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Abstract Details

Unilateral Cortical Autoimmune Encephalitis: A Case Series and Comparison to Late-onset Rasmussen’s Encephalitis
Autoimmune Neurology
P7 - Poster Session 7 (11:45 AM-12:45 PM)
14-013

To report a novel anatomical pattern of autoimmune encephalitis characterized by strictly unilateral cortical inflammation and a clinical picture overlapping with late-onset Rasmussen’s encephalitis.
NA

We retrospectively gathered data of patients identified at two tertiary referral academic centers who met inclusion criteria.
We identified twelve cases (average age 65, +/- 19.8 years, 58% female). All patients had unilateral cortical inflammation manifesting with focal seizures, cognitive decline, hemicortical deficits, and unilateral MRI and/or EEG changes. Six cases were idiopathic, two paraneoplastic, two iatrogenic (in the setting of immune checkpoint inhibitors), and two post-COVID-19. Serologically, ten patients were seronegative, one had high titer anti-GAD65, and one had anti-NMDAR. Five patients met Rasmussen’s encephalitis criteria, and six did not fully meet the criteria but had symptoms significantly overlapping with the condition. Most patients had significant improvement with immunotherapy.
Unilateral cortical autoimmune encephalitis seems to be more prevalent in the elderly and more frequently idiopathic and seronegative. Patients with this anatomical variant of autoimmune encephalitis have overlapping features with late-onset Rasmussen’s encephalitis but are more responsive to immunotherapy. In cases refractory to immunotherapy, interventions used in refractory Rasmussen’s encephalitis may be considered, such as functional hemispherectomy.
Authors/Disclosures
Sophia F. Damman
PRESENTER 		Ms. Damman has nothing to disclose.
Persen Sukpornchairak, MD (University Hospitals Cleveland Medical Center) Dr. Sukpornchairak has nothing to disclose.
Amit Ahituv (NYU Langone Health) Ms. Ahituv has nothing to disclose.
Alex Y. Chen, MD Dr. Chen has nothing to disclose.
David Wang, MD (UnityPoint Health) Dr. Wang has nothing to disclose.
Komal Sawlani, MD Dr. Sawlani has nothing to disclose.
Claude Steriade, MD (NYU) The institution of Dr. Steriade has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for The Epilepsy Study Consortium. Dr. Steriade has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Dynamed. Dr. Steriade has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for DOJ. The institution of Dr. Steriade has received research support from NIH. Dr. Steriade has received personal compensation in the range of $500-$4,999 for serving as a Consultant with Epitel. Dr. Steriade has received personal compensation in the range of $500-$4,999 for serving as a Consultant with Jazz Pharmaceuticals. Dr. Steriade has received personal compensation in the range of $10,000-$49,999 for serving as a Speakers Bureau with SK Life Sciences. Dr. Steriade has received personal compensation in the range of $5,000-$9,999 for serving as a Speakers Bureau with Neurelis. Dr. Steriade has received personal compensation in the range of $5,000-$9,999 for serving as a Advisory Board with Xenon Pharmaceuticals.
Hesham A. Abboud, MD (University Hospitals Cleveland Medical Center) Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. The institution of Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech . Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Horizon. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alpine Pharma. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cycle Pharma. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Axonics. Dr. Abboud 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. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen. Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Genentech. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for BMS. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Horizon. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for TG Therapeutics. The institution of Dr. Abboud has received research support from Genentech . The institution of Dr. Abboud has received research support from Novartis. The institution of Dr. Abboud has received research support from BMS. The institution of Dr. Abboud has received research support from Sanofi-Genzyme. The institution of Dr. Abboud has received research support from The Guthy-Jackson Charitable Foundation. The institution of Dr. Abboud has received research support from UCB. Dr. Abboud has received publishing royalties from a publication relating to health care.