Abstract Details

Title Progressive Multifocal Leukoencephalopathy with Negative CSF PCR for JC Virus in an Immunocompetent Patient with Discoid Lupus

Topic Autoimmune Neurology

Presentation(s) Autoimmune Neurology Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 102

Objective To describe a case of progressive multifocal leukoencephalopathy (PML) associated with discoid (cutaneous) lupus.

Background PML is an opportunistic demyelinating infection caused by the human polyomavirus, JC virus (JCV). Although PML has been reported in patients with systemic lupus erythematosus (SLE), its association with discoid lupus has not been previously described.

Design/Methods Case Report

Results This 58-year-old male presented with worsening encephalopathy and incoordination. He had a history of alcohol use disorder and a 30-year history of discoid lupus. His discoid lupus never progressed to SLE, and he had never received systemic immunosuppression. Neurological examination was significant for expressive aphasia, left sided neglect, and upper extremity incoordination. ANA was positive at 1:160 without other positive rheumatological serologies. All infectious studies including those for HIV, hepatitis, and syphilis were negative. Quantitative immunoglobulins and lymphocyte panel were normal including CD4+/CD8+ values. Liver function tests were normal and abdominal CT scan showed no evidence of cirrhosis. CSF was acellular with protein of 54 mg/dL. MRI showed T2/FLAIR signal hyperintensities in bilateral parietal and temporal subcortical white matter, with involvement of subcortical U-fibers. These lesions were hypointense on the T1-weighted sequences and without contrast enhancement. JCV PCR was negative in CSF. Brain biopsy, however, showed oligodendrocytes with enlarged nuclei and intranuclear inclusions typical of PML; these cells were positive for polyomavirus (SV40) antigen, confirming the diagnosis. The patient was started on mirtazapine, but his family declined additional interventions; he was transitioned to comfort care.

Conclusions PML is rarely diagnosed in immunocompetent patients and has not, to our knowledge, been associated with discoid lupus. This case highlights the need for vigilance in patients without traditional risk factors and emphasizes the fact that failure to detect JCV by CSF PCR does not exclude PML.

Authors/Disclosures
Justin Abbatemarco, MD PRESENTER	Dr. Abbatemarco has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Abbatemarco has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Abbatemarco 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 . Dr. Abbatemarco has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics, Inc.. Dr. Abbatemarco has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. The institution of Dr. Abbatemarco has received research support from Amgen.
Justin Abbatemarco, MD	Dr. Abbatemarco has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Abbatemarco has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Abbatemarco 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 . Dr. Abbatemarco has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics, Inc.. Dr. Abbatemarco has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. The institution of Dr. Abbatemarco has received research support from Amgen.
Eric Stulberg, MD (University of Utah)	Dr. Stulberg has nothing to disclose.
	No disclosure on file
Robert Kadish, MD	Dr. Kadish has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. The institution of Dr. Kadish has received research support from Alexion Pharmaceuticals.
M. M. Paz Soldan, MD, PhD (Mayo Clinic)	Dr. Paz Soldan has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. The institution of Dr. Paz Soldan has received research support from National Institutes of Health. The institution of Dr. Paz Soldan has received research support from National Multiple Sclerosis Society. The institution of Dr. Paz Soldan has received research support from Western Institute for Biomedical Research. The institution of Dr. Paz Soldan has received research support from Biogen. The institution of Dr. Paz Soldan has received research support from Novartis. The institution of Dr. Paz Soldan has received research support from Clene Nanomedicine.
Stacey Clardy, MD, PhD, FAAN (University of Utah)	Dr. Clardy has received personal compensation for serving as an employee of Veterans Health Administration (VHA). Dr. Clardy has received personal compensation for serving as an employee of University of Utah Health. Dr. Clardy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca/Alexion. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen/Horizon. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. Dr. Clardy has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology/AAN Publications. The institution of Dr. Clardy has received research support from NIH/NINDS. The institution of Dr. Clardy has received research support from SRNA. The institution of Dr. Clardy has received research support from Alexion/AstraZeneca. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a AAN Summer Meeting CoDirector Travel and Lodging with AAN. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with U of Iowa, Miami, Stanford, Barrow, Beaumont Health, CCF, Emory, Penn State, Mayo Clinic, Walter Reed.
John E. Greenlee, MD, FAAN (University of Utah)	Dr. Greenlee has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Medlink. Dr. Greenlee has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Zeigler Cohen Roche. Dr. Greenlee has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Sommers Schwartz PC. Dr. Greenlee has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for St Francis Hospital. Dr. Greenlee has received publishing royalties from a publication relating to health care. Dr. Greenlee has received publishing royalties from a publication relating to health care.

��ɫ��

��ɫ��