Abstract Details

Title JC Virus Granule Cell Neuronopathy Mimicking Paraneoplastic Cerebellar Degeneration

Topic Infectious Disease

Presentation(s) P7 - Poster Session 7 (11:45 AM-12:45 PM)

Poster/Presentation
Number 13-004

Objective To report a case of JC virus granule cell neuronopathy (GCN) mimicking paraneoplastic cerebellar degeneration (PCD).

Background JC virus is known to cause opportunistic infections of the central nervous system in immunocompromised patients. GCN is a unique manifestation of JCV infection that often poses diagnostic challenges.

Design/Methods A 78-year-old woman with a history of rheumatoid arthritis and invasive ductal carcinoma of the right breast in remission presented with two months of progressive gait impairment, dysarthria, visual disturbance, and weight loss. Home medications included azathioprine and low-dose prednisone for rheumatoid arthritis treatment. Serum testing was notable for prominent lymphopenia (absolute lymphocyte count 0.5 x 10⁹ cells/L) and negative HIV antibody screening. Brain MRI revealed moderate cerebellar atrophy, T2/FLAIR hyperintensities in the bilateral middle cerebellar peduncles without associated enhancement, and a small area of left parieto-occipital T2/FLAIR hyperintensity. Cerebrospinal fluid (CSF) analysis revealed slightly elevated protein (62 mg/dL), normal cell counts, normal glucose, and absence of oligoclonal bands. Given breast cancer history and prominent weight loss, a diagnosis of PCD was initially suspected. However, evaluations for malignancy and paraneoplastic autoantibody testing were negative. Given negative malignancy screening, atypical MRI features beyond isolated cerebellar atrophy, and known immunosuppression, the diagnosis was re-evaluated.

Results CSF polymerase chain reaction testing for JC virus was positive. Given prominent cerebellar symptoms and imaging findings, a diagnosis of JC virus GCN was made with likely concomitant progressive multifocal leukoencephalopathy. Further testing revealed a CD4 lymphocyte count of 100 cells/microliter. Azathioprine and prednisone were discontinued. The patient was subsequently treated with pembrolizumab to accelerate immune reconstitution.

Conclusions GCN is a rare manifestation of JC virus infection in immunocompromised patients and can mimic PCD in patients with a history of relevant malignancy. Our case illustrates the importance of including GCN in the differential diagnosis when evaluating patients with progressive cerebellar symptoms.

Authors/Disclosures
Rumyar V. Ardakani, MD PRESENTER	Dr. Ardakani has nothing to disclose.
Barrie L. Zerwic, MD (University of Colorado, Anschutz Medical Campus)	Dr. Schmitt has nothing to disclose.
Aaron M. Carlson, MD (University of Colorado, School of Medicine, Department of Neurology)	Dr. Carlson has received research support from Horizon Therapeutics (Amgen).
Andrew Wolf, MD	The institution of Dr. Wolf has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. The institution of Dr. Wolf has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. The institution of Dr. Wolf has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Horizon. The institution of Dr. Wolf has received research support from Genentech. The institution of Dr. Wolf has received research support from Rocky Mountain MS Center. The institution of Dr. Wolf has received research support from Michael J. Fox Foundation. Dr. Wolf has received publishing royalties from a publication relating to health care.
Kenneth L. Tyler, MD, FAAN (University of Colorado School of Medicine)	Dr. Tyler has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Protagonist Therapeutics, Newark CA. Dr. Tyler has received personal compensation in the range of $50,000-$99,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for American Neurological Association/Wiley. Dr. Tyler has received intellectual property interests from a discovery or technology relating to health care. Dr. Tyler has received publishing royalties from a publication relating to health care. Dr. Tyler has a non-compensated relationship as a Director (ex officio) with American Neurological association that is relevant to AAN interests or activities. Dr. Tyler has a non-compensated relationship as a Director with International Society for Neurovirology that is relevant to AAN interests or activities.
Daniel Pastula, MD, MHS, FAAN (University of Colorado, Department of Neurology)	Dr. Pastula has nothing to disclose.
Amanda L. Piquet, MD, FAAN (University of Colorado)	The institution of Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genentech/Roche. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Kyverna . The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech/Roche. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. The institution of Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Sands Anderson PC. Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Joe Jones Law Firm. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Cortez & Associates. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Falk Waas. The institution of Dr. Piquet has received research support from Rocky Mountain MS Center. The institution of Dr. Piquet has received research support from Roche/Genentech. The institution of Dr. Piquet has received research support from NYU. The institution of Dr. Piquet has received research support from Anokion. The institution of Dr. Piquet has received research support from UCB . The institution of Dr. Piquet has received research support from Foundation for Sarcoidosis. The institution of Dr. Piquet has received research support from Kyverna . Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Litigative Consultant with US-Dept HHS/DICP. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Autoimmune Encephalitis Alliance (AEA) that is relevant to AAN interests or activities. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Stiff Person Syndrome Research Foundation (SPSRF) that is relevant to AAN interests or activities.

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