Journal of the Formosan Medical Association
Volume 106, Issue 2, Supplement , Pages S60-S64, 2007

Progressive Multifocal Leukoencephalopathy in an Immunocompetent Taiwanese Patient

  • Yung-Yee Chang

      Affiliations

    • Department of Neurology, Kaohsiung Chang-Gung Memorial Hospital, Kaohsiung, Taiwan
  • ,
  • Min-Yu Lan

      Affiliations

    • Department of Neurology, Kaohsiung Chang-Gung Memorial Hospital, Kaohsiung, Taiwan
  • ,
  • Cheng-Huei Peng

      Affiliations

    • Department of Neurology, Kaohsiung Chang-Gung Memorial Hospital, Kaohsiung, Taiwan
  • ,
  • Hsiu-Shan Wu

      Affiliations

    • Department of Neurology, Kaohsiung Chang-Gung Memorial Hospital, Kaohsiung, Taiwan
  • ,
  • DeChing Chang

      Affiliations

    • Institute of Molecular Biology, National Chung-Cheng University, Chia-Yi, Taiwan
  • ,
  • Jia-Shou Liu

      Affiliations

    • Department of Neurology, Kaohsiung Chang-Gung Memorial Hospital, Kaohsiung, Taiwan
    • Corresponding Author InformationCorrespondence to: Dr Jia-Shou Liu, Department of Neurology, Kaohsiung Chang-Gung Memorial Hospital, 123, Ta-Pei Road, Niao-Sung, Kaohsiung 833, Taiwan

Received 31 August 2005; received in revised form 2 November 2005; accepted 10 January 2006.

Article Outline

Progressive multifocal leukoencephalopathy (PML) is a deadly demyelinating brain disease caused by JC virus (JCV). Genomic analysis of viral isolates in these cases often shows prototype-like JCV and its variants, which is a virulent strain compared to the latent archetype virions mostly found in the kidney. Here, we report a 57-year-old man who suffered from a subacute course of cognitive impairment and multiple neurologic deficits. Neuroimaging, pathology, and virology studies showed multifocal leukoencephalopathy and the presence of JCV deoxyribonucleic acid in the cerebrospinal fluid. The prototype type 1 (Mad-1) strain of JCV was identified on viral genotyping obtained from brain tissue. No immune deficits were found. He responded poorly to a-interferon and antiviral treatment. This case suggests that lack of immune deficiency cannot exclude the possibility of PML as a cause of subacute leukoencephalopathy. Accumulated data with respect to the disease course, pathologic feature, and viral genomic subtyping may pave the way for future treatment against this devastating disease. [J Formos Med Assoc 2007;106(2 Suppl):S60-S64]

Key Words:  genotyping , JC virus , magnetic resonance imaging , polymerase chain reaction , progressive multifocal leukoencephalopathy

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References 

  1. Frisque RJ , Bream GL , Cannella MT . Human polyomavirus JC virus genome . J Virol . 1984;51:458–469
  2. Koralnik IJ . Polyomavirus-induced demyelination . In:  Dangond F editors. Disorders of Myelin in the Central and Peripheral Nervous System . Woburn: Butterworth-Heinemann; 2002;p. 293–310
  3. Padgett BL , Walker DL . Prevalence of antibodies in human sera against JC virus, an isolate from a case of progressive multifocal leukoencephalopathy . J Infect Dis . 1973;127:467–470
  4. Sabath BF , Major EO . Traffic of JC virus from sites of initial infection to the brain: the path to progressive multifocal leukoencephalopathy . J Infect Dis . 2002;186:S180–S186
  5. Chang D , Tsai RT , Wang M , et al.   Different genotypes of human polyomaviruses found in patients with autoimmune diseases in Taiwan . J Med Virol . 1996;48:204–209
  6. Wang M , Tsai RT , Ou WC , et al.   Treatment with cytotoxic immunosuppression agents increases urinary excretion of JCV in patients with autoimmune disease . J Med Virol . 2000;62:505–510
  7. Johnson RT . Evidence for polyomaviruses in human neurological diseases . Prog Clin Biol Res . 1983;105:183–190
  8. Safak M , Khalili K . An overview: human polyomavirus JC virus and its associated disorders . J Neurovirol . 2003;9(Suppl 1):3–9
  9. Padgett BL , Walker DL , ZuRhein GM , et al.   Cultivation of papova-like virus from human brain with progressive multifocal leucoencephalopathy . Lancet . 1971;1:1257–1260
  10. Agostini HT , Ryschkewitsch CF , Singer EJ , et al.   JC virus regulatory region rearrangements and genotypes in progressive multifocal leukoencephalopathy: two independent aspects of virus variation . J Gen Virol . 1997;78:659–664
  11. Pfister LA , Letvin NL , Koralnik IJ . JC virus regulatory region tandem repeats in plasma and central nervous system isolates correlate with poor clinical outcome in patients with progressive multifocal leukoencephalopathy . J Virol . 2001;75:5672–5676
  12. Telenti A , Aksamit AJ , Proper J , et al.   Detection of JC virus DNA by polymerase chain reaction in patients with progressive multifocal leukoencephalopathy . J Infect Dis . 1990;162:858–861
  13. Seth P , Diaz F , Major EO . Advances in the biology of JC virus and induction of progressive multifocal leukoencephalopathy . J Neurovirol . 2003;9:236–246
  14. Jensen PN , Major EO . A classification scheme for human polyomavirus JCV variants based on the nucleotide sequence of the noncoding regulatory region . J Neurovirol . 2001;7:280–287
  15. Arai Y , Tsutsui Y , Nagashima K , et al.   Autopsy case of the cerebellar form of progressive multifocal leukoencephalopathy without immunodeficiency . Neuropathology . 2002;22:48–56
  16. Antinori A , Ammassari A , De Luca A , et al.   Diagnosis of AIDS-related focal brain lesions: a decision-making analysis based on clinical and neuroradiologic characteristics combined with polymerase chain reaction assays in CSF . Neurology . 1997;48:687–694
  17. Major EO , Vacante DA . Human fetal astrocytes in culture support the growth of the neurotropic human polyomavirus, JCV . J Neuropathol Exp Neurol . 1989;48:425–436
  18. Jochum W , Weber T , Frye S , et al.   Detection of JC virus by anti-VP1 immunohistochemistry in brains with progressive multifocal leukoencephalopathy . Acta Neuropathol (Berl) . 1997;94:226–231
  19. Vago L , Cinque P , Sala E , et al.   JCV-DNA and BKV-DNA in the CNS tissue and CSF of AIDS patients and normal subjects. Study of 41 cases and review of the literature . J Acquir Immune Defic Syndr Hum Retrovirol . 1996;12:139–146
  20. McGuire D , Barhite S , Hollander H , et al.   JC virus DNA in cerebrospinal fluid of human immunodeficiency virus-infected patients: predictive value for progressive multifocal leukoencephalopathy . Ann Neurol . 1995;37:395–399
  21. Weber T , Turner RW , Frye S , et al.   Progressive multifocal leukoencephalopathy diagnosed by amplification of JC virus-specific DNA from cerebrospinal fluid . AIDS . 1994;8:49–57

PII: S0929-6646(09)60355-7

doi:10.1016/S0929-6646(09)60355-7

Journal of the Formosan Medical Association
Volume 106, Issue 2, Supplement , Pages S60-S64, 2007