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.
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.
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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