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Chronic Neurological Diseases

Subacute Sclerosing Panencephalitis,Progressive Multifocal Leukoencephalopathy, Kuru,Creutzfeldt—Jakob Disease
  • Jacob A. Brody
  • Clarence Joseph GibbsJr.

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.

Keywords

Measle Virus Subacute Sclerosing Panencephalitis Chronic Neurological Disease Measle Infection Presenile Dementia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. 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, p. 13, Barcelona, September 9–14, 1973, Int. Congr. Ser No. 319, Excerpta Medica, Amsterdam, 1973.Google Scholar
  2. 2.
    Alpers, M., Epidemiological changes in kuru, 1957 to 1963, in: Slow Latent and Ternpurate Virus Infections Nindb Monogr. No. 2 (D. C. Gajdusek C. J. Gibbs JR., and M. Alpers 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. 3.
    Alters M. P., Kuru: Implications of its transmissibility for the interpretation of its changing epidemiologic pattern, in: The Central Nervous System Some Experimental Models of Neurological Diseases, (O. T. Baileyand D. E. Smith 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. 3a.
    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. Prusinerand W. J. Hadlow pp. 67–90, Academic Press, New York, 1979.Google Scholar
  5. 4.
    Baguley D. M., and Glasgow G. L., Subacute sclerosing panencephalitis and Salk vaccine, Lancet 2: 763–765 (1973).PubMedGoogle Scholar
  6. 4a.
    Bernoulli C., Siegfried J., Baumgartner C., Regli F., Rabinowicz T., Gajdusek D. C., and Gibbs C. J., JR., Danger of accidental person-to-person trans-mission of Creutzfeldt-Jakob disease by surgery, Lancet 1: 478–479 (1979).Google Scholar
  7. 5.
    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).PubMedGoogle Scholar
  8. 6.
    Bolton C. F., Rozdilsky B., Primary progressive multifocal leukoencephalopathy, Neurology 21: 72–77 (1971).PubMedGoogle Scholar
  9. 7.
    Brody J. A., Detels R., Subacute sderosing panencephalitis: A zoonosis following aberrant measles: Hypothesis, Lancet 2: 500–501 (1970).PubMedGoogle Scholar
  10. 8.
    Brody J. A., Detels R., sever J. L., Measles-antibody titers in sibships of patients with subacute sclerosing panencephalitis and controls, Lancet 1: 177–178 (1972).PubMedGoogle Scholar
  11. 8a.
    Brown F., Cathala F., Creutzfeldt-Jakob disease in France, in: Slow Transmissible Diseases of the Nervous System Vol. I, Clinical Epidemiological Genetic andPathological Aspects of the Spongiform Encephalopathies (S. B. PrusinerW. J. Hadlow pp. 213–227, Academic Press, New York, 1979.Google Scholar
  12. 8b.
    Brown P., Cathala F., 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).PubMedGoogle Scholar
  13. 9.
    Brown P., Hooks J., Roos R., Gajdusek D. C., 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).Google Scholar
  14. 9a.
    Brown P., Tsai T.,Gajdusek C., Seroepidemiology of human papovaviruses, Am. J. Epidemiol 102: 331–340 (1975).PubMedGoogle Scholar
  15. 10.
    Brun A., Nordenfeldt E.,Kjellen L., Aspects on the variability of progressive multifocal leukoencephalopathy, Acta Neuropathol. 24: 232–243 (1973).PubMedGoogle Scholar
  16. 11.
    Byington D. P.,Burnstein T., Measles encephalitis produced in suckling rats, Exp. Mol. Pathol 19: 36–43 (1973).PubMedGoogle Scholar
  17. 12.
    Canal N.,Torck P., An epidemiological study of subacute sclerosing leucoencephalitis in Belgium, J. Neurol. Sci 1: 380–389 (1964).Google Scholar
  18. 12a.
    Candelas 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. 13.
    Canelas H. M., Juliao O. F., Lefevre A. B., LA-Martinede ASSIS, Tognola W. A., De Jorge F. B., Fonseca, L. C., Xavier-Lima, A., Subacute sclerosing panencephalitis: epidemiological, clinical and biochemical study of 31 cases, Arch. Neuro-Psychiatr. (Sao Paulo) 25: 255–268 (1967).Google Scholar
  20. 14.
    Castaigne P., Rondot P., Escourelle R., Ridadeau D., Lumas J. L., Cathala F., Hauw J.J., Leucoencephalopathe multifocale progressive et “gliomes” multiples, Rev. Neurol 130: 379–393 (1974).PubMedGoogle Scholar
  21. 15.
    Connolly J. H., Haire M., Hadden D. S. M., Measles immunoglobulins in subacute sclerosing panencephalitis, Br. Med. J 1: 23–25 (1971).PubMedGoogle Scholar
  22. 16.
    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).PubMedGoogle Scholar
  23. 17.
    Detels R., Brody J. A., Mcnew J., Edgar A. H., Further epidemiologic studies of subacute sclerosing panecephalitis, Lancet 2: 11–14 (1973).PubMedGoogle Scholar
  24. 18.
    Donner M., Halonen H., Haltia M., Subakuutti sklerosoiva panenkefaliitti, Duodecim 85: 541–553 (1969).PubMedGoogle Scholar
  25. 19.
    Duffy P., Wolf J., Collins G., Devoe A. G., Streeten B., Cowen D., Person-to-person transmission of Creutzfeldt-Jakob disease, N. Engl. J. Med 299: 692–693 (1974).Google Scholar
  26. 20.
    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, 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. 21.
    Fermaglich J Hardman J. M., and Earle K. M., Spontaneous progressive multifocal leukoencephalopathy, Neurology 20:479–484 (1970).PubMedGoogle Scholar
  28. 22.
    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. 22a.
    Gajdusek D. C., Slow infections with unconventional viruses, Harvey Lect. 72: 283–353 (1978).PubMedGoogle Scholar
  30. 23.
    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, pp. 288–299, Cold Spring Harbor Press, Cold Spring Harbor, New York, 1973.Google Scholar
  31. 24.
    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. 25.
    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 Dis-orders ( B. S. Meldrum and C D. Marsden, pp. 291–375, Raven Press, New York, 1975.Google Scholar
  33. 26.
    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).PubMedGoogle Scholar
  34. 27.
    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 Köhlmeier-Degos, in: Proceedings of the Tenth Internationational Congress on Neurology (A. Subirana and J. Burrows.), pp. 390–392, Int. Cong. Ser No. 319, Excerpta Medica, Amsterdam, 1973.Google Scholar
  35. 28.
    Gajdusek, D. C., Gibbs, C. J., JR., and Alpers, M.( 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. 28a.
    Gajdusek, D. C., Gibbs, C J., JR., Asher, D. M., Brown, P., Duvan, A., Hoffman, P., Nemo, G., Roh-Wer, 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).PubMedGoogle Scholar
  37. b. 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. 29.
    Gardner, S. DPrevalence in England of antibody to human polyomavirus (B.K.), Br. Med. J 1: 77–78 (1973).PubMedGoogle Scholar
  39. 30.
    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).PubMedGoogle Scholar
  40. 30a.
    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. 31.
    Gerson K. L Haslam R. H. A., Subtle immunologic abnormalities in SSPE, N. Engl. J. Med 285:78–82 (1971).Google Scholar
  42. 31a.
    Gibbs C. J., JR., Amyx, H. L., Bacote, A., Masters,C. L., and GajdusekD. C., Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates, J. Infect. Dis 142: 205–208 (1980).Google Scholar
  43. 32.
    Gibbs, C. J., JR.,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. 33.
    Gibbs, C. J., JR.,Gajdusek, D. C., Experimental subacute spongiform virus encephalopathies in primates and other laboratory animals, Science 182: 67–68 (1973).PubMedGoogle Scholar
  45. 34.
    Gibbs C. J., JR., and Gajdusek D. C., Biology of kuru and Creutzfeldt-Jakob disease, in: Slow Virus Diseases (W. Zeman E. H. Lennette pp. 39–48, Williams and Wilkins, Baltimore, 1973.Google Scholar
  46. 35.
    Gibbs, C. J., JR., Gajdusek, D. C., Asher, D. M., Al-Pers, M. P., Beck, E., Daniel, P. M., Matthews,W. B., Creutzfeldt-Jakob disease (subacute spongiform encephalopathy): Transmission to the chimpanzee, Science 161: 388–389 (1968).PubMedGoogle Scholar
  47. 35a.
    Goldberg H., Alter, M., and Kahana, E., The Ly-bian 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, pp. 195–211, Academic Press, New York, 1979.Google Scholar
  48. 36.
    Hadlow W. J., Scrapie and kuru, Lancet 2: 289–290 (1959).Google Scholar
  49. 36a.
    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).PubMedGoogle Scholar
  50. 36b.
    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).PubMedGoogle Scholar
  51. 36c.
    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).PubMedGoogle Scholar
  52. 37.
    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. 38.
    Herzberg L Herzberg B W., Gibbs, C. J., JR., Sullivan, W., Amyx H and Gajdusek, D. C., Creutz-feldt-Jakob disease: Hypothesis for high incidence in Libyan Jews in Israel, Science 186:848 (1974).Google Scholar
  54. 39.
    Hirano, A., Personal communication, 1974.Google Scholar
  55. 39a.
    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).Google Scholar
  56. 40.
    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).PubMedGoogle Scholar
  57. 41.
    Hornabrook R W., Kuru-A subacute cerebellar degeneration: The natural history and clinical features, Brain 91:53–74 (1968).PubMedGoogle Scholar
  58. 42.
    Horta-Barbosa L., Subacute sclerosing panencephalitis: Isolation of suppressed measles virus from lymph node biopsies, Science 173: 840–841 (1971).PubMedGoogle Scholar
  59. 43.
    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).PubMedGoogle Scholar
  60. 44.
    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).Google Scholar
  61. 45.
    Jabbour J T., and Sever J. L., Serum measles antibody titers in patients with subacute sclerosing pan-encephalitis, compared with parents and siblings, J. Pediatr 73:905–907 (1968).PubMedGoogle Scholar
  62. 46.
    Jellinger, V. K., Seitelberger, F., Heiss, W. D., and Holczabek, WKonjugale Form der subakutem spongiosen Enzephalopathie (Jakob-Creutzfeldt-Erkrankung), Wien. Klin. Wochenschr 84: 245–249 (1972).PubMedGoogle Scholar
  63. 47.
    Joasoo, A., and Wolfenden, W. HSubacute spongiform encephalopathy, Med. J. Aust 1: 354–356 (1968).PubMedGoogle Scholar
  64. 48.
    Kahana, E., Alter, M., Braham, J., and Sofer, DCreutzfeldt-Jakob disease: A focus among Libyan Jews in Israel, Science 183: 90–91 (1974).PubMedGoogle Scholar
  65. 48a.
    Katz, M., and Koprowski, HThe significance of failure to isolate infectious viruses in cases of subacute sclerosing panencephalitis, Arch. Gesamte Virusforsch 41: 390–393 (1973).PubMedGoogle Scholar
  66. 49.
    Katz M., Rorke L. B., Masland W. S., Brodano, G. B., and Koprowski H., Subacute sclerosing pan-encephalitis: Isolation of a virus encephalitogenic for ferrets, J. Infect. Dis 121: 188–195 (1970).PubMedGoogle Scholar
  67. 50.
    Kirschbaum, W. R Jakob-Creutzfeldt Disease American Elsevier, New York, 1968, 251 pp.Google Scholar
  68. 51.
    Koprowski H., Interaction between papova-like virus and paramyxovirus in human brain cells: A hypothesis, Nature (London) 225: 1045–1047 (1970).PubMedGoogle Scholar
  69. a. Kuroda, Y., Gibbs, C. J., JR., Amyx, H. L., and Gajdusek, D. CThe pathogenesis of Creutzfeldt-Jakob disease in the mouse, Infect. Immun (in press) (1981).Google Scholar
  70. 52a.
    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).PubMedGoogle Scholar
  71. 52b.
    Machamer, C. E., Hayes, E. C., Gollobin, S. D., Westfall, L. K., and Zweerink, H. JAntibodies against the measles matrix polypeptide after clinical infection and vaccination, Infect. Immun 27: 817–825 (1980).PubMedGoogle Scholar
  72. 52c Malmgren, R., Kurland, L., Mokri, B., and Kurtzke J., The epidemiology of Creutzfeldt-Jakob disease, in: Slow Transmissible Diseases of the Nervous SystemVol. I Clinical Epidemiological Genetic and Pathological Aspects of the Spongiform Encephalopathies(S. B. Prusiner and W. J. Hadlow, pp. 93–112, Academic Press, New York, 1979.Google Scholar
  73. 52d.
    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).PubMedGoogle Scholar
  74. 52e.
    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).PubMedGoogle Scholar
  75. 52f.
    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).PubMedGoogle Scholar
  76. 52g.
    Manuelidis, E. E., and Manuelidis, LExperiments on maternal transmission of Creutzfeldt-Jakob disease in guinea pigs, Proc. Soc. Exp. Biol. Med 160: 233–236 (1979).PubMedGoogle Scholar
  77. 52h.
    Maneulidis, E. E., Manuelidis, L., Pincus, J. H., and Collins, W. FTransmission, from man to the hamster, of Creutzfeldt-Jakob disease with clinical recovery, Lancet 2: 40–42 (1978).Google Scholar
  78. 53.
    Manz, H. J., Dinsdale, H. B. and Movrin, P. A. FProgressive multifocal encephalopathy after renal transplantation, Ann. Intern. Med 75: 77–81 (1971).PubMedGoogle Scholar
  79. 53a.
    Masters, C. L., Gajdusek, D. C., Gibbs, C. J., JRBernoulli, 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, pp. 143–194, Academic Press, New York, 1979.Google Scholar
  80. 53b.
    Amyx, H. L., Gibbs, C. J., JR., Gajdusek, D. C., and Greer, W. EAbsence of vertical transmission of subacute spongiform viral encephalopathies in experimental primates, Proc. Soc. Exp. Biol. Med 166 (14): 469–471 (1981).PubMedGoogle Scholar
  81. 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
  82. 53d.
    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, pp. 113–142, Academic Press, New York, (1979).Google Scholar
  83. 54.
    Mathur, W. V. and Karani, H. JJakob-Creutzfeldt syndrome, J. Indian Med. Assoc 49 (3): 142–143 (1967).PubMedGoogle Scholar
  84. 55.
    Matsuoka, T., Hamanaka, T., Tail, S., Tatebayashi, Y., Kijima, S., and Nishikawa, T., Subacute spongi-form encephalopathy as a subtype of Creutzfeldt-Jakob disease-A report of two cases, Psychiatr. Neurol. Jpn 72: 669–690 (1970).Google Scholar
  85. 55a.
    Matthews, W. B., Tomlinson, A. H., and Hughes,J. TTransmission of Creutzfeldt-Jakob disease to guinea pigs, Lancet 2: 752 (1979).PubMedGoogle Scholar
  86. 56.
    May, W. W Creutzfeldt-Jakob disease. I. Survey of the literature and clinical diagnosis, Acta Neurol. Scand 44: 1–32 (1968).PubMedGoogle Scholar
  87. 57.
    Mcdonald, R., Kipps, A., and Leary, P. MSubacute sclerosing panencephalitis in the Cape Province, S. Afr. Med. J 48: 7–9 (1974).Google Scholar
  88. 57a.
    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).PubMedGoogle Scholar
  89. 57b.
    Modlin, J. F., Jabbour, J. T., Witte, J. J., AND Halsey,N. AEpidemiologic studies of measles, measles vaccine, and subacute sclerosing panencephalitis, Pediatrics 59: 505–512 (1977).PubMedGoogle Scholar
  90. 58.
    Narayan, O., Penney, J. B., Johnson, R. T., Herndon, R. M., and Weiner, L. PEtiology of progressive multifocal leukoencephalopathy: Identification of papovavirus, N. Engl. J. Med 289 (24): 1278–1282 (1973).PubMedGoogle Scholar
  91. 58a.
    Nathanson, NSlow viruses and chronic disease: The contribution of epidemiology, Public Health Rep. 95: 436–443 (1980).PubMedGoogle Scholar
  92. 59.
    Nevin, S., Mcmenemey, W. H., Behrman, S., and Jones, D. PSubacute spongiform encephalopathyA subacute form of encephalopathy attributable to vascular dysfunction (spongiform cerebral atrophy), Brain 83: 519–564 (1960).PubMedGoogle Scholar
  93. 60.
    Notermans, S. L. H., Tijl, W. F. J., Willens, F. T.C., and Slooff, J. LExperimentally induced subacute sclerosing panencephalitis in young dogs, Neurology 23: 543–553 (1973).PubMedGoogle Scholar
  94. 61.
    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).PubMedGoogle Scholar
  95. 62.
    Padgett, B. L., ZU Rhein, G. M., Walker, D. L., Eckroade, R. J., and Dessel, B. HCultivation of pa-pova-like virus from human brain with progressive multifocal leukoencephalopathy, Lancet 1: 1257–1260 (1971).PubMedGoogle Scholar
  96. 63.
    Payne, F. E., Baublis, J. V., and Itabashi, H. HIsolation of measles virus from cell cultures of brain from a patient with subacute sclerosing panencephalitis, N. Engl. J. Med 281: 585–589 (1969).PubMedGoogle Scholar
  97. 63a.
    Pettay, O., Donner, M., Halonen, H., Palosuo, T.,and Almi, ASubacute sclerosing panencephalitis: Preceding intellectual deterioration and deviant measles serology, J. Infect. Dis 124: 439 444 (1971).Google Scholar
  98. 63b Prusiner, S. B., Andhadlow, W. J( Slow Transmissible Diseases of the Nervous SystemVol. I, Clinical Epidemiological Genetic and Pathological Aspects of theSpongiform EncephalopathiesAcademic Press, New York, 1979, 472 pp.Google Scholar
  99. 63c.
    Rewcastle, N. B., Gibbs, C. J., JR., and Gajdusek, D.CTransmission of familial Alzheimer’s disease to primates, J. Neuropathol. Exp. Neurol 37: 679 (1978).Google Scholar
  100. 64.
    Richardson, E. PProgressive multifocal leukoencephalopathy, in: The Remote Effects of Cancer of the Nervous System (L. Brain and F. H. Norris, JR., pp. 6–16, Grune and Stratton, New York, 1965.Google Scholar
  101. 65.
    Roos, R., Gajdusek, D. C., and Gibbs, C. J., JRThe clinical characteristics of transmissible Creutzfeldt-Jakob disease, Brain 96: 1–20 (1973).PubMedGoogle Scholar
  102. 66.
    Sanders, D., and Poskanzer, D. CPersonal communication, 1973.Google Scholar
  103. 66a.
    Schluederberg, A. E., Chavanich, S., Lipman, M. B., and Carter, CComparative 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
  104. 67.
    Sever, J. L., Jabbour, J. T., Beadle, E., and Krebs, HConstant incubation period for subacute sclerosing panencephalitis (abstract), Proc. Soc. Exp. Biol. Med. (April 1974).Google Scholar
  105. 68.
    Sever, J. L., and Zeman, WConference on Measles Virus and Subacute Sclerosing Panencephalitis, Bethesda, Maryland, September 13, 1967, Neurology19 Part 2 30–51 1968Google Scholar
  106. 69.
    Shah, K. V., Daniel, R. W., and Warszawski, R. MHigh prevalence of antibodies to BK virus, and SV-40 related papovavirus, in residents of Maryland, J. Infect. Dis 128: 784–787 (1973).PubMedGoogle Scholar
  107. 70.
    Shaw, C. M., Buchan, G. C., and Carlson, C. BMyxovirus as a possible etiologic agent in subacute inclusion-body encephalitis, N. Engl. J. Med 277: 511–515 (1967).PubMedGoogle Scholar
  108. 71.
    Siedler, H., and Malamud, NCreutzfeldt-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).PubMedGoogle Scholar
  109. 72.
    Silverman, L., and Rubinstein, L. GElectron mi-croscopic observations on a case of progressive multifocal leukoencephalopathy, Acta Neuropathol. 5: 215–224 (1965).PubMedGoogle Scholar
  110. 72a.
    Sotelo, J., Gibbs, C. J., JR., and Gajdusek, D. CAutoantibodies against axonal neurofilaments in patients with kuru and Creutzfeldt-Jakob disease, Science 210: 190–193 (1981).Google Scholar
  111. 73a.
    Surron, R. N. PSlow viruses and chronic disease of the central nervous system, Postgrad. Med. J 55: 143–149 (1979).Google Scholar
  112. 73b.
    Tateishi, J., Ohta, M., Koga, M., Sato, Y., and Ku-RoIwA, YTransmission of chronic spongiform en-cephalopathy with kuru plaques from humans to small rodents Ann. Neurol 5: 581–584 (1979).PubMedGoogle Scholar
  113. 73c.
    Tatelshi, J., Sato, Y., Koga, M., Doi, H., and Ohta,MExperimental transmission of human subacute spongiform encephalopathy to small rodents. 1. Clinical and histological observations, Acta Neuropathol. 51: 127–134 (1980).Google Scholar
  114. 73d.
    Ter Meulen, V., and Hall, W. WSlow virus infections of the nervous system: Virological, immunological and pathogenetic considerations, J. Gen. Virol 41: 1–25 (1978).PubMedGoogle Scholar
  115. 74.
    Thew, P., Mayr, A., Ter Meulen, V., Korrowski, 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).Google Scholar
  116. 75.
    Thormar, H., JERVIS, G. A., Karl, S. C., and Brown, H. RPassage 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).PubMedGoogle Scholar
  117. 76.
    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, pp. 91–146, Spectrum, New York, 1977.Google Scholar
  118. 77.
    Vernon, M. L., Horta-Barbosa, L., Fuccillo, D. A., Sever, J. L., Baringer, J. R., and Birnbaum, G Virus-like particles and nucleoprotein-type filaments in brain tissue from two patients with Creutzfeldt-Jakob disease, Lancet 1: 964–967 (1970).PubMedGoogle Scholar
  119. 77a.
    Walker, D. LProgressive 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 pp. 307–329, Elsevier/ North-Holland, New York, 1978.Google Scholar
  120. 77b.
    Walker, D. LPersonal communication, 1981.Google Scholar
  121. 78.
    Walker, D. L., Padgett, B. L., ZU Rhein, G. M., Albert, A. E., and Marsh, R. FHuman papovavirus (JC): Induction of brain tumors in hamsters, Science 181: 674–676 (1973).PubMedGoogle Scholar
  122. 79.
    Wear, D., and Rapp, FLatent measles virus infection of the hamster central nervous system, J. Immunol 107: 1593–1598 (1971).PubMedGoogle Scholar
  123. 79a.
    Wechsler, S. L., Weiner, H. L., and Fields, B. NImmune response in subacute sclerosing panencephalitis: Reduced antibody response to the matrix protein of measles virus, J. Immunol 123: 884–889 (1979).PubMedGoogle Scholar
  124. 80.
    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 leukoencephalopathy, N. Engl. J. Med 286: 385–390 (1972).PubMedGoogle Scholar
  125. 81.
    Weiner, L. P., Narayan, OPenney, J. B., JR., Hern-Google Scholar
  126. Don R M., Feringa, E. R., Tourtellotte, W. W., and Johnson, R. TPapovavirus of JC type in progressive multifocal leukoencephalopathy, Arch. Neurol 29:1–3 (1973).Google Scholar
  127. 82.
    Whrraker, 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).Google Scholar
  128. Zu Rhein G. MAssociation of papova-virions with a human demyelinating disease (progressive multi-focal leukoencephalopathy), Prog. Med. Virol.11:185–247 (1969).Google Scholar
  129. 84.
    Zu Rhein, G., and Varakis, JProgressive multifocal leucoencephalopathy in a renal allograft regiment, N. Engl. J. Med 291: 798 (1974).Google Scholar
  130. 85.
    Zu Rhein, G. M., Padgett, B. L., Walker, D. L., Chun, R. W. M., Horowitz, S. D., and Hong, RLetter 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

  1. Gajdusek, D. C., Kuru andCreutzfeldt-Jacob disease: Experimental models of non-inflammatory degenerative slow virus disease of the central nervous system, Ann. Clin. Res 5: 254–261 (1973).PubMedGoogle Scholar
  2. Gajdusek, D. CUnconventional viruses and the origin and disappearance of kuru, Science 197: 943–960 (1977).PubMedGoogle Scholar
  3. Gajdusek, D. CSlow infections with unconventional viruses, Harvey Lect. 72: 283–353 (1978).PubMedGoogle Scholar
  4. Gajdusek, D. C., and Gibbs, C. J., JRFamilial 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 pp. 291–375, Raven Press, New York, 1975.Google Scholar
  5. Gibbs, C. J., JR., and Gajdusek, D. CTransmission 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. Row-Land, ed.), Res. Publ. A.R.N.M.D., pp. 383–410, Williams and Wilkins, Baltimore, 1971.Google Scholar
  6. Gibbs, C. J., JR., and Gajdusek, D. CAtypical 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,), pp. 161–198, Raven Press, New York, 1978.Google Scholar
  7. Gibbs, C. J., JR., Gajdusek, D. C., and Amyx, H. LStrain 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, pp. 87–110, Academic Press, New York, 1979.Google Scholar
  8. 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).PubMedGoogle Scholar
  9. Prusiner S. B., and Hadlow W. J. ( 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
  10. 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. WBruyn,), pp. 307–329, Elsevier/North-Holland, New York, 1978.Google Scholar
  11. Weiner L. P Johnson, R. T., and Herdon, R. M., Viral infections and demyelinating diseases, N. Engl. J. Med 288:1103–1110 (1973).PubMedGoogle Scholar
  12. Zu Rhein, G. M., Association of papova-virions with a human demyelinating disease (progressive multifocal leucoencephalopathy), Prog. Med. Virol 11: 185–247 (1969).PubMedGoogle Scholar

Copyright information

© Plenum Publishing Corporation 1984

Authors and Affiliations

  • Jacob A. Brody
    • 1
  • Clarence Joseph GibbsJr.
    • 2
  1. 1.Epidemiology, Demography, Biometry, National Institute on AgingNational Institutes of HealthBethesdaUSA
  2. 2.Laboratory of Central Nervous System Studies, National Institute of Neurological and Communicative Disorders and StrokeNational Institutes of HealthBethesdaUSA

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