JC virus granule cell neuronopathy in the setting of chronic lymphopenia treated with recombinant interleukin-7

J Neurovirol. 2017 Feb;23(1):141-146. doi: 10.1007/s13365-016-0465-0. Epub 2016 Jul 15.

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.

Keywords: Cerebellar ataxia; Granule cell neuronopathy; Il-7; JC virus; Lymphopenia.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Ataxia / diagnosis
  • Ataxia / drug therapy*
  • Ataxia / immunology
  • Ataxia / virology
  • Chronic Disease
  • Hamartoma Syndrome, Multiple / diagnosis
  • Hamartoma Syndrome, Multiple / drug therapy*
  • Hamartoma Syndrome, Multiple / immunology
  • Hamartoma Syndrome, Multiple / virology
  • Humans
  • Immunocompromised Host*
  • Immunoglobulins, Intravenous / therapeutic use
  • Interleukin-7 / therapeutic use*
  • JC Virus / immunology
  • JC Virus / pathogenicity
  • JC Virus / physiology
  • Leukoencephalopathy, Progressive Multifocal / diagnosis
  • Leukoencephalopathy, Progressive Multifocal / drug therapy*
  • Leukoencephalopathy, Progressive Multifocal / immunology
  • Leukoencephalopathy, Progressive Multifocal / virology
  • Lymphopenia / diagnosis
  • Lymphopenia / drug therapy*
  • Lymphopenia / immunology
  • Lymphopenia / virology
  • Male
  • Malformations of Cortical Development, Group I / diagnosis
  • Malformations of Cortical Development, Group I / drug therapy*
  • Malformations of Cortical Development, Group I / immunology
  • Malformations of Cortical Development, Group I / virology
  • Mefloquine / therapeutic use
  • Methylprednisolone / therapeutic use
  • Mianserin / analogs & derivatives
  • Mianserin / therapeutic use
  • Mirtazapine
  • Recombinant Proteins / therapeutic use

Substances

  • IL7 protein, human
  • Immunoglobulins, Intravenous
  • Interleukin-7
  • Recombinant Proteins
  • Mianserin
  • Mirtazapine
  • Mefloquine
  • Methylprednisolone

Supplementary concepts

  • Cerebellar Granule Cell Hypertrophy and Megalencephaly