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 language | English (US) |
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Pages (from-to) | 487-489 |
Number of pages | 3 |
Journal | JAMA Neurology |
Volume | 71 |
Issue number | 4 |
DOIs | |
State | Published - Apr 2014 |
ASJC Scopus subject areas
- Clinical Neurology