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Progressive Multifocal Leukoencephalopathy: Why Gray and White Matter.
MedLine Citation:
PMID:  23092189     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Since it was first described in 1958, progressive multifocal leukoencephalopathy (PML), a demyelinating disease of the brain caused by the polyomavirus JC (JCV), has evolved tremendously. It was once considered a noninflammatory disease that affected exclusively oligodendrocytes and astrocytes in the white matter of immunosuppressed individuals and was almost always fatal. Today, we understand that PML can present during the course of an immune reconstitution inflammatory syndrome and that it affects a broader range of individuals, including patients with minimal immunosuppression and those who are treated with novel immunomodulatory medications. Furthermore, JCV-infected glial cells are frequently located at the gray matter-white matter junction or within the gray matter, causing demyelinating lesions within cortical areas. Finally, JCV variants can also infect neurons, leading to the recognition of two distinct clinical entities: JCV granule cell neuronopathy and JCV encephalopathy. Expected final online publication date for the Annual Review of Pathology: Mechanisms of Disease Volume 8 is January 24, 2013. Please see http://www.annualreviews.org/catalog/pubdates.aspx for revised estimates.
Authors:
Sarah Gheuens; Christian Wüthrich; Igor J Koralnik
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-10-18
Journal Detail:
Title:  Annual review of pathology     Volume:  -     ISSN:  1553-4014     ISO Abbreviation:  Annu Rev Pathol     Publication Date:  2012 Oct 
Date Detail:
Created Date:  2012-10-24     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101275111     Medline TA:  Annu Rev Pathol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Division of Neurovirology and Departments of Neurology and Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02215; email: sgheuens@bidmc.harvard.edu.
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