JC polyomavirus granule cell neuronopathy in a patient treated with rituximab

Louis Dang, Xin Dang, Igor J. Koralnik, Peter K. Todd*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

22 Scopus citations

Abstract

IMPORTANCE: Progressive multifocal leukoencephalopathy results from lytic infection of the glia by the JC polyomavirus (JCV); JCV granule cell neuronopathy is caused by infection with a mutated form of JCV, leading to a shift in viral tropism from the glia to cerebellar granule cells. This shift results in a clinical syndrome dominated by progressive cerebellar dysfunction that might elude standard diagnostic workup strategies for ataxia. OBSERVATIONS: We present the case report of a patient receiving long-term rituximab therapy who developed progressive cerebellar ataxia and marked isolated cerebellar degeneration. This syndrome resulted from JCV granule cell neuronopathy associated with a novel JCV mutation. CONCLUSIONS AND RELEVANCE: New onset or worsening of isolated cerebellar ataxia in patients being treated with rituximab or natalizumab warrants early assessment for JCV infection.

Original languageEnglish (US)
Pages (from-to)487-489
Number of pages3
JournalJAMA Neurology
Volume71
Issue number4
DOIs
StatePublished - Apr 2014

ASJC Scopus subject areas

  • Clinical Neurology

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