Progressive multifocal leukoencephalopathy: Why gray and white matter

Sarah Gheuens, Christian Wüthrich, Igor J. Koralnik

Research output: Contribution to journalReview articlepeer-review

87 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)189-215
Number of pages27
JournalAnnual Review of Pathology: Mechanisms of Disease
Volume8
DOIs
StatePublished - Jan 2013

Keywords

  • Glial cells
  • HIV
  • Immune reconstitution inflammatory syndrome
  • JC virus
  • Natalizumab
  • Neurons

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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