HIV-Infekt pp 517-527 | Cite as

Clinical and Neuropathological Features in AIDS Patients with Progressive Multifocal Leukoencephalopathy (PML) Including a Novel Approach by in situ PCR

  • R. W. von Einsiedel
  • I. W. Samorei
  • M. Pawlita
  • M. Deubel
  • C. Mundt
  • H. V. Vinters
Conference paper


Opportunistic infection of the central nervous system by human Polyomavirus JC (JCV) can cause a devastating disease, progressive multifocal leukoencephalopathy (PML). We reviewed the clinical features of 15 patients with acquired immune deficiency syndrome (AIDS) and PML who came to autopsy or biopsy between 1983 and 1990, and investigated brain tissue with conventional and modern molecular biology techniques.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Brooks BR, Walker DL (1984) Progressive multifocal leukoenephalopathy. Neurol Clin 2: 299 - 313PubMedGoogle Scholar
  2. 2.
    Aström KE, Mancali EL, Richardson EP (1958) Progressive multifocal leukoencephalopathy. Brain 81: 93 - 111PubMedCrossRefGoogle Scholar
  3. 3.
    Einsiedel von RW, Fife TD, Aksamit AJ, Cornford ME, Secor DL, Tomiyasu U, Itabashi HH, Vinters HV (1993) Progressive multifocal leukoencephalopathy in AIDS: a clinicopathologic study and review of the literature. J Neurol 240: 391–406CrossRefGoogle Scholar
  4. 4.
    Aksamit AJ, Mourrain P, Sever JL, Major EO (1985) Progressive multifocal leukoencephalopathy: investigation of three cases using in situ hybridization with JC virus biotinylated DNA probe. Ann Neurol 18: 490–496.PubMedCrossRefGoogle Scholar
  5. 5.
    Houff SA, Major E, Katz DA, Kufta CV, Sever JL, Pittaluga S, Roberts JR, Gitt J, Saini N, Lux W (1988) Involvement of JC virus-infected mononuclear cells from the bone marrow and spleen in the pathogenesis of progressive multifocal leukoencephalopathy. N Engl J Med 318: 301 - 305PubMedCrossRefGoogle Scholar
  6. 6.
    Nuovo GJ, Gallery F, MacConnell P, Becker J, Bloch W (1991a) An improved technique for the in situ detection of DNA after polymerase chain reaction amplification. Ain J Pathol 139: 1239–1244Google Scholar
  7. 7.
    Bagasra O, Hauptman SP, Lischner HW, Sachs M, Pomerantz RJ (1992) Detection of human immunodeficiency virus type 1 provirus in mononuclear cells by in situ polymerase chain reaction. N Engl J Med 326: 1385–1391PubMedCrossRefGoogle Scholar
  8. 8.
    ZuRhein GM and Chou SM (1965) Particles resembling papova viruses in human cerebral demy- elinating disease. Science 148: 1477–1479PubMedCrossRefGoogle Scholar
  9. 9.
    Samorei IW, Schmid M, Pawlita M, Vinters HV, Diebold K, Mündt Ch, von Einsiedel RW (1999) High sensitivity detection of JC-virus DNA in postmortem brain tissue by in situ PCR. (in press)Google Scholar
  10. 10.
    Cummings JL, Benson DF (1984) Subcortical dementia: review of an emerging concept. Arch Neurol 41: 874–879PubMedGoogle Scholar
  11. 11.
    Price RW, Brew B, Sidtis J, Rosenblum M, Scheck AC, Cleary P (1988) The brain in AIDS: central nervous system HIV infection and AIDS dementia complex. Science 239: 586 - 592PubMedCrossRefGoogle Scholar
  12. 12.
    Richardson EP (1961) Progressive multifocal leukoencephalopathy. N Engl J Med 265: 815– 823PubMedCrossRefGoogle Scholar
  13. 13.
    Anders KH, Guerra WF, Tomiyasu U, Verity MA, Vinters HV (1986) The neuropathology of AIDS. UCLA experience and review. Am J Pathol 124: 537–558PubMedGoogle Scholar
  14. 14.
    Wiley CA, Gräfe M, Kennedy C, Nelson JA (1988) Human immunodeficiency virus (HIV) and JC virus in aquired immunodeficiency syndrome ( AIDS) patients with progressive multifocal leukoencephalopathy. Acta Neuropathol (Berl) 76: 338–346CrossRefGoogle Scholar
  15. 15.
    Vazeux R, Cumont M, Girard PM, Nassif X, Trotot P, Marche C, Matthiessen L, Vedrenne C, Mikol J, Henin D, Katlama C, Bolgert F, Montagnier L (1990) Severe encephalitis resulting from coinfection with HIV and JC virus. Neurology 40: 944 - 948PubMedGoogle Scholar
  16. 16.
    Berger JR, Kaszovitz B, Post MJ, Dickinson G (1987) Progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection: a review of the literature with a report of sixteen cases. Ann Intern Med 107: 78 - 87PubMedGoogle Scholar
  17. 17.
    Krupp LB, Lipton RB, Swerdlow ML, Leeds NE, Llena J (1985) Progressive multifocal encephalopathy: clinical and radiographic features. Annals of Neurology 17: 344 - 349PubMedCrossRefGoogle Scholar
  18. 18.
    von Einsiedel RW, Minami E, Secor DL, Pang S, Chen ISY, Vinters HV (1990) Structural components of the AIDS dementia complex: correlation of brain parenchymal astrogliosis with HIV-1 DNA in cerebral tissues. J Neuropathol Exp Neurol 49: 351CrossRefGoogle Scholar
  19. 19.
    Pang S, Koyanagi Y, Miles S, Vinters HV, Chen ISY (1990) High levels of unintegrated HIV-1 DNA in brain tissue of AIDS dementia patients. Nature 343: 85 - 89PubMedCrossRefGoogle Scholar
  20. 20.
    Chesters PM, Heritage J, McCance DJ (1983) Persistence of DNA sequences of BK virus and JC virus in normal human tissues and in diseased tissues. J Infect Dis 147: 676 - 684PubMedCrossRefGoogle Scholar
  21. 21.
    Mori M, Kurata H, Tajima M, Shimada H (1991) JC virus detection by in situ hybridization in brain tissue from elderly patients. Ann Neurol 29: 4287–432PubMedCrossRefGoogle Scholar
  22. 22.
    Boldorini R, Cristina S, Vago L, Tosoni A, Guzzetti S, Costanzi G (1993) Ultrastructural studies in the lytic phase of progressive multifocal leukoencephalopathy in AIDS patients. Ultrastruct Pathol 17: 599–609PubMedCrossRefGoogle Scholar
  23. 23.
    Kepes J, Chou S, Price LJ (1975) Progressive leukoencephalopathy with 10 year survival in a patient with nontropical sprue: report of a case with unusual light and electron microscopic features. Neurology 25: 1006–1012PubMedGoogle Scholar
  24. 24.
    Weber T, Major EO (1997b) Progressive multifocal leukoencephalopathy: molecular biology, pathogenesis and clinical impact. Intervirology 40: 98–111PubMedCrossRefGoogle Scholar
  25. 25.
    von Einsiedel RW, Samorei IW, Pawlita M, Deubel ML, Mündt Ch, Schmid M, Vinters HV (1999) New JCV infection patterns by in situ PCR in different intracerebral cell types of AIDS patients with PML. SubmittedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2000

Authors and Affiliations

  • R. W. von Einsiedel
  • I. W. Samorei
  • M. Pawlita
  • M. Deubel
  • C. Mundt
  • H. V. Vinters

There are no affiliations available

Personalised recommendations