title: Fulminant JC virus encephalopathy with productive infection of cortical pyramidal neurons.
abstract: 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.
authors: Division of Viral Pathogenesis, Beth Israel Deaconess Medical Center, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA.
authors: Christian
authors: C
authors: Wüthrich
authors: Xin
authors: X
authors: Dang
authors: Susan
authors: S
authors: Westmoreland
authors: John
authors: J
authors: McKay
authors: Atul
authors: A
authors: Maheshwari
authors: Matthew P
authors: MP
authors: Anderson
authors: Allan H
authors: AH
authors: Ropper
authors: Raphael P
authors: RP
authors: Viscidi
authors: Igor J
authors: IJ
authors: Koralnik
mesh_terms: D000368:Aged; D002540:Cerebral Cortex / Q000473:pathology* / Q000821:virology; D005260:Female; D006801:Humans; D007577:JC Virus / Q000302:isolation & purification; D007968:Leukoencephalopathy, Progressive Multifocal / Q000175:diagnosis* / Q000821:virology; D017966:Pyramidal Cells / Q000473:pathology* / Q000821:virology