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

Research output: Contribution to journalArticlepeer-review

10 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
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

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