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Case Report/Case Series |

JC Polyomavirus Granule Cell Neuronopathy in a Patient Treated With Rituximab

Louis Dang, MD, PhD1; Xin Dang, PhD2; Igor J. Koralnik, MD2; Peter K. Todd, MD, PhD1
[+] Author Affiliations
1Department of Neurology, University of Michigan, Ann Arbor
2Division of Neurovirology, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts
JAMA Neurol. 2014;71(4):487-489. doi:10.1001/jamaneurol.2013.4668.
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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.

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Brain Magnetic Resonance Imaging (MRI) in a Case of JC Polyomavirus Granule Cell Neuronopathy

At symptom onset, no significant cerebellar or white matter abnormalities were observed (A-C). One year after symptom onset, T1-weighted image demonstrates significant cerebellar atrophy (D). In addition, abnormal T2-weighted fluid-attenuated inversion recovery (FLAIR) signal involved the white matter tracts of the dorsal midbrain (E) and pons (F) and the cerebellar peduncles (F). The supratentorial white matter remained relatively spared.

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