Article

Journal of NeuroVirology

, Volume 11, Issue 1, pp 51-57

First online:

A case of a progressive multifocal leukoencephalopathy patient with four different JC virus transcriptional control region rearrangements in cerebrospinal fluid, blood, serum, and urine

  • Serena DelbueAffiliated withLaboratory of Biology, Don C. Gnocchi Foundation ONLUS, IRCCS
  • , Giovanni SotgiuAffiliated withClinic of Infectious Diseases, University of Pavia, Policlinico San Matteo
  • , Daniela FumagalliAffiliated withDepartment of Biomedical Science and Technology, University of Milan
  • , Marilena ValliAffiliated withLaboratory of Biology, Don C. Gnocchi Foundation ONLUS, IRCCS
  • , Elisa BorghiAffiliated withDepartment of Biomedical Science and Technology, University of Milan
  • , Roberta MancusoAffiliated withLaboratory of Biology, Don C. Gnocchi Foundation ONLUS, IRCCS
  • , Enrico MarchioniAffiliated withNeurology Department, Mondino Hospital
  • , Renato MaseratiAffiliated withClinic of Infectious Diseases, Policlinico San Matteo
  • , Pasquale FerranteAffiliated withLaboratory of Biology, Don C. Gnocchi Foundation ONLUS, IRCCSDepartment of Biomedical Science and Technology, University of Milan Email author 

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Abstract

JC virus (JCV) is the etiological agent of progressive multifocal leukoencephalopathy (PML), a fatal demyelinating disease of the central nervous system (CNS). During the acquired immunodeficiency syndrome (AIDS) epidemic, it was the cause of the death in up to 8% of AIDS patients. The genomic organization of JCV and, in particular, the hypervariability of the transcriptional control region (TCR), a regulatory noncoding region, are well known. Given that the TCR plays a central role in the viral replication of JCV, a crucial role in the determination of the neurotropism and in the pathogenic capabilities of the virus is also suspected. Here the authors describe a case of PML that did not respond to highly active antiretroviral therapy (HAART) therapy. There was a simultaneous presence of JCV strains with four different TCR structures in urine, peripheral blood cells, serum, and cerebrospinal fluid (CSF) samples. These data confirmed that the presence of the archetype TCR is restricted to urine, while also suggesting that the degree of the rearrangement varies and increases from the peripheral blood to CSF.

Keywords

cerebrospinal fluid (CSF) JC virus progressive multifocal leukoencephalopathy (PML) transcriptional control region (TCR) rearrangements