John Cunningham virus
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John Cunningham virus, also known as human polyomavirus 2, universally known as the JC virus, is a ubiquitous double-stranded DNA virus of the polyomaviridae family 1. In immunocompromised individuals, reactivation can lead to a variety of disease of the central nervous system, the most common of which is progressive multifocal leukoencephalopathy (PML).
It has been reported that ~70% of adults have been exposed to this virus; however, no significant clinical syndrome has been associated with this acquisition in the majority of immunocompetent hosts 1.
Numerous causes of immunosuppression have been implicated in reactivation of the virus, including HIV/AIDS, malignancy (e.g. lymphoma), immonusuppressants, and immunomodulators (e.g. natalizumab for multiple sclerosis).
Infection with JC virus, usually asymptomatic, results in latent infection in various organs and tissues of the body. When patients are immunocompromised, the virus can reactivate and migrate to the central nervous system, with a predilection for glial cells, thus resulting in the classical leukoencephalopathy of progressive multifocal leukoencephalopathy (PML) 6.
Occasionally, mutations of the virus result in other cell types being targeted, with resultant distinct clinical manifestations, including 4-6:
infection of glial cells
infection of the cerebellar granular cells
infection of cortical pyramidal neurons
infection of the meninges and choroid plexus
History and etymology
The virus was first described by Åström et al. in 1958 2, and was first isolated in 1971 by Padgett et al. The latter group assigned the name of the patient to the virus which would nowadays almost certainly not be done due to contravention of confidentiality 3.
- 1. Bellizzi A, Anzivino E, Rodio D, Palamara A, Nencioni L, Pietropaolo V. New Insights on Human Polyomavirus JC and Pathogenesis of Progressive Multifocal Leukoencephalopathy. Clin Dev Immunol. 2013;2013:839719. doi:10.1155/2013/839719 - Pubmed
- 2. Astrom K, Mancall E, Richardson E. Progressive Multifocal Leuko-Encephalopathy; a Hitherto Unrecognized Complication of Chronic Lymphatic Leukaemia and Hodgkin's Disease. Brain. 1958;81(1):93-111. doi:10.1093/brain/81.1.93 - Pubmed
- 3. Padgett B, Walker D, ZuRhein G, Eckroade R, Dessel B. Cultivation of Papova-Like Virus from Human Brain with Progressive Multifocal Leucoencephalopathy. Lancet. 1971;1(7712):1257-60. doi:10.1016/s0140-6736(71)91777-6 - Pubmed
- 4. Bag A, Curé J, Chapman P, Roberson G, Shah R. JC Virus Infection of the Brain. AJNR Am J Neuroradiol. 2010;31(9):1564-76. doi:10.3174/ajnr.A2035 - Pubmed
- 5. Tan C & Koralnik I. Progressive Multifocal Leukoencephalopathy and Other Disorders Caused by JC Virus: Clinical Features and Pathogenesis. Lancet Neurol. 2010;9(4):425-37. doi:10.1016/S1474-4422(10)70040-5 - Pubmed
- 6. Dang L, Dang X, Koralnik I, Todd P. JC Polyomavirus Granule Cell Neuronopathy in a Patient Treated with Rituximab. JAMA Neurol. 2014;71(4):487-9. doi:10.1001/jamaneurol.2013.4668 - Pubmed