Fulminant JC virus encephalopathy with productive infection of cortical pyramidal neurons

Ann Neurol. 2009 Jun;65(6):742-8. doi: 10.1002/ana.21619.


The polyomavirus JC (JCV) is the causative agent of progressive multifocal leukoencephalopathy and of JCV granule cell neuronopathy. We present a human immunodeficiency virus-negative patient who experienced development of multiple cortical lesions, aphasia, and progressive cognitive decline after chemotherapy for non-small-cell lung cancer. Brain biopsy and cerebrospinal fluid polymerase chain reaction demonstrated JCV, and she had a rapidly fatal outcome. Postmortem analysis showed diffuse cortical lesions and areas of necrosis at the gray-white junction. Immunostaining showed a productive JCV infection of cortical pyramidal neurons, confirmed by electron microscopy, with limited demyelination. This novel gray matter syndrome expands the scope of JCV clinical presentation and pathogenesis.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Aged
  • Cerebral Cortex / pathology*
  • Cerebral Cortex / virology
  • Female
  • Humans
  • JC Virus* / isolation & purification
  • Leukoencephalopathy, Progressive Multifocal / diagnosis*
  • Leukoencephalopathy, Progressive Multifocal / virology
  • Pyramidal Cells / pathology*
  • Pyramidal Cells / virology