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. 1980 Jan-Feb;53(1):101–107.

Types of immunological failure in the "slow-virus" encephalopathies and multiple sclerosis.

J Booss
PMCID: PMC2595833  PMID: 6990633

Abstract

The pathogenesis of the slow virus encephalopathies and multiple sclerosis is reviewed within the framework of the immune response. The diseases are analyzed for the component of the immune response that appears to be crucial to the host's failure to control the disease. Thus, the absence of an immune response in the spongiform encephalopathies appears to reflect a failure of antigen recognition. Progressive multifocal leukoencephalopathy (PML), subacute sclerosing panencephalitis (SSPE), and progressive rubella panencephalitis (PRP) may result principally from a failure of effector mechanisms. In PML the failure usually occurs within the setting of an immunosuppressive illness. Conversely, in SSPE and PRP the effector failure seems to result from the nature of the host-virus interaction itself. Finally, evidence is accumulating that a defect of immunoregulation plays a significant role in multiple sclerosis.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Adams C. W. The onset and progression of the lesion in multiple sclerosis. J Neurol Sci. 1975 Jun;25(2):165–182. doi: 10.1016/0022-510x(75)90138-0. [DOI] [PubMed] [Google Scholar]
  2. Ahmed A., Strong D. M., Sell K. W., Thurman G. B., Knudsen R. C., Wistar R., Jr, Grace W. R. Demonstration of a blocking factor in the plasma and spinal fluid of patients with subacute sclerosing panencephalitis. I. Partial characterization. J Exp Med. 1974 Apr 1;139(4):902–924. doi: 10.1084/jem.139.4.902. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Asher D. M., Gibbs C. J., Jr, Gajdusek D. C. Pathogenesis of subacute spongiform encephalopathies. Ann Clin Lab Sci. 1976 Jan-Feb;6(1):84–103. [PubMed] [Google Scholar]
  4. Compston D. A., Batchelor J. R., McDonald W. I. B-lymphocyte alloantigens associated with multiple sclerosis. Lancet. 1976 Dec 11;2(7998):1261–1265. doi: 10.1016/s0140-6736(76)92027-4. [DOI] [PubMed] [Google Scholar]
  5. Connolly J. H., Allen I. V., Hurwitz L. J., Millar J. H. Measles-virus antibody and antigen in subacute sclerosing panencephalitis. Lancet. 1967 Mar 11;1(7489):542–544. doi: 10.1016/s0140-6736(67)92117-4. [DOI] [PubMed] [Google Scholar]
  6. Delmotte P., Gonsette R. Biochemical findings in multiple sclerosis IV. Isoelectric focusing of the CSF gamma globulins in multiple sclerosis (262 cases) and other neurological diseases (272 cases). J Neurol. 1977 Apr 28;215(1):27–37. doi: 10.1007/BF00312547. [DOI] [PubMed] [Google Scholar]
  7. Esiri M. M. Immunoglobulin-containing cells in multiple-sclerosis plaques. Lancet. 1977 Sep 3;2(8036):478–478. doi: 10.1016/s0140-6736(77)91603-8. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Ewan P. W., Lachmann P. J. Demonstration of T-cell and K-cell cytotoxicity against measles-infected cells in normal subjects, multiple sclerosis and subacute sclerosing panencephalitis. Clin Exp Immunol. 1977 Oct;30(1):22–31. [PMC free article] [PubMed] [Google Scholar]
  9. Fujinami R. S., Oldstone M. B. Antiviral antibody reacting on the plasma membrane alters measles virus expression inside the cell. Nature. 1979 Jun 7;279(5713):529–530. doi: 10.1038/279529a0. [DOI] [PubMed] [Google Scholar]
  10. Gajdusek D. C., Gibbs C. J., Jr, Asher D. M., Brown P., Diwan A., Hoffman P., Nemo G., Rohwer R., White L. Precautions in medical care of, and in handling materials from, patients with transmissible virus dementia (Creutzfeldt-Jakob disease). N Engl J Med. 1977 Dec 8;297(23):1253–1258. doi: 10.1056/NEJM197712082972304. [DOI] [PubMed] [Google Scholar]
  11. Gajdusek D. C. Unconventional viruses and the origin and disappearance of kuru. Science. 1977 Sep 2;197(4307):943–960. doi: 10.1126/science.142303. [DOI] [PubMed] [Google Scholar]
  12. Hadlow W. J., Eklund C. M., Kennedy R. C., Jackson T. A., Whitford H. W., Boyle C. C. Course of experimental scrapie virus infection in the goat. J Infect Dis. 1974 May;129(5):559–567. doi: 10.1093/infdis/129.5.559. [DOI] [PubMed] [Google Scholar]
  13. Haire M. Significance of virus antibodies in multiple sclerosis. Br Med Bull. 1977 Jan;33(1):40–44. doi: 10.1093/oxfordjournals.bmb.a071394. [DOI] [PubMed] [Google Scholar]
  14. Hall W. W., Kiessling W., ter Meulen V. Membrane proteins of subacute sclerosing panencephalitis and measles viruses. Nature. 1978 Mar 30;272(5652):460–462. doi: 10.1038/272460a0. [DOI] [PubMed] [Google Scholar]
  15. Hall W. W., Lamb R. A., 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. 1979 Apr;76(4):2047–2051. doi: 10.1073/pnas.76.4.2047. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Hall W. W., ter Meulen V. RNA homology between subacute sclerosing panencephalitis and measles viruses. Nature. 1976 Dec 2;264(5585):474–477. doi: 10.1038/264474a0. [DOI] [PubMed] [Google Scholar]
  17. Horta-Barbosa L., Fuccillo D. A., Sever J. L., Zeman W. Subacute sclerosing panencephalitis: isolation of measles virus from a brain biopsy. Nature. 1969 Mar 8;221(5184):974–974. doi: 10.1038/221974a0. [DOI] [PubMed] [Google Scholar]
  18. Huddlestone J. R., Oldstone M. B. T suppressor (TG) lymphocytes fluctuate in parallel with changes in the clinical course of patients with multiple sclerosis. J Immunol. 1979 Oct;123(4):1615–1618. [PubMed] [Google Scholar]
  19. Jabbour J. T., Garcia J. H., Lemmi H., Ragland J., Duenas D. A., Sever J. L. Subacute sclerosing panencephalitis. A multidisciplinary study of eight cases. JAMA. 1969 Mar 24;207(12):2248–2254. doi: 10.1001/jama.207.12.2248. [DOI] [PubMed] [Google Scholar]
  20. Kreth H. W., Wiegand G. Cell-mediated cytotoxicity against measles virus in SSPE. II. Analysis of cytotoxic effector cells. J Immunol. 1977 Jan;118(1):296–301. [PubMed] [Google Scholar]
  21. Masters C. L., Richardson E. P., Jr Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). The nature and progression of spongiform change. Brain. 1978 Jun;101(2):333–344. doi: 10.1093/brain/101.2.333. [DOI] [PubMed] [Google Scholar]
  22. Modlin J. F., Halsey N. A., Eddins D. L., Conrad J. L., Jabbour J. T., Chien L., Robinson H. Epidemiology of subacute sclerosing panencephalitis. J Pediatr. 1979 Feb;94(2):231–236. doi: 10.1016/s0022-3476(79)80829-x. [DOI] [PubMed] [Google Scholar]
  23. Modlin J. F., Jabbour J. T., Witte J. J., Halsey N. A. Epidemiologic studies of measles, measles vaccine, and subacute sclerosing panencephalitis. Pediatrics. 1977 Apr;59(4):505–512. [PubMed] [Google Scholar]
  24. Narayan O., Penney J. B., Jr, Johnson R. T., Herndon R. M., Weiner L. P. Etiology of progressive multifocal leukoencephalopathy. Identification of papovavirus. N Engl J Med. 1973 Dec 13;289(24):1278–1282. doi: 10.1056/NEJM197312132892405. [DOI] [PubMed] [Google Scholar]
  25. Neighbour P. A., Bloom B. R. Absence of virus-induced lymphocyte suppression and interferon production in multiple sclerosis. Proc Natl Acad Sci U S A. 1979 Jan;76(1):476–480. doi: 10.1073/pnas.76.1.476. [DOI] [PMC free article] [PubMed] [Google Scholar]
  26. Norrby E., Link H., Olsson J. E., Panelius M., Salmi A., Vandvik B. Comparison of antibodies against different viruses in cerebrospinal fluid and serum samples from patients with multiple sclerosis. Infect Immun. 1974 Oct;10(4):688–694. doi: 10.1128/iai.10.4.688-694.1974. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Padgett B. L., Walker D. L. New human papovaviruses. Prog Med Virol. 1976;22:1–35. [PubMed] [Google Scholar]
  28. Padgett B. L., Walker D. L. Prevalence of antibodies in human sera against JC virus, an isolate from a case of progressive multifocal leukoencephalopathy. J Infect Dis. 1973 Apr;127(4):467–470. doi: 10.1093/infdis/127.4.467. [DOI] [PubMed] [Google Scholar]
  29. Perrin L. H., Tishon A., Oldstone M. B. Immunologic injury in measles virus infection. III. Presence and characterization of human cytotoxic lymphocytes. J Immunol. 1977 Jan;118(1):282–290. [PubMed] [Google Scholar]
  30. Risk W. S., Haddad F. S., Chemali R. Substantial spontaneous long-term improvement in subacute sclerosing panencephalitis. Six cases from the Middle East and a review of the literature. Arch Neurol. 1978 Aug;35(8):494–502. doi: 10.1001/archneur.1978.00500320014004. [DOI] [PubMed] [Google Scholar]
  31. Simpson J. F., Tourtellotte W. W., Kokmen E., Parker J. A., Itabashi H. H. Fluorescent protein tracing in multiple sclerosis brain tissue. Arch Neurol. 1969 Apr;20(4):373–377. doi: 10.1001/archneur.1969.00480100049007. [DOI] [PubMed] [Google Scholar]
  32. Takemoto K. K., Howley P. M., Miyamura T. JC human papovavirus replication in human amnion cells. J Virol. 1979 Apr;30(1):384–389. doi: 10.1128/jvi.30.1.384-389.1979. [DOI] [PMC free article] [PubMed] [Google Scholar]
  33. Terasaki P. I., Park M. S., Opelz G., Ting A. Multiple sclerosis and high incidence of a B lymphocyte antigen. Science. 1976 Sep 24;193(4259):1245–1247. doi: 10.1126/science.1085490. [DOI] [PubMed] [Google Scholar]
  34. Townsend J. J., Wolinsky J. S., Baringer J. R. The neuropathology of progressive rubella panencephalitis of late onset. Brain. 1976 Mar;99(1):81–90. doi: 10.1093/brain/99.1.81. [DOI] [PubMed] [Google Scholar]
  35. Vandvik B., Weil M. L., Grandien M., Norrby E. Progressive rubella virus panencephalitis: synthesis of oligoclonal virus-specific IgG antibodies and homogeneous free light chains in the central nervous system. Acta Neurol Scand. 1978 Jan;57(1):53–64. doi: 10.1111/j.1600-0404.1978.tb04497.x. [DOI] [PubMed] [Google Scholar]
  36. Wechsler S. L., Fields B. N. Differences between the intracellular polypeptides of measles and subacute sclerosing panencephalitis virus. Nature. 1978 Mar 30;272(5652):458–460. doi: 10.1038/272458a0. [DOI] [PubMed] [Google Scholar]
  37. Weil M. L., Itabashi H., Cremer N. E., Oshiro L., Lennette E. H., Carnay L. Chronic progressive panencephalitis due to rubella virus simulating subacute sclerosing panencephalitis. N Engl J Med. 1975 May 8;292(19):994–998. doi: 10.1056/NEJM197505082921903. [DOI] [PubMed] [Google Scholar]
  38. Wolinsky J. S., Dau P. C., Buimovici-Klein E., Mednick J., Berg B. O., Lang P. B., Cooper L. Z. Progressive rubella panencephalitis: immunovirological studies and results of isoprinosine therapy. Clin Exp Immunol. 1979 Mar;35(3):397–404. [PMC free article] [PubMed] [Google Scholar]
  39. Zu Rhein G. M. Association of papova-virions with a human demyelinating disease (progressive multifocal leukoencephalopathy). Prog Med Virol. 1969;11:185–247. [PubMed] [Google Scholar]

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