Objective: To describe a case of JC virus associated GCN.

Background: JC virus associated CNS disease most commonly presents as progressive multifocal leukoencephalopathy. A less recognized form of JC virus infection is GCN.

Methods: Case report.

Results: A 65 year old man, with history of diabetes mellitus and chronic lymphocytic leukemia in remission, treated with radiation, intermittent chlorambucil and 6 cycles of bendamustine and rituximab (last dose 9 months prior), presented with a 7 month history of progressive incoordination, imbalanced gait, falls, dizziness and nausea. Examination showed horizontal left beating nystagmus, vertical nystagmus on upgaze and subtle dysarthria. He had hypotonia, titubation and severe appendicular ataxia, left more than right. MRI brain showed moderate cerebellar atrophy without white matter changes or enhancement. Laboratory testing showed low serum thiamine levels of 3 nmol/L and mild elevation of anti-GAD 65 antibody to 6.5 Units/mL. Other serum autoimmune and paraneoplastic marker screening were negative. Serum HIV antibody and JC virus was negative. JC virus PCR was positive twice in CSF with low titer, between 200 – 500 copies/mL. Other CSF studies, including basic studies, cytology x 3, oligoclonal bands, IgG index and viral studies were negative. Whole body malignancy screening was negative. Thiamine repletion and two courses of IVIG with 3 days of high dose iv steroids lead to no improvement. A trial of mirtazapine was not helpful. Patient declined brain biopsy. Symptoms progressively worsened and he transitioned to palliative treatment with discharge to hospice 9 months after symptom onset.

Conclusions: This is a case of probable JC virus associated GCN. JC virus infection should be on the differential when evaluating rapidly progressive cerebellar ataxia in patients with history of immunosuppression, even without T2 flair white matter changes evident on the MRI.

This abstract was also submitted to the AAN conference in Vancouver April 15 – 21, 2016 (acceptance status unknown at this time).

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MDS Abstracts - https://www.mdsabstracts.org/abstract/sub-acute-rapidly-progressive-cerebellar-ataxia-a-case-of-jc-virus-associated-granule-cell-neuronopathy-gcn/