296:1334–1336 (1977).

    PubMed  CAS  Google Scholar 

  75. Manuelidis, E. E., Gorgacz, E. J., and Manuelidis, L., Interspecies transmission of Creutzfeldt-Jakob disease to the Syrian hamster with reference to clinical syndromes and strains of agent, Proc. Natl Acad. Sci. U.S.A. 75:3432–3436 (1978).

    PubMed  CAS  Google Scholar 

  76. Manuelidis, E. E., Kim, J., Angelo, J. N., and Maneulidis, L., Serial propagation of Creutzfeldt-Jakob disease in guinea pigs, Proc. Natl Acad. Sci. U.S.A. 73:223–227 (1976).

    PubMed  CAS  Google Scholar 

  77. Manuelidis, E. E., and Manuelidis, L., Experiments on maternal transmission of Creutzfeldt-Jakob disease in guinea pigs, Proc. Soc. Exp. Biol Med. 160:233–236 (1979).

    PubMed  CAS  Google Scholar 

  78. Maneulidis, E. E., Manuelidis, L., Pincus, J. H., and Collins, W. F., Transmission, from man to the hamster, of Creutzfeldt-Jakob disease with clinical recovery, Lancet 2:40–42 (1978).

    Google Scholar 

  79. Manz, H. J., Dinsdale, H. B. and Movrin, P. A. F., Progressive multifocal encephalopathy after renal transplantation, Ann. Intern. Med. 75:77–81 (1971).

    PubMed  CAS  Google Scholar 

  80. Masters, C. L., Gajdusek, D. C., Gibbs, C. J., Jr., Bernoulli, C., and Asher, D. M., Familial Creutzfeldt-Jakob disease and other familial dementias: An inquiry into possible modes of transmission of virus-induced familial disease, in: Slow Transmissible Diseases of the Nervous System, Vol. I, Clinical, Epidemiological, Genetic, and Pathological Aspects of the Spongiform Encephalopathies (S. B. Prusiner and W. J. Hadlow, eds.), pp. 143–194, Academic Press, New York, 1979.

    Google Scholar 

  81. Amyx, H. L., Gibbs, C. J., Jr., Gajdusek, D. C., and Greer, W. E., Absence of vertical transmission of subacute spongiform viral encephalopathies in experimental primates, Proc. Soc. Exp. Biol Med. 166(14):469–471 (1981).

    PubMed  CAS  Google Scholar 

  82. Masters, C. L., Gajdusek, D. C., and Gibbs, C. J., Jr., The familial occurrence of Creutzfeldt-Jakob disease and Alzheimer’s disease, Brain (in press) (1981).

    Google Scholar 

  83. Masters, C. L., Harris, J. O., and Gajdusek, D. C., Creutzfeldt-Jakob disease: Patterns of worldwide occurrence, in: Slow Transmissible Diseases of the Nervous System, Vol. I, Clinical Epidemiological, Genetic and Pathological Aspects of the Spongiform Encephalopathies (S. B. Prusiner and W. J. Hadlow, eds.), pp. 113–142, Academic Press, New York, (1979).

    Google Scholar 

  84. Mathur, W. V. and Karani, H. J., Jakob-Creutzfeldt syndrome, J. Indian Med. Assoc. 49(3):142–143 (1967).

    PubMed  CAS  Google Scholar 

  85. Matsuoka, T., Hamanaka, T., Tail, S., Tatebayashi, Y., Kijima, S., and Nishikawa, T., Subacute spongiform encephalopathy as a subtype of Creutzfeldt-Jakob disease—A report of two cases, Psychiatr. Neurol. Jpn. 72:669–690 (1970).

    CAS  Google Scholar 

  86. Matthews, W. B., Tomlinson, A. H., and Hughes, J. T., Transmission of Creutzfeldt-Jakob disease to guinea pigs, Lancet 2:752 (1979).

    PubMed  CAS  Google Scholar 

  87. May, W. W., Creutzfeldt-Jakob disease. I. Survey of the literature and clinical diagnosis, Acta Neurol. Scand. 44:1–32 (1968).

    PubMed  CAS  Google Scholar 

  88. McDonald, R., Kipps, A., andLeary, P. M., Subacute sclerosing panencephalitis in the Cape Province, S. Afr. Med. J. 48:7–9 (1974).

    PubMed  CAS  Google Scholar 

  89. Modlin, J. F., Halsey, N. A., Eddins, D. L., Conrad, J. L., Jabbour, J. T., Chien, L., and Robinson, H., Subacute sclerosing panencephalitis: A report of the national registry, J. Pediatr. 94:231–236 (1979).

    PubMed  CAS  Google Scholar 

  90. Modlin, J. F., Jabbour, J. T., Witte, J. J., and Halsey, N. A., Epidemiologic studies of measles, measles vaccine, and subacute sclerosing panencephalitis, Pediatrics 59:505–512 (1977).

    PubMed  CAS  Google Scholar 

  91. Narayan, O., Penney, J. B., Johnson, R. T., Herndon, R. M., andWeiner, L. P., Etiology of progressive multifocal leukoencephalopathy: Identification of pa-povavirus, N. Engl. J. Med. 289(24):1278–1282 (1973).

    PubMed  CAS  Google Scholar 

  92. Nathanson, N., Slow viruses and chronic disease: The contribution of epidemiology, Public Health Rep. 95:436–443 (1980).

    PubMed  CAS  Google Scholar 

  93. Nevin, S., McMenemey, W. H., Behrman, S., and Jones, D. P., Subacute spongiform encephalopathy— A subacute form of encephalopathy attributable to vascular dysfunction (spongiform cerebral atrophy), Brain 83:519–564 (1960).

    PubMed  CAS  Google Scholar 

  94. Notermans, S. L. H., Tijl, W. F. J., Willens, F. T. C., and Slooff, J. L., Experimentally induced subacute sclerosing panencephalitis in young dogs, Neurology 23:543–553 (1973).

    PubMed  CAS  Google Scholar 

  95. Padgett, B. L., and Walker, D. L., Prevalence of antibodies in human sera against JC virus, an isolate from a case of progressive multifocal leukoencephalopathy, J. Infect. Dis. 127:467–470 (1973).

    PubMed  CAS  Google Scholar 

  96. Padgett, B. L., Zu Rhein, G. M., Walker, D. L., Eckroade, R. J., andDessel, B. H., Cultivation of pa-pova-like virus from human brain with progressive multifocal leukoencephalopathy, Lancet 1:1257–1260 (1971).

    PubMed  CAS  Google Scholar 

  97. Payne, F. E., Baublis, J. V., and Itabashi, H. H., Isolation of measles virus from cell cultures of brain from a patient with subacute sclerosing panencephalitis, N. Engl. J. Med. 281:585–589 (1969).

    PubMed  CAS  Google Scholar 

  98. Pettay, O., Donner, M., Halonen, H., Palosuo, T., and Almi, A., Subacute sclerosing panencephalitis: Preceding intellectual deterioration and deviant measles serology, J. Infect. Dis. 124:439–444 (1971).

    PubMed  CAS  Google Scholar 

  99. Prusiner, S. B., and Hadlow, W. J. (eds.), Slow Transmissible Diseases of the Nervous System, Vol. I, Clinical, Epidemiological, Genetic, and Pathological Aspects of the Spongiform Encephalopathies, Academic Press New York, 1979, 472 pp.

    Google Scholar 

  100. Rewcastle, N. B., Gibbs, C. J., Jr., and Gajdusek, D. C., Transmission of familial Alzheimer’s disease to primates, J. Neuropathol. Exp. Neurol. 37:679 (1978).

    Google Scholar 

  101. Richardson, E. P., Progressive multifocal leukoencephalopathy, in: The Remote Effects of Cancer of the Nervous System (L. Brain and F. H. Norris, Jr., eds.), pp. 6–16, Grune and Stratton, New York, 1965.

    Google Scholar 

  102. Roos, R., Gajdusek, D. C., and Gibbs, C. J., Jr., The clinical characteristics of transmissible Creutzfeldt-Jakob disease, Brain 96:1–20 (1973).

    PubMed  CAS  Google Scholar 

  103. Sanders, D., and Poskanzer, D. C., Personal communication, 1973.

    Google Scholar 

  104. Schluederberg, A. E., Chavanich, S., Lipman, M. B., and Carter, C., Comparative molecular weight estimates of measles and subacute sclerosing panencephalitis virus structural polypeptides by simultaneous electrophoresis in acrylamide slab gels, Biochem. Biophysics Res. Commun. 58:547–551 (1974).

    Google Scholar 

  105. Sever, J. L., Jabbour, J. T., Beadle, E., andKrebs, H., Constant incubation period for subacute sclerosing panencephalitis (abstract), Proc. Soc. Exp. Biol. Med. (April 1974).

    Google Scholar 

  106. Sever, J. L., and Zeman, W. (eds.), Conference on Measles Virus and Subacute Sclerosing Panencephalitis, Bethesda, Maryland, September 13, 1967, Neurology 19(Part 2):30–51 (1968).

    Google Scholar 

  107. Shah, K. V., Daniel, R. W., and Warszawski, R. M., High prevalence of antibodies to BK virus, and SV- 40 related papovavirus, in residents of Maryland, J. Infect. Dis. 128:784–787 (1973).

    PubMed  CAS  Google Scholar 

  108. Shaw, C. M., Buchan, G. C., and Carlson, C. B., Myxovirus as a possible etiologic agent in subacute inclusion-body encephalitis, N. Engl. J. Med. 277:511–515 (1967).

    PubMed  CAS  Google Scholar 

  109. Siedler, H., and Malamud, N., Creutzfeldt-Jakob disease: Clinicopathologic report of 15 cases and review of the literature (with special reference to a related disorder designated as subacute spongiform encephalopathy), J. Neuropathol. Exp. Neurol. 22:381–402 (1963).

    PubMed  CAS  Google Scholar 

  110. Silverman, L., and Rubinstein, L. G., Electron microscopic observations on a case of progressive multifocal leukoencephalopathy, Acta Neuropathol. 5:215–224 (1965).

    PubMed  CAS  Google Scholar 

  111. Sotelo, J., Gibbs, C. J., Jr., and Gajdusek, D. C., Autoantibodies against axonal neurofilaments in patients with kuru and Creutzfeldt-Jakob disease, Science 210:190–193 (1981).

    Google Scholar 

  112. Sutton, R. N. P., Slow viruses and chronic disease of the central nervous system, Postgrad. Med. J. 55:143–149 (1979).

    PubMed  CAS  Google Scholar 

  113. Tateishi, J., Ohta, M., Koga, M., Sato, Y., andKuroiwa, Y., Transmission of chronic spongiform en- cephalopathy with kuru plaques from humans to small rodents, Ann. Neurol. 5:581–584 (1979).

    PubMed  CAS  Google Scholar 

  114. Tateishi, J., Sato, Y., Koga, M., Doi, H., andOhta, M., Experimental transmission of human subacute spongiform encephalopathy to small rodents. 1. Clinical and histological observations, Acta Neuropathol. 51:127–134 (1980).

    PubMed  CAS  Google Scholar 

  115. TerMeulen, V., and Hall, W. W., Slow virus infections of the nervous system: Virological, immunological and pathogenetic considerations, J. Gen. Virol. 41:1–25 (1978).

    CAS  Google Scholar 

  116. Thein, P., Mayr, A., TerMeulen, V., Koprowski, H., Kackell, M. Y., Muller, D., and Meyermann, R., Subacute sclerosing panencephalitis: Transmission of the virus to calves and lambs, Arch. Neurol. 27:540–548 (1972).

    PubMed  CAS  Google Scholar 

  117. Thormar, H., Jervis, G. A., Karl, S. C., and Brown, H. R., Passage in ferrets of encephalitogenic cell-associated measles virus isolated from brain of a patient with subacute sclerosing panencephalitis, J. Infect. Dis. 127:678–685 (1973).

    PubMed  CAS  Google Scholar 

  118. Traub, R. D., Gajdusek, D. C, and Gibbs, C. J., Jr., Transmissible virus dementia: The relation of transmissible spongiform encephalopathy to Creutzfeldt-Jakob disease, in: Aging, Dementia and Cerebral Function (M. Kinsbourne and L. Smith, eds.), pp. 91–146, Spectrum, New York, 1977.

    Google Scholar 

  119. Vernon, M. L., Horta-Barbosa, L., Fuccillo, D. A., Sever, J. L., Baringer, J. R., andBirnbaum, G., Viruslike particles and nucleoprotein-type filaments in brain tissue from two patients with Creutzfeldt-Jakob disease, Lancet 1:964–967 (1970).

    PubMed  CAS  Google Scholar 

  120. Walker, D. L., Progressive multifocal leukoenceph-alopathy: An opportunistic viral infection of the central nervous system, in: Handbook of Clinical Neurology, Vol. 34, Infections of the Nervous System (P. J. Vinken and G. W. Bruyn, eds.), pp. 307–329, Elsevier/ North-Holland, New York, 1978.

    Google Scholar 

  121. Walker, D. L., Personal communication, 1981.

    Google Scholar 

  122. Walker, D. L., Padgett, B. L., ZuRhein, G. M., Albert, A. E., and Marsh, R. F., Human papovavirus (JC): Induction of brain tumors in hamsters, Science 181:674–676 (1973).

    PubMed  CAS  Google Scholar 

  123. Wear, D., andRapp, F., Latent measles virus infection of the hamster central nervous system, J. Immunol. 107:1593–1598 (1971).

    PubMed  CAS  Google Scholar 

  124. Wechsler, S. L., Weiner, H. L., and Fields, B. N., Immune response in subacute sclerosing panencephalitis: Reduced antibody response to the matrix protein of measles virus, J. Immunol. 123:884–889 (1979).

    PubMed  CAS  Google Scholar 

  125. Weiner, L. P., Herndon, R. M., Narayan, O., Johnson, R. T., Shah, K., Rubinstein, L. J., Preziosi, T. J., and Conley, F. K., Isolation of virus related to SV- 40 from patients with progressive multifocal leukoen- cephalopathy, N. Engl. J. Med. 286:385–390 (1972).

    PubMed  CAS  Google Scholar 

  126. Weiner, L. P., Narayan, O., Penney, J. B., Jr., Herndon, R. M., Feringa, E. R., Tourtellotte, W. W., and Johnson, R. T., Papovavirus of JC type in progressive multifocal leukoencephalopathy Arch. Neurol. 29:1–3 (1973).

    PubMed  CAS  Google Scholar 

  127. Whitaker, J. N., Sever, J. L., and Engel, W. K., Subacute sclerosing panencephalitis in only one of identical twins, N. Engl. J. Med. 287:864–866 (1972).

    PubMed  CAS  Google Scholar 

  128. Zu Rhein, G. M., Association of papova-virions with a human demyelinating disease (progressive multifocal leukoencephalopathy), Prog. Med. Virol. 11:185–247 (1969).

    CAS  Google Scholar 

  129. Zu Rhein, G., and Varakis, J., Progressive multifocal leucoencephalopathy in a renal allograft regiment, N. Engl. J. Med. 291:798 (1974).

    PubMed  CAS  Google Scholar 

  130. Zu Rhein, G. M., Padgett, B. L., Walker, D. L., Chun, R. W. M., Horowitz, S. D., and Hong, R., Letter to the editor: Progressive multifocal leukoencephalopathy in a child with severe combined immunodeficiency N. Engl. J. Med. 299:256–257 (1978).

    Google Scholar 

Suggested Reading

  • Gajdusek, D. C, Kuru and Creutzfeldt-Jacob disease: Experimental models of non-inflammatory degenerative slow virus disease of the central nervous system, Ann. Clin. Res. 5:254–261 (1973).

    PubMed  CAS  Google Scholar 

  • Gajdusek, D. C, Kuru in the New Guinea highlands, in: Tropical Neurology, Vol. 29 (J. D. Spillane, ed.), pp. 376–383, Oxford University Press, London, 1973.

    Google Scholar 

  • Gajdusek, D. C, Unconventional viruses and the origin and disappearance of kuru, Science 197:943–960 (1977).

    PubMed  CAS  Google Scholar 

  • Gajdusek, D. C, Slow infections with unconventional viruses, Harvey Led. 72:283–353 (1978).

    CAS  Google Scholar 

  • Gajdusek, D. C., and Gibbs, C. J., Jr., Familial and sporadic chronic neurological degenerative disorders transmitted from man to primates, in: Advances in Neurology, Vol. 10, Primate Models of Neurological Disorders, B. S. Meldrum and C. D. Marsden eds.), pp. 291–375, Raven Press, New York, 1975.

    Google Scholar 

  • Gibbs, C. J., Jr., and Gajdusek, D. C, Transmission and characterization of the agents of spongiform virus encephalopathies, kuru, Creutzfeldt-Jakob disease, scrapie and mink encephalopathy, in: Immunological Disorders of the Nervous System, Vol. XLIX (L. P. Rowland, ed.), Res. Publ. A.R.N.M.D., pp. 383–410, Williams and Wilkins, Baltimore, 1971.

    Google Scholar 

  • Gibbs, C. J., Jr., and Gajdusek, D. C., Atypical viruses as the cause of sporadic, epidemic, and familial chronic diseases in man, in: Slow Viruses and Human Diseases, Vol. 10, Perspectives in Virology (M. Pollard, ed.), pp. 161–198, Raven Press, New York, 1978.

    Google Scholar 

  • Gibbs, C. J., Jr., Gajdusek, D. C, and Amyx, H. L., Strain variation in the viruses of Creutzfeldt-Jakob disease and kuru, in: Slow Transmissible Diseases of the Nervous System, Vol. 2 (S. B. Prusiner and W. J. Hadlow, eds.), pp. 87–110, Academic Press, New York, 1979.

    Google Scholar 

  • Masters, C. L., Harris, J. O., Gajdusek, D. C, Gibbs, C. J., Jr., Bernoulli, C., and Asher, D. M., Creutzfeldt-Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering, Ann. Neurol. 5:177–188 (1979).

    PubMed  CAS  Google Scholar 

  • Narayan, O., Penny, J. B., Johnson, R. T., Herndon, R. M., and Weiner, L. P., Etiology of progressive multifocal leucoencephalopathy: Identification of papovavirus, N. Engl. J. Med. 289(14):1278–1282 (1973).

    PubMed  CAS  Google Scholar 

  • Prusiner, S. B., and Hadlow, W. J. (eds.), Slow Transmissible Diseases of the Nervous System, Vol. 1, Clinical, Epidemiological, Genetic, and Pathological Aspects of the Spongiform Encephalopathies, Academic Press, New York, 1979, 472 pp.

    Google Scholar 

  • Walker, D. L., Progressive multifocal leukoencephalopathy: An opportunistic viral infection of the central nervous system, in: Handbook of Clinical Neurology, Vol. 34, Infections of the Nervous System (P. J. Vinken and g. W. Bruyn, eds.), pp. 307–329, Elsevier/North-Holland, New York, 1978.

    Google Scholar 

  • Weiner, L. P., Johnson, R. T., and Herdon, R. M., Viral infections and demy elina ting diseases, N. Engl. J. Med. 288:1103–1110 (1973).

    PubMed  CAS  Google Scholar 

  • Zu Rhein, G. M., Association of papova-virions with a human demyelinating disease (progressive multifocal leucoencephalopathy), Prog. Med. Virol. 11:185–247 (1969).

    CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1982 Plenum Publishing Corporation

About this chapter

Cite this chapter

Brody, J.A., Gibbs, C.J. (1982). Chronic Neurological Diseases. In: Evans, A.S. (eds) Viral Infections of Humans. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-3237-4_27

Download citation

  • DOI: https://doi.org/10.1007/978-1-4613-3237-4_27

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-3239-8

  • Online ISBN: 978-1-4613-3237-4

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics