title: JC virus granule cell neuronopathy in the setting of chronic lymphopenia treated with recombinant interleukin-7.
abstract: JC virus (JCV) is a human polyomavirus that infects the central nervous system (CNS) of immunocompromised patients. JCV granule cell neuronopathy (JCV-GCN) is caused by infection of cerebellar granule cells, causing ataxia. A 77-year-old man with iatrogenic lymphopenia presented with severe ataxia and was diagnosed with JCV-GCN. His ataxia and cerebrospinal fluid (CSF) improved with intravenous immunoglobulin, high-dose intravenous methylprednisolone, mirtazapine, and mefloquine. Interleukin-7 (IL-7) therapy reconstituted his lymphocytes and reduced his CSF JCV load. One month after IL-7 therapy, he developed worsening ataxia and CSF inflammation, which raised suspicion for immune reconstitution inflammatory syndrome. Steroids were restarted and his ataxia stabilized.
authors.affiliation: Department of Neurology, Washington University School of Medicine, Campus Box 8111. 660 South Euclid Ave., St. Louis, MO, 63110, USA.
authors.forename: David N
authors.initials: DN
authors.lastname: Soleimani-Meigooni
authors.affiliation: Department of Pathology and Immunology, Washington University, St. Louis, MO, USA.
authors.forename: Katherine E
authors.initials: KE
authors.lastname: Schwetye
authors.affiliation: Department of Infectious Diseases, Washington University, St. Louis, MO, USA.
authors.forename: Maria Reyes
authors.initials: MR
authors.lastname: Angeles
authors.affiliation: Laboratory of Molecular Medicine and Neuroscience, National Institute of Neurological Disorders and Stroke, Bethesda, MD, USA.
authors.forename: Caroline F
authors.initials: CF
authors.lastname: Ryschkewitsch
authors.affiliation: Laboratory of Molecular Medicine and Neuroscience, National Institute of Neurological Disorders and Stroke, Bethesda, MD, USA.
authors.forename: Eugene O
authors.initials: EO
authors.lastname: Major
authors.affiliation: Department of Neurology, Division of Neuro-Immunology and Center for Virology and Vaccine Research, Beth Israel Deaconess Medical Center, Boston, MA, USA.
authors.forename: Xin
authors.initials: X
authors.lastname: Dang
authors.affiliation: Department of Neurology, Division of Neuro-Immunology and Center for Virology and Vaccine Research, Beth Israel Deaconess Medical Center, Boston, MA, USA.
authors.forename: Igor J
authors.initials: IJ
authors.lastname: Koralnik
authors.affiliation: Department of Pathology and Immunology, Washington University, St. Louis, MO, USA.
authors.forename: Robert E
authors.initials: RE
authors.lastname: Schmidt
authors.affiliation: Department of Neurology, Washington University School of Medicine, Campus Box 8111. 660 South Euclid Ave., St. Louis, MO, 63110, USA.
authors.forename: David B
authors.initials: DB
authors.lastname: Clifford
authors.affiliation: Department of Infectious Diseases, Washington University, St. Louis, MO, USA.
authors.forename: F Matthew
authors.initials: FM
authors.lastname: Kuhlmann
authors.affiliation: Department of Neurology, Washington University School of Medicine, Campus Box 8111. 660 South Euclid Ave., St. Louis, MO, 63110, USA. bucellir@neuro.wustl.edu.
authors.forename: Robert C
authors.initials: RC
authors.lastname: Bucelli
mesh_terms: D000368:Aged; D001259:Ataxia / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000821:virology; D002908:Chronic Disease; D006223:Hamartoma Syndrome, Multiple / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000821:virology; D006801:Humans; D016867:Immunocompromised Host; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D015851:Interleukin-7 / Q000627:therapeutic use*; D007577:JC Virus / Q000276:immunology / Q000472:pathogenicity / Q000502:physiology; D007968:Leukoencephalopathy, Progressive Multifocal / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000821:virology; D008231:Lymphopenia / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000821:virology; D008297:Male; D065703:Malformations of Cortical Development, Group I / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000821:virology; D015767:Mefloquine / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D008803:Mianserin / Q000031:analogs & derivatives / Q000627:therapeutic use; D000078785:Mirtazapine; D011994:Recombinant Proteins / Q000627:therapeutic use
chemical_list: C508605:IL7 protein, human; D016756:Immunoglobulins, Intravenous; D015851:Interleukin-7; D011994:Recombinant Proteins; D008803:Mianserin; D000078785:Mirtazapine; D015767:Mefloquine; D008775:Methylprednisolone
keywords: Cerebellar ataxia; Granule cell neuronopathy; Il-7; JC virus; Lymphopenia