A fatal case of JC virus meningitis presenting with hydrocephalus in a human immunodeficiency virus–seronegative patient

Authors

  • Shruti P. Agnihotri MD,

    1. Division of Neuro-Virology, Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA
    2. Center for Virology and Vaccine Research, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA
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  • Christian Wuthrich PhD,

    1. Division of Neuro-Virology, Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA
    2. Center for Virology and Vaccine Research, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA
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  • Xin Dang PhD,

    1. Division of Neuro-Virology, Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA
    2. Center for Virology and Vaccine Research, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA
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  • David Nauen MD,

    1. Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, MD
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  • Reza Karimi MD,

    1. Department of Neurosurgery, Hackensack University Medical Center, Hackensack, NJ
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  • Raphael Viscidi MD, PhD,

    1. Department of Pediatrics, Johns Hopkins Medical Institutions, Baltimore, MD
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  • Evelyn Bord BS,

    1. Division of Neuro-Virology, Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA
    2. Center for Virology and Vaccine Research, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA
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  • Stephanie Batson BS,

    1. Division of Neuro-Virology, Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA
    2. Center for Virology and Vaccine Research, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA
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  • Juan Troncoso MD,

    1. Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, MD
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  • Igor J. Koralnik MD

    Corresponding author
    1. Division of Neuro-Virology, Department of Neurology, Beth Israel Deaconess Medical Center, Boston, MA
    2. Center for Virology and Vaccine Research, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA
    • Address correspondence to Dr Koralnik, Division of Neuro-Virology, Beth Israel Deaconess Medical Center, CLS 1005, 330 Brookline Avenue, Boston, MA 02215. E-mail: ikoralni@bidmc.harvard.edu

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Abstract

JC virus (JCV) is the etiologic agent of progressive multifocal leukoencephalopathy, JCV granule cell neuronopathy, and JCV encephalopathy. Whether JCV can also cause meningitis has not yet been demonstrated. We report a case of aseptic meningitis resulting in symptomatic hydrocephalus in a human immunodeficiency virus–seronegative patient. Brain imaging showed enlargement of ventricles but no parenchymal lesion. She had a very high JC viral load in the cerebrospinal fluid (CSF) and developed progressive cognitive dysfunction despite ventricular drainage. She was diagnosed with pancytopenia and passed away after 5.5 months. Postmortem examination revealed productive JCV infection of leptomeningeal and choroid plexus cells, and limited parenchymal involvement. Sequencing of JCV CSF strain showed an archetype-like regulatory region. Further studies of the role of JCV in aseptic meningitis and in idiopathic hydrocephalus are warranted. Ann Neurol 2014;76:140–147

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