Journal of NeuroVirology

, 17:500

Progressive multifocal leukoencephalopathy associated with isolated CD8+ T-lymphocyte deficiency mimicking tumefactive MS

  • Jennifer L. McGuire
  • Vera Fridman
  • Christian Wüthrich
  • Igor J. Koralnik
  • Dina Jacobs
Case Report


  1. Berger JR, Levy RM, Flomenhoft D, Dobbs M (1998a) Predictive factors for prolonged survival in acquired immunodeficiency syndrome-associated progressive multifocal leukoencephalopathy. Ann Neurol 44(3):341–349PubMedCrossRefGoogle Scholar
  2. Berger JR, Pall L, Lanska D, Whiteman M (1998b) Progressive multifocal leukoencephalopathy in patients with HIV infection. J Neurovirol 4(1):59–68PubMedCrossRefGoogle Scholar
  3. Boerman R, Bax J, Beekhuis-Brussee J, Medaer R, Bollen L (1993) JC virus and multiple sclerosis: a refutation? Acta Neurol Scand 87:353–355PubMedCrossRefGoogle Scholar
  4. Boothpur R, Brennan DC (2010) Human polyoma viruses and disease with emphasis on clinical BK and JC. J Clin Virol 47(4):306–312PubMedCrossRefGoogle Scholar
  5. Brickelmaier M, Lugovskoy A, Kartikeyan R, Reviriego-Mendoza MM, Allaire N, Simon K et al (2009) Identification and characterization of mefloquine efficacy against JC virus in vitro. Antimicrob Agents Chemother 53(5):1840–1849PubMedCrossRefGoogle Scholar
  6. Cettomai D, McArthur JC (2009) Mirtazapine use in human immunodeficiency virus-infected patients with progressive multifocal leukoencephalopathy. Arch Neurol 66(2):255–258PubMedCrossRefGoogle Scholar
  7. Du Pasquier RA, Autissier P, Zheng Y, Jean-Jacques J, Koralnik IJ (2005) Presence of JC virus-specific CTL in the cerebrospinal fluid of PML patients: rationale for immune-based therapeutic strategies. AIDS 19(18):2069–2076PubMedCrossRefGoogle Scholar
  8. Epker JL, van Biezen P, van Daele PLA, van Gelder T, Vossen A, van Saase JLCM (2009) Progressive multifocal leukoencephalopathy, a review and an extended report of five patients with different immune compromised states. Eur J Inter Med 20(3):261–267CrossRefGoogle Scholar
  9. Ferrante P, Omodeo-Zorini E, Caldarelli-Stefano R, Mediatai M, Fainardi E, Granieri E et al (1998) Detection of JC virus DNA in cerebrospinal fluid from multiple sclerosis patients. Mult Scler 4:49–54PubMedGoogle Scholar
  10. Friedman R (2011) Malaria drug fails to fulfill promise in PML. Neurology Today 11(8):8CrossRefGoogle Scholar
  11. Gergely P (1999) Drug-induced lymphopenia: focus on CD4+ and CD8+ cells. Drug Saf 21(2):91–100PubMedCrossRefGoogle Scholar
  12. Gheuens S, Bord E (2011) Role of CD4+ and CD8+ T-cell responses against JC virus in the outcome of patients with progressive multifocal leukoencephalopathy (PML) and PML with immune reconstitution inflammatory syndrome. J Virol 85(14):2506–2510CrossRefGoogle Scholar
  13. Gheuens S, Pierone G, Peeters P, Koralnik IJ (2010) Progressive multifocal leukoencephalopathy in individuals with minimal or occult immunosuppression. J Neurol Neurosurg Psychiatr 81(3):247–254PubMedCrossRefGoogle Scholar
  14. Hogan BV, Peter MB, Shenoy HG, Horgan K, Hughes TA (2011) Surgery induced immunosuppression. Surgeon 9(1):38–43PubMedCrossRefGoogle Scholar
  15. Koralnik IJ (2004) New insights into progressive multifocal leukoencephalopathy. Curr Opin Neurol 17(3):365–370PubMedCrossRefGoogle Scholar
  16. Koralnik IJ (2006) Progressive multifocal leukoencephalopathy revisited: has the disease outgrown its name? Ann Neurol 60(2):162–173PubMedCrossRefGoogle Scholar
  17. Narula S, Larosa DF, Kamoun M, Dalmau J, Levinson AI (2007) Progressive multifocal leukoencephalopathy in a patient with common variable immunodeficiency and abnormal CD8+ T-cell subset distribution. Ann Allergy Asthma Immunol 98(5):483–489PubMedCrossRefGoogle Scholar
  18. Nishimura T, Kamikura I, Kamei S, Takasu T, Sugawara T, Hirayama H (1993) A unilateral large demyelinating lesion in the cerebral white matter with slowly progressive hemiplegia and intrathecal JC virus antibody production. Rinsho Shinkeigaku 33(3):301–306PubMedGoogle Scholar
  19. Sabath BF, Major EO (2002) Traffic of JC virus from sites of initial infection to the brain: the path to progressive multifocal leukoencephalopathy. J Infect Dis 186(Suppl 2):S180–S186PubMedCrossRefGoogle Scholar
  20. Stoner G (1991) Implications of progressive multifocal leukoencephalopathy and JC virus for the etiology of MS. Acta Neurol Scand 83:20–33PubMedCrossRefGoogle Scholar
  21. Tan CS, Koralnik IJ (2010) Progressive multifocal leukoencephalopathy and other disorders caused by JC virus: clinical features and pathogenesis. Lancet Neurol 9(4):425–437PubMedCrossRefGoogle Scholar
  22. Vermersch P, Kappos L, Gold R, Foley JF, Olsson T, Cadavid D et al (2011) Clinical outcomes of natalizumab-associated progressive multifocal leukoencephalopathy. Neurology 76(20):1697–1704PubMedCrossRefGoogle Scholar
  23. Weber T (2008) Progressive multifocal leukoencephalopathy. Neurol Clin 26(3):833–854, x-xiPubMedCrossRefGoogle Scholar

Copyright information

© Journal of NeuroVirology, Inc. 2011

Authors and Affiliations

  • Jennifer L. McGuire
    • 1
    • 2
  • Vera Fridman
    • 3
  • Christian Wüthrich
    • 4
    • 5
  • Igor J. Koralnik
    • 4
    • 5
  • Dina Jacobs
    • 6
  1. 1.Division of NeurologyThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  2. 2.Center for Clinical Epidemiology and BiostatisticsUniversity of Pennsylvania School of MedicinePhiladelphiaUSA
  3. 3.Department of NeurologyMassachusetts General HospitalBostonUSA
  4. 4.Division of Viral Pathogenesis, Department of MedicineBeth Israel Deaconess Medical CenterBostonUSA
  5. 5.Division of Neurovirology, Department of NeurologyBeth Israel Deaconess Medical CenterBostonUSA
  6. 6.Department of NeurologyHospital of the University of PennsylvaniaPhiladelphiaUSA

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