Abstract Details

Title Autoimmune Encephalitis in Patients on Immune Checkpoint Inhibitor Therapy: a Multi-center Retrospective Study

Topic Autoimmune Neurology

Presentation(s) P6 - Poster Session 6 (5:00 PM-6:00 PM)

Poster/Presentation
Number 2-002

Objective To characterize immune-related encephalitis (irE) disease trajectory and identify predictors of recovery.

Background Immune checkpoint inhibitors (ICI) are increasingly used and have improved outcomes in cancer treatment; however, a subset of patients experience immune-related adverse events (irAEs), including autoimmune encephalitis (irE).

Design/Methods We retrospectively reviewed patients who received ICI and had treating physician-documented diagnosis of irE. We fit a multivariable ordinal regression model after screening candidate predictors of irE recovery.

Results We identified 41 patients (median age 71.7, 29.3% female) with irE. Melanoma (46.3%) and lung cancer (22.0%) were most frequently represented. 48.8% of patients had received ≥2 ICIs. 29.3% had CNS cancer involvement and 19.5% had prior CNS radiation. Presenting symptoms of irE included encephalopathy (90.2%), gait instability (24.4%), psychiatric/behavioral changes (19.5%), and movement disorder (17.1%). 67.5% of patients had lymphocytic pleocytosis with 2 patients testing positive for clinically significant autoantibodies (1 in serum, 1 in CSF). 10% had MRI findings of encephalitis. Only 3 patients fit definite or probable autoimmune encephalitis 2016 Graus criteria. 53.7% of patients had other systemic irAEs at time of presentation. All patients were treated with steroids: 52.5% had full and 35.0% had partial recovery, with 31 days median recovery time. 56.1% were deceased at the last follow up (median 8.4 months). ICI was discontinued in all cases and 4 patients were re-challenged (2 partial remission, 1 no remission, 1 deceased); none had recurrence of irE at last follow up. Higher antibody prevalence in epilepsy/encephalopathy (APE2) score (OR 0.20) and prior CNS radiation (OR 0.03) were significantly associated with lower odds of better recovery.

Conclusions Traditional clinical and imaging markers of autoimmune encephalitis are insensitive for irE. Patients with irE were steroid-responsive with most patients having a monophasic course. Higher APE2 scores and prior CNS radiation portend worse outcomes.

Authors/Disclosures
Prashanth Rajarajan, MD, PhD (Brigham and Women's Hospital) PRESENTER	Dr. Rajarajan has nothing to disclose.
Dylan Kirschenbaum, MD	Dr. Kirschenbaum has nothing to disclose.
Shalen Desai	Mr. Desai has nothing to disclose.
Jeffrey E. Dunn, MD, FAAN (Stanford University Medical Center)	Dr. Dunn has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech. Dr. Dunn has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genzyme. The institution of Dr. Dunn has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Progentec Diagnostics. Dr. Dunn has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna Therapeutics. Dr. Dunn has received intellectual property interests from a discovery or technology relating to health care.
Jamie C. McDonald, MD (Stanford University)	Dr. McDonald has nothing to disclose.
Shamik Bhattacharyya, MD, FAAN (Brigham and Women's Hospital)	Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NeuroLambda. Dr. Bhattacharyya has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Bhattacharyya has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Continuum. Dr. Bhattacharyya 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 Wiley. Dr. Bhattacharyya has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Merck. The institution of Dr. Bhattacharyya has received research support from Alexion Pharmaceuticals. The institution of Dr. Bhattacharyya has received research support from National Institute of Health. The institution of Dr. Bhattacharyya has received research support from UCB. The institution of Dr. Bhattacharyya has received research support from Genentech. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care.
Kristin M. Galetta, MD (Stanford University)	Dr. Galetta has received personal compensation in the range of $0-$499 for serving as a Speaker with Can Do MS.

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