Abstract Details

Title MRI Features of Progressive Multifocal Leukoencephalopathy: Cortical Susceptibility Changes

Topic Multiple Sclerosis

Presentation(s) P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 9-016

Objective To describe the MRI features (including susceptibility changes) of progressive multifocal leukoencephalopathy (PML).

Background PML is a rare central nervous system (CNS) infection caused by reactivation of the John Cunningham virus (JCV).

Design/Methods Cases were identified via electronic medical record data search for PML diagnosis from 2008-2018 at our institution followed by retrospective review of medical records and MRI. Descriptive statistics and pairwise comparisons were applied.

Results Twenty-one PML cases were identified. 86% were male, mean age was 54 years ± 12 (mean ± SD). Five cases were secondary to disease-modifying therapy for multiple sclerosis (MS) (natalizumab), 8 were secondary to HIV infection, and 5 were secondary to other conditions (lymphoma, leukemia, or organ transplant). Most cases (81%) were diagnosed with JCV PCR in CSF, although four cases (19%) required brain biopsy. 7 cases (33%) were complicated by immune reconstitution syndrome (IRIS) and natalizumab was associated with higher risk of IRIS (RR = 8, 95% CI = 2.2-29.2). MRI involvement included frontal (80%), parietal (35%), brain stem (35%), cerebellar (35%), and occipital (25%) regions. There was a suggestion that PML in non-MS was more likely to be multifocal (RR = 3, 95% CI = 0.9-10.4). Enhancement was noted in 7 cases (35%) at onset. Cortical susceptibility changes were seen in 2 of 4 (50%) pre-PML MRIs and 6 of 16 (38%) of post-PML MRIs. Susceptibility changes were exclusively seen in the frontal lobe lesions in 83% of cases (n = 5). Four patients (18%) died; death was associated with absence of enhancement (RR = 5.1, 95% CI = 0.3-83.7) and both cerebellar and brainstem involvement (RR = 9, 95% CI = 1.2-68.1).

Conclusions

PML is a rare condition with high mortality. MS related PML appears milder and is associated with more inflammatory changes. Cortical susceptibility changes can be an early marker for PML.

Authors/Disclosures
Moein Amin, MD (Cleveland Clinic) PRESENTER	Dr. Amin has nothing to disclose.
Robert J. Fox, MD, FAAN (Cleveland Clinic)	Dr. Fox has received personal compensation in the range of $500-$4,999 for serving as a Consultant for AB Science. Dr. Fox has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Fox has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for BMS. Dr. Fox has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for EMD Serono. Dr. Fox has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech. Dr. Fox has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Immunic. Dr. Fox has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis. Dr. Fox has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Sanofi. Dr. Fox has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. Dr. Fox has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Siemens. Dr. Fox has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Roche. Dr. Fox has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astoria Biologica. Dr. Fox has received personal compensation in the range of $500-$4,999 for serving as a Consultant for InnoCare Pharma. Dr. Fox has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Fox has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Immunic. Dr. Fox has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Fox has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AB Science. Dr. Fox has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. Dr. Fox has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for BMS. The institution of Dr. Fox has received research support from National Institutes of Health. The institution of Dr. Fox has received research support from National MS Society. Dr. Fox has received publishing royalties from a publication relating to health care.
	No disclosure on file
Daniel Ontaneda, MD, PhD, FAAN (Cleveland Clinic)	Dr. Ontaneda has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Ontaneda has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech/Roche. Dr. Ontaneda has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen Idec. Dr. Ontaneda has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS. Dr. Ontaneda has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi. The institution of Dr. Ontaneda has received research support from NIH. The institution of Dr. Ontaneda has received research support from PCORI. The institution of Dr. Ontaneda has received research support from NMSS. The institution of Dr. Ontaneda has received research support from Genetech.