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Fulminant JC virus encephalopathy with productive infection of cortical pyramidal neurons

Authors

  • Christian Wüthrich PhD,

    1. Division of Viral Pathogenesis, Beth Israel Deaconess Medical Center, Beth Israel Deaconess Medical Center, Boston, MA
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  • Xin Dang PhD,

    1. Division of Viral Pathogenesis, Beth Israel Deaconess Medical Center, Beth Israel Deaconess Medical Center, Boston, MA
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  • Susan Westmoreland VMD,

    1. Department of Comparative Pathology, New England Primate Research Center, Beth Israel Deaconess Medical Center, Boston, MA
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  • John McKay BSc,

    1. Department of Comparative Pathology, New England Primate Research Center, Beth Israel Deaconess Medical Center, Boston, MA
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  • Atul Maheshwari MD,

    1. Department of Neurology, Brigham and Women's Hospital, Beth Israel Deaconess Medical Center, Boston, MA
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  • Matthew P. Anderson MD, PhD,

    1. Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA
    2. Department of Pathology, Beth Israel Deaconess Medical Center, Boston, MA
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  • Allan H. Ropper MD,

    1. Department of Neurology, Brigham and Women's Hospital, Beth Israel Deaconess Medical Center, Boston, MA
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  • Raphael P. Viscidi MD,

    1. Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD
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  • Igor J. Koralnik MD

    Corresponding author
    1. Division of Viral Pathogenesis, Beth Israel Deaconess Medical Center, Beth Israel Deaconess Medical Center, Boston, MA
    2. Department of Comparative Pathology, New England Primate Research Center, Beth Israel Deaconess Medical Center, Boston, MA
    3. Department of Neurology, Brigham and Women's Hospital, Beth Israel Deaconess Medical Center, Boston, MA
    4. Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA
    • BIDMC, 330 Brookline Avenue, E/CLS A005, Boston, MA 02215
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  • Potential conflict of interest: Nothing to report.

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. Ann Neurol 2009;65:742–748

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