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

David N. Soleimani-Meigooni, Katherine E. Schwetye, Maria Reyes Angeles, Caroline F. Ryschkewitsch, Eugene O. Major, Xin Dang, Igor J. Koralnik, Robert E. Schmidt, David B. Clifford, F. Matthew Kuhlmann, Robert C. Bucelli*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

8 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)141-146
Number of pages6
JournalJournal of neurovirology
Volume23
Issue number1
DOIs
StatePublished - Feb 1 2017

Keywords

  • Cerebellar ataxia
  • Granule cell neuronopathy
  • Il-7
  • JC virus
  • Lymphopenia

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Virology

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