Format

Send to:

Choose Destination
See comment in PubMed Commons below
Annu Rev Pathol. 2013 Jan 24;8:189-215. doi: 10.1146/annurev-pathol-020712-164018. Epub 2012 Oct 18.

Progressive multifocal leukoencephalopathy: why gray and white matter.

Author information

  • 1Division of Neurovirology and Departments of Neurology and Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA. sgheuens@bidmc.harvard.edu

Abstract

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.

PMID:
23092189
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Atypon
    Loading ...
    Write to the Help Desk