Progressive multifocal leukoencephalopathy and other forms of JC virus disease

Bruce J. Brew, Nicholas W S Davies, Paola Cinque, David B. Clifford, Avindra Nath

Research output: Contribution to journalArticle

Abstract

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the brain caused by the JC virus (JCV). PML usually occurs via reactivation of JCV when an immune system becomes compromised. A diagnosis of PML is normally made on the basis of distinguishing neurological features at presentation, characteristic brain MRI changes and the presence of JCV DNA in cerebrospinal fluid. PML has a 3 month mortality rate of 20-50%, so prompt intervention is essential. Currently, reconstitution of the immune system affords the best prognosis for this condition. When PML is first suspected, and where possible, immunosuppressant or immunomodulatory therapy should be suspended or reduced. If PML is associated with a protein therapy that has a long half-life the use of plasma exchange to accelerate the removal of the drug from the circulation may aid the restoration of immune system function. Rapid improvements in immune function, however, might lead to transient worsening of the disease. In this Review, we critically appraise the controversies surrounding JCV infection, and provide practical management guidelines for PML.

Original languageEnglish (US)
Pages (from-to)667-679
Number of pages13
JournalNature Reviews Neurology
Volume6
Issue number12
DOIs
StatePublished - Dec 2010

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JC Virus
Progressive Multifocal Leukoencephalopathy
Virus Diseases
Immune System
Plasma Exchange
Immunomodulation
Brain
Demyelinating Diseases
Immunosuppressive Agents
Half-Life
Cerebrospinal Fluid
Guidelines
Mortality
DNA

ASJC Scopus subject areas

  • Clinical Neurology
  • Cellular and Molecular Neuroscience

Cite this

Brew, B. J., Davies, N. W. S., Cinque, P., Clifford, D. B., & Nath, A. (2010). Progressive multifocal leukoencephalopathy and other forms of JC virus disease. Nature Reviews Neurology, 6(12), 667-679. https://doi.org/10.1038/nrneurol.2010.164

Progressive multifocal leukoencephalopathy and other forms of JC virus disease. / Brew, Bruce J.; Davies, Nicholas W S; Cinque, Paola; Clifford, David B.; Nath, Avindra.

In: Nature Reviews Neurology, Vol. 6, No. 12, 12.2010, p. 667-679.

Research output: Contribution to journalArticle

Brew, BJ, Davies, NWS, Cinque, P, Clifford, DB & Nath, A 2010, 'Progressive multifocal leukoencephalopathy and other forms of JC virus disease', Nature Reviews Neurology, vol. 6, no. 12, pp. 667-679. https://doi.org/10.1038/nrneurol.2010.164
Brew, Bruce J. ; Davies, Nicholas W S ; Cinque, Paola ; Clifford, David B. ; Nath, Avindra. / Progressive multifocal leukoencephalopathy and other forms of JC virus disease. In: Nature Reviews Neurology. 2010 ; Vol. 6, No. 12. pp. 667-679.
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