TY - JOUR
T1 - Progressive multifocal leukoencephalopathy and other forms of JC virus disease
AU - Brew, Bruce J.
AU - Davies, Nicholas W.S.
AU - Cinque, Paola
AU - Clifford, David B.
AU - Nath, Avindra
N1 - Funding Information:
N. W. S. Davies receives funding from the Peel Medical Trust. Laurie Barclay, freelance writer and reviewer, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.
PY - 2010/12
Y1 - 2010/12
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=78649969090&partnerID=8YFLogxK
U2 - 10.1038/nrneurol.2010.164
DO - 10.1038/nrneurol.2010.164
M3 - Review article
C2 - 21131916
AN - SCOPUS:78649969090
SN - 1759-4758
VL - 6
SP - 667
EP - 679
JO - Nature Reviews Neurology
JF - Nature Reviews Neurology
IS - 12
ER -